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Sample records for causing severe neutropenia

  1. Genetics Home Reference: severe congenital neutropenia

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    ... Home Health Conditions severe congenital neutropenia severe congenital neutropenia Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Severe congenital neutropenia is a condition that causes affected individuals to ...

  2. Severe congenital neutropenia

    DEFF Research Database (Denmark)

    Borregaard, Niels

    2014-01-01

    In this issue of Blood, Tidwell et al1 demonstrate that mutations in the start codon (protein synthesis is initiated at the codon ATG) of neutrophil elastase (ELANE) result in the production of N-terminally truncated elastase, which mislocates to the nucleus and results in severe congenital neutr...... neutropenia (SCN)....

  3. Paternal Somatic Mosaicism of a Novel Frameshift Mutation in ELANE Causing Severe Congenital Neutropenia.

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    Kim, Hee-Jung; Song, Min-Jung; Lee, Ki-O; Kim, Sun-Hee; Kim, Hee-Jin

    2015-12-01

    Severe congenital neutropenia (SCN) is a bone marrow failure disease with an autosomal dominant inheritance from mutations in ELANE. Here, we report a 7-week-old Korean male with SCN. His elder sister died from pneumonia at 2 years. Direct sequencing of ELANE in the proband identified a heterozygous novel frameshift mutation: c.658delC (p.Arg220Glyfs20*). Family study involving his asymptomatic parents with normal cell counts revealed that his father had the same mutation, but at a lower burden than expected in a typical heterozygous state. Further molecular investigation demonstrated somatic mosaicism with ~18% mutant alleles. We concluded the proband inherited the mutation from his somatic mosaic father.

  4. [An unusual cause of febrile neutropenia: brucellosis].

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    Solmaz, Soner; Asma, Süheyl; Ozdoğu, Hakan; Yeral, Mahmut; Turunç, Tuba

    2014-10-01

    Febrile neutropenia which is a common complication of cancer treatment, is one of the major causes of morbidity and mortality. Several gram-negative and gram-positive bacteria are responsible for infections in neutropenic patients, however the most common microorganisms are Escherichia coli and coagulase-negative staphylococci, in decreasing order. Although Brucella spp. infections are endemic in Turkey, brucellosis-related febrile neutropenia has only rarely been reported. In this report, a case of brucellosis-related febrile neutropenia in a patient with acute myeloblastic leukemia (AML) was presented. A 56-year-old male patient presenting with fever, petechiae/purpura, leukocytosis, thrombocytopenia, and anemia was admitted to our hospital. Laboratory studies revealed a hemoglobin level of 8.27 g/dl, leukocyte count of 77.100 k/ml, absolute neutrophil count of 200 k/ml, and platelets at 94.200 k/ml. The patient was diagnosed as AML-M1 and piperacillin/tazobactam was started as the first-line antibiotic therapy due to the febrile neutropenia. On admission, blood and urine cultures were negative. Once the fever was controlled, remission/induction chemotherapy was initiated. However, fever developed again on the eight day, and vancomycin was added to the therapy. Since the fever persisted, the antibiotic therapy was gradually replaced with meropenem and linezolid. However, fever continued and the patient's general condition deteriorated. Subsequently performed Brucella tube agglutination test revealed positivity at 1/320 titer and the microorganism grown in blood culture (Bactec 9050; BD, USA) was identified as B.melitensis by conventional methods. Rifampicin and doxycycline therapy was started immediately, however, the patient died due to septic shock. If the tests for brucellosis were performed earlier when response to second step antibiotic therapy lacked in this patient, it was assumed that mortality could be prevented by the prompt initiation of the

  5. Severe neutropenia in dengue patients: prevalence and significance.

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    Thein, Tun-Linn; Lye, David C; Leo, Yee-Sin; Wong, Joshua G X; Hao, Ying; Wilder-Smith, Annelies

    2014-06-01

    Studies on severe neutropenia in dengue are scarce, and its clinical significance is uncertain. We analyzed a cohort of 1,921 reverse transcription polymerase chain reaction-confirmed adult dengue patients admitted to the Communicable Disease Center in Singapore between 2005 and 2008. Time trend analyses for daily absolute neutrophil counts (ANCs) were done using Bayesian hierarchical and Markov models. We found that severe neutropenia, defined as ANC ≤ 0.5 × 10(9)/L, was found in 11.8% with a median duration of 1 day. ANC nadir occurred on illness day 5. Severe neutropenia was not predictive of more severe disease and not associated with secondary bacterial infections, prolonged hospital stay, prolonged fever, or fatal outcome. We concluded that prophylactic antibiotics are not indicated in patients with severe neutropenia without indication for bacterial infection.

  6. Intensified prophylaxis of febrile neutropenia with ofloxacin plus rifampin during severe short-duration neutropenia in patients with lymphoma.

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    Muñoz, L; Martino, R; Subirà, M; Brunet, S; Sureda, A; Sierra, J

    1999-08-01

    To analyse the impact of intensified prophylaxis with ofloxacin plus rifampin (O+R) in neutropenic patients we used this combination in 40 consecutive cycles of ifosfamide, cytarabine, prednisolone and etoposide (IAPVP-16). This salvage chemotherapy regimen for lymphoma usually produces four to six days of severe neutropenia without significant extrahematologic toxicities. We compared the infectious morbidity during neutropenia under O+R with 58 consecutives cycles using either norfloxacin or no prophylaxis (control group). Fifty-three percent of control group patients and 20% of the O+R group developed febrile neutropenia that required hospital admission (pfebril neutropenia was 42 days and 158 days, respectively (pfebrile neutropenia and GPC bacteremia in patients with short and severe neutropenia, which translates into a reduction in the need for hospitalization.

  7. Double de novo mutations of ELA2 in cyclic and severe congenital neutropenia.

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    Salipante, Stephen J; Benson, Kathleen F; Luty, Joanna; Hadavi, Valeh; Kariminejad, Roxana; Kariminejad, Mohamad H; Rezaei, Nima; Horwitz, Marshall S

    2007-09-01

    Heterozygous mutations of ELA2, encoding the protease neutrophil elastase (NE), cause either autosomal dominant cyclic neutropenia or severe congenital neutropenia (SCN). Three hypotheses have been proposed for how allelic mutations produce these different disorders: 1) disruption of proteolytic activity; 2) mislocalization of the protein; or 3) destabilization of the protein resulting in induction of the unfolded protein response. As with other dominant diseases with reduced reproductive fitness, sporadic cases can result from new mutations not inherited from either parent. Here we report an exceptional genetic phenomenon in which both a cyclic neutropenia patient and an SCN patient each possess two new ELA2 mutations. Because of the rarity of the phenomenon, we investigated the origins of the mutations and found that both arise nonmosaically and in cis from the paternally-inherited allele. Moreover, these cases offer a unique opportunity to investigate molecular pathways distinguishing these two forms of hereditary neutropenia. We have characterized the mutants separately and in combination, with respect to their effects on proteolysis, subcellular trafficking, and induction of the unfolded protein response. Each pair of mutations acts more or less additively to produce equivalent net effects on reducing proteolytic activity and induction of the unfolded protein response, yet each has different and somewhat opposing effects on disturbing subcellular localization, thus offering support for a role for protein mistrafficking as a disease mechanism. PMID:17436313

  8. Copper deficiency as a cause of neutropenia in a case of coeliac disease.

    Science.gov (United States)

    Khera, Daisy; Sharma, Baldev; Singh, Kuldeep

    2016-01-01

    We report a 17 year-old male patient, who presented with chronic diarrhoea, progressive pallor, short stature, anaemia (haemoglobin of 4.9 g/dL) and neutropenia and was diagnosed as coeliac disease. His neutropenia did not respond to 8 months of gluten-free diet, iron, folic acid and vitamin B12 therapy. So we suspected copper deficiency and his serum copper levels were tested, which was low. His neutrophil counts normalised after 2 months of copper supplementation. Hence we concluded that the cause of neutropenia in our case was copper deficiency. PMID:27635061

  9. Cotrimoxazole prophylaxis and risk of severe anemia or severe neutropenia in HAART-exposed, HIV-uninfected infants.

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    Scott Dryden-Peterson

    Full Text Available BACKGROUND: Prophylactic cotrimoxazole is recommended for infants born to HIV-infected mothers. However, cotrimoxazole may increase the risk of severe anemia or neutropenia. METHODS: We compared the proportion of HIV-exposed uninfected (HIV-EU infants experiencing incident severe anemia (and separately, severe neutropenia between a prospective cohort receiving prophylactic cotrimoxazole from 1 to 6 months vs. infants from two prior trials who did not receive cotrimoxazole. Infants were from rural and urban communities in southern Botswana. RESULTS: A total of 1705 HIV-EU infants were included. Among these 645 (37.8% were fed with iron-supplemented formula from birth. Severe anemia developed in 87 (5.1% infants, and severe neutropenia in 164 (9.6% infants. In an analysis stratified by infant feeding method, there were no significant differences in the risk of severe anemia by prophylactic cotrimoxazole exposure-risk difference, -0.69% (95% confidence interval [CI] -2.1 to 0.76%. Findings were similar in multivariable analysis, adjusted odds ratio (aOR 0.35 (95% CI 0.07 to 1.65. There were also no significant differences observed for severe neutropenia by cotrimoxazole exposure, risk difference 2.0% (95% CI -1.3 to 5.2% and aOR 0.80 (95% CI 0.33 to 1.93. CONCLUSIONS: Severe anemia and severe neutropenia were infrequent among HIV-exposed uninfected infants receiving cotrimoxazole from 1-6 months of age. Concerns regarding hematologic toxicity should not limit the use of prophylactic cotrimoxazole in HIV-exposed uninfected infants. CLINICALTRIAL.SGOV REGISTRATION NUMBERS: NCT01086878 (http://clinicaltrials.gov/show/NCT01086878, NCT00197587 (http://clinicaltrials.gov/show/NCT00197587, and NCT00270296 (http://clinicaltrials.gov/show/NCT00270296.

  10. Severe congenital neutropenia with neurological impairment due to a homozygous VPS45 p.E238K mutation: A case report suggesting a genotype-phenotype correlation.

    Science.gov (United States)

    Meerschaut, Ilse; Bordon, Victoria; Dhooge, Catharina; Delbeke, Patricia; Vanlander, Arnaud V; Simon, Amos; Klein, Christoph; Kooy, R Frank; Somech, Raz; Callewaert, Bert

    2015-12-01

    VPS45 mutations cause severe congenital neutropenia (SCN). We report on a girl with SCN and neurological impairment harboring a homozygous p.E238K mutation in VPS45 (vacuolar sorting protein 45). She successfully underwent hematopoietic stem cell transplantation. Our findings delineate the phenotype and indicate a possible genotype-phenotype correlation for neurological involvement. PMID:26358756

  11. Heterozygous M1V variant of ELA-2 gene mutation associated with G-CSF refractory severe congenital neutropenia.

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    Setty, Bhuvana A; Yeager, Nicholas D; Bajwa, Rajinder P

    2011-09-01

    Severe congenital neutropenia is an autosomal recessive disorder characterized by maturation arrest at the promyelocyte/myelocyte phase in the bone marrow, absolute neutrophil count ELA-2 have been described. We report the case of a premature male infant with congenital neutropenia, associated with multiple infections, refractory to treatment with granulocyte colony stimulating factor who subsequently underwent matched sibling donor stem-cell transplant. He was found to be heterozygous for the M1V variant of the ELA-2 gene that we postulate to be causative for his severe neutropenia

  12. Genetics Home Reference: cyclic neutropenia

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    ... Understand Genetics Home Health Conditions cyclic neutropenia cyclic neutropenia Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Cyclic neutropenia is a disorder that causes frequent infections and ...

  13. Dengue fever causing febrile neutropenia in children with acute lymphoblastic leukemia: an unknown entity.

    Science.gov (United States)

    Ramzan, Mohammed; Yadav, Satya Prakash; Dinand, Veronique; Sachdeva, Anupam

    2013-06-01

    Dengue fever is endemic in many parts of the world but it has not been described as a cause of febrile neutropenia. We describe here clinical features, laboratory values and outcome in 10 children with acute lymphoblastic leukemia (ALL) and with dengue fever as a cause of febrile neutropenia. These data are compared to an age-matched control population of 22 children with proven dengue infection without ALL. Except for fever in all patients and plethoric face in one patient, typical symptoms of dengue such as abdominal pain, myalgias, and headaches, were absent. Mean duration of hospital stay was 6.3±2.0 days in ALL patients vs. 5.0±2.0 in controls (p=0.096). Median platelet count was 13,000/cmm (range 1000-28,000) in cases vs. 31,500 (range 13,000-150,000) in controls (p=0.018). Mean time for recovery for platelet was 6.0±1.3days in ALL patients vs. 2.5±0.9days in controls (pfebrile neutropenia although typical symptoms may be lacking. Platelet recovery may be significantly delayed.

  14. Which Variables Are Useful for Predicting Severe Infection in Children With Febrile Neutropenia?

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    Delebarre, Mathilde; Garnier, Nathalie; Macher, Emilie; Thebaud, Estelle; Mazingue, Françoise; Leblond, Pierre; Duhamel, Alain; Martinot, Alain; Dubos, François

    2015-11-01

    To distinguish children with chemotherapy-induced febrile neutropenia (FN) at low risk of severe infection, the variables that are significant risk factors must be identified. Our objective was to identify them by applying evidence-based standards. This retrospective 2-center cohort study included all episodes of chemotherapy-induced FN in children in 2005 and 2006. The medical history, clinical, and laboratory data available at admission were collected. Severe infection was defined by bacteremia, a positive culture of a normally sterile body fluid, invasive fungal infection, or localized infection at high risk of extension. Univariate analysis identified potential predictive variables. A generalized mixed model was used to determine the adjusted variables that predict severe infection. We analyzed 372 FN episodes. Severe infections occurred in 16.1% of them. Variables predictive of severe infection at admission were: disease with high risk of prolonged neutropenia (adjusted odds ratio [aOR]=2.5), blood cancer (aOR=1.9), fever ≥38.5°C (aOR=3.7), and C-reactive protein level ≥90 mg/L (aOR=4.5). Now that we have identified these variables significantly associated with the risk of severe infection, they must be validated prospectively before combining the best predictive variables in a decision rule that can be used to distinguish children at low risk.

  15. Bacteria causing bacteremia in pediatric cancer patients presenting with febrile neutropenia-species distribution and susceptibility patterns

    NARCIS (Netherlands)

    Miedema, Karin G. E.; Winter, Rik H. L. J.; Ammann, Roland A.; Droz, Sara; Spanjaard, Lodewijk; de Bont, Eveline S. J. M.; Kamps, Willem A.; van de Wetering, Marianne D.; Tissing, Wim J. E.

    2013-01-01

    Infections are a major cause of morbidity and mortality in pediatric cancer patients. The aim of this study was to establish the microbiological spectrum and the susceptibility patterns of bacteremia-causing bacteria in pediatric cancer patients with febrile neutropenia in relation to the use of pro

  16. Emergence of MRSA in positive blood cultures from patients with febrile neutropenia--a cause for concern.

    LENUS (Irish Health Repository)

    Morris, Patrick G

    2008-09-01

    Febrile neutropenia (FN) causes considerable morbidity in patients on cytotoxic chemotherapy. Recently, there has been a trend towards fewer Gram-negative and more Gram-positive infections with increasing antibiotic resistance. To assess these patterns, data from a supra-regional cancer centre in Ireland were reviewed.

  17. Relato de um caso de neutropenia congênita grave em um lactente jovem A case report of severe congenital neutropenia in a young infant

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    Lucas Fadel M. dos Santos

    2011-12-01

    Full Text Available OBJETIVO: Relatar um caso de neutropenia congênita grave e alertar os pediatras sobre tal diagnóstico em pacientes jovens, com infecções recorrentes. DESCRIÇÃO DO CASO: Lactente jovem com 45 dias de vida, com história de febre alta, letargia, recusa alimentar e hemogramas repetidos com leucopenia importante à custa de polimorfonucleares. A hipótese diagnóstica foi confirmada pelo aspirado de medula óssea, que mostrou hipoplasia de série granulocítica e completa ausência de neutrófilos maduros. Foi introduzida antibioticoterapia de largo espectro e estimulador da formação de colônias de granulócitos. O paciente evoluiu para óbito em decorrência de complicações infecciosas após 21 dias de internação. COMENTÁRIOS: Trata-se de um lactente jovem, portador de uma rara desordem congênita que leva à intensa neutropenia, deixando-o vulnerável a infecções graves e potencialmente fatais. À internação, o paciente apresentava sinais e sintomas sugestivos de sepse, sendo introduzido antibioticoterapia de amplo espectro, necessária por se tratar de lactente jovem, neutropênico e febril. A hipótese diagnóstica se baseou na história clínica e nos leucogramas alterados, sendo posteriormente confirmada pelo aspirado de medula óssea. Foi introduzido o estimulador da formação de colônias de granulócitos, que geralmente é efetivo, porém, nesse caso, não houve sucesso e o paciente evoluiu para óbito devido à grave infecção.OBJECTIVE: To report a case of severe congenital neutropenia and alert pediatricians about its diagnosis in young patients with recurrent infectious diseases. CASE DESCRIPTION: Young infant with 45 days of life, with a history of high fever, lethargy, poor feeding and repeated blood counts showing significant leucopenia due to a significant decrease of polymorphonuclear cells. The diagnosis was confirmed by bone marrow aspirate showing hypoplasia of the granulocytic series and complete absence of

  18. [Analysis of the risk factors for severe neutropenia in advanced non-small cell lung cancer after the first course of chemotherapy with third-generation agents].

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    Shibuya, Midori; Kogo, Mari; Kurihara, Tatsuya; Shikama, Yusuke; Nakajima, Hiroaki; Yoneyama, Keiichiro; Kiuchi, Yuji

    2013-01-01

      We retrospectively evaluated clinical data before therapy to determine the risk factors for severe neutropenia in advanced non-small-cell lung cancer (NSCLC) patients treated with third-generation agents. We analyzed 100 patients who received such agents (paclitaxel, docetaxel, gemcitabine, irinotecan, or vinorelbine) for advanced NSCLC. The endpoint of the survey was the occurrence of severe neutropenia (grade 4). Risk factors significantly related to severe neutropenia were identified using logistic regression analysis. Of the 100 patients studied, the median age was 62.0 (32-81 years), and 77 (77.0%) were male. CEA 6.6 (0-2220) ng/dL and cytokeratin 19 fragment 21-1 (CYFRA) 4.8 (0.2-173.8) ng/dL before chemotherapy were higher than normal range. Severe neutropenia occurred in 36.0%, the incidence being highest in the first cycle (61.1%). In the univariate analysis, variables associated with severe neutropenia were sex, chest pain, absolute neutrophil count (ANC), Cr, CRP, and CYFRA. In the multivariate analysis, low CYFRA level was identified as a significant risk factor that contributed independently to chemotherapy-induced severe neutropenia (pCYFRA level is the most important risk factor for severe neutropenia in advanced NSCLC patients after the first course of chemotherapy with third-generation agents. PMID:23728094

  19. [Serum lactate as a biomarker of severe sepsis in children with cancer, neutropenia and fever].

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    Pacheco-Rosas, Daniel Octavio; Huelgas-Plaza, Ana Celia; Miranda-Novales, María Guadalupe

    2014-01-01

    INTRODUCCIÓN: la neutropenia es una complicación frecuente secundaria a la quimioterapia en los pacientes con cáncer, en quienes la prevalencia de sepsis es de 12.9 a 17.4 % y la letalidad es de 16 %. El objetivo de este estudio fue determinar la utilidad del lactato como biomarcador de sepsis grave en niños con cáncer, fiebre y neutropenia. MÉTODOS: se realizó un estudio de prueba diagnóstica fase II. Se midieron los niveles del lactato al ingreso. Los episodios de neutropenia se clasificaron en tres grupos: I, con sepsis II, sin sepsis; III, pacientes neutropénicos sin fiebre (controles). Se calculó sensibilidad, especificidad, valores predictivos positivo y negativo e índices de verosimilitud. El estándar de oro fue el diagnóstico clínico de sepsis grave.

  20. Adult siblings with homozygous G6PC3 mutations expand our understanding of the severe congenital neutropenia type 4 (SCN4 phenotype

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    Fernandez Bridget A

    2012-11-01

    Full Text Available Abstract Background Severe congenital neutropenia type 4 (SCN4 is an autosomal recessive disorder caused by mutations in the third subunit of the enzyme glucose-6-phosphatase (G6PC3. Its core features are congenital neutropenia and a prominent venous skin pattern, and affected individuals have variable birth defects. Oculocutaneous albinism type 4 (OCA4 is caused by autosomal recessive mutations in SLC45A2. Methods We report a sister and brother from Newfoundland, Canada with complex phenotypes. The sister was previously reported by Cullinane et al., 2011. We performed homozygosity mapping, next generation sequencing and conventional Sanger sequencing to identify mutations that cause the phenotype in this family. We have also summarized clinical data from 49 previously reported SCN4 cases with overlapping phenotypes and interpret the medical histories of these siblings in the context of the literature. Results The siblings’ phenotype is due in part to a homozygous mutation in G6PC3, [c.829C > T, p.Gln277X]. Their ages are 38 and 37 years respectively and they are the oldest SCN4 patients published to date. Both presented with congenital neutropenia and later developed Crohn disease. We suggest that the latter is a previously unrecognized SCN4 manifestation and that not all affected individuals have an intellectual disability. The sister also has a homozygous mutation in SLC45A2, which explains her severe oculocutaneous hypopigmentation. Her brother carried one SLC45A2 mutation and was diagnosed with “partial OCA” in childhood. Conclusions This family highlights that apparently novel syndromes can in fact be caused by two known autosomal recessive disorders.

  1. Neutropenia - infants

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    ... this page: //medlineplus.gov/ency/article/007230.htm Neutropenia - infants To use the sharing features on this page, please enable JavaScript. Neutropenia is an abnormally low number of white blood ...

  2. Serum Endocan Levels in Children with Febrile Neutropenia.

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    Kiral, Eylem; Dinleyici, Ener Cagri; Bozkurt-Turhan, Ayse; Bor, Ozcan; Akgun, Yurdanur; Akgun, Necat Akdeniz

    2016-03-17

    Endocan is an endotelial cell specific molecule; previous studies have shown that serum endocan levels increased in cancer and sepsis and are also related to the severity of sepsis. There are no clinical study about serum endocan levels in children with febrile neutropenia. The aim of this study was to evaluate serum endocan levels in pediatric leukemia patients with febrile neutropenia (n=33) and compare them with children with leukemia without fever (n=33) and also with healthy children (n=24). The median serum endocan level in the first group (children with febrile neutropenia) was statistically significantly higher compared to the leukemic children without febrile neutropenia and also control group (Pfebrile neutropenia and the healthy control group (P>0.05). Serum endocan levels were also similar with febrile neutropenia due to bacterial causes comparing with the idiopathic febril neutropenia. The results of this study showed increased serum endocan in children with leukemia during the febrile neutropenia episode, and no changes of serum endocan levels in children without leukemia without infection/fever. The monitoring of a series of serum endocan levels would be helpful for the course of febrile neutropenia.

  3. Serum endocan levels in children with febrile neutropenia

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    Eylem Kiral

    2016-03-01

    Full Text Available Endocan is an endotelial cell specific molecule; previous studies have shown that serum endocan levels increased in cancer and sepsis and are also related to the severity of sepsis. There are no clinical study about serum endocan levels in children with febrile neutropenia. The aim of this study was to evaluate serum endocan levels in pediatric leukemia patients with febrile neutropenia (n=33 and compare them with children with leukemia without fever (n=33 and also with healthy children (n=24. The median serum endocan level in the first group (children with febrile neutropenia was statistically significantly higher compared to the leukemic children without febrile neutropenia and also control group (P<0.01 for both. No difference was determined between the serum endocan levels of the leukaemia patients without febrile neutropenia and the healthy control group (P>0.05. Serum endocan levels were also similar with febrile neutropenia due to bacterial causes comparing with the idiopathic febril neutropenia. The results of this study showed increased serum endocan in children with leukemia during the febrile neutropenia episode, and no changes of serum endocan levels in children without leukemia without infection/fever. The monitoring of a series of serum endocan levels would be helpful for the course of febrile neutropenia.

  4. Neutropenia Due to Very Long Time Propylthiouracil Usage in Toxic Multinodular Goiter

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    Ahmet Kaya

    2016-03-01

    Full Text Available Thyrotoxicosis affects hematopoiesis in several ways and thioamides may cause myelosuppression. We report a case of febrile neutropenia in a patient with hyperthyroidism who was using propylthiouracil for nearly 20 years for the treatment of toxic multinodular goitre. After surgery, the patient was euthyroid and neutropenia resolved. Postoperative pathology was evaluated as micropapillary thyroid carcinoma.

  5. Clozapine-Induced Late Agranulocytosis and Severe Neutropenia Complicated with Streptococcus pneumonia, Venous Thromboembolism, and Allergic Vasculitis in Treatment-Resistant Female Psychosis

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    Christina Voulgari

    2015-01-01

    Full Text Available Clozapine is a second-generation antipsychotic agent from the benzodiazepine group indicated for treatment-resistant schizophrenia and other psychotic conditions. Using clozapine earlier on once a case appears to be refractory limits both social and personal morbidity of chronic psychosis. However treatment with second-generation antipsychotics is often complicated by adverse effects. We present a case of a 33-year-old Caucasian woman with a 25-year history of refractory psychotic mania after switching to a 2-year clozapine therapy. She presented clozapine-induced absolute neutropenia, agranulocytosis, which were complicated by Streptococcus pneumonia and sepsis. Clozapine-induced thromboembolism of the common femoral and right proximal iliac vein, as well as allergic vasculitis, was diagnosed. She achieved full remission on granulocyte-colony stimulating factor and specific antibiotic treatment. Early detection of severe clozapine-induced absolute neutropenia and agranulocytosis enabled the effective treatment of two among its most severe complications. Additional evidence to the previously reported possible causal relation between clozapine and venous thromboembolism is offered. Finally, clozapine-induced allergic vasculitis is confirmed as a late adverse effect of clozapine therapy.

  6. ''Hair-on-end'' skull induced by long-term G-CSF treatment in severe congenital neutropenia

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    Albert, Michael H. [Dr. von Haunersches Kinderspital, Department of Haematology/Oncology, Munich (Germany); Notheis, Gundula; Wintergerst, Uwe [Dr. von Haunersches Kinderspital, Department of Infection/Immunity, Munich (Germany); Born, Christine [Klinikum Innenstadt, General Radiology, Munich (Germany); Schneider, Karl [Dr. von Haunersches Kinderspital, Department of Pediatric Radiology, Munich (Germany)

    2007-02-15

    ''Hair-on-end'' skull changes are typically seen in individuals suffering from thalassaemia. They are induced by widening of the diploic space due to marrow expansion that is a consequence of ineffective and excessive erythropoiesis. We present a child with severe congenital neutropenia who exhibited the typical hair-on-end sign on plain skull radiographs and MRI. In this patient the skull changes were very likely induced by the expansion of white blood cell precursors induced by long-term daily injections of recombinant human granulocyte colony stimulating factor (G-CSF) to treat his confounding disease. This case report is the first description of hair-on-end changes associated with the use of G-CSF. (orig.)

  7. IMPORTANCE OF SERUM PROCALCITONIN IN FEBRILE NEUTROPENIA

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    Mohd. Riyaz

    2014-07-01

    Full Text Available Febrile neutropenia is defined as a fever >101°F for 1 hour, with an absolute neutrophil count of ≤500 cells/microliter, or an ANC of ≤1000 cells/microliter with a projected nadir of ≤500 cells/microliter. In haematological malignancies it is the common complication and requires broad-spectrum antibacterial therapy. Clinical examination and cultures fail to detect a pathogen or an infectious focus in 25–50%, which are classified as pyrexia of unknown origin (PUO. Patient with pyrexia of unknown origin may receive long duration of antibiotic treatment as the cause is unclear of being infective or not. Febrile neutropenia is a common complication of many chemotherapeutic regimens for all types of cancers. Mortality and Morbidity is high particularly in elderly, immuno-compromised. Approximately 20- 40 % of patients with severe sepsis and 45-60% patients with septic shock die within 15-20 days. This study was done to know the sources of infection and to assess the diagnostic value of serum Procalcitonin and its relation with mortality in various stages of sepsis. Sepsis incidence was more in patient age more than 55yrs. the most common source of sepsis was respiratory tract infection. Serum PCT proved to be an indicator of sepsis in ill patients, with sensitivity of 91%. Presence of both persistent and profound neutropenia was associated with a much higher mortality. The occurrence of infection is directly proportional to the degree of neutropenia, at the onset of fever the PCT levels will not be helpful for the decision to start or stop the antibacterial therapy, and a PCT value higher than 0.5ng/ml in pyrexia of unknown origin might suggest a possibility of occult infection, i.e. with lacking microbiological and clinical documentation. A delayed PCT peak higher than0.5ng/ml contributes to the early diagnosis of fungal disease.

  8. Results of high-risk neutropenia therapy of hematology–oncology patients in a university hospital in Uruguay

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    Boada Burutaran, Matilde; Guadagna, Regina; Grille, Sofia; Stevenazzi, Mariana; Guillermo, Cecilia; Diaz, Lilian

    2014-01-01

    Background Febrile neutropenia is an important cause of mortality and morbidity in hematology–oncology patients undergoing chemotherapy. The management of febrile neutropenia is typically algorithm-driven. The aim of this study was to assess the results of a standardized protocol for the treatment of febrile neutropenia. Methods A retrospective cohort study (2011–2012) was conducted of patients with high-risk neutropenia in a hematology–oncology service. Results Forty-four episodes of 17 patients with a median age of 48 years (range: 18–78 years) were included. The incidence of febrile neutropenia was 61.4%. The presence of febrile neutropenia was associated with both the duration and severity of neutropenia. Microbiological agents were isolated from different sources in 59.3% of the episodes with bacteremia isolated from blood being the most prevalent (81.3%). Multiple drug-resistant gram-negative bacilli were isolated in 62.5% of all microbiologically documented infections. Treatment of 63% of the episodes in which the initial treatment was piperacillin/tazobactam needed to be escalated to meropenem. The mortality rate due to febrile neutropenia episodes was 18.5%. Conclusion The high rate of gram-negative bacilli resistant to piperacillin/tazobactam (front-line antibiotics in our protocol) and the early need to escalate to carbapenems raises the question as to whether it is necessary to change the current protocol. PMID:25638764

  9. Results of high-risk neutropenia therapy of hematology-oncology patients in a university hospital in Uruguay

    Directory of Open Access Journals (Sweden)

    Matilde Boada Burutaran

    2015-02-01

    Full Text Available Background: Febrile neutropenia is an important cause of mortality and morbidity in hematology-oncology patients undergoing chemotherapy. The management of febrile neutropenia is typically algorithm-driven. The aim of this study was to assess the results of a standardized protocol for the treatment of febrile neutropenia. Methods: A retrospective cohort study (2011-2012 was conducted of patients with high-risk neutropenia in a hematology-oncology service. Results: Forty-four episodes of 17 patients with a median age of 48 years (range: 18-78 years were included. The incidence of febrile neutropenia was 61.4%. The presence of febrile neutropenia was associated with both the duration and severity of neutropenia. Microbiological agents were isolated from different sources in 59.3% of the episodes with bacteremia iso- lated from blood being the most prevalent (81.3%. Multiple drug-resistant gram-negative bacilli were isolated in 62.5% of all microbiologically documented infections. Treatment of 63% of the episodes in which the initial treatment was piperacillin/tazobactam needed to be escalated to meropenem. The mortality rate due to febrile neutropenia episodes was 18.5%. Conclusion: The high rate of gram-negative bacilli resistant to piperacillin/tazobactam (frontline antibiotics in our protocol and the early need to escalate to carbapenems raises the question as to whether it is necessary to change the current protocol.

  10. Simultaneous occurrence of foetal and neonatal alloimmune thrombocytopenia and neonatal neutropenia due to maternal neutrophilic autoantibodies

    DEFF Research Database (Denmark)

    Morling Taaning, Ellen Birkerod; Jensen, Lise; Varming, Kim

    2012-01-01

    Foetal and neonatal alloimmune thrombocytopenia (FNAIT) and neonatal neutropenia caused by maternal autoantibodies against neutrophils are rare disorders. We describe a newborn with severe thrombocytopenia and intracerebral bleeding caused by maternal anti-HPA-3a alloantibodies and mild neutropenia...... caused by maternal autoantibodies against HNA-1b. This appears to be the first case of simultaneous occurrence of these two conditions. Conclusion:  This case report and review of the literature demonstrate that anti-HPA-3a antibodies can be overlooked by standard assays....

  11. Deletion of GPIHBP1 causing severe chylomicronemia

    OpenAIRE

    Rios, Jonathan J.; Shastry, Savitha; Jasso, Juan; Hauser, Natalie; Garg, Abhimanyu; Bensadoun, André; Cohen, Jonathan C.; Hobbs, Helen H.

    2011-01-01

    Lipoprotein lipase (LPL) is a hydrolase that cleaves circulating triglycerides to release fatty acids to the surrounding tissues. The enzyme is synthesized in parenchymal cells and is transported to its site of action on the capillary endothelium by glycophosphatidylinositol (GPI)-anchored high-density lipoprotein-binding protein 1 (GPIHBP1). Inactivating mutations in LPL; in its cofactor, apolipoprotein (Apo) C2; or in GPIHBP1 cause severe hypertriglyceridemia. Here we describe an individual...

  12. An Unexpected Cause of Severe Hypokalemia

    Directory of Open Access Journals (Sweden)

    Fernando Caravaca-Fontan

    2015-01-01

    Full Text Available We describe an unusual case of severe hypokalemia with electrocardiographic changes, due to licorice consumption, in a 15-year-old female student with no previous medical history. Prompt replacement of potassium and cessation of licorice ingestion resulted in a favourable outcome. We also discuss the pathophysiology and diagnosis, emphasizing the importance of a detailed anamnesis to rule out an often forgotten cause of hypokalemia as the licorice poisoning.

  13. Risk Factors Study for Lung Cancer Patients with Chemotherapy-induced Severe Neutropenia%化疗致肺癌患者严重粒细胞减少相关危险因素研究

    Institute of Scientific and Technical Information of China (English)

    樊迪; 尤海生; 胡萨萨; 王茂义; 封卫毅; 董亚琳

    2016-01-01

    Objective:To discuss the risk factors in lung cancer patients with chemotherapy-induced severe neutropenia to provide reference for clinical drug use. Methods:A retrospective analysis was performed for the patients with lung cancer,and the risk factors of severe neutropenia were statistically analyzed and found out. Results:The results of single factor experiments showed that the incidence of severe neutropenia was related with radiotherapy history,cycles of chemotherapy and the use time of granulocyte colony factor. Based on a binary logistic regression analysis,the history of radiotherapy and the use of granulocyte colony factor were the significant risk factors of severe neutropenia in the lung cancer patients. Conclusion:For the patients with radiotherapy history,it is better to choose chemotherapy drugs with lower toxicity,decrease drug dosage or preventively use granulocyte colony factor. The rational use of rhG-CSF can alleviate chemotherapy-induced severe neutropenia.%目的:探讨化疗致肺癌患者严重粒细胞减少的相关危险因素,为临床用药提供参考。方法:回顾性分析肺癌患者临床资料,统计分析找出与严重粒细胞减少有关的危险因素。结果:单因素分析显示严重粒细胞减少与放疗史、化疗周期和粒细胞集落因子使用时机具有相关性。Logistic 回归分析显示,放疗史、粒细胞集落因子使用时机是肺癌患者严重粒细胞减少的危险因素。结论:对于有放疗史的患者,可选用细胞毒性小的化疗方案,或降低药物剂量,或预防使用集落刺激因子;合理应用粒细胞集落刺激因子可减轻肺癌化疗引起的严重粒细胞减少。

  14. Deletion of GPIHBP1 causing severe chylomicronemia.

    Science.gov (United States)

    Rios, Jonathan J; Shastry, Savitha; Jasso, Juan; Hauser, Natalie; Garg, Abhimanyu; Bensadoun, André; Cohen, Jonathan C; Hobbs, Helen H

    2012-05-01

    Lipoprotein lipase (LPL) is a hydrolase that cleaves circulating triglycerides to release fatty acids to the surrounding tissues. The enzyme is synthesized in parenchymal cells and is transported to its site of action on the capillary endothelium by glycophosphatidylinositol (GPI)-anchored high-density lipoprotein-binding protein 1 (GPIHBP1). Inactivating mutations in LPL; in its cofactor, apolipoprotein (Apo) C2; or in GPIHBP1 cause severe hypertriglyceridemia. Here we describe an individual with complete deficiency of GPIHBP1. The proband was an Asian Indian boy who had severe chylomicronemia at 2 months of age. Array-based copy-number analysis of his genomic DNA revealed homozygosity for a 17.5-kb deletion that included GPIHBP1. A 44-year-old aunt with a history of hypertriglyceridemia and pancreatitis was also homozygous for the deletion. A bolus of intravenously administered heparin caused a rapid increase in circulating LPL and decreased plasma triglyceride levels in control individuals but not in two GPIHBP1-deficient patients. Thus, short-term treatment with heparin failed to attenuate the hypertriglyceridemia in patients with GPIHBP1 deficiency. The increasing resolution of copy number microarrays and their widespread adoption for routine cytogenetic analysis is likely to reveal a greater role for submicroscopic deletions in Mendelian conditions. We describe the first neonate with complete GPIHBP1 deficiency due to homozygosity for a deletion of GPIHBP1. PMID:22008945

  15. Congenital neutropenia: diagnosis, molecular bases and patient management

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    Chantelot Christine

    2011-05-01

    Full Text Available Abstract The term congenital neutropenia encompasses a family of neutropenic disorders, both permanent and intermittent, severe ( When neutropenia is detected, an attempt should be made to establish the etiology, distinguishing between acquired forms (the most frequent, including post viral neutropenia and auto immune neutropenia and congenital forms that may either be isolated or part of a complex genetic disease. Except for ethnic neutropenia, which is a frequent but mild congenital form, probably with polygenic inheritance, all other forms of congenital neutropenia are extremely rare and have monogenic inheritance, which may be X-linked or autosomal, recessive or dominant. About half the forms of congenital neutropenia with no extra-hematopoetic manifestations and normal adaptive immunity are due to neutrophil elastase (ELANE mutations. Some patients have severe permanent neutropenia and frequent infections early in life, while others have mild intermittent neutropenia. Congenital neutropenia may also be associated with a wide range of organ dysfunctions, as for example in Shwachman-Diamond syndrome (associated with pancreatic insufficiency and glycogen storage disease type Ib (associated with a glycogen storage syndrome. So far, the molecular bases of 12 neutropenic disorders have been identified. Treatment of severe chronic neutropenia should focus on prevention of infections. It includes antimicrobial prophylaxis, generally with trimethoprim-sulfamethoxazole, and also granulocyte-colony-stimulating factor (G-CSF. G-CSF has considerably improved these patients' outlook. It is usually well tolerated, but potential adverse effects include thrombocytopenia, glomerulonephritis, vasculitis and osteoporosis. Long-term treatment with G-CSF, especially at high doses, augments the spontaneous risk of leukemia in patients with congenital neutropenia.

  16. The antimicrobial propeptide hCAP-18 plasma levels in neutropenia of various aetiologies

    DEFF Research Database (Denmark)

    Ye, Ying; Carlsson, Göran; Karlsson-Sjöberg, Jenny M T;

    2015-01-01

    The underlying cause of neutropenia may be difficult to determine due to similar clinical presentation in many neutropenic conditions. The neutrophil protein hCAP-18 (pro-LL-37) is a major component of neutrophil secondary granules and in this prospective study we assessed the use of hCAP-18 levels...... in blood plasma for differential diagnosis of neutropenic patients (n = 133) of various aetiologies. Plasma levels of hCAP-18 were determined using immunoblot and ELISA. Patients with severe congenital neutropenia (n = 23) presented with the lowest levels of plasma hCAP-18 and differential diagnostic...... diagnostic value in differential diagnosis of chronic neutropenia. Neutropenic patients with Shwachman-Diamond syndrome, Barth syndrome, Cohen syndrome, acute myeloid leukaemia and specific granule deficiency presented with reduced plasma hCAP-18 levels as well. The blood plasma level of hCAP-18 was thus low...

  17. {sup 18}F-FDG PET/CT for diagnosing infectious complications in patients with severe neutropenia after intensive chemotherapy for haematological malignancy or stem cell transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Vos, Fidel J.; Kullberg, Bart-Jan; Bleeker-Rovers, Chantal P. [Radboud University Nijmegen Medical Centre, Department of Internal Medicine, PO Box 9101, Nijmegen (Netherlands); Nijmegen Institute for Infection, Inflammation and Immunity (N4i), Radboud University Nijmegen Medical Centre, Nijmegen (Netherlands); Donnelly, J.P.; Blijlevens, Nicole M.A. [Radboud University Nijmegen Medical Centre, Department of Hematology, Nijmegen (Netherlands); Nijmegen Institute for Infection, Inflammation and Immunity (N4i), Radboud University Nijmegen Medical Centre, Nijmegen (Netherlands); Oyen, Wim J.G. [Radboud University Nijmegen Medical Centre, Department of Nuclear Medicine, Nijmegen (Netherlands); Nijmegen Institute for Infection, Inflammation and Immunity (N4i), Radboud University Nijmegen Medical Centre, Nijmegen (Netherlands)

    2012-01-15

    Between 30 and 50% of febrile neutropenic episodes are accounted for by infection. C-reactive protein (CRP) is a nonspecific parameter for infection and inflammation but might be employed as a trigger for diagnosis. The aim of the study was to evaluate whether {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT can be used to detect inflammatory foci in neutropenic patients with elevated CRP and whether it helps to direct treatment. Twenty-eight consecutive patients with neutropenia as a result of intensive chemotherapy for haematological malignancies or myeloablative therapy for haematopoietic stem cell transplantation were prospectively included. {sup 18}F-FDG PET/CT was added to the regular diagnostic workup once the CRP level rose above 50 mg/l. Pathological FDG uptake was found in 26 of 28 cases despite peripheral neutrophil counts less than 0.1 x 10{sup -9}/l in 26 patients: in the digestive tract in 18 cases, around the tract of the central venous catheter (CVC) in 9 and in the lungs in 7 cases. FDG uptake in the CVC tract was associated with coagulase-negative staphylococcal bacteraemia (p < 0.001) and deep venous thrombosis (p = 0.002). The number of patients having Streptococcus mitis bacteraemia appeared to be higher in patients with grade 3 oesophageal FDG uptake (p = 0.08). Pulmonary FDG uptake was associated with the presence of invasive fungal disease (p = 0.04). {sup 18}F-FDG PET/CT scanning during chemotherapy-induced febrile neutropenia and increased CRP is able to detect localized foci of infection and inflammation despite the absence of circulating neutrophils. Besides its potential role in detecting CVC-related infection during febrile neutropenia, the high negative predictive value of {sup 18}F-FDG PET/CT is important for avoiding unnecessary diagnostic tests and therapy. (orig.)

  18. Clozapine Rechallenge After Neutropenia or Leucopenia.

    Science.gov (United States)

    Prokopez, Cintia R; Armesto, Arnaldo R; Gil Aguer, María F; Balda, María V; Papale, Rosa M; Bignone, Inés M; Daray, Federico M

    2016-08-01

    To rechallenge with clozapine for a patient who previously has experienced neutropenia or leucopenia or during clozapine treatment is a difficult clinical decision. Herein, we analyzed the results of such a rechallenge in 19 patients. We analyzed all the reports, from the database of the pharmacovigilance department of the Argentine National Administration of Drugs, Foods, and Medical Devices, of patients who were rechallenged with clozapine after a leucopenia or a neutropenia. Nineteen cases of rechallenge after leucopenia or neutropenia were reported between 1996 and 2014. One third of the patients re-exposed to clozapine developed a new hematologic adverse reaction. The second blood dyscrasia was less severe in 83% of the cases and had a shorter median latency as compared with the first (8 weeks vs 182 weeks, P = 0.0045). There were no significant differences for demographic and clinical characteristics of patients who developed a second dyscrasia as compared with those who did not. The present study shows that almost 70% of the patients rechallenged with clozapine after a leucopenia or a neutropenia did not develop a new hematological adverse effect, whereas the remaining 30% had a faster but less serious neutropenia. PMID:27232877

  19. Triagem para o tratamento ambulatorial da neutropenia febril Screening for the outpatient treatment of febrile neutropenia

    Directory of Open Access Journals (Sweden)

    Marcelo Bellesso

    2010-01-01

    Full Text Available A neutropenia febril (NF é uma complicação frequente e potencialmente fatal nos pacientes em tratamento quimioterápico. Entendemos hoje que a neutropenia febril é considerada uma emergência clínica e que a administração de antibióticos de amplo espectro diminui drasticamente a mortalidade. Estudos sugerem que a neutropenia febril compreende um grupo extremamente heterogêneo e que dados clínicos como febre domiciliar, ausência de hipotensão, ausência de desidratação, ausência de doença pulmonar obstrutiva crônica, ausência de outros sintomas, ausência de infecção fúngica prévia e idade Febrile neutropenia is a frequent and potentially fatal adverse event of chemotherapy. Nowadays, febrile neutropenia is considered an emergency and it is known that prompt infusion of antibiotics decreases mortality. Several studies demonstrated that febrile neutropenia is a heterogeneous group of diseases and that factors such as outpatient status, no hypotension, no dehydration, no chronic obstructive pulmonary disease, no symptoms, no previous fungal infection and age < 60 years are protective factors against serious complications as demonstrated by the Multinational Association for Supportive Care in Cancer (MASCC. These data show that outpatient treatment and early discharge is safer and much research has shown lower costs for outpatient treatment in low-risk patients with febrile neutropenia. The aim of this work is to review and discuss tools (in particular the MASCC index for safe screening of febrile neutropenia for outpatient treatment in addition to demonstrate results of research.

  20. Severe Vitamin D Deficiency Causing Kyphoscoliosis.

    Science.gov (United States)

    Singhai, Abhishek; Banzal, Subodh

    2013-01-01

    Vitamin D deficiency is common among Indian population. Women are especially at risk for severe vitamin D deficiency. The risk is higher for those who are multiparous and postmenopausal. Poor exposure to sunlight, higher latitude, winter season, inadequate diet, older age, obesity and malabsorption are also important risk factors. Symptoms of hypovitaminosis D, including diffuse or migratory pain affecting several sites (especially the shoulder, pelvis, ribcage and lower back) have also been misdiagnosed as musculoskeletal disorders, including fibromyalgia, polymyalgia rheumatica and ankylosing spondylitis. Here, we report two cases presented with kyphoscoliosis, diagnosed to have severe vitamin D deficiency. PMID:26664847

  1. Severe Vitamin D Deficiency Causing Kyphoscoliosis

    OpenAIRE

    Singhai, Abhishek; Banzal, Subodh

    2013-01-01

    Vitamin D deficiency is common among Indian population. Women are especially at risk for severe vitamin D deficiency. The risk is higher for those who are multiparous and postmenopausal. Poor exposure to sunlight, higher latitude, winter season, inadequate diet, older age, obesity and malabsorption are also important risk factors. Symptoms of hypovitaminosis D, including diffuse or migratory pain affecting several sites (especially the shoulder, pelvis, ribcage and lower back) have also been ...

  2. Urinothorax: an unexpected cause of severe dyspnea.

    Science.gov (United States)

    Tortora, Alessandra; Casciani, Emanuele; Kharrub, Zaher; Gualdi, Gianfranco

    2006-05-01

    We report an unusual cause of the pleural effusion due to extravasation of urine from the retroperitoneal space into the thoracic cavity. In our case, the urinoma occurred owing to obstructing urinary tract lesion due to opaque stone. Although rare, urinothorax should be considered when pleural effusion occurs in patients with urinary tract obstruction accompanied by retroperitoneal urinoma.

  3. Severe hyponatremia caused by nab-paclitaxel-induced syndrome of inappropriate antidiuretic hormone secretion

    Science.gov (United States)

    Neuzillet, Cindy; Babai, Samy; Kempf, Emmanuelle; Pujol, Géraldine; Rousseau, Benoît; Le-Louët, Hervé; Christophe Tournigand

    2016-01-01

    Abstract Incidence of pancreatic ductal adenocarcinoma (PDAC) is increasing. Most patients have advanced disease at diagnosis and therapeutic options in this setting are limited. Gemcitabine plus nab-paclitaxel regimen was demonstrated to increase survival compared with gemcitabine monotherapy and is therefore indicated as first-line therapy in patients with metastatic PDAC and performance status Eastern Cooperative Oncology Group (ECOG) 0-2. The safety profile of gemcitabine and nab-paclitaxel combination includes neutropenia, fatigue, and neuropathy as most common adverse events of grade 3 or higher. No case of severe hyponatremia associated with the use of nab-paclitaxel for the treatment of PDAC has been reported to date. We report the case of a 72-year-old Caucasian man with a metastatic PDAC treated with gemcitabine and nab-paclitaxel regimen, who presented with a severe hyponatremia (grade 4) caused by a documented syndrome of inappropriate antidiuretic hormone secretion (SIADH). This SIADH was attributed to nab-paclitaxel after a rigorous imputability analysis, including a rechallenge procedure with dose reduction. After dose and schedule adjustment, nab-paclitaxel was pursued without recurrence of severe hyponatremia and with maintained efficacy. Hyponatremia is a rare but potentially severe complication of nab-paclitaxel therapy that medical oncologists and gastroenterologists should be aware of. Nab-paclitaxel-induced hyponatremia is manageable upon dose and schedule adaptation, and should not contraindicate careful nab-paclitaxel reintroduction. This is of particular interest for a disease in which the therapeutic options are limited. PMID:27368013

  4. Forgotten Kirschner Wire Causing Severe Hematuria

    Directory of Open Access Journals (Sweden)

    Santosh Kumar

    2014-01-01

    Full Text Available Kirschner wire (K-wire is commonly used in the treatment of hip fracture and its migration into pelvis leading to bladder injury is a very rare complication. Nonremoval of these devices either because of lack of followup or because of prolonged requirement due to disease process is associated with this complication. We report a case of a patient who presented with acute onset severe hematuria with clot retention secondary to perforation of bladder by a migrated K-wire placed earlier, for the treatment of hip fracture. Initial imaging showed its presence in the soft tissues of the pelvis away from the major vascular structures. Patient was taken for emergency laparotomy and wire was removed after cystotomy. Postoperative period was uneventful and patient was discharged in satisfactory condition. K-wires are commonly used in the management of fracture bones and their migration has been reported in the literature although such migration in the intrapelvic region involving bladder is very rare. Early diagnosis and prompt removal of such foreign bodies are required to avert potentially fatal involvement of major structures.

  5. Elevated Fra-1 expression causes severe lipodystrophy.

    Science.gov (United States)

    Luther, Julia; Driessler, Frank; Megges, Matthias; Hess, Andreas; Herbort, Bettina; Mandic, Vice; Zaiss, Mario M; Reichardt, Anne; Zech, Christine; Tuckermann, Jan P; Calkhoven, Cornelis F; Wagner, Erwin F; Schett, Georg; David, Jean-Pierre

    2011-05-01

    A shift from osteoblastogenesis to adipogenesis is one of the underlying mechanisms of decreased bone mass and increased fat during aging. We now uncover a new role for the transcription factor Fra-1 in suppressing adipogenesis. Indeed, Fra1 (Fosl1) transgenic (Fra1tg) mice, which developed progressive osteosclerosis as a result of accelerated osteoblast differentiation, also developed a severe general lipodystrophy. The residual fat of these mice appeared immature and expressed lower levels of adipogenic markers, including the fatty acid transporter Cd36 and the CCAAT/enhancer binding protein Cebpa. Consequently accumulation of triglycerides and free fatty acids were detected in the serum of fasting Fra1tg mice. Fra-1 acts cell autonomously because the adipogenic differentiation of Fra1 transgenic primary osteoblasts was drastically reduced, and overexpression of Fra-1 in an adipogenic cell line blocked their differentiation into adipocytes. Strikingly, Cebpa was downregulated in the Fra-1-overexpressing cells and Fra-1 could bind to the Cebpa promoter and directly suppress its activity. Thus, our data add to the known common systemic control of fat and bone mass, a new cell-autonomous level of control of cell fate decision by which the osteogenic transcription factor Fra-1 opposes adipocyte differentiation by inhibiting C/EBPα. PMID:21486951

  6. Effects of Traditional Chinese Medicine on Chemotherapy-Induced Myelosuppression and Febrile Neutropenia in Breast Cancer Patients

    Directory of Open Access Journals (Sweden)

    Huan Tian

    2015-01-01

    Full Text Available Title. Chemotherapy-induced myelosuppression lowers the quality of life in breast cancer patients and causes many complications. Traditional Chinese Medicine (TCM is a widely used complementary and alternative medicine therapies. Objective. To study whether TCM can reduce the incidence of chemotherapy-induced leukopenia, neutropenia, and febrile neutropenia (FN in breast cancer patients. Methods. The data were analyzed retrospectively between patients who received TCM treatment (group 1, n=453 and patients who did not receive TCM treatment (group 2, n=359. Significant risk factors associated with the occurrence of chemotherapy-induced leukopenia, neutropenia, and FN were identified using multivariate analysis. Propensity score-matched patients were analyzed to adjust for any baseline differences. Results. Group 1 patients had a significantly lower rate of chemotherapy-induced severe leukopenia, neutropenia, and FN, compared with group 2 (43% versus 71%, P<0.0001, 72% versus 78%, P=0.005, 6% versus 24%, P<0.0001, resp.. Multivariate analysis revealed that chemotherapy regimens containing anthracyclines combined with paclitaxel or docetaxel were the most significant predictor. Subgroup analysis indicated that TCM treatment showed benefit in relieving chemotherapy-induced leukopenia and FN in most chemotherapy regimens. Conclusions. TCM treatment could lower the risk of severe chemotherapy-induced leukopenia, neutropenia, and FN in breast cancer patients.

  7. A Case of Neonatal Neutropenia Due to Anti-Fc Gamma Receptor IIIb Isoantibodies Treated with Recombinant Human Granulocyte Colony Stimulating Factor

    Directory of Open Access Journals (Sweden)

    Maja Tomicic

    2009-01-01

    Full Text Available Alloimmunization to granulocyte-specific antigens can occur during pregnancy. Maternal antibodies of IgG class can cross the placenta to result in alloimmune neonatal neutropenia. Antibodies to human neutrophil antigens anti-HNA-1a, HNA-1b, and HNA-2a have been most commonly reported to cause alloimmune neonatal neutropenia. Isoantibodies to Fc gamma RIIIb (CD16 if mother is a HNA-null phenotype are rarely involved in neonatal neutropenia. We report on a case of severe neutropenia (440 neutrophils/μL due to anti-Fc gamma RIIIb (CD16 isoimmunization. On day 14 severe omphalitis developed, which was treated for 7 days by an antibiotic (ceftriaxone in a dose of 80 mg/kg/d according to umbilical swab finding. Omphalitis persisted for 10 days in spite of antibiotic therapy and only resolved upon the introduction of rhG-CSF therapy. Therapy with rh-GCSF proved efficient and led to neutrophil count increase to 1970/μL and cure of omphalitis. However, therapeutic effect on granulocyte count was of transient nature, as granulocyte count fell to 760 n/μL on day 4 of therapy discontinuation. Neutropenia persisted for 2 months. The newborn was discharged from the hospital on day 26 with normal clinical status with clinical and laboratory control examinations at 2-week intervals. No additional infections were observed during the course of neutropenia.

  8. REFRACTORY THROMBOCYTOPENIA AND NEUTROPENIA: A DIAGNOSTIC CHALLENGE

    Directory of Open Access Journals (Sweden)

    Emmanuel Gyan

    2015-02-01

    Full Text Available Background. The 2008 WHO classification identified refractory cytopenia with unilineage dysplasia (RCUD as a composite entity encompassing refractory anemia, refractory thrombocytopenia (RT, and refractory neutropenia (RN, characterized by 10% or more dysplastic cells in the bone marrow respective lineage. The diagnosis of RT and RN is complicated by several factors.  Diagnosing RT first requires exclusion of familial thrombocytopenia, chronic auto-immune thrombocytopenia, concomitant medications, viral infections, or hypersplenism. Diagnosis of RN should also be made after ruling out differential diagnoses such as ethnic or familial neutropenia, as well as acquired, drug-induced, infection-related or malignancy-related neutropenia. An accurate quantification of dysplasia should be performed in order to distinguish RT or RN from the provisional entity named idiopathic cytopenia of unknown significance (ICUS. Cytogenetic analysis, and possibly in the future somatic mutation analysis (of genes most frequently mutated in MDS, and flow cytometry analysis aberrant antigen expression on myeloid cells may help in this differential diagnosis. Importantly, we and others found that, while isolated neutropenia and thrombocytopenia are not rare in MDS, those patients can generally be classified (according to WHO 2008 classification as refractory cytopenia with multilineage dysplasia or refractory anemia with excess blasts, while RT and RN (according to WHO 2008 are quite rare.These results suggest in particular that identification of RT and RN as distinct entities could be reconsidered in future WHO classification updates.

  9. Ipilimumab-Induced Neutropenia in Melanoma.

    Science.gov (United States)

    Ban-Hoefen, Makiko; Burack, Richard; Sievert, Lynn; Sahasrabudhe, Deepak

    2016-01-01

    Ipilimumab is a human monoclonal IgG1 antibody against CTLA-4 that has been shown to prolong the overall survival of advanced melanoma. The most common adverse events associated with ipilimumab are immune-related. Severe hematological toxicity is rare. We report a case of severe neutropenia following ipilimumab therapy that fully resolved after the administration of prednisone, cyclosporine, and anti-thymocyte globulin therapies. PMID:27570779

  10. Ipilimumab-Induced Neutropenia in Melanoma

    Science.gov (United States)

    Ban-Hoefen, Makiko; Burack, Richard; Sievert, Lynn; Sahasrabudhe, Deepak

    2016-01-01

    Ipilimumab is a human monoclonal IgG1 antibody against CTLA-4 that has been shown to prolong the overall survival of advanced melanoma. The most common adverse events associated with ipilimumab are immune-related. Severe hematological toxicity is rare. We report a case of severe neutropenia following ipilimumab therapy that fully resolved after the administration of prednisone, cyclosporine, and anti-thymocyte globulin therapies. PMID:27570779

  11. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia

    OpenAIRE

    Mariangelí Arroyo-Ávila; Fred-Jiménez, Ruth M.; Vilá, Luis M.

    2015-01-01

    Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L) a...

  12. Effect of therapy on the neutropenia of hyperthyroidism

    Energy Technology Data Exchange (ETDEWEB)

    Eakin, D.L.; Peake, R.L.; Weiss, G.B.

    1983-03-01

    Observations in a patient with recurrent hyperthyroidism, each time associated with neutropenia which resolved after therapy, prompted a chart review of other patients referred for radioactive iodine therapy. Of 99 untreated patients, 18 had neutrophil counts of less than 2,000/cu mm. After therapy with either thionamides or /sup 131/I, 41 of 53 (77%) evaluable patients had an increase in neutrophil count. Eleven of these evaluable patients had neutropenia before therapy; after therapy, all 11 had an increase in their neutrophil counts into the normal range, with a mean increase of 170%. In one patient, studies on the mechanism of neutropenia indicate that bone marrow production and reserve remain normal and that circulating neutrophils are normally marginated. A decreased neutrophil circulation time may be the cause of neutropenia associated with hyperthyroidism.

  13. Role of myeloid growth factors in chemotherapy induced neutropenia

    Directory of Open Access Journals (Sweden)

    Ravinutala Srinath Bharadwaj

    2016-10-01

    Full Text Available Neutropenia is a major dose limiting toxicity of many chemo therapeutic regimens. Haemopoietic colony - stimulating factors (CSFs have been shown to reduce the duration and severity of chemotherapy induced neutropenia (CIN and risk of febrile neutropenia. Supportive care with myeloid growth factors improve chemotherapy delivery by minimizing chemotherapy dose reductions or treatment delays by enabling the delivery of full dose chemotherapy (dose dense in short time intervals. The goal of this article is to give comprehensive review of current literature regarding medical practice guidelines and risk assessment models for appropriate use of myeloid growth factors and management of febrile neutropenia. [Int J Basic Clin Pharmacol 2016; 5(5.000: 1715-1721

  14. Sustained receptor activation and hyperproliferation in response to granulocyte colony-stimulating factor (G-CSF) in mice with a severe congenital neutropenia/acute myeloid leukemia-derived mutation in the G-CSF receptor gene.

    Science.gov (United States)

    Hermans, M H; Antonissen, C; Ward, A C; Mayen, A E; Ploemacher, R E; Touw, I P

    1999-02-15

    In approximately 20% of cases of severe congenital neutropenia (SCN), mutations are found in the gene encoding the granulocyte colony-stimulating factor receptor (G-CSF-R). These mutations introduce premature stop codons, which result in truncation of 82-98 COOH-terminal amino acids of the receptor. SCN patients who develop secondary myelodysplastic syndrome and acute myeloid leukemia almost invariably acquired a GCSFR mutation, suggesting that this genetic alteration represents a key step in leukemogenesis. Here we show that an equivalent mutation targeted in mice (gcsfr-Delta715) results in the selective expansion of the G-CSF- responsive progenitor (G-CFC) compartment in the bone marrow. In addition, in vivo treatment of gcsfr-Delta715 mice with G-CSF results in increased production of neutrophils leading to a sustained neutrophilia. This hyperproliferative response to G-CSF is accompanied by prolonged activation of signal transducer and activator of transcription (STAT) complexes and extended cell surface expression of mutant receptors due to defective internalization. In view of the continuous G-CSF treatment of SCN patients, these data provide insight into why progenitor cells expressing truncated receptors clonally expand in vivo, and why these cells may be targets for additional genetic events leading to leukemia. PMID:9989983

  15. Sustained Receptor Activation and Hyperproliferation in Response to Granulocyte Colony-stimulating Factor (G-CSF) in Mice with a Severe Congenital Neutropenia/Acute Myeloid Leukemia–derived Mutation in the G-CSF Receptor Gene

    Science.gov (United States)

    Hermans, Mirjam H.A.; Antonissen, Claudia; Ward, Alister C.; Mayen, Angelique E.M.; Ploemacher, Rob E.; Touw, Ivo P.

    1999-01-01

    In approximately 20% of cases of severe congenital neutropenia (SCN), mutations are found in the gene encoding the granulocyte colony-stimulating factor receptor (G-CSF–R). These mutations introduce premature stop codons, which result in truncation of 82–98 COOH-terminal amino acids of the receptor. SCN patients who develop secondary myelodysplastic syndrome and acute myeloid leukemia almost invariably acquired a GCSFR mutation, suggesting that this genetic alteration represents a key step in leukemogenesis. Here we show that an equivalent mutation targeted in mice (gcsfr-Δ715) results in the selective expansion of the G-CSF– responsive progenitor (G-CFC) compartment in the bone marrow. In addition, in vivo treatment of gcsfr-Δ715 mice with G-CSF results in increased production of neutrophils leading to a sustained neutrophilia. This hyperproliferative response to G-CSF is accompanied by prolonged activation of signal transducer and activator of transcription (STAT) complexes and extended cell surface expression of mutant receptors due to defective internalization. In view of the continuous G-CSF treatment of SCN patients, these data provide insight into why progenitor cells expressing truncated receptors clonally expand in vivo, and why these cells may be targets for additional genetic events leading to leukemia. PMID:9989983

  16. Leukopenia and neutropenia caused by sertraline%舍曲林致白细胞和中性粒细胞减少

    Institute of Scientific and Technical Information of China (English)

    庄红艳; 刘珊珊; 杜海霞; 崔永华

    2016-01-01

    1例11岁女性患儿因童年情绪障碍服用舍曲林25 mg、喹硫平25 mg,1次/ d。2 d 后实验室检查:WBC 4.6×109/ L,中性粒细胞计数(NEUT)1.8×109/ L。诊断:中性粒细胞减少。停用喹硫平,继续服用舍曲林,给予盐酸小檗胺84 mg、利可君20 mg,3次/ d 口服。6 d 后为改善病情,舍曲林剂量增至50 mg,1次/ d。同时加用维生素 B610 mg,3次/ d 口服。舍曲林治疗第13天,患儿 WBC 3.5×109/ L ,NEUT 1.4×109/ L,诊断为白细胞减少。舍曲林治疗第24天,患儿 WBC 3.3×109/ L, NEUT 1.4×109/ L。加用安多霖胶囊0.64 g,2次/ d 口服。次日舍曲林减量至25 mg,1次/ d,2 d 后停用。5 d 后复查,患儿 WBC 4.4×109/ L,NEUT 2.0×109/ L。给予氟伏沙明改善情绪。7 d 后,患儿WBC 4.6×109/ L,NEUT 2.3×109/ L。%An 11-year-old female patient was given sertraline 25 mg once daily and quetiapine 25 mg once daily for childhood emotional disorders. The patient's white blood cells count(WBC)was 4. 6 ×109 / L and neutrophil count( NEUT)was 1. 8 ×109 / L two days later. The patient was diagnosed as neutropenia. Quetiapine was stopped and sertraline was given continually. At the same time berbamine hydrochloride 84 mg thrice daily and leucogen 20 mg thrice daily were given. Six days later,the dosage of sertraline was increased to 50 mg once daily to improve the symptoms and vitamin B6 10 mg thrice daily was given. On day 13 of sertraline therapy,the WBC was 3. 5 ×109 / L and the NEUT was 1.4×109 / L. Leukopenia was diagnosed. On day 24 of sertraline therapy,the WBC was 3. 3 ×109 / L and the NEUT was 1.4×109 / L. Anduolin(安多霖)capsules 0. 64 g twice daily were added to her regimen. Sertraline dose was reduced to 25 mg once daily the next day and was stopped 2 days later. Five days later,the patient's WBC was 4.4×109 / L and NEUT was 2. 0 ×109 / L. Then,fluvoxamine was given to improve mood. Seven days later,the patient's WBC was 4. 6

  17. 幼儿急疹引起粒细胞减少症的中西医结合治疗临床研究%Clinical study of children acute rash caused neutropenia in integrative medicine

    Institute of Scientific and Technical Information of China (English)

    彭丽琨

    2015-01-01

    Objective:To discuss the clinical study of children acute rash caused neutropenia in integrative medicine.Methods:120 patients with acute rash were selected.They were divided into the experimental group and the control group.Two groups were given Siji antiviral mixture,and the experimental group added oral leucogen on the basis of oral Siji antiviral mixture.Then we analyzed peripheral blood test for the children in the administration of third,7 and 14 days.Results:The control group and the experimental group were released after 7~14 days,and there was no significant difference.The average recovery time of the experimental group was earlier than that of the control group.Conclusion:The effect of the integrated traditional Chinese and western medicine therapy in the treatment of children acute rash caused by neutropenia was better,and recovered faster.%目的:探讨中西医结合治疗幼儿急疹引起粒细胞减少症的临床研究。方法:收治急疹患儿120例,分成对照组和试验组,两组均口服四季抗病毒合剂,试验组在口服四季抗病毒合剂的基础上,加口服利血生。在给药后3 d、7 d 和14 d 对患儿进行外周血象检测分析。结果:对照组和试验组的患儿均在发病后第7~14天恢复,无明显差异。试验组平均恢复时间较对照组短。结论:中西医结合治疗幼儿急疹引起粒细胞减少症恢复较快,效果较好。

  18. Severe upper airway obstruction caused by ulcerative laryngitis

    OpenAIRE

    Hatherill, M.; Reynolds, L; Waggie, Z; Argent, A

    2001-01-01

    AIMS—To present our experience of severe upper airway obstruction caused by ulcerative laryngitis in children.
METHODS—Retrospective case note review of 263 children with severe upper airway obstruction and a clinical diagnosis of croup admitted to a paediatric intensive care unit (PICU) over a five year period.
RESULTS—A total of 148 children (56%) underwent microlaryngoscopy (Storz 3.0 rigid telescope). Laryngeal ulceration with oedema was documented in 15 of these childre...

  19. Microbiology of destructive periodontal disease in adolescent patients with congenital neutropenia - A report of 3 cases

    NARCIS (Netherlands)

    van Winkelhoff, AJ; Schouten-van Meeteren, AYN; Baart, JA; Vandenbroucke-Grauls, CMJE

    2000-01-01

    Background, aims: Congenital neutropenia is one condition that may predispose for destructive periodontal disease at a young age. In this report, we describe the microbiology of 3 adolescent patients with congenital neutropenia two of whom suffered from severe periodontitis. Method: Microbiological

  20. CEREBRITIS AND NEUTROPENIA IN A CHILD WITH ANA NEGATIVE LUPUS

    Directory of Open Access Journals (Sweden)

    J. Akhoondian

    2009-04-01

    Full Text Available ObjectiveSystemic lupus erythematosus (SLE, an autoimmune systemic disease with unknown etiology, affects virtually every part of the body; involvement of the central nervous system (CNS is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE patients and is the least understood aspect of the disease. neutropenia is very uncommon in childhood lupus. True negative anti nuclear antibody (ANA tests in patients with lupus are now very rare. The patient reported here was a 12-year-old girl with ANA negative lupus cerebritis who presented with left hemiparesia after a generalized seizure, with neutropenia observed during its course.Key words:lupus cerebritis, neutropenia, ANA negative lupus, children

  1. CEREBRITIS AND NEUTROPENIA IN A CHILD WITH ANA NEGATIVE LUPUS

    Directory of Open Access Journals (Sweden)

    J. Akhoondian

    2009-01-01

    Full Text Available ObjectiveSystemic lupus erythematosus (SLE, an autoimmune systemic disease with unknown etiology, affects virtually every part of the body; involvement of the central nervous system (CNS is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE patients and is the least understood aspect of the disease. neutropenia is very uncommon in childhood lupus. True negative anti nuclear antibody (ANA tests in patients with lupus are now very rare. The patient reported here was a 12-year-old girl with ANA negative lupus cerebritis who presented with left hemiparesia after a generalized seizure, with neutropenia observed during its course.

  2. Chemotherapy-induced neutropenia and febrile neutropenia in patients with gynecologic malignancy.

    Science.gov (United States)

    Hashiguchi, Yasunori; Kasai, Mari; Fukuda, Takeshi; Ichimura, Tomoyuki; Yasui, Tomoyo; Sumi, Toshiyuki

    2015-11-01

    Chemotherapy-induced neutropenia is a common complication in cancer treatment. In this study, we investigated chemotherapy-induced neutropenia that was recently detected in all patients with gynecologic malignancy. Between January 2009 and December 2011, we examined cases of chemotherapy-induced neutropenia reported in our hospital. We analyzed the incidence and clinical features of chemotherapy-induced neutropenia and febrile neutropenia in patients with gynecologic malignancy. During the study period, we administered over 1614 infusions (29 regimens) to 291 patients. The median age of the patients was 60 years (range 24-84 years). Chemotherapy-induced neutropenia occurred in 147 (50.5%) patients over 378 (23.4%) chemotherapy cycles. Febrile neutropenia occurred in 20 (6.9%) patients over 25 (1.5%) cycles. The mean duration of neutropenia and fever was 3.6 days (range 1-12 days) and 3.4 days (range 1-9 days), respectively. The source of fever was unexplained by examination or cultures in 14 (56.0%) cycles. There were two cases of neutropenia-related death. Chemotherapy-induced neutropenia was associated with older age (over 70 years) (PFebrile neutropenia was associated with poor performance status (Pneutropenia nor febrile neutropenia was associated with bone marrow metastases or previous radiotherapy. By identifying risk factors for febrile neutropenia, such as performance status, no previous chemotherapy, disseminated disease, and distant metastatic disease, the safe management of chemotherapy-induced neutropenia may be possible in patients with gynecologic malignancy.

  3. Generation of poikiloderma with neutropenia (PN) induced pluripotent stem cells (iPSCs).

    Science.gov (United States)

    Mills, Jason A; Hudock, Kristin M; Sullivan, Spencer K; Herrera, Pamela; Sullivan, Lisa M; Gadue, Paul; French, Deborah L

    2015-11-01

    Poikiloderma with neutropenia (PN, Clericuzio-type poikiloderma with neutropenia) is a rare autosomal recessive disorder caused by biallelic mutations in the USB1 gene (Alias C16orf57 and MPN1). To date, there have been only 37 reported cases worldwide of this disorder that presents with neutropenia, early onset poikiloderma, respiratory infections, palmo-plantar hyperkeratosis, and skeletal defects. Here we described the generation of human induced pluripotent stem cell lines (PN1 and PN2) from the peripheral blood of a 1-year-old patient using the dox-inducible STEMCCA vector. This patient presented with bacteremia, pneumonia, and neutropenia. Analysis of bone marrow demonstrated normal cellularity with trilineage hematopoiesis and neutropenia.

  4. [Severe atopic dermatitis caused by rare immunodeficiency in childhood].

    Science.gov (United States)

    Wolsk, Helene Mygind; Marquart, Hanne V; Laub, Bodil; Gniadecki, Robert; Nysom, Karsten; Ifversen, Marianne

    2015-12-14

    Two children are presented with autosomal recessive hyper IgE syndrome caused by a mutation in the dedicator of cytokinesis 8 gene (DOCK8). The manifestations are typically severe atopic dermatitis, food allergies, elevated serum IgE concentration, viral skin infections and risk of malignancies. DOCK8 deficiency was first reported in 2009, following the death of the oldest sibling. The youngest sibling was cured after allogenic stem cell transplantation. This case report illustrates the need of awareness of primary immunodeficiency in children with atypical manifestation of atopic dermatitis in combination with recurrent infections. PMID:26692033

  5. Fever and neutropenia in cancer patients : the diagnostic role of cytokines in risk assessment strategies

    NARCIS (Netherlands)

    Nijhuis, CSMO; Daenen, SMGJ; Vellenga, E; van der Graaf, WTA; Gietema, JA; Groen, HJM; Kamps, WA; de Bont, ESJM

    2002-01-01

    Cancer patients treated with chemotherapy are susceptible to bacterial infections. Therefore, all neutropenic cancer patients with fever receive standard therapy consisting of broad-spectrum antibiotics and hospitalization. However, febrile neutropenia in cancer patients is often due to other causes

  6. Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia Characterization of neutropenic pediatric patients sent to a referral centre

    Directory of Open Access Journals (Sweden)

    Gustavo Adolfo Lazo-Páez

    2010-06-01

    ógica relativamente simple. Las infecciones documentadas en los casos analizados suelen ser causadas por los mismos gérmenes descritos en otras series. El conteo absoluto de neutrófilos al diagnóstico no incide sobre la frecuencia de infección recurrente, pero el tipo o curso clínico de la neutropenia sí lo hace.Aim: Neutropenia is a relatively common cause of patient referral to the Immunology and Pediatric Rheumatology Department of the National Children’s Hospital. The present study characterizes the cases of neutropenia referred to this department between November 1988 and June 2008. Methods: Eighty four patients between 0 and 12 years of age, were referred from November 6th, 1988 and June 1st, 2008. We performed a comprehensive descriptive analysis of the characteristics exhibited by these patients in terms of clinical course, pattern of infection, most common causative germs, complications and treatment applied. Results: Neutropenia resolved spontaneously in 52.2% of the patients, and they were classified as transient neutropenia, 21.7% of the cases developed cyclic neutropenia, 13% of were categorized as benign chronic neutropenia, 7.2% developed severe chronic symptomatic neutropenia, 2.9% had neutropenia associated with type 1B glycogenosis and 2.9% of the cases were not classifiable in any of the proposed categories. More than 50% of the cases were associated with an abnormal pattern of infection in terms of frequency, severity, multiplicity of systems involved, or the presence of opportunistic microorganisms. The upper respiratory tract was the most commonly affected system with infection. Prophylactic antibiotics were used in 39.1% of the cases and granulocyte colony stimulating factor was required in 11.6% at some stage of the course. The most frequently involved pathogens in infection were Pseudomonas aeruginosa, Staphylococcus sp and E. coli. Conclusion: The vast majority of neutropenic patients had a benign clinical course. The same germs described in other

  7. Role of biosimilars in neutropenia prevention in cancer patients

    Directory of Open Access Journals (Sweden)

    V. V. Ptushkin

    2014-01-01

    Full Text Available Decreasing the neutrophils count in peripheral blood after intensive chemotherapy (CT dramatically increases the risk of infectious complications.As a consequence, treatment costs significantly increased and patients quality of life reduced. Correction of neutropenia is possible with granulocyte colony stimulating factor (G-CSF – a human protein produced by recombinant technology and is able to support the survival and proliferation of hematopoietic stem cells. Pharmacoeconomic studies have shown that G-CSF reduces the frequency of hospitalization and antibiotics using, which can reduce the treatment cost. The use of G-CSF allows to reduce early and infection mortality after chemotherapy, providing background to prolonging life especially for the elderly (over 65 years and debilitated patients. The drug is included in all international recommendations. However, its use in Russia is limited due to high cost.Part of the policy aimed to reducing protein drugs cost and increase their availability is the creation of biosimilars protein drugs with proven effective. At the same time biosimilars as the original protein molecules are living cells products, causing serious difficulties in achieving their identity. To eliminate the risk of reducing the effectiveness or increase the toxicity, the European Union established regulations for the determination the bioproducts quality, a detailed description of the requirements for pre-clinical and clinical research, as well as the requirements for pharmacovigilance. Registered in the EEC countries G-CSF biosimilars have been first studied in healthy volunteers, and then in controlled clinical trials in comparison with the reference drug. High efficacy of one such G-CSF biosimilars (Zarsio® was shown in controlled clinical trials of 170 patients with breast cancer receiving intensive chemotherapy with Docetaxel and Doxorubicin. Total in the study only 6 % cases of febrile neutropenia (FN was

  8. Re-challenge with Etanercept in patients with Etanercept-induced Neutropenia.

    LENUS (Irish Health Repository)

    Haroon, Muhammad

    2011-08-05

    TNF blockers have rarely been associated with haematological complications; however, there are scattered case reports of marked neutropenia with their use and necessitating in their withdrawal. We would like to report a series of five patients who developed neutropenia with etanercept use; however, all these patients were re-challenged with etanercept with a mean follow up of 30 months. These patients developed neutropenia within 2 months of starting etanercept. Two patients were eventually taken off etanercept; one of them needed switching to a different form of TNF blockers, and the second patient is in clinical remission with low-dose corticosteroids. All our patients continued to have mild-moderate degree of neutropenia; however, they are being monitored very closely and they are enjoying complete disease remission. It was interesting to note that none of our patients had increased infections during the re-challenge phase, even though they had grade 2 to grade 4 neutropenia. We have re-challenged these patients without any clinical complications, revealing that patients with mild to moderate neutropenia can be safely exposed to TNF blockers as long as they are monitored with regular cell count checks. Although largely noted to be clinically insignificant in our patient series, the potential of drug-induced neutropenia in causing higher rate of infections do exist. Careful clinical and hematologic monitoring is the best way to recognize this adverse event.

  9. Secondary Infections in Cancer Patients with Febrile Neutropenia

    Directory of Open Access Journals (Sweden)

    Alpay Azap

    2012-09-01

    Full Text Available OBJECTIVE: Patients with neutropenia due to cancer chemotherapy are prone to severe infections. Cancer patients can experience >1 infectious episode during the same period of neutropenia. This study aimed to determine the etiological and clinical characteristics of secondary infectious episodes in cancer patients with febrile neutropenia and to identify the factors associated with the risk of secondary infectious episodes. METHODS: All cancer patients that received antineoplastic chemotherapy at Ankara University, School of Medicine, Department of Hematology between May 2004 and May 2005 and developed neutropenia were included in the study. Data were collected using survey forms that were completed during routine infectious diseases consultation visits. Categorical data were analyzed using the chi-square test, whereas Student’s t-test was used for continuous variables. Multivariate logistic regression analysis was performed to identify independent predictors of secondary infections (SIs. RESULTS: SIs were observed during 138 (53% of 259 febrile neutropenic episodes. Of the 138 episodes, 89 (64.5% occurred in male patients with a mean age of 40.9 years (range: 17-76 years. In total, 80% of the SIs were clinically or microbiologically documented. Factors on d 4 of the initial febrile episode were analyzed via a logistic regression model. The presence of a central intravenous catheter (OR: 3.01; P < 0.001, acute myeloid leukemia (AML as the underlying disease (OR: 2.12; P = 0.008, diarrhea (OR: 4.59; P = 0.005, and invasive aspergillosis (IA during the initial febrile episode (OR: 3.96; P = 0.009 were statistically significant risk factors for SIs. CONCLUSION: Among the cancer patients with neutropenia in the present study, AML as the underlying disease, the presence of a central venous catheter, diarrhea, and IA during the initial febrile episode were risk factors for the development of SIs.

  10. CEREBRITIS AND NEUTROPENIA IN A CHILD WITH ANA NEGATIVE LUPUS

    OpenAIRE

    J. Akhoondian; Z. Rezaii yazdi; F Behmanesh; Talebi, S.

    2009-01-01

    ObjectiveSystemic lupus erythematosus (SLE), an autoimmune systemic disease with unknown etiology, affects virtually every part of the body; involvement of the central nervous system (CNS) is one of the major causes of morbidity and mortality in systemic lupus erythematosus (SLE) patients and is the least understood aspect of the disease. neutropenia is very uncommon in childhood lupus. True negative anti nuclear antibody (ANA) tests in patients with lupus are now very rare. The patient repor...

  11. Mefenamic acid-induced neutropenia and renal failure in elderly females with hypothyroidism.

    OpenAIRE

    Handa, S I; FREESTONE, S.

    1990-01-01

    We report mefenamic acid-induced non-oliguric renal failure and severe neutropenia occurring simultaneously in two elderly females. The neutropenia was due to maturation arrest of the myeloid series in one patient. Both patients were also hypothyroid, but it is not clear whether this was a predisposing factor to the development of these adverse reactions. However, it would seem prudent not to use mefenamic acid in hypothyroid patients until the hypothyroidism has been corrected.

  12. Lipegfilgrastim in the management of chemotherapy-induced neutropenia of cancer patients

    Directory of Open Access Journals (Sweden)

    Guariglia R

    2016-01-01

    Full Text Available Roberto Guariglia,1 Maria Carmen Martorelli,1 Rosa Lerose,2 Donatella Telesca,2 Maria Rita Milella,2 Pellegrino Musto3 1Unit of Hematology and Stem Cell Transplantation, 2Pharmacy Service, 3Scientific Direction, IRCCS, Referral Cancer Center of Basilicata, Rionero in Vulture, Potenza, Italy Abstract: Neutropenia and febrile neutropenia (FN are frequent and potentially fatal toxicities of myelosuppressive anticancer treatments. The introduction of granulocyte colony-stimulating factors (G-CSFs in clinical practice has remarkably reduced the duration and severity of neutropenia, as well as the incidence of FN, thus allowing the administration of chemotherapeutic agents at the optimal dose and time with lower risk. The current scenario of G-CSFs in Europe includes filgrastim, lenograstim, some G-CSF biosimilars, and pegfilgrastim. Recently, a novel long-acting G-CSF, lipegfilgrastim, became available. Lipegfilgrastim is a glycopegylated G-CSF, alternative to pegfilgrastim, and has shown in randomized trials, to be equivalent to pegfilgrastim in reducing the incidence of severe neutropenia and FN in patients with breast cancer receiving chemotherapy, with a similar safety profile. Furthermore, lipegfilgrastim was more effective than the placebo in reducing the incidence of severe neutropenia, its duration, and time to absolute neutrophil count recovery, in patients with non-small cell lung cancer receiving myelosuppressive therapy. Although the number of studies currently published is still limited, lipegfilgrastim seems to be a promising drug in the management of chemotherapy-induced neutropenia. Keywords: neutropenia, febrile neutropenia, granulocyte colony-stimulating factors, G-CSF, pegfilgrastim, lipegfilgrastim

  13. ABCB1基因多态性与乳腺癌患者化疗所致严重中性粒细胞减少症的相关性研究%Association between ABCB1 Gene Polymorphisms and Chemotherapy-induced Severe Neutropenia in Pa-tients with Breast Cancer

    Institute of Scientific and Technical Information of China (English)

    付正传; 钱芳; 杨旭环; 宫素红; 程曙光; 刘思海

    2016-01-01

    OBJECTIVE:To discuss the association between ABCB1 gene polymorphisms and adriamycin and cyclophospha-mide(AC)combined with chemotherapy-induced severe neutropenia in patients with breast cancer. METHODS:218 breast cancer patients receiving AC combined with chemotherapy were selected from our hospital during 2012-2015;PCR-RFLP was used to de-tect polymorphisms of ABCB1 2677G>T/A and 3435C>T. The associated between different age,BMI,clinical stages genotypes, etc and AC combined with chemotherapy-induced severe neutropenia were investigated,and risk factors of neutropenia were ana-lyzed by multivariate logistic regression. RESULTS:Among 218 breast cancer patients,170 patients suffered from severe neutrope-nia,accounting for 78.0%. Among ABCB1 2677G>T/A polymorphisms,distribution frequency of GT or GA genotype,TT,TA or AA genotype,GG genotype in severe neutropenia were 80.6%,86.2% and 60.0%,with statistical significance (PT polymorphisms,distribution frequency of TT,CT and CC genotype in severe neutropenia were 86.4%, 78.4% and 72.7%,there was no statistical significance(P>0.05). AST and ABCB1 2677G>T/A polymorphisms were correlated with severe neutropenia (PT/A polymorphism was a strong predictor of neutropenia [OR=3.875, 95%CI(1.555,9.922),P=0.008]. CONCLUSIONS:ABCB1 2677>T/A polymorphisms may be aggravate AC combined with che-motherapy-induced neutropenia in patients with breast cancer.%目的:探讨乳腺癌患者三磷酸腺苷结合盒转运子B亚家族成员1(ABCB1)基因多态性与多柔比星和环磷酰胺(AC)联合化疗所致严重中性粒细胞减少症的相关性。方法:选择我院2012-2015年接受AC联合化疗的乳腺癌患者218例,采用聚合酶链-限制性片段长度多态性分析法进行ABCB12677G>T/A、3435C>T基因多态性检测,考察患者不同年龄、体质量指数、临床分期、基因型等各因素与AC联合化疗所致严重中性粒细胞减少症的相关性,并采用多元逻辑回归分析

  14. Severe lymphedema caused by repeated self-injury.

    Science.gov (United States)

    Mihara, M; Hara, H; Murai, N; Todokoro, T; Iida, T; Narushima, M; Koshima, I

    2011-12-01

    Lymphedema is divided into primary and secondary forms. Primary lymphedema often develops in young people and may be caused by lymphvascular aplasia, hypoplasia, and hyperplasia. The most frequent cause of secondary lymphedema after lymphatic filariasis is regional lymph node dissection for treatment of a malignant tumor, and this complication occurs most frequently in middle aged or older patients. Here, we describe a relatively young patient (27 years old) in whom collecting lymph vessels in the upper limb were disrupted by repeated self-injury, with resultant lymphedema. There have been very few reports on lymphedema caused by self-induced trauma. This case report illustrates that secondary lymphedema should also be considered and evaluated appropriately when diagnosed in a relatively young patient without a history of cancer or infection. PMID:22458120

  15. A cause of severe thigh injury: Battery explosion

    Directory of Open Access Journals (Sweden)

    Tahsin Görgülü

    2016-02-01

    Discussion: Battery explosion causing lower extremity tissue defect is a type of injury that is rarely seen in the literature. Regardless of battery size and energy level, they should be considered as potential explosive material and protector masks, clothing should be worn during contact with this type of material.

  16. A rare cause of severe hepatomegaly with an improving outcome

    DEFF Research Database (Denmark)

    Godskesen, Line Elberg; Abildgaard, Niels; Kjeldsen, Jens;

    2014-01-01

    A previously healthy 43-year-old man presented with dyspnoea, 15 kg weight loss, severe hepatomegaly and alkaline phosphatase at 5400 U/L. Examinations seemed to suggest cirrhosis, but blood samples did not show any signs of underlying liver disease. Liver biopsy revealed amyloid light chain (AL...

  17. Hormonal Treatment for Severe Hydronephrosis Caused by Bladder Endometriosis

    Directory of Open Access Journals (Sweden)

    Erkan Efe

    2014-01-01

    Full Text Available The incidence of endometriosis cases involving the urinary system has recently increased, and the bladder is a specific zone where endometriosis is most commonly seen in the urinary system. In the case presented here, a patient presented to the emergency department with the complaint of side pain and was examined and diagnosed with severe hydronephrosis and bladder endometriosis was determined in the etiology. After the patient was pathologically diagnosed, Levonorgestrel-Releasing Intrauterine System (LNG-IUS was administered to the uterine cavity. At the 12-month follow-up, endometriosis was not observed in the cystoscopy and symptoms had completely regressed. Hydronephrosis may be observed after exposure of the ureter, and silent renal function loss may develop in patients suffering from endometriosis with bladder involvement. For patients with moderate or severe hydronephrosis associated with bladder endometriosis, LNG-IUS application may be separately and successfully used after conservative surgery.

  18. Acute starvation in pregnancy: a cause of severe metabolic acidosis.

    Science.gov (United States)

    Patel, A; Felstead, D; Doraiswami, M; Stocks, G M; Waheed, U

    2011-07-01

    We report a case of starvation-induced metabolic ketoacidosis in a previously healthy 29-year-old, nulliparous woman at 32 weeks of gestation. She was admitted to hospital with mild preeclampsia associated with persistent nausea and vomiting that progressed to severe preeclampsia requiring urgent control of hypertension before caesarean delivery. Prolonged and severe vomiting limited oral caloric intake and led to starvation ketoacidosis, characterised by ketonuria and a raised anion gap metabolic acidosis that required intensive care support. Despite significant metabolic derangement the patient appeared clinically well. Intravascular volume was replenished. Fluid restriction used as part of our preeclampsia treatment regimen delayed the therapeutic administration of sufficient dextrose, which rapidly corrected her metabolic derangement when commenced after delivery. Electrolyte supplementation was given to prevent re-feeding syndrome. Both mother and baby were discharged without sequelae.

  19. Gelsolin amyloid angiopathy causes severe disruption of the arterial wall.

    Science.gov (United States)

    Koskelainen, Susanna; Pihlamaa, Tiia; Suominen, Sinikka; Zhao, Fang; Salo, Tuula; Risteli, Juha; Baumann, Marc; Kalimo, Hannu; Kiuru-Enari, Sari

    2016-08-01

    Hereditary gelsolin amyloidosis (HGA) is a dominantly inherited systemic disease reported worldwide. HGA is characterized by ophthalmological, neurological, and dermatological manifestations. AGel amyloid accumulates at basal lamina of epithelial and muscle cells, thus amyloid angiopathy is encountered in nearly every organ. HGA patients have cardiovascular, hemorrhagic, and potentially vascularly induced neurological problems. To clarify pathomechanisms of AGel angiopathy, we performed histological, immunohistochemical, and electron microscopic analyses on facial temporal artery branches from 8 HGA patients and 13 control subjects. We demonstrate major pathological changes in arteries: disruption of the tunica media, disorganization of vascular smooth muscle cells, and accumulation of AGel fibrils in arterial walls, where they associate with the lamina elastica interna, which becomes fragmented and diminished. We also provide evidence of abnormal accumulation and localization of collagen types I and III and an increase of collagen type I degradation product in the tunica media. Vascular smooth muscle cells appear to be morphologically and semi-quantitatively normal, only their basal lamina is often thickened. In conclusion, angiopathy in HGA results in severe disruption of arterial walls, characterized by prominent AGel deposition, collagen derangement and severe elastolysis, and it may be responsible for several, particularly hemorrhagic, disease manifestations in HGA. PMID:27198069

  20. Radiation-induced oesophagitis in lung cancer patients. Is susceptibility for neutropenia a risk factor?

    Energy Technology Data Exchange (ETDEWEB)

    Ruysscher, D. de [MAASTRO Clinic, Maastricht (Netherlands). Dept. of Radiation Oncology; Meerbeeck, J. van [Ghent Univ. Hospital (Belgium). Dept. of Respiratory Medicine; Vandecasteele, K. [Ghent Univ. Hospital (BE). Dept. of Radiation Oncology] (and others)

    2012-07-15

    Background: Radiation-induced oesophagitis is a major side effect of concurrent chemotherapy and radiotherapy. A strong association between neutropenia and oesophagitis was previously shown, but external validation and further elucidation of the possible mechanisms are lacking. Methods and patients: A total of 119 patients were included at two institutions. The concurrent group comprised 34 SCLC patients treated with concurrent carboplatin and etoposide, and concurrent chest irradiation, and 36 NSCLC patients with concurrent cisplatin and etoposide, and concurrent radiotherapy, while the sequential group comprised 49 NSCLC patients received sequential cisplatin and gemcitabine, and radiotherapy. Results: Severe neutropenia was very frequent during concurrent chemoradiation (grade: 4 41.4%) and during induction chemotherapy in sequentially treated patients (grade 4: 30.6%), but not during radiotherapy (only 4% grade 1). In the concurrent group, the odds ratios of grade 3 oesophagitis vs. neutropenia were the following: grade 2 vs. grade 0/1: 5.60 (95% CI 1.55-20.26), p = 0.009; grade 3 vs. grade 0/1: 10.40 (95% CI 3.19-33.95); p = 0.0001; grade 4 vs. grade 0/1: 12.60 (95% CI 4.36-36.43); p < 0.00001. There was no correlation between the occurrence of neutropenia during induction chemotherapy and acute oesophagitis during or after radiotherapy alone. In the univariate analysis, total radiation dose (p < 0.001), overall treatment time of radiotherapy (p < 0.001), mean oesophageal dose (p = 0.038) and neutropenia (p < 0.001) were significantly associated with the development of oesophagitis. In a multivariate analysis, only neutropenia remained significant (p = 0.023). Conclusion: We confirm that neutropenia is independently correlated with oesophagitis in concurrent chemoradiation, but that the susceptibility for chemotherapy-induced neutropenia is not associated with radiation-induced oesophagitis. Further studies focusing on the underlying mechanisms are thus

  1. Severe head injury caused by motorcycle traffic accident

    Institute of Scientific and Technical Information of China (English)

    李钢

    1999-01-01

    Objective To explore the characteristic and treatment of the severe head injury due to motorcycle accident.Methods Review and analysis of 27 motorcycle traffic trauma cases who were admitted to our hospital from Oct.1995 to Sep.1997.Results Young men were the main composition of these patients.Multiple injuries associated with brain ste or diffuse axonal injury were common,which were the main factors influencing the consciousness and prognosis of the patients.The wound was usually severely contaminated.Evacuation of hematomas,decompression by depleting skull flap,hypotheymia and artificial hibernation were conducted in this series.Among them,14 cases were cured ,3 cases were seriously disabled,10 cases died.Conclusions Motorcycle's weight is light so it easily loses its balance.The riders and the passengers are exposed and lack protection.Driving against traffic regulations is frquently seen.All these are the reasons why the motorcycle traffic accidents often take place. When the traffic accident happens,the patients' head generally is thrown a long distance and dashed against the barrier or the ground.The psture nd mechanism of injury were complicated and varied.The decelerated injury and rolling injury occurred frequently and they were the main reasons for brain stem or diffuse axonal injury.The patients who have surgical indication should be operated upon as soon as possible.Hibernation and low temoerature therapy are conducive to the protection of the brain function at the early stage of postinjury or postoperation.A careful epluchage is essential to reduce infection of the open injury.

  2. [Scorpionism causing severe acute flaccid paralysis. Case report].

    Science.gov (United States)

    Villa-Manzano, Alberto I; Vázquez-Solís, Ma Guadalupe; Zamora-López, Xochitl Xitlalli; Arias-Corona, Fernando; Palomera-Ávila, Francisco Miguel; Pulido-Galaviz, Carlos; Pacifuentes-Orozco, Adán

    2016-01-01

    Introducción: el alacranismo es un problema de salud pública en diversas regiones del mundo, siendo México el país que tiene mayor número de casos. Las manifestaciones clínicas oscilan desde sintomatología local hasta cuadros graves con repercusiones a nivel cardiovascular, respiratorio y neurológico, e incluso la muerte. No existen reportes de parálisis flácida como una manifestación del cuadro clínico por picadura del alacrán del género Centruroides, familia Buthidae, especie altamente tóxica, endémica y causante de altos índices de morbimortalidad en nuestra región. Caso clínico: documentamos un caso de alacranismo grave, provocado por escorpión de la familia Buthidae del género Centruroides, que causó parálisis flácida aguda, posterior a resolución de otras manifestaciones severas. Solo existe un reporte de caso de alacranismo que produce parálisis flácida aguda en la literatura médica, pero relacionado con el escorpión de la familia Parabuthus, endémico de Sudáfrica, el cual no es endémico en México. Conclusiones: conocer esta complicación, nueva para nuestra región, permitirá maximizar esfuerzos para diagnosticar y manejar oportunamente esta entidad con la aplicación temprana de faboterápico específico y soporte vital avanzado.

  3. Early-onset neutropenia induced by rituximab in a patient with lupus nephritis and hemolytic anemia.

    Science.gov (United States)

    Arroyo-Ávila, Mariangelí; Fred-Jiménez, Ruth M; Vilá, Luis M

    2015-01-01

    Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 10(9)/L) after the second weekly rituximab infusion (375 mg/m(2) weekly × 4) given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections. PMID:25767732

  4. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia

    Directory of Open Access Journals (Sweden)

    Mariangelí Arroyo-Ávila

    2015-01-01

    Full Text Available Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L after the second weekly rituximab infusion (375 mg/m2 weekly × 4 given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections.

  5. Febrile neutropenia in children treated for malignancy.

    Science.gov (United States)

    Barton, Chris D; Waugh, Lucy K; Nielsen, Maryke J; Paulus, Stéphane

    2015-06-01

    Febrile neutropenia (FN) in children treated for malignancy is a common and direct sequela of chemotherapy. Episodes of FN can be life-threatening, and demand prompt recognition, assessment and treatment with broad spectrum antibiotics. While in the majority of episodes no causal infection is identified, 10-20% are secondary to a bloodstream infection (BSI). A reduction in episodes of BSI could be achieved through robust infection prevention strategies, such as CVL care bundles. Alongside good antimicrobial stewardship, these strategies could reduce the risk of emergent, multi-drug resistant (MDR) infections. Emerging bacterial pathogens in BSI include Viridans Group Streptococci (VGS) and Enterobacteriaceae such as Klebsiella spp. which are known for their ability to carry MDR genes. There is also increased recognition of the role of invasive fungal infection (IFI) in FN, in particular with Aspergillus spp. Novel diagnostics, including multiplex blood and respiratory polymerase chain reaction assays can identify infections early in FN, facilitating targeted therapy, and reducing unnecessary antimicrobial exposure. Given appropriate, and sensitive rapid diagnostics, potential also exists to safely inform the risk assessment of patients with FN, identifying those at low risk of complication, who could be treated in the out-patient setting. Several clinical decision rules (CDR) have now been developed and validated in defined populations, for the risk assessment of children being treated for cancer. Future research is needed to develop a universal CDR to improve the management of children with FN.

  6. Bone Marrow Suppression and Hemophagocytic Histiocytes Are Common Findings in Korean Severe Fever with Thrombocytopenia Syndrome Patients.

    Science.gov (United States)

    Shin, Sang Yong; Cho, Oh Hyun; Bae, In Gyu

    2016-09-01

    The causes of cytopenia in patients with severe fever with thrombocytopenia syndrome (SFTS) are not fully understood until now. We reviewed the bone marrow (BM) findings of patients with SFTS to unravel the cause of the cytopenia. Three Korean SFTS were enrolled in this study. Thrombocytopenia, neutropenia, and anemia were detected in all three patients. Severe hypocellular marrow (overall cellularity Korean SFTS. PMID:27401664

  7. Neutropenia in the Elderly: A Rheumatology Perspective.

    Science.gov (United States)

    Yeoh, Su-Ann; Fox, Christine; Hull, Richard

    2016-08-01

    The majority of rheumatic diseases are chronic and require long-term use of disease-modifying agents to confer the best chance of controlling the disease. A significant proportion of these drugs have a risk, albeit small, of potentially serious side effects, such as neutropenia; therefore, there has been an understandable concern over the use of potentially toxic rheumatic drugs in the elderly. Factors that may contribute to this concern include age, pre-existing co-morbidities, polypharmacy, difficulty in monitoring side effects, and patient perception. The risk of using such medication needs to be balanced with their benefits in controlling chronic disease. This review discusses how rheumatic disease and anti-rheumatic medication are associated with neutropenia in an older age group. Of the rheumatic diseases, we give special focus to rheumatoid arthritis and the use of methotrexate, as well as touching on management considerations in neutropenia. PMID:27402172

  8. Technical evaluation of methods for identifying chemotherapy-induced febrile neutropenia in healthcare claims databases

    Directory of Open Access Journals (Sweden)

    Weycker Derek

    2013-02-01

    Full Text Available Abstract Background Healthcare claims databases have been used in several studies to characterize the risk and burden of chemotherapy-induced febrile neutropenia (FN and effectiveness of colony-stimulating factors against FN. The accuracy of methods previously used to identify FN in such databases has not been formally evaluated. Methods Data comprised linked electronic medical records from Geisinger Health System and healthcare claims data from Geisinger Health Plan. Subjects were classified into subgroups based on whether or not they were hospitalized for FN per the presumptive “gold standard” (ANC 9/L, and body temperature ≥38.3°C or receipt of antibiotics and claims-based definition (diagnosis codes for neutropenia, fever, and/or infection. Accuracy was evaluated principally based on positive predictive value (PPV and sensitivity. Results Among 357 study subjects, 82 (23% met the gold standard for hospitalized FN. For the claims-based definition including diagnosis codes for neutropenia plus fever in any position (n=28, PPV was 100% and sensitivity was 34% (95% CI: 24–45. For the definition including neutropenia in the primary position (n=54, PPV was 87% (78–95 and sensitivity was 57% (46–68. For the definition including neutropenia in any position (n=71, PPV was 77% (68–87 and sensitivity was 67% (56–77. Conclusions Patients hospitalized for chemotherapy-induced FN can be identified in healthcare claims databases--with an acceptable level of mis-classification--using diagnosis codes for neutropenia, or neutropenia plus fever.

  9. Ticagrelor as an alternative in clopidogrel-associated neutropenia.

    Science.gov (United States)

    Shah, Rahman; Keough, Leigh Anne; Belalcazar-Portacio, Astrid; Ramanathan, Kodangudi B

    2015-01-01

    Aspirin in combination with platelet P2Y12 receptor blocker has become the mainstay antiplatelet treatment strategy for the prevention of stent thrombosis. Ticlopidine was the first widely used P2Y12 receptor blockers, but clopidogrel has mostly replaced the use of ticlopidine due to its more favorable adverse event profile on bone marrow. However, when clopidogrel induced bone marrow toxicity occurs, little is known about the efficacy and safety of alternative treatments, and thus, in these cases, medical decisions may be very difficult. We report a case of clopidogrel-induced severe neutropenia in a patient treated with coronary stent and safety of alternative treatment with ticagrelor. PMID:24433137

  10. The Value of C-Reactive Protein and Procalcitonin in Febrile Neutropenia

    Directory of Open Access Journals (Sweden)

    Solmaz Çelebi

    2009-06-01

    Full Text Available Aim: Febrile neutropenia is the major cause of mortality and morbidity in cancer patients. For this reason, early diagnosis of severe infections and appropriate antimicrobial therapy are very important. The aim of this study was to investigate the difference between C-reactive protein (CRP and procalcitonin in determining the sepsis and its severity. Materials and Method: A total of 30 children (35 episodes with febrile neutropenia who were hospitalized in the Uludag University, Pediatric Hematology and Oncology Unit were included in this prospective study. The blood samples for CRP and procalcitonin were collected daily between 0 to 5th days. Serum CRP and procalcitonin levels were compared with culture positivity, prolonged fever, mucositis and absolute granulosit count (AGC. Results: A total of 16 patients (56% diagnosed with acute leukemia and, 14 patients (46% having solid tumours were evaluated. In sequential analysis of febrile episodes, both the median of procalcitonin and the CRP concentrations showed the same tendency and there was no significant correlation between them (r=0.2, p>0.05. There was no significant association between CRP and procalcitonin among those having positive culture and mucositis. However, CRP values at the 3rd, 4th and 5th days were significantly higher in the patients with AGC100/mm3. Similarly, CRP values were significantly higher at the 1st, 2nd, 3rd and 4th days among the patients having prolonged fever. Conclusion: Our study suggests that there is no difference between CRP and procalcitonin in determining sepsis and its severity. Although procalcitonin is a valuable acute phase reactant in non-neutropenic patients, larger prospective investigations are needed to show the prognostic value of procalcitonin in neutropenic patients. (Journal of Current Pediatrics 2009; 7: 7-12

  11. Chronic neutropenia. A new canine model induced by human granulocyte colony-stimulating factor.

    OpenAIRE

    Hammond, W. P.; Csiba, E; Canin, A; Hockman, H; Souza, L M; Layton, J E; Dale, D C

    1991-01-01

    Normal dogs were treated with recombinant human granulocyte colony-stimulating factor (rhG-CSF) at 10 micrograms/kg/day for 30 d, which caused an initial neutrophilia, followed by a prolonged period of chronic neutropenia. A control dog treated with recombinant canine G-CSF (rcG-CSF) showed persistent neutrophilia over 3 mo. Serum from dogs during neutropenia contained an antibody to rhG-CSF, which neutralized the stimulatory effects of both rhG-CSF and rcG-CSF on dog marrow neutrophilic prog...

  12. Neutropenia crónica e infección por el virus de la inmunodeficiencia humana Chronic neutropenia and human immunodeficiency virus infection

    Directory of Open Access Journals (Sweden)

    Ronald A Noguera-Valverde

    2008-09-01

    associated with acute human immunodeficiency virus infection. As antiretroviral therapy establishes and viral activity diminishes, hematological disturbances improve. However, some antiretroviral drugs, e.g., zidovudine, exhibits medullary toxicity and it can worsen the hematological findings in these patients, forcing subsequent changes on therapeutic schemes. Cytotoxics used in associated neoplasms have known antimedullary activity. Some antimicrobial agents like trimethoprimsulfamethoxazole, used prophylactically, also exhibit medullary toxicity, because of this, they should be used with caution or should be avoided. Finally, other mechanisms which can cause neutropenia are enlisted: formation of anti- neutrophiles antibodies, primary damage of granulocyte progenitor, imbalance of neutrophil production, antibodies against gp120 viral envelope glycoprotein, or vitamins deficiencies. The approach of the neutropenic febrile patient in whom severe bacterial infection is suspected, includes the use of granulocyte-colonies stimulating factors in order to increase the absolute neutrophil count and to achieve a better clinical outcome.

  13. Risk assessment in fever and neutropenia in children with cancer : What did we learn?

    NARCIS (Netherlands)

    Poele, Esther M. te; Tissing, Wim J. E.; Kamps, Willem A.; de Bont, Eveline S. J. M.

    2009-01-01

    Children with cancer treated with chemotherapy are susceptible to bacterial infections and serious infectious complications. However, fever and neutropenia can also result from other causes, for which no antibiotic treatment is needed. In the past decades attempts have been made to stratify the hete

  14. CLPB Variants Associated with Autosomal-Recessive Mitochondrial Disorder with Cataract, Neutropenia, Epilepsy, and Methylglutaconic Aciduria

    DEFF Research Database (Denmark)

    Saunders, Carol; Smith, Laurie; Wibrand, Flemming;

    2015-01-01

    of type IV 3-MGA-uria characterized by cataracts, severe psychomotor regression during febrile episodes, epilepsy, neutropenia with frequent infections, and death in early childhood. Four of the individuals were of Greenlandic descent, and one was North American, of Northern European and Asian descent...

  15. Interleukin 6 blockage-induced neutropenia in a patient with rheumatoid arthritis and resolved hepatitis B.

    Science.gov (United States)

    Chmielińska, Magdalena; Olesińska, Marzena; Felis-Giemza, Anna

    2015-01-01

    The authors present a case report of a 59-year-old woman with rheumatoid arthritis after documented recovery from hepatitis C (HCV) infection and with resolved HBV infection who has been undergoing successful tocilizumab treatment. The patient experienced moderate to severe neutropenia after consecutive tocilizumab administrations. However, no serious infections or HBV reactivation was recorded during that period.

  16. Critical appraisal of biosimilar filgrastim (Nivestim™ for febrile and chemotherapy-induced neutropenia

    Directory of Open Access Journals (Sweden)

    Waller CF

    2012-08-01

    Full Text Available Cornelius F WallerFreiburg University Medical Center, Department of Hematology/Oncology, Freiburg, GermanyAbstract: Recombinant granulocyte colony-stimulating factor (filgrastim stimulates the proliferation and differentiation of hematopoietic stem and progenitor cells committed to neutrophil and granulocyte lineages. Filgrastim has been used as an adjunct to chemotherapy for ameliorating neutropenia, one of the major side effects of chemotherapy in cancer patients. Its use has led to reduction of infections and hospital admissions for patients with cancer undergoing chemotherapy. In addition, filgrastim has multiple other indications in hematology and oncology. Following the European Union patent expiry of Neupogen® (filgrastim; Amgen Inc in 2006, a biosimilar filgrastim has been developed (Nivestim™; Hospira. In accordance with the requirements of the European Medicines Agency, Nivestim has been studied in a development program that included preclinical studies, two Phase I clinical trials, and one Phase III clinical study. Preclinical studies showed pharmacodynamic as well as pharmacokinetic bioequivalence with the original product, Neupogen. Two randomized, single-center, Phase I trials compared both the pharmacokinetic, pharmacodynamic, and safety profiles of Nivestim and Neupogen in healthy volunteers. In both studies, 90% confidence intervals for the primary endpoints were within the predefined range (0.80–1.25 necessary to demonstrate bioequivalence. Nivestim was well tolerated, with no additional safety concerns over Neupogen. Bioequivalence was demonstrated in a randomized, double-blind multicenter Phase III trial of 279 patients with breast cancer receiving myelosuppressive chemotherapy. The mean duration of severe neutropenia in cycle 1, the primary endpoint, was similar between Nivestim (1.6 days, n = 165 and Neupogen (1.3 days, n = 85, meeting predefined criteria for bioequivalence. Secondary endpoints supporting

  17. A Rare Cause of Retinal Artery Occlusion in Severe Hypernatremic Dehydration in Newborns.

    Science.gov (United States)

    Ozer, Pinar Altiaylik; Kabatas, Emrah Utku; Kurtul, Bengi Ece; Dilli, Dilek; Zenciroglu, Aysegul; Okumus, Nurullah

    2016-05-01

    Neonatal hypernatremia is an important electrolyte disorder that may have serious complications. It may be a rare and underdiagnosed cause of venous and arterial thrombosis, leading to severe brain damage by cerebral edema and intracranial hemorrhage. Here, the authors present a case of bilateral central retinal artery occlusion in a newborn with severe hypernatremic dehydration who is found to be normal in terms of other causes of retinal arterial thromboembolization. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:482-485.]. PMID:27183555

  18. Clinical profile of high-risk febrile neutropenia in a tertiary care hospital

    Directory of Open Access Journals (Sweden)

    Mohan V Bhojaraja

    2016-06-01

    Full Text Available Background Infection in the immunocompromised host has been a reason of concern in the clinical setting and a topic of debate for decades. In this study, the aim was to analyse the clinical profile of high-risk febrile neutropenic patients. Aims To study the clinical profile of high risk febrile neutropenia patients with the objective of identifying the most common associated malignancy, most common associated pathogen, the source of infection, to correlate the treatment and management with that of the Infectious Diseases Society of America (IDSA 2010 guidelines and to assess the clinical outcome. Methods A cross-sectional time bound study was carried out and a total of 80 episodes of high-risk febrile neutropenia were recorded among patients with malignancies from September 2011 to July 2013 with each episode being taken as a new case. Results Non-Hodgkin’s lymphoma (30 per cent was the most common malignancy associated, commonest source of infection was due to central venous catheters, the commonest pathogens were gram negative (52 per cent the treatment and management of each episode of high risk febrile neutropenia correlated with that of IDSA 2010 guidelines and the mortality rate was 13.75 per cent. Conclusion Febrile neutropenia is one of the major complications and cause of mortality in patients with malignancy and hence understanding its entire spectrum can help us reduce morbidity and mortality.

  19. Streptoccocus pyogenes: a forgotten cause of severe community-acquired pneumonia.

    Science.gov (United States)

    Birch, C; Gowardman, J

    2000-02-01

    We report a case of severe community-acquired pneumonia caused by Streptococcus pyogenes (Lancefield Group A streptoccocus) that was complicated by a streptococcal toxic shock syndrome. Although this micro-organism is an uncommon cause of community-acquired pneumonia, previously well individuals may be infected and the clinical course may be fulminant. A household contact was the likely point of infection. Invasive group A streptococcal disease continues to remain an important cause of morbidity and mortality in the community and therefore will continue to be encountered by intensive care physicians. Treatment of Group A streptococcal infection remains penicillin; however, clindamycin should be added in severe infection. PMID:10701045

  20. Efficacy of Adenine in the Treatment of Leukopenia and Neutropenia Associated with an Overdose of Antipsychotics or Discontinuation of Lithium Carbonate Administration: Three Case Studies

    Science.gov (United States)

    Tomita, Takashi; Goto, Hidekazu; Sumiya, Kenji; Yoshida, Tadashi; Tanaka, Katsuya; Kohda, Yukinao

    2016-01-01

    Because adenine is effective for managing cases of radiation-induced and drug-induced leukopenia, it may be effective in cases of antipsychotic-induced leukopenia and neutropenia. Here, we report our experience with patients with leukopenia and neutropenia caused by an antipsychotic overdose or discontinuation of lithium carbonate, in whom adenine administration ameliorated the white blood cell and neutrophil counts. The progress of patients suggests that adenine is effective in cases of leukopenia and neutropenia associated with lithium carbonate discontinuation and an antipsychotic overdose. PMID:27776394

  1. A rare cause for severe recurrent lower gastrointestinal bleeding in a 12 year old patient

    Directory of Open Access Journals (Sweden)

    D. Belsha

    2015-09-01

    Full Text Available The cause for severe, recurrent lower gastrointestinal (LGI bleeding in children can usually be diagnosed readily by means of the commonly used investigative/diagnostic techniques such as colonoscopy, laparoscopy ± laparotomy. Occasionally less commonly used investigations may be necessary to look for more elusive causes of LGI bleeding such as capsule endoscopy, angiography, technetium-99m (99m Tc-labeled red blood cell (RBC scintigraphy, cross-sectional imaging such as CT/MRI (including angiography and laparotomy combined with on-table small bowel enteroscopy. We report a case of severe, recurrent LGI bleeding that had occurred over several years, where the cause remained elusive despite numerous investigations and interventions. The etiology of this was eventually found to be a gastric duplication cyst infiltrating into adjacent transverse colon and causing bleeding from peptic ulceration in the colon. The process by which this diagnosis was made and the lessons learned are discussed.

  2. Do Low Molecular Weight Agents Cause More Severe Asthma than High Molecular Weight Agents?

    Science.gov (United States)

    Meca, Olga; Cruz, María-Jesús; Sánchez-Ortiz, Mónica; González-Barcala, Francisco-Javier; Ojanguren, Iñigo; Munoz, Xavier

    2016-01-01

    Introduction The aim of this study was to analyse whether patients with occupational asthma (OA) caused by low molecular weight (LMW) agents differed from patients with OA caused by high molecular weight (HMW) with regard to risk factors, asthma presentation and severity, and response to various diagnostic tests. Methods Seventy-eight patients with OA diagnosed by positive specific inhalation challenge (SIC) were included. Anthropometric characteristics, atopic status, occupation, latency periods, asthma severity according to the Global Initiative for Asthma (GINA) control classification, lung function tests and SIC results were analysed. Results OA was induced by an HMW agent in 23 patients (29%) and by an LMW agent in 55 (71%). A logistic regression analysis confirmed that patients with OA caused by LMW agents had a significantly higher risk of severity according to the GINA classification after adjusting for potential confounders (OR = 3.579, 95% CI 1.136–11.280; p = 0.029). During the SIC, most patients with OA caused by HMW agents presented an early reaction (82%), while in patients with OA caused by LMW agents the response was mainly late (73%) (p = 0.0001). Similarly, patients with OA caused by LMW agents experienced a greater degree of bronchial hyperresponsiveness, measured as the difference in the methacholine dose-response ratio (DRR) before and after SIC (1.77, range 0–16), compared with patients with OA caused by HMW agents (0.87, range 0–72), (p = 0.024). Conclusions OA caused by LMW agents may be more severe than that caused by HMW agents. The severity of the condition may be determined by the different mechanisms of action of these agents. PMID:27280473

  3. New developments in the treatment of chemotherapy-induced neutropenia: focus on balugrastim.

    Science.gov (United States)

    Ghidini, Michele; Hahne, Jens Claus; Trevisani, Francesco; Panni, Stefano; Ratti, Margherita; Toppo, Laura; Tomasello, Gianluca

    2016-01-01

    Neutropenia and febrile neutropenia are two major complications of chemotherapy. Dose reductions, delays in treatment administration, and the use of granulocyte colony-stimulating factors are equally recommended options to preserve absolute neutrophil count in case of chemotherapy regimens bringing a risk of febrile neutropenia of 20% or higher. Recombinant granulocyte colony-stimulating factors, such as filgrastim and lenograstim, have a short elimination half-life (t1/2) and need to be used daily, while others, like pegfilgrastim and lipegfilgrastim, are characterized by a long t1/2 requiring only a single administration per cycle. Balugrastim is a novel long-acting recombinant granulocyte colony-stimulating factor obtained by means of a genetic fusion between recombinant human serum albumin and granulocyte colony-stimulating factor. Albumin binding increases the molecular weight and determines a high plasmatic stability leading to a t1/2 of ~19 days. Balugrastim's efficacy, safety, and tolerability have been assessed in four different clinical trials involving breast cancer patients treated with doxorubicin and docetaxel. Pegfilgrastim was chosen as a comparator. Balugrastim was noninferior to pegfilgrastim with regard to the reduction of mean duration of severe neutropenia during cycle 1. Moreover, both treatments were comparable in terms of efficacy and safety profile. Balugrastim was well tolerated, with the only related adverse event being mild to moderate bone pain. The aim of this review is to summarize the currently available literature data on balugrastim. PMID:27445479

  4. Rationalizing the approach to children with fever in neutropenia

    NARCIS (Netherlands)

    Ammann, Roland A.; Tissing, Wim J. E.; Phillips, Bob

    2012-01-01

    Purpose of review Fever in neutropenia is the most frequent potentially life-threatening complication of chemotherapy in children and adolescents with cancer. This review summarizes recent studies that refine our knowledge of how to manage pediatric fever in neutropenia, and their implications for c

  5. Rhabdomyolysis due to Severe Hypernatremia Caused by Dehydration, in a Child with Gastroenteritis: a Case Report

    Directory of Open Access Journals (Sweden)

    Mitra Basiratnia

    2016-06-01

    Full Text Available Background Rhabdomyolysis is considered a rare medical condition in pediatric population. Case Report We report our experience on a one year old girl referred to Shiraz Nemazee Hospital, Southern Iran with rhabdomyolysis due to severe hypernatremia, secondary to gastroenteritis. Discussion Rhabdomyolysis should be taken in to consideration in hypernatremic states, as it may lead to severe consequences. Treatment of underlying cause and proper management of hypernatremia could be helpful while handling this complicated situation.

  6. Rhabdomyolysis due to Severe Hypernatremia Caused by Dehydration, in a Child with Gastroenteritis: a Case Report

    OpenAIRE

    Mitra Basiratnia; Yasna Pouralborz; Forough Saki

    2016-01-01

    Background Rhabdomyolysis is considered a rare medical condition in pediatric population. Case Report We report our experience on a one year old girl referred to Shiraz Nemazee Hospital, Southern Iran with rhabdomyolysis due to severe hypernatremia, secondary to gastroenteritis. Discussion Rhabdomyolysis should be taken in to consideration in hypernatremic states, as it may lead to severe consequences. Treatment of underlying cause and proper management of hypernatremia could be helpful while...

  7. SEVERE MALARIA CAUSED BY PLASMODIUM VIVAX IN PREGNANCY MASQUERADING AS HELLP SYNDROME

    Directory of Open Access Journals (Sweden)

    Monika

    2014-01-01

    Full Text Available The following is a case report of a woman who presented to the hospital with severe anemia with features of HELLP syndrome and initial slide for P .vivax negative but on constan t observation and monitoring turned out to be a case of severe malaria caused by vivax species of malaria. Primigravida with 35week5day pregnancy was admitted with complaints of severe anemia and history of fever 14 days back. Init i ally 3 pint blood was transfused i/v/o severe anemia . Investigations revealed Hb - 3gm% and platelet count 30 , 000 , LFT deranged , Hemolysis in peripheral smear , smear negative for malarial parasite. HELLP syndrome was in mind and this required termination but on repeat investigations and smear vivax was positive . Her platelet count kept deteriorating despite t ransfusions till antimalarial regimen was not started. Once smear positive the fastest antimalarial artesunate regimen with clindamycin was started and after 48 hrs. her counts improved. Overall 14 pints of platelets were transfused and patient delivered u neventfully with mild PPH at platelet count of 30 , 000. Severe malaria is usually caused by p falciparum but recently reports of severe malaria caused by vivax is increasing and HELLP syndrome is an important differential diagnosis which needs to be exclud ed as management is entirely different.

  8. SEVERE MALARIA CAUSED BY PLASMODIUM VIVAX IN PREGNANCY MASQUERADING AS HELLP SYNDROME

    OpenAIRE

    Monika; Premila; Geetika; Pranjal

    2014-01-01

    The following is a case report of a woman who presented to the hospital with severe anemia with features of HELLP syndrome and initial slide for P .vivax negative but on constan t observation and monitoring turned out to be a case of severe malaria caused by vivax species of malaria. Primigravida with 35week5day pregnancy was admitted with complaints of severe anemia and history of fever 14 days back. Init i ally 3 pint blood was transfused i/v/o s...

  9. Risk assessment on severe hazards to China caused by West Nile virus

    Institute of Scientific and Technical Information of China (English)

    CHEN Jiming; SUN Yingxue; WANG Zhiliang; SHEN Chaojian; XIE Zhonglun; WANG Ximing

    2004-01-01

    Recent West Nile virus epidemics in USA draw worldwide concerns. Here the basic biological and epidemiologicai features of the virus are analyzed along with the special immunity of humans and animals, the immigration of birds and the natural environments in China. The risk of severe hazards to China caused by West Nile virus is assessed not high thereafter.

  10. Unique clone of Coxiella burnetii causing severe Q fever, French Guiana.

    Science.gov (United States)

    Mahamat, Aba; Edouard, Sophie; Demar, Magalie; Abboud, Philippe; Patrice, Jean-Yves; La Scola, Bernard; Okandze, Antoine; Djossou, Félix; Raoult, Didier

    2013-07-01

    Acute Q fever is an emergent and severe disease in French Guiana. We obtained 5 Coxiella burnetii isolates from samples of patients from Cayenne and found an epidemic clone circulating in Cayenne. This clone has caused pneumonia and endocarditis and seems to be more virulent than previously described strains.

  11. Severe hemolysis caused by graft-derived anti-B production after lung transplantation

    DEFF Research Database (Denmark)

    Taaning, E; Morling, N; Mortensen, S A;

    1996-01-01

    Anti-B antibody causing sever hemolytic anemia and renal failure was found in the serum of a blood group B patient who had received a bilateral lung transplant from a blood group O donor. Although the donor origin of the antibody was not confirmed, it is likely that the anti-B antibody was produced...

  12. Four-Rod Stabilization of Severely Destabilized Lumbar Spine Caused by Metastatic Tumor

    Directory of Open Access Journals (Sweden)

    Isao Shibuya

    2013-01-01

    Full Text Available We report a case of a 67-year-old female with severely destabilized lumbar spine caused by metastatic malignant tumor. The primary lesion was a thyroid follicular adenocarcinoma. Complete destruction of the L3, L4, and L5 vertebrae had resulted in severe instability, which left the patient with severe back pain and bed-ridden. Since the vertebrae were so severely damaged at 3 levels, 4 rods were used to stabilize the spine. Following stabilization, the pain was alleviated and the patient’s quality of life improved. We introduce here the 4-rod technique to stabilize the spine over 3 vertebral levels following severe destruction by metastatic tumor.

  13. Medical visits for chemotherapy and chemotherapy-induced neutropenia: a survey of the impact on patient time and activities

    Directory of Open Access Journals (Sweden)

    Moore Kelley

    2004-05-01

    Full Text Available Abstract Background Patients with cancer must make frequent visits to the clinic not only for chemotherapy but also for the management of treatment-related adverse effects. Neutropenia, the most common dose-limiting toxicity of myelosuppressive chemotherapy, has substantial clinical and economic consequences. Colony-stimulating factors such as filgrastim and pegfilgrastim can reduce the incidence of neutropenia, but the clinic visits for these treatments can disrupt patients' routines and activities. Methods We surveyed patients to assess how clinic visits for treatment with chemotherapy and the management of neutropenia affect their time and activities. Results The mean amounts of time affected by these visits ranged from approximately 109 hours (hospitalization for neutropenia and 8 hours (physician and chemotherapy to less than 3 hours (laboratory and treatment with filgrastim or pegfilgrastim. The visits for filgrastim or pegfilgrastim were comparable in length, but treatment with filgrastim requires several visits per chemotherapy cycle and treatment with pegfilgrastim requires only 1 visit. Conclusions This study provides useful information for future modelling of additional factors such as disease status and chemotherapy schedule and provides information that should be considered in managing chemotherapy-induced neutropenia.

  14. Severe Cenani-Lenz syndrome caused by loss of LRP4 function.

    Science.gov (United States)

    Kariminejad, Ariana; Stollfuß, Barbara; Li, Yun; Bögershausen, Nina; Boss, Karin; Hennekam, Raoul C M; Wollnik, Bernd

    2013-06-01

    Limb patterning and growth are complex embryonic processes in which the elaborately orchestrated interplay of diverse endocrine and paracrine factors is crucial to limb integrity. LRP4 is a lipoprotein receptor known for its regulatory effects on LRP5- and LRP6-mediated Wnt signaling, a pathway that plays a pivotal role in limb development. Recessive mutations in LRP4 have been shown to cause Cenani-Lenz syndrome, which is characterized by severe limb malformations, an unusual face, and renal abnormalities. We report on a child with severe Cenani-Lenz syndrome caused by a novel homozygous nonsense mutation in LRP4. The severity of the phenotype in a patient with absent residual LRP4 function may point to a genotype-phenotype correlation.

  15. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

    Directory of Open Access Journals (Sweden)

    An-Shine Chao

    2009-01-01

    Full Text Available We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebral artery was 0.8 m/s, indicating severe fetal anemia. Multiple intrauterine transfusions for the anemic fetus were administered. However, persistent severe fetal anemia and placentomegaly caused poor neonatal death and mirror syndrome in the mother. Uncommon red blood cell alloimmunization has to be watched for early in any population, especially in a woman with a history of unexplained perinatal loss.

  16. Third generation cephalosporin resistant Enterobacteriaceae and multidrug resistant gram-negative bacteria causing bacteremia in febrile neutropenia adult cancer patients in Lebanon, broad spectrum antibiotics use as a major risk factor, and correlation with poor prognosis

    Directory of Open Access Journals (Sweden)

    Rima eMoghnieh

    2015-02-01

    Full Text Available Bacteremia remains a major cause of life-threatening complications in patients receiving anticancer chemotherapy. The spectrum and susceptibility profiles of causative microorganisms differ with time and place. Data from Lebanon are scarce. We aim at evaluating the epidemiology of bacteremia in cancer patients in a university hospital in Lebanon, emphasizing antibiotic resistance and risk factors of multi-drug resistant organism (MDRO-associated bacteremia.This is a retrospective study of 75 episodes of bacteremia occurring in febrile neutropenic patients admitted to the hematology-oncology unit at Makassed General Hospital, Lebanon, from October 2009-January 2012.It corresponds to epidemiological data on bacteremia episodes in febrile neutropenic cancer patients including antimicrobial resistance and identification of risk factors associated with third generation cephalosporin resistance (3GCR and MDRO-associated bacteremia. Out of 75 bacteremias, 42.7% were gram-positive (GP, and 57.3% were gram-negative (GN. GP bacteremias were mostly due to methicillin-resistant coagulase negative staphylococci (28% of total bacteremias and 66% of GP bacteremias. Among the GN bacteremias, Escherichia coli (22.7% of total, 39.5% of GN organisms and Klebsiellapneumoniae(13.3% of total, 23.3% of GN organisms were the most important causative agents. GN bacteremia due to 3GC sensitive (3GCS bacteria represented 28% of total bacteremias, while 29% were due to 3GCR bacteria and 9% were due to carbapenem-resistant organisms. There was a significant correlation between bacteremia with MDRO and subsequent intubation, sepsis and mortality. Among potential risk factors, only broad spectrum antibiotic intake >4 days before bacteremia was found to be statistically significant for acquisition of 3GCR bacteria. Using carbapenems or piperacillin/ tazobactam>4 days before bacteremia was significantly associated with the emergence of MDRO (p value<0.05.

  17. Traumatic retropharyngeal emphysema as a cause for severe respiratory distress in a newborn

    International Nuclear Information System (INIS)

    Traumatic injury to the pharynx or esophagus in a newborn from intubation or tube suctioning may have various presentations. Difficulty passing a gastric tube or feeding problems may erroneously suggest the diagnosis of esophageal atresia. Associated respiratory distress may be caused by pneumothorax or pleural effusion if the pleural space is entered. We report the case of a full-term newborn presenting with severe respiratory distress caused by a large retropharyngeal air collection resulting from hypopharyngeal perforation from prior intubation and suctioning. Chest abnormality, sufficient to account for the degree of respiratory distress, was not demonstrated. (orig.)

  18. Traumatic retropharyngeal emphysema as a cause for severe respiratory distress in a newborn

    Energy Technology Data Exchange (ETDEWEB)

    Barlev, Dan M. [Division of Pediatric Radiology, Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, New York (United States); Division of Pediatric Radiology, Schneider Children' s Hospital, Long Island Jewish Medical Center, Albert Einstein College of Medicine, 270-05 76th Avenue, New Hyde Park, NY 11040 (United States); Nagourney, Beth A.; Saintonge, Ronald [Division of Neonatology, Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, New York (United States)

    2003-06-01

    Traumatic injury to the pharynx or esophagus in a newborn from intubation or tube suctioning may have various presentations. Difficulty passing a gastric tube or feeding problems may erroneously suggest the diagnosis of esophageal atresia. Associated respiratory distress may be caused by pneumothorax or pleural effusion if the pleural space is entered. We report the case of a full-term newborn presenting with severe respiratory distress caused by a large retropharyngeal air collection resulting from hypopharyngeal perforation from prior intubation and suctioning. Chest abnormality, sufficient to account for the degree of respiratory distress, was not demonstrated. (orig.)

  19. Use of FDG PET/CT for investigation of febrile neutropenia: evaluation in high-risk cancer patients

    International Nuclear Information System (INIS)

    Febrile neutropenia (FNP) is a frequent complication of cancer care and evaluation often fails to identify a cause. [18 F]FDG PET/CT has the potential to identify inflammatory and infectious foci, but its potential role as an investigation for persistent FNP has not previously been explored. The aim of this study was to prospectively evaluate the clinical utility of FDG PET/CT in patients with cancer and severe neutropenia and five or more days of persistent fever despite antibiotic therapy. Adult patients with a diagnosis of an underlying malignancy and persistent FNP (temperature ≥38 C and neutrophil count <500 cells/μl for 5 days) underwent FDG PET/CT as an adjunct to conventional evaluation and management. The study group comprised 20 patients with FNP who fulfilled the eligibility criteria and underwent FDG PET/CT in addition to conventional evaluation. The median neutrophil count on the day of the FDG PET/CT scan was 30 cells/μl (range 0-730 cells/μl). Conventional evaluation identified 14 distinct sites of infection, 13 (93 %) of which were also identified by FDG PET/CT, including all deep tissue infections. FDG PET/CT identified 9 additional likely infection sites, 8 of which were subsequently confirmed as ''true positives'' by further investigations. FDG PET/CT was deemed to be of 'high' clinical impact in 15 of the 20 patients (75 %). This study supports the utility of FDG PET/CT scanning in severely neutropenic patients with five or more days of fever. Further evaluation of the contribution of FDG PET/CT in the management of FNP across a range of underlying malignancies is required. (orig.)

  20. Use of FDG PET/CT for investigation of febrile neutropenia: evaluation in high-risk cancer patients

    Energy Technology Data Exchange (ETDEWEB)

    Guy, Stephen D.; Tramontana, Adrian R. [Western Health, Department of Infectious Diseases, Private Bag, Footscray, Victoria (Australia); University of Melbourne, Parkville, Victoria (Australia); Worth, Leon J.; Thursky, Karin A.; Slavin, Monica A. [University of Melbourne, Parkville, Victoria (Australia); Peter MacCallum Cancer Centre, Department of Infectious Diseases, Melbourne, Victoria (Australia); Lau, Eddie; Hicks, Rodney J. [University of Melbourne, Parkville, Victoria (Australia); Peter MacCallum Cancer Centre, Centre for Cancer Imaging, Melbourne, Victoria (Australia); Seymour, John F. [University of Melbourne, Parkville, Victoria (Australia); Peter MacCallum Cancer Centre, Department of Haematology, Melbourne, Victoria (Australia)

    2012-08-15

    Febrile neutropenia (FNP) is a frequent complication of cancer care and evaluation often fails to identify a cause. [{sup 18} F]FDG PET/CT has the potential to identify inflammatory and infectious foci, but its potential role as an investigation for persistent FNP has not previously been explored. The aim of this study was to prospectively evaluate the clinical utility of FDG PET/CT in patients with cancer and severe neutropenia and five or more days of persistent fever despite antibiotic therapy. Adult patients with a diagnosis of an underlying malignancy and persistent FNP (temperature {>=}38 C and neutrophil count <500 cells/{mu}l for 5 days) underwent FDG PET/CT as an adjunct to conventional evaluation and management. The study group comprised 20 patients with FNP who fulfilled the eligibility criteria and underwent FDG PET/CT in addition to conventional evaluation. The median neutrophil count on the day of the FDG PET/CT scan was 30 cells/{mu}l (range 0-730 cells/{mu}l). Conventional evaluation identified 14 distinct sites of infection, 13 (93 %) of which were also identified by FDG PET/CT, including all deep tissue infections. FDG PET/CT identified 9 additional likely infection sites, 8 of which were subsequently confirmed as ''true positives'' by further investigations. FDG PET/CT was deemed to be of 'high' clinical impact in 15 of the 20 patients (75 %). This study supports the utility of FDG PET/CT scanning in severely neutropenic patients with five or more days of fever. Further evaluation of the contribution of FDG PET/CT in the management of FNP across a range of underlying malignancies is required. (orig.)

  1. Causes and anatomical site of blindness and severe visual loss in Isfahan, Islamic Republic of Iran.

    Science.gov (United States)

    Dehghan, A; Kianersi, F; Moazam, E; Ghanbari, H

    2010-02-01

    This study in 2005 evaluated the causes and major anatomical site of blindness and severe visual loss at a school for blind children in Isfahan province, Islamic Republic of Iran. All 211 students were examined according to the modified WHO/PBL eye examination record: 70.4% were blind, 24.3% had severe visual loss and 5.3% were visually impaired. The major causes of abnormality were hereditary factors (42.7%), prenatal/neonatal (18.5%) and unknown etiology (35.5%). The main sites of abnormality were the retina (62.6%), whole globe (17.5%), lens (7.1%) and optic nerve (7.1%). A high proportion of parents were in a consanguineous marriage (49.2%). The pattern of blindness in Isfahan encompasses characteristics of both developed and developing countries. PMID:20799580

  2. A case of odontogenic orbital cellulitis causing blindness by severe tension orbit.

    Science.gov (United States)

    Park, Chang Hyun; Jee, Dong Hyun; La, Tae Yoon

    2013-02-01

    We report a very rare case of odontogenic orbital cellulitis causing blindness by severe tension orbit. A 41-yr old male patient had visited the hospital due to severe periorbital swelling and nasal stuffiness while he was treated for a periodontal abscess. He was diagnosed with odontogenic sinusitis and orbital cellulitis, and treated with antibiotics. The symptoms were aggravated and emergency sinus drainage was performed. On the next day, a sudden decrease in vision occurred with findings of ischemic optic neuropathy and central retinal artery occlusion. Deformation of the eyeball posterior pole into a cone shape was found from the orbital CT. A high-dose steroid was administered immediately resulting in improvements of periorbital swelling, but the patient's vision had not recovered. Odontogenic orbital cellulitis is relatively rare, but can cause blindness via rapidly progressing tension orbit. Therefore even the simplest of dental problems requires careful attention.

  3. Root Causes and Impacts of Severe Accidents at Large Nuclear Power Plants

    OpenAIRE

    Högberg, Lars

    2013-01-01

    The root causes and impacts of three severe accidents at large civilian nuclear power plants are reviewed: the Three Mile Island accident in 1979, the Chernobyl accident in 1986, and the Fukushima Daiichi accident in 2011. Impacts include health effects, evacuation of contaminated areas as well as cost estimates and impacts on energy policies and nuclear safety work in various countries. It is concluded that essential objectives for reactor safety work must be: (1) to prevent accidents from d...

  4. Lingual tonsil hypertrophy causing severe dysphagia: treatment with plasma-mediated radiofrequency-based ablation (Coblation).

    Science.gov (United States)

    Mowry, Sarah E; Ament, Marvin; Shapiro, Nina L

    2010-03-01

    Lingual tonsil hypertrophy is an uncommon cause of upper aerodigestive tract pathology. We present the case of a 17-year-old boy who developed severe dysphagia and subsequent weight loss as a result of lingual tonsil hypertrophy. He was successfully treated with plasma-mediated radiofrequency-based ablation (Coblation). In the past, traditional surgical procedures for lingual tonsil hypertrophy were difficult to perform and recovery was difficult, but the introduction of Coblation has made lingual tonsillectomy much easier.

  5. Comparing the Pain Severity Caused by Muscular Injection of Tramadole in Z and Bulb Methods

    OpenAIRE

    SH Najafi Doulatabad; Z Mohebi Nobandegani; J Malekzadeh

    2008-01-01

    ABSTRACT: Introduction & Objective: Pain, rather than any other problems, would persuade people to pursue remedy and treatment. Muscular injections are among pain producing factors. This technique of prescription can be followed by some complications from which pain is the most prevalent one. This study was performed to compare the pain severity caused by muscular injection of Tramadole in Z and Bulb methods. Materials & Methods: This is a clinical trial study in which 90 women who re...

  6. Kachchh (India) earthquake 2001--causes, severity and impact on groundwater resources

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Earthquake 2001 in Bhuj region of Kachchh district of Gujarat(India) was one of the most devastating earthquakes in the Indianhistory. This earthquake has caused severe damage to human life and properties. The impact of earthquake on groundwater resources at manylocations was significant. Steep rise in static water level due to earthquake was observed at Bhachau and Chandarani. At other locationgroundwater followed the declining trend.

  7. Anti-E Alloimmunization: A Rare Cause of Severe Fetal Hemolytic Disease Resulting in Pregnancy Loss

    OpenAIRE

    Reyin Lien; Yao-Lung Chang; Szu Ying Ho; Angel Chao; An-Shine Chao

    2010-01-01

    We report a case of severe intrauterine hemolysis caused by sole anti-E alloimmunization. A 36-year-old multipara woman presented with hydrops fetalis at 27 weeks of gestation. She had a history of previous neonatal death. In this pregnancy, she was found to have very high titer of anti-E antibody. Ultrasonography detected marked skin edema, cardiomegaly, hepatosplenomegaly, pleural effusion, ascites, placentomegaly, and polyhydramnios. The Doppler peak systolic velocity in the middle cerebra...

  8. Characterisation of the Cullin-3 mutation that causes a severe form of familial hypertension and hyperkalaemia.

    Science.gov (United States)

    Schumacher, Frances-Rose; Siew, Keith; Zhang, Jinwei; Johnson, Clare; Wood, Nicola; Cleary, Sarah E; Al Maskari, Raya S; Ferryman, James T; Hardege, Iris; Yasmin; Figg, Nichola L; Enchev, Radoslav; Knebel, Axel; O'Shaughnessy, Kevin M; Kurz, Thimo

    2015-10-01

    Deletion of exon 9 from Cullin-3 (CUL3, residues 403-459: CUL3(Δ403-459)) causes pseudohypoaldosteronism type IIE (PHA2E), a severe form of familial hyperkalaemia and hypertension (FHHt). CUL3 binds the RING protein RBX1 and various substrate adaptors to form Cullin-RING-ubiquitin-ligase complexes. Bound to KLHL3, CUL3-RBX1 ubiquitylates WNK kinases, promoting their ubiquitin-mediated proteasomal degradation. Since WNK kinases activate Na/Cl co-transporters to promote salt retention, CUL3 regulates blood pressure. Mutations in both KLHL3 and WNK kinases cause PHA2 by disrupting Cullin-RING-ligase formation. We report here that the PHA2E mutant, CUL3(Δ403-459), is severely compromised in its ability to ubiquitylate WNKs, possibly due to altered structural flexibility. Instead, CUL3(Δ403-459) auto-ubiquitylates and loses interaction with two important Cullin regulators: the COP9-signalosome and CAND1. A novel knock-in mouse model of CUL3(WT) (/Δ403-459) closely recapitulates the human PHA2E phenotype. These mice also show changes in the arterial pulse waveform, suggesting a vascular contribution to their hypertension not reported in previous FHHt models. These findings may explain the severity of the FHHt phenotype caused by CUL3 mutations compared to those reported in KLHL3 or WNK kinases. PMID:26286618

  9. Sepsis Caused by Achromobacter Xylosoxidans in a Child with Cystic Fibrosis and Severe Lung Disease

    Science.gov (United States)

    Stobbelaar, Kim; Van Hoorenbeeck, Kim; Lequesne, Monique; De Dooy, Jozef; Ho, Erwin; Vlieghe, Erika; Ieven, Margaretha; Verhulst, Stijn

    2016-01-01

    Patient: Female, 10 Final Diagnosis: Sepsis Symptoms: Fever • hypotension • not tollerating enteral feeds • respiratory deterioration Medication: — Clinical Procedure: IV antibiotics • lungtransplantion Specialty: Pediatrics and Neonatology Objective: Unusual clinical course Background: Achromobacter xylosoxidans is an aerobic, motile, Gram-negative, opportunistic pathogen that can be responsible for various severe nosocomial and community-acquired infections. It has been found in immunocompromised patients and patients with several other underlying conditions, but the clinical role of this microorganism in cystic fibrosis is unclear. Case Report: We describe a case of septic shock caused by A. xylosoxidans in a 10-year-old child with cystic fibrosis and severe lung disease. Conclusions: As the prevalence of A. xylosoxidans in cystic fibrosis patients is rising and patient-to-patient transmission is highly probable, further studies are warranted to determine its role and to document the appropriate treatment strategy for eradication and long-term treatment of this organism. PMID:27498677

  10. Sepsis Caused by Achromobacter Xylosoxidans in a Child with Cystic Fibrosis and Severe Lung Disease.

    Science.gov (United States)

    Stobbelaar, Kim; Van Hoorenbeeck, Kim; Lequesne, Monique; De Dooy, Jozef; Ho, Erwin; Vlieghe, Erika; Ieven, Margaretha; Verhulst, Stijn

    2016-01-01

    BACKGROUND Achromobacter xylosoxidans is an aerobic, motile, Gram-negative, opportunistic pathogen that can be responsible for various severe nosocomial and community-acquired infections. It has been found in immunocompromised patients and patients with several other underlying conditions, but the clinical role of this microorganism in cystic fibrosis is unclear. CASE REPORT We describe a case of septic shock caused by A. xylosoxidans in a 10-year-old child with cystic fibrosis and severe lung disease. CONCLUSIONS As the prevalence of A. xylosoxidans in cystic fibrosis patients is rising and patient-to-patient transmission is highly probable, further studies are warranted to determine its role and to document the appropriate treatment strategy for eradication and long-term treatment of this organism. PMID:27498677

  11. A retrspective study of rescuing severe open craniocerebral injuries caused by traffic accidents

    Institute of Scientific and Technical Information of China (English)

    陈长才; 宁可; 等

    1999-01-01

    Objective:To investigate the rescuing principles of severe open craniocerebral injuries caused by traffic accidents.Methods:A retrospective study was performed for 36 patients admitted to our hospital from January 1986 to December 1995,who suffered from severe open craniocerebral injuries in traffic accidents.Results:These 36 cases occupied 52.10% of all the severe open craniocerebral injuries during the same period.The clinical features included confusion of consciousness, extensive cerebral contusion and laceration,severe contamination of the wound,high incidence of intracranial hematoma and multiple system injuries.Nineteen patients.(63.34%)ecovered normal neurological function,7 were (23.33%)mild disabled,4(13.33%)severe disabled,2(5.56%) vegetative survival,and 4(11.11%)dead.Conclusions:The main principles of salvage should emphasize the importance of emergent prehospital rescue,and be transfered to a specialized hospital as soon as possible.Postoperative complications included severe brain edema,intracerebral infection,and pneumonia,Debriding thoroughly at early stage and treating complications effectively would lower the rate of mortality and disability.

  12. Concomitant Use of Topiramate Inducing Neutropenia in a Schizophrenic Male Stabilized on Clozapine

    Directory of Open Access Journals (Sweden)

    Pravesh Sharma

    2016-01-01

    Full Text Available This is a case of a 23-year-old African American male with a history of paranoid schizophrenia that developed neutropenia on a clozapine-topiramate therapy. Clozapine had well addressed the patient’s psychotic symptoms, while topiramate was used as a weight-lowering agent. The patient had fairly stable leukocyte counts for eight months on clozapine 300 mg and topiramate 100 mg daily. Doubling the dosage of topiramate led to severe neutropenia after two months. Reviewing the patient’s laboratory reports showed a gradual decline of neutrophils occurring at a lower dosage, followed by a rapid decline after an increased dosage. In this case, we report that not only did topiramate act as the neutropenic agent, but also it might have done so in a dose-dependent manner.

  13. Clopidogrel-Induced Neutropenia after Coronary Stenting: Is Cilostazol a Good Alternative?

    Directory of Open Access Journals (Sweden)

    Massimo Montalto

    2011-01-01

    Full Text Available Dual antiplatelet therapy with aspirin plus thienopyridines has become the standard treatment of patients undergoing coronary stenting. Clopidogrel has mostly replaced the use of ticlopidine due to its more favourable adverse event profile. However, also the use of clopidogrel is not without side effects. Clopidogrel major adverse events are represented by marrow suppression, manifesting with aplastic anaemia, thrombocytopenia and neutropenia. When clopidogrel toxicity occurs, there are few and unsubstantiated alternative treatments and thus, in these cases, medical decisions may be very difficult. We report a case of clopidogrel-induced bone marrow toxicity manifesting with severe neutropenia in a patient treated with multiple coronary stents and provide suggestions for an alternative treatment.

  14. Two Cases of Severe Combined Immunodeficiency Caused By Adenosine Deaminase Deficiency

    Directory of Open Access Journals (Sweden)

    Turkan Patiroglu

    2014-08-01

    Full Text Available Severe Combined Immune Deficiency (SCID is a primary immune deficiency disorder manifested with severe infections upon first months of life, which is characterized by diverse genetic defects in T and B lymphocyte functions and occasionally in NK cells. ADA deficiency is a form of SCID progressing with severe lymphopenia and immune deficiency caused by toxic metabolites of ADA. Bone marrow transplantation (BMT is the only curative treatment although prophylactic anti-microbial therapy, intravenous immunoglobulin (IVIG and enzyme replacement can achieve transient improvements. Early diagnosis before development of severe infections and organ injury and referral to pediatric immunology clinics will make considerable contributions to prognosis. Here, we presented 2 cousins with SCID who had positive family history with deceased sibling; presented with tanning at skin, severe neonatal infections and Q246X (c736C>T non-sense mutation in exon 8 in ADA gene  in order to emphasize this rare mutation and pediatric emergencies associated with this disorder.

  15. Severe Hydronephrosis Caused by Uterine Prolapse with Non Specific Symptoms and Normal Renal Function

    Directory of Open Access Journals (Sweden)

    Muammer Altok

    2013-09-01

    Full Text Available Pelvic organ prolapse (POP, is the descent of pelvic organs (bladder, uterus, rectum until protrusion through the vagina and related to the loss of normal attachment and support of the pelvic floor. Pelvic organ prolapse (POP is a common problem in elderly women and usually causes various urologic symptoms. One of the most important complications of POP is hydronephrosis. The hydronephrosis is usually mild. Severe hydronephrosis is very rare. Hydronephrosis caused by POP may lead to renal dysfunction and serious urinary infections. Early detection and treatment is very important for preventing irreversible renal damage.Hereby, we report a case of 65-years-old woman who had massive uterine prolapse with silent severe bilateral hydronephrosis. She had no complaint except a protruding tissue out of her vagina. Renal function was normal and hydronephrosis was detected incidentally. The symptoms may be non-specific and silent until a serious infection or renal dysfunction. Therefore patients with a POP especially with severe POP need to have evaluation and imaging about the condition or the urinary system before a serious pathology occur

  16. Tratamiento ambulatorio del paciente con neutropenia febril Outpatient therapy in patients with febrile neutropenia

    Directory of Open Access Journals (Sweden)

    Andrés Londoño Gallo

    2008-01-01

    Full Text Available

    El tratamiento de los pacientes con neoplasia y neutropenia febril plantea muchas dudas. Una de ellas, que genera ansiedad en el personal de la salud, el paciente y sus familiares, es la necesidad de hospitalización porque ésta implica exponer a gérmenes intrahospitalarios potencialmente resistentes a un paciente cuyo sistema inmune puede no estar en las mejores condiciones; incluso con un aislamiento óptimo existe el riesgo de adquirir una infección nosocomial. Muchos estudios han tratado de validar métodos para clasificar a los pacientes con fiebre y neutropenia en grupos de diferente riesgo, como fundamento para implementar estrategias de tratamiento selectivo; así se ha abierto la posibilidad de utilizar medidas más conservadoras para el tratamiento de los episodios de bajo riesgo, entre ellas la administración de regímenes orales ambulatorios de antibióticos de amplio espectro; ello sin demeritar la necesidad de aplicar un juicio clínico adecuado, hacer un buen seguimiento y tener acceso a la atención médica inmediata. La neutropenia es una de las consecuencias graves de la quimioterapia para el cáncer, y se ha demostrado que el tratamiento del paciente neutropénico febril con antibióticos intravenosos reduce la mortalidad. La terapia oral podría ser una alternativa aceptable para pacientes bien seleccionados. Ella puede mejorar la calidad de vida de los pacientes con cáncer, evitar las complicaciones asociadas con la terapia intravenosa y disminuir los costos del tratamiento.

    Treatment of patients with neoplasia and febrile neutropenia, as a consequence of chemotherapy, poses many doubts, among them the need for hospitalization, since this implies exposure to potentially resistant nosocomial microorganisms. Even under the best isolation techniques, there may

  17. Diagnosis of autoimmune neutropenia by neutrophil-bound IgG and IgM antibodies.

    Science.gov (United States)

    Ito, Taichi; Taniuchi, Shoichiro; Tsuji, Shoji; Iharada, Anna; Hasui, Masafumi; Kaneko, Kazunari

    2011-10-01

    Autoimmune neutropenia (AIN) in infancy is caused by antineutrophil (granulocyte-specific) autoantibodies. These antibodies are rarely found in circulation because their serum levels are extremely low. We hypothesized that a direct granulocyte immunofluorescence test (D-GIFT) that enables us to detect neutrophil-bound autoantibodies consisting of both immunoglobulin (Ig) G and IgM has better diagnostic value than the detection of circulating autoantibodies. Whole blood (100 μL) was obtained from 50 infants with AIN, 12 infants with transient neutropenia, and 37 control infants. D-GIFT was performed using both fluorescein isothiocyanate-conjugated antihuman IgG Fc portion monoclonal antibodies and fluorescein isothiocyanate antihuman IgM monoclonal antibodies. Results were assessed as relative fluorescence intensity (RFI). The RFIs of antineutrophil IgG-bound and antineutrophil IgM-bound cells in patients with AIN were significantly higher than those in patients with transient neutropenia and in controls. Positive results, as assessed by RFI scores of more than 1.81 in either antineutrophil IgG-bound or antineutrophil IgM-bound cells, showed the sensitivity and specificity of D-GIFT, and the areas under the receiver operating characteristic curve (0.98, 0.98, and 0.997, respectively) in the diagnosis of AIN. D-GIFT detecting both neutrophil-bound IgG autoantibodies and IgM autoantibodies has discriminatory power for identifying patients with AIN and, therefore, can be a useful diagnostic test. PMID:21941149

  18. Duodenal duplication cyst causing severe pancreatitis:Imaging findings and pathological correlation

    Institute of Scientific and Technical Information of China (English)

    Alessandro Guarise; Niccolo' Faccioli; Mauro Ferrari; Luigi Romano; Alice Parisi; Massimo Falconi

    2006-01-01

    We here report a case of a 18-year-old man with a history of recurrent abdominal pain and a previous episode of severe acute pancreatitis. Abdominal ultrasonography,contrast enhanced multislice computer tomography,endoscopic retrograde cholangiopancreatography, endoscopic ultrasonography and magnetic resonance imaging demonstrated a cystic mass lesion. Only on delayed phase magnetic resonance images after GadoliniumBOPTA injection, it was possible to demonstrate the lesion's relationship with the biliary tree, differentiating the lesion from intraluminal duodenal diverticulum, and to achieve the diagnosis of duodenal duplication cyst, a recognized rare cause of acute pancreatitis. The diagnosis was confirmed by histology.

  19. A pilot study examining garment severance damage caused by a trained sharp-weapon user

    OpenAIRE

    Cowper, E J; Mahoney, P F; Godhania, K; Carr, D. J.; Harrison, K.

    2016-01-01

    The pilot study summarized in this paper aimed to raise awareness of a gap that exists in the forensic textile science literature about damage caused to clothing by trained sharp-weapon users. A male trained in the Filipino martial arts discipline of Eskrima performed attack techniques on a physical model of a male torso covered with a 97% cotton/3% elastane knitted T-shirt, that is, a garment commonly worn by males. Fabric severance appearance created by three different, but commonly availab...

  20. Incidence, causes, severity and treatment of throat discomfort: a four-region online questionnaire survey

    Directory of Open Access Journals (Sweden)

    Addey Dilys

    2012-08-01

    Full Text Available Abstract Background Acute sore throat is commonly associated with viral infections. Consumers typically rely on over-the-counter treatments and other remedies to treat symptoms; however, limited information is available regarding consumer perceptions of sore throat or treatment needs. The aim of this study was to investigate perceptions of throat discomfort and how these influence attitudes and consumer behaviour with regard to treatment. Methods Online consumer surveys were completed by participants invited by email between 2003 and 2004 in four markets: the UK, France, Poland, and Malaysia. The questionnaire consisted of 24 questions that covered key issues surrounding throat discomfort including incidence in the past 12 months, causes, severity, effects on functionality and quality of life, actions taken to relieve throat discomfort, the efficacy of these approaches and the reasons behind using specific products. Results In total, 6465 men and women aged ≥18 years were surveyed, identifying 3514 participants who had suffered throat discomfort/irritation in the past 12 months (response rate of 54%. These participants completed the full survey. The breakdown of throat discomfort sufferers was: UK, 912; France, 899; Poland, 871; Malaysia, 832. A high proportion of respondents experienced one or more instances of throat discomfort in the previous 12 months, with an overall incidence of 54%. Infections including the common cold/influenza and other bacteria/viruses were commonly perceived causes of throat discomfort (72% and 46%, respectively. Physical and environmental factors were also perceived to be causative, including airborne pollution (28%, smoking (23%, and air conditioning (31%. Symptoms perceived to be caused by an infection were associated with a higher degree of suffering (mean degree of suffering for bacteria/virus and common cold/influenza; 3.4 and 3.0, respectively. Medicinal products were used for all perceived causes, but more

  1. Evidence of increasing drought severity caused by temperature rise in southern Europe

    International Nuclear Information System (INIS)

    We use high quality climate data from ground meteorological stations in the Iberian Peninsula (IP) and robust drought indices to confirm that drought severity has increased in the past five decades, as a consequence of greater atmospheric evaporative demand resulting from temperature rise. Increased drought severity is independent of the model used to quantify the reference evapotranspiration. We have also focused on drought impacts to drought-sensitive systems, such as river discharge, by analyzing streamflow data for 287 rivers in the IP, and found that hydrological drought frequency and severity have also increased in the past five decades in natural, regulated and highly regulated basins. Recent positive trend in the atmospheric water demand has had a direct influence on the temporal evolution of streamflows, clearly identified during the warm season, in which higher evapotranspiration rates are recorded. This pattern of increase in evaporative demand and greater drought severity is probably applicable to other semiarid regions of the world, including other Mediterranean areas, the Sahel, southern Australia and South Africa, and can be expected to increasingly compromise water supplies and cause political, social and economic tensions among regions in the near future. (paper)

  2. TUBA1A mutation can cause a hydranencephaly-like severe form of cortical dysgenesis.

    Science.gov (United States)

    Yokoi, Setsuri; Ishihara, Naoko; Miya, Fuyuki; Tsutsumi, Makiko; Yanagihara, Itaru; Fujita, Naoko; Yamamoto, Hiroyuki; Kato, Mitsuhiro; Okamoto, Nobuhiko; Tsunoda, Tatsuhiko; Yamasaki, Mami; Kanemura, Yonehiro; Kosaki, Kenjiro; Kojima, Seiji; Saitoh, Shinji; Kurahashi, Hiroki; Natsume, Jun

    2015-10-23

    TUBA1A mutations cause a wide spectrum of lissencephaly and brain malformations. Here, we report two patients with severe cortical dysgeneses, one with an extremely thin cerebral parenchyma apparently looking like hydranencephaly and the other with lissencephaly accompanied by marked hydrocephalus, both harbouring novel de novo missense mutations of TUBA1A. To elucidate how the various TUBA1A mutations affect the severity of the phenotype, we examined the capacity of the mutant protein to incorporate into the endogenous microtubule network in transfected COS7 cells by measuring line density using line extraction in an immunofluorescence study. The mutants responsible for severe phenotypes were found to incorporate extensively into the network. To determine how each mutant alters the microtubule stability, we examined cold-induced microtubule depolymerisation in fibroblasts. The depolymerisation of patients' fibroblasts occurred earlier than that of control fibroblasts, suggesting that microtubules bearing mutated tubulins are unstable. Both mutations are predicted to participate in lateral interactions of microtubules. Our data suggest that the TUBA1A mutations disrupting lateral interactions have pronounced dominant-negative effects on microtubule dynamics that are associated with the severe end of the lissencephaly spectrum.

  3. Comparing the Pain Severity Caused by Muscular Injection of Tramadole in Z and Bulb Methods

    Directory of Open Access Journals (Sweden)

    SH Najafi Doulatabad

    2008-01-01

    Full Text Available ABSTRACT: Introduction & Objective: Pain, rather than any other problems, would persuade people to pursue remedy and treatment. Muscular injections are among pain producing factors. This technique of prescription can be followed by some complications from which pain is the most prevalent one. This study was performed to compare the pain severity caused by muscular injection of Tramadole in Z and Bulb methods. Materials & Methods: This is a clinical trial study in which 90 women who referred to the emergency ward of Shahid Beheshti hospital in Yasooj in 2006 were randomly selected. Data collection instrument was questionnaire. The pain severity was evaluated by visual scale. Data were analyzed by descriptive statistical method, independent T and chi square test using SPSS software. Results: Mean of pain severity in bulb method was 2.84±1.242 and in Z method was 4.56±1.659. The independent T test indicated that the differences in pain severity in two injection methods were statistically significant (p=0.036. No significant differences were found between age, educational level, job, marriatal status, number of previous injections and BMI of samples between two groups. Conclusion: Bulb method injection induces less pain in comparison with Z method therefore it is an ideal method for muscular injections. As such, health care workers, especially nurses, can, through this method, minimize the patients’ pain, which is one of the most important injection complaints.

  4. Superior Mesenteric Vein Occlusion Causing Severe Gastrointestinal Haemorrhage in Two Paediatric Cases

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    Anna L. Fox

    2012-01-01

    Full Text Available Reports about superior mesenteric vein thrombosis in childhood are very rare and have not been associated with gastrointestinal bleeding. We describe two cases of severe bleeding from the upper and lower gastrointestinal tract in children who had undergone complex abdominal surgery at considerable time before. The first child had a tracheoesophageal fistula, corrected by division, gastrostomy insertion, and repair of duodenal rupture. The child presented with severe bleeding from the gastrostomy site and was diagnosed with a thrombosis of the proximal superior mesenteric vein. The second child had a gastroschisis and duodenal atresia, and required duodenoplasty, gastrostomy insertion, hemicolectomy, and adhesiolysis. The child presented with intermittent severe lower gastrointestinal bleeding, resulting from collateral vessels at location of the surgical connections. He was diagnosed with a thrombosis of the superior mesenteric vein. In both children, the extensive previous surgery and anastomosis were considered the cause of the mesenteric thrombosis. CT angiography confirmed the diagnosis in both cases, in addition to characteristic findings on endoscopy. Paediatricians should suspect this condition in children with severe gastrointestinal bleeding, particularly in children with previous, complex abdominal surgery.

  5. Adrenomedullin--A New Marker in Febrile Neutropenia: Comparison With CRP and Procalcitonin.

    Science.gov (United States)

    Demirkaya, Metin; Tugcu, Deniz; Akcay, Arzu; Aydogan, Gönül; Akıcı, Ferhan; Salcioglu, Zafer; Ekmekci, Hakan; Sevinir, Betül; Balci Ekmekci, Ozlem

    2015-01-01

    In this study, we aimed to determine serum adrenomedullin levels and compare them with levels of C-reactive protein (CRP) and procalcitonin (PCT). Cancer patients aged 0-18 years who experienced febrile neutropenia attacks were included in the study. Adrenomedullin, CRP, and PCT were analyzed at admission, day 3, and days 7-10 later. Fifty episodes of febrile neutropenia that developed in 37 patients were analyzed in this study. The mean age of the patients was 7.5 ± 4.7 (1-18) years. The patients had leukemia (73%), solid tumors (19%), and lymphoma (8%). The percentages of the patients in the clinically documented infection (CDI), fever of unknown origin (FUO), sepsis, and microbiological documented infection (MDI) categories were 34%, 34%, 20%, and 12%, respectively. During the study period, four patients were lost. In the MDI group, adrenomedullin levels on day 3 were significantly higher than those in the CDI and FUO groups. PCT levels were significantly higher in the sepsis group than those in the CDI group at admission, day 3, and days 7-10. In the sepsis group, PCT levels on days 7-10 days were significantly higher than those in the sepsis group. PCT values from the deceased patients on days 7-10 were significantly higher than those from patients who survived. CRP levels did not differ significantly among the febrile neutropenia groups. First, in our study, adrenomedullin was used as a biomarker in the febrile neutropenia episodes of children with cancer. Among adrenomedullin, CRP, and PCT, procalcitonin demonstrates the highest correlation with the severity of infection.

  6. Severe Community-Acquired Pneumonia Caused by Human Adenovirus in Immunocompetent Adults: A Multicenter Case Series.

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    Dingyu Tan

    Full Text Available Severe community-acquired pneumonia (CAP caused by human adenovirus (HAdV, especially HAdV type 55 (HAdV-55 in immunocompetent adults has raised increasing concerns. Clinical knowledge of severe CAP and acute respiratory distress syndrome induced by HAdV-55 is still limited, though the pathogen has been fully characterized by whole-genome sequencing.We conducted a multicentre retrospective review of all consecutive patients with severe CAP caused by HAdV in immunocompetent adults admitted to the Emergency Department Intensive Care Unit of two hospitals in Northern China between February 2012 and April 2014. Clinical, laboratory, radiological characteristics, treatments and outcomes of these patients were collected and analyzed.A total of 15 consecutive severe CAP patients with laboratory-confirmed adenovirus infections were included. The median age was 30 years and all cases were identified during the winter and spring seasons. HAdV-55 was the most frequently (11/15 detected HAdV type. Persistent high fever, cough and rapid progression of dyspnea were typically reported in these patients. Significantly increased pneumonia severity index (PSI, respiratory rate, and lower PaO2/FiO2, hypersensitive CRP were reported in non-survivors compared to survivors (P = 0.013, 0.022, 0.019 and 0.026, respectively. The rapid development of bilateral consolidations within 10 days after illness onset were the most common radiographic finding, usually accompanied by adjacent ground glass opacities and pleural effusions. Total mortality was 26.7% in this study. Corticosteroids were prescribed to 14 patients in this report, but the utilization rate between survivors and non-survivors was not significant.HAdV and the HAdV-55 sub-type play an important role among viral pneumonia pathogens in hospitalized immunocompetent adults in Northern China. HAdV should be tested in severe CAP patients with negative bacterial cultures and a lack of response to antibiotic

  7. Mutations in FKBP10 can cause a severe form of isolated Osteogenesis imperfecta

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    Steinlein Ortrud K

    2011-11-01

    Full Text Available Abstract Background Mutations in the FKBP10 gene were first described in patients with Osteogenesis imperfecta type III. Two follow up reports found FKBP10 mutations to be associated with Bruck syndrome type 1, a rare disorder characterized by congenital contractures and bone fragility. This raised the question if the patients in the first report indeed had isolated Osteogenesis imperfecta or if Bruck syndrome would have been the better diagnosis. Methods The patients described here are affected by severe autosomal recessive Osteogenesis imperfecta without contractures. Results Homozygosity mapping identified FKBP10 as a candidate gene, and sequencing revealed a base pair exchange that causes a C-terminal premature stop codon in this gene. Conclusions Our study demonstrates that FKBP10 mutations not only cause Bruck syndrome or Osteogenesis imperfecta type III but can result in a severe type of isolated Osteogenesis imperfecta type IV with prenatal onset. Furthermore, it adds dentinogenesis imperfecta to the spectrum of clinical symptoms associated with FKBP10 mutations.

  8. Severe visual Impairment and blindness in infants: Causes and opportunities for control

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    Parikshit Gogate

    2011-01-01

    Full Text Available Childhood blindness has an adverse effect on growth, development, social, and economic opportunities. Severe visual impairment (SVI and blindness in infants must be detected as early as possible to initiate immediate treatment to prevent deep amblyopia. Although difficult, measurement of visual acuity of an infant is possible. The causes of SVI and blindness may be prenatal, perinatal, and postnatal. Congenital anomalies such as anophthalmos, microphthalmos, coloboma, congenital cataract, infantile glaucoma, and neuro-ophthalmic lesions are causes of impairment present at birth. Ophthalmia neonatorum, retinopathy of prematurity, and cortical visual impairment are acquired during the perinatal period. Leukocoria or white pupillary reflex can be cause by congenital cataract, persistent hyperplastic primary vitreous, or retinoblastoma. While few medical or surgical options are available for congenital anomalies or neuro-ophthalmic disorders, many affected infants can still benefit from low vision aids and rehabilitation. Ideally, surgery for congenital cataracts should occur within the first 4 months of life. Anterior vitrectomy and primary posterior capsulotomy are required, followed by aphakic glasses with secondary intraocular lens implantation at a later date. The treatment of infantile glaucoma is surgery followed by anti-glaucoma medication. Retinopathy of prematurity is a proliferation of the retinal vasculature in response to relative hypoxia in a premature infant. Screening in the first few weeks of life can prevent blindness. Retinoblastoma can be debulked with chemotherapy; however, enucleation may still be required. Neonatologists, pediatricians, traditional birth attendants, nurses, and ophthalmologists should be sensitive to a parent′s complaints of poor vision in an infant and ensure adequate follow-up to determine the cause. If required, evaluation under anesthesia should be performed, which includes funduscopy, refraction

  9. Staphylococcus aureus seroproteomes discriminate ruminant isolates causing mild or severe mastitis

    Directory of Open Access Journals (Sweden)

    Le Maréchal Caroline

    2011-02-01

    Full Text Available Abstract Staphylococcus aureus is a major cause of mastitis in ruminants. In ewe mastitis, symptoms range from subclinical to gangrenous mastitis. S. aureus factors or host-factors contributing to the different outcomes are not completely elucidated. In this study, experimental mastitis was induced on primiparous ewes using two S. aureus strains, isolated from gangrenous (strain O11 or subclinical (strain O46 mastitis. Strains induced drastically distinct clinical symptoms when tested in ewe and mice experimental mastitis. Notably, they reproduced mild (O46 or severe (O11 mastitis in ewes. Ewe sera were used to identify staphylococcal immunoreactive proteins commonly or differentially produced during infections of variable severity and to define core and accessory seroproteomes. Such SERological Proteome Analysis (SERPA allowed the identification of 89 immunoreactive proteins, of which only 52 (58.4% were previously identified as immunogenic proteins in other staphylococcal infections. Among the 89 proteins identified, 74 appear to constitute the core seroproteome. Among the 15 remaining proteins defining the accessory seroproteome, 12 were specific for strain O11, 3 were specific for O46. Distribution of one protein specific for each mastitis severity was investigated in ten other strains isolated from subclinical or clinical mastitis. We report here for the first time the identification of staphylococcal immunogenic proteins common or specific to S. aureus strains responsible for mild or severe mastitis. These findings open avenues in S. aureus mastitis studies as some of these proteins, expressed in vivo, are likely to account for the success of S. aureus as a pathogen of the ruminant mammary gland.

  10. Depletion of WRN protein causes RACK1 to activate several protein kinase C isoforms

    DEFF Research Database (Denmark)

    Massip, L; Garand, C; Labbé, A;

    2010-01-01

    show that a knock down of the WRN protein in normal human fibroblasts induces phosphorylation and activation of several protein kinase C (PKC) enzymes. Using a tandem affinity purification strategy, we found that WRN physically and functionally interacts with receptor for activated C-kinase 1 (RACK1...... activity in vitro. Interestingly, knocking down RACK1 increased the cellular frequency of DNA breaks. Depletion of the WRN protein in return caused a fraction of nuclear RACK1 to translocate out of the nucleus to bind and activate PKCdelta and PKCbetaII in the membrane fraction of cells. In contrast......), a highly conserved anchoring protein involved in various biological processes, such as cell growth and proliferation. RACK1 binds strongly to the RQC domain of WRN and weakly to its acidic repeat region. Purified RACK1 has no impact on the helicase activity of WRN, but selectively inhibits WRN exonuclease...

  11. Agricultural measures to reduce radiation doses to man caused by severe nuclear accidents

    International Nuclear Information System (INIS)

    Agricultural land and products may become contaminated after a severe nuclear accident. If radiation doses to man caused by the ingestion of contaminated agricultural products from such areas will be unacceptably high, measures to reduce this radiation dose will have to be taken. Radiation doses to man can be estimated by using models which describe quantitatively the transfer of radionuclides through the biosphere. The following processes and pathways are described in this study: accidental releases into atmospheric environments and subsequent nearby deposition; contamination of crops by direct deposition and the subsequent short term pathway (e.g. grass-cow-milk-man); contamination of soil and the subsequent long term pathway (e.g. soil-crop-man, soil-grass-cattle-milk/meat-man). Depending on the degree of contamination and on the estimated radiation doses to man, various measures are advised. (Auth.)

  12. Neutropenia in chronic hepatitis C during Interferon and Ribavirin Therapy.

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    Saadia Farid, Hala Morad and Samya Sweilam.

    2011-10-01

    Full Text Available Background: Neutropenia is a condition characterized by an abnormally low number of a type of white blood cells called Neutrophils, up to 25 % of people who take pegylated interferon, ribavirin and an HCV protease inhibitor experience Neutropenia. Aim of the work: The study will be intended to analyze neutrophil counts and associated conditions of the liver and spleen , platelet count, liver enzymes and infections, during Interferon and Ribavirin therapy. Patients and methods: One hundred forty two patients with chronic hepatitis C virus infection, their age between (18-59 years, selected from the National Hepatology and Tropical Medicine Research Institute were included in this study, during Interferon and Ribavirin therapy. All the patients were subjected to the following history, through clinical examination, abdominal ultrasonography and collection of blood samples for routine investigations, CBCs and serological assay for ALT, Bilirubin. Resuls: Our results revealed presence of 32.4 % anaemia, 18.3 % Thrombocytopenia, 16.9 % elevated ALT, 2.8 % elevated bilirubine, 16.9 % coarse liver, 25.4 % hepatomegaly, 16.2 % splenomegaly, and 16.9 % of cases complained different shapes of infection, associated with Neutropenia in patients of chronic hepatitis C during interferon and ribavirin therapy. Conclusion: Our study concluded that the prevalence of Neutropenia in chronic hepatitis C virus infection patients 23.8 % during interferon and ribavirin therapy but it is not usually associated with infection. Recommendations: Neutropenia is a complicated process that requires expert guidance from a medical provider.

  13. Obstructive sleep apnoea in Treacher Collins syndrome: prevalence, severity and cause.

    Science.gov (United States)

    Plomp, R G; Bredero-Boelhouwer, H H; Joosten, K F M; Wolvius, E B; Hoeve, H L J; Poublon, R M L; Mathijssen, I M J

    2012-06-01

    This cohort study in 35 patients (13 children) evaluates the prevalence, severity and anatomical cause of obstructive sleep apnoea syndrome (OSAS) in patients with Treacher Collins syndrome. Ambulatory polysomnography was performed cross-sectionally to determine OSAS prevalence and severity. All upper airway related surgical interventions were evaluated retrospectively. In 11 patients, sleep endoscopy, and flexible and rigid endoscopy were applied to determine the level of anatomical obstruction of the upper airway. The overall prevalence of OSAS in Treacher Collins patients was 46% (54% in children; 41% in adults). Thirty-eight upper airway related surgical interventions were performed in 17 patients. Examination of the upper airway revealed various anatomical levels of obstruction, from the nasal septum to the trachea. Most significant obstruction was found at the level of the oro/hypopharynx. OSAS in Treacher Collins patients is an important problem so all patients should be screened for OSAS by polysomnography. Endoscopy of the upper airways was helpful in determining the level of obstruction. Surgical treatment at one level will not resolve OSAS in most patients because OSAS in Treacher Collins has a multilevel origin. Non-invasive ventilation (continuous positive airway pressure or bilevel positive airway pressure) or tracheotomy should be considered as a treatment modality.

  14. Imbalances in serum angiopoietin concentrations are early predictors of septic shock development in patients with post chemotherapy febrile neutropenia

    Directory of Open Access Journals (Sweden)

    Lorand-Metze Irene

    2010-05-01

    Full Text Available Abstract Background Febrile neutropenia carries a high risk of sepsis complications, and the identification of biomarkers capable to identify high risk patients is a great challenge. Angiopoietins (Ang - are cytokines involved in the control microvascular permeability. It is accepted that Ang-1 expression maintains endothelial barrier integrity, and that Ang-2 acts as an antagonizing cytokine with barrier-disrupting functions in inflammatory situations. Ang-2 levels have been recently correlated with sepsis mortality in intensive care units. Methods We prospectively evaluated concentrations of Ang-1 and Ang-2 at different time-points during febrile neutropenia, and explored the diagnostic accuracy of these mediators as potential predictors of poor outcome in this clinical setting before the development of sepsis complications. Results Patients that evolved with septic shock (n = 10 presented higher levels of Ang-2 measured 48 hours after fever onset, and of the Ang-2/Ang-1 ratio at the time of fever onset compared to patients with non-complicated sepsis (n = 31. These levels correlated with sepsis severity scores. Conclusions Our data suggest that imbalances in the concentrations of Ang-1 and Ang-2 are independent and early markers of the risk of developing septic shock and of sepsis mortality in febrile neutropenia, and larger studies are warranted to validate their clinical usefulness. Therapeutic strategies that manipulate this Ang-2/Ang-1 imbalance can potentially offer new and promising treatments for sepsis in febrile neutropenia.

  15. Continuing clozapine treatment with lithium in schizophrenic patients with neutropenia or leukopenia: brief review of literature with case reports

    Science.gov (United States)

    Aydin, Memduha; Ilhan, Bilge Cetin; Calisir, Saliha; Yildirim, Seda; Eren, Ibrahim

    2016-01-01

    Objective: Clozapine is a second-generation antipsychotic used for treatment-resistant schizophrenia. Despite its effectiveness, clozapine is largely underused due to serious side effects such as leukopenia or neutropenia. We aimed to review whether to continue, discontinue or rechallenge clozapine treatment after such haematological side effects. Methods: We reviewed and summarized the literature on the use of clozapine, how to deal with its side effects, and suitable options in case of any haematological problems. Then, we described several cases successfully treated with clozapine and lithium after development of neutropenia or leukopenia Results: We present three patients with treatment-resistant schizophrenia. While they had demonstrated poor response to multiple antipsychotic trials, clozapine was started. Clozapine induced neutropenia; or leukopenia developed in some cases that was successfully reversed after lithium onset. Increased serious side effects related with coprescription of lithium and clozapine were not observed. Conclusion: Lithium increases neutrophil and total white blood cell count as a side effect that may be useful in patients who develop neutropenia or leukopenia while being treated with clozapine. PMID:26913176

  16. Norepinephrine-induced nerve growth factor depletion causes cardiac sympathetic denervation in severe heart failure.

    Science.gov (United States)

    Kimura, Kensuke; Kanazawa, Hideaki; Ieda, Masaki; Kawaguchi-Manabe, Haruko; Miyake, Yoshiko; Yagi, Takashi; Arai, Takahide; Sano, Motoaki; Fukuda, Keiichi

    2010-08-25

    In severe congestive heart failure (CHF), sympathetic overactivity correlates with the exacerbation of cardiac performance. To test the hypothesis that the cardiac sympathetic nerve density dramatically changes with the acceleration of circulating norepinephrine (NE) concentration, we investigated the temporal association of nerve growth factor (NGF) expression in the heart and cardiac sympathetic nerve density during the development of CHF in the continuous NE-infused rats. The animals were analyzed at 0-, 1-, 3-, 7-, 14-, and 28-day after implantation of osmotic pump at a rate of 0.05 mg/kg/hr. The cardiac performance was temporally facilitated in NE-exposed rats at 3-day in accordance with the sympathetic hyper-innervation induced by the augmentation of NGF mRNA expression in the heart. In NE-treated rats, left ventricular end-diastolic pressure was significantly increased after 7-day and marked left ventricular hypertrophy and systemic fluid retention were observed at 28-day. CHF-induced sympathetic overactivity further increased plasma NE concentration in NE-treated rats and finally reached to 16.1+/-5.6 ng/ml at 28-day (control level was 0.39+/-0.1 ng/ml, pcardiac performance. The cardiac sympathetic fiber loss was also confirmed in NE-exposed DBH (dopamine beta-hydroxylase)-Cre/Floxed-EGFP (enhanced green fluorescent protein) mice with severe CHF, in which sympathetic nerve could be traced by EGFP. Our results suggest that the cardiac sympathetic nerve density is strictly regulated by the NGF expression in the heart and long-exposure of high plasma NE concentration caused myocardial NGF reduction, following sympathetic fiber loss in severe CHF animals.

  17. Pulmonary Veno-Occlusive Disease: A Newly Recognized Cause of Severe Pulmonary Hypertension in Dogs.

    Science.gov (United States)

    Williams, K; Andrie, K; Cartoceti, A; French, S; Goldsmith, D; Jennings, S; Priestnall, S L; Wilson, D; Jutkowitz, A

    2016-07-01

    Pulmonary hypertension is a well-known though poorly characterized disease in veterinary medicine. In humans, pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Eleven adult dogs (5 males, 6 females; median age 10.5 years, representing various breeds) were examined following the development of severe respiratory signs. Lungs of affected animals were evaluated morphologically and with immunohistochemistry for alpha smooth muscle actin, desmin, CD31, CD3, CD20, and CD204. All dogs had pulmonary lesions consistent with PVOD, consisting of occlusive remodeling of small- to medium-sized pulmonary veins, foci of pulmonary capillary hemangiomatosis (PCH), and accumulation of hemosiderophages; 6 of 11 dogs had substantial pulmonary arterial medial and intimal thickening. Ultrastructural examination and immunohistochemistry showed that smooth muscle cells contributed to the venous occlusion. Increased expression of CD31 was evident in regions of PCH indicating increased numbers of endothelial cells in these foci. Spindle cells strongly expressing alpha smooth muscle actin and desmin co-localized with foci of PCH; similar cells were present but less intensely labeled elsewhere in non-PCH alveoli. B cells and macrophages, detected by immunohistochemistry, were not co-localized with the venous lesions of canine PVOD; small numbers of CD3-positive T cells were occasionally in and around the wall of remodeled veins. These findings indicate a condition in dogs with clinically severe respiratory disease and pathologic features resembling human PVOD, including foci of pulmonary venous remodeling and PCH.

  18. Pulmonary Veno-Occlusive Disease: A Newly Recognized Cause of Severe Pulmonary Hypertension in Dogs.

    Science.gov (United States)

    Williams, K; Andrie, K; Cartoceti, A; French, S; Goldsmith, D; Jennings, S; Priestnall, S L; Wilson, D; Jutkowitz, A

    2016-07-01

    Pulmonary hypertension is a well-known though poorly characterized disease in veterinary medicine. In humans, pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Eleven adult dogs (5 males, 6 females; median age 10.5 years, representing various breeds) were examined following the development of severe respiratory signs. Lungs of affected animals were evaluated morphologically and with immunohistochemistry for alpha smooth muscle actin, desmin, CD31, CD3, CD20, and CD204. All dogs had pulmonary lesions consistent with PVOD, consisting of occlusive remodeling of small- to medium-sized pulmonary veins, foci of pulmonary capillary hemangiomatosis (PCH), and accumulation of hemosiderophages; 6 of 11 dogs had substantial pulmonary arterial medial and intimal thickening. Ultrastructural examination and immunohistochemistry showed that smooth muscle cells contributed to the venous occlusion. Increased expression of CD31 was evident in regions of PCH indicating increased numbers of endothelial cells in these foci. Spindle cells strongly expressing alpha smooth muscle actin and desmin co-localized with foci of PCH; similar cells were present but less intensely labeled elsewhere in non-PCH alveoli. B cells and macrophages, detected by immunohistochemistry, were not co-localized with the venous lesions of canine PVOD; small numbers of CD3-positive T cells were occasionally in and around the wall of remodeled veins. These findings indicate a condition in dogs with clinically severe respiratory disease and pathologic features resembling human PVOD, including foci of pulmonary venous remodeling and PCH. PMID:26926086

  19. Potential of a COX-2 inhibitor in lowering chemotherapy-induced neutropenia%Potential of a COX-2 inhibitor in lowering chemotherapy induced neutropenia

    Institute of Scientific and Technical Information of China (English)

    Louis Wing-Cheong Chow; Adrian Yun-San Yip; Eleanor Yuen-Yuen Ong; Chi-Kei Lam; Masakazu Toi

    2010-01-01

    Objective This study was initially designed to evaluate the effect of celecoxib on the regimen of 5 fluorouracil, epirubicin, and cyclophosphamide (FEC) combination, followed by docetaxel (T) in neoadjuvant setting. An unplanned preliminary review on safety was conducted after a halt of the study due to the concerned potential cardiovascular risk of using COX 2 inhibitors.Methods We studied 23 consecutive cases of operable breast cancer having received four cycles of FEC(500 mg/m2, 100 mg/m2, 500 mg/m2) followed by four cycles of T(100 mg/m2) with concurrent celecoxib (400 mg twice daily) (group A) or same chemotherapy regimen but without concurrent celecoxib (group B). These combined chemotherapies were administered every 3 weeks. The Chi square test or Fisher's exact test were used to assess the difference in incidence of limiting hematological toxicites between groups. Results 23 patients (group A: n=12; group B, n=11) received a total of 183 out of 184 planned treatment cycles; one (4%, 1/23) of them omitted the fourth cycle of FEC owing to repeated incidences of febrile neutropenia. Received dose intensity (RDI) for FEC in group A (90%±11%) was higher than that in group B (80%±8%) while RDI for T was similar between group A (93%±8%) and group B (96%±9%). Of the first 91 treatment cycles of FEC, limiting hematological toxicity, severe neutropenia including febrile neutropenia, was significantly different between group A and B [(10.4%, 5/48) vs.( 32.6%, 14/43), P=0.009]. Other toxicities commonly observed in chemotherapy receiving patients were manageable. Conclusions Neoadjuvant use of FEC followed by T with concurrent celecoxib appeared to be safe for treatment of operable invasive breast cancer. The observed lower incidence of chemotherapy induced neutropenia is possibly contributed by the administration of COX inhibitor. We believe that further investigation might provide more evidence on the use of COX 2 inhibitors in breast cancer.

  20. Brachial plexus palsy caused by halo traction before posterior correction in patients with severe scoliosis

    Institute of Scientific and Technical Information of China (English)

    QIAN Bang-ping; QIU Yong; WANG Bin; YU Yang; ZHU Ze-zhang

    2007-01-01

    Objective: To explore the clinical features and treatment results of brachial plexus palsy caused by halo traction before posterior correction in patients with severe scoliosis.Methods: A total of 300 cases of severe scoliosis received halo traction before posterior correction in our department from July 1997 to November 2004. Among them, 7 cases were complicated with brachial plexus palsy.The average Cobb angle was 110° (range, 90°-135°).Diagnoses were made as idiopathic scoliosis in 1 case,congenital scoliosis in 3 cases, and neuromuscular scoliosis in 3 cases. Additionally, diastematomyelia and tethered cord syndrome were found in 3 cases and thoracolumbar kyphosis in 2 cases. Weight of traction was immediately reduced when the patient developed any abnormal neurological symptoms in the upper extremity, and rehabilitation training was undertaken. Simultaneously,neurotrophic pharmacotherapy was applied, and the neurological function restoration of the upper limbs and the recovery time were documented.Results: Traction was used for an average of 3.5 weeks (range, 2-6 weeks) before spinal fusion for these 7 patients. The average traction weight was 8 kg, which was 19% on average (range, 13%-26%) of the average body weight (40.2 kg). These 7 patients had long and thin body configuration with a mean height of 175 cm. The duration between symptoms of brachial plexus paralysis and the diagnosis was 1-3 hours. All of these 7 patients presented various degrees of numbness in the ulnar side of the hand and forearm. Median nerve paresis was found in 3 cases and ulnar nerve paresis in 4 cases. Complete recovery of the neurological function had been achieved by the end of three months.Conclusions: The clinical features of brachial plexus palsy caused by halo traction include median nerve paresis,ulnar nerve paralysis, and numbness in the ulnar side of the hand and forearm, which may be due to the injury of the inferior part of the brachial plexus, i.e. , damage of Cs and

  1. Fluoroquinolone prophylaxis against febrile neutropenia in areas with high fluoroquinolone resistance--an Asian perspective.

    Science.gov (United States)

    Ng, Esther Shu-Ting; Liew, Yixin; Koh, Liang Piu; Hsu, Li Yang

    2010-09-01

    Febrile neutropenia remains a major cause of morbidity and mortality in patients receiving chemotherapy. Major prophylactic strategies include granulocyte colony-stimulating factor and antibiotics, the most widely used of which are fluoroquinolones. While fluoroquinolone prophylaxis has been shown to be effective in areas where fluoroquinolone resistance is low, this same efficacy has not been proven in areas where resistance is high, such as in Asia. Given the increase in antimicrobial resistance with the use of prophylaxis, the risks and benefits of this strategy need to be carefully considered. This review presents the evidence for and against fluoroquinolone prophylaxis in areas of high fluoroquinolone resistance.

  2. Guanylate cyclase C deficiency causes severe inflammation in a murine model of spontaneous colitis.

    Directory of Open Access Journals (Sweden)

    Eleana Harmel-Laws

    Full Text Available BACKGROUND: Guanylate Cyclase C (GC-C; Gucy2c is a transmembrane receptor expressed in intestinal epithelial cells. Activation of GC-C by its secreted ligand guanylin stimulates intestinal fluid secretion. Familial mutations in GC-C cause chronic diarrheal disease or constipation and are associated with intestinal inflammation and infection. Here, we investigated the impact of GC-C activity on mucosal immune responses. METHODS: We utilized intraperitoneal injection of lipopolysaccharide to elicit a systemic cytokine challenge and then measured pro-inflammatory gene expression in colonic mucosa. GC-C(+/+ and GC-C(-/- mice were bred with interleukin (IL-10 deficient animals and colonic inflammation were assessed. Immune cell influx and cytokine/chemokine expression was measured in the colon of wildtype, IL-10(-/-, GC-C(+/+IL-10(-/- and GC-C(-/-IL-10(-/- mice. GC-C and guanylin production were examined in the colon of these animals and in a cytokine-treated colon epithelial cell line. RESULTS: Relative to GC-C(+/+ animals, intraperitoneal lipopolysaccharide injection into GC-C(-/- mice increased proinflammatory gene expression in both whole colon tissue and in partially purified colonocyte isolations. Spontaneous colitis in GC-C(-/-IL-10(-/- animals was significantly more severe relative to GC-C(+/+IL-10(-/- mice. Unlike GC-C(+/+IL-10(-/- controls, colon pathology in GC-C(-/-IL-10(-/- animals was apparent at an early age and was characterized by severely altered mucosal architecture, crypt abscesses, and hyperplastic subepithelial lesions. F4/80 and myeloperoxidase positive cells as well as proinflammatory gene expression were elevated in GC-C(-/-IL-10(-/- mucosa relative to control animals. Guanylin was diminished early in colitis in vivo and tumor necrosis factor α suppressed guanylin mRNA and protein in intestinal goblet cell-like HT29-18-N2 cells. CONCLUSIONS: The GC-C signaling pathway blunts colonic mucosal inflammation that is initiated by

  3. Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.

    Science.gov (United States)

    Alston, Charlotte L; Compton, Alison G; Formosa, Luke E; Strecker, Valentina; Oláhová, Monika; Haack, Tobias B; Smet, Joél; Stouffs, Katrien; Diakumis, Peter; Ciara, Elżbieta; Cassiman, David; Romain, Nadine; Yarham, John W; He, Langping; De Paepe, Boel; Vanlander, Arnaud V; Seneca, Sara; Feichtinger, René G; Płoski, Rafal; Rokicki, Dariusz; Pronicka, Ewa; Haller, Ronald G; Van Hove, Johan L K; Bahlo, Melanie; Mayr, Johannes A; Van Coster, Rudy; Prokisch, Holger; Wittig, Ilka; Ryan, Michael T; Thorburn, David R; Taylor, Robert W

    2016-07-01

    Complex I deficiency is the most common biochemical phenotype observed in individuals with mitochondrial disease. With 44 structural subunits and over 10 assembly factors, it is unsurprising that complex I deficiency is associated with clinical and genetic heterogeneity. Massively parallel sequencing (MPS) technologies including custom, targeted gene panels or unbiased whole-exome sequencing (WES) are hugely powerful in identifying the underlying genetic defect in a clinical diagnostic setting, yet many individuals remain without a genetic diagnosis. These individuals might harbor mutations in poorly understood or uncharacterized genes, and their diagnosis relies upon characterization of these orphan genes. Complexome profiling recently identified TMEM126B as a component of the mitochondrial complex I assembly complex alongside proteins ACAD9, ECSIT, NDUFAF1, and TIMMDC1. Here, we describe the clinical, biochemical, and molecular findings in six cases of mitochondrial disease from four unrelated families affected by biallelic (c.635G>T [p.Gly212Val] and/or c.401delA [p.Asn134Ilefs(∗)2]) TMEM126B variants. We provide functional evidence to support the pathogenicity of these TMEM126B variants, including evidence of founder effects for both variants, and establish defects within this gene as a cause of complex I deficiency in association with either pure myopathy in adulthood or, in one individual, a severe multisystem presentation (chronic renal failure and cardiomyopathy) in infancy. Functional experimentation including viral rescue and complexome profiling of subject cell lines has confirmed TMEM126B as the tenth complex I assembly factor associated with human disease and validates the importance of both genome-wide sequencing and proteomic approaches in characterizing disease-associated genes whose physiological roles have been previously undetermined.

  4. Biallelic Mutations in TMEM126B Cause Severe Complex I Deficiency with a Variable Clinical Phenotype.

    Science.gov (United States)

    Alston, Charlotte L; Compton, Alison G; Formosa, Luke E; Strecker, Valentina; Oláhová, Monika; Haack, Tobias B; Smet, Joél; Stouffs, Katrien; Diakumis, Peter; Ciara, Elżbieta; Cassiman, David; Romain, Nadine; Yarham, John W; He, Langping; De Paepe, Boel; Vanlander, Arnaud V; Seneca, Sara; Feichtinger, René G; Płoski, Rafal; Rokicki, Dariusz; Pronicka, Ewa; Haller, Ronald G; Van Hove, Johan L K; Bahlo, Melanie; Mayr, Johannes A; Van Coster, Rudy; Prokisch, Holger; Wittig, Ilka; Ryan, Michael T; Thorburn, David R; Taylor, Robert W

    2016-07-01

    Complex I deficiency is the most common biochemical phenotype observed in individuals with mitochondrial disease. With 44 structural subunits and over 10 assembly factors, it is unsurprising that complex I deficiency is associated with clinical and genetic heterogeneity. Massively parallel sequencing (MPS) technologies including custom, targeted gene panels or unbiased whole-exome sequencing (WES) are hugely powerful in identifying the underlying genetic defect in a clinical diagnostic setting, yet many individuals remain without a genetic diagnosis. These individuals might harbor mutations in poorly understood or uncharacterized genes, and their diagnosis relies upon characterization of these orphan genes. Complexome profiling recently identified TMEM126B as a component of the mitochondrial complex I assembly complex alongside proteins ACAD9, ECSIT, NDUFAF1, and TIMMDC1. Here, we describe the clinical, biochemical, and molecular findings in six cases of mitochondrial disease from four unrelated families affected by biallelic (c.635G>T [p.Gly212Val] and/or c.401delA [p.Asn134Ilefs(∗)2]) TMEM126B variants. We provide functional evidence to support the pathogenicity of these TMEM126B variants, including evidence of founder effects for both variants, and establish defects within this gene as a cause of complex I deficiency in association with either pure myopathy in adulthood or, in one individual, a severe multisystem presentation (chronic renal failure and cardiomyopathy) in infancy. Functional experimentation including viral rescue and complexome profiling of subject cell lines has confirmed TMEM126B as the tenth complex I assembly factor associated with human disease and validates the importance of both genome-wide sequencing and proteomic approaches in characterizing disease-associated genes whose physiological roles have been previously undetermined. PMID:27374774

  5. Divergent Simian Arteriviruses Cause Simian Hemorrhagic Fever of Differing Severities in Macaques

    Directory of Open Access Journals (Sweden)

    Victoria Wahl-Jensen

    2016-02-01

    Full Text Available Simian hemorrhagic fever (SHF is a highly lethal disease in captive macaques. Three distinct arteriviruses are known etiological agents of past SHF epizootics, but only one, simian hemorrhagic fever virus (SHFV, has been isolated in cell culture. The natural reservoir(s of the three viruses have yet to be identified, but African nonhuman primates are suspected. Eleven additional divergent simian arteriviruses have been detected recently in diverse and apparently healthy African cercopithecid monkeys. Here, we report the successful isolation in MARC-145 cell culture of one of these viruses, Kibale red colobus virus 1 (KRCV-1, from serum of a naturally infected red colobus (Procolobus [Piliocolobus] rufomitratus tephrosceles sampled in Kibale National Park, Uganda. Intramuscular (i.m. injection of KRCV-1 into four cynomolgus macaques (Macaca fascicularis resulted in a self-limiting nonlethal disease characterized by depressive behavioral changes, disturbance in coagulation parameters, and liver enzyme elevations. In contrast, i.m. injection of SHFV resulted in typical lethal SHF characterized by mild fever, lethargy, lymphoid depletion, lymphoid and hepatocellular necrosis, low platelet counts, increased liver enzyme concentrations, coagulation abnormalities, and increasing viral loads. As hypothesized based on the genetic and presumed antigenic distance between KRCV-1 and SHFV, all four macaques that had survived KRCV-1 injection died of SHF after subsequent SHFV injection, indicating a lack of protective heterotypic immunity. Our data indicate that SHF is a disease of macaques that in all likelihood can be caused by a number of distinct simian arteriviruses, although with different severity depending on the specific arterivirus involved. Consequently, we recommend that current screening procedures for SHFV in primate-holding facilities be modified to detect all known simian arteriviruses.

  6. Clericuzio-type Poikiloderma with Neutropenia Syndrome in a Turkish Family: a Three Report of Siblings with Mutation in the C16orf57 gene

    OpenAIRE

    Turkan Patiroglu; H Haluk Akar

    2015-01-01

    Clericuzio-type poikiloderma with neutropenia (PN) is characterized by poikiloderma, non-cyclic  neutropenia,  recurrent  sinopulmonary  infections,  pachyonychia,  and  palmo- plantar hyperkeratosis. Mutations in the C16orf57 gene, which is located on chromosome 16q13, have been identified as the cause of PN. PN was first described by Clericuzio in Navajo Indians. Herein, we reported the clinical presentations and laboratory investigations of PN in three siblings from Turkey.The older siblin...

  7. Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica).

    Science.gov (United States)

    Fioredda, Francesca; Calvillo, Michaela; Bonanomi, Sonia; Coliva, Tiziana; Tucci, Fabio; Farruggia, Piero; Pillon, Marta; Martire, Baldassarre; Ghilardi, Roberta; Ramenghi, Ugo; Renga, Daniela; Menna, Giuseppe; Barone, Angelica; Lanciotti, Marina; Dufour, Carlo

    2011-07-15

    Congenital and acquired neutropenia are rare disorders whose frequency in pediatric age may be underestimated due to remarkable differences in definition or misdiagnosed because of the lack of common practice guidelines. Neutropenia Committee of the Marrow Failure Syndrome Group (MFSG) of the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica) elaborated this document following design and methodology formerly approved by the AIEOP board. The panel of experts reviewed the literature on the topic and participated in a conference producing a document which includes a classification of neutropenia and a comprehensive guideline on diagnosis of neutropenia.

  8. Disruption of germination and seedling development in Brassica napus by mutations causing severe seed hormonal imbalance

    Directory of Open Access Journals (Sweden)

    Tung eNguyen

    2016-03-01

    Full Text Available The Brassica napus (oilseed rape accession 1012-98 shows a disturbed germination phenotype that was thought to be associated with its lack of testa pigmentation and thin seed coat. Here we demonstrate that the disturbed germination and seedling development are actually due to independent mutations that disrupt the balance of hormone metabolites and their regulators in the seeds. High-throughput UPLC-MS/MS hormone profiling of seeds and seedlings before and after germination revealed that 1012-98 has a severely disturbed hormone balance with extremely atypical, excessive quantities of auxin and ABA metabolites. The resulting hypersensitivity to abscisic acid (ABA and a corresponding increase in dormancy often results in death of the embryo after imbibition or high frequencies of disturbed, often lethal developmental phenotypes, resembling Arabidopsis mutants for the auxin regulatory factor gene ARF10 or the auxin-overproducing transgenic line iaaM-OX. Molecular cloning of Brassica ARF10 orthologues revealed four loci in normal B. napus, two derived from the Brassica A genome and two from the C genome. On the other hand, the phenotypic mutant 1012-98 exhibited amplification of C-genome BnaC.ARF10 copy number along with a chimeric allele originating from recombination between homoeologous A and C genome loci which lead to minor increase of Bna.ARF10 transcription on the critical timepoint for seed germination, the indirect regulator of ABI3, the germinative inhibitor. Bna.GH3.5 expression was upregulated to conjugate free auxin to IAA-asp between 2-6 DAS. Functional amino acid changes were also found in important DNA binding domains of one BnaC.ARF10 locus, suggesting that regulatory changes in Bna.ARF10 are collectively responsible for the observed phenotpyes in 1012-98. To our knowledge, this study is the first to report disruption of germination and seedling development in Brassica napus caused by the crosstalk of auxin-ABA and the

  9. Lacrimal punctum occlusion in the treatment of severe keratoconjunctivitis sicca caused by Sjogren syndrome - A uniocular evaluation

    NARCIS (Netherlands)

    Mansour, Khaled; Leonhardt, Carolien J.; Kalk, Wouter W.; Bootsma, Hendrika; Bruin, Klaas J.; Blanksma, Lieuwe J.

    2007-01-01

    Purpose: A controlled uniocular study to evaluate the short-term efficacy of lacrimal punctum occlusion in the treatment of severe dry eye caused by Sjogren syndrome. Methods: Uniocular punctum occlusion by punctum plug in the upper and lower puncta in 1 eye was performed in 20 patients with severe

  10. Fluconazole for empiric antifungal therapy in cancer patients with fever and neutropenia

    Directory of Open Access Journals (Sweden)

    Peterson Josh F

    2006-12-01

    Full Text Available Abstract Background Several clinical trials have demonstrated the efficacy of fluconazole as empiric antifungal therapy in cancer patients with fever and neutropenia. Our objective was to assess the frequency and resource utilization associated with treatment failure in cancer patients given empiric fluconazole antifungal therapy in routine inpatient care. Methods We performed a retrospective cohort study of cancer patients treated with oral or intravenous fluconazole between 7/97 and 6/01 in a tertiary care hospital. The final study cohort included cancer patients with neutropenia (an absolute neutrophil count below 500 cells/mm3 and fever (a temperature above 38°C or 100.4°F, who were receiving at least 96 hours of parenteral antibacterial therapy prior to initiating fluconazole. Patients' responses to empiric therapy were assessed by reviewing patient charts. Results Among 103 cancer admissions with fever and neutropenia, treatment failure after initiating empiric fluconazole antifungal therapy occurred in 41% (95% confidence interval (CI 31% – 50% of admissions. Patients with a diagnosis of hematological malignancy had increased risk of treatment failure (OR = 4.6, 95% CI 1.5 – 14.8. When treatment failure occurred the mean adjusted increases in length of stay and total costs were 7.4 days (95% CI 3.3 – 11.5 and $18,925 (95% CI 3,289 – 34,563, respectively. Conclusion Treatment failure occurred in more than one-third of neutropenic cancer patients on fluconazole as empiric antifungal treatment for fever in routine clinical treatment. The increase in costs when treatment failure occurs is substantial.

  11. Severe Spinal Cord Injury Causes Immediate Multi-cellular Dysfunction at the Chondro-Osseous Junction

    OpenAIRE

    Morse, Leslie R; XU, Yan(Department of Music,Guangxi Normal University); Solomon, Bethlehem; Boyle, Lara; Yoganathan, Subbiah; Stashenko, Philip; Battaglino, Ricardo A.

    2011-01-01

    Spinal cord injury is associated with rapid bone loss and arrested long bone growth due to mechanisms that are poorly understood. In this study, we sought to determine the effects of severe T10 contusion spinal cord injury on the sublesional bone microenvironment in adolescent rats. A severe lower thoracic (vertebral T10) spinal cord injury was generated by weight drop (10 g×50 mm). Severely injured and body weight-matched uninjured male Sprague–Dawley rats were studied. At 3 and 5 days post-...

  12. Severe chronic osteomyelitis caused by Morganella morganii with high population diversity

    Directory of Open Access Journals (Sweden)

    Jialiang Zhu

    2016-09-01

    Full Text Available A case of chronic osteomyelitis probably caused by Morganella morganii, occurring over a period of 30 years, is reported. The organism was identified through a combination of sample culture, direct sequencing, and 16S RNA gene amplicon sequencing. Further whole-genome sequencing and population structure analysis of the isolates from the patient showed the bacterial population to be highly diverse. This case provides a valuable example of a long-term infection caused by an opportunistic pathogen, M. morganii, with high diversity, which might evolve during replication within the host.

  13. Severe chronic osteomyelitis caused by Morganella morganii with high population diversity.

    Science.gov (United States)

    Zhu, Jialiang; Li, Haifeng; Feng, Li; Yang, Min; Yang, Ronggong; Yang, Lin; Li, Li; Li, Ruoyan; Liu, Minshan; Hou, Shuxun; Ke, Yuehua; Li, Wenfeng; Bai, Fan

    2016-09-01

    A case of chronic osteomyelitis probably caused by Morganella morganii, occurring over a period of 30 years, is reported. The organism was identified through a combination of sample culture, direct sequencing, and 16S RNA gene amplicon sequencing. Further whole-genome sequencing and population structure analysis of the isolates from the patient showed the bacterial population to be highly diverse. This case provides a valuable example of a long-term infection caused by an opportunistic pathogen, M. morganii, with high diversity, which might evolve during replication within the host.

  14. Severe tissue destruction in the ear caused by alkaline button batteries.

    OpenAIRE

    Premachandra, D. J.; McRae, D.

    1990-01-01

    Button batteries spontaneously leak corrosive electrolyte solution on exposure to moisture. Tissue in contact with such solution will undergo liquefaction necrosis. Three cases of skin, bone and tympanic membrane necrosis caused by a leaking button battery lodged in the external auditory meatus are described.

  15. Severe renal bleeding caused by a ruptured renal sheath: case report of a rare complication of percutaneous nephrolithotomy

    Directory of Open Access Journals (Sweden)

    Gunes Ali

    2002-09-01

    Full Text Available Abstract Background Percutaneous nephrolithotomy is a minimally invasive intervention for renal stone disease. Complications, which are rare and usually presented as case reports, are diversified as the utilization of the procedure is expanded. The procedure causes less blood loss and less morbidity when compared to open surgical procedures. Yet, there are some reports involving severe bleeding and relevant morbidity during surgery. These are usually related with the surgical technique or experience of the surgeon. Renal sheaths are designed to cause minimal trauma inside the kidney and, to our knowledge, there are no reports presenting the rupture of a sheath causing severe bleeding during the procedure. Case report We present an adult patient who had severe bleeding during percutaneous nephrolithotomy due to parenchymal injury caused by a ruptured renal sheath. During retrieval, due probably to rough handling of the equipment, a piece of stone with serrated edges ruptured the tip of the sheath, and this tip caused damage inside the kidney. The operation was terminated and measures were taken to control bleeding. The patient was transfused with a total of 1600 ml of blood, and the stones were cleared in a second look operation. Conclusion Although considered to be a minimally invasive procedure, some unexpected complications may arise during percutaneous nephrolithotomy. After being fragmanted, stone pieces may damage surgical equipment, causing acute and severe harm to the kidney. Surgeons must manipulate the equipment with fine and careful movements in order to prevent this situation.

  16. Neutropenia, neutrophil dysfunction, and inflammatory bowel disease in glycogen storage disease type Ib : Results of the European Study on Glycogen Storage Disease Type I

    NARCIS (Netherlands)

    Visser, G; Rake, JP; Fernandes, J; Labrune, P; Leonard, JV; Moses, S; Ullrich, K; Smit, GPA

    2000-01-01

    Objective: To investigate the incidence, the severity, and the course of neutropenia, neutrophil dysfunction, and inflammatory bowel disease (IBD) in glycogen storage disease (GSD) type Ib. Method: As part of a collaborative European Study on GSD type I, a retrospective registry was established in 1

  17. Aberrant coagulation causes a hyper-inflammatory response in severe influenza pneumonia.

    Science.gov (United States)

    Yang, Yan; Tang, Hong

    2016-07-01

    Influenza A virus (IAV) infects the respiratory tract in humans and causes significant morbidity and mortality worldwide each year. Aggressive inflammation, known as a cytokine storm, is thought to cause most of the damage in the lungs during IAV infection. Dysfunctional coagulation is a common complication in pathogenic influenza, manifested by lung endothelial activation, vascular leak, disseminated intravascular coagulation and pulmonary microembolism. Importantly, emerging evidence shows that an uncontrolled coagulation system, including both the cellular (endothelial cells and platelets) and protein (coagulation factors, anticoagulants and fibrinolysis proteases) components, contributes to the pathogenesis of influenza by augmenting viral replication and immune pathogenesis. In this review, we focus on the underlying mechanisms of the dysfunctional coagulatory response in the pathogenesis of IAV. PMID:27041635

  18. Premature closure of the upper esophageal sphincter as a cause of severe deglutition disorder in infancy

    DEFF Research Database (Denmark)

    Nielsen, Rasmus; Husby, Steffen; Kruse-Andersen, Søren

    2005-01-01

    Deglutition disorders in infancy are often associated with birth asphyxia or structural abnormalities in the hypopharynx, the trachea, or the esophagus. Manometry can be crucial for clarifying the dynamics of the swallowing disorder in the infant with deglutition problems and without signs...... of these causes. An 8-week-old infant was referred because of suspicion of cricopharyngeal achalasia causing persistent swallowing problems and failure to thrive. Manometry results showed normal resting tone and relaxation but premature closure of the upper esophageal sphincter. The infant was treated...... with balloon dilatation of the upper esophageal sphincter and expectance. A maturation process of the swallowing sequence was noted over time and documented by repeated manometric procedures....

  19. Celiac disease causing severe osteomalacia: an association still present in Morocco!

    OpenAIRE

    Tahiri, Latifa; Azzouzi, Hamida; Squalli, Ghita; Abourazzak, Fatimazahra; Harzy, Taoufik

    2014-01-01

    Celiac disease (CD), a malabsorption syndrome caused by hypersensitivity to gliadin fraction of gluten. CD can manifest with classic symptoms; however, significant myopathy and multiple fractures are rarely the predominant presentation of untreated celiac disease. Osteomalacia complicating celiac disease had become more and more rare. We describe here a case of osteomalacia secondary to a longstanding untreated celiac disease. This patient complained about progressive bone and muscular pain, ...

  20. Staphylococcus aureus seroproteomes discriminate ruminant isolates causing mild or severe mastitis

    OpenAIRE

    Le Maréchal Caroline; Jardin Julien; Jan Gwenaël; Even Sergine; Pulido Coralie; Guibert Jean-Michel; Hernandez David; François Patrice; Schrenzel Jacques; Demon Dieter; Meyer Evelyne; Berkova Nadia; Thiéry Richard; Vautor Eric; Le Loir Yves

    2011-01-01

    Abstract Staphylococcus aureus is a major cause of mastitis in ruminants. In ewe mastitis, symptoms range from subclinical to gangrenous mastitis. S. aureus factors or host-factors contributing to the different outcomes are not completely elucidated. In this study, experimental mastitis was induced on primiparous ewes using two S. aureus strains, isolated from gangrenous (strain O11) or subclinical (strain O46) mastitis. Strains induced drastically distinct clinical symptoms when tested in ew...

  1. Severe community-acquired pneumonia caused by Mycoplasma pneumoniae in young female patient

    Directory of Open Access Journals (Sweden)

    Milancic Nena

    2015-09-01

    Full Text Available Mycoplasma pneumonia is common agent causing community acquired pneumonia in younger population. However, the course of illness is usually benign and is rarely associated with pulmonary complications. We report a 27 years old female patient with unilateral pneumonia followed by pleural effusion and adhesions on the same side. This potential source of infection should be considered in young patients where resolution of symptoms from pneumonia is delayed.

  2. Severe disseminated phaeohyphomycosis in an immunocompetent patient caused by Veronaea botryosa

    NARCIS (Netherlands)

    A. Bonifaz; M.M. Davoudi; G.S. de Hoog; C. Padilla-Desgarennes; D. Vázquez-González; G. Navarrete; J.F. Meis; H. Badali

    2013-01-01

    We present a severe case of disseminated phaeohyphomycosis due to Veronaea botryosa. A 32-year-old female, native from Cuautla, Morelos, Mexico, presented a chronic dermatosis which started 10 years earlier with multiple exophytic, multilobulated, soft, and pedunculated or sessile neoformations of d

  3. Evaluation of febrile neutropenia in patients undergoing hematopoietic stem cell transplantation.

    Directory of Open Access Journals (Sweden)

    Shahideh Amini

    2014-01-01

    Full Text Available The aim of this study was to determine the incidence and causes of fever as a major problem contributing to transplantation related mortality among patients undergoing hematopoietic stem cell transplantation (HSCT and evaluation of antibiotic use, according to reliable guidelines.We retrospectively reviewed hospital records of 195 adult patients who underwent HSCT between 2009-2011 at hematology-oncology and bone marrow transplantation research center. Baseline information and also data related to fever and neutropenia, patient's outcomes, duration of hospitalization and antibiotic use pattern were documented.A total of 195 patients were analyzed and a total of 268 febrile episodes in 180 patients were recorded (mean 1.5 episodes per patient. About 222 episodes (82% were associated with neutropenia which one-fourth of them were without any documented infection sources. Microbiologic documents showed that the relative frequencies of gram positive and gram negative bacteria were 62.5% and 37.5%, respectively. The hospital stay duration was directly related to the numbers of fever episodes (P<0.0001.The rate of febrile episodes in autologous stem cell transplantation was significantly higher compared to allogeneic type (P<0.05.It is necessary to determine not only the local profile of microbiologic pattern, but also antibiotic sensitivities in febrile neutropenic patients following hematopoietic stem cell transplantation, and reassess response to antibiotic treatment to establish any necessity for modifications to treatment guidelines in order to prevent any fatal complications from infection.

  4. Hijos de madre toxémica: neutropenia y trombocitopenia.

    OpenAIRE

    Fabio D. Pereira; Reynaldo Miranda; Carmen de Rosero; Jorge Estupiñán

    2009-01-01

    La enfermedad hipertensiva del embarazo, particulamente la toxemia, se ha asociado con neutropenia y trombocitopenia en el recién nacido. En un estudio previo se había encontrado muy poca asociación con estos eventos. En la presente investigación, mediante métodos hematológicos, se estudiaron 70 hijos de madres con toxemia severa los días 1 y 5 de vida; sólo 44 niños asistieron a control el día 5. Se encontró que la trombocitopenia en la práctica era inexistente y que la neutropenia fue más e...

  5. Hijos de madre toxémica: neutropenia y trombocitopenia.

    Directory of Open Access Journals (Sweden)

    Fabio D. Pereira

    2009-09-01

    Full Text Available La enfermedad hipertensiva del embarazo, particulamente la toxemia, se ha asociado con neutropenia y trombocitopenia en el recién nacido. En un estudio previo se había encontrado muy poca asociación con estos eventos. En la presente investigación, mediante métodos hematológicos, se estudiaron 70 hijos de madres con toxemia severa los días 1 y 5 de vida; sólo 44 niños asistieron a control el día 5. Se encontró que la trombocitopenia en la práctica era inexistente y que la neutropenia fue más escasa de lo informado en la literatura.

  6. X-ray analysis of 80 patients with severe endemic fluorosis caused by coal burning

    Energy Technology Data Exchange (ETDEWEB)

    Zhao, Z.P.; Yuan, M.B.; Liu, G.F. [Luzhou Medical College, Luzhou (China)

    1996-05-01

    Radiographs of 80 patients with severe endemic fluorosis of coalburning type (CBFF) - 49 males and 31 females aged 30 to 70 years - were analysed to examine the changes to the bone substance, peripheral structure of bone, and joints. The changes to bone substance were: (1) osteosclerosis type, 62 cases (77.5%); (2) mixed type, 16 cases (21.25%); (3) osteoporosis type, one case (1.25%); (4) osteomalacia type, one case (1.25%). The changes to the joints were found in the hips and elbows in 79 cases (98.75%), and in the knees in 75 cases (93.75%). When combinations of the above three changes occur, the classification of the disease is according to the most severe one of the three. Our findings can increase the accuracy of X-ray diagnosis, making it more consistent with clinical diagnosis, thus improving prevention and treatment of CBEF.

  7. Severe esophageal injuries caused by accidental button battery ingestion in children

    OpenAIRE

    Sara Fuentes; Indalecio Cano; María Isabel Benavent; Andrés Gómez

    2014-01-01

    Introduction: Button batteries represent a low percentage of all foreign bodies swallowed by children and esophageal location is even less frequent. However, these cases are more likely to develop severe injuries. The aim of this essay is to report three cases treated in our institution and review previous reports. Material and Methods: Chart review and literature search. Case Reports: We treated three children between 2-7- years old with button batteries lodged at esophagus. They all present...

  8. Linoleic acid metabolite drives severe asthma by causing airway epithelial injury

    OpenAIRE

    Mabalirajan, Ulaganathan; Rehman, Rakhshinda; Ahmad, Tanveer; Kumar, Sarvesh; Singh, Suchita; Leishangthem, Geeta D.; Aich, Jyotirmoi; Kumar, Manish; Khanna, Kritika; Singh, Vijay P.; Dinda, Amit K; Biswal, Shyam; Agrawal, Anurag; Ghosh, Balaram

    2013-01-01

    Airway epithelial injury is the hallmark of various respiratory diseases, but its mechanisms remain poorly understood. While 13-S-hydroxyoctadecadienoic acid (13-S-HODE) is produced in high concentration during mitochondrial degradation in reticulocytes little is known about its role in asthma pathogenesis. Here, we show that extracellular 13-S-HODE induces mitochondrial dysfunction and airway epithelial apoptosis. This is associated with features of severe airway obstruction, lung remodeling...

  9. Severe diabetes and leptin resistance cause differential hepatic and renal transporter expression in mice

    OpenAIRE

    More, Vijay R.; Wen, Xia; Thomas, Paul E.; Aleksunes, Lauren M.; Slitt, Angela L.

    2012-01-01

    Background Type-2 Diabetes is a major health concern in the United States and other Westernized countries, with prevalence increasing yearly. There is a need to better model and predict adverse drug reactions, drug-induced liver injury, and drug efficacy in this population. Because transporters significantly contribute to drug clearance and disposition, it is highly significant to determine whether a severe diabetes phenotype alters drug transporter expression, and whether diabetic mouse mode...

  10. Haemorrhagic pancreatitis--a cause of death in severe potassium permanganate poisoning.

    OpenAIRE

    Middleton, S J; Jacyna, M.; McClaren, D.; Robinson, R; Thomas, H C

    1990-01-01

    Severe potassium permanganate poisoning (more than 10 g of potassium permanganate) is invariably associated with massive systemic upset and death. Multiple organ damage has been recognized as an inevitable consequence of such an overdose, although pancreatitis has not been previously reported. Death due to cardiovascular collapse and profound hypotension is a common end point in those who reach hospital, but the pathogenesis is uncertain. We report a case of haemorrhagic pancreatitis followin...

  11. Alkaptonuria: A rare cause of recurrent severe back pain in the emergency department

    Directory of Open Access Journals (Sweden)

    MSeidahmed

    2012-09-01

    Full Text Available We report a 45 year-old male patient who presented to the emergency department of Hamad General Hospital with recurrent severe low back pain. Clinical examination revealed characteristic deposition of blue-brownish pigment in the sclera and ear. X-ray revealed diffuse intervertebral disc calcification. Alkaptonuria was suspected and the diagnosis was confirmed by detection of high levels of homogentisic acid in the urine.

  12. One-stage treatment of acquired facial deformity caused by severe unilateral condylar hyperplasia.

    Science.gov (United States)

    Butt, Fawzia M A; Guthua, Simon Wangombe; Nganga, Peter; Edalia, Michael; Dimba, Elizabeth A O

    2011-09-01

    Condylar hyperplasia (CH) is a rare and self-limiting process manifesting between the first and third decades of life. It causes facial asymmetry and derangement of occlusion. The management involves resection of the condylar head and orthognathic surgery followed by orthodontic treatment. We present a 33-year-old man with spontaneous onset of CH during a span of 10 years. This was managed with resection of the condyle alone, which dramatically improved facial asymmetry in addition to restoration of the occlusion within a few months of follow-up. Therefore, orthognathic surgery or orthodontic treatment was not needed. PMID:21959484

  13. Severe Acute Infection Due to Serratia marcescens Causing Respiratory Distress in An Immunocompetent Adult.

    Science.gov (United States)

    Ruiz-Sada, Pablo; Escalante, Mikel; Lizarralde, Eva

    2016-01-01

    The role of Serratia marcescens changed from a harmless saprophytic microorganism to an important opportunistic human pathogen. It often causes nosocomial device-associated outbreaks and rarely serious invasive community acquired infections. We present a case of a community-acquired Serratia marcescens bacteremia leading to Respiratory Distress Syndrome in a previously healthy 51-year-old man without identifiable risk factors. Full recovery was achieved with solely medical treatment and observation in ICU during three days. To our knowledge it is an extremely uncommon presentation and just few cases have been previously reported in the literature.

  14. 中性粒细胞弹性蛋白酶基因突变致先天性粒细胞减少症2例并文献复习%Two Chinese cases of congenital neutropenia caused by ELANE gene mutations and literature review

    Institute of Scientific and Technical Information of China (English)

    侯佳; 王莹; 刘丹如; 应文静; 孙金峤; 惠晓莹; 王晓川

    2014-01-01

    Objective To identify ELANE gene mutations in 2 Chinese cases with congenital neutropenia and to better understand the clinical characters,diagnosis and treatment of this rare disease. Methods Clinical data of the two cases with congenital neutropenia were collected,including clinical manifestations,trends of the absolute neutrophil count( ANC ),and immunological function. All 5 exons and flanking regions of ELANE gene were sequenced for the two cases and their families. Results Two cases were diagnosed as neutropenia at the age of 3 months and 1 month respectively,characterized with recurrent infections,including recurrent pneumonia in one case and oral mucosa ulcer in the other. Two cases presented with persistent neutropenia with the ANC less than 1. 5 × 109 ·L-1 ,the lowest ANC reached 0. 01 × 109 ·L-1 for case 1 and 0. 09 × 109 ·L-1 for case 2,respectively. Screening of blood serum and bone marrow was performed to exclude pathogenic infection,autoimmune disease and hematological malignancies. The respiratory burst of neutrophils and cellular immune function of both cases were normal,except for the elevated serum IgG level. Case 1 had c. 661G>GT(p. G221GX)heterozygous nonsense mutation in ELANE gene,case 2 had c. 377C>CT(p. S126SL)heterozygous missense mutation. No mutation was found in their family members. Case 2 received hematopoietic stem cell transplantation( HSCT)and presented with normal ELANE gene afterwards,case 1 was treated with G-CSF,both were followed up. Conclusion ELANE gene is the critical pathogenic gene for congenital neutropenia. HSCT is the effective radical treatment for this rare disease.%目的:总结2例中性粒细胞弹性蛋白酶( ELANE)基因突变的先天性粒细胞减少症患儿的临床特征、基因诊断及治疗方法,提高对该病的认识。方法分析2例先天性粒细胞减少症患儿的临床表现,外周血中性粒细胞绝对计数( ANC)变化,中性粒细胞呼吸爆发功能及其体

  15. Biosimilars in the management of neutropenia: focus on filgrastim

    Directory of Open Access Journals (Sweden)

    Caselli D

    2016-02-01

    Full Text Available Désirée Caselli,1 Simone Cesaro,2 Maurizio Aricò1 1Medical Department, Pediatric Unit, Azienda Sanitaria Provinciale Ragusa, Ragusa, 2Department of Pediatrics, Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy Abstract: Advances in chemotherapy and surgery allows the majority of patients to survive cancer diseases. Yet, the price may be a proportion of patients dying of complications due to treatment-induced infectious complications, such as neutropenia. With the aim of decreasing morbidity and mortality related to infectious complications, recombinant human granulocyte colony-stimulating factor (G-CSF, filgrastim, and pegylated filgrastim have been used to reduce time and degree of neutropenia. A biosimilar is a copy of an approved original biologic medicine whose data protection has expired. The patent for filgrastim expired in Europe in 2006 and in the US in 2013. This review analyses the available evidence to be considered in order to design a strategy of use of G-CSF and its biosimilars. The clinical and safety outcomes of biosimilars are well within the range of historically reported data for originator filgrastim. This underscores the clinical effectiveness and safety of biosimilar filgrastim in daily clinical practice. Biosimilars can play an important role by offering the opportunity to reduce costs, thus contributing to the financial sustainability of treatment programs. Keywords: neutropenia, filgrastim, biosimilars, G-CSF, fever, prophylaxis

  16. Neutropenia in rheumatoid arthritis and large granular lymphocyte leucosis

    Directory of Open Access Journals (Sweden)

    V A Doronin

    2003-01-01

    Full Text Available Objective. Pts with chronic clonal proliferation of large granular lymphocytes (LGL leukemia often have neutropenia, splenomegaly, and rheumatoid arthritis (RA, thereby resembling the manifestations observed in pts with Felty’s syndrome. The present study sought to indicate that pts with these disorders represent two distinct subsets. We compare clinical, hematological, immunophenotiping and immunogenetic features in Felty’s syndrome pts with and without the LGL leukemia. Material and methods 10 pts with T-LGL leukemia were studied. Surface phenotype was estimated using monoclonal antibodies CD8-PE and CD3-FITC/CD16-PE (two-color (Caltag, USA by the flow cytometric analysis (Partec, Daco. Analysis of TCR gene rearrangement was performed by using PCR-LIS SSCP (low ionic strength single strand conformational polymorphism. Comparison with Felty s syndrome and RA pts based on the review of literature. Results. LGL leukemia is a distinct clinicopathologic entity often associated with RA. LGL leukemia pts with RA showed the same immunogenetis associations seen in RA/Felty’s syndrome, while LGL leukemia pts without arthritis did not. Conclusion. Hematologic, immunophenotyping and molecular genetic analysis are very important and highly representative tools in differential diagnosis of neutropenia in RA, and propose that Felty’s syndrome and LGL leukemia represent different variants of broader syndrome comprising RA, neutropenia, LGL expansions, and splenomegaly.

  17. Mild and severe muscular dystrophy caused by a single {gamma}-sarcoglycan mutation

    Energy Technology Data Exchange (ETDEWEB)

    McNally, E.M.; Boennemann, C.G.; Lidov, H.G.W. [Brigham and Women`s Hospital, Boston, MA (United States)] [and others

    1996-11-01

    Autosomal recessive muscular dystrophy is genetically heterogeneous. One form of this disorder, limb-girdle muscular dystrophy type 2C (LGMD 2C), is prevalent in northern Africa and has been shown to be associated with a single mutation in the gene encoding the dystrophin-associated protein {gamma}-sarcoglycan. The previous mutation analysis of {gamma}-sarcoglycan required the availability of muscle biopsies. To establish a mutation assay for genomic DNA, the intron-exon structure of the {gamma}-sarcoglycan gene was determined, and primers were designed to amplify each of the exons encoding {gamma}-sarcoglycan. We studied a group of Brazilian muscular dystrophy patients for mutations in the {gamma}-sarcoglycan gene. These patients were selected on the basis of autosomal inheritance and/or the presence of normal dystrophin and/or deficiency of {alpha}-sarcoglycan immunostaining. Four of 19 patients surveyed had a single, homozygous mutation in the {gamma}-sarcoglycan gene. The mutation identified in these patients, all of African-Brazilian descent, is identical to that seen in the North African population, suggesting that even patients of remote African descent may carry this mutation. The phenotype in these patients varied considerably. Of four families with an identical mutation, three have a severe Duchenne-like muscular dystrophy. However, one family has much milder symptoms, suggesting that other loci may be present that modify the severity of the clinical course resulting from {gamma}-sarcoglycan gene mutations. 19 refs., 5 figs., 3 tabs.

  18. [Rare infection--prolonged A. naeslundii bacteremia caused by severe caries].

    Science.gov (United States)

    Abozaid, Said; Peretz, Avi; Nasser, Wael; Zarfin, Yehoshua

    2013-07-01

    Actinomyces is an anaerobic, gram positive, rod shape bacteria that doesn't create spores. Actinomyces is part of the mouth, intestines, vagina and upper respiratory system flora. The infection appears mostly on the face, neck, abdomen and pelvis in cases of mucosa injury and most common in immunosuppressed patients. The spread of Actinomyces through the blood system is rare. In this article we present a 9 year old male patient with no history of diseases who was diagnosed with prolonged bacteremia of A. naeslundii without specific infection excluding severe caries. Characterization of bacteria from the blood culture was performed by molecular biology and the patient was treated with Ampicillin and tooth extraction that led to the disappearance of the bacteremia. PMID:23957079

  19. Dilated aortic root and severe aortic regurgitation causing dilated cardiomyopathy in classic Ehlers-Danlos syndrome.

    Science.gov (United States)

    Zainal, Abir; Hamad, Mahmoud Nidal; Naqvi, Syed Yaseen

    2016-01-01

    Ehlers-Danlos syndrome (EDS) is a group of heritable disorders characterised by vast clinical heterogeneity ranging from the classic constellation of symptoms including skin hyperextensibility, joint hypermobility and skin fragility to the exceedingly critical consequences of arterial rupture and visceral perforation. We describe the case of a 65-year-old male with a history of classic EDS who reported of dyspnoea on exertion, orthopnoea, fatigue and palpitations. He was found to have dilated cardiomyopathy with an ejection fraction of 35%, aortic root dilation and severe aortic valve regurgitation. The authors intend to draw attention to the rare cardiac manifestations of this condition and the therapeutic challenges involved in managing such patients. PMID:27413024

  20. Severe necrotizing myocarditis caused by serratia marcescens infection in an axolotl (Ambystoma mexicanum).

    Science.gov (United States)

    Del-Pozo, J; Girling, S; Pizzi, R; Mancinelli, E; Else, R W

    2011-05-01

    This report provides the first account of the pathological changes associated with infection by Serratia marcescens in an adult male axolotl. The infection resulted in septicaemia with severe multifocal necrotizing myocarditis. The latter lesion evolved to cardiac rupture, haemopericardium and death resulting from cardiac tamponade. This animal was exposed to higher than usual temperatures (24-25 °C) 2 weeks before the onset of disease and this may have resulted in immunocompromise and opportunistic bacterial infection. S. marcescens was isolated from the coelomic and pericardial cavity. Both isolates were identical and were resistant to β-lactam antibiotics, but not to aminoglycosides or fluoroquinolones. The production of red prodigiosin pigment by the bacterium suggested an environmental origin. Overall, the clinical and histopathological presentation suggests that S. marcescens should be included in the list of aetiological agents of the 'red-leg'/bacterial dermatosepticaemia syndrome of amphibians.

  1. An outbreak in 1965 of severe respiratory illness caused by the Legionnaires' disease bacterium.

    Science.gov (United States)

    Thacker, S B; Bennett, J V; Tsai, T F; Fraser, D W; McDade, J E; Shepard, C C; Williams, K H; Stuart, W H; Dull, H B; Eickhoff, T C

    1978-10-01

    In January 1977 an unsolved outbreak of infection at St. Elizabeth's Hospital (Washington, D.C.) that occurred in 1965 was linked with Legionnaires' disease. The link was made by fluorescent antibody testing with the bacterium isolated from tissues of persons with Legionnaires' disease in the 1976 outbreak in Philadelphia. In July and August 1965, an epidemic of severe respiratory disease characterized by abrupt onset of high fever, weakness, malaise, and nonproductive cough, frequently accompanied by radiographic evidence of pneumonia, affected at least 81 patients at St. Elizabeth's Hospital, a general psychiatric hospital. Fourteen (17%) of the affected patients died. Intensive epidemiologic and laboratory investigations in 1965 did not determine the etiology. The etiologic organism may have become airborne from sites of soil excavation. PMID:361897

  2. NK cells are intrinsically functional in pigs with Severe Combined Immunodeficiency (SCID) caused by spontaneous mutations in the Artemis gene

    Science.gov (United States)

    We have identified Severe Combined Immunodeficiency (SCID) in a line of Yorkshire pigs at Iowa State University. These SCID pigs lack B-cells and T-cells, but possess Natural Killer (NK) cells. This SCID phenotype is caused by recessive mutations in the Artemis gene. Interestingly, two human tumor c...

  3. Gingival overgrowth caused by vitamin C deficiency associated with metabolic syndrome and severe periodontal infection: a case report.

    Science.gov (United States)

    Omori, Kazuhiro; Hanayama, Yoshihisa; Naruishi, Koji; Akiyama, Kentaro; Maeda, Hiroshi; Otsuka, Fumio; Takashiba, Shogo

    2014-12-01

    It has been suggested that vitamin C deficiency/scurvy is associated with gingival inflammatory changes; however, the disorder is very infrequently encountered in the modern era. Here, we report a case of extensive gingival overgrowth caused by vitamin C deficiency associated with metabolic syndrome and severe periodontal infection. PMID:25548632

  4. Gingival overgrowth caused by vitamin C deficiency associated with metabolic syndrome and severe periodontal infection: a case report

    OpenAIRE

    Omori, Kazuhiro; Hanayama, Yoshihisa; Naruishi, Koji; Akiyama, Kentaro; Maeda, Hiroshi; Otsuka, Fumio; Takashiba, Shogo

    2014-01-01

    It has been suggested that vitamin C deficiency/scurvy is associated with gingival inflammatory changes; however, the disorder is very infrequently encountered in the modern era. Here, we report a case of extensive gingival overgrowth caused by vitamin C deficiency associated with metabolic syndrome and severe periodontal infection.

  5. [Life threatening salicylate poisoning caused by percutaneous absorption in severe ichthyosis vulgaris].

    Science.gov (United States)

    Germann, R; Schindera, I; Kuch, M; Seitz, U; Altmeyer, S; Schindera, F

    1996-08-01

    In a 7-year-old boy, ichthyosis vulgaris was treated with a 10% ointment for application over a large area of the body surface. In this way, the child received 400 g salicylic acid (0.6 g/kg body weight per day) percutaneously over a period of 4 weeks. The patient was referred to hospital by the family doctor: he was in a deep somnolent state, apparently caused by hyperventilation following wheezing, vomiting, tinnitus and vertigo. Salicylate intoxication was suspected because of metabolic acidosis, an anion gap and respiratory overcompensation. The diagnosis was confirmed by a serum salicylate level of 985 micrograms/ml (therapeutic level 150-300 micrograms/ml). Following forced diuresis and alkalization with sodium bicarbonate, haemodialysis was unnecessary. As the salicylate level declined to values within the therapeutic range, the patient started to recover consciousness, waking on the 4th day. By day 6 there were still obvious neurological deficiencies. Fecal incontinence, bilateral ptosis and intermittent diverging strabismus on the right persisted for some weeks. It was 6 months before complete neurological resolution was achieved. The pathogenesis of salicylate toxicity and the need for safer therapies for ichthyosis vulgaris are discussed.

  6. [Severe myocardial damage caused by leptospirosis. Report of a fatal case in Mexico].

    Science.gov (United States)

    Velasco-Castrejón, Oscar; Rivas-Sánchez, Beatriz; Soriano-Rosas, Juan; Rivera-Reyes, Héctor Hugo

    2009-01-01

    Chagasic cardiomyopathy is a common disease in Latin America, however similar clinical pictures exist that can be confused with this, as they give negative results to the tests that detects T. cruzi, like non Chagasic rural endemic chronic cardiopathy, highly common in Venezuela. Using histopathology techniques, "idiopathic cardiomyopathy" is frequently found as the cause of death when the etiology of this disease is not known. This paper presents the case of a man of 26-years-old who died of dilated cardiomyopathy secondary to leptospirosis. Clinically, in addition to the cardiac failure, jaundice, hyperbilirubinemia, transaminases increase, proteinuria and hematuria were present. Initially it was suspected Chagasic cardiomyopathy but the epidemiologic background and the parasitologic and serologic tests for T. cruzi gave negative results. The dark field microscopy videorecording of blood and urine samples, argentic impregnation and immunohistochemistry tests as well as haemoculture in EMJH medium were positive for L. interrogans serovar Pomona. Postmortem we confirmed the presence of leptospira in different tissues through of histopathology, argentic impregnation, indirect immunofluorescence and immunohistochemistry. PMID:20191987

  7. An oblique muscle hematoma as a rare cause of severe abdominal pain: a case report

    Directory of Open Access Journals (Sweden)

    Shimodaira Masanori

    2013-01-01

    Full Text Available Abstract Background Abdominal wall hematomas are an uncommon cause of acute abdominal pain and are often misdiagnosed. They are more common in elderly individuals, particularly in those under anticoagulant therapy. Most abdominal wall hematomas occur in the rectus sheath, and hematomas within the oblique muscle are very rare and are poorly described in the literature. Here we report the case of an oblique muscle hematoma in a middle-aged patient who was not under anticoagulant therapy. Case presentation A 42-year-old Japanese man presented with a painful, enlarging, lateral abdominal wall mass, which appeared after playing baseball. Abdominal computed tomography and ultrasonography showed a large soft tissue mass located in the patient’s left internal oblique muscle. A diagnosis of a lateral oblique muscle hematoma was made and the patient was treated conservatively. Conclusion Physicians should consider an oblique muscle hematoma during the initial differential diagnosis of pain in the lateral abdominal wall even in the absence of anticoagulant therapy or trauma.

  8. Outcome of Severe Dengue Viral Infection-caused Acute Liver Failure in Thai Children.

    Science.gov (United States)

    Laoprasopwattana, Kamolwish; Jundee, Puthachat; Pruekprasert, Pornpimol; Geater, Alan

    2016-06-01

    To determine clinical course and outcomes of liver functions in children with dengue viral infection-caused acute liver failure (ALF), the records of patients aged dengue hemorrhagic fever grade II, III and IV, respectively. Multiorgan failure including respiratory failure, massive bleeding and acute kidney injury occurred in 80.0%, 96.0% and 84.0% of the ALF cases, respectively, with an overall fatality rate of 68.3%. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels were highest on the day that the patient developed ALF. Lactate dehydrogenase levels had positive correlations with AST (r = 0.95) and ALT (r = 0.87) (all p < 0.01). The median (interquartile range) days before the AST and ALT levels returned to lower than 200 U/L after the ALF were 10.5 (8.8, 12.8) and 10.5 (7.8, 14.0) days, respectively. PMID:26851434

  9. Treatment of Febrile Neutropenia and Prophylaxis in Hematologic Malignancies: A Critical Review and Update

    Directory of Open Access Journals (Sweden)

    Paola Villafuerte-Gutierrez

    2014-01-01

    Full Text Available Febrile neutropenia is one of the most serious complications in patients with haematological malignancies and chemotherapy. A prompt identification of infection and empirical antibiotic therapy can prolong survival. This paper reviews the guidelines about febrile neutropenia in the setting of hematologic malignancies, providing an overview of the definition of fever and neutropenia, and categories of risk assessment, management of infections, and prophylaxis.

  10. Febrile neutropenia in children with acute lymphoblastic leukemia: single center experience

    Science.gov (United States)

    Özdemir, Nihal; Tüysüz, Gülen; Çelik, Nigar; Yantri, Leman; Erginöz, Ethem; Apak, Hilmi; Özkan, Alp; Yıldız, İnci; Celkan, Tiraje

    2016-01-01

    Aim: An important life-threatening complication of intensive chemotherapy administered in children with leukemia is febrile neutropenia. The objective of this study was to evaluate the clinical features and consequences of febrile neutropenia attacks in children who were treated for acute lymphoblastic leukemia. Material and Methods: Nighty-six children who received chemotherapy for acute lymphoblastic leukemia in our center between January 1995 and December 2010 were included in the study. The data related to demographic characteristics, treatment features, relapse and febrile neutropenia incidences, risk factors, culture results and prognosis were retrospectively evaluated from the patients’ files. Results: A total of two hundred-ninety nine febrile neutropenia attacks observed in the patients during initial treatment and relapse treatment were evaluated. When the incidence of febrile neutropenia was evaluated by years, it was observed that the patients treated after year 2000 had statistically significantly more febrile neutopenia attacks compared to the patients treated before year 2000. When the incidences of febrile neutropenia during initial treatment and during relapse treatment were compared, it was observed that more febrile neutropenia attacks occured during relapse treatment. Fifty-nine percent of all febrile neutropenia attacks were fever of unknown origin. Eighty microorganisms grew in cultures during febrile neutropenia throughout treatment in 75 patients; 86% were bacterial infections (50% gram positive and 50% gram negative), 8% were viral infections and 6% were fungal infections. Coagulase negative staphylococcus (n=17) was the most frequent gram positive pathogen; E. Coli (n=17) was the most commonly grown gram negative pathogen. Conclusions: In this study, it was found that an increase in the incidence of febrile neutropenia occured in years. Increments in treatment intensities increase the incidence of febrile neutropenia while improving

  11. Impact of severe disease caused by respiratory syncytial virus in children living in developed countries.

    Science.gov (United States)

    Simoes, Eric A; Carbonell-Estrany, Xavier

    2003-02-01

    Among industrialized nations, the rate of rehospitalization in the United States for respiratory syncytial virus (RSV) is approximately 30 per 1000, exceptions being noted for American Indians and Alaskan natives, two ethnic groups who tend toward higher rates of RSV hospitalization. In distinction Japan reports an admission rate of 60 per 1000 for RSV disease. Yet Japan ranks considerably lower than many of its western counterparts in premature births. Whether an RSV subtype, a new viral genotype or some other unifying characteristic exists that might explain the severity of adenovirus, parainfluenza and RSV infections in this region of Asia remains to be determined. Outcomes trials in the United States, Canada, United Kingdom, Denmark and Japan all identified crowding and exposure to tobacco smoke as significant and independent risk factors for disease severity of RSV. The epidemiology of RSV is largely consistent throughout Europe, with peak outbreaks occurring in December and January. In Europe RSV accounts for 42 to 45% of hospital admissions for lower respiratory tract infections in children younger than 2 years of age, and inpatient populations tend to be younger and to experience greater disease severity. For RSV bronchiolitis lengths of stay in European hospitals range from a low of 4 days to a high of 10 days. The Infección Respiratoria Infantil por Virus Respiratorio Sincitial Study Group in Spain conducted 2 prospective observational studies in 14 and 26 neonatal units, respectively, on nonprophylaxed neonates to determine hospitalization rates for respiratory syncytial viral illness during 2 consecutive RSV seasons. Throughout each respiratory season the study group followed premature infants of < or =32 weeks gestational age at birth, representing an annual birth cohort of approximately 100 000 infants. A total of 584 infants who were < or =32 weeks gestational age in the first season and 999 in the second season were followed at monthly intervals

  12. Gene disruption of dematin causes precipitous loss of erythrocyte membrane stability and severe hemolytic anemia.

    Science.gov (United States)

    Lu, Yunzhe; Hanada, Toshihiko; Fujiwara, Yuko; Nwankwo, Jennifer O; Wieschhaus, Adam J; Hartwig, John; Huang, Sha; Han, Jongyoon; Chishti, Athar H

    2016-07-01

    Dematin is a relatively low abundance actin binding and bundling protein associated with the spectrin-actin junctions of mature erythrocytes. Primary structure of dematin includes a loosely folded core domain and a compact headpiece domain that was originally identified in villin. Dematin's actin binding properties are regulated by phosphorylation of its headpiece domain by cyclic adenosine monophosphate-dependent protein kinase. Here, we used a novel gene disruption strategy to generate the whole body dematin gene knockout mouse model (FLKO). FLKO mice, while born at a normal Mendelian ratio, developed severe anemia and exhibited profound aberrations of erythrocyte morphology and membrane stability. Having no apparent effect on primitive erythropoiesis, FLKO mice show significant enhancement of erythroblast enucleation during definitive erythropoiesis. Using membrane protein analysis, domain mapping, electron microscopy, and dynamic deformability measurements, we investigated the mechanism of membrane instability in FLKO erythrocytes. Although many membrane and cytoskeletal proteins remained at their normal levels, the major peripheral membrane proteins spectrin, adducin, and actin were greatly reduced in FLKO erythrocytes. Our results demonstrate that dematin plays a critical role in maintaining the fundamental properties of the membrane cytoskeleton complex. PMID:27073223

  13. Severe esophageal injuries caused by accidental button battery ingestion in children

    Directory of Open Access Journals (Sweden)

    Sara Fuentes

    2014-01-01

    Full Text Available Introduction: Button batteries represent a low percentage of all foreign bodies swallowed by children and esophageal location is even less frequent. However, these cases are more likely to develop severe injuries. The aim of this essay is to report three cases treated in our institution and review previous reports. Material and Methods: Chart review and literature search. Case Reports: We treated three children between 2-7- years old with button batteries lodged at esophagus. They all presented esophageal burns (EB, which evolved in esophageal stenosis in two out of the three cases. Results: We found 29 more cases in literature and the injuries included EB, esophageal perforation (EP and tracheoesophageal fistula (TEF. Discussion: Swallowed button batteries rarely remain in esophagus, but these cases present a higher risk of tisular damage. Injuries can take place even after few hours; and therefore, endoscopy must be performed as soon as possible. Further study on button batteries′ safety and the establishment of a maximum size for them would be good preventive measures.

  14. Severe disseminated phaeohyphomycosis in an immunocompetent patient caused by Veronaea botryosa.

    Science.gov (United States)

    Bonifaz, Alexandro; Davoudi, Mehrnaz Mohammad; de Hoog, G S; Padilla-Desgarennes, Carmen; Vázquez-González, Denisse; Navarrete, Gisela; Meis, Jacques F; Badali, Hamid

    2013-06-01

    We present a severe case of disseminated phaeohyphomycosis due to Veronaea botryosa. A 32-year-old female, native from Cuautla, Morelos, Mexico, presented a chronic dermatosis which started 10 years earlier with multiple exophytic, multilobulated, soft, and pedunculated or sessile neoformations of diverse sizes from 2 to 10 cm in diameter, which became verrucose and increased in size. The patient was immunocompetent, and no hereditary or familiar precedents of importance were known. No treatment was given, and the dermatosis remained relatively stable until the patient became pregnant in 2001 and 2003. The infection then exacerbated and worsened, leading to dissemination to the extremities, trunk, and face. The initial diagnosis was chromoblastomycosis which was treated with terbinafine and itraconazole but without visible improvement. Histopathology revealed pigmented, irregular, unbranched, and septate hyphae. Veronaea botryosa was isolated (CBS 127264 = JX566723), and its identity was confirmed by sequencing the internal transcribed spacer (ITS) rDNA. Therapy with posaconazole (800 mg/day) was started showing a gradual improvement of lesions with a reduction in size and flattening of the eruptions.

  15. TFIIH subunit alterations causing xeroderma pigmentosum and trichothiodystrophy specifically disturb several steps during transcription.

    Science.gov (United States)

    Singh, Amita; Compe, Emanuel; Le May, Nicolas; Egly, Jean-Marc

    2015-02-01

    Mutations in genes encoding the ERCC3 (XPB), ERCC2 (XPD), and GTF2H5 (p8 or TTD-A) subunits of the transcription and DNA-repair factor TFIIH lead to three autosomal-recessive disorders: xeroderma pigmentosum (XP), XP associated with Cockayne syndrome (XP/CS), and trichothiodystrophy (TTD). Although these diseases were originally associated with defects in DNA repair, transcription deficiencies might be also implicated. By using retinoic acid receptor beta isoform 2 (RARB2) as a model in several cells bearing mutations in genes encoding TFIIH subunits, we observed that (1) the recruitment of the TFIIH complex was altered at the activated RARB2 promoter, (2) TFIIH participated in the recruitment of nucleotide excision repair (NER) factors during transcription in a manner different from that observed during NER, and (3) the different TFIIH variants disturbed transcription by having distinct consequences on post-translational modifications of histones, DNA-break induction, DNA demethylation, and gene-loop formation. The transition from heterochromatin to euchromatin was disrupted depending on the variant, illustrating the fact that TFIIH, by contributing to NER factor recruitment, orchestrates chromatin remodeling. The subtle transcriptional differences found between various TFIIH variants thus participate in the phenotypic variability observed among XP, XP/CS, and TTD individuals.

  16. Risk factors for neutropenia with lenalidomide plus dexamethasone therapy for multiple myeloma.

    Science.gov (United States)

    Mitani, Y; Usami, E; Kimura, M; Nakao, T; Okada, K; Matsuoka, T; Kokuryou, T; Yoshimura, T; Yamakawa, M

    2016-06-01

    Neutropenia may develop as an adverse event in patients with multiple myeloma receiving lenalidomide (LEN) plus dexamethasone (DEX) therapy. In the present study, we examined the risk factors associated with grade 3/4 neutropenia during the first cycle of LEN plus DEX therapy. We observed that hemoglobin level (≤ 8.5 g/dl) was a significant risk factor for grade 3/4 neutropenia during the first cycle of therapy (odds ratio: 19.40; 95% confidence interval: 2.68-141.00; p neutropenia in patients receiving LEN plus DEX therapy. PMID:27455556

  17. Mycoplasma ovipneumoniae - A Primary Cause of Severe Pneumonia Epizootics in the Norwegian Muskox (Ovibos moschatus) Population

    Science.gov (United States)

    Handeland, Kjell; Tengs, Torstein; Kokotovic, Branko; Vikøren, Turid; Ayling, Roger D.; Bergsjø, Bjarne; Sigurðardóttir, Ólöf G.; Bretten, Tord

    2014-01-01

    The Norwegian muskox (Ovibos moschatus) population lives on the high mountain plateau of Dovre and originates from animals introduced from Greenland. In the late summers of 2006 and 2012, severe outbreaks of pneumonia with mortality rates of 25-30% occurred. During the 2012 epidemic high quality samples from culled sick animals were obtained for microbiological and pathological examinations. High throughput sequencing (pyrosequencing) of pneumonic lung tissue revealed high concentrations of Mycoplasma ovipneumoniae in all six animals examined by this method and Pasteurella multocida subsp. multocida in four animals, whereas no virus sequences could be identified. Mycoplasma ovipneumoniae and P. multocida multocida were also isolated by culture. Using real time PCR on lung swabs, M. ovipneumoniae was detected in all of the 19 pneumonic lungs examined. Gross pathological examination revealed heavy consolidations primarily in the cranial parts of the lungs and it also identified one case of otitis media. Histologically, lung lesions were characterized as acute to subacute mixed exudative and moderately proliferative bronchoalveolar pneumonia. Immunohistochemical (IHC) examination revealed high load of M. ovipneumoniae antigens within lung lesions, with particularly intensive staining in the neutrophils. Similar IHC finding were observed in archived lung tissue blocks from animals examined during the 2006 epidemic. An M. ovipneumoniae specific ELISA was applied on bio-banked muskox sera from stray muskoxen killed in the period 2004–2013 and sick muskoxen culled, as well as sera from wild reindeer (Rangifer tarandus tarandus) on Dovre and muskoxen from Greenland. Serology and mycoplasma culturing was also carried out on sheep that had been on pasture in the muskox area during the outbreak in 2012. Our findings indicated separate introductions of M. ovipneumoniae infection in 2006 and 2012 from infected co-grazing sheep. Salt licks shared by the two species were a

  18. Identification of Mutations in TMEM5 and ISPD as a Cause of Severe Cobblestone Lissencephaly

    Science.gov (United States)

    Vuillaumier-Barrot, Sandrine; Bouchet-Séraphin, Céline; Chelbi, Malika; Devisme, Louise; Quentin, Samuel; Gazal, Steven; Laquerrière, Annie; Fallet-Bianco, Catherine; Loget, Philippe; Odent, Sylvie; Carles, Dominique; Bazin, Anne; Aziza, Jacqueline; Clemenson, Alix; Guimiot, Fabien; Bonnière, Maryse; Monnot, Sophie; Bole-Feysot, Christine; Bernard, Jean-Pierre; Loeuillet, Laurence; Gonzales, Marie; Socha, Koryna; Grandchamp, Bernard; Attié-Bitach, Tania; Encha-Razavi, Férechté; Seta, Nathalie

    2012-01-01

    Cobblestone lissencephaly is a peculiar brain malformation with characteristic radiological anomalies. It is defined as cortical dysplasia that results when neuroglial overmigration into the arachnoid space forms an extracortical layer that produces agyria and/or a “cobblestone” brain surface and ventricular enlargement. Cobblestone lissencephaly is pathognomonic of a continuum of autosomal-recessive diseases characterized by cerebral, ocular, and muscular deficits. These include Walker-Warburg syndrome, muscle-eye-brain disease, and Fukuyama muscular dystrophy. Mutations in POMT1, POMT2, POMGNT1, LARGE, FKTN, and FKRP identified these diseases as alpha-dystroglycanopathies. Our exhaustive screening of these six genes, in a cohort of 90 fetal cases, led to the identification of a mutation in only 53% of the families, suggesting that other genes might also be involved. We therefore decided to perform a genome-wide study in two multiplex families. This allowed us to identify two additional genes: TMEM5 and ISPD. Because TMEM has a glycosyltransferase domain and ISPD has an isoprenoid synthase domain characteristic of nucleotide diP-sugar transferases, these two proteins are thought to be involved in the glycosylation of dystroglycan. Further screening of 40 families with cobblestone lissencephaly identified nonsense and frameshift mutations in another four unrelated cases for each gene, increasing the mutational rate to 64% in our cohort. All these cases displayed a severe phenotype of cobblestone lissencephaly A. TMEM5 mutations were frequently associated with gonadal dysgenesis and neural tube defects, and ISPD mutations were frequently associated with brain vascular anomalies. PMID:23217329

  19. Imaging manifestations and pathological analysis of severe pneumonia caused by human infected avian influenza (H7N9

    Directory of Open Access Journals (Sweden)

    Zheng Zeng

    2015-03-01

    Conclusion: The imaging features of severe pneumonia caused by human infected avian influenza (H7N9 include obvious ground-glass opacity and pulmonary consolidation, mainly at lower lobes and dorsal of lungs, with rapid changes. The cross-analysis of imaging and pathology preliminary can elucidate the pathological mechanisms of ground-glass opacities and pulmonary consolidation of severe pneumonia. Such an intensive study is beneficial to prompt clinicians to observe and evaluate the progress of the disease. In addition, it is also in favor of managing the symptoms and reducing the mortality rate.

  20. [Consensus: Rational approach towards the patient with cancer, fever and neutropenia].

    Science.gov (United States)

    Santolaya, María Elena; Rabagliati, Ricardo; Bidart, Teresa; Payá, Ernesto; Guzmán, Ana M; Morales, Ricardo; Braun, Stephanie; Bronfman, Lucía; Ferrés, Marcela; Flores, Claudio; García, Patricia; Letelier, Luz M; Puga, Bárbara; Salgado, Carmen; Thompson, Luis; Tordecilla, Juan; Zubieta, Marcela

    2005-01-01

    The severity and duration of post chemotherapy neutropenia were recognized during the 1960s as main predisposing factors for infections in cancer patients. At the beginning of the 70's a standard management approach for all febrile neutropenia (FN) episodes was proposed, based on hospitalization and intravenous empirical broad spectrum antibiotic therapy. Widespread use of this approach resulted in a significant reduction in mortality attributable to bacterial infections. During the last 10 to 15 years, reappraisal of this standard approach has been done by several research groups who question the benefit of treating all FN patients similarly without taking in to consideration differences in severity of the FN episodes. This reappraisal has led during the 1990s to the development of the concept of high and low risk FN episodes that has been the base for the adoption of selective therapies based on the risk categorization of the individual patient. The Chilean Infectious Diseases Society called upon two government National Programs responsible for the appropriate distribution of chemotherapeutic drugs to all pediatric and adults cancer patients within the public health system, and upon the Chilean Hematology Society for the development of a Consensus on Diagnosis, Treatment and Prevention of Infections during FN Episodes in Cancer patients. The need for this Consensus is based on two main aspects: the new approaches proposed during the past year for management of these episodes, and the increasing population of cancer patients receiving improved chemotherapeutic agents that has increased there survival possibilities as well as there possibility to suffer a FN episode. The topics discussed in this document are based on an updated systematic and analytic review of the medical literature including epidemiology, laboratory diagnostics, risk categorization, treatment and prophylaxis. National data was included when available in order to provide the healthcare personnel

  1. Surgical treatment of severe chronic venous insufficiency caused by pulsatile varicose veins in a patient with tricuspid regurgitation.

    Science.gov (United States)

    Casian, D; Gutsu, E; Culiuc, V

    2009-04-01

    A case of severe chronic venous insufficiency caused by pulsatile varicose veins in a 46-year-old man with tricuspid regurgitation is presented. Active venous leg ulcer complicated with recurrent venous bleeding and inefficacy of conservative management serve as indications for surgical treatment. This case demonstrates the possibility of radical surgical correction of pathological venous reflux by means of saphenofemoral ligation, foam sclerotherapy and subfascial endoscopic perforator surgery.

  2. Late-onset dysphagia caused by severe spastic peristalsis of a free jejunal graft in a case of hypopharyngeal cancer.

    Science.gov (United States)

    Imai, Takayuki; Goto, Takahiro; Matsumoto, Ko; Kurosawa, Koreyuki; Asada, Yukinori; Saijo, Shigeru; Matsuura, Kazuto

    2016-12-01

    Free jejunal transfer is the main technique used for reconstructing a circumferential defect caused by total pharyngo-laryngo-cervical-esophagectomy in certain cancer cases. We report a rare case of severe late-onset dysphagia caused by autonomous spastic peristalsis, which led to complete obstruction of the free jejunal route. A 70-year-old man underwent treatment for hypopharyngeal cancer involving total pharyngolaryngectomy with free jejunal transfer. After uneventful peri- and postoperative recovery, he developed sudden-onset severe dysphagia 22 months later. Gastrografin fluoroscopy revealed abnormal peristalsis and contraction of the transferred jejunum, leading to complete obstruction. Nutritional treatment, application of depressants of peristalsis, and xylocaine injection into the outer space of the jejunal mucosa all failed to alleviate the dysphagia. Surgical treatment involving a longitudinal incision of the jejunal graft, and interposing a cutaneous flap, as a fixed wall, between the incised jejunal margins to prevent obstruction was performed. After further reconstructive surgery involving using a pectoralis major musculocutaneous flap and a split-thickness skin graft to close a refractory jejunum-skin fistula, the dysphagia was permanently alleviated. To our knowledge, this is the first report of severe dysphagia caused by peristalsis of a free jejunal graft. PMID:27068782

  3. Identification and Clinical Characterization of Children With Benign Ethnic Neutropenia.

    Science.gov (United States)

    Ortiz, Michael V; Meier, Emily R; Hsieh, Matthew M

    2016-04-01

    Benign ethnic neutropenia (BEN) is an asymptomatic condition reported in adults of African and Middle Eastern descent. The clinical description in children is currently lacking. In our urban outpatient pediatric hematology clinic, the median neutrophil count of children with BEN was lower than previous reports in adults at 893×10 cells/L, but increased with older age. There was an equal male to female ratio and 24% of our BEN children reported ethnicities other than African or Middle Eastern. Children with BEN had a clinical course comparable with other healthy children including otherwise normal blood counts, except for mild anemia.

  4. Unilateral posterior vertebral column resection for severe thoracolumbar kyphotic deformity caused by old compressive vertebrae fracture: a technical improvement.

    Science.gov (United States)

    Wang, Hui; Zhang, Di; Sun, Ya-Peng; Ma, Lei; Ding, Wen-Yuan; Shen, Yong; Zhang, Ying-Ze

    2015-01-01

    Severe thoracolumbar kyphotic deformity caused by old compressive vertebrae fracture remains a big challenge for spine surgeons. When symptoms related to significant deformities cannot be adequately managed conservatively, posterior vertebral column resection (PVCR) is required, but with long operating time and severe blood loss. We develop a UPVCR technique, which is done through a unilateral approach instead of a bilateral approach, vertebral body resection advancing to cross the midline in an abrasive way from an extreme oblique orientation enable the resection of most contralateral vertebral body. In the present study, the effects of UPVCR for severe thoracolumbar kyphotic deformity were investigated. We did find that satisfactory correction of sagittal deformity, functional improvement and pain relief can be achieved by UPVCR, and it has the advantage of shortening surgery time, reducing blood loss and incidence of nerve root impingement over PVCR.

  5. The use of FDG-PET/CT in patients with febrile neutropenia

    NARCIS (Netherlands)

    Vos, F.J.; Bleeker-Rovers, C.P.; Oyen, W.J.G.

    2013-01-01

    Fever is a frequent complication of neutropenia induced by the treatment of various neoplasms. This is referred to as febrile neutropenia, which is considered to be a sign of a potentially life-threatening infectious complication until proven otherwise. However, most infectious foci do not have loca

  6. Novas diretrizes na abordagem clínica da neutropenia febril e da sepse em oncologia pediátrica New guidelines for the clinical management of febrile neutropenia and sepsis in pediatric oncology patients

    Directory of Open Access Journals (Sweden)

    Ana Verena Almeida Mendes

    2007-05-01

    precoce são fundamentais para a melhora da sobrevida.OBJECTIVES: To provide a foundation for the diagnostic, prophylactic and therapeutic management of febrile neutropenia and sepsis in children with oncological diseases, with special attention to new protocols and guidelines. SOURCES: A review of the scientific literature utilizing an electronic bibliographic search on MEDLINE, Medscape, SciELO, Google, Cochrane and PubMED using the keywords febrile, neutropenic, cancer, children, sepsis, intensive, care. Articles published between 1987 and 2007 were selected, with preference given to review articles, protocols, systematic reviews, epidemiological studies, task force recommendations and phase III clinical trials. Consensus documents published by the Infectious Diseases Society of America, the Center for Diseases Control and the Infectious Diseases Working Party of the German Society of Hematology and Oncology, in addition to the recommendations of the World Federation of Pediatric Intensive and Critical Care Societies and Society of Critical Care Medicine, were also reviewed. SUMMARY OF THE FINDINGS: The use of aggressive chemotherapy regimens, bone marrow transplantation and intensive care resources have increased the survival rates of children with cancer and also their infectious morbidity, with septic complications as the principal cause of mortality. Several risk factors have been identified, such as neutropenia, oncology type, clinical signs and inflammatory response markers (polymerase chain reaction, procalcitonin and also increased resistance to antimicrobials and antifungal agents. Protocols for risk classification, diagnosis and treatment should be established at each service, taking into account the microbiological flora of each population. Pediatric intensive care has increased the short and long-term survival of these patients. CONCLUSIONS: Oncology patients are particularly vulnerable to infectious complications. Early identification and treatment are

  7. Vitamin B12 and Vitamin D Deficiencies: An Unusual Cause of Fever, Severe Hemolytic Anemia and Thrombocytopenia

    Science.gov (United States)

    Mishra, Vikas A.; Harbada, Rishit; Sharma, Akhilesh

    2015-01-01

    The array of diagnostic workup for pyrexia of unknown origin (PUO) generally revolves in searching for infections, inflammatory/autoimmune, and endocrine etiologies. A differential diagnosis of fever, hemolytic anemia, and thrombocytopenia can have etiologies varying from infections like malaria, dengue, cytomegalovirus, Ebstein barr virus, Parvovirus, infective endocarditis, to autoimmune disorder (systemic lupus erythromatosis), vasculitis, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (TTP), autoimmune hemolytic anemia/Evan's syndrome, paroxysmal nocturnal hemoglobinuri (PNH), or drugs. Nutritional deficiencies (especially vitamin B12 deficiency) as a cause of fever, hemolytic anemia, and thrombocytopenia are very rare and therefore rarely thought of. Severe vitamin B12 deficiency may cause fever and if accompanied by concurrent hyper-homocysteinemia and hypophosphatemia can sometimes lead to severe hemolysis mimicking the above-mentioned conditions. We present a case that highlights vitamin B12 and vitamin D deficiency as an easily treatable cause of PUO, hemolytic anemia, and thrombocytopenia, which should be actively looked for and treated before proceeding with more complicated and expensive investigation or starting empiric treatments. PMID:25811010

  8. Use of tunnelled catheters in haematological malignancy patients with neutropenia.

    Science.gov (United States)

    Sariosmanoglu, N; Uğurlu, B; Turgut, N H; Demirkan, F; Ozsan, H; Ergor, G; Gulay, Z; Hazan, E; Oto, O

    2008-01-01

    This prospective study analysed 83 patients (age 45 +/- 17 years) with haematological neoplasms, implanted with 93 tunnelled catheters, who were neutropenic or developed neutropenia during treatment. Catheters were implanted in the right (n = 82) or left (n = 11) jugular vein by the same surgical team using the same technique. They remained in place for 124 +/- 88 days: 29% were removed due to infection; 18% due to treatment termination and 2% due to mechanical problems. Seventeen patients died with catheters in place. At 30, 60, 90, 120 and 200 days mean catheter duration rates were 82%, 75%, 65%, 60% and 35%, respectively, and freedom from catheter removal due to infection was 92%, 88%, 80%, 77% and 67%, respectively. Patient diagnosis and history of previous catheter infection did not increase catheter infection risk, but patients undergoing stem cell transplantation had an increased infection risk. Tunnelled catheters can be used in high-risk patients with neutropenia. Systemic infections can be managed in most patients without catheter removal. PMID:18831907

  9. Biosimilars in the management of neutropenia: focus on filgrastim.

    Science.gov (United States)

    Caselli, Désirée; Cesaro, Simone; Aricò, Maurizio

    2016-01-01

    Advances in chemotherapy and surgery allows the majority of patients to survive cancer diseases. Yet, the price may be a proportion of patients dying of complications due to treatment-induced infectious complications, such as neutropenia. With the aim of decreasing morbidity and mortality related to infectious complications, recombinant human granulocyte colony-stimulating factor (G-CSF), filgrastim, and pegylated filgrastim have been used to reduce time and degree of neutropenia. A biosimilar is a copy of an approved original biologic medicine whose data protection has expired. The patent for filgrastim expired in Europe in 2006 and in the US in 2013. This review analyses the available evidence to be considered in order to design a strategy of use of G-CSF and its biosimilars. The clinical and safety outcomes of biosimilars are well within the range of historically reported data for originator filgrastim. This underscores the clinical effectiveness and safety of biosimilar filgrastim in daily clinical practice. Biosimilars can play an important role by offering the opportunity to reduce costs, thus contributing to the financial sustainability of treatment programs. PMID:26937170

  10. A combined LDL receptor/LDL receptor adaptor protein 1 mutation as the cause for severe familial hypercholesterolemia.

    Science.gov (United States)

    Soufi, Muhidien; Rust, Stephan; Walter, Michael; Schaefer, Juergen R

    2013-05-25

    Familial hypercholesterolemia (FH) results from impaired catabolism of plasma low density lipoproteins (LDL), thus leading to high cholesterol, atherosclerosis, and a high risk of premature myocardial infarction. FH is commonly caused by defects of the LDL receptor or its main ligand apoB, together mediating cellular uptake and clearance of plasma LDL. In some cases FH is inherited by mutations in the genes of PCSK9 and LDLRAP1 (ARH) in a dominant or recessive trait. The encoded proteins are required for LDL receptor stability and internalization within the LDLR pathway. To detect the underlying genetic defect in a family of Turkish descent showing unregular inheritance of severe FH, we screened the four candidate genes by denaturing gradient gel electrophoresis (DGGE) mutation analysis. We identified different combinatory mixtures of LDLR- and LDLRAP1-gene defects as the cause for severe familial hypercholesterolemia in this family. We also show for the first time that a heterozygous LDLR mutation combined with a homozygous LDLRAP1 mutation produces a more severe hypercholesterolemia phenotype in the same family than a homozygous LDLR mutation alone. PMID:23510778

  11. CHEMOTHERAPY-INDUCED NEUTROPENIA IN HIV POSITIVE PATIENTS WITH LYMPHOMA: COMPARISON OF PEGFILGRASTIM WITH DAILY FILGRASTIM ADMINISTRATION.

    Directory of Open Access Journals (Sweden)

    Luciana Teofili

    2012-10-01

    Full Text Available We retrospectively compared the incidence of neutropenia  in two groups of  HIV patients with lymphoma,  who underwent chemotherapy supported by once-per-cycle administration of pegfilgrastim or by daily subcutaneous injection of filgrastim, respectively. Our findings indicate that pegfilgrastim and filgastrim produce similar results in preventing both neutropenia and febrile neutropenia.

  12. Loss-of-function mutations in SCN4A cause severe foetal hypokinesia or 'classical' congenital myopathy

    DEFF Research Database (Denmark)

    Zaharieva, Irina T; Thor, Michael G; Oates, Emily C;

    2016-01-01

    of disorders. SCN4A encodes the α-subunit of the skeletal muscle voltage-gated sodium channel (Nav1.4). This channel is essential for the generation and propagation of the muscle action potential crucial to muscle contraction. Dominant SCN4A gain-of-function mutations are a well-established cause of myotonia......, severe muscle weakness resulted in death during the third trimester or shortly after birth. The remaining four cases had marked congenital or neonatal-onset hypotonia and weakness associated with mild-to-moderate facial and neck weakness, significant neonatal-onset respiratory and swallowing difficulties...... and childhood-onset spinal deformities. All four surviving cohort members experienced clinical improvement in the first decade of life. Muscle biopsies showed myopathic features including fibre size variability, presence of fibrofatty tissue of varying severity, without specific structural abnormalities...

  13. Comorbidities among patients with cancer who do and do not develop febrile neutropenia during the first chemotherapy cycle.

    Science.gov (United States)

    Li, Xiaoyan; Luthra, Rakesh; Morrow, Phuong K; Fisher, Maxine D; Reiner, Maureen; Barron, Richard L; Langeberg, Wendy J

    2016-10-01

    Patients receiving myelosuppressive chemotherapy with certain comorbidities are at increased risk of febrile neutropenia. A comprehensive evaluation of febrile neutropenia-related comorbidities across cancers is needed. This study compared comorbidity prevalence among patients with cancer who did and did not develop febrile neutropenia during the first chemotherapy cycle. This case-control study used administrative claims from adult patients with non-Hodgkin lymphoma or breast, lung, colorectal, ovarian, or gastric cancer who received chemotherapy between 2007 and 2012. Each patient who developed febrile neutropenia (case) was matched with up to four patients without febrile neutropenia (controls) by cancer type, metastasis, chemotherapy regimen, age group, and sex. For each comorbidity (identified in the year before chemotherapy began), the adjusted odds ratio (aOR) for febrile neutropenia by cancer type was evaluated using conditional logistic regression models adjusted for potential confounding factors. Of 31,331 eligible patients, 672 developed febrile neutropenia in the first chemotherapy cycle. A total of 3312 febrile neutropenia cases and matched controls were analyzed. Across tumor types, comorbidity prevalence was higher in patients who developed febrile neutropenia than in those without febrile neutropenia. Among patients with breast cancer, osteoarthritis was more prevalent in patients with febrile neutropenia (aOR, 1.85; 95% CI, 1.07 to 3.18). Among patients with non-Hodgkin lymphoma, renal disease was more prevalent in patients with febrile neutropenia (aOR, 2.25; 95% CI, 1.23 to 4.11). Patients who developed febrile neutropenia in the first chemotherapy cycle presented with comorbidities more often than otherwise similar patients who did not develop febrile neutropenia. These findings warrant further investigation and support the inclusion of comorbidities into febrile neutropenia risk models.

  14. Severe respiratory illness caused by a novel coronavirus, in a patient transferred to the United Kingdom from the Middle East, September 2012

    OpenAIRE

    Bermingham, Alison; Chand, M.A.; Brown, C S; Aarons, E.; Tong, C; Langrish, C; Hoschler, K; Brown, Kevin; Galiano, Monica; Myers, Richard; Pebody, R. G.; Green, H.K.; Boddington, N.L.; Gopal, Robin; Price, N

    2012-01-01

    textabstractCoronaviruses have the potential to cause severe transmissible human disease, as demonstrated by the severe acute respiratory syndrome (SARS) outbreak of 2003. We describe here the clinical and virological features of a novel coronavirus infection causing severe respiratory illness in a patient transferred to London, United Kingdom, from the Gulf region of the Middle East.

  15. Severe respiratory illness caused by a novel coronavirus, in a patient transferred to the United Kingdom from the Middle East, September 2012.

    Science.gov (United States)

    Bermingham, A; Chand, M A; Brown, C S; Aarons, E; Tong, C; Langrish, C; Hoschler, K; Brown, K; Galiano, M; Myers, R; Pebody, R G; Green, H K; Boddington, N L; Gopal, R; Price, N; Newsholme, W; Drosten, C; Fouchier, R A; Zambon, M

    2012-01-01

    Coronaviruses have the potential to cause severe transmissible human disease, as demonstrated by the severe acute respiratory syndrome (SARS) outbreak of 2003. We describe here the clinical and virological features of a novel coronavirus infection causing severe respiratory illness in a patient transferred to London, United Kingdom, from the Gulf region of the Middle East. PMID:23078800

  16. APOA5 Q97X Mutation Identified through homozygosity mapping causes severe hypertriglyceridemia in a Chilean consanguineous family

    Directory of Open Access Journals (Sweden)

    Dussaillant Catalina

    2012-11-01

    Full Text Available Abstract Background Severe hypertriglyceridemia (HTG has been linked to defects in LPL, APOC2, APOA5, LMF1 and GBIHBP1 genes. However, a number of severe HTG cases are probably caused by as yet unidentified mutations. Very high triglyceride plasma levels (>112 mmol/L at diagnosis were found in two sisters of a Chilean consanguineous family, which is strongly suggestive of a recessive highly penetrant mutation. The aim of this study was to determine the genetic locus responsible for the severe HTG in this family. Methods We carried out a genome-wide linkage study with nearly 300,000 biallelic markers (Illumina Human CytoSNP-12 panel. Using the homozygosity mapping strategy, we searched for chromosome regions with excess of homozygous genotypes in the affected cases compared to non-affected relatives. Results A large homozygous segment was found in the long arm of chromosome 11, with more than 2,500 consecutive homozygous SNP shared by the proband with her affected sister, and containing the APOA5/A4/C3/A1 cluster. Direct sequencing of the APOA5 gene revealed a known homozygous nonsense Q97X mutation (p.Gln97Ter found in both affected sisters but not in non-affected relatives nor in a sample of unrelated controls. Conclusion The Q97X mutation of the APOA5 gene in homozygous status is responsible for the severe hypertriglyceridemia in this family. We have shown that homozygosity mapping correctly pinpointed the genomic region containing the gene responsible for severe hypertriglyceridemia in this consanguineous Chilean family.

  17. The D173G mutation in ADAMTS-13 causes a severe form of congenital thrombotic thrombocytopenic purpura

    KAUST Repository

    Lancellotti, S.

    2015-08-13

    Congenital thrombotic thrombocytopenic purpura (TTP) is a rare form of thrombotic microangiopathy, inherited with autosomal recessive mode as a dysfunction or severe deficiency of ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats Nr. 13), caused by mutations in the ADAMTS-13 gene. About 100 mutations of the ADAMTS-13 gene were identified so far, although only a few characterised by in vitro expression studies. A new Asp to Gly homozygous mutation at position 173 of ADAMTS-13 sequence was identified in a family of Romanian origin, with some members affected by clinical signs of TTP. In two male sons, this mutation caused a severe (< 3 %) deficiency of ADAMTS-13 activity and antigen level, associated with periodic thrombocytopenia, haemolytic anaemia and mild mental confusion. Both parents, who are cousins, showed the same mutation in heterozygous form. Expression studies of the mutant ADAMTS-13, performed in HEK293 cells, showed a severe decrease of the enzyme’s activity and secretion, although the protease was detected inside the cells. Molecular dynamics found that in the D173G mutant the interface area between the metalloprotease domain and the disintegrin-like domain significantly decreases during the simulations, while the proline-rich 20 residues linker region (LR, 285–304) between them undergoes extensive conformational changes. Inter-domain contacts are also significantly less conserved in the mutant compared to the wild-type. Both a decrease of the inter-domain contacts along with a substantial conformational rearrangement of LR interfere with the proper maturation and folding of the mutant ADAMTS-13, thus impairing its secretion.

  18. Neonatal lupus manifests as isolated neutropenia and mildly abnormal liver functions.

    Science.gov (United States)

    Kanagasegar, Sivalingam; Cimaz, Rolando; Kurien, Biji T; Brucato, Antonio; Scofield, R Hal

    2002-01-01

    Neonatal lupus is characterized by typical clinical features and the presence of maternal autoantibodies. Mothers can have systemic lupus erythematosus (SLE) or Sjögren's syndrome, but are commonly not affected with any clinical disease. The major clinical manifestations in the infants are cardiac, dermatological and hepatic with rare instances of hemolytic anemia, thrombocytopenia or neutropenia. We describe an infant born to a mother with anti-Ro and anti-La, who had neutropenia and mildly abnormal liver functions without other major clinical features of neonatal lupus such as cardiac or dermatological manifestations. Neutropenia improved as maternal antibody was metabolized. Antibodies from both the infant and mother bound intact neutrophils, and this binding was inhibited by 60 kDa Ro. These data imply neutropenia may be an isolated manifestation of neonatal lupus. We studied the anti-Ro antibodies of 2 other mothers who gave birth to infants with complete congenital heart block and neutropenia. Their sera also bound neutrophils. Because healthy infants do not commonly undergo complete blood counts, the incidence of neutropenia among infants of anti-Ro-positive mothers may be much higher than previously recognized. Furthermore, although other factors may contribute, these data suggest that anti-60 kDa Ro is directly involved in the pathogenesis of neutropenia.

  19. Community-Associated Methicillin-Resistant Staphylococcus aureus Lacking PVL, as a Cause of Severe Invasive Infection Treated with Linezolid

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    Catarina Gouveia

    2013-01-01

    Full Text Available Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA is an emerging public health problem worldwide. Severe invasive infections have been described, mostly associated with the presence of Panton-Valentine leukocidin (PVL. In Portugal limited information exists regarding CA-MRSA infections. In this study we describe the case of a previously healthy 12-year-old female, sport athlete, who presented to the hospital with acetabulofemoral septic arthritis, myositis, fasciitis, acetabulum osteomyelitis, and pneumonia. The MRSA isolated from blood and synovial fluid was PVL negative and staphylococcal enterotoxin type P (SEP and type L (SEL positive, with a vancomycin MIC of 1.0 mg/L and resistant to clindamycin and ciprofloxacin. The patient was submitted to multiple surgical drainages and started on vancomycin, rifampicin, and gentamycin. Due to persistence of fever and no microbiological clearance, linezolid was started with improvement. This is one of the few reported cases of severe invasive infection caused by CA-MRSA in Portugal, which was successfully treated with linezolid. In spite of the severity of infection, the MRSA isolate did not produce PVL.

  20. Survival and differentiation defects contribute to neutropenia in glucose-6-phosphatase-β (G6PC3) deficiency in a model of mouse neutrophil granulocyte differentiation.

    Science.gov (United States)

    Gautam, S; Kirschnek, S; Gentle, I E; Kopiniok, C; Henneke, P; Häcker, H; Malleret, L; Belaaouaj, A; Häcker, G

    2013-08-01

    Differentiation of neutrophil granulocytes (neutrophils) occurs through several steps in the bone marrow and requires a coordinate regulation of factors determining survival and lineage-specific development. A number of genes are known whose deficiency disrupts neutrophil generation in humans and in mice. One of the proteins encoded by these genes, glucose-6-phosphatase-β (G6PC3), is involved in glucose metabolism. G6PC3 deficiency causes neutropenia in humans and in mice, linked to enhanced apoptosis and ER stress. We used a model of conditional Hoxb8 expression to test molecular and functional differentiation as well as survival defects in neutrophils from G6PC3(-/-) mice. Progenitor lines were established and differentiated into neutrophils when Hoxb8 was turned off. G6PC3(-/-) progenitor cells underwent substantial apoptosis when differentiation was started. Transgenic expression of Bcl-XL rescued survival; however, Bcl-XL-protected differentiated cells showed reduced proliferation, immaturity and functional deficiency such as altered MAP kinase signaling and reduced cytokine secretion. Impaired glucose utilization was found and was associated with ER stress and apoptosis, associated with the upregulation of Bim and Bax; downregulation of Bim protected against apoptosis during differentiation. ER-stress further caused a profound loss of expression and secretion of the main neutrophil product neutrophil elastase during differentiation. Transplantation of wild-type Hoxb8-progenitor cells into irradiated mice allowed differentiation into neutrophils in the bone marrow in vivo. Transplantation of G6PC3(-/-) cells yielded few mature neutrophils in bone marrow and peripheral blood. Transgenic Bcl-XL permitted differentiation of G6PC3(-/-) cells in vivo. However, functional deficiencies and differentiation abnormalities remained. Differentiation of macrophages from Hoxb8-dependent progenitors was only slightly disturbed. A combination of defects in differentiation

  1. The Importance of Serum Cytokine Levels in Febrile Neutropenia

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    Nuray Buyukberber

    2003-02-01

    Full Text Available The most important evaluation of the neutropenic patients is to determine the risk group. The desired approach to patients with low risks should be either not to hospitalize or to hospitalize for a short period of time which both decreases the cost and exposure to the resistant flora. The early diagnosis of sepsis in patients with high risk may be life saving. Recently, the determination of low and high-risk groups only by the clinical variables is not found to be a reliable method. The laboratory parameters supported by the clinical variables may be more practical. The determination of serum cytokines levels in febrile neutropenia may be helpful for the early risk diagnosis, new treatment approaches, and prognosis. [Archives Medical Review Journal 2003; 12(1.000: 12-19

  2. Loss-of-function mutations in SCN4A cause severe foetal hypokinesia or 'classical' congenital myopathy.

    Science.gov (United States)

    Zaharieva, Irina T; Thor, Michael G; Oates, Emily C; van Karnebeek, Clara; Hendson, Glenda; Blom, Eveline; Witting, Nanna; Rasmussen, Magnhild; Gabbett, Michael T; Ravenscroft, Gianina; Sframeli, Maria; Suetterlin, Karen; Sarkozy, Anna; D'Argenzio, Luigi; Hartley, Louise; Matthews, Emma; Pitt, Matthew; Vissing, John; Ballegaard, Martin; Krarup, Christian; Slørdahl, Andreas; Halvorsen, Hanne; Ye, Xin Cynthia; Zhang, Lin-Hua; Løkken, Nicoline; Werlauff, Ulla; Abdelsayed, Mena; Davis, Mark R; Feng, Lucy; Phadke, Rahul; Sewry, Caroline A; Morgan, Jennifer E; Laing, Nigel G; Vallance, Hilary; Ruben, Peter; Hanna, Michael G; Lewis, Suzanne; Kamsteeg, Erik-Jan; Männikkö, Roope; Muntoni, Francesco

    2016-03-01

    Congenital myopathies are a clinically and genetically heterogeneous group of muscle disorders characterized by congenital or early-onset hypotonia and muscle weakness, and specific pathological features on muscle biopsy. The phenotype ranges from foetal akinesia resulting in in utero or neonatal mortality, to milder disorders that are not life-limiting. Over the past decade, more than 20 new congenital myopathy genes have been identified. Most encode proteins involved in muscle contraction; however, mutations in ion channel-encoding genes are increasingly being recognized as a cause of this group of disorders. SCN4A encodes the α-subunit of the skeletal muscle voltage-gated sodium channel (Nav1.4). This channel is essential for the generation and propagation of the muscle action potential crucial to muscle contraction. Dominant SCN4A gain-of-function mutations are a well-established cause of myotonia and periodic paralysis. Using whole exome sequencing, we identified homozygous or compound heterozygous SCN4A mutations in a cohort of 11 individuals from six unrelated kindreds with congenital myopathy. Affected members developed in utero- or neonatal-onset muscle weakness of variable severity. In seven cases, severe muscle weakness resulted in death during the third trimester or shortly after birth. The remaining four cases had marked congenital or neonatal-onset hypotonia and weakness associated with mild-to-moderate facial and neck weakness, significant neonatal-onset respiratory and swallowing difficulties and childhood-onset spinal deformities. All four surviving cohort members experienced clinical improvement in the first decade of life. Muscle biopsies showed myopathic features including fibre size variability, presence of fibrofatty tissue of varying severity, without specific structural abnormalities. Electrophysiology suggested a myopathic process, without myotonia. In vitro functional assessment in HEK293 cells of the impact of the identified SCN4A

  3. Diarrheagenic Escherichia coli carrying supplementary virulence genes are an important cause of moderate to severe diarrhoeal disease in Mexico.

    Directory of Open Access Journals (Sweden)

    Sandra Patzi-Vargas

    2015-03-01

    Full Text Available Diarrheagenic Escherichia coli (DEC cause acute and persistent diarrhoea worldwide, but little is known about their epidemiology in Mexico. We determined the prevalence of bacterial enteropathogens in 831 children with acute diarrhoea over a four-year period in Yucatan, Mexico. Six DEC supplementary virulence genes (SVG, mainly associated with enteroaggregative E. coli (EAEC, were sought in 3100 E. coli isolates. DEC was the most common bacterial enteropathogen (28%, surpassing Salmonella (12% and Shigella (9%. Predominant DEC groups were diffusely adherent E. coli (DAEC (35%, EAEC (24%, and enteropathogenic E. coli (EPEC (19%. Among children with DEC infections, 14% had severe illness mainly caused by EPEC (26% and DAEC (18%; 30% had moderate diarrhoea mainly caused by DAEC (36%, mixed DEC infections (33% and EAEC (32%. DAEC was most prevalent during spring, while ETEC, EAEC and EPEC predominated in summer. EAEC was more frequent in children 6-24 months old than in those younger than 6 months of age (P = 0.008, OR = 4.2, 95% CI, 1.3-13.9. The presence of SVG dispersin, (aatA, dispersin-translocator (aatA, enteroaggregative heat-stable toxin 1 (astA, plasmid encoded toxin (pet, cytolethal distending toxin (cdt was higher in DEC than non-DEC strains, (36% vs 26%, P <0.0001, OR = 1.5, 95% CI, 1.3-1.8. 98% of EAEC-infected children harboured strains with SVG; 85% carried the aap-aatA gene combination, and 33% of these also carried astA. 28% of both EPEC and ETEC, and 6% of DAEC patients had strains with SVG. 54% of EPEC patients carried pet-positive strains alone or in combination with astA; only this DEC group harboured cdt-positive isolates. All ETEC patients carried astA- or astA-aap-positive strains. astA and aap were the most common SVG in DAEC (3% and 2% and non-DEC strains (21% and 13%. DEC carrying SVG are an important cause of moderate to severe bacterial diarrhoea in Mexican children.

  4. Genetic and structural analyses suggest that a novel SPG3A mutation causes severe phenotypes of hereditary spastic paraplegia

    Institute of Scientific and Technical Information of China (English)

    CHEN Suqin; ZHOU Yan; LI Xunhua; Labu; HUANG Shuang; HUANG Weijun; ZHOU Chunlong; liu; WANG Yiming

    2006-01-01

    Hereditary spastic paraplegia (HSP) is a group of neurodegenerative diseases. The genotypes and phenotypes of HSP are extremely heterogenous. SPG3A is one of the identified genes underlying HSP, and codes for a GTPase, atlastin. Mutations in SPG3A are currently believed to be associated with early onset and mild phenotypes. And most structural predictions could not detect gross changes in the mutant protein. However, in a severely affected HSP family we have identified a novel SPG3A mutation, c.1228G>A (p.G410R), in a Tibetan kindred. The mutation occurred at the highly conserved nucleotide and co-segregated with the disease, and was absent in the control subjects. Structural predictions showed that the Tibetan mutation occurred at the linking part between the guanylate-binding protein domain (GB, the ball region) and the transmembrane helices (TM, the rod region) at the start point of an α-helix, which may disrupt the helix, and cause changes in the overall structure of the transmembrane region of the molecule. Our results indicate that severe phenotypes can also arise from SPG3A mutations and the linking part of the guanylate-binding protein domain and the transmembrane helices might be crucial in determining the severity of the disease. This paper not only presents the first SPG3A mutational report from the Chinese population, but also provides potential evidence for a possible correlation between the severity of the phenotypes of HSP with the extension of the changes in the protein structures of atlastin.

  5. A brucellosis case presenting with vesicular and maculopapular rash and febrile neutropenia

    Directory of Open Access Journals (Sweden)

    Selmin Dirgen Çaylak

    2014-03-01

    Full Text Available Brucellosis is a systemic disease in which all kind of tissues and organs can be affected. Brucellosis may present with different symptoms and symptoms are non-specific. A broad spectrum of clinical manifestations can be seen, therefore diagnosis can be difficult. Cutaneous complications and febrile neutropenia have been rarely reported. Here, a rare brucellosis case was reported that he applied with fever, skin eruption and neutropenia. We emphasized that especially in endemic areas brucellosis should always be kept on mind in the differential diagnosis of patient with skin eruption and febril neutropenia.J Microbiol Infect Dis 2014;4(1: 39-41

  6. Severe congenital lipodystrophy and a progeroid appearance: Mutation in the penultimate exon of FBN1 causing a recognizable phenotype.

    Science.gov (United States)

    Takenouchi, Toshiki; Hida, Mariko; Sakamoto, Yoshiaki; Torii, Chiharu; Kosaki, Rika; Takahashi, Takao; Kosaki, Kenjiro

    2013-12-01

    Recently, three marfanoid patients with congenital lipodystrophy and a neonatal progeroid appearance were reported. Although their phenotype was distinct from that of classic Marfan syndrome, they all had a truncating mutation in the penultimate exon, i.e., exon 64, of FBN1, the causative gene for Marfan syndrome. These patients might represent a new entity, but the exact phenotypic and genotypic spectrum remains unknown. Here, we report on a girl born prematurely who exhibited severe congenital lipodystrophy and a neonatal progeroid appearance. The patient exhibited a characteristic growth pattern consisting of an accelerated growth in height with a discrepant poor weight gain. She had a characteristic facial appearance with craniosynostosis. A mutation analysis identified c.8175_8182del8bp, p.Arg2726Glufs*9 in exon 64 of the FBN1 gene. A review of similar, recently reported patients revealed that the cardinal features of these patients include (1) congenital lipodystrophy, (2) premature birth with an accelerated linear growth disproportionate to the weight gain, and (3) a progeroid appearance with distinct facial features. Lines of molecular evidence suggested that this new progeroid syndrome represents a neomorphic phenotype caused by truncated transcripts with an extremely charged protein motif that escapes from nonsense-mediated mRNA decay, altering FBN1-TGF beta signaling, rather than representing the severe end of the hypomorphic phenotype of the FBN1-TGF beta disorder spectrum. We propose that this marfanoid entity comprised of congenital lipodystrophy, a neonatal progeroid appearance, and a peculiar growth profile and caused by rare mutations in the penultimate exon of FBN1, be newly referred to as marfanoid-progeroid syndrome. PMID:24039054

  7. Severe congenital lipodystrophy and a progeroid appearance: Mutation in the penultimate exon of FBN1 causing a recognizable phenotype.

    Science.gov (United States)

    Takenouchi, Toshiki; Hida, Mariko; Sakamoto, Yoshiaki; Torii, Chiharu; Kosaki, Rika; Takahashi, Takao; Kosaki, Kenjiro

    2013-12-01

    Recently, three marfanoid patients with congenital lipodystrophy and a neonatal progeroid appearance were reported. Although their phenotype was distinct from that of classic Marfan syndrome, they all had a truncating mutation in the penultimate exon, i.e., exon 64, of FBN1, the causative gene for Marfan syndrome. These patients might represent a new entity, but the exact phenotypic and genotypic spectrum remains unknown. Here, we report on a girl born prematurely who exhibited severe congenital lipodystrophy and a neonatal progeroid appearance. The patient exhibited a characteristic growth pattern consisting of an accelerated growth in height with a discrepant poor weight gain. She had a characteristic facial appearance with craniosynostosis. A mutation analysis identified c.8175_8182del8bp, p.Arg2726Glufs*9 in exon 64 of the FBN1 gene. A review of similar, recently reported patients revealed that the cardinal features of these patients include (1) congenital lipodystrophy, (2) premature birth with an accelerated linear growth disproportionate to the weight gain, and (3) a progeroid appearance with distinct facial features. Lines of molecular evidence suggested that this new progeroid syndrome represents a neomorphic phenotype caused by truncated transcripts with an extremely charged protein motif that escapes from nonsense-mediated mRNA decay, altering FBN1-TGF beta signaling, rather than representing the severe end of the hypomorphic phenotype of the FBN1-TGF beta disorder spectrum. We propose that this marfanoid entity comprised of congenital lipodystrophy, a neonatal progeroid appearance, and a peculiar growth profile and caused by rare mutations in the penultimate exon of FBN1, be newly referred to as marfanoid-progeroid syndrome.

  8. Patterns of neutropenia and risk factors for febrile neutropenia of diffuse large B-cell lymphoma patients treated with rituximab-CHOP.

    Science.gov (United States)

    Choi, Yong Won; Jeong, Seong Hyun; Ahn, Mi Sun; Lee, Hyun Woo; Kang, Seok Yun; Choi, Jin-Hyuk; Jin, U Ram; Park, Joon Seong

    2014-11-01

    Febrile neutropenia (FN) is the major toxicity of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) regimen in the treatment of diffuse large B-cell lymphoma (DLBCL). The prediction of neutropenia and FN is mandatory to continue the planned R-CHOP therapy resulting in successful anti-cancer treatment. The clinical features and patterns of neutropenia and FN from 181 DLBCL patients treated with R-CHOP were analyzed retrospectively. Sixty percent (60.2%) of patients experienced at least one episode of grade 4 neutropenia. Among them, 42.2% of episodes progressed to FN. Forty-eight percent (48.8%) of patients with FN was experienced their first FN during the first cycle of R-CHOP. All those patients never experienced FN again during the rest cycles of R-CHOP. Female, higher stage, international prognostic index (IPI), age ≥65 yr, comorbidities, bone marrow involvement, and baseline serum albumin ≤3.5 mg/dL were significant risk factors for FN by univariate analysis. Among these variables, comorbidities (P=0.009), bone marrow involvement (P=0.006), and female gender (P=0.024) were independent risk factors for FN based on multivariate analysis. On observing the patterns of neutropenia and FN, primary prophylaxis of granulocyte colony-stimulating factor (G-CSF) and antibiotics should be considered particularly in female patients, patients with comorbidities, or when there is bone marrow involvement of disease.

  9. Clericuzio-type Poikiloderma with Neutropenia Syndrome in a Turkish Family: a Three Report of Siblings with Mutation in the C16orf57 gene.

    Science.gov (United States)

    Patiroglu, Turkan; Akar, H Haluk

    2015-06-01

    Clericuzio-type poikiloderma with neutropenia (PN) is characterized by poikiloderma, non-cyclic neutropenia, recurrent sinopulmonary infections, pachyonychia, and palmo-plantar hyperkeratosis. Mutations in the C16orf57 gene, which is located on chromosome 16q13, have been identified as the cause of PN. PN was first described by Clericuzio in Navajo Indians. Herein, we reported the clinical presentations and laboratory investigations of PN in three siblings from Turkey. The older siblings presented with typical cutaneous poikiloderma, plantar keratoderma, pachyonychia of toenails, and recurrent upper respiratory infections. As the most affected patient, in addition to classic manifestations, the youngest sibling had recurrent pneumonia, hepatosplenomegaly, dental caries, failure to thrive, and hand malformation. Genetic study revealed a homozygous mutation (c.531delA) in the C16orf57 gene in siblings. With the presented study, we aimed to draw attention to PN which can be a predisposing factor to malignancies. PMID:26546903

  10. Promotive effect of recombinant human granulocyte colony-stimulating factor (rhG-CSF) on recovery from neutropenia induced by fractionated irradiation in mice

    International Nuclear Information System (INIS)

    The effect of recombinant human granulocyte colony-stimulating factor (rhG-CSF) on the recovery from neutropenia induced by fractionated whole-body irradiation was investigated in mice. Male 7-week old C3H/HeN mice received a total of ten exposures of 0.25 Gy/day from day 1 to 5 and from day 8 to 12. Peripheral neutropenia with a nadir on day 17 was caused by the fractionated irradiation. Daily subcutaneous injections of rhG-CSF at 0.25 and 2.5 μg/body/day from day from day 1 to 21 promoted the recovery of neutrophils in a dose-dependent manner. The kinetics of morphologically identifiable bone marrow cells were studied to clarify the mechanism behind the promotive effect of this factor. A slight decrease in mitotic immature granulocytes, such as myeloblasts, promyelocytes and myelocytes on day 5, and a drastic decrease in metamyelocytes and marrow neutrophils on days 5, 9, and 17 were seen in the femur of irradiated mice. Treatment using rhG-CSF caused an increase in immature granulocytes of all differential stages in the femur. Microscopic findings of the femurs and spleens also reveals an increase in immature granulocytes in these organs in mice injected with rhG-CSF. These results indicate that rhG-CSF accelerates granulopoiesis in the femur and spleen, thereby promoting recovery from neutropenia induced by fractionated irradiation. (author)

  11. Duplication of the MECP2 region is a frequent cause of severe mental retardation and progressive neurological symptoms in males.

    Science.gov (United States)

    Van Esch, Hilde; Bauters, Marijke; Ignatius, Jaakko; Jansen, Mieke; Raynaud, Martine; Hollanders, Karen; Lugtenberg, Dorien; Bienvenu, Thierry; Jensen, Lars Riff; Gecz, Jozef; Moraine, Claude; Marynen, Peter; Fryns, Jean-Pierre; Froyen, Guy

    2005-09-01

    Loss-of-function mutations of the MECP2 gene at Xq28 are associated with Rett syndrome in females and with syndromic and nonsyndromic forms of mental retardation (MR) in males. By array comparative genomic hybridization (array-CGH), we identified a small duplication at Xq28 in a large family with a severe form of MR associated with progressive spasticity. Screening by real-time quantitation of 17 additional patients with MR who have similar phenotypes revealed three more duplications. The duplications in the four patients vary in size from 0.4 to 0.8 Mb and harbor several genes, which, for each duplication, include the MR-related L1CAM and MECP2 genes. The proximal breakpoints are located within a 250-kb region centromeric of L1CAM, whereas the distal breakpoints are located in a 300-kb interval telomeric of MECP2. The precise size and location of each duplication is different in the four patients. The duplications segregate with the disease in the families, and asymptomatic carrier females show complete skewing of X inactivation. Comparison of the clinical features in these patients and in a previously reported patient enables refinement of the genotype-phenotype correlation and strongly suggests that increased dosage of MECP2 results in the MR phenotype. Our findings demonstrate that, in humans, not only impaired or abolished gene function but also increased MeCP2 dosage causes a distinct phenotype. Moreover, duplication of the MECP2 region occurs frequently in male patients with a severe form of MR, which justifies quantitative screening of MECP2 in this group of patients.

  12. Erectile dysfunction severity as a risk marker for cardiovascular disease hospitalisation and all-cause mortality: a prospective cohort study.

    Directory of Open Access Journals (Sweden)

    Emily Banks

    Full Text Available BACKGROUND: Erectile dysfunction is an emerging risk marker for future cardiovascular disease (CVD events; however, evidence on dose response and specific CVD outcomes is limited. This study investigates the relationship between severity of erectile dysfunction and specific CVD outcomes. METHODS AND FINDINGS: We conducted a prospective population-based Australian study (the 45 and Up Study linking questionnaire data from 2006-2009 with hospitalisation and death data to 30 June and 31 Dec 2010 respectively for 95,038 men aged ≥45 y. Cox proportional hazards models were used to examine the relationship of reported severity of erectile dysfunction to all-cause mortality and first CVD-related hospitalisation since baseline in men with and without previous CVD, adjusting for age, smoking, alcohol consumption, marital status, income, education, physical activity, body mass index, diabetes, and hypertension and/or hypercholesterolaemia treatment. There were 7,855 incident admissions for CVD and 2,304 deaths during follow-up (mean time from recruitment, 2.2 y for CVD admission and 2.8 y for mortality. Risks of CVD and death increased steadily with severity of erectile dysfunction. Among men without previous CVD, those with severe versus no erectile dysfunction had significantly increased risks of ischaemic heart disease (adjusted relative risk [RR] = 1.60, 95% CI 1.31-1.95, heart failure (8.00, 2.64-24.2, peripheral vascular disease (1.92, 1.12-3.29, "other" CVD (1.26, 1.05-1.51, all CVD combined (1.35, 1.19-1.53, and all-cause mortality (1.93, 1.52-2.44. For men with previous CVD, corresponding RRs (95% CI were 1.70 (1.46-1.98, 4.40 (2.64-7.33, 2.46 (1.63-3.70, 1.40 (1.21-1.63, 1.64 (1.48-1.81, and 2.37 (1.87-3.01, respectively. Among men without previous CVD, RRs of more specific CVDs increased significantly with severe versus no erectile dysfunction, including acute myocardial infarction (1.66, 1.22-2.26, atrioventricular and left bundle branch

  13. Moxifloxacin Compared With Ciprofloxacin/Amoxicillin in Treating Fever and Neutropenia in Patients With Cancer

    Science.gov (United States)

    2012-09-20

    Chronic Myeloproliferative Disorders; Fever, Sweats, and Hot Flashes; Infection; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neutropenia; Precancerous Condition; Unspecified Adult Solid Tumor, Protocol Specific

  14. Sunitinib-associated hypertension and neutropenia as efficacy biomarkers in metastatic renal cell carcinoma patients

    DEFF Research Database (Denmark)

    Donskov, Frede; Michaelson, M Dror; Puzanov, Igor;

    2015-01-01

    BACKGROUND: Metastatic renal cell carcinoma (mRCC) prognostic models may be improved by incorporating treatment-induced toxicities. METHODS: In sunitinib-treated mRCC patients (N=770), baseline prognostic factors and treatment-induced toxicities (hypertension (systolic blood pressure ⩾140 mm Hg......), neutropenia (grade ⩾2), thrombocytopenia (grade ⩾2), hand-foot syndrome (grade >0), and asthenia/fatigue (grade >0)) were analysed in multivariate analyses of progression-free survival (PFS) and overall survival (OS) end points. RESULTS: On-treatment neutropenia and hypertension were associated with longer....... Considering hypertension and neutropenia (developing both vs neither) changed IMDC-predicted median OS in each IMDC risk group (favourable: 45.3 vs 19.5 months; intermediate: 32.5 vs 8.0 months; poor: 21.1 vs 4.8 months). CONCLUSIONS: On-treatment neutropenia and hypertension are independent biomarkers...

  15. Cost Minimization Analysis of the Use of Meropenem and Ceftazidime in Febrile Neutropenia Therapy

    Directory of Open Access Journals (Sweden)

    Rizky Abdulah

    2016-06-01

    Full Text Available Use of antibiotics is required in febrile neutropenia therapy. The variety choice on the use of antibiotics has increased the role of pharmacoeconomics study to determine the most effective and efficient antibiotic in a specific area. The purpose of this study was to investigate the lowest cost antibiotic between meropenem and ceftazidime that were used as one of febrile neutropenia treatments at one of referral hospitals in West Java province during 2011–2013. This study was a retrospective, observational and analytical study that was performed on February 2014 by collecting medical record data related to febrile neutropenia inpatient who received meropenem or ceftazidime therapy. The result showed that although it was not statistically significant, the total cost for ceftazidime therapy was IDR7,082,523, which was lower than meropenem therapy (IDR11,094,147. Hopefully, this result can assist the health professionals in the management of febrile neutropenia therapy.

  16. Loss-of-function mutations in SCN4A cause severe foetal hypokinesia or ‘classical’ congenital myopathy

    Science.gov (United States)

    Zaharieva, Irina T.; Thor, Michael G.; Oates, Emily C.; van Karnebeek, Clara; Hendson, Glenda; Blom, Eveline; Witting, Nanna; Rasmussen, Magnhild; Gabbett, Michael T.; Ravenscroft, Gianina; Sframeli, Maria; Suetterlin, Karen; Sarkozy, Anna; D’Argenzio, Luigi; Hartley, Louise; Matthews, Emma; Pitt, Matthew; Vissing, John; Ballegaard, Martin; Krarup, Christian; Slørdahl, Andreas; Halvorsen, Hanne; Ye, Xin Cynthia; Zhang, Lin-Hua; Løkken, Nicoline; Werlauff, Ulla; Abdelsayed, Mena; Davis, Mark R.; Feng, Lucy; Phadke, Rahul; Sewry, Caroline A.; Morgan, Jennifer E.; Laing, Nigel G.; Vallance, Hilary; Ruben, Peter; Hanna, Michael G.; Lewis, Suzanne; Kamsteeg, Erik-Jan; Männikkö, Roope

    2016-01-01

    See Cannon (doi:10.1093/brain/awv400) for a scientific commentary on this article. Congenital myopathies are a clinically and genetically heterogeneous group of muscle disorders characterized by congenital or early-onset hypotonia and muscle weakness, and specific pathological features on muscle biopsy. The phenotype ranges from foetal akinesia resulting in in utero or neonatal mortality, to milder disorders that are not life-limiting. Over the past decade, more than 20 new congenital myopathy genes have been identified. Most encode proteins involved in muscle contraction; however, mutations in ion channel-encoding genes are increasingly being recognized as a cause of this group of disorders. SCN4A encodes the α-subunit of the skeletal muscle voltage-gated sodium channel (Nav1.4). This channel is essential for the generation and propagation of the muscle action potential crucial to muscle contraction. Dominant SCN4A gain-of-function mutations are a well-established cause of myotonia and periodic paralysis. Using whole exome sequencing, we identified homozygous or compound heterozygous SCN4A mutations in a cohort of 11 individuals from six unrelated kindreds with congenital myopathy. Affected members developed in utero- or neonatal-onset muscle weakness of variable severity. In seven cases, severe muscle weakness resulted in death during the third trimester or shortly after birth. The remaining four cases had marked congenital or neonatal-onset hypotonia and weakness associated with mild-to-moderate facial and neck weakness, significant neonatal-onset respiratory and swallowing difficulties and childhood-onset spinal deformities. All four surviving cohort members experienced clinical improvement in the first decade of life. Muscle biopsies showed myopathic features including fibre size variability, presence of fibrofatty tissue of varying severity, without specific structural abnormalities. Electrophysiology suggested a myopathic process, without myotonia. In vitro

  17. Recessive Inactivating Mutations in TBCK, Encoding a Rab GTPase-Activating Protein, Cause Severe Infantile Syndromic Encephalopathy.

    Science.gov (United States)

    Chong, Jessica X; Caputo, Viviana; Phelps, Ian G; Stella, Lorenzo; Worgan, Lisa; Dempsey, Jennifer C; Nguyen, Alina; Leuzzi, Vincenzo; Webster, Richard; Pizzuti, Antonio; Marvin, Colby T; Ishak, Gisele E; Ardern-Holmes, Simone; Richmond, Zara; Bamshad, Michael J; Ortiz-Gonzalez, Xilma R; Tartaglia, Marco; Chopra, Maya; Doherty, Dan

    2016-04-01

    Infantile encephalopathies are a group of clinically and biologically heterogeneous disorders for which the genetic basis remains largely unknown. Here, we report a syndromic neonatal encephalopathy characterized by profound developmental disability, severe hypotonia, seizures, diminished respiratory drive requiring mechanical ventilation, brain atrophy, dysgenesis of the corpus callosum, cerebellar vermis hypoplasia, and facial dysmorphism. Biallelic inactivating mutations in TBCK (TBC1-domain-containing kinase) were independently identified by whole-exome sequencing as the cause of this condition in four unrelated families. Matching these families was facilitated by the sharing of phenotypic profiles and WES data in a recently released web-based tool (Geno2MP) that links phenotypic information to rare variants in families with Mendelian traits. TBCK is a putative GTPase-activating protein (GAP) for small GTPases of the Rab family and has been shown to control cell growth and proliferation, actin-cytoskeleton dynamics, and mTOR signaling. Two of the three mutations (c.376C>T [p.Arg126(∗)] and c.1363A>T [p.Lys455(∗)]) are predicted to truncate the protein, and loss of the major TBCK isoform was confirmed in primary fibroblasts from one affected individual. The third mutation, c.1532G>A (p.Arg511His), alters a conserved residue within the TBC1 domain. Structural analysis implicated Arg511 as a required residue for Rab-GAP function, and in silico homology modeling predicted impaired GAP function in the corresponding mutant. These results suggest that loss of Rab-GAP activity is the underlying mechanism of disease. In contrast to other disorders caused by dysregulated mTOR signaling associated with focal or global brain overgrowth, impaired TBCK function results in progressive loss of brain volume. PMID:27040692

  18. Recessive Inactivating Mutations in TBCK, Encoding a Rab GTPase-Activating Protein, Cause Severe Infantile Syndromic Encephalopathy

    Science.gov (United States)

    Chong, Jessica X.; Caputo, Viviana; Phelps, Ian G.; Stella, Lorenzo; Worgan, Lisa; Dempsey, Jennifer C.; Nguyen, Alina; Leuzzi, Vincenzo; Webster, Richard; Pizzuti, Antonio; Marvin, Colby T.; Ishak, Gisele E.; Ardern-Holmes, Simone; Richmond, Zara; Bamshad, Michael J.; Ortiz-Gonzalez, Xilma R.; Tartaglia, Marco; Chopra, Maya; Doherty, Dan

    2016-01-01

    Infantile encephalopathies are a group of clinically and biologically heterogeneous disorders for which the genetic basis remains largely unknown. Here, we report a syndromic neonatal encephalopathy characterized by profound developmental disability, severe hypotonia, seizures, diminished respiratory drive requiring mechanical ventilation, brain atrophy, dysgenesis of the corpus callosum, cerebellar vermis hypoplasia, and facial dysmorphism. Biallelic inactivating mutations in TBCK (TBC1-domain-containing kinase) were independently identified by whole-exome sequencing as the cause of this condition in four unrelated families. Matching these families was facilitated by the sharing of phenotypic profiles and WES data in a recently released web-based tool (Geno2MP) that links phenotypic information to rare variants in families with Mendelian traits. TBCK is a putative GTPase-activating protein (GAP) for small GTPases of the Rab family and has been shown to control cell growth and proliferation, actin-cytoskeleton dynamics, and mTOR signaling. Two of the three mutations (c.376C>T [p.Arg126∗] and c.1363A>T [p.Lys455∗]) are predicted to truncate the protein, and loss of the major TBCK isoform was confirmed in primary fibroblasts from one affected individual. The third mutation, c.1532G>A (p.Arg511His), alters a conserved residue within the TBC1 domain. Structural analysis implicated Arg511 as a required residue for Rab-GAP function, and in silico homology modeling predicted impaired GAP function in the corresponding mutant. These results suggest that loss of Rab-GAP activity is the underlying mechanism of disease. In contrast to other disorders caused by dysregulated mTOR signaling associated with focal or global brain overgrowth, impaired TBCK function results in progressive loss of brain volume. PMID:27040692

  19. Cost Minimization Analysis of the Use of Meropenem and Ceftazidime in Febrile Neutropenia Therapy

    OpenAIRE

    Rizky Abdulah; Raine D. Kumamba; Rano K. Sinuraya; Cherry Rahayu; Melisa I. Barliana

    2016-01-01

    Use of antibiotics is required in febrile neutropenia therapy. The variety choice on the use of antibiotics has increased the role of pharmacoeconomics study to determine the most effective and efficient antibiotic in a specific area. The purpose of this study was to investigate the lowest cost antibiotic between meropenem and ceftazidime that were used as one of febrile neutropenia treatments at one of referral hospitals in West Java province during 2011–2013. This study was a retrospective,...

  20. Prospective cohort study of febrile neutropenia in breast cancer patients with neoadjuvant and adjuvant chemotherapy: CSPOR-BC FN study.

    Science.gov (United States)

    Ishikawa, Takashi; Sakamaki, Kentaro; Narui, Kazutaka; Kaise, Hiroshi; Tsugawa, Koichiro; Ichikawa, Yasushi; Mukai, Hirofumi

    2016-07-01

    With the increasing use of adjuvant chemotherapy for treating early breast cancer, febrile neutropenia management has become crucial. Guidelines for febrile neutropenia management are mostly based on a Caucasian population survey although ethnic differences are reported in terms of adverse events. We survey the current status of febrile neutropenia and risk factors in Japanese female breast cancer patients receiving neoadjuvant and adjuvant chemotherapy regimens potential for febrile neutropenia. Subsequently, we plan to conduct a multicenter prospective cohort study involving 1000 patients with operable breast cancer. With the current state of oral antibiotics being routinely prescribed without hematology tests, we survey febrile neutropenia based on two different definitions, namely, true febrile neutropenia: ≥37.5°C and Grade 4 neutropenia, and surrogate febrile neutropenia: ≥37.5°C and oral antibiotic and antipyretic intake. The comparison of true febrile neutropenia and surrogate febrile neutropenia incidences is anticipated to provide information on the safety and feasibility of chemotherapy management without performing blood tests. PMID:27162322

  1. Prospective cohort study of febrile neutropenia in breast cancer patients with neoadjuvant and adjuvant chemotherapy: CSPOR-BC FN study.

    Science.gov (United States)

    Ishikawa, Takashi; Sakamaki, Kentaro; Narui, Kazutaka; Kaise, Hiroshi; Tsugawa, Koichiro; Ichikawa, Yasushi; Mukai, Hirofumi

    2016-07-01

    With the increasing use of adjuvant chemotherapy for treating early breast cancer, febrile neutropenia management has become crucial. Guidelines for febrile neutropenia management are mostly based on a Caucasian population survey although ethnic differences are reported in terms of adverse events. We survey the current status of febrile neutropenia and risk factors in Japanese female breast cancer patients receiving neoadjuvant and adjuvant chemotherapy regimens potential for febrile neutropenia. Subsequently, we plan to conduct a multicenter prospective cohort study involving 1000 patients with operable breast cancer. With the current state of oral antibiotics being routinely prescribed without hematology tests, we survey febrile neutropenia based on two different definitions, namely, true febrile neutropenia: ≥37.5°C and Grade 4 neutropenia, and surrogate febrile neutropenia: ≥37.5°C and oral antibiotic and antipyretic intake. The comparison of true febrile neutropenia and surrogate febrile neutropenia incidences is anticipated to provide information on the safety and feasibility of chemotherapy management without performing blood tests.

  2. Association of oesophageal radiation dose volume metrics, neutropenia and acute radiation oesophagitis in patients receiving chemoradiotherapy for non-small cell lung cancer

    International Nuclear Information System (INIS)

    The relationship between oesophageal radiation dose volume metrics and dysphagia in patients having chemoradiation (CRT) for non-small cell lung cancer (NSCLC) is well established. There is also some evidence that neutropenia is a factor contributing to the severity of oesophagitis. We retrospectively analysed acute radiation oesophagitis (ARO) rates and severity in patients with NSCLC who received concurrent chemotherapy and high dose radiation therapy (CRT). We investigated if there was an association between grade of ARO, neutropenia and radiation dose volume metrics. Patients with NSCLC having concurrent CRT who had RT dose and toxicity data available were eligible. Exclusion criteria included previous thoracic RT, treatment interruptions and non-standard dose regimens. RT dosimetrics included maximum and mean oesophageal dose, oesophagus dose volume and length data. Fifty four patients were eligible for analysis. 42 (78 %) patients received 60 Gy. Forty four (81 %) patients received carboplatin based chemotherapy. Forty eight (89 %) patients experienced ARO ≥ grade 1 (95 % CI: 78 % to 95 %). ARO grade was associated with mean dose (rs = 0.27, p = 0.049), V20 (rs = 0.31, p = 0.024) and whole oesophageal circumference receiving 20 Gy (rs = 0.32 p = 0.019). In patients who received these doses, V20 (n = 51, rs = 0.36, p = 0.011), V35 (n = 43, rs = 0.34, p = 0.027) and V60 (n = 25, rs = 0.59, P = 0.002) were associated with RO grade. Eleven of 25 (44 %) patients with ARO ≥ grade 2 also had ≥ grade 2 acute neutropenia compared with 5 of 29 (17 %) patients with RO grade 0 or 1 (p = 0.035). In addition to oesophageal dose-volume metrics, neutropenia may also be a risk factor for higher grades of ARO

  3. A recessive homozygous p.Asp92Gly SDHD mutation causes prenatal cardiomyopathy and a severe mitochondrial complex II deficiency.

    Science.gov (United States)

    Alston, Charlotte L; Ceccatelli Berti, Camilla; Blakely, Emma L; Oláhová, Monika; He, Langping; McMahon, Colin J; Olpin, Simon E; Hargreaves, Iain P; Nolli, Cecilia; McFarland, Robert; Goffrini, Paola; O'Sullivan, Maureen J; Taylor, Robert W

    2015-08-01

    Succinate dehydrogenase (SDH) is a crucial metabolic enzyme complex that is involved in ATP production, playing roles in both the tricarboxylic cycle and the mitochondrial respiratory chain (complex II). Isolated complex II deficiency is one of the rarest oxidative phosphorylation disorders with mutations described in three structural subunits and one of the assembly factors; just one case is attributed to recessively inherited SDHD mutations. We report the pathological, biochemical, histochemical and molecular genetic investigations of a male neonate who had left ventricular hypertrophy detected on antenatal scan and died on day one of life. Subsequent postmortem examination confirmed hypertrophic cardiomyopathy with left ventricular non-compaction. Biochemical analysis of his skeletal muscle biopsy revealed evidence of a severe isolated complex II deficiency and candidate gene sequencing revealed a novel homozygous c.275A>G, p.(Asp92Gly) SDHD mutation which was shown to be recessively inherited through segregation studies. The affected amino acid has been reported as a Dutch founder mutation p.(Asp92Tyr) in families with hereditary head and neck paraganglioma. By introducing both mutations into Saccharomyces cerevisiae, we were able to confirm that the p.(Asp92Gly) mutation causes a more severe oxidative growth phenotype than the p.(Asp92Tyr) mutant, and provides functional evidence to support the pathogenicity of the patient's SDHD mutation. This is only the second case of mitochondrial complex II deficiency due to inherited SDHD mutations and highlights the importance of sequencing all SDH genes in patients with biochemical and histochemical evidence of isolated mitochondrial complex II deficiency. PMID:26008905

  4. Evaluation of ior ® Leukocim efficacy on patients with Neutropenia.

    Directory of Open Access Journals (Sweden)

    Ana María Ramos Cedeño

    2007-12-01

    Full Text Available Bakground: Neutropenia and infections are the most restrictive side effects in chemotherapy application. The granulocytic colonies stimulating factor activates the neutrophils, shortens the neutropenic period and can be effective against the potential risk of infection. There is a need of studies for its commercialization to endorse their effectiveness and security. Objective: To evaluate the impact of the G-CSF in primary and secondary prevention and neutropenia episodes, in onco-haematological patients in Cienfuegos. Method: 95 neutropenic episodes were studied, picked up in clinical histories and data collection notebooks belonging to 47 patients treated in the University Hospital ¨Dr. Gustavo Aldereguía Lima¨ in Cienfuegos and happened during one year (2005-2006. It was analyzed: demographic data, absolute neutrophils count values, number of administered doses, treatment regime and possible treatment interruptions Results: 50, 5% received treatment in an ambulatory way. There was treatment interruption in 9 episodes, 9,5% and the mean dose number administered to obtain the recovery of neutrophils absolute count was 6,69. Conclusions: The product was effective, sine the decrease of neutropenic episodes could be verified, as the elevation of neutrophil values in approximately one week, what allowed a better pursuit of chemotherapy to patients of the studied series.AbstractTo demonstrate the effectiveness of Leukocim an open, phase IV clinical trial was designed, 39 patients from Cienfuegos were evaluated with 73 neutropenic episodes in primary or secondary prophylaxis or treatment, the demographic parameters behaved with an age 49.86 year old average, the predominant sex was the feminine 64.38%, 73.97% belonged to the white race, when the effectiveness was analyzed we obtained that 46.5% received the treatment in an ambulatory way, whereas 52.1% was hospitalized; one patient abandoned before beginning the treatment, the 12,3% of patients

  5. Successful use of N-acetylcysteine to treat severe hepatic injury caused by a dietary fitness supplement.

    Science.gov (United States)

    El Rahi, Cynthia; Thompson-Moore, Nathaniel; Mejia, Patricia; De Hoyos, Patricio

    2015-06-01

    In the absence of adequate premarketing efficacy and safety evaluations, adverse events from over-the-counter supplements are emerging as a public health concern. Specifically, bodybuilding products are being identified as a frequent cause of drug-induced liver injury. We present a case of a 20-year-old Hispanic male who presented with acute nausea and vomiting accompanied by severe right upper quadrant abdominal pain, shivering, and shortness of breath. Laboratory data pointed to mixed cholestatic and hepatocellular damage, and after exclusion of known alternate etiologies, the patient was diagnosed with acute drug-induced liver injury secondary to the use of "Friction," a bodybuilding supplement. Treatment with N-acetylcysteine (NAC) 20% oral solution was initiated empirically at a dose of 4000 mg [DOSAGE ERROR CORRECTED] (70 mg/kg) every 4 hours and was continued once the diagnosis was made. Within 48 hours of admission to our hospital, the patient began to show clinical resolution of right abdominal pain and tolerance to oral diet associated with a significant decline toward normal in his liver function tests and coagulopathy. The WHO-UMC causality assessment system suggested a "certain causality" between exposure to the supplement and the acute liver injury. In the event of suspected drug-induced liver injury, treatment with NAC should be considered given its favorable risk-benefit profile.

  6. Successful use of N-acetylcysteine to treat severe hepatic injury caused by a dietary fitness supplement.

    Science.gov (United States)

    El Rahi, Cynthia; Thompson-Moore, Nathaniel; Mejia, Patricia; De Hoyos, Patricio

    2015-06-01

    In the absence of adequate premarketing efficacy and safety evaluations, adverse events from over-the-counter supplements are emerging as a public health concern. Specifically, bodybuilding products are being identified as a frequent cause of drug-induced liver injury. We present a case of a 20-year-old Hispanic male who presented with acute nausea and vomiting accompanied by severe right upper quadrant abdominal pain, shivering, and shortness of breath. Laboratory data pointed to mixed cholestatic and hepatocellular damage, and after exclusion of known alternate etiologies, the patient was diagnosed with acute drug-induced liver injury secondary to the use of "Friction," a bodybuilding supplement. Treatment with N-acetylcysteine (NAC) 20% oral solution was initiated empirically at a dose of 4000 mg [DOSAGE ERROR CORRECTED] (70 mg/kg) every 4 hours and was continued once the diagnosis was made. Within 48 hours of admission to our hospital, the patient began to show clinical resolution of right abdominal pain and tolerance to oral diet associated with a significant decline toward normal in his liver function tests and coagulopathy. The WHO-UMC causality assessment system suggested a "certain causality" between exposure to the supplement and the acute liver injury. In the event of suspected drug-induced liver injury, treatment with NAC should be considered given its favorable risk-benefit profile. PMID:25823877

  7. Cryptosporidiosis causing severe persistent diarrhea in a patient with multiple myeloma: A Case report and brief review of literature.

    Science.gov (United States)

    Tandon, Nidhi; Gupta, Sudeep

    2014-01-01

    Cryptosporidiosis is a protozoal infection that leads to self-limited diarrheal disease in immunocompetent individuals and a more severe illness in immunocompromised patients especially those infected with the human immunodeficiency virus. Although patients with hematolymphoid malignancies can develop this infection, it is an uncommon cause of diarrhea in these patients. The patient was a 64-year-old woman, a known case of multiple myeloma for 17 years, who had been treated with multiple lines of chemotherapy earlier. She was being treated with lenalidomide plus dexamethasone for active myeloma at the time of this episode. She presented with profuse watery diarrhea of 15 days duration that was proven to be due to Cryptosporidium parvum on stool examination. The diarrheal illness resolved after treatment with nitazoxanide. Although uncommon, cryptosporidial infection should be suspected in patients with hematological malignancies who have persistent diarrhea. Stool examination with the modified acid-fast Kenyoun stain establishes the diagnosis in the majority of cases. Antiparasitic treatment is effective in controlling the infection. PMID:25006294

  8. Cryptosporidiosis causing severe persistent diarrhea in a patient with multiple myeloma: A Case report and brief review of literature

    Directory of Open Access Journals (Sweden)

    Nidhi Tandon

    2014-01-01

    Full Text Available Cryptosporidiosis is a protozoal infection that leads to self-limited diarrheal disease in immunocompetent individuals and a more severe illness in immunocompromised patients especially those infected with the human immunodeficiency virus. Although patients with hematolymphoid malignancies can develop this infection, it is an uncommon cause of diarrhea in these patients. The patient was a 64-year-old woman, a known case of multiple myeloma for 17 years, who had been treated with multiple lines of chemotherapy earlier. She was being treated with lenalidomide plus dexamethasone for active myeloma at the time of this episode. She presented with profuse watery diarrhea of 15 days duration that was proven to be due to Cryptosporidium parvum on stool examination. The diarrheal illness resolved after treatment with nitazoxanide. Although uncommon, cryptosporidial infection should be suspected in patients with hematological malignancies who have persistent diarrhea. Stool examination with the modified acid-fast Kenyoun stain establishes the diagnosis in the majority of cases. Antiparasitic treatment is effective in controlling the infection.

  9. Key Molecular Mechanisms of Chaiqinchengqi Decoction in Alleviating the Pulmonary Albumin Leakage Caused by Endotoxemia in Severe Acute Pancreatitis Rats

    Science.gov (United States)

    Wu, Wei; Luo, Ruijie; Lin, Ziqi; Xia, Qing

    2016-01-01

    To reveal the key molecular mechanisms of Chaiqinchengqi decoction (CQCQD) in alleviating the pulmonary albumin leakage caused by endotoxemia in severe acute pancreatitis (SAP) rats. Rats models of SAP endotoxemia-induced acute lung injury were established, the studies in vivo provided the important evidences that the therapy of CQCQD significantly ameliorated the increases in plasma levels of lipopolysaccharide (LPS), sCd14, and Lbp, the elevation of serum amylase level, the enhancements of systemic and pulmonary albumin leakage, and the depravation of airways indicators, thus improving respiratory dysfunction and also pancreatic and pulmonary histopathological changes. According to the analyses of rats pulmonary tissue microarray and protein-protein interaction network, c-Fos, c-Src, and p85α were predicted as the target proteins for CQCQD in alleviating pulmonary albumin leakage. To confirm these predictions, human umbilical vein endothelial cells were employed in in vitro studies, which provide the evidences that (1) LPS-induced paracellular leakage and proinflammatory cytokines release were suppressed by pretreatment with inhibitors of c-Src (PP1) or PI3K (LY294002) or by transfection with siRNAs of c-Fos; (2) fortunately, CQCQD imitated the actions of these selective inhibitions agents to inhibit LPS-induced high expressions of p-Src, p-p85α, and c-Fos, therefore attenuating paracellular leakage and proinflammatory cytokines release.

  10. [Efficacy of Levofloxacin Hydrate in Febrile Neutropenia for Outpatient Chemotherapy].

    Science.gov (United States)

    Inagaki, Manato; Sato, Junya; Nihei, Satoru; Kashiwaba, Masahiro; Kudo, Kenzo

    2016-05-01

    Management of febrile neutropenia (FN) is important for the safety of patients undergoing outpatient chemotherapy. Oral antimicrobials are usually prescribed as the initial treatment for FN, and outpatients are instructed to begin medication prior to chemotherapy. However, the effectiveness and safety of the use of these oral antibiotics have not yet been established. In this study, we investigated the effectiveness and safety of levofloxacin hydrate (LVFX) for breast cancer patients with FN, and the factors associated with the onset of FN in 134 breast cancer patients who underwent chemotherapy including the anticancer drug anthracycline (total, 513 courses), in an outpatient chemotherapy department. The effectiveness and safety of LVFX were defined respectively as defervescence within 5 days, and the appearance of side effects such as diarrhea and rashes. Fever was observed in 89 (66%) of the 134 patients, and during 164 (32%) of 513 courses. Defervescence was observed with the LVFX medication in 149 (93%) of 160 courses. The primary side effect was the development of rashes, and only 2 (1%) of the 160 courses were discontinued. Onset of stomatitis during chemotherapy was observed as a factor of FN (odds ratio: 1.36, p<0.05). Our results suggest that the use of LVFX according to the patients' discretion might be an effective and safe option for the management of FN during outpatient chemotherapy.

  11. 先天性中性粒细胞减少症的研究进展%Progress of congenital neutropenia

    Institute of Scientific and Technical Information of China (English)

    王娟娟

    2010-01-01

    先天性中性粒细胞减少症是一种少见的原发性免疫缺陷病,属于吞噬细胞数目先天性缺陷.主要的临床症状为婴幼儿期严重的中性粒细胞减少伴感染,骨髓分化受累和向白血病转化的风险.有散发、常染色体显性遗传、常染色体隐性遗传和X-连锁四种遗传方式.近年来多种基因缺陷的发现加深了对该病的认识.治疗上主要是粒细胞集落刺激因子和造血干细胞移植.%Congenital neutropenia is a rare primary immunodeficiency,which is classified into congenital defects of phagocyte number. It is characterized by significantly reduced number of circulating neutrophiles,often associated with early-onset severe infections, a block in bone marrow myeloid differentiation at the promyelocyte stage and high risk for development of leukemia. Congenital neutropenia occurs with sporadic, autosomal dominant,autosomal recessive and Xlinked inheritance. Recently, the numerous genes mutated in congenital neutropenia were found. Definitive cmre is provided by granulocyte colony-stimulating factor treatment and hematopoietic stem cell transplantation.

  12. Neutropenia y fiebre en el paciente con cáncer Neutropenia and fever in the patient with cancer

    Directory of Open Access Journals (Sweden)

    A. Manterola

    2004-01-01

    Full Text Available La infección en el huésped inmunocomprometido supone una situación clínica de gravedad por su alta morbi-mortalidad y es una de las complicaciones más frecuentes del paciente con cáncer. En los pacientes tratados con quimioterapia, el riesgo de infección depende fundamentalmente de la duración e intensidad de la neutropenia. Es fundamental evaluar cuál es el patógeno involucrado con mayor probabilidad para iniciar el tratamiento, a priori, más adecuado, así como la situación clínica general del paciente, que nos obligará a realizar un tratamiento más o menos agresivo desde el inicio, teniendo en cuenta que es posible el manejo domiciliario en aquel grupo de pacientes considerado de "bajo riesgo" de complicaciones. Estas cuestiones las podremos conocer evaluando los antecedentes y la historia clínica del paciente, la exploración física y los datos de exploraciones de laboratorio y radiológicas. El inicio precoz de la antibioterapia de amplio espectro es crucial, y revisaremos en este capítulo, las recomendaciones terapéuticas más recientes.Infection in the immunocompromised host is a serious clinical situation due to its high morbi-mortality and is one of the most frequent complications in the patient with cancer. In patients treated with chemotherapy, the risk of infection basically depends on the duration and intensity of the neutropenia. It is essential to evaluate, the most probable pathogen involved to initiate, a priori, the most suitable treatment, and also to evaluate the general clinical situation of the patient, because from the very beginning the treatment is quite aggressive. Outpatient care is possible for patients at "low risk" of complications. By evaluating the antecedents and clinical history of the patient, through physical exploration and from the data of laboratory and radiological explorations these points can be acknowledged. The early start of broad spectrum antibiotherapy is crucial, and in this

  13. CHEMOTHERAPY-INDUCED NEUTROPENIA IN HIV POSITIVE PATIENTS WITH LYMPHOMA: COMPARISON OF PEGFILGRASTIM WITH DAILY FILGRASTIM ADMINISTRATION.

    Directory of Open Access Journals (Sweden)

    Luciana Teofili

    2012-01-01

    Full Text Available

    We retrospectively compared the incidence of neutropenia  in two groups of  HIV patients with lymphoma,  who underwent chemotherapy supported by once-per-cycle administration of pegfilgrastim or by daily subcutaneous injection of filgrastim, respectively. Our findings indicate that pegfilgrastim and filgastrim produce similar results in preventing both neutropenia and febrile neutropenia.

  14. Searching of Main Cause Leading to Severe Influenza A Virus Mutations and Consequently to Influenza Pandemics/Epidemics

    Directory of Open Access Journals (Sweden)

    Guang Wu

    2005-01-01

    Full Text Available The unpredictable mutations in the proteins from influenza A virus lead to the great difficulty in prevention of possible outbreak of bird flu and pandemic/epidemic of influenza. This unpredictability is due to the fact that we know little about the causes that lead to the mutations. In three of our recent studies on the hemagglutinins from influenza A virus, we unintentionally noticed the periodicity of mutations in hemagglutinins similar to the periodicity of sunspot. We calculated the amino-acid pair predictability and amino-acid distribution rank, which are developed by us over last several years and can numerically present the evolution of proteins in question, of 1217 full-length hemagglutinins from influenza A viruses. We then used the fast Fourier transform to determine the periodicity of mutations in the hemagglutinins. We compare the periodicities of mutations in influenza A virus hemagglutinins with those of solar and galactic cosmic rays and find a main periodicity of the mutations identical to that of sunspot and neutron rate (11 years/circle. Then we plot the sunspot number with respect to the historical pandemics/epidemics/non-pandemic new strains over last three centuries and compare the recorded sunspots with the historical pandemics before 1700. Both show a good agreement between sunspot activity and influenza related events. As the histories of Sun and galaxy are incomparably much longer than the history of influenza virus, the only logical deduction is that the hemagglutinin periodicities, which are identical to the periodicities of solar and galactic cosmic rays, are attribute to the solar and galactic activity. As the hemagglutinin is a sample of influenza A virus, we can logically deduce the role of migratory wild birds on the outbreak of bird flu and influenza, that is, cosmic rays are heading towards the polar regions, where more mutations occur in influenza A virus either within the wild birds or in their living

  15. Appearance of febrile neutropenia episodes after cytostatic therapy on oncology patients

    International Nuclear Information System (INIS)

    Treatment of oncology patient using cytotoxic drugs has the neutropenia and its infectious complications as the commonest dose-limiting toxicity. Its appearance provokes dose delays and reduction during post-chemotherapy cycles, as well as the quality of life deterioration of patients. Oncology Medicine Group including the Pharmacy Service carried out a study to analyze the appearance of febrile neutropenia after cytotoxic therapy administration, and the presence of other factors that may to increase the risk to these reactions. A total of 42 patients were studied admitted with febrile neutropenia after above therapy from February to August, 2007. Biomedical variables from included patient group were achieved and the previously applied cytostatic therapy. The prevalent age-group was those patients aged over 50 and predominance of male sex and advanced stages with associated affections. The more frequent tumor locations were in breast, lung, and non-Hodgkin lymphoma. The cytostatic agent more used in cases of febrile neutropenia was Adriamycin (71.4 %) followed by Cyclophosphamide (52.4 %). The factors more associated with febrile neutropenia appearance were: Anthracycline chemotherapy, age over 50, advanced stages, and presence of associated diseases

  16. 547 An Earlier, More Severe Presentation of G6pc3 Deficiency in a Male Infant From Mexico

    OpenAIRE

    Cruz, Alonso

    2012-01-01

    Background Severe congenital neutropenia is a bone marrow failure syndrome characterized by severe neutropenia present from birth. We present a case of G6PC3 deficiency presenting at an earlier age, with a more severe clinical picture than previously reported. Case report A 3-month-old boy, born to nonconsanguineous parents was delivered by C-section at 35 weeks gestation. He was admitted to neonatal intensive care unit for prematurity and poor respiratory effort requiring mechanical ventilat...

  17. Higher Total Serum Cholesterol Levels Are Associated With Less Severe Strokes and Lower All-Cause Mortality

    DEFF Research Database (Denmark)

    Olsen, Tom Skyhøj; Christensen, Rune Haubo Bojesen; Kammersgaard, Lars;

    2007-01-01

    Background and Purpose - Evidence of a causal relation between serum cholesterol and stroke is inconsistent. We investigated the relation between total serum cholesterol and both stroke severity and poststroke mortality to test the hypothesis that hyperch....

  18. Changes in severe thunderstorm environment frequency during the 21st century caused by anthropogenically enhanced global radiative forcing

    OpenAIRE

    Trapp, Robert J.; Diffenbaugh, Noah S.; Brooks, Harold E.; Baldwin, Michael E.; Robinson, Eric D.; Pal, Jeremy S.

    2007-01-01

    Severe thunderstorms comprise an extreme class of deep convective clouds and produce high-impact weather such as destructive surface winds, hail, and tornadoes. This study addresses the question of how severe thunderstorm frequency in the United States might change because of enhanced global radiative forcing associated with elevated greenhouse gas concentrations. We use global climate models and a high-resolution regional climate model to examine the larger-scale (or “environmental”) meteoro...

  19. Stochastic hypothesis of transition from inborn neutropenia to AML: Interactions of cell population dynamics and population genetics

    Directory of Open Access Journals (Sweden)

    Marek eKimmel

    2013-04-01

    Full Text Available We present a stochastic model of driver mutations in the transition from severe congenital neutropenia to myelodysplastic syndrome to acute myeloid leukemia (AML. The model has the form of a multitype branching process. We derive equations for the distributions of the times to consecutive driver mutations and set up simulations involving a range of hypotheses regarding acceleration of the mutation rates in successive mutant clones. Our model reproduces the clinical distribution of times at diagnosis of secondary AML. Surprisingly, within the framework of our assumptions, stochasticity of the mutation process is incapable of explaining the spread of times at diagnosis of AML in this case; it is necessary to additionally assume a wide spread of proliferative parameters among disease cases. This finding is unexpected but generally consistent with the wide heterogeneity of characteristics of human cancers.

  20. A Novel Mutation in the EDAR Gene Causes Severe Autosomal Recessive Hypohidrotic Ectodermal Dysplasia

    DEFF Research Database (Denmark)

    Henningsen, Emil; Svendsen, Mathias Tiedemann; Lildballe, D. L.;

    2014-01-01

    nasal discharge. The girl was the second born child of first-cousin immigrants from Northern Iraq. A novel homozygous mutation (c.84delC) in the EDAR gene was identified. This mutation most likely causes a frameshift in the protein product (p.S29fs*74). This results in abolition of all ectodysplasin...

  1. Airway Management in a Patient with Severe Ankylosing Spondylitis Causing Bamboo Spine: Use of Aintree Intubation Catheter.

    Science.gov (United States)

    Ul Haq, Muhammad Irfan; Shamim, Faisal; Lal, Shankar; Shafiq, Faraz

    2015-12-01

    Management of a case of ankylosing spondylitis can be very challenging as the airway and the central neuraxial blockade are extremely difficult to handle. Fiberoptic intubation may lead to predictable success in the face of difficult airway. We are presenting a new technique of fiberoptic intubation in a young patient, suffering from severe ankylosing spondylitis, came for total hip replacement surgery. There was anticipated difficult airway due to severe limitation in neck movement and it was successfully managed by using Aintree Intubation Catheter (AIC) with intubating fiberoptic bronchoscope. PMID:26691367

  2. Anesthetic Management of a Patient with Sustained Severe Metabolic Alkalosis and Electrolyte Abnormalities Caused by Ingestion of Baking Soda

    OpenAIRE

    Jose Soliz; Jeffrey Lim; Gang Zheng

    2014-01-01

    The use of alternative medicine is prevalent worldwide. However, its effect on intraoperative anesthetic care is underreported. We report the anesthetic management of a patient who underwent an extensive head and neck cancer surgery and presented with a severe intraoperative metabolic alkalosis from the long term ingestion of baking soda and other herbal remedies.

  3. Anesthetic Management of a Patient with Sustained Severe Metabolic Alkalosis and Electrolyte Abnormalities Caused by Ingestion of Baking Soda

    Directory of Open Access Journals (Sweden)

    Jose Soliz

    2014-01-01

    Full Text Available The use of alternative medicine is prevalent worldwide. However, its effect on intraoperative anesthetic care is underreported. We report the anesthetic management of a patient who underwent an extensive head and neck cancer surgery and presented with a severe intraoperative metabolic alkalosis from the long term ingestion of baking soda and other herbal remedies.

  4. Dihydrofolate Reductase Deficiency Due to a Homozygous DHFR Mutation Causes Megaloblastic Anemia and Cerebral Folate Deficiency Leading to Severe Neurologic Disease

    OpenAIRE

    Cario, Holger; Smith, Desirée E. C.; Blom, Henk; Blau, Nenad; Bode, Harald; Holzmann, Karlheinz; Pannicke, Ulrich; Hopfner, Karl-Peter; Rump, Eva-Maria; Ayric, Zuleya; Kohne, Elisabeth; Debatin, Klaus-Michael; Smulders, Yvo; Schwarz, Klaus

    2011-01-01

    The importance of intracellular folate metabolism is illustrated by the severity of symptoms and complications caused by inborn disorders of folate metabolism or by folate deficiency. We examined three children of healthy, distantly related parents presenting with megaloblastic anemia and cerebral folate deficiency causing neurologic disease with atypical childhood absence epilepsy. Genome-wide homozygosity mapping revealed a candidate region on chromosome 5 including the dihydrofolate reduct...

  5. A 3 Mb deletion in 14q12 causes severe mental retardation, mild facial dysmorphisms and Rett-like features.

    Science.gov (United States)

    Papa, Filomena Tiziana; Mencarelli, Maria Antonietta; Caselli, Rossella; Katzaki, Eleni; Sampieri, Katia; Meloni, Ilaria; Ariani, Francesca; Longo, Ilaria; Maggio, Angela; Balestri, Paolo; Grosso, Salvatore; Farnetani, Maria Angela; Berardi, Rosario; Mari, Francesca; Renieri, Alessandra

    2008-08-01

    The present report describes a 7-year-old girl with a de novo 3 Mb interstitial deletion of chromosome 14q12, identified by oligo array-CGH. The region is gene poor and contains only five genes two of them, FOXG1B and PRKD1 being deleted also in a previously reported case with a very similar phenotype. Both patients present prominent metopic suture, epicanthic folds, bulbous nasal tip, tented upper lip, everted lower lip and large ears and a clinical course like Rett syndrome, including normal perinatal period, postnatal microcephaly, seizures, and severe mental retardation. FOXG1B (forkhead box G1B) is a very intriguing candidate gene since it is known to promote neuronal progenitor proliferation and to suppress premature neurogenesis and its disruption is reported in a patient with postnatal microcephaly, corpus callosum agenesis, seizures, and severe mental retardation.

  6. Novel α-spectrin mutation in trans with α-spectrin causing severe neonatal jaundice from hereditary spherocytosis.

    Science.gov (United States)

    Nussenzveig, Roberto H; Christensen, Robert D; Prchal, Josef T; Yaish, Hassan M; Agarwal, Archana M

    2014-01-01

    We evaluated a neonate with severe jaundice but a negative family history. Spherocytes were present and suspected hereditary spherocytosis was confirmed by osmotic fragility and eosin-5-maleimide erythrocyte staining. We found he was a compound heterozygote for two pathogenic mutations in the gene encoding α-spectrin: a previously reported α(LEPRA) inherited from his asymptomatic mother, and a novel α-spectrin mutation in intron 45 +1 disrupting the consensus splice site, from his asymptomatic father.

  7. Severe Hypertriglyceridemia Causing Acute Pancreatitis in a Child with New Onset Type I Diabetes Mellitus Presenting in Ketoacidosis

    OpenAIRE

    Wolfgram, Peter M.; MacDonald, Michael J.

    2013-01-01

    A 10 year old girl presented with severe diabetic ketoacidosis (DKA) and a hemoglobin A1C of 17.9%. On hospital day 2 after acidosis had improved it worsened and she developed excruciating abdominal pain. Her serum triglycerides and lipase levels were found to be extremely high and ultrasound analysis of the pancreas was consistent with acute pancreatitis. She was diagnosed with acute pancreatitis secondary to hypertriglyceridemia. The pancreatitis resolved completely and two months later her...

  8. Primary blast causes mild, moderate, severe and lethal TBI with increasing blast overpressures: Experimental rat injury model

    Science.gov (United States)

    Mishra, Vikas; Skotak, Maciej; Schuetz, Heather; Heller, Abi; Haorah, James; Chandra, Namas

    2016-06-01

    Injury severity in blast induced Traumatic Brain Injury (bTBI) increases with blast overpressure (BOP) and impulse in dose-dependent manner. Pure primary blast waves were simulated in compressed gas shock-tubes in discrete increments. Present work demonstrates 24 hour survival of rats in 0–450 kPa (0–800 Pa•s impulse) range at 10 discrete levels (60, 100, 130, 160, 190, 230, 250, 290, 350 and 420 kPa) and determines the mortality rate as a non-linear function of BOP. Using logistic regression model, predicted mortality rate (PMR) function was calculated, and used to establish TBI severities. We determined a BOP of 145 kPa as upper mild TBI threshold (5% PMR). Also we determined 146–220 kPa and 221–290 kPa levels as moderate and severe TBI based on 35%, and 70% PMR, respectively, while BOP above 290 kPa is lethal. Since there are no standards for animal bTBI injury severity, these thresholds need further refinements using histopathology, immunohistochemistry and behavior. Further, we specifically investigated mild TBI range (0–145 kPa) using physiological (heart rate), pathological (lung injury), immuno-histochemical (oxidative/nitrosative and blood-brain barrier markers) as well as blood borne biomarkers. With these additional data, we conclude that mild bTBI occurs in rats when the BOP is in the range of 85–145 kPa.

  9. Procalcitonin-guided protocol is not useful to manage antibiotic therapy in febrile neutropenia: a randomized controlled trial.

    Science.gov (United States)

    Lima, Stella Sala Soares; Nobre, Vandack; de Castro Romanelli, Roberta Maia; Clemente, Wanessa Trindade; da Silva Bittencourt, Henrique Neves; Melo, Ana Catarina Mourão; Salomão, Luciana Caetano Botelho; Serufo, José Carlos

    2016-06-01

    Febrile neutropenia (FN) requires immediate use of antibiotics (ATB), and procalcitonin (PCT) is proven to be useful in guiding antibiotic therapy in different settings. This study investigated the use of PCT as a guide for the duration of ATB in FN. A randomized controlled trial was carried out from January-December 2010. A total of 62 hematological adult patients with FN were randomized, in 1:1 ratio, into two groups: (1) PCT group: length of ATB guided by institutional protocol plus PCT dynamics, and (2) control group: duration of ATB in accordance with institutional protocol. There was no difference between groups regarding the use of ATB for the first episode of fever (HR 1.14, 95 % CI 0.66-1.95, p = 0.641), with equivalent median duration of ATB therapy (PCT group 9.0 days and control group 8.0 days, p = 0.67), and median number of days without ATB (0 days, IQR 0-2 days for both groups, p = 0.96). We observed no difference in clinical cure rate (p = 0.68), infection relapse (p = 1.0), superinfection (p = 0.85), length of hospitalization (p = 0.64), and mortality at 28 days (p = 0.39) and at 90 days (p = 0.72). Considering the cut-off of 0.5 ng/ml, PCT was correlated with bacteremia (sensitivity of 51.9 % and specificity of 76.5 %). In this randomized controlled trial, adding a PCT-guided protocol to the standard recommendations did not reduce the use of antibiotics in febrile neutropenia, although no apparent harm was caused. PCT proved to be a marker of bacteremia in this setting. PMID:27118539

  10. Coronavirus MHV-A59 infects the lung and causes severe pneumonia in C57BL/6 mice

    Institute of Scientific and Technical Information of China (English)

    Zhangsheng; Yang; Jun; Du; Gang; Chen; Jie; Zhao; Xuanming; Yang; Lishan; Su; Genhong; Cheng; Hong; Tang

    2014-01-01

    It remains challenging to develop animal models of lung infection and severe pneumonia by severe acute respiratory syndrome coronavirus(SARS-CoV) and Middle East respiratory syndrome cornavirus(MERS-Co V) without high level of containment. This inevitably hinders understanding of virushost interaction and development of appropriate countermeasures. Here we report that intranasal inoculation of sublethal doses of murine coronavirus mouse hepatitis virus A-59(MHV-A59), a hepatic and neuronal tropic coronavirus, can induce acute pneumonia and severe lung injuries in C57BL/6 mice. Inflammatory leukocyte infiltrations, hemorrhages and fibrosis of alveolar walls can be observed 2-11 days after MHV-A59 infection. This pathological manifestation is associated with dramatical elevation of tissue IP-10 and IFN-γ and moderate increase of TNF-α and IL-1β, but inability of anti-viral type I interferon response. These results suggest that intranasal infection of MHV-A59 would serve as a surrogate mouse model of acute respiratory distress syndrome by SARS-CoV and MERS-CoV infections.

  11. Correlation between urinary incontinence and localization of brain lesion and severity of neurological lesion caused by a stroke

    Directory of Open Access Journals (Sweden)

    Vetra A.

    2012-10-01

    Full Text Available Urinary incontinence is one of the medical problems, which may develop as a result of a stroke, and, according to several authors, its occurrence frequency may reach up to 80%. Clinical symptoms of urinary incontinence are considered to be one of the symptoms, which, along with the severity of the stroke and the level of functional limitations, allows to develop reliable predictions and to make targeted use of resources. Aim of the study, materials and methods. The aim of the study is to explore whether post-stroke urinary incontinence correlates with localization of lesion and clinical symptoms. The study includes 180 patients after a stroke, who have received treatment at Riga Eastern Clinic University Hospital Stroke Unit. All had a comprehensive clinical and functional assessment and cerebral computed tomography (CT. The examination took place at the hospital a few days after the stroke had occurred. Results. There are 180 participants to the study. Symptoms of urinary incontinence were reported for 70.6% of study participants. In 64% of cases new incontinence problems had developed, while in 6.5% of cases the previous symptoms of urinary incontinence (prior to the stroke had worsened. The most common types of urinary incontinence in the acute period were urge and mixed incontinence (functional and urge incontinence. Patients with low Barthel Index showed higher frequency of urinary incontinence. A comparison of patient groups with and without symptoms of urinary incontinence showed that patients older than 75 years showed more symptoms of urinary incontinence (p = 0.013, and the same can be said about patients with low indicators of Barthel (p = 0.001 and patients with cognitive disorders (p = 0.001. Severity of paresis, aphasia and sensory disorders show a reliable correlation with the type of urinary incontinence. Conclusion. Symptoms of post-stroke urinary incontinence are linked both to a person's age, and functional and cognitive

  12. Impact of multiplex PCR on antimicrobial treatment in febrile neutropenia: a randomized controlled study.

    Science.gov (United States)

    Idelevich, Evgeny A; Silling, Gerda; Niederbracht, Yvonne; Penner, Hanna; Sauerland, Maria Cristina; Tafelski, Sascha; Nachtigall, Irit; Berdel, Wolfgang E; Peters, Georg; Becker, Karsten

    2015-10-01

    Multiplex PCR (mPCR) directly from blood has been suggested as a promising method for rapid identification of pathogens causing sepsis. This study aimed to investigate whether mPCR has any impact on antimicrobial treatment. Hematological patients with febrile neutropenia were randomized into two groups. In the study group, mPCR was performed as an addition to standard diagnostics, and PCR finding was immediately communicated to the clinicians, thus being available for decision making. In the control group, clinicians were not aware of PCR result. PCR samples were collected simultaneously with clinically indicated blood culture specimens from peripheral vein and/or central venous catheter at fever onset and once again if fever persisted up to 72 h. Overall, 74 patients of the study group and 76 patients of the control group were enrolled and 253 samples collected. Therapy was changed to targeted antimicrobial therapy (AMT) in 12 patients (16.2%) in the study group and in 12 patients (15.8%) in the control group. For patients with changes, the median time to change to the targeted AMT was 21.4 h in the study group and 47.5 h in the control group (p = 0.018). In the study group, 57.1% (8/14) of changes to targeted AMT was due to PCR finding. PCR led to AMT change in 9.5% (7/74) of study group patients, i.e., in 33.3% (7/21) of patients who had positive PCR finding. There were no significant differences in patient outcomes (secondary endpoints). In conclusion, PCR method accelerates change to the targeted AMT in febrile neutropenic patients.

  13. Assessing patients' risk of febrile neutropenia: is there a correlation between physician-assessed risk and model-predicted risk?

    Science.gov (United States)

    Lyman, Gary H; Dale, David C; Legg, Jason C; Abella, Esteban; Morrow, Phuong Khanh; Whittaker, Sadie; Crawford, Jeffrey

    2015-08-01

    This study evaluated the correlation between the risk of febrile neutropenia (FN) estimated by physicians and the risk of severe neutropenia or FN predicted by a validated multivariate model in patients with nonmyeloid malignancies receiving chemotherapy. Before patient enrollment, physician and site characteristics were recorded, and physicians self-reported the FN risk at which they would typically consider granulocyte colony-stimulating factor (G-CSF) primary prophylaxis (FN risk intervention threshold). For each patient, physicians electronically recorded their estimated FN risk, orders for G-CSF primary prophylaxis (yes/no), and patient characteristics for model predictions. Correlations between physician-assessed FN risk and model-predicted risk (primary endpoints) and between physician-assessed FN risk and G-CSF orders were calculated. Overall, 124 community-based oncologists registered; 944 patients initiating chemotherapy with intermediate FN risk enrolled. Median physician-assessed FN risk over all chemotherapy cycles was 20.0%, and median model-predicted risk was 17.9%; the correlation was 0.249 (95% CI, 0.179-0.316). The correlation between physician-assessed FN risk and subsequent orders for G-CSF primary prophylaxis (n = 634) was 0.313 (95% CI, 0.135-0.472). Among patients with a physician-assessed FN risk ≥ 20%, 14% did not receive G-CSF orders. G-CSF was not ordered for 16% of patients at or above their physician's self-reported FN risk intervention threshold (median, 20.0%) and was ordered for 21% below the threshold. Physician-assessed FN risk and model-predicted risk correlated weakly; however, there was moderate correlation between physician-assessed FN risk and orders for G-CSF primary prophylaxis. Further research and education on FN risk factors and appropriate G-CSF use are needed.

  14. Severe coagulation factor VII deficiency caused by a novel homozygous mutation (p. Trp284Gly) in loop 140s.

    Science.gov (United States)

    Hao, Xiuping; Cheng, XiaoLi; Ye, Jiajia; Wang, Yingyu; Yang, LiHong; Wang, Mingshan; Jin, Yanhui

    2016-06-01

    Congenital coagulation factor VII (FVII) deficiency is a rare disorder caused by mutation in F7 gene. Herein, we reported a patient who had unexplained hematuria and vertigo with consanguineous parents. He has been diagnosed as having FVII deficiency based on the results of reduced FVII activity (2.0%) and antigen (12.8%). The thrombin generation tests verified that the proband has obstacles in producing thrombin. Direct sequencing analysis revealed a novel homozygous missense mutation p.Trp284Gly. Also noteworthy is the fact that the mutational residue belongs to structurally conserved loop 140s, which majorly undergo rearrangement after FVII activation. Model analysis indicated that the substitution disrupts these native hydrophobic interactions, which are of great importance to the conformation in the activation domain of FVIIa. PMID:26761581

  15. Implementing hospital-based surveillance for severe acute respiratory infections caused by influenza and other respiratory pathogens in New Zealand

    Directory of Open Access Journals (Sweden)

    Q Sue Huang

    2014-05-01

    Full Text Available Background: Recent experience with pandemic influenza A(H1N1pdm09 highlighted the importance of global surveillance for severe respiratory disease to support pandemic preparedness and seasonal influenza control. Improved surveillance in the southern hemisphere is needed to provide critical data on influenza epidemiology, disease burden, circulating strains and effectiveness of influenza prevention and control measures. Hospital-based surveillance for severe acute respiratory infection (SARI cases was established in New Zealand on 30 April 2012. The aims were to measure incidence, prevalence, risk factors, clinical spectrum and outcomes for SARI and associated influenza and other respiratory pathogen cases as well as to understand influenza contribution to patients not meeting SARI case definition. Methods/Design: All inpatients with suspected respiratory infections who were admitted overnight to the study hospitals were screened daily. If a patient met the World Health Organization’s SARI case definition, a respiratory specimen was tested for influenza and other respiratory pathogens. A case report form captured demographics, history of presenting illness, co-morbidities, disease course and outcome and risk factors. These data were supplemented from electronic clinical records and other linked data sources. Discussion: Hospital-based SARI surveillance has been implemented and is fully functioning in New Zealand. Active, prospective, continuous, hospital-based SARI surveillance is useful in supporting pandemic preparedness for emerging influenza A(H7N9 virus infections and seasonal influenza prevention and control.

  16. A novel biallelic splice site mutation of TECTA causes moderate to severe hearing impairment in an Algerian family.

    Science.gov (United States)

    Behlouli, Asma; Bonnet, Crystel; Abdi, Samia; Hasbellaoui, Mokhtar; Boudjenah, Farid; Hardelin, Jean-Pierre; Louha, Malek; Makrelouf, Mohamed; Ammar-Khodja, Fatima; Zenati, Akila; Petit, Christine

    2016-08-01

    Congenital deafness is certainly one of the most common monogenic diseases in humans, but it is also one of the most genetically heterogeneous, which makes molecular diagnosis challenging in most cases. Whole-exome sequencing in two out of three Algerian siblings affected by recessively-inherited, moderate to severe sensorineural deafness allowed us to identify a novel splice donor site mutation (c.5272+1G > A) in the gene encoding α-tectorin, a major component of the cochlear tectorial membrane. The mutation was present at the homozygous state in the three affected siblings, and at the heterozygous state in their unaffected, consanguineous parents. To our knowledge, this is the first reported TECTA mutation leading to the DFNB21 form of hearing impairment among Maghrebian individuals suffering from congenital hearing impairment, which further illustrates the diversity of the genes involved in congenital deafness in the Maghreb.

  17. Increasing severity of damage caused by floods in the Spanish Mediterranean coast (1960-2014), climate change or vulnerability?

    Science.gov (United States)

    Perez, Alfredo; Gil, Salvador; Lopez, Francisco; Barriendos, Mariano

    2016-04-01

    In recent decades, there has been an increase in physical and economic losses (WMO, CRED and UCL, 2014) that raises serious concerns in society. Climate change projections may explain the rise in flood losses; however, these shouldn't be considered yet (Bouwer, 2011). According to IPCC (2014), there is low confidence in anthropogenic climate change affecting the frequency and magnitude of fluvial floods on a global scale. In other words, this increase in flood events is not completely related to the higher frequency of heavy rainfall. To illustrate the aforementioned, a spatial example can be seen in the study area. In the Spanish Mediterranean coast, we see an increase in economic losses within the last 50 years due to flood events (Gil et al., 2014). It seems that the socio-economic growth and the rise of housing construction (Gaja, 2008) have led to an increase in vulnerability and exposure which are mainly responsible for those losses and the increase in severity of flood events (Pérez et al., 2015). Furthermore, this situation will probably become more precarious if some climate forecasts are met [IPCC, 2014; AEMET, 2015], and if the economic model fails to adopt efficient adaptive measures. Therefore, it is interesting to focus attention on social factors either within the present or future scenario in order to minimise the potential consequences and improve the adaptation. The main objective of this work focuses on the study of the evolution of the severity of the floods in the Spanish Mediterranean coast for the period (1960-2015). To do that, a statistical analysis of the data base [Gil et al., 2014; extended to the entire Spanish Mediterranean coast (MEDIFLOOD)] and a multiscale mapping (local, provincial and regional level) of the frequency of these events will take place in order to make comparisons and show spatiotemporal patterns according to the severity events evolution. Preliminary results show some interesting statistically significant

  18. Neutropenia: occurrence and management in women with breast cancer receiving chemotherapy

    Directory of Open Access Journals (Sweden)

    Talita Garcia do Nascimento

    2014-04-01

    Full Text Available OBJECTIVES: to identify the prevalence, and describe the management of, neutropenia throughout the chemotherapy treatment among women with breast cancer.METHODS: observational study, cycles of chemotherapy. 116 neutropenic events were recorded, and 63.3% of the patients presented neutropenia at some point of their treatment, 46.5% of these presenting grade II. The management used was temporary suspension between the cycles and the mean number of delays was 6 days. The study was prospective and longitudinal, where the evaluation of the hematological toxicities was undertaken at each cycle of chemotherapy, whether neoadjuvant or adjuvant.RESULTS: 79 women were included, who received 572 cycles. However, the reasons for the suspensions were the lack of a space in the chemotherapy center, followed by neutropenia.CONCLUSION: neutropenia is one of the most common and serious adverse events observed during the chemotherapy. Nursing must invest in research regarding this adverse event and in management strategies for organizing the public health system, so as to offer quality care.

  19. A patient with common glycogen storage disease type Ib mutations without neutropenia or neutrophil dysfunction

    NARCIS (Netherlands)

    Martens, DHJ; Kuijpers, TW; Maianski, NA; Rake, JP; Smit, GPA; Visser, G

    2006-01-01

    We describe a 16-year old boy with glycogen storage disease type Ib, homozygous for the common 1211-1212delCT mutation, who never experienced neutropenia, and did not suffer from frequent infections or inflammatory bowel disease. In addition, neutrophil function tests showed no abnormalities.

  20. Amphotericin B lipid soluble formulations versus amphotericin B in cancer patients with neutropenia

    DEFF Research Database (Denmark)

    Johansen, Helle Krogh; Gøtzsche, Peter C

    2014-01-01

    fever. OBJECTIVES: To compare the benefits and harms of lipid soluble formulations of amphotericin B with conventional amphotericin B in cancer patients with neutropenia. SEARCH METHODS: We searched PubMed from 1966 to 7 July 2014 and the reference lists of identified articles. SELECTION CRITERIA...

  1. G-CSF in Peg-IFN induced neutropenia in liver transplanted patients with HCV recurrence

    Institute of Scientific and Technical Information of China (English)

    Francesca Lodato; Francesco Azzaroli; Maria Rosa Tamè; Maria Di Girolamo; Federica Buonfiglioli; Natalia Mazzella; Paolo Cecinato; Enrico Roda; Giuseppe Mazzella

    2009-01-01

    AIM: To evaluate the efficacy of granulocyte colony stimulating factors (G-CSF) in liver transplanted patients with hepatitis C (HCV) recurrence and Pegylated-IFN α-2b induced neutropenia, and to evaluate the impact of G-CSF administration on virological response.METHODS: Sixty-eight patients undergoing antiviral treatment for post-liver transplantation (OLT) HCV recurrence were enrolled.All patients developing neutropenia received G-CSF.RESULTS: Twenty three (34%) received G-CSF.Mean neutrophil count at the onset of neutropenia was 700/mmc (range 400-750/mmc); after 1 mo of G-CSF it increased to 1210/mmc (range 300-5590/mmc) ( P < 0.0001).Three patients did not respond to G-CSF.Treatment duration was similar in neutropenic and non-neutropenic patients.No differences in the rate of discontinuation, infections or virological response were observed between the two groups.G-CSF was protective for the onset of de novo autoimmune hepatitis ( P < 0.003).CONCLUSION: G-CSF administration is effective in the case of Peg-IFN induced neutropenia increasing neutrophil count, prolonging treatment and leading to sustained virological response (SVR) rates comparable to non-neutropenic patients.It prevents the occurrence of de novo autoimmune hepatitis.

  2. The role of mannose-binding lectin (MBL) in paediatric oncology patients with febrile neutropenia

    NARCIS (Netherlands)

    F.N.J. Frakking; M.D. van de Wetering; N. Brouwer; K.M. Dolman; J. Geissler; B. Lemkes; H.N. Caron; T.W. Kuijpers

    2006-01-01

    Children with cancer often have fever during chemotherapy-induced neutropenia, but only some develop serious infectious complications. Mannose-binding lectin (MBL) deficiency might increase infection susceptibility in these children. MBL genotype and phenotype were prospectively determined in 110 pa

  3. [Esophageal aspergillosis in a patient with acute myelogenous leukemia and febrile neutropenia].

    Science.gov (United States)

    Besa, Santiago; Kattan, Eduardo; Cid, Ximena; Claro, Juan C

    2014-04-01

    Aspergillosis usually compromises the respiratory system, but can also affect others. We report a 46 yo female with acute myeloid leukemia, developed febrile neutropenia and dysphagia. Endoscopy revealed esophageal cytomegalovirus-like ulcers, but biopsies showed Aspergillus spp. It's important to consider aspergillosis in the differential diagnosis of esophageal lesions in high-risk patients.

  4. Mutation p.Leu354Pro in EDA causes severe hypohidrotic ectodermal dysplasia in a Chinese family.

    Science.gov (United States)

    Liu, Ying; Yu, Xiaoyan; Wang, Lei; Li, Chang; Archacki, Stephen; Huang, Changzheng; Liu, Jing Yu; Wang, Qing; Liu, Mugen; Tang, Zhaohui

    2012-01-10

    X-linked recessive hypohidrotic ectodermal dysplasia (XLHED) is characterized by the defective morphogenesis of teeth, hair, and eccrine sweat glands. It is associated with mutations in the EDA gene. Up to now, more than 100 mutations in the EDA gene have been reported to cause XLHED. The product of EDA gene is a trimeric type II transmembrane protein that belongs to the tumor necrosis factor (TNF) family of ligands. In this study, we identified a Chinese family with XLHED. Direct DNA sequencing of the whole coding region of EDA revealed a novel missense mutation, p.Leu354Pro in a patient affected with XLHED. This mutation was not found in either unaffected male individuals of the family or 168 normal controls. The substitution of Leu354 with Pro was found to be located in the TNF-like domain of EDA and may influence the epithelial signaling pathway required for the normal ectodermal development through altering the topology of EDA. Our finding broadens the spectrum of EDA mutations and may help to understand the molecular basis of XLHED and aid genetic counseling. PMID:22008666

  5. Analysis of causes and sequences of the accident on Fukushima NPP as a factor of sever accidents prevention in the vessel reactor

    International Nuclear Information System (INIS)

    In this monograph, the provisional analysis of the causes and sequences of the sever accidents on the Fukushima NPP is presented. The analysis of the possibility of the origin of extreme events connected with the flooding of Zaporizhzhia NPP industrial site, emergency of the steam-gas explosions on NPPs with WWER and other phenomena occurred under sever accidents was carried out. It was presented the authors original working-out on symptom-oriented approaches of sever accident initiating event list identification, on criteria substantiation of explosion safety and optimization of processes management at sever accidents, as well as on the methodological support of the accident beyond the design basis management at the WWER for prevention of their transition in the stage of sever accidents.

  6. Reprodaetion of an animal model of multiple intestinal injuries mimicking "lethal triad" caused by severe penetrating abdominal trauma

    Directory of Open Access Journals (Sweden)

    Peng-fei WANG

    2011-03-01

    Full Text Available Objective To reproduce an animal model of multi-intestinal injuries with "lethal triad" characterized by low body temperature,acidosis and coagulopathy.Methods Six female domestic outbred pigs were anesthetized,and the carotid artery and jugular vein were cannulated for monitoring the blood pressure and heart rate and for infusion of fluid.The animals were shot with a gun to create a severe penetrating abdominal trauma.Immediately after the shooting,50% of total blood volume(35ml/kg hemorrhage was drawn from the carotid artery in 20min.After a 40min shock period,4h of pre-hospital phase was mimicked by normal saline(NS resuscitation to maintain systolic blood pressure(SBP > 80mmHg or mean arterial pressure(MAP > 60mmHg.When SBP > 80mmHg or MAP > 60mmHg,no fluid infusion or additional bleeding was given.Hemodynamic parameters were recorded,and pathology of myocardium,lung,small intestine and liver was observed.Results There were multiple intestinal perforations(8-10 site injuries/pig leading to intra-abdominal contamination,mesenteric injury(1-2 site injuries/pig resulted in partial intestinal ischemia and intra-abdominal hemorrhage,and no large colon and mesenteric vascular injury.One pig died before the completion of the model establishment(at the end of pre-hospital resuscitation.The typical symptoms of trauma-induced hemorrhagic shock were observed in survival animals.Low temperature(33.3±0.5℃,acidosis(pH=7.242±0.064,and coagulopathy(protrombin time and activated partial thromboplasting time prolonged were observed after pre-hospital resuscitation.Pathology showed that myocardium,lung,small intestine and liver were severely injured.Conclusions A new model,simulating three stages of "traumatic hemorrhagic shock,pre-hospital recovery and hospital treatment" and inducing the "lethal triad" accompanied with abdominal pollution,has been successfully established.This model has good stability and high reproducibility.The survival animals can be

  7. Oak forest exploitation and black-locust invasion caused severe shifts in epiphytic lichen communities in Northern Italy.

    Science.gov (United States)

    Nascimbene, Juri; Marini, Lorenzo

    2010-10-15

    In the last two centuries, native European oak forests have undergone a dramatic decline related to increasing human pressure for agriculture and urbanization. Oak forests were either completely eradicated and transformed into agricultural landscapes or replaced by second-growth formations. Intensive forest management and the replacement of native forests with production forests or arable lands are recognized amongst the main threats to many lichens in Europe. In this study, we used historical information on the epiphytic lichen biota which was hosted in a native oak-dominated forest of Northern Italy to identify shifts of lichen communities due to the changes in land use which occurred during the last two centuries. We also compared the epiphytic lichen communities inhabiting remnant oak forests with those found in the habitats that have replaced native forests: black-locust forests and agrarian landscapes. Almost all the species sampled during the 19th century are now extinct. The loss of native habitat and the subsequent invasion by black locust were probably the most influential factors which affected the composition of lichen communities, causing the local extinction of most of the species historically recorded. Despite the fact that oak remnants host only a few species which were historically recorded, and that they currently are the lichen poorest habitat in the study region, they host lichen assemblages differing from those of black-locust forests and agrarian stands. In these habitats lichen assemblages are mainly composed of species adapted to well-lit, dry conditions and tolerating air pollution and eutrophication. This pattern is likely to be common also in other lowland and hilly regions throughout Northern Italy where oak forests are targeted among the habitats of conservation concern at the European level. For this reason, a national strategy for biodiversity conservation and monitoring of lowlands forests should provide the framework for local

  8. Severe Weather Caused by Heat Island and Sea Breeze Effects in the Metropolitan Area of São Paulo, Brazil

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    Felipe Vemado

    2016-01-01

    Full Text Available The Metropolitan Area of São Paulo (MASP is one of the most populated regions of the planet with one of the largest impervious regions as well. This research work aims to characterize MASP heat island (HI effect and its interaction with the local sea breeze (SB inflow in rainfall amounts and deep convection. The combined SB-HI produces direct circulation over the MASP and produces severe weather and socioeconomic impacts. All SB-HI episodes between 2005 and 2008 are identified and analyzed with surface and upper air measurements, weather radar, and satellite data. The current work indicates that intense SB-HI episodes are related to air and dew point temperatures above 30°C and 20°C, respectively, right after the passage of the SB front over MASP. Results indicate that the precipitation related to SB-HI episodes is up to 600 mm or about four times higher than that in rural or less urbanized areas in its surroundings. Measurements indicate that 74% of SB-HI episodes are related to NW winds in earlier afternoon hours. Moving cold fronts in southern Brazil tend to intensify the SB-HI circulation in MASP. A conceptual model of these patterns is presented in this paper.

  9. Osteogenesis imperfecta Type VI with severe bony deformities caused by novel compound heterozygous mutations in SERPINF1.

    Science.gov (United States)

    Cho, Sung Yoon; Ki, Chang-Seok; Sohn, Young Bae; Kim, Su Jin; Maeng, Se Hyun; Jin, Dong-Kyu

    2013-07-01

    Osteogenesis imperfecta (OI) comprises a heterogeneous group of disorders characterized by bone fragility, frequent fractures, and low bone mass. Dominantly inherited COL1A1 or COL1A2 mutations appear to be causative in the majority of OI types, but rare recessively inherited genes have also been reported. Recently, SERPINF1 has been reported as another causative gene in OI type VI. To date, only eight SERPINF1 mutations have been reported and all are homozygous. Our patient showed no abnormalities at birth, frequent fractures, osteopenia, and poor response on pamidronate therapy. At the time of her most recent evaluation, she was 8 yr old, and could not walk independently due to frequent lower-extremity fractures, resulting in severe deformity. No clinical signs were seen of hearing impairment, blue sclera, or dentinogenesis imperfecta. In this study, we describe the clinical and radiological findings of one Korean patient with novel compound heterozygous mutations (c.77dupC and c.421dupC) of SERPINF1.

  10. Lipoxin Inhibits Fungal Uptake by Macrophages and Reduces the Severity of Acute Pulmonary Infection Caused by Paracoccidioides brasiliensis

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    Laura R. R. Ribeiro

    2015-01-01

    Full Text Available Cysteinyl leukotrienes (CysLTs and lipoxins (LXs are lipid mediators that control inflammation, with the former inducing and the latter inhibiting this process. Because the role played by these mediators in paracoccidioidomycosis was not investigated, we aimed to characterize the role of CysLT in the pulmonary infection developed by resistant (A/J and susceptible (B10.A mice. 48 h after infection, elevated levels of pulmonary LTC4 and LXA4 were produced by both mouse strains, but higher levels were found in the lungs of susceptible mice. Blocking the CysLTs receptor by MTL reduced fungal loads in B10.A, but not in A/J mice. In susceptible mice, MLT treatment led to reduced influx of PMN leukocytes, increased recruitment of monocytes, predominant synthesis of anti-inflammatory cytokines, and augmented expression of 5- and 15-lipoxygenase mRNA, suggesting a prevalent LXA4 activity. In agreement, MTL-treated macrophages showed reduced fungal burdens associated with decreased ingestion of fungal cells. Furthermore, the addition of exogenous LX reduced, and the specific blockade of the LX receptor increased the fungal loads of B10.A macrophages. This study showed for the first time that inhibition of CysLTs signaling results in less severe pulmonary paracoccidioidomycosis that occurs in parallel with elevated LX activity and reduced infection of macrophages.

  11. Mutations in CIT, encoding citron rho-interacting serine/threonine kinase, cause severe primary microcephaly in humans.

    Science.gov (United States)

    Shaheen, Ranad; Hashem, Amal; Abdel-Salam, Ghada M H; Al-Fadhli, Fatima; Ewida, Nour; Alkuraya, Fowzan S

    2016-10-01

    Primary microcephaly is a clinical phenotype in which the head circumference is significantly reduced at birth due to abnormal brain development, primarily at the cortical level. Despite the marked genetic heterogeneity, most primary microcephaly-linked genes converge on mitosis regulation. Two consanguineous families segregating the phenotype of severe primary microcephaly, spasticity and failure to thrive had overlapping autozygomes in which exome sequencing identified homozygous splicing variants in CIT that segregate with the phenotype within each family. CIT encodes citron, an effector of the Rho signaling that is required for cytokinesis specifically in proliferating neuroprogenitors, as well as for postnatal brain development. In agreement with the critical role assigned to the kinase domain in effecting these biological roles, we show that both splicing variants predict variable disruption of this domain. The striking phenotypic overlap between CIT-mutated individuals and the knockout mice and rats that are specifically deficient in the kinase domain supports the proposed causal link between CIT mutation and primary microcephaly in humans. PMID:27503289

  12. Severe vitamin D deficiency in a case of primary hyperparathyroidism caused by parathyroid lipoadenoma, effect of 25OHD3 treatment.

    Science.gov (United States)

    Coen, G; Bondatti, F; de Matteis, A; Ballanti, P; Mazzaferro, S; Sardella, D; Smacchi, A

    1989-01-01

    This report describes the case of a 60-year-old woman with severe metabolic bone disease and fractures due to vitamin D deficiency and hyperparathyroidism. 25OHDH3 and 1,25(OH)2D3 serum levels were undetectable and increased immediately following 25OHD3 oral administration. Serum 1,25(OH)2D3 following vitamin D repletion reached values above the normal range, and remained elevated with strict dependence on the serum 25OHD3 levels. Parathyroid hormone and alkaline phosphatase decreased during treatment, without reaching normality during 1 year of observation. Bone biopsies before and after 8-month 25OHD3 treatment showed disappearance of the osteomalacic and hyperparathyroid lesions. During treatment an increase in serum and urine calcium and formation of renal stones were observed. The patient underwent neck exploration with the finding and removal of a lipoadenoma, a rare parathyroid tumor, followed by complete and permanent remission of the disease. In conclusion, this case is suggestive of the key role played by the long-term vitamin D status in the clinical expression of primary hyperparathyroidism. PMID:2615720

  13. Mycoplasma ovipneumoniae--a primary cause of severe pneumonia epizootics in the Norwegian Muskox (Ovibos moschatus) population.

    Science.gov (United States)

    Handeland, Kjell; Tengs, Torstein; Kokotovic, Branko; Vikøren, Turid; Ayling, Roger D; Bergsjø, Bjarne; Sigurðardóttir, Olöf G; Bretten, Tord

    2014-01-01

    The Norwegian muskox (Ovibos moschatus) population lives on the high mountain plateau of Dovre and originates from animals introduced from Greenland. In the late summers of 2006 and 2012, severe outbreaks of pneumonia with mortality rates of 25-30% occurred. During the 2012 epidemic high quality samples from culled sick animals were obtained for microbiological and pathological examinations. High throughput sequencing (pyrosequencing) of pneumonic lung tissue revealed high concentrations of Mycoplasma ovipneumoniae in all six animals examined by this method and Pasteurella multocida subsp. multocida in four animals, whereas no virus sequences could be identified. Mycoplasma ovipneumoniae and P. multocida multocida were also isolated by culture. Using real time PCR on lung swabs, M. ovipneumoniae was detected in all of the 19 pneumonic lungs examined. Gross pathological examination revealed heavy consolidations primarily in the cranial parts of the lungs and it also identified one case of otitis media. Histologically, lung lesions were characterized as acute to subacute mixed exudative and moderately proliferative bronchoalveolar pneumonia. Immunohistochemical (IHC) examination revealed high load of M. ovipneumoniae antigens within lung lesions, with particularly intensive staining in the neutrophils. Similar IHC finding were observed in archived lung tissue blocks from animals examined during the 2006 epidemic. An M. ovipneumoniae specific ELISA was applied on bio-banked muskox sera from stray muskoxen killed in the period 2004-2013 and sick muskoxen culled, as well as sera from wild reindeer (Rangifer tarandus tarandus) on Dovre and muskoxen from Greenland. Serology and mycoplasma culturing was also carried out on sheep that had been on pasture in the muskox area during the outbreak in 2012. Our findings indicated separate introductions of M. ovipneumoniae infection in 2006 and 2012 from infected co-grazing sheep. Salt licks shared by the two species were a

  14. Mycoplasma ovipneumoniae--a primary cause of severe pneumonia epizootics in the Norwegian Muskox (Ovibos moschatus population.

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    Kjell Handeland

    Full Text Available The Norwegian muskox (Ovibos moschatus population lives on the high mountain plateau of Dovre and originates from animals introduced from Greenland. In the late summers of 2006 and 2012, severe outbreaks of pneumonia with mortality rates of 25-30% occurred. During the 2012 epidemic high quality samples from culled sick animals were obtained for microbiological and pathological examinations. High throughput sequencing (pyrosequencing of pneumonic lung tissue revealed high concentrations of Mycoplasma ovipneumoniae in all six animals examined by this method and Pasteurella multocida subsp. multocida in four animals, whereas no virus sequences could be identified. Mycoplasma ovipneumoniae and P. multocida multocida were also isolated by culture. Using real time PCR on lung swabs, M. ovipneumoniae was detected in all of the 19 pneumonic lungs examined. Gross pathological examination revealed heavy consolidations primarily in the cranial parts of the lungs and it also identified one case of otitis media. Histologically, lung lesions were characterized as acute to subacute mixed exudative and moderately proliferative bronchoalveolar pneumonia. Immunohistochemical (IHC examination revealed high load of M. ovipneumoniae antigens within lung lesions, with particularly intensive staining in the neutrophils. Similar IHC finding were observed in archived lung tissue blocks from animals examined during the 2006 epidemic. An M. ovipneumoniae specific ELISA was applied on bio-banked muskox sera from stray muskoxen killed in the period 2004-2013 and sick muskoxen culled, as well as sera from wild reindeer (Rangifer tarandus tarandus on Dovre and muskoxen from Greenland. Serology and mycoplasma culturing was also carried out on sheep that had been on pasture in the muskox area during the outbreak in 2012. Our findings indicated separate introductions of M. ovipneumoniae infection in 2006 and 2012 from infected co-grazing sheep. Salt licks shared by the two

  15. ENU mutagenesis identifies mice with morbid obesity and severe hyperinsulinemia caused by a novel mutation in leptin.

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    Chen-Jee Hong

    Full Text Available BACKGROUND: Obesity is a multifactorial disease that arises from complex interactions between genetic predisposition and environmental factors. Leptin is central to the regulation of energy metabolism and control of body weight in mammals. METHODOLOGY/PRINCIPAL FINDINGS: To better recapitulate the complexity of human obesity syndrome, we applied N-ethyl-N-nitrosourea (ENU mutagenesis in combination with a set of metabolic assays in screening mice for obesity. Mapping revealed linkage to the chromosome 6 within a region containing mouse Leptin gene. Sequencing on the candidate genes identified a novel T-to-A mutation in the third exon of Leptin gene, which translates to a V145E amino acid exchange in the leptin propeptide. Homozygous Leptin(145E/145E mutant mice exhibited morbid obesity, accompanied by adipose hypertrophy, energy imbalance, and liver steatosis. This was further associated with severe insulin resistance, hyperinsulinemia, dyslipidemia, and hyperleptinemia, characteristics of human obesity syndrome. Hypothalamic leptin actions in inhibition of orexigenic peptides NPY and AgRP and induction of SOCS1 and SOCS3 were attenuated in Leptin(145E/145E mice. Administration of exogenous wild-type leptin attenuated hyperphagia and body weight increase in Leptin(145E/145E mice. However, mutant V145E leptin coimmunoprecipitated with leptin receptor, suggesting that the V145E mutation does not affect the binding of leptin to its receptor. Molecular modeling predicted that the mutated residue would form hydrogen bond with the adjacent residues, potentially affecting the structure and formation of an active complex with leptin receptor within that region. CONCLUSIONS/SIGNIFICANCE: Thus, our evolutionary, structural, and in vivo metabolic information suggests the residue 145 as of special function significance. The mouse model harboring leptin V145E mutation will provide new information on the current understanding of leptin biology and novel mouse

  16. Congenital and acquired neutropenias consensus guidelines on therapy and follow-up in childhood from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato-Oncologia Pediatrica).

    Science.gov (United States)

    Fioredda, Francesca; Calvillo, Michaela; Bonanomi, Sonia; Coliva, Tiziana; Tucci, Fabio; Farruggia, Piero; Pillon, Marta; Martire, Baldassarre; Ghilardi, Roberta; Ramenghi, Ugo; Renga, Daniela; Menna, Giuseppe; Pusiol, Anna; Barone, Angelica; Gambineri, Eleonora; Palazzi, Giovanni; Casazza, Gabriella; Lanciotti, Marina; Dufour, Carlo

    2012-02-01

    The management of congenital and acquired neutropenias presents some differences according to the type of the disease. Treatment with recombinant human granulocyte-colony stimulating factor (G-CSF) is not standardized and scanty data are available on the best schedule to apply. The frequency and the type of longitudinal controls in patients affected with neutropenias are not usually discussed in the literature. The Neutropenia Committee of the Marrow Failure Syndrome Group (MFSG) of the Associazione Italiana di Emato-Oncologia Pediatrica (AIEOP) elaborated this document following design and methodology formerly approved by the AIEOP board. The panel of experts reviewed the literature on the topic and participated in a conference producing a document that includes recommendations on neutropenia treatment and timing of follow-up.

  17. Performance of Interleukin-6 and Interleukin-8 serum levels in pediatric oncology patients with neutropenia and fever for the assessment of low-risk

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    Kontny Udo

    2008-03-01

    Full Text Available Abstract Background Patients with chemotherapy-related neutropenia and fever are usually hospitalized and treated on empirical intravenous broad-spectrum antibiotic regimens. Early diagnosis of sepsis in children with febrile neutropenia remains difficult due to non-specific clinical and laboratory signs of infection. We aimed to analyze whether IL-6 and IL-8 could define a group of patients at low risk of septicemia. Methods A prospective study was performed to assess the potential value of IL-6, IL-8 and C-reactive protein serum levels to predict severe bacterial infection or bacteremia in febrile neutropenic children with cancer during chemotherapy. Statistical test used: Friedman test, Wilcoxon-Test, Kruskal-Wallis H test, Mann-Whitney U-Test and Receiver Operating Characteristics. Results The analysis of cytokine levels measured at the onset of fever indicated that IL-6 and IL-8 are useful to define a possible group of patients with low risk of sepsis. In predicting bacteremia or severe bacterial infection, IL-6 was the best predictor with the optimum IL-6 cut-off level of 42 pg/ml showing a high sensitivity (90% and specificity (85%. Conclusion These findings may have clinical implications for risk-based antimicrobial treatment strategies.

  18. A novel mutation (4040-4045 nt. delA in exon 14 of the factor VIII gene causing severe hemophilia A

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    Habib Onsori

    2011-01-01

    Full Text Available Hemophilia A is an X-linked congenital bleeding disorder caused by Factor VIII deficiency. Different mutations including point mutations, deletions, insertions and inversions have been reported in the FVIII gene, which cause hemophilia A. In the current study, with the use of conformational sensitive gel electrophoresis (CSGE analysis, we report a novel 1-nt deletion in the A6 sequence at codons 1328-1330 (4040-4045 nt delA occurring in exon 14 of the FVIII gene in a seven-year-old Iranian boy with severe hemophilia A. This mutation that causes frameshift and premature stop-codon at 1331 has not previously been reported in the F8 Hemophilia A Mutation, Structure, Test and Resource Site (HAMSTeRS database.

  19. Aspergilosis pulmonar secundaria a neutropenia inducida por metimazol: reporte de un caso Pulmonary aspergillosis due to methimazole-induced neutropenia: a case report

    OpenAIRE

    Miguel E. Pinto; Claudia Banda; Carlos Seas

    2012-01-01

    Se reporta el caso de una paciente de 48 años de edad con diagnóstico reciente de enfermedad de Graves, quien acudió a emergencia por presentar fiebre, palpitaciones y dolor faríngeo. Su tratamiento regular incluía metimazol. Al ingreso, los análisis mostraron TSH suprimido, T4 libre elevado y neutropenia. La paciente fue hospitalizada, se administraron antibióticos y factor estimulante de colonia. Después de diez días de tratamiento, la paciente presentó leucocitosis, fiebre y hemoptisis. La...

  20. Features and prognosis of severe malaria caused by Plasmodium falciparum, Plasmodium vivax and mixed Plasmodium species in Papua New Guinean children.

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    Laurens Manning

    Full Text Available BACKGROUND: Mortality from severe pediatric falciparum malaria appears low in Oceania but Plasmodium vivax is increasingly recognized as a cause of complications and death. The features and prognosis of mixed Plasmodium species infections are poorly characterized. Detailed prospective studies that include accurate malaria diagnosis and detection of co-morbidities are lacking. METHODS AND FINDINGS: We followed 340 Papua New Guinean (PNG children with PCR-confirmed severe malaria (77.1% P. falciparum, 7.9% P. vivax, 14.7% P. falciparum/vivax hospitalized over a 3-year period. Bacterial cultures were performed to identify co-incident sepsis. Clinical management was under national guidelines. Of 262 children with severe falciparum malaria, 30.9%, 24.8% and 23.2% had impaired consciousness, severe anemia, and metabolic acidosis/hyperlactatemia, respectively. Two (0.8% presented with hypoglycemia, seven (2.7% were discharged with neurologic impairment, and one child died (0.4%. The 27 severe vivax malaria cases presented with similar phenotypic features to the falciparum malaria cases but respiratory distress was five times more common (P=0.001; one child died (3.7%. The 50 children with P. falciparum/vivax infections shared phenotypic features of mono-species infections, but were more likely to present in deep coma and had the highest mortality (8.0%; P=0.003 vs falciparum malaria. Overall, bacterial cultures were positive in only two non-fatal cases. 83.6% of the children had alpha-thalassemia trait and seven with coma/impaired consciousness had South Asian ovalocytosis (SAO. CONCLUSIONS: The low mortality from severe falciparum malaria in PNG children may reflect protective genetic factors other than alpha-thalassemia trait/SAO, good nutrition, and/or infrequent co-incident sepsis. Severe vivax malaria had similar features but severe P. falciparum/vivax infections were associated with the most severe phenotype and worst prognosis.

  1. [Temozolomide, an oral chemotherapeutic agent with potential severe toxicity

    NARCIS (Netherlands)

    Soetekouw, P.M.M.B.; Gijtenbeek, J.M.M.; Maazen, R.W.M. van der; Kappelle, A.C.; Herpen, C.M.L. van

    2007-01-01

    Two patients, a 58-year-old man and a 55-year-old woman, both under treatment for glioblastoma multiforme, were admitted with fever and neutropenia a few weeks after starting to take the oncolytic agent temozolomide. The man died of a cerebral haemorrhage against a background of severe thrombocytope

  2. Atypical radiological and intraoperative findings of acute cerebral hemorrhage caused by ruptured cerebral aneurysm in a patient with severe chronic anemia.

    Science.gov (United States)

    Matano, Fumihiro; Murai, Yasuo; Nakagawa, Shunsuke; Kato, Taisei; Kitamura, Takayuki; Sekine, Tetsuro; Takagi, Ryo; Teramoto, Akira

    2014-01-01

    Acute intracerebral hemorrhage (ICH) associated with mild anemia is commonly observed on radiological examination, and there are several reports of ruptured aneurysms occurring with ICH but without accompanying subarachnoid hemorrhage. However, the relationship among computed tomography (CT), magnetic resonance imaging (MRI), and intraoperative findings of ICH caused by ruptured cerebral aneurysm in patients with severe chronic anemia has been rarely reported and is poorly understood. Here, we report atypical radiological and intraoperative findings of acute ICH caused by ruptured cerebral aneurysm in a patient with severe chronic anemia. A 64-year-old man with anemia was admitted to our hospital after he experienced left hemiparesis and a disturbance of consciousness. At a referring institution, he showed evidence of macrocytic anemia (white blood cell count, 9,000/μL; red blood cell count, 104×10(4)/μL; hemoglobin, 4.0 g/dL; hematocrit, 12.2%; and platelet count, 26.6×10(4)/μL). Both CT and MRI showed a right frontal ICH. The outer ring of the hematoma appeared as low-density area on CT, a low-intensity area on T1-weighted MRI, and a high-intensity area on T2-weighted MRI with a serous component. The patient received a blood transfusion and underwent surgical removal of the hematoma the following day. The white serous effusion visualized with CT and MRI was identified as a blood clot in the hematoma cavity. The blood that leaks from blood vessels appears as a high-intensity area on CT because it undergoes plasma absorption in a solidification shrinkage process, and is, therefore, concentrated. Although we did not examine the white effusion to determine if serous components were present, we speculated that the effusion may have contained serous components. Therefore, we removed the part of the effusion that appeared as a low-density area on CT. The presence of ICH without subarachnoid hemorrhage suggested the possible adhesion and rupture of a previous

  3. Clericuzio-type Poikiloderma with Neutropenia Syndrome in a Turkish Family: a Three Report of Siblings with Mutation in the C16orf57 gene

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    Turkan Patiroglu

    2015-10-01

    Full Text Available Clericuzio-type poikiloderma with neutropenia (PN is characterized by poikiloderma, non-cyclic  neutropenia,  recurrent  sinopulmonary  infections,  pachyonychia,  and  palmo- plantar hyperkeratosis. Mutations in the C16orf57 gene, which is located on chromosome 16q13, have been identified as the cause of PN. PN was first described by Clericuzio in Navajo Indians. Herein, we reported the clinical presentations and laboratory investigations of PN in three siblings from Turkey.The older siblings presented with typical cutaneous poikiloderma, plantar keratoderma, pachyonychia of toenails, and recurrent upper respiratory infections. As the most affected patient, in addition to classic manifestations, the youngest sibling had recurrent pneumonia, hepatosplenomegaly, dental caries, failure to thrive, and hand malformation.Genetic study revealed a homozygous mutation (c.531delA in the C16orf57 gene in siblings.With the presented study, we aimed to draw attention to PN which can be a predisposing factor to malignancies.

  4. Inter-Ethnic Differences in Quantified Coronary Artery Disease Severity and All-Cause Mortality among Dutch and Singaporean Percutaneous Coronary Intervention Patients.

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    Crystel M Gijsberts

    Full Text Available Coronary artery disease (CAD is a global problem with increasing incidence in Asia. Prior studies reported inter-ethnic differences in the prevalence of CAD rather than the severity of CAD. The angiographic "synergy between percutaneous coronary intervention (PCI with taxus and cardiac surgery" (SYNTAX score quantifies CAD severity and predicts outcomes. We studied CAD severity and all-cause mortality in four globally populous ethnic groups: Caucasians, Chinese, Indians and Malays.We quantified SYNTAX scores of 1,000 multi-ethnic patients undergoing PCI in two tertiary hospitals in the Netherlands (Caucasians and Singapore (Chinese, Indians and Malays. Within each ethnicity we studied 150 patients with stable CAD and 100 with ST-elevated myocardial infarction (STEMI. We made inter-ethnic comparisons of SYNTAX scores and all-cause mortality.Despite having a younger age (mean age Indians: 56.8 and Malays: 57.7 vs. Caucasians: 63.7 years, multivariable adjusted SYNTAX scores were significantly higher in Indians and Malays than Caucasians with stable CAD: 13.4 [11.9-14.9] and 13.4 [12.0-14.8] vs. 9.4 [8.1-10.8], p<0.001. Among STEMI patients, SYNTAX scores were highest in Chinese and Malays: 17.7 [15.9-19.5] and 18.8 [17.1-20.6] vs. 15.5 [13.5-17.4] and 12.7 [10.9-14.6] in Indians and Caucasians, p<0.001. Over a median follow-up of 709 days, 67 deaths (stable CAD: 37, STEMI: 30 occurred. Among STEMI patients, the SYNTAX score independently predicted all-cause mortality: HR 2.5 [1.7-3.8], p<0.001 for every 10-point increase. All-cause mortality was higher in Indian and Malay STEMI patients than Caucasians, independent of SYNTAX score (adjusted HR 7.2 [1.5-34.7], p=0.01 and 5.8 [1.2-27.2], p=0.02.Among stable CAD and STEMI patients requiring PCI, CAD is more severe in Indians and Malays than in Caucasians, despite having a younger age. Moreover, Indian and Malay STEMI patients had a greater adjusted risk of all-cause mortality than Caucasians

  5. Comparison of anti-anaerobic antimicrobial strategies in cancer patients with febrile neutropenia and gastrointestinal symptoms

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    Rosa, Regis G; dos Santos, Rodrigo P; Goldani, Luciano Z.

    2014-01-01

    Background The current study sought to compare 28-day mortality rates in cancer patients with febrile neutropenia (FN) and gastrointestinal (GI) symptoms who underwent monotherapy using an antibiotic with antipseudomonal and anti-anaerobic activity (piperacillin-tazobactam or a carbapenem) and a group treated with a combination of cefepime-metronidazole. Findings We performed a prospective cohort study in a single tertiary hospital from October 2009 to August 2011. All consecutive adult cance...

  6. Role of recombinant granulocyte-macrophage colony - stimulating factors in reducing the duration of neutropenia

    International Nuclear Information System (INIS)

    Objective: To evaluate the role of recombinant Granulocyte-Macrophage Colony-Stimulating Factor (rGM-CSF) in reducing the duration of neutropenia and hospital stay after induction chemotherapy in acute myeloid leukemia (AML). Design: A randomised control trial. Place and Duration of Study: The study was carried out at Liaquat National Postgraduate Medical Center, Karachi from December 1995 to January 1999. Subjects and Methods: Twenty two newly diagnosed cases of AML< 11 males and 11 females with median age of 27.5(6-60) years were selected. The induction chemotherapy given was Doxorubicin 45 mg/m2/day intravenous for three consecutive days (day 1-3) and Ara C 100mg/m2/day intravenous infusion for seven consecutive days (day 1-7). Bone marrow aspiration was repeated on day 7 to exclude the presence of residual blast cells. The patients were then randomized to receive rGM-CSF in a dose of 7 micrograms /kg/day subcutaneously (Group-A) or placebo (Group-B). Duration of neutropenia (ANC<0.5*109/L) and length of hospital stay was recorded. Results: In Group-A mean duration of neutropenia was 19.3 days which is statistically non-significant (p=<0.05) as compared to Group-B i. e. 17.4 days and mean duration of hospital stay was 22.2 days which is also statistically non-significant (p=<0.05) as compared to Group-B i. e. 19.6 days. Conclusion: rGM-CSF failed to reduce the duration of neutropenia and hospital stay after induction chemotherapy in AML. (author)

  7. Clopidogrel-Induced Neutropenia after Coronary Stenting: Is Cilostazol a Good Alternative?

    OpenAIRE

    Massimo Montalto; Italo Porto; Antonella Gallo; Claudia Camaioni; Roberta Della Bona; Antonio Grieco; Filippo Crea; Raffaele Landolfi

    2011-01-01

    Dual antiplatelet therapy with aspirin plus thienopyridines has become the standard treatment of patients undergoing coronary stenting. Clopidogrel has mostly replaced the use of ticlopidine due to its more favourable adverse event profile. However, also the use of clopidogrel is not without side effects. Clopidogrel major adverse events are represented by marrow suppression, manifesting with aplastic anaemia, thrombocytopenia and neutropenia. When clopidogrel toxicity occurs, there are few a...

  8. The Effect of Recombinant Granulocyte Colony-Stimulating Factor on Oral and Periodontal Manifestations in a Patient with Cyclic Neutropenia: A Case Report

    Directory of Open Access Journals (Sweden)

    Sergio Matarasso

    2009-01-01

    Full Text Available Cyclic Neutropenia (CN is characterized by recurrent infections, fever, oral ulcerations, and severe periodontitis as result of the reduced host defences. The previous studies have established the effectiveness of recombinant granulocyte colony-stimulating factor (GCSF to increase the number and the function of neutrophils in the peripheral blood in this disease. In a 20-year-old Caucasian female with a diagnosis of cyclic neutropenia, oral clinical examination revealed multiple painful ulcerations of the oral mucosa, poor oral hygiene conditions, marginal gingivitis, and moderate periodontitis. The patient received a treatment with G-CSF (Pegfilgrastim, 6 mg/month in order to improve her immunological status. Once a month nonsurgical periodontal treatment was carefully performed when absolute neutrophil count (ANC was ≥500/L. The treatment with G-CSF resulted in a rapid increase of circulating neutrophils that, despite its short duration, leaded to a reduction in infection related events and the resolution of the multiple oral ulcerations. The disappearance of oral pain allowed an efficacy nonsurgical treatment and a normal tooth brushing that determined a reduction of probing depth (PD≤4 mm and an improvement of the oral hygiene conditions recorded at 6-month follow-up.

  9. Outpatient management of febrile neutropenia: time to revise the present treatment strategy

    DEFF Research Database (Denmark)

    Carstensen, M.; Sørensen, Jens Benn

    2008-01-01

    We reviewed medical literature on the efficacy and safety of outpatient versus hospital-based therapy of low-risk febrile neutropenia in adult cancer patients. A PubMed search for all studies evaluating the outpatient treatment of adults diagnosed with solid tumors who suffered from low-risk febr...... stable; they have no organ failure, they are able to take oral medications, and they do not suffer from acute leukemia. Low-risk prediction also may be based on the Multinational Association for Supportive Care in Cancer risk index Udgivelsesdato: 2008/5......We reviewed medical literature on the efficacy and safety of outpatient versus hospital-based therapy of low-risk febrile neutropenia in adult cancer patients. A PubMed search for all studies evaluating the outpatient treatment of adults diagnosed with solid tumors who suffered from low-risk...... treatment failure (P risk febrile neutropenia is safe, effective, and comparable to standard hospital-based therapy. Patients at low risk are outpatients and are hemodynamically...

  10. Review of five investigation committees' reports on the Fukushima Dai-ichi Nuclear Power Plant severe accident. Focusing on accident progression and causes

    International Nuclear Information System (INIS)

    On March 11, 2011, the Tohoku District-off the Pacific Ocean Earthquake and the subsequent tsunami resulted in the severe core damage at TEPCO's Fukushima Dai-ichi Nuclear Power Station Units 1-3, involving hydrogen explosions at Units 1, 3, and 4 and the large release of radioactive materials to the environment. Four independent committees were established by the Japanese government, the Diet of Japan, the Rebuild Japan Initiative Foundation, and TEPCO to investigate the accident and published their respective reports. Also, the Nuclear and Industrial Safety Agency carried out an analysis of accident causes to obtain the lessons learned from the accident and made its report public. This article reviews the reports and clarifies the differences in their positions, from the technological point of view, focusing on the accident progression and causes. Moreover, the undiscussed issues are identified to provide insights useful for the near-term regulatory activities including accident investigation by the Nuclear Regulation Authority. (author)

  11. Mutations in CDC14A, Encoding a Protein Phosphatase Involved in Hair Cell Ciliogenesis, Cause Autosomal-Recessive Severe to Profound Deafness.

    Science.gov (United States)

    Delmaghani, Sedigheh; Aghaie, Asadollah; Bouyacoub, Yosra; El Hachmi, Hala; Bonnet, Crystel; Riahi, Zied; Chardenoux, Sebastien; Perfettini, Isabelle; Hardelin, Jean-Pierre; Houmeida, Ahmed; Herbomel, Philippe; Petit, Christine

    2016-06-01

    By genetic linkage analysis in a large consanguineous Iranian family with eleven individuals affected by severe to profound congenital deafness, we were able to define a 2.8 Mb critical interval (at chromosome 1p21.2-1p21.1) for an autosomal-recessive nonsyndromic deafness locus (DFNB). Whole-exome sequencing allowed us to identify a CDC14A biallelic nonsense mutation, c.1126C>T (p.Arg376(∗)), which was present in the eight clinically affected individuals still alive. Subsequent screening of 115 unrelated individuals affected by severe or profound congenital deafness of unknown genetic cause led us to identify another CDC14A biallelic nonsense mutation, c.1015C>T (p.Arg339(∗)), in an individual originating from Mauritania. CDC14A encodes a protein tyrosine phosphatase. Immunofluorescence analysis of the protein distribution in the mouse inner ear showed a strong labeling of the hair cells' kinocilia. By using a morpholino strategy to knockdown cdc14a in zebrafish larvae, we found that the length of the kinocilia was reduced in inner-ear hair cells. Therefore, deafness caused by loss-of-function mutations in CDC14A probably arises from a morphogenetic defect of the auditory sensory cells' hair bundles, whose differentiation critically depends on the proper growth of their kinocilium.

  12. Novel mutations in DNAJB6 gene cause a very severe early-onset limb-girdle muscular dystrophy 1D disease.

    Science.gov (United States)

    Palmio, Johanna; Jonson, Per Harald; Evilä, Anni; Auranen, Mari; Straub, Volker; Bushby, Kate; Sarkozy, Anna; Kiuru-Enari, Sari; Sandell, Satu; Pihko, Helena; Hackman, Peter; Udd, Bjarne

    2015-11-01

    DNAJB6 is the causative gene for limb-girdle muscular dystrophy 1D (LGMD1D). Four different coding missense mutations, p.F89I, p.F93I, p.F93L, and p.P96R, have been reported in families from Europe, North America and Asia. The previously known mutations cause mainly adult-onset proximal muscle weakness with moderate progression and without respiratory involvement. A Finnish family and a British patient have been studied extensively due to a severe muscular dystrophy. The patients had childhood-onset LGMD, loss of ambulation in early adulthood and respiratory involvement; one patient died of respiratory failure aged 32. Two novel mutations, c.271T > A (p.F91I) and c.271T > C (p.F91L), in DNAJB6 were identified by whole exome sequencing as a cause of this severe form of LGMD1D. The results were confirmed by Sanger sequencing. The anti-aggregation effect of the mutant DNAJB6 was investigated in a filter-trap based system using transient transfection of mammalian cell lines and polyQ-huntingtin as a model for an aggregation-prone protein. Both novel mutant proteins show a significant loss of ability to prevent aggregation. PMID:26338452

  13. Mutations in CDC14A, Encoding a Protein Phosphatase Involved in Hair Cell Ciliogenesis, Cause Autosomal-Recessive Severe to Profound Deafness.

    Science.gov (United States)

    Delmaghani, Sedigheh; Aghaie, Asadollah; Bouyacoub, Yosra; El Hachmi, Hala; Bonnet, Crystel; Riahi, Zied; Chardenoux, Sebastien; Perfettini, Isabelle; Hardelin, Jean-Pierre; Houmeida, Ahmed; Herbomel, Philippe; Petit, Christine

    2016-06-01

    By genetic linkage analysis in a large consanguineous Iranian family with eleven individuals affected by severe to profound congenital deafness, we were able to define a 2.8 Mb critical interval (at chromosome 1p21.2-1p21.1) for an autosomal-recessive nonsyndromic deafness locus (DFNB). Whole-exome sequencing allowed us to identify a CDC14A biallelic nonsense mutation, c.1126C>T (p.Arg376(∗)), which was present in the eight clinically affected individuals still alive. Subsequent screening of 115 unrelated individuals affected by severe or profound congenital deafness of unknown genetic cause led us to identify another CDC14A biallelic nonsense mutation, c.1015C>T (p.Arg339(∗)), in an individual originating from Mauritania. CDC14A encodes a protein tyrosine phosphatase. Immunofluorescence analysis of the protein distribution in the mouse inner ear showed a strong labeling of the hair cells' kinocilia. By using a morpholino strategy to knockdown cdc14a in zebrafish larvae, we found that the length of the kinocilia was reduced in inner-ear hair cells. Therefore, deafness caused by loss-of-function mutations in CDC14A probably arises from a morphogenetic defect of the auditory sensory cells' hair bundles, whose differentiation critically depends on the proper growth of their kinocilium. PMID:27259055

  14. Aspergilosis pulmonar secundaria a neutropenia inducida por metimazol: reporte de un caso Pulmonary aspergillosis due to methimazole-induced neutropenia: a case report

    Directory of Open Access Journals (Sweden)

    Miguel E. Pinto

    2012-06-01

    Full Text Available Se reporta el caso de una paciente de 48 años de edad con diagnóstico reciente de enfermedad de Graves, quien acudió a emergencia por presentar fiebre, palpitaciones y dolor faríngeo. Su tratamiento regular incluía metimazol. Al ingreso, los análisis mostraron TSH suprimido, T4 libre elevado y neutropenia. La paciente fue hospitalizada, se administraron antibióticos y factor estimulante de colonia. Después de diez días de tratamiento, la paciente presentó leucocitosis, fiebre y hemoptisis. La tomografía de tórax mostró una cavidad con múltiples nódulos en el lóbulo superior derecho. Los cultivos fueron positivos a Aspergillus fumigatus y Aspergillus flavus. Se inició tratamiento con anfotericina B y luego se cambió a voriconazol, a pesar de lo cual no hubo mejoría del cuadro. La paciente falleció por falla multiorgánica.A 48-year old woman with a recent diagnosis of Graves’ disease arrived at the emergency room with fever, palpitations, and a sore throat. Her regular treatment included methimazole. On admission, laboratory results showed suppressed TSH, elevated free thyroxine, and neutropenia. She was admitted and started on antibiotics and granulocyte-macrophage colony stimulating factor (gm-csf. After ten days, the patient developed leukocytosis, fever, and hemoptysis. Chest CT scan showed a lung cavity with multiple nodules in the upper right lobe. Cultures from a lung biopsy were positive for Aspergillus Fumigatus and Aspergillus Flavus. Amphotericin B was started but then switched to voriconazole, with both treatments failing to result in clinical improvement. The patient died of multi-organ failure.

  15. Prevalence of Resistant Gram-Negative Bacilli in Bloodstream Infection in Febrile Neutropenia Patients Undergoing Hematopoietic Stem Cell Transplantation: A Single Center Retrospective Cohort Study.

    Science.gov (United States)

    Wang, Ling; Wang, Ying; Fan, Xing; Tang, Wei; Hu, Jiong

    2015-11-01

    Bloodstream infection (BSI) is an important cause of morbidity and mortality in patients undergoing hematopoietic stem cell transplantation (HSCT). To evaluate the causative bacteria and identify risk factors for BSI associated mortality in febrile neutropenia patients undergoing HSCT, we collected the clinical and microbiological data from patients underwent HSCT between 2008 and 2014 and performed a retrospective analysis. Throughout the study period, among 348 episodes of neutropenic fever in patients underwent HSCT, 89 episodes in 85 patients had microbiological defined BSI with a total of 108 isolates. Gram-negative bacteria (GNB) were the most common isolates (76, 70.3%) followed by gram-positive bacteria (GPB, 29, 26.9%) and fungus (3, 2.8%). As to the drug resistance, 26 multiple drug resistance (MDR) isolates were identified. Resistant isolates (n = 23) were more common documented in GNB, mostly Escherichia coli (9/36, 25%) and Klebsiella pneumonia (6/24, 25%). A total of 12 isolated were resistant to carbapenem including 4 K pneumoniae (4/24, 16.7%), 3 Stenotrophomonas maltophilia, and 1 Pseudomonas aeruginosa and other 4 GNB isolates (Citrobacter freumdii, Pseudomonas stutzeri, Acinetobacter baumanii, and Chryseobacterium indologenes). As to the GPB, only 3 resistant isolates were documented including 2 methicillin-resistant isolates (Staphylococcus hominis and Arcanobacterium hemolysis) and 1 vancomycin-resistant Enterococcus faecium. Among these 85 patients with documented BSI, 11 patients died of BSI as primary or associated cause with a BSI-related mortality of 13.1 ± 3.7% and 90-day overall survival after transplantation at 80.0 ± 4.3%. Patients with high-risk disease undergoing allo-HSCT, prolonged neutropenia (≥15 days) and infection with carbapenem-resistant GNB were associated with BSI associated mortality in univariate and multivariate analyses. Our report revealed a prevalence of GNB in BSI of neutropenic patients undergoing

  16. Prevalence of Resistant Gram-Negative Bacilli in Bloodstream Infection in Febrile Neutropenia Patients Undergoing Hematopoietic Stem Cell Transplantation: A Single Center Retrospective Cohort Study.

    Science.gov (United States)

    Wang, Ling; Wang, Ying; Fan, Xing; Tang, Wei; Hu, Jiong

    2015-11-01

    Bloodstream infection (BSI) is an important cause of morbidity and mortality in patients undergoing hematopoietic stem cell transplantation (HSCT). To evaluate the causative bacteria and identify risk factors for BSI associated mortality in febrile neutropenia patients undergoing HSCT, we collected the clinical and microbiological data from patients underwent HSCT between 2008 and 2014 and performed a retrospective analysis. Throughout the study period, among 348 episodes of neutropenic fever in patients underwent HSCT, 89 episodes in 85 patients had microbiological defined BSI with a total of 108 isolates. Gram-negative bacteria (GNB) were the most common isolates (76, 70.3%) followed by gram-positive bacteria (GPB, 29, 26.9%) and fungus (3, 2.8%). As to the drug resistance, 26 multiple drug resistance (MDR) isolates were identified. Resistant isolates (n = 23) were more common documented in GNB, mostly Escherichia coli (9/36, 25%) and Klebsiella pneumonia (6/24, 25%). A total of 12 isolated were resistant to carbapenem including 4 K pneumoniae (4/24, 16.7%), 3 Stenotrophomonas maltophilia, and 1 Pseudomonas aeruginosa and other 4 GNB isolates (Citrobacter freumdii, Pseudomonas stutzeri, Acinetobacter baumanii, and Chryseobacterium indologenes). As to the GPB, only 3 resistant isolates were documented including 2 methicillin-resistant isolates (Staphylococcus hominis and Arcanobacterium hemolysis) and 1 vancomycin-resistant Enterococcus faecium. Among these 85 patients with documented BSI, 11 patients died of BSI as primary or associated cause with a BSI-related mortality of 13.1 ± 3.7% and 90-day overall survival after transplantation at 80.0 ± 4.3%. Patients with high-risk disease undergoing allo-HSCT, prolonged neutropenia (≥15 days) and infection with carbapenem-resistant GNB were associated with BSI associated mortality in univariate and multivariate analyses. Our report revealed a prevalence of GNB in BSI of neutropenic patients undergoing

  17. Severe glomerular disease in juvenile grey snapper Lutjanus griseus L. in the Gulf of Mexico caused by the myxozoan Sphaerospora motemarini n. sp.

    Science.gov (United States)

    Holzer, Astrid S; Pecková, Hanka; Patra, Sneha; Brennan, Nathan P; Yanes-Roca, Carlos; Main, Kevan L

    2013-12-01

    In the eastern Gulf of Mexico, off the coast of Florida, grey snapper, Lutjanus griseus was found to be infected with the myxozoan parasite Sphaerospora motemarini n. sp., with high prevalence (83%) and intensity of infection occuring in age-0 fish, and with parasite levels decreasing with age (age-1 snapper 40%; age-2 snapper 0%). The morphological, molecular and phylogenetic characterisation of the myxozoan showed that it is a member of the typically marine, polysporoplasmid Sphaerospora spp. which form a subclade within the Sphaerospora sensu stricto clade of myxozoans, which is characterised by large expansion segments in their SSU rDNA sequences. Presporogonic stages of S. motemarini n. sp. were detected in the blood, using PCR. Pseudoplasmodia and spores were found to develop in the renal corpuscles of the host, causing their massive expansion. Macroscopic and histopathological changes were observed in age-0 fish and show that S. motemarini n. sp. causes severe glomerulonephritis in L. griseus leading to a compromised host condition, which makes it more susceptible to stress (catch-and-release, predators, water quality) and can result in mortalities. These results are discussed in relation to the exploitation of grey snapper populations by commercial and recreational fisheries and with the observed increased mortalities with temperature along the coast of Florida. In the future, we would like to determine prevalence and intensity of infection with S. motemarini n. sp. in juvenile L. griseus in different areas of the Gulf of Mexico in order to be able to estimate the temperature dependence of S. motemarini n. sp. proliferation and to be able to predict its distribution and severity during climatic changes in the Gulf.

  18. L712V mutation in the androgen receptor gene causes complete androgen insensitivity syndrome due to severe loss of androgen function.

    Science.gov (United States)

    Rajender, Singh; Gupta, Nalini J; Chakrabarty, Baidyanath; Singh, Lalji; Thangaraj, Kumarasamy

    2013-12-11

    Inability to respond to the circulating androgens is named as androgen insensitivity syndrome (AIS). Mutations in the androgen receptor (AR) gene are the most common cause of AIS. A cause and effect relationship between some of these mutations and the AIS phenotype has been proven by in vitro studies. Several other mutations have been identified, but need to be functionally validated for pathogenicity. Screening of the AR mutations upon presumptive diagnosis of AIS is recommended. We analyzed a case of complete androgen insensitivity syndrome (CAIS) for mutations in the AR gene. Sequencing of the entire coding region revealed C>G mutation (CTT-GTT) at codon 712 (position according to the NCBI database) in exon 4 of the gene, resulting in replacement of leucine with valine in the ligand-binding domain of the AR protein. No incidence of this mutation was observed in 230 normal male individuals analyzed for comparison. In vitro androgen binding and transactivation assays using mutant clone showed approximately 71% loss of ligand binding and about 76% loss of transactivation function. We conclude that CAIS in this individual was due to L712V substitution in the androgen receptor protein.

  19. Disease-associated mutations in the actin-binding domain of filamin B cause cytoplasmic focal accumulations correlating with disease severity

    DEFF Research Database (Denmark)

    Daniel, Philip B; Morgan, Tim; Alanay, Yasemin;

    2012-01-01

    Dominant missense mutations in FLNB, encoding the actin-cross linking protein filamin B (FLNB), cause a broad range of skeletal dysplasias with varying severity by an unknown mechanism. Here these FLNB mutations are shown to cluster in exons encoding the actin-binding domain (ABD) and filamin rep...... actin-binding activity but suggest that substitutions affecting repeats near the flexible hinge region of FLNB precipitate the same phenotypes through a different mechanism....... repeats surrounding the flexible hinge 1 region of the FLNB rod domain. Despite being positioned in domains that bind actin, it is unknown if these mutations perturb cytoskeletal structure. Expression of several full-length FLNB constructs containing ABD mutations resulted in the appearance of actin...... exception disease-associated substitutions surrounding hinge 1 demonstrated no tendency to form actin-filamin foci. The exception, a substitution in filamin repeat 6, lies within a region previously implicated in filamin-actin binding. These data are consistent with mutations in the ABD conferring enhanced...

  20. [Septic shock following platelet transfusion contaminated with Citrobacter koseri in a child with postchemotherapy febrile neutropenia].

    Science.gov (United States)

    Tichit, R; Saumet, L; Marchandin, H; Haouy, S; Latry, P; Sirvent, N

    2016-01-01

    The bacterial transfusion risk is currently the greatest infectious risk of blood transfusion. We report the case of a child with postchemotherapy febrile neutropenia who presented septic shock following platelet transfusion contaminated with Citrobacter koseri. The life-threatening development could have been avoided by strict compliance with good clinical practice. The stability of mortality rates due to adverse effects of bacterial proliferation during platelet transfusions in France since 1994 calls for optimization of all preventive measures throughout the transfusion chain and perfect knowledge of transfusion rules by medical staff and care givers.

  1. An international, multicenter, prospective, observational study of neutropenia in patients being treated with lenalidomide + dexamethasone for relapsed or relapsed/refractory multiple myeloma (RR-MM).

    Science.gov (United States)

    Leleu, Xavier; Terpos, Evangelos; Sanz, Ramón García; Cooney, Julian; O'Gorman, Peter; Minarik, Jiri; Greil, Richard; Williams, Catherine; Gray, Diep; Szabo, Zsolt

    2016-08-01

    Neutropenia is a well-known dose-limiting toxicity associated with lenalidomide plus dexamethasone treatment in patients with multiple myeloma; however, little is known about its management and associated outcomes in the real world setting. The present prospective, multicenter, observational study evaluated the incidence, management, and outcomes of grade 3/4 neutropenia in patients with relapsed or relapsed/refractory multiple myeloma who initiated treatment with lenalidomide plus dexamethasone. Of 198 patients, 62 (31%, 95% CI: 25, 38) experienced grade 3/4 neutropenia, and half of these patients experienced 3 or more events during the 12-month observational period. Grade 3/4 neutropenia occurred throughout lenalidomide treatment, with a median time to first event of 8.8 weeks (Q1, Q3: 5.9, 17.3). In a multivariate analysis, diagnosis of relapsed and refractory disease was associated with grade 3/4 neutropenia. Lenalidomide exposure reduction, use of G-CSF, unplanned hospitalization, and outpatient clinic visits were more common in patients who experienced grade 3/4 neutropenia than in those who did not. In conclusion, grade 3/4 neutropenia is a common toxicity and patients are at continued risk throughout treatment with lenalidomide and dexamethasone. Further efforts should be made to improve the recommendations for neutropenia management in this population. Am. J. Hematol. 91:806-811, 2016. © 2016 Wiley Periodicals, Inc. PMID:27169523

  2. The diagnostic value of CRP, IL-8, PCT, and sTREM-1 in the detection of bacterial infections in pediatric oncology patients with febrile neutropenia

    NARCIS (Netherlands)

    Miedema, Karin G. E.; de Bont, Eveline S. J. M.; Elferink, Rob F. M. Oude; van Vliet, Michel J.; Nijhuis, Claudi S. M. Oude; Kamps, Willem A.; Tissing, Wim J. E.

    2011-01-01

    In this study, we evaluated C-reactive protein (CRP), interleukin (IL)-8, procalcitonin (PCT), and soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) as predictors for bacterial infection in febrile neutropenia, plus their usefulness in febrile neutropenia during chemotherapy-induced

  3. Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia

    OpenAIRE

    Gustavo Adolfo Lazo-Páez; Oscar Porras

    2010-01-01

    Objetivo: La neutropenia es un motivo relativamente frecuente de referencia al Servicio de Inmunología y Reumatología Pediátrica del Hospital Nacional de Niños; el estudio pretende caracterizar los casos de neutropenia referidos a este Servicio en el periodo comprendido entre noviembre de 1988 y junio de 2008. Métodos: Se estudiaron 84 pacientes entre 0 y 12 años de edad, referidos entre el 6 de noviembre de 1988 y el 1 de junio de 2008. Se efectuó un análisis descriptivo global de las caract...

  4. Correlations between event-related potentials with pictures recognition and WMS-RC scores in patients with memory disorder caused by severe traumatic brain injury.

    Science.gov (United States)

    Liu, Zilong; Liu, Liang; Fan, Zebing; Chen, Xiaorui; Zhao, Xiaohong; Zhang, Lingli; Rao, Guangxun; Li, Haixia

    2008-12-01

    This study explored the possibility of using event-related potentials (ERP) for the measurement of picture-recognition memory and examined its correlation with the Chinese Wechsler Memory Scale-revised (WMS-RC) in patients with memory disorder caused by severe traumatic brain injury (sTBI). The subjects included 20 sTBI patients with memory disorder and 22 healthy individuals. Memory function was measured by using WMS-RC. Behavioral and ERP responses were recorded on-line during performance on a battery of picture recognition and the responses were analyzed off-line for recognition memory effects. Mean memory quotient (MQ) of patients with sTBI was significantly lower than that of the control group. Mean reaction time (RT) was significantly longer and the mean correctness rate (CR) of picture recognition was significantly lower in sTBI group than that of the controls. In controls, the main components of average ERP of picture recognition includes two positive-going waves, designated as P(170) and P(500), that appear 170 ms and 500 ms after stimulation when the subject could later successfully recall and recognize the pictures. P(500) amplitude of target stimulus was significantly higher than that of non-target stimulus. Compared to controls, P(500) responses of sTBI group were significantly delayed in latency (PWMS-RC. ERP of picture recognition provides a neurophysiological approach to directly assess memory impairment, and P(500) may serve as a helpful index for memory disorder caused by sTBI in forensic practice.

  5. Correlations between Event-related Potentials with Pictures Recognition and WMS-RC Scores in Patients with Memory Disorder Caused by Severe Traumatic Brain Injury

    Institute of Scientific and Technical Information of China (English)

    Zilong LIU; Liang LIU; Zebing FAN; Xiaorui CHEN; Xiaohong ZHAO; Lingli ZHANG; Guangxun Rao; Haixia LI

    2008-01-01

    This study explored the possibility of using event-related potentials (ERP) for the measurement of picture-recognition memory and examined its correlation with the Chinese Wechsler Memory Scale-revised (WMS-RC) in patients with memory disorder caused by severe traumatic brain injury (sTBI). The subjects included 20 sTBI patients with memory disorder and 22 healthy individuals. Memory function was measured by using WMS-RC. Behavioral and ERP responses were recorded on-line during performance on a battery of picture recognition and the responses were analyzed off-line for recognition memory effects. Mean memory quotient (MQ) of patients with sTBI was significantly lower than that of the control group. Mean reaction time (RT) was significantly longer and the mean correctness rate (CR) of picture recognition was significantly lower in sTBI group than that of the controls. In controls, the main components of average ERP of picture recognition includes two positive-going waves, designated as P170 and P500, that appear 170 ms and 500 ms after stimulation when the subject could later successfully recall and recognize the pictures. P500 amplitude of target stimulus was significantly higher than that of non-target stimulus. Compared to controls, P500 responses of sTBI group were significantly delayed in latency (P<0.001) and lower in amplitude (P<0.001). P500 latency showed significant negative correlation with MQ and the scores of "addition", "visual recognition", "picture recall", "visual reproduction" and "tactile memory" in WMS-RC. ERP of picture recognition provides a neurophysiological approach to directly assess memory impairment, and P500 may serve as a helpful index for memory disorder caused by sTBI in forensic practice.

  6. Ocorrência de neutropenia em mulheres com câncer de mama durante tratamento quimioterápico Ocurrencia de neutropenia en mujeres con cáncer de mama durante el tratamiento quimioterápico Occurrence of neutropenia in women with breast cancer during chemotherapy treatment

    Directory of Open Access Journals (Sweden)

    Thaís de Oliveira Gozzo

    2011-01-01

    Full Text Available OBJETIVO: Analisar a ocorrência de neutropenia induzida por drogas utilizadas no tratamento quimioterápico de mulheres com câncer de mama. MÉTODOS: Estudo retrospectivo, com avaliação de 72 prontuários, durante 2003-2006. RESULTADOS: Dos 558 ciclos de quimioterapia realizados, foram registrados 152 eventos adversos nos períodos de neoadjuvância e adjuvância, totalizando 43 casos por toxicidade hematológica. Quanto à ocorrência de neutropenia, 43% apresentaram, pelo menos, um episódio durante o tratamento. Testes de hipótese para comparar as médias dos valores de glóbulos brancos entre as mulheres que apresentaram ou não neutropenia apontaram para valores estatisticamente significantes, nos ciclos dois e três da neoadjuvância e nos ciclos dois, três e quatro da adjuvância. CONCLUSÃO: A neutropenia, tanto na neoadjuvância como na adjuvância ocorreu a partir do segundo ciclo e manteve-se durante o tratamento e foi estatisticamente significante quando foram compadas as mulheres que tiveram ou não esta ocorrência.OBJETIVO: Analizar la ocurrencia de neutropenia inducida por drogas utilizadas en el tratamiento quimioterápico de mujeres con cáncer de mama. MÉTODOS: Estudio retrospectivo, realizado con la evaluación de 72 historias clínicas, durante loa años 2003-2006. RESULTADOS: De los 558 ciclos de quimioterapia realizados, fueron registrados 152 eventos adversos en los períodos de neoadyuvante y adyuvante, totalizando 43 casos por toxicidad hematológica. En cuanto a la ocurrencia de neutropenia, el 43% presentaron, por lo menos, un episodio durante el tratamiento. Las pruebas de hipótesis para comparar los promedios de los valores de glóbulos blancos entre las mujeres que presentaron o no neutropenia apuntaron hacia valores estadísticamente significativos, en los ciclos dos y tres de la neoadyuvancia y en los ciclos dos, tres y cuatro de la adyuvancia. CONCLUSIÓN: La neutropenia, tanto en la neoadyuvancia como

  7. A novel splice variant in the N-propeptide of COL5A1 causes an EDS phenotype with severe kyphoscoliosis and eye involvement.

    Directory of Open Access Journals (Sweden)

    Sofie Symoens

    Full Text Available BACKGROUND: The Ehlers-Danlos Syndrome (EDS is a heritable connective tissue disorder characterized by hyperextensible skin, joint hypermobility and soft tissue fragility. The classic subtype of EDS is caused by mutations in one of the type V collagen genes (COL5A1 and COL5A2. Most mutations affect the type V collagen helical domain and lead to a diminished or structurally abnormal type V collagen protein. Remarkably, only two mutations were reported to affect the extended, highly conserved N-propeptide domain, which plays an important role in the regulation of the heterotypic collagen fibril diameter. We identified a novel COL5A1 N-propeptide mutation, resulting in an unusual but severe classic EDS phenotype and a remarkable splicing outcome. METHODOLOGY/PRINCIPAL FINDINGS: We identified a novel COL5A1 N-propeptide acceptor-splice site mutation (IVS6-2A>G, NM_000093.3_c.925-2A>G in a patient with cutaneous features of EDS, severe progressive scoliosis and eye involvement. Two mutant transcripts were identified, one with an exon 7 skip and one in which exon 7 and the upstream exon 6 are deleted. Both transcripts are expressed and secreted into the extracellular matrix, where they can participate in and perturb collagen fibrillogenesis, as illustrated by the presence of dermal collagen cauliflowers. Determination of the order of intron removal and computational analysis showed that simultaneous skipping of exons 6 and 7 is due to the combined effect of delayed splicing of intron 7, altered pre-mRNA secondary structure, low splice site strength and possibly disturbed binding of splicing factors. CONCLUSIONS/SIGNIFICANCE: We report a novel COL5A1 N-propeptide acceptor-splice site mutation in intron 6, which not only affects splicing of the adjacent exon 7, but also causes a splicing error of the upstream exon 6. Our findings add further insights into the COL5A1 splicing order and show for the first time that a single COL5A1 acceptor-splice site

  8. Aerosol exposure to Rift Valley fever virus causes earlier and more severe neuropathology in the murine model, which has important implications for therapeutic development.

    Directory of Open Access Journals (Sweden)

    Christopher Reed

    Full Text Available Rift Valley fever virus (RVFV is an important mosquito-borne veterinary and human pathogen that can cause severe disease including acute-onset hepatitis, delayed-onset encephalitis, retinitis and blindness, or a hemorrhagic syndrome. Currently, no licensed vaccine or therapeutics exist to treat this potentially deadly disease. Detailed studies describing the pathogenesis of RVFV following aerosol exposure have not been completed and candidate therapeutics have not been evaluated following an aerosol exposure. These studies are important because while mosquito transmission is the primary means for human infection, it can also be transmitted by aerosol or through mucosal contact. Therefore, we directly compared the pathogenesis of RVFV following aerosol exposure to a subcutaneous (SC exposure in the murine model by analyzing survival, clinical observations, blood chemistry, hematology, immunohistochemistry, and virus titration of tissues. Additionally, we evaluated the effectiveness of the nucleoside analog ribavirin administered prophylactically to treat mice exposed by aerosol and SC. The route of exposure did not significantly affect the survival, chemistry or hematology results of the mice. Acute hepatitis occurred despite the route of exposure. However, the development of neuropathology occurred much earlier and was more severe in mice exposed by aerosol compared to SC exposed mice. Mice treated with ribavirin and exposed SC were partially protected, whereas treated mice exposed by aerosol were not protected. Early and aggressive viral invasion of brain tissues following aerosol exposure likely played an important role in ribavirin's failure to prevent mortality among these animals. Our results highlight the need for more candidate antivirals to treat RVFV infection, especially in the case of a potential aerosol exposure. Additionally, our study provides an account of the key pathogenetic differences in RVF disease following two potential

  9. Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism

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    Milger K

    2015-05-01

    Full Text Available Katrin Milger,1 Lesca Miriam Holdt,2 Daniel Teupser,2 Rudolf Maria Huber,1 Jürgen Behr,1 Nikolaus Kneidinger1 1Department of Internal Medicine V, University of Munich, Comprehensive Pneumology Center, Member of the German Center for Lung Research, 2Institute of Laboratory Medicine, University of Munich, Munich, Germany Abstract: Deficiency in the serine protease inhibitor, alpha-1 antitrypsin (AAT, is known to cause emphysema and liver disease. Other manifestations, including airway disease or skin disorders, have also been described. A 44-year-old woman presented to our emergency department with dyspnea and respiratory insufficiency. She had never smoked, and had been diagnosed with COPD 9 years earlier. Three months previously, she had suffered a pulmonary embolism. Chest computed tomography scan revealed severe cystic bronchiectasis with destruction of the lung parenchyma. The sweat test was normal and there was no evidence of the cystic fibrosis transmembrane conductance regulator (CFTR mutation. Capillary zone electrophoresis showed a decrease of alpha-1 globin band and AAT levels were below the quantification limit (<25 mg/dL. No S or Z mutation was identified, but sequencing analysis found a homozygous cytosine and adenine (CA insertion in exon 2 of the SERPINA-1 gene, probably leading to a dysfunctional protein (PI Null/Null. This mutation has not been previously identified. The atypical presentation of the patient, with severe cystic bronchiectasis, highlights AAT deficiency as a differential diagnosis in bronchiectasis. Further, awareness should be raised regarding a possible increased risk of thromboembolism associated with AAT deficiency. Keywords: alpha-1 antitrypsin deficiency, bronchiectasis, SERPINA-1 mutation, pulmonary embolism

  10. Is preemptive antifungal therapy a good alternative to empirical treatment in prolonged febrile neutropenia?

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    Erica Koch

    2016-06-01

    Full Text Available La neutropenia febril prolongada conlleva un alto riesgo de desarrollar infecciones fúngicas invasoras, por lo que habitualmente se administra terapia antifúngica empírica en estos casos. Sin embargo, esta se asocia a importantes efectos adversos, por lo que se ha propuesto como alternativa la estrategia "preemptive" o anticipada, es decir, la indicación de antifúngicos sólo ante la evidencia indirecta de infección fúngica invasora. Utilizando la base de datos Epistemonikos, la cual es mantenida mediante búsquedas en 30 bases de datos, identificamos tres revisiones sistemáticas que en conjunto incluyen doce estudios. Cuatro estudios aleatorizados evaluaron la pregunta abordada en este artículo. Realizamos un metanálisis y tablas de resumen de los resultados utilizando el método GRADE. Concluimos que no está claro si la estrategia "preemptive" tiene algún efecto sobre la mortalidad porque la certeza de la evidencia es muy baja, pero podría disminuir levemente el uso de antifúngicos en pacientes con neutropenia febril prolongada.

  11. Is the addition of aminoglycosides to beta-lactams in cancer patients with febrile neutropenia needed?

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    Valeria Contreras

    2016-03-01

    Full Text Available En pacientes con cáncer que se presentan con neutropenia febril existe controversia sobre si es mejor utilizar una combinación de antibióticos betalactámicos y aminoglicósidos o si bastaría la monoterapia con betalactámicos de amplio espectro como tratamiento empírico inicial. Utilizando la base de datos Epistemonikos, la cual es mantenida mediante búsquedas en 30 bases de datos, identificamos tres revisiones sistemáticas que en conjunto incluyen 14 estudios aleatorizados pertinentes a esta pregunta. Realizamos un metanálisis y tablas de resumen de los resultados utilizando el método GRADE. Concluimos que adicionar aminoglicósidos a los betalactámicos en el tratamiento de la neutropenia febril en pacientes con cáncer aumenta la nefrotoxicidad y podría aumentar la mortalidad en comparación con la monoterapia con betalactámicos.

  12. Prophylaxis against febrile neutropenia with pegfilgrastim in Italy: a budget impact analysis

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    Giovanni Rosti

    2011-09-01

    Full Text Available Introduction: prophylaxis with granulocyte colony-stimulating factors (G-CSF is indicated for reduction in the duration of neutropenia and the incidence of febrile neutropenia in patients treated with cytotoxic chemotherapy for malignancy.
Objective: to evaluate the budgetary impact for the Italian NHS.
Design: a decision-analytic model has been developed to analyze the budget impact from the national health care system perspective. Costs include direct healthcare costs to the public payer of G-CSFs as well as their administration costs and costs of FN-related events. The comparison has been done using prophylaxis with G‑CSF (filgrastim for 11 days, pegfilgrastim, lenograstim for 11 days and antibiotics.
Patients and participants: The population of interest for the analysis were patients with breast cancer in stage II and III and patients with non-Hodgkin’s lymphoma (NHL.
Main outcome measures and results: for all the three patients group (NHL, Breast II and III, and for all the chemotherapy regimens (CHOP 21 and R-CHOP 21 for NHL, AC-T, TAC and TC for Breast stage II and III the budget impact analyses shows a cost reduction for the Italian NHS, as a result of an increase of the use of pegfilgrastim.
Conclusions: in Italy, a treatment strategy including pegfilgrastim as either primary or secondary prophylaxis provides value for money.


  13. A series of abnormal climatic conditions caused the most severe outbreak of first-generation adults of the meadow moth ( Loxostege sticticalis L.) in China

    Science.gov (United States)

    Chen, Xiao; Zeng, Juan; Zhai, Baoping

    2016-06-01

    The meadow moth, Loxostege sticticalis L., is a destructive migratory pest in the northern temperate zone. The outbreak mechanism of first-generation adults in China remains unclear. In 2008, the density of first-generation larvae was very low or even negligible in most sites in China. However, a great number of first-generation adults appeared unexpectedly in late July, and their offspring caused the most severe infestation on record. The present study aims to determine where the large influx of immigrant adults originated from and how this unprecedented population was established. Source areas were explored by trajectory analysis, and climatic patterns related to the population increase were investigated. Results showed that the outbreak population mainly immigrated from Northeast Mongolia and the Chita State of Russia, and the buildup of such a large population could be attributed to an exceptional northward migration of overwintered adults from North China to East Mongolia in the spring of 2007 and unusually favourable climatic conditions in the next two growth seasons. These results indicated that the population dynamics of meadow moth in Northeast Asia would be difficult to predict when only considering local climatic factors and population size within one country. International joint monitoring and information sharing related to this pest between China, Mongolia and Russia should be implemented.

  14. Toxic epidermal necrolysis caused by acetaminophen featuring almost 100% skin detachment: Acetaminophen is associated with a risk of severe cutaneous adverse reactions.

    Science.gov (United States)

    Watanabe, Hideaki; Kamiyama, Taisuke; Sasaki, Shun; Kobayashi, Kae; Fukuda, Kenichiro; Miyake, Yasufumi; Aruga, Tohru; Sueki, Hirohiko

    2016-03-01

    Toxic epidermal necrolysis (TEN) is an adverse reaction that can be induced by various drugs; the associated mortality rate is 20-25%. A previous report showed a weak association between TEN and acetaminophen. Recently, the US Food and Drug Administration declared that acetaminophen is associated with a risk of serious skin reactions, including TEN. Here, we describe the case of a 43-year-old Japanese woman with TEN caused by acetaminophen. She had poorly controlled ulcerative colitis and was treated with high doses of prednisolone, infliximab, acetaminophen and lansoprazole. Nine days after administrating acetaminophen, targetoid erythematous and bullous lesions appeared on the patient's trunk, palms and the soles of her feet. The skin lesions expanded rapidly; within 3 weeks, skin detachment was detected across nearly 100% of the patient's body. However, no mucosal involvement of the eyes, oral cavity or genitalia was found. We performed lymphocyte transformation tests using various drugs; however, a high stimulation index was obtained only with acetaminophen. The patient recovered following treatment with plasmapheresis, i.v. immunoglobulin therapy, topical medication and supportive therapy. Acetaminophen is included in many prescription and over-the-counter products; thus, clinicians should monitor their patients for severe drug reactions, including TEN. PMID:26362011

  15. Analisi Costo-Efficacia di Amfotericina B Liposomiale (L-AmB versus Amfotericina B Complesso Lipidico (ABLC nel trattamento empirico della neutropenia febbrile

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    Mario Eandi

    2005-12-01

    Full Text Available Current international guidelines for the management of immuno-compromised patients with febrile neutropenia recommend a systemic antimicrobial therapy if fever hasn’t receded after three days of antibiotic treatment. Amphotericin B remains the gold standard because of its broad spectrum fungicidal action and minimal resistance development risk. Nonetheless, therapeutic use of the standard formulation, Amphotericin B deoxycholate, is limited by its toxicity, especially on the kidneys. To counteract this, amphotericin B has been encapsulated in liposomes, a process which reduces its toxicity and allows higher doses to be given. Three lipid formulations have been developed and are now available in most countries: amB colloidal dispersion (ABCD, amB lipid complex (ABLC, and liposomal amB (L-AmB. These lipid formulations differ in pharmacodynamics and pharmacokinetics, and can’t therefore be considered interchangeable. Besides, they are more expensive than Amphotericin B deoxycholate. Aim of the study is to perform a cost/effectiveness analysis (CEA comparing L-AmB (3mg/kg/die or 5mg/kg/ die and ABLC (5mg/kg/die as first-line antimicrobial empirical treatments in immuno-compromised patients with febrile neutropenia resistant to broad spectrum antibiotics. Secondly, we present a cost-minimization analysis (CMA of the considered alternatives, assuming the same efficacy for all treatments. At the end we value the principal cost items from the point of view of the Italian Health Service, with a particular focus on the economic burden caused by adverse reactions.

  16. Failure of filgrastim to prevent severe clozapine-induced agranulocytosis.

    Science.gov (United States)

    Majczenko, Tricia G; Stewart, Jonathan T

    2008-06-01

    Although a highly effective medication, the usage of clozapine is limited mostly by its 2.7% incidence of neutropenia. It is often a treatment of last resort for patients with severe psychiatric illnesses, and therefore often the only medication to which a patient has responded. There has thus been a great deal of interest in ways to continue the medication in spite of emergent blood dyscrasias. There have been several reports documenting the successful continuation of clozapine in spite of neutropenia by adding granulocyte colony-stimulating factors such as filgrastim. This strategy was unsuccessful for a 63-year-old man, resulting in severe, prolonged agranulocytosis. Although a promising strategy for such refractory patients, its inherent dangers should not be underestimated. PMID:18475227

  17. A clinical analysis of 361 cases of Graves disease combined with neutropenia or agranulocytosis%Graves 病合并粒细胞减少或缺乏361例临床分析

    Institute of Scientific and Technical Information of China (English)

    闫文博; 秦贵军

    2016-01-01

    Objective To explore the prevalence and the correlation factors of neutropenia or agranulocytosis in patients with Graves disease.Methods The clinical data of 361 patients diagnosed as Graves disease in General Hospital of Pingmei Shenma Medical Group from February of 201 0 to February of 201 5 were analyzed retrospectively.Results Among 361 patients,there were altogether 1 27 patients with neutropenia or agranulocytosis(35.1 8%),1 23 with neutropenia(34.07%),4 with agranulocy-tosis(1 .1 1 %).Among the 1 27 cases,72 (56.69%)were caused by Graves disease,and 55 were caused by antithyroid drugs (ATD)(43.31 %).Among neutropenia or agranulocytosis caused by Graves disease itself,the neutrophil count were negatively correlated with age,free triiodothyronine (FT3 ),free thyroxine(FT4 )and TSH receptor stimulation antibody(TSAb).Conclu-sion The incidence of neutropenia was relatively high in patients with Graves disease,and the neutropenia induced by Graves disease itself may be related with the degree of hyperthyroidism.%目的:探讨 Graves 病患者合并粒细胞减少或缺乏的发生率及相关因素。方法对平煤神马医疗集团总医院2010年2月至2015年2月收治的361例 Graves 患者的临床资料进行回顾性分析。结果361例 Graves 患者中,粒细胞减少或缺乏127例(35.18%):粒细胞减少123例(34.07%),粒细胞缺乏4例(1.11%)。其中 Graves 疾病本身导致者72例(56.69%),抗甲状腺药物(ATD)导致者55例(43.31%)。在 Graves 疾病本身所致的粒细胞减少或缺乏中,粒细胞计数与年龄、FT3、FT4、TSH 受体刺激性抗体(TSAb)呈负相关。结论Graves 病患者合并粒细胞减少的发生率较高,其疾病本身所导致的粒细胞减少可能与甲亢的病情程度相关。

  18. Evaluation of ior ® Leukocim efficacy on patients with Neutropenia. Evaluación de la eficacia del ior® Leukocim en pacientes con neutropenia.

    Directory of Open Access Journals (Sweden)

    Hailen Bobillo López

    2007-02-01

    Full Text Available

    Bakground: Neutropenia and infections are the most restrictive side effects in chemotherapy application. The granulocytic colonies stimulating factor activates the neutrophils, shortens the neutropenic period and can be effective against the potential risk of infection. There is a need of studies for its commercialization to endorse their effectiveness and security. Objective: To evaluate the impact of the G-CSF in primary and secondary prevention and neutropenia episodes, in onco-haematological patients in Cienfuegos. Method: 95 neutropenic episodes were studied, picked up in clinical histories and data collection notebooks belonging to 47 patients treated in the University Hospital ¨Dr. Gustavo Aldereguía Lima¨ in Cienfuegos and happened during one year (2005-2006. It was analyzed: demographic data, absolute neutrophils count values, number of administered doses, treatment regime and possible treatment interruptions Results: 50, 5% received treatment in an ambulatory way. There was treatment interruption in 9 episodes, 9,5% and the mean dose number administered to obtain the recovery of neutrophils absolute count was 6,69. Conclusions: The product was effective, sine the decrease of neutropenic episodes could be verified, as the elevation of neutrophil values in approximately one week, what allowed a better pursuit of chemotherapy to patients of the studied series.

    Abstract

    To demonstrate the effectiveness of Leukocim an open, phase IV clinical trial was designed, 39 patients from Cienfuegos were evaluated with 73 neutropenic episodes in primary or secondary prophylaxis or treatment, the demographic parameters behaved with an age 49.86 year old average, the predominant sex was the feminine 64.38%, 73.97% belonged to the white race, when the effectiveness was analyzed we obtained that 46.5% received the treatment in an

  19. Severe Psychological Distress of Evacuees in Evacuation Zone Caused by the Fukushima Daiichi Nuclear Power Plant Accident: The Fukushima Health Management Survey.

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    Yasuto Kunii

    Full Text Available Following the Great East Japan Earthquake on March 11, 2011, the nuclear disaster at the Fukushima Daiichi Nuclear Power Plant has continued to affect the mental health status of residents in the evacuation zone. To examine the mental health status of evacuee after the nuclear accident, we conducted the Mental Health and Lifestyle Survey as part of the ongoing Fukushima Health Management Survey.We measured mental health status using the Kessler 6-item psychological distress scale (K6 in a total of 73,569 (response rate: 40.7% evacuees aged 15 and over who lived in the evacuation zone in Fukushima Prefecture. We then dichotomized responders using a 12/13 cutoff on the K6, and compared the proportion of K6 scores ≥13 and ≤12 in each risk factor including demographic information, socioeconomic variables, and disaster-related variables. We also performed bivariate analyses between mental health status and possible risk factors using the chi-square test. Furthermore, we performed multivariate regression analysis using modified Poisson regression models.The median K6 score was 5 (interquartile range: 1-10. The number of psychological distress was 8,717 (14.6%. We found that significant differences in the prevalence of psychological distress by almost all survey items, including disaster-related risk factors, most of which were also associated with increased Prevalence ratios (PRs. Additionally, we found that psychological distress in each evacuation zone was significantly positively associated with the radiation levels in their environment (r = 0.768, p = 0.002.The earthquake, tsunami and subsequent nuclear accident likely caused severe psychological distress among residents in the evacuation zone in Fukushima Prefecture. The close association between psychological distress and the radiation levels shows that the nuclear accident seriously influenced the mental health of the residents, which might be exacerbated by increased risk perception. To

  20. Effect of Elevated Atmospheric CO2 and Temperature on the Disease Severity of Rocket Plants Caused by Fusarium Wilt under Phytotron Conditions.

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    Walter Chitarra

    Full Text Available The severity of F. oxysporum f.sp. conglutinans on rocket plants grown under simulated climate change conditions has been studied. The rocket plants were cultivated on an infested substrate (4 log CFU g-1 and a non-infested substrate over three cycles. Pots were placed in six phytotrons in order to simulate different environmental conditions: 1 400-450 ppm CO2, 18-22°C; 2 800-850 ppm CO2, 18-22°C; 3 400-450 ppm CO2, 22-26°C, 4 800-850 ppm CO2, 22-26°C, 5 400-450 ppm CO2, 26-30°C; 6 800-850 ppm CO2, 26-30°C. Substrates from the infested and control samples were collected from each phytotron at 0, 60 and 120 days after transplanting. The disease index, microbial abundance, leaf physiological performances, root exudates and variability in the fungal profiles were monitored. The disease index was found to be significantly influenced by higher levels of temperature and CO2. Plate counts showed that fungal and bacterial development was not affected by the different CO2 and temperature levels, but a significant decreasing trend was observed from 0 up to 120 days. Conversely, the F. oxysporum f.sp. conglutinans plate counts did not show any significantly decrease from 0 up to 120 days. The fungal profiles, evaluated by means of polymerase chain reaction denaturing gradient gel electrophoresis (PCR-DGGE, showed a relationship to temperature and CO2 on fungal diversity profiles. Different exudation patterns were observed when the controls and infested plants were compared, and it was found that both CO2 and temperature can influence the release of compounds from the roots of rocket plants. In short, the results show that global climate changes could influence disease incidence, probably through plant-mediated effects, caused by soilborne pathogens.

  1. Effect of Elevated Atmospheric CO2 and Temperature on the Disease Severity of Rocket Plants Caused by Fusarium Wilt under Phytotron Conditions.

    Science.gov (United States)

    Chitarra, Walter; Siciliano, Ilenia; Ferrocino, Ilario; Gullino, Maria Lodovica; Garibaldi, Angelo

    2015-01-01

    The severity of F. oxysporum f.sp. conglutinans on rocket plants grown under simulated climate change conditions has been studied. The rocket plants were cultivated on an infested substrate (4 log CFU g-1) and a non-infested substrate over three cycles. Pots were placed in six phytotrons in order to simulate different environmental conditions: 1) 400-450 ppm CO2, 18-22°C; 2) 800-850 ppm CO2, 18-22°C; 3) 400-450 ppm CO2, 22-26°C, 4) 800-850 ppm CO2, 22-26°C, 5) 400-450 ppm CO2, 26-30°C; 6) 800-850 ppm CO2, 26-30°C. Substrates from the infested and control samples were collected from each phytotron at 0, 60 and 120 days after transplanting. The disease index, microbial abundance, leaf physiological performances, root exudates and variability in the fungal profiles were monitored. The disease index was found to be significantly influenced by higher levels of temperature and CO2. Plate counts showed that fungal and bacterial development was not affected by the different CO2 and temperature levels, but a significant decreasing trend was observed from 0 up to 120 days. Conversely, the F. oxysporum f.sp. conglutinans plate counts did not show any significantly decrease from 0 up to 120 days. The fungal profiles, evaluated by means of polymerase chain reaction denaturing gradient gel electrophoresis (PCR-DGGE), showed a relationship to temperature and CO2 on fungal diversity profiles. Different exudation patterns were observed when the controls and infested plants were compared, and it was found that both CO2 and temperature can influence the release of compounds from the roots of rocket plants. In short, the results show that global climate changes could influence disease incidence, probably through plant-mediated effects, caused by soilborne pathogens.

  2. Overexpression of AtDREB1A causes a severe dwarf phenotype by decreasing endogenous gibberellin levels in soybean [Glycine max (L. Merr].

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    Haicui Suo

    Full Text Available Gibberellic acids (GAs are plant hormones that play fundamental roles in plant growth and developmental processes. Previous studies have demonstrated that three key enzymes of GA20ox, GA3ox, and GA2ox are involved in GA biosynthesis. In this study, the Arabidopsis DREB1A gene driven by the CaMV 35S promoter was introduced into soybean plants by Agrobacterium- mediated transformation. The results showed that the transgenic soybean plants exhibited a typical phenotype of GA-deficient mutants, such as severe dwarfism, small and dark-green leaves, and late flowering compared to those of the non-transgenic plants. The dwarfism phenotype was rescued by the application of exogenous GA(3 once a week for three weeks with the concentrations of 144 µM or three times in one week with the concentrations of 60 µM. Quantitative RT-PCR analysis revealed that the transcription levels of the GA synthase genes were higher in the transgenic soybean plants than those in controls, whereas GA-deactivated genes except GmGA2ox4 showed lower levels of expression. The transcript level of GmGA2ox4 encoding the only deactivation enzyme using C(20-GAs as the substrates in soybean was dramatically enhanced in transgenic plants compared to that of wide type. Furthermore, the contents of endogenous bioactive GAs were significantly decreased in transgenic plants than those of wide type. The results suggested that AtDREB1A could cause dwarfism mediated by GA biosynthesis pathway in soybean.

  3. Mannose-Binding Lectin (MBL) and the Risk for Febrile Neutropenia and Infection in Pediatric Oncology Patients With Chemotherapy

    NARCIS (Netherlands)

    F.N.J. Frakking; J. Israëls; L.C.M. Kremer; T.W. Kuijpers; H.N. Caron; M.D. van de Wetering

    2011-01-01

    Background. We determined whether mannose-binding lectin (MBL) deficiency is associated with an increased risk of febrile neutropenia (FN) and/or infection in pediatric oncology patients. Procedure. We systematically searched and reviewed all the literature on MBL and infections in children with can

  4. Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia

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    Gustavo Adolfo Lazo-Páez

    2010-06-01

    Full Text Available Objetivo: La neutropenia es un motivo relativamente frecuente de referencia al Servicio de Inmunología y Reumatología Pediátrica del Hospital Nacional de Niños; el estudio pretende caracterizar los casos de neutropenia referidos a este Servicio en el periodo comprendido entre noviembre de 1988 y junio de 2008. Métodos: Se estudiaron 84 pacientes entre 0 y 12 años de edad, referidos entre el 6 de noviembre de 1988 y el 1 de junio de 2008. Se efectuó un análisis descriptivo global de las características presentadas por estos pacientes en términos de evolución clínica, patrón de infección, gérmenes más frecuentes causantes de infección, complicaciones y tratamiento. Resultados: El 52.2% de los pacientes analizados resolvieron su neutropenia espontáneamente, por lo que fueron catalogados como neutropenia transitoria; el 21.7% de los casos evolucionó como neutropenia cíclica; el 13% de los pacientes fueron catalogados como neutropenia crónica benigna; el 7.2% evolucionaron como neutropenia crónica grave sintomática; el 2.9% tuvieron neutropenia asociada a glucogenosis tipo 1B, y el 2.9% de los casos no fueron clasificables en las categorías propuestas. El 56.5% de los casos se asoció a un patrón de infección anormal, sea por incremento en la frecuencia, mayor gravedad, compromiso multisistémico o presencia de microorganismos oportunistas. El sistema más afectado por infección fue la vía respiratoria superior. El 39.1% de los casos de neutropenia evaluados ameritaron uso de antibióticos profilácticos, y el 11.6% de los casos requirieron usar factor estimulante de colonias granulocíticas, en algún momento de su evolución. Los gérmenes más frecuentemente involucrados en infección fueron Pseudomona aeruginosa, Staphylococcus spp y E. coli. Conclusión: La gran mayoría de los pacientes neutropénicos estudiados tiene un curso clínico benigno caracterizado por pocas hospitalizaciones y una intervención farmacol

  5. Chronic Refractory Uveitis in a Patient with Childhood-Onset Cyclic Neutropenia

    Directory of Open Access Journals (Sweden)

    Li-Li Chen

    2011-05-01

    Full Text Available We report a rare case of chronic refractory uveitis in a patient with childhood-onset cyclic neutropenia (CN. A 19-year-old woman, who had a history of CN beginning at age 2, presented with bilateral chronic nongranulomatous uveitis, complicated cataract, retinal vasculitis, cystoids macular edema, and vitreous hemorrhage. She had recurrent episodes of oral ulcers, tonsillitis, genital ulcers, and folliculitis during neutropenic nadir. After the resumption of granulocyte colony-stimulating factor therapy for her CN, vitreous hemorrhage in both eyes followed. Her eyes were treated with topical corticosteroids, retinal photocoagulation, and cataract surgery. Blood and bone marrow test results confirmed the diagnosis of CN. She also fulfilled the diagnostic criteria of Behçet’s disease, though clinical features of her uveitis were dissimilar to those found in that disease.

  6. Voriconazole versus amphotericin B or fluconazole in cancer patients with neutropenia

    DEFF Research Database (Denmark)

    Jørgensen, Karsten Juhl; Gøtzsche, Peter C; Dalbøge, Christina S;

    2014-01-01

    and fluconazole when used for prevention or treatment of invasive fungal infections in cancer patients with neutropenia. SEARCH METHODS: Cochrane Central Register of Controlled Trials (CENTRAL) in The Cochrane Library (2014, Issue 1 2014), MEDLINE (to January 2014). Letters, abstracts and unpublished trials were...... accepted. Contact was made with trial authors and industry. SELECTION CRITERIA: Randomised clinical trials comparing voriconazole with amphotericin B or fluconazole. DATA COLLECTION AND ANALYSIS: Data on mortality, invasive fungal infection, colonisation, use of additional (escape) antifungal therapy...... cancer patients (849 patients, 58 deaths). The second trial compared voriconazole to amphotericin B deoxycholate in the treatment of confirmed and presumed invasive Aspergillus infections (391 patients, 98 deaths). The third trial compared fluconazole to voriconazole for prophylaxis of fungal infections...

  7. Prolonged or Standard Infusion of Cefepime Hydrochloride in Treating Patients With Febrile Neutropenia

    Science.gov (United States)

    2013-07-10

    Adult Acute Lymphoblastic Leukemia; Adult Acute Myeloid Leukemia; Adult Burkitt Lymphoma; Adult Diffuse Large Cell Lymphoma; Adult Diffuse Mixed Cell Lymphoma; Adult Diffuse Small Cleaved Cell Lymphoma; Adult Hodgkin Lymphoma; Adult Immunoblastic Large Cell Lymphoma; Adult Lymphoblastic Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Breast Cancer; Chronic Eosinophilic Leukemia; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; Cutaneous T-cell Non-Hodgkin Lymphoma; Disseminated Neuroblastoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3 Follicular Lymphoma; Malignant Testicular Germ Cell Tumor; Mantle Cell Lymphoma; Marginal Zone Lymphoma; Multiple Myeloma; Mycosis Fungoides/Sezary Syndrome; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neutropenia; Nodal Marginal Zone B-cell Lymphoma; Ovarian Epithelial Cancer; Ovarian Germ Cell Tumor; Plasma Cell Neoplasm; Poor Prognosis Metastatic Gestational Trophoblastic Tumor; Primary Myelofibrosis; Prolymphocytic Leukemia; Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

  8. Economic burden of chemotherapy-induced febrile neutropenia in patients with lymphoma: a systematic review.

    Science.gov (United States)

    Wang, Xiao Jun; Lopez, Shaun Eric; Chan, Alexandre

    2015-05-01

    The primary objective of this review was to identify the cost components that were most frequently associated with the economic burden of febrile neutropenia (FN) among patients with lymphoma. The secondary objective was to identify any parameter associated with higher FN cost. Ten cost of illness (COI) studies were identified. General characteristics on study design, country, perspective, and patient population were extracted and systematically reported. It was observed that majority (70%) of the studies employed the perspective of healthcare provider. 20% of the studies considered long-term costs. Estimated costs were adjusted to 2013 US dollars and ranged from US$5819 to US$34,756. The cost components that were most frequently associated with economic burden were ward and medication costs. Inpatient management, male gender, discharged dead, and comorbidity were positively associated with higher FN costs. Future COI studies on FN should focus on the accurate estimation on ward and medication costs.

  9. Rituximab-induced neutropenia in a patient with inflammatory myopathy and systemic sclerosis overlap disease.

    Science.gov (United States)

    Akram, Qasim; Roberts, Mark; Oddis, Chester; Herrick, Arianne; Chinoy, Hector

    2016-01-01

    Rituximab (RTX) is a monoclonal chimeric antibody directed against the CD20 antigen of B lymphocytes. Late onset neutropenia (LON) is a recognised complication of rituximab usually occurring 4 weeks after the last dose and is reported in both haematological and rheumatological conditions. However, it has never been described in a patient with myositis and systemic sclerosis overlap disease. We describe a case of LON in a 54-year-old man who was diagnosed with myositis and then systemic sclerosis overlap disease. It resolved within 7 days, and the patient did not suffer neutropenic sepsis or any other complications. We propose similar mechanisms for LON as described in other conditions and routine blood monitoring in such patients.

  10. Rituximab-induced neutropenia in a patient with inflammatory myopathy and systemic sclerosis overlap disease

    Science.gov (United States)

    Roberts, Mark; Oddis, Chester; Herrick, Arianne; Chinoy, Hector

    2016-01-01

    Rituximab (RTX) is a monoclonal chimeric antibody directed against the CD20 antigen of B lymphocytes. Late onset neutropenia (LON) is a recognised complication of rituximab usually occurring 4 weeks after the last dose and is reported in both haematological and rheumatological conditions. However, it has never been described in a patient with myositis and systemic sclerosis overlap disease. We describe a case of LON in a 54-year-old man who was diagnosed with myositis and then systemic sclerosis overlap disease. It resolved within 7 days, and the patient did not suffer neutropenic sepsis or any other complications. We propose similar mechanisms for LON as described in other conditions and routine blood monitoring in such patients. PMID:27407275

  11. Fever and neutropenia hospital discharges in children with cancer: A 2012 update.

    Science.gov (United States)

    Mueller, Emily L; Croop, James; Carroll, Aaron E

    2016-02-01

    Fever and neutropenia (FN) is a common precipitant for hospitalization among children with cancer, but hospital utilization trends are not well described. This study describes national trends for hospital discharges for FN among children with cancer for the year 2012, compared with the authors' previous analysis from 2009. Data were analyzed from the Kids' Inpatient Database (KID), an all-payer US hospital database, for 2012. Pediatric patients with cancer who had a discharge for FN were identified using age ≤19 years, urgent or emergent admit type, nontransferred, and a combination of International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes for fever and neutropenia. The authors evaluated factors associated with a "short length of stay" (SLOS). Sampling weights were used to permit national inferences. In 2012, children with cancer accounted for 1.8% of pediatric hospital discharges (n = 120,675), with 12.2% (n = 13,456) of cancer-related discharges meeting FN criteria. Two fifths of FN discharges had a SLOS, which accounted for $91 million (2015 US$) in hospital charges. The majority had no serious infections; most common infections were viral infection (9.6%) or upper respiratory infection (9.6%). Factors significantly associated with SLOS included having a diagnosis of ear infection (odds ratio [OR] = 1.54, 95% confidence interval [CI]: 1.16-2.03), soft tissue sarcoma (OR = 1.47, CI: 1.10-1.95), and Hodgkin lymphoma (OR = 1.51, CI: 1.09-2.10), as compared with not having those diagnoses. SLOS admissions continue to be rarely associated with serious infections, but contribute substantially to the burden of hospitalization for pediatric FN. Implementation of risk stratification schemas to identify patients who meet low-risk criteria may decrease financial burden. PMID:26900730

  12. Death caused by severe acute pancreatitis:an anlaysis of 144 cases%重症胰腺炎病死原因分析:附144例

    Institute of Scientific and Technical Information of China (English)

    何满西; 李建水; 张肇达; 刘续宝; 田伯乐; 胡伟明; 李耿; 姚有贵; 蒲道深; 陆慧敏

    2010-01-01

    Objective To explore the main causes for death due to severe acute pancreatitis (SAP) to improve the level of diagnosis and treatment. Methods The clinical data of 1162 SAP cases treated in our hospital from June 1997 to May 2005 were retrospectively analyzed. Among which, 144patients (12. 39%) died, 82(7.06%)abandoned treatment and 936(80.55%)were cured. Results the APACHE Ⅱ scores and pancreas Balthazar CT scores of the death group were higher than that of the survival group. The percentage of single one organ dysfunction and multiple organ dysfunction syndrome (MODS) was significantly higher in the death group than in the survival group. The mortality rate of SAP without obvious inducing factors was significantly higher than that of SAP with inducing factors. Conclusion Integrated traditional and western non-surgical treatment is effective for SAP.The treatment for SAP without obvious inducing factors is a challenge. The mortality rate of SAP is primarily related to the pathological changes of pancreas and the patient's general condition. Early diagnosis and treatment is important to decrease mortality rate and maintaining the function of important organs is basic to ensure curative effect.%目的 分析重症急性胰腺炎(severe acute pancreatitis,SAP)的主要病死原因,提高诊治水平.方法 回顾性分析1997年6月至2006年5月收治的1162例SAP病人,其中病死144例(12.39%),放弃治疗82例(7.06%),痊愈936例(80.55%),对病死组与痊愈组的临床资料进行统计学分析.结果 病死组APACHEⅡ评分和胰腺Balthazar CT评分高于存活组;病死组单一器官功能不全、多器官功能障碍综合征(MODS)的百分率与存活组比较有统计学差异;无明显诱因的SAP病死率明显高于有诱因者.结论 中西医结合非手术疗法是治疗SAP可供选择的方法,无明显诱因的SAP是当前治疗的难题,胰腺的病变程度轻重及全身情况好坏是影响SAP病死率的首要原因,早期诊断和治

  13. Xenopus laevis P23H rhodopsin transgene causes rod photoreceptor degeneration that is more severe in the ventral retina and is modulated by light

    OpenAIRE

    Zhang, Rui; Oglesby, Ericka; Marsh-Armstrong, Nicholas

    2008-01-01

    Rhodopsin transgenes carrying mutations that cause autosomal dominant retinitis pigmentosa in humans have been used to study rod photoreceptor degeneration in various model organisms including Xenopus laevis. To date, the only transgenes shown to cause rod photoreceptor degeneration in Xenopus laevis have been either mammalian rhodopsins or chimeric versions of rhodopsin based mainly on Xenopus laevis rhodopsin sequences but with a mammalian C-terminus. Since the C-terminal sequence of rhodop...

  14. 重症烧伤休克期代谢性碱中毒原因分析%Cause analysis of Severe burns shock stage metabolic alkalosis

    Institute of Scientific and Technical Information of China (English)

    邢溢庆; 骆永红; 徐琦; 赵全; 王玉莲

    2009-01-01

    目的 了解重症烧伤休克期代谢性碱中毒(Metabolic alkalosis)的病因、治疗方法以及监测手段等相关因素,以预防其发生.方法 收集13例重症烧伤患者自入院至发生代谢性碱中毒发现时所有临床资料,从治疗措施、液体入量及种类、碱性药物、利尿剂、血管扩张剂的应用情况,以及代谢性碱中毒发现后钾入量和盐酸精氨酸的应用情况等方面,比较二氧化碳结合力与血气分析的临床敏感性.结果 13例患者休克期第1个24 h平均液体总入量达到(3.3 ±0.80)m1.kg-1·1%TBSA.其中乳酸钠林格氏液(5285±2003)ml,血浆为(3450±2498)ml:13例患者中,在伤后第一天发生代谢性碱中毒的6例患者,均于伤后短时间内应用5%碳酸氢钠125 ml~570 ml.13例患者休克期分别应用呋塞米、多巴胺、20%甘露醇、利尿,致第1个24 h尿量平均达到(125±49)ml.经对血气分析中BE、HCO3与血C02-CP同时相、同量标本数监测凝据比较,前两者均高于正常参考值,而后者均在正常参考值内.13例患者经积极处理原发病,适当补钾,静脉输入盐酸精氨酸等临床治疗,所有患者的代谢性碱中毒全部得到纠正.结论 重症烧伤休克期补液量应在有创和无创监测下平稳达到复苏指标,尿量以1 ml·kg-1·h-1为度.患者入院后即行血气分析监测,或可预警代谢性碱中毒的发生.%Objective To investigate cause,therapy and monitoring measures of severe burns with metabolic alkalosis so as to prevent metabolic alkalosis. Methods Therapy measures of 13 severe burns patients were studied from their admission to onset of metabolic alkalosis, including liquid amount and category, usage of alkaline drug, diuretic agent and vasodilator, dosage of potassium and arginine hydrochloride after metabolic alkalosis. The clinical sensitivity of carbon dioxide combining power and blood gas analysis were compared. Results The occurrence of metabolic alkalosis in 13 patients

  15. Neutropenia febril em pacientes com câncer de mama submetidas à quimioterapia: experiência de 12 anos Febrile neutropenia in patients with breast cancer submitted to chemotherapy: a 12 year experience

    Directory of Open Access Journals (Sweden)

    Omero Benedicto Poli Neto

    2004-12-01

    Full Text Available OBJETIVOS: Identificar as características das pacientes com câncer de mama que desenvolveram neutropenia febril, estabelecer fatores de risco para a sua ocorrência e indicadores de evolução desfavorável. MÉTODOS: Realizamos um estudo caso-controle com 65 pacientes. Foram incluídas 13 pacientes que desenvolveram neutropenia febril e quatro controles por caso pareados por data e número de ciclos de quimioterapia prévios, drogas e doses empregadas. Os dados clínicos e laboratoriais foram obtidos dos prontuários médicos. Utilizamos odds ratio (OR e intervalo de confiança (IC de 95% para estimar a significância dos fatores de risco. RESULTADOS: Identificamos dois fatores de risco associados à ocorrência de neutropenia febril: a realização de quimioterapia nas primeiras 24 horas após a cirurgia (OR: 159,9 IC 95%: 9,5 a 2699 e a realização concomitante de quimioterapia e radioterapia da mama (OR: 108,3 IC 95%: 4,9 a 2391. Não observamos diferenças significativas entre casos e controles quanto à idade, índice de massa corporal e contagem de neutrófilos e monócitos antes da quimioterapia. Três pacientes foram a óbito (23,1%. Duas delas tinham idade superior a 60 anos, não apresentavam comorbidades, tinham recebido o primeiro ciclo de CMF nas primeiras 24 horas após a cirurgia e tiveram infecção de sítio cirúrgico. CONCLUSÕES: Os principais fatores de risco associados a neutropenia febril em pacientes com câncer de mama foram quimioterapia nas primeiras 24 horas após a cirurgia, e uso concomitante de quimioterapia e radioterapia da mama. Nosso estudo mostra, portanto, que estas situações devem ser evitadas.PURPOSE: To identify the characteristis of patients with breast cancer who developed febrile neutropenia and to establish risk factors for its incidence and parameters for an unfavorable evolution. PATIENTS AND METHODS: A case-control study was performed and included 65 patients: 13 patients presented febrile

  16. Gene expression analysis of children with acute hematogenous osteomyelitis caused by Methicillin-resistant Staphylococcus aureus: correlation with clinical severity of illness.

    Directory of Open Access Journals (Sweden)

    Claudia Gaviria-Agudelo

    Full Text Available Children with acute hematogenous osteomyelitis (AHO demonstrate a broad spectrum of clinical manifestations, ranging from mild to severe. Several advances have been achieved in the study of host immune response to acute invasive Staphylococcus aureus infections through gene expression analysis. However, previous research has neither attempted to evaluate the response of children with AHO specific to Methicillin-resistant Staphylococcus aureus (MRSA nor to correlate gene expression with clinical phenotype. Study objective was to correlate gene expression of children with AHO due to MRSA with clinical severity of illness. Whole blood samples were obtained in Tempus tubes from 12 children with osteomyelitis once cultures obtained directly from the site of infection confirmed to be positive for MRSA. Using an Illumina platform and a systems-wide modular analysis, microarray findings from ten of these children were compared to that of nine healthy (age, ethnicity and gender matched controls and correlated with clinical severity of illness. Children with AHO from MRSA demonstrated over-expression of innate immunity with respect to neutrophil activity, coagulation, inflammatory response, and erythrocyte development. Concurrently, these children demonstrated under-expression of adaptive immunity with respect to lymphocyte activation and activity of T-cell, cytotoxic or NK cell, and B-cell lines. Three over-expressed genes, P2RX1, SORT1, and RETN, and two under-expressed genes, LOC641788 and STAT 4, were significantly correlated with severity of illness. STAT 4 showed the strongest correlation (R2 = -0.83. STAT4 downregulation could potentially explain under-expression of genes related to adaptive immunity in this cohort of patients with AHO. This study identified specific genes which correspond to disease severity during the early hospitalization of children with AHO from MRSA. Pattern recognition of this combination of genes could help to identify

  17. Effect of phosphate and the arbuscular mycorrhizal fungus Glomus intraradices on disease severity of root rot of peas ( Pisum sativum ) caused by Aphanomyces euteiches

    DEFF Research Database (Denmark)

    Bødker, Lars; Kjøller, Rasmus; Rosendahl, Søren

    1998-01-01

    The effects of inorganic phosphate levels and the presence of arbuscular mycorrhiza on disease severity of Aphanomyces euteiches in pea roots were studied. Disease severity on roots and epicotyl as well as the oospore number within infected root tissue were correlated with the phosphorus (P) level...... in the growth medium. The arbuscular mycorrhizal fungus Glomus intraradices increased P uptake and the P concentration in the plant but reduced disease development in peas. Polyacrylamide gel electrophoresis followed by densitometry of glucose-6-phosphate dehydrogenase specific to A.euteiches was used...... to measure the activity of the pathogen in roots. The enzyme activity increased with disease severity and disease incidence, except in plants supplemented with P at the highest level, where a peak in activity was seen 12 days after inoculation with the pathogen, followed by a decrease in activity...

  18. [Clinical Investigation of the Effects of Filgrastim BS1 on Neutropenia Following Oral Cancer Chemotherapy (TPF Therapy)].

    Science.gov (United States)

    Uchiyama, Kimio; Yamada, Manabu; Tamate, Shusuke; Iwasaki, Konomi; Mitomo, Keisuke; Nakayama, Seiichi

    2015-09-01

    The time for the neutrophil count to recover after subcutaneous injection of filgrastim BS1 or lenograstim was studied in patients suffering from neutropenia following preoperative combined chemotherapy using docetaxel, nedaplatin, or cisplatin (in divided doses for 5 days)and 5-fluorouracil for oral cancer. 1. There was no significant difference in the minimum leukocyte and neutrophil counts after chemotherapy. 2. There was no significant difference in the maximum leukocyte and neutrophil counts after chemotherapy. 3. Time for leukocytes to recover from their minimum count(>4,000/mm3)or for neutrophils to recover from their minimum count(>2,000/mm3)and the number of days on which treatment was administered tended to be shorter in the filgrastim BS1 group. Thus, it was concluded that filgrastim BS1 is just as effective as other prior G-CSF agents in treating patients suffering from neutropenia following chemotherapy(TPF therapy). PMID:26469162

  19. Giant Cell Arteritis which Developed after the Administration of Granulocyte-colony Stimulating Factor for Cyclic Neutropenia.

    Science.gov (United States)

    Umeda, Masataka; Ikenaga, Jin; Koga, Tomohiro; Michitsuji, Toru; Shimizu, Toshimasa; Fukui, Shoichi; Nishino, Ayako; Nakasima, Yoshikazu; Kawashiri, Sin-Ya; Iwamoto, Naoki; Ichinose, Kunihiro; Hirai, Yasuko; Tamai, Mami; Nakamura, Hideki; Origuchi, Tomoki; Kawakami, Atsushi

    2016-01-01

    A 78-year-old woman diagnosed with cyclic neutropenia 5 years previously had been treated with recombinant granulocyte-colony stimulating factor (G-CSF). She developed fever, tenderness and distension of temporal arteries after the treatment with G-CSF. Magnetic resonance imaging and ultrasonography revealed wall thickening of the temporal arteries. She was therefore diagnosed with giant cell arteritis (GCA). Small vessel vasculitis has been reported as a complication of G-CSF. However, the development of large vessel vasculitis after G-CSF treatment is quite rare. To our knowledge, the present case is the first report of GCA suspected to be associated with coexisting cyclic neutropenia and G-CSF treatment. PMID:27523011

  20. CONTENTS OF LYMPHOCYTE SUB-POPULATIONS IN THE CHILDREN WITH ACUTE LEUKEMIA AND LYMPHOMAS DEPENDENT ON INFECTIOUS COMPLICATION AND NEUTROPENIA

    Directory of Open Access Journals (Sweden)

    M. V. Peshikova

    2005-01-01

    Full Text Available Abstract. The aim of the present work was to evaluate the contents of some lymphocyte sub-populations in peripheral blood of the children with tumors of hematopoietic and lymphoid tissues, depending on infectious complication of cytostatic therapy and neutropenia. In all children undergoing cytostatic therapy for acute lympho-blastic leukemia and non-B cell non-Hodgkinґs lymphomas, we found significant decrease in the numbers of CD95 lymphocytes, absolute amounts of natural killer cells (CD16, CD56-lymphocytes and activated lymphocytes (СD11b, HLA-DR-cells, irrespective of neutrophile numbers in their blood and infectious complications. However, absolute number of CD25- lymphocytes was significantly decreased in the children with neutropenia. Relative contents of CD16, CD56, СD11b, HLA-DR, CD25-lymphocytes did not significantly differ from those in healthy children, or they were found to be significantly increased.

  1. CONTENTS OF LYMPHOCYTE SUB-POPULATIONS IN THE CHILDREN WITH ACUTE LEUKEMIA AND LYMPHOMAS DEPENDENT ON INFECTIOUS COMPLICATION AND NEUTROPENIA

    Directory of Open Access Journals (Sweden)

    M. V. Peshikova

    2014-07-01

    Full Text Available Abstract. The aim of the present work was to evaluate the contents of some lymphocyte sub-populations in peripheral blood of the children with tumors of hematopoietic and lymphoid tissues, depending on infectious complication of cytostatic therapy and neutropenia. In all children undergoing cytostatic therapy for acute lympho-blastic leukemia and non-B cell non-Hodgkinґs lymphomas, we found significant decrease in the numbers of CD95 lymphocytes, absolute amounts of natural killer cells (CD16, CD56-lymphocytes and activated lymphocytes (СD11b, HLA-DR-cells, irrespective of neutrophile numbers in their blood and infectious complications. However, absolute number of CD25- lymphocytes was significantly decreased in the children with neutropenia. Relative contents of CD16, CD56, СD11b, HLA-DR, CD25-lymphocytes did not significantly differ from those in healthy children, or they were found to be significantly increased.

  2. Rapid lung MRI - paradigm shift in evaluation of febrile neutropenia in children with leukemia: a pilot study.

    Science.gov (United States)

    Sodhi, Kushaljit Singh; Khandelwal, Niranjan; Saxena, Akshay Kumar; Bhatia, Anmol; Bansal, Deepak; Trehan, Amita; Singh, Meenu; Agarwal, Ritesh

    2016-01-01

    Immunocompromised children with hematological malignancies are at increased risk of developing potentially fatal pulmonary infections. Early detection and prompt treatment is critical to combat morbidity and mortality in these children. Twenty-six children with leukemia (age range: 5-13years) presenting with fever and neutropenia were included in this prospective study, which was approved by the institutional ethics committee. All patients underwent HRCT and MRI of the chest on the same day. The findings of HRCT and MRI were compared, with HRCT as the standard of reference. There was perfect agreement between MRI and CT examinations findings by kappa test (κ = 1). No significant difference was observed between the two modalities by the McNemar test (p > 0.05). Rapid lung MRI is technically feasible; has a high correlation, sensitivity and specificity to CT scan; and can emerge as the first line modality for the detection of pulmonary nodules in children with leukemia and persistent febrile neutropenia.

  3. Plasmodium falciparum variant surface antigen expression varies between isolates causing severe and nonsevere malaria and is modified by acquired immunity

    DEFF Research Database (Denmark)

    Nielsen, Morten A; Staalsoe, Trine; Kurtzhals, Jørgen;

    2002-01-01

    of immunity, as disease-causing parasites appear to be those not controlled by preexisting VSA-specific Abs. In this work we report that VSA expressed by parasites from young Ghanaian children with P. falciparum malaria were commonly and strongly recognized by plasma Abs from healthy children in the same area......, and that the VSA expression pattern is modulated by immunity. This opens the possibility of developing morbidity-reducing vaccines targeting a limited subset of common and particularly virulent VSA....

  4. Cytokine and acute phase protein mRNA expression in liver tissue from pigs with severe sepsis caused by intravenous inoculation of Staphylococcus aureus

    DEFF Research Database (Denmark)

    Nielsen, Ole Lerberg; Olsen, Helle Gerda; Iburg, Tine;

    2010-01-01

    The aim was to substantiate previous findings of hepatic dysfunction in a porcine model of Staphylococcus aureus induced severe sepsis. Nine pigs were inoculated intravenously once or twice with 108S. aureus per kilogram body weight and killed 12, 24 and 48 h later. Three pigs served as controls...

  5. Prevalence, severity, causes and drugs used for depression, stress and anxiety among junior doctors in a tertiary care teaching hospital in South India

    Directory of Open Access Journals (Sweden)

    Prasanand Sasidharan

    2016-06-01

    Conclusions: Overall higher stress was observed among post graduates compared to interns and females compared to males. Anxiety was more severe compared to depression and stress among junior doctors. Reducing working hours and increasing workplace flexibility are some measures to reduce stress among the junior doctors. [Int J Basic Clin Pharmacol 2016; 5(3.000: 1118-1124

  6. Neutropenia relacionada con la quimioterapia en tumores sólidos. Hospital General Universitario Camilo Cienfuegos Enero 2009- 2013

    Directory of Open Access Journals (Sweden)

    Yania Luisa Jiménez Madrigal

    2015-09-01

    Full Text Available Se realizó un estudio descriptivo de corte transversal y retrospectivo referente al comportamiento de la neutropenia relacionada con la quimioterapia en tumores sólidos en el período comprendido Enero 2009- 2013 atendido en el servicio de oncología del Hospital General Universitario Camilo Cienfuegos de la provincia Sancti-Spíritus. La población la conformaron 230 enfermos neutropénicos relacionados con la quimioterapia. La investigación describe de forma general la relación existente entre la neutropenia con la quimioterapia, localización tumoral, entidades nosológicas asociadas, edad, sexo, tratamientos quimioterapéuticos y antibióticos más utilizados. Pudo observarse que existe alta incidencia de neutropenia relacionada con la quimioterapia en pacientes con cáncer de pulmón, el grupo más afectado fue el de 50-59 años predominando el sexo femenino con un alto índice de hipertensos asociados. Los tratamientos más utilizados fueron Etopóxido-Platino y Ceftrixone-Amikacina.

  7. Sustained oxidative stress causes late acute renal failure via duplex regulation on p38 MAPK and Akt phosphorylation in severely burned rats.

    Directory of Open Access Journals (Sweden)

    Yafei Feng

    Full Text Available BACKGROUND: Clinical evidence indicates that late acute renal failure (ARF predicts high mortality in severely burned patients but the pathophysiology of late ARF remains undefined. This study was designed to test the hypothesis that sustained reactive oxygen species (ROS induced late ARF in a severely burned rat model and to investigate the signaling mechanisms involved. MATERIALS AND METHODS: Rats were exposed to 100°C bath for 15 s to induce severe burn injury (40% of total body surface area. Renal function, ROS generation, tubular necrosis and apoptosis, and phosphorylation of MAPK and Akt were measured during 72 hours after burn. RESULTS: Renal function as assessed by serum creatinine and blood urea nitrogen deteriorated significantly at 3 h after burn, alleviated at 6 h but worsened at 48 h and 72 h, indicating a late ARF was induced. Apoptotic cells and cleavage caspase-3 in the kidney went up slowly and turned into significant at 48 h and 72 h. Tubular cell ROS production shot up at 6 h and continuously rose during the 72-h experiment. Scavenging ROS with tempol markedly attenuated tubular apoptosis and renal dysfunction at 72 h after burn. Interestingly, renal p38 MAPK phosphorylation elevated in a time dependent manner whereas Akt phosphorylation increased during the first 24 h but decreased at 48 h after burn. The p38 MAPK specific inhibitor SB203580 alleviated whereas Akt inhibitor exacerbated burn-induced tubular apoptosis and renal dysfunction. Furthermore, tempol treatment exerted a duplex regulation through inhibiting p38 MAPK phosphorylation but further increasing Akt phosphorylation at 72 h postburn. CONCLUSIONS: These results demonstrate that sustained renal ROS overproduction induces continuous tubular cell apoptosis and thus a late ARF at 72 h after burn in severely burned rats, which may result from ROS-mediated activation of p38 MAPK but a late inhibition of Akt phosphorylation.

  8. Poliartritis y tenosinovitis grave por Streptococcus agalactiae en un paciente con hipoesplenia funcional Severe polyarthritis and tenosynovitis caused by Streptococcus agalactiae in a patient with functional hyposplenia

    Directory of Open Access Journals (Sweden)

    Domingo C. Balderramo

    2002-08-01

    Full Text Available La artritis por Streptococcus agalactiae es infrecuente. No conocemos publicaciones de casos sobre la afección tendinosa por este microorganismo. Se presenta una mujer de 46 años que consultó por fiebre, poliartralgias, mialgias, diarrea y vómitos. Como antecedentes presentaba carcinoma papilar de tiroides e hipoesplenia funcional. Al examen se encontraba hemodinámicamente inestable, febril, con artritis de mano izquierda, muñecas, codos, hombro derecho y tobillo izquierdo. Presentaba tenosinovitis en ambos pies y en la mano izquierda. Los hemocultivos y el cultivo de la bursa olecraniana derecha fueron positivos para S. agalactiae. La ecografía mostró signos de tenosinovitis del tibial anterior izquierdo. Completó 20 días de tratamiento endovenoso con cefazolina y 12 días de cefuroxima oral. El cuadro articular revirtió completamente en 60 días. El Streptococcus agalactiae puede causar, en forma infrecuente, un síndrome de poliartritis, tenosinovitis y fiebre similar al producido por la infección gonocócica.Cases of arthritis caused by Streptococcus agalactiae are infrequent and in our knowledge there are no case reports of tenosynovitis caused by S. agalactiae. A 46-year-old woman presented with fever, polyarthralgia, myalgia, diarrhea and vomiting. She had a history of papillary thyroid carcinoma and functional hyposplenia. She was febrile, with arthritis in hands, wrists, elbows, right shoulder and left ankle joints, and presented tenosynovitis in both feet and left hand. Blood and right olecranon bursa sample cultures were positive for S. agalactiae. An ultrasound scan made at the musculus tibialis anterior of left foot revealed signs of tenosynovitis. She was treated with intravenous cefazolin for 20 days and oral cefuroxime for 12 days. The joint involvement completely subsided in 60 days. Streptococcus agalactiae can cause, infrequently, a polyarthritis and tenosynovitis syndrome similar to disseminated gonococcal

  9. Clinical analysis of 280 children with infection associated neutropenia%感染相关性儿童中性粒细胞减少症280例临床分析

    Institute of Scientific and Technical Information of China (English)

    刘雪; 刘瑞清; 邓军霞; 张国成; 成胜权; 陈彩平; 孙新; 许东亮; 邓跃林; 林海波

    2013-01-01

    Objectives To explore the related factors and clinical characteristics of infection associted neutropenia in children. Methods Retrospectively reviewed the medical records of 280 neutropenia in-patients from September 2010 to August 2011. Their clinical features, laboratory investigations, treatment and prognosis were analyzed. Serum immunoglobulins were compared with 65 healthy control children. Results Among 280 children, 78% of them were younger than 3 years old and 62.5% were male, arid 90.7% were with respiratory infection. The medical records indicated the number of patients was slightly increased in January, March and August when compared with other months throughout the year. Virus infection was the most common cause of acquired neutropenia. In patients younger than 12 month old, IgM was higher and IgG was lower than those of control group (all P<0.05). In the first week of infection 79.7% of patients were found with neutropenia, and drugs that can increase granuloeytes would shorten the recovery time. Conclusions Neutropenia associated infection are more common in male infants and usually caused by viral infection accompanied with immune dysfunction. Used properly and timely, antiviral drugs and antibiotics can shorten the duration of symptoms.%目的 探讨儿童感染相关性中性粒细胞减少症的影响因素及临床特点.方法 收集2010年9月至2011年8月住院并符合中性粒细胞减少症诊断标准的患儿280例,对其病史资料、临床表现、实验室检查、临床治疗及预后进行回顾性分析,并与65名正常对照儿童作血清免疫球蛋白比较.结果 280例中性粒细胞减少症患儿中,3岁以下占78%,男性占62.5%;呼吸道感染占90.7%;全年均可发病,1、3、8月份患儿数量增多.病毒感染是引起儿童继发性中性粒细胞减少的主要原因.<12个月患儿的IgM高于正常对照儿童,IgG低于正常对照儿童,差异均有统计学意义 (P 均<0.05).感染第1

  10. Factores de mal pronóstico en pacientes internados con Neutropenia al inicio del episodio febril Prognostic risk factors for serious complications in an inpatient population with neutropenia at the onset of a febrile episode

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    Carlos Gómez Roca

    2006-10-01

    Full Text Available Los pacientes con neutropenia y fiebre constituyen una población heterogénea con riesgo variable para el desarrollo de complicaciones serias y mortalidad. El objetivo de este trabajo es identificar factores que, presentes al ingreso, estuvieran asociados a mayor riesgo de complicaciones graves en pacientes que se internan por neutropenia y fiebre. Se trata de un estudio de seguimiento de una cohorte de 238 episodios de neutropenia y fiebre (neutrófilos 38.3 °C en 167 pacientes internados en sala general en nuestra institución desde 1997 a 2004. Ochenta y dos por ciento de los pacientes tenían enfermedad hematológica, 14% tumores sólidos y 4% no asociados a quimioterapia. Se registraron 67 eventos adversos (46% de insuficiencia renal, 27% de hipotensión refractaria, 15% de insuficiencia respiratoria y 12% con sangrado mayor. Se hallaron diferencias significativas en presencia de comorbilidades previas, temperatura mayor a 39 °C, frecuencia cardíaca mayor a 120 latidos por minuto, frecuencia respiratoria mayor a 24 por minuto, tensión arterial sistólica menor a 90 mm Hg, presencia de 3 o más valores de laboratorio alterados al ingreso, presencia de foco clínico y hemocultivos positivos. En el análisis multivariado de regresión logística mantuvieron asociación independiente con mayor riesgo de eventos graves: hipotensión arterial sistólica (OR=7, pPatients with neutropenia and fever conform a heterogeneous population with a variable risk of serious complications and mortality. The goal of this study was to identify prognostic risk factors present at the beginning of the episode, for adverse events and serious complications in patients admitted in a general ward with fever and neutropenia. A cohort of 238 episodes with neutropenia and fever (neutrophils 38.3 °C in 167 patients admitted to our general hospital between 1997 and 2004 was followed. Eighty two percent of the patients had hematologic malignancies, 14% solid tumors

  11. Genetic background alters the severity and onset of neuromuscular disease caused by the loss of ubiquitin-specific protease 14 (usp14.

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    Andrea G Marshall

    Full Text Available In this study, we identified and characterized an N-ethyl-N-nitrosourea (ENU induced mutation in Usp14 (nmf375 that leads to adult-onset neurological disease. The nmf375 mutation causes aberrant splicing of Usp14 mRNA, resulting in a 95% reduction in USP14. We previously showed that loss of USP14 in ataxia (ax (J mice results in reduced ubiquitin levels, motor endplate disease, Purkinje cell axonal dystrophy and decreased hippocampal paired pulse facilitation (PPF during the first 4-6 weeks of life, and early postnatal lethality by two months of age. Although the loss of USP14 is comparable between the nmf375 and ax (J mice, the nmf375 mice did not exhibit these ax (J developmental abnormalities. However, by 12 weeks of age the nmf375 mutants present with ubiquitin depletion and motor endplate disease, indicating a continual role for USP14-mediated regulation of ubiquitin pools and neuromuscular junction (NMJ structure in adult mice. The observation that motor endplate disease was only seen after ubiquitin depletion suggests that the preservation of NMJ structure requires the stable maintenance of synaptic ubiquitin pools. Differences in genetic background were shown to affect ubiquitin expression and dramatically alter the phenotypes caused by USP14 deficiency.

  12. CAUSES THAT LEAD TO THE SEVERE DECLINE IN THE NUMBER OF CATTLE BRED BY SUBSISTENCE FARMERS IN PLATARESTI COMMUNE, CALARASI COUNTY, ROMANIA

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    Ana-Maria-Loredana PREDA

    2013-01-01

    Full Text Available Romania is a country of contrasts especially as agriculture is concerned. On one side we have state of the art machinery and equipment being used on farms larger than 100 ha and on the other side we have small subsistence farms operated by an aging and poor population. The paper aims to highlight few causes that have stressed even more the difficult situation of the rural population of this country; especially those that have been brought about by the financial crisis initiated in 2008 and that have gotten even worse with the passing of time. We have performed a qualitative and quantitative analysis showing the decline in the number of cattle bred, either for milk or meat, by the subsistence farmers from Plataresti commune, Calarasi County, Romania, allowing us to identify few causes that could be counteracted by a proactive social measure. The results of our research indicate one possible measure meant to counteract negative effects that subsistence farmers are now facing.

  13. Survey for incidence and severity of bacterial blight of cotton caused by Xanthomonas axonopodis pv. malvacearum in different districts of Marathwada region

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    D. Utpal

    2012-07-01

    Full Text Available An extensive roving survey was conducted in different districts of Marathwada region of Maharashtra State to isolate the pathogen associated with the bacterial blight disease of cotton. In all 76 cotton fields were surveyed and average disease incidence (PI to the tune of 51.12 per cent has been observed. Highest disease incidence noticed in Parbhani district (67% followed by Hingoli (63%, Nanded (58% and Latur (54%. The lowest disease incidence noticed in Jalna district (36%. Highest disease severity noticed in Parbhani district followed by Hingoli, Latur, Nanded and Aurangabad.

  14. Report of 15 injuries caused by lionfish (pterois volitans) in aquarists in Brazil: a critical assessment of the severity of envenomations.

    Science.gov (United States)

    Haddad, Vidal; Stolf, Hamilton Ometto; Risk, José Yamin; França, Francisco Os; Cardoso, João Luiz Costa

    2015-01-01

    Lionfish are venomous fish that belong to the Scorpaenidae family. Individuals of this family and those of the Synanceiidae family comprise most of the existing venomous fish in the world. Lionfish are originally found in the Indo-Pacific, but they have received special attention in the last years for their dissemination in the Atlantic Ocean, with the emergence of large populations in the USA, Caribbean and South America. Because of its beauty, this fish has always been present in private and commercial aquariums around the world. Herein, we describe 15 envenomations in aquarists registered in a period of eighteen years (1997-2014). The stings caused excruciating pain and marked inflammation, with local erythema, edema, heat, paleness and cyanosis. In one case, it was possible to observe vesicles and blisters. There were no skin necroses or marked systemic manifestations. We discuss the possible coming of the fish to South America and the circumstances and clinical impact of the envenomations. PMID:25810712

  15. Selective Interaction Between Chloroplast β-ATPase and TGB1L88 Retards Severe Symptoms Caused by Alternanthera mosaic virus Infection

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    Eun-Young Seo

    2014-03-01

    Full Text Available The multifunctional triple gene block protein 1 (TGB1 of the Potexvirus Alternanthera mosaic virus (AltMV has been reported to have silencing suppressor, cell-to-cell movement, and helicase functions. Yeast two hybrid screening using an Arabidopsis thaliana cDNA library with TGB1 as bait, and co-purification with TGB1 inclusion bodies identified several host proteins which interact with AltMV TGB1. Host protein interactions with TGB1 were confirmed by biomolecular fluorescence complementation, which showed positive TGB1 interaction with mitochondrial ATP synthase delta′ chain subunit (ATP synthase delta′, light harvesting chlorophyll-protein complex I subunit A4 (LHCA4, chlorophyll a/b binding protein 1 (LHB1B2, chloroplast-localized IscA-like protein (ATCPISCA, and chloroplast β-ATPase. However, chloroplast β-ATPase interacts only with TGB1L88, and not with weak silencing suppressor TGB1P88. This selective interaction indicates that chloroplast β-ATPase is not required for AltMV movement and replication; however, TRV silencing of chloroplast β-ATPase in Nicotiana benthamiana induced severe tissue necrosis when plants were infected by AltMV TGB1L88 but not AltMV TGB1P88, suggesting that β-ATPase selectively responded to TGB1L88 to induce defense responses.

  16. The first report in Brazil of severe infection caused by community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA

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    R. Rozenbaum

    2009-08-01

    Full Text Available Community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA is an emergent pathogen in Brazil. However, there are no data on the prevalence of CA-MRSA. We report here the first well-characterized case of severe life-threatening CA-MRSA infection in a child living in Rio de Janeiro city. The patient had many complications including hematogenous osteomyelitis and involvement of multiple sites requiring drainage of soft-tissue abscess, and pleural and pericardial empyema. The MRSA isolates recovered were genotyped using PFGE, SCCmec typing and multilocus sequence typing. Disk diffusion tests were performed following Clinical and Laboratory Standards Institute recommendations. In addition, the presence of Panton-Valentine leukocidin (PVL was assessed by PCR amplification, using specific primers for lukF-pv (encoding for the F subunit of the PVL. The bacterial isolates were related to the ST30-SCCmecIV lineage (Oceania Southwest Pacific clone, a PVL producer CA-MRSA previously detected in Porto Alegre, RS, Brazil. Also, the isolates analyzed were susceptible to all non-β-lactam antibiotics tested. The present report demonstrates that disseminated CA-MRSA disease is also occurring in Rio de Janeiro. Thus, the empirical treatment of moderate or severe infections suspected of being associated with CA-MRSA needs to be reviewed in order to allow prompt initiation of an effective therapy that also covers these microorganisms.

  17. [Hypopotassemia and prolongation of the Q-T interval in a patient with severe malnutrition caused by bulimia and post-prandial vomiting].

    Science.gov (United States)

    Contaldo, F; Di Paolo, M R; Mazzacano, C; Di Biase, G; Giumetti, D

    1990-04-01

    Patient L.A. (f., 20 yrs), affected by bulimia and self-induced vomiting, was hospitalized because of severe malnutrition (BMI 13.1), hypopotassemia (2.8 mEq/l) and prolonged QTc interval (0.469"). Intensive care treatment aimed to normalize mineral balance mainly serum potassium, consisted of administering e.v. potassium (mg 2346/day), magnesium (mg 72/day), calcium (mg 80/day), phosphorus (mg 769/day), chloride (mg 710/day), iron (mg 40/day). Dietary treatment was deliberately chosen to be slightly above minimum energy requirements in order to avoid possible side effects of forced hyperalimentation. The patient, immediately after hospitalization, interrupted vomiting and 2 wks later weight increased by 5 kg (from 34.9 kg to 40.0 kg). On the other hand normalization of serum potassium levels was slow and QTc interval reached normal range only after the 10th day of treatment (0.447"). This case supports the hypothesis that major ECG abnormalities may be present in severe malnutrition due to anorexia nervosa or bulimia with self-induced vomiting. The dangers of these complications were substantiated by the fact that intensive care treatment allowed prompt body weight recovery but normalization of electrolytic balance and cardiac function was very slow. For such patients, electrocardiographic monitoring should be routine. PMID:2377804

  18. A convenient model of severe, high incidence autoimmune gastritis caused by polyclonal effector T cells and without perturbation of regulatory T cells.

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    Eric Tu

    Full Text Available Autoimmune gastritis results from the breakdown of T cell tolerance to the gastric H(+/K(+ ATPase. The gastric H(+/K(+ ATPase is responsible for the acidification of gastric juice and consists of an α subunit (H/Kα and a β subunit (H/Kβ. Here we show that CD4(+ T cells from H/Kα-deficient mice (H/Kα(-/- are highly pathogenic and autoimmune gastritis can be induced in sublethally irradiated wildtype mice by adoptive transfer of unfractionated CD4(+ T cells from H/Kα(-/- mice. All recipient mice consistently developed the most severe form of autoimmune gastritis 8 weeks after the transfer, featuring hypertrophy of the gastric mucosa, complete depletion of the parietal and zymogenic cells, and presence of autoantibodies to H(+/K(+ ATPase in the serum. Furthermore, we demonstrated that the disease significantly affected stomach weight and stomach pH of recipient mice. Depletion of parietal cells in this disease model required the presence of both H/Kα and H/Kβ since transfer of H/Kα(-/- CD4(+ T cells did not result in depletion of parietal cells in H/Kα(-/- or H/Kβ(-/- recipient mice. The consistency of disease severity, the use of polyclonal T cells and a specific T cell response to the gastric autoantigen make this an ideal disease model for the study of many aspects of organ-specific autoimmunity including prevention and treatment of the disease.

  19. Percutaneous transluminal angioplasty and stenting for severe stenosis of the intracranial extradural internal carotid artery causing transient ischemic attack or minor stroke.

    Science.gov (United States)

    Ko, Jun Kyeung; Choi, Chang Hwa; Cha, Seung Heon; Choi, Byung Kwan; Cho, Won Ho; Kang, Tae Ho; Sung, Sang Min; Cho, Han Jin; Lee, Tae Hong

    2015-08-01

    The purpose of this study is to assess the technical feasibility and clinical efficacy of percutaneous transluminal angioplasty and stenting (PTAS) for symptomatic stenosis of the intracranial extradural (petrous and cavernous) internal carotid artery (ICA).Review of medical records identified 26 consecutive patients who underwent PTAS using a balloon-expandable coronary stent (n = 15, 57.7%) or a Wingspan self-expandable stent (n = 11, 42.3%) for treatment of severe stenosis (>70%) involving the intracranial extradural ICA. The inclusion criteria were transient ischemic attack with an ABCD(2) score of ≥3 (n = 12, 46.2%) or minor stroke with an NIHSS score of ≤4 (n = 14, 53.8%). Technical success rates, complications, and angiographic and clinical outcomes were analyzed retrospectively.PTAS was technically successful in all patients. The mean stenosis ratio decreased from 77.1% to 10.0% immediately after PTAS. The overall incidence of procedural complications was 23.1%, and the postoperative permanent morbidity/mortality rate was 7.7%. A total of 22 patients were tracked over an average period of 29.9 months. During the observation period, 20 patients (90.9%) had no further cerebrovascular events and stroke recurrence occurred in two patients (9.1%), resulting in an annual stroke risk of 3.7%. Two cases (11.1%) of significant in-stent restenosis (>50%) were found on follow-up angiography (n = 18).PTAS for severe stenosis (>70%) involving the intracranial extradural ICA showed a good technical feasibility and favorable clinical outcome in patients with transient ischemic attack or minor stroke.

  20. Severe diarrhea outbreak in beef calves (Bos indicus caused by G6P[11], an emergent genotype of bovine rotavirus group A

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    Thais N.S. Medeiros

    2014-08-01

    Full Text Available The episodes of diarrhea caused by neonatal bovine rotavirus group A (BoRVA constitute one of the major health problems in the calf rearing worldwide. The main G (VP7 and P (VP4 genotypes of BoRVA strains involved in the etiology of diarrhea in calves are G6P[1], G10P[11], G6P[5], and G8P[1]. However, less frequently, other G and P genotypes have been described in BoRVA strains identified in diarrheic fecal samples of calves. This study describes the identification and molecular characterization of an emerging genotype (G6P[11] in BoRVA strains involved in the etiology of a diarrhea outbreak in beef calves in a cattle herd of high production in extensive management system. The diarrhea outbreak, which showed high morbidity (60% and lethality (7% rates, occurred in calves (n= 384 Nelore (Bos indicus up to 30-day-old from the State of Mato Grosso do Sul, Brazil. BoRVA was identified in 80% (16/20 of the fecal samples analyzed by polyacrylamide gel electrophoresis (PAGE technique. In all PAGE-positive fecal samples were amplified products with 1,062-bp and 876-bp in the RT-PCR assays for VP7 (G type and VP4 (VP8* (P type of BoRVA, respectively. The nucleotide sequence analysis of VP7 and VP4 genes of four wild-type BoRVA strains showed G6-III P[11]-III genotype/lineage. The G6P[11] genotype has been described in RVA strains of human and animal hosts, however, in calves this genotype was only identified in some cross-sectional studies and not as a single cause of diarrhea outbreaks in calves with high morbidity and lethality rates as described in this study. The monitoring of the G and P genotypes of BoRVA strains involved in diarrhea outbreaks in calves is important for both animal and public health by allowing the identification of the most frequent genotypes, the characterization of novel genotypes and to identify reassortments with genotypes described in animal and human hosts. The results of this study show the importance of the monitoring of

  1. 静注人免疫球蛋白致严重过敏反应1例%One Case of Severe Allergic Reaction Caused by Intravenous Immunoglobulin

    Institute of Scientific and Technical Information of China (English)

    张金连; 贾书平; 雷招宝

    2016-01-01

    A female patient at age of 25 years,with primary immune-related infertility was given 50 mL(2.5 g) of intravenous human immunoglobulin(PH4)by intravenous drip at a rate of 20 drops / min. Systemic skin rash, most of which in neck and face,appeared 5 mins,while the symptoms such as sudden screaming,abdominal pain, feeling of shit,systemic skin flushing,rash(especially in neck and face)and shortness of breath,10 mins after drip,which was considered as allergic reaction caused by intravenous immunoglobulin. The symptoms were relieved 30 mins and disappeared 1 h after the drip was stopped immediately and anti-anaphylaxis therapy was adopted. It suggests that the allergic reactions caused by intravenous immunoglobulin shoud be cautioned.%1例25岁女性患者,因原发性不孕症(免疫相关性)就诊,予静注人免疫球蛋白(PH4)50 mL(2.5 g)静脉滴注(滴速20滴/min),5 min后患者出现全身皮肤点状皮疹(以颈部、脸部多见),至10 min时患者突然出现尖叫、腹痛、便意、全身皮肤发红、皮疹(以颈部、面部为甚,皮疹高出皮肤)、呼吸急促等症状。考虑为静注人免疫球蛋白(PH4)所致的过敏反应。立即停药,予抗过敏治疗,30 min后症状缓解,1 h后恢复正常。提示应警惕静注人免疫球蛋白的过敏反应。

  2. Deletion of apolipoprotein E receptor-2 in mice lowers brain selenium and causes severe neurological dysfunction and death when a low-selenium diet is fed.

    Science.gov (United States)

    Burk, Raymond F; Hill, Kristina E; Olson, Gary E; Weeber, Edwin J; Motley, Amy K; Winfrey, Virginia P; Austin, Lori M

    2007-06-01

    Selenoprotein P (Sepp1) is a plasma and extracellular protein that is rich in selenium. Deletion of Sepp1 results in sharp decreases of selenium levels in the brain and testis with dysfunction of those organs. Deletion of Sepp1 also causes increased urinary selenium excretion, leading to moderate depletion of whole-body selenium. The lipoprotein receptor apolipoprotein E receptor-2 (apoER2) binds Sepp1 and facilitates its uptake by Sertoli cells, thus providing selenium for spermatogenesis. Experiments were performed to assess the effect of apoER2 on the concentration and function of selenium in the brain and on whole-body selenium. ApoER2-/- and apoER2+/+ male mice were fed a semipurified diet with selenite added as the source of selenium. ApoER2-/- mice had depressed brain and testis selenium, but normal levels in liver, kidney, muscle, and the whole body. Feeding a selenium-deficient diet to apoER2-/- mice led to neurological dysfunction and death, with some of the characteristics exhibited by Sepp1-/- mice fed the same diet. Thus, although it does not affect whole-body selenium, apoER2 is necessary for maintenance of brain selenium and for prevention of neurological dysfunction and death under conditions of selenium deficiency, suggesting an interaction of apoER2 with Sepp1 in the brain.

  3. A Severe Accident Caused by an Ocellate River Stingray (Potamotrygon motoro in Central Brazil: How Well Do We Really Understand Stingray Venom Chemistry, Envenomation, and Therapeutics?

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    Nelson Jorge da Silva

    2015-06-01

    Full Text Available Freshwater stingrays cause many serious human injuries, but identification of the offending species is uncommon. The present case involved a large freshwater stingray, Potamotrygon motoro (Chondrichthyes: Potamotrygonidae, in the Araguaia River in Tocantins, Brazil. Appropriate first aid was administered within ~15 min, except that an ice pack was applied. Analgesics provided no pain relief, although hot compresses did. Ciprofloxacin therapy commenced after ~18 h and continued seven days. Then antibiotic was suspended; however, after two more days and additional tests, cephalosporin therapy was initiated, and proved successful. Pain worsened despite increasingly powerful analgesics, until debridement of the wound was performed after one month. The wound finally closed ~70 days after the accident, but the patient continued to have problems wearing shoes even eight months later. Chemistry and pharmacology of Potamotrygon venom and mucus, and clinical management of freshwater stingray envenomations are reviewed in light of the present case. Bacterial infections of stingray puncture wounds may account for more long-term morbidity than stingray venom. Simultaneous prophylactic use of multiple antibiotics is recommended for all but the most superficial stingray wounds. Distinguishing relative contributions of venom, mucus, and bacteria will require careful genomic and transcriptomic investigations of stingray tissues and contaminating bacteria.

  4. An in-vitro evaluation of antibacterial effect of Amalgomer CR and Fuji VII against bacteria causing severe early childhood caries

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    Rajesh Hemant Bariker

    2016-01-01

    Full Text Available Objective: To evaluate the antibacterial action of Amalgomer CR and Fuji VII against bacteria causing S-early childhood caries. Materials and Methods: Antibacterial activity of Amalgomer CR and Fuji VII was assessed using the agar diffusion test in triplicate. The powder and liquid of each test material was mixed and inserted in the punched wells (6 mm × 2 mm. A composition of 0.2% chlorhexidine digluconate acted as control. The agar plates were incubated at 37°C for 24 h for Streptococcus mutans, S. salivarius, S. parasanguinis and Actinomyces viscosus, whereas Lactobacillus casei was incubated for 48 h. Sizes of the inhibition zones were calculated by subtracting the diameter of the specimen (6 mm from the average of the three measurements of the halo. For each test material against each bacteria, 9 measurements were made (3 measurements × 3 times. Kruskal-Wallis test was done to compare the zones of inhibition of test materials against individual bacteria. Pair-wise comparison was done by Mann-Whitney U-test. Results: Amalgomer CR had the most antibacterial against S. mutans (31.0 mm, followed by A. viscosus (21.87 mm, S. salivarius (13.87 mm, S. parasanguinis (10.80 mm, and L. casei (9.69 mm. Fuji VII had the most antibacterial action against S. salivarius (10.65 mm, followed by A. viscosus (9.10 mm. However, it did not inhibit the growth of S. mutans (0 mm, S. parasanguinis (0 mm, and L. casei (0 mm. Conclusion: Amalgomer CR and Fuji VII showed wide variation in antibacterial action against all test organisms.

  5. Depletion of Aspergillus nidulans cotA causes a severe polarity defect which is not suppressed by the nuclear migration mutation nudA2.

    Science.gov (United States)

    Johns, Sarah Anne; Leeder, Abigail Claire; Safaie, Mehran; Turner, Geoffrey

    2006-06-01

    The Aspergillus nidulans homologue of Neurospora crassa cot-1, cotA, encoding a member of the NDR protein kinase family, has been cloned and expressed under the control of the conditional alcA promoter. Depletion of CotA by repression of the alcA promoter led to a severe growth defect accompanied by loss of polarity. Germlings show greatly enlarged volume of the spores and hyphae, accompanied by an increase in number of nuclei per compartment, though the nucleus/volume ratio is not significantly altered. The depleted CotA phenotype was not suppressed by a nuclear migration mutation nudA2. Double mutants showed an additive, defective phenotype, unlike the suppression of the cot-1 ts mutation by ropy mutations seen in N. crassa, suggesting a different relationship between nuclear migration and the cot signalling pathway in A. nidulans. A functional CotA-GFP fusion protein was found in punctate regions of fluorescence similar to the distribution reported for human NDR2, and as a cap at the hyphal tip.

  6. 儿童恶性肿瘤化疗后中性粒细胞减少并发脓毒症的早期识别%Early identification of sepsis in cancer children with neutropenia after chemotherapy

    Institute of Scientific and Technical Information of China (English)

    杨燕文; 王莹; 汤静燕; 李璧如

    2012-01-01

    .Conclusion High initial temperature,long duration of neutropenia,severely reduced ANC,increases of CRP and PCT,and culture-positive are correlated with sepsis in cancer children.

  7. 严重创伤漏诊引发医疗纠纷二例反思%Reflection of Medical Disputes Caused by 2 Cases of Severe Trauma

    Institute of Scientific and Technical Information of China (English)

    张连阳

    2015-01-01

    严重创伤救治时效性的提高需建立在救治人员掌握正确的伤情评估策略和技术的基础上,本文回顾分析2例严重创伤漏诊引发医疗纠纷病例资料,阐述在院内紧急救治阶段可能发生的误漏诊情况,包括未根据血流动力学状态决定患者的救治策略,未有效评估胸部创伤,影像学检查流程和策略不当,缺乏复苏效果的动态评估;聋哑创伤患者未及时行腹腔穿刺或诊断性腹腔灌洗,漏诊小肠破裂及脓毒性休克等。我国应积极推进创伤分级救治体系建设,规范伤情评估策略和流程,是成功救治创伤患者的前提。%The improved treatment for severe trauma needs the mastery of correct injury assessment strategies and techniques. By analyzing two cases of medical identification, this paper elaborates on misdiagnosis which may happen during in-hospital emergency treatment stages, including determining treatment strategies without the information about patient's hemo-dynamic status, being unable to clarify more valuable clinical manifestations for deaf patients, failing to timely perform abdom-inal paracentesis or diagnostic peritoneal lavage, failing to conduct effective assessment for chest trauma, un-appropriate sec-ondary inspection process and strategy, lacking dynamic assessment for recovery, and miss-diagnosing septic shock, etc.

  8. The quantum yield of CO2 fixation is reduced for several minutes after prior exposure to darkness. Exploration of the underlying causes.

    Science.gov (United States)

    Kirschbaum, M U F; Oja, V; Laisk, A

    2005-01-01

    Previous work has shown that the apparent quantum yield of CO2 fixation can be reduced for up to several minutes after prior exposure to darkness. In the work reported here, we investigated this phenomenon more fully and have deduced information about the underlying processes. This was done mainly by concurrent measurements of O2 and CO2 exchange in an oxygen-free atmosphere. Measurements of O2 evolution indicated that photochemical efficiency was not lost through dark adaptation, and that O2 evolution could proceed immediately at high rates provided that there were reducible pools of photosynthetic intermediates. Part of the delay in reaching the full quantum yield of CO2 fixation could be attributed to the need to build up pools of photosynthetic intermediates to high enough levels to support steady rates of CO2 fixation. There was no evidence that Rubisco inactivation contributed towards delayed CO2 uptake (under measurement conditions of low light). However, we obtained evidence that an enzyme in the reaction path between triose phosphates and RuBP must become completely inactivated in the dark. As a consequence, in dark-adapted leaves, a large amount of triose phosphates were exported from the chloroplast over the first minute of light rather than being converted to RuBP for CO2 fixation. That pattern was not observed if the pre-incubation light level was increased to just 3-5 micromol quanta m(-2) s(-1). The findings from this work underscore that there are fundamental differences in enzyme activation between complete darkness and even a very low light level of only 3-5 micromol quanta m(-2) s(-1) which predispose leaves to different gas exchange patterns once leaves are transferred to higher light levels. PMID:15666215

  9. Isquemia grave de membros inferiores por arterite por HIV Severe ischemia of lower limbs due to arteritis caused by HIV infection

    Directory of Open Access Journals (Sweden)

    Guilherme Benjamin Brandão Pitta

    2011-12-01

    Full Text Available A isquemia aguda de membros pode se manifestar, embora de forma incomum, como consequência à vasculite associada ao vírus da imunodeficiência humana (HIV. O presente caso descreve a evolução de uma paciente soropositiva para o HIV, que apresentou quadro de isquemia distal bilateral, com diminuição da temperatura de terço distal das pernas e pés, dor intensa, cianose fixa de pododátilos e ausência de pulsos distais. Submetida ao tratamento com terapia trombolítica, apresentou sinais de lesões decorrentes da isquemia e lesão tecidual de reperfusão com perda tecidual em regiões distais dos dedos, porém com melhora dos sinais e sintomas dos membros inferiores. Trata-se de um caso raro na literatura em função da associação da vasculite com o HIV e do acometimento dos vasos distais nos membros inferiores. Entretanto, o conhecimento desta associação é de extrema importância devido à repercussão na vida dos pacientes acometidos.The acute limb ischemia may manifest itself, albeit unusual, as a consequence of vasculitis associated with human immunodeficiency virus (HIV. This case report described a patient seropositive for HIV who developed bilateral distal ischemia with temperature decrease of distal legs and feet, severe pain, cyanosis of fixed toes, and absence of distal pulses. She underwent treatment with thrombolytic therapy, showed signs of injury resulting from ischemia and reperfusion tissue injury with tissue loss in the distal regions of the fingers, but with improvement of the signs and symptoms of lower limbs. It is a rare case in literature due to the association of vasculitis with HIV and to the torment of distal vases of the lower limbs. Despite of that, the knowledge of the pathology is extremely important because of the repercussion in the patients' lives.

  10. Dose-Dependent Effect of Granulocyte Transfusions in Hematological Patients with Febrile Neutropenia.

    Science.gov (United States)

    Teofili, Luciana; Valentini, Caterina Giovanna; Di Blasi, Roberta; Orlando, Nicoletta; Fianchi, Luana; Zini, Gina; Sica, Simona; De Stefano, Valerio; Pagano, Livio

    2016-01-01

    It is still under debate whether granulocyte transfusions (GTs) substantially increase survival in patients with febrile neutropenia. We retrospectively examined data relative to 96 patients with hematological malignancies receiving 491 GTs during 114 infectious episodes (IE). Patients were grouped according to the median doses of granulocytes transfused during the infectious episode (low-dose group: 3.0x108 cells/Kg). The impact of clinical, microbiological and GT-related variables on the infection-related mortality (IRM) was investigated. The IRM was not influenced by the number of GTs or by the total amount of granulocytes received, whereas a dose-related effect of the median dose received for IE was detected at univariate analysis (IRM of 18.4% in the standard-dose group, 44.4% in the low-dose group and 48.4% in the high-dose group, p = 0.040) and confirmed at multivariate analysis (OR 3.7, IC 95% 1.5-8.9; 0.004 for patients not receiving standard doses of GTs). Moreover, patients receiving GTs at doses lower or greater than standard had increased risk for subsequent ICU admission and reduced overall survival. The dose-related effect of GTs was confirmed in bacterial but not in fungal infections. Preliminary findings obtained from a subgroup of patients candidate to GTs revealed that levels of inflammatory response mediators increase in a dose-related manner after GTs, providing a possible explanation for the detrimental effect exerted by high-dose transfusions. GTs can constitute a valuable tool to improve the outcome of infections in neutropenic patients, provided that adequate recipient-tailored doses are supplied. Further investigations of the immunomodulatory effects of GTs are recommended. PMID:27487075

  11. Incidence and Impact of Baseline Electrolyte Abnormalities in Patients Admitted with Chemotherapy Induced Febrile Neutropenia

    Directory of Open Access Journals (Sweden)

    Asim Jamal Shaikh, Samira Ahmed Bawany, Nehal Masood, Ausaf Ahmed Khan, Ahmed Nadeem Abbasi, Syed Najeeb Niamutullah, Adnan Zaidi, Salman Adil, Shiyam Kumar

    2011-01-01

    Full Text Available BACKGROUND: Febrile neutropenia (FN and myelosupression remain a challenging oncologic medical emergency and dose limiting toxicity associated with chemotherapy for cancers. Various factors are known to affect the outcomes for patients diagnosed with FN. Electrolyte abnormalities have commonly been observed, but the real incidence and their impact has been only scarcely studied in literature.METHODS: This was a prospective, observational study. A total of two hundred and fifteen (215 patients admitted between January 2007 and August 2008 were included. Analysis of data was made using SPSS version16.0.Toxicity profile was graded according to CTC version 3.0.RESULTS: Almost equal number of FN was observed in both solid tumors and hematological cancers with almost equal gender distribution. Of all 83.5% patients demonstrated some electrolyte abnormalities. All grades combined, hypokalemia was seen in 48% of patients, with 51.4% having grade I, 33.3% grade III and 15.2% G IV (life threatening hypokalemia. Hyponatremia of all grades was seen in 67.9% patients, of them 60.3% had Grade I, 33.3% grade III and 0.7% patients had grade IV hyponatremia. Hypomagnesaemia (70 patients assessed was seen in 54.3% patient, 94.7% having grade I decline. Average length of stay for patients who received IV electrolyte replacement was 6.3 days compared to 4.9 days in those who did not. Out of 90 patients who required special care unit 75 had electrolyte abnormalities, of 15 patients who expired 13 had electrolyte abnormalitiesCONCLUSION: This analysis, which is first of its kind, suggests that decline in electrolyte levels is frequently observed in patients presenting with FN. These abnormalities can have independent negative impact on the outcome for such patients. Special attention should be paid to electrolyte imbalance right from the outset.

  12. Neutrophil dynamics during concurrent chemotherapy and G-CSF administration: Mathematical modelling guides dose optimisation to minimise neutropenia.

    Science.gov (United States)

    Craig, Morgan; Humphries, Antony R; Nekka, Fahima; Bélair, Jacques; Li, Jun; Mackey, Michael C

    2015-11-21

    The choice of chemotherapy regimens is often constrained by the patient's tolerance to the side effects of chemotherapeutic agents. This dose-limiting issue is a major concern in dose regimen design, which is typically focused on maximising drug benefits. Chemotherapy-induced neutropenia is one of the most prevalent toxic effects patients experience and frequently threatens the efficient use of chemotherapy. In response, granulocyte colony-stimulating factor (G-CSF) is co-administered during chemotherapy to stimulate neutrophil production, increase neutrophil counts, and hopefully avoid neutropenia. Its clinical use is, however, largely dictated by trial and error processes. Based on up-to-date knowledge and rational considerations, we develop a physiologically realistic model to mathematically characterise the neutrophil production in the bone marrow which we then integrate with pharmacokinetic and pharmacodynamic (PKPD) models of a chemotherapeutic agent and an exogenous form of G-CSF (recombinant human G-CSF, or rhG-CSF). In this work, model parameters represent the average values for a general patient and are extracted from the literature or estimated from available data. The dose effect predicted by the model is confirmed through previously published data. Using our model, we were able to determine clinically relevant dosing regimens that advantageously reduce the number of rhG-CSF administrations compared to original studies while significantly improving the neutropenia status. More particularly, we determine that it could be beneficial to delay the first administration of rhG-CSF to day seven post-chemotherapy and reduce the number of administrations from ten to three or four for a patient undergoing 14-day periodic chemotherapy. PMID:26343861

  13. Use of inflammatory molecules to predict the occurrence of fever in onco-hematological patients with neutropenia

    Energy Technology Data Exchange (ETDEWEB)

    Ribeiro, A.F. Tibúrcio; Nobre, V.; Neuenschwander, L.C. [Departamento de Clínica Médica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG (Brazil); Teixeira, A.L. [Laboratório de Imunofarmacologia, Instituto de Ciências Biológicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG (Brazil); Xavier, S.G.; Paula, F.D.F. [Departamento de Propedêutica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG (Brazil); Teixeira, M.M. [Laboratório de Imunofarmacologia, Instituto de Ciências Biológicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG (Brazil); Teixeira, J.C.A.; Bittencourt, H. [Departamento de Clínica Médica, Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte, MG (Brazil)

    2013-02-01

    Febrile neutropenia remains a frequent complication in onco-hematological patients, and changes in the circulating level of inflammatory molecules (IM) may precede the occurrence of fever. The present observational prospective study was carried out to evaluate the behavior of plasma tumor necrosis factor alpha (TNF-α), soluble TNF-α I and II receptors (sTNFRI and sTNFRII), monocyte chemoattractant protein-1 [MCP-1 or chemokine (c-c motif) ligand 2 (CCL2)], macrophage inflammatory protein-1α (MIP-1α or CCL3), eotaxin (CCL11), interleukin-8 (IL-8 or CXCL8), and interferon-inducible protein-10 (IP-10 or CXCL10) in 32 episodes of neutropenia in 26 onco-hematological patients. IM were tested on enrollment and 24-48 h before the onset of fever and within 24 h of the first occurrence of fever. Eight of 32 episodes of neutropenia did not present fever (control group) and the patients underwent IM tests on three different occasions. sTNFRI levels, measured a median of 11 h (1-15) before the onset of fever, were significantly higher in patients presenting fever during follow-up compared to controls (P = 0.02). Similar results were observed for sTNFRI and CCL2 levels (P = 0.04 for both) in non-transplanted patients. A cut-off of 1514 pg/mL for sTNFRI was able to discriminate between neutropenic patients with or without fever during follow-up, with 65% sensitivity, 87% specificity, and 93% positive predictive value. Measurement of the levels of plasma sTNFRI can be used to predict the occurrence of fever in neutropenic patients.

  14. Use of inflammatory molecules to predict the occurrence of fever in onco-hematological patients with neutropenia

    Directory of Open Access Journals (Sweden)

    A.F. Tiburcio Ribeiro

    2013-02-01

    Full Text Available Febrile neutropenia remains a frequent complication in onco-hematological patients, and changes in the circulating level of inflammatory molecules (IM may precede the occurrence of fever. The present observational prospective study was carried out to evaluate the behavior of plasma tumor necrosis factor alpha (TNF-α, soluble TNF-α I and II receptors (sTNFRI and sTNFRII, monocyte chemoattractant protein-1 [MCP-1 or chemokine (c-c motif ligand 2 (CCL2], macrophage inflammatory protein-1α (MIP-1α or CCL3, eotaxin (CCL11, interleukin-8 (IL-8 or CXCL8, and interferon-inducible protein-10 (IP-10 or CXCL10 in 32 episodes of neutropenia in 26 onco-hematological patients. IM were tested on enrollment and 24-48 h before the onset of fever and within 24 h of the first occurrence of fever. Eight of 32 episodes of neutropenia did not present fever (control group and the patients underwent IM tests on three different occasions. sTNFRI levels, measured a median of 11 h (1-15 before the onset of fever, were significantly higher in patients presenting fever during follow-up compared to controls (P = 0.02. Similar results were observed for sTNFRI and CCL2 levels (P = 0.04 for both in non-transplanted patients. A cut-off of 1514 pg/mL for sTNFRI was able to discriminate between neutropenic patients with or without fever during follow-up, with 65% sensitivity, 87% specificity, and 93% positive predictive value. Measurement of the levels of plasma sTNFRI can be used to predict the occurrence of fever in neutropenic patients.

  15. Diversity and severity of adverse reactions to quinine: A systematic review.

    Science.gov (United States)

    Liles, Nathan W; Page, Evaren E; Liles, Amber L; Vesely, Sara K; Raskob, Gary E; George, James N

    2016-05-01

    Quinine is a common cause of drug-induced thrombocytopenia and the most common cause of drug-induced thrombotic microangiopathy. Other quinine-induced systemic disorders have been described. To understand the complete clinical spectrum of adverse reactions to quinine we searched 11 databases for articles that provided sufficient data to allow evaluation of levels of evidence supporting a causal association with quinine. Three reviewers independently determined the levels of evidence, including both immune-mediated and toxic adverse reactions. The principal focus of this review was on acute, immune-mediated reactions. The source of quinine exposure, the involved organ systems, the severity of the adverse reactions, and patient outcomes were documented. One hundred-fourteen articles described 142 patients with definite or probable evidence for a causal association of quinine with acute, immune-mediated reactions. These reactions included chills, fever, hypotension, painful acral cyanosis, disseminated intravascular coagulation, hemolytic anemia, thrombocytopenia, neutropenia, acute kidney injury, rhabdomyolysis, liver toxicity, cardiac ischemia, respiratory failure, hypoglycemia, blindness, and toxic epidermal necrolysis. One hundred-two (72%) reactions were caused by quinine pills; 28 (20%) by quinine-containing beverages; 12 (8%) by five other types of exposures. Excluding 41 patients who had only dermatologic reactions, 92 (91%) of 101 patients had required hospitalization for severe illness; 30 required renal replacement therapy; three died. Quinine, even with only minute exposure from common beverages, can cause severe adverse reactions involving multiple organ systems. In patients with acute, multi-system disorders of unknown origin, an adverse reaction to quinine should be considered.

  16. Diversity and severity of adverse reactions to quinine: A systematic review.

    Science.gov (United States)

    Liles, Nathan W; Page, Evaren E; Liles, Amber L; Vesely, Sara K; Raskob, Gary E; George, James N

    2016-05-01

    Quinine is a common cause of drug-induced thrombocytopenia and the most common cause of drug-induced thrombotic microangiopathy. Other quinine-induced systemic disorders have been described. To understand the complete clinical spectrum of adverse reactions to quinine we searched 11 databases for articles that provided sufficient data to allow evaluation of levels of evidence supporting a causal association with quinine. Three reviewers independently determined the levels of evidence, including both immune-mediated and toxic adverse reactions. The principal focus of this review was on acute, immune-mediated reactions. The source of quinine exposure, the involved organ systems, the severity of the adverse reactions, and patient outcomes were documented. One hundred-fourteen articles described 142 patients with definite or probable evidence for a causal association of quinine with acute, immune-mediated reactions. These reactions included chills, fever, hypotension, painful acral cyanosis, disseminated intravascular coagulation, hemolytic anemia, thrombocytopenia, neutropenia, acute kidney injury, rhabdomyolysis, liver toxicity, cardiac ischemia, respiratory failure, hypoglycemia, blindness, and toxic epidermal necrolysis. One hundred-two (72%) reactions were caused by quinine pills; 28 (20%) by quinine-containing beverages; 12 (8%) by five other types of exposures. Excluding 41 patients who had only dermatologic reactions, 92 (91%) of 101 patients had required hospitalization for severe illness; 30 required renal replacement therapy; three died. Quinine, even with only minute exposure from common beverages, can cause severe adverse reactions involving multiple organ systems. In patients with acute, multi-system disorders of unknown origin, an adverse reaction to quinine should be considered. PMID:26822544

  17. The R46Q, R131Q and R154H Polymorphs of Human DNA Glycosylase/β-Lyase hOgg1 Severely Distort the Active Site and DNA Recognition Site but do not Cause Unfolding†

    OpenAIRE

    Anderson, Peter C.; Daggett, Valerie

    2009-01-01

    Reactive oxygen species can cause widespread cellular damage, including base alterations and strand breaks in DNA. An array of DNA-repair enzymes constitutes an essential part of the line of defense that cells use against oxidative damage to the genome. A DNA glycosylase/β-lyase enzyme, Ogg1, scavenges the genome for 8-oxoguanine, a major mutagenic DNA adduct induced by reactive oxygen species, and catalyzes its excision and subsequent cleavage of the DNA phosphate backbone. Several polymorph...

  18. Clinical study on immune neutropenia%免疫性中性粒细胞减少症临床研究

    Institute of Scientific and Technical Information of China (English)

    银广悦; 王文红; 龚庆辉; 张继领

    2012-01-01

    Objective To explore the diagnosis and of immune neutopenia.Methods Clinical data of 150 patients with immune neutropenia were collected and analyzed.Results Of 150 patients with immune neutropenia,35 cases went male(23.3%),115 cases were female(76.7%),and male to female ratio was 1 ∶ 3.3.Based on the difference produced way of granulocytes associated antibody,all patients could be divided into alloimmune neonatal neutropenia 9 cases (6.0%),drug-induced immune neutropenia 28 cases (18.7 %),primary autoimmune neutropenia 22 cases(14.6%),and secondary autoimmune neutropenia 91 cases (60.7%).Secondary autoimmune neutropenia included 39 cases of systemic lupus erythematous,21 cases of rheumatoid arthritis,2 cases of immune hemolytic anemia accompany with neutropenia,6 cases of immune thrombocytopenia accompany with neutropenia,3 cases of autoimmune hepatitis,16 cases of autoimmune thyroiditis,and 4 cases of Sjogren's syndrome.Conclusion The majority of primary immune neutropenia are female,which is usually secondary to autoimmune diseases.The diagnosis of primary immune neutropenia is very complicated,which requires a long period of clinical observation and follow-up.The majority of granulocytes associated antibody is IgG in the autoimmune neutropenia patients.Yet sometimes two or three kinds of antibodies were increased as well.%目的 探讨免疫性粒细胞减少症的诊断及治疗.方法 收集150例住院及门诊免疫性中性粒细胞减少症患者临床资料.利用SPSS 13.0进行数据的统计分析.结果 150例患者中,男性35例(23.3%),女性115例(76.7%),男女之比为1∶3.3.依据中性粒细胞抗体产生方式不同分为新生儿同种免疫性中性粒细胞减少症9例(6.0%);药物性免疫粒细胞减少症28例(18.7%);原发性自身免疫性中性粒细胞减少症22例(14.6%);继发性免疫性粒细胞减少症91例(60.7%):包括系统性红斑狼疮39例、类风湿关节炎21例、免

  19. Febrile Neutropenia Risk Assessment and Granulocyte-Colony Stimulating Factor Support in Patients with Diffuse Large B Cell Lymphoma Receiving R-CHOP Regimens

    DEFF Research Database (Denmark)

    Salar, Antonio; Haioun, Corinne; Rossi, Francesca Gaia;

    2009-01-01

    BACKGROUND: ASCO and EORTC guidelines recommend granulocyte colony-stimulating factor (G-CSF) primary prophylaxis for cancer patients with a ≥20% overall risk of febrile neutropenia (FN), and to support delivery of dose-dense regimens. CHOP-like regimens (with rituximab [R]) are the current...... standard of care for the management of aggressive non-Hodgkin lymphoma (NHL), but they are often associated with significant myelosuppression. Neutropenic events, particularly febrile neutropenia (FN), can be life-threatening and may lead to dose delays or reductions that compromise the efficacy......-CSF primary prophylaxis. Across all cycles, 29% of R-CHOP-21 patients had an unplanned hospitalization, with neutropenia/FN being the main reason. Subsequently, 67% of patients achieved a relative dose intensity (RDI) of ≥90% of their planned treatment (with respect to cyclophosphamide, doxorubicin...

  20. Caracterización de pacientes pediátricos con neutropenia enviados a un hospital de referencia Characterization of neutropenic pediatric patients sent to a referral centre

    OpenAIRE

    Gustavo Adolfo Lazo-Páez; Oscar Porras

    2010-01-01

    Objetivo: La neutropenia es un motivo relativamente frecuente de referencia al Servicio de Inmunología y Reumatología Pediátrica del Hospital Nacional de Niños; el estudio pretende caracterizar los casos de neutropenia referidos a este Servicio en el periodo comprendido entre noviembre de 1988 y junio de 2008. Métodos: Se estudiaron 84 pacientes entre 0 y 12 años de edad, referidos entre el 6 de noviembre de 1988 y el 1 de junio de 2008. Se efectuó un análisis descriptivo global de las caract...

  1. Variation in Management of Fever and Neutropenia Among Pediatric Patients With Cancer: A Survey of Providers in Michigan.

    Science.gov (United States)

    Mueller, Emily L; Walkovich, Kelly J; Yanik, Gregory A; Clark, Sarah J

    2015-01-01

    Considerable variation in the management of fever and neutropenia (FN) exists, with factors associated with treatment variation not well described. An online survey of 90 pediatric cancer providers in Michigan was performed in Spring 2014. The survey frame was pediatric patients with cancer receiving treatment, with a Port-a-cath, who were clinically stable. Criteria for "Decreased" and "Increased" risk groups were defined by respondents. Survey questions addressed FN definitions, risk groups conceptualization, routine clinical practice, and management guidelines, in the context of risk groups and distance to treating institution. Fifty providers responded (56%); the majority defined a febrile event as temperature >38.3°C and/or 2 events >38.0°C within a 24-hour period. Neutropenia was defined as current or anticipated absolute neutrophil count (ANC) 2 hours away. Respondents were significantly more likely to have a "Decreased Risk" patient travel over 2 hours if they rated the local ED as "Poor to Fair" on ability to access Port-a-caths (P = .048). Most respondents would discharge patients who are afebrile for 24 hours, blood cultures negative for 48 hours, and neutrophil count of greater than 200/μL; 40% preferred discharge on oral antibiotics when the ANC febrile pediatric patients with cancer is significantly influenced by the providers' perceptions of local EDs. Future investigation of local hospitals' ability to provide urgent evaluation, combined with parental perspectives, could lead to improvements in timely and effective management.

  2. The efficiency of granulocyte colony-stimulating factor in hemorrhagic mucositis and febrile neutropenia resulted from methotrexate toxicity.

    Science.gov (United States)

    Ozkol, Hatice Uce; Toptas, Tayfur; Calka, Omer; Akdeniz, Necmettin

    2015-01-01

    Methotrexate (MTX) remains one of the most frequently used anti-metabolite agents in dermatology. MTX is an analog of folate that competitively and irreversibly inhibits dihydrofolate reductase. Oral mucositis is a common side effect of chemotherapy drugs and is characterized by erythema, pain, poor oral intake, pseudomembranous destruction, open ulceration and hemorrhage of the oral mucosa. In this paper, we report a 32-year-old female with a case of mucositis due to MTX intoxication that resulted from an overdose for rheumatoid arthritis. The patient had abdominal pain, vomiting, and nausea. During follow-up, the patient's white blood cell count was found to be 0.9 × 10(9)/L (4-10 × 10(9)/L). The patient developed fever exceeding 40 °C. The patient was consulted to the hematology service. They suggested using granulocyte colony-stimulating factor for febrile neutropenia. On the fifth day of treatment, the white blood cell count reached 5.3 × 10(9)/L and the patient's fever and mucositis started to resolve. Here, we presented a case of hemorrhagic mucositis and febrile neutropenia resulted from high-dose MTX that responded very well to granulocyte colony-stimulating factor treatment and we reviewed the literature.

  3. Association of severe myoclonic epilepsy of infancy (SMEI) with probable autoimmune lymphoproliferative syndrome-variant

    OpenAIRE

    A. Berio; MANGIANTE, G.; Piazzi, A.

    2014-01-01

    The paper reported on a case of severe myoclonic epilepsy of infancy (SMEI) associated with a probable autoimmune lymphoproliferative syndrome variant (Dianzani autoimmune lymphoproliferative disease) (DALD). A male patient with typical features of SMEI and a SCN1A gene variant presented in the first year of life with multiple lymph nodes, palpable liver at 2 cm from the costal margin, neutropenia, dysgammaglobulinemia, relative and sometimes absolute lymphocytosis. Subsequently the patient p...

  4. Effect of clinical medicine in lung infection caused by severe head trauma in infensive care unit%重症监护室重型颅脑损伤致肺部感染的临床用药分析

    Institute of Scientific and Technical Information of China (English)

    罗志建

    2015-01-01

    Objective:To investigate the clinical condition of pulmonary infection caused by severe head trauma infensive care unit, and observe its clinical manifestations and drug effects. Methods:The clinical diagnosis and treatment data of 110 cases with pulmonary infection caused by severe head trauma were retrospectively analyzed. Results:The incidence of pulmonary infection caused by severe head trauma was high. Eighty-eight strains of gram-negative bacteria(73. 3%),26 strains of gram-positive bacteria(21. 7%) and 6 strains of fungi (5. 0%) were found. The sensitivities of imipenem and vancomycin were the highest. All patients were alleviated after treatment. Conclusions:The gram-negative bacteria in pulmonary infection caused by severe head trauma infensive care unit is the major pathogenic bacteria. The patient is multiple drug resistance,the use of antibiotics should be strictly control.%目的::了解重症监护室( ICU)重型颅脑损伤致肺部感染的发生情况、临床表现及用药效果。方法:对110例ICU重型颅脑损伤出现肺部感染患者的临床诊治资料进行回顾性分析。结果:ICU重型颅脑损伤出现肺部感染的发生率较高,病原菌中革兰阴性菌88株,占73.3%,革兰阳性菌26株,占21.7%,真菌6株,占5.0%。革兰阴性菌和阳性菌分别以铜绿假单胞杆菌和金黄色葡萄球菌检出率最高,分别对头胞哌酮和万古霉素的敏感率最高。经治疗,所有病例均得到缓解。结论:ICU重型颅脑损伤致肺部感染的致病菌以革兰阴性菌为主,且多重耐药情况严重,须严格控制抗生素的使用。

  5. Plasma IL-8 and IL-6 levels can be used to define a group with low risk of septicaemia among cancer patients with fever and neutropenia

    NARCIS (Netherlands)

    de Bont, ESJM; Vellenga, E; Swaanenburg, JCJM; Fidler, [No Value; Visser-van Brummen, PJ; Kamps, WA

    1999-01-01

    The standard therapy for patients with fever and chemotherapy-related neutropenia is hospitalization and infusion of broad-spectrum antibiotics. Early discharge of a defined group of patients at low risk for septicaemia would be of great advantage for these patients. Ih this study plasma interleukin

  6. Cefepime Monotherapy is as Effective as Ceftriaxone Plus Amikacin in Pediatric Patients with Cancer and High-Risk Febrile Neutropenia: A Randomized Comparison

    Directory of Open Access Journals (Sweden)

    Carlos Alberto Pires Pereira

    2008-01-01

    Full Text Available The empirical use of antibiotic therapies is widely accepted in patients with fever and neutropenia during cancer chemotherapy. The use of intravenous monotherapy with broad-spectrum antibiotics in patients with high-risk of complications is an appropriate alternative. However, few data are available in pediatric patients. We conducted a prospective, randomized, open study in patients with lymphoma or leukemia who had fever and neutropenia during chemotherapy. Patients were randomized to receive cefepime (CFP or ceftriaxone plus amikacin (CFT+AK. A total of 57 patients with 125 episodes of fever and neutropenia were evaluated (CFP, 62 and CFT + AK, 63 episodes. The mean neutrophil count at admission was 118.6 cells mm-3 (CFP and 107 cells mm-3 (CFT+AK. The mean duration of neutropenia was 9.0 days (CFP and 8.0 days (CFT+AK. Analyzing only the first episodes of each patient, CFP treatment was successful in 65.5% of the episodes and CFT+AK were successful in 64.3%. Overall rates of success with modification were 90% (CFP and 89% (CFT+AK. No major treatment-emergent toxicity was reported. Monotherapy with CFP seems to be as effective and safe as the combination of CFT+AK for initial empirical therapy in children and adolescents with NF.

  7. Fever and neutropenia due to cytomegalovirus infection in a breast cancer patient under chemotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Ahmadinejad Z

    2010-06-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Fever in neutropenic patients is a medical emergency which may happen in patients undergoing chemotherapy. The definition of neutropenia varies from institution to institution but is usually defined as an absolute neutrophil count (ANC    < 500 cells/ml or < 1,000 cells/ml with a predicted nadir of < 500 cells/ml. Bacterial and fungal infections are the most important in neutropenic patients. Viral infections with agents such as herpes simplex virus and cytomegalovirus are common but less than other pathogens."n"nCase presentation: We report a patient with fever & neutropenia following cytomegalovirus infection during chemotherapy course for breast cancer."n"nConclusion: Although fever and neutropenia after cytomegalovirus infection is not very common but prompt diagnosis and treatment of this disease reduces the mortality and morbidity associated with cytomegalovirus. For this reason, screen testing for CMV infection in high risk patients including patients with cancer and preemptive therapy in patients with viremia, for prevention of CMV disease could be considered as a strategy for prevention of CMV infection.

  8. Research Progress in Hand-Foot-Mouth Disease Caused by Severe Enterovirus 71 Infection%重症肠道病毒71型感染手足口病研究进展

    Institute of Scientific and Technical Information of China (English)

    郭靖

    2015-01-01

    常见的手足口病由重症肠道病毒71型引起,并在世界范围内流行。为了更加科学地认识肠道病毒71型与手足口病的关系,该文就重症肠道病毒71型引起的手足口病的病原学、发病机制、临床症状、流行病学、预防与诊治等方面的研究进展进行了综述。%Common hand-foot-mouth disease is caused by the severe enterovirus 71, and is prevalent around the world. In order to more scientifically understand the relationship between enterovirus 71 and hand-foot-mouth disease, this paper conducts the detailed review on the research progress of the severe enterovirus 71 caused hand-foot-mouth disease in the aspects of the etiology, pathogenesis, clinical symptom, epidemiology, prevention and treatment.

  9. Quality of Life and Neutropenia in Patients with Early Stage Breast Cancer: A Randomized Pilot Study Comparing Additional Treatment with Mistletoe Extract to Chemotherapy Alone

    Directory of Open Access Journals (Sweden)

    Wilfried Tröger

    2009-01-01

    Full Text Available Background: Chemotherapy for breast cancer often deteriorates quality of life, augments fatigue, and induces neutropenia. Mistletoe preparations are frequently used by cancer patients in Central Europe. Physicians have reported better quality of life in breast cancer patients additionally treated with mistletoe preparations during chemotherapy. Mistletoe preparations also have immunostimulant properties and might therefore have protective effects against chemotherapy-induced neutropenia.Patients and Methods: We conducted a prospective randomized open label pilot study with 95 patients randomized into three groups. Two groups received Iscador® M special (IMS or a different mistletoe preparation, respectively, additionally to chemotherapy with six cycles of cyclophosphamide, adriamycin, and 5-fluoro-uracil (CAF. A control group received CAF with no additional therapy. Here we report the comparison IMS (n = 30 vs. control (n = 31. Quality of life including fatigue was assessed with the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC-QLQ-C30. Neutropenia was defined as neutrophil counts <1,000/µl and assessed at baseline and one day before each CAF cycle.Results: In the descriptive analysis all 15 scores of the EORTC-QLQ-C30 showed better quality of life in the IMS group compared to the control group. In 12 scores the differences were significant (p < 0.02 and nine scores showed a clinically relevant and significant difference of at least 5 points. Neutropenia occurred in 3/30 IMS patients and in 8/31 control patients (p = 0.182.Conclusions: This pilot study showed an improvement of quality of life by treating breast cancer patients with IMS additionally to CAF. CAF-induced neutropenia showed a trend to lower frequency in the IMS group.

  10. The role of carbon dioxide (and intracellular pH) in the pathomechanism of several mental disorders. Are the diseases of civilization caused by learnt behaviour, not the stress itself?

    Science.gov (United States)

    Sikter, András; Faludi, Gábor; Rihmer, Zoltán

    2009-09-01

    The role of carbon dioxide (CO2) is underestimated in the pathomechanism of neuropsychiatric disorders, though it is an important link between psyche and corpus. The actual spiritual status also influences respiration (we start breathing rarely, frequently, irregularly, etc.) causing pH alteration in the organism; on the other hand the actual cytosolic pH of neurons is one of the main modifiers of Ca2+-conductance, hence breathing directly, quickly, and effectively influences the second messenger system through Ca2+-currents. (Decreasing pCO2 turns pH into alkalic direction, augments psychic arousal, while increasing pCO2 turns pH acidic, diminishes arousal.) One of the most important homeostatic function is to maintain or restore the permanence of H+-concentration, hence the alteration of CO2 level starts cascades of contraregulation. However it can be proved that there is no perfect compensation, therefore compensational mechanisms may generate psychosomatic disorders causing secondary alterations in the "milieu interieur". Authors discuss the special physico-chemical features of CO2, the laws of interweaving alterations of pCO2 and catecholamine levels (their feedback mechanism), the role of acute and chronic hypocapnia in several hyperarousal disorders (delirium, panic disorder, hyperventilation syndrome, generalized anxiety disorder, bipolar disorder), the role of "locus minoris resistentiae" in the pathomechanism of psychosomatic disorders. It is supposed that the diseases of civilization are caused not by the stress itself but the lack of human instinctive reaction to it, and this would cause long-lasting CO2 alteration. Increased brain-pCO2, acidic cytosol pH and/or increased basal cytosolic Ca2+ level diminish inward Ca2+-current into cytosol, decrease arousal--they may cause dysthymia or depression. This state usually co-exists with ATP-deficiency and decreased cytosolic Mg2+ content. This energetical- and ion-constellation is also typical of ageing

  11. Ciprofloxacin versus colistin prophylaxis during neutropenia in acute myeloid leukemia: two parallel patient cohorts treated in a single center

    Science.gov (United States)

    Pohlen, Michele; Marx, Julia; Mellmann, Alexander; Becker, Karsten; Mesters, Rolf M.; Mikesch, Jan-Henrik; Schliemann, Christoph; Lenz, Georg; Müller-Tidow, Carsten; Büchner, Thomas; Krug, Utz; Stelljes, Matthias; Karch, Helge; Peters, Georg; Gerth, Hans U.; Görlich, Dennis; Berdel, Wolfgang E.

    2016-01-01

    Patients undergoing intensive chemotherapy for acute myeloid leukemia are at high risk for bacterial infections during therapy-related neutropenia. However, the use of specific antibiotic regimens for prophylaxis in afebrile neutropenic acute myeloid leukemia patients is controversial. We report a retrospective evaluation of 172 acute myeloid leukemia patients who received 322 courses of myelosuppressive chemotherapy and had an expected duration of neutropenia of more than seven days. The patients were allocated to antibiotic prophylaxis groups and treated with colistin or ciprofloxacin through 2 different hematologic services at our hospital, as available. The infection rate was reduced from 88.6% to 74.2% through antibiotic prophylaxis (vs. without prophylaxis; P=0.04). A comparison of both antibiotic drugs revealed a trend towards fewer infections associated with ciprofloxacin prophylaxis (69.2% vs. 79.5% in the colistin group; P=0.07), as determined by univariate analysis. This result was confirmed through multivariate analysis (OR: 0.475, 95%CI: 0.236–0.958; P=0.041). The prophylactic agents did not differ with regard to the microbiological findings (P=0.6, not significant). Of note, the use of ciprofloxacin was significantly associated with an increased rate of infections with pathogens that are resistant to the antibiotic used for prophylaxis (79.5% vs. 9.5% in the colistin group; P<0.0001). The risk factors for higher infection rates were the presence of a central venous catheter (P<0.0001), mucositis grade III/IV (P=0.0039), and induction/relapse courses (vs. consolidation; P<0.0001). In conclusion, ciprofloxacin prophylaxis appears to be of particular benefit during induction and relapse chemotherapy for acute myeloid leukemia. To prevent and control drug resistance, it may be safely replaced by colistin during consolidation cycles of acute myeloid leukemia therapy. PMID:27470601

  12. Neutropenia crónica e infección por el virus de la inmunodeficiencia humana

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    Ronald A Noguera-Valverde

    2008-09-01

    Full Text Available Se presenta el caso de un paciente masculino con neutropenia crónica e infección por el virus de inmunodeficiencia humana, con una revisión de los posibles mecanismos patogénicos. Las alteraciones hematológicas como anemia, trombocitopenia y leucopenia se presentan asociadas con frecuencia a la infección aguda por el virus de inmunodeficiencia humana. Al establecer la terapia antirretroviral y disminuir la actividad del virus, estas alteraciones tienden a mejorar. Sin embargo, algunos fármacos antirretrovirales, como la zidovudina, poseen toxicidad medular y pueden producir o empeorar las alteraciones hematológicas en estos pacientes, lo cual lleva a cambios en los esquemas de tratamiento. Los citotóxicos y antimetabolitos empleados en el tratamiento de neoplasias asociadas tienen conocida actividad depresora sobre la médula ósea. Algunos antimicrobianos utilizados en la profilaxis de infecciones poseen también toxicidad hematológica conocida, como el trimetoprim-sulfametoxazol, por lo que deben ser utilizados con precaución en pacientes con infección por el virus de inmunodeficiencia humana. Por otro lado, se plantean mecanismos alternativos que causan neutropenia en estos pacientes, como la formación de anticuerpos antineutrófilos, daño primario del progenitor granulocítico, por desbalance en la producción de neutrófilos, por anticuerpos contra la glicoproteína gp120 de la cápside viral del VIH, y deficiencias vitamínicas. En el caso del paciente neutropénico febril, en quien se sospecha infección bacteriana grave, se pueden utilizar los factores estimulantes de las colonias de granulocitos para aumentar los conteos absolutos de neutrófilos y mejorar la recuperación clínica.

  13. 成功抢救体内带毒致急性重度海洛因中毒5例%Successful rescue of 5 cases of acute severe heroin poisoning caused by drugs in vivo

    Institute of Scientific and Technical Information of China (English)

    高尚琪

    2014-01-01

    目的:分析研究因体内藏毒导致急性重度海洛因中毒,采用急救处理措施的临床救治效果。方法:近年来收治急性重度海洛因中毒患者5例,对其相关临床资料进行回顾性分析。结果:所有患者均通过手术取出毒品,并给予纳洛酮进行拮抗治疗。结论:对急性重度海洛因中毒采取相对应的急救措施,可以取得显著临床治疗效果。%Objective:To explore the clinical therapeutic effect of emergency treatment measures for patients of acute severe heroin poisoning caused by drugs in vivo.Methods:In recent years,5 patients with acute severe heroin poisoning were selected,the relevant clinical data were retrospectively analyzed.Results:The drugs in patients were all removed by operation,and patients were given naloxone antagonism treatment.Conclusion:Corresponding emergency measures adopted for acute severe heroin poisoning can achieve significant clinical effect,it is worth spreading in clinical practice.

  14. 血浆置换术抢救25例蜂毒致急性重度溶血性贫血临床观察%The clinical abservation of TPE rescue acute severe hemolysis caused by bee venom

    Institute of Scientific and Technical Information of China (English)

    陈劲松; 吴华新; 范萍

    2009-01-01

    目的:观察血浆置换术(TPE)治疗蜂毒致急性重度溶血性贫血的疗效.方法:对25例已被确诊为蜂毒致急性重度溶血性贫血患者,在应用糖皮质激素的基础上进行TPE治疗,并观察其治疗效果.结果:25例患者中,22例患者经1~2次TPE治疗后,中毒症状很快缓解,血氧饱和度上升到90%~99%,溶血基本控制,血红蛋白尿、高胆红素血症消失,降低了多脏器功能衰竭的发生率,有效率为88%,平均住院12.5 d.结论:TPE能快速有效地清除蜂毒及红细胞溶解产物,可阻断因溶血及蜂毒导致的脏器功能衰竭,是抢救蜂毒危重者的有效方法.%Objective:To observe the effect of the rapeutic plasma exchange in acute severe hemolysis which was caused by bee venom.Methods:25 patients were made a definite diagnosis to acute severe hemolysis which was caused by bee venom.Corticosteroid and plasma exchange were given to them.Results:Among 25 patients,22 patients who have undergone 1 to 2 times TPE.During the operation and end of TPE, the symptom of toxicosis remitted at once.The SaO2 rised to 90%- 99%.Hospitalization time was 12.5 days,hemolysis were controlled,hemoglobinnria and hyperbilirubinemia disappeared, the incidence of organ function failure decreased,effective rate was 88%.Conclusion:Plasma exchange can quickly remove the apitoxin and product of crythrolysis,TPE can bolck the organ function failure caused by hemolysis and apitoxin and is an effective method for rescue severe patients by bee venom.

  15. Analysis on death situation and causes of severe mental in remote mountainous areas%边远山区严重精神障碍患者三年死亡现状及原因分析

    Institute of Scientific and Technical Information of China (English)

    张腾; 王晖; 李明松; 杨家芳; 郑维斌

    2015-01-01

    [目的]分析边远山区严重精神障碍患者3年死亡现状及原因,为保山市提高该类患者社区居家生存质量提供科学依据。[方法]收集保山市社区2011-2013年内266例死亡的严重精神障碍患者信息和死亡原因,结合国家重性精神疾病基本数据收集分析系统进行统计学分析。[结果]2011-2013年,保山市共死亡严重精神障碍患者266例,3年平均死亡率13.8‰,2012年死亡率最高(为17.3‰),60岁及以上、离婚、文盲患者死亡率最高(分别为31.6‰、33.0‰和17.5‰);躯体疾病死亡率最高(9.8‰),具体死因中脑出血最高(为3.2‰);不同性别、年龄、民族、文化程度、婚姻状况的患者死亡状况不同。[结论]边远山区严重精神障碍患者死亡率(13.8‰)较普通人群高(6.7‰),以躯体疾病、自杀及意外为主要死亡原因,应加强社区对患者的管理、护理措施,提高该类患者生存质量。%Objective To provide scientific basis for improving life quality through analyzing death situation and causes in three years of severe mental in remote mountainous areas.Methods Collected 266 cases information death causes of severe mental from 2011 to 2013 in Baoshan. Statistic analysis combined with national severe mental basic data. Results From 2011 to 2013, 266 severe mental was died, 3-years average mortality rate was 13.8‰, the highest of mortality rate was in 2012. The highest of mortality rate in aged 60 above or divorced or illiterate patients( 31.6‰、33.0‰、17.5‰).The mortality rate of physical illness was highest to 9.8‰, 3.2‰ was died in hematencephaion. The death status was different in different sex, age, nationality, education and marital status. Conclusions The mortality rate of severe mental(13.8‰) was higher than normal population(6.7‰) in remote mountainous areas. Main death causes was physical illness, suicide and accidents. So we must

  16. Study on cause and risky factor of hysterectomy due to severe postpartum hemorrhage%严重产后出血致子宫切除原因及危险因素分析

    Institute of Scientific and Technical Information of China (English)

    杨艳华; 梁秀坤

    2015-01-01

    目的:探讨严重产后出血( postpartum hemorrhage,PPH)致子宫切除的原因和危险因素。方法将2004年10月至2014年10月在天门市第一人民医院妇产科因严重PPH行子宫切除术31例患者纳入观察组,同期62例PPH但未达到子宫切除指征产妇为对照组。分析两组产妇临床资料和难治性PPH致子宫切除的危险因素。结果31例子宫切除原因为凝血功能障碍14例(45.2%),胎盘因素11例(35.5%),宫缩乏力5例(16.1%),贫血与血小板减少1例(3.2%),观察组中剖宫产与自然分娩原因构成有差异(P<0.05),观察组与对照组构成有差异(P<0.05)。Logistic回归分析显示剖宫产(OR=1.883,95% CI:1.177~1.980)、多胎妊娠(OR=2.587,95% CI:1.718~4.882)、巨大儿(OR=1.438,95% CI:1.280~2.736)是独立影响因素。结论严重PPH子宫切除常见病因为凝血功能障碍、胎盘因素和宫缩乏力;不同分娩方式出血原因构成不同;剖宫产、巨大儿和多胎妊娠是危险因素。%Objective To study the cause and risk factors of hysterectomy due to severe postpartum hemorrhage ( PPH ) . Methods The 31 cases with hysterectomy due to severe PPH in Tianmen First People 's Hospital from Oct 2004 to Oct 2014 were chosen as observation group, 62 cases with PPH who did not reach the hysterectomy indications in the same period were selected as control group. Clinical data and risk factors of hysterectomy due to severe PPH were analyzed. Results Reasons of hysterectomy of the 31 cases included:14 cases (45. 2%) were coagulation disorder, 11 cases (35. 5%) were placental factor, 5 cases (16. 1%) were uterine inertia, 1 case (3. 2 %) was anemia. There were significant differences in hemorrhage reasons between caesarean section and spontaneous labor in observation group( P<0. 05 ) , and there was a significant difference in hemorrhage reason between observation group and control group(P<0. 05). Logistic aggression revealed caesarean

  17. Discussion about traditional Chinese medicine causes and pathogenesis of moderate and severe primary dysmenorrhea based on questionnaire investigation%基于调查的中重度原发性痛经中医病因病机探讨

    Institute of Scientific and Technical Information of China (English)

    刘玉祁; 解秸萍; 苑鸿雯; 佘延芬; 朱江; 宋佳杉; 陈寅萤; 郑媛媛; 马增斌; 陈旭; 杨欢

    2012-01-01

    目的:对女大学生进行问卷调查以探讨中重度原发性痛经的中医病因病机.方法:以中重度原发性痛经女大学生为调查对象,调查其平时、痛经及证型分布情况并进行描述性统计分析.结果:163例被调查者中,有69.94%平时较怕冷,57.06%易手脚冰冷,77.30%喜热饮热食,83.44%每周运动少于3次.78.53%、64.42%的患者因吃冰品冷饮或用冷水洗衣服引起或加重腹痛,66.26%、74.23%因吃热食热饮或热敷腹部缓解腹痛.腹痛时69.33%的患者排便次数增加且大便变稀软;61.35%、56.44%左侧或右侧尺脉沉.68.10%的患者被诊断为寒湿凝滞证.结论:调查发现中重度原发性痛经的中医病因为寒邪,病机以外感寒邪而致寒湿凝滞胞宫为主.%Objective: To discuss traditional Chinese medicine (TCM) causes and pathogenesis of moderate and severe primary dysmenorrheal (PD) through questionnaire investigation among female college students. Methods: Female college students with moderate and severe PD were investigated. The ordinary times status, menstrual period conditions and pattern distribution of these patients were surveyed and analyzed by descriptive statistics. Results: Of all the 163 female college students with moderate and severe PD who finishied this investigation, at ordinary times, 69.94% averted to cold, 57.06% had cold limbs, 77.30% preferred warm food and drinks and 83.44% did physical activities less than 3 times a week. There were 78.53% and 64.42% patients whose menstrual pain were caused or aggravated by taking cold food and drinks or by washing clothes with cold water. While there were 66.26% patients whose menstrual pain were relieved by eating warm food and drinks and 74.23% by applying hot compress on lower abdomen. When menstrual pain occurred, 69.33% patients had more motions and more watery, soft stools than usual and 61.35% and 56.44% had sunken pulse in the left and right Chi position respectively

  18. Cortical synaptic transmission in CaV2.1 knockin mice with the S218L missense mutation which causes a severe familial hemiplegic migraine syndrome in humans.

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    Dania eVecchia

    2015-02-01

    Full Text Available Familial hemiplegic migraine type 1 (FHM1 is caused by gain-of-function mutations in CaV2.1 (P/Q-type Ca2+ channels. Knockin (KI mice carrying the FHM1 R192Q missense mutation show enhanced cortical excitatory synaptic transmission at pyramidal cell synapses but unaltered cortical inhibitory neurotransmission at fast-spiking interneuron synapses. Enhanced cortical glutamate release was shown to cause the facilitation of cortical spreading depression (CSD in R192Q KI mice. It, however, remains unknown how other FHM1 mutations affect cortical synaptic transmission. Here, we studied neurotransmission in cortical neurons in microculture from KI mice carrying the S218L mutation, which causes a severe FHM syndrome in humans and an allele-dosage dependent facilitation of experimental CSD in KI mice, which is larger than that caused by the R192Q mutation. We show gain-of-function of excitatory neurotransmission, due to increased action-potential evoked Ca2+ influx and increased probability of glutamate release at pyramidal cell synapses, but unaltered inhibitory neurotransmission at multipolar interneuron synapses in S218L KI mice. In contrast with the larger gain-of-function of neuronal CaV2.1 current in homozygous than heterozygous S218L KI mice, the gain-of-function of evoked glutamate release, the paired-pulse ratio and the Ca2+ dependence of the EPSC were all similar in homozygous and heterozygous S218L KI mice, suggesting compensatory changes in the homozygous mice. Furthermore, we reveal a unique feature of S218L KI cortical synapses which is the presence of a fraction of mutant CaV2.1 channels being open at resting potential. Our data suggest that, while the gain-of-function of evoked glutamate release may explain the facilitation of CSD in heterozygous S218L KI mice, the further facilitation of CSD in homozygous S218L KI mice is due to other CaV2.1-dependent mechanisms, that likely include Ca2+ influx at voltages sub-threshold for action

  19. Abnormal cortical synaptic transmission in CaV2.1 knockin mice with the S218L missense mutation which causes a severe familial hemiplegic migraine syndrome in humans.

    Science.gov (United States)

    Vecchia, Dania; Tottene, Angelita; van den Maagdenberg, Arn M J M; Pietrobon, Daniela

    2015-01-01

    Familial hemiplegic migraine type 1 (FHM1) is caused by gain-of-function mutations in CaV2.1 (P/Q-type) Ca(2+) channels. Knockin (KI) mice carrying the FHM1 R192Q missense mutation show enhanced cortical excitatory synaptic transmission at pyramidal cell synapses but unaltered cortical inhibitory neurotransmission at fast-spiking interneuron synapses. Enhanced cortical glutamate release was shown to cause the facilitation of cortical spreading depression (CSD) in R192Q KI mice. It, however, remains unknown how other FHM1 mutations affect cortical synaptic transmission. Here, we studied neurotransmission in cortical neurons in microculture from KI mice carrying the S218L mutation, which causes a severe FHM syndrome in humans and an allele-dosage dependent facilitation of experimental CSD in KI mice, which is larger than that caused by the R192Q mutation. We show gain-of-function of excitatory neurotransmission, due to increased action-potential evoked Ca(2+) influx and increased probability of glutamate release at pyramidal cell synapses, but unaltered inhibitory neurotransmission at multipolar interneuron synapses in S218L KI mice. In contrast with the larger gain-of-function of neuronal CaV2.1 current in homozygous than heterozygous S218L KI mice, the gain-of-function of evoked glutamate release, the paired-pulse ratio and the Ca(2+) dependence of the excitatory postsynaptic current were similar in homozygous and heterozygous S218L KI mice, suggesting compensatory changes in the homozygous mice. Furthermore, we reveal a unique feature of S218L KI cortical synapses which is the presence of a fraction of mutant CaV2.1 channels being open at resting potential. Our data suggest that, while the gain-of-function of evoked glutamate release may explain the facilitation of CSD in heterozygous S218L KI mice, the further facilitation of CSD in homozygous S218L KI mice is due to other CaV2.1-dependent mechanisms, that likely include Ca(2+) influx at voltages sub

  20. The study on the causes of Early death and the pireoperative management of cardiac valve replacement in patients with severe diseases%危重心脏瓣膜置换早期死亡原因及对策

    Institute of Scientific and Technical Information of China (English)

    李鲁; 马黎明; 褚衍林; 林毅; 程前进; 王志鸿; 李祥

    2012-01-01

    Objective To study the causes of early death and peri-operative management of cardiac valve replacement in patients with severe diseases. Methods Valve replacements were performed in 420 cases with severe cardiac valve diseases from April 1996 to July 2009. There were 202 male and 218 female, with an age ranged from 12 to 78 years. Preoperative heart function (NYHA) in 92 cases were chass III and 328 cases were in class IV, Mitral valve replacement were performed in 237 cases, Aortic replacement in 20, both mitral and aortic in 142, three valves with mitral, aortic and tricuspid in 15, and bicuspid in 6. 569 mechanical valves and 23 bioprostheses were implanted. Associated procedures were tricuspid annuloplasty in 242 cases, left atrium thrombectomy in 62, annuloplasty in 42, coronary artery bypass grafting in 52 cases, closing of patent ductus arteriosus transpuhnonary artery in 14,repair of ventricular septal defect in 6, and repair of aneurysm of the sinus of valsalva in 12, and repair of unroofed coronary sinus and surgical interruption of the Bundle of Kent in patient with W-P-W syndrome in 2 respectively. Results Thirteen(3.09%) patients were defined as early deaths. Low cardiac output, ventricular fibrillation, respiratory failure and multiple organ failure were the most frequent causes of mortality. Conclusion According to characteristics of severe patients, paying attention In preoperative preparation, selecting the most appropriate operative chance and correcting pathological changes, avoiding operative complications, and strengthening management of postoperative complication may have an excellent results in severe patients.%目的 探讨危重心脏瓣膜病手术早期死亡因素及对策.方法 总结420例危重心脏瓣膜病施行瓣膜置换的患者的临床资料,男202例,女218例,年龄(40.0±8.7)岁.术前心功能Ⅲ级92例,Ⅳ级328例.二尖瓣置换237例,主动脉瓣置换20例;二尖瓣+主动脉瓣置换142例;二尖瓣置换+

  1. Efficacy and safety of tazobactam/piperacillin as an empirical treatment for the patients of adult and child with febrile neutropenia in Japan.

    Science.gov (United States)

    Tamura, Kazuo; Akiyama, Nobu; Kanda, Yoshinobu; Saito, Masahiro

    2015-09-01

    Tazobactam/piperacillin (4.5 g for adults and 90 mg/kg body weight for children, every 6 h) was administered to Japanese patients with febrile neutropenia to evaluate its defervescence and clinical efficacy and safety. The pharmacokinetics in children were also examined. Defervescence efficacy at day 4 of the treatment was achieved in 50.0% of 94 adult and 62.5% of 8 pediatric patients, respectively. The defervescence efficacy rate in relation to the neutrophil count in adults was 37.5% for the patients with a neutrophil count of less than 100/μL and 62.5% for that between 100 and 500/μL. The clinical efficacy rate at day 7 and at the end or discontinuation of the treatment was 79.6% and 59.1% in adult patients, respectively, and 57.1% and 75.0% in pediatric patients, respectively. Fifteen strains of causative bacteria were isolated in 13 adult patients at baseline. All strains were eradicated within 4 days of the treatment. The side effects that occurred in adult and pediatric patients during the treatment were all known and not specific to febrile neutropenia patients. The pharmacokinetics profiles of tazobactam/piperacillin in children with febrile neutropenia are unlikely to be different from those in children with a common bacterial infection and without any immunosuppressive conditions. The study results in Japanese patients with febrile neutropenia demonstrate that tazobactam/piperacillin treatment is efficacious and safe in adults. As for pediatric patients, given the limited number of cases studied, further investigation is needed (Clinical trial number: Japic CTI-121728).

  2. Evaluation of ticarcillin/clavulanic acid versus ceftriaxone plus amikacin for fever and neutropenia in pediatric patients with leukemia and lymphoma

    Directory of Open Access Journals (Sweden)

    Petrilli Antonio Sérgio

    2003-01-01

    Full Text Available BACKGROUND: The empirical use of antibiotic treatments is widely accepted as a means to treat cancer patients in chemotherapy who have fever and neutropenia. Intravenous monotherapy, with broad spectrum antibiotics, of patients with a high risk of complications is a possible alternative. METHODS: We conducted a prospective open-label, randomized study of patients with lymphoma or leukemia who had fever and neutropenia during chemotherapy. Patients received either monotherapy with ticarcillin/clavulanic acid (T or ceftriaxone plus amikacin (C+A. RESULTS: Seventy patients who presented 136 episodes were evaluated, 68 in each arm of the study. The mean neutrophil counts at admission were 217cells/mm³ (T and 201cells/mm³ (C+A. The mean duration of neutropenia was 8.7 days (T and 7.6 days (C+A. Treatment was successful without the need for modifications in 71% of the episodes in the T group and 81% in the C+A group (p=0.23. Treatment was considered to have failed because of death in two episodes (3% in the T group and three episodes (4% in the C+A group, and because of a change in the drug applied in one episode in the T group and two episodes in the C+A group. Overall success was 96% (T and 93% (C+A. Adverse events that occurred in group T were not related to the drugs used in this study. CONCLUSION: In pediatric and adolescent patients with leukemia or lymphoma, who presented with fever and neutropenia, during chemotherapy, ticarcillin/clavulanic acid was as successful as the combination of ceftriaxone plus amikacin. It should be considered an appropriate option for this group of patients at high risk for infections.

  3. Novel C16orf57 mutations in patients with Poikiloderma with Neutropenia: bioinformatic analysis of the protein and predicted effects of all reported mutations

    Directory of Open Access Journals (Sweden)

    Colombo Elisa A

    2012-01-01

    Full Text Available Abstract Background Poikiloderma with Neutropenia (PN is a rare autosomal recessive genodermatosis caused by C16orf57 mutations. To date 17 mutations have been identified in 31 PN patients. Results We characterize six PN patients expanding the clinical phenotype of the syndrome and the mutational repertoire of the gene. We detect the two novel C16orf57 mutations, c.232C>T and c.265+2T>G, as well as the already reported c.179delC, c.531delA and c.693+1G>T mutations. cDNA analysis evidences the presence of aberrant transcripts, and bioinformatic prediction of C16orf57 protein structure gauges the mutations effects on the folded protein chain. Computational analysis of the C16orf57 protein shows two conserved H-X-S/T-X tetrapeptide motifs marking the active site of a two-fold pseudosymmetric structure recalling the 2H phosphoesterase superfamily. Based on this model C16orf57 is likely a 2H-active site enzyme functioning in RNA processing, as a presumptive RNA ligase. According to bioinformatic prediction, all known C16orf57 mutations, including the novel mutations herein described, impair the protein structure by either removing one or both tetrapeptide motifs or by destroying the symmetry of the native folding. Finally, we analyse the geographical distribution of the recurrent mutations that depicts clusters featuring a founder effect. Conclusions In cohorts of patients clinically affected by genodermatoses with overlapping symptoms, the molecular screening of C16orf57 gene seems the proper way to address the correct diagnosis of PN, enabling the syndrome-specific oncosurveillance. The bioinformatic prediction of the C16orf57 protein structure denotes a very basic enzymatic function consistent with a housekeeping function. Detection of aberrant transcripts, also in cells from PN patients carrying early truncated mutations, suggests they might be translatable. Tissue-specific sensitivity to the lack of functionally correct protein accounts for the

  4. Meta-Analysis and Cost Comparison of Empirical versus Pre-Emptive Antifungal Strategies in Hematologic Malignancy Patients with High-Risk Febrile Neutropenia.

    Directory of Open Access Journals (Sweden)

    Monica Fung

    Full Text Available Invasive fungal disease (IFD causes significant morbidity and mortality in hematologic malignancy patients with high-risk febrile neutropenia (FN. These patients therefore often receive empirical antifungal therapy. Diagnostic test-guided pre-emptive antifungal therapy has been evaluated as an alternative treatment strategy in these patients.We conducted an electronic search for literature comparing empirical versus pre-emptive antifungal strategies in FN among adult hematologic malignancy patients. We systematically reviewed 9 studies, including randomized-controlled trials, cohort studies, and feasibility studies. Random and fixed-effect models were used to generate pooled relative risk estimates of IFD detection, IFD-related mortality, overall mortality, and rates and duration of antifungal therapy. Heterogeneity was measured via Cochran's Q test, I2 statistic, and between study τ2. Incorporating these parameters and direct costs of drugs and diagnostic testing, we constructed a comparative costing model for the two strategies. We conducted probabilistic sensitivity analysis on pooled estimates and one-way sensitivity analyses on other key parameters with uncertain estimates.Nine published studies met inclusion criteria. Compared to empirical antifungal therapy, pre-emptive strategies were associated with significantly lower antifungal exposure (RR 0.48, 95% CI 0.27-0.85 and duration without an increase in IFD-related mortality (RR 0.82, 95% CI 0.36-1.87 or overall mortality (RR 0.95, 95% CI 0.46-1.99. The pre-emptive strategy cost $324 less (95% credible interval -$291.88 to $418.65 pre-emptive compared to empirical than the empirical approach per FN episode. However, the cost difference was influenced by relatively small changes in costs of antifungal therapy and diagnostic testing.Compared to empirical antifungal therapy, pre-emptive antifungal therapy in patients with high-risk FN may decrease antifungal use without increasing mortality

  5. The diagnostic value of soluble urokinase plasminogen activator receptor compared with C-reactive protein and procalcitonin in children with febrile neutropenia.

    Science.gov (United States)

    Sirinoglu, Melis; Soysal, Ahmet; Karaaslan, Ayşe; Kepenekli Kadayifci, Eda; Cinel, Ismail; Koç, Ahmet; Tokuç, Gülnur; Yaman, Ali; Haklar, Goncagül; Şirikçi, Önder; Turan, Serap; Altınkanat Gelmez, Gülşen; Söyletir, Güner; Bakır, Mustafa

    2016-04-01

    The aim of the present study was to determine the diagnostic value of soluble urokinase plasminogen activator receptor (suPAR) in pediatric patients with febrile neutropenia. A prospective case-control study was performed. Patients included 29 children with febrile neutropenia (FN) and 27 control subjects without any infection or immunosuppressive condition. Blood samples were obtained on the day of admission and on the 4th to 7th days of the hospital stay. The median (minimum-maximum) serum levels of suPAR obtained on the first day of the admission were 2.08 (0.93-9.42) and 2.22 (1.08-5.13) ng/mL for the FN group and the control group, respectively. The median serum levels of suPAR in the FN and control groups were not significantly different (P = .053). The mean serum suPAR level was significantly higher in nonsurvivors than in survivors in the FN group (P < .05). In the FN group, the area under the receiver operating characteristics curve (AUCROC) for suPAR was 0.546, but no optimum cutoff value, sensitivity, specificity, negative predictive value (NPV), or positive predictive value (PPV) was obtained. We conclude that suPAR is not useful as a diagnostic biomarker in children with febrile neutropenia; however, persistent high serum suPAR level may predict mortality in FN in children. PMID:27057782

  6. The diagnostic value of soluble urokinase plasminogen activator receptor compared with C-reactive protein and procalcitonin in children with febrile neutropenia.

    Science.gov (United States)

    Sirinoglu, Melis; Soysal, Ahmet; Karaaslan, Ayşe; Kepenekli Kadayifci, Eda; Cinel, Ismail; Koç, Ahmet; Tokuç, Gülnur; Yaman, Ali; Haklar, Goncagül; Şirikçi, Önder; Turan, Serap; Altınkanat Gelmez, Gülşen; Söyletir, Güner; Bakır, Mustafa

    2016-04-01

    The aim of the present study was to determine the diagnostic value of soluble urokinase plasminogen activator receptor (suPAR) in pediatric patients with febrile neutropenia. A prospective case-control study was performed. Patients included 29 children with febrile neutropenia (FN) and 27 control subjects without any infection or immunosuppressive condition. Blood samples were obtained on the day of admission and on the 4th to 7th days of the hospital stay. The median (minimum-maximum) serum levels of suPAR obtained on the first day of the admission were 2.08 (0.93-9.42) and 2.22 (1.08-5.13) ng/mL for the FN group and the control group, respectively. The median serum levels of suPAR in the FN and control groups were not significantly different (P = .053). The mean serum suPAR level was significantly higher in nonsurvivors than in survivors in the FN group (P febrile neutropenia; however, persistent high serum suPAR level may predict mortality in FN in children.

  7. What Causes Cystic Fibrosis?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Causes Cystic Fibrosis? A defect in the CFTR gene causes cystic ... in the severity of the disease. How Is Cystic Fibrosis Inherited? Every person inherits two CFTR genes—one ...

  8. Enteral nutrition support and nursing to batched severe burn patients caused by explosions%成批爆炸致特重度烧伤患者肠内营养支持的护理

    Institute of Scientific and Technical Information of China (English)

    王静云; 李小珍

    2015-01-01

    目的:探讨成批爆炸致特重度烧伤患者肠内营养支持护理要点。方法对本科室收治的15例特重度烧伤患者进行肠内营养支持治疗,并配合做好护理工作。结果5例患者伤后45~87 d因缺少自体皮源,并发脓毒血症死亡,10例患者经过肠内营养支持、抗感染等治疗护理,营养状况得到改善,安全度过感染期,进入恢复期;患者ICU住院时间54~115 d,平均(90.63±30.37)d。结论特重度烧伤患者采取早期肠内营养支持及护理,能够有效改善患者营养状况,促进患者康复。营养支持期间应做好管饲护理,预防早期肠内营养发生的并发症等,其是顺利完成肠内营养支持的重要措施。%Objective To explore nursing points on the enteral nutrition support to batched patients with severe burn caused by explosion. Method An enteral nutrition support was carried out for 15 patients with severe burns admitted in our department and pertinent nursing was performed. Results Five patients died because of lack of autologous skin sources and complications of sepsis in 45~87 d after burn. Ten patients got a good recovery after enteral nutrition support, anti-infection treatment and nursing. They lived through the infection period, went into the recovery period. The hospital stay of these patients in ICU was 54~115 d with an average of (90.63 ± 30.37)d. Conclusion Early enteral nutrition support and nursing to the patients with severe burn can improve the nutritional status of patients effectively and promote the rehabilitation of patients.

  9. Analysis of 73 Cases of Severe Adverse Drug Reactions Caused by Cephalosporin%73例头孢菌素类药物严重不良反应分析

    Institute of Scientific and Technical Information of China (English)

    吕冬莲; 龙萍; 吴方建

    2012-01-01

    目的:了解头孢菌素类药物引起的严重不良反应(SADR)的发生规律,发掘药物警戒信号,为临床安全合理用药提供参考.方法:收集武汉市不良反应监测系统数据库中2009~2011年头孢菌素类严重的(包括新的严重的)不良反应报告,从中筛选出符合标准的73例进行分析.结果:73例SADR涉及头孢菌素19个品种,其中以头孢曲松的比例最高;临床表现以过敏性休克和过敏样反应为主的全身性损害最为突出;41例SADR发生时间在30 min内;50岁以上的老年人及10岁以下儿童ADR构成比较高.从ADR病例报告中反映存在相当数量的不合理用药现象.结论:临床应高度重视头孢菌素类药物引起的过敏性休克等SADR,增强合理用药意识,以减少和避免SADR的发生.%Ohjective:To realize the regularity of severe adverse drug reaction(SADR) of cephalosporin, exploring the signal of drug alert, and providing the reference of rational use of drugs for clinical safety. Methods; Analyzed 73 oases which were up to standard from severe( including new severe) adverse reaction reports of cephalosporin from 2009 to 2011 in Wuhan adverse reaction monitoring system database. Results:'The SADR involved 19 kinds of cephalosporin, and the highest rercenlage was ceftriaxone Clinical perfor mance based on the principle of anaphylactoid reaction and anaphylac-tic shock. 41 cases SADRs appeared wilhin 30 minutes after administration, the constituent ratios of elder above 50 years old and the children under the age of 10 were a relatively high. And the reports of the SADR cases reflected a number of irrational drug use phenomenon. Conclusion: Clinical should pay high attention to anaphylactic shock and other SADRs caused by cephalosporin. To enhance the consciousness of rational drug use, in order lo reduce and avoid the happening of SADRs.

  10. 370例重症患者医院感染病原菌调查与分析%Pathogenic microorganism causing nosocomial infections in 370 cases of sever patients: investigation and analysis

    Institute of Scientific and Technical Information of China (English)

    徐丽丽; 侯彩妍; 吴琼; 范静; 翟红岩; 王国权

    2011-01-01

    目的 了解重症患者医院感染病原菌及医院感染情况.方法 对370例住院重症患者采集痰、咽拭子、血液、拔除的大静脉导管、尿、粪便、胸水及穿刺液标本,进行细菌培养,并进行分析.结果 采集的标本细菌培养阳性率为34.43%,最高阳性率的标本为痰液,阳性率为43.53%,其次为穿刺液和尿;痰培养主要的菌群为白色假丝酵母菌、铜绿假单胞菌和鲍氏不动杆菌等;尿培养主要的菌群为白色假丝酵母菌、肺炎克雷伯菌和大肠埃希菌等;血培养主要菌群为铜绿假单胞菌、肺炎克雷伯菌和大肠埃希菌等.结论 重症患者医院感染的首位是呼吸道感染;居高位是尿路感染;高发率是血液感染.%OBJECTIVE To evaluate pathogenic microorganism causing nosocomial infections in severe patients and status of hospital infection in our hospital. METHODS Phlegm, throat swabs, blood, pulling out of filling a catheter, urine, feces, chest water and biopsy specimens were collected from 370 cases of severe patients, then the bacterial culture was analyzed. RESULTS The germiculture positive rate of the specimens was 34. 43%; The highest rate in sputum specimens was 43. 53 %, followed by puncture fluid and urine. The main microbes of sputum culture were Candida albicans, Pseudomonas aeruginosa and Acinetobacter baumannii. The main microbes of urine culture were C. albicans, Klebsiella pneumonia and Escherichia coli, and so on. The main microbes of blood culture were P. aeruginosa, K. pneumonia and E. coli etc. CONCLUSIONS Respiratory tract infection is the first hospital infections in severe patients; the higher isurinary infection; the higher incidences is blood infection.

  11. The causes and prevention methods of severe burn patients complicated with stress ulcer bleeding%重度烧伤并发应激性溃疡出血的原因及防治

    Institute of Scientific and Technical Information of China (English)

    李峥; 张兵; 李巍; 王强

    2014-01-01

    目的:探讨重度烧伤并发应激性溃疡出血发生的原因及防治方法。方法我院2008年1月至2013年12月共收治892例重度烧伤患者,回顾分析其中53例并发应激性溃疡出血患者的临床资料。结果重度烧伤并发应激性溃疡出血的53例患者中,经积极治疗,显效24例,有效22例,总有效率为87%;休克患者出血发生率明显高于无休克患者(χ2=138.20,P<0.05),应激性溃疡出血与休克之间存在较强关联性(χ2=62.82,P<0.05,r=0.65)。结论对于存在休克的重度烧伤患者需提高警惕,注意监测有无应激性溃疡出血发生,早期行综合治疗,防治休克是预防应激性溃疡出血的有利措施。%Objective To study the causes and prevention methods of severe burn patients complicated with stress ulcer bleed -ing.Methods From January 2001 to December 2012 , a total of 892 cases of severe burn patients were treated in our hospital .Clinical data of 53 cases complicated with stress ulcer bleeding were retrospectively analyzed .Results Of the 53 patients, therapy was marked-ly effective in 24 cases and effective in 22 patients.The total effective rate was 87%.The incidence of bleeding in shock patients was significantly higher than that in non-shock patients (χ2=138.20 , P<0.05 ) .There was a significant correlation between shock and stress ulcer bleeding (χ2=62.82 ,P<0.05 ,r=0.65 ) .Conclusion It should be wary of severe burn patients with shock to monitor the presence of stress ulcer hemorrhage , so that to carry out early comprehensive treatment .The prevention and treatment of shock are fa-vorable measures for stress ulcer bleeding .

  12. Malnutrition and neutropenia in children treated for Burkitt Lymphoma in Malawi

    NARCIS (Netherlands)

    T. Israëls; M.D. van de Wetering; P. Hesseling; N. van Geloven; H.N. Caron; E.M. Molyneux

    2009-01-01

    Background. infection in neutropenic children is a major cause of morbidity and mortality in children treated for cancer. In developing countries, children with cancer are often malnourished at diagnosis. In Blantyre, Malawi, children with Burkitt lymphoma are treated with a local protocol with limi

  13. 严重肝病伴糖尿病患者大肠埃希菌血流感染临床分析%Clinical analysis of bloodstream infections caused by Escherichia coli in severe liver diseases compared with diabetes

    Institute of Scientific and Technical Information of China (English)

    何卫平; 崔恩博; 蔡少平; 鲍春梅; 张文瑾; 范振平; 曲芬

    2014-01-01

    Objective To investigate the clinical characteristic and drug resistance of bloodstream infections caused by Escherichia coli (E.coli)in patients with severe liver diseases and diabetes,and to provide evidence for optimal therapy of antibiotics in clinical practice. Methods The clinical features and drug susceptibility of E.coli-caused bloodstream infections among inpatients with severe liver diseases and diabetes from 2009 to 2012 were analyzed retrospectively. Results A total of 47 strains of E. coli were isolated from those inpatients,among which 22 strains of extended-spectrumβ-lactamases (ESBLs)(46.81% )were positive. The overwhelming majority patients were liver cirrhosis with infection resulting from spontaneous bacterial peritonitis. The drug resistance of ESBLs-positive strains was higher than that of ESBLs-negative strains,with no statistical differences existed in age,sex,basic disease,infection source,peak temperature,white blood cell count and the percentage of neutrophils between ESBLs-positive strains and negative strains. Mortality rate in patients accompanied with septic shock was higher than that in patients without shock. Conclusion Due to the severity and bad prognosis of bloodstream infection,early identification and comprehensive treatment should be taken to reduce mortality.%目的:分析严重肝病伴糖尿病患者大肠埃希菌血流感染的临床特点及耐药性,为临床合理应用抗菌药物提供依据。方法回顾性分析2009年至2012年住院的严重肝病合并糖尿病血流感染大肠埃希菌患者的临床特点及药敏结果。结果严重肝病合并糖尿病血流感染大肠埃希菌患者共47例,基础疾病以肝硬化为主,感染来源以自发性细菌性腹膜炎为主,ESBL阳性22株,阳性率46.81%。ESBL阳性大肠埃希菌耐药性高于 ESBL 阴性大肠埃希菌,但两者在年龄、性别、基础疾病、原发病灶、体温峰值、白细胞计数及中性粒细胞百分比

  14. A comparative study of severe scorpion envenomation in children caused by Tityus bahiensis and Tityus serrulatus Estudo comparativo do envenenamento escorpiônico grave em crianças provocado por Tityus bahiensis e Tityus serrulatus

    Directory of Open Access Journals (Sweden)

    Fábio Bucaretchi

    1995-08-01

    Full Text Available From January 1984 to May 1994, 17 of 239 children under 15 years old stung by Tityus serrulatus (15.1% or Tityus bahiensis (84.9% presented severe envenoming. Of these 17 patients (1-11 years old; median=2 yr 14 were stung by T.serrulatus and three by T.bahiensis. All of them received scorpion antivenom i.v. at times ranging from 45 min. to 5 h after the accident (median=2h. On admission, the main clinical manifestations and laboratory and electrocardiographic changes were: vomiting (17, diaphoresis (15, tachycardia (14, prostration (10, tachypnea (8, arterial hypertension (7, arterial hypotension (5, tremors (5, hypothermia (4, hyperglycemia (17, leukocytosis (16/16, hypokalemia (13/17, increased CK-MB enzyme activity (>6% of the total CK, 11/12, hyperamylasemia (11/14, sinusal tachycardia (16/17 and a myocardial infarction-like pattern (11/17. Six patients stung by T.serrulatus had depressed left ventricular systolic function assessed by means of echocardiography. Of these, five presented pulmonary edema and four had shock. A child aged two-years old presented severe respiratory failure and died 65 h after being stung by T.serrulatus. Severe envenomations caused by T.serrulatus were 26.2 times more frequent than those caused by T.bahiensis (pNo período de janeiro de 1984 a maio de 1994, de 239 crianças com até 15 anos de idade, picadas por escorpiões pertencentes às espécies T.bahiensis (84,9% e T.serrulatus (15,1%, 17 apresentaram envenenamento grave. Destes 17 pacientes, cuja idade variou de 1 a 11 anos (mediana=2anos, 14 foram picados por T.serrulatus e 3 por T.bahiensis. Todos receberam anti-veneno escorpiônico i.v. entre 45 min. e 5 hs após o acidente (mediana=2 horas. À admissão, as principais manifestações clínicas, alterações laboratoriais e eletrocardiográficas observadas foram: vômitos(17, sudorese profusa(15, taquicardia(14, prostração(10, taquipnéia (8, hipertensão arterial(7, hipotensão arterial(5, tremores(5

  15. Sever's Disease

    Science.gov (United States)

    ... results from physical activities and sports that involve running and jumping, especially those that take place on hard surfaces, such as track, basketball, soccer, and gymnastics. Sever's disease also can result from ...

  16. 69例小儿中性粒细胞减少症的治疗方法及疗效分析%The treatment method and curative effect of 69 children with neutropenia disease

    Institute of Scientific and Technical Information of China (English)

    牛江英

    2012-01-01

    Objective: To investigate the treatment method and curative effect of the children with neutropenia disease. Methods; Sixty - nine children with neutropenia disease treated in our hospital from January 2009 to August 2010 were chosen and randomly divided into group A (treatment group) with 35 cases and group B (control group) with 34 cases. The children in group A were taken routine use of antiviral drugs and supportive care plus Mannatide or Lee Jun - liter white blood cells trealmeny. The children in group B were used support routine use of antiviral drugs and treatment. The leukocytes and neutrophils for the two groups before and after treatment were compared. The cure rate for the two groups after treatment were also compared. Results; After treatment and intensive care, 26 patients returned to normal neutrophils in the treatment group, the total effective rate was 74. 3% , while 20 cases were normal in the control group, the total effective rate was 58. 8%. There was significant difference between the two groups, P <0.01. Conclusion; Children neutropenia is a serious disease endangering the health of children, it should be based on clinical prevention, take rational use of antibiotics, early detection and early action for the cause of comprehensive treatment for treatment to avoid serious damage to the children.%目的:分析小儿中性粒细胞减少症的治疗方法及疗效.方法:将2009年1月~2010年8月收治的小儿中性粒细胞减少症69例患儿随机分为A组(治疗组)35例和B组(对照组)34例.A组除常规应用抗病毒药物及支持治疗外,加用甘露聚糖肽或利可君升白细胞药物治疗;B组常规应用抗病毒药物及支持治疗.比较两组治疗前后的白细胞总数、中性粒细胞绝对值及治愈率.结果:治疗组26例恢复正常,总有效率为74.3%;对照组20例恢复正常,总有效率为58.8%.两组比较P<0.01.结论:小儿粒细胞减少症是严重危害小儿健康的疾病,临床应以

  17. CORRECTION OF NEUTROPENIA BY GM-CSF IN PATIENTS WITH A LARGE GRANULAR LYMPHOCYTE-PROLIFERATION

    NARCIS (Netherlands)

    MULDER, AB; DEWOLF, JTM; SMIT, JW; VANOOSTVEEN, JW; VELLENGA, E

    1992-01-01

    The in vivo and in vitro effects of GM-CSF were tested in four patients with large granular lymphocyte proliferation (LGLP) and severe granulocytopenia. All patients had an increased percentage of LGL cells (> 20%), whereas 3/4 patients demonstrated rearranged T-cell-receptor genes. An effect on the

  18. The clinical value of procalcitonin in patients with chemotherapy-induced febrile neutropenia%降钙素原在化疗致粒细胞减少伴发热患者中的临床意义

    Institute of Scientific and Technical Information of China (English)

    张忠伟; 申丽华; 傅凤鸣; 王朋妹; 朱彪

    2016-01-01

    Background and purpose:Previous researches have shown that procalcitonin differentiates infec-tious from non-infectious fever and assesses the severity of infectious diseases. This study aimed to investigate the clin-ical value of procalcitonin in patients with chemotherapy-induced febrile neutropenia.Methods:A total of 147 patients with chemotherapy-induced febrile neutropenia admitted to intensive care unit from Jan. 2012 to Dec. 2014 were di-vided into infectious group and fever of unknown origin group according to clinical symptoms, signs and etiology. The infectious group was divided into sepsis, severe sepsis, and septic shock groups according to the severity of infection. The procalcitonin levels were compared between different groups.Results:A procalcitonin cut-off value>0.935 ng/mL provided a sensitivity of 90.0%, speciifcity of 90.0% and AUC=0.905. The procalcitonin level of the infectious group was signiifcantly higher than that of the fever of unknown origin group [1.805 (1.268-2.523) ng/mLvs 0.555 (0.398-0.818) ng/mL,P<0.001]. There is a signiifcant difference between the severe sepsis group and the sepsis group [13.885 (7.600-17.961) ng/mLvs 1.805 (1.268-2.563) ng/mL,P<0.001]. Compared with the severe sepsis group, the value of procalcitonin in the septic shock group was signiifcantly higher [23.800 (20.050-30.478) ng/mLvs 13.885 (4.955-19.133) ng/mL,P<0.001].Conclusion:Plasma procalcitonin is a useful marker for diagnosing neutropenia in patients with infection. Meanwhile, procalcitonin can be used to assess the severity of infection in patients with neutropenia.%背景与目的:以往研究显示血清降钙素原可用于发热性疾病的诊断及其严重程度的评估。该研究旨在探讨血清降钙素原在化疗致粒细胞减少伴发热患者中的临床意义。方法:回顾性分析2012年1月—2014年12月以化疗致粒细胞减少伴发热收入复旦大学附属肿瘤医院ICU治疗的147例患者的临床资料。根据患

  19. Necrosis cutánea severa por picadura de raya en el miembro inferior: presentación de un caso y revisión de la literatura Stingray injury causing severe cutaneous necrosis: case report and literature review

    Directory of Open Access Journals (Sweden)

    M.A. Hoyos Franco

    2009-12-01

    Full Text Available El propósito de este trabajo es presentar nuestra experiencia con un caso de picadura por raya en el área maleolar interna del pie derecho de un hombre de 25 años, que sufrió mientras pescaba. Inicialmente presentó dolor urente, seguido de edema severo, con necrosis de la piel suprayacente. Ingresa a nuestra institución 24 horas después y con edema, eritema y secreción purulenta en el sitio afectado. Se inicia tratamiento con antibióticos sistémicos y se realizan tres lavados y desbridamientos en días subsecuentes. Evolucionó con solución de continuidad y exposición ósea sobre el maléolo interno en un área de 7 x 5 cms que se cubrió con un colgajo safeno interno de flujo reverso que evolucionó satisfactoriamente. Las lesiones ocasionadas por la picadura de raya (Myliobatidae son escasamente referidas en Cirugía Plástica, pues la lesión tisular severa de las extremidades es poco frecuente. Revisamos la fisiopatología, los tipos de lesiones que se producen y los principios de tratamiento.The purpose of this paper is to present our experience with one case of stingray injury, in a 25 years old man, while he was fishing, compromising his right feet at the internal malleolus. At the beginning he had severe pain, and during the first hours after the accident progressive edema was evident. He arrived to our hospital 24 hours later, when purulent discharge and eritem was obvious. Intravenous antibiotics were initiated and three surgical debridements where performed during the following week. After the process was complete, a soft tissue defect was created on the internal malleolus (7 x 5 cm. Reconstruction was achieved using a reverse internal saphenous flap that evolved satisfactorily. Severe skin injuries caused by stingrays (Myliobatidae have been reported shortly in plastic surgery literature, because these lesions usually heal without surgical procedures. We review skin injury physiopathology, showing the different kind of

  20. Hematological abnormalities in severe anorexia nervosa.

    Science.gov (United States)

    Sabel, Allison L; Gaudiani, Jennifer L; Statland, Barbara; Mehler, Philip S

    2013-05-01

    Little is known about the prevalence of hematologic abnormalities in adults with severe anorexia nervosa. We report the first major analysis of hematologic dysfunction in such patients. We retrospectively analyzed the charts of 53 men and women with severe anorexia nervosa, admitted between October 2008 and December 2010 for medical stabilization to our center, which has a national referral base. Patients were predominantly female (89 %), with a median age of 28 years (range 17-65), and were hospitalized for a median duration of 15 days (I.Q.R. 9-29). Nadir body mass index during hospitalization was markedly low at 12.4 kg/m(2) (range 8.4-15.7), and the mean discharge BMI was 13.8 kg/m(2) (range 10.2-16.8). 83 % of patients were anemic (hematocrit  400 k/μL) during their hospitalization. Eighty-nine percent of patients had resolved their neutropenia by discharge. Marked hematologic deficiencies are often present in patients with severe anorexia nervosa, generally attributed to starvation-mediated gelatinous marrow transformation which resolves with proper nutritional rehabilitation. Improved provider awareness of this association may reduce unnecessary testing and costly treatment interventions.

  1. Analysis of concurrent severe sciatica without pressure cause following lumbar spinal surgery%腰椎管术后并发无致压因素剧烈坐骨神经痛的剖析

    Institute of Scientific and Technical Information of China (English)

    倪凤民; 宋恒平; 王平均; 张皓; 陈睿; 杨小龙

    2013-01-01

    Objective To explore the reasons of concurrent severe sciatica without pressure cause following lumbar spinal surgery in a short term,to provide ideas for processing. Methods The patient characteristics and treatment of 12 cases of concurrent severe sciatica following lumbar spinal surgery shortly which appeared between October 2004 to August 2011 lumbar are reviewed, analyze the possible factors,and evaluate the effectiveness of treatment measures. Results The males in 12 cases are 3 cases, females are 9 cases,2 cases broke out after anesthesia disappear hours latter, the others for 1-2 days after the operations,presented lower limb pain just like which were tored or were cut by knife, pure sick limb pain are 10 cases,only healthy limb pain in 1,1 case of sick limb pain with healthy limb. Nothing had been found to pressure roots during the healing process. Calm、diminish inflammation、 dehydration,nourish nerve and so on,all patients get rid of their pain during staying be in hospital, the longest condition lasted 1 month,follow-up half year-2 years, did not see the repetition symptoms. Conclusion The physique factors, segmental aseptic inflammation around nerve root, root sensitization and root reaction caused by the sudden change of roots pathological environment and so on may be the reasons which lead to their emergency response sciatica.%目的 探讨腰椎管手术后短期内出现无椎管内致压因素的剧烈坐骨神经痛的原因,为处理提供思路.方法 对2004-10-2011-08月腰椎术后短期内发生剧烈坐骨神经痛的12例患者的病情特点及治疗进行回顾,分析产生症状的可能因素,评价处置措施的有效性.结果 12例中男3例,女9例,2例术后麻醉消失数小时后发作,其他为术后1~2 d发作,呈现撕裂样、刀割样下肢串痛,单纯患肢痛10例,单纯健肢痛1例,伴有健肢痛1例.治疗过程中未发现手术节段有任何致压物.镇静、消炎、脱水、神经营养神经治疗后,

  2. Analysis of the Causes of Several Costly Chinese Medicine Adulteration and Identiifcation Methods%几种贵重中药掺假成因分析及鉴别方法分析

    Institute of Scientific and Technical Information of China (English)

    张举良

    2015-01-01

    目的:分析几种贵重中药掺假成因分析及鉴别方法。方法购买海马、麝香、冬虫夏草等贵重中药,并采购相应的掺假中药进行对比分析,总结两者特性,并记录其鉴别方法。结果在本次研究中,对海马、麝香、冬虫夏草等三种名贵掺假中药进行了详细对比分析,总结了其鉴别方法,为其鉴别提供了重要价值。结论利用多种方法对本次研究的这几味中药进行鉴定,可提高掺假中药的鉴别率,实际应用价值良好。%Objective To analysis the causes and several precious Chinese medicine adulteration identification method. Methods The purchase of hippocampus, musk, Cordyceps sinensis and precious traditional Chinese medicine, Chinese medicine and procurement adulteration corresponding comparative analysis, summarize the characteristics of the two, and record the differential method. Results In this study, the hippocampus, musk, Cordyceps three kinds of precious Chinese medicine contrast adulteration in detail, summarizes the identification method, and provides an important value for its identiifcation. Conclusion Through this a few herbs were identiifed in this study from many aspects, and can improve the identiifcation of adulterated in traditional Chinese medicine rate, good practical application value.

  3. Analysis of the Causes of Several Costly Chinese Medicine Adulteration and Identiifcation Methods%几种贵重中药掺假成因分析及鉴别方法分析

    Institute of Scientific and Technical Information of China (English)

    张举良

    2015-01-01

    Objective To analysis the causes and several precious Chinese medicine adulteration identification method. Methods The purchase of hippocampus, musk, Cordyceps sinensis and precious traditional Chinese medicine, Chinese medicine and procurement adulteration corresponding comparative analysis, summarize the characteristics of the two, and record the differential method. Results In this study, the hippocampus, musk, Cordyceps three kinds of precious Chinese medicine contrast adulteration in detail, summarizes the identification method, and provides an important value for its identiifcation. Conclusion Through this a few herbs were identiifed in this study from many aspects, and can improve the identiifcation of adulterated in traditional Chinese medicine rate, good practical application value.%目的:分析几种贵重中药掺假成因分析及鉴别方法。方法购买海马、麝香、冬虫夏草等贵重中药,并采购相应的掺假中药进行对比分析,总结两者特性,并记录其鉴别方法。结果在本次研究中,对海马、麝香、冬虫夏草等三种名贵掺假中药进行了详细对比分析,总结了其鉴别方法,为其鉴别提供了重要价值。结论利用多种方法对本次研究的这几味中药进行鉴定,可提高掺假中药的鉴别率,实际应用价值良好。

  4. Acute promyelocytic leukemia after renal transplant and filgrastim treatment for neutropenia

    Science.gov (United States)

    Krause, John R.

    2016-01-01

    Prolonged immunosuppression in solid organ transplant recipients has been considered a risk for developing opportunistic infections and malignancies. Acute leukemia is a rare complication. We report a case of acute promyelocytic leukemia (APL) (FAB M3) after cadaveric renal transplant for focal segmental glomerulosclerosis in a 24-year-old woman. Her immunosuppressive therapy included tacrolimus, mycophenolate mofetil, and prednisone. Approximately 2 years after transplant, she became pancytopenic, prompting administration of filgrastim. A few doses caused a markedly increased blast count, resulting in a diagnosis of APL. She was successfully treated with all-trans-retinoic acid and arsenic trioxide. Myeloproliferative neoplasms after organ transplant or due to filgrastim are rare. PMID:27695174

  5. 乳腺癌FE100C类方案新辅助化疗后粒细胞减少和肝功能损害的发生及对策%Neoadjuvant chemotherapy using epirubicin, cyclophosphamide and fluorouracil:neutropenia and elevation of transaminase, and their management

    Institute of Scientific and Technical Information of China (English)

    王歆光; 范铁; 范照青; 王天峰; 解云涛; 李金锋; 欧阳涛

    2015-01-01

    目的:分析乳腺癌FE100 C类方案新辅助化疗后粒细胞减少和肝功能损害的发生率及其对策。方法从北京大学肿瘤医院乳腺癌预防治疗中心数据库中筛选2011年1月至2012年12月确诊的303例原发性乳腺癌并接受FE100 C类方案新辅助化疗4周期后手术的连续病例,分析乳腺癌患者粒细胞减少和肝功能损害的发生情况。结果全组共有200例(66.0%)患者曾出现中性粒细胞绝对值或白细胞3~4度减少,其中176例患者发生于第1周期化疗。粒细胞减少性发热( FN)13例14例次;转氨酶升高46例(15.2%),其中2度及以上升高15例(5.0%)。预防性使用粒细胞集落刺激因子32例,均未发生FN以及因粒细胞减少导致的化疗推迟。采用化疗开始后每周1次、化疗前+化疗第10天以及化疗前+化疗第7、10、14天3种方式监测化疗后血常规变化,发生因粒细胞减少不符合化疗标准导致化疗推迟分别为3例(5.0%)、7例(3.9%)和2例(3.2%)。3种监测方式发生FN分别为2例(3.3%)、8例(4.4%)和3例(4.8%)。不同血常规监测方式之间因粒细胞减少导致化疗推迟发生率的差异及FN发生率的差异均无统计学意义(均P>0.05)。不同氟尿嘧啶使用方式之间3~4度粒细胞减少及转氨酶升高发生率的差异均无统计学意义(均P>0.05)。结论在无常规预防性使用粒细胞集落刺激因子的情况下,FE100 C类方案4周期化疗中3~4度中性粒细胞绝对值或白细胞减少的发生率较高,但FN的发生率较低。无基础肝脏疾病患者无需预防性使用保肝药。%Objective To retrospectively investigate the incidence of severe neutropenia and elevation of transaminase during neoadjuvant chemotherapy using epirubicin, cyclophosphamide and fluorouracil in breast cancer patients.Methods From January 2011 to December 2012, 303

  6. 重型肝炎与肝硬化患者医院感染病原菌的耐药性分析%Drug resistance of pathogens causing nosocomial infections in patients with severe hepatitis and liver cirrhosis

    Institute of Scientific and Technical Information of China (English)

    郑颖; 陈亮; 鲁艳

    2014-01-01

    OBJECTIVE To approach the species of pathogenic bacteria causing nosocomial infections in the patients with severe hepatitis and liver cirrhosis and analyze the drug resistance so as to provide guidance clinical use of an-tibiotics .METHODS The clinical data of 251 severe hepatitis and liver cirrhosis patients complicated with secondary nosocomial infections were reviewed ,then the isolation ,culture ,and identification of the pathogens were per-formed by referring to the criteria of clinical microorganism laboratory examination ;the drug susceptibility testing was performed by using K-B method ,the drug susceptibility ,intermediate ,and resistance were determined ac-cording to the latest breakpoints of CLSI ,and the whole process of examination was carried out under the qualified indoor quality control .RESULTS Among the patients with severe hepatitis and liver cirrhosis ,the patients with ab-dominal infections accounted for 27 .6% ,the patients with respiratory tract infections 19 .2% ,the patients with urinary tract infections 16 .9% .A total of 195 strains of pathogens have been isolated ,including 195 (70 .1% ) strains of gram-negative bacteria ,60 (21 .6% ) strains of gram-positive bacteria and 23 (8 .3% ) strains of fungi;the Escherichia coli ,Enterococcus ,K lebsiella pneumoniae ,and Pseudomonas aeruginosa ranked the top four spe-cies of pathogens ,accounting for 23 .4% ,17 .6% ,16 .9% ,and 11 .9% ,respectively .The drug susceptibility rates of the E .coli and K .pneumoniae to imipenem and meropenem were 100 .0% ,and the drug resistance rates to cefoperazone-sulbactam and amikacin were less than 15 .4% ;the drug resistance rates of the P .aeruginosa to pip-eracillin-tazobactam and cefoperazone-sulbactam were less than 18 .2% .The isolation rates of the imipenem-resist-ant P .aeruginosa and Acinetobacter baumannii were 38 .5% and 33 .3% ,respectively ;the isolation rates of the extended-spectrum β-lactamases (ESBLs)-producing E .coli and K .pneumoniae were

  7. Analysis of a Severe Convective Weather Caused by Isolated Cloud Cluster on 22 May 2013 in Shanxi Province%孤立云团造成的一次强对流天气分析

    Institute of Scientific and Technical Information of China (English)

    赵桂香; 王思鳭; 邱贵强; 王晓丽

    2015-01-01

    ,and excited isolated cloud clusters nearby. The vertical structure with high - level divergence and low - level convergence above the cloud clusters made the upward motion strengthen continuously. Consequently,the isolated cloud clusters maintained and developed. These meso-γand meso-αscale clus-ters were the direct cause leading to this severe convective weather. The dry frontogenesis caused by surface continuous strengthening of water vapor from the sea at the back of high pressure system and the invading of dry air at the front of ridge,was an important triggering mechanism of the strong convective weather. From the radar mosaic products of composite reflectivity,this process resulted from the de-velopment and merging of convective cells. The evolution of these cells went through a process from“convective cell,merging,ribbon echo,bow echo to weakening and vanishing”,and the whole process was divided into two stages,when the area,intensity and moving speed of echoes were different,the characteristics of convective weather were different too. Obvious differences among three types dur-ing this process existed in temperature and humidity profiles and environmental parameters,which could be served as judging the severe convective weathers.

  8. Granulocyte colony-stimulating factors for febrile neutropenia prophylaxis following chemotherapy: systematic review and meta-analysis

    Directory of Open Access Journals (Sweden)

    Stevenson Matt D

    2011-09-01

    Full Text Available Abstract Background Febrile neutropenia (FN occurs following myelosuppressive chemotherapy and is associated with morbidity, mortality, costs, and chemotherapy reductions and delays. Granulocyte colony-stimulating factors (G-CSFs stimulate neutrophil production and may reduce FN incidence when given prophylactically following chemotherapy. Methods A systematic review and meta-analysis assessed the effectiveness of G-CSFs (pegfilgrastim, filgrastim or lenograstim in reducing FN incidence in adults undergoing chemotherapy for solid tumours or lymphoma. G-CSFs were compared with no primary G-CSF prophylaxis and with one another. Nine databases were searched in December 2009. Meta-analysis used a random effects model due to heterogeneity. Results Twenty studies compared primary G-CSF prophylaxis with no primary G-CSF prophylaxis: five studies of pegfilgrastim; ten of filgrastim; and five of lenograstim. All three G-CSFs significantly reduced FN incidence, with relative risks of 0.30 (95% CI: 0.14 to 0.65 for pegfilgrastim, 0.57 (95% CI: 0.48 to 0.69 for filgrastim, and 0.62 (95% CI: 0.44 to 0.88 for lenograstim. Overall, the relative risk of FN for any primary G-CSF prophylaxis versus no primary G-CSF prophylaxis was 0.51 (95% CI: 0.41 to 0.62. In terms of comparisons between different G-CSFs, five studies compared pegfilgrastim with filgrastim. FN incidence was significantly lower for pegfilgrastim than filgrastim, with a relative risk of 0.66 (95% CI: 0.44 to 0.98. Conclusions Primary prophylaxis with G-CSFs significantly reduces FN incidence in adults undergoing chemotherapy for solid tumours or lymphoma. Pegfilgrastim reduces FN incidence to a significantly greater extent than filgrastim.

  9. [Severe asthma].

    Science.gov (United States)

    González, Claudio D

    2016-01-01

    The objectives of this work were to investigate the frequency of severe asthma (SA) according to WHO definition and to compare SA patients' characteristics with those of non-severe asthma (NSA); secondly, to investigate the level of control reached throughout a period of regular treatment. Between 1-1-2005 and 12-31-2014, 471 medical records from patients with bronchial asthma assisted in Buenos Aires City were analyzed. SA frequency was 40.1% (189/471), being significantly higher among patients from the public health system (47.7%, 108/226 vs. 33%, 81/245, p = 0.001). SA patients were older than NSA ones (51.3 ± 17.4 vs. 42.6 ± 17.1 years, p = 0.000), presented longer time since onset of the disease (median 30 vs. 20 years, p = 0.000), lower educational levels (secondary level or higher 41.7% vs. 58.1%, p = 0.000), lower frequency of rhinitis (47% vs. 60.6%, p = 0.004), more severe levels of airway obstruction (FEV% 50.2 ± 13.7 vs. 77.7 ± 12.4, p = 0.000), more frequent antecedents of Near Fatal Asthma (11.1% vs. 2.8%, p = 0.000), higher levels of serum IgE (median of 410 vs. 279 UI/l, p = 0.01) and higher demand of systemic steroids requirements and hospitalizations (68.7% vs. 50.7%, p = 0.000 and 37.5% vs. 15.9%, p = 0.000, respectively). A 30.6% of SA patients (58/189) reached a follow-up period of 12 months, 13 (22.5%) of whom reached the controlled asthma level. The frequency of SA found seems to be considerable. Multicenter studies to investigate the levels of control reached by SA patients with access to proper treatment are recommended.

  10. Ontong Java volcanism initiated long-term climate warming that caused substantial changes in terrestrial vegetation several tens of thousand years before the onset of OAE1a (Early Aptian, Cretaceous)

    Science.gov (United States)

    Keller, Christina E.; Hochuli, Peter A.; Giorgioni, Martino; Garcia, Therese I.; Bernasconi, Stefano M.; Weissert, Helmut

    2010-05-01

    During Cretaceous times, several intense volcanic episodes are proposed as trigger for episodic climate warming, for changes in marine circulation patterns and for elevated marine productivity, which resulted in the widespread black shale deposits of the Oceanic Anoxic Events (OAE). In the sediments underlying the early Aptian OAE1a black shales, a prominent negative carbon isotope excursion is recorded. Its origin had long been controversial (e.g. Arthur, 2000; Jahren et al., 2001) before recent studies attributed it to the Ontong Java volcanism (Méhay et al., 2009; Tejada et al., 2009). Volcanic outgassing results in an increased pCO2 and should lead to a rise in global temperatures. We therefore investigated if the volcanically-induced increase in pCO2 at the onset of OAE1a in the early Aptian led to a temperature rise that was sufficient to affect terrestrial vegetation assemblages. In order to analyse changes in terrestrial palynomorph assemblages, we examined 15 samples from 12 black shale horizons throughout the early Aptian negative C-isotope spike interval of the Pusiano section (Maiolica Formation; N-Italy). These sediments were deposited at the southern continental margin of the alpine Tethys Ocean and have been bio- and magnetostratigraphically dated by Channell et al. (1995). In order to obtain a continuous palynological record of the negative C-isotope spike interval and the base of OAE1a, we combined this pre-OAE1a interval of Pusiano with the OAE1a interval of the nearby Cismon section (Hochuli et al., 1999). The sporomorph assemblages at the base of this composite succession feature abundant bisaccate pollen, which reflects a warm-temperate climate. Rather arid conditions are inferred from low trilete spore percentages. Several tens of thousand years before the onset of OAE1a, C-isotope values started to decrease. Some thousand years later, bisaccate pollen began to decrease, whereas an increase of Classopollis spp. and Araucariacites spp

  11. Capnocytophaga ochracea-related Bacterium Bacteremia in a Hypertrophic Cardiomyopathy Patient without Neutropenia.

    Science.gov (United States)

    Ito, Shimpei; Hagiya, Hideharu; Kimura, Keigo; Nishi, Isao; Yoshida, Hisao; Kioka, Hidetaka; Ohtani, Tomohito; Yamaguchi, Osamu; Tanabe, Kazuaki; Tomono, Kazunori; Sakata, Yasushi

    2016-01-01

    Gram-negative fusiform rods were detected in a blood culture obtained from a 63-year-old man who had been hospitalized for a long duration for severe heart failure. Although the organism could not be identified using a conventional method, it was finally identified as a bacterium of the Capnocytophaga ochracea group based on the results of biochemical testing, 16S rRNA sequencing and a matrix-assisted laser desorption ionization time-of-flight mass spectrometry analysis. Although neutropenic patients with poor oral hygiene are exclusively vulnerable to Capnocytophaga bacteremia, this case was unique because such predisposing conditions were not noted. A multi-centered investigation is warranted for a better understanding of this clinically rare, but potentially pathogenic organism. PMID:27629977

  12. Microbial translocation contribute to febrile episodes in adults with chemotherapy-induced neutropenia.

    Directory of Open Access Journals (Sweden)

    Michelle Wong

    Full Text Available In this study we sought to determine the contribution of microbial translocation to febrile episodes with no attributable microbiological cause (Fever of Unknown Origin, FUO in an adult febrile neutropaenic cohort. Endotoxin concentrations were measured with the chromogenic Limulus Amoebocyte Assay and used as a direct measure of bacterial products whilst soluble CD14 (sCD14, measured with ELISA was selected as an indicator of the early host response to endotoxins. Endotoxin concentrations in this cohort were generally elevated but did not differ with the presentation of fever. Further stratification of the febrile episodes based on the microbiological findings revealed significantly (p = 0.0077 elevated endotoxin concentrations in FUO episodes compared with episodes with documented bacterial and viral findings. sCD14 concentrations were however, elevated in febrile episodes (p = 0.0066 and no association was observed between sCD14 concentration and microbiological findings. However, FUO episodes and episodes with Gram-negative bacteraemia were associated with higher median sCD14 concentrations than episodes with Gram-positive bacteraemia (p = 0.030. In conclusion, our findings suggest that in the absence of microbiological findings, microbial translocation could contribute to febrile episodes in an adult neutropaenic cohort. We further observed an association between prophylactic antibiotic use and increased plasma endotoxin concentrations (p = 0.0212.

  13. Chest computed tomography findings in severe influenza pneumonia occurring in neutropenic cancer patients

    Directory of Open Access Journals (Sweden)

    Rosana Souza Rodrigues

    2012-01-01

    Full Text Available OBJECTIVE: To describe the chest computed tomography findings for severe influenza H1N1 infection in a series of hospitalized neutropenic cancer patients. METHODS: We performed a retrospective systematic analysis of chest computed tomography scans for eight hospitalized patients with fever, neutropenia, and confirmed diagnoses of influenza H1N1. The clinical data had been prospectively collected. RESULTS: Six of eight patients (75% developed respiratory failure and required intensive care. Prolonged H1N1 shedding was observed in the three mechanically ventilated patients, and overall hospital mortality in our series was 25%. The most frequent computed tomography findings were ground-glass opacity (all patients, consolidation (7/8 cases, and airspace nodules (6/8 cases that were frequently moderate or severe. Other parenchymal findings were not common. Five patients had features of pneumonia, two had computed tomography findings compatible with bronchitis and/or bronchiolitis, and one had tomographic signs of chronicity. CONCLUSION: In this series of neutropenic patients with severe influenza H1N1 infection, chest computed tomography demonstrated mainly moderate or severe parenchymatous disease, but bronchiolitis was not a common feature. These findings associated with febrile neutropenia should elicit a diagnosis of severe viral infection.

  14. Neutropenia induced in outbred mice by a simplified low-dose cyclophosphamide regimen: characterization and applicability to diverse experimental models of infectious diseases

    Directory of Open Access Journals (Sweden)

    Zapata Ana X

    2006-03-01

    Full Text Available Abstract Background For its low cost and ease of handling, the mouse remains the preferred experimental animal for preclinical tests. To avoid the interaction of the animal immune system, in vivo antibiotic pharmacodynamic studies often employ cyclophosphamide (CPM to induce neutropenia. Although high doses (350–450 mg/kg are still used and their effects on mouse leukocytes have been described, a lower dose (250 mg/kg is widely preferred today, but the characteristics and applicability of this approach in outbred mice have not been determined. Methods Fifteen female ICR mice were injected intraperitoneally with 150 and 100 mg/kg of CPM on days 1 and 4, respectively. Blood samples (~160 μL were drawn from the retro-orbital sinus of each mouse on days 1, 4, 5, 6, 7 and 11. Leukocytes were counted manually and the number of granulocytes was based on microscopic examination of Wright-stained smears. The impact of neutropenia induced by this method was then determined with a variety of pathogens in three different murine models of human infections: pneumonia (Klebsiella pneumoniae, Streptococcus pneumoniae, Staphylococcus aureus, meningoencephalitis (S. pneumoniae, and the thigh model (S. aureus, Escherichia coli, Bacteroides fragilis. Results The basal count of leukocytes was within the normal range for outbred mice. On day 4, there was an 84% reduction in total white blood cells, and by day 5 the leukopenia reached its nadir (370 ± 84 cells/mm3. Profound neutropenia (≤10 neutrophils/mm3 was demonstrated at day 4 and persisted through days 5 and 6. Lymphocytes and monocytes had a 92% and 96% decline between days 1 and 5, respectively. Leukocytes recovered completely by day 11. Mice immunosupressed under this protocol displayed clinical and microbiological patterns of progressive and lethal infectious diseases after inoculation in different organs with diverse human pathogens. Conclusion A CPM total dose of 250 mg/kg is sufficient to induce

  15. Filgrastim as a Rescue Therapy for Persistent Neutropenia in a Case of Dengue Hemorrhagic Fever with Acute Respiratory Distress Syndrome and Myocarditis

    Directory of Open Access Journals (Sweden)

    Desh Deepak

    2011-01-01

    Full Text Available Pathogenesis of dengue involves suppression of immune system leading to development of characteristic presentation of haematological picture of thrombocytopenia and leucopenia. Sometimes, this suppression in immune response is responsible for deterioration in clinical status of the patient in spite of all specific and supportive therapy. Certain drugs like steroids are used for rescue therapy in conditions like sepsis. We present a novel use of filgrastim as a rescue therapy in a patient with dengue hemorrhagic fever (DHF with acute respiratory distress syndrome (ARDS, myocarditis, and febrile neutropenia and not responding to standard management.

  16. A case report of severe meningoencephalitis caused by Listeria monocytogenes%重症单核细胞增多性李斯特菌脑膜脑炎1例

    Institute of Scientific and Technical Information of China (English)

    刘袁媛; 张文宏; 王冯滨

    2011-01-01

    李斯特菌脑膜脑炎是单核细胞增多性李斯特菌(LM)引起的细菌性脑膜脑炎,通常发生于免疫功能低下者.LM是人畜共患致病菌,通过污染的食物传播.欧美国家报道较多,国内报道较少.李斯特菌脑膜脑炎病死率高,临床表现与其他细菌性脑膜炎类似.脑脊液检查与结核性脑膜炎亦难鉴别,确诊依赖于脑脊液涂片及培养.本文报道1例重症李斯特菌脑膜脑炎,患者为免疫力正常的菲律宾籍中年女性,急性起病,先后以病毒性脑膜脑炎及结核性脑膜炎治疗,病情进展快,发病3周出现呼吸衰竭,气管插管呼吸机辅助通气.脑脊液培养见LM后,予氨苄西林联合氨基糖苷类药物治疗3周,患者意识恢复,病情好转出院.本病例报道总结了李斯特菌脑膜脑炎的临床特点、危险因素及治疗转归.%Listeria meningoencephalitis is a kind of bacterial meningoencephalitis caused by Listeria monocytogenes(LM), which most often occurs in immunocompromised persons. LM is a zoonotic pathogen transmitted through contaminated food. Historically, Europe and the United States have reported a greater number of infections, while infections in China are reported rarely. With its high mortality, it is difficult to diagnose Listeria meningoencephalitis due to the fact that the clinical manifestations are similar to other bacterial meningitis. It is also difficult to distinguish Listeria meningoencephalitis from tuberculous meningitis via cerebrospinal fluid. Diagnosis depends on the results of the cerebrospinal fluid smear and culture. In the present paper, a case of severe Listeria meningoencephalitis is reported. A middle-aged woman from the Philippines with normal immune function is presented with acute onset of illness whose condition deteriorated rapidly despite receiving routine therapy for viral meningoencephalitis and tuberculous meningitis. Respiratory failure occurred three weeks after onset of illness and tracheal

  17. Prognostic impact of neoadjuvant chemotherapy induced neutropenia on operable breast cancer%新辅助化疗诱导的粒细胞减少症对乳腺癌预后的影响

    Institute of Scientific and Technical Information of China (English)

    韩芸蔚; 王欣; 张斌; 温绍艳; 刘伟; 曹旭晨

    2011-01-01

    Objective To evaluate the relationship between neoadjuvant chemotherapy (combination of taxanes and anthracyclines ) induced-neutropenia and the efficacy of neoadjuvant chemotherapy and long-term survival in operable breast cancer patients. Methods Two hundred and eleven patients received 4 cycles of neoadjuvant chemotherapy (combination of taxanes and anthracyclines).Clinicopathological characteristics were compared between patients with neoadjuvant chemotherapy-induced neutropenia and patients without neutropenia. The efficacy of neoadjuvant chemotheray and long-term survival rate were analyzed. Results Among 211 patients there were 51 (24. 2% ) cases suffering from neutropenia and 160 (75.8%) cases were of no-neutropenia. The response to chemotherapy in patients with neutropenia were more effective than in no- neutropenia ones ( P < 0. 05 ). The 5-year disease-free survival (DFS) in patients with neutropenia was 82. 4%, while the 5-year disease-free survival ( DFS) with nonneutropenia was 60% ( P < 0. 01 ). Additionally, the 5-year overall survival ( OS ) in patients with neutropenia was 90. 2% and in patients with non-neutropenia patients was 67. 5% ( P < 0. 01 ).Conclusions Chemotherapy-induced neutropenia during neoadjuvant chemotherapy combination of taxanes and anthracyclines in patients with operable breast cancer has a better prognosis. The sensitivity of tumors given to chemotherapeutic drugs could be evaluated by chemotherapy-induced neutropenia.%目的 探讨新辅助化疗诱导的粒细胞减少症与采用蒽环类联合紫杉类新辅助化疗方案进行新辅助化疗患者的疗效及远期生存率之间的关系.方法 对接受4个周期蒽环类联合紫杉类新辅助化疗方案治疗的211例乳腺癌患者的资料进行回顾性分析.结果 211例中51(24.2%)例为嗜中性粒减少症患者,160(75.8%)例为非嗜中性粒细胞减少症患者.嗜中性粒细胞减少症患者组的新辅助化疗反应较非嗜中性粒细

  18. 恶性肿瘤患者化疗所致中性粒细胞减少与其疗效的相关性%Correlation of Neutropenia of Therioma Patients Occurring during Postoperative Chemotherapy with the Curative Effect

    Institute of Scientific and Technical Information of China (English)

    沈飞; 茅力平; 孙雅君

    2011-01-01

    Objective To investigate the correlation of neutropenia with the disease-free survival in patients with malignant tumors receiving chemotherapy. Methods Clinical data of 136 patients with malignant tumors receiving postoperative chemotherapy were retrospectively reviewed. Results The disease free survival was significantly different in patients with neutropenia,neutrophile granulocyte deficiency and no neutropenia ( P < 0. 05 ) ,hut there was no significant difference hetween the neutropenia group and the neutrophile granulocyte deficiency group( P > 0. 05 ). Conclusion Neutropenia during postoperative chemotherapy is significantly related to the disease-free survival time , while non-neutropenia cases possibly indicate lack of enough drug doses.%目的 探讨恶性肿瘤患者术后化疗过程中出现中性粒细胞减少与其无瘤生存期的相关性.方法 回顾性分析136例术后辅助化疗恶性肿瘤患者的临床资料.结果 中性粒细胞减少组/缺乏组无瘤生存期与无中性粒细胞减少组比较有显著差异(P<0.05),中性粒细胞减少组无瘤生存期与中性粒细胞缺乏组比较无显著差异(P>0.05).结论 恶性肿瘤患者化疗过程中出现中性粒细胞减少,与其无瘤生存期明显相关,而未出现中性粒细胞减少者可能提示药物剂量不足.

  19. Predictive modeling of the outcomes of chemotherapy-induced (febrile) neutropenia prophylaxis with biosimilar filgrastim (MONITOR-GCSF study)

    Science.gov (United States)

    Aapro, M.; Ludwig, H.; Bokemeyer, C.; Gascón, P.; Boccadoro, M.; Denhaerynck, K.; Krendyukov, A.; Gorray, M.; MacDonald, K.; Abraham, I.

    2016-01-01

    Background Risk models of chemotherapy-induced (CIN) and febrile neutropenia (FN) have to date focused on determinants measured at the start of chemotherapy. We extended this static approach with a dynamic approach of CIN/FN risk modeling at the start of each cycle. Design We applied predictive modeling using multivariate logistic regression to identify determinants of CIN/FN episodes and related hospitalizations and chemotherapy disturbances (CIN/FN consequences) in analyses at the patient (‘ever’ during the whole period of chemotherapy) and cycle-level (during a given chemotherapy cycle). Statistical dependence of cycle data being ‘nested’ under patients was managed using generalized estimation equations. Predictive performance of each model was evaluated using bootstrapped c concordance statistics. Results Static patient-level risk models of ‘ever’ experiencing CIN/FN adverse events and consequences during a planned chemotherapy regimen included predictors related to history, risk factors, and prophylaxis initiation and intensity. Dynamic cycle-level risk models of experiencing CIN/FN adverse events and consequences in an upcoming cycle included predictors related to history, risk factors, and prophylaxis initiation and intensity; as well as prophylaxis duration, CIN/FN in prior cycle, and treatment center characteristics. Conclusion(s) These ‘real-world evidence’ models provide clinicians with the ability to anticipate CIN/FN adverse events and their consequences at the start of a chemotherapy line (static models); and, innovatively, to assess risk of CIN/FN adverse events and their consequences at the start of each cycle (dynamic models). This enables individualized patient treatment and is consistent with the EORTC recommendation to re-appraise CIN/FN risk at the start of each cycle. Prophylaxis intensity (under-, correctly-, or over-prophylacted relative to current EORTC guidelines) is a major determinant. Under-prophylaxis is clinically

  20. Severe Anemia in Malawian Children

    NARCIS (Netherlands)

    Calis, J.C.J.; Kamija, S.P.; Faragher, E.B.; Brabin, B.J.; Bates, I.; Cuevas, L.E.; Haan, de R.J.; Phiri, A.I.; Malange, P.; Khoka, M.; Hulshof, P.J.M.; Lieshout, L.; Beld, M.G.H.M.; Teo, Y.Y.; Rockett, K.A.; Richardson, A.; Kwiatkowski, D.P.; Molyneux, M.E.; Hensbroek, van M.B.

    2008-01-01

    Background Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. Methods We conducted a case¿control study of 381 preschool children with severe anemia (hemoglobin concentration,

  1. The role of ¹⁸F-FDG PET/CT for the diagnosis of infections in patients with hematological malignancies and persistent febrile neutropenia.

    Science.gov (United States)

    Gafter-Gvili, Anat; Paul, Mical; Bernstine, Hanna; Vidal, Liat; Ram, Ron; Raanani, Pia; Yeshurun, Moshe; Tadmor, Boaz; Leibovici, Leonard; Shpilberg, Ofer; Groshar, David

    2013-09-01

    We assessed the performance of PET/CT for diagnosis and management of infections in high-risk hematological cancer patients with persistent febrile neutropenia in a prospective study. (18)F-FDG PET/CT with contrast-enhanced CT was performed on day 5-7 of persistent fever. Between 2008 and 2011, 91 PET/CT examinations were performed for different episodes in 79 patients, resulting in 117 diagnoses. The sensitivity of the PET/CT was 79.8% (71/89) compared to 51.7% (46/89) with chest/sinus CT alone. Specificities were 32.14% (9/28) vs. 42.85% (12/28), respectively. PET/CT resulted in a change from the pre-test diagnosis in 63/91 (69%) of episodes and in modification of patients' management in 46/91 (55%). PET/CT was beneficial in diagnosing abdominal infections. PET/CT has a potential role in the diagnostic evaluation of patients with persistent febrile neutropenia.

  2. One case of severe combined immunodeficiency caused by JAK3 mutation and literature Review%JAK3突变致严重联合免疫缺陷1例报告并文献复习

    Institute of Scientific and Technical Information of China (English)

    周雪莲; 孙立峰; 吴俊峰; 刘玮; 赵晓东

    2012-01-01

    Objective To report the first JAK3 deficiency patient in China and to have literature review. Methods On April 25,2011, the blood samples of 1 case of suspected SCID child, his parents and grandparents were detected with PCR amplification and sequencing of JAK3 gene, single nucleotide polymorphism(SNPs) and TCRVB analysis in Children's Hospital of Chongqing Medical University. Results The mutation of this patient was a compound heterozygous mutation, both alleles being missense mutations. One allele (1308G>A: R403H) was located in the eighth exon of JAK3, which came from her father, the other (3354G> A:R1085Q) located in the twenty-third exon of JAK3, which came from her mother.Her parents and grandmother were carries.We confirmed these mutations were disease-caused mutations not SNPs by sequencing the 9th and 24th exon of JAK3 gene of twenty common people. Unfortunately, we failed to detect The TCRVβ of this patient because of the limited number of circulating T cells. Up to 2007, thirty mutations and thirty-five patients had been registered for JAK3 deficiency in JAK3base. Their immunophenotype was uniformly TB+ NK-SCID, but the clinical phenotype of them varied from classical SCID to almost normal immune function. Conclusion JAK3 deficiency is a rare autosomal recessive severe combined immunodeficiency disease (SCID),characterized by recurrent bacteria and virus infection,absence of T and NK cells but normal number of poorly functioning B cells in the peripheral blood. The diagnosis depends on stat5 phosphorylation,sequencing of JAK3 gene and detect the JAK3 protein by western blot or flow cytometry, when JAK3 deficiency is suspected. The most effective treatment for JAK3 deficiency is hematopoietic stem cell transplantation (HSCT) ,and patients who don't receive HSCT will usually die in infancy.%目的 报道我国首例JAK3突变致严重联合免疫缺陷(SCID)患儿资料并文献复习.方法 2011-04-25重庆医科大学附属儿童医院对1例疑似SCID

  3. Urgent treatment of severe symptomatic direct carotid cavernous fistula caused by ruptured cavernous internal carotid artery aneurysm using high-flow bypass, proximal ligation, and direct distal clipping: Technical case report

    Directory of Open Access Journals (Sweden)

    Hirotaka Hasegawa

    2014-01-01

    Full Text Available Background: Direct carotid cavernous fistula (CCF secondary to ruptured carotid cavernous aneurysms (CCAs is rare, but patients with this condition who develop acutely worsening and severe neuro-ophthalmic symptoms require urgent treatment. Endovascular methods are the first-line option, but this modality may not be available on an urgent basis. Case Description: In this article, we report a 45-year-old female with severe direct CCF due to rupture of the CCA. She presented with intractable headache and acute worsening of double vision and visual acuity. Emergent radiographic study revealed high-flow fistula tracked from the CCA toward the contralateral cavernous sinus and drained into the engorged left superior orbital vein. To prevent permanent devastating neuro-ophthalmic damages, urgent high-flow bypass with placement of a radial artery graft was performed followed by right cervical internal carotid artery (ICA ligation and the clipping of the ICA at the C3 portion, proximal to the ophthalmic artery. In the immediate postoperative period, her symptoms resolved and angiography confirmed patency of the high-flow bypass and complete occlusion of the CCF. Conclusion: With due consideration of strategy and techniques to secure safety, open surgical intervention with trapping and bypass is a good treatment option for direct severe CCF when the endovascular method is not available, not possible, or is unsuccessful.

  4. [VIRAL INFECTIONS: HUMAN PAPILLOMAVIRUS AND GENITAL HERPES TYPE 1 AND TYPE 2 AS A CAUSE OF CHRONIC RECURRENT CYSTITIS WITH SEVERE DYSURIA IN WOMEN WITH URETHRAL HYPERMOBILITY AND HYPOSPADIAS].

    Science.gov (United States)

    Derevjanko, T I; Ryzhkov, V V

    2015-01-01

    Female hypospadias presenting as a misplaced urethral opening is a common cause of chronic recurrent cystitis. Cystitis occurs when urogenital infection and anaerobic bacteria enter the urethra and bladder from the vagina. The authors argue that chronic infections of the lower urinary tract in women with hypospadias should be treated surgically by meatal transposition. They present a study confirming the role of the antiviral drug Panavir in prevention of inflammatory complications in the postoperative period in patients with a history of viral infection (human papillomavirus and herpes). PMID:26665761

  5. 试论爱伦·坡小说的几个特点及其成因%Discussing about Several Characteristics andCauses of Edgar Allan Poe's Novels

    Institute of Scientific and Technical Information of China (English)

    任亚杰

    2011-01-01

    19世纪美国著名小说家爱伦·坡的小说,偏爱冷色调,常以恐怖、变态心理为主要内容,对人物的双重性格的刻画细致入微,善于用作品的幻想表现他本人的清醒与睿智,强调文章应产生的效果,并且善于用第一人称来宣泄自身情感.这一系列特点的形成,与爱伦·坡本人的身世成长经历及其爱好密不可分。%Edgar Allan Poe was the famous American novelist in the nineteenth century. His novels were usually in the cool colour tone, and based on the terror and the abnormal psychology as the main substance. In his novels, he portrayed the person's double character perfectly. He was good at using the fantasy of the work to show his sober and wise and emphasize the effects causing by the essay. He was also adept in using first person to unbosom himself. The causes of these characteristics concerned with Allan Poe's life experience and interests.

  6. Síndrome respiratória aguda grave causada por influenza A (subtipo H1N1) Severe acute respiratory syndrome caused by the influenza A (H1N1) virus

    OpenAIRE

    Sandra Aparecida Ribeiro; Graziela Sgreccia Brasileiro; Luciana Novaes Campello Soleiman; Cristiano Cruz Silva; Cláudio Shoki Kavaguti

    2010-01-01

    Frente à pandemia causada por um novo vírus, influenza A (H1N1), descrevemos o caso de um paciente de 56 anos com síndrome respiratória aguda grave causada por influenza A (H1N1) sem fatores de risco importantes. Os resultados dos exames laboratoriais e de imagem (radiografia e TC de tórax) são apresentados aqui. O paciente teve boa evolução e recebeu alta hospitalar em 14 dias.In view of the pandemic caused by a new virus, influenza A (H1N1), we report the case of a 56-year-old patient witho...

  7. Several Problems about Prevention and Control of Oil Pollution Caused by Ships in Zhu-ao Waters%防治船舶在珠澳水域油污染的几个问题

    Institute of Scientific and Technical Information of China (English)

    李连健

    2001-01-01

    系统分析了船舶在珠澳水域造成的油污染情况,从现场管理的角度,结合近10年来发生的油污事故,针对不同污染途径找出了防治工作中的一些不足,并对水质分析、油品鉴定、防治重点、防治基金分配等方面提出一些建议。%Based on the analysis of oil pollution in Zhu-ao Waters caused by vessels in the past 10 years,some deficiencies in pollution prevention and control are pointed out from aspect of the field management.And some proposals are made for water quality analysis,oil type identification,prevention focal points and distribution of pollution prevention funds.

  8. 变压器几种常见故障产生原因和其处理方法解析%Several Common Causes of Transformer Fault and Its Processing Method Analysis

    Institute of Scientific and Technical Information of China (English)

    宋加波

    2015-01-01

    The factors affected by the transformer structure, process, operation mode, transformer failure often occurs in the process of operation, which have a great impact on normal operation of transformer. Through the analysis and the causes of common faults of transformer in operation, put forward the corresponding fault processing method.%受变压器结构、工艺、运维方式等因素的影响,变压器在运行过程中经常发生故障,这对变压器的正常运行有很大的影响。通过分析变压器运行中的常见故障及其产生原因,提出了相应的故障处理方法。

  9. Interleukin-5, interleukin-6, interleukin-8 and tumour necrosis factor-alpha levels obtained within 24-h of admission do not predict high-risk infection in children with febrile neutropenia

    Directory of Open Access Journals (Sweden)

    R Aggarwal

    2013-01-01

    Full Text Available Purpose: Biomarkers that can predict the severity of febrile neutropenia (FN are potential tools for clinical practice. Objective: The objective of this study is to evaluate the reliability of plasma interleukin (IL levels as indicators of high-risk FN. Materials and Methods: Children with haematological malignancies and FN were enrolled prospectively. A blood sample was obtained within 24-h of admission for estimation of IL-5, IL-6, IL-8 and tumour necrosis factor-alpha (TNF-α level by the enzyme-linked immunosorbent assay. Patients were stratified into three groups. Group I (low-risk: No focus of infection; Group II: Clinical/radiological focus of infection; Group III: Microbiologically proven infection or FN related mortality. Groups II and III were analysed as high-risk. The cytokines were assessed at three different cut-off levels. Results: A total of 52 episodes of FN in 48 patients were evaluated. The mean age was 6 years (range: 2-13. Primary diagnosis included acute lymphoblastic leukaemia (82%, non-Hodgkin′s lymphoma (13% and acute myeloid leukaemia (5%. Absolute neutrophil count was < 200 cells/μl in half and 200-500 in 23%. Majority were categorised as Group I (69%, followed by Group II (16% and III (15%. The range of IL-5 was too narrow and similar in the two risk-groups to be of any relevance. The best sensitivity of TNF-α and IL-6 for high-risk group was 78% and 70%, respectively. The highest specificity observed was 35%. The negative predictive value of IL-6, IL-8 and TNF-α exceeded 80%. Conclusion: IL-5, IL-6, IL-8 and TNF-α failed as predictors of clinically localised or microbiologically documented infection in children with chemotherapy induced FN. However, IL-6, IL-8 and TNF-α could be useful in excluding the possibility of high-risk infection.

  10. Epidemiology of severe trauma.

    Science.gov (United States)

    Alberdi, F; García, I; Atutxa, L; Zabarte, M

    2014-12-01

    Major injury is the sixth leading cause of death worldwide. Among those under 35 years of age, it is the leading cause of death and disability. Traffic accidents alone are the main cause, fundamentally in low- and middle-income countries. Patients over 65 years of age are an increasingly affected group. For similar levels of injury, these patients have twice the mortality rate of young individuals, due to the existence of important comorbidities and associated treatments, and are more likely to die of medical complications late during hospital admission. No worldwide, standardized definitions exist for documenting, reporting and comparing data on severely injured trauma patients. The most common trauma scores are the Abbreviated Injury Scale (AIS), the Injury Severity Score (ISS) and the Trauma and Injury severity Score (TRISS). Documenting the burden of injury also requires evaluation of the impact of post-trauma impairments, disabilities and handicaps. Trauma epidemiology helps define health service and research priorities, contributes to identify disadvantaged groups, and also facilitates the elaboration of comparable measures for outcome predictions.

  11. MRI评价前中盆腔器官脱垂程度及其病因%MRI assessment of the severity and causes of female pelvic organ prolapse in the anterior and middle compartment

    Institute of Scientific and Technical Information of China (English)

    蔡香然; 邱麟; 罗新; 吴何嘉; 刘斯润

    2013-01-01

    Objective To observe the correlation with vaginal support level Ⅰ and Ⅱ fascial defects, atrophy or rupture of levator ani muscles and the severity of female pelvic organ prolapse (POP) in the anterior and middle compartments. Methods Static and dynamic MR data of 51 female patients with POP in the anterior and middle compartments were retrospectively reviewed. POP was divided into mild and moderate-severe group according to the clinical POP Quantification (POP-Q). Level Ⅰ and Ⅱ fascial defects, atrophy or rupture of the iliococcygeal muscles and puborectal muscles were compared between mild and moderate-severe POP. Results POP was associated with level Ⅰ and Ⅱ fascial defects in 10% of patients, with levator muscle defects in 30%, with both defects in 40% , whereas without both defects in 20% patients. Proportions of presenting level Ⅰ and Ⅱ fascial defects, atrophy or rupture of the iliococcygeal muscles and puborectal muscles of the moderate-severe group were higher than those of the mild group. Conclusion Proportions of level Ⅰ and Ⅱ fascial defects and atrophy or rupture of levator ani muscles are associated with the severity of POP. Levator ani defects play an important role in the development of POP.%目的 观察盆腔器官脱垂(POP)程度与阴道支持Ⅰ、Ⅱ水平筋膜缺陷、肛提肌萎缩或断裂的关系.方法 回顾性分析经临床POP-Q评估诊断为POP的51例中老年女性的静态及动态MRI资料.根据POP-Q评价将POP分为轻度和中-重度,比较轻、中-重度前中POP间筋膜缺陷、髂尾肌和耻骨直肠肌萎缩或断裂发生率的差异.结果 约10%前中POP患者仅有Ⅰ、Ⅱ水平筋膜缺陷,约30%仅有肛提肌缺陷,约40%二者并存,约20%既无筋膜缺陷又无肛提肌缺陷;与轻度组比较,中-重度膀胱膨出、子宫脱垂组中筋膜缺陷、髂尾肌和耻骨直肠肌萎缩或断裂的比率增高.结论 Ⅰ、Ⅱ水平筋膜缺陷和肛提肌萎缩或断裂的

  12. Study design: two long-term observational studies of the biosimilar filgrastim Nivestim™ (Hospira filgrastim) in the treatment and prevention of chemotherapy-induced neutropenia

    International Nuclear Information System (INIS)

    Nivestim™ (filgrastim) is a follow-on biologic agent licensed in the EU for the treatment of neutropenia and febrile neutropenia induced by myelosuppressive chemotherapy. Nivestim™ has been studied in phase 2 and 3 clinical trials where its efficacy and safety was found to be similar to its reference product, Neupogen®. Follow-on biologics continue to be scrutinised for safety. We present a design for two observational phase IV studies that are evaluating the safety profile of Nivestim™ for the prevention and treatment of febrile neutropenia (FN) in patients treated with cytotoxic chemotherapy in general clinical practice. The NEXT (Tolérance de Nivestim chez les patiEnts traités par une chimiothérapie anticancéreuse cytotoXique en praTique courante) and VENICE (VErträglichkeit von NIvestim unter zytotoxischer Chemotherapie in der Behandlung malinger Erkrankungen) trials are multicentre, prospective, longitudinal, observational studies evaluating the safety profile of Nivestim™ in 'real-world’ clinical practice. Inclusion criteria include patients undergoing cytotoxic chemotherapy for malignancy and receiving Nivestim as primary or secondary prophylaxis (NEXT and VENICE), or as treatment for ongoing FN (NEXT only). In accordance with European Union pharmacovigilance guidelines, the primary objective is to evaluate the safety of Nivestim™ by gathering data on adverse events in all system organ classes. Secondary objectives include obtaining information on patient characteristics, efficacy of Nivestim™ therapy (including chemotherapy dose intensity), patterns of use of Nivestim™, and physician knowledge regarding filgrastim prescription and the reasons for choosing Nivestim™. Data will be gathered at three visits: 1. At the initial inclusion visit, 2. At a 1-month follow-up visit, and 3. At the end of chemotherapy. Recruitment for VENICE commenced in July 2011 and in November 2011 for NEXT. VENICE completed recruitment in July 2013 with

  13. Causes of acute hospitalization in adolescence: burden and spectrum of HIV-related morbidity in a country with an early-onset and severe HIV epidemic: a prospective survey.

    Directory of Open Access Journals (Sweden)

    Rashida A Ferrand

    2010-02-01

    Full Text Available BACKGROUND: Survival to older childhood with untreated, vertically acquired HIV infection, which was previously considered extremely unusual, is increasingly well described. However, the overall impact on adolescent health in settings with high HIV seroprevalence has not previously been investigated. METHODS AND FINDINGS: Adolescents (aged 10-18 y systematically recruited from acute admissions to the two public hospitals in Harare, Zimbabwe, answered a questionnaire and underwent standard investigations including HIV testing, with consent. Pre-set case-definitions defined cause of admission and underlying chronic conditions. Participation was 94%. 139 (46% of 301 participants were HIV-positive (median age of diagnosis 12 y: interquartile range [IQR] 11-14 y, median CD4 count = 151; IQR 57-328 cells/microl, but only four (1.3% were herpes simplex virus-2 (HSV-2 positive. Age (median 13 y: IQR 11-16 y and sex (57% male did not differ by HIV status, but HIV-infected participants were significantly more likely to be stunted (z-score<-2: 52% versus 23%, p<0.001, have pubertal delay (15% versus 2%, p<0.001, and be maternal orphans or have an HIV-infected mother (73% versus 17%, p<0.001. 69% of HIV-positive and 19% of HIV-negative admissions were for infections, most commonly tuberculosis and pneumonia. 84 (28% participants had underlying heart, lung, or other chronic diseases. Case fatality rates were significantly higher for HIV-related admissions (22% versus 7%, p<0.001, and significantly associated with advanced HIV, pubertal immaturity, and chronic conditions. CONCLUSION: HIV is the commonest cause of adolescent hospitalisation in Harare, mainly due to adult-spectrum opportunistic infections plus a high burden of chronic complications of paediatric HIV/AIDS. Low HSV-2 prevalence and high maternal orphanhood rates provide further evidence of long-term survival following mother-to-child transmission. Better recognition of this growing phenomenon is

  14. 感染相关性中性粒细胞减少症患儿的常见特点分析%Clinical study on features of children with infection related neutropenia

    Institute of Scientific and Technical Information of China (English)

    周立萍; 鞠秀丽

    2011-01-01

    目的:观察中性粒细胞减少症患儿的年龄分布、骨髓细胞学改变及血小板、肝功能、心肌酶和免疫功能的临床变化特点.方法:将60例中性粒细胞减少症患儿设为观察组,并分为婴儿组和儿童组,另选取58名健康儿童作为健康对照组,也分为婴儿组和儿童组.检测所有研究对象的血小板、ALT、磷酸肌酸激酶同工酶、细胞免疫、体液免疫指标,比较两组差异.结果与结论:① 1岁以下患儿占所有患儿的63%,其中又以6~8月龄最多,占35%;②骨髓常呈增生活跃或减低,易于见到异型淋巴细胞;③观察组的婴儿组血小板、ALT、磷酸肌酸激酶同工酶、IgM、CD8+高于健康对照组的同龄组;观察组婴儿组的IgG、CD3+、CD4+、CD4+/CD8+低于健康对照组的同龄组(P<0.05或0.01);④观察组的儿童组血小板、磷酸肌酸激酶同工酶、IgM、CD8+高于健康对照组的同龄组;观察组的儿童组IgG、CD3+、CD4+、CD4+/CD8+低于对照组的同龄组(P<0.05或0.01);⑤中性粒细胞减少症患儿血小板、肝功能、心肌酶和免疫功能的改变可能与病毒感染以及体内细胞免疫功能紊乱有关.%Objective: To observe age distribution, laboratory parameter characteristics (bone marrow cytology, platelet count, liver function, myocardial enzyme and immune function) and the possible causes of neutropenia in children.Methods: Sixty children diagnosed as neutropenia were included in observation group and were divided into two subgroups, infant observation group ( aged between 1 month and 1 year old, n = 37) and child observation group (aged between 1 year and 12 years old, n =23).At the same period, fifty-eight healthy children were selected into control group, who were divided into two subgroups according to the same age standard.Platelet count, alanine aminotransferase (ALT) level, MB isoenzyme of creatine kinase (CK-MB) , cellular and humoral immunity function were examined in both

  15. Modified apolipoprotein (apo) A-I by artificial sweetener causes severe premature cellular senescence and atherosclerosis with impairment of functional and structural properties of apoA-I in lipid-free and lipid-bound state.

    Science.gov (United States)

    Jang, Wookju; Jeoung, Nam Ho; Cho, Kyung-Hyun

    2011-05-01

    Long-term consumption of artificial sweeteners (AS) has been the recent focus of safety concerns. However, the potential risk of the AS in cardiovascular disease and lipoprotein metabolism has not been investigated sufficiently. We compared the influence of AS (aspartame, acesulfame K, and saccharin) and fructose in terms of functional and structural correlations of apolipoprotein (apo) A-I and high-density lipoproteins (HDL), which have atheroprotective effects. Long-term treatment of apoA-I with the sweetener at physiological concentration (3 mM for 168 h) resulted in loss of antioxidant and phospholipid binding activities with modification of secondary structure. The AS treated apoA-I exhibited proteolytic cleavage to produce 26 kDa-fragment. They showed pro-atherogenic properties in acetylated LDL phagocytosis of macrophages. Each sweetener alone or sweetener-treated apoA-I caused accelerated senescence in human dermal fibroblasts. These results suggest that long-term consumption of AS might accelerate atherosclerosis and senescence via impairment of function and structure of apoA-I and HDL. PMID:21533907

  16. PCI postoperative tirofiban cause severe thrombocytopenia three cases of clinical analysis%PCI术后应用替罗非班致重度血小板减少3例临床分析

    Institute of Scientific and Technical Information of China (English)

    赵国伟

    2015-01-01

    ObjectiveDiscussion after PCI tirofiban-induced platelet reducing acute severe clinical symptoms, diagnosis and treatment strategies.MethodsOur hospital in January 2014 --2015 in three cases in May after PCI tirofiban-induced thrombocytopenia in patients with severe reduction of clinical data were retrospectively analyzed.Result 3 patients on admission were within the normal range of platelets, PCI postoperative intravenous infusion of tirofiban people, 0.5-2 hours of the onset chills, fever and other symptoms, severe thrombocytopenia Jicha confirmed, promptly disable tirofiban and other antiplatelet drugs, hormone therapy, the platelet count in 10 hours to 5 days to return to normal, fatal bleeding and thrombosis and other adverse events did not occur.ConclusionApplication should be after PCI tirofiban should be closely observed for the disease, chills, fever and other symptoms of acute thrombocytopenia taking into account the possibility of acute check platelets, disable the anti-platelet drugs, hormone and other drugs, to avoid serious adverse events occur.%目的:探讨P C I术后替罗非班致急性血小板重度减少的临床症状,诊断及处理策略。方法:收集我院2014年1月-2015年5月的3例PCI术后替罗非班致血小板重度减少患者的病例资料,进行回顾性分析。结果:3例患者入院时血小板均在正常范围,PCI术后应用替罗非班静脉泵人,0.5-2小时发生寒颤,高热等症状,急查证实血小板重度减少,及时停用替罗非班及其他抗血小板药物,应用激素等治疗,血小板计数于10小时-5天恢复正常,未发生致命性出血及血栓等不良事件。结论:PCI术后应用替罗非班时应严密观察病情,出现寒颤,高热等症状时应考虑到急性血小板减少的可能,急性查血小板,停用抗血小板药物,应用激素等药物治疗,避免严重不良事件发生。

  17. 大隐静脉曲张手术所致严重血管损伤的治疗%The management of severe vascular injuries caused by stripping of great saphenous vein varicosis

    Institute of Scientific and Technical Information of China (English)

    程志华; 王宏飞; 陈光; 赵文光; 王嘉桔

    2010-01-01

    目的 总结大隐静脉曲张手术导致严重血管损伤并发症的治疗经验.方法 回顾性分析2004年12月至2009年4月收治的4例(5条肢体)大隐静脉曲张手术发生严重血管损伤患者的临床资料.对1条股总动脉末端至胫后动脉上段动脉被剥脱的肢体应用人工血管行动脉重建术,术后肢体坏疽行膝上截肢术;对3条股浅动脉被剥脱10~15 cm的肢体取自体大隐静脉行动脉重建术,术后5 d对1条坏死肢体行膝上截肢术.1条股浅静脉被切除2 cm的肢体取自体大隐静脉行股浅静脉重建术.结果 4例患者中,围手术期无死亡病例,4条动脉损伤肢体中2条肢体(50%)行膝上截肢术;2条肢体(50%)得到保伞,随访12~46个月,平均29个月,血管通畅良好.1例股浅静脉损伤患者恢复良好.结论 提高对医源性血管损伤的认识,术中规范操作,辨明解剖,可以减少和预防严重血管并发症的发生;一旦有血管损伤应早期而有效的处理.%Objective To summarize our experience on the treatment for severe vascular injuries in the operation of great saphenous varicose vein. Methods The clinical data of 4 cases (5 lower limbs) from December 2004 to April 2009 of severe vascular injuries were retrospectively analyzed. For the lower limbs in which from the end of femoral artery to the upper part of posterior tibial artery were stripped, reconstruction operation using blood vessel prothesis was performed, above knee amputation was performed because of limb gangrene. For three limbs in which 10 cm to 15 cm superficial femoral artery were stripped, reconstruction operation using autologous saphenous vein were performed, above knee amputation was performed for one limb 5 days after the operation. For the limb in which 2 cm superficial femoral vein were cut, reconstruction operation using autologous saphenous vein were performed. Results No cases died perioperatively,for four limbs of injuried artery, ampution were performed

  18. Direct observation from sounding of the warming caused by homogeneous freezing in a severe storm%强对流风暴中同质冻结增温的直接探空观测

    Institute of Scientific and Technical Information of China (English)

    徐小红; 余兴; 戴进; 刘贵华; 朱延年; 岳治国

    2011-01-01

    通过两个强对流风暴个例,利用探空资料辅以卫星反演方法,揭示了强对流风暴中同质冻结增温这一事实。探空观测到在强风暴中同质冻结潜热释放造成该层内的空气增温达5℃以上,风暴中强上升气流使云滴没有足够的时间长大,云水向雨水转化和云的晶化会被推到更高的高度,在到达同质冻结高度之前,异质核化过程没有太多的时间消耗大量云水,多数云滴被带到同质冻结高度以上时迅速冻结,造成潜热的集中释放,使周围的空气增温。NOAA卫星观测到的云顶的大量由同质冻结形成的小冰粒子以及过顶现象,进一步证实了同质冻结潜热增温。当探空仪靠近风暴、对%By using the radiosonde data and the satellite retrieval and observation,the phenomenon that the homogeneous freezing latent heat warms the air in severe storms is revealed.It was found that the temperature can increase by as much as 5.0 ℃ within that layer in the severe convective storm because of homogeneous freezing.It is mainly because the updrafts are so strong that the cloud drops have no enough time to grow larger and the conversion of cloud drops to hydrometeors and the glaciation of the cloud are delayed to higher heights.Heterogeneous ice nucleation does not have time to deplete much of the cloud water before reaching the homogeneous glaciation level.Thus most cloud drops are carried to the height of homogenous freezing and are instantly frozen into ice crystals,which releases the latent and warm the ambient air.The phenomenon is also verified by the NOAA satellite observation that small ice particles formed by homogenous freezing on the cloud top and overshooting top.The warm effect of homogeneous freezing could be detected by the sounding when it is close to the storm and the tropopause is close to the height of homogeneous freezing.

  19. 宫腔镜黏膜下子宫肌瘤电切致重度低钠血症临床分析%Severe hyponatremia caused by hysteroscopic resection of submucous uterine fibroids

    Institute of Scientific and Technical Information of China (English)

    褚黎; 张军

    2011-01-01

    Objective To summarize the causes,diagnosis and treatment of the heavy transurethral resection of prostate syndrome (TURP) during the electric rescecting submucous myoma of uterus with uteroscopy.Methods Twelve cases who had the heavy TURP of the 350 cases undergoing electric rescecting submucous myoma of uterus with uteroscopy were retrospectively analyzed.Results The mean operative time was 65 min and the meanliquid volume was 10 000 ml, one of the twelve cases who had the heavy TURP suffered from left heart failure.The serum sodium,blood glucose,the central venous pressure(CVP)and the hemoglobin were significantly different but the serum potassium, serum chloride and the packed cell volume were not significantly different before and after opemtion.11IIe 12 patients were cured and discharged.Conclusions The heavy TURP is the serious complication oft11e electric rescecting submucous myoma of uterus with uteroscopy,which Can cause the high sequela of the mortality rate and the nervous system.%目的 探讨宫腔镜子宫黏膜下子宫肌瘤电切致重度低钠血症的发生、防治.方法 回顾性分析2003年1月至2009年6月北京安贞医院12例宫腔镜子宫黏膜下子宫肌瘤电切术中出现重度低钠血症.结果 同期手术患者350例,其中出现重度低钠血症12例,发生率为3.4%.其中1例出现急性左心衰竭.12例患者手术时间平均为65 min,灌液量平均10 000 ml.患者术中发生稀释性低钠血症(TURP综合征)时血钠、血糖、中心静脉压及血红蛋白与术后相比差异均有统计学意义[分别为(143.2±2.2)mmol/L比(110.3±4.2)mmol/L,(6.2±1.2)mmol/L比(22.2±12.2)mmol/L,(4.2±1.2)cm H2O(1 cm H2O=0.098 kPa)比(16.2±6.2)cm H2O,(134.6±14.5)∥L比(110.1±17.7)∥L,均P<0.01),而血钾、血氯、红细胞压积虽降低,但差异无统计学意义.经积极抢救和治疗,12例患者痊愈出院,预后良好.结论 重度低钠血症的发生是由于手术时间长、灌洗液量大、术中切割

  20. Review on Several Causes of Planning to Trafifc Congestion and Planning Paradigm%交通拥堵的几个规划致因与规划范式检讨

    Institute of Scientific and Technical Information of China (English)

    张奇云; 王树盛

    2015-01-01

    Transportation congestion alleviation is one of the most important tasks of urban and transportation planning. The paper focuses on the topics of the planning compilation of urban and transportation planning, urban space layout and trafifc volume reduction, the matching between transportation system and urban spatial organization, job-housing balance. The causes of planning to trafifc congestion have been analyzed from the point of criticism. And then one new planning paradigm has been presented with four characteristics different from the old one: from excessive emphasis on GDP to coordination among economy, transportation, ecology; from rule-based plan to model-based plan; from one scenario plan to many scenarios compared plan;from blueprint-based plan to progress-based plan.%缓解交通拥堵一直是城市规划、城市交通规划关注的重要课题。以批判视角从城市规划与交通规划的编制机制、城市空间布局与交通减量、交通系统模式与城市空间组织的匹配性以及对职住平衡理念认识的偏差等方面,深层次分析了交通拥堵等城市病的规划致因,并结合对现有城市规划、城市交通规划范式的反思,提出了规划范式变革的4个转向:由唯经济目标转向协同规划、由原则规划转向模型规划、由单一规划转向情景规划、由蓝图规划转向过程规划。