What Is Aplastic Anemia?

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... Home / Anemia Aplastic Anemia Also known as What Is Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is ... heart, heart failure , infections, and bleeding. Severe aplastic anemia can even cause death. Overview Aplastic anemia is ...

Identification of novel microRNA signatures linked to acquired aplastic anemia.

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Hosokawa, Kohei; Muranski, Pawel; Feng, Xingmin; Keyvanfar, Keyvan; Townsley, Danielle M; Dumitriu, Bogdan; Chen, Jichun; Kajigaya, Sachiko; Taylor, James G; Hourigan, Christopher S; Barrett, A John; Young, Neal S

2015-12-01

Emerging evidence indicates that microRNA control and modulate immunity. MicroRNA have not been investigated in acquired aplastic anemia, a T-cell-mediated immune disease. Analysis of 84 microRNA expression levels in CD4(+) and CD8(+) T cells of patients with aplastic anemia revealed concurrent down-regulation of miR-126-3p, miR-145-5p, miR-223-3p, and miR-199a-5p (>3-fold change, Paplastic anemia compared to other hematologic disorders. MiR-126-3p and miR-223-3p were down-regulated in CD4(+) T effector memory cells, and miR-126-3p, miR-145-5p, and miR-223-3p were down-regulated in CD8(+) T effector memory and terminal effector cells. Successful immunosuppressive therapy was associated with restoration to normal expression levels of miR-126-3p, miR-145-5p, and miR-223-3p (>2-fold change, Paplastic anemia patients, MYC and PIK3R2 were up-regulated and proved to be targets of miR-145-5p and miR-126-3p, respectively. MiR-126-3p and miR-145-5p knockdown promoted proliferation and increased interferon-γ and granzyme B production in both CD4(+) and CD8(+) T cells. Our work describes previously unknown regulatory roles of microRNA in T-cell activation in aplastic anemia, which may open a new perspective for development of effective therapy. Clinicaltrials.gov identifier: NCT 01623167. Copyright© Ferrata Storti Foundation.

Hematopoietic stem cell transplantation for acquired aplastic anemia

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Georges, George E.; Storb, Rainer

2016-01-01

Purpose of review There has been steady improvement in outcomes with allogeneic bone marrow transplantation (BMT) for severe aplastic anemia (SAA), due to progress in optimization of the conditioning regimens, donor hematopoietic cell source and supportive care. Here we review recently published data that highlight the improvements and current issues in the treatment of SAA. Recent findings Approximately one-third of AA patients treated with immune suppression therapy (IST) have acquired mutations in myeloid cancer candidate genes. Because of the greater probability for eventual failure of IST, human leukocyte antigen (HLA)-matched sibling donor BMT is the first-line of treatment for SAA. HLA-matched unrelated donor (URD) BMT is generally recommended for patients who have failed IST. However, in younger patients for whom a 10/10-HLA-allele matched URD can be rapidly identified, there is a strong rationale to proceed with URD BMT as first-line therapy. HLA-haploidentical BMT using post-transplant cyclophosphamide (PT-CY) conditioning regimens, is now a reasonable second-line treatment for patients who failed IST. Summary Improved outcomes have led to an increased first-line role of BMT for treatment of SAA. The optimal cell source from an HLA-matched donor is bone marrow. Additional studies are needed to determine the optimal conditioning regimen for HLA-haploidentical donors. PMID:27607445

How I treat acquired aplastic anemia

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Young, Neal S.

2012-01-01

Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive biologics and drugs, and supportive care. However, management of SAA patients remains challenging, both acutely in addressing the immediate consequences of pancytopenia and in the long term because of the disease's natural history and the consequences of therapy. Recent insights into pathophysiology have practical implications. We review key aspects of differential diagnosis, considerations in the choice of first- and second-line therapies, and the management of patients after immunosuppression, based on both a critical review of the recent literature and our large personal and research protocol experience of bone marrow failure in the Hematology Branch of the National Heart, Lung, and Blood Institute. PMID:22517900

Circulating thrombopoietin levels in normal healthy blood donors and in aplastic anemia patients in relation to disease severity

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Abhay Singh

2015-01-01

Full Text Available Background: Thrombopoietin (TPO is the key hematopoietic growth factor regulating the production of platelets from bone marrow megakaryocytes and maintaining platelet hemostasis. This study was done to find any relationship between the levels of thrombopoietin and the severity of disease in patients with aplastic anemia. Materials and Methods: Serum samples were collected from 52 patients with a confirmed diagnosis of aplastic anemia and 45 normal healthy blood donors of both sexes over a period of 2 years, and TPO was estimated by using commercially available TPO-specific-enzyme-linked immunosorbent assay. Results: The median TPO level of 1190 pg/ml (range 625-7651 pg/ml in aplastic anemia patients was significantly higher than the median TPO level of 121.1 pg/ml (81.25-237.7 pg/ml in normal healthy blood donors (P = 0.000. No significant difference was observed in TPO levels of male and female patients (P = 0.453. The median TPO concentrations observed in very severe aplastic anemia, severe aplastic anemia, and nonsevere aplastic anemia were 2765 pg/ml (range 625-6451 pg/ml, 1190 pg/ml (range 672.1-7651 pg/ml, and 1111.5 pg/ml (range 761.1-2289.2 pg/ml, respectively. TPO in patients of very severe aplastic anemia was significantly higher than patients of nonsevere aplastic anemia (P = 0.043, with no significant relation among rest of the groups. Discussion: TPO levels in aplastic anemia patients were significantly higher than in healthy blood donors; however, in aplastic anemia patients TPO levels were significantly higher only in patients with very severe disease.

Disrupted lymphocyte homeostasis in hepatitis‐associated acquired aplastic anemia is associated with short telomeres

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Babushok, Daria V.; Grignon, Anne‐Laure; Li, Yimei; Atienza, Jamie; Xie, Hongbo M.; Lam, Ho‐Sun; Hartung, Helge; Bessler, Monica

2016-01-01

Hepatitis‐associated aplastic anemia (HAA) is a variant of acquired aplastic anemia (AA) in which immune‐mediated bone marrow failure (BMF) develops following an acute episode of seronegative hepatitis. Dyskeratosis congenita (DC) is an inherited BMF syndrome characterized by the presence of short telomeres, mucocutaneous abnormalities, and cancer predisposition. While both conditions may cause BMF and hepatic impairment, therapeutic approaches are distinct, making it imperative to establish the correct diagnosis. In clinical practice, lymphocyte telomere lengths (TL) are used as a first‐line screen to rule out inherited telomeropathies before initiating treatment for AA. To evaluate the reliability of TL in the HAA population, we performed a retrospective analysis of TL in 10 consecutively enrolled HAA patients compared to 19 patients with idiopathic AA (IAA). HAA patients had significantly shorter telomeres than IAA patients (P = 0.009), including four patients with TL at or below the 1st percentile for age‐matched controls. HAA patients had no clinical features of DC and did not carry disease‐causing mutations in known genes associated with inherited telomere disorders. Instead, short TLs were significantly correlated with severe lymphopenia and skewed lymphocyte subsets, features characteristic of HAA. Our results indicate the importance of caution in the interpretation of TL measurements in HAA, because, in this patient population, short telomeres have limited specificity. Am. J. Hematol. 91:243–247, 2016. © 2015 The Authors. American Journal of Hematology Published by Wiley Periodicals, Inc. PMID:26615915

Successful allogeneic stem cells transplantation in severe aplastic anaemia complicated by dengue fever

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Ullah, K.; Satti, T.M.; Ahmed, P.; Raza, A.; Akhtar, F.M.; Tariq, W.U.Z.

2007-01-01

Aplastic anaemia is characterized by severe compromise of haematopoiesis and hypocellular bone marrow. Haemorrhagic episodes in patients with aplastic anemia occur usually secondary to thrombocytopenia and require frequent support with platelet concentrates and other blood products. Infection with dengue virus (particularly dengue sero type-2 of South Asian genotype) is associated with dengue haemorrhagic fever. Dengue infection further worsens the disease process in patients with aplastic anaemia due to uncontrolled haemorrhagic diathesis and major organ failure, which may prove fatal in these already immunocompromised patients, if not treated in time. Recent epidemics of dengue haemorrhagic fever has not only affected the southern region of our country but also spread to other areas of the country. With this background, we report a case of aplastic anaemia complicated by dengue haemorrhagic fever who achieved successful engraftment after allogeneic stem cell transplantation from sibling brother and is having normal healthy post transplant life. (author)

Clonal hematopoiesis in acquired aplastic anemia

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Ogawa, Seishi

2016-01-01

Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolut...

[Favorable current prognosis after HLA-identical bone marrow transplantation for children with required severe aplastic anemia; evaluation of 30 years of bone marrow transplantation at the Leiden University Medical Center

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Steekelenburg, M. van; Weel-Sipman, M.H. van; Zwinderman, A.H.; Hoogerbrugge, P.M.; Vossen, J.M.J.J.; Egeler, R.M.

2002-01-01

OBJECTIVE: To evaluate the results of 30 years of allogeneic HLA-identical bone marrow transplantation (BMT) as the treatment for children with acquired severe aplastic anaemia. DESIGN: Retrospective, descriptive. METHOD: Of all patients who underwent an HLA-identical sibling-donor BMT for severe

Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia.

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Kekre, Natasha; Zhang, Ying; Zhang, Mei-Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem; Deeg, H Joachim; Kapoor, Neena; Lee, Jong-Wook; Nakamura, Ryotaro; Pulsipher, Michael A; Eapen, Mary; Antin, Joseph H

2017-07-01

For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using human leukocyte antigen (HLA)-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft. There were no differences in hematopoietic recovery by type of antithymocyte globulin. Among recipients of HLA-matched sibling transplants, day 100 incidence of acute (17% versus 6%, P aplastic anemia. Copyright© 2017 Ferrata Storti Foundation.

A successful desensitization protocol for horse-derived antithymocyte globulin in severe aplastic anemia.

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Demir, Esen; Cigerci Günaydın, Nurşen; Karadaş, Nihal; Gülen, Figen; Tanac, Remziye; Yılmaz, Deniz

2015-03-01

Horse antithymocyte globulin (h-ATG) (ATGAM(®) ) is the first choice of treatment in very severe patients with aplastic anemia who do not have any HLA matched sibling donor. h-ATG is a heterologous serum that may cause anaphylaxis. Alternative treatment strategies must be planned in case of hypersensitivity. Desensitization must be considered in patients without an alternative treatment of choice. We aimed to present the h-ATG desensitization protocol and consider its effectiveness in patients with aplastic anemia who are hypersensitized with h-ATG and do not have an alternative treatment of choice. Skin prick tests were performed with non-diluted solution in eight very severe patients with aplastic anemia who are followed up in Ege University Children's Hospital. Although skin prick test was found negative in these eight patients, different dilution h-ATG intradermal tests were performed and found positive in all patients. h-ATG desensitization program was started to these hypersensitized patients. Desensitization program was started to six male and two female very severe patients with aplastic anemia whose ages were between seven and 19 yr (median: 12.9 yr). All of the patients completed the desensitization program. While local reaction was seen in two patients, systemic reaction was seen in one patient and late reaction was seen in one patient during and after desensitization program. A successful desensitization program with h-ATG in children with aplastic anemia is presented. Even though there is not an exposure before to such high allergy potential heterologous serum, skin tests should be performed and desensitization must be started to patients who are hypersensitized to h-ATG. As the expected effectiveness of the treatment is so much, the desensitization protocol can be carried out safely and effectively with trained stuff although allergic reactions can be seen. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Clinical significance of acquired somatic mutations in aplastic anaemia.

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Marsh, J C W; Mufti, G J

2016-08-01

Aplastic anaemia (AA) is frequently associated with other disorders of clonal haemopoiesis such as paroxysmal nocturnal haemoglobinuria (PNH), myelodysplastic syndrome (MDS) and T-large granular lymphocytosis. Certain clones may escape the immune attack within the bone marrow environment and proliferate and attain a survival advantage over normal haemopoietic stem cells, such as trisomy 8, loss of heterozygosity of short arm of chromosome 6 and del13q clones. Recently acquired somatic mutations (SM), excluding PNH clones, have been reported in around 20-25 % of patients with AA, which predispose to a higher risk of later malignant transformation to MDS/acute myeloid leukaemia. Furthermore, certain SM, such as ASXL1 and DNMT3A are associated with poor survival following immunosuppressive therapy, whereas PIGA, BCOR/BCORL1 predict for good response and survival. Further detailed and serial analysis of the immune signature in AA is needed to understand the pathogenetic basis for the presence of clones with SM in a significant proportion of patients.

Bone marrow transplantation in severe aplastic anemia and acute or chronic leukemia

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Schaefer, U W; Mahmoud, H K; Beelen, D W; Hoffmann, W; Becher, R; Schmidt, C G; Bamberg, M; Quast, U; Haralambie, E; Linzenmeier, G

1986-04-01

In Essen 121 bone marrow transplantations were carried out. The indications were severe aplastic anemia, acute leukemia in relapse, acute leukemia in remission or chronic myeloid leukemia. The conditioning regimen consisted of cyclophosphamide or the combination of cyclophosphamide and total body irradiation. All patients were treated under strict gnotobiotic care. To mitigate the risk of CMV infections intravenous CMV-hyperimmunoglobulin and CMV-negative blood products have been applied routinely since two years. MTX was used as prophylaxis against GVH-disease. In case of severe aplastic anemia 13 patients (72%) are still alive with a median observation time of 24 months. In the prognostically unfavourable group of acute leukemia in relapse only one patient showed long term survival. In this patient leukemic relapse occurred six years after transplantation. The survival rate of AML patients grafted during the first remission is 55% with a median observation time of 40 months. For patients grafted in the first consecutive remission of ALL the survival rate is 42% with a maximal observation time of 29 months. Out of 37 patients grafted because of CML, eight were in an advanced stage of the disease. 13 patients are still alive, the maximal observation time is 37 months. The overall incidence of GVHD in patients at risk was 28% in aplastic anemia, 26% in AML, 9% in ALL and 63% in CML. In aplastic anemia no patient developed an interstitial pneumonia. In leukemia the risk of fatal interstitial pneumonia was 34%.

Bone marrow transplantation in severe aplastic anemia and acute or chronic leukemia

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Schaefer, U.W.; Mahmoud, H.K.; Beelen, D.W.; Hoffmann, W.; Becher, R.; Schmidt, C.G.; Bamberg, M.; Quast, U.; Haralambie, E.; Linzenmeier, G.; Stollmann, B.; Grosse-Wilde, H.; Richter, H.J.; Hantschke, D.; Henneberg, K.; Luboldt, W.

1986-01-01

In Essen 121 bone marrow transplantations were carried out. The indications were severe aplastic anemia, acute leukemia in relapse, acute leukemia in remission or chronic myeloid leukemia. The conditioning regimen consisted of cyclophosphamide or the combination of cyclophosphamide and total body irradiation. All patients were treated under strict gnotobiotic care. To mitigate the risk of CMV infections intravenous CMV-hyperimmunoglobulin and CMV-negative blood products have been applied routinely since two years. MTX was used as prophylaxis against GVH-disease. In case of severe aplastic anemia 13 patients (72%) are still alive with a median observation time of 24 months. In the prognostically unfavourable group of acute leukemia in relapse only one patient showed long term survival. In this patient leukemic relapse occurred six years after transplantation. The survival rate of AML patients grafted during the first remission is 55% with a median observation time of 40 months. For patients grafted in the first consecutive remission of ALL the survival rate is 42% with a maximal observation time of 29 months. Out of 37 patients grafted because of CML, eight were in an advanced stage of the disease. 13 patients are still alive, the maximal observation time is 37 months. The overall incidence of GVHD in patients at risk was 28% in aplastic anemia, 26% in AML, 9% in ALL and 63% in CML. In aplastic anemia no patient developed an interstitial pneumonia. In leukemia the risk of fatal interstitial pneumonia was 34%. (orig.) [de

Outcome of Cyclosporine Monotherapy in Patients of Aplastic Anemia: Experience of a Tertiary Care Hospital in Eastern India.

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Mandal, Prakas Kumar; Baul, Suvraneel; Dolai, Tuphan Kanti; De, Rajib; Chakrabarti, Prantar

2017-03-01

Immune suppression is a crucial pillar for treatment of aplastic anemia. Cyclosporine monotherapy is an easily available, affordable therapeutic option with good safety profile. This prospective study was conducted over a period of 2 years from June 2012 to July 2014. The diagnosis and response to treatment of aplastic anemia was established as per published criteria. Follow up was done at 3 and 6 months in order to assess the response. 57 patients of acquired aplastic anemia with median age of 37 years (6 to 81 years) were included in the study. 35 (62 %) cases were severe aplastic anemai, 16 (28 %) non severe aplastic anemia and 6 (10 %) were very severe aplastic anemia. At 3 months overall response rate (OR) was 7 (14 %) and at 6 months the OR rate of 11 (19.6 %) was achieved. Transiently raised creatinine, liver function abnormality and gum hypertrophy were the main side effects observed in this cohort. Oral cyclosporine monotherapy at dose of 5 mg/kg/day is a relatively safe treatment option for resource poor patients with aplastic anemia.

Total lymphoid irradiation and cyclophosphamide conditioning prior to bone marrow transplantation for patients with severe aplastic anemia

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Ramsay, N.K.; Kim, T.H.; McGlave, P.; Goldman, A.; Nesbit, M.E. Jr.; Krivit, W.; Woods, W.G.; Kersey, J.H.

1983-01-01

A preparative regimen, consisting of total lymphoid irradiation and cyclophosphamide, was utilized in 40 patients with severe aplastic anemia undergoing allogeneic marrow transplantation. This regimen was successful in decreasing rejection in these previously transfused patients, as only one patient rejected the marrow graft. Twenty-nine of the 40 transplanted patients are surviving from 1.5 to 59 mo, with a median follow-up of 24 mo. The actuarial survival rate for these heavily transfused patients with aplastic anemia is 72% at 2 yr. This preparative regimen is extremely effective in decreasing rejection following transplantation for severe aplastic anemia. Future efforts in this area must be aimed at the elimination of graft-versus-host disease and control of fatal infections

Aplastic anemia: clinico haematological features, treatment and outcome analysis

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Wali, R.; Fadoo, Z.; Naqvi, M.A.

2011-01-01

To determine the clinico haematological features, treatment and outcome of children diagnosed with aplastic anemia at a single institution. Study Design: Observational study. Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 1999 till December 2008. Methodology: Medical records of children aged less than 15 years of age diagnosed with aplastic anemia were reviewed. Clinico haematological features, treatment and its response to therapy and outcome were recorded. Results were described in percentages. Results: Ninety patients were diagnosed to have aplastic anemia (AA); 65 were male during the study period. Age ranged from 1 to 15 years. Fever in 65 patients (72.2%), pallor in 53 (58.8%), skin bleeding in 49 (54.4%) and epistaxis in 31(34.4%) were the most common and frequent presenting features. Congenital (Fanconi's) anemia was found in 15 (16.6%) and acquired idiopathic in 75 (83.4%) of patients. Very severe aplastic anemia (VSAA) was seen in 29 (32.2%), 26 (28.9%) had severe AA and 17 (18.9%) had moderate AA. Eight patients (8.9%) underwent haematopoietic stem cell transplantation (HSCT), 12 (13.3%) received immunosuppressive therapy (IST) and 70 patients (77.7%) received other and supportive therapy. Five (62.5%) patients showed complete response to HSCT and 3 (37.5%) failed to engraft. IST showed complete response in 3 (25%), partial response in 5 (41.6%) and no response in 4 (33.3%). Twenty two patients (24.4%) expired either due to infection in 16 (72.7%, fungal in 6, bacterial in 10) and intracranial haemorrhage in 6 (27.3%) cases. Conclusion: Majority of cases with AA were acquired and idiopathic in etiology. VSAA and SAA were frequent. Response to HSCT and IST was sub-optimal. (author)

Deep sequencing and flow cytometric characterization of expanded effector memory CD8+CD57+ T cells frequently reveals T-cell receptor Vβ oligoclonality and CDR3 homology in acquired aplastic anemia.

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Giudice, Valentina; Feng, Xingmin; Lin, Zenghua; Hu, Wei; Zhang, Fanmao; Qiao, Wangmin; Ibanez, Maria Del Pilar Fernandez; Rios, Olga; Young, Neal S

2018-05-01

Oligoclonal expansion of CD8 + CD28 - lymphocytes has been considered indirect evidence for a pathogenic immune response in acquired aplastic anemia. A subset of CD8 + CD28 - cells with CD57 expression, termed effector memory cells, is expanded in several immune-mediated diseases and may have a role in immune surveillance. We hypothesized that effector memory CD8 + CD28 - CD57 + cells may drive aberrant oligoclonal expansion in aplastic anemia. We found CD8 + CD57 + cells frequently expanded in the blood of aplastic anemia patients, with oligoclonal characteristics by flow cytometric Vβ usage analysis: skewing in 1-5 Vβ families and frequencies of immunodominant clones ranging from 1.98% to 66.5%. Oligoclonal characteristics were also observed in total CD8 + cells from aplastic anemia patients with CD8 + CD57 + cell expansion by T-cell receptor deep sequencing, as well as the presence of 1-3 immunodominant clones. Oligoclonality was confirmed by T-cell receptor repertoire deep sequencing of enriched CD8 + CD57 + cells, which also showed decreased diversity compared to total CD4 + and CD8 + cell pools. From analysis of complementarity-determining region 3 sequences in the CD8 + cell pool, a total of 29 sequences were shared between patients and controls, but these sequences were highly expressed in aplastic anemia subjects and also present in their immunodominant clones. In summary, expansion of effector memory CD8 + T cells is frequent in aplastic anemia and mirrors Vβ oligoclonal expansion. Flow cytometric Vβ usage analysis combined with deep sequencing technologies allows high resolution characterization of the T-cell receptor repertoire, and might represent a useful tool in the diagnosis and periodic evaluation of aplastic anemia patients. (Registered at clinicaltrials.gov identifiers: 00001620, 01623167, 00001397, 00071045, 00081523, 00961064 ). Copyright © 2018 Ferrata Storti Foundation.

Worse outcome and more chronic GVHD with peripheral blood progenitor cells than bone marrow in HLA-matched sibling donor transplants for young patients with severe acquired aplastic anemia.

NARCIS (Netherlands)

Schrezenmeier, H.; Passweg, J.R.; Marsh, J.C.; Bacigalupo, A.; Bredeson, C.N.; Bullorsky, E.; Camitta, B.M.; Champlin, R.E.; Gale, R.P.; Fuhrer, M.; Klein, J.P.; Locasciulli, A.; Oneto, R.; Schattenberg, A.V.M.B.; Socie, G.; Eapen, M.

2007-01-01

We analyzed the outcome of 692 patients with severe aplastic anemia (SAA) receiving transplants from HLA-matched siblings. A total of 134 grafts were peripheral blood progenitor cell (PBPC) grafts, and 558 were bone marrow (BM) grafts. Rates of hematopoietic recovery and grades 2 to 4 chronic

Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

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Barone, Angelica; Lucarelli, Annunziata; Onofrillo, Daniela; Verzegnassi, Federico; Bonanomi, Sonia; Cesaro, Simone; Fioredda, Francesca; Iori, Anna Paola; Ladogana, Saverio; Locasciulli, Anna; Longoni, Daniela; Lanciotti, Marina; Macaluso, Alessandra; Mandaglio, Rosalba; Marra, Nicoletta; Martire, Baldo; Maruzzi, Matteo; Menna, Giuseppe; Notarangelo, Lucia Dora; Palazzi, Giovanni; Pillon, Marta; Ramenghi, Ugo; Russo, Giovanna; Svahn, Johanna; Timeus, Fabio; Tucci, Fabio; Cugno, Chiara; Zecca, Marco; Farruggia, Piero; Dufour, Carlo; Saracco, Paola

2015-06-01

Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary, evidence-based document issued by a group of pediatric hematologists was discussed, modified and approved during a series of "Consensus Conferences" according to procedures previously validated by the AIEOP Board. The guidelines highlight the importance of referring pediatric patients with AA to pediatric centers with long experience in diagnosis, differential diagnosis, management, supportive care and follow-up of AA. Copyright © 2015. Published by Elsevier Inc.

Severe aplastic anaemia and Grave's disease in a paediatric patient.

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Kumar, Manjusha; Goldman, Jeffrey

2002-07-01

Severe aplastic anaemia (SAA) is considered to be an autoimmune disorder affecting the haematopoietic cells and most often is idiopathic. An association between SAA and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus and thyroid disorders. We describe the first paediatric patient with chronic relapsing SAA and Grave's disease. We discuss the difficulty in diagnosis of Grave's disease, the possibility of its manifestation due to withdrawal of immunosuppressants, and issues to consider in the treatment of this disease in the setting of bone marrow failure.

[Avascular necrosis of the femoral head in a patient with severe aplastic anaemia].

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Plichta, Piotr; Pawelec, Katarzyna

2011-01-01

Avascular necrosis (AVN) is a pathological condition associated with numerous processes. Most frequent causes of ischaemia of the femoral head include trauma, corticosteroid therapy, radiation therapy, alcoholism, Gaucher's disease, systemic lupus erythematosus, rheumatoid arthritis. Corticosteroid therapy is considered the most frequent risk factor for developing avascular necrosis. In this paper we report a case of a 19-year old female patient who developed avascular necrosis of the right femoral head following treatment of severe aplastic anaemia. Clinical symptoms included groin pain and fever, painful abduction and internal rotation, tenderness over the right hip joint. The plain X-ray was not specific. However magnetic resonance imaging of the hip revealed changes characteristic for avascular necrosis. Conservative treatment was administrated. Two years after the onset of first clinical symptoms the patient is able to walk without pain, although the range of motion of the right hip joint is partially diminished. We evaluated the possible risk factors of AVN, diagnostic methods and prognosis. The onset of avascular necrosis should be considered as one of the relevant complications in patients with severe aplastic anaemia following immunosuppressive treatment.

Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia

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Kekre, Natasha; Zhang, Ying; Zhang, Mei Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem M.; Joachim Deeg, H.; Kapoor, Neena; Lee, Jong Wook; Nakamura, Ryotaro; Pulsipher, Michael A.; Eapen, Mary; Antin, Joseph H

2017-01-01

For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin

APLASTIC ANEMIA

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Ni Made Dharma Laksmi

2013-07-01

Full Text Available Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 Aplastic Anemia describes a disorder of the clinical syndrome is marked by a deficiency of red blood cells, neutrophils, monocytes and platelets in the absence of other forms of bone marrow damage. Aplastic anemia is classified as a rare disease in developed countries the incidence of 3-6 cases / 1 million inhabitants / year. The exact cause of someone suffering from aplastic anemia also can not be established with certainty, but there are several sources of potential risk factors. Prognosis or course of the disease varies widely aplastic anemia, but without treatment generally gives a poor prognosis /* 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:"Times New Roman"; mso-bidi-theme-font:minor-bidi;}

Graft failure following bone marrow transplantation for severe aplastic anemia risk factors and treatment results

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Champlin, R.E.; Horowitz, M.M.; Bekkum, D.W. van; Camitta, B.M. Elfenbein, G.E.; Gale, R.P.; Gluckman, E.; Good, R.A.; Rimm, A.A. Rozman, C.; Speck, B. Bortin, M.M

1989-01-01

Graft failure was analyzed in 625 patients receiving allogeneic bone marrow transplants from HLA-identical sibling donors as treatment for severe aplastic anemia. Sixty-eight (11%) had no or only transient engraftment. Second bone marrow transplants were successful in achieving extended survival in

[Results of immunosupressive therapy in children with severe aplastic anaemia. Report of the Polish Paediatric Haematology Group].

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Pawelec, Katarzyna; Matysiak, Michał; Niewiadomska, Edyta; Rokicka-Milewska, Roma; Kowalczyk, Jerzy; Stefaniak, Jolanta; Balwierz, Walentyna; Załecka-Czerpko, Ewa; Chybicka, Alicja; Szmyd, Krzysztof; Sońta-Jakimczyk, Danuta; Bubała, Halina; Krauze, Agnieszka; Wysocki, Mariusz; Kurylak, Andrzej; Wachowiak, Jacek; Grund, Grzegorz; Młynarski, Wojciech; Bulas, Monika; Krawczuk-Rybak, Maryna; Leszczyńska, Elzbieta; Urasiński, Tomasz; Peregud-Pogorzelski, Jarosław; Balcerska, Anna; Włazłowski, Marek

2008-01-01

Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given. evaluation of results of immunosupressive therapy in children with severe aplastic anaemia. SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy. complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients. 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia

Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000-2011.

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Vaht, Krista; Göransson, Magnus; Carlson, Kristina; Isaksson, Cecilia; Lenhoff, Stig; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per; Brune, Mats; Andersson, Per-Ola

2017-10-01

A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70-80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000-2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06-2.64) cases per million inhabitants per year. Median age was 60 years (range: 2-92), and median follow up was 76 (0-193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0-18 years, 90.5% in patients aged 19-39 years, 70.7% in patients aged 40-59 years, and 38.1% in patients aged ≥60 years. Multivariate analysis showed that age (both 40-59 and ≥60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients ≥60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted. Copyright© 2017 Ferrata Storti Foundation.

Upfront haploidentical transplant for acquired severe aplastic anemia: registry-based comparison with matched related transplant.

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Xu, Lan-Ping; Jin, Song; Wang, Shun-Qing; Xia, Ling-Hui; Bai, Hai; Gao, Su-Jun; Liu, Qi-Fa; Wang, Jian-Min; Wang, Xin; Jiang, Ming; Zhang, Xi; Wu, De-Pei; Huang, Xiao-Jun

2017-01-21

Haploidentical donor (HID) hematopoietic stem cell transplantation (HSCT) is an alternative treatment method for severe aplastic anemia (SAA) patients lacking suitable identical donors and those who are refractory to immunosuppressive therapy (IST). The current study evaluated the feasibility of upfront haploidentical HSCT in SAA patients. We conducted a multicenter study based on a registry database. One hundred fifty-eight SAA patients who underwent upfront transplantation between June 2012 and September 2015 were enrolled. Eighty-nine patients had haploidentical donors (HIDs), and 69 had matched related donors (MRDs) for HSCT. The median times for myeloid engraftment in the HID and MRD cohorts were 12 (range, 9-20) and 11 (range, 8-19) days, with a cumulative incidence of 97.8 and 97.1% (P = 0.528), respectively. HID recipients had an increased cumulative incidence of grades II-IV acute graft-versus-host disease (aGVHD) (30.3 vs. 1.5%, P < 0.001), grades III-IV aGVHD (10.1 vs. 1.5%, P = 0.026), and chronic GVHD (cGVHD) (30.6 vs. 4.4%, P < 0.001) at 1 year but similar extensive cGVHD (3.4 vs. 0%, P = 0.426). The three-year estimated overall survival (OS) rates were 86.1 and 91.3% (P = 0.358), while the three-year estimated failure-free survival (FFS) rates were 85.0 and 89.8% (P = 0.413) in the HID and MRD cohorts, respectively. In multivariate analysis, survival outcome for the entire population was significantly adversely associated with increased transfusions and poor performance status pre-SCT. We did not observe differences in primary engraftment and survival outcomes by donor type. Haploidentical SCT as upfront therapy was an effective and safe option for SAA patients, with favorable outcomes in experienced centers.

[Chronologic analysis of clonal evolution in acquired aplastic anemia and sMDS].

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Yoshizato, Tetsuichi

2016-04-01

Acquired aplastic anemia (AA) is a prototype of idiopathic bone marrow failure, which is caused by immune-mediated destruction of hematopoietic progenitors but is also characterized by frequent evolution to clonal myeloid disorders, such as myelodysplastic syndromes or acute myeloid leukemia. However, the chronological behavior of the clonality and its link to myelodysplastic syndrome or acute myeloid leukemia has not been fully explored. To define the clonality and its chronological behavior in AA, we performed targeted sequencing (N=439) in cases with AA. Somatic mutations were detected in 1/3 of our cases. Mutations were most frequently found in DNMT3A, followed by BCOR, PIGA and ASXL1. The prevalence of mutations increased with age. The clone sizes in DNMT3A and ASXL1 were prone to increase, whereas those of BCOR and PIGA were more likely to decrease or remain stable. Mutations in PIGA, BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and more favorable survival. On the other hand, other mutations were associated with worse outcomes. The chronological dynamics of clonality showed marked variability and were not necessarily associated with prognosis.

APLASTIC ANEMIA AND VIRAL HEPATITIS

Directory of Open Access Journals (Sweden)

Laura Cudillo

2009-11-01

Liver histology is characterized by T cell infiltrating the parenchyma as reported in acute hepatitis. Recently in HAA it has been demonstrated intrahepatic  and blood lymphocytes with  T cell repertoire similar to that of confirmed viral acute hepatitis. The expanded T cell clones return to a normal distribution after response to immunosuppressive treatment, suggesting the antigen or T cell clearance. Therapeutic options are the same as acquired aplastic anemia.

Somatic Mutations and Clonal Hematopoiesis in Aplastic Anemia.

Science.gov (United States)

Yoshizato, Tetsuichi; Dumitriu, Bogdan; Hosokawa, Kohei; Makishima, Hideki; Yoshida, Kenichi; Townsley, Danielle; Sato-Otsubo, Aiko; Sato, Yusuke; Liu, Delong; Suzuki, Hiromichi; Wu, Colin O; Shiraishi, Yuichi; Clemente, Michael J; Kataoka, Keisuke; Shiozawa, Yusuke; Okuno, Yusuke; Chiba, Kenichi; Tanaka, Hiroko; Nagata, Yasunobu; Katagiri, Takamasa; Kon, Ayana; Sanada, Masashi; Scheinberg, Phillip; Miyano, Satoru; Maciejewski, Jaroslaw P; Nakao, Shinji; Young, Neal S; Ogawa, Seishi

2015-07-02

In patients with acquired aplastic anemia, destruction of hematopoietic cells by the immune system leads to pancytopenia. Patients have a response to immunosuppressive therapy, but myelodysplastic syndromes and acute myeloid leukemia develop in about 15% of the patients, usually many months to years after the diagnosis of aplastic anemia. We performed next-generation sequencing and array-based karyotyping using 668 blood samples obtained from 439 patients with aplastic anemia. We analyzed serial samples obtained from 82 patients. Somatic mutations in myeloid cancer candidate genes were present in one third of the patients, in a limited number of genes and at low initial variant allele frequency. Clonal hematopoiesis was detected in 47% of the patients, most frequently as acquired mutations. The prevalence of the mutations increased with age, and mutations had an age-related signature. DNMT3A-mutated and ASXL1-mutated clones tended to increase in size over time; the size of BCOR- and BCORL1-mutated and PIGA-mutated clones decreased or remained stable. Mutations in PIGA and BCOR and BCORL1 correlated with a better response to immunosuppressive therapy and longer and a higher rate of overall and progression-free survival; mutations in a subgroup of genes that included DNMT3A and ASXL1 were associated with worse outcomes. However, clonal dynamics were highly variable and might not necessarily have predicted the response to therapy and long-term survival among individual patients. Clonal hematopoiesis was prevalent in aplastic anemia. Some mutations were related to clinical outcomes. A highly biased set of mutations is evidence of Darwinian selection in the failed bone marrow environment. The pattern of somatic clones in individual patients over time was variable and frequently unpredictable. (Funded by Grant-in-Aid for Scientific Research and others.).

Diabetes mellitus caused by secondary hemochromatosis after multiple blood transfusions in 2 patients with severe aplastic anemia

Directory of Open Access Journals (Sweden)

Hyun Jin Kim

2017-03-01

Full Text Available Hemochromatosis is an inherited or secondary disorder caused by excessive iron storage leading to multiple organ damage. We describe 2 patients with diabetes mellitus caused by hemochromatosis secondary to multiple blood transfusions due to severe aplastic anemia. Subject 1, who was diagnosed with severe aplastic anemia at 15 years of age, received multiple red blood cell transfusions before he underwent autologous peripheral blood stem cell transplantation (PBSCT at 22 years of age. At 21 years of age, hyperglycemia was detected with increased hemoglobin A1c and serum ferritin levels, 9.7% and 12,910 ng/mL (normal range, 20–320 ng/mL, respectively. The 24-hour urine C-peptide level was normal with negative antiglutamic acid decarboxylase antibody. Subsequently, metformin and an iron-chelating agent were administered. However, an intensive insulin regimen was necessary 2 years after the onset of diabetes. Subject 2, who was diagnosed with severe aplastic anemia at 2 years of age, received multiple blood transfusions until she underwent haploidentical PBSCT at 13 years of age. At 11 years of age, she developed diabetes mellitus with a high serum ferritin level (12,559.8 ng/mL. She is currently 18 years old and has been treated with an intensive insulin regimen and estrogen/progesterone replacement therapy because of hypogonadotropic hypogonadism. It is presumed that the loss of insulin secretory capacity and insulin resistance played a role in the pathogenesis of diabetes mellitus due to hemochromatosis in these cases.

APLASTIC ANEMIA AND VIRAL HEPATITIS

Directory of Open Access Journals (Sweden)

Laura Cudillo

2009-11-01

Full Text Available

Acquired aplastic anemia(aAA is a severe and rare disease, characterized by hematopoietic bone marrow failure and peripheral cytopenia. The pathophysiology is immune mediated in most cases, activated T1 lymphocytes have been identified as effector cells . The disease can be successfully treated with combined immunosuppressive therapy or allogeneic hematopoietic stem cell transplantation.

Hepatitis-associated aplastic anemia (HAA  is a syndrome of bone marrow failure following the development of acute seronegative hepatitis. HAA syndrome most often affects young males who presented severe pancytopenia two to three months after an episode of acute hepatitis. The clinical course of hepatitis is more frequently benign but a fulminant severe course is also described. The bone marrow failure can be explosive and severe and it is usually fatal if untreated, no correlations have been observed between severity of hepatitis and AA.

In none of the  studies a specific virus could be identified and most cases are seronegative for known hepatitis viruses. The clinical characteristics  and response to immunotherapy indicate a central role for immune-mediated mechanism in the pathogenesis of HAA. The initial

Incidence and outcome of acquired aplastic anemia: real-world data from patients diagnosed in Sweden from 2000–2011

Science.gov (United States)

Vaht, Krista; Göransson, Magnus; Carlson, Kristina; Isaksson, Cecilia; Lenhoff, Stig; Sandstedt, Anna; Uggla, Bertil; Winiarski, Jacek; Ljungman, Per; Brune, Mats; Andersson, Per-Ola

2017-01-01

A plastic anemia is a rare life-threatening disease. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, the outcome has improved considerably, and the 5-year survival is reported to be 70–80% in selected patient cohorts. Yet, contemporary population-based data on incidence and survival are lacking. We performed a national retrospective study to determine the incidence, treatment, and survival of patients with aplastic anemia diagnosed in Sweden from 2000–2011. Patients were included via the National Patient Registry, and diagnosed according to the Camitta criteria. In total, 257 confirmed cases were identified, with an overall incidence of 2.35 (95% CI: 2.06–2.64) cases per million inhabitants per year. Median age was 60 years (range: 2–92), and median follow up was 76 (0–193) months. Primary treatments included immunosuppressive therapy (63%), allogenic stem cell transplantation (10%), or single-agent cyclosporine/no specific therapy (27%). The 5-year survival was 90.7% in patients aged 0–18 years, 90.5% in patients aged 19–39 years, 70.7% in patients aged 40–59 years, and 38.1% in patients aged ≥60 years. Multivariate analysis showed that age (both 40–59 and ≥60 age groups), very severe aplastic anemia and single-agent cyclosporine/no specific therapy were independent risk factors for inferior survival. In conclusion, younger aplastic anemia patients experience a very good long-term survival, while that of patients ≥60 years in particular remains poor. Apparently, the challenge today is to improve the management of older aplastic anemia patients, and prospective studies to address this medical need are warranted. PMID:28751565

T-cell receptor Vbeta CDR3 oligoclonality frequently occurs in childhood refractory cytopenia (MDS-RC) and severe aplastic anemia

DEFF Research Database (Denmark)

Vries, A.C. de; Langerak, A.W.; Verhaaf, B.

2008-01-01

(Very) severe acquired aplastic anemia ((v)SAA) and myelodysplastic syndrome (MDS) are rare diseases in childhood. (V)SAA is a bone marrow (BM) failure syndrome characterized by immune-mediated destruction of hematopoietic progenitors. MDS is a malignant clonal stem cell disorder, of which...... the hypoplastic variant is, in case of absence of a cytogenetic clone, difficult to separate from (v)SAA. Recently, studies provided a molecular signature of autoimmunity in adult (v)SAA, by showing oligoclonality based on the length of the TCR Vbeta CDR3 region. We investigated retrospectively the frequency...... and the discriminative value of TCR Vbeta CDR3 oligoclonality in pediatric (v)SAA and MDS patients. Peripheral blood (PB) and/or BM mononuclear cell samples of pediatric patients with (v)SAA (n=38), refractory cytopenia (MDS-RC) (n=28) and 18 controls were analysed via TCR Vbeta heteroduplex PCR analysis of extracted...

MRI of the femoral bone marrow in the assessment of aplastic anemia

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Tanaka, Osamu; Matsuura, Katsuhiko; Ichikawa, Tamaki; Kobayashi, Yasuyuki; Nagai, Jun; Takagi, Shojiro [Jichi Medical School, Saitama (Japan) Omiya Medical Center

1995-11-01

MR imaging of the femoral bone marrow was performed in 12 patients with untreated aplastic anemia and six patients with hypoplastic myelodysplastic syndrome (MDS). The MRI appearance was classified into four patterns; fatty marrow faint signal, nodular pattern and heterogeneous infiltration. The MRI patterns of aplastic anemia were evaluated and compared with those of hypoplastic MDS. In spite of hypocellular biopsies, MRI of the femoral marrow showed unexpected abnormal signal intensities in aplastic anemia; nodular pattern in five and heterogeneous infiltration pattern in two patients. Completely fatty marrow was depicted in four patients mainly with severe aplastic anemia. The nodular pattern with a background of fatty marrow was commonly seen in moderate or severe cases, while the heterogeneous infiltration pattern was noted in mild cases of the disease. Compared with hypoplastic MDS, asymmetrical nodular pattern suggesting patchy hematopoiesis was thought to be a characteristic finding of aplastic anemia. One patient clinically diagnosed as aplastic anemia, who had shown heterogeneous infiltration pattern, evolved to acute myeloid leukemia. We concluded that MRI of the femoral marrow could be useful in the assessment of aplastic anemia and detection of myelodysplastic or leukemic transformation. (author).

MRI of the femoral bone marrow in the assessment of aplastic anemia

International Nuclear Information System (INIS)

Tanaka, Osamu; Matsuura, Katsuhiko; Ichikawa, Tamaki; Kobayashi, Yasuyuki; Nagai, Jun; Takagi, Shojiro

1995-01-01

MR imaging of the femoral bone marrow was performed in 12 patients with untreated aplastic anemia and six patients with hypoplastic myelodysplastic syndrome (MDS). The MRI appearance was classified into four patterns; fatty marrow faint signal, nodular pattern and heterogeneous infiltration. The MRI patterns of aplastic anemia were evaluated and compared with those of hypoplastic MDS. In spite of hypocellular biopsies, MRI of the femoral marrow showed unexpected abnormal signal intensities in aplastic anemia; nodular pattern in five and heterogeneous infiltration pattern in two patients. Completely fatty marrow was depicted in four patients mainly with severe aplastic anemia. The nodular pattern with a background of fatty marrow was commonly seen in moderate or severe cases, while the heterogeneous infiltration pattern was noted in mild cases of the disease. Compared with hypoplastic MDS, asymmetrical nodular pattern suggesting patchy hematopoiesis was thought to be a characteristic finding of aplastic anemia. One patient clinically diagnosed as aplastic anemia, who had shown heterogeneous infiltration pattern, evolved to acute myeloid leukemia. We concluded that MRI of the femoral marrow could be useful in the assessment of aplastic anemia and detection of myelodysplastic or leukemic transformation. (author)

Hemoperitoneum from corpus luteum rupture in patients with aplastic anemia.

Science.gov (United States)

Wang, Huaquan; Guo, Lifang; Shao, Zonghong

2015-01-01

Aplastic anemia is a rare hematopoietic stem-cell disorder that results in pancytopenia and hypocellular bone marrow. Women with aplastic anemia usually are at increased risk of corpus luteum rupture due to thrombocytopenia and infection. Here we report two cases had hemoperitoneum from corpus luteum rupture in patients with aplastic anemia in our center. Case 1 involved two episodes of hemoperitoneum resulting from rupture of the corpus luteum in a 23-year-old unmarried female with severe aplastic anemia. This patient was managed conservatively with platelet and packed red cell transfusion. Case 2 involved two episodes of hemoperitoneum resulting from rupture of the corpus luteum in a 33-year-old married patient with aplastic anemia. Emergency laparoscopy revealed massive hemoperitoneum. Bilateral salpingo-oophorectomy were performed successively with platelet and packed red cell transfusion. Hemoperitoneum resulting from a ruptured corpus luteum is a life-threatening condition in patients with aplastic anemia. Prompt and appropriate evaluation of corpus luteum rupture and emergent therapy are needed.

Nationwide survey on the use of eltrombopag in patients with severe aplastic anemia: a report on behalf of the French Reference Center for Aplastic Anemia.

Science.gov (United States)

Lengline, Etienne; Drenou, Bernard; Peterlin, Pierre; Tournilhac, Olivier; Abraham, Julie; Berceanu, Ana; Dupriez, Brigitte; Guillerm, Gaelle; Raffoux, Emmanuel; de Fontbrune, Flore Sicre; Ades, Lionel; Balsat, Marie; Chaoui, Driss; Coppo, Paul; Corm, Selim; Leblanc, Thierry; Maillard, Natacha; Terriou, Louis; Socié, Gerard; de Latour, Regis Peffault

2018-02-01

Few therapeutic options are available for patients with aplastic anemia who are ineligible for transplantation or refractory to immunosuppressive therapy. Eltrombopag was recently shown to produce trilineage responses in refractory patients. However, the effects of real-life use of this drug remain unknown. This retrospective study (2012-2016) was conducted by the French Reference Center for Aplastic Anemia on patients with relapsed/refractory aplastic anemia, and patients ineligible for antithymocyte globulin or transplantation, who received eltrombopag for at least 2 months. Forty-six patients with aplastic anemia were given eltrombopag without prior antithymocyte globulin treatment (n=11) or after antithymocyte globulin administration (n=35) in a relapsed/refractory setting. Eltrombopag (median daily dose 150 mg) was introduced 17 months (range, 8-50) after the diagnosis of aplastic anemia. At last followup, 49% were still receiving treatment, 9% had stopped due to a robust response, 2% due to toxicity and 40% due to eltrombopag failure. Before eltrombopag treatment, all patients received regular transfusions. The overall rates of red blood cell and platelet transfusion independence were 7%, 33%, 46% and 46% at 1, 3, 6 months and last follow-up. Responses were slower to develop in antithymocyte treatment-naïve patients. In patients achieving transfusion independence, hemoglobin concentration and platelet counts improved by 3 g/dL (interquartile range, 1.4-4.5) and 42×10 9 /L (interquartile range, 11-100), respectively. Response in at least one lineage (according to National Institutes of Health criteria) was observed in 64% of antithymocyte treatment-naïve and 74% of relapsed/refractory patients, while trilineage improvement was observed in 27% and 34%, respectively. We found high rates of hematologic improvement and transfusion independence in refractory aplastic anemia patients but also in patients ineligible for antithymocyte globulin receiving first

Telomerase gene therapy rescues telomere length, bone marrow aplasia, and survival in mice with aplastic anemia.

Science.gov (United States)

Bär, Christian; Povedano, Juan Manuel; Serrano, Rosa; Benitez-Buelga, Carlos; Popkes, Miriam; Formentini, Ivan; Bobadilla, Maria; Bosch, Fatima; Blasco, Maria A

2016-04-07

Aplastic anemia is a fatal bone marrow disorder characterized by peripheral pancytopenia and marrow hypoplasia. The disease can be hereditary or acquired and develops at any stage of life. A subgroup of the inherited form is caused by replicative impairment of hematopoietic stem and progenitor cells due to very short telomeres as a result of mutations in telomerase and other telomere components. Abnormal telomere shortening is also described in cases of acquired aplastic anemia, most likely secondary to increased turnover of bone marrow stem and progenitor cells. Here, we test the therapeutic efficacy of telomerase activation by using adeno-associated virus (AAV)9 gene therapy vectors carrying the telomerase Tert gene in 2 independent mouse models of aplastic anemia due to short telomeres (Trf1- and Tert-deficient mice). We find that a high dose of AAV9-Tert targets the bone marrow compartment, including hematopoietic stem cells. AAV9-Tert treatment after telomere attrition in bone marrow cells rescues aplastic anemia and mouse survival compared with mice treated with the empty vector. Improved survival is associated with a significant increase in telomere length in peripheral blood and bone marrow cells, as well as improved blood counts. These findings indicate that telomerase gene therapy represents a novel therapeutic strategy to treat aplastic anemia provoked or associated with short telomeres. © 2016 by The American Society of Hematology.

Idiopathic aplastic anemia: diagnosis and classification.

Science.gov (United States)

Dolberg, Osnat Jarchowsky; Levy, Yair

2014-01-01

Aplastic anemia (AA) is a disease characterized by pancytopenia and hypoplastic bone marrow caused by the decrease of hematopoietic stem cells. The pathogenesis of AA is complex and involves an abnormal hematopoietic microenvironment, hematopoietic stem cell/progenitor cell deficiencies and immunity disorders. Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. Herein, we will update the main issues concern AA according to our literature review. Copyright © 2014 Elsevier B.V. All rights reserved.

[Long-term Follow-up of Patients with Hepatitis-Associated Aplastic Anemia].

Science.gov (United States)

You, Ya-Hong; Meng, Xian-Bin; Li, Xing-Xin; Ge, Mei-Li; Nie, Neng; Huang, Jin-Bo; Zhang, Jing; Huang, Zhen-Dong; Shao, Ying-Qi; Shi, Jun; Zheng, Yi-Zhou

2017-08-01

To explore the clinical characteristic, therapeutic efficacy and prognosis of patients with hepatitis-associated aplasitc anemia (HAAA). the clinical data and labrotatory examination results of 30 cases of HAAA were analyzed retrospectively, the 6-month response ratio and overall survival (OS) were assessed. HAAA most commonly occured in males, with the occurence rate of males and females was 4:1, the median onset age was 16 (4-43) years old, HAAA oriented focus on sever aplastic anemia (SAA)(4 cases,13%) and very sever aplastic anemia (VSAA)(22 cases,73%). Aplastic anemia (AA) could be seen on occurence of hepatitis (accompanied aplastic anemia) (7 cases,23%), or after the onset of hepatits (delayed aplastic anemia) (23 cases,77%), but more often occured in the latter. Statistical analysis showed that when compared with the patients of delayed aplastic anemia, patients accompanied aplastic anemia possesses lower levels of glutamic-pyruvic transaminase(ALT), aspertate aminotransferase (AST) and total bilirubin (TBIL)(P=0.042,0.012,0.001), and possessed a more obvious lymphoid cell disorder when AA occured, with more lower peripheral blood CD19 + B cells proportion (P=0.046) and more obvious imbalance of CD4 + /CD8 + ratio, but the difference was no statistical significant (P=0538). Factors affecting the 6-month respose were the severity of AA (P=0.044), the peak level of bilirubin of hepatitis (P=0.006) and the propotion of mature monocyte in bone marrow (P=0.034). The long-term follow-up showed that the 2-year OS of HAAA was 64.3±9.2%, the 6-month curative efficacy significantly affect the prognosis (Paplastic anemia, patients usually have a high incidence of early infection. Patients acompanied with aplastic anemia possess more obvious immunological derangement; the treatment efficacy for HAAA is poor, patients who haven't obtained 6-month response indicate a sinister prognosis, allogeneic hematopoietic stem cell transplantion is a better choice for these

Aplastic anaemia in Christchurch Hospital 1979-89.

Science.gov (United States)

Baker, B W; Smith, M P; Abbott, G D; Beard, M E; Spearing, R L; Heaton, D C

1991-11-13

We have reviewed the records of all patients referred to our departments with aplastic anaemia during the 11 years from 1979 to 1989. Of the 22 patients identified, 19 fulfilled the standard criteria for severe aplastic anaemia. There were 11 females and 11 males. Their mean age was 35 (range 2-85 years). Five cases followed exposure to drugs known to cause aplastic anaemia and one had a recent history of viral hepatitis. A variety of treatments were used. Four patients received an allogeneic bone marrow transplant (BMT) from matched sibling donors and two of these were alive and well 65 and 120 months post BMT. Antithymocyte globulin (ATG) treatment has been followed by lasting complete remission in two of the six patients treated and a partial response was seen in one other patient. Cyclosporin therapy was associated with unmaintained complete remission in one of the three patients given this drug after ATG had failed. The remaining 13 patients received only supportive care with or without androgens and six (46%) had early recovery of bone marrow function with lasting complete remission. These patients illustrate some of the therapeutic options available for aplastic anaemia.

Cyclophosphamide and TNI in aplastic anemias

International Nuclear Information System (INIS)

Scotti, G.; Rigon, A.; Polico, C.

1987-01-01

Personal experience is outlined with a preparative regimen consisting of total nodal irradiation (TNI) and cyclophosphamide in patients with severe aplastic anemia undergoing bone marrow transplantation (BMT). Nine patients (median age 23) previously having blood transfusions received BMT at the BMT Center in Pesaro. All patients were prepared for transplantation with cyclophosphamide 50 mg/kg/day (day -6, -5, -4, -3), and 7,5 Gy total nodal irradiation day -1, with a dose rate of 26 cGy/m. Six out of eight evaluable transplanted patients are still surviving 3 to 23 months with a median follow-up of 16,5 months. This preoperative regimen is extremely effective in decreasing rejection following transplantation for severe aplastic anemia. Future investigation must be aimed at the elimination of graft-versus-host-disease and control of fatal infections

Treatment of severe aplastic anaemia with total lymphoid irradiation and methylprednisolone

International Nuclear Information System (INIS)

Mehta, J.; Singhal, S.; Huilgol, N.; Merchant, R.; Mehta, B.C.

1992-01-01

This case report briefly summarizes the treatment of aplastic anaemia with total lymphoid irradiation and methylprednisolone and recommends that this procedure should be considered a therapeutic option in patients who are not candidates for bone marrow transplantation or antithymocyte globulin, or those who have failed one course of the latter. (Author)

Outcome of Allogeneic Stem Cell Transplantation for Patients Transformed to Myelodysplastic Syndrome or Leukemia from Severe Aplastic Anemia: A Report from the MDS Subcommittee of the Chronic Malignancies Working Party and the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation

NARCIS (Netherlands)

Hussein, A.A.; Halkes, C.M.; Socie, G.; Tichelli, A.; Borne, P.A. von dem; Schaap, M.N.; Foa, R.; Ganser, A.; Dufour, C.; Bacigalupo, A.; Locasciulli, A.; Aljurf, M.; Peters, C.; Robin, M.; Biezen, A.A. van; Volin, L.; Witte, T.J. de; Marsh, J.; Passweg, J.R.; Kroger, N.; et al.,

2014-01-01

One hundred and forty patients who had undergone hematopoietic stem cell transplantation (HSCT) for myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML) transformation after treatment of severe aplastic anemia (SAA) were identified in the European Group for Blood and Marrow

Aplastic Anemia in Two Patients with Sex Chromosome Aneuploidies.

Science.gov (United States)

Rush, Eric T; Schaefer, G Bradley; Sanger, Warren G; Coccia, Peter F

2015-01-01

Sex chromosome aneuploidies range in incidence from rather common to exceedingly rare and have a variable phenotype. We report 2 patients with sex chromosome aneuploidies who developed severe aplastic anemia requiring treatment. The first patient had tetrasomy X (48,XXXX) and presented at 9 years of age, and the second patient had trisomy X (47,XXX) and presented at 5 years of age. Although aplastic anemia has been associated with other chromosomal abnormalities, sex chromosome abnormalities have not been traditionally considered a risk factor for this condition. A review of the literature reveals that at least one other patient with a sex chromosome aneuploidy (45,X) has suffered from aplastic anemia and that other autosomal chromosomal anomalies have been described. Despite the uncommon nature of each condition, it is possible that the apparent association is coincidental. A better understanding of the genetic causes of aplastic anemia remains important. © 2015 S. Karger AG, Basel.

Special Issues for People with Aplastic Anemia

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... Menu Donate Special Issues for People with Aplastic Anemia Because you have aplastic anemia , everyday events can ... bleeding, such as contact sports. Pregnancy and Aplastic Anemia Pregnancy is possible for women who have been ...

Clonal hematopoiesis in acquired aplastic anemia.

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Ogawa, Seishi

2016-07-21

Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolution. Genetic abnormalities are found in ∼50% of patients with AA and, except for PIGA mutations and copy-neutral loss-of-heterozygosity, or uniparental disomy (UPD) in 6p (6pUPD), are most frequently represented by mutations involving genes commonly mutated in myeloid malignancies, including DNMT3A, ASXL1, and BCOR/BCORL1 Mutations exhibit distinct chronological profiles and clinical impacts. BCOR/BCORL1 and PIGA mutations tend to disappear or show stable clone size and predict a better response to IST and a significantly better clinical outcome compared with mutations in DNMT3A, ASXL1, and other genes, which are likely to increase their clone size, are associated with a faster progression to MDS/AML, and predict an unfavorable survival. High frequency of 6pUPD and overrepresentation of PIGA and BCOR/BCORL1 mutations are unique to AA, suggesting the role of autoimmunity in clonal selection. By contrast, DNMT3A and ASXL1 mutations, also commonly seen in CH in the general population, indicate a close link to CH in the aged bone marrow, in terms of the mechanism for selection. Detection and close monitoring of somatic mutations/evolution may help with prediction and diagnosis of clonal evolution of MDS/AML and better management of patients with AA. © 2016 by The American Society of Hematology.

Selective engraftment of the granulocyte compartment after allogeneic bone marrow transplantation in a patient with severe aplastic anemia.

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Barriga, F J; Legues, M E; Bertin, P

1996-05-01

We present a patient with severe aplastic anemia who had partial engraftment with full chimerism after allogeneic bone marrow transplantation from an HLA identical sibling. A 3-year-old girl with severe aplastic anemia (SAA) received a bone marrow transplantation (BMT) from an HLA identical brother 9 months after her diagnosis. Before BMT she was red blood cell tranfusion dependent, had an absolute neutrophil count (ANC) of 1,000-1,500 x 10(9)/1 and a platelet count of 15-19,000 x 10(9)/1. She was conditioned with 800 cGy total body irradiation (TBI) and cyclophosphamide and received 3X10(8) nucleated cells/kg. She reached an ANC of 1500 x 10(9)/1 on day +35 but her reticulocyte and platelet counts did not recover. A bone marrow aspirate and biopsy post BMT showed hypoplasia with marked decrease in megakaryocyte and red blood cell precursors. The granulocyte compartment showed a left shift with predominance of promyelocytes and myelocytes. The karyotype showed full chimerism (46,XY) with no 46,XX metaphases. This case illustrates the possibility of a bone marrow microenvironment defect as the cause of SAA.

Genetic Associations in Acquired Immune-Mediated Bone Marrow Failure Syndromes: Insights in Aplastic Anemia and Chronic Idiopathic Neutropenia

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Mavroudi, Irene; Papadaki, Helen A.

2012-01-01

Increasing interest on the field of autoimmune diseases has unveiled a plethora of genetic factors that predispose to these diseases. However, in immune-mediated bone marrow failure syndromes, such as acquired aplastic anemia and chronic idiopathic neutropenia, in which the pathophysiology results from a myelosuppressive bone marrow microenvironment mainly due to the presence of activated T lymphocytes, leading to the accelerated apoptotic death of the hematopoietic stem and progenitor cells, such genetic associations have been very limited. Various alleles and haplotypes of human leucocyte antigen (HLA) molecules have been implicated in the predisposition of developing the above diseases, as well as polymorphisms of inhibitory cytokines such as interferon-γ, tumor necrosis factor-α, and transforming growth factor-β1 along with polymorphisms on molecules of the immune system including the T-bet transcription factor and signal transducers and activators of transcription. In some cases, specific polymorphisms have been implicated in the outcome of treatment on those patients. PMID:22956967

Emergence of clonal hematopoiesis in the majority of patients with acquired aplastic anemia.

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Babushok, Daria V; Perdigones, Nieves; Perin, Juan C; Olson, Timothy S; Ye, Wenda; Roth, Jacquelyn J; Lind, Curt; Cattier, Carine; Li, Yimei; Hartung, Helge; Paessler, Michele E; Frank, Dale M; Xie, Hongbo M; Cross, Shanna; Cockroft, Joshua D; Podsakoff, Gregory M; Monos, Dimitrios; Biegel, Jaclyn A; Mason, Philip J; Bessler, Monica

2015-04-01

Acquired aplastic anemia (aAA) is a nonmalignant disease caused by autoimmune destruction of early hematopoietic cells. Clonal hematopoiesis is a late complication, seen in 20-25% of older patients. We hypothesized that clonal hematopoiesis in aAA is a more general phenomenon, which can arise early in disease, even in younger patients. To evaluate clonal hematopoiesis in aAA, we used comparative whole exome sequencing of paired bone marrow and skin samples in 22 patients. We found somatic mutations in 16 patients (72.7%) with a median disease duration of 1 year; of these, 12 (66.7%) were patients with pediatric-onset aAA. Fifty-eight mutations in 51 unique genes were found primarily in pathways of immunity and transcriptional regulation. Most frequently mutated was PIGA, with seven mutations. Only two mutations were in genes recurrently mutated in myelodysplastic syndrome. Two patients had oligoclonal loss of the HLA alleles, linking immune escape to clone emergence. Two patients had activating mutations in key signaling pathways (STAT5B (p.N642H) and CAMK2G (p.T306M)). Our results suggest that clonal hematopoiesis in aAA is common, with two mechanisms emerging-immune escape and increased proliferation. Our findings expand conceptual understanding of this nonneoplastic blood disorder. Future prospective studies of clonal hematopoiesis in aAA will be critical for understanding outcomes and for designing personalized treatment strategies. Copyright © 2015 Elsevier Inc. All rights reserved.

Evaluation of severity in aplastic anemia by MR imaging

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Park, Jeong Mi; Lim, Gye Yeon; Kim, Euy Neyng; Lee, Jae Mun; Kim, Dong Wook; Han, Chi Wha; Kim, Chun Choo [The Catholic University College of Medicine, Seoul (Korea, Republic of)

1999-02-01

To evaluate the role of bone marrow (BM) magnetic resonance (MR) imaging for assessment of the severity of aplastic anemia (AA). Eighty patients with AA, ranging in age from 16 to 44 years underwent MR imaging. Fifty four patients had clinically severe AA(SAA), while in 26 the condition was moderate(MAA). Sagittal T1-weighted images (T1WI) and short tau inversion recovery (STIR) images of lumbar vertebral BM were analysed. Bulk T1, T2 and rho values (msec) were also measured, with mixed sequences. Signal intensity (SI) on both T1WI and STIR was classified into four patterns according to the amount fatty marrow : pattern I, homogeneous fatty marrow ; 2, fatty marrow with focal cellular nodules ; 3, mixed fatty and cellular marrow ; 4, cellular marrow with focal fatty nodules. These SI patterns and bulk T1, T2 and rho values of the lumbar BM were compared with the clinical severity of AA. On both T1WI and STIR sequences, MR imaging of lumbar vertebral BM in patients with AA showed various SI patterns. Pattern 1, 2 and 3 were much frequently seen in the SAA group (48 of 54 patients on T1WI and 43 of 54 on STIR) and pattern 4 was common in the MAA group (16 of 26 patients on T1WI and 18 of 26 on STIR). The SI patterns of AA seen on both T1WI and STIR sequences closely correlated with clinical severity (x 2 test, p=0.0001). Bulk T1 value was significantly different between SAA and MAA (SAA : 382.82msec {+-} 113.91 ; MAA : 517.99msec {+-} 151.92 ; t test, p=0.0001). The SI pattern seen on MR imaging, and T1 relaxation time of lumbar spinal BM can be useful for assessing the severity of AA.

Evaluation of severity in aplastic anemia by MR imaging

International Nuclear Information System (INIS)

Park, Jeong Mi; Lim, Gye Yeon; Kim, Euy Neyng; Lee, Jae Mun; Kim, Dong Wook; Han, Chi Wha; Kim, Chun Choo

1999-01-01

To evaluate the role of bone marrow (BM) magnetic resonance (MR) imaging for assessment of the severity of aplastic anemia (AA). Eighty patients with AA, ranging in age from 16 to 44 years underwent MR imaging. Fifty four patients had clinically severe AA(SAA), while in 26 the condition was moderate(MAA). Sagittal T1-weighted images (T1WI) and short tau inversion recovery (STIR) images of lumbar vertebral BM were analysed. Bulk T1, T2 and rho values (msec) were also measured, with mixed sequences. Signal intensity (SI) on both T1WI and STIR was classified into four patterns according to the amount fatty marrow : pattern I, homogeneous fatty marrow ; 2, fatty marrow with focal cellular nodules ; 3, mixed fatty and cellular marrow ; 4, cellular marrow with focal fatty nodules. These SI patterns and bulk T1, T2 and rho values of the lumbar BM were compared with the clinical severity of AA. On both T1WI and STIR sequences, MR imaging of lumbar vertebral BM in patients with AA showed various SI patterns. Pattern 1, 2 and 3 were much frequently seen in the SAA group (48 of 54 patients on T1WI and 43 of 54 on STIR) and pattern 4 was common in the MAA group (16 of 26 patients on T1WI and 18 of 26 on STIR). The SI patterns of AA seen on both T1WI and STIR sequences closely correlated with clinical severity (x 2 test, p=0.0001). Bulk T1 value was significantly different between SAA and MAA (SAA : 382.82msec ± 113.91 ; MAA : 517.99msec ± 151.92 ; t test, p=0.0001). The SI pattern seen on MR imaging, and T1 relaxation time of lumbar spinal BM can be useful for assessing the severity of AA

Aplastic anemia in Japanese radiological technicians

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Kitabatake, T.; Watanabe, T.; Saito, A.; Nakamura, M.; Shiohama Hospital, Mie

1976-01-01

Among the Japanese radiological technicians, four deaths from aplastic anemia have been reported after 1930. On the other hand, during the period from 1930 to 1960, the population of radiological technicians is estimated to be 74,400 man-years, in which 0.5 aplastic anemias are expected. However, actually three died from aplastic anemia. This difference is statistically significant at the 1% level. While, in the period from 1961 to 1973, the observed value is 1 against 0.7 expected. It is concluded that aplastic anemia had been induced frequently among the Japanese radiological technicians in the era when there was much exposure to occupational radiation. (orig.) [de

Paroxysmal nocturnal hemoglobinuria and telomere length predicts response to immunosuppressive therapy in pediatric aplastic anemia.

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Narita, Atsushi; Muramatsu, Hideki; Sekiya, Yuko; Okuno, Yusuke; Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Yoshida, Nao; Wang, Xinan; Xu, Yinyan; Kawashima, Nozomu; Doisaki, Sayoko; Hama, Asahito; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Kobayashi, Masao; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

2015-12-01

Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive therapy responsiveness in aplastic anemia. We enrolled 113 aplastic anemia patients (63 boys and 50 girls) in this study to evaluate their response to immunosuppressive therapy. The paroxysmal nocturnal hemoglobinuria populations and telomere length were detected by flow cytometry. Forty-seven patients (42%) carried a minor paroxysmal nocturnal hemoglobinuria population. The median telomere length of aplastic anemia patients was -0.99 standard deviation (SD) (range -4.01-+3.01 SD). Overall, 60 patients (53%) responded to immunosuppressive therapy after six months. Multivariate logistic regression analysis identified the absence of a paroxysmal nocturnal hemoglobinuria population and a shorter telomere length as independent unfavorable predictors of immunosuppressive therapy response at six months. The cohort was stratified into a group of poor prognosis (paroxysmal nocturnal hemoglobinuria negative and shorter telomere length; 37 patients) and good prognosis (paroxysmal nocturnal hemoglobinuria positive and/or longer telomere length; 76 patients), respectively. The response rates of the poor prognosis and good prognosis groups at six months were 19% and 70%, respectively (P<0.001). The combined absence of a minor paroxysmal nocturnal hemoglobinuria population and a short telomere length is an efficient predictor of poor immunosuppressive therapy response, which should be considered while deciding treatment options: immunosuppressive therapy or first-line hematopoietic stem cell transplantation. The trial was registered in www.umin.ac.jp with number UMIN000017972. Copyright© Ferrata Storti Foundation.

ATG-Fresenius S combined with cyclosporine a: an effective immunosuppressive therapy for children with aplastic anemia.

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Luo, Cheng-Juan; Gao, Yi-Jin; Tang, Jing-Yan; Zhu, Xiao-Hua; Xue, Hui-Liang; Lu, Feng-Juan; Pan, Ci; Jiang, Hua; Luo, Chang-Ying; Ye, Qi-Dong; Zhou, Min; Chen, Jing

2014-07-01

For the first time, we conducted a 2-center retrospective study to show the efficacy of antithymocyte globulin (ATG)-Fresenius S plus cyclosporine treatment of children with severe aplastic anemia. From March 1997 to May 2011, a total of 124 patients (median age, 7.5 y; range, 1.5 to 16 y) from 2 centers with acquired AA treated with an immunosuppressive therapy (IST) regimen, consisting of ATG-Fresenius S (5 mg/kg per day for 5 d) and cyclosporine, were enrolled. The response rate was 55.6%. The median time between IST and response was 6 (0.5 to 18) months. After a median follow-up time of 29 (6 to 153) months, the rates of relapse and clonal evolution were 3.2% and 0.8%, respectively. Overall, 17 patients (13.7%) died in this study: 14 resulted from sepsis, 1 resulted from intracranial hemorrhage, 1 occurred after hematopoietic stem cell transplantation, and 1 resulted from clonal disease progression. The 5-year overall survival rate for the entire cohort was 74.7%. IST responders had a better survival rate (100%) than nonresponders (70.7%). The use of ATG-Fresenius S plus cyclosporine as a first-line immunosuppressive treatment appeared to be effective for children with severe aplastic anemia in our study. ATG-Fresenius S could be another option in the treatment arsenal, especially in countries where the other ATG products are harder to acquire.

Meta-analysis of Huangqi injection for the adjunctive therapy of aplastic anemia

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Zhu, Changtai; Gao, Yulu; Jiang, Ting; Hao, Cao; Gao, Zongshuai; Sun, Yongning

2015-01-01

Aplastic anemia therapy remains difficult, due to lack of effective treatment regimens. In recent years, Huangqi injection for the adjunctive therapy of aplastic anemia has been reported in many clinical trials. Considering that Huangqi injection may be a novel approach to aplastic anemia treatment, we conducted a meta-analysis of clinical controlled trials to assess the clinical value of Huangqi injection in the treatment of aplastic anemia. We searched the Chinese Biomedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), Chinese Scientific Journals Full-text Database (VIP), Wanfang Database, PubMed and EMBASE database to collect the data about the trials of Huangqi injection combined with androgens for treating aplastic anemia. A total of ten studies involving 720 patients with aplastic anemia were included in this study. The meta-analysis showed significant increases in the pool effectiveness rate, white blood cells (WBC), haematoglobin (Hb), platelets (PLT), and reticulocytes (Ret) between the experimental group versus the control group. No severe side effects were found in this study. However, the lower Jadad scores and asymmetric funnel plot degrades the validity of the meta-analysis as the clinical evidence. Therefore, Huangqi injection may significantly enhance the efficacy of androgens for aplastic anemia, suggesting that the novel approach of Chinese traditional medicine combined with Western medicine is promising. The exact outcome required confirmation with rigorously well-designed multi-center trials. PMID:26379817

Severe Aplastic Anemia following Acute Hepatitis from Toxic Liver Injury: Literature Review and Case Report of a Successful Outcome

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Kamran Qureshi

2014-01-01

Full Text Available Hepatitis associated aplastic anemia (HAAA is a rare syndrome in which severe aplastic anemia (SAA complicates the recovery of acute hepatitis (AH. HAAA is described to occur with AH caused by viral infections and also with idiopathic cases of AH and no clear etiology of liver injury. Clinically, AH can be mild to fulminant and transient to persistent and precedes the onset SAA. It is assumed that immunologic dysregulation following AH leads to the development of SAA. Several observations have been made to elucidate the immune mediated injury mechanisms, ensuing from liver injury and progressing to trigger bone marrow failure with the involvement of activated lymphocytes and severe T-cell imbalance. HAAA has a very poor outcome and often requires bone marrow transplant (BMT. The findings of immune related myeloid injury implied the use of immunosuppressive therapy (IST and led to improved survival from HAAA. We report a case of young male who presented with AH resulting from the intake of muscle building protein supplements and anabolic steroids. The liver injury slowly resolved with supportive care and after 4 months of attack of AH, he developed SAA. He was treated with IST with successful outcome without the need for a BMT.

Genetic features of myelodysplastic syndrome and aplastic anemia in pediatric and young adult patients

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Keel, Siobán B.; Scott, Angela; Sanchez-Bonilla, Marilyn; Ho, Phoenix A.; Gulsuner, Suleyman; Pritchard, Colin C.; Abkowitz, Janis L.; King, Mary-Claire; Walsh, Tom; Shimamura, Akiko

2016-01-01

The clinical and histopathological distinctions between inherited versus acquired bone marrow failure and myelodysplastic syndromes are challenging. The identification of inherited bone marrow failure/myelodysplastic syndromes is critical to inform appropriate clinical management. To investigate whether a subset of pediatric and young adults undergoing transplant for aplastic anemia or myelodysplastic syndrome have germline mutations in bone marrow failure/myelodysplastic syndrome genes, we performed a targeted genetic screen of samples obtained between 1990–2012 from children and young adults with aplastic anemia or myelodysplastic syndrome transplanted at the Fred Hutchinson Cancer Research Center. Mutations in inherited bone marrow failure/myelodysplastic syndrome genes were found in 5.1% (5/98) of aplastic anemia patients and 13.6% (15/110) of myelodysplastic syndrome patients. While the majority of mutations were constitutional, a RUNX1 mutation present in the peripheral blood at a 51% variant allele fraction was confirmed to be somatically acquired in one myelodysplastic syndrome patient. This highlights the importance of distinguishing germline versus somatic mutations by sequencing DNA from a second tissue or from parents. Pathological mutations were present in DKC1, MPL, and TP53 among the aplastic anemia cohort, and in FANCA, GATA2, MPL, RTEL1, RUNX1, SBDS, TERT, TINF2, and TP53 among the myelodysplastic syndrome cohort. Family history or physical examination failed to reliably predict the presence of germline mutations. This study shows that while any single specific bone marrow failure/myelodysplastic syndrome genetic disorder is rare, screening for these disorders in aggregate identifies a significant subset of patients with inherited bone marrow failure/myelodysplastic syndrome. PMID:27418648

[Aplastic anaemia associated with pregnancy].

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Bozhinova, S; Kirovakov, Zl; Porozhanova, K; Kostova, S; Bozhinov, P

2012-01-01

Aplastic anaemia is rear disease caused by destruction of pluripotent stem cells in bone marrow. Pregnancy is one of the main factor that lead to immunosuppression. During pregnancy aplastic anaemia could be life-threatening for both mother and child, because of the variety of complications like bleeding and infections. We introduce the first case of pregnant woman with aplastic anaemia in Bulgaria. The woman was diagnosed in 12-13 gestational week. All biometric characteristics of the foetus were normal. The patient was consulted with oncohaematologists, pediatricians, specialists of Obstetrics and Gynaecology, and intensivists. Methylprednisolone, antibiotics, packed cells and platelet transfusions were initiated. However, the moment for interruption of the pregnancy was missed (first trimester). The woman developed a fever and vomited bloody material. Despite the optimal supportive treatment, the patient died. The pathoanatomy diagnose is Aplastic anaemia, induced by the pregnancy. From our experience with that case and other references from the literature we conclude that all pregnant woman with aplastic anaemia should interrupt their pregnancy during first trimester. In those patients who are diagnosed at later terms of pregnancy very supportive infusions and immunosuppressive therapy should be made, including antithymocyte globulin and/or cyclosporine. Women with no improvement from that therapy should achieve a bone-marrow transplantation.

Aplastic anemia due to radiation

International Nuclear Information System (INIS)

Sakai, Kunio; Saito, Akira

1978-01-01

The relationship between radiation exposure and aplastic anemia, clarified previously, is discussed. When persons such as radiological technicians receive whole-body irradiation in rather large doses, it is possible that aplastic anemia will result later on. However, this is difficult to determine because the irradiated region is limited despite large doses of radiation. (Bell, E.)

Clinical study of aplastic anemia among A-bomb survivors

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Oguma, N.; Dohy, H.; Kyo, T.; Saito, O.; Okita, H. (Hiroshima Univ. (Japan). Research Inst. for Nuclear Medicine and Biology)

1980-11-01

In 90 patients with aplastic anemia who were seen at Dept. Med. RINMB, Hiroshima Univ. from 1962 to March, 1980, clinical findings of 33 A-bomb survivors (which included the second generation of the survivors) and those of 57 nonexposed patients were compared. No relationship was found between the age at the time of exposure and the period preceding onset of the disease. The A-bomb survivors showed higher neutrophil counts and higher reticulocyte counts than the nonexposed patients. There were less severe cases in the A-bomb survivors. There was no difference in the incidence of atypical aplastic anemia between the exposed patients and the nonexposed ones. No difference was found in overall survival (one-year and five-year survival rates) between the exposed and the nonexposed. The A-bomb survivors often had complete remission or maintenance of remission, and rarely had acute progression. These results suggested that clinical picture of aplastic anemia in the A-bomb survivors is different from that in the nonexposed patients.

Chromosome anomalies in bone marrow as primary cause of aplastic or hypoplastic conditions and peripheral cytopenia: disorders due to secondary impairment of RUNX1 and MPL genes

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Marletta Cristina

2012-10-01

Full Text Available Abstract Background Chromosome changes in the bone marrow (BM of patients with persistent cytopenia are often considered diagnostic for a myelodysplastic syndrome (MDS. Comprehensive cytogenetic evaluations may give evidence of the real pathogenetic role of these changes in cases with cytopenia without morphological signs of MDS. Results Chromosome anomalies were found in the BM of three patients, without any morphological evidence of MDS: 1 an acquired complex rearrangement of chromosome 21 in a boy with severe aplastic anaemia (SAA; the rearrangement caused the loss of exons 2–8 of the RUNX1 gene with subsequent hypoexpression. 2 a constitutional complex rearrangement of chromosome 21 in a girl with congenital thrombocytopenia; the rearrangement led to RUNX1 disruption and hypoexpression. 3 an acquired paracentric inversion of chromosome 1, in which two regions at the breakpoints were shown to be lost, in a boy with aplastic anaemia; the MPL gene, localized in chromosome 1 short arms was not mutated neither disrupted, but its expression was severely reduced: we postulate that the aplastic anaemia was due to position effects acting both in cis and in trans, and causing Congenital Amegakaryocytic Thrombocytopenia (CAMT. Conclusions A clonal anomaly in BM does not imply per se a diagnosis of MDS: a subgroup of BM hypoplastic disorders is directly due to chromosome structural anomalies with effects on specific genes, as was the case of RUNX1 and MPL in the patients here reported with diagnosis of SAA, thrombocytopenia, and CAMT. The anomaly may be either acquired or constitutional, and it may act by deletion/disruption of the gene, or by position effects. Full cytogenetic investigations, including a-CGH, should always be part of the diagnostic evaluation of patients with BM aplasia/hypoplasia and peripheral cytopenias.

Hepatitis Associated Aplastic Anemia: A review

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2011-01-01

Hepatitis-associated aplastic anemia (HAAA) is an uncommon but distinct variant of aplastic anemia in which pancytopenia appears two to three months after an acute attack of hepatitis. HAAA occurs most frequently in young male children and is lethal if leave untreated. The etiology of this syndrome is proposed to be attributed to various hepatitis and non hepatitis viruses. Several hepatitis viruses such as HAV, HBV, HCV, HDV, HEV and HGV have been associated with this set of symptoms. Viruses other than the hepatitis viruses such as parvovirus B19, Cytomegalovirus, Epstein bar virus, Transfusion Transmitted virus (TTV) and non-A-E hepatitis virus (unknown viruses) has also been documented to develop the syndrome. Considerable evidences including the clinical features, severe imbalance of the T cell immune system and effective response to immunosuppressive therapy strongly present HAAA as an immune mediated mechanism. However, no association of HAAA has been found with blood transfusions, drugs and toxins. Besides hepatitis and non hepatitis viruses and immunopathogenesis phenomenon as causative agents of the disorder, telomerase mutation, a genetic factor has also been predisposed for the development of aplastic anemia. Diagnosis includes clinical manifestations, blood profiling, viral serological markers testing, immune functioning and bone marrow hypocellularity examination. Patients presenting the features of HAAA have been mostly treated with bone marrow or hematopoietic cell transplantation from HLA matched donor, and if not available then by immunosuppressive therapy. New therapeutic approaches involve the administration of steroids especially the glucocorticoids to augment the immunosuppressive therapy response. Pancytopenia following an episode of acute hepatitis response better to hematopoietic cell transplantation than immunosuppressive therapy. PMID:21352606

Association of severe thrombocytopenia and poor prognosis in pregnancies with aplastic anemia.

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Jae Eun Shin

Full Text Available PURPOSE: We sought to estimate the risks of adverse obstetric outcomes and disease outcomes associated with severe thrombocytopenia in pregnant women with aplastic anemia (AA. METHODS: In a retrospective study, we compared demographics, clinical characteristics, laboratory results, and outcomes between severe thrombocytopenia (ST and non-severe thrombocytopenia (non-ST groups comprising pregnant women with AA. RESULTS: Of 61 AA patients, 43 (70% were diagnosed as AA before pregnancy and 18 (30% were AA during pregnancy. The ST group exhibited lower gestational age at nadir of platelet count (26.0 versus 37.0 weeks, p<0.001 and at delivery (37.3 versus 39.1 weeks, p = 0.008, and a higher rate of bleeding gums (33.8 versus 7.7%, p = 0.015 than the non-ST group. In addition, the ST group exhibited more transfusions during pregnancy (72.7 versus 15.4%, p<0.001 and postpartum period (45.0 versus 2.7%, p<0.001, and more bone marrow transplant after delivery (25.0 versus 0.0%, p<0.001 than the non-ST group. The ST group had a higher odds ratio of composite disease complications (OR, 9.63; 95% CI, 2.82-32.9; p<0.001 and composite obstetric complications (OR, 6.78; 95% CI, 2.11-21.8; p = 0.001 than the non-ST group. CONCLUSIONS: Severe thrombocytopenia is more associated with obstetric and disease complications than is non-severe thrombocytopenia in pregnant women with AA.

Modified immunosuppressive therapy with porcine antilymphocyte globulin plus delayed cyclosporine A in children with severe aplastic anemia.

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Cui, Qingya; Sha, Pingping; Chen, Haifei; Shen, Hongshi; Qin, Longmei; Li, Zhengyang; Wu, Tianqin; Wang, Zhaoyue

2018-01-01

Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CsA) is the standard treatment for children with severe aplastic anemia (SAA) with no human leukocyte antigen-matched siblings. Due to the unavailability of horse ATG in China, porcine antilymphocyte globulin (p-ALG), which is less expensive and more effective than rabbit ATG, is widely used. We sought to evaluate the efficacy and safety profile of modified IST with p-ALG plus delayed CsA at day 21 in 50 SAA children. Eighteen SAA patients who progressed from nonsevere aplastic anemia (NSAA) were classified as SAA-II; the other 32 patients were classified as SAA-I. Overall response (OR) rates at 3, 6 and 12 months were 56, 64 and 62%, respectively. The 10-year overall survival (OS) rate and disease-free survival (DFS) rate were 80 and 56%. The OR, OS and DFS rates in the SAA-I group were clearly better than those in the SAA-II group. Death rate from infection within 30 days was 4%. Modified IST with p-ALG plus delayed CsA is a reliable and well-tolerated treatment for children with SAA, and reduces early death due to infection. Modified IST is more suitable for children with SAA-I.

Long-term outcome of pregnancy complicating with severe aplastic anemia under supportive care.

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Chen, Kuan-Ju; Chang, Yao-Lung; Chang, Horng; Su, Shen-Yuan; Peng, Hsiu-Huei; Chang, Shuenn-Dyh; Chao, An-Shine

2017-10-01

Pregnancy associated with aplastic anemia (AA) is a rare and heterogeneous disorder. We aimed to identify and evaluate the maternal and pregnant outcomes of pregnancy-associated severe AA treated with supportive care. A 25-year retrospective study was conducted at in a single center between 1990 and 2014 with pregnancy associated severe AA. In addition, relevant published cases of antenatally diagnosed pregnancy-associated severe AA after 1990 were identified by PubMed. The main goal was to determine the impact of various risk factors on maternal and fetal outcomes. 15 women with 18 pregnancies were enrolled. With addition of the published reports in literature, a total of 36 cases were included for reference review. Univariate analysis showed that low platelet counts (<2.0 × 10 9 /L), bone marrow hypocellularity (<25%), and late diagnosis during pregnancy were predictors of poor maternal outcomes (P < 0.05). The complication rate of pregnancy outcomes was 53.3%, including preterm delivery, small gestational age (SGA), preterm premature ruptured of membranes (PPROM) and preeclampsia. This study identified the risk factors of mortality and morbidity in pregnant women with severe AA, as well as the obstetrical complications associated with neonatal outcome. Copyright © 2017. Published by Elsevier B.V.

Postpartum Aplastic Anemia Presenting as Pancytopenia Due to Malarial Infection

International Nuclear Information System (INIS)

Shah, M. U.; Sundhu, M. A.; Hussain, M. Z.

2013-01-01

Pancytopenia is a condition with decreased numbers of all cell lines. Aplastic anemia is a common cause although malarial infection causing lysis of RBCs may also partly mimic this condition. The infection may also damage the patient's bone marrow resulting in pancytopenia as well. We present the case of a post-partum female patient who reported with fever, body aches and shortness of breath one month after the delivery of her baby. All blood cell counts were decreased and peripheral blood smear showed malarial parasites. Anti-malarial treatment was initiated following which the fever subsided but, despite regular transfusions, the blood counts remained low. Bone marrow biopsy report revealed P. falciparum pigments along with hypocellularity characteristic of severe aplastic anemia. Consequently, bone marrow transplantation was advised as a therapeutic measure. This case report highlights the increased susceptibility of pregnant women to malaria in endemic areas and subsequent aplastic anemia. (author)

Aplastic Anemia

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Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... blood cells. There are different types, including Fanconi anemia. Causes include Toxic substances, such as pesticides, arsenic, ...

Evolving Hematopoietic Stem Cell Transplantation Strategies in Severe Aplastic Anemia

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Dietz, Andrew C.; Lucchini, Giovanna; Samarasinghe, Sujith; Pulsipher, Michael A.

2016-01-01

Purpose of Review Significant improvements in unrelated donor hematopoietic stem cell transplantation (HSCT) in recent years has solidified its therapeutic role in severe aplastic anemia (SAA) and led to evolution of treatment algorithms, particularly for children. Recent Findings Advances in understanding genetics of inherited bone marrow failure syndromes (IBMFS) have allowed more confidence in accurately diagnosing SAA and avoiding treatments that could be dangerous and ineffective in individuals with IBMFS, which can be diagnosed in 10–20% of children presenting with a picture of SAA. Additionally long-term survival after matched sibling donor (MSD) and matched unrelated donor (MUD) HSCT now exceed 90% in children. Late effects after HSCT for SAA are minimal with current strategies and compare favorably to late effects after up-front immunosuppressive therapy (IST), except for patients with chronic graft versus host disease (GVHD). Summary 1) Careful assessment for signs or symptoms of IBMFS along with genetic screening for these disorders is of major importance. 2) MSD HSCT is already considered standard of care for up-front therapy and some groups are evaluating MUD HSCT as primary therapy. 3) Ongoing studies will continue to challenge treatment algorithms and may lead to an even more expanded role for HSCT in SAA. PMID:26626557

Aplastic anemia and related disorders in atomic bomb survivors

International Nuclear Information System (INIS)

Ichimaru, Michito; Tomonaga, Yu; Matsunaga, Masako; Sadamori, Naoki; Ishimaru, Toranosuke.

1978-01-01

Whether the incidence of aplastic anemia significantly increases due to the later effect of atomic-bomb radiation was studied. After the data of aplastic anemia which occurred within 1950 - 1973 were evaluated and the diagnoses of the cases were certified, the incidence of aplastic anemia per 109,000 inhabitants of the cities of Hiroshima and Nagasaki was calculated and compared according to the dose of atomic-bomb radiation. There was no increase in the incidence according to an increase in radiation dose, and there was no fact that aplastic anemia increased in a certain period either. Most of the atomic-bomb survivors who were close to the epicenter and were clinically diagnosed as aplastic anemia had leukemia lesion or myeloid proliferating lesion, and it is likely to be that pathological changes resembling aplastic anemia may appear in a certain phase of myeloid proliferation or as a phenotype of myeloid proliferation. An evaluation was made on cases of aplastic anemia of other groups, but the doses of atomic-bomb radiation which they received were not so much to give effect on the bone marrow except only two cases. (Ueda, J.)

Clinical management of aplastic anemia

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DeZern, Amy E; Brodsky, Robert A

2011-01-01

Acquired aplastic anemia is a potentially fatal bone marrow failure disorder that is characterized by pancytopenia and a hypocellular bone marrow. Hematopoietic stem-cell transplantation or bone marrow transplantation (BMT) is the treatment of choice for young patients who have a matched sibling donor. Immunosuppression with either anti-thymocyte globulin and cyclosporine or high-dose cyclophosphamide is an effective therapy for patients who are not suitable BMT candidates owing to age or lack of a suitable donor. Results of BMT from unrelated and mismatched donors are improving, but presently this treatment option is best reserved for those patients who do not respond, relapse or develop secondary clonal disorders following immunosuppressive therapy. Efforts are currently underway to both improve immunosuppressive regimens and to expand the application of BMT. PMID:21495931

Epidemiology of aplastic anemia in Japanese radiological technicians

International Nuclear Information System (INIS)

Kitabatake, Takashi; Watanabe, Tsuyoshi; Saito, Akira; Nakamura, Minoru.

1976-01-01

Among Japanese radiological technicians, four deaths from aplastic anemia have been recorded. Based on this fact, some epidemiological considerations are tried. During the period from 1930 to 1960, the population of radiological technicians is estimated to be 74,400 man-years, in which 0.5 aplastic anemias are expected. However actually three died from aplastic anemia. This difference is statistically significant at the 1% level. On the other hand, in the period from 1961 to 1973, the observed value is 1 against 0.7 expected. It is concluded that aplastic anemia had been frequently induced among Japanese radiological technicians in the era when there was much exposure to occupational radiation. (auth.)

Hematopoietic Acute Radiation Syndrome (Bone marrow syndrome, Aplastic Anemia): Molecular Mechanisms of Radiation Toxicity.

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Popov, Dmitri

Key Words: Aplastic Anemia (AA), Pluripotential Stem Cells (PSC) Introduction: Aplastic Anemia (AA) is a disorder of the pluripotential stem cells involve a decrease in the number of cells of myeloid, erythroid and megakaryotic lineage [Segel et al. 2000 ]. The etiology of AA include idiopathic cases and secondary aplastic anemia after exposure to drugs, toxins, chemicals, viral infections, lympho-proliferative diseases, radiation, genetic causes, myelodisplastic syndromes and hypoplastic anemias, thymomas, lymphomas. [Brodskyet al. 2005.,Modan et al. 1975., Szklo et al. 1975]. Hematopoietic Acute Radiation Syndrome (or Bone marrow syndrome, or Radiation-Acquired Aplastic Anemia) is the acute toxic syndrome which usually occurs with a dose of irradiation between 0.7 and 10 Gy (70- 1000 rads), depending on the species irradiated. [Waselenko et al., 2004]. The etiology of bone morrow damage from high-level radiation exposure results depends on the radiosensitivity of certain bone marrow cell lines. [Waselenko et al. 2004] Aplastic anemia after radiation exposure is a clinical syndrome that results from a marked disorder of bone marrow blood cell production. [Waselenko et al. 2004] Radiation hematotoxicity is mediated via genotoxic and other specific toxic mechanisms, leading to aplasia, cell apoptosis or necrosis, initiation via genetic mechanisms of clonal disorders, in cases such as the acute radiation-acquired form of AA. AA results from radiation injury to pluripotential and multipotential stem cells in the bone marrow. The clinical signs displayed in reticulocytopenia, anemia, granulocytopenia, monocytopenia, and thrombocytopenia. The number of marrow CD34+ cells (multipotential hematopoietic progenitors) and their derivative colony-forming unit{granulocyte-macrophage (CFU-GM) and burst forming unit {erythroid (BFU{E) are reduced markedly in patients with AA. [Guinan 2011, Brodski et al. 2005, Beutler et al.,2000] Cells expressing CD34 (CD34+ cell) are normally

Clinical study of aplastic anemia among A-bomb survivors

International Nuclear Information System (INIS)

Oguma, Nobuo; Dohy, Hiroo; Kyo, Taiichi; Saito, Osamu; Okita, Hajime

1980-01-01

In 90 patients with aplastic anemia who were seen at Dept. Med. RINMB, Hiroshima Univ. from 1962 to March, 1980, clinical findings of 33 A-bomb survivors (which included the second generation of the survivors) and those of 57 nonexposed patients were compared. No relationship was found between the age at the time of exposure and the period preceding onset of the disease. The A-bomb survivors showed higher neutrophil counts and higher reticulocyte counts than the nonexposed patients. There were less severe cases in the A-bomb survivors. There was no difference in the incidence of atypical aplastic anemia between the exposed patients and the nonexposed ones. No difference was found in overall survival (one-year and five-year survival rates) between the exposed and the nonexposed. The A-bomb survivors often had complete remission or maintenance of remission, and rarely had acute progression. These results suggested that clinical picture of aplastic anemia in the A-bomb survivors is different from that in the nonexposed patients. (Ueda, J.)

Clonal Evolution and Clinical Significance of Copy Number Neutral Loss of Heterozygosity of Chromosome Arm 6p in Acquired Aplastic Anemia

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Betensky, Marisol; Babushok, Daria; Roth, Jacquelyn J.; Mason, Philip J; Biegel, Jaclyn A.; Busse, Tracy M; Li, Yimei; Lind, Curt; Papazoglou, Anna; Monos, Dimitri; Podsakoff, Gregory; Bessler, Monica; Olson, Timothy S.

2015-01-01

Acquired aplastic anemia (aAA) results from the T cell-mediated autoimmune destruction of hematopoietic stem cells. Factors predicting response to immune suppression therapy (IST) or development of myelodysplastic syndrome (MDS) are beginning to be elucidated. Our recent data suggest most patients with aAA treated with IST develop clonal somatic genetic alterations in hematopoietic cells. One frequent acquired abnormality is copy-number neutral loss of heterozygosity on chromosome 6p (6p CN-LOH) involving the human leukocyte antigen (HLA) locus. We hypothesized that because 6p CN-LOH clones may arise from selective pressure to escape immune surveillance through deletion of HLA alleles, the development of 6p CN-LOH may affect response to IST. We used single nucleotide polymorphism array genotyping and targeted next-generation sequencing of HLA alleles to assess frequency of 6p CN-LOH, identity of HLA alleles lost through 6p CN-LOH, and impact of 6p CN-LOH on response to IST. 6p CN-LOH clones were present in 11.3% of patients, remained stable over time, and were not associated with development of MDS-defining cytogenetic abnormalities. Notably, no patient with 6p CN-LOH treated with IST achieved a complete response. In summary, clonal 6p CN-LOH in aAA defines a unique subgroup of patients that may provide insights into hematopoietic clonal evolution. PMID:26702937

A rare association of celiac disease and aplastic anemia: case report of a child and review of literature.

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Badyal, Rama Kumari; Sachdeva, Man Updesh Singh; Varma, Neelam; Thapa, Babu Ram

2014-01-01

An association between severe aplastic anemia and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus, and thyroid disorders. Herein we report a patient with celiac disease who was not strictly following a gluten-free diet and presented with progressive pallor, fever, and weakness of 1 month's duration. On investigation, he had pancytopenia, which on subsequent evaluation revealed aplastic anemia. An association between aplastic anemia and celiac disease has rarely been reported. To the best of author's knowledge, only 1 pediatric case of celiac disease associated with aplastic anemia has been published. This is the second report to suggest such an association in children.

[Comparison of two types of antithymocyte globulin in the treatment of children with aplastic anemia].

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Xie, X T; He, W; Shi, W; Zhou, X X; Qiao, X H

2016-04-01

This study was designed to compare the effects of the anti-human T lymphocyte globulin (Fresenius, ATG-F)and rabbit anti-human thymocyte immunoglobulin (Genzyme, R-ATG)in the treatment of childhood aplastic anemia (AA) and their effects. A total of 59 children with aplastic anemia were analyzed in the present study, including 34 cases of severe aplastic anemia (SAA), 12 cases of very severe aplastic anemia (VSAA) and 13 cases of transfusion-dependent non-severe aplastic anemia (NSAA). While receiving immunosuppressive therapy (IST), 30 and 29 patients, with long-term oral supplement with cyclosporin A (CSA), androgen and Chinese traditional medicines, were treated with ATG-F and R-ATG, respectively. When it was necessary, some supportive cares such as component transfusion and infection control were also employed. Absolute counts of peripheral blood lymphocyte (ALC) at various time points were dynamically detected after ATG therapy. According to the International Aplastic Anemia Treatment and Effect standards. There were no statistically significant differences in the overall response rate (67%(20/30)vs. 69%(20/29), χ(2)=0.036, P=0.676) and the survival rate (87%(26/30)vs. 83%(24/29), χ(2)=0.173, P=0.676) between the ATG-F and R-ATG groups. There were significant and long-term ALC decrease after ATG therapy, the rate of ALC decrease in ATG-F and R-ATG group, the ALC only recovered to 47.8% (ATG-F group) and 47.4% (R-ATG group) of the pre-treatment level respectively. ATG-F 5 mg/(kg·d) and R-ATG 3.75 mg/(kg·d)could achieve similar effects in the treatment of childhood AA, through similar significant clearance of T cells. Therefore, all of these suggest that ATG-F and R-ATG might serve as the drugs of front-line choice for IST in childhood AA patients who do not have an available human leukocyte antigen identical related donor.

APLASTIC ANEMIA ET CAUSA OF SUSPECT VIRAL HEPATITIS INFECTION: A CASE REPORT

OpenAIRE

I Wayan Wawan Lismana

2014-01-01

Aplastic anemia is anemia that occurs because of a failure of hematopoiesis is relatively rarebut can be life threatening. The cause of aplastic anemia itself is still largely unknown oridiopathic. Minority of cases mainly due to a virus infection, one of which is viral hepatitishas long been known to cause symptoms of aplastic anemia. This report discusses thesuspected aplastic anemia caused by hepatitis virus infection. Course of the disease or theprognosis of aplastic anemia varies, but a ...

[Meta-analysis of association between organophosphorus pesticides and aplastic anemia].

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Zhang, Ji; Yang, Tubao

2015-09-01

To evaluate the association between organophosphorus pesticides and aplastic anemia, and provide scientific evidence for the primary prevention of aplastic anemia. The published papers of case control studies on the association between organophosphorus pesticides and aplastic anemia from January 1990 to August 2014 were collected from Chinese BioMedical Literature Base (CBM), Chinese National Knowledge Infrastructure (CNKI), PubMed and EMBASE. The papers which met the inclusion criteria were evaluated. The pooled odds ratios (OR) and 95% confidence interval (CI) of organophosphorus pesticides were calculated with software Review Manager 5.0. Subgroup analysis were conducted for different population and different usage of organophosphorus pesticides. A total of 9 papers were selected, involving 5 833 subjects (1 404 cases and 4 429 controls). The results showed that organophosphorus pesticides could increase the risk of aplastic anemia (OR=1.97, 95% CI: 1.60-2.44) . Subgroup analysis showed that Asian (OR=2.01, 95% CI: 1.52-2.66) had higher risk of aplastic anemia than American or European (OR=1.93, 95% CI: 1.39-2.67) . Using pure organophosphorus pesticides (OR=2.15, 95% CI: 1.60-2.88) was more prone to cause aplastic anemia than using the mixture of organophosphorus pesticides (OR=1.82, 95% CI: 1.34-2.47). The analysis indicated that organophosphorus pesticides might be a risk factor for aplastic anemia. Reducing organophosphorus pesticides exposure in daily life and industrial or agricultural production could prevent the incidence of aplastic anemia.

Syngeneic transplantation in aplastic anemia

DEFF Research Database (Denmark)

Gerull, Sabine; Stern, Martin; Apperley, Jane

2013-01-01

Aplastic anemia is usually treated with immunosuppression or allogeneic transplant, depending on patient and disease characteristics. Syngeneic transplant offers a rare treatment opportunity with minimal transplant-related mortality, and offers an insight into disease mechanisms. We present here...... a retrospective analysis of all syngeneic transplants for aplastic anemia reported to the European Group for Blood and Marrow Transplantation. Between 1976 and 2009, 88 patients received 113 transplants. Most transplants (n=85) were preceded by a conditioning regimen, 22 of these including anti-thymocyte globulin...

High Dose Cyclophosphamide without Stem Cell Rescue in 207 Patients with Aplastic anemia and other Autoimmune Diseases

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DeZern, Amy E.; Petri, Michelle; Drachman, Daniel B.; Kerr, Doug; Hammond, Edward R.; Kowalski, Jeanne; Tsai, Hua-Ling; Loeb, David M.; Anhalt, Grant; Wigley, Fredrick; Jones, Richard J.; Brodsky, Robert A.

2011-01-01

High-dose cyclophosphamide has long been used an anticancer agent, a conditioning regimen for hematopoietic stem cell transplantation and as potent immunosuppressive agent in autoimmune diseases including aplastic anemia. High-dose cyclophosphamide is highly toxic to lymphocytes but spares hematopoietic stem cells because of their abundant levels of aldehyde dehydrogenase, the major mechanism of cyclophosphamide inactivation. High dose cyclophosphamide therapy induces durable remissions in most patients with acquired aplastic anemia. Moreover, high-dose cyclophosphamide without hematopoietic stem cell rescue has shown activity in a variety of other severe autoimmune diseases. Here we review the history of cyclophosphamide as is applies to aplastic anemia (AA) and other autoimmune diseases. Included here are the historical data from early patients treated for AA as well as an observational retrospective study in a single tertiary care hospital. This latter component was designed to assess the safety and efficacy of high-dose cyclophosphamide therapy without stem cell rescue in patients with refractory autoimmune diseases. We analyzed fully the 140 patients with severe, progressive autoimmune diseases treated. All patients discussed here received cyclophosphamide, 50 mg/kg per day for 4 consecutive days. Response, relapse and overall survival were measured. Response was defined as a decrease in disease activity in conjunction with a decrease or elimination of immune modulating drugs. Relapse was defined as worsening disease activity and/or a requirement of an increase in dose of, or administration of new, immunosuppressive medications. Hematologic recovery occurred in all patients. The overall response rate of the was 95%, and 44% of those patients remain progression-free with a median follow up time of 36 (range 1–120) months for the 140 patients analyzed together. The overall actuarial and event free survival across all diseases at 60 months is 90.7% and 20

Cotransplantation of haploidentical hematopoietic and umbilical cord mesenchymal stem cells for severe aplastic anemia: Successful engraftment and mild GVHD

Directory of Open Access Journals (Sweden)

Wu Yamei

2014-01-01

Full Text Available Haploidentical hematopoietic stem-cell transplantation (haplo-HSCT is associated with an increased risk of graft failure and severe graft-versus-host disease (GVHD. Mesenchymal stromal cells (MSCs have been shown to support in vivo normal hematopoiesis and to display potent immunesuppressive effects. We cotransplanted the culture-expanded third-party donor-derived umbilical cord MSCs (UC-MSCs in 21 young people with severe aplastic anemia (SAA undergoing haplo-HSCT without T-cell-depleted. We observed that all patients had sustained hematopoietic engraftment without any adverse UC-MSC infusion-related events. Furthermore, we did not observe any increase in severe aGVHD. These data suggest that UC-MSCs, possibly thanks to their potent immunosuppressive effect on allo-reactive host T lymphocytes escaping the preparative regimen, reduce the risk of graft failure and severe GVHD in haplo-HSCT.

Prospective study of rabbit antithymocyte globulin and cyclosporine for aplastic anemia from the EBMT Severe Aplastic Anaemia Working Party.

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Marsh, Judith C; Bacigalupo, Andrea; Schrezenmeier, Hubert; Tichelli, Andre; Risitano, Antonio M; Passweg, Jakob R; Killick, Sally B; Warren, Alan J; Foukaneli, Theodora; Aljurf, Mahmoud; Al-Zahrani, H A; Höchsmann, Britta; Schafhausen, Philip; Roth, Alexander; Franzke, Anke; Brummendorf, Tim H; Dufour, Carlo; Oneto, Rosi; Sedgwick, Philip; Barrois, Alain; Kordasti, Shahram; Elebute, Modupe O; Mufti, Ghulam J; Socie, Gerard

2012-06-07

Rabbit antithymocyte globulin (rATG; thymoglobulin, Genzyme) in combination with cyclosporine, as first-line immunosuppressive therapy, was evaluated prospectively in a multicenter, European, phase 2 pilot study, in 35 patients with aplastic anemia. Results were compared with 105 age- and disease severity-matched patients from the European Blood and Marrow Transplant registry, treated with horse ATG (hATG; lymphoglobulin) and cyclosporine. The primary end point was response at 6 months. At 3 months, no patients had achieved a complete response to rATG. Partial response occurred in 11 (34%). At 6 months, complete response rate was 3% and partial response rate 37%. There were 10 deaths after rATG (28.5%) and 1 after subsequent HSCT. Infections were the main cause of death in 9 of 10 patients. The best response rate was 60% for rATG and 67% for hATG. For rATG, overall survival at 2 years was 68%, compared with 86% for hATG (P = .009). Transplant-free survival was 52% for rATG and 76% for hATG (P = .002). On multivariate analysis, rATG (hazard ratio = 3.9, P = .003) and age more than 37 years (hazard ratio = 4.7, P = .0008) were independent adverse risk factors for survival. This study was registered at www.clinicaltrials.gov as NCT00471848.

Clonal evolution and clinical significance of copy number neutral loss of heterozygosity of chromosome arm 6p in acquired aplastic anemia.

Science.gov (United States)

Betensky, Marisol; Babushok, Daria; Roth, Jacquelyn J; Mason, Philip J; Biegel, Jaclyn A; Busse, Tracy M; Li, Yimei; Lind, Curt; Papazoglou, Anna; Monos, Dimitri; Podsakoff, Gregory; Bessler, Monica; Olson, Timothy S

2016-01-01

Acquired aplastic anemia (aAA) results from the T cell-mediated autoimmune destruction of hematopoietic stem cells. Factors predicting response to immune suppression therapy (IST) or development of myelodysplastic syndrome (MDS) are beginning to be elucidated. Our recent data suggest most patients with aAA treated with IST develop clonal somatic genetic alterations in hematopoietic cells. One frequent acquired abnormality is copy-number neutral loss of heterozygosity on chromosome 6p (6p CN-LOH) involving the human leukocyte antigen (HLA) locus. We hypothesized that because 6p CN-LOH clones may arise from selective pressure to escape immune surveillance through deletion of HLA alleles, the development of 6p CN-LOH may affect response to IST. We used single nucleotide polymorphism array genotyping and targeted next-generation sequencing of HLA alleles to assess frequency of 6p CN-LOH, identity of HLA alleles lost through 6p CN-LOH, and impact of 6p CN-LOH on response to IST. 6p CN-LOH clones were present in 11.3% of patients, remained stable over time, and were not associated with development of MDS-defining cytogenetic abnormalities. Notably, no patient with 6p CN-LOH treated with IST achieved a complete response. In summary, clonal 6p CN-LOH in aAA defines a unique subgroup of patients that may provide insights into hematopoietic clonal evolution. Copyright © 2016 Elsevier Inc. All rights reserved.

Aplastic Anemia and MDS International Foundation (AAMDSIF): Bone marrow failure disease scientific symposium 2016.

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Zeidan, Amer M; Battiwalla, Minoo; Berlyne, Deborah; Winkler, Thomas

2017-02-01

Patients with acquired and inherited bone marrow failure syndromes (BMFS) have ineffective hematopoiesis due to impairments of the hematopoietic stem cell compartment. Common manifestations of BMFS include varying degrees of peripheral blood cytopenias and, sometimes, progression to acute myelogenous leukemia. Research efforts have been made all over the world to improve understanding of the pathogenesis of these diseases and their clinical implications. The Aplastic Anemia and MDS International Foundation (AAMDSIF) is an independent nonprofit organization whose mission is to help patients and family members cope with BMFS. Here, we summarize recent scientific discoveries in several BMFS that were presented at the fifth International Bone Marrow Failure Disease Scientific Symposium 2016 that AAMDSIF sponsored on March 17-18, 2016, in Rockville, Maryland. Copyright © 2016 Elsevier Ltd. All rights reserved.

Epidemiological inference on induction of aplastic anemia following radiotherapy

International Nuclear Information System (INIS)

Kitabatake, Takashi; Saito, Akira; Sakai, Kunio; Inakoshi, Hideki; Watanabe, Tsuyoshi

1977-01-01

Some epidemiological inferences on possibility of induction of aplastic anemia following radiotherapy are tried. In Japan after 1969, there are detected and reported 11 cases of radiation-related aplastic anemia after radiotherapy diagnosed by hematologists. Of 11 cases, 2 are males and 9 are females, ranging 28 to 66 years of age. All these patients were irradiated for malignant diseases. The population at risk after 1969 was estimated to be 674, 660 man-years, based on the additional survey on number and survival rate of patients irradiated. The expected value of aplastic anemia calculated from this population at risk is 10.2, against 11 cases observed above-described. There is no statistically significant difference. Namely, there is no epidemiological evidence that aplastic anemia is induced by local-body irradiation such as radiotherapy. (auth.)

Colon cancer in a patient with underlying aplastic anemia: A clinical challenge

OpenAIRE

Wong, Hilda; Chan, Pierre; Yau, Thomas

2012-01-01

The association of gastrointestinal malignancy with aplastic anemia has rarely been reported in the literature. Although it is not clear whether there is any direct relationship between aplastic anemia and gastrointestinal cancers, a retrospective analysis did suggest the notion that patients with aplastic anemia might have a higher incidence of colorectal cancer. Here, we report the diagnostic and therapeutic challenges in managing a patient with aplastic anemia and advanced colorectal cance...

Acquired factor VII deficiency associated with acute myeloid leukemia.

Science.gov (United States)

Anoun, Soumaya; Lamchahab, Mouna; Oukkache, Bouchra; Qachouh, Maryam; Benchekroun, Said; Quessar, Asmaa

2015-04-01

Isolated acquired factor VII deficiency is a rare coagulopathy. It has been reported in 31 patients with malignancy, sepsis, postoperatively, aplastic anemia, and during bone marrow transplantation. We discuss, through a new case of acquired factor VII deficiency, the characteristics of this disease when it is associated with acute myeloid leukemia. Acquired factor VII deficiency in hematological diseases can be caused by intensive chemotherapy, infections, or hepatic dysfunction. The best treatment in developing countries remains corticosteroids associated with plasma exchange, frozen plasma, and antibiotics.

Long-term outcome of 25 children and adolescents with severe aplastic anemia treated with antithymocyte globulin

Directory of Open Access Journals (Sweden)

de-Medeiros C.R.

2000-01-01

Full Text Available Severe aplastic anemia (SAA is probably an immune-mediated disorder, and immunosuppressive therapy is recommended for patients with no available donor for bone marrow transplant. Between October 1984 and November 1987, 25 consecutive children and adolescents with SAA with no HLA-compatible marrow donor received equine antithymocyte globulin (ATG (15 mg kg-1 day-1 for 10 days. The patients were evaluated 6 weeks, 6 months, and 12 months after starting ATG treatment. Thereafter, patients were evaluated yearly until July 1998. Median age was 10 years (range, 1.5-20 years, granulocyte counts on referral ranged from 0.032 to 1.4 x 10(9/l (median 0.256 x 10(9/l, and 12 patients had granulocyte counts <0.2 x 10(9/l. At a median follow-up of 9.6 years (range, 8.6-11.8 years, 10 patients (40% remained alive with good marrow function. No morphologic evidence of hematological clonal disorders has been observed, although two patients probably have acquired clonal chromosomal abnormalities (trisomy 8 and del(6q21, respectively. Responses to ATG were observed between 6 weeks and 6 months from the start of treatment in 60% of evaluable patients. The response rate was not different in patients whose granulocyte count at diagnosis was <0.2 x 10(9/l, or in those who were <10 years of age. This study supports the view that, when compared with supportive measures, ATG is an effective treatment for children or adolescents with SAA. Although these results are inferior to those reported for marrow transplantation or more intensive immunosuppressive regimens, these patients who responded to ATG are long-term survivors with stable peripheral blood counts and a low rate of relapse.

Meta-analysis of treatment with rabbit and horse antithymocyte globulin for aplastic anemia.

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Hayakawa, Jin; Kanda, Junya; Akahoshi, Yu; Harada, Naonori; Kameda, Kazuaki; Ugai, Tomotaka; Wada, Hidenori; Ishihara, Yuko; Kawamura, Koji; Sakamoto, Kana; Ashizawa, Masahiro; Sato, Miki; Terasako-Saito, Kiriko; Kimura, Shun-Ichi; Kikuchi, Misato; Yamazaki, Rie; Kako, Shinichi; Kanda, Yoshinobu

2017-05-01

Aplastic anemia patients who received rabbit antithymocyte globulin exhibited response and survival rates inferior to those who received horse antithymocyte globulin in several studies. Therefore, we conducted a meta-analysis to compare rabbit and horse antithymocyte globulin as immunosuppressive therapy for aplastic anemia. We searched online databases for studies that compared antithymocyte globulin regimens as first-line treatment for aplastic anemia, including both randomized and non-randomized controlled trials. The early mortality rate at 3 months and overall response rate at 6 months were evaluated. Thirteen studies were included in the analysis. The risk ratio (RR) of early mortality for rabbit vs. horse antithymocyte globulin was 1.33 [95% confidence interval (CI) 0.69-2.57; P = 0.39], with significant heterogeneity. A sensitivity analysis suggested higher early mortality rate in patients who received rabbit antithymocyte globulin. The overall response rate was significantly higher in patients who received horse antithymocyte globulin (RR 1.27; 95% CI 1.05-1.54; P = 0.015). In conclusion, in aplastic anemia patients treated with ATG, early mortality rate was not significantly different in patients receiving horse or rabbit ATG, although a sensitivity analysis showed higher early mortality in the rabbit ATG group. Horse ATG was associated with significantly higher response rate than rabbit ATG.

Expectation of aplastic anemia following radiotherapy for malignancy

International Nuclear Information System (INIS)

Kitabatake, T.; Sakai, K.; Saito, A.

1978-01-01

In Japan after 1969, 11 cases of aplastic anemia following radiotherapy for malignant disease were detected. The population at risk in irradiated patients was estimated at 674,664 man-years. The expected cases of aplastic anemia in this population were calculated as 10.1. There is no statistically significant difference between the expected and the observed values

Aplastic anaemia: a review

NARCIS (Netherlands)

S. Sleijfer (Stefan); P.J. Lugtenburg (Pieternella)

2003-01-01

textabstractAplastic anaemia is featured by bone marrow hypocellularity and peripheral pancytopenia and is a potentially fatal disease. In recent years, insight in it pathogenesis has increased. It appears that activated autoreactive T lymphocytes induce apoptosis of haematopoietic

Clinical studies on bone marrow transplantation of acute leukemia and aplastic anemia

International Nuclear Information System (INIS)

Morishima, Yasuo

1979-01-01

Since 1974, we have done bone marrow transplantation (BMT) in six patients of acute leukemia and two of aplastic anemia. Leukemia patients were premedicated by CY+TBI method; cyclophosphamide (CY) 60 mg/kg/day was administered for two successive days and two days later, total body irradiation (TBI) was done in a dose of 800 - 1000 rad at a rate of 20-28 rad/min by linear accerelator. Patients with aplastic anemia were premedicated by CY method; CY 50 mg/kg/day for four successive days. Bone marrow graft was obtained from donor under general anesthesia. The nucleated bone marrow cells, ranged from 0.7 x 10 10 to 1.4 x 10 10 were transfused into the patient intravenously. Any lethal side effects did not develop in all patient during these procedures. Two died on day 10 and 12 with septicemia. The other 6 patients showed engraftment of bone marrow indicated by rising blood counts, return of marrow cellularity and in one case by blood cytogenetic markers. Relapse of leukemia did not occur in five patients treated with CY + TBI method. Three patients with allogeneic BMT developed moderately severe to severe Graft versus Host Disease. Survival time after BMT were 12, 35, 63, 68, 98, 125 days. 15 months in leukemia, and 10 days, 12 + months in aplastic anemia. (author)

Management of aplastic anaemia in pregnancy in a resource poor ...

African Journals Online (AJOL)

Management of aplastic anaemia in pregnancy in a resource poor centre. ... This is case of a 37 year old Nigerian woman G6P0+5managed with repeated blood ... aplastic anaemia following presentation with weakness and gingival bleeds.

Outcome of pregnancy and disease course among women with aplastic anemia treated with immunosuppression

OpenAIRE

MCCANN, SHAUN

2002-01-01

PUBLISHED Background: Aplastic anemia may develop during pregnancy and sometimes improves spontaneously after delivery. The effects of pregnancy on aplastic anemia after immunosuppressive treatment and of aplastic anemia on the outcome of pregnancy have not been described. Objective: To determine the outcome of pregnancy and the disease course among women with aplastic anemia who received immunosuppressive therapy. Design: Retrospective multicenter study. Setting: Twelve cen...

Features of Hepatitis in Hepatitis-associated Aplastic Anemia: Clinical and Histopathologic Study.

Science.gov (United States)

Patel, Kalyani R; Bertuch, Alison; Sasa, Ghadir S; Himes, Ryan W; Wu, Hao

2017-01-01

Hepatitis-associated aplastic anemia (HAA) is a rare variant of aplastic anemia in which patients present with severe pancytopenia after an episode of acute hepatitis. The marrow failure is often rapid, severe, and usually fatal if untreated. The preceding hepatitis is largely under-studied. Retrospective study of the clinical and histopathologic features of hepatitis in pediatric patients who subsequently developed aplastic anemia and comparison with consecutive cases of acute liver failure and random cases of autoimmune hepatitis during the same time frame. All 7 patients of HAA had significant elevations in aminotransferases and conjugated hyperbilirubinemia at initial presentation. Echoing liver function indices, cholestatic hepatitis with sinusoidal obstruction-type endothelial injury was seen histomorphologically. Autoimmune hepatitis serology such as anti-F-actin, anti-liver/kidney microsome, and hypergammaglobulinemia was negative in all patients. Five of 7 patients (71.4%) had, however, elevated antinuclear antibody, all with a speckled pattern. Hepatitis virus serology was negative in all patients. By immunohistochemical staining, the lobular CD8/CD4 lymphocyte ratio was markedly elevated in all of the initial samples with significant reduction in this ratio (P = 0.03) in 3 patients post treatment (ursodiol, antibiotics, and/or immunosuppressive therapy). Hepatitis preceding HAA is characterized by marked elevation of aminotransferases, conjugated hyperbilirubinemia, elevated antinuclear antibody with a speckled pattern, cholestatic hepatitis with sinusoidal obstruction morphology, and CD8 dominant lobular infiltrates. The present study suggests HAA may result from cytotoxic T-cell-mediated sinusoidal endothelial and hepatocytic injury.

Transforming growth factor 15 increased in severe aplastic anemia patients.

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Shao, Yuanyuan; Wang, Honglei; Liu, Chunyan; Cao, Qiuying; Fu, Rong; Wang, Huaquan; Wang, Ting; Qi, Weiwei; Shao, Zonghong

2017-10-01

The patients with severe aplastic anemia (SAA) usually rely on red cell transfusion which lead to secondary iron overload. Transforming growth differentiation factor-15 (GDF-15) plays an important role in erythropoiesis and iron regulation. In this study, we investigated the level of GDF-15 and other indexes of iron metabolism in SAA patients to explore the correlation with GDF-15 and iron overload in SAA. The levels of serum GDF-15, hepcidin (Hepc), and erythropoietin (EPO) were determined by ELISA. The levels of serum iron (SI), ferritin, TIBC, and transferrin saturation (TS) were measured by an auto analyzer. Iron staining of bone marrow cells was used for testing extracellular and intracellular iron. The GDF-15 level in the experimental group was higher than that of the case-control group and normal control group (all p < 0.05). The Hepc level in the experimental group and case-control group were both higher than that of healthy controls (all p < 0.05). The Hepc level was significantly lower in the experimental group patients who had excessive GDF-15 (r = -0.766, p = 0.000). There was a positive correlation between the level of GDF15 and EPO in the experimental group (r = 0.68, p < 0.000). The level of GDF15 in SAA patients was positively correlated with SI levels (r = 0.537, p = 0.008), TS levels (r = 0.466, p = 0.025), and sideroblasts (%) (r = 0.463, p = 0.026). Moreover, there was a positive correlation between GDF-15 level and blood transfusion-dependent time (r = 0.739, p = 0.000). Our data indicated that GDF-15 plays an important role in iron metabolism in SAA. GDF-15 might be a novel target for SAA therapy.

[Immunotherapy in aplastic anaemia as a cause of reactivation of hepatitis B virus-immunologic aspects].

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Luczyński, Włodzimierz; Muszyńska-Rosłan, Katarzyna; Krawczuk-Rybak, Maryna; Lebensztejn, Dariusz M

2005-01-01

We present history of 16-year-old boy, HBsAg carrier, treated with interferon alpha at the age of 6 because of hepatitis B (HBeAg/antyHBe seroconversion). In August 2002--admitted to Department of Pediatric Oncology due to pancytopenia--diagnosis of severe aplastic anaemia was made (bone marrow cellularity--10%). We found no relative donor for hematopoietic cells transplantation and started immunosuppresive therapy (ATG, G-CSF, methyloprednisolon, cyclosporin). Haematologic parameters were improving. At day +60 he was admitted to our Department due to the increase in aminotransferases and cyclosporin activity. He was treated with cefuroxim, acyclovir and drugs improving liver cell function, cyclosporin was stopped. Presence of HBV DNA in serum confirmed HBV reactivation--a boy received lamivudine and cyclosporin again (as a maintenance therapy of aplastic anaemia). Aminotransferase activity and haematological parameters returned to normal. This case indicates the possibility of HBV reactivation in the course of immunosuppressive therapy (e.g. after antithymocytic globulin and cyclosporin) for aplastic anaemia.

Immunosuppressive treatment for aplastic anemia: are we hitting the ceiling?

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Passweg, Jakob R.; Tichelli, André

2009-01-01

The combination of antithymocyte globulin of horse origin and cyclosporine A is the standard treatment for aplastic anemia in patients not eligible for bone marrow transplantation. In this perspective article, Drs. Passweg and Tichelli discuss the current immunosuppressive therapy of aplastic anemia. See related article on page 348.

The cutting edge of aplastic anemia treatment.

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Obara, Naoshi

2017-01-01

Aplastic anemia is a syndrome in which hematopoietic stem cells are decreased and bone marrow hypoplasia and pancytopenia are observed; it is considered as a T cell-mediated autoimmune disease. Recently, it has been reported that gene mutations suggestive of clonal hematopoiesis are detected in approximately one third of the patients with aplastic anemia. Among treatment approaches other than hematopoietic stem cell transplantation, immunosuppressive therapy with antithymocyte globulin (ATG) plus cyclosporin is a basic approach, although it has been shown that eltrombopag, a thrombopoietin receptor agonist, is effective and that the recovery of hematopoiesis in three blood lineage is observed in some patients. Studies on the optimum dose of ATG are in progress. Regarding hematopoietic stem cell transplantation for aplastic anemia, regimens are being designed in which cyclophosphamide as a pretreatment is reduced and fludarabine is instead used in combination for the reduction of cardiotoxicity. Because HLA haploidentical transplantation has been developed and its reports are increasing for patients who cannot find appropriate donors, transplantation may be possible in patients who had previously given up on it.

Anemia Aplástica e Gravidez: Relato de Caso Aplastic Anemia and Pregnancy: A Case Report

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Rosiane Alves de Sousa Teles

2002-06-01

Full Text Available A anemia aplástica é distúrbio caracterizado por pancitopenia e medula óssea hipocelular, com substituição gordurosa dos elementos e sem nenhum sinal de transformação maligna ou doença mieloproliferativa. Acomete geralmente adultos jovens e idosos, sem preferência sexual. A maioria dos casos é adquirida, mas pode ocorrer hereditariamente, por distúrbio molecular (anemia de Fanconi. A associação com gravidez é rara, estando relacionada com alta morbidade e mortalidade materna e fetal. Os autores descrevem o caso de uma paciente com anemia aplástica, diagnosticada previamente, cuja gestação complicou com infecção do trato urinário, doença hipertensiva específica da gestação e restrição de crescimento fetal, com parto prematuro eletivo. Apesar das condições adversas na gravidez e parto, mãe e recém-nascido tiveram evolução clínica satisfatória.Aplastic anemia is characterized by a circulating pancytopenia, hypocellularity, and fatty replacement of cellular marrow elements, without evidence of malignant transformation or myeloproliferative disease. It usually affects young and senior adults, without any sexual preference. Most cases of aplastic anemia are acquired, but the disease may also be inherited due to a molecular disorder (Fanconi's anemia. Aplastic anemia in pregnancy is an extremely rare condition with high maternal and fetal morbidity and mortality rates. The authors describe a case of a patient with previously diagnosed aplastic anemia, whose pregnancy was complicated with urinary tract infection, preeclampsia and fetal growth restriction, with elective preterm birth. In spite of the adverse conditions in pregnancy and delivery, mother and newborn had a satisfactory clinical evolution.

Incidence and risk factors of aplastic anemia in Latin American countries: the LATIN case-control study

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Maluf, Eliane; Hamerschlak, Nelson; Cavalcanti, Alexandre Biasi; Júnior, Álvaro Avezum; Eluf-Neto, José; Falcão, Roberto Passetto; Lorand-Metze, Irene G.; Goldenberg, Daniel; Santana, Cézar Leite; de Oliveira Werneck Rodrigues, Daniela; da Motta Passos, Leny Nascimento; Rosenfeld, Luis Gastão Mange; Pitta, Marimilia; Loggetto, Sandra; Feitosa Ribeiro, Andreza A.; Velloso, Elvira Deolinda; Kondo, Andrea Tiemi; de Miranda Coelho, Erika Oliveira; Pintão, Maria Carolina Tostes; de Souza, Hélio Moraes; Borbolla, José Rafael; Pasquini, Ricardo

2009-01-01

Background Associations between aplastic anemia and numerous drugs, pesticides and chemicals have been reported. However, at least 50% of the etiology of aplastic anemia remains unexplained. Design and Methods This was a case-control, multicenter, multinational study, designed to identify risk factors for agranulocytosis and aplastic anemia. The cases were patients with diagnosis of aplastic anemia confirmed through biopsy or bone marrow aspiration, selected through an active search of clinical laboratories, hematology clinics and medical records. The controls did not have either aplastic anemia or chronic diseases. A total of 224 patients with aplastic anemia were included in the study, each case was paired with four controls, according to sex, age group, and hospital where the case was first seen. Information was collected on demographic data, medical history, laboratory tests, medications, and other potential risk factors prior to diagnosis. Results The incidence of aplastic anemia was 1.6 cases per million per year. Higher rates of benzene exposure (≥30 exposures per year) were associated with a greater risk of aplastic anemia (odds ratio, OR: 4.2; 95% confidence interval, CI: 1.82–9.82). Individuals exposed to chloramphenicol in the previous year had an adjusted OR for aplastic anemia of 8.7 (CI: 0.87–87.93) and those exposed to azithromycin had an adjusted OR of 11.02 (CI 1.14–108.02). Conclusions The incidence of aplastic anemia in Latin America countries is low. Although the research study centers had a high coverage of health services, the underreporting of cases of aplastic anemia in selected regions can be discussed. Frequent exposure to benzene-based products increases the risk for aplastic anemia. Few associations with specific drugs were found, and it is likely that some of these were due to chance alone. PMID:19734415

Severe recurrent achalasia cardia responding to treatment of severe autoimmune acquired haemophilia

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Al-Jafar, H; Laffan, M; Al-Sabah, S; Elmorsi, M; Habeeb, M; Alnajar, F

2012-01-01

Acquired haemophilia A and severe acquired achalasia are both very rare conditions with unknown aetiology. Haemophilia A is a haemorrhagic disease induced by deficiency or malfunction of coagulation factor VIII. Congenital haemophilia is an inherited disease transmitted by the mother through X-linked inheritance and primarily affects males. However, acquired haemophilia A is a serious, sudden-onset, autoimmune disease that affects either sex. In addition, achalasia is a disease of the oesopha...

Recurrent life-threatening reactions to platelet transfusion in an aplastic anaemia patient with a paroxysmal nocturnal haemoglobinuria clone.

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Mohamed, M; Bates, G; Richardson, D; Burrows, L

2014-09-01

A 60-year-old woman was diagnosed with non-severe aplastic anaemia when she presented with anaemia and thrombocytopenia. She developed recurrent life-threatening hypotensive reactions during transfusion of leukodepleted platelet concentrates, and washed platelet concentrates prevented the development of such reactions subsequently. A paroxysmal nocturnal haemoglobinuria clone was detected on investigating for aplastic anaemia, which has been speculated to play a role in the recurrent hypotensive reactions. © 2014 The Authors; Internal Medicine Journal © 2014 Royal Australasian College of Physicians.

A comparative immunohistochemical study of aplastic thymuses for lymphocytic, epithelial, proliferative and apoptotic indices

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Mahjoub F.

2008-03-01

Full Text Available Background: Immune deficiency is one of the major causes of morbidity and mortality in the modern world. Primary immunodeficiency comprises a wide range of disorders that mainly manifest in early childhood as devastating infections with opportunistic organisms. Thymic aplasia is found on autopsy of some patients afflicted with immune deficiency disorders, such as DiGeorge syndrome and severe combined immunodeficiency (SCID. After a thorough search of the literature, we found little information on the cellular characteristics of these thymuses. Our study aims to elucidate role of apoptosis in the pathogenesis of thymic aplasia and compare various lymphocytic and epithelial markers in normal and aplastic thymuses. Methods: We selected 12 subjects who died of severe infections with aplastic thymus found on autopsy, and 11 control subjects who died of unrelated causes, such as congenital heart disease. The presence of several markers, including Bcl2, P53, lymphocytic markers, and CD68, was examined using immunohistochemical methods on paraffin-embedded thymus sections. Positively-stained cells were counted per 1000 cells and the results stated as percentage of positive cells.                        Results: The mean age of the control group was between 7 days to 18 months (mean: 4.5 months. Parental consanguinity was present in 45.5% and 9.1% of the control and case groups, respectively; however, this was not statistically significant. We found significantly lower expression of Bcl2 in the case group (p value: 0.038. Furthermore, expression of CD68 was significantly higher in the case group. Epithelial markers were significantly higher in case subjects, although CD8 expression was higher in the control group. The presence of other markers was not significantly different between the two groups.Conclusions: Increase in apoptosis has a role in aplastic thymuses and prevention of apoptosis may halt this process. Also high CD68

Psoas abscess localization by gallium scan in aplastic anemia

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Oster, M.W.; Gelrud, L.G.; Lotz, M.J.; Herzig, G.P.; Johnston, G.S.

1975-01-01

Gallium 67 scanning is an effective method of detecting inflammatory lesions, especially abscesses. A 10-year-old boy with aplastic anemia and severe leukopenia and granulocytopenia had a psoas abscess diagnosed by gallium scan. The patient died with Candida sepsis 18 days after bone marrow transplantation. At autopsy, a chronic psoas abscess with Candida was found. The gallium scan offers a clinically effective and noninvasive means of evaluating suspected infection in the granulocytopenia patient. (U.S.)

Epidemiologic and HLA Antigen Profile in Patients with Aplastic Anemia

International Nuclear Information System (INIS)

Taj, M.; Shamsi, T. S.; Ansari, S. H.; Farzana, T.; Nazi, A.; Nadeem, M.; Queresi, R. N.; Sheikh, K.; Kazmi, J. H.

2014-01-01

Objective: To analyze patients suffering from aplastic anemia (AA, peripheral pancytopenia and hypocellular bone marrow in the absence of dysplasia, infiltration and fibrosis) for documenting patient's baseline characteristics and association with various human leucocyte antigens. Study Design: An observational, cross-sectional study. Place and Duration of Study: The National Institute of Blood Disease (NIBD), Karachi, from March 2003 to August 2008. Methodology: All consecutive patients with confirmed diagnosis of AA were evaluated. Data included the baseline characteristics, complete blood counts (CBC), bone marrow biopsy findings, severity of disease, exposure to drugs or chemicals, viral serology and their HLA expression. The data was analyzed on SPSS programme and frequencies were documented. Results: Among 318 patients, there were 236 (74.21%) males and 82 (25.78%) females. Median age was 16 and 70% belonged to urban population. Drug exposure could be established in 23 (7.23%) of cases, while 4 (1.25%) were HBV surface antigen positive and 7 (2.2%) were HCV antibodies positive. In all, 73 (22.9%) had very severe AA, 195 (61.32%) had severe AA while 50 (15.7%) cases had non-severe AA. HLA B5 (52) showed high expression in 83 patients (26%) in comparison to 5.9% reported in healthy population. Conclusion: AA was found to affect young adult males living in urban areas. HLA B5 (52) showed higher expression in patients with aplastic anemia. (author)

Pre-existing anti-HLA antibodies negatively impact survival of pediatric aplastic anemia patients undergoing HSCT.

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Zhu, Hua; He, Jun; Cai, Junchao; Yuan, Xiaoni; Jiang, Hua; Luo, Changying; Wang, Jianmin; Luo, Chengjuan; Pan, Zhijuan; Terasaki, Paul I; Ding, Lixia; Chen, Jing

2014-11-01

Graft failure and survival are the major problems for patients with aplastic anemia undergoing hematopoietic stem cell transplantation (HSCT). Previous studies showed that anti-HLA antibodies negatively impact engraftment in HSCT. This retrospective study of 51 pediatric patients with acquired aplastic anemia who underwent allogeneic HSCT at a single institution between 2006 and 2012 investigated the influence of anti-HLA antibodies on the outcome of HSCT. Serum samples collected before HSCT were tested for the presence of anti-HLA antibodies. Pre-existing anti-HLA antibodies were detected in 54.9% (28/51) of patients, among whom 39.2% (20/51) had anti-HLA class I antibodies. Anti-HLA antibodies were associated with worse five-yr survival (78.6% vs. 100%, p = 0.021) and higher treatment-related mortality (21.4% vs. 0%, p = 0.028) compared with antibody-negative patients. Anti-HLA class I antibody-positive patients had poorer five-yr survival (75.0%) than anti-HLA class I&II antibody-positive and antibody-negative patients (87.5% and 100.0%, respectively, p = 0.039). Presence of anti-HLA class I antibodies (p = 0.024) and older age (10 yr or more; p = 0.027) significantly increased the risk of post-HSCT mortality. Pre-existing anti-HLA antibodies negatively affect the outcome of HSCT in pediatric patients with aplastic anemia. Routine testing for anti-HLA antibodies concurrent with efficient treatment should be conducted prior to HSCT. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

[A comparative study of unrelated donor and matched-sibling donor allogeneic hematopoietic stem cell transplantation in children and adolescents with acquired severe aplastic anemia].

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Zhou, J; Fu, Y W; Liang, L J; Wang, Q; Han, L J; Zu, Y L; Zhang, Yanli; Zhu, X H; Yu, F K; Fang, B J; Wei, X D; Song, Y P

2016-12-01

Objective: To evaluate the efficacy of unrelated donor allogeneic hematopoietic stem cell transplantation(URD allo-HSCT) for children and adolescents with severe aplastic anemia (SAA). Methods: Clinical data of 34 SAA children and adolescents undergoing allo-HSCT were retrospectively analyzed from October 2001 to October 2015. According to the source of donor, the patients were divided into matched sibling donor allo-HSCT group (MSD group) and unrelated donor group (URD group). The clinical outcome of SAA children and adolescents receiving URD allo-HSCT was assessed, and patients in MSD allo-HSCT group were enrolled as control at the same period. Results: The rate of hematopoietic reconstitution, the time of neutrophil and platelet engraftment, incidence of chimerism and graft rejection between two groups were not statistically different.The incidence of acute graft-versus-host disease (GVHD) in URD group was significantly higher than that in MSD group [42.9%(6/14) vs 10.5%(2/19), P =0.047]. The incidence of grade Ⅱ-Ⅳ acute GVHD and chronic GVHD in URD were higher than those in MSD group [21.4%(3/14) vs 5.3%(1/19), P =0.288; 35.7%(5/14) vs 5.3%(1/19), P =0.062, respectively], yet without significant difference between two groups. Other transplant-related complications including pulmonary complications, hemorrhagic cystitis, incidence of EBV and CMV reactivation and venous occlusive disease were comparable with two regimens. Estimated 5-years overall survival (OS) rate and disease free survival (DFS) rate were not statistically significant between URD group and MSD group [(84.4±6.6)% vs (89.4±7.1)%, (82.5±5.4)% vs (82.1±4.3)%; P =0.766, P =0.884, respectively]. Conclusions: By multivariate analysis, the outcome of URD allo-HSCT in SAA children and adolescent is similar to MSD allo-HSCT. It could be an alternative option as the first-line treatment for SAA children and adolescents without HLA matched sibling donors.

Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.

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Schrezenmeier, Hubert; Körper, Sixten; Höchsmann, Britta

2015-02-01

Aplastic anemia is a rare life-threatening bone marrow failure that is characterized by bicytopenia or pancytopenia in the peripheral blood and a hypoplastic or aplastic bone marrow. The patients are at risk of infection and hemorrhage due to neutropenia and thrombocytopenia and suffer from symptoms of anemia. The main treatment approaches are allogeneic stem cell transplantation and immunosuppression. Here, we review current standard immunosuppression and the attempts that have been made in the past two decades to improve results: review of recent developments also reveals that sometimes not only the advent of new drugs, good ideas and well-designed clinical trials decide the progress in the field but also marketing considerations of pharmaceutical companies. Aplastic anemia experts unfortunately had to face the situation that efficient drugs were withdrawn simply for marketing considerations. We will discuss the current options and challenges in first-line treatment and management of relapsing and refractory patients with an emphasis on adult patients. Some promising new approaches are currently under investigation in prospective, randomized trials.

Severe Recurrent Achalasia Cardia Responding to Treatment of Severe Autoimmune Acquired Haemophilia

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H. Al-Jafar

2012-09-01

Full Text Available Acquired haemophilia A and severe acquired achalasia are both very rare conditions with unknown aetiology. Haemophilia A is a haemorrhagic disease induced by deficiency or malfunction of coagulation factor VIII. Congenital haemophilia is an inherited disease transmitted by the mother through X-linked inheritance and primarily affects males. However, acquired haemophilia A is a serious, sudden-onset, autoimmune disease that affects either sex. In addition, achalasia is a disease of the oesophagus caused by abnormal function of the nerves and muscles. It causes swallowing difficulties due to the inability of the lower oesophageal sphincter to relax during swallowing, leading to dysphagia, regurgitation and chest pain. In this report, we describe the case of a patient with severe, newly diagnosed, acquired haemophilia A with long-standing, recurrent achalasia; the achalasia had recurred 3 times despite complete and proper surgical fixation. Acquired haemophilia A is treated with immunosuppressive therapy. High-dose steroid therapy was administered for 7 months, during which the patient responded well; moreover, the achalasia did not recur for more than 2 years. The response of the achalasia to immunosuppressive therapy suggests that achalasia may be an autoimmune disorder and that there may be an association between both diseases. The findings of the present case suggest that achalasia may favourably respond to steroid therapy as a first-line treatment prior to surgery.

Incidence of aplastic anemia and agranulocytosis in Latin America: the LATIN study

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Nelson Hamerschlak

Full Text Available CONTEXT AND OBJECTIVE: Aplastic anemia and agranulocytosis are rare but life-threatening disorders, often caused by drugs and other environmental exposures. Reported incidence of these diseases seems to vary between different geographic regions, and few data on their incidence are available for Latin American countries. The aim of this work is to determine the incidence of agranulocytosis and aplastic anemia in Brazil. DESIGN AND SETTING: Incidence study. Seven centers took part in the pilot phase, so as to represent all Brazilian regions. METHODS: Each center conducted an active search for new cases in a defined region by means of regular contacts with all hematologists, main clinical laboratories and clinicians in hospitals of the region. RESULTS: 74 patients with aplastic anemia and 16 with agranulocytosis were identified. Patients with agranulocytosis had a median age of 31 years (interquartile range, IQR: 12.5-48.2; 32.2% were male and 81.2% were white. The median age of aplastic anemia patients was 21 years (IQR 15.0-35.2; 62.2% were male, 50.0% were white and 39.2% mulatto. The incidence of agranulocytosis was estimated to be 0.5 cases per million individuals per year, ranging from 0.0 to 1.1 cases per million per year between regions. The incidence of aplastic anemia was 2.7 cases per million per year, ranging from 1.1 to 7.1 cases per million per year between regions. CONCLUSIONS: Aplastic anemia and agranulocytosis are rare diseases in Brazil. However, there is considerable variability in their incidences between different regions.

Nutritional status survey of aplastic anemia patients--a single center experience in China.

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Li, Xinli; Feng, Yanyan; Wang, Hongyan; Song, Meijuan; Jin, Jingjing; Cui, Zhenzhu; Zheng, Yizhou

2016-05-01

To analyze the nutritional status of aplastic anemia (AA) patients. The nutrition-related anthropometric indicators and blood biochemical index of 622 newly-diagnosed AA patients were retrospectively analyzed. Of the cohort of AA patients, body mass index of non-severe AA (NSAA) patients were higher than those of severe AA (SAA) (phemoglobin was correlated with lower serum albumin protein concentration (peducation for patients. Copyright © 2015 Elsevier Inc. All rights reserved.

Management of aplastic anemia in a woman during pregnancy: A case report

NARCIS (Netherlands)

K.J.M. Stibbe (Krista J. M.); H.I.J. Wildschut (Hajo); P.J. Lugtenburg

2011-01-01

textabstractIntroduction. Aplastic anemia is a rare disease caused by destruction of pluripotent stem cells in bone marrow. During pregnancy it could be life-threatening for both mother and child. The only causal therapy for aplastic anemia is bone marrow transplantation, which is contraindicated

Parvovirus B19 infection presenting with severe erythroid aplastic crisis during pregnancy in a woman with autoimmune hemolytic anemia and alpha-thalassemia trait: a case report.

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Chen, Chi-Ching; Chen, Chin-Shan; Wang, Wei-Yao; Ma, Jui-Shan; Shu, Hwei-Fan; Fan, Frank S

2015-03-12

Parvovirus B19 virus commonly causes subclinical infection, but it can prove fatal to the fetus during pregnancy and cause severe anemia in an adult with hemolytic diseases. We present the case of a woman with autoimmune hemolytic anemia who was diagnosed with parvovirus B19-induced transient aplastic crisis during her second trimester of pregnancy and faced the high risk of both fetal and maternal complications related to this specific viral infection. To the best of our knowledge, the experience of successful intravenous immunoglobulin treatment for B19 virus infection during pregnancy, as in our case, is limited. A 28-year-old and 20-week pregnant Chinese woman with genetically confirmed alpha-thalassemia trait was diagnosed with cold antibody autoimmune hemolytic anemia and suffered from transient aplastic crisis caused by B19 virus infection. She received intravenous immunoglobulin treatment to reduce the risk of hydrops fetalis. Her peripheral blood reticulocyte percentage recovered, but anemia persisted, so she underwent several courses of high dose intravenous dexamethasone for controlling her underlying hemolytic problem. Finally, her hemoglobin levels remained stable with no need of erythrocyte transfusion, and a healthy baby boy was naturally delivered. Parvovirus B19 virus infection should be considered when a sudden exacerbation of anemia occurs in a patient with hemolytic disease, and the possible fetal complications caused by maternal B19 virus infection during pregnancy should not be ignored. Close monitoring and adequate management can keep both mother and fetus safe.

Proton MR spectroscopy of hyperplastic hematopoietic marrow in aplastic anemia

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Amano, Yasuo; Kumazaki, Tatsuo [Nippon Medical School, Tokyo (Japan); Arai, Nobuyuki

1997-04-01

The purpose of this study was to compare the findings of magnetic resonance (MR) spectroscopy of hyperplastic hematopoietic marrow with those of normal bone marrow. Twenty-four samples of normal marrow from eight control subjects and 19 samples of hyperplastic marrow in aplastic anemia were examined with a 1.5 T MR unit. The former showed low intensity on opposed-phase T1-weighted images, while the latter showed high intensity on both fast STIR and opposed-phase T1-weighted images. MR spectroscopy quantitatively confirmed that the water; fat ratio was increased and the transverse relaxation time of water was changed in hyperplastic bone marrow, compared with normal bone marrow. In summary, MR imaging is able to detect hematopoietic regions among a wide range of bone marrow of aplastic anemia, while MR spectroscopy allowed us to quantitatively analyze the cell population of hyperplastic hematopoietic marrow in aplastic anemia. (author)

Proton MR spectroscopy of hyperplastic hematopoietic marrow in aplastic anemia

International Nuclear Information System (INIS)

Amano, Yasuo; Kumazaki, Tatsuo; Arai, Nobuyuki.

1997-01-01

The purpose of this study was to compare the findings of magnetic resonance (MR) spectroscopy of hyperplastic hematopoietic marrow with those of normal bone marrow. Twenty-four samples of normal marrow from eight control subjects and 19 samples of hyperplastic marrow in aplastic anemia were examined with a 1.5 T MR unit. The former showed low intensity on opposed-phase T1-weighted images, while the latter showed high intensity on both fast STIR and opposed-phase T1-weighted images. MR spectroscopy quantitatively confirmed that the water; fat ratio was increased and the transverse relaxation time of water was changed in hyperplastic bone marrow, compared with normal bone marrow. In summary, MR imaging is able to detect hematopoietic regions among a wide range of bone marrow of aplastic anemia, while MR spectroscopy allowed us to quantitatively analyze the cell population of hyperplastic hematopoietic marrow in aplastic anemia. (author)

Prognostic value of lactate clearance in severe community acquired pneumonia

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Mohamed, Kamel Abd Elaziz; Ahmed, Dief Abd Elgalil

2014-01-01

Introduction: Severe community acquired pneumonia (SCAP) occurs in approximately 18–36% of all CAP and the mortality rate could be as high as 67% in patients with SCAP. Several studies have described a correlation between baseline lactate concentration and mortality of ICU patients. Aim of the work: To follow lactate clearance after admission for 24 h which could be an indicator of outcome in severe community acquired pneumonia. Patients and methods: Forty-six consecutively admitted adu...

Bone marrow imaging with MR and indium-111-chloride scintigraphy in patients with myelodysplasia and aplastic anemia

International Nuclear Information System (INIS)

Kumar, R.; Juneja, H.S.; Sayle, B.A.; Johnson, R.F.

1989-01-01

This paper reports twenty-one patients with myelodysplasia and aplastic anemia studied with MR and bone marrow radionuclide imaging to determine the roles of these imaging modalities, with seven normal patients serving as controls. All patients underwent In-111 chloride bone marrow scintigraphy prior to MR imaging. MR studies of the lumbar spine were acquired at 0.6 T with a T1-weighted (500/24 [repetition time (TR) msec/echo time (TE) msec]) spin-echo technique and an inversion-recovery technique (200/26/148 [TR msec/TE msec/inversion time msec])

Aplastic anemia as a feature of systemic lupus erythematosus: a case report and literature review.

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Chalayer, Émilie; Ffrench, Martine; Cathébras, Pascal

2015-06-01

Peripheral cytopenias are common in systemic lupus erythematosus, but bone marrow involvement is rarely reported. Aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and characterized by an empty bone marrow. This rare but serious disease has been described as an unusual manifestation of systemic lupus erythematosus. We reviewed the 25 cases published in the English language literature and discuss the clinical presentation, outcome, treatment, and pathophysiology of aplastic anemia as a complication of systemic lupus erythematosus. We report here the first case of aplastic anemia associated with systemic lupus erythematosus treated with an allogeneic hematopoietic stem cell transplant. Over one half of patients received concomitantly the diagnoses of systemic lupus erythematosus and aplastic anemia. No clinical or histological features can distinguish primary aplastic anemia from aplastic anemia occurring in systemic lupus erythematosus patients. The overall mortality is about 15% and corticosteroid-based therapy alone or in combination with other immunomodulatory drugs can restore bone marrow function. Systemic lupus erythematosus may be complicated by bone marrow involvement. The diagnosis of peripheral cytopenias should be confirmed by bone marrow aspiration. All these patients should receive cortisone as a first treatment. Plasma exchanges seem to have some efficacy. Other different immunomodulatory therapies were used with variable results.

Тhe features of severe community acquired pneumonia

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Avramenko I.V.

2015-06-01

Full Text Available Based on data from a prospective analysis for the year of observation, the article presents information about the features of severe community acquired pneumonia in patients who were hospitalized at the department of pulmonology (or therapy, as well as department of the intensive care from three teaching hospitals in Dnepropetrovsk, namely "Dnipropetrovsk City Hospital №6», "Dnipropetrovsk City Hospital №2», "Dnipropetrovsk City Hospital №16», which are the clinical ones of "Dnepropetrovsk Medical Academy of the Ministry of Health Ukraine". Dependence of the severity of the condition shown on duration of illness before admission, features of season character of disease. The effect of breathing exercises on the course of the disease. The results can be the basis for a more personal approach to the development of diagnostic and therapeutic programs for patients with severe community-acquired pneumonia.

Subtotal body irradiation with linear accelerator as preparation for marrow engraftment in aplastic anemia

International Nuclear Information System (INIS)

Walbom-Joergensen, S.; Ernst, P.

1979-01-01

Two cases of multitransfused severe aplastic anemia were retransplanted with bone marrow from the same HLA compatible sibling donors after subtotal body irradiation (800 r). Only minor non hematologic toxicity was observed. No permanent take was seen in relation to this procedure. During the survival time of the patients (78-120 days) no signs of interstitial pneumonia were observed

Management of aplastic anaemia in pregnancy in a resource poor centre.

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John, Celestine Osita; Korubo, Kaladada; Ogu, Rosemary; Mmom, Chigozirim Faith; Mba, Alpheus Gogo; Chidiadi, Ezenwa-Ahanene; Akani, Chris

2016-01-01

Aplastic anaemia occurring in pregnancy is a rare event with life threatening challenges for both mother and child. We present a successful fetomaternal outcome despite the challenges in the management of this rare condition in a tertiary but resource poor centre. This is case of a 37 year old Nigerian woman G6P0 +5 managed with repeated blood transfusions from 28 weeks of gestation for bone marrow biopsy confirmed aplastic anaemia following presentation with weakness and gingival bleeds. She had a cesarean section at 37 weeks for pre-eclampsia and oligohydraminous with good feto-maternal outcome. She was managed entirely with fresh whole blood and received 21 units. Aplastic Anaemia in Pregnancy is a rare event with poor feto maternal prognosis. Successful management is possible with good multi-disciplinary approach and availability of supportive comprehensive obstetric care.

Predictors of early mortality after rabbit antithymocyte globulin as first-line treatment in severe aplastic anemia.

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Atta, Elias H; Lima, Carlos B L; Dias, Danielle S P; Clé, Diego V; Bonduel, Mariana M; Sciuccati, Gabriela B; Medeiros, Larissa A; Oliveira, Michel M; Salvino, Marco A; Garanito, Marlene P; Blum Fonseca, Patricia B; Saad, Sara Teresinha O; Calado, Rodrigo T; Scheinberg, Phillip

2017-11-01

Despite being recommended as first-line immunosuppressive therapy in severe aplastic anemia (SAA), horse antithymocyte globulin (ATG) is still unavailable in many countries outside the USA. Rabbit ATG is more lymphocytoxic than horse ATG, and this might result in a higher incidence of severe infections and early mortality. This study was designed to identify the risk factors for early mortality and overall survival (OS) after rabbit ATG in patients with SAA. We retrospectively reviewed 185 patients with SAA who underwent rabbit ATG and cyclosporine. The incidence of death in 3 months following rabbit ATG therapy was 15.1% (28/185). Early mortality was mainly related to infectious complications, despite adequate antibiotic and/or antifungal treatment. Age > 35 years (odds ratio [OR] 5.06, P = 0.001) and baseline absolute neutrophil count (ANC) ≤ 0.1 × 10 9 /L (OR 7.64, P  35 years (OR 1.88, P = 0.03), baseline ANC ≤ 0.1 × 10 9 /L (OR 2.65, P < 0.001), and lack of response to rabbit ATG (OR 11.40, P < 0.001) were independently associated with mortality. Alternative strategies are needed for the treatment of SAA patients in countries were horse ATG is unavailable, particularly for those at high risk for early mortality after rabbit ATG due to a higher age and very low pre-treatment neutrophil count.

Myelodysplastic syndrome evolving from aplastic anemia treated with immunosuppressive therapy: efficacy of hematopoietic stem cell transplantation.

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Kim, Sung-Yong; Le Rademacher, Jennifer; Antin, Joseph H; Anderlini, Paolo; Ayas, Mouhab; Battiwalla, Minoo; Carreras, Jeanette; Kurtzberg, Joanne; Nakamura, Ryotaro; Eapen, Mary; Deeg, H Joachim

2014-12-01

A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoietic stem cell transplantation, and in a matched-pair analysis compared outcome to that in 393 patients with de novo myelodysplastic syndrome. There was no difference in overall survival. There were no significant differences with regard to 5-year probabilities of relapse, non-relapse mortality, relapse-free survival and overall survival; these were 14%, 40%, 46% and 49% for post-aplastic anemia myelodysplastic syndrome, and 20%, 33%, 47% and 49% for de novo myelodysplastic syndrome, respectively. In multivariate analysis, relapse (hazard ratio 0.71; P=0.18), non-relapse mortality (hazard ratio 1.28; P=0.18), relapse-free survival (hazard ratio 0.97; P=0.80) and overall survival (hazard ratio 1.02; P=0.88) of post-aplastic anemia myelodysplastic syndrome were similar to those of patients with de novo myelodysplastic syndrome. Cytogenetic risk was independently associated with overall survival in both groups. Thus, transplant success in patients with post-aplastic anemia myelodysplastic syndrome was similar to that in patients with de novo myelodysplastic syndrome, and cytogenetics was the only significant prognostic factor for post-aplastic anemia myelodysplastic syndrome patients. Copyright© Ferrata Storti Foundation.

Recent advances in understanding clonal haematopoiesis in aplastic anaemia.

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Stanley, Natasha; Olson, Timothy S; Babushok, Daria V

2017-05-01

Acquired aplastic anaemia (AA) is an immune-mediated bone marrow failure disorder inextricably linked to clonal haematopoiesis. The majority of AA patients have somatic mutations and/or structural chromosomal abnormalities detected as early as at diagnosis. In contrast to other conditions linked to clonal haematopoiesis, the clonal signature of AA reflects its immune pathophysiology. The most common alterations are clonal expansions of cells lacking glycophosphotidylinositol-anchored proteins, loss of human leucocyte antigen alleles, and mutations in BCOR/BCORL1, ASXL1 and DNMT3A. Here, we present the current knowledge of clonal haematopoiesis in AA as it relates to aging, inherited bone marrow failure, and the grey-zone overlap of AA and myelodysplastic syndrome (MDS). We conclude by discussing the significance of clonal haematopoiesis both for improved diagnosis of AA, as well as for a more precise, personalized approach to prognostication of outcomes and therapy choices. © 2017 John Wiley & Sons Ltd.

Congenital aplastic anemia caused by mutations in the SBDS gene: A rare presentation of Shwachman-Diamond syndrome

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Kuijpers, Taco W.; Nannenberg, Eline; Alders, Marielle; Bredius, Robbert; Hennekam, Raoul C. M.

2004-01-01

Clinical Findings. Aplastic anemia was diagnosed at birth for a first child from healthy nonconsanguineous parents. The girl had hypoglycemia, which normalized within 2 months. Cow milk allergy was suspected initially, because of skin lesions and diarrhea, followed by severe growth retardation.

Aplastic crisis in occult hereditary spherocytosis caused by human parvovirus (HPV B19).

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Rappaport, E S; Quick, G; Ransom, D; Helbert, B; Frankel, L S

1989-02-01

We have reported a case of aplastic crisis occurring in an 11-year-old black boy with occult hereditary spherocytosis. An etiologic diagnosis of human parvovirus (HPV) B19 infection was confirmed serologically. The Coulter Model S + IV proved useful for both diagnosis and treatment monitoring through serial histograms. The relationship of HPV infection and aplastic crisis is discussed.

Peripheral blood lymphocyte telomere length as a predictor of response to immunosuppressive therapy in childhood aplastic anemia

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Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Hama, Asahito; Kawashima, Nozomu; Wang, Xinan; Narita, Atsushi; Doisaki, Sayoko; Xu, Yinyan; Muramatsu, Hideki; Yoshida, Nao; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Nakamura, Kazuhiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

2014-01-01

Predicting the response to immunosuppressive therapy could provide useful information to help the clinician define treatment strategies for patients with aplastic anemia. In our current study, we evaluated the relationship between telomere length of lymphocytes at diagnosis and the response to immunosuppressive therapy in 64 children with aplastic anemia, using flow fluorescence in situ hybridization. Median age of patients was ten years (range 1.5–16.2 years). Severity of the disease was classified as very severe in 23, severe in 21, and moderate in 20 patients. All patients were enrolled in multicenter studies using antithymocyte globulin and cyclosporine. The response rate to immunosuppressive therapy at six months was 52% (33 of 64). The probability of 5-year failure-free survival and overall survival were 56% (95% confidence interval (CI): 41–69%) and 97% (95%CI: 87–99%), respectively. Median telomere length in responders was −0.4 standard deviation (SD) (−2.7 to +3.0 SD) and −1.5 SD (−4.0 to +1.6 (SD)) in non-responders (Paplastic anemia. PMID:24816243

Aplastic anemia during pregnancy: a review of obstetric and anesthetic considerations

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Riveros-Perez, Efrain; Hermesch, Amy C; Barbour, Linda A; Hawkins, Joy L

2018-01-01

Aplastic anemia is a hematologic condition occasionally presenting during pregnancy. This pathological process is associated with significant maternal and neonatal morbidity and mortality. Obstetric and anesthetic management is challenging, and treatment requires a coordinated effort by an interdisciplinary team, in order to provide safe care to these patients. In this review, we describe the current state of the literature as it applies to the complexity of aplastic anemia in pregnancy, focusing on pathophysiologic aspects of the disease in pregnancy, as well as relevant obstetric and anesthetic considerations necessary to treat this challenging problem. A multidisciplinary-team approach to the management of aplastic anemia in pregnancy is necessary to coordinate prenatal care, optimize maternofetal outcomes, and plan peripartum interventions. Conservative transfusion management is critical to prevent alloimmunization. Although a safe threshold-platelet count for neuraxial anesthesia has not been established, selection of anesthetic technique must be evaluated on a case-to-case basis. PMID:29535558

Preceding immunosuppressive therapy with antithymocyte globulin and ciclosporin increases the incidence of graft rejection in children with aplastic anaemia who underwent allogeneic bone marrow transplantation from HLA-identical siblings.

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Kobayashi, Ryoji; Yabe, Hiromasa; Hara, Junichi; Morimoto, Akira; Tsuchida, Masahiro; Mugishima, Hideo; Ohara, Akira; Tsukimoto, Ichiro; Kato, Koji; Kigasawa, Hisato; Tabuchi, Ken; Nakahata, Tatsutoshi; Ohga, Shoichi; Kojima, Seiji

2006-12-01

The incidence of graft rejection was determined in 66 children with acquired aplastic anaemia (AA) following bone marrow transplantation (BMT) from a related donor. Eleven of 65 evaluable patients experienced either early or late rejection. Multivariate analysis identified previous immunosuppressive therapy with antithymocyte-globulin (ATG) and ciclosporin (CsA) as a risk factor for graft rejection (relative risk: 16.6, P = 0.001). Patients who received ATG and CsA had a significantly lower probability of failure-free survival than those who did not (69.7 +/- 6.2% vs. 87.9 +/- 8.0%, P = 0.044). These results suggest that BMT should be instituted immediately in children with severe AA who have human leucocyte antigen-identical siblings.

Successful treatment of pulmonary mucormycosis in a child with aplastic anemia

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Ye. A. Nikitina

2014-07-01

Full Text Available Mucormycosis (zygomycosis is a frequently fatal fungal infection in immunocompromized patients. We describe a case of a successfull treatment of pulmonary mucormycosis in 11-year-child with a very severe aplastic anemia. The diagnosis of invasive mycosis has been proved according to EORTC 2008 criteria. Computed tomography showed bilobar right-sided pulmonary infiltration. Lichtheimia corymbifera cultured from BAL. The child achieved complete clinical, laboratory and instrumental response on long-term amphotericin B lipid-formulated therapy combined with posaconazole.

Total lymphoid irradiation based conditioning for hematopoietic stem cell transplantation in severe aplastic anemia

International Nuclear Information System (INIS)

Lee, Yun Hee; Kim, Ji Yoon; Choi, Byung Ock; Ryu, Mi Ryeong; Chung, Su Mi

2012-01-01

To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

Total lymphoid irradiation based conditioning for hematopoietic stem cell transplantation in severe aplastic anemia

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Lee, Yun Hee; Kim, Ji Yoon; Choi, Byung Ock; Ryu, Mi Ryeong; Chung, Su Mi [Dept. of Radiation Oncology, The Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

2012-12-15

To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

Multiparameter FLAER-based flow cytometry for screening of paroxysmal nocturnal hemoglobinuria enhances detection rates in patients with aplastic anemia.

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Sachdeva, Man Updesh Singh; Varma, Neelam; Chandra, Dinesh; Bose, Parveen; Malhotra, Pankaj; Varma, Subhash

2015-05-01

Flow cytometry is the gold standard methodology for screening of paroxysmal nocturnal hemoglobinuria. In the last few years, proaerolysin conjugated with fluorescein (FLAER) has become an important component of antibody panel used for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study aimed to compare PNH clone detection by flow cytometry in the pre-FLAER era versus the FLAER era. This was a retrospective analysis of 4 years and included 1004 individuals screened for PNH clone, either presenting as hemolytic anemia or as aplastic anemia. In the pre-FLAER time period, the RBCs and neutrophils were screened with antibodies against CD55 and CD59. With the introduction of FLAER, neutrophils were screened with FLAER/CD24/CD15 and monocytes with FLAER/CD14/CD33 combination. A comparative analysis was done for detection of PNH clone in aplastic anemia patients versus non-aplastic anemia patients, as well as between pre-FLAER and FLAER era. Out of a total of 1004 individuals, 59 (5.8%) were detected to have PNH clone positivity. The frequency of PNH clone detected in aplastic anemia and non-aplastic anemia groups was 12.02 and 3.36%, respectively. The detection rate of PNH clone increased from 4.5% (32/711) in the pre-FLAER era to 9.2% (27/293) with the introduction of FLAER. However, this increase could be attributed to increased detection of PNH clone in the aplastic anemia group, which showed a significant increase from 8.3 to 18.2% after use of FLAER. In the non-aplastic group, PNH clone was detected with similar frequencies before and after use of FLAER (3.2 versus 3.8%, respectively). Mean PNH clone size was lower in the aplastic anemia group when compared with the non-aplastic group. RBCs always showed a lower clone size than neutrophils. PNH clone on neutrophils and monocytes was however similar. Inclusion of FLAER increases the sensitivity of the test which is especially useful in picking up small PNH clones in patients of aplastic anemia.

The Role of Histone Demethylase Jmjd3 in Immune-Mediated Aplastic Anemia

Science.gov (United States)

2017-03-01

AWARD NUMBER: W81XWH-16-1-0055 TITLE: The Role of Histone Demethylase Jmjd3 in Immune-Mediated Aplastic Anemia PRINCIPAL INVESTIGATOR: Yi...Immune-Mediated Aplastic Anemia 5a. CONTRACT NUMBER 5b. GRANT NUMBER W81XWH-16-1-0055 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) Yi Zhang 5d... anemia (AA) is a condition of bone marrow failure (BMF) characterized by blood pancytopenia and BM hypoplasia. In most cases, AA is an immune-mediated

Therapeutic effect of androgen therapy in a mouse model of aplastic anemia produced by short telomeres.

Science.gov (United States)

Bär, Christian; Huber, Nicolas; Beier, Fabian; Blasco, Maria A

2015-10-01

Aplastic anemia is a rare but life-threatening disorder characterized by cytopenia in at least two of the three blood lineages. A frequent feature of patients with aplastic anemia is that they have shorter telomeres than those of age-matched controls. Testosterone has been used for over half a century in the treatment of aplastic anemia. However, although remissions are frequent following hormone therapy, the molecular mechanism underlying the response to treatment has remained unknown. Here we explored the possibility that the recently described regulation of telomerase activity by sex hormones may be the mechanism responsible. To this end, we used a mouse model of aplastic anemia induced by short telomeres in the bone marrow compartment. We found that testosterone therapy results in telomerase up-regulation, improved blood counts, and a significant extension of life-span of these mice. Importantly, longitudinal follow-up studies revealed longer telomeres in peripheral blood in mice subjected to hormone treatment. Our results demonstrate that testosterone-mediated telomerase activation can attenuate or reverse aplastic anemia disease progression associated with the presence of short telomeres. Copyright© Ferrata Storti Foundation.

Bone marrow transplantation in aplastic anaemia using cyclophosphamide and total lymphoid irradiation

International Nuclear Information System (INIS)

Jansen, J.; Zwaan, F.E.; Noordijk, E.M.

1980-01-01

Six patients with severe aplastic anaemia received a bone-marrow graft after conditioning with cyclophosphamide and total lymphoid irradiation (TLI). No rejections occurred. Acute graft-versus-host disease developed in 3 patients and was fatal in one. Another patient died from systemic aspergillus infection. Chronic GVHD of the skin developed in a patient who was grafted with bone marrow from her HLA-phenotypically identical father. These data suggest that conditioning with cyclophosphamide and TLI is a promising regimen. (orig.) [de

Total lymph-node irradiation and pretreatment with cyclophosphamide in preparation for bone-marrow grafting for aplastic anaemia

International Nuclear Information System (INIS)

Jansen, J.; Zwaan, F.E.; Noordijk, E.M.

1981-01-01

Bone-marrow transplantation (BMT), using bone marrow from an HLA-identical brother or sister constitutes the treatment of choice in most young patients with severe aplastic amaemia. The cases are described of 6 patients who were prepared for grafting by administration of cyclophosphamide (4 days, 50 mg/kg body weight day) and total lymph-node irradiation (750 rad in a single dose). One patient died on the 26th day after BMT from a disseminated Aspergillus infection, and another on the 28th day from the consequences of graft-versus-host disease (GVH disease). One patient recovered after an episode of GVH disease. Two patients developed no complications after the grafting. One female patient, who for the lack of an HLA-identical brother or sister had been grafted with bone marrow of her father whose HLA-phenotype was identical, was normalized haematologically but developed chronic GVH disease of the skin. This method of preparation for BMT for aplastic anaemia reduces the risk of rejection of the bone marrow to a minimum, and may well reduce the frequency and severity of GVH disease. (Auth.)

Hereditary Spherocytosis Unmasked by Human Parvovirus B19 Induced Aplastic Crisis in a Family

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Samin Alavi

2015-09-01

Full Text Available Human parvovirus (HPV B19 induced aplastic crisis in a family leading to the diagnosis of hereditary spherocytosis (HS is a very rare condition being barely reported in the literature. We herein report a 4-year-old girl, her brother, and their mother who all presented with progressive pallor and jaundice after a febrile illness. The HPV B19 was diagnosed using polymerase chain reaction (PCR and positive serology for specific anti-HPV B19 IgM. They were further diagnosed with having HS. The clinical importance of this report is that in the case of an abrupt onset of unexplained severe anemia and jaundice, one should consider underlying hemolytic anemias mostly hereditary spherocytosis complicated by HPV B19 aplastic crisis. Herein, we report the occurrence of this condition, simultaneously in three members of a family. The distinguished feature of this report is that all affected family members developed some degrees of transient pancytopenia, not only anemia, all simultaneously in the course of their disease.

Identification of high-risk patients with aplastic anaemia in selection for allogeneic 0one-marrow transplantation.

Science.gov (United States)

Lohrmann, H P; Kern, P; Niethammer, D; Heimpel, H

1976-09-25

Of 75 patients with asplastic anaemia treated between 1968 and 1975, 33 were retrospectively considered as potential candidates for allogenegic bone-marrow transplantation on the basis of their age and severity of marrow failure. The prognosis of these patients with conservative treatment was assessed from parameters obtained at the time of the initial diagnosis. Initial peripheral-blood granulocyte or platelet concentrations were not of porgnostic value. In contrast, initial reticulocyte concentrations, allowed separation of the patients into two groups with poor and good prognosis. Low initial reticulocyte concentrations (less than 10 000/mu1) indicated those patients at extremely high risk of succumbing to their marrow aplasia (there were no survivors 36 months after disgnosis). In contrast, 75% of those patients with more than 10 000 reticulocytes per mu1 at diagnosis survived for 3 years. Initial peripheral-blood reticulocyte concentrations thus appear to indicate the extent of the marrow failure in aplastic anaemia more accurately than granulocytes or platelets. Low initial reticulocyte concentrations may indicate, among patients with severe aplastic anaemia, those for whom allogeneic bone-marrow transplantation should be seriously considered; patients with higher initial reticulocyte concentrations may benefit from conservation treatment.

Changing patterns of radiosensitivity of hematopoietic progenitors from chronically irradiated dogs prone either to aplastic anemia or to myeloproliferative disease

International Nuclear Information System (INIS)

Seed, T.M.; Kaspar, L.V.

1990-01-01

Hematopoietic patterns have been assessed in chronic 60 Co gamma irradiated dogs during preclinical phases of evolving aplastic anemia (AA) or myeloproliferative disease (MPD), principally myeloid leukemia. The results support the concept that acquired radioresistance of vital granulocyte/monocyte lineage-committed hematopoietic progenitors is temporally, perhaps causally, linked to the processes mediating hematopoietic recovery and accommodation under chronic irradiation, and in turn to preclinical events of evolving MPD. In addition, the marked differential responses of progenitors to gamma and neutron irradiation in vitro might suggest differences in the nature of cellular lesions elicited by chronic gamma irradiation, in vivo. (author)

Changing patterns of radiosensitivity of hematopoietic progenitors from chronically irradiated dogs prone either to aplastic anemia or to myeloproliferative disease

Energy Technology Data Exchange (ETDEWEB)

Seed, T.M.; Kaspar, L.V. (Oak Ridge National Lab., TN (USA))

1990-01-01

Hematopoietic patterns have been assessed in chronic {sup 60}Co gamma irradiated dogs during preclinical phases of evolving aplastic anemia (AA) or myeloproliferative disease (MPD), principally myeloid leukemia. The results support the concept that acquired radioresistance of vital granulocyte/monocyte lineage-committed hematopoietic progenitors is temporally, perhaps causally, linked to the processes mediating hematopoietic recovery and accommodation under chronic irradiation, and in turn to preclinical events of evolving MPD. In addition, the marked differential responses of progenitors to gamma and neutron irradiation in vitro might suggest differences in the nature of cellular lesions elicited by chronic gamma irradiation, in vivo. (author).

Intense muscle uptake of Ga-67 citrate and Tc-99m MDP in a patient with aplastic anemia

International Nuclear Information System (INIS)

Batte, W.G. Jr.; Yeh, S.D.; Rosenblum, M.K.; Larson, S.M.

1991-01-01

An unusual distribution of Tc-99m MDP and Ga-67 citrate in several muscle groups is described in a 14-year-old boy with aplastic anemia and normal renal function at the time of imaging. Although the patient did not develop musculo-skeletal signs or symptoms, a subsequent autopsy revealed widespread muscle calcification

Prognostic value of severity indicators of nursing-home-acquired pneumonia versus community-acquired pneumonia in elderly patients

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Ugajin M

2014-02-01

Full Text Available Motoi Ugajin, Kenichi Yamaki, Natsuko Hirasawa, Takanori Kobayashi, Takeo Yagi Department of Respiratory Medicine, Ichinomiya-Nishi Hospital, Ichinomiya City, Japan Background: The credibility of prognostic indicators in nursing-home-acquired pneumonia (NHAP is not clear. We previously reported a simple prognostic indicator in community-acquired pneumonia (CAP: blood urea nitrogen to serum albumin (B/A ratio. This retrospective study investigated the prognostic value of severity indicators in NHAP versus CAP in elderly patients. Methods: Patients aged ≥65 years and hospitalized because of NHAP or CAP within the previous 3 years were enrolled. Demographics, coexisting illnesses, laboratory and microbiological findings, and severity scores (confusion, urea, respiratory rate, blood pressure, and age ≥65 [CURB-65] scale; age, dehydration, respiratory failure, orientation disturbance, and pressure [A-DROP] scale; and pneumonia severity index [PSI] were retrieved from medical records. The primary outcome was mortality within 28 days of admission. Results: In total, 138 NHAP and 307 CAP patients were enrolled. Mortality was higher in NHAP (18.1% than in CAP (4.6% (P<0.001. Patients with NHAP were older and had lower functional status and a higher rate of do-not-resuscitate orders, heart failure, and cerebrovascular diseases. The NHAP patients more frequently had typical bacterial pathogens. Using the receiver-operating characteristics curve for predicting mortality, the area under the curve in NHAP was 0.70 for the A-DROP scale, 0.69 for the CURB-65 scale, 0.67 for the PSI class, and 0.65 for the B/A ratio. The area under the curve in CAP was 0.73 for the A-DROP scale, 0.76 for the CURB-65 scale, 0.81 for the PSI class, and 0.83 for the B/A ratio. Conclusion: Patient mortality was greater in NHAP than in CAP. Patient characteristics, coexisting illnesses, and detected pathogens differed greatly between NHAP and CAP. The existing severity indicators

Diagnostic and management dilemma of a pancreas-kidney transplant recipient with aplastic anaemia.

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Viecelli, Andrea; Hessamodini, Hannah; Augustson, Bradley; Lim, Wai Hon

2014-09-25

We report a case of a 57-year-old woman with type I diabetes who had received a simultaneous pancreas-kidney (SPK) transplant maintained on tacrolimus, mycophenolic acid (MPA) and prednisolone. Her renal allograft failed 6 years post-transplant but she continued to have a normal functioning pancreatic allograft. Over the course of 5 years, she developed progressive bone marrow failure with repeat bone marrow aspirates demonstrating an evolution from erythroid hypoplasia to hypocellular marrow and eventual aplastic anaemia despite discontinuation of MPA and reduction of tacrolimus. She was transfusion-dependent and had frequent admissions for sepsis. Despite treatment with antithymocyte globulin and cyclosporine for aplastic anaemia, she developed fatal invasive pulmonary aspergillosis within 3 weeks of treatment. Even though the cause of aplastic anaemia is likely multifactorial, this case highlights the difficulty in balancing the need for versus the risk of ongoing immunosuppression in a SPK transplant recipient who continues to have normal pancreatic graft function. 2014 BMJ Publishing Group Ltd.

Development of Medical Technology for Contingency Response to Marrow Toxic Agents

Science.gov (United States)

2012-07-26

resulting from military or terrorist actions, as well as patients suffering from leukemia, aplastic anemia and other life-threatening diseases...Contamination • Diagnosis & Management of Acute Local Radiation Injury & Case Review: Yanango Peru • Radiation Sources & Radiological Terrorism • Radiation...Hematopoietic Stem Cell Transplant in Patients with Acquired Severe Aplastic Anemia S. Gadalla 650 samples Telomere length and Telomerase

Pathophysiology and management of thrombocytopenia in bone marrow failure: possible clinical applications of TPO receptor agonists in aplastic anemia and myelodysplastic syndromes

Science.gov (United States)

Desmond, Ronan; Dunbar, Cynthia E.; Young, Neal S.

2014-01-01

Aplastic anemia is a bone marrow failure syndrome that causes pancytopenia and can lead to life-threatening complications. Bone marrow transplantation remains the standard of care for younger patients and those with a good performance status but many patients may not have a suitable donor. Immunosuppressive therapy is able to resolve cytopenias in a majority of patients with aplastic anemia but relapses are not uncommon and some patients remain refractory to this approach. Patients may require frequent blood and platelet transfusion support which is expensive and inconvenient. Life-threatening bleeding complications still occur despite prophylactic platelet transfusion. Thrombopoietin (TPO) mimetics, such as romiplostim and eltrombopag, were developed to treat patients with refractory immune thrombocytopenia but are now being investigated for the treatment of bone marrow failure syndromes. TPO is the main regulator for platelet production and its receptor (c-Mpl) is present on megakaryocytes and hematopoietic stem cells. Trilineage hematopoietic responses were observed in a recent clinical trial using eltrombopag in patients with severe aplastic anemia refractory to immunosuppression suggesting that these agents can provide a new therapeutic option for enhancing blood production. In this review, we discuss these recent results and ongoing investigation of TPO mimetics for aplastic anemia and other bone marrow failure states like myelodysplastic syndromes. Clonal evolution or progression to acute myeloid leukemia remains a concern when using these drugs in bone marrow failure and patients should only be treated in the setting of a clinical trial. PMID:23690288

Aplastic Anemia Secondary to Methimazole: Case Report and Review

International Nuclear Information System (INIS)

Hong, Kee Suk; Lee, Gwon Jun; Kim, Byoung Kook; Koh, Chang Soon; Lee, Mun Ho

1977-01-01

A case is reported of aplastic anemia occurring during Methimazole therapy. Since this seems to be a kind of self-limiting disease having considerably rapid recovery, much more detailed supportive therapy is considered to be crucial rather than any other corticosteroid or the androgen therapy.

Aplastic Anemia Secondary to Methimazole: Case Report and Review

Energy Technology Data Exchange (ETDEWEB)

Hong, Kee Suk; Lee, Gwon Jun; Kim, Byoung Kook; Koh, Chang Soon; Lee, Mun Ho [Seoul National University College of Medicine, Seoul (Korea, Republic of)

1977-03-15

A case is reported of aplastic anemia occurring during Methimazole therapy. Since this seems to be a kind of self-limiting disease having considerably rapid recovery, much more detailed supportive therapy is considered to be crucial rather than any other corticosteroid or the androgen therapy.

Susceptibility to aplastic anemia is associated with HLA-DRB1*1501 in an aboriginal population in Sabah, Malaysia.

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Dhaliwal, J S; Wong, Lily; Kamaluddin, Muhammad Amir; Yin, Lee Yin; Murad, Shahnaz

2011-10-01

The incidence of aplastic anemia is reported to be higher in Asia than elsewhere. We studied the frequency of human leukocyte antigen (HLA) DRB1 alleles in aplastic anemia patients from 2 genetically similar aboriginal groups, the Kadazan and the Dusun, and compared them with genetically matched community and hospital controls. HLA-DRB1*15 was significantly higher in the patients compared with controls (p = 0.005), confirming similar findings in Japanese and Caucasian studies. Further testing indicated a significantly higher frequency of HLA-DRB1*1501 in patients compared with controls (p = 0.0004) but no significant difference in the frequency of HLA-DRB1*1502. The high frequency of HLA-DRB1*15 in the Kadazan and Dusun population combined with the wide variety of environmental factors associated with aplastic anemia could be the reason for the elevated incidence of aplastic anemia in the Kadazan and Dusun in Sabah. Copyright © 2011 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.

Bacterial pyomyositis in a patient with aplastic anaemia.

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Mitsuyasu, R.; Gale, R. P.

1980-01-01

Bacterial pyomyositis is common in the tropids but is rare in temperate climates. A patient with aplastic anaemia who had never left the continental United States developed bacterial pyomyositis secondary to Staphylococcus aureus which responded to antibiotics and surgical drainage. Bacterial pyomyositis should be considered in the differential diagnosis of fever and myalgias in the immunocompromised patient.

Reduced intensity conditioning, combined transplantation of haploidentical hematopoietic stem cells and mesenchymal stem cells in patients with severe aplastic anemia.

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Xiao-Hong Li

Full Text Available We examined if transplantation of combined haploidentical hematopoietic stem cells (HSC and mesenchymal stem cells (MSC affected graft failure and graft-versus-host disease (GVHD in patients with severe aplastic anemia (SAA. Patients with SAA-I (N = 17 received haploidentical HSCT plus MSC infusion. Stem cell grafts used a combination of granulocyte colony-stimulating factor (G-CSF-primed bone marrow and G-CSF-mobilized peripheral blood stem cells of haploidentical donors and the culture-expanded third-party donor-derived umbilical cord MSCs (UC-MSCs, respectively. Reduced intensity conditioning consisted of fludarabine (30 mg/m2·d+cyclosphamide (500 mg/m2·d+anti-human thymocyte IgG. Transplant recipients also received cyclosporin A, mycophenolatemofetil, and CD25 monoclonal antibody. A total of 16 patients achieved hematopoietic reconstitution. The median mononuclear cell and CD34 count was 9.3×10(8/kg and 4.5×10(6/kg. Median time to ANC was >0.5×10(9/L and PLT count >20×10(9/L were 12 and 14 days, respectively. Grade III-IV acute GVHD was seen in 23.5% of the cases, while moderate and severe chronic GVHD were seen in 14.2% of the cases. The 3-month and 6-month survival rates for all patients were 88.2% and 76.5%, respectively; mean survival time was 56.5 months. Combined transplantation of haploidentical HSCs and MSCs on SAA without an HLA-identical sibling donor was safe, effectively reduced the incidence of severe GVHD, and improved patient survival.

Yin and Yang of mesenchymal stem cells and aplastic anemia

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Broglie, Larisa; Margolis, David; Medin, Jeffrey A

2017-01-01

Acquired aplastic anemia (AA) is a bone marrow failure syndrome characterized by peripheral cytopenias and bone marrow hypoplasia. It is ultimately fatal without treatment, most commonly from infection or hemorrhage. Current treatments focus on suppressing immune-mediated destruction of bone marrow stem cells or replacing hematopoietic stem cells (HSCs) by transplantation. Our incomplete understanding of the pathogenesis of AA has limited development of targeted treatment options. Mesenchymal stem cells (MSCs) play a vital role in HSC proliferation; they also modulate immune responses and maintain an environment supportive of hematopoiesis. Some of the observed clinical manifestations of AA can be explained by mesenchymal dysfunction. MSC infusions have been shown to be safe and may offer new approaches for the treatment of this disorder. Indeed, infusions of MSCs may help suppress auto-reactive, T-cell mediated HSC destruction and help restore an environment that supports hematopoiesis. Small pilot studies using MSCs as monotherapy or as adjuncts to HSC transplantation have been attempted as treatments for AA. Here we review the current understanding of the pathogenesis of AA and the function of MSCs, and suggest that MSCs should be a target for further research and clinical trials in this disorder. PMID:29321823

Scintigraphic and Radiologic Findings of Pancake Kidney in a Patient with Fanconi Aplastic Anemia

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Adem Maman

2016-06-01

Full Text Available In this case, we have presented that a patient has fankoni aplastic anemia with pancakes kidney in scintigraphy and ultrasonography. The patient is 10 years old and a girl who fanconi aplastic anemia had been diagnosed since three years. In physical examination her general status is good. There was not left hand thumb and she had double the distal phalanx in his right hand thumb in her inspection. We observed 2/6 sistolic murmur in cardiovascular system examınation. Other systems were natural. Abdominal ultrasonography was observed that both the kidney were ectopic location and fused view in the left lower quadrant. Similarly in Tc-99m DTPA and DMSA renal scintigraphy, both kidneys were fused and in the left hemipelvis. The right kidney function were significantly lower by comparison with the left kidney functions. Radiological imaging is necessary in patients with Fanconi aplastic anemia without present clinical symptoms. The renal ultrasonography is important for determining pancakes. In addition, static and dynamic renal scintigraphy plays an important role in revealing the functional status of the kidneys

Significance of bone-marrow scintigraphy in aplastic anemia: concise communication

International Nuclear Information System (INIS)

Najean, Y.; Le Danvic, M.; Le Mercier, N.; Pecking, A.; Colonna, P.; Rain, J.D.

1980-01-01

Tc-99m colloid and In-111 transferrin were used in a semiquantitative scintigraphic study of bone-marrow activity in 76 patients with aplastic anemia, the majority of which were severe cases. The results are compared with other known prognostic parameters and with a predictive index formulated from a prior multiparametric analysis performed in 352 cases. In 47 cases parallel abnormality of Tc and In uptakes was noted and was well correlated with other prognostic factors. Indium uptake is apparently a good indicator of the severity of aplasia; extension of active erythroid tissue, demonstrated with this method, is correlated with prognosis. In nine cases, excessive in uptake is explained by dyserythropoiesis associated with granulo- and thrombocytopenia (Fanconi's anemia in most cases). In 20 of our patients, TcSC uptake was excessive compared with that of In and with other prognostic factors. Statistically, this phenomenon carries an unfavorable prognosis but its physiological meaning remains to be defined

Bone-marrow imaging with indium-111 chloride in aplastic anemia and myelofibrosis: concise communication

International Nuclear Information System (INIS)

Sayle, B.A.; Helmer, R.E.; Birdsong, B.A.; Balachandran, S.; Gardner, F.H.

1982-01-01

Twenty-nine patients with aplastic anemia and 11 patients with myelofibrosis were evaluated with indium-111 chloride bone-marrow imaging, ferrokinetics, and bone-marrow core biopsies. There was good correlation between the erythrocyte cellularity of the marrow and the In-111 bone-marrow scan grades in most patients. In some, the overall scan grade tended to underestimate the erythroid elements because the core biopsy had been taken from the area of the greatest radionuclide concentration on the scan. In patients with aplastic anemia, there was good correlation between the plasma iron clearance t1/2 and the scan grade. Less agreement was found in the comparison between the Fe-59 sacral and organ counts and the red-cell iron utilization. In patients with myelofibrosis, there was poor correlation between the surface counts over the sacrum and the red-cell iron utilization. Plasma iron clearances were abnormally short and were unrelated to the transferrin saturation levels. Eighteen patients were studied several times to evaluate their responses to steroid therapy. In all, there was good correlation between the bone-marrow imaging, the erythrocyte cellularity, ferrokinetics, and the patient's response to therapy. Indium-111 bone-marrow imaging is useful both in evaluating marrow erythroid activity and in following the response to therapy in patients with these diseases

Bedside Evaluation of Cerebral Energy Metabolism in Severe Community-Acquired Bacterial Meningitis

DEFF Research Database (Denmark)

Rom Poulsen, Frantz; Schulz, Mette; Jacobsen, Anne

2015-01-01

BACKGROUND: Mortality and morbidity have remained high in bacterial meningitis. Impairment of cerebral energy metabolism probably contributes to unfavorable outcome. Intracerebral microdialysis is routinely used to monitor cerebral energy metabolism, and recent experimental studies indicate...... that this technique may separate ischemia and non-ischemic mitochondrial dysfunction. The present study is a retrospective interpretation of biochemical data obtained in a series of patients with severe community-acquired meningitis. METHODS: Cerebral energy metabolism was monitored in 15 patients with severe...... community-acquired meningitis utilizing intracerebral microdialysis and bedside biochemical analysis. According to previous studies, cerebral ischemia was defined as lactate/pyruvate (LP) ratio >30 with intracerebral pyruvate level

The clinical application study of bone marrow immunoscintigraphy using 99Tcm-BW250/183 in evaluating patients with aplastic anemia

International Nuclear Information System (INIS)

Liu Zengli; Wu Jinchang; Tang Jun; Wang Wei

2002-01-01

Objective: To study the clinical value of bone marrow immunoscintigraphy for evaluation of patients with aplastic anemia. Methods: Twelve patients with aplastic anemia underwent bone marrow immunoscintigraphy using 99 Tc m labelled anti-granulocyte monoclonal antibody BW250/183, 10 of them also underwent bone marrow imaging using 99 Tc m -sulfur colloid (SC) 2 - 3 days later. The semiquantitative indexes of bone marrow immunoscintigraphy of the patients were compared with those of control patients. Results: Bone marrow immunoscintigraphy was superior to 99 Tc m -SC bone marrow imaging. In patients with aplastic anemia, the accumulation of 99 Tc m -BW250/183 in bone marrow and spleen was lower and in liver and kidney was higher than those of control patients. Nine patients were found with multiple focal accumulation in bone marrow. Conclusion: Bone marrow immunoscintigraphy with 99 Tc m -BW250/183 plays an important role in evaluating patients with aplastic anemia

Increased apoptosis and peripheral blood mononuclear cell suppression of bone marrow mesenchymal stem cells in severe aplastic anemia.

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Chao, Yu-Hua; Lin, Chiao-Wen; Pan, Hui-Hsien; Yang, Shun-Fa; Weng, Te-Fu; Peng, Ching-Tien; Wu, Kang-Hsi

2018-06-05

Although immune-mediated pathogenesis is considered an important aspect of severe aplastic anemia (SAA), its underlying mechanisms remain unclear. Mesenchymal stem cells (MSCs) are essential to the formation of specialized microenvironments in the bone marrow (BM), and MSC insufficiency can trigger the development of SAA. To find MSC alterations in the SAA BM, we compared BM MSCs from five children with SAA and five controls. Peripheral blood mononuclear cells (PBMCs) were cocultured with MSCs to evaluate the supportive effects of MSCs on hematopoiesis. Cytometric bead array immunoassay was used to determine cytokine excretion by MSCs. The immune functions of MSCs and their conditioned medium (CM) were evaluated by PBMC proliferation assays. SAA MSCs were characterized by a high percentage of cells in the abnormal sub-G1 phase of the cell cycle, which suggests an increased rate of apoptosis in SAA MSCs. In comparison with control MSCs, PBMCs cocultured with SAA MSCs displayed significantly reduced PBMC proliferation (P = 0.009). Aberrant cytokine profiles were secreted by SAA MSCs, with increased concentrations of interleukin-6, interferon-γ, tumor necrosis factor-α, and interleukin-1β in the CM. PBMC proliferation assays demonstrated additional immunosuppressive effects of SAA MSCs (P = 0.016) and their CM (P = 0.013). Our data revealed increased apoptosis and PBMC suppression of SAA MSCs. The alterations of MSCs may contribute to the formation of functionally abnormal microenvironments in SAA BM. © 2018 Wiley Periodicals, Inc.

Effects and Predictive Factors of Immunosuppressive Therapy Combined with Umbilical Cord Blood Infusion in Patients with Severe Aplastic Anemia.

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Zhang, Xia; Li, Zhangzhi; Geng, Wei; Song, Bin; Wan, Chucheng

2018-07-01

To investigate the efficacy and safety of umbilical cord blood (UCB) infusion (UCBI) plus immunosuppressive therapy (IST) treatment in comparison to IST treatment, as well as predictive factors for clinical responses, in severe aplastic anemia (SAA) patients. Totally, 93 patients with SAA were enrolled in this cohort study. In the IST group, rabbit antithymocyte globulin (r-ATG) combined with cyclosporine A (CsA) was administered, while in the IST+UBCI group, r-ATG, CsA, and UCB were used. After 6 months of treatment, UCBI+IST achieved a higher complete response (CR) rate (p=0.002) and an elevated overall response rate (ORR) (p=0.004), compared to IST. Regarding hematopoietic recovery at month 6, platelet responses in the UCBI+IST group were better than those in the IST group (p=0.002), and UCBI+IST treatment facilitated increasing trends in absolute neutrophil count (ANC) response (p=0.056). Kaplan-Meier curves illuminated UCBI+IST achieved faster ANC response (paplastic anemia (VSAA) and ANC could predict clinical responses as well. However, Cox proportional hazard regression indicated that VSAA (p=0.003), but not UCBI+IST, affected OS. Safety profiles showed that UCBI+IST therapy did not elevate adverse events, compared with IST treatment. UCBI+IST achieved better clinical responses and hematopoietic recovery than IST, and was well tolerated in SAA patients. © Copyright: Yonsei University College of Medicine 2018.

Detection and Significance of CD4+CD25+CD127dim Regulatory T Cells in Individuals with Severe Aplastic Anemia

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Weiwei Qi

2015-09-01

Full Text Available Objective: To investigate the relationship between CD4+CD25+CD127dim regulatory T cells (Tregs and immune imbalance in acquired severe aplastic anemia (SAA. Materials and Methods: The quantity of CD4+CD25+CD127dim Tregs in 44 SAA patients and 23 normal controls was measured by flow cytometry. Correlations between Tregs and T cell subsets, dendritic cell (DC subsets, granulocyte counts, and percentage of reticulocytes (RET% were analyzed. Results: The percentage of CD4+CD25+CD127dim Tregs in peripheral blood lymphocytes (PBLs of untreated patients was lower than in recovery patients and normal controls (0.83±0.44% vs. 2.91±1.24% and 2.18±0.55%, respectively, p<0.05. The percentage of CD4+CD25+CD127dim Tregs in CD4+ T lymphocytes of recovery patients was higher than that of untreated patients and normal controls (9.39±3.51% vs. 7.61±5.3% and 6.83±1.4%, respectively, p<0.05. The percentage of CD4+ T lymphocytes in PBLs of untreated patients was lower than in recovery patients and normal controls (13.55±7.37% vs. 31.82±8.43% and 32.12±5.88%, respectively, p<0.05. T cell subset (CD4+/CD8+ ratio was 0.41±0.24 in untreated patients, which was lower than in recovery patients (1.2±0.4 and normal controls (1.11±0.23 (p<0.05. DC subset (myeloid DC/plasmacytoid DC ratio, DC1/DC2 ratio was 3.08±0.72 in untreated patients, which was higher than in recovery patients (1.61±0.49 and normal controls (1.39±0.36 (p<0.05. The percentage of CD4+CD25+CD127dim Tregs in PBLs was positively associated with T cell subset (r=0.955, p<0.01 and negatively associated with DC subset (r=-0.765, p<0.01. There were significant positive correlations between CD4+CD25+CD127dim Tregs/PBL and granulocyte counts and RET% (r=0.739 and r=0.749, respectively, p<0.01. Conclusion: The decrease of CD4+CD25+CD127dim Tregs in SAA patients may cause excessive functioning of T lymphocytes and thus lead to hematopoiesis failure in SAA.

Bone marrow transplantation in aplastic anemia, acute leukemia and solid tumors

International Nuclear Information System (INIS)

Champlin, R.; Feig, S.; Gale, R.P.

1980-01-01

Results of bone marrow transplantation for the treatment of aplastic anemia, acute leukemia and solid tumors in the first 141 patients treated between September 1973 and January 1980 are reviewed. Preparation for transplantation with total body irradiation is described. (Auth.)

[Aplastic crisis in sickle cell anemia induced by parvovírus B19

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Borsato, M L; Bruniera, P; Cusato, M P; Spewien, K E; Durigon, E L; Toporovski, J

2000-01-01

PURPOSE: Transient aplastic crisis is reported in an eight-month old child with sickle cell anemia and acute B19 parvovirus infection. This fact is uncommon in this age. PATIENT AND METHODS: The authors review the literature and describe a clinical case of an eight-month old child with sickle cell anemia presented with profound anemia and reticulocytopenia. His peripheral blood was analyzed for parvovirus B19 using the polymerase chain reaction (PCR), and for anti B19 immunoglobulin Ig M, and Ig G by enzyme-linked immunosorbent assay (ELISA). RESULTS: An eight-month old child with sickle cell anemia was admitted to the hospital with fever and profound anemia (HB = 3.8g/ dl) and reticulocytopenia (2%). A diagnosis of aplastic crisis was established. The results indicate that Ig M and PCR were positive and Ig G negative. The patient needed erytrocyte transfusion, and was discharged on hospital day 4. CONCLUSIONS: The clinical and laboratory features indicate that human parvovirus B19 was the etiologic agent of an aplastic crisis in an eight-month old child. According to the international literature this event is uncommon for this age; in addition, this is the first time it appears in the Brazilian literature.

Listeria monocytogenes meningitis in an atomic bomb survivor receiving corticosteroid therapy for aplastic anemia

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Fujihara, Kazuo; Shida, Norihiko; Ohta, Michiya [Hiroshima Atomic Bomb Hospital (Japan)

1995-12-01

We report a case of successfully treated Listeria monocytogenes (Lm) meningitis in a atomic bomb survivor receiving steroid therapy for aplastic anemia. The patient was a 62-year-old woman and the past medical history included hypothyroidism due to radioiodide therapy for Basedow disease, breast cancer, aplastic anemia, steroid-induced diabetes mellitus, and pulmonary tuberculosis. At the time of onset, she was receiving corticosteroid, anabolic steroid, an H{sub 2}-blocker (famotidine), and other medication. Since she developed symptoms of meningitis when she visited our hospital for regular medical check-up for aplastic anemia, she was hospitalized and given antibiotic therapy, including ABPC, without delay. With this effective antibiotic therapy and successful management of the co-existing medical conditions, she was cured except for being a little euphoric. Lm meningitis is known to occur in aged and immunocompromised patients. Since most of the atomic bomb survivors are now aged and the prevalence of malignancy, diabetes mellitus, and other diseases which cause immunodeficiency have been rising year by year, Lm meningitis is one of the emergency neurologic conditions whose diagnosis should not be delayed in this population. (author).

Listeria monocytogenes meningitis in an atomic bomb survivor receiving corticosteroid therapy for aplastic anemia

International Nuclear Information System (INIS)

Fujihara, Kazuo; Shida, Norihiko; Ohta, Michiya

1995-01-01

We report a case of successfully treated Listeria monocytogenes (Lm) meningitis in a atomic bomb survivor receiving steroid therapy for aplastic anemia. The patient was a 62-year-old woman and the past medical history included hypothyroidism due to radioiodide therapy for Basedow disease, breast cancer, aplastic anemia, steroid-induced diabetes mellitus, and pulmonary tuberculosis. At the time of onset, she was receiving corticosteroid, anabolic steroid, an H 2 -blocker (famotidine), and other medication. Since she developed symptoms of meningitis when she visited our hospital for regular medical check-up for aplastic anemia, she was hospitalized and given antibiotic therapy, including ABPC, without delay. With this effective antibiotic therapy and successful management of the co-existing medical conditions, she was cured except for being a little euphoric. Lm meningitis is known to occur in aged and immunocompromised patients. Since most of the atomic bomb survivors are now aged and the prevalence of malignancy, diabetes mellitus, and other diseases which cause immunodeficiency have been rising year by year, Lm meningitis is one of the emergency neurologic conditions whose diagnosis should not be delayed in this population. (author)

[Correlation of SNP of IL-2-330T/G Gene with Genetic Susceptibility and Efficacy of Immunosuppressive Therapy in Patients with Aplastic Anemia].

Science.gov (United States)

Zeng, Qiang; Chang, Hong

2016-10-01

To investigate the correlation of single nucleotide polymorphism (SNP) of Interleukin-2(IL-2)-330T/G with genetic susceptibility and the efficacy of immunosuppressive therapy in patients with aplastic anemia. The peripheral blood samples from 103 patients with aplastic anemia in our hospital were collected. Out of 103 patients 46 received immuosuppressive therapy and were observed for 4 months, and 100 healthy adults were selected as control. The electrophoresis and DNA sequence were performed. The polymerase chain reaction(PCR) was used to amplify the polymorphic gene segment of IL-2 -330T/G from 103 aplastic anemia patients and 100 healthy adults. The frequencis of IL-2-330 GG genotype and G allele were a little higher in patients with aplastic anemia than that in the healthy adults(12.6% vs 12.0%, P>0.05; 27.7% vs 33.5%, P>0.05), but not statistically significant(P>0.05); in the 103 patients with aplastic anemia, 46 received immunosuppressive therapy, whereas 29 patients showed response, no significant difference was found between the responders and non-responders in the IL-2-330 GG genotype and G allele (31.0% vs 48.3%, P>0.05; 64.8% vs 61.8%, P>0.05). IL-2 -330T/G gene polymorphism may not correlate with the susceptibility of aplastic anemia or the efficacy of immunosuppressive therapy.

Why is it necessary to examine retina when the patient suffers from aplastic anemia?

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Tomcikova, D; Gerinec, A; Busanyova, B; Gresikova, M; Biskup, S; Hortnagel, K

2018-01-01

To inform about a case of Revesz syndrome (RS) with initial ophthalmological symptomatology of severe proliferative vitreoretinopathy of the left eye (LE). After the aplastic anemia had developed, RS was established. The exudative retinopathy was successfully treated with photocoagulation on the right eye (RE). RS is characterized by fatal bone marrow failure, exudative retinopathy, neuroradiographic abnormalities, neurodevelopmental delay and skin abnormalities. Non-treated exudative retinopathy leads to blindness. We report ophthalmological findings as follows: fundus photography and fluorescein angiography (FA) acquired by examinations under general anesthesia in patient with RS. Results of genetic tests helped to establish the diagnosis. Two‑year old Caucasian male was examined due to total retinal detachment on LE and signs of chorioretinal scarring on RE. In preoperative screening, thrombocytopenia was detected; later, severe pancytopenia developed. Considering the hematological findings and clinical appearance, we suspected RS, which was confirmed by genetic tests. We found a pathogenic mutation in gene TINF2 (variant c.865C>T;p.Pro289Ser) in a mosaic state with autosomal dominant mode of inheritance. This mutation has not been described in RS yet. Blind LE was enucleated because of dolorous neovascular glaucoma. FA of RE shows excessive areas of capillary nonperfusion with vascular abnormalities and exudation. After the photocoagulation, the visual acuity (VA) on RE remains 0.9 at the age of 7 years. RS is an extremely rare condition.  The initial symptomatology could be ophthalmological or hematological. The positive finding of TINF2 gene mutation helped in establishing the correct diagnosis. The ischemic retinopathy was successfully treated by photocoagulation (Fig. 6, Ref. 6). Text in PDF www.elis.sk.

Further Evaluation of Androgen Therapy In Aplastic Anemia: With Special Reference to Correlation Between Response to Androgen and EEI

International Nuclear Information System (INIS)

Whang, Kee Suk

1967-01-01

Patients with aplastic anemia were treated with a combination of depo-testosterone cyclopentylpropionate (Upjohn) and dexamethasone. In 7 of 15 patients treated, there was response in which either a significant increase in hemoglobin concentration, a prolonged interval or a cessation of blood transfusion requirement developed during androgen therapy. Younger patients with cellular marrow appeared to be better responding to androgen. EEI (Effective Erythropoietic Index) formulated by Gardner and Nathan (1966) which was a helpful measurement as to whether patients with myelofibrosis would response to androgen, was evaluated in patients with aplastic anemia. It was concluded that EEI as well as ferrokinetics indices (Plasma- 59 Fe-disappearance rate, RBC 59 Fe net incorporation) did not significantly correlate with the degree of response to androgen in aplastic anemia.

Prognostic value of severity indicators of nursing-home-acquired pneumonia versus community-acquired pneumonia in elderly patients.

Science.gov (United States)

Ugajin, Motoi; Yamaki, Kenichi; Hirasawa, Natsuko; Kobayashi, Takanori; Yagi, Takeo

2014-01-01

The credibility of prognostic indicators in nursing-home-acquired pneumonia (NHAP) is not clear. We previously reported a simple prognostic indicator in community-acquired pneumonia (CAP): blood urea nitrogen to serum albumin (B/A) ratio. This retrospective study investigated the prognostic value of severity indicators in NHAP versus CAP in elderly patients. Patients aged ≥65 years and hospitalized because of NHAP or CAP within the previous 3 years were enrolled. Demographics, coexisting illnesses, laboratory and microbiological findings, and severity scores (confusion, urea, respiratory rate, blood pressure, and age ≥65 [CURB-65] scale; age, dehydration, respiratory failure, orientation disturbance, and pressure [A-DROP] scale; and pneumonia severity index [PSI]) were retrieved from medical records. The primary outcome was mortality within 28 days of admission. In total, 138 NHAP and 307 CAP patients were enrolled. Mortality was higher in NHAP (18.1%) than in CAP (4.6%) (Pscale, 0.69 for the CURB-65 scale, 0.67 for the PSI class, and 0.65 for the B/A ratio. The area under the curve in CAP was 0.73 for the A-DROP scale, 0.76 for the CURB-65 scale, 0.81 for the PSI class, and 0.83 for the B/A ratio. Patient mortality was greater in NHAP than in CAP. Patient characteristics, coexisting illnesses, and detected pathogens differed greatly between NHAP and CAP. The existing severity indicators had less prognostic value for NHAP than for CAP.

Aplasia medular após transplante hepático em pediatria Aplastic anemia after pediatric liver transplantation

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Marlene P. Garanito

2009-01-01

Full Text Available A aplasia de medula é uma das mais raras (Aplastic anemia (AA is one of the rarest (<1% and most serious complications of liver transplantation for fulminant non-A, non-B and non-C hepatitis. It was first described in 1987 by Stock; the mechanism involved is an immunologically mediated condition secondary to an unknown viral infection. The disease is associated with a dismal prognosis. Spontaneous recovery from acquired AA is very rare however some patients (50-70% recover after immunosuppressive therapy, such as Cyclosporin A (CsA and Antithymocyte globulin (ATG, even after liver transplantation. Another treatment option is bone marrow transplantation. We report on a child who developed AA following liver transplantation for fulminant viral hepatitis that was treated with intensive immunosuppression including CsA and ATG and achieved complete recovery.

Recent advances in treatment of aplastic anemia

Science.gov (United States)

Shin, Seung Hwan; Lee, Sung Eun

2014-01-01

Recent advances in the treatment of aplastic anemia (AA) made most of patients to expect to achieve a long-term survival. Allogeneic stem cell transplantation (SCT) from HLA-matched sibling donor (MSD-SCT) is a preferred first-line treatment option for younger patients with severe or very severe AA, whereas immunosuppressive treatment (IST) is an alternative option for others. Horse anti-thymocyte globuline (ATG) with cyclosporin A (CsA) had been a standard IST regimen with acceptable response rate. Recently, horse ATG had been not available and replaced with rabbit ATG in most countries. Subsequently, recent comparative studies showed that the outcomes of patients who received rabbit ATG/CsA were similar or inferior compared to those who received horse ATG/CsA. Therefore, further studies to improve the outcomes of IST, including additional eltrombopag, are necessary. On the other hand, the upper age limit of patients who are able to receive MSD-SCT as first-line treatment is a current issue because of favorable outcomes of MSD-SCT of older patients using fludarabine-based conditioning. In addition, further studies to improve the outcomes of patients who receive allogeneic SCT from alternative donors are needed. In this review, current issues and the newly emerging trends that may improve their outcomes in near futures will be discussed focusing the management of patients with AA. PMID:25378968

Further Evaluation of Androgen Therapy In Aplastic Anemia: With Special Reference to Correlation Between Response to Androgen and EEI

Energy Technology Data Exchange (ETDEWEB)

Whang, Kee Suk [Kyungpook National University School of Medicine, Deagu (Korea, Republic of)

1967-03-15

Patients with aplastic anemia were treated with a combination of depo-testosterone cyclopentylpropionate (Upjohn) and dexamethasone. In 7 of 15 patients treated, there was response in which either a significant increase in hemoglobin concentration, a prolonged interval or a cessation of blood transfusion requirement developed during androgen therapy. Younger patients with cellular marrow appeared to be better responding to androgen. EEI (Effective Erythropoietic Index) formulated by Gardner and Nathan (1966) which was a helpful measurement as to whether patients with myelofibrosis would response to androgen, was evaluated in patients with aplastic anemia. It was concluded that EEI as well as ferrokinetics indices (Plasma-{sup 59}Fe-disappearance rate, RBC {sup 59}Fe net incorporation) did not significantly correlate with the degree of response to androgen in aplastic anemia.

Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia

Energy Technology Data Exchange (ETDEWEB)

Schuster, F R; Hubner, B [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Führer, M [Department of Pediatric Oncology and Hematology, Dr von Haunersches Children' s Hospital, University of Munich, Munich (Germany); Eckermann, O; Gombert, M [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Dornmair, K [Department for Clinical Neuroimmunology, University of Munich, Munich (Germany); Binder, V; Reuther, S; Krell, P [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany); Keller, T [Acomed, statistical analysis GmbH, Leipzig (Germany); Borkhardt, A [Clinic of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich Heine University, Düsseldorf (Germany)

2011-03-01

One of the major obstacles of immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA) comes from the often months-long unpredictability of bone-marrow (BM) recovery. In this prospective study in children with newly diagnosed very severe AA (n=10), who were enrolled in the therapy study SAA-BFM 94, we found a dramatically reduced diversity of both CD4+ and CD8+ BM cells, as scored by comprehensive V-beta chain T-cell receptor (TCR) analysis. Strongly skewed TCR V-beta pattern was highly predictive for good or at least partial treatment response (n=6, CD8+ complexity scoring median 35.5, range 24–73). In contrast, IST in patients with rather moderate reduction of TCR V-beta diversity (n=4, CD8+ complexity scoring median 109.5, range 82–124) always failed (P=0.0095). If confirmed in a larger series of patients, TCR V-beta repertoire in BM may help to assign children with SAA up-front either to IST or to allogeneic stem-cell transplantation.

Highly skewed T-cell receptor V-beta chain repertoire in the bone marrow is associated with response to immunosuppressive drug therapy in children with very severe aplastic anemia

International Nuclear Information System (INIS)

Schuster, F R; Hubner, B; Führer, M; Eckermann, O; Gombert, M; Dornmair, K; Binder, V; Reuther, S; Krell, P; Keller, T; Borkhardt, A

2011-01-01

One of the major obstacles of immunosuppressive therapy (IST) in children with severe aplastic anemia (SAA) comes from the often months-long unpredictability of bone-marrow (BM) recovery. In this prospective study in children with newly diagnosed very severe AA (n=10), who were enrolled in the therapy study SAA-BFM 94, we found a dramatically reduced diversity of both CD4+ and CD8+ BM cells, as scored by comprehensive V-beta chain T-cell receptor (TCR) analysis. Strongly skewed TCR V-beta pattern was highly predictive for good or at least partial treatment response (n=6, CD8+ complexity scoring median 35.5, range 24–73). In contrast, IST in patients with rather moderate reduction of TCR V-beta diversity (n=4, CD8+ complexity scoring median 109.5, range 82–124) always failed (P=0.0095). If confirmed in a larger series of patients, TCR V-beta repertoire in BM may help to assign children with SAA up-front either to IST or to allogeneic stem-cell transplantation

Clinical significance of determination of changes of serum TNF-α, IFN-γ and T-cell subsets distribution pattern in pediatric patients with aplastic anemia

International Nuclear Information System (INIS)

Feng Yue

2008-01-01

Objective: To explore the changes of serum TNF-α, IFN-γ and T-cell subsets distribution pattern in pediatric patients with aplastic anemia. Methods: Serum TNF-α levels (with RIA), IFN-γ levels (with ELISA), peripheral blood T-cell subsets distribution pattern (with monoclonal antibody technique) were determined in 33 pediatric patients with aplastic anemia, as well as in 35 controls. Results: The serum levels of TNF-α and IFN-γ in the patients with aplastic anemia were significantly higher than those in the controls (P<0.01), while the CD3, CD4 percentages and CD4/CD8 ratio were significantly lower (P<0.01). Conclusion: Detection of changes of serum TNF-α, IFN-γ levels and T-cell subsets ratio was clinically useful for outcome prediction in pediatric patients with aplastic anemia. (authors)

Deregulation of vital mitotic kinase-phosphatase signaling in hematopoietic stem/progenitor compartment leads to cellular catastrophe in experimental aplastic anemia.

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Chatterjee, Ritam; Chattopadhyay, Sukalpa; Law, Sujata

2016-11-01

Aplastic anemia, the paradigm of bone marrow failure, is characterized by pancytopenic peripheral blood and hypoplastic bone marrow. Among various etiologies, inappropriate use of DNA alkylating drugs like cyclophosphamide and busulfan often causes the manifestation of the dreadful disease. Cell cycle impairment in marrow hematopoietic stem/progenitor compartment together with cellular apoptosis has been recognized as culpable factors behind aplastic pathophysiologies. However, the intricate molecular mechanisms remain unrevealed till date. In the present study, we have dealt with the mechanistic intervention of the disease by peripheral blood hemogram, bone marrow histopathology, cytopathology, hematopoietic kinetic study, scanning electron microscopy, DNA damage assessment and flowcytometric analysis of cellular proliferation and apoptosis in hematopoietic stem/progenitor cell (HSPC) rich marrow compartment using busulfan and cyclophosphamidemediated mouse model. To unveil the molecular mechanisms behind aplastic pathophysiology, we further investigated the role of some crucial mitotic and apoptotic regulators like Protein kinase-B (PKB), Gsk-3β, Cyclin-D1, PP2A, Cdc25c, Plk-1, Aurora kinase-A, Chk-1 regarding the hematopoietic catastrophe. Our observations revealed that the alteration of PKB-GSK-3β axis, Plk-1, and Aurora kinase-A expressions in HSPC compartment due to DNA damage response was associated with the proliferative impairment and apoptosis during aplastic anemia. The study established the correlation between the accumulation of DNA damage and alteration of the mentioned molecules in aplastic HSPCs that lead to the hematopoietic catastrophe. We anticipate that our findings will be beneficial for developing better therapeutic strategies for the dreadful disease concerned.

Efficacy and safety of immunosuppressive therapy in the treatment of seronegative hepatitis associated aplastic anemia

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Chen HF

2014-09-01

Full Text Available Hai-Fei Chen,* Bin-Xian Xu, Hong-Shi Shen,* Zheng-Yang Li, Ling-Juan Jin, Jie-Qing Tang, Jing Wang, Jing-Jing Zhu, Long-Mei Qin, Qing-Ya Cui, Yong-Ya Ren, Tian-Qin Wu Department of hematology, 100th hospital of People’s Liberation Army, Suzhou, Jiangsu province, The People’s Republic of China *These authors have contributed equally to this paper Objective: To investigate the clinical characteristics of seronegative hepatitis-associated aplastic anemia (AA (SNHAA and hepatitis B virus (HBV infection complicating AA (HBVAA, and thereby compare the efficacy of immunosuppressive therapy (IST.Methods: An analysis was conducted on the clinical data of ten patients with SNHAA out of 332 cases of AA from our center at AA diagnosis, and on the efficacy of IST. This was compared to 22 cases of HBVAA at AA onset as well as the associated IST outcomes.Results: Nine patients with SNHAA developed severe aplastic anemia, with a median age of 18 years. After IST, six (60% of the SNHAA patients achieved complete remission and two achieved partial remission. The patients with HBVAA had a total response rate of 82.3%. The disease recurred in two HBVAA patients. No statistically significant differences were observed in response rate, mortality, and recurrence rate between both groups. As compared with HBVAA, patients with SNHAA had a shorter interval from the acute episode of hepatitis to AA onset (4 months versus 92 months, P=0.00, a quicker response to IST (2.5 months versus 4.5 months, P=0.018, a lower proportion of bone marrow hematopoietic tissues (20.6% versus 23.6%, P=0.03, and lower white blood cell and absolute neutrophil count (0.8×109/L versus 1.23×109/L and 0.26×109/L versus 0.58×109/L, P=0.026 and P=0.0009, respectively. No significant liver damage or hepatitis B fulminant infection was observed in either group during the follow-up. Conclusion: The prevalence of SNHAA is 3.01%. SNHAA often presents as severe AA and responds to IST quickly

Outcomes and healthcare utilization in children and young adults with aplastic anemia: A multiinstitutional analysis.

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Gupta, Ashish; Fu, Pingfu; Hashem, Hasan; Vatsayan, Anant; Shein, Steven; Dalal, Jignesh

2017-12-01

Aplastic anemia is a bone marrow failure syndrome with high mortality affecting children and young adults. Although current treatment guidelines recommend hematopoietic stem cell transplant (HCT) for patients with matched sibling donors, outcomes with alternate donor options have been improving. We analyzed a validated multiinstitutional pediatric cohort using one of the largest pediatric and young adult database, the Pediatric Health Information System, for patients diagnosed with aplastic anemia (AA) from 2006 to 2015. Outcomes with upfront and salvage transplants were analyzed along with healthcare utilization. Among 2,169 patients in the study period, almost 20% underwent HCT, while others received immunosuppressive therapy. In a multivariate model, there was no significant difference in mortality with upfront or salvage transplants (odds ratio [OR] 1.24, 95% confidence interval [CI] 0.6-2.58, P = 0.567), while every platelet transfusion was associated with higher mortality (OR 1.37, 95% CI 1.12-1.67, P = 0.002). Healthcare utilization was significantly higher in salvage transplants requiring frequent hospitalization and transfusion requirements. Treatment mortality and graft failure rates were significantly reduced in the salvage transplant group in recent years (2011-2015 as compared to 2006-2010). As outcomes with HCT continue to improve in severe AA, transplant with good alternate donors should be considered upfront in children and young adults. © 2017 Wiley Periodicals, Inc.

CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation

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2017-07-13

Bone Marrow Failure Syndrome; Severe Aplastic Anemia; Severe Congenital Neutropenia; Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Schwachman Diamond Syndrome; Primary Immunodeficiency Syndromes; Acquired Immunodeficiency Syndromes; Histiocytic Syndrome; Familial Hemophagocytic Lymphocytosis; Lymphohistiocytosis; Macrophage Activation Syndrome; Langerhans Cell Histiocytosis (LCH); Hemoglobinopathies; Sickle Cell Disease; Sickle Cell-beta-thalassemia

Comparable Outcomes after HLA-Matched Sibling and Alternative Donor Hematopoietic Cell Transplantation for Children with Fanconi Anemia and Severe Aplastic Anemia.

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Ebens, Christen L; DeFor, Todd E; Tryon, Rebecca; Wagner, John E; MacMillan, Margaret L

2018-04-01

Fanconi anemia (FA)-associated severe aplastic anemia (SAA) requires allogeneic hematopoietic cell transplantation (HCT) for cure. With the evolution of conditioning regimens over time, outcomes of alternative donor HCT (AD-HCT) have improved dramatically. We compared outcomes of HLA-matched sibling donor HCT (MSD-HCT; n = 17) and AD-HCT (n = 57) performed for FA-associated SAA at a single institution between 2001 and 2016. Overall survival at 5 years was 94% for MSD-HCT versus 86% for AD-HCT, neutrophil engraftment was 100% versus 95%, platelet recovery was 100% versus 89%, grade II-IV acute graft-versus-host disease (GVHD) was 6% versus 12%, grade III-IV acute GVHD was 6% versus 4%, and chronic GVHD was 0 versus 7%, with no statistically significant differences by type of transplant. The use of UCB was associated with decreased rates of neutrophil recovery in AD-HCT and platelet recovery in both MSD-HCT and AD-HCT. A trend toward a higher serious infection density before day +100 post-HCT was observed in AD-HCT compared with MSD-HCT (P = .02). These data demonstrate that AD-HCT should be considered at the same time as MSD-HCT for patients with FA-associated SAA. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

Aplastic anemia as a cause of death in a patient with glioblastoma multiforme treated with temozolomide

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Kopecky, Jindrich; Priester, Peter; Slovacek, Ladislav; Petera, Jiri; Macingova, Zuzana; Kopecky, Otakar

2010-01-01

Background: Standard treatment of glioblastoma multiforme consists of postoperative radiochemotherapy with temozolomide, followed by a 6-month chemotherapy. Serious hematologic complications are rarely reported. Case Report and Results: The authors present the case of a 61-year-old female patient with glioblastoma multiforme treated with external-beam radiation therapy and concomitant temozolomide. After completion of treatment, the patient developed symptoms of serious aplastic anemia that eventually led to death due to prolonged neutro- and thrombocytopenia followed by infectious complications. Conclusion: Lethal complications following temozolomide are, per se, extremely rare, however, a total of four other cases of aplastic anemia have been reported in the literature so far. (orig.)

Aplastic anemia as a cause of death in a patient with glioblastoma multiforme treated with temozolomide

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Kopecky, Jindrich; Priester, Peter; Slovacek, Ladislav; Petera, Jiri; Macingova, Zuzana [Dept. of Clinical Oncology and Radiotherapy, Charles Univ. Hospital and Faculty of Medicine in Hradec Kralove (Czech Republic); Kopecky, Otakar [Clinical Oncology, Regional Hospital Nachod (Czech Republic)

2010-08-15

Background: Standard treatment of glioblastoma multiforme consists of postoperative radiochemotherapy with temozolomide, followed by a 6-month chemotherapy. Serious hematologic complications are rarely reported. Case Report and Results: The authors present the case of a 61-year-old female patient with glioblastoma multiforme treated with external-beam radiation therapy and concomitant temozolomide. After completion of treatment, the patient developed symptoms of serious aplastic anemia that eventually led to death due to prolonged neutro- and thrombocytopenia followed by infectious complications. Conclusion: Lethal complications following temozolomide are, per se, extremely rare, however, a total of four other cases of aplastic anemia have been reported in the literature so far. (orig.)

Study of erythropoiesis with 59Fe and erythrocyte survival with 51Cr in aplastic anemia

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Avramova, D.; Karakostov, K.; Dimtrov, L.; Osmanliev, P.

1979-01-01

A study of erythropoiesis with 59 Fe and erythrocyte survival with 51 Cr was carried out in 25 patients with aplastic anemia and in a control group of 23 healthy persons. Bone marrow aplasia was uniform in all patients but differences in erythropoiesis were established. According to the state of erythropoiesis, the patients were divided into two groups: 1) patients with severe damage of erythropoiesis and 2) patients with preserved erythropoiesis. Erythrocyte survival in both groups was shortened. The data obtained correlate with the anemic syndrome and help to explain its pathogenesis. It is suggested that the investigation of erythropoiesis with 59 Fe and 51 Cr is of prognostic value. (author)

Severe Dermatophytosis and Acquired or Innate Immunodeficiency: A Review

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Claire Rouzaud

2015-12-01

Full Text Available Dermatophytes are keratinophilic fungi responsible for benign and common forms of infection worldwide. However, they can lead to rare and severe diseases in immunocompromised patients. Severe forms include extensive and/or invasive dermatophytosis, i.e., deep dermatophytosis and Majocchi’s granuloma. They are reported in immunocompromised hosts with primary (autosomal recessive CARD9 deficiency or acquired (solid organ transplantation, autoimmune diseases requiring immunosuppressive treatments, HIV infection immunodeficiencies. The clinical manifestations of the infection are not specific. Lymph node and organ involvement may also occur. Diagnosis requires both mycological and histological findings. There is no consensus on treatment. Systemic antifungal agents such as terbinafine and azoles (itraconazole or posaconazole are effective. However, long-term outcome and treatment management depend on the site and extent of the infection and the nature of the underlying immunodeficiency.

Syngeneic transplantation in aplastic anemia: pre-transplant conditioning and peripheral blood are associated with improved engraftment: an observational study on behalf of the Severe Aplastic Anemia and Pediatric Diseases Working Parties of the European Group for Blood and Marrow Transplantation

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Gerull, Sabine; Stern, Martin; Apperley, Jane; Beelen, Dietrich; Brinch, Lorentz; Bunjes, Donald; Butler, Andrew; Ganser, Arnold; Ghavamzadeh, Ardeshir; Koh, Mickey B; Komarnicki, Mieczyslaw; Kröger, Nicolaus; Maertens, Johan; Maschan, Alexei; Peters, Christina; Rovira, Montserrat; Sengeløv, Henrik; Socié, Gerard; Tischer, Johanna; Oneto, Rosi; Passweg, Jakob; Marsh, Judith

2013-01-01

Aplastic anemia is usually treated with immunosuppression or allogeneic transplant, depending on patient and disease characteristics. Syngeneic transplant offers a rare treatment opportunity with minimal transplant-related mortality, and offers an insight into disease mechanisms. We present here a retrospective analysis of all syngeneic transplants for aplastic anemia reported to the European Group for Blood and Marrow Transplantation. Between 1976 and 2009, 88 patients received 113 transplants. Most transplants (n=85) were preceded by a conditioning regimen, 22 of these including anti-thymocyte globulin. About half of transplants with data available (39 of 86) were followed by posttransplant immunosuppression. Graft source was bone marrow in the majority of cases (n=77). Transplant practice changed over time with more transplants with conditioning and anti-thymocyte globulin as well as peripheral blood stem cells performed in later years. Ten year overall survival was 93% with 5 transplant-related deaths. Graft failure occurred in 32% of transplants. Risk of graft failure was significantly increased in transplants without conditioning, and with bone marrow as graft source. Lack of posttransplant immunosuppression also showed a trend towards increased risk of graft failure, while anti-thymocyte globulin did not have an influence. In summary, syngeneic transplant is associated with a significant risk of graft failure when no conditioning is given, but has an excellent long-term outcome. Furthermore, our comparatively large series enables us to recommend the use of pre-transplant conditioning rather than not and possibly to prefer peripheral blood as a stem cell source. PMID:23894010

Clinical significance of determination of serum TNF-α, VEGF and TSGF levels after treatment in patients with aplastic anemia

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Hu Mingqiu; Xu Yanli

2009-01-01

Objective: To explore the clinical significance changes of serum TNF-α, VEGF and TSGF levels after treatment in patients with aplastic Anemia. Methods: Serum TNF-α(with RIA), VEGF(with ELISA) and TSGF(with biochemistry) levels were determined in 33 patients with aplastic anemia both before and after treatment and 35 controls. Results: Before treatment, the serum TNF-α, TSGF levels were significantly higher in the patients than those in controls (P<0.01), but serum VEGF levels were significantly lower in the patients (P<0.01). Serum TNF-α, TSGF levels were negatively correlated with levels of VEGF(r=-0.5192, -0.6018, P<0.01). After a course of treatment, the serum TNF-α, VEGF and TSGF levels, though corrected markedly, remained significantly different from those in controls (P<0.05). Conclusion: Determination of serum TNF-α, VEGF and TSGF levels after treatment might be of prognostic importance in patients with aplastic anemia. (authors)

Somatic HLA mutations expose the role of class I–mediated autoimmunity in aplastic anemia and its clonal complications

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Duke, Jamie L.; Xie, Hongbo M.; Stanley, Natasha; Atienza, Jamie; Perdigones, Nieves; Nicholas, Peter; Ferriola, Deborah; Li, Yimei; Huang, Hugh; Ye, Wenda; Morrissette, Jennifer J. D.; Kearns, Jane; Porter, David L.; Podsakoff, Gregory M.; Eisenlohr, Laurence C.; Biegel, Jaclyn A.; Chou, Stella T.; Monos, Dimitrios S.; Bessler, Monica; Olson, Timothy S.

2017-01-01

Acquired aplastic anemia (aAA) is an acquired deficiency of early hematopoietic cells, characterized by inadequate blood production, and a predisposition to myelodysplastic syndrome (MDS) and leukemia. Although its exact pathogenesis is unknown, aAA is thought to be driven by human leukocyte antigen (HLA)–restricted T cell immunity, with earlier studies favoring HLA class II-mediated pathways. Using whole-exome sequencing (WES), we recently identified 2 patients with aAA with somatic mutations in HLA class I genes. We hypothesized that HLA class I mutations are pathognomonic for autoimmunity in aAA, but were previously underappreciated because the major histocompatibility complex (MHC) region is notoriously difficult to analyze by WES. Using a combination of targeted deep sequencing of HLA class I genes and single nucleotide polymorphism array (SNP-A) genotyping, we screened 66 patients with aAA for somatic HLA class I loss. We found somatic HLA loss in 11 patients (17%), with 13 loss-of-function mutations in HLA-A*33:03, HLA-A*68:01, HLA-B*14:02, and HLA-B*40:02 alleles. Three patients had more than 1 mutation targeting the same HLA allele. Interestingly, HLA-B*14:02 and HLA-B*40:02 were significantly overrepresented in patients with aAA compared with ethnicity-matched controls. Patients who inherited the targeted HLA alleles, regardless of HLA mutation status, had a more severe disease course with more frequent clonal complications as assessed by WES, SNP-A, and metaphase cytogenetics, and more frequent secondary MDS. The finding of recurrent HLA class I mutations provides compelling evidence for a predominant HLA class I-driven autoimmunity in aAA and establishes a novel link between immunogenetics and clonal evolution of patients with aAA. PMID:28971166

Somatic HLA Mutations Expose the Role of Class I-Mediated Autoimmunity in Aplastic Anemia and its Clonal Complications.

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Babushok, Daria V; Duke, Jamie L; Xie, Hongbo M; Stanley, Natasha; Atienza, Jamie; Perdigones, Nieves; Nicholas, Peter; Ferriola, Deborah; Li, Yimei; Huang, Hugh; Ye, Wenda; Morrissette, Jennifer J D; Kearns, Jane; Porter, David L; Podsakoff, Gregory M; Eisenlohr, Laurence C; Biegel, Jaclyn A; Chou, Stella T; Monos, Dimitrios S; Bessler, Monica; Olson, Timothy S

2017-10-10

Acquired aplastic anemia (aAA) is an acquired deficiency of early hematopoietic cells, characterized by inadequate blood production, and a predisposition to myelodysplastic syndrome (MDS) and leukemia. Although its exact pathogenesis is unknown, aAA is thought to be driven by Human Leukocyte Antigen (HLA)-restricted T cell immunity, with earlier studies favoring HLA class II-mediated pathways. Using whole exome sequencing (WES), we recently identified two aAA patients with somatic mutations in HLA class I genes. We hypothesized that HLA class I mutations are pathognomonic for autoimmunity in aAA, but were previously underappreciated because the Major Histocompatibility Complex (MHC) region is notoriously difficult to analyze by WES. Using a combination of targeted deep sequencing of HLA class I genes and single nucleotide polymorphism array (SNP-A) genotyping we screened 66 aAA patients for somatic HLA class I loss. We found somatic HLA loss in eleven patients (17%), with thirteen loss-of-function mutations in HLA-A *33:03, HLA-A *68:01, HLA-B *14:02 and HLA-B *40:02 alleles. Three patients had more than one mutation targeting the same HLA allele. Interestingly, HLA-B *14:02 and HLA-B *40:02 were significantly overrepresented in aAA patients, compared to ethnicity-matched controls. Patients who inherited the targeted HLA alleles, regardless of HLA mutation status, had a more severe disease course with more frequent clonal complications as assessed by WES, SNP-A, and metaphase cytogenetics, and more frequent secondary MDS. The finding of recurrent HLA class I mutations provides compelling evidence for a predominant HLA class I-driven autoimmunity in aAA, and establishes a novel link between aAA patients' immunogenetics and clonal evolution.

Outcomes of Optimized over Standard Protocol of Rabbit Antithymocyte Globulin for Severe Aplastic Anemia: A Single-Center Experience

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Ge, Meili; Shao, Yingqi; Huang, Jinbo; Huang, Zhendong; Zhang, Jing; Nie, Neng; Zheng, Yizhou

2013-01-01

Background Previous reports showed that outcome of rabbit antithymocyte globulin (rATG) was not satisfactory as the first-line therapy for severe aplastic anemia (SAA). We explored a modifying schedule of administration of rATG. Design and Methods Outcomes of a cohort of 175 SAA patients, including 51 patients administered with standard protocol (3.55 mg/kg/d for 5 days) and 124 cases with optimized protocol (1.97 mg/kg/d for 9 days) of rATG plus cyclosporine (CSA), were analyzed retrospectively. Results Of all 175 patients, response rates at 3 and 6 months were 36.6% and 56.0%, respectively. 51 cases received standard protocol had poor responses at 3 (25.5%) and 6 months (41.2%). However, 124 patients received optimized protocol had better responses at 3 (41.1%, P = 0.14) and 6 (62.1%, P = 0.01). Higher incidences of infection (57.1% versus 37.9%, P = 0.02) and early mortality (17.9% versus 0.8%, P<0.001) occurred in patients received standard protocol compared with optimized protocol. The 5-year overall survival in favor of the optimized over standard rATG protocol (76.0% versus. 50.3%, P<0.001) was observed. By multivariate analysis, optimized protocol (RR = 2.21, P = 0.04), response at 3 months (RR = 10.31, P = 0.03) and shorter interval (<23 days) between diagnosis and initial dose of rATG (RR = 5.35, P = 0.002) were independent favorable predictors of overall survival. Conclusions Optimized instead of standard rATG protocol in combination with CSA remained efficacious as a first-line immunosuppressive regimen for SAA. PMID:23554855

Acquired Severe Disabilities and Complex Health Care Needs: Access to Inclusive Education

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Ballard, Sarah L.; Dymond, Stacy K.

2016-01-01

This case study examined one high school student's access to inclusive education and experiences in an inclusive English class after he acquired severe disabilities and complex health care needs from a nontraumatic brain injury. Multiple sources of data (i.e., interviews, field notes, and documents) were collected and analyzed to formulate…

Clinical evaluation of determination of changes of serum IL-8, TNF-α and VEGF levels after treatment in patients with aplastic anemia

International Nuclear Information System (INIS)

Yu Guifen; Wang Ying; Yan Dongmei

2011-01-01

Objective: To explore the clinical significance of changes of serum IL-8, TNF-α and VEGF levels after treatment in patients with aplastic anemia. Methods: Serum IL-8, TNF-α (with RIA), serum VEGF (with ELISA) levels were determined both before and after treatment in 30 patients with aplastic anemia and 35 normal controls. Results: Before treatment, serum IL-8, TNF-α levels in the patients were significantly higher than those in controls (P<0.01), while the serum VEGF level was absolutely lower (P<0.01), after 3 months treatment, the serum IL-8, TNF-α and VEGF levels were still significant (P<0.05). Conclusion: Detection of changes of serum IL-8, TNF-α and VEGF levels exist important clinical value for prediction in patients with aplastic anemia. (authors)

A retrospective comparison of cyclophosphamide plus antithymocyte globulin with cyclophosphamide plus busulfan as the conditioning regimen for severe aplastic anemia

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L.V.M. Ommati

2009-03-01

Full Text Available Allogeneic hematopoietic stem cell transplantation (AHSCT is the treatment of choice for young patients with severe aplastic anemia (SAA. The association of antithymocyte globulin (ATG and cyclophosphamide (CY is the most frequently used conditioning regimen for this disease. We performed this retrospective study in order to compare the outcomes of HLA-matched sibling donor AHSCT in 41 patients with SAA receiving cyclophosphamide plus ATG (ATG-CY, N = 17 or cyclophosphamide plus busulfan (BU-CY, N = 24. The substitution of BU for ATG was motivated by the high cost of ATG. There were no differences in the clinical features between the two groups, including age, gender, cytomegalovirus status, ABO match, interval between diagnosis and transplant, and number of total nucleated cells infused. No differences were observed in the time to neutrophil and platelet engraftment, or in the risk of veno-occlusive disease and hemorrhage. However, there was a higher risk of mucositis in the BU-CY group (71 vs 24%, P = 0.004. There were no differences in the incidence of neutrophil and platelet engraftment, acute and chronic graft-versus-host disease, and transplant-related mortality. There was a higher incidence of late rejection in the ATG-CY group (41 vs 4%, P = 0.009. Although the ATG-CY group had a longer follow-up (101 months than the BU-CY group (67 months, P = 0.04, overall survival was similar between the groups (69 vs 58%, respectively, P = 0.32. We conclude that the association BU-CY is a feasible option to the conventional ATG-CY regimen in this population.

Clinical significance of measurement of plasma relevant cytokines (GM-CSF, IL-2, TPO, EPO) levels in patients with aplastic anemia

International Nuclear Information System (INIS)

Yu Tintin

2006-01-01

Objective: To investigate the role of relevant cytokines in the development and pathogenesis of aplastic anemia. Methods: Plasma GM-CSF, IL-2, TPO (with RIA) and EPO (with CLIA) contents were measured in 100 patients (acute 43, chronic 57) with aplastic anemia and 50 controls. Complete blood count was also performed in all these subjects. Results: The peripheral RBC, WBC, platelet counts and GM-CSF contents were significantly lower in the patients with aplastic anemia than those in the controls (P<0.05), while the IL-2, EPO and TPO contents were significantly higher in the patients (P<0.05). GM-CSF contents were positively correlated with the WBC numbers. EPO contents were negatively correlated with the RBC counts and TPO contents were correlated (negatively) with the platelet counts. Conclusion: There was correlationship between each blood elements (WBC, RBC, platelet) and its corresponding cytokine (GS-CSF, EPO, TPO respectively). IL-2 contents were not correlated with WBC counts. (authors)

Total lymphoid irradiation (TLI) for allogeneic bone marrow transplantation in aplastic anemia

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Yamanashi, Syunji; Yamashita, Takashi; Mochizuki, Sachio; Hoshi, Masataka

1985-01-01

We used TLI as immunosuppression for BMT in a patient with aplastic anemia. He recieved high dose cyclophosphamide and single dose TLI with 750 cGy, 12 cGy/min at his midplane, and bone marrow from HLA-matched twin brother. He is surviving without complications at 15 months. This procedure is well tolerated regimen. (author)

Patients with severe acquired brain injury show increased arousal in tilt-table training

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Riberholt, Christian G; Thorlund, Jonas Bloch; Mehlsen, Jesper

2013-01-01

Patients with severe acquired brain injury (ABI) are often mobilised using a tilt-table. Complications such as orthostatic intolerance have been reported. The primary objective of this study was to investigate if using a tilt-table was feasible for mobilising patients with severe ABI admitted...... for sub-acute rehabilitation. We also investigated change in arousal, treatment duration before termination due to orthostatic reactions and change in muscle tone....

Iron chelation therapy with deferasirox in patients with aplastic anemia: a subgroup analysis of 116 patients from the EPIC trial

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Lee, Jong Wook; Yoon, Sung-Soo; Shen, Zhi Xiang

2010-01-01

The prospective 1-year Evaluation of Patients' Iron Chelation with Exjade (EPIC) study enrolled a large cohort of 116 patients with aplastic anemia; the present analyses evaluated the efficacy and safety of deferasirox in this patient population. After 1 year, median serum ferritin decreased...... neutrophil and platelet counts remained stable during treatment, and there were no drug-related cytopenias. This prospective dataset confirms the efficacy and well characterizes the tolerability profile of deferasirox in a large population of patients with aplastic anemia. This study was registered at www...

Short-term therapeutic effects of combined therapy with metformin hydrochloride for aplastic anemia

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Xue-chun LU

2012-03-01

Full Text Available Objective　To screen and select new drugs for aplastic anemia (AA and evaluate their clinical efficacy by clinical bioinformatics methods. Methods　First, we established genome expression profiles of AA patients, and conducted similarity analyses with the pharmacogenomics database to screen and select drugs with possible efficacy. Intractable AA patients who received immunosuppressors and/or androgen for more than six months showing no clinical efficacy were enrolled in the study to evaluate therapeutic effects of the therapeutic regime. Clinical efficacy and adverse effects were evaluated after six months. Results　The clinical bioinformatics results showed therapeutic effects of metformin hydrochloride on AA. Forty-three intractable AA patients (15 with severe AA were treated with metformin hydrochloride combined with cyclosporin A (CsA and stanozolol. Twenty-seven transfusion-dependent patients (100% became transfusion independent after a 6-month therapy. The hemoglobin level completely returned to normal in 37 out of 40 anemia patients (92.5%. In the 40 patients with platelet count lower than 20×109/L, the platelet count of 28 patients (90.3% increased to higher than 50×109/L. The white cell count increased to higher than 3.5×109/L in 30 out of 35 patients (88.6% with white cell count lower than 2.5×109/L. Among 40 anemic patients, 1 was found to have abnormal renal function, but it recovered to the normal range after ending CsA treatment. Eighteen patients were found to have elevated transaminase levels which were lowered to normal range after using liver protectants and reducing the dosage of stanozolol. There were no instances of hypoglycemia in all patients throughout the treatment. Conclusion　Combination of metformin hydrochloride, CsA and stanozolol is effective in refractory aplastic anemia with acceptable toxicity.

Haematological disorders in Thorotrast-administered patients in Japan

International Nuclear Information System (INIS)

Kamiyama, Ryuichi; Hatakeyama, Shigeru

1989-01-01

Fifteen haematological disorders including ten leukaemia cases, one primary acquired sideroblastic anaemia and four aplastic anaemia cases were studied clinicopathologically in autopsies from patients who had been administered Thorotrast in Japan. The leukaemia group, the primary acquired sideroblastic anaemia and the aplastic anaemia cases after Thorotrast administration were considered to be mainly atypical, and it was speculated that damage induced by Thorotrast may affect the haemopoietic stem cell level and the haemopoietic microenvironment. Both dose rate and absorbed dose estimated in bone marrow, spleen and liver at autopsy showed no significant difference between the leukaemia group, primary acquired sideroblastic anaemia, aplastic anaemia and non-haematological disorders excluding the malignant hepatic tumours and liver cirrhosis. (author)

Disease: H01132 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available H01132 Aplastic anemia (AA) Aplastic anemia (AA) is a rare disease in which the re...duction of the circulating blood cells results from damage to the stem cell pool in bone marrow. Most cases of acquired aplastic anem...ia are the consequence of an immune-mediated destruction of hematopoiesis. Autoreac

Hematopoietic stem cell transplantation in children and young adults with secondary myelodysplastic syndrome and acute myelogenous leukemia after aplastic anemia.

Science.gov (United States)

Yoshimi, Ayami; Strahm, Brigitte; Baumann, Irith; Furlan, Ingrid; Schwarz, Stephan; Teigler-Schlegel, Andrea; Walther, Joachim-Ulrich; Schlegelberger, Brigitte; Göhring, Gudrun; Nöllke, Peter; Führer, Monika; Niemeyer, Charlotte M

2014-03-01

Secondary myelodysplastic syndrome and acute myelogenous leukemia (sMDS/sAML) are the most serious secondary events occurring after immunosuppressive therapy in patients with aplastic anemia. Here we evaluate the outcome of hematopoietic stem cell transplantation (HSCT) in 17 children and young adults with sMDS/sAML after childhood aplastic anemia. The median interval between the diagnosis of aplastic anemia and the development of sMDS/sAML was 2.9 years (range, 1.2 to 13.0 years). At a median age of 13.1 years (range, 4.4 to 26.7 years), patients underwent HSCT with bone marrow (n = 6) or peripheral blood stem cell (n = 11) grafts from HLA-matched sibling donors (n = 2), mismatched family donors (n = 2), or unrelated donors (n = 13). Monosomy 7 was detected in 13 patients. The preparative regimen consisted of busulfan, cyclophosphamide, and melphalan in 11 patients and other agents in 6 patients. All patients achieved neutrophil engraftment. The cumulative incidence of grade II-IV acute graft-versus-host disease (GVHD) was 47%, and that of chronic GVHD was 70%. Relapse occurred in 1 patient. The major cause of death was transplant-related complication (n = 9). Overall survival and event-free survival at 5 years after HSCT were both 41%. In summary, this study indicates that HSCT is a curative therapy for some patients with sMDS/sAML after aplastic anemia. Future efforts should focus on reducing transplantation-related mortality. Copyright © 2014 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

Perceived difficulty in use of everyday technology in persons with acquired brain injury of different severity: a comparison with controls.

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Fallahpour, Mandana; Kottorp, Anders; Nygård, Louise; Lund, Maria Larsson

2014-07-01

To compare the perceived difficulty in use of everyday technology in persons with acquired brain injury with different levels of severity of disability with that of controls. This comparison study recruited 2 samples of persons with acquired brain injury and controls, comprising a total of 161 participants, age range 18-64 years. The long and short versions of the Everyday Technology Use Questionnaire and the Extended Glasgow Outcome Scale were used to evaluate participants. Persons with acquired brain injury demonstrated lower mean levels of perceived ability in use of everyday technology than controls (F = 21.84, degrees of freedom = 1, p technology between persons with severe disability and good recovery, between persons with severe disability and controls, and between persons with moderate disability and controls. No significant mean difference was found between persons with severe disability and moderate disability, between persons with moderate disability and good recovery, and between persons with good recovery and controls. Perceived difficulty in using everyday technology is significantly increased among persons with acquired brain injury with severe to moderate disability compared with controls. Rehabilitation services should consider the use of everyday technology in order to increase participation in everyday activities after acquired brain injury.

Double whammy- acute splenic sequestration crisis in patient with aplastic crisis due to acute parvovirus infection.

Science.gov (United States)

Minhas, Parminder S; K Virdi, Jaspreet; Patel, Rajeshkumar

2017-07-01

Splenic dysfunction is a major feature of sickle cell disease (SCD) and can manifest as acute splenic sequestration crisis (ASSC), which is the earliest life-threatening complication seen in patients with SCD. Aplastic crisis is another potentially deadly complication of sickle cell disease that develops when erythrocyte production temporarily drops. Infection with parvovirus B-19 frequently causes aplastic crises. These two complications are known to be mutually exclusive due to their classic presentation signs and symptoms but there have been few cases where a patient can have concomitant presentation of both phenomena, which can result in a fatal outcome. These few cases force us to rethink the etiology and subsequent management guidelines of these complications. We present to you a case of an unfortunate 23-year-old female who had both complications occurring at the same time, resulting in death.

Evaluation of renal uptake on 111InCl3 bone marrow scintigraphy in patients with aplastic anemia and myelodysplastic syndrome

International Nuclear Information System (INIS)

Koizumi, Mitsuru; Goto, Masafumi; Nomura, Toshiharu; Watari, Tsutomu; Saito, Kenji

1993-01-01

High renal uptake on bone marrow scan with indium-111 chloride is often shown in patients with bone marrow abnormality. We evaluated the renal uptake on bone marrow scan in 27 cases of aplastic anemia, 20 cases of myelodysplastic syndrome (MDS) and 10 cases of other diseases. The high renal uptake was observed in patients not only with aplastic anemia but also with MDS. The renal uptake correlated with blood transfusion units, unsaturated iron binding capacity (UIBC), blood pool imaging and bone marrow uptake. The renal uptake correlated with UIBC better than with the blood transfusion units. Following mechanism of the renal uptake is proposed that frequent blood transfusion makes low UIBC, and the low UIBC causes the failure to chelate indium with transferrin. The fast blood clearance of un-chelated indium via kidneys is followed. Hypoplastic bone marrow may also play an important role for the high renal uptake because all patients with the high renal uptake could not be explained by above mentioned mechanism. Caution should be paid to the scans with the high renal uptake because both aplastic anemia and MDS patients show the high renal uptake. (author)

Marrow uptake index (MUI): A quantitative scintigraphic study of bone marrow in aplastic anaemia

International Nuclear Information System (INIS)

Padhy, A.K.; Garg, A.; Kochupillai, V.; Gopinath, P.G.; Basu, A.K.

1987-01-01

Aplastic anaemia affects the entire bone marrow. This prospective study was undertaken to develop and standardise a new nuclear medicine technique called 'dynamic bone marrow imaging'. Eleven patients and ten controls were studied. Serial images of the pelvis were obtained in frame mode following intravenous injection of 185-370 mBq of 99m Tc S. Colloid, and an index, called the bone marrow uptake index was calculated by taking into consideration the time activity curve obtained over the iliac crest. This was followed by static imaging of the entire bone marrow in all cases. It was possible to obtain excellent information regarding topographic distribution of bone marrow as well as detect early changes in bone marrow function following treatment. An attempt was also made to correlate bone marrow cellularity as obtained by bone marrow biopsy with results of dynamic bone marrow scintigraphy. On the basis of the encouraging results obtained in the present study, the authors feel that dynamic bone marrow imaging is an excellent technique for the objective evaluation of bone marrow in aplastic anaemia. 20 refs.; 4 figs.; 5 tabs

Treatment of acquired arteriovenous fistula with severe hemodynamic effects: therapeutic challenge

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Bruna Ferreira Pilan

2014-03-01

Full Text Available A 34-year-old female patient with severe heart failure and pulmonary hypertension was diagnosed late with a high-output acquired arteriovenous fistula between the right common iliac vein and artery. The most probable cause was an iatrogenic vascular injury inflicted during a prior laparoscopic cholecystectomy. Treatment was conducted by placement of an endoprosthesis in the common iliac artery, achieving total exclusion of the fistula and complete remission of symptoms. Considering the options available for treating this type of lesion, endovascular techniques are becoming ever more effective and are now the option of first-choice for management of this pathology.

Characteristic focal hot spots of bone marrow scintigraphic finding in aplastic anemia

International Nuclear Information System (INIS)

Liu Yong

1991-01-01

The bone marrow scintigraphy with 99m Tc sulfur colloid has been performed in 168 patients with Aplastic anemia(AA) and 100 patients with others hematological disorders. Bone marrow imaging is a useful method to demonstrate the existence of active hematopoietic foci in living body. The features and clinical significance of these focal hot spots have been discussed. The bone marrow scintigraphy is proved to be helpful in diagnosis, therapy and assessing prognosis of A.A

Short- and long-term mortality in patients with community-acquired severe sepsis and septic shock

DEFF Research Database (Denmark)

Storgaard, Merete; Hallas, Jesper; Gahrn-Hansen, Bente

2013-01-01

Background: Severe sepsis and septic shock have a high 30-day mortality (10-50%), but the long-term mortality is not well described. The purpose of this study was to describe long-term mortality among patients with community-acquired severe sepsis or septic shock compared to a population-based re......Background: Severe sepsis and septic shock have a high 30-day mortality (10-50%), but the long-term mortality is not well described. The purpose of this study was to describe long-term mortality among patients with community-acquired severe sepsis or septic shock compared to a population...... extracted from the National Danish Patient Registry. We analyzed the hazard ratio for mortality at predefined intervals. Results: Absolute mortality within the first 30 days was 69/211 (33%, 95% confidence interval (CI) 25-41%), with a cumulative mortality of 121/211 (57%, 95% CI 48-69%) for the entire...... follow-up. Among septic patients who survived the first 30 days, the mortality hazard ratio was 2.7 (95% CI 1.7-4.3) until day 365, and among septic patients who survived the first year, the 1-4 y mortality hazard ratio was 2.3 (95% CI 1.7-3.3), compared to the community-based reference persons...

T cell costimulation blockade promotes transplantation tolerance in combination with sirolimus and post-transplantation cyclophosphamide for haploidentical transplantation in children with severe aplastic anemia.

Science.gov (United States)

Jaiswal, Sarita Rani; Bhakuni, Prakash; Zaman, Shamsuz; Bansal, Satish; Bharadwaj, Priyanka; Bhargava, Sneh; Chakrabarti, Suparno

2017-08-01

We conducted a pilot study employing extended T cell costimulation blockade (COSBL) with Abatacept along with sirolimus and post-transplantation cyclophosphamide (PTCy) in 10 patients (median age 12) with severe aplastic anemia (SAA). Nine patients engrafted in the COSBL group, compared to all 10 patients (median 14 vs 13days) treated on PTCy protocols without abatacept (CONTROL group). The incidence of acute graft-versus-host disease (GVHD) was 10.5% in the COSBL group compared to 50% in the CONTROL group (p=0.04). Chronic GVHD (12.5% vs 56%, p=0.02) and CMV reactivation (30% vs 80%, p=0.03) were also reduced in the COSBL group. T and NK cell subset analysis revealed higher CD56 bright CD16 - NK cells in the CONTROL group (p=0.004), but similar CD56 dim CD16 + NK cells in both groups at day+30. Tregs (CD4 + CD25 + CD127 dim/- FoxP3+) were markedly higher in the COSBL group at day+30 (8.4% vs 1.1%) and the trend was maintained through day+90 (p<0.01). The GVHD and Disease-free survival at one year in the COSBL group was 80% vs. 30% in the CONTROL group (p=0.05). Our preliminary findings suggest that COSBL in combination with PTCy and sirolimus might augment transplantation tolerance in children with SAA, probably due to synergistic effect on early recovery of Tregs. Copyright © 2017 Elsevier B.V. All rights reserved.

Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.

Science.gov (United States)

Hama, Asahito; Takahashi, Yoshiyuki; Muramatsu, Hideki; Ito, Masafumi; Narita, Atsushi; Kosaka, Yoshiyuki; Tsuchida, Masahiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

2015-11-01

The 2008 World Health Organization classification proposed a new entity in childhood myelodysplastic syndrome, refractory cytopenia of childhood. However, it is unclear whether this morphological classification reflects clinical outcomes. We retrospectively reviewed bone marrow morphology in 186 children (median age 8 years; range 1-16 years) who were enrolled in the prospective study and received horse antithymocyte globulin and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range 1-146 months). Out of 186 patients, 62 (33%) were classified with aplastic anemia, 94 (49%) with refractory cytopenia of childhood, and 34 (18%) with refractory cytopenia with multilineage dysplasia. Aplastic anemia patients received granulocyte colony-stimulating factor more frequently and for longer durations than other patients (Paplastic anemia, 4 patients with refractory cytopenia of childhood, and 3 patients with refractory cytopenia with multilineage dysplasia. Although the cumulative incidence of total clonal evolution at ten years was not significantly different among the 3 groups, the cumulative incidence of monosomy 7 development was significantly higher in aplastic anemia than in the other groups (P=0.02). Multivariate analysis revealed that only granulocyte colony-stimulating factor administration duration of 40 days or more was a significant risk factor for monosomy 7 development (P=0.02). These findings suggest that even the introduction of a strict morphological distinction from hypoplastic myelodysplastic syndrome cannot eradicate clonal evolution in children with aplastic anemia. Copyright© Ferrata Storti Foundation.

Evaluation of renal uptake on [sup 111]InCl[sub 3] bone marrow scintigraphy in patients with aplastic anemia and myelodysplastic syndrome

Energy Technology Data Exchange (ETDEWEB)

Koizumi, Mitsuru; Goto, Masafumi; Nomura, Toshiharu; Watari, Tsutomu; Saito, Kenji (Dokkyo Univ. School of Medicine, Mibu, Tochigi (Japan))

1993-04-01

High renal uptake on bone marrow scan with indium-111 chloride is often shown in patients with bone marrow abnormality. We evaluated the renal uptake on bone marrow scan in 27 cases of aplastic anemia, 20 cases of myelodysplastic syndrome (MDS) and 10 cases of other diseases. The high renal uptake was observed in patients not only with aplastic anemia but also with MDS. The renal uptake correlated with blood transfusion units, unsaturated iron binding capacity (UIBC), blood pool imaging and bone marrow uptake. The renal uptake correlated with UIBC better than with the blood transfusion units. Following mechanism of the renal uptake is proposed that frequent blood transfusion makes low UIBC, and the low UIBC causes the failure to chelate indium with transferrin. The fast blood clearance of un-chelated indium via kidneys is followed. Hypoplastic bone marrow may also play an important role for the high renal uptake because all patients with the high renal uptake could not be explained by above mentioned mechanism. Caution should be paid to the scans with the high renal uptake because both aplastic anemia and MDS patients show the high renal uptake. (author).

Transient engraftment of syngeneic bone marrow after conditioning with high-dose cyclophosphamide and thoracoabdominal irradiation in a patient with aplastic anemia

International Nuclear Information System (INIS)

Matsue, K.; Niki, T.; Shiobara, S.; Ueda, M.; Ohtake, S.; Mori, T.; Matsuda, T.; Harada, M.

1990-01-01

We describe the clinical course of a 16 year old girl with aplastic anemia who was treated by syngeneic bone marrow transplantation. Engraftment was not obtained by simple infusion of bone marrow without immunosuppression. The patient received a high-dose cyclophosphamide and thoracoabdominal irradiation, followed by second marrow transplantation from the same donor. Incomplete but significant hematologic recovery was observed; however, marrow failure recurred 5 months after transplantation. Since donor and recipient pairs were genotypically identical, graft failure could not be attributed to immunological reactivity of recipient cells to donor non-HLA antigens. This case report implies that graft failure in some cases of aplastic anemia might be mediated by inhibitory cells resistant to cyclophosphamide and irradiation

Early rehabilitation and participation in focus - a Danish perspective on patients with severe acquired brain injury

DEFF Research Database (Denmark)

Smidt, Helle Rønn; Pallesen, Hanne; Buhl, Inge

2016-01-01

Early neurorehabilitation is an interdisciplinary field. Thus, in order to eliminate unnecessary barriers for individuals with severe acquired brain injury in early rehabilitation, we need rehabilitation science that supports both quantitative and qualitative research methods. Participation can b...

Does C-reactive protein independently predict mortality in adult community-acquired bacteremia patients with known sepsis severity?

DEFF Research Database (Denmark)

Gradel, Kim O; Jensen, Thøger G; Kolmos, Hans J

2013-01-01

We evaluated whether sepsis severity and C-reactive protein (CRP) level on admission prognostically corroborated or annulled each other in adult patients with incident community-acquired bacteremia (Funen, Denmark, 2000-2008). We used logistic regression and area under the receiver operating.......06), thus CRP contributed as much as sepsis severity to prognosis....

A Study of Emotionalism in Patients Undergoing Rehabilitation following Severe Acquired Brain Injury

Directory of Open Access Journals (Sweden)

Joanna McGrath

2000-01-01

Full Text Available The present study describes the phenomenon of emotionalism in a sample of brain injured patients of mixed aetiology, with a view to identifying issues relevant to clinical management, and possible causal factors. 82 subjects with severe acquired brain injury undergoing rehabilitation participated in a structured interview in which they were asked to report the presence/absence of emotionalism and degree of distress associated with it. Their overt crying behaviour was also observed and recorded. Independent variables that predicted crying during the interview were identified using a multiple logistic regression procedure. Prevalence rates of emotionalism-tearfulness were high in this sample (52% self-report, 36–41% Emotionalism-laughter was much less common (13% Emotionalism-tearfulness was usually accompanied by negative affect, occurred in response to identifiable precipitants, and was often controllable. It was associated with major personal distress in about half the subjects who reported it. Independent variables which predicted crying behaviour were female gender and focal damage to the right cerebral hemisphere. It is concluded that an increased readiness to cry is common in people with severe acquired brain injury of mixed aetiology. The behaviour is meaningful, though not always distressing. The intensity of the behaviour is variable, and it may be most appropriate to regard emotionalism as a dimension rather than a syndrome. Implications for clinical management are discussed.

B-cell-rich T-cell lymphoma associated with Epstein-Barr virus-reactivation and T-cell suppression following antithymocyte globulin therapy in a patient with severe aplastic anemia

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Nobuyoshi Hanaoka

2015-09-01

Full Text Available B-cell lymphoproliferative disorder (B-LPD is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

Life goals and social identity in people with severe acquired brain injury: an interpretative phenomenological analysis.

Science.gov (United States)

Martin, Rachelle; Levack, William M M; Sinnott, K Anne

2015-01-01

While there is a growing body of literature exploring life goals in rehabilitation, little research has been undertaken that includes the voice of the end-user. This study examined the views and experiences of people with severe acquired brain injury regarding the place of "life goals" in residential rehabilitation. Interpretative phenomenological analysis was used to collect and analyze data from five semi-structured interviews with participants in a residential rehabilitation setting. Three inter-related themes emerged from this study. Social connectedness (being 'part of things') emerged as a life goal of central importance for all participants (Theme 1). However, in order to achieve this sense of belonging, the participants needed to tentatively balance the opportunities arising within their environmental milieu (Theme 2) with the interpersonal factors relating to their unchanged, changed and changing self-identity (Theme 3). This study suggests that social identity and social connectedness ought to be primary foci of rehabilitation rather than matters only of secondary concern. Consideration needs to be given to both the environmental contexts and the intrapersonal strategies that support people who require residential rehabilitation services to achieve social connection, and thus their life goals, following a severe acquired brain injury. Implications for Rehabilitation There is a need to better support people with severe acquired brain injury (ABI) in terms of their social relationships and social identity during the delivery of person-centered rehabilitation services. Within the clinical setting there should be regular, in depth and open dialogue in which the individuals' values and preferences are discovered. A focus on the coherence between daily activities and the person's life goals is required for people with severe ABI. Clinicians need to consider how life goals for individual people change or are re-prioritized over the life span.

Severity assessment scores to guide empirical use of antibiotics in community acquired pneumonia.

Science.gov (United States)

Singanayagam, Aran; Chalmers, James D

2013-10-01

Severity assessment scores were first developed to predict the 30 day mortality in community acquired pneumonia; however, several guidelines have extended their use to guide empirical antibiotic prescription decisions. This approach has theoretical advantages because a decrease in broad-spectrum antibiotic treatment in low-risk patients might reduce antibiotic-related side-effects, and to give broad-spectrum therapy to patients at higher risk of death is intuitive. However, evidence in support of this approach is not clear. In particular, the British Thoracic Society guidelines suggest withholding a macrolide from patients with low CURB 65 scores, despite evidence that these patients have a higher frequency of atypical pathogens than do those with a higher severity of pneumonia. Severity scores do not perform well in some groups and might overestimate disease severity in elderly people, leading to inappropriate broad-spectrum treatment to those at high risk of complications such as Clostridium difficile infection. In this Review, we discuss the evidence for antibiotic prescribing guided by severity score and suggest that more evidence of effect and implementation is needed before this approach can be universally adopted. Copyright © 2013 Elsevier Ltd. All rights reserved.

Human parvovirus B19-induced aplastic crisis in an adult patient with hereditary spherocytosis: a case report and review of the literature.

Science.gov (United States)

Kobayashi, Yujin; Hatta, Yoshihiro; Ishiwatari, Yusaku; Kanno, Hitoshi; Takei, Masami

2014-03-11

Although there are several case reports of human parvovirus B19 infection in patients with hereditary spherocytosis, no systematic reviews of adult patients with hereditary spherocytosis with human parvovirus B19 infection have been published as clinical case reports. In this study, we report a case of aplastic crisis due to human parvovirus B19 infection in an adult patient with hereditary spherocytosis. A 33-year-old woman with hereditary spherocytosis and gallstones was admitted because of rapid progress in marked anemia and fever. Although empiric antibiotic therapy was prescribed, her clinical symptoms and liver function test worsened. Because the anti-human parvovirus B19 antibody and deoxyribonucleic acid levels assessed by polymerase chain reaction were positive, the patient was diagnosed with aplastic crisis due to the human parvovirus B19 infection. We collected and reviewed several case reports of patients with hereditary spherocytosis aged > 18 years with human parvovirus B19 infection between 1984 and 2010. A total of 19 reports with 22 cases [median age, 28 years (range, 18-43 range); male: female ratio, 6:16], including the present case were identified. The male-to-female ratio of 6:16 implied that younger females were predominantly affected. Although fever and abdominal symptoms were common initial symptoms, liver dysfunction or skin eruptions were less commonly documented. Anti-human parvovirus B19 antibody or deoxyribonucleic acid levels assessed by polymerase chain reaction was commonly used to diagnose human parvovirus B19 infection and may be useful to distinguish human parvovirus B19 infection from other abdominal infection in patients with hereditary spherocytosis.

Causes of non-adherence to therapeutic guidelines in severe community-acquired pneumonia

Science.gov (United States)

Gattarello, Simone; Ramírez, Sergio; Almarales, José Rafael; Borgatta, Bárbara; Lagunes, Leonel; Encina, Belén; Rello, Jordi

2015-01-01

Objective To assess the adherence to Infectious Disease Society of America/American Thoracic Society guidelines and the causes of lack of adherence during empirical antibiotic prescription in severe pneumonia in Latin America. Methods A clinical questionnaire was submitted to 36 physicians from Latin America; they were asked to indicate the empirical treatment in two fictitious cases of severe respiratory infection: community-acquired pneumonia and nosocomial pneumonia. Results In the case of communityacquired pneumonia, 11 prescriptions of 36 (30.6%) were compliant with international guidelines. The causes for non-compliant treatment were monotherapy (16.0%), the unnecessary prescription of broad-spectrum antibiotics (40.0%) and the use of non-recommended antibiotics (44.0%). In the case of nosocomial pneumonia, the rate of adherence to the Infectious Disease Society of America/American Thoracic Society guidelines was 2.8% (1 patient of 36). The reasons for lack of compliance were monotherapy (14.3%) and a lack of dual antibiotic coverage against Pseudomonas aeruginosa (85.7%). If monotherapy with an antipseudomonal antibiotic was considered adequate, the antibiotic treatment would be adequate in 100% of the total prescriptions. Conclusion The compliance rate with the Infectious Disease Society of America/American Thoracic Society guidelines in the community-acquired pneumonia scenario was 30.6%; the most frequent cause of lack of compliance was the indication of monotherapy. In the case of nosocomial pneumonia, the compliance rate with the guidelines was 2.8%, and the most important cause of non-adherence was lack of combined antipseudomonal therapy. If the use of monotherapy with an antipseudomonal antibiotic was considered the correct option, the treatment would be adequate in 100% of the prescriptions. PMID:25909312

MR imaging of hematopoietic regions in bone marrow of aplastic anemia. Diagnostic usefulness of opposed phase T1-weighted images

Energy Technology Data Exchange (ETDEWEB)

Amano, Yasuo; Tanabe, Yoshihiro; Amano, Maki; Kumazaki, Tatsuo [Nippon Medical School, Tokyo (Japan)

1996-01-01

The signal intensity of hematopoietic regions in the marrow of aplastic anemia were investigated on opposed phase T1-weighted images (op-T1WI) with a 0.5-Tesla MR unit. Hematopoietic regions were classified into two groups: low intensity hematopoietic areas (LH) isointense to normal marrow and high intensity hematopoietic regions (HH) with higher intensity than normal marrow on op-T1WI. The signal intensity of LH was significantly lower than that of HH on STIR. LH converted into HH with improvement of laboratory data after therapy, whereas HH decreased with impairment of data. HH were hyperintense to cerebrospinal fluid on op-T1WI. These results indicated that the signal intensity of hematopoietic regions on op-T1WI reflected the cellularity in these regions and that aplastic anemia included hypercellular regions relative to normal marrow. (author).

Anemia aplásica adquirida e anemia de Fanconi - Diretrizes Brasileiras em Transplante de Células-Tronco Hematopoéticas Acquired aplastic anemia and Fanconi anemia - Brazilian Guidelines in Hematopoietic Stem Cell Transplantation

Directory of Open Access Journals (Sweden)

Larissa A. Medeiros

2010-05-01

Full Text Available As diretrizes apresentadas neste trabalho foram elaboradas e aprovadas na I Reunião de Diretrizes Brasileiras em Transplante de Células-Tronco Hematopoéticas (TCTH realizada no Rio de Janeiro, entre os dias 19 e 21 de julho de 2009. O evento foi promovido pela SBTMO (Sociedade Brasileira de Transplante de Medula Óssea. Neste artigo, tratamos da anemia aplásica severa (AAS, considerada uma urgência hematológica, que, identificada e manejada de forma precoce, apresenta grande possibilidade de recuperação da hematopoese seja através de transplante de medula óssea ou terapia imunossupressora. Objetiva-se nortear o manejo terapêutico no contexto do transplante e indicar formas de condicionamento, de acordo com as características clínicas dos pacientes, como o número de transfusões, com intuito de minimizar a rejeição primária e secundária, garantindo a melhora da sobrevida global e livre de doença (observadas pela literatura e já validadas por resultados na população brasileira. No que concerne à anemia de Fanconi, o transplante é a única modalidade curativa para o componente aplásico de medula óssea; embora não modificando as outras características da síndrome também demanda perícia e agilidade na busca de um doador com resultados expressivos de sobrevida.The guidelines presented in this article have been prepared and approved in the I Meeting of Brazilian Guidelines in Hematopoietic Stem Cell Transplantation (HSCT - Rio de Janeiro, July 19-21, 2009. The event was sponsored by SBTMO (Brazilian Society of Bone Marrow Transplantation. In this paper, we treat the severe aplastic anemia (SAA, considered a hematological emergency, that when identified and medically treated early, shows a great chance of recovery of the hematopoiesis, either through bone marrow transplantation or immunosuppressive therapy. Its objective is to guide the management of the transplantation, and indicate methods of conditioning, according to

Immunosuppression prior to marrow transplantation for sensitized aplastic anemia patients: comparison of TLI with TBI

International Nuclear Information System (INIS)

Shank, B.; Brochstein, J.A.; Castro-Malaspina, H.; Yahalom, J.; Bonfiglio, P.; O'Reilly, R.J.

1988-01-01

From May 1980 through July 1986, 26 patients with severe aplastic anemia, sensitized with multiple transfusions of blood products, were treated on either of two immunosuppressive regimens in preparation for bone marrow transplantation from a matched donor. There were 10 patients treated with total body irradiation (TBI), 200 cGy/fraction X 4 daily fractions (800 cGy total dose), followed by cyclophosphamide, 60 mg/kg/d X 2 d. An additional 16 patients were treated with total lymphoid irradiation (TLI) [or, if they were infants, a modified TLI or thoracoabdominal irradiation (TAI)], 100 cGy/fraction, 3 fractions/d X 2 d (600 cGy total dose), followed by cyclophosphamide, 40 mg/kg/d X 4 d. The extent of immunosuppression was similar in both groups as measured by peripheral blood lymphocyte depression at the completion of the course of irradiation (5% of initial concentration for TBI and 24% for TLI), neutrophil engraftment (10/10 for TBI and 15/16 for TLI), and time to neutrophil engraftment (median of 22 d for TBI and 17 d for TLI). Marrow and peripheral blood cytogenetic analysis for assessment of percent donor cells was also compared in those patients in whom it was available. 2/2 patients studied with TBI had 100% donor cells, whereas 6/11 with TLI had 100% donor cells. Of the five who did not, three were stable mixed chimeras with greater than or equal to 70% donor cells, one became a mixed chimera with about 50% donor cells, but became aplastic again after Cyclosporine A cessation 5 mo post-transplant, and the fifth reverted to all host cells by d. 18 post-transplant. Overall actuarial survival at 2 years was 56% in the TLI group compared with 30% in the TBI group although this was not statistically significant. No survival decrement has been seen after 2 years in either group

Social communication features in children following moderate to severe acquired brain injury: a cross-sectional pilot study.

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Breau, Lynn M; Clark, Brenda; Scott, Ori; Wilkes, Courtney; Reynolds, Shawn; Ricci, Florencia; Sonnenberg, Lyn; Zwaigenbaum, Lonnie; Rashid, Marghalara; Goez, Helly R

2015-04-01

We compared the social communication deficits of children with moderate to severe acquired brain injury or autism spectrum disorder, while accounting for the role of attention-deficit hyperactivity disorder (ADHD) symptoms. Parents of 20 children aged 6 to 10 years (10 acquired brain injury; 10 autism spectrum disorder) completed the Social Communication Questionnaire, and Conners 3 Parent Short. A multivariate analysis of covariance revealed significant differences between groups in Social Communication Questionnaire restricted repetitive behavior scores, but not reciprocal social interaction or social communication. Multiple linear regressions indicated diagnosis did not predict reciprocal social interaction or social communication scores and that Conners 3 Parent Short Form hyperactivity scores were the strongest predictor of Social Communication Questionnaire reciprocal social interaction scores after accounting for age and Intelligence Quotient. The lack of difference in social communication deficits between groups may help in understanding the pathophysiology underlying the behavioral consequences of acquired brain injury. The link between hyperactivity and reciprocal interaction suggests that targeting hyperactivity may improve social outcomes in children following acquired brain injury. © The Author(s) 2014.

Case of subcutaneous abscess caused by Nocardia farcinica in an aplastic anemia patient.

Science.gov (United States)

Yokota, Sho; Kawabe, Keitaro; Yamada, Hideki; Nunomura, Maki

2010-01-01

We report a case of subcutaneous abscess caused by Nocardia farcinica in a 44-year-old man, who had been treated with systemic prednisolone and cyclosporin for aplastic anemia. He had been affected by aplastic anemia for 8 years, and was previously treated with antithymocyte globulin. The effect was insufficient, and platelet and erythrocyte transfusion was required. Bone marrow transplantation was not adopted due to a psychological problem. He had also been treated with prednisolone and cyclosporin for 3 years. Without apparent cause, swelling and pain of left upper extremity developed in April, 2008. There was no abnormality in cutaneous macroscopic findings, such as a wound or a sting. He was administered antibiotics, but they were ineffective and effusion were excreted into the skin. After hospitalization, he was treated by incision and drainage and antibiotics were started. The pathogen was identified as Nocardia farcinica by its biochemical characters. There was no dissemination to other organs like lung or brain and he recovered completely with a treatment of antibiotics for 1 year. We studied the epidemiological and clinical characteristics of Nocardia infections reported in Japan from 2000 to 2008 and identified 92 cases using the medical article search engine Ichushi-Web (Japan Medical Abstract Society). The results indicate that the most important risk factor in systemic nocardiosis ia an immunosuppressive agent such as prednisolone, cyclosporine, or azathioprine. We believe that we should take the possibility of Nocardia infection into consideration in a compromised host.

Quantitative and qualitative assessment of reactive hematopoietic bone marrow in aplastic anemia using MR spectroscopy with variable echo times

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Amano, Yasuo; Kumazaki, Tatsuo [Department of Radiology, Nippon Medical School, Tokyo (Japan)

2002-01-01

Objective: To assess quantitative and qualitative differences in water components between normal bone marrow and reactive hematopoietic marrow in aplastic anemia using magnetic resonance (MR) spectroscopy with variable echo times (TEs). Design: Water content, T2 value of the water component, and signal change in water related to TE were assessed in normal bone marrow and reactive hematopoietic bone marrow by a stimulated echo acquisition mode with TEs of 30, 45, 60, and 90 ms. Patients: Six patients with aplastic anemia (13-84 years) and seven normal volunteers (25-38 years) were examined. Results and conclusion: Reactive hematopoietic marrow showed significantly higher water content than normal bone marrow. The T2 value of water components tended to be longer in reactive hematopoietic marrow. Water signal ratio related to TE was significantly higher in reactive hematopoietic marrow. These results suggest a quantitative and qualitative difference in water components between normal and reactive hematopoietic bone marrow. (orig.)

Pregnancy shortly after an acute episode of severe acquired thrombotic thrombocytopenic purpura.

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Panaitescu, Anca M; Stoia, Razvan; Ciobanu, Anca M; Demetrian, Mihaela; Peltecu, Gheorghe

2016-12-01

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal condition. In women with a previous history of TTP there is increased risk of recurrence during pregnancy and the puerperium. There is some evidence that the risk of relapse during pregnancy is increased if the interval between the event and conception is short. We present a case in which pregnancy was achieved a few days after full recovery from an acute episode of severe acquired TTP (ADAMTS13 activity <0.1%) which was successfully treated with four courses of plasma exchange. There was no relapse of TTP during pregnancy and a healthy baby was delivered at term; the puerperium was uneventful. Copyright © 2016 Elsevier Ltd. All rights reserved.

Outcome of children with severe acquired aplastic anemia treated with rabbit antithymocyte globulin and cyclosporine A

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Marlene Pereira Garanito

2014-09-01

Conclusions: The present results confirm the poor response rate with rabbit antithymocyte globulin as first therapy in pediatrics patients, similar to what has been reported for patients of all ages. This confirmation is problematic in Brazil, given the lack of horse antithymocyte globulin in many markets outside the United States.

Acquired Factor Xiii Deficiency: An Uncommon But Easily Missed Cause Of Severe Bleeding

LENUS (Irish Health Repository)

Fogarty, H

2018-05-01

Factor XIII (FXIII) is a plasma clotting protein involved in clot stabilization. Severe FXIII deficiency may present with severe, even fatal bleeding. Critically however, routine coagulation assays may be normal and only specific FXIII assays will detect the abnormality. Herein we discuss a case report of a patient with acquired FXIII deficiency in order to highlight the clinical challenges associated with establishing the diagnosis and discuss the treatment approach. A 70-year-old man presented with a gluteal haematoma despite no preceding personal history of bleeding. Extensive initial haemostatic investigations were normal until a specific FXIII assay showed a marked reduction in FXIII levels. With directed treatment, bleeding episodes ceased and remission was achieved. Clinical awareness of FXIII deficiency is important, so appropriate testing can be implemented in patients with unexplained bleeding diatheses, particularly those in whom bleeding responds poorly to standard replacement therapy.

Severe Community-acquired Pneumonia Due to Legionella pneumophila Serogroup 6

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Chung-Yu Chen

2006-01-01

Full Text Available Legionella pneumophila is a common cause of sporadic community-acquired pneumonia, but culture-proven legionellosis is rarely diagnosed. There is no laboratory test for Legionnaires' disease that can detect all patients with the disease. Culture is the standard diagnostic method and should be initiated as soon as possible in suspected cases. We describe a rare case of community-acquired pneumonia caused by L. pneumophila serogroup 6. A 77-year-old man was admitted to a tertiary care hospital because of high fever, productive cough, and progressive dyspnea. Chest radiography showed bilateral pneumonia, which led to respiratory failure necessitating mechanical ventilatory support. Despite antibiotic therapy, his condition continued to deteriorate and acute renal failure also developed. Urine was negative for L. pneumophila. Culture of the sputum yielded L. pneumophila serogroup 6, although there was no elevation of the serum antibody titer. Pneumonia resolved gradually and he was extubated after treatment with levofloxacin followed by erythromycin. L. pneumophila other than serogroup 1 should be included in the differential diagnosis of patients with suspected atypical community-acquired pneumonia.

Nutritional support contributes to recuperation in a rat model of aplastic anemia by enhancing mitochondrial function.

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Yang, Guang; Zhao, Lifen; Liu, Bing; Shan, Yujia; Li, Yang; Zhou, Huimin; Jia, Li

2018-02-01

Acquired aplastic anemia (AA) is a hematopoietic stem cell disease that leads to hematopoietic disorder and peripheral blood pancytopenia. We investigated whether nutritional support is helpful to AA recovery. We established a rat model with AA. A nutrient mixture was administered to rats with AA through different dose gavage once per day for 55 d. Animals in this study were assigned to one of five groups: normal control (NC; group includes normal rats); AA (rats with AA); high dose (AA + nutritional mixture, 2266.95 mg/kg/d); medium dose (1511.3 mg/kg/d); and low dose (1057.91 mg/kg/d). The effects of nutrition administration on general status and mitochondrial function of rats with AA were evaluated. The nutrient mixture with which the rats were supplemented significantly improved weight, peripheral blood parameters, and histologic parameters of rats with AA in a dose-dependent manner. Furthermore, we observed that the number of mitochondria in the liver, spleen, kidney, and brain was increased after supplementation by transmission electron microscopy analysis. Nutrient administration also improved mitochondrial DNA content, adenosine triphosphate content, and membrane potential but inhibited oxidative stress, thus, repairing the mitochondrial dysfunction of the rats with AA. Taken together, nutrition supplements may contribute to the improvement of mitochondrial function and play an important role in the recuperation of rats with AA. Copyright © 2017 Elsevier Inc. All rights reserved.

Repair of UV-induced DNA damage in aplastic anaemia: Changes after treatment with antilymphocyte globulin (ALG)

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Kovacs, E.; Nissen, C.; Speck, B.; Signer, E.

1988-01-01

The extent of DNA-repair induced by UV-C irradiation was measured in peripheral unstimulated lymphocytes of 24 patients with aplastic anaemia at different stages of disease and compared with the results obtained in 92 controls. As parameter of the DNA-repair synthesis, the incorporation of (/sup 3/H)thymidine in the presence of 2 mmol/l hydroxyurea (HU) was taken. Of 19 patients tested after treatment with antilymphocyte globulin (ALG), 5 were in complete autologous haemopoietic remission, defined as > 1000 granulocytes/mm/sup 3/, > 100 000 platelets/mm/sup 3/ and a nontransfused haemoglobin value > 10 g%. 14 patients were in partial remission, defined as improvement of haemopoietic function, not meeting the criteria for complete remission. 4/5 patients in complete remission had normal DNA-repair synthesis, compared to 4/14 patients in partial remission. In 92 controls, a normal level was found in 70 cases. In 4/5 patients examined at diagnosis and at various intervals after ALG-treatment, DNA-repair synthesis was low at diagnosis. It increased after therapy and paralleled improvement of haemopoietic function to some extent. It is suggested that in aplastic anaemia there are different populations of lymphocytes with differing DNA-repair capacity; ALG treatment seems to favour expansion of the normal population, which is associated with improvement of haemopoietic function.

Remission of aplastic anemia induced by treatment for Graves disease in a pediatric patient.

Science.gov (United States)

Das, Prabodh Kumar; Wherrett, Diane; Dror, Yigal

2007-08-01

Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and mostly idiopathic in children. We describe a child, who developed AA following Graves' disease which could not be attributed to antithyroid drugs. We hypothesized that both diseases were caused by similar autoimmune process. We monitored the blood counts and did not administer any conventional treatment for AA assuming that the existing anti- hematopoietic stem cell humoral and cellular immunity might subside with induction of remission of Grave's disease. The child went into complete remission with the treatment of the Graves' disease.

Identification of novel regulators in T-cell differentiation of aplastic anemia patients

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Probst-Kepper Michael

2006-10-01

Full Text Available Abstract Background Aplastic anemia (AA is a bone marrow failure syndrome mostly characterized by an immune-mediated destruction of marrow hematopoietic progenitor/stem cells. The resulting hypocellularity limits a detailed analysis of the cellular immune response. To overcome this technical problem we performed a microarray analysis of CD3+ T-cells derived from bone marrow aspirates and peripheral blood samples of newly diagnosed AA patients and healthy volunteers. Two AA patients were additionally analyzed after achieving a partial remission following immunosuppression. The regulation of selected candidate genes was confirmed by real-time RT-PCR. Results Among more than 22.200 transcripts, 583 genes were differentially expressed in the bone marrow of AA patients compared to healthy controls. Dysregulated genes are involved in T-cell mediated cytotoxicity, immune response of Th1 differentiated T-cells, and major regulators of immune function. In hematological remission the expression levels of several candidate genes tend to normalize, such as immune regulators and genes involved in proinflammatory immune response. Conclusion Our study suggests a pivotal role of Th1/Tc1 differentiated T-cells in immune-mediated marrow destruction of AA patients. Most importantly, immune regulatory genes could be identified, which are likely involved in the recovery of hematopoiesis and may help to design new therapeutic strategies in bone marrow failure syndromes.

Acute Lung Injury during Antithymocyte Globulin Therapy for Aplastic Anemia

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Ewan Christopher Goligher

2009-01-01

Full Text Available The case of a 33-year-old man with aplastic anemia who experienced recurrent episodes of hypoxemia and pulmonary infiltrates during infusions of antithymocyte globulin (ATG is described. With the use of high-dose corticosteroids, the patient’s original episodes resolved, and were subsequently prevented before additional administrations of ATG. Rare reports of an association between ATG and acute lung injury are found in the literature, but this is the first report of successful steroid-supported re-exposure. Although the mechanism of ATG-related acute lung injury remains uncertain, it may be parallel to the mechanism of transfusion-related acute lung injury because the pathogenesis of the latter relies, in part, on antileukocyte antibodies. ATG-related toxicity should be included in the differential diagnosis of new, infusion-associated pulmonary infiltrates, and corticosteroids may be a useful therapeutic consideration in the management.

Usefulness of Plasma YKL-40 in Management of Community-Acquired Pneumonia Severity in Patients

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Hsiang-Ling Wang

2013-11-01

Full Text Available Plasma YKL-40 level has been reported as playing a significant role in community-acquired pneumonia (CAP. However, the correlation between plasma level of YKL-40 and the severity of CAP has not been reported. This study identifies the relationship between plasma level changes of the YKL-40 gene in adult patients hospitalized with CAP. The ELISA was used to measure the plasma YKL-40 level from 61 adult CAP patients before and after antibiotic treatment and from 60 healthy controls. The plasma YKL-40 levels were significantly increased in patients with CAP compared to normal controls. Moreover, the plasma concentration of YKL-40 correlated with the severity of CAP based on the pneumonia severity index (PSI score (r = 0.630, p < 0.001, the CURB-65 (confusion, uremia, respiratory rate, BP, age 65 years score (r = 0.640, p < 0.001, the Acute Physiology And Chronic Health Evaluation II (APACHE II score (r = 0.539, p < 0.001 and length of hospital stay (r = 0.321, p = 0.011, respectively. In conclusion, plasma YKL-40 may play a role in the diagnosis and clinical assessment of CAP severity, which could potentially guide the development of treatment strategies.

Total lymphoid irradiation and total body irradiation for allogeneic bone marrow transplantation in aplastic anemia

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Kurisu, Koichi; Hishikawa, Yoshio; Taniguchi, Midori; Kamikonya, Norihiko; Miura, Takashi; Kanamaru, Akihisa; Kakishita, Eizo; Kai, Shunro; Hara, Hiroshi (Hyogo Coll. of Medicine, Nishinomiya (Japan))

Between April 1980 and June 1989, 15 patients with severe aplastic anemia (SAA) were treated at Hyogo College of Medicine with bone marrow transplantation (BMT) after preparation consisting of cyclophosphamide (CY) and total lymphoid irradiation (TLI) or total body irradiation (TBI) for the purpose of reducing the incidence of graft rejection. All patients had initial evidence of engraftment after the first transplantation except for one patient who died of heart failure due to CY on the third day after transplantation and could not be evaluated for engraftment. Rejection later occurred in four of these 14 patients, who then underwent successful regrafting. One of these four patients, who was conditioned with CY alone at the first grafting, underwent successful regrafting after a conditioning regimen of CY and TBI. In the other three patients, irradiation was performed twice as the conditioning regimen. Thus, 14 of 15 patients underwent successful BMT and are alive with restored hematopoietic function. From the above results, the combination of TLI or TBI and CY was considered to be very useful as a conditioning regimen for BMT in patients with SAA. (author).

F-18 FLT PET : A Noninvasive Diagnostic Tool for Visualization of the Bone Marrow Compartment in Patients With Aplastic Anemia A Pilot Study

NARCIS (Netherlands)

Agool, Ali; Slart, Riemer H. J. A.; Kluin, Philip M.; de Wolf, Joost Th. M.; Dierckx, Rudi A. J. O.; Vellenga, Edo

Rationale: A discordant relationship between bone marrow cellularity and peripheral blood findings is regularly noticed in patients with aplastic anemia (AA). Therefore, the feasibility of 3-F-18 fluoro-3-deoxy-L-thymidine (F-18 FLT PET was tested as a noninvasive tool to visualize the total

Total body irradiation as preparation for bone marrow transplantation in treatment of acute leukemia and aplastic anemia

International Nuclear Information System (INIS)

Serota, F.T.; Burkey, E.D.; August, C.S.; D'Angio, G.J.

1983-01-01

In an attempt to improve survival while minimizing toxicity, many bone marrow transplant centers are now studying the use of cytoreduction regimens with an increased amount of radiation in single-dose or fractionated-exposure schedules for patients with leukemia and aplastic anemia. In order to review the current results, the literature prior to September, 1982 was surveyed and data were tabulated for each transplant center regarding the number of patients receiving transplants, diagnoses, cytoreduction regimen, clinical status, remission duration, relapse rate, causes of death and incidence of interstitial pneumonia. The incidence and severity of cataracts, growth failure, hypothyroidism and second malignant neoplasms were noted, and the data obtained from the literature search were updated and expanded by telephone questionnaire when possible. Marked variation in the technique of tranplantation was found among the participating institutions, making it difficult to determine the contribution of the various TBI doses, dose rates and fractionation schedules to the efficacy and toxicity of the combined regimen. In order to define the risk-benefit ratio of the various TBI regimens more clearly, prospective controlled, randomized studies will be required

Impact of cyclophosphamide dose of conditioning on the outcome of allogeneic hematopoietic stem cell transplantation for aplastic anemia from human leukocyte antigen-identical sibling.

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Mori, Takehiko; Koh, Hideo; Onishi, Yasushi; Kako, Shinichi; Onizuka, Makoto; Kanamori, Heiwa; Ozawa, Yukiyasu; Kato, Chiaki; Iida, Hiroatsu; Suzuki, Ritsuro; Ichinohe, Tatsuo; Kanda, Yoshinobu; Maeda, Tetsuo; Nakao, Shinji; Yamazaki, Hirohito

2016-04-01

The standard conditioning regimen in allogeneic hematopoietic stem cell transplantation (HSCT) for aplastic anemia from a human leukocyte antigen (HLA)-identical sibling has been high-dose cyclophosphamide (CY 200 mg/kg). In the present study, results for 203 patients with aplastic anemia aged 16 years or older who underwent allogeneic HSCT from HLA-identical siblings were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. Conditioning regimens were defined as a (1) high-dose CY (200 mg/kg or greater)-based (n = 117); (2) reduced-dose CY (100 mg/kg or greater, but less than 200 mg/kg)-based (n = 38); and (3) low-dose CY (less than 100 mg/kg)-based (n = 48) regimen. Patient age and the proportion of patients receiving fludarabine were significantly higher in the reduced- and low-dose CY groups than the high-dose CY group. Engraftment was comparable among the groups. Five-year overall survival (OS) tended to be higher in the low-dose CY group [93.0 % (95 % CI 85.1-100.0 %)] than the high-dose CY [84.2 % (95 % CI 77.1-91.3 %)] or reduced-dose CY groups [83.8 % (95 % CI 71.8-95.8 %); P = 0.214]. Age-adjusted OS was higher in the low-dose CY group than the high- and reduced-dose CY groups with borderline significance (P = 0.067). These results suggest that CY dose can safely be reduced without increasing graft rejection by adding fludarabine in allogeneic HSCT for aplastic anemia from an HLA-identical sibling.

Characteristic of the Oxidative Stress in Blood of Patients in Dependence of Community-Acquired Pneumonia Severity.

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Muravlyova, Larissa; Molotov-Luchankiy, Vilen; Bakirova, Ryszhan; Klyuyev, Dmitriy; Demidchik, Ludmila; Lee, Valentina

2016-03-15

At the present time the alternation of the oxidative metabolism is considered as one of the leading pathogenic mechanisms in the development and progression of community-acquired pneumonia (CAP). However the nature and direction of the oxidative protein changes in CAP patient's blood had been almost unexplored. To define oxidative and modified proteins in erythrocytes and blood plasma of CAP patients. Blood plasma and erythrocytes obtained from: 42 patients with moderate severity pneumonia, 12 patients with grave severity pneumonia and 32 healthy volunteers. Content of advanced oxidation protein products, malondialdehyde and reactive carbonyl derivatives were estimated as indicators of the oxidative stress and oxidative damage of proteins. In patients with grave severity the level of oxidative proteins and MDA in erythrocytes exceeded both: control values and similar meanings in CAP patients with moderate severity. The further growth of MDA in this group patients' blood plasma was observed, but the level of oxidative proteins decreased in comparison with those in CAP patients with moderate severity. To sum up, our derived data show, that injury of erythrocytes' redox-status and blood plasma components plays an essential role in development and progression CAP.

Immunosuppressive therapy in patients with aplastic anemia: a single-center retrospective study.

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Hasan Jalaeikhoo

Full Text Available Aplastic anemia (AA is a rare disease in which hematopoietic stem cells are severely diminished resulting in hypocellular bone marrow and pancytopenia. Etiology of AA includes auto immunity, toxins, infection, ionizing radiation, drugs and rare genetic disorders, but in the majority of cases no cause can be identified. In the present study we assessed response rate, survival, relapse and clonal evolution in patients with AA treated with immunosuppressive therapy.Patients with AA who received immunosuppressive therapy between May 1998 and September 2013 were included in this study. Patients with non-severe AA (NSAA were treated with cyclosporine (CsA and danazol while patients with severe AA (SAA as well as patients with NSAA who progressed to SAA after beginning of the treatment, were candidates for receiving antithymocyte globulin in addition to CsA and danazol.Among the 63 studied patients, 29 (46% had NSAA and 34 (54% had SAA. Three months after treatment, overall response was 58.6% in NSAA and 12.9% in patients with SAA. Survival of all patients at 5, 10 and 15 years were 73%, 55% and 49%, respectively. Survival rates were significantly higher in patients with NSAA compared to patients with SAA as well as in patients who responded at 6 months compared to non-responders. The relapse risk was 39.7% at 10 years. Relapse occurred in patients who discontinued the therapy more than those who continued taking CsA (p value<0.01. The risk of clonal evolution was 9.9% at 10 years and 22.8% at 15 years after treatment.This long-term retrospective study indicated that immunosuppressive therapy should be recommended to patients with AA. Also, our experience indicated that immunosuppressive therapy should not be discontinued after response to therapy in patients with both NSAA and SAA due to high risk of relapse. Low dose of CsA should be continued indefinitely.

Endogenous carboxyhemoglobin concentrations in the assessment of severity in patients with community-acquired pneumonia.

Science.gov (United States)

Corbacioglu, Seref Kerem; Kilicaslan, Isa; Bildik, Fikret; Guleryuz, Atacan; Bekgoz, Burak; Ozel, Ayca; Keles, Ayfer; Demircan, Ahmet

2013-03-01

Previous studies have shown that carbon monoxide, which is endogenously produced, is increased in community-acquired pneumonia (CAP). However, it has not been studied enough whether severity of pneumonia is correlated with increased carboxyhemoglobin (COHb) concentrations in CAP. The aim of this study was to determine whether endogenous carbon monoxide levels in patients with CAP were higher compared with the control group and, if so, to determine whether COHb concentrations could predict severity in CAP. Eighty-two patients with CAP were evaluated in this cross-sectional study during a 10-month period. Demographic data, pneumonia severity index and confusion, uremia, rate respiratory, pressure blood, age>65 (CURB-65) scores, hospital admission or discharge decisions, and 30-day hospital mortality rate were recorded. In addition, 83 control subjects were included to study. The COHb concentration was measured in arterial blood sample. The levels of COHb in patients with CAP were 1.70% (minimum-maximum, 0.8-3.2), whereas those in control subjects, 1.40% (minimum-maximum, 0.8-2.9). The higher COHb concentrations in patients with CAP were statistically significant (P < .05). Concentration of COHb correlated with pneumonia severity index (P = .04, r = 0.187); however, it did not correlate with CURB-65 (P = .218, r = 0.112). Although COHb concentrations show an increase in patients with pneumonia, it was concluded that this increase did not act as an indicator in diagnosis process or prediction of clinical severity for the physicians. Copyright © 2013 Elsevier Inc. All rights reserved.

Enhanced Adipogenicity of Bone Marrow Mesenchymal Stem Cells in Aplastic Anemia

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Naresh Kumar Tripathy

2014-01-01

Full Text Available Fatty bone marrow (BM and defective hematopoiesis are a pathologic hallmark of aplastic anemia (AA. We have investigated adipogenic and osteogenic potential of BM mesenchymal stem cells (BM-MSC in 10 AA patients (08 males and 02 females with median age of 37 years (range: 06 to 79 years and in the same number of age and sex matched controls. It was observed that BM-MSC of AA patients had a morphology, phenotype, and osteogenic differentiation potential similar to control subjects but adipocytes differentiated from AA BM-MSC had a higher density and larger size of lipid droplets and they expressed significantly higher levels of adiponectin and FABP4 genes and proteins as compared to control BM-MSC (P<0.01 for both. Thus our data shows that AA BM-MSC have enhanced adipogenicity, which may have an important implication in the pathogenesis of the disease.

Flow cytometric analysis of lymphocytes in aplastic anemia among atomic bomb survivors

International Nuclear Information System (INIS)

Imamura, Nobutaka; Inada, Tominari; Asaoku, Hideki; Abe, Kazuhiro; Oguma, Nobuo; Kuramoto, Atsushi

1986-01-01

In 6 patients with aplastic anemia and 3 patients with pernicious anemia, lymphocyte subpopulations in the peripheral blood were measured, before and after steroid therapy, with a fluorescence-activated cell sorder using various monoclonal antibodies. The ratio of OKT4-positive lymphocytes (T4) to OKT8-positive lymphocytes (T8) in the peripheral blood was reduced in 2 patients (20 %). The T4/T8 ratio returned to normal during remission of anemia. Hematological improvement was seen after a large amount of steroid therapy in 3 patients. The number of Tac-positive cells tended to decrease and the T4/T8 ratio tended to return to normal with hematological improvement, although there was no correlation to hydrocortisone reaction. Some patients were supposed to have abnormal number of suppressor and inducer T cells. (Namekawa, K.)

Aplastic anaemia preceding acute lymphoblastic leukaemia in an adult with isolated deletion of chromosome 9q.

LENUS (Irish Health Repository)

Kelly, Kevin

2008-12-01

Aplastic anaemia (AA) can precede acute lymphoblastic leukaemia (ALL) in 2% of children but this is rarely reported to occur in adults. A 21-year-old male presented with bone marrow failure and bone marrow biopsy showed a profoundly hypocellular marrow. He recovered spontaneously but represented 2 months later when he was diagnosed with pre-B acute lymphoblastic leukaemia. Chromosomal examination revealed 46,XY,del(9)(q13q34). To the best of our knowledge this is the first case to be reported of aplasia preceding ALL with 9q minus as the sole chromosomal abnormality.

Community-acquired pneumonia: economics of inpatient medical care vis-à-vis clinical severity,

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Vojislav Cupurdija

2015-02-01

Full Text Available Objective: To assess the direct and indirect costs of diagnosing and treating community-acquired pneumonia (CAP, correlating those costs with CAP severity at diagnosis and identifying the major cost drivers. Methods: This was a prospective cost analysis study using bottom-up costing. Clinical severity and mortality risk were assessed with the pneumonia severity index (PSI and the mental Confusion-Urea-Respiratory rate-Blood pressure-age ≥ 65 years (CURB-65 scale, respectively. The sample comprised 95 inpatients hospitalized for newly diagnosed CAP. The analysis was run from a societal perspective with a time horizon of one year. Results: Expressed as mean ± standard deviation, in Euros, the direct and indirect medical costs per CAP patient were 696 ± 531 and 410 ± 283, respectively, the total per-patient cost therefore being 1,106 ± 657. The combined budget impact of our patient cohort, in Euros, was 105,087 (66,109 and 38,979 in direct and indirect costs, respectively. The major cost drivers, in descending order, were the opportunity cost (lost productivity; diagnosis and treatment of comorbidities; and administration of medications, oxygen, and blood derivatives. The CURB-65 and PSI scores both correlated with the indirect costs of CAP treatment. The PSI score correlated positively with the overall frequency of use of health care services. Neither score showed any clear relationship with the direct costs of CAP treatment. Conclusions: Clinical severity at admission appears to be unrelated to the costs of CAP treatment. This is mostly attributable to unwarranted hospital admission (or unnecessarily long hospital stays in cases of mild pneumonia, as well as to over-prescription of antibiotics. Authorities should strive to improve adherence to guidelines and promote cost-effective prescribing practices among physicians in southeastern Europe.

Plasma Monocyte Chemoattractant Protein-1 Level as a Predictor of the Severity of Community-Acquired Pneumonia

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Kok-Khun Yong

2016-01-01

Full Text Available Monocyte chemoattractant protein (MCP-1 increases in the serum of immunocompetent patients with community-acquired pneumonia (CAP. However, the correlation between the circulating level of MCP-1 and severity of CAP remains unclear. This study investigated differential changes in the plasma MCP-1 levels of patients with CAP before and after an antibiotic treatment and further analyzes the association between the CAP severity and MCP-1 levels. We measured the plasma MCP-1 levels of 137 patients with CAP and 74 healthy controls by using a commercial enzyme-linked immunosorbent assay. Upon initial hospitalization, Acute Physiology and Chronic Health Evaluation II (APACHE II; confusion, urea level, respiratory rate, blood pressure, and age of >64 years (CURB-65; and pneumonia severity index (PSI scores were determined for assessing the CAP severity in these patients. The antibiotic treatment reduced the number of white blood cells (WBCs and neutrophils as well as the level of C-reactive protein (CRP and MCP-1. The plasma MCP-1 level, but not the CRP level or WBC count, correlated with the CAP severity according to the PSI (r = 0.509, p < 0.001, CURB-65 (r = 0.468, p < 0.001, and APACHE II (r = 0.360, p < 0.001 scores. We concluded that MCP-1 levels act in the development of CAP and are involved in the severity of CAP.

I-131 therapy for hyperthyroidism in carbimazole induced acute aplastic anaemia

International Nuclear Information System (INIS)

Jenkin, B.A.; Van Every, B.; Kelly, M.J.

1998-01-01

Full text: We present a case of a 39 year old previously well woman who developed carbimazole-induced acute aplastic anaemia. Following 6 weeks of carbimazole for Grave's disease she presented with fever, sore throat and lethargy and was found to be pancytopaenic. Haemoglobin fell to 79g/l (normal>120g/l), neutrophils to an undetectable level and platelets to 4,000/ml (normal>150,000/ml). From the MIRD schema it was estimated that a therapeutic dose of 444 MBq (12mCi) to treat her hyperthyroidism would deliver a red marrow radiation dose of about 30 mSv, at least an order of magnitude below any expected demonstrable deleterious effect on the marrow. Subsequently a dose of 444 MBq of I-131 was given and within 2 weeks the marrow had begun to recover, eventually to normal. This case would support the use of 1-131 for hyperthyroidism in carbimazole-induced marrow aplasia, even in profoundly depressed marrow

I-131 therapy for hyperthyroidism in carbimazole induced acute aplastic anaemia

Energy Technology Data Exchange (ETDEWEB)

Jenkin, B.A.; Van Every, B.; Kelly, M.J. [Alfred Hospital, Prahran, VIC (Australia). Nuclear Medicine Department

1998-06-01

Full text: We present a case of a 39 year old previously well woman who developed carbimazole-induced acute aplastic anaemia. Following 6 weeks of carbimazole for Grave`s disease she presented with fever, sore throat and lethargy and was found to be pancytopaenic. Haemoglobin fell to 79g/l (normal>120g/l), neutrophils to an undetectable level and platelets to 4,000/ml (normal>150,000/ml). From the MIRD schema it was estimated that a therapeutic dose of 444 MBq (12mCi) to treat her hyperthyroidism would deliver a red marrow radiation dose of about 30 mSv, at least an order of magnitude below any expected demonstrable deleterious effect on the marrow. Subsequently a dose of 444 MBq of I-131 was given and within 2 weeks the marrow had begun to recover, eventually to normal. This case would support the use of 1-131 for hyperthyroidism in carbimazole-induced marrow aplasia, even in profoundly depressed marrow

MR imaging follow-up studies in patients with aplastic anemia

International Nuclear Information System (INIS)

Tscholakoff, D.; Herold, C.; Pongracz, I.; Hinterberger, W.; Kier, P.; Chott, A.; Imhof, H.

1988-01-01

Eight patients with aplastic anemia underwent MR imaging of the pelvis and thoracolumbar spine before and during treatment. Bone marrow patterns on MR images were correlated with stem-cell and peripheral-blood cell counts and Prussian blue stain of bone marrow (to estimate iron overload). Before treatment, two different bone marrow patterns were observed. Three patients had diffuse, homogeneous high signal intensity throughout the bone marrow, indicating fatty replacement of hematopoietic bone marrow. Bone marrow stains were negative for iron overload. Follow-up studies in these three patients showed multiple areas of hypointense foci in the bone marrow at the time when stem cell and blood cell counts returned to normal. The remaining five patients displayed inhomogeneous signal distribution (hypointense foci on T1-weighted images) in the bone marrow on the first and following MR studies. All five patients did not respond to therapy and had multiple transfusions. Bone marrow signal intensities (correlated to a phantom) were similar in patients with recovery of hematopoietic bone marrow and patients with hemochromatosis

Non-HIV Pneumocystis pneumonia: do conventional community-acquired pneumonia guidelines under estimate its severity?

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Asai, Nobuhiro; Motojima, Shinji; Ohkuni, Yoshihiro; Matsunuma, Ryo; Nakasima, Kei; Iwasaki, Takuya; Nakashita, Tamao; Otsuka, Yoshihito; Kaneko, Norihiro

2012-06-11

Non-HIV Pneumocystis pneumonia (PCP) can occur in immunosuppressed patients having malignancy or on immunosuppressive agents. To classify severity, the A-DROP scale proposed by the Japanese Respiratory Society (JRS), the CURB-65 score of the British Respiratory Society (BTS) and the Pneumonia Severity Index (PSI) of the Infectious Diseases Society of America (IDSA) are widely used in patients with community-acquired pneumonia (CAP) in Japan. To evaluate how correctly these conventional prognostic guidelines for CAP reflect the severity of non-HIV PCP, we retrospectively analyzed 21 patients with non-HIV PCP. A total of 21 patients were diagnosed by conventional staining and polymerase chain reaction (PCR) for respiratory samples with chest x-ray and computed tomography (CT) findings. We compared the severity of 21 patients with PCP classified by A-DROP, CURB-65, and PSI. Also, patients' characteristics, clinical pictures, laboratory results at first visit or admission and intervals from diagnosis to start of specific-PCP therapy were evaluated in both survivor and non-survivor groups. Based on A-DROP, 18 patients were classified as mild or moderate; respiratory failure developed in 15 of these 18 (83.3%), and 7/15 (46.7%) died. Based on CURB-65, 19 patients were classified as mild or moderate; respiratory failure developed in 16/19 (84.2%), and 8 of the 16 (50%) died. In contrast, PSI classified 14 as severe or extremely severe; all of the 14 (100%) developed respiratory failure and 8/14 (57.1%) died. There were no significant differences in laboratory results in these groups. The time between the initial visit and diagnosis, and the time between the initial visit and starting of specific-PCP therapy were statistically shorter in the survivor group than in the non-survivor group. Conventional prognostic guidelines for CAP could underestimate the severity of non-HIV PCP, resulting in a therapeutic delay resulting in high mortality. The most important factor to

Using data-driven rules to predict mortality in severe community acquired pneumonia.

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Chuang Wu

Full Text Available Prediction of patient-centered outcomes in hospitals is useful for performance benchmarking, resource allocation, and guidance regarding active treatment and withdrawal of care. Yet, their use by clinicians is limited by the complexity of available tools and amount of data required. We propose to use Disjunctive Normal Forms as a novel approach to predict hospital and 90-day mortality from instance-based patient data, comprising demographic, genetic, and physiologic information in a large cohort of patients admitted with severe community acquired pneumonia. We develop two algorithms to efficiently learn Disjunctive Normal Forms, which yield easy-to-interpret rules that explicitly map data to the outcome of interest. Disjunctive Normal Forms achieve higher prediction performance quality compared to a set of state-of-the-art machine learning models, and unveils insights unavailable with standard methods. Disjunctive Normal Forms constitute an intuitive set of prediction rules that could be easily implemented to predict outcomes and guide criteria-based clinical decision making and clinical trial execution, and thus of greater practical usefulness than currently available prediction tools. The Java implementation of the tool JavaDNF will be publicly available.

Beneficial Role of Low-Dose Antithymocyte Globulin in Unrelated Stem Cell Transplantation for Adult Patients with Acquired Severe Aplastic Anemia: Reduction of Graft-versus-Host Disease and Improvement of Graft-versus-Host Disease-Free, Failure-Free Survival Rate.

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Park, Sung-Soo; Kwak, Dae Hun; Jeon, Young-Woo; Yoon, Jae-Ho; Lee, Sung-Eun; Cho, Byung-Sik; Eom, Ki-Seong; Kim, Yoo-Jin; Kim, Hee-Je; Lee, Seok; Min, Chang-Ki; Cho, Seok-Goo; Kim, Dong-Wook; Min, Woo-Sung; Lee, Jong Wook

2017-09-01

Stem cell transplantation (SCT) from an unrelated donor (URD) is often considered in patients with severe aplastic anemia (SAA) whom immunosuppressive therapy failed and matched sibling donor is not available. To reduce the incidence of graft-versus-host disease (GVHD) in URD SCT, introducting antithymocyte globulin (ATG) into the conditioning regimen has been proposed. Although ATG was shown to play a role in reducing GVHD in a cohort with diverse hematologic diseases, its role in SAA remains uncertain. The aim of this study was to determine the efficacy and toxicity of ATG in URD SCT for adult patients with SAA. We investigated 83 adult patients with SAA who underwent URD SCT between 2003 and 2014. The transplantation strategy consisted of total body irradiation (total 800 cGy) and cyclophosphamide (total 100 mg/kg to 120 mg/kg), followed by tacrolimus and a short-term methotrexate. We divided patients into 2 groups: group 1 (n = 25), which received HLA-matched (8/8) bone marrow (BM) without ATG, and group 2 (n = 58), which received SCT from either an HLA-mismatched donor or peripheral blood (PB). Thereafter, group 2 was subdivided according to ATG use into group 2A (without ATG, n = 26), which served as a historical cohort, and group 2B (with ATG, n = 32). Rabbit ATG (Thymoglobulin; Genzyme-Sanofi, Lyon, France) was used in group 2B at a dose of 2.5 mg/kg. The median age of all patients was 30 years (range, 17 to 59 years). The incidence of GVHD was significantly lower in group 2B than group 2A, as demonstrated by the rate of grade II to IV acute GVHD at day 100 (31.2% versus 61.5%, P = .003) and the rate of chronic GVHD at 3 years (21.9% versus 65.4%, P = .002). The overall survival rates of the 3 groups were similar. However, GVHD-free, failure-free survival (GFFS) was significantly higher in group 2B than group 2A (P = .034). A multivariable model identified use of ATG as an independent factor affecting grades II to IV acute

Isolated acquired factor VII deficiency: review of the literature.

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Mulliez, Sylvie M N; Devreese, Katrien M J

2016-04-01

Isolated acquired factor VII (FVII) deficiency is a rare haemorrhagic disorder. We report what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of acquired FVII deficiency. We performed a literature search and included all articles published between 1980 and August 2015. Acquired FVII deficiency has been reported in 42 patients. There are well-established clinical diseases associated with acquired FVII deficiency, most notably infections, malignancy and haematological stem cell transplantation. The exact pathogenesis of the diseases is still unknown, but different pathophysiological hypotheses have been suggested. The clinical manifestation of acquired FVII deficiency varies greatly in severity; asymptomatic course as well as severe life-threatening bleeding diathesis and fatal bleedings have been described.

Self-esteem as a predictor of psychological distress after severe acquired brain injury: an exploratory study.

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Cooper-Evans, Samantha; Alderman, Nick; Knight, Caroline; Oddy, Michael

2008-01-01

This study explored the effects of severe acquired brain injury (ABI) on self-esteem. A within-subjects design investigated 22 severe ABI survivors' self-reported responses on measures of self-esteem, mood and awareness of deficit. Data on cognitive ability and awareness of degree of executive impairment were included in the analysis. Self-esteem was measured using Rosenberg's Self-Esteem Scale (Rosenberg) and psychological distress by the Hospital Anxiety and Depression Scale (HADS). Self-esteem was found to be consistent over a two-week interval. Participants reported that their self-esteem had suffered following ABI when contrasting their current self-esteem with their retrospective perceptions. Self-esteem was highly correlated with psychological distress. More intact cognitive functioning and awareness of deficit were associated with lower self-esteem. The paradoxical finding that survivors who were more impaired cognitively and/or less aware of their deficits reported higher self-esteem poses an ethical dilemma for clinicians. It is hoped that this finding, along with the consistency of self-esteem ratings sparks further debate about how best to address issues of self-esteem among severe ABI survivors, particularly in the context of psychological distress, during rehabilitation.

Association of aplastic anemia and FoxP3 gene polymorphisms in Koreans.

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In, Ji Won; Lee, Nuri; Roh, Eun Youn; Shin, Sue; Park, Kyoung Un; Song, Eun Young

2017-04-01

Aplastic anemia (AA) is characterized by pancytopenia and bone marrow failure, and most acquired AA is an immune-mediated disorder. Regulatory T cells (T regs ) suppressing autoreactive T cells were decreased in AA patients. FoxP3 is a major regulator for the development and function of T regs . Polymorphism in FoxP3 was shown to be associated with various autoimmune diseases, however, has not yet been studied in AA. In this study, we examined the association between FoxP3 polymorphisms and AA in Korean patients. The study population consisted of 94 patients diagnosed by bone marrow examination in Seoul National University Hospital (SNUH) during 1997-2012 and 195 healthy controls. FoxP3 polymorphisms (rs5902434 del/ATT, rs3761548 C/A, rs3761549 C/T, rs2232365 A/G) were analyzed by PCR-sequencing method. We analyzed differences of genotype and allele frequencies between patients and controls. We also compared differences of genotype and allele frequencies between responder and non-responder in patients treated with immunosuppressive therapy (IST). For the statistical analysis, the chi-square test and Fisher's exact test were used and P < 0.05 was regarded as statistically significant. There was no significant difference in the genotype frequencies of FoxP3 polymorphisms between patients and controls. With regards to the allele frequencies, rs3761548 C allele was significantly higher in AA patients than in controls (87.4% vs. 79.7%, P = 0.047). In patients treated with IST, rs3761549 C allele was significantly higher in non-responder patients than in responders (89.6% vs. 66.7%, P = 0.036) and female rs3761549 C/C genotype carriers were associated with greater risk for non-response to IST (84.2% vs. 16.7%, P = 0.006). Polymorphisms in rs3761548 and rs3761549 of FoxP3 in our population were associated with disease susceptibility and response for IST, respectively. This study suggests an association between FoxP3 polymorphisms and AA in Korean patients

Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia Crise aplástica devido à infecção por parvovirus humano B19 em anemia hemolítica hereditária

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R. C. N. Cubel

1992-10-01

Full Text Available Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.IgM específica anti-B19 foi demonstrada nos soros de três crianças apresentando aplasia transitória de medula. Um menino de dois anos de idade vivendo no Rio de Janeiro e sendo portador de anemia falciforme, apresentou a crise durante Agosto de 1990. Dois irmãos vivendo em Santa Maria - RS, desenvolveram crise de aplasia em Maio de 1991, quando foram também diagnosticados como portadores de microesferocitose. IgM anti-B19 não foi detectada no soro de uma terceira criança, desta mesma família, a qual primeiramente apresentou crise de aplasia.

Treatment with macrolides and glucocorticosteroids in severe community-acquired pneumonia: A post-hoc exploratory analysis of a randomized controlled trial.

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Adrian Ceccato

Full Text Available Systemic corticosteroids have anti-inflammatory effects, whereas macrolides also have immunomodulatory activity in addition to their primary antimicrobial actions. We aimed to evaluate the potential interaction effect between corticosteroids and macrolides on the systemic inflammatory response in patients with severe community-acquired pneumonia to determine if combining these two immunomodulating agents was harmful, or possibly beneficial.We performed a post-hoc exploratory analysis of a randomized clinical trial conducted in three tertiary hospitals in Spain. This trial included patients with severe community-acquired pneumonia with high inflammatory response (C-reactive protein [CRP] >15 mg/dL who were randomized to receive methylprednisolone 0.5 mg/kg/tpd or placebo. The choice of antibiotic treatment was at the physician's discretion. One hundred and six patients were classified into four groups according to antimicrobial therapy combination (β-lactam plus macrolide or β-lactam plus fluoroquinolone and corticosteroid arm (placebo or corticosteroids. The primary outcome was treatment failure (composite outcome of early treatment failure, or of late treatment failure, or of both early and late treatment failure.The methylprednisolone with β-lactam plus macrolide group had more elderly patients, with comorbidities, and higher pneumonia severity index (PSI risk class V, but a lower proportion of intensive care unit admission, compared to the other groups. We found non differences in treatment failure between groups (overall p = 0.374; however, a significant difference in late treatment failure was observed (4 patients in the placebo with β-lactam plus macrolide group (31% vs. 9 patients in the placebo with β-lactam plus fluoroquinolone group (24% vs. 0 patients in the methylprednisolone with β-lactam plus macrolide group (0% vs. 2 patients [5%] in the methylprednisolone with β-lactam plus fluoroquinolone group overall p = 0.009. We found

Non-HIV Pneumocystis pneumonia: do conventional community-acquired pneumonia guidelines under estimate its severity?

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Asai Nobuhiro

2012-06-01

Full Text Available Abstract Background Non-HIV Pneumocystis pneumonia (PCP can occur in immunosuppressed patients having malignancy or on immunosuppressive agents. To classify severity, the A-DROP scale proposed by the Japanese Respiratory Society (JRS, the CURB-65 score of the British Respiratory Society (BTS and the Pneumonia Severity Index (PSI of the Infectious Diseases Society of America (IDSA are widely used in patients with community-acquired pneumonia (CAP in Japan. To evaluate how correctly these conventional prognostic guidelines for CAP reflect the severity of non-HIV PCP, we retrospectively analyzed 21 patients with non-HIV PCP. Methods A total of 21 patients were diagnosed by conventional staining and polymerase chain reaction (PCR for respiratory samples with chest x-ray and computed tomography (CT findings. We compared the severity of 21 patients with PCP classified by A-DROP, CURB-65, and PSI. Also, patients’ characteristics, clinical pictures, laboratory results at first visit or admission and intervals from diagnosis to start of specific-PCP therapy were evaluated in both survivor and non-survivor groups. Results Based on A-DROP, 18 patients were classified as mild or moderate; respiratory failure developed in 15 of these 18 (83.3%, and 7/15 (46.7% died. Based on CURB-65, 19 patients were classified as mild or moderate; respiratory failure developed in 16/19 (84.2%, and 8 of the 16 (50% died. In contrast, PSI classified 14 as severe or extremely severe; all of the 14 (100% developed respiratory failure and 8/14 (57.1% died. There were no significant differences in laboratory results in these groups. The time between the initial visit and diagnosis, and the time between the initial visit and starting of specific-PCP therapy were statistically shorter in the survivor group than in the non-survivor group. Conclusions Conventional prognostic guidelines for CAP could underestimate the severity of non-HIV PCP, resulting in a therapeutic delay

Nutritional support in the treatment of aplastic anemia.

Science.gov (United States)

Jia, Li; Yu, Jingda; He, Ling; Wang, Huaxin; Jiang, Lili; Miao, Xiaoyan; Wu, Wenguo; Yang, Peiman

2011-01-01

Whether a specific nutritional support promotes healing of aplastic anemia (AA) patients is still unclear. Therefore, we explored the potential of a high-nucleotide, arginine, and micronutrient nutritional supplement on the nutritional rehabilitation of AA mice. The BALB/c AA mice model was treated with hypodermic injections of acetylphenylhydrazine (100 mg/kg), x-ray (2.0 Gy), and intraperitoneal injections of a cyclophosphamide (80 mg/kg) combination. Then AA mice were fed with nutritional supplements in a dose-dependent manner (1445.55, 963.7, 674.59 mg/kg/d) for 7 wk. At the end of the experimental period, mice were autopsied. A full blood count was performed, and femoral marrow cell suspensions were prepared to assess the total femoral nucleated cell count and the number of committed hemopoietic progenitor cells (colony-forming units). The pathologic changes of liver and spleen were analyzed. The significant increases of nutrient mixture groups were evident in many peripheral blood parameters. The femoral nucleated cell count and colony-forming units of nutritional supplements groups were markedly increased, compared with the AA group. Transmission electron microscopy showed that the number of mitochondria in similar bone marrow cells was increased in nutritional supplements groups. The nutritional supplements also affected the recovery of livers and spleens of AA mice. Specific nutritional supplements accelerated rehabilitation of AA mice and can be used as nutritional support in the treatment of AA. Copyright © 2011 Elsevier Inc. All rights reserved.

Intravenous saline administration in patients with severe acquired brain injury and orthostatic intolerance for tilt-table mobilization

DEFF Research Database (Denmark)

Riberholt, Christian; Olesen, Niels; Hovind, Peter

2018-01-01

Primary objective: This study aimed to investigate the effect of intravenous saline administration on orthostatic hypotension (OH) during head up tilt (HUT) and the change in the renin–angiotensin–aldosterone system before and after HUT in patients with severe acquired brain injury (ABI). Research...... artery blood flow velocity. Blood samples were collected before and after two HUT sessions separated by 1 hour and saline was administered in between. Main outcomes and results: Patients’ ability to stand upright did not change after saline administration due to OH. The patients showed signs of reduced...... fluid administration. Research focusing on the ability to retain fluid after bed rest is warranted....

First line treatment of aplastic anemia with thymoglobuline in Europe and Asia: Outcome of 955 patients treated 2001-2012.

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Bacigalupo, Andrea; Oneto, Rosi; Schrezenmeier, Hubert; Hochsmann, Britta; Dufour, Carlo; Kojima, Seiji; Zhu, Xiaofan; Chen, Xiaojuan; Issaragrisil, Surapol; Chuncharunee, Suporn; Jeong, Dae Chul; Giammarco, Sabrina; Van Lint, Maria Teresa; Zheng, Yizhou; Vallejo, Carlos

2018-05-01

The aim of this study was to assess the outcome of patients with aplastic anemia (AA), receiving rabbit anti-thymocyte globulin (Thymoglobulin, SANOFI) and cyclosporin, as first line treatment. Eligible were 955 patients with AA, treated first line with Thymoglobulin, between 2001 and 2008 (n = 492), or between 2009 and 2012 (n = 463). The median age of the patients was 21 years (range 1-84). Mortality within 90 days was 5.7% and 2.4%, respectively in the two time periods (P = .007).The actuarial 10-year survival for the entire population was 70%; transplant free survival was 64%. Predictors of survival in multivariate analysis, were severity of the disease, patients age and the interval between diagnosis and treatment. Survival was 87% vs 61% for responders at 6 months versus nonresponders (P < .0001). The 10-year survival of nonresponders at 6 months, undergoing a subsequent transplant (n = 110), was 64%, vs 60% for patient not transplantated (n = 266) (P = .1). The cumulative incidence of response was 37%, 52%, 65% respectively, at 90, 180, and 365 days. In multivariate analysis, negative predictors of response at 6 months, were older age, longer interval diagnosis treatment, and greater severity of the disease. In conclusion, early mortality is low after first line treatment of AA with Thymoglobulin, and has been further reduced after year 2008. Patients age, together with interval diagnosis-treament and severity of the disease, remain strong predictors of response and survival. © 2018 Wiley Periodicals, Inc.

Acquired ventricular septal defect due to infective endocarditis

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Randi E Durden

2018-01-01

Full Text Available Acquired intracardiac left-to-right shunts are rare occurrences. Chest trauma and myocardial infection are well-known causes of acquired ventricular septal defect (VSD. There have been several case reports describing left ventricle to right atrium shunt after infective endocarditis (IE. We present here a patient found to have an acquired VSD secondary to IE of the aortic and tricuspid valves in the setting of a known bicuspid aortic valve. This is the first case reported of acquired VSD in a pediatric patient in the setting of IE along with literature review of acquired left-to-right shunts.

Respiratory viruses among children with non-severe community-acquired pneumonia: A prospective cohort study.

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Nascimento-Carvalho, Amanda C; Vilas-Boas, Ana-Luisa; Fontoura, Maria-Socorro H; Vuorinen, Tytti; Nascimento-Carvalho, Cristiana M

2018-06-06

Community-acquired pneumonia (CAP) causes a major burden to the health care system among children under-5 years worldwide. Information on respiratory viruses in non-severe CAP cases is scarce. To estimate the frequency of respiratory viruses among non-severe CAP cases. Prospective study conducted in Salvador, Brazil. Out of 820 children aged 2-59 months with non-severe CAP diagnosed by pediatricians (respiratory complaints and radiographic pulmonary infiltrate/consolidation), recruited in a clinical trial (ClinicalTrials.gov Identifier NCT01200706), nasopharyngeal aspirate samples were obtained from 774 (94.4%) patients and tested for 16 respiratory viruses by PCRs. Viruses were detected in 708 (91.5%; 95%CI: 89.3-93.3) cases, out of which 491 (69.4%; 95%CI: 65.9-72.7) harbored multiple viruses. Rhinovirus (46.1%; 95%CI: 42.6-49.6), adenovirus (38.4%; 95%CI: 35.0-41.8), and enterovirus (26.5%; 95%CI: 23.5-29.7) were the most commonly found viruses. The most frequent combination comprised rhinovirus plus adenovirus. No difference was found in the frequency of RSVA (16.1% vs. 14.6%; P = 0.6), RSVB (10.9% vs. 13.2%; P = 0.4) influenza (Flu) A (6.3% vs. 5.1%; P = 0.5), FluB (4.5% vs. 1.8%; P = 0.09), parainfluenza virus (PIV) 1 (5.1% vs. 2.8%; P = 0.2), or PIV4 (7.7% vs. 4.1%; P = 0.08), when children with multiple or sole virus detection were compared. Conversely, rhinovirus, adenovirus, enterovirus, bocavirus, PIV2, PIV3, metapneumovirus, coronavirus OC43, NL63, 229E were significantly more frequent among cases with multiple virus detection. Respiratory viruses were detected in over 90% of the cases, out of which 70% had multiple viruses. Several viruses are more commonly found in multiple virus detection whereas other viruses are similarly found in sole and in multiple virus detection. Copyright © 2018 Elsevier B.V. All rights reserved.

Social skills treatment for people with severe, chronic acquired brain injuries: a multicenter trial.

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McDonald, Skye; Tate, Robyn; Togher, Leanne; Bornhofen, Cristina; Long, Esther; Gertler, Paul; Bowen, Rebecca

2008-09-01

To determine whether social skills deficits including unskilled, inappropriate behavior, problems reading social cues (social perception), and mood disturbances (such as depression and anxiety) could be remediated after severe traumatic brain injuries. Randomized controlled trial comparing a social skills program with social activity alone or with waitlist control. Several participants were reassigned after randomization. Hospital outpatient and community facilities. Fifty-one outpatients from 3 brain injury units in Sydney, Australia, with severe, chronic acquired brain injuries were recruited. A total of 39 people (13 in skills training, 13 in social activity, 13 in waitlist) completed all phases of the study. Twelve-week social skills treatment program encompassing weekly 3-hour group sessions focused on shaping social behavior and remediating social perception and 1-hour individual sessions to address psychologic issues with mood, self-esteem, etc. Primary outcomes were: (1) social behavior during encounters with a confederate as rated on the Behaviorally Referenced Rating System of Intermediary Social Skills-Revised (BRISS-R), (2) social perception as measured by The Awareness of Social Inference Test, and (3) depression and anxiety as measured by the Depression, Anxiety and Stress Scale. Secondary outcomes were: relative report on social behavior and participation using: the Katz Adjustment Scale-R1; the Social Performance Survey Schedule; the La Trobe Communication Questionnaire; and the Sydney Psychosocial Reintegration Scale (both relative and self-report). Repeated-measures analysis of variance indicated that social activity alone did not lead to improved performance relative to waitlist (placebo effect) on any outcome variable. On the other hand, the skills training group improved differentially on the Partner Directed Behavior Scale of the BRISS-R, specifically the self-centered behavior and partner involvement behavior subscales. No treatment effects

Corpus callosum demyelination associated with acquired stuttering.

Science.gov (United States)

Decker, Barbara McElwee; Guitar, Barry; Solomon, Andrew

2018-04-21

Compared with developmental stuttering, adult onset acquired stuttering is rare. However, several case reports describe acquired stuttering and an association with callosal pathology. Interestingly, these cases share a neuroanatomical localisation also demonstrated in developmental stuttering. We present a case of adult onset acquired stuttering associated with inflammatory demyelination within the corpus callosum. This patient's disfluency improved after the initiation of immunomodulatory therapy. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Ocular manifestations of idiopathic aplastic anemia: retrospective study and literature review

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Mansour AM

2014-04-01

Full Text Available Ahmad M Mansour,1 Jong Wook Lee,2 Seung Ah Yahng,2 Kyu Seop Kim,3 Maha Shahin,4 Nelson Hamerschlak,5 Rubens N Belfort,6 Shree K Kurup71Department of Ophthalmology, American University of Beirut, Rafic Hariri University Hospital, Beirut, Lebanon; 2Division of Hematology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 3Department of Ophthalmology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 4Department of Ophthalmology, Mansoura University, Mansoura City, Egypt; 5Oncology and Hematology Program, Instituto Israelita de Ensino e Pesquisa Albert Einstein, São Paulo, Brazil; 6Vision Institute, Hospital São Paulo, Federal University of São Paulo, Brazil; 7Department of Ophthalmology, Wake Forest University Baptist Medical Center, Winston-Salem, NC, USAAbstract: Aplastic anemia (AA is a rare disease with few reports on its ophthalmic ­manifestations. The ocular findings are described in a retrospective consecutive series of 719 AA Korean patients followed at the Hematology Clinic of The Catholic University of Korea. Out of a total of 719 patients, 269 patients had eye examinations, 156 patients had retinal evaluation, and 37 (23.7% had retinal findings. These 37 patients had unilateral retinal ­hemorrhage in seven and bilateral retinal hemorrhage in 30 with mean hemoglobin of 6.6 g/dL (range 2.7–12.6 g/dL and platelet counts of 18.8×109/L (range 4–157×109/L; central retinal vein occlusion-like picture occurred in nine patients and these had similar rheology to the rest of the subjects; optic disc edema, cotton-wool spots, macular edema, and dry eyes occurred in two, three, five, and three patients, respectively. In this Korean series of 141 subjects with AA, systemic bleeding occurred in 24.8% of subjects, retinal hemorrhage in 37% of subjects, and any bleeding site (eye or elsewhere occurred in 47.5% of subjects with AA. A

Silent hepatic lesions detected with computed tomography in aplastic anemia patients administered androgens for a long period

International Nuclear Information System (INIS)

Yamagishi, Morihisa; Hiraoka, Atsunobu; Uchino, Haruto.

1982-01-01

Macroscopic liver lesions were investigated with the use of computed tomography (CT) and radionuclide imaging (RN) in 15 aplastic anemia patients who were administered anabolic steroids for over one year and who showed no apparent physical and biochemical sign of liver tumor. In 3 patients, CT scans showed radiolucent areas in the liver. Contrast enhancements revealed these lesions to be well vascularized, suggesting they were not cysts but probably tumors. RN imaging could not demonstrate any definite space occupying lesions. Total dose of AS administered to each of the three patients exceeded 30,000 mg. It was felt that attention should be paid to the possible development of hepatic tumor when the dose of AS administered exceeds 30,000 mg. (author)

Single dose total lymphoid irradiation combined with cyclophosphamide as immunosuppression for human marrow transplantation in aplastic anemia

International Nuclear Information System (INIS)

Kim, T.H.; Kersey, J.H.; Khan, F.M.; Sewchand, W.; Ramsey, N.; Krivit, W.; Coccia, P.; Nesbit, M.E.; Levitt, S.H.

1979-01-01

Six patients with aplastic anemia underwent bone marrow transplantation following conditioning with high dose cyclophosphamide and single dose total lymphoid irradiation with 750 rad, 26 rad/min at the midplane of the patient. They all received bone marrow from human leukocyte antigens/mixed lymphocyte culture (HLA/MLC) matched siblings. Five of 6 patients were alive without complications at 12, 11, 7, 4 and 4 months respectively. The remaining patient died from sepis which he had prior to transplantation. There were no graft rejection, graft-vs-Host Disease (GVHD) or interstitial pneumonitis among these patients. The procedure was well tolerated with minimal side effects. The results will be compared with those of groups whose bone marrow was previously transplanted with different immunosuppressive methods

Silent hepatic lesions detected with computed tomography in aplastic anemia patients administered androgens for a long period

Energy Technology Data Exchange (ETDEWEB)

Yamagishi, Morihisa (Shiga Univ., Otsu (Japan)); Hiraoka, Atsumobu; Uchino, Haruto

1982-07-01

Macroscopic liver lesions were investigated with the use of computed tomography (CT) and radionuclide imaging (RN) in 15 aplastic anemia patients who were administered anabolic steroids for over one year and who showed no apparent physical and biochemical sign of liver tumor. In 3 patients, CT scans showed radiolucent areas in the liver. Contrast enhancements revealed these lesions to be well vascularized, suggesting they were not cysts but probably tumors. RN imaging could not demonstrate any definite space occupying lesions. Total dose of AS administered to each of the three patients exceeded 30,000 mg. It was felt that attention should be paid to the possible development of hepatic tumor when the dose of AS administered exceeds 30,000 mg.

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

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Milačić Nena

2015-07-01

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.

A composite mouse model of aplastic anemia complicated with iron overload.

Science.gov (United States)

Wu, Dijiong; Wen, Xiaowen; Liu, Wenbin; Xu, Linlong; Ye, Baodong; Zhou, Yuhong

2018-02-01

Iron overload is commonly encountered during the course of aplastic anemia (AA), but no composite animal model has been developed yet, which hinders drug research. In the present study, the optimal dosage and duration of intraperitoneal iron dextran injection for the development of an iron overload model in mice were explored. A composite model of AA was successfully established on the principle of immune-mediated bone marrow failure. Liver volume, peripheral hemogram, bone marrow pathology, serum iron, serum ferritin, pathological iron deposition in multiple organs (liver, bone marrow, spleen), liver hepcidin, and bone morphogenetic protein 6 (BMP6), SMAD family member 4 (SMAD4) and transferrin receptor 2 (TfR2) mRNA expression levels were compared among the normal control, AA, iron overload and composite model groups to validate the composite model, and explore the pathogenesis and features of iron overload in this model. The results indicated marked increases in iron deposits, with significantly increased liver/body weight ratios as well as serum iron and ferritin in the iron overload and composite model groups as compared with the normal control and AA groups (Poverload and AA was successfully established, and AA was indicated to possibly have a critical role in abnormal iron metabolism, which promoted the development of iron deposits.

Short telomeres: from dyskeratosis congenita to sporadic aplastic anemia and malignancy.

Science.gov (United States)

Gramatges, Maria M; Bertuch, Alison A

2013-12-01

Telomeres are DNA-protein structures that form a protective cap on chromosome ends. As such, they prevent the natural ends of linear chromosomes from being subjected to DNA repair activities that would result in telomere fusion, degradation, or recombination. Both the DNA and protein components of the telomere are required for this essential function, because insufficient telomeric DNA length, loss of the terminal telomeric DNA structure, or deficiency of key telomere-associated factors may elicit a DNA damage response and result in cellular senescence or apoptosis. In the setting of failed checkpoint mechanisms, such DNA-protein defects can also lead to genomic instability through telomere fusions or recombination. Thus, as shown in both model systems and in humans, defects in telomere biology are implicated in cellular and organismal aging as well as in tumorigenesis. Bone marrow failure and malignancy are 2 life-threatening disease manifestations in the inherited telomere biology disorder dyskeratosis congenita. We provide an overview of basic telomere structure and maintenance. We outline the telomere biology defects observed in dyskeratosis congenita, focusing on recent discoveries in this field. Last, we review the evidence of how telomere biology may impact sporadic aplastic anemia and the risk for various cancers. Copyright © 2013 Mosby, Inc. All rights reserved.

Bone marrow scintigraphy using 111Indium chloride in patients with aplastic anemia

International Nuclear Information System (INIS)

Mabuchi, Nobuhisa; Kumano, Machiko; Matsumoto, Fumiko; Arita, Shigehiro; Nakagawa, Kenichi; Fujii, Koichi; Yoshioka, Hiroyasu; Hamada, Tatsumi; Ishida, Osamu

1987-01-01

Bone marrow scintigraphy using 111 Indium chloride ( 111 In-chloride) was performed in 18 patients with aplastic anemia. The scintigrams were taken 48 hours after an intravenous injection of 111 In-chloride 3 mCi. The distribution patterns on scintigram were classified into 5 types: Type I (4 cases) showed no accumulation, Type II (6 cases) showed low accumulation in usual bone marrow sites. Type III (7 cases) showed island-like distribution in bone marrow sites. Type IV, although no case was included in the 18 patients, shows uneven distribution between pelvis and sternum or vertebrae. Type V (one case) showed almost normal accumulation in usual bone marrow sites. Bone marrow uptake of 111 In-chloride correlated well with the cellularity of bone marrow. There was a tendency for the cases of markedly increased saturated iron-binding capacity to show increased renal activity. In type III, both the percentage of cases who had been treated and the count of reticulocytes were higher than those in the other types, which suggested that island-like distribution on scintigram showed the regeneration responded to the therapy, and related to the erythropoietic function. (author)

Diagnosis and Treatment of Community-Acquired Pneumonia in Children

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I.A. Karymdzhanov

2016-02-01

The initial antibiotic therapy of community-acquired pneumonia is carried out empirically. In the treatment of severe community-acquired pneumonia in children from 2 months to 5 years, the drug of choice is amoxicillin orally. Macrolides are the drugs of choice for children aged 5 to 16 years. In severe pneumonia, drugs of choice are amoxicillin clavulanate, 2nd–4th generation cephalosporins. In general, the duration of antibiotic therapy in the community-acquired pneumonia caused by typical bacteria is 7–10 days, by atypical bacteria — 10–14 days. In the real clinical practice, the errors associated with the choice of drug, route of administration, dosage, regimen of application, length of treatment are frequent during antibacterial therapy.

Investigating the Acquisition, Generalization, and Emergence of Untrained Verbal Operants for Mands Acquired Using the Picture Exchange Communication System in Adults with Severe Developmental Disabilities

Science.gov (United States)

Ziomek, M. M.; Rehfeldt, R. A.

2008-01-01

This study compared the total amount of training time and total number of trial blocks for individuals with severe developmental disabilities to acquire mands under control of unconditioned establishing operations and mands under control of transitive conditioned establishing operations for manual sign and for the Picture Exchange Communication…

Non-ST Elevation Myocardial Infarction and Severe Peripheral Artery Disease in a 20-Year-Old with Perinatally Acquired Human Immunodeficiency Virus Infection

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Purva Sharma

2018-01-01

Full Text Available Human immunodeficiency virus (HIV infection confers an increased risk of cardiovascular disease, including acute coronary syndrome (ACS. Patients with perinatally acquired HIV may be at increased risk due to the viral infection itself and exposure to HAART in utero or as part of treatment. A 20-year-old female with transplacentally acquired HIV infection presented with symptoms of transient aphasia, headache, palpitations, and blurry vision. She was admitted for hypertensive emergency with blood pressure 203/100 mmHg. Within a few hours, she complained of typical chest pain, and ECG showed marked ST depression. Troponin I levels escalated from 0.115 to 10.8. She underwent coronary angiogram showing 95% stenosis of the right coronary artery (RCA and severe peripheral arterial disease including total occlusion of both common iliacs and 95% infrarenal aortic stenosis with collateral circulation. She underwent successful percutaneous intervention with a drug-eluting stent to the mid-RCA. Patients with HIV are at increased risk for cardiovascular disease. Of these, coronary artery disease is one of the most critical complications of HIV. Perinatally acquired HIV infection can be a high-risk factor for cardiovascular disease. A high degree of suspicion is warranted in such patients, especially if they are noncompliant to their ART.

Studies on the clinical usefulness of the 111indium chloride bone marrow scintigraphy in patients with aplastic anemia

International Nuclear Information System (INIS)

Ino, Teruo; Ito, Naoya; Takeda, Nobuhide

1985-01-01

111 Indium chloride ( 111 In-Cl 3 ) appears to be a radionuclide appropriate for a bone marrow scanning because of its physical and chemical characteristics; it is, like iron, bound to and transported by transferrin and it has a photopeak suitable for scintigraphy. This study was undertaken to assess the bone marrow function by 111 In-Cl 3 bone marrow scintigraphy in patients with asplastic anemia. Bone marrow scintigraphy was performed in 21 patients with aplastic anemia with varying degrees of severity including 15 who had no previous treatments. The scintigrams were taken 48 hours after intravenous injection of 111 MBq(3 mCi) of 111 In-Cl 3 . All of them showed various degrees of reduction in uptake of 111 In-Cl 3 by the marrow. The abnormal scan grade was classified into 3 types: Type I, non-accumulation (5); Type II, low-accumulation (15); Type III, uneven accumulation (1). In type III, degree of radioisotope accumulation between the sternum and the ilium was different, and this difference correlated with that of bone marrow cellularity. In 2 patients of type II, hematological improvement occurred after treatment, which was also accompanied by simultaneous increase of 111 In-Cl 3 uptake by the marrow. These findings suggested that the degree of radioisotope accumulation in the marrow reflects that of bone marrow cellularity. Type I patients were clinically severe and poor in prognosis as compared to type II and type III patients; four out of 5 died within 2 months. Bone marrow transplantation may be the treatment of choice in type I patients when feasible. (author)

Increased Bone Marrow (BM) Plasma Level of Soluble CD30 and Correlations with BM Plasma Level of Interferon (IFN)-γ, CD4/CD8 T-Cell Ratio and Disease Severity in Aplastic Anemia

Science.gov (United States)

Shi, Jun; Ge, Meili; Li, Xingxin; Shao, Yingqi; Yao, Jianfeng; Zheng, Yizhou

2014-01-01

Idiopathic aplastic anemia (AA) is an immune-mediated bone marrow failure syndrome. Immune abnormalities such as decreased lymphocyte counts, inverted CD4/CD8 T-cell ratio and increased IFN-γ-producing T cells have been found in AA. CD30, a surface protein belonging to the tumor necrosis factor receptor family and releasing from cell surface as a soluble form (sCD30) after activation, marks a subset of activated T cells secreting IFN-γ when exposed to allogeneic antigens. Our study found elevated BM plasma levels of sCD30 in patients with SAA, which were closely correlated with disease severity, including absolute lymphocyte count (ALC) and absolute netrophil count (ANC). We also noted that sCD30 levels were positively correlated with plasma IFN-γ levels and CD4/CD8 T-cell ratio in patients with SAA. In order to explain these phenomena, we stimulated T cells with alloantigen in vitro and found that CD30+ T cells were the major source of IFN-γ, and induced CD30+ T cells from patients with SAA produced significantly more IFN-γ than that from healthy individuals. In addition, increased proportion of CD8+ T cells in AA showed enhanced allogeneic response by the fact that they expressed more CD30 during allogeneic stimulation. sCD30 levels decreased in patients responded to immunosuppressive therapy. In conclusion, elevated BM plasma levels of sCD30 reflected the enhanced CD30+ T cell-mediated immune response in SAA. CD30 as a molecular marker that transiently expresses on IFN-γ-producing T cells, may participate in mediating bone marrow failure in AA, which also can facilitate our understanding of AA pathogenesis to identify new therapeutic targets. PMID:25383872

Role of marrow architecture and stromal cells in the recovery process of aplastic marrow of lethally irradiated rats parabiosed with healthy litter mates

International Nuclear Information System (INIS)

Hayashi, K.; Kagawa, K.; Awai, M.; Irino, S.

1986-01-01

Bone marrow aplasia was induced in rats by whole body lethal irradiation (1,000 rads by x-ray), and rats died of irradiation injury within 7 days. Correlative studies at light (LM), transmission (TEM) and scanning electron microscopy (SEM) demonstrated swelling of endothelial and reticular cells and hemorrhage due to detachment of sinus endothelial cells on days 1 and 2. With time, structural recovery occurred without hemopoietic recovery. Reticular cells developed small intracytoplasmic lipid droplets on days 3 and 4. This resulted in fatty aplastic marrow within 7 days. On the other hand, in the marrow of irradiated rats parabiosed with healthy mates by aortic anastomosis, hemopoiesis was initiated by adhesion of nucleated blood cells to fine cytoplasmic pseudopods of fat-stored cells on days 1 and 2 after parabiosis. On days 3 to 5, reticular cells with large lipid droplets and fine pseudopods increased, then hemopoietic foci became clear and extensive. On day 8 after parabiosis, the aplastic bone marrow recovered completely both its structure and hemopoietic activity. Thus, hemopoietic recovery in lethally irradiated marrow begins with recovery of vascular endothelial cells, re-establishment of sinusoidal structure, and morphological and functional recoveries of reticular cells from fat-storage cells by releasing intracytoplasmic lipid droplets. Marrow stromal cells, namely reticular, fat-storage and fibroblastoid cells, share a common cellular origin, and regain their structure and function when fat-storage cells and fibroid cells are placed in contact with hemopoietic precursor cells

Severe virus associated community acquired pneumonia: predictors of lethality

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T. O. Pertseva

2016-06-01

Full Text Available Despite the fact that the influenza virus pathogenicity factors have been well studied in vitro, in vivo lack is presented in understanding of the those risk factors, objective and laboratory parameters, which related most of all to the fatal virus-associated community-aquired pneumonia (CAP. That is why the purpose of the study was to study the clinical and laboratory characteristics of patients with severe virus-associated CAP during the 2015–2016 influenza epidemic and their role as predictors of patients’ mortality. To do this, patients with severe virus-associated CAP were examined. They were divided into 2 groups depending on the outcome of treatment: 1st- deaths from the virus-associated severe CAP and 2nd - patients with successful treatment of the severe virus-associated CAP. Special statistical method was used – one-dimensional analysis of variance to compare individual parameters between the two groups of patients (surviving and deceased. Pearson χ2 test (contingency table was used for categorical variables. Factors that were significant predictors of mortality as a result of univariate analysis were tested using multifactorial analysis using logistic regression. In the final model, each parameter must have had a significant impact on mortality. It was found that risk factors for death in patients with severe virus-associated CAP according to univariate analysis were: presence of obesity, disorders of consciousness, BH≥35 min, SaO2<80%, PaO2<50 mm Hg, mmHg PaCO2 ≥50 mmHg during hospitalization. Independent predictors of mortality according to the logistic regression are the presence of obesity, disorders of consciousness, PaO2<50 mm Hg, mmHg PaCO2 ≥50 mmHg. Given that among clinical and laboratory parameters key parameters that significantly influence the outcome, are indicators of the severity of hypoxia and hypoxemia, a major step in determining the severity of the patients with virus-associated severe emergency is

Outcomes of allogeneic stem cell transplantation in patients with paroxysmal nocturnal hemoglobinuria with or without aplastic anemia.

Science.gov (United States)

Lee, Sung-Eun; Park, Sung Soo; Jeon, Young-Woo; Yoon, Jae-Ho; Cho, Byung-Sik; Eom, Ki-Sung; Kim, Yoo-Jin; Lee, Seok; Min, Chang-Ki; Kim, Hee-Je; Cho, Seok-Goo; Kim, Dong-Wook; Min, Woo-Sung; Lee, Jong Wook

2017-10-01

The aim of this study was to evaluate the long-term outcomes of allogeneic stem cell transplantation (SCT) in patients with paroxysmal nocturnal hemoglobinuria (PNH) with or without aplastic anemia (AA). A total of 33 patients with PNH clones who underwent allogeneic SCT were analyzed. After a median follow-up of 57 months (range, 6.0-151.3), the 5-year estimated overall survival rate was 87.9±5.7%. Four patients died of transplant-related mortality (TRM). With the exception of one patient with early TRM, 32 patients were engrafted. Two patients who had developed delayed GF received a second transplant and recovered. The cumulative incidences of acute graft-vs-host disease (GVHD) (≥grade II) and chronic GVHD (≥moderate) were 27.3±7.9% and 18.7±7.0%, respectively. Twenty-one patients receiving SCT with reduced-intensity conditioning (RIC) had available follow-up data for PNH cell population for the first 6 months post-transplant. Analysis of these data revealed that the PNH clones disappeared within approximately 2 months. RIC regimen was sufficient to eradicate PNH clones with sustained donor-type engraftment after allogeneic SCT. Therefore, application of allogeneic SCT with RIC should be considered in patients with PNH, in accordance with the severity of the underlying bone marrow failure. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

[Validation of the Pneumonia Severity Index for hospitalizing patients with community-acquired pneumonia].

Science.gov (United States)

Querol-Ribelles, José M; Tenías, José M; Querol-Borrás, José M; González-Granda, Damiana; Hernández, Manuel; Ferreruela, Rosa; Martínez, Isidoro

2004-04-10

Our main objective was to assess the utility of the Pneumonia Severity Index (PSI) to decide the site of care home or hospital of patients with community-acquired pneumonia (CAP). All CAP patients who came to the emergency department from 1 January to 31 December, 2000, were prospectively assessed with a protocol based on the PSI and additional admission criteria applied to classes I, II and III. Mortality within 30 days and poor outcome were used as endpoints. We tested the diagnostic efficacy of the PSI scale in predicting mortality or unfavourable events by calculating the area below the ROC curve. Of the 243 CAP patients included, 124 (51%) belonged to classes I, II and III, and 119 (49%) belonged to classes IV and V. One hundred and fifty six (64%) patients were admitted. Fifteen (6.2%) patients died, all of them belonging to classes IV and V. Forty four (18%) patients showed a poor outcome. Only one patient who was initially sent home had a poor outcome. The prognostic value of the PSI scale to predict mortality (ROC = 0.92; CI 95%, 0.88-0.95) was high. Our results confirm that the PSI scale is a good prognostic index in clinical practice for predicting mortality due to CAP. In order to use the PSI to decide the site of care of patients with CAP, not only the score obtained but also additional factors should be taken into account.

T cell responses in senior patients with community-acquired pneumonia related to disease severity.

Science.gov (United States)

Bian, Lu-Qin; Bi, Ying; Zhou, Shao-Wei; Chen, Zi-Dan; Wen, Jun; Shi, Jin; Mao, Ling; Wang, Ling

2017-12-01

Senior individuals older than 65 years of age are at a disproportionally higher risk of developing pneumonia. Impaired capacity to defend against airway infections may be one of the reasons. It is generally believed that weaker regulatory T cell responses may be beneficial to host defense against pathogens. In senior patients with community-acquired bacterial pneumonia, we investigated the frequencies and functions of regulatory T cells. Interestingly, we found that compared to age- and sex-matched healthy controls, senior pneumonia patients presented lower frequencies of Foxp3-expressing and Helios-expressing CD4 + T cells. The quantity of Foxp3 and Helios being expressed, measured by their mRNA transcription levels, was also lower in CD4 + T cells from pneumonia patients. Furthermore, following TCR and TGF-β stimulation, pneumonia patients presented impaired capacity to upregulate Foxp3 and Helios. Functional analyses revealed that CD4 + T cells from pneumonia patients secreted lower amounts of IL-10 and TGF-β, two cytokines critical to regulatory T cell-mediated suppression. Also, the expression of granzyme B and perforin, which were cytolytic molecules potentially utilized by regulatory T cells to mediate the elimination of antigen-presenting cells and effector T cells, were reduced in CD4 + CD25 + T cells from senior pneumonia patients. In addition, the CD4 + CD25 + T cells from senior pneumonia patients presented reduced capacity to suppress effector CD4 + and CD8 + T cell proliferation. Moreover, the value of pneumonia severity index was inversely correlated with several parameters of regulatory T cell function. Together, our results demonstrated that senior pneumonia patients presented a counterintuitive impairment in regulatory T cell responses that was associated with worse prognosis. Copyright © 2017 Elsevier Inc. All rights reserved.

C-reactive protein, procalcitonin, clinical pulmonary infection score, and pneumonia severity scores in nursing home acquired pneumonia.

Science.gov (United States)

Porfyridis, Ilias; Georgiadis, Georgios; Vogazianos, Paris; Mitis, Georgios; Georgiou, Andreas

2014-04-01

Patients with nursing home acquired pneumonia (NHAP) present a distinct group of lower respiratory track infections with different risk factors, clinical presentation, and mortality rates. To evaluate the diagnostic value of clinical pulmonary infection score (CPIS), C-reactive protein, and procalcitonin and to compare the accuracy of pneumonia severity scores (confusion, urea nitrogen, breathing frequency, blood pressure, ≥ 65 y of age [CURB-65]; pneumonia severity index; NHAP index; systolic blood pressure, multilobar involvement, albumin, breathing frequency, tachycardia, confusion, oxygen, arterial pH [SMART-COP]; and systolic blood pressure, oxygen, age > 65 y, breathing frequency [SOAR]) in predicting in-patient mortality from NHAP. Nursing home residents admitted to the hospital with acute respiratory illness were enrolled in the study. Subjects were classified as having NHAP (Group A) or other pulmonary disorders (Group B). Clinical, imaging, and laboratory data were assessed to compute CPIS and severity scores. C-reactive protein and procalcitonin were measured by immunonephelometry and immunoassay, respectively. Fifty-eight subjects were diagnosed with NHAP (Group A) and 29 with other pulmonary disorders (Group B). The mean C-reactive protein ± SD was 16.38 ± 8.6 mg/dL in Group A and 5.2 ± 5.6 mg/dL in Group B (P 1.1 ng/mL was an independent predictor of in-patient mortality. Of the pneumonia severity scores, CURB-65 showed greater accuracy in predicting in-patient mortality (area under the curve of 0.68, 95% CI 0.53-0.84, P = .06). CPIS, procalcitonin, and C-reactive protein are reliable for the diagnosis of NHAP. Procalcitonin and CURB-65 are accurate in predicting in-patient mortality in NHAP.

How Did Light Acquire a Velocity?

Science.gov (United States)

Lauginie, Pierre

2013-01-01

We discuss how light acquired a velocity through history, from the ancient Greeks to the early modern era. Combining abstract debates, models of light, practical needs, planned research and chance, this history illustrates several key points that should be brought out in science education.

Aldehyde dehydrogenase 2 in aplastic anemia, Fanconi anemia and hematopoietic stem cells.

Science.gov (United States)

Van Wassenhove, Lauren D; Mochly-Rosen, Daria; Weinberg, Kenneth I

2016-09-01

Maintenance of the hematopoietic stem cell (HSC) compartment depends on the ability to metabolize exogenously and endogenously generated toxins, and to repair cellular damage caused by such toxins. Reactive aldehydes have been demonstrated to cause specific genotoxic injury, namely DNA interstrand cross-links. Aldehyde dehydrogenase 2 (ALDH2) is a member of a 19 isoenzyme ALDH family with different substrate specificities, subcellular localization, and patterns of expression. ALDH2 is localized in mitochondria and is essential for the metabolism of acetaldehyde, thereby placing it directly downstream of ethanol metabolism. Deficiency in ALDH2 expression and function are caused by a single nucleotide substitution and resulting amino acid change, called ALDH2*2. This genetic polymorphism affects 35-45% of East Asians (about ~560 million people), and causes the well-known Asian flushing syndrome, which results in disulfiram-like reactions after ethanol consumption. Recently, the ALDH2*2 genotype has been found to be associated with marrow failure, with both an increased risk of sporadic aplastic anemia and more rapid progression of Fanconi anemia. This review discusses the unexpected interrelationship between aldehydes, ALDH2 and hematopoietic stem cell biology, and in particular its relationship to Fanconi anemia. Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

Bone marrow scintigraphy using /sup 111/Indium chloride in patients with aplastic anemia

Energy Technology Data Exchange (ETDEWEB)

Mabuchi, Nobuhisa; Kumano, Machiko; Matsumoto, Fumiko; Arita, Shigehiro; Nakagawa, Kenichi; Fujii, Koichi; Yoshioka, Hiroyasu; Hamada, Tatsumi; Ishida, Osamu

1987-12-01

Bone marrow scintigraphy using /sup 111/Indium chloride (/sup 111/In-chloride) was performed in 18 patients with aplastic anemia. The scintigrams were taken 48 hours after an intravenous injection of /sup 111/In-chloride 3 mCi. The distribution patterns on scintigram were classified into 5 types: Type I (4 cases) showed no accumulation, Type II (6 cases) showed low accumulation in usual bone marrow sites. Type III (7 cases) showed island-like distribution in bone marrow sites. Type IV, although no case was included in the 18 patients, shows uneven distribution between pelvis and sternum or vertebrae. Type V (one case) showed almost normal accumulation in usual bone marrow sites. Bone marrow uptake of /sup 111/In-chloride correlated well with the cellularity of bone marrow. There was a tendency for the cases of markedly increased saturated iron-binding capacity to show increased renal activity. In type III, both the percentage of cases who had been treated and the count of reticulocytes were higher than those in the other types, which suggested that island-like distribution on scintigram showed the regeneration responded to the therapy, and related to the erythropoietic function.

Principles of Antibiotic Management of Community-Acquired Pneumonia.

Science.gov (United States)

Bender, Michael T; Niederman, Michael S

2016-12-01

Community-acquired pneumonia (CAP) encompasses a broad spectrum of disease severity and may require outpatient, inpatient, or intensive care management. Successful treatment hinges on expedient delivery of appropriate antibiotic therapy tailored to both the likely offending pathogens and the severity of disease. This review summarizes key principles in starting treatment and provides recommended empiric therapy regimens for each site of care. In addition, we discuss the antimicrobial and anti-inflammatory role macrolides play in CAP, as well as specific information for managing individual CAP pathogens such as community-acquired methicillin-resistant Staphylococcus aureus and drug-resistant Streptococcus pneumoniae . We also examine several novel antibiotics being developed for CAP and review the evidence guiding duration of therapy and current best practices for the transition of hospitalized patients from intravenous antibiotics to oral therapy. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

[The variation and clinical significance of paroxysmal nocturnal hemoglobinuria clone in patients with aplastic anemia before and after immunosuppressive therapy].

Science.gov (United States)

Sun, Ying-xin; Zhu, Ming-qing; He, Guang-sheng; Wang, Xiu-li; Fang, Bao-zhi; Lu, Cong; Liu, Zhen-zhen; Wu, Qian; Yang, Yong; Wu, De-pei; Sun, Ai-ning

2013-07-01

To evaluate the evolution of paroxysmal nocturnal hemoglobinuria (PNH) clone and its clinical significance before and after immunosuppressive therapy (IST) in patients with aplastic anemia (AA). A total of 186 patients diagnosed as AA were enrolled in this study. Among them, 55 patients were diagnosed as severe AA (SAA) and treated with cyclosporine (CsA) plus anti-thymocyte globulin (ATG), 131 were diagnosed as non SAA (NSAA) and treated with CsA alone. All patients were screened for PNH clone by flow cytometry before treatment and followed up for 18-76 months, with a median time of 22 months. Positive PNH clones were detected in 10 SAA (18.9%) patients, significantly more than that of NSAA group [9 patients (7.4%), t = 5.041, P = 0.025]. The proportions of PNH clones in SAA group at 6, 12, 24 and > 24 months were 13.38%, 14.88%, 20.00% and 18.85%, respectively, also significantly higher than those of NSAA patients (5.67%, 5.31%, 5.47% and 9.08%, all P values clone was positive or negative. PNH clone are detectable in AA patients either treated with ATG plus CsA or CsA alone, and more significant by ATG plus CsA. Whether PNH clone occurred before or after IST does not affect the therapeutic efficacy.

Botulinum toxin in the management of sialorrhoea in acquired brain injury

LENUS (Irish Health Repository)

Carroll, A

2016-06-01

Sialorrhoea as a consequence of severe acquired brain injury can significantly negatively impact on quality of life. Medications used in its management have many side effects which can cause problems in the severely disabled. Botulinum toxin is an effective treatment of sialorrhoea in a number of neurological conditions but may also have a role to play in the management of sialorrhoea following severe ABI. We report on 4 cases of sialorrhoea following acquired brain injury causing a variety of problems, whose parotid glands were injected with Botulinum toxin type A (Dysport) 50mu each, under ultrasound guidance. All cases had a clinically and statistically significant reduction in drooling as measured by the teacher drooling scale (p=0.005) and carers Visual Analogue Scale (p=0.012). There were no side effects reported. Botulinum toxin is an effective treatment for sialorrhoea associated with acquired brain injury.

Family function and its relationship to injury severity and psychiatric outcome in children with acquired brain injury: a systematized review.

Science.gov (United States)

Lax Pericall, Maria Teresa; Taylor, Eric

2014-01-01

The psychological and psychiatric outcome of children with acquired brain injury is influenced by many variables. A review was undertaken to clarify the contribution of family function, how it relates to injury severity, and what particular aspects of family function influence psychological outcome in this group. A systematized review of the literature of studies published between 1970 and 2012 from OvidMedline, PsychoInfo, PsycARTICLES, and Cochrane was undertaken focusing on family function, injury severity, and psychiatric outcome. Thirty-six papers met the inclusion criteria. Injury severity was linked to the development of organic personality change. Family function before injury, measured by the Family Assessment Device or the Clinical Rating Scale, had a statistically significant effect on general psychological functioning in six out of eight studies. Family function had a significant effect for oppositional defiant disorder and secondary attention-deficit-hyperactivity disorder. The effects of family function may differ depending on the age of the child and the severity of the injury. Some styles of parenting moderated recovery. After injury, family function was related to the child's contemporaneous psychiatric symptoms. The level of evidence for these papers was 3 or 4 (Oxford Centre for Evidence-based Medicine criteria). Screening for some aspects of family functioning before injury and family function during the rehabilitation phase may identify children at risk of psychiatric disorders. © 2013 Mac Keith Press.

The impact of blood glucose on community-acquired pneumonia

DEFF Research Database (Denmark)

Jensen, Andreas Vestergaard; Egelund, Gertrud Baunbæk; Andersen, Stine Bang

2017-01-01

Hyperglycaemia is common in patients with community-acquired pneumonia (CAP) and is a predictor of severe outcomes. Data are scarce regarding whether this association is affected by diabetes mellitus (DM) and also regarding its importance for severe outcomes in hospital. We determined the impact...

Experimental immunologically mediated aplastic anemia (AA) in mice: cyclosporin A fails to protect against AA

International Nuclear Information System (INIS)

Knospe, W.H.; Steinberg, D.; Gratwohl, A.; Speck, B.

1984-01-01

Immunologically mediated aplastic anemia (AA) in mice was induced by the i.v. injection of 10(7) lymph node cells (LNC) from H-2k identical but Mls mismatched CBA/J donor mice into previously irradiated (600 rad total body gamma) C3H/HeJ mice. Cyclosporin A (CsA), 25 mg/kg, was administered subcutaneously from day -1 to day 30. Control mice included C3H/HeJ mice which received 600 rad alone, C3H/HeJ mice which received 600 rad plus CsA as above, and C3H/HeJ mice which received 600 rad total body irradiation followed by 10(7) LNC from CBA/J donors. CsA failed to prevent lethal AA. These results suggest that the pathogenetic mechanisms operating in immunologically mediated AA differ from the mechanisms operating in rodents transplanted with allogeneically mismatched marrow or spleen cells which develop graft-versus-host disease. The results are consistent with a non-T cell-dependent mechanism causing the AA

The usefulness of the surgical knowledge and skills acquired via the university curriculum for doctors' medical practice several years after graduation.

Science.gov (United States)

Zyluk, Andrzej; Puchalski, Piotr; Szlosser, Zbigniew

2015-01-01

Teaching surgery during university curriculum comprises transferring theoretical knowledge traditionally and simultaneously acquiring manual skills, i.e., suturing, stitch removal, limb immobilization, catheterization, and assisting operations. Observations of doctors several years after graduation led to the reflection that teachers' ideas about surgical knowledge and skills that are useful in daily practice frequently fail to meet the facts of the case. The objective of this study was to determine which part of the surgical knowledge and skills taught via the university surgical curriculum proved to be useful in the daily practice of young doctors. A custom-made questionnaire was designed and mailed to 200 randomly chosen doctors who had graduated from the medical faculty at the authors' university 5 to 6 years previously. The questionnaire comprised 9 items concerning the knowledge and skills that proved to be the most useful in participants' daily practice, regardless of their specialty. A total of 64 completed questionnaires were returned (32% of 200 sent) and were the subject of analysis. The most useful knowledge in daily practice was that acquired from general surgery, followed by oncological and vascular surgery. The most useful was knowledge about the rational interpretation of clinical symptoms and signs acquired from examination of the patient, followed by arriving at an accurate diagnosis through logical analysis, and next developing "oncological sensitivity" to diagnosing neoplasms. The most effective teaching model was specialized outpatient clinic rounds, followed by training manual skills on a model and classical ward-round teaching. The most frequently learned (acquired) manual skills were removal of stitches, rectal examination, and examination of the abdomen. Of these skills, the most useful in daily practice appeared to be removal of stitches, catheterization of the urinary bladder, and wound suturing. Learning and practicing manual skills

Cytogenetic study is not essential in patients with aplastic anemia

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Dutta, Atreyee; De, Rajib; Dolai, Tuphan K; Mitra, Pradip K; Halder, Ajanta

2017-01-01

Depending on contemporary treatment approach of aggressive immunosuppression, Aplastic Anemia (AA) is caused by immunological destruction of otherwise normal hematopoietic stem cells. The aim was to summarize the cytogenetic abnormalities in AA patients and the frequency of Fanconi Anemia (FA) in morphologically normal AA patients in eastern India. Ethical clearances were obtained from both institutions involved in this study. Out of 72800 patients attending the outpatient department, 520 pancytopenia patients were screened for AA after Bone marrow (BM) aspiration and biopsy. Samples were collected from 117 cases in 3 phases. 51 peripheral venous blood (PVB) samples in the first phase, 19 BM & PVB paired samples in the second phase and 47 BM samples in third phase were collected followed by leukocyte and/or BM stem cell culture. Next GTG banding and karyotyping were performed. PVB was collected from 63 (< 50 years) AA patients and stress cytogenetics was done to diagnose FA. In the first phase of the study, out of 51 PVB samples, 1 (1.96%) showed a unique chromosomal abnormality, i.e. 45,XY,rob(14:21)(p10:q10)[20]. In the second phase of study, among 19 BM & PVB paired samples, 1 (5.26%) showed abnormal karyotype i.e. 45,X,-Y[3]/46,XY[47]. In the third phase of the study, 47 BM samples showed normal karyotype. Only 6 (9.52%) cases were found positive for stress cytogenetics. A negligible percentage showing cytogenetic abnormality in such a considerable number of AA cases indicates that routine cytogenetic analysis of AA patient is not essential. A significant percentage was positive for stress cytogenetics; suggestive for FA, even the patients were morphologically normal. PMID:29181263

Cytogenetic study is not essential in patients with aplastic anemia.

Science.gov (United States)

Dutta, Atreyee; De, Rajib; Dolai, Tuphan K; Mitra, Pradip K; Halder, Ajanta

2017-01-01

Depending on contemporary treatment approach of aggressive immunosuppression, Aplastic Anemia (AA) is caused by immunological destruction of otherwise normal hematopoietic stem cells. The aim was to summarize the cytogenetic abnormalities in AA patients and the frequency of Fanconi Anemia (FA) in morphologically normal AA patients in eastern India. Ethical clearances were obtained from both institutions involved in this study. Out of 72800 patients attending the outpatient department, 520 pancytopenia patients were screened for AA after Bone marrow (BM) aspiration and biopsy. Samples were collected from 117 cases in 3 phases. 51 peripheral venous blood (PVB) samples in the first phase, 19 BM & PVB paired samples in the second phase and 47 BM samples in third phase were collected followed by leukocyte and/or BM stem cell culture. Next GTG banding and karyotyping were performed. PVB was collected from 63 (cytogenetics was done to diagnose FA. In the first phase of the study, out of 51 PVB samples, 1 (1.96%) showed a unique chromosomal abnormality, i.e. 45,XY,rob(14:21)(p10:q10)[20]. In the second phase of study, among 19 BM & PVB paired samples, 1 (5.26%) showed abnormal karyotype i.e. 45,X,-Y[3]/46,XY[47]. In the third phase of the study, 47 BM samples showed normal karyotype. Only 6 (9.52%) cases were found positive for stress cytogenetics. A negligible percentage showing cytogenetic abnormality in such a considerable number of AA cases indicates that routine cytogenetic analysis of AA patient is not essential. A significant percentage was positive for stress cytogenetics; suggestive for FA, even the patients were morphologically normal.

Ezh2 regulates transcriptional and post-translational expression of T-bet and promotes Th1 cell responses mediating aplastic anemia in mice1

Science.gov (United States)

Tong, Qing; He, Shan; Xie, Fang; Mochizuki, Kazuhiro; Liu, Yongnian; Mochizuki, Izumi; Meng, Lijun; Sun, Hongxing; Zhang, Yanyun; Guo, Yajun; Hexner, Elizabeth; Zhang, Yi

2014-01-01

Acquired aplastic anemia (AA) is a potentially fatal bone marrow (BM) failure syndrome. IFN-γ-producing T helper (Th)1 CD4+ T cells mediate the immune destruction of hematopoietic cells, and are central to the pathogenesis. However, the molecular events that control the development of BM-destructive Th1 cells remain largely unknown. Ezh2 is a chromatin-modifying enzyme that regulates multiple cellular processes primarily by silencing gene expression. We recently reported that Ezh2 is crucial for inflammatory T cell responses after allogeneic BM transplantation. To elucidate whether Ezh2 mediates pathogenic Th1 responses in AA and the mechanism of Ezh2 action in regulating Th1 cells, we studied the effects of Ezh2 inhibition in CD4+ T cells using a mouse model of human AA. Conditionally deleting Ezh2 in mature T cells dramatically reduced the production of BM-destructive Th1 cells in vivo, decreased BM-infiltrating Th1 cells, and rescued mice from BM failure. Ezh2 inhibition resulted in significant decrease in the expression of Tbx21 and Stat4 (which encode transcription factors T-bet and STAT4, respectively). Introduction of T-bet but not STAT4 into Ezh2-deficient T cells fully rescued their differentiation into Th1 cells mediating AA. Ezh2 bound to the Tbx21 promoter in Th1 cells, and directly activated Tbx21 transcription. Unexpectedly, Ezh2 was also required to prevent proteasome-mediated degradation of T-bet protein in Th1 cells. Our results identify T-bet as the transcriptional and post-translational Ezh2 target that acts together to generate BM-destructive Th1 cells, and highlight the therapeutic potential of Ezh2 inhibition in reducing AA and other autoimmune diseases. PMID:24760151

Blood responses under chronic low daily dose gamma irradiation: Pt. 1; Differential preclinical responses of irradiated male dogs in progression to either aplastic anemia or myeloproliferative disease

Energy Technology Data Exchange (ETDEWEB)

Seed, T.M.; Carnes, B.A.; Tolle, D.V.; Fritz, T.E. (Argonne National Lab., IL (USA))

1989-01-01

Male beagles chronically exposed to low daily doses of {sup 60}Co {gamma} rays show one of three hematopoietic patterns, which reflect three different distinctly responding subgroups: (1) low radioresistance with progressing aplastic anemia and shortened survival ({sup -S}-AA subgroup); (2) high radioresistance with a complex of progressing myeloproliferative disorders ({sup +}R-MPD group); or (3) high radioresistance with other nonMPD syndromes ({sup +}R-nonMPD group). Blood cell levels (granulocytes, monocytes, erythrocytes, lymphocytes, and platelets) were assessed and fitted to a flexible polynomial spline model. Results showed that relative to the overall magnitude of blood cell loss as well as to the maximum rate of suppression during the initial phase, the subgroups were generally ranked {sup -}S-AA >> {sup +}R-MPD > {sup +}R-nonMPD. Relative to the overall strength of the recovery response, the subgroups were generally ranked {sup +}R-MPD > {sup +}R-nonMPD >>> {sup -}S-AA. In terms of overall maintenance levels of circulating blood cells during the recovery phase, however, the {sup +}R-nonMPD subgroup consistently exhibited stronger responses than the {sup +}R-MPD subgroup. These results support our contention that selected subgroups of dogs have strong propensities to specific hematopathologies (i.e. aplastic anemia and myeloid leukemia) under chronic irradiation and that these pathology-prone animals exhibit a series of marked differential hematopoietic responses during early preclinical phases, which serve effectively to prognosticate subsequent pathological progression. (author).

Influence of genetic polymorphisms of IL23R, STAT3, IL12B, and STAT4 on the risk of aplastic anemia and the effect of immunosuppressive therapy.

Science.gov (United States)

Zhao, Li; Zhu, Huanling; Han, Bing; Wang, Lixin; Sun, Yuming; Lu, Xiaojun; Huang, Chunyan; Tan, Bin; Chen, Chunxia; Qin, Li

2018-04-01

Studies have suggested that IL-23/STAT3 and IL-12/STAT4 signaling pathways associate with aplastic anemia (AA) occurrence. Polymorphisms in pathway-related genes may contribute to AA risk. In the current study, we investigated the association between polymorphisms in genes of IL23R, STAT3, IL12B, and STAT4 and occurrence, severity, and immunosuppressive outcome of AA in the Han population in southwest China. In the current 164 AA cases and 211 controls study, we found T allele and TT genotype of rs7574865 were more frequent in the cases than that in the controls. In the additive model, individual carrying rs7574865 T allele demonstrated a 37% (OR (95% CI) = 1.37 (1.02-1.85), Pper = 0.036) increased AA risk. In the recessive model, carrier with rs7574865 TT genotype showed a 2.08-fold increased AA risk (OR (95% CI) = 2.08 (1.14-3.70), Pper = 0.017). Additionally, we showed that G allele and GG genotype of rs11209032 were more frequent in the 88 non-severe AA cases than that in the 76 severe AA ones. Our study also found G allele and GG genotype of rs11209032, and GG-genotype of rs744166 associated with the immunosuppressive therapy outcome in AA patients. Current study results support that functional STAT4 (rs7574865), IL23R (rs11209032), and STAT3 (rs744166) variants may associate with occurrence, severity, and immunosuppressive outcome of AA in the Han population in southwest China.

Possibilities of predicting of the non-severe community-acquired pneumonia outcomes in patients with type 2 diabetes mellitus or chronic heart failure

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O. S. Makharynska

2014-06-01

Full Text Available Aim. Сommunity-acquired pneumonia is life-threatening disease with level of fatal events in hospitals within 12-36 %. In turn, presence of congestive heart failure or type 2 diabetes increases the risk of adverse outcomes in patients with community-acquired pneumonia. Methods and results. In the modern world’s literature there are many models predicting clinical outcomes of community-acquired pneumonia, but none of them includes questionnaires data for such patients. In our study, we used two questionnaires: "The scale of assessment community-acquired pneumonia" R. el Moussaoui and CapSym-12. C Сonclusion. Using logistic regression, we have found statistically significant indices sample questionnaires prognostic opportunities which were used in this study to assess the health and dynamics of symptoms of community-acquired pneumonia and that allows us to predict the outcome community-acquired pneumonia.

Clinical, general, hemocoagulation and pathologicanatomical features of patients with moderate and severe community acquired pneumonia by the data of retrospective analysis

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Pertseva T.A.

2017-10-01

Full Text Available According to literature data, patients with community acquired pneumonia (CAP fall into several groups of unfavorable prognostic factors. Development of thrombotic complications is one of causes of mortality of hospitalized patients. In this case systemic inflammation, which is always present in moderate and severe CAP, is the starting mechanism of formation of disorders in the hemostasis system. The aim of our work was to determine anamnestic, clinical, laboratory and pathologic anatomical features in patients with CAP, taking into account markers of systemic inflammation and coagulogram indices, as well as predicting the occurrence of complications. In the course of the work, a retrospective analysis of 151 medical histories of hospitalized patients with CAP was made. We analyzed anthropometric indicators, complaints at the time of hospitalization, results of physical examination, results of the chest X-ray, clinical and laboratory indicators, microbiological sputum analysis and to assess the possibility of thrombotic complications a RAM scale was used. It was found that late asking for medical help, the presence of mixed infection, underestimation of the severity of condition and severe systemic inflammation increase the risk of lethal outcome in patients with CAP. Considering a high risk of thrombosis in patients with CAP, it is necessary to assess the risk of thrombotic complications with the help of special scales, as well as timely detection and correction of disorders from the hemostasis system.

Differences in microbiological profile between community-acquired, healthcare-associated and hospital-acquired infections.

Science.gov (United States)

Cardoso, Teresa; Ribeiro, Orquídea; Aragão, Irene; Costa-Pereira, Altamiro; Sarmento, António

2013-01-01

Microbiological profiles were analysed and compared for intra-abdominal, urinary, respiratory and bloodstream infections according to place of acquisition: community-acquired, with a separate analysis of healthcare-associated, and hospital-acquired. Prospective cohort study performed at a university tertiary care hospital over 1 year. Inclusion criteria were meeting the Centers for Disease Control definition of intra-abdominal, urinary, respiratory and bloodstream infections. A total of 1035 patients were included in the study. More than 25% of intra-abdominal infections were polymicrobial; multi-drug resistant gram-negatives were 38% in community-acquired, 50% in healthcare-associated and 57% in hospital-acquired. E. coli was the most prevalent among urinary infections: 69% in community-acquired, 56% in healthcare-associated and 26% in hospital-acquired; ESBL producers' pathogens were 10% in healthcare-associated and 3% in community-acquired and hospital-acquired. In respiratory infections Streptococcus pneumoniae was the most prevalent in community-acquired (54%) and MRSA in healthcare-associated (24%) and hospital-acquired (24%). A significant association was found between MRSA respiratory infection and hospitalization in the previous year (adjusted OR = 6.3), previous instrumentation (adjusted OR = 4.3) and previous antibiotic therapy (adjusted OR = 5.7); no cases were documented among patients without risk factors. Hospital mortality rate was 10% in community-acquired, 14% in healthcare-associated and 19% in hospital-acquired infection. This study shows that healthcare-associated has a different microbiologic profile than those from community or hospital acquired for the four main focus of infection. Knowledge of this fact is important because the existing guidelines for community-acquired are not entirely applicable for this group of patients.

Rhizomucor and Scedosporium Infection Post Hematopoietic Stem-Cell Transplant

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Dânia Sofia Marques

2011-01-01

Full Text Available Hematopoietic stem-cell transplant recipients are at increased risk of developing invasive fungal infections. This is a major cause of morbidity and mortality. We report a case of a 17-year-old male patient diagnosed with severe idiopathic acquired aplastic anemia who developed fungal pneumonitis due to Rhizomucor sp. and rhinoencephalitis due to Scedosporium apiospermum 6 and 8 months after undergoing allogeneic hematopoietic stem-cell transplant from an HLA-matched unrelated donor. Discussion highlights risk factors for invasive fungal infections (i.e., mucormycosis and scedosporiosis, its clinical features, and the factors that must be taken into account to successfully treat them (early diagnosis, correction of predisposing factors, aggressive surgical debridement, and antifungal and adjunctive therapies.

Case of acute myelogenous leukemia following aplastic anemia after radiotherapy and chemotherapy for breast cancer

Energy Technology Data Exchange (ETDEWEB)

Takano, Y; Chinen, T; Ogawa, M; Kato, Y; Kitagawa, T [Japanese Foundation for Cancer Research, Tokyo. Hospital

1980-12-01

A 53 years old mastectomized woman for breast cancer treated with radiotherapy (total doses 12,600 rad) and with long term oral administration of cyclophosphamide (CPM) and ftorafur (FT), developed aplastic anemia and thereafter acute myelogenous leukemia. About six months after discontinuation of the above therapies, she developed anemia and leukopenia and was referred to our clinic. Hematological improvement was obtained by the administration of anabolic hormone, however, two months later she became pancytopenic again. At that time, quite atypical myeloblasts contained peroxidase positive granules, were found 39% in the peripheral blood and 89.4% in the bone marrow, respectively. Leukemic hiatus was present. A bone marrow biopsy revealed coexistence of leukemic cells and breast cancer cells. A diagnosis of breast cancer complicated with acute myelogenous leukemia was made. A combined therapy of adriamycin, CPM and FT was ineffective. OAP regimen of vincristine, cytosine arabinoside and predonisolone revealed transient hematologic improvement. Finally, the patient died of septicemia due to klebsiella. Autopsy revealed wide spread coexistence of leukemia and cancer in the bone marrow, liver, and thyroid. The authors discuss some possible explanations for development of acute leukemia after radiotherapy and chemotherapy.

Music evoked autobiographical memory after severe acquired brain injury: preliminary findings from a case series.

Science.gov (United States)

Baird, A; Samson, S

2014-01-01

Music evoked autobiographical memories (MEAMs) have been characterised in the healthy population, but not, to date, in patients with acquired brain injury (ABI). Our aim was to investigate music compared with verbal evoked autobiographical memories. Five patients with severe ABI and matched controls completed the experimental music (MEAM) task (a written questionnaire) while listening to 50 "Number 1 Songs of the Year" (from 1960 to 2010). Patients also completed the Autobiographical Memory Interview (AMI) and a standard neuropsychological assessment. With the exception of Case 5, who reported no MEAMs and no autobiographical incidents on the AMI and who also had impaired pitch perception, the range of frequency and type of MEAMs in patients was broadly in keeping with their matched controls. The relative preservation of MEAMs in four cases was particularly noteworthy given their impaired verbal and/or visual anterograde memory, and in three cases, autobiographical memory impairment. The majority of MEAMs in both cases and matched controls were of a person/people or a period of life. In three patients music was more efficient at evoking autobiographical memories than the AMI verbal prompts. This is the first study of MEAMs after ABI. The findings suggest that music is an effective stimulus for eliciting autobiographical memories, and may be beneficial in the rehabilitation of autobiographical amnesia, but only in patients without a fundamental deficit in autobiographical recall memory and intact pitch perception.

Differential diagnosis of myelodysplastic syndrome and aplastic anemia using MRI

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Jung, Seung Eun; Park, Jung Mi; Lee, Jae Mun; Kim, Ki Tae; Kim, Dong Wook; Kim, Chun Choo; Kim, Chun Yul; Shinn, Kyung Sub [Catholic University Medical College, Seoul (Korea, Republic of)

1995-04-15

To assess the patterns of myelodysplastic syndrome (MDS) and aplastic anemia (AA) on MRI of the spinal bone marrow and to find the differential points between the two groups. Fourteen patients with MDS (n=7) and AA (n=7) were studied using magnetic resonance imaging. Sagittal images from the lower thoracic and lumbar vertebral marrow were evaluated on T1-weighted and STIR images. Five distinct patterns of signal intensity of the T1-weighted and STIR images were classified. T1 and T2 relaxation times and T1 marrow/fat signal intensity ratio were measured and analyzed (t-test). The cellularity of bone marrow was evaluated on histologic slides. MDS showed homogeneously low signal intensity on T1WI and high signal intensity on STIR image, indicating hypercellular marrow, whereas AA showed relative high signal intensity on T1WI and low signal intensity on STIR image, representing fatty marrow. T1 and T2 relaxation time (T1 for MDS=750.26 msec {+-} 177.50, T1 for AA=413.21 msec {+-} 167.39 ({rho} < 0.000), T2 for MDS=91.86 msec {+-} 14.16, T2 for AA=81.44 msec {+-} 15.31 ({rho} < 0.001) and T1 marrow/fat signal intensity ratio (0.22 {+-} 0.048 in MDS, 0.30 {+-} 0.083 in AA ({rho} < 0.000) revealed statistically significant difference between the two groups. Although the marrow aspiration and needle biopsy are mandatory in hematologic disease for diagnosis, there are limited in assessing the change of total marrow mass. Therefore MRI of bone marrow might be useful in distinguishing MDS from AA because of its ability of representation of total marrow mass.

Differential diagnosis of myelodysplastic syndrome and aplastic anemia using MRI

International Nuclear Information System (INIS)

Jung, Seung Eun; Park, Jung Mi; Lee, Jae Mun; Kim, Ki Tae; Kim, Dong Wook; Kim, Chun Choo; Kim, Chun Yul; Shinn, Kyung Sub

1995-01-01

To assess the patterns of myelodysplastic syndrome (MDS) and aplastic anemia (AA) on MRI of the spinal bone marrow and to find the differential points between the two groups. Fourteen patients with MDS (n=7) and AA (n=7) were studied using magnetic resonance imaging. Sagittal images from the lower thoracic and lumbar vertebral marrow were evaluated on T1-weighted and STIR images. Five distinct patterns of signal intensity of the T1-weighted and STIR images were classified. T1 and T2 relaxation times and T1 marrow/fat signal intensity ratio were measured and analyzed (t-test). The cellularity of bone marrow was evaluated on histologic slides. MDS showed homogeneously low signal intensity on T1WI and high signal intensity on STIR image, indicating hypercellular marrow, whereas AA showed relative high signal intensity on T1WI and low signal intensity on STIR image, representing fatty marrow. T1 and T2 relaxation time (T1 for MDS=750.26 msec ± 177.50, T1 for AA=413.21 msec ± 167.39 (ρ < 0.000), T2 for MDS=91.86 msec ± 14.16, T2 for AA=81.44 msec ± 15.31 (ρ < 0.001) and T1 marrow/fat signal intensity ratio (0.22 ± 0.048 in MDS, 0.30 ± 0.083 in AA (ρ < 0.000) revealed statistically significant difference between the two groups. Although the marrow aspiration and needle biopsy are mandatory in hematologic disease for diagnosis, there are limited in assessing the change of total marrow mass. Therefore MRI of bone marrow might be useful in distinguishing MDS from AA because of its ability of representation of total marrow mass

Clinical implications of somatic mutations in aplastic anemia and myelodysplastic syndrome in genomic age.

Science.gov (United States)

Maciejewski, Jaroslaw P; Balasubramanian, Suresh K

2017-12-08

Recent technological advances in genomics have led to the discovery of new somatic mutations and have brought deeper insights into clonal diversity. This discovery has changed not only the understanding of disease mechanisms but also the diagnostics and clinical management of bone marrow failure. The clinical applications of genomics include enhancement of current prognostic schemas, prediction of sensitivity or refractoriness to treatments, and conceptualization and selective application of targeted therapies. However, beyond these traditional clinical aspects, complex hierarchical clonal architecture has been uncovered and linked to the current concepts of leukemogenesis and stem cell biology. Detection of clonal mutations, otherwise typical of myelodysplastic syndrome, in the course of aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria has led to new pathogenic concepts in these conditions and created a new link between AA and its clonal complications, such as post-AA and paroxysmal nocturnal hemoglobinuria. Distinctions among founder vs subclonal mutations, types of clonal evolution (linear or branching), and biological features of individual mutations (sweeping, persistent, or vanishing) will allow for better predictions of the biologic impact they impart in individual cases. As clonal markers, mutations can be used for monitoring clonal dynamics of the stem cell compartment during physiologic aging, disease processes, and leukemic evolution. © 2016 by The American Society of Hematology. All rights reserved.

Visual and quantitative approach to bone marrow foci of increased glucose uptake on PET/CT in a case of aplastic anaemia

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Cicone, F. [Sant' Andrea Hospital, Univ. La Sapienza, Rome (Italy). Nuclear Medicine Dept.; Centre Hospitalier Univ. Vaudois (Switzerland). Nuclear Medicine; Lausanne Univ. (Switzerland); Stalder, M. [Institut Central des Hopitaux Valaisans, Sion (Switzerland). Service of Hematology; Cairoli, A. [Centre Hospitalier Univ. Vaudois (Switzerland). Service of Hematology; Lausanne Univ. (Switzerland); Bischof Delaloye, A.; Prior, J.O. [Centre Hospitalier Univ. Vaudois (Switzerland). Nuclear Medicine; Lausanne Univ. (Switzerland); Geiger, D.

2010-07-01

This case report shows the clinical impact of a FDG-PET/CT in the assessment of bone marrow (BM) of a patient with aplastic anemia. The feasibility of a quantitative approach to BM intensities on FDG-PET is also discussed. In the authors' opinion, a deeper understanding of the factors that might independently affect FDG uptake and the definition of normal ranges of BM SUV (standardized uptake value) might help to interpret PET/CT images. Further research is needed to understand the physio-pathological basis of FDG uptake in BM and the potential value of its quantification. The analysis of the bone marrow on PET/CT is an interesting field of research. A PET/CT scan contributed to differential diagnosis in a patient with suspected bone marrow aplasia for guiding bone marrow biopsies.

Visual and quantitative approach to bone marrow foci of increased glucose uptake on PET/CT in a case of aplastic anaemia

International Nuclear Information System (INIS)

Cicone, F.; Stalder, M.; Bischof Delaloye, A.; Prior, J.O.; Geiger, D.

2010-01-01

This case report shows the clinical impact of a FDG-PET/CT in the assessment of bone marrow (BM) of a patient with aplastic anemia. The feasibility of a quantitative approach to BM intensities on FDG-PET is also discussed. In the authors' opinion, a deeper understanding of the factors that might independently affect FDG uptake and the definition of normal ranges of BM SUV (standardized uptake value) might help to interpret PET/CT images. Further research is needed to understand the physio-pathological basis of FDG uptake in BM and the potential value of its quantification. The analysis of the bone marrow on PET/CT is an interesting field of research. A PET/CT scan contributed to differential diagnosis in a patient with suspected bone marrow aplasia for guiding bone marrow biopsies.

An inter-laboratory comparison of PNH clone detection by high-sensitivity flow cytometry in a Russian cohort.

Science.gov (United States)

Sipol, Alexandra A; Babenko, Elena V; Borisov, Vyacheslav I; Naumova, Elena V; Boyakova, Elena V; Yakunin, Dimitry I; Glazanova, Tatyana V; Chubukina, Zhanna V; Pronkina, Natalya V; Popov, Alexander M; Saveliev, Leonid I; Lugovskaya, Svetlana A; Lisukov, Igor A; Kulagin, Alexander D; Illingworth, Andrea J

2015-01-01

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by partial or absolute deficiency of glycophosphatidyl-inositol (GPI) anchor-linked surface proteins on blood cells. A lack of precise diagnostic standards for flow cytometry has hampered useful comparisons of data between laboratories. We report data from the first study evaluating the reproducibility of high-sensitivity flow cytometry for PNH in Russia. PNH clone sizes were determined at diagnosis in PNH patients at a central laboratory and compared with follow-up measurements in six laboratories across the country. Analyses in each laboratory were performed according to recommendations from the International Clinical Cytometry Society (ICCS) and the more recent 'practical guidelines'. Follow-up measurements were compared with each other and with the values determined at diagnosis. PNH clone size measurements were determined in seven diagnosed PNH patients (five females, two males: mean age 37 years); five had a history of aplastic anemia and three (one with and two without aplastic anemia) had severe hemolytic PNH and elevated plasma lactate dehydrogenase. PNH clone sizes at diagnosis were low in patients with less severe clinical symptoms (0.41-9.7% of granulocytes) and high in patients with severe symptoms (58-99%). There were only minimal differences in the follow-up clone size measurement for each patient between the six laboratories, particularly in those with high values at diagnosis. The ICCS-recommended high-sensitivity flow cytometry protocol was effective for detecting major and minor PNH clones in Russian PNH patients, and showed high reproducibility between laboratories.

Hydrocephalus is a rare outcome in community-acquired bacterial meningitis in adults

DEFF Research Database (Denmark)

Bodilsen, Jacob; Schønheyder, Henrik Carl; Nielsen, Henrik I

2013-01-01

BACKGROUND: Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid in this ......BACKGROUND: Community-acquired bacterial meningitis (CABM) continues to have a high mortality rate and often results in severe sequelae among survivors. Lately, an increased effort has been focused on describing the neurological complications of meningitis including hydrocephalus. To aid...... in this field of research we set out to ascertain the risk and outcome of hydrocephalus in patients with community-acquired bacterial meningitis (CABM) in North Denmark Region. METHODS: We conducted a retrospective population-based cohort study of CABM cases above 14 years of age. Cases diagnosed during a 13......-year period, 1998 through 2010, were identified in a laboratory register and data were acquired through patient records. Cases not confirmed by culture met other strict inclusion criteria. The diagnosis of hydrocephalus relied upon the radiologists' reports on cranial imaging. Outcome was graded...

Análise da rejeição nos pacientes transplantados por anemia aplástica severa condicionados com ciclofosfamida ou a associação desta ao bussulfano Analysis of rejection in transplanted patients suffering severe aplastic anemia conditioned with cyclophosphamide alone or associated with busulfan

Directory of Open Access Journals (Sweden)

Lídice C. Lenz e Silva

2005-03-01

Full Text Available O transplante de medula óssea é um tratamento eficaz para pacientes com anemia aplástica severa (AAS e é a modalidade terapêutica de escolha para pacientes jovens com doador aparentado HLA idêntico. A rejeição é uma importante complicação do transplante de medula, que, independentemente do tipo de tratamento imunossupressor pré e pós-transplante, ocorre em 55% a 60% dos pacientes. O serviço de TMO da Universidade Federal do Paraná (UFPR acumula a experiência de 178 casos de AAS transplantados no período de 1993 a 2001, usando como condicionamento tanto a ciclofosfamida (CFA como a combinação desta ao bussulfano (CFA + BU. Dentre eles, 39 apresentaram rejeição ou falha de pega. Dos pacientes condicionados com ciclofosfamida, 24 (46% apresentaram rejeição, sendo 3 (6% com falha primária de pega (FPP e 21 (40% com pega transitória (PT. Entre os pacientes condicionados com BU+CFA, 15 (12% apresentaram rejeição, sendo 4 (3% com FPP e 11 (9% com pega transitória. Os pacientes condicionados com ciclofosfamida (200 mg/kg que apresentaram rejeição tiveram uma sobrevida global alta (aproximadamente 80%, pois conseguiram ser resgatados por um novo transplante ou pelo tratamento imunossupressor com ciclosporina. A sobrevida dos pacientes politransfundidos condicionados com a associação de ciclofosfamida e bussulfano foi de aproximadamente 35%.Bone marrow transplantation is an effective therapy for severe aplastic anemia and is generally considered the preferable treatment for young patients who have an HLA (Human Leukocyte Antigen identical sibling donor. Recent studies report 55% to 80% extended survival. Graft failure owing to rejection or others causes remains an important life-threatening complication following allogeneic bone marrow transplantation for aplastic anemia. It occurs in 55% to 60% of patients receiving HLA identical transplants, using different immunosuppressive therapies before and after transplant. The BMT

Lichen Striatus Occurring after Allogenic Peripheral Blood Stem Cell Transplantation in an Adult with Aplastic Anemia

OpenAIRE

Mun, Je-Ho; Park, Hyun-Je; Kim, Hoon-Soo; Kim, Su-Han; Ko, Hyun-Chang; Kim, Byung-Soo; Kim, Moon-Bum

2012-01-01

Lichens striatus (LS) is an acquired, self-limiting inflammatory dermatosis that follows the lines of Blaschko. The etiology of the eruption is unknown, but several theories have been proposed with focus on environmental factors, viral infection, cutaneous injury, hypersensitivity, and genetic predisposition. We describe a 19-year-old woman who developed a unilateral linear eruption 17 months after allogenic peripheral blood stem cell transplantation. Histopathology revealed features, which w...

Severe acute hepatitis and cold agglutinin-related hemolytic anemia secondary to prime infection with Epstein-Barr virus

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Guillermo Ontanilla-Clavijo

Full Text Available Epstein-Barr virus, a member of the Herpesviridae family, is responsible for the infectious mononucleosis clinical syndrome, which mainly includes the pharyngitis, fever, and lymphadenopathy triad after incubation for 30-50 days. The liver is involved in 80-90% of patients in a self-limiting transient manner, with jaundice being much more uncommon (5%. From a hematological standpoint it may manifest aplastic anemia, neutropenia, and thrombocytopenia. We report a case of infectious mononucleosis that included severe acute hepatitis and was associated with severe hemolytic anemia secondary to cold agglutinins. After exclusion of other etiologies, and given the clinical suspicion of the above association, which was later confirmed by lab tests, empiric therapy was initiated with antiviral agents (aciclovir + valganciclovir and corticoids, which resulted in a progressive clinical improvement until complete remission. Therefore, we believe that this case report will reinforce the clinical evidence in support of the above combined therapy for serious infectious mononucleosis as a step prior to liver transplantation.

Learning-by-Being-Acquired

DEFF Research Database (Denmark)

Colombo, Massimo Gaetano; Moreira, Solon; Rabbiosi, Larissa

2016-01-01

In horizontal acquisitions, the post-acquisition integration of the R&D function often damages the inventive labor force and results in lower innovative productivity of acquired inventors. In this paper we study post-acquisition integration in terms of R&D team reorganization-i.e., the creation...... of new teams with both inventors of the acquiring and acquired firms-and assess the impact of this integration action in the period that immediately follows the acquisition. Drawing on social identity and self-categorization theories, we argue that R&D team reorganization increases the acquired inventors...

Acquired alopecia, mental retardation, short stature, microcephaly, and optic atrophy

NARCIS (Netherlands)

Hennekam, R. C.; Renckens-Wennen, E. G.

1990-01-01

We report on a female patient who had acquired total alopecia, short stature, microcephaly, optic atrophy, severe myopia, and mental retardation. A survey of published reports failed to show an identical patient, despite various similar cases

Age-related pattern and monocyte-acquired haemozoin associated production of erythropoietin in children with severe malarial anaemia in Ghana.

Science.gov (United States)

Abugri, James; Tetteh, John Kweku Amissah; Oseni, Lateef Adebayo; Mensah-Brown, Henrietta Esi; Delimini, Rupert Kantunye; Obuobi, David Osei; Akanmori, Bartholomew Dicky

2014-08-20

Malaria continues to be a global health challenge, affecting more than half the world's population and causing approximately 660,000 deaths annually. The majority of malaria cases are caused by Plasmodium falciparum and occur in sub-Saharan Africa. One of the major complications asscociated with malaria is severe anaemia, caused by a cycle of haemoglobin digestion by the parasite. Anaemia due to falciparum malaria in children has multifactorial pathogenesis, which includes suppression of bone marrow activity. Recent studies have shown that haemozoin, which is a by-product of parasite haemoglobin digestion, may play an important role in suppression of haemoglobin production, leading to anaemia. In this study we correlated the levels of erythropoietin (EPO), as an indicator of stimulation of haemoglobin production, to the levels of monocyte acquired haemozoin in children with both severe and uncomplicated malaria. There was a significantly negative correlation between levels of haemozoin-containing monocytes and EPO, which may suggest that haemozoin suppresses erythropoiesis in severe malaria. A multiple linear regression analysis and simple bar was used to investigate associations between various haematological parameters. To examine the levels of erythropoietin in the age categories, the levels of erythropoietin was measured using a commercial Enyme-Linked Immunosorbent Assay (ELISA). Giemsa-stained blood smears were used to determine percentage pigment containing monocytes. The haemozoin containing monocytes was expressed as a percentage of the total number of monocytes. To obtain the number of haemozoin containing monocytes/μL the percentage of haemozoin containing monocytes was multiplied by the absolute number of monocytes/μL from the automated haematology analyzer. The levels of erythropoietin in younger children (<3 years) was significantly higher than in older children with a similar degree of malaria anaemia (Hb levels) (p < 0.005). Haemozoin

Frequency of hospital acquired hyponatremia in a pediatric tertiary care setting

International Nuclear Information System (INIS)

Bibi, S.; Haq, A.U.; Billo, A.G.; Bibi, S.; Gilani, S.Y.H.; Shah, S.R.A.

2015-01-01

Background: Hyponatremia is the most commonly encountered electrolyte disorder in children. In our country the epidemiology of hospital acquired hyponatremia has hardly ever been explored whereas the administration of hypotonic IV fluids is widely practiced here. Therefore we pioneered to conduct this study to determine the frequency of hospital acquired hyponatremia. Method: This was a cross sectional study carried out at Aga Khan University Hospital, Karachi in paediatric ward and ICU over a period of 12 months. All children (>1 month and <15 years of age) admitted in paediatric units and on maintenance IV fluids who had serum sodium level measured on admission were included in the study and followed to identify patients who had a drop in serum sodium during hospitalization. Informed consent was taken from parents and collected data was recorded on a proforma. Results: A total of 865 patients were enrolled in the study. Hyponatremia was recorded in 405 patients on admission (46.8 percentage) while hospital acquired hyponatremia was documented in 240. children (27.7 percentage). Out of these 142 (59.2 percentage) were male and 98 (40.8 percentage) were female. Mean age of children in hospital acquired hyponatremia group was 60.67 months. Severity of hospital acquired hyponatremia was recorded as mild in 191 (79.6 percentage), moderate in 35 (14.6 percentage) and severe in 14 (5.8) children. Major disease categories included gastrointestinal disorder (30.4 percentage), respiratory illness (12.5 percentage), oncological disease (16.3 percentage), cardiovascular disease (11.7 percentage), infectious disease (9.2 percentage) and neurological illness (8.3 percentage). Conclusion: Hospital acquired hyponatremia is frequently encountered in our hospitalized children with majority of them receiving hypotonic IV solutions. (author)

Cosmic ray runs acquired with ATLAS muon stations

CERN Multimedia

Cerutti, F.

Starting in the fall 2005 several cosmic ray runs have been acquired in the ATLAS pit with six muon stations. These were three large outer and three large middle chambers of the feet sector (sector 13) that have been readout in the ATLAS cavern. In the first data taking period the trigger was based on two large scintillators (~300x30 cm2) positioned in sector 13 just below the large chambers. In this first run the precision chambers (the Monitored Drift Tubes) were operated in a close to final configuration. Typical trigger rates with this setup were of the order of 1 Hz. Several data sets of 10k events were acquired with final electronics up to the muon ROD and analysed with ATHENA-based software. These data allowed the first checks of the functionality and efficiency of the MDT stations in the ATLAS pit and the first measurement of the FE electronics noise in the ATLAS environment. A few event were also collected in a combined run with the TILE barrel calorimeter. An event display of a cosmic ray a...

Acquired neuropathies.

Science.gov (United States)

Lozeron, Pierre; Trocello, Jean-Marc; Kubis, Nathalie

2013-09-01

Acquired neuropathies represent most of the neuropathies encountered in clinical practice. Hundreds of causes have been identified even though up to 41% of patients are still classified as idiopathic (Rajabally and Shah in J Neurol 258:1431-1436, 1). Routine evaluation relies on comprehensive medical history taking, clinical examination, nerve conduction studies and laboratory tests. Other investigations such as nerve biopsy or nerve or muscle imaging are performed in specific settings. This review focuses on recent advances in acquired neuropathies.

Flow cytometric analysis of lymphocytes in aplastic anemia among atomic bomb survivors. With special reference to immunological mechanisms and bolus methylprednisolone therapy

Energy Technology Data Exchange (ETDEWEB)

Imamura, Nobutaka; Inada, Tominari; Asaoku, Hideki; Abe, Kazuhiro; Oguma, Nobuo; Kuramoto, Atsushi

1986-11-01

In 6 patients with aplastic anemia and 3 patients with pernicious anemia, lymphocyte subpopulations in the peripheral blood were measured, before and after steroid therapy, with a fluorescence-activated cell sorder using various monoclonal antibodies. The ratio of OKT4-positive lymphocytes (T4) to OKT8-positive lymphocytes (T8) in the peripheral blood was reduced in 2 patients (20%). The T4/T8 ratio returned to normal during remission of anemia. Hematological improvement was seen after a large amount of steroid therapy in 3 patients. The number of Tac-positive cells tended to decrease and the T4/T8 ratio tended to return to normal with hematological improvement, although there was no correlation to hydrocortisone reaction. Some patients were supposed to have abnormal number of suppressor and inducer T cells. (Namekawa, K.).

Bone marrow transplantation for girls with aplastic anemia utilizing modified field of total lymphoid irradiation and cyclophosphamide

International Nuclear Information System (INIS)

Hanada, Ryoji; Kawakami, Tetsuo; Akuta, Naoko; Moriwaki, Kohichi; Kato, Shizue; Inaba, Toshiya; Hayashi, Yasuhide; Yamamoto, Keiko

1990-01-01

A preparative regimen for allogeneic bone marrow transplantation, consisting of total lymphoid irradiation (TLI) with 750 cGy and cyclophosphamide (CY), was used in five girls with aplastic anemia. All patients received bone marrow from HLA matched/mixed lymphocyte culture negative siblings. In our regimen the 'inverted Y' field to irradiate the pelvic nodes was modified, which did not include the whole pelvic cavity in an attempt to protect the ovaries from irradiation. Although some of the pelvic nodes was supported not to be irradiated in order to protect the ovaries, engraftment occurred in all five patients including four who had been transfused prior to transplantation. All five are alive from 47 days to 1378 days (median 285 days) after transplantation without tranplantation-associated complications. The calculated dose to the ovaries was sixteen percent of the entire dose of the regimen. Both of the two evaluable patients that had received tranplantation just before or during the puberty are developing normal sex maturity including menstruation. This study suggests that our preparative regimen is effective not only for engraftment of the donor marrow but also for protecting the ovaries from irradiation. (author)

Quantitative Detection of ID4 Gene Aberrant Methylation in the Differentiation of Myelodysplastic Syndrome from Aplastic Anemia

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Mian-Yang Li

2015-01-01

Full Text Available Background: The diagnosis of myelodysplastic syndrome (MDS, especially hypoplastic MDS, and MDS with low blast counts or normal karyotype may be problematic. This study characterized ID4 gene methylation in patients with MDS and aplastic anemia (AA. Methods: The methylation status of ID4 was analyzed by bisulfite sequencing polymerase chain reaction (PCR and quantitative real-time methylation-specific PCR (MethyLight PCR in 100 patients with MDS and 31 patients with AA. Results: The MDS group had a higher ID4 gene methylation positivity rate (22.22% and higher methylation levels (0.21 [0-3.79] than the AA group (P < 0.05. Furthermore, there were significant differences between the hypoplastic MDS and AA groups, the MDS with low blast count and the AA groups, and the MDS with normal karyotype and the AA groups. The combination of genetic and epigenetic markers was used in much more patients with MDS (62.5% [35/56] than the use of genetic markers only (51.79% [29/56]. Conclusions: These results showed that the detection of ID4 methylation positivity rates and levels could be a useful biomarker for MDS diagnosis.

Validation of the Infectious Diseases Society of America/American Thoracic Society criteria to predict severe community-acquired pneumonia caused by Streptococcus pneumoniae.

Science.gov (United States)

Kontou, Paschalina; Kuti, Joseph L; Nicolau, David P

2009-10-01

Severe community-acquired pneumonia (CAP) is usually defined as pneumonia that requires intensive care unit (ICU) admission; the primary pathogen responsible for ICU admission is Streptococcus pneumoniae. In this study, the 2007 Infectious Diseases Society of America/American Thoracic Society (IDSA/ATS) consensus criteria for ICU admission were compared with other severity scores in predicting ICU admission and mortality. We retrospectively studied 158 patients with pneumococcal CAP (1999-2003). Clinical and laboratory features at the emergency department were recorded and used to calculate the 2007 IDSA/ATS rule, the 2001 ATS rule, 2 modified 2007 IDSA/ATS rules, the Pneumonia Severity Index (PSI), and the CURB (confusion, urea, respiratory rate, blood pressure) score. The sensitivity, specificity, positive predictive value, and negative predictive value (NPV) were assessed for the various indices. We also determined the criteria that were independently predictive of ICU admission and of mortality in our population. The 2007 IDSA/ATS criteria performed as well as the 2001 ATS rule in predicting ICU admission both demonstrated high sensitivity (90%) and NPV (97%). For the prediction of mortality, the best tool proved to be the PSI score (sensitivity, 95%; NPV, 99%). The variables associated with ICU admission in this patient population included tachypnea, confusion, Pao(2)/Fio(2) ratio of 250 or lower, and hypotension requiring fluid resuscitation. Mechanical ventilation and PSI class V were independently associated with mortality. This study confirms the usefulness of the new criteria in predicting severe CAP. The 2001 ATS criteria seem an attractive alternative because they are simple and as effective as the 2007 IDSA/ATS criteria.

Lentiviral gene transfer regenerates hematopoietic stem cells in a mouse model for Mpl-deficient aplastic anemia.

Science.gov (United States)

Heckl, Dirk; Wicke, Daniel C; Brugman, Martijn H; Meyer, Johann; Schambach, Axel; Büsche, Guntram; Ballmaier, Matthias; Baum, Christopher; Modlich, Ute

2011-04-07

Thpo/Mpl signaling plays an important role in the maintenance of hematopoietic stem cells (HSCs) in addition to its role in megakaryopoiesis. Patients with inactivating mutations in Mpl develop thrombocytopenia and aplastic anemia because of progressive loss of HSCs. Yet, it is unknown whether this loss of HSCs is an irreversible process. In this study, we used the Mpl knockout (Mpl(-/-)) mouse model and expressed Mpl from newly developed lentiviral vectors specifically in the physiologic Mpl target populations, namely, HSCs and megakaryocytes. After validating lineage-specific expression in vivo using lentiviral eGFP reporter vectors, we performed bone marrow transplantation of transduced Mpl(-/-) bone marrow cells into Mpl(-/-) mice. We show that restoration of Mpl expression from transcriptionally targeted vectors prevents lethal adverse reactions of ectopic Mpl expression, replenishes the HSC pool, restores stem cell properties, and corrects platelet production. In some mice, megakaryocyte counts were atypically high, accompanied by bone neo-formation and marrow fibrosis. Gene-corrected Mpl(-/-) cells had increased long-term repopulating potential, with a marked increase in lineage(-)Sca1(+)cKit(+) cells and early progenitor populations in reconstituted mice. Transcriptome analysis of lineage(-)Sca1(+)cKit(+) cells in Mpl-corrected mice showed functional adjustment of genes involved in HSC self-renewal.

[Ceftaroline fosamil in community-acquired and nosocomial pneumonia].

Science.gov (United States)

Calbo, Esther; Zaragoza, Rafael

2014-03-01

Community-acquired pneumonia (CAP) is a common infection in developed countries and causes a large number of hospital admissions and deaths. In recent years, the incidence of this disease has increased, caused by progressive population aging. Following the introduction of the conjugate vaccine against Streptococcus pneumoniae, there have been significant epidemiological changes that require close monitoring because of the possible emergence of new patterns of resistance. This article aims to review the role of ceftaroline fosamil, a new parenteral cephalosporin with antibacterial activity against Gram-negative and Gram-positive pathogens, in the treatment of pneumonia. Several in vitro and in vivo studies have shown the efficacy of ceftaroline fosamil against penicillin-resistant S. pneumoniae and methicillin-resistant Staphylococcus aureus (MRSA). Additionally, ceftaroline has shown similar efficacy and safety to ceftriaxone in the treatment of community-acquired pneumonia with severe prognosis (prognostic severity index III and IV) in two phase III clinical trials. Although a non-inferiority design was used for these clinical trials, some data suggest a superior efficacy of ceftaroline, with earlier clinical response and higher cure rate in infections caused by S. pneumoniae, making this drug particularly interesting for critically-ill patients admitted to the intensive care unit. Ceftaroline may also be considered for empirical and directed treatment of MRSA pneumonia. Copyright © 2014 Elsevier España, S.L. All rights reserved.

Report of a case of paroxysmal nocturnal hemoglobinuria (PNH) with complex evolution and liver transplant

OpenAIRE

Alencar,Railene Célia B.; Guimarães,Andréa M.; Brito Junior,Lacy C.

2016-01-01

ABSTRACT The paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disease, with thrombotic episodes and frequent pancytopenia. We report the case of a 32 year-old female PNH patient with bone marrow aplasia, which followed a complex course, diagnosed with aplastic anemia associated with PNH, evolving in three years with Budd-Chiari syndrome and liver transplantation. Post-transplant complications, hepatic arterial thrombosis, graft rejection, liver retransplantation and treatment of P...

Acquired Credit Unions: Drivers of Takeover

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R. Raymond Sant

2015-08-01

Full Text Available In this paper we study acquired credit unions and analyze their financial performance up to six years prior to merger, on a quarterly basis. The primary focus is on balance sheet (asset liability management and profitability variables (return on assets. We find that acquired credit unions during the period 2008 (third quarter to 2014 (first quarter experienced negative return on assets for several quarters prior to their takeover. This was the result of a declining loan portfolio and increasing charge offs. In spite of decreasing lending activity, such credit unions continued to increase their deposits, i.e., adding to their cost base. Due to declining loans, their net interest margin as a proportion of deposits was also in decline. We argue that this is an indicator of poor management ability. Furthermore, our analysis finds that operating expenses were increasing over time, something that has been documented in previous literature also for smaller credit unions and is attributable to lack of economies of scale. The average asset size of the acquired credit unions in our sample is about $22 million just before acquisition. We attribute our findings to poor business strategy followed by such credit unions. We also conclude that signs of trouble are evident up to two years before merger on average and regulatory policy may have to become more proactive to manage the consolidation challenge faced by the credit union industry in general.

Visual rehabilitation with Retimax Vision Trainer in patients with severe Acquired Brain Injury: report of two cases

Directory of Open Access Journals (Sweden)

Margherita Chiari

2014-04-01

Full Text Available Retimax Vision Trainer is a device that has the purpose to improve visual function by means of the detection of a visual evoked potential associated with a sound feedback. We evaluated the effectiveness of rehabilitative treatment in two patients with Acquired Brain Injury (ABI. Results, subjectively appreciated, are objectively confirmed by the improvement of visual function.

Behavior Management for Children and Adolescents with Acquired Brain Injury

Science.gov (United States)

Slifer, Keith J.; Amari, Adrianna

2009-01-01

Behavioral problems such as disinhibition, irritability, restlessness, distractibility, and aggression are common after acquired brain injury (ABI). The persistence and severity of these problems impair the brain-injured individual's reintegration into family, school, and community life. Since the early 1980s, behavior analysis and therapy have…

Alefacept and Allogeneic Hematopoietic Stem Cell Transplantation

Science.gov (United States)

2017-07-24

Thalassemia; Sickle Cell Disease; Glanzmann Thrombasthenia; Wiskott-Aldrich Syndrome; Chronic-granulomatous Disease; Severe Congenital Neutropenia; Leukocyte Adhesion Deficiency; Schwachman-Diamond Syndrome; Diamond-Blackfan Anemia; Fanconi Anemia; Dyskeratosis-congenita; Chediak-Higashi Syndrome; Severe Aplastic Anemia

Phase II Study Evaluating Busulfan and Fludarabine as Preparative Therapy in Adults With Hematopoietic Disorders Undergoing MUD SCT

Science.gov (United States)

2009-01-22

Chronic Myeloid Leukemia; Acute Myelogenous Leukemia; Myelodysplasia; Acute Lymphocytic Leukemia; Severe Aplastic Anemia; Non-Hodgkin's Lymphoma; Lymphoproliferative Disease; Multiple Myeloma; Advanced Myeloproliferative Disease

Severe community-acquired Enterobacter pneumonia: a plea for greater awareness of the concept of health-care-associated pneumonia

Science.gov (United States)

2011-01-01

Background Patients with Enterobacter community-acquired pneumonia (EnCAP) were admitted to our intensive care unit (ICU). Our primary aim was to describe them as few data are available on EnCAP. A comparison with CAP due to common and typical bacteria was performed. Methods Baseline clinical, biological and radiographic characteristics, criteria for health-care-associated pneumonia (HCAP) were compared between each case of EnCAP and thirty age-matched typical CAP cases. A univariate and multivariate logistic regression analysis was performed to determine factors independently associated with ENCAP. Their outcome was also compared. Results In comparison with CAP due to common bacteria, a lower leukocytosis and constant HCAP criteria were associated with EnCAP. Empiric antibiotic therapy was less effective in EnCAP (20%) than in typical CAP (97%) (p < 0.01). A delay in the initiation of appropriate antibiotic therapy (3.3 ± 1.6 vs. 1.2 ± 0.6 days; p < 0.01) and an increase in duration of mechanical ventilation (8.4 ± 5.2 vs. 4.0 ± 4.3 days; p = 0.01) and ICU stay were observed in EnCAP patients. Conclusions EnCAP is a severe infection which is more consistent with HCAP than with typical CAP. This retrospectively suggests that the application of HCAP guidelines should have improved EnCAP management. PMID:21569334

Identification and Severity Determination of Wheat Stripe Rust and Wheat Leaf Rust Based on Hyperspectral Data Acquired Using a Black-Paper-Based Measuring Method

Science.gov (United States)

Ruan, Liu; Wang, Rui; Liu, Qi; Ma, Zhanhong; Li, Xiaolong; Cheng, Pei; Wang, Haiguang

2016-01-01

It is important to implement detection and assessment of plant diseases based on remotely sensed data for disease monitoring and control. Hyperspectral data of healthy leaves, leaves in incubation period and leaves in diseased period of wheat stripe rust and wheat leaf rust were collected under in-field conditions using a black-paper-based measuring method developed in this study. After data preprocessing, the models to identify the diseases were built using distinguished partial least squares (DPLS) and support vector machine (SVM), and the disease severity inversion models of stripe rust and the disease severity inversion models of leaf rust were built using quantitative partial least squares (QPLS) and support vector regression (SVR). All the models were validated by using leave-one-out cross validation and external validation. The diseases could be discriminated using both distinguished partial least squares and support vector machine with the accuracies of more than 99%. For each wheat rust, disease severity levels were accurately retrieved using both the optimal QPLS models and the optimal SVR models with the coefficients of determination (R2) of more than 0.90 and the root mean square errors (RMSE) of less than 0.15. The results demonstrated that identification and severity evaluation of stripe rust and leaf rust at the leaf level could be implemented based on the hyperspectral data acquired using the developed method. A scientific basis was provided for implementing disease monitoring by using aerial and space remote sensing technologies. PMID:27128464

Identification and Severity Determination of Wheat Stripe Rust and Wheat Leaf Rust Based on Hyperspectral Data Acquired Using a Black-Paper-Based Measuring Method.

Science.gov (United States)

Wang, Hui; Qin, Feng; Ruan, Liu; Wang, Rui; Liu, Qi; Ma, Zhanhong; Li, Xiaolong; Cheng, Pei; Wang, Haiguang

2016-01-01

It is important to implement detection and assessment of plant diseases based on remotely sensed data for disease monitoring and control. Hyperspectral data of healthy leaves, leaves in incubation period and leaves in diseased period of wheat stripe rust and wheat leaf rust were collected under in-field conditions using a black-paper-based measuring method developed in this study. After data preprocessing, the models to identify the diseases were built using distinguished partial least squares (DPLS) and support vector machine (SVM), and the disease severity inversion models of stripe rust and the disease severity inversion models of leaf rust were built using quantitative partial least squares (QPLS) and support vector regression (SVR). All the models were validated by using leave-one-out cross validation and external validation. The diseases could be discriminated using both distinguished partial least squares and support vector machine with the accuracies of more than 99%. For each wheat rust, disease severity levels were accurately retrieved using both the optimal QPLS models and the optimal SVR models with the coefficients of determination (R2) of more than 0.90 and the root mean square errors (RMSE) of less than 0.15. The results demonstrated that identification and severity evaluation of stripe rust and leaf rust at the leaf level could be implemented based on the hyperspectral data acquired using the developed method. A scientific basis was provided for implementing disease monitoring by using aerial and space remote sensing technologies.

Differential gene expression profile associated with the abnormality of bone marrow mesenchymal stem cells in aplastic anemia.

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Jianping Li

Full Text Available Aplastic anemia (AA is generally considered as an immune-mediated bone marrow failure syndrome with defective hematopoietic stem cells (HSCs and marrow microenvironment. Previous studies have demonstrated the defective HSCs and aberrant T cellular-immunity in AA using a microarray approach. However, little is known about the overall specialty of bone marrow mesenchymal stem cells (BM-MSCs. In the present study, we comprehensively compared the biological features and gene expression profile of BM-MSCs between AA patients and healthy volunteers. In comparison with healthy controls, BM-MSCs from AA patients showed aberrant morphology, decreased proliferation and clonogenic potential and increased apoptosis. BM-MSCs from AA patients were susceptible to be induced to differentiate into adipocytes but more difficult to differentiate into osteoblasts. Consistent with abnormal biological features, a large number of genes implicated in cell cycle, cell division, proliferation, chemotaxis and hematopoietic cell lineage showed markedly decreased expression in BM-MSCs from AA patients. Conversely, more related genes with apoptosis, adipogenesis and immune response showed increased expression in BM-MSCs from AA patients. The gene expression profile of BM-MSCs further confirmed the abnormal biological properties and provided significant evidence for the possible mechanism of the destruction of the bone marrow microenvironment in AA.

Post-injury personality in the prediction of outcome following severe acquired brain injury.

Science.gov (United States)

Cattran, Charlotte Jane; Oddy, Michael; Wood, Rodger Llewellyn; Moir, Jane Frances

2011-01-01

The aim of the study was to examine the utility of five measures of non-cognitive neurobehavioural (NCNB) changes that often occur following acquired brain injury, in predicting outcome (measured in terms of participation and social adaptation) at 1-year follow-up. The study employed a longitudinal, correlational design. Multiple regression was employed to investigate the value of five new NCNB measures of social perception, emotional regulation, motivation, impulsivity and disinhibition in the prediction of outcome as measured by the Mayo-Portland Adaptability Inventory (MPAI). Two NCNB measures (motivation and emotional regulation) were found to significantly predict outcome at 1-year follow-up, accounting for 53% of the variance in MPAI total scores. These measures provide a method of quantifying the extent of NCNB changes following brain injury. The predictive value of the measures indicates that they may represent a useful tool which could aid clinicians in identifying early-on those whose symptoms are likely to persist and who may require ongoing intervention. This could facilitate the planning of rehabilitation programmes.

Community-acquired bacterial meningitis

NARCIS (Netherlands)

van de Beek, Diederik; Brouwer, Matthijs; Hasbun, Rodrigo; Koedel, Uwe; Whitney, Cynthia G.; Wijdicks, Eelco

2016-01-01

Meningitis is an inflammation of the meninges and subarachnoid space that can also involve the brain cortex and parenchyma. It can be acquired spontaneously in the community - community-acquired bacterial meningitis - or in the hospital as a complication of invasive procedures or head trauma

Acquired Methemoglobinaemia

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Adil Al-Lawati

2012-05-01

Full Text Available Acquired methemoglobinaemia is a relatively rare condition and, therefore infrequently encountered in acute medical practice. Suspicion of the condition may be triggered when the measured PaO2 is ‘out of keeping’ with the oxygen saturations that are discovered with pulse oximetry. We describe two separate cases of acquired methemoglobinaemia secondary to the recreational use of alkyl nitrites (’poppers’. The patients presented at separate times to two different teaching hospitals in London, UK. The similarity of these cases has led the authors to conclude that a raised awareness of this potentially fatal condition, and its association with a widely-available recreational drug, is necessary to ensure a correct and timely diagnosis.

Acquired hyperostosis syndrome. Pt. 2

Energy Technology Data Exchange (ETDEWEB)

Dihlmann, W.; Hering, L.; Bargon, G.W.

1988-12-01

In the second part of this publication, we describe some additional findings in cases of sternocostoclavicular hyperostosis (SCCH). These include focal hyperostosis of the spine, in the pelvis and in the extremities and psoriatric skin lesions and severe forms of acne (acne conglobata, acne fulminans). An analysis of our 13 patients and of the relevant literature indicates that the hyperostosis is due to increased bone metabolism and heterotopic ossification of fibrous tissue and that these are the pathogenic bases of the changes in the axial skeleton, the pelvis and the bones of the extremities. We have suggested a scheme which would categorise the syndrom into complete, incomplete and possibly acquired forms. (orig./GDG).

Laboratory-acquired brucellosis

DEFF Research Database (Denmark)

Fabiansen, C.; Knudsen, J.D.; Lebech, A.M.

2008-01-01

Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9......Brucellosis is a rare disease in Denmark. We describe one case of laboratory-acquired brucellosis from an index patient to a laboratory technician following exposure to an infected blood culture in a clinical microbiology laboratory Udgivelsesdato: 2008/6/9...

Experimental immunologically mediated aplastic anemia (AA) in H-2k identical, Mls (M) locus different mice

Energy Technology Data Exchange (ETDEWEB)

Knospe, W.H.; Steinberg, D.; Speck, B.

1983-07-01

Immunologically mediated aplastic anemia (AA) was experimentally induced in mice by injecting 10(7) lymph node cells (LNC) from donor mice of one inbred strain to another H-2k identical but Mls mismatched strain previously given 600 rad total body gamma irradiation (TBI). AA developed after 2 weeks to 6 months in selected strain combinations used and usually 60 to 90% of the mice died. Clinical signs of graft-versus-host disease did not occur and splenic atrophy rather than splenomegaly was the rule. Histologically these mice had a lesion of the hematopoietic microenvironment characterized by sinusoidal injury and stromal necrosis. Others have demonstrated injury to hematopoietic stem cells. C3H/He LNC induced AA whereas C3H/HeJ LNC failed to induce AA. The C3H/HeJ strain carries a macrophage defect and these results suggest that a macrophage-like cell may be a mediator of immunological injury in this experimental model. Although all strain combinations evaluated were H-2k identical and Mls mismatched, certain Mls combinations resulted in AA and identical Mls mismatched but different strains did not. Both strong (Mlsd) and weak (Mlsc) stimulating LNC induce AA but simple Mls differences do not explain the AA as similar Mls combinations but different strain combinations fail to induce AA.

Challenges in dengue fever in the elderly: atypical presentation and risk of severe dengue and hospital-acquired infection [corrected].

Directory of Open Access Journals (Sweden)

Emily K Rowe

2014-04-01

Full Text Available BACKGROUND/METHODS: To better understand dengue fever in the elderly, we compared clinical features, World Health Organization (WHO dengue classification and outcomes between adult (<60 and elderly (≥60 dengue patients. We explored the impact of co-morbidity and hospital-acquired infection (HAI on clinical outcomes in the elderly. All patients managed at the Communicable Disease Centre, Singapore, between 2005 and 2008 with positive dengue polymerase chain reaction (PCR or who fulfilled WHO 1997 or 2009 probable dengue criteria with positive dengue IgM were included. RESULTS: Of the 6989 cases, 295 (4.4% were elderly. PCR was positive in 29%. The elderly suffered more severe disease with more dengue haemorrhagic fever (DHF (29.2% vs. 21.4% and severe dengue (SD (20.3% vs. 14.6% (p<0.05. Classic dengue symptoms were more common in the adult group. The elderly were less likely to fulfill WHO 1997 (93.6% vs. 96.4% (p = 0.014, but not WHO 2009 probable dengue (75.3% vs. 71.5%. Time to dengue diagnosis was similar. There was no significant difference in the frequency of warning signs between the two groups, but the elderly were more likely to have hepatomegaly (p = 0.006 and malaise/lethargy (p = 0.033 while the adults had significantly more mucosal bleeding (p<0.001. Intensive care admission occurred in 15 and death in three, with no age difference. Notably, the elderly stayed in hospital longer (median 5 vs. 4 days, and suffered more pneumonia (3.8% vs. 0.7% and urinary infection (1.9% vs. 0.3% (p = 0.003. Predictors of excess length of stay were age (adjusted odds ratio [aOR] 2.01, 95% confidence interval [CI] 1.37-2.88, critical illness (aOR 5.13, 95%CI 2.59-9.75, HAI (aOR 12.06, 95%CI 7.39-19.9, Charlson score (aOR 6.9, 95%CI 2.02-22.56 and severe dengue (DHF/dengue shock syndrome/SD (aOR 2.24, 95%CI 1.83-2.74. CONCLUSION: Elderly dengue patients present atypically and are at higher risk of DHF, SD and HAI. Aside from dengue

Community-Acquired Pneumonia Visualized on CT Scans but Not Chest Radiographs: Pathogens, Severity, and Clinical Outcomes.

Science.gov (United States)

Upchurch, Cameron P; Grijalva, Carlos G; Wunderink, Richard G; Williams, Derek J; Waterer, Grant W; Anderson, Evan J; Zhu, Yuwei; Hart, Eric M; Carroll, Frank; Bramley, Anna M; Jain, Seema; Edwards, Kathryn M; Self, Wesley H

2018-03-01

The clinical significance of pneumonia visualized on CT scan in the setting of a normal chest radiograph is uncertain. In a multicenter prospective surveillance study of adults hospitalized with community-acquired pneumonia (CAP), we compared the presenting clinical features, pathogens present, and outcomes of patients with pneumonia visualized on a CT scan but not on a concurrent chest radiograph (CT-only pneumonia) and those with pneumonia visualized on a chest radiograph. All patients underwent chest radiography; the decision to obtain CT imaging was determined by the treating clinicians. Chest radiographs and CT images were interpreted by study-dedicated thoracic radiologists blinded to the clinical data. The study population included 2,251 adults with CAP; 2,185 patients (97%) had pneumonia visualized on chest radiography, whereas 66 patients (3%) had pneumonia visualized on CT scan but not on concurrent chest radiography. Overall, these patients with CT-only pneumonia had a clinical profile similar to those with pneumonia visualized on chest radiography, including comorbidities, vital signs, hospital length of stay, prevalence of viral (30% vs 26%) and bacterial (12% vs 14%) pathogens, ICU admission (23% vs 21%), use of mechanical ventilation (6% vs 5%), septic shock (5% vs 4%), and inhospital mortality (0 vs 2%). Adults hospitalized with CAP who had radiological evidence of pneumonia on CT scan but not on concurrent chest radiograph had pathogens, disease severity, and outcomes similar to patients who had signs of pneumonia on chest radiography. These findings support using the same management principles for patients with CT-only pneumonia and those with pneumonia seen on chest radiography. Copyright © 2017 American College of Chest Physicians. All rights reserved.

Hospital-acquired pneumonia is an independent predictor of poor global outcome in severe traumatic brain injury up to 5 years after discharge.

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Kesinger, Matthew Ryan; Kumar, Raj G; Wagner, Amy K; Puyana, Juan Carlos; Peitzman, Andrew P; Billiar, Timothy R; Sperry, Jason L

2015-02-01

Long-term outcomes following traumatic brain injury (TBI) correlate with initial head injury severity and other acute factors. Hospital-acquired pneumonia (HAP) is a common complication in TBI. Limited information exists regarding the significance of infectious complications on long-term outcomes after TBI. We sought to characterize risks associated with HAP on outcomes 5 years after TBI. This study involved data from the merger of an institutional trauma registry and the Traumatic Brain Injury Model Systems outcome data. Individuals with severe head injuries (Abbreviated Injury Scale [AIS] score ≥ 4) who survived to rehabilitation were analyzed. Primary outcome was Glasgow Outcome Scale-Extended (GOSE) at 1, 2, and 5 years. GOSE was dichotomized into low (GOSE score GOSE score ≥ 6). Logistic regression was used to determine adjusted odds of low GOSE score associated with HAP after controlling for age, sex, head and overall injury severity, cranial surgery, Glasgow Coma Scale (GCS) score, ventilation days, and other important confounders. A general estimating equation model was used to analyze all outcome observations simultaneously while controlling for within-patient correlation. A total of 141 individuals met inclusion criteria, with a 30% incidence of HAP. Individuals with and without HAP had similar demographic profiles, presenting vitals, head injury severity, and prevalence of cranial surgery. Individuals with HAP had lower presenting GCS score. Logistic regression demonstrated that HAP was independently associated with low GOSE scores at follow-up (1 year: odds ratio [OR], 6.39; 95% confidence interval [CI], 1.76-23.14; p = 0.005) (2 years: OR, 7.30; 95% CI, 1.87-27.89; p = 0.004) (5-years: OR, 6.89; 95% CI, 1.42-33.39; p = 0.017). Stratifying by GCS score of 8 or lower and early intubation, HAP remained a significant independent predictor of low GOSE score in all strata. In the general estimating equation model, HAP continued to be an independent

Hospital Acquired Pneumonia is an Independent Predictor of Poor Global Outcome in Severe Traumatic Brain Injury up to 5 Years after Discharge

Science.gov (United States)

Kesinger, Matthew R.; Kumar, Raj G.; Wagner, Amy K.; Puyana, Juan C.; Peitzman, Andrew P.; Billiar, Timothy R.; Sperry, Jason L.

2016-01-01

Objectives Long-term outcomes following traumatic brain injury (TBI) correlate with initial head injury severity and other acute factors. Hospital-acquired pneumonia (HAP) is a common complication in TBI. Little information exists regarding the significance of infectious complications on long-term outcomes post-TBI. We sought to characterize risks associated with HAP on outcomes 5 years post-TBI. Methods Ddata from the merger of an institutional trauma registry and the TBI Model Systems outcome data. Individuals with severe head injuries (Abbreviated Injury Scale≥4), who survived to rehabilitation were analyzed. Primary outcome was Glasgow Outcome Scaled-Extended (GOSE) at 1, 2, and 5 years. GOSE was dichotomized into LOW (GOSEGOSE≥6). Logistic regression was utilized to determine adjusted odds of LOW-GOSE associated with HAP after controlling for age, sex, head and overall injury severity, cranial surgery, Glasgow Coma Scale (GCS), ventilation days, and other important confounders. A general estimating equation (GEE) model was used to analyze all outcome observations simultaneously while controlling for within-patient correlation. Results A total of 141 individuals met inclusion criteria, with a 30% incidence of HAP. Individuals with and without HAP had similar demographic profiles, presenting vitals, head injury severity, and prevalence of cranial surgery. Individuals with HAP had lower presenting GCS. Logistic regression demonstrated that HAP was independently associated with LOW-GOSE scores at follow-up (1year: OR=6.39, 95%CI: 1.76-23.14, p=0.005; 2-years: OR=7.30, 95%CI 1.87-27.89, p=0.004; 5-years: OR=6.89, 95%CI: 1.42-33.39, p=0.017). Stratifying by GCS≤8 and early intubation, HAP remained a significant independent predictor of LOW-GOSE in all strata. In the GEE model, HAP continued to be an independent predictor of LOW-GOSE (OR: 4.59; 95%CI: 1.82-11.60′ p=0.001). Conclusion HAP is independently associated with poor outcomes in severe-TBI extending 5

BMT Abatacept for Non-Malignant Diseases

Science.gov (United States)

2018-05-16

Hurler Syndrome; Fanconi Anemia; Glanzmann Thrombasthenia; Wiskott-Aldrich Syndrome; Chronic Granulomatous Disease; Severe Congenital Neutropenia; Leukocyte Adhesion Deficiency; Shwachman-Diamond Syndrome; Diamond-Blackfan Anemia; Dyskeratosis-congenita; Chediak-Higashi Syndrome; Severe Aplastic Anemia; Thalassemia Major; Hemophagocytic Lymphohistiocytosis; Sickle Cell Disease

Multiple myeloma associated with acquired cutis laxa.

Science.gov (United States)

Cho, S Y; Maguire, R F

1980-08-01

Acquired cutis laxa is a rare disorder characterized by diffuse laxity of the skin and loss of connective tissue support with involvement of the lungs, gastrointestinal tract, pelvic organs, and aorta. The case report presented herein describes a forty-six year old woman with multiple myeloma and cutis laxa. Her history included several severe allergic reactions and the gradual development of lax skin, loss of connective tissue support throughout the body, and emphysema. At autopsy, multiple myeloma, diffuse laxity of the skin, and panacinar emphysema were found. The amount of elastic fiber in the skin, lungs, and aorta was decreased and showed abnormal fragmentation. Results of direct immunofluorescence study demonstrated IgG bound to dermal elastic fibers. Speculation regarding an immunologic etiology of the elastic tissue abnormality is presented herein.

Association of the position of a hospital-acquired condition diagnosis code with changes in medicare severity diagnosis-related group assignment.

Science.gov (United States)

Johnson, Tricia; Kane, Jason M; Odwazny, Richard; McNutt, Robert

2014-11-01

Incentives to improve quality include paying less for adverse events, including the Centers for Medicare and Medicaid Services' policy to not pay additionally for events classified as hospital-acquired conditions (HACs). This policy is controversial, as variable coding practices at hospitals may lead to differences in the inclusion and position of HACs in the list of codes used for Medicare Severity Diagnosis-Related Group (MS-DRG) assignment. Evaluate changes in MS-DRG assignment for patients with an HAC and test the association of the position of an HAC in the list of International Classification of Diseases, 9th Revision (ICD-9) diagnosis codes with change in MS-DRG assignment. Retrospective analysis of patients discharged from hospital members of the University HealthSystem Consortium's Clinical Data Base between October 2007 and April 2008. Comparisons were made between the MS-DRG assigned when the HAC was not included in the list of ICD-9 diagnosis codes and the MS-DRG that would have been assigned had the HAC code been included in the assignment. Of the 7027 patients with an HAC, 13.8% changed MS-DRG assignment when the HAC was removed. An HAC in the second position versus third position or lower was associated with a 40-fold increase in the likelihood of MS-DRG change. The position of an HAC in the list of diagnosis codes, rather than the presence of an HAC, is associated with a change in MS-DRG assignment. HACs have little effect on reimbursement unless the HAC is in the second position and patients have minor severity of illness. © 2014 Society of Hospital Medicine.

The dynamics of naturally acquired immunity to Plasmodium falciparum infection.

Directory of Open Access Journals (Sweden)

Mykola Pinkevych

Full Text Available Severe malaria occurs predominantly in young children and immunity to clinical disease is associated with cumulative exposure in holoendemic settings. The relative contribution of immunity against various stages of the parasite life cycle that results in controlling infection and limiting disease is not well understood. Here we analyse the dynamics of Plasmodium falciparum malaria infection after treatment in a cohort of 197 healthy study participants of different ages in order to model naturally acquired immunity. We find that both delayed time-to-infection and reductions in asymptomatic parasitaemias in older age groups can be explained by immunity that reduces the growth of blood stage as opposed to liver stage parasites. We found that this mechanism would require at least two components - a rapidly acting strain-specific component, as well as a slowly acquired cross-reactive or general immunity to all strains. Analysis and modelling of malaria infection dynamics and naturally acquired immunity with age provides important insights into what mechanisms of immune control may be harnessed by malaria vaccine strategists.

Neurosis of acquired helplessness and role of hypoxia in the formation of this disorder in rats.

Science.gov (United States)

Vvedenskaya, O Yu; Avrushchenko, M A; Bol'shakova, T D; Khitrov, N K; Moroz, V V

2003-04-01

Acquisition of instrumental defense response with pain reinforcement uncertainty (25% reinforcement) induced the development of acquired helplessness in 50% rats. Acquired helplessness is characterized by the absence of responses to conditioned (light) and unconditioned stimuli (pain), minor response of plasma corticosterone to learning, gas markers of circulatory cerebral hypoxia (Delta A/V pO2 carotid artery/jugular vein), low sensitivity to severe hypobaric conditions, and high resistance of Purkinje cells in the cerebellum. Piracetam improved learning and prevented the development of acquired helplessness. Local changes in cerebral blood flow and energy deficit in neurons responsible for emotional stress during acquired helplessness impair adaptive capacity, but reduce energy consumption and protect neuronal structures.

Lichen striatus occurring after allogenic peripheral blood stem cell transplantation in an adult with aplastic anemia.

Science.gov (United States)

Mun, Je-Ho; Park, Hyun-Je; Kim, Hoon-Soo; Kim, Su-Han; Ko, Hyun-Chang; Kim, Byung-Soo; Kim, Moon-Bum

2012-02-01

Lichens striatus (LS) is an acquired, self-limiting inflammatory dermatosis that follows the lines of Blaschko. The etiology of the eruption is unknown, but several theories have been proposed with focus on environmental factors, viral infection, cutaneous injury, hypersensitivity, and genetic predisposition. We describe a 19-year-old woman who developed a unilateral linear eruption 17 months after allogenic peripheral blood stem cell transplantation. Histopathology revealed features, which were consistent with LS. To the best of our knowledge, our patient is the first case describing the appearance of LS occurring after allogenic stem cell transplantation. We speculate that this condition represents an unusual form of localized, chronic graft-versus-host disease.

Effects of baseline and early acquired thrombocytopaenia on long-term mortality in patients undergoing percutaneous coronary intervention with bivalirudin.

Science.gov (United States)

Ali, Ziad A; Qureshi, Yasir H; Karimi Galougahi, Keyvan; Poludasu, Shyam; Roye, Swathi; Krishnan, Prakash; Zalewski, Adrian; Shah, Zainab Z; Bhatti, Navdeep; Kalapatapu, Kumar; Mehran, Roxana; Dangas, George; Kini, Annapoorna S; Sharma, Samin K

2016-04-08

Bivalirudin use as a procedural anticoagulant in patients undergoing percutaneous coronary intervention (PCI) is associated with a lower incidence of thrombocytopaenia compared to other antithrombotic agents. We aimed to evaluate the prognostic impact of baseline thrombocytopaenia and early changes in platelet counts among patients undergoing PCI with exclusive use of bivalirudin. We evaluated 7,505 patients who underwent PCI over a period of eight years. Patients who received unfractionated heparin and glycoprotein IIb/IIIa receptor inhibitors were specifically excluded. Eight hundred and fifty-eight (11.4%) patients had baseline thrombocytopaenia and 451 (6.0%) developed acquired thrombocytopaenia. After adjustment for potential covariates, moderate to severe acquired thrombocytopaenia was the strongest independent predictor (HR 4.34, 95% CI: 2.13-8.84; pevents, which included major adverse cardiac events and major bleeding complications. Age, male gender, baseline platelet count and intra-aortic balloon pump (IABP) insertion were independent predictors of in-hospital acquired thrombocytopaenia. After a mean follow-up of 2.6±1.7 years, moderate to severe baseline thrombocytopaenia (HR 2.42, 95% CI: 1.79-3.29; p67 k) were significant predictors of mortality. In patients undergoing PCI with bivalirudin, moderate to severe baseline and acquired thrombocytopaenia along with severe changes in platelet count are associated with higher long-term mortality.

Arthritis in adults with community-acquired bacterial meningitis: a prospective cohort study

NARCIS (Netherlands)

Weisfelt, M.; van de Beek, D.; Spanjaard, L.; de Gans, J.

2006-01-01

Background: Although the coexistence of bacterial meningitis and arthritis has been noted in several studies, it remains unclear how often both conditions occur simultaneously. Methods: We evaluated the presence of arthritis in a prospective nationwide cohort of 696 episodes of community-acquired

Therapeutic Effects of Hydrogen-Rich Solution on Aplastic Anemia in Vivo

Directory of Open Access Journals (Sweden)

Sanhu Zhao

2013-08-01

recovery of either hematological or immunological recovery on aplastic anemia mice. This finding suggests hydrogen-rich solution as a potential clinical therapeutic agent for AA.

[Fungemia and septic arthritis caused by Saprochaete capitata in a patient with fanconi aplastic anemia: a case report].

Science.gov (United States)

Parkan, Ömür Mustafa; Atalay, Mustafa Altay; Koç, Ayşe Nedret; Pala, Çiğdem; Aydemir, Gonca; Kaynar, Leylagül

2017-01-01

Saprochaete capitata (formerly known as Blastoschizomyces capitatus, Trichosporon capitatum, Geotrichum capitatum) is a rare but emerging yeast-like fungus. It is commonly found in environmental sources and can be isolated from skin, gastrointestinal system and respiratory tract of healthy individuals as well. It mainly infects patients with hematological malignancies such as acute myeloid leukemia (AML), especially in the presence of neutropenia; and mortality rates are high in those patients. Although the data about the in vitro antifungal susceptibility are limited, it is being reported that amphotericin B and voriconazole are more effective on S.capitata isolates whereas caspofungin had no activity. Here, we report a case of fungemia and septic arthritis due to S.capitata in a patient with Fanconi aplastic anemia. A 22-year-old male patient with Fanconi aplastic anemia was hospitalized in our hematology department for bone marrow transplantation. Two days after the hospitalization, neutropenic fever developed and multiple nodules similar to candidiasis were detected in his liver with the whole abdomen magnetic resonance imaging (MRI). Caspofungin treatment (single 70 mg/kg loading dose, followed by 1 x 50 mg/kg/day) was started. The patient remained febrile, and his blood culture yielded S.capitata. The treatment regimen was changed to a combination of liposomal amphotericin B (3 mg/kg/day) and voriconazole (2 x 4 mg/kg/day). A few days later, pain and swelling came out on patient's left knee and he underwent a surgical process with the prediagnosis of septic arthritis. Culture of synovial fluid was also positive for S.capitata. On the 26th day of the hospitalization, the patient died due to sepsis and multiple organ failure. Patient's blood and synovial fluid samples were incubated in BacT/Alert automated blood culture system (bioMérieux, France). After receiving the growth signal, yeast cells were seen in Gram staining and cream-coloured, wrinkled, yeast

Reduced dose cyclophosphamide, fludarabine and antithymocyte globulin for sibling and unrelated transplant of children with severe and very severe aplastic anemia.

Science.gov (United States)

Chung, Nack-Gyun; Lee, Jae Wook; Jang, Pil-Sang; Jeong, Dae-Chul; Cho, Bin; Kim, Hack-Ki

2013-06-01

We evaluated the results of a novel conditioning regimen of reduced dose cyclophosphamide (Cy, 25 mg/kg for four days), fludarabine (Flu, 30 mg/m(2) for four days), and rabbit ATG (2.5 mg/kg for three days) for allogeneic transplant of children with SAA, implemented since January 2009. Overall, 23 patients were treated with this regimen (16 male, seven female), including 10 diagnosed with VSAA. Donors included eight-MSD and 15 UD (five-matched UD, and 10 mismatched UD). All patients showed neutrophil and platelet engraftment. Cumulative incidence of acute (grade 2 or above) and chronic GVHD was 26.1% and 8.7%, respectively. Estimated two-yr FFS and OS for the entire cohort was 90.3 ± 6.5%. Rates of TRM and graft failure were 5.3% and 4.3%, respectively. No difference in OS was found according to disease severity (SAA vs. VSAA, p = 0.184), or according to donor type (MSD vs. UD, p = 0.699). Excellent outcomes of patients with VSAA underscore the efficacy of allogeneic transplant as a means of expediting hematopoietic recovery. Improved survival of UD transplant reaffirms its role as a valid therapeutic alternative in the absence of MSD. © 2013 John Wiley & Sons A/S.

Chinese Herbal Formula, Modified Danggui Buxue Tang, Attenuates Apoptosis of Hematopoietic Stem Cells in Immune-Mediated Aplastic Anemia Mouse Model

Directory of Open Access Journals (Sweden)

Jingwei Zhou

2017-01-01

Full Text Available A derivative formula, DGBX, which is composed of three herbs (Radix astragali, Radix Angelicae sinensis, and Coptis chinensis Franch, is derived from a famous Chinese herbal formula, Danggui Buxue Tang (DBT (Radix astragali and Radix Angelicae sinensis. We aimed to investigate the effects of DGBX on the regulation of the balance between proliferation and apoptosis of hematopoietic stem cells (HSCs due to the aberrant immune response in a mouse model of aplastic anemia (AA. Cyclosporine (CsA, an immunosuppressor, was used as the positive control. Our results indicated that DGBX could downregulate the production of IFNγ in bone marrow cells by interfering with the binding between SLAM and SAP and the expressions of Fyn and T-bet. This herbal formula can also inhibit the activation of Fas-mediated apoptosis, interferon regulatory factor-1-induced JAK/Stat, and eukaryotic initiation factor 2 signaling pathways and thereby induce proliferation and attenuate apoptosis of HSCs. In conclusion, DGBX can relieve the immune-mediated destruction of HSCs, repair hematopoietic failure, and recover the hematopoietic function of HSCs in hematogenesis. Therefore, DGBX can be used in traditional medicine against AA as a complementary and alternative immunosuppressive therapeutic formula.

Brain injury and severe eating difficulties at admission

DEFF Research Database (Denmark)

Kjærsgaard, Annette; Kaae Kristensen, Hanne

Objective: The objective of this pilot study was to explore and interpret the way that individuals with acquired brain injury, admitted to inpatient neurorehabilitation with severe eating difficulties, experienced eating nine to fifteen months after discharge. Methods: Four individuals with acqui......Objective: The objective of this pilot study was to explore and interpret the way that individuals with acquired brain injury, admitted to inpatient neurorehabilitation with severe eating difficulties, experienced eating nine to fifteen months after discharge. Methods: Four individuals...... with acquired brain injury were interviewed via qualitative semi-structured interviews. An explorative study was conducted to study eating difficulties. Qualitative content analysis was used. Results: Four main themes emerged from the analysis: personal values related to eating, swallowing difficulties, eating......-of-life. The preliminary findings provide knowledge regarding the patient perspective of adapting to and developing new strategies for activities related to eating, however, further prospective, longitudinal research in a larger scale and with repeated interviews is needed....

The acquired hyperostosis syndrome. Pt. 2

International Nuclear Information System (INIS)

Dihlmann, W.; Hering, L.; Bargon, G.W.

1988-01-01

In the second part of this publication, we describe some additional findings in cases of sternocostoclavicular hyperostosis (SCCH). These include focal hyperostosis of the spine, in the pelvis and in the extremities and psoriatric skin lesions and severe forms of acne (acne conglobata, acne fulminans). An analysis of our 13 patients and of the relevant literature indicates that the hyperostosis is due to increased bone metabolism and heterotopic ossification of fibrous tissue and that these are the pathogenic bases of the changes in the axial skeleton, the pelvis and the bones of the extremities. We have suggested a scheme which would categorise the syndrom into complete, incomplete and possibly acquired forms. (orig./GDG) [de

Community-acquired pneumonia.

Science.gov (United States)

Falguera, M; Ramírez, M F

2015-11-01

This article not only reviews the essential aspects of community-acquired pneumonia for daily clinical practice, but also highlights the controversial issues and provides the newest available information. Community-acquired pneumonia is considered in a broad sense, without excluding certain variants that, in recent years, a number of authors have managed to delineate, such as healthcare-associated pneumonia. The latter form is nothing more than the same disease that affects more frail patients, with a greater number of risk factors, both sharing an overall common approach. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Medicina Interna (SEMI). All rights reserved.

Comparison of immune manifestations between refractory cytopenia of childhood and aplastic anemia in children: A single-center retrospective study.

Science.gov (United States)

Wu, Jun; Cheng, Yifei; Zhang, Leping

2015-12-01

This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis. Copyright © 2015 Elsevier Ltd. All rights reserved.

Severe Weather Field Experience: An Undergraduate Field Course on Career Enhancement and Severe Convective Storms

Science.gov (United States)

Godfrey, Christopher M.; Barrett, Bradford S.; Godfrey, Elaine S.

2011-01-01

Undergraduate students acquire a deeper understanding of scientific principles through first-hand experience. To enhance the learning environment for atmospheric science majors, the University of North Carolina at Asheville has developed the severe weather field experience. Participants travel to Tornado Alley in the Great Plains to forecast and…

Impact of chronic liver disease in intensive care unit acquired pneumonia: a prospective study.

Science.gov (United States)

Di Pasquale, Marta; Esperatti, Mariano; Crisafulli, Ernesto; Ferrer, Miquel; Bassi, Gianluigi Li; Rinaudo, Mariano; Escorsell, Angels; Fernandez, Javier; Mas, Antoni; Blasi, Francesco; Torres, Antoni

2013-10-01

To assess the impact of chronic liver disease (CLD) on ICU-acquired pneumonia. This was a prospective, observational study of the characteristics, microbiology, and outcomes of 343 consecutive patients with ICU-acquired pneumonia clustered according to the presence of CLD. Sixty-seven (20%) patients had CLD (67% had liver cirrhosis, LC), MELD score 26 ± 9, 20% Child-Pugh class C). They presented higher severity scores than patients without CLD both on admission to the ICU (APACHE II, LC 19 ± 6 vs. other CLD 18 ± 6 vs. no CLD 16 ± 6; p CLD patients. LC patients had higher 28- and 90-day mortality (63 vs. 28%, p CLD patients. Presence of LC was independently associated with decreased 28- and 90-day survival (95% confidence interval [CI], 1.982-17.250; p = 0.001; 95% confidence interval [CI], 2.915-20.699, p = 0.001, respectively). In critically ill patients with ICU-acquired pneumonia, CLD is associated with a more severe clinical presentation and poor clinical outcomes. Moreover, LC is independently associated with 28- and 90-day mortality. The results of this study are important for future trials focused on mortality.

Independent radiographic prognostic factors in patients with hospital-treated community-acquired pneumonia

International Nuclear Information System (INIS)

Wilhelm, K.; Textor, J.; Schild, H.; Ewig, S.; Luederitz, B.; Krollmann, G.

1999-01-01

Purpose: To evaluate the independent prognostic impact of the chest radiograph for mortality from community-acquired pneumonia requiring hospitalization. Methods: Chest radiographs of 67 patients with hospital-treated community-acquired pneumonia were analyzed with regard to the prognostic implications of radiographic patterns, extent and density of infiltrates, and its evolution during treatment. Results: Non-survivors had a significantly higher extent of infiltrates (p=0.008), density of infiltrates (p=0.05), and radiographic spread during follow-up within 48-72 hours (p=0.0001). In multivariate analysis, persistent or progressive infiltrates were associated with a 47fold increase, and persistent or progressive density of infiltrates with an 18fold increase in risk of mortality. The presence of both parameters could correctly predict 96% of survivors and 90% of non-survivors. Conclusions: The chest radiograph is an independent predictor of the severity of pneumonia. Both persistent or progressive infiltrates and persistent or progressive density of infiltrates are independently associated with mortality from community-acquired pneumonia. (orig.) [de

Iris recognition: on the segmentation of degraded images acquired in the visible wavelength.

Science.gov (United States)

Proença, Hugo

2010-08-01

Iris recognition imaging constraints are receiving increasing attention. There are several proposals to develop systems that operate in the visible wavelength and in less constrained environments. These imaging conditions engender acquired noisy artifacts that lead to severely degraded images, making iris segmentation a major issue. Having observed that existing iris segmentation methods tend to fail in these challenging conditions, we present a segmentation method that can handle degraded images acquired in less constrained conditions. We offer the following contributions: 1) to consider the sclera the most easily distinguishable part of the eye in degraded images, 2) to propose a new type of feature that measures the proportion of sclera in each direction and is fundamental in segmenting the iris, and 3) to run the entire procedure in deterministically linear time in respect to the size of the image, making the procedure suitable for real-time applications.

48 CFR 1845.502-70 - Contractor-acquired property.

Science.gov (United States)

2010-10-01

... 48 Federal Acquisition Regulations System 6 2010-10-01 2010-10-01 true Contractor-acquired... Possession of Contractors 1845.502-70 Contractor-acquired property. All contractor-acquired property must be... contractor-acquired. (2) Submission of DD Form 1419, DOD Industrial Plant Requisition, or equivalent format...

The VCU Pressure Ulcer Summit: Collaboration to Operationalize Hospital-Acquired Pressure Ulcer Prevention Best Practice Recommendations.

Science.gov (United States)

Brindle, C Tod; Creehan, Sue; Black, Joyce; Zimmermann, Deb

2015-01-01

This executive summary reports outcomes of an interprofessional collaboration between experts in pressure ulcer prevention, bedside clinicians, regulatory agencies, quality improvement, informatics experts, and professional nursing organizations. The goal of the collaboration was to develop a framework to assist facilities to operationalize best practice recommendations to sustain organizational culture change in hospital-acquired pressure ulcer prevention, to develop a hospital-acquired pressure ulcer severity score, and to address topics related to the unavoidable pressure ulcer.

Acquiring taste in home economics?

DEFF Research Database (Denmark)

Stenbak Larsen, Christian

Objective: To explore how home economics was taught in Denmark before the recent Danish school reform, which also revised the objectives and content of home economics, naming it Food Knowledge (Madkundskab) Methods: Participant observation was done in home economic lessons in two case schools...... appreciated by the group of boys, and others again learned to stick with their idiosyncrasies when pressured by the teacher. Conclusions: Children were acquiring taste in the home economic lessons, but not only the kind of tastes that the teacher had planned for. This leads to reflections on the very complex...... process of taste acquiring and to a call for further research into taste acquiring in complex real life contexts as home economics lessons....

Acquired bleeding disorders

African Journals Online (AJOL)

B one marrow aplasia ... Laboratory approach to a suspected acquired bleeding disorder. (LER = leuko- .... lymphocytic leukaemia, and lymphoma). ... cells), a bone marrow aspirate and trephine biopsy (BMAT) is not ..... transplantation.

Thinking Allowed: Use of Egocentric Speech after Acquired Brain Injury (ABI)

Science.gov (United States)

Rees, Sian A.; Skidmore, David

2011-01-01

This paper explores the use of thinking aloud made by young people who have sustained a severe acquired brain injury (ABI). The phenomenon is compared with the concepts of egocentric speech and inner speech before the form of thinking aloud by pupils with ABI is examined. It is suggested that by using thinking aloud, this group of pupils is able…

Clinical study of serum interleukin-6 in children with community-acquired pneumonia

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Ahmed A. Khattab

2018-06-01

Full Text Available Background: Community-acquired pneumonia (CAP is an important childhood killer. Excessive production of cytokines, including interleukin-6 (IL-6, might be associated with severe disease course but pediatric data is limited. Aim: To assess value of IL-6 in predicting CAP severity in children. Methods: A prospective study conducted on 73 children hospitalized for CAP and 15 healthy controls. Pneumonia severity was evaluated according to World Health Organization (WHO classification, Respiratory Index of Severity Score (RISC, Predisposition, Insult, Response, Organ dysfunction modified (PIROm score, and Pediatric Respiratory Severity Score (PRESS. Serum IL-6 was measured within 24 h of admission. The primary outcome was occurrence of any pneumonia complications or death within 30 days. Results: IL-6 was significantly higher among patients compared with controls. Unlike CRP, IL-6 was significantly higher among children with severe pneumonia as determined by WHO, PRESS, and RISC (p = 0.001 for all. IL-6 was significantly higher among children with PICU admission, mechanical ventilation, shock (p = 0.001 for all, hypoxia (p < 0.001, and lobar consolidation (p = 0.042. IL-6 had positive correlations with PRESS (rs=0.8, P < 0.001, RISC (rs=0.6, p < 0.001, and PIROm (rs=0.59, p < 0.001 while a negative correlation was found with Oxygen saturation [r = −0.61, p = 0.001]. IL-6 was not significantly correlated with CRP. Receiver Operating Characteristic curve (ROC analysis revealed large area under the curve (AUC of IL-6 for prediction of severe pneumonia as classified by WHO, PRESS, and RISC (AUC = 0.95, 0.94, and 0.89 respectively. Conclusion: IL-6 appears to be valuable for assessment of CAP severity in children compared with conventional biomarkers. Keywords: Interleukin-6, Community acquired pneumonia, C-reactive protein, Prognosis, Pediatric

Chronic obstructive pulmonary disease severity is associated with severe pneumonia

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Jung Seop Eom

2015-01-01

Full Text Available CONTEXT: Chronic obstructive pulmonary disease (COPD is a heterogeneous disorder, and various aspects of COPD may be associated with the severity of pneumonia in such patients. AIMS: We examined the risk factors associated with severe pneumonia in a COPD population. MATERIALS AND METHODS: We performed a retrospective observational study using a prospectively collected database of pneumonia patients who were admitted to our hospital through emergency department between 2008 and 2012. Patients with hospital-acquired pneumonia and those with an immunocompromised status were excluded. RESULTS: Of 148 pneumonia patients with COPD for whom chest computed tomography (CT scans were available, 106 (71.6% and 42 (28.4% were classified as non-severe and severe pneumonia, respectively. Multivariate logistic regression analysis revealed that the severity of airflow limitation [odds ratio (OR, 2.751; 95% confidence interval (CI, 1.074-7.050; P = 0.035] and the presence of emphysema on a chest CT scan (OR, 3.366; 95% CI, 1.104-10.265; P = 0.033 were independently associated with severe pneumonia in patients with COPD. CONCLUSIONS: The severity of COPD including the airflow limitation grade and the presence of pulmonary emphysema were independently associated with the development of severe pneumonia.

Severe aortic valve stenosis and nosebleed

NARCIS (Netherlands)

Schoedel, Johannes; Obergfell, Achim; Maass, Alexander H.; Schodel, Johannes

2007-01-01

Aortic valve stenosis is known to be associated with loss of high molecular von Willebrand multimers. This can lead to gastrointestinal bleeding in patients with gastrointestinal angiodysplasia, the Heyde syndrome. Here we present a case of anaemia and severe epistaxis associated with acquired von

Severe pneumococcal pneumonia: impact of new quinolones on prognosis

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Meybeck Agnes

2011-03-01

Full Text Available Abstract Background Most guidelines have been proposing, for more than 15 years, a β-lactam combined with either a quinolone or a macrolide as empirical, first-line therapy of severe community acquired pneumonia (CAP requiring ICU admission. Our goal was to evaluate the outcome of patients with severe CAP, focusing on the impact of new rather than old fluoroquinolones combined with β-lactam in the empirical antimicrobial treatments. Methods Retrospective study of consecutive patients admitted in a 16-bed general intensive care unit (ICU, between January 1996 and January 2009, for severe (Pneumonia Severity Index > or = 4 community-acquired pneumonia due to non penicillin-resistant Streptococcus pneumoniae and treated with a β-lactam combined with a fluoroquinolone. Results We included 70 patients of whom 38 received a β-lactam combined with ofloxacin or ciprofloxacin and 32 combined with levofloxacin. Twenty six patients (37.1% died in the ICU. Three independent factors associated with decreased survival in ICU were identified: septic shock on ICU admission (AOR = 10.6; 95% CI 2.87-39.3; p = 0.0004, age > 70 yrs. (AOR = 4.88; 95% CI 1.41-16.9; p = 0.01 and initial treatment with a β-lactam combined with ofloxacin or ciprofloxacin (AOR = 4.1; 95% CI 1.13-15.13; p = 0.03. Conclusion Our results suggest that, when combined to a β-lactam, levofloxacin is associated with lower mortality than ofloxacin or ciprofloxacin in severe pneumococcal community-acquired pneumonia.

Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia

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Ramesh Kumar Pandey

2017-01-01

Full Text Available Introduction. Acquired thrombotic thrombocytopenic purpura (TTP has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests’ results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10% with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved. Discussion. While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization.

Acquired pulmonary artery stenosis in four dogs.

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Scansen, Brian A; Schober, Karsten E; Bonagura, John D; Smeak, Daniel D

2008-04-15

4 dogs with acquired pulmonary artery stenosis (PAS) were examined for various clinical signs. One was a mixed-breed dog with congenital valvular PAS that subsequently developed peripheral PAS, one was a Golden Retriever with pulmonary valve fibrosarcoma, one was a Pembroke Welsh Corgi in which the left pulmonary artery had inadvertently been ligated during surgery for correction of patent ductus arteriosus, and one was a Boston Terrier with a heart-base mass compressing the pulmonary arteries. All 4 dogs were evaluated with 2-dimensional and Doppler echocardiography to characterize the nature and severity of the stenoses; other diagnostic tests were also performed. The mixed-breed dog with valvular and peripheral PAS was euthanized, surgical resection of the pulmonic valve mass was performed in the Golden Retriever, corrective surgery was performed on the Pembroke Welsh Corgi with left pulmonary artery ligation, and the Boston Terrier with the heart-base mass was managed medically. Acquired PAS in dogs may manifest as a clinically silent heart murmur, syncope, or right-sided heart failure. The diagnosis is made on the basis of imaging findings, particularly results of 2-dimensional and Doppler echocardiography. Treatment may include surgical, interventional, or medical modalities and is targeted at resolving the inciting cause.

Iron-Deficiency Anemia

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Full Text Available ... making new blood cells. Visit our Aplastic Anemia Health Topic to learn more. ... recommend that you take iron supplements, also called iron pills or oral iron, by mouth once or several times a ...

Systolic blood pressure is superior to other haemodynamic predictors of outcome in community acquired pneumonia.

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Chalmers, J D; Singanayagam, A; Hill, A T

2008-08-01

Admission blood pressure (BP) assessment is a central component of severity assessment for community acquired pneumonia. The aim of this study was to establish which readily available haemodynamic measure on admission is most useful for predicting severity in patients admitted with community acquired pneumonia. A prospective observational study of patients admitted with community acquired pneumonia was conducted in Edinburgh, UK. The measurements compared were systolic and diastolic BP, mean arterial pressure and pulse pressure. The outcomes of interest were 30 day mortality and the requirement for mechanical ventilation and/or inotropic support. Admission systolic BP pressure pressure AUC values for each predictor of 30 day mortality were as follows: systolic BP pressure pressure AUC values for each predictor of need for mechanical ventilation and/or inotropic support were as follows: systolic BP pressure pressure blood pressure AUC 0.76 vs 0.74) and to the standard CURB65 score (0.76 vs 0.76) for the prediction of 30 day mortality. The simplified CRB65 score was equivalent for prediction of mechanical ventilation and/or inotropic support to standard CRB65 (0.77 vs 0.77) and to CURB65 (0.77 vs 0.78). Systolic BP is superior to other haemodynamic predictors of 30 day mortality and need for mechanical ventilation and/or inotropic support in community acquired pneumonia. The CURB65 score can be simplified to a modified CRB65 score by omission of the diastolic BP criterion without compromising its accuracy.

Somatically acquired structural genetic differences

DEFF Research Database (Denmark)

Magaard Koldby, Kristina; Nygaard, Marianne; Christensen, Kaare

2016-01-01

Structural genetic variants like copy number variants (CNVs) comprise a large part of human genetic variation and may be inherited as well as somatically acquired. Recent studies have reported the presence of somatically acquired structural variants in the human genome and it has been suggested t...... with age.European Journal of Human Genetics advance online publication, 20 April 2016; doi:10.1038/ejhg.2016.34....

Comparative efficacy of amoxicillin, cefuroxime and clarithromycin in the treatment of community acquired pneumonia in children

International Nuclear Information System (INIS)

Aurangzeb, B.; Hameed, A.

2003-01-01

Objective: To compare the clinical response to amoxicillin, cefuroxime and clarithromycin in the treatment of community-acquired pneumonia in children and to see the cost effectiveness of each treatment. Subjects and Methods: Patients between 3 to 72 months of age, admitted in the hospital with community acquired pneumonia, were randomly divided into three groups 1,2,3. They were started on amoxicillin, cefuroxime and clarithromycin respectively. The patients were assessed daily. If there was no clinical improvements at 48 hours the antibiotic was changed. ANOVA statistical test was applied to see the clinical response to the treatment in the three groups. Cost effectiveness of the treatment was compared. Results: There was no statistical difference in the clinical response at 48 hours of initiating treatment and discharge (p>0.01 each). The mean hospital stay in group 1 and 2 was 3.3 days and group 3 was 3.2 days respectively (p>0.01). Ninety-seven percent patients in group 1 and 3, and 95% patients in group 2 showed clinical improvement. The cost of treatment of community acquired pneumonia for 8 days was Rs. 496/-, 730/-, 1018/- for amoxicillin, clarithromycin and cefuroxime respectively. Conclusion: Amoxicillin was found in the most cost effective followed by clarithromycin and cefuroxime respectively in the treatment of non-severe and severe community-acquired pneumonia. (author)

Yeasts acquire resistance secondary to antifungal drug treatment by adaptive mutagenesis.

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David Quinto-Alemany

Full Text Available Acquisition of resistance secondary to treatment both by microorganisms and by tumor cells is a major public health concern. Several species of bacteria acquire resistance to various antibiotics through stress-induced responses that have an adaptive mutagenesis effect. So far, adaptive mutagenesis in yeast has only been described when the stress is nutrient deprivation. Here, we hypothesized that adaptive mutagenesis in yeast (Saccharomyces cerevisiae and Candida albicans as model organisms would also take place in response to antifungal agents (5-fluorocytosine or flucytosine, 5-FC, and caspofungin, CSP, giving rise to resistance secondary to treatment with these agents. We have developed a clinically relevant model where both yeasts acquire resistance when exposed to these agents. Stressful lifestyle associated mutation (SLAM experiments show that the adaptive mutation frequencies are 20 (S. cerevisiae -5-FC, 600 (C. albicans -5-FC or 1000 (S. cerevisiae--CSP fold higher than the spontaneous mutation frequency, the experimental data for C. albicans -5-FC being in agreement with the clinical data of acquisition of resistance secondary to treatment. The spectrum of mutations in the S. cerevisiae -5-FC model differs between spontaneous and acquired, indicating that the molecular mechanisms that generate them are different. Remarkably, in the acquired mutations, an ectopic intrachromosomal recombination with an 87% homologous gene takes place with a high frequency. In conclusion, we present here a clinically relevant adaptive mutation model that fulfils the conditions reported previously.

Effect of severe anaemia on renal function: a case-control study

International Nuclear Information System (INIS)

Kumar, A.; Hentok, P.; Chandrashekar, N.; Thomas, E.J.; Tripathi, M.; Bal, C.S.; Ghosh, A.; Jailkhani, B.L.; Malhotra, O.P.

2002-01-01

Aim: Anaemia, if severe, causes multi systemic functional changes. We tried to find out the effect of severe anaemia on renal function. Materials and Methods: A total of 66 patients with severe anemia and 10 healthy controls were recruited in this study. The cases were divided into following groups: group A: patients with Hb≤3 gm/dl (n=33); group B: patients with Hb≤6 but > 3 gm/dl (n=33); group C: healthy controls with normal renal function and Hb>12gm/dl. Out of 66 anaemic patients, 36 had nutritional anaemia (mainly iron deficiency; group A=20, group B=16), 24 patients were suffering from aplastic anaemia (group A=11, group B=13) and rest 6 had megaloblastic anaemia (group A=2, group B=4). No subject had hypertension, diabetes, primary renal dysfunction or any other systemic illness, affecting kidney. Various renal function test parameters and diagnostic renal failure indices were obtained for all subjects. GFR with 2-sample method after injection of 99m-Tc DTPA and ERPF with single sample method after injection of 131-I OIH were also calculated. Results: Fourteen patients had mild to moderate pedal edema (10 in group A and 4 in group B). Out of these patients, 8 had palpable liver and signs of systemic congestion. Signs of raised systemic venous pressure (raised JVP) were found in 7 patients of group A. In about 55% of patients, chest x-ray showed mild to moderately enlarged heart with disturbed cardiophrenic angle. Urine output was >600 ml/day in all cases. Results are presented. All renal functional parameters and indices were significantly reduced in anaemic patients and were suggestive of pre-renal failure. The reduction was correlating well with the severity of anaemia.Conclusion: Severe anaemia leads to renal dysfunction with alteration of minor and major renal failure indices, which can be characterized by sub-clinical and pre-biochemical non-oliguric pre-renal failure

[Effective immunosuppresive therapies including steroid pulse treatment for intramuscular hematoma in iliopsoas in acquired hemophilia].

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Mohri, Hiroshi; Tanabe, Juichi; Takagi, Hiroshi; Murata, Takashi

2007-12-01

Acquired hemophilia is a life-threatening bleeding disorder by the development of autoantibody against factor VIII. The therapeutic approach relies on steroid, cyclophosphamide and/or cyclosporine. A 64-year-old man was referred to our hospital with extensive hematoma in both psoas muscles, severe anemia of 6.8 g/dl, prolonged activated partial thromboplastin time over 200 seconds, and factor VIII coagulation activity (FVIII: C) of 1.9%. A factor VIII inhibitor was detected at 118 Bethesda units (BU). The diagnosis of acquired hemophilia was made in the absence of a detectable cause. The inhibitor was IgG with a subclass of IgG4 and reacted with 72 kDa fragment of factor VIII light chain. Steroid pulse therapy following steroid treatment resulted in the resolution of acquired hemophila with marked and prolonged efficacy.

Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).

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Knoebl, P; Marco, P; Baudo, F; Collins, P; Huth-Kühne, A; Nemes, L; Pellegrini, F; Tengborn, L; Lévesque, H

2012-04-01

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA. © 2012 International Society on Thrombosis and Haemostasis.

Penicillin treatment for patients with Community-Acquired Pneumonia in Denmark

DEFF Research Database (Denmark)

Egelund, Gertrud Baunbæk; Jensen, Andreas Vestergaard; Andersen, Stine Bang

2017-01-01

BACKGROUND: Community-acquired pneumonia (CAP) is a severe infection, with high mortality. Antibiotic strategies for CAP differ across Europe. The objective of the study was to describe the epidemiology of CAP in Denmark and evaluate the prognosis of patients empirically treated with penicillin......-G/V was commonly used and not associated with increased mortality in patients with mild to moderate pneumonia. Our results are in agreement with current conservative antibiotic strategy as outlined in the Danish guidelines....

Ability to manage everyday technology after acquired brain injury.

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Kassberg, Ann-Charlotte; Malinowsky, Camilla; Jacobsson, Lars; Lund, Maria Larsson

2013-01-01

To investigate and describe how persons with an acquired brain injury (ABI) manage everyday technology (ET) in their daily activities and to explore whether the ability to manage ET was related to the severity of the disability. Eighty-one persons with ABI were observed while managing ET by using the Management of Everyday Technology Assessment (META). The Glasgow Outcome Scale-Extended (GOSE) was used to assess the severity of disability after the ABI. A computer application of a Rasch measurement model was used to generate measures of the participants' ability to manage ET and the measures were compared groupwise with analysis of covariance (ANCOVA). The degree of severity of disability had a significant main effect on the ability to manage ET. The groups with severe and moderate disability exhibited a significantly lower ability to manage ET compared to the group with good recovery. The result indicates that the ability to manage ET in daily activities can be related to the global severity of disability after ABI. This demonstrates the importance of considering the ability to manage ET to support the performance of activities at home, at work and in society in persons with ABI.

Extracorporeal Membrane Oxygenation for Adult Community-Acquired Pneumonia: Outcomes and Predictors of Mortality.

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Ramanathan, Kollengode; Tan, Chuen Seng; Rycus, Peter; MacLaren, Graeme

2017-05-01

Extracorporeal membrane oxygenation is a rescue therapy used to support severe cardiorespiratory failure. Data on outcomes from severe community-acquired pneumonia in adults receiving rescue extracorporeal membrane oxygenation are mainly confined to single-center experiences or specific pathogens. We examined data from the Extracorporeal Life Support Organisation registry to identify risk factors for poor outcomes in adult patients with community-acquired pneumonia. Retrospective data analysis. Extracorporeal Life Support Organization Registry database. We collected deidentified data on adult patients (> 18 yr) receiving extracorporeal membrane oxygenation for community-acquired pneumonia between 2002 and 2012. Patients with incomplete data or brain death were excluded. The primary outcome measure was in-hospital mortality. Other measurements included demographic information, pre-extracorporeal membrane oxygenation mechanical ventilation and biochemical variables, inotrope requirements, extracorporeal membrane oxygenation mode, duration, and complications. Initial univariate analysis assessed potential associations between survival and various pre-extracorporeal membrane oxygenation and extracorporeal membrane oxygenation factors. Variables with p values of less than 0.1 were considered for logistic regression analysis to identify predictors of mortality. None. One thousand fifty-five patients, who satisfied inclusion criteria, were included in the final analysis. There was an increase in the number of patients cannulated per annum over the 10-year period studied. Univariate analysis identified pre-extracorporeal membrane oxygenation and extracorporeal membrane oxygenation variables associated with high mortality. Further multiple regression analysis identified certain pre-extracorporeal membrane oxygenation factors as predictors of mortality, including duration of mechanical ventilation prior to extracorporeal membrane oxygenation, lower arterial pressure, fungal

Acquired Inventors’ Productivity after Horizontal Acquisition

DEFF Research Database (Denmark)

Colombo, Massimo G.; Moreira, Solon; Rabbiosi, Larissa

Effective integration of the R&D functions of the acquired and acquiring firms is essential for knowledge recombination after acquisition. However, prior research suggests that the post-acquisition integration process often damages the inventive labor force. We argue that an examination of the mu...

Clinical Predictors of Hospital-acquired Pneumonia Associated with Acute Ischemic

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Alexis Suárez Quesada

2015-06-01

Full Text Available Background: hospital-acquired pneumonia is a constant challenge given the current microbiological spectrum, antimicrobial resistance together with its high mortality, morbidity and hospital costs. Objective: to identify the clinical predictors of pneumonia associated with acute ischemic stroke. Methods: a prospective cohort study was conducted in 201 patients diagnosed with acute ischemic stroke consecutively admitted to the stroke unit of the General Carlos Manuel de Céspedes Teaching Hospital during the first seven days after the onset, from January 2012 through December 2013. The independent predictors of hospital-acquired pneumonia were obtained using multivariable logistic regression. Results: fifty six point seven percent were male. The mean age was 64.17 ± 14.33 years. Cases of hospital-acquired pneumonia associated with stroke accounted for 19, 9 %. Subjects who developed pneumonia were older (68.55 ± 13.51 vs. 63.08 ± 14.36 years, had a lower score in the Glasgow Coma Scale (8.00 ± 2.60 vs. 14.00 ± 2.82, and an increased number of leukocytes at admission (10.888 ± 3.487 vs. 9.233 ± 2.539 × 109/L. The following independent factors were identified: Glasgow Coma Scale ≤ 11 (OR: 26.099; 95 % CI 7.164-85.075, history of chronic obstructive pulmonary disease (OR: 8.896; 95 % CI 1.203-65.779, dysphagia (OR: 7.652; 95 % CI 2.369- 24.720, history of heart failure (OR: 4.583; 95 % CI 1.240- 16.932 and dysarthria/severe motor aphasia (OR: 4.222; 95 % CI 1.374- 12.975. Conclusions: the resulting logistic regression model is valid for predicting post-stroke pneumonia based on data routinely acquired.

[PECULIARITIES OF COMMUNITY-ACQUIRED PNEUMONIA IN CHILDREN WITH NEUROLOGICAL PATHOLOGY].

Science.gov (United States)

Zubarenko, O; Kopiyka, G; Kravchenko, T; Koval, L; Gurienko, K

2017-06-01

Neurological disorders in children highly affect the course of pneumonia, its outcome and the development of possible complications. The aim of the study was to reveal clinical and paraclinical features of community-acquired pneumonia in younger children with neurologic pathology infantile cerebral palsy. Under observation were 37 children with community-acquired pneumonia aged 1 to 3 years that suffered from spastic forms of infantile cerebral palsy. The comparison group consisted of 30 children with community-acquired pneumonia without any concomitant neurological pathology. The age of the children in the comparison and study groups was the same. The results of the study show that the presence of infantile cerebral palsy allow to relate the child to the risk group of respiratory pathology development. The course of community-acquired pneumonia in children affected by infantile cerebral palsy is characterized by rapid progression of symptoms and severity of the condition, and the clinical picture also has a number of characteristic features. Thus, cough, local physical data, classical laboratory signs of inflammation in the form of leukocytosis with neutrophil shift were noticed significantly less often in children with infantile cerebral palsy. The debut of the disease was often accompanied by bronchial obstruction, the inflammatory process was localized in the lower parts of the lungs and often matched the side of the neurologically affected part of the body. Children with cerebral palsy required a longer hospital-stay and a prolonged course of antibiotic therapy. Therefore, the risk of pneumonia in children with infantile cerebral palsy should be taken into account at the primary stage of medical care for the creation of preventive programs.

Acquired high titre factor VIII inhibitor with underlying polyarteritis nodosa.

Science.gov (United States)

Snowden, J A; Hutchings, M; Spearing, R; Patton, W N

1997-05-01

We here present the case of a 70-year-old woman referred to our unit for investigation of bleeding. Investigations confirmed a high titre acquired Factor VIII inhibitor. In association there was relapse of systemic illness associated with anti-neutrophil cytoplasmic antibodies (atypical pattern) for which she had been treated five years previously. Immunosuppression was attempted, but it failed to have an impact both on the inhibitor titre and on the underlying disorder. The patient died from multi-organ failure and massive chest hemorrhage. Post-mortem showed necrotizing vasculitis of medium sized vessels at several sites, including the kidney, consistent with a diagnosis of polyarteritis nodosa. Although it is well recognised that Factor VIII inhibitors are found in conjunction with autoimmune disorders, this case is significant in that it is the first associated with histologically proven polyarteritis nodosa type vasculitis. The case illustrates the difficulties in the investigation and management of patients with acquired high titre Factor VIII inhibitors.

Gaps in patient care practices to prevent hospital-acquired delirium.

Science.gov (United States)

Alagiakrishnan, Kannayiram; Marrie, Thomas; Rolfson, Darryl; Coke, William; Camicioli, Richard; Duggan, D'Arcy; Launhardt, Bonnie; Fisher, Bruce; Gordon, Debbie; Hervas-Malo, Marilou; Magee, Bernice; Wiens, Cheryl

2009-10-01

To evaluate the current patient care practices that address the predisposing and precipitating factors contributing to the prevention of hospital-acquired delirium in the elderly. Prospective cohort (observational) study. Patients 65 years of age and older who were admitted to medical teaching units at the University of Alberta Hospital in Edmonton over a period of 7 months and who were at risk of delirium. Medical teaching units at the University of Alberta. Demographic data and information on predisposing factors for hospital-acquired delirium were obtained for all patients. Documented clinical practices that likely prevent common precipitants of delirium were also recorded. Of the 132 patients enrolled, 20 (15.2%) developed hospital-acquired delirium. At the time of admission several predisposing factors were not documented (eg, possible cognitive impairment 16 [12%], visual impairment 52 [39.4%], and functional status of activities of daily living 99 [75.0%]). Recorded precipitating factors included catheter use, screening for dehydration, and medications. Catheters were used in 35 (26.5%) patients, and fluid intake-and-output charting assessed dehydration in 57 (43.2%) patients. At the time of admission there was no documentation of hearing status in 69 (52.3%) patients and aspiration risk in 104 (78.8%) patients. After admission, reorientation measures were documented in only 16 (12.1%) patients. Although all patients had brief mental status evaluations performed once daily, this was not noted to occur twice daily (which would provide important information about fluctuation of mental status) and there was no formal attention span testing. In this study, hospital-acquired delirium was also associated with increased mortality (P < .004), increased length of stay (P < .007), and increased institutionalization (P < .027). Gaps were noted in patient care practices that might contribute to hospital-acquired delirium and also in measures to identify the development

A case of an immunocompetent young man obtaining community-acquired disseminated Nocardia brasiliensis.

Science.gov (United States)

Li, Jinna; Cao, Jie; Wu, Yueqing; Wan, Nansheng; Pan, Li; Chen, Yuanbao

2014-01-01

Nocardiosis is a rare but severe pyogenic or granulomatous disease and caused by Nocardia that mainly infects immunocompromised patients. We report here a case of an immunocompetent 24-year-old male student with community-acquired pneumonia with asymptomatic disseminated cerebral abscess by Brasiliensis nocardiosis. The patient was fully recovered after receiving optimized antimicrobial therapy without relapse. This case suggests the health professionals such as the physicians of pulmonary, infection, neurology department and et al should always think about unusual cause of community acquired pneumonia, even in immunocompetent patients and when having pulmonary nocardiosis we should do a radiological neurological work up, even with the absence of neurological finding or symptom. Copyright © 2014 Elsevier Inc. All rights reserved.

Acquired ectropion uveae and secondary glaucoma due to trauma: report of 3 cases.

Science.gov (United States)

Markovic, Vujica; Vukovic, Dragan; Radosavljevic, Aleksandra; Marjanovic, Ivan

2017-01-19

To investigate the possible association between acquired ectropion uveae and blunt trauma to the eye. We present 3 cases of acquired ectropion uveae that occurred after blunt trauma to the eye. There are no previously published data on possible association of these conditions. A retrospective review was conducted of patients with ectropion uveae and eye injury at University Eye Hospital over a 10-year period (2006-2016). We analyzed medical records and clinical findings. Three eyes of 3 male patients with ocular trauma and ectropion uveae, ages 71, 68, and 5 years, were reviewed. The period between the eye injury and the diagnosis of ectropion uveae ranged from 10 to 36 months. All 3 eyes developed clinical evidence of secondary glaucoma with moderately to severely elevated intraocular pressure (IOP) (ranging from 29 to 48 mm Hg). Surgical treatment (trabeculectomy) was needed in 2 cases in order to control secondary glaucoma and conservative treatment was sufficient in 1 case. Mean patient follow-up was 19.3 ± 4.6 months. Trauma can be considered as a cause of acquired ectropion uveae. Acquired ectropion uveae following eye trauma may be associated with significant increase in IOP. All patients in our series had secondary glaucoma and 2 of 3 required surgical treatment for IOP control.

Acquired intrathoracic kidney in thoracic kyphosis

International Nuclear Information System (INIS)

Murayama, Sadayuki; Kawashima, Akira; Ohuchida, Toshiyuki; Russell, W.J.

1986-12-01

Two cases of acquired intrathoracic kidney associated with thoracic kyphosis are reported, with emphasis on the radiographic manifestations. A search of the scientific literature disclosed that the acquired type of this abnormality is rare. The importance of recognizing this entity from a differential diagnostic standpoint is underscored. (author)

The Host Response in Patients with Sepsis Developing Intensive Care Unit-acquired Secondary Infections.

Science.gov (United States)

van Vught, Lonneke A; Wiewel, Maryse A; Hoogendijk, Arie J; Frencken, Jos F; Scicluna, Brendon P; Klein Klouwenberg, Peter M C; Zwinderman, Aeilko H; Lutter, Rene; Horn, Janneke; Schultz, Marcus J; Bonten, Marc M J; Cremer, Olaf L; van der Poll, Tom

2017-08-15

Sepsis can be complicated by secondary infections. We explored the possibility that patients with sepsis developing a secondary infection while in the intensive care unit (ICU) display sustained inflammatory, vascular, and procoagulant responses. To compare systemic proinflammatory host responses in patients with sepsis who acquire a new infection with those who do not. Consecutive patients with sepsis with a length of ICU stay greater than 48 hours were prospectively analyzed for the development of ICU-acquired infections. Twenty host response biomarkers reflective of key pathways implicated in sepsis pathogenesis were measured during the first 4 days after ICU admission and at the day of an ICU-acquired infection or noninfectious complication. Of 1,237 admissions for sepsis (1,089 patients), 178 (14.4%) admissions were complicated by ICU-acquired infections (at Day 10 [6-13], median with interquartile range). Patients who developed a secondary infection showed higher disease severity scores and higher mortality up to 1 year than those who did not. Analyses of biomarkers in patients who later went on to develop secondary infections revealed a more dysregulated host response during the first 4 days after admission, as reflected by enhanced inflammation, stronger endothelial cell activation, a more disturbed vascular integrity, and evidence for enhanced coagulation activation. Host response reactions were similar at the time of ICU-acquired infectious or noninfectious complications. Patients with sepsis who developed an ICU-acquired infection showed a more dysregulated proinflammatory and vascular host response during the first 4 days of ICU admission than those who did not develop a secondary infection.

An experimental investigation of the hemodynamic variations due to aplastic vessels within three-dimensional phantom models of the Circle of Willis.

LENUS (Irish Health Repository)

Fahy, Paul

2013-09-10

A complete circle of Willis (CoW) is found in approximately 30-50% of the population. Anatomical variations, such as absent or surgically clamped vessels, can result in undesirable flow patterns. These can affect the brain\\'s ability to maintain cerebral perfusion and the formation of cerebral aneurysms. An experimental test system was developed to simulate cerebral physiological conditions through three flexible 3D patient-specific models of complete and incomplete CoW geometries. Flow visualizations were performed with isobaric dyes and the mapped dye streamlines were tracked throughout the models. Three to seven flow impact locations were observed for all configurations, corresponding to known sites for aneurysmal formation. Uni and bi-directional cross-flows occurred along the communicating arteries. The greatest shunting of flow occurred for a missing pre-communicating anterior (A1) and posterior (P1) cerebral arteries. The anterior cerebral arteries had the greatest reduction (15-37%) in efferent flow rates for missing either a unilateral A1 or bilateral P1 segments. The bi-directional cross-flows, with multiple afferent flow mixing, observed along the communicating arteries may explain the propensity of aneurysm formation at these sites. Reductions in efferent flow rates due to aplastic vessel configurations may affect normal brain function.

Interventions for treating acute bleeding episodes in people with acquired hemophilia A.

Science.gov (United States)

Zeng, Yan; Zhou, Ruiqing; Duan, Xin; Long, Dan; Yang, Songtao

2014-08-28

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation factor VIII (FVIII). In most cases, bleeding episodes are spontaneous and severe at presentation. The optimal hemostatic therapy is controversial. To determine the efficacy of hemostatic therapies for acute bleeds in people with acquired hemophilia A; and to compare different forms of therapy for these bleeds. We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2014, Issue 4) and MEDLINE (Ovid) (1948 to 30 April 2014). We searched the conference proceedings of the: American Society of Hematology; European Hematology Association; International Society on Thrombosis and Haemostasis (ISTH); and the European Association for Haemophilia and Allied Disorders (EAHAD) (from 2000 to 30 April 2014). In addition to this we searched clinical trials registers. All randomised controlled trials and quasi-randomised trials of hemostatic therapies for people with acquired hemophilia A, with no restrictions on gender, age or ethnicity. No trials matching the selection criteria were eligible for inclusion. No trials matching the selection criteria were eligible for inclusion. No randomised clinical trials of hemostatic therapies for acquired hemophilia A were found. Thus, we are not able to draw any conclusions or make any recommendations on the optimal hemostatic therapies for acquired hemophilia A based on the highest quality of evidence. GIven that carrying out randomized controlled trials in this field is a complex task, the authors suggest that, while planning randomised controlled trials in which patients can be enrolled, clinicians treating the disease continue to base their choices on alternative, lower quality sources of evidence, which hopefully, in the future, will also be appraised and incorporated in a Cochrane Review.

And the Winner is – Acquired

DEFF Research Database (Denmark)

Henkel, Joachim; Rønde, Thomas; Wagner, Marcus

value in case of success—that is, a more radical innovation. In the second stage, successful entrants bid to be acquired by the incumbent. We assume that entrants cannot survive on their own, so being acquired amounts to a ‘prize’ in a contest. We identify an equilibrium in which the incumbent chooses...

The role of haloaerosolotherapy in immunorehabilitation of convalescents after community acquired pneumonia

Directory of Open Access Journals (Sweden)

Olha Lemko

2015-02-01

Full Text Available Aim: Investigation of the peculiarities of different haloaerosoltherapy regimes influence (treatment with different intensity of haloaerosol load upon non-specific defense and cellular immunity at convalescents after community acquired pneumonia. Objectives: patients with community acquired pneumonia in the early convalescence period (after completing antibiotic therapy, who received treatment in conditions of artificial rock salt aerosol medium (haloaerosoltherapy. Material and Methods. 42 patients with non-severe community acquired pneumonia were examined in the early convalescence period before and after the course of haloaerosoltherapy, which was prescribed after antibacterial therapy. Immunological studies included: evaluation of phagocytic activity of neutrophils (PhAN - the percentage of phagocytic neutrophils, phagocytic number (PhN - average number of latex particles absorbed by a neutrophil; metabolism of neutrophils in the test with nitroblue tetrasolium (NBT-test spontaneous and induced, which allowed to assess the functional reserve of neutrophils (FR; calculation of cytochemical coefficient (CCC for lysosomal cationic proteins (LCP and for myeloperoxidase (MPO of neutrophils; number of T- and B-lymphocytes and their subpopulations (CD3+ -, CD4+ -, CD8+ -, CD22+ - lymphocytes, calculation the number of 0- lymphocytes and the ratio of CD4+ /CD8+ lymphocytes. Laboratory examinations were also conducted in 21 practically healthy individuals (control group. Two regimes of haloaerosoltherapy were used in recovery treatment of patients with community acquired pneumonia: treating complex №1 (TC-1 with standard haloaerosol load and with increased haloaerosol load (TC-2. Results. After completion the antibiotic therapy at patients with community acquired pneumonia the moderate inhibition of phagocytic activity of neutrophils (47,6±0,58% to 55,5±1,14% in control group remained and was accompanied with a decrease in neutrophil bactericidal

Donor Telomere Length SAA

Science.gov (United States)

A new NCI study has found that, among patients with severe aplastic anemia who received a hematopoietic cell transplant from an unrelated donor, those whose donor white blood cells had longer telomeres had higher survival rates five-years after transplant

Original Research: Parvovirus B19 infection in children with sickle cell disease in the hydroxyurea era

Science.gov (United States)

Penkert, Rhiannon R; Lavoie, Paul; Tang, Li; Sun, Yilun; Hurwitz, Julia L

2016-01-01

Parvovirus B19 infection causes transient aplastic crisis in sickle cell disease (SCD) due to a temporary interruption in the red blood cell production. Toxicity from hydroxyurea includes anemia and reticulocytopenia, both of which also occur during a transient aplastic crisis event. Hydroxyurea inhibits proliferation of hematopoietic cells and may be immunosuppressive. We postulated that hydroxyurea could exacerbate parvovirus B19-induced aplastic crisis and inhibit the development of specific immune responses in children with SCD. We conducted a retrospective review of parvovirus B19 infection in 330 children with SCD. Altogether there were 120 known cases of aplastic crisis attributed to parvovirus B19 infection, and 12% of children were on hydroxyurea treatment during the episode. We evaluated hematological and immune responses. Children with HbSS or HbSβ0-thalassemia treated with hydroxyurea, when compared with untreated children, required fewer transfusions and had higher Hb concentration nadir during transient aplastic crisis. Duration of hospital stays was no different between hydroxyurea-treated and untreated groups. Children tested within a week following aplastic crisis were positive for parvovirus-specific IgG. Immune responses lasted for the duration of the observation period, up to 13 years after transient aplastic crisis, and there were no repeat aplastic crisis episodes. The frequencies of parvovirus-specific antibodies in all children with SCD increased with age, as expected due to the increased likelihood of a parvovirus exposure, and were comparable to frequencies reported for healthy children. Approximately one-third of children had a positive parvovirus B19-specific IgG test without a documented history of transient aplastic crisis, and 64% of them were treated with hydroxyurea. Hydroxyurea may reduce requirements for blood transfusions and may attenuate symptoms during transient aplastic crisis episodes caused by parvovirus B19 infections

Original Research: Parvovirus B19 infection in children with sickle cell disease in the hydroxyurea era.

Science.gov (United States)

Hankins, Jane S; Penkert, Rhiannon R; Lavoie, Paul; Tang, Li; Sun, Yilun; Hurwitz, Julia L

2016-04-01

Parvovirus B19 infection causes transient aplastic crisis in sickle cell disease (SCD) due to a temporary interruption in the red blood cell production. Toxicity from hydroxyurea includes anemia and reticulocytopenia, both of which also occur during a transient aplastic crisis event. Hydroxyurea inhibits proliferation of hematopoietic cells and may be immunosuppressive. We postulated that hydroxyurea could exacerbate parvovirus B19-induced aplastic crisis and inhibit the development of specific immune responses in children with SCD. We conducted a retrospective review of parvovirus B19 infection in 330 children with SCD. Altogether there were 120 known cases of aplastic crisis attributed to parvovirus B19 infection, and 12% of children were on hydroxyurea treatment during the episode. We evaluated hematological and immune responses. Children with HbSS or HbSβ(0)-thalassemia treated with hydroxyurea, when compared with untreated children, required fewer transfusions and had higher Hb concentration nadir during transient aplastic crisis. Duration of hospital stays was no different between hydroxyurea-treated and untreated groups. Children tested within a week following aplastic crisis were positive for parvovirus-specific IgG. Immune responses lasted for the duration of the observation period, up to 13 years after transient aplastic crisis, and there were no repeat aplastic crisis episodes. The frequencies of parvovirus-specific antibodies in all children with SCD increased with age, as expected due to the increased likelihood of a parvovirus exposure, and were comparable to frequencies reported for healthy children. Approximately one-third of children had a positive parvovirus B19-specific IgG test without a documented history of transient aplastic crisis, and 64% of them were treated with hydroxyurea. Hydroxyurea may reduce requirements for blood transfusions and may attenuate symptoms during transient aplastic crisis episodes caused by parvovirus B19 infections

7 CFR 1779.90 - Disposition of acquired property.

Science.gov (United States)

2010-01-01

... Disposition of acquired property. (a) General. When the lender acquires title to the collateral and the final... 7 Agriculture 12 2010-01-01 2010-01-01 false Disposition of acquired property. 1779.90 Section... develop a plan to fully protect the collateral, and the lender must dispose of the collateral without...

Mortality predictors in community-acquired pneumonia | Tanimowo ...

African Journals Online (AJOL)

acquired pneumonia to themedicalwards of Ladoke Akintola University ofTeaching Hospital between Jan. 2003 andDec. 2005. The case notes of 65 patients admitted for community-acquired pneumoniawere studiedwith respect to their admission ...

An extra X does not prevent acquired hemophilia - Pregnancy-associated acquired hemophilia A.

Science.gov (United States)

Barg, Assaf A; Livnat, Tami; Kenet, Gili

2017-03-01

Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). With an estimated annual incidence of 1.3 to 1.5 per million, AHA is a rare disease. An extremely rare form of AHA has been described among women in the peripartum period, and may present with peripartum hemorrhage. Notably, although hemorrhagic symptoms commonly present 1-4 months around delivery, they may occur up to 1 year after parturition. When caring for a mother with AHA it is important to note that Factor VIII inhibitor may be transferred via the placenta from the mother to the fetus. Hence the newborn may also be affected. It is important to increase the awareness of Gynecologists for clinical symptoms and laboratory signs of AHA in order to avoid delayed diagnosis. Treatment may involve use of bypass agents to control hemorrhage, despite the risk of thrombosis, while immunomodulation (with increasing role for Rituximab) may be required to eradicate the inhibiting antibodies. Our review will evaluate the epidemiology, diagnosis, clinical course and treatment of peripartum AHA, focusing upon mother and infant care. © 2017 Elsevier Ltd. All rights reserved.

Evidence for short duration of antibiotic treatment for non-severe community acquired pneumonia (CAP in children - are we there yet? A systematic review of randomised controlled trials

Directory of Open Access Journals (Sweden)

Shalom Ben-Shimol

2014-03-01

Full Text Available Context: The ideal duration of antibiotic treatment for childhood community acquired pneumonia (CAP has not yet been established. Objective: A literature search was conducted to evaluate the efficacy of shorter than 7 days duration of oral antibiotic treatment for childhood non-severe CAP. Data sources: A systematic literature search was performed using the PubMed database. The search was limited to randomised controlled trials (RCTs conducted between January 1996 and May 2013 in children up to 18 years old. Search terms included pneumonia, treatment, duration, child, children, days, short, respiratory infection and non-severe (nonsevere. Study selection: Only RCTs of oral antibiotic treatment for non-severe CAP in children were included. Data extraction: Independent extraction of articles was done by 3 authors using a preformed questionnaire. Data synthesis: Eight articles meeting the selection criteria were identified: 7 from 2 developing countries (India and Pakistan, and 1 from a developed country (The Netherlands. Studies from developing countries used the World Health Organization clinical criteria for diagnosing CAP, which includes mainly tachypnoea. None of those studies included fever, chest radiography or any laboratory test in their case definition. The Dutch study case definition used laboratory tests and chest radiographies (x-rays in addition to clinical criteria. Five articles concluded that 3 days of treatment are sufficient for non-severe childhood CAP, 2 articles found 5 days treatment to be sufficient, and one article found no difference between 3 days of amoxicillin treatment and placebo. Conclusions: The efficacy of short duration oral antibiotic treatment for non-severe CAP in children has not been established in developed countries. Current RCTs from developing countries used clinical criteria that may have failed to appropriately identify children with true bacterial pneumonia necessitating antibiotic treatment. More RCTs

Surveillance for Travel and Domestically Acquired Multidrug-Resistant Human Shigella Infections-Pennsylvania, 2006-2014.

Science.gov (United States)

Li, Yu Lung; Tewari, Deepanker; Yealy, Courtney C; Fardig, David; M'ikanatha, Nkuchia M

2016-01-01

Shigellosis is a leading cause of enteric infections in the United States. We compared antimicrobial resistance in Shigella infections related to overseas travel (travel-associated) and in those acquired domestically by analyzing antimicrobial resistance patterns, geographic distributions, and pulsed-field gel electrophoresis (PFGE) patterns. We tested samples (n = 204) from a collection of isolates recovered from patients in Pennsylvania between 2006 and 2014. Isolates were grouped into travel- and non-travel-associated categories. Eighty-one (79.4%) of the Shigella isolates acquired during international travel were resistant to multiple antibiotics compared to 53 (52.1%) of the infections transmitted in domestic settings. A majority (79.4%) of isolates associated with international travel demonstrated resistance to aminoglycosides and tetracyclines, whereas 47 (46.1%) of the infections acquired domestically were resistant to tetracycline. Almost all isolates (92.2%) transmitted in domestic settings were resistant to aminoglycosides, and 5 isolates from adult male patients were resistant to azithromycin, a drug often used for empiric treatment of severe shigellosis. Twenty (19.6%) isolates associated with illnesses acquired during overseas travel in 4 countries were resistant to quinolones. One S. sonnei PFGE pattern was traced to a multidrug-resistant isolate acquired overseas that had caused a multistate outbreak of shigellosis, suggesting global dissemination of a drug-resistant species. Resistance to certain drugs-for example, tetracycline-increased in both overseas- and domestic-acquired infections during the study period. The prevalence of resistance to macrolides (azithromycin) and third-generation cephalosporins (ceftriaxone) was less than 1%; however, efforts to better monitor changes in drug resistance over time combined with increased antimicrobial stewardship are essential at the local, national, and global levels.

Preschoolers Acquire General Knowledge by Sharing in Pretense

Science.gov (United States)

Sutherland, Shelbie L.; Friedman, Ori

2012-01-01

Children acquire general knowledge about many kinds of things, but there are few known means by which this knowledge is acquired. In this article, it is proposed that children acquire generic knowledge by sharing in pretend play. In Experiment 1, twenty-two 3- to 4-year-olds watched pretense in which a puppet represented a "nerp" (an unfamiliar…

Retention of laparoscopic procedural skills acquired on a virtual-reality surgical trainer

DEFF Research Database (Denmark)

Nielsen, Mathilde Maagaard; Sørensen, J L; Oestergaard, Jeanett

2011-01-01

BACKGROUND: Virtual-reality (VR) simulator training has been shown to improve surgical performance in laparoscopic procedures in the operating room. We have, in a randomised controlled trial, demonstrated transferability to real operations. The validity of the LapSim virtual-reality simulator...... as an assessment tool has been demonstrated in several reports. However, an unanswered question regarding simulator training is the durability, or retention, of skills acquired during simulator training. The aim of the present study is to assess the retention of skills acquired using the LapSim VR simulator, 6...... and 18 months after an initial training course. METHODS AND MATERIALS: The investigation was designed as a 6- and 18-month follow-up on a cohort of participants who earlier participated in a skills training programme on the LapSim VR. The follow-up cohort consisted of trainees and senior consultants...

Undiagnosed diabetes mellitus in community-acquired pneumonia

DEFF Research Database (Denmark)

Jensen, Andreas Vestergaard; Faurholt-Jepsen, Daniel; Egelund, Gertrud Baunbæk

2017-01-01

Background: Diabetes mellitus is an important risk factor for community-acquired pneumonia, whereas the prevalence of undiagnosed diabetes mellitus and prediabetes in patients with community-acquired pneumonia is largely unknown. We aimed to determine the prevalence of prediabetes, undiagnosed......-acquired pneumonia included in the German Community-Acquired Pneumonia Competence Network (CAPNETZ) study between 2007 and 2014. The prevalence of undiagnosed diabetes mellitus and prediabetes was estimated based on hemoglobin A1c measurements. Logistic regression was used to assess risk factors for undiagnosed...... diabetes mellitus. Results: Fifteen percent of patients had known diabetes mellitus. Among patients without known diabetes mellitus, 5.0% had undiagnosed diabetes mellitus and 37.5% had prediabetes. Male sex (odds ratio [OR], 2.45 [95% confidence interval {CI}, 1.35-4.45]), body mass index ≥25 kg/m2 (OR, 2...

Shorter leukocyte telomere length is associated with higher risk of infections

DEFF Research Database (Denmark)

Helby, Jens; Nordestgaard, Børge G; Benfield, Thomas

2017-01-01

In the general population, older age is associated with short leukocyte telomere length and with high risk of infections. In a recent study of allogeneic hematopoietic cell transplantation for severe aplastic anemia, long donor leukocyte telomere length was associated with improved survival...

Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer

Directory of Open Access Journals (Sweden)

Chirag Sheth

2016-09-01

Full Text Available Acquired factor VIII deficiency (acquired hemophilia A is a rare condition characterized by the acquisition of autoantibodies that affect the clotting activity of factor VIII (fVIII. The most common manifestation in affected patients is a hemorrhagic diathesis. This disorder is associated with autoimmune diseases, pregnancy, postpartum period, drugs, and malignancy. Management of this condition begins with attempts to arrest an acute bleed based on the site and severity of bleeding and inhibitor titer. The next priority is eradication of the fVIII antibodies using immunosuppressive therapies. We report the case of a 66-year-old male who presented with spontaneous right thigh hematoma with prolonged activated partial prothrombin time and normal prothrombin time. Mixing studies confirmed the presence of an inhibitor. Further investigation for the underlying etiology of acquired hemophilia A leads to diagnosis of prostate cancer. Treatment consisted of bypassing agents including activated factor VII and activated prothrombin plasma concentrate to arrest the bleeding. Steroids and cyclophosphamide were added to suppress the fVIII inhibitors. Concomitant treatment of locally advanced prostate cancer with chemotherapy confirmed the eradication of the inhibitors. To our knowledge, this is the first reported case of prostate cancer diagnosed and treated simultaneously with acquired hemophilia A resulting in favorable patient outcome.

Precision grip in congenital and acquired hemiparesis: similarities in impairments and implications for neurorehabilitation - review

Directory of Open Access Journals (Sweden)

Yannick eBleyenheuft

2014-06-01

Full Text Available Background: Patients with congenital and acquired hemiparesis incur long-term functional deficits, among which the loss of prehension that may impact their functional independence. Identifying, understanding and comparing the underlying mechanisms of prehension impairments represent an opportunity to better adapt neurorehabilitationObjective: The present review aims to provide a better understanding of precision grip deficits in congenital and acquired hemiparesis and to determine whether the severity and type of fine motor control impairments depend on whether or not the lesions are congenital or acquired in adulthood. Methods: Using combinations of the following key words: fingertip force, grip force, precision grip, cerebral palsy, stroke, pubmed and Scopus databases were used to search studies from 1984 to 2013. Results: Individuals with both congenital and acquired hemiparesis were able to some extent to use anticipatory motor control in precision grip tasks, even if this control was impaired in the paretic hand. In both congenital or acquired hemiparesis, the ability to plan efficient anticipatory motor control when the less-affected hand is used provides a possibility to remediate impairments in anticipatory motor control of the paretic hand. Conclusion: Surprisingly we observed very few differences between the results of studies in children with congenital hemiplegia and stroke patients. We suggest that the underlying specific strategies of neurorehabilitation developed for each one could benefit the other.

The changing face of community-acquired methicillin-resistant Staphylococcus aureus

Directory of Open Access Journals (Sweden)

P Kale

2016-01-01

Full Text Available Methicillin-resistant Staphylococcus aureus (MRSA is an important cause of infection, both in hospitalised patients with significant healthcare exposure and in patients without healthcare risk factors. Community-acquired methicillin-resistant S. aureus (CA-MRSA are known for their rapid community transmission and propensity to cause aggressive skin and soft tissue infections and community-acquired pneumonia. The distinction between the healthcare-associated (HA-MRSA and CA-MRSA is gradually fading owing to the acquisition of multiple virulence factors and genetic elements. The movement of CA-MRSA strains into the nosocomial setting limits the utility of using clinical risk factors alone to designate community or HA status. Identification of unique genetic characteristics and genotyping are valuable tools for MRSA epidemiological studies. Although the optimum pharmacotherapy for CA-MRSA infections has not been determined, many CA-MRSA strains remain broadly susceptible to several non-β-lactam antibacterial agents. This review aimed at illuminating the characteristic features of CA-MRSA, virulence factors, changing clinical settings and molecular epidemiology, insurgence into the hospital settings and therapy with drug resistance.

Felbamate in an adult population with severe refractory epilepsy.

LENUS (Irish Health Repository)

Kearney, H

2011-04-05

Felbamate (FBM) is efficacious in treating patients with refractory epilepsy but was withdrawn due to cases of aplastic anaemia, hepatic failure and five reported deaths. FBM is currently used in specialist centres and is only being used in one Irish centre to date. This papers aim is to review the efficacy and safety experience of FBM in Irish adult patients with refractory epilepsy. A retrospective chart review was done on patients\\' medical records. Patients were subdivided into responders and non responders based on change in seizure frequency and side effects were recorded for all. Of the 13 patients on FBM nine patients responded to FBM, four patients did not. FBM is a safe and efficacious alternative in an Irish adult population with refractory epilepsy. However close monitoring is still required given the potential fatal side effects that are possible with this anticonvulsant.

Alteration of Hepatic Gene Expression along with the Inherited Phenotype of Acquired Fatty Liver in Chicken

Science.gov (United States)

Zhang, Yonghong; Liu, Zhen; Liu, Ranran; Wang, Jie; Zheng, Maiqing; Li, Qinghe; Cui, Huanxian; Zhao, Guiping; Wen, Jie

2018-01-01

Fatty liver is a widespread disease in chickens that causes a decrease in egg production and even death. The characteristics of the inherited phenotype of acquired fatty liver and the molecular mechanisms underlying it, however, are largely unknown. In the current study, fatty liver was induced in 3 breeds by a high-fat (HF) diet and a methionine choline-deficient (MCD) diet. The results showed that the dwarf Jingxing-Huang (JXH) chicken was more susceptible to fatty liver compared with the layer White Leghorns (WL) and local Beijing-You (BJY) breeds. In addition, it was found that the paternal fatty livers induced by HF diet in JXH chickens were inherited. Compared to birds without fatty liver in the control group, both offsprings and their sires with fatty livers in the paternal group exhibited altered hepatic gene expression profiles, including upregulation of several key genes involved in fatty acid metabolism, lipid metabolism and glucose metabolism (ACACA, FASN, SCD, ACSL5, FADS2, FABP1, APOA4 and ME1). This study uniquely revealed that acquired fatty liver in cocks can be inherited. The hepatic gene expression profiles were altered in chickens with the inherited phenotype of acquired paternal fatty liver and several genes could be candidate biomarkers. PMID:29642504

Alteration of Hepatic Gene Expression along with the Inherited Phenotype of Acquired Fatty Liver in Chicken.

Science.gov (United States)

Zhang, Yonghong; Liu, Zhen; Liu, Ranran; Wang, Jie; Zheng, Maiqing; Li, Qinghe; Cui, Huanxian; Zhao, Guiping; Wen, Jie

2018-04-08

Fatty liver is a widespread disease in chickens that causes a decrease in egg production and even death. The characteristics of the inherited phenotype of acquired fatty liver and the molecular mechanisms underlying it, however, are largely unknown. In the current study, fatty liver was induced in 3 breeds by a high-fat (HF) diet and a methionine choline-deficient (MCD) diet. The results showed that the dwarf Jingxing-Huang (JXH) chicken was more susceptible to fatty liver compared with the layer White Leghorns (WL) and local Beijing-You (BJY) breeds. In addition, it was found that the paternal fatty livers induced by HF diet in JXH chickens were inherited. Compared to birds without fatty liver in the control group, both offsprings and their sires with fatty livers in the paternal group exhibited altered hepatic gene expression profiles, including upregulation of several key genes involved in fatty acid metabolism, lipid metabolism and glucose metabolism ( ACACA , FASN , SCD , ACSL5 , FADS2 , FABP1 , APOA4 and ME1 ). This study uniquely revealed that acquired fatty liver in cocks can be inherited. The hepatic gene expression profiles were altered in chickens with the inherited phenotype of acquired paternal fatty liver and several genes could be candidate biomarkers.

Alteration of Hepatic Gene Expression along with the Inherited Phenotype of Acquired Fatty Liver in Chicken

Directory of Open Access Journals (Sweden)

Yonghong Zhang

2018-04-01

Full Text Available Fatty liver is a widespread disease in chickens that causes a decrease in egg production and even death. The characteristics of the inherited phenotype of acquired fatty liver and the molecular mechanisms underlying it, however, are largely unknown. In the current study, fatty liver was induced in 3 breeds by a high-fat (HF diet and a methionine choline-deficient (MCD diet. The results showed that the dwarf Jingxing-Huang (JXH chicken was more susceptible to fatty liver compared with the layer White Leghorns (WL and local Beijing-You (BJY breeds. In addition, it was found that the paternal fatty livers induced by HF diet in JXH chickens were inherited. Compared to birds without fatty liver in the control group, both offsprings and their sires with fatty livers in the paternal group exhibited altered hepatic gene expression profiles, including upregulation of several key genes involved in fatty acid metabolism, lipid metabolism and glucose metabolism (ACACA, FASN, SCD, ACSL5, FADS2, FABP1, APOA4 and ME1. This study uniquely revealed that acquired fatty liver in cocks can be inherited. The hepatic gene expression profiles were altered in chickens with the inherited phenotype of acquired paternal fatty liver and several genes could be candidate biomarkers.

An Update on the Management Of Hospital-Acquired Pneumonia in the Elderly

Directory of Open Access Journals (Sweden)

Chao-Hsien Lee

2008-12-01

Full Text Available Pneumonia is the leading cause of infection-related death and represents the fifth cause of mortality in the elderly. There are several reported risk factors for acquiring pneumonia at an older age, such as alcoholism, lung and heart diseases, nursing home residence, and swallowing disorders. Hospital-acquired pneumonia (HAP is reviewed, with an emphasis on multidrug-resistant (MDR bacterial pathogens, such as Pseudomonas aeruginosa, Acinetobacter species, and methicillin-resistant Staphylococcus aureus. The clinical characteristics of pneumonia in the elderly differ substantially compared with younger patients, and the severity of the disease is strongly associated with increased age and age-related comorbid disorders. Streptococcus pneumoniae is the pathogen most frequently responsible for pneumonia in the elderly with early HAP without risk factors for MDR; enteric Gram-negative rods should be considered in nursing home-associated pneumonia, as well as anaerobes in patients with aspiration pneumonia. Special attention should be given to preventive measures such as vaccination, oral care, and nutrition. The management of HAP should be instituted early with: appropriate use of antibiotics in adequate doses; avoidance of excessive use of antibiotics by de-escalation of initial antibiotic therapy, based on microbiologic cultures and the clinical response of the patient; and reduction of the duration of treatment to the minimum effective period.

Community acquired urinary tract infection: etiology and bacterial susceptibility

Directory of Open Access Journals (Sweden)

Dias Neto José Anastácio

2003-01-01

Full Text Available PURPOSE: Urinary tract infections (UTI are one of the most common infectious diseases diagnosed. UTI account for a large proportion of antibacterial drug consumption and have large socio-economic impacts. Since the majority of the treatments begins or is done completely empirically, the knowledge of the organisms, their epidemiological characteristics and their antibacterial susceptibility that may vary with time is mandatory. OBJECTIVE: The aim of this study was to report the prevalence of uropathogens and their antibiotic susceptibility of the community acquired UTI diagnosed in our institution and to provide a national data. METHODS: We analyzed retrospectively the results of urine cultures of 402 patients that had community acquired urinary tract infection in the year of 2003. RESULTS: The mean age of the patients in this study was 45.34 ± 23.56 (SD years. There were 242 (60.2% females and 160 (39.8% males. The most commonly isolated organism was Escherichia coli (58%. Klebsiella sp. (8.4% and Enterococcus sp.(7.9% were reported as the next most common organisms. Of all bacteria isolated from community acquired UTI, only 37% were sensitive to ampicillin, 51% to cefalothin and 52% to trimethoprim/sulfamethoxazole. The highest levels of susceptibility were to imipenem (96%, ceftriaxone (90%, amikacin (90%, gentamicin (88%, levofloxacin (86%, ciprofloxacin (73%, nitrofurantoin (77% and norfloxacin (75%. CONCLUSION: Gram-negative agents are the most common cause of UTI. Fluoroquinolones remains the choice among the orally administered antibiotics, followed by nitrofurantoin, second and third generation cephalosporins. For severe disease that require parenteral antibiotics the choice should be aminoglycosides, third generation cephalosporins, fluoroquinolones or imipenem, which were the most effective.

Determining the quality of life in persons with acquired brain injury

OpenAIRE

Balanč, Mateja

2017-01-01

Identifying and assessing the quality of life of persons with acquired brain injury is an important element of rehabilitation, and enables assistance and support to both the injured and their families. By identifying and assessing quality of life, we can better understand the impact of injuries on the individual and his/her life. Different levels of injuries can severely affect cognitive, physical, emotional-behavioral and social life not only of a injured person but also of members of his f...

Total body irradiation in bone marrow transplantation

International Nuclear Information System (INIS)

Gluckman, E.; Devergie, A.; Boiron, M.; Bernard, Jean; Dutreix, A.; Dutreix, J.

1979-01-01

Total body irradiation was used in 22 patients as part of their conditioning regimen for bone marrow transplantation. Nine patients with acute leukemia received 1000 cGy TBI in addition with chemotherapy. None of them survived and the main cause of death was interstitial pneumonitis (50%). 4 patients received 1000 cGy with a lung shielding of 500 cGy. Two patients with acute leukemia died of leukemia and sepsis, two patients had aplastic anemia, one is surviving, the other died of severe GVHD and infectious complications. Nine patients with severe aplastic anemia strongly immunized by previous blood transfusions received 800 cGy TBI with a lung shielding of 400 cGy. No rejection was observed and 7 patients (63%) are currently alive. One patient died of interstitial pneumonitis probably related to CMV infection, one of subacute necrotizing hepatitis, two of severe acute GVHD. It is concluded from this study that TBI remains the best immunosuppressive conditioning regimen even in strongly immunized patients. It may be a contributing factor of the incidence and severity of interstitial pneumonitis. A reduction of the dose of the lung to 400-500 cGy seems to decrease the severity of this complication

Myelofibrosis and acquired hemophilia A: a case report.

Science.gov (United States)

Wrobel, Marie; Comio, Emilie; Gay, Valerie; Baroudi, Noureddine; Meyer, Pascal; Chuniaud-Louche, Christine; Hacini, Maya; Pica, Gian Matteo

2016-05-07

Myelofibrosis and acquired hemophilia A is a rare association. To the best of our knowledge only one case of myelofibrosis and acquired hemophilia A has been previously described. A 66-year-old Caucasian man diagnosed with myelofibrosis evolving in acute myeloid leukemia was referred to us for postoperative bleeding. Hemostatic studies showed prolonged activated partial thromboplastin time, decreased factor VIII coagulation, and a high factor VIII inhibitor titer; these findings led to a diagnosis of acquired hemophilia A for which he was treated with methylprednisolone and recombinant activated factor VII on admission. Due to a lack of response he was subsequently treated with rituximab combined with activated prothrombin complex concentrates. Furthermore, he received azacytidine to treat the underlying hematological malignancies. Immunosuppressive rituximab therapy resolved acquired hemophilia A with marked efficacy. Rapid and accurate diagnosis, effective hemostatic therapy, and timely treatment for underlying disease are important in the management of acquired hemophilia A secondary to hematological malignancy.

14 CFR 1274.402 - Contractor acquired property.

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2010-01-01

... 14 Aeronautics and Space 5 2010-01-01 2010-01-01 false Contractor acquired property. 1274.402 Section 1274.402 Aeronautics and Space NATIONAL AERONAUTICS AND SPACE ADMINISTRATION COOPERATIVE AGREEMENTS WITH COMMERCIAL FIRMS Property § 1274.402 Contractor acquired property. As provided in § 1274.923...

Missing cells: pathophysiology, diagnosis and management of (pancytopenia in childhood

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Miriam eErlacher

2015-07-01

Full Text Available Peripheral blood cytopenia in children can be due to a variety of acquired or inherited diseases. Genetic disorders affecting a single hematopoietic lineage are frequently characterized by typical bone marrow findings such as lack of progenitors or maturation arrest in congenital neutropenia or a lack of megakaryocytes in congenital amegakaryocytic thrombocytopenia whereas antibody mediated diseases such as autoimmune neutropenia are associated with a rather unremarkable bone marrow morphology. In contrast, pancytopenia is frequently associated with a hypocellular bone marrow and the differential diagnosis includes acquired aplastic anemia, myelodysplastic syndrome, inherited bone marrow failure syndromes such as Fanconi anemia and dyskeratosis congenita and a variety of immunological disorders including hemophagocytic lymphohistiocytosis. Thorough bone marrow analysis is of special importance for the diagnostic work-up of most patients. Cellularity, cellular composition and dysplastic signs are the cornerstones of the differential diagnosis. Pancytopenia in the presence of a normo- or hypercellular marrow with dysplastic changes may indicate myelodysplastic syndrome. More challenging for the hematologist is the evaluation of the hypocellular bone marrow. Although aplastic anemia and hypocellular refractory cytopenia of childhood (RCC can reliably be differentiated on a morphological level the overlapping pathophysiology remains a significant challenge for the choice of the therapeutic strategy. Furthermore, inherited bone marrow failure syndromes are usually associated with the morphological picture of RCC and the recognition of these entities is essential as they often present a multisystem disease requiring different diagnostic and therapeutic approaches. This paper gives an overview over the different disease entities presenting with (pancytopenia, their pathophysiology, characteristic bone marrow findings and therapeutic approaches.

A Case of Severe Neutropenia From Short-Term Exposure to Moxifloxacin

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Weihan Chen BS

2017-03-01

Full Text Available Moxifloxacin is commonly prescribed in the inpatient and outpatient management of community-acquired pneumonia and other common infections. We report a case of a 76-year-old man who developed severe neutropenia after several days of treatment for community-acquired pneumonia. The patient had a history of alcohol abuse; however, there were no other offending medications prescribed, and a thorough laboratory workup for other possible causes of neutropenia was negative. The patient’s neutrophils and white blood count responded quickly to cessation of fluoroquinolones. This case highlights the importance of identifying patients that might be at high risk for neutropenia that may need closer monitoring on this commonly prescribed medication.

Inherited and acquired immunodeficiencies underlying tuberculosis in childhood

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Boisson-Dupuis, Stéphanie; Bustamante, Jacinta; El-Baghdadi, Jamila; Camcioglu, Yildiz; Parvaneh, Nima; Azbaoui, Safaa El; Agader, Aomar; Hassani, Amal; Hafidi, Naima El; Mrani, Nidal Alaoui; Jouhadi, Zineb; Ailal, Fatima; Najib, Jilali; Reisli, Ismail; Zamani, Adil; Yosunkaya, Sebnem; Gulle-Girit, Saniye; Yildiran, Alisan; Cipe, Funda Erol; Torun, Selda Hancerli; Metin, Ayse; Atikan, Basak Yildiz; Hatipoglu, Nevin; Aydogmus, Cigdem; Kilic, Sara Sebnem; Dogu, Figen; Karaca, Neslihan; Aksu, Guzide; Kutukculer, Necil; Keser-Emiroglu, Melike; Somer, Ayper; Tanir, Gonul; Aytekin, Caner; Adimi, Parisa; Mahdaviani, Seyed Alireza; Mamishi, Setareh; Bousfiha, Aziz; Sanal, Ozden; Mansouri, Davood; Casanova, Jean-Laurent; Abel, Laurent

2015-01-01

Summary Tuberculosis (TB), caused by Mycobacterium tuberculosis (M.tb) and a few related mycobacteria, is a devastating disease, killing more than a million individuals per year worldwide. However, its pathogenesis remains largely elusive, as only a small proportion of infected individuals develop clinical disease either during primary infection or during reactivation from latency or secondary infection. Subacute, hematogenous, and extrapulmonary disease tends to be more frequent in infants, children, and teenagers than in adults. Life-threatening primary TB of childhood can result from known acquired or inherited immunodeficiencies, although the vast majority of cases remain unexplained. We review here the conditions conferring a predisposition to childhood clinical diseases caused by mycobacteria, including not only M.tb but also weakly virulent mycobacteria, such as BCG vaccines and environmental mycobacteria. Infections with weakly virulent mycobacteria are much rarer than TB, but the inherited and acquired immunodeficiencies underlying these infections are much better known. Their study has also provided genetic and immunological insights into childhood TB, as illustrated by the discovery of single-gene inborn errors of IFN-γ immunity underlying severe cases of TB. Novel findings are expected from ongoing and future human genetic studies of childhood TB in countries that combine a high proportion of consanguineous marriages, a high incidence of TB, and an excellent clinical care, such as Iran, Morocco, and Turkey. PMID:25703555

How are Concepts of Infinity Acquired?

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Trzęsicki Kazimierz

2015-03-01

Full Text Available Concepts of infinity have been subjects of dispute since antiquity. The main problems of this paper are: is the mind able to acquire a concept of infinity? and: how are concepts of infinity acquired? The aim of this paper is neither to say what the meanings of the word “infinity” are nor what infinity is and whether it exists. However, those questions will be mentioned, but only in necessary extent.

Acquired hypofibrinogenemia: current perspectives

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Besser MW

2016-09-01

Full Text Available Martin W Besser,1 Stephen G MacDonald2 1Department of Haematology, 2Department of Specialist Haemostasis, The Pathology Partnership, Addenbrooke’s Hospital, Cambridge, UK Abstract: Acquired hypofibrinogenemia is most frequently caused by hemodilution and consumption of clotting factors. The aggressive replacement of fibrinogen has become one of the core principles of modern management of massive hemorrhage. The best method for determining the patient’s fibrinogen level remains controversial, and particularly in acquired dysfibrinogenemia, could have major therapeutic implications depending on which quantification method is chosen. This review introduces the available laboratory and point-of-care methods and discusses the relative advantages and limitations. It also discusses current strategies for the correction of hypofibrinogenemia. Keywords: Clauss fibrinogen assay, fibrinogen antigen, viscoelastic testing, ­gravimetric fibrinogen assay, PT-derived fibrinogen, functional fibrinogen, direct oral anticoagulant, dysfibrinogenemia, afibrinogenemia

Pneumonia - children - community acquired

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Bronchopneumonia - children; Community-acquired pneumonia - children; CAP - children ... Viruses are the most common cause of pneumonia in infants and children. Ways your child can get CAP include: Bacteria and viruses living in the nose, sinuses, or mouth may spread ...

Sequential changes in bone marrow architecture during continuous low dose gamma irradiation

International Nuclear Information System (INIS)

Seed, T.M.; Chubb, G.T.; Tolle, D.V.

1981-01-01

Beagles continuously exposed to low daily doses (10 R) of whole-body 60Co gamma-radiation are prone to develop either early occurring aplastic anemia or late occurring myeloproliferative disorders (Seed et al., 1977). In this study, we have examined by a combination of light microscopy and scanning and transmission electron microscopy the sequential changes in the morphology of biopsied rib bone marrow of continuously irradiated dogs that developed either aplastic anemia, myelofibrosis, or myelogenous leukemia. Characteristic modification of key elements of marrow architecture have been observed during preclinical and clinical phases of these hemopathological conditions. The more prominent of these changes include the following. (i) In developing aplastic anemia: severe vascular sinus and parenchymal cord compression, and focally degenerate endosteal surfaces. (ii) In developing myelofibrosis: hyperplasia of endosteal and reticular stomal elements. (iii) In developing leukemia: hypertrophy of reticular and endothelial elements in the initial restructuring of the stromal matrix and the subsequent aberrant hemopoietic repopulation of the initially depleted stromal matrix. These architectural changes during preclinical phases appear to be related to the pathological progression to each of the radiation-induced hemopathological end points

Monitoring Agitated Behavior After acquired Brain Injury

DEFF Research Database (Denmark)

Aadal, Lena; Mortensen, Jesper; Nielsen, Jørgen Feldbaek

2016-01-01

Purpose: To describe the onset, duration, intensity, and nursing shift variation of agitated behavior in patients with acquired brain injury (ABI) at a rehabilitation hospital. Design: Prospective descriptive study. Methods: A total of 11 patients with agitated behavior were included. Agitated...... behavior was registered with the Agitated Behavior Scale (ABS). The nurse or therapist allocated the individual patient assessed ABS during each shift. Intensity of agitated behavior was tested using exact test. A within-subject shift effect was analyzed with repeated-measure ANOVA. Findings: The onset...... of agitated behavior was at a median of 14 (1–28) days from admission. Seven patients remained agitated beyond 3 weeks from onset. Severe intensity of agitation was observed in 86 of 453 nursing shifts. Differences in agitated behavior between day, evening, and night shifts were found, F(2.20) = 7.90, p...

Connectionist neuropsychology: uncovering ultimate causes of acquired dyslexia.

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Woollams, Anna M

2014-01-01

Acquired dyslexia offers a unique window on to the nature of the cognitive and neural architecture supporting skilled reading. This paper provides an integrative overview of recent empirical and computational work on acquired dyslexia within the context of the primary systems framework as implemented in connectionist neuropsychological models. This view proposes that damage to general visual, phonological or semantic processing abilities are the root causes of different forms of acquired dyslexia. Recent case-series behavioural evidence concerning pure alexia, phonological dyslexia and surface dyslexia that supports this perspective is presented. Lesion simulations of these findings within connectionist models of reading demonstrate the viability of this approach. The commitment of such models to learnt representations allows them to capture key aspects of performance in each type of acquired dyslexia, particularly the associated non-reading deficits, the role of relearning and the influence of individual differences in the premorbid state of the reading system. Identification of these factors not only advances our understanding of acquired dyslexia and the mechanisms of normal reading but they are also relevant to the complex interactions underpinning developmental reading disorders.

Emergence of community-acquired Clostridium difficile infection: the experience of a French hospital and review of the literature

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Maja Ogielska

2015-08-01

Conclusions: CDI can cause community-acquired diarrhoea, and CA-CDI may be more severe than HCA-CDI. Prospective studies of CDI involving people from the general community without risk factors are required to confirm this observation.

Vascular and perivascular niches, but not the osteoblastic niche, are numerically restored following allogeneic hematopoietic stem cell transplantation in patients with aplastic anemia.

Science.gov (United States)

Wu, Liangliang; Mo, Wenjian; Zhang, Yuping; Zhou, Ming; Li, Yumiao; Zhou, Ruiqing; Xu, Shiling; Pan, Shiyi; Deng, Hui; Mao, Ping; Wang, Shunqing

2017-07-01

Bone marrow (BM) niches, including the osteoblastic, vascular, and perivascular niches, are numerically impaired in patients with aplastic anemia (AA). It remains unclear whether these niches are numerically restored in AA patients after allogenic hematopoietic stem cell transplantation (allo-HSCT). To investigate changes in BM niches, we monitored 52 patients with AA who had undergone allo-HSCT and performed immunohistochemical studies of BM niches using antibodies against CD34, CD146, and osteopontin. After allo-HSCT, patients with AA exhibited a remarkable increase in the number of cellular elements in the BM niches, including the vascular and perivascular cells. However, no significant differences in endosteal cells were detected. We explored the cause of this restoration by analyzing the origin of BM mesenchymal stem cells (BM-MSCs) and the expression of cytokines in BM plasma. STR-PCR revealed that the BM-MSCs were derived from the host, not the donor. In addition, significantly elevated levels of vascular endothelial growth factor (VEGF) were found after allo-HSCT. Our data indicates that vascular and perivascular niches are numerically restored, but the endosteal niche remains numerically impaired in patients with AA after allo-HSCT, and that levels of VEGF, but not donor-derived BM-MSCs, may correlate with the restoration of BM niches.

19 CFR 148.33 - Articles acquired abroad.

Science.gov (United States)

2010-04-01

... 19 Customs Duties 2 2010-04-01 2010-04-01 false Articles acquired abroad. 148.33 Section 148.33... Articles acquired abroad. (a) Exemption. Each returning resident is entitled to bring in free of duty and..., Harmonized Tariff Schedule of the United States (19 U.S.C. 1202), articles for his personal or household use...

26 CFR 1.9002-6 - Acquiring corporation.

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2010-04-01

... 26 Internal Revenue 13 2010-04-01 2010-04-01 false Acquiring corporation. 1.9002-6 Section 1.9002... (CONTINUED) INCOME TAXES General Actuarial Valuations § 1.9002-6 Acquiring corporation. Section 5(d) of the... corporation by another corporation in a distribution or transfer described in section 381(a) of the Code the...

Acquired hypertrichosis lanuginosa

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Kumar Pramod

1993-01-01

Full Text Available Acquired hypertirichosis lanuginose developed rapidly in a patient with no detectable malignancy. Soft, fine, downy hair growth was noticed on the face, ears, limbs and trunk. Bilaterally symmetrical vitiliginous macules were present on the ear and preauricular region. This case is reported because of its rarity, absence of any detectable malignancy and development of vitiligo, which to our knowledge has not been reported earlier.

Application of Component Transfusion in the Treatment of Childhood Leukemia and Aplastic Anemia%成份输血在儿童白血病和再障治疗中的应用

Institute of Scientific and Technical Information of China (English)

张咏芳

2016-01-01

Objective To study the composition blood transfusion in the treatment of childhood leukemia and aplastic ane-mia application effect. Methods Convenient selection retrospective analysis in June 2010 - June 2015 in our hospital com-ponent blood transfusion treatment of leukemia and aplastic anemia children 28 cases, 24 cases respectively, contrast com-position blood transfusion before and after treatment the patient's clinical manifestations, inspection index, etc. Results Af-ter treatment in children with anemia, corrected and improved mental state; Bleeding, shortness of breath, weakness, pale face, such as infection symptoms were significantly improved; Children with appetite also increase, the illness tends to be stable;Children with hemoglobin, white blood cells, neutrophils, platelet count (45.62±11.5)g/L before treatment respective-ly, (10.53±0.26)×109/L, (0.22±0.19)×109/L, (13.6±6.3)×109/L increased after the treatment (76.2 ± 10.3)g/L, (2.61±0.53)×109/L, (0.75±0.24)×109/L, (75.4±9.6)×109/L, P﹤ 0.05, with statistical significance. Conclusion The ingredients of blood has high concentration and purity, which can effectively improve the clinical curative effect of childhood leukemia and aplastic anemia, reduce risk.%目的：探讨成份输血在儿童白血病和再生障碍性贫血治疗中的应用效果。方法方便选取并回顾性分析2010年6月—2015年6月在该院接受成份输血治疗的白血病、再生障碍性贫血儿童分别为28例、24例，对比成份输血治疗前后患儿的临床表现、检验指标等。结果治疗后患儿贫血得以纠正，精神状态好转；出血、气促、虚弱无力、面色苍白、感染等症状均明显改善；患儿食欲也增加，病情趋于稳定；患儿血红蛋白、白细胞、中性粒细胞、血小板计数分别治疗前的(45.62±11.5)g/L、（10.53±0.26)×109/L、（0.22±0.19)×109/L、（13.6±6.3)×109/L升高到治疗后的（76.2±10.3)g/L、（2.61±0

Effectiveness of a Very Early Stepping Verticalization Protocol in Severe Acquired Brain Injured Patients: A Randomized Pilot Study in ICU.

Science.gov (United States)

Frazzitta, Giuseppe; Zivi, Ilaria; Valsecchi, Roberto; Bonini, Sara; Maffia, Sara; Molatore, Katia; Sebastianelli, Luca; Zarucchi, Alessio; Matteri, Diana; Ercoli, Giuseppe; Maestri, Roberto; Saltuari, Leopold

2016-01-01

Verticalization was reported to improve the level of arousal and awareness in patients with severe acquired brain injury (ABI) and to be safe in ICU. We evaluated the effectiveness of a very early stepping verticalization protocol on their functional and neurological outcome. Consecutive patients with Vegetative State or Minimally Conscious State were enrolled in ICU on the third day after an ABI. They were randomized to undergo conventional physiotherapy alone or associated to fifteen 30-minute sessions of verticalization, using a tilt table with robotic stepping device. Once stabilized, patients were transferred to our Neurorehabilitation unit for an individualized treatment. Outcome measures (Glasgow Coma Scale, Coma Recovery Scale revised -CRSr-, Disability Rating Scale-DRS- and Levels of Cognitive Functioning) were assessed on the third day from the injury (T0), at ICU discharge (T1) and at Rehab discharge (T2). Between- and within-group comparisons were performed by the Mann-Whitney U test and Wilcoxon signed-rank test, respectively. Of the 40 patients enrolled, 31 completed the study without adverse events (15 in the verticalization group and 16 in the conventional physiotherapy). Early verticalization started 12.4±7.3 (mean±SD) days after ABI. The length of stay in ICU was longer for the verticalization group (38.8 ± 15.7 vs 25.1 ± 11.2 days, p = 0.01), while the total length of stay (ICU+Neurorehabilitation) was not significantly different (153.2 ± 59.6 vs 134.0 ± 61.0 days, p = 0.41). All outcome measures significantly improved in both groups after the overall period (T2 vs T0, pverticalization protocol, started since the acute stages, improves the short-term and long-term functional and neurological outcome of ABI patients. clinicaltrials.gov NCT02828371.

Bone marrow transplantation for girls with aplastic anemia utilizing modified field of total lymphoid irradiation and cyclophosphamide; With emphasis on the field of pelvic cavity

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Hanada, Ryoji; Kawakami, Tetsuo; Akuta, Naoko; Moriwaki, Kohichi; Kato, Shizue; Inaba, Toshiya; Hayashi, Yasuhide; Yamamoto, Keiko (Saitama Children' s Medical Center, Saitama (Japan))

1990-12-01

A preparative regimen for allogeneic bone marrow transplantation, consisting of total lymphoid irradiation (TLI) with 750 cGy and cyclophosphamide (CY), was used in five girls with aplastic anemia. All patients received bone marrow from HLA matched/mixed lymphocyte culture negative siblings. In our regimen the 'inverted Y' field to irradiate the pelvic nodes was modified, which did not include the whole pelvic cavity in an attempt to protect the ovaries from irradiation. Although some of the pelvic nodes was supported not to be irradiated in order to protect the ovaries, engraftment occurred in all five patients including four who had been transfused prior to transplantation. All five are alive from 47 days to 1378 days (median 285 days) after transplantation without tranplantation-associated complications. The calculated dose to the ovaries was sixteen percent of the entire dose of the regimen. Both of the two evaluable patients that had received tranplantation just before or during the puberty are developing normal sex maturity including menstruation. This study suggests that our preparative regimen is effective not only for engraftment of the donor marrow but also for protecting the ovaries from irradiation. (author).

Toxic shock syndrome due to community-acquired methicillin-resistant Staphylococcus aureus infection: Two case reports and a literature review in Japan.

Science.gov (United States)

Sada, Ryuichi; Fukuda, Saori; Ishimaru, Hiroyasu

2017-01-01

Community-acquired methicillin-resistant Staphylococcus aureus has been spreading worldwide, including in Japan. However, few cases of toxic shock syndrome caused by Community-acquired methicillin-resistant Staphylococcus aureus have been reported in Japan. We report 2 cases, in middle-aged women, of toxic shock syndrome due to Community-acquired methicillin-resistant Staphylococcus aureus via a vaginal portal of entry. The first patient had used a tampon and the second patient had vaginitis due to a cleft narrowing associated with vulvar lichen sclerosus. Both patients were admitted to our hospital with septic shock and severe acute kidney injury and subsequently recovered with appropriate antibiotic treatment. In our review of the literature, 8 cases of toxic shock syndrome caused by Community-acquired methicillin-resistant Staphylococcus aureus were reported in Japan. In these 8 cases, the main portals of entry were the skin and respiratory tract; however, the portal of entry of Community-acquired methicillin-resistant Staphylococcus aureus from a vaginal lesion has not been reported in Japan previously.

From Bad to Worse: Anemia on Admission and Hospital-Acquired Anemia.

Science.gov (United States)

Koch, Colleen G; Li, Liang; Sun, Zhiyuan; Hixson, Eric D; Tang, Anne S; Phillips, Shannon C; Blackstone, Eugene H; Henderson, J Michael

2017-12-01

Anemia at hospitalization is often treated as an accompaniment to an underlying illness, without active investigation, despite its association with morbidity. Development of hospital-acquired anemia (HAA) has also been associated with increased risk for poor outcomes. Together, they may further heighten morbidity risk from bad to worse. The aims of this study were to (1) examine mortality, length of stay, and total charges in patients with present-on-admission (POA) anemia and (2) determine whether these are exacerbated by development of HAA. In this cohort investigation, from January 1, 2009, to August 31, 2011, a total of 44,483 patients with POA anemia were admitted to a single health system compared with a reference group of 48,640 without POA anemia or HAA. Data sources included the University HealthSystem Consortium database and electronic medical records. Risk-adjustment methods included logistic and linear regression models for mortality, length of stay, and total charges. Present-on-admission anemia was defined by administrative coding. Hospital-acquired anemia was determined by changes in hemoglobin values from the electronic medical record. Approximately one-half of the patients experienced worsening of anemia with development of HAA. Risk for death and resource use increased with increasing severity of HAA. Those who developed severe HAA had 2-fold greater odds for death; that is, mild POA anemia with development of severe HAA resulted in greater mortality (odds ratio, 2.57; 95% confidence interval, 2.08-3.18; P < 0.001), increased length of stay (2.23; 2.16-2.31; P < 0.001), and higher charges (2.09; 2.03-2.15; P < 0.001). Present-on-admission anemia is associated with increased mortality and resource use. This risk is further increased from bad to worse when patients develop HAA. Efforts to address POA anemia and HAA deserve attention.

Predictability of depression severity based on posterior alpha oscillations

NARCIS (Netherlands)

Jiang, H.; Popov, T.; Jylänki, P.P.; Bi, K.; Yao, Z.; Lu, Q.; Jensen, O.; Gerven, M.A.J. van

2016-01-01

Objective: We aimed to integrate neural data and an advanced machine learning technique to predict individual major depressive disorder (MDD) patient severity. Methods: MEG data was acquired from 22 MDD patients and 22 healthy controls (HC) resting awake with eyes closed. Individual power spectra

Epidemiological Study of Hospital-Acquired Bacterial Conjunctivitis in a Level III Neonatal Unit

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Catarina Dias

2013-01-01

Full Text Available Background. Conjunctivitis is one of the most frequently occurring hospital-acquired infections among neonates, although it is less studied than potentially life-threatening infections, such as sepsis and pneumonia. Objectives. The aims of our work were to identify epidemiologic characteristics, pathogens, and susceptibility patterns of bacterial hospital-acquired conjunctivitis (HAC in a level III neonatal unit. Materials and Methods. Data were collected retrospectively from patient charts and laboratory databases. Hospital-acquired conjunctivitis was defined in accordance with the Centers for Disease Control/National Healthcare Safety Network (CDC/NHSN diagnostic criteria. Results. One or more episodes of HAC were diagnosed in 4,0% ( of 1492 neonates admitted during the study period. Most of the episodes involved premature (75,4% and low birth weight (75,4% neonates. Infection rates were higher among patients undergoing noninvasive mechanical ventilation (46,7%, parenteral nutrition (13,6%, and phototherapy (6,8%. Predominant pathogens included Serratia marcescens (27,9%, Escherichia coli (23%, and Pseudomonas aeruginosa (18%. Susceptibility patterns revealed bacterial resistances to several antibiotic classes. Gentamicin remains the adequate choice for empirical treatment of HAC in our NICU. Conclusion. It is important to know the local patterns of the disease in order to adjust prevention strategies. Our work contributes to the epidemiological characterization of a sometimes overlooked disease.

[Increasing incidence of community-acquired pneumonia caused by atypical microorganisms].

Science.gov (United States)

Tazón-Varela, M A; Alonso-Valle, H; Muñoz-Cacho, P; Gallo-Terán, J; Piris-García, X; Pérez-Mier, L A

2017-09-01

Knowing the most common microorganisms in our environment can help us to make proper empirical treatment decisions. The aim is to identify those microorganisms causing community-acquired pneumonia. An observational, descriptive and prospective study was conducted, including patients over 14 years with a clinical and radiographic diagnosis of community-acquired pneumonia during a 383 consecutive day period. A record was made of sociodemographic variables, personal history, prognostic severity scales, progress, and pathogenic agents. The aetiological diagnosis was made using blood cultures, detection of Streptococcus pneumoniae and Legionella pneumophila urinary antigens, sputum culture, influenza virus and Streptococcus pyogenes detection. Categorical variables are presented as absolute values and percentages, and continuous variables as their means and standard deviations. Of the 287 patients included in the study (42% women, mean age 66±22 years), 10.45% died and 70% required hospital admission. An aetiological diagnosis was achieved in 43 patients (14.98%), with 16 microorganisms found in 59 positive samples. The most frequently isolated pathogen was Streptococcus pneumonia (24/59, 41%), followed by gram-negative enteric bacilli, Klebsiella pneumonia, Escherichia coli, Serratia marcescens and Enterobacter cloacae isolated in 20% of the samples (12/59), influenza virus (5/59, 9%), methicillin-resistant Staphylococcus aureus (3/59, 5%), Pseudomonas aeruginosa (2/59, 3%), Moraxella catarrhalis (2/59, 3%), Legionella pneumophila (2/59, 3%), and Haemophilus influenza (2/59, 3%). Polymicrobial infections accounted for 14% (8/59). A high percentage of atypical microorganisms causing community-acquired pneumonia were found. Copyright © 2016 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.

An Acquired Factor VIII Inhibitor in a Patient with HIV and HCV: A Case Presentation and Literature Review

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S. B. Zeichner

2013-01-01

Full Text Available Introduction. Despite its low incidence, acquired factor VIII inhibitor is the most common autoantibody affecting the clotting cascade. The exact mechanism of acquisition remains unclear, but postpartum patients, those with autoimmune conditions or malignancies, and those with exposure to particular drugs appear most susceptible. There have been several case reports describing acquired FVIII inhibitors in patients receiving interferon alpha for HCV treatment and in patients being treated for HIV. To our knowledge, this is the first case of a patient with HCV and HIV who was not actively receiving treatment for either condition. Case Presentation. A 57-year-old Caucasian male with a history of HIV and HCV was admitted to our hospital for a several day history of progressively worsening right thigh bruising and generalized weakness. CTA of the abdominal arteries revealed large bilateral retroperitoneal hematomas. Laboratory studies revealed the presence of a high titer FVIII inhibitor. Conclusion. Our case of a very rare condition highlights the importance of recognizing and understanding the diagnosis of acquired FVIII inhibitor. Laboratory research and clinical data on the role of newer agents are needed in order to better characterize disease pathogenesis, disease associations, genetic markers, and optimal disease management.

Long-Term Cognitive Impairment after Hospitalization for Community-Acquired Pneumonia: a Prospective Cohort Study.

Science.gov (United States)

Girard, Timothy D; Self, Wesley H; Edwards, Kathryn M; Grijalva, Carlos G; Zhu, Yuwei; Williams, Derek J; Jain, Seema; Jackson, James C

2018-06-01

Recent studies suggest older patients hospitalized for community-acquired pneumonia are at risk for new-onset cognitive impairment. The characteristics of long-term cognitive impairment after pneumonia, however, have not been elucidated. To characterize long-term cognitive impairment among adults of all ages hospitalized for community-acquired pneumonia. Prospective cohort study. Adults without severe preexisting cognitive impairment who were hospitalized with community-acquired pneumonia. At enrollment, we estimated baseline cognitive function with the Short Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE). At 2- and 12-month follow-up, we assessed cognition using the Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) and tests of executive function, diagnosing cognitive impairment when results were ≥ 1.5 standard deviations below published age-adjusted means for the general population. We also identified subtypes of mild cognitive impairment using standard definitions. We assessed 58 (73%) of 80 patients who survived to 2-month follow-up and 57 (77%) of 74 who survived to 12-month follow-up. The median [range] age of survivors tested was 57 [19-97] years. Only 8 (12%) had evidence of mild cognitive impairment at baseline according to the Short IQCODE, but 21 (38%) at 2 months and 17 (30%) at 12 months had mild cognitive impairment per the RBANS. Moderate-to-severe cognitive impairment was common among adults ≥ 65 years [4/13 (31%) and 5/13 (38%) at 2 and 12 months, respectively] but also affected many of those cognitive domains affected one-third of patients ≥ 65 years old and 20% of younger patients, and another third of survivors had mild cognitive impairment.

Hospital-Acquired Condition Reduction Program

Data.gov (United States)

U.S. Department of Health & Human Services — In October 2014, CMS began reducing Medicare payments for subsection (d) hospitals that rank in the worst performing quartile with respect to hospital-acquired...

Effects of hereditary and acquired risk factors of venous thrombosis on a thrombin generation-based APC resistance test

NARCIS (Netherlands)

Curvers, J; Thomassen, MCLGD; Rimmer, J; Hamulyak, K; van der Meer, J; Tans, G; Preston, FE; Rosing, J

Background. Several hereditary and acquired risk factors for venous thromboembolism (VTE) are associated with impaired down-regulation of thrombin formation via the protein C pathway. To identify individuals at risk, functional tests are needed that estimate the risk to develop venous thrombosis.

Clinicians' ability, motivation, and opportunity to acquire and transfer knowledge: An age-driven perspective.

Science.gov (United States)

Profili, Silvia; Sammarra, Alessia; Dandi, Roberto; Mascia, Daniele

2017-11-08

Many countries are seeing a dramatic increase in the average age of their clinicians. The literature often highlights the challenges of high replacement costs and the need for strategies to retain older personnel. Less discussed are the potential pitfalls of knowledge acquisition and transfer that accompany this aging issue. We propose a conceptual framework for understanding how clinicians' age interact with ability, motivation, and opportunity to predict clinical knowledge transfer and acquisition in health care organizations. This study integrates life-span development perspectives with the ability-motivation-opportunity framework to develop a number of testable propositions on the interaction between age and clinicians' ability, motivation, and opportunity to acquire and transfer clinical knowledge. We posit that the interaction between ability (the knowledge and skills to acquire knowledge), motivation (the willingness to acquire and transfer knowledge), and opportunity (resources required for acquiring and transferring knowledge) is a determinant of successful knowledge management. We also suggest that clinicians' age-and more specifically, the cognitive and motivational changes that accompany aging-moderates these relationships. This study contributes to existing research by offering a set of testable propositions for future research. These propositions will hopefully encourage empirical research into this important topic and lead to guidelines for reducing the risks of organizational knowledge loss due to aging. We suggest several ways that health care organizations can tailor managerial practices in order to help capitalize on the knowledge-based resources held by their younger and older clinicians. Such initiatives may affect employees' ability (e.g., by providing specific training programs), motivation (e.g., by expanding subjective perceptions of future time at work), and opportunities (e.g., by providing mentoring, reverse mentoring, and coaching

AETIOLOGY OF ACQUIRED LOWER MOTOR NEURON TYPE OF FACIAL NERVE PARALYSIS– A DESCRIPTIVE STUDY

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Arya Devi Karangat

2018-02-01

Full Text Available BACKGROUND This study was conducted to evaluate the various aetiologies of acquired lower motor neuron type of facial nerve paralysis, assessment of severity of lesion and outcome through follow up. 47 patients between 15-75 years were studied. MATERIALS AND METHODS All patients with acquired LMN facial paralysis who presented to our department were included in the study. They were evaluated with history, clinical examination and investigations. They were treated and followed up for a period of 6 months. RESULTS The most common aetiology identified for facial palsy was trauma which was non-iatrogenic. The anatomic level which predominated in our patients was infrastapedial. Maximum number of patients presented with grade 4 facial palsy. CONCLUSION Non- iatrogenic trauma was the most common cause among the patients studied and follow up of these patients had a good recovery of 60%.

Acquired ichthyosis with hoffman's syndrome

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Sathyanarayana B

2003-01-01

Full Text Available A middle aged man presented with features of acquired ichthyosis with Hoffman's syndrome. Laboratory tests support hypothyodism. Myoedema and hypertrophy of muscles were present. Patient was previously treated for Pellagra.

Acquired Von Willebrand’s Syndrome in Systemic Lupus Erythematosus

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Sara Taveras Alam

2014-01-01

Full Text Available Acquired von Willebrand syndrome (AVWS is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT with severely decreased levels of von Willebrand factor (VWF measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.

The Short Wave Aerostat-Mounted Imager (SWAMI): A novel platform for acquiring remotely sensed data from a tethered balloon

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Vierling, L.A.; Fersdahl, M.; Chen, X.; Li, Z.; Zimmerman, P.

2006-01-01

We describe a new remote sensing system called the Short Wave Aerostat-Mounted Imager (SWAMI). The SWAMI is designed to acquire co-located video imagery and hyperspectral data to study basic remote sensing questions and to link landscape level trace gas fluxes with spatially and temporally appropriate spectral observations. The SWAMI can fly at altitudes up to 2 km above ground level to bridge the spatial gap between radiometric measurements collected near the surface and those acquired by other aircraft or satellites. The SWAMI platform consists of a dual channel hyperspectral spectroradiometer, video camera, GPS, thermal infrared sensor, and several meteorological and control sensors. All SWAMI functions (e.g. data acquisition and sensor pointing) can be controlled from the ground via wireless transmission. Sample data from the sampling platform are presented, along with several potential scientific applications of SWAMI data.

Revisiting the Neural Basis of Acquired Amusia: Lesion Patterns and Structural Changes Underlying Amusia Recovery.

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Sihvonen, Aleksi J; Ripollés, Pablo; Rodríguez-Fornells, Antoni; Soinila, Seppo; Särkämö, Teppo

2017-01-01

Although, acquired amusia is a common deficit following stroke, relatively little is still known about its precise neural basis, let alone to its recovery. Recently, we performed a voxel-based lesion-symptom mapping (VLSM) and morphometry (VBM) study which revealed a right lateralized lesion pattern, and longitudinal gray matter volume (GMV) and white matter volume (WMV) changes that were specifically associated with acquired amusia after stroke. In the present study, using a larger sample of stroke patients ( N = 90), we aimed to replicate and extend the previous structural findings as well as to determine the lesion patterns and volumetric changes associated with amusia recovery. Structural MRIs were acquired at acute and 6-month post-stroke stages. Music perception was behaviorally assessed at acute and 3-month post-stroke stages using the Scale and Rhythm subtests of the Montreal Battery of Evaluation of Amusia (MBEA). Using these scores, the patients were classified as non-amusic, recovered amusic, and non-recovered amusic. The results of the acute stage VLSM analyses and the longitudinal VBM analyses converged to show that more severe and persistent (non-recovered) amusia was associated with an extensive pattern of lesions and GMV/WMV decrease in right temporal, frontal, parietal, striatal, and limbic areas. In contrast, less severe and transient (recovered) amusia was linked to lesions specifically in left inferior frontal gyrus as well as to a GMV decrease in right parietal areas. Separate continuous analyses of MBEA Scale and Rhythm scores showed extensively overlapping lesion pattern in right temporal, frontal, and subcortical structures as well as in the right insula. Interestingly, the recovered pitch amusia was related to smaller GMV decreases in the temporoparietal junction whereas the recovered rhythm amusia was associated to smaller GMV decreases in the inferior temporal pole. Overall, the results provide a more comprehensive picture of the lesions

Revisiting the Neural Basis of Acquired Amusia: Lesion Patterns and Structural Changes Underlying Amusia Recovery

Science.gov (United States)

Sihvonen, Aleksi J.; Ripollés, Pablo; Rodríguez-Fornells, Antoni; Soinila, Seppo; Särkämö, Teppo

2017-01-01

Although, acquired amusia is a common deficit following stroke, relatively little is still known about its precise neural basis, let alone to its recovery. Recently, we performed a voxel-based lesion-symptom mapping (VLSM) and morphometry (VBM) study which revealed a right lateralized lesion pattern, and longitudinal gray matter volume (GMV) and white matter volume (WMV) changes that were specifically associated with acquired amusia after stroke. In the present study, using a larger sample of stroke patients (N = 90), we aimed to replicate and extend the previous structural findings as well as to determine the lesion patterns and volumetric changes associated with amusia recovery. Structural MRIs were acquired at acute and 6-month post-stroke stages. Music perception was behaviorally assessed at acute and 3-month post-stroke stages using the Scale and Rhythm subtests of the Montreal Battery of Evaluation of Amusia (MBEA). Using these scores, the patients were classified as non-amusic, recovered amusic, and non-recovered amusic. The results of the acute stage VLSM analyses and the longitudinal VBM analyses converged to show that more severe and persistent (non-recovered) amusia was associated with an extensive pattern of lesions and GMV/WMV decrease in right temporal, frontal, parietal, striatal, and limbic areas. In contrast, less severe and transient (recovered) amusia was linked to lesions specifically in left inferior frontal gyrus as well as to a GMV decrease in right parietal areas. Separate continuous analyses of MBEA Scale and Rhythm scores showed extensively overlapping lesion pattern in right temporal, frontal, and subcortical structures as well as in the right insula. Interestingly, the recovered pitch amusia was related to smaller GMV decreases in the temporoparietal junction whereas the recovered rhythm amusia was associated to smaller GMV decreases in the inferior temporal pole. Overall, the results provide a more comprehensive picture of the lesions

Revisiting the Neural Basis of Acquired Amusia: Lesion Patterns and Structural Changes Underlying Amusia Recovery

Directory of Open Access Journals (Sweden)

Aleksi J. Sihvonen

2017-07-01

Full Text Available Although, acquired amusia is a common deficit following stroke, relatively little is still known about its precise neural basis, let alone to its recovery. Recently, we performed a voxel-based lesion-symptom mapping (VLSM and morphometry (VBM study which revealed a right lateralized lesion pattern, and longitudinal gray matter volume (GMV and white matter volume (WMV changes that were specifically associated with acquired amusia after stroke. In the present study, using a larger sample of stroke patients (N = 90, we aimed to replicate and extend the previous structural findings as well as to determine the lesion patterns and volumetric changes associated with amusia recovery. Structural MRIs were acquired at acute and 6-month post-stroke stages. Music perception was behaviorally assessed at acute and 3-month post-stroke stages using the Scale and Rhythm subtests of the Montreal Battery of Evaluation of Amusia (MBEA. Using these scores, the patients were classified as non-amusic, recovered amusic, and non-recovered amusic. The results of the acute stage VLSM analyses and the longitudinal VBM analyses converged to show that more severe and persistent (non-recovered amusia was associated with an extensive pattern of lesions and GMV/WMV decrease in right temporal, frontal, parietal, striatal, and limbic areas. In contrast, less severe and transient (recovered amusia was linked to lesions specifically in left inferior frontal gyrus as well as to a GMV decrease in right parietal areas. Separate continuous analyses of MBEA Scale and Rhythm scores showed extensively overlapping lesion pattern in right temporal, frontal, and subcortical structures as well as in the right insula. Interestingly, the recovered pitch amusia was related to smaller GMV decreases in the temporoparietal junction whereas the recovered rhythm amusia was associated to smaller GMV decreases in the inferior temporal pole. Overall, the results provide a more comprehensive picture of

Occupationally Acquired American Cutaneous Leishmaniasis

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Maria Edileuza Felinto de Brito

2012-01-01

Full Text Available We report two occupationally acquired cases of American cutaneous leishmaniasis (ACL: one accidental laboratory autoinoculation by contaminated needlestick while handling an ACL lesion sample, and one acquired during field studies on bird biology. Polymerase chain reaction (PCR assays of patient lesions were positive for Leishmania, subgenus Viannia. One isolate was obtained by culture (from patient 2 biopsy samples and characterized as Leishmania (Viannia naiffi through an indirect immunofluorescence assay (IFA with species-specific monoclonal antibodies (mAbs and by multilocus enzyme electrophoresis (MLEE. Patients were successfully treated with N-methyl-glucamine. These two cases highlight the potential risks of laboratory and field work and the need to comply with strict biosafety procedures in daily routines. The swab collection method, coupled with PCR detection, has greatly improved ACL laboratory diagnosis.

Association of hypercapnia on admission with increased length of hospital stay and severity in patients admitted with community-acquired pneumonia: a prospective observational study from Pakistan.

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Iqbal, Nousheen; Irfan, Muhammad; Zubairi, Ali Bin Sarwar; Awan, Safia; Khan, Javaid A

2017-06-15

To determine whether the presence of hypercapnia on admission in adult patients admitted to a university-based hospital in Karachi, Pakistan with community-acquired pneumonia (CAP) correlates with an increased length of hospital stay and severity compared with no hypercapnia on admission. A prospective observational study. Tertiary care hospital in Karachi, Pakistan. Patients who met the inclusion criteria were enrolled in the study. The severity of pneumonia was assessed by CURB-65 and PSI scores. An arterial blood gas analysis was obtained within 24 hours of admission. Based on arterial PaCO 2 levels, patients were divided into three groups: hypocapnic (PaCO 2 45 mm Hg) and normocapnic (PaCO 2 <35-45 mm Hg). The primary outcome was the association of hypercapnia on admission with mean length of hospital stay. Secondary outcomes were the need for mechanical ventilation, ICU admission and in-hospital mortality. A total of 295 patients of mean age 60.20±17.0 years (157 (53.22%) men) were enrolled over a 1-year period. Hypocapnia was found in 181 (61.35%) and hypercapnia in 57 (19.32%) patients. Hypercapnic patients had a longer hospital stay (mean 9.27±7.57 days), increased requirement for non-invasive mechanical ventilation (NIMV) on admission (n=45 (78.94%)) and longer mean time to clinical stability (4.39±2.0 days) compared with the other groups. Overall mortality was 41 (13.89%), but there was no statistically significant difference in mortality (p=0.35) and ICU admission (p=0.37) between the three groups. On multivariable analysis, increased length of hospital stay was associated with NIMV use, ICU admission, hypercapnia and normocapnia. Hypercapnia on admission is associated with severity of CAP, longer time to clinical stability, increased length of hospital stay and need for NIMV. It should be considered as an important criterion to label the severity of the illness and also a determinant of patients who will require a higher level of hospital

Naturally acquired immunity to Plasmodium falciparum malaria in Africa

DEFF Research Database (Denmark)

Hviid, Lars

2005-01-01

Infection by Plasmodium falciparum parasites can lead to substantial protective immunity to malaria, and available evidence suggest that acquisition of protection against some severe malaria syndromes can be fairly rapid. Although these facts have raised hopes that the development of effective...... protective immunity to P. falciparum malaria is acquired following natural exposure to the parasites is beginning to emerge, not least thanks to studies that have combined clinical and epidemiological data with basic immunological research. This framework involves IgG with specificity for clonally variant...... antigens on the surface of the infected erythrocytes, can explain some of the difficulties in relating particular immune responses with specificity for well-defined antigenic targets to clinical protection, and suggests a radically new approach to controlling malaria-related morbidity and mortality...

Recovering With Acquired Apraxia of Speech: The First 2 Years.

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Haley, Katarina L; Shafer, Jennifer N; Harmon, Tyson G; Jacks, Adam

2016-12-01

This study was intended to document speech recovery for 1 person with acquired apraxia of speech quantitatively and on the basis of her lived experience. The second author sustained a traumatic brain injury that resulted in acquired apraxia of speech. Over a 2-year period, she documented her recovery through 22 video-recorded monologues. We analyzed these monologues using a combination of auditory perceptual, acoustic, and qualitative methods. Recovery was evident for all quantitative variables examined. For speech sound production, the recovery was most prominent during the first 3 months, but slower improvement was evident for many months. Measures of speaking rate, fluency, and prosody changed more gradually throughout the entire period. A qualitative analysis of topics addressed in the monologues was consistent with the quantitative speech recovery and indicated a subjective dynamic relationship between accuracy and rate, an observation that several factors made speech sound production variable, and a persisting need for cognitive effort while speaking. Speech features improved over an extended time, but the recovery trajectories differed, indicating dynamic reorganization of the underlying speech production system. The relationship among speech dimensions should be examined in other cases and in population samples. The combination of quantitative and qualitative analysis methods offers advantages for understanding clinically relevant aspects of recovery.

7 CFR 3575.90 - Disposition of acquired property.

Science.gov (United States)

2010-01-01

... property. (a) General. When the lender acquires title to the collateral and the final loss claim is not... 7 Agriculture 15 2010-01-01 2010-01-01 false Disposition of acquired property. 3575.90 Section... protect the collateral, and the lender must dispose of the collateral without delay. (b) Re-title...

Relative Contribution of Dengue IgG Antibodies Acquired during Gestation or Breastfeeding in Mediating Dengue Disease Enhancement and Protection in Type I Interferon Receptor-Deficient Mice.

Directory of Open Access Journals (Sweden)

Pei Xuan Lee

2016-06-01

Full Text Available Dengue virus (DENV causes a spectrum of diseases ranging from self-limiting dengue fever to severe conditions such as haemorrhagic fever and dengue shock syndrome. Antibody-dependent enhancement (ADE is thought to explain the occurrence of severe dengue whereby pre-existing binding but non-neutralising antibodies enhance DENV infection. The ADE phenomenon is supported by epidemiological findings that infants that born to dengue immune mothers are at greater risk to develop severe dengue upon primary infection. The role of maternally acquired dengue-specific antibodies in disease enhancement was recently recapitulated in a mouse model where mice born to DENV1-immune mothers experienced enhanced disease severity upon DENV2 infection. Here, this study investigates the relative contribution of maternal dengue-specific antibodies acquired during gestation and breastfeeding in dengue disease. Using a surrogate breastfeeding mother experimental approach, we showed that majority of the maternal dengue-specific antibodies were acquired during breastfeeding and conferred an extended enhancement window. On the other hand, in the context of homologous infection, breastfeeding conferred protection. Furthermore, measurement of dengue-specific antibody titres over time in mice born to dengue immune mothers revealed a biphasic pattern of antibody decay as reported in humans. Our work provides evidence of the potential contribution of breast milk-acquired dengue-specific IgG antibodies in enhancement and protection against dengue. Should such contribution be established in humans as well, it may have important implications for the development of guidelines to dengue-immune breastfeeding mothers.

Relative Contribution of Dengue IgG Antibodies Acquired during Gestation or Breastfeeding in Mediating Dengue Disease Enhancement and Protection in Type I Interferon Receptor-Deficient Mice.

Science.gov (United States)

Lee, Pei Xuan; Ong, Li Ching; Libau, Eshele Anak; Alonso, Sylvie

2016-06-01

Dengue virus (DENV) causes a spectrum of diseases ranging from self-limiting dengue fever to severe conditions such as haemorrhagic fever and dengue shock syndrome. Antibody-dependent enhancement (ADE) is thought to explain the occurrence of severe dengue whereby pre-existing binding but non-neutralising antibodies enhance DENV infection. The ADE phenomenon is supported by epidemiological findings that infants that born to dengue immune mothers are at greater risk to develop severe dengue upon primary infection. The role of maternally acquired dengue-specific antibodies in disease enhancement was recently recapitulated in a mouse model where mice born to DENV1-immune mothers experienced enhanced disease severity upon DENV2 infection. Here, this study investigates the relative contribution of maternal dengue-specific antibodies acquired during gestation and breastfeeding in dengue disease. Using a surrogate breastfeeding mother experimental approach, we showed that majority of the maternal dengue-specific antibodies were acquired during breastfeeding and conferred an extended enhancement window. On the other hand, in the context of homologous infection, breastfeeding conferred protection. Furthermore, measurement of dengue-specific antibody titres over time in mice born to dengue immune mothers revealed a biphasic pattern of antibody decay as reported in humans. Our work provides evidence of the potential contribution of breast milk-acquired dengue-specific IgG antibodies in enhancement and protection against dengue. Should such contribution be established in humans as well, it may have important implications for the development of guidelines to dengue-immune breastfeeding mothers.

Relative Contribution of Dengue IgG Antibodies Acquired during Gestation or Breastfeeding in Mediating Dengue Disease Enhancement and Protection in Type I Interferon Receptor-Deficient Mice

Science.gov (United States)

Lee, Pei Xuan; Ong, Li Ching; Libau, Eshele Anak; Alonso, Sylvie

2016-01-01

Dengue virus (DENV) causes a spectrum of diseases ranging from self-limiting dengue fever to severe conditions such as haemorrhagic fever and dengue shock syndrome. Antibody-dependent enhancement (ADE) is thought to explain the occurrence of severe dengue whereby pre-existing binding but non-neutralising antibodies enhance DENV infection. The ADE phenomenon is supported by epidemiological findings that infants that born to dengue immune mothers are at greater risk to develop severe dengue upon primary infection. The role of maternally acquired dengue-specific antibodies in disease enhancement was recently recapitulated in a mouse model where mice born to DENV1-immune mothers experienced enhanced disease severity upon DENV2 infection. Here, this study investigates the relative contribution of maternal dengue-specific antibodies acquired during gestation and breastfeeding in dengue disease. Using a surrogate breastfeeding mother experimental approach, we showed that majority of the maternal dengue-specific antibodies were acquired during breastfeeding and conferred an extended enhancement window. On the other hand, in the context of homologous infection, breastfeeding conferred protection. Furthermore, measurement of dengue-specific antibody titres over time in mice born to dengue immune mothers revealed a biphasic pattern of antibody decay as reported in humans. Our work provides evidence of the potential contribution of breast milk-acquired dengue-specific IgG antibodies in enhancement and protection against dengue. Should such contribution be established in humans as well, it may have important implications for the development of guidelines to dengue-immune breastfeeding mothers. PMID:27341339

Duodenal Tumor Presenting as Acquired Hemophilia in an 88-Year-Old Woman: A Clinical Case and Review of the Literature

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Nigel P. Murray

2012-01-01

Full Text Available Acquired hemophilia is a rare disease, presenting with severe hemorrhage, we present a case caused by a duodenal tumor, the clinical management, ethical implications, treatment recommendations, and a review of the literature.

Physical Plant Design and Engineering Controls to Reduce Hospital-Acquired Infections

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JM Conly

2006-01-01

Full Text Available The importance of the environment as a reservoir for microorganisms implicated in disease transmission in the hospital setting has been increasingly recognized, especially with respect to dialysis units, ventilation in specialized areas, and the proper use of disinfectants (1. Inherent within the environmental setting is the importance of physical plant design. Several studies have underscored the importance of optimizing design standards to maximize patient and health care worker (HCW safety, including the prevention of hospital-acquired infections in patients (2-6. Ulrich et al (7 recently completed an evidence-based review, entitled 'The role of the physical environment in the hospital of the 21st century: A once-in-a-lifetime opportunity', for the Center for Health Design in California (USA, which was funded by the Robert Wood Johnson Foundation. Ulrich and colleagues identified over 600 studies that examined the hospital environment and its effects on staff effectiveness, patient safety, patient and family stress, quality and costs. They suggested that one of the important elements in improving patient safety is the reduction of the risk of hospital-acquired infections through improved facility design.

Comparative antimicrobial susceptibility of aerobic and facultative bacteria from community-acquired bacteremia to ertapenem in Taiwan

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Fung Chang-Phone

2007-07-01

-tazobactam, or ciprofloxacin against oxacillin-susceptible S. aureus (17/17, 100%and was more active than any of these agents against enterobacteriaceae (82/82, 100%. Conclusion Based on the microbiology pattern of community-acquired bacteremia, initial empiric treatment that requires coverage of a broad spectrum of both gram-negative and gram-positive aerobic bacteria, such as ertapenem, may be justified in moderately severe cases of community-acquired bacteremia in non-immunocompromised hosts.

Long-term psychological functioning of adults with severe congenital facial disfigurement.

Science.gov (United States)

Versnel, Sarah L; Plomp, Raul G; Passchier, Jan; Duivenvoorden, Hugo J; Mathijssen, Irene M J

2012-01-01

In adults with severe congenital facial disfigurement, assessment of long-term psychological impact remains limited. This study determines the long-term psychological functioning in these patients and evaluates differences compared with patients with acquired facial disfigurement and a non-facially disfigured reference group. Also explored is the extent to which psychological functioning of the congenital group is related to satisfaction with facial appearance, fear of negative appearance evaluation by others, self-esteem, and severity of the facial deformity. Fifty-nine adults with severe congenital facial disfigurement, 59 adults with a traumatically acquired facial deformity in adulthood, and 120 non-facially disfigured adults completed standardized psychological, physical, and demographic questionnaires, including the Fear of Negative Appearance Evaluation Scale, the Rosenberg Self-Esteem Scale, the Hospital Anxiety and Depression Scale, the Achenbach Adult Self-Report, the 36-Item Short-Form Health Survey, and a visual analogue scale. Adults with severe congenital facial disfigurement had relatively normal psychological functioning but appeared more prone to internalizing problems than the non-facially disfigured adults. Compared with patients with an acquired facial deformity, the congenital group displayed fewer problems on the physical component score of quality of life only. Satisfaction with facial appearance, fear of negative appearance evaluation, and self-esteem were good predictors of the different aspects of psychological functioning, with the exception of the physical component score of quality of life. Improving satisfaction with facial appearance (by surgery), enhancing self-esteem, or lowering fear of negative appearance evaluation (by psychological support) may enhance long-term psychological functioning. Future research should focus on the individual patient and risk factors for maladjustment. Risk, II.

Association between Obstructive Sleep Apnea and Community-Acquired Pneumonia.

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Eusebi Chiner

Full Text Available We hypothesized that obstructive sleep apnea (OSA can predispose individuals to lower airway infections and community-acquired pneumonia (CAP due to upper airway microaspiration. This study evaluated the association between OSA and CAP.We performed a case-control study that included 82 patients with CAP and 41 patients with other infections (control group. The controls were matched according to age, sex and body mass index (BMI. A respiratory polygraph (RP was performed upon admission for patients in both groups. The severity of pneumonia was assessed according to the Pneumonia Severity Index (PSI. The associations between CAP and the Epworth Sleepiness Scale (ESS, OSA, OSA severity and other sleep-related variables were evaluated using logistic regression models. The associations between OSA, OSA severity with CAP severity were evaluated with linear regression models and non-parametric tests.No significant differences were found between CAP and control patients regarding anthropometric variables, toxic habits and risk factors for CAP. Patients with OSA, defined as individuals with an Apnea-Hypopnea Index (AHI ≥10, showed an increased risk of CAP (OR = 2·86, 95%CI 1·29-6·44, p = 0·01. Patients with severe OSA (AHI≥30 also had a higher risk of CAP (OR = 3·18, 95%CI 1·11-11·56, p = 0·047. In addition, OSA severity, defined according to the AHI quartile, was also significantly associated with CAP (p = 0·007. Furthermore, OSA was significantly associated with CAP severity (p = 0·0002, and OSA severity was also associated with CAP severity (p = 0·0006.OSA and OSA severity are associated with CAP when compared to patients admitted to the hospital for non-respiratory infections. In addition, OSA and OSA severity are associated with CAP severity. These results support the potential role of OSA in the pathogenesis of CAP and could have clinical implications. This link between OSA and infection risk should be explored to investigate the

Long-term psychological functioning of adults with severe congenital facial disfigurement

NARCIS (Netherlands)

Passchier, J.; Versnel, S.L.; Plomp, R.G.; Duivenvoorden, H.J.; Mathijssen, I.M.

2012-01-01

BACKGROUND: In adults with severe congenital facial disfigurement, assessment of long-term psychological impact remains limited. This study determines the long-term psychological functioning in these patients and evaluates differences compared with patients with acquired facial disfigurement and a