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Sample records for hemolytic anaemia patients

  1. Study of chronic hemolytic anaemia patients in Rio de Janeiro: prevalence of anti-human parvovirus B19 IgG antibodies and the developement aplastic crises

    Directory of Open Access Journals (Sweden)

    SANT'ANNA Anadayr L.M.

    2002-01-01

    Full Text Available The prevalence of anti-human parvovirus B19 IgG antibodies was determined in sera from 165 chronic hemolytic anemia patients, receiving medical care at Instituto Estadual de Hematologia (IEHE, Rio de Janeiro, during the year of 1994. This sample represents around 10% of the chronic hemolytic anemia patients attending at IEHE. Most of these patients (140 have sickle cell disease. Anti-B19 IgG antibodies were detected in 32.1% of patients. No statistically significant difference (p > 0.05 was seen between IgG antibody prevalence in male (27.8% and female (35.5% patients. Anti-B19 IgG antibodies were more frequent in older (37.6% than younger (28.2% than 20 years old patients, although this difference had no statistical significance (p > 0.05. Anti-B19 IgG antibody prevalence showed that 67.9% of patients enrolled in the study were susceptible to B19 acute infection. With the aim to detect acute B19 infection, patients follow up continued until February 1996. During this period four patients presented transient aplastic crisis due to human parvovirus B19 as confirmed by the detection of specific IgM antibodies. All four patients were younger than 20 years old, and 3 were younger than 10 years old. Three of them were sickle cell disease patients. Three of the four acute B19 infection occurred during 1994 springtime.

  2. Alpha thalassemia among sickle cell anaemia patients in Kampala ...

    African Journals Online (AJOL)

    Keywords: Alpha thalassemia, sickle cell anaemia patients, Kampala, Uganda. DOI: http://dx.doi.org/10.4314/ahs.v15i2.48. Introduction. In the early 1960's many adults with sickle cell anaemia. (SCA) as well as those with mild disease were reported in Jamaica1. Various factors, both genetic and environmental, are.

  3. THE IMMUNOLOGICAL CHARACTERISTIC OF RA PATIENTS WITH ANAEMIA

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    A. E. Sizikov

    2014-07-01

    Full Text Available Abstract. The aim of the investigation was to study the immunological characteristics of RA patients with anaemia. Clinical and laboratory data including the percentage of the main lymphocyte subclasses, phagocyte and DTH-effector activity, serum concentration of immunoglobulins, the percentage of cells producing IFNγ and/or IL-4 and percent of monocytes producing TNF. We revealed some significant clinical, laboratory and immunological differences between RA patients and healthy donors and between patients with and without anaemia. Our data demonstrate RA anemic patients to have more severe disorders than patients without anaemia. We also revealed some significant immunological differences between RA patients and healthy donors and between patients with and without anaemia, including percent of cells producing IFNγ and/or IL-4. Our data permit to conclude that RA patients have many different immunological disturbances, more severe in anaemic patients.

  4. Anaemia in Patients with Diabetes Mellitus attending regular ...

    African Journals Online (AJOL)

    Anaemia in Patients with Diabetes Mellitus attending regular Diabetic ... Nigerian Journal of Health and Biomedical Sciences ... some patients may omit important food items in their daily diet for fear of increasing their blood sugar level.

  5. Erectile Dysfunction in patients with Sickle Cell Anaemia | Ibidapo ...

    African Journals Online (AJOL)

    Erectile Dysfunction in patients with Sickle Cell Anaemia. ... leading to an increased haemolysis as well as vaso-occlusive complications including ... bone pain crises, blood transfusion (over a 3 year period) as well as erectile dysfunction.

  6. Cardio–Pulmonary Response Of Patients With Sickle Cell Anaemia ...

    African Journals Online (AJOL)

    Cardio–Pulmonary Response Of Patients With Sickle Cell Anaemia ... any risk of adverse cardio-respiratory response during the course of physical rehabilitation. A total of 70 subjects participated in the study; 30 of these had Haemoglobin ...

  7. Fanconi anaemia in South African patients with Afrikaner ancestry

    African Journals Online (AJOL)

    Fanconi anaemia (FA) is a rare genetic condition of impaired DNA ... An earlier age of diagnosis of FA in Afrikaner patients, a high frequency of somatic anomalies and a ... median current age and median age of presentation with symptoms in.

  8. Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda.

    Science.gov (United States)

    Lubega, Irene; Ndugwa, Christopher M; Mworozi, Edison A; Tumwine, James K

    2015-06-01

    Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described. To determine the prevalence of α+thalassaemia among sickle cell anaemia patients in Mulago Hospital and to describe the clinical and laboratory findings in these patients. A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction. Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003). The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemia patients more than 60 months of age at Mulago hospital.

  9. [Microalbuminuria in pediatric patients diagnosed with hemolytic uremic syndrome].

    Science.gov (United States)

    Cubillos C, María Paz; Del Salas, Paulina; Zambrano, Pedro O

    2015-01-01

    Hemolytic uremic syndrome (HUS) is characterized by the presence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. It is the leading cause of acute kidney failure in children under 3 years of age. A variable number of patients develop proteinuria, hypertension, and chronic renal failure. To evaluate the renal involvement in pediatric patients diagnosed with HUS using the microalbumin/creatinine ratio. Descriptive concurrent cohort study that analyzed the presence of microalbuminuria in patients diagnosed with HUS between January 2001 and March 2012, who evolved without hypertension and normal renal function (clearance greater than 90ml/min using Schwartz formula). Demographic factors (age, sex), clinical presentation at time of diagnosis, use of antibiotics prior to admission, and need for renal replacement therapy were evaluated. Of the 24 patients studied, 54% were male. The mean age at diagnosis was two years. Peritoneal dialysis was required in 45%, and 33% developed persistent microalbuminuria. Antiproteinuric treatment was introduce in 4 patients, with good response. The mean follow-up was 6 years (range 6 months to 11 years). The serum creatinine returned to normal in all patients during follow up. The percentage of persistent microalbuminuria found in patients with a previous diagnosis of HUS was similar in our group to that described in the literature. Antiproteinuric treatment could delay kidney damage, but further multicenter prospective studies are necessary. Copyright © 2015. Publicado por Elsevier España, S.L.U.

  10. [Treatment and results of therapy in autoimmune hemolytic anemia].

    Science.gov (United States)

    Tasić, J; Macukanović, L; Pavlović, M; Koraćević, S; Govedarević, N; Kitić, Lj; Tijanić, I; Bakić, M

    1994-01-01

    Basic principles in the therapy of idiopathic autoimmune hemolytic anemia induced by warm antibody were glucocorticoides and splenectomy. Immunosupresive drugs, plasmaferesis and intravenous high doses gamma globulin therapy are also useful. In secundary autoimmune hemolytic anemia induced by warm antibody we treated basic illness. During the period of 1990-1992 we treated 21 patients with primary autoimmune hemolytic anemia and 6 patients with secondary /4 CLL and 2 Non-Hodgkin's lymphoma/. Complete remission we found as a normalisation of reticulocites and hemoglobin level respectively. Complete remission by corticoides we got in 14/21 patients, partial response in 2/21 respectively. Complete response by splenectomy we got in 2/3 splenoctomized patients (idiopathic type). For successful treatment secondary hemolytic anemias we treated primary diseases (CLL and malignant lymphoma) and we got in 4/6 patients complete remission. Our results were standard in both type of autoimmune hemolytic anaemias induced by warm antibody.

  11. ORIGINAL ARTICLES Anaemia among patients with congestive ...

    African Journals Online (AJOL)

    2009-12-01

    Dec 1, 2009 ... 25% of men and 38% of women still alive at 5 years.5 Effective management depends on knowledge ... average length of stay in hospital of 7.4 days.6 There are limited data on long- and ..... to evolve. Mechanisms of anaemia ...

  12. Positive predictive value of diagnosis coding for hemolytic anemias in the Danish National Patient Register

    DEFF Research Database (Denmark)

    Hansen, Dennis Lund; Overgaard, Ulrik Malthe; Pedersen, Lars

    2016-01-01

    . Patients with mechanical reason for hemolysis such as an artificial heart valve, and patients with vitamin-B12 or folic acid deficiency were excluded. RESULTS: We identified 412 eligible patients: 249 with a congenital hemolytic anemia diagnosis and 163 with acquired hemolytic anemia diagnosis. In all...

  13. Leukemoid reaction, a rare manifestation of autoimmune hemolytic anemia in a case of small duct primary sclerosing cholangitis.

    Science.gov (United States)

    Salagre, Kaustubh D; Sahay, Ravindra Nath; Patil, Anuja; Pati, Anuja; Joshi, Amita; Shukla, Akash

    2013-10-01

    A 48 year old lady presented with jaundice and exertional breathlesness. Her laboratory reports showed anaemia, reticulocytosis, leucocytosis, elevated Lactate Dehydrogenase (LDH), alkaline phosphatase levels, hyperbillirubinemia and positive direct Coomb's test. After ruling out all the other causes of autoimmunity and hemolytic anemia, she was diagnosed as leukemoid reaction due to autoimmune hemolytic anemia with primary sclerosing cholangitis. Patient showed immediate improvement after corticosteroids.

  14. Bacterial pyomyositis in a patient with aplastic anaemia.

    OpenAIRE

    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.

  15. Gastroscopic screening in 80 patients with pernicious anaemia.

    OpenAIRE

    Stockbrügger, R W; Menon, G G; Beilby, J O; Mason, R R; Cotton, P B

    1983-01-01

    We have studied 80 patients with pernicious anaemia. Upper gastrointestinal endoscopy (with biopsy and cytology) showed no lesion other than atrophic gastritis in 34 patients. Thirty three patients, however, had varying degrees of gastric mucosal dysplasia, which was detected more frequently by histology than by cytology. The endoscopic appearance of the mucosa was abnormal in four of the six patients with moderate dysplasia, and in all three patients with severe dysplasia. One patient was fo...

  16. Beta thalassaemia traits in Nigerian patients with sickle cell anaemia ...

    African Journals Online (AJOL)

    Journal of Medicine and Biomedical Research ... These three patients (1.2%) were found to have positive co-inheritance of thalassaemia trait and sickle cell anaemia. The erythrocyte indices were all reduced in these selected families except for one family whose mean cell haemoglobin concentration was within normal ...

  17. Alloimmunization in autoimmune hemolytic anemia patient: The differential adsorption approach

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    Ravi C Dara

    2017-01-01

    Full Text Available Patients of β-thalassemia major are dependent on regular blood transfusions for their entire lifetime. Development of antibodies against red blood cell (RBC antigen which may be alloantibody or autoantibody, several times as a result of frequent red cell component transfusions, further complicates the subsequent transfusion therapy. Among the autoantibodies, warm-reactive autoantibodies are commoner and interfere in the pretransfusion testing. These RBC autoantibodies present in patient's serum potentially react with all the cells of antibody identification panel giving “pan-reactive” picture and making alloantibody identification complex. In this report, we present our approach in a thalassemia patient who presented with warm-type autoimmune hemolytic anemia, low hemoglobin of 5.8 g/dl, and three significant alloantibodies (anti-D, anti-S, and anti-Jk b which were masked by pan-reactive warm autoantibody(s. Differential adsorption was used to unmask underlying alloantibodies. We suggest that differential adsorption procedure is an effective and efficient method for autoantibody adsorption, detection, and identification of masked alloantibody(s, especially in patients with low hemoglobin and history of recent blood transfusion.

  18. Prevalence and risk factors of anaemia in paediatric patients in ...

    African Journals Online (AJOL)

    intrinsic potential to cause anaemia,[8,9] and the relative contribution of these ... aim of preventing and controlling childhood anaemia.[18,19] In spite ..... moderate anaemia in pregnancy and helminth, malaria and HIV infection in Entebbe ...

  19. Investigation of FANCA gene in Fanconi anaemia patients in Iran

    Science.gov (United States)

    Saffar Moghadam, Ali Akbar; Mahjoubi, Frouzandeh; Reisi, Nahid; Vosough, Parvaneh

    2016-01-01

    Background & objectives: Fanconi anaemia (FA) is a syndrome with a predisposition to bone marrow failure, congenital anomalies and malignancies. It is characterized by cellular hypersensitivity to cross-linking agents such as mitomycin C (MMC). In the present study, a new approach was selected to investigate FANCA (Fanconi anaemia complementation group A) gene in patients clinically diagnosed with cellular hypersensitivity to DNA cross-linking agent MMC. Methods: Chromosomal breakage analysis was performed to prove the diagnosis of Fanconi anaemia in 318 families. Of these, 70 families had a positive result. Forty families agreed to molecular genetic testing. In total, there were 27 patients with unknown complementary types. Genomic DNA was extracted and total RNA was isolated from fresh whole blood of the patients. The first-strand cDNA was synthesized and the cDNA of each patient was then tested with 21 pairs of overlapping primers. High resolution melting curve analysis was used to screen FANCA, and LinReg software version 1.7 was utilized for analysis of expression. Results: In total, six sequence alterations were identified, which included two stop codons, two frames-shift mutations, one large deletion and one amino acid exchange. FANCA expression was downregulated in patients who had sequence alterations. Interpretation & conclusions: The results of the present study show that high resolution melting (HRM) curve analysis may be useful in the detection of sequence alteration. It is simpler and more costeffective than the multiplex ligation-dependent probe amplification (MLPA) procedure. PMID:27121516

  20. Investigation of FANCA gene in Fanconi anaemia patients in Iran.

    Science.gov (United States)

    Moghadam, Ali Akbar Saffar; Mahjoubi, Frouzandeh; Reisi, Nahid; Vosough, Parvaneh

    2016-02-01

    Fanconi anaemia (FA) is a syndrome with a predisposition to bone marrow failure, congenital anomalies and malignancies. It is characterized by cellular hypersensitivity to cross-linking agents such as mitomycin C (MMC). In the present study, a new approach was selected to investigate FANCA (Fanconi anaemia complementation group A) gene in patients clinically diagnosed with cellular hypersensitivity to DNA cross-linking agent MMC. Chromosomal breakage analysis was performed to prove the diagnosis of Fanconi anaemia in 318 families. Of these, 70 families had a positive result. Forty families agreed to molecular genetic testing. In total, there were 27 patients with unknown complementary types. Genomic DNA was extracted and total RNA was isolated from fresh whole blood of the patients. The first-strand cDNA was synthesized and the cDNA of each patient was then tested with 21 pairs of overlapping primers. High resolution melting curve analysis was used to screen FANCA, and LinReg software version 1.7 was utilized for analysis of expression. In total, six sequence alterations were identified, which included two stop codons, two frames-shift mutations, one large deletion and one amino acid exchange. FANCA expression was downregulated in patients who had sequence alterations. The results of the present study show that high resolution melting (HRM) curve analysis may be useful in the detection of sequence alteration. It is simpler and more cost-effective than the multiplex ligation-dependent probe amplification (MLPA) procedure.

  1. Anaemia and long term mortality in heart failure patients: a retrospective study

    DEFF Research Database (Denmark)

    Charlot, Mette; Torp-Pedersen, Christian; Valeur, Nana

    2010-01-01

    Anaemia has been demonstrated as a risk factor in patients with heart failure over periods of a few years, but long term data are not available. We examined the long-term risk of anaemia in heart failure patients during 15 years of follow-up.......Anaemia has been demonstrated as a risk factor in patients with heart failure over periods of a few years, but long term data are not available. We examined the long-term risk of anaemia in heart failure patients during 15 years of follow-up....

  2. Autoimmune hemolytic anemia in a patient with Malaria

    Directory of Open Access Journals (Sweden)

    Rajesh Sonani

    2013-01-01

    Full Text Available Autoimmune Hemolytic Anemia (AIHA, a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct were high. This patient′s blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT, antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2 nd day of starting treatment. Hb was raised to 6.1 gm% on 4 th day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this.

  3. Prevalence and risk factors of anaemia in paediatric patients in ...

    African Journals Online (AJOL)

    milestones. Anaemia was not associated with ... contrast to expectation, as the region has seen an overall reduction in malaria burden. .... M. Epidemiological factors that promote the development of severe malaria anaemia in children in ...

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

    Science.gov (United States)

    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.

  5. The prevalence of iron deficiency anaemia in patients undergoing bariatric surgery.

    Science.gov (United States)

    Khanbhai, M; Dubb, S; Patel, K; Ahmed, A; Richards, T

    2015-01-01

    As bariatric surgery rates continue to climb, anaemia will become an increasing concern. We assessed the prevalence of anaemia and length of hospital stay in patients undergoing bariatric surgery. Prospective data (anaemia [haemoglobin bariatric surgery. Results from a prospective database of 1530 patients undergoing elective general surgery were used as a baseline. Fifty-seven patients (14%) were anaemic pre-operatively, of which 98% were females. Median MCV (fL) and overall median ferritin (μg/L) was lower in anaemic patients (83 vs. 86, p=0.001) and (28 vs. 61, psurgery patients, prevalence of anaemia was similar (14% vs. 16%) but absolute iron deficiency was more common in those undergoing bariatric surgery; microcytosis pbariatric surgery. In bariatric patients with anaemia there was an overall increased length of hospital stay. Copyright © 2013 Asian Oceanian Association for the Study of Obesity. Published by Elsevier Ltd. All rights reserved.

  6. Hemolytic anemia

    Science.gov (United States)

    Anemia - hemolytic ... bones that helps form all blood cells. Hemolytic anemia occurs when the bone marrow isn't making ... destroyed. There are several possible causes of hemolytic anemia. Red blood cells may be destroyed due to: ...

  7. The yield of colorectal cancer among fast track patients with normocytic and microcytic anaemia.

    Science.gov (United States)

    Panagiotopoulou, I G; Fitzrol, D; Parker, R A; Kuzhively, J; Luscombe, N; Wells, A D; Menon, M; Bajwa, F M; Watson, M A

    2014-05-01

    We receive fast track referrals on the basis of iron deficiency anaemia (IDA) for patients with normocytic anaemia or for patients with no iron studies. This study examined the yield of colorectal cancer (CRC) among fast track patients to ascertain whether awaiting confirmation of IDA is necessary prior to performing bowel investigations. A review was undertaken of 321 and 930 consecutive fast track referrals from Centre A and Centre B respectively. Contingency tables were analysed using Fisher's exact test. Logistic regression analyses were performed to investigate significant predictors of CRC. Overall, 229 patients were included from Centre A and 689 from Centre B. The odds ratio for microcytic anaemia versus normocytic anaemia in the outcome of CRC was 1.3 (95% confidence interval [CI]: 0.5-3.9) for Centre A and 1.6 (95% CI: 0.8-3.3) for Centre B. In a logistic regression analysis (Centre B only), no significant difference in CRC rates was seen between microcytic and normocytic anaemia (adjusted odds ratio: 1.9, 95% CI: 0.9-3.9). There was no statistically significant difference in the yield of CRC between microcytic and normocytic anaemia (p=0.515, Fisher's exact test) in patients with anaemia only and no colorectal symptoms. Finally, CRC cases were seen in both microcytic and normocytic groups with or without low ferritin. There is no significant difference in the yield of CRC between fast track patients with microcytic and normocytic anaemia. This study provides insufficient evidence to support awaiting confirmation of IDA in fast track patients with normocytic anaemia prior to requesting bowel investigations.

  8. Fanconi Anaemia in South African Patients with Afrikaner Ancestry

    Directory of Open Access Journals (Sweden)

    C Feben

    2017-10-01

    Full Text Available Background. Fanconi anaemia (FA is a rare genetic disorder of impaired DNA repair that results in physical and haematological consequences in affected individuals. In South Africa (SA, individuals with Afrikaner ancestry are at an increased risk of inheriting disease-causing FA mutations, owing to the three common FANCA (FA, complementation group A founder mutations present in this population subgroup. Objectives. To describe the physical phenotype of SA patients with FANCA mutations for the purpose of recommending appropriate care for affected individuals. Methods. A structured clinical examination and file-based review were used to evaluate the physical phenotype of 7 patients with compound heterozygous and homozygous FANCA founder mutations, and 1 patient with confirmed FANCA complementation analysis. Descriptive statistical analysis was used to determine the frequency of physical anomalies in Afrikaner patients and to compare the described phenotype to other FA cohorts, including a previously clinically characterised black SA FA cohort. Results. An earlier age of diagnosis of FA in Afrikaner patients, a high frequency of somatic anomalies and a higher-than-expected incidence of the VACTERL/H phenotype were noted. Conclusions. Based on our findings, recommendations for the care of FA patients with Afrikaner ancestry are made, including renal ultrasound evaluation at diagnosis and hearing screening

  9. Prevalence of anaemia among patients with heart failure at the Brazzaville University Hospital.

    Science.gov (United States)

    Ikama, Méo Stéphane; Nsitou, Bernice Mesmer; Kocko, Innocent; Mongo, Ngamami Solange; Kimbally-Kaky, Gisèle; Nkoua, Jean Louis

    2015-01-01

    Heart failure (HF) is a frequent cause of ospitalisation in cardiology. Its prognosis depends on several risk factors, one of which is anaemia. We aimed to determine the prevalence of anaemia in patients with heart failure, and evaluate its impact on their prognosis. This article describes a cross-sectional study with prospective collection of data, carried out from 1 January to 31 December 2010 in the Department of Cardiology at Brazzaville University Hospital, Congo. Patients admitted for heart failure were included. Anaemia was defined as a haemoglobin level failure, and it had a negative effect on the prognosis.

  10. Excessive apoptosis of bone marrow erythroblasts in a patient with autoimmune haemolytic anaemia with reticulocytopenia

    NARCIS (Netherlands)

    Van de Loosdrecht, AA; Blom, NR; Smit, JW; De Wolf, JTM; Vellenga, E

    We report a patient with autoimmune haemolytic anaemia (AIHA) with reticulocytopenia, who showed excessive apoptosis of erythroblasts. Ultrastructural analysis of bone marrow cells showed that 50% of erythroblasts had characteristic features of apoptosis, which was confirmed by staining with

  11. Pentazocine abuse in sickle cell anaemia patients: a report of two ...

    African Journals Online (AJOL)

    Pentazocine abuse in sickle cell anaemia patients: a report of two case vignetes. ... African Journal of Drug and Alcohol Studies ... to review guidelines for the use of analgesics in the management of bone pain crisis or other chronic pains.

  12. Relationship between dynamic infrared thermal images and blood perfusion rate of the tongue in anaemia patients

    Science.gov (United States)

    Xie, Haiwei; Zhang, Yan

    2018-03-01

    The relationship between dynamic infrared (IR) thermal images and blood perfusion rate of the tongues of anaemia patients was investigated. Blood perfusion rates at multiple locations on the tongues of 62 anaemia patients and 70 control subjects were measured. For both groups of subjects, dynamic IR thermal images were also recorded within 16 s after the mouth opened. The results showed that the blood perfusion rates at different sites (apex, middle, left side and right side) on the tongues in anaemia patients (3.49, 3.71, 3.85 and 3.77 kg/s m-3) were significantly lower than those at the corresponding sites in control subjects (4.45, 4.66, 4.81 and 4.70 kg/s m-3). After the mouth opened, the tongue temperature decreased more rapidly in anaemia patients than in control subjects. To analyse the heat transfer mechanism, a transient heat transfer model of the tongue was developed. The tongue temperatures in anaemia patients and control subjects were calculated using this model and compared to the tongue temperatures measured by the IR thermal imager. The relationship between the tongue surface temperature and the tongue blood perfusion rate was analysed. The simulation results indicated that the low blood perfusion rate and the correlated changes in anaemia patients can cause faster temperature decreases of the tongue surface.

  13. Hemolytic crisis

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/003270.htm Hemolytic crisis To use the sharing features on this page, please enable JavaScript. Hemolytic crisis occurs when large numbers of red blood cells ...

  14. Hemolytic Anemia

    Science.gov (United States)

    ... worsen your condition or lead to complications. Hemolytic Anemia and Children Parents of children who have hemolytic anemia usually ... members, friends, and your child's classmates about hemolytic anemia. You also may want to tell your child's teachers or other caregivers about the condition. Let ...

  15. Hemolytic anemia in two patients with glioblastoma multiforme: A possible interaction between vorinostat and dapsone.

    Science.gov (United States)

    Lewis, Jennifer A; Petty, William J; Harmon, Michele; Peacock, James E; Valente, Kari; Owen, John; Pirmohamed, Munir; Lesser, Glenn J

    2015-06-01

    Patients undergoing treatment for glioblastoma multiforme are routinely placed on prophylactic treatment for Pneumocystis jirovecii pneumonia because of significant therapy-induced lymphopenia. In patients with sulfa allergies, dapsone prophylaxis is often used due to its efficacy, long half-life, cost effectiveness, and general safety at low doses. However, dapsone may uncommonly induce a hemolytic anemia, particularly in patients deficient of glucose-6-phosphate dehydrogenase. This hemolysis is thought to be a result of oxidative stress on red blood cells induced by dapsone metabolites which produce reactive oxygen species that disrupt the red blood cell membrane and promote splenic sequestration. A single case report of dapsone-induced hemolytic anemia in a patient with glioblastoma multiforme has been reported. We present two patients with glioblastoma multiforme who developed severe hemolytic anemia shortly after initiating therapy with vorinostat, a pan-active histone deacetylase inhibitor, while on prophylactic dapsone. There are several potential mechanisms by which histone deacetylase inhibition may alter dapsone metabolism including changes in hepatic acetylation or N-glucuronidation leading to an increase in the bioavailability of dapsone's hematotoxic metabolites. In addition, vorinostat may lead to increased hemolysis through inhibition of heat shock protein-90, a chaperone protein that maintains the integrity of the red blood cell membrane cytoskeleton. The potential interaction between dapsone and vorinostat may have important clinical implications as more than 10 clinical trials evaluating drug combinations with vorinostat in patients with malignant glioma are either ongoing or planned in North America. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

  16. Fanconi anaemia in South African patients with Afrikaner ancestry ...

    African Journals Online (AJOL)

    Background. Fanconi anaemia (FA) is a rare genetic disorder of impaired DNA repair that results in physical and haematological consequences in affected individuals. In South Africa (SA), individuals with Afrikaner ancestry are at an increased risk of inheriting disease-causing FA mutations, owing to the three common ...

  17. The impact of anaemia on treatment outcome in patients with squamous cell carcinoma of anal canal and anal margin

    Directory of Open Access Journals (Sweden)

    Oblak Irena

    2016-03-01

    Full Text Available Radiochemotherapy is the main treatment for patients with squamous cell carcinoma of the anal canal. Anaemia is reported to have adverse effect on survival in cancer patients. The aim of the study was to evaluate the influence of anaemia on radiochemotherapy treatment outcome in patients with squamous cell carcinoma of the anal canal.

  18. Frequency of anaemia an d renal insufficiency in patients with heart failure

    International Nuclear Information System (INIS)

    Khan, M.; Jehangir, W.; Daood, M.S.; Khan, A.; Mallick, N.H.

    2010-01-01

    Background: Heart Failure (HF) is a common disease with a high mortality rate. Anaemia and renal failure (RF) are often present in patients with HF and associated with worse prognosis. Objective of study was to evaluate the prevalence of anaemia and RF in patients with HF. Methods: Patients admitted in Punjab institute of cardiology Lahore with diagnosis of heart failure were enrolled from February, 2008 to December, 2008. Anaemia was defined as haemoglobin levels <13 mg/dl for men and 12 mg/dl for women. Renal function was assessed by the glomerular filtration rate (GFR), calculated by the simplified formula of the MDRD (Modification of Diet in Renal Disease) study. Results : Of the 276 patients included in this study, 42.03% (116) had anaemia and 38.40% (106) had moderate to severe renal failure (GFR <60 ml/min). Conclusion: The prevalence of anaemia and renal failure was high in this population and was associated with the severity of the HF (functional classes III and IV). (author)

  19. Normal cellular radiosensitivity in an adult Fanconi anaemia patient with marked clinical radiosensitivity

    International Nuclear Information System (INIS)

    Marcou, Yiola; D'Andrea, Andrew; Jeggo, Penelope A.; Plowman, Piers N.

    2001-01-01

    Background: Fanconi anaemia is a rare disease associated with cellular sensitivity to chemicals (e.g. mitomycin C and diepoxybutane); variable but mild cellular radiosensitivity has also been reported. Materials and methods: A 32-year-old patient with Fanconi anaemia and tonsillar carcinoma, treated by radiotherapy, was found to exhibit profound clinical radiosensitivity. Confluent, ulcerating oropharyngeal mucositis developed after a conventionally fractionated dose of 34 Gy and healing was incomplete by 2 months after cessation of therapy. Results: Cellular radiosensitivity assays and RPLD studies from this patient did not suggest any major detectable radiosensitivity. Conclusion: There is a discrepancy between the observed clinical radiosensitivity and the usual 'predictive' radiosensitivity assays in this patient with Fanconi anaemia

  20. Subclinical anaemia of chronic disease in adult patients with cystic fibrosis.

    LENUS (Irish Health Repository)

    O'connor, T M

    2012-02-03

    Patients with chronic hypoxaemia develop secondary polycythaemia that improves oxygen-carrying capacity. Therefore, normal haemoglobin and haematocrit values in the presence of chronic arterial hypoxaemia in cystic fibrosis constitute \\'relative anaemia\\'. We sought to determine the cause of this relative anaemia in patients with cystic fibrosis. We studied haematological indices and oxygen saturation in healthy volunteers (n=17) and in adult patients with cystic fibrosis (n=15). Patients with cystic fibrosis had lower resting arterial oxygen saturation when compared with normal volunteers (P<0.0001), and exercise led to a greater reduction in arterial oxygen saturation (P<0.0001). However, haemoglobin and haematocrit values in patients with cystic fibrosis did not significantly differ from normal volunteers. Serum iron (P=0.002), transferrin (P=0.02), and total iron-binding capacity (P=0.01) were lower in patients with cystic fibrosis. There were no significant differences in serum ferritin, percentage iron saturation, serum erythropoietin or red cell volume between the groups. The data presented demonstrate a characteristic picture of anaemia of chronic disease in adult patients with cystic fibrosis, except for normal haemoglobin and haematocrit values. Normal haemoglobin and haematocrit values in patients with cystic fibrosis appear to represent a combination of the effects of arterial hypoxaemia promoting polycythaemia, counterbalanced by chronic inflammation promoting anaemia of chronic disease.

  1. Anaemia and Iron Homeostasis in a Cohort of HIV-Infected Patients: A Cross-Sectional Study in Ghana

    Directory of Open Access Journals (Sweden)

    Christian Obirikorang

    2016-01-01

    Full Text Available Aim. We determined the prevalence of anaemia and evaluated markers of iron homeostasis in a cohort of HIV patients. Methods. A comparative cross-sectional study on 319 participants was carried out at the Tamale Teaching Hospital from July 2013 to December 2013, 219 patients on HAART (designated On-HAART and 100 HAART-naive patients. Data gathered include sociodemography, clinical history, and selected laboratory assays. Results. Prevalence of anaemia was 23.8%. On-HAART participants had higher CD4/CD3 lymphocyte counts, Hb, HCT/PCV, MCV, MCH, iron, ferritin, and TSAT (P<0.05. Hb, iron, ferritin, and TSAT decreased from grade 1 to grade 3 anaemia and CD4/CD3 lymphocyte count was lowest in grade 3 anaemia (P<0.05. Iron (P=0.0072 decreased with disease severity whilst transferrin (P=0.0143 and TIBC (P=0.0143 increased with disease severity. Seventy-six (23.8% participants fulfilled the criteria for anaemia, 86 (26.9% for iron deficiency, 41 (12.8% for iron deficiency anaemia, and 17 (5.3% for iron overload. The frequency of anaemia was higher amongst participants not on HAART (OR 2.6 for grade 1 anaemia; OR 3.0 for grade 3 anaemia. Conclusion. In this study population, HIV-associated anaemia is common and is related to HAART status and disease progression. HIV itself is the most important cause of anaemia and treatment of HIV should be a priority compared to iron supplementation.

  2. Splenic Size in Sickle Cell Anaemia Patients in A Tertiary Hospital ...

    African Journals Online (AJOL)

    Sickle cell disease is one of the common haemoglobinopathies in the world. It can affect any organ in the body and one of the most common and an early organ to be affected in SCA is the spleen. Reports have shown that patients with sickle cell anaemia (HbSS) have an increased susceptibility to infection leading to ...

  3. Gene expression profiles associated with anaemia and ITPA genotypes in patients with chronic hepatitis C (CH-C).

    Science.gov (United States)

    Birerdinc, A; Estep, M; Afendy, A; Stepanova, M; Younossi, I; Baranova, A; Younossi, Z M

    2012-06-01

    Anaemia is a common side effect of ribavirin (RBV) which is used for the treatment of hepatitis C. Inosine triphosphatase gene polymorphism (C to A) protects against RBV-induced anaemia. The aim of our study was to genotype patients for inosine triphosphatase gene polymorphism rs1127354 SNP (CC or CA) and associate treatment-induced anaemia with gene expression profile and genotypes. We used 67 hepatitis C patients with available gene expression, clinical, laboratory data and whole-blood samples. Whole blood was used to determine inosine triphosphatase gene polymorphism rs1127354 genotypes (CC or CA). The cohort with inosine triphosphatase gene polymorphism CA genotype revealed a distinct pattern of protection against anaemia and a lower drop in haemoglobin. A variation in the propensity of CC carriers to develop anaemia prompted us to look for additional predictors of anaemia during pegylated interferon (PEG-IFN) and RBV. Pretreatment blood samples of patients receiving a full course of PEG-IFN and RBV were used to assess expression of 153 genes previously implicated in host response to viral infections. The gene expression data were analysed according to presence of anaemia and inosine triphosphatase gene polymorphism genotypes. Thirty-six genes were associated with treatment-related anaemia, six of which are involved in the response to hypoxia pathway (HIF1A, AIF1, RHOC, PTEN, LCK and PDGFB). There was a substantial overlap between sustained virological response (SVR)-predicting and anaemia-related genes; however, of the nine JAK-STAT pathway-related genes associated with SVR, none were implicated in anaemia. These observations exclude the direct involvement of antiviral response in the development of anaemia associated with PEG-IFN and RBV treatment, whereas another, distinct component within the SVR-associated gene expression response may predict anaemia. We have identified baseline gene expression signatures associated with RBV-induced anaemia and identified

  4. A comprehensive strategy for the subtyping of patients with Fanconi anaemia: conclusions from the Spanish Fanconi Anemia Research Network.

    Science.gov (United States)

    Antonio Casado, José; Callén, Elsa; Jacome, Ariana; Río, Paula; Castella, Maria; Lobitz, Stephan; Ferro, Teresa; Muñoz, Arturo; Sevilla, Julián; Cantalejo, Angeles; Cela, Elena; Cervera, José; Sánchez-Calero, Jesús; Badell, Isabel; Estella, Jesús; Dasí, Angeles; Olivé, Teresa; José Ortega, Juan; Rodriguez-Villa, Antonia; Tapia, María; Molinés, Antonio; Madero, Luis; Segovia, José C; Neveling, Kornelia; Kalb, Reinhard; Schindler, Detlev; Hanenberg, Helmut; Surrallés, Jordi; Bueren, Juan A

    2007-04-01

    Fanconi anaemia is a heterogeneous genetic disease, where 12 complementation groups have been already described. Identifying the complementation group in patients with Fanconi anaemia constitutes a direct procedure to confirm the diagnosis of the disease and is required for the recruitment of these patients in gene therapy trials. To determine the subtype of Fanconi anaemia patients in Spain, a Mediterranean country with a relatively high population (23%) of Fanconi anaemia patients belonging to the gypsy race. Most patients could be subtyped by retroviral complementation approaches in peripheral blood T cells, although some mosaic patients were subtyped in cultured skin fibroblasts. Other approaches, mainly based on western blot analysis and generation of nuclear RAD51 and FANCJ foci, were required for the subtyping of a minor number of patients. From a total of 125 patients included in the Registry of Fanconi Anaemia, samples from 102 patients were available for subtyping analyses. In 89 cases the subtype could be determined and in 8 cases exclusions of common complementation groups were made. Compared with other international studies, a skewed distribution of complementation groups was observed in Spain, where 80% of the families belonged to the Fanconi anaemia group A (FA-A) complementation group. The high proportion of gypsy patients, all of them FA-A, and the absence of patients with FA-C account for this characteristic distribution of complementation groups.

  5. Iron deficiency and preoperative anaemia in patients scheduled for elective hip- and knee arthroplasty - an observational study

    DEFF Research Database (Denmark)

    Jans, Ø; Nielsen, C S; Khan, N

    2018-01-01

    BACKGROUND AND OBJECTIVES: Preoperative anaemia is prevalent in elderly patients scheduled for major orthopaedic surgery and is associated with increased transfusion risk and postoperative morbidity. New guidelines recommend preoperative correction of anaemia and iron deficiency in all patients......-five (10·8%) and 243 (27·6%) of the study population were WHO anaemic or had a Hb Transfusion was more common in anaemic vs. non-anaemic patients 43 vs. 13%; (P 13 g/dl 28 vs. 11% (P

  6. Optimal and continuous anaemia control in a cohort of dialysis patients in Switzerland

    Directory of Open Access Journals (Sweden)

    Kiss Denes

    2008-12-01

    Full Text Available Abstract Background Guidelines for the management of anaemia in patients with chronic kidney disease (CKD recommend a minimal haemoglobin (Hb target of 11 g/dL. Recent surveys indicate that this requirement is not met in many patients in Europe. In most studies, Hb is only assessed over a short-term period. The aim of this study was to examine the control of anaemia over a continuous long-term period in Switzerland. Methods A prospective multi-centre observational study was conducted in dialysed patients treated with recombinant human epoetin (EPO beta, over a one-year follow-up period, with monthly assessments of anaemia parameters. Results Three hundred and fifty patients from 27 centres, representing 14% of the dialysis population in Switzerland, were included. Mean Hb was 11.9 ± 1.0 g/dL, and remained stable over time. Eighty-five % of the patients achieved mean Hb ≥ 11 g/dL. Mean EPO dose was 155 ± 118 IU/kg/week, being delivered mostly by subcutaneous route (64–71%. Mean serum ferritin and transferrin saturation were 435 ± 253 μg/L and 30 ± 11%, respectively. At month 12, adequate iron stores were found in 72.5% of patients, whereas absolute and functional iron deficiencies were observed in only 5.1% and 17.8%, respectively. Multivariate analysis showed that diabetes unexpectedly influenced Hb towards higher levels (12.1 ± 0.9 g/dL; p = 0.02. One year survival was significantly higher in patients with Hb ≥ 11 g/dL than in those with Hb Conclusion In comparison to European studies of reference, this survey shows a remarkable and continuous control of anaemia in Swiss dialysis centres. These results were reached through moderately high EPO doses, mostly given subcutaneously, and careful iron therapy management.

  7. Laboratory diagnosis of the rare anaemias: external quality assessment benefits patient care

    Directory of Open Access Journals (Sweden)

    Barbara De La Salle

    2013-03-01

    Full Text Available Since its introduction in the 1960s, external quality assessment has developed to become an essential component of the quality management system of the diagnostic laboratory. External quality assessment provides a long term, retrospective view of laboratory performance, demonstrating the competence of the laboratory to others. The ENERCA project (the European Network for Rare and Congenital Anaemias has established a list of core laboratory tests that are used in the diagnosis of rare and congenital anaemias, which has been used as the basis for questionnaires to laboratories, to establish the use and quality assurance of diagnostic testing in the congenital and rare anaemias, and to European EQA providers for services in this key area. In general, the provision of EQA for rare and congenital anaemias is widely variable with little provision for the very rare disorders. For the more common congenital anaemias, such as the haemoglobinopathies and thalassaemias, provision is better but there is variation in aspects of the scheme design, especially the frequency of distribution. Where laboratories did not take part in EQA for individual tests, or there was no EQA available, a desire to participate was expressed in 66% (102/154 of cases. The provision of external quality assessment (EQA services for rare disorders is a challenge. For many of these conditions, the number of patients in any one member state is very small with only a few laboratories providing diagnostic testing. In these cases, the development of pan-European or cross-border EQA may be the only means by which standardisation of methods and results can be achieved. An EQA survey of 243 laboratories for performance in Hb A2 quantification showed encouraging results in that there was a clear differentiation in the results from a beta Thalassaemia carrier and an individual with no evidence of Thalassaemia; however, a bias was observed between different methods of measurement.

  8. The impact of renal insufficiency and anaemia on survival in patients with cardiovascular disease: a cohort study.

    LENUS (Irish Health Repository)

    Anderson, Jocelyn

    2009-01-01

    BACKGROUND: The simultaneous occurrence of cardiovascular disease (CVD), kidney disease, and anaemia is associated with increased morbidity and mortality. In the community setting, little data exists about the risk associated with milder levels of anaemia when it is present concurrently with CVD and chronic kidney disease (CKD). The aim of this study was to establish the prevalence of CKD and anaemia in patients with CVD in the community and to examine whether the presence of anaemia was associated with increased morbidity and mortality. METHODS: This study was designed as a retrospective cohort study and involved a random sample of 35 general practices in the West of Ireland. A practice-based sample of 1,609 patients with established cardiovascular disease was generated in 2000\\/2001 and followed for five years. The primary endpoint was death from any cause. Statistical analysis involved using one-way ANOVA and Chi-squared tests for baseline data and Cox proportional-hazards models for mortality data. RESULTS: Of the study sample of 617 patients with blood results, 33% (n = 203) had CKD while 6% (n = 37) had CKD and anaemia. The estimated risk of death from any cause, when compared to patients with cardiovascular disease only, was almost double (HR = 1.98, 95% CI 0.99 to 3.98) for patients with both CVD and CKD and was over 4 times greater (HR = 4.33, 95% CI 1.76 to 10.68) for patients with CVD, CKD and anaemia. CONCLUSION: In patients with cardiovascular disease in the community, chronic kidney disease and anaemia occur commonly. The presence of chronic kidney disease carries an increased mortality risk which increases in an additive way with the addition of anaemia. These results suggest that early primary care diagnosis and management of this high risk group may be worthwhile.

  9. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients

    OpenAIRE

    Lara Primo; Harvey Danielle; Levandovsky Mark; Wun Ted

    2008-01-01

    Abstract Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS) are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for imp...

  10. Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.

    Science.gov (United States)

    Fabron, A; Moreira, G; Bordin, J O

    1999-01-07

    Patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA. A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N+s+, Le(a+b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.

  11. Megadose Methylprednisolone (MDMP Treatment in a Patient with Autoimmune Hemolytic Anemia (AIHA Resistant to Conventional Corticosteroid Administration: A Case Report

    Directory of Open Access Journals (Sweden)

    Şinasi Özsoylu

    2013-06-01

    Full Text Available A female in the Netherlands with severe autoimmune hemolytic anemia (AIHA was treated with conventional corticosteroid (2 mg/kg/d in divided doses and blood transfusions for 18 months without improvement. The presented patient responded to megadose methylprednisolone (MDMP 30 mg/kg/d for 3 d, followed by 20 mg/kg for 4 d, and subsequently 10, 5, 2, and 1 mg/kg/d each for 1 week.

  12. Association of extraintestinal manifestations and anaemia with disease outcomes in patients with inflammatory bowel disease.

    Science.gov (United States)

    Vegh, Zsuzsanna; Kurti, Zsuzsanna; Gonczi, Lorant; Golovics, Petra Anna; Lovasz, Barbara Dorottya; Szita, Istvan; Balogh, Mihaly; Pandur, Tunde; Vavricka, Stephan R; Rogler, Gerhard; Lakatos, Laszlo; Lakatos, Peter Laszlo

    2016-07-01

    The association between extraintestinal manifestations (EIMs) and disease activity suggest a common pathogenetic link with inflammatory bowel disease (IBD). We report on the association of EIMs and anaemia with long-term disease outcomes, including treatment steps, hospitalization, and surgery in the prospective population-based IBD inception cohort from Veszprem province. Data of 678 incident IBD patients (Crohn's disease/ulcerative colitis(CD/UC): 331/347) diagnosed from 1st January 2000 to 31st December 2012 were analyzed (CD: m/f: 176/155, median age at diagnosis: 28, IQR: 21-40 years, disease duration: 6, IQR: 2-9 years; UC: m/f: 200/147, median age at diagnosis: 36, IQR: 26-50 years, duration: 7, IQR: 4-10 years). EIMs were present in 30% of the CD and 17.3% of the UC patients. In CD, female gender (p = 0.02) need for steroid (p  < 0.001) and azathioprine (AZA) (p = 0.02), while in UC, young age at onset (p = 0.03), extensive disease (p = 0.003), female gender (p = 0.07), need for steroids (p < 0.001) and AZA (p = 0.004) and need for IBD-related hospitalization (p = 0.01) were associated with the presence of EIMs. Anaemia was present in 56.7% of the CD and 30.2% of the UC patients. In both CD and UC anaemia was associated with age at onset (pCD = 0.001, pUC = 0.04), disease location/extent (pCD = 0.02, pUC < 0.001), steroid and AZA use (for both pCD,UC < 0.001), need for surgery/colectomy (pCD < 0.001, pUC = 0.002) and hospitalization (pCD = 0.004, pUC < 0.001) and in CD, it was associated with anti TNF therapy(p = 0.002). The presence of EIMs was associated with disease phenotype in UC and with treatment strategy in both CD and UC. Additionally, anaemia was associated with hospitalization and surgery in both CD and UC, suggesting that EIMs and anaemia may be helpful in stratifying disease severity in IBD.

  13. Hemolytic anemia following high dose intravenous immunoglobulin in patients with chronic neurological disorders

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Christiansen, I; Harbo, Thomas

    2014-01-01

    High dose intravenous immunoglobulin (IVIG) is an established treatment for various neuromuscular disorders. Recently, cases of hemolytic anemia following IVIG have been observed. The objective of this study was to determine the extent of anemia and hemolysis after IVIG and its relationship...

  14. Resolution of anaemia in a cohort of HIV-infected patients with a high prevalence and incidence of tuberculosis receiving antiretroviral therapy in South Africa

    NARCIS (Netherlands)

    Kerkhoff, Andrew D.; Wood, Robin; Cobelens, Frank G.; Gupta-Wright, Ankur; Bekker, Linda-Gail; Lawn, Stephen D.

    2014-01-01

    Background: Anaemia is frequently associated with both HIV-infection and HIV-related tuberculosis (TB) in antiretroviral therapy (ART)-naive patients in sub-Saharan Africa and is strongly associated with poor prognosis. However, the effect of ART on the resolution of anaemia in patient cohorts with

  15. Fanconi's anaemia and anaesthesia

    African Journals Online (AJOL)

    Adele

    endocrinopathies such as growth hormone deficiency and hy- ... International Fanconi Anaemia Registry of 388 patients they calculated ... transplantation), renal ultrasound, hearing tests and cardiac ... oral androgens enhance erythropoietin production and in- crease bone marrow cellularity. Cytokine therapy can improve.

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

    Science.gov (United States)

    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.

  17. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia

    DEFF Research Database (Denmark)

    Birgens, Henrik Sverre; Frederiksen, Henrik; Hasselbalch, Hans C

    2013-01-01

    The impact of first-line treatment with the anti-CD 20 chimeric monoclonal antibody rituximab in patients with warm-antibody reactive autoimmune haemolytic anaemia (WAIHA) is unknown. We report the first randomized study of 64 patients with newly diagnosed WAIHA who received prednisolone and ritu...

  18. Intravenous versus oral iron supplementation for correction of post-transplant anaemia in renal transplant patients

    Directory of Open Access Journals (Sweden)

    Mudge David W

    2009-06-01

    Full Text Available Abstract Background Post-transplant anaemia remains a common problem after kidney transplantation, with an incidence ranging from nearly 80% at day 0 to about 25% at 1 year. It has been associated with poor graft outcome, and recently has also been shown to be associated with increased mortality. Our transplant unit routinely administers oral iron supplements to renal transplant recipients but this is frequently accompanied by side effects, mainly gastrointestinal intolerance. Intravenous iron is frequently administered to dialysis patients and we sought to investigate this mode of administration in transplant recipients after noticing less anaemia in several patients who had received intravenous iron just prior to being called in for transplantation. Methods This study is a single-centre, prospective, open-label, randomised, controlled trial of oral versus intravenous iron supplements in renal transplant recipients and aims to recruit approximately 100 patients over a 12-month period. Patients will be randomised to receive a single dose of 500 mg iron polymaltose (intravenous iron group or 2 ferrous sulphate slow-release tablets daily (oral iron group. The primary outcome is time to normalisation of haemoglobin post-transplant. Prospective power calculations have indicated that a minimum of 48 patients in each group would have to be followed up for 3 months in order to have a 90% probability of detecting a halving of the time to correction of haemoglobin levels to ≥110 g/l in iron-treated patients, assuming an α of 0.05. All eligible adult patients undergoing renal transplantation at the Princess Alexandra Hospital will be offered participation in the trial. Exclusion criteria will include iron overload (transferrin saturation >50% or ferritin >800 μg/l, or previous intolerance of either oral or intravenous iron supplements. Discussion If the trial shows a reduction in the time to correction of anaemia with intravenous iron or less side

  19. The Iron Status of Sickle Cell Anaemia Patients in Ilorin, North Central Nigeria

    Directory of Open Access Journals (Sweden)

    Musa A. Sani

    2015-01-01

    Full Text Available Objectives. Sickle cell anaemia (SCA is one of the commonest genetic disorders in the world. It is characterized by anaemia, periodic attacks of thrombotic pain, and chronic systemic organ damage. Recent studies have suggested that individuals with SCA especially from developing countries are more likely to be iron deficient rather than have iron overload. The study aims to determine the iron status of SCA patients in Ilorin, Nigeria. Methods. A cross-sectional study of 45 SCA patients in steady state and 45 non-SCA controls was undertaken. FBC, blood film, sFC, sTfR, and sTfR/log sFC index were done on all subjects. Results. The mean patients’ serum ferritin (589.33 ± 427.61 ng/mL was significantly higher than the mean serum ferritin of the controls (184.53 ± 119.74 ng/mL. The mean serum transferrin receptor of the patients (4.24 ± 0.17 μg/mL was higher than that of the controls (3.96 ± 0.17 μg/mL (p=0.290. The mean serum transferrin receptor (sTfR/log serum ferritin index of the patients (1.65 ± 0.27 μg/mL was significantly lower than that of the control (1.82 ± 0.18 μg/mL (p=0.031. Conclusion. Iron deficiency is uncommon in SCA patients and periodic monitoring of the haematological, biochemical, and clinical features for iron status in SCA patients is advised.

  20. Association between preoperative anaemia with length of hospital stay among patients undergoing primary total knee arthroplasty in Singapore: a single-centre retrospective study.

    Science.gov (United States)

    Abdullah, Hairil Rizal; Sim, Yilin Eileen; Hao, Ying; Lin, Geng Yu; Liew, Geoffrey Haw Chieh; Lamoureux, Ecosse L; Tan, Mann Hong

    2017-06-08

    Studies in western healthcare settings suggest that preoperative anaemia is associated with poor outcomes after elective orthopaedic surgery. We investigated the prevalence of preoperative anaemia among patients with primary unilateral total knee arthroplasty (TKA) in Singapore and its association with length of hospital stay (LOS), perioperative blood transfusion and hospital readmission rates. Retrospective cohort study performed in a tertiary academic medical centre in Singapore, involving patients who underwent primary unilateral TKA between January 2013 and June 2014. Demographics, comorbidities, preoperative haemoglobin (Hb) level, LOS and 30-day readmission data were collected. Anaemia was classified according to WHO definition. Prolonged LOS was defined as more than 6 days, which corresponds to >75th centile LOS of the data. We analysed 2394 patients. The prevalence of anaemia was 23.7%. 403 patients (16.8%) had mild anaemia and 164 patients (6.8%) had moderate to severe anaemia. Overall mean LOS was 5.4±4.8 days. Based on multivariate logistic regression, preoperative anaemia significantly increased LOS (mild anaemia, adjusted OR (aOR) 1.71, p70 years were associated with prolonged LOS. Our 30-day related readmission rate was 1.7% (42) cases. Anaemia is common among patients undergoing elective TKA in Singapore and is independently associated with prolonged LOS and increased perioperative blood transfusion. We suggest measures to correct anaemia prior to surgery, including the use of non-gender-based Hb cut-off for establishing diagnosis. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  1. Anaemia in patients with HIV-associated tuberculosis in South Africa: predictive/prognostic value, aetiologies and treatment

    NARCIS (Netherlands)

    Kerkhoff, A.D.

    2016-01-01

    Tuberculosis remains the leading cause of death among people living with HIV in sub-Saharan Africa and worldwide. The clinical presentation in such patients is often so non-specific that much tuberculosis remains unsuspected, undiagnosed and is therefore untreated prior to death. Anaemia is one of

  2. Fanconi anaemia

    Directory of Open Access Journals (Sweden)

    Masthan Saheb D

    2002-01-01

    Full Text Available A case of Fanconi anaemia in a 7 - year-old male child is reported. He was grossly anaemic with typical cutaneous pigmentary changes. Peripheral smear revealed normocytic hypochromic anaemia, leukopenia and thrombocytopenia. Abdominal ultrasonography revealed absence of right kidney.

  3. Development of gastric dysplasia in pernicious anaemia: a clinical and endoscopic follow up study of 80 patients.

    OpenAIRE

    Armbrecht, U; Stockbrügger, R W; Rode, J; Menon, G G; Cotton, P B

    1990-01-01

    The development of gastric dysplasia and neoplasia in patients with pernicious anaemia has been evaluated in a prospective clinical and endoscopic follow up study. After initial screening of 80 patients between 1978 and 1980, one patient underwent total gastrectomy for a gastric malignancy and 12 were kept under surveillance and underwent endoscopy at a mean interval of 14 months. In the remaining 67 patients further investigation was attempted six to seven years after the initial investigati...

  4. Myeloneuropathy following nitrous oxide anesthaesia in a patient with macrocytic anaemia

    Energy Technology Data Exchange (ETDEWEB)

    Sesso, R.M.C.C.; Iunes, Y.; Melo, A.C.P. [Department of Neurology, Instituto de Assistencia Medica ao Servidor Publico Estadual, Sao Paulo (Brazil)

    1999-08-01

    The neurological condition triggered by anaesthesia with nitrous oxide involves the cyanocobalamine pathway and is characterised by progressive demyelination and axonal lesions of the peripheral nerves and cervicothoracic spinal cord (posterior and anterolateral columns) giving a peripheral neuropathy and very frequently subacute combined degeneration of the spinal cord. It is possible to show these demyelinating lesions by MRI of the spine, allowing early diagnosis and follow-up. We describe a case of myeloneuropathy with onset a few hours after nitrous oxide anaesthesia in a patient with macrocytic anaemia and possible subclinical vitamin B{sub 12} deficiency and MRI evidence of a lesion of the cervical spinal cord. Neurological and haematological improvement followed cyanocobalamine replacement. (orig.) With 2 figs., 15 refs.

  5. Exfoliative Cytology in the Oral Mucosa of Patients with Fanconi Anaemia: A Morphometric Approach

    Directory of Open Access Journals (Sweden)

    Maria Angela Naval Machado

    2017-04-01

    Full Text Available Patients with Fanconi anaemia (FA are prone to develop squamous cell carcinomas at an early age. Exfoliative cytology can be used to detect changes to the health of the oral mucosa. Objective: The aim of this study was to evaluate morphometrical and morphological changes using exfoliative cytology and to analyse and quantify the proliferative activity using silver nucleolar organiser regions (AgNOR in epithelial cells of the tongue in FA patients,after haematopoietic stem cell transplantation (HSCT. Methods: Oral smears were collected from the tongues of 20 FA children and adolescents after haematopoietic stem cell transplantation (FA and 20 healthy children (C using exfoliative cytology. The smears were stained using the Papanicolaou technique and silver impregnation. The cells were morphologically analysed and the nuclear area (NA, the cytoplasmic area (CA, and the nucleusto-cytoplasm area ratio (NA/CA were calculated. Results: Mean values for the FA and C groups were: NA (71.85 and 55.21μm.; p< 0.01; CA (2127.48 and 1441.61μm.; p< 0.01; NA/CA (0.03 and 0.04; p< 0.01, respectively. A significant increase in the NA and CA for the FA group (p< 0.01 was seen, and an alteration in the NA/CA ratio. No morphological differences were found between the groups. Class I smears were predominant in both groups. No differences were found between the groups for the mean values of AgNORs per nucleus. Conclusion: This study suggests that morphological changes occurred in the oral epithelium cells of children and adolescents with Fanconi anaemia when subjected to HSCT.

  6. Risk factors for development of hemolytic uremic syndrome in a cohort of adult patients with STEC 0104:H4 infection.

    Directory of Open Access Journals (Sweden)

    Alexander Zoufaly

    Full Text Available The outbreak of Shiga toxin producing E.coli O104:H4 in northern Germany in 2011 was one of the largest worldwide and involved mainly adults. Post-diarrheal hemolytic uremic syndrome (HUS occurred in 22% of STEC positive patients. This study's aim was to assess risk factors for HUS in STEC-infected patients and to develop a score from routine hospital parameters to estimate patient risks for developing HUS. In a cohort analysis, adult patients with STEC infection were included in five participating hospitals in northern Germany between May and July 2011. Clinical data were obtained from questionnaires and medical records, laboratory data were extracted from hospitals' electronic data systems. HUS was defined as thrombocytopenia, hemolytic anemia and acute renal dysfunction. Random forests and multivariate logistic regression were used to identify risk factors for HUS and develop a score using the estimated coefficients as weights. Among 259 adults with STEC infection, vomiting (OR 3.48,95%CI 1.88-6.53, visible blood in stools (OR 3.91,95%CI1.20-16.01, age above 75 years (OR 3.27, 95%CI 1.12-9.70 and elevated leukocyte counts (OR 1.20, 95%CI 1.10-1.31, per 1000 cells/mm(3 were identified as independent risk factors for HUS. A score using these variables has an area under the ROC curve of 0.74 (95%CI 0.68-0.80. Vomiting, visible blood in stools, higher leukocyte counts, and higher age indicate increased risk for developing HUS. A score using these variables might help to identify high risk patients who potentially benefit from aggressive pre-emptive treatment to prevent or mitigate the devastating consequences of HUS.

  7. Variable pathogenicity of exon 43del (FAA) in four Fanconi anaemia patients within a consanguineous family.

    Science.gov (United States)

    Koc, A; Pronk, J C; Alikasifoglu, M; Joenje, H; Altay, C

    1999-01-01

    Four Fanconi anaemia group A (FAA) patients within two related consanguineous families are presented: the propositus (male, 13 years, transplanted at age 10), and his three cousins (one male, 8 years, and two female newborns). Assignment of the patients to FAA was based on the functional complementation analysis by somatic cell hybridization and confirmed by mutation screening showing a homozygous deletion of exon 43 (4267-4404del) in the FAA gene to be present in all four patients. The newborn patients had been diagnosed prenatally by DNA analysis. In spite of identical molecular pathology and close familial relationship the clinical phenotypes of the four patients were not concordant. Discordant symptoms included birthweight, pigmentation abnormalities, skeletal, renal and genital abnormalities, whereas microcephaly and possibly the haematological course were concordant. Differences in environmental conditions and/or genetic make-up along with chance effects during development may explain discordant phenotypes despite identical molecular pathology in these patients. However, our results do not rule out the possibility that the exon 43del mutation may have prognostic value for the haematological course of the disease.

  8. 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.

  9. Molecular analysis of the most prevalent mutations of the FANCA and FANCC genes in Brazilian patients with Fanconi anaemia

    OpenAIRE

    Rodriguez, David Enrique Aguilar; Lima, Carmen Silvia Passos; Lourenço, Gustavo Jacob; Figueiredo, Maria Estela; Carneiro, Jorge David Aivazoglu; Tone, Luiz Gonzaga; Llerena Jr., Juan Clinton; Toscano, Raquel Alves; Brandalise, Silvia; Pinto Júnior, Walter; Costa, Fernando Ferreira; Bertuzzo, Carmen Sílvia

    2005-01-01

    Fanconi anaemia (FA) is a recessive autosomal disease determined by mutations in genes of at least eleven complementation groups, with distinct distributions in different populations. As far as we know, there are no reports regarding the molecular characterisation of the disease in unselected FA patients in Brazil. OBECTIVE: This study aimed to investigate the most prevalent mutations of FANCA and FANCC genes in Brazilian patients with FA. METHODS: Genomic DNA obtained from 22 racially and et...

  10. Vitamin B12 deficiency - a major cause of megaloblastic anaemia in patients attending a tertiary care hospital

    International Nuclear Information System (INIS)

    Iqbal, S.P.; Kakepoto, G.N.; Iqbal, S.P.

    2009-01-01

    Folate and vitamin B12 deficiencies have been known to cause megaloblastic anaemia. Since the deficiencies of these two vitamins are very common in Pakistani population, it would be imperative to investigate their role in causing megaloblastic anaemia. The objective of this study was to find out the contribution of folate and vitamin B12 deficiencies in causing megaloblastic anaemia in our patient population. Methods: In this retrospective cohort study, clinical records of 220 patients (101 females and 119 males with an age range of 1 - 80 years) who presented themselves with macrocytic anaemia at the Aga Khan University Hospital were collected. Data pertaining to complete blood count and serum levels of folate and vitamin B12 were analysed. Results: The mean haemoglobin (Hb) level was 6.8 +- 0.2 gm/dl. Sixty-nine percent of the patients had severe anaemia (Hb<8 gm/dl). Mean +- SEM values of haemoglobin, serum folate and serum B12 were not significantly different between males and females (Hb 6.4 +- 0.3 gm/dl vs 6.3 +- 0.3 gm/dl; folate 6.9 +- 0.8 mu g/ml vs 7.8 +- 1 mu g/ml; B12 259 +- 65 mu g/ml vs 225 +- 45 mu g/ml, respectively). Linear regression analysis showed that serum folate was inversely related with the mean corpuscular volume (MCV, p=0.04). Spearman's correlation analysis indicated an inverse mild association between MCV and serum folate (correlation coefficient= -0.18). Folate deficiency was 43.4%, while vitamin B12 deficiency was 78.5% in these patients. Seventy-one percent of folate-deficient patients had vitamin B12 deficiency as well, while 26.1% of patients with B12 deficiency had a co-occurrence of folate deficiency. Conclusion: Vitamin B12 deficiency appears to be the major factor leading to megaloblastic anaemia in our study population. Inadequate dietary intake, over-cooking of our food and poor absorption might be contributing to high prevalence of vitamin B12 deficiency in this population. (author)

  11. Anti-HI can cause a severe delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease patients.

    Science.gov (United States)

    Ibanez, Clara; Habibi, Anoosha; Mekontso-Dessap, Armand; Chadebech, Philippe; Chami, Btissam; Bierling, Philippe; Galactéros, Frédéric; Rieux, Claire; Nataf, Joëlle; Bartolucci, Pablo; Peyrard, Thierry; Pirenne, France

    2016-07-01

    Delayed hemolytic transfusion reaction (DHTR) is a life-threatening condition in sickle cell disease (SCD) patients that is frequently complicated by hyperhemolysis. Antibodies resulting from antigen disparity between donors of European ancestry and patients of African ancestry are common, but situations involving antibodies not classically of clinical significance are also encountered. Anti-HI is generally considered to be an innocuous naturally occurring antibody. We describe two cases of hyperhemolysis with anti-HI and provide details of the reported cases. Both SCD patients were polyimmunized and belonged to blood group B. They developed anti-HI that was reactive at 37°C, after the transfusion of group O red blood cell units matched for all known and produced antibodies classically considered to be clinically significant. Both patients developed DHTR with hyperhemolysis. In the first case, a pregnant woman, a second transfusion was unavoidable and the patient died from cardiac arrest. The state of the second patient improved without the need for further transfusion. Three other cases of DHTR with anti-HI have been described in the literature in SCD patients. The two additional cases reported here definitively demonstrate that anti-HI is dangerous in SCD patients. As a result, ABO-identical matching (including A1 status) must be considered in SCD patients with anti-HI. © 2016 AABB.

  12. Resolution of anaemia in a cohort of HIV-infected patients with a high prevalence and incidence of tuberculosis receiving antiretroviral therapy in South Africa.

    Science.gov (United States)

    Kerkhoff, Andrew D; Wood, Robin; Cobelens, Frank G; Gupta-Wright, Ankur; Bekker, Linda-Gail; Lawn, Stephen D

    2014-12-21

    Anaemia is frequently associated with both HIV-infection and HIV-related tuberculosis (TB) in antiretroviral therapy (ART)-naïve patients in sub-Saharan Africa and is strongly associated with poor prognosis. However, the effect of ART on the resolution of anaemia in patient cohorts with a high prevalence and incidence of tuberculosis is incompletely defined and the impact of TB episodes on haemoglobin recovery has not previously been reported. We therefore examined these issues using data from a well-characterised cohort of patients initiating ART in South Africa. Prospectively collected clinical and haematological data were retrospectively analysed from patients receiving ART in a South African township ART service. TB diagnoses and time-updated haemoglobin concentrations, CD4 counts and HIV viral loads were recorded. Anaemia severity was classified according to WHO criteria. Multivariable logistic regression analysis was used to determine factors independently associated with anaemia after 12 months of ART. Of 1,140 patients with baseline haemoglobin levels, 814 were alive in care and had repeat values available after 12 months of ART. The majority of patients were female (73%), the median CD4 count was 104 cells/uL and 30.5% had a TB diagnosis in the first year of ART. At baseline, anaemia (any severity) was present in 574 (70.5%) patients and was moderate/severe in 346 (42.5%). After 12 months of ART, 218 (26.8%) patients had anaemia of any severity and just 67 (8.2%) patients had moderate/severe anaemia. Independent predictors of anaemia after 12 months of ART included greater severity of anaemia at baseline, time-updated erythrocyte microcytosis and receipt of an AZT-containing regimen. In contrast, prevalent and/or incident TB, gender and baseline and time-updated CD4 cell count and viral load measurements were not independent predictors. Although anaemia was very common among ART-naive patients, the anaemia resolved during the first year of ART in a

  13. Cytotoxicity and DNA damage in the neutrophils of patients with sickle cell anaemia treated with hydroxyurea

    Directory of Open Access Journals (Sweden)

    Alano Martins Pedrosa

    2014-04-01

    Full Text Available Hydroxyurea (HU is the most important advance in the treatment of sickle cell anaemia (SCA for preventing complications and improving quality of life for patients. However, some aspects of treatment with HU remain unclear, including their effect on and potential toxicity to other blood cells such as neutrophils. This study used the measurement of Lactate Dehydrogenase (LDH and Methyl ThiazolTetrazolium (MTT and the comet assay to investigate the cytotoxicity and damage index (DI of the DNA in the neutrophils of patients with SCA using HU.In the LDH and MTT assays, a cytoprotective effect was observed in the group of patients treated, as well as an absence of toxicity. When compared to patients without the treatment, the SS group (n=20, 13 women and 07 men, aged 18-69 years, and the group of healthy individuals (AA used as a control group (n=52, 28 women and 24 men, aged 19-60 years, The SSHU group (n=21, 11 women and 10 men, aged 19-63 years showed a significant reduction (p20 months, demonstrating that despite the cytoprotective effects in terms of cell viability, the use of HU can induce DNA damage in neutrophils.

  14. Beta-hemolytic Streptococcal Bacteremia

    DEFF Research Database (Denmark)

    Nielsen, Hans Ulrik; Kolmos, Hans Jørn; Frimodt-Møller, Niels

    2002-01-01

    Bacteremia with beta-hemolytic Streptococci groups A, B, C and G has a mortality rate of approximately 20%. In this study we analyzed the association of various patient risk factors with mortality. Records from 241 patients with beta-hemolytic streptococcal bacteremia were reviewed with particular...... attention to which predisposing factors were predictors of death. A logistic regression model found age, burns, immunosuppressive treatment and iatrogenic procedures prior to the infection to be significant predictors of death, with odds ratios of 1.7 (per decade), 19.7, 3.6 and 6.8, respectively...

  15. Anticardiolipin antibodies in D+ hemolytic uremic syndrome.

    NARCIS (Netherlands)

    Loo, D.M.W.M. te; Alfen-van der Velden, J. van; Onland, W.; Heuvel, L.P.W.J. van den; Monnens, L.A.H.

    2002-01-01

    The diarrhea-associated form of the hemolytic uremic syndrome (D+ HUS) is characterized by a triad of symptoms, namely thrombocytopenia, hemolytic anemia, and acute renal failure. Histopathological studies of patients with D+ HUS show microthrombi in arterioles and glomeruli of the kidney. Recently,

  16. Somatostatin and serum gastrin in normal subjects and in patients with pernicious anaemia, chronic liver and renal disease

    Energy Technology Data Exchange (ETDEWEB)

    Le Roith, D; Vinik, A I; Epstein, S; Baron, P; Olkenitzky, M N; Pimstone, B L

    1975-09-13

    The effects of somatostatin (growth hormone release inhibiting hormone) on basal gastrin were studied in patients suffering from pernicious anaemia and chronic renal and liver disease, and during sequential arginine/insulin-stimulated gastrin release in normal subjects. When basal gastrin concentrations were normal (10-50 pg/ml) in controls and in patients who were in renal and liver failure, somatostatin had no effect on gastrin levels. Raised basal gastrin levels in pernicious anaemia and in 2 cases of chronic renal disease, were significantly inhibited by somatostatin with a half-life (T-half) of 3 to 4 minutes. Arginine infusion caused an insignificant rise in serum gastrin which was unaffected by somatostatin, whereas insulin hypoglycaemia significantly stimulated gastrin release, which was inhibited by somatostatin.

  17. Impact of Pentoxifylline and Vitamin E on Ribavirin-Induced Haemolytic Anaemia in Chronic Hepatitis C Patients: An Egyptian Survey

    Directory of Open Access Journals (Sweden)

    M. Assem

    2011-01-01

    Full Text Available Background/Aim. We evaluate the impact of combined pentoxifylline and high-dose vitamins E to standard antiviral treatment on RBV-induced haemolytic anaemia. Patients and Methods. Selected 200 naïve chronic HCV patients, were randomized to receive either the standard antiviral therapy (peginterferon α-2b and RBV plus pentoxifylline (800 mg and high-dose vitamin E (1000 iu daily (combined group or received standard antiviral therapy plus placebo only (control group. They were followed up during treatment course and for 6 months posttreatment to assess the occurrence of anaemia and virological response, respectively. Results. RBV dose modification due to anaemia were significantly less in combined group (8.5 versus 21.5%. P<.05.Withdrawal, secondary to sever anemia (Hb<8.5 gm%, was recorded only in 6 (28.6% patients of the control group. Both (ETR and (SVR were significantly higher in combined group than control group by both intention-to-treat analysis (71 versus 56%, P<.05 and 66 versus 49%, P<.05 and per-protocol analysis (85.5 versus 70.9%, P<.05 and 79.5 versus 62%, P<.05. Conclusion. Pentoxifylline and vitamin E can ameliorate RBV-associated haemolysis; improve compliance and virologic clearance when combined with the standard antiviral therapy in patients with chronic hepatitis C.

  18. Resolution of alloimmunization and refractory autoimmune hemolytic anemia in a multi-transfused beta-thalassemia major patient

    Directory of Open Access Journals (Sweden)

    Joseph Philip

    2014-01-01

    Full Text Available Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA, and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg and rituximab (anti-CD20 monoclonal antibody.

  19. Efficacy of rituximab and plasmapharesis in an adult patient with antifactor H autoantibody-associated hemolytic uremic syndrome

    Science.gov (United States)

    Deville, Clemence; Garrouste, Cyril; Coppo, Paul; Evrard, Bertrand; Lautrette, Alexandre; Heng, Anne Elisabeth

    2016-01-01

    Abstract Antifactor H antibody (anti-CFHAb) is found in 6% to 25% cases of atypical hemolytic uremic syndrome (aHUS) in children, but has been only exceptionally reported in adults. There is no consensus about the best treatment for this type of aHUS. We report the case of an adult patient treated successfully with plasma exchange (PE), steroids, and rituximab. A 27-year-old Caucasian male presented to hospital with anemia, thrombocytopenia, and acute renal failure. One week earlier, he had digestive problems with diarrhea. The diagnosis of anti-CFHAb-associated aHUS (82,000 AU/mL) without CFHR gene mutations was established. He received Rituximab 375 mg/m2 (4 pulses) with PE and steroids. This treatment achieved renal and hematological remission at day (D) 31 and negative anti-CFHAb at D45 (<100 AU/mL). At D76, a fifth rituximab pulse was performed while CD19 was higher than 10/mm3. Steroids were stopped at month (M) 9. The patient has not relapsed during long-term follow-up (M39). Rituximab therapy can be considered for anti-CFHAb-associated aHUS. Monitoring of anti-CFHAb titer may help to guide maintenance therapeutic strategies including Rituximab infusion. PMID:27684863

  20. Hydroxyurea responses in clinically varied beta, HbE-beta thalassaemia and sickle cell anaemia patients of Eastern India.

    Science.gov (United States)

    Chatterjee, Tridip; Chakravarty, Amit; Chakravarty, Sudipa

    2018-05-01

    The haematological and clinical response to hydroxyurea was estimated in HbE-beta, beta thalassaemia and sickle cell anaemia patients of Eastern India, with variable clinical severity and transfusion requirement to determine whether hydroxyurea can help these patients to maintain their steady haemoglobin level without blood transfusions. Three hundred patients (189 HbE-beta thalassaemia, 95 beta thalassaemia and 16 other haemoglobinopathies including sickle cell anaemia) were selected for hydroxyurea therapy and were followed up for 48-60 months. Results suggest significant response to hydroxyurea therapy in 19 beta and 99 HbE-beta patients in the transfusion-dependent group (GR-I). All of them became transfusion-independent while on hydroxyurea therapy. The majority of responding patients were IVS1-5(G-C) in one of their alleles in HbE-beta cases (83 out of 119). Though IVS1-5(G-C) was found to be the commonest mutation in our selected patients, the mutational background of the patients does not found to have any significant correlation with the response category towards hydroxyurea as per the results observed in our study. But, the drug works pretty well in most of the transfusion-dependent patients, as these patients were withdrawn from regular blood transfusion. At the same time, partial or no response to the drug hydroxyurea was also recorded in our study.

  1. Use of CME to impact self-reported changes in the evaluation and management of anaemia in geriatric patients

    Directory of Open Access Journals (Sweden)

    Kathleen Farmer

    2015-05-01

    Full Text Available Objective. The Third US National Health and Nutrition Examination Survey (NHANES III prompted the recognition of geriatric anaemia as a public health concern since ~10% of people aged 65 years or older were anaemic. The objective of this study was to design and implement a continuing medical education (CME event that motivates and guides Primary Care Health Practitioners (PCHPs to adopt medical practices that improve outcomes among geriatric patients with anaemia by employing effective diagnostic workup. Research design and methods. A total of 4196 PCHPs participated in 11 highly interactive 75-minute live conferences conducted throughout the US from 2011 through 2013 that featured case-based interactive discussions on the workup of microcytic, normocytic, and macrocytic anaemia by a PCHP and local haematologist expert. A standardised diagnostic algorithm for geriatric anaemia was used and distributed as a handout at the live activity. A reinforcing mobile application based on this algorithm was introduced in 2012. Main outcome measures. Data from participants were gathered immediately after the event, 10–12 weeks post-event, and 1–3 years post-event. Outcomes were evaluated according to Moore's levels. Chi-squared analyses compared the proportion of respondents who committed to one or more of the five major behavioural changes over time. Results. The Chi-squared test analysed data from each of the three timelines for five medical behavioural changes. A comparison of participants’ responses showed that there was a significant increase in the proportion of responders committing to behavioural change #1, “Avoid indiscriminant use of erythropoiesis-stimulating agents” and #5, “Refer patients with unexplained mild anaemia to a haematologist” from post-event to 1–3 years (p<0.001 (see Table 2. The proportion of respondents who committed to the other three behavioural changes remained consistent over time, suggesting that actual change

  2. 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

    Directory of Open Access Journals (Sweden)

    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.

  3. Soluble transferrin receptor: a differentiating marker between iron deficiency anaemia and anaemia of chronic disorders

    International Nuclear Information System (INIS)

    Saboor, M.; Moinuddin, A.; Naureen, A.

    2012-01-01

    Background: Iron deficiency anaemia and anaemia of chronic disorders are the two major causes of microcytic and hypochromic anaemia. Many times the diagnosis of these conditions becomes difficult through conventional laboratory tests. Determination of soluble transferrin receptors is a helpful laboratory test for the differential diagnosis of these conditions. The study was conducted to evaluate the role of soluble transferrin receptors in the differential diagnosis between iron deficiency anaemia and anaemia of chronic disorders. Methods: A total of 80 blood samples were evaluated, i.e., 20 samples from normal adult male, 20 samples from normal adult female, 20 samples from iron deficiency anaemia group and 20 samples from patients with anaemia of chronic disorders. Soluble transferrin receptors were determined by ELISA technique using Quantikine IVD kit (R and D Systems). Results: There was significant difference in the levels of sTfR in iron deficiency anaemia and anaemia of chronic disorders. Statistically non-significant difference was observed between the levels of sTfR in patients with anaemia of chronic disorders as compared to normal control group. Conclusion: The sTfR determination can be used as a reliable differentiating marker in the diagnosis of iron deficiency anaemia and anaemia of chronic disorders. (author)

  4. Variations in the β-Globin genes of Sickle Cell Anaemia Patients in ...

    African Journals Online (AJOL)

    2016-07-28

    Jul 28, 2016 ... Context: Sickle Cell Anaemia (SCA) is a genetic disorder with a life-long disability .... also cause diagnostic difficulties with routine diagnostic facilities.[10] ..... doi: 10.1038/npg.els.0005005. Available at www.els.net. [Last.

  5. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS: a 24-year clinical experience with 178 patients

    Directory of Open Access Journals (Sweden)

    Lara Primo

    2008-12-01

    Full Text Available Abstract Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for important short- and long-term outcome measures. Results Overall 30-day mortality was 16% (n = 27. 171 patients (96% received plasma exchange as the principal treatment, with a mean of 8 exchanges and a mean cumulative infused volume of 42 ± 71 L of fresh frozen plasma. The rate of complete response was 65% or 55% depending on whether this was defined by a platelet count of 100,000/μl or 150,000/μl, respectively. The rate of relapse was 18%. The Clinical Severity Score did not predict for 30-day mortality or relapse. The time to complete response did not predict for relapse. Renal insufficiency at presentation was associated with a decreased risk of relapse, with each unit increase in serum creatinine associated with a 40% decreased odds of relapse. 72% of our cohort had an idiopathic TTP-sporadic HUS, while 17% had an underlying cancer, received a solid organ transplant or were treated with a mitomycin-based therapy. The estimated overall 5-year survival was 55% and was significantly better in those without serious underlying conditions. Conclusion Plasma exchange therapy produced both high response and survival rates in this large cohort of patients with TTP-HUS. The Clinical Severity Score did not predict for 30-day mortality or relapse, contrary to our previous findings. Interestingly, the presence of renal insufficiency was associated with a decreased risk of relapse. The most important predictor of mortality was the presence or absence of a serious

  6. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): a 24-year clinical experience with 178 patients

    Science.gov (United States)

    Levandovsky, Mark; Harvey, Danielle; Lara, Primo; Wun, Ted

    2008-01-01

    Background Thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome (TTP-HUS) are related and uncommon disorders with a high fatality and complication rate if untreated. Plasma exchange therapy has been shown to produce high response rates and improve survival in patients with many forms of TTP-HUS. We performed a retrospective cohort study of 178 consecutively treated patients with TTP-HUS and analyzed whether clinical or laboratory characteristics could predict for important short- and long-term outcome measures. Results Overall 30-day mortality was 16% (n = 27). 171 patients (96%) received plasma exchange as the principal treatment, with a mean of 8 exchanges and a mean cumulative infused volume of 42 ± 71 L of fresh frozen plasma. The rate of complete response was 65% or 55% depending on whether this was defined by a platelet count of 100,000/μl or 150,000/μl, respectively. The rate of relapse was 18%. The Clinical Severity Score did not predict for 30-day mortality or relapse. The time to complete response did not predict for relapse. Renal insufficiency at presentation was associated with a decreased risk of relapse, with each unit increase in serum creatinine associated with a 40% decreased odds of relapse. 72% of our cohort had an idiopathic TTP-sporadic HUS, while 17% had an underlying cancer, received a solid organ transplant or were treated with a mitomycin-based therapy. The estimated overall 5-year survival was 55% and was significantly better in those without serious underlying conditions. Conclusion Plasma exchange therapy produced both high response and survival rates in this large cohort of patients with TTP-HUS. The Clinical Severity Score did not predict for 30-day mortality or relapse, contrary to our previous findings. Interestingly, the presence of renal insufficiency was associated with a decreased risk of relapse. The most important predictor of mortality was the presence or absence of a serious underlying disorder. PMID

  7. Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala, Uganda

    Directory of Open Access Journals (Sweden)

    Mpalampa Lena

    2012-09-01

    Full Text Available Abstract Background Sickle cell anaemia (SCA is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Disease modifying drugs can increase the Hb F levels and modify the presentation of SCA. Methods This was a cross sectional study in which we determined foetal haemoglobin levels and examined the relationship between HbF levels and disease severity in SCA patients in Mulago Hospital, Kampala, Uganda. We consecutively enrolled 216 children aged 1 year to 18 years with SCA attending the Sickle Cell Clinic at Mulago Hospital whose guardians had given consent. The history included age at onset of initial symptoms and diagnosis, number of hospitalisations and blood transfusions and other complications of SCA (cardiovascular accidents, avascular hip necrosis and priapism. A detailed physical examination was performed to assess the current state and help describe the disease severity for each patient. Blood samples were drawn for HbF levels. HbF levels ≥10% was defined as high. Results Of the 216 children, (80 37% had HbF levels ≥10%. Significant correlations were observed between HbF level and several clinical parameters independent of age including age at diagnosis (p value 0.013, number of hospitalisations (p value 0.024 and transfusions (p value 0.018 since birth. Conclusion A third of the children with SCA attending the Sickle cell clinic in Mulago Hospital have high HbF levels. Higher HbF level is associated with later onset of symptoms and presentation, and less severe disease characterised by fewer hospitalisations and blood transfusions. We suggest HbF levels should be determined at initial contact for patients with SCA to

  8. The challenge of microangiopathic hemolytic anemia

    Directory of Open Access Journals (Sweden)

    Hassanain Hani Hassan

    2017-01-01

    Full Text Available Microangiopathic hemolytic anemia (MAHA is a Coomb's-negative hemolytic anemia characterized by red cell fragmentation (schistocytes. Thrombotic microangiopathy anemia, including thrombotic thrombocytopenia and hemolytic-uremic syndrome, malignant hypertension, preeclampsia are among the most common causes. We present a case of MAHA presenting with thrombocytopenia initially diagnosed as MAHA secondary to thrombotic thrombocytopenic purpura and received five sessions plasmapheresis without improvement but with worsening of anemia and thrombocytopenia. On further inquiry, glucose-6-phosphate dehydrogenase deficiency was identified, and the patient showed dramatic recovery after the trial of B12 and folate.

  9. Anaemia among HIV infected children attending care and treatment ...

    African Journals Online (AJOL)

    Introduction: Anaemia is common among HIV infected patients; causes of anaemia in these patients are multifactorial. Anemia is noted as one of important predictors of outcome in HIV infected patients. Tis study was carried out to determine the prevalence of anaemia among HIV infected children attending HIV clinic at ...

  10. Laboratory diagnosis of the rare anaemias: external quality assessment benefits patient care

    OpenAIRE

    Barbara De La Salle; Andrea Mosca; Renata Paleari; Vasileios Rapanakis; Keith Hyde

    2013-01-01

    Since its introduction in the 1960s, external quality assessment has developed to become an essential component of the quality management system of the diagnostic laboratory. External quality assessment provides a long term, retrospective view of laboratory performance, demonstrating the competence of the laboratory to others. The ENERCA project (the European Network for Rare and Congenital Anaemias) has established a list of core laboratory tests that are used in the diagnosis of rare and co...

  11. Pegylated interferon de novo-induce autoimmune haemolytic anaemia in chronic hepatitis C patient

    OpenAIRE

    Said, Ashraf; Elbahrawy, Ashraf; Alfiomy, Mohamed; Abdellah, Mohamed; Shahat, Khaled; Salah, Mohamed; Mostafa, Sadek; Elwassief, Ahmed; Aboelfotoh, Attef; Abdelhafeez, Hafez; El-Sherif, Assem

    2011-01-01

    A 55-year-old Egyptian woman with chronic hepatitis C undergoing treatment with pegylated interferon (Peg-IFN) alfa-2a plus ribavirin was referred to our hospital on November 2010 with prolonged easy fatigability and an attack of syncope; she had no prior history of autoimmune disorders or allergy. Laboratory investigations documented the presence of Peg-IFN induced autoimmune haemolytic anaemia and autoimmune thyroiditis. Intravenous γ globulin (IVGG) failed to correct the autoimmune process...

  12. Aggressive Recurrence of Oral Squamous Cell Carcinoma in a patient with Fanconi’s Anaemia (FA)

    LENUS (Irish Health Repository)

    Nolan, M.

    2017-03-01

    Fanconi’s Anaemia is a rare autosomal recessive disease for which the incidence of head and neck cancer can be increased 700-fold1. We report a case of a 31-year old Caucasian male with FA who initially presented in July 2007 with oral squamous cell carcinoma for which he received radical surgery and radiotherapy. He was disease-free until August 2015 when he presented with an extremely aggressive recurrence.

  13. The role of group A beta hemolytic streptococcal infections in patients with tic and tourett's disorders

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    Noorbakhsh S

    2010-12-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Recently, many cases diagnosed as pediatric autoimmune neuropsy-chiatric disorders associated with group A beta hemolytic streptococcus infection (PANDAS due to production of autoimmune antibodies. Object of this study was comparison the titer of antibodies against group A beta hemolytic streptococcus (ASOT, Anti-DNase B, and Anti streptokinase between children with movement disorders (tic and tourett's disorders; pediatric autoimmune psychiatric disorders and healthy control."n"nMethods: A cross sectional/ cases control study in pediatric neuropsychology ward and clinics in two referral hospitals (Rasoul & Aliasghar affiliated by IUMS had done in Tehran, Iran (2008-2010. We selected 53 children with tic disorder and 76 healthy controls (age matched children. The antibody titers (IU/ml in their area were compared and analyzed statistically. The area under ROC, sensitivity, specificity and positive predictive value of tests calculated."n"nResults: Age of cases was between 4-16 years. All antibody titers had significant difference between two groups (p<0.0001; p=0.05; p=0.002 for ASOT, Anti-DNase and Antihyaloronidase respectively. ASOT (cut off level> 200IU/ml had 75% sensitivity; 84% specificity and 80

  14. Blood thixotropy in patients with sickle cell anaemia: role of haematocrit and red blood cell rheological properties.

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    Jens Vent-Schmidt

    Full Text Available We compared the blood thixotropic/shear-thinning properties and the red blood cells' (RBC rheological properties between a group of patients with sickle cell anaemia (SS and healthy individuals (AA. Blood thixotropy was determined by measuring blood viscosity with a capillary viscometer using a "loop" protocol: the shear rate started at 1 s-1 and increased progressively to 922 s-1 and then re-decreased to the initial shear rate. Measurements were performed at native haematocrit for the two groups and at 25% and 40% haematocrit for the AA and SS individuals, respectively. RBC deformability was determined by ektacytometry and RBC aggregation properties by laser backscatter versus time. AA at native haematocrit had higher blood thixotropic index than SS at native haematocrit and AA at 25% haematocrit. At 40% haematocrit, SS had higher blood thixotropic index than AA. While RBC deformability and aggregation were lower in SS than in AA, the strength of RBC aggregates was higher in the former population. Our results showed that 1 anaemia is the main modulator of blood thixtropy and 2 the low RBC deformability and high RBC aggregates strength cause higher blood thixotropy in SS patients than in AA individuals at 40% haematocrit, which could impact blood flow in certain vascular compartments.

  15. Risk factors of post renal transplant anaemia among Sudanese patients, a study in three renal transplant centres

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    Elmusharaf Khalifa

    2011-08-01

    Full Text Available Abstract Background There is a relative lack of recent information about late post kidney transplantation anaemia (PTA, especially in the developing countries; data are scarce about the prevalence and risk factors of PTA. Sudan was a leading country in Africa and Arab world in kidney transplantation. The first kidney transplantation in Sudan was in 1973. Methods This is a cross-sectional hospital analytic study enrolling all kidney transplanted recipients following in the transplant referral clinics at Ahmed Gassim, Selma and Ibn Sina Hospitals, Khartoum/Sudan, in the period from 1/8/2010 to 1/9/2010, clinical and laboratory data were obtained from 114 patients, anaemia was defined as Hb levels of Results The study showed that 39.5% of the patients were anaemic. Univariate analysis showed that late PTA is significantly associated with not using Erythropoietin (EPO in the pre-transplant period (p = Conclusion The study concluded that late PTA is common and under recognized. Risk factors for late PTA include renal dysfunction, history of rejection, longer duration of transplantation and not using EPO in the pre-transplant period. Renal dysfunction and not using EPO in the pre-transplant period are major predictors of late PTA.

  16. Jaw Osteomyelitis as a Complication of Sickle Cell Anaemia in Three Omani Patients; Case reports and literature review

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    Hilal Al-Ismaili

    2017-03-01

    Full Text Available Sickle cell anaemia (SCA is a common haemoglobinopathy among people from the Middle East, the Afro-Caribbean region, the Mediterranean and East India. While osteomyelitis of the long bones is a welldocumented complication of SCA, there are few documented cases of SCA patients presenting with jaw osteomyelitis. We report three SCA patients with chronic jaw osteomyelitis who presented to the Department of Oral Health, Sultan Qaboos University Hospital, Muscat, Oman, between 2009 and 2013. Two of the patients had osteomyelitis of the mandible and the third had osteomyelitis of the maxilla. In addition, a brief review of the literature is presented focusing on the clinical presentation, diagnosis and management of jaw osteomyelitis among patients with SCA.

  17. A predictable but life-threatening complication of hydroxyurea in a patient with sickle cell anaemia: an experience learned from a Jehovah's Witness.

    Science.gov (United States)

    Tun, Aung Myint; Naing, Ei Ei; Tun, Nay Min; Guevara, Elizabeth

    2015-09-30

    It is well known that hydroxyurea can cause pancytopaenia secondary to bone marrow suppression, which is reversible with short-term discontinuation of the therapy. However, it is important to note that bone marrow suppressive effects caused by hydroxyurea could be easily potentiated in patients with sickle cell anaemia complicated by chronic kidney disease (CKD). We present a case of a Jehovah's Witness with sickle cell anaemia, who developed severe bone marrow suppression due to the combined effects of hydroxyurea and CKD, resulting in a prolonged recovery period after discontinuation of hydroxyurea. 2015 BMJ Publishing Group Ltd.

  18. Thrombotic Microangiopathic Hemolytic Anemia without Evidence of Hemolytic Uremic Syndrome

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    Şinasi Özsoylu

    2016-03-01

    Full Text Available In a recent issue of this journal Dr. Oymak and her colleagues presented a clinically and genetically well-studied 5-year-old boy who was seen with severe microangiopathic hemolytic anemia without laboratory findings of renal involvement despite complement factor H gene mutations [1]. Because of Yeneral’s extensive review [2] on atypical hemolytic uremic syndrome (aHUS published recently in the Turkish Journal of Hematology, I brought it to readers’ attention that more recently some authors do not use ‘aHUS’, which was historically used to distinguish heterogeneous uncharacterized syndromes from Shiga toxin-related HUS, since the term lacks both specificity and suggested causes [3]. Though in our patient with thrombotic thrombocytopenic purpura renal involvement was documented at the beginning but not in the last two recurrences, neither serum nor urinary findings indicated kidney involvement [4]. Although the discussions of Dr. Oymak et al. are well taken, the term ‘microangiopathic hemolytic anemia’ is covering the syndrome to a large extent as suggested by George and Nester

  19. Efficacy of intravenous iron in treating iron deficiency anaemia in patients with inflammatory bowel disease: Are there predictors of response?

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    Rocío Ferreiro Iglesias

    Full Text Available Introduction: in inflammatory bowel disease (IBD iron deficiency anaemia (IDA is a very common disorder. Until recently, oral iron has been the mainstay therapy, nevertheless it has been associated with intolerance and noncompliance. Therefore, the goal of our study was to evaluate the efficacy of intravenous iron in IDA in IBD patients and the secondary aim was to investigate whether other potential factors could influence in the response to the treatment. Design: an open-label, prospective, consecutive, single centre study. Material and methods: we performed our study in patients with ulcerative colitis (UC or Crohn's disease (CD with severe anaemia or intolerance with oral iron. All of them received intravenous sacarose iron and did biochemistry profile with haemoglobin (Hb. Moreover, the correlation with other variables was studied: age, sex, smoking habit, IBD type, previous surgery and type of surgery and other treatments. Response was defined as Hb increase of ≥ 2 g/dL or normalization of the levels. Results: fifty-four patients were included into the study, 34 (63% with UC y 20 (37% with CD, 18 (33.3% men and 36 women (66.6% and the average was 48 ± 14 years. The total proportion of responders was 52% (SD ± 05; 43% of the patients reached Hb ≥ 2 g/dl and y 9% of them normalized Hb. Only the utilization of 5-ASA was associated with low response to iron treatment (p < 0.05. Conclusions: our study suggests that response to intravenous iron is achievable in the majority of patients with IBD and severe IDA or intolerance treatment with oral iron. Moreover, the patients with consumption of 5-ASA could had less response to the treatment.

  20. Oral supplementation of vitamin E reduces osmotic fragility of RBC in hemolytic anemic patients with G6PD deficiency

    International Nuclear Information System (INIS)

    Sultana, N.; Begum, S.; Begum, N.; Ali, T.

    2009-01-01

    haemolytic anaemia due to G6PD deficiency in comparison to those of the healthy control. After supplementation with vitamin E starting and completing points of osmotic fragility of RBC were significantly decreased whereas, MCV, MCH, MCHC were significantly increased towards those of healthy of healthy control in supplemented group of patients in comparison to those of their pre-supplemented (day-1) and non-supplemented groups both on day 1 and day 60. From this study it may be concluded that, disturbances of some of the haematological parameter like higher osmotic fragility of RBC and lower MCV, MCH, MCHC occur in G6PD deficient haemolytic anaemic patients, which returned towards normal after supplementation of vitamin E, which clearly indicates? the role of this anti-oxidant vitamin in maintaining red cell membrane integrity and thereby decreases the rate of haemolysis in this group of patients. So, vitamin E can be supplemented along with other drugs for better management of the patients. (author)

  1. [Anaemia as a cause of haemodynamic angina in a patient with chronic ischaemic heart disease].

    Science.gov (United States)

    Miguéns Blanco, I; Bravo Amaro, M

    2014-01-01

    Ischaemic heart disease is the leading cause of mortality and morbidity and one of the primary causes of morbidity in Spain. The variability in the clinical presentation of this condition at both primary care and emergency services level requires a careful history and a thorough physical examination. In the case presented, the main symptoms of angina and dyspnea reported in the anamnesis, and the obvious pallor in the physical examination, were the key data to identify anaemia as a cause of angina. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.

  2. Intestinal parasitosis and anaemia among patients in a Health Center, North Ethiopia.

    Science.gov (United States)

    Alemu, Megbaru; Kinfe, Birhane; Tadesse, Desalegn; Mulu, Wondemagegn; Hailu, Tadesse; Yizengaw, Endalew

    2017-11-28

    The aim of this cross-sectional study was to determine the magnitude of intestinal parasitosis and anaemia in a Health Center, North Ethiopia. A total of 427 outpatients were enrolled and the median age of the participants was 22 years. The prevalence of intestinal parasitosis was 143 (33.5%). Age, place of residence and occupation were significantly associated with intestinal parasitosis. When we see parasite specific factors, significant associations were observed for source of drinking water (P = 0.02), age (P intestinal parasite -infected and non-infected participants was 10.7 and 7.0%, respectively. Study participants infected with S. stercoralis and hookworm were more likely to develop anaemia than the non- infected ones; AOR (adjusted odds ratio) = 5.3, 95% CI (1.01-27.4); P = 0.028 and AOR = 11.1, 95% CI (3.36-36.9); P = 0.000, respectively.

  3. [Aplastic anaemia associated with pregnancy].

    Science.gov (United States)

    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.

  4. Expert services for rare anaemias across Europe

    Directory of Open Access Journals (Sweden)

    Beatrice Gulbis

    2013-03-01

    Full Text Available New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One of the ENERCA partners objectives was the mapping of existing centres that take care of patients with rare anaemias in Europe. Another goal was to obtain a directory of facilities available per centre for patients with rare anaemias. We thought that with those results it could realistically help to define a consensus regarding the criteria to be recognised as a centre of expertise for haemoglobinopathies and very rare anaemias.

  5. A Coincidental Discovery of a New Stable Variant (Hb Hachioji or HBB: c.187C>T) in a Patient with Chronic Hemolytic Anemia of Unexplained Origin.

    Science.gov (United States)

    Mella, Ferania; Yamashiro, Yasuhiro; Adhiyanto, Chris; Tanaka, Tatehiko; Nitta, Takenori; Amao, Yuki; Kimoto, Masafumi

    2018-01-01

    We report a new hemoglobin (Hb) variant, Hb Hachioji (HBB: c.187C>T), which was detected in a 32-year-old male with hemolytic anemia. The proband had undergone splenectomy in his childhood after being diagnosed with hereditary spherocytosis (HS) with no clinical improvement. A recent study showed that Heinz bodies were frequently observed in his red cells, however, no abnormal band was separated by isoelectric focusing (IEF), and the isopropanol (instability) test was negative. Direct sequencing revealed that the proband was a heterozygous carrier of a novel mutation (GCT>GTT) at codon 62 of the β-globin gene, leading to an alanine to valine substitution. This variant was named Hb Hachioji. Characterization at the mRNA level by cDNA sequencing detected β Hachioji mRNA, as well as β A mRNA. Subsequently, study of the proband's family indicated that his father was a carrier of this Hb variant, although unexpectedly, the father was asymptomatic and clinically healthy. Oxygen affinity measurement of total Hb showed no alteration in the P 50 and oxygen equilibrium curve. The presence of Hb Hachioji was confirmed by mass spectrometry (MS). Hb Hachioji comprised approximately 50.0% of the total Hb and was a stable variant. The phenotypic discrepancy between these two carriers suggests that Hb Hachioji may not be associated with the hemolytic involvement in the proband. P4.2Nippon, which is the primary cause of most cases of Japanese HS, was absent in the proband's parents. The coexistence of glucose-6-phosphate dehydrogenase (G6PD) deficiency was ruled out. Thus, the cause of hemolytic involvement in this patient remains unclear.

  6. The impact of anaemia on treatment outcome in patients with squamous cell carcinoma of anal canal and anal margin.

    Science.gov (United States)

    Oblak, Irena; Cesnjevar, Monika; Anzic, Mitja; Hadzic, Jasna But; Ermenc, Ajra Secerov; Anderluh, Franc; Velenik, Vaneja; Jeromen, Ana; Korosec, Peter

    2016-03-01

    Radiochemotherapy is the main treatment for patients with squamous cell carcinoma of the anal canal. Anaemia is reported to have adverse effect on survival in cancer patients. The aim of the study was to evaluate the influence of anaemia on radiochemotherapy treatment outcome in patients with squamous cell carcinoma of the anal canal. One hundred consecutive patients with histologically confirmed squamous cell carcinoma of the anal canal were treated radically with 3-dimensional conformal or intensity-modulated radiation therapy followed by brachytherapy or external beam radiotherapy boost and with concurrent mitomycin C and 5-fluorouracil. The influence on survival of pre-treatment, mean on-treatment and end-of-treatment haemoglobin (Hb) concentrations was studied. The 5-year locoregional control, disease free survival, disease specific survival and overall survival rates for all patients were 72%, 71%, 77% and 62%, respectively. In univariate analysis, patients with pre-treatment and end-of-treatment Hb > 120 g/L survived statistically significantly better compared to patients with Hb ≤ 120 g/L. Patients with mean on-treatment Hb > 120 g/L only had statistically significant better locoregional control and overall survival than patients with Hb ≤ 120 g/L. In multivariate analysis, independent prognostic factors were pre-treatment Hb (> 120 g/L vs. ≤ 120 g/L) for overall survival (hazard ratio [HR] = 0.419, 95% confidence interval [CI] = 0.190-0.927, p = 0.032) and stage (I & II vs. III) for disease specific (HR = 3.523, 95% CI = 1.375-9.026, p = 0.009) and overall survival (HR = 2.230, 95% CI = 1.167-4.264, p = 0.015). The pre-treatment, mean on-treatment and end-of-treatment Hb concentration > 120 g/L carried better prognosis for patients for with squamous cell carcinoma of the anal canal treated with radiochemotherapy. The pre-treatment Hb > 120 g/L was an independent prognostic factor for overall survival of patients with anal canal cancer.

  7. The impact of anaemia on treatment outcome in patients with squamous cell carcinoma of anal canal and anal margin

    International Nuclear Information System (INIS)

    Oblak, Irena; Cesnjevar, Monika; Anzic, Mitja; Hadzic, Jasna But; Ermenc, Ajra Secerov; Anderluh, Franc; Velenik, Vaneja; Jeromen, Ana; Korosec, Peter

    2016-01-01

    Radiochemotherapy is the main treatment for patients with squamous cell carcinoma of the anal canal. Anaemia is reported to have adverse effect on survival in cancer patients. The aim of the study was to evaluate the influence of anaemia on radiochemotherapy treatment outcome in patients with squamous cell carcinoma of the anal canal. One hundred consecutive patients with histologically confirmed squamous cell carcinoma of the anal canal were treated radically with 3-dimensional conformal or intensity-modulated radiation therapy followed by brachytherapy or external beam radiotherapy boost and with concurrent mitomycin C and 5-fluorouracil. The influence on survival of pre-treatment, mean on-treatment and end-of-treatment haemoglobin (Hb) concentrations was studied. The 5-year locoregional control, disease free survival, disease specific survival and overall survival rates for all patients were 72%, 71%, 77% and 62%, respectively. In univariate analysis, patients with pre-treatment and end-of-treatment Hb > 120 g/L survived statistically significantly better compared to patients with Hb ≤ 120 g/L. Patients with mean on-treatment Hb > 120 g/L only had statistically significant better locoregional control and overall survival than patients with Hb ≤ 120 g/L. In multivariate analysis, independent prognostic factors were pre-treatment Hb (> 120 g/L vs. ≤ 120 g/L) for overall survival (hazard ratio [HR] = 0.419, 95% confidence interval [CI] = 0.190–0.927, p = 0.032) and stage (I & II vs. III) for disease specific (HR = 3.523, 95% CI = 1.375–9.026, p = 0.009) and overall survival (HR = 2.230, 95% CI = 1.167–4.264, p = 0.015). The pre-treatment, mean on-treatment and end-of-treatment Hb concentration > 120 g/L carried better prognosis for patients for with squamous cell carcinoma of the anal canal treated with radiochemotherapy. The pre-treatment Hb > 120 g/L was an independent prognostic factor for overall survival of patients with anal canal cancer

  8. Pegylated interferon de novo-induce autoimmune haemolytic anaemia in chronic hepatitis C patient.

    Science.gov (United States)

    Said, Ashraf; Elbahrawy, Ashraf; Alfiomy, Mohamed; Abdellah, Mohamed; Shahat, Khaled; Salah, Mohamed; Mostafa, Sadek; Elwassief, Ahmed; Aboelfotoh, Attef; Abdelhafeez, Hafez; El-Sherif, Assem

    2011-08-11

    A 55-year-old Egyptian woman with chronic hepatitis C undergoing treatment with pegylated interferon (Peg-IFN) alfa-2a plus ribavirin was referred to our hospital on November 2010 with prolonged easy fatigability and an attack of syncope; she had no prior history of autoimmune disorders or allergy. Laboratory investigations documented the presence of Peg-IFN induced autoimmune haemolytic anaemia and autoimmune thyroiditis. Intravenous γ globulin (IVGG) failed to correct the autoimmune process; on the other hand steroid therapy dramatically corrected both haematological and thyroid values, and step down the immune process. Our report indicated that Peg-IFN de novo-induce autoimmune haemolysis, documenting a previous report. IVGG failed to step down the immune process in our case.

  9. Circulating microRNAs in patients with Shiga-Toxin-producing E. coli O104:H4 induced hemolytic uremic syndrome.

    Directory of Open Access Journals (Sweden)

    Johan M Lorenzen

    Full Text Available BACKGROUND: In early May 2011, an outbreak of hemorrhagic colitis associated with hemolytic-uremic syndrome (HUS first developed in Northern Germany and spread to 15 other countries in Europe. The outbreak-strain O104:H4, which combined virulence factors of typical enteroaggregative and Shiga-Toxin-producing E. coli was associated with an unusual high rate of hemolytic uremic syndrome. Also an unexpected high rate of coma and seizures leading to mechanical ventilation and ICU treatment was observed. MicroRNAs are small ribonucleotides orchestrating gene expression. We tested whether circulating microRNAs in serum of HUS patients during the 2011 epidemics are altered in this patient cohort and related to clinical manifestations. METHODOLOGY/PRINCIPAL FINDINGS: We profiled microRNAs using RNA isolated from serum of patients and healthy age-matched controls. The results were validated in 38 patients at baseline, 29 patients during follow-up and 21 age-matched healthy controls by miRNA-specific quantitative RT-PCR. Circulating levels of miR-24, miR-126 were increased in HUS patients versus controls. There was no association between these microRNAs and renal function or the need for renal replacement therapy. In contrast, levels of miR-126 were associated with neurological symptoms at baseline and during follow-up. In addition, miR-126 (on admission and miR-24 (on admission and during follow-up were associated with platelet count. CONCLUSIONS/SIGNIFICANCE: Circulating microRNAs are strongly altered in this patient cohort and associated with neurological symptoms as well as platelet count.

  10. Development of Hemolytic Anemia in a Nivolumab-Treated Patient with Refractory Metastatic Squamous Cell Skin Cancer and Chronic Lymphatic Leukemia

    Directory of Open Access Journals (Sweden)

    K.S. Schwab

    2016-06-01

    Full Text Available Management of patients with metastatic squamous cell skin cancer, refractory to initial therapy with standard chemotherapy and radiation protocols, remains difficult with poor overall prognosis and limited therapeutic options. Recently, promising response rates with nivolumab, a programmed death receptor-1-blocking antibody, in squamous cancer of the head and neck have been demonstrated. Considering the similar histological patterns of squamous cell cancer of the skin and squamous cell cancer of the head and neck, we assumed that nivolumab could also be effective in our patients with refractory metastatic squamous cell cancer of the skin. So far, there have been no clinical data on the therapeutic efficacy of nivolumab in squamous cell skin cancer. We here present a case of a patient with metastatic squamous cell skin cancer refractory to previous therapies, who showed a good response to nivolumab over a period of 5 months, but developed a serious hemolytic crisis under nivolumab treatment after eight applications.

  11. Anaemia and thyrotoxicosis: The need to look for an alternative cause.

    Science.gov (United States)

    Sehgal, S; Tamatea, J A U; Conaglen, J V; Elston, M S

    2018-03-22

    Anaemia and thyrotoxicosis are both relatively common. It is unclear whether thyrotoxicosis results in anaemia in the absence of other causes. The aim of this study was to determine the prevalence and characteristics of anaemia in patients with thyrotoxicosis. A prospective cohort study of patients with thyrotoxicosis. 353 patients referred to a regional endocrinology centre in New Zealand from March 2013 to November 2014 for new-onset thyrotoxicosis. Detailed assessment including thyroid function tests, full blood count, inflammatory markers, haematological parameters and coeliac serology. Anaemia was defined as a haemoglobin value cause(s) for the anaemia. Only 10 patients (2.8% of the entire cohort) had anaemia not clearly attributable to another cause. Median free thyroid hormone levels were higher in those with anaemia of unknown cause compared to patients with thyrotoxicosis alone. The median duration of anaemia was shorter in patients with thyrotoxicosis-associated anaemia compared to those with anaemia due to an underlying cause (1 vs 6 months, P = .001). In all patients with thyrotoxicosis-associated anaemia, the anaemia resolved, either prior to, or on becoming euthyroid. Anaemia coexisting with thyrotoxicosis is less common than previously reported and is mild and transient. Patients with thyrotoxicosis and significant anaemia should be investigated for other potential causes, particularly when anaemia persists. © 2018 John Wiley & Sons Ltd.

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

    Science.gov (United States)

    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.

  13. Genetic diagnosis for congenital hemolytic anemia.

    Science.gov (United States)

    Ohga, Shouichi

    2016-01-01

    Congenital hemolytic anemia is a group of monogenic diseases presenting with anemia due to increased destruction of circulating erythrocytes. The etiology of inherited anemia accounts for germline mutations of the responsible genes coding for the structural components of erythrocytes and extra-erythrocytes. The erythrocyte abnormalities are classified into three major disorders of red cell membrane defects, hemoglobinopathies, and red cell enzymopathies. The extra-erythrocyte abnormalities, typified by consumption coagulopathy and intravascular hemolysis, include Upshaw-Schulman syndrome and atypical hemolytic uremic syndrome. The clinical manifestations of congenital hemolytic anemia are anemia, jaundice, cholelithiasis and splenomegaly, while the onset mode and severity are both variable. Genetic overlapping of red cell membrane protein disorders, and distinct frequency and mutation spectra differing among races make it difficult to understand this disease entity. On the other hand, genetic modifiers for the phenotype of β-globin diseases provide useful information for selecting the optimal treatment and for long-term management. Recently, next generation sequencing techniques have enabled us to determine the novel causative genes in patients with undiagnosed hemolytic anemias. We herein review the concept and strategy for genetic diagnosis of inherited hemolytic anemias.

  14. Inosine triphosphatase allele frequency and association with ribavirin-induced anaemia in Brazilian patients receiving antiviral therapy for chronic hepatitis C

    Directory of Open Access Journals (Sweden)

    Nathália Delvaux

    2015-08-01

    Full Text Available Inosine triphosphatase (ITPA single nucleotide polymorphisms (SNPs are strongly associated with protection against ribavirin (RBV-induced anaemia in European, American and Asian patients; however, there is a paucity of data for Brazilian patients. The aim of this study was to evaluate the ITPA SNP (rs7270101/rs1127354 frequency in healthy and hepatitis C virus (HCV-infected patients from Brazil and the association with the development of severe anaemia during antiviral therapy. ITPA SNPs were determined in 200 HCV infected patients and 100 healthy individuals by sequencing. Biochemical parameters and haemoglobin (Hb levels were analysed in 97 patients who underwent antiviral therapy. A combination of AArs7270101+CCrs1127354 (100% ITPase activity was observed in 236/300 individuals. Anaemia was observed in 87.5% and 86.2% of treated patients with AA (rs7270101 and CC genotypes (rs1127354, respectively. Men with AA (rs7270101 showed a considerable reduction in Hb at week 12 compared to those with AC/CC (p = 0.1475. In women, there was no influence of genotype (p = 0.5295. For rs1127354, men with the CC genotype also showed a sudden reduction in Hb compared to those with AC. Allelic distribution of rs7270101 and rs1127354 shows high rates of the genotypes AA and CC, respectively, suggesting that the study population had a great propensity for developing RBV-induced anaemia. A progressive Hb reduction during treatment was observed; however, this reduction was greater in men at week 12 than in women.

  15. [Insufficient evidence supporting iron supplementation in anaemia during pregnancy].

    Science.gov (United States)

    Wiegerinck, Melanie M; Mol, Ben Willem J

    2012-01-01

    The Royal Dutch Organization of Midwives (KNOV) recently presented their practice guideline 'Anaemia in midwifery practice'. The guideline identified available evidence on diagnosis, prognosis and treatment of anaemia in pregnancy. Anaemia based on iron deficiency and subsequent treatment with iron supplementation are probably the most frequent aspects of care for pregnant women. However, there is surprisingly enough no evidence of the efficacy of iron supplementation treatment on relevant clinical outcomes in pregnant women with anaemia. We plead to make the next guideline a multidisciplinary one. Such a guideline may lead to a large pragmatic trial evaluating the efficacy of iron supplementation treatment for patients with anaemia.

  16. Pulmonary aspergillosis and central nervous system hemorrhage as complications of autoimmune hemolytic anemia treated with corticosteroids.

    Science.gov (United States)

    Cleri, Dennis J; Moser, Robert L; Villota, Francisco J; Wang, Yue; Husain, Syed A; Nadeem, Shahzinah; Anjari, Tarek; Sajed, Mohammad

    2003-06-01

    Warm, active antibody adult autoimmune hemolytic anemia is the most common form of hemolytic anemia not related to drug therapy. Mortality in adult autoimmune hemolytic anemia is related to the inability to successfully treat patients' underlying disease, or the infectious complications of splenectomy and prolonged steroid therapy. Predisposing factors for invasive aspergillosis are neutropenia and steroid therapy. We present a fatal case of aspergillosis complicating a nonneutropenic case of warm active antibody adult autoimmune hemolytic anemia treated with prolonged steroid therapy.

  17. Hemolytic uremic syndrome after bone marrow transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Arai, Ayako; Sakamaki, Hisashi; Tanikawa, Shu [Tokyo Metropolitan Komagome Hospital (Japan)] [and others

    1998-06-01

    One hundred and thirteen patients who underwent autologous or allogeneic bone marrow transplantation (BMT) were investigated for the subsequent development of hemolytic uremic syndrome (HUS). HUS developed in seven patients (four males and three females, five acute lymphocytic leukemia (ALL), one acute myelogenous leukemia, one non-Hodgkin`s lymphoma) between 36-196 days after BMT. Four patients were recipients of autologous BMT and three were those of allogeneic BMT. Six patients were preconditioned with the regimens including fractionated total body irradiation (TBI). ALL and preconditioning regimen with TBI were suspected to be the risk factors for the development of HUS. Cyclosporin A (CSP) administration was discontinued in three patients who had been given CSP for graft-versus-host disease prophylaxis. Predonisolone was given to the three patients and plasma exchange was performed in one patient. Both hemolytic anemia and thrombocytopenia were resolved in virtually all patients, while creatinine elevation has persisted along with hypertension in one patient. (author)

  18. Molecular analysis of the most prevalent mutations of the FANCA and FANCC genes in Brazilian patients with Fanconi anaemia

    Directory of Open Access Journals (Sweden)

    David Enrique Aguilar Rodriguez

    2005-01-01

    Full Text Available Fanconi anaemia (FA is a recessive autosomal disease determined by mutations in genes of at least eleven complementation groups, with distinct distributions in different populations. As far as we know, there are no reports regarding the molecular characterisation of the disease in unselected FA patients in Brazil. OBECTIVE: This study aimed to investigate the most prevalent mutations of FANCA and FANCC genes in Brazilian patients with FA. METHODS: Genomic DNA obtained from 22 racially and ethnically diverse unrelated FA patients (mean age ± SD: 14.0 ± 7.8 years; 10 male, 12 female; 14 white, 8 black was analysed by polymerase chain reaction and restriction site assays for identification of FANCA (delta3788-3790 and FANCC (delta322G, IVS4+4A -> T, W22X, L496R, R548X, Q13X, R185X, and L554P gene mutations. RESULTS: Mutations in FANCA and FANCC genes were identified in 6 (27.3% and 14 (63.6% out of 22 patients, respectively. The disease could not be attributed to the tested mutations in the two remaining patients enrolled in the study (9.1%. The registry of the two most prevalent gene abnormalities (delta3788-3790 and IVS4 + 4 -> T revealed that they were present in 18.2% and 15.9% of the FA alleles, respectively. Additional FANCC gene mutations were found in the study, with the following prevalence: delta322G (11.4%, W22X (9.1%, Q13X (2.3%, L554P (2.3%, and R548X (2.3% of total FA alleles. CONCLUSION: These results suggest that mutations of FANCA and FANCC genes are the most prevalent mutations among FA patients in Brazil.

  19. Anaemia with inflammation responding to lenalidomide.

    Science.gov (United States)

    Haroun, Faysal; Mener, Andrew; Elkon, Jacob; Tabbara, Imad

    2016-10-13

    We present a case of a 73-year-old woman with transfusion-dependent anaemia thought to be secondary to an unidentifiable inflammatory condition. Anaemia evaluation including multiple bone marrow biopsies was unrevealing, with the exception of a non-specific elevation of her erythrocyte sedimentation rate and C-reactive protein. She had no identifiable inflammatory condition and did not meet the criteria for myelodysplastic syndrome. She was empirically treated with lenalidomide and achieved a complete response, suggesting that this immunomodulatory drug could potentially have a role in treating a subgroup of patients with immune-mediated anaemia. 2016 BMJ Publishing Group Ltd.

  20. Drug-induced immune hemolytic anemia

    Science.gov (United States)

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... Drugs that can cause this type of hemolytic anemia include: Cephalosporins (a class of antibiotics), most common ...

  1. Quantitative MRI shows cerebral microstructural damage in hemolytic-uremic syndrome patients with severe neurological symptoms but no changes in conventional MRI

    Energy Technology Data Exchange (ETDEWEB)

    Weissenborn, Karin; Worthmann, Hans; Heeren, Meike [Hannover Medical School, Clinic for Neurology, Hannover (Germany); Bueltmann, Eva; Donnerstag, Frank; Giesemann, Anja M.; Goetz, Friedrich; Lanfermann, Heinrich; Ding, Xiao-Qi [Hannover Medical School, Institute of Diagnostic and Interventional Neuroradiology, Hannover (Germany); Kielstein, Jan; Schwarz, Anke [Hannover Medical School, Clinic for Nephrology and Hypertension, Hannover (Germany)

    2013-07-15

    Severe neurological symptoms in Shiga toxin-producing Escherichia coli infection associated hemolytic-uremic syndrome (STEC-HUS) are often accompanied by none or only mild alterations of cerebral magnetic resonance imaging (MRI). This study aims to analyze if quantitative MRI is able to reveal cerebral pathological alterations invisible for conventional MRI. In nine patients with STEC-HUS associated severe neurological symptoms but inconspicuous cerebral MRI findings maps of the parameters T2 relaxation time, relative proton density (PD), apparent diffusion coefficient (ADC), and fractional anisotropy (FA) were generated. Quantitative values of these parameters were measured at the basal ganglia, thalamus, and white matter of the frontal and parietal lobe and compared to those of nine age- and sex-matched controls. Significant T2 prolongation (p < 0.01) was found in the basal ganglia of all patients compared to controls. PD and ADC were not significantly altered. A significant reduction of FA in patients was seen at caput nuclei caudati (p < 0.01). Prolonged T2 relaxation time indicates cerebral microstructural damages in these patients despite their inconspicuous MRI findings. T2 relaxometry could be used as a complementary tool for the assessment of metabolic-toxic brain syndromes. (orig.)

  2. Expert services for rare anaemias across Europe

    OpenAIRE

    Beatrice Gulbis

    2013-01-01

    New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One o...

  3. Cerebral microcirculation during experimental normovolaemic anaemia

    Directory of Open Access Journals (Sweden)

    Judith eBellapart

    2016-02-01

    Full Text Available Anaemia is accepted amongst critically ill patients as an alternative to elective blood transfusion. This practice has been extrapolated to head injury patients with only one study comparing the effects of mild anaemia on neurological outcome. There are no studies quantifying microcirculation during anaemia. Experimental studies suggest that anaemia leads to cerebral hypoxia and increased rates of infarction, but the lack of clinical equipoise when testing the cerebral effects of transfusion amongst critically injured patients, supports the need of experimental studies. The aim of this study was to quantify cerebral microcirculation and the potential presence of axonal damage in an experimental model exposed to normovolaemic anaemia, with the intention of describing possible limitations within management practices in critically ill patients. Under non-recovered anaesthesia, six Merino sheep were instrumented using an intracardiac transeptal catheter to inject coded microspheres into the left atrium to ensure systemic and non-chaotic distribution. Cytometric analyses quantified cerebral microcirculation at specific regions of the brain. Amyloid precursor protein staining was used as an indicator of axonal damage. Animals were exposed to normovolaemic anaemia by blood extractions from the indwelling arterial catheter with simultaneous fluid replacement through a venous central catheter. Simultaneous data recording from cerebral tissue oxygenation, intracranial pressure and cardiac output was monitored. A regression model was used to examine the effects of anaemia on microcirculation with a mixed model to control for repeated measures. Homogeneous and normal cerebral microcirculation with no evidence of axonal damage was present in all cerebral regions, with no temporal variability, concluding that acute normovolaemic anaemia does not result in short term effects on cerebral microcirculation in the ovine brain.

  4. Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: concentration of C3 p.I1157T mutation.

    Science.gov (United States)

    Matsumoto, Takeshi; Fan, Xinping; Ishikawa, Eiji; Ito, Masaaki; Amano, Keishirou; Toyoda, Hidemi; Komada, Yoshihiro; Ohishi, Kohshi; Katayama, Naoyuki; Yoshida, Yoko; Matsumoto, Masanori; Fujimura, Yoshihiro; Ikejiri, Makoto; Wada, Hideo; Miyata, Toshiyuki

    2014-11-01

    Atypical hemolytic uremic syndrome (aHUS) is caused by abnormalities of the complement system and has a significantly poor prognosis. The clinical phenotypes of 12 patients in nine families with aHUS with familial or recurrent onset and ADAMTS13 activity of ≥20 % treated at the Mie University Hospital were examined. In seven of the patients, the first episode of aHUS occurred during childhood and ten patients experienced a relapse. All patients had renal dysfunction and three had been treated with hemodialysis. Seven patients experienced probable triggering events including common cold, influenza, bacterial infection and/or vaccination for influenza. All patients had entered remission, and renal function was improved in 11 patients. DNA sequencing of six candidate genes, identified a C3 p.I1157T missense mutation in all eight patients in six families examined and this mutation was causative for aHUS. A causative mutation THBD p.D486Y was also identified in an aHUS patient. Four missense mutations, CFH p.V837I, p.Y1058H, p.V1060L and THBD p.R403K may predispose to aHUS manifestation; the remaining seven missense mutations were likely neutral. In conclusion, the clinical phenotypes of aHUS are various, and there are often trigger factors. The C3 p.I1157T mutation was identified as the causative mutation for aHUS in all patients examined, and may be geographically concentrated in or around the Mie prefecture in central Japan.

  5. Cerebral Hemodynamics in Patients with Hemolytic Uremic Syndrome Assessed by Susceptibility Weighted Imaging and Four-Dimensional Non-Contrast MR Angiography.

    Science.gov (United States)

    Löbel, Ulrike; Forkert, Nils Daniel; Schmitt, Peter; Dohrmann, Thorsten; Schroeder, Maria; Magnus, Tim; Kluge, Stefan; Weiler-Normann, Christina; Bi, Xiaoming; Fiehler, Jens; Sedlacik, Jan

    2016-01-01

    Conventional magnetic resonance imaging (MRI) of patients with hemolytic uremic syndrome (HUS) and neurological symptoms performed during an epidemic outbreak of Escherichia coli O104:H4 in Northern Europe has previously shown pathological changes in only approximately 50% of patients. In contrast, susceptibility-weighted imaging (SWI) revealed a loss of venous contrast in a large number of patients. We hypothesized that this observation may be due to an increase in cerebral blood flow (CBF) and aimed to identify a plausible cause. Baseline 1.5T MRI scans of 36 patients (female, 26; male, 10; mean age, 38.2±19.3 years) were evaluated. Venous contrast was rated on standard SWI minimum intensity projections. A prototype four-dimensional (time resolved) magnetic resonance angiography (4D MRA) assessed cerebral hemodynamics by global time-to-peak (TTP), as a surrogate marker for CBF. Clinical parameters studied were hemoglobin, hematocrit, creatinine, urea levels, blood pressure, heart rate, and end-tidal CO2. SWI venous contrast was abnormally low in 33 of 36 patients. TTP ranged from 3.7 to 10.2 frames (mean, 7.9 ± 1.4). Hemoglobin at the time of MRI (n = 35) was decreased in all patients (range, 5.0 to 12.6 g/dL; mean, 8.2 ± 1.4); hematocrit (n = 33) was abnormally low in all but a single patient (range, 14.3 to 37.2%; mean, 23.7 ± 4.2). Creatinine was abnormally high in 30 of 36 patients (83%) (range, 0.8 to 9.7; mean, 3.7 ± 2.2). SWI venous contrast correlated significantly with hemoglobin (r = 0.52, P = 0.0015), hematocrit (r = 0.65, P effect of blood transfusions in patients with HUS and neurological symptoms.

  6. Upregulation of Shiga toxin receptor CD77/Gb3 and interleukin-1β expression in the brain of EHEC patients with hemolytic uremic syndrome and neurologic symptoms.

    Science.gov (United States)

    Hagel, Christian; Krasemann, Susanne; Löffler, Judith; Püschel, Klaus; Magnus, Tim; Glatzel, Markus

    2015-03-01

    In 2011, a large outbreak of Shiga toxin-producing enterohemorrhagic Escherichia coli (EHEC) infections occurred in northern Germany, which mainly affected adults. Out of 3842 patients, 104 experienced a complicated course comprising hemolytic uremic syndrome and neurological complications, including cognitive impairment, aphasia, seizures and coma. T2 hyperintensities on magnet resonance imaging (MRI) bilateral in the thalami and in the dorsal pons were found suggestive of a metabolic toxic effect. Five of the 104 patients died because of toxic heart failure. In the present study, the post-mortem neuropathological findings of the five EHEC patients are described. Histological investigation of 13 brain regions (frontal, temporal, occipital cortex, corpora mammillaria, thalamus, frontal operculum, corona radiata, gyrus angularis, pons, medulla oblongata, cerebellar vermis and cerebellar hemisphere) showed no thrombosis, ischemic changes or fresh infarctions. Further, no changes were found in electron microscopy. In comparison with five age-matched controls, slightly increased activation of microglia and a higher neuronal expression of interleukin-1β and of Shiga toxin receptor CD77/globotriaosylceramide 3 was observed. The findings were confirmed by Western blot analyses. It is suggested that CD77/globotriaosylceramide upregulation may be a consequence to Shiga toxin exposure, whereas increased interleukin-1β expression may point to activation of inflammatory cascades. © 2014 International Society of Neuropathology.

  7. FIND-CKD: a randomized trial of intravenous ferric carboxymaltose versus oral iron in patients with chronic kidney disease and iron deficiency anaemia.

    Science.gov (United States)

    Macdougall, Iain C; Bock, Andreas H; Carrera, Fernando; Eckardt, Kai-Uwe; Gaillard, Carlo; Van Wyck, David; Roubert, Bernard; Nolen, Jacqueline G; Roger, Simon D

    2014-11-01

    The optimal iron therapy regimen in patients with non-dialysis-dependent chronic kidney disease (CKD) is unknown. Ferinject® assessment in patients with Iron deficiency anaemia and Non-Dialysis-dependent Chronic Kidney Disease (FIND-CKD) was a 56-week, open-label, multicentre, prospective and randomized study of 626 patients with non-dialysis-dependent CKD, anaemia and iron deficiency not receiving erythropoiesis-stimulating agents (ESAs). Patients were randomized (1:1:2) to intravenous (IV) ferric carboxymaltose (FCM), targeting a higher (400-600 µg/L) or lower (100-200 µg/L) ferritin or oral iron therapy. The primary end point was time to initiation of other anaemia management (ESA, other iron therapy or blood transfusion) or haemoglobin (Hb) trigger of two consecutive values <10 g/dL during Weeks 8-52. The primary end point occurred in 36 patients (23.5%), 49 patients (32.2%) and 98 patients (31.8%) in the high-ferritin FCM, low-ferritin FCM and oral iron groups, respectively [hazard ratio (HR): 0.65; 95% confidence interval (CI): 0.44-0.95; P = 0.026 for high-ferritin FCM versus oral iron]. The increase in Hb was greater with high-ferritin FCM versus oral iron (P = 0.014) and a greater proportion of patients achieved an Hb increase ≥1 g/dL with high-ferritin FCM versus oral iron (HR: 2.04; 95% CI: 1.52-2.72; P < 0.001). Rates of adverse events and serious adverse events were similar in all groups. Compared with oral iron, IV FCM targeting a ferritin of 400-600 µg/L quickly reached and maintained Hb level, and delayed and/or reduced the need for other anaemia management including ESAs. Within the limitations of this trial, no renal toxicity was observed, with no difference in cardiovascular or infectious events. NCT00994318. © The Author 2014. Published by Oxford University Press on behalf of ERA-EDTA.

  8. CLINICAL PROFILE AND COMMON CAUSES OF HAEMOLYTIC ANAEMIA IN A TERTIARY CARE HOSPITAL, NORTHERN KERALA

    Directory of Open Access Journals (Sweden)

    Jog Antony

    2016-09-01

    Full Text Available BACKGROUND Haemolytic anaemia is a well-recognised clinical problem. This study looks into the clinical profile of haemolytic anaemia and also attempts to find out the common underlying causative disease. It also tries to group the patients according to the clinical manifestations and underlying causes. MATERIALS AND METHODS This is a hospital-based observational study conducted in a tertiary care centre in Northern Kerala. Forty-four adult patients with clinical manifestations and laboratory evidence of haemolytic anaemia were identified and studied for a period of one year. RESULTS Maximum number of cases were seen in the age group of 20-40 years. The overall male-female ratio was 1.1:1. The most common presenting symptoms were features of anaemia like breathlessness, easy fatigability, headache and tiredness. Family history of anaemia was present in 34.1%. The most common signs observed were pallor and jaundice. The most common causes were autoimmune haemolytic anaemia and sickle cell anaemia. CONCLUSION Haemolytic anaemia mostly affects individuals in their 3rd and 4th decade. There is no significant difference in gender distribution of haemolytic anaemia. Haemolytic anaemia most commonly presents with symptoms of anaemia and jaundice. Commonest causes of haemolytic anaemia are autoimmune haemolytic anaemia and sickle cell anaemia.

  9. A preliminary study of placental umbilical cord whole blood transfusion in under resourced patients with malaria in the background of anaemia.

    Science.gov (United States)

    Bhattacharya, Niranjan

    2006-03-23

    Malaria is an annual killer of over one million people globally and its essential co-morbidity is anaemia. Cord blood, because of its rich mix of foetal and adult haemoglobin, high platelet and WBC counts, hypo-antigenic nature, altered metabolic profile and high affinity for oxygen as well as its anti-malarial effect, is an ideal choice in malaria with anaemia, necessitating blood transfusion. This paper presents an alternative protocol for fresh whole blood/packed cell transfusion from the hospital's biological waste resources, i.e., the placenta, after the birth of a healthy baby from a healthy mother. This collected blood was routinely transfused to patients admitted in our hospital with severe anaemia in the background of confirmed malaria. 94 units of placental umbilical cord whole blood were collected after lower uterine caesarean section (LUCS) from consenting mothers (from 1st April 1999 to April 2005), and safely transfused to 39 informed, consenting patients (age varying from 8 to 72 years). The collected volume of cord blood from each placenta (Unit) varied from 52 ml to 143 ml, with a mean packed cell volume of 48.9 +/- 4.1 SD and a mean haemoglobin concentration of 16.4 Gm percent +/- 1.6 Gm percent SD. The blood was immediately transfused after following the standard adult blood transfusion protocol of screening and cross-matching between the donor and the recipient. On occasion, the collected cord blood was preserved in the refrigerator, if no volunteer was readily available, and transfused within 72 hours of collection. Cord blood transfusion was tested on twenty two patients infected with Plasmodium falciparum and 17 patients with Plasmodium vivax. For inclusion in this study, the patient's plasma haemoglobin had to be 8 gm percent or less (the pre-transfusion haemoglobin in the malaria-infected patients in this series varied from 5.4 gm/dl to 7.9 gm/dl). The rise of haemoglobin within 72 hours of two units of freshly collected cord blood

  10. A preliminary study of placental umbilical cord whole blood transfusion in under resourced patients with malaria in the background of anaemia

    Directory of Open Access Journals (Sweden)

    Bhattacharya Niranjan

    2006-03-01

    Full Text Available Abstract Background Malaria is an annual killer of over one million people globally and its essential co-morbidity is anaemia. Cord blood, because of its rich mix of foetal and adult haemoglobin, high platelet and WBC counts, hypo-antigenic nature, altered metabolic profile and high affinity for oxygen as well as its anti-malarial effect, is an ideal choice in malaria with anaemia, necessitating blood transfusion. Methods This paper presents an alternative protocol for fresh whole blood/packed cell transfusion from the hospital's biological waste resources, i.e., the placenta, after the birth of a healthy baby from a healthy mother. This collected blood was routinely transfused to patients admitted in our hospital with severe anaemia in the background of confirmed malaria. 94 units of placental umbilical cord whole blood were collected after lower uterine caesarean section (LUCS from consenting mothers (from 1st April 1999 to April 2005, and safely transfused to 39 informed, consenting patients (age varying from 8 to 72 years. The collected volume of cord blood from each placenta (Unit varied from 52 ml to 143 ml, with a mean packed cell volume of 48.9 ± 4.1 SD and a mean haemoglobin concentration of 16.4 Gm percent ± 1.6 Gm percent SD. The blood was immediately transfused after following the standard adult blood transfusion protocol of screening and cross-matching between the donor and the recipient. On occasion, the collected cord blood was preserved in the refrigerator, if no volunteer was readily available, and transfused within 72 hours of collection. Results Cord blood transfusion was tested on twenty two patients infected with Plasmodium falciparum and 17 patients with Plasmodium vivax. For inclusion in this study, the patient's plasma haemoglobin had to be 8 gm percent or less (the pre-transfusion haemoglobin in the malaria-infected patients in this series varied from 5.4 gm/dl to 7.9 gm/dl. The rise of haemoglobin within 72 hours of

  11. Efficacy of rituximab and plasmapharesis in an adult patient with antifactor H autoantibody-associated hemolytic uremic syndrome: A case report and literature review.

    Science.gov (United States)

    Deville, Clemence; Garrouste, Cyril; Coppo, Paul; Evrard, Bertrand; Lautrette, Alexandre; Heng, Anne Elisabeth

    2016-09-01

    Antifactor H antibody (anti-CFHAb) is found in 6% to 25% cases of atypical hemolytic uremic syndrome (aHUS) in children, but has been only exceptionally reported in adults. There is no consensus about the best treatment for this type of aHUS. We report the case of an adult patient treated successfully with plasma exchange (PE), steroids, and rituximab.A 27-year-old Caucasian male presented to hospital with anemia, thrombocytopenia, and acute renal failure. One week earlier, he had digestive problems with diarrhea. The diagnosis of anti-CFHAb-associated aHUS (82,000 AU/mL) without CFHR gene mutations was established.He received Rituximab 375 mg/m (4 pulses) with PE and steroids. This treatment achieved renal and hematological remission at day (D) 31 and negative anti-CFHAb at D45 (<100 AU/mL). At D76, a fifth rituximab pulse was performed while CD19 was higher than 10/mm. Steroids were stopped at month (M) 9. The patient has not relapsed during long-term follow-up (M39).Rituximab therapy can be considered for anti-CFHAb-associated aHUS. Monitoring of anti-CFHAb titer may help to guide maintenance therapeutic strategies including Rituximab infusion.

  12. Anaesthetic implications of laparoscopic splenectomy in patients with sickle cell anaemia.

    LENUS (Irish Health Repository)

    Doodnath, R.

    2010-04-01

    With the increasing immigrant population in the Republic of Ireland, the number of patients with sickle cell disease (SCD) seen in the paediatric hospitals is climbing. In this case report, we review the anaesthetic implications and outcome of the first two paediatric patients with SCD to have a laparoscopic splenectomy due to repeated splenic infarcts in the Republic of Ireland.

  13. Anaesthetic implications of laparoscopic splenectomy in patients with sickle cell anaemia.

    LENUS (Irish Health Repository)

    Doodnath, R

    2012-02-01

    With the increasing immigrant population in the Republic of Ireland, the number of patients with sickle cell disease (SCD) seen in the paediatric hospitals is climbing. In this case report, we review the anaesthetic implications and outcome of the first two paediatric patients with SCD to have a laparoscopic splenectomy due to repeated splenic infarcts in the Republic of Ireland.

  14. Aetiological considerations of acquired aplastic anaemia

    International Nuclear Information System (INIS)

    Malik, S.; Sarwar, I.; Mehmood, T.; Naz, F.

    2009-01-01

    Acquired aplastic anaemia is one of the important causes of pancytopenia. This study was conducted to observe the mode of presentation of acquired aplastic anaemia and to find out its possible etiological factors. Methods: It is a hospital based descriptive study of 100 patients of acquired aplastic anaemia. Results: Out of 100 patients 60 were male and 40 female. Majority (44%) of the patients were between 12 - 20 years of age. Patient presented with variable symptoms majority (40%) with fever. Most of the patients had haemoglobin levels between 4 - 6 gm/dl. (53%). Seventy percent of the cases had no obvious cause, while in 30% some known causative factors were found. Chloramphenicol was found to be the most common causative drug. Mortality was 35%. Thirty patients were partially treated and 15 were lost to follow up. Twenty patients showed improvement with treatment. Conclusions: Acquired aplastic anaemia is common among males and more prevalent in younger age group. It is idiopathic in 70% cases while 30% had some cause. It has very high mortality. Doctors need to keep in mind this fatal condition in patients presenting with anaemia and should properly investigate before prescribing antibiotics and haematinics. (author)

  15. Multiple bone and joint disease in a sickle cell anaemia patient: a case report

    Directory of Open Access Journals (Sweden)

    John Ayodele Olaniyi

    2012-05-01

    Multidisciplinary approach was applied to her management and she was finally discharged home on a wheelchair. This case reflects not only the high susceptibility of SCD patients to infection, but also the morbidity and the attendant complications. It also highlights the need to forestall vaso-occlusive crisis (VOC which often predisposes them to developing osteomyelitis. There is a need to have a highly organized, well-equipped and highly subsidized Sickle Cell and rehabilitation Center in order to further improve the care of SCD patients.

  16. NURSE STAFFING AND RENAL ANAEMIA OUTCOMES IN HAEMODIALYSIS CARE.

    Science.gov (United States)

    Erlingmark, Julia; Hedström, Mariann; Lindberg, Magnus

    2016-09-01

    Current trends in renal anaemia management place greater emphasis, and thus increased workload, on the role of the nurse in haemodialysis settings. However, there is little evidence that demonstrates the relationship between nurse staffing and patient outcomes. To describe nurse staffing in haemodialysis settings, its relationship with target levels of renal anaemia management and to describe target level achievement for different ways of organising anaemia management. Cross-sectional audit. Forty (out of 78) haemodialysis centres in Sweden reported quality assurance data. The numbers of bedside registered nurses, licensed nurse assistants and patients undergoing haemodialysis during a predefined morning shift; type of anaemia management and achieved target levels of anaemia management. The mean patient:registered nurse ratio was 2.4 and the mean patient:nurse assistant ratio was 12.8. There were no significant relationships between registered nurse staffing and target level achievement. On average, 45.6% of the patients had haemoglobin within the target levels at centres applying nurse-driven anaemia management, compared with 47.3% at physician-driven centres. These cross-sectional data suggest that renal anaemia outcomes are unrelated to the patient:registered nurse ratio. There is, however, room for improvement in renal anaemia management in the units included in this study, particularly the achievement of target levels of haemoglobin and transferrin saturation. © 2016 European Dialysis and Transplant Nurses Association/European Renal Care Association.

  17. 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

  18. Severe Anaemia during Late Pregnancy

    Directory of Open Access Journals (Sweden)

    Mahenaz Akhtar

    2012-01-01

    Full Text Available Vitamin B12 deficiency is uncommon in pregnancy, it occurs in 10–28% of uncomplicated pregnancies, and is associated with a few complications. We present a case report of a 21-year-old patient with severe anaemia during late pregnancy caused by vitamin B12 deficiency. At 38 weeks gestation and with a BMI of 48.9, a history of rupture of membranes was given but not confirmed. On examination, she appeared pale and therefore full blood counts were done. Interestingly her haemoglobin (Hb levels were 3.7 g/dL. Folate and vitamin B12 levels were also found to be low, and the diagnosis of anaemia caused by vitamin B12 deficiency was made. After treatment with vitamin B12 injections, folic acid and blood transfusions, the patient’s haemoglobin levels improved from 3.7 g/dL to 10.7 g/dL. The conclusion is that effective history taking, diagnosis, and management can prevent many complications that are usually associated with vitamin B12 deficiency anaemia.

  19. Transplant results in adults with Fanconi anaemia

    NARCIS (Netherlands)

    Bierings, Marc; Bonfim, Carmem M.; Peffault De Latour, Regis; Aljurf, Mahmoud; Mehta, Parinda A.; Knol, Cora; Boulad, Farid; Tbakhi, Abdelghani; Esquirol, Albert; Mcquaker, Grant; Sucak, Gulsan A.; Othman, Tarek B.; Halkes, Constantijn J.M.; Carpenter, Ben; Niederwieser, Dietger; Zecca, Marco; Kro¨ger, Nicolaus; Michallet, Mauricette; Risitano, Antonio M.; Ehninger, Gerhard; Porcher, Raphael; Dufour, Carlo

    The outcomes of adult patients transplanted for Fanconi anaemia (FA) have not been well described. We retrospectively analysed 199 adult patients with FA transplanted between 1991 and 2014. Patients were a median of 16 years of age when diagnosed with FA, and underwent transplantation at a median

  20. Prevalence of Anaemia Among Human Immunodeficiency Virus (HIV)

    African Journals Online (AJOL)

    Background: Anaemia is the most commonly encountered haematological abnormality in human immunodeficiency virus (HIV) positive patients with estimates climbing as high as 95% depending on clinical settings. The twin effects of HIV infection and anaemia in pregnancy is associated with adverse maternal and ...

  1. Cytogenetic profile of aplastic anaemia in Indian children

    Directory of Open Access Journals (Sweden)

    Vineeta Gupta

    2013-01-01

    Interpretation & conclusions: Five (11.9% patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings.

  2. Effectiveness of biologic and non-biologic antirheumatic drugs on anaemia markers in 153,788 patients with rheumatoid arthritis: New evidence from real-world data.

    Science.gov (United States)

    Paul, Sanjoy Ketan; Montvida, Olga; Best, Jennie H; Gale, Sara; Pethoe-Schramm, Attila; Sarsour, Khaled

    2018-02-01

    To evaluate the impact of treatment with disease-modifying antirheumatic drugs (DMARDs), including IL-6 receptor inhibitor tocilizumab (TCZ), on anaemia markers in patients with rheumatoid arthritis. Using the Centricity Electronic Medical Records from USA, patients with rheumatoid arthritis diagnosed between January 2000 and April 2016, who initiated TCZ (n = 3732); tofacitinib (TOFA, n = 3126); other biologic DMARD (obDMARD, n = 55,964); or other non-biologic DMARD (onbDMARD, n = 91,236) were identified. Changes in haemoglobin (Hb) and haematocrit (Hct) over 2 years of treatment initiation were evaluated, adjusting and balancing for confounders. Mean (95% CI) adjusted increase in Hb and Hct levels at 24 months in TCZ group were 0.23g/dL (0.14, 0.42) and 0.96% (0.41, 1.52) respectively. Among patients with anaemia in the TCZ group, Hb and Hct increased significantly by 0.72g/dL and 2.06%, respectively. Patients in the TCZ group were 86% (95% CI of OR: 1.43, 2.00) more likely to increase Hb ≥ 1g/dL compared to the other groups combined. No clinically significant changes in Hb were observed in the other groups. The obDMARD group demonstrated lower Hct increase than TCZ group, while no significant changes were observed in the remaining groups. Compared to those who initiated TCZ therapy after 1 year of diagnosis of rheumatoid arthritis, those who initiated earlier were 95% (OR = 1.95; 95% CI: 1.19, 3.21; p < 0.001) more likely to increase Hb within 6 months. This real-world study suggests significant increase in Hb and Hct levels after TCZ therapy in anaemic and non-anaemic patients with rheumatoid arthritis, compared with other biologic and non-biologic DMARDs. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

  3. Cerebral Hemodynamics in Patients with Hemolytic Uremic Syndrome Assessed by Susceptibility Weighted Imaging and Four-Dimensional Non-Contrast MR Angiography.

    Directory of Open Access Journals (Sweden)

    Ulrike Löbel

    Full Text Available Conventional magnetic resonance imaging (MRI of patients with hemolytic uremic syndrome (HUS and neurological symptoms performed during an epidemic outbreak of Escherichia coli O104:H4 in Northern Europe has previously shown pathological changes in only approximately 50% of patients. In contrast, susceptibility-weighted imaging (SWI revealed a loss of venous contrast in a large number of patients. We hypothesized that this observation may be due to an increase in cerebral blood flow (CBF and aimed to identify a plausible cause.Baseline 1.5T MRI scans of 36 patients (female, 26; male, 10; mean age, 38.2±19.3 years were evaluated. Venous contrast was rated on standard SWI minimum intensity projections. A prototype four-dimensional (time resolved magnetic resonance angiography (4D MRA assessed cerebral hemodynamics by global time-to-peak (TTP, as a surrogate marker for CBF. Clinical parameters studied were hemoglobin, hematocrit, creatinine, urea levels, blood pressure, heart rate, and end-tidal CO2.SWI venous contrast was abnormally low in 33 of 36 patients. TTP ranged from 3.7 to 10.2 frames (mean, 7.9 ± 1.4. Hemoglobin at the time of MRI (n = 35 was decreased in all patients (range, 5.0 to 12.6 g/dL; mean, 8.2 ± 1.4; hematocrit (n = 33 was abnormally low in all but a single patient (range, 14.3 to 37.2%; mean, 23.7 ± 4.2. Creatinine was abnormally high in 30 of 36 patients (83% (range, 0.8 to 9.7; mean, 3.7 ± 2.2. SWI venous contrast correlated significantly with hemoglobin (r = 0.52, P = 0.0015, hematocrit (r = 0.65, P < 0.001, and TTP (r = 0.35, P = 0.036. No correlation of SWI with blood pressure, heart rate, end-tidal CO2, creatinine, and urea level was observed. Findings suggest that the loss of venous contrast is related to an increase in CBF secondary to severe anemia related to HUS. SWI contrast of patients with pathological conventional MRI findings was significantly lower compared to patients with normal MRI (mean SWI score, 1

  4. Anaemia in pregnancy.

    Science.gov (United States)

    Goonewardene, Malik; Shehata, Mishkat; Hamad, Asma

    2012-02-01

    Anaemia in pregnancy, defined as a haemoglobin concentration (Hb) anaemia in pregnancy, nutritional iron deficiency anaemia (IDA) being the commonest. Underlying inflammatory conditions, physiological haemodilution and several factors affecting Hb and iron status in pregnancy lead to difficulties in establishing a definitive diagnosis. IDA is associated with increased maternal and perinatal morbidity and mortality, and long-term adverse effects in the new born. Strategies to prevent anaemia in pregnancy and its adverse effects include treatment of underlying conditions, iron and folate supplementation given weekly for all menstruating women including adolescents and daily for women during pregnancy and the post partum period, and delayed clamping of the umbilical cord at delivery. Oral iron is preferable to intravenous therapy for treatment of IDA. B12 and folate deficiencies in pregnancy are rare and may be due to inadequate dietary intake with the latter being more common. These vitamins play an important role in embryo genesis and hence any relative deficiencies may result in congenital abnormalities. Finding the underlying cause are crucial to the management of these deficiencies. Haemolytic anaemias rare also rare in pregnancy, but may have life-threatening complications if the diagnosis is not made in good time and acted upon appropriately. Copyright © 2011 Elsevier Ltd. All rights reserved.

  5. Hemolytic potential of hydrodynamic cavitation.

    Science.gov (United States)

    Chambers, S D; Bartlett, R H; Ceccio, S L

    2000-08-01

    The purpose of this study was to determine the hemolytic potentials of discrete bubble cavitation and attached cavitation. To generate controlled cavitation events, a venturigeometry hydrodynamic device, called a Cavitation Susceptibility Meter (CSM), was constructed. A comparison between the hemolytic potential of discrete bubble cavitation and attached cavitation was investigated with a single-pass flow apparatus and a recirculating flow apparatus, both utilizing the CSM. An analytical model, based on spherical bubble dynamics, was developed for predicting the hemolysis caused by discrete bubble cavitation. Experimentally, discrete bubble cavitation did not correlate with a measurable increase in plasma-free hemoglobin (PFHb), as predicted by the analytical model. However, attached cavitation did result in significant PFHb generation. The rate of PFHb generation scaled inversely with the Cavitation number at a constant flow rate, suggesting that the size of the attached cavity was the dominant hemolytic factor.

  6. CLINICAL PROFILE OF ANAEMIA IN A TERTIARY CARE HOSPITAL

    Directory of Open Access Journals (Sweden)

    Ather Akhtar

    2016-04-01

    Full Text Available BACKGROUND Anaemia causes a reduction in the oxygen carrying capacity of the blood resulting in tissue hypoxia. Cardiac output at rest is not usually increased in most chronic anaemia until haemoglobin levels fall below 7 g/dL, but abnormal rise in output with exercise may occur with levels as high as 10 g/dL. The increase in cardiac output has been observed to correlate well with the degree of anaemia. Other compensatory mechanisms to chronic anaemia available to the body include decreased circulation time and increased tissue oxygen uptake. The latter is facilitated by a shift to the right of the oxygen haemoglobin dissociation curve. Cardiomegaly may also be as a result of the increased workload on the heart from the increased viscosity of blood in anaemia patients. Anaemia in the elderly is an extremely common problem that is associated with increased mortality and poorer health-related quality of life, regardless of the underlying cause of the low haemoglobin. A study of anaemia in elderly patients found a wide variation in prevalence, ranging from 2.9% to 61% in men and 3.3% to 41% in women. Higher rates were found in hospitalised patients than in community dwellers. It is easy to overlook anaemia in the elderly, since such symptoms as fatigue, weakness, or shortness of breath may be attributed to the ageing process itself. Our objective is to show the prevalence of anaemia even in a tertiary health care centre. METHODS One hundred patients were identified who were admitted in the Department of Medicine, Deccan College of Medical Sciences. Among the 100 patients, 38 were male and 62 were female. The study was conducted from Jan 2014 To Jan 2015. Patients having haemoglobin less than 10 g% in the medical wards were enrolled in the study. RESULTS Among the 100 patients, 38 were male and 62 were female. The average haemoglobin was 6.4 g%, the lowest being 2.8 g%. Peripheral blood smear showed hypochromic picture in 58, macrocytic picture in

  7. SEVERE IMMUNE HEMOLYTIC ANEMIA AFTER LIVER TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2013-01-01

    Full Text Available Clinical case of successful treatment of severe immune hemolytic anemia after liver transplantation is represen- ted in this article. The cause of complication was so-called passenger lymphocyte syndrome (a type of graft- versus-host disease. Two plasmapheresis sessions and Ig (0.5 g/kg in combination with increased maintenance immunosuppression with a short course of oral methylprednisolone in a total dose of 150 mg during 12 days were effective. The patient was discharged from hospital 34 days after transplantation in a satisfactory condition with a stable hemoglobin level. 

  8. Pulmonary hypertension in chronic hemolytic anemias: Pathophysiology and treatment.

    Science.gov (United States)

    Haw, Alexandra; Palevsky, Harold I

    2018-04-01

    Pulmonary hypertension has emerged as a major cause of morbidity and mortality in patients with hemoglobinopathies and chronic hemolytic anemias. These hematological diseases include - but are not limited to - sickle cell disease (SCD), thalassemia, paroxysmal nocturnal hematuria, and hereditary spherocytosis. Although most studies have been based on the use of echocardiography as a screening tool for pulmonary hypertension as opposed to the gold standard of right heart catheterization for definitive diagnosis, the association between chronic hemolytic anemia and pulmonary hypertension is evident. Studies have shown that patients with SCD and a tricuspid regurgitant velocity (TRV) ≥ 2.5 m/sec are at increased risk of pulmonary hypertension and are at increased mortality risk. Additional markers of risk of pulmonary hypertension and increased mortality include a pro-BNP >160 pg/mL combined with a 6-min walk distance of pulmonary hypertension in chronic hemolytic anemias. Copyright © 2018 Elsevier Ltd. All rights reserved.

  9. Association of anaemia in primary care patients with chronic kidney disease: cross sectional study of quality improvement in chronic kidney disease (QICKD) trial data

    Science.gov (United States)

    2013-01-01

    Background Anaemia is a known risk factor for cardiovascular disease and treating anaemia in chronic kidney disease (CKD) may improve outcomes. However, little is known about the scope to improve primary care management of anaemia in CKD. Methods An observational study (N = 1,099,292) with a nationally representative sample using anonymised routine primary care data from 127 Quality Improvement in CKD trial practices (ISRCTN5631023731). We explored variables associated with anaemia in CKD: eGFR, haemoglobin (Hb), mean corpuscular volume (MCV), iron status, cardiovascular comorbidities, and use of therapy which associated with gastrointestinal bleeding, oral iron and deprivation score. We developed a linear regression model to identify variables amenable to improved primary care management. Results The prevalence of Stage 3–5 CKD was 6.76%. Hb was lower in CKD (13.2 g/dl) than without (13.7 g/dl). 22.2% of people with CKD had World Health Organization defined anaemia; 8.6% had Hb ≤ 11 g/dl; 3% Hb ≤ 10 g/dl; and 1% Hb ≤ 9 g/dl. Normocytic anaemia was present in 80.5% with Hb ≤ 11; 72.7% with Hb ≤ 10 g/dl; and 67.6% with Hb ≤ 9 g/dl; microcytic anaemia in 13.4% with Hb ≤ 11 g/dl; 20.8% with Hb ≤ 10 g/dl; and 24.9% where Hb ≤ 9 g/dl. 82.7% of people with microcytic and 58.8% with normocytic anaemia (Hb ≤ 11 g/dl) had a low ferritin (stores may be depleted in over >60% of people with normocytic anaemia. Prescribing oral iron has not corrected anaemia. PMID:23351270

  10. Cytogenetic profile of aplastic anaemia in Indian children.

    Science.gov (United States)

    Gupta, Vineeta; Kumar, Akash; Saini, Isha; Saxena, Ajit Kumar

    2013-03-01

    Aplastic anaemia is a rare haematological disorder characterized by pancytopenia with a hypocellular bone marrow. It may be inherited/genetic or acquired. Acquired aplastic anaemia has been linked to many drugs, chemicals and viruses. Cytogenetic abnormalities have been reported infrequently with acquired aplastic anaemia. Majority of the studies are in adult patients from the West. We report here cytogenetic studies on paediatric patients with acquired aplastic anaemia seen in a tertiary care hospital in north India. Patients (n=71, age 4-14 yr) were diagnosed according to the guidelines of International Agranulocytosis and Aplastic Anaemia Study. Conventional cytogenetics with Giemsa Trypsin Giemsa (GTG) banding was performed. Karyotyping was done according to the International System for Human Cytogenetics Nomenclature (ISCN). Of the 71 patients, 42 had successful karyotyping where median age was 9 yr; of these 42, 27 (64.3%) patients had severe, nine (21.4%) had very severe and six (14.3%) had non severe aplastic anaemia. Five patients had karyotypic abnormalities with trisomy 12 (1), trisomy 8 (1) and monosomy 7 (1). Two patients had non numerical abnormalities with del 7 q - and t (5:12) in one each. Twenty nine patients had uninformative results. There was no difference in the clinical and haematological profile of patients with normal versus abnormal cytogenetics although the number of patients was small in the two groups. Five (11.9%) patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings.

  11. EVALUATION OF ANAEMIA USING RED CELL AND RETICULOCYTE PARAMETERS USING AUTOMATED HAEMATOLOGY ANALYSER

    Directory of Open Access Journals (Sweden)

    Vidyadhar Rao

    2016-06-01

    Full Text Available Use of current models of Automated Haematology Analysers help in calculating the haemoglobin contents of the mature Red cells, Reticulocytes and percentages of Microcytic and hypochromic Red cells. This has helped the clinician in reaching early diagnosis and management of Different haemopoietic disorders like Iron Deficiency Anaemia, Thalassaemia and anaemia of chronic diseases. AIM This study is conducted using an Automated Haematology Analyser to evaluate anaemia using the Red Cell and Reticulocyte parameters. Three types of anaemia were evaluated; iron deficiency anaemia, anaemia of long duration and anaemia associated with chronic disease and Iron deficiency. MATERIALS AND METHODS The blood samples were collected from 287 adult patients with anaemia differentiated depending upon their iron status, haemoglobinopathies and inflammatory activity. Iron deficiency anaemia (n=132, anaemia of long duration (ACD, (n=97 and anaemia associated with chronic disease with iron deficiency (ACD Combi, (n=58. Microcytic Red cells, hypochromic red cells percentage and levels of haemoglobin in reticulocytes and matured RBCs were calculated. The accuracy of the parameters was analysed using receiver operating characteristic analyser to differentiate between the types of anaemia. OBSERVATIONS AND RESULTS There was no difference in parameters between the iron deficiency group or anaemia associated with chronic disease and iron deficiency. The hypochromic red cells percentage was the best parameter in differentiating anaemia of chronic disease with or without absolute iron deficiency with a sensitivity of 72.7% and a specificity of 70.4%. CONCLUSIONS The parameters of red cells and reticulocytes were of reasonably good indicators in differentiating the absolute iron deficiency anaemia with chronic disease.

  12. The straight and marrow - a primary care approach to anaemia

    African Journals Online (AJOL)

    therefore requires a structured approach.3. This article is by no ... Several independent studies have reported the .... cause of the anaemia is critical for appropriate management of patients. ... Peripheral blood film – A review. Ann Ibd Pg Med.

  13. Anaemia impedes functional mobility after hip fracture surgery

    DEFF Research Database (Denmark)

    Foss, N.B.; Kristensen, M.T.; Kehlet, H.

    2008-01-01

    mobility in the early post-operative phase after a hip fracture surgery and is an independent risk factor for patients not being able to walk post-operatively. The potential for a liberal transfusion policy to improve the rehabilitation potential in hip fracture patients with anaemia should be investigated......BACKGROUND: the impact of anaemia on the outcome after a hip fracture surgery is controversial, but anaemia can potentially decrease the physical performance and thereby impede post-operative rehabilitation. We therefore conducted a prospective study to establish whether anaemia affected functional...... mobility in the early post-operative phase after a hip fracture surgery. PATIENTS AND METHODS: four hundred and eighty seven consecutive hip fracture patients, treated according to a well-defined multimodal rehabilitation programme with a uniform, liberal transfusion threshold, were studied. Hb...

  14. The prevalence and risk factors associated with anaemia among ...

    African Journals Online (AJOL)

    The prevalence and risk factors associated with anaemia among HIV patients ... haematological complications of HIV, and most of the studies in Nigeria and outside ... Methods: This was a cross – sectional study conducted over a period of 6 ...

  15. A study on the association between parvovirus B19 infection, serum tumour necrosis factor and C-reactive protein levels among Nigerian patients with sickle cell anaemia.

    Science.gov (United States)

    Iwalokun, Bamidele Abiodun; Iwalokun, Senapon Olusola; Hodonu, Semande Olufunmilayo; Aina, Olugbenga Ayoola; Omilabu, Sunday

    2012-11-01

    Microbial burden involving parvovirus B19 infection has been recognised as a major cause of morbidity and mortality in sickle cell anaemia (SCA) patients. Given the recent reports of parvovirus B19 infection in Nigeria and the role of inflammation in sickle cell crisis, knowledge of the relationship between the two may be essential for deploying appropriate interventions in infected patients. This study determined the serum levels of tumour necrosis factor alpha (TNF-α) and C-reactive protein (CRP) as inflammatory markers in Nigerian SCA patients with and without parvovirus B19 infections. A total of 64 SCA patients aged 5-25 years and 41 age-matched apparently healthy volunteers with haemoglobin genotypes AA or AS were enrolled with consent into the study. Parvovirus B19 infection and serum levels of TNF-α and CRP were determined by the ELISA method. The overall prevalence rate of parvovirus B19 infection in the study subjects was 13.3%. This rate further showed gender variation and negative correlation with age. Significant (p Parvovirus B19 infection was found to elicit greater increases in these inflammatory markers than in infected non-SCA controls. We conclude that parvovirus B19 infection is common in this environment, and that serum TNF-α and CRP are predictors of clinical inflammatory episodes in infected SCA patients.

  16. Hemolytic Anemia after Aortic Valve Replacement: a Case Report

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2015-10-01

    Full Text Available Hemolytic anemia is exceedingly rare and an underestimated complication after aortic valve replacement (AVR.The mechanism responsible for hemolysis most commonly involves a regurgitated flow or jet that related to paravalvar leak or turbulence of subvalvar stenosis. It appears to be independent of its severity as assessed by echocardiography. We present a case of a 24-year-old man with a history of AVR in 10 year ago that developed severe hemolytic anemia due to a mild subvalvar stenosis caused by pannus formation and mild hypertrophic septum. After exclusion of other causes of hemolytic anemia and the lack of clinical and laboratory improvement, the patient underwent redo valve surgery with pannus and subvalvar hypertrophic septum resection. Anemia and heart failure symptoms gradually resolved after surgery

  17. Autoimmune hemolytic anemia: transfusion challenges and solutions

    Directory of Open Access Journals (Sweden)

    Barros MM

    2017-03-01

    Full Text Available Melca M O Barros, Dante M Langhi Jr, José O Bordin Department of Clinical and Experimental Oncology, Universidade Federal de São Paulo, São Paulo, Brazil Abstract: Autoimmune hemolytic anemia (AIHA is defined as the increased destruction of red blood cells (RBCs in the presence of anti-RBC autoantibodies and/or complement. Classification of AIHA is based on the optimal auto-RBC antibody reactivity temperatures and includes warm, cold-reactive, mixed AIHA, and drug-induced AIHA subtypes. AIHA is a rare disease, and recommendations for transfusion are based mainly on results from retrospective data and relatively small cohort studies, including heterogeneous patient samples or single case reports. In this article, we will review the challenges and solutions to safely transfuse AIHA patients. We will reflect on the indication for transfusion in AIHA and the difficulty in the accomplishment of immunohematological procedures for the selection of the safest and most compatible RBC units. Keywords: hemolytic anemia, RBC autoantibodies, autoimmunity, hemolysis, direct ­antiglobulin test

  18. ABO incompatibility hemolytic disease following exchange transfusion 96 newborn

    Directory of Open Access Journals (Sweden)

    Khatami S.F

    2007-09-01

    Full Text Available Background: ABO incompatibility hemolytic disease of the newborn is a common cause of clinical jaundice and causes two-thirds of the hemolytic disease in newborns. This study was undertaken to determine the frequency of ABO incompatibility hemolytic disease and its complications in newborns undergoing exchange transfusion.Methods: This prospective and descriptive study was performed in jaundiced newborn infants during a three-year period. Inclusion criteria were: maternal blood type O, newborn blood type A or B, rising indirect hyperbilirubinemia in the first two days of life, positive immunohematologic test for newborns and exchange transfusion. Exclusion criteria were: incomplete information, other accompanying diseases that induce hyperbilirubinemia. All newborn infants received phototherapy before and after exchange transfusion. We did not use intravenous immunoglobulin, hemoxygenase inhibitor drugs and blood products before exchange transfusion.Results: Double-volume exchange transfusion via umbilical cord catheter was performed in 96 patients, 19 (20% of whom suffered from ABO incompatibility. Of these 19 newborns, two-thirds (13 were preterm infants. The minimum level of serum bilirubin was 10 mg/dl and the maximum serum bilirubin level was 35 mg/dl. In six patients (32% serum bilirubin levels were >25mg/dl. The most common blood group was type A for newborns. Immunohematologic tests were positive in 84% of the mothers. ABO incompatibility hemolytic disease was the fourth and second most common reasons for blood exchange transfusion in preterm and term infants, respectively. Laboratory complications were more common than clinical complications. The etiology of 48% of the alloimmunization and 42% of the hemolytic disease in these newborns was ABO incompatibility.Conclusions: Mothers with blood group O and newborns with blood group A or B with positive immunohematologic tests in first hours of life are at high risk for hemolytic disease

  19. [Severe macrocytic anaemia and secondary hyperparathyroidism in a vegan].

    Science.gov (United States)

    Førland, Elizabeth Siren Bjerga; Lindberg, Mats Jacob Hermansson

    2015-08-10

    Nutritional deficiency anaemia in vegans is common and usually due to lack of vitamin B12, as this vitamin is found almost exclusively in animal-based food products. In this case report we present a 39-year-old male vegan with severe macrocytic anaemia due to vitamin B12 deficiency as well as secondary hyperparathyroidism due to severe vitamin D deficiency. We want to emphasize the importance of a detailed nutritional history for patients with anaemia, and the need for vitamin B12 and vitamin D supplements for people who comply with a vegan diet.

  20. Intravenous ferric carboxymaltose for anaemia in pregnancy.

    Science.gov (United States)

    Froessler, Bernd; Collingwood, Joshua; Hodyl, Nicolette A; Dekker, Gustaaf

    2014-03-25

    Iron deficiency is a common nutritional deficiency amongst women of childbearing age. Peri-partum iron deficiency anaemia (IDA) is associated with significant maternal, fetal and infant morbidity. Current options for treatment are limited: these include oral iron supplementation, which can be ineffective and poorly tolerated, and red blood cell transfusions, which carry an inherent risk and should be avoided. Ferric carboxymaltose is a new treatment option that may be better tolerated.The study was designed to assess the safety and efficacy of iron deficiency anaemia (IDA) correction with intravenous ferric carboxymaltose in pregnant women with mild, moderate and severe anaemia in the second and third trimester. Prospective observational study; 65 anaemic pregnant women received ferric carboxymaltose up to 15 mg/kg between 24 and 40 weeks of pregnancy (median 35 weeks gestational age, SD 3.6). Treatment effectiveness was assessed by repeat haemoglobin (Hb) measurements and patient report of well-being in the postpartum period. Safety was assessed by analysis of adverse drug reactions and fetal heart rate monitoring during the infusion. Intravenous ferric carboxymaltose infusion significantly increased Hb values (p anaemia in pregnancy.

  1. No early effect of storage time of transfused red blood cells on fatigue and plasma cytokines in patients with anaemia from non-acute gastrointestinal bleeding

    DEFF Research Database (Denmark)

    Mynster, Tommie; Dziegiel, Morten H; Kofoed, Kristian

    2007-01-01

    Background: Fatigue in anaemia is empirically reduced by blood transfusion. Long storage time of red cells may be associated with immunomodulatory effects, and blood stored for a long time may cause tissue hypoxia upon transfusion. Patients and Methods: 22 patients admitted with haemoglobin ... scale. Clinical observations and blood samples were obtained before transfusion was started, and were repeated 2-8 h after transfusion of the 2nd unit. Measured plasma parameters included IL- 1ß, IL-6, IL-8, IL-10, IL-12 and TNF-a. Results: There were no significant differences between group S and L (ns......SL) in demographic data, observational data and blood plasma values. Haemoglobin increased from mean (± SD) 5.2 ± 0.6 to 6.4 ± 0.7 mmol/l after transfusion (nsSL). Fatigue score significantly decreased from a pre-transfusion median 6.6 (range 0.1-9.9) to post-transfusion 4.7 (0.6-10.0) (p = 0.02) for all patients...

  2. Pregnancy in fanconi anaemia with bone marrow failure: a case report and review of the literature

    OpenAIRE

    Sorbi, Flavia; Mecacci, Federico; Di Filippo, Alessandro; Fambrini, Massimiliano

    2017-01-01

    Background Fanconi anaemia is a rare inherited disease characterized by congenital abnormalities, progressive bone marrow failure and predisposition to malignancy. Successful pregnancies in transplanted patients have been reported. In this paper we will describe the pregnancy of a patient with Fanconi anaemia without transplantation. Case presentation A 34-year-old nulliparous woman with Fanconi anaemia was referred to our institution. Pregnancy was complicated by progressive pancytopenia and...

  3. Frequency of Anaemia in Married Women in Jutial, Gilgit

    International Nuclear Information System (INIS)

    Hassan, U.

    2013-01-01

    Objectives: To determine the frequency of anaemia in married women in Jutial, and to find out the relationship of risk factors of anaemia with levels of anaemia. Study Design: Cross-sectional study. Place and Duration of Study: Household survey carried out in Jutial, Gilgit 1st February 2008 to 30th April 2009. Patients and Method: Interview administered questionnaire along with blood sample collection using sterilized disposable syringes was used in this study on a total of 382 randomly selected, willing, married, non-pregnant, non-lactating women with one or more children. Results were considered significant if r-value was more than 0.5 with p-value less than 0.05. Results: Clinical analysis of the blood samples showed that the average haemoglobin (Hb) level was 12.8 g/dl. According to WHO standards, no woman had severe anaemia with Hb level below 7 g/dl. Majority of the women (77.5%) had normal Hb >= 12 g/dl. The calculated anaemia frequency of 22.5% was found to be little lower than that of national anaemia frequency of around 29 to 33%. Conclusion: Frequency of anaemia 22.5% can be attributed to good education system in Northern Area and the awareness of people towards health concerns. Another reason can be the initiatives by different NGOs working in the area specially Aga Khan Foundation to eradicate anaemia by developing a vast network of health facilities. Further research on the native foods, their extract nutritional values / iron contents and any special foods at higher altitudes may be more than 1500 meters can open new horizon to our understanding of anaemia in the northern areas. (author)

  4. e-ENERCA: telemedicine platform for rare anaemias

    OpenAIRE

    Béatrice Gulbis; María del Mar Mañú Pereira; Emilio J. Armaza Armaza; Pilar Nicolás; for the ENERCA working group

    2014-01-01

    The creation of a telemedicine, tele-expertise platform opens a new challenge within the European Network for Rare and Congenital Anaemias (ENERCA; www.enerca.org). This is a cornerstone in the field of rare anaemias, in which national expertise is usually scarce and a significant number of patients remain undiagnosed. Experts in rare diseases are specially needed of shared knowledge platforms offering the possibility of a faster and more accurate diagnosis and the availability of a better pa...

  5. PREVALENCE OF ANAEMIA IN A SEMI-URBAN POPULATION OF PREGNANT WOMEN

    Directory of Open Access Journals (Sweden)

    Suganthi Ramalingam

    2016-05-01

    Full Text Available INTRODUCTION Anaemia is a serious and prominent problem in the developing countries. This study evaluates the prevalence of anaemia in pregnant women attending the outpatient clinic in a semi-urban hospital for a period of six months. MATERIALS AND METHODS Data from a sample of 500 pregnant women was collected and the patients were screened for anaemia on their booking visit in KFMS&R for a period of six months from March-August 2015. Haemoglobin was estimated by cyanmethaemoglobin method using Systronic photocolorimeter on their first antenatal visit. The degree of anaemia was categorised according to ICMR datamild (10-10.99 g/dL, moderate (7-9.99 g/dL, severe (<7 g/dL. RESULTS It was observed that the prevalence of anaemia was 51.8% in the population under study. The prevalence of mild anaemia was 18.53%, that of moderate anaemia was 63.70% and that of severe anaemia was 17.76%. It was also noticed that the prevalence of anaemia was higher in young pregnant women between 17-21 years of age (63.26%. CONCLUSION Anaemia continues to be a major health problem in India and prevention and early diagnosis will significantly reduce maternal and perinatal morbidity and mortality.

  6. Low plasma concentrations of interleukin 10 in severe malarial anaemia compared with cerebral and uncomplicated malaria

    DEFF Research Database (Denmark)

    Kurtzhals, J A; Adabayeri, V; Goka, B Q

    1998-01-01

    -back regulation of TNF, stimulates bone-marrow function in vitro and counteracts anaemia in mice. We investigated the associations of these cytokines with malarial anaemia. METHODS: We enrolled 175 African children with malaria into two studies in 1995 and 1996. In the first study, children were classified...... as having severe anaemia (n=10), uncomplicated malaria (n=26), or cerebral anaemia (n=41). In the second study, patients were classified as having cerebral malaria (n=33) or being fully conscious (n=65), and the two groups were subdivided by measured haemoglobin as normal (>110 g/L), moderate anaemia (60...... anaemia was 270 pg/mL (95% CI 152-482) compared with 725 pg/mL (465-1129) in uncomplicated malaria and 966 pg/mL (612-1526) in cerebral malaria (pcerebral...

  7. A STUDY OF MORPHOLOGICAL TYPES OF ANAEMIA IN A TERTIARY CARE HOSPITAL - A TWO-YEAR STUDY

    Directory of Open Access Journals (Sweden)

    Kiran Kumar Epari

    2016-08-01

    Full Text Available BACKGROUND In this tertiary care hospital, one of the common condition of all the patients attending the hospital is Anaemia, which is a decrease in haemoglobin content or decrease in haematocrit below the lower limit of the 95% reference range for the individual’s age and sex. The patient presents with varied symptoms of different grades, depending on the severity of anaemia, in different clinical settings. Common presenting symptoms of anaemia are generalised weakness, malaise, loss of appetite and muscular pains. METHODS All the patient samples received at the central laboratory for haemogram, complete blood counts and peripheral smear examination over the period of two years between June 2014 to May 2016 were included in the study. Anaemia cases were diagnosed depending on the criteria of the definition of anaemia, and morphological typing of anaemia was done based on the peripheral smear examination of all the cases with decreased haemoglobin level. Standard cell counter was used to estimate the Hb and other red cell indices, and corroborated with peripheral blood smear examination by standard Romanowsky stains. RESULTS A total of 810 cases of anaemia were diagnosed over the period of two years, of which morphological typing yielded 685 cases of Microcytic and hypochromic anaemia, 15 cases of Dimorphic anaemia, 22 cases of Macrocytic anaemia and 88 cases of Normocytic and normochromic anaemia. CONCLUSION Anaemia is one of the most common problems of patients attending this tertiary care hospital, and detection and morphological typing of anaemia is very helping in guiding the clinicians in diagnosis and further management of anaemias for better patient care.

  8. Rasburicase-induced Hemolytic Anemia in an Adolescent With Unknown Glucose-6-Phosphate Dehydrogenase Deficiency.

    Science.gov (United States)

    Akande, Manzilat; Audino, Anthony N; Tobias, Joseph D

    2017-01-01

    Rasburicase, used in the prevention and treatment of tumor lysis syndrome (TLS), may cause hemolytic anemia and methemoglobinemia in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Although routine screening for G6PD deficiency has been recommended, given the turnaround time for test results and the urgency to treat TLS, such screening may not be feasible. We report a case of rasburicase-induced hemolytic anemia without methemoglobinemia in an adolescent with T-cell lymphoblastic lymphoma, TLS, and previously unrecognized G6PD deficiency. Previous reports of hemolytic anemia with rasburicase are reviewed, mechanisms discussed, and preventative strategies presented.

  9. An uncommon cause of anaemia: Sheehan's syndrome.

    Science.gov (United States)

    Melchardt, Thomas; Namberger, Konrad; Weiss, Lukas; Egle, Alexander; Faber, Viktoria; Greil, Richard

    2010-12-01

    Ischemic pituitary necrosis due to severe postpartum haemorrhage called Sheehan's syndrome is a rare cause of hypopituitarism in the western world, but much more common in developing countries. A 45-year-old female patient being a war refugee from Chechnya with severe anaemia and fatigue was diagnosed at our outpatient department with Sheehan's syndrome after severe postpartum haemorrhage and emergency hysterectomy 15 years ago. Panhypopituitarism was adequately treated with substitution of hydrocortisone, thyroxine and transdermal oestrogen which resulted in haemoglobin increase to nearly normal levels and symptoms improved immediately. Severe anaemia caused by panhypopituitarism shows the importance of the hormonal system for erythropoiesis. Clinical and basic scientific evidence indicates thyroidal hormones to be the main cause.

  10. High rate of sickle cell anaemia in Sub-Saharan Africa underlines the need to screen all children with severe anaemia for the disease.

    Science.gov (United States)

    Kadima, Bertin Tshimanga; Gini Ehungu, Jean Lambert; Ngiyulu, René Makwala; Ekulu, Pépé Mfutu; Aloni, Michel Ntetani

    2015-12-01

    Neonatal screening for sickle cell anaemia is not common practice in the Democratic Republic of Congo, and we determined the prevalence in children with unknown electrophoresis of haemoglobin and anaemia. A cross-sectional study was conducted in four hospitals in the country's capital Kinshasa. We screened 807 patients with anaemia (Hb history of hand foot syndrome, in children who had received more than three transfusions and in children up to 36 months of age at their first transfusion. The high prevalence of sickle cell anaemia in children in Sub-Saharan Africa underlines the need for neonatal screening or, if that is not possible, screening of all children with severe anaemia to identify patients with the disease and provide early management. ©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  11. [Pregnancy-induced haemolytic anaemia].

    Science.gov (United States)

    Karagiozova, J; Masseva, A; Ivanov, St; Marinov, B; Kulinska, R; Boiadjiev, D; Jordanova, D

    2014-01-01

    This is the clinical case of a primiparous eight month pregnant female, presenting with symptoms of pregnancy-induced acute haemolytic anaemia (haemolytic aneamia provoked by an immune mechanism, intra- and extra-erythrocyte defects, and HELLP syndrome were excluded). The anaemia progressed to become life-threatening for both the pregnant women and the foetus, which brought the following questions into consideration: diagnosis of anaemia during pregnancy; dosing of corticosteroid therapy; possibility of giving birth to a viable foetus and prognosis for next pregnancies. Owing to the inter-disciplinary efforts, the life and health of this pregnant woman were preserved, but the foetus was lost.

  12. Anaemia – a pale ale?

    African Journals Online (AJOL)

    literal translation as a definition would be a gross exaggeration. Still, the modern definition is simple: anaemia is any condition characterised by an abnormal decrease in the body's total red blood cell mass. Historical perspective. The ancients ...

  13. Clonal Diversity of Chilean Isolates of Enterohemorrhagic Escherichia coli from Patients with Hemolytic-Uremic Syndrome, Asymptomatic Subjects, Animal Reservoirs, and Food Products

    Science.gov (United States)

    Rios, Maritza; Prado, Valeria; Trucksis, Michele; Arellano, Carolina; Borie, Consuelo; Alexandre, Marcela; Fica, Alberto; Levine, Myron M.

    1999-01-01

    To determine clonal relationship among Chilean enterohemorrhagic Escherichia coli (EHEC) strains from different sources (clinical infections, animal reservoirs, and food), 54 EHEC isolates (44 of E. coli O157, 5 of E. coli O111, and 5 of E. coli O26) were characterized for virulence genes by colony blot hybridization and by pulsed-field gel electrophoresis (PFGE). By colony blotting, 12 different genotypes were identified among the 44 E. coli O157 isolates analyzed, of which the genetic profile stx1+ stx2+ hly+ eae+ was the most prevalent. All human O157 strains that were associated with sporadic cases of hemolytic-uremic syndrome (HUS) carried both the stx1 and stx2 toxin-encoding genes and were eaeA positive. Only 9 of 13 isolates from human controls were stx1+ stx2+, and 8 carried the eaeA gene. Comparison of profiles obtained by PFGE of XbaI-digested genomic DNA showed a great diversity among the E. coli O157 isolates, with 37 different profiles among 39 isolates analyzed. Cluster analysis of PFGE profiles showed a wide distribution of clinical isolates obtained from HUS cases and asymptomatic individuals and a clonal relationship among O157 isolates obtained from HUS cases and pigs. Analysis of virulence genes showed that a correlation exists among strains with the genotype stx1+ stx2+ eae+ and pathogenic potential. A larger difference in the PFGE restriction patterns was observed among the EHEC strains of serogroups O26 and O111. These results indicate that several different EHEC clones circulate in Chile and suggest that pigs are an important animal reservoir for human infections by EHEC. Guidelines have been proposed for better practices in the slaughter of animals in Chile. PMID:9986852

  14. Complement Mutations in Diacylglycerol Kinase-ε–Associated Atypical Hemolytic Uremic Syndrome

    Science.gov (United States)

    Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

    2014-01-01

    Background and objectives Atypical hemolytic uremic syndrome is characterized by vascular endothelial damage caused by complement dysregulation. Consistently, complement inhibition therapies are highly effective in most patients with atypical hemolytic uremic syndrome. Recently, it was shown that a significant percentage of patients with early-onset atypical hemolytic uremic syndrome carry mutations in diacylglycerol kinase-ε, an intracellular protein with no obvious role in complement. These data support an alternative, complement-independent mechanism leading to thrombotic microangiopathy that has implications for treatment of early-onset atypical hemolytic uremic syndrome. To get additional insights into this new form of atypical hemolytic uremic syndrome, the diacylglycerol kinase-ε gene in a cohort with atypical hemolytic uremic syndrome was analyzed. Design, setting, participants, & measurements Eighty-three patients with early-onset atypical hemolytic uremic syndrome (<2 years) enrolled in the Spanish atypical hemolytic uremic syndrome registry between 1999 and 2013 were screened for mutations in diacylglycerol kinase-ε. These patients were also fully characterized for mutations in the genes encoding factor H, membrane cofactor protein, factor I, C3, factor B, and thrombomodulin CFHRs copy number variations and rearrangements, and antifactor H antibodies. Results Four patients carried mutations in diacylglycerol kinase-ε, one p.H536Qfs*16 homozygote and three compound heterozygotes (p.W322*/p.P498R, two patients; p.Q248H/p.G484Gfs*10, one patient). Three patients also carried heterozygous mutations in thrombomodulin or C3. Extensive plasma infusions controlled atypical hemolytic uremic syndrome recurrences and prevented renal failure in the two patients with diacylglycerol kinase-ε and thrombomodulin mutations. A positive response to plasma infusions and complement inhibition treatment was also observed in the patient with concurrent diacylglycerol

  15. Complement mutations in diacylglycerol kinase-ε-associated atypical hemolytic uremic syndrome.

    Science.gov (United States)

    Sánchez Chinchilla, Daniel; Pinto, Sheila; Hoppe, Bernd; Adragna, Marta; Lopez, Laura; Justa Roldan, Maria Luisa; Peña, Antonia; Lopez Trascasa, Margarita; Sánchez-Corral, Pilar; Rodríguez de Córdoba, Santiago

    2014-09-05

    Atypical hemolytic uremic syndrome is characterized by vascular endothelial damage caused by complement dysregulation. Consistently, complement inhibition therapies are highly effective in most patients with atypical hemolytic uremic syndrome. Recently, it was shown that a significant percentage of patients with early-onset atypical hemolytic uremic syndrome carry mutations in diacylglycerol kinase-ε, an intracellular protein with no obvious role in complement. These data support an alternative, complement-independent mechanism leading to thrombotic microangiopathy that has implications for treatment of early-onset atypical hemolytic uremic syndrome. To get additional insights into this new form of atypical hemolytic uremic syndrome, the diacylglycerol kinase-ε gene in a cohort with atypical hemolytic uremic syndrome was analyzed. Eighty-three patients with early-onset atypical hemolytic uremic syndrome (<2 years) enrolled in the Spanish atypical hemolytic uremic syndrome registry between 1999 and 2013 were screened for mutations in diacylglycerol kinase-ε. These patients were also fully characterized for mutations in the genes encoding factor H, membrane cofactor protein, factor I, C3, factor B, and thrombomodulin CFHRs copy number variations and rearrangements, and antifactor H antibodies. Four patients carried mutations in diacylglycerol kinase-ε, one p.H536Qfs*16 homozygote and three compound heterozygotes (p.W322*/p.P498R, two patients; p.Q248H/p.G484Gfs*10, one patient). Three patients also carried heterozygous mutations in thrombomodulin or C3. Extensive plasma infusions controlled atypical hemolytic uremic syndrome recurrences and prevented renal failure in the two patients with diacylglycerol kinase-ε and thrombomodulin mutations. A positive response to plasma infusions and complement inhibition treatment was also observed in the patient with concurrent diacylglycerol kinase-ε and C3 mutations. Data suggest that complement dysregulation influences

  16. A Rare Case; Hemolytic Disease of Newborn Associated with Anti-jkb

    OpenAIRE

    İlknur Tolunay; Meral Oruç; Orkun Tolunay

    2015-01-01

    Jka and Jkb antibodies (Kidd blood group system) can cause acute and delayed type transfusion reactions as well as hemolytic disease of newborn. Jka and Jkb antibodies are seen after events like blood transfusions, pregnancy, abortion and curettage. Hemolytic disease of newborn related to Kidd-Jkb incompatibility is rare and mostly has a good prognosis. The patient was consulted to our department because of 20 hours of jaundice after birth. He was treated with intensive phot...

  17. Roth spots in pernicious anaemia

    OpenAIRE

    Macauley, Mavin; Nag, Satyajit

    2011-01-01

    Roth spots are white-centred retinal haemorrhages, previously thought to be pathognomonic for subacute bacterial endocarditis. A number of other conditions can be associated with Roth spots. In this case, the authors describe the association of Roth spots and pernicious anaemia. This association has been rarely described in the medical literature. Correct diagnosis and treatment with intramuscular vitamin B12 injections resulted in complete resolution of the anaemia and Roth spots. The author...

  18. Retinal phlebitis associated with autoimmune hemolytic anemia.

    Science.gov (United States)

    Chew, Fiona L M; Tajunisah, Iqbal

    2009-01-01

    To describe a case of retinal phlebitis associated with autoimmune hemolytic anemia. Observational case report. A 44-year-old Indian man diagnosed with autoimmune hemolytic anemia presented with a 1-week history of blurred vision in both eyes. Fundus biomicroscopy revealed bilateral peripheral retinal venous sheathing and cellophane maculopathy. Fundus fluorescent angiogram showed bilateral late leakage from the peripheral venous arcades and submacular fluid accumulation. The retinal phlebitis resolved following a blood transfusion and administration of systemic steroids. Retinopathy associated with autoimmune hemolytic anemia is not well known. This is thought to be the first documentation of retinal phlebitis occurring in this condition.

  19. Thyroid storm and warm autoimmune hemolytic anemia.

    Science.gov (United States)

    Moore, Joseph A; Gliga, Louise; Nagalla, Srikanth

    2017-08-01

    Graves' disease is often associated with other autoimmune disorders, including rare associations with autoimmune hemolytic anemia (AIHA). We describe a unique presentation of thyroid storm and warm AIHA diagnosed concurrently in a young female with hyperthyroidism. The patient presented with nausea, vomiting, diarrhea and altered mental status. Laboratory studies revealed hemoglobin 3.9g/dL, platelets 171×10 9 L -1 , haptoglobin storm and warm AIHA. She was started on glucocorticoids to treat both warm AIHA and thyroid storm, as well as antithyroid medications, propranolol and folic acid. Due to profound anemia and hemodynamic instability, the patient was transfused two units of uncrossmatched packed red blood cells slowly and tolerated this well. She was discharged on methimazole as well as a prolonged prednisone taper, and achieved complete resolution of the thyrotoxicosis and anemia at one month. Hyperthyroidism can affect all three blood cell lineages of the hematopoietic system. Anemia can be seen in 10-20% of patients with thyrotoxicosis. Several autoimmune processes can lead to anemia in Graves' disease, including pernicious anemia, celiac disease, and warm AIHA. This case illustrates a rarely described presentation of a patient with Graves' disease presenting with concurrent thyroid storm and warm AIHA. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. Rational Management of Iron-Deficiency Anaemia in Inflammatory Bowel Disease

    Science.gov (United States)

    Vikner, Malene Elbaek; Weiss, Günter

    2018-01-01

    Anaemia is the most frequent, though often neglected, comorbidity of inflammatory bowel disease (IBD). Here we want to briefly present (1) the burden of anaemia in IBD, (2) its pathophysiology, which mostly arises from bleeding-associated iron deficiency, followed by (3) diagnostic evaluation of anaemia, (4) a balanced overview of the different modes of iron replacement therapy, (5) evidence for their therapeutic efficacy and subsequently, (6) an updated recommendation for the practical management of anaemia in IBD. Following the introduction of various intravenous iron preparations over the last decade, questions persist about when to use these preparations as opposed to traditional and other novel oral iron therapeutic agents. At present, oral iron therapy is generally preferred for patients with quiescent IBD and mild iron-deficiency anaemia. However, in patients with flaring IBD that hampers intestinal iron absorption and in those with inadequate responses to or side effects with oral preparations, intravenous iron supplementation is the therapy of choice, although information on the efficacy of intravenous iron in patients with active IBD and anaemia is scare. Importantly, anaemia in IBD is often multifactorial and a careful diagnostic workup is mandatory for optimized treatment. Nevertheless, limited information is available on optimal therapeutic start and end points for treatment of anaemia. Of note, neither oral nor intravenous therapies seem to exacerbate the clinical course of IBD. However, additional prospective studies are still warranted to determine the optimal therapy in complex conditions such as IBD. PMID:29342861

  1. Pure red cell aplasia following autoimmune hemolytic anemia: An enigma

    Directory of Open Access Journals (Sweden)

    M Saha

    2013-01-01

    Full Text Available A 26-year-old previously healthy female presented with a 6-month history of anemia. The laboratory findings revealed hemolytic anemia and direct antiglobulin test was positive. With a diagnosis of autoimmune hemolytic anemia (AIHA, prednisolone was started but was ineffective after 1 month of therapy. A bone marrow trephine biopsy revealed pure red cell aplasia (PRCA showing severe erythroid hypoplasia. The case was considered PRCA following AIHA. This combination without clear underlying disease is rare. Human parvovirus B19 infection was not detected in the marrow aspirate during reticulocytopenia. The patient received azathioprine, and PRCA improved but significant hemolysis was once again documented with a high reticulocyte count. The short time interval between AIHA and PRCA phase suggested an increased possibility of the evolution of a single disease.

  2. Intravenous Immunoglobulin G Treatment in ABO Hemolytic Disease of the Newborn, is it Myth or Real?

    Science.gov (United States)

    Beken, Serdar; Hirfanoglu, Ibrahim; Turkyilmaz, Canan; Altuntas, Nilgun; Unal, Sezin; Turan, Ozden; Onal, Esra; Ergenekon, Ebru; Koc, Esin; Atalay, Yildiz

    2014-03-01

    Intravenous Immunoglobulin G (IVIG) therapy has been used as a component of the treatment of hemolytic disease of the newborn. There is still no consensus on its use in ABO hemolytic disease of the newborn routinely. The aim of this study is to determine whether administration of IVIG to newborns with ABO incompatibility is necessary. One hundred and seventeen patients with ABO hemolytic disease and positive Coombs test were enrolled into the study. The subjects were healthy except jaundice. Infants were divided into two groups: Group I (n = 71) received one dose of IVIG (1 g/kg) and LED phototherapy whereas Group II (n = 46) received only LED phototherapy. One patient received erythrocyte transfusion in Group I, no exchange transfusion was performed in both groups. Mean duration of phototherapy was 3.1 ± 1.3 days in Group I and 2.27 ± 0.7 days in Group II (p hemolytic disease. Meticulus follow-up of infants with ABO hemolytic disease and LED phototherapy decreases morbidity. IVIG failed to show preventing hemolysis in ABO hemolytic disease.

  3. Celiac disease, iron deficiency anaemia, grave's disease, osteopenia and short stature in single patient

    International Nuclear Information System (INIS)

    Radaideh, A.M.

    2015-01-01

    Celiac disease is an intestinal immune mediated disorder, triggered by ingestion of gluten-containing diet in genetically susceptible individuals. The genetic pre-disposition is related to human leukocyte antigen (HLA) class II genes, especially HLA-DQ2 positive patients. The prevalence of celiac disease in high worldwide and it has been estimated to be 1-26% in Western countries. Many auto-immune diseases can be associated with celiac disease including auto-immune thyroid disease; hashimoto thyroiditis and grave's disease. The opposite also appears to be true, celiac disease is found on persons with auto-immune thyroid disorders at high rates than the general population. Celiac disease is also associated with other extraintestinal diseases other the auto-immune diseases like anemia, short stature, metabolic bone disease and others. Screening for celiac disease should be considered in patients with auto-immune thyroid disease, anemia, short stature and metabolic bone disease. The life-long adherence to gluten-free diet is the only cure in celiac disease and can improve the quality of patients life and prevent future complications. This report describes a case of Grave's disease, Iron deficiency anemia, Short stature, Osteopenia, diagnosed to have Celiac disease. (author)

  4. 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.

  5. Autoimmune hemolytic anemia: From lab to bedside

    Directory of Open Access Journals (Sweden)

    R K Chaudhary

    2014-01-01

    Full Text Available Autoimmune hemolytic anemia (AIHA is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The "best match" or "least incompatible units" can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue "best match" packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services.

  6. Pernicious anaemia and Campylobacter like organisms; is the gastric antrum resistant to colonisation?

    OpenAIRE

    Flejou, J F; Bahame, P; Smith, A C; Stockbrugger, R W; Rode, J; Price, A B

    1989-01-01

    Gastric biopsies from 86 patients with pernicious anaemia were examined for Campylobacter like organisms with particular attention to those showing an antral gastritis in addition to the usual pattern of body gastritis. All the patients had chronic atrophic gastritis in the body but Campylobacter like organisms were found at this site in only three patients. Thus the Type A pattern of gastritis (autoimmune) seen in patients with pernicious anaemia is only rarely associated with Campylobacter ...

  7. The frequency and severity of epistaxis in children with sickle cell anaemia in eastern Uganda

    DEFF Research Database (Denmark)

    Nardo-Marina, Amina Nielsen; Williams, Thomas N; Olupot-Olupot, Peter

    2017-01-01

    BACKGROUND: There are a paucity of data on epistaxis as it pertains to sickle cell anaemia. Some case studies suggest epistaxis to be a significant complication in patients with sickle cell anaemia in sub-Saharan Africa; however, no robust studies have sought to establish the epidemiology...... or pathophysiology of this phenomenon. METHODS: We conducted a case-control study with the aim of investigating the importance of epistaxis among children presenting with sickle cell anaemia at the Mbale Regional Referral Hospital in eastern Uganda. Cases were children aged 2-15 years with an existing diagnosis...... of laboratory confirmed sickle cell anaemia, while controls were children without sickle cell anaemia who were frequency matched to cases on the basis of age group and gender. The frequency and severity of epistaxis was assessed using a structured questionnaire developed specifically for this study. Odds ratios...

  8. International consensus statement on the peri-operative management of anaemia and iron deficiency

    DEFF Research Database (Denmark)

    Muñoz, M.; Acheson, A. G.; Auerbach, M.

    2017-01-01

    Despite current recommendations on the management of pre-operative anaemia, there is no pragmatic guidance for the diagnosis and management of anaemia and iron deficiency in surgical patients. A number of experienced researchers and clinicians took part in an expert workshop and developed...... in the peri-operative period. These statements include: a diagnostic approach for anaemia and iron deficiency in surgical patients; identification of patients appropriate for treatment; and advice on practical management and follow-up. We urge anaesthetists and peri-operative physicians to embrace...

  9. Anaemia in pregnancy at booking in Gombe, North-eastern Nigeria.

    Science.gov (United States)

    Bukar, M; Audu, B M; Yahaya, U R; Melah, G S

    2008-11-01

    Anaemia in pregnancy is an important reproductive health problem associated with increased maternal and perinatal morbidity and mortality. This study was undertaken to determine the prevalence of anaemia in pregnancy at booking in Gombe, North-eastern Nigeria. A cross-sectional study of 461 women attending the antenatal clinic was carried out. Anaemia in pregnancy was defined as a packed cell volume (PCV) of prevalence of anaemia at booking of 51.8%. The majority of these patients, 67.4%, were mildly anaemic, 30.5% were moderately anaemic while only 2.1% had severe anaemia. Most, 316 (68.5%) of the women booked in the second trimester while only 3.0% booked in the first trimester. There was no relationship between parity and anaemia in pregnancy in this study The majority of the women, 293 (63.5%) were in the lower social class. Because the majority of the anaemic gravidae are in the low social class, provision of haematinics at little or no cost will go a long way towards reducing the high prevalence of anaemia in pregnancy. In the long run, educational and economic empowerment of the women is the key to reducing the overall prevalence of anaemia to the barest minimum.

  10. Prevalence of anaemia at booking in a semi-urban community in north-central Nigeria.

    Science.gov (United States)

    Adewara, E O; Omokanye, L O; Olatinwo, A W O; Durowade, K A; Panti, A A; Salaudeen, A G

    2014-12-01

    This study was carried out to determine the prevalence of anaemia at booking clinic, describe the antenatal booking pattern, and categorize the degree of anaemia with certain demographic features. This is a descriptive cross-sectional study carried out over a six month period between 1st April and 30th September 2008. A questionnaire was used to obtain demographic information and venous blood samples were collected from 1,086 consecutive patients who consented to participate in the study. The blood samples were tested for haemoglobin levels, genotype and blood group. Seven hundred and thirty two (67.4%) of the women anaemic at booking. Anaemia was more prevalent among multgravidae than primigravidae (panaemia while 40(4.4%) had moderate anaemia and 15 (1.4%) were severely anaemic, of which 8 (53.3%) were below 18 years of age. Varied degrees of anaemia were more common among women aged 24-28 years and in the 3rd trimester of pregnancy (80.7%) (ppregnancies. Thirteen (1.2%) had sickle cell anaemia. Prevalence of anaemia at booking remains high in our society. Urgent need for public health education on early antenatal booking and improved literacy level of women is suggested to reduce the burden of anaemia in pregnancy.

  11. Recommendations regarding splenectomy in hereditary hemolytic anemias

    Science.gov (United States)

    Iolascon, Achille; Andolfo, Immacolata; Barcellini, Wilma; Corcione, Francesco; Garçon, Loïc; De Franceschi, Lucia; Pignata, Claudio; Graziadei, Giovanna; Pospisilova, Dagmar; Rees, David C.; de Montalembert, Mariane; Rivella, Stefano; Gambale, Antonella; Russo, Roberta; Ribeiro, Leticia; Vives-Corrons, Jules; Martinez, Patricia Aguilar; Kattamis, Antonis; Gulbis, Beatrice; Cappellini, Maria Domenica; Roberts, Irene; Tamary, Hannah

    2017-01-01

    Hereditary hemolytic anemias are a group of disorders with a variety of causes, including red cell membrane defects, red blood cell enzyme disorders, congenital dyserythropoietic anemias, thalassemia syndromes and hemoglobinopathies. As damaged red blood cells passing through the red pulp of the spleen are removed by splenic macrophages, splenectomy is one possible therapeutic approach to the management of severely affected patients. However, except for hereditary spherocytosis for which the effectiveness of splenectomy has been well documented, the efficacy of splenectomy in other anemias within this group has yet to be determined and there are concerns regarding short- and long-term infectious and thrombotic complications. In light of the priorities identified by the European Hematology Association Roadmap we generated specific recommendations for each disorder, except thalassemia syndromes for which there are other, recent guidelines. Our recommendations are intended to enable clinicians to achieve better informed decisions on disease management by splenectomy, on the type of splenectomy and the possible consequences. As no randomized clinical trials, case control or cohort studies regarding splenectomy in these disorders were found in the literature, recommendations for each disease were based on expert opinion and were subsequently critically revised and modified by the Splenectomy in Rare Anemias Study Group, which includes hematologists caring for both adults and children. PMID:28550188

  12. Cytokine Expression in Homozygous Sickle Cell Anaemia

    Directory of Open Access Journals (Sweden)

    Nnodim Johnkennedy

    2015-01-01

    Full Text Available Background: Sickle cell anaemia is an inherited disease in which the red blood cells become rigid and sticky, and change from being disc-shaped to being crescent-shaped. The change in shape is due to the presence of an abnormal form of haemoglobin. This results in severe pain and damage to some organs. Aim and Objective: The study was carried out to determine the levels of cytokine in sickle cell anemia. Material and Methods: Thirty confirmed sickle cell patients in steady state (HbSS-SS and thirty persons with normal haemoglobin (HbAA as well as sixteen sickle cell disease in crises (HbSS-cr between the ages of 15 to 30 years were selected in this study. Cytokines including interleukin 1 beta (IL- 1β, interleukin 2 (IL- 2, interleukin (IL-6, tumour necrosis factor alpha (TNF-α, and interferon gamma (IFN- λ were measured by commercially available ELISA kits. Results: The results obtained showed that the levels of TNF-α and IL-6 in sickle cell anaemia patients in crisis were significantly elevated when compared with sickle cell in steady state (P<0.05. Similarly, the levels of IL-1β, IL-6, and IFN- λ were significantly increased in sickle cell anaemia stable state when compared to HbAA subjects (P<0.05. Conclusion: This may probably implies that cytokine imbalance is implicated in the pathogenesis of sickle cell crisis. Also, cytokines could be used as an inflammatory marker as well as related marker in disease severity and hence therapeutic intervention.

  13. Roth spots in pernicious anaemia.

    Science.gov (United States)

    Macauley, Mavin; Nag, Satyajit

    2011-04-19

    Roth spots are white-centred retinal haemorrhages, previously thought to be pathognomonic for subacute bacterial endocarditis. A number of other conditions can be associated with Roth spots. In this case, the authors describe the association of Roth spots and pernicious anaemia. This association has been rarely described in the medical literature. Correct diagnosis and treatment with intramuscular vitamin B(12) injections resulted in complete resolution of the anaemia and Roth spots. The authors hope to alert clinicians to think of various differentials of Roth spots, and initiate prompt investigation and management.

  14. Occurrence of Anaemia in the First Year of Inflammatory Bowel Disease in a European Population-based Inception Cohort-An ECCO-EpiCom Study.

    Science.gov (United States)

    Burisch, Johan; Vegh, Zsuzsanna; Katsanos, Konstantinnos H; Christodoulou, Dimitrios K; Lazar, Daniela; Goldis, Adrian; O'Morain, Colm; Fernandez, Alberto; Pereira, Santos; Myers, Sally; Sebastian, Shaji; Pedersen, Natalia; Olse, Jóngerð; Rubek Nielsen, Kári; Schwartz, Doron; Odes, Selwyn; Almer, Sven; Halfvarson, Jonas; Turk, Niksa; Cukovic-Cavka, Silvja; Nikulina, Inna; Belousova, Elena; Duricova, Dana; Bortlik, Martin; Shonová, Olga; Salupere, Riina; Barros, Louisa; Magro, Fernando; Jonaitis, Laimas; Kupcinskas, Limas; Turcan, Svetlana; Kaimakliotis, Ioannis; Ladefoged, Karin; Kudsk, Karen; Andersen, Vibeke; Vind, Ida; Thorsgaard, Niels; Oksanen, Pia; Collin, Pekka; Dal Piaz, Giulia; Santini, Alessia; Niewiadomski, Ola; Bell, Sally; Moum, Bjørn; Arebi, Naila; Kjeldsen, Jens; Carlsen, Katrine; Langholz, Ebbe; Lakatos, Peter Laszlo; Munkholm, Pia; Gerdes, Lars Ulrik; Dahlerup, Jens Frederik

    2017-10-01

    Anaemia is an important complication of inflammatory bowel disease [IBD]. The aim of this study was to determine the prevalence of anaemia and the practice of anaemia screening during the first year following diagnosis, in a European prospective population-based inception cohort. Newly diagnosed IBD patients were included and followed prospectively for 1 year in 29 European and one Australian centre. Clinical data including demographics, medical therapy, surgery and blood samples were collected. Anaemia was defined according to the World Health Organization criteria. A total of 1871 patients (Crohn's disease [CD]: 686, 88%; ulcerative colitis [UC]: 1,021, 87%; IBD unclassified [IBDU] 164. 81%) were included in the study. The prevalence of anaemia was higher in CD than in UC patients and, overall, 49% of CD and 39% of UC patients experienced at least one instance of anaemia during the first 12 months after diagnosis. UC patients with more extensive disease and those from Eastern European countries, and CD patients with penetrating disease or colonic disease location, had higher risks of anaemia. CD and UC patients in need of none or only mild anti-inflammatory treatment had a lower risk of anaemia. In a significant proportion of patients, anaemia was not assessed until several months after diagnosis, and in almost half of all cases of anaemia a thorough work-up was not performed. Overall, 42% of patients had at least one instance of anaemia during the first year following diagnosis. Most patients were assessed for anaemia regularly; however, a full anaemia work-up was frequently neglected in this community setting. Copyright © 2017 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: journals.permissions@oup.com

  15. Left ventricular systolic function in sickle cell anaemia: an ...

    African Journals Online (AJOL)

    Keywords: Left ventricular systolic function, sickle cell anaemia, echocardiographic evaluation, adult Nigerian patients. ..... Quadratic .505. -0.390. 12.231. 8.587 .001*. Cubic .510. -0.180. 8.264. 8.619 .001*. This relationship was further evaluated by means of scat- ter plots and subsequently by regression analysis. The.

  16. Haemolytic anaemia as a complication to intravenous immunoglobulin infusion

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Harbo, Thomas; Christiansen, Ingelise

    performed before and two weeks after infusion of IVIg. Following treatment blood haemoglobin declined from 8.6±0.8 to 8.1±1.3mmol/l, p... naive patients are susceptible to develop haemolysis. Haemolytic anaemia is a severe side effect that seems to be more frequent after immunoglobulin infusions than previously recognized....

  17. the prevalence and risk factors associated with anaemia among hiv ...

    African Journals Online (AJOL)

    associated with the development of anaemia among HIV patients attending the special ... Methods: This was a cross – sectional study conducted over a period of 6 months from June ..... reflected in other studies (Mukund B.K. et al 2015, Alem.

  18. Renoscintigraphy in assessment of renal lesions in children after hemolytic-uremic syndrome

    International Nuclear Information System (INIS)

    Lass, P.; Marczak, E.; Romanowicz, G.; and others.

    1994-01-01

    The aim of the study was to assess the role of renoscintigraphic examination in monitoring of patients after the hemolytic-uremic syndrome. 27 children mean 9 years the hemolytic-uremic syndrome underwent the complex of biochemical, ultrasound and renoscintigraphic examinations. The abnormal renoscintigraphic was seen in 85.1% of children, while the alternative test described the renal lesion in 29-66%. Renoscintigraphic examination seems to be the most sensitive in monitoring of remote sequel in patients after HUS. Those patients should undergone long-lasting observation, for the sake of possibility of development of renal insufficiency. (author). 14 refs

  19. Hemolytic disease of the fetus and newborn due to multiple maternal antibodies.

    Science.gov (United States)

    Markham, Kara Beth; Rossi, Karen Q; Nagaraja, Haikady N; O'Shaughnessy, Richard W

    2015-07-01

    The objective of the study was to determine whether women with combinations of red blood cell antibodies are more likely to develop significant hemolytic disease of the fetus and newborn than those with single antibodies. A retrospective exposure cohort study was conducted of pregnant women with red blood cell antibodies. The development of significant hemolytic disease of the fetus and newborn was then compared between patients with single antibodies and those with multiple antibodies. Data analysis was limited to pregnancies delivering since the year 2000. Thirteen percent of the patients referred to our program had multiple red blood cell antibodies. Odds of developing significant hemolytic disease of the fetus and newborn for patients with anti-Rh(D) combined with at least 1 additional red blood cell antibody were 3.65 times the odds for women with anti-Rh(D) antibodies in isolation (95% confidence interval, 1.84-7.33). In the setting of multiple antibodies including anti-Rh(D), Rh-positive fetuses/neonates have an increased odds of developing significant hemolytic disease even if the fetus is negative for the other corresponding red blood cell antigen. Women with multiple red blood cell antibodies are more likely to develop significant hemolytic disease of the fetus and newborn than those with a single antibody especially in the presence of anti-(Rh)D. This pathophysiology may suggest a more aggressive immune response in women who develop more than 1 red blood cell antibody. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Prepartum anaemia: prevention and treatment.

    Science.gov (United States)

    Milman, Nils

    2008-12-01

    This review focuses on the occurrence, prevention and treatment of anaemia during pregnancy in Western societies. Iron deficiency anaemia (IDA) is the most prevalent deficiency disorder and the most frequent form of anaemia in pregnant women. Minor causes of anaemia are folate and vitamin B12 deficiency, haemoglobinopathy and haemolytic anaemia. Anaemia is defined as haemoglobin of pregnancy. The prevalence of prepartum anaemia in the third trimester ranges 14-52% in women taking placebo and 0-25% in women taking iron supplements, dependent on the doses of iron. In studies incorporating serum ferritin, the frequency of IDA in placebo-treated women ranges 12-17% and in iron-supplemented women 0-3%. Requirements for absorbed iron increase during pregnancy from 0.8 mg/day in the first trimester to 7.5 mg/day in the third trimester, on the average approximately 4.4 mg/day, and dietary measures are inadequate to reduce the frequency of prepartum IDA. However, IDA is efficiently prevented by oral iron supplements in doses of 30-40 mg ferrous iron taken between meals from early pregnancy to delivery. Treatment of IDA should aim at replenishing body iron deficits by oral and/or intravenous administration of iron. In women with slight to moderate IDA, i.e. haemoglobin of 90-105 g/L, treatment with oral ferrous iron of approximately 100 mg/day between meals is the therapeutic option in the first and second trimester; haemoglobin should be checked after 2 weeks and provided an increase of >or=10 g/L, oral iron therapy has proved effective and should continue. Treatment with intravenous iron is superior to oral iron with respect to the haematological response. Intravenous iron is considered safe in the second and third trimester, while there is little experience in the first trimester. Intravenous iron of 600-1,200 mg should be considered: (1) as second option if oral iron fails to increase haemoglobin within 2 weeks; (2) as first option at profound IDA, i.e. haemoglobin of

  1. Pregnancy in fanconi anaemia with bone marrow failure: a case report and review of the literature.

    Science.gov (United States)

    Sorbi, Flavia; Mecacci, Federico; Di Filippo, Alessandro; Fambrini, Massimiliano

    2017-02-03

    Fanconi anaemia is a rare inherited disease characterized by congenital abnormalities, progressive bone marrow failure and predisposition to malignancy. Successful pregnancies in transplanted patients have been reported. In this paper we will describe the pregnancy of a patient with Fanconi anaemia without transplantation. A 34-year-old nulliparous woman with Fanconi anaemia was referred to our institution. Pregnancy was complicated by progressive pancytopenia and two severe infections. C-section was performed at 36 weeks. Both infant and mother are well. Successful pregnancy in a Fanconi anaemia patient with bone marrow failure is possible. The mode of delivery in patients with bone marrow failure should be determined by obstetric indications. The case highlights the safe outcome of the pregnancy with strict clinical and laboratory control by a multidisciplinary team.

  2. CLINICAL PROFILE AND COMMON CAUSES OF HAEMOLYTIC ANAEMIA IN A TERTIARY CARE HOSPITAL, NORTHERN KERALA

    OpenAIRE

    Jog Antony; Reeta J; Sreelakshmi S; Rohit Mathew4; Adarsh Surendran

    2016-01-01

    BACKGROUND Haemolytic anaemia is a well-recognised clinical problem. This study looks into the clinical profile of haemolytic anaemia and also attempts to find out the common underlying causative disease. It also tries to group the patients according to the clinical manifestations and underlying causes. MATERIALS AND METHODS This is a hospital-based observational study conducted in a tertiary care centre in Northern Kerala. Forty-four adult patients with clinical manifestati...

  3. ANAEMIA AS A RISK FACTOR FOR MICROVASCULAR COMPLICATIONS IN TYPE 2 DM- A CROSS-SECTIONAL STUDY

    Directory of Open Access Journals (Sweden)

    Kamanuru Ethirajulu Govindarajulu

    2016-11-01

    Full Text Available BACKGROUND It is well known that diabetes adversely affects the kidneys finally leading to anaemia by various mechanisms. Several studies had postulated that anaemia developing before renal complications has an independent association with microvascular complication in type 2 diabetic patients. The aim of the study is to estimate the prevalence of anaemia in persons with type 2 diabetes mellitus and its role as a risk factor for the presence and the severity of microvascular complication in a populationbased study. MATERIALS AND METHODS This is a cross-sectional study conducted in patients coming to OPD of the Department of General Medicine in Government Vellore Medical College for a duration of 3 months from June 01, 2016, to August 31, 2016. Type 2 DM patients between the age group 20-60 years attending our diabetic clinic of both sex were included in our study. RESULTS From a total of 100 patients, 41% had anaemia including 34% with normochromic normocytic, 65.85% with hyperchromic microcytic anaemia and none of the patient had macrocytic anaemia. Patients who are anaemic had more frequent microvascular complications. There was no significant difference between males and females. The average duration of diabetes has a positive correlation with anaemia. All the microvascular complications like neuropathy, nephropathy and retinopathy had significant association with the presence of anaemia in type 2 patients. Nephropathy had a significant higher frequency compared to others as a complication in type 2 DM. CONCLUSION Our study shows that there is increased prevalence of anaemia in type 2 DM patients and the prevalence of microvascular complications is significantly higher among the diabetic patients with anaemia.

  4. Hematological outcome in neonatal alloimmune hemolytic disease

    NARCIS (Netherlands)

    Rath, Mirjam Eva Aafke

    2013-01-01

    This thesis focuses on several aspects related to the hematological outcome of infants with hemolytic disease of the fetus and newborn (HDFN) due to red blood cell alloimmunization, including pathogenesis and management of the disease. The presence of leukocytopenie and thrombocytopenia support the

  5. Fanconi anaemia and the repair of Watson and Crick DNA crosslinks.

    Science.gov (United States)

    Kottemann, Molly C; Smogorzewska, Agata

    2013-01-17

    The function of Fanconi anaemia proteins is to maintain genomic stability. Their main role is in the repair of DNA interstrand crosslinks, which, by covalently binding the Watson and the Crick strands of DNA, impede replication and transcription. Inappropriate repair of interstrand crosslinks causes genomic instability, leading to cancer; conversely, the toxicity of crosslinking agents makes them a powerful chemotherapeutic. Fanconi anaemia proteins can promote stem-cell function, prevent tumorigenesis, stabilize replication forks and inhibit inaccurate repair. Recent advances have identified endogenous aldehydes as possible culprits of DNA damage that may induce the phenotypes seen in patients with Fanconi anaemia.

  6. Autoimmune Hemolytic Anemia as a Complication of Nivolumab Therapy.

    Science.gov (United States)

    Palla, Amruth R; Kennedy, Devin; Mosharraf, Hossain; Doll, Donald

    2016-01-01

    Recently, immunotherapeutic drugs, including PD-1 inhibitors (nivolumab, pembrolizumab), PD-L1 inhibitors (atezolizumab, avelumab), and CTLA4 inhibitors (ipiliumumab), have emerged as important additions to the armamentarium against certain malignancies and have been incorporated into therapeutic protocols for first-, second-, or third-line agents for these metastatic cancers. Immune checkpoint inhibitor nivolumab is currently FDA approved for the treatment of patients with metastatic malignant melanoma [Redman et al.: BMC Med 2016;14: 20], metastatic non-small cell lung cancer [Guibert and Mazières: Expert Opin Biol Ther 2015;15: 1789-1797], metastatic renal cell cancer [Farolfi et al.: Expert Opin Drug Metab Toxicol 2016;12: 1089-1096], and relapsed or refractory classic Hodgkin's lymphoma [Villasboas and Ansell: Expert Rev Anticancer Ther 2016;16: 5-12]. Given the current and increasing indications for these drugs, it is essential for all physicians to become well versed with their common adverse effects and to be observant for other less documented clinical conditions that could be unmasked with the use of such medications. A definite association between autoimmune hemolytic anemia and the immune checkpoint inhibitor nivolumab has not been clearly documented, although a few cases have been reported recently [Kong et al.: Melanoma Res 2016;26: 202-204; Schwab et al.: Case Rep Oncol 2016;9: 373-378; Tardy et al.: Hematol Oncol 2016, DOI: 10.1002/hon.2338]. We report a case of fatal autoimmune hemolytic anemia refractory to steroids in a patient treated with nivolumab for metastatic lung cancer, and reflect on the other reported cases of autoimmune hemolytic anemia after the use of nivolumab.

  7. Autoimmune Hemolytic Anemia as a Complication of Nivolumab Therapy

    Directory of Open Access Journals (Sweden)

    Amruth R. Palla

    2016-11-01

    Full Text Available Recently, immunotherapeutic drugs, including PD-1 inhibitors (nivolumab, pembrolizumab, PD-L1 inhibitors (atezolizumab, avelumab, and CTLA4 inhibitors (ipiliumumab, have emerged as important additions to the armamentarium against certain malignancies and have been incorporated into therapeutic protocols for first-, second-, or third-line agents for these metastatic cancers. Immune checkpoint inhibitor nivolumab is currently FDA approved for the treatment of patients with metastatic malignant melanoma [Redman et al.: BMC Med 2016;14: 20], metastatic non-small cell lung cancer [Guibert and Mazières: Expert Opin Biol Ther 2015;15: 1789–1797], metastatic renal cell cancer [Farolfi et al.: Expert Opin Drug Metab Toxicol 2016;12: 1089–1096], and relapsed or refractory classic Hodgkin’s lymphoma [Villasboas and Ansell: Expert Rev Anticancer Ther 2016;16: 5–12]. Given the current and increasing indications for these drugs, it is essential for all physicians to become well versed with their common adverse effects and to be observant for other less documented clinical conditions that could be unmasked with the use of such medications. A definite association between autoimmune hemolytic anemia and the immune checkpoint inhibitor nivolumab has not been clearly documented, although a few cases have been reported recently [Kong et al.: Melanoma Res 2016;26: 202–204; Schwab et al.: Case Rep Oncol 2016;9: 373–378; Tardy et al.: Hematol Oncol 2016, DOI: 10.1002/hon.2338]. We report a case of fatal autoimmune hemolytic anemia refractory to steroids in a patient treated with nivolumab for metastatic lung cancer, and reflect on the other reported cases of autoimmune hemolytic anemia after the use of nivolumab.

  8. Advanced Prostate Cancer Presenting as Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    R. Ramos

    2013-01-01

    Full Text Available Introduction. Hemolytic uremic syndrome (HUS is characterized by endothelial dysfunction, consumption thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. HUS generally has a dismal prognosis, except when associated with gastroenteritis caused by verotoxin-producing bacteria. Cancer associated HUS is uncommon, and there are only scarce reports on prostate cancer presenting with HUS. Case Presentation. A 72-year-old man presented to the emergency department with oliguria, hematuria, and hematemesis. Clinical evaluation revealed acute renal failure, hemolysis, normal blood-clotting studies, and prostate-specific antigen value of 1000 ng/mL. The patient was started on hemodialysis, ultrafiltration with plasma exchange, and androgen blockade with bicalutamide and completely recovered from HUS. The authors review the 14 published cases on this association. Conclusion. The association of HUS and prostate cancer occurs more frequently in patients with high-grade, clinically advanced prostate cancer. When readily recognized and appropriately treated, HUS does not seem to worsen prognosis in prostate cancer patients.

  9. Anaemia and pregnancy: Anaesthetic implications

    Directory of Open Access Journals (Sweden)

    Anju Grewal

    2010-01-01

    Full Text Available Anaemia in pregnancy defined as haemoglobin (Hb level of < 10 gm/dL, is a qualitative or quantitative deficiency of Hb or red blood cells in circulation resulting in reduced oxygen (O 2 -carrying capacity of the blood. Compensatory mechanisms in the form of increase in cardiac output (CO, PaO 2 , 2,3 diphosphoglycerate levels, rightward shift in the oxygen dissociation curve (ODC, decrease in blood viscosity and release of renal erythropoietin, get activated to variable degrees to maintain tissue oxygenation and offset the decreases in arterial O 2 content. Parturients with concomitant medical diseases or those with acute ongoing blood losses may get decompensated, leading to serious consequences like right heart failure, angina or tissue hypoxemia in severe anaemia. Preoperative evaluation is aimed at assessing the severity and cause of anaemia. The concept of an acceptable Hb level varies with the underlying medical condition, extent of physiological compensation, the threat of bleeding and ongoing blood losses. The main anaesthetic considerations are to minimize factors interfering with O 2 delivery, prevent any increase in oxygen consumption and to optimize the partial pressure of O 2 in the arterial blood. Both general anaesthesia and regional anaesthesia can be employed judiciously. Monitoring should focus mainly on the adequacy of perfusion and oxygenation of vital organs. Hypoxia, hyperventilation, hypothermia, acidosis and other conditions that shift the ODC to left should be avoided. Any decrease in CO should be averted and aggressively treated.

  10. Maternal Anaemia and Neonatal Outcomes

    Directory of Open Access Journals (Sweden)

    I.A. Deswanto

    2013-11-01

    Full Text Available This cross-sectional study aims to determine whether maternal anaemia would affect birth outcome – birth weight and length – of the baby and compare this with that of non-anaemicmothers. We used secondary data from Puskesmas Kecamatan Ciracas medical records. Alldelivery records from October – November 2012 were collected and analysed; samples weredivided into two group: Group 1 included anaemic pregnant women and Group 2 non-anaemicpregnant women. Inclusion criteria was all pregnant women 16 years and older and a singletonpregnancy with a complete medical record. All women with a past history of preterm delivery,obstetrical complications or any medical illness, except anaemia, were excluded from the study inorder to control for the confounding factors. Out of one hundred and one records, 79 non anaemicsubjects and 22 anaemic subjects, were included for the analysis. No significant difference wasfound in both groups in terms of baby’s birth weight and birth length.Keywords: maternity anaemia, birth weight, birth length

  11. Anti-Mur as the most likely cause of mild hemolytic disease of the newborn.

    Science.gov (United States)

    Bakhtary, Sara; Gikas, Anastasia; Glader, Bertil; Andrews, Jennifer

    2016-05-01

    Although rare in the United States, anti-Mur is relatively common in Southeast Asia and has been reported to have clinical significance in Chinese and Taiwanese populations. The infant was full term and the second child of a Chinese mother and Vietnamese father, presenting with jaundice. He was clinically diagnosed with immune-mediated hemolytic anemia. The direct antiglobulin test indicated that the infant's red blood cells were coated only with anti-IgG. Anti-Mur was identified in the maternal serum and the neonate's plasma. The father was found to be positive for the Mur antigen. The cause of the infant's hemolytic anemia was determined to be most likely anti-Mur. Since anti-Mur is implicated in causing hemolytic disease of the newborn, it is important to recognize this antibody more commonly found in Asian patients in the United States as the Mur+ phenotype has a higher prevalence in this population. © 2016 AABB.

  12. Association between anaemia and infections (HIV, malaria and hookworm) among children admitted at Muhimbili National Hospital.

    Science.gov (United States)

    Magesa, A S; Magesa, P M

    2012-09-01

    Anaemia is the major cause of morbidity and mortality in paediatric age with much aetiology. The magnitude of childhood anaemia has been inadequately studied at Muhimbili National Hospital (MNH). The study was aimed at determining the frequency of anaemia and associated infections in patients admitted in general paediatric wards at MNH in Dar es Salaam. This was a descriptive cross-sectional study. This was conducted at MNH in general paediatric wards from 20th August, 2009 to 15th December, 2009. Patients, aged 1-84 months, consecutively admitted were recruited in the study. After informed verbal consent from the guardian or parent was obtained, information on demographic and clinical characteristics was collected from the parent or guardian. Physical examination and laboratory tests on blood ; stool samples for hookworm screening; blood slides for malaria parasites; Human Immunodeficiency Virus (HIV) screening; and blood peripheral smears were done on all subjects. Additional information was taken from medical files. Data management: The prevalence of anemia was determined as a percentage of all paediatric patients recruited during the time of data collection. All information was recorded using questionnaires and analysis was done using SPSS version 13.0. A p value of 1.0, p > 0.05). Anaemia in paediatric patients admitted at MNH is a disease of high public health importance in Dar es Salaam and may well carry a high burden in the rest of the country. Other risk factors of anaemia should be investigated with a goal of reducing the burden of anaemia.

  13. Pernicious anaemia and mucosal endocrine cell proliferation of the non-antral stomach.

    OpenAIRE

    Rode, J; Dhillon, A P; Papadaki, L; Stockbrügger, R; Thompson, R J; Moss, E; Cotton, P B

    1986-01-01

    There is a recognised association between pernicious anaemia and the development of gastric carcinoma, endocrine cell hyperplasia, and carcinoid tumour. Multiple endoscopic biopsies from the body mucosa of seven patients with pernicious anaemia showed small intestinal metaplasia with varying degrees of inflammation, fibrosis, and expansion of the lamina propria. Using conventional silver and lead stains, endocrine cells were inconspicuous. Staining for the general neural and neuroendocrine ma...

  14. Reporting new cases of anaemia in primary care settings in Crete, Greece: a rural practice study

    Directory of Open Access Journals (Sweden)

    Lionis Christos

    2012-04-01

    Full Text Available Abstract Background Early diagnosis of anaemia represents an important task within primary care settings. This study reports on the frequency of new cases of anaemia among patients attending rural primary care settings in Crete (Greece and to offer an estimate of iron deficiency anaemia (IDA frequency in this study group. Methods All patients attending the rural primary health care units of twelve general practitioners (GPs on the island of Crete for ten consecutive working days were eligible to participate in this study. Hemoglobin (Hb levels were measured by portable analyzers. Laboratory tests to confirm new cases of anaemia were performed at the University General Hospital of Heraklion. Results One hundred and thirteen out of 541 recruited patients had a low value of Hb according to the initial measurement obtained by the use of the portable analyzer. Forty five (45.5% of the 99 subjects who underwent laboratory testing had confirmed anaemia. The mean value of the Hb levels in the group with confirmed anaemia, as detected by the portable analyzer was 11.1 g/dl (95% Confidence Interval (CI from 10.9 to 11.4 and the respective mean value of the Hb levels obtained from the full blood count was 11.4 g/dl (95% CI from 11.2 to 11.7 (P = 0.01. Sixteen out of those 45 patients with anaemia (35.6% had IDA, with ferritin levels lower than 30 ng/ml. Conclusion Keeping in mind that this paper does not deal with specificity or sensitivity figures, it is suggested that in rural and remote settings anaemia is still invisible and point of care testing may have a place to identify it.

  15. Anti-hemolytic and anti-inflammatory activities of the methanolic extract of Solenostemon Monostachyus (P.Beauv.) Briq. leaves in 2-butoxyethanol-hemolytic induced rats

    Science.gov (United States)

    Osikoya, Iyanuoluwa Olubukola; Afolabi, Israel Sunmola; Rotimi, Solomon Oladapo; Okafor, Adaobi Mary-Joy

    2018-04-01

    Traditional medicine is largely used to sustain global health requirements. Determining the biological activities of Solenostemon monostachyus is essential to provide a platform for treating hemolytic diseases. The methanolic extract of the leaves was orally administered for 5 days at 150 mg/kg, 200 mg/kg and 250 mg/kg of body weight doses to determine concentration of tumor necrosis factor-alpha (TNF-α), and the activities of the heme oxygenase-1 (HO-1) and cyclooxygenase 2 (COX-2) of plasma in the kidney, spleen and liver of 2-butoxyethanol hemolytic-induced rats. A dose of 150 mg of extract/kg of body weight significantly increased (p<0.05) HO-1 in the kidney. COX-2 activity was significantly reduced (p<0.05) mainly in the kidney untreated hemolytic induced rats. All treatments significantly increased (p<0.05) TNF-α concentrations in the kidney and spleen. HO-1 gene expression was downregulated, indicating stress reduction in the liver, by an extract dose of 200 mg/kg of body weight and caffeic acid and was upregulated, indicating stress in the spleen, by an extract dose of 150-200 mg/kg of body weight. A dose of 200-250 mg of extract/kg of body weight resulted in relatively good anti-inflammatory properties, and may possess healing properties in patients with hemolytic related diseases.

  16. 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

  17. Hemolytic uremic syndrome after high dose chemotherapy with autologous stem cell support

    NARCIS (Netherlands)

    van der Lelie, H.; Baars, J. W.; Rodenhuis, S.; Van Dijk, M. A.; de Glas-Vos, C. W.; Thomas, B. L.; van Oers, R. H.; von dem Borne, A. E.

    1995-01-01

    BACKGROUND: Chemotherapy intensification may lead to new forms of toxicity such as hemolytic uremic syndrome. METHODS: Three patients are described who developed this complication 4 to 6 months after high dose chemotherapy followed by autologous stem cell support. The literature on this subject is

  18. Prevalence and nature of anaemia in a prospective, population-based sample of people with diabetes: Teesside anaemia in diabetes (TAD) study.

    Science.gov (United States)

    Jones, S C; Smith, D; Nag, S; Bilous, M T; Winship, S; Wood, A; Bilous, R W

    2010-06-01

    Anaemia occurs in 25% of people attending hospital diabetes clinics, but this may not be representative of all people with diabetes. We aimed to determine the prevalence of anaemia in a prospective population-based sample stratified by estimated glomerular filtration rate (eGFR) using the 4-point Modification of Diet in Renal Disease (MDRD) formula. All 7331 patients on our district register were stratified by eGFR. Seven hundred and thirty were approached by letter on two occasions. Two hundred and thirty-four (32%) returned questionnaires and blood samples. Responders (R), non-responders (NR) and the whole cohort (C) were similar: mean +/- sd age R 61.7 +/- 12.7 years; NR 61.3 +/- 15.1 years; C 61.8 +/- 14.2 years; diabetes duration R 8.8 +/- 8.6 years; NR 8.2 +/- 7.9 years; C 7.5 +/- 7.8 years, Type 1 diabetes R 10.1%, NR 10.8%, C 9.4%. Anaemia was defined using World Health Organization criteria: haemoglobin 60 ml/min per 1.73 m(2). Anaemia was as a result of erythropoietin deficiency in 34%, abnormal haematinics in 40% and was unexplained in 26% of patients. Five per cent of the patients had anaemia below the treatment threshold of 11 g/dl. The prevalence of unrecognized anaemia in population-based cohorts is lower than that in hospital-based studies. Current clinical surveillance in the UK is failing to detect anaemia in stage 3-5 chronic kidney disease (eGFR 60 ml/min per 1.73 m(2).

  19. Reação transfusional hiper-hemolítica em pacientes portadores de anemia falciforme: relato de dois casos Hyper-hemolytic transfusional reaction in sickle cell patients: two case reports

    Directory of Open Access Journals (Sweden)

    Cláudia C.S. Naufel

    2002-12-01

    increased frequency of transfusions to which these patients are submitted, knowledge of the main risks and an adequate diagnosis of the complications caused by transfusional therapy are of fundamental importance. An atypical form of transfusional reaction, denominated hyperhemolytic transfusional reaction was recently described in sickle cell anemia patients after the transfusion of apparently compatible hemacias. In this case, previous conditions can exacerbate the hemolytic condition and put the life of the patient at risk. The pathophysiological conditions of this disease are not yet understood well and the treatment consists of suspending transfusions, corticoid therapy and / or administration of immunoglobulin. The aim of this work is o present two case reports of hyperhemolytic transfusional reaction in sickle cell anemia patients.

  20. NON-INVASIVE MONITORING OF FOETAL ANAEMIA IN KELL SENSITIZED PREGNANCY.

    Science.gov (United States)

    Memon, Zaibunnisa; Sheikh, Sana Sadiq

    2015-01-01

    We report a case of Kell sensitized pregnancy with good neonatal outcome. Anti-K antibodies were detected in maternal serum in early pregnancy as a part of routine antibody screening test. The middle cerebral artery doppler monitoring and serial titers were carried out to screen for foetal anaemia. Despite of rising antibody titers, serial middle cerebral artery doppler was normal and did not showed foetal anaemia. The pregnancy was carried out till term and patient delivered at 37 weeks of pregnancy with no evidence of foetal anaemia. This case underlines the need of general screening on rare antibodies in all pregnant women and that non-invasive monitoring of foetal anaemia can be done with anti-k titers and middle cerebral artery Doppler.

  1. Prevalence & consequences of anaemia in pregnancy.

    Science.gov (United States)

    Kalaivani, K

    2009-11-01

    Prevalence of anaemia in India is among the highest in the world. Prevalence of anaemia is higher among pregnant women and preschool children. Even among higher income educated segments of population about 50 per cent of children, adolescent girls and pregnant women are anaemic. Inadequate dietary iron, folate intake due to low vegetable consumption, perhaps low B12 intake and poor bioavailability of dietary iron from the fibre, phytate rich Indian diets are the major factors responsible for high prevalence of anaemia. Increased requirement of iron during growth and pregnancy and chronic blood loss contribute to higher prevalence in specific groups. In India, anaemia is directly or indirectly responsible for 40 per cent of maternal deaths. There is 8 to 10-fold increase in MMR when the Hb falls below 5 g/dl. Early detection and effective management of anaemia in pregnancy can contribute substantially to reduction in maternal mortality. Maternal anaemia is associated with poor intrauterine growth and increased risk of preterm births and low birth weight rates. This in turn results in higher perinatal morbidity and mortality, and higher infant mortality rate. A doubling of low birth weight rate and 2 to 3 fold increase in the perinatal mortality rates is seen when the Hb is anaemia contributes to intergenerational cycle of poor growth in the offspring. Early detection and effective management of anaemia in pregnancy can lead to substantial reduction in undernutrition in childhood, adolescence and improvement in adult height.

  2. Hemolytic Uremic Syndrome-associated Encephalopathy Successfully Treated with Corticosteroids.

    Science.gov (United States)

    Hosaka, Takashi; Nakamagoe, Kiyotaka; Tamaoka, Akira

    2017-11-01

    The encephalopathy that occurs in association with hemolytic uremic syndrome (HUS), which is caused by enterohemorrhagic Escherichia coli (E. coli), has a high mortality rate and patients sometimes present sequelae. We herein describe the case of a 20-year-old woman who developed encephalopathy during the convalescent stage of HUS caused by E.coli O26. Hyperintense lesions were detected in the pons, basal ganglia, and cortex on diffusion-weighted brain MRI. From the onset of HUS encephalopathy, we treated the patient with methylprednisolone (mPSL) pulse therapy alone. Her condition improved, and she did not present sequelae. Our study shows that corticosteroids appear to be effective for the treatment of some patients with HUS encephalopathy.

  3. Hemolytic Uremic Syndrome: New Developments in Pathogenesis and Treatment

    Directory of Open Access Journals (Sweden)

    Olivia Boyer

    2011-01-01

    Full Text Available Hemolytic uremic syndrome is defined by the characteristic triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. In children, most cases of HUS are caused by Shiga-toxin-producing bacteria, especially Escherichia coli O157:H7. Common vehicles of transmission include ground beef, unpasteurized milk, and municipal or swimming water. Shiga-toxin-associated HUS is a main cause of acute renal failure in young children. Management remains supportive as there is at present no specific therapy to ameliorate the prognosis. Immediate outcome is most often favourable but long-term renal sequelae are frequent due to nephron loss. Atypical HUS represents 5% of cases. In the past 15 years, mutations in complement regulators of the alternative pathway have been identified in almost 60% of cases, leading to excessive complement activation. The disease has a relapsing course and more than half of the patients either die or progress to end-stage renal failure. Recurrence after renal transplantation is frequent.

  4. e-ENERCA: telemedicine platform for rare anaemias

    Directory of Open Access Journals (Sweden)

    Béatrice Gulbis

    2014-12-01

    Full Text Available The creation of a telemedicine, tele-expertise platform opens a new challenge within the European Network for Rare and Congenital Anaemias (ENERCA; www.enerca.org. This is a cornerstone in the field of rare anaemias, in which national expertise is usually scarce and a significant number of patients remain undiagnosed. Experts in rare diseases are specially needed of shared knowledge platforms offering the possibility of a faster and more accurate diagnosis and the availability of a better patients’ follow-up. The platform developed by e- ENERCA will be user friendly and intuitive so it will be used by the majority of professionals without requiring a specific formation. The idea of inter professional consultation is to bring medical experts together for collaborative involvement in activities that maximize the benefits and improvement in patient care.

  5. Severe Hemolytic Jaundice in a Neonate with a Novel COL4A1 Mutation.

    Science.gov (United States)

    Tomotaki, Seiichi; Mizumoto, Hiroshi; Hamabata, Takayuki; Kumakura, Akira; Shiota, Mitsutaka; Arai, Hiroshi; Haginoya, Kazuhiro; Hata, Daisuke

    2016-12-01

    We report our experience with a preterm infant with severe hemolytic jaundice who required exchange transfusion just after birth. The patient was negative for alloimmune hemolysis as a result of maternal-fetal blood type incompatibility, and tests for inherited defects in erythrocyte metabolism, membrane function, and hemoglobin synthesis were normal. We also performed a bone marrow examination, but could not identify the cause of hemolysis. The patient had several other complications, including porencephaly, epilepsy, elevated serum levels of creatine kinase, and persistent microscopic hematuria. Later, we detected a genetic mutation in COL4A1, which was recently found to be associated with hemolytic anemia. We therefore believe that all of the patient's clinical features, including hemolytic anemia, were due to the mutation in COL4A1. Genetic testing for COL4A1 mutations is recommended in neonates who exhibit hemolytic disease of unknown etiology, especially when other complications compatible with COL4A1-related disorders are present. Copyright © 2014. Published by Elsevier B.V.

  6. Pernicious anaemia and Campylobacter like organisms; is the gastric antrum resistant to colonisation?

    Science.gov (United States)

    Flejou, J F; Bahame, P; Smith, A C; Stockbrugger, R W; Rode, J; Price, A B

    1989-01-01

    Gastric biopsies from 86 patients with pernicious anaemia were examined for Campylobacter like organisms with particular attention to those showing an antral gastritis in addition to the usual pattern of body gastritis. All the patients had chronic atrophic gastritis in the body but Campylobacter like organisms were found at this site in only three patients. Thus the Type A pattern of gastritis (autoimmune) seen in patients with pernicious anaemia is only rarely associated with Campylobacter like organisms. Forty four of these patients had biopsies from body and antrum, 16 showed an antral gastritis of whom only one had Campylobacter like organism present. Twenty five of this latter group of patients were rebiopsied after five years. There was no change in the pattern of gastritis, and the same single patient remained colonised. The frequency of an antral gastritis in patients with pernicious anaemia was 36% yet the frequency of antral colonisation by Campylobacter like organisms was very low (6%). These results show that, as in the body, Campylobacter like organisms are not associated with gastritis when it occurs at this site in pernicious anaemia. The antral gastritis that may accompany body gastritis in pernicious anaemia seems more likely therefore to be an extension of primary type A body gastritis (autoimmune) rather than a secondary type B (chronic) gastritis and, it is argued, the antrum may exhibit resistance to colonisation. PMID:2920929

  7. ABO incompatibility hemolytic disease following exchange transfusion 96 newborn

    OpenAIRE

    Khatami S.F; Behjati SH.

    2007-01-01

    Background: ABO incompatibility hemolytic disease of the newborn is a common cause of clinical jaundice and causes two-thirds of the hemolytic disease in newborns. This study was undertaken to determine the frequency of ABO incompatibility hemolytic disease and its complications in newborns undergoing exchange transfusion.Methods: This prospective and descriptive study was performed in jaundiced newborn infants during a three-year period. Inclusion criteria were: maternal blood type O, newbor...

  8. Perioperative transfusion management in patients with sickle cell anaemia undergoing a total hip arthroplasty. Is there a role of red-cell exchange transfusion? A retrospective study in the CHU of Fort-de-France Martinique.

    Science.gov (United States)

    Ould Amar, K; Rouvillain, J-L; Loko, G

    2013-03-01

    We conducted a retrospective study to examine the optimal regimen of transfusion and whether preoperative transfusion is needed in patients with Sickle cell anaemia (SCA) undergoing a Total hip arthroplasty (THA). Then, we assessed the incidence of perioperative complications rates among patients assigned to different transfusion regimens to propose finally the safety transfusion protocol. Preoperative transfusions are usually given to reduce or prevent perioperative complications to SCA patients undergoing THA. There is no consensus however on the best regimen of transfusion. During the period of 2000 to 2010, 14 patients with SCA (sex-ratio 0.4) with a mean age of 36 years underwent 16 THA (primary or revision). Three groups were differentiated according preoperatively protocol transfusion. Group 1: exchange transfusion (EXT), group 2: simple transfusion (ST), group 3: no transfusion (NT). Overall, preoperative transfusion was performed in 43.7% of cases and complications rate was 50%. In the group 1 (EXT) including five patients (31%), severe complications occurred in four patients (80%). in the group 2, including two patients (12.5%), no complications were observed. In the group 3, including nine patients (56%), complications occurred in four procedures (44.5%), the half of them were haemolytic complications. Our results support the decision to transfuse, ST, preoperatively only if the patient is significantly below their steady-state haemoglobin (Hb) level. Transfusion can be used intraoperatively according Hb level and/or the blood loss volume. Exchange transfusion appeared mostly to be related to postoperative morbidity rates. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  9. Improving efficiency and value in health care. Intravenous iron management for anaemia associated with chronic kidney disease: linking treatment to an outpatient clinic, optimizing service provision and patient choice.

    Science.gov (United States)

    Bhandari, Sunil; Naudeer, Sarah

    2008-12-01

    The National Service Framework advocates correction of anaemia in patients with chronic kidney disease (CKD). Oral iron is insufficient, while intravenous (IV) supplementation replenishes and maintains iron stores. In Yorkshire numerous peripheral clinics exist to reduce travel for patients, but patients must travel to the main unit for IV iron therapy. Therefore an outpatient service in tandem with a routine clinic for administration of IV CosmoFer was created. To evaluate the feasibility and benefits of IV iron therapy in the outpatient clinic during active patient review for CKD patients. A cross-sectional study of patients attending for total dose IV iron (n = 57) at a peripheral clinic. Iron was administered and monitored according to protocol by one of the clinic nurses with medical staff available in the adjoining room. Haemoglobin, ferritin and renal function were recorded pre-infusion and after 4-6 months. Results are given as medians/means +/- standard error. A total of 76 IV infusions were carried out with no reported side effects or haemodynamic instability. Haemoglobin (median 10.9 vs. 11.3 g dL(-1), P = NS), creatinine and estimated glomerular filtration rate (eGFR) over the 6-month period remained stable. Serum ferritin rose significantly [80.9 +/- 6.2 vs. 186.4 +/- 18.2 g L(-1) (P Hospital time saved 380 day case bed hours, doctor hours saved 76 hours, and patient hours saved 3 hours/patient. Cost savings for TDI CosmoFer in peripheral clinic versus in centre therapy and versus sucrose, respectively, for 76 treatments was pound 5749.40 and pound 46,320.80 respectively. We have demonstrated, in a resource-limited service, the feasibility and cost-effectiveness of a management care pathway for patients with CKD, in a peripheral clinic, to receive total dose IV CosmoFer without disruption of a functioning renal clinic.

  10. The anaemia control model: Does it help nephrologists in therapeutic decision-making in the management of anaemia?

    Science.gov (United States)

    Bucalo, María Laura; Barbieri, Carlo; Roca, Susana; Ion Titapiccolo, Jasmine; Ros Romero, Maria Soledad; Ramos, Rosa; Albaladejo, Mercedes; Manzano, Diana; Mari, Flavio; Molina, Manuel

    2018-06-03

    Anaemia is common in haemodialysis patients and treating it with erythropoiesis-stimulating agents (ESAs) is complex due to many factors. To assess the usefulness of the Anaemia Control Model (ACM) in the treatment of anaemia in haemodialysis. ACM is a software that predicts the optimal dose of darbepoetin and iron sucrose to achieve target haemoglobin (Hb) and ferritin levels, and makes prescription suggestions. Study conducted in dialysis clinics lasting 18months with two intervention phases (IPs) with ACM (IP1, n:213; IP2, n:218) separated by a control phase (CP, n:219). The primary outcome was the percentage of Hb in range and the median dose of ESAs, and the secondary outcomes were transfusion, hospitalisation and cardiovascular events. Clinical and patient analyses were performed. Hb variability was assessed by the standard deviation (SD) of the Hb. We also analysed the patients with most of the suggestions confirmed (ACM compliant group). ACM increased the percentage of Hb in range: 80.9% in IP2, compared with 72.7% in the CP and reduced the intake of darbepoetin (IP1: 20 [70]; CP 30 [80] μg P=0.032) with less Hb fluctuation (0.91±0.49 in the CP to 0.82±0.37g/dl in IP2, P<0.05), improving in the ACM compliant group. The secondary outcomes decreased with the use of ACM. ACM helps to obtain better anaemia results in haemodialysis patients, minimising the risks of treatment with ESAs and reducing costs. Copyright © 2018 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  11. Púrpura trombocitopênica e anemia hemolítica auto-imune em pacientes internados com lúpus eritematoso sistêmico juvenil Trombocytopenic purpura and autoimmune hemolytic anemia in hospitalized patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Jochebed Kyoung Kim

    2007-02-01

    esplenectomia. CONCLUSÕES: o CHE isolado foi uma manifestação grave em pacientes internados com LESJ, habitualmente associado a uma doença ativa e sistêmica.OBJECTIVE: to evaluate the hematological involvement (HI in hospitalized patients with juvenile systemic lupus erythematosus (JSLE. METHODS: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança - University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl, reticulocytosis, increase in lactate dehydrogenase (LDH and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. RESULTS: HI occurred in 14 patients (18.9%, with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis. All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. CONCLUSIONS: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally

  12. Iron deficiency anaemia -a risk factor for febrile seizures in children

    International Nuclear Information System (INIS)

    Sherjil, A.; Saeed, Z.U.; Shehzad, S.; Amjad, R.

    2010-01-01

    Background: Iron deficiency anaemia and febrile seizures are two common diseases in children worldwide as well as in our country. Iron insufficiency is known to cause neurological symptoms like behavioural changes, poor attention span and learning deficits in children. Therefore, it may also be associated with other neurological disturbances like febrile seizures in children. Objective of our case-control study was to find association between iron deficiency anaemia and febrile seizures in children. Methods: This multicentre study was conducted in Department of Paediatrics HIT Hospital Taxila Cantt, Department of Paediatrics CMH Mangla and Department of Paediatrics POF Hospital Wah Cantt, from June 2008 to June 2010. Three hundred and ten children aged between 6 months to 6 years were included in the study. One hundred and fifty-seven children who presented with febrile seizures were our cases, while, 153 children who presented with febrile illnesses without seizures were recruited as controls. All patients were assessed for iron deficiency anaemia by measuring haemoglobin level, serum ferritin level, Mean Corpuscular Haemoglobin Concentration (MCHC) and Mean Corpuscular Volume (MCV). Patients with iron deficiency anaemia amongst controls and cases were documented. Percentages and Odds ratio were derived from the collected data. Results: 31.85% of cases (50 out of 157) had iron deficiency anaemia whereas, 19.6% of controls (30 out of 153) were found to have iron deficiency anaemia as revealed by low levels of haemoglobin level, serum ferritin level, Mean Corpuscular Haemoglobin Concentration and Mean Corpuscular Volume. Odds ratio was 1.93. Conclusion: Patients with febrile seizures are 1.93 times more likely to have iron deficiency anaemia compared to febrile patients without seizures. (author)

  13. Successful Management of a Rare Cause of Hemolytic Uremic Syndrome With Eculizumab in a Child.

    Science.gov (United States)

    Alparslan, Caner; Yavaşcan, Önder; Kasap Demir, Belde; Atmiş, Bahriye; Karabay Bayazit, Aysun; Leblebisatan, Göksel; Öncel, Elif P; Alaygut, Demet; Mutlubaş, Fatma; Aksu, Nejat

    2018-03-23

    Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. It very rarely coexists with acute lymphoblastic leukemia (ALL) emerging before, simultaneously, or after the diagnosis has been made, and management of the patient may be difficult. We present the case of a 7-year-old boy who was diagnosed with HUS and initially managed by hemodialysis (HD). Thereafter, HUS progressed, and neurological findings developed. The patient was treated with eculizumab, agressive blood pressure control, and antiepileptic drugs. At the fifth month of follow-up, the patient was diagnosed with acute B-cell lymphoblastic leukemia with fever, bone pain, hepatosplenomegaly, and pancytopenia. After initiation of ALL treatment, he had no episodes of HUS, despite cessation of eculizumab. In conclusion, eculizumab may be a treatment of choice to prevent further systemic damage in recurrent HUS episodes of patients with borderline changes in the bone marrow until ALL is constantly diagnosed.

  14. Hemolytic anemia caused by kinking of dacron grafts implanted in ...

    African Journals Online (AJOL)

    Background: Hemolytic anemia caused by a kinked Dacron graft is a rare complication after repair of acute aortic dissection. We present a case of hemolytic anemia due to kinking of previously implanted Dacron graft for ascending aorta dissection treated by surgery and replaced with new Dacron. Case Details: We report a ...

  15. Genetics Home Reference: atypical hemolytic-uremic syndrome

    Science.gov (United States)

    ... Kidney Diseases: Kidney Failure: Choosing a Treatment That's Right for You Educational Resources (6 links) Disease InfoSearch: Hemolytic uremic syndrome, atypical MalaCards: genetic atypical hemolytic-uremic syndrome Merck Manual Consumer Version: Overview of Anemia Merck Manual Consumer Version: ...

  16. A noninvasive method for the prediction of fetal hemolytic disease

    Directory of Open Access Journals (Sweden)

    E. N. Kravchenko

    2017-01-01

    Full Text Available Objective: to improve the diagnosis of fetal hemolytic disease.Subjects and methods. A study group consisted of 42 pregnant women whose newborn infants had varying degrees of hemolytic disease. The women were divided into 3 subgroups according to the severity of neonatal hemolytic disease: 1 pregnant women whose neonates were born with severe hemolytic disease (n = 14; 2 those who gave birth to babies with moderate hemolytic disease (n = 11; 3 those who delivered infants with mild hemolytic disease (n = 17. A comparison group included 42 pregnant women whose babies were born without signs of hemolytic disease. Curvesfor blood flow velocity in the middle cerebral artery were analyzed in a fetus of 25 to 39 weeks’ gestation.Results. The peak systolic blood flow velocity was observed in Subgroup 1; however, the indicator did not exceed 1.5 MoM even in severe fetal anemic syndrome. The fetal middle artery blood flow velocity rating scale was divided into 2 zones: 1 the boundary values of peak systolic blood flow velocity from the median to the obtained midscore; 2 the boundary values of peak systolic blood flow velocity of the obtained values of as high as 1.5 MoM.Conclusion. The value of peak systolic blood flow velocity being in Zone 2, or its dynamic changes by transiting to this zone can serve as a prognostic factor in the development of severe fetal hemolytic disease. 

  17. [Revised practice guideline 'Anaemia in midwifery practice'

    NARCIS (Netherlands)

    Beentjes, M.; Jans, S.M.P.J.

    2012-01-01

    The practice guideline of the Royal Dutch Organization of Midwives 'Anaemia in primary care midwifery practice' published in 2000, has recently been revised. The revised guideline takes physiological haemodilution during pregnancy into consideration and provides gestation specific reference values

  18. Report of a case with Hodgkin's lymphoma, tuberculosis and autoimmune hemolytic anemia

    OpenAIRE

    TUĞCU, Deniz; KEBUDİ, Rejin; ZÜLFİKAR, Bülent; AYAN, İnci; GÖRGÜN, Ömer; AĞAN, Mehmet; SOMER, Alper; AKINCI, Ferhan

    2007-01-01

    Tuberculosis has been described in association with malignancies including Hodgkin's disease (HD). In this article, a patient with diagnoses of H D, tuberculosis and hemolytic anemia is reported. Both tuberculosis and HD may present with similar symptoms and signs, and one of the diagnoses may be overlooked. The physicians should be aware of the simultaneous occurrence of both of these diseases when they are faced with initial therapeutic failure, during care of H D and tuberculosis patients.

  19. Four decades of stem cell transplantation for Fanconi anaemia in the Netherlands

    NARCIS (Netherlands)

    Smetsers, Stephanie E.; Smiers, Frans J.; Bresters, Dorine; Sonnevelt, Martine C.; Bierings, Marc B.

    2016-01-01

    This article presents the haematopoietic stem cell transplantation (SCT) results of the complete Dutch Fanconi anaemia (FA) patient cohort. Sixty-eight Dutch FA patients have been transplanted since 1972. In total, 63 (93%) patients engrafted, 54 after first SCT and 9 after second SCT. Fludarabine

  20. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2010-09-01

    Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

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

    International Nuclear Information System (INIS)

    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)

  2. Iron Refractory Iron Deficiency Anaemia: A Rare Cause of Iron Deficiency Anaemia

    LENUS (Irish Health Repository)

    McGrath, T

    2018-01-01

    We describe the case of a 17-month-old boy with a hypochromic microcytic anaemia, refractory to oral iron treatment. After exclusion of dietary and gastrointestinal causes of iron deficiency, a genetic cause for iron deficiency was confirmed by finding two mutations in the TMPRSS6 gene, consistent with a diagnosis of iron-refractory iron deficiency anaemia (IRIDA).

  3. Incidence of nutritional anaemia among the under five children attending Ahmed Gasim hospital

    International Nuclear Information System (INIS)

    Mohamed, Hager Elrasheed Ali

    1998-11-01

    A survey was carried out in Khartoum North Ahmed Gasim specialist Hospital for children to identify aetiological factors that lead to incidence of nutritional anaemia among children under under five years of age. The sample consists of 192 patients taken from the hospital wards (experimental group), and 60 healthy children taken from out patient vaccination department of same hospital. A questionnaire was used as a tool for collection data regarding children and their families with emphasis to general information, socio-economic information, dietary information, anthropometric information, medical history and laboratory investigations including haemoglobin, hematocrit (PCV)%, peripheral blood picture, serum ferritin, serum folate and serum B 12 . Results show no correlation between anaemia and age R(0.1048) p 1 2 deficiency. Some children affected had mixed deficiency anaemia (3.182). Iron deficiency without anaemia was common among healthy children (control) 22.8%. Some recommendations were set for the improvement of the existing situation e.g. health education, nutrition education with emphasis on intake of supplements and weaning diets rich in iron and folate. Follow up and surveillance program to compact nutritional anaemia should be adopted.(Author)

  4. [Anaemia in pregnancy and in the immediate postpartum period. Prevalence and risk factors in pregnancy and childbirth].

    Science.gov (United States)

    Urquizu I Brichs, Xavier; Rodriguez Carballeira, Mónica; García Fernández, Antonio; Perez Picañol, Emilio

    2016-05-20

    The objective of the study was to assess the prevalence of anaemia in the immediate postpartum period (48-72hours), determine the risk factors and the value of haemoglobin before birth to reduce postpartum anaemia. A prospective, observational and longitudinal study that included 1,426 women who delivered consecutively and agreed to participate in the study. Different variables, analytical, epidemiological, foetal and maternal symptoms were studied. The prevalence of anaemia in the postpartum period was 49.7%. The most important risk factors were antepartum anaemia and type of delivery. The types of delivery most influencing postpartum anaemia were, forceps (82.3%), the ventouse 67%, cesarean section (58,2%) and vaginal delivery (37.2%). In the multivariate study was found as the most important independent risk factors, the haemoglobin in the delivery day (OR 6.16, CI: 3.73 to 10.15) and instrumental delivery (OR: 4.61, CI: 3.44 to 6, 19). Other independent risk factors were haemoglobin in the third trimester, episiotomy and perineal tears, ethnicity, birth weight, parity and intra/postpartum complications. Anaemia in the immediate postpartum is a prevalent problem. The factors most associated postpartum anaemia were antepartum anaemia and instrumental delivery. If patients arrive at the day of delivery with haemoglobins≥12,6g/dl and were restricted to necessary instrumented deliveries and cesarean sections, episiotomies and we could avoid perineal tears we can decrease anaemia in the immediate postpartum period very significantly. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  5. Prevalence and socio-demographic factors associated with anaemia in pregnancy in a primary health centre in Rivers State, Nigeria

    Directory of Open Access Journals (Sweden)

    Geraldine U. Ndukwu

    2012-06-01

    Full Text Available Background: Anaemia, though a common problem in Nigeria, has not been adequately studied amongst pregnant women in primary health care facilities. Objective: This study is aimed at determining the prevalence of anaemia and sociodemographic factors associated with anaemia in pregnancy in a primary health centre in Rivers State, Nigeria. Methodology: This is a cross-sectional study carried out in a primary health centre. Association between variables was analysed using the Chi-square test. Results: Two hundred and twenty-seven pregnant women whose ages ranged from 16 to 40 years with a mean age of 26.8 ± 4.3 years were recruited for the study. The haemoglobin concentration ranged from 6 g/dL – 15 g/dL with a mean of 10.10 ± 1.27g/dL. A total of 142 (62.6% participants were anaemic. Anaemia was observed to be least prevalent in women within the extremes of reproductive age (≤ 20 years and 36–40 years. There was no statistically significant association between age, educational level and marital status (p > 0.05. The association of anaemia with social class was statistically significant (p = 0.000. Severe anaemia was not a common finding amongst the patients but it was significantly associated with educational status (p = 0.02 and socio-economic status (p = 0.03. Conclusion: The prevalence of anaemia amongst the pregnant participants in the primary health centre was high. Out of all the socio-demographic characteristics, only socio-economic status was significantly associated with anaemia. It is recommended that the socio-economic status of women should be enhanced in line with the Millennium Development Goals to prevent anaemia and to enhance pregnancy outcomes.

  6. Hydroxyurea therapy in UK children with sickle cell anaemia: A single-centre experience.

    Science.gov (United States)

    Phillips, Kate; Healy, Laura; Smith, Louise; Keenan, Russell

    2018-02-01

    Despite the demonstrated efficacy of hydroxyurea therapy, children with sickle cell anaemia in the UK are preferentially managed with supportive care or transfusion. Hydroxyurea is reserved for children with severe disease phenotype. This is in contrast to North America and other countries where hydroxyurea is widely used for children of all clinical phenotypes. The conservative UK practice may in part be due to concerns about toxicity, in particular marrow suppression with high doses, and growth in children. We monitored 37 paediatric patients with sickle cell anaemia who were treated with hydroxyurea at a single UK treatment centre. Therapy was well tolerated and mild transient cytopenias were the only toxicity observed. Comparative analysis of patients receiving ≥26 mg/kg/day versus hydroxyurea as a disease-modifying therapy, which we advocate for all children with sickle cell anaemia. © 2017 The Authors. Pediatric Blood & Cancer Published by Wiley Periodicals, Inc.

  7. Parvovirus B19-triggered acute hemolytic anemia and thrombocytopenia in a child with Evans syndrome

    Directory of Open Access Journals (Sweden)

    ELPIS MANTADAKIS

    2018-03-01

    Full Text Available Background: Human parvovirus B19 (HPV-B19 is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. Case report. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thromocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg. Conclusion: Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present with reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

  8. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl

    Directory of Open Access Journals (Sweden)

    Tahar Gargah

    2012-01-01

    Full Text Available The hemolytic uremic syndrome (HUS, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, represents one of the major causes of acute renal failure in infancy and childhood. The typical form occurring after an episode of diarrhea caused by Escherichia coli is the most frequent in children. Other microorganisms also may be responsible for HUS, such as Streptococcus pneumoniae, which causes more severe forms of the disease. We report an 8-month-old girl who presented with pneumonia and subsequently developed HUS. Renal biopsy showed characteristic lesion of thrombotic microangiopathy and extensive cortical necrosis. She was managed with peritoneal dialysis but did not improve and developed severe sepsis due to staphylococcal peritonitis, resulting in the death of the patient. Streptococcus pneumoniae-induced HUS is uncommon, but results in severe disease in the young. There is a high risk of these patients developing end-stage kidney disease in the long term.

  9. Parvovirus B19-triggered Acute Hemolytic Anemia and Thrombocytopenia in a Child with Evans Syndrome.

    Science.gov (United States)

    Zikidou, Panagiota; Grapsa, Anastassia; Bezirgiannidou, Zoe; Chatzimichael, Athanassios; Mantadakis, Elpis

    2018-01-01

    Human parvovirus B19 (HPV-B19) is the etiologic agent of erythema infectiosum, of transient aplastic crises in individuals with underlying chronic hemolytic disorders, and of chronic pure red cell aplasia in immunocompromised individuals. We describe a 14-year-old girl with long-standing Evans syndrome, who presented with severe anemia, reticulocytopenia and thrombocytopenia. A bone marrow aspirate revealed severe erythroid hypoplasia along with the presence of giant pronormoblasts, while serological studies and real-time PCR of whole blood were positive for acute parvovirus B19 infection. The patient was initially managed with corticosteroids, but both cytopenias resolved only after administration of intravenous gamma globulin 0.8g/kg. Acute parvovirus B19 infection should be suspected in patients with immunologic diseases, who present reticulocytopenic hemolytic anemia and thrombocytopenia. In this setting, intravenous gamma globulin is effective for both cytopenias.

  10. Hepcidin in chronic kidney disease : not an anaemia management tool, but promising as a cardiovascular biomarker

    NARCIS (Netherlands)

    van der Weerd, N. C.; Grooteman, M. P. C.; Nube, M. J.; ter Wee, P. M.; Swinkels, D. W.; Gaillard, C. A. J. M.

    Hepcidin is a key regulator of iron homeostasis and plays a role in the pathogenesis of anaemia of chronic disease. Its levels are increased in patients with chronic kidney disease (CKD) due to diminished renal clearance and an inflammatory state. Increased hepcidin levels in CKD patients are

  11. Hemolytic anemias during pregnancy and the reproductive years

    Energy Technology Data Exchange (ETDEWEB)

    Mintz, U.; Moohr, J.W.; Ultmann, J.E.

    1977-11-01

    Anemia is a common phenomenon in women during the reproductive years. In pregnancy, it is associated with an increased incidence of maternal-fetal morbidity and mortality. The approach to the investigation of anemic women suspected of having hemolytic anemia of either congenital or acquired etiology is the subject of this article. Various conditions in the pregnant women can have hematologic consequences for the newborn infant; these conditions include sensitization to fetal blood cells, infections, drug ingestion and the possession of genes for hereditary hemolytic disorders, which may be transmitted to the fetus. Because several forms of hemolytic anemias are hereditary or are caused by an altered gene, genetic consultation is important.

  12. Socio-Demographic and Maternal Factors in Anaemia in Pregnancy ...

    African Journals Online (AJOL)

    Erah

    Anaemia in pregnancy still causes significant maternal morbidity and mortality in the developing countries including ... Blood film of 74.5%, 15.7% and 11.8% anaemic women showed ...... is the primary cause of megaloblastic anaemia in.

  13. 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.

  14. Patterns of Perception of Causes and Prevalence of Anaemia at ...

    African Journals Online (AJOL)

    Patterns of Perception of Causes and Prevalence of Anaemia at Booking in Tropical ... of pregnant women attending antenatal clinic on common causes of anaemia. ... half (53.3%) knew its mode of transmission and 33.5% knew its prevention.

  15. ADAMTS13 Gene Mutations in Children with Hemolytic Uremic Syndrome

    Science.gov (United States)

    Choi, Hyoung Soo; Cheong, Hae Il; Kim, Nam Keun

    2011-01-01

    We investigated ADAMTS13 activity as well as the ADAMTS13 gene mutation in children with hemolytic uremic syndrome (HUS). Eighteen patients, including 6 diarrhea-negative (D-HUS) and 12 diarrhea-associated HUS (D+HUS) patients, were evaluated. The extent of von Willebrand factor (VWF) degradation was assayed by multimer analysis, and all exons of the ADAMTS13 gene were PCR-amplified using Taq DNA polymerase. The median and range for plasma activity of ADAMTS13 in 6 D-HUS and 12 D+HUS patients were 71.8% (22.8-94.1%) and 84.9% (37.9-119.9%), respectively, which were not statistically significantly different from the control group (86.4%, 34.2-112.3%) (p>0.05). Five ADAMTS13 gene mutations, including 2 novel mutations [1584+2T>A, 3941C>T (S1314L)] and 3 polymorphisms (Q448E, P475S, S903L), were found in 2 D-HUS and one D+HUS patients, which were not associated with deficiency of ADAMTS13 activity. Whether these mutations without reduced ADAMTS13 activity are innocent bystanders or predisposing factors in HUS remains unanswered. PMID:21488199

  16. Anticardiolipin antibodies in classic pediatric hemolytic-uremic syndrome: a possible pathogenic role.

    Science.gov (United States)

    Ardiles, L G; Olavarría, F; Elgueta, M; Moya, P; Mezzano, S

    1998-01-01

    Anticardiolipin (aCL) antibodies have been associated with thrombocytopenia, hemolytic anemia and an increased risk of thrombosis in different vascular locations, even in the absence of lupus. The classic hemolytic-uremic syndrome is a postinfectious acute renal failure characterized by hemolytic anemia, thrombocytopenia and the presence of widespread glomerular thrombosis in the kidney, with pathogenic mechanisms that remain to be identified. In order to establish the frequency of aCL antibodies in this syndrome and to identify a possible role in the pathogenesis and clinical manifestations, 17 patients were studied during the reactant phase of the disease looking for an association between the presence of aCL antibodies (isotypes IgG, IgA and IgM) and the main clinical variables of the syndrome. In 8 patients IgG aCL was present, 2 patients had IgM aCL, and 1 had IgA antibodies on the solid-phase ELISA aCL assays, but no association could be demonstrated with the clinical variables studied. Although it might correspond to an epiphenomenon related to the triggering intestinal infection, a pathogenic role cannot be discarded and additional studies should be performed.

  17. Prevalence of Anaemia in Pregnancy at Uthungulu Health district of ...

    African Journals Online (AJOL)

    Maternal anaemia is a risk factor for infant iron deficiency anaemia and, if left uncorrected, can be associated with adverse behavioural and cognitive development in children. The prevalence of anaemia in pregnancy is estimated at between 35% and 75% in sub-Saharan Africa. However, the area-specific health problems ...

  18. Anaemia in Pregnancy in Abia State University Teaching Hospital, Aba

    African Journals Online (AJOL)

    A prospective study of incidence of anaemia in pregnancy at Abia state University Teaching Hospital, Aba was conducted over a six-month period spanning from 31st January 2000 to 31st July 2000. The incidence of anaemia in pregnancy was 29%. The vast majority (97.6%) had mild anaemia. The result showed that most ...

  19. Estimation of serum concentration of parvovirus B19 DNA by PCR in patients with chronic anemia

    DEFF Research Database (Denmark)

    Hornsleth, A.; Carlsen, K. M.; Christensen, Laurids Siig

    1994-01-01

    Parvovirus B19 DNA was detected in serum samples from 10 out of 42 patients with chronic anaemia, the majority of whom suffered from aplastic anaemia, haemolytic anaemia, pure red cell anaemia or myelodysplastic syndrome. Nested PCR methods with sensitivities of 0.005-0.05 fg DNA were developed. ...

  20. Antimicrobial, hemolytic and thrombolytic activities of some new N ...

    African Journals Online (AJOL)

    . Hemolytic and thrombolytic activities were determined by measuring absorbance before and after incubation of blood cells with test compound. Results: Compound 9d strongly inhibited Bacillus subtilis and Escherichia coli with zone of ...

  1. Hemolytic activity of Trichomonas vaginalis and Tritrichomonas foetus

    Directory of Open Access Journals (Sweden)

    Geraldo A De Carli

    1996-02-01

    Full Text Available The hemolytic activity of live isolates and clones of Trichomonas vaginalis and Tritrichomonas foetus was investigated. The isolates were tested against human erythrocytes. No hemolytic activity was detected by the isolates of T. foetus. Whereas the isolates of T. vaginalis lysed erythrocytes from all human blood groups. No hemolysin released by the parasites could be detected. Our preliminary results suggest that hemolysis depend on the susceptibility of red cell membranes to destabilization and the intervention of cell surface receptors as a mechanism of the hemolytic activity. The mechanism could be subject to strain-species-genera specific variation of trichomonads. The hemolytic activity of T. vaginalis is not due to a hemolysin or to a product of its metabolism. Pretreatment of trichomonads with concanavalin A reduced levels of hemolysis by 40%.

  2. Fanconi anaemia in South Africa: Past present and future

    Directory of Open Access Journals (Sweden)

    C Feben

    2018-04-01

    Full Text Available Fanconi anaemia (FA is an inherited genetic disorder characterised by somatic anomalies, bone marrow failure and an increased predisposition to solid tumours and haematological malignancies. South African (SA black and Afrikaner individuals are at higher than average risk for this condition owing to genetic founder mutations in certain Fanconi-associated genes. This review explores the epidemiology, clinical presentation, diagnostic modalities and recommended care of affected patients, focusing on the founder population groups in SA. The early diagnosis of FA is important and provides improved opportunities for early intervention, but remains challenging.

  3. A thermolabile aldolase A mutant causes fever-induced recurrent rhabdomyolysis without hemolytic anemia.

    Directory of Open Access Journals (Sweden)

    Asmaa Mamoune

    2014-11-01

    Full Text Available Aldolase A deficiency has been reported as a rare cause of hemolytic anemia occasionally associated with myopathy. We identified a deleterious homozygous mutation in the ALDOA gene in 3 siblings with episodic rhabdomyolysis without hemolytic anemia. Myoglobinuria was always triggered by febrile illnesses. We show that the underlying mechanism involves an exacerbation of aldolase A deficiency at high temperatures that affected myoblasts but not erythrocytes. The aldolase A deficiency was rescued by arginine supplementation in vitro but not by glycerol, betaine or benzylhydantoin, three other known chaperones, suggesting that arginine-mediated rescue operated by a mechanism other than protein chaperoning. Lipid droplets accumulated in patient myoblasts relative to control and this was increased by cytokines, and reduced by dexamethasone. Our results expand the clinical spectrum of aldolase A deficiency to isolated temperature-dependent rhabdomyolysis, and suggest that thermolability may be tissue specific. We also propose a treatment for this severe disease.

  4. A case of atypical hemolytic uremic syndrome as an early manifestation of acute lymphoblastic leukemia

    Directory of Open Access Journals (Sweden)

    Dong Kyun Han

    2010-02-01

    Full Text Available Hemolytic uremic syndrome (HUS is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a tendency to relapse with a poorer prognosis. HUS has been reported to be associated with acute lymphoblastic leukemia (ALL in children. The characteristics and the mechanisms underlying this condition are largely unknown. In this study, we describe the case of an 11-year-old boy in whom the diagnosis of ALL was preceded by the diagnosis of atypical HUS. Thus, patients with atypical HUS should be diagnosed for the possibility of developing ALL.

  5. Hemolytic uremic syndrome and hypertensive crisis post dengue hemorrhagic fever: a case report

    Directory of Open Access Journals (Sweden)

    Mervin Tri Hadianto

    2011-12-01

    Full Text Available Hemolytic-uremic syndrome (HUS clinically manifests as acute renal failure, hemolytic anemia and thrombocytopenia. Acute renal failure with oliguria, hypertension, and proteinuria usually develops in affected patients.1,2 In children under 15 years of age, typical HUS occurs at a rate of 0.91 cases per 100,000 population.3 The initial onset of this disease usually happens in children below 3 years of age. Incidence is similar in boys and girls. Seasonal variation occurs, with HUS peaking in the summer and fall. In young children, spontaneous recovery is common. In adults, the probability of recovery is low when HUS is associated with severe hypertension.2

  6. Proteolytic activity and cooperative hemolytic effect of dermatophytes with different species of bacteria

    Science.gov (United States)

    Pakshir, K; Mohamadi, T; Khodadadi, H; Motamedifar, M; Zomorodian, K; Alipour, S; Motamedi, M

    2016-01-01

    Background and Purpose: Globally, dermatophytes are the most common filamentous group of fungi causing cutaneous mycoses. Dermatophytes were shown to secrete a multitude of enzymes that play a role in their pathogenesis. There is limited data on co-hemolytic (CAMP-like) effect of different bacterial species on dermatophyte species. In this study, we sought to the evaluate exoenzyme activity and co-hemolytic effect of four bacteria on clinical dermatophytes isolated from patients in Shiraz, Iran. Materials and Methods: A total of 84 clinical dermatophyte species were isolated from patients suffering dermatophytosis and identified by conventional methods. Hemolytic activity was evaluated with Columbia 5% sheep blood agar. Proteolytic activity was determined by plate clearance assay method, using gelatin 8% agar. CAMP-like factor was evaluated with four bacteria, namely, S. areus, S. saprophyticus, S. pyogenes, and S. agalactiae. Fisher's exact test was run for statistical analysis. Results: T. mentagrophytes was the most predominant agent (27 [32.1%]) followed by T. verrucosum(20 [23.8%]), T. tonsurans (10 [11.9%]), Microsporum canis (7 [8.3%]), T. rubrum (6 [7.1%]), E. floccosum (6 [7.1%]), M. gypseum (5 [6%]), and T. violaceum (3[3.6%]). The most common clinical area of dermatophytosis was the skin. All the isolates expressed the zone of incomplete alpha hemolysis. All the isolates had CAMP- positive reaction with S. aureus and the other bacteria were CAMP-negative. All the isolates expressed proteolytic activity and no significant differences were noted among diverse genera of dermatophytes and severities of proteolytic activity. Conclusion: This study indicated that hemolysin and proteolytic enzymes potentially play a role in dermatophyte pathogenesis and S. aureus could be considered as a main bacterium for creation of co-hemolytic effect in association with dermatophyte species. PMID:28959790

  7. Haemoglobin changes and risk of anaemia following treatment for uncomplicated falciparum malaria in sub-Saharan Africa.

    Science.gov (United States)

    Zwang, Julien; D'Alessandro, Umberto; Ndiaye, Jean-Louis; Djimdé, Abdoulaye A; Dorsey, Grant; Mårtensson, Andreas A; Karema, Corine; Olliaro, Piero L

    2017-06-23

    Anaemia is common in malaria. It is important to quantitate the risk of anaemia and to distinguish factors related to the natural history of disease from potential drug toxicity. Individual-patient data analysis based on nine randomized controlled trials of treatments of uncomplicated falciparum malaria from 13 sub-Saharan African countries. Risk factors for reduced haemoglobin (Hb) concentrations and anaemia on presentation and after treatment were analysed using mixed effect models. Eight thousand eight hundred ninety-seven patients (77.0% <5 years-old) followed-up through 28 days treated with artemisinin combination therapy (ACT, 90%, n = 7968) or non-ACT. At baseline, under 5's had the highest risk of anaemia (77.6% vs. 32.8%) and higher parasitaemia (43,938 μl) than older subjects (2784 μl). Baseline anaemia increased the risk of parasitological recurrence. Hb began to fall after treatment start. In under 5's the estimated nadir was ~35 h (range 29-48), with a drop of -12.8% from baseline (from 9.8 g/dl to 8.7 g/dl, p = 0.001); in under 15's, the mean Hb decline between day 0-3 was -4.7% (from 9.4 to 9.0 g/dl, p = 0.001). The degree of Hb loss was greater in patients with high pre-treatment Hb and parasitaemia and with slower parasite reduction rates, and was unrelated to age. Subsequently, Hb increased linearly (+0.6%/day) until day 28, to reach +13.8% compared to baseline. Severe anaemia (<5 g/dl, 2 per 1000 patients) was transient and all patients recovered after day 14, except one case of very severe anaemia associated with parasite recurrence at day 28. There was no systematic difference in Hb concentrations between treatments and no case of delayed anaemia. On presentation with acute malaria young children with high parasitaemia have the highest risk of anaemia. The majority of patients experience a drop in Hb while on treatment as early as day 1-2, followed by a linear increase through follow-up. The degree of the early Hb dip is

  8. Pathogenesis of chronic rhinosinusitis in patients affected by β-thalassemia major and sickle cell anaemia post allogenic bone marrow transplant.

    Science.gov (United States)

    Martino, F; Di Mauro, R; Paciaroni, K; Gaziev, J; Alfieri, C; Greco, L; Floris, R; Di Girolamo, S; Di Girolamo, M

    2018-03-01

    Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet. Therefore, the aim of this study was to investigate a possible increased risk of rhinosinusitis and the real pathogenetic mechanism of it, comparing these two hematological diseases using msCT, gold standard for paranasal sinuses evaluation. A retrospective analysis of 90 patients affected by β-thalassemia major or SCA (respectively 59 and 31) underwent allogeneic bone marrow transplantation (BMT), and 44 control subjects was performed. Both patient categories and control group have been subjected to hematological and radiological evaluation using 64-multidetector-row CT scanner without contrast injection. Statistical analysis reveals that patients of the two study groups exhibit a significantly increased risk of sinusitis in comparison with the normal controls (RR: 3.55 for β-thalassemic pediatric subjects; RR: 3.35 for SCA pediatric subjects). A significant difference (p < 0,5) was found between the β -thalassemic patients on the one side, and SCA and control group on the other side, with regard to the evaluation of the typical anatomic alteration of maxillary sinus: β-thalassemic children had significant increase in the bone thickness of anterior and lateral sinus walls and significant reduction in volume and density compared to SCA patients and control group, with normal conditions of these parameters. In these hematological patients, there is an increased incidence of sinonasal infections due their therapy-induced immunosuppression post transplantation. In

  9. 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)

  10. Bi-allelic silencing of the Fanconi anaemia gene FANCF in acute myeloid leukaemia.

    NARCIS (Netherlands)

    Tischkowitz, M; Ameziane, N.; Waisfisz, Q.; Winter, de J.P.; Harris, R; Taniguchi, T; Andrea, d' A; Hodgson, SV; Mathew, C.G.; Joenje, H.

    2003-01-01

    Fanconi anaemia (FA) is a chromosomal instability disorder associated with a high risk of acute myeloid leukaemia (AML). Previous work has shown that the AML cell line CHRF-288, derived from a sporadic AML-M7 patient, does not express FANCF protein and exhibits a cellular FA phenotype. We show that

  11. Clonal structure of Staphylococcus aureus colonizing children with sickle cell anaemia and healthy controls

    NARCIS (Netherlands)

    Schaumburg, F.; Biallas, B.; Alabi, A. S.; Grobusch, M. P.; Feugap, E. N.; Lell, B.; Mellmann, A.; Peters, G.; Kremsner, P. G.; Becker, K.; Adegnika, A. A.

    2013-01-01

    Children with sickle cell anaemia (SCA) might carry hospital-associated bacterial lineages due to frequent hospital stays and antibiotic treatments. In this study we compared Staphylococcus aureus from SCA patients (n=73) and healthy children (n=143) in a cross-sectional study in Gabon. S. aureus

  12. Iron dextran in the treatment of iron-deficiency anaemia of ...

    African Journals Online (AJOL)

    deficiency anaemia were randomly allocated to two treatment groups. Group A received the usual recommended dose of iron dextran (Imferon; Fisons) and group 8 received two-thirds of the recommended dose. A further 30 patients received oral iron ...

  13. Prevalence and socio-demographic factors associated with anaemia in pregnancy in a primary health centre in Rivers State, Nigeria

    Directory of Open Access Journals (Sweden)

    Geraldine U. Ndukwu

    2012-06-01

    Full Text Available Background: Anaemia, though a common problem in Nigeria, has not been adequatelystudied amongst pregnant women in primary health care facilities.Objective: This study is aimed at determining the prevalence of anaemia and sociodemographicfactors associated with anaemia in pregnancy in a primary health centre inRivers State, Nigeria.Methodology: This is a cross-sectional study carried out in a primary health centre. Associationbetween variables was analysed using the Chi-square test.Results: Two hundred and twenty-seven pregnant women whose ages ranged from 16 to40 years with a mean age of 26.8 ± 4.3 years were recruited for the study. The haemoglobinconcentration ranged from 6 g/dL – 15 g/dL with a mean of 10.10 ± 1.27g/dL. A total of142 (62.6% participants were anaemic. Anaemia was observed to be least prevalent inwomen within the extremes of reproductive age (≤ 20 years and 36–40 years. There was nostatistically significant association between age, educational level and marital status (p > 0.05.The association of anaemia with social class was statistically significant (p = 0.000. Severeanaemia was not a common finding amongst the patients but it was significantly associatedwith educational status (p = 0.02 and socio-economic status (p = 0.03.Conclusion: The prevalence of anaemia amongst the pregnant participants in the primaryhealth centre was high. Out of all the socio-demographic characteristics, only socio-economicstatus was significantly associated with anaemia. It is recommended that the socio-economicstatus of women should be enhanced in line with the Millennium Development Goals toprevent anaemia and to enhance pregnancy outcomes.

  14. Timing of umbilical cord-clamping and infant anaemia: the role of maternal anaemia.

    Science.gov (United States)

    Blouin, Brittany; Penny, Mary E; Maheu-Giroux, Mathieu; Casapía, Martín; Aguilar, Eder; Silva, Hermánn; Creed-Kanashiro, Hilary M; Joseph, Serene A; Gagnon, Anita; Rahme, Elham; Gyorkos, Theresa W

    2013-05-01

    Evidence from randomized controlled trials has shown that delayed cord-clamping is beneficial to infant iron status. The role of maternal anaemia in this relationship, however, has not been established. To determine the effect of maternal anaemia at delivery on the association between timing of umbilical cord-clamping and infant anaemia at 4 and 8 months of age. A cohort of pregnant women admitted to the labour room of Hospital Iquitos (Iquitos, Peru) and their newborns were recruited into the study during two time periods (18 May to 3 June and 6-20 July 2009). Between the two recruitment periods, the hospital's policy changed from early to delayed umbilical cord-clamping. Maternal haemoglobin levels were measured before delivery, and the time between delivery and cord-clamping was recorded at delivery for the entire cohort. Mother-infant pairs were followed-up at 4 (n = 207) and 8 months (n = 184) post partum. Infant haemoglobin levels were measured at follow-up visits. Data were analysed using logistic regression models. The prevalence of maternal anaemia (Hb anaemia was modified by the mother's anaemia status. Significant benefits of delayed cord-clamping in preventing anaemia were found in infants born to anaemic mothers at both 4 months (aOR = 0.59, 95% CI 0.36-0.99) and 8 months (aOR = 0.38, 95% CI 0.19-0.76) of age. The study contributes additional evidence in support of delayed cord-clamping. This intervention is likely to have most public health impact in areas with a high prevalence of anaemia during pregnancy.

  15. Incidence of nutritional anaemia among the under five children attending Ahmed Gasim hospital

    Energy Technology Data Exchange (ETDEWEB)

    Mohamed, Hager Elrasheed Ali [Home Science Department, Faculty of Education, University of Khartoum, Khartoum (Sudan)

    1998-11-01

    A survey was carried out in Khartoum North Ahmed Gasim specialist Hospital for children to identify aetiological factors that lead to incidence of nutritional anaemia among children under under five years of age. The sample consists of 192 patients taken from the hospital wards (experimental group), and 60 healthy children taken from out patient vaccination department of same hospital. A questionnaire was used as a tool for collection data regarding children and their families with emphasis to general information, socio-economic information, dietary information, anthropometric information, medical history and laboratory investigations including haemoglobin, hematocrit (PCV)%, peripheral blood picture, serum ferritin, serum folate and serum B{sup 12}. Results show no correlation between anaemia and age R(0.1048) p<0.148 and there was no significant difference between anaemia and sex p<(0.104). There is a high significant difference between control and experimental group for Hb and PCV (p<0.00). Aetiological factors related to nutritional anaemia are: poor intake as a supplement or weaning food was poor in iron and folate content. Weaning is sudden because of pregnancy, insufficient milk, mother or child illness and also other causes: low income and many mouths to feed, crowded and unhealthy living conditions, some families had latrines in their houses while others hadn`t. Drinking water is brought from a far distance. The most prevailing type of anaemia is iron deficiency about 26% followed by megaloblastic (10.69%). Which is due to folate deficiency (6.41%), folate and B{sup 1}2 deficiency. Some children affected had mixed deficiency anaemia (3.182). Iron deficiency without anaemia was common among healthy children (control) 22.8%. Some recommendations were set for the improvement of the existing situation e.g. health education, nutrition education with emphasis on intake of supplements and weaning diets rich in iron and folate. Follow up and surveillance program

  16. Hemolytic disease of the fetus and newborn caused by anti-E

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    Adiyyatu Sa′idu Usman

    2013-01-01

    Full Text Available Objective: Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother′s red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs. Case summary: We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion. Conclusion: There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.

  17. Iron deficiency anaemia in Sri Lanka

    International Nuclear Information System (INIS)

    Liyanage, K.D.C.E.

    1992-01-01

    The commonest cause of nutritional anaemia in the Sri Lankan population is iron deficiency. The diets of the population belonging to the lower socio-economic groups contain little food of animal origin. Thus, their diets are deficient in easily absorbable (haem) iron; and are also heavily cereal-based. Therefore interference in the absorption of dietary iron also occurs. Iron-deficiency anaemia is not restricted to the so-called ''vulnerable groups'' in Sri Lanka, however, their greater demands make the problem not only commoner but also more severe. Among pregnant and lactating women anaemia is often associated with folate deficiency. It must also be noted that the low availability of dietary iron is compounded in large population groups. Malaria, presently raging on an epidemic scale is also a major contributory factor to the incidence of anaemia. The purpose of this study was to examine the iron status of pre-school children and pregnant women; to establish normal levels of biochemical indices at different trimesters; to record the effect of iron supplementation during pregnancy; and to record the bioavailability of iron from weaning foods and common adult diets. 6 figs, 14 tabs

  18. prevalence of parasitaemia, anaemia and treatment outcomes

    African Journals Online (AJOL)

    User

    2 Johns Hopkins Bloomberg School of Public Health, Dept. of Population Family and ... anaemia among school children is high and warrants investment to reduce these levels. These ... nism for interventions but data are lacking to ... consented to their participation in the study, and their weights and temperatures were re-.

  19. Anaemia prevalence and factors associated with haemoglobin ...

    African Journals Online (AJOL)

    Information on social-clinical characteristics, cancer type and associated factors as well as haemoglobin level before and after radiation were obtained. The prevalence of anaemia was determined as a proportion and linear regression was used to determine factors associated with haemoglobin change. Results: A total of ...

  20. Anaemia in Apparently Healthy Adult Nigerians | Olayemi ...

    African Journals Online (AJOL)

    Background: There is a direct relationship between health and social position, especially between anaemia, level of education and social development. ... Among female students 69.4% had hypochromic red cells while all the male students had normochromic red cell; 75.0% of female students had mircrocytic red cells ...

  1. Anaemia management in cardio renal disease.

    Science.gov (United States)

    Silverberg, Donald S; Wexler, Dov; Iaina, Adrian; Schwartz, Doron

    2010-05-01

    Anaemia is common in congestive heart failure (CHF) and is associated with increased mortality, morbidity and progressive renal failure. The common causes of the anaemia are the associated renal failure and excessive cytokine production, both of which can cause depression of the erythropoietin (EPO) production in the kidney and depression of EPO response in bone marrow. The cytokines can also induce iron deficiency by increasing hepcidin production from the liver, which both reduces gastrointestinal iron absorption and reduces iron release from iron stores located in the macrophages and hepatocytes. Attempts to control this anaemia will have to consider the use of both erythropoiesis stimulating agents (ESA) as well as oral and, probably more importantly, intravenous (IV) iron. Studies of anaemia in CHF with ESA and oral or IV iron and even with IV iron alone have shown a positive effect on hospitalisation, fatigue and shortness of breath, cardiac and renal function, quality-of-life, exercise capacity and reduced beta natriuretic peptide and have not demonstrated an increase in cardiovascular damage related to therapy. Although some studies and meta-analyses have revealed improvement in these parameters others have not. Adequately powered long-term placebo-controlled studies of ESA and of IV iron in CHF are needed and are currently being carried out.

  2. Auto-immune Haemolytic Anaemia and Paroxys

    African Journals Online (AJOL)

    who presented with an acute auto-immune haemolytic anaemia. In addition to a persistently positive Coombs test, with specific red cell auto-antibodies, the acidified serum test and the sucrose haemolysis test were repeatedly positive. CASE REPORT. A 24-year-old Indian woman was admitted to hospital in. July 1969.

  3. 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

  4. Evaluation of Ferric and Ferrous Iron Therapies in Women with Iron Deficiency Anaemia

    Science.gov (United States)

    Berber, Ilhami; Erkurt, Mehmet Ali; Aydogdu, Ismet; Kuku, Irfan

    2014-01-01

    Introduction. Different ferric and ferrous iron preparations can be used as oral iron supplements. Our aim was to compare the effects of oral ferric and ferrous iron therapies in women with iron deficiency anaemia. Methods. The present study included 104 women diagnosed with iron deficiency anaemia after evaluation. In the evaluations performed to detect the aetiology underlying the iron deficiency anaemia, it was found and treated. After the detection of the iron deficiency anaemia aetiology and treatment of the underlying aetiology, the ferric group consisted of 30 patients treated with oral ferric protein succinylate tablets (2 × 40 mg elemental iron/day), and the second group consisted of 34 patients treated with oral ferrous glycine sulphate tablets (2 × 40 mg elemental iron/day) for three months. In all patients, the following laboratory evaluations were performed before beginning treatment and after treatment. Results. The mean haemoglobin and haematocrit increases were 0.95 g/dL and 2.62% in the ferric group, while they were 2.25 g/dL and 5.91% in the ferrous group, respectively. A significant difference was found between the groups regarding the increase in haemoglobin and haematocrit values (P < 0.05). Conclusion. Data are submitted on the good tolerability, higher efficacy, and lower cost of the ferrous preparation used in our study. PMID:25006339

  5. A Rare Association of Autoimmune Hemolytic Anemia with Gastric Adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Kavita Agrawal

    2017-01-01

    Full Text Available An 80-year-old male presented with dyspnea on exertion for at least two months. He also complained of progressive dysphagia and weight loss of 35 pounds over the last eight months. Initial blood tests showed hemoglobin of 6.1 g/dl, reticulocytes count of 19.7%, total bilirubin of 3.2 mg/dl, lactate dehydrogenase of 600 U/L, and haptoglobin of less than 8 mg/dl, and direct Coombs test was positive for warm immunoglobulin G. The impression was autoimmune hemolytic anemia (AIHA. The evaluation of dysphagia with esophagogastroduodenoscopy revealed a single irregular 4 cm malignant appearing ulcerated mass at the incisura angularis of the stomach. The mass was confirmed as adenocarcinoma on biopsy. Diagnostic laparoscopy was positive for malignant cells and he was diagnosed with stage IV adenocarcinoma of the stomach. Other extensive workup to determine the etiology of AIHA was negative (described in detail below. Surgery was deferred primarily due to metastasis of cancer. Initially, hemoglobin was stabilized by intravenous methylprednisolone, high dose immunoglobulins, and packed red blood cell transfusions. After a few weeks, hemoglobin started trending down again. The patient was weaned off steroids and paradoxically IgG-mediated autohemolysis was controlled with the initiation of palliative chemotherapy. Our case highlights a rare occurrence of AIHA in association with gastric adenocarcinoma.

  6. Fanconi anaemia with bilateral diffuse pulmonary arterio venous fistulae: a case report

    Directory of Open Access Journals (Sweden)

    Samarakoon Lasitha

    2012-03-01

    Full Text Available Abstract Background We report a patient with cytogenetically confirmed Fanconi anaemia with associated diffuse bilateral pulmonary arterio-venous fistulae. This is only the second reported case of diffuse pulmonary arterio-venous fistulae with Fanconi anaemia. Case Presentation A 16 year old Sri Lankan boy, with a cytogenetically confirmed Fanconi anaemia was admitted to University Medical Unit, National Hospital of Sri Lanka for further assessment and treatment. Both central and peripheral cyanosis plus clubbing were noted on examination. The peripheral saturation was persistently low on room air and did not improve with supplementary Oxygen. Contrast echocardiography failed to demonstrate an intra cardiac shunt but showed early crossover of contrast, suggesting the possibility of pulmonary arterio-venous fistulae. Computed tomography pulmonary angiogram was inconclusive. Subsequent right heart catheterisation revealed bilateral diffuse arterio-venous fistulae not amenable for device closure or surgical intervention. Conclusion To our knowledge, this is the second reported patient with diffuse pulmonary arterio-venous fistulae associated with Fanconi anaemia. We report this case to create awareness among clinicians regarding this elusive association. We recommend screening patients with Fanconi anaemia using contrast echocardiography at the time of assessment with transthoracic echocardiogram. Though universal screening may be impossible given the cost constraints, such screening should at least be performed in patients with clinical evidence of desaturation or when a therapeutic option such as haematopoietic stem cell transplantation is considered. Treatment of pulmonary arteriovenous fistulae would improve patient outcome as desaturation by shunting worsens the anaemic symptoms by reducing the oxygen carrying capacity of blood.

  7. Hemolytic uremic syndrome associated with Plasmodium vivax malaria successfully treated with plasma exchange

    Directory of Open Access Journals (Sweden)

    V S Keskar

    2014-01-01

    Full Text Available We report a case of hemolytic uremic syndrome (HUS in an adult patient with Plasmodium vivax malaria. The patient presented with worsening anemia, persistent thrombocytopenia and acute kidney injury. HUS was diagnosed based on the high serum lactate dehydrogenase, elevated reticulocyte count and presence of schistocytes on peripheral blood smear. Kidney biopsy showed features of thrombotic microangiopathy. Complete hematological remission was achieved after five sessions of therapeutic plasma exchange. Renal function partially recovered and stabilized at discharge. Vivax malaria, generally considered benign, may be rarely associated with HUS.

  8. The clinical and radiological features of Fanconi's anaemia pictorial review

    International Nuclear Information System (INIS)

    De Kerviler, E.; Guermazi, A.; Zagdanski, A.-M.; Gluckman, E.; Frija, J.

    2000-01-01

    Fanconi's anaemia is a severe refractory anaemia, associated with congenital malformations in approximately two-thirds of cases. Although these malformations may involve every organ system, suggestive dysmorphic features include growth retardation, radial ray deformities and urinary malformations. These malformations are not specific for Fanconi's anaemia, but should be recognized during pregnancy, or later in childhood, and suggest the possibility of inherited haematopoiesis disorders. De Kerviler, E. (2000)

  9. Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength.

    Directory of Open Access Journals (Sweden)

    Kizzy-Clara Cita

    Full Text Available To investigate the association between priapism in men with sickle cell anemia (SCA and hemorheological and hemolytical parameters.Fifty-eight men with SCA (median age: 38 years were included; 28 who had experienced priapism at least once during their life (priapism group and 30 who never experienced this complication (control group. Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion. Hematological and biochemical parameters were obtained through routine procedures. The Laser-assisted Optical Rotational Cell Analyzer was used to measure red blood cell (RBC deformability at 30 Pa (ektacytometry and RBC aggregation properties (laser backscatter versus time. Blood viscosity was measured at a shear rate of 225 s-1 using a cone/plate viscometer. A principal component analysis was performed on 4 hemolytic markers (i.e., lactate dehydrogenase (LDH, aspartate aminotransferase (ASAT, total bilirubin (BIL levels and reticulocyte (RET percentage to calculate a hemolytic index.Compared to the control group, patients with priapism exhibited higher ASAT (p = 0.01, LDH (p = 0.03, RET (p = 0.03 levels and hemolytic indices (p = 0.02. Higher RBC aggregates strength (p = 0.01 and lower RBC deformability (p = 0.005 were observed in patients with priapism compared to controls. After removing the hydroxycarbamide-treated patients, RBC deformability (p = 0.01 and RBC aggregate strength (p = 0.03 were still different between the two groups, and patients with priapism exhibited significantly higher hemolytic indices (p = 0.01 than controls.Our results confirm that priapism in SCA is associated with higher hemolytic rates and show for the first time that this complication is also associated with higher RBC aggregate strength and lower RBC deformability.

  10. Anaemia in pregnancy is associated with advanced HIV disease.

    Science.gov (United States)

    Nandlal, Vikesh; Moodley, Dhayendre; Grobler, Anneke; Bagratee, Jayanthilall; Maharaj, Niren R; Richardson, Paul

    2014-01-01

    Anaemia is a common clinical finding in HIV infected women and has been associated with advanced disease. The use of antiretroviral drugs such as Zidovudine (ZDV) either for prevention of mother to child transmission (MTCT) of HIV or used in combination with other antiretrovirals have been implicated in the development or increased severity of anaemia. We report the prevalence, type, severity and incidence of anaemia in a cohort of HIV infected women who initiated antiretroviral prophylaxis or treatment during pregnancy. This is a retrospective cohort data analysis of 408 HIV infected pregnant women who participated in a breastfeeding intervention study (HPTN 046 Study, ClinicalTrials.gov NCT 00074412) in South Africa. Women initiated zidovudine prophylaxis for PMTCT or triple antiretroviral treatment in pregnancy according to the standard of care. Laboratory and clinical data in pregnancy, anaemia (Hbpregnancy, 48/146 (32.9%) subsequently developed anaemia intrapartum or postpartum and 89/310 (28.7%) of all cases of anaemia remained unresolved by 2 weeks postdelivery. In a univariate analysis, CD4 count and gravidity were significant risk factors for anaemia in pregnancy, RR 1.41; 1.23-1.61 (panaemia in pregnancy and postdelivery. In conclusion, anaemia was most common among women in the advanced stage of HIV infection (CD4anaemia.

  11. Determinants of anaemia among pregnant women in rural Uganda.

    Science.gov (United States)

    Mbule, Marjorie A; Byaruhanga, Yusuf B; Kabahenda, Magaret; Lubowa, Abdulrahman

    2013-01-01

    In spite of intervention efforts, in Uganda, as in other developing countries, high levels of anaemia among pregnant women continue. Anaemia among women of reproductive age (15-49 years) is a matter of national concern. This study was carried out to assess determinants of anaemia in Kiboga district. This was a single cross-sectional, descriptive survey. The anaemia status of the pregnant women was determined by measuring their haemoglobin levels. Possible determinant factors including socio-economic characteristics, knowledge, attitudes, practices and food intake were assessed using a structured questionnaire. Results showed that the prevalence of anaemia among pregnant women in Kiboga district was high enough (63.1%) to be described as a severe public health problem. The uptake and utilisation of the public-health intervention package to combat anaemia in pregnancy was low, with iron/folic acid supplementation at 13.2%, use of intermittent preventive treatment of malaria 45.4%, and use of de-worming medicines 14.5%. Women from households without a functional radio were 2.07 times more likely be anaemic (95%CI, 1.08-3.00) compared with women from households where there was a functional radio. There was little awareness and functional knowledge about anaemia among pregnant women. The high prevalence of anaemia observed in Kiboga district can be attributed to poverty and limited access to nutrition and health education information which lead to low uptake and utilization of the public-health intervention package to combat anaemia in pregnancy.

  12. Immunoglobulin for alloimmune hemolytic disease in neonates.

    Science.gov (United States)

    Zwiers, Carolien; Scheffer-Rath, Mirjam Ea; Lopriore, Enrico; de Haas, Masja; Liley, Helen G

    2018-03-18

    Exchange transfusion and phototherapy have traditionally been used to treat jaundice and avoid the associated neurological complications. Because of the risks and burdens of exchange transfusion, intravenous immunoglobulin (IVIg) has been suggested as an alternative therapy for alloimmune hemolytic disease of the newborn (HDN) to reduce the need for exchange transfusion. To assess the effect and complications of IVIg in newborn infants with alloimmune HDN on the need for and number of exchange transfusions. We performed electronic searches of CENTRAL, PubMed, Embase (Ovid), Web of Science, CINAHL (EBSCOhost), Academic Search Premier, and the trial registers ClinicalTrials.gov and controlled-trials.com in May 2017. We also searched reference lists of included and excluded trials and relevant reviews for further relevant studies. We considered all randomized and quasi-randomized controlled trials of IVIg in the treatment of alloimmune HDN. Trials must have used predefined criteria for the use of IVIg and exchange transfusion therapy to be included. We used the standard methods of Cochrane and its Neonatal Review Group. We assessed studies for inclusion and two review authors independently assessed quality and extracted data. We discussed any differences of opinion to reach consensus. We contacted investigators for additional or missing information. We calculated risk ratio (RR), risk difference (RD) and number needed to treat for an additional beneficial outcome (NNTB) for categorical outcomes. We calculated mean difference (MD) for continuous variables. We used GRADE criteria to assess the risk of bias for major outcomes and to summarize the level of evidence. Nine studies with 658 infants fulfilled the inclusion criteria. Term and preterm infants with Rh or ABO (or both) incompatibility were included. The use of exchange transfusion decreased significantly in the immunoglobulin treated group (typical RR 0.35, 95% CI 0.25 to 0.49; typical RD -0.22, 95% CI -0.27 to

  13. Intravenous iron isomaltoside 1000 (Monofer®) reduces postoperative anaemia in preoperatively non-anaemic patients undergoing elective or subacute coronary artery bypass graft, valve replacement or a combination thereof: a randomized double-blind placebo-controlled clinical trial (the PROTECT trial).

    Science.gov (United States)

    Johansson, P I; Rasmussen, A S; Thomsen, L L

    2015-10-01

    This trial explores whether intravenous iron isomaltoside 1000 (Monofer®) results in a better regeneration of haemoglobin levels and prevents anaemia compared to placebo in preoperative non-anaemic patients undergoing cardiac surgery. The trial is a prospective, double-blind, comparative, placebo-controlled trial of 60 non-anaemic patients undergoing cardiac surgery. The patients were randomized 1:1 to either 1000 mg intravenous iron isomaltoside 1000 administered perioperatively by infusion or placebo. Mean preoperative haemoglobin in the active treatment group was 14·3 g/dl vs. 14·0 g/dl in the placebo group. At discharge 5 days after surgery, haemoglobin levels were reduced to 10·7 and 10·5 g/dl, respectively. One month after surgery, haemoglobin concentration had increased to an average of 12·6 g/dl vs. 11·8 g/dl (p = 0·012) and significantly more patients were non-anaemic in the intravenous iron isomaltoside 1000-treated group compared to the placebo group (38·5% vs. 8·0%; p = 0·019). There were no differences in side-effects between the groups. A single perioperative 1000 mg dose of intravenous iron isomaltoside 1000 significantly increased the haemoglobin level and prevented anaemia 4 weeks after surgery, with a short-term safety profile similar to placebo. Future trials on potential clinical benefits of preoperative treatment with intravenous iron in non-anaemic patients are needed. © 2015 The Authors ISBT Science Series published by John Wiley & Sons Ltd on behalf of International Society of Blood Transfusion.

  14. The Bacterial Overgrowth Syndrome is Uncommon in Pernicious Anaemia: Results of a Follow-up Study

    OpenAIRE

    Stockbrügger, R. W.; Armbrecht, U.; Rode, J. W.; Teall, A. J.; Oberholzer, V. G.; Croker, J. R.; Cotton, P. B.

    2011-01-01

    It is still uncertain whether upper gastrointestinal bacterial overgrowth in patients with permanent achlorhydria causes malassimilation in more than just the occasional case. In an attempt to clarify this, 19 patients with pernicious anaemia who had undergone a thorough investigation 6.6 y (mean) previously, were reinvestigated with clinical history, upper GI endoscopy including multiple duodenal biopsies, microbial cultures of gastric juice and duodenal mucosa, a xylose absorption test, and...

  15. Gastric antral vascular ectasia--a cause of refractory anaemia in systemic sclerosis.

    LENUS (Irish Health Repository)

    Busteed, S

    2012-02-03

    Recurrent gastrointestinal haemorrhage is an uncommon manifestation of systemic sclerosis. We report a case of gastrointestinal bleeding due to gastric antral vascular ectasia (GAVE) in a patient with systemic sclerosis. Failure to recognise the condition as a cause of gastrointestinal bleeding may delay the instigation of appropriate treatment. GAVE should be considered in the differential diagnosis of anaemia in patients with autoimmune conditions such as systemic sclerosis and primary biliary cirrhosis.

  16. Evaluation of Neonatal Hemolytic Jaundice: Clinical and Laboratory Parameters

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    Anet Papazovska Cherepnalkovski

    2015-12-01

    CONCLUSIONS: The laboratory profile in ABO/Rh isoimmunisation cases depicts hemolytic mechanism of jaundice. These cases carry a significant risk for early and severe hyperbilirubinemia and are eligible for neurodevelopmental follow-up. Hematological parameters and blood grouping are simple diagnostic methods that assist the etiological diagnosis of neonatal hyperbilirubinemia.

  17. Atypical relapse of hemolytic uremic syndrome after transplantation

    NARCIS (Netherlands)

    Olie, Karolien H.; Florquin, Sandrine; Groothoff, Jaap W.; Verlaak, René; Strain, Lisa; Goodship, Timothy H. J.; Weening, Jan J.; Davin, Jean-Claude

    2004-01-01

    Atypical hemolytic uremic syndrome (HUS) frequently leads to end-stage renal failure and can relapse after transplantation. A 12-year-old girl presenting with familial atypical HUS with a factor H mutation was successfully transplanted 6 years after a first transplant that had failed because of

  18. Predictors of Persistent Anaemia in the First Year of Antiretroviral Therapy: A Retrospective Cohort Study from Goma, the Democratic Republic of Congo.

    Directory of Open Access Journals (Sweden)

    Pierre Zalagile Akilimali

    Full Text Available Anaemia is associated with adverse outcomes including early death in the first year of antiretroviral therapy (ART. This study reports on the factors associated with persistent anaemia among HIV-infected patients initiating ART in the Democratic Republic of Congo (DR Congo.We conducted a retrospective cohort study and analyzed data from patients receiving HIV care between January 2004 and December 2012 at two major hospitals in Goma, DR Congo. Haemoglobin concentrations of all patients on ART regimen were obtained prior to and within one year of ART initiation. A logistic regression model was used to identify the predictors of persistent anaemia after 12 months of ART.Of 756 patients, 69% of patients were anaemic (IC95%: 65.7-72.3 at baseline. After 12 months of follow up, there was a 1.2 g/dl average increase of haemoglobin concentration (P < 0.001 with differences depending on the therapeutic regimen. Patients who received zidovudine (AZT gained less than those who did not receive AZT (0.99 g/dl vs 1.33 g/dl; p< 0.001. Among 445 patient who had anaemia at the beginning, 33% (147/445 had the condition resolved. Among patients with anaemia at ART initiation, those who did not receive cotrimoxazole prophylaxis before starting ART(AOR 3.89; 95% CI 2.09-7.25; P < 0.001 and a AZT initial regimen (AOR 2.19; 95% CI 1.36-3.52; P < 0.001 were significantly at risk of persistent anaemia.More than two thirds of patients had anaemia at baseline. The AZT-containing regimen and absence of cotrimoxazole prophylaxis before starting ART were associated with persistent anaemia 12 months, after initiation of treatment. Considering the large proportion of patients with persistence of anaemia at 12 months, we suggest that it is necessary to conduct a large study to assess anaemia among HIV-infected patients in Goma.

  19. Eculizumab Therapy Leads to Rapid Resolution of Thrombocytopenia in Atypical Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    Han-Mou Tsai

    2014-01-01

    Full Text Available Eculizumab is highly effective in controlling complement activation in patients with the atypical hemolytic uremic syndrome (aHUS. However, the course of responses to the treatment is not well understood. We reviewed the responses to eculizumab therapy for aHUS. The results show that, in patients with aHUS, eculizumab therapy, when not accompanied with concurrent plasma exchange therapy, led to steady increase in the platelet count and improvement in extra-renal complications within 3 days. By day 7, the platelet count was normal in 15 of 17 cases. The resolution of hemolytic anemia and improvement in renal function were less predictable and were not apparent for weeks to months in two patients. The swift response in the platelet counts was only observed in one of five cases who received concurrent plasma exchange therapy and was not observed in a case of TMA due to gemcitabine/carboplatin. In summary, eculizumab leads to rapid increase in the platelet counts and resolution of extrarenal symptoms in patients with aHUS. Concurrent plasma exchange greatly impedes the response of aHUS to eculizumab therapy. Eculizumab is ineffective for gemcitabine/carboplatin associated TMA.

  20. Radiosensitivity in Fanconi anaemia: application to the conditioning regimen for bone marrow transplantation

    International Nuclear Information System (INIS)

    Gluckman, E.; Devergie, A.; Dutreix, J.

    1983-01-01

    Fanconi anaemia, an autosomal recessive constitutional aplastic anaemia, seems to be related to a DNA repair mechanism defect. Bone marrow transplantation is the only treatment which can cure these patients. Previous attempts at BMT have shown an increased sensitivity to Cyclophosphamide used for the conditioning. Such a sensitivity has also been observed in vitro when Fanconi anaemia cells were incubated with alkylating agents. We have tested the in vivo radiosensitivity and cell repair after skin contact radiotherapy to calculate the irradiation dose which could be tolerated by FA patients. Eight patients have been tested and the results confirmed the suspected increased radiosensitivity in the majority of patients. Following these results, four patients were conditioned with low dose Cyclophosphamide (20 mg/kg) associated with 5 Grays thoraco-abdominal irradiation. all had a take and no major complication of the conditioning regimen. All are alive in good condition from day 51 to day 330 after transplant. Oesophagitis was one major unexpected complication. This study confirms the possibility of curing FA patients with BMT when the conditioning regimen is modified according to the pathophysiology of the disease. (author)

  1. Radiosensitivity in Fanconi anaemia: application to the conditioning regimen for bone marrow transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Gluckman, E.; Devergie, A. (Hopital Saint-Louis, 75 - Paris (France)); Dutreix, J. (Institut Gustave Roussy, 94 - Villejuif (France))

    1983-07-01

    Fanconi anaemia, an autosomal recessive constitutional aplastic anaemia, seems to be related to a DNA repair mechanism defect. Bone marrow transplantation is the only treatment which can cure these patients. Previous attempts at BMT have shown an increased sensitivity to Cyclophosphamide used for the conditioning. Such a sensitivity has also been observed in vitro when Fanconi anaemia cells were incubated with alkylating agents. We have tested the in vivo radiosensitivity and cell repair after skin contact radiotherapy to calculate the irradiation dose which could be tolerated by FA patients. Eight patients have been tested and the results confirmed the suspected increased radiosensitivity in the majority of patients. Following these results, four patients were conditioned with low dose Cyclophosphamide (20 mg/kg) associated with 5 Grays thoraco-abdominal irradiation. All had a take and no major complication of the conditioning regimen. All are alive in good condition from day 51 to day 330 after transplant. Oesophagitis was one major unexpected complication. This study confirms the possibility of curing FA patients with BMT when the conditioning regimen is modified according to the pathophysiology of the disease.

  2. Intravenous iron vs blood for acute post-partum anaemia (IIBAPPA): a prospective randomised trial.

    Science.gov (United States)

    Chua, Seng; Gupta, Sarika; Curnow, Jennifer; Gidaszewski, Beata; Khajehei, Marjan; Diplock, Hayley

    2017-12-19

    Acute post-partum anaemia can be associated with significant morbidity including a predisposition for postnatal depression. Lack of clear practice guidelines means a number of women are treated with multiple blood transfusions. Intravenous iron has the potential to limit the need for multiple blood transfusions but its role in the post-partum setting is unclear. IIBAPPA is a multi-centre randomised non-inferiority trial. Women with a primary post-partum haemorrhage (PPH) >1000 mL and resultant haemoglobin (Hb) 5.5-8.0 g/dL after resuscitation with ongoing symptomatic anaemia who are otherwise stable (no active bleeding) are eligible to participate. Patients with sepsis or conditions necessitating rapid Hb restoration are excluded. Eligible participants are randomised to receive a blood transfusion or a single dose of intravenous iron polymaltose calculated using the Ganzoni formula. Primary outcome measures include Hb, Ferritin and C-Reactive Protein levels on Day 7. Secondary outcomes evaluate (i) Hb, Ferritin and CRP levels on Day 14, 28, (ii) anaemia symptoms on Day 0, 7, 14 and 28 using structured health related quality of life questionnaires, (iii) treatment safety by assessing adverse reactions and infection endpoints and (iv) the quantitative impact of anaemia on breast feeding quality using a hospital designed questionnaire. If equivalence in Hb and ferritin levels, symptom scores and safety endpoints is demonstrated, intravenous iron may become the preferred treatment for women with acute post-partum anaemia to minimise transfusion reactions and costs. Australian and New Zealand Clinical Trials Registry: ACTRN12615001370594 on 16th December, 2015 (prospective approval).

  3. In vitro assessment of recombinant, mutant immunoglobulin G anti-D devoid of hemolytic activity for treatment of ongoing hemolytic disease of the fetus and newborn

    DEFF Research Database (Denmark)

    Nielsen, Leif K; Green, Trine H; Sandlie, Inger

    2008-01-01

    A specific treatment for ongoing hemolytic disease of the fetus and newborn (HDFN) due to anti-D would be very attractive. One approach could be administration to the mother of nonhemolytic anti-D, which by crossing the placenta can block the binding of hemolytic maternal anti-D.......A specific treatment for ongoing hemolytic disease of the fetus and newborn (HDFN) due to anti-D would be very attractive. One approach could be administration to the mother of nonhemolytic anti-D, which by crossing the placenta can block the binding of hemolytic maternal anti-D....

  4. 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.

    Science.gov (United States)

    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.

  5. Recent advances in understanding clonal haematopoiesis in aplastic anaemia.

    Science.gov (United States)

    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.

  6. A Fatal Case of Severe Hemolytic Disease of Newborn Associated with Anti-Jkb

    Science.gov (United States)

    Kim, Won Duck

    2006-01-01

    The Kidd blood group is clinically significant since the Jk antibodies can cause acute and delayed transfusion reactions as well as hemolytic disease of newborn (HDN). In general, HDN due to anti-Jkb incompatibility is rare and it usually displays mild clinical symptoms with a favorable prognosis. Yet, we apparently experienced the second case of HDN due to anti-Jkb with severe clinical symptoms and a fatal outcome. A female patient having the AB, Rh(D)-positive boodtype was admitted for jaundice on the fourth day after birth. At the time of admission, the patient was lethargic and exhibited high pitched crying. The laboratory data indicated a hemoglobin value of 11.4 mg/dL, a reticulocyte count of 14.9% and a total bilirubin of 46.1 mg/dL, a direct bilirubin of 1.1 mg/dL and a strong positive result (+++) on the direct Coomb's test. As a result of the identification of irregular antibody from the maternal serum, anti-Jkb was detected, which was also found in the eluate made from infant's blood. Despite the aggressive treatment with exchange transfusion and intensive phototherapy, the patient died of intractable seizure and acute renal failure on the fourth day of admission. Therefore, pediatricians should be aware of the clinical courses of hemolytic jaundice due to anti-Jkb, and they should be ready to treat this disease with active therapeutic interventions. PMID:16479082

  7. Mucoepidermoid carcinoma of the lung with initial presentation of microangiopathic hemolytic anemia and thrombocytopenia

    Directory of Open Access Journals (Sweden)

    Yuan-Chun Huang

    2017-12-01

    Full Text Available Mucoepidermoid carcinoma is a rare entity of lung malignancy that is subclassified into high-grade or low-grade types according to its histological features. High-grade mucoepidermoid carcinoma is a more aggressive form of malignancy, with a tendency towards lymph node involvement and distant metastasis. Cancer-related microangiopathic hemolytic anemia as a less common situation of paraneoplastic syndrome may be encountered with metastatic malignancy, but has not been reported previously in mucoepidermoid carcinoma of the lung. Herein, we report a 78-year-old male patient who presented with hemoptysis for one day. Laboratory tests showed microangiopathic hemolytic anemia and thrombocytopenia. A chest X-ray demonstrated consolidation in the left lung field. Chest computed tomography revealed a mass in the left upper lobe, and a subsequent bronchoscopic biopsy was performed. The histopathological results indicated a high-grade mucoepidermoid carcinoma. Magnetic resonance imaging of the brain demonstrated leptomeningeal carcinomatosis. The patient refused systemic chemotherapy, and palliative radiation therapy only was conducted for local disease control. The patient has performed well for 12 months to date since diagnosis of the tumor.

  8. Socio-Demographic and Maternal Factors in Anaemia in Pregnancy ...

    African Journals Online (AJOL)

    Low educational attainment [Adjusted Odds Ratio (AOR)=2.13], being single or divorced [AOR=2.02], high parity [AOR=2.06], late booking [AOR=2.71] and short intervals between pregnancies [AOR=2.37] were significant predictors of anaemia in pregnancy. The high prevalence of anaemia in pregnancy related to low ...

  9. Correlates and management of anaemia of chronic kidney disease ...

    African Journals Online (AJOL)

    Background: Anaemia is a common complication of chronic kidney disease. There is paucity of published local and regional data regarding its associated factors and management. Objective: To assess the correlates and management of anaemia in chronic kidney disease. Design: Cross sectional descriptive study

  10. Prevalence of iron deficiency anaemia in anaemic under-5 children ...

    African Journals Online (AJOL)

    Background: Iron deficiency anaemia has been described as the commonest type of nutritional anaemia in infancy and childhood. The associated adverse health sequelae include permanent behavioural and cognitive impairments. Early detection and prompt treatment are necessary to prevent these complications. Aim: To ...

  11. Iron deficiency anaemia among apparently healthy pre-school ...

    African Journals Online (AJOL)

    Background: Iron deficiency, and specifically iron deficiency anaemia, remains one of the most severe and important nutritional deficiencies in the world today. Objective: To estimate the prevalence and associated factors for iron deficiency anaemia among pre-school children in Lagos. Methodology: The study was ...

  12. Relationship between vitamin A status and anaemia among school ...

    African Journals Online (AJOL)

    Background: Anaemia and vitamin A deficiency are two major public health problems affecting many developing countries world-wide. Vitamin A deficiency, in addition to other health problems, can contribute to anaemia. Therefore, the objective of this study is to determine the relationship between vitamin A status and ...

  13. Malaria, anaemia and antimalarial drug resistance in African children

    NARCIS (Netherlands)

    Obonyo, C.O.

    2006-01-01

    Malaria-associated anaemia is a potentially preventable cause of severe morbidity and mortality in children < 5years of age, in areas of high malaria transmission in sub-Saharan Africa. In a cross-sectional study of 3586 children, 80% were anaemic (haemoglobin [Hb]<11g/dL) and 3% had severe anaemia

  14. Sociodemographic factors in anaemia in pregnancy in south ...

    African Journals Online (AJOL)

    Background: Anaemia in pregnancy persists, especially in third world countries where poor diet, low levels of literacy, infections, infestations and cultural practices predispose pregnant women to being anaemic. The aim of this study was to determine the prevalence of anaemia in pregnancy and to identify the possible ...

  15. Anaemia in pregnancy: a survey of pregnant women in Abeokuta ...

    African Journals Online (AJOL)

    Background: Anaemia in pregnancy is a common problem in most developing countries and a major cause of morbidity and mortality especially in malaria endemic areas. In pregnancy, anaemia has a significant impact on the health of the foetus as well as that of the mother. 20% of maternal deaths in Africa have been ...

  16. Pyridoxine responsive megaloblastic anaemia in pregnancy: a case ...

    African Journals Online (AJOL)

    In pregnancy megaloblastic anaemia commonly results from folic acid deficiency partly due to placenta transfer to fetus, but mainly because of increased folate catabolism due to cleavage of folate coenzymes in rapidly proliferating tissues. Cobalamin deficiency causing megaloblastic anaemia has been described in infants ...

  17. Anaemia and its associated factors among pregnant women in Koko ...

    African Journals Online (AJOL)

    Background: Anaemia in pregnancy remains a common problem affecting women in northern Nigeria. It is associated with several adverse consequences. Objective: The aim of this study was to determine the prevalence of anaemia and its associated factors among pregnant women in Koko/Besse local government area of ...

  18. Worm Infestation And Anaemia Among Pre-school Children Of ...

    African Journals Online (AJOL)

    Background: Worm infection and anaemia are common childhood conditions in Nigeria. We assessed the status of helminthiasis and associated anaemia among pre school children of peasant farmers aged 1-5 years living in a rubber plantation near Calabar, Nigeria. Design: Cross sectional. Method: Three hundred and ...

  19. Intestinal Worm Infestation and Anaemia in Pregnant Women

    Directory of Open Access Journals (Sweden)

    Krishna Bahadur Raut

    2016-03-01

    Conclusions: Aanaemia is prevalent in pregnant women of PHCRC, chapagaun and there was a significant correlation between anaemia and worm infestation. However, the relation among the haemoglobin level, iron, folic acid and albendazole was not significant. Keywords: anaemia; infestation; pregnant women; worm. | PubMed

  20. Risk factors for anaemia among HIV infected children attending care ...

    African Journals Online (AJOL)

    There is paucity of data describing the risk factors for anaemia among HIV infected children in Tanzania. This cross sectional study was carried out to determine the contributing factors for anaemia among HIV-infected children attending Muhimbili National Hospital in Dar es Salaam. Both univariate and multivariate logistic ...

  1. Ameliorative effects of Cnidoscolus aconitifolius on anaemia and ...

    African Journals Online (AJOL)

    This study was designed to evaluate the ameliorative effect of dietary supplementation of Cnidoscolus aconitifolius leaf on anaemia and changes in erythrocyte osmotic fragility in protein energy malnourished rats. Protein energy malnutrition has been associated with anaemia and changes in osmotic fragility, deformability ...

  2. Fulminant limb and retroperitoneal necrotizing fasciitis in a 15-year-old girl with Fanconi anaemia

    International Nuclear Information System (INIS)

    O'Regan, Kevin; Maher, Michael M.; O'Mahony, Edward; MacEneaney, Peter; Fitzgerald, Edward

    2009-01-01

    Necrotizing fasciitis (NF) is an uncommon soft-tissue infection in children that carries a high mortality rate. We present a 15-year-old girl with chronic pancytopenia secondary to Fanconi anaemia who developed extensive NF of the lower limb, which unfortunately resulted in a fatal outcome. Immunodeficiency is a known risk factor for the development of this condition. The findings in this case demonstrate that patients with Fanconi anaemia may be susceptible to NF and that the clinical course may be more aggressive due to underlying immunosuppression. Prompt diagnosis of NF is vital in order to initiate appropriate treatment and to optimize patient outcome. Radiological investigation demonstrated extensive soft-tissue gas and destruction affecting the entire lower limb, abdominal wall and retroperitoneum, which led to timely definitive diagnosis and management. (orig.)

  3. Fulminant limb and retroperitoneal necrotizing fasciitis in a 15-year-old girl with Fanconi anaemia

    Energy Technology Data Exchange (ETDEWEB)

    O' Regan, Kevin; Maher, Michael M. [Mercy University Hospital, Department of Radiology, Cork (Ireland); Cork University Hospital, Department of Radiology, Cork (Ireland); O' Mahony, Edward; MacEneaney, Peter; Fitzgerald, Edward [Mercy University Hospital, Department of Radiology, Cork (Ireland)

    2009-10-15

    Necrotizing fasciitis (NF) is an uncommon soft-tissue infection in children that carries a high mortality rate. We present a 15-year-old girl with chronic pancytopenia secondary to Fanconi anaemia who developed extensive NF of the lower limb, which unfortunately resulted in a fatal outcome. Immunodeficiency is a known risk factor for the development of this condition. The findings in this case demonstrate that patients with Fanconi anaemia may be susceptible to NF and that the clinical course may be more aggressive due to underlying immunosuppression. Prompt diagnosis of NF is vital in order to initiate appropriate treatment and to optimize patient outcome. Radiological investigation demonstrated extensive soft-tissue gas and destruction affecting the entire lower limb, abdominal wall and retroperitoneum, which led to timely definitive diagnosis and management. (orig.)

  4. Mathematical analysis of 51Cr-labelled red cell survival curves in congenital haemolytic anaemias

    International Nuclear Information System (INIS)

    Kasfiki, A.G.; Antipas, S.E.; Dimitriou, P.A.; Gritzali, F.A.; Melissinos, K.G.

    1982-01-01

    The parameters of 51 Cr labelled red cell survival curves were calculated in 26 patients with homozygous β-thalassaemia, 8 with sickle-cell anaemia and 3 with s-β-thalassaemia, using a non-linear weighted least squares analysis computer program. In thalassaemic children the calculated parameters denote that the shorting of the mean cell life is due to early senescence alone, while there is some evidence that in thalassaemic adults additional extracellular destruction mechanisms participate as well. Red cell survival curves from patients with sickle-cell anaemia and s-β-thalassaemia resemble each other, while their parameters indicate an initial rapid loss of radioactivity, early senescence and the presence of extracellular red cell destruction factors. (orig.)

  5. Fulminant limb and retroperitoneal necrotizing fasciitis in a 15-year-old girl with Fanconi anaemia.

    LENUS (Irish Health Repository)

    O'Regan, Kevin

    2009-10-01

    Necrotizing fasciitis (NF) is an uncommon soft-tissue infection in children that carries a high mortality rate. We present a 15-year-old girl with chronic pancytopenia secondary to Fanconi anaemia who developed extensive NF of the lower limb, which unfortunately resulted in a fatal outcome. Immunodeficiency is a known risk factor for the development of this condition. The findings in this case demonstrate that patients with Fanconi anaemia may be susceptible to NF and that the clinical course may be more aggressive due to underlying immunosuppression. Prompt diagnosis of NF is vital in order to initiate appropriate treatment and to optimize patient outcome. Radiological investigation demonstrated extensive soft-tissue gas and destruction affecting the entire lower limb, abdominal wall and retroperitoneum, which led to timely definitive diagnosis and management.

  6. Fulminant limb and retroperitoneal necrotizing fasciitis in a 15-year-old girl with Fanconi anaemia.

    LENUS (Irish Health Repository)

    O'Regan, Kevin

    2012-01-31

    Necrotizing fasciitis (NF) is an uncommon soft-tissue infection in children that carries a high mortality rate. We present a 15-year-old girl with chronic pancytopenia secondary to Fanconi anaemia who developed extensive NF of the lower limb, which unfortunately resulted in a fatal outcome. Immunodeficiency is a known risk factor for the development of this condition. The findings in this case demonstrate that patients with Fanconi anaemia may be susceptible to NF and that the clinical course may be more aggressive due to underlying immunosuppression. Prompt diagnosis of NF is vital in order to initiate appropriate treatment and to optimize patient outcome. Radiological investigation demonstrated extensive soft-tissue gas and destruction affecting the entire lower limb, abdominal wall and retroperitoneum, which led to timely definitive diagnosis and management.

  7. Anaemia in pregnancy is associated with advanced HIV disease.

    Directory of Open Access Journals (Sweden)

    Vikesh Nandlal

    Full Text Available Anaemia is a common clinical finding in HIV infected women and has been associated with advanced disease. The use of antiretroviral drugs such as Zidovudine (ZDV either for prevention of mother to child transmission (MTCT of HIV or used in combination with other antiretrovirals have been implicated in the development or increased severity of anaemia. We report the prevalence, type, severity and incidence of anaemia in a cohort of HIV infected women who initiated antiretroviral prophylaxis or treatment during pregnancy.This is a retrospective cohort data analysis of 408 HIV infected pregnant women who participated in a breastfeeding intervention study (HPTN 046 Study, ClinicalTrials.gov NCT 00074412 in South Africa. Women initiated zidovudine prophylaxis for PMTCT or triple antiretroviral treatment in pregnancy according to the standard of care. Laboratory and clinical data in pregnancy, <72 hours and 2 weeks postdelivery were extracted from the main database and analysed.The mean Hb concentration was 10.6 g/dL at baseline and 262/408 (64.2% women were diagnosed with anaemia (Hb<11 g/dL in pregnancy, 48/146 (32.9% subsequently developed anaemia intrapartum or postpartum and 89/310 (28.7% of all cases of anaemia remained unresolved by 2 weeks postdelivery. In a univariate analysis, CD4 count and gravidity were significant risk factors for anaemia in pregnancy, RR 1.41; 1.23-1.61 (p<0.001 and 1.10; 1.01-1.18 (p = 0.02 respectively. After adjusting for antiretroviral regimen, age and gravidity in a multivariable analysis, only the CD4 count remains a significant risk factor for anaemia in pregnancy and postdelivery.In conclusion, anaemia was most common among women in the advanced stage of HIV infection (CD4<200 cells/mm3. There was no evidence of an association between ZDV or triple ARVs and anaemia.

  8. Clinical Outcomes of Splenectomy in Children: Report of the Splenectomy in Congenital Hemolytic Anemia (SICHA) Registry

    Science.gov (United States)

    Rice, Henry E; Englum, Brian R; Rothman, Jennifer; Leonard, Sarah; Reiter, Audra; Thornburg, Courtney; Brindle, Mary; Wright, Nicola; Heeney, Matthew M; Smithers, Charles; Brown, Rebeccah L; Kalfa, Theodosia; Langer, Jacob C; Cada, Michaela; Oldham, Keith T; Scott, J Paul; St. Peter, Shawn; Sharma, Mukta; Davidoff, Andrew M.; Nottage, Kerri; Bernabe, Kathryn; Wilson, David B; Dutta, Sanjeev; Glader, Bertil; Crary, Shelley E; Dassinger, Melvin S; Dunbar, Levette; Islam, Saleem; Kumar, Manjusha; Rescorla, Fred; Bruch, Steve; Campbell, Andrew; Austin, Mary; Sidonio, Robert; Blakely, Martin L

    2014-01-01

    The outcomes of children with congenital hemolytic anemia (CHA) undergoing total splenectomy (TS) or partial splenectomy (PS) remain unclear. In this study, we collected data from 100 children with CHA who underwent TS or PS from 2005–2013 at 16 sites in the Splenectomy in Congenital Hemolytic Anemia (SICHA) consortium using a patient registry. We analyzed demographics and baseline clinical status, operative details, and outcomes at 4, 24, and 52 weeks after surgery. Results were summarized as hematologic outcomes, short-term adverse events (AEs) (≤ 30 days after surgery), and long-term AEs (31–365 days after surgery). For children with hereditary spherocytosis, after surgery there was an increase in hemoglobin (baseline 10.1 ± 1.8 gm/dl, 52 week 12.8 ± 1.6 gm/dl; mean ± SD), decrease in reticulocyte and bilirubin as well as control of symptoms. Children with sickle cell disease had control of clinical symptoms after surgery, but had no change in hematologic parameters. There was an 11% rate of short-term AEs and 11% rate of long-term AEs. As we accumulate more subjects and longer follow-up, use of a patient registry should enhance our capacity for clinical trials and engage all stakeholders in the decision-making process. PMID:25382665

  9. A fast-track anaemia clinic in the Emergency Department: cost-analysis of intravenous iron administration for treating iron-deficiency anaemia.

    Science.gov (United States)

    Quintana-Díaz, Manuel; Muñoz-Romo, Raúl; Gómez-Ramírez, Susana; Pavía, José; Borobia, Alberto M; García-Erce, José A; Muñoz, Manuel

    2017-09-01

    A fast-track anaemia clinic (FTAC) for the management of moderate-to-severe iron-deficiency anaemia (IDA) was established in our Emergency Department in 2010. In this FTAC, the replacement of packed red cell transfusion by ferric carboxymaltose administration was proven to be safe and effective. The aim of this study was a cost-analysis of IDA management in the FTAC, comparing this management with the previous standard care pathway consisting of packed red cell transfusion, if needed, and referral to outpatient specialised care. A cost study was performed for patients with IDA who were at risk of requiring transfusion (haemoglobin costs in the FTAC were compared to those theoretically incurred if these patients had been managed using the standard care pathway. In addition, a sensitivity analysis considering variations of up to ±30% in ferric carboxymaltose and packed red cell acquisition costs was performed (49 possible scenarios). Between 2012 and 2015, 238 IDA patients were treated in the FTAC. The average treatment cost was € 594±337/patient in the FTAC group and € 672±301/patient in the standard care pathway group, with a saving of € 78±28/patient (95% CI, 22-133; pcosts in the FTAC (€ 480-722/patient), compared with those of the standard care pathway (€ 550-794/patient), resulted in significant cost-savings for all studied scenarios (€ 51-104/patient; pcost-saving compared with the standard care pathway.

  10. Evaluation of anticonvulsant, antimicrobial and hemolytic activity of Aitchisonia rosea

    Directory of Open Access Journals (Sweden)

    Shahid Rasool

    2015-12-01

    Full Text Available The purpose of this study was to evaluate the anticonvulsant, antimicrobial and hemolytic effect of Aitchisonia rosea. The anticonvulsant effect was studied at doses 400 and 800 mg/kg against pentylenetetrazole, strychnine and picrotoxin-induced seizures in albino mice. The antimicrobial assay was conducted by disc diffusion method and minimum inhibitory concentration. Hemolytic effect was analyzed by reported method. Phenolic compounds present in the n-butanol fraction of the plant were estimated by HPLC. The plant showed maximum response against drug-induced convulsions and provided protection to animals at both doses. It also showed maximum zone of inhibition and highly significant MIC against all bacterial and fungal strains. The plant protected the RBCs from hemolysis. The highest amount of phenolics found was caffeic acid (7.5 ± 0.04.

  11. Severe late anemia of hemolytic disease of the newborn

    Science.gov (United States)

    Mitchell, Simon; James, Andrew

    1999-01-01

    Late anemia is a well-recognized complication of Rhesus hemolytic disease of the newborn (HDN). The incidence of Rhesus HDN is declining, with a tendency for more severely affected pregnancies to be managed in specialist centres. Consequently, many paediatric departments may see relatively few affected infants with comparatively mild disease, and the risk of late anemia in such cases may not always be appreciated. Two cases of infants born with evidence of Rhesus isoimmunization noted at birth and encountering no immediate problems other than mild hyperbilirubinemia are described. After an uneventful early neonatal course, both infants were discharged without follow-up and presented in the second to third weeks of life with severe, life-threatening anemia, leading to neurological sequelae in one case. The importance of close surveillance, including hemoglobin measurements, in all infants with Rhesus hemolytic disease, irrespective of initial severity, is reiterated. PMID:20212966

  12. Severe hemolytic disease of the newborn due to anti-Di b treated with phototherapy and intravenous immunoglobulin.

    Science.gov (United States)

    Oh, Eun-Jee; Jekarl, Dong Wook; Jang, Hyun-Sik; Park, Hae-Il; Park, Yeon-Joon; Choi, Hyun Ah; Chun, Chung-Sik; Kim, Yonggoo; Kim, Hyung Hoi

    2008-01-01

    The Di(b) antigen usually occurs with high incidence, except in certain Asian and South American Indian populations. In general, hemolysis caused by anti-Di(b) is not severe and its clinical course is benign. We report a Korean neonate with severe hemolytic disease of the newborn caused by anti-Di(b). The phenotype and genotype of the Diego blood group system of the patient and his mother were Di(a+b+) and Di(a+b-), respectively. The mother's serum and eluate from the neonate's erythrocytes contained anti-Di(b). This case was successfully managed with phototherapy and high dose iv immunoglobulin. Since most commercial antibody detection panels do not contain Di(b-) red cells, it is important to consider anti-Di(b) in cases of hemolytic disease of the newborn caused by an antibody against a high frequency antigen.

  13. Coeliac disease as the cause of resistant sideropenic anaemia in children with Down's syndrome: Case report

    Directory of Open Access Journals (Sweden)

    Pavlović Momčilo

    2010-01-01

    Full Text Available Introduction. Coeliac disease (CD is a permanent intolerance of gluten, i.e. of gliadin and related proteins found in the endosperm of wheat, rye and barley. It is characterized by polygenic predisposition, autoimmune nature, predominantly asymptomatic or atypical clinical course, as well as by high prevalence in patients with Down's syndrome (DS and some other diseases. Outline of Cases. We are presenting a girl and two boys, aged 6-7 (X=6.33 years with DS and CD recognized under the feature of sideropenic anaemia resistant to oral therapy with iron. Beside mental retardation, low stature and the morphological features characteristic of DS, two patients had a congenital heart disease; one ventricular septal defect and the other atrioventricular canal. In two patients, trisomy on the 21st chromosome pair (trisomy 21 was disclosed in all cells, while one had a mosaic karyotype. All three patients had classical laboratory parameters of sideropenic anaemia: blood Hb 77-89 g/l (X=81.67, HCT 0.26-0.29% (X=0.28, MCV 69-80 fl (X=73, MCH 24.3-30 pg (X=26.77 and serum iron 2-5 μmol/L (X=4.0. Beside anaemia and in one patient a mild isolated hypertransaminasemia (AST 67 U/l, ALT 62 U/l, other indicators of CD were not registered in any of the children. In addition, in all three patients, we also detected an increased level of antibodies to tissue transglutaminase (atTG of IgA class (45-88 U/l so that we performed endoscopic enterobiopsy in order to reliably confirm the diagnosis of CD. In all three patients, the pathohistological finding of the duodenal mucosa specimen showed mild to moderate destructive enteropathy associated with high intraepithelial lymphocyte infiltration, cryptic hyperplasia and lympho-plasmocytic infiltration of the stroma. In all three patients, the treatment with a strict gluten-free diet and iron therapy applied orally for 3-4 months resulted in blood count normalization and the correction of sideropenia. Serum level of the at

  14. Anti-M causing delayed hemolytic transfusion reaction

    International Nuclear Information System (INIS)

    Alperin, J.B.; Riglin, H.; Branch, D.R.; Gallagher, M.T.; Petz, L.D.

    1983-01-01

    A 52-year-old gravida 1, para 1 woman with M- red cells experienced a delayed hemolytic transfusion reaction and exhibited an anti-M antibody following the infusion of four units of M+ red cells. Measurements of erythrocyte survival using 51 Cr-labeled donor M+ and M- red cells and in vitro studies of monocyte-macrophage phagocytosis of sensitized reagent red cells implicate anti-M in the pathogenesis of hemolysis

  15. Intravenous immunoglobulin in ABO and Rh hemolytic diseases of newborn.

    Science.gov (United States)

    Nasseri, Fatemeh; Mamouri, Gholam A; Babaei, Homa

    2006-12-01

    To evaluate whether the use of intravenous immunoglobulin in newborn infants with isoimmune hemolytic jaundice due to Rh and ABO incompatibility is an effective treatment in reducing the need for exchange transfusion. This study included all direct Coombs' test positive Rh and ABO isoimmunized babies, who admitted in the Neonatal Intensive Care Unit of Ghaem Hospital of Mashhad University of Medical Sciences, Iran, from October 2003 to October 2004. Significant hyperbilirubinemia was defined as rising by >or=0.5 mg/dl per hour. Babies were randomly assigned to received phototherapy with intravenous immunoglobulin (IVIg) 0.5 g/kg over 4 hours, every 12 hours for 3 doses (study group) or phototherapy alone (control group). Exchange transfusion was performed in any group if serum bilirubin exceeded >or=20mg/dl or rose by >or=1mg/dl/h. A total of 34 babies were eligible for this study (17 babies in each group). The number of exchange transfusion, duration of phototherapy and hospitalization days, were significant shorter in the study group versus control group. When we analyzed the outcome results in ABO and Rh hemolytic disease separately, the efficacy of IVIg was significantly better in Rh versus ABO isoimmunization. Late anemia was more common in the IVIg group 11.8% versus 0%, p=0.48. Adverse effects were not observed during IVIg administration. Administration of IVIg to newborns with significant hyperbilirubinemia due to Rh hemolytic disease reduced the need for exchange transfusion but in ABO hemolytic disease there was no significant difference between IVIg and double surface blue light phototherapy.

  16. Hemolytic disease of the newborn- anti c antibody induced hemolysis.

    Science.gov (United States)

    Murki, Srinivas; Kandraju, Hemasree; Devi, Surekha A

    2012-02-01

    Hemolytic disease in the newborn, as a cause of early jaundice, is not uncommon. This is mostly due to Rh (D), ABO incompatibility and rarely due to other minor blood group incompatibility. The authors report two cases of Rh anti c isoimmunization presenting as significant early neonatal jaundice within the 20 h of life. Both the babies were treated with intensive phototherapy. One baby underwent exchange transfusion and the other required packed cell transfusion for anemia.

  17. Severe hemolytic disease of the newborn from anti-e.

    Science.gov (United States)

    McAdams, R M; Dotzler, S A; Winter, L W; Kerecman, J D

    2008-03-01

    Maternal antibody-mediated fetal red blood cell destruction secondary to non-D Rhesus (Rh) antibodies is a significant cause of hemolytic disease of the newborn (HDN). Here, we report a rare case of severe HDN associated with maternal antibody to Rh e. In addition to severe anemia, the infant developed thrombocytopenia, conjugated hyperbilirubinemia and cholelithiasis. Resolution of the infant's cholelithiasis occurred following treatment with ursodeoxycholic acid.

  18. Management of hemolytic-uremic syndrome in children

    OpenAIRE

    Grisaru, Silviu

    2014-01-01

    Silviu GrisaruUniversity of Calgary, Alberta Children's Hospital, Calgary, Alberta, CanadaAbstract: Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%), this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there ...

  19. [The immunometaboic effects of benfotiamine and riboxine on hemolytic anemia].

    Science.gov (United States)

    Uteshev, B S; Lazareva, G A; Prokopenko, L G

    2002-01-01

    Single (80 mg/kg) or multiply repeated (30 mg/kg) intramuscular injections of phenylhydrazine decrease the functional activity of mononuclear blood cells and the reduces immunological reactivity of the organism. Benfotiamine and riboxin decrease the extent of changes in the immunological response to a single administration of phenylhydrazine but do not significantly influence the immunological functions impaired by repeated injections of the hemolytic toxin.

  20. Antibodies to actin in autoimmune haemolytic anaemia

    Directory of Open Access Journals (Sweden)

    Ritzmann Mathias

    2010-03-01

    Full Text Available Abstract Background In autoimmune haemolytic anaemia (AIHA, autoreactive antibodies directed against red blood cells are up-regulated, leading to erythrocyte death. Mycoplasma suis infections in pigs induce AIHA of both the warm and cold types. The aim of this study was to identify the target autoantigens of warm autoreactive IgG antibodies. Sera from experimentally M. suis-infected pigs were screened for autoreactivity. Results Actin-reactive antibodies were found in the sera of 95% of all animals tested. The reactivity was species-specific, i.e. reactivity with porcine actin was significantly higher than with rabbit actin. Sera of animals previously immunised with the M. suis adhesion protein MSG1 showed reactivity with actin prior to infection with M. suis indicating that molecular mimicry is involved in the specific autoreactive mechanism. A potentially cross-reactive epitope was detected. Conclusions This is the first report of autoreactive anti-actin antibodies involved in the pathogenesis of autoimmune haemolytic anaemia.

  1. Serratamolide is a hemolytic factor produced by Serratia marcescens.

    Directory of Open Access Journals (Sweden)

    Robert M Q Shanks

    Full Text Available Serratia marcescens is a common contaminant of contact lens cases and lenses. Hemolytic factors of S. marcescens contribute to the virulence of this opportunistic bacterial pathogen. We took advantage of an observed hyper-hemolytic phenotype of crp mutants to investigate mechanisms of hemolysis. A genetic screen revealed that swrW is necessary for the hyper-hemolysis phenotype of crp mutants. The swrW gene is required for biosynthesis of the biosurfactant serratamolide, previously shown to be a broad-spectrum antibiotic and to contribute to swarming motility. Multicopy expression of swrW or mutation of the hexS transcription factor gene, a known inhibitor of swrW expression, led to an increase in hemolysis. Surfactant zones and expression from an swrW-transcriptional reporter were elevated in a crp mutant compared to the wild type. Purified serratamolide was hemolytic to sheep and murine red blood cells and cytotoxic to human airway and corneal limbal epithelial cells in vitro. The swrW gene was found in the majority of contact lens isolates tested. Genetic and biochemical analysis implicate the biosurfactant serratamolide as a hemolysin. This novel hemolysin may contribute to irritation and infections associated with contact lens use.

  2. Assesment, treatment and prevention of atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Azar Nickavar

    2013-01-01

    Full Text Available Hemolytic uremic syndrome (HUS is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1 D+ HUS: Presentation with a preceding diarrhea; (2 typical HUS: D+ HUS with a single and self-limited episode; (3 atypical HUS (aHUS: Indicated those with complement dysregulation; (4 recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA after improvement of hematologic abnormalities; and (5 familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

  3. Cardiovascular features in adolescents and adults with sickle cell anaemia.

    Science.gov (United States)

    Ayuo, P O; Abinya, N A; Joshi, M D; Lore, W

    1993-05-01

    Fifty five sickle cell anaemia (SCA) patients at the Kenyatta National Hospital were studied with a view to elucidating their cardiovascular status. Their age range was 13 to 27 years (median 18.9 years). They comprised 27 males and 28 females and their mean haemoglobin concentration was 8.5 +/- 1.4 g/dl. Haemoglobin level of 8.0-9.9 g/dl seen in 30 patients was noted to confer the lowest incidence of exertional dyspnoea and palpitation. Similarly, patients with this haemoglobin level had the lowest mean heart rate. The mean blood pressure was 114.9 +/- 9.9 mmHg systolic and 64.6 +/- 10 mmHg diastolic. Blood pressures, ejection fraction (EF) and differential fibre shortening (%D) were found to be directly related to haemoglobin level, whereas cardio-thoracic index (CTI) and left ventricular dimensions were inversely related to haemoglobin level. Mean echocardiographic measurements were within normal limits and left ventricular functions were found to be normal in 80.9% of the patients indicating that the majority of SCA patients at the Kenyatta National Hospital have good cardiac function.

  4. Body mass index and other anthropometric variables in children with sickle cell anaemia.

    Science.gov (United States)

    Odetunde, Odutola Israel; Chinawa, Josephat Maduabuchi; Achigbu, Kingsley Ihedioha; Achigbu, Eberechukwu O

    2016-01-01

    The objectives of this study were to determine the anthropometric variables of children with sickle cell anaemia and comparing it with those with normal haemoglobin genotype. A cross sectional study of anthropometric measurements was conducted over a period of six months. Children with sickle cell anaemia in steady state aged between 6-20 years were recruited. Nutritional assessment was done using anthropometrical variables. Data were analyzed using the Statistical Package for Social Sciences program (SPSS), version 20. The sickle cell patients comprised of 20 males and 20 females. There were an equal number of controls with an equal male to female ratio of 1:1. Forty eight percent (19) of the children with sickle cell anemia were underweight (sickle cell anemia were low when compared with children with normal Haemoglobin genotype.

  5. Post Blood Transfusion Hypertensive Encephalopathy in a Child with Congenital Hemolytic Anemia: A Case Report

    Directory of Open Access Journals (Sweden)

    Dhiman Arshpreet

    2017-11-01

    Full Text Available Introduction: Children having hemolytic anemias who have received multiple blood transfusions exhibit a rare complication of development of hypertension and seizures following transfusion, which may or may not be associated with intracranial hemorrhage. Case description: A 9-year-old boy presented with history of progressive paleness of body and weakness for the 30 days. There was a history of blood transfusion one week ago and multiple transfusions for one year of age. Examination revealed tachycardia, tachypnea, severe pallor and splenohepatomegaly. Blood work revealed a hemoglobin level of 4.0 grams with peripheral smear findings suggestive of hemolytic anemia. After blood transfusion, child complained of difficulty in breathing, vomiting and visual loss, followed by convulsions. Blood pressure was 180/110 mmHg. Seizure was controlled with intravenous midazolam and hypertension with furosemide and labetalol. CT brain was normal. As hypertension got under control, child gradually gained consciousness. Conclusion: A less intensive transfusion regimen among such patients along with prompt management of hypertension can prevent this potentially fatal syndrome.

  6. Perinatal care in British Columbia: Diagnosis and management of hemolytic disease of the newborn

    Science.gov (United States)

    Hardyment, A. F.; Manning, R. Elizabeth; Kinnis, Claire

    1974-01-01

    We undertook to measure standards of perinatal care in British Columbia by studying the management of hemolytic disease of the newborn as the sample situation. Our data show that many isoimmunized pregnant women are delivered in hospitals that have infrequent experience with this problem, and by physicians who have little experience with this disease. The physician referral pattern, in regard to maternal isoimmunization, indicated that the more severely affected patients were managed by specialists, particularly those attached to teaching hospitals. However, 25% of the infants treated by exchange transfusion were managed by nonspecialists in nonteaching hospitals. Hospital record search, used as a method of medical audit and as a source of data for comparison with physician reports, did not result in dependable or complete information. Rates of disagreement between items from two data sources, physician report and hospital record, were frequently very high. Our experience suggests that comparison of these two data sources is not an ideal method of assessment of quality of care. A smaller caseload of isoimmunized pregnant women will result from the present prevention program. Nevertheless, cases will continue to occur. Our work supports the conclusion that a program of continuing education covering the diagnosis and management of hemolytic disease of the newborn is still necessary. PMID:4213290

  7. Atypical hemolytic uremic syndrome: Laboratory characteristics, complement-amplifying conditions, renal biopsy, and genetic mutations

    Directory of Open Access Journals (Sweden)

    Mohammad A Hossain

    2018-01-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS. First, because of the lack of a single specific laboratory test for aHUS, other forms of thrombotic microangiopathies such as thrombotic thrombocytopenic purpura and Shiga toxin-associated HUS must be excluded to successfully establish the diagnosis of aHUS. Second, application of the knowledge of complement-amplifying conditions is critically important in making an accurate diagnosis. Third, when available, a renal biopsy can reveal changes consistent with thrombotic microangiopathy. Fourth, genetic mutations are increasingly clarifying the underlying complement dysfunction and gaining importance in the diagnosis and management of patients with aHUS. This review concentrates on the four aspects of aHUS and calls for heightened awareness in making an accurate diagnosis of aHUS.

  8. Familial Atypical Hemolytic Uremic Syndrome: A Review of Its Genetic and Clinical Aspects

    Directory of Open Access Journals (Sweden)

    Fengxiao Bu

    2012-01-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare renal disease (two per one million in the USA characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Both sporadic (80% of cases and familial (20% of cases forms are recognized. The study of familial aHUS has implicated genetic variation in multiple genes in the complement system in disease pathogenesis, helping to define the mechanism whereby complement dysregulation at the cell surface level leads to both sporadic and familial disease. This understanding has culminated in the use of Eculizumab as first-line therapy in disease treatment, significantly changing the care and prognosis of affected patients. However, even with this bright outlook, major challenges remain to understand the complexity of aHUS at the genetic level. It is possible that a more detailed picture of aHUS can be translated to an improved understanding of disease penetrance, which is highly variable, and response to therapy, both in the short and long terms.

  9. Acute Systolic Heart Failure Associated with Complement-Mediated Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    John L. Vaughn

    2015-01-01

    Full Text Available Complement-mediated hemolytic uremic syndrome (otherwise known as atypical HUS is a rare disorder of uncontrolled complement activation that may be associated with heart failure. We report the case of a 49-year-old female with no history of heart disease who presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given her normal ADAMSTS13 activity, evidence of increased complement activation, and renal biopsy showing evidence of thrombotic microangiopathy, she was diagnosed with complement-mediated HUS. She subsequently developed acute hypoxemic respiratory failure secondary to pulmonary edema requiring intubation and mechanical ventilation. A transthoracic echocardiogram showed evidence of a Takotsubo cardiomyopathy with an estimated left ventricular ejection fraction of 20%, though ischemic cardiomyopathy could not be ruled out. Treatment was initiated with eculizumab. After several failed attempts at extubation, she eventually underwent tracheotomy. She also required hemodialysis to improve her uremia and hypervolemia. After seven weeks of hospitalization and five doses of eculizumab, her renal function and respiratory status improved, and she was discharged in stable condition on room air and independent of hemodialysis. Our case illustrates a rare association between acute systolic heart failure and complement-mediated HUS and highlights the potential of eculizumab in stabilizing even the most critically-ill patients with complement-mediated disease.

  10. A case of autoimmune haemolytic anaemia after 39 cycles of nivolumab.

    Science.gov (United States)

    Shaikh, Hira; Daboul, Nour; Albrethsen, Mary; Fazal, Salman

    2018-04-18

    With growing use of nivolumab, rare but serious side effects have surfaced in some patients. We present a case of autoimmune haemolytic anaemia that developed after 39 cycles of nivolumab. A 78-year-old man with metastatic lung adenocarcinoma, refractory to multiple lines of chemotherapy was switched to nivolumab. After around 2 years of stable course on nivolumab, he developed transfusion-dependent anaemia with haemoglobin of 8.6 g/dL. Nivolumab was held immediately. Bone marrow biopsy findings were inconclusive of myelodysplastic syndrome. Further testing was suggestive of haemolysis with haptoglobin <10 mg/dL, elevated reticulocyte count and identification of immunoglobulin G antibody. Haemoglobin improved significantly with initiation of 1 mg/kg prednisone in addition to rituximab weekly × four doses. The development of transfusion-dependent anaemia with the exposure to cytotoxic chemotherapy usually raises the question for myelodysplastic syndrome. In contradiction, our patient was diagnosed to have a haematological autoimmune complication related to immunotherapy. © 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.

  11. Iron Deficiency Anaemia in Pregnancy and Postpartum: Pathophysiology and Effect of Oral versus Intravenous Iron Therapy

    Directory of Open Access Journals (Sweden)

    Alhossain A. Khalafallah

    2012-01-01

    Full Text Available Nutritional iron-deficiency anaemia (IDA is the most common disorder in the world, affecting more than two billion people. The World Health Organization’s global database on anaemia has estimated a prevalence of 14% based on a regression-based analysis. Recent data show that the prevalence of IDA in pregnant women in industrialized countries is 17.4% while the incidence of IDA in developing countries increases significantly up to 56%. Although oral iron supplementation is widely used for the treatment of IDA, not all patients respond adequately to oral iron therapy. This is due to several factors including the side effects of oral iron which lead to poor compliance and lack of efficacy. The side effects, predominantly gastrointestinal discomfort, occur in a large cohort of patients taking oral iron preparations. Previously, the use of intravenous iron had been associated with undesirable and sometimes serious side effects and therefore was underutilised. However, in recent years, new type II and III iron complexes have been developed, which offer better compliance and toleration as well as high efficacy with a good safety profile. In summary, intravenous iron can be used safely for a rapid repletion of iron stores and correction of anaemia during and after pregnancy.

  12. Clinical characteristics of hemolytic uremic syndrome secondary to cobalamin C disorder in Chinese children.

    Science.gov (United States)

    Li, Qi-Liang; Song, Wen-Qi; Peng, Xiao-Xia; Liu, Xiao-Rong; He, Le-Jian; Fu, Li-Bing

    2015-08-01

    The present study was undertaken to investigate the clinical characteristics of hemolytic uremic syndrome (HUS) secondary to cobalamin C disorder (cbl-C disorder). We reviewed retrospectively the medical records of 3 children with HUS secondary to cbl-C disorder who had been treated between April 1, 2009 and October 31, 2013. The 3 patients with HUS secondary to cbl-C disorder presented with progressive hemolytic anemia, acute renal failure, thrombocytopenia, poor feeding, and failure to thrive. Two of the 3 patients once had high blood pressure. The mutations of c.609G>A (p.W203X), c.217C>T (p.R73X) and c.365A>T (p.H122L) in the methylmalonic aciduria (cobalamin deficiency) cbl-C type, with homocystinuria gene were detected in the 3 patients. In these patients the levels of lactate dehydrogenase and homocysteine in serum were elevated and the level of methylmalonic acid (MMA) in urine was also elevated. After treatment with hydroxocobalamin, 2 patients were discharged with no obvious abnormal growth and neurological development and 1 patient died of multiple organ failure. The results of this study demonstrated that cbl-C disorder should be investigated in any child presenting with HUS. The high concentrations of homocysteine and MMA could be used for timely recognization of the disease. Once the high levels of plasma homocystein and/or plasma or urine MMA are detected, the treatment with parenteral hydroxocobalamin should be prescribed immediately. The early diagnosis and treatment would contribute to the good prognosis of the disease.

  13. Anaemia is typical of pregnancies: capturing community perception and management of anaemia in pregnancy in Anambra State, Nigeria.

    Science.gov (United States)

    Onyeneho, Nkechi G; Igweonu, Obianuju U

    2016-08-31

    Anaemia during pregnancy continues to constitute significant challenge to maternal health in Nigeria and contributes substantially to the worsening maternal mortality ratio (MMR) in Nigeria despite a global reduction in MMR in response to effort to improve safe motherhood. The incidence of anaemia during pregnancy is still high (>40 %) in Nigeria, and attitudes and management practices are yet unclear as the peoples' understanding of the phenomenon remains unclear. This study explored the perceptions/attitudes on anaemia during pregnancy and practices to prevent and/or manage it in Anambra State. In-depth interview and focus group discussion data were collected from health workers and mothers who delivered within 6 months preceding the study and from mothers and husbands of women who delivered within 6 months preceding the study, respectively. The people expressed some knowledge of anaemia, being common in pregnancies. However, some expressed the view that anaemia being a typical sign of pregnancy cannot be prevented. Some mothers expressed desire for focused antenatal care services to control anaemia but lamented the attitude of the health workers, who make access to these interventions difficult. Control of anaemia in pregnancy should start with providing health education to pregnant women and their partners, who reinforce what the women are told during antenatal care, and with training health workers for friendlier attitudes to clients.

  14. Tc-99m red blood cells for the study of rapid hemolytic processes associated with heterologous blood transfusions

    International Nuclear Information System (INIS)

    Benedetto, A.R.; Harrison, C.R.; Blumhardt, R.; Trow, L.L.

    1984-01-01

    Chromium-51 labeled erythrocytes (Cr-51 RBC) are suitable for the study of hematologic disorders which involve relatively slow destruction of circulating erythrocytes, taking several days to several weeks. However, Cr-51 RBC are not suitable for investigating rapid hemolytic processes which occur within a matter of a few hours due to the variable and unpredictable elution of Cr-51 from the erythrocytes during the first 24 hours or so. Imaging, which could be useful in identifying organ systems involved in the hemolytic process, cannot be performed with Cr-51 RBC because of the high dose commitment caused by the low yield of gamma rays from Cr-51 (2). A method of labeling RBC with Tc-99m, which results in a radiopharmaceutical that combines the excellent dosimetric and imaging qualities of Tc-99m with an extremely stable bond between the Tc-99m and the RBC, is reported. The successful application of this technique in providing red cell support for a cancer patient with an unusual history of intravascular hemolytic transfusion reactions is also reported

  15. [Etiologies of non-hemolytic jaundice in infants: a retrospective analysis of 3113 cases].

    Science.gov (United States)

    Peng, Xiaorong; Xu, Hongmei

    2015-06-01

    To investigate the causes of non-hemolytic jaundice among infants in Chongqing, China from the period of 1982 to 2011 and to determine whether the etiologies have changed over the past 30 years. The medical records of 3 113 infants,aged 1 month to 1 year,admitted to our hospital with non-hemolytic jaundice were collected and stratified according to decade-long time periods: group A (1982-1991), n=537; group B (1992-2001), n=786; group C (2002-2011), n=1 790. Data on sex, age, etiology and bilirubin level were retrospectively assessed using the chi-square test. In the three groups, boys consistently accounted for the majority of cases (group A:74.3%, group B:66.7%, group C:62.6%). In group A, 52% of the patients were 1-2 months of age; the peak age of patients in both group B and C was 2-3 months (group B:67.8%, group C:61.0%). Group A showed the highest level of patients with mildly elevated total bilirubin level (80.3%); however, moderately elevated total bilirubin level was most frequent in group B (53.4%) and group C (49.7%). The main etiologic diagnoses of the patients in group A were cytomegalovirus (CMV) infection (31.7%), sepsis (18.2%), hepatitis B virus (HBV) (1.3%), and biliary tract anomalies (1.3%); 46.6% of the cases had unclear cause. The main etiologic diagnoses of the cases in group B were CMV infection (36.0%), sepsis (21.5%), breast milk jaundice (2.0%), and HBV (1.9%); 37.9% of the cases had unclear cause. The main etiologic diagnoses of the cases in group C were CMV infection (42.6%), sepsis (7.5%), breast milk jaundice (17.7%), and biliary tract anomalies (2.46%); 29.1% of the cases had unclear cause. In Chongqing, infective factors, especially CMV, remain the main cause of nonhemolytic jaundice in infants, but bacterial etiologies have declined over the past 30 years.Non-infective factors, such as biliary tract anomalies and inherited metabolic diseases, have trended upwards. Although there has been great progress in the clinical management of

  16. Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Bures Vladimir

    2005-04-01

    Full Text Available Abstract We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. Conclusion Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

  17. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  18. Evaluation of some predisposing factors to malaria related anaemia ...

    African Journals Online (AJOL)

    Evaluation of some predisposing factors to malaria related anaemia among children in Benin City, Nigeria. ... Tropical Journal of Health Sciences ... It was carried out at the University of Benin Teaching Hospital, Benin City between June and ...

  19. Role of cytokines in Trypanosoma brucei-induced anaemia: A ...

    African Journals Online (AJOL)

    species Trypanosoma brucei that are transmitted by a tsetse fly (Glossina spp.) ... of autologous immunoglobulin antibodies on the red cell surfaces and also to ... development for the detection and management of anaemia in trypanosomiasis.

  20. IS OBESITY ASSOCIATED WITH ANAEMIA IN KIDNEY TRANSPLANT RECIPIENTS

    Directory of Open Access Journals (Sweden)

    Winnie Chan

    2012-06-01

    This study demonstrated that overall adiposity correlates positively with inflammation but this is not applicable to indices of visceral fat. However, no correlation was established between obesity and anaemia in this study.

  1. Investigation of manganese homeostasis in dogs with anaemia and ...

    African Journals Online (AJOL)

    Investigation of manganese homeostasis in dogs with anaemia and chronic enteropathy. Marisa da Fonseca Ferreira, Arielle Elizabeth Ann Aylor, Richard John Mellanby, Susan Mary Campbell, Adam George Gow ...

  2. Iron Deficiency Anaemia In Reproductive Age Women Attending ...

    African Journals Online (AJOL)

    Iron Deficiency Anaemia In Reproductive Age Women Attending Obstetrics And ... prevalence of iron deficiency anemia in reproductive age women, and their relation to ... Thus iron deficiency anemia during pregnancy in well-educated set up ...

  3. Current opinion on the management of iron deficiency anaemia in gastrointestinal diseases.

    Science.gov (United States)

    Derovs, Aleksejs; Pokrotnieks, Juris; Derova, Jelena; Danilans, Anatolijs; Pukitis, Aldis; Dombure, Polina; Leiniece, Sandra; Zeltina Indra

    2014-01-01

    Iron deficiency is the most common cause of anaemia in the world. Despite frequently weak and masked clinical presentation of iron deficiency anaemia (IDA), this disease is very serious with complications leading to early mortality. In the developed countries IDA is predominantly diagnosed as the complication of another disease or as the result of major bleeding events. Diagnosis of IDA should be based on laboratory findings i.e. haemoglobin, mean corpuscular hemoglobin concentration and ferritin. Latter is the most sensitive marker for iron deficiency. Anaemia of chronic disease should be taken into an account as a potential differential diagnosis or coexisting state. For women in fertility age with IDA, gynaecological disorders should be ruled out first. Males and postmenopausal women with IDA should undergo upper, lower and in certain cases capsule endoscopy and/or enteroscopy to find a plausible cause of IDA. The ultimate goal of therapy is to find out and treat the primary cause of IDA. Iron body stores should be restored using either oral or parenteral iron preparations. The use of parenteral iron preparations in patients with gastrointestinal pathologies is often clinically substantiated for the treatment of IDA. Red blood cell transfusion should be administered in emergency cases only.

  4. Transfusion-dependent anaemia of undetermined origin: a distinctive syndrome in paediatric medical tourism.

    Science.gov (United States)

    Lee, Anselm C W

    2012-07-01

    The underlying diagnosis of severe anaemic illnesses in children may not be easy to identify at times, especially when regular blood transfusion has been started. International children patients attending a haematology clinic for diagnostic evaluation were identified retrospectively if they had to receive repeated blood transfusions with an undiagnosed illness or an incorrect diagnosis. Their demographic data, presenting features, and eventual diagnosis were described. Twelve children including 7 boys were enrolled from March 2007 to August 2011. Five came from Vietnam; 2 each came from Bangladesh and Indonesia; and 1 each from Hong Kong, Myanmar, and Ukraine. Their illnesses started at a mean age of 1.5 years (0.1 to 6.6) and they had been receiving blood transfusion for a mean duration of 2.5 years (0.1 to 9.9) years prior to the evaluation. Thalassemia major was the fi rst diagnosis in 5 cases; one had been treated for autoimmune haemolytic anaemia while the rest had not been given a diagnosis. After the evaluation, 4 children were diagnosed with Diamond Blackfan anaemia, 3 were diagnosed with hereditary spherocytosis, and one each with hereditary pyropoikilocytosis, congenital sideroblastic anaemia, congenital thrombotic thrombocytopenic purpura, transient erythroblastopenia of childhood, and autoimmune myelofibrosis associated with human immunodeficiency virus infection. A definitive diagnosis can be identified in this cohort of children on medical tourism with severe anaemic illnesses requiring repeated transfusions with diagnostic approaches that circumvent the interference of transfused cells.

  5. P-wave dispersion: relationship to left ventricular function in sickle cell anaemia.

    Science.gov (United States)

    Oguanobi, N I; Onwubere, B J; Ike, S O; Anisiuba, B C; Ejim, E C; Ibegbulam, O G

    2011-01-01

    The prognostic implications of P-wave dispersion in patients with a variety of cardiac disease conditions are increasingly being recognised. The relationship between P-wave dispersion and left ventricular function in sickle cell anaemia is unknown. This study was aimed at evaluating the relationship between P-wave dispersion and left ventricular function in adult Nigerian sickle cell anaemia patients. Between February and August 2007, a total of 62 sickle cell anaemia patients (aged 18-44 years; mean 28.27 ± 5.58) enrolled in the study. These were drawn from patients attending the adult sickle cell clinic of the University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu. An equal number of age- and gender-matched normal subjects served as controls. All the participants were evaluated with electrocardiography and echocardiography. P-wave dispersion was defined as the difference between the maximum and minimum P-wave duration measured in a 12-lead electrocardiogram. P-wave duration and P-wave dispersion were significantly higher in patients than in controls. Significant correlation was demonstrated between P-wave dispersion and age in the patients (r = 0.387; p = 0.031). A comparison of subsets of sickle cell anaemia patients and controls with comparable haematocrit values (30-35%) showed significantly higher P-wave duration and P-wave dispersion in the patients than in the controls. The P-wave duration in patients and controls, respectively, was 111.10 ± 14.53 ms and 89.14 ± 16.45 ms (t = 3.141; p = 0.006). P-wave dispersion was 64.44 ± 15.86 ms in the patients and 36.43 ± 10.35 ms in the controls (t = 2.752; p = 0.013). Significant negative correlation was found between P-wave dispersion and left ventricular transmitral E/A ratio (r = -0.289; p = 0.023). These findings suggest that P-wave dispersion could be useful in the evaluation of sickle cell patients with left ventricular diastolic dysfunction. Further prospective studies are recommended to evaluate

  6. Trichuris and hookworm infections associated with anaemia during pregnancy.

    Science.gov (United States)

    Gyorkos, Theresa W; Gilbert, Nicolas L; Larocque, Renée; Casapía, Martín

    2011-04-01

    To assess the following associations between the second and third trimesters of pregnancy: (i) the intensity of soil-transmitted helminth (STH) infection and haemoglobin/anaemia, (ii) the effect of mebendazole treatment on the occurrence of STH infection, and (iii) the effect of mebendazole treatment on haemoglobin/anaemia. Data originated from a trial of 1042 pregnant women recruited in their second trimester and followed to delivery. Baseline assessments included socio-demographic/health information from questionnaires, haemoglobin/anaemia from HemoCue ascertainment of fingerprick blood, and the presence and intensity of STH (Ascaris lumbricoides, hookworms and Trichuris trichiura) infections from Kato-Katz examination. All women were given iron supplements; half were randomly allocated to receive single dose 500 mg mebendazole, and half, placebo. Haemoglobin/anaemia and STH infection status were determined again in the third trimester of pregnancy. Complete information was available from 935 (89.7%) women. Mebendazole significantly reduced the prevalence and intensity of all three STH infections. Higher intensities of hookworm and Trichuris infections in the second trimester were associated with a higher risk of anaemia in the third trimester. Overall, women with moderate/heavy Trichuris infection were found to be at a higher risk of anaemia; the highest risk was observed among those with moderate/heavy hookworm co-infection (adjusted OR = 2.77; 95% CI: 1.26, 6.11). Mebendazole treatment did not reduce the risk of anaemia. Higher intensities of both Trichuris and hookworm infections are associated with anaemia in pregnancy. The importance of Trichuris infections during pregnancy requires renewed attention. © 2011 Blackwell Publishing Ltd.

  7. Incompatible blood transfusion: Challenging yet lifesaving in the management of acute severe autoimmune hemolytic anemia

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    Sudipta Sekhar Das

    2014-01-01

    Full Text Available Background and Aim: Autoimmune hemolytic anemia (AIHA is characterized by the production of autoantibodies directed against red cell antigens. Most patients of AIHA arrive in the emergency or out-patient department (OPD with severe anemia requiring urgent blood transfusion. Here we share our experience of managing these patients with incompatible blood transfusions and suggest the minimal test required to assure patient safety. Materials and Methods: A total of 14 patients admitted with severe anemia, diagnosed with AIHA and requiring blood transfusion urgently were included in the study. A series of immunohematological investigations were performed to confirm the diagnosis and issue "best match" packed red blood cells (PRBC to these patients. Results: A total of 167 PRBC units were crossmatched for 14 patients of which 46 units (28% were found to be best match ones and 26 (56.5% of these units were transfused. A mean turn around time of 222 min was observed in issuing the ′best match′ blood. Severe hemolysis was observed in all patients with a median hemoglobin increment of 0.88 g/dl after each unit PRBC transfusion. Conclusion: Decision to transfuse in AIHA should be based on the clinical condition of the patient. No critical patient should be denied blood transfusion due to serological incompatibility. Minimum investigations such as direct antiglobulin test (DAT, antibody screening and autocontrol should be performed to ensure transfusion safety in patients. All transfusion services should be capable of issuing "best match" PRBCs in AIHA.

  8. Incompatible blood transfusion: Challenging yet lifesaving in the management of acute severe autoimmune hemolytic anemia.

    Science.gov (United States)

    Das, Sudipta Sekhar; Zaman, Rafiq Uz; Safi, Mohammad

    2014-07-01

    Autoimmune hemolytic anemia (AIHA) is characterized by the production of autoantibodies directed against red cell antigens. Most patients of AIHA arrive in the emergency or out-patient department (OPD) with severe anemia requiring urgent blood transfusion. Here we share our experience of managing these patients with incompatible blood transfusions and suggest the minimal test required to assure patient safety. A total of 14 patients admitted with severe anemia, diagnosed with AIHA and requiring blood transfusion urgently were included in the study. A series of immunohematological investigations were performed to confirm the diagnosis and issue best match packed red blood cells (PRBC) to these patients. A total of 167 PRBC units were crossmatched for 14 patients of which 46 units (28%) were found to be best match ones and 26 (56.5%) of these units were transfused. A mean turn around time of 222 min was observed in issuing the "best match" blood. Severe hemolysis was observed in all patients with a median hemoglobin increment of 0.88 g/dl after each unit PRBC transfusion. Decision to transfuse in AIHA should be based on the clinical condition of the patient. No critical patient should be denied blood transfusion due to serological incompatibility. Minimum investigations such as direct antiglobulin test (DAT), antibody screening and autocontrol should be performed to ensure transfusion safety in patients. All transfusion services should be capable of issuing "best match" PRBCs in AIHA.

  9. Intraclutch variation in avian eggshell pigmentation: the anaemia hypothesis.

    Science.gov (United States)

    De Coster, Greet; De Neve, Liesbeth; Lens, Luc

    2012-10-01

    Many passerine species lay eggs that are speckled with dark protoporphyrin pigmentation. Because protoporphyrin is mainly derived from the blood, we here formulate and test a new hypothesis that links an increase in anaemia along the laying sequence to within-clutch variation in egg pigmentation. More intense pigmentation is expected if pigments accumulate during enhanced red blood cell production in response to anaemia. Reduced pigmentation is expected if pigments are derived from the degradation of red blood cells that circulate in smaller numbers due to blood loss. To test this hypothesis, we manipulated anaemia in great tit (Parus major) females by infesting the nests with hen fleas (Ceratophyllus gallinae) prior to egg laying. Polychromatophil (i.e., immature red blood cells) percentage, as a measure of blood cell production, was positively correlated with parasite load confirming that female great tits experienced stronger anaemia when infested with haematophagous parasites during egg laying. We found a positive relationship between spot darkness and laying order that weakened under high parasite load. This result suggests that anaemia in females due to blood-sucking parasites led to diminished protoporphyrin from disintegrated red blood cells and hence a decreased deposition of protoporphyrin. However, the overall increase in pigment darkness along the laying sequence suggests that pigments also accumulate by enhanced red blood cell production caused by anaemia due to egg production itself.

  10. MATERNAL ANAEMIA AND NEWBORN MORBIDITY IN A TERTIARY HOSPITAL

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    Garuda Rama

    2017-02-01

    Full Text Available BACKGROUND Anaemia is the commonest nutritional deficiency disorder in the world and in India prevalence in pregnancy is 50-80%. It is a risk factor for preterm delivery, low birth weight, stillbirth and foetal anaemia. The aim of the study is to find out correlation between maternal and cord haemoglobin and birth weight of newborn. MATERIALS AND METHODS Prospective observational study of 150 pregnant women delivering in KIMS from December 2014 to June 2016 for antenatal and newborn haemoglobin and newborn weight. RESULTS Of 150 mothers, 89.33% are anaemic (24% mild, 44% moderate and 21.3% severe. The incidence of low birth weight and low cord haemoglobin varied directly with severity of anaemia. Out of 150 babies, 119 (79.3% of babies had anaemia in our study. The mean haemoglobin among babies was 12.9 g/dL with a S.D. of 2.01. CONCLUSION A significant positive correlation with maternal haemoglobin and newborn weight and cord haemoglobin. RecommendationAntenatal prophylaxis with iron and folic acid to prevent anaemia and incidence of low birth weight and infantile anaemia.

  11. Neutropenia in infants with hemolytic disease of the newborn.

    Science.gov (United States)

    Blanco, Esther; Johnston, Donna L

    2012-06-01

    This study examined the incidence, outcome and risk factors of neutropenia in infants with hemolytic disease of the newborn (HDN). A retrospective chart review was performed on infants with HDN. Of 69 evaluable infants, 45% developed neutropenia. Only one infectious complication was recorded. In most instances the neutropenia resolved spontaneously, but in seven infants it persisted for a median of 397 days. Males were at higher risk for developing neutropenia, but severity of HDN, antibody specificity, or therapy were not significant risk factors. Neutropenia is a common feature of HDN, regardless of severity of disease, treatment received, or antibody specificity. Copyright © 2011 Wiley Periodicals, Inc.

  12. Atypical hemolytic uremic syndrome triggered by varicella infection

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    Pauline Condom

    2017-01-01

    The current case describes an aHUS associated to varicella infection as demonstrated by the simultaneous occurrence of the viral infection and aHUS manifestations. Apart from typical Hemolytic Uremic Syndrome which is triggered by bacteria mostly Shiga toxin producing Echerichia coli and Streptococcus pneumoniae or Shigella, aHUS may be linked to viral infections such as HIV, EBV and enteroviruses, but very rarely by varicella. This case highlights a possible even rare complication of varicella infection a very common childhood disease. This complication could be avoided by to anti-VZV vaccination.

  13. Anaemia is typical of pregnancies: capturing community perception and management of anaemia in pregnancy in Anambra State, Nigeria

    OpenAIRE

    Onyeneho, Nkechi G.; Igweonu, Obianuju U.

    2016-01-01

    Background: Anaemia during pregnancy continues to constitute significant challenge to maternal health in Nigeria and contributes substantially to the worsening maternal mortality ratio (MMR) in Nigeria despite a global reduction in MMR in response to effort to improve safe motherhood. The incidence of anaemia during pregnancy is still high (>40 %) in Nigeria, and attitudes and management practices are yet unclear as the peoples’ understanding of the phenomenon remains unclear. This study expl...

  14. Clinical significance of acquired somatic mutations in aplastic anaemia.

    Science.gov (United States)

    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.

  15. [Iron-deficiency anaemia in everyday gynaecological practice].

    Science.gov (United States)

    Lukanova, M; Popov, I

    2004-01-01

    Iron-deficiency anaemia /IDA/ is of utmost significance to clinical practice. Chronic haemorrhages from the genital tract are the major etiological factor for its appearance in 60-70% of the patients. Abnormal genital bleeding for the specialist in Obstetrics and gynaecology and IDA for the haematologist are frequently met problems in their everyday practice, which require detailed examination, good colaboration and synchronization between the work of both specialists. Diagnosing and etiological treatment of IDA of gynaecologic origin by mutual timely and adequate co-operation of gynaecologist and haematologist. Clinical survey based on the algorithm worked out. Its everyday application started in July-August 2001 and till today /30.04.2003/ 253 cases with IDA in the Department of Gynaecology are taken in. A record of proceedings was made for every patient and that helped the further diagnostic and therapeutic activity and respective data processing. The data and results obtained verify the achievement of final diagnostic specification of IDA, the role of the algorithm as a stepping-stone to its etiological treatment, complete and durable correction of iron deficiency.

  16. Hemolytic uremic syndrome (HUS) secondary to cobalamin C (cblC) disorder.

    Science.gov (United States)

    Sharma, Ajay P; Greenberg, Cheryl R; Prasad, Asuri N; Prasad, Chitra

    2007-12-01

    Diarrhea-positive hemolytic uremic syndrome (HUS) is a common cause of acute renal failure in children. Diarrhea-negative (D-), or atypical HUS, is etiologically distinct. A Medline search identified seven previously reported D- cases of HUS secondary to cobalamin C (cblC) disease presenting in infancy. An infantile presentation is reported to be associated with a high mortality rate (6/7 cases). We describe the results of a 5-year longitudinal follow-up in a child diagnosed with D- HUS secondary to cblC disease in infancy. Mutation analysis in this patient identified homozygosity for the 271 dupA mutation (c.271 dupA) in the cblC MMACHC gene. We briefly review the published experience in cblC-associated HUS to highlight the clinical characteristics of this uncommon, but potentially treatable, condition.

  17. Hemolytic disease of the fetus and newborn: managing the mother, fetus, and newborn.

    Science.gov (United States)

    Delaney, Meghan; Matthews, Dana C

    2015-01-01

    Hemolytic disease of the fetus and newborn (HDFN) affects 3/100 000 to 80/100 000 patients per year. It is due to maternal blood group antibodies that cause fetal red cell destruction and in some cases, marrow suppression. This process leads to fetal anemia, and in severe cases can progress to edema, ascites, heart failure, and death. Infants affected with HDFN can have hyperbilirubinemia in the acute phase and hyporegenerative anemia for weeks to months after birth. The diagnosis and management of pregnant women with HDFN is based on laboratory and radiographic monitoring. Fetuses with marked anemia may require intervention with intrauterine transfusion. HDFN due to RhD can be prevented by RhIg administration. Prevention for other causal blood group specificities is less studied. © 2015 by The American Society of Hematology. All rights reserved.

  18. Abordagem ambulatorial do nutricionista em anemia hemolítica Nutritional ambulatory approach in hemolytic anemia

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    Maria Aparecida Vieira

    1999-04-01

    Full Text Available Descreve a atuação do nutricionista em ambulatório de Hematologia Pediátrica em um hospital escola e relata as condutas dietéticas necessárias na abordagem de crianças com anemia hemolítica com e sem sobrecarga de ferro, e também as atitudes mais freqüentes dos familiares em relação à alimentação desses pacientes.The Authors describe the performance of the Dietitian in a Pediatric Hematology Ambulatory. They emphasize the necessary dietetic procedures for adequate management of children with hemolytic anemia, with and without iron overload. Furthermore, they approach the family's attitude towards the patient's nutrition.

  19. Treatments for iron-deficiency anaemia in pregnancy.

    Science.gov (United States)

    Reveiz, Ludovic; Gyte, Gillian Ml; Cuervo, Luis Gabriel; Casasbuenas, Alexandra

    2011-10-05

    Iron deficiency, the most common cause of anaemia in pregnancy worldwide, can be mild, moderate or severe. Severe anaemia can have very serious consequences for mothers and babies, but there is controversy about whether treating mild or moderate anaemia provides more benefit than harm. To assess the effects of different treatments for anaemia in pregnancy attributed to iron deficiency (defined as haemoglobin less than 11 g/dL or other equivalent parameters) on maternal and neonatal morbidity and mortality. We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (7 June 2011), CENTRAL (2011, Issue 5), PubMed (1966 to June 2011), the International Clinical Trials Registry Platform (ICTRP) (2 May 2011), Health Technology Assessment Program (HTA) (2 May 2011) and LATINREC (Colombia) (2 May 2011). Randomised controlled trials comparing treatments for anaemia in pregnancy attributed to iron deficiency. We identified 23 trials, involving 3.198 women. We assessed their risk of bias. Three further studies identified are awaiting classification. Many of the trials were from low-income countries; they were generally small and frequently methodologically poor. They covered a very wide range of differing drugs, doses and routes of administration, making it difficult to pool data. Oral iron in pregnancy showed a reduction in the incidence of anaemia (risk ratio 0.38, 95% confidence interval 0.26 to 0.55, one trial, 125 women) and better haematological indices than placebo (two trials). It was not possible to assess the effects of treatment by severity of anaemia. A trend was found between dose and reported adverse effects. Most trials reported no clinically relevant outcomes nor adverse effects. Although the intramuscular and intravenous routes produced better haematological indices in women than the oral route, no clinical outcomes were assessed and there were insufficient data on adverse effects, for example, on venous thrombosis and severe allergic reactions

  20. Cobalt-doped nanohydroxyapatite: synthesis, characterization, antimicrobial and hemolytic studies

    Energy Technology Data Exchange (ETDEWEB)

    Tank, Kashmira P., E-mail: kashmira_physics@yahoo.co.in [Saurashtra University, Crystal Growth Laboratory, Physics Department (India); Chudasama, Kiran S.; Thaker, Vrinda S. [Saurashtra University, Bioscience Department (India); Joshi, Mihir J., E-mail: mshilp24@rediffmail.com [Saurashtra University, Crystal Growth Laboratory, Physics Department (India)

    2013-05-15

    Hydroxyapatite (Ca{sub 10}(PO{sub 4}){sub 6}(OH){sub 2}; HAP) is a major mineral component of the calcified tissues, and it has various applications in medicine and dentistry. In the present investigation, cobalt-doped hydroxyapatite (Co-HAP) nanoparticles were synthesized by surfactant-mediated approach and characterized by different techniques. The EDAX was carried out to estimate the amount of doping in Co-HAP. The transmission electron microscopy result suggested the transformation of morphology from needle shaped to spherical type on increasing the doping concentration. The powder XRD study indicated the formation of a new phase of brushite for higher concentration of cobalt. The average particle size and strain were calculated using Williamson-Hall analysis. The average particle size was found to be 30-60 nm. The FTIR study confirmed the presence of various functional groups in the samples. The antimicrobial activity was evaluated against four organisms Pseudomonas aeruginosa and Shigella flexneri as Gram negative as well as Micrococcus luteus and Staphylococcus aureus as Gram positive. The hemolytic test result suggested that all samples were non-hemolytic. The photoluminescence study was carried out to identify its possible applicability as a fluorescent probe.

  1. Cobalt-doped nanohydroxyapatite: synthesis, characterization, antimicrobial and hemolytic studies

    International Nuclear Information System (INIS)

    Tank, Kashmira P.; Chudasama, Kiran S.; Thaker, Vrinda S.; Joshi, Mihir J.

    2013-01-01

    Hydroxyapatite (Ca 10 (PO 4 ) 6 (OH) 2 ; HAP) is a major mineral component of the calcified tissues, and it has various applications in medicine and dentistry. In the present investigation, cobalt-doped hydroxyapatite (Co-HAP) nanoparticles were synthesized by surfactant-mediated approach and characterized by different techniques. The EDAX was carried out to estimate the amount of doping in Co-HAP. The transmission electron microscopy result suggested the transformation of morphology from needle shaped to spherical type on increasing the doping concentration. The powder XRD study indicated the formation of a new phase of brushite for higher concentration of cobalt. The average particle size and strain were calculated using Williamson–Hall analysis. The average particle size was found to be 30–60 nm. The FTIR study confirmed the presence of various functional groups in the samples. The antimicrobial activity was evaluated against four organisms Pseudomonas aeruginosa and Shigella flexneri as Gram negative as well as Micrococcus luteus and Staphylococcus aureus as Gram positive. The hemolytic test result suggested that all samples were non-hemolytic. The photoluminescence study was carried out to identify its possible applicability as a fluorescent probe.

  2. Neonatal management and outcome in alloimmune hemolytic disease.

    Science.gov (United States)

    Ree, Isabelle M C; Smits-Wintjens, Vivianne E H J; van der Bom, Johanna G; van Klink, Jeanine M M; Oepkes, Dick; Lopriore, Enrico

    2017-07-01

    Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management. Areas covered: This review focusses on the current neonatal management and outcome of hemolytic disease and discusses postnatal treatment options as well as literature on long-term neurodevelopmental outcome. Expert commentary: Despite major advances in neonatal management, multiple issues have to be addressed to optimize postnatal management and completely eradicate kernicterus. Except for strict adherence to guidelines, improvement could be achieved by clarifying the epidemiology and pathophysiology of HDFN. Several pharmacotherapeutic agents should be further researched as alternative treatment options in hyperbilirubinemia, including immunoglobulins, albumin, phenobarbital, metalloporphyrins, zinc, clofibrate and prebiotics. Larger trials are warranted to evaluate EPO, folate and vitamin E in neonates. Long-term follow-up studies are needed in HDFN, especially on thrombocytopenia, iron overload and cholestasis.

  3. Management of hemolytic-uremic syndrome in children

    Directory of Open Access Journals (Sweden)

    Grisaru S

    2014-06-01

    Full Text Available Silviu GrisaruUniversity of Calgary, Alberta Children's Hospital, Calgary, Alberta, CanadaAbstract: Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS. In most cases (90%, this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there are no proven treatment options that can directly inactivate the toxin or effectively interfere with the cascade of destructive events triggered by the toxin once it gains access to the bloodstream and binds its receptor. However, HUS is self-limited, and effective supportive management during the acute phase is proven to be a life saver for children affected by HUS. A minority of childhood HUS cases, approximately 5%, are caused by various genetic mutations causing uncontrolled activation of the complement system. These children, who used to have a poor prognosis leading to end-stage renal disease, now have access to exciting new treatment options that can preserve kidney function and avoid disease recurrences. This review provides a summary of the current knowledge on the epidemiology, pathophysiology, and clinical presentation of childhood HUS, focusing on a practical approach to best management measures.Keywords: hemolytic, uremic, E.coli O157:H7, thrombotic, microangiopathy, complement system

  4. Hemolytic porcine intestinal Escherichia coli without virulence-associated genes typical of intestinal pathogenic E. coli.

    Science.gov (United States)

    Schierack, Peter; Weinreich, Joerg; Ewers, Christa; Tachu, Babila; Nicholson, Bryon; Barth, Stefanie

    2011-12-01

    Testing 1,666 fecal or intestinal samples from healthy and diarrheic pigs, we obtained hemolytic Escherichia coli isolates from 593 samples. Focusing on hemolytic E. coli isolates without virulence-associated genes (VAGs) typical for enteropathogens, we found that such isolates carried a broad variety of VAGs typical for extraintestinal pathogenic E. coli.

  5. Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland.

    Science.gov (United States)

    Brocklebank, Vicky; Johnson, Sally; Sheerin, Thomas P; Marks, Stephen D; Gilbert, Rodney D; Tyerman, Kay; Kinoshita, Meredith; Awan, Atif; Kaur, Amrit; Webb, Nicholas; Hegde, Shivaram; Finlay, Eric; Fitzpatrick, Maggie; Walsh, Patrick R; Wong, Edwin K S; Booth, Caroline; Kerecuk, Larissa; Salama, Alan D; Almond, Mike; Inward, Carol; Goodship, Timothy H; Sheerin, Neil S; Marchbank, Kevin J; Kavanagh, David

    2017-11-01

    Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort analysis. Here we examined 175 children presenting with atypical hemolytic uremic syndrome in the United Kingdom and Ireland for factor H autoantibodies that included 17 children with titers above the international standard. Of the 17, seven had a concomitant rare genetic variant in a gene encoding a complement pathway component or regulator. Two children received supportive treatment; both developed established renal failure. Plasma exchange was associated with a poor rate of renal recovery in seven of 11 treated. Six patients treated with eculizumab recovered renal function. Contrary to global practice, immunosuppressive therapy to prevent relapse in plasma exchange-treated patients was not adopted due to concerns over treatment-associated complications. Without immunosuppression, the relapse rate was high (five of seven). However, reintroduction of treatment resulted in recovery of renal function. All patients treated with eculizumab achieved sustained remission. Five patients received renal transplants without specific factor H autoantibody-targeted treatment with recurrence in one who also had a functionally significant CFI mutation. Thus, our current practice is to initiate eculizumab therapy for treatment of factor H autoantibody-mediated atypical hemolytic uremic syndrome rather than plasma exchange with or without immunosuppression. Based on this retrospective analysis we see no suggestion of inferior treatment, albeit the strength of our conclusions is limited by the small sample size. Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.

  6. PARENTERAL IRON SUCROSE AS AN ALTERNATIVE TO PACKED CELLS/BLOOD TRANSFUSION IN MODERATE-TO-SEVERE ANAEMIA IN PREGNANCY

    Directory of Open Access Journals (Sweden)

    Rama Sarala

    2016-03-01

    Full Text Available AIM This case study focuses on the efficacy of iron sucrose in moderate-to-severe anaemia in pregnancy and to compare the efficacy of iron sucrose with packed cell transfusion and based on the study to establish whether iron sucrose could be an alternative to packed cells transfusion for the management of moderate-to-severe anaemia complicating pregnancy remote from the term gestation. MATERIALS AND METHODS It is a case control study for a period of 2 years. Women were randomly selected where for the study group 50 patients intravenous iron sucrose was given and for control group 50 patients packed cells transfusion was given. RESULTS The study group and the control group had 50 subjects each. On an average 80% were in the age group of 15-24 yrs. in both groups. In both groups, on an average 85% were with moderate anaemia (6-8 g/dL and 15% were with severe anaemia (<6 g/dL. Mean requirement of iron sucrose for moderate anaemia was 1100 mg and for severe anaemia it was 1300 mg. Mean requirement of packed cells for moderate anaemia was 3 units and for severe anaemia 4-5 units. In iron sucrose group, mean haemoglobin% at baseline 7.1±0.8 g/dL, after 1 week 7.9±0.6, after 4 weeks 11±0.5 g/dL and at delivery 11.7±0.6 g/dL. In packed cells group, mean haemoglobin% at baseline 7.0±0.7 g/dL, after 1 week 10.2±0.5 g/dL, after 4 weeks 10.3±0.5 g/dL and at delivery 10.4±0.4 g/dL. The mean haematocrit values in iron sucrose group at baseline 20.9±2.5%, after 1 week 25.3±2.2% and after 4 weeks 33.6±2.0%. The mean haematocrit values in packed cells group at baseline 20.8±2.3%, after 1 week 30.0±1.9% and after 4 weeks 30.2±2.0%. Mean rise of haematocrit from baseline to 1 week in iron sucrose and packed cells group were 4.4±1.3% and 9.1±2.0% respectively. Mean rise of haematocrit from baseline to 4 weeks in iron sucrose and packed cell group were 12.7±2.1% and 9.3±2.3 respectively. The mean ferritin values in iron sucrose group at baseline

  7. Limited referral to nephrologists from a tertiary geriatric outpatient clinic despite a high prevalence of chronic kidney disease and anaemia

    Directory of Open Access Journals (Sweden)

    Boudville Neil

    2012-08-01

    Full Text Available Abstract Background Chronic kidney disease (CKD is increasing in prevalence world-wide with the largest growth being in the elderly. The aim of this study was to examine the prevalence of CKD in a geriatric outpatient clinic within a tertiary hospital and its association with anaemia and mortality with a focus on the referral patterns towards nephrologists. Methods Retrospective study utilising administrative databases. The cohort was defined as all patients that attended the geriatric outpatient clinics of a single tertiary hospital within the first 3 months of 2006. Patients were followed for 18 months for mortality and referral to a nephrologist. Results The mean Glomerular filtration rate (eGFR of the 439 patients was 67.4 ± 29.1 mL/min/1.73 m2 (44% 2. 11.8% had a haemoglobin 2 (p = 0.0092. Kidney function and anaemia were significantly associated with mortality on multivariate analysis (p = 0.019 and p = 0.0074. After 18 months, 8.8% of patients with CKD were referred to a nephrologist. Conclusion Despite a high prevalence of CKD in patients attending a geriatric outpatient clinic and its association with anaemia and mortality, few of these patients were referred to a nephrologist. An examination of the reasons behind this bias is required.

  8. Safety and Efficacy of Intravenous Ferric Carboxy Maltose in Iron Deficiency Anaemia During Post-partum Period.

    Science.gov (United States)

    Mishra, Vineet; Roy, Priyankar; Gandhi, Khushali; Choudhary, Sumesh; Aggarwal, Rohina; Sokabaj, Shaheen

    2018-01-01

    Iron deficiency is the commonest treatable cause of postpartum anaemia. Parenteral iron therapy results in faster and higher replenishment of iron stores and correction of haemoglobin levels with better compliance. Ferric Carboxy Maltose is an effective and a safe option which can be administered intravenously in single total correction dose without any serious adverse effects.The study was done to evaluate the efficacy and safety of Ferric Carboxy Maltose in the treatment of iron deficiency anaemia in post-natal patients. It was an open, single arm study including 615 women with diagnosis of Iron deficiency anaemia and haemoglobin (Hb) levels between 4gm% and 11gm% from January 2013 to December 2016. Intravenous Ferric Carboxy Maltose(500-1500mg) was administered and the improvement in haemoglobin levels and iron stores were assessed after three weeks of total dose infusion. Out of the 615 women, 595 women were included in the analysis. Most of the women were in the age group of 27-30 years. Most of the women had mild anaemia as per World Health Organisation guidelines. Mean hemoglobin levels significantly increased over a period of three weeks after Ferric Carboxy Maltose administration. Other parameters like total iron binding capacity, Ferritin and Iron also had a significant improvement after Ferric Carboxy Maltose administration. No serious adverse events were observed after Ferric Carboxy Maltose. Intravenous Ferric Carboxy Maltose was an effective and a safe treatment option for iron deficiency anaemia and has an advantage of single administration of high doses without serious adverse effects.

  9. Development of pro-inflammatory phenotype in monocytes after engulfing Hb-activated platelets in hemolytic disorders.

    Science.gov (United States)

    Singhal, Rashi; Chawla, Sheetal; Rathore, Deepak K; Bhasym, Angika; Annarapu, Gowtham K; Sharma, Vandana; Seth, Tulika; Guchhait, Prasenjit

    2017-02-01

    Monocytes and macrophage combat infections and maintain homeostatic balance by engulfing microbes and apoptotic cells, and releasing inflammatory cytokines. Studies have described that these cells develop anti-inflammatory properties upon recycling the free-hemoglobin (Hb) in hemolytic conditions. While investigating the phenotype of monocytes in two hemolytic disorders-paroxysmal nocturnal hemoglobinuria (PNH) and sickle cell disease (SCD), we observed a high number of pro-inflammatory (CD14 + CD16 hi ) monocytes in these patients. We further investigated in vitro the phenotype of these monocytes and found an estimated 55% of CD14 + cells were transformed into the CD14 + CD16 hi subset after engulfing Hb-activated platelets. The CD14 + CD16 hi monocytes, which were positive for both intracellular Hb and CD42b (platelet marker), secreted significant amounts of TNF-α and IL-1β, unlike monocytes treated with only free Hb, which secreted more IL-10. We have shown recently the presence of a high number of Hb-bound hyperactive platelets in patients with both diseases, and further investigated if the monocytes engulfed these activated platelets in vivo. As expected, we found 95% of CD14 + CD16 hi monocytes with both intracellular Hb and CD42b in both diseases, and they expressed high TNF-α. Furthermore our data showed that these monocytes whether from patients or developed in vitro after treatment with Hb-activated platelets, secreted significant amounts of tissue factor. Besides, these CD14 + CD16 hi monocytes displayed significantly decreased phagocytosis of E. coli. Our study therefore suggests that this alteration of monocyte phenotype may play a role in the increased propensity to pro-inflammatory/coagulant complications observed in these hemolytic disorders-PNH and SCD. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Preoperative anaemia and newly diagnosed cancer 1 year after elective total hip and knee arthroplasty

    DEFF Research Database (Denmark)

    Jørgensen, C. C.; Jans, Ø.; Kehlet, H.

    2015-01-01

    BACKGROUND: Preoperative anaemia is a well-established risk factor for use of blood transfusions and postoperative morbidity. Consequently, focus on preoperative evaluation of haemoglobin levels is increasing. In this context, iron deficiency anaemia may be a symptom of undiscovered gastrointesti......BACKGROUND: Preoperative anaemia is a well-established risk factor for use of blood transfusions and postoperative morbidity. Consequently, focus on preoperative evaluation of haemoglobin levels is increasing. In this context, iron deficiency anaemia may be a symptom of undiscovered...

  11. Anaemia in women of reproductive age in Tanzania : A study in Dar es Salaam

    OpenAIRE

    Massawe, Siriel Nanzia

    2002-01-01

    The overall aims of the study were to determine the prevalence of anaemia in women of reproductive age and to investigate the underlying causes, as well as assess the effectiveness of antenatal care (ANC) interventions for anaemia prevention. Consecutive pregnant women booking for ANC (n=2235) were screened for anaemia, followed up and screened again late in pregnancy. Basic ANC interventions included iron and folate supplementation, malaria chemoprophylaxis and referral of severe anaemia cas...

  12. Rh(D) fraction incompatibility causing hemolytic disease of the newborn. Report of two cases in a Chinese family.

    Science.gov (United States)

    Lee, S K; Tham, K T; Cheung, K P; Jenkins, W J

    1982-07-01

    Two cases of hemolytic disease of new born in a Chinese family are reported. The hemolysis was due to the production in the mother of antibodies against fractions A, C, and D of Rh(D) antigen. The fractions were absent in the mother's red blood cells which are Rh(DB) but present in her babies. Rh(DB) may be detected by the use of two types of anti-D sera, one with and the other without anti-DB activity. For transfusion purpose, all DB patients so tested, would be regarded as Rh(D) negative.

  13. A novel strategy for hemolytic uremic syndrome: successful treatment with thrombomodulin α.

    Science.gov (United States)

    Honda, Takashi; Ogata, Shohei; Mineo, Eri; Nagamori, Yukako; Nakamura, Shinya; Bando, Yuki; Ishii, Masahiro

    2013-03-01

    Hemolytic uremic syndrome (HUS) is a life-threatening infectious disease in childhood for which there is no confirmed therapeutic strategy. Endothelial inflammation leading to microthrombosis formation via complement activation is the main pathology of HUS. Thrombomodulin is an endothelial membrane protein that has anticoagulation and anti-inflammatory effects, including the suppression of complement activity. Recombinant human soluble thrombomodulin (rTM) is a novel therapeutic medicine for disseminated intravascular coagulation. We administered rTM to 3 patients with HUS for 7 days and investigated the outcomes in view of the patients' prognoses, changes in biochemical markers, complications, and adverse effects of rTM. Symptoms and laboratory data improved after initiation of rTM in all 3 patients. Abnormal activation of complements was also dramatically suppressed in 1 patient. The patients recovered without any complications or adverse effects of rTM. They were discharged having normal neurologic status and with no renal dysfunction. To our knowledge, this is the first report of rTM being used to treat HUS. These case reports show the positive effect of rTM in patients with HUS. Randomized controlled studies should be performed to assess the efficacy and safety of rTM for children with HUS.

  14. Risk factors for fetal anaemia in a malarious area of Malawi

    NARCIS (Netherlands)

    Brabin, B. J.; Kalanda, B. F.; Verhoeff, F. H.; Chimsuku, L. H.; Broadhead, R. L.

    2004-01-01

    The prevalence of infants born with low cord haemoglobin (fetal anaemia) is high in areas where malaria and iron deficiency anaemia in pregnancy are common. The objective of the present study was to determine risk factors for fetal anaemia in an area of high malaria transmission in southern Malawi.

  15. Susceptibility to β-lactams in β-hemolytic streptococci.

    Science.gov (United States)

    Bonofiglio, Laura; Gagetti, Paula; García Gabarrot, Gabriela; Kaufman, Sara; Mollerach, Marta; Toresani, Inés; Vigliarolo, Laura; von Specht, Martha; Lopardo, Horacio A

    2018-03-13

    Group A (GAS), B (GBS), C (GCS) and G (GGS) β-hemolytic streptococci are important human pathogens. They cause infections of different severity and frequency. Nowadays, after 70 years of use, penicillin is still universally active against GAS, GCS and GGS. However, therapeutic failures have been recorded in 2-28% of pharyngitis cases (median: 12%) attributable to different causes. By contrast, some GBS with reduced susceptibility to penicillin have been described, especially in Japan. In this group of bacteria, it is important to highlight that confirmation by reference methods is mandatory when decreased susceptibility to penicillin is suspected as well as checked for the detection of the mechanisms involved. Copyright © 2017 Asociación Argentina de Microbiología. Publicado por Elsevier España, S.L.U. All rights reserved.

  16. Factors contributing to the development of anaemia in Plasmodium falciparum malaria: what about drug-resistant parasites?

    DEFF Research Database (Denmark)

    Quashie, Neils Ben; Akanmori, Bartholomew D; Ofori-Adjei, David

    2006-01-01

    implicated in its pathogenesis. Since resolution of malaria restores erythropoiesis, we hypothesized that drug-resistant strains of Plasmodium falciparum would increase the risk of severe anaemia developing from initially uncomplicated malaria. Using both in vivo and in vitro drug-sensitivity tests we...... compared the prevalence of drug-resistant malaria between severe malarial anaemia SA and non-anaemic malaria NAM patients. Assessment of treatment outcome using the WHO in vivo criteria showed no significant difference in parasite resistance between the two groups. The mean parasite clearance time was also......-treatment blood levels of chloroquine did not differ much between the two groups. Findings from this study could not therefore implicate drug-resistant parasites in the pathogenesis of severe malarial anaemia....

  17. Research Priorities in the field of Anaemia in India

    Directory of Open Access Journals (Sweden)

    Anju Sinha

    2018-04-01

    Full Text Available Anaemia is a health problem that caused most disability in India over a decade (2005-2016 according to the Global Burden of Disease study(1.  India has the maximum number of anaemic women and children in the world(2. As per the recent National Family Health Survey-4 (2015-16, about 58% children (6-59 months, 53% women (15-49 y and 23% men were suffering from various degrees of anaemia(3. Aanaemia affects the general health of the total population taking toll for high mortality as well as morbidity and attenuating the optimal expression of the potentials of both physical and mental capacity of our population. Improving nutritional status of the vulnerable sections of the population and reducing anaemia is of paramount importance for improving health and the human capital development in the country(4.

  18. Management of autoimmune hemolytic anemia in children and adolescents: A single center experience

    Directory of Open Access Journals (Sweden)

    Nazan Sarper

    2011-09-01

    Full Text Available Objective: To present and discuss the treatment of autoimmune hemolytic anemia (AIHA. Materials and Methods: The medical records of all patients (n=19 diagnosed in a tertiary hematology center between 1999 and 2010 were retrospectively reviewed.Results: Median age at diagnosis of AIHA was 5 years (range: 4 months-17 years. In all, 13 patients had primary (idiopathic AIHA, whereas 2 had primary Evans Syndrome (ES, 2 had autoimmune lymphoproliferative syndrome (ALPS+ES, and 1 had Wiskott-Aldrich syndrome (WAS+AIHA. Among the 13 primary idiopathic AIHA patients, 9 recovered following a 4-8-week course of prednisolone treatment without relapses, whereas 3 patients required a longer course of prednisolone. One AIHA patient that was very resistant to prednisolone recovered after cyclosporine A was added to the treatment. All patients with primary idiopathic AIHA were in remission for a median of 3 years (range: 4 months-10 years at the time this manuscript was written. Among the patients with primary ES, 2 had relapses similar to the ALPS patients. Splenectomy was performed in 1 primary ES patient, who at the time this report was written was also in remission. One ALPS patient required the addition of mycophenolate mofetil due to prednisolone resistance. The WAS patient was treatment resistant and died due to septicemia.Conclusions: Primary AIHA in pediatric patients generally has an acute onset and good response to corticosteroids. Primary or secondary ES has a chronic or relapsing course, and treatment may require other immunosuppressive agents in addition to corticosteroids. Complications of splenectomy must not be underestimated in patients with underlying immunodeficiency. AIHA often causes considerable morbidity and mortality in WAS.

  19. Characterization of autoantibodies in autoimmune hemolytic anemia following treatment with interferon alfa

    International Nuclear Information System (INIS)

    Bencomo Hernandez, Antonio; Gutierrez Diaz, Adys; Avila Cabrera, Onel; Rodriguez, Luis Ramon

    2012-01-01

    We studied 13 patients with chronic myeloid leukemia and autoimmune hemolytic anemia induced by interferon alfa. They underwent tests for immune protein detection and characterization of IgG subclasses in RBCs by direct antiglobulin test (PAD) and the microplate technique. Also they were applied ELISA test for quantifying immunoglobulins in the red blood cells. It was detected the presence of IgG and C3 in 53.84 % of cases, IgG alone in 23.07 % and in 15.38 % were identified IgG and IgA autoantibodies. In 11 patients the presence of IgG1 was showed and also in one case the subclass IgG3 autoantibodies was identified. The ELISA detected antibodies at concentrations of 183 IgG molecules per erythrocyte in a patient with negative PAD. In high-grade hemolysis patients, it was found a concentration of autoantibodies between 1 500 and 3 180 molecules of IgG per erythrocyte, while in low-grade hemolysis patients it behaved between 183 and 1 000 molecules. There was a negative correlation between Hb and plasma haptoglobin values with the number of IgG molecules per erythrocyte and a positive correlation between the latter with the reticulocyte count

  20. C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important Manifestations of Complement System Dysfunction

    Directory of Open Access Journals (Sweden)

    Ravneet Bajwa

    2018-02-01

    Full Text Available The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS, renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement. In this report, we present 2 cases of alternative pathway dysfunction. The 60-year-old female patient had biopsy-proven C3 glomerulopathy, while the 32-year-old female patient was diagnosed with aHUS based on renal dysfunction, thrombocytopenia, anemia, and normal ADAMTS-13 level. The aHUS patient was successfully treated with the monoclonal antibody (eculizumab for complement blockade. The patient with C3 glomerulopathy did not receive the monoclonal antibody. In this patient, management focused on blood pressure and proteinuria control with an angiotensin-converting enzyme inhibitor. This article focuses on the clinical differences, pathophysiology, and treatment of aHUS and C3 glomerulopathy.

  1. Hemolytic Uremic Syndrome: Late Renal Injury and Changing Incidence—A Single Centre Experience in Canada

    Directory of Open Access Journals (Sweden)

    Pierre Robitaille

    2012-01-01

    Full Text Available Aims. To assess trends in the incidence of pediatric diarrhea-associated hemolytic uremic syndrome (D+ HUS and document long-term renal sequelae. Methods. We conducted a retrospective cohort study of children with D+ HUS admitted to a tertiary care pediatric hospital in Montreal, Canada, from 1976 to 2010. In 2010, we recontacted patients admitted before 2000. Results. Of 337 cases, median age at presentation was 3.01 years (range 0.4–14. Yearly incidence peaked in 1988 and 1994-95, returning to near-1977 levels since 2003. Twelve patients (3.6% died and 19 (5.6% experienced long-term renal failure. Almost half (47% The patients required dialysis. Need for dialysis was the best predictor of renal sequelae, accounting for 100% of severe complications. Of children followed ≥1 year (, mean follow-up years, 19 had severe and 18 mild-to-moderate kidney injury, a total sequelae rate, of 18.6%. Ten years or more after-HUS (, mean follow-up years, 8 (9.4% patients demonstrated serious complications and 22 (25.9% mild-to-moderate, including 14 (16% microalbuminuria: total sequelae, 35.3%. Conclusions. Patients with D+ HUS should be monitored at least 5 years, including microalbuminuria testing, especially if dialysis was required. The cause of the declining incidence of D+HUS is elusive. However, conceivably, improved public health education may have played an important role in the prevention of food-borne disease.

  2. Pathophysiological mechanisms of severe anaemia in Malawian children.

    Directory of Open Access Journals (Sweden)

    Michaël Boele van Hensbroek

    2010-09-01

    Full Text Available Severe anaemia is a major cause of morbidity and mortality in African children. The aetiology is multi-factorial, but interventions have often targeted only one or a few causal factors, with limited success.We assessed the contribution of different pathophysiological mechanisms (red cell production failure [RCPF], haemolysis and blood loss to severe anaemia in Malawian children in whom etiological factors have been described previously. More complex associations between etiological factors and the mechanisms were explored using structural equation modelling. In 235 children with severe anaemia (haemoglobin<3.2 mMol/L [5.0 g/dl] studied, RCPF, haemolysis and blood loss were found in 48.1%, 21.7% and 6.9%, respectively. The RCPF figure increased to 86% when a less stringent definition of RCPF was applied. RCPF was the most common mechanism in each of the major etiological subgroups (39.7-59.7%. Multiple aetiologies were common in children with severe anaemia. In the final model, nutritional and infectious factors, including malaria, were directly or indirectly associated with RCPF, but not with haemolysis.RCPF was the most common pathway leading to severe anaemia, from a variety of etiological factors, often found in combination. Unlike haemolysis or blood loss, RCPF is a defect that is likely to persist to a significant degree unless all of its contributing aetiologies are corrected. This provides a further explanation for the limited success of the single factor interventions that have commonly been applied to the prevention or treatment of severe anaemia. Our findings underline the need for a package of measures directed against all of the local aetiologies of this often fatal paediatric syndrome.

  3. Anaemia among pregnant women in northern Tanzania: prevalence, risk factors and effect on perinatal outcomes.

    Science.gov (United States)

    Msuya, Sia E; Hussein, Tamara H; Uriyo, Jacqueline; Sam, Noel E; Stray-Pedersen, Babill

    2011-01-01

    Anaemia during pregnancy is associated with negative maternal and neonatal outcomes. However, there is limited data regarding prevalence and effects of anaemia during pregnancy in northern Tanzania. The objective of this study was to determine the prevalence and possible risk factors for anaemia and its effect on perinatal outcomes among pregnant women attending antenatal care in Moshi Municipality in northern Tanzania. A cohort of pregnant women aged 14-43 years and in their 3rd trimester, was recruited from two primary health care clinics between June 2002 and March 2004. Interviews, anthropometric measurements and haematological examinations were conducted on 2654 consenting women. Perinatal outcomes were recorded during delivery and at 1 week after delivery. Of the 2654 participants, 47.4% had anaemia (haemoglobin [Hb] anaemia (Hb= 9-10.9g/dl), 9.9% had moderate anaemia (Hb =7- 8.9g/dl), and 2.1% had severe anaemia (Hb Anaemia was significantly more prevalent in HIV-positive (56.4%) than in HIV-negative women (46.7%), (P = 0.01). In logistic regression anaemia was independently associated with maternal HIV (OR= 1.5), malaria (OR= 5.2), clinic of recruitment (OR= 1.5) and low income (OR= 1.9). Pregnant women with anaemia were more likely to have low birth weight (LBW) infants. Compared with non-anaemic women, the risk of LBW was 1.6 times and 4.8 times higher for children born to women with moderate and severe anaemia, respectively. In conclusion, anaemia in pregnancy is a severe public health problem in northern Tanzania. Control of maternal anaemia may be one important strategy to prevent LBW in this setting. Measures to prevent malaria and to control anaemia among all pregnant women irrespective of HIV status, should be strengthened. Outside of the health sector broader approaches for anaemia prevention targeting women of lower income, are required.

  4. Radioisotopes of iron in investigation of anaemia in malnutrition

    Energy Technology Data Exchange (ETDEWEB)

    Ahmed, K [Institute of Nuclear Medicine, Dacca (Bangladesh)

    1979-06-01

    Iron absorption from a simple breakfast meal by a normal adult was done by the whole body counting system and blood radioactivity measurement. Results seem to be in good agreement and both the methods are found to be standard for measurements of iron absorption from food. In iron deficiency anaemia associated with malnutrition improvement of nutritional condition has been found to increase both haemoglobin level and iron absorption possibly by way of improving the ability of intestinal mucosa to absorb more iron from food and by better utilization of iron by erythropoietic system. Thus improvement of nutritional status is a prerequisite to treatment of iron deficiency anaemia.

  5. Radioisotopes of iron in investigation of anaemia in malnutrition

    International Nuclear Information System (INIS)

    Ahmed, K.

    1979-01-01

    Iron absorption from a simple breakfast meal by a normal adult was done by the whole body counting system and blood radioactivity measurement. Results seem to be in good agreement and both the methods are found to be standard for measurements of iron absorption from food. In iron deficiency anaemia associated with malnutrition improvement of nutritional condition has been found to increase both haemoglobin level and iron absorption possibly by way of improving the ability of intestinal mucosa to absorb more iron from food and by better utilization of iron by erythropoietic system. Thus improvement of nutritional status is a prerequisite to treatment of iron deficiency anaemia. (author)

  6. Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

    Directory of Open Access Journals (Sweden)

    matteo molica

    2016-04-01

    Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

  7. Dehydration at admission increased the need for dialysis in hemolytic uremic syndrome children.

    Science.gov (United States)

    Balestracci, Alejandro; Martin, Sandra Mariel; Toledo, Ismael; Alvarado, Caupolican; Wainsztein, Raquel Eva

    2012-08-01

    Oligoanuric forms of postdiarrheal hemolytic uremic syndrome (D+ HUS) usually have more severe acute stage and higher risk of chronic sequelae than nonoligoanuric forms. During the diarrheal phase, gastrointestinal losses could lead to dehydration with pre-renal injury enhancing the risk of oligoanuric D+ HUS. Furthermore, it had been shown that intravenous volume expansion during the prodromal phase could decrease the frequency of oligoanuric renal failure. Thus, we performed this retrospective study to determine whether dehydration on admission is associated with increased need for dialysis in D+ HUS patients. Data from 137 children was reviewed, which were divided into two groups according to their hydration status at admission: normohydrated (n = 86) and dehydrated (n = 51). Laboratory parameters of the dehydrated patients reflected expected deteriorations (higher urea, higher hematocrit and lower sodium, bicarbonate, and pH) than normohydrated ones. Likewise, the dehydrated group had a higher rate of vomiting and need for dialysis (70.6 versus 40.7 %, p = 0.0007). Our data suggests that dehydration at hospital admission might represent a concomitant factor aggravating the intrinsic renal disease in D+ HUS patients increasing the need for dialysis. Therefore, the early recognition of patients at risk of D+ HUS is encouraged to guarantee a well-hydrated status.

  8. Investigation of an outbreak of bloody diarrhea complicated with hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Otar Chokoshvili

    2014-12-01

    Full Text Available In July–August 2009, eight patients with bloody diarrhea complicated by hemolytic uremic syndrome (HUS were admitted to hospitals in Tbilisi, Georgia. We started active surveillance in two regions for bloody diarrhea and post-diarrheal HUS. Of 25 case-patients who developed HUS, including the initial 8 cases, half were ⩾15 years old, 67% were female and seven (28% died. No common exposures were identified. Among 20 HUS case-patients tested, Shiga toxin was detected in the stools of 2 patients (one with elevated serum IgG titers to several Escherichia coli serogroups, including O111 and O104. Among 56 persons with only bloody diarrhea, we isolated Shiga toxin-producing E. coli (STEC O104:H4 from 2 and Shigella from 10; 2 had serologic evidence of E. coli O26 infection. These cases may indicate a previously unrecognized burden of HUS in Georgia. We recommend national reporting of HUS and improving STEC detection capacity.

  9. Anaemia and fever in Kidney transplant. The role of human parvovirus B19.

    Science.gov (United States)

    Parodis López, Yanet; Santana Estupiñán, Raquel; Marrero Robayna, Silvia; Gallego Samper, Roberto; Henríquez Palop, Fernando; Rivero Vera, José Carlos; Camacho Galán, Rafael; Pena López, María José; Sablón González, Nery; González Cabrera, Fayna; Oliva Dámaso, Elena; Vega Díaz, Nicanor; Rodríguez Pérez, José Carlos

    Infections remain an issue of particular relevance in renal transplant patients, particularly viral infections. Human parvovirus B19 infection causes severe refractory anaemia, pancytopenia and thrombotic microangiopathy. Its presence is recognized by analysing blood polymerase chain reaction (PCR) and by the discovery of typical giant proerythroblasts in the bone marrow. We report the case of a 65 year-old man with a history of deceased donor renal transplant in September 2014. At 38 days after the transplant, the patient presented progressive anaemia that was resistant to erythropoiesis-stimulating agents. At 64 days after transplant, hyperthermia occurred with progressive deterioration of the patient's general condition. The viral serology and the first blood PCR for human parvovirus B19 were both negative. At 4 months and 19 days after, a bone marrow biopsy was conducted, showing giant erythroblasts with nuclear viral inclusions that were compatible with parvovirus; a PCR in the tissue confirmed the diagnosis. A second blood PCR was positive for parvovirus. After treatment with intravenous immunoglobulin and the temporary discontinuation of mycophenolate mofetil, a complete remission of the disease occurred, although the blood PCR for parvovirus B19 remained positive, so monitoring is necessary for future likely recurrence. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  10. Efficacy of intravenous iron in treating iron deficiency anaemia in patients with inflammatory bowel disease: Are there predictors of response? Eficacia del hierro intravenoso en el tratamiento de la anemia ferropénica en pacientes con enfermedad inflamatoria intestinal: ¿Existen factores predictivos de respuesta?

    Directory of Open Access Journals (Sweden)

    Rocío Ferreiro Iglesias

    2011-05-01

    Full Text Available Introduction: in inflammatory bowel disease (IBD iron deficiency anaemia (IDA is a very common disorder. Until recently, oral iron has been the mainstay therapy, nevertheless it has been associated with intolerance and noncompliance. Therefore, the goal of our study was to evaluate the efficacy of intravenous iron in IDA in IBD patients and the secondary aim was to investigate whether other potential factors could influence in the response to the treatment. Design: an open-label, prospective, consecutive, single centre study. Material and methods: we performed our study in patients with ulcerative colitis (UC or Crohn's disease (CD with severe anaemia or intolerance with oral iron. All of them received intravenous sacarose iron and did biochemistry profile with haemoglobin (Hb. Moreover, the correlation with other variables was studied: age, sex, smoking habit, IBD type, previous surgery and type of surgery and other treatments. Response was defined as Hb increase of ≥ 2 g/dL or normalization of the levels. Results: fifty-four patients were included into the study, 34 (63% with UC y 20 (37% with CD, 18 (33.3% men and 36 women (66.6% and the average was 48 ± 14 years. The total proportion of responders was 52% (SD ± 05; 43% of the patients reached Hb ≥ 2 g/dl and y 9% of them normalized Hb. Only the utilization of 5-ASA was associated with low response to iron treatment (p Introducción: la anemia por déficit de hierro es un problema frecuente en la enfermedad inflamatoria intestinal (EII. Un número no despreciable de pacientes no responde o presenta intolerancia al hierro oral. El objetivo de nuestro estudio es evaluar la eficacia del hierro sacarosa intravenoso (Venofer® en los pacientes con EII así como los potenciales factores que pueden influir en la respuesta al mismo. Diseño: estudio abierto, unicéntrico y con una inclusión consecutiva de casos. Material y métodos: se incluyeron pacientes con colitis ulcerosa (CU y enfermedad

  11. Lung papillary adenocarcinoma complicated with paraneoplastic autoimmune hemolytic anemia: A case report

    OpenAIRE

    Xing, Limin; Wang, Huaquan; Qu, Wen; Fang, Fang; Dong, Qi-e; Shao, Zonghong

    2014-01-01

    A middle-aged woman presented at our facility and was diagnosed after surgery with lung papillary adenocarcinoma. Seven years earlier, she had suffered from autoimmune hemolytic anemia (AIHA), which was refractory. Following lung surgery, the AIHA was cured.

  12. Proteolytic activity and cooperative hemolytic effect of dermatophytes with different species of bacteria

    Directory of Open Access Journals (Sweden)

    Keyvan Pakshir

    2016-12-01

    Conclusion: This study indicated that hemolysin and proteolytic enzymes potentially play a role in dermatophyte pathogenesis and S. aureus could be considered as a main bacterium for creation of co-hemolytic effect in association with dermatophyte species.

  13. Marfan syndrome with antineutrophil cytoplasmic antibody-associated systemic vasculitis presenting as severe anaemia and haematuria after the Bentall procedure.

    Science.gov (United States)

    Sijia, Li; Shuangxin, Liu; Wei, Shi; Yanhai, Cui

    2013-08-01

    One month previously, a 28-year old male underwent an emergency modified Bentall procedure because of Marfan syndrome with acute aortic dissection Stanford Class A. Computed tomography of the chest did not reveal severe graft stenosis of the anastomosis. To explore the cause of anaemia, renal dysfunction and macroscopic haematuria, the patient was tested for antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV). Antimyeloperoxidase antibodies (MPO)-ANCA and antiproteinase 3 antibodies (PR3)-ANCA were strongly positive. Corticosteroid therapy was applied, followed by cyclophosphamide and azathioprine. In response to treatment, the MPO-ANCA and PR3-ANCA levels gradually decreased, proteinuria was alleviated and haemoglobin levels returned to normal after 6 months. This is the first report to highlight haemolytic anaemia and AASV with Marfan syndrome after surgery for aortic dissection.

  14. Single-dose intravenous iron infusion versus red blood cell transfusion for the treatment of severe postpartum anaemia

    DEFF Research Database (Denmark)

    Holm, C; Thomsen, L L; Norgaard, A

    2017-01-01

    BACKGROUND AND OBJECTIVES: There are no randomized trials comparing intravenous iron to RBC transfusion for the treatment of severe postpartum anaemia. The objectives of this study were to evaluate the feasibility of randomizing women with severe postpartum anaemia secondary to postpartum...... haemorrhage to RBC transfusion or intravenous iron, and to describe patient-reported outcomes, and haematological and iron parameters. MATERIALS AND METHODS: Women with a postpartum haemorrhage exceeding 1000 ml and an Hb between 5·6 and 8·1 g/dl were randomized to 1500 mg of intravenous iron (n = 7......) isomaltoside or RBC transfusion (n = 6). Participants completed the Multidimensional Fatigue Inventory and Edinburgh Postnatal Depression Scale, and blood samples were drawn at inclusion, daily during the first week and at weeks 3, 8 and 12. RESULTS: We screened 162 women and included 13 (8...

  15. Primary Biliary Cirrhosis-Related Autoimmune Hemolytic Anemia: Three Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Yu Tian

    2009-08-01

    Full Text Available The association between primary biliary cirrhosis (PBC and autoimmune hemolytic anemia (AIHA is uncommon; only fourteen such case reports have been described. In this report, three patients who developed AIHA on the basis of PBC underwent successful therapy with corticosteroids and ursodeoxycholic acid (UDCA. Patient 3 was more complicated, suffering from PBC, Evans syndrome, Sjögren syndrome and Klinefelter syndrome simultaneously. This has not previously been reported in the world literature. Review of all fifteen cases showed that there is a prominent occurrence sequence that AIHA might take place on the basis of PBC. With sufficient doses of corticosteroids or immunosuppressant therapy, besides hemolysis under effective control, liver function also improved. According to the criteria of secondary AIHA, we may call them PBC-related AIHA. Thus, patients with PBC with serum bilirubin levels rising suddenly should undergo screening for associated hemolysis. Recommended treatment for PBC-related AIHA includes sufficient doses of corticosteroids to control the hemolysis in the acute phase, and immunosuppressant or adequate dose of UDCA to maintain therapy. These case reports have been increasing in recent years, so further reserch is needed to illustrate the incidence and natural courses of these two organ-specific autoimmune diseases.

  16. The adaptive response of mouse tumours to anaemia and retransfusion

    International Nuclear Information System (INIS)

    Hirst, D.G.; Wood, P.J.

    1987-01-01

    Exchange transfusion methods have been developed to alter the haematocrit of tumour-bearing mice. The effects of anaemia and its correction by blood transfusion on the radiosensitivity of two mouse tumours (SCCVII/St and RIF-1) were studied using excision, in vivo/in vitro assay. Acute reduction in haematocrit caused a high degree of radioresistance equivalent to an increase in the hypoxic fractions by factors of 10 (SCCVII/St) and 30 (RIF-1). As the duration of anaemia was prolonged, radioresistance was lost until within about 6 h normal radiosensitivity was observed even though the anaemia persisted. The restoration of the normal haematocrit by red blood cell transfusion after 24 h of anaemia caused increased radiosensitivity equivalent to a reduction in the hypoxic fraction by factors of 5 (SCCVII/St) and 10 (RIF-1), but again the effect was transient and normal radiosensitivity re-established within 24-48 h of retransfusion. Measurements of 14 C misonidazole (MISO) binding to RIF-1 tumours after these procedures indicated changes in the number of hypoxic cells which were qualitatively almost identical to those using the cell survival endpoint, leading to the belief that changes in oxygenation were reponsible for the altered radiosensitivity. (author)

  17. Severe anaemia in childhood cerebral malaria is associated with ...

    African Journals Online (AJOL)

    Background: Severe anaemia in children with cerebral malaria has been associated with respiratory distress secondary to lactic acidosis and/or hypoxia. The ensuing metabolic derangement may further depress the level of consciousness culminating in presentation with profound coma. This association has poorly been ...

  18. Anaemia among clinically well under-fives attending a community ...

    African Journals Online (AJOL)

    A blood sample was collected from each child, and haemoglobin levels were assessed with a point-of-care haemoglobin testing system. Anaemia was defined as having a haemoglobin value <2 standard deviations below age-altitude adjusted normal values. Results. Three-quarters (39/52 – 75%) of children were anaemic.

  19. Nutrition education and anaemia outcome in inner city black children

    African Journals Online (AJOL)

    Seugnet

    die “Special Supplementary Food Program for Wo- ... from the third National Health and Nutrition Ex- amination Survey ... of sickle cell-anaemia or Thalassemia trait, (4) .... iron were whole grain breads, green peas, broc- ... garding risk factors for childhood iron deficiency ... processed by an office-based screening instru-.

  20. Left ventricular systolic function in sickle cell anaemia: an ...

    African Journals Online (AJOL)

    42) and 24.17 ± 4.39 ... Aortic root diameter (Ao), left atrial dimension (LAD), left ventricu- ... Key: *Statistically significant, a= Chi-square, SCA= sickle cell anaemia, SD= standard deviation. ..... of fluctuations in response to changes in sympathetic.

  1. Dietary determination of stunting and anaemia among pre ...

    African Journals Online (AJOL)

    Morocco is undergoing nutrition transition with more than one third of women and children presenting anaemia while about 20% of children under the age of 15 years have stunted growth. Meanwhile the prevalence of obesity is increasing yearly by 0.5 to 1 point among women. Many nutritional strategies have been ...

  2. Risk Factors of Anaemia Among Rural School Children in Kenitra ...

    African Journals Online (AJOL)

    Objective: To determine the prevalence of anaemia and factors associated with iron deficiency among school children in rural Kenitra, Morocco. Methods: 295 students between 6 and 16 years old composed the study group. The level of haemoglobin was measured in a group of 295 school children. The iron status was ...

  3. Prevalence of vitamin a, zinc, iodine deficiency and anaemia among ...

    African Journals Online (AJOL)

    Children are the most nutritionally vulnerable group of society as children are dependants and they are also at a critical stage of the growing process. They need adequate vitamin A, zinc, iron and iodine for their development and school performance. Most often iron deficiency causes anaemia with resultant fatigue and low ...

  4. The effects of iron deficiency and anaemia on primary school ...

    African Journals Online (AJOL)

    the age of 5 years do not reach their developmental potential.[1,2]. ID and ID anaemia (IDA) are widespread health problems among children. Approximately 40% of ..... withdraw at any time without providing a reason, or it affecting their schooling. ... also included in the cases, leaving 45 cases (23.2%) of mild non-. IDA.

  5. Prognosis in canine idiopathic immune-mediated haemolytic anaemia

    NARCIS (Netherlands)

    Piek, C.J.

    2011-01-01

    Canine idiopathic immune-mediated haemolytic anaemia (iIMHA) is one of the most frequently occurring immune-mediated diseases in dogs. A gel-based Coombs' test was shown to perform equally well as a classical Coombs' test. Since the gel-based Coombs' test can be commercially produced and is easy and

  6. Management of severe malarial anaemia in Gambian children

    NARCIS (Netherlands)

    Bojang, K. A.; Palmer, A.; Boele van Hensbroek, M.; Banya, W. A.; Greenwood, B. M.

    1997-01-01

    The optimum management of children with severe malarial anaemia is still uncertain. Hence, we have undertaken a study to determine whether iron treatment is as effective at restoring haemoglobin levels one month after presentation as blood transfusion without iron treatment in children with

  7. Anaemia in HIV infected Nigerian children on HAART | Sadoh ...

    African Journals Online (AJOL)

    Annals of Biomedical Sciences ... Background: Most studies evaluating anaemia and associated factors in HIV infected children have been done on those that are HAART naïve. This study evaluated ... Data on age, gender, CD4 counts, CD4 percentage in those younger than 5years and tuberculosis status were obtained.

  8. p53 downregulates the Fanconi anaemia DNA repair pathway.

    Science.gov (United States)

    Jaber, Sara; Toufektchan, Eléonore; Lejour, Vincent; Bardot, Boris; Toledo, Franck

    2016-04-01

    Germline mutations affecting telomere maintenance or DNA repair may, respectively, cause dyskeratosis congenita or Fanconi anaemia, two clinically related bone marrow failure syndromes. Mice expressing p53(Δ31), a mutant p53 lacking the C terminus, model dyskeratosis congenita. Accordingly, the increased p53 activity in p53(Δ31/Δ31) fibroblasts correlated with a decreased expression of 4 genes implicated in telomere syndromes. Here we show that these cells exhibit decreased mRNA levels for additional genes contributing to telomere metabolism, but also, surprisingly, for 12 genes mutated in Fanconi anaemia. Furthermore, p53(Δ31/Δ31) fibroblasts exhibit a reduced capacity to repair DNA interstrand crosslinks, a typical feature of Fanconi anaemia cells. Importantly, the p53-dependent downregulation of Fanc genes is largely conserved in human cells. Defective DNA repair is known to activate p53, but our results indicate that, conversely, an increased p53 activity may attenuate the Fanconi anaemia DNA repair pathway, defining a positive regulatory feedback loop.

  9. Coinheritance of Β-Thalassemia and Sickle Cell Anaemia

    African Journals Online (AJOL)

    GB

    2016-11-06

    Nov 6, 2016 ... ABSTRACT. BACKGROUND: Genes for haemoglobin S are found in high frequencies in Nigeria. However, there is little information on beta thalassemia in sickle cell anaemia in this population. The clinical presentation of HbS- β thalassemia is enormously variable, ranging from an asymptomatic state to a ...

  10. Anaemia in pregnancy among pregnant women in Lusaka District ...

    African Journals Online (AJOL)

    Objectives: This study investigated the problem of anaemia in pregnancy and its associated factors. Methods: The study involved 216 women booking for antenatal care between September 2015 and January 2016. A structured questionnaire was administered to all eligible women to determine their socio demographic and ...

  11. Prevalence of malaria and anaemia in pregnancy in Ibadan, South ...

    African Journals Online (AJOL)

    The study investigated the episode of malaria infection and anaemia in pregnancy of 226 women. The overall prevalence of malaria infection among pregnant women was 23.08%, while only 7.1% of non-pregnant women were malaria positive. The mean parasite density was significantly higher in the primigravidae than in ...

  12. Malaria Parasitemia and Anaemia among Pregnant Women in ...

    African Journals Online (AJOL)

    A study on the prevalence of malaria parasitaemia and anaemia among pregnant women attending Federal Medical Centre (FMC), Umuahia and Nwachinemere Maternity and Child-Care (NMCCD) Ihie in Umuahia metropolis in Abia State Nigeria was carried out between April and October 2010. Blood samples were ...

  13. Effect of aqueous extract of Hibiscus sabdariffa Calyces on anaemia ...

    African Journals Online (AJOL)

    The effect of aqueous extract of Hibiscus sabdariffa Calyces on anaemia-induced and normal wistar albino rats were investigated using standard protocols. The haematological parameters which include haemoglobin concentration, packed cell volume, erythrocyte sedimentation rate, Red Blood Cell count and White Blood ...

  14. Malnutrition and iron deficiency anaemia in lactating women in ...

    African Journals Online (AJOL)

    Objective: To determine the status of iron deficiency anaemia (IDA) and malnutrition in lactating women. Design: A cross-sectional study. Setting: Six urban slum communities in Teklehaimanot district, Addis Ababa. Subjects: One thousand and seventeen lactating women were enrolled and assessed for their haemoglobin ...

  15. Cardiac Arrhythmias in Children with Sickle Cell Anaemia | Bode ...

    African Journals Online (AJOL)

    Background : Sickle cell anaemia (SCA) is an important cause of morbidity and mortality in tropical Africa. Recurrent episodes of vaso-occlusive crisis often lead to organ ischaemia and/or infarction. Arrythmias are common and reliable manifestations of myocardial ischaemia and often follow infarction. The prevalence and ...

  16. Urinary abnormalities in children with sickle cell anaemia | Ugwu ...

    African Journals Online (AJOL)

    Background: Sickle cell anaemia (SCA) is a health problem worldwide. Almost all the organs of the body are affected by the combined effect of chronic hypoxia, repeated infarction and recurrent infections. Renal function may be progressively impaired in them as a result of sickling in the renal medulla. Microscopic ...

  17. Anaemia in pregnant adolescent girls with malaria and practicing pica.

    Science.gov (United States)

    Intiful, Freda Dzifa; Wiredu, Edwin Kwame; Asare, George Awuku; Asante, Matilda; Adjei, David Nana

    2016-01-01

    Pregnancy during the adolescent period is challenging mainly because of the nutritional demands of both the adolescent and pregnancy period. The risk for anaemia increases especially in developing countries such as Ghana where malaria is endemic and the practice of pica is common. In this study, we sought to determine the prevalence of anaemia, pica practice and malaria infection among pregnant adolescent girls and assess the extent to which these factors are associated. Two hundred and sixty five (265) pregnant adolescent girls were recruited from three hospitals in Accra. Haemoglobin levels, malaria infection and the practice of pica were assessed. Pearson's Chi squared tests were used to determine associations and logistic regression analysis was used to determine the odds of being anaemic. Significance was set at p≤0.05. Anaemia prevalence was 76% with severity ranging from mild (47.8%) to severe (0.8%). About 27.5% were moderately anaemic. Pica was practiced in only 9.1% of the girls. Malaria infection was prevalent in 17.7% of the girls. The logistic regression analysis indicated that pregnant girls with malaria infection were 3.56 times more likely to be anaemic when compared to those without malaria. Also, those who practiced pica were 1.23 times more likely to be anaemic when compared to those who did not practice pica. Anaemia is very prevalent in pregnant adolescent girls and is a public health problem. Drastic measures should be taken to reduce the high prevalence.

  18. Plasmodium falciparum multiplicity correlates with anaemia in symptomatic malaria

    NARCIS (Netherlands)

    Mockenhaupt, Frank P.; Ehrhardt, Stephan; Eggelte, Teunis A.; Markert, Miriam; Anemana, Sylvester; Otchwemah, Rowland; Bienzle, Ulrich

    2003-01-01

    In 366 Ghanaian children with symptomatic Plasmodium falciparum malaria, low haemoglobin levels and severe anaemia were associated with a high multiplicity of infection (MOI) and with distinct merozoite surface protein alleles. High MOI not only reflects premunition but may also contribute to

  19. Immunoglobulin transfusion in hemolytic disease of the newborn: place in therapy

    OpenAIRE

    Mundy CA; Bhatia J

    2015-01-01

    Cynthia A Mundy, Jatinder Bhatia Department of Pediatrics, Division of Neonatology, Georgia Regents University, Children's Hospital of Georgia, GA, USA Abstract: Hemolytic disease of the newborn continues to be a common neonatal disorder that requires a comprehensive understanding on the part of those caring for infants. Common treatments include hydration and phototherapy. Exchange transfusion is used in severe hemolytic disease, but infants undergoing this treatment are exposed to ...

  20. [Contribution of blue-green pigments to hemolytic activity of Pseudomonas aeruginosa cultural fluid].

    Science.gov (United States)

    Pyzh, A É; Nikandrov, V N

    2011-01-01

    To assess the contribution of blue-green pigments of Pseudomonas aeruginosa to hemolytic activity of its cultural fluid. MATERIALS AND METHODS. Eight hospital strains and reference strain ATCC 15442 were used. Growth dynamics of strains as well as features of accumulation of hemolytic and phospholipase activity were studied. Purified samples of pyoverdin and pyocyanin were extracted by gel-chromatography and chloroform extraction methods. Hemolytic and lecitinase activities of the samples as well as effect of active oxygen scavengers and chelating agents on these activities were studied. Dynamics of accumulation of hemolytic activity significantly differed from that of phospholipase activity when strains were grown in liquid medium. Chromatographic separation of the pigments from cultural fluid supernatants sharply reduced its hemolytic activity. Purified samples of pyoverdin and pyocyanin were capable to lyse erythrocytes and chicken egg lecitin. These characteristics of the pigments were inhibited by nitroblue tetrazolium and sensitive to chelating agents. Conclusion. Pyoverdin and pyocyanin of pathogenic strains of P. aeruginosa are capable to lyse erythrocytes and suspension of purified chicken egg lecitin, they contribute to total hemolytic activity of pathogenic strains of Pseudomonas, which is not determined only by phospholipase C produced by microorganism. Lytic activity of the pigments is blocked by nitroblue tetrazolium and susceptible to some chelating agents. Apparently, this activity is mediated by superoxide radical and determined by presence of metals with transient valence in pigments' molecules.

  1. Fatal case of hemolytic-uremic syndrome in an adult due to a rare serogroup O91 Entero hemorrhagic Escherichia coli associated with a Clostridium difficile infection. More than meets the eye

    Directory of Open Access Journals (Sweden)

    Thomas Guillard

    2015-08-01

    Full Text Available Hemolytic-uremic syndrome due to enterohemorrhagic Escherichia coli, belonging to serogroup O91 has rarely been described. We report here a case of post-diarrheal HUS due to EHEC O91 in an elderly patient for whom diagnosis was delayed given a previously diagnosed C. difficile infection. This case highlights the usefulness of Shiga-toxin detection.

  2. Ways to develop the prophylaxis of post-transfusion hemolytic complications

    Directory of Open Access Journals (Sweden)

    B. B. Bahovadinov

    2015-01-01

    Full Text Available Post-transfusion hemolytic complications (РНС remain аn urgent рrоblem in medical practice despite the improvement of selecting methods of compatible blood transfusion for patients. The numbеr of РНС remains still high (1 in 6 000 - 29 000 transfusions. Aim: to analyze cases of РНС registered in health care facilities (HCF in the Republic of Tajikistan. Method of investigation. Retrospective analysis of materials of national аnd regional committees оп investigation of РНС cases, histories fro hospital archives. During the period 1989-2014 in health facilities were registered 86 cases of РНС approximately 850 000 doses of red bооd cell transfusions containing blооd components, or 1 in 9418 doses of red blood cell-containing blood components. РНС reasons were: incompatibility of АВО blооd group system - 32 (37,3 %, antigen D of blооd group Rhesus factor system - 34 (39,53 %, according to minor blood group antigens of Rhesus factor and Kell blood group system (С, с, Е, е, К - 16 (18,6 %. In 4 cases (4,6 % the cases of РНС were hemolytic transfusions of erythrocyte-containing bags as а result of improper storage in domestic refrigeration without control of temperature storage. Causes of development 78 out of 86 РНС (90,69 % were HCF doctors' mistakes, 8 (9,31 % - mistakes of health personnel of health facilities departments of blood transfusion аnd regional blооd centers. Reducing the frequency of PHC is impossible without training physicians оn transfusion medicine, introduction of modern methods of phenotyping erythrocyte antigens of recipients and donors оn major transfusion significant blood group antigens the АВО system by direct and cross-over methods, Rhesus (С, с, Е, е, Kell (К of patients requiring multiple transfusions, as well as to girls and women of childbearing age.

  3. [Wavelength Selection in Hemolytic Evaluation Systems with Spectrophotometry Detection].

    Science.gov (United States)

    Zhang, Hong; Su, Baochang; Ye, Xunda; Luo, Man

    2016-04-01

    Spectrophotometry is a simple hemolytic evaluation method commonly used in new drugs,biomedical materials and blood products.It is for the quantitative analysis of the characteristic absorption peaks of hemoglobin.Therefore,it is essential to select the correct detection wavelength when the evaluation system has influences on the conformation of hemoglobin.Based on the study of changes in the characteristic peaks over time of the hemolysis supernatant in four systems,namely,cell culture medium,phosphate buffered saline(PBS),physiological saline and banked blood preservation solution,using continuous wavelength scanning,the selections of detection wavelength were proposed as follows.In the cell culture medium system,the wavelength of 415 nm should be selected within 4h;,near 408 nm should be selected within 4~72h.In PBS system,within 4h,541 nm,577nm or 415 nm should be selected;4~72h,541 nm,577nm or near 406 nm should be selected.In physiological saline system,within 4h,414 nm should be selected;4~72h,near 405 nm should be selected;within 12 h,541nm or 577 nm could also be selected.In banked blood preservation solution system,within 72 h,415nm,540 nm or 576 nm should be selected.

  4. [Detection and analysis of ABO Hemolytic disease in newborn].

    Science.gov (United States)

    Lin, Zhao-Xia; Dong, Qing-Song

    2014-10-01

    This study was purposed to investigate the incidence and the model of ABO hemolytic disease in newborn (ABO-HDN) and the results of the three hemolysis test, so as to provide the evidences for clinical diagnosis and therapy. A total of 227 cases of maternal-fetal ABO incompatibility from January 2013 to October 2013 in the First Affiliated Hospital of Xiamen University were enrolled in the study. The ABO blood group of newborn and mother was detemined and three hemolysis tests (direct antiglobulin test, free antibody test, RBC antibody release test) were performed. The results indicated that in 227 cases of ABO incompatible pregnancies,186 cases were ABO-HDN (81.94%). There was no significant difference in the incidence between O-A and O-B incompatible pregnancies (P > 0.05). The positive ratio of direct antiglobulin test, free antibody test and RBC antibody release test were 59.14% (110/186), 84.78% (156/186) and 94.62% (176/186) respectively. It is concluded that the incidence of ABO-HDN is high. The main models of ABO-HDN were O-A and O-B. There was no significant difference in the incidence between O-A and O-B incompatible pregnancies. Three hemolysis tests are high sensitivity and are helpful in early diagnosis and early treatment of HDN.

  5. Management of hemolytic-uremic syndrome in children.

    Science.gov (United States)

    Grisaru, Silviu

    2014-01-01

    Acute renal failure associated with a fulminant, life-threatening systemic disease is rare in previously healthy young children; however, when it occurs, the most common cause is hemolytic-uremic syndrome (HUS). In most cases (90%), this abrupt and devastating illness is a result of ingestion of food or drink contaminated with pathogens that produce very potent toxins. Currently, there are no proven treatment options that can directly inactivate the toxin or effectively interfere with the cascade of destructive events triggered by the toxin once it gains access to the bloodstream and binds its receptor. However, HUS is self-limited, and effective supportive management during the acute phase is proven to be a life saver for children affected by HUS. A minority of childhood HUS cases, approximately 5%, are caused by various genetic mutations causing uncontrolled activation of the complement system. These children, who used to have a poor prognosis leading to end-stage renal disease, now have access to exciting new treatment options that can preserve kidney function and avoid disease recurrences. This review provides a summary of the current knowledge on the epidemiology, pathophysiology, and clinical presentation of childhood HUS, focusing on a practical approach to best management measures.

  6. Quiescent complement in nonhuman primates during E coli Shiga toxin-induced hemolytic uremic syndrome and thrombotic microangiopathy.

    Science.gov (United States)

    Lee, Benjamin C; Mayer, Chad L; Leibowitz, Caitlin S; Stearns-Kurosawa, D J; Kurosawa, Shinichiro

    2013-08-01

    Enterohemorrhagic Escherichia coli (EHEC) produce ribosome-inactivating Shiga toxins (Stx1, Stx2) responsible for development of hemolytic uremic syndrome (HUS) and acute kidney injury (AKI). Some patients show complement activation during EHEC infection, raising the possibility of therapeutic targeting of complement for relief. Our juvenile nonhuman primate (Papio baboons) models of endotoxin-free Stx challenge exhibit full spectrum HUS, including thrombocytopenia, hemolytic anemia, and AKI with glomerular thrombotic microangiopathy. There were no significant increases in soluble terminal complement complex (C5b-9) levels after challenge with lethal Stx1 (n = 6) or Stx2 (n = 5) in plasma samples from T0 to euthanasia at 49.5 to 128 hours post-challenge. d-dimer and cell injury markers (HMGB1, histones) confirmed coagulopathy and cell injury. Thus, complement activation is not required for the development of thrombotic microangiopathy and HUS induced by EHEC Shiga toxins in these preclinical models, and benefits or risks of complement inhibition should be studied further for this infection.

  7. [Recombinant erythropoietin as treatment for hyporegenerative anemia following hemolytic disease of the newborn].

    Science.gov (United States)

    Donato, Hugo; Bacciedoni, Viviana; García, Cecilia; Schvartzman, Gabriel; Vain, Néstor

    2009-04-01

    The aim of the study is to report results of erythropoietin treatment for late hyporegenerative anemia in the hemolytic disease of the newborn (HDN). Reports previously published concern only a few cases, with controversial results. Case series report concerning 50 neonates with HDN due to Rh, ABO or KpA antigens, aged more than 7 days. Erythropoietin treatment started when hematocrit dropped to levels requiring transfusion, with an inappropriate reticulocyte response (Reticulocyte Production Index <1). At start of treatment mean age was 24.3 +/- 12.0 days (range 8-65 days), hematocrit 24.1 +/- 2.8% (range 18-30%), and Reticulocyte Production Index 0.34 +/- 0.25 (range 0.05-0.98). Hematocrit and Reticulocyte Production Index showed significant increases after 7 and 14 days of treatment (p <0.001). No difference was observed either between infants with Rh-HDN and ABO-HDN or between Rh-HDN patients with or without intrauterine transfusions. Seven infants (14%) required one packed RBC transfusion during erythropoietin therapy, 2 of them within 72 hours from starting treatment. The percentage of transfused infants showed no difference either between ABO-HDN and Rh-HDN or between Rh-HDN with and without intrauterine transfusions. Moderate, short-lasting neutropenia, not associated to infections, was observed in 11 patients. No other adverse effect was observed. The administration of erythropoietin appears to be a safe and useful therapy. Its efficacy should be confirmed by randomized studies.

  8. Clostridium sordellii as a Cause of Fatal Septic Shock in a Child with Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    Rebekah Beyers

    2014-01-01

    Full Text Available Clostridium sordellii is a toxin producing ubiquitous gram-positive anaerobe, mainly associated with trauma, soft tissue skin infections, and gynecologic infection. We report a unique case of a new strain of Clostridium sordellii (not present in the Center for Disease Control (CDC database infection induced toxic shock syndrome in a previously healthy two-year-old male with colitis-related hemolytic uremic syndrome (HUS. The patient presented with dehydration, vomiting, and bloody diarrhea. He was transferred to the pediatric critical care unit (PICU for initiation of peritoneal dialysis (PD. Due to increased edema and intolerance of PD, he was transitioned to hemodialysis through a femoral vascular catheter. He subsequently developed severe septic shock with persistent leukocytosis and hypotension, resulting in subsequent death. Stool culture confirmed Shiga toxin producing Escherichia coli 0157:H7. A blood culture was positively identified for Clostridium sordellii. Clostridium sordelli is rarely reported in children; to our knowledge this is the first case described in a pediatric patient with HUS.

  9. ADAMTS-13 level in children with severe diarrhea-associated hemolytic uremic syndrome: Unmasking new association

    Directory of Open Access Journals (Sweden)

    Naglaa A Khalifa

    2018-01-01

    Full Text Available Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS. We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case–control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014. The study included 15 consecutive children with typical HUS as well as 15 healthy children as a control group. Routine laboratory investigations were performed. Assessment of serum ADAMTS-13 level was performed using the Quantikine human ADAMTS-13 ELISA kit. Data were analyzed using Statistical Package for Social Sciences version 16. Nonparametric values were expressed as median and range, and the median of two groups was tested by Mann–Whitney test. The serum ADAMTS-13 level was significantly lower in HUS patients when compared to the control group (P < 0.05. There were significant negative correlations between ADAMTS-13 level and duration on dialysis, as well as serum urea and creatinine. Furthermore, there were significant positive correlations between serum ADAMTS-13 level and both hemoglobin level and platelet count. Our study suggests that the ADAMTS-13 level was decreased in children with severe typical HUS and its deficiency correlated with disease severity.

  10. Anaemia worsens early functional outcome after traumatic brain injury: a preliminary study.

    Science.gov (United States)

    Litofsky, N Scott; Miller, Douglas C; Chen, Zhenzhou; Simonyi, Agnes; Klakotskaia, Diana; Giritharan, Andrew; Feng, Qi; McConnell, Diane; Cui, Jiankun; Gu, Zezong

    2018-01-01

    To determine early effects on outcome from traumatic brain injury (TBI) induced by controlled cortical impact (CCI) associated with anaemia in mice. Outcome from TBI with concomitant anaemia would be worse than TBI without anaemia. CCI was induced with electromagnetic impaction in four groups of C57BL/6J mice: sham, sham+anaemia; TBI; and TBI+anaemia. Anaemia was created by withdrawal of 30% of calculated intravascular blood volume and saline replacement of equal volume. Functional outcome was assessed by beam-walking test and open field test (after pre-injury training) on post-injury days 3 and 7. After functional assessment, brains removed from sacrificed animals were pathological reviewed with haematoxylin and eosin, cresyl violet, Luxol Fast Blue, and IBA-1 immunostains. Beam-walking was similar between animals with TBI and TBI+anaemia (p = 0.9). In open field test, animals with TBI+anaemia walked less distance than TBI alone or sham animals on days 3 (p < 0.001) and 7 (p < 0.05), indicating less exploratory and locomotion behaviours. No specific pathologic differences could be identified. Anaemia associated with TBI from CCI is associated with worse outcome as measured by less distance travelled in the open field test at three days than if anaemia is not present.

  11. Elephant trunk in a small-calibre true lumen for chronic aortic dissection: cause of haemolytic anaemia?

    Science.gov (United States)

    Araki, Haruna; Kitamura, Tadashi; Horai, Tetsuya; Shibata, Ko; Miyaji, Kagami

    2014-12-01

    The elephant trunk technique for aortic dissection is useful for reducing false lumen pressure; however, a folded vascular prosthesis inside the aorta can cause haemolysis. The purpose of this study was to investigate whether an elephant trunk in a small-calibre lumen can cause haemolysis. Inpatient and outpatient records were retrospectively reviewed. Two cases of haemolytic anaemia after aortic surgery using the elephant trunk technique were identified from 2011 to 2013. A 64-year-old man, who underwent graft replacement of the ascending aorta for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta and moderate aortic regurgitation. A two-stage surgery was scheduled. Total arch replacement with an elephant trunk in the true lumen and concomitant aortic valve replacement were performed. Postoperatively, he developed severe haemolytic anaemia because of the folded elephant trunk. The anaemia improved after the second surgery, including graft replacement of the descending aorta. Similarly, a 61-year-old man, who underwent total arch replacement for acute Stanford type A aortic dissection, presented with enlargement of the chronic dissection of the descending aorta. Graft replacement of the descending aorta with an elephant trunk inserted into the true lumen was performed. The patient postoperatively developed haemolytic anaemia because of the folded elephant trunk, which improved after additional stent grafting into the elephant trunk. A folded elephant trunk in a small-calibre lumen can cause haemolysis. Therefore, inserting an elephant trunk in a small-calibre true lumen during surgery for chronic aortic dissection should be avoided. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  12. 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

  13. Red cell 2,3-diphosphoglycerate levels in children with hereditary haemolytic anaemias.

    Science.gov (United States)

    Haidas, S; Zannos-Mariolea, L; Matsaniotis, N

    1975-12-01

    The role of red cell 2,3-diphosphoglycerate (2,3-DPG) in increasing the availability of haemoglobin oxygen in neonatal jaundice and hereditary haemolytic anaemias was investigated. Measurements of 2,3-DPG were carried out on 58 normal children and six normal adults, 18 full-term newborns with neonatal jaundice and 57 cases (51 children and six adults) with hereditary haemolytic anaemias. In normal children and adults, with a mean haemoglobin of 12.69 g/dl, mean 2,3-DPG was 14.90 mumol/g Hb. In jaundiced newborns with a mean haemoglobin of 16.04 g/dl mean 2,3-DPG levels were 14.51 mumol/g Hb, i.e. normal. 2,3-DPG levels were increased in patients with beta-thalassaemia major, alpha-thalassaemia, sickle-cell disease, favism, hereditary spherocytosis and in heterozygotes for beta-thalassaemia with increased haemoglobin F. In heterozygotes for beta-thalassaemia with increased haemoglobin A2 only and in sickle cell trait 2,3-DPG levels were normal.

  14. Twin pregnancy complicated by severe hemolytic disease of the fetus and newborn due to anti-g and anti-C.

    Science.gov (United States)

    Trevett, Thomas N; Moise, Kenneth J

    2005-11-01

    Hemolytic disease of the fetus and newborn caused by anti-G antibodies is rare, and in most previously reported cases, leads to a mild anemia. The RhG antigen is usually found in association with both RhD and RhC. We report a case of a twin pregnancy affected by both anti-G and anti-C alloantibodies leading to severe hemolytic disease of the fetus and newborn requiring multiple intrauterine transfusions and prolonged postnatal therapy. A patient with a prolonged history of previously affected pregnancies due to anti-D and anti-C was subsequently found to be affected with anti-G instead. She required aggressive therapy during her pregnancy, initially with intravenous immune globulin and plasmapheresis until umbilical blood sampling and intrauterine transfusions were feasible. Although hemolytic disease of the fetus and newborn due to anti-G antibodies is rare and usually mild, these pregnancies should be followed up closely and in utero therapy should be offered if necessary.

  15. Erythrokinetics, ferrokinetics and red cell survival in sickle cell anaemia under subtropical climatic conditions

    International Nuclear Information System (INIS)

    Cardenas, R.

    1975-01-01

    Ferrokinetic parameters were evaluated with 59 Fe and red-cell survival with 51 Cr by classical techniques in a total of 17 patients with sickle-cell disease. The mean plasma 59 Fe half-disappearance time in these patients was 29.5 min as compared with a normal value of 92 min, and the t1/2 51 Cr 8.0 days as compared with a normal value of 26.0 days. The mean red-cell iron turnover rate was elevated to 9 times normal. The increased destruction of red cells appeared to take place predominantly, though not entirely, in the spleen. Eight of the 17 patients studied were identified as having intercurrent complications, but these did not significantly affect the results of the investigations. A group of 5 boys in whom the red-cell iron turnover rate was elevated to a lesser degree than in the other patients were subjected to more detailed studies of plasma 59 Fe clearance with particular reference to ineffective erythropoiesis. In these patients, the plasma 59 Fe clearance curves showed precocious humps characteristic of ineffective erythropoiesis. Detailed analysis of the results indicated ineffective erythropoiesis corresponding to 3.6, 16.0, 22.6, 32.0 and 50.0 % of the iron initially taken up by the bone marrow. It is concluded that while the anaemia in most patients with sickle-cell disease is mainly due to shortened survival of the circulating red cells, with increased destruction of red cells in the spleen, ineffective erythropoiesis may none the less be an important factor determining the actual degree of this anaemia

  16. Specific features of a neonatal period in infants following intrauterine intravascular blood transfusion for fetal hemolytic disease

    Directory of Open Access Journals (Sweden)

    A. V. Ivanova

    2015-01-01

    Full Text Available The paper gives data on the characteristics of a neonatal period in infants following intrauterine blood transfusion for Rh-induced fetal hemolytic disease. It is shown that the early diagnosis and detection of the signs of fetal hemolytic disease, and intrauterine intravascular blood transfusion may prolong pregnancy, ensure the birth of a baby with normal anthropometric indicators, optimize his/her neonatal period and prognosis of severe hemolytic disease in the fetus and newborn.

  17. Intravenous immunoglobulin G (IVIG) therapy for significant hyperbilirubinemia in ABO hemolytic disease of the newborn.

    Science.gov (United States)

    Miqdad, A M; Abdelbasit, O B; Shaheed, M M; Seidahmed, M Z; Abomelha, A M; Arcala, O P

    2004-09-01

    Although intravenous immunoglobulin G (IVIG) therapy has been reported in hyperbilirubinemia of Rh hemolytic disease, its use in ABO hemolytic disease has been reported in only a few studies. In our institute we have observed that almost 30% of babies with hyperbilirubinemia due to ABO hemolytic disease required exchange transfusion. To determine whether administration of IVIG to newborns with significant hyperbilirubinemia due to ABO hemolytic disease would reduce the need for exchange transfusion as a primary goal in these babies. This was a prospective study involving all newborns with significant hyperbilirubinemia due to direct Coombs-positive ABO hemolytic disease. All healthy term babies with ABO hemolytic disease with positive direct Coombs test in the period between 2000 and 2002 were identified. Significant hyperbilirubinemia was defined as hyperbilirubinemia requiring phototherapy and/or rising by 8.5 micromol/l per h (0.5 mg/dl per h) or more to require exchange transfusion. Babies were randomly assigned into two groups: group 1 (study group) received phototherapy plus IVIG (500 mg/kg); and group 2 (control group) received phototherapy alone. Exchange transfusion was carried out in any group if at any time the bilirubin level reached 340 micromol/l (20 mg/dl) or more, or rose by 8.5 micromol/l per h (0.5 mg/dl per h) in group 2. A total of 112 babies were enrolled over 2 years, 56 in each group. Exchange transfusion was carried out in four babies in the study group, while 16 babies in the control group required exchange. Late anemia was not of concern in either group. No adverse effects related to IVIG administration were recorded. Administration of IVIG to newborns with significant hyperbilirubinemia due to ABO hemolytic disease with positive direct Coomb's test reduces the need for exchange transfusion without producing immediate adverse effects.

  18. End-stage renal disease from hemolytic uremic syndrome in the United States, 1995-2010.

    Science.gov (United States)

    Sexton, Donal J; Reule, Scott; Solid, Craig A; Chen, Shu-Cheng; Collins, Allan J; Foley, Robert N

    2015-10-01

    Management of hemolytic uremic syndrome (HUS) has evolved rapidly, and optimal treatment strategies are controversial. However, it is unknown whether the burden of end-stage renal disease (ESRD) from HUS has changed, and outcomes on dialysis in the United States are not well described. We retrospectively examined data for patients initiating maintenance renal replacement therapy (RRT) (n = 1,557,117), 1995-2010, to define standardized incidence ratios (SIRs) and outcomes of ESRD from HUS) (n = 2241). Overall ESRD rates from HUS in 2001-2002 were 0.5 cases/million per year and were higher for patients characterized by age 40-64 years (0.6), ≥65 years (0.7), female sex (0.6), and non-Hispanic African American race (0.7). Standardized incidence ratios remained unchanged (P ≥ 0.05) between 2001-2002 and 2009-2010 in the overall population. Compared with patients with ESRD from other causes, patients with HUS were more likely to be younger, female, white, and non-Hispanic. Over 5.4 years of follow-up, HUS patients differed from matched controls with ESRD from other causes by lower rates of death (8.3 per 100 person-years in cases vs. 10.4 in controls, P < 0.001), listing for renal transplant (7.6 vs. 8.6 per 100 person-years, P = 0.04), and undergoing transplant (6.9 vs. 9 per 100 person-years, P < 0.001). The incidence of ESRD from HUS appears not to have risen substantially in the last decade. However, given that HUS subtypes could not be determined in this study, these findings should be interpreted with caution. © 2015 International Society for Hemodialysis.

  19. Acute dialysis-associated peritonitis in children with D+ hemolytic uremic syndrome.

    Science.gov (United States)

    Adragna, Marta; Balestracci, Alejandro; García Chervo, Laura; Steinbrun, Silvina; Delgado, Norma; Briones, Liliana

    2012-04-01

    Acute peritoneal dialysis (PD) is the preferred therapy for renal replacement in children with post-diarrheal hemolytic uremic syndrome (D+ HUS), but peritonitis remains a frequent complication of this procedure. We reviewed data from 149 patients with D+ HUS who had undergone acute PD with the aim of determining the prevalence and risk factors for the development of peritonitis. A total of 36 patients (24.2%) presented peritonitis. The median onset of peritonitis manifestations was 6 (range 2-18) days after the initiation of dialysis treatment, and Gram-positive microorganisms were the predominant bacterial type isolated (15/36 patients). The patients were divided into two groups: with or without peritonitis, respectively. Univariate analysis revealed that a longer duration of the oligoanuric period, more days of dialysis, catheter replacement, stay in the intensive care unit, and hypoalbuminemia were significantly associated to the development of peritonitis. The multivariate analysis, controlled by duration of PD, identified the following independent risk factors for peritonitis: catheter replacement [p = 0.037, odds ratio (OR) 1.33, 95% confidence interval (CI) 1.02-1.73], stay in intensive care unit (p = 0.0001, OR 2.62, 95% CI 1.65-4.19), and hypoalbuminemia (p = 0.0076, OR 1.45, 95% CI 1.10-1.91). Based on these findings, we conclude that the optimization of the aseptic technique during catheter manipulation and early nutritional support are targets for the prevention of peritonitis, especially in critically ill patients.

  20. Microangiopathic Hemolytic Anemia Following Three Different Species of Hump-Nosed Pit Viper (Genus: Hypnale) Envenoming in Sri Lanka.

    Science.gov (United States)

    Namal Rathnayaka, Rathnayaka Mudiyanselage M K; Ranathunga, Anusha Nishanthi; Kularatne, Senanayake A M; Rajapakse, Jayanthe; Ranasinghe, Shirani; Jayathunga, Radha

    2018-03-01

    There are 3 species of hump-nosed pit vipers in Sri Lanka: Hypnale hypnale, Hypnale zara, and Hypnale nepa. The latter 2 are endemic to the country. Microangiopathic hemolytic anemia (MAHA) is a known complication of hump-nosed pit viper bites. It was previously documented as a complication of general viper bites and not species specific. We report a series of 3 patients who developed MAHA after being bitten by each species of hump-nosed pit viper. The first patient was bitten by H hypnale and developed a severe form of MAHA associated with acute kidney injury and thrombocytopenia falling into the category of thrombotic microangiopathy. The other 2 developed MAHA that resolved without any complications. Copyright © 2017 Wilderness Medical Society. Published by Elsevier Inc. All rights reserved.

  1. N-terminal amphipathic helix as a trigger of hemolytic activity in antimicrobial peptides: a case study in latarcins.

    Science.gov (United States)

    Polyansky, Anton A; Vassilevski, Alexander A; Volynsky, Pavel E; Vorontsova, Olga V; Samsonova, Olga V; Egorova, Natalya S; Krylov, Nicolay A; Feofanov, Alexei V; Arseniev, Alexander S; Grishin, Eugene V; Efremov, Roman G

    2009-07-21

    In silico structural analyses of sets of alpha-helical antimicrobial peptides (AMPs) are performed. Differences between hemolytic and non-hemolytic AMPs are revealed in organization of their N-terminal region. A parameter related to hydrophobicity of the N-terminal part is proposed as a measure of the peptide propensity to exhibit hemolytic and other unwanted cytotoxic activities. Based on the information acquired, a rational approach for selective removal of these properties in AMPs is suggested. A proof of concept is gained through engineering specific mutations that resulted in elimination of the hemolytic activity of AMPs (latarcins) while leaving the beneficial antimicrobial effect intact.

  2. Radiation resistance of a hemolytic micrococcus isolated from chicken meat

    International Nuclear Information System (INIS)

    Tan, S.T.

    1982-01-01

    The effects of environmental factors on a highly radiation-resistant hemolytic micrococcus isolated from chicken meat were studied. NaCl tolerance and gamma radiation resistance of the cells were growth phase-related. The cells were resistant to injury from drying or freezing/thawing. Under certain conditions, cells in the frozen state required approximately 5 Mrad to inactivate 90% of the population; 0.2 Mrad injured an equivalent proportion. Survival curve of the cells heated at 60 0 C showed a unique pattern which was in three distinct phases. Heat-stressed cells were much more sensitive to radiation inactivation than unheated cells. When suspended in fresh m-Plate Count Broth (PCB), the injured cells repaired without multiplication during incubation at 32 0 C. The repair process in this bacterium, however, was slower compared to thermally injured organisms studied by other workers. An improved replica-plating technique, was devised for isolation of radiation-sensitive mutants of pigmented bacteria. A simple method to demonstrate radiation-inducible radiation resistance in microbial cells was developed. The new method required neither washing/centrifugation nor procedures for cell enumeration. Mutagenesis treatment of radiation-resistant micrococcal bacterium with N-methyl-N'-nitro-N-nitrosoguanidine (NTG) followed by FPR and screening steps resulted in isolation of two radiation-sensitive mutants. The more sensitive mutant strain, designated as 702, was seven times as sensitive to gamma or UC radiation as the wild type. No apparent difference was observed between 702 and the wild type in (1) cell morphology, colonial morphology, and pigment production or (2) tolerance to NaCl, drying/storage, freezing/thawing, and heating. Sodium dodecyl sulfate treatment (for curing) of wild type did not result in isolation of a radiation-sensitive mutant

  3. Late Onset Cobalamin Disorder and Hemolytic Uremic Syndrome: A Rare Cause of Nephrotic Syndrome

    Directory of Open Access Journals (Sweden)

    Gianluigi Ardissino

    2017-01-01

    Full Text Available Hemolytic uremic syndrome (HUS is an unrare and severe thrombotic microangiopathy (TMA caused by several pathogenetic mechanisms among which Shiga toxin-producing Escherichia coli infections and complement dysregulation are the most common. However, very rarely and particularly in neonates and infants, disorders of cobalamin metabolism (CblC can present with or be complicated by TMA. Herein we describe a case of atypical HUS (aHUS related to CblC disease which first presented in a previously healthy boy at age of 13.6 years. The clinical picture was initially dominated by nephrotic range proteinuria and severe hypertension followed by renal failure. The specific treatment with high dose of hydroxycobalamin rapidly obtained the remission of TMA and the complete recovery of renal function. We conclude that plasma homocysteine and methionine determinations together with urine organic acid analysis should be included in the diagnostic work-up of any patient with TMA and/or nephrotic syndrome regardless of age.

  4. Prevention of hemolytic disease of the fetus and newborn: what have we learned from animal models?

    Science.gov (United States)

    Cruz-Leal, Yoelys; Marjoram, Danielle; Lazarus, Alan H

    2017-11-01

    This review aims to highlight recent advances in our understanding of how anti-red blood cell (RBC) antibodies prevent erythrocyte immunization with an emphasis on new murine models. New murine models with clinically relevant human erythrocyte antigens have been used to understand the alloimmunization process and its inhibition. The search to elucidate the mechanism of action of IgG-mediated inhibition of erythrocyte alloimmunization has provided new evidence in support of a potential role for epitope masking, immune deviation and/or antigen modulation in this process. In addition, recent evidence suggests that blends of monoclonal antibodies targeting nonoverlapping epitopes on the RBC surface can improve the efficacy of monoclonal antibodies approaching that of polyclonal IgG. Animal models with defined alloantigens have helped to identify important mechanistic components that lead to alloimmunization and its inhibition by IgG. A better understanding of the underlying mechanisms leading to hemolytic disease of the fetus and newborn is required to develop the most effective prevention strategies for future patients.

  5. The clinical and radiological features of Fanconi's anaemia pictorial review

    Energy Technology Data Exchange (ETDEWEB)

    De Kerviler, E.; Guermazi, A.; Zagdanski, A.-M.; Gluckman, E.; Frija, J

    2000-05-01

    Fanconi's anaemia is a severe refractory anaemia, associated with congenital malformations in approximately two-thirds of cases. Although these malformations may involve every organ system, suggestive dysmorphic features include growth retardation, radial ray deformities and urinary malformations. These malformations are not specific for Fanconi's anaemia, but should be recognized during pregnancy, or later in childhood, and suggest the possibility of inherited haematopoiesis disorders. De Kerviler, E. (2000)

  6. Risk factors and birth outcomes of anaemia in early pregnancy in a nulliparous cohort.

    Directory of Open Access Journals (Sweden)

    Gwinyai Masukume

    Full Text Available Anaemia in pregnancy is a major public health and economic problem worldwide, that contributes to both maternal and fetal morbidity and mortality.The aim of the study was to calculate the prevalence of anaemia in early pregnancy in a cohort of 'low risk' women participating in a large international multicentre prospective study (n = 5 609, to identify the modifiable risk factors for anaemia in pregnancy in this cohort, and to compare the birth outcomes between pregnancies with and without anaemia in early gestation.The study is an analysis of data that were collected prospectively during the Screening for Pregnancy Endpoints study. Anaemia was defined according to the World Health Organization's definition of anaemia in pregnancy (haemoglobin < 11g/dL. Binary logistic regression with adjustment for potential confounders (country, maternal age, having a marital partner, ethnic origin, years of schooling, and having paid work was the main method of analysis.The hallmark findings were the low prevalence of anaemia (2.2%, that having no marital partner was an independent risk factor for having anaemia (OR 1.34, 95% CI 1.01-1.78, and that there was no statistically significant effect of anaemia on adverse pregnancy outcomes (small for gestational age, pre-tem birth, mode of delivery, low birth weight, APGAR score < 7 at one and five minutes. Adverse pregnancy outcomes were however more common in those with anaemia than in those without.In this low risk healthy pregnant population we found a low anaemia rate. The absence of a marital partner was a non-modifiable factor, albeit one which may reflect a variety of confounding factors, that should be considered for addition to anaemia's conceptual framework of determinants. Although not statistically significant, clinically, a trend towards a higher risk of adverse pregnancy outcomes was observed in women that were anaemic in early pregnancy.

  7. A study on status of anaemia in pregnant women attending urban health training centre, RIMS, Ranchi

    Directory of Open Access Journals (Sweden)

    Vijay Kumar

    2014-12-01

    Full Text Available Background: Anaemia in pregnant women has been regarded as very dangerous as it causes many maternal, fetal and neonatal complications. Fetal growth and pregnancy outcome largely depend upon the status of anaemia in pregnant women. Anaemia affects pregnant  women all over the world - 52% in  developing  countries  compared  with  23%  in  the  developed  world. The difference in prevalence of anaemia in different parts of India including Jharkhand can be attributed to the different factors. A knowledge of these factors associated with anemia will help to formulate multipronged strategies to curtail this important public health problem in pregnancy. Aims & Objectives: (1 To know the socio-demographic profile of pregnant women attending Urban Health and Training Centre (UHTC, RIMS, Ranchi. (2 To know the status of anaemia among those pregnant women and its association with different factors. Material & Methods: A descriptive cross-sectional study done at ANC clinic of UHTC, RIMS, Ranchi to determine the status of anaemia in pregnant women and various socio-demographic factors associated with it. Hemoglobin level of 149 pregnant women selected by consecutive sampling was estimated by Cyanmethemoglobin method. Statistical Analysis: Template generated in MS excel sheet and analysis was done on SPSS software. Result: Out of total 149 pregnant women anaemia was found to be present in 99 (66.4% women. A statistically significant association of anaemia (p<.05 was found with parity and birth interval from last birth.  But the association of anaemia with ethnicity, education and other factors like gestational age (trimester was not found to be statistically significant (p>.05.  Conclusion: Occurrence of anaemia was much higher in this area as compared to national average. It indicates that the anaemia continues to be a major public health problem.  Efforts should be geared towards the early detection and treatment of anaemia before delivery. 

  8. Risk factors and birth outcomes of anaemia in early pregnancy in a nulliparous cohort.

    Science.gov (United States)

    Masukume, Gwinyai; Khashan, Ali S; Kenny, Louise C; Baker, Philip N; Nelson, Gill

    2015-01-01

    Anaemia in pregnancy is a major public health and economic problem worldwide, that contributes to both maternal and fetal morbidity and mortality. The aim of the study was to calculate the prevalence of anaemia in early pregnancy in a cohort of 'low risk' women participating in a large international multicentre prospective study (n = 5 609), to identify the modifiable risk factors for anaemia in pregnancy in this cohort, and to compare the birth outcomes between pregnancies with and without anaemia in early gestation. The study is an analysis of data that were collected prospectively during the Screening for Pregnancy Endpoints study. Anaemia was defined according to the World Health Organization's definition of anaemia in pregnancy (haemoglobin prevalence of anaemia (2.2%), that having no marital partner was an independent risk factor for having anaemia (OR 1.34, 95% CI 1.01-1.78), and that there was no statistically significant effect of anaemia on adverse pregnancy outcomes (small for gestational age, pre-tem birth, mode of delivery, low birth weight, APGAR score pregnancy outcomes were however more common in those with anaemia than in those without. In this low risk healthy pregnant population we found a low anaemia rate. The absence of a marital partner was a non-modifiable factor, albeit one which may reflect a variety of confounding factors, that should be considered for addition to anaemia's conceptual framework of determinants. Although not statistically significant, clinically, a trend towards a higher risk of adverse pregnancy outcomes was observed in women that were anaemic in early pregnancy.

  9. Iron deficiency anaemia in pregnancy: The role of parenteral iron.

    Science.gov (United States)

    Esen, Umo I

    2017-01-01

    Maternal and perinatal morbidity and mortality remain major challenges in the delivery of safe maternity care worldwide. Anaemia in pregnancy is an important contributor to this dismal picture, especially where blood transfusion services are poorly developed. An early diagnosis and treatment of iron deficiency anaemia in pregnancy using the new generation dextran-free parenteral iron preparations can save lives and reduce morbidity in selected pregnancies. It is time to cast aside the fears associated with the use of the old parenteral iron preparations which were associated a high incidence of anaphylaxis, and embrace the use of new parenteral iron products which have better side effect profiles and can deliver total dose infusions without the need for test dosing. In selected women, the benefits of this treatment far outweigh any disadvantages.

  10. INTRAVENOUS IRON-SUCROSE COMPLEX THERAPY IN PREGNANT WOMEN WITH IRON DEFICIENCY ANAEMIA- A STUDY IN TERTIARY CENTRE

    Directory of Open Access Journals (Sweden)

    Todak Taba

    2017-11-01

    Full Text Available BACKGROUND Anaemia in pregnancy continues to be a major public health problem with 54.96% of the pregnant population suffering from it in our setup. Despite the National Anaemia Prophylaxis Programme, anaemia complicating pregnancy continues to be a widespread problem with adverse effects on maternal and foetal outcome. The aim of the study is to find out an alternate iron therapy in the form of intravenous iron-sucrose and to determine its therapeutic effectiveness, safety and compliance in the management of anaemic expectant mother and to compare it with that of conventional oral iron therapy. MATERIALS AND METHODS The study was a randomised controlled clinical trial carried out in the Department of Obstetrics and Gynaecology in collaboration with the Department of Biochemistry, Regional Institute of Medical Sciences (RIMS, Imphal. 100 pregnant women in second or third trimester with mild or moderate anaemia were selected, 50 as study group (intravenous iron and 50 as controls (oral iron. Initial evaluation included complete blood count and serum ferritin level and reevaluated on the 14th and 28th day of initiation of therapy. RESULTS Majority of patients (42% in the study as well as control group were between 26-30 years of age. The mean ± SD increase in haemoglobin and ferritin levels on 28th day were 2.66 ± 0.34 gm/dL and 27.65 ± 1.80 ng/mL in study group and 1.55 ± 0.23 gm/dL and 16.89 ± 0.76 ng/mL in control group respectively, both of which were statistically significant. CONCLUSION The mean haemoglobin and serum ferritin levels throughout the treatment were significantly higher in the intravenous ironsucrose group than in the orally administered iron group and significantly higher number of patients achieved the target haemoglobin of 11.0 gm/dL after 28 days of treatment. This reduces the blood transfusion rates in pregnant women with severe anaemia near term.

  11. Rationale and design of the oral HEMe iron polypeptide Against Treatment with Oral Controlled Release Iron Tablets trial for the correction of anaemia in peritoneal dialysis patients (HEMATOCRIT trial

    Directory of Open Access Journals (Sweden)

    Isbel Nicole M

    2009-07-01

    Full Text Available Abstract Background The main hypothesis of this study is that oral heme iron polypeptide (HIP; Proferrin® ES administration will more effectively augment iron stores in erythropoietic stimulatory agent (ESA-treated peritoneal dialysis (PD patients than conventional oral iron supplementation (Ferrogradumet®. Methods Inclusion criteria are peritoneal dialysis patients treated with darbepoietin alpha (DPO; Aranesp®, Amgen for ≥ 1 month. Patients will be randomized 1:1 to receive either slow-release ferrous sulphate (1 tablet twice daily; control or HIP (1 tablet twice daily for a period of 6 months. The study will follow an open-label design but outcome assessors will be blinded to study treatment. During the 6-month study period, haemoglobin levels will be measured monthly and iron studies (including transferring saturation [TSAT] measurements will be performed bi-monthly. The primary outcome measure will be the difference in TSAT levels between the 2 groups at the end of the 6 month study period, adjusted for baseline values using analysis of covariance (ANCOVA. Secondary outcome measures will include serum ferritin concentration, haemoglobin level, DPO dosage, Key's index (DPO dosage divided by haemoglobin concentration, and occurrence of adverse events (especially gastrointestinal adverse events. Discussion This investigator-initiated multicentre study has been designed to provide evidence to help nephrologists and their peritoneal dialysis patients determine whether HIP administration more effectively augments iron stores in ESP-treated PD patients than conventional oral iron supplementation. Trial Registration Australia New Zealand Clinical Trials Registry number ACTRN12609000432213.

  12. Atypical Hemolytic Uremic Syndrome post Kidney Transplantation: Two Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sami eAlasfar

    2014-12-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1-2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (Infection-induced HUS and is frequently characterized by relapses that leads to end stage renal disease (ESRD. For a long time, kidney transplantation for these patients was contraindicated because of high rate of recurrence and subsequent renal graft loss. The post-kidney transplantation recurrence rate largely depends on the pathogenetic mechanisms involved. However, over the past several years, advancements in the understanding and therapeutics of aHUS have allowed successful kidney transplantation in these patients. Eculizumab, which is a complement C5 antibody that inhibits complement factor 5a (C5a and subsequent formation of the membrane attack complex, has been used in prevention and treatment of post-transplant aHUS recurrence. In this paper, we present two new cases of aHUS patients who underwent successful kidney transplantation in our center with the use of prophylactic and maintenance eculizumab therapy that have not been published before. The purpose of reporting these two cases is to emphasize the importance of using eculizumab as a prophylactic therapy to prevent aHUS recurrence post transplant in high-risk patients. We will also review the current understanding of the genetics of aHUS, the pathogenesis of its recurrence after kidney transplantation, and strategies for prevention and treatment of post-transplant aHUS recurrence.

  13. [Acute oliguric renal failure and haemolytic anaemia following infectious mononucleosis].

    Science.gov (United States)

    Brkovic, Natasa; Jørgensen, Kit Riegels; Rosenbæk, Jeppe Bakkestrøm; Pedersen, Erling Bjerregaard

    2015-11-09

    A 19-year-old man was admitted to hospital due to fatigue, nausea, abdominal pain and faint. He was pale and icteric, awake with sufficient respiration and circulation. He had infectious mononucleosis complicated with acute oliguric renal failure and severe haemolytic anaemia with a positive Coombs test. He had a cold agglutinin syndrome. The treatment comprised intermittent haemodialysis, plasmapheresis and heating. He recovered completely after two months.

  14. Maternal Risk Factors for Childhood Anaemia in Ethiopia

    African Journals Online (AJOL)

    AJRH Managing Editor

    A total of 8260 children between the ages of 6-59 months were analyzed to ... Maternal anaemia and socio-economic status were found to be associated with ... était de 10,7 (2,2) g / dl et 50,3% étaient anémiques. ... economic status, environmental factors, food ... For the current ... Anthelmintic treatment in the previous six.

  15. Prevention of post-operative anaemia in hip and knee arthroplasty - a systematic review

    DEFF Research Database (Denmark)

    Khan, Nissa; Troelsen, Anders; Husted, Henrik

    2015-01-01

    and length of hospital stay. The papers were evidence-graded. Non-randomised clinical studies and papers not concerning total hip or knee arthroplasty were excluded as were studies lacking a control group. Subanalyses were performed for tran-examic acid, tourniquet and fibrin use. RESULTS: A total of 49...... studies were found eligible which is equivalent to a total of 4,752 patients. Tranexamic acid administered either orally, topically, intravenously or in combination decreased blood loss, increased the post-operative haemoglobin level, decreased the number of patients receiving blood transfusions...... and minimised the length of stay. A similar result was found for fibrin spray in total hip arthroplasty. However, for total knee arthroplasty, the outcome was blurred. Tourniquet use was uniformly not significant in the measured parameters. CONCLUSIONS: Tranexamic acid is useful in managing anaemia and blood...

  16. Anaemia and fever in kidney transplant. The role of human parvovirus B19

    Directory of Open Access Journals (Sweden)

    Yanet Parodis López

    2017-03-01

    We report the case of a 65 year-old man with a history of deceased donor renal transplant in September 2014. At 38 days after the transplant, the patient presented progressive anaemia that was resistant to erythropoiesis-stimulating agents. At 64 days after transplant, hyperthermia occurred with progressive deterioration of the patient's general condition. The viral serology and the first blood PCR for human parvovirus B19 were both negative. At 4 months and 19 days after, a bone marrow biopsy was conducted, showing giant erythroblasts with nuclear viral inclusions that were compatible with parvovirus; a PCR in the tissue confirmed the diagnosis. A second blood PCR was positive for parvovirus. After treatment with intravenous immunoglobulin and the temporary discontinuation of mycophenolate mofetil, a complete remission of the disease occurred, although the blood PCR for parvovirus B19 remained positive, so monitoring is necessary for future likely recurrence.

  17. Stevia rebaudiana Bertoni effect on the hemolytic potential of Listeria monocytogenes.

    Science.gov (United States)

    Sansano, S; Rivas, A; Pina-Pérez, M C; Martinez, A; Rodrigo, D

    2017-06-05

    The effect of Stevia rebaudiana Bertoni on the hemolytic potential of Listeria monocytogenes was studied by means of the assessment of the Listeriolysin O (LLO) production. The three factors under study, stevia concentration in the range [0-2.5] % (w/v), incubation temperature (10 and 37°C), and exposure time (0-65h) significantly affected (p≤0.05) the hemolytic activity of L. monocytogenes. Results showed that at the lower incubation temperature the hemolytic potential of the bacterium was significantly reduced, from 100% at 37°C to 8% at 10°C (after 65h of incubation) in unsupplemented substrate (0% stevia). Irrespective of the temperature, 10 or 37°C, supplementation of the medium with stevia at 2.5 % (w/v) reduced the bacterium's hemolytic activity by a maximum of 100%. Furthermore, the time of exposure to 2.5 % (w/v) stevia concentration was also a significant factor reducing the hemolytic capability of L. monocytogenes. The possibility of reducing the pathogenic potential of L. monocytogenes (hemolysis) by exposure to stevia should be confirmed in real food matrices, opening a research niche with a valuable future impact on food safety. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. A retrospective study of the prevalence of anaemia in pregnancy at booking in Niger Delta, Nigeria.

    Science.gov (United States)

    Okoh, Dorathry Adaunwo; Iyalla, Caroline; Omunakwe, Hannah; Iwo-Amah, Rose Sitonma; Nwabuko, Collins

    2016-07-01

    We reviewed the records of antenatal clinic attendees over a period of 9 years to determine the prevalence of anaemia at booking. The laboratory records of 8751 out of a total of 37,506 pregnant women who booked for antenatal care between 2004 and 2013 at the BMSH were reviewed. The effects of maternal age, educational status, parity, gestational age, haemoglobin genotype and infections on the prevalence of anaemia were investigated. The prevalence of anaemia at booking was 69.6%, most of whom had moderate anaemia. Anaemia was significantly prevalent in the 10-19 year age group, and in women with secondary education, in their 2nd trimester and with SS genotype. Anaemia also increased with gestational age, this however was not statistically significant. There was no statistical difference between those who are human immunodeficiency virus (HIV) positive and had anaemia and those who are HIV negative who also had anaemia. This study shows that anaemia in pregnant women is still unacceptably high considering the consequences and despite interventions on the ground to reduce prevalence. There is a need to review the intervention measures with emphasis on programmes that would increase awareness among pregnant women and the general public.

  19. Assessment of knowledge level on anaemia among pregnant women in Putrajaya

    Science.gov (United States)

    Adznam, Siti Nur'Hidayah; Sedek, Razalee; Kasim, Zalifah Mohd

    2018-04-01

    Anaemia during pregnancy is a common problem which affects both the mother's and her child's health. The aim of this study was to determine knowledge level on anaemia among pregnant women in Putrajaya. This study was also been carried out to identify the relationship between knowledge according to socio-demographic and antenatal characteristics. A total of 370 subjects were participated in this study. Subjects comprised of pregnant women who attended four health clinics in Putrajaya to undergo first antenatal visit for the current pregnancy. Socio-demographic information, antenatal characteristics and knowledge related to anaemia were collected using questionnaires. Blood samples were taken to identify hemoglobin level of subjects using Sysmex Hematology Analyzer machine (Sysmex Europe GmbH). The mean age of subjects was 30.2 ± 4.2 years and the mean hemoglobin level was 12.1 ± 4.8 g/dL. The median score for subject's knowledge towards anaemia was 84.2 corresponding to a high level of anaemia knowledge. Result of this study revealed that 55.7% of subjects had high knowledge on anaemia during pregnancy while 28.6% had moderate knowledge followed by 15.7% with low knowledge score. Most subjects correctly answered the general questions on the survey but under the assumption regarding the cause of anaemia. They were also lacking in knowledge regarding the risks of anaemia. Knowledge score was significantly associated with gestational week (peducation for pregnant women with regard to anaemia.

  20. Malaria, Moderate to Severe Anaemia, and Malarial Anaemia in Children at Presentation to Hospital in the Mount Cameroon Area: A Cross-Sectional Study

    Science.gov (United States)

    Taiwe, Germain Sotoing

    2016-01-01

    Background. Malaria remains a major killer of children in Sub-Saharan Africa, while anaemia is a public health problem with significant morbidity and mortality. Examining the factors associated with moderate to severe anaemia (MdSA) and malarial anaemia as well as the haematological characteristics is essential. Methodology. Children (1–14 years) at presentation at the Regional Hospital Annex-Buea were examined clinically and blood samples were collected for malaria parasite detection and full blood count evaluation. Results. Plasmodium falciparum, anaemia, and malarial anaemia occurred in 33.8%, 62.0%, and 23.6% of the 216 children, respectively. Anaemia prevalence was significantly higher in malaria parasite positive children and those with fever than their respective counterparts. MdSA and moderate to severe malarial anaemia (MdSMA) were detected in 38.0% and 15.3% of the participants, respectively. The prevalence of MdSA was significantly higher in children whose household head had no formal education, resided in the lowland, or was febrile, while MdSMA was significantly higher in febrile children only. Children with MdSMA had significantly lower mean white blood cell, lymphocyte, and platelet counts while the mean granulocyte count was significantly higher. Conclusion. Being febrile was the only predictor of both MdSA and MdSMA. More haematological insult occurred in children with MdSMA compared to MdSA. PMID:27895939

  1. Origins of the E. coli strain causing an outbreak of hemolytic-uremic syndrome in Germany

    DEFF Research Database (Denmark)

    Rasko, David A; Webster, Dale R; Sahl, Jason W

    2011-01-01

    A large outbreak of diarrhea and the hemolytic-uremic syndrome caused by an unusual serotype of Shiga-toxin-producing Escherichia coli (O104:H4) began in Germany in May 2011. As of July 22, a large number of cases of diarrhea caused by Shiga-toxin-producing E. coli have been reported--3167 without...... the hemolytic-uremic syndrome (16 deaths) and 908 with the hemolytic-uremic syndrome (34 deaths)--indicating that this strain is notably more virulent than most of the Shiga-toxin-producing E. coli strains. Preliminary genetic characterization of the outbreak strain suggested that, unlike most of these strains......, it should be classified within the enteroaggregative pathotype of E. coli....

  2. Polymorphisms in genes of interleukin 12 and its receptors and their association with protection against severe malarial anaemia in children in western Kenya

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    Slutsker Laurence

    2010-03-01

    Full Text Available Abstract Background Malarial anaemia is characterized by destruction of malaria infected red blood cells and suppression of erythropoiesis. Interleukin 12 (IL12 significantly boosts erythropoietic responses in murine models of malarial anaemia and decreased IL12 levels are associated with severe malarial anaemia (SMA in children. Based on the biological relevance of IL12 in malaria anaemia, the relationship between genetic polymorphisms of IL12 and its receptors and SMA was examined. Methods Fifty-five tagging single nucleotide polymorphisms covering genes encoding two IL12 subunits, IL12A and IL12B, and its receptors, IL12RB1 and IL12RB2, were examined in a cohort of 913 children residing in Asembo Bay region of western Kenya. Results An increasing copy number of minor variant (C in IL12A (rs2243140 was significantly associated with a decreased risk of SMA (P = 0.006; risk ratio, 0.52 for carrying one copy of allele C and 0.28 for two copies. Individuals possessing two copies of a rare variant (C in IL12RB1 (rs429774 also appeared to be strongly protective against SMA (P = 0.00005; risk ratio, 0.18. In addition, children homozygous for another rare allele (T in IL12A (rs22431348 were associated with reduced risk of severe anaemia (SA (P = 0.004; risk ratio, 0.69 and of severe anaemia with any parasitaemia (SAP (P = 0.004; risk ratio, 0.66. In contrast, AG genotype for another variant in IL12RB1 (rs383483 was associated with susceptibility to high-density parasitaemia (HDP (P = 0.003; risk ratio, 1.21. Conclusions This study has shown strong associations between polymorphisms in the genes of IL12A and IL12RB1 and protection from SMA in Kenyan children, suggesting that human genetic variants of IL12 related genes may significantly contribute to the development of anaemia in malaria patients.

  3. 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)

  4. Whole-Genome Characterization and Strain Comparison of VT2f-Producing Escherichia coli Causing Hemolytic Uremic Syndrome

    Science.gov (United States)

    Michelacci, Valeria; Bondì, Roslen; Gigliucci, Federica; Franz, Eelco; Badouei, Mahdi Askari; Schlager, Sabine; Minelli, Fabio; Tozzoli, Rosangela; Caprioli, Alfredo; Morabito, Stefano

    2016-01-01

    Verotoxigenic Escherichia coli infections in humans cause disease ranging from uncomplicated intestinal illnesses to bloody diarrhea and systemic sequelae, such as hemolytic uremic syndrome (HUS). Previous research indicated that pigeons may be a reservoir for a population of verotoxigenic E. coli producing the VT2f variant. We used whole-genome sequencing to characterize a set of VT2f-producing E. coli strains from human patients with diarrhea or HUS and from healthy pigeons. We describe a phage conveying the vtx2f genes and provide evidence that the strains causing milder diarrheal disease may be transmitted to humans from pigeons. The strains causing HUS could derive from VT2f phage acquisition by E. coli strains with a virulence genes asset resembling that of typical HUS-associated verotoxigenic E. coli. PMID:27584691

  5. Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

    Directory of Open Access Journals (Sweden)

    Kim Ma

    2016-01-01

    Full Text Available Warm autoimmune hemolytic anemia (wAIHA is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA.

  6. Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

    Directory of Open Access Journals (Sweden)

    Fatih Demircioğlu

    2010-09-01

    Full Text Available A 33 weeks’ gestation, a baby with rhesus hemolytic disease (RHD, who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.

  7. IgG4-Related Disease Combined with Autoimmune Hemolytic Anemia and Steroid-Responsive Transient Hypercalcemia

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    Sho Hasegawa

    2015-01-01

    Full Text Available A 67-year-old man with elevated serum immunoglobulin G4 (IgG4 levels, systemic lymphadenopathy infiltrated by IgG4-positive plasma cells, and Coombs-positive autoimmune hemolytic anemia (AIHA showed marked hypercalcemia. Although the intact parathyroid hormone (PTH level was elevated, 99mTc-MIBI scintigraphy and thyroid ultrasonography revealed no evidence of primary hyperparathyroidism. Liver biopsy showed marked infiltration of IgG4-positive plasma cells, which confirmed the diagnosis of IgG4-related disease (IgG4-RD. Corticosteroid therapy was initiated, and subsequently, intact PTH and serum calcium levels gradually normalized. Transient hypercalcemia in a patient with AIHA may therefore be associated with IgG4-RD.

  8. Ceftriaxone-induced immune hemolytic anemia as a life-threatening complication of antibiotic treatment of 'chronic Lyme disease'.

    Science.gov (United States)

    De Wilde, Maarten; Speeckaert, Marijn; Callens, Rutger; Van Biesen, Wim

    2017-04-01

    'Chronic Lyme disease' is a controversial condition. As any hard evidence is lacking that unresolved systemic symptoms, following an appropriately diagnosed and treated Lyme disease, are related to a chronic infection with the tick-borne spirochaetes of the Borrelia genus, the term 'chronic Lyme disease' should be avoided and replaced by the term 'post-treatment Lyme disease syndrome.' The improper prescription of prolonged antibiotic treatments for these patients can have an impact on the community antimicrobial resistance and on the consumption of health care resources. Moreover, these treatments can be accompanied by severe complications. In this case report, we describe a life-threatening ceftriaxone-induced immune hemolytic anemia with an acute kidney injury (RIFLE-stadium F) due to a pigment-induced nephropathy in a 76-year-old woman, who was diagnosed with a so-called 'chronic Lyme disease.'

  9. Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency in patients ...

    African Journals Online (AJOL)

    This is a study of Glucose-6-phosphate dehydrogenase(G6PD) deficiency in sickle cell anaemia patients attending the haematology clinic of the Jos University Teaching Hospital (JUTH), Jos- Nigeria. The prevalence of G6PD deficiency among the 130 sickle cell anaemia patients studied was found to be 18.5%. G6PD ...

  10. Contribution of hly homologs to the hemolytic activity of Prevotella intermedia.

    Science.gov (United States)

    Suzuki, Naoko; Fukamachi, Haruka; Arimoto, Takafumi; Yamamoto, Matsuo; Igarashi, Takeshi

    2012-06-01

    Prevotella intermedia is a periodontal pathogen that requires iron for its growth. Although this organism has hemolytic activity, the precise nature of its hemolytic substances and their associated hemolytic actions are yet to be fully determined. In the present study, we identified and characterized several putative hly genes in P. intermedia ATCC25611 which appear to encode hemolysins. Six hly genes (hlyA, B, C, D, E, and hlyI) of P. intermedia were identified by comparing their nucleotide sequences to those of known hly genes of Bacteroides fragilis NCTC9343. The hlyA-E, and hlyI genes were overexpressed individually in the non-hemolytic Escherichia coli strain JW5181 and examined its contribution to the hemolytic activity on sheep blood agar plates. E. coli cells expressing the hlyA and hlyI genes exhibited hemolytic activity under anaerobic conditions. On the other hand, only E. coli cells stably expressing the hlyA gene were able to lyse the red blood cells when cultured under aerobic conditions. In addition, expression of the hlyA and hlyI genes was significantly upregulated in the presence of red blood cells. Furthermore, we found that the growth of P. intermedia was similar in an iron-limited medium supplemented with either red blood cells or heme. Taken together, our results indicate that the hlyA and hlyI genes of P. intermedia encode putative hemolysins that appear to be involved in the lysis of red blood cells, and suggest that these hemolysins might play important roles in the iron-dependent growth of this organism. Copyright © 2012 Elsevier Ltd. All rights reserved.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  12. A 3-marker index improves the identification of iron disorders in CKD anaemia.

    Directory of Open Access Journals (Sweden)

    Lucile Mercadal

    Full Text Available BACKGROUND: Iron disorders are common and complex in chronic kidney disease (CKD. We sought to determine whether a 3-marker index would improve the classification of iron disorders in CKD anaemia. METHODS: We studied the association between Hb level and iron indexes combining 2 or 3 of the following markers: serum ferritin (<40 ng/mL, transferrin saturation (TSAT<20% and total iron binding capacity (TIBC<50 µmol/L in 1011 outpatients with non-dialysis CKD participating in the Nephrotest study. All had glomerular filtration rates measured (mGFR by (51Cr-EDTA renal clearance; 199 also had hepcidin measures. RESULTS: The TSAT-TIBC-ferritin index explained Hb variation better than indexes combining TSAT-TIBC or ferritin-TSAT. It showed hypotransferrinaemia and non-inflammatory functional iron deficiency (ID to be more common than either absolute or inflammatory ID: 20%, 19%, 6%, and 2%, respectively. Hb was lower in all abnormal, compared with normal, iron profiles, and decreased more when mGFR was below 30 mL/min/1.73 m(2 (interaction p<0.0001. In patients with mGFR<30 mL/min/1.73 m(2, the Hb decreases associated with hypotransferrinaemia, non-inflammatory functional ID, and absolute ID were 0.83±0.16 g/dL, 0.51±0.18 and 0.89±0.29, respectively. Compared with normal iron profiles, hepcidin was severely depressed in absolute ID but higher in hypotransferrinaemia. CONCLUSIONS: The combined TSAT-TIBC-ferritin index identifies hypotransferrinaemia and non-inflammatory functional ID as the major mechanisms of iron disorders in CKD anaemia. Both disorders were associated with a greater decrease in Hb when mGFR was <30 mL/min/1.73 m(2. Taking these iron profiles into account may be useful in stratifying patients in clinical trials of CKD anaemia and might improve the management of iron therapy.

  13. Anaemia in pregnancy: a cross-sectional study of pregnant women in a Sahelian tertiary hospital in Northeastern Nigeria.

    Science.gov (United States)

    Kagu, M B; Kawuwa, M B; Gadzama, G B

    2007-10-01

    This prospective study was carried out between June 2005 and June 2006, to determine the prevalence and determinants of anaemia among pregnant women attending a tertiary Sahelian Hospital in Northeastern Nigeria. A total of 1,040 pregnant women enrolled at their first antenatal visit were monitored through pregnancy for anaemia. The overall prevalence of anaemia, malaria parasitaemia and schistosomiasis was 72.0%, 22.1% and 3.8%, respectively. Mild, moderate and severe anaemia constituted 31.8%, 39.4% and 0.9%, respectively. Anaemia was most common among the multipara and women presenting in late stages of pregnancy. More multipara and primigravidae had malaria parasitaemia than grandmultipara. Schistosomiasis, malaria infestation and a short birth interval as well as illiteracy are additional risk factors for anaemia in pregnancy. This study confirms the high prevalence of anaemia in pregnancy in this area. Appropriate intervention strategies are necessary to reduce the prevalence of anaemia.

  14. A study on status of anaemia in pregnant women attending urban health training centre, RIMS, Ranchi

    Directory of Open Access Journals (Sweden)

    Vijay Kumar

    2014-12-01

    Full Text Available Background: Anaemia in pregnant women has been regarded as very dangerous as it causes many maternal, fetal and neonatal complications. Fetal growth and pregnancy outcome largely depend upon the status of anaemia in pregnant women. Anaemia affects pregnant  women all over the world - 52% in  developing  countries  compared  with  23%  in  the  developed  world. The difference in prevalence of anaemia in different parts of India including Jharkhand can be attributed to the different factors. A knowledge of these factors associated with anemia will help to formulate multipronged strategies to curtail this important public health problem in pregnancy. Aims & Objectives: (1 To know the socio-demographic profile of pregnant women attending Urban Health and Training Centre (UHTC, RIMS, Ranchi. (2 To know the status of anaemia among those pregnant women and its association with different factors. Material & Methods: A descriptive cross-sectional study done at ANC clinic of UHTC, RIMS, Ranchi to determine the status of anaemia in pregnant women and various socio-demographic factors associated with it. Hemoglobin level of 149 pregnant women selected by consecutive sampling was estimated by Cyanmethemoglobin method. Statistical Analysis: Template generated in MS excel sheet and analysis was done on SPSS software. Result: Out of total 149 pregnant women anaemia was found to be present in 99 (66.4% women. A statistically significant association of anaemia (p.05.  Conclusion: Occurrence of anaemia was much higher in this area as compared to national average. It indicates that the anaemia continues to be a major public health problem.  Efforts should be geared towards the early detection and treatment of anaemia before delivery. 

  15. Cold agglutinin disease (CADwith autoimmune haemolytic anaemia: a case report of a coronary artery disease patient Doença por aglutininas a frio (DAC com anemia hemolítica auto-imune: relato de caso de um coronariopata

    Directory of Open Access Journals (Sweden)

    Leandro A. Barbosa

    2008-02-01

    Full Text Available Cold agglutinin disease (CAD with autoimmune haemolytic anemia is characterized by the production of harmful cold autoantibodies associated with increased red cell destruction during exposure to cold. The treatment of CAD is very difficult and a great effort is required to obtain therapeutic success. Cyclophosphamide is a potent immunosuppressive agent which is widely used in all bone marrow transplantation conditioning regimens for patients with acquired severe aplastic anemia. In this report, we describe the case of a coronary artery disease patient with severe CAD, but without lymphoproliferative disease, in which general measures and immunosuppressive therapies were adopted, there by avoiding blood transfusions.A doença por aglutininas a frio (CAD cursando com anemia hemolítica auto-imune (AHAI é decorrente da produção de autoanticorpos que reagem muito bem a baixas temperaturas, dirigidos contra hemácias autólogas. A habilidade desses anticorpos em destruir as hemácias encontra-se diretamente relacionada à sua capacidade em fixar complemento durante a exposição do paciente a baixas temperaturas. A AHAI por anticorpos frios pode ser idiopática - ausência de doença de base - ou secundária, geralmente associada a desordens linfoproliferativas de células B ou determinados processos infecciosos. A hemólise é intravascular, através de aglutininas da classe IgM, com teste direto da antiglobulina humana positivo para complemento. O tratamento da CAD é difícil, exigindo um esforço contínuo, necessário para se obter sucesso terapêutico. A ciclofosfamida é um agente imunossupressor potente, amplamente utilizado em transplantes de medula óssea, particularmente nos portadores de anemia aplástica. Descrevemos o caso de um coronariopata portador de CAD severa, cuja exploração diagnóstica excluiu doença linfoproliferativa. Adotamos medidas gerais de suporte e terapia imunossupressora, coibindo o uso de hemotransfusões.

  16. Alpha-Methyldopa-Induced Autoimmune Hemolytic Anemia in the Third Trimester of Pregnancy

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    Charalampos Grigoriadis

    2013-01-01

    Full Text Available Alpha-methyldopa has been demonstrated to be safe for use during pregnancy and is now used to treat gestational hypertension. In pregnancy, alpha-methyldopa-induced autoimmune hemolytic anemia does not have typical features and the severity of symptoms ranges from mild fatigue to dyspnea, respiratory failure, and death if left untreated. A case of alpha-methyldopa-induced autoimmune hemolytic anemia in a 36-year-old gravida 2, para 1 woman at 37+6 weeks of gestation is reported herein along with the differential diagnostic procedure and the potential risks to the mother and the fetus.

  17. Immunoglobulin transfusion in hemolytic disease of the newborn: place in therapy

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    Mundy CA

    2015-06-01

    Full Text Available Cynthia A Mundy, Jatinder Bhatia Department of Pediatrics, Division of Neonatology, Georgia Regents University, Children's Hospital of Georgia, GA, USA Abstract: Hemolytic disease of the newborn continues to be a common neonatal disorder that requires a comprehensive understanding on the part of those caring for infants. Common treatments include hydration and phototherapy. Exchange transfusion is used in severe hemolytic disease, but infants undergoing this treatment are exposed to many adverse effects. Intravenous immunoglobulin is a newer strategy that is showing promise in the treatment of the disease. This review discusses the current use and future expectations of intravenous immunoglobulin therapy in newborns. Keywords: hyperbilirubinemia, ABO incompatibility, neonatal jaundice 

  18. Hemolytic and urease activities in vibrios isolated from fresh and frozen oysters

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    Renata Albuquerque Costa

    2013-01-01

    Full Text Available INTRODUCTION: The present study aimed to survey the Vibrio microbiota of oysters (Crassostrea rhizophorae obtained from restaurants in Fortaleza, State of Ceará, Brazil, and to identify virulence factors. METHODS: The isolated vibrios were submitted to biochemical identification and were tested for hemolytic and urease activities. RESULTS: The isolated strains belonged to 13 species, with predominance of Vibrio mimicus. Of the strain isolates only from fresh samples, 20.5% and 2.8% showed hemolytic and urease activities, respectively. CONCLUSIONS: The findings support the little-publicized claim that Vibrio species other than V. parahaemolyticus and V. vulnificus can represent a health risk to public health.

  19. Hemolytic disease of the fetus and newborn caused by anti-E

    OpenAIRE

    Usman, Adiyyatu Sa?idu; Mustaffa, Rapiaah; Ramli, Noraida; Diggi, Sirajo A.

    2013-01-01

    Objective: Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother′s red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs. Case summary: We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting wit...

  20. Hemolytic Disease of the Newborn Due to Anti-c Isoimmunization: A Case Report.

    Science.gov (United States)

    Sheeladevi, C S; Suchitha, S; Manjunath, G V; Murthy, Srinivas

    2013-09-01

    The Rhesus (Rh) blood group is one of the most complex blood groups known in humans. It has remained of primary importance in obstetrics, being the main cause of hemolytic disease of the newborn (HDN). Anti-D causes the most severe form of HDN. Other Rh allo antibodies that are capable of causing severe HDN include anti-c, which clinically is the most important Rh antigen after the D antigen. We report a case of hemolytic disease of the newborn due to Rh anti-c in an infant of an Rh positive mother.