Sample records for hemolytic anemia due

  1. Hemolytic anemia

    MedlinePLUS

    Anemia - hemolytic ... Hemolytic anemia occurs when the bone marrow is unable to replace the red blood cells that are being destroyed. Immune hemolytic anemia occurs when the immune system mistakenly sees your ...

  2. Hemolytic Anemia

    MedlinePLUS

    ... from the NHLBI on Twitter. What Is Hemolytic Anemia? Hemolytic anemia (HEE-moh-lit-ick uh-NEE-me-uh) ... blood cells to replace them. However, in hemolytic anemia, the bone marrow can't make red blood ...

  3. Types of Hemolytic Anemia

    MedlinePLUS

    ... from the NHLBI on Twitter. Types of Hemolytic Anemia There are many types of hemolytic anemia. The ... the condition, but you develop it. Inherited Hemolytic Anemias With inherited hemolytic anemias, one or more of ...

  4. HEMOLYTIC ANEMIA Erythrocytes premature

    E-print Network

    9/16/2013 1 HEMOLYTIC ANEMIA Erythrocytes premature destruction SCHISTOCYTES & SPHEROCYTES · Gallstones · Dark or Red Urine · Symptoms of Anemia · Thinning of Cortical Bone · Extramedullary RBC Defects · Immunohemolytic Anemias #12;9/16/2013 3 INTRINSIC DEFECTS · Membrane Defects

  5. Anemia Due to Excessive Bleeding

    MedlinePLUS

    ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ...

  6. Hemolytic anemia caused by chemicals and toxins

    MedlinePLUS

    Anemia - hemolytic - caused by chemicals or toxins ... Possible substances that can cause hemolytic anemia include: Anti-malaria drugs (quinine compounds) Arsenic Dapsone Intravenous water infusion (not half-normal saline or normal saline) Metals (chromium/chromates, ...

  7. Drug-induced immune hemolytic anemia

    MedlinePLUS

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... In some cases, a drug can cause the immune system to mistake your own red blood cells for foreign substances. The body responds by making ...

  8. Congenital Non-Spherocytic Hemolytic Anemia

    PubMed Central

    Zipursky, A.; Rowland, Marlene; Peters, J. C.; Israels, L. G.

    1965-01-01

    A family with congenital non-spherocytic hemolytic anemia associated with glucose-6-phosphate dehydrogenase (G6PD) deficiency was studied. Two females, heterozygous for the enzyme deficency, had evidence of a hemolytic anemia. The results of chromium-51 erythrocyte life span studies prior to, during, and after periods of primaquine administration suggested that the hemolytic anemia in these women was due to the presence of two populations of red blood cells in their circulation. One population had normal G6PD levels and a normal life span, whereas the other had diminished enzyme activity and a shortened life span. In vitro metabolic studies of the erythrocytes of a heterozygous female and a hemizygous male suggested that, in spite of G6PD deficiency, the synthesis and breakdown of adenosine triphosphate and 2,3-diphosphoglyceric acid was similar to that in normal erythrocytes. PMID:5320918

  9. Neonatal hemolytic anemia due to inherited harderoporphyria: clinical characteristics and molecular basis.

    PubMed

    Lamoril, J; Puy, H; Gouya, L; Rosipal, R; Da Silva, V; Grandchamp, B; Foint, T; Bader-Meunier, B; Dommergues, J P; Deybach, J C; Nordmann, Y

    1998-02-15

    Porphyrias, a group of inborn errors of heme synthesis, are classified as hepatic or erythropoietic according to clinical data and the main site of expression of the specific enzymatic defect. Hereditary coproporphyria (HC) is an acute hepatic porphyria with autosomal dominant inheritance caused by deficient activity of coproporphyrinogen III oxidase (COX). Typical clinical manifestations of the disease are acute attacks of neurological dysfunction; skin photosensitivity may also be present. We report a variant form of HC characterized by a unifying syndrome in which hematologic disorders predominate: harderoporphyria. Harderoporphyric patients exhibit jaundice, severe chronic hemolytic anemia of early onset associated with hepatosplenomegaly, and skin photosensitivity. Neither abdominal pain nor neuropsychiatric symptoms are observed. COX activity is markedly decreased. In a first harderoporphyric family, with three affected siblings, a homozygous K404E mutation has been previously characterized. In the present study, molecular investigations in a second family with neonatal hemolytic anemia and harderoporphyria revealed two heterozygous point mutations in the COX gene. One allele bore the missense mutation K404E previously described. The second allele bore an A-->G transition at the third position of the donor splice site in intron 6. This new COX gene mutation resulted in exon 6 skipping and the absence of functional protein production. In contrast with other COX gene defects that produce the classical hepatic porphyria presentation, our data suggest that the K404E substitution (either in the homozygous or compound heterozygous state associated with a mutation leading to the absence of functional mRNA or protein) is responsible for the specific hematologic clinical manifestations of harderoporphyria. PMID:9454777

  10. Anti-M Antibody Induced Prolonged Anemia Following Hemolytic Disease of the Newborn Due to Erythropoietic Suppression in 2 Siblings.

    PubMed

    Ishida, Atsushi; Ohto, Hitoshi; Yasuda, Hiroyasu; Negishi, Yutaka; Tsuiki, Hideki; Arakawa, Takeshi; Yagi, Yoshihito; Uchimura, Daisuke; Miyazaki, Toru; Ohashi, Wataru; Takamoto, Shigeru

    2015-08-01

    Hemolytic disease of the newborn (HDN) arising from MNSs incompatibility is rare, with few reports of prolonged anemia and reticulocytopenia following HDN. We report the younger of 2 male siblings, both of whom had anti-M-induced HDN and anemia persisting for over a month. Peripheral reticulocytes remained inappropriately low for the degree of anemia, and they needed multiple red cell transfusions. Viral infections were ruled out. Corticosteroids were given for suspected pure red cell aplasia. Anemia and reticulocytopenia subsequently improved. Colony-forming unit erythroid assay revealed erythropoietic suppression of M antigen-positive erythroid precursor cells cultured with maternal or infant sera containing anti-M. In conclusion, maternal anti-M caused HDN and prolonged anemia by erythropoietic suppression in 2 siblings. PMID:25929611

  11. Treatment of autoimmune hemolytic anemias

    PubMed Central

    Zanella, Alberto; Barcellini, Wilma

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. The first-line therapy for warm AIHA are corticosteroids, which are effective in 70–85% of patients and should be slowly tapered over a time period of 6–12 months. For refractory/relapsed cases, the current sequence of second-line therapy is splenectomy (effective approx. in 2 out of 3 cases but with a presumed cure rate of up to 20%), rituximab (effective in approx. 80–90% of cases), and thereafter any of the immunosuppressive drugs (azathioprine, cyclophosphamide, cyclosporin, mycophenolate mofetil). Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases. In CAD, rituximab is now recommended as first-line treatment. PMID:25271314

  12. Published Reports of Delayed Hemolytic Anemia After Treatment...

    Science.gov Websites

    artesunate be followed for 4 weeks after treatment and evaluated for hemolytic anemia. A literature search was performed using the terms "artesunate" and either "hemolytic anemia"...

  13. How Is Hemolytic Anemia Treated?

    MedlinePLUS

    ... medicines rituximab and cyclosporine. If you have severe sickle cell anemia , your doctor may recommend a medicine called hydroxyurea. ... hemoglobin that newborns have. In people who have sickle cell anemia, fetal hemoglobin helps prevent red blood cells from ...

  14. A case study of a child with chronic hemolytic anemia due to a Donath-Landsteiner positive, IgM anti-I autoantibody.

    PubMed

    Karafin, Matthew S; Shirey, R Sue; Ness, Paul M; King, Karen E; Keefer, Jeffrey

    2012-11-01

    In children, paroxysmal cold hemoglobinuria (PCH) is generally considered an acute self-limited autoimmune hemolytic anemia caused by an IgG biphasic auto-anti-P antibody identified by the Donath-Landsteiner (D-L) test. We report a case of a 5-year-old female with a chronic hemolytic anemia. The etiology of the hemolysis appears to be an unusual D-L positive, IgM antibody with specificity for the I antigen. The clinical course is described and a discussion of PCH and the D-L antibody is presented. We also discuss intravenous immunoglobulin infusions as a therapy for children with this form of severe chronic autoimmune hemolytic anemia. PMID:22553072

  15. Chronic nonspherocytic hemolytic anemia due to glucose-6-phosphate dehydrogenase deficiency: report of two families with novel mutations causing G6PD Bangkok and G6PD Bangkok Noi

    Microsoft Academic Search

    Voravarn S. Tanphaichitr; Akira Hirono; Parichat Pung-amritt; Ajjima Treesucon; Wanchai Wanachiwanawin

    2011-01-01

    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is one of the most common hereditary enzymopathies worldwide. Mostly G6PD\\u000a deficient cases are asymptomatic though they may have the risk of neonatal jaundice (NNJ) and acute intravascular hemolysis\\u000a during oxidative stress. Chronic nonspherocytic hemolytic anemia (CNSHA) due to G6PD deficiency is rare. In Thailand, one\\u000a case was reported 40 years ago and by biochemical study this

  16. Autoimmune hemolytic anemia in chronic mucocutaneous candidiasis.

    PubMed Central

    Oyefara, B I; Kim, H C; Danziger, R N; Carroll, M; Greene, J M; Douglas, S D

    1994-01-01

    Chronic mucocutaneous candidiasis is an immunodeficiency disease characterized by T-cell dysregulation and chronic superficial candidal infections. We report on three patients with chronic mucocutaneous candidiasis who developed autoantibodies to erythrocytes. Our first patient, a 19-year-old female, developed autoimmune hemolytic anemia (AIHA) that required multiple courses of treatment, including corticosteroids, intravenous immunoglobulin, and danazol. During the last exacerbation of AIHA, intensive treatment with corticosteroids and intravenous immunoglobulin failed and yet the patient responded to plasmapheresis. Our second patient, a 21-year-old male, developed AIHA which responded to oral corticosteroid therapy. Our third patient, a 6-year-old female without evidence of hemolysis, was found to have erythrocyte autoantibodies on routine screening. These three patients had positive direct antiglobulin tests, and the first patient had both immunoglobulin G (IgG) and IgM erythrocyte autoantibodies, while the remaining two patients had only IgG autoantibody. This is the first report of the association of AIHA with chronic mucocutaneous candidiasis. We suggest that all patients with chronic mucocutaneous candidiasis be screened periodically for erythrocyte autoantibodies. Plasmapheresis, a safe ancillary procedure in the management of AIHA, may be life-saving in some cases. The occurrence of erythrocyte autoantibodies in mucocutaneous candidiasis may be related to immunoregulatory disorders in this disease. PMID:7496919

  17. Autoimmune hemolytic anemia: From lab to bedside.

    PubMed

    Chaudhary, R K; Das, Sudipta Sekhar

    2014-01-01

    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. PMID:24678166

  18. Impairment of Bone Health in Pediatric Patients with Hemolytic Anemia

    PubMed Central

    Schündeln, Michael M.; Goretzki, Sarah C.; Hauffa, Pia K.; Wieland, Regina; Bauer, Jens; Baeder, Lena; Eggert, Angelika; Hauffa, Berthold P.; Grasemann, Corinna

    2014-01-01

    Introduction Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may also display impaired bone health. Study Design To assess bone health in pediatric patients with chronic hemolytic anemia, a cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i.e., 17 homozygous sickle cell disease and 14 hereditary spherocytosis patients). Biochemical, radiographic and anamnestic parameters of bone health were assessed. Results Vitamin D deficiency with 25 OH-vitamin D serum levels below 20 ng/ml was a common finding (80.5%) in this cohort. Bone pain was present in 31% of patients. Analysis of RANKL, osteoprotegerin (OPG) and osteocalcin levels indicated an alteration in bone modeling with significantly elevated RANKL/OPG ratios (control: 0.08+0.07; patients: 0.26+0.2, P?=?0.0007). Osteocalcin levels were found to be lower in patients compared with healthy controls (68.5+39.0 ng/ml vs. 118.0+36.6 ng/ml, P?=?0.0001). Multiple stepwise regression analysis revealed a significant (P<0.025) influence of LDH (partial r2?=?0.29), diagnosis of hemolytic anemia (partial r2?=?0.05) and age (partial r2?=?0.03) on osteocalcin levels. Patients with homozygous sickle cell anemia were more frequently and more severely affected by impaired bone health than patients with hereditary spherocytosis. Conclusion Bone health is impaired in pediatric patients with hemolytic anemia. In addition to endocrine alterations, an imbalance in the RANKL/OPG system and low levels of osteocalcin may contribute to this impairment. PMID:25299063

  19. Studies on the Specificity of Autoantibodies in Acquired Hemolytic Anemia

    Microsoft Academic Search

    BERTHA A. BounoNcLE

    IN THE PAST, the autoantibodies found iii autoimmune hemolytic anemia were considered capable imot only of sensitizing the imidividual's own erythro- cytes, but also of reacting nonspecifically with all human red cells. Autoanti- 1)odies were presumed to 1)e directed against some common antigen possessed by all human erythrocytes, umirelated to the known blood group antigens. However, recent evidence indicates that

  20. Role of oxidant stress in lawsone-induced hemolytic anemia.

    PubMed

    McMillan, David C; Sarvate, Snehal D; Oatis, John E; Jollow, David J

    2004-12-01

    Lawsone (2-hydroxy-1,4-naphthoquinone) is the active ingredient of henna (Lawsonia alba), the crushed leaves of which are used as a cosmetic dye. Application of henna can induce a severe hemolytic anemia, and lawsone is thought to be the causative agent. Administration of lawsone to rats has been shown to induce a hemolytic response that is associated with oxidative damage to erythrocytes. However, direct exposure of isolated erythrocytes to lawsone did not provoke oxidative damage, suggesting that lawsone must undergo extra-erythrocytic bioactivation in vivo. In the present study, the survival of rat 51Cr-labeled erythrocytes in vivo after in vitro exposure to lawsone and its hydroquinone form, 1,2,4-trihydroxynaphthalene (THN) has been examined. Neither lawsone nor THN were directly hemolytic or methemoglobinemic, even at high concentrations (>3 mM). Lawsone had no effect on erythrocytic GSH levels, whereas THN (3 mM) induced a modest depletion (approximately 30%). Cyclic voltammetry revealed that the lack of hemotoxicity of lawsone was associated with a poor capacity to undergo redox cycling. In contrast, ortho-substituted 1,4-naphthoquinones without a 2-hydroxy group, such as 2-methyl- and 2-methoxy-1,4-naphthoquinone, were redox active, were able to deplete GSH, and were direct-acting hemolytic agents. An oxidant stress-associated hemolytic response to lawsone could be provoked, however, if it was incubated with GSH-depleted erythrocytes. The data suggest that lawsone is a weak direct-acting hemolytic agent that does not require extra-erythrocytic metabolism to cause hemotoxicity. Thus, the hemolytic response to henna may be restricted to individuals with compromised antioxidant defenses. PMID:15456924

  1. Severe microangiopathic hemolytic anemia and thrombocytopenia in a child with Brucella infection

    Microsoft Academic Search

    A. Yaramis; M. Kervancioglu; I. Yildirim; M. Soker; O. Derman; M. A. Tas

    2001-01-01

    We present a case of severe microangiopathic hemolytic anemia and thrombocytopenia with epistaxis, gross hematuria, hemoglobinuria, and skin purpura in a child with Brucella septicemia proven by culture. The patient showed the features of this illness: leukopenia, severe hemolytic anemia, thrombocytopenia, fragmentation of erythrocytes in the peripheral blood smear, increased erythropoiesis, megakaryopoiesis, and granulomata cell invasion in the bone marrow.

  2. CNS intravascular large cell lymphoma in a patient with autoimmune hemolytic anemia

    E-print Network

    2015-01-01

    O, Turner J, et al. Autoimmune disorders and risk of non-autoimmune hemolytic anemia, leukoencephalopathy INTRODUCTION Intravascular large cell lymphoma (IVLCL) is a rare and aggressive lymphoproliferative disorder

  3. Hemolytic Anemia as a Presenting Feature of Wilson's Disease: A Case Report.

    PubMed

    Sharma, Sunita; Toppo, Anupa; Rath, B; Harbhajanka, Aparna; Lalita Jyotsna, P

    2010-09-01

    Wilson's disease is a rare inherited disorder of copper metabolism causing severe damage to vital organs. Liver and brain disorders are the main manifestations. Severe hemolytic anemia is an unusual complication of Wilson's disease. We present a case who developed spherocytic acute hemolytic anemia (Coomb's negative) as the initial manifestation of Wilson's disease. On examination Kayser- Fleischer ring was found. Laboratory data supported a diagnosis of Wilson's disease. PMID:21886393

  4. Hemolytic Anemia as a Presenting Feature of Wilson’s Disease: A Case Report

    PubMed Central

    Toppo, Anupa; Rath, B.; Harbhajanka, Aparna; Lalita Jyotsna, P.

    2010-01-01

    Wilson’s disease is a rare inherited disorder of copper metabolism causing severe damage to vital organs. Liver and brain disorders are the main manifestations. Severe hemolytic anemia is an unusual complication of Wilson’s disease. We present a case who developed spherocytic acute hemolytic anemia (Coomb’s negative) as the initial manifestation of Wilson’s disease. On examination Kayser- Fleischer ring was found. Laboratory data supported a diagnosis of Wilson’s disease. PMID:21886393

  5. BAFF level increased in patients with autoimmune hemolytic anemia

    PubMed Central

    Zhao, Yu-Bing; Li, Jun-Min; Wei, Bei-Wen; Xu, Zi-Zhen

    2015-01-01

    Introduction: BAFF (B-cell activating factor of the TNF family), an important regulator of B-cell, has been observed to be over-expressed in a variety of autoimmune diseases. Autoimmune hemolytic anemia (AIHA) is an acquired autoimmune disease occurred when antibodies directed against autologous red blood cells. We assessed serum levels of BAFF in AIHA patients with different serological characteristics. Methods: Serum BAFF levels were measured in 44 AIHA patients with different direct antiglobulin test (DAT) results and 25 healthy controls. The correlation of BAFF expression with DAT results and serological characteristics was assessed. Results: Serum levels of BAFF in AIHA patients were significantly higher than in healthy subjects (AIHA: 1382.7 ± 1412.8 pg/ml, healthy control: 725.0 ± 415.7 pg/ml, P = 0.0057). Serum BAFF levels were significantly higher in patients with IgG(+)C3(+) or IgG(+) than healthy controls (DAT: negative) (P = 0.012, 0.004, respectively). No significant correlations were presented between serum BAFF levels and four serological parameters: hemoglobine, percentage of reticulocyte, total serum bilirubin, and lactate dehydrogenase. Conclusions: AIHA patients present higher serum BAFF levels than healthy controls, especially for those of IgG(+)C3(+) DAT result. This might lead to a new approach of AIHA treatment.

  6. A fatal case of ceftriaxone (Rocephin)-induced hemolytic anemia associated with intravascular immune hemolysis.

    PubMed

    Garratty, G; Postoway, N; Schwellenbach, J; McMahill, P C

    1991-02-01

    Fatal hemolytic anemia developed in a 52-year-old woman who was treated with a cephalosporin, ceftriaxone. The patient's red cells (RBCs) were coated with C3, but no RBC-bound IgG, IgA, or IgM was detected. Her serum contained an antibody that did not react with cephalosporin-coated RBCs but reacted strongly with RBCs in vitro when her serum was added to drug and RBCs. This is the first case of immune hemolytic anemia associated with ceftriaxone, the first case of fatal cephalosporin-induced hemolytic anemia, and the second case in which a cephalosporin antibody showed in vitro and in vivo characteristics usually thought to be associated with the so-called immune complex mechanism. PMID:1825363

  7. Hemolytic anemia as first presentation of Wilson's disease with uncommon ATP7B mutation.

    PubMed

    Ye, Xing-Nong; Mao, Li-Ping; Lou, Yin-Jun; Tong, Hong-Yan

    2015-01-01

    Wilson's disease (WD) is a rare inherited disorder of copper metabolism and the main manifestations are liver and brain disorders. Hemolytic anemia is an unusual complication of WD. We describe a 15-year-old girl who developed hemolytic anemia as the first manifestation of Wilson's disease. An Arg952Lys mutation was found in exon 12 of the ATP7B gene, which is uncommon among Chinese Han individuals. From this case and reviews, we can achieve a better understanding of WD. Besides, we may conclude that the probable diagnosis of WD should be considered in young patients with unexplained hemolytic anemia, especially in patients with hepatic and/or neurologic disorder. PMID:26064408

  8. Hemolytic anemia as first presentation of Wilson’s disease with uncommon ATP7B mutation

    PubMed Central

    Ye, Xing-Nong; Mao, Li-Ping; Lou, Yin-Jun; Tong, Hong-Yan

    2015-01-01

    Wilson’s disease (WD) is a rare inherited disorder of copper metabolism and the main manifestations are liver and brain disorders. Hemolytic anemia is an unusual complication of WD. We describe a 15-year-old girl who developed hemolytic anemia as the first manifestation of Wilson’s disease. An Arg952Lys mutation was found in exon 12 of the ATP7B gene, which is uncommon among Chinese Han individuals. From this case and reviews, we can achieve a better understanding of WD. Besides, we may conclude that the probable diagnosis of WD should be considered in young patients with unexplained hemolytic anemia, especially in patients with hepatic and/or neurologic disorder.

  9. Acute hemolytic anemia in glucose-6-phosphate dehydrogenase deficiency complicated by Ginkgo biloba.

    PubMed

    Lai, Shiue-Wei; Chen, Jia-Hong; Kao, Woei-Yau

    2013-01-01

    We report on a patient with glucose-6-phosphate dehydrogenase (G6PD) deficiency who developed acute hemolytic anemia after having received an injection of Ginkgo biloba for dementia prophylaxis without medical advice. She suddenly developed general malaise, generalized yellowish skin color, and tea-colored urine. Intravenous fluid infusion and cessation of G. biloba quickly relieved her clinical symptoms. To the best of our knowledge, this is the first case report of G. biloba-induced acute hemolytic anemia in vivo. PMID:23970095

  10. Bacillus cereus bacteremia and hemolytic anemia in a patient with hemoglobin SC disease.

    PubMed

    Rodgers, G M; Barrera, E; Martin, R R

    1980-08-01

    A patient with hemoglobin SC disease and cholelithiasis was found to have Bacillus cereus bacteremia. Hemolytic anemia developed, for which common causes of hemolysis were excluded, suggesting a relationship with the bacteremia. Following in vitro incubation, type O erythrocytes were hemolyzed by the culture, but not by a bacteria-free filtrate. This case confirms the association between sickle cell disorders and cholelithiasis with B cereus infections. In addition, it provides evidence for in vivo hemolysis with B cereus bacteremia, an organism not previously associated with hemolytic anemia. PMID:6772119

  11. The Immunologic Specificity of Antiserum for Trypsin Treated Red Blood Cells and Its Reactions with Normal and Hemolytic Anemia Cells

    Microsoft Academic Search

    J. ALBERT BAXTER; A. BOURONCLE; ALVINZA E. BUNNER; HENRY J. WINN

    with enzymes or viruses, or by storage have showis properties in common with the abnormal, short-lived erythrocytes from patients with certain hemolyt-ic anemias. These are increased fragility, increased susceptil)ility to phagocytosis,' and agglutination in normal serum. The characteristic agglutination of suital)ly trypsinized red cells by the incomplete antibody found in the sera of hemolytic anemias has proved of definite clinical

  12. Hepatocellular carcinoma with chronic B-type hepatitis complicated by autoimmune hemolytic anemia: A case report

    PubMed Central

    Okada, Toshie; Kubota, Keiichi; Kita, Junji; Kato, Masato; Sawada, Tokihiko

    2007-01-01

    A 57-year-old man consulted a local hospital because of a persistent slight fever. At the age of 37 years he was diagnosed having B-type hepatitis, but left the liver dysfunction untreated. Twenty years later, he was diagnosed having chronic hepatitis B, hepatocellular carcinoma (HCC) and macrocytic anemia, and referred to our hospital for further investigation. A HCC with a maximum diameter of 5.2 cm was detected in segment 8. Results of blood tests included 1.8 mg/dL serum total bilirubin, 0.9 mg/dL bilirubin, less than 10 mg/dL haptoglobin, 7.9 g/dL hemoglobin, 130 fL MCV, and 14.5% reticulocytes. A bone marrow sample showed erythroid hyperplasia. The direct Coombs test gave a positive result. We diagnosed the anemia as autoimmmune hemolytic anemia (AIHA), for which prednisolone could not be administered due to positivity for HBsAg and HBeAg. After preparation of washed blood cells for later transfusion, the patient underwent systematic resection of segment 8. The cut surface of the resected specimen demonstrated an encapsulated yellow-brownish tumor measuring 52 mm × 40 mm which was diagnosed pathologicaly as moderately differentiated HCC. On the 9th postoperative day, the patient’s temperature rose to 38°C, and exacerbated hemolysis was observed. The maximum total bilirubin value was 5.8 mg/dL and minimum hemoglobin level was 4.6 g/dL. He tolerated this period without blood transfusion. Currently he is being followed up as an outpatient, and shows no signs of HCC recurrence or symptoms of anemia. AIHA associated with HBV infection has been described in only three previous cases, and the present case is the first in which surgery was performed for accompanying HCC. PMID:17708620

  13. Phosphatidylserine Exposure and Red Cell Viability in Red Cell Aging and in Hemolytic Anemia

    Microsoft Academic Search

    Franz Edward Boas; Linda Forman; Ernest Beutler

    1998-01-01

    Phosphatidylserine (PS) normally localizes to the inner leaflet of cell membranes but becomes exposed in abnormal or apoptotic cells, signaling macrophages to ingest them. Along similar lines, it seemed possible that the removal of red cells from circulation because of normal aging or in hemolytic anemias might be triggered by PS exposure. To investigate the role of PS exposure in

  14. Unexplained hemolytic anemia of pregnancy: Case report with review of related literature

    Microsoft Academic Search

    Paban Sharma; Rekha Sthapit

    2008-01-01

    We present a case of a multipara lady who developed hemolytic anemia during pregnancy with spontaneous remission after delivery. She was managed with low dose steroid for hemolysis during pregnancy with good feto-maternal outcome. Despite extensive investigations carried out to determine the cause, they remained in vain. Very few of such cases have been described in the literature, which merits

  15. [Pancytopenia, Hemolytic Anemia and Schizocytes: a Pragmatic Approach].

    PubMed

    Gökok, Nil; Stalder, Grégoire; Alberio, Lorenzo; Lamy, Olivier; Schwotzer, Nora

    2015-07-01

    A 58 year old woman presents with a progressive fatigue and dyspnea associated with paresthesia. Laboratory tests show pancytopenia with hypersegmented neutrophiles, macrocytic hyporegenerative anemia and arguments for hemolysis, in particular highly increased LDH. This constellation strongly suggests vitamin B12 deficiency, which was confirmed with an undetectable cobalamine concentration in the blood of our patient. The etiologic work up shows the presence of anti-parietal cells antibodies at a titer of 1/640, diagnostic of Biermer anemia. PMID:26135726

  16. The kinetics of hematopoiesis in the light horse III. The hematological response to hemolytic anemia.

    PubMed Central

    Lumsden, H J; Valli, V E; McSherry, B J; Robinson, G A; Claxton, M J

    1975-01-01

    The hematological response to acetylphenylhydrazine hemolytic anemia was studied in three standardbred horses. The lifespan of erythrocytes produced during the most severe phase of the anemia were measured with 75-selenomethionine and found to be 144 days as compared to the 139 day lifespan in response to hemorrhagic anemia or 155 days in normal standardbred horses measured previously using the same technique. The erythrocyte counts returned to initial values in 42 days (37, 34 and 54 days) a mean erythrocyte production of 6.4 times 10-12 erythrocytes/day. The mean hemoglobin production was 0.31 gm/kg body weight/day as compared to 0.11 gm Hb/kg/day previously observed in response to hemorrhagic anemia. The mean increase in erythrocyte mean cell volume was 12 mu-3 during the acute response phase to hemolytic anemia in contrast to the absence of a significant increase in the mean cell volume as previously observed during response to hemorrhagic anemia. Free Heinz bodies separated from erythrocytes during the acute phase could not be differentiated from platelets on the hemocytometer counting chamber with standard techniques. PMID:1139414

  17. Peroxiredoxin II is essential for preventing hemolytic anemia from oxidative stress through maintaining hemoglobin stability.

    PubMed

    Han, Ying-Hao; Kim, Sun-Uk; Kwon, Tae-Ho; Lee, Dong-Seok; Ha, Hye-Lin; Park, Doo-Sang; Woo, Eui-Jeon; Lee, Sang-Hee; Kim, Jin-Man; Chae, Ho-Byoung; Lee, Sang Yeol; Kim, Bo Yeon; Yoon, Do Young; Rhee, Sue Goo; Fibach, Eitan; Yu, Dae-Yeul

    2012-09-28

    The pathophysiology of oxidative hemolytic anemia is closely associated with hemoglobin (Hb) stability; however, the mechanism of how Hb maintains its stability under oxidative stress conditions of red blood cells (RBCs) carrying high levels of oxygen is unknown. Here, we investigated the potential role of peroxiredoxin II (Prx II) in preventing Hb aggregation induced by reactive oxygen species (ROS) using Prx II knockout mice and RBCs of patients with hemolytic anemia. Upon oxidative stress, ROS and Heinz body formation were significantly increased in Prx II knockout RBCs compared to wild-type (WT), which ultimately accelerated the accumulation of hemosiderin and heme-oxygenase 1 in the Prx II knock-out livers. In addition, ROS-dependent Hb aggregation was significantly increased in Prx II knockout RBCs. Interestingly, Prx II interacted with Hb in mouse RBCs, and their interaction, in particular, was severely impaired in RBCs of patients with thalassemia (THAL) and sickle cell anemia (SCA). Hb was bound to the decameric structure of Prx II, by which Hb was protected from oxidative stress. These findings suggest that Prx II plays an important role in preventing hemolytic anemia from oxidative stress by binding to Hb as a decameric structure to stabilize it. PMID:22960070

  18. Bendamustine-induced immune hemolytic anemia in a chronic lymphocytic leukemia patient: A case report and review of the literature.

    PubMed

    Haddad, Housam; Mohammad, Farhan; Dai, Qun

    2014-12-01

    Bendamustine is an alkylating agent approved for the treatment of chronic lymphocytic leukemia (CLL) and B-cell non-Hodgkin lymphoma. There are scant reports on bendamustine-induced immune hemolytic anemia occurring mainly in CLL patients. We report a case of immune hemolytic anemia that developed after exposure to bendamustine in a 70-year-old female with CLL who was previously exposed to fludarabine. Previous exposure to fludarabine is a common finding in the majority of reported cases of bendamustine drug-induced immune hemolytic anemia (DIIHA), including our case. Bendamustine should be suspected as the cause of any hemolytic anemia that develops while on this drug, especially in CLL patients treated previously with fludarabine. PMID:24785506

  19. A Thermolabile Aldolase A Mutant Causes Fever-Induced Recurrent Rhabdomyolysis without Hemolytic Anemia

    PubMed Central

    Mamoune, Asmaa; Bahuau, Michel; Hamel, Yamina; Serre, Valérie; Pelosi, Michele; Habarou, Florence; Nguyen Morel, Marie-Ange; Boisson, Bertrand; Vergnaud, Sabrina; Viou, Mai Thao; Nonnenmacher, Luc; Piraud, Monique; Nusbaum, Patrick; Vamecq, Joseph; Romero, Norma; Ottolenghi, Chris; Casanova, Jean-Laurent; de Lonlay, Pascale

    2014-01-01

    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. PMID:25392908

  20. Coombs-Negative Autoimmune Hemolytic Anemia in Crohn’s Disease

    PubMed Central

    Park, Bong Soo; Park, Sihyung; Jin, Kyubok; Kim, Yeon Mee; Park, Kang Min; Lee, Jeong-Nyeo; Kamesaki, Toyomi; Kim, Yang Wook

    2014-01-01

    Patient: Female, 41 Final Diagnosis: Coombs negative autoimmune hemolytic anemia Symptoms: Dark urine • dizziness • dyspnea Medication: — Clinical Procedure: Immunoradiometric assay for RBC-IgG Specialty: Hematology Objective: Rare disease Background: Anemia is a common, important extraintestinal complication of Crohn’s disease. The main types of anemia in patients with Crohn’s disease are iron deficiency anemia and anemia of chronic disease. Although patients with Crohn’s disease may experience various type of anemia, autoimmune hemolytic anemia (AIHA) in patients with Crohn’s disease, especially Coombs-negative AIHA, is very rare. Case Report: A 41-year-old woman with Crohn’s disease presented to our emergency room (ER) with dark urine, dizziness, and shortness of breath. The activity of Crohn’s disease had been controlled, with Crohn’s disease activity index (CDAI) score below 100 point. On physical examination, the patient had pale conjunctivae and mildly icteric sclerae. Serum bilirubin was raised at 3.1 mg/dL, lactate dehydrogenase (LDH) level was 1418 U/L and the haptoglobin level was <3 mg/dL. Results of direct and the indirect Coombs tests were all negative. We then measured the RBC-IgG to evaluate the possibility of Coombs-negative AIHA. The result revealed that RBC-IgG level was 352 IgG molecules/cell, with the cut-off value at 78.5 IgG molecules/cell. Conclusions: We report a case of Coombs-negative AIHA in a patient with Crohn’s disease with chronic anemia, diagnosed by red blood cell-bound immunoglobulin G (RBC-IgG) and treated with steroids therapy. PMID:25488633

  1. Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia

    Microsoft Academic Search

    Yuki Hirai; Masayuki Iyoda; Takanori Shibata; Eijin Ashikaga; Nozomu Hosaka; Hiroki Suzuki; Hisako Nagai; Masanori Mukai; Hirokazu Honda; Aki Kuroki; Kozo Kitazawa; Tadao Akizawa

    2008-01-01

    A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic\\u000a anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was\\u000a diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities,\\u000a and antinuclear and anti-double-stranded DNA antibodies, along

  2. Occurrence of hemolytic anemia in patients with GBS treated with high-dose IVIg

    PubMed Central

    Biliciler, Suur; Wahed, Amer; Sheikh, Kazim

    2014-01-01

    Objective: We describe an underrecognized side effect of high-dose IV immunoglobulin (IVIg), hemolytic anemia. Background: There are no established guidelines on treating patients with Guillain-Barré syndrome (GBS) who relapse or do not improve after a standard course of treatment (IVIg or plasma exchange). Some centers will opt for a second course of the initial treatment. There is an ongoing trial of a second course of IVIg in patients with severe GBS. Methods: We retrospectively reviewed 4 patients with severe GBS who received high-dose IVIg. One patient inadvertently received a high dose of IVIg for Miller Fisher syndrome. All patients received a total of at least 2 courses of the standard dose of IVIg (total >4 g/kg). We review their clinical course and side effects. Results: All patients with non-O blood types developed clinically significant hemolytic anemia requiring blood transfusion. Conclusion: Hemolytic anemia may limit doses of IVIg for treatment of severe GBS in patients with non-O blood types. PMID:25520957

  3. The Effect of Erythropoiesis-Stimulating Agents in Patients with Therapy-Refractory Autoimmune Hemolytic Anemia

    PubMed Central

    Salama, Abdulgabar; Hartnack, Dirk; Lindemann, Hans-Walter; Lange, Hans-Joachim; Rummel, Mathias; Loew, Andreas

    2014-01-01

    Summary Background Many patients with autoimmune hemolytic anemia (AIHA) do not respond to standard therapy and/or may develop severe complications which can be of fatal outcome. There is some evidence that erythropoiesis-stimulating agents (ESAs) may be helpful in the management of such patients. Methods We describe the effect of ESAs in 12 new patients with therapy-refractory AIHA (7 of warm type and 5 of cold type) and review 5 previously reported cases. Serological testing was performed using standard methods. Results All patients responded well to treatment with ESAs. At least 5 of the 17 patients demonstrated complete recovery, and none of the patients developed significant adverse reactions due to treatment with ESAs. Conclusion The mechanism by which ESAs improves hemolysis in AIHA is not completely clear. In addition to increased production and prolonged RBC survival, it may inhibit eryptosis (programmed cell death). ESAs represent a new option in the treatment of decompensated and/or refractory AIHA of warm and cold type. However, more information is required to assess which patients can be treated with ESAs. PMID:25670934

  4. Anemia

    MedlinePLUS

    Anemia is a condition in which the body does not have enough healthy red blood cells. Red ... provide oxygen to body tissues. Other types of anemia include: Anemia due to B12 deficiency Anemia due ...

  5. Spleen cell population changes and hemolytic anemia in F344 rats with large granular lymphocyte leukemia.

    PubMed

    Stromberg, P C; Kociba, G J; Grants, I S; Krakowka, G S; Rinehart, J J; Mezza, L E

    1990-11-01

    A spontaneous large granular lymphocyte leukemia from a F344 rat was transplanted to 36 syngeneic recipients to study the interactions among leukemia, T lymphocytes, and the development of immunemediated hemolytic anemia. Six rats were euthanatized at biweekly intervals, and spleen weight, total spleen cellularity, and differential spleen cell counts were correlated with hemograms and osmotic fragility. Sequential changes in splenic architecture were correlated with hematologic parameters. Monoclonal antibodies defining all T lymphocytes (W3/13), T helper-inducer cells (W3/25), and T suppressor cells (OX-8) were used to identify T cells in immunocytochemical techniques on spleen sections, as well as in fluorescence activated cell sorter analysis of spleen cell suspensions. The onset of hemolytic anemic at 7 weeks after transplantation coincided with the first detection of tumor cells in the spleen and peripheral blood. Tumor cells first accumulated in the marginal zones, and then they infiltrated the red pulp sinusoids. Although the leukemia caused dispersion of the splenic lymphoid tissue, there was no significant lymphopenia, and the relative number of helper (W3/25+) and suppressor (OX-8+) lymphocytes did not change. Because the induction of anemia was a relatively early event in splenic involvement, we concluded that anemia was unrelated to disruption of lymphoid architecture; furthermore, it does not appear to be caused by changes in the numbers of regulatory T lymphocytes. PMID:2148994

  6. Zinc-induced hemolytic anemia caused by ingestion of pennies by a pup.

    PubMed

    Latimer, K S; Jain, A V; Inglesby, H B; Clarkson, W D; Johnson, G B

    1989-07-01

    A 4-month-old Pomeranian pup was examined because of anorexia, salivation, and persistent vomiting. Initial laboratory testing revealed marked hemolytic anemia with spherocytosis. Survey abdominal radiography revealed 4 metal objects which, when removed by gastrotomy, were identified as pennies. Of 4 pennies, 3 were minted since 1983 and were heavily pitted over the surface and rim. Partially digested pennies were composed of a copper-plated high zinc concentration alloy. Further laboratory testing indicated a marked increase in serum zinc concentration in the pup (28.8 mg/L), confirming metal toxicosis. Serum zinc concentrations decreased during recovery. PMID:2759899

  7. Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT.

    PubMed

    Hartert, A; Willenbacher, W; Günzelmann, S; Roemer, E; Basara, N; Fauser, A A; Kiehl, M G

    2001-02-01

    Hematopoietic stem cell transplantation (HSCT) is a treatment option for autoimmune diseases but can also cause clinical features similar to those of autoimmune diseases. In some of these cases the autoimmune-like condition is associated with autoimmune cytopenia, a complication that can be unresponsive to established treatment strategies and which may be fatal. The majority of cases reported on immune hemolytic anemia have been of alloimmune origin due to ABO red blood cell antigen incompatibilities between donor and recipient. We now report a patient with a lupus-like syndrome, presenting with severe thrombocytopenia and hemolytic anemia 9 months after HLA-mismatch, ABO compatible-related PBSCT who experienced no response to high-dose steroids, but who had a sustained response to repeated IvIG therapy. PMID:11277184

  8. Vitamin B12 and vitamin d deficiencies: an unusual cause of Fever, severe hemolytic anemia and thrombocytopenia.

    PubMed

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

  10. Direct antiglobulin ("Coombs") test-negative autoimmune hemolytic anemia: a review.

    PubMed

    Segel, George B; Lichtman, Marshall A

    2014-04-01

    We have reviewed the literature to identify and characterize reports of warm-antibody type, autoimmune hemolytic anemia in which the standard direct antiglobulin reaction was negative but a confirmatory test indicated that the red cells were opsonized with antibody. Three principal reasons account for the absence of a positive direct antiglobulin test in these cases: a) IgG sensitization below the threshold of detection by the commercial antiglobulin reagent, b) low affinity IgG, removed by preparatory washes not conducted at 4°C or at low ionic strength, and c) red cell sensitization by IgA alone, or rarely (monomeric) IgM alone, but not accompanied by complement fixation, and thus not detectable by a commercial antiglobulin reagent that contains anti-IgG and anti-C3. In cases in which the phenotype is compatible with warm-antibody type, autoimmune hemolytic anemia and the direct antiglobulin test is negative, an alternative method to detect low levels of IgG sensitization, use of 4°C, low ionic strength washes to prepare the cells for the direct antiglobulin test reaction to permit retention and identification of low affinity IgG antibodies, and, if the latter are uninformative, testing for sensitization with an anti-IgA, and, if necessary, an anti-IgM reagent identifies cases of warm-antibody type, immune hemolysis not verified by a commercial reagent. PMID:24411920

  11. Clinical outcomes of splenectomy in children: report of the splenectomy in congenital hemolytic anemia registry.

    PubMed

    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

    2015-03-01

    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 to 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 g/dl, 52 week 12.8?±?1.6 g/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

  12. Interferon-Beta-1b Induced Autoimmune Hemolytic Anemia in a Patient with MS: A Case Report.

    PubMed

    Saeedi, M; Forughipour, M; Sasannezhad, P; Shoeibi, A

    2011-03-01

    A 26-year-old lady with the diagnosis of multiple sclerosis who had received interferon beta1-b for eleven months was visited in MS clinic of our hospital because of icter and fatigue. Laboratory tests showed anemia, indirect hyperbillirubinemia, increased LDH, positive direct and indirect coomb's tests, and increased reticulocyte count and percentage. Other causes of autoimmune hemolytic anemia (AHA) and pre-existing AHA in the patient were ruled out. After INF discontinuation, symptoms disappeared, hemoglobin increased, and indirect coomb's test became negative. We concluded that autoimmune hemolytic anemia should be considered in all MS patients who receive interferon beta1-b and present with symptoms and signs of anemia. PMID:22737466

  13. Heinz-body hemolytic anemia from the ingestion of crude oil: a primary toxic effect in marine birds.

    PubMed

    Leighton, F A; Peakall, D B; Butler, R G

    1983-05-20

    Hemolytic anemia developed in young herring gulls and Atlantic puffins given daily oral doses of a Prudhoe Bay crude oil. Anemia developed 4 to 5 days after the initiation of oil ingestion and was accompanied by Heinz-body formation and a strong regenerative response. The data evince a toxic effect on circulating red blood cells involving an oxidative biochemical mechanism and the first clear evidence of a primary mechanism of toxicity from the ingestion of crude oil by birds. PMID:6844918

  14. Heinz-body hemolytic anemia from the ingestion of crude oil: a primary toxic effect in marine birds

    SciTech Connect

    Leighton, F.A. (Cornell Univ., Ithaca, NY); Peakall, D.B.; Butler, R.G.

    1983-05-20

    Hemolytic anemia developed in young herring gulls and Atlantic puffins given daily oral doses of a Prudhoe Bay crude oil. Anemia developed 4 to 5 days after the initiation of oil ingestion and was accompainied by Heinz-body formation and a strong regenerative response. The data evince a toxic effect on circulating red blood cells involving an oxidative biochemical mechanism and the first clear evidence of a primary mechanism of toxicity from the ingestion of crude oil by birds.

  15. Gingival overgrowth in a dog that received long-term cyclosporine for immune-mediated hemolytic anemia

    PubMed Central

    Namikawa, Kazuhiko; Maruo, Takuya; Honda, Mayumi; Hirata, Hitomi; Lynch, Jonathan; Madarame, Hiroo

    2012-01-01

    Gingival mass lesions developed when cyclosporine was administered for 600 days to a female, 7-year-old, longhaired dachshund diagnosed with intractable immune-mediated hemolytic anemia (IMHA). Histopathology indicated hyperplastic suppurative gingivitis. As the anemia improved, the dosage of cyclosporine A (CsA) was markedly decreased, and the mass lesions decreased in size and disappeared, thus suggesting that the mass lesions were an adverse reaction to CsA. PMID:22753966

  16. Myeloid Glycosylation Defects Lead to Spontaneous a CVID-Like Condition with Associated Hemolytic Anemia and Anti-Lymphocyte Autoimmunity

    PubMed Central

    Ryan, Sean O.; Abbott, Derek W.; Cobb, Brian A.

    2014-01-01

    Common variable immunodeficiency (CVID), the most frequent symptomatic primary immune deficiency in humans, is a heterogeneous group of immunologic disorders estimated to affect 1:10,000 – 1:50,000. Although a clear disease etiology remains elusive, a common characteristic of CVID is deficient IgG antibody production in response to infection or vaccination. Patients often also exhibit autoimmune cytopenias with symptoms of abnormal T cell function, including reductions in naïve T cells which correlate with clinical severity. Here, we discovered that targeted alterations in the glycome of the myeloid lineage lead to spontaneous immunodeficiency characteristic of both humoral and T cell dysfunction regularly found in human CVID. Mice carrying a myeloid-specific knockout of the Mgat2 gene encoding UDP-GlcNAc:?-6-D-mannoside ?-1,2-N-acetylglucosaminyltransferase II enzyme exhibit deficiencies in IgG responses to both protein and polysaccharide conjugate vaccines. Interestingly, the immunodeficiency is associated with decreased T cell activity due to a persistent autoimmune-mediated depletion of naïve T cells, which is induced by changes in erythrocyte surface glycosylation. The N-glycosylation dependent auto-epitopes that emerge on erythrocytes lead to autoimmune hemolytic anemia, and the causative auto-IgM cross-reacts with naïve T cells despite the lack of glycan change on T cells. These findings demonstrate that alterations in erythrocyte glycosylation trigger the development of autoantibodies directed at both erythrocytes and naïve T cells, revealing a possible mechanistic link between the induction of autoimmune hemolytic anemia, the reduction in naïve T cells, and poor antibody responses to vaccine in severe CVID patients. PMID:24795453

  17. [Successful rituximab treatment for acquired amegakaryocytic thrombocytopenic purpura complicated with Coombs-negative autoimmune hemolytic anemia].

    PubMed

    Hashimoto, Akari; Fujimi, Akihito; Kanisawa, Yuji; Matsuno, Teppei; Okuda, Toshinori; Minami, Shinya; Doi, Tadashi; Ishikawa, Kazuma; Uemura, Naoki; Tomaru, Utano

    2013-06-01

    Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m(2) per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab. PMID:23823096

  18. Erythrocyte membrane defects in hemolytic anemias found through derivative thermal analysis of electric impedance.

    PubMed

    Ivanov, I T; Tolekova, A; Chakaarova, P

    2007-06-10

    Hereditary hemolytic anemias originate mainly from defects in hemoglobin and plasma membrane proteins. Here, we propose a new method, thermal analysis of impedance, sensitive to membrane defects. It detects three processes in erythrocyte membrane; fall in membrane capacity at 49.5 degrees C and activation of passive PO(4)(2+) permeability at 37 degrees C and inorganic ions at 61.5 degrees C. The denaturation of spectrin is involved in the first process whilst the anion channel is involved in latter processes. Using this method three persons with xerocytosis were found whereby the fall in membrane capacity and spherization of erythrocytes were both postponed (53 degrees C) compared to control (49.5 degrees C). In contrast to control cells, strong activation of passive permeability for Cl(-) at 37 degrees C and sucrose at 61 degrees C were detected that were both eliminated by pre-inhibition of the anion channel with 4,4'-diisothiocyanato-stilbene-2,2'-disulfonic acid (DIDS). In addition, erythrocytes from 15 patients with various forms of anemia were studied in intact state and after refreshment. The results were compared with the data of clinical laboratory and osmotic fragility test. The final conclusion is that this method detects membrane defects with altered spectrin and anion channel syndrome (hereditary xerocytosis, spherocytosis, poikilocytosis and pyropoikilocytosis, elliptocytosis and stomatocytosis) and, after refreshment, helps differentiate them from the anemia with hemoglobinopathy. PMID:17395266

  19. T cell deficiency in patients with autoimmune hemolytic anemia ('warm type').

    PubMed

    Krüger, J; Rahman, A; Mogk, K U; Mueller-Eckhardt, C

    1976-01-01

    19 patients with chronic 'warm type' autoimmune hemolytic anemia were studied for abnormalities of cellular immune reactions. Evidence was obtained for a reduction of rosette-forming cells (RFC). Lymphocytotoxic antibodies were present in only 8 patients and correlated, with only one exception, with a reduced number of RFC. No significant deviation from normal ranges of the three major immunoglobulin classes in the patients' sera were found. C3 and C4 complement components were also, with one exception, within normal limits. In 18 of 19 patients no apparent association existed between the type or the amount of autoantibodies and/or complement components fixed on red cells and the levels of the respective immunoglobulins or complement in the sera. PMID:1084624

  20. Demyelinating neuropathy and autoimmune hemolytic anemia in a patient with pancreatic cancer.

    PubMed

    Koike, Haruki; Yoshida, Hitoshi; Ito, Takahiko; Ohyama, Ken; Hashimoto, Rina; Kawagashira, Yuichi; Iijima, Masahiro; Sobue, Gen

    2013-01-01

    We herein report the case of a patient with pancreatic cancer who manifested features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and autoimmune hemolytic anemia (AIHA). A 78-year-old Japanese man presented with AIHA and was treated with steroids and splenectomy. Although the AIHA improved following splenectomy, the patient suffered from sensorimotor neuropathy soon after undergoing surgery. The electrophysiological features indicated demyelinating neuropathy. The neuropathy was refractory to immunomodulatory treatment, and intensive investigations revealed pancreatic cancer. The patient's neurological deficits improved significantly after the surgery for cancer. Although the combination of AIHA and CIDP has been reported anecdotally, this is the first case of the coexistence of these diseases as paraneoplastic syndromes. PMID:23903509

  1. Erythrocytic Pyruvate Kinase Mutations Causing Hemolytic Anemia, Osteosclerosis, and Secondary Hemochromatosis in Dogs

    PubMed Central

    Gultekin, G. Inal; Raj, K.; Foureman, P.; Lehman, S.; Manhart, K.; Abdulmalik, O.; Giger, U.

    2013-01-01

    Background Erythrocytic pyruvate kinase (PK) deficiency, first documented in Basenjis, is the most common inherited erythroenzymopathy in dogs. Objectives To report 3 new breed-specific PK-LR gene mutations and a retrospective survey of PK mutations in a small and selected group of Beagles and West Highland White Terriers (WHWT). Animals Labrador Retrievers (2 siblings, 5 unrelated), Pugs (2 siblings, 1 unrelated), Beagles (39 anemic, 29 other), WHWTs (22 anemic, 226 nonanemic), Cairn Terrier (n = 1). Methods Exons of the PK-LR gene were sequenced from genomic DNA of young dogs (<2 years) with persistent highly regenerative hemolytic anemia. Results A nonsense mutation (c.799C>T) resulting in a premature stop codon was identified in anemic Labrador Retriever siblings that had osteosclerosis, high serum ferritin concentrations, and severe hepatic secondary hemochromatosis. Anemic Pug and Beagle revealed 2 different missense mutations (c.848T>C, c.994G>A, respectively) resulting in intolerable amino acid changes to protein structure and enzyme function. Breed-specific mutation tests were developed. Among the biased group of 248 WHWTs, 9% and 35% were homozygous (affected) and heterozygous, respectively, for the previously described mutation (mutant allele frequency 0.26). A PK-deficient Cairn Terrier had the same insertion mutation as the affected WHWTs. Of the selected group of 68 Beagles, 35% were PK-deficient and 3% were carriers (0.37). Conclusions and Clinical Importance Erythrocytic PK deficiency is caused by different mutations in different dog breeds and causes chronic severe hemolytic anemia, hemosiderosis, and secondary hemochromatosis because of chronic hemolysis and, an as yet unexplained osteosclerosis. The newly developed breed-specific mutation assays simplify the diagnosis of PK deficiency. PMID:22805166

  2. Peritoneal EMH in a dog with immune-mediated hemolytic anemia.

    PubMed

    Brenner, Karen; Pohlman, Lisa; Muldowney, Ian; Petersen, Don; Schermerhorn, Thomas

    2013-01-01

    Extramedullary hematopoiesis (EMH) is the process by which normal blood cells are produced outside the bone marrow. In humans, EMH effusions are rare and are characterized by the presence of megakaryocytes, immature erythrocytes, immature leukocytes, or combinations of those cells. To the authors' knowledge, this is the first report to describe a case of peritoneal EMH effusion in a dog. A 5 yr old castrated male shorthaired dachshund presented with a 2 day history of pigmenturia and inappetence. A complete blood count revealed regenerative anemia with marked agglutination, spherocytosis, and an acute inflammatory leukogram characterized by a neutrophilia, regenerative left shift, and monocytosis. Ultrasound-guided aspiration of peritoneal effusion yielded a sample of high nucleated cellularity predominantly composed of mature and immature neutrophils and erythroid precursor cells. The patient was diagnosed with primary immune-mediated hemolytic anemia with concurrent EMH peritoneal effusion. The following case description and discussion explore the clinical findings associated with the unusual effusion and outline the possible pathogenesis by which the EMH effusion may have arisen in the dog. PMID:23690489

  3. Marrow transplantation in the treatment of a murine heritable hemolytic anemia

    SciTech Connect

    Barker, J.E.; McFarland-Starr, E.C.

    1989-05-15

    Mice with hemolytic anemia, sphha/sphha, have extremely fragile RBCs with a lifespan of approximately one day. Neither splenectomy nor simple transplantation of normal marrow after lethal irradiation cures the anemia but instead causes rapid deterioration and death of the mutant unless additional prophylactic procedures are used. In this report, we show that normal marrow transplantation preceded by sublethal irradiation increases but does not normalize RBC count. The mutant RBCs but not all the WBCs are replaced by donor cells. Splenectomy of the improved recipient causes a dramatic decrease in RBC count, indicating that the mutant spleen is a site of donor-origin erythropoiesis as well as of RBC destruction. Injections of iron dextran did not improve RBC counts. Transplantation of primary recipient marrow cells into a secondary host with a heritable stem cell deficiency (W/Wv) corrects the defect caused by residence of the normal cells in the sphha/sphha host. The original +/+ donor cells replace the RBCs of the secondary host, and the RBC count is normalized. Results indicate that the environment in the sphha/sphha host is detrimental to normal (as well as mutant) erythroid cells but the restriction is not transmitted.

  4. Reappraisal of the etiology of extracorpuscular non-autoimmune acquired hemolytic anemia in 2657 hospitalized patients with non-neoplastic disease

    Microsoft Academic Search

    V. C. Kok; C. K. Lee; J. T. Horng; C. C. Lin; F. C. Sung

    2014-01-01

    INTRODUCTION: Unlike autoimmune hemolytic anemia (AIHA), literature on the etiological study of non-autoimmune hemolytic anemia (non-AIHA) is scarce. The incidence and prevalence of non-AIHA in different geographic regions are largely unknown perhaps owing to the lack of perspective investigation and different profiles of etiologies from different geographic regions. We aimed to examine the real-world etiology or mechanisms of the non-hereditary

  5. Immune-mediated Hemolytic Anemia and Thrombocytopenia in the Dog: A retrospective study of 55 cases diagnosed from 1979 through 1983 at the Western College of Veterinary Medicine

    PubMed Central

    Jackson, Marion L.; Kruth, Stephen A.

    1985-01-01

    All recognized cases (n = 55) of immune-mediated hemolytic anemia and immune-mediated thrombocytopenia in dogs presented to the Western College of Veterinary Medicine from 1969 through 1983 were reviewed. Specific areas of concern were: association with other conditions, therapeutic response, prognosis, relapse rate and final outcome. Of these 55 cases, 19 were immune-mediated hemolytic anemia, 26 were immune-mediated thrombocytopenia and 10 were both immune-mediated hemolytic anemia and thrombocytopenia. Females were slightly over-represented and the mean age was 6.4 years. Therapy consisted of various combinations of immuno-suppressive drugs and in some cases, whole blood transfusion and splenectomy. No firm conclusions could be made regarding therapeutic efficacy, as a result of variation in treatment protocol and the occasional unavailability of follow-up data. Well over half of all cases were diagnosed as idiopathic. Precipitating factors or diseases most frequently implicated in secondary immune-mediated thrombocytopenia or hemolytic anemia were: recent vaccination, drug therapy, obstetrical complications, stress, recent viral infection and neoplasia. Twice as many cases of immune-mediated hemolytic anemia were seen in the cooler months (October to March), although this could not be related to antibody class or thermal reactivity. Immune-mediated thrombocytopenia both as a single disease and combined with immune-mediated hemolytic anemia had no seasonal incidence. History, clinical findings and hematological and clinical chemistry findings were consistent with data previously reported, with the exception of icterus, which appeared to be of higher incidence than most reports, being present in almost 50% of immune-mediated hemolytic anemia cases. Just over half of all dogs survived, although the survival rate was highest for immune-mediated hemolytic anemia, followed closely by immune-mediated thrombocytopenia and lowest for the combined disease. Immune-mediated thrombocytopenia most frequently ran a relapsing course requiring long-term or intermittent therapy. PMID:17422562

  6. [Serological characteristics and transfusion efficacy evaluation in 61 cases of autoimmune hemolytic anemia].

    PubMed

    Yu, Yang; Sun, Xiao-Lin; Ma, Chun-Ya; Guan, Xiao-Zhen; Zhang, Xiao-Juan; Chen, Lin-Fen; Wang, Ke; Luo, Yuan-Yuan; Wang, Yi; Li, Ming-Wei; Feng, Yan-Nan; Tong, Shan; Yu, Shuai; Yang, Lu; Wu, Yue-Qing; Zhuang, Yuan; Pan, Ji-Chun; Fen, Qian; Zhang, Ting; Wang, De-Qing

    2013-10-01

    This study was aimed to analyze the serological characteristics, efficacy and safety of incompatible RBC transfusion in patients with autoimmune hemolytic anemia (AIHA). The patients with idiopathic or secondary AIHA were analyzed retrospectively, then the serological characteristics and the incidence of adverse transfusion reactions were investigated, and the efficacy and safety of incompatible RBC transfusion were evaluated according to the different autoantibody type and infused different RBC components. The results showed that out of 61 cases of AIHA, 21 cases were idiopathic, and 40 cases were secondary. 8 cases (13.1%) had IgM cold autoantibody, 50 cases (82.0%) had IgG warm autoantibody, and 3 cases (4.9%) had IgM and IgG autoantibodies simultaneously. There were 18 cases (29.5%) combined with alloantibodies. After the exclusion of alloantibodies interference, 113 incompatible RBC transfusions were performed for 36 patients with AIHA, total efficiency rate, total partial efficiency rate and total inefficiency rate were 56.6%, 15.1% and 28.3%, respectively. Incompatible RBC transfusions were divided into non-washed RBC group and washed RBC group. The efficiency rate, partial efficiency rate and inefficiency rate in non-washed RBC group were 57.6%, 13.0% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in washed RBC group were 53.6%, 21.4% and 25.0%, respectively. There was no significant difference of transfusion efficacy (P > 0.05) in two groups. Incompatible RBC transfusions were also divided into IgM cold autoantibody group and IgG warm autoantibody group. The efficiency rate, partial efficiency rate and inefficiency rate in IgM cold autoantibody group were 46.2%, 30.8% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in IgG warm autoantibody group were 56.7%, 13.4% and 29.9%, respectively. There was no significant difference of transfusion efficacy (P > 0.05 ) in two groups. Hemolytic transfusion reaction was not observed in all incompatible RBC transfusions. It is concluded that the same ABO type of non-washed RBC transfusion and O type washed RBC transfusion are all relatively safe for the AIHA patients with severe anemia after the exclusion of alloantibodies interference. There is no significant difference of transfusion efficacy in two groups. The same ABO type of non-washed RBC transfusion is more convenient and efficient than washed RBC transfusion, and excessive use of type O RBCs can also be avoided. PMID:24156449

  7. Establishment of permanent chimerism in a lactate dehydrogenase-deficient mouse mutant with hemolytic anemia

    SciTech Connect

    Datta, T.; Doermer, P.

    1987-12-01

    Pluripotent hemopoietic stem cell function was investigated in the homozygous muscle type lactate dehydrogenase (LDH-A) mutant mouse using bone marrow transplantation experiments. Hemopoietic tissues of LDH-A mutants showed a marked decreased in enzyme activity that was associated with severe hemolytic anemia. This condition proved to be transplantable into wild type mice (+/+) through total body irradiation (TBI) at a lethal dose of 8.0 Gy followed by engraftment of mutant bone marrow cells. Since the mutants are extremely radiosensitive (lethal dose50/30 4.4 Gy vs 7.3 Gy in +/+ mice), 8.0-Gy TBI followed by injection of even high numbers of normal bone marrow cells did not prevent death within 5-6 days. After a nonlethal dose of 4.0 Gy and grafting of normal bone marrow cells, a transient chimerism showing peripheral blood characteristics of the wild type was produced that returned to the mutant condition within 12 weeks. The transfusion of wild type red blood cells prior to and following 8.0-Gy TBI and reconstitution with wild type bone marrow cells prevented the early death of the mutants and permanent chimerism was achieved. The chimeras showed all hematological parameters of wild type mice, and radiosensitivity returned to normal. It is concluded that the mutant pluripotent stem cells are functionally comparable to normal stem cells, emphasizing the significance of this mouse model for studies of stem cell regulation.

  8. Autoimmune hemolytic anemia and thrombocytopenia attributed to an intrauterine contraceptive device

    PubMed Central

    Khawandanah, Mohamad O.; Weiss, Susan M.; Cherry, Mohamad A.; Maymani, Hossein; Selby, George B.; Aster, Richard H.; George, James N.; Holter Chakrabarty, Jennifer L.

    2015-01-01

    BACKGROUND Evans syndrome is a rare condition manifested by combined autoimmune hemolytic anemia (AIHA) and thrombocytopenia or neutropenia. It is often associated with other autoimmune disorders, immunodeficiencies, and non-Hodgkin’s lymphoma. CASE REPORT We describe a patient with Evans syndrome that may have been related to exposure to a polyethylene-based intrauterine contraceptive device (IUD). A 26-year-old white female presented with severe, symptomatic AIHA and subsequently developed severe thrombocytopenia. She had a refractory course resistant to multiple treatments including corticosteroids, intravenous immune globulin, rituximab, splenectomy, cyclophosphamide, cyclosporine, eculizumab, and plasma exchange. It was then noticed that her serum autoantibody agglutinated red blood cells (RBCs) in the presence of polyethylene glycol (PEG) but not in the absence of PEG nor when an alternative agglutination enhancing technique, low-ionic-strength solution, was used. Therefore, her polyethylene-containing IUD, which was a polyethylene frame with a levonorgestrel-releasing device, was removed. Norgestrel-dependent, platelet (PLT)-reactive antibodies were not identified by either flow cytometry or in vivo in a NOD/SCID mouse. Testing for PEG-dependent antibodies was not possible. Remission, with no requirement for RBC or PLT transfusions and return of her hemoglobin and PLT counts to normal, followed removal of the IUD. CONCLUSION The patient’s recovery after removal of the IUD and the PEG dependence of RBC agglutination suggested a possibility that the IUD may have been a contributing factor to the etiology of Evans syndrome in this patient. PMID:25208591

  9. The role of the spleen in a lactate dehydrogenase mutant mouse (Ldh-1c/Ldh-1c) with hemolytic anemia.

    PubMed

    Datta, T; Kremer, J P; Hültner, L; Dörmer, P

    1988-05-01

    The lactate dehydrogenase mouse mutant Ldh-1c/Ldh-1c is afflicted with a severe hemolytic anemia associated with extreme reticulocytosis (95%) and splenomegaly. Ninety-one percent of the total body colony-forming units--erythroid (CFU-E) have been quantified in the seven- to ten-times enlarged spleens of the mutant mice. Moreover, the splenic fraction of morphologically recognizable erythroid precursors was 134 times normal. From these data it was apparent that the spleen crucially contributes to the maintenance of steady state erythropoiesis in the mutants. On the other hand, an enhanced sequestration of red blood cells in the enlarged spleen may augment the anemia. Splenectomy experiments were performed with LDH mutant and wild type mice in order to investigate the role of the spleen in this particular hemolytic disease. Following splenectomy, the peripheral blood values and the frequency of femoral stem and progenitor cells were determined, and histological investigations were carried out. The life span of the splenectomized mutants was not shortened, in spite of a very low red blood cell count (25% of the untreated mutant value). Compared to the splenic loss only a moderate increase in bone marrow erythropoiesis was observed, such as a 250% increase of CFU-E. It is concluded that the reduction in red blood cell survival due to splenic sequestration in the mutants is of such a magnitude that it counterbalances a significant portion of splenic erythropoiesis. PMID:3360065

  10. [Characteristics of warm autoimmune hemolytic anemia and Evans syndrome in adults].

    PubMed

    Michel, Marc

    2008-09-01

    The diagnosis of autoimmune hemolytic anemia (AIHA) relies mainly on the direct antiglobulin test (DAT), that is, Coombs' direct test, which has a sensitivity of about 95%. The classification of different forms of AIHA depends on the characteristics of the autoantibody and is an essential part of the diagnostic procedure. AIHAs mediated by warm-reactive autoantibodies (wAIHA) account for approximately 70% of all types: they may be either idiopathic or secondary to another autoimmune disorder (such as systemic lupus, lymphoma or primary immune deficiencies) or drug-induced. In adults, AIHA may also precede by many years the onset of non-Hodgkin lymphoma (NHL) or myelodysplastic syndrome. The management of wAIHA, based mainly on empirical data and uncontrolled studies, relies principally on corticosteroids as a first-line therapy. In cases of steroid resistance ( approximately 5-10% of the cases) and in cases of steroid-dependency, the most frequent second-line options are splenectomy or immunosuppressive agents. More recently, rituximab has shown promising results in small uncontrolled and retrospective studies and it is now widely used in refractory wAIHA. Future prospective studies should assess its efficacy earlier in the course of disease as a steroid-sparing strategy. Evans syndrome is an autoimmune disorder defined by the simultaneous or sequential combination of AIHA and immune thrombocytopenia or immune neutropenia. It may reveal an underlying condition (e.g., lupus, common variable immunodeficiency, etc.). Its management is usually extrapolated from the standard of care for isolated wAIHA. Before the availability of rituximab, the overall prognosis was relatively poor for adults with both wAIHA and ES, with a mortality rate of 15 to 20%. PMID:18644324

  11. Severe Autoimmune Hemolytic Anemia in an Infant Caused by Warm-reactive IGM and IGA Autoantibodies: A Case Report and Review of the Literature.

    PubMed

    Branstetter, Cristyn N; Hankins, Jane S; Moreau, Dawn; Nottage, Kerri A

    2015-08-01

    Warm-reactive IgM autoimmune hemolytic anemia is uncommon and carries a poor prognosis in adults. There have been rare reports in children, generally associated with an underlying immunologic deficiency, and outcomes are quite variable. Warm IgM in combination with other antibodies has not been reported in children. We report the first case of severe, steroid-responsive autoimmune hemolytic anemia caused by both warm-reactive IgM and IgA autoantibodies in an otherwise healthy 3-month-old. PMID:26181418

  12. IgG4-Related Disease Combined with Autoimmune Hemolytic Anemia and Steroid-Responsive Transient Hypercalcemia

    PubMed Central

    Hasegawa, Sho; Mine, Sohtaro; Hagiwara, Shotaro

    2015-01-01

    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.

  13. [Acute epiglottitis due to group A ?-hemolytic streptococcus in a child].

    PubMed

    Mazenq, J; Retornaz, K; Vialet, R; Dubus, J-C

    2015-06-01

    Acute epiglottitis has become an exceptional observation in pediatrics. The introduction of Haemophilus influenzae type B vaccine changed the morbidity, mortality, and microbiology of this disease. We report the case of an 11-month-old infant with acute epiglottitis due to group A ?-hemolytic streptococcus. PMID:25282454

  14. Anemia (For Parents)

    MedlinePLUS

    About Anemia Anemia, one of the more common blood disorders, occurs when the level of healthy red blood cells ( ... cancer, or exposure to a drug or toxin. Anemia Caused by Destruction of RBCs Hemolytic anemia occurs ...

  15. Human\\/mouse radiation chimera generated from PBMC of B chronic lymphocytic leukemia patients with autoimmune hemolytic anemia produce anti-human red cell antibodies

    Microsoft Academic Search

    H Marcus; A Shimoni; D Ergas; A Canaan; B Dekel; D Ben-David; M David; E Sigler; Y Reisner; A Berrebi

    1997-01-01

    Previous studies performed in our laboratory have shown that B-CLL cells are involved in the production of anti-red cell auto-antibodies, providing a possible mechanism for the auto-immune hemolytic anemia occurring during the course of B-CLL. In order to confirm this hypothesis, we attempted to transfer human B-CLL with AIHA to immunodeficient mice. Peripheral blood mononuclear cells (PBMC) from 11 B-CLL

  16. IgA-mediated human autoimmune hemolytic anemia as a result of hemagglutination in the spleen, but independent of complement activation and Fc?RI.

    PubMed

    Chadebech, Philippe; Michel, Marc; Janvier, Daniel; Yamada, Kazunori; Copie-Bergman, Christiane; Bodivit, Gwellaouen; Bensussan, Armand; Fournie, Jean-Jacques; Godeau, Bertrand; Bierling, Philippe; Izui, Shozo; Noizat-Pirenne, France

    2010-11-18

    Autoimmune hemolytic anemia (AIHA) due to warm-acting IgA autoantibodies is rare. We explored the pathogenic mechanisms underlying destruction of red blood cells (RBCs) in a patient with severe AIHA mediated exclusively by polymeric immunoglobulin A (pIgA) anti-Band 3 autoantibodies. The follow-up period was 17 months. RBCs were not destroyed by complement activation as no deposition of complement was observed on the patient's RBCs. pIgA eluted from the patient's RBCs did not induce RBC destruction through phagocytosis by monocytes or antibody-dependent cell-mediated cytotoxicity by natural killer cells. Induction of eryptosis (ie, RBC apoptosis) due to direct alteration of the RBC membrane by pIgA autoantibodies was also excluded. By contrast, upon incubation with pIgA-opsonized RBCs, substantial RBC membrane transfers (ie, trogocytosis) to monocytes were observed that might contribute to RBC immune destruction. This effect was poorly inhibited by blockers of Fc receptors, excluding a major contribution of Fc?RI to this process. Histologic analysis revealed a massive accumulation of agglutinated RBCs with little sign of erythrophagocytosis in the spleen. These results, together with the efficacy of splenectomy 17 months after AIHA onset, suggest that the trapping and subsequent sequestration of agglutinated RBCs in the spleen are the principal pathogenic mechanisms of pIgA-mediated AIHA. PMID:20644119

  17. Anemias

    Microsoft Academic Search

    Rosalind Bryant

    \\u000a Anemia is defined as a reduction in the red cell mass due to decreased production, increased loss\\/ decreased survival, or\\u000a increased destruction of red blood cells (RBCs). As most of the oxygen is transported by the RBCs to the body tissues, a reduction\\u000a in the red cell mass causes reduced oxygen supply to the body cells. Consequently, anemia is a

  18. Deficiency of Nicotinamide Mononucleotide Adenylyltransferase 3 (Nmnat3) Causes Hemolytic Anemia by Altering the Glycolytic Flow in Mature Erythrocytes*

    PubMed Central

    Hikosaka, Keisuke; Ikutani, Masashi; Shito, Masayuki; Kazuma, Kohei; Gulshan, Maryam; Nagai, Yoshinori; Takatsu, Kiyoshi; Konno, Katsuhiro; Tobe, Kazuyuki; Kanno, Hitoshi; Nakagawa, Takashi

    2014-01-01

    NAD biosynthesis is of substantial interest because of its important roles in regulating various biological processes. Nicotinamide mononucleotide adenylyltransferase 3 (Nmnat3) is considered a mitochondria-localized NAD synthesis enzyme involved in de novo and salvage pathways. Although the biochemical properties of Nmnat3 are well documented, its physiological function in vivo remains unclear. In this study, we demonstrated that Nmnat3 was localized in the cytoplasm of mature erythrocytes and critically regulated their NAD pool. Deficiency of Nmnat3 in mice caused splenomegaly and hemolytic anemia, which was associated with the findings that Nmnat3-deficient erythrocytes had markedly lower ATP levels and shortened lifespans. However, the NAD level in other tissues were not apparently affected by the deficiency of Nmnat3. LC-MS/MS-based metabolomics revealed that the glycolysis pathway in Nmnat3-deficient erythrocytes was blocked at a glyceraldehyde 3-phosphate dehydrogenase (GAPDH) step because of the shortage of the coenzyme NAD. Stable isotope tracer analysis further demonstrated that deficiency of Nmnat3 resulted in glycolysis stall and a shift to the pentose phosphate pathway. Our findings indicate the critical roles of Nmnat3 in maintenance of the NAD pool in mature erythrocytes and the physiological impacts at its absence in mice. PMID:24739386

  19. A deep intronic mutation in the ankyrin-1 gene causes diminished protein expression resulting in hemolytic anemia in mice.

    PubMed

    Huang, Hua; Zhao, PengXiang; Arimatsu, Kei; Tabeta, Koichi; Yamazaki, Kazuhisa; Krieg, Lara; Fu, Emily; Zhang, Tian; Du, Xin

    2013-10-01

    Linkage between transmembrane proteins and the spectrin-based cytoskeleton is necessary for membrane elasticity of red blood cells. Mutations of the proteins that mediate this linkage result in various types of hemolytic anemia. Here we report a novel N-ethyl-N-nitrosourea-induced mutation of ankyrin-1, named hema6, which causes hereditary spherocytosis in mice through a mild reduction of protein expression. The causal mutation was traced to a single nucleotide transition located deep into intron 13 of gene Ank1. In vitro minigene splicing assay revealed two abnormally spliced transcripts containing cryptic exons from fragments of Ank1 intron 13. The inclusion of cryptic exons introduced a premature termination codon, which leads to nonsense-mediated decay of the mutant transcripts in vivo. Hence, in homozygous mice, only wild-type ankyrin-1 is expressed, albeit at 70% of the level in wild-type mice. Heterozygotes display a similar hereditary spherocytosis phenotype stemming from intermediate protein expression level, indicating the haploinsufficiency of the mutation. Weakened linkage between integral transmembrane protein, band 3, and underlying cytoskeleton was observed in mutant mice as the result of reduced high-affinity binding sites provided by ankyrin-1. Hema6 is the only known mouse mutant of Ank1 allelic series that expresses full-length canonical ankyrin-1 at a reduced level, a fact that makes it particularly useful to study the functional impact of ankyrin-1 quantitative deficiency. PMID:23934996

  20. Narrative Review: Paroxysmal Nocturnal Hemoglobinuria: The Physiology of Complement-Related Hemolytic Anemia

    NSDL National Science Digital Library

    Robert A. Brodsky (Johns Hopkins Medicine)

    2008-04-15

    Physiology in Medicine review article. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem-cell disorder caused by a somatic mutation in a gene known as phosphatidylinositol glycan class A (PIGA). It may arise de novo or in the setting of acquired aplastic anemia.The absence of GPI-anchored proteins leads to complement-mediated intravascular hemolysis, because 2 important complement regulatory proteins (CD55 and CD59) are missing from PNH cells. Therapeutic options include supportive care, bone marrow transplantation, and monoclonal antibody therapy with the terminal complement inhibitor eculizumab.

  1. IgG red blood cell autoantibodies in autoimmune hemolytic anemia bind to epitopes on red blood cell membrane band 3 glycoprotein

    SciTech Connect

    Victoria, E.J.; Pierce, S.W.; Branks, M.J.; Masouredis, S.P. (Univ. of California, San Diego (USA))

    1990-01-01

    Red blood cell (RBC) autoantibodies from patients with IgG warm-type autoimmune hemolytic anemia were labeled with iodine 125 and their RBC binding behavior characterized. Epitope-bearing RBC membrane polypeptides were identified after autoantibody immunoprecipitation of labeled membranes and immunoblotting. Immunoaffinity isolation of labeled membrane proteins with 12 different IgG hemolytic autoantibodies with protein A-agarose revealed a major polypeptide at Mr 95 to 110 kd, which coelectrophoresed on sodium dodecylsulfate-polyacrylamide gel electrophoresis with a membrane component isolated with sheep IgG anti-band 3. Immunoprecipitation studies with chymotrypsinized RBCs resulted in the recovery of two labeled membrane polypeptides with molecular weights characteristically resulting from the chymotryptic fragmentation of band 3. Immunoblotting with sheep IgG anti-band 3 of the immunoprecipitated polypeptides confirmed that hemolytic autoantibody binding led to recovery of band 3 or its fragments. Two 125I-labeled IgG hemolytic autoantibodies showed binding behavior consistent with epitope localization on band 3. The labeled RBC autoantibodies bound immunospecifically to all types of human RBC tested, including those of rare Rh type (Rh-null, D--) at a site density of approximately 10(6) per RBC. The 125I-IgG in two labeled autoantibodies was 84% and 92% adsorbable by human and higher nonhuman primate RBCs. Antigen-negative animal RBC bound less than 10%, consistent with immunospecific RBC binding. IgG-1 was the major subclass in five autoantibodies tested; one of six fixed complement; and autoantibody IgG appeared polyclonal by isoelectric focusing. We conclude that IgG eluted from RBCs of patients with autoimmune hemolytic anemia consists predominantly of a single totally RBC-adsorbable antibody population that binds to antigenic determinants on band 3.

  2. AMPD3-deficient mice exhibit increased erythrocyte ATP levels but anemia not improved due to PK deficiency.

    PubMed

    Cheng, Jidong; Morisaki, Hiroko; Toyama, Keiko; Ikawa, Masahito; Okabe, Masaru; Morisaki, Takayuki

    2012-11-01

    AMP deaminase (AMPD) catalyzes AMP to IMP and plays an important role in energy charge and nucleotide metabolism. Human AMPD3 deficiency is a type of erythrocyte-specific enzyme deficiency found in individuals without clinical symptoms, although an increased level of ATP in erythrocytes has been reported. To better understand the physiological and pathological roles of AMPD3 deficiency, we established a line of AMPD3-deficient [A3(-/-)] mice. No AMPD activity and a high level of ATP were observed in erythrocytes of these mice, similar to human RBC-AMPD3 deficiency, while other characteristics were unremarkable. Next, we created AMPD3 and pyruvate kinase (PK) double-deficient [PKA(-/-,-/-)] mice by mating A3(-/-) mice with CBA-Pk-1slc/Pk-1slc mice [PK(-/-)], a spontaneous PK-deficient strain showing hemolytic anemia. In PKA(-/-,-/-) mice, the level of ATP in red blood cells was increased 1.5 times as compared to PK(-/-) mice, although hemolytic anemia in those animals was not improved. In addition, we observed osmotic fragility of erythrocytes in A3(-/-) mice under fasting conditions. In contrast, the ATP level in erythrocytes was elevated in A3(-/-) mice as compared to the control. In conclusion, AMPD3 deficiency increases the level of ATP in erythrocytes, but does not improve anemia due to PK deficiency and leads to erythrocyte dysfunction. PMID:23078545

  3. A molecular defect in two families with hemolytic poikilocytic anemia: reduction of high affinity membrane binding sites for ankyrin.

    PubMed Central

    Agre, P; Orringer, E P; Chui, D H; Bennett, V

    1981-01-01

    Patients from two families with chronic hemolytic anemia have been studied. The erythrocytes are very fragile and appear microcytic with a great variety of shapes. Clinical evaluation failed to identify traditionally recognized causes of hemolysis. Sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE) showed no significant abnormality of the major polypeptide bands. Erythrocytes spectrin-ankyrin and ankyrin-membrane interactions were analyzed with 125I-labeled spectrin, 125I-labeled ankyrin, and inside-out vesicles. Patients' vesicles bound 125I-spectrin normally. Likewise, patients' spectrin and ankyrin competed normally for the binding sites on control membranes. None of the individual components appeared to have abnormal thermal sensitivity. Ankyrin-stripped, inside-out vesicles prepared from the patients bound less 125I-ankyrin than did vesicles prepared from normals (P less than 0.05 for all corresponding points in the high-affinity region). Scatchard analysis showed the most significant abnormality to be a 50% reduction in the high affinity ankyrin binding sites. Similar experiments were performed with blood from patients with spherocytosis and splenectomized controls, but no abnormalities were detected. The water soluble 43,000-dalton fragments of band 3 (the high-affinity ankyrin binding sites) were prepared from one of the patients and competed normally for 125I-ankyrin binding in solution. This suggests that the primary structural defect is a reduction in the number of high affinity membrane binding sites for ankyrin, and is consistent with an abnormal organization of band 3 in the membrane. Images PMID:6459341

  4. Lenoir et al., 1 Iron deficiency anemia due to matriptase-2 inactivation is dependent upon

    E-print Network

    Paris-Sud XI, Université de

    Lenoir et al., 1 1 Iron deficiency anemia due to matriptase-2 inactivation is dependent upon, hepcidin, anemia, Bmp6, hemojuvelin inserm-00552073,version1-5Jan2011 Author manuscript, published by increased hepcidin levels and anemia) and Bmp6-/- mice (exhibiting severe iron overload due to hepcidin

  5. Anemia

    MedlinePLUS

    What Is Anemia? Lots of teens are tired. With all the demands of school and other activities, it's easy to understand ... get enough iron in their diets. Iron Deficiency Anemia Iron deficiency anemia is the most common type ...

  6. Anemia

    MedlinePLUS

    ... 3 million Americans. Jump To: The Role of Red Blood Cells in Anemia Red blood cells carry hemoglobin, an iron-rich protein ... Anemia occurs when you do not have enough red blood cells or when your red blood cells ...

  7. Severe hemolytic transfusion reaction due to anti-D in a D+ patient with sickle cell disease.

    PubMed

    Ipe, Tina S; Wilkes, Jennifer J; Hartung, Helge D; Westhoff, Connie M; Chou, Stella T; Friedman, David F

    2015-03-01

    A 5-year-old male with sickle cell disease presented with pain, dark urine, and fatigue 10 days after a red blood cell (RBC) transfusion. Laboratory evaluation demonstrated severe anemia, blood type O+, and anti-D in the serum. Anti-D in a D+ patient led to RH genotyping, which revealed homozygosity for RHD*DAU4 that encodes partial D antigen. Anti-D in this patient whose RBCs exclusively express partial D caused a delayed hemolytic transfusion reaction after exposure to D+ RBCs. The finding of anti-D in a D+patient should be investigated by molecular methods to help distinguish an alloantibody from an autoantibody. PMID:25171447

  8. Anti Rh Hemolytic Disease due to Anti C Antibody: Is Testing for Anti D Antibodies Enough?

    PubMed

    Negi, Gita; Singh, Gaur Dushyant

    2012-06-01

    Rh blood group system is a complex blood group system. Rh antibodies are produced in Rh negative individuals following exposure to foreign RBCs after transfusion or pregnancy. Anti C is a rare cause of hemolytic disease of newborn and is very scarcely reported in the literature. The aim of the present case report of Hemolytic disease caused by Anti C antibody is to bring out the fact that antibodies other than anti D should be considered in cases that give a suggestive history but no evidence of Anti D. PMID:23730022

  9. Hemolytic uremic syndrome.

    PubMed

    Webster, Kathleen; Schnitzler, Eugene

    2014-01-01

    The thrombotic microangiopathies include both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Although debate exists as to whether these are separate entities or a spectrum of disease, both result in the clinical picture of thrombocytopenia, hemolytic anemia, and varying degrees of renal and neurologic involvement. Etiology of HUS includes diarrheal infection due to Shiga toxin-producing bacteria, complement deficiency, pneumococcal infection, and cobalamin deficiency. In disease ascribed to TTP, the main etiologic factor is deficiency of an enzyme known as a disintegrin-like and metalloprotease with thrombospondin type 1 repeats, number 13 (ADAMTS-13). The clinical manifestations may vary, but neurologic involvement can be significant, with reports of hypertensive encephalopathy, seizures, thrombosis and infarct. In nondiarrheal forms of disease, recurrence may occur and clinical diagnosis is essential in order to provide a targeted therapy for the suspected etiology. Therapies include supportive care, cobalamin supplementation, as well as plasma infusion and exchange. End stage renal disease may result and transplantation is curative for some forms of the disease. More recent research focuses on targeted immunotherapy to prevent autoantibody prevention. As of yet, there is no one cure for these potentially devastating diseases, and diagnosis and treatment selection presents a challenge to the clinician. PMID:24365375

  10. Identification, Molecular Characterization, and Experimental Transmission of a New Hemoplasma Isolate from a Cat with Hemolytic Anemia in Switzerland

    Microsoft Academic Search

    Barbara Willi; Felicitas S. Boretti; Valentino Cattori; Severine Tasker; Marina L. Meli; Claudia Reusch; Hans Lutz; Regina Hofmann-Lehmann

    2005-01-01

    Recently, there has been a growing interest in hemotropic mycoplasmal species (also known as the hemo- plasmas), the causative agents of infectious anemia in several mammalian species. In felids, two different hemoplasma species have been recognized: Mycoplasma haemofelis (formerly Haemobartonella felis) and \\

  11. Zinc-induced hemolytic anemia in a dog caused by ingestion of a game-playing die

    PubMed Central

    Clancey, Noel P.; Murphy, Megan C.

    2012-01-01

    A 16-month-old spayed female mixed breed dog was presented with a 1-week history of anorexia, lethargy, diarrhea, vomiting, and difficulty rising. Hematologic evaluation indicated a marked macrocytic hypo-chromic, markedly regenerative anemia. A metallic foreign object in the gastrointestinal tract was identified on abdominal radiographs. Serum zinc concentration was markedly increased. PMID:23024383

  12. Hemolytic uremic syndrome due to Capnocytophaga canimorsus bacteremia after a dog bite

    Microsoft Academic Search

    Tom J. M. Tobé; Casper F. M. Franssen; Jan G. Zijlstra; Paul E. de Jong; Coen A. Stegeman

    1999-01-01

    The hemolytic uremic syndrome (HUS) is known to have several causes, including infectious diseases, drugs, pregnancy, and malignant disease. We report a patient who developed acute renal failure attributable to HUS in the course of Capnocytophaga canimorsus bacteremia. Acute tubular necrosis as well as HUS should be considered as a cause of acute renal failure in the setting of Capnocytophaga

  13. Anemia

    MedlinePLUS

    If you have anemia, your blood does not carry enough oxygen to the rest of your body. The most common cause of anemia is not having enough ... rich protein that gives the red color to blood. It carries oxygen from the lungs to the ...

  14. Severe methemoglobinemia and hemolytic anemia from aniline purchased as 2C-E (4-ethyl-2,5-dimethoxyphenethylamine), a recreational drug, on the Internet - Oregon, 2011.

    PubMed

    2012-02-10

    In August 2011, two men in Oregon drank a liquid they believed to be 2C-E (4-ethyl-2,5-dimethoxyphenethylamine), a psychoactive stimulant used as a recreational drug, after purchasing it on the Internet. Fifteen minutes after ingestion, the men became cyanotic and subsequently were treated for refractory methemoglobinemia and hemolytic anemia. The Oregon Poison Center, Oregon Public Health Division, Drug Enforcement Administration (DEA), and Food and Drug Administration (FDA) jointly investigated to determine the cause of the poisoning and identify other cases. The Oregon Poison Center and Oregon Public Health Division promptly alerted health-care providers and public health agencies and searched for additional cases. DEA confiscated all product remaining in the men's possession, and FDA identified the substance as aniline, an industrial solvent known to cause methemoglobinemia. One patient reported purchasing the substance from the Internet site of a Chinese chemical company. No additional cases were identified by investigators. Purchase of chemicals from unregulated Internet sources poses a serious risk to purchasers from product contamination and substitution. PMID:22318470

  15. [Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjögren's syndrome].

    PubMed

    Shimoji, Sonoko; Takiuchi, Yohko; Maruoka, Hayato; Inoue, Daichi; Kimura, Takaharu; Mori, Minako; Nagai, Yuya; Togami, Katuhiro; Tabata, Sumie; Kurata, Masayuki; Matsushita, Akiko; Nagai, Kenichi; Takahashi, Takayuki

    2011-07-01

    A 73-year-old woman with Sjögren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×10(9)/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sjögren's syndrome. PMID:21821986

  16. Aplastic anemia and red cell aplasia due to pentachlorophenol

    SciTech Connect

    Roberts, H.J.

    1983-01-01

    Repeated exposure to commercial (technical grade) pentachlorophenol (PCP) preceded aplastic anemia in four patients and pure red cell aplasia in two. Two patients developed concomitant or subsequent Hodgkin's disease and acute leukemia. The hematologic, mutagenic, and carcinogenic effect of PCP and its chemical contaminants have been documented in other clinical and experimental reports. In view of the widespread contamination of our environment by PCP, clinicians and public health investigators must seek out such exposure in these and related disorders and initiate measures to reduce it.

  17. Bendamustine and rituximab combination in the management of chronic lymphocytic leukemia-associated autoimmune hemolytic anemia: a multicentric retrospective study of the French CLL intergroup (GCFLLC/MW and GOELAMS).

    PubMed

    Quinquenel, Anne; Willekens, Christophe; Dupuis, Jehan; Royer, Bruno; Ysebaert, Loic; De Guibert, S; Michallet, Anne-Sophie; Feugier, Pierre; Guieze, Romain; Levy, Vincent; Delmer, Alain

    2015-03-01

    We report our experience on bendamustine and rituximab (BR) combination in 26 patients with chronic lymphocytic leukemia (CLL) complicated by autoimmune hemolytic anemia (AIHA). At the time of BR initiation, 88% of the patients had already been treated for AIHA and CLL was progressive regardless of AIHA in all patients but one. Overall response rates were 81% for AIHA and 77% for CLL. Median time to next treatment was 28.3 months and 26.2 months for AIHA and CLL, respectively. BR therapy may represent a good and safe therapeutic option in this setting where adequate control of CLL seems important for long-term AIHA response. PMID:25428829

  18. Y O U R G U I D E T O Iron-Deficiency Anemia

    E-print Network

    Bandettini, Peter A.

    Y O U R G U I D E T O Anemia Iron-Deficiency Anemia Pernicious Anemia Aplastic Anemia Hemolytic Anema Anemia Healthy Lifestyle Changes Prevent Treat Control #12;#12;Y O U R G U I D E T O Anemia AnemiaHealthy Lifestyle Changes Prevent Treat Control Iron-Deficiency Anemia Pernicious Anemia Aplastic

  19. Hemolytic disease of the newborn due to anti-jkb: case report and review of the literature.

    PubMed

    Velasco Rodríguez, Diego; Pérez-Segura, G; Jiménez-Ubieto, A; Rodríguez, M A; Montejano, L

    2014-06-01

    Although anti-Jkb is a well-defined cause of severe acute or delayed hemolytic transfusion reactions, it is rarely associated with severe Hemolytic Disease of the Newborn (HDN), even with high antibody titer. To date, only 13 cases have been reported, so the possible reasons for that still remain unclear. Most of HDN due to anti-Jkb are mild-to-moderate, and usually have a good prognosis. A 41-years-old woman, who had a positive antibody screening test in her 13th week of pregnancy, was sent to the blood bank for study before an amniocentesis. Antibody identification and red blood cell (RBC) phenotyping of the patient and his husband were performed, plus arrays study in the amniotic fluid. An anti-Jkb was identified in the patient's serum with a titer of 1:1, and her RBC phenotype was O Rh(D) positive, C(+), c(+), E(-), e(+), K(-), Jka(+), Jkb(-). The RBC genotype of the fetus was B Rh(D) positive, Jka(+), Jkb(+). Antibody titer remained stable and the pregnancy was uneventful. At birth, there was no need of phototherapy or exchange transfusion for the newborn and her Jk(b+) typing result was confirmed in a cord blood sample. Although most of HDN cases due to anti-Jkb have a good outcome, monitoring antibody titer should be done to prevent fatal complications. Furthermore, antenatal antibody screening should be performed in every pregnant woman irrespective of her Rh(D) antigen status in order to detect red cell alloimmunization to other clinically significant blood group antigens. PMID:24839369

  20. Case Report: Severe Hemolytic Disease of the Newborn Due to anti-Dib Treated with Phototherapy and Intravenous Immunoglobulin

    Microsoft Academic Search

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

    The Di b antigen usually occurs with high incidence, except in certain Asian and South American Indian populations. In general, hemolysis caused by anti-Dib is not severe and its clinical course is benign. We report a Korean neonate with severe hemolytic disease of the newborn caused by anti-Dib. The phenotype and genotype of the Diego blood group system of the

  1. Epstein-Barr virus associated diffuse large B-cell lymphoma complicated by autoimmune hemolytic anemia and pure red cell aplasia.

    PubMed

    Katayama, H; Takeuchi, M; Yoshino, T; Munemasa, M; Tada, A; Soda, R; Takahashi, K

    2001-07-01

    A 53-year old man with systemic lymphadenopathy and hepatosplenomegaly was diagnosed with diffuse large B cell-lymphoma after inguinal lymph node biopsy. Anemia was noted, direct and indirect Coombs tests were positive, and the haptoglobin level was low. However, the bone marrow aspirate revealed erythroid aplasia. Co-existing autoimmune haemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were diagnosed. In situ hybridization with Epstein-Barr virus (EBV) encoded small RNA (EBER) showed positive findings in lymphoma cells. Southern blot hybridization revealed immunoglobulin heavy chain gene rearrangement and a clonal EBV terminal repeat, indicating monoclonal proliferation of EBV in infected B cells. The patient was treated with CHOP, resulting in a complete remission (CR). AIHA and PRCA subsided after 3 courses of chemotherapy. In conclusion, this case demonstrates not only the association of B-cell lymphoma with autoimmune disorders but also the involvement of EBV in these conditions. PMID:11699422

  2. Folate-deficiency anemia

    MedlinePLUS

    Folate-deficiency anemia is a decrease in red blood cells ( anemia ) due to a lack of folate. Folate is a type ... B vitamin. It is also called folic acid. Anemia is a condition in which the body does ...

  3. Evaluation of stem cell reserve using serial bone marrow transplantation and competitive repopulation in a murine model of chronic hemolytic anemia

    SciTech Connect

    Maggio-Price, L.; Wolf, N.S.; Priestley, G.V.; Pietrzyk, M.E.; Bernstein, S.E.

    1988-09-01

    Serial transplantation and competitive repopulation were used to evaluate any loss of self-replicative capacity of bone marrow stem cells in a mouse model with increased and persistent hemopoietic demands. Congenic marrows from old control and from young and old mice with hereditary spherocytic anemia (sphha/sphha) were serially transplanted at 35-day intervals into normal irradiated recipients. Old anemic marrow failed or reverted to recipient karyotype at a mean of 3.5 transplants, and young anemic marrow reverted at a mean of 4.0 transplants, whereas controls did so at a mean of 5.0 transplants. In a competitive assay in which a mixture of anemic and control marrow was transplanted, the anemic marrow persisted to 10 months following transplantation; anemic marrow repopulation was greater if anemic marrow sex matched with the host. It is possible that lifelong stress of severe anemia decreases stem cell reserve in the anemic sphha/sphha mouse marrow. However, marginal differences in serial transplantation number and the maintenance of anemic marrow in a competition assay would suggest that marrow stem cells, under prolonged stress, are capable of exhibiting good repopulating and self-replicating abilities.

  4. Hemolytic uremic syndrome associated with glomerular disease.

    PubMed

    Siegler, R L; Brewer, E D; Pysher, T J

    1989-02-01

    Secondary hemolytic uremic syndrome (HUS) is uncommon. When it occurs, it is usually in association with pregnancy, malignancy, severe hypertension, drugs, or collagen vascular diseases. It has rarely been reported in patients with glomerular disease. Two such patients with secondary HUS are described. A 17-month-old girl with hematuria and the nephrotic syndrome, negative antistreptolycin O (ASO) titer, and low serum levels of C3 and C4 developed oliguria, progressive azotemia, thrombocytopenia, and microangiopathic hemolytic anemia. A kidney biopsy showed fibrin in glomerular capillaries and cresentic membranoproliferative glomerulonephritis. A 22-year-old man with a 16-year history of relapsing minimal change nephrotic syndrome had been in remission for 5 years when he experienced nephrotic syndrome relapse and developed thrombocytopenia, microangiopathic hemolytic anemia, and renal failure. A kidney biopsy revealed foot process fusion and obstruction of glomerular capillaries with fibrin and platelets. These cases illustrate that HUS can occur in association with other glomerular diseases and should be considered when thrombocytopenia and hemolytic anemia occur in a nephritic or nephrotic patient. PMID:2916569

  5. Inborn anemias in mice: (Annual report, 1981-1982)

    SciTech Connect

    Bernstein, S.E.

    1982-07-19

    Hereditary anemias of mice are the chief objects of investigation, specificially four macrocytic anemias, 3 types of hemolytic anemia, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, the autoimmune hemolytic anemia of NZB mice, an ..cap alpha..-thalassemia and a new hypochromic anemia with hemochromatosis. New types of anemia may be analyzed as new mutations appear. Three new mutations have been identified during the past 18 months. These anemias are studied through characterization of peripheral blood values, determinations of radiosensitivity under a variety of conditions, measurements of iron metabolism and heme synthesis, study of normal and abnormal erythrocyte membrane proteins, histological and biochemical characterization of blood-forming tissue, functional tests of the stem-cell component, examination of responses to erythroid stimuli, and transplantation of tissue and parabiosis between individuals of differently affected genotypes. 31 refs.

  6. Inborn anemias in mice

    SciTech Connect

    Bernstein, S.E.; Barker, J.E.; Russell, E.S.

    1981-06-01

    hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, five hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus our wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values, (b) determinations of radiosensitivity under a variety of conditions, (c) measurements of iron metabolism and heme synthesis, (d) histological and biochemical study of blood-forming tissue, (e) functional tests of the stem cell component, (f) examination of responses to erythroid stimuli, and (g) transplantation of tissue between individuals of differently affected genotypes.

  7. Blood loss anemia due to adenocarcinoma of the jejunum: case report and review of the literature

    Microsoft Academic Search

    João Figueira-Coelho; Sofia Lourenço; Michele Costa; Paula Mendonça; António Murinello; Jorge Neta

    2009-01-01

    BACKGROUND: Small bowel tumors are rare, accounting for only 3-6% of gastrointestinal neoplasms, 1-2% of these being malignant. They must be considered whenever a patient presents with gastrointestinal bleeding, with normal upper gastrointestinal endoscopy and colonoscopy. CASE PRESENTATION: We report a case of jejunal adenocarcinoma presenting as a blood loss anemia in a 65 year-old male, doing a brief review

  8. Pernicious anemia

    MedlinePLUS

    Macrocytic achylic anemia; Congenital pernicious anemia; Juvenile pernicious anemia; Vitamin B12 deficiency (malabsorption) ... Pernicious anemia is a type of vitamin B12 anemia. The body needs vitamin B12 to make red blood cells. ...

  9. A New Alkaline pH-Adjusted Medium Enhances Detection of ?-Hemolytic Streptococci by Minimizing Bacterial Interference Due to Streptococcus salivarius

    PubMed Central

    Dierksen, Karen P.; Ragland, Nancy L.; Tagg, John R.

    2000-01-01

    A new selective medium (CNA-P) that reduces or eliminates the inhibitory activity of bacteriocin-producing Streptococcus salivarius against ?-hemolytic streptococci has been developed and compared with sheep blood agar (SBA) for the sensitive detection of small numbers of ?-hemolytic streptococci in clinical specimens. CNA-P has as its basis a commercial medium (Difco Columbia CNA agar) supplemented with 5% (vol/vol) sheep blood, and the CNA is further modified by addition of 100 mM PIPES buffer [piperazine-N,N?-bis(2-ethanesulfonic acid)] (pH 7.5) to maintain cultures at an alkaline pH during incubation. CNA-P was shown to inhibit the production and/or release of four different types of S. salivarius bacteriocins or bacteriocin-like inhibitory molecules. The efficacies of CNA-P and SBA for detection of ?-hemolytic streptococci in 1,352 pharyngeal samples from 376 children were compared. The ?-hemolytic streptococcal isolates recovered from the samples included 314 group A (S. pyogenes), 61 group G, 33 group B, and 5 group C streptococci. Of 314 samples that yielded S. pyogenes, 300 were positive on CNA-P (96%) and 264 (86%) were positive on SBA. A significantly greater number of S. pyogenes isolates from these samples were recovered only on CNA-P (50 of 314) compared with the number of isolates recovered only on SBA (14 of 314). In addition, the degree of positivity, a measure of the total numbers of S. pyogenes isolates on the plate, was significantly higher on CNA-P than on SBA (2.40 versus 2.07; P < 0.001). Interestingly, CNA-P was also found to enhance the hemolytic activity of streptolysin O, allowing detection of streptolysin S-deficient S. pyogenes strains which might otherwise go undetected on SBA and other isolation media. PMID:10655361

  10. A rare cause of anemia due to intestinal tuberculosis in a renal transplant recipient.

    PubMed

    Kandutsch, S; Feix, A; Haas, M; Häfner, M; Sunder-Plassmann, G; Soleiman, A

    2004-08-01

    A renal transplant recipient with stable allograft function presented with massive hemorrhagic diarrhea and severe anemia. No microbial infection could be found in stool cultures. Early colonoscopy showed severe colitis with ulceration. Histological samples confirmed granulomatous inflammation with acid-resistant Ziehl-Neelson-positive microorganisms of mycobacterial type. Polymerase chain reaction (PCR) analysis of native mucosal biopsies specified the infectious organism as Mycobacterium tuberculosis complex. The patient responded well to antimycobacterial therapy and was still asymptomatic after 6 months with a stable graft function. Our case shows that tuberculosis can be a severe clinical problem in transplant recipients. Most of the patients with intestinal tuberculosis, reported to literature, were diagnosed post mortem or after explorative laparotomy and bowel resection. Thus, intestinal tuberculosis should be considered when a transplant recipient shows abdominal symptoms with no clear evidence of another infection. Proper diagnosis and treatment resulted in a beneficial outcome in our patient. PMID:15356975

  11. Optimal management of iron deficiency anemia due to poor dietary intake

    PubMed Central

    Aspuru, Kattalin; Villa, Carlos; Bermejo, Fernando; Herrero, Pilar; López, Santiago García

    2011-01-01

    Iron is necessary for the normal development of multiple vital processes. Iron deficiency (ID) may be caused by several diseases, even by physiological situations that increase requirements for this mineral. One of its possible causes is a poor dietary iron intake, which is infrequent in developed countries, but quite common in developing areas. In these countries, dietary ID is highly prevalent and comprises a real public health problem and a challenge for health authorities. ID, with or without anemia, can cause important symptoms that are not only physical, but can also include a decreased intellectual performance. All this, together with a high prevalence, can even have negative implications for a community’s economic and social development. Treatment consists of iron supplements. Prevention of ID obviously lies in increasing the dietary intake of iron, which can be difficult in developing countries. In these regions, foods with greater iron content are scarce, and attempts are made to compensate this by fortifying staple foods with iron. The effectiveness of this strategy is endorsed by multiple studies. On the other hand, in developed countries, ID with or without anemia is nearly always associated with diseases that trigger a negative balance between iron absorption and loss. Its management will be based on the treatment of underlying diseases, as well as on oral iron supplements, although these latter are limited by their tolerance and low potency, which on occasions may compel a change to intravenous administration. Iron deficiency has a series of peculiarities in pediatric patients, in the elderly, in pregnant women, and in patients with dietary restrictions, such as celiac disease. PMID:22114518

  12. Aplastic Anemia

    MedlinePLUS

    ... from the NHLBI on Twitter. What Is Aplastic Anemia? Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is ... heart, heart failure , infections, and bleeding. Severe aplastic anemia can even cause death. Overview Aplastic anemia is ...

  13. Intrauterine transfusion for fetal anemia due to red blood cell alloimmunization: 14 years experience in Leuven

    PubMed Central

    Pasman, S.A.; Claes, L.; Lewi, L.; Van Schoubroeck, D.; Debeer, A.; Emonds, M.; Geuten, E.; De Catte, L.; Devlieger, R.

    2015-01-01

    Objective: The purpose of this study is to report on the pregnancy and neonatal outcome of intrauterine transfusion (IUT) for red blood cell (RBC-)alloimmunization. Material and Methods: Retrospective cohort study of all IUT for RBC-alloimmunization in the University Hospital of Leuven, between January 2000 and January 2014. The influence of hydrops, gestational age and technique of transfusion on procedure related adverse events were examined. Results: 135 IUTs were performed in 56 fetuses. In none of the cases fetal or neonatal death occurred. Mild adverse events were noted in 10% of IUTs, whereas severe adverse events occurred in 1.5%. Hydrops and transfusion in a free loop were associated with an increased risk of adverse events whereas gestational age (GA) at transfusion after 34 weeks was not. Median GA at birth was 35.6 weeks and 9% was born before 34 weeks. Besides phototherapy 65.4% required additional neonatal treatment for alloimmune anemia. Non-hematologic complications occurred in 23.6% and were mainly related to preterm birth. Conclusion: In experienced hands, IUT for RBC-alloimmunization is a safe procedure in this era. Patients should be referred to specialist centers prior to the development of hydrops. IUT in a free loop of cord and unnecessary preterm birth are best avoided. PMID:26175890

  14. A Case of Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab

    PubMed Central

    Thajudeen, B.; Sussman, A.; Bracamonte, E.

    2013-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal failure. Eculizumab, a monoclonal complement C5 antibody which prevents the induction of the terminal complement cascade, has recently emerged as a therapeutic option for aHUS. We report a case of aHUS successfully treated with eculizumab. A 51-year-old male was admitted to the hospital following a mechanical fall. His past medical history was significant for rheumatic valve disease and mitral valve replacement; he was on warfarin for anticoagulation. A computed tomography scan of the head revealed a right-sided subdural hematoma due to coagulopathy resulting from a supratherapeutic international normalized ratio (INR). Following treatment with prothrombin complex concentrate to reverse the INR, urine output dropped and his serum creatinine subsequently increased to 247.52 ?mol/l from the admission value of 70.72 ?mol/l. Laboratory evaluation was remarkable for hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase (LDH), low haptoglobin, and low complement C3. A renal biopsy was consistent with TMA, favoring a diagnosis of aHUS. Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH. Hemodialysis was terminated after 2.5 months due to improvement in urine output and solute clearance. The interaction between thrombin and complement pathway might be responsible for the pathogenesis of aHUS in this case. Eculizumab is an effective therapeutic agent in the treatment of aHUS. Early targeting of the complement system may modify disease progression and thus treat aHUS more effectively. PMID:24570684

  15. A case of atypical hemolytic uremic syndrome successfully treated with eculizumab.

    PubMed

    Thajudeen, B; Sussman, A; Bracamonte, E

    2013-07-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy (TMA) characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal failure. Eculizumab, a monoclonal complement C5 antibody which prevents the induction of the terminal complement cascade, has recently emerged as a therapeutic option for aHUS. We report a case of aHUS successfully treated with eculizumab. A 51-year-old male was admitted to the hospital following a mechanical fall. His past medical history was significant for rheumatic valve disease and mitral valve replacement; he was on warfarin for anticoagulation. A computed tomography scan of the head revealed a right-sided subdural hematoma due to coagulopathy resulting from a supratherapeutic international normalized ratio (INR). Following treatment with prothrombin complex concentrate to reverse the INR, urine output dropped and his serum creatinine subsequently increased to 247.52 ?mol/l from the admission value of 70.72 ?mol/l. Laboratory evaluation was remarkable for hemolytic anemia, thrombocytopenia, elevated lactate dehydrogenase (LDH), low haptoglobin, and low complement C3. A renal biopsy was consistent with TMA, favoring a diagnosis of aHUS. Treatment with eculizumab was initiated which resulted in the stabilization of his hemoglobin, platelets, and LDH. Hemodialysis was terminated after 2.5 months due to improvement in urine output and solute clearance. The interaction between thrombin and complement pathway might be responsible for the pathogenesis of aHUS in this case. Eculizumab is an effective therapeutic agent in the treatment of aHUS. Early targeting of the complement system may modify disease progression and thus treat aHUS more effectively. PMID:24570684

  16. [Hemolytic uremic syndrome caused by enterohaemorrhagic Escherichia coli].

    PubMed

    Ibarra, Cristina; Goldstein, Jorge; Silberstein, Claudia; Zotta, Elsa; Belardo, Marcela; Repetto, Horacio A

    2008-10-01

    Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, plaquetopenia and kidney damage. It is the leading cause of acute renal failure in pediatric age and the second for chronic renal failure. Shiga toxin-producing Escherichia coli (STEC) is the first etiologic agent of HUS being its main reservoir cattle and transmitted via contaminated food. At present, there is no specific treatment to reduce the progression of HUS. The study of the mechanisms by which STEC infects and Shiga toxin induces HUS can help to find new strategies to prevent this disease. PMID:19030644

  17. Shiga toxin-associated hemolytic uremic syndrome: pathophysiology of endothelial dysfunction

    Microsoft Academic Search

    Carla Zoja; Simona Buelli; Marina Morigi

    2010-01-01

    Shiga toxin (Stx)-producing enterohemorrhagic Escherichia coli O157:H7 has become a global threat to public health, as a primary cause of a worldwide spread of hemorrhagic colitis complicated\\u000a by diarrhea-associated hemolytic uremic syndrome (HUS), a disorder of thrombocytopenia, microangiopathic hemolytic anemia,\\u000a and acute renal failure that mainly affects early childhood. Endothelial dysfunction has been recognized as the trigger event\\u000a in the

  18. Pernicious Anemia

    MedlinePLUS

    ... from the NHLBI on Twitter. What Is Pernicious Anemia? Pernicious anemia (per-NISH-us uh-NEE-me-uh) is ... nervous system working properly. People who have pernicious anemia can't absorb enough vitamin B12 from food. ...

  19. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks

    PubMed Central

    Camc?o?lu, Burcu; Bo?nak-Güçlü, Meral; Karadall?, Mü?errefe Nur; Ak?, ?ahika Zeynep; Türköz-Sucak, Gülsan

    2015-01-01

    Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS.

  20. Anemia in hemodialysis patients.

    PubMed

    Higgins, M R; Grace, M; Ulan, R A; Silverberg, D S; Bettcher, K B; Dossetor, J B

    1977-02-01

    The association between anemia and chronic renal failure has been recognized since the early 19th century. With the introduction of regular dialysis treatment, an understanding of all aspects of this uremic complication has become of great importance, including an appreciation of the hazards of multiple blood transfusions. This analysis of hemoglobin levels and transfusion requirements in 84 dialysis patients focuses specific attention on hemolytic mechanisms, blood loss, and the effect of bilateral nephrectomy on erythropoiesis. Because no replacement for renal erythropoietin is available, particular attention must be paid to less important, but partially correctable factors that contribute to anemia. Blood transfusion requirements can then be reduced to a minimum, together with the risks of hypersplenism, hepatitis, and sensitization of the patient to alloantigens. PMID:836115

  1. Immune-mediated severe hemolytic crisis with a hemoglobin level of 1.6 g/dl caused by anti-piperacillin antibodies in a patient with cystic fibrosis.

    PubMed

    Kunzmann, S; Thomas, W; Mayer, B; Kuhn, S; Hebestreit, H

    2010-04-01

    We report a 23-year-old female patient with cystic fibrosis developing severe intravascular hemolysis with a minimal hemoglobin level of 1.6 g/dl after 7 days of treatment with piperacillin, consistent with an immune-mediated hemolytic crisis. Twenty days later, the patient could leave the hospital in good condition without any neurological deficit. To our knowledge, this is the lowest reported hemoglobin value caused by hemolytic anemia with intact survival. As piperacillin is commonly used in patients with cystic fibrosis, it is important to monitor the full-blood counts of patients during treatment with piperacillin and to be aware of the potential for hemolytic anemia to develop. Anti-piperacillin antibodies should be considered whenever these patients develop hemolytic anemia or a positive direct antiglobulin test (DAT). Furthermore, drug-fever under piperacillin application could be a warning sign for the development of hemolytic anemia. PMID:20213286

  2. About Anemia

    MedlinePLUS

    ... the shape changes, as is the case in sickle cell anemia , the red blood cells can get stuck and ... hard for them to move throughout the body. Sickle cell anemia is one of many genetic conditions that can ...

  3. Aplastic anemia

    MedlinePLUS

    Aplastic anemia is a condition in which the bone marrow does not make enough new blood cells. Bone marrow ... Aplastic anemia results from injury to the blood stem cells. These are immature cells in the bone marrow that ...

  4. Aplastic Anemia

    MedlinePLUS

    Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... cause is unknown. Your doctor will diagnose aplastic anemia based on your medical and family histories, a ...

  5. Fanconi Anemia

    MedlinePLUS

    ... on Twitter. What Is Fanconi Anemia? Fanconi anemia (fan-KO-nee uh-NEE-me-uh), or FA, ... much more likely than others to develop cancerous solid tumors. The risk of solid tumors increases with ...

  6. Hematologic Disorders: Anemia.

    PubMed

    Baltierra, David; Harper, Tiffany; Jones, Matthew Page; Nau, Konrad C

    2015-06-01

    Anemia occurs in up to 25% of the US population. Normal hemoglobin levels vary by race, sex, and age. Classification of anemia by mean corpuscular volume guides the differential diagnosis and evaluation. Iron studies, reticulocyte count, the red blood cell distribution width index, and blood test results are used to make the diagnosis. Iron deficiency anemia is the most common microcytic anemia and is managed with iron therapy. Parenteral iron is available when the oral route cannot be used. Patients who do not benefit from therapy should be evaluated for adherence, malabsorption, occult bleeding, systemic disease, or less common inherited disorders. A source of gastrointestinal bleeding is found in 60% to 70% of patients with iron deficiency anemia who are referred for endoscopy. Normocytic anemia has a broad differential, including nutritional deficiencies, blood loss, renal disease, malignancy (solid tumors or hematologic cancer), rheumatologic disorders, endocrine disorders, and other systemic diseases. Macrocytic anemias are seen with vitamin B12 and folate deficiency, alcohol use, thyroid disease, hydroxyurea, antiretroviral drugs, myelodysplastic syndromes, and myeloma. Oral vitamin B12 is underused, and can be as effective as intramuscular vitamin B12 in managing anemia due to vitamin B12 deficiency. PMID:26080453

  7. Glucose-6-phosphate dehydrogenase deficiency

    MedlinePLUS

    G-6-PD deficiency; Hemolytic anemia due to G6PD deficiency; Anemia - hemolytic due to G6PD deficiency ... Churchill Livingston; 2008:chap 45. Golan DER. Hemolytic anemias: red cell membrane and metabolic defects. In: Goldman ...

  8. Studies on the role of chicken blood groups A and B in the induction of anemia in chicks 

    E-print Network

    Johnson, Lewis Warren

    1956-01-01

    . The assistance of the author's wife, Marilyn Johnson, was valuable in compiling these data and preparing the manu? script. INTRODUCTION This study deals principally with three considerations: the in? duction of hemolytic anemia in chicks as a result... of the chicken. These studies constitute an extension of work initiated earlier by Briles (l94Sa). He discovered that baby chicks receiving the 3 ^ allele from their heterozygous sire suffered severe hemolytic anemia after their dam had been sensitized...

  9. Haptoglobin blood test

    MedlinePLUS

    ... disease Collection of blood (hematoma) Drug-induced immune hemolytic anemia Blood disorder in a fetus or newborn called erythroblastosis fetalis Hemolytic anemia due to G6PD deficiency Idiopathic autoimmune hemolytic anemia ...

  10. Development of an experimental hemolytic uremic syndrome in rats.

    PubMed

    Zotta, Elsa; Lago, Nestor; Ochoa, Federico; Repetto, Horacio A; Ibarra, Cristina

    2008-04-01

    Escherichia coli strains producing Shiga toxins (Stxs) colonize the lower gastrointestinal tract and cause watery diarrhea, hemorrhagic colitis, and hemolytic-uremic syndrome (HUS). HUS is characterized by hemolytic anemia, thrombocytopenia, and acute renal failure. Oliguria associated with acute tubular necrosis and microangiopathic thrombosis has been reported as the most common cause of renal failure in Argentinean children. Our study was undertaken to obtain a model of HUS in rats that was similar to the clinical and renal histopathology findings described in humans. Rats were intraperitoneally inoculated with culture supernatant from recombinant E. coli expressing Stx2. Glomerular filtrate volume evaluated from clearance of creatinine resulted in a progressive reduction (from 53% at 24 h to 90% at 48 h). Urine volume increased significantly at 24 h but returned to normal levels at 48 h. Evidence of thrombocytopenia, anemia and leukocytosis was documented. Macroscopic analysis revealed a hyperemic peritoneal face with intestinal water accumulation. The kidneys were friable and congestive. Histopathological analysis showed glomerular and tubular necrosis as well as microangiopathic thrombosis. Our findings indicated vascular damage and kidney lesions similar to those described in humans with HUS. PMID:18253762

  11. Pathogenesis of Hemolytic Anemia in Homozygous Hemoglobin C Disease*

    PubMed Central

    Charache, Samuel; Conley, C. Lockard; Waugh, David F.; Ugoretz, Richard J.; Spurrell, J. Richard

    1967-01-01

    Hemoglobin C is less soluble than hemoglobin A in red cells, in hemolysates, and in dilute phosphate buffer. Its relative insolubility may be explained by electrostatic interactions between positively charged ?6-lysyl groups and negatively charged groups on adjacent molecules. Red cells from patients with homozygous hemoglobin C (CC) disease exhibit aberrant physical properties which suggest that the cells are more rigid than normal erythrocytes. They pass through membrane filters less readily than normal red cells do, and their viscosity is higher than that of normal cells. Differences from normal cells are exaggerated if mean corpuscular hemoglobin concentration (MCHC) is increased, by suspension in hypertonic salt solution. Increased rigidity of CC cells, by accelerating their fragmentation, may be responsible for formation of microspherocytes. These small dense cells are exceptionally rigid, and probably are even more susceptible to fragmentation and sequestration. Rigidity of CC cells can be attributed to a “precrystalline” state of intracellular hemoglobin, in which crystallization does not occur, although the MCHC exceeds the solubility of hemoglobin in hemolysates. Images PMID:6061750

  12. [Severe hemolytic disease of the newborn as a result of late and undiagnosed alloimmunization--case report].

    PubMed

    Drozdowska-Szymczak, Agnieszka; Czapli?ska, Natalia; Borek-Dziecio?, Beata; Kociszewska-Najman, Bozena; Bartkowiak, Robert; Wielgo?, Miros?aw

    2014-03-01

    We report a case of a hemolytic disease in a newborn from the first pregnancy due to anti-D antibodies. The maternal blood group was A Rhesus negative. She had an antibody screening test twice during the pregnancy (in the second trimester) and it was negative. The pregnancy was uneventful, without any invasive procedures and bleeding. The infant was born at 39 weeks of gestation in good overall condition. After the delivery the blood group of the neonate was indicated - A Rhesus positive, BOC positive. Anti-D antibodies were detected in maternal blood. Neonatal blood tests revealed severe anemia (hemoglobin level: 6.0g/dl, hematocrit: 22.2%, erythrocytes: 2.01T/L). During the first day of neonatal life, the newborn received two transfusions of red blood cells. Bilirubin level and rate of rise were not recommendation enough for exchange transfusion. The newborn was treated with continuous phototherapy since the delivery The perinatal period was complicated with intrauterine infection and respiratory failure. Hematopoietic vitamins and iron supplementation was initiated in the second week of neonatal life due to persistent anemia. The child remained under medical care of a hematologic clinic and received human recombinant erythropoietin treatment. PMID:24783436

  13. Anaerobic induction of Bacillus anthracis hemolytic activity

    Microsoft Academic Search

    Vladimir I Klichko; James Miller; Aiguo Wu; Serguei G Popov; Ken Alibek

    2003-01-01

    A number of genes in Bacillus anthracis encode for proteins homologous to the membrane-damaging factors known as pathogenic determinants in different bacteria. B. anthracis, however, has been traditionally considered non-hemolytic, and the recently identified hemolytic genes have been suggested to be transcriptionally silent. We found that the hemolytic genes of B. anthracis, collectively designated as anthralysins (Anls), could be induced

  14. Inborn anemias in mice. Progress report, 1 August 1979-15 July 1980

    SciTech Connect

    Bernstein, S.E.; Russell, E.S.

    1980-08-01

    Four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia are under investigation in mice. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values; (b) determinations of radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme synthesis; (d) histological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli; and (g) transplantation of tissue between individuals of differently affected genotypes.

  15. Genetics Home Reference: Anemia

    MedlinePLUS

    ... Home Conditions Genes Chromosomes Handbook Glossary Resources Conditions > Anemia Related topics on Genetics Home Reference: acute promyelocytic ... syndrome beta thalassemia Coats plus syndrome congenital dyserythropoietic anemia Diamond-Blackfan anemia Fanconi anemia Ghosal hematodiaphyseal dysplasia ...

  16. [The importance of antenatal immunoprophylaxis for prevention of hemolytic disease of the fetus and newborn].

    PubMed

    Starcevi?, Mirta; Mataija, Marina; Sovi?, Dragica; Dodig, Javorka; Matijevi?, Ratko; Kukuruzovi?, Monika

    2011-03-01

    Hemolytic disease of the fetus and newborn (HDFN) is a consequence of maternal alloimmunization against fetal red blood cell antigens. Alloimmunization against D antigen from Rhesus (Rh) blood group system is particularly important because of its strong immunogenicity. During the last few decades, the introduction of RhD prophylaxis by postpartum administration of anti-D immunoglobulin to RhD negative women, now improved with antenatal prophylaxis, has led to a dramatic decrease in perinatal mortality and morbidity from HDFN. However, severe cases have not disappeared, mostly due to prophylaxis failure. In our case, inappropriate prenatal care during the first pregnancy in an RhD negative mother resulted in primary immunization. In the next pregnancy with an RhD positive child, the mother's secondary immune response was extremely strong and led to early development of severe fetal anemia. The fetus survived thanks to the treatment with intrauterine transfusions (IUT), but they caused suppression of erythropoiesis, which lasted for months after birth. The long lasting, late anemia was treated with repeated postnatal red cell transfusions and recombinant human erythropoietin (rHuEPO). Despite the severity of HDFN in our case, the short-term outcome is good. The boy has normal growth until now, but due to the possibility of an adverse long-term neurodevelopmental outcome, this case requires continuous follow up. It also reminds of the fact that RhD alloimmunization remains an actual problem in daily routine. Antenatal prophylaxis is a crucial step in quality care of those who are at a risk of HDFN. PMID:21568074

  17. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in relapsing-remitting multiple sclerosis patients on high-dose interferon ?.

    PubMed

    Larochelle, Catherine; Grand'maison, François; Bernier, Gilles P; Latour, Mathieu; Cailhier, Jean-François; Prat, Alexandre

    2014-11-01

    Three women aged 34-47 years old, on high dose interferon beta-1a for relapsing-remitting multiple sclerosis, were hospitalized between 2009-2012 for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Patients sought medical attention for neurological symptoms including cephalalgia, blurred vision, confusion, focal deficits and seizures. All patients presented thrombocytopenia, hemolytic anemia and arterial hypertension. Despite plasma exchanges, corticosteroids and anti-CD20 treatments, all patients progressed towards severe renal insufficiency and one patient died of hemorrhagic shock. In this report we identify a rare but morbid complication of interferon beta-1a treatment associated with female gender, Caucasian background and low body mass index. PMID:24534079

  18. Hemolytic-Uremic Syndrome in childhood.

    PubMed

    Vaisbich, Maria Helena

    2014-01-01

    There is a group of diseases that may manifest with thrombotic microangiopathy and present clinical overlap. Among these we emphasize the thrombotic thrombocytopenic purpura and Hemolytic Uremic Syndrome, and the latter can occur by the action of toxins, systemic diseases, overactivation of the alternative complement system pathway, which can occur due to changes in regulatory proteins (atypical HUS) and finally, idiopathic. You must carry out a series of tests to differentiate them. aHUS is a diagnosis of exclusion of other causes of MAT. The treatment of aHUS with plasma therapy, results in most cases with good shortterm response, especially hematological; however, it is a progressive and devastating disease and can lead to death and terminal chronic renal disease. Treatment with plasma displays great recurrence of long-term disease and renal insufficiency. Eculizumab, a monoclonal antibody anti-C5, has been associated with hematological remission, benefits on renal function and no need of plasma therapy. PMID:25055362

  19. What Causes Aplastic Anemia?

    MedlinePLUS

    ... to aplastic anemia. Examples include Fanconi anemia , Shwachman-Diamond syndrome, dyskeratosis (DIS-ker-ah-TO-sis) congenita, and Diamond-Blackfan anemia. Rate This Content: NEXT >> Featured Video ...

  20. Sickle cell anemia - resources

    MedlinePLUS

    Resources - sickle cell anemia ... The following organizations are good resources for information on sickle cell anemia : American Sickle Cell Anemia Association - www.ascaa.org/ National Heart, Blood, and Lung Institute - www. ...

  1. Anemia of chronic disease

    MedlinePLUS

    Anemia of inflammation; AOCD; ACD ... Anemia is a lower-than-normal number of red blood cells in the blood. Some conditions can lead to anemia of chronic disease include: Autoimmune disorders , such as ...

  2. How Is Anemia Treated?

    MedlinePLUS

    ... page from the NHLBI on Twitter. How Is Anemia Treated? Treatment for anemia depends on the type, cause, and severity of ... is to treat the underlying cause of the anemia. Dietary Changes and Supplements Low levels of vitamins ...

  3. Severe Aplastic Anemia (SAA)

    MedlinePLUS

    ... Email this page Print this page Severe aplastic anemia (SAA) Severe aplastic anemia (SAA) is a disease in which the bone ... make enough blood cells for the body. Aplastic anemia is rare and occurs more frequently in eastern ...

  4. Living with Anemia

    MedlinePLUS

    ... page from the NHLBI on Twitter. Living With Anemia Often, you can treat and control anemia. If ... by an inherited or chronic disease or trauma. Anemia and Children/Teens Infants and young children have ...

  5. Iron deficiency anemia

    MedlinePLUS

    Anemia - iron deficiency ... Iron deficiency anemia is the most common form of anemia. Red blood cells bring oxygen to the ... such as your spleen, remove old blood cells. Iron is a key part of red blood cells. ...

  6. Molecular pathogenesis in Diamond–Blackfan anemia

    Microsoft Academic Search

    Etsuro Ito; Yuki Konno; Tsutomu Toki; Kiminori Terui

    2010-01-01

    Diamond–Blackfan anemia (DBA) is a congenital anemia and a broad spectrum of developmental abnormalities that presents soon\\u000a after birth. The anemia is due to a failure of erythropoiesis with normal platelet and myeloid lineages. Approximately 10–20%\\u000a of DBA cases are inherited. Genetic studies have identified heterozygous mutations in at least one of eight ribosomal protein\\u000a genes in up to 50%

  7. Sexuality and sickle cell anemia

    PubMed Central

    Côbo, Viviane de Almeida; Chapadeiro, Cibele Alves; Ribeiro, João Batista; Moraes-Souza, Helio; Martins, Paulo Roberto Juliano

    2013-01-01

    Background Sickle cell disease, the most common hereditary blood disease in the world, is the result of an atypical hemoglobin called S (Hb S) which, when homozygous (Hb SS) is the cause of sickle cell anemia. Changes of puberty, correlated with a delayed growth spurt, begin late in both male and female sickle cell anemia individuals with repercussions on sexuality and reproduction. The objectives of this exploratory and descriptive study were to characterize the development of sexuality in adults with sickle cell anemia by investigating the patient's perception of their sex life, as well as the information they had and needed on this subject. Methods Twenty male and female sickle cell anemia patients treated at the Hemocentro Regional de Uberaba (UFTM) with ages between 19 and 47 years old were enrolled. A socioeconomic questionnaire and a semi-structured interview on sexuality, reproduction and genetic counseling were applied. Results This study shows that the sickle cell anemia patients lacked information on sexuality especially about the risks of pregnancy and the possible inheritance of the disease by their children. Moreover, the sexual life of the patients was impaired due to pain as well as discrimination and negative feelings experienced in close relationships. Conclusion The health care of sickle cell anemia patients should take into account not only the clinical aspects of the disease, but also psychosocial aspects by providing counseling on sexuality, reproduction and genetics, in order to give this population the possibility of a better quality of life. PMID:23741184

  8. Antibacterial and Hemolytic Activities of Quaternary Pyridinium

    E-print Network

    Antibacterial and Hemolytic Activities of Quaternary Pyridinium Functionalized Polynorbornenesa] They found that polymers with higher cationic functionality had stronger antibacterial activity. Sepa- rately, it was shown that methacrylate based polymers with pendant pyridinium moieties exhibited antibacterial activity

  9. Clostridium septicum infection and hemolytic uremic syndrome.

    PubMed Central

    Barnham, M.; Weightman, N.

    1998-01-01

    Five cases of Clostridium septicum infection secondary to Escherichia coli O157-induced hemolytic uremic syndrome have been reported. We report on three cases (one of which is included in the above five) of dual Cl. septicum and E. coil infection; all three patients were exposed to farm animals. A common zoonotic source for Cl. septicum and E. coli O157 infections should be considered. Patients with hemolytic uremic syndrome should be treated aggressively and monitored closely for Cl. septicum superinfection. PMID:9621207

  10. Inborn anemias in mice: (Annual report, 1982-1983)

    SciTech Connect

    Bernstein, S.E.

    1983-09-09

    The nature of the defects that shorten the effective lifespan of red blood cells in the circulation and which gave rise to anemia, jaundice and to spleen, liver and heart enlargement are studied because they so closely parallel inherited hemolytic anemias in man. In mice, ''hemolytic disease'' initiated by the ja, sph, sph/sup ha/, or the nb genes has been traced to abnormalities in the protein components of their red cell membranes. Polyacrylamide gel electrophoresis of detergent solubilized membranes reveal that in the different genetic types one or more of the major high molecular weight proteins called spectrins is decreased or totally missing. It is one thing to observe a correlation between missing or defective components in selected analytical procedures, and another to establish a causal relationship between the two. To investigate the possible interrelationships, we examined the associations between spectrin or ankyrin content, the severity of the resulting anemia, red cell osmotic fragilities, and the capacity of cells from each genotype to be deformed in a continuous osmotic gradient at constant sheer stress. Our findings indicate that sensitivity to osmotic stress, cell rigidity (inadequate deformability), deficiency of spectrin or ankyrin, and the severity of the anemia, are statistically highly correlated. 11 refs., 3 tabs.

  11. The pathogenesis of Trypanosoma congolense infection in calves. II. Anemia and erythroid response.

    PubMed

    Valli, V E; Forsberg, C M; McSherry, B J

    1978-11-01

    The anemia caused by Trypanosoma conogolense TREU 112 in Holstein calves was of moderate severity and normochromic, macrocytic in the acute phase changing to normochromic, normocytic with chronicity. The anemia was hemolytic and responsive as shown by sharply decreased myeloid:erythroid ratio and increased mean corpuscular volume. 51Cr red cell labelling studies showed that red cell lifespan was halved in the acute phase and there was an increase in plasma volume. Surface organ counting of liver showed it to be the major site of red cell destruction. The anemia was partly dilutional as a result of the increased plasma volume and primarily hemolytic likely of a non-specific immune (innocent bystander) pathogenesis. PMID:751311

  12. Blood . Author manuscript Iron-deficiency anemia from matriptase-2 inactivation is dependent on the

    E-print Network

    Boyer, Edmond

    Blood . Author manuscript Page /1 6 Iron-deficiency anemia from matriptase-2 inactivation (characterized by increased hepcidin levels and anemia) and mice (exhibitingTmprss6 /- - Bmp6 /- - severe iron deficiency anemia are due to excess signaling through the Bmp6/Hjv pathway. MESH Keywords Anemia, Iron

  13. Nitrite-induced anemia in channel catfish, Ictalurus punctatus Rafinesque

    SciTech Connect

    Tucker, C.S. (Mississippi Agricultural and Forestry Experiment Station, Stoneville (USA)); Francis-Floyd, R.; Beleau, M.H. (College of Veterinary Medicine, Stoneville, MS (USA))

    1989-08-01

    Since 1983 numerous cases of anemia have been reported in populations of channel catfish Ictalurus punctatus Rafinesque cultured in the southeastern United States. Environmental nitrite-nitrogen concentrations of 4 mg/L or more occur sporadically in channel catfish culture ponds, and the frequency of occurrence is greatest in the fall and spring. The authors have observed that some cases of anemia in populations of pond-raised channel catfish follow prolonged exposure to high concentrations of environmental nitrite. However, there was no evidence that exposure of channel catfish to environmental nitrite was the cause of the observed anemia. Hemolytic anemia following nitrite exposure has been described for sea bass Dicentrarchus labrax (L.) and rainbow trout Salmo gairdneri, but not for channel catfish. In the present study the authors show that a variable, but generally mild, anemia develops in channel catfish exposed to nitrite. They also offer a management procedure for preventing the development of anemia during periods of elevated environmental nitrite concentrations.

  14. Anemia and Pregnancy

    MedlinePLUS

    ... at the 2014 ASH Annual Meeting Home Anemia & Pregnancy Your body goes through significant changes when you ... risk for becoming anemic. back to top Is Pregnancy-Related Anemia Preventable? Good nutrition is the best ...

  15. Sickle cell anemia

    MedlinePLUS

    Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease ... Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells ...

  16. Anemia in the Newborn

    MedlinePLUS

    ... Need Repeat Surgery: Study Additional Content Medical News Anemia in the Newborn by Arthur E. Kopelman, MD ... Prematurity (ROP) Necrotizing Enterocolitis (NEC) Jaundice in Newborns Anemia in the Newborn Polycythemia in the Newborn Thyroid ...

  17. Living with Fanconi Anemia

    MedlinePLUS

    ... from the NHLBI on Twitter. Living With Fanconi Anemia Improvements in blood and marrow stem cell transplants ... Rate This Content: NEXT >> November 1, 2011 Fanconi Anemia Clinical Trials Clinical trials are research studies that ...

  18. The Anemias of Athletes.

    ERIC Educational Resources Information Center

    Eichner, Edward R.

    1986-01-01

    Diagnosing anemia in athletes is complicated because athletes normally have a pseudoanemia that needs no treatment. Athletes, however, can develop anemia from iron deficiency or footstrike hemolysis, which require diagnosis and treatment. (Author/MT)

  19. What Causes Anemia?

    MedlinePLUS

    ... red blood cells to cause anemia. Lack of Red Blood Cell Production Both acquired and inherited conditions ... also can cause aplastic anemia. High Rates of Red Blood Cell Destruction Both acquired and inherited conditions ...

  20. How Is Anemia Diagnosed?

    MedlinePLUS

    ... page from the NHLBI on Twitter. How Is Anemia Diagnosed? Your doctor will diagnose anemia based on your medical and family histories, a ... exam, and results from tests and procedures. Because anemia doesn't always cause symptoms, your doctor may ...

  1. Mount St. Helens' volcanic ash: hemolytic activity.

    PubMed

    Vallyathan, V; Mentnech, M S; Stettler, L E; Dollberg, D D; Green, F H

    1983-04-01

    Volcanic ash samples from four Mount St. Helens' volcanic eruptions were subjected to mineralogical, analytical, and hemolytic studies in order to evaluate their potential for cytotoxicity and fibrogenicity. Plagioclase minerals constituted the major component of the ash with free crystalline silica concentrations ranging from 1.5 to 7.2%. The in vitro hemolytic activity of the volcanic ash was compared to similar concentrations of cytotoxic and inert minerals. The ash was markedly hemolytic, exhibiting an activity similar to chrysotile asbestos, a known fibrogenic agent. The hemolysis of the different ash samples varied with particle size but not with crystalline silica concentration. The results of these studies taken in conjunction with the results of our animal studies indicate a fibrogenic potential of volcanic ash in heavily exposed humans. PMID:6832120

  2. Group A ?-hemolytic streptococcal pharyngotonsillitis outbreak.

    PubMed

    Culqui, Dante R; Manzanares-Laya, Sandra; Van Der Sluis, Sarah Lafuente; Fanlo, Albert Anton; Comas, Rosa Bartolomé; Rossi, Marcello; Caylá, Joán A

    2014-04-01

    The aim was to describe an outbreak of group A ?-hemolytic streptococcal pharyngotonsillitis in health care professionals. This is a cross-sectional descriptive study of 17 clients who dined at the same table in a restaurant in Barcelona in July 2012. The frequency, timing and severity of symptoms were analyzed, as were demographic variables and others concerning the food ingested. The attack rate was 58.8%. Six of the 10 clients were positive for group A ?-hemolytic streptococcal. Six of the 13 individuals who handled the food involved in the dinner had symptoms. No association was identified with the food consumed. There is epidemiological evidence of foodborne group A ?-hemolytic streptococcal transmission, but respiratory transmission could not be ruled out. PMID:24897054

  3. Group A ?-hemolytic streptococcal pharyngotonsillitis outbreak

    PubMed Central

    Culqui, Dante R; Manzanares-Laya, Sandra; Van Der Sluis, Sarah Lafuente; Fanlo, Albert Anton; Comas, Rosa Bartolomé; Rossi, Marcello; Caylá, Joán A

    2014-01-01

    The aim was to describe an outbreak of group A ?-hemolytic streptococcal pharyngotonsillitis in health care professionals. This is a cross-sectional descriptive study of 17 clients who dined at the same table in a restaurant in Barcelona in July 2012. The frequency, timing and severity of symptoms were analyzed, as were demographic variables and others concerning the food ingested. The attack rate was 58.8%. Six of the 10 clients were positive for group A ?-hemolytic streptococcal. Six of the 13 individuals who handled the food involved in the dinner had symptoms. No association was identified with the food consumed. There is epidemiological evidence of foodborne group A ?-hemolytic streptococcal transmission, but respiratory transmission could not be ruled out. PMID:24897054

  4. Tuning the Hemolytic and Antibacterial Activities of Amphiphilic Polynorbornene Derivatives

    E-print Network

    Tuning the Hemolytic and Antibacterial Activities of Amphiphilic Polynorbornene Derivatives M polydispersities (PDI ) 1.1-1.3). The antibacterial activity determined by growth inhibition assays molecular weight on antibacterial and hemolytic activities were determined. The hydrophobicity of the repeat

  5. Docetaxel-induced thrombotic thrombocytopenic purpura/hemolytic uremic syndrome-related complex in a patient with metastatic prostate cancer?

    PubMed

    Shrestha, Anuj; Khosla, Pam; Wei, Yunfei

    2011-09-01

    A 63-year-old African American man was diagnosed with prostate cancer by biopsy for elevated prostrate-specific antigen. He was initially treated with radiation and then with intermittent androgen ablation because of biochemical relapse. He continued to have rising prostrate-specific antigen and he was thought to have hormone-resistant prostate cancer. So he received chemotherapy with docetaxel. He returned to the hospital within 3 days of the first cycle of treatment with fever, altered mental status, acute renal failure, anemia, and thrombocytopenia. He was started on empiric antibiotics but his cultures from most sites were negative. His platelets dropped from 119,000 to a nadir of 10,000 during hospital stay. Patient had microangiopathic hemolytic anemia suggested by elevated lactate dehydrogenase, decreased haptoglobin, increased indirect bilirubin, and schistocytes in peripheral smear. His coagulation profile was normal. A presumptive diagnosis of chemotherapy-related thrombotic thrombocytopenic purpura (TTP)-hemolytic uremic syndrome was made and patient was started on fresh frozen plasma infusion and hemodialysis for renal failure and steroids. Patient improved symptomatically, platelet count was stable, and lactate dehydrogenase was in a declining trend after 5 days of fresh frozen plasma infusion. The ADAMS TS-13 activity was 37% suggestive of chemotherapy-related TTP. Patient also had sickle cell beta thalassemia disease and glucose 6 phosphate dehydrogenase deficiency. Docetaxel, like some other vascular endothelial growth factor inhibiting chemotherapeutic drugs may cause TTP-hemolytic uremic syndrome. PMID:20592666

  6. Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

    PubMed Central

    Mijatovic, Dino; Blagaic, Ana; Zupan, Zeljko

    2014-01-01

    Introduction: Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF). Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions. PMID:25075296

  7. Passenger Lymphocyte Syndrome: A Forgotten Cause of Postliver Transplant Jaundice and Anemia

    PubMed Central

    Peck, Joshua R.; Elkhammas, Elmahdi A.; Li, Feng; Stanich, Peter P.; Latchana, Nicholas; Black, Sylvester; Michaels, Anthony

    2015-01-01

    A 48-year-old man with cirrhosis secondary to nonalcoholic steatohepatitis and chronic hepatitis C infection underwent a successful orthotopic liver transplant from a B+ donor without intraoperative complications. His postoperative course was complicated by hemolytic anemia, and he was ultimately diagnosed as having passenger lymphocyte syndrome. Passenger lymphocyte syndrome is a complication of both solid-organ and stem cell transplants. It is caused by donor B lymphocyte production of antibodies causing a primary or secondary immune response to recipient erythrocytes. Most commonly, it is in the setting of minor ABO mismatches, such as with a group B liver transplanted into a group AB recipient. Typically, passenger lymphocyte syndrome presents as a mild, self-limiting hemolytic anemia. Laboratory findings are consistent with other forms of hemolytic anemia including decreased hemoglobin and haptoglobin, elevated reticulocyte count, and indirect hyperbilirubinemia There is no definitive treatment for passenger lymphocyte syndrome or strong evidence to favor a particular treatment regimen. Passenger lymphocyte syndrome has been successfully treated with supportive care and blood transfusions matched to the liver donor. It is prudent that physicians caring for patients who receive ABO mismatched organs have a high index of clinical suspicion for passenger lymphocyte syndrome during the early postoperative period when posttransplant patients present with jaundice and anemia. PMID:25077954

  8. Fatal delayed transfusion reaction in a sickle cell anemia patient with Serratia marcescens sepsis.

    PubMed

    Seeyave, Desiree; Desai, Ninad; Miller, Scott; Rao, Sreedhar P; Piecuch, Steve

    2006-10-01

    Patients with sickle cell anemia may require repeated red cell transfusion, putting them at risk for minor blood group alloimmunization and the development of delayed hemolytic transfusion reactions. Although Streptococcus pneumoniae is the most common cause of life-threatening infection in patients with sickle cell anemia, those who have been recently hospitalized are at risk for infection with resistant hospital-associated organisms, and blood transfusion may put the patient at risk of infection with transfusion-associated organisms such as Serratia marcescens and Yersinic enterocolitica. We recently cared for an adolescent with sickle cell anemia who presented to the emergency department with a severe, delayed hemolytic transfusion reaction and Serratia marcescens infection. The patient had been discharged from the hospital five days previously, and had been transfused and treated with antibiotics while hospitalized. In addition to demonstrating the potential severity of delayed hemolytic transfusion reactions, our case illustrates the importance of providing relatively broad-spectrum antibiotic coverage to patients with sickle cell anemia and possible infection who have recently been hospitalized. PMID:17052065

  9. Fatal delayed transfusion reaction in a sickle cell anemia patient with Serratia marcescens sepsis.

    PubMed Central

    Seeyave, Desiree; Desai, Ninad; Miller, Scott; Rao, Sreedhar P.; Piecuch, Steve

    2006-01-01

    Patients with sickle cell anemia may require repeated red cell transfusion, putting them at risk for minor blood group alloimmunization and the development of delayed hemolytic transfusion reactions. Although Streptococcus pneumoniae is the most common cause of life-threatening infection in patients with sickle cell anemia, those who have been recently hospitalized are at risk for infection with resistant hospital-associated organisms, and blood transfusion may put the patient at risk of infection with transfusion-associated organisms such as Serratia marcescens and Yersinic enterocolitica. We recently cared for an adolescent with sickle cell anemia who presented to the emergency department with a severe, delayed hemolytic transfusion reaction and Serratia marcescens infection. The patient had been discharged from the hospital five days previously, and had been transfused and treated with antibiotics while hospitalized. In addition to demonstrating the potential severity of delayed hemolytic transfusion reactions, our case illustrates the importance of providing relatively broad-spectrum antibiotic coverage to patients with sickle cell anemia and possible infection who have recently been hospitalized. PMID:17052065

  10. Inborn anemias in mice: (Annual report, 1980-1981)

    SciTech Connect

    Bernstein, S.E.

    1981-07-02

    The basic purpose of this study is the delineation and exploitation of inborn anemias of the laboratory mouse, carried out by utilization of genetically homogeneous stocks segregating only for anemia-producing genes; by physiological and histological descriptions of each condition at all stages in the life history; by determination of tissue sites of primary gene action through tissue culture studies, tissue transplantation and parabiosis experiments; by analysis of reactions of normal and anemic mice to a variety of stressful stimuli, including x-irradiation, hypoxia, and toxic chemicals, and by biochemical comparisons between tissues, especially erythrocytes and hemopoietic cells of normal vs each type of anemic mouse. At present 16 single-locus anemias are known in the mouse, plus one with multifactorial inheritance (the autoimmune hemolytic anemia of NZB inbred mice). Of these, six are maintained only by the Jackson Laboratory, and two others have but one additional source. Effects of anemia-producing mutant alleles of these loci (an; f; ja; ha; Hba/sup th/; mk; nb; Sl and Sl/sup d/; sla; sph; and W, W/sup v/, W/sup J/ and 10 other putative W-alleles) are currently under investigation at the Jackson Laboratory. 15 refs.

  11. Early Complication in Sickle Cell Anemia Children due to A(TA)nTAA Polymorphism at the Promoter of UGT1A1 Gene

    PubMed Central

    Chaouch, Leila; Talbi, Emna; Moumni, Imen; Ben Chaabene, Arij; Kalai, Miniar; Chaouachi, Dorra; Mallouli, Fethi; Ghanem, Abderraouf; Abbes, Salem

    2013-01-01

    Aim. To determine the implication of the polymorphism, namely, A(TA)nTAA of UGT1A1 in lithogenesis for the first time in Tunisia among sickle cell anemia (SCA) children patients. Material and Methods. Our study was performed in 2010 and it involved 76 subjects chosen as control group characterized with normal hemoglobin status and presence of cholelithiasis and 102 SCA pediatric patients among whom 52 have cholelithiasis. We analyzed the polymorphism A(TA)nTAA at the UGT1A1 promoter and the relationships between the various A(TA)nTAA genotypes and alleles and bilirubin levels and occurrence of cholelithiasis. Results and Discussion. The repartition of genotypes found according to serum bilirubin level shows a significant association between genotypes carrying variant (TA)7 and hyperbilirubinemia (P < 0.05). We demonstrated the association of two genotypes with gallstones formation among SCA children patients: (TA)7/(TA)7 and (TA)7/(TA)8 with P = 8.1 × 10?8 and P = 0.01, respectively. (TA)7 and (TA)8 allele variants act as a risk factor for early gallstones formation in SCA patients with P = 5.8 × 10?9 and P = 0.01, respectively. As for the control group only the genotype (TA)7/(TA)7 presented a risk factor for gallstones formation. Conclusion. The novelty of this report is that it is the first time that a similar study was made on the Tunisian children sickle cell population and that the results show a clear association of (TA)7 variant in early gallstones formation in Tunisian SCA children. Interestingly our findings highlighted the association of (TA)8 variant as well, which was not found in previous studies. PMID:24167350

  12. Early complication in Sickle Cell Anemia children due to A(TA)_n TAA polymorphism at the promoter of UGT1A1 gene.

    PubMed

    Chaouch, Leila; Talbi, Emna; Moumni, Imen; Ben Chaabene, Arij; Kalai, Miniar; Chaouachi, Dorra; Mallouli, Fethi; Ghanem, Abderraouf; Abbes, Salem

    2013-04-25

    AIM: To determine the implication of the polymorphism namely A(TA)nTAA of UGT1A1 in lithogenesis for the first time in Tunisia among sickle cell anemia (SCA) children patients. MATERIAL AND METHODS: Our study was performed in 2010 and it involved 76 subjects chosen as control group characterized with normal hemoglobin status and presence of cholelithiasis and 102 SCA pediatric patients among whom 52 have cholelithiasis. We analyzed the polymorphism A(TA)_{n} TAA at the UGT1A1 promoter and the relationships between the various A(TA)_{n} TAA genotypes and alleles and bilirubin levels and occurrence of cholelithiasis. RESULTS AND DISCUSSION: The repartition of genotypes found according to serum bilirubin level shows a significant association between genotypes carried variant (TA)_{7} and hyperbilirubinemia (p< 0.05). We demonstrated the association of two genotypes with gallstones formation among SCA children patients: (TA)_{7}/(TA)_{7} and (TA)_{7}/(TA)_{8} with p=8.1 × 10^{ - 8} and p=0.01 respectively. (TA)_{7} and (TA)_{8} allele variants act as a risk factor for early gallstones formation in SCA patients with p=5.8 × 10^{ -9} and p=0.01 respectively. As for the control group only the genotype (TA)_{7}/(TA)_{7} presented a risk factor for gallstones formation. CONCLUSION: The novelty of this report is that it is the first time that a similar study was made on the Tunisian children sickle cell population and that the results show a clear association of (TA)_{7} variant in early gallstones formation in Tunisian SCA children. Interestingly our findings highlighted the association of (TA)_{8} variant as well, which was not found in previous studies. PMID:23619273

  13. Heme-regulated eIF2? kinase plays a crucial role in protecting erythroid cells against Pb-induced hemolytic stress.

    PubMed

    Wang, Xiaoyan; Wang, Lixin; Liu, Sijin

    2015-03-16

    Lead (Pb) is a heavy metal with considerable environmental contamination. It is toxic to diverse cells and has been reported to cause a wide array of detrimental health problems including neurological disorders and anemia. In light of the mechanisms underlying Pb-induced anemia, the current understanding is still limited, in spite of efforts for years. Our previous studies recognized a protective role for the heme-regulated eIF2? kinase (Hri) in erythroid cells against oxidative stress exerted by arsenic and cadmium. Whether Hri is involved in Pb-induced hemolytic stress has not been scrutinized. In the current study, to more stringently address this question, we looked into erythropoiesis upon Pb(NO3)2 exposure by using an in vivo mouse model and ex vivo cultured E14.5 fetal liver cells. Diagnoses of hemolytic anemia, decreased red cell count, reduced hemoglobin concentration, and elevated bilirubin level were observed in Hri knockout (Ko) mice only, upon low-dose Pb administration. Significantly different from Ko mice, wild type (Wt) mice did not develop hemolytic anemia. Enforced extramedullary and medullary erythropoieses were found in Ko mice with Pb exposure. However, anemia was not compensated in Hri-deficient mice, as in vivo and ex vivo results manifested that expanded Hri-null erythroid precursors experienced blocked differentiation and enhanced apoptosis, leading to ineffective erythropoiesis under Pb exposure. Additionally, Pb treatment also promoted hepcidin expression and consequentially increased splenic iron storage, resulting in restrained iron availability for erythropoiesis. All considered, Hri-null erythroid precursors were prone to Pb-induced hemolytic stress. Hri deficiency gave rise to ineffective erythropoiesis and reduced iron availability for erythropoiesis under Pb stimulation, and these events together exacerbated Pb-induced hemolytic anemia. It is thus conceivable that this study delineated an indispensable function of Hri in maintaining red cell membrane integrity and guiding erythroid cell differentiation under Pb exposure. Our findings therefore deciphered a crucial role for Hri in protecting erythroid cells against Pb-induced toxicity. PMID:25411909

  14. How Is Pernicious Anemia Treated?

    MedlinePLUS

    ... from the NHLBI on Twitter. How Is Pernicious Anemia Treated? Doctors treat pernicious anemia by replacing the missing vitamin B12 in the body. People who have pernicious anemia may need lifelong treatment. The goals of treating ...

  15. Managing Chemotherapy Side Effects: Anemia

    MedlinePLUS

    ... National Institutes of Health Managing Chemotherapy Side Effects Anemia Call your doctor or nurse if you feel: ? ... tired ? Your heart beating very fast What is anemia? Anemia is when your body doesn’t have ...

  16. Iron deficiency anemia

    PubMed Central

    Naigamwalla, Dinaz Z.; Webb, Jinelle A.; Giger, Urs

    2012-01-01

    Iron is essential to virtually all living organisms and is integral to multiple metabolic functions. The most important function is oxygen transport in hemoglobin. Iron deficiency anemia in dogs and cats is usually caused by chronic blood loss and can be discovered incidentally as animals may have adapted to the anemia. Severe iron deficiency is characterized by a microcytic, hypochromic, potentially severe anemia with a variable regenerative response. Iron metabolism and homeostasis will be reviewed, followed by a discussion of diagnostic testing and therapeutic recommendations for dogs and cats with iron deficiency anemia. PMID:22942439

  17. Fatal hemolysis induced by ceftriaxone in a child with sickle cell anemia

    Microsoft Academic Search

    Juan C. Bernini; Mahmoud M. Mustafa; Laurie J. Sutor; George R. Buchanan

    1995-01-01

    A 2-year-old boy with sickle cell anemia had a massive, fatal hemolytic reaction after administration of an intravenous dose of ceftriaxone. Laboratory studies demonstrated the presence of an IgM antibody against ceftriaxone, binding to and destroying the patient's erythrocytes by an immune complex mechanism. This rare complication should be considered in the differential diagnosis when hemoglobinuria develops in a child

  18. Fifth Cooley's anemia symposium

    SciTech Connect

    Bank, A.; Anderson, W.F.; Zaino, E.C.

    1985-01-01

    This book discusses the topics presented at the symposium on the subject of 'Thalassemia'. Sickle cell anemia is also briefly discussed. The aspects discussed are chromosomal defects of anemias particularly globin synthesis, and the role of messenger RNA and other chromosomes.

  19. Sickle Cell Anemia

    MedlinePLUS

    ... reduce painful crises and episodes of acute chest syndrome for adults and kids with sickle cell. Bone marrow transplant, a complex and risky procedure, is the only cure for sickle cell anemia. Scientists are also studying gene therapy as a treatment for sickle cell anemia. One ...

  20. Reassessment of the microcytic anemia of lead poisoning

    SciTech Connect

    Cohen, A.R.; Trotzky, M.S.; Pincus, D.

    1981-06-01

    Hematologic abnormalities in childhood lead poisoning may be due, in part, to the presence of other disorders, such as iron deficiency or thalassemia minor. In order to reassess increased lead burden as a cause of microcytic anemia, we studied 58 children with class III or IV lead poisoning, normal iron stores, and no inherited hemoglobinopathy. Anemia occurred in 12% and microcytosis in 21% of these children. The combination of anemia and microcytosis was found in only one of 58 patients (2%). When only children with class IV lead poisoning were studied, the occurrence of microcytosis increased to 46%. However, the combination of microcytosis and anemia was found in only one of these 13 more severely affected patients. Microcytic anemia was similarly uncommon in children with either blood lead concentration greater than or equal to 50 microgram/100 ml. These data indicate that microcytosis and anemia occur much less commonly than previously reported in childhood lead poisoning uncomplicated by other hematologic disorders.

  1. Initiation and Regulation of Complement during Hemolytic Transfusion Reactions

    PubMed Central

    Stowell, Sean R.; Winkler, Anne M.; Maier, Cheryl L.; Arthur, C. Maridith; Smith, Nicole H.; Girard-Pierce, Kathryn R.; Cummings, Richard D.; Zimring, James C.; Hendrickson, Jeanne E.

    2012-01-01

    Hemolytic transfusion reactions represent one of the most common causes of transfusion-related mortality. Although many factors influence hemolytic transfusion reactions, complement activation represents one of the most common features associated with fatality. In this paper we will focus on the role of complement in initiating and regulating hemolytic transfusion reactions and will discuss potential strategies aimed at mitigating or favorably modulating complement during incompatible red blood cell transfusions. PMID:23118779

  2. MEGALOBLASTIC AND OTHER MACROCYTIC ANEMIA

    E-print Network

    9/16/2013 1 MEGALOBLASTIC AND OTHER MACROCYTIC ANEMIA MACROCYTOSIS MCV > 100 fL MCHC ­ Normal False) Absorption Transport VITAMIN B 12 DEFICIENCY Pernicious Anemia Shilling Test Other Causes of Malabsorption Oral Parenteral ­ Pernicious Anemia OTHER MEGALOBLASTIC ANEMIAS Drugs Enzyme Deficiencies Congenital

  3. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation

    PubMed Central

    Matsukuma, Eiji; Imamura, Atsushi; Iwata, Yusuke; Takeuchi, Takamasa; Yoshida, Yoko; Fujimura, Yoshihiro; Fan, Xinping; Miyata, Toshiyuki; Kuwahara, Takashi

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underwent surgical repair of tetralogy of Fallot. Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed. Further evaluation revealed that his complement factor H (CFH) level was normal and that anti-FH antibodies were not detected in his plasma. Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal. His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13) activity was also normal. However, he had a potentially causative mutation (R425C) in complement component C3. Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS. We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation. PMID:25431709

  4. Cobalamin C disease presenting as hemolytic-uremic syndrome in the neonatal period.

    PubMed

    Kind, Terry; Levy, Joseph; Lee, Margaret; Kaicker, Shipra; Nicholson, John F; Kane, Steven A

    2002-05-01

    Anew case of cobalamin C disease associated with hemolytic-uremic syndrome (HUS) in the neonatal period is described. A 28-day-old boy presented with failure to thrive, hypotonia, pancytopenia, and features of HUS (microangiopathic hemolytic anemia, thrombocytopenia, and renal failure). The possibility of the diagnosis of an underlying vitamin B12 disorder was prompted by evidence of megaloblastic changes on the peripheral smear and by finding in the literature a suggested association of neonatal HUS with this cobalamin-related metabolic disorder. Amino acid analysis showed elevated homocysteine levels in the plasma and increased levels of both homocysteine and methyl malonic acid in the urine. Diagnosis of cobalamin C disease was confirmed by complementation studies using skin fibroblasts. Therapy included parenteral hydroxocobalamin, carnitine, and leucovorin calcium (folinic acid). Cobalamin C disease should be considered in the diagnosis of patients presenting with HUS in infancy who have unexplained megaloblastosis, pancytopenia, neurologic impairment, and failure to thrive. Early diagnosis and institution of therapy may be effective in improving survival and quality of life. PMID:11972107

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

    PubMed Central

    Bu, Fengxiao; Borsa, Nicolo; Gianluigi, Ardissino; Smith, Richard J. H.

    2012-01-01

    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. PMID:23251215

  6. Diagnosis of Fanconi anemia in patients with bone marrow failure

    PubMed Central

    Pinto, Fernando O.; Leblanc, Thierry; Chamousset, Delphine; Le Roux, Gwenaelle; Brethon, Benoit; Cassinat, Bruno; Larghero, Jérôme; de Villartay, Jean-Pierre; Stoppa-Lyonnet, Dominique; Baruchel, André; Socié, Gérard; Gluckman, Eliane; Soulier, Jean

    2009-01-01

    Background Patients with bone marrow failure and undiagnosed underlying Fanconi anemia may experience major toxicity if given standard-dose conditioning regimens for hematopoietic stem cell transplant. Due to clinical variability and/or potential emergence of genetic reversion with hematopoietic somatic mosaicism, a straightforward Fanconi anemia diagnosis can be difficult to make, and diagnostic strategies combining different assays in addition to classical breakage tests in blood may be needed. Design and Methods We evaluated Fanconi anemia diagnosis on blood lymphocytes and skin fibroblasts from a cohort of 87 bone marrow failure patients (55 children and 32 adults) with no obvious full clinical picture of Fanconi anemia, by performing a combination of chromosomal breakage tests, FANCD2-monoubiquitination assays, a new flow cytometry-based mitomycin C sensitivity test in fibroblasts, and, when Fanconi anemia was diagnosed, complementation group and mutation analyses. The mitomycin C sensitivity test in fibroblasts was validated on control Fanconi anemia and non-Fanconi anemia samples, including other chromosomal instability disorders. Results When this diagnosis strategy was applied to the cohort of bone marrow failure patients, 7 Fanconi anemia patients were found (3 children and 4 adults). Classical chromosomal breakage tests in blood detected 4, but analyses on fibroblasts were necessary to diagnose 3 more patients with hematopoietic somatic mosaicism. Importantly, Fanconi anemia was excluded in all the other patients who were fully evaluated. Conclusions In this large cohort of patients with bone marrow failure our results confirmed that when any clinical/biological suspicion of Fanconi anemia remains after chromosome breakage tests in blood, based on physical examination, history or inconclusive results, then further evaluation including fibroblast analysis should be made. For that purpose, the flow-based mitomycin C sensitivity test here described proved to be a reliable alternative method to evaluate Fanconi anemia phenotype in fibroblasts. This global strategy allowed early and accurate confirmation or rejection of Fanconi anemia diagnosis with immediate clinical impact for those who underwent hematopoietic stem cell transplant. PMID:19278965

  7. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe

    PubMed Central

    Nouraie, Mehdi; Lee, Janet S.; Zhang, Yingze; Kanias, Tamir; Zhao, Xuejun; Xiong, Zeyu; Oriss, Timothy B.; Zeng, Qilu; Kato, Gregory J.; Gibbs, J. Simon R.; Hildesheim, Mariana E.; Sachdev, Vandana; Barst, Robyn J.; Machado, Roberto F.; Hassell, Kathryn L.; Little, Jane A.; Schraufnagel, Dean E.; Krishnamurti, Lakshmanan; Novelli, Enrico; Girgis, Reda E.; Morris, Claudia R.; Rosenzweig, Erika Berman; Badesch, David B.; Lanzkron, Sophie; Castro, Oswaldo L.; Goldsmith, Jonathan C.; Gordeuk, Victor R.; Gladwin, Mark T.

    2013-01-01

    The intensity of hemolytic anemia has been proposed as an independent risk factor for the development of certain clinical complications of sickle cell disease, such as pulmonary hypertension, hypoxemia and cutaneous leg ulceration. A composite variable derived from several individual markers of hemolysis could facilitate studies of the underlying mechanisms of hemolysis. In this study, we assessed the association of hemolysis with outcomes in sickle cell anemia. A hemolytic component was calculated by principal component analysis from reticulocyte count, serum lactate dehydrogenase, aspartate aminotransferase and total bilirubin concentrations in 415 hemoglobin SS patients. Association of this component with direct markers of hemolysis and clinical outcomes was assessed. As primary validation, both plasma red blood cell microparticles and cell-free hemoglobin concentration were higher in the highest hemolytic component quartile compared to the lowest quartile (P?0.0001 for both analyses). The hemolytic component was lower with hydroxyurea therapy, higher hemoglobin F, and alpha-thalassemia (P?0.0005); it was higher with higher systemic pulse pressure, lower oxygen saturation, and greater values for tricuspid regurgitation velocity, left ventricular diastolic dimension and left ventricular mass (all P<0.0001). Two-year follow-up analysis showed that a high hemolytic component was associated with an increased risk of death (hazard ratio, HR 3.44; 95% confidence interval, CI: 1.2–9.5; P=0.02). The hemolytic component reflects direct markers of intravascular hemolysis in patients with sickle cell disease and allows for adjusted analysis of associations between hemolytic severity and clinical outcomes. These results confirm associations between hemolytic rate and pulse pressure, oxygen saturation, increases in Doppler-estimated pulmonary systolic pressures and mortality (Clinicaltrials.gov identifier: NCT00492531). PMID:22983573

  8. Your Guide to Anemia

    MedlinePLUS

    ... for rheumatoid arthritis l Autoimmune disorders (e.g., lupus) l Pregnancy l Fanconi anemia l Shwachman-Diamond ... inherited condition. Certain diseases or infections, such as lupus or hepatitis, are exam ples of acquired conditions ...

  9. Iron-Deficiency Anemia

    MedlinePLUS Videos and Cool Tools

    ... blood has a lower than normal number of red blood cells. Red blood cells carry oxygen and remove carbon dioxide ( ... your body. Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE- ...

  10. Anemia mexicana (Native) 

    E-print Network

    Robert W. Corbett

    2011-08-02

    A STUDY OF THE PATHOGENESIS OF EQUINE INFECTIOUS ANEMIA BY FLUORESCENT ANTIBODY TECHNIQUES A Thesis By DAVID EUGENE MOREMAN Submitted to the Graduate College of the Texas A6 M University in partial fulfillment of the requirements... for the degree of MASTER OF SCIENCE January 1968 Major Subject: Veterinary Microbiology A STUDY OF THE PATHOGENESIS OF EQUINE INFECTIOUS ANEMIA BY FLUORESCENT ANTIBODY TECHNIQUES A Thesis By DAVID EUGENE MOREMAN Approved as to style and content by: , M...

  11. Hemolytic activity of five different calcium silicates.

    PubMed Central

    Skaug, V; Gylseth, B

    1983-01-01

    Mineral characteristics and the in vitro hemolytic activity of three synthetic and two natural calcium silicates (CaSi) are compared. Hemolysis is higher for the synthetic compounds than for the natural ones. The difference is accentuated by weak ultrasonication of the minerals. No variation was observed within the two groups, including both acicular and fibrous forms. Calcium was released from the minerals during storage in Tris-buffered saline. At the same time, hemolysis decreased, and crystallographic alterations occurred in the leached minerals. Treatment of the CaSi with calcium chelators (EGTA and EDTA) did not change hemolytic activity. An increase was observed when 30 mM calcium was added. Hemolysis is related to specific surface areas and the crystalline structure of the minerals. Calcium may also be a contributing factor. Images FIGURE 1. a FIGURE 1. b FIGURE 1. c FIGURE 1. d FIGURE 1. e FIGURE 1. f FIGURE 7. a FIGURE 7. b FIGURE 7. c FIGURE 7. d FIGURE 7. e FIGURE 7. f PMID:6315361

  12. Treatment of iron deficiency anemia with Ferro-Folgamma.

    PubMed

    Ghinea, Mihaela Maria

    2004-01-01

    Iron deficiency anemia is a hypochromic anemia in which hemoglobin poor synthesis is due to a decrease in the amount of iron in the body. The decrease of iron quantity has many causes: insufficient intake of aliments rich in iron (meat, viscera, green vegetables), increased necessities during growth period, pregnancy, erythrocytes hyperregeneration, high-performance sportsmen, increased loss by digestive way, genito-urinary way, respiratory, hemorrhagic syndromes. Clinically, symptoms and signs specific to all types of anemia and those specific to lack of iron occur besides the symptoms and signs of the underlying disease: atrophic glositis, angular stomatitis, sideropenic dysphagia, pica, skin and nails changes. Laboratory investigations useful for diagnosis are: microcytic, hypochromic anemia, decreased serum iron level, total capacity of iron binding increased, medullar iron store absent, good response to iron therapy. Ferro-Folgamma is one of the most indicated medicines in iron deficiency anemia. Due to its components this medicine has many indications: insufficient alimentary intake concerning iron, folic acid, B12 vitamin, vegetarian alimentation, increased needs during growth period, iron deficiency anaemia secondary to chronic hemorrhages, malnutrition, anemias associated with chronic alcohol intake, preventive treatment of iron deficiency anemia and megaloblastic anemia during pregnancy and lactation. PMID:15529613

  13. How Is Iron-Deficiency Anemia Treated?

    MedlinePLUS

    ... the NHLBI on Twitter. How Is Iron-Deficiency Anemia Treated? Treatment for iron-deficiency anemia will depend ... may be advised. Treatments for Severe Iron-Deficiency Anemia Blood Transfusion If your iron-deficiency anemia is ...

  14. Anemia, tumor hypoxemia, and the cancer patient

    SciTech Connect

    Varlotto, John [Department of Radiation Oncology, Boston VA Medical Center, Boston, MA (United States) and Department of Radiation Oncology, Beth (Israel) and Deaconess Medical Center, Harvard Medical School, Boston, MA (United States)]. E-mail: jvarlott@bidmc.harvard.edu; Stevenson, Mary Ann [Department of Radiation Oncology, Boston VA Medical Center, Boston, MA (United States); Department of Radiation Oncology, Beth Israel/Deaconess Medical Center, Harvard Medical School, Boston, MA (United States)

    2005-09-01

    Purpose: To review the impact of anemia/tumor hypoxemia on the quality of life and survival in cancer patients, and to assess the problems associated with the correction of this difficulty. Methods: MEDLINE searches were performed to find relevant literature regarding anemia and/or tumor hypoxia in cancer patients. Articles were evaluated in order to assess the epidemiology, adverse patient effects, anemia correction guidelines, and mechanisms of hypoxia-induced cancer cell growth and/or therapeutic resistance. Past and current clinical studies of radiosensitization via tumor oxygenation/hypoxic cell sensitization were reviewed. All clinical studies using multi-variate analysis were analyzed to show whether or not anemia and/or tumor hypoxemia affected tumor control and patient survival. Articles dealing with the correction of anemia via transfusion and/or erythropoietin were reviewed in order to show the impact of the rectification on the quality of life and survival of cancer patients. Results: Approximately 40-64% of patients presenting for cancer therapy are anemic. The rate of anemia rises with the use of chemotherapy, radiotherapy, and hormonal therapy for prostate cancer. Anemia is associated with reductions both in quality of life and survival. Tumor hypoxemia has been hypothesized to lead to tumor growth and resistance to therapy because it leads to angiogenesis, genetic mutations, resistance to apoptosis, and a resistance to free radicals from chemotherapy and radiotherapy. Nineteen clinical studies of anemia and eight clinical studies of tumor hypoxemia were found that used multi-variate analysis to determine the effect of these conditions on the local control and/or survival of cancer patients. Despite differing definitions of anemia and hypoxemia, all studies have shown a correlation between low hemoglobin levels and/or higher amounts of tumor hypoxia with poorer prognosis. Radiosensitization through improvements in tumor oxygenation/hypoxic cell sensitization has met with limited success via the use of hyperbaric oxygen, electron-affinic radiosensitizers, and mitomycin. Improvements in tumor oxygenation via the use of carbogen and nicotinamide, RSR13, and tirapazamine have shown promising clinical results and are all currently being tested in Phase III trials. The National Comprehensive Cancer Network (NCCN) guidelines recommend transfusion or erythropoietin for symptomatic patients with a hemoglobin of 10-11 g/dl and state that erythropoietin should strongly be considered if hemoglobin falls to less than 10 g/dl. These recommendations were based on studies that revealed an improvement in the quality of life of cancer patients, but not patient survival with anemia correction. Phase III studies evaluating the correction of anemia via erythropoietin have shown mixed results with some studies reporting a decrease in patient survival despite an improvement in hemoglobin levels. Diverse functions of erythropoietin are reviewed, including its potential to inhibit apoptosis via the JAK2/STAT5/BCL-X pathway. Correction of anemia by the use of blood transfusions has also shown a decrement in patient survival, possibly through inflammatory and/or immunosuppressive pathways. Conclusions: Anemia is a prevalent condition associated with cancer and its therapies. Proper Phase III trials are necessary to find the best way to correct anemia for specific patients. Future studies of erythropoietin must evaluate the possible anti-apoptotic effects by directly assessing the tumor for erythropoietin receptors or the presence of the JAK2/STAT5/BCL-X pathway. Due to the ability of transfusions to cause immunosuppression, most probably through inflammatory pathways, it may be best to study the effects of transfusion with the prolonged use of anti-inflammatory medications.

  15. Hemolytic Uremic Syndrome following Infection with O111 Shiga Toxin-Producing Escherichia coli Revealed through Molecular Diagnostics

    PubMed Central

    Operario, Darwin J.; Moonah, Shannon

    2014-01-01

    We report a case of hemolytic uremic syndrome in a 69-year-old woman due to Shiga toxin-producing Escherichia coli, possibly serotype O111, to illustrate the potentially deleterious implications of a Campylobacter enzyme immunoassay (EIA) result and the increasing importance of molecular testing when conventional methods are limited. PMID:24371241

  16. Facts about Diamond Blackfan Anemia

    MedlinePLUS

    ... Form Controls NCBDDD Cancel Submit Search The CDC Diamond Blackfan Anemia (DBA) Note: Javascript is disabled or ... Español (Spanish) Recommend on Facebook Tweet Share Compartir Diamond Blackfan anemia (DBA) is a rare blood disorder ...

  17. Zinc deficiency anemia and effects of zinc therapy in maintenance hemodialysis patients.

    PubMed

    Fukushima, Tatsuo; Horike, Hideyuki; Fujiki, Shigeatsu; Kitada, Shingo; Sasaki, Tamaki; Kashihara, Naoki

    2009-06-01

    Quantitative adjuvant zinc therapy using polaprezinc was performed to examine the correlation between zinc concentration and anemia in maintenance hemodialysis patients to propose appropriate treatment. Anemia and serum zinc concentration were measured in 117 patients with chronic renal failure receiving outpatient maintenance hemodialysis at Tsuyama Chuo Kinen Hospital. Two bags of polaprezinc (containing zinc 34 mg/day) were administered to 58 patients with lower than normal zinc levels (Zn < 80 mg/dl) as adjuvant zinc therapy to assess anemia improvement. Zinc concentration and all anemia parameters showed significant positive correlation, indicating that anemia improves in patients with high serum zinc levels. Regarding the effects of adjuvant zinc therapy for improving anemia, hemoglobin levels were found to increase significantly to the highest value at 3 weeks. During treatment, the dosage of erythropoietin was reduced significantly from baseline at all assessment points. No zinc poisoning from therapy was seen, but two patients had diarrhea (1.9%). Zinc-treated patients required iron therapy due to the development of iron deficiency. Most maintenance hemodialysis patients suffer from zinc deficiency anemia, and zinc-based polaprezinc has been confirmed to be an effective and safe adjuvant zinc treatment. Most patients diagnosed as refractory anemia with no response to erythropoietin also suffer from zinc deficiency anemia, many of whom are expected to benefit from zinc therapy to improve their anemia. Possible zinc deficiency anemia should be considered in the treatment of refractory anemia with no response to erythropoietin. PMID:19527468

  18. How Is Fanconi Anemia Treated?

    MedlinePLUS

    ... from the NHLBI on Twitter. How Is Fanconi Anemia Treated? Doctors decide how to treat Fanconi anemia (FA) based on a person's age and how ... Long-term treatments for FA can: Cure the anemia. Damaged bone marrow cells are replaced with healthy ...

  19. How Is Fanconi Anemia Diagnosed?

    MedlinePLUS

    ... from the NHLBI on Twitter. How Is Fanconi Anemia Diagnosed? People who have Fanconi anemia (FA) are born with the disorder. They may ... questions about: Any personal or family history of anemia Any surgeries you’ve had related to the ...

  20. How Is Aplastic Anemia Diagnosed?

    MedlinePLUS

    ... from the NHLBI on Twitter. How Is Aplastic Anemia Diagnosed? Your doctor will diagnose aplastic anemia based on your medical and family histories, a ... your primary care doctor thinks you have aplastic anemia, he or she may refer you to a ...

  1. Hemoglobin C, S-C, and E Diseases

    MedlinePLUS

    ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ...

  2. Reticulocytopenia in severe autoimmune hemolytic anemia (AIHA) of the warm antibody type.

    PubMed

    Hauke, G; Fauser, A A; Weber, S; Maas, D

    1983-06-01

    A patient with severe AIHA of the warm antibody type, absence of reticulocytes and red cell hyperplasia of the bone marrow is described. In order to maintain a reasonable hemoglobin level 38 units of washed packed red cells were required within 24 days. The treatment with high doses of steroids showed no permanent beneficial effect. After splenectomy the red cell destruction was immediately reduced and the patient went into a remission. Bone marrow culture studies during the acute phase of the disease and at the time of complete hemato- and immunological remission, i.e. 4 months after splenectomy suggested a circulating autoantibody directed to early erythroid progenitors (BFU-E). The inhibitory activity in the patient's plasma did not influence granulocytic or mixed colony formation (CFU-GEMM). In addition to autoantibodies directed to erythroblasts and erythropoietin involved in the pathogenic mechanisms leading to red cell aplasia type I and II the culture studies suggest an unusual autoantibody that might cause the observed reticulocytopenia and erythropoietic hyperplasia of the bone marrow in AIHA. After the splenectomy the patient recovered, he required no further blood transfusions and his disease has not recurred. PMID:6850101

  3. Lethal autoimmune hemolytic anemia in CD47-deficient nonobese diabetic (NOD) mice

    Microsoft Academic Search

    Per-Arne Oldenborg; Hattie D. Gresham; Yongmei Chen; Shozo Izui; Frederik P. Lindberg

    2002-01-01

    The glycoprotein CD47 (integrin-associ- ated protein, IAP) is present on the sur- face of virtually all cells, including red blood cells (RBCs). CD47 acts like a marker of self by ligating the macrophage inhibitory receptor signal regulatory pro- tein (SIRP). In this manner mild reactiv- ity of wild-type RBCs with macrophage phagocytic receptors is tolerated, whereas otherwise identical CD47-deficient RBCs

  4. Acute methemoglobinemia with hemolytic anemia following bio-organic plant nutrient compound exposure: Two case reports.

    PubMed

    Malkarnekar, Santoshi Balkrishna; Anjanappa, Raveesha; Naveen, L; Kiran, B G

    2014-02-01

    Two young women, were reffered to our hospital on two different occasions with history of breathlessness and mental confusion, following consumption of two different bio-organic plant nutrient compounds with a suicidal intent. On examination, they had cyanotic mucous membranes, and their blood samples showed the classic 'dark chocolate brown' appearance. Work up revealed cyanosis unresponsive to oxygen supplementation and absence of cardiopulmonary abnormality. Pulse oximetry revealed saturation of 75% in case 1 and 80% in case 2, on 8 liters oxygen supplementation via face masks, although their arterial blood gas analysis was normal, suggestive of "saturation gap". Methemoglobinemia was suspected based on these findings and was confirmed by Carbon monoxide-oximetry (CO-oximetry). Methylene blue was administered and the patients showed dramatic improvement. Both the patients developed evidence of hemolysis approximately 72 hours following admission which improved with blood transfusion and supportive treatment. The patients were eventually discharged without any neurological sequalae. PMID:24678158

  5. A life-threatening case of autoimmune hemolytic anemia successfully treated by plasma-exchange.

    PubMed

    Cerdas-Quesada, César

    2010-06-01

    A case of severe AIHA caused by pan-agglutinant IgG-class antibodies was resolved with therapeutic plasma exchange, transfusions and steroids to maintain acceptable hemoglobin levels, remove free hemoglobin, reduce the title of autoantibodies and sustain cardiopulmonary functions. PMID:20371214

  6. Inborn anemias in mice. Comprehensive progress report, 1 August 1979-1 June 1982, to accompany twenty-seventh renewal proposal

    SciTech Connect

    Bernstein, S.E.; Russell, E.S.; Barker, J.E.

    1982-07-01

    Hereditary anemias of mice have been investigated including four macrocytic anemias, three hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules controlling a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse and by extension to man from an understanding of mammalian mechanisms utilized in the control of erythropoiesis. Each of the different anemias is studied through: (a) biochemical and biophysical characterization of peripheral blood cells; (b) determinations of cellular and organismic radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme biosynthesis; (d) morphological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli and inhibitors; and (g) physiological complementation analysis via transplantation of tissue between individuals of differently affected genotypes.

  7. Helicobacter pylori pore-forming cytolysin orthologue TlyA possesses in vitro hemolytic activity and has a role in colonization of the gastric mucosa.

    PubMed

    Martino, M C; Stabler, R A; Zhang, Z W; Farthing, M J; Wren, B W; Dorrell, N

    2001-03-01

    Hemolysins have been found to possess a variety of functions in bacteria, including a role in virulence. Helicobacter pylori demonstrates hemolytic activity when cultured on unlysed blood agar plates which is increased under iron-limiting conditions. However, the role of an H. pylori hemolysin in virulence is unclear. Scrutiny of the H. pylori 26695 genome sequence suggests the presence of at least two distinct hemolysins, HP1086 and HP1490, in this strain. Previous studies have shown that the in vitro hemolytic activity of H. pylori is reduced when it is coincubated with dextran 5000, suggesting the presence of a pore-forming cytolysin. HP1086 has homology to pore-forming cytolysins (TlyA) from other bacterial species, and the introduction of the cloned H. pylori tlyA gene into a nonhemolytic Escherichia coli strain conferred hemolytic activity. An H. pylori tlyA defined mutant showed reduced in vitro hemolytic activity, which appears to be due to pore formation, as the hemolytic activity of the wild-type strain is reduced to the same level as the tlyA mutant by the addition of dextran 5000. The mutant also showed reduced adhesion to human gastric adenocarcinoma cells and failed to colonize the gastric mucosa of mice. These data clearly suggest a role in virulence for H. pylori TlyA, contrary to the suggestion that hemolytic activity is an in vitro phenomenon for this pathogen. PMID:11179345

  8. [Study on hemolytic activity of Chattonella marina Hong Kong strain].

    PubMed

    Jiang, Tao; Wang, Rui; Wu, Ni; Jiang, Tian-Jiu

    2011-10-01

    Hemolytic activity of Chattonella marina Hong Kong strain (CMHK) in different growth phase and nutrients structure was studied under laboratory conditions. According to the growth curve of CMHK, cells in culture were collected in logarithmic phase, stationary phase and decline phase, respectively, to determine hemolytic activity. Hemolytic activity of CHHK, which was cultured in different nutrients structure, was also determined. Furthermore, acute toxicity test of CMHK on Artemia salina was performed in early logarithmic phase (5 d), late logarithmic phase (13 d), stationary phase (15 d) and decline phase (17 d). The results showed that the highest hemolytic activity was detected in early logarithmic phase. After 5 d culture, the hemolytic activity reached 1.80 x 10(-7) HU/cell, then decreased gradually with culture time increasing. Hemolytic activity of total cells in culture in different growth phase coincided with the growth curve with the maximum reaching 5.32 HU/L in late logarithmic phase. Growth rate of CMHK was greatly depressed by Fe limitation while hemolytic activity per cell was enhanced to 8.37 x 10(-7) HU/cell, nearly 6.5 times higher than that with no Fe limitation (1.33 x 10(-7)HU/cell). Similarly, N, Mn and P limitation could also increase the hemolytic activity of CMHK. Acute toxicity test of CMHK on A. salina showed that the maximum toxicity of the algae appeared at late logarithmic phase. It was found that death rate of A. sinica reached to 77% in 48 h when exposed to the algal culture at late logarithmic phase with algae density being 4.0 x 10(4) cells/mL, and the lowest toxicity on A. sinica appeared in early logarithmic phase with death rate of 20%. PMID:22279902

  9. Anemia and School Participation

    ERIC Educational Resources Information Center

    Bobonis, Gustavo J.; Miguel, Edward; Puri-Sharma, Charu

    2006-01-01

    Anemia is among the most widespread health problems for children in developing countries. This paper evaluates the impact of a randomized health intervention delivering iron supplementation and deworming drugs to Indian preschool children. At baseline, 69 percent were anemic and 30 percent had intestinal worm infections. Weight increased among…

  10. Sickle Cell Anemia Bibliography.

    ERIC Educational Resources Information Center

    Christy, Steven C.

    Presents sources for the acquisition of medical, social, psychological, educational, and practical knowledge of sickle cell anemia. The materials listed are designed to help parents, educators, and public service workers. Materials include journal articles, films, brochures, slides, and fact sheets. The usual bibliographic information is given.…

  11. Hemolytic uremic syndrome - clinical aspects and outcome of an outbreak: Report of 28 cases.

    PubMed

    Elzouki, A Y; Mirza, K; Mahmood, A; Al-Sowailem, A M

    1995-03-01

    Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. There are two main subgroups: the typical form of HUS follows a diarrheal prodrome (D+HUS) and the atypical form is without the diarrheal prodrome (D-HUS). We have studied 28 children with HUS over a period of 15 months between 1992 and 1993. The median age was 2.2 years (range from six months to six years). All children had prodromal diarrhea. Hypertension was present in 71% and neurological complications in 39%. All the patients had oliguria or anuria (16 oliguric and 12 anuric). The mean duration of anuria was 16 days (range seven to 42 days). Serum creatinine on admission ranged between 112 and 1064 Amicromol/L (mean 453 Amicromol/L). The lowest hemoglobin level and platelet count during hospitalization ranged between 38 and 87 g/L and 7 to 147x109/L respectively. Leukocytosis on admission was present in 22 patients, low C3 was documented in 11 patients (34%), and four patients had low C4. All patients received fresh frozen plasma transfusion, a total of 25 patients received dialysis therapy, 19 patients were treated with peritoneal dialysis (PD), one patient had hemodialysis (HD), and five patients had both HD and PD. The mean duration of dialysis was 18 days (range three to 56 days). Only one patient died (mortality rate 3%). The median duration of hospital stay was 28 days (range eight to 90 days). We conclude that HUS is emerging as an important clinical and public health problem and that early comprehensive management including dialysis therapy, aggressive management of hypertension, fresh frozen plasma transfusion, and nutritional support all improve the outcome and decrease the mortality and morbidity in patients with HUS. PMID:17587918

  12. Atypical hemolytic uremic syndrome diagnosed four years after ABO-incompatible kidney transplantation.

    PubMed

    Kawaguchi, Keiko; Kawanishi, Kunio; Sato, Masayo; Itabashi, Mitsuyo; Fujii, Akiko; Kanetsuna, Yukiko; Huchinoue, Shouhei; Ohashi, Ryuji; Koike, Junki; Honda, Kazuho; Nagashima, Yoji; Nitta, Kosaku

    2015-07-01

    Atypical hemolytic uremic syndrome (aHUS) in allograft kidney transplantation is caused by various factors including rejection, infection, and immunosuppressive drugs. We present a case of a 32 year old woman with aHUS four years after an ABO-incompatible kidney transplantation from a living relative. The primary cause of end-stage renal disease was unknown; however, IgA nephropathy (IgAN) was suspected from her clinical course. She underwent pre-emptive kidney transplantation from her 60 year old mother. The allograft preserved good renal function [serum creatinine (sCr) level 110-130??mol/L] until a sudden attack of abdominal pain four years after transplant, with acute renal failure (sCr level, 385.3??mol/L), decreasing platelet count, and hemolytic anemia with schizocytes. On allograft biopsy, there was thrombotic microangiopathy in the glomeruli, with a cellular crescent formation and mesangial IgA and C3 deposition. Microvascular inflammation, such as glomerulitis, peritubular capillaritis, and arteriole endarteritis were also detected. A disintegrin-like and metalloproteinase with thrombospondin type 1 motifs 13 (ADAMTS13) did not decrease and Shiga toxin was not detected. Donor-specific antibodies or autoantibodies, including anti-neutrophil cytoplasmic antibody and anti-glomerular basement membrane (anti-GBM) antibody, were negative. The patient was diagnosed with aHUS and received three sessions of plasmapheresis and methylprednisolone pulse therapy, followed by oral methylprednisolone (0.25-0.5?mg/kg) instead of tacrolimus. She temporarily required hemodialysis (sCr level, 658.3??mol/L). Thereafter, her sCr level improved to 284.5??mol/L without dialysis therapy. This case is clinically considered as aHUS after kidney transplantation, associated with various factors, including rejection, glomerulonephritis, and toxicity from drugs such as tacrolimus. PMID:26031589

  13. 21 CFR 866.5490 - Hemopexin immunological test system.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...of various hematologic disorders, such as hemolytic anemia (anemia due to shortened in vivo survival of mature red blood...compensate for their decreased life span) and sickle cell anemia. (b) Classification. Class II (special...

  14. 21 CFR 866.5490 - Hemopexin immunological test system.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...of various hematologic disorders, such as hemolytic anemia (anemia due to shortened in vivo survival of mature red blood...compensate for their decreased life span) and sickle cell anemia. (b) Classification. Class II (special...

  15. 21 CFR 866.5490 - Hemopexin immunological test system.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...of various hematologic disorders, such as hemolytic anemia (anemia due to shortened in vivo survival of mature red blood...compensate for their decreased life span) and sickle cell anemia. (b) Classification. Class II (special...

  16. Inborn anemias in mice: (Annual report, 1983-1984)

    SciTech Connect

    Bernstein, S.E.

    1984-09-01

    The hypotranserrinemic-hemochromatosis mutation in mice discovered in our laboratory is an almost exact duplicate of human atransferrinemia. Just as in man, the condition is inherited as a recessive lethal. The disease appears to stem from a congenital deficiency in transferrin. The new mutation arose spontaneously in BALB/c mice and results in death before 12 days of age. It is characterized by stunted growth, low numbers of erythrocytes, hypochromia, and in the absence of jaundice. Treatments with Imferon or other iron preparations were uniformly unsuccessful, but the use of normal mouse serum proved successful as a therapeutic measure. We find that we are able to keep these afflicted mice alive for more than a year with small amounts of normal serum, and transferrin bands are missing on cellulose acetate electrophoresis of serum proteins from affected individuals receiving no treatment. Genetic tests indicated that the new mutation was not an allele of any of the other known iron deficiency anemias in the mouse: sex linked anemia (sla), microcytic anemia (mk), or flexed anemia (f) or any of the members of the hemolytic disease group (sph, sph/sup ha/, nb, or ja). Biochemical and genetic analyses carried out during the past year indicate that the new mutation, tentatively designated hpx is not likely to be a mutation at the transferrin (Trf) locus on Chromosome 9. We observed no unusual serum proteins on cellulose acetate electrophoresis, such as might be expected if the Trf gene had mutated. Moreover, radial immunodiffusion examination and Ouchterlony analysis did not show the presence of smaller molecules (or fragments) with transferrin antigenic specificities. Instead they showed a total loss in serum transferrin. 14 refs., 5 tabs.

  17. ANEMIA OF DISORDERED IRON METABOLISM AND HEME

    E-print Network

    9/16/2013 1 ANEMIA OF DISORDERED IRON METABOLISM AND HEME SYNTHESIS Defect in Heme Synthesis Defect Deficiency Anemia Sideroachristic ­ adequate iron but defective utilization Sideroblastic Anemia Anemia;9/16/2013 4 IRON DEFICIENCY ANEMIA CAUSES Dietary Deficiency Blood Loss Hemodialysis Malabsorption IDA

  18. Hemolytic disease of the newborn caused by a new deletion of the entire beta-globin cluster.

    PubMed Central

    Pirastu, M; Kan, Y W; Lin, C C; Baine, R M; Holbrook, C T

    1983-01-01

    We describe a new type of gamma delta beta-thalassemia in four generations of a family of Scotch-Irish descent. The proposita presented with hemolytic disease of the newborn, which was characterized by a microcytic anemia. Initial restriction endonuclease analysis of the DNA showed no grossly abnormal patterns, but studies of polymorphic restriction sites and gene dosage revealed an extensive deletion that removed all the beta- and beta-like globin genes from the affected chromosome. In situ hybridization of chromosome preparations with radioactive beta-globin gene probes showed that only one 11p homolog contained the beta-globin gene cluster in the affected family members. Images FIGURE 2 FIGURE 4 FIGURE 5 PMID:6308057

  19. Non-invasive cardiac output and oxygen delivery measurement in an infant with critical anemia

    Microsoft Academic Search

    Garry M. Steil; Olive S. Eckstein; Julie Caplow; Michael S. D. Agus; Brian K. Walsh; Jackson Wong

    2011-01-01

    Objective  To assess the combination of a non-invasive blood oxygen content (CaO2) monitor and a non-invasive cardiac output (CO) monitor to continuously measure oxygen delivery (DO2; DO2 = CaO2 × CO).\\u000a \\u000a \\u000a \\u000a \\u000a Methods  DO2 was assessed during blood transfusions in an infant with acute hemolytic anemia following admission (~48 h). CaO2 was measured by Pulse Co-Oximetry, which also provides estimates of hemoglobin (Hgb) concentration and percent

  20. Prediction of S-1-induced anemia

    Microsoft Academic Search

    Hyun Cheol Chung

    2009-01-01

    S-1, a novel oral fluoropyrimidine, has shown remarkably good tolerability in Korean gastric and colorectal cancer patients\\u000a due to its favorable safety profile. Myelosuppression and diarrhea were the events that precluded dose escalation in Japan,\\u000a whereas gastrointestinal toxicity and skin reaction were the major limiting factors in Western countries. In contrast, the\\u000a major adverse event in Korean patients was anemia,

  1. Current treatment of atypical hemolytic uremic syndrome

    PubMed Central

    Kaplan, Bernard S.; Ruebner, Rebecca L.; Spinale, Joann M.; Copelovitch, Lawrence

    2014-01-01

    Summary Tremendous advances have been made in understanding the pathogenesis of atypical Hemolytic Uremic Syndrome (aHUS), an extremely rare disease. Insights into the molecular biology of aHUS resulted in rapid advances in treatment with eculizumab (Soliris®, Alexion Pharmaceuticals Inc.). Historically, aHUS was associated with very high rates of mortality and morbidity. Prior therapies included plasma therapy and/or liver transplantation. Although often life saving, these were imperfect and had many complications. We review the conditions included under the rubric of aHUS: S. pneumoniae HUS (SpHUS), inborn errors of metabolism, and disorders of complement regulation, emphasizing their differences and similarities. We focus on the clinical features, diagnosis, and pathogenesis, and treatment of aHUS that results from mutations in genes encoding alternative complement regulators, SpHUS and HUS associated with inborn errors of metabolism. Mutations in complement genes, or antibodies to their protein products, result in unregulated activity of the alternate complement pathway, endothelial injury, and thrombotic microangiopathy (TMA). Eculizumab is a humanized monoclonal antibody that inhibits the production of the terminal complement components C5a and the membrane attack complex (C5b-9) by binding to complement protein C5a. This blocks the proinflammatory and cytolytic effects of terminal complement activation. Eculizumab use has been reported in many case reports, and retrospective and prospective clinical trials in aHUS. There have been few serious side effects and no reports of tachphylaxis or drug resistance. The results are very encouraging and eculizumab is now recognized as the treatment of choice for aHUS. PMID:25343125

  2. Iron-Deficiency Anemia (For Parents)

    MedlinePLUS

    ... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ... red blood cells. This is a condition called anemia . When someone has anemia, less oxygen reaches the ...

  3. Genetics Home Reference: Diamond-Blackfan anemia

    MedlinePLUS

    ... literature OMIM Genetic disorder catalog Conditions > Diamond-Blackfan anemia On this page: Description Genetic changes Inheritance Diagnosis ... definitions Reviewed February 2012 What is Diamond-Blackfan anemia? Diamond-Blackfan anemia is a disorder of the ...

  4. Genetics Home Reference: Dyserythropoietic anemia and thrombocytopenia

    MedlinePLUS

    ... Recent literature OMIM Genetic disorder catalog Conditions > Dyserythropoietic anemia and thrombocytopenia On this page: Description Genetic changes ... Glossary definitions Reviewed October 2014 What is dyserythropoietic anemia and thrombocytopenia? Dyserythropoietic anemia and thrombocytopenia is a ...

  5. Hyperemic peripheral red marrow in a patient with sickle cell anemia demonstrated on Tc-99m labeled red blood cell venography

    SciTech Connect

    Heiden, R.A.; Locko, R.C.; Stent, T.R. (Columbia Univ. College of Physicians and Surgeons, New York, NY (USA))

    1991-03-01

    A 25-year-old gravid woman, homozygous for sickle cell anemia, with a history of recent deep venous thrombosis, was examined using Tc-99m labeled red blood cell venography for recurrent thrombosis. Although negative for thrombus, the study presented an unusual incidental finding: the patient's peripheral bone marrow was hyperemic in a distribution consistent with peripheral red bone marrow expansion. Such a pattern has not been documented before using this technique. This report supports other literature that has demonstrated hyperemia of peripheral red bone marrow in other hemolytic anemias. This finding may ultimately define an additional role of scintigraphy in assessing the pathophysiologic status of the sickle cell patient.

  6. Modified Ham test for atypical hemolytic uremic syndrome.

    PubMed

    Gavriilaki, Eleni; Yuan, Xuan; Ye, Zhaohui; Ambinder, Alexander J; Shanbhag, Satish P; Streiff, Michael B; Kickler, Thomas S; Moliterno, Alison R; Sperati, C John; Brodsky, Robert A

    2015-06-01

    Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by excessive activation of the alternative pathway of complement (APC). Atypical HUS is frequently a diagnosis of exclusion. Differentiating aHUS from other TMAs, especially thrombotic thrombocytopenic purpura (TTP), is difficult due to overlapping clinical manifestations. We sought to develop a novel assay to distinguish aHUS from other TMAs based on the hypothesis that paroxysmal nocturnal hemoglobinuria cells are more sensitive to APC-activated serum due to deficiency of glycosylphosphatidylinositol- anchored complement regulatory proteins (GPI-AP). Here, we demonstrate that phosphatidylinositol-specific phospholipase C-treated EA.hy926 cells and PIGA-mutant TF-1 cells are more susceptible to serum from aHUS patients than parental EA.hy926 and TF-1 cells. We next studied 31 samples from 25 patients with TMAs, including 9 with aHUS and 12 with TTP. Increased C5b-9 deposition was evident by confocal microscopy and flow cytometry on GPI-AP-deficient cells incubated with aHUS serum compared with heat-inactivated control, TTP, and normal serum. Differences in cell viability were observed in biochemically GPI-AP-deficient cells and were further increased in PIGA-deficient cells. Serum from patients with aHUS resulted in a significant increase of nonviable PIGA-deficient TF-1 cells compared with serum from healthy controls (P < .001) and other TMAs (P < .001). The cell viability assay showed high reproducibility, sensitivity, and specificity in detecting aHUS. In conclusion, we developed a simple, rapid, and serum-based assay that helps to differentiate aHUS from other TMAs. PMID:25862562

  7. Hemolytic activity reevaluation of putative nonpathogenic Listeria monocytogenes strains.

    PubMed

    Lachica, R V

    1996-11-01

    Identification of 12 strains originally characterized as nonpathogenic Listeria monocytogenes was reassured following the evaluation of their hemolytic capability with a newly developed horse blood agar plate. Seven of the strains were observed consistently to be hemolytic and confirmed as L. monocytogenes with the use of two commercial systems: the Gene-Trak L. monocytogenes-specific colorimetric DNA hybridization assay and the API Listeria system. Except for one strain that formed typical smooth colonies, these hemolytic strains formed rough colonies on a selective medium, lithium chloride-ceftazidime agar. The rest of the strains were nonhemolytic and did not hybridize with the DNA probe; they were identified as Listeria innocua on the basis of their API Listeria system biochemical profile. All but one of these nonhemolytic strains formed smooth colonies on lithium chloride-ceftazidime agar. PMID:8984907

  8. Hemolytic activity reevaluation of putative nonpathogenic Listeria monocytogenes strains.

    PubMed Central

    Lachica, R V

    1996-01-01

    Identification of 12 strains originally characterized as nonpathogenic Listeria monocytogenes was reassured following the evaluation of their hemolytic capability with a newly developed horse blood agar plate. Seven of the strains were observed consistently to be hemolytic and confirmed as L. monocytogenes with the use of two commercial systems: the Gene-Trak L. monocytogenes-specific colorimetric DNA hybridization assay and the API Listeria system. Except for one strain that formed typical smooth colonies, these hemolytic strains formed rough colonies on a selective medium, lithium chloride-ceftazidime agar. The rest of the strains were nonhemolytic and did not hybridize with the DNA probe; they were identified as Listeria innocua on the basis of their API Listeria system biochemical profile. All but one of these nonhemolytic strains formed smooth colonies on lithium chloride-ceftazidime agar. PMID:8984907

  9. 78 FR 79469 - Strategies To Address Hemolytic Complications of Immune Globulin Infusions; Public Workshop

    Federal Register 2010, 2011, 2012, 2013, 2014

    2013-12-30

    ...Hemolytic Complications of Immune Globulin Infusions; Public Workshop AGENCY: Food and Drug...Hemolytic Complications of Immune Globulin Infusions.'' The purpose of the public workshop...Globulin Intravenous (IGIV) (Human) infusion. Complications of hemolysis...

  10. ITPA gene variant may protect against anemia induced during pegylated interferon alfa and ribavirin combination treatment in Ukrainian patients with chronic hepatitis C.

    PubMed

    Kucherenko, A; Pampukha, V; Bobrova I; Moroz, L; Livshits, L

    2015-01-01

    The aim of this study was to clarify the association between the inosine triphosphate pyrophosphatase (ITPA) gene variants and PEG-IFNalpha/RBV combination treatment induced anemia in chronic hepatitis C (CHC) Ukrainian patients. The data were collected from 80 CHC patients with HCV genotype 1 infection. All study participants received standard doses of PEG-IFNalpha and RBV According to the Hb level changes patients were distributed into: case group--42 patients with combination treatment induced anemia, and control group--38 patients with no signs of anemia. Genotyping for ITPA gene rs1127354 and rs7270101 variants was performed using PCR followed by RFLP assay. Fisher's exact test was used to estimate the difference in genotype and allelic distribution. Distribution of rs 7270101 genotypes was not significantly different between groups of CHC patients with RB Vinduced anemia and without it. The frequency of rs1127354 A allele carriers was significantly higher (P < < 0,05) in group of CHC patients without anemia (23.7%) comparing to the group ofpatients with anemia (7.3%). The respective allele frequency in control group (13.2%) was almost 3-fold higher (P < 0,05) comparing to the case group (4.9%). Significant association of ITPA gene rs1127354 with protection against RB V-induced hemolytic anemia was found in Ukrainian patients with CHC infection. Rs1127354 variant may assist as a pharmacogenetic marker in HCV antiviral therapy correction for side effect avoidance.. PMID:26030972

  11. The Molecular Connection Between Aluminum Toxicity, Anemia,

    E-print Network

    Appanna, Vasu

    25 The Molecular Connection Between Aluminum Toxicity, Anemia, Inflammation and Obesity D. Appanna Laurentian University Canada 1. Introduction Anemia is reported to be the most common of the types of anemia has different underlying causes. Iron (Fe) deficiency, a potent instigator of anemia

  12. Cationic amphiphilic non-hemolytic polyacrylates with superior antibacterial activity.

    PubMed

    Punia, Ashish; He, Edward; Lee, Kevin; Banerjee, Probal; Yang, Nan-Loh

    2014-07-01

    Acrylic copolymers with appropriate compositions of counits having cationic charge with 2-carbon and 6-carbon spacer arms can show superior antibacterial activities with concomitant very low hemolytic effect. These amphiphilic copolymers represent one of the most promising synthetic polymer antibacterial systems reported. PMID:24854366

  13. Identification of a hemolytic activity elaborated by Haemophilus ducreyi.

    PubMed Central

    Palmer, K L; Grass, S; Munson, R S

    1994-01-01

    Haemophilus ducreyi is the causative agent of the sexually transmitted disease chancroid. We have identified a hemolytic activity expressed by H. ducreyi. This activity is most readily detected when horse erythrocytes are used as a target; however, low levels of activity can be detected with sheep, human, or rabbit erythrocyte targets. The activity is heat labile and protease sensitive. PMID:8005696

  14. A hemolytic pigment of Group B Streptococcus allows bacterial penetration of human placenta

    PubMed Central

    Whidbey, Christopher; Harrell, Maria Isabel; Burnside, Kellie; Ngo, Lisa; Becraft, Alexis K.; Iyer, Lakshminarayan M.; Aravind, L.; Hitti, Jane

    2013-01-01

    Microbial infection of the amniotic fluid is a significant cause of fetal injury, preterm birth, and newborn infections. Group B Streptococcus (GBS) is an important human bacterial pathogen associated with preterm birth, fetal injury, and neonatal mortality. Although GBS has been isolated from amniotic fluid of women in preterm labor, mechanisms of in utero infection remain unknown. Previous studies indicated that GBS are unable to invade human amniotic epithelial cells (hAECs), which represent the last barrier to the amniotic cavity and fetus. We show that GBS invades hAECs and strains lacking the hemolysin repressor CovR/S accelerate amniotic barrier failure and penetrate chorioamniotic membranes in a hemolysin-dependent manner. Clinical GBS isolates obtained from women in preterm labor are hyperhemolytic and some are associated with covR/S mutations. We demonstrate for the first time that hemolytic and cytolytic activity of GBS is due to the ornithine rhamnolipid pigment and not due to a pore-forming protein toxin. Our studies emphasize the importance of the hemolytic GBS pigment in ascending infection and fetal injury. PMID:23712433

  15. How Is Aplastic Anemia Treated?

    MedlinePLUS

    ... need for blood transfusions. Medicines To Suppress the Immune System Research suggests that aplastic anemia may sometimes occur because the body's immune system attacks its own cells by mistake. For this ...

  16. Anemia in People with Cancer

    MedlinePLUS

    ... past and current medical conditions and do a physical exam. Other tests may be needed to help to find the cause of your anemia. These could include: Blood chemistry tests to check organ function and levels of ...

  17. Age-related changes in adaptation to severe anemia in childhood in developing countries

    PubMed Central

    O'Donnell, Angela; Premawardhena, A.; Arambepola, M.; Allen, S. J.; Peto, T. E. A.; Fisher, C. A.; Rees, D. C.; Olivieri, Nancy F.; Weatherall, D. J.

    2007-01-01

    Severe forms of anemia in children in the developing countries may be characterized by different clinical manifestations at particular stages of development. Whether this reflects developmental changes in adaptation to anemia or other mechanisms is not clear. The pattern of adaptation to anemia has been assessed in 110 individuals with hemoglobin (Hb) E ?-thalassemia, one of the commonest forms of inherited anemia in Asia. It has been found that age and Hb levels are independent variables with respect to erythropoietin response and that there is a decline in the latter at a similar degree of anemia during development. To determine whether this finding is applicable to anemia due to other causes, a similar study has been carried out on 279 children with severe anemia due to Plasmodium falciparum malaria; the results were similar to those in the patients with thalassemia. These observations may have important implications both for the better understanding of the pathophysiology of profound anemia in early life and for its more logical and cost-effective management. PMID:17517643

  18. What Are the Signs and Symptoms of Anemia?

    MedlinePLUS

    ... Twitter. What Are the Signs and Symptoms of Anemia? The most common symptom of anemia is fatigue ( ... mild symptoms or none at all. Complications of Anemia Some people who have anemia may have arrhythmias ( ...

  19. Autoimmune Polyglandular Syndrome Type 3c with Ectodermal Dysplasia, Immune Deficiency and Hemolytic-Uremic Syndrome

    PubMed Central

    Büyükçelik, Mithat; Keskin, Mehmet; Keskin, Özlem; Bay, Ali; Demircio?lu K?l?ç, Beltinge; Kor, Y?lmaz; K?l?nç, M. Arda; Balat, Ay?e

    2014-01-01

    Autoimmune polyglandular syndrome (APS) is a disorder which is associated with multiple endocrine gland insufficiency and also with non-endocrine manifestations. The pathophysiology of APS is poorly understood, but the hallmark evidence of APS is development of autoantibodies against multiple endocrine and non-endocrine organs. These autoantibodies are responsible for the dysfunction of the affected organs and sometimes may also cause non-endocrine organ dysfunction. The hemolytic-uremic syndrome (HUS) is a serious and life-threatening disease which develops due to many etiological factors including autoimmune disorders. Here, we present an unusual case of APS. Ectodermal dysplasia with immune deficiency and HUS occurred concomitantly in the same patient with APS type 3c. Once the autoantibody generation was initiated in the human body, development of multiple disorders due to organ dysfunction and also autoantibody-related diseases may have occurred. PMID:24637310

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

    SciTech Connect

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

    1984-10-01

    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.

  1. Hemolytic properties of miltefosine in liposomes of various lipid compositions

    Microsoft Academic Search

    M. V. Zhukova; O. V. Romanenko; V. A. Nikolaevich; M. A. Kisel’

    2010-01-01

    The hemolytic activity of hexadecylphosphocholine (HePC) included in liposomes of phosphatidylcholine (PC), phosphatidylethanol\\u000a (PET), and their mixture in various ratios has been studied. Spectroscopic data suggest that supramolecular particles containing\\u000a less than 15% HePC occur as liposomes. Hemolysis at the same HePC concentration is less pronounced for liposomes (10% HePC)\\u000a than for micelles (30% HePC). The inclusion of anionic phosphatidylethanol

  2. Peripheral gangrene complicating idiopathic and recessive hemolytic uremic syndromes

    Microsoft Academic Search

    Bernard S. Kaplan; Clotilde D. Garcia; Russell W. Chesney; William E. Segar; Katia Giugno; Roberto Chem

    2000-01-01

    Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred\\u000a in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the\\u000a second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more\\u000a than 1

  3. Arsenic Intoxication as a Cause of Megaloblastic Anemia

    Microsoft Academic Search

    D. Douglas Westhoff; Richard J. Samaha; Asa Barnes

    1975-01-01

    We have described a case of chronic arse- maturation in the marrow. The patient's nic intoxication associated with pancyto- serum folate and vitamin B12were normal, penia and megaloblastic erythropoiesis. and the anemia regressed without ther- The patient had the typical laboratory apy. Our case suggests that the combina- manifestations of ineffective erythropoie- tion of megaloblastosis with normoblastic sis due to

  4. Hemolytic venoms from marine cnidarian jellyfish – an overview

    PubMed Central

    Mariottini, Gian Luigi

    2014-01-01

    Cnidarian jellyfish are viewed as an emergent problem in several coastal zones throughout the world. Recurrent outbreaks pose a serious threat to tourists and bathers, as well as to sea-workers, involving health and economical aspects. As a rule, cnidarian stinging as a consequence of nematocyst firing induces merely local symptoms but cardiovascular or neurological complications can also occur. Hemolysis is a frequent effect of cnidarian stinging; this dangerous condition is known to be caused by several venoms and can sometimes be lethal. At present, the bulk of data concerning hemolytic cnidarian venoms comes from the study of benthic species, such as sea anemones and soft corals, but hemolytic factors were found in venoms of several siphonophore, cubozoan and scyphozoan jellyfish, which are mainly involved in the envenomation of bathers and sea-workers. Therefore, the aim of this paper is to review the scientific literature concerning the hemolytic venoms from cnidarian jellyfish taking into consideration their importance in human pathology as well as health implications and possible therapeutic measures. PMID:25386336

  5. Cloning and characterization of hemolytic genes from Helicobacter pylori.

    PubMed Central

    Drazek, E S; Dubois, A; Holmes, R K; Kersulyte, D; Akopyants, N S; Berg, D E; Warren, R L

    1995-01-01

    Strains of Helicobacter pylori, the bacterium associated with gastritis, peptic ulcer disease, and gastric cancer in humans, express different degrees of hemolysis on agar containing erythrocytes (RBC). Here we report the isolation and characterization of six recombinant clones from a genomic library of H. pylori ATCC 49503 that confer on Escherichia coli the ability to lyse sheep RBC. DNA hybridizations indicated no sequence homology among these hemolytic clones. Hybridization mapping of them to an ordered H. pylori cosmid library identified their separate chromosomal locations. One clone hybridized to two regions separated by approximately 200 kb. The specificities of the hemolytic activities of these clones were tested with RBC from humans, monkeys, cattle, horses, guinea pigs, rabbits, and chickens as well as with RBC from sheep. One clone conferred the ability to lyse RBC from five species, a second clone allowed the lysis of RBC from four of these species, three other clones allowed the lysis of RBC from three of these species, and the sixth clone allowed the lysis of RBC from just two species. We propose that some or all of the genes that confer these various hemolytic activities contribute to pathogen-host tissue interactions and that the different specificities seen here are important for H. pylori infections of humans of different genotypes or disease states. PMID:7591069

  6. Tuning the hemolytic and antibacterial activities of amphiphilic polynorbornene derivatives.

    PubMed

    Ilker, M Firat; Nüsslein, Klaus; Tew, Gregory N; Coughlin, E Bryan

    2004-12-01

    Amphiphilic cationic polynorbornene derivatives, soluble in water, were prepared from modular norbornene monomers, with a wide range of molecular weights (M(n) = 1600-137 500 g/mol) and narrow polydispersities (PDI = 1.1-1.3). The antibacterial activity determined by growth inhibition assays and the hemolytic activity against human red blood cells were measured and compared to determine the selectivity of the polymers for bacterial over mammalian cells. The effects of monomer repeat unit hydrophobicity and polymer molecular weight on antibacterial and hemolytic activities were determined. The hydrophobicity of the repeat unit was observed to have dramatic effects on antibacterial and hemolytic activities. Lipid membrane disruption activities of the polymers was confirmed by measuring polymer-induced dye leakage from large unilamellar vesicles. By tuning the overall hydrophobicity of the polymer through random copolymerizations of modular norbornene derivatives, highly selective, nonhemolytic antibacterial activities were obtained. For appropriate monomer composition, selectivity against bacteria versus human red blood cells was determined to be over 100. PMID:15571411

  7. Chicken anemia virus.

    PubMed

    Schat, K A

    2009-01-01

    Chicken anemia virus (CAV), the only member of the genus Gyrovirus of the Circoviridae, is a ubiquitous pathogen of chickens and has a worldwide distribution. CAV shares some similarities with Torque teno virus (TTV) and Torque teno mini virus (TTMV) such as coding for a protein inducing apoptosis and a protein with a dual-specificity phosphatase. In contrast to TTV, the genome of CAV is highly conserved. Another important difference is that CAV can be isolated in cell culture. CAV produces a single polycistronic messenger RNA (mRNA), which is translated into three proteins. The promoter-enhancer region has four direct repeats resembling estrogen response elements. Transcription is enhanced by estrogen and repressed by at least two other transcription factors, one of which is COUP-TF1. A remarkable feature of CAV is that the virus can remain latent in gonadal tissues in the presence or absence of virus-neutralizing antibodies. In contrast to TTV, CAV can cause clinical disease and subclinical immunosuppression especially affecting CD8+ T lymphocytes. Clinical disease is associated with infection in newly hatched chicks lacking maternal antibodies or older chickens with a compromised humoral immune response. PMID:19230563

  8. Lack of the lectin-like domain of thrombomodulin worsens Shiga toxin-associated hemolytic uremic syndrome in mice.

    PubMed

    Zoja, Carlamaria; Locatelli, Monica; Pagani, Chiara; Corna, Daniela; Zanchi, Cristina; Isermann, Berend; Remuzzi, Giuseppe; Conway, Edward M; Noris, Marina

    2012-10-01

    Shiga toxin (Stx)-producing Escherichia coli is a primary cause of diarrhea-associated hemolytic uremic syndrome (HUS), a disorder of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathophysiology of renal microvascular thrombosis in Stx-HUS is still ill-defined. Based on evidence that abnormalities in thrombomodulin (TM), an anticoagulant endothelial glycoprotein that modulates complement and inflammation, predispose to atypical HUS, we assessed whether impaired TM function may adversely affect evolution of Stx-HUS. Disease was induced by coinjection of Stx2/LPS in wild-type mice (TM(wt/wt)) and mice that lack the lectin-like domain of TM (TM(LeD/LeD)), which is critical for its anti-inflammatory and cytoprotective properties. After Stx2/LPS, TM(LeD/LeD) mice exhibited more severe thrombocytopenia and renal dysfunction than TM(wt/wt) mice. Lack of lectin-like domain of TM resulted in a stronger inflammatory reaction after Stx2/LPS with more neutrophils and monocytes/macrophages infiltrating the kidney, associated with PECAM-1 and chemokine upregulation. After Stx2/LPS, intraglomerular fibrin(ogen) deposits were detected earlier in TM(LeD/LeD) than in TM(wt/wt) mice. More abundant fibrin(ogen) deposits were also found in brain and lungs. Under basal conditions, TM(LeD/LeD) mice exhibited excess glomerular C3 deposits, indicating impaired complement regulation in the kidney that could lead to local accumulation of proinflammatory products. TM(LeD/LeD) mice with HUS had a higher mortality rate than TM(wt/wt) mice. If applicable to humans, these findings raise the possibility that genetic or acquired TM defects might have an impact on the severity of microangiopathic lesions after exposure to Stx-producing E. coli infections and raise the potential for using soluble TM in the treatment of Stx-HUS. PMID:22942429

  9. Delayed Anemia after Treatment with Injectable Artesunate in the Democratic Republic of the Congo: A Manageable Issue

    PubMed Central

    Burri, Christian; Ferrari, Giovanfrancesco; Ntuku, Henry Maggi; Kitoto, Antoinette Tshefu; Duparc, Stephan; Hugo, Pierre; Mitembo, Didier Kalemwa; Lengeler, Christian

    2014-01-01

    Cases of delayed hemolytic anemia have been described after treatment with injectable artesunate, the current World Health Organization (WHO)–recommended first-line drug for the treatment of severe malaria. A total of 350 patients (215 [61.4%] < 5 years of age and 135 [38.6%] ? 5 years of age) were followed-up after treatment with injectable artesunate for severe malaria in hospitals and health centers of the Democratic Republic of the Congo. Complete series of hemoglobin (Hb) measurements were available for 201 patients. A decrease in Hb levels between 2 and 5 g/dL was detected in 23 (11.4%) patients during the follow-up period. For five patients, Hb levels decreased below 5 g/dL during at least one follow-up visit. All cases of delayed anemia were clinically manageable and resolved within one month. PMID:25071004

  10. The spectrum of PIG-A gene mutations in aplastic anemia\\/paroxysmal nocturnal hemoglobinuria (AA\\/PNH): a high incidence of multiple mutations and evidence of a mutational hot spot

    Microsoft Academic Search

    Yousef Mortazavi; Bruno Merk; Jenny McIntosh; Judith C. W. Marsh; Hubert Schrezenmeier; Tim R. Rutherford

    2002-01-01

    Paroxysmal nocturnal hemoglobinuria (PNH) may arise during long-term follow- up of aplastic anemia (AA), and many AA patients have minor glycosylphosphatidy- linositol (GPI) anchor-deficient clones, even at presentation. PIG-A gene muta- tions in AA\\/PNH and hemolytic PNH are thought to be similar, but studies on AA\\/PNH have been limited to individual cases and a few small series. We have studied

  11. Clinico-aetiologic profile of macrocytic anemias with special reference to megaloblastic anemia

    Microsoft Academic Search

    Vineetha Unnikrishnan; Tarun Kumar Dutta; Bhawana A. Badhe; Zachariah Bobby; Ashish K. Panigrahi

    2008-01-01

    Purpose of study This study was conducted to study the clinical and laboratory parameters in patients with macrocytic anemia and to determine the etiology of macrocytic anemia with special reference to megaloblastic anemia. Materials and methods This study was a cross-sectional descriptive study carried over a period of 18 months on 60 adult patients (age ?13 years) of macrocytic anemia.

  12. Genetics Home Reference: Congenital dyserythropoietic anemia

    MedlinePLUS

    ... literature OMIM Genetic disorder catalog Conditions > Congenital dyserythropoietic anemia (often shortened to CDA ) On this page: Description ... Reviewed July 2009 What is CDA? Congenital dyserythropoietic anemia (CDA) is an inherited blood disorder that affects ...

  13. Anemia in inflammatory bowel disease: A neglected issue with relevant effects

    PubMed Central

    Guagnozzi, Danila; Lucendo, Alfredo J

    2014-01-01

    Anemia, a common complication associated with inflammatory bowel disease (IBD), is frequently overlooked in the management of IBD patients. Unfortunately, it represents one of the major causes of both decreased quality of life and increased hospital admissions among this population. Anemia in IBD is pathogenically complex, with several factors contributing to its development. While iron deficiency is the most common cause, vitamin B12 and folic acid deficiencies, along with the effects of pro-inflammatory cytokines, hemolysis, drug therapies, and myelosuppression, have also been identified as the underlying etiology in a number of patients. Each of these etiological factors thus needs to be identified and corrected in order to effectively manage anemia in IBD. Because the diagnosis of anemia in IBD often presents a challenge, combinations of several hematimetric and biochemical parameters should be used. Recent studies underscore the importance of determining the ferritin index and hepcidin levels in order to distinguish between iron deficiency anemia, anemia due to chronic disease, or mixed anemia in IBD patients. With regard to treatment, the newly introduced intravenous iron formulations have several advantages over orally-administered iron compounds in treating iron deficiency in IBD. In special situations, erythropoietin supplementation and biological therapies should be considered. In conclusion, the management of anemia is a complex aspect of treating IBD patients, one that significantly influences the prognosis of the disease. As a consequence, its correction should be considered a specific, first-line therapeutic goal in the management of these patients. PMID:24707137

  14. Erythroblast transferrin receptors and transferrin kinetics in iron deficiency and various anemias

    SciTech Connect

    Muta, K.; Nishimura, J.; Ideguchi, H.; Umemura, T.; Ibayashi, H.

    1987-06-01

    To clarify the role of transferrin receptors in cases of altered iron metabolism in clinical pathological conditions, we studied: number of binding sites; affinity; and recycling kinetics of transferrin receptors on human erythroblasts. Since transferrin receptors are mainly present on erythroblasts, the number of surface transferrin receptors was determined by assay of binding of /sup 125/I-transferrin and the percentage of erythroblasts in bone marrow mononuclear cells. The number of binding sites on erythroblasts from patients with an iron deficiency anemia was significantly greater than in normal subjects. Among those with an aplastic anemia, hemolytic anemia, myelodysplastic syndrome, and polycythemia vera compared to normal subjects, there were no considerable differences in the numbers of binding sites. The dissociation constants (Kd) were measured using Scatchard analysis. The apparent Kd was unchanged (about 10 nmol/L) in patients and normal subjects. The kinetics of endocytosis and exocytosis of /sup 125/I-transferrin, examined by acid treatment, revealed no variations in recycling kinetics among the patients and normal subjects. These data suggest that iron uptake is regulated by modulation of the number of surface transferrin receptors, thereby reflecting the iron demand of the erythroblast.

  15. Marzo de 2012 Lucha contra la anemia

    E-print Network

    N° 399 Marzo de 2012 Lucha contra la anemia: una estrategia más eficaz Scientific news Actualidad cientifica Actualité scientifique La carencia de hierro y la anemia1 que ésta puede provocar constituyen un. África y la India registran los mayores índices de anemia, con casi un 50% de las mujeres afectadas. En

  16. (Inborn anemias of mice): Terminal progress report

    SciTech Connect

    Bernstein, S.E.

    1987-01-01

    Mutations located at 11 different chromosomal locations in the mouse all affecting hemopoiesis have been studied. These include: Hertwig's anemia (an), W-anemias (W, W/sup v/, W/sup 17J/ to W/sup 41J/), Steel anemias (Sl, Sl/sup d/, etc.), Normoblastic anemia (nb), Jaundiced (ja), Spherocytic anemias (sph, sph/sup ha/), sph/sup 2J/, sph/sup 2BC/, Flexed-tail anemia (f), Microcytic anemia (mk), Sex-linked anemia (Sla), Alpha thallasemia (Hba/sup th/), and a hypochromic anemia associated with low transferrin levels (hpx). Our findings indicate that the erythroid defect in W-anemias stem from an intrinsic defect in the erythroid progenitor cells, and that all other erythroid hemostatic mechanisms are fully functional. Hertwig's anemia (an) is affected in a similar fashion. However, in the case of Steel anemias, the erythroid progenitors are repressed, but when transplanted to appropriate recipients were found to be fully functional. 70 refs., 4 tabs.

  17. Iron supplementation in renal anemia.

    PubMed

    Fishbane, Steven

    2006-07-01

    Iron-deficiency frequently develops in patients with chronic kidney disease who are treated with recombinant human erythropoietin (rHuEPO). It results in reduced effectiveness of anemia therapy; patients may fail to reach hemoglobin targets or may require excessively large doses of rHuEPO. It has been recognized widely that iron management, monitoring for iron deficiency, and effective iron supplementation forms a core component of anemia therapy. This review discusses the physiology of iron balance, derangements in iron balance in chronic kidney disease (CKD), and the diagnosis and treatment of iron deficiency in patients treated with rHuEPO. PMID:16949471

  18. Diagnosis of Atypical Hemolytic Uremic Syndrome and Response to Eculizumab Therapy

    PubMed Central

    Mathew, Jacob J; Denunzio, Troy M; Carmichael, Mark G

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) has a high mortality rate if not detected and treated early. While in the past, it was associated with renal failure in children, today, it has become increasingly identified among adults. Due to recent advances in the pathogenesis of aHUS and other major thrombotic microangiopathies (TMA), diagnosing it has become a lot easier. We present a case of a 62-year-old man who was initially thought to have thrombotic thrombocytopenic purpura (TTP), but after further evaluation was diagnosed with aHUS. We will discuss how to distinguish aHUS from other major TMA and the role of eculizumab in the management of aHUS. PMID:25285252

  19. Perioperative anemia management in colorectal cancer patients: A pragmatic approach

    PubMed Central

    Muñoz, Manuel; Gómez-Ramírez, Susana; Martín-Montañez, Elisa; Auerbach, Michael

    2014-01-01

    Anemia, usually due to iron deficiency, is highly prevalent among patients with colorectal cancer. Inflammatory cytokines lead to iron restricted erythropoiesis further decreasing iron availability and impairing iron utilization. Preoperative anemia predicts for decreased survival. Allogeneic blood transfusion is widely used to correct anemia and is associated with poorer surgical outcomes, increased post-operative nosocomial infections, longer hospital stays, increased rates of cancer recurrence and perioperative venous thromboembolism. Infections are more likely to occur in those with low preoperative serum ferritin level compared to those with normal levels. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management, minimizes or eliminates allogeneic blood transfusion. This includes restrictive transfusion policy, thromboprophylaxis and anemia management to improve outcomes. Normalization of preoperative hemoglobin levels is a World Health Organization recommendation. Iron repletion should be routinely ordered when indicated. Oral iron is poorly tolerated with low adherence based on published evidence. Intravenous iron is safe and effective but is frequently avoided due to misinformation and misinterpretation concerning the incidence and clinical nature of minor infusion reactions. Serious adverse events with intravenous iron are extremely rare. Newer formulations allow complete replacement dosing in 15-60 min markedly facilitating care. Erythropoiesis stimulating agents may improve response rates. A multidisciplinary, multimodal, individualized strategy, collectively termed Patient Blood Management used to minimize or eliminate allogeneic blood transfusion is indicated to improve outcomes. PMID:24587673

  20. Anemia prevalence and treatment practice in patients with non-myeloid tumors receiving chemotherapy

    PubMed Central

    Merlini, Laura; Cartenì, Giacomo; Iacobelli, Stefano; Stelitano, Caterina; Airoldi, Mario; Balcke, Peter; Keil, Felix; Haslbauer, Ferdinand; Belton, Laura; Pujol, Beatriz

    2013-01-01

    Purpose To describe the prevalence and management of anemia in cancer patients. Methods This cross-sectional, observational survey was conducted in Italy and Austria. Centers prespecified one day, during a 4-month enrollment window, to report specific data collected during normal clinical practice for patients with non-myeloid tumors attending for chemotherapy (±radiotherapy) treatment. The primary endpoint was the prevalence of anemia as determined using a prespecified algorithm: hemoglobin (Hb) ?10 g/dL on/within 3 days prior to visit; ongoing anemia treatment; physician diagnosis of anemia, together with ?1 anemia symptom. Results Between November 18, 2010 and March 18, 2011, data for 1412 patients were collected (Italy n = 1130; Austria n = 282). Most patients (n = 1136; 80%) had solid tumors; 809 (57%) had received ?3 chemotherapy cycles. The prevalence of anemia was 32% (95% confidence interval: 29.4%–34.2%); 196 patients (14%) were deemed anemic based on Hb ?10 g/dL, 131 (9%) on ongoing anemia treatment, and 121 (9%) on physician diagnosis/anemia symptom. Overall, 1153 patients (82%) had Hb data; mean (standard deviation [SD]) Hb levels were 11.7 (1.7) g/dL. In total, 456 patients (32%) had anemia symptoms: fatigue (n = 392; 28%), depression (n = 122; 9%), and dyspnea (n = 107; 8%) were most common. Fifty-one patients (4%) had had their current chemotherapy cycle delayed due to anemia. On visit day, or ?28 days prior, 91 (6%), 188 (13%), and 81 patients (6%) had evidence of whole blood/red blood cell transfusion, erythropoiesis-stimulating agent use, or iron use, respectively. Conclusion On the prespecified study day, one-third of patients with non-myeloid tumors undergoing chemotherapy were found to be anemic and 13% had evidence of erythropoiesis-stimulating agent use then or in the 28 days prior. PMID:23946669

  1. Hypothesis: Hemolytic Transfusion Reactions Represent an Alternative Type of Anaphylaxis

    PubMed Central

    Hod, Eldad A.; Sokol, Set A.; Zimring, James C.; Spitalnik, Steven L.

    2009-01-01

    Classical anaphylaxis is the most severe, and potentially fatal, type of allergic reaction, manifested by hypotension, bronchoconstriction, and vascular permeability. Similarly, a hemolytic transfusion reaction (HTR) is the most feared consequence of blood transfusion. Evidence for the existence of an alternative, IgG-mediated pathway of anaphylaxis may be relevant for explaining the pathophysiology of IgG-mediated-HTRs. The purpose of this review is to summarize the evidence for this alternative pathway of anaphylaxis and to present the hypothesis that an IgG-mediated HTR is one example of this type of anaphylaxis. PMID:18830382

  2. Erythropoietic response to acute anemia.

    PubMed

    Rosen, A L; Gould, S A; Sehgal, L R; Levine, E A; Sehgal, H L; Goldwasser, E; Beaver, C W; Moss, G S

    1990-03-01

    Reliance on a brisk erythropoietic response to untreated blood loss is an alternative to transfusion of homologous blood. Slow erythropoiesis has been observed in ICU patients who refused blood. Many of these patients received supplemental oxygen therapy and Fluosol-DA, a temporary red cell substitute. This study reports the erythropoietic response, in the baboon, to moderate (Hct 20%) and severe (Hct 10%) anemia. In addition, the effect of oxygen therapy (FIO2 0.6 for 1 wk) and fluorocarbon emulsions (Oxypherol) on erythropoiesis was evaluated. Baboons uniformly survived acute normovolemic anemia with Hct 10%. In all cases, the response to anemia was characterized by a lag period (with no change in Hct), and a nonlinear recovery period. A lag period of 3 days was observed in both moderate and severe anemia for baboons breathing room air or FIO2 0.6. The lag period was prolonged to 1 wk in the presence of Oxypherol. The recovery period exhibited a uniform and negative correlation between the rate of Hct change and the Hct, in all cases. The theoretical maximum rate of increase of Hct was 2.6%/day. In untreated blood loss, shortening the lag period and increasing the slope of the recovery period will decrease the length of time that the patient is anemic. PMID:1689236

  3. Erythropoietin in anemia of prematurity

    Microsoft Academic Search

    Suraj Gupte

    2003-01-01

    Sir, May I support the projections made by Atasay et alI that early erythropoietin administration may not be considered a dependable therapeutic measure in immediately reducing the blood transfusion need in anemia of prematurity. Instead, preferred recourse should be at providing minimal phlebotomy losses by employing noninvasive monitorization techniques and microlaboratory methods, and iron and protein supplementation with optimal nutrition.

  4. Cooley's Anemia: A Psychosocial Directory.

    ERIC Educational Resources Information Center

    National Center for Education in Maternal and Child Health, Washington, DC.

    The directory is intended to aid patients and their families who are coping with the genetic disorder of Cooley's anemia. A brief review of the disease covers background, genetics, symptoms, effect on the patient, treatment, and current research. The next section looks at psychosocial needs at various times (time of diagnosis, infancy and toddler…

  5. An anemia of Alzheimer's disease.

    PubMed

    Faux, N G; Rembach, A; Wiley, J; Ellis, K A; Ames, D; Fowler, C J; Martins, R N; Pertile, K K; Rumble, R L; Trounson, B; Masters, C L; Bush, A I

    2014-11-01

    Lower hemoglobin is associated with cognitive impairment and Alzheimer's disease (AD). Since brain iron homeostasis is perturbed in AD, we investigated whether this is peripherally reflected in the hematological and related blood chemistry values from the Australian Imaging Biomarker and Lifestyle (AIBL) study (a community-based, cross-sectional cohort comprising 768 healthy controls (HC), 133 participants with mild cognitive impairment (MCI) and 211 participants with AD). We found that individuals with AD had significantly lower hemoglobin, mean cell hemoglobin concentrations, packed cell volume and higher erythrocyte sedimentation rates (adjusted for age, gender, APOE-?4 and site). In AD, plasma iron, transferrin, transferrin saturation and red cell folate levels exhibited a significant distortion of their customary relationship to hemoglobin levels. There was a strong association between anemia and AD (adjusted odds ratio (OR)=2.43, confidence interval (CI) (1.31, 4.54)). Moreover, AD emerged as a strong risk factor for anemia on step-down regression, even when controlling for all other available explanations for anemia (adjusted OR=3.41, 95% CI (1.68, 6.92)). These data indicated that AD is complicated by anemia, which may itself contribute to cognitive decline. PMID:24419041

  6. [Anemia as a surgical risk factor].

    PubMed

    Moral García, Victoria; Ángeles Gil de Bernabé Sala, M; Nadia Diana, Kinast; Pericas, Bartolomé Cantallops; Nebot, Alexia Galindo

    2013-07-01

    Perioperative anemia is common in patients undergoing surgery and is associated with increased morbidity and mortality and a decreased quality of life. The main causes of anemia in the perioperative context are iron deficiency and chronic inflammation. Anemia can be aggravated by blood loss during surgery, and is most commonly treated with allogeneic transfusion. Moreover, blood transfusions are not without risks, once again increasing patient morbidity and mortality. Given these concerns, we propose to review the pathophysiology of anemia in the surgical environment, as well as its treatment through the consumption of iron-rich foods and by oral or intravenous iron therapy (iron sucrose and iron carboxymaltose). In chronic inflammatory anemia, we use erythropoiesis-stimulating agents (erythropoietin alpha) and, in cases of mixed anemia, the combination of both treatments. The objective is always to reduce the need for perioperative transfusions and speed the recovery from postoperative anemia, as well as decrease the patient morbidity and mortality rate. PMID:24314568

  7. Decreased Hematocrit-To-Viscosity Ratio and Increased Lactate Dehydrogenase Level in Patients with Sickle Cell Anemia and Recurrent Leg Ulcers

    PubMed Central

    Connes, Philippe; Lamarre, Yann; Hardy-Dessources, Marie-Dominique; Lemonne, Nathalie; Waltz, Xavier; Mougenel, Danièle; Mukisi-Mukaza, Martin; Lalanne-Mistrih, Marie-Laure; Tarer, Vanessa; Tressières, Benoit; Etienne-Julan, Maryse; Romana, Marc

    2013-01-01

    Leg ulcer is a disabling complication in patients with sickle cell anemia (SCA) but the exact pathophysiological mechanisms are unknown. The aim of this study was to identify the hematological and hemorheological alterations associated with recurrent leg ulcers. Sixty-two SCA patients who never experienced leg ulcers (ULC-) and 13 SCA patients with a positive history of recurrent leg ulcers (ULC+) - but with no leg ulcers at the time of the study – were recruited. All patients were in steady state condition. Blood was sampled to perform hematological, biochemical (hemolytic markers) and hemorheological analyses (blood viscosity, red blood cell deformability and aggregation properties). The hematocrit-to-viscosity ratio (HVR), which reflects the red blood cell oxygen transport efficiency, was calculated for each subject. Patients from the ULC+ group were older than patients from the ULC- group. Anemia (red blood cell count, hematocrit and hemoglobin levels) was more pronounced in the ULC+ group. Lactate dehydrogenase level was higher in the ULC+ group than in the ULC- group. Neither blood viscosity, nor RBC aggregation properties differed between the two groups. HVR was lower and RBC deformability tended to be reduced in the ULC+ group. Our study confirmed increased hemolytic rate and anemia in SCA patients with leg ulcers recurrence. Furthermore, our data suggest that although systemic blood viscosity is not a major factor involved in the pathophysiology of this complication, decreased red blood cell oxygen transport efficiency (i.e., low hematocrit/viscosity ratio) may play a role. PMID:24223994

  8. Cerebral sinovenous thrombosis associated with iron deficiency anemia secondary to severe menorrhagia: a case report.

    PubMed

    Corrales-Medina, Fernando F; Grant, Leon; Egas-Bejar, Daniela; Valdivia-Ascuna, Zoila; Rodriguez, Nidra; Mancias, Pedro

    2014-09-01

    Cerebral sinovenous thrombosis is a rare condition presenting with a wide spectrum of nonspecific symptoms that can make early diagnosis difficult. Cerebral sinovenous thrombosis has been associated with various etiologies. Iron deficiency anemia associated with cerebral sinovenous thrombosis in teenagers is rare. We present a teenage patient with complete thrombosis of the vein of Galen, straight sinus, and left internal cerebral vein associated with iron deficiency anemia due to severe menorrhagia. Mechanisms that can explain the association between iron deficiency anemia and thrombosis are discussed. PMID:24056151

  9. A comparison between the hemolytic and antibacterial activities of new quaternary ammonium polymers

    Microsoft Academic Search

    M. A. Marchisio; P. Bianciardi; T. Longo; P. Ferruti; E. Ranucci; M. G. Neri

    1995-01-01

    —New quaternary ammonium polymers, which in a previous work had shown relevant antibacterial properties, have been investigated as regards to their hemolytic activity (HA) in comparison with a low molecular weight commercial antibacterial agent, Steramine G (SG). All polymers exhibit negligible, or at most modest, HA at dosages and contact times at which SG is strongly hemolytic.

  10. Hemolytic action and surface activity of triterpene saponins from Anchusa officinalis L. Part 21: On the costituents of Boraginaceae.

    PubMed

    Romussi, G; Cafaggi, S; Bignardi, G

    1980-08-01

    Relationship between chemical structure, hemolytic action and surface activity of five glucosides of oleanolic acid were studied. Monodesmosidic saponins are more hemolytic than bisdesmosidic. The hemolytic activity of monodesmosides decreased with the length and the branching of the glucosidic chain. Surface activity is major in bisdesmosides. This property increases with the length of the glucosidic chain in monodesmosides. Increase of hemolytic action is accompanied by a decrease of surface activity. PMID:7433502

  11. Alleviated anemia by bendamustine in cold agglutinin disease associated with small lymphocytic lymphoma.

    PubMed

    Kuno, Masatomo; Inoue, Atsushi; Aimoto, Mizuki; Nakao, Takafumi; Kameda, Kazuaki; Yoshida, Masahiro; Kanashima, Hiroshi; Hirai, Manabu; Yamane, Takahisa

    2015-01-01

    A 77-year-old man was diagnosed with cold agglutinin disease in 2004. He had been treated with prednisolone with stabilization of hemoglobin in the 6- to 8-g/dl range. However, his hemolytic anemia worsened, and computed tomography showed systemic lymphadenopathy in May 2012. A pathological diagnosis of small lymphocytic lymphoma was made based on an inguinal lymph node biopsy. Treatment was started with rituximab. However, there was no response to 6 doses of rituximab monotherapy. He next received 6 courses of bendamustine in combination with rituximab. This resulted in stabilization of hemoglobin and independence from transfusion support. To the best of our knowledge, this is only the second case report describing bendamustine plus rituximab treatment for non-Hodgkin lymphoma complicated by cold agglutinin disease. Our results in this case suggest bendamustine to potentially be a useful therapeutic option in patients with cold agglutinin disease. PMID:25765801

  12. Acute Renal Failure, Microangiopathic Haemolytic Anemia, and Secondary Oxalosis in a Young Female Patient

    PubMed Central

    Stepien, Karolina M.; Prinsloo, Peter; Hitch, Tony; McCulloch, Thomas A.; Sims, Rebecca

    2011-01-01

    A 29-year old female presented with a one-week history of vomiting, diarrhoea, abdominal pain, and headache. On admission, she had acute renal failure requiring dialysis. Tests revealed a hemolytic anemia with thrombocytopenia. An initial diagnosis of thrombotic thrombocytopenic microangiopathy was made and plasma exchange was instigated. However, renal biopsy did not show thrombotic microangiopathy but instead revealed acute kidney injury with mild tubulointerstitial nephritis and numerous oxalate crystals, predominantly in the distal tubules. The patient had been taking large doses (>1100?mg daily) of vitamin C for many months. She also gave a history of sclerotherapy using injections of an ethylene glycol derivative for superficial leg veins. The patient completed five sessions of plasma exchange and was able to discontinue dialysis. She eventually achieved full renal recovery. She has now discontinued sclerotherapy and vitamin supplementation. PMID:21785726

  13. Recent approaches for reducing hemolytic activity of chemotherapeutic agents.

    PubMed

    Jeswani, Gunjan; Alexander, Amit; Saraf, Shailendra; Saraf, Swarnlata; Qureshi, Azra; Ajazuddin

    2015-08-10

    Drug induced hemolysis is a frequent complication associated with chemotherapy. It results from interaction of drug with erythrocyte membrane and leads to cell lysis. In recent past, various approaches were made to reduce drug-induced hemolysis, which includes drug polymer conjugation, drug delivery via colloidal carriers and hydrogels, co-administration of botanical agents and modification in molecular chemistry of drug molecules. The basic concept behind these strategies is to protect the red blood cells from membrane damaging effects of drugs. There are several examples of drug polymer conjugate that either are approved by Food and Drug Administration or are under clinical trial for delivering drugs with reduced toxicities. Likewise, colloidal carriers are also used successfully nowadays for the delivery of various chemotherapeutic agents like gemcitabine and amphotericin B with remarkable decrease in their hemolytic activity. Similarly, co-administration of botanical agents with drugs works as secondary system proving protection and strength to erythrocyte membranes. In addition to the above statement, interaction hindrance between RBC and drug molecule by molecular modification plays an important role in reducing hemolysis. This review predominantly describes the above recent approaches explored to achieve the reduced hemolytic activity of drugs especially chemotherapeutic agents. PMID:26047758

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

    PubMed Central

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

    2009-01-01

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

  15. [Immunomodulating activity of polyunsaturated phospholipids and regulators of energy metabolism in toxic anemia].

    PubMed

    Lazareva, G A; Brovkina, I L

    2004-01-01

    Possible potentiation of the immunomodulating effects of carbohydrate and lipid metabolism regulators by their use in combination with polyunsaturated phospholipids was studied. The polyunsaturated phospholipids in toxic anemia icreased the immunomodulating effects of thiamine and inosine which activated glucose catabolism in erythrocytes. The combined use of the polyunsaturated phospholipids and thiamine normalized the oxidation--energy status and lowered manifestation of the immunosuppressing properties of light erythrocytes in laboratory rats exposed to hemolytic poison. The use of the combination of the polyunsaturated phospholipids and inosine normalized the oxidation--energy status and induced manifestation of the immunomodulating properties in heavy erythrocytes of the poisoned rats. The globulin fraction of the rat serum containing antibodies to erythrocytes of the poisoned rats exposed to the polyunsaturated phospholipids and inosine increased the immunity status of the poisoned rats treated with the above mentioned agents. Carnitine and biotin in combination with the polyunsatured phospholipids showed no effect on the phagocytic and metabolic activity of leukocytes and the immunity status of the rats exposed to hemolytic poison. PMID:15573896

  16. Managing anemia in lymphoma and multiple myeloma

    PubMed Central

    Birgegård, Gunnar

    2008-01-01

    Anemia is common in cancer, and lymphoproliferative disease is no exception. Erythropoiesis-stimulating agents (ESA) have been used for renal anemia since 1986, and considerably later in cancer anemia. The first studies were published around 1993, but the use of ESA did not become common in cancer anemia until in the late 1990s. Cancer anemia is still under-treated. This review gives an overview of the use of ESA in hematologic malignancies. A background is given about this treatment in the cancer field generally. The pathophysiology of cancer anemia is described with special emphasis on the disturbances in iron metabolism. Functional iron deficiency has been shown to be both frequent and important as a hindrance for response to ESA treatment, and recent studies are reported in some detail, where the use of intravenous iron was shown to improve the response rate of ESA treatment. PMID:18728848

  17. Diagnosis and treatment of unexplained anemia with iron deficiency without overt bleeding.

    PubMed

    Dahlerup, Jens Frederik; Eivindson, Martin; Jacobsen, Bent Ascanius; Jensen, Nanna Martin; Jørgensen, Søren Peter; Laursen, Stig Borbjerg; Rasmussen, Morten; Nathan, Torben

    2015-04-01

    A general overview is given of the causes of anemia with iron deficiency as well as the pathogenesis of anemia and the para-clinical diagnosis of anemia. Anemia with iron deficiency but without overt GI bleeding is associated with a risk of malignant disease of the gastrointestinal tract; upper gastrointestinal cancer is 1/7 as common as colon cancer. Benign gastrointestinal causes of anemia are iron malabsorption (atrophic gastritis, celiac disease, chronic inflammation, and bariatric surgery) and chronic blood loss due to gastrointestinal ulcerations. The following diagnostic strategy is recommended for unexplained anemia with iron deficiency: conduct serological celiac disease screening with transglutaminase antibody (IgA type) and IgA testing and perform bidirectional endoscopy (gastroscopy and colonoscopy). Bidirectional endoscopy is not required in premenopausal women < 40 years of age. Small intestine investigation (capsule endoscopy, CT, or MRI enterography) is not recommended routinely after negative bidirectional endoscopy but should be conducted if there are red flags indicating malignant or inflammatory small bowel disease (e.g., involuntary weight loss, abdominal pain or increased CRP). Targeted treatment of any cause of anemia with iron deficiency found on diagnostic assessment should be initiated. In addition, iron supplementation should be administered, with the goal of normalizing hemoglobin levels and replenishing iron stores. Oral treatment with a 100-200 mg daily dose of elemental iron is recommended (lower dose if side effects), but 3-6 months of oral iron therapy is often required to achieve therapeutic goals. Intravenous iron therapy is used if oral treatment lacks efficacy or causes side effects or in the presence of intestinal malabsorption or prolonged inflammation. Three algorithms are given for the following conditions: a) the paraclinical diagnosis of anemia with iron deficiency; b) the diagnostic work-up for unexplained anemia with iron deficiency without overt bleeding; and c) how to proceed after negative bidirectional endoscopy of the gastrointestinal tract. PMID:25872536

  18. Gua breve sobre la La anemia es un trastorno de la sangre. La sangre es

    E-print Network

    Bandettini, Peter A.

    Anemia Guía breve sobre la La anemia es un trastorno de la sangre. La sangre es un líquido esencial de anemia, como la anemia por deficien- cia de hierro, la anemia perniciosa, la anemia aplásica y la anemia hemolítica. Los distintos tipos de anemia tienen relación con diversas enfermedades y problemas de

  19. Factors influencing hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye.

    PubMed

    Yu, Huahua; Li, Cuiping; Li, Ronggui; Xing, Ronge; Liu, Song; Li, Pengcheng

    2007-07-01

    In this study, hemolytic activity of venom from the jellyfish Rhopilema esculentum Kishinouye and some factors affecting it were assayed. The HU(50) of R. esculentum full venom (RFV) against chicken erythrocytes was 3.40 microg/ml and a Hill coefficient value was 1.73 suggesting at least two molecules participated in hemolytic activity. The hemolytic activity of RFV was affected by some chemical and physical factors such as divalent cations, EDTA, (NH(4))(2)SO(4), pH and temperature. In the presence of Mg(2+), Cu(2+), Zn(2+), Fe(2+), Ca(2+) (>or=2 mM), Mn(2+) ((>or=1 mM), EDTA ((>or=2 mM) and (NH(4))(2)SO(4), the hemolytic activity of RFV was reduced. RFV had strong hemolytic activity at the pH 6-10 and the hemolytic ratios were 0.95-1.19. Hemolytic activity was temperature-sensitive and when RFV was pre-incubated at temperatures over 40 degrees C, it was sharply reduced. PMID:17306433

  20. Genetics Home Reference: Iron-refractory iron deficiency anemia

    MedlinePLUS

    ... Genetic disorder catalog Conditions > Iron-refractory iron deficiency anemia On this page: Description Genetic changes Inheritance Diagnosis ... July 2014 What is iron-refractory iron deficiency anemia? Iron-refractory iron deficiency anemia is one of ...

  1. Genetics Home Reference: Thiamine-responsive megaloblastic anemia syndrome

    MedlinePLUS

    ... OMIM Genetic disorder catalog Conditions > Thiamine-responsive megaloblastic anemia syndrome On this page: Description Genetic changes Inheritance ... Reviewed February 2009 What is thiamine-responsive megaloblastic anemia syndrome? Thiamine-responsive megaloblastic anemia syndrome is a ...

  2. Genetics Home Reference: X-linked sideroblastic anemia and ataxia

    MedlinePLUS

    ... OMIM Genetic disorder catalog Conditions > X-linked sideroblastic anemia and ataxia On this page: Description Genetic changes ... Reviewed April 2009 What is X-linked sideroblastic anemia and ataxia? X-linked sideroblastic anemia and ataxia ...

  3. Genetics Home Reference: X-linked sideroblastic anemia

    MedlinePLUS

    ... OMIM Genetic disorder catalog Conditions > X-linked sideroblastic anemia On this page: Description Genetic changes Inheritance Diagnosis ... Reviewed April 2009 What is X-linked sideroblastic anemia? X-linked sideroblastic anemia is an inherited disorder ...

  4. Child with aplastic anemia: Anesthetic management

    PubMed Central

    Kaur, Manpreet; Gupta, Babita; Sharma, Aanchal; Sharma, Sanjeev

    2012-01-01

    Aplastic anemia is a rare heterogeneous disorder of hematopoietic stem cells causing pancytopenia and marrow hypoplasia with the depletion of all types of blood cells. This results in anemia, neutropenia and thrombocytopenia, which pose a challenge to both surgical and anesthetic management of such cases. We report a child with aplastic anemia who sustained traumatic ulcer on the arm and underwent split-thickness skin grafting under general anesthesia. There are only two case reports on anesthetic considerations in aplastic anemia patients in the literature. The anesthetic management is challenging because of the rarity of the disease, associated pancytopenia and immunosuppression. PMID:23162410

  5. Anemia - Multiple Languages: MedlinePlus

    MedlinePLUS

    ... ????) French (français) Hindi (??????) Japanese (???) Korean (???) Russian (???????) Somali (af Soomaali) Spanish (español) ... ??? - ??? (Japanese) Bilingual PDF Health Information Translations Korean (???) Anemia ?? - ??? (Korean) Bilingual PDF Health ...

  6. Molecular Basis for Group B ? -hemolytic Streptococcal Disease

    NASA Astrophysics Data System (ADS)

    Hellerqvist, Carl G.; Sundell, Hakan; Gettins, Peter

    1987-01-01

    Group B ? -hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin.

  7. Molecular basis for group B beta-hemolytic streptococcal disease.

    PubMed Central

    Hellerqvist, C G; Sundell, H; Gettins, P

    1987-01-01

    Group B beta-hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin. PMID:3540959

  8. [Regional differences in prevalence of anemia found by periodic health checkups at workplaces in Japan].

    PubMed

    Shimomura, Tomoko; Wakabayashi, Ichiro

    2010-01-01

    Anemia-related blood examinations are included in examinations for periodic health checkups at workplaces designated by the Industrial Safety and Health Law in Japan. The aim of this study was to determine whether there were regional differences in the prevalence of anemia in workers and, if so, to investigate possible reasons for the differences. Relationships between prevalence of anemia found by periodic health checkups and some common factors related to anemia in each prefecture of Japan were investigated by ecological regression analysis using Spearman's rank correlation coefficient. There were regional differences in the prevalence of anemia in the prefectures of Japan (5.2-11.7%), and high prevalence was observed in prefectures in the northeastern district, such as Iwate, Akita and Yamagata Prefectures, and in Fukui, Shimane and Nagasaki Prefectures. Prevalence of anemia in each prefecture was significantly correlated with the prevalence of hypertension, dyslipidemia, liver dysfunction, abnormality in ECG, hyperglycemia or glucosuria at health checkups in each prefecture. Prevalence of anemia in each prefecture was significantly correlated with the percentage of patients receiving therapy for anemia in each prefecture but not with the prevalence of myoma uteri, endometriosis uteri or mortality of uterus cancer in each prefecture. There was also no significant correlation of the prevalence of anemia with the prevalence of iron-deficiency anemia or dietary iron intake in each prefecture. The prevalence of anemia in each prefecture showed significant positive correlations with the ratio of female population to total population and the ratio of female workers to total workers in each prefecture; it also showed a significant negative correlation with the ratio of the number of large-sized workplaces (300 or more workers) to the number of workplaces with 50 or more workers in each prefecture. A considerable regional difference in the prevalence of anemia was found by periodic health checkups at workplaces, and we consider that this difference is not due to regional differences in the incidence of diseases causing genital bleeding in women but to regional differences in the ratio of female workers to total workers and the status of health control at the workplace, which depends on size of the workplace. PMID:19942817

  9. Large twisted ovarian fibroma associated with Meigs’ syndrome, abdominal pain and severe anemia treated by laparoscopic surgery

    PubMed Central

    2014-01-01

    Background The Meigs' syndrome is a rare but well-known syndrome defined as the triad of benign solid ovarian tumor, ascites, and pleural effusion. Meigs' syndrome always requires surgical treatment. However, the optimal approach for its management has not been sufficiently investigated. Case presentation We report a patient with a large twisted ovarian fibroma associated with Meigs’ syndrome, abdominal pain and severe hemolytic anemia that was treated by laparoscopic surgery. This case highlights the difficulties that may be encountered in the management of patients with Meigs’ syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach and the adverse impact that Meigs’ syndrome can have on the patient’s condition, especially if it is associated with acute pain and severe anemia. Considering the patient’s serious clinical condition and assuming that she had Meigs' syndrome with a twisted large ovarian mass and possible hemolytic anemia, we first concentrated on effective medical management of our patient and chose the most appropriate surgical treatment after laparoscopic examination. The main aim of our initial approach was preoperative management of the anemia. Blood transfusions and glucocorticoid therapy resulted in stabilization of the hemoglobin level and normalization of the bilirubin levels, which confirmed the appropriateness of this approach. Laparoscopic surgery 4 days after admission enabled definitive diagnosis of the tumor, confirmed torsion and removed the bulky ovarian fibroma, resulting in timely resolution of symptoms, short hospitalization, relatively low morbidity and a rapid return to her social and professional life. Conclusions This case highlights the difficulties that may be encountered in the management of patients with Meigs' syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach, and the adverse impact that Meigs' syndrome can have on the patient's condition, especially if it is associated with acute pain and severe anemia. The present case suggests that laparoscopic surgery for potentially large malignant tumors is feasible and safe, but requires an appropriate medical and gynecological oncology expertise. PMID:24962423

  10. [The characteristics of iron metabolism under iron-deficiency anemia and chronic disorders anemia].

    PubMed

    Smorkalova, E V; Aznabaeva, L F; Nikulicheva, V I; Safuanova, G Sh; Chepurnaia, A N

    2011-07-01

    The study investigated the issues of iron metabolism under iron-deficiency anemia and chronic disorders anemia and dependencies of production of IL-1? and sICAM-1 immunoinflammatory markers from degree of severity and duration of anemia. The study data indicates that under iron-deficiency anemia lactoferrin and sICAM-1 are the negative regulators of hemopoiesis. The inhibition of transferrin expression by the proinflammatory cytokines is one of the causes of inefficient hemopoiesis under chronic disorders anemia. PMID:21899115

  11. Anemia in Children with Down Syndrome

    PubMed Central

    Tenenbaum, Ariel; Malkiel, Sarah; Wexler, Isaiah D.; Levy-Khademi, Floris; Revel-Vilk, Shoshana; Stepensky, Polina

    2011-01-01

    Background. Iron deficiency anemia impacts on cognitive development. The objective of this study was to determine the prevalence of anemia and iron deficiency in children with Down syndrome and identify risk factors for anemia. Methods. We conducted a prolective cross-sectional study of children attending a multidisciplinary Down syndrome medical center. One hundred and forty nine children with Down syndrome aged 0–20 years were enrolled in the study. Information obtained included a medical history, physical and developmental examination, nutritional assessment, and the results of blood tests. Results. Of the patients studied, 8.1% were found to have anemia. Among the 38 children who had iron studies, 50.0% had iron deficiency. In a multivariate analysis, Arab ethnicity and low weight for age were significantly associated with anemia. Gender, height, the presence of an eating disorder, and congenital heart disease were not risk factors for anemia. Conclusions. Children with Down syndrome are at risk for anemia and iron deficiency similar to the general population. Children with Down syndrome should be monitored for anemia and iron deficiency so that prompt intervention can be initiated. PMID:21941570

  12. Anemia treatment in chronic renal insufficiency

    Microsoft Academic Search

    Steven Fishbane

    2002-01-01

    Anemia is a common complication of chronic renal insufficiency, one that leads to a reduced quality of life and an increased burden on the heart. In recent years, it has been shown that anemia is underrecognized and undertreated in these patients. The benefits of recombinant human erythropoietin treatment in this patient population have been well shown. The major side effect,

  13. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2014 CFR

    2014-01-01

    ...Animal Products 2 2014-01-01 2014-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  14. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2011 CFR

    2011-01-01

    ...Animal Products 2 2011-01-01 2011-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  15. The Student with Sickle Cell Anemia.

    ERIC Educational Resources Information Center

    Tetrault, Sylvia M.

    1981-01-01

    Sickle cell anemia is the most common and severe of inherited chronic blood disorders. In the United States, sickle cell anemia is most common among the Black population. Among the most commonly occurring symptoms are: an enlarged spleen, episodes of severe pain, easily contracted infections, skin ulcers, and frequent urination. (JN)

  16. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2013 CFR

    2013-01-01

    ...Animal Products 2 2013-01-01 2013-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  17. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2010 CFR

    2010-01-01

    ...Animal Products 2 2010-01-01 2010-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  18. 9 CFR 311.34 - Anemia.

    Code of Federal Regulations, 2012 CFR

    2012-01-01

    ...Animal Products 2 2012-01-01 2012-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products...DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses of livestock too anemic to produce wholesome...

  19. [Pathology and treatment of anemia in the elderly].

    PubMed

    Takasaki, M; Tsurumi, N; Harada, M; Rokugo, N; Arai, H; Katsunuma, H; Ebihara, Y; Wakasugi, K

    1999-05-01

    An important background characteristic of anemia in the elderly is decrease in hematopoiesis due to aging. Factors influencing hematopoiesis in the elderly include changes in the distribution of hematopoietic tissue, changes in hematopoietic stem cell density and changes in the hematopoietic inductive microenvironment. In the present study, in order to assess changes in the bone marrow with aging, the fat tissue area, uncleated cell-count and cellularity in the bone marrow, in addition to changes in the diameter of the vascular lumen which result primarily from sclerotic changes in the dorsomedial artery of the bone marrow were determined in different age groups. The results revealed that all of the aforementioned factors changed significantly with aging. We also describe on the results of assays of inflammatory cytokines (IL-1, IL-6, TNF-alpha), lactoferrin and transferrin receptors in cases of anemia of chronic disorders (ACD) which own secondary to chronic inflammatory diseases and is known to frequently afflict the elderly. PMID:10466349

  20. Sigma E Regulators Control Hemolytic Activity and Virulence in a Shrimp Pathogenic Vibrio harveyi

    E-print Network

    Rattanama, Pimonsri

    Members of the genus Vibrio are important marine and aquaculture pathogens. Hemolytic activity has been identified as a virulence factor in many pathogenic vibrios including V. cholerae, V. parahaemolyticus, V. alginolyticus, ...

  1. Sigma E Regulators Control Hemolytic Activity and Virulence in a Shrimp Pathogenic Vibrio harveyi

    E-print Network

    Mekalanos, John

    ,764 mutants screened, five mutants showed reduced hemolytic activity on sheep blood agar and exhibited activity in sheep blood agar, and were 3-to 7-fold attenuated for colonization in shrimp. Comparison

  2. Pathology Case Study: Macrocytic Anemia

    NSDL National Science Digital Library

    Bahler, David

    This is a case study presented by the University of Pittsburgh Department of Pathology in which an older man suffering from chronic bronchitis and macrocytic anemia also developed persistent flu symptoms. Visitors view the microscopic and gross descriptions, including images, and have the opportunity to diagnose the patient. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to use to introduce or test student learning in hematopathology.

  3. Ochratoxin A-induced iron deficiency anemia.

    PubMed Central

    Huff, W E; Chang, C F; Warren, M F; Hamilton, P B

    1979-01-01

    Ochratoxin A at 8 micrograms per g of diet, but not at lower doses, fed to chickens from 1 day to 3 weeks of age resulted in significantly (P less than 0.05) decreased packed blood cell volume and hemoglobin concentration without altering the number of circulating erythrocytes. Serum iron and percentage of transferrin saturation were lowered at 4 and 8 micrograms/g. Therefore, anemia was characteristic of severe ochratoxicosis of young chickens, and the anemia was categorized as a hypochromic-microcytic anemia of the iron deficiency type. These data indicate that ochratoxin A by itself does not cause hemorrhagic anemia syndrome of chickens and that an anemia caused by a nutritional deficiency can be elicited by a mycotoxin. PMID:453831

  4. In vitro evaluation of the cytotoxic, hemolytic and clastogenic activities of Rhizostoma pulmo toxin(s).

    PubMed

    Allavena, A; Mariottini, G L; Carli, A M; Contini, S; Martelli, A

    1998-06-01

    Cytotoxic, hemolytic and clastogenic activities of Rhizostoma pulmo toxin(s) contained in the jelly tissue free of nematocysts were investigated in mammalian cells with in vitro procedures. At the concentration of 37.6 microg/ml the tissue protein produced the death of 50% V79 cells; a similar potency was observed in terms of hemolytic activity. The toxin(s) was not clastogenic for human lymphocytes in culture at the concentration of 5 microg/ml. PMID:9663699

  5. The evolution of hemolytic saponin content in wild and cultivated Alfalfa ( Medicago sativa , Fabaceae)

    Microsoft Academic Search

    Ernest Small; Marian Jurzysta; Constance Nozzolillo

    1990-01-01

    Hemolytic saponin content was determined of the leaves of 1213 plants of different variants ofMedicago sativa s.l. (including wild and cultivated alfalfa), and a close ally,M. papillosa. The latter species had a much higher content than any of the groups ofM. sativa. Medicago sativa ssp. caerulea, the most important ancestor of alfalfa, had a very low content of hemolytic saponins.

  6. Effects of co-existing microalgae and grazers on the production of hemolytic toxins in Karenia mikimotoi

    NASA Astrophysics Data System (ADS)

    Yang, Weidong; Zhang, Naisheng; Cui, Weimin; Xu, Yanyan; Li, Hongye; Liu, Jiesheng

    2011-11-01

    Karenia mikimotoi (Miyake & Kominami ex Oda) Hansen & Moestrup is associated with harmful algal blooms in temperate and subtropical zones of the world. The hemolytic substances produced by K. mikimotoi are thought to cause mortality in fishes and invertebrates. We evaluated the composition of the hemolytic toxin produced by K. mikimotoi cultured in the laboratory using thin-layer chromatography. In addition, we evaluated the effect of co-occuring algae ( Prorocentrum donghaiense and Alexandrium tamarense) and the cladoceran grazer Moina mongolica on hemolytic toxin production in K. mikimotoi. The hemolytic toxins from K. mikimotoi were a mixture of 2 liposaccharides and 1 lipid. Waterborne clues from P. donghaiense and A. tamarense inhibited the growth of K. mikimotoi but increased the production of hemolytic toxins. Conversely, K. mikimotoi strongly inhibited the growth of caged P. donghaiense and A. tamarense. In addition, the ingestion of K. mikimotoi by M. mongolica induced the production of hemolytic toxins in K. mikimotoi. Taken together, our results suggest that the presence of other microalgae and grazers may be as important as environmental factors for controlling the production of hemolytic substances. K. mikimotoi secreted allelochemicals other than unstable fatty acids with hemolytic activity. The production of hemolytic toxins in dinoflagellates was not only dependent on resource availability, but also on the risk of predation. Hemolytic toxins likely play an important role as chemical deterrents secreted by K. mikimotoi.

  7. Contribution of malnutrition and malaria to anemia in children in rural communities of Edo state, Nigeria

    PubMed Central

    Osazuwa, Favour; Ayo, Oguntade Michael

    2010-01-01

    Background: The most common cause of anemia is an iron deficiency; however, the condition may also be caused by deficiencies in folate, vitamin B12 and protein. Some anemia is not caused by nutritional factors, but by congenital factors and parasitic diseases such as malaria. Aim: This study attempted to estimate the prevalence of anemia among children in three rural communities of the Ovia North East Local government area, and to determine whether its cause was nutritional or could be attributed to malaria. Patients and Methods: A total of 316 children between the ages of 1 and 15 years were included in the study. Children were examined for malaria parasites by microscopy. The World Health Organization (WHO) age-adjusted cut-off for hemoglobin was used to classify anemia. Results: 38.6% of the children were anemic, with hemoglobin levels lower than 11g/dL, although parasite prevalence and density were low. Malnutrition was patent; 37.0% of the children were stunted, 19.3% wasted and 44.0% underweight. Serum ferritin was more sensitive than hemoglobin concentration in detecting anemic children. Anemia was also significantly higher in the Evbuomore village school than in the Ekosodin and Isiohor villages (P<0.001). Conclusion: Anemia detected in this population may be due more to malnutrition than to malaria. PMID:22558561

  8. Management of Iron-Deficiency Anemia in Inflammatory Bowel Disease: A Systematic Review.

    PubMed

    Nielsen, Ole Haagen; Ainsworth, Mark; Coskun, Mehmet; Weiss, Günter

    2015-06-01

    Anemia is the most frequent complication of inflammatory bowel disease (IBD), but anemia, mostly due to iron deficiency, has long been neglected in these patients.The aim was to briefly present the pathophysiology, followed by a balanced overview of the different forms of iron replacement available, and subsequently, to perform a systematic review of studies performed in the last decade on the treatment of iron-deficiency anemia in IBD.Given that intravenous therapies have been introduced in the last decade, a systematic review performed in PubMed, EMBASE, the Cochrane Library, and the websites of WHO, FDA, and EMA covered prospective trials investigating the management of iron-deficiency anemia in IBD published since 2004.A total of 632 articles were reviewed, and 13 articles (2906 patients) with unique content were included. In general, oral supplementation in iron-deficiency anemia should be administered with a target to restore/replenish the iron stores and the hemoglobin level in a suitable way. However, in patients with IBD flares and inadequate responses to or side effects with oral preparations, intravenous iron supplementation is the therapy of choice. Neither oral nor intravenous therapy seems to exacerbate the clinical course of IBD, and intravenous iron therapy can be administered even in active disease stages and concomitantly with biologics.In conclusion, because many physicians are in doubt as to how to manage anemia and iron deficiency in IBD, there is a clear need for the implementation of evidence-based recommendations on this matter. Based on the data presented, oral iron therapy should be preferred for patients with quiescent disease stages and trivial iron deficiency anemia unless such patients are intolerant or have an inadequate response, whereas intravenous iron supplementation may be of advantage in patients with aggravated anemia or flares of IBD because inflammation hampers intestinal absorption of iron. PMID:26061331

  9. Eculizumab in Typical Hemolytic Uremic Syndrome (HUS) With Neurological Involvement.

    PubMed

    Pape, Lars; Hartmann, Hans; Bange, Franz Christoph; Suerbaum, Sebastian; Bueltmann, Eva; Ahlenstiel-Grunow, Thurid

    2015-06-01

    In typical hemolytic uremic syndrome (HUS) approximately 25% of patients show central nervous system (CNS) involvement often leading to serious long-term disabilities. We used the C5-complement inhibitor Eculizumab as rescue therapy.From 2011 to 2014, 11 children (median age 22 months, range 11-175) with enterohemorrhagic Escherichia coli-positive HUS requiring dialysis who had seizures (11/11) and/or were in a stupor or coma (10/11) were treated with Eculizumab. Two patients enrolled on the Safety and Efficacy Study of Eculizumab in Shiga-Toxin Producing E coli Hemolytic-Uremic Syndrome (STEC-HUS) each received 6 doses of Eculizumab, 3 patients 2 doses, and 6 patients 1 dose. Laboratory diagnostics of blood samples and magnetic resonance imaging (MRI) were performed as per center practice. Data were analyzed retrospectively.Cranial MRI was abnormal in 8 of 10 patients with findings in the basal ganglia and/or white matter. A 2-year-old boy with severe cardiac involvement and status epilepticus needed repeated cardio-pulmonary resuscitation and extracorporeal membrane oxygenation. He died 8 days after start of Eculizumab treatment. Two patients with hemorrhagic colitis and repeated seizures required artificial ventilation for 6 and 16 days, respectively. At the time of discharge, 1 patient showed severe neurological impairment and 1 mild neurological impairment. The 8 surviving patients experienced no further seizures after the first dose of Eculizumab. Three patients showed mild neurological impairment at discharge, whilst the remaining 5 showed no impairment. The platelets normalized 4 days (median) after the first dose of Eculizumab (range 0-20 days). The mean duration of dialysis after the first dose of Eculizumab was 14.1?±?6.1 days.In children with typical HUS and CNS involvement early use of Eculizumab appears to improve neurological outcome. In severe HUS cases which progress rapidly with multiple organ involvement, late treatment with Eculizumab seems to show less benefit. We speculate that prophylactic Eculizumab therapy before development of neurological symptoms could be advantageous. PMID:26091445

  10. Retrospective cohort study of 205 cases with congenital dyserythropoietic anemia type II: definition of clinical and molecular spectrum and identification of new diagnostic scores.

    PubMed

    Russo, Roberta; Gambale, Antonella; Langella, Concetta; Andolfo, Immacolata; Unal, Sule; Iolascon, Achille

    2014-10-01

    Congenital Dyserythropoietic Anemia II (CDA II) is a rare hyporegenerative anemia of variable degree, whose causative gene is SEC23B. More than 60 causative mutations in 142 independent pedigrees have been described so far. However, the prevalence of the CDA II is probably underestimated, since its clinical spectrum was not yet well-defined and thus it is often misdiagnosed with more frequent clinically-related anemias. This study represents the first meta-analysis on clinical and molecular spectrum of CDA II from the largest cohort of cases ever described. We characterized 41 new cases and 18 mutations not yet associated to CDA II, thus expanding the global series to 205 cases (172 unrelated) and the total number of causative variants to 84. The 68.3% of patients are included in our International Registry of CDA II (Napoli, Italy). A genotype-phenotype correlation in three genotypic groups of patients was assessed. To quantify the degree of severity in each patient, a method based on ranking score was performed. We introduced a clinical index to easily discriminate patients with a well-compensated hemolytic anemia from those with ineffective erythropoiesis. Finally, the worldwide geographical distribution of SEC23B alleles highlighted the presence of multiple founder effects in different areas of the world. PMID:25044164

  11. Homozygosity mapping of Fanconi anemia

    SciTech Connect

    Gschwend, M.; Botstein, D. [Stanford Univ., CA (United States); Kruglyak, L. [Whitehead Institute, Cambridge, MA (United States)] [and others

    1994-09-01

    Fanconi anemia (FA) is a rare, recessive, genetically heterogeneous disease characterized by progressive insufficiency of the bone marrow and increased cellular sensitivity to DNA crosslinking agents. Complementation tests among different FA cells have indicated the presence of at least 4 FA-causing genes. One of the genes, FACC, was identified by functional complementation but appears unlikely to account for many phenotypically indistinguishable FA caes. We have begun a linkage study of FA using {open_quotes}homozygosity mapping{close_quotes}, a method that involves genotyping with DNA markers on affected individuals whose parents are related. Because FA is a rare recessive disease, it is most likely that probands are homozygous by descent at the disease locus and, therefore, at nearby DNA markers. Although the probability that any given marker will be homozygous in an inbred individual is high, given markers with moderate heterozygosities, the chance that two unrelated inbred individuals will be homozygous at the same marker is considerably lower. By locating overlapping regions of homozygosity between different families we hope to identify genes that cause FA. Sixteen consanguineous non-FACC FA families from the International Fanconi Anemia Registry at Rockefeller University are under study. An efficient algorithm for data analysis was developed and incorporated into software that can quickly compute exact multipoint lod scores using all markers on an entire chromosome. At the time of this writing, 171 of 229 microsatellite markers spaced at 20 cM intervals across the genome have been analyzed.

  12. The CXCR4/CXCR7/SDF-1 pathway contributes to the pathogenesis of Shiga toxin–associated hemolytic uremic syndrome in humans and mice

    PubMed Central

    Petruzziello-Pellegrini, Tania N.; Yuen, Darren A.; Page, Andrea V.; Patel, Sajedabanu; Soltyk, Anna M.; Matouk, Charles C.; Wong, Dennis K.; Turgeon, Paul J.; Fish, Jason E.; Ho, J.J. David; Steer, Brent M.; Khajoee, Vahid; Tigdi, Jayesh; Lee, Warren L.; Motto, David G.; Advani, Andrew; Gilbert, Richard E.; Karumanchi, S. Ananth; Robinson, Lisa A.; Tarr, Phillip I.; Liles, W. Conrad; Brunton, James L.; Marsden, Philip A.

    2012-01-01

    Hemolytic uremic syndrome (HUS) is a potentially life-threatening condition. It often occurs after gastrointestinal infection with E. coli O157:H7, which produces Shiga toxins (Stx) that cause hemolytic anemia, thrombocytopenia, and renal injury. Stx-mediated changes in endothelial phenotype have been linked to the pathogenesis of HUS. Here we report our studies investigating Stx-induced changes in gene expression and their contribution to the pathogenesis of HUS. Stx function by inactivating host ribosomes but can also alter gene expression at concentrations that minimally affect global protein synthesis. Gene expression profiling of human microvascular endothelium treated with Stx implicated a role for activation of CXCR4 and CXCR7 by their shared cognate chemokine ligand (stromal cell–derived factor-1 [SDF-1]) in Stx-mediated pathophysiology. The changes in gene expression required a catalytically active Stx A subunit and were mediated by enhanced transcription and mRNA stability. Stx also enhanced the association of CXCR4, CXCR7, and SDF1 mRNAs with ribosomes. In a mouse model of Stx-mediated pathology, we noted changes in plasma and tissue content of CXCR4, CXCR7, and SDF-1 after Stx exposure. Furthermore, inhibition of the CXCR4/SDF-1 interaction decreased endothelial activation and organ injury and improved animal survival. Finally, in children infected with E. coli O157:H7, plasma SDF-1 levels were elevated in individuals who progressed to HUS. Collectively, these data implicate the CXCR4/CXCR7/SDF-1 pathway in Stx-mediated pathogenesis and suggest novel therapeutic strategies for prevention and/or treatment of complications associated with E. coli O157:H7 infection. PMID:22232208

  13. Hemolytic-uremic syndrome with acute encephalopathy in a pregnant woman infected with epidemic enterohemorrhagic Escherichia coli: characteristic brain images and cytokine profiles.

    PubMed

    Ito, M; Shiozaki, A; Shimizu, M; Saito, S

    2015-05-01

    A food-poisoning outbreak due to enterohemorrhagic Escherichia coli (EHEC) occurred in Toyama, Japan. The case of a 26-year-old pregnant woman with hemolytic-uremic syndrome who developed acute encephalopathy due to EHEC infection after eating raw meat is presented herein. On day 2 following admission, a cesarean section was performed because of a non-reassuring fetal status. Fecal bacterial culture confirmed an O111/O157 superinfection. Intensive care therapies including continuous hemodiafiltration and plasma exchange were performed. After the operation, the patient developed encephalopathy for which steroid pulse therapy was added. Her condition improved gradually and she was discharged 55 days after delivery. PMID:25841635

  14. Erythropoietic agents in the management of cancer patients. Part 1: Anemia, quality of life, and possible effects on survival.

    PubMed

    Smith, Robert E

    2003-01-01

    Erythropoietic agents have been shown to be well tolerated and highly effective in correcting the anemia associated with cancer. Studies aimed at optimizing their use in this regard are ongoing and are evaluating the potential value of early dose intensification through "front-loading" schedules, the feasibility of reducing the frequency of dosing, and the value of early initiation of erythropoietic agents to prevent anemia. The potential for erythropoietic therapy to contribute to survival in cancer patients also is being investigated. Anemia is an independent poor prognostic factor in several cancer settings, such as head-and-neck, lung, and cervical cancer. In patients with B-cell chronic lymphocytic leukemia, it is possible that the period of stable disease--that is, postponement of the need for cytotoxic chemotherapy--may be prolonged when patients are "downstaged" through correction of their anemia. In addition, anemia may affect the patient's quality of life in ways that impact compliance and/or the ability to tolerate therapy. Retrospective analysis of large phase III trials designed to evaluate the effect of erythropoietic agents on anemia in cancer patients has reported a trend toward improved survival among patients who received erythropoietic therapy. Trials to evaluate how correction of anemia in cancer patients might impact survival are ongoing. In this first installment of a two-part article, the author reviews the clinical data supporting the use of erythropoietic agents for the management of the fatigue due to anemia, the effect of anemia control on quality of life, and the evidence available so far that control of such anemia may actually lengthen survival. In the next issue, Dr. Smith will discuss the possible role of erythropoietic agents in neuroprotection and neurotherapy, including cognitive dysfunction in cancer patients. PMID:15334867

  15. Partial ADAMTS13 deficiency in atypical hemolytic uremic syndrome.

    PubMed

    Feng, Shuju; Eyler, Stephen J; Zhang, Yuzhou; Maga, Tara; Nester, Carla M; Kroll, Michael H; Smith, Richard J; Afshar-Kharghan, Vahid

    2013-08-22

    Complement dysregulation leads to atypical hemolytic uremic syndrome (aHUS), while ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. We investigated whether genetic variations in the ADAMTS13 gene partially explain the reduced activity known to occur in some patients with aHUS. We measured complement activity and ADAMTS13 function, and completed mutation screening of multiple complement genes and ADAMTS13 in a large cohort of aHUS patients. In over 50% of patients we identified complement gene mutations. Surprisingly, 80% of patients also carried at least 1 nonsynonymous change in ADAMTS13, and in 38% of patients, multiple ADAMTS13 variations were found. Six of the 9 amino acid substitutions in ADAMTS13 were common single nucleotide polymorphisms; however, 3 variants-A747V, V832M, and R1096H- were rare, with minor allele frequencies of 0.0094%, 0.5%, and 0.32%, respectively. Reduced complement and ADAMTS13 activity (<60% of normal activity) were found in over 60% and 50% of patients, respectively. We concluded that partial ADAMTS13 deficiency is a common finding in aHUS patients and that genetic screening and functional tests of ADAMTS13 should be considered in these patients. PMID:23847193

  16. Gemcitabine-associated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.

    PubMed

    Held-Warmkessel, Jeanne

    2014-09-01

    A patient being treated for metastatic adenocarcinoma of the pancreas presents to the clinic for a routine appointment. A complete blood count reveals hemoglobin of 6.5 g/dl and a platelet count of 30,000 K/mm3 thought to be from the last of many doses of gemcitabine. On assessment, the only complaint was fatigue with no evidence of bleeding or other abnormal physical findings other than pallor. Past medical history includes hypertension managed with three antihypertensive agents. Additional laboratory tests reveal elevated blood urea nitrogen (69 mg/dl), creatinine (2.76 mg/dl), and lactic dehydrogenase (LDH), was well as indirect bilirubin (2.1 mg/dl). The patient is admitted and transfused with packed red blood cells (pRBCs). The next day, the platelet count drops to 9,000 K/mm3 and the hemoglobin increases, appropriately, to 8.9 g/dl. Urinalysis is positive for hemoglobin (+ 3). The peripheral blood smear is positive for schistocytes (fragmented RBCs). A pheresis catheter is placed after the patient was evaluated by a hematologist and a nephrologist. A presumptive diagnosis of thrombotic thrombocytopenic purpura (TTP) with hemolytic uremic syndrome (HUS) was made. PMID:25158661

  17. Management of hemolytic-uremic syndrome in children

    PubMed Central

    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. PMID:24966691

  18. Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation.

    PubMed

    Zuber, J; Le Quintrec, M; Krid, S; Bertoye, C; Gueutin, V; Lahoche, A; Heyne, N; Ardissino, G; Chatelet, V; Noël, L-H; Hourmant, M; Niaudet, P; Frémeaux-Bacchi, V; Rondeau, E; Legendre, C; Loirat, C

    2012-12-01

    Eculizumab (anti-C5) has been sporadically reported as an efficient therapy for atypical hemolytic uremic syndrome (aHUS). However, the lack of series precludes any firm conclusion about the optimal use of anti-C5 for preventing or treating aHUS posttransplant aHUS recurrence. We thoroughly studied 22 renal transplant recipients with aHUS who received off-label therapy with anti-C5, including 12 cases, which have not been reported yet. Nine patients, all carrying a complement genetic abnormality associated with a high risk of aHUS recurrence, received prophylactic anti-C5 therapy to prevent posttransplant recurrence. Eight of them had a successful recurrence-free posttransplant course and achieved a satisfactory graft function, while the remaining patient experienced early arterial thrombosis of the graft. Thirteen renal transplant recipients were given anti-C5 for posttransplant aHUS recurrence. A complete reversal of aHUS activity was obtained in all of them. Importantly, the delay of anti-C5 initiation after the onset of the aHUS episode inversely correlated with the degree of renal function improvement. Three patients in whom anti-C5 was subsequently stopped experienced a relapse. Altogether these data suggest that long-term eculizumab is highly effective for preventing and treating posttransplant aHUS recurrence. Our study also indicates that anti-C5 should be promptly started if a recurrence occurs. PMID:22958221

  19. Immunologic basis and immunoprophylaxis of RhD induced hemolytic disease of the newborn (HDN).

    PubMed

    Payam Khaja Pasha, Roya; Shokri, Fazel

    2008-12-01

    RhD antigen is the most immunogenic and clinically significant antigen of red blood cells after ABO system. It has historically been associated with hemolytic disease of the newborn (HDN) which is now routinely prevented by the administration of polyclonal anti-D immunoglobulin. This management of HDN has proven to be one of the most successful cases of prophylactic treatment based on antibody mediated immune suppression (AMIS). Despite the increasing efficiency of treatment, the mechanism of action of anti-D is not completely defined. There is a widespread interest in obtaining a reliable therapeutic monoclonal anti-D, due to difficulty of maintaining a pool of high titer volunteer donors for plasma collection and also increasing demand for antenatal prophylaxis and safety issues with plasma derived products. Candidate monoclonal anti-D preparations should demonstrate appropriate functionality in both in vitro and in vivo assays comparable to polyclonal anti-D immunoglobulin. These criteria are reviewed in addition to the factors regulating development of D specific immune response in D negative individuals and its suppression in HDN prophylaxis. PMID:19098362

  20. Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.

    PubMed

    Schrezenmeier, Hubert; Körper, Sixten; Höchsmann, Britta

    2015-02-01

    Aplastic anemia is a rare life-threatening bone marrow failure that is characterized by bicytopenia or pancytopenia in the peripheral blood and a hypoplastic or aplastic bone marrow. The patients are at risk of infection and hemorrhage due to neutropenia and thrombocytopenia and suffer from symptoms of anemia. The main treatment approaches are allogeneic stem cell transplantation and immunosuppression. Here, we review current standard immunosuppression and the attempts that have been made in the past two decades to improve results: review of recent developments also reveals that sometimes not only the advent of new drugs, good ideas and well-designed clinical trials decide the progress in the field but also marketing considerations of pharmaceutical companies. Aplastic anemia experts unfortunately had to face the situation that efficient drugs were withdrawn simply for marketing considerations. We will discuss the current options and challenges in first-line treatment and management of relapsing and refractory patients with an emphasis on adult patients. Some promising new approaches are currently under investigation in prospective, randomized trials. PMID:25572607

  1. Anemia

    MedlinePLUS

    ... amount of iron they need. Infants who drink cow's milk in the first year of life are at ... common dietary cause of iron deficiency in infants. Cow’s milk does not have enough of the iron infants ...

  2. Anemia

    MedlinePLUS

    ... up of many parts, including red blood cells, white blood cells, platelets (PLATE-lets), and plasma (the fluid portion of blood). Red blood cells are disc-shaped and look like doughnuts without holes in the center. They carry oxygen and remove ...

  3. Protrusio acetabuli in sickle-cell anemia

    SciTech Connect

    Martinez, S.; Apple, J.S.; Baber, C.; Putman, C.E.; Rosse, W.F.

    1984-04-01

    Of 155 adults with sickle-cell anemia (SS, SC), radiographs of the pelvis or hip demonstrated protrusio acetabuli on at least one side in 14 (3 men and 11 women), as indicated by projection of the acetabular line medial to the ilio-ischial line. All 14 patients had bone changes attributable to sickle-cell anemia, including marrow hyperplasia and osteonecrosis; however, the severity of femoral or acetabular osteonecrosis did not appear directly related to the protrusion. The authors conclude that sickle-cell anemia can predispose to development of protrusio acetabuli.

  4. Rotavirus and hemolytic enteropathogenic Escherichia coli in weanling diarrhea of pigs.

    PubMed Central

    Lecce, J G; Balsbaugh, R K; Clare, D A; King, M W

    1982-01-01

    Since the turn of the century, Escherichia coli has been implicated in the etiology of weanling diarrhea (colibacillosis). However, rotavirus--a virus that destroys enterocytes--has been shown recently to be causally associated with weanling diarrhea of pigs. The role of both rotavirus and hemolytic enteropathogenic E. coli in weanling diarrhea was assessed in this study. Pigs from a closed herd were farrowed and weaned by two markedly different systems: an "intensive care sanitary" system and a "conventional unsanitary" system. Pigs weaned at 3 weeks of age in the sanitary system usually experienced a rotaviral diarrhea about 16 days postweaning. No hemolytic E. coli were detected in feces from these pigs. Peers weaned at the same time by the unsanitary system commenced diarrhea 3 days postweaning. Rotavirus and nonhemolytic E. coli were detected in the feces at the onset of diarrhea and for a few days thereafter. Then, the aerobic fecal flora shifted to nearly pure hemolytic enteropathogenic E. coli. About 10 days later, the diarrhea waned, and the fecal flora shifted back to nonhemolytic E. coli. This hemolytic E. coli shedding pattern could not be duplicated in artificially inoculated sanitary pigs unless they were inoculated with the hemolytic E. coli during a rotaviral-associated diarrhea. Otherwise, the shedding of hemolytic E. coli was fleeting, and the diarrhea, if present, was mild. Pigs developed humoral antibodies to the rotavirus but not to the hemolytic E. coli. We conclude that rotavirus damages the epithelium of the small intestines, which changes the luminal environment to one that favors colonization by enteropathogenic E. coli. Images PMID:6296193

  5. The anemia of chronic disease.

    PubMed

    Lee, G R

    1983-04-01

    The anemia of chronic disease (ACD) is defined as a mild anemia associated with a chronic inflammatory, infectious or neoplastic illness and with a characteristic disturbance of iron metabolism. Many of the findings in ACD can be accounted for by release of a monokine called leukocyte endogenous mediator (LEM), endogenous pyrogen, or interleukin-1. This substance is released from "activated" monocytes. Bacterial endotoxins, certain lymphokines and phagocytic challenges are among the factors stimulating its biosynthesis. LEM induces fever, leukocytosis, biosynthesis. LEM induces fever, leukocytosis, and a variety of biochemical changes, including hypoferremia and alterations in plasma protein synthesis, collectively known as the "acute phase response." It is proposed that ACD results from the long-term elaboration of LEM and that release of this material is the common pathogenetic factor found in the illnesses that are associated with ACD. Some suggestions are made for testing the hypothesis. The hypoferremia associated with ACD is probably caused by defective release of iron from cells--particularly from macrophages, but also from hepatocytes and intestinal epithelium. Two possible mechanisms for this abnormality have been proposed: liberation of lactoferrin from neutrophilic leukocytes and induction of apoferritin synthesis. Neither mechanism has been established. Erythrokinetic studies in ACD have detected a modest reduction of erythrocyte survival without an adequate compensatory increase in the rate of red cell production. The reduced erythrocyte survival is probably related to an increase in phagocytic activity by activated macrophages. Impaired bone marrow response is partly related to the restricted iron supply, but there is substantial evidence for an additional defect in erythropoietin secretion. In some malignant diseases, there is evidence of an additional abnormality: impaired marrow response to a normal amount of erythropoietin. The nature of the erythropoietic defects and the relation of LEM to them remain to be established. PMID:6348957

  6. Successful Treatment of Severe Anemia using Erythropoietin in a Jehovah Witness with Non-Hodgkin Lymphoma

    PubMed Central

    Agapidou, Alexandra; Vakalopoulou, Sofia; Papadopoulou, Theodosia; Chadjiaggelidou, Christina; Garypidou, Vasileia

    2014-01-01

    Blood transfusion many times works in a life-saving way when a patient is facing a critical situation. However, some patients, such as Jehovah’s Witnesses, may refuse their administration because it opposes to their religion beliefs. Thus, clinicians are forced to respect patients’ preferences and seek other treatments in order to overcome the obstacle of the transfusion. In 1989, recombinant human erythropoietin (rHuEPO) was approved by the United States Food and Drug Administration (FDA) for the treatment of anemia associated with chronic renal failure. This is an amino acid glycol-protein that stimulates red blood cell production in the same manner as endogenous erythropoietin. Other treatment indications approved by the FDA include anemia due to chronic kidney disease, anemia secondary to zidovudine therapy in patients with human immunodeficiency virus infection, and anemia secondary to cancer chemotherapy. The drug also has been used for many off-label indications. Many Jehovah’s Witnesses have accepted rHuEPO as a treatment option to maintain and enhance erythropoiesis. This paper reports the case of a 57-year-old Jehovah’s Witness man, who was diagnosed with severe anemia due to aggressive non Hodgkin lymphoma and refused transfusion of blood; thanks to the treatment with rHuEPO he has managed to complete chemotherapy and has survived a life threatening situation. PMID:25568760

  7. Successful Treatment of Severe Anemia using Erythropoietin in a Jehovah Witness with Non-Hodgkin Lymphoma.

    PubMed

    Agapidou, Alexandra; Vakalopoulou, Sofia; Papadopoulou, Theodosia; Chadjiaggelidou, Christina; Garypidou, Vasileia

    2014-11-19

    Blood transfusion many times works in a life-saving way when a patient is facing a critical situation. However, some patients, such as Jehovah's Witnesses, may refuse their administration because it opposes to their religion beliefs. Thus, clinicians are forced to respect patients' preferences and seek other treatments in order to overcome the obstacle of the transfusion. In 1989, recombinant human erythropoietin (rHuEPO) was approved by the United States Food and Drug Administration (FDA) for the treatment of anemia associated with chronic renal failure. This is an amino acid glycol-protein that stimulates red blood cell production in the same manner as endogenous erythropoietin. Other treatment indications approved by the FDA include anemia due to chronic kidney disease, anemia secondary to zidovudine therapy in patients with human immunodeficiency virus infection, and anemia secondary to cancer chemotherapy. The drug also has been used for many off-label indications. Many Jehovah's Witnesses have accepted rHuEPO as a treatment option to maintain and enhance erythropoiesis. This paper reports the case of a 57-year-old Jehovah's Witness man, who was diagnosed with severe anemia due to aggressive non Hodgkin lymphoma and refused transfusion of blood; thanks to the treatment with rHuEPO he has managed to complete chemotherapy and has survived a life threatening situation. PMID:25568760

  8. Anemia: progress in molecular mechanisms and therapies.

    PubMed

    Sankaran, Vijay G; Weiss, Mitchell J

    2015-03-01

    Anemia is a major source of morbidity and mortality worldwide. Here we review recent insights into how red blood cells (RBCs) are produced, the pathogenic mechanisms underlying various forms of anemia, and novel therapies derived from these findings. It is likely that these new insights, mainly arising from basic scientific studies, will contribute immensely to both the understanding of frequently debilitating forms of anemia and the ability to treat affected patients. Major worldwide diseases that are likely to benefit from new advances include the hemoglobinopathies (?-thalassemia and sickle cell disease); rare genetic disorders of RBC production; and anemias associated with chronic kidney disease, inflammation, and cancer. Promising new approaches to treatment include drugs that target recently defined pathways in RBC production, iron metabolism, and fetal globin-family gene expression, as well as gene therapies that use improved viral vectors and newly developed genome editing technologies. PMID:25742458

  9. Avoiding Anemia: Boost Your Red Blood Cells

    MedlinePLUS

    ... our exit disclaimer . Subscribe Avoiding Anemia Boost Your Red Blood Cells If you’re feeling constantly exhausted ... when your body doesn’t have enough healthy red blood cells. You may either have too few ...

  10. Molecular analysis of hemolytic and phospholipase C activities of Pseudomonas cepacia.

    PubMed Central

    Vasil, M L; Krieg, D P; Kuhns, J S; Ogle, J W; Shortridge, V D; Ostroff, R M; Vasil, A I

    1990-01-01

    By using a gene-specific fragment from the hemolytic phospholipase C (PLC) gene of Pseudomonas aeruginosa as a probe and data from Southern hybridizations under reduced stringency conditions, we cloned a 4.2-kb restriction fragment from a beta-hemolytic Pseudomonas cepacia strain which expressed hemolytic and PLC activities in Escherichia coli under the control of the lac promoter. It was found, by using a T7 phage promoter-directed expression system, that this DNA fragment carries at least two genes. One gene which shares significant DNA homology with both PLC genes from P. aeruginosa encodes a 72-kDa protein, while the other gene encodes a 22-kDa protein. When both genes on the 4.2-kb fragment were expressed from the T7 promoter in the same cell, hemolytic and PLC activities could be detected in the cell lysate. In contrast, when each individual gene was expressed in different cells or when lysates containing the translated products of each separate gene were mixed, neither hemolytic activity nor PLC activity could be detected. Clinical and environmental isolates of P. cepacia were examined for beta-hemolytic activity, PLC activity, sphingomyelinase activity, and reactivity in Southern hybridizations with a probe from P. cepacia which is specific for the larger gene which encodes the 72-kDa protein. There were considerable differences in the ability of the different strains to express hemolytic and PLC activities, and the results of Southern DNA-DNA hybridizations of the genomic DNAs of these strains revealed considerable differences in the probe-reactive fragments between high- and medium-stringency conditions as well as remarkable variation in size and number of probe-reactive fragments among different strains. Analysis of the genomic DNAs from hemolytic and nonhemolytic variants of an individual strain (PC-69) by agarose gel electrophoresis. Southern hybridization, and transverse alternating pulsed field gel electrophoresis suggests that the conversion of the hemolytic phenotype to the nonhemolytic phenotype is associated with either the loss of a large plasmid (greater than 200 kb) or a large deletion of the chromosome of P. cepacia PC-69. Images PMID:2254027

  11. Gametophyte development in Anemia mexicana Klotzsch 

    E-print Network

    Nester, Joan Elizabeth

    1979-01-01

    GAMETOPHYTE DEVELOPMENT IN ANEMIA MEXICANA KLOTZSCH A Thesis by JOAN ELIZABETH NESTER Submitted to the Graduate College of Texas A&M University in partial fulfillment of the requirement for the degree of MASTER OF SCIENCE August 1979 Major... Subject: Botany GAMETOPHYTE DEVELOPMENT IN ANEMIA MEXICANA KLOTZSCH A Thesis by JOAN ELIZABETH NESTER Approved as sty e and content by: rl, udge(~u Chatrman of Committee ember e ber Head o Department August 1979 ABSTRACT Gametophyte Development...

  12. Detection of Phospholipase C in Nontuberculous Mycobacteria and Its Possible Role in Hemolytic Activity

    PubMed Central

    Gomez, Arley; Mve-Obiang, Armand; Vray, Bernard; Rudnicka, Wieslawa; Shamputa, Isdore C.; Portaels, Françoise; Meyers, Wayne M.; Fonteyne, Pierre-Alain; Realini, Laurence

    2001-01-01

    Phospholipase C plays a key role in the pathogenesis of several bacterial infections, for example, those caused by Clostridium perfringens and Listeria monocytogenes. Previous studies have reported multiple copies of plc genes homologous to Pseudomonas aeruginosa plcH and plcN genes encoding the hemolytic and nonhemolytic phospholipase C enzymes in the genomes of Mycobacterium tuberculosis, M. marinum, M. bovis, and M. ulcerans. In this study we analyzed the possible relationship between phospholipase C and hemolytic activity in 21 strains of nontuberculous mycobacteria representing nine different species. Detection of phospholipase C enzymatic activity was carried out using thin-layer chromatography to detect diglycerides in the hydrolysates of radiolabeled phosphatidylcholine. DNA sequences of M. kansasii and M. marinum homologous to the genes encoding phospholipase C from M. tuberculosis and M. ulcerans were identified by DNA-DNA hybridization and sequencing. Finally, we developed a direct and simple assay to detect mycobacterial hemolytic activity. This assay is based on a modified blood agar medium that allows the growth and expression of hemolysis of slow-growing mycobacteria. Hemolytic activity was detected in M. avium, M. intracellulare, M. ulcerans, M. marinum, M. tuberculosis, and M. kansasii mycobacteria with phospholipase C activity, but not in M. fortuitum. No hemolytic activity was detected in M. smegmatis, M. gordonae, and M. vaccae. Whether or not phospholipase C enzyme plays a role in the pathogenesis of nontuberculous mycobacterial diseases needs further investigation. PMID:11283062

  13. Family structure and child anemia in Mexico.

    PubMed

    Schmeer, Kammi K

    2013-10-01

    Utilizing longitudinal data from the nationally-representative Mexico Family Life Survey, this study assesses the association between family structure and iron-deficient anemia among children ages 3-12 in Mexico. The longitudinal models (n = 4649), which control for baseline anemia status and allow for consideration of family structure transitions, suggest that children living in stable-cohabiting and single-mother families and those who have recently experienced a parental union dissolution have higher odds of anemia than those in stable-married, father-present family structures. Interaction effects indicate that unmarried family contexts have stronger associations with anemia in older children (over age five); and, that the negative effects of parental union dissolution are exacerbated in poorer households. Resident maternal grandparents have a significant beneficial effect on child anemia independent of parental family structure. These results highlight the importance of family structure for child micronutrient deficiencies and suggest that understanding social processes within households may be critical to preventing child anemia in Mexico. PMID:23294876

  14. Anemia in elderly hospitalized patients: prevalence and clinical impact.

    PubMed

    Migone De Amicis, Margherita; Poggiali, Erika; Motta, Irene; Minonzio, Francesca; Fabio, Giovanna; Hu, Cinzia; Cappellini, Maria Domenica

    2015-08-01

    Anemia is a common finding in elderly individuals. Several studies have shown a strong relationship between anemia, morbidity and mortality, suggesting anemia as a significant independent predictor of adverse outcome in elderly hospitalized patients. The pathophisiology of anemia in the elderly is not yet completely understood. Several mechanisms are involved. We investigated the prevalence of anemia in a cohort of 193 elderly patients admitted to the Internal Medicine Ward of Ca'Granda Policlinico Hospital along 6 months, and its relationship to comorbidities and to the length of hospitalization. Anemia was classified according to the WHO criteria. The majority of patients (48 %) had a mildmoderate, normocytic anemia; severe anemia was found in 8 out of 92 anemic patients. In a subgroup of patients erythropoietin was tested and resulted statistically higher if compared to non-anemic controls (p = 0.003). Considering the most common cause of anemia, nutritional deficiency, chronic renal disease and anemia of chronic disease were found respectively in 36, 15 and 25 % of cases. Unexplained anemia was diagnosed in 24 % of patients, according to the literature. Anemia was independently associated with increased length of hospital stay. Our study confirmed a high prevalence of anemia in elderly patients, and its association with a higher number of comorbidities and a longer stay. A correct clinical approach to anemia in elderly hospitalized patients is essential, considering its negative impact on patients' quality of life, and its social burden in term of healthcare needs and costs. PMID:25633233

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

    PubMed

    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 < 0.05). Mean duration of hospital stay was 5.34 ± 2.2 days in Group I and 3.53 ± 1.3 days in Group II (p < 0.05). Mean duration of phototherapy was 4.0 ± 1.5 days and 2.73 ± 1.1 days in double and single doses of IVIG respectively, and this was statistically significant (p < 0.05). IVIG therapy didn't decrease neither phototherapy nor hospitalization duration in infants with ABO 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. PMID:24554813

  16. Anti-Rh(c), "little c," isoimmunization: the role of rHuEpo in preventing late anemia.

    PubMed

    Zuppa, Antonio A; Cardiello, Valentina; Alighieri, Giovanni; Cota, Francesco; D'Antuono, Annamaria; Riccardi, Riccardo; Catenazzi, Piero; Romagnoli, Costantino

    2013-08-01

    The overall prevalence of non-Rh-D isoimmunization seems to lie between 0.15% and 1.1%. Anti-Rh(c) alloimmunization, "little c," occurs in 0.07% of pregnancies and shows a quite broad clinical presentation. Late anemia is a frequent problem occurring in the setting of isoimmunization. It occurs more frequently after intrauterine blood transfusions or exsanguinotransfusion, and it can be thought as a hyporegenerative anemia. The authors describe the use of human recombinant erythropoietin in preventing late anemia in a case of anti-Rh(c) isoimmunization. The use of human recombinant erythropoietin is a valid tool for preventing late-onset anemia due to either anti-Rh-D or non-anti-Rh-D isoimmunization. PMID:23073047

  17. Bed bugs reproductive life cycle in the clothes of a patient suffering from Alzheimer's disease results in iron deficiency anemia.

    PubMed

    Sabou, Marcela; Imperiale, Delphine Gallo; Andrès, Emmanuel; Abou-Bacar, Ahmed; Foeglé, Jacinthe; Lavigne, Thierry; Kaltenbach, Georges; Candolfi, Ermanno

    2013-01-01

    We report the case of an 82-year-old patient, hospitalized for malaise. Her clothes were infested by numerous insects and the entomological analysis identified them as being Cimex lectularius (bed bugs). The history of the patient highlighted severe cognitive impairment. The biological assessment initially showed a profound microcytic, aregenerative, iron deficiency anemia. A vitamin B12 deficiency due to pernicious anemia (positive intrinsic factor antibodies) was also highlighted, but this was not enough to explain the anemia without macrocytosis. Laboratory tests, endoscopy and a CT scan eliminated a tumor etiology responsible for occult bleeding. The patient had a mild itchy rash which was linked to the massive colonization by the bed bugs. The C. lectularius bite is most often considered benign because it is not a vector of infectious agents. Far from trivial, a massive human colonization by bed bugs may cause such a hematic depletion that severe microcytic anemia may result. PMID:23673315

  18. Anemia management in patients receiving chronic hemodialysis.

    PubMed

    Thakuria, Mayuri; Ofsthun, Norma J; Mullon, Claudy; Diaz-Buxo, Jose A

    2011-01-01

    Anemia treatment in hemodialysis-dependent (HDD) CKD patients involves adequate supply of iron and an erythropoiesis-stimulating agent (ESA). Despite widespread usage of these agents, there is no generally accepted "standard dosing algorithm" for treating anemia in HDD-CKD patients. The new anemia Quality Incentive Program (QIP) introduced by the Centers for Medicare & Medicaid Services represents a motivation to standardize and harmonize iron and ESA regimens with interactive electronic algorithms and novel modes of deliveries for IV iron and ESA doses. In addition, quality assessment and performance improvement programs at dialysis facilities include achieving measurable improvement in health outcomes, healthcare cost, and reductions in medical errors. Thus, the Corporate Medical Advisory Board for Fresenius Medical Services (FMS) is evaluating an anemia algorithm that will be incorporated into the automated workflow of a new clinical system at FMS clinics. In the future, such systems might communicate with medication pumps incorporated into state-of-the-art HD machines, thereby eliminating manual data entry of medication orders and other potential errors related to data entry or administration of medications such as ESA and IV iron. In addition, the CritLine III TQA Monitor, which allows real-time blood volume, oxygen, and anemia monitoring during HD in acute and chronic settings, may become an integrated diagnostic tool to improve volume and anemia management through better fluid management and ESA dose adjustment algorithms. These novel interactive electronic algorithms, delivery and monitoring methods, and data transfer may be integrated in the Pharmatech process to meet patient-specific anemia therapy. PMID:21999745

  19. Alloantibodies to a paternally derived RBC KEL antigen lead to hemolytic disease of the fetus/newborn in a murine model.

    PubMed

    Stowell, Sean R; Henry, Kate L; Smith, Nicole H; Hudson, Krystalyn E; Halverson, Greg R; Park, Jaekeun C; Bennett, Ashley M; Girard-Pierce, Kathryn R; Arthur, C Maridith; Bunting, Silvia T; Zimring, James C; Hendrickson, Jeanne E

    2013-08-22

    Exposure to nonself red blood cell (RBC) antigens, either from transfusion or pregnancy, may result in alloimmunization and incompatible RBC clearance. First described as a pregnancy complication 80 years ago, hemolytic disease of the fetus and newborn (HDFN) is caused by alloimmunization to paternally derived RBC antigens. Despite the morbidity/mortality of HDFN, women at risk for RBC alloimmunization have few therapeutic options. Given that alloantibodies to antigens in the KEL family are among the most clinically significant, we developed a murine model with RBC-specific expression of the human KEL antigen to evaluate the impact of maternal/fetal KEL incompatibility. After exposure to fetal KEL RBCs during successive pregnancies with KEL-positive males, 21 of 21 wild-type female mice developed anti-KEL alloantibodies; intrauterine fetal anemia and/or demise occurred in a subset of KEL-positive pups born to wild type, but not agammaglobulinemic mothers. Similar to previous observations in humans, pregnancy-associated alloantibodies were detrimental in a transfusion setting, and transfusion-associated alloantibodies were detrimental in a pregnancy setting. This is the first pregnancy-associated HDFN model described to date, which will serve as a platform to develop targeted therapies to prevent and/or mitigate the dangers of RBC alloantibodies to fetuses and newborns. PMID:23801629

  20. Alloantibodies to a paternally derived RBC KEL antigen lead to hemolytic disease of the fetus/newborn in a murine model

    PubMed Central

    Stowell, Sean R.; Henry, Kate L.; Smith, Nicole H.; Hudson, Krystalyn E.; Halverson, Greg R.; Park, Jaekeun C.; Bennett, Ashley M.; Girard-Pierce, Kathryn R.; Arthur, C. Maridith; Bunting, Silvia T.; Zimring, James C.

    2013-01-01

    Exposure to nonself red blood cell (RBC) antigens, either from transfusion or pregnancy, may result in alloimmunization and incompatible RBC clearance. First described as a pregnancy complication 80 years ago, hemolytic disease of the fetus and newborn (HDFN) is caused by alloimmunization to paternally derived RBC antigens. Despite the morbidity/mortality of HDFN, women at risk for RBC alloimmunization have few therapeutic options. Given that alloantibodies to antigens in the KEL family are among the most clinically significant, we developed a murine model with RBC-specific expression of the human KEL antigen to evaluate the impact of maternal/fetal KEL incompatibility. After exposure to fetal KEL RBCs during successive pregnancies with KEL-positive males, 21 of 21 wild-type female mice developed anti-KEL alloantibodies; intrauterine fetal anemia and/or demise occurred in a subset of KEL-positive pups born to wild type, but not agammaglobulinemic mothers. Similar to previous observations in humans, pregnancy-associated alloantibodies were detrimental in a transfusion setting, and transfusion-associated alloantibodies were detrimental in a pregnancy setting. This is the first pregnancy-associated HDFN model described to date, which will serve as a platform to develop targeted therapies to prevent and/or mitigate the dangers of RBC alloantibodies to fetuses and newborns. PMID:23801629

  1. Epoetin beta for the treatment of chemotherapy-induced anemia: an update.

    PubMed

    Galli, Luca; Ricci, Clara; Egan, Colin Gerard

    2015-01-01

    Epoetin beta belongs to the class of erythropoiesis-stimulating agents (ESAs) that are currently available to treat anemic patients receiving chemotherapy. Chemotherapy-induced anemia affects a high percentage of cancer patients and, due to its negative effects on disease outcome and the patient's quality of life, should be treated when first diagnosed. Initial trials with ESAs have shown efficacy in improving quality of life and reducing the need for blood transfusions in patients with chemotherapy-induced anemia. However, recent meta-analyses have provided conflicting data on the impact of ESAs on survival and tumor progression. Here we provide an overview of these recent data and review the role of epoetin beta in the treatment of chemotherapy-induced anemia over the past 20 years. PMID:25784818

  2. Epoetin beta for the treatment of chemotherapy-induced anemia: an update

    PubMed Central

    Galli, Luca; Ricci, Clara; Egan, Colin Gerard

    2015-01-01

    Epoetin beta belongs to the class of erythropoiesis-stimulating agents (ESAs) that are currently available to treat anemic patients receiving chemotherapy. Chemotherapy-induced anemia affects a high percentage of cancer patients and, due to its negative effects on disease outcome and the patient’s quality of life, should be treated when first diagnosed. Initial trials with ESAs have shown efficacy in improving quality of life and reducing the need for blood transfusions in patients with chemotherapy-induced anemia. However, recent meta-analyses have provided conflicting data on the impact of ESAs on survival and tumor progression. Here we provide an overview of these recent data and review the role of epoetin beta in the treatment of chemotherapy-induced anemia over the past 20 years. PMID:25784818

  3. A Novel Quantitative Hemolytic Assay Coupled with Restriction Fragment Length Polymorphisms Analysis Enabled Early Diagnosis of Atypical Hemolytic Uremic Syndrome and Identified Unique Predisposing Mutations in Japan

    PubMed Central

    Yoshida, Yoko; Miyata, Toshiyuki; Matsumoto, Masanori; Shirotani-Ikejima, Hiroko; Uchida, Yumiko; Ohyama, Yoshifumi; Kokubo, Tetsuro; Fujimura, Yoshihiro

    2015-01-01

    For thrombotic microangiopathies (TMAs), the diagnosis of atypical hemolytic uremic syndrome (aHUS) is made by ruling out Shiga toxin-producing Escherichia coli (STEC)-associated HUS and ADAMTS13 activity-deficient thrombotic thrombocytopenic purpura (TTP), often using the exclusion criteria for secondary TMAs. Nowadays, assays for ADAMTS13 activity and evaluation for STEC infection can be performed within a few hours. However, a confident diagnosis of aHUS often requires comprehensive gene analysis of the alternative complement activation pathway, which usually takes at least several weeks. However, predisposing genetic abnormalities are only identified in approximately 70% of aHUS. To facilitate the diagnosis of complement-mediated aHUS, we describe a quantitative hemolytic assay using sheep red blood cells (RBCs) and human citrated plasma, spiked with or without a novel inhibitory anti-complement factor H (CFH) monoclonal antibody. Among 45 aHUS patients in Japan, 24% (11/45) had moderate-to-severe (?50%) hemolysis, whereas the remaining 76% (34/45) patients had mild or no hemolysis (<50%). The former group is largely attributed to CFH-related abnormalities, and the latter group has C3-p.I1157T mutations (16/34), which were identified by restriction fragment length polymorphism (RFLP) analysis. Thus, a quantitative hemolytic assay coupled with RFLP analysis enabled the early diagnosis of complement-mediated aHUS in 60% (27/45) of patients in Japan within a week of presentation. We hypothesize that this novel quantitative hemolytic assay would be more useful in a Caucasian population, who may have a higher proportion of CFH mutations than Japanese patients. PMID:25951460

  4. What Are the Signs and Symptoms of Rh Incompatibility?

    MedlinePLUS

    ... Blood and Marrow Stem Cell Transplant Heart Failure Hemolytic Anemia Send a link to NHLBI to someone by ... In a baby, the condition can lead to hemolytic anemia . Hemolytic anemia is a condition in which red ...

  5. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  6. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  7. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  8. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  9. 21 CFR 500.27 - Methylene blue-containing drugs for use in animals.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...methylene blue cause Heinz body hemolytic anemia in cats when used according to label...animals. (ii) The Heinz body hemolytic anemia reaction to methylene blue has also...Heinz bodies) and associated hemolytic anemia is unclear. (2) The...

  10. Anemia among school children in eastern Nepal.

    PubMed

    Khatiwada, Saroj; Gelal, Basanta; Gautam, Sharad; Tamang, Man Kumar; Shakya, Prem Raj; Lamsal, Madhab; Baral, Nirmal

    2015-06-01

    Anemia is one of the most common public health problems in developing countries like Nepal. This study was done to find the prevalence of anemia among the children aged 4-13 years in eastern Nepal. A cross-sectional study was conducted in 2012 in four districts (Morang, Udayapur, Bhojpur and Ilam) of eastern Nepal to find the prevalence of anemia among the school children of eastern Nepal. Children aged 4-13 years were selected randomly from different schools of above districts and 618 venous blood samples were collected. Hemoglobin level was estimated by using cyanmethemoglobin method. The mean hemoglobin level was 12.2?±?1.82?gm/dl. About 37.9% (n?=?234) children were found anemic. Anemia prevalence was 42.4% (n?=?78), 31.6% (n?=?60), 45.3% (n?=?48) and 34.8% (n?=?48) among school children of Morang, Udayapur, Bhojpur and Ilam district, respectively. The study finds anemia as a significant health problem among the school children of eastern Nepal. PMID:25828831

  11. For Parents of Children with Diamond Blackfan Anemia

    MedlinePLUS

    ... Disorders For Parents of Children with Diamond Blackfan Anemia Parenting Corner Q&A When your child is ... Starlight Children’s Foundation www.starlight.org Diamond Blackfan Anemia Foundation (DBAF) http://www.dbafoundation.org/ DBA Nurse ...

  12. What Are the Signs and Symptoms of Fanconi Anemia?

    MedlinePLUS

    ... What Are the Signs and Symptoms of Fanconi Anemia? Major Signs and Symptoms Your doctor may suspect ... sisters also should be tested for the disorder. Anemia The most common symptom of all types of ...

  13. What Are the Signs and Symptoms of Aplastic Anemia?

    MedlinePLUS

    ... What Are the Signs and Symptoms of Aplastic Anemia? Lower than normal numbers of red blood cells, ... most of the signs and symptoms of aplastic anemia. Signs and Symptoms of Low Blood Cell Counts ...

  14. Probing vasoocclusion phenomena in sickle cell anemia via mesoscopic simulations

    PubMed Central

    Lei, Huan; Karniadakis, George E.

    2013-01-01

    Vasoocclusion crisis is a key hallmark of sickle cell anemia. Although early studies suggest that this crisis is caused by blockage of a single elongated cell, recent experiments have revealed that vasoocclusion is a complex process triggered by adhesive interactions among different cell groups in multiple stages. However, the quantification of the biophysical characteristics of sickle cell anemia remains an open issue. Based on dissipative particle dynamics, we develop a multiscale model for the sickle red blood cells (SS-RBCs), accounting for diversity in both shapes and cell rigidities, to investigate the precise mechanism of vasoocclusion. First, we investigate the adhesive dynamics of a single SS-RBC in shear flow and static conditions, and find that the different cell groups (SS2: young-deformable SS-RBCs, ISCs: rigid-irreversible SS-RBCs) exhibit heterogeneous adhesive behavior due to the diverse cell morphologies and membrane rigidities. We quantify the observed adhesion behavior (in static conditions) in terms of a balance of free energies due to cell adhesion and deformation, and propose a power law that relates the free-energy increase as a function of the contact area. We further simulate postcapillary flow of SS-RBC suspensions with different cell fractions. The more adhesive SS2 cells interact with the vascular endothelium and trap ISC cells, resulting in vasoocclusion in vessels less than depending on the hematocrit. Under inflammation, adherent leukocytes may also trap ISC cells, resulting in vasoocclusion in even larger vessels. PMID:23798393

  15. Probing vasoocclusion phenomena in sickle cell anemia via mesoscopic simulations.

    PubMed

    Lei, Huan; Karniadakis, George E

    2013-07-01

    Vasoocclusion crisis is a key hallmark of sickle cell anemia. Although early studies suggest that this crisis is caused by blockage of a single elongated cell, recent experiments have revealed that vasoocclusion is a complex process triggered by adhesive interactions among different cell groups in multiple stages. However, the quantification of the biophysical characteristics of sickle cell anemia remains an open issue. Based on dissipative particle dynamics, we develop a multiscale model for the sickle red blood cells (SS-RBCs), accounting for diversity in both shapes and cell rigidities, to investigate the precise mechanism of vasoocclusion. First, we investigate the adhesive dynamics of a single SS-RBC in shear flow and static conditions, and find that the different cell groups (SS2: young-deformable SS-RBCs, ISCs: rigid-irreversible SS-RBCs) exhibit heterogeneous adhesive behavior due to the diverse cell morphologies and membrane rigidities. We quantify the observed adhesion behavior (in static conditions) in terms of a balance of free energies due to cell adhesion and deformation, and propose a power law that relates the free-energy increase as a function of the contact area. We further simulate postcapillary flow of SS-RBC suspensions with different cell fractions. The more adhesive SS2 cells interact with the vascular endothelium and trap ISC cells, resulting in vasoocclusion in vessels less than 12-14 ?m depending on the hematocrit. Under inflammation, adherent leukocytes may also trap ISC cells, resulting in vasoocclusion in even larger vessels. PMID:23798393

  16. Hemophagocytosis causes a consumptive anemia of inflammation

    PubMed Central

    Zoller, Erin E.; Lykens, Jennifer E.; Terrell, Catherine E.; Aliberti, Julio; Filipovich, Alexandra H.; Henson, Peter M.

    2011-01-01

    Cytopenias of uncertain etiology are commonly observed in patients during severe inflammation. Hemophagocytosis, the histological appearance of blood-eating macrophages, is seen in the disorder hemophagocytic lymphohistiocytosis and other inflammatory contexts. Although it is hypothesized that these phenomena are linked, the mechanisms facilitating acute inflammation-associated cytopenias are unknown. We report that interferon ? (IFN-?) is a critical driver of the acute anemia observed during diverse microbial infections in mice. Furthermore, systemic exposure to physiologically relevant levels of IFN-? is sufficient to cause acute cytopenias and hemophagocytosis. Demonstrating the significance of hemophagocytosis, we found that IFN-? acts directly on macrophages in vivo to alter endocytosis and provoke blood cell uptake, leading to severe anemia. These findings define a unique pathological process of broad clinical and immunological significance, which we term the consumptive anemia of inflammation. PMID:21624938

  17. Diagnosis of fanconi anemia by diepoxybutane analysis.

    PubMed

    Auerbach, Arleen D

    2015-01-01

    Fanconi anemia (FA) is a genetically and phenotypically heterogeneous disorder characterized by congenital malformations, progressive bone marrow failure, and predisposition to cancer, particularly hematological malignancies and solid tumors of the head and neck. The main role of FA proteins is in the repair of DNA interstrand crosslinks (ICLs). FA results from pathogenic variants in at least sixteen distinct genes, causing genomic instability. Although the highly variable phenotype makes accurate diagnosis on the basis of clinical manifestations difficult in some patients, diagnosis based on a profound sensitivity to DNA-crosslinking agents can be used to identify the pre-anemia patient as well as patients with aplastic anemia or leukemia who may or may not have the physical stigmata associated with the syndrome. Diepoxybutane (DEB) analysis is the preferred test for FA because other agents have higher rates of false-positive and false-negative results. © 2015 by John Wiley & Sons, Inc. PMID:25827349

  18. Binding to and killing of human renal epithelial cells by hemolytic P-fimbriated E. coli

    Microsoft Academic Search

    Anna L Trifillis; Michael S Donnenberg; Xiaoling Cui; Robert G Russell; Simon J Utsalo; Harry L T Mobley; John W Warren

    1994-01-01

    Binding to and killing of human renal epithelial cells by hemolytic P-fimbriated E. coli. Acute pyelonephritis is a common invasive infection frequently caused by E. coli that possess P-fimbriae and secrete hemolysin. We have examined the role of P fimbriae and hemolysin in the killing of putative target cells of acute pyelonephritis, that is, human renal epithelial cells (HRPTEC). Cultures

  19. The Hemolytic Enterotoxin HBL Is Broadly Distributed among Species of the Bacillus cereus Group

    Microsoft Academic Search

    BIRGIT M. PRU; RICHARD DIETRICH; BIRGIT NIBLER; ERWIN MARTLBAUER; SIEGFRIED SCHERER

    1999-01-01

    The prevalence of the hemolytic enterotoxin complex HBL was determined in all species of the Bacillus cereus group with the exception of Bacillus anthracis. hblA, encoding the binding subunit B, was detected by PCR and Southern analysis and was confirmed by partial sequencing of 18 strains. The sequences formed two clusters, one including B. cereus and Bacillus thuringiensis strains and

  20. Long-term neurodevelopmental outcome after intrauterine transfusion for the treatment of fetal hemolytic disease

    Microsoft Academic Search

    Lynda Hudon; Kenneth J. Moise; Suzanne E. Hegemier; Reba M. Hill; Alicia A. Moise; E. O’Brian Smith; Robert J. Carpenter

    1998-01-01

    Objective: The aim of the study was to assess the developmental outcome of neonatal survivors of hemolytic disease of the neonate treated with modern intrauterine transfusion techniques. Study Design: In this prospective, observational study, auditory evoked-response tests were performed in the nursery. Neurodevelopmental evaluation with the Gesell Developmental Schedules was performed between 9 and 18 months of corrected age to

  1. The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children

    Microsoft Academic Search

    Chantal Loirat; Patrick Niaudet

    2003-01-01

    We reviewed the literature to analyze the risk of recurrence of hemolytic uremic syndrome (HUS) after renal transplantation in children. Among 118 children transplanted after post-diarrheal (D+) HUS, 1 (0.8%) had recurrence with graft loss. Among 63 children transplanted after HUS not associated with a prodrome of diarrhea (D-) of unknown mechanism, 13 (21%) had recurrence with graft loss. Of

  2. Comparing micellar, hemolytic, and antibacterial properties of di-and tricarboxyl dendritic amphiphiles

    E-print Network

    Falkinham, Joseph

    amphiphiles Bhadreshkumar B. Maisuria a, , Marcelo L. Actis a,à , Shauntrece N. Hardrict a,§ , Joseph O April 2011 Keywords: Dendritic amphiphiles Staphylococcus aureus MRSA Critical micelle concentrations Hemolytic activities a b s t r a c t Homologous dicarboxyl dendritic amphiphiles--RCONHC(CH3)(CH2CH2COOH)2

  3. Conjugated Hyperbilirubinemia in a Child with Streptococcus pneumoniae-associated Hemolytic Uremic Syndrome

    PubMed Central

    Porto, Anthony F.

    2013-01-01

    Conjugated hyperbilirubinemia is a rare complication of hemolytic uremic syndrome (HUS). We report a case of a 2-year-old female with Streptococcus pneumonia-associated HUS (SP+ HUS) who developed severe cholestasis. It is important for pediatric gastroenterologists to be aware of manifestations of HUS, and that although rare, cholestasis can be one of the early findings in patients with SP+ HUS.

  4. Managing Anemia in the Cancer Patient: Old Problems, Future Solutions

    Microsoft Academic Search

    MICHAEL S. GORDON

    Anemia and associated symptoms commonly mani- fest in cancer patients and may have a considerable impact on outcomes. Preliminary studies suggest that overall survival and locoregional control following radi- ation therapy may be compromised by anemia, and recent preliminary data also suggest that anemia may be related to poorer outcomes following chemotherapy. Health-related quality of life of cancer patients is

  5. Short Report Menstruation Does Not Cause Anemia: Endometrial Thickness

    E-print Network

    Lummaa, Virpi

    Short Report Menstruation Does Not Cause Anemia: Endometrial Thickness Correlates Positively for iron-deficiency anemia. This study tested whether normal, premenopausal women's luteal endometrial), and therefore whether a high ET put women at risk for anemia. Endometrial thickness can be con- sidered

  6. Practical approach to the diagnosis and treatment of anemia associated with CKD in elderly.

    PubMed

    Agarwal, Anil K

    2006-11-01

    Anemia is a frequent complication of chronic kidney disease (CKD). Inadequate production of erythropoietin by the failing kidneys leads to decreased stimulation of the bone marrow to produce red blood cells (RBCs). Anemia of CKD develops early and worsens with progressive renal insufficiency. Although over 40% of patients with CKD are anemic, anemia in this population is under-recognized and undertreated. Of considerable importance, anemia is a risk factor for cardiovascular disease and is associated with higher rates of hospitalization and mortality. Despite the availability of erythropoiesis-stimulating proteins (ESPs) to stimulate RBC production in CKD patients, approximately three fourths of patients initiating dialysis have a hemoglobin <11 g/dL. The recognition of anemia of CKD begins with an estimation of glomerular filtration rate (GFR), which can be far lower than a normal serum creatinine might suggest, especially in the elderly and in those with poor nutrition and muscle mass. If GFR is <60 mL/min/1.73 m(2), hemoglobin should be checked. The anemia is diagnosed when the hemoglobin is <12 g/dL in a man or a postmenopausal woman, or <11 g/dL in a premenopausal woman. The cause of anemia should be investigated in these individuals; this can range from erythropoietin deficiency due to CKD, to deficiency of vitamin B(12) and/or folate, iron deficiency, blood loss, inflammation, malignancy, and aluminum intoxication. After other causes of anemia have been excluded, CKD is the most likely etiology, and it should be treated with an ESP. Currently, epoetin alfa and darbepoetin alfa are the only 2 ESPs approved for use in the United States. Extended dosing of ESP has potential advantages for the patient and may also improve resource utilization. Consequently, both agents have been tested for dosing at extended intervals. Adequate iron stores--defined as transferrin saturation >20% and ferritin >100 mg--as well as ESP administration are needed to produce an appropriate increase in hemoglobin. Poor response to treatment with ESP can be due to many factors, including presence of iron deficiency, inflammation, continued blood loss, and hemoglobinopathy. PMID:17098634

  7. Genetic modulation of sickle cell anemia

    SciTech Connect

    Steinberg, M.H. [Univ. of Mississippi School of Medicine, Jackson, MS (United States)

    1995-05-01

    Sickle cell anemia, a common disorder associated with reduced life span of the red blood cell and vasoocclusive events, is caused by a mutation in the {Beta}-hemoglobin gene. Yet, despite this genetic homogeneity, the phenotype of the disease is heterogeneous. This suggests the modulating influence of associated inherited traits. Some of these may influence the accumulation of fetal hemoglobin, a hemoglobin type that interferes with the polymerization of sickle hemoglobin. Another inherited trait determines the accumulation of {alpha}-globin chains. This review focuses on potential genetic regulators of the phenotype of sickle cell anemia. 125 refs., 6 figs., 3 tabs.

  8. Photosensitivity, abnormal porphyrin profile, and sideroblastic anemia.

    PubMed

    Lim, H W; Cooper, D; Sassa, S; Dosik, H; Buchness, M R; Soter, N A

    1992-08-01

    Cutaneous photosensitivity in a 43-year-old man with idiopathic sideroblastic anemia associated with an abnormal porphyrin profile is reported. This condition was associated with elevated free erythrocyte porphyrin, plasma protoporphyrin, urine porphyrins (predominantly coproporphyrin), stool porphyrins (predominantly protoporphyrin), decreased ferrochelatase activity, and deletion of portions of the long arms of chromosomes 18 and 20. Five other patients with sideroblastic anemia and abnormal porphyrin profiles have been described; all but one of these patients had photosensitivity. The porphyrin profile of this patient is similar to that of three other previously described patients. PMID:1517489

  9. Assessment of phytochemicals, antioxidant, anti-lipid peroxidation and anti-hemolytic activity of extract and various fractions of Maytenus royleanus leaves

    PubMed Central

    2013-01-01

    Background Maytenus royleanus is traditionally used in gastro-intestinal disorders. The aim of this study was to evaluate the methanol extract of leaves and its derived fractions for various antioxidant assays and for its potential against lipid peroxidation and hemolytic activity. Methods Various parameters including scavenging of free-radicals (DPPH, ABTS, hydroxyl and superoxide radical), hydrogen peroxide scavenging, Fe3+ to Fe2+ reducing capacity, total antioxidant capacity, anti-lipid peroxidation and anti-hemolytic activity were investigated. Methanol extract and its derived fractions were also subjected for chemical constituents. LC-MS was also performed on the methanol extract. Results Qualitative analysis of methanol extract exhibited the presence of alkaloids, anthraquinones, cardiac glycosides, coumarins, flavonoids, saponins, phlobatannins, tannins and terpenoids. LC-MS chromatogram indicated the composition of diverse compounds including flavonoids, phenolics and phytoestrogens. Methanol extract, its ethyl acetate and n-butanol fractions constituted the highest amount of total phenolic and flavonoid contents and showed a strong correlation coefficient with the IC50 values for the scavenging of DPPH, hydrogen peroxide radicals, superoxide radicals, anti-lipid peroxidation and anti-hemolytic efficacy. Moreover, n-butanol fraction showed the highest scavenging activity for ABTS radicals and for reduction of Fe3+ to Fe2+. Conclusions Present results suggested the therapeutic potential of Maytenus royleanus leaves, in particular, methanol extract, ethyl acetate and n-butanol fraction as therapeutic agent against free-radical associated damages. The protective potential of the extract and or fraction may be attributed due to the high concentration of phenolic, flavonoid, tannins and terpenoids. PMID:23800043

  10. Endometriosis presenting with hemorrhagic ascites, severe anemia, and shock.

    PubMed

    Morgan, Trent L; Tomich, Eric B; Heiner, Jason D

    2013-01-01

    Hemorrhagic ascites due to endometriosis is an exceedingly uncommon diagnosis rarely reported in the medical literature. We present a case of a 27-year-old woman who presented to the emergency department for flank and neck pain and was found to be hypotensive with massive hemorrhagic ascites and severe anemia. After emergency department resuscitation and hospitalization, her condition was found to be due to complications of endometriosis. A paracentesis of more than 4000 mL of bloody ascitic fluid revealed no evidence of cancer, and she was discharged on hospital day 3 with hormone therapy and no recurrence of symptoms upon outpatient follow-up. This case illustrates the clinical management, diagnostic approach, and underlying etiology of an infrequent but life-threatening complication of endometriosis. PMID:22809773

  11. [Establishing aplastic anemia animal model based on different pathogenesis].

    PubMed

    Zhang, Yu-Chen; Zhao, Ming-Feng

    2015-02-01

    Aplastic anemia(AA) is a disease,including congenital AA and acquired AA, characterized by an extremely hypocellular marrow and peripheral blood pancytopenia due to bone marrow failure. Congenital AA is a autosomal recessive disease due to gene mutation. Persently, acquired AA is recognized as a disease caused by destruction of hematopoietic stem cells, defective marrow microenvironment and aberrant T cellular-immunity. In order to further study its pathogenesis and to choose effective therapeutic target, it has important clinical significance to establish correspondent animal model based on different pathogenesis. This article summarizes the congenital AA amimal models including Fanc A(-/-) mouse, Fanc C(-/-) mouse, Fanc G(-/-) mouse, Fanc D1(-/-)/Fanc 2(-/-) mouse, Fanc D2(-/-) mouse and other gene deficiency mouse AA models, and the acguired AA models resulting from the hematopoietic stem cell decrease, hematopoietic microenvironment injury, immune mediation and combination of hematopoietic stem cell dicrease with immune mediation. PMID:25687089

  12. Iron-deficiency anemia caused by a proton pump inhibitor.

    PubMed

    Hashimoto, Rintaro; Matsuda, Tomoki; Chonan, Akimichi

    2014-01-01

    A 59-year-old man was orally administered rabeprazole, a proton pump inhibitor (PPI), for gastroesophageal reflux disease, after which he gradually developed iron-deficiency anemia. The anemia did not improve following the administration of ferrous fumarate, and endoscopic screening of the entire gastrointestinal tract, including the small intestine, did not reveal any findings indicating the cause of the anemia. The patient was then switched from rabeprazole to famotidine and the anemia was cured within three months. There is much debate as to whether the long-term use of PPIs causes iron-deficiency. However, this case strongly suggests that PPIs can induce iron-deficiency anemia. PMID:25318791

  13. Benign gastric ulceration in pernicious anemia

    Microsoft Academic Search

    J. Reid; T. V. Taylor; S. Holt; R. C. Heading

    1980-01-01

    Summary Benign gastric ulceration occurred in a patient with pernicious anemia in association with aspirin therapy. Previous reports of benign gastric ulceration in patients with achlorhydria are reviewed, and the potential role of aspirin in the pathogenesis of benign ulceration in the absence of acid is discussed.

  14. [Disseminated lymphangiomatosis: a rare cause of anemia].

    PubMed

    Ben Abdallah Chabchoub, R; Kamoun, F; Hidouri, S; Nouri, A; Hachicha, M; Mahfoudh, A

    2015-04-01

    Disseminated lymphangiomatosis is a congenital lymphovenous vascular malformation. It can occur in different regions, some of which are unusual. The treatment of this vascular malformation is based on surgical excision, sclerotherapy, or recombinant interferon therapy. We report the case of disseminated lymphangiomatosis in a 13-year-old girl who presented with anemia. PMID:25725973

  15. Hb F in sickle cell anemia

    Microsoft Academic Search

    A. D. Adekile; T. H. J. Huisman

    1993-01-01

    We have reviewed the methodology for an accurate quantitation of Hb F in the blood of patients with sickle cell anemia, values observed in hundreds of patients of different (racial or ethnic) backgrounds and with differences in severity of the disease, and the various factors that affect the level of Hb F. The latter include sex, age, genetic background or

  16. Bone Marrow Transplantation for Fanconi Anemia

    Microsoft Academic Search

    Eliane Gluckrnan; Arleen D. Auerbach; Mary M. Horowitz; Kathleen A. Sobocinski; Robert C. Ash; Mortimer M. Bortin; Anna Butturini; Bruce M. Carnitta; Richard E. Charnplin; Wilhelrn Friedrich; Robert A. Good; Edward C. Gordon-Smith; Richard E. Harris; John P. Klein; Juan J. Ortega; Ricardo Pasquini; Norma K. C. Rarnsay; Bruno Speck; Marcus R. Vowels; Mei-Jie Zhang; Robert Peter Gale

    1995-01-01

    Fanconi anemia is a genetic disorder associated with diverse congenital abnormalities, progressive bone marrow failure, and increased risk of leukemia and other cancers. Affected persons often die before 30 years of age. Bone marrow trans- plantation is an effective treatment, but there are few data regarding factors associated with transplant outcome. We analyzed outcomes of HLA-identical sibling (N = 151)

  17. Lung function in children with sickle cell anemia.

    PubMed

    Wall, M A; Platt, O S; Strieder, D J

    1979-07-01

    Lung volumes and expiratory flows were measured in 12 children with sickle cell anemia and 12 height-matched black control subjects. Diffusing capacity of the lung for CO, pulmonary capillary blood volume, the membrane component of diffusing capacity, arterial blood gases on breathing room air and 100 per cent O2 were measured in the subjects with sickle cell anemia. The lung volumes and expiratory flows of subjects with sickle cell anemia were no different from those of the control subjects. Diffusing capacity for CO was maintined in the noraml range despite the severe anemia by increases in pulmonary capillary blood volume and the membrane component of diffusing capacity. All subjects with sickle cell anemia had mild hypoxemia and abnormal increases in calculated shunt. Pulmonary function in children with sickle cell anemia appears to be determined by their race and anemia. PMID:464381

  18. Cytomorphometric and cytomorphologic analysis of oral mucosa in children with sickle cell anemia

    PubMed Central

    Paraizo, Juliana Umetsu; Rech, Itauana Aliete Vettorello; Azevedo-Alanis, Luciana Reis; Pianovski, Mara Albonei Dudeque; De Lima, Antonio Adilson Soares; Machado, Maria Ângela Naval

    2013-01-01

    Background: Sickle cell anemia (SCA) is an autosomal recessive genetic disorder, characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs, consequence of vaso occlusive phenomenon and vasculopathy. Several forms of the chronic anemia, consequence of hemolysis, can be associated with oral epithelial cells changes. Exfoliative cytology can be used to detect real changes in the oral mucosa in SCA. Aims: To evaluate morphometric and morphological changes in oral epithelial cells by exfoliative cytology in children with SCA. Materials and Methods: Oral smears were collected from clinically normal-appearing mucosa by liquid-based exfoliative cytology in 20 SCA children (SCA group) and 20 healthy children (C group), matched for age and gender. The slides were prepared and stained by the Papanicolaou technique. Cell morphology and cellularity were analyzed and compared by Chi-square test (P < 0.05). Images of 50 cells per slide were captured and the nuclear area (NA) and cytoplasmic area (CA) were analyzed using an image analysis system. The nucleus-to-cytoplasmic area ratio (NA/CA) was calculated. To compare the means of groups SCA and C, the Student's t-test (P < 0.05) was applied to NA and CA; test non-parametric Mann Whitney U (P < 0.05) was used to compare NA/CA. Results: Mean values for SCA and C groups were: NA (69.38 and 59.63 ?m²; P = 0.01); CA (2321.85 and 2185.60 ?m²; P = 0.24); NA/CA (0.03 and 0.02; P = 0.13), respectively. A significant increase in NA for SCA group (P = 0.01) was seen. No morphological differences were found between the groups. There was a predominance of nucleated cells of the superficial layer in the smears of both groups. Class I smears were predominant in both groups. Conclusions: This study revealed that SCA was able to induce significant changes on nuclear area of the oral epithelial cells. PMID:23833399

  19. Clinical efficacy of two forms of intravenous iron--saccharated ferric oxide and cideferron--for iron deficiency anemia.

    PubMed

    Araki, T; Takaai, M; Miyazaki, A; Ohshima, S; Shibamiya, T; Nakamura, T; Yamamoto, K

    2012-12-01

    Over 90% of iron deficiency anemia cases are due to iron deficiency associated with depletion of stored iron or inadequate intake. Parenteral iron supplementation is an important part of the management of anemia, and some kinds of intravenous iron are used. However, few studies have evaluated the clinical efficacy of these drugs. The purpose of this study was to compare and assess the clinical efficacy of two types of intravenous iron injection, saccharated ferric oxide (SFO) and cideferron (CF). Medical records were obtained for 91 unrelated Japanese anemia patients treated with SFO (n = 37) or CF (n = 54) from May 2005 to May 2010 at Gunma University Hospital. Patients treated with blood transfusion, erythropoietin or oral iron were excluded. Hemoglobin (Hb) values measured on day 0, 7 and 14 were used to assess the efficacy of intravenous irons. A significant increase was observed in the mean Hb value by day 14 of administration in both the CF group and SFO group, and the mean Hb increase due to administration of CF for 7 days was comparable to that of SFO for 14 days. Age and sex did not affect improvement of Hb value. CF is fast acting and highly effective compared with SFO for the treatment of iron deficiency anemia. The use of CF may shorten a therapeutic period for iron deficiency anemia, and CF may be feasible for reducing the hospitalization period. PMID:23346769

  20. Grafting synthetic transmembrane units to the engineered low-toxicity ?-hemolysin to restore its hemolytic activity.

    PubMed

    Ui, Mihoko; Harima, Kousuke; Takei, Toshiaki; Tsumoto, Kouhei; Tabata, Kazuhito V; Noji, Hiroyuki; Endo, Sumire; Akiyama, Kimio; Muraoka, Takahiro; Kinbara, Kazushi

    2014-12-01

    The chemical modification of proteins to provide desirable functions and/or structures broadens their possibilities for use in various applications. Usually, proteins can acquire new functions and characteristics, in addition to their original ones, via the introduction of synthetic functional moieties. Here, we adopted a more radical approach to protein modification, i.e., the replacement of a functional domain of proteins with alternative chemical compounds to build "cyborg proteins." As a proof of concept model, we chose staphylococcal ?-hemolysin (Hla), which is a well-studied, pore-forming toxin. The hemolytic activity of Hla mutants was dramatically decreased by truncation of the stem domain, which forms a ?-barrel pore in the membrane. However, the impaired hemolytic activity was significantly restored by attaching a pyrenyl-maleimide unit to the cysteine residue that was introduced in the remaining stem domain. In contrast, negatively charged fluorescein-maleimide completely abolished the remaining activity of the mutants. PMID:25267196

  1. Cholelithiasis following Escherichia coli O157?:?H7-associated hemolytic uremic syndrome

    Microsoft Academic Search

    John R. Brandt; Mark W. Joseph; Laurie S. Fouser; Phillip I. Tarr; Israel Zelikovic; Ruth A. McDonald; Ellis D. Avner; Nancy G. McAfee; Sandra L. Watkins

    1998-01-01

    .   Sequelae of Escherichia coli O157?:?H7-associated hemolytic uremic syndrome (HUS) 2?–?3 years following an outbreak in Washington State have been prospectively\\u000a studied to identify predictors of adverse sequelae. Logistic regression analysis was used to examine associations between\\u000a findings in the acute course and long-term renal and gastrointestinal outcomes. Twenty-one percent of patients had gastrointestinal\\u000a sequelae, which included cholelithiasis resulting in

  2. Antioxidant, hemolytic and cytotoxic activities of Senecio species used in traditional medicine of northwestern Argentina.

    PubMed

    Lizarraga, Emilio; Castro, Felipe; Fernández, Francisco; de Lampasona, Marina P; Catalán, César A N

    2012-05-01

    Senecio nutans Sch. Bip., S. viridis var. viridis Phill. and S. spegazzinii Cabrera are native species used in traditional medicine of northwestern Argentina. The total phenolics, flavonoids and caffeoylquinic acids contents, as well as radical scavenging, antioxidant, hemolytic and cytotoxic activities of aqueous extracts (infusion and decoction) of all three species were determined. S. nutans was the most active. The extracts did not show antibacterial activity. Alkaloids were not detected in any of the aqueous extracts of the three studied species. PMID:22799087

  3. Complete genome sequence of the highly hemolytic strain Bacillus cereus F837/76.

    PubMed

    Auger, Sandrine; Galleron, Nathalie; Ségurens, Béatrice; Dossat, Carole; Bolotin, Alexander; Wincker, Patrick; Sorokin, Alexei

    2012-03-01

    Highly hemolytic strain Bacillus cereus F837/76 was isolated in 1976 from a contaminated prostate wound. The complete nucleotide sequence of this strain reported here counts nearly 36,500 single-nucleotide differences from the closest sequenced strain, Bacillus thuringiensis Al Hakam. F827/76 also contains a 10-kb plasmid that was not detected in the Al Hakam strain. PMID:22374959

  4. Complete Genome Sequence of the Highly Hemolytic Strain Bacillus cereus F837/76

    PubMed Central

    Auger, Sandrine; Galleron, Nathalie; Ségurens, Béatrice; Dossat, Carole; Bolotin, Alexander; Wincker, Patrick

    2012-01-01

    Highly hemolytic strain Bacillus cereus F837/76 was isolated in 1976 from a contaminated prostate wound. The complete nucleotide sequence of this strain reported here counts nearly 36,500 single-nucleotide differences from the closest sequenced strain, Bacillus thuringiensis Al Hakam. F827/76 also contains a 10-kb plasmid that was not detected in the Al Hakam strain. PMID:22374959

  5. Bone marrow necrosis – initial presentation in sickle cell anemia

    PubMed Central

    Shafiq, Maria; Ali, Natasha

    2013-01-01

    Patient: Male, 20 Final Diagnosis: Sickle cell anemia Symptoms: Bone marrow necrosis • bone pain • fever • hepatomegaly • icterus • splenomegaly • weakness Medication: — Clinical Procedure: — Specialty: Hematology Objective: Unusual clinical course Background: In sickle cell disease, bone involvement is the commonest clinical presentation in the acute as well as chronic setting presenting as painful vaso-occlusive crisis and avascular necrosis, respectively. Other complications include bone marrow necrosis and infarction. Case Report: We report a case of a 20-year-old male who was referred for bone marrow evaluation due to symptoms of fever, weakness, and repeated episodes of bone pains. Bone trephine biopsy revealed multiple areas of central necrosis surrounded by fibroblasts. Conclusions: Recognition of necrosis through bone trephine biopsy is important for early initiation of therapy. PMID:24167641

  6. Pathology Case Study: Anemia, Thrombocytopenia and Renal Insufficiency

    NSDL National Science Digital Library

    Najjar, Hazim

    This clinical immunology case study provided by the University of Pittsburgh Department of Pathology is an excellent resource for students and instructors in the health science fields. This particular case involves the diagnosis of a 68-year-old female admitted due to â??worsening anemia, thrombocytopenia, and renal insufficiency.â? Additional information on the patientâ??s history as well as laboratory results including urine protein electrophoresis, blood tests, and a biopsy were used to diagnose the patient in this case. The official diagnosis of this patient is provided in the â??Final Diagnosisâ? section, and is accompanied by a discussion of the case and a short list of references. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose patientâ??s conditions. It is also a helpful site for educators to use to introduce or test student knowledge of clinical immunology.

  7. [Bacillus cereus septicemia in a patient with severe aplastic anemia].

    PubMed

    Ueda, K; Konishi, M; Maeda, K; Hamada, K; Sakamoto, M; Yoshimoto, E; Majima, T; Mikasa, K; Kita, E; Sano, R; Masutani, T; Narita, N

    1998-12-01

    A 78-year-old female was admitted with complaints of malaise and fatigue in the legs. The patient was diagnosed as severe aplastic anemia and treatment was started with metenolone and steroid pulse therapy. Administration of antibiotics and granulocyte-colony stimulating factor which led to a resolution of the high fever. About four months after admission, the patient developed vomiting and abdominal pain with a spiking fever. The next day after suddenly losing consciousness, she died. B. cereus was isolated from blood cultures. Autopsy specimens of the liver, cardiac muscle and lung showed changes due to B. cereus. This pathogen is widely distributed in nature. We should not overlook B. cereus as a contamination, but rather should consider it a potential pathogen in immunocompromised hosts, when it is isolated from blood cultures. PMID:9916422

  8. 21 CFR 862.1365 - Glutathione test system.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...Glutathione measurements are used in the diagnosis and treatment of certain drug-induced hemolytic (erythrocyte destroying) anemias due to an inherited enzyme deficiency. (b) Classification. Class I (general controls). The device is exempt...

  9. 21 CFR 862.1365 - Glutathione test system.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...Glutathione measurements are used in the diagnosis and treatment of certain drug-induced hemolytic (erythrocyte destroying) anemias due to an inherited enzyme deficiency. (b) Classification. Class I (general controls). The device is exempt...

  10. 21 CFR 862.1365 - Glutathione test system.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...Glutathione measurements are used in the diagnosis and treatment of certain drug-induced hemolytic (erythrocyte destroying) anemias due to an inherited enzyme deficiency. (b) Classification. Class I (general controls). The device is exempt...

  11. Schilling evaluation of pernicious anemia: current status

    SciTech Connect

    Zuckier, L.S.; Chervu, L.R.

    1984-09-01

    The Schilling examination remains a popular means of evaluating in vivo absorption of vitamin B/sub 12/. When absorption is abnormally low, the test may be repeated with addition to exogenous intrinsic factor (IF) in order to correct the IF deficiency that characterizes pernicious anemia. A dual-isotope variation provides a means of performing both stages of the test simultaneously, thereby speeding up the test and reducing dependence on complete urine collection. In vivo studies indicate that, when administered simultaneously, the absorption of unbound B/sub 12/ is elevated, and IF-bound B/sub 12/ is reduced, in pernicious-anemia patients, relative to the classic two-stage examination. A number of clinical studies indicate significant difficulty in resolving clincial diagnoses with the dual-tracer test. An algorithm is offered for selecting the most suitable variation of the Schilling test to improve the accuracy of test results and the ease of performance.

  12. Gametophyte development in Anemia mexicana Klotzsch

    E-print Network

    Nester, Joan Elizabeth

    1979-01-01

    . Anther i di a were first initiated, 1 9 to 27 days after sowing . Antheridia were present on gametophytes with and without notched meristems and were morphologically similar to those of other Anemia species. Some notched gametophytes lacked antheridia... terminated antheridia formation and had begun to form archegonia. After 60 days, six variations in ga ietophyte morphology were displayed: 1. with notched meristem and antheridia, 76%%u, 2. with notched mer istem and archegonia, 6%, 3. with notched...

  13. The relationship of aplastic anemia and PNH

    Microsoft Academic Search

    Neal S. Young; Jaroslaw P. Maciejewski; Elaine Sloand; Guiben Chen; Weihua Zeng; Antonio Risitano; Akira Miyazato

    2002-01-01

    Bone marrow failure has been regarded as one of the triad of clinical manifestations of paroxysmal nocturnal hemoglobinuria\\u000a (PNH), and PNH in turn has been described as a late clonal disease evolving in patients recovering from aplastic anemia. Better\\u000a understanding of the pathophysiology of both diseases and improved tests for cell surface glycosylphosphatidylinositol (GPI)-linked\\u000a proteins has radically altered this view.

  14. Urology and nephrology update: anemia of chronic kidney disease.

    PubMed

    Fiore, David C; Fox, Cara-Louise

    2014-01-01

    Anemia is associated with chronic kidney disease (CKD) at all stages, and it is nearly universal among patients with stage 5 CKD. Nonetheless, anemia of CKD is a diagnosis of exclusion. When anemia is detected in a patient with CKD, etiologies other than CKD must be considered and ruled out. Iron deficiency also is common among patients with CKD, and iron replenishment improves the anemia and the response to erythropoiesis-stimulating agents. Current guidelines for managing anemia of CKD recommend a hemoglobin goal of 11 to 12 g/dL, but lower hemoglobin may be acceptable for asymptomatic patients. Some patients do not benefit from erythropoiesis-stimulating agents, or they lose their responsiveness to treatment and transfusions must be considered. Other agents are being investigated as management for anemia of CKD, with vitamin C (ascorbic acid) showing some promise. PMID:24432707

  15. Recombinant human erythropoietin (r-HuEPO) for the treatment of the anemia of cancer

    Microsoft Academic Search

    R. I. Abels; Kay M. Larholt; Kenneth D. Krantz; Edward C. Bryant

    1991-01-01

    Advanced cancer is frequently associated with a signif- icant anemia that may be due to the disease itself or the effect of concomitantly administered chemotherapeutic agents. In a series of double-blind, placebo-controlled tri- als, three populations of anemic cancer patients were ran- domized to rHuEPO or placebo. The three populations were: A) patients not receiving concomitant chemother- apy, B) patients

  16. A novel ubiquitin ligase is deficient in Fanconi anemia

    Microsoft Academic Search

    Amom Ruhikanta Meetei; Johan P de Winter; Annette L Medhurst; Michael Wallisch; Quinten Waisfisz; Henri J van de Vrugt; Anneke B Oostra; Zhijiang Yan; Chen Ling; Colin E Bishop; Maureen E Hoatlin; Hans Joenje; Weidong Wang

    2003-01-01

    Fanconi anemia is a recessively inherited disease characterized by congenital defects, bone marrow failure and cancer susceptibility. Cells from individuals with Fanconi anemia are highly sensitive to DNA-crosslinking drugs, such as mitomycin C (MMC). Fanconi anemia proteins function in a DNA damage response pathway involving breast cancer susceptibility gene products, BRCA1 and BRCA2 (refs. 1,2). A key step in this

  17. Treatment of Anemia in Inflammatory Bowel Disease– Systematic Review and Meta-Analysis

    PubMed Central

    Avni, Tomer; Bieber, Amir; Steinmetz, Tali; Leibovici, Leonard; Gafter-Gvili, Anat

    2013-01-01

    Background Anemia is considered the most common systemic complication of inflammatory bowel disease (IBD). We aimed to provide all available evidence regarding the safety and efficacy of therapy existing today to correct anemia in IBD. Methods Systematic review and meta-analysis of randomized controlled trials that compared any treatment for anemia in patients with IBD. We searched electronic databases, conference proceedings and clinical trials registries. Two reviewers independently extracted data from included trials. The primary outcome was the effect of treatment for anemia in IBD on the hemoglobin (Hb) response, defined as rate of patients who achieved an increase of 2 g/dl in Hb concentration at the end of the follow-up. Secondary outcomes included disease severity scores, iron indices, Hb levels, inflammatory markers, adverse effects, and mortality. Dichotomous data were analysed by calculating the relative risk (RR) for each trial with the uncertainty in each result being expressed using 95% confidence intervals (CI). A fixed effect model was used, except in the event of significant heterogeneity between the trials (P<0.10, I2>40%), in which we used a random effects model. Results Nine trials fulfilled the inclusion criteria, to a total of 973 patients. We were able to perform meta-analysis for intravenous (IV) versus oral iron and for ESAs versus placebo. IV iron was associated with a higher rate of achieving Hb response in comparison to oral iron; RR 1.25 (95% CI 1.04–1.51, I2?=?2%, 4 trials), CRP levels and disease activity indexes were not significantly affected by IV iron. IV iron was associated with a decrease in adverse events that required discontinuation of intervention and without an increase in serious adverse. Discussion Treatment for anemia in IBD should include IV iron and not oral iron replacement, due to improved Hb response, no added toxicity and no negative effect on disease activity. PMID:24312441

  18. 21 CFR 250.201 - Preparations for the treatment of pernicious anemia.

    Code of Federal Regulations, 2010 CFR

    2010-04-01

    ...Preparations for the treatment of pernicious anemia. 250.201 Section 250.201 Food...Preparations for the treatment of pernicious anemia. (a) The ninth announcement of the Anti-anemia Preparations Advisory Board of the...

  19. 21 CFR 250.201 - Preparations for the treatment of pernicious anemia.

    Code of Federal Regulations, 2014 CFR

    2014-04-01

    ...Preparations for the treatment of pernicious anemia. 250.201 Section 250.201 Food...Preparations for the treatment of pernicious anemia. (a) The ninth announcement of the Anti-anemia Preparations Advisory Board of the...

  20. 21 CFR 250.201 - Preparations for the treatment of pernicious anemia.

    Code of Federal Regulations, 2012 CFR

    2012-04-01

    ...Preparations for the treatment of pernicious anemia. 250.201 Section 250.201 Food...Preparations for the treatment of pernicious anemia. (a) The ninth announcement of the Anti-anemia Preparations Advisory Board of the...

  1. 21 CFR 250.201 - Preparations for the treatment of pernicious anemia.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ...Preparations for the treatment of pernicious anemia. 250.201 Section 250.201 Food...Preparations for the treatment of pernicious anemia. (a) The ninth announcement of the Anti-anemia Preparations Advisory Board of the...

  2. 21 CFR 250.201 - Preparations for the treatment of pernicious anemia.

    Code of Federal Regulations, 2011 CFR

    2011-04-01

    ...Preparations for the treatment of pernicious anemia. 250.201 Section 250.201 Food...Preparations for the treatment of pernicious anemia. (a) The ninth announcement of the Anti-anemia Preparations Advisory Board of the...

  3. ACOG Practice Bulletin No. 95: anemia in pregnancy.

    PubMed

    2008-07-01

    Anemia, the most common hematologic abnormality, is a reduction in the concentration of erythrocytes or hemoglobin in blood. The two most common causes of anemia in pregnancy and the puerperium are iron deficiency and acute blood loss. Iron requirements increase during pregnancy, and a failure to maintain sufficient levels of iron may result in adverse maternal-fetal consequences. The purpose of this document is to provide a brief overview of the causes of anemia in pregnancy, review iron requirements, and provide recommendations for screening and clinical management of anemia during pregnancy. PMID:18591330

  4. Biomarkers for the differentiation of anemia and their clinical usefulness

    PubMed Central

    Northrop-Clewes, Christine A; Thurnham, David I

    2013-01-01

    The World Health Organization defines anemia as the point at which the amount of hemoglobin in the circulation falls below World Health Organization cutoffs for specific age and sex groups. Anemia is a worldwide problem of complex etiology and is associated with many factors. The purpose of this review was to describe the biomarkers used to identify the nature of anemia in patients and in the community. The important biomarkers are the automated red cell counts, tests for nutritional deficiencies, hemoglobinopathies, and inflammation. Diseases are important potential initiators of anemia, but biomarkers of specific diseases are not included in this review, only the underlying feature common to all disease – namely, inflammation. PMID:23687454

  5. Co-assessment of iron, vitamin A and growth status to investigate anemia in preschool children in suburb Chongqing, China

    Microsoft Academic Search

    Ke Chen; Xuan Zhang; Ting-Yu Li; Li Chen; Ping Qu; You-Xue Liu

    2009-01-01

    Background  Anemia is a widespread public health problem, which is due to many factors, nutritional or non-nutritional. Iron, vitamin\\u000a A and growth status were assessed to investigate anemia of preschool children in suburb Chongqing, China.\\u000a \\u000a \\u000a \\u000a Methods  A descriptive, cross-sectional survey was performed on 459 preschool children aged 2 to 7 years randomly chosen from the kindergartens\\u000a in 6 suburban districts of Chongqing.

  6. Epidural anesthesia for laparoscopic cholecystectomy in a patient with sickle cell anemia, beta thalassemia, and Crohn's disease -A case report-

    PubMed Central

    Özlü, Onur

    2012-01-01

    A 37-year-old woman diagnosed with sickle cell anemia (SCA), beta (+) thalassemia, Crohn's disease, and liver dysfunction was scheduled for laparoscopic cholecystectomy (LC) due to acute cholecystitis with gall bladder. Regional anesthesia was performed. An epidural catheter was inserted into the 9-10 thoracal epidural space and then 15 ml of 0.5% bupivacaine was injected through the catheter. The level of sensorial analgesia tested with pinprick test reached up to T4. Here we describe the first case of the combination of sickle cell anemia (SCA), beta (+) thalassemia, and Crohn's disease successful anesthetic management with attention to hemodynamics, particularly with regards to liver dysfunction. PMID:23115690

  7. Acute Fetal Anemia Diagnosed by Middle Cerebral Artery Doppler Velocimetry in Stage V Twin–Twin Transfusion Syndrome

    PubMed Central

    Salcedo, Jennifer; Friedrich, Esther; Wing, Deborah A.; Porto, Manuel

    2011-01-01

    In stage V twin–twin transfusion syndrome (TTTS), up to 50% of surviving twins die or experience permanent disabilities, likely due to acute intertwin hemorrhage resulting in sudden severe anemia of the survivor. Although fetal middle cerebral artery (MCA) Doppler studies demonstrate strong correlation with fetal hemoglobin values, acute hemorrhagic events are more difficult to diagnose, and optimal timing of delivery of the survivor poses an obstetric dilemma. We report a case of newly diagnosed stage V TTTS at 28 weeks gestation, complicated by acute severe anemia diagnosed by significantly abnormal fetal MCA Doppler studies. The anemic twin was urgently delivered and is doing well without significant sequelae. PMID:23705095

  8. [Successful use of "juzen-taiho-to", a kampo medicine, for the treatment of perioperative anemia and hypoalbuminemia].

    PubMed

    Tanaka, Tomohiro; Komasawa, Nobuyasu; Kataiwa, Maiko; Hashimoto, Tsutomu; Ohue, Mutsumi; Minami, Toshiaki

    2014-08-01

    An 88-year-old woman suffering from femoral neck fracture was transported to the emergency room of a hospital. The patient and her family refused transfusion, despite anemia, stating their affiliation with Jehova's Witnesses. Surgery was performed under general anesthesia, and the following day, anemia (hemoglobin, 7.5 g x dl(-1)) and hypoalbuminemia (albumin, 2.7 g x dl(-1)) were observed, in addition to anorexia and general fatigue. The patient underwent nutritional treatment with a kampo medicine (Juzen-taiho-to), which was administered as a medication due to difficulties with swallowing the powdered form. On the 18th day after admission, anemia (hemoglobin, 8.9 g x dl(-1)) and hypoalbuminemia (3.6 g x dl(-1)) improved, as did anorexia and general fatigue. It is thought that the components Shimotsu-to, a component known to improve anemia, and Shikunshi-to, a vital energy supplementing component, were the main ingredients that conferred the improvements in anemia and hypoalbuminemia. These findings suggest that Chinese herbal medicine for the nutritional treatment of the elderly has minimal side effects. PMID:25199335

  9. A first case of congenital TTP on the African continent due to a new homozygous mutation in the catalytic domain of ADAMTS13.

    PubMed

    Meyer, Sara C; Jeddi, Ramzi; Meddeb, Balkis; Gouider, Emna; Lämmle, Bernhard; Kremer Hovinga, Johanna A

    2008-08-01

    Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by occlusive microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia. Homozygous or compound heterozygous mutations in the ADAMTS13 gene result in a congenital severe ADAMTS13 deficiency and subsequent accumulation of ultra-large von Willebrand factor multimers, which tend to form platelet thrombi in the microcirculation. We report a first case of congenital TTP on the African continent with a new, homozygous mutation in the metalloprotease domain of ADAMTS13. An initially oligo-symptomatic presentation was followed by acute exacerbation with ischemic stroke and acute renal failure highlighting the severity of this syndrome. PMID:18443791

  10. [Rapidly progressive ANCA-negative glomerulonephritis in the course of pauci immune microscopic vasculitis with hemolytic anemia probable in the course of Wilson's disease].

    PubMed

    Wieczorek-Surdacka, Ewa; Kaczmarczyk, Ireneusz; Jasik, Piotr; Przepiórkowska-Hoyer, Bernadetta; Su?owicz, W?adys?aw

    2011-01-01

    Pauci-immune glomerulonephritis, i.e., with no evidence of immune deposits in the blood vessel, is the most prevalent form of rapidly progressive glomerulonephritis (RPGN). In the pathogenesis of pauci-immune renal disease inflammation of blood vessels in the presence of circulating anti-neutrophil cytoplasm antibodies (ANCA) takes place. However the lack of ANCA (about 5-30% of patients) does not exclude pauci-immune vasculitis. The patients without circulating ANCA might have fewer extrarenal symptoms than those who are ANCA-positive. We describe a case of a 40-year old women with ANCA-negative renal limited pauci-immune small-vessel vasculitis with rapidly decreasing kidney function. She was ineffectively treated with plasmapheresis combined with a puls of cyclophosphamide (i.v.) and 3 pulses of methyloprednisolone (i.v.). The patient progressed to end-stage renal disease and should be treated with renal replacement therapy. In differential diagnosis we excluded other causes of pauci-immune vasculitis (Churg-Strauss syndrome, Wegener's granulomatosis), vasculitis with immune complexes deposition (systemic lupus erythematosus, Schoenlein-Henoch purpura, post-infection RPGN), Goodpasture disease, haemolytic-uremic syndrome (HUS), disseminated intravascular coagulation (DIC) and Wilson's disease. PMID:21751523

  11. Vanadium induced erythropoiesis secondary to a hemolytic effect

    SciTech Connect

    Hogan, G.R.

    1988-01-01

    Vanadium (V) has received considerable experimental attention because of its possible role as an essential micronutrient in certain mammalian and avian species, a contributor to environmental toxicity through its mobilization by fuel combustion, and an agent promoting variable functional responses of vulnerable target tissues. In regard to the latter, V has been reported to be accumulated in critical organs involved in the processes of red blood cell production and release, i.e., erythropoiesis. Such accumulation and retention may account for the published erythropoietic stimulation in rodents as reflected by increases in the packed red blood cell volume and iron utilization by erythrocyte-producing tissues. Experiments were designed and conducted to test this hypothesis. Radioactive iron incorporation was used as the parameter to determine the erythropoietic effects of V in ICR mice. Test animals received a single intraperitoneal injection of V and were sacrificed at daily intervals for a 12-day period. Data from investigations reported here show that V induced an erythropoietic stimulation. The time course, however, was significantly different compared to responses due to a direct mode of erythropoietic stimulation.

  12. The role of tryptophan residues in the hemolytic activity of stonustoxin,a lethal factor from stonefish (Synanceja horrida) venom.

    PubMed

    Yew, W S; Khoo, H E

    2000-03-01

    Stonustoxin (SNTX) is a pore-forming cytolytic lethal factor, isolated from the venom of the stonefish Synanceja horrida, that has potent hemolytic activity. The role of tryptophan residues in the hemolytic activity of SNTX was investigated. Oxidation of tryptophan residues of SNTX with N-bromosuccinimide (NBS) resulted in loss of hemolytic activity. Binding of 8-anilino-1-naphthalenesulphonate (ANS) to SNTX resulted in occlusion of tryptophan residues that resulted in loss of hemolytic activity. Circular dichroism and fluorescence studies indicated that ANS binding resulted in a conformational change of SNTX, in particular, a relocation of surface tryptophan residues to the hydrophobic interior. NBS-modification resulted in oxidised surface tryptophan residues that did not relocate to the hydrophobic interior. These results suggest that native surface tryptophan residues play a pivotal role in the hemolytic activity of STNX, possibly by being an essential component of a hydrophobic surface necessary for pore-formation. This study is the first report on the essentiality of tryptophan residues in the activity of a lytic and lethal factor from a fish venom. PMID:10863009

  13. Iron, anemia and hepcidin in malaria

    PubMed Central

    Spottiswoode, Natasha; Duffy, Patrick E.; Drakesmith, Hal

    2014-01-01

    Malaria and iron have a complex but important relationship. Plasmodium proliferation requires iron, both during the clinically silent liver stage of growth and in the disease-associated phase of erythrocyte infection. Precisely how the protozoan acquires its iron from its mammalian host remains unclear, but iron chelators can inhibit pathogen growth in vitro and in animal models. In humans, iron deficiency appears to protect against severe malaria, while iron supplementation increases risks of infection and disease. Malaria itself causes profound disturbances in physiological iron distribution and utilization, through mechanisms that include hemolysis, release of heme, dyserythropoiesis, anemia, deposition of iron in macrophages, and inhibition of dietary iron absorption. These effects have significant consequences. Malarial anemia is a major global health problem, especially in children, that remains incompletely understood and is not straightforward to treat. Furthermore, the changes in iron metabolism during a malaria infection may modulate susceptibility to co-infections. The release of heme and accumulation of iron in granulocytes may explain increased vulnerability to non-typhoidal Salmonella during malaria. The redistribution of iron away from hepatocytes and into macrophages may confer host resistance to superinfection, whereby blood-stage parasitemia prevents the development of a second liver-stage Plasmodium infection in the same organism. Key to understanding the pathophysiology of iron metabolism in malaria is the activity of the iron regulatory hormone hepcidin. Hepcidin is upregulated during blood-stage parasitemia and likely mediates much of the iron redistribution that accompanies disease. Understanding the regulation and role of hepcidin may offer new opportunities to combat malaria and formulate better approaches to treat anemia in the developing world. PMID:24910614

  14. Translational efficiency in patients with Diamond-Blackfan anemia

    Microsoft Academic Search

    Jana Cmejlova; Ludmila Dolezalova; Dagmar Pospisilova; Kvetoslava Petrtylova; Jiri Petrak; Radek Cmejla

    2006-01-01

    Background and Objectives. Diamond-Blackfan anemia (DBA) is a rare congenital pure red cell aplasia characterized by normochromic macrocytic anemia, reticulocytopenia, and normocellular bone marrow with a selective deficiency of erythroid precursors. Ribosomal protein S19 (RPS19), currently the only gene associated with DBA, is mutat- ed in 25% of DBA patients, but its role in erythropoiesis is unknown. We attempted to

  15. Congenital dyserythropoietic anemia type II: exclusion of seven candidate genes

    Microsoft Academic Search

    Carmela Lanzara; Romina Ficarella; Angela Totaro; Xin Chen; Roberta Roberto; Silverio Perrotta; Carla Lasalandra; Paolo Gasparini; Achille Iolascon; Massimo Carella

    2003-01-01

    Congenital dyserythropoietic anemias (CDA) are genetic disorders characterized by anemia and ineffective erythropoiesis. Three main types of CDA have been distinguished: CDA I, CDAII and CDA III, whose loci have been already mapped. After the identification of the locus for CDA II, also known as HEMPAS (hereditary erythroblast multinuclearity with positive acidified serum test), on the long arm of chromosome

  16. Current concepts in the pathophysiology and treatment of aplastic anemia

    Microsoft Academic Search

    Neal S. Young; Rodrigo T. Calado; Phillip Scheinberg; Hematology Branch

    2006-01-01

    Aplastic anemia, an unusual hematologic disease, is the paradigm of the human bone marrow failure syndromes. Almost universally fatal just a few decades ago, aplastic anemia can now be cured or ameliorated by stem-cell transplantation or immunosuppressive drug therapy. The pathophysiology is immune mediated in most cases, with activated type 1 cyto- toxic T cells implicated. The molecular basis of

  17. Cardiac Manifestations in Thiamine-Responsive Megaloblastic Anemia Syndrome

    Microsoft Academic Search

    A. Lorber; A. Z. Gazit; A. Khoury; Y. Schwartz; H. Mandel

    2003-01-01

    Thiamine-responsive megaloblastic anemia (TRMA) syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to thiamine treatment. Other features of this syndrome gradually develop. We describe three TRMA patients with heart rhythm abnormalities and structural cardiac anomalies. Eight other reported TRMA patients also had cardiac anomalies. Recently,

  18. X-linked inheritance of Fanconi anemia complementation group B

    Microsoft Academic Search

    Amom Ruhikanta Meetei; Marieke Levitus; Yutong Xue; Annette L Medhurst; Michel Zwaan; Chen Ling; Martin A Rooimans; Patrick Bier; Maureen Hoatlin; Gerard Pals; Johan P de Winter; Weidong Wang; Hans Joenje

    2004-01-01

    Fanconi anemia is an autosomal recessive syndrome characterized by diverse clinical symptoms, hypersensitivity to DNA crosslinking agents, chromosomal instability and susceptibility to cancer. Fanconi anemia has at least 11 complementation groups (A, B, C, D1, D2, E, F, G, I, J, L); the genes mutated in 8 of these have been identified. The gene BRCA2 was suggested to underlie complementation

  19. Neurologic Complications After Allogeneic Marrow Transplantation for Sickle Cell Anemia

    Microsoft Academic Search

    Mark C. Walters; Keith M. Sullivan; Francoise Bernaudin; Gerard Souillet; Jean-Pierre Vannier; F. Leonard Johnson; Carl Lenarsky; Darleen Powars; Nancy Bunin; Kwaku Ohene-Frempong; Donna Wall; G. Michel; E. Plouvier; P. Bodigoni; P. Lutz; Jean E. Sanders; Dana C. Matthews; Frederick R. Appelbaum; Rainer Storb

    1995-01-01

    ARROW transplantation from HLA-identical siblings is effective therapy in children with nonmalignant disorders, including aplastic anemia, congenital immunode- ficiency syndromes, thalassemia major, and certain inborn errors of metabolism.',2 Its use in the treatment of sickle cell anemia has been limited to date but initial reports confirm that bone marrow transplantation is curative treatment for this di~order.~\\

  20. Etiology of Strokes in Children with Sickle Cell Anemia

    ERIC Educational Resources Information Center

    DeBaun, Michael R.; Derdeyn, Colin P.; McKinstry, Robert C., III

    2006-01-01

    The most devastating complication of sickle cell anemia is cerebral infarction, affecting [approximately]30% of all individuals with sickle cell anemia. Despite being one of the most common causes of stroke in infants and children, the mechanism of cerebral infarction in this population has not been extensively studied and is poorly understood.…

  1. Convergence of the Fanconi Anemia and Ataxia Telangiectasia Signaling Pathways

    Microsoft Academic Search

    Toshiyasu Taniguchi; Irene Garcia-Higuera; Bo Xu; Paul R. Andreassen; Richard C. Gregory; Seong-Tae Kim; Michael B. Kastan; Alan D. D'Andrea

    2002-01-01

    Fanconi anemia (FA) and ataxia telangiectasia (AT) are clinically distinct autosomal recessive disorders characterized by spontaneous chromosome breakage and hematological cancers. FA cells are hypersensitive to mitomycin C (MMC), while AT cells are hypersensitive to ionizing radiation (IR). Here, we identify the Fanconi anemia protein, FANCD2, as a link between the FA and ATM damage response pathways. ATM phosphorylates FANCD2

  2. The Fanconi Anemia Polypeptide FACC is Localized to the Cytoplasm

    Microsoft Academic Search

    Takayuki Yamashita; Dwayne L. Barber; Yuan Zhu; Nan Wu; Alan D. D'Andrea

    1994-01-01

    Fanconi anemia (FA) is an autosomal recessive disease characterized by congenital anomalies, aplastic anemia, and chromosomal instability. A cDNA encoding the FA complementation group C (FACC) polypeptide was recently cloned [Strathdee, C. A., Gavish, H., Shannon, W. R. & Buchwald, M. (1992) Nature (London) 356, 763-767]. To further characterize this polypeptide, we generated a rabbit polyclonal antiserum against its carboxyl

  3. Management of Anemia of Inflammation in the Elderly

    PubMed Central

    Macciò, Antonio; Madeddu, Clelia

    2012-01-01

    Anemia of any degree is recognized as a significant independent contributor to morbidity, mortality, and frailty in elderly patients. Among the broad types of anemia in the elderly a peculiar role seems to be played by the anemia associated with chronic inflammation, which remains the most complex form of anemia to treat. The origin of this nonspecific inflammation in the elderly has not yet been clarified. It seems more plausible that the oxidative stress that accompanies ageing is the real cause of chronic inflammation of the elderly and that the same oxidative stress is actually a major cause of this anemia. The erythropoietic agents have the potential to play a therapeutic role in this patient population. Despite some promising results, rHuEPO does not have a specific indication for the treatment of anemia in the elderly. Moreover, concerns about their side effects have spurred the search for alternatives. Considering the etiopathogenetic mechanisms of anemia of inflammation in the elderly population, an integrated nutritional/dietetic approach with nutraceuticals that can manipulate oxidative stress and related inflammation may prevent the onset of this anemia and its negative impact on patients' performance and quality of life. PMID:23091709

  4. Adolescents with sickle-cell anemia deal with life and death.

    PubMed

    LePontois, J

    1975-01-01

    Confusion associated with the developmental problems of adolescence is intensified and prolonged for those suffering from sickle-cell anemia. Feelings of isolation and dependence due to a life-threatening disease can impede the transition to responsible, self-actualizing maturity. Weekly group meetings with nine young women suffering form sickle-cell anemia helped them move toward maturation and a sense of their own competence. The experience of closeness with other adolescents who shared the same vulnerabilities was mutually facilitating. These young women had been caught by a sense that it was impossible to progress to independent maturity, and so became resigned and passive. Yet an inner sense of vitality and their emerging sexuality occasionally sparked rebellion against this helplessness. Sexual senations and potential for family life were available counter-balances against recurrent association with pain and death. Through group support these linkages with hope were strengthened. PMID:1235186

  5. Comparing micellar, hemolytic, and antibacterial properties of di- and tricarboxyl dendritic amphiphiles

    Microsoft Academic Search

    Bhadreshkumar B. Maisuria; Marcelo L. Actis; Shauntrece N. Hardrict; Joseph O. Falkinham; Michael F. Cole; Ronald L. Cihlar; Stephen M. Peters; Richard V. Macri; Eko W. Sugandhi; André A. Williams; Michael A. Poppe; Alan R. Esker; Richard D. Gandour

    2011-01-01

    Homologous dicarboxyl dendritic amphiphiles—RCONHC(CH3)(CH2CH2COOH)2, 4(n); and ROCONHC(CH3)(CH2CH2COOH)2, 5(n), where R=n-CnH2n+1 and n=13–22 carbon atoms—were synthesized. Critical micelle concentrations (CMCs) in aqueous triethanolamine solutions and at pH 7.4 were measured along with hemolytic activity (effective concentrations, EC10) in phosphate-buffered saline (PBS). LogCMC showed a linear dependence on chain length (n); the longest chain in each series had the lowest CMC—in triethanolamine:

  6. Stabilization of lethal and hemolytic activities of box jellyfish (Chironex fleckeri) venom.

    PubMed

    Comis, A; Hartwick, R F; Howden, M E

    1989-01-01

    The stability of both the lethal and hemolytic activities of box jellyfish (Chironex fleckeri) tentacle extract was assessed after various extraction procedures. Both activities were higher when no buffers or water were used during the initial extraction. Also, when the extract was first filtered through a Sep-pak C18 cartridge, the residual lethal titre, after incubation for 24 hr at room temperature, was increased 16-fold and hemolysis was increased 2.6-fold. Evidence for proteolytic activity in the extract was also obtained and monitored by size exclusion HPLC. PMID:2567076

  7. Hydrophilic Modifications of an Amphiphilic Polynorbornene and the Effects on its Hemolytic and Antibacterial Activity

    PubMed Central

    Colak, Semra; Nelson, Christopher F.; Nüsslein, Klaus; Tew, Gregory N.

    2013-01-01

    Here, we report the modification of an amphiphilic antibacterial polynorbornene, Poly3, via incorporation of hydrophilic, biocompatible groups. The sugar, zwitterionic, and polyethylene glycol based moieties were incorporated in varying ratios by copolymerization and post-polymerization techniques. Well-defined copolymers with molecular weights of 3 kDa and narrow polydispersity indices from 1.08 to 1.15 were obtained. The effects of these modifications on the biological activity of these polymers were analyzed by determining their minimum inhibitory concentrations (MIC) and their hemolytic activities (HC50). PMID:19138065

  8. Management and prevention of neonatal anemia: current evidence and guidelines.

    PubMed

    von Lindern, Jeannette S; Lopriore, Enrico

    2014-04-01

    Neonatal anemia is a common disorder, particularly in (very) preterm neonates. Management of neonatal anemia is based principally on red blood cell (RBC) transfusion. Although the use of blood products is nowadays widespread in neonatal medicine, evidence on the potential benefit is extremely limited. Recent studies suggest that RBC transfusions in newborns may be associated with an increased risk for necrotizing enterocolitis, transfer of infectious agents and negative effects on neurodevelopmental outcome. Whether the benefits of RBC transfusions outweigh the risks is controversial and requires further studies. In this review, we summarize the current evidence on the management of neonatal anemia and compare the various international guidelines. In addition, we discuss the various strategies to prevent neonatal anemia and reduce the need for RBC transfusions and discuss important trials currently enrolling patients to improve the management in neonatal anemia. PMID:24524256

  9. Contribution of propylene glycol-induced Heinz body formation to anemia in cats.

    PubMed

    Christopher, M M; Perman, V; Eaton, J W

    1989-04-15

    Propylene glycol (PG) is a common preservative and source of synthetic carbohydrates in soft-moist pet foods. Propylene glycol was fed to cats for 5 weeks at concentrations found in commercial diets (1.6 g/kg of body weight; 12% of diet on a dry-weight basis) and for 3 weeks at concentrations exceeding usual intake (8 g/kg; 41% of diet). There was a dose-dependent increase in Heinz body percentage to 28% in cats fed the low dose of PG and to 92% in cats fed the high dose. Erythrocyte half-life, measured using [14C]-cyanate hemoglobin (Hb), decreased significantly (P less than 0.05) by 18.8% and 60% in cats fed the low and high PG doses, respectively. The PCV in cats fed the low dose was unaffected, whereas cats fed the high dose had a mean (+/- SEM) decrease in PCV from 33.5 +/- 1.05% to 26.3 +/- 1.45%, accompanied by punctate reticulocytosis and bone marrow erythroid hyperplasia. A dose-dependent increase in iron pigment was found in the liver and spleen of all cats. In cats fed the low dose of PG, erythrocyte reduced glutathione concentration actually increased from 7.02 +/- 0.56 to 9.74 +/- 0.69 mumol/g of Hb, but decreased to 2.96 +/- 0.27 mumol/g of Hb in cats fed the high dose. There was no significant increase in methemoglobin concentration. These results indicated that PG cannot be considered innocuous even at concentrations consumed by cats eating commercial diets. Heinz body-induced acceleration of RBC destruction develops in a dose-dependent manner, so that cats with greater food intake, ie, lactating queens and nursing kittens, are at greater risk for development of PG-induced Heinz body hemolytic anemia. PMID:2708106

  10. Microfluidic approach of Sickled Cell Anemia

    NASA Astrophysics Data System (ADS)

    Abkarian, Manouk; Loiseau, Etienne; Massiera, Gladys

    2012-11-01

    Sickle Cell Anemia is a disorder of the microcirculation caused by a genetic point mutation that produces an altered hemoglobin protein called HbS. HbS self-assembles reversibly into long rope like fibers inside the red blood cells. The resulting distorded sickled red blood cells are believed to block the smallest capillaries of the tissues producing anemia. Despite the large amount of work that provided a thorough understanding of HbS polymerization in bulk as well as in intact red blood cells at rest, no consequent cellular scale approaches of the study of polymerization and its link to the capillary obstruction have been proposed in microflow, although the problem of obstruction is in essence a circulatory problem. Here, we use microfluidic channels, designed to mimic physiological conditions (flow velocity, oxygen concentration, hematocrit...) of the microcirculation to carry out a biomimetic study at the cellular scale of sickled cell vaso-occlusion. We show that flow geometry, oxygen concentration, white blood cells and free hemoglobin S are essential in the formation of original cell aggregates which could play a role in the vaso-occlusion events.

  11. [The ability to pharmacological control of anemia and thrombocytopenia in patients with acute coronary syndromes].

    PubMed

    Obrebska, Agnieszka; Kowalski, Jan

    2014-11-01

    Recently, attention has been paid to anemia (decreased number of red blood cells in peripheral blood and less than the normal quantity of hemoglobin) apart from the traditional methods used in determining prognosis in patients with acute cardiac syndrome (ACS). Thrombocytopenia and increased mean platelet volume (MPV) are listed among other--yet simple--laboratory blood tests which have prognostic significance in patients with ACS. In ACS patients it is necessary to distinguish between anemia which is established on admission to hospital (chronic anemia) and that acquired in the hospital (HAA). Anemia diagnosed on admission is an independent predictor of in-hospital mortality, short term and long term. In tum, hospital-acquired anemia is associated with the increase of in-hospital mortality, which further increases with greater decrease in Hb concentration. The prognostic value of Hb concentration was evaluated among others in patients with myocardial infarction. There was no beneficial effect of erythropoietin in patients with STEMI undergoing PCI, on the contrary, the increase of the adverse cardiovascular events and the risk of thromboembolic events was observed in selected groups of patients. The need of the administration of iron preparations is indicated in the case of anemia associated with iron deficiency or due to major bleeding. It was found that in patients with ACS even mild thrombocytopenia increases the risk of bleeding complications and in-hospital mortality, the risk of reinfarction, cardiogenic shock and stroke. Worse prognosis in patients with ACS is also associated with a decreased platelet count on admission to hospital and with increased mean platelet volume. In patients with ACS, we deal with heparin-induced thrombocytopenia or thrombocytopenia caused by the use of antiplatelet agents. The treatment of these causes of thrombocytopenia is an important issue. Increased platelet reactivity is observed in patients with increased MPV. This results in the development of restenosis, worse blood flow after PCI, in the increase in mortality and a higher incidence of the phenomenon of "no-reflow". The use of abciximab in STEMI and NSTEMI was associated with a decrease in MPV. Statins also caused a significant decrease in MPV. PMID:25546993

  12. Effect of amino acid substitution in the staphylococcal peptides warnericin RK and PSM? on their anti-Legionella and hemolytic activities.

    PubMed

    Marchand, Adrienne; Augenstreich, Jacques; Loiseau, Clémence; Verdon, Julien; Lecomte, Sophie; Berjeaud, Jean-Marc

    2015-07-01

    Warnericin RK from Staphylococcus warneri and PSM? from Staphylococcus epidermidis are anti-Legionella peptides which were differently classified in a previous study according to their mode of action. Indeed, warnericin RK is highly hemolytic with a bactericidal mode of action, whereas PSM? is poorly hemolytic with a bacteriostatic mode of action toward L. pneumophila. In order to find anti-Legionella peptides which are not hemolytic, a collection of peptides varying in sequence from warnericin RK to PSM? were designed and synthesized, and their anti-Legionella activities, in terms of growth inhibition, permeabilization, and bactericidal effect, as well as their hemolytic activities, were measured and compared. The results showed that some residues, at position 14 for both peptides for instance, were of major importance for bactericidal and hemolytic activities. PMID:25869678

  13. Isolation and characterization of genetic variability in bacteria with ?-hemolytic and antifungal activity isolated from the rhizosphere of Medicago truncatula plants.

    PubMed

    Hernández-Salmerón, J E; Prieto-Barajas, C M; Valencia-Cantero, E; Moreno-Hagelsieb, G; Santoyo, G

    2014-01-01

    In the present study, we analyzed the frequency of hemolytic and antifungal activities in bacterial isolates from the rhizosphere of Medicago truncatula plants. Of the 2000 bacterial colonies, 96 showed ?-hemolytic activities (frequency, 4.8 x 10(-2)). Hemolytic isolates were analyzed for their genetic diversity by using random amplification of polymorphic DNA, yielding 88 haplotypes. The similarity coefficient of Nei and Li showed a polymorphic diversity ranging from 0.3 to 1. Additionally, 8 of the hemolytic isolates showed antifungal activity toward plant pathogens, Diaporthe phaseolorum, Colletotrichum acutatum, Rhizoctonia solani, and Fusarium oxysporum. The 16S ribosomal sequencing analysis showed that antagonistic bacterial isolates corresponded to Bacillus subtilis (UM15, UM33, UM42, UM49, UM52, and UM91), Bacillus pumilus (UM24), and Bacillus licheniformis (UM88). The present results revealed a higher genetic diversity among hemolytic isolates compared to that of isolates with antifungal action. PMID:25062484

  14. Permeabilizing and hemolytic action of large and small polyene antibiotics on human erythrocytes.

    PubMed Central

    Brajtburg, J; Medoff, G; Kobayashi, G S; Elberg, S; Finegold, C

    1980-01-01

    The effects of large polyenes (heptaenes and the "degenerate heptaene" nystatin) on human erythrocytes were found to occur in three separate stages. Stage I was a reversible increase in cell membrane permeability to monovalent cations and occurred at low antibiotic concentrations. At intermediate antibiotic concentrations, an irreversible increase in cell membrane permeability to cations (stage II) occurred, which then led to swelling of cells and hemolysis (stage III). Hemolysis could be prevented by sucrose, mannitol, or melezitose, but stages I and II still occurred under these conditions. The effects of the small polyenes (pentaenes and a tetraene) occurred in only one stage. Changes in cell membrane permeability (stages I and II) were not noted before hemolysis (stage III) even in the presence of carbohydrate. Carbohydrates gave only weak, transient protection from the hemolytic action of small polyenes, probably because the membrane damage induced by these antibiotics was more extensive than that induced by the large polyenes. In the presence of sucrose, large polyenes were able to inhibit the hemolytic action of small polyenes, implying that both antibiotics have the same binding sites. PMID:7447419

  15. Bone marrow replacement in the treatment of hemolytic disease in mice

    SciTech Connect

    Bernstein, S.E.; Deveau, S.A. (Jackson Lab., Bar Harbor, ME (USA))

    1989-11-01

    Bone marrow replacement therapy following whole-body x- or gamma-irradiation has until now proven to be of limited value in the treatment of individuals with hemolytic disease. The large doses of radiation required for destruction of defective erythropoietic tissues coupled with their resultant high mortality appears to limit its usefulness. Techniques have been developed by the authors to limit the extent of exposure and to improve survival following irradiation. These techniques include shielding of all parts of the body except the hind limbs, prophylactic use of antibiotics, and preparatory blood transfusion to suppress the development of indigenous defective erythrocytes. Using these combined techniques we were able to establish high rates of survival, successful engraftment, and long-term clinical improvement in mice with several hemolytic disorders emanating from hereditary defects in spectrin production and incorporation. Evidence is presented indicating that complete bone marrow replacement occurs even in nonirradiated portions of the erythron and that only donor type red blood cells appear in the circulation.

  16. Primary plate identification of group A beta-hemolytic streptococci utilizing a two-disk technique.

    PubMed Central

    Baron, E J; Gates, J W

    1979-01-01

    A two-disk system is described which allows primary plate identification of group A beta-hemolytic streptococci. Group A beta-hemolytic streptococci could be visualized on primary throat culture plates by using trimethoprim-sulfamethoxazole to inhibit normal flora. In the heavily inoculated area of Trypticase soy agar plates containing 5% sheep blood, a 25-microgram/ml trimethoprim-sulfamethoxazole disk was placed contiguous to a 0.04-U bacitracin disk. A total of 259 throat specimens were examined with this two-disk system. The swabs from these throat specimens were incubated in Todd-Hewitt broth. The bacterial pellet from the broths was stained by fluorescent antibody as a control. Of the cultures that were determined to be positive on the plates, 75% could be read unequivocally after overnight incubation, whereas the remaining 25% required subculture. The plates recovered 91% of the cultures which were considered as true positives by the broth-fluorescent-antibody technique. This method provided a significant savings in time compared with standard plate methods and in cost of materials compared with broth-fluorescent-antibody methods. This technique is particularly valuable for producing rapid results in laboratories where fluorescence microscopy would not be cost-effective. Images PMID:387811

  17. Simplification of an Erythropoiesis Model for Design of Anemia Management Protocols in End Stage Renal Disease

    E-print Network

    Massachusetts at Amherst, University of

    Simplification of an Erythropoiesis Model for Design of Anemia Management Protocols in End Stage be used for anemia management protocol (AMP) design based on formal feedback control methods. · In end of anemia; EPO resistance is often observed. A shortened RBC life-span further contributes to the anemia

  18. Hemolytic crisis

    MedlinePLUS

    ... ask about your medical history and symptoms. The physical exam may occasionally show swelling of the spleen ( splenomegaly ). Tests that may be done include: Blood chemistry panel Complete blood count ( CBC ) Coombs test Haptoglobin ...

  19. Vasculopathy, inflammation, and blood flow in leg ulcers of patients with sickle cell anemia

    PubMed Central

    Minniti, Caterina P.; Delaney, Kara-Marie H.; Gorbach, Alexander M.; Xu, Dihua; Lee, Chyi-Chia Richard; Malik, Nitin; Koroulakis, Antony; Antalek, Matthew; Maivelett, Jordan; Peters-Lawrence, Marlene; Novelli, Enrico M.; Lanzkron, Sophie M.; Axelrod, Karen C.; Kato, Gregory J.

    2013-01-01

    Chronic leg ulcers are frequent and debilitating complications of sickle cell anemia. Inadequate blood supply has been postulated to be an important factor in their occurrence and delayed healing. Little is known about their microcirculatory and histopathological changes. We evaluated the microcirculation of lower extremity ulcers with laser speckle contrast imaging and infrared thermography and obtained clinical and laboratory characteristics in 18 adults with sickle cell anemia and chronic leg ulcers. Skin biopsies were obtained in four subjects. Subjects had markers of severe disease, anemia, high degree of hemolysis, inflammation, and thrombophilia. The highest blood flow was present in the ulcer bed, progressively less in the immediate periwound area, and an unaffected control skin area in the same extremity. Microscopic examination showed evidence of venostasis, inflammation, and vasculopathy. Blood vessels were increased in number, had activated endothelium and evidence of thrombosis/recanalization. High blood flow may be due to chronic inflammation, cutaneous vasodilatation, venostasis, and in situ thrombosis. These changes in skin microcirculation are similar to chronic venous ulcers in the non-sickle cell disease (SCD) population, thus suggesting that leg ulcers may be another end-organ complication with endothelial dysfunction that appears in patients with SCD at a younger age and with higher frequency than in the general population. PMID:23963836

  20. Sociodemographic aspects and quality of life of patients with sickle cell anemia

    PubMed Central

    dos Santos, Juliana Pereira; Gomes Neto, Mansueto

    2013-01-01

    Background Sickle cell anemia is a chronic inherited disease, widespread in the Brazilian population due to the high degree of miscegenation in the country. Despite the high prevalence, there are few studies describing the characteristics of patients and the impact of the disease on quality of life. Objective To describe the sociodemographic profile and the impact of the disease on the quality of life of sickle cell anemia patients. Methods Over 18-year-old patients with sickle cell anemia who attended meetings held by the Associação Baiana de Portadores de Doenças Falciformes, an association for sickle cell anemia patients in Bahia, were interviewed. Sociodemographic data were collected and the generic the Medical Outcomes 36-Item Short-Form Health Survey (SF-36) questionnaire, which is used to assess quality of life, was applied. The analysis of the descriptive statistics was performed using the Statistics Program for the Social Sciences software. Results Thirty-two mostly female (65.6%) patients were interviewed. The mean age was 31.9 ± 12.67 years, 50.0% considered themselves black, 68.8% did not work and 87.5% had per capita income below the poverty line (up to one and a half minimum wages). The SF-36 scores were: limitation by physical aspects 26.56, functional capacity 28.9, emotional aspects 30.20, social aspects, 50.0, pain 50.31, mental health 54.62, general health status 56.09 and vitality 56.71. This shows that the disease has a huge impact on the patients' quality of life. Conclusion The disease interferes in the working capacity of individuals, who mostly have low incomes and impaired access to healthcare services and significantly impacts on their quality of life. PMID:24106440

  1. Rapid progression of acquired amegakaryocytic thrombocytopenia to aplastic anemia.

    PubMed

    King, J A; Elkhalifa, M Y; Latour, L F

    1997-01-01

    Acquired amegakaryocytic thrombocytopenia is a rare disorder characterized by severe thrombocytopenia and selective, marked decrease or absence of megakaryocytes. Although immunosuppressive therapy (prednisone and/or antithymocyte globulin) has been shown to induce remissions in a subset of patients, most patients do not respond, and progression to aplastic anemia occurs in some cases. We report a case of acquired amegakaryocytic thrombocytopenia which, despite aggressive immunosuppressive treatment, rapidly progressed to aplastic anemia. Clinical, laboratory, and immunologic features of our patient's case are described and compared to those of the previously reported six cases that progressed from amegakaryocytic thrombocytopenia to aplastic anemia. PMID:9003837

  2. The Fanconi anemia-BRCA Pathway and Cancer

    Microsoft Academic Search

    Toshiyasu Taniguchi

    \\u000a The Fanconi anemia (FA)-BRCA pathway has emerged as an important pathway in cancer biology. Fanconi anemia (FA) is a rare\\u000a genetic disease characterized by aplastic anemia, developmental defects, cancer susceptibility and cellular hypersensitivity\\u000a to interstrand DNA crosslinking agents. Thirteen FA genes have been identified (FANCA, FANCB, FANCC, FANCD1\\/BRCA2, FANCD2, FANCE, FANCF, FANCG, FANCI, FANCJ\\/BRIP1, FANCL,\\u000a FANCM, and FANCN\\/PALB2). The FA

  3. Anemia management: development of a rapidaccess anemia and intravenous iron service.

    PubMed

    Radia, Deepti; Momoh, Ibrahim; Dillon, Richard; Francis, Yvonne; Cameron, Laura; Fagg, Toni-Lee; Overland, Hannah; Robinson, Susan; Harrison, Claire N

    2013-01-01

    This article describes the initiation and evolution of the Rapid-Access Anemia Clinic (RAAC) at Guy's and St Thomas' Hospitals, London, UK. This clinic was set up to provide diagnosis and treatment, and to coordinate investigative procedures, where necessary, into the underlying causes of anemia. Initially piloted with anemic preoperative orthopedic patients, the clinic now treats a wide range of conditions, deriving from both internal and external referrals. Treatment includes dietary advice, supplementation with iron, vitamin B12 and folate, and blood transfusion. Most patients at the RAAC need iron replacement, the majority of which require intravenous (IV) iron. Therefore the first-line IV iron-administration protocol is carefully considered to ensure viability of the service and patient satisfaction. Four IV irons available in the UK are discussed, with explanation of the benefits and drawbacks of each product and the reasoning behind the IV iron choice at different stages of the RAAC's development. Costs to the service, affected by IV iron price and administration regimen, are considered, as well as the product's contraindications. Finally, the authors reflect on the success of the RAAC and how it has improved patients' quality-of-treatment experience, in addition to benefiting the hospital and National Health Service in achieving specific health-care mandates and directives. Drawing from the authors' experiences, recommendations are given to assist others in setting up and providing a successful rapid-access anemia service or similar facility. PMID:23950666

  4. Anemia management: development of a rapidaccess anemia and intravenous iron service

    PubMed Central

    Radia, Deepti; Momoh, Ibrahim; Dillon, Richard; Francis, Yvonne; Cameron, Laura; Fagg, Toni-Lee; Overland, Hannah; Robinson, Susan; Harrison, Claire N

    2013-01-01

    This article describes the initiation and evolution of the Rapid-Access Anemia Clinic (RAAC) at Guy’s and St Thomas’ Hospitals, London, UK. This clinic was set up to provide diagnosis and treatment, and to coordinate investigative procedures, where necessary, into the underlying causes of anemia. Initially piloted with anemic preoperative orthopedic patients, the clinic now treats a wide range of conditions, deriving from both internal and external referrals. Treatment includes dietary advice, supplementation with iron, vitamin B12 and folate, and blood transfusion. Most patients at the RAAC need iron replacement, the majority of which require intravenous (IV) iron. Therefore the first-line IV iron-administration protocol is carefully considered to ensure viability of the service and patient satisfaction. Four IV irons available in the UK are discussed, with explanation of the benefits and drawbacks of each product and the reasoning behind the IV iron choice at different stages of the RAAC’s development. Costs to the service, affected by IV iron price and administration regimen, are considered, as well as the product’s contraindications. Finally, the authors reflect on the success of the RAAC and how it has improved patients’ quality-of-treatment experience, in addition to benefiting the hospital and National Health Service in achieving specific health-care mandates and directives. Drawing from the authors’ experiences, recommendations are given to assist others in setting up and providing a successful rapid-access anemia service or similar facility. PMID:23950666

  5. Ubiquitylation and the Fanconi anemia pathway.

    PubMed

    Garner, Elizabeth; Smogorzewska, Agata

    2011-09-16

    The Fanconi anemia (FA) pathway maintains genome stability through co-ordination of DNA repair of interstrand crosslinks (ICLs). Disruption of the FA pathway yields hypersensitivity to interstrand crosslinking agents, bone marrow failure and cancer predisposition. Early steps in DNA damage dependent activation of the pathway are governed by monoubiquitylation of FANCD2 and FANCI by the intrinsic FA E3 ubiquitin ligase, FANCL. Downstream FA pathway components and associated factors such as FAN1 and SLX4 exhibit ubiquitin-binding motifs that are important for their DNA repair function, underscoring the importance of ubiquitylation in FA pathway mediated repair. Importantly, ubiquitylation provides the foundations for cross-talk between repair pathways, which in concert with the FA pathway, resolve interstrand crosslink damage and maintain genomic stability. PMID:21605559

  6. How I treat Diamond-Blackfan anemia

    PubMed Central

    Muir, Ellen

    2010-01-01

    Diamond-Blackfan anemia (DBA) is characterized by red cell failure, the presence of congenital anomalies, and cancer predisposition. In addition to being an inherited bone marrow failure syndrome, DBA is also categorized as a ribosomopathy as, in more than 50% of cases, the syndrome appears to result from haploinsufficiency of either a small or large subunit-associated ribosomal protein. Nonetheless, the exact mechanism by which haploinsufficiency results in erythroid failure, as well as the other clinical manifestations, remains uncertain. New knowledge regarding genetic and molecular mechanisms combined with robust clinical data from several international patient registries has provided important insights into the diagnosis of DBA and may, in the future, provide new treatments as well. Diagnostic criteria have been expanded to include patients with little or no clinical findings. Patient management is therefore centered on accurate diagnosis, appropriate use of transfusions and iron chelation, corticosteroids, hematopoietic stem cell transplantation, and a coordinated multidisciplinary approach to these complex patients. PMID:20651069

  7. Assessing Chaos in Sickle Cell Anemia Crises

    NASA Astrophysics Data System (ADS)

    Harris, Wesley; Le Floch, Francois

    2006-11-01

    Recent developments in sickle cell research and blood flow modeling allow for new interpretations of the sickle cell crises. With an appropriate set of theoretical and empirical equations describing the dynamics of the red cells in their environment, and the response of the capillaries to major changes in the rheology, a complete mathematical system has been derived. This system of equations is believed to be of major importance to provide new and significant insight into the causes of the disease and related crises. With simulations, it has been proven that the system transition from a periodic solution to a chaotic one, which illustrates the onset of crises from a regular blood flow synchronized with the heart beat. Moreover, the analysis of the effects of various physiological parameters exposes the potential to control chaotic solutions, which, in turn, could lead to the creation of new and more effective treatments for sickle cell anemia. .

  8. [Cardiopulmonary complications in sickle cell anemia].

    PubMed

    Rojas-Jiménez, Sara; Lopera-Valle, Johan; Yabur-Espítia, Mirna

    2013-01-01

    Sickle cell anemia, considered the most prevalent genetic disease among African Americans, is a disease with autosomal recessive inheritance pattern, characterized by the production of hemoglobin S. This abnormal protein polymerizes and facilitates the formation of fibrillar aggregates that alters the erythrocyte morphology. The stiffness of the red blood cells hinders the adequate transit across microcirculation, leading to hemolysis and increased blood viscosity, which ease thrombogenesis and vascular occlusion, resulting in tissue ischemia and microinfarcts. This disease has a high rate of morbidity and mortality, especially in the first three years of life, when a rapid diagnosis and appropriate treatment are essential. Cardiovascular complications such as heart failure and pulmonary hypertension may develop independently, and each one contributes to increased mortality, being the combination of both risk factors, an important aggravating factor for prognosis and a determinant indicator of mortality. PMID:24215682

  9. Cardiovascular Effect Is Independent of Hemolytic Toxicity of Tentacle-Only Extract from the Jellyfish Cyanea capillata

    PubMed Central

    Qianqian, Wang; Sihua, Liu; Yang, Wang; Guoyan, Liu; Jia, Lu; Xuting, Ye; Liming, Zhang

    2012-01-01

    Our previous studies have confirmed that the crude tentacle-only extract (cTOE) from the jellyfish Cyanea capillata (Cyaneidae) exhibits hemolytic and cardiovascular toxicities simultaneously. So, it is quite difficult to discern the underlying active component responsible for heart injury caused by cTOE. The inactivation of the hemolytic toxicity from cTOE accompanied with a removal of plenty of precipitates would facilitate the separation of cardiovascular component and the investigation of its cardiovascular injury mechanism. In our research, after the treatment of one-step alkaline denaturation followed by twice dialysis, the protein concentration of the treated tentacle-only extract (tTOE) was about 1/3 of cTOE, and SDS-PAGE showed smaller numbers and lower density of protein bands in tTOE. The hemolytic toxicity of tTOE was completely lost while its cardiovascular toxicity was well retained. The observations of cardiac function, histopathology and ultrastructural pathology all support tTOE with significant cardiovascular toxicity. Blood gas indexes and electrolytes changed far less by tTOE than those by cTOE, though still with significant difference from normal. In summary, the cardiovascular toxicity of cTOE can exist independently of the hemolytic toxicity and tTOE can be employed as a better venom sample for further purification and mechanism research on the jellyfish cardiovascular toxic proteins. PMID:22905209

  10. A Comparative Study of the Hemolytic and Cytotoxic Activities of Triterpenoids Isolated from Ginseng and Sea Cucumbers

    Microsoft Academic Search

    A. M. Popov

    2002-01-01

    Specific features of cytotoxic (against tumor cells), hemolytic, and liposomal (effect on permeability) activities of triterpenoids isolated from sea cucumbers and ginseng roots were studied. It was shown that oleanolic acid, protopanaxatriol, and protopanaxadiol at 5 to 20 µg\\/ml inhibited the growth of tumor cells, while at doses up to 100 µg\\/ml, they did not induce hemolysis or changes in

  11. Beta-Hemolytic, Multi-Lancefield Antigen-Agglutinating Enterococcus durans from a Pregnant Woman, Mimicking Streptococcus agalactiae

    PubMed Central

    Franco, Alessia; Gherardi, Giovanni; Marrollo, Roberta; Argentieri, Angela Valentina; Pimentel de Araujo, Fernanda; Amoruso, Roberta; Battisti, Antonio; Fazii, Paolo; Carretto, Edoardo

    2014-01-01

    A beta-hemolytic Lancefield antigen A-, B-, C-, D-, F-, and G-positive Enterococcus durans strain was cultivated from the rectovaginal swab of a pregnant woman who underwent antenatal screening for Streptococcus agalactiae. The isolate raised concern as to what extent similar strains are misrecognized and lead to false diagnosis of group B streptococci. PMID:24671782

  12. Interference by Aerobic and Anaerobic Bacteria in Children With Recurrent Group A b-Hemolytic Streptococcal Tonsillitis

    Microsoft Academic Search

    Itzhak Brook; Alan E. Gober

    1999-01-01

    Objective: To compare the frequency of recovery of aero- bic and anaerobic bacteria with interfering capability of group A b-hemolytic streptococci (GABHS) in the ton- sils of children with and without a history of recurrent GABHS pharyngotonsillitis. Patients and Methods: Tonsillar cultures were taken from a group of 20 children with and 20 without history of recurrent GABHS pharyngotonsillitis. Results:

  13. Rate of eradication of group A beta-hemolytic streptococci in children with pharyngo-tonsillitis by amoxicillin and cefdinir

    Microsoft Academic Search

    Itzhak Brook; Alan E. Gober

    2009-01-01

    BackgroundCephalosporins were found to be more effective than penicillins in the eradication of group A ?-hemolytic streptococcal (GABHS) from tonsillar tissues. This study investigated the effect of amoxicillin and cefdinir therapies on the rate of eradication of GABHS from the tonsils of children with acute pharyngo-tonsillitis (PT).

  14. Duodenal perforation: an unusual complication of sickle cell anemia

    PubMed Central

    Ac?payam, Can; Ald?ç, Güliz; Akçora, Bülent; Çelikkaya, Mehmet Emin; A?kar, Hasan; Dorum, Bayram Ali

    2014-01-01

    Duodenal perforation in childhood is a rare condition with a high mortality rate if not treated surgically. Primary gastroduodenal perforation is frequently associated with peptic ulcer and exhibits a positive family history. Helicobacter pylorus is the most significant agent. Secondary gastroduodenal perforation may be a finding of specific diseases, such as Crohn disease, or more rarely may be associated with diseases such as cystic fibrosis or sickle cell anemia. A 14-year-old boy presented with abdominal and back pain. The patient was operated on for acute abdomen and diagnosed with duodenal perforation. Helicobacter pylorus was negative. There was no risk factor to account for duodenal perforation other than sickle cell anemia. Surgical intervention was successful and without significant sequelae. Duodenal perforation is a rare entity described in patients with sickle cell anemia. To our knowledge, this is the first report of duodenal perforation in a patient sickle cell anemia. PMID:25422692

  15. [Prevalence and treatment of anemia in critically ill patients].

    PubMed

    Muñoz, M; Leal-Noval, S R; García-Erce, J A; Naveira, E

    2007-10-01

    Anemia is a common condition among medical and surgical patients admitted to the intensive care unit (ICU) and generally has a multifactorial origin. In order to avoid the deleterious effects of anemia, 40% of ICU patients receive allogenic blood transfusion (ABT). This figure increases up to 70% if the ICU stay is longer than 7 days. However, ABT is associated with a dose-dependent increase in morbidity and mortality. In contrast, the administration of exogenous erythropoietin plus iron supplements, especially iv iron, improves anemia and reduces ABT requirements, although it does not reduce mortality. To ascertain whether treatment of anemia in the critically ill with exogenous erythropoietin and iron might improve outcomes and to optimize drug administration schedules and dosage, further studies with sufficient statistical power and adequate follow-up are warranted. PMID:17942062

  16. Diphyllobothrium pacificum Infection is Seldom Associated with Megaloblastic Anemia

    PubMed Central

    Jimenez, Juan A.; Rodriguez, Silvia; Gamboa, Ricardo; Rodriguez, Lourdes; Garcia, Hector H.

    2012-01-01

    Twenty cases of Dyphillobothrium pacificum (fish tapeworm) infections were prospectively studied to determine whether this tapeworm is associated with megaloblastic anemia, as commonly reported for D. latum infections. The most frequent symptoms were fatigue and mild abdominal pain, which were identified in approximately 66.6% of the 18 patients interviewed. Fourteen patients received treatment with niclosamide and all were cured. The other six patients spontaneously eliminated the tapeworms. One patient, who also had chronic diabetes and gastric atrophy, had low vitamin B12 levels and megaloblastic anemia. In all other patients, including three other patients with anemia, baseline vitamin B12 levels were in the reference range and did not significantly change when re-assessed three months later. Unlike D. latum, infection with D. pacificum is seldom associated with megaloblastic anemia or vitamin B12 deficit. PMID:22987655

  17. Altered translation of GATA1 in Diamond-Blackfan anemia

    E-print Network

    Ludwig, Leif S

    Ribosomal protein haploinsufficiency occurs in diverse human diseases including Diamond-Blackfan anemia (DBA)[superscript 1, 2], congenital asplenia[superscript 3] and T cell leukemia[superscript 4]. Yet, how mutations in ...

  18. Characterization of Antibacterial and Hemolytic Activity of Synthetic Pandinin 2 Variants and Their Inhibition against Mycobacterium tuberculosis

    PubMed Central

    Rodríguez, Alexis; Villegas, Elba; Montoya-Rosales, Alejandra; Rivas-Santiago, Bruno; Corzo, Gerardo

    2014-01-01

    The contention and treatment of Mycobacterium tuberculosis and other bacteria that cause infectious diseases require the use of new type of antibiotics. Pandinin 2 (Pin2) is a scorpion venom antimicrobial peptide highly hemolytic that has a central proline residue. This residue forms a structural “kink” linked to its pore-forming activity towards human erythrocytes. In this work, the residue Pro14 of Pin2 was both substituted and flanked using glycine residues (P14G and P14GPG) based on the low hemolytic activities of antimicrobial peptides with structural motifs Gly and GlyProGly such as magainin 2 and ponericin G1, respectively. The two Pin2 variants showed antimicrobial activity against E. coli, S. aureus, and M. tuberculosis. However, Pin2 [GPG] was less hemolytic (30%) than that of Pin2 [G] variant. In addition, based on the primary structure of Pin2 [G] and Pin2 [GPG], two short peptide variants were designed and chemically synthesized keeping attention to their physicochemical properties such as hydrophobicity and propensity to adopt alpha-helical conformations. The aim to design these two short antimicrobial peptides was to avoid the drawback cost associated to the synthesis of peptides with large sequences. The short Pin2 variants named Pin2 [14] and Pin2 [17] showed antibiotic activity against E. coli and M. tuberculosis. Besides, Pin2 [14] presented only 25% of hemolysis toward human erythrocytes at concentrations as high as 100 µM, while the peptide Pin2 [17] did not show any hemolytic effect at the same concentration. Furthermore, these short antimicrobial peptides had better activity at molar concentrations against multidrug resistance M. tuberculosis than that of the conventional antibiotics ethambutol, isoniazid and rifampicin. Therefore, Pin2 [14] and Pin2 [17] have the potential to be used as an alternative antibiotics and anti-tuberculosis agents with reduced hemolytic effects. PMID:25019413

  19. Altered translation of GATA1 in Diamond-Blackfan anemia

    PubMed Central

    Ludwig, Leif S.; Gazda, Hanna T.; Eng, Jennifer C.; Eichhorn, Stephen W.; Thiru, Prathapan; Ghazvinian, Roxanne; George, Tracy I.; Gotlib, Jason R.; Beggs, Alan H.; Sieff, Colin A.; Lodish, Harvey F.; Lander, Eric S.; Sankaran, Vijay G.

    2014-01-01

    Ribosomal protein haploinsufficiency occurs in diverse human diseases including Diamond-Blackfan anemia (DBA),1,2 congenital asplenia,3 and T-cell leukemia.4 Yet how mutations in such ubiquitously expressed proteins result in cell-type and tissue specific defects remains a mystery.5 Here, we show that GATA1 mutations that reduce full-length protein levels of this critical hematopoietic transcription factor can cause DBA in rare instances. We show that ribosomal protein haploinsufficiency, the more common cause of DBA, can similarly reduce translation of GATA1 mRNA - a phenomenon that appears to result from this mRNA having a higher threshold for initiation of translation. In primary hematopoietic cells from patients with RPS19 mutations, a transcriptional signature of GATA1 target genes is globally and specifically reduced, confirming that the activity, but not the mRNA level, of GATA1 is reduced in DBA patients with ribosomal protein mutations. The defective hematopoiesis observed in DBA patients with ribosomal protein haploinsufficiency can be at least partially overcome by increasing GATA1 protein levels. Our results provide a paradigm by which selective defects in translation due to mutations in ubiquitous ribosomal proteins can result in human disease. PMID:24952648

  20. Mechanism of vaso-occlusion in sickle cell anemia

    NASA Astrophysics Data System (ADS)

    Lei, Huan; Karniadakis, George

    2012-11-01

    Vaso-occlusion crisis is one of the key hallmark of sickle cell anemia. While early studies suggested that the crisis is caused by blockage of a single elongated cell, recent experimental investigations indicate that vaso-occlusion is a complex process triggered by adhesive interactions among different cell groups in multiple stages. Based on dissipative particle dynamics, a multi-scale model for the sickle red blood cells (SS-RBCs), accounting for diversity in both shapes and cell rigidities, is developed to investigate the mechanism of vaso-occlusion crisis. Using this model, the adhesive dynamics of single SS-RBC was investigated in arterioles. Simulation results indicate that the different cell groups (deformable SS2 RBCs, rigid SS4 RBCs, leukocytes, etc.) exhibit heterogeneous adhesive behavior due to the different cell morphologies and membrane rigidities. We further simulate the tube flow of SS-RBC suspensions with different cell fractions. The more adhesive SS2 cells interact with the vascular endothelium and further trap rigid SS4 cells, resulting in vaso-occlusion in vessels less than 15 ?m . Under inflammation, adherent leukocytes may also trap SS4 cells, resulting in vaso-occlusion in even larger vessels. This work was supported by the NSF grant CBET-0852948 and the NIH grant R01HL094270.

  1. Dysregulated Ca2+ homeostasis in Fanconi anemia cells.

    PubMed

    Usai, Cesare; Ravera, Silvia; Cuccarolo, Paola; Panfoli, Isabella; Dufour, Carlo; Cappelli, Enrico; Degan, Paolo

    2015-01-01

    Fanconi Anemia (FA) is a rare and complex inherited blood disorder associated with bone marrow failure and malignancies. Many alterations in FA physiology appear linked to red-ox unbalance including alterations in the morphology and structure of nuclei, intermediate filaments and mitochondria, defective respiration, reduced ATP production and altered ATP/AMP ratio. These defects are consistently associated with impaired oxygen metabolism indeed treatment with antioxidants N-acetylcysteine (NAC) and resveratrol (RV) does rescue FA physiology. Due to the importance of the intracellular calcium signaling and its key function in the control of intracellular functions we were interested to study calcium homeostasis in FA. We found that FANCA cells display a dramatically low intracellular calcium concentration ([Ca(2+)]i) in resting conditions. This condition affects cellular responses to stress. The flux of Ca(2+) mobilized by H2O2 from internal stores is significantly lower in FANCA cells in comparison to controls. The low basal [Ca(2+)]i in FANCA appears to be an actively maintained process controlled by a finely tuned interplay between different intracellular Ca(2+) stores. The defects associated with the altered Ca(2+) homeostasis appear consistently overlapping those related to the unbalanced oxidative metabolism in FA cells underlining a contiguity between oxidative stress and calcium homeostasis. PMID:25627108

  2. Studies of the restriction of Equine Infectious Anemia Virus 

    E-print Network

    Geyer, David Scott

    1981-01-01

    STUDIES OF THE RESTRICTION OF EOUINE INFECTIOUS ANEMIA VIRUS A Thesis by DAVID SCOTT GEYER Submitted to the Graduate College of Texas AAM University in partial fulfillment of the requirement for the degree of MASTER OF SCIENCE May 1981... Major Subject: Veterinary Microbiology STUDIES OF THE RESTRICTION OF EQUINE INFECTIOUS ANEMIA VIRUS A Thesis by DAVID SCOTT GEYER Approved as to style and content by: (Chairman of Committee) (Co-Chairman) (Member) (Member) O, w P (Head...

  3. Sickle cell anemia causes a distinct pattern of glomerular dysfunction

    Microsoft Academic Search

    Antonio Guasch; Millicent Cua; Wei You; William E Mitch

    1997-01-01

    Sickle cell anemia causes a distinct pattern of glomerular dysfunction. We characterized glomerular function in adults with sickle cell anemia (SSA): 12 with normal renal function (SSA-controls), and 15 with renal insufficiency (SSA-CRF). GFR was similar in SSA-controls and healthy-controls, however, renal plasma flow was increased in SSA-controls. In SSA-CRF, the albumin and IgG excretion rates were enhanced. The fractional

  4. Discontinuing penicillin prophylaxis in children with sickle cell anemia

    Microsoft Academic Search

    John M. Falletta; Gerald M. Woods; Joel I. Verter; George R. Buchanan; Charles H. Pegelow; Rathi V. Iyer; Scott T. Miller; C. Tate Holbrook; Thomas R. Kinney; Elliott Vichinsky; David L. Becton; Winfred Wang; Helen S. Johnstone; Doris L. Wethers; Gregory H. Reaman; Michael R. DeBaun; Neil J. Grossman; Karen Kalinyak; James H. Jorgensen; Ann Bjornson; Marilyn D. Thomas; Clarice Reid

    1995-01-01

    Objective: To evaluate the consequences of discontinuing penicillin prophylaxis at 5 years of age in children with sickle cell anemia who had received prophylactic penicillin for much of their lives. Design: Randomized, double-blind, placebo-controlled trial. Setting: Eighteen teaching hospitals throughout the United States. Patients: Children with sickle cell anemia (hemoglobin SS or hemoglobin S ?0-thalassemia) who had received prophylactic penicillin

  5. C. elegans: A model of Fanconi anemia and ICL repair

    Microsoft Academic Search

    Jillian L. Youds; Louise J. Barber; Simon J. Boulton

    2009-01-01

    Fanconi anemia (FA) is a severe recessive disorder with a wide range of clinical manifestations [M. Levitus, H. Joenje, J.P. de Winter, The Fanconi anemia pathway of genomic maintenance, Cell Oncol. 28 (2006) 3–29]. In humans, 13 complementation groups have been identified to underlie FA: A, B, C, D1, D2, E, F, G, I, J, L, M, and N [W.

  6. Recombinant human erythropoietin in the treatment of nonrenal anemia

    Microsoft Academic Search

    Michael Heuser; Arnold Ganser

    2006-01-01

    Recombinant human erythropoietins (rhEPO) reliably increase hemoglobin levels in cancer patients experiencing chemotherapy-associated\\u000a anemia. However, in patients with “anemia of cancer” not being treated with chemotherapy, rhEPO appears less effective. Recently,\\u000a two studies have been broadly discussed which have raised concern on the concomitant use of erythropoietin and chemo- or radiation\\u000a therapy in cancer patients. In addition, use of rhEPO

  7. Relationship Between Aplastic Anemia and Paroxysmal Nocturnal Hemoglobinuria

    Microsoft Academic Search

    Taroh Kinoshita; Norimitsu Inoue

    2002-01-01

    Since aplastic anemia—paroxysmal nocturnal hemoglobinuria syndrome was reported in 1967, the overlap of idiopathic aplastic\\u000a anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) has been well known.The link between the 2 diseases became even\\u000a more evident when immunosuppressive therapy improved survival of patients with severe AA. More than 10% of patients with AA\\u000a develop clinically evident PNH. Moreover, flow cytometric analysis

  8. Pernicious anemia in a young man with systemic lupus erythematosus.

    PubMed

    Benjilali, L; Tazi-Mezalek, Z; Harmouche, H; Lebbar, K; Aouni, M; Adnaoui, M; Maaouni, A

    2007-01-01

    Association of pernicious anemia and systemic lupus erythematosus is rare, although both diseases are autoimmune origin. We describe the case of a 40-year old man with systemic lupus erythematosus (SLE) who developed pernicious anemia. The cobalamin deficiency was revealed by macrocytic pancytopenia. After 1 month of vitamin B12 treatment, hemoglobin and white blood cell count remain normal but thrombocytopenia persists and was considered as immunologic from SLE origin requiring corticosteroids. PMID:17895307

  9. Phenotypic features and phylogenetic background of extraintestinal hemolytic Escherichia coli responsible of mortality in puppies.

    PubMed

    Turchetto, Sara; Ustulin, Martina; Citterio, Carlo Vittorio; Zanardello, Claudia; Vascellari, Marta; Vio, Denis; Conedera, Gabriella; Milone, Nicola Maria Ferro; Cocchi, Monia

    2015-08-31

    A 10-day-old litter of five puppies of Bracco Italiano dog breed showed weakness and diarrhea and, 2 days later, four of them died. At the same time, the bitch showed high hyperthermia (40°C) and endometritis. The necropsy of a puppy revealed a severe lobar pneumonia accompanied with a bilateral nephrosis. No gross lesions were detected in other organs. Histopathology of the lung revealed severe multifocal fibrino-suppurative necrotizing bronchiolar-alveolitis associated with rod-shaped bacterial aggregates and diffuse interstitial lymphocytic infiltration. The kidney showed severe multifocal necrosis of the tubular epithelium and diffuse severe congestion of the parenchyma. A pure culture of hemolytic Escherichia coli carrying the Cnf-1 gene was identified, from both the puppy organs and bitch's milk. Moreover, phylo-typing assigned them to the phylogroup B2. Two weeks later, fecal samples from the bitch and the survived puppy were collected for a second microbiological analysis, identifying two hemolytic E. coli strains, Cnf positive and Cdt negative and Cnf and Cdt negative, respectively. Some E. coli pathogenic strains may cause enteric or extraintestinal disease. In dogs and cats, strains of extraintestinal pathogenic E. coli (ExPEC) produce specific virulent factors such as hemolysis and cytotoxin necrotizing factors (Cnf). In this episode, we hypothesize that the bitch's milk could be the main source of ExPEC infection causing high puppies mortality. The role of the bitch as a carrier could not be excluded: stressful conditions, such as pregnancy and delivery, would change the host-pathogen dynamics possibly increasing the release of the infectious burden. PMID:25835470

  10. Anemia in Inflammatory Bowel Disease: An Under-Estimated Problem?

    PubMed Central

    Rogler, Gerhard; Vavricka, Stephan

    2015-01-01

    Anemia is one of the most frequent complications and/or extraintestinal manifestations of inflammatory bowel disease (IBD). Iron deficiency is the most important cause of anemia in Crohn’s disease and ulcerative colitis patients. Iron deficiency even without anemia may impact the quality of life of our IBD patients. In the last 10?years, the understanding of the pathology of iron-deficiency anemia and “anemia of chronic diseases” has increased; new diagnostic tools have been developed and new therapeutic strategies have been discussed. Hepcidin has been identified to be a central regulator of iron absorption from the intestine and of iron plasma levels. Hepcidin is regulated by iron deficiency but also as an acute phase protein by pro-inflammatory mediators such as interleukin-6. Innovative diagnostic tools have not been introduced in clinical routine or are not available for routine diagnostics. As iron substitution therapy is easy these days with a preference for intravenous substitution, the impact of differential diagnosis of anemia in IBD patients is underestimated. PMID:25646159

  11. Red blood cell transfusion in pediatric patients with severe chronic anemia: how slow is necessary?

    PubMed

    Agrawal, Anurag K; Hsu, Edmund; Quirolo, Keith; Neumayr, Lynne D; Flori, Heidi R

    2012-03-01

    Historic practice recommends slow transfusion for children with chronic anemia and hemoglobin less than 5.0?g/dl due to the theoretical risk of transfusion-associated circulatory overload (TACO). In our pediatric intensive care unit (PICU), we have been utilizing a more liberal transfusion practice in patients without underlying cardiopulmonary disease, and a faster transfusion rate appears safe in this population. Rate of transfusion must be based on multiple factors including convenience, timeliness of procedures and transport to an appropriate care facility, risk of alloimmunization and wastage of blood, stress for the family, and need for PICU monitoring. PMID:21793178

  12. Phagocytosis, Oxidative Burst, and Produced Reactive Species are Affected by Iron Deficiency Anemia and Anemia of Chronic Diseases in Elderly

    Microsoft Academic Search

    I. M. M. Paino; J. C. Miranda; C. M. Marzocchi-Machado; E. J. Cesarino; F. A. de Castro; A. M. de Souza

    2009-01-01

    Iron and oxidative stress have a regulatory interplay. During the oxidative burst, phagocytic cells produce free radicals\\u000a such as hypochlorous acid (HOCl). Nevertheless, scarce studies evaluated the effect of either iron deficiency anemia (IDA)\\u000a or anemia of chronic disease (ACD) on phagocyte function in the elderly. The aim of the present study was to determine the\\u000a oxidative burst, phagocytosis, and

  13. Neurological abnormalities in chronic benzene poisoning. A study of six patients with aplastic anemia and two with preleukemia

    SciTech Connect

    Baslo, A.; Aksoy, M.

    1982-04-01

    Neurological, electromyographical and motor conduction velocity examinations were applied to 6 patients with aplastic anemia and two cases of preleukemia due to chronic exposure to benzene. In addition, sensory conduction velocities were measured in three patients. Neurological abnormalities were found in four out of six pancytopenic individuals. There was a certain relationship between the presence of neurological abnormalities and the period of cessation of the exposure. In the two patients with preleukemia similar neurologic abnormalities were found.

  14. Fanconi Anemia Proteins and Endogenous Stresses

    PubMed Central

    Pang, Qishen; Andreassen, Paul R.

    2009-01-01

    Each of the thirteen identified Fanconi anemia (FA) genes is required for resistance to DNA interstrand crosslinking agents, such as mitomycin C, cisplatin, and melphalan. While these agents are an excellent tool for understanding the function of FA proteins in DNA repair, it is uncertain whether a defect in the removal of DNA interstrand crosslinks (ICLs) is the basis for the pathophysiology of FA. For example, DNA interstrand crosslinking agents induce other types of DNA damage, in addition to ICLs. Further, other DNA damaging agents, such as ionizing or ultraviolet radiation, activate the FA pathway, leading to monoubiquitination of FANCD2 and FANCI. Also, FA patients display congenital abnormalities, hematologic deficiencies, and a predisposition to cancer in the absence of an environmental source of ICLs that is external to cells. Here we consider potential sources of endogenous DNA damage, or endogenous stresses, to which FA proteins may respond. These include ICLs formed by products of lipid peroxidation, and other forms of oxidative DNA damage. FA proteins may also potentially respond to telomere shortening or replication stress. Defining these endogenous sources of DNA damage or stresses is critical for understanding the pathogenesis of deficiencies for FA proteins. We propose that FA proteins are centrally involved in the response to replication stress, including replication stress arising from oxidative DNA damage. PMID:19774700

  15. Recent advances in treatment of aplastic anemia

    PubMed Central

    Shin, Seung Hwan; Lee, Sung Eun

    2014-01-01

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

  16. High frequency of ribosomal protein gene deletions in Italian Diamond-Blackfan anemia patients detected by multiplex ligation-dependent probe amplification assay

    PubMed Central

    Quarello, Paola; Garelli, Emanuela; Brusco, Alfredo; Carando, Adriana; Mancini, Cecilia; Pappi, Patrizia; Vinti, Luciana; Svahn, Johanna; Dianzani, Irma; Ramenghi, Ugo

    2012-01-01

    Diamond-Blackfan anemia is an autosomal dominant disease due to mutations in nine ribosomal protein encoding genes. Because most mutations are loss of function and detected by direct sequencing of coding exons, we reasoned that part of the approximately 50% mutation negative patients may have carried a copy number variant of ribosomal protein genes. As a proof of concept, we designed a multiplex ligation-dependent probe amplification assay targeted to screen the six genes that are most frequently mutated in Diamond-Blackfan anemia patients: RPS17, RPS19, RPS26, RPL5, RPL11, and RPL35A. Using this assay we showed that deletions represent approximately 20% of all mutations. The combination of sequencing and multiplex ligation-dependent probe amplification analysis of these six genes allows the genetic characterization of approximately 65% of patients, showing that Diamond-Blackfan anemia is indisputably a ribosomopathy. PMID:22689679

  17. High frequency of ribosomal protein gene deletions in Italian Diamond-Blackfan anemia patients detected by multiplex ligation-dependent probe amplification assay.

    PubMed

    Quarello, Paola; Garelli, Emanuela; Brusco, Alfredo; Carando, Adriana; Mancini, Cecilia; Pappi, Patrizia; Vinti, Luciana; Svahn, Johanna; Dianzani, Irma; Ramenghi, Ugo

    2012-12-01

    Diamond-Blackfan anemia is an autosomal dominant disease due to mutations in nine ribosomal protein encoding genes. Because most mutations are loss of function and detected by direct sequencing of coding exons, we reasoned that part of the approximately 50% mutation negative patients may have carried a copy number variant of ribosomal protein genes. As a proof of concept, we designed a multiplex ligation-dependent probe amplification assay targeted to screen the six genes that are most frequently mutated in Diamond-Blackfan anemia patients: RPS17, RPS19, RPS26, RPL5, RPL11, and RPL35A. Using this assay we showed that deletions represent approximately 20% of all mutations. The combination of sequencing and multiplex ligation-dependent probe amplification analysis of these six genes allows the genetic characterization of approximately 65% of patients, showing that Diamond-Blackfan anemia is indisputably a ribosomopathy. PMID:22689679

  18. Severe Soft Tissue Infection Caused by a Non-Beta-Hemolytic Streptococcus pyogenes Strain Harboring a Premature Stop Mutation in the sagC Gene

    PubMed Central

    Gerlach, Roman G.; Ensser, Armin; Dahesh, Samira; Popp, Isabel; Heeg, Christiane; Bleiziffer, Oliver; Merz, Thomas; Schulz, Theresia; Horch, Raymund E.; Bogdan, Christian; Nizet, Victor; van der Linden, Mark

    2013-01-01

    We recovered a non-beta-hemolytic Streptococcus pyogenes strain from a severe soft tissue infection. In this isolate, we detected a premature stop codon within the sagC gene of the streptolysin S (SLS) biosynthetic operon. Reintroduction of full-length sagC gene on a plasmid vector restored the beta-hemolytic phenotype to our clinical isolate, indicating that the point mutation in sagC accounted for loss of hemolytic activity. To the best of our knowledge, this is the first report to demonstrate that a severe soft tissue infection can be caused by a non-beta-hemolytic S. pyogenes strain lacking a functional SagC. PMID:23515542

  19. Iron deficiency anemia in patients with inflammatory bowel disease.

    PubMed

    Goldberg, Neil D

    2013-01-01

    Iron deficiency anemia is the most common form of anemia worldwide, caused by poor iron intake, chronic blood loss, or impaired absorption. Patients with inflammatory bowel disease (IBD) are increasingly likely to have iron deficiency anemia, with an estimated prevalence of 36%-76%. Detection of iron deficiency is problematic as outward signs and symptoms are not always present. Iron deficiency can have a significant impact on a patient's quality of life, necessitating prompt management and treatment. Effective treatment includes identifying and treating the underlying cause and initiating iron replacement therapy with either oral or intravenous iron. Numerous formulations for oral iron are available, with ferrous fumarate, sulfate, and gluconate being the most commonly prescribed. Available intravenous formulations include iron dextran, iron sucrose, ferric gluconate, and ferumoxytol. Low-molecular weight iron dextran and iron sucrose have been shown to be safe, efficacious, and effective in a host of gastrointestinal disorders. Ferumoxytol is the newest US Food and Drug Administration-approved intravenous iron therapy, indicated for iron deficiency anemia in adults with chronic kidney disease. Ferumoxytol is also being investigated in Phase 3 studies for the treatment of iron deficiency anemia in patients without chronic kidney disease, including subgroups with IBD. A review of the efficacy and safety of iron replacement in IBD, therapeutic considerations, and recommendations for the practicing gastroenterologist are presented. PMID:23766655

  20. Iron deficiency anemia in patients with inflammatory bowel disease

    PubMed Central

    Goldberg, Neil D

    2013-01-01

    Iron deficiency anemia is the most common form of anemia worldwide, caused by poor iron intake, chronic blood loss, or impaired absorption. Patients with inflammatory bowel disease (IBD) are increasingly likely to have iron deficiency anemia, with an estimated prevalence of 36%–76%. Detection of iron deficiency is problematic as outward signs and symptoms are not always present. Iron deficiency can have a significant impact on a patient’s quality of life, necessitating prompt management and treatment. Effective treatment includes identifying and treating the underlying cause and initiating iron replacement therapy with either oral or intravenous iron. Numerous formulations for oral iron are available, with ferrous fumarate, sulfate, and gluconate being the most commonly prescribed. Available intravenous formulations include iron dextran, iron sucrose, ferric gluconate, and ferumoxytol. Low-molecular weight iron dextran and iron sucrose have been shown to be safe, efficacious, and effective in a host of gastrointestinal disorders. Ferumoxytol is the newest US Food and Drug Administration-approved intravenous iron therapy, indicated for iron deficiency anemia in adults with chronic kidney disease. Ferumoxytol is also being investigated in Phase 3 studies for the treatment of iron deficiency anemia in patients without chronic kidney disease, including subgroups with IBD. A review of the efficacy and safety of iron replacement in IBD, therapeutic considerations, and recommendations for the practicing gastroenterologist are presented. PMID:23766655

  1. New strategies for managing anemia of chronic kidney disease.

    PubMed

    Schmid, Holger; Schiffl, Helmut; Lederer, Stephan R

    2012-12-01

    Anemia is a prevalent and premature comorbidity in chronic kidney disease (CKD) and associated with multiple adverse clinical consequences including increased mortality. Today Erythropoiesis-stimulating agents (ESAs), together with iron supplementation, are the cornerstones of therapy for correcting anemia in CKD patients. As no generally accepted dosing algorithms for these agents exist, current recommendations prefer a partial but not complete anemia correction thereby favoring a more conservative and individualized ESA and iron dosing. Here we discuss in detail current evidence derived from large randomized trials about the proposed hemoglobin targets to aim at in CKD and End-Stage renal disease patients and report recent data from the thriving European market of biosimilar erythropoietins. We summarize promising investigational strategies including peginesatide and prolyl hydroxylase inhibitors for stabilization of the hypoxia inducible factor and provide a clinical review of novel high dose iron formulations like ferumoxytol or iron (III)-carboxymaltose. Taking these findings together, treatment strategies for anemia of CKD have got considerably more complicated so that a careful balance between maximization of patient`s quality of life while minimizing all risks associated with anemia treatment has become a major task of current nephrology. PMID:22642238

  2. Thiamine-Responsive Megaloblastic Anemia Syndrome: A Disorder of High-Affinity Thiamine Transport

    Microsoft Academic Search

    Ellis J. Neufeld; Judith C. Fleming; Elena Tartaglini; Mara P. Steinkamp

    2001-01-01

    ABSTRACTThiamine-responsive megaloblastic anemia (TRMA) syndrome (OMIM No. 249270) comprises a distinctive triad of clinical features: megaloblastic anemia with ringed sideroblasts, diabetes mellitus, and progressive sensorineural deafness. The TRMA gene has been mapped and cloned. Designated \\

  3. In vitro Protection of Group A Beta-Hemolytic Streptococci from Penicillin and Cephalothin by Bacteroides fragilis

    Microsoft Academic Search

    Itzhak Brook; Paula Yokum

    1983-01-01

    Beta-lactamase produced by Bacteroides fragilis could protect from antibiotics group A beta-hemolytic streptococci (GABHS), penicillin-susceptible pathogens frequently isolated from acute tonsillitis in children. To test this hypothesis we determined the minimal bactericidal concentration (MBC) to penicillin and cephalothin of GABHS alone and in mixed culture with eleven beta-lactamase-producing strains of B. fragilis. B. fragilis strains with MBC values ? 32

  4. Acute encephalopathy associated with hemolytic uremic syndrome caused by Escherichia coli O157: H7 and rotavirus infection.

    PubMed

    Imataka, G; Wake, K; Suzuki, M; Yamanouchi, H; Arisaka, O

    2015-05-01

    We reported a case of a 22-months child with hemolytic uremic syndrome associated with encephalopathy. As the cause of this case, the involvements of verotoxin 1 and 2 caused by O157: the H7 strain of the enterohemorrhagic Escherichia coli and rotavirus were presumed. We administered brain hypothermic therapy and steroid pulse therapy in the intensive care unit, but we were not able to save his life and the child died on the 6th day from the onset. PMID:26044229

  5. Isolation, phylogenetic analysis and screening of marine mollusc-associated bacteria for antimicrobial, hemolytic and surface activities.

    PubMed

    Romanenko, Lyudmila A; Uchino, Masataka; Kalinovskaya, Natalia I; Mikhailov, Valery V

    2008-01-01

    This study was undertaken to survey culturable heterotrophic bacteria associated with the marine ark shell Anadara broughtoni inhabiting in the Sea of Japan, and to test isolates for their antimicrobial, hemolytic and surface activities with an emphasis on low-molecular-weight metabolites search. A total of 149 strains were isolated and identified phenotypically. A total of 27 strains were selected to be investigated phylogenetically by 165 rRNA gene sequence analysis. The most bacteria were affiliated with members of the Gammaproteobacteria and Alphaproteobacteria, and Less with Firmicutes, Actinobacteria, and Cytophaga-Flavobacterium-Bacteroides (CFB) group. The isolates capable of hemolysis were numerically abundant in the genera Pseudoalteromonas, Aeromonas and Bacillus. The six Gram-positive isolates belonging to the genera Bacillus, Paenibacillus and Saccharothrix and two Gram-negative strains related to Pseudomonas and Sphingomonas, possessed antimicrobial activity against indicator strains and to each other. Antimicrobial, hemolytic and surface activities were revealed in butanot extracts of cells or cell-free supernatant of six active strains. This points to availability of active low-molecular-weight metabolites. Substances with hemolytic and surface activities were isolated from strain Bacillus pumilus An 112 and characterized as cyclic depsipeptides with molecular masses 1021, 1035, 1049, 1063 and 1077 Da. The recovery of strains producing antimicrobial and surface-active substances suggests that microorganisms associated with the marine bivalve are potential source of bioactive metabolites. PMID:19216104

  6. Prevention of anemia alleviates heart hypertrophy in copper deficient rats

    SciTech Connect

    Lure, M.D.; Fields, M.; Lewis, C.G. (Dept. of Agriculture, Beltsville, MD (United States) Univ. of Maryland, College Park (United States) Georgetown Univ., Washington, DC (United States))

    1991-03-11

    The present investigation was designed to examine the role of anemia in the cardiomegaly and myocardial pathology of copper deficiency. Weanling rats were fed a copper deficient diet containing either starch (ST) or fructose (FRU) for five weeks. Six rats consuming the FRU diet were intraperitoneally injected once a week with 1.0 ml/100g bw of packed red blood cells (RBC) obtained from copper deficient rats fed ST. FRU rats injected with RBC did not develop anemia. Additionally, none of the injected rats exhibited heart hypertrophy or gross pathology and all survived. In contrast, non-injected FRU rats were anemic, exhibited severe signs of copper deficiency which include heart hypertrophy with gross pathology, and 44% died. Maintaining the hematocrit with RBC injections resulted in normal heart histology and prevented the mortality associated with the fructose x copper interaction. The finding suggest that the anemia associated with copper deficiency contributes to heart pathology.

  7. nm1054: a spontaneous, recessive, hypochromic, microcytic anemia mutation in the mouse

    Microsoft Academic Search

    R. S. Ohgami; D R Campagna; B Antiochos; E B Wood; J J Sharp; J E Barker; M D Fleming

    2005-01-01

    Hypochromic, microcytic anemias are typically the result of inadequate hemoglobin production because of globin defects or iron deficiency. Here, we describe the phenotypic characteristics and pathogenesis of a new recessive, hypochromic, microcytic anemia mouse mutant, nm1054. Although the mutation nm1054 is pleiotropic, also resulting in sparse hair, male infertility, failure to thrive, and hydrocephaly, the anemia is the focus of

  8. Germ Line Fanconi Anemia Complementation Group C Mutations and Pancreatic Cancer

    Microsoft Academic Search

    Fergus J. Couch; Michele R. Johnson; Kari Rabe; Lisa Boardman; Robert McWilliams; Mariza de Andrade; Gloria Petersen

    Biallelic mutations in Fanconi anemia complementation group genes disrupt DNA repair and result in the complex Fanconi anemia phenotype. In addition, germ line mutations in the BRCA2\\/FANCD1 Fanconi anemia complementation group gene have also been implicated in predisposition to a number of cancers including pancreatic cancer. The recent identification of FANCC and FANCG mutations in resected pancreatic tumors selected for

  9. Assessment of Anemia Knowledge, Attitudes and Behaviors among Pregnant Women in Sierra Leone

    ERIC Educational Resources Information Center

    M'Cormack, Fredanna A. D.; Drolet, Judy C.

    2012-01-01

    Introduction: Iron deficiency anemia prevalence of pregnant Sierra Leone women currently is reported to be 59.7%. Anemia is considered to be a direct cause of 3-7% of maternal deaths and an indirect cause of 20-40% of maternal deaths. This study explores knowledge, attitudes, and behaviors of urban pregnant Sierra Leone women regarding anemia

  10. ForPeerReview Molecular basis of Diamond-Blackfan anemia

    E-print Network

    Paris-Sud XI, Université de

    ForPeerReview Molecular basis of Diamond-Blackfan anemia: Structure and function analysis of RPS19 basis of Diamond-Blackfan anemia: Structure and function analysis of RPS19 Running title : Crystal) Diamond-Blackfan anemia (DBA) is a rare congenital disease linked to mutations in the ribosomal protein

  11. From a Dry Bone to a Genetic Portrait: A Case Study of Sickle Cell Anemia

    E-print Network

    From a Dry Bone to a Genetic Portrait: A Case Study of Sickle Cell Anemia MARINA FAERMAN,1* ALMUT identification; Y chromosome polymorphic markers; sickle cell anemia ABSTRACT The potential and reliability sample, which represented a documented case of sickle cell anemia. -globin gene sequences obtained from

  12. Plenary paper Carboxy terminal region of the Fanconi anemia protein, FANCG/XRCC9,

    E-print Network

    Plenary paper Carboxy terminal region of the Fanconi anemia protein, FANCG/XRCC9, is required D. D'Andrea Fanconi anemia (FA) is an autosomal recessive cancer susceptibility syndrome with eight Introduction Fanconi anemia (FA) is an autosomal recessive cancer suscepti- bility syndrome characterized

  13. PREDICTING THE EFFECTIVENESS OF HYDROXYUREA IN INDIVIDUAL SICKLE CELL ANEMIA PATIENTS

    E-print Network

    Valafar, Faramarz

    1 PREDICTING THE EFFECTIVENESS OF HYDROXYUREA IN INDIVIDUAL SICKLE CELL ANEMIA PATIENTS Homayoun patients with sickle cell anemia. The study described in this paper was undertaken to develop the ability therapy in patients with sickle cell anemia. Adult hemoglobin (HbA) is a tetrameric protein composed

  14. A stochastic model for infectious salmon anemia (ISA) in Atlantic salmon farming

    E-print Network

    Aldrin, Magne

    A stochastic model for infectious salmon anemia (ISA) in Atlantic salmon farming Ida Scheel1 salmon anemia (ISA) is one of the main infectious diseases in Atlantic salmon farming with major; infectious disease dynamics; spatio-temporal point processes; partial likelihood; infectious salmon anemia

  15. Mechanisms of Homogeneous Nucleation of Polymers of Sickle Cell Anemia Hemoglobin in Deoxy State

    E-print Network

    Vekilov, Peter

    Mechanisms of Homogeneous Nucleation of Polymers of Sickle Cell Anemia Hemoglobin in Deoxy State, TX 77204-4004, USA The primary pathogenic event of sickle cell anemia is the polymerization reserved. Keywords: sickle cell anemia; hemoglobin S polymerization; fiber nucleation; homogeneous

  16. Hierarchical Models for Screening of Iron Deficiency Anemia Technical Report No. 99--14

    E-print Network

    Smyth, Padhraic

    Hierarchical Models for Screening of Iron Deficiency Anemia Technical Report No. 99--14 Department and Background Anemia, a reduction in the circulating red cell mass that may diminish the oxygen­carrying capacity of the blood, is one of the most common medical problems. For diagnostic evalu­ ation of anemia

  17. Reduction of costs for anemia-management drugs associated with the use of ferric citrate

    PubMed Central

    Thomas, Anila; Peterson, Leif E

    2014-01-01

    Background Ferric citrate is a novel phosphate binder which has the potential to reduce usage of erythropoietin-stimulating agents (ESAs) and intravenous (IV) iron used for anemia management during hemodialysis (HD) among patients with end-stage renal disease (ESRD). Currently, the potential health care cost savings on a national scale due to the use of ferric citrate in ESRD are undetermined. Methods Per-patient-per-year costs of ESAs (Epogen® and Aranesp® [Amgen Inc., CA, USA]) and IV iron (Venofer® [American Regent, Inc., NY, USA] and Ferrlecit® [Sanofi US, Bridgewater, NJ, USA]) were based on RED BOOK™ (Truven Health Analytics New York, NY, USA) costs combined with the Centers for Medicare and Medicaid Services (CMS) base rate and actual usage in 2011 for the four drugs. The annual number of outpatients undergoing HD in the US was based on frequencies reported by the USRDS (United States Renal Data System). Monte Carlo uncertainty analysis was performed to determine total annual costs and cost reduction based on ferric citrate usage. Results Total annual cost of ESAs and IV iron for anemia management in ESRD determined by Monte Carlo analysis assuming CMS base rate value was 5.127 (3.664–6.260) billion USD. For actual utilization in 2011, total annual cost of ESAs and IV iron was 3.981 (2.780–4.930) billion USD. If ferric citrate usage reduced ESA utilization by 20% and IV iron by 40%, then total cost would be reduced by 21.2% to 4.038 (2.868–4.914) billion USD for the CMS base rate, and by 21.8% to 3.111 (2.148–3.845) billion USD, based on 2011 actual utilization. Conclusion It is likely that US health care costs for anemia-management drugs associated with ESRD among HD patients can be reduced by using ferric citrate as a phosphate binder. PMID:24899820

  18. Adult-onset eculizumab-resistant hemolytic uremic syndrome associated with cobalamin C deficiency.

    PubMed

    Cornec-Le Gall, Emilie; Delmas, Yahsou; De Parscau, Loïc; Doucet, Laurent; Ogier, Hélène; Benoist, Jean-François; Fremeaux-Bacchi, Véronique; Le Meur, Yannick

    2014-01-01

    A 20-year-old man was hospitalized for malignant hypertension, mechanical hemolysis, and kidney failure. Kidney biopsy confirmed glomerular and arteriolar thrombotic microangiopathy. Etiologic analyses, which included ADAMTS13 activity, stool culture, complement factor proteins (C3, C4, factor H, factor I, and MCP [membrane cofactor protein]), anti-factor H antibodies, HIV (human immunodeficiency virus) serology, and antinuclear and antiphospholipid antibodies, returned normal results. Malignant hypertension was diagnosed. Ten months later, we observed a relapse of acute kidney injury and mechanical hemolysis. Considering a diagnosis of complement dysregulation-related atypical hemolytic uremic syndrome (HUS), we began treatment with eculizumab. Despite the efficient complement blockade, the patient's kidney function continued to decline. We performed additional analyses and found that the patient's homocysteine levels were dramatically increased, with no vitamin B12 (cobalamin) or folate deficiencies. We observed very low plasma methionine levels associated with methylmalonic aciduria, which suggested cobalamin C disease. We stopped the eculizumab infusions and initiated specific treatment, which resulted in complete cessation of hemolysis. MMACHC (methylmalonic aciduria and homocystinuria type C protein) sequencing revealed compound heterozygosity for 2 causative mutations. To our knowledge, this is the first report of adult-onset cobalamin C-related HUS. Considering the wide availability and low cost of the homocysteine assay, we suggest that it be included in the diagnostic algorithm for adult patients who present with HUS. PMID:24210589

  19. [Epidemiology of hemolytic uremic syndrome in two regions of Buenos Aires Province].

    PubMed

    Rivero, Mariana; Passucci, Juan; Lucchesi, Paula; Signorini, Marcelo; Alconcher, Laura; Rodríguez, Edgardo; Rocha Martín, Vanesa; Meneguzzi, Belén; San Juan, Fernando; Ballesteros, Bernarda; Tarabla, Héctor

    2013-01-01

    The objectives of this study were (a) to describe and estimate the frequency of hemolytic uremic syndrome (HUS) in rural and urban populations in two regions of Buenos Aires Province, and (b) to compare the presentation and distribution of factors hypothetically associated with HUS. A total of 82 HUS cases, recorded during the years 2005-2010 in rural and urban areas of the south-central region of Buenos Aires Province, were clinically and epidemiologically characterized. Statistical data analysis included Chi square or Fisher test and median test. The incidence rate of HUS was significantly higher in the rural population, being 12.7 cases per 100 000 (CI 0-23.5) in rural inhabitants vs. 7.1 cases per 100 000 (CI 0-9.5) in urban inhabitants. The median age of the patients was 27 months (5-139 months), significantly lower in children from the rural area. This could be explained by a more frequent contact with bovine feces, the consumption of raw milk and a higher proportion of relatives who work in risk labors found in the rural population. Although HUS is often associated with the consumption of undercooked minced meat, most of the children cases here included did not present this antecedent. Clinical manifestations were similar in both subpopulations. One-third of urban patients had received antibiotics prior to HUS development. PMID:23570760

  20. Concerted Action of Sphingomyelinase and Non-Hemolytic Enterotoxin in Pathogenic Bacillus cereus

    PubMed Central

    Doll, Viktoria M.

    2013-01-01

    Bacillus cereus causes food poisoning and serious non-gastrointestinal-tract infections. Non-hemolytic enterotoxin (Nhe), which is present in most B. cereus strains, is considered to be one of the main virulence factors. However, a B. cereus ?nheBC mutant strain lacking Nhe is still cytotoxic to intestinal epithelial cells. In a screen for additional cytotoxic factors using an in vitro model for polarized colon epithelial cells we identified B. cereus sphingomyelinase (SMase) as a strong inducer of epithelial cell death. Using single and double deletion mutants of sph, the gene encoding for SMase, and nheBC in B. cereus we demonstrated that SMase is an important factor for B. cereus cytotoxicity in vitro and pathogenicity in vivo. SMase substantially complemented Nhe induced cytotoxicity in vitro. In addition, SMase but not Nhe contributed significantly to the mortality rate of larvae in vivo in the insect model Galleria mellonella. Our study suggests that the role of B. cereus SMase as a secreted virulence factor for in vivo pathogenesis has been underestimated and that Nhe and SMase complement each other significantly to cause full B. cereus virulence hence disease formation. PMID:23613846