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Hemolytic anemia

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https://www.readbyqxmd.com/read/28531196/co-inheritance-of-glucose-6-phosphate-dehydrogenase-deficiency-mutations-and-hemoglobin-e-in-a-kachin-population-in-a-malaria-endemic-region-of-southeast-asia
#1
Zeshuai Deng, Fang Yang, Yao Bai, Lijun He, Qing Li, Yanrui Wu, Lan Luo, Hong Li, Limei Ma, Zhaoqing Yang, Yongshu He, Liwang Cui
Glucose-6-phosphate dehydrogenase (G6PD) deficiency and hemoglobin E (HbE, β26 Glu-Lys) are two common red cell disorders in Southeast Asia. G6PD deficiency produces hemolytic anemia, which can be triggered by certain drugs or infections. HbE is asymptomatic or is manifested as microcytic, minimally hemolytic anemia. The association between G6PD deficiency and HbE is little understood. This study aimed to investigate G6PD deficiency and HbE in a Kachin ethnic group in the China-Myanmar border area. G6PD enzyme activity was measured using a quantitative G6PD assay, G6PD variants genotyped by the SNaPshot assay, and an HbE gene mutation identified by an amplification refractory mutation system and subsequently confirmed by using a reverse dot blot hybridization assay from 100 unrelated individuals in the study area...
2017: PloS One
https://www.readbyqxmd.com/read/28529915/hemolytic-uremic-syndrome-in-adults-a-case-report
#2
Fabiel Gerardo Pérez-Cruz, Patricia Villa-Díaz, María Consuelo Pintado-Delgado, María Loreto Fernández Rodríguez, Ana Blasco-Martínez, María Pérez-Fernández
Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by platelet aggregation and mechanical damage to erythrocytes, clinically characterized by microangiopatic haemolytic anemia, thrombocytopenia and organ injury. We are reporting a case of a woman patient with severe hemolytic uremic syndrome associated to infectious diarrhoea caused by Shiga toxin-producing pathogen, who were admitted to our intensive care unit. The patient described developed as organ injury, neurological failure and acute renal failure, with need of haemodialysis technique...
May 4, 2017: World Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28524208/microfluidic-analysis-of-red-blood-cell-deformability-as-a-means-to-assess-hemin-induced-oxidative-stress-resulting-from-plasmodium-falciparum-intraerythrocytic-parasitism
#3
Kerryn Matthews, Simon P Duffy, Marie-Eve Myrand-Lapierre, Richard R Ang, Li Li, Mark D Scott, Hongshen Ma
Hemolytic anemia is one of the hallmarks of malaria and leads to an increase in oxidized heme (hemin) within the plasma of infected individuals. While scavenger proteins sequester much of the circulating heme, it has been hypothesized that extracellular heme may play a central role in malaria pathogenesis. We have previously developed the multiplex fluidic plunger (MFP) device for the measurement of red blood cell (RBC) deformability. Here, we demonstrate that the measurement of changes in RBC deformability is a sensitive method for inferring heme-induced oxidative stress...
May 19, 2017: Integrative Biology: Quantitative Biosciences From Nano to Macro
https://www.readbyqxmd.com/read/28516093/iatrogenic-supravalvular-aortic-stenosis
#4
Paolo Bosco, Antonella Ferrara, Samer A M Nashef
We describe a case of hemolytic anemia and proximal anastomotic site stenosis following emergency repair of a Type A aortic dissection. This rare complication led to a reoperation to correct the iatrogenic aortic stenosis and cure the consequent hemolysis. A "sandwich" technique (with two Teflon strips on the outside and inside of the aortic wall) was used in the initial repair to reinforce the suture line and prevent bleeding from the aortic anastomoses. At the time of reoperation, the inner Teflon strip at the proximal aortic anastomosis was found to have inverted into the aortic lumen, as suggested by the preoperative magnetic resonance imaging...
October 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/28515936/pulse-dose-steroids-in-severe-pulmonary-arterial-hypertension-secondary-to-systemic-lupus-erythematosus
#5
Cody Lee, Jean Elwing
OBJECTIVE: The pulmonary vascular targeted treatment for systemic lupus erythematosus-associated pulmonary arterial hypertension is similar to other connective tissue disease-associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28508588/at-the-cross-section-of-thrombotic-microangiopathy-and-atypical-hemolytic-uremic-syndrome-a-narrative-review-of-differential-diagnostics-and-a-problematization-of-nomenclature
#6
REVIEW
Alexander Åkesson, Eva Zetterberg, Jenny Klintman
Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease associated with high mortality and morbidity. Renal biopsies often indicate thrombotic microangiopathy (TMA). The condition is caused by an excessive activation of the alternative pathway leading to depositions of membrane attack complexes (MAC) on host cells. It may depend on mutations in complement components and regulatory proteins, or the formation of complement-specific antibodies. Mainly, an environmental trigger (e.g. infection) is needed for the excessive response to develop...
May 16, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28505305/efficacy-and-safety-of-ledipasvir-sofosbuvir-for-the-treatment-of-chronic-hepatitis-c-in-persons-with-sickle-cell-disease
#7
Juhi Moon, Robert H Hyland, Fangqiu Zhang, Diana M Brainard, Sophie Lanzkron, John G McHutchison, Mark Sulkowski
Patients with sickle cell disease are at high risk for chronic hepatitis C infection. Prior treatment has been limited due to the use of ribavirin causing hemolytic anemia and interferon causing cytopenias. We demonstrate the safety and efficacy of fixed dose combination, ledipasvir and sofosbuvir for 12 weeks in this population.
May 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28503958/neonatal-management-and-outcome-in-alloimmune-hemolytic-disease
#8
Isabelle M C Ree, Vivianne E H J Smits-Wintjens, Johanna G van der Bom, Jeanine M M van Klink, Dick Oepkes, Enrico Lopriore
Hemolytic disease of the fetus and newborn (HDFN) occurs when fetal and neonatal erythroid cells are destroyed by maternal erythrocyte alloantibodies, it leads to anemia and hydrops in the fetus, and hyperbilirubinemia and kernicterus in the newborn. Postnatal care consists of intensive phototherapy and exchange transfusions to treat severe hyperbilirubinemia and top-up transfusions to treat early and late anemia. Other postnatal complications have been reported such as thrombocytopenia, iron overload and cholestasis requiring specific management...
May 13, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28502324/favorable-long-term-outcomes-of-isolated-liver%C3%A2-transplantation-in-a-child-with-atypical-hemolytic-uremic-syndrome-caused-by-a%C3%A2-novel%C3%A2-complement-factor-h-mutation%C3%A2
#9
Heeyeon Cho, Yeonhee Lee
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury that is usually caused by complement dysregulation. Complement factor H (CFH) is a regulator of the complement system produced in the liver, and CFH gene mutations are the most frequent causes of aHUS. To date, the therapeutic options for aHUS with CFH mutations have consisted of plasma infusions, plasma exchange, kidney transplantation, isolated liver transplantation, or combined liver and kidney transplantation...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28496185/-gardos-channelopathy-a-variant-of-hereditary-stomatocytosis-with-complex-molecular-regulation
#10
Elisa Fermo, Anna Bogdanova, Polina Petkova-Kirova, Anna Zaninoni, Anna Paola Marcello, Asya Makhro, Pascal Hänggi, Laura Hertz, Jens Danielczok, Cristina Vercellati, Nadia Mirra, Alberto Zanella, Agostino Cortelezzi, Wilma Barcellini, Lars Kaestner, Paola Bianchi
The Gardos channel is a Ca(2+) sensitive, K(+) selective channel present in several tissues including RBCs, where it is involved in cell volume regulation. Recently, mutations at two different aminoacid residues in KCNN4 have been reported in patients with hereditary xerocytosis. We identified by whole exome sequencing a new family with two members affected by chronic hemolytic anemia carrying mutation R352H in the KCNN4 gene. No additional mutations in genes encoding for RBCs cytoskeletal, membrane or channel proteins were detected...
May 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28484163/progress-in-diagnosis-and-treatment-of-autoimmune-hemolytic-disorders
#11
Toyomi Kamesaki
Discovery of the Coombs' test was an epoch-making event in the history of managing autoimmune hemolytic anemia (AIHA). The Coombs' test allows immune-related hemolytic anemia to be distinguished from nonimmune acquired hemolytic anemia, but also creates a complicated category; Coombs-negative AIHA. To resolve this problem, several trials have been conducted to detect immunoglobulin (Ig) G molecules on erythrocytes (RBC-IgG) that the Coombs' test cannot detect. In Japan, RBC-IgG can be quantitated using a radioimmunoassay, but this procedure is time-consuming and expensive...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28482712/heme-mediated-cell-activation-the-inflammatory-puzzle-of-sickle-cell-anemia
#12
Caroline Conceição da Guarda, Rayra Pereira Santiago, Luciana Magalhães Fiuza, Milena Magalhães Aleluia, Júnia Raquel Dutra Ferreira, Camylla Vilas Boas Figueiredo, Setondji Cocou Modeste Alexandre Yahouedehou, Rodrigo Mota de Oliveira, Isa Menezes Lyra, Marilda de Souza Gonçalves
Hemolysis triggers the onset of several clinical manifestations of sickle cell anemia (SCA). During hemolysis, heme, which is derived from hemoglobin (Hb), accumulates due to the inability of detoxification systems to scavenge sufficiently. Heme exerts multiple harmful effects, including leukocyte activation and migration, enhanced adhesion molecule expression by endothelial cells and the production of pro-oxidant molecules. Area covered: In this review, we describe the effects of heme on leukocytes and endothelial cells, as well as the features of vascular endothelial cells related to vaso-occlusion in SCA...
May 17, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28481070/-negative-pressure-system-in-the-treatment-of-enterocutaneous-fistulas-in-the-pediatric-population
#13
V Núñez Cerezo, M I Romo Muñoz, M V Amesty Morello, A Vilanova Sánchez, M Dore Reyes, M Gómez Cervantes, A M Andrés Moreno, E Martínez-Ojinaga Nodal, L Martínez Martínez, M López Santamaría
AIM OF THE STUDY: The management of children with enterocutaneous fistulas (EF) along with large abdominal wall defects secondary to multiple surgical interventions can be difficult and sometimes lead to intestinal failure (IF). The aim of this study is to present the results of negative pressure systems and their properties (edema reduction angiogenesis promotion and granulation tissue formation) in children with enterocutaneous fistulas (EF) and their prognosis. MATERIALS AND METHODS: A retrospective analysis of children with refractory, high output EF treated with NPS between 2008-2014...
October 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28480960/itpa-gene-polymorphism-94c-a-effects-on-ribavirin-induced-anemia-during-therapy-in-egyptian-patients-with-chronic-hepatitis-c
#14
Maissa El Raziky, Naglaa Ali Zayed, Amin Abdel Baki, Shimaa Afify Mansour, Rasha Mohamad Hosny Shahin
PROBLEM: Inosine triphosphatase (ITPA) gene variants can protect against ribavirin (RBV)-induced anemia in patients treated for chronic hepatitis C. The aim of this study was to determine the relationship between genetic variants of ITPA polymorphism, anemia, RBV dose reduction and treatment response in hepatitis C virus (HCV)-infected patients. METHOD OF STUDY: This study was conducted on 97 Egyptian chronic HCV patients who were scheduled for pegylated-interferon (PEG-INF) /RBV therapy...
May 8, 2017: Journal of Medical Virology
https://www.readbyqxmd.com/read/28477093/clinical-and-serological-autoimmune-complications-in-chronic-lymphocytic-leukemia
#15
Cengiz Demir, Ömer Ekinci
BACKGROUND: Autoimmune disorders often develop during the course of chronic lymphocytic leukemia (CLL). The aim of our study was to investigate the incidence of autoimmune complications (AIC) and serological autoantibodies, and to assess the relationship of these to patient characteristics. METHODS: We prospectively collected screenings of AIC and serological markers from a total of 192 patients. RESULTS: AIC was observed in 18 (9.4%) patients...
May 5, 2017: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/28473690/clinical-and-laboratory-features-of-cd5-negative-chronic-lymphocytic-leukemia
#16
Cengiz Demir, Erdal Kara, Ömer Ekinci, Senar Ebinç
BACKGROUND Chronic lymphocytic leukemia (CLL) usually expresses CD5 antigen. However, 7-20% of patients are CD5 negative. We report here a series of 19 CD5-negative B-CLL cases. MATERIAL AND METHODS We reviewed 19 consecutive CD5-negative B-CLL cases seen in our medical center from 2009 to 2015 and compared them with 105 CD5-positive B-CLL cases. The two groups were compared in terms of clinical parameters, laboratory parameters, and survival characteristics. RESULTS Lymphadenopathy was present in 31.5% of the CD5-negative group and 51...
May 5, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28472790/triggering-of-eryptosis-the-suicidal-erythrocyte-death-by-perifosine
#17
Jasmin Egler, Florian Lang
BACKGROUND/AIMS: The alkylphospholipid perifosine is used for the treatment of malignancy. The substance is effective by triggering suicidal tumor cell death or apoptosis. Side effects of perifosine include anemia. At least in theory, perifosine-induced anemia could result from stimulation of suicidal erythrocyte death or eryptosis. Hallmarks of eryptosis are cell shrinkage and cell membrane scrambling with phosphatidylserine translocation to the erythrocyte surface. Cellular mechanisms participating in the orchestration of eryptosis include increase of cytosolic Ca2+ activity ([Ca2+]i), oxidative stress, increase of ceramide abundance, as well as activation of staurosporine sensitive protein kinase C and/or of SB203580 sensitive p38 kinase...
May 4, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28469947/successful-long-term-use-of-eltrombopag-in-a-patient-with-refractory-severe-thrombocytopenia-associated-with-chronic-lymphocytic-leukemia-that-allowed-oral-anticoagulant-treatment-for-severe-cardiomyopathy
#18
Juárez Salcedo Luis Miguel, Gil-Fernández Juan José
Autoimmune cytopenias (AICs) are frequently associated with chronic lymphocytic leukemia (CLL). The most common of these AICs is autoimmune hemolytic anemia (AIHA); the second most is immune thrombocytopenia (ITP). Here, we report on a patient with CLL-associated ITP, with thrombocytopenia refractory to corticosteroids and intravenous immunoglobulins, in which continuous oral treatment with Eltrombopag allowed initiation and maintenance of an oral anticoagulation treatment with Acenocoumarol that was indicated because of a severe arrhythmogenic cardiomyopathy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28468084/-chinese-expert-consensus-on-the-diagnosis-and-treatment-of-autoimmune-hemolytic-anemia-2017-red-blood-cell-disease-anemia
#19
(no author information available yet)
No abstract text is available yet for this article.
April 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28467346/chronic-anemia-and-thyroid-function
#20
Ashraf T Soliman, Vincenzo De Sanctis, Mohamed Yassin, Magda Wagdy, Nada Soliman
Anaemia is a global public health problem affecting both developing and developed countries with major consequences for human health as well as social and economic development. It occurs at all stages of the life cycle, but is more prevalent in pregnant women and young children. Iron deficiency anaemia (IDA) impairs thyroid metabolism in animals and human and may negatively affect growth and develpment of children. On the other hand both overt and subclinical hypothyroidism are associated with anemia and adding iron to thyroxine therapy improves both conditions compared to thyroxine therapy alone...
April 28, 2017: Acta Bio-medica: Atenei Parmensis
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