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hemolytic anemia treatment

Jose Maria Bastida, Maria Luisa Giros, Rocio Benito, Kamila Janusz, Jesus Maria Hernandez-Rivas, Jose Ramon Gonzalez-Porras
Sitosterolemia is a recessive inherited metabolic disorder of unknown prevalence, characterized by increased levels of plasma plant sterols. It is caused by 28 and 31 variants in ABCG5 and ABCG8 genes, respectively, and is characterized by a predisposition to hyperabsorption and accumulation of toxic levels of plant sterols in plasma. Its clinical picture is extremely heterogeneous. The main clinical features are tendinous and cutaneous xanthomas, arthritis or arthralgia, premature cardiovascular disease and atherosclerosis...
July 5, 2018: Current Medicinal Chemistry
Adrien Joseph, Cédric Rafat, Lara Zafrani, Patricia Mariani-Kurkdjian, Agnès Veyradier, Alexandre Hertig, Eric Rondeau, Eric Mariotte, Elie Azoulay
OBJECTIVES: Thrombotic microangiopathy syndromes are a heterogeneous group of severe diseases that often require ICU admission. Prompt initiation of targeted therapies is required for atypical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, whereas there is no specific consensus therapy for Shiga toxin-associated hemolytic uremic syndrome. We sought to compare the characteristics of Shiga toxin-associated hemolytic uremic syndrome, atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura patients at admission in the ICU to allow early differentiation of Shiga toxin-associated hemolytic uremic syndrome from other thrombotic microangiopathy syndromes and help to tailor initial treatment...
July 2, 2018: Critical Care Medicine
Mital Parekh, Abhijit Konnur, Sishir Gang
A 14-year-old female presented with oliguric dialysis requiring kidney injury due to acute poststreptococcal glomerulonephritis (PSGN) with hypertension strongly suggestive of atypical hemolytic uremic syndrome (aHUS) with microangiopathic hemolytic anemia and elevated factor H antibody levels. Renal biopsy revealed crescentic glomerulonephritis with typical subepithelial, intramembranous and mesangial electron-dense deposits (humps) on electron microscopy. She was treated with glucocorticoids following which she recovered, remained dialysis free and her Factor H antibody levels and depressed complement 3 levels normalized...
May 2018: Saudi Journal of Kidney Diseases and Transplantation
Raphaelle Prevost, Guillaume Feugueur, Hervé Moizan, Pierre Keribin, Jihane Kimakhe, Alexis Veyssiere
Sickle cell disease, the most common genetic disease in the world, is a chronic hemolytic disease that can be complicated by severe vaso-occlusive pain crises resulting in tissue necrosis and even death. Due to its increasing incidence, oral surgeons will inevitably be faced with the management of these patients. To limit the risk of crises, our goal is to share recommendations for the management of sickle cell patients in oral surgery. The vaso-occlusive crises (VOC), heart attacks and chronic anemia endured by these patients cause chronic lesions in various organs...
June 27, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
Yi Guo, Xin Tian, Xuefeng Wang, Zheng Xiao
Immunoglobulin has been widely used in a variety of diseases, including primary and secondary immunodeficiency diseases, neuromuscular diseases, and Kawasaki disease. Although a large number of clinical trials have demonstrated that immunoglobulin is effective and well tolerated, various adverse effects have been reported. The majority of these events, such as flushing, headache, malaise, fever, chills, fatigue and lethargy, are transient and mild. However, some rare side effects, including renal impairment, thrombosis, arrhythmia, aseptic meningitis, hemolytic anemia, and transfusion-related acute lung injury (TRALI), are serious...
2018: Frontiers in Immunology
Nicolas Werner, Uwe Zeymer, Boris Fraiture, Caroline Kilkowski, Patrick Riedmaier, Steffen Schneider, Ralf Zahn
BACKGROUND: Interventional closure of paravalvular leaks (PVL) by plug implantation has emerged as an alternative to surgical correction, which is associated with high mortality rates for re-operation. To date, data on procedural efficacy and clinical outcome after transcatheter closure is sparse. We present our experience with interventional PVL closure at our site. METHODS AND RESULTS: From 08/2014 to 10/2016 ten patients (three women, seven men) at high surgical risk for repeat surgery underwent interventional PVL closure for severe paravalvular regurgitation (PVR) in 14 procedures at our site...
June 12, 2018: Clinical Research in Cardiology: Official Journal of the German Cardiac Society
Tim R de Back, Arnon P Kater, Sanne H Tonino
Chronic lymphocytic leukemia (CLL) is frequently complicated by cytopenias, either due to bone marrow infiltration or autoimmunity, resulting in autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia (PRCA) or autoimmune neutropenia (AIN). Morbidity due to autoimmune cytopenias (AIC) can be substantial; in addition, infection risk increases and pre-existing infections might deteriorate due to immunosuppressive medication. In the aging population, CLL occurs more frequently and AIC related to CLL represent a growing clinical challenge...
June 20, 2018: Expert Review of Hematology
Carolien Zwiers, Johanna G van der Bom, Inge L van Kamp, Nan Van Geloven, Enrico Lopriore, John Smoleniec, Roland Devlieger, Pauline E Sim, Marie Anne Ledingham, Eleonor Tiblad, Kenneth J Moise, Karl-Philip Gloning, Mark D Kilby, Timothy G Overton, Ditte S Jørgensen, Katrine V Schou, Bettina Paek, Martin Walker, Emma Parry, Dick Oepkes, Masja de Haas
BACKGROUND: Intrauterine transfusion for severe alloimmunization in pregnancy performed before 20 weeks' gestation is associated with a higher fetal death rate. Intravenous immunoglobulins may prevent hemolysis and could therefore be a non-invasive alternative for early transfusions. OBJECTIVE(S): We evaluated whether maternal treatment with intravenous immunoglobulins defers the development of severe fetal anemia and its consequences in a retrospective cohort to which 12 fetal therapy centers contributed...
June 11, 2018: American Journal of Obstetrics and Gynecology
Jadwiga Szymura, Magdalena Wiecek, Marcin Maciejczyk, Joanna Gradek, Malgorzata Kantorowicz, Zbigniew Szygula
Objective: Endurance runners may experience "sports anemia" resulting from intravascular hemolysis. In addition, aging has negative impact on hematopoiesis and rheological properties of blood, and erythrocyte membranes in older people are more vulnerable to oxidative damage, which together can lead to anemia. Whole-body cryostimulation (WBCST) is increasingly used in the elderly as a method of biological regeneration of athletes or therapy and preventive treatment. That is why the aim of the study was to determine whether repeated WBCST had an effect on the erythrocyte system in master marathon runners, compared to non-training men...
2018: Frontiers in Physiology
Megan Clancy, Ryan McGhan, Jeremy Gitomer, Ana Marie Inocencio, Christina Aldrich, Roberto Iaderosa, Ryan Stevens
PURPOSE: A case report of fatal disseminated cryptococcosis in a patient treated with eculizumab is presented along with a review of literature suggesting a possible etiologic mechanism. SUMMARY: A 23-year-old man with a history of minimal change nephrotic syndrome was hospitalized for acute kidney injury and abdominal pain and swelling. He was found to have disseminated pneumococcal disease, including peritonitis, bacteremia, and pulmonic endocarditis. The patient developed evidence of microangiopathic hemolytic anemia, leading to a diagnosis of atypical hemolytic uremic syndrome, and was started on eculizumab...
June 12, 2018: American Journal of Health-system Pharmacy: AJHP
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
June 11, 2018: European Journal of Haematology
Aleksandra Rymarz, Stanisław Niemczyk
BACKGROUND: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome (APS) involving many organs and leading to their insufficiency. The pathogenesis of CAPS is associated with the presence of antiphospholipid antibodies (aPL). Typical therapy includes anticoagulation, glucocorticoids, therapeutic plasma exchanges and/or intravenous immunoglobulin. Despite this aggressive treatment, the mortality rate of 37% is still high. Novel therapeutic agents are required...
June 8, 2018: BMC Nephrology
Joseph Drummond, Daron Bennet, Loyd V Allen
The first recorded mention of a pharmacogenomic response may be that of Pythagoras in 510 BC, when he noted that hemolytic anemia developed in some but not all people who ingested fava beans. The application of such accounts to pharmacotherapy was inevitable, and customized medications have been compounded since antiquity to treat the needs of individual patients. Today, advances in pharmacogenomic testing yield results that enable more effective targeted therapies sooner in the course of treatment, prevent drug-related adverse effects, save cost, and ensure a better therapeutic outcome...
March 2018: International Journal of Pharmaceutical Compounding
Maria Grazia Rumi, Vito Di Marco, Massimo Colombo
Chronic infection with the hepatitis C virus (HCV) has long been the dominant complication of substitution therapy in patients with inherited blood disorders and the cause of anticipated death due to end-stage liver disease. In hemophilia, transmission of HCV with clotting factors concentrates started to be curbed in the mid-1980s following the adoption of procedures of virus inactivation of concentrates based on heat, whereas in the 1990s treatment of HCV infection with interferon monotherapy was attempted, however, with little success...
May 2018: Seminars in Liver Disease
N Gopalakrishnan, J Dhanapriya, C Padmakumar, T Dineshkumar, A A Kurien, R Sakthirajan, T Balasubramaniyan
Collapsing glomerulopathy (CG) is a distinct histopathologic pattern of glomerular injury characterized by global/segmental wrinkling of the glomerular basement membrane with podocyte hyperplasia and hypertrophy along with tubulointerstitial changes. There is no specific treatment for CG due to etiological heterogeneity, and newer insights into the pathogenesis may lead to the development of targeted therapy. The most common form of CG is the primary or idiopathic followed by secondary (due to viral infections, autoimmune disease, drugs, etc...
March 2018: Indian Journal of Nephrology
Yoshiyasu Ueda, Takashi Miwa, Damodar Gullipalli, Sayaka Sato, Daisuke Ito, Hangsoo Kim, Matthew Palmer, Wen-Chao Song
Background Properdin (P) is a positive regulator of the alternative pathway of complement activation. Although P inhibition is expected and has been shown to ameliorate the alternative pathway of complement-mediated tissue injury in several disease models, it unexpectedly exacerbated renal injury in a murine model of C3 glomerulopathy. The role of P in atypical hemolytic uremic syndrome (aHUS) is uncertain. Methods We blocked P function by genetic deletion or mAb-mediated inhibition in mice carrying a factor H (FH) point mutation, W1206R (FHR/R ), that causes aHUS and systemic thrombophilia with high mortality...
July 2018: Journal of the American Society of Nephrology: JASN
Aparna Vasanthakumar, Justin W Davis, Manal Abunimeh, Jonas Söderholm, Jiuhong Zha, Emily O Dumas, Daniel E Cohen, Jeffrey F Waring, Martin Lagging
BACKGROUND: Genetic variants of inosine triphosphatase (ITPA) that confer reduced ITPase activity are associated with protection against ribavirin(RBV)-induced hemolytic anemia in peginterferon(IFN)/RBV-based treatment of hepatitis C virus (HCV). Patients with reduced ITPase activity showed improved treatment efficacy when treated with IFN/RBV. In addition, a genetic polymorphism near the IL28B gene is associated with an improved response to IFN/RBV treatment. RBV has been an important component of IFN-containing regimens, and is currently recommended in combination with several IFN-free regimens for treatment of harder to cure HCV infections...
2018: PloS One
Maria Celeste Fatone, Fabio Pavone, Gianfranco Lauletta, Sabino Russi
Autoimmune hemolytic anemia (AIHA) is an acquired condition characterized by the presence of autoantibodies recognizing erythrocyte-related antigens. Several components of the immune system are involved in disease pathogenesis. Among them, as for other autoimmune disorders, a role for specific CD8+ CD57+ regulatory cells subset could be hypothesized. We evaluated this lymphocyte subset by flow cytometry in 18 AIHA patients randomly selected in a retrospective population of 29 cases. Secondary forms were observed in 65...
May 30, 2018: Autoimmunity
Alay Mansurov, Eric Christenson
A 47-year-old male presented with a groin lesion in 2011. Initial treatment with antifungals and vinegar was unsuccessful. In 2016, biopsy of this lesion was pursued with diagnosis of extramammary Paget's disease (EMPD). Prior to the scheduled excision, he developed constant lower back pain with radiation to his lower extremities. MRI confirmed vertebral metastasis. Despite surgical and radiation therapy, his back pain progressed, and repeat imaging showed epidural spread of tumor in the thoracic spine. Later, the patient was admitted to the hospital due to severe anemia and thrombocytopenia...
2018: Case Reports in Hematology
Rachael F Grace, Bertil Glader
Mature red blood cells are reliant on the glycolytic pathway for energy production and the hexose monophosphate shunt for cell protection from oxidative insults. The most common red blood cell enzyme disorders are characterized by hemolysis but with wide clinical variability. Glucose-6-phosphate dehydrogenase deficiency is the most common red cell enzyme disorder worldwide. Frequent clinical presentations include neonatal jaundice and episodic hemolysis after exposure to oxidative stress. Symptoms of pyruvate kinase deficiency and other glycolytic enzyme disorders include neonatal jaundice, chronic hemolytic anemia, gallstones, and transfusion-related and transfusion-independent iron overload...
June 2018: Pediatric Clinics of North America
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