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hemolytic anemia treatment

Chisa Fukasawa, Saori Ooishi, Takuma Kumagai, Megumi Koshiisi, Yuki Sueki, Kei Nakajima, Toru Mitsumori, Yoko Yoshida, Hideki Kato, Masaomi Nangaku, Toshiyuki Miyata, Keita Kirito
Herein, we present an elderly onset case of aHUS successfully treated with eculizumab. An 80-year-old woman with severe anemia, thrombocytopenia, and acute renal dysfunction was admitted to our hospital. A laboratory test revealed steep elevation in the LDH level, and the peripheral blood smear showed erythrocyte fragmentations. Accordingly, we diagnosed thrombotic microangiopathy, and treatment with plasma exchange was immediately initiated. In addition, she required hemodialysis because of rapid impairment of the renal function...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Serkan Yıldız, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are disorders characterized by endothelial cell activation, microangiopathic hemolytic anemia, thrombocytopenia and organ failure of variable intensity. The pathophysiology of various types of TMAs have become an interesting field of study. Alternative complement system activation plays an important role in several pathophysiological conditions. Complement activation is also described in an increasing number of TMAs. Inherited defects in complement regulatory genes and acquired autoantibodies against complement regulatory proteins have been described...
February 21, 2018: Transfusion and Apheresis Science
Qurrat Ul Ain, Huzaifa Saleem, Sarwat Iqbal, Rabia Ghayas
Paroxysmal Nocturnal Haemoglobinuria (PNH) is an acquired, rare life-threatening disorder characterised by compliment mediated hemolytic anemia, thrombosis and impaired bone marrow function. It occasionally presents in childhood or adolescence. This is a case of a 14-year old female presented with complaints of shortness of breath, palpitation and abdominal pain whose laboratory test results were consistent with Coomb's test negative haemolytic anaemia. Contrast enhanced Computed Tomography Scan (CT scan) of abdomen revealed splanchnic circulation thrombosis as well as partially occluding thrombus in the inferior vena cava...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Emre Tekgündüz, Mehmet Yılmaz, Mehmet Ali Erkurt, Ilhami Kiki, Ali Hakan Kaya, Leylagul Kaynar, Inci Alacacioglu, Guven Cetin, Ibrahim Ozarslan, Irfan Kuku, Gulden Sincan, Ozan Salim, Sinem Namdaroglu, Abdullah Karakus, Volkan Karakus, Fevzi Altuntas, Ismail Sari, Gulsum Ozet, Ismet Aydogdu, Vahap Okan, Emin Kaya, Rahsan Yildirim, Esra Yildizhan, Gokhan Ozgur, Osman Ilhami Ozcebe, Bahriye Payzin, Seval Akpinar, Fatih Demirkan
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission...
February 20, 2018: Transfusion and Apheresis Science
Daniel Brustolin Ludwig, Luciana Erzinger Alves de Camargo, Najeh Maissar Khalil, Marcos Ereno Auler, Rubiana Mara Mainardes
Amphotericin B (AmB) is one of the most used drugs for the treatment of systemic fungal infections; however, the treatment causes several toxic manifestations, including nephrotoxicity and hemolytic anemia. Chitosan-coated poly(lactide-co-glycolide) (PLGA) nanoparticles containing AmB were developed with the aim to decrease AmB toxicity and propose the oral route for AmB delivery. In this work, the antifungal efficacy of chitosan-coated PLGA nanoparticles containing AmB was evaluated in 20 strains of fungus isolates from patients with vulvovaginal candidiasis (01 Candida glabrata and 03 Candida albicans), bloodstream infections (04 C...
March 1, 2018: Mycopathologia
T M Archer, J V Stokes, E Kummari, C Fellman, J Thomason, J Haraschak, R Wills, L Pinchuk, A Mackin
Cyclosporine and aspirin are routinely used in combination to treat immune-mediated hemolytic anemia (IMHA) in dogs. Cyclosporine is a potent immunosuppressive agent that targets T cell production of the cytokines IL-2 and IFN-γ. Low-dose aspirin is often used to inhibit platelet function in dogs with IMHA, since these animals are prone to life-threatening thromboembolic disease. In rodents and humans, aspirin and cyclosporine have both been shown to variably affect T cell cytokine production, and also numbers of circulating regulatory T cells (Tregs)...
March 2018: Veterinary Immunology and Immunopathology
Mira Choi, Jörg Hofmann, Anja Köhler, Bo Wang, Claus-Thomas Bock, Eckart Schott, Petra Reinke, Peter Nickel
Background: Elevated liver enzymes are frequently observed in renal transplant recipients and warrant further exploration. In immunosuppressed patients, hepatitis E virus (HEV) infection may cause chronic hepatitis, cirrhosis, and extrahepatic manifestations such as renal injury. Methods: We performed a retrospective cross-sectional study investigating the prevalence, clinical correlates, and outcome of chronic HEV infection in a cohort of renal transplant recipients with elevated liver enzymes...
February 2018: Transplantation Direct
Kapil Kumar Gupta, Mukesh Srivastava, Vikrant Sudan, Shanker Kumar Singh, Soumen Choudhury, Daya Shanker
The present study was designed to assess the deleterious effects of bovine tropical theileriosis on the cardiovascular system and the consequent myocardial involvement in young calves. Myocardial effects in parasitic diseases are often neglected. Hemolytic anemia, associated secondary hypoxia, and vasculitis are cardinal features of bovine theileriosis. In the present study, electrocardiogram (ECG) alongside serum cardiac troponin I (cTnI) and creatinine phosphokinase-myocardial band (CPK-MB) concentrations were analyzed in infected, treated, and control groups of young calves...
February 17, 2018: Tropical Animal Health and Production
Fukumi Nakamura-Uchiyama, Yuichi Katanami, Tadashi Kikuchi, Saho Takaya, Satoshi Kutsuna, Taiichiro Kobayashi, Yasutaka Mizuno, Tetsuo Hasegawa, Michiko Koga, Yukihiro Yoshimura, Chihiro Hasegawa, Yasuyuki Kato, Mikio Kimura, Haruhiko Maruyama
BACKGROUND: The Research Group on Chemotherapy of Tropical Diseases, Japan, introduced artemether-lumefantrine (AL) in late 2002, mainly for treating uncomplicated Plasmodium falciparum malaria. Because AL was on the market in Japan in March 2017, the effectiveness and safety of AL were analyzed to help medical personnel use AL optimally. METHODS: Case report forms submitted by the attending physicians were analyzed. When necessary, direct contact with the attending physicians was made to obtain detailed information...
February 14, 2018: Travel Medicine and Infectious Disease
Shun-Ichi Wakabayashi, Takefumi Kimura, Naoki Tanaka, Satoru Joshita, Kazuhito Kawata, Takeji Umemura, Yuki Hiroshima, Hiromitsu Mori, Hikaru Kobayashi, Shuichi Wada, Eiji Tanaka
A 68-year-old woman was referred to our hospital due to fever and rash on the neck and extremities. Laboratory findings revealed hepatic dysfunction and positivity for anti-mitochondrial M2 antibody (AMA-M2). Hepatosplenomegaly and systemic lymphadenopathy were detected by enhanced computed tomography. One week after her first visit, hypoxemia, ascites, and Coomb test-positive autoimmune hemolytic anemia had newly appeared in addition to worsened fever, hepatosplenomegaly, and lymphadenopathy. Results of axillary lymph node, skin, and bone-marrow biopsies led to the diagnosis of angioimmunoblastic T-cell lymphoma (AITL), for which CEPP therapy (cyclophosphamide, etoposide, procarbazine, and prednisolone) was initiated...
February 10, 2018: Clinical Journal of Gastroenterology
Suzanne S Puentes, Michele Dunstan
Escherichia coli is a bacterium that is an important part of the intestinal tract; however, it has the potential to become pathogenic. Shiga toxin-producing E coli (STEC) is a leading cause of E coli infections and has led to outbreaks in North America. Transmission is through ingestion of contaminated food sources, and via infected humans and animals. Young children infected with STEC are at high risk for developing hemolytic uremic syndrome (HUS). HUS is a clinical syndrome characterized by macroangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure...
March 2018: Critical Care Nursing Clinics of North America
Xiaolei Liu, Roi Treister, Magdalena Lang, Anne Louise Oaklander
Objectives: Small-fiber polyneuropathy (SFPN) has various underlying causes, including associations with systemic autoimmune conditions. We have proposed a new cause; small-fiber-targeting autoimmune diseases akin to Guillain-Barré and chronic inflammatory demyelinating polyneuropathy (CIDP). There are no treatment studies yet for this 'apparently autoimmune SFPN' (aaSFPN), but intravenous immunoglobulin (IVIg), first-line for Guillain-Barré and CIDP, is prescribed off-label for aaSFPN despite very high cost...
2018: Therapeutic Advances in Neurological Disorders
Roberta Russo, Immacolata Andolfo, Francesco Manna, Antonella Gambale, Roberta Marra, Barbara Eleni Rosato, Paola Caforio, Valeria Pinto, Piero Pignataro, Kottayam Radhakrishnan, Sule Unal, Giovanna Tomaiuolo, Gian Luca Forni, Achille Iolascon
Mutations in more than 70 genes cause hereditary anemias (HA), a highly heterogeneous group of rare/low frequency disorders in which we included: hyporegenerative anemias, as congenital dyserythropoietic anemia (CDA) and Diamond-Blackfan anemia; hemolytic anemias due to erythrocyte membrane defects, as hereditary spherocytosis and stomatocytosis; hemolytic anemias due to enzymatic defects. The study describes the diagnostic workflow for HA, based on the development of two consecutive versions of a targeted-NGS panel, including 34 and 71 genes, respectively...
February 3, 2018: American Journal of Hematology
Ameni Jerbi, Sawsan Feki, Salma Ben Ameur, Hend Hachicha, Lamia Gargouri, Thouraya Kamoun, Abdelmajid Mahfoudh, Mongia Hachicha, Hatem Masmoudi
In adults, anti-neutrophil cytoplasmic antibodies (ANCA) are considered as serological markers of several diseases, especially vasculitis and glomerulonephritis. Since ANCA are rarely positive in children, few data about the clinical relevance of these auto-antibodies in pediatric population have been reported. Therefore, our study aims to describe the spectrum of disorders associated with positive ANCA in Tunisian children. This study had been carried out over a period of 12 years and a half. All patients under the age of 15 for whom ANCA screening was performed in our laboratory were included...
January 1, 2018: Annales de Biologie Clinique
Mark D Parker
The SLC4 family Cl- -HCO3 - exchangers (AE1, AE2, and AE3) and Na+ /HCO3 - cotransporters (NBCe1, NBCe2, NBCn1, and NBCn2) contribute to a variety of vital physiological processes including pH regulation and epithelial fluid secretion. Accordingly, their dysfunction can have devastating effects. Disorders such as epilepsy, hemolytic anemia, glaucoma, hearing loss, osteopetrosis and renal tubular acidosis are all genetically linked to SLC4-family gene loci. This review summarizes how studies of Slc4-modified mice have enhanced our understanding of the etiology of SLC4-linked pathologies and the interpretation of genetic linkage studies...
January 31, 2018: American Journal of Physiology. Cell Physiology
J Morser, Z Shao, T Nishimura, Q Zhou, L Zhao, J Higgins, L L K Leung
BACKGROUND: There are two basic carboxypeptidases in plasma. Carboxypeptidase B2 (CPB2) is activated from a circulating zymogen, proCPB2, and carboxypeptidase N (CPN) is constitutively active with both inactivating complement C3a and C5a. AIMS: To test the roles of CPB2 and CPN in complement-driven mouse models of cobra venom factor (CVF) challenge and hemolytic-uremic syndrome (HUS). METHODS: Cpb2-/- , Cpn-/- and wild type (WT) mice were compared in a HUS model induced by Shiga toxin and lipopolysaccharide administration and following CVF administration...
January 31, 2018: Journal of Thrombosis and Haemostasis: JTH
Olivia Alejandra Flores-Montes, Martha Cecilia Escobar-Orduño, Mónica Lozano-Garcidueñas, Jaime Guadalupe Valle-Leal
BACKGROUND: Evans syndrome is characterized by the reduction of at least two blood cell lineages in the absence of other diagnoses; it was previously described as the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with unknown etiology. An incidence of 37% and mortality rate of 10% were reported for Evans syndrome. CLINICAL CASES: We report the clinical presentation and evolution of Evans syndrome in two infants who were initially diagnosed with immune thrombocytopenia...
March 2017: Boletín Médico del Hospital Infantil de México
Gheyath K Nasrallah, Nadin N Younes, Missbah H Baji, Amjad M Shraim, Ibrahim Mustafa
OBJECTIVES: Thalassemia is the most common genetically inherited blood disorder arising from a defect in hemoglobin production, resulting in ineffective erythropoiesis and severe hemolytic anemia. While transfusion therapy corrects the anemia, it gives rise to secondary iron overload. Current iron chelation therapy performed using deferoxamine, and the efficiency of this drug was demonstrated here using the zebrafish animal model. METHODS: Zebrafish larvae were exposed for three days to iron [100 μM ferric ammonium citrate; 3-6 days post fertilization (dpf)]...
January 27, 2018: European Journal of Haematology
Q Y Gao, C X Liu, Y Li, G X Peng, J P Li, Y Li, L Ye, H H Fan, L Song, L Zhang, L P Jing, K Zhou, X Zhao, W R Yang, Z J Wu, Y Yang, Y Z Xiong, F K Zhang
No abstract text is available yet for this article.
December 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Maria di Ioia, Deborah Farina, Valeria di Tommaso, Daniela Travaglini, Erika Pietrolongo, Marco Onofrj, Giovanna de Luca
BACKGROUND: Alemtuzumab, approved for multiple sclerosis (MS), can cause secondary autoimmune adverse events including thyroid disorders, immune thrombocytopenia (ITP), and glomerular nephropathies. Non-ITP autoimmune cytopenias are rarely reported. OBJECTIVE: To report a case of autoimmune hemolytic anemia (AIHA) and nephropathy in a MS patient treated with alemtuzumab. CASE REPORT: A 34-year-old man with MS developed albuminuria and AIHA after the first and only alemtuzumab treatment, with positive Coombs' direct and indirect tests and IgG autoantibodies...
January 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
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