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Cholecystectomy-induced thrombotic microangiopathy (TMA) in a postpartum patient successfully treated with eculizumab: a case report.
J Med Case Rep
December 2024
Department of Internal Medicine, University of California Irvine Medical Center, 333 City Blvd West, Suite 500, Orange, CA, 92868, USA.
Background: Thrombotic microangiopathy (TMA) is a rare, life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. Atypical hemolytic uremic syndrome (aHUS) is even less common, comprising less than 10% of hemolytic uremic syndrome (HUS) cases. aHUS in postpartum is associated with poor maternal outcomes, with the majority of cases resulting in end-stage renal disease.
View Article and Find Full Text PDFTreatment discontinuation in adults with atypical hemolytic uremic syndrome (aHUS): a qualitative study of international experts' perspectives with associated cost-consequence analysis.
BMC Nephrol
November 2024
Division of Hematology and Medical Oncology, Weill Cornell Medical College, 1300 York Avenue, New York, NY, 10065, USA.
Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) related to congenital mutations impeding control of the alternative pathway of complement. Following approval of the complement C5 inhibitor eculizumab by the European Medicines Agency and the US Food and Drug Administration, initial guidelines suggested lifelong therapy. Yet, growing evidence indicates that discontinuation of eculizumab, or its long-acting form ravulizumab, is possible for many patients.
View Article and Find Full Text PDFProspective validation of initial eculizumab dosing in adults with atypical hemolytic uremic syndrome.
Nephrol Dial Transplant
November 2024
Department of Pharmacy, Radboud University Medical Center, Nijmegen, Netherlands.
[Thrombotic microangiopathy as a rare complication after lung transplantation].
Praxis (Bern 1994)
October 2024
Klinik für Pneumologie, Universitätsspital Zürich, Rämistrasse 100, CH-8091 Zürich.
Thrombotic microangiopathy (TMA) is defined by the typical triad of severe thrombocytopenia, hemolytic anemia and endorgan dysfunction and can be characterized by the pathophysiology of ischemia-inducing microthrombi in arterioles and capillaries possibly leading to severe organ dysfunction up to acutely life-threatening endorgan damage. In terms of etiology, management, therapy and prognosis, the following manifestations are distinguished: thrombotic thrombocytopenic purpura (TTP), shigatoxin-induced hemolytic-uremic syndrome (STEC-HUS), secondary comorbidity-related TMA and atypical hemolytic-uremic syndrome (aHUS). We present the case of a 49 year old lung transplant recipient developing aHUS.
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