Atypical haemolytic uraemic syndrome and pregnancy: outcome with ongoing eculizumab

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Eculizumab in secondary atypical haemolytic uraemic syndrome

Background Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. Methods We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA r...

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Early treatment with eculizumab in atypical haemolytic uraemic syndrome

Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysi...

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Atypical haemolytic uraemic syndrome.

The haemolytic uraemic syndrome (HUS) is characterized by the triad of thrombocytopenia, microangiopathic haemolytic anaemia and acute renal failure. HUS may be classified as either diarrhoeal-associated or non-diarrhoeal/atypical (aHUS). aHUS has recently been shown to be a disease of complement dysregulation, with 50% of cases involving the complement regulatory genes, factor H (CFH), membran...

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Eculizumab and drug-induced haemolytic–uraemic syndrome

The monoclonal anti-C5 antibody eculizumab has been successfully tested in atypical haemolytic-uraemic syndrome (aHUS), with or without mutations in the regulatory proteins of the alternative pathway of the complement, and less convincingly in enterohaemorrhagic Escherichia coli-associated HUS. Here, we report a patient with mitomycin-C-induced HUS unresponsive to plasma exchanges. Eculizumab i...

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Atypical haemolytic uraemic syndrome

Introduction Atypical haemolytic uremic syndrome is a result of a spectrum of diseases. Disorders of complement regulation are the most important reasons in the aetiology. It is associated with defective regulation of the alternative complement pathway in over 50% of cases. Clinical abnormalities are related with the presence of thrombotic microangiopathy. Patients with atypical haemolytic urem...

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ژورنال

عنوان ژورنال: Nephrology Dialysis Transplantation

سال: 2016

ISSN: 0931-0509,1460-2385

DOI: 10.1093/ndt/gfw314