Atypical uraemic haemolytic syndrome in pregnancy
نویسندگان
چکیده
منابع مشابه
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...
متن کاملAtypical 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...
متن کاملEculizumab 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...
متن کاملAdvances in understanding the aetiology of atypical Haemolytic Uraemic Syndrome.
Atypical Haemolytic Uraemic Syndrome (aHUS) is a thrombotic microangiopathy that often provokes irreversible renal damage and post-transplantation recurrence. Studies performed during the last decade have shown that 50-60% of aHUS patients present genetic or acquired defects in the complement system that enhance the initial endothelial damage and favour disease development. This review analyses...
متن کاملEarly 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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ژورنال
عنوان ژورنال: Cirugía y Cirujanos (English Edition)
سال: 2016
ISSN: 2444-0507
DOI: 10.1016/j.circen.2016.06.012