Interventions for atypical haemolytic uraemic syndrome
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 کاملInterventions for haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura.
BACKGROUND Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are related conditions with similar clinical features of variable severity. Survival of patients with HUS and TTP has improved greatly over the past two decades with improved supportive care for patients with HUS and by the use of plasma exchange (PE) with fresh frozen plasma (FFP) for patients with TTP. ...
متن کاملAtypical Haemolytic Uraemic Syndrome Associated with a Hybrid Complement Gene
BACKGROUND Sequence analysis of the regulators of complement activation (RCA) cluster of genes at chromosome position 1q32 shows evidence of several large genomic duplications. These duplications have resulted in a high degree of sequence identity between the gene for factor H (CFH) and the genes for the five factor H-related proteins (CFHL1-5; aliases CFHR1-5). CFH mutations have been describe...
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ژورنال
عنوان ژورنال: Cochrane Database of Systematic Reviews
سال: 2017
ISSN: 1465-1858
DOI: 10.1002/14651858.cd012862