Chapin, J., Wekslezr, B., Magro, C. & Laurence, J. (2012) Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. British Journal of Haematology, 157, 772–774. Furlan, M., Robles, R., Galbusera, M., Remuzzi, G., Kyrle, P.A., Brenner, B., Krause, M., Scharrer, I., Aumann, V., Mittler, U., Solenthaler, M. & Lammle, B. (1998) von Willebrand factor-cleaving proteas...

The biology of atypical hemolytic uremic syndrome has been shown to involve inability to limit activation of the alternative complement pathway, with subsequent damage to systemic endothelial beds and the vasculature, resulting in the prototypic findings of a thrombotic microangiopathy. Central to this process is the formation of the terminal membrane attack complex C5b-9. Recently, application...

Atypical hemolytic uremic syndrome (HUS) is a rare disorder in children, therefore it may lead to misdiagnosis, delay in treatment, or acute kidney injury. Patients with atypical HUS present signs and symptoms of hemolytic anemia, thrombocytopenia, and higher lactate dehydrogenase and uric acid levels. The main risk factor highlighted is consanguinity, gene mutations, and viral infections. Ecul...

Large and unusually large von Willebrand factor (vWf) multimers may be responsible for systemic platelet aggregation in thrombotic thrombocytopenic purpura (TTP). This possibility is supported by studies that show deficient vWf-cleaving metalloproteinase and increased platelet-vWf binding during TTP episodes. In acute idiopathic TTP, decreased vWf metalloproteinase is the result of autoantibodi...

Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia, leading to end-organ ischemia and infarction, affecting particularly the kidney and brain. TMA is a pathological feature of a number of clinical disorders including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and ...

The uremic syndrome is a complex mixture of organ dysfunctions, which is attributed to the retention of a myriad of compounds that under normal conditions are excreted by healthy kidneys. During recent years major steps have been taken in the area of identification and characterization of uremic retention solutes and in the knowledge of their pathophysiological importance; however, our knowledg...

BACKGROUND Atypical hemolytic uremic syndrome associated with autoantibodies to complement factor H is an important cause of acute kidney injury; most patients require dialysis and are at risk of progressive renal failure. CASE CHARACTERISTICS 7 patients with gastrointestinal symptoms, acute kidney injury, thrombotic microangiopathy and elevated levels of anti-complement factor H antibodies. ...