Atypical hemolytic uremic syndrome. Case reports

Atypical hemolytic uremic syndrome (aHUS) is a rare disorder, characterized by the development of hemolytic anemia, thrombocytopenia, and acute renal injury due to a thrombotic microangiopathy. Under our supervision is six patients with aHUS., three of which have passed in-patient treatment, medical examination, three others - were consulted in absentia. Three patients -are male, three - female; age of the patients varied from 10 to 73 years (two children and four adults). All patients, regardless of age first sign of aHUS disease arose sharply with rapid deterioration caused by oliguria, azotemia, edema, anemic and hypertension syndromes. All patients on the background of the therapy aggravation was cut short, however, three patients (disease duration of which more than 1 year), in subsequent disease had anticipated 5 (patient 1), 3 (patient 3), 6 times (patient 4), 3 times (patient 6). Patients with aHUS are suggested the therapy with infusions of fresh frozen plasma, plasmaexchange. In our observation after ineffective plasma exchange and starting renal replacement therapy (peritoneal dialysis) eculizumab appointed to one patient (ID 10). With renal replacement therapy during the year, managed to reduce the azotemia phenomenon, however, remained hypertensive syndrome, anemia. Eculizumab therapy caused regression of symptoms and necessity of renal replacement therapy.

atypical hemolytic-uremic syndrome, thrombotic microangiopathy, eculizumab, atypical hemolytic-uremic syndrome, thrombotic microangiopathy, eculizumab

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