Author/Editor		Meglič, A; Grosek, Š; Benedik-Dolničar, M; Avčin, T
Title		Atypical haemolytic uremic syndrome complicated by microangiopathic antiphospholipid-associated syndrome
Type		članek
Source		Lupus
Vol. and No.		Letnik 17, št. 9
Publication year		2008
Volume		str. 842-5
Language		eng
Abstract		A 4-year-old boy with an atypical course of haemolytic uremic syndrome (HUS), who developed microangiopathic antiphospholipid-associated syndrome (MAPS) with signs of multiple organ failure during the course of his disease, is reported. Early and aggressive treatment with intravenous gammaglobulin, pulse methylprednisolone and plasmapheresis resulted in an excellent clinical recovery. Our patient showed a concomitant presence of multiple factors that could precipitate atypical HUS, including positive antiphospholipid antibodies, decreased level of factor H and positive anti-ADAMTS-13 antibodies. We suggest that, along with infections, autoimmune conditions or defined genetic abnormalities of complement regulatory genes, MAPS should be considered among the pathogenic mechanisms in patients with atypical HUS.
Descriptors		HEMOLYTIC-UREMIC SYNDROME ANTIPHOSPHOLIPID SYNDROME VASCULITIS THROMBOCYTOPENIA THROMBOSIS CHILD, PRESCHOOL METHYLPREDNISOLONE GAMMA-GLOBULINS PLASMAPHERESIS