Avtor/Urednik     Meglič, A; Grosek, Š; Benedik-Dolničar, M; Avčin, T
Naslov     Atypical haemolytic uremic syndrome complicated by microangiopathic antiphospholipid-associated syndrome
Tip     članek
Vir     Lupus
Vol. in št.     Letnik 17, št. 9
Leto izdaje     2008
Obseg     str. 842-5
Jezik     eng
Abstrakt     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.
Deskriptorji     HEMOLYTIC-UREMIC SYNDROME
ANTIPHOSPHOLIPID SYNDROME
VASCULITIS
THROMBOCYTOPENIA
THROMBOSIS
CHILD, PRESCHOOL
METHYLPREDNISOLONE
GAMMA-GLOBULINS
PLASMAPHERESIS