Atypical haemolytic uremic syndrome complicated by microangiopathic antiphospholipid-associated syndrome

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,...

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Bibliographic Details
Published inLupus Vol. 17; no. 9; pp. 842 - 845
Main Authors Meglic, A, Grosek, S, Benedik-Dolnicar, M, Avcin, T
Format Journal Article
LanguageEnglish
Published London, England SAGE Publications 01.09.2008
Sage Publications Ltd
Subjects
ADAM Proteins - blood
ADAM Proteins - immunology
ADAMTS13 Protein
Antibodies, Antiphospholipid - blood
Antiphospholipid Syndrome - blood
Antiphospholipid Syndrome - complications
Antiphospholipid Syndrome - therapy
Child, Preschool
Complement Factor H - deficiency
Drug Therapy, Combination
Fingers - blood supply
gamma-Globulins - therapeutic use
Glucocorticoids - therapeutic use
Hemolytic-Uremic Syndrome - blood
Hemolytic-Uremic Syndrome - complications
Hemolytic-Uremic Syndrome - therapy
Humans
Immunologic Factors - therapeutic use
Male
Methylprednisolone - therapeutic use
Microcirculation - immunology
Peripheral Vascular Diseases - blood
Peripheral Vascular Diseases - complications
Peripheral Vascular Diseases - therapy
Plasmapheresis
microangiopathic antiphospholipid-associated syndrome
ADAMTS-13
factor H
haemolytic uremic syndrome
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Summary: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.
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ISSN:0961-2033
1477-0962
DOI:10.1177/0961203308091634