Budd-Chiari syndrome associated with coagulation abormalities in a child with carbohydrate deficient glycoprotein syndrome type Ix - 05/09/11
Doi : 10.1067/mpd.2000.105130
Martina Huemer, MD, Wolf-Dietrich Huber, MD, Wolfgang Schima, MD, Dorothea Moeslinger, MD, Ulrike Holzbach, MD, Ron Wevers, MD, Hans Wank, MD, Sylvia Stoeckler-Ipsiroglu, MD ⁎
Department of Pediatrics and the Department of Radiology, University of Vienna, Vienna, Austria; the Department of Pediatrics, Georg-August-University of Goetlingen, Goetlingen, Germany; Stichting Klinisch-Genetisch Centrum Nijmegen, Academisch Ziekenhuis Nijmegen, Sint Radboudziekenhuis, Lab. Kindergeneeskunde/Neurologie, Nijmegen, The Netherlands; and St. Anna Kinderspital, Vienna, Austria.
aReprint requests: Sylvia Stoeckler-Ipsiroglu, MD, Department of Pediatrics, University of Vienna, Waehringer Guertel 18-20, 1090 Vienna. Austria.
Résumé |
A 6-year-old male patient presented with Budd-Chiari syndrome and glyco-protein abnormalities associated with carbohydrate deficient glycoproteln syndrome type I with yet unidentified molecular defect (type Ix). Budd-Chiari syndrome most likely developed after hepatic venous thrombosis caused by coagulation abnormalities resulting from hypoglycosylation and functional impairment of anticoagulant proteins.
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Publié par Elsevier Masson SAS.
Vol 136 - N° 5
P. 691-695 - mai 2000 Retour au numéro
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