We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Recurrent thrombo-embolism in a child with a congenital disorder of glycosylation (CDG) type Ib and treatment with mannose.
- Authors
Tamminga, R Y J; Lefeber, D J; Kamps, W A; van Spronsen, F J
- Abstract
Thrombosis is a multifactorial disorder. Congenital disorders of glycosylation (CDG) are one of the known risk factors for its occurrence. These disorders result in glycosylation defects of glycoproteins, including those of the (anti-)coagulation system. CDG-Ib can specifically be treated with mannose, as illustrated by the case of a 4-year-old girl in whom deep venous thrombosis was the presenting symptom after a common viral infection. The diagnosis was made after recurrent episodes of thrombo-embolism and consumptive coagulopathy. After treatment with mannose no such episodes recurred. The pathophysiology of CDG as a risk factor for thrombotic disease is discussed.
- Publication
Pediatric hematology and oncology, 2008, Vol 25, Issue 8, p762
- ISSN
1521-0669
- Publication type
Journal Article
- DOI
10.1080/08880010802394616