Document Detail

Bleeding tendency in children with Alagille syndrome.
MedLine Citation:
PMID:  12509572     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Spontaneous intracranial bleeding is now a widely recognized complication and cause of mortality in patients with Alagille syndrome. The pathogenesis of intracranial bleeding in these patients remains unclear. The aim of the study was to look for other sites of bleeding in these patients that could suggest a factor of multiorgan morbidity. METHODS: The records of 174 patients with Alagille syndrome were reviewed, and 38 (22%) patients without liver failure who experienced hemorrhage that led to a drop in hemoglobin level of at least 3 g/dL or to blood transfusion were identified. RESULTS: In 38 patients, 49 bleeding episodes occurred at a median age of 3.75 years (range: 1 month-27 years). Seventeen patients had 23 episodes of spontaneous bleeding; 21 patients bled during surgery or other medical procedures, and 5 among these 21 patients also had a spontaneous bleeding episode. Nine patients bled at least twice. Median platelets count and prothrombin time were normal. Severe cholestasis existed in 33 patients. One patient has a deletion of the 20p12 region, and 13 of 17 patients studied have a JAGGED1 mutation. Blood transfusion was necessary in 23 patients. Eight patients died secondary to bleeding (4 after surgery, 2 after gastrointestinal bleeding, 1 after needle liver biopsy, and 1 after intracranial bleeding). CONCLUSION: These results suggest that patients with Alagille syndrome are at special risk for bleeding; this should be taken into account before deciding on an invasive procedure. The mechanism of the bleeding is still unclear; the role of hypercholesterolemia cannot be excluded, but it may be speculated that JAGGED1 signaling abnormalities may impair the hemostatic function.
Panayotis Lykavieris; Cécile Crosnier; Catherine Trichet; Michèle Meunier-Rotival; Michelle Hadchouel
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatrics     Volume:  111     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2003 Jan 
Date Detail:
Created Date:  2003-01-01     Completed Date:  2003-02-06     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  167-70     Citation Subset:  AIM; IM    
Service d'Hépatologie pédiatrique, Hôpital de Bicêtre, Le Kremlin Bicêtre Cedex, France.
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MeSH Terms
Alagille Syndrome / epidemiology*
Child, Preschool
Hemorrhage / epidemiology*
Intracranial Hemorrhages / epidemiology,  mortality
Retrospective Studies
Survival Rate
Comment In:
J Pediatr Gastroenterol Nutr. 2003 Apr;36(4):509   [PMID:  12872779 ]

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