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Gastric angiodysplasia in a child with Bernard-Soulier syndrome: efficacy of octreotide in long-term management.

Abstract
Gastrointestinal angiodysplasia in association with Bernard-Soulier syndrome has been previously described in adults. The authors report on a 14year-old boy presenting with massive upper gastrointestinal bleeding due to a large gastric angiodysplasia, in whom medical history and laboratory investigations were consistent with Bernard-Soulier syndrome. The vascular lesion was so widespread that surgical or endoscopic therapy was not considered. Therefore, treatment with octreotide, a somatostatin analog, was commenced, following a short course of tranexamic acid and proton pump inhibitor. During the 16-month follow-up with octreotide therapy, no occult or gross bleeding occurred. This case illustrates the utility of using octreotide for the long-term treatment of children with bleeding disorders and angiodysplasia.
AuthorsZühre Kaya, Türkiz Gürsel, Buket Dalgic, Deniz Aslan
JournalPediatric hematology and oncology (Pediatr Hematol Oncol) 2005 Apr-May Vol. 22 Issue 3 Pg. 223-7 ISSN: 0888-0018 [Print] England
PMID16020106 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Octreotide
Topics
  • Adolescent
  • Angiodysplasia (complications, diagnosis, drug therapy)
  • Bernard-Soulier Syndrome (complications, diagnosis, drug therapy)
  • Endoscopy, Gastrointestinal
  • Humans
  • Male
  • Octreotide (administration & dosage, therapeutic use)
  • Stomach Diseases (complications, diagnosis, drug therapy)
  • Time Factors
  • Treatment Outcome

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