Document Detail


Chronic ulcerative gastroduodenitis as a first gastrointestinal manifestation of Hermansky-Pudlak syndrome in a 10-year-old child.
MedLine Citation:
PMID:  18473428     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 10-year-old Chinese boy who had a history of congenital thrombocytopathy presented with severe iron deficiency anemia secondary to chronic gastric inflammation and duodenal ulcerations. Subtle oculocutaneous albinism led to the finding of diminished dense bodies in the platelets under electron microscopy, hence the diagnosis of Hermansky-Pudlak syndrome (HPS). Biopsies from the stomach and duodenum revealed a lymphocytic infiltration in the submucosa, but H pylori infection was absent. The gastroduodenitis responded to the treatment with omeprazole while iron deficiency anemia was corrected by oral iron therapy. HPS is a rare cause of congenital bleeding disorder with multisystemic manifestations. Upper gastrointestinal involvement is rare and should be distinguished from a mere manifestation of the bleeding diathesis.
Authors:
Anselm-Chi-Wai Lee; Kin-Hung Poon; Wing-Hong Lo; Lap-Gate Wong
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  World journal of gastroenterology : WJG     Volume:  14     ISSN:  1007-9327     ISO Abbreviation:  World J. Gastroenterol.     Publication Date:  2008 May 
Date Detail:
Created Date:  2008-05-13     Completed Date:  2008-08-26     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  100883448     Medline TA:  World J Gastroenterol     Country:  China    
Other Details:
Languages:  eng     Pagination:  2939-41     Citation Subset:  IM    
Affiliation:
Children's Hematology & Cancer Centre, East Shore Medical Centre #05-01, 319 Joo Chiat Place, 427989, Singapore. aclee@graduate.hku.hk
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MeSH Terms
Descriptor/Qualifier:
Albinism, Oculocutaneous / diagnosis,  pathology
Child
Diagnosis, Differential
Duodenal Ulcer / diagnosis*,  etiology
Gastroenteritis / diagnosis*,  etiology
Hermanski-Pudlak Syndrome / complications,  diagnosis*,  pathology
Humans
Male
Comments/Corrections

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