| Chronic ulcerative gastroduodenitis as a first gastrointestinal manifestation of Hermansky-Pudlak syndrome in a 10-year-old child. | |
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MedLine Citation:
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PMID: 18473428 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Anselm-Chi-Wai Lee; Kin-Hung Poon; Wing-Hong Lo; Lap-Gate Wong |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: World journal of gastroenterology : WJG Volume: 14 ISSN: 1007-9327 ISO Abbreviation: World J. Gastroenterol. Publication Date: 2008 May |
Date Detail:
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Created Date: 2008-05-13 Completed Date: 2008-08-26 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 100883448 Medline TA: World J Gastroenterol Country: China |
Other Details:
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Languages: eng Pagination: 2939-41 Citation Subset: IM |
Affiliation:
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Children's Hematology & Cancer Centre, East Shore Medical Centre #05-01, 319 Joo Chiat Place, 427989, Singapore. aclee@graduate.hku.hk |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Albinism, Oculocutaneous
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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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