| Gastrointestinal manifestations of Sipple syndrome in children. | |
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MedLine Citation:
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PMID: 2888844 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The diagnosis and management of patients with multiple endocrine neoplasia (MEN) type IIA and type IIB are of special challenge to pediatric surgeons. Patients characteristically present early in life with significant intestinal symptoms at a time when the characteristic phenotypic features of MEN IIB are frequently absent. We are reporting 12 patients with MEN type II (9 with type IIA and 3 type IIB or Sipple's syndrome), all of whom presented with gastrointestinal manifestations. All 12 patients had signs and symptoms of bowel obstruction during the neonatal period. An unusual association of Hirschsprung's disease and MEN IIA was noted in our nine patients found among a kindred of 92 individuals. All three patients with Sipple's syndrome (MEN IIB) had severe gastrointestinal symptoms since birth, including recurrent pseudoobstruction. The possibility of MEN type II should be considered in all cases of bowel obstruction in the newborn period. Screening for medullary carcinoma of the thyroid must be carried out from infancy. A detailed family history is very important to avoid unnecessary surgery for bowel obstruction in Sipple's syndrome. |
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Authors:
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A H Khan; J G Desjardins; S Youssef; H Grégoire; E Seidman |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of pediatric surgery Volume: 22 ISSN: 0022-3468 ISO Abbreviation: J. Pediatr. Surg. Publication Date: 1987 Aug |
Date Detail:
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Created Date: 1987-11-17 Completed Date: 1987-11-17 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0052631 Medline TA: J Pediatr Surg Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 719-23 Citation Subset: IM |
Affiliation:
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Department of Pediatric Surgery, Ste-Justine Hospital, Montreal, Quebec, Canada. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Female Ganglioneuroma / diagnosis* Gastrointestinal Diseases / etiology* Hirschsprung Disease / etiology Humans Infant, Newborn Male Mouth Neoplasms / diagnosis Multiple Endocrine Neoplasia / diagnosis* Phenotype Retrospective Studies Thyroid Neoplasms / diagnosis |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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