| Peutz-jegher syndrome in childhood: need for updated recommendations? | |
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MedLine Citation:
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PMID: 23325439 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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BACKGROUND AND AIMS: : We reviewed our institution's experience with Peutz-Jegher syndrome (PJS) in children to determine whether current recommendations on timing of screening and follow-up should be modified. METHODS: : We reviewed the charts of all of the children with a diagnosis of PJS at our institution from 2000 to 2011 abstracting data on intussusceptions events, polyp characteristics, Sertoli cell (SC) tumors, family history, imaging, and interventions. RESULTS: : Of 14 children identified, 10 were boys. Median age at first clinical evaluation was 4.5 years, and family history and/or mucocutaneous pigmentation were the 2 most common factors stimulating screening. Median age at first screening test was 5 years (range 1-16), and at first polyp identification, 5 years (range 1 to 18). There were 7 intussusception events in 5 children, with median age of 10 and range 5 to 16 for first event. Two boys had SC tumors at 8 and 11 years. Polyps were identified during initial screening in 9 of 14 patients. Polyps were found in the stomach or duodenum in 5 (36%), small bowel in 7, (50%) and colon in 3 (21%) children. Large polyps were identified in 9 children at median age of 7 years. CONCLUSIONS: : Polyps causing significant clinical consequences can occur frequently in children with PJS younger than 8 years. Revised guidelines should consider initial screening at age 4 to 5 with capsule endoscopy and upper and lower endoscopy as well as evaluation for SC tumors and re-evaluation whenever symptoms suggest polyp-associated complications. |
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Authors:
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Stephanie A Goldstein; Edward J Hoffenberg |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of pediatric gastroenterology and nutrition Volume: 56 ISSN: 1536-4801 ISO Abbreviation: J. Pediatr. Gastroenterol. Nutr. Publication Date: 2013 Feb |
Date Detail:
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Created Date: 2013-01-17 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8211545 Medline TA: J Pediatr Gastroenterol Nutr Country: United States |
Other Details:
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Languages: eng Pagination: 191-5 Citation Subset: IM |
Affiliation:
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*University of Colorado School of Medicine †Digestive Health Institute, Children's Hospital Colorado and Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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