Document Detail

Peutz-jegher syndrome in childhood: need for updated recommendations?
MedLine Citation:
PMID:  23325439     Owner:  NLM     Status:  In-Data-Review    
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.
Stephanie A Goldstein; Edward J Hoffenberg
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric gastroenterology and nutrition     Volume:  56     ISSN:  1536-4801     ISO Abbreviation:  J. Pediatr. Gastroenterol. Nutr.     Publication Date:  2013 Feb 
Date Detail:
Created Date:  2013-01-17     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8211545     Medline TA:  J Pediatr Gastroenterol Nutr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  191-5     Citation Subset:  IM    
*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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