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Generalized juvenile polyposis coli. Clinical management based on long-term observations.

Authors
  • Grosfeld, J L
  • West, K W
Type
Published Article
Journal
Archives of Surgery
Publisher
American Medical Association
Publication Date
May 01, 1986
Volume
121
Issue
5
Pages
530–534
Identifiers
PMID: 3707330
Source
Medline
License
Unknown

Abstract

Generalized juvenile polyposis occurred in five patients (age range, 18 months to 16 years). Clinical findings included abdominal pain, weakness, rectal bleeding, diarrhea, rectal prolapse, intussusception, clubbing, and failure to thrive. Laboratory findings included anemia, hypoalbuminemia, hypokalemia, and skin test anergy. Diagnosis is achieved by double contrast enema, endoscopy, and biopsy. Unlike patients with solitary juvenile polyps, patients with generalized involvement require surgical intervention. Subtotal colectomy and ileoproctostomy are the procedures of choice, and we performed them in four cases. An ileoanal-endorectal pull-through procedure was required in one patient with continued rectal disease. All five patients are currently alive and well. Long-term follow-up is important as polyps may persist into adult life. Family members are at risk for developing gastrointestinal tract tumors and should be screened.

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