The association of syndromic paucity of the interlobular bile ducts and congenital mechanical obstruction of the small intestine
Journal
Journal of Pediatric Gastroenterology and Nutrition
Journal Volume
21
Journal Issue
3
Pages
304-307
Date Issued
1995
Author(s)
Abstract
Three patients with congenital mechanical intestinal obstruction and features of syndromic paucity of the interlobular bile ducts (SPILBD) are presented. Two of them have siblings with syndromic paucity of the interlobular bile ducts. Two patients had jejunoileal atresia, while the other had a meconium plug with intestinal perforation. All subjects underwent surgery for intestinal obstruction within the first 24 h of life. Progressive disappearance of the bile ductules was observed in one case. We believe that an insult such as vascular insufficiency to both bile ductal epithelium and the small intestine may be the cause of this congenital disorder. ? 1995 Lippincott-Raven Publishers, Philadelphia.
SDGs
Other Subjects
adult; article; bile duct malformation; case report; disease association; female; functional anatomy; human; human cell; human tissue; infant; intestine atresia; intestine blood flow; intestine perforation; male; meconium ileus; priority journal; small intestine obstruction; Abnormalities, Multiple; Bile Ducts; Case Report; Female; Human; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Male; Syndrome
Type
journal article