Hereditary multiple atresias of the gastrointestinal tract: report of a case and review of the literature.
J Pediatr Surg
; 33(5): 794-7, 1998 May.
Article
em En
| MEDLINE
| ID: mdl-9607506
ABSTRACT
Hereditary multiple atresia of the gastrointestinal tract is an extremely rare subgroup of intestinal atresia. The aim of this study was to report a new case, to review the literature, and to describe the unique features of this malformation. A computer-generated list of articles on this subject was obtained, and all articles relative to this malformation were reviewed. Thirty-five other well-documented cases were found in the literature. Hereditary multiple atresias have several unique features (1) the abdominal x-ray shows signs of gastric or duodenal atresia combined with typical large rounded or oval homogeneous calcifications in the abdominal cavity, (2) intraoperatively widespread atresias (exclusively type I and II) extending mostly from stomach to rectum are found, (3) cystic dilatation of the bile ducts can be present in cases with both complete pyloric and duodenal or proximal jejunal atresia, (4) the pathogenesis is still speculative; a combined immunodeficiency should be excluded, and (5) a fatal outcome is the rule.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Anormalidades Múltiplas
/
Obstrução Duodenal
/
Atresia Intestinal
/
Intestino Grosso
/
Intestino Delgado
Tipo de estudo:
Diagnostic_studies
Limite:
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
J Pediatr Surg
Ano de publicação:
1998
Tipo de documento:
Article
País de afiliação:
Alemanha