Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report

Abstract Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus...

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Bibliographic Details
Published inAmerican journal of perinatology reports Vol. 3; no. 2; pp. 119 - 122
Main Authors Nakao, Atsushi, Takeda, Tomohiro, Hisaeda, Yoshiya, Hirota, Atsushi, Amagata, Syusuke, Sakurai, Yuko, Kawakami, Tadashi
Format Journal Article
LanguageEnglish
Published 333 Seventh Avenue, New York, NY 10001, USA Thieme Medical Publishers 01.10.2013
Thieme Medical Publishers, Inc
Subjects
Case Report
congenital diaphragmatic hernia
congenital heart disease
pancreatic agenesis
therapy
congenital heart disease
congenital diaphragmatic hernia
therapy
pancreatic agenesis
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Summary:Abstract Pancreatic agenesis is a rare disease that causes neonatal diabetes mellitus and exocrine pancreatic insufficiency. We report the case of a very low birth weight infant with congenital diaphragmatic hernia, and congenital heart disease (ventricular septal defect and patent ductus arteriosus). Failure to gain weight, despite well-managed respiratory and heart failure, was improved by infusion of subcutaneous insulin, administration of pancreatic enzyme, and nutrition of medium-chain-triglyceride -enriched formula. This is the first case of pancreatic agenesis with both malformations where the patient is discharged from the hospital. Early diagnosis and adequate treatments to compensate pancreatic function may prevent mortality and improve growth.
ISSN:2157-6998
2157-7005
DOI:10.1055/s-0033-1353388