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

Atsushi Nakao; Tomohiro Takeda; Yoshiya Hisaeda; Atsushi Hirota; Syusuke Amagata; Yuko Sakurai; Tadashi Kawakami

doi:10.1055/s-0033-1353388

American Journal of Perinatology Reports, Inhaltsverzeichnis

AJP Rep 2013; 03(02): 119-122
DOI: 10.1055/s-0033-1353388

Case Report

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

Authors

Atsushi Nakao

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Tomohiro Takeda

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Yoshiya Hisaeda

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Atsushi Hirota

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Syusuke Amagata

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Yuko Sakurai

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
Tadashi Kawakami

¹Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan

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.

Keywords

pancreatic agenesis - congenital diaphragmatic hernia - congenital heart disease - therapy

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Referenzen

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