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...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Thieme Medical Publishers, Inc.
2013-08-01
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| Series: | American Journal of Perinatology Reports |
| Subjects: | |
| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0033-1353388 |
| _version_ | 1818159386038632448 |
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| author | Atsushi Nakao Tomohiro Takeda Yoshiya Hisaeda Atsushi Hirota Syusuke Amagata Yuko Sakurai Tadashi Kawakami |
| author_facet | Atsushi Nakao Tomohiro Takeda Yoshiya Hisaeda Atsushi Hirota Syusuke Amagata Yuko Sakurai Tadashi Kawakami |
| author_sort | Atsushi Nakao |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-11T15:45:09Z |
| format | Article |
| id | doaj.art-feacb7371dae4cc0ac3db1390ca527d9 |
| institution | Directory Open Access Journal |
| issn | 2157-6998 2157-7005 |
| language | English |
| last_indexed | 2024-12-11T15:45:09Z |
| publishDate | 2013-08-01 |
| publisher | Thieme Medical Publishers, Inc. |
| record_format | Article |
| series | American Journal of Perinatology Reports |
| spelling | doaj.art-feacb7371dae4cc0ac3db1390ca527d92022-12-22T00:59:43ZengThieme Medical Publishers, Inc.American Journal of Perinatology Reports2157-69982157-70052013-08-01030211912210.1055/s-0033-1353388Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case ReportAtsushi Nakao0Tomohiro Takeda1Yoshiya Hisaeda2Atsushi Hirota3Syusuke Amagata4Yuko Sakurai5Tadashi Kawakami6Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanDepartment of Neonatology, Japanese Red Cross Medical Center, Tokyo, JapanAbstract 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.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0033-1353388pancreatic agenesiscongenital diaphragmatic herniacongenital heart diseasetherapy |
| spellingShingle | Atsushi Nakao Tomohiro Takeda Yoshiya Hisaeda Atsushi Hirota Syusuke Amagata Yuko Sakurai Tadashi Kawakami Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report American Journal of Perinatology Reports pancreatic agenesis congenital diaphragmatic hernia congenital heart disease therapy |
| title | Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report |
| title_full | Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report |
| title_fullStr | Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report |
| title_full_unstemmed | Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report |
| title_short | Pancreatic Agenesis with Congenital Diaphragmatic Hernia and Congenital Heart Disease: A Case Report |
| title_sort | pancreatic agenesis with congenital diaphragmatic hernia and congenital heart disease a case report |
| topic | pancreatic agenesis congenital diaphragmatic hernia congenital heart disease therapy |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0033-1353388 |
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