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Diabetes mellitus due to agenesis of the dorsal pancreas in a patient with heterotaxy syndrome

Authors
 Jo Eun Jung  ;  Jin Ho Hur  ;  Mo Kyung Jung  ;  Ahreum Kwon  ;  Hyun Wook Chae  ;  Duk Hee Kim  ;  Ho-Seong Kim 
Citation
 ANNALS OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, Vol.22(2) : 125-128, 2017 
Journal Title
 ANNALS OF PEDIATRIC ENDOCRINOLOGY & METABOLISM 
ISSN
 2287-1012 
Issue Date
2017
Keywords
Diabetes mellitus ; Dorsal pancreas agenesis ; Heterotaxy syndrome
Abstract
Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.
Files in This Item:
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DOI
10.6065/apem.2017.22.2.125
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kwon, Ah Reum(권아름) ORCID logo https://orcid.org/0000-0002-9692-2135
Kim, Ho Seong(김호성) ORCID logo https://orcid.org/0000-0003-1135-099X
Jung, Mo Kyung(정모경)
Chae, Hyun Wook(채현욱) ORCID logo https://orcid.org/0000-0001-5016-8539
Hur, Jin Ho(허진호)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/160380
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