AI Article Synopsis
- Heterotaxy syndrome (HS) is a congenital disorder characterized by the abnormal placement of internal organs, often associated with cardiovascular and digestive system defects.
- A case study discusses a 13-year-old girl who developed diabetes mellitus due to dorsal pancreatic agenesis, along with HS manifesting as a double-outlet right ventricle, pulmonary stenosis, and intestinal malrotation.
- The girl's condition was effectively managed with timely diagnosis and insulin treatment, leading to improved blood sugar control.
Article 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.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5495979 | PMC |
| http://dx.doi.org/10.6065/apem.2017.22.2.125 | DOI Listing |
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