The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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A Case of Complete Agenesis of the Dorsal Pancreas with Left Renal Agenesis and Absence of the Left Vertebral Pedicle in T12

좌측 신장 무형성과 척추기형을 동반한 선천적 배측 췌장 발육 부전증 1예

  • Ryu, Ah-Jeong (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Lee, Jae-Jun (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Moon, Hyeon-Jin (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Jeon, Seok-Jae (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Park, Soon-Hyeon (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Lee, Jae-Min (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine) ;
  • Park, Kang-Seo (Department of Internal Medicine, Eulji University Hospital, Eulji University School of Medicine)
  • 류아정 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 이재준 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 문현진 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 전석재 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 박순현 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 이재민 (을지대학교 의과대학 을지대학교병원 내과) ;
  • 박강서 (을지대학교 의과대학 을지대학교병원 내과)
  • Published : 2012.08.01
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Abstract

Agenesis of the dorsal pancreas is a rare congenital anomaly that arises from the failure of the dorsal pancreatic bud of endodermal cells to form the body and tail of the pancreas and can manifest as diabetes. A 24-year-old man, who had been treated with insulin for 7 years, presented with epigastric pain, vomiting, and watery diarrhea. Abdominal computed tomography showed only the head of the pancreas without visualization of the pancreatic body and tail. Left renal agenesis and absence of the left vertebral pedicle in T12 were also observed. The duct of Santorini and the duct in the body and tail were not visible in magnetic resonance cholangiopancreatography. The associated anomalies reported here are very rare globally. We report a case of complete agenesis of the dorsal pancreas with multiple congenital abnormalities and diabetes mellitus.

7년 전부터 당뇨병으로 인슐린 치료 중이던 24세 남자 환자가 내원일 오전에 발생한 복부 불편감을 주소로 내원하였고, 복통에 대한 검사 중 복부 전산화 단층촬영 결과 배측췌장 발육 부전증과 좌측 신장 및 12번 흉추의 좌측 추궁근 결손이 발견되었다. 자기공명 담췌관조영술에서 주 췌관은 정상적이었으며 산토리니관(Santorini-duct) 및 체부와 미부의 췌관은 관찰되지 않았다. 저자는 복부 전산화 단층촬영과 자기공명 담췌관조영술을 통해 선천적 배측 췌장 발육 부전증과 동반된 신장 및 척추의 기형을 진단하였고, 이에 다발성 선천성 기형과 당뇨병이 동반된 배측 췌장 발육 부전증 1예를 보고하는 바이다.

Keywords

References

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