Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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지방간을 가진 소아에서 두개인두종 절제술 후의 뇌하수체기능저하증으로 인해 급격하게 진행된 간-폐 증후군 1예

A case of hepatopulmonary syndrome in a child with fatty liver disease secondary to hypopituitarism after craniopharyngioma resection

  • 임선주 (부산대학교 의과대학 소아과학교실) ;
  • 박현석 (부산대학교 의과대학 소아과학교실) ;
  • 이형두 (부산대학교 의과대학 소아과학교실) ;
  • 박재홍 (부산대학교 의과대학 소아과학교실) ;
  • 박희주 (부산대학교 의과대학 소아과학교실)
  • Im, Sun Ju (Departments of Pediatrics, College of Medicine, Pusan National University) ;
  • Park, Hyeon Seok (Departments of Pediatrics, College of Medicine, Pusan National University) ;
  • Lee, Hyoung Doo (Departments of Pediatrics, College of Medicine, Pusan National University) ;
  • Park, Jae Hong (Departments of Pediatrics, College of Medicine, Pusan National University) ;
  • Park, Hee Ju (Departments of Pediatrics, College of Medicine, Pusan National University)
  • 투고 : 2006.09.12
  • 심사 : 2007.08.03
  • 발행 : 2007.08.15
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초록

간-폐 증후군이란 진행된 간경변을 가진 환자에서 폐혈관의 확장으로 인해 동맥혈 산소 포화도가 감소하는 것을 특징으로 한다. 간경화 환자에서 동맥 산소 포화도의 감소가 보고된 이후로 간-폐 증후군은 말기 간질환을 가진 성인의 13-47% 정도에서 발생한다고 보고하고 있으나, 소아에서 발생한 국내 보고는 없다. 또한 시상하부 및 뇌하수체 기능 저하를 보이는 환자에서 비알코올성 지방간이 급격하게 간부전으로 진행되는 증례가 최근에 보고되고 있다. 저자들은 지방간을 가진 소아에서 두개인두종 절제술 후에 발생한 뇌하수체기능저하증으로 인해 간질환이 급격하게 진행하여 간-폐 증후군으로 발현된 증례를 경험하였기에 보고하는 바이다. 두부 수술 후에 시상하부 및 뇌하수체 기능 저하를 보이는 환아에서는 내분비 질환이나 대사 장애 뿐 아니라, 간질환에 대한 추적 관찰이 필요할 것이다.

Hepatopulmonary syndrome is a triad that includes: hepatic dysfunction, intrapulmonary vascular dilatation and abnormal arterial oxygenation. The incidence of intrapulmonary vascular dilatations, in adults with end-stage liver disease, has been reported to be 13% to 47%, however the incidence in children is unclear and the cases in Korean children have never been reported. The hepatopulmonary syndrome may occur as a result of chronic liver disease following nonalcoholic steatohepatitis in children with hypothalamic or pituitary dysfunction. We report a case of hepatopulmonary syndrome in a 13-year-old child who had rapidly progressive liver dysfunction secondary to panhypopituitarism after craniopharyngioma resection. Careful monitoring and treatment of endocrine abnormalities and metabolic status, as well as liver function, are required in all children undergoing pituitary tumor resection.

키워드

참고문헌

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