Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Macroamylasemia in a 4-year-old girl with abdominal pain

복통을 주소로 내원한 4세 여아에서 진단된 마크로아밀라제혈증 1예

  • Ko, Jeong Hee (Department of Pediatrics, College of Medicine, Hallym University) ;
  • Lee, Dae Hyoung (Department of Pediatrics, College of Medicine, Hallym University)
  • 고정희 (한림대학교 의과대학 소아과학교실) ;
  • 이대형 (한림대학교 의과대학 소아과학교실)
  • Received : 2009.07.21
  • Accepted : 2009.10.05
  • Published : 2009.11.15
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Abstract

Macroamylasemia is a benign condition characterized by abnormally large-sized serum amylase; it has been reported to occur in 1-2% of the population. In macroamylasemia, a macromolecular complex consisting of amylase linked to immunoglobulins circulates in the plasma and usually causes hyperamylasemia with low or normal amylasuria. Macroamylasemia is extremely rare in children. We report a case of a 4-year-old girl with abdominal pain and macroamylasemia, who was initially misdiagnosed as having acute pancreatitis. Failure to immediately identify macroamylase as the cause of the unexplained but benign hyperamylasemia can lead to the misdiagnosis of the condition, necessitating costly analyses for ruling out pancreatic disease and unnecessary prescriptions such as fasting and intravenous replacement therapies, as was observed in our patient.

마크로아밀라아제혈증은 대개 혈중 아밀라아제 분자의 크기가 큰 양성 질환으로 1-2% 까지 보고된다. 마크로아밀라아제혈증에서는 아밀라아제가 면역 글로불린과 결합하여 고분자 복합체를 형성하여 소변을 통한 배출이 정상 혹은 저하되어 혈청 내 고아밀라아제혈증을 일으킨다. 소아에서는 매우 드문 질환이다. 본 저자들은 초기 급성 췌장염으로 오인된 4세 여아가 마크로아밀라아제혈증으로 진단되어 보고하는 바이다. 고아밀라아제혈증의 원인중 하나인 마크로아밀라아제혈증을 조기 진단하지 못하면 췌장질환과 감별을 위한 고 비용의 검사를 하게 되고 금식이나 정맥 영양과 같은 필요 없는 치료를 할 수 있다.

Keywords

References

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