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Macroamylasemia in a 4-year-old girl with abdominal pain

Korean Journal of Pediatrics 2009;52(11):1283-1285.
Published online November 15, 2009.
Macroamylasemia in a 4-year-old girl with abdominal pain
Jeong Hee Ko, Dae Hyoung Lee
Department of Pediatrics, College of Medicine, Hallym University, Seoul, Korea
복통을 주소로 내원한 4세 여아에서 진단된 마크로아밀라제혈증 1예
고정희, 이대형
한림대학교 의과대학 소아과학교실
Dae Hyoung Lee, Email: mdleedh@hallym.or.kr
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.
Key Words: Macroamylasemia, Hyperamylasemia, Pancreatitis

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