Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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복통을 주소로 내원한 4세 여아에서 진단된 마크로아밀라제혈증 1예

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

  • 고정희 (한림대학교 의과대학 소아과학교실) ;
  • 이대형 (한림대학교 의과대학 소아과학교실)
  • Ko, Jeong Hee (Department of Pediatrics, College of Medicine, Hallym University) ;
  • Lee, Dae Hyoung (Department of Pediatrics, College of Medicine, Hallym University)
  • 투고 : 2009.07.21
  • 심사 : 2009.10.05
  • 발행 : 2009.11.15
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초록

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.

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.

키워드

참고문헌

  1. Wilding P, Cooke WT, Nicholson GI. Globulin-bound amylase: a cause of persistently elevated levels in serum. Ann Intern Med 1964;60:1053-9 https://doi.org/10.7326/0003-4819-60-6-1053
  2. Berk JE, Kizu H, Wilding P, Searcy RL. Macroamylasemia: a newly recognized cause for elevated serum amylase activity. N Engl J Med 1967;277:941-6 https://doi.org/10.1056/NEJM196711022771801
  3. Barrows D, Berk JE, Fridhandler L. Macroamylasemia: survey of prevalence in a mixed population. N Engl J Med 1972;286:1352 https://doi.org/10.1056/NEJM197206222862509
  4. Helfat A, Berk JE, Fridhandler L. The prevalence of macroamylasemia. Further study. Am J Gastroenterol 1974;62:54- 8
  5. Van Gossum A. Macroamylasemia: a biochemical or clinical problem? Dig Dis 1989;7:19-27 https://doi.org/10.1159/000171203
  6. Kameya S, Hayakawa T, Kameya A, Watanabe T. Clinical value of routine isoamylase analysis of hyperamylasemia. Am J Gastroenterol 1986;81:358-64 https://doi.org/10.1111/j.1572-0241.1986.tb01498.x
  7. Levitt MD, Rapoport M, Cooperband SR. The renal clearance of amylase in renal insufficiency, acute pancreatitis, and macroamylasemia. Ann Intern Med. 1969;71:919-25 https://doi.org/10.7326/0003-4819-71-5-919
  8. Kobayashi T, Nakayama T, Kitamura M. Electrophoretic identification of serum immunoglobulins linked to amylase: macroamylase. Clin Chim Acta 1978;86:261-5 https://doi.org/10.1016/0009-8981(78)90380-7
  9. Klonoff DC. Macroamylasemia and other immunoglobulin- complexed enzyme disorders. West J Med 1980;133:392-407
  10. Barera G, Bazzigaluppi E, Viscardi M, Renzetti F, Bianchi C, Chiumello G, et al. Macroamylasemia attributable to gluten- related amylase autoantibodies: a case report. Pediatrics 2001;107:E93 https://doi.org/10.1542/peds.107.6.e93
  11. Yoshida K, Minegishi Y, Okawa H, Yata J, Tokoi S, Kitagawa T, et al. Epstein-Barr virus-associated malignant lymphoma with macroamylasemia and monoclonal gammopathy in a patient with Wiskott-Aldrich syndrome. Pediatr Hematol Oncol 1997;14:85-9
  12. Forsman RW. Macroamylase: prevalence, distribution of age, sex, amylase activity, and electrophoretic mobility. Clin Biochem 1986;19:250-3 https://doi.org/10.1016/S0009-9120(86)80036-4
  13. Um JW, Kim KH, Kang MS, Choe JH, Bae JW, Hong YS, et al. Macroamylasemia in a patient with acute appendicitis: a case report. J Korean Med Sci 1999;14:679-81 https://doi.org/10.3346/jkms.1999.14.6.679
  14. Lim PS, Kim SY, Yeom DH, Jo EY, Choi CS, Kim HC, et al. Macroamylasemia and macrolipasemia in patient with alcoholic liver cirrhosis. Korean J Med 2008;75:459-62

피인용 문헌

  1. Transient Macroamylasemia in a Severely Multiple-Handicapped Child Following the Development of Acute Bronchitis vol.5, pp.3, 2009, https://doi.org/10.4236/ojped.2015.53035