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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2019.1
- 수록면
- 43 - 47 (5page)
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Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (anti- CFH-aHUS) primarily affects children aged 9–13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFHaHUS) in Laos.
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참고문헌 (15)
참고문헌 신청
Noris M / 2009 / Atypical hemolytic-uremic syndrome / N Engl J Med 361:1676~1687
Lee JM / 2015 / Atypical hemolytic uremic syndrome : Korean pediatric series / Pediatr Int 57:431~438
Fremeaux-Bacchi V / 2013 / Genetics and outcome of atypical hemolytic uremic syndrome : A nationwide French series comparing children and adults / Clin J Am Soc Nephrol 8:554~562
ragon-Durey MA / 2005 / Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome / J Am Soc Nephrol 16:555~563
Lee BH / 2009 / Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency / Pediatr Res 66:336~340
Lee JM / 2015 / Atypical hemolytic uremic syndrome : Korean pediatric series / Pediatr Int 57:431~438
Fremeaux-Bacchi V / 2013 / Genetics and outcome of atypical hemolytic uremic syndrome : A nationwide French series comparing children and adults / Clin J Am Soc Nephrol 8:554~562
ragon-Durey MA / 2005 / Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome / J Am Soc Nephrol 16:555~563
Lee BH / 2009 / Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency / Pediatr Res 66:336~340
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