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Journal of the Korean Society of Pediatric Nephrology 2006;10(1): 45-51.
수막구균 뇌수막염과 보체 결핍이 동반된 막증식성 사구체신염 1례
권상미, 박관규, 이경훈
1대구가톨릭대학교 의과대학 소아과학교실
2대구가톨릭대학교 의과대학 병리학교실
3대구가톨릭대학교 의과대학 소아과학교실
A Case of Membranoproliferative Glomerulonephritis Associated with Complement Deficiency and Meningococcal Meningitis
Sang-Mi Kwon, Kwan-Kyu Park, Gyeong-Hoon Lee
1Department of Pediatrics, School of Medicine, Catholic University of Daegu
2Department of Pathology, School of Medicine, Catholic University of Daegu
3Department of Pediatrics, School of Medicine, Catholic University of Daegu
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ABSTRACT
Hypocomplementemia is found in all types of membranoproliferative glomerulonephritis (MPGN) but not in all patients. Hypocomplementemia can be ascribed to at least two circulating complement reactive modalities. The activation of the classical pathway produced by circulating immune complexes and the presence in the blood of anticomplement autoantibodies, called 'nephritic factor'(NF). The activation of the classical pathway by circulating immune complexes is probably the major mechanism responsible for hypocomplementemia in idiopathic MPGN type I. Nephritic factors have been shown to be responsible for the hypocomplementemia in both MPGN type II and III. NFa is probably the major mechanism responsible for the hypocomplementemia of idiopathic MPGN type II. NFt appears to be solely responsible for the hypocomplementemia in MPGN type III. Judging from the complement profile, NFt also may be present in some patients with MPGN type I. Although infection by meningococcus has been associated with deficiency of any of the plasmatic proteins of complement, it more commonly involves deficiency of the terminal components of the complement pathway(C5-C9). We experienced a patient who had MPGN and meningococcal meningitis. We examined the complement level and significantly lower levels of C3, C5 were found persistently. C7 was low at first and it returned to normal range after 2 months. C9 was normal at first, and was low after 2 months. This is the first reported case in which MPGN with meningococcal meningitis occurred.
Key words: Membranoproliferative glomerulonephritis | Complement deficiency | Nephritic factor | Meningococcal meningitis
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