남매에서 발생한 C1q 신증 2례
Two Cases of C1q Nephropathy in Siblings
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초록
C1q 신병증은 1985년 Jennett와 Hipp에 의해 발표된 이래 어린 영아에서부터 청 장년층에 까지 발표되어 왔으나 아직 임상병리학적으로 논란이 많은 질환이다. 저자들은 어린 남매에서 스테로이드 저항성의 콩팥증후군의 임상 양상을 보이며 병리 조직학적으로 국소분절사구체경화증과 메산지움에 C1q의 현저한 침착을 보인, C1q 신증을 발표하는 바이다.
Abstract
C1q nephropathy is a distinct clinicopathologic entity, characterized by mesangial immunoglobulin and complement deposits, predominantly C1q, with no evidence for systemic lupus erythematosus. Clinically it may present as nephrotic syndrome and non-nephrotic proteinuria per se or associated with microscopic hematuria, gross hematuria, hypertension, or renal insufficiency. So far there is only one report about a familial case of C1q nephropathy (in two sisters). We present two cases of familial C1q nephropathy with nephrotic syndrome which was steroid resistant, but partially remitted with cyclosporine
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