Berthe-Aucejo Aurore, Sacquépée Mathieu, Fila Marc, Peuchmaur Michel, Perrier-Cornet Emilia, Frémeaux-Bacchi Véronique, Deschênes Georges
Service de Pharmacie, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.
Service de Néphrologie, Centre Hospitalier Territorial de Nouvelle Calédonie, Gaston Bourret, BP J5, 98849 Nouméa, New Caledonia.
Case Rep Nephrol. 2014;2014:201568. doi: 10.1155/2014/201568. Epub 2014 Feb 6.
A patient aged 17 with dense deposit disease associated with complement activation, circulating C3 Nef, and Factor H mutation presented with nephrotic syndrome and hypertension. Steroid therapy, plasma exchange, and rituximab failed to improve proteinuria and hypertension despite a normalization of the circulating sC5b9 complex. Eculizumab, a monoclonal antibody directed against C5, was used to block the terminal product of the complement cascade. The dose was adapted to achieve a CH50 below 10%, but proteinuria and blood pressure were not improved after 3 months of treatment.
一名17岁的患者患有与补体激活、循环C3 Nef和因子H突变相关的致密沉积物病，表现为肾病综合征和高血压。尽管循环sC5b9复合物恢复正常，但类固醇治疗、血浆置换和利妥昔单抗未能改善蛋白尿和高血压。依库珠单抗是一种针对C5的单克隆抗体，用于阻断补体级联反应的终末产物。调整剂量以使CH50低于10%，但治疗3个月后蛋白尿和血压并未改善。
