Lagrue G, Branellec A, Niaudet P, Heslan J M, Guillot F, Lang P
Service de Néphrologie, Association Claude-Bernard, INSERM U 139, Hôpital Henri-Mondor, Créteil.
Presse Med. 1991 Feb 16;20(6):255-7.
We report a transient neonatal nephrotic syndrome in two infants born to a mother with idiopathic nephrotic syndromes (INS). The mother, born in Mali in 1966, had a normal first pregnancy, with a normal live child. Six months later a nephrotic syndrome appeared with normal renal function; renal biopsy disclosed slight lesions of focal and segmental glomerulosclerosis. Prednisone and ciclosporin were totally ineffective. In 1986 and 1988 two pregnancies occurred with normal gestation outcome; however, the two births were premature (35 weeks) with hypotrophic infantas (2.160 and 2.080 kg). In both cases urine analysis revealed neonatal heavy proteinuria, with low serum protein and hypoalbuminemia; proteinuria decreased and disappeared within 2 and 3 weeks respectively; simultaneously protidemia and albuminemia were normalized. Thus, a transitory nephrotic syndrome, resolving spontaneously, occurred in two successive offsprings of a patient with INS. These cases are in keeping with the hypothesis that heavy proteinuria in nephrotic syndrome might be linked to a circulating humoral factor and that INS is a disorder linked to T-lymphocyte function with enhanced production of a lymphokine acting on vascular permeability.
我们报告了两例患有特发性肾病综合征（INS）母亲所生婴儿的短暂性新生儿肾病综合征。这位母亲于1966年出生在马里，第一次怀孕正常，生下一个健康的活产婴儿。六个月后出现肾病综合征，肾功能正常；肾活检显示局灶性节段性肾小球硬化的轻微病变。泼尼松和环孢素完全无效。1986年和1988年又有两次怀孕，妊娠结局正常；然而，两次分娩均早产（35周），婴儿发育不良（分别为2.160和2.080千克）。在这两例中，尿液分析均显示新生儿重度蛋白尿，血清蛋白降低和低白蛋白血症；蛋白尿分别在2周和3周内减少并消失；同时，蛋白血症和白蛋白血症恢复正常。因此，一名INS患者的两个连续后代均出现了自发缓解的短暂性肾病综合征。这些病例符合以下假设：肾病综合征中的重度蛋白尿可能与循环体液因子有关，而INS是一种与T淋巴细胞功能相关的疾病，其淋巴因子产生增加，作用于血管通透性。
