先天性肾病国内报道较少,现将我们见到同胞兄妹同患先天性肾病(芬兰型)报告如下。例1.男,56天。第1胎。孕35周,于1981年10月23日顺产,体重2.1kg,胎盘(26×28×2.5cm),约为正常的2.5倍。父母非近亲婚配。母孕期无服特殊药物史。生后第1周出现会阴部、下腹壁皮肤发硬、发亮,继之肿胀。并渐蔓延至双下肢,足背压之有凹痕。时常出现呼吸困难、紫绀及四肢轻度抽搐。二便无异常。病情不见缓解,于1981年12月18日因阵发性呼吸困难,明显紫绀入院。查体:反应差,重病容,消瘦。面颊、躯干及双大腿皮肤发硬,无弹性,双下肢凹陷性水肿。肢凉,皮肤有花斑纹。心界不大。肺部无罗音,右下肺呼吸音弱。腹部膨隆,移动性浊音(+),肝脾触诊不清。血常规:红细胞2.05×10~(12)/ Congenital kidney disease less reported in the country, now we see siblings with siblings with congenital nephropathy (Finnish type) report is as follows. Example 1. Male, 56 days. First child. Pregnancy 35 weeks, on October 23, 1981 birth, weight 2.1kg, placenta (26 × 28 × 2.5cm), about 2.5 times the normal. Parents non-relatives marriage. Pregnancy unobtrusive special drug history. Perineal appeared in the first week after birth, the skin under the abdominal wall hair hard, shiny, followed by swelling. And gradually spread to the lower extremities, foot back pressure dent. Breathing difficulties often occur, cyanosis and limbs mild convulsions. Two will be no exception. The disease did not ease, on December 18, 1981 due to paroxysmal respiratory distress, obviously cyanosis admission. Physical examination: poor response, serious illness, weight loss. Cheeks, torso and thigh skin stiff, inelastic, both lower extremity edema. Cold limbs, skin patterns have markings. Heart is small. Lung no Luo sound, lower right lung breath sounds weak. Abdominal bulging, shifting dullness (+), palpation of liver and spleen unclear. Blood: red blood cells 2.05 × 10 ~ (12) /