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家族性肾病综合征为遗传性进行性肾脏疾病,甚为少见,现报道一家3例。观察时间最短者4个月,最长者达5年。例1 女,2岁半,第1胎。8个月早产。因全身浮肿、少尿、并逐渐加重于1971年1月15日入院。体检:血压10.7/8KPa,神志清,颜面和全身呈可凹性浮肿,以下肢明显。咽充血,心音有力,肺背部可闻少量湿性罗音。腹部叩诊呈移动性浊音,肝脾未触及。实验室检查:尿蛋白(+++),红细胞0—1。血浆蛋白50g/L,白蛋白28g/L,球蛋白22g/L。胆固醇7mmol/L。诊断为肾病综合征。入院后,经用泼尼松(2mg/Kg)治疗,于同年3月7日病情好转出院。出院时无浮
Familial nephrotic syndrome is a hereditary progressive renal disease, is rare, is now reported in 3 cases. The shortest observation time of 4 months, the longest up to 5 years. Example 1 female, 2 years and a half, first child. 8 months premature. Due to systemic edema, oliguria, and gradually increased in January 15, 1971 admission. Physical examination: blood pressure 10.7 / 8KPa, clear mind, face and body were concave edema, obvious to the lower limbs. Pharyngeal hyperemia, strong heart sounds, lungs can smell a small amount of wet rales back. Abdominal percussion was mobile dullness, liver and spleen not touched. Laboratory tests: urinary protein (+++), red blood cells 0-1. Plasma protein 50g / L, albumin 28g / L, globulin 22g / L. Cholesterol 7mmol / L. Diagnosis of nephrotic syndrome. After admission, with prednisone (2mg / Kg) treatment, in March 7 the same year the condition improved and discharged. No floating when discharged from hospital