Bogdanović R, Komar P, Cvorić A, Nikolić V, Sinotić M, Zdravković D, Ognjanović M, Abinun M
Institute of Mother and Child Health, Belgrade, Yugoslavia.
Nephron. 1994;66(2):219-24. doi: 10.1159/000187804.
The association of a spondyloepiphyseal dysplasia and disproportionate short stature with focal glomerular sclerosis is reported in two girls. Renal disease manifested by proteinuria at the age of 2.5 and 11 years, leading to treatment-resistant nephrotic syndrome over 15 and 45 months, respectively. One patient went into end-stage renal failure shortly after nephrotic syndrome developed, the other died from sepsis. The association of spondyloepiphyseal dysplasia and focal glomerular sclerosis with nephrotic syndrome may represent a distinct disease entity.
两名女孩被报道患有脊椎骨骺发育不良、身材不成比例矮小并伴有局灶性节段性肾小球硬化。肾脏疾病分别在2.5岁和11岁时表现为蛋白尿,分别在15个月和45个月内发展为难治性肾病综合征。一名患者在肾病综合征出现后不久进入终末期肾衰竭,另一名患者死于败血症。脊椎骨骺发育不良与局灶性节段性肾小球硬化合并肾病综合征可能代表一种独特的疾病实体。