Gulati S, Sural S, Sharma R K, Gupta A, Gupta R K
Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India.
Pediatr Nephrol. 2001 Dec;16(12):1045-8. doi: 10.1007/s004670100023.
There are few data regarding adolescent-onset nephrotic syndrome (NS) and no guidelines for biopsy criteria and treatment protocol. This study was conducted to analyze the clinical spectrum of adolescent-onset NS and evaluate possible biopsy criteria in these children. A prospective analysis was carried out on all patients with idiopathic NS (fulfilling the ISKDC criteria) with onset between 1 and 18 years of age. They were evaluated clinically, followed by biochemical investigations and kidney biopsy. These characteristics of patients with onset between 1 and 12 years (group A) were compared with the same parameters in patients with onset between 12 and 18 years of age (group B) referred to our hospital over the same period. Among all clinical parameters, microhematuria was significantly more prevalent in adolescents (P<0.001). Kidney biopsy was performed in 88% of adolescent patients. Focal segmental glomerulosclerosis (FSGS) was the most-common histopathology in group B (46.3%) compared with minimal change disease (MCD) in group A (42.9%). Group B had a significantly higher frequency of membranoproliferative glomerulonephritis (MPGN) (P<0.005) and a significantly lower frequency of MCD (P<0.001). The biochemical parameters at the onset were similar. On comparing microhematuria, hypertension, and renal insufficiency at presentation, we observed that two or more of these features were present in all patients with MPGN and only in 19.6% of adolescents with MCD, mesangioproliferative glomerulonephritis, and FSGS. The frequency of steroid resistance was significantly higher in group B (P<0.001). In conclusion, adolescent-onset NS differs from the childhood variety in having a significantly higher frequency of hematuria, steroid resistance, and evidence of non-MCD, especially MPGN, on histopathology. Kidney biopsy can be restricted to those adolescents who have at least two abnormal clinical/biochemical features or are steroid non-responders.
关于青少年起病的肾病综合征(NS)的数据较少,且尚无活检标准和治疗方案的指南。本研究旨在分析青少年起病的NS的临床谱,并评估这些儿童可能的活检标准。对所有1至18岁起病的特发性NS(符合ISKDC标准)患者进行了前瞻性分析。对他们进行了临床评估,随后进行了生化检查和肾活检。将1至12岁起病的患者(A组)的这些特征与同期转诊至我院的12至18岁起病的患者(B组)的相同参数进行比较。在所有临床参数中,镜下血尿在青少年中明显更为常见(P<0.001)。88%的青少年患者进行了肾活检。与A组中微小病变性肾病(MCD)(42.9%)相比,局灶节段性肾小球硬化(FSGS)是B组中最常见的组织病理学类型(46.3%)。B组中膜增生性肾小球肾炎(MPGN)的发生率显著更高(P<0.005),而MCD的发生率显著更低(P<0.001)。起病时的生化参数相似。在比较就诊时的镜下血尿、高血压和肾功能不全时,我们观察到所有MPGN患者均存在这些特征中的两项或更多项,而在患有MCD、系膜增生性肾小球肾炎和FSGS的青少年中仅19.6%存在这些特征。B组中激素抵抗的发生率显著更高(P<0.001)。总之,青少年起病的NS与儿童期NS不同,其血尿、激素抵抗的发生率显著更高,且在组织病理学上有非MCD尤其是MPGN的证据。肾活检可限于那些至少有两项异常临床/生化特征或对激素无反应的青少年。