Schärer K, Essigmann H C, Schaefer F
Division of Pediatric Nephrology, University Children's Hospital, Im Neuenheimer Feld 150, D-69120 Heidelberg, Germany.
Pediatr Nephrol. 1999 Nov;13(9):828-34. doi: 10.1007/s004670050709.
Whilst it is assumed that body growth is retarded in children with steroid-resistant nephrotic syndrome (NS), the degree of growth failure and the pathomechanisms involved are poorly understood. We collected serial growth data in 45 children (24 males) with steroid-resistant NS usually from onset to end-stage renal disease (ESRD) during childhood (n=10) or until final height was attained (n=27). Mean follow-up time was 9 (2-19) years. Mean initial standardized height was -0.3+/-1.2 standard deviation scores (SDS). Mean final height was +0.4 SDS in males and -1.0 SDS in females (sex difference not significant). In 16 patients with serum creatinine levels consistently <1.2 mg/dl, mean final height SDS was 0.3 SDS higher than that obtained within 6 months of onset. In contrast, 9 children who entered ESRD lost an average of 1.3 SDS from the initial record to ESRD (P=0.017). In prepubertal patients without renal insufficiency, mean height SDS decreased during corticosteroid treatment by 0.3 SDS, followed by a partial catch-up after discontinuation of treatment; the change from initial to final height SDS was inversely correlated with the total prednisone dose given (r=-0.50, P=0.03). In 16 prepubertal children with serial height and serum protein measurements who were off steroids and maintained normal creatinine levels, mean individual albumin concentrations correlated with the change in height SDS per year (r=0.65, P=0.0006) and in boys with final height (r=0.73, P=0.03). In conclusion, growth in steroid-resistant NS depends on the preservation of renal function, the cumulative dose of steroids applied, and the severity of hypoproteinemia.
虽然一般认为类固醇抵抗性肾病综合征(NS)患儿的身体生长会受到抑制,但对生长发育迟缓的程度及相关发病机制却知之甚少。我们收集了45例(24例男性)类固醇抵抗性NS患儿的系列生长数据,这些数据通常从发病开始,直至儿童期进入终末期肾病(ESRD)(n = 10),或直至达到最终身高(n = 27)。平均随访时间为9(2 - 19)年。平均初始标准化身高为 -0.3 ± 1.2标准差评分(SDS)。男性的平均最终身高为 +0.4 SDS,女性为 -1.0 SDS(性别差异不显著)。16例血清肌酐水平持续 <1.2 mg/dl的患者,平均最终身高SDS比发病后6个月内测得的数值高0.3 SDS。相比之下,9例进入ESRD的患儿从初始记录到ESRD平均身高SDS下降了1.3 SDS(P = 0.017)。在青春期前无肾功能不全的患者中,皮质类固醇治疗期间平均身高SDS下降了0.3 SDS,治疗停止后出现部分追赶生长;从初始到最终身高SDS的变化与泼尼松总剂量呈负相关(r = -0.50,P = 0.03)。在16例青春期前停用类固醇且血清蛋白测量结果连续、肌酐水平正常的患儿中,平均个体白蛋白浓度与每年身高SDS的变化相关(r = 0.65,P = 0.0006),在男孩中与最终身高相关(r = 0.73,P = 0.03)。总之,类固醇抵抗性NS患儿的生长取决于肾功能的维持、所用类固醇的累积剂量以及低蛋白血症的严重程度。