Fakhouri Fadi, Bocquet Nathalie, Taupin Pierre, Presne Claire, Gagnadoux Marie-France, Landais Paul, Lesavre Philippe, Chauveau Dominique, Knebelmann Bertrand, Broyer Michel, Grünfeld Jean-Pierre, Niaudet Patrick
Department of Nephrology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Paris, France.
Am J Kidney Dis. 2003 Mar;41(3):550-7. doi: 10.1053/ajkd.2003.50116.
The clinical presentation, treatment, and outcome of steroid-sensitive nephrotic syndrome (SSNS) during childhood have been extensively studied. Conversely, few data regarding the outcome in adulthood of childhood SSNS have been published previously. We undertook to conduct a retrospective study of the outcome in adulthood of a large cohort of patients diagnosed with an SSNS during childhood.
We identified all children born between 1970 and 1975 who had been admitted to our institution for an SSNS. Data regarding the outcome in adulthood of these patients were obtained through mailed questionnaires or phone calls to patients and/or their parents or through attending physicians.
One hundred seventeen patients were identified. Data regarding the outcome of SSNS in adulthood were available for 102 patients (87.2%). Forty-three patients (42.2%) experienced at least one relapse of nephrotic syndrome in adulthood. By univariate analysis, young age at onset (<6 years) and more severe disease in childhood, indicated by a greater number of relapses (12.9 for adulthood relapsers versus 5.4 for adulthood nonrelapsers; P < 0.0001) and more frequent use of immunosuppressors (74.4% versus 31.6%; P < 0.0001) or cyclosporine (42.9% versus 7.3%; P < 0.0001) were predictive of the occurrence of SSNS relapse in adulthood. Conversely, relapse rate in the first 6 months of disease was not predictive of further relapses in adulthood. By multivariate analysis, only number of relapses during childhood was predictive of adulthood relapses (P < 0.0058). Long-term side effects of steroids were found in 44.2% of adulthood relapsers; the most frequent were osteoporosis and excess weight.
The incidence of childhood SSNS relapses in adulthood was relatively high in our study. Further studies are required to assess long-term complications in adults with relapses and a history of prolonged steroid and immunosuppressor use.
儿童期激素敏感性肾病综合征（SSNS）的临床表现、治疗及预后已得到广泛研究。相反，此前关于儿童期SSNS成年期预后的数据鲜有发表。我们对一大群儿童期诊断为SSNS的患者成年期预后进行了一项回顾性研究。
我们确定了1970年至1975年间因SSNS入住我院的所有儿童。通过向患者和/或其父母邮寄问卷或打电话，或通过主治医生获取这些患者成年期预后的数据。
共识别出117例患者。102例患者（87.2%）有成年期SSNS预后的数据。43例患者（42.2%）成年期经历了至少一次肾病综合征复发。单因素分析显示，发病年龄小（<6岁）以及儿童期病情更严重，表现为复发次数更多（成年期复发者为12.9次，成年期未复发者为5.4次；P<0.0001）、免疫抑制剂使用更频繁（74.4%对31.6%；P<0.0001）或环孢素使用更频繁（42.9%对7.3%；P<0.0001）可预测成年期SSNS复发情况。相反，疾病最初6个月内的复发率不能预测成年期的进一步复发。多因素分析显示，仅儿童期复发次数可预测成年期复发（P<0.0058）。44.2%的成年期复发者出现了类固醇的长期副作用；最常见的是骨质疏松和体重超标。
在我们的研究中，儿童期SSNS成年期复发率相对较高。需要进一步研究以评估成年期复发且有长期使用类固醇和免疫抑制剂病史患者的长期并发症。
