Initial Steroid Sensitivity in Children with Steroid-Resistant Nephrotic Syndrome Predicts Post-Transplant Recurrence

Initial Steroid Sensitivity in Children with Steroid-Resistant Nephrotic Syndrome Predicts Post-Transplant Recurrence
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DOI:
10.1681/asn.2013080852
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发表时间:
2014-06-01
影响因子:
13.6
通讯作者:
Saleem, Moin A.
Saleem, Moin A.
中科院分区:
医学1区
文献类型:
--
作者:
Ding, Wen Y.;Koziell, Ania;Saleem, Moin A.

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在患有特发性肾病综合征的儿童中,10%-20%的儿童对类固醇没有反应或出现继发性类固醇抵抗(称为初始类固醇敏感性),大多数进展到移植。虽然这些患者中有30%-50%的患者在移植后出现疾病复发,但长期效果不佳,目前还没有确定可靠的复发指标。值得注意的是,由于非原发疾病导致的激素抵抗型肾病综合征(SRNS)患者移植后复发的发生率似乎降低了。我们回顾了150名患有SRNS的移植患者,以确定一致预测移植后SRNS结果的生物标志物。总共有25名儿童有遗传性或家族性SRN,移植后没有复发。我们回顾了其余125名儿童的表型因素,包括最初的类固醇敏感性、供体类型、年龄、种族、接受ESRD的时间和透析时间。在这些患者中,57例(45.6%)发生移植后复发;28例最初对类固醇敏感的患者中有26例(92.9%)在移植后复发,而86例从一开始就耐药的患者中只有26例(30.2%)复发(优势比,30;95%可信区间,6.62%至135.86;P
Of children with idiopathic nephrotic syndrome, 10%-20% fail to respond to steroids or develop secondary steroid resistance (termed initial steroid sensitivity) and the majority progress to transplantation. Although 30%-50% of these patients suffer disease recurrence after transplantation, with poor long-term outcome, no reliable indicator of recurrence has yet been identified. Notably, the incidence of recurrence after transplantation appears reduced in patients with steroid-resistant nephrotic syndrome (SRNS) due to nnonogenic disorders. We reviewed 150 transplanted patients with SRNS to identify biomarkers that consistently predict outcome of SRNS after transplantation. In all, 25 children had genetic or familial SRNS and did not experience post-transplant recurrence. We reviewed phenotypic factors, including initial steroid sensitivity, donor type, age, ethnicity, time to ESRD, and time on dialysis, in the remaining 125 children. Of these patients, 57 (45.6%) developed post-transplant recurrence; 26 of 28 (92.9%) patients with initial steroid sensitivity recurred after transplantation, whereas only 26 of 86 (30.2%) patients resistant from the outset recurred (odds ratio, 30; 95% confidence interval, 6.62 to 135.86; P