Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, 060-8638, Japan.
Department of Pediatrics, Health Sciences University of Hokkaido Hospital, Sapporo, Hokkaido, 002-8072, Japan.
Pediatr Nephrol. 2021 Aug;36(8):2319-2325. doi: 10.1007/s00467-021-04956-6. Epub 2021 Feb 3.
Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, especially in children. Owing to the short-term observational period and the small number of patients analyzed in previous reports, the long-term clinical and laboratory characteristics and renal prognosis of children with TINU syndrome remain unclear.
In this retrospective observational study, we enrolled 29 children with TINU syndrome from February 1990 to February 2019.
During the median follow-up duration of 38 months, the kidney function, urinary β2 microglobulin-creatinine ratio (U-β2MG/Cr), and uveitis in the patients had significantly improved at 24, 6, and 36 months after diagnosis. Higher U-β2MG/Cr was associated with longer duration of kidney function normalization. Half of the patients required uveitis treatment for 5 years after the diagnosis.
Patients with severe low-molecular weight proteinuria at diagnosis needed a longer duration to achieve improvements in kidney function. Uveitis has a much longer treatment period than tubulointerstitial nephritis. This study demonstrates the good prognosis of children with TINU syndrome in terms of their long-term clinical and laboratory characteristics.
肾小管间质性肾炎和葡萄膜炎（TINU）综合征是一种罕见的疾病，特别是在儿童中。由于之前的报告观察期较短且分析的患者数量较少，因此儿童 TINU 综合征的长期临床和实验室特征及肾脏预后仍不清楚。
在这项回顾性观察研究中，我们纳入了 1990 年 2 月至 2019 年 2 月期间的 29 例 TINU 综合征患儿。
在中位数为 38 个月的随访期间，与诊断后 24、6 和 36 个月相比，患者的肾功能、尿β2 微球蛋白/肌酐比值（U-β2MG/Cr）和葡萄膜炎均有显著改善。更高的 U-β2MG/Cr 与肾功能正常化的时间更长相关。半数患者在诊断后需要 5 年的葡萄膜炎治疗。
诊断时有严重低分子量蛋白尿的患者需要更长的时间才能改善肾功能。葡萄膜炎的治疗时间比肾小管间质性肾炎长得多。本研究表明，儿童 TINU 综合征在长期临床和实验室特征方面具有良好的预后。
