Asako Hayashi1,2, Toshiyuki Takahashi1, Yasuhiro Ueda1, Yasuyuki Sato1, Takayuki Okamoto3. 1. Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, 060-8638, Japan. 2. Department of Pediatrics, Health Sciences University of Hokkaido Hospital, Sapporo, Hokkaido, 002-8072, Japan. 3. Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Hokkaido, 060-8638, Japan. okamon@med.hokudai.ac.jp.
Abstract
BACKGROUND: 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. METHODS: In this retrospective observational study, we enrolled 29 children with TINU syndrome from February 1990 to February 2019. RESULTS: 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. CONCLUSIONS: 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.
BACKGROUND: 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. METHODS: In this retrospective observational study, we enrolled 29 children with TINU syndrome from February 1990 to February 2019. RESULTS: 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. CONCLUSIONS: 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.
Authors: T Takemura; M Okada; S Hino; K Fukushima; S Yamamoto; H Miyazato; K Maruyama; K Yoshioka Journal: Am J Kidney Dis Date: 1999-12 Impact factor: 8.860
Authors: Ville Saarela; Matti Nuutinen; Marja Ala-Houhala; Pekka Arikoski; Kai Rönnholm; Timo Jahnukainen Journal: Ophthalmology Date: 2013-03-16 Impact factor: 12.079