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Long-term clinical characteristics and renal prognosis of children with tubulointerstitial nephritis and uveitis syndrome

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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.

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Acknowledgements

We are grateful for the work of the past and present members of our laboratory.

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Correspondence to Takayuki Okamoto.

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All procedures performed in studies involving human participants were reviewed and approved by the Institutional Review Board of Hokkaido University Hospital and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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The authors declare no competing interests.

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Supplementary information

Supplementary Table 1

Baseline clinical and laboratory characteristics of the 29 enrolled children with TINU syndrome. Diagnostic criteria of D; Definite and P; probable. Gender F; Female, Gender M; Male. ESR; erythrocyte sedimentation rate (normal male range, 2-10 mm/h; normal female range 3-15 mm/h), Hb; blood hemoglobin, KL-6; sialylated carbohydrate antigen KL-6 (normal range < 500 U/mL), Pyuria (in urinary sediment 5 white blood cells (WBCs), > 400 times field of vision), PRO; proteinuria (> 1+ by dipstick method),GLU; glucosuria (> 1 ± by dipstick). N/A; not available. (PPTX 55.5 kb).

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Hayashi, A., Takahashi, T., Ueda, Y. et al. Long-term clinical characteristics and renal prognosis of children with tubulointerstitial nephritis and uveitis syndrome. Pediatr Nephrol 36, 2319–2325 (2021). https://doi.org/10.1007/s00467-021-04956-6

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