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OBJECTIVE: To examine the clinical features of juvenile gout and its possible association with familial juvenile hyperuricemic nephropathy (FJHN). METHODS: A total of 543 cases of juvenile gout from the Ho-Ping Gout Database were enrolled, and 5269 gouty cases with onset age of 40 to 50 years were selected as a control group. Clinical and laboratory data were compared between the 2 groups. RESULTS: In patients with juvenile gout, body mass index, serum urate concentration, 24-hour urinary uric acid excretion, and creatinine clearance were significantly higher than those in the control group (p < 0.0001), while fractional excretion of uric acid was significantly lower. Only 15% of the juvenile gout cases fulfilled the features of FJHN. The percentage of familial aggregation in juvenile gout was about 1.9-fold higher than that in the control group (44.3% vs 23.8%; p < 0.0001). CONCLUSION: Juvenile gout in Taiwan is associated with overweight and hereditary background, while FJHN may not be primarily responsible.