ABSTRACT
Objective To study renal involvement in hepatic glycogen storage disease(GSD) in childhood. Methods One hundred and eight patients aged less than 21 years old with type Ⅰa GSD (54 cases), type Ⅲ (29 cases) and uncertain type hepatic GSD (25 cases). Urine analysis, urine albumin, urine protein of 24 h, urine ?_2-MG, BUN, creatinine, Ccr were evaluated. Results Of 108 patients with hepatic GSD, 16 patients (20.8%) had proteinuria proven by urine albumin or urine protein of 24 h, their ages first found proteinuria were 8～15 years. Two 15-year-old patients had proteinuria over 1.0g/24h. Among 72 patients, urine ?_2-MG of 51 cases (70.8%) increased (175～10 623mg/L), and the mean urine ?_2-MG of type Ⅰ a GSD was much higher than that of type Ⅲ GSD, 4138.2 and 1790.1mg/L respectively. Of 91 patients, 10 had renal insufficiency, 1/10 (15-year-old girl) had heavy proteinuria (3.5g/24h), elevated BUN (9.3mmol/L) and Scr(1061 ?mol/L). Five elder patients (11～21 years old) had hematuria with renal colic caused by renal calculus. Conclusions Persistent protenuria, increased urine ?_2-MG, decreased Ccr, and renal stones are common complications of hepatic GSD in childhood. Renal function should be thoroughly evaluated during follow-up.