Department of Pediatrics, Peking Union Medical College Hospital, Beijing 100730, China
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History+
Received
Revised
Published
2005-06-08
1900-01-01
2005-11-15
Issue Date
2005-11-15
Abstract
ObjectiveTo study renal involvement in hepatic glycogen storage disease(GSD) in childhood. MethodsOne hundred and eight patients aged less than 21 years old with typeⅠ a GSD (54 cases), typeⅢ (29 cases) and uncertain type hepatic GSD (25 cases). Urine analysis, urine albumin, urine protein of 24 h, urine β2-MG, BUN, creatinine, Ccr were evaluated. ResultsOf 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 1.0 g/24 h. Among 72 patients, urine β2-MG of 51 cases (70.8%)increased (175~10 623 mg/L), and the mean urine β2-MG of typeⅠa GSD was much higher than that of typeⅢ GSD, 4138.2 and 1790.1 mg/L respectively. Of 91 patients, 10 had renal insufficiency, 1/10 (15-year-old girl) had heavy proteinuria (3.5 g/24 h), elevated BUN (9.3 mmol/L) and Scr (1061 μmol/L). Five elder patients (11~21 years old) had hematuria with renal colic caused by renal calculus. ConclusionsPersistent protenuria, increased urine β2-MG, decreased Ccr, and renal stones are common complications of hepatic GSD in childhood. Renal function should be thoroughly evaluated during follow-up.
