METABOLIC CONTROL AND RENAL DYSFUNCTION IN TYPE-I GLYCOGEN-STORAGE-DISEASE

This study was undertaken to determine the effect on renal function of continuous glucose therapy from early childhood. Twenty-three subject s, median age 13.9 years, range 5.9-26.9 years, with type I glycol:en storage disease (GSDI) treated with continuous glucose therapy from a median age of 1.3 years, range 0.1-12.9 years, had 24h monitoring of m etabolites and glucoregulatory hormones on their home feeding regimen to assess metabolic control at approximately yearly intervals for a me dian duration of 8 years. During the most recent evaluation, 24h urina ry albumin excretion rate (AER), kidney size, and creatinine clearance (Ccr) were measured. CCr was unrelated to age and was increased (>2.3 3 ml/s per 1.73 m(2)) in 10/23 (43%). Mean kidney length exceeded 2SD in 16/23 (70%). AER was normal in all five subjects <10 years and was increased (>10 mu g/min) in 8/23 (35%), all >10 years of age. AER was significantly greater in subject of similar age who started continuous glucose therapy later in childhood and was significantly higher in su bjects with lower mean 24h plasma glucose concentrations and higher me an 24h blood lactate concentrations, both at the time of assessment of renal function and over the preceding 5 years. GSDI subjects with per sistently elevated concentrations of blood lactate, serum lipids and u ric acid are at increased risk of nephropathy. Optimal dietary therapy instituted early in life may delay, prevent, or slow the progression of renal disease.