METHODS: Twenty-seven children known to have GSD were included in this study. Fifteen healthy age-and gender-matched children were also included as controls. Routine urine analysis and measurement of urinary  2 -microglobulin and microalbumin levels were performed for all patients and controls. Renal-function tests, measurement of serum electrolyte, alkaline phosphatase, urinary calcium, blood, and urine pH levels, creation of a urinary and plasma aminogram, calculation of the glomerular filtration rate, bone radiography to detect rachitic manifestations, and abdominal ultrasound to measure renal size were performed for all patients. RESULTS: Twenty-one patients had Ն1 renal abnormality. The most common was increased urinary  2 -microglobulin level (15 of 21) followed by an abnormal glomerular filtration rate, whether low or high (8 of 21), and microalbuminuria (6 of 21). Sonographically, there was nephrocalcinosis in 1 case and renal stone in another. The area under the receiver operating characteristic curve for  2 -microglobulin was 0.86 (P ϭ .01) and 0.7 for the urinary microalbumin/creatinine ratio (P ϭ .15). The best cutoff level for predicting renal abnormality for urinary  2 -microglobulin was 0.22 mg/L with 70% sensitivity and 100% specificity, and the best cutoff value for the urinary microalbumin/creatinine ratio was 4.5 with 86% sensitivity and 50% specificity. CONCLUSIONS: Renal abnormalities are common in patients with GSD. Urinary B 2 -microglobulin level can be considered the gold standard for early detection of renal dysfunction in these patients. LEPTIN AND LEPTIN RECEPTOR IN SERUM INTRODUCTION:Hyperlipidemia may cause glomerulosclerosis in children with nephrotic syndrome (NS). OBJECTIVE: Our goal was to observe the role of soluble leptin receptor (sOBR) and leptin in serum and urine on the mechanism of hyperlipidemia in children with NS. METHODS: Twenty-three children with untreated NS and 15 age-, gender-, and BMI-matched healthy controls were enrolled onto the study. Leptin and sOBR in serum and urine were measured by enzyme-linked immunosorbent assay, and plasma lipid and insulin levels were detected by automatic biochemistry analyzer and radioimmunoassay, respectively. sOBR messenger RNA and membrane protein expression in peripheral blood mononuclear cells were detected by reverse-transcription polymerase chain reaction and immunocytochemistry. RESULTS: Low-density lipoprotein, total cholesterol, triglyceride, and apolipoprotein A levels were increased. sOBR messenger RNA and membrane protein expression by peripheral blood mononuclear cells were significantly lower in the patient group compared with controls. The ratio of serum leptin versus sOBR (free leptin index) was significantly higher in the NS group. Urinary leptin in the patient group was higher than that in the control group. The free leptin index showed no correlation with BMI or total cholesterol, triglyceride, or apolipoprotein B levels in both groups but did show a correlation with plasma albumin, low-density lipoprotein...
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