A Japanese girl with mental retardation, severe microretrognathia, and brachydactyly: another case of the Gurrieri syndrome.
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Biomedical subjects
Publications and source records attributed to Takaya Nakane.
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OBJECTIVE: To determine whether serum adiponectin is decreased in obesity and is restored toward normal level after treatment in children. RESEARCH METHODS AND PROCEDURES: Subjects were 53 Japanese obese children, 33 boys and 20 girls (6 to 14 years old), and 30 age-matched nonobese controls for measuring adiponectin (16 boys and 14 girls). Blood was drawn after an overnight fast, and the obese children were subjected to anthropometric measurements including waist and hip circumferences and skinfold thicknesses. Paired samples were obtained from 21 obese children who underwent psychoeducational therapy. Visceral adipose tissue area was measured by computed tomography. Adiponectin was assayed by an enzyme-linked immunosorbent assay. RESULTS: The serum levels of alanine aminotransferase, uric acid, triglyceride, total cholesterol, low-density lipoprotein-cholesterol, total cholesterol/high-density lipoprotein-cholesterol, apo B, apo B/apo A(1), and insulin in obese children were higher than the reference values. Serum adiponectin level was lower in the obese children than in the controls (6.4 +/- 0.6 vs. 10.2 +/- 0.8 mg/L, means +/- SEM, p < 0.001). In 21 obese children whose percent overweight declined during therapy, the adiponectin level increased (p = 0.002). The adiponectin level was correlated inversely with visceral adipose tissue area in obese children (r = -0.531, p < 0.001). The inverse correlations of adiponectin with alanine aminotransferase, uric acid, and insulin were significant after being adjusted for percentage overweight, percentage body fat, or sex. DISCUSSION: Serum adiponectin level is decreased in obese children depending on the accumulation of visceral fat and is restored toward normal level by slimming.
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OBJECTIVE: To determine whether serum cholesteryl ester transfer protein (CETP), which is one of the physiologically active gene products secreted from adipose tissue, is increased and associated with atherogenic lipoprotein profile in obese children. RESEARCH METHODS AND PROCEDURES: Subjects were 42 consecutive outpatient Japanese obese children, 29 boys and 13 girls, ranging in age from 5 to 14 years, and 25 age-matched non-obese children, 13 boys and 12 girls, as the control group for measuring CETP mass. Blood was drawn after an overnight fast and, at the same time, and anthropometric measurements including height, body weight, waist girth, hip girth, and triceps and subscapular skinfold thicknesses were taken. Paired samples were obtained from 15 obese children who underwent psychoeducational therapy. Serum CETP mass was assayed by an enzyme-linked immunosorbent assay. RESULTS: The serum levels of triglyceride, total cholesterol (TC), low-density lipoprotein cholesterol, TC/high-density lipoprotein cholesterol (HDLC), apolipoproteins (apo) B, apo B/apo A(1), and insulin in obese children were significantly higher than the respective reference values. Serum CETP level was approximately 2-fold higher (98.7 +/- 3.6 vs. 50.9 +/- 4.0 nM, means +/- SEM, p < 0.001) in the obese children than in the controls. In 15 obese children, whose percentage of overweight declined during therapy, CETP levels decreased significantly. CETP level was correlated with HDLC, TC/HDLC, and insulin, and with percentage of overweight when the data of the obese and non-obese children were combined. DISCUSSION: CETP is increased and associated with the atherogenic lipoprotein profile in obese children.
A Japanese boy with a hearing deficit, cataracts, mental retardation, and brachycephaly without craniosynostosis is described. We believe that this patient represents a variant of the Fine-Lubinsky syndrome, and is the first report from a racial group other than Caucasian.