Characterization of alterations in carbohydrate metabolism in children with Prader-Willi syndrome.
OBJECTIVE
To study carbohydrate metabolism and insulin sensitivity and secretion in children and adolescents with Prader-Willi syndrome (PWS) compared with multifactorial obesity (MO) controls.


METHODS
Seventy-five patients with PWS and 395 controls with MO were studied by oral glucose tolerance test. Insulin resistance (IR) and beta-cell function were assessed by homeostasis model assessment (HOMA), insulin glucose index, fasting insulin and insulin sensitivity index.


RESULTS
The incidence of diabetes mellitus was 0% in PWS and 1.5% in MO, while carbohydrate intolerance was 9.3% in the former group and 7.6% in the latter (NS); basal insulin level (12 +/- 8.2 vs 22.3 +/- 25 mU/ml) and HOMA-IR (2.47 +/- 1.6 vs 4.18 +/- 5.05) were lower in PWS (p = 0.004 and 0.04, respectively), whereas HOMA beta-cell index was lower in PWS than in MO (59 +/- 42 vs 102 +/- 119, p = 0.03). ISI Composite was higher in PWS compared to MO (6 +/- 5.7 vs 4.18 +/- 5.05, p = 0.04).


CONCLUSIONS
Patients with PWS presented lower insulin resistance and a dissociation between beta-cell secretion and the degree of obesity.
Krochik
Andrea Gabriela
AG
Nutrition Department, Hospital de Pediatría J P Garrahan, Buenos Aires, Argentina.
Ozuna
Blanca
B
Torrado
María
M
Chertkoff
Lilien
L
Mazza
Carmen
C
D000293:Adolescent; D000328:Adult; D050260:Carbohydrate Metabolism; D002648:Child; D002675:Child, Preschool; D003924:Diabetes Mellitus, Type 2 / Q000209:etiology; D003940:Diagnostic Techniques, Endocrine; D005260:Female; D005947:Glucose / Q000378:metabolism*; D005951:Glucose Tolerance Test; D006801:Humans; D015994:Incidence; D007328:Insulin / Q000378:metabolism*; D007333:Insulin Resistance; D008297:Male; D009765:Obesity / Q000235:genetics / Q000503:physiopathology; D011218:Prader-Willi Syndrome / Q000378:metabolism* / Q000473:pathology
D007328:Insulin; D005947:Glucose