Background: PraderWilli syndrome (PWS) has a relative hypoinsulinemia and a lower insulin resistance than non-PWS subjects. Mostly on the basis of such higher insulin sensitivity, PWS subjects have been hypothesized to be at lower risk of non-alcoholic fatty liver disease (NAFLD).
Objective and hypotheses: In this cross-sectional study, we analyzed the presence and the severity of NAFLD in a group of PWS children compared to matched pairs.
Method: Twenty-two PWS patients (eight males, mean age 12.4±2.4 years, Tanner 2.2±1.3; BMISDS 3.7±2.2) were compared to a control group (CNT) of a one to one age (12.0±2.5 years), sex (eight males), pubertal status (Tanner 2.4±1.7), and BMISDS (3.1±1.5) matched pairs. All patients underwent standard oral glucose tolerance test, liver ultrasonography (US) and body composition by DEXA scan (free fat mass (FFM); total fat mass (FM); trunk fat mass (TFM) as percentage of body weight). NAFLD was established by a validated method of US grading (ranged G0=no NAFLD-G3=moderate NAFLD)
Results: A not significant lower HOMA-IR and insulin were found in PWS compared to CNT (3.1±2.0 vs 6.4±8.4 and 17.4±9.6 vs 32.1±44.8 respectively). No difference of insulin sensitivity index and body composition were detected between the two groups. The grading of NAFLD showed a higher percentage of PWS at G0 (28.3 vs 6.3% of CNT; P>0.05) and lower at G1 and G2 (47.6 vs 56.3 and 19.1 vs 31.3% respectively; P<0.05). Univariate analysis showed a positive correlation in PWS between NAFLD grading and BMISDS (r=0.644), FM (r=0. 667), and negative with FFM (r=−0.673), FFM/FM (r=−0.655) while in CNT no correlation were found. When inserted in a regression model, adjusted also for GH therapy we did not find any correlation.
Conclusion: Our data seems to support the hypothesis that PWS children were protected by NAFLD compared to CNT. BMI and metabolic parameters, and GH therapy, did not seem to have influence on NAFLD developing.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology