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Non-Alcoholic Fatty Liver Disease (NAFLD) in children and adolescents with Prader-Willi Syndrome (PWS)

D Fintini1, E Inzaghi2,3, M Colajacomo4

  • 1Autoimmune Endocrine Diseases Unit, Bambino Gesù Children's Hospital, Research Institute, Palidoro, Rome, Italy.

Pediatric Obesity
|July 2, 2015
PubMed

Insights

Patients with Prader-Willi syndrome (PWS) show a reduced risk of non-alcoholic fatty liver disease (NAFLD). This may be linked to improved insulin sensitivity and a better metabolic profile in PWS children.

Area of Science:

  • Pediatric Endocrinology
  • Metabolic Disorders
  • Hepatology

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder.
  • Non-alcoholic fatty liver disease (NAFLD) is a growing health concern, particularly in children.
  • Insulin resistance is a key factor in NAFLD development.

Purpose of the Study:

  • To investigate the hypothesis that PWS patients have a lower risk of NAFLD due to enhanced insulin sensitivity.
  • To compare the metabolic profiles and NAFLD prevalence between PWS patients and a control group.

Main Methods:

  • A cohort study involving 21 PWS patients and 42 age-, sex-, and BMI-matched controls.
  • Assessment of metabolic profiles, including glucose, lipids, and insulin resistance (HOMA-IR).
  • Evaluation of NAFLD using validated ultrasound grading (G0-G3) and body composition analysis.

Main Results:

  • PWS patients exhibited significantly better metabolic profiles: lower waist circumference, fasting glucose, HOMA-IR, cholesterol, transaminases, and trunk fat.
  • NAFLD G1 stage was more frequent in PWS subjects (P<0.05), while G2 stage was more frequent in controls (P<0.05).
  • NAFLD grading correlated with body composition in PWS, independent of sex and GH treatment.

Conclusions:

  • This study suggests a reduced risk of NAFLD in children with Prader-Willi syndrome.
  • Improved insulin sensitivity and metabolic health may contribute to this protective effect in PWS.
  • Further research is warranted to elucidate the mechanisms underlying NAFLD risk in PWS.

Keywords:
NAFLDPrader-Williobesitypaediatric

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