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Body composition during GH treatment in Prader-Labhardt-Willi syndrome
1Paediatric Department, Scientific Institute H San Raffaele, University of Milan, Italy.
Journal of Pediatric Endocrinology & Metabolism : JPEM
|March 4, 2000
Summary
Prader-Labhardt-Willi syndrome (PLWS) patients exhibit distinct body composition, with higher fat mass and lower lean mass. Growth hormone (GH) therapy shows promise in improving body composition and potentially stature in these individuals.
Area of Science:
- Endocrinology
- Genetics
- Pediatrics
Background:
- Prader-Labhardt-Willi syndrome (PLWS) presents unique challenges in growth hormone (GH) secretion and body composition.
- Understanding the specific body composition abnormalities in PLWS is crucial for effective therapeutic strategies.
Purpose of the Study:
- To investigate the body composition of Prader-Labhardt-Willi syndrome patients.
- To evaluate the effects of growth hormone (GH) therapy on body composition in PLWS patients.
Main Methods:
- Dual-energy X-ray absorptiometry (DXA) was used to assess body composition in 27 PLWS patients compared to age- and sex-matched controls.
- Abdominal CT scans were performed in adult PLWS patients undergoing GH therapy.
- Growth hormone (GH) therapy was administered to a cohort of children and adults with PLWS.
Main Results:
- PLWS patients displayed significantly greater fat mass and lower lean body mass (LM) and bone mineral content (BMC) compared to both obese and normal-weight controls.
- GH therapy in children led to increased LM and decreased fat mass (FM) within 12 months.
- Adults with PLWS showed reduced adiposity and increased leg LM after GH treatment, but no significant reduction in intra-abdominal fat.
Conclusions:
- The body composition in PLWS resembles that seen in GH deficiency.
- GH therapy may positively influence body composition and potentially improve final stature in Prader-Labhardt-Willi syndrome patients.