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Cardiovascular risk factors improve during 3 years of growth hormone therapy in Prader-Willi syndrome
D l'Allemand1, U Eiholzer, M Schlumpf
1Foundation Growth Puberty Adolescence, Zurich, Switzerland.
Insights
Prader-Willi syndrome (PWS) children show early cardiovascular risks like obesity and abnormal lipids. Growth hormone therapy improves body composition and lipid profiles in these patients.
Area of Science:
- Pediatric Endocrinology
- Cardiovascular Health
- Metabolic Disorders
Background:
- Prader-Willi syndrome (PWS) is associated with cardiovascular risk factors, potentially linked to obesity or growth hormone (GH) deficiency.
- Understanding these risk factors' patterns and interrelations in children with PWS is crucial for early intervention.
Purpose of the Study:
- To investigate cardiovascular risk factors in children with PWS.
- To assess the age-dependency and interrelations of these factors.
- To evaluate the long-term effects of GH therapy on cardiovascular risk markers.
Main Methods:
- Observational study of 23 children with PWS (aged 0.3-14.6 years).
- Assessed body composition (fat mass, waist-to-hip ratio), lipid profiles (TG, LDL-C, HDL-C, Lp(a)), and apolipoproteins (Apo A-I, Apo B).
- Monitored effects of GH therapy (0.037 mg/kg/day for ~3 years).
Main Results:
- Children over 4 years exhibited increased body fat and abnormal waist-to-hip ratio.
- Abnormalities in LDL-C, Apo B, HDL-C, and TG were noted in several participants.
- GH therapy normalized body fat, waist-to-hip ratio, and improved LDL-C/HDL-C ratio by decreasing LDL-C and increasing HDL-C.
Conclusions:
- Cardiovascular risk factors are prevalent in prepubertal children with PWS.
- Growth hormone treatment positively impacts both body composition and lipid metabolism in PWS patients.
- GH therapy offers a therapeutic strategy for mitigating cardiovascular risks in PWS.
Unlabelled:
Cardiovascular risk factors in Prader-Willi syndrome (PWS, OMIM 176270) may be independently caused by overweight or hypothalamic growth hormone (GH) deficiency. The present observational study in 23 children with PWS, aged 0.3-14.6 years, focuses on the specific pattern, age-dependency and interrelation of cardiovascular risk factors, namely percentage fat mass and regional fat distribution, triglycerides (TG), lipoprotein cholesterols (LDL-C, HDL-C), lipoprotein (a) (Lp(a)), apolipoproteins A-I (Apo A-I) and B (Apo B), as well as on the longer-term effects of GH therapy (ca. 0.037 mg/kg per day for 3 years on average). We report that in children above 4 years, percentage body fat was increased in all and waist-to-hip-ratio (WHR) in 35%. Abnormal levels of LDL-C, Apo B, HDL-C and TG were found in 6, 7, 6 and 3 children, respectively. Lp(a) was above 300 mg/l in 5 patients and remained unchanged during GH therapy. However, percentage fat mass dropped to the upper normal range and WHR became normal in all patients receiving GH therapy, as did the ratio of LDL-C to HDL-C, subsequent to decreasing LDL-C and increasing HDL-C. Nevertheless, we could not find any significant correlation between parameters of total fat mass or fat distribution and serum lipid parameters, except for abdominal fat distribution (trunk-/leg-fat ratio) to TG before therapy.
Conclusion:
Several cardiovascular risk factors are already present in prepubertal children with Prader-Willi-syndrome and they are improved by growth hormone treatment, acting both on body composition and lipid metabolism.