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Endocrine and Metabolic Illnesses in Young Adults with Prader-Willi Syndrome
Eu-Seon Noh1, Min-Sun Kim1, Chiwoo Kim1
1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Korea.
Insights
Young adults with Prader-Willi syndrome (PWS) face higher risks of metabolic and endocrine conditions like type 2 diabetes and obesity. Early screening and intervention are crucial for better health outcomes in PWS patients.
Area of Science:
- Endocrinology
- Genetics
- Metabolic Disorders
Background:
- Prader-Willi syndrome (PWS) is a rare genetic disorder causing insatiable appetite and obesity.
- Previous research on PWS health issues focused on older adults, leaving a gap in understanding younger adult complications.
- Endocrine and metabolic illnesses in young adults with PWS remain understudied.
Purpose of the Study:
- To investigate the prevalence of endocrine and metabolic illnesses in young adults with PWS.
- To compare these health issues with a matched healthy control group.
- To assess the impact of recombinant human growth hormone (rhGH) treatment on obesity in adult PWS patients.
Main Methods:
- Retrospective cohort study of 68 individuals with PWS (aged 19-34 years).
- Comparison with an age-, sex-, and BMI-matched healthy control group.
- Analysis of endocrine and metabolic conditions, including metabolic syndrome, type 2 diabetes, hypertension, dyslipidemia, bone density, and sleep apnea.
Main Results:
- Young adults with PWS showed significantly higher rates of metabolic syndrome (35.3% vs. 4.4%), type 2 diabetes (50.0% vs. 5.4%), hypertension (30.8% vs. 16.1%), dyslipidemia (38.2% vs. 14.7%), decreased bone density (26.4% vs. 0.9%), and sleep apnea (32.3% vs. 4.4%).
- Adult PWS patients on rhGH treatment had a lower likelihood of a BMI ≥ 30 (OR = 0.106).
- These conditions may manifest in early adolescence.
Conclusions:
- Young adults with PWS have a high burden of endocrine and metabolic comorbidities.
- Continuous rhGH treatment may help manage obesity in adulthood.
- Early screening and intervention are essential for managing PWS complications and improving long-term health.
Abstract:
Prader−Willi syndrome (PWS) is a rare genetic disorder characterized by an insatiable appetite that leads to morbid obesity. Previous studies reported health problems in adults with PWS. However, studies on younger adults are lacking, and there are no specific studies of endocrine and metabolic illness in this age group. We performed a retrospective cohort study of 68 individuals with PWS aged 19 to 34 years at Samsung Medical Center. The prevalence of endocrine and metabolic illnesses were compared with those in an age-, sex-, and BMI-matched healthy control group. Young adults with PWS had a higher prevalence of metabolic syndrome (35.3% vs. 4.4%), type 2 diabetes mellitus (50.0% vs. 5.4%), hypertension (30.8% vs. 16.1%), dyslipidemia (38.2% vs. 14.7%), decreased bone density (26.4% vs. 0.9%), and sleep apnea (32.3% vs. 4.4%) than controls (all p < 0.05). The PWS group that maintained recombinant human growth (rhGH) treatment in adulthood had a lower probability of having a BMI ≥ 30 at the last follow-up (odds ratio = 0.106 (0.012−0.948), p = 0.045). Endocrine and metabolic illnesses in individuals with PWS may have already started in the early teens; therefore, appropriate screening and early intervention are important. Better understanding of the natural history of PWS and age-related complications will lead to better-quality medical care for individuals with PWS.
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