Obesity management in Prader-Willi syndrome: current perspectives

Area of Science:

• Genetics
• Endocrinology
• Pediatrics

Background:

• Prader-Willi syndrome (PWS) is a complex genetic disorder caused by absent paternal gene expression on chromosome 15.
• It is the most common genetic cause of obesity, affecting 1:10,000-1:30,000 live births.
• PWS is characterized by neonatal hypotonia, poor feeding, followed by hyperphagia and severe obesity after age 2-3.

Purpose of the Study:

• To review the current understanding of Prader-Willi syndrome (PWS), focusing on its genetic basis, clinical manifestations, and obesity management.
• To highlight the challenges in controlling hyperphagia and obesity in PWS patients.
• To discuss current and emerging therapeutic strategies for obesity in PWS.

Main Methods:

• Literature review of Prader-Willi syndrome (PWS) focusing on genetics, clinical features, and obesity management.
• Analysis of the effectiveness of current interventions including dietary restriction, physical activity, and behavioral management.
• Evaluation of ongoing clinical trials for new obesity therapies and the role of bariatric surgery.

Main Results:

• PWS management requires strict food intake control from an early age to prevent severe obesity and associated comorbidities.
• Current therapeutic tools often fall short in achieving successful weight loss and maintenance.
• Emerging drug therapies show promising preliminary results, and bariatric surgery is considered for severe cases.

Conclusions:

• Obesity prevention and management in Prader-Willi syndrome (PWS) are critical due to life-threatening complications.
• While dietary, physical activity, and behavioral interventions are fundamental, they are often insufficient.
• New pharmacological treatments and bariatric surgery represent potential advancements in managing PWS-related obesity.