44 Nutritional approach to weight management of morbid obesity and type 2 diabetes mellitus in Prader-Willi syndrome

BackgroundPrader-Willi syndrome (PWS) is a genetic disorder that occurs in approximately one out of every 15,000 births. PWS affects males and females with equal frequency and affects all races and ethnicities. PWS is recognized as the most common genetic cause of life-threatening childhood obesity. The common symptoms included small hands and feet, abnormal growth and body composition (small stature, very low lean body mass, and early-onset childhood obesity), hypotonia (weak muscles) at birth, insatiable hunger, extreme obesity, and intellectual disability.Symptoms of PSW like poor feeding and lack of appetite in infancy reverse to an uncontrolled appetite and lack of satiety that lead to weight gain, mostly after the age of 2–3 years. They are frequently unable to sense when they are full after eating. This reverse in appetite and weight status is also noted in other conditions such as Trisomy 21.In the absence of rigorous supervision of food intake, many PWS patients weigh more than twice their ideal body weight. Most PWS research has been targeted towards treating specific symptoms such as insatiable hunger and extreme obesity.Improvement in weight loss continues to be the most important goal of any treatment of PWS patients but the management of obesity and hyperphagia which includes excessive eating behaviors in these patients are not easy. Previous pharmacotherapy options aimed at achieving weight loss in PWS by targeting appetite suppression has also, been shown to be extremely difficult.The 2013 Obesity Guidelines recommends a moderate weight loss of 5–10% as the goal for medically supervised weight loss. Furthermore, a 5% weight loss from baseline is considered a clinically significant amount within a period of one month. A multidisciplinary clinical management approach during childhood can help to change the history of PWS and achieve favorable outcomes including significant weight loss in the first 6 months of its initiation.ObjectiveTo objective of this case report was to focus on nutritional outcomes as part of a multidisciplinary clinical management approach in a child with severe morbid obesity and Type 2 diabetes mellitus (T2D) with PWS.MethodA 9.5-year-old girl with PWS was referred to our hospital for the management of severe obesity with multiple co-morbidities. She had been to multiple centers around the world for the same. She was the 3rd child of non-consanguineous parents who was diagnosed with at the age of 2 years. The onset of p