Bariatric surgery would be a hope for a lasting treatment for this rare genetic disease.
It is the Tantalus torture of medicine: the sick suffer from a permanent appetite. Prader-Willi syndrome has no effective treatment to date. According to a study carried out in Saudi Arabia, obesity surgery would be a viable solution to the eating disorders afflicting these young patients. Its authors present their results in the journal Surgery for Obesity and Related Diseases.
No complication
24 children and adolescents (5-18 years) with Prader-Willi syndrome (see box) were followed 5 years after having undergone bariatric surgery. To this group were added 72 control subjects to assess the effectiveness of the intervention. The surgeons performed a sleeve gastrectomy, which removes a large part of the stomach. This technique has the double advantage of reducing the absorption capacity of food, while reducing the production of an appetite hormone.
Indeed, obesity is one of the main causes of complications and death in patients with this genetic disease.
At the start of the study, the young patients had an average BMI of 46.2, which puts them in morbid obesity. In fact, all the patients suffered from diseases associated with their overweight: sleep apnea (100%), excess cholesterol (62%), hypertension (43%), type 2 diabetes (29%).
But at the end of the study, all the patients experienced significant weight loss without major complications. “Most of the weight loss occurs in the first two years after surgery, and patients reduced their food intake and felt satisfied with smaller amounts of food, thanks to the reduced capacity of the stomach.” , welcomes Dr. Aayed Alqahtani, co-author of the study.
The question of recommendations
Despite these good results, should we open bariatric surgery to very young patients? The debate is still raging in the medical community. This study seems to argue in favor of a widening of the indications, in this very particular case. “Our study indicates that bariatric surgery should be recommended for young patients with Prader-Willi syndrome,” says Dr. Alqahtani. Our results are not equivalent to any other treatment. ”
But this researcher recognizes that questions arise both about the safety of this type of intervention and its long-term effects, especially on growth.
Further studies are needed to make recommendations, slice Dr. Thomas Inge, professor of surgery and pediatrics at the Cincinnati Children’s Hospital Medical Center, Ohio. “It is clearly not possible to issue treatment recommendations on the use of surgery in this complex population without in-depth research to assess its physiological impact,” he said.
>> Find our survey devoted to bariatric surgery: 500,000 operated within 5 years
A multifaceted syndrome
Prader-Willi syndrome is a disease of genetic origin, which affects one in 20,000 to 25,000 people. If the disease remains active throughout life, the symptoms themselves change over time: lack of muscle tone (hypotonia) and difficulty eating. But with growth, the situation turns around completely: disturbances in the regulation of several functions occur – such as growth, sleep, emotions. The most well-known manifestation of this is excessive appetite: patients suffer from a constant feeling of hunger, which often leads to morbid obesity and associated comorbidities.
Symptoms that severely handicap, but little treatment. To treat hypotonia, physical therapy is recommended. In order to compensate for excess appetite, patients must undergo a strict diet. An approach that sometimes lacks effectiveness.
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