Titled “Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome,” the case report was published in the Annals of Pediatric Endocrinology & Metabolism.
People with PWS lack effective treatments for their excessive appetite and progressive obesity. Morbid obesity can lead to cardiovascular complications, chronic swelling in the legs, and type 2 diabetes.
Researchers in Korea detailed the case of a woman diagnosed with PWS at age 14, following rapid weight gain and intellectual disability in childhood. She had also been diagnosed with type 2 diabetes and hypertension (high blood pressure), and was receiving medications for both disorders.
At 18, the patient went to the hospital due to shortness of breath and cellulitis (a type of skin infection) on the abdomen and limbs. She was unable to walk or sit unassisted due to pain in the joints. In the two weeks prior, the patient had rapidly gained 20 kg (about 44 lbs).
When she was admitted to the hospital, the patient was 143 cm (4 feet 8 inches) tall and weighed 145 kg (320 lbs), equating to a body mass index of 71 kg/m2.
Antibiotics were given to treat cellulitis, and the patient was placed on a restricted diet (800 kcal/day). However, after a week, her body weight had increased to 164 kg (362 lbs), and her breathing problems had worsened.
She was put on a mechanical ventilator, and her diet was further restricted to 200 kcal/day. Fluid restriction (including 5% dextrose with 600 mL of electrolytes and 400 mL of water) and furosemide (a diuretic) were used to induce massive weight reduction.
“Fluid accumulation may play a role in rapid weight gain and dyspnea in morbid obesity,” the scientists wrote. “Therefore, aggressive diuretic use and massive fluid restriction may be effective at reducing body weight for a short time, despite concerns of electrolyte imbalance and blood pressure drops during treatment.”
In accordance with these concerns, the patient’s electrolyte levels were carefully monitored.
After 20 days, she was taken off the ventilator. While on the ventilator, the patient had been sedated, which made it easier to administer the heavily restricted diet. However, once sedatives were no longer administered, dietary control became a challenge.
She was then given Victoza (liraglutide, by Novo Nordisk), a GLP-1 receptor agonist previously associated with appetite reduction and lower blood glucose levels. Victoza was started at a dose of 0.6 mg/day, later increased to 1.2 mg/day without side effects.
In addition, the patient received physical therapy for an hour a day, five days a week.
Three months after being admitted to the hospital, the patient was discharged. Her weight had decreased to 104 kg (229 lbs), her BMI had decreased to 50.8 kg/m2, and she was able to walk unassisted. After a year, her BMI was maintained at 48.5 kg/m2, and the patient was continuing daily exercise and was dealing well with a diet of 1,000 kcal/day. Also, her total weight loss had reached 62.7 kg (138 lbs).
“In this study, we successfully treated a severely obese, adolescent PWS patient with multidisciplinary management and GLP-1 receptor agonist use,” the team wrote.
“GLP-1 receptor agonists aid in appetite and weight reduction and could be an effective treatment for PWS,” the scientists added, although “large cohort, controlled, and longterm trials in patients with PWS will be required to confirm the efficacy and safety of GLP-1 receptor agonists.”
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