Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome

Abstract

Prader-Willi syndrome (PWS), an imprinting disorder, results from the loss of expression of a paternal gene on chromosome 15q11-q13. Progressive obesity and its associated complications lead to increased morbidity and early death in PWS patients. The management techniques available for morbid obesity in adolescents and adults with PWS are limited. Herein, we report successful weight reduction in an adolescent PWS case showing morbid obesity and respiratory failure. An 18-year-old girl with PWS presented with diffuse cellulitis and dyspnea due to severe obesity. Her body weight had increased from 146 to 161 kg despite dietary restriction to 800 kcal/day, and a mechanical ventilator was required for dyspnea. During mechanical ventilation, the patient was managed using diuretics and by restricting fluid intake; her daily calorie intake was reduced to 200 kcal. This aggressive calorie and water restriction continued for 3 weeks and reduced her body weight to 118.6 kg. After transfer to the general ward, the patient was provided with growth hormone therapy and intensive aquatic rehabilitation and was administered liraglutide; as a result, her weight further decreased to 104 kg (body mass index [BMI], 50.8 kg/m 2), and she was discharged. Following discharge, she maintained her BMI and adapted to 1,000 kcal/day for 1 year. Aggressive water and calorie restriction were observed as an effective method for rapid weight reduction in PWS patients, and liraglutide appeared useful in maintaining weight reduction in adolescent and adult PWS.

Hypothalamic neuropeptides and neurocircuitries in Prader Willi syndrome

“…Importantly, the authors did not observe BMI changes during the short term administration and suggested further studies with long‐term administration as follow up, as well a combination of exenatide with behavioural modifications 244 . Further, a more recent case report from Kim and colleagues demonstrated rapid and marked weight loss in a PWS adolescent (female, 18 years old) upon liraglutide (another GLP‐1R agonist) administration 245 . Remarkably, the effects of GLP‐1R activation modulate hypothalamic neuronal populations engaged in energy homeostasis 246 …”

“…244 Further, a more recent case report from Kim and colleagues demonstrated rapid and marked weight loss in a PWS adolescent (female, 18 years old) upon liraglutide (another GLP-1R agonist) administration. 245 Remarkably, the effects of GLP-1R activation modulate hypothalamic neuronal populations engaged in energy homeostasis. 246 Even though the therapeutic approach in PWS is still evolving, it is clear by now that hypothalamic dysfunction needs to be a key…”

Anti-Obesity Medication Use in Children and Adolescents with Prader–Willi Syndrome: Case Review and Literature Search

“…There were six results for PWS and liraglutide, with four case reports meeting inclusion criteria. These included: a 17-year-old with PWS on liraglutide and metformin who had weight loss, increased satiety, as well as lower HbA1c and fasting glucose; an 18-year-old with PWS and dyspnea requiring mechanical ventilation due to severe obesity who maintained weight reduction on liraglutide; a 13-year-old with PWS and diabetes, with a decrease in glucose and HbA1c on liraglutide and empagliflozin; and a 19-year-old with PWS and diabetes who had improved glycemic control, HbA1c, insulin resistance, and weight loss on liraglutide and empagliflozin [ 21 , 24 , 25 , 26 , 27 ]. The remaining cases were focused on populations over the age limit or in a different language.…”

Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity

“…Less is known about the efficacy of GLP1-RA on syndromic obesity. To the best of our knowledge liraglutide effects on morbid obesity were described in 7 PWS patients all affected also by T2DM (15)(16)(17)(18) and in a prospective case-control nonrandomized study on 14 patients affected by monogenic obesity related to melanocortin-4 receptor (MC4R) mutations (19).…”