Ann Pediatr Endocrinol Metab.  2020 Mar;25(1):52-56. 10.6065/apem.2020.25.1.52.

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

• Kim YM
• Lee YJ
• Kim SY
• Cheon CK
• Lim HH

• KMID: 2501038
• DOI: http://doi.org/10.6065/apem.2020.25.1.52

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/m²), 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.

Keyword

Liraglutide; Prader-Willi syndrome; Adolescent; Obesity; Morbid

Figure

• 

  Fig. 1. Clinical photographs of the patient at her first visit (A, B) and 1 year after discharge (C, D).

• 

  Fig. 2. The short-term and long-term effects on body mass index (BMI) reduction due to food restriction, intensive aquatic rehabilitation, and medication in a patient with Prader-Willi syndrome. ICU, intensive care unit; HbA1c, glycosylated hemoglobin.

Reference

References

1. Cassidy SB, Schwartz S, Miller JL, Driscoll DJ. Prader-Willi syndrome. Genet Med. 2012; 14:10–26.

2. Goldstone AP, Holland AJ, Hauffa BP, Hokken-Koelega AC, Tauber M; speakers contributors at the Second Expert Meeting of the Comprehensive Care of Patients with PWS. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab. 2008; 93:4183–97.

3. Deal CL, Tony M, Höybye C, Allen DB, Tauber M, Christiansen JS, et al. GrowthHormone Research Society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013; 98:E1072–87.

4. Miller JL, Strong TV, Heinemann J. Medication trials for hyperphagia and food-related behaviors in Prader-Willi syndrome. Diseases. 2015; 3:78–85.

5. Sze L, Purtell L, Jenkins A, Loughnan G, Smith E, Herzog H, et al. Effects of a single dose of exenatide on appetite, gut hormones, and glucose homeostasis in adults with Prader-Willi syndrome. J Clin Endocrinol Metab. 2011; 96:E1314–9.

6. Nishikawa M, Mizutani T, Nakao T, Kamoda T, Takahashi S, Toyooka H. Respiratory failure due to morbid obesity in a patient with Prader-Willi syndrome: an experience of longterm mechanical ventilation. J Anesth. 2006; 20:300–3.

7. Yosipovitch G, DeVore A, Dawn A. Obesity and the skin: skin physiology and skin manifestations of obesity. J Am Acad Dermatol. 2007; 56:901–16.

8. Khera R, Murad MH, Chandar AK, Dulai PS, Wang Z, Prokop LJ, et al. Association of pharmacological treatments for obesity with weight loss and adverse events: a systematic review and meta-analysis. JAMA. 2016; 315:2424–34.

9. Seetho IW, Jones G, Thomson GA, Fernando DJ. Treating diabetes mellitus in Prader-Willi syndrome with exenatide. Diabetes Res Clin Pract. 2011; 92:e1. –2.

10. Senda M, Ogawa S, Nako K, Okamura M, Sakamoto T, Ito S. The glucagon-like peptide-1 analog liraglutide suppresses ghrelin and controls diabetes in a patient with Prader-Willi syndrome. Endocr J. 2012; 59:889–94.

11. Fintini D, Grugni G, Brufani C, Bocchini S, Cappa M, Crinò A. Use of GLP-1 receptor agonists in Prader-Willi syndrome: report of six cases. Diabetes Care. 2014; 37:e76. –7.

12. Salehi P, Hsu I, Azen CG, Mittelman SD, Geffner ME, Jeandron D. Effects of exenatide on weight and appetite in overweight adolescents and young adults with Prader-Willi syndrome. Pediatr Obes. 2017; 12:221–8.

13. Kweh FA, Miller JL, Sulsona CR, Wasserfall C, Atkinson M, Shuster JJ, et al. Hyperghrelinemia in Prader-Willi syndrome begins in early infancy long before the onset of hyperphagia. Am J Med Genet A. 2015; 167A:69–79.

14. Allas S, Caixàs A, Poitou C, Coupaye M, Thuilleaux D, Lorenzini F, et al. AZP-531, an unacylated ghrelin analog, improves food-related behavior in patients with Prader-Willi syndrome: a randomized placebo-controlled trial. PLoS One. 2018; 13:e0190849.

15. Dykens EM, Miller J, Angulo M, Roof E, Reidy M, Hatoum HT, et al. Intranasal carbetocin reduces hyperphagia in individuals with Prader-Willi syndrome. JCI Insight. 2018; 3.