Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome
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 obesit...
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| Format: | Article |
| Language: | English |
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Korean Society of Pediatric Endocrinology
2020-03-01
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| Series: | Annals of Pediatric Endocrinology & Metabolism |
| Subjects: | |
| Online Access: | http://e-apem.org/upload/pdf/apem-2020-25-1-52.pdf |
| _version_ | 1818902686640111616 |
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| author | Yoo-Mi Kim Yeoun Joo Lee Soo Yeon Kim Chong Kun Cheon Han Hyuk Lim |
| author_facet | Yoo-Mi Kim Yeoun Joo Lee Soo Yeon Kim Chong Kun Cheon Han Hyuk Lim |
| author_sort | Yoo-Mi Kim |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-19T20:39:36Z |
| format | Article |
| id | doaj.art-0f5659f6031a488f98fb7ae3a632eff3 |
| institution | Directory Open Access Journal |
| issn | 2287-1012 2287-1292 |
| language | English |
| last_indexed | 2024-12-19T20:39:36Z |
| publishDate | 2020-03-01 |
| publisher | Korean Society of Pediatric Endocrinology |
| record_format | Article |
| series | Annals of Pediatric Endocrinology & Metabolism |
| spelling | doaj.art-0f5659f6031a488f98fb7ae3a632eff32022-12-21T20:06:26ZengKorean Society of Pediatric EndocrinologyAnnals of Pediatric Endocrinology & Metabolism2287-10122287-12922020-03-01251525610.6065/apem.2020.25.1.52804Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndromeYoo-Mi Kim0Yeoun Joo Lee1Soo Yeon Kim2Chong Kun Cheon3Han Hyuk Lim4 Department of Pediatrics, Chungnam National University Hospital, Chungnam National College of Medicine, Daejeon, Korea Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National College of Medicine, Yangsan, Korea Department of Rehabilitation, Pusan National University Children’s Hospital, Pusan National College of Medicine, Yangsan, Korea Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National College of Medicine, Yangsan, Korea Department of Pediatrics, Chungnam National University Hospital, Chungnam National College of Medicine, Daejeon, KoreaPrader-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.http://e-apem.org/upload/pdf/apem-2020-25-1-52.pdfliraglutideprader-willi syndromeadolescentobesitymorbid |
| spellingShingle | Yoo-Mi Kim Yeoun Joo Lee Soo Yeon Kim Chong Kun Cheon Han Hyuk Lim Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome Annals of Pediatric Endocrinology & Metabolism liraglutide prader-willi syndrome adolescent obesity morbid |
| title | Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome |
| title_full | Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome |
| title_fullStr | Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome |
| title_full_unstemmed | Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome |
| title_short | Successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent Prader-Willi syndrome |
| title_sort | successful rapid weight reduction and the use of liraglutide for morbid obesity in adolescent prader willi syndrome |
| topic | liraglutide prader-willi syndrome adolescent obesity morbid |
| url | http://e-apem.org/upload/pdf/apem-2020-25-1-52.pdf |
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