Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome

Prader–Willi syndrome (PWS) is a rare genetic disorder characterized by an insatiable appetite that leads to morbid obesity. Previous studies reported health problems in adults with PWS. However, studies on younger adults are lacking, and there are no specific studies of endocrine and metabolic illn...

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Main Authors: Eu-Seon Noh, Min-Sun Kim, Chiwoo Kim, Kyeongman Jeon, Seonwoo Kim, Sung Yoon Cho, Dong-Kyu Jin
Format: Article
Language:English
Published: MDPI AG 2022-05-01
Series:Journal of Personalized Medicine
Subjects:
Online Access:https://www.mdpi.com/2075-4426/12/6/858
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author Eu-Seon Noh
Min-Sun Kim
Chiwoo Kim
Kyeongman Jeon
Seonwoo Kim
Sung Yoon Cho
Dong-Kyu Jin
author_facet Eu-Seon Noh
Min-Sun Kim
Chiwoo Kim
Kyeongman Jeon
Seonwoo Kim
Sung Yoon Cho
Dong-Kyu Jin
author_sort Eu-Seon Noh
collection DOAJ
description Prader–Willi syndrome (PWS) is a rare genetic disorder characterized by an insatiable appetite that leads to morbid obesity. Previous studies reported health problems in adults with PWS. However, studies on younger adults are lacking, and there are no specific studies of endocrine and metabolic illness in this age group. We performed a retrospective cohort study of 68 individuals with PWS aged 19 to 34 years at Samsung Medical Center. The prevalence of endocrine and metabolic illnesses were compared with those in an age-, sex-, and BMI-matched healthy control group. Young adults with PWS had a higher prevalence of metabolic syndrome (35.3% vs. 4.4%), type 2 diabetes mellitus (50.0% vs. 5.4%), hypertension (30.8% vs. 16.1%), dyslipidemia (38.2% vs. 14.7%), decreased bone density (26.4% vs. 0.9%), and sleep apnea (32.3% vs. 4.4%) than controls (all <i>p</i> < 0.05). The PWS group that maintained recombinant human growth (rhGH) treatment in adulthood had a lower probability of having a BMI ≥ 30 at the last follow-up (odds ratio = 0.106 (0.012–0.948), <i>p</i> = 0.045). Endocrine and metabolic illnesses in individuals with PWS may have already started in the early teens; therefore, appropriate screening and early intervention are important. Better understanding of the natural history of PWS and age-related complications will lead to better-quality medical care for individuals with PWS.
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spelling doaj.art-0b28e54e01b14d16a2ebd63a4738064c2023-11-23T17:26:37ZengMDPI AGJournal of Personalized Medicine2075-44262022-05-0112685810.3390/jpm12060858Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi SyndromeEu-Seon Noh0Min-Sun Kim1Chiwoo Kim2Kyeongman Jeon3Seonwoo Kim4Sung Yoon Cho5Dong-Kyu Jin6Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaDivision of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaAcademic Research Service Headquarter, LSK Global PS, Seoul 04535, KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaDepartment of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, KoreaPrader–Willi syndrome (PWS) is a rare genetic disorder characterized by an insatiable appetite that leads to morbid obesity. Previous studies reported health problems in adults with PWS. However, studies on younger adults are lacking, and there are no specific studies of endocrine and metabolic illness in this age group. We performed a retrospective cohort study of 68 individuals with PWS aged 19 to 34 years at Samsung Medical Center. The prevalence of endocrine and metabolic illnesses were compared with those in an age-, sex-, and BMI-matched healthy control group. Young adults with PWS had a higher prevalence of metabolic syndrome (35.3% vs. 4.4%), type 2 diabetes mellitus (50.0% vs. 5.4%), hypertension (30.8% vs. 16.1%), dyslipidemia (38.2% vs. 14.7%), decreased bone density (26.4% vs. 0.9%), and sleep apnea (32.3% vs. 4.4%) than controls (all <i>p</i> < 0.05). The PWS group that maintained recombinant human growth (rhGH) treatment in adulthood had a lower probability of having a BMI ≥ 30 at the last follow-up (odds ratio = 0.106 (0.012–0.948), <i>p</i> = 0.045). Endocrine and metabolic illnesses in individuals with PWS may have already started in the early teens; therefore, appropriate screening and early intervention are important. Better understanding of the natural history of PWS and age-related complications will lead to better-quality medical care for individuals with PWS.https://www.mdpi.com/2075-4426/12/6/858Prader–Willi syndromeendocrineyoung adultobesitydiabetes mellitusdyslipidemia
spellingShingle Eu-Seon Noh
Min-Sun Kim
Chiwoo Kim
Kyeongman Jeon
Seonwoo Kim
Sung Yoon Cho
Dong-Kyu Jin
Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
Journal of Personalized Medicine
Prader–Willi syndrome
endocrine
young adult
obesity
diabetes mellitus
dyslipidemia
title Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
title_full Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
title_fullStr Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
title_full_unstemmed Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
title_short Endocrine and Metabolic Illnesses in Young Adults with Prader–Willi Syndrome
title_sort endocrine and metabolic illnesses in young adults with prader willi syndrome
topic Prader–Willi syndrome
endocrine
young adult
obesity
diabetes mellitus
dyslipidemia
url https://www.mdpi.com/2075-4426/12/6/858
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