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Effects of Growth Hormone Treatment on Sleep-Related Parameters in Adults With Prader-Willi Syndrome
被引:11
|作者:
Shukur, Hasanain Hamid
[1
]
Hussain-Alkhateeb, Laith
[2
]
Farholt, Stense
[3
]
Norregaard, Ole
[4
]
Jorgensen, Anders Palmstrom
[5
]
Hoybye, Charlotte
[1
,6
]
机构:
[1] Karolinska Inst, Dept Mol Med & Surg, L1 00,Anna Stecksens Gata 53, SE-17176 Solna, Sweden
[2] Univ Gothenburg, Sahlgrenska Acad, Inst Med, Global Hlth,Sch Publ Hlth & Community Med, S-40530 Gothenburg, Sweden
[3] Aarhus Univ Hosp, Ctr Rare Dis, Dept Pediat & Adolescent Med, DK-8200 Aarhus N, Denmark
[4] Aarhus Univ Hosp, Danish Resp Ctr West, Dept Anaesthesiol & Intens Care, DK-8200 Aarhus N, Denmark
[5] Oslo Univ Hosp, Rikshosp, Dept Endocrinol, Sect Specialized Endocrinol, N-0424 Oslo, Norway
[6] Karolinska Univ Hosp, Dept Endocrinol, SE-17176 Stockholm, Sweden
关键词:
Prader-Willi syndrome;
GH treatment;
polysomnography;
sleep-related breathing disorders;
BREATHING DISORDERS;
SUDDEN-DEATH;
CHILDREN;
PEOPLE;
PREVALENCE;
MORTALITY;
HEALTH;
D O I:
10.1210/clinem/dgab300
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Context: Prader-Willi syndrome (PWS) is a rare, genetic, multisymptom, neurodevelopmental disease due to lack of the expression of the paternal genes in the q11 to q13 region of chromosome 15. The main characteristics of PWS are muscular hypotonia, hyperphagia, obesity, behavioral problems, cognitive disabilities, and endocrine deficiencies, including growth hormone (GH) deficiency. Sleep apnea and abnormal sleep patterns are common in PWS. GH treatment might theoretically have a negative impact on respiration. Objective: Here we present the effect of GH treatment on polysomnographic measurements. Methods: Thirty-seven adults, 15 men and 22 women, with confirmed PWS were randomly assigned to 1 year of GH treatment (n = 19) or placebo (n = 18) followed by 2 years of GH treatment to all. Polysomnographic measurements were performed every 6 months. A mixed-effect regression model was used for comparison over time in the subgroup that received GH for 3 years. Results: At baseline median age was 29.5 years, body mass index 27.1, insulin-like growth factor 115 mu g/L, apnea-hypopnea index (AHI) 1.4 (range, 0.0-13.9), and sleep efficiency (SE) 89.0% (range, 41.0%-99.0%). No differences in sleep or respiratory parameters were seen between GH- and placebo-treated patients. SE continuously improved throughout the study, also after adjustment for BMI, and the length of the longest apnea increased. AHI inconsistently increased within normal range. Conclusion: SE improved during GH treatment and no clinical, significantly negative impact on respiration was seen.The etiology of breathing disorders is multifactorial and awareness of them should always be present in adults with PWS with or without GH treatment.
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页码:E3634 / E3643
页数:10
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