scholarly journals Sleep-Related Breathing Disorders in Young Adults With Prader-Willi Syndrome: A Placebo-Controlled, Crossover GH Trial

2019 ◽  
Vol 104 (9) ◽  
pp. 3931-3938
Author(s):  
Stephany H Donze ◽  
Al W de Weerd ◽  
Renilde A S van den Bossche ◽  
Koen F M Joosten ◽  
Anita C S Hokken-Koelega
Keyword(s):  
Young Adults ◽  
Adult Height ◽  
Uniparental Disomy ◽  
Apnea Hypopnea Index ◽  
Prader Willi Syndrome ◽  
Double Blind ◽  
Sleep Related Breathing Disorders ◽  
Maternal Uniparental Disomy ◽  
Breathing Disorders ◽  
Obstructive Sleep

Abstract Context Sleep-related breathing disorders (SRBD) are common in people with Prader-Willi syndrome (PWS). Young adults with PWS benefit from GH continuation after attaining adult height by maintaining the improved body composition obtained during childhood. There are, no studies about the effects of GH on SRBD in young adults with PWS who were treated with GH during childhood. Objective Investigate the effects of GH vs placebo on SRBD in young adults with PWS who were treated with GH during childhood and had attained adult height. Design Two-year, randomized, double-blind, placebo-controlled, crossover study in 27 young adults with PWS, stratified for sex and body mass index. Setting Dutch PWS Reference Center. Intervention Crossover intervention with GH (0.67 mg/m2/d) and placebo, both over one year. Main Outcome Measures Apnea hypopnea index (AHI), obstructive apnea index (OAI), central apnea index (CAI), measured by polysomnography. Results Compared with placebo, GH did not increase AHI, CAI, or OAI (P > 0.35). The effect of GH vs placebo was neither different between men and women, nor between patients with a deletion or maternal uniparental disomy/imprinting center defect. After two years, there was no difference in AHI, CAI, or OAI compared with baseline (P > 0.18). Two patients (7%) fulfilled the criteria of obstructive sleep apnea regardless of GH or placebo. Conclusions GH compared with placebo does not cause a substantial increase in AHI, CAI, or OAI in adults with PWS who were treated with GH during childhood and have attained adult height. Our findings are reassuring and prove that GH can be administered safely.

scholarly journals Comparison of Frequency and Severity of Sleep-Related Breathing Disorders in Children with Simple Obesity and Paediatric Patients with Prader–Willi Syndrome

2021 ◽  
Vol 11 (2) ◽  
pp. 141
Author(s):  
Agnieszka Lecka-Ambroziak ◽  
Marta Wysocka-Mincewicz ◽  
Anna Świercz ◽  
Małgorzata Jędrzejczak ◽  
Mieczysław Szalecki
Keyword(s):  
Insulin Resistance ◽  
Apnea Hypopnea Index ◽  
Oral Glucose ◽  
Prader Willi Syndrome ◽  
Model Assessment ◽  
Sleep Related Breathing Disorders ◽  
Breathing Disorders ◽  
Simple Obesity ◽  
Rhgh Treatment ◽  
Group 1

Sleep-related breathing disorders (SRBDs) can be present in children with simple obesity and with Prader–Willi syndrome (PWS) and influence an individual diagnostic and treatment approach. We compared frequency and severity of SRBDs in children with simple obesity and with PWS, both without and on recombinant human growth hormone (rhGH) treatment, and correlation of SRBDs with insulin resistance tests. A screening polysomnography-polygraphy (PSG), the oral glucose tolerance test (OGTT) and homeostasis model assessment of insulin resistance (HOMA-IR) were analysed in three groups of patients—with simple obesity (group 1, n = 30, mean age 14.2 years), patients with PWS without the rhGH therapy (group 2, n = 8, mean age 13.0 years) and during the rhGH treatment (group 3, n = 17, mean age 8.9 years). The oxygen desaturation index (ODI) was significantly higher in groups 2 and 3, compared to group 1 (p = 0.00), and hypopnea index (HI) was higher in group 1 (p = 0.03). Apnea–hypopnea index (AHI) and apnea index (AI) results positively correlated with the insulin resistance parameters in groups 1 and 3. The PSG values worsened along with the increasing insulin resistance in children with simple obesity and patients with PWS treated with rhGH that may lead to a change in the patients’ care.

scholarly journals P.2.35 Heart rate variability and sleep-related breathing disorders in commercial drivers

2019 ◽  
Vol 76 (Suppl 1) ◽  
pp. A96.1-A96
Author(s):  
Wei-te Wu ◽  
Yu-Jen Lin ◽  
Saou-Hsing Liou
Keyword(s):  
Heart Rate ◽  
Heart Rate Variability ◽  
Sleep Apnea ◽  
Fasting Blood Glucose ◽  
Apnea Hypopnea Index ◽  
Sleep Related Breathing Disorders ◽  
Cross Sectional ◽  
Breathing Disorders ◽  
Obstructive Sleep ◽  
Commercial Drivers

BackgroundSleep-related breathing disorders, such as obstructive sleep apnea (OSA), seems to have an important influence on the autonomic nervous function on the heart, and associated with cardiovascular mortality. Therefore, we assessed the relations of sleep apnea-related parameters with 5 min heart rate variability (HRV) in a large cohort of male commercial drivers.MethodsThis cross-sectional study recruited 245 male bus drivers from one transportation company in Taiwan. Each participant was evaluated by a polysomnography (PSG) test and by blood lipids examination. Severity of OSA was categorized according to the apnea-hypopnea index (AHI). In addition, short-term HRV was tested.ResultsSubjects were categorized into severe OSA group (n=44; 17.9%), moderate and mild OSA group (n=117; 47.8%), and non-OSA group (n=84; 34.3%). AHI had negative correlations with SDNN (β=−0.075, p=0.007) and RMSSD (β=−0.121, p<0.001), adjusting for age, body mass index, smoking, drinking, systolic blood pressure, HDL, and fasting blood glucose. RMSSD level was decreased with severe OSA (β=−0.266, p=0.042) even adjusting for confounders.ConclusionsThe findings showed a low level of RMSSD in OSA among commercial drivers, especially in the severe group category. Decreased SDNN and RMSSD levels are associated with AHI level in commercial drivers, suggesting that they are tightly linked with autonomic dysfunction.

scholarly journals Effect of continuous positive airway pressure treatment of obstructive sleep apnea-hypopnea in multiple sclerosis: A randomized, double-blind, placebo-controlled trial (SAMS-PAP study)

2021 ◽  
pp. 135245852110103
Author(s):  
Sulaiman Khadadah ◽  
R John Kimoff ◽  
Pierre Duquette ◽  
Vincent Jobin ◽  
Yves Lapierre ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Continuous Positive Airway Pressure ◽  
Airway Pressure ◽  
Primary Outcome ◽  
Positive Airway Pressure ◽  
Apnea Hypopnea Index ◽  
Double Blind ◽  
Obstructive Sleep

Objective: The aim of this study was to evaluate the effect of continuous positive airway pressure (CPAP) treatment on the Fatigue Severity Scale (FSS, preplanned primary outcome), another fatigue measure, sleep quality, somnolence, pain, disability, and quality of life in multiple sclerosis (MS) patients with obstructive sleep apnea-hypopnea (OSAH). Methods: In a randomized, double-blind trial (NCT01746342), MS patients with fatigue, poor subjective sleep quality, and OSAH (apnea-hypopnea index of ⩾ 15 events per hour/sleep), but without severe OSAH (apnea-hypopnea index > 30, and 4% oxygen desaturation index > 15 events/hour or severe somnolence), were randomized to fixed CPAP or sham CPAP for 6 months. Outcome assessments were performed at 3 and 6 months. Results: Of 49 randomized patients, 34 completed the protocol. Among completers, FSS did not improve with CPAP compared to sham at 6 months. FSS tended to improve ( p = 0.09), and sleepiness (Epworth Sleepiness Scale) improved significantly ( p = 0.03) at 3 months with CPAP compared to sham, but there were no other improvements with CPAP at either study evaluation. Conclusion: In non-severe OSAH patients, CPAP did not significantly improve the primary outcome of FSS change at 6 months. In secondary analyses, we found a trend to improved FSS, and a significant reduction in somnolence with CPAP at 3 months.

857 Too sleepy: A pediatric case of Prader Willi Syndrome and Narcolepsy

SLEEP ◽  
2021 ◽  
Vol 44 (Supplement_2) ◽  
pp. A333-A333
Author(s):  
Elizabeth Lam ◽  
Sonal Malhotra ◽  
Daniel Glaze
Keyword(s):  
Daytime Sleepiness ◽  
Sleep Latency ◽  
Chromosome Region ◽  
Genetic Disorder ◽  
Sleep Onset ◽  
Apnea Hypopnea Index ◽  
Prader Willi Syndrome ◽  
Obstructive Sleep ◽  
Patient Reported

Abstract Introduction Prader Willi syndrome (PWS) is a genetic disorder due to deletion of the paternal copies of genes within the chromosome region 15q11-q13. Individuals with PWS are commonly seen with obesity, sleep disordered breathing, and excessive daytime sleepiness (EDS). While the exact cause of EDS in individuals with PWS is not fully understood, there have been reports of PWS with narcolepsy-like syndrome. We report a case of a patient with PWS with findings suggesting the diagnosis of Narcolepsy Type 2. Report of case(s) Our patient is a 12-year-old male with PWS and 2nd degree heart block who was evaluated in our pediatric sleep center. He has a previous diagnosis of mild obstructive sleep apnea (OSA) with an apnea hypopnea index (oAHI) of 3.2. At 12 years of age, mother and patient reported that he had increased snoring, weight gain, EDS with a Pediatric Daytime Sleepiness Score (PDSS) of 10 and frequent refreshing naps during the daytime. Patient denied cataplexy during that visit. Subsequently, 2-week actigrapghy was performed which demonstrated an average total night sleep of 8 hours and 8 minutes. Overnight PSG with Multiple Sleep Latency Test (MSLT) demonstrated an oAHI of 4.8, total sleep time of 6.88 hours. During the MSLT, the mean sleep latency was 6.2 minutes and 5 sleep onset REM periods were observed over 5 nap opportunities. At his follow-up visit, methylphenidate was initiated after clearance by his cardiologist. Patient and mother opted for medical management of his mild OSA with Fluticisone and Montelukast. At his follow-up appointment, the patient had improvement in daytime sleepiness with a PDSS of 2 despite taking his Methylphenidate at night. Patient was instructed to switch to morning Methylphenidate dosing to optimize treatment of his EDS. Conclusion EDS is a common complaint seen in patients with PWS, however the etiology of it is not entirely understood. It is thought to be centrally mediated with components of hypersomnia and narcolepsy like-symptoms. More research is needed to better understand, diagnose and adequately treat patients with PWS and EDS. Support (if any):

Adenotonsillectomy for the Treatment of Obstructive Sleep Apnea in Children with Prader-Willi Syndrome: A Meta-analysis

2019 ◽  
Vol 162 (2) ◽  
pp. 168-176
Author(s):  
Chia-Hsuan Lee ◽  
Wei-Chung Hsu ◽  
Jenq-Yuh Ko ◽  
Te-Huei Yeh ◽  
Ming-Tzer Lin ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Success Rate ◽  
Cochrane Review ◽  
Velopharyngeal Insufficiency ◽  
Apnea Hypopnea Index ◽  
Prader Willi Syndrome ◽  
Postoperative Changes ◽  
Obstructive Sleep ◽  
Osa Treatment

Objective Adenotonsillectomy outcomes in obstructive sleep apnea (OSA) treatment among children with Prader-Willi syndrome (PWS) remain unclear. This study aimed to elucidate the effectiveness of adenotonsillectomy in OSA treatment among children with PWS. Data Source PubMed, MEDLINE, Embase, and Cochrane Review up to February 2019. Review Methods The registry number of the protocol published on PROSPERO was CRD42015027053. Two authors independently searched the relevant database. Polysomnography outcomes in these children were examined, including net postoperative changes in the apnea-hypopnea index (AHI), net postoperative changes in the minimum and mean oxygen saturation, the overall success rate for a postoperative AHI <1, and the overall success rate for a postoperative AHI <5. Results Six studies with 41 patients were analyzed (mean age, 5.0 years; 55% boys; mean sample size, 6.8 patients). All children had PWS and received adenotonsillectomy for the treatment of OSA. The AHI was 13.1 events per hour (95% CI, 11.0-15.1) before surgery and 4.6 events per hour (95% CI, 4.1-5.1) after surgery. The mean change in the AHI was a significant reduction of 8.0 events per hour (95% CI, −10.8 to −5.1). The overall success rate was 21% (95% CI, 11%-38%) for a postoperative AHI <1 and 71% (95% CI, 54%-83%) for a postoperative AHI <5. Some patients developed velopharyngeal insufficiency postoperatively. Conclusion Adenotonsillectomy was associated with OSA improvement among children with PWS. However, residual OSA was frequently observed postoperatively in these patients.

Safety and effectiveness of growth hormone therapy in infants with Prader-Willi syndrome younger than 2 years: a prospective study

2019 ◽  
Vol 32 (8) ◽  
pp. 879-884 ◽  
Author(s):  
Raquel Corripio ◽  
Carla Tubau ◽  
Laura Calvo ◽  
Carme Brun ◽  
Núria Capdevila ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Prospective Study ◽  
Mixed Model ◽  
Standard Deviation Score ◽  
Uniparental Disomy ◽  
Chromosome 15 ◽  
Prader Willi Syndrome ◽  
Gh Treatment ◽  
Maternal Uniparental Disomy ◽  
A Prospective Study

Abstract Background There is little evidence of the effects of early treatment with growth hormone (GH) in infants with Prader-Willi syndrome (PWS). A prospective study was conducted to assess the safety of GH therapy in infants younger than 2 years of age with PWS. Methods A total of 14 patients with PWS started treatment with GH under the age of 2 years and were followed over a 2-year period. A deletion of chromosome 15 was present in nine infants (64.3%) and maternal uniparental disomy 15 in five infants (35.7%). The median age at start of GH treatment was 9.6 months (interquartile range [IQR] 9.0–18.3 months). Changes in height standard deviation score (SDS), body mass index (BMI) SDS and subcapsular and tricipital skinfolds in the follow-up period were evaluated with a mixed-model regression analysis using the Package R. Results There were no fatal adverse events. A significant decrease (p < 0.001) in tricipital and subcapsular skinfold thickness, with an upward trend of height SDS and a downward trend of BMI SDS, was observed. Infants who started GH before 15 months of age started walking at a median of 18.0 [17.0–19.5] months vs. 36.6 [36.3–37.8] months for those who began treatment with GH after 15 months of age (p = 0.024). Conclusions GH treatment in infants with PWS less than 2 years of age is safe and improved body composition. Infants who received GH before the age of 15 months started to walk earlier.

scholarly journals The prevalence of common sleep disorders in young adults: a descriptive population-based study

SLEEP ◽  
2020 ◽  
Vol 43 (10) ◽  
Author(s):  
Nigel McArdle ◽  
Sarah V Ward ◽  
Romola S Bucks ◽  
Kathleen Maddison ◽  
Anne Smith ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Young Adults ◽  
Sleep Disorders ◽  
Sleep Disorder ◽  
Restless Legs Syndrome ◽  
Survey Methods ◽  
Apnea Hypopnea Index ◽  
Restless Legs ◽  
Obstructive Sleep

Abstract Sleep disorders in adults are associated with adverse health effects including reduced quality of life and increased mortality. However, there is little information on sleep disorders in young adults. A cross-sectional observational study was undertaken in 1,227 young adults participating in the Western Australian Pregnancy (Raine) Study (2012–2014) to describe the prevalence of common sleep disorders. In-laboratory polysomnography (PSG) and validated survey methods were used, including the Epworth Sleepiness Scale, Pittsburgh Sleep Symptom Questionnaire-Insomnia, and International Restless Legs Syndrome Study Group criteria. A total of 1,146 participants completed a core questionnaire, 1,051 completed a sleep-focused questionnaire and 935 had analyzable PSG data. Participants had a mean age of 22.2 years and female to male ratio of 1.1 to 1. The respective sleep disorder prevalences in females and males were: obstructive sleep apnea (OSA) (apnea-hypopnea index [AHI]: ≥5 events/hour) 14.9% (95% CI: 11.8–18.5) and 26.9% (95% CI: 22.9–31.2); chronic insomnia, 19.3% (95% CI: 16.7–23.9) and 10.6% (95% CI: 8.3–13.9); restless legs syndrome, 3.8% (95% CI: 2.4–5.6) and 1.9% (95% CI: 0.9–3.4); and abnormal periodic leg movements during sleep (&gt;5 movements/hour), 8.6% (95% CI: 6.3–11.5) and 9.6% (95% CI: 7.1–12.7). There were statistically significant differences in prevalence between sexes for OSA and insomnia, which persisted after adjustment for body mass index and education. In those with complete data on all sleep-related assessments (n = 836), at least one sleep disorder was present in 41.0% of females and 42.3% of males. Sleep disorders are very common in young adults. Health practitioners should be aware of these high prevalences, as early identification and treatment can improve quality of life and may reduce later morbidity and mortality.

scholarly journals Prevalence of Sleep-Related Disordered Breathing among Acromegaly Patients and its Correlation to Cephalometric Parameters: An Indian Perspective

2020 ◽  
Vol 56 (01) ◽  
pp. 09-14
Author(s):  
Sreejith M. ◽  
Mohd Ashraf Ganie ◽  
Ravinder Goswami ◽  
Nikhil Tandon ◽  
Randeep Guleria ◽  
...  
Keyword(s):  
Sleep Apnea ◽  
Soft Tissues ◽  
Central Sleep Apnea ◽  
Human Growth ◽  
Apnea Hypopnea Index ◽  
Sleep Related Breathing Disorders ◽  
Obstructive Sleep ◽  
Long Term Follow Up ◽  
Treatment Naïve ◽  
Magnetic Resonance Imaging Mri

Abstract Introduction Sleep-related breathing disorders (SRBDs) including obstructive sleep apnea (OSA) and central sleep apnea (CSA) are quite common and are the leading causes of mortality in acromegaly. OSA in acromegaly is generally attributed to changes in oropharyngeal soft tissues. Data on OSA in Indian acromegaly are scant, especially cephalometric findings. The aim of this study is to evaluate the burden of SRBDs in acromegaly and its correlation to cephalometric parameters. Materials and Methods A total of 32 subjects (20 men and 12 women), diagnosed with acromegaly on the basis of standard clinical, biochemical, and hormonal measurements were recruited. In addition to the above parameters, polysomnography and magnetic resonance imaging (MRI) of the pharynx were performed in all subjects. Results The mean age of the subjects was 42.66 ± 11.13 years (range = 26–66) and mean duration of study after first presentation was 7.6 ± 6.3 years (range = 0.25–32). A total of 28 of 32 (93.3%) subjects had sellar MRI documented macroadenomas while 20 (62.5%) patients were treatment naive at the time of assessment. Twenty-nine (90.6%) patients had evidence of SRBD and all of them had OSA subtype. The Apnea–Hypopnea Index (AHI) indicating severity of OSA (mild 21.8%, moderate 34.4%, and severe 34.4%) correlated positively with tongue length, uvula length, and uvula thickness on MRI. However, AHI had no correlation with the severity of GH excess or disease activity or individual parameters such as weight, body mass index, blood pressure, hemoglobin A1c, serum human growth hormone, and insulin-like growth factor-1 level. Conclusion SRBD, the generally overlooked comorbidity, is highly prevalent in subjects with acromegaly and is almost always due to OSA, the severity of which correlates positively with tongue and uvula size. Well-designed, long-term follow-up study on a large cohort of acromegalic patients is required to improve our understanding on the subject.

Sign in / Sign up

Export Citation Format

Share Document