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      Sleep-Disordered Breathing in Children with Prader-Willi Syndrome in Relation to Growth Hormone Therapy Onset

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          Objective: The aim of this study was to consider sleep apnea in Prader-Willi syndrome (PWS) children depending on age at growth hormone (GH) therapy onset. Study Design: We analyzed longitudinally cardiorespiratory polygraphy of 62 PWS children (aged 0–2.5 years at baseline). Twenty-one children (Group A) started GH-therapy during and 41 children (Group B) after their first year of life. Data were acquired before, at 3 and 6 months, then 1.2, 2.2, and 3.2 years after GH onset. Outcomes were determined with the obstructive apnea hypopnea index (OAHI), central apnea index (CAI), oxygen desaturation index (ODI), and by measuring obstructive sleep apnea (OSA) and peripheral blood oxygen saturation (SpO<sub>2</sub>). Results: We observed no significant differences in OAHI, CAI, ODI, and SpO<sub>2</sub> depending on treatment onset. At baseline, 5/21 patients (23.8%) in Group A versus 15/41 patients (36.6%) in Group B showed pathological sleep apnea (OAHI ≥1.5). Pathological OSA increased significantly in Group A during the first 3 months of therapy but dropped below baseline after 1 year in both groups. ODI changed during GH therapy in both groups (from 4.0 to 2.6 in Group A, and 3.6 to 1.6 in Group B; baseline to 3.2 years; p < 0.05). Conclusions: OSA in PWS children appears to develop independently of treatment onset. Treatment may therefore safely be initiated early but should be accompanied by regular sleep analysis.

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          Most cited references 15

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          Epidemiology of pediatric obstructive sleep apnea.

          Pediatric obstructive sleep apnea (OSA) has become widely recognized only in the last few decades as a likely cause of significant morbidity among children. Many of the clinical characteristics of pediatric OSA, and the determinants of its epidemiology, differ from those of adult OSA. We systematically reviewed studies on the epidemiology of conditions considered part of a pediatric sleep-disordered breathing (SDB) continuum, ranging from primary snoring to OSA. We highlight a number of methodologic challenges, including widely variable methodologies for collection of questionnaire data about symptomatology, definitions of habitual snoring, criteria for advancing to further diagnostic testing, and objective diagnostic criteria for SDB or OSA. In the face of these limitations, estimated population prevalences are as follows: parent-reported "always" snoring, 1.5 to 6%; parent-reported apneic events during sleep, 0.2 to 4%; SDB by varying constellations of parent-reported symptoms on questionnaire, 4 to 11%; OSA diagnosed by varying criteria on diagnostic studies, 1 to 4%. Overall prevalence of parent-reported snoring by any definition in meta-analysis was 7.45% (95% confidence interval, 5.75-9.61). A reasonable preponderance of evidence now suggests that SDB is more common among boys than girls, and among children who are heavier than others, with emerging data to suggest a higher prevalence among African Americans. Less convincing data exist to prove differences in prevalence based on age. We conclude by outlining specific future research needs in the epidemiology of pediatric SDB.
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            Long-term treatment with recombinant insulin-like growth factor (IGF)-I in children with severe IGF-I deficiency due to growth hormone insensitivity.

            Children with severe IGF-I deficiency due to congenital or acquired defects in GH action have short stature that cannot be remedied by GH treatment. The objective of the study was to examine the long-term efficacy and safety of recombinant human IGF-I (rhIGF-I) therapy for short children with severe IGF-I deficiency. Seventy-six children with IGF-I deficiency due to GH insensitivity were treated with rhIGF-I for up to 12 yr under a predominantly open-label design. The study was conducted at general clinical research centers and with collaborating endocrinologists. Entry criteria included: age older than 2 yr, sd scores for height and circulating IGF-I concentration less than -2 for age and sex, and evidence of resistance to GH. rhIGF-I was administered sc in doses between 60 and 120 microg/kg twice daily. Height velocity, skeletal maturation, and adverse events were measured. Height velocity increased from 2.8 cm/yr on average at baseline to 8.0 cm/yr during the first year of treatment (P < 0.0001) and was dependent on the dose administered. Height velocities were lower during subsequent years but remained above baseline for up to 8 yr. The most common adverse event was hypoglycemia, which was observed both before and during therapy. It was reported by 49% of treated subjects. The next most common adverse events were injection site lipohypertrophy (32%) and tonsillar/adenoidal hypertrophy (22%). Treatment with rhIGF-I stimulates linear growth in children with severe IGF-I deficiency due to GH insensitivity. Adverse events are common but are rarely of sufficient severity to interrupt or modify treatment.
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              Review of 64 cases of death in children with Prader-Willi syndrome (PWS).

              Several deaths have been reported in children with Prader-Willi syndrome (PWS) following treatment with growth hormone (GH). We collected all of the reports of deaths in PWS children, both in treatment and non-treatment groups, analyzed the causes of the death and compared the two groups. We conducted an exhaustive search for reports using bibliographic databases, toxicology pharmacovigilance databases, and personal communications. Sixty-four PWS children (42M/22F) aged from a few days to 19 years were identified, 28 received GH treatment. Our results show that respiratory disorders were the most common cause of death (respiratory insufficiency or infections) which were reported in 61% of the children (68% in GH-treated and 55.5% in -untreated patients). We found no significant differences in gender, prevalence of obesity or prevalence of sleep apnea, between the patients treated with GH and the untreated patients. Nevertheless, most of the deaths in GH-treated children (75%) occurred during the first 9 months after the initiation of GH treatment. Our analysis shows the high frequency of respiratory infections in both GH-treated and -untreated PWS children. The first 9 months of GH treatment seems to be a high-risk period emphasizing the need for comprehensive care before and during GH treatment. Copyright 2008 Wiley-Liss, Inc.

                Author and article information

                Horm Res Paediatr
                Hormone Research in Paediatrics
                S. Karger AG
                August 2020
                12 June 2020
                : 93
                : 2
                : 85-93
                aPediatric Endocrinology and Diabetology, Children’s Hospital, University of Bonn, Bonn, Germany
                bPediatric Endocrinology and Diabetology, St. Bernward Hospital, Hildesheim, Germany
                cChildren’s University Hospital Erlangen, Erlangen, Germany
                dUniversity Hospital of Bonn, Institute for Medical Biometry, Bonn, Germany
                Author notes
                *Bettina Gohlke, Children’s University Hospital Bonn, Department of Paediatric Endocrinology and Diabetology, Adenauerallee 119, DE–53113 Bonn (Germany), bettina-gohlke@t-online.de
                506943 Horm Res Paediatr 2020;93:85–93
                © 2020 S. Karger AG, Basel

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                Page count
                Figures: 1, Tables: 2, Pages: 9
                Research Article


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