open access

Vol 68, No 6 (2017)
Original paper
Submitted: 2017-03-22
Accepted: 2017-07-24
Published online: 2017-09-25
Get Citation

Sleep-related breathing disorders in patients with Prader-Willi syndrome depending on the period of growth hormone treatment

Agnieszka Lecka-Ambroziak, Małgorzata Jędrzejczak, Marta Wysocka-Mincewicz, Mieczysław Szalecki
·
Pubmed: 29022650
·
Endokrynol Pol 2017;68(6):676-681.

open access

Vol 68, No 6 (2017)
Original Paper
Submitted: 2017-03-22
Accepted: 2017-07-24
Published online: 2017-09-25

Abstract

Introduction: Sleep-related breathing disorders (SRBD) are commonly present in patients with Prader-Willi syndrome (PWS). Recombinant human growth hormone (rhGH) treatment is reported to improve breathing function in PWS, but the findings are not explicit. Material and methods: Screening polysomnography- polygraphy (PSG), assessing nasal respiratory flow, respiratory effort, and blood oxygen saturation, was used. Group 1 — before rhGH therapy (n = 11, mean age 3.0 years); PSG was repeated after the start of rhGH therapy in a mean time of 0.9 years in six patients (Group 1a). Group 2 — on rhGH treatment, for a mean time of four years (n = 17, mean age 8.8 years). Group 3 — without rhGH therapy due to severe obesity (n = 8, mean age 13.1 years). Results: Group 1 — mean apnoea-hypopnoea index (AHI) was 10.2, oxygen desaturation index (ODI)- 36.3, Group 1a- AHI 12.0, ODI 60.9, Group 2-AHI 9.0, ODI 25.1, Group 3- AHI 8.2, ODI 22.0. ODI was significantly higher in Group 1a than in the other groups (p < 0.005), but not strictly related to SRBD. The results in Group 2 did not differ significantly from those of Group 1. Conclusions: Our study proves the high frequency of SRBD among PWS patients, with worsening of ODI after short-term rhGH therapy.

Abstract

Introduction: Sleep-related breathing disorders (SRBD) are commonly present in patients with Prader-Willi syndrome (PWS). Recombinant human growth hormone (rhGH) treatment is reported to improve breathing function in PWS, but the findings are not explicit. Material and methods: Screening polysomnography- polygraphy (PSG), assessing nasal respiratory flow, respiratory effort, and blood oxygen saturation, was used. Group 1 — before rhGH therapy (n = 11, mean age 3.0 years); PSG was repeated after the start of rhGH therapy in a mean time of 0.9 years in six patients (Group 1a). Group 2 — on rhGH treatment, for a mean time of four years (n = 17, mean age 8.8 years). Group 3 — without rhGH therapy due to severe obesity (n = 8, mean age 13.1 years). Results: Group 1 — mean apnoea-hypopnoea index (AHI) was 10.2, oxygen desaturation index (ODI)- 36.3, Group 1a- AHI 12.0, ODI 60.9, Group 2-AHI 9.0, ODI 25.1, Group 3- AHI 8.2, ODI 22.0. ODI was significantly higher in Group 1a than in the other groups (p < 0.005), but not strictly related to SRBD. The results in Group 2 did not differ significantly from those of Group 1. Conclusions: Our study proves the high frequency of SRBD among PWS patients, with worsening of ODI after short-term rhGH therapy.
Get Citation

Keywords

sleep-related breathing disorders, Prader-Willi Syndrome, growth hormone treatment

About this article
Title

Sleep-related breathing disorders in patients with Prader-Willi syndrome depending on the period of growth hormone treatment

Journal

Endokrynologia Polska

Issue

Vol 68, No 6 (2017)

Article type

Original paper

Pages

676-681

Published online

2017-09-25

Page views

1670

Article views/downloads

1425

DOI

10.5603/EP.a2017.0057

Pubmed

29022650

Bibliographic record

Endokrynol Pol 2017;68(6):676-681.

Keywords

sleep-related breathing disorders
Prader-Willi Syndrome
growth hormone treatment

Authors

Agnieszka Lecka-Ambroziak
Małgorzata Jędrzejczak
Marta Wysocka-Mincewicz
Mieczysław Szalecki

References (27)
  1. Goldstone AP, Holland AJ, Hauffa BP, et al. speakers contributors at the Second Expert Meeting of the Comprehensive Care of Patients with PWS. Recommendations for the diagnosis and management of Prader-Willi syndrome. J Clin Endocrinol Metab. 2008; 93(11): 4183–4197.
  2. Menendez AA. Abnormal ventilatory responses in patients with Prader-Willi syndrome. Eur J Pediatr. 1999; 158(11): 941–942.
  3. Eiholzer U. Deaths in Children with Prader-Willi Syndrome. Horm Res Paediatr. 2005; 63(1): 33–39.
  4. Bakker B, Maneatis T, Lippe B. Sudden Death in Prader-Willi Syndrome: Brief Review of Five Additional Cases. Horm Res. 2007; 67: 203–204.
  5. Haqq AM, Stadler DD, Jackson RH, et al. Effects of growth hormone on pulmonary function, sleep quality, behavior, cognition, growth velocity, body composition, and resting energy expenditure in Prader-Willi syndrome. J Clin Endocrinol Metab. 2003; 88(5): 2206–2212.
  6. Festen DAM, de Weerd AW, van den Bossche RAS, et al. Sleep-related breathing disorders in prepubertal children with Prader-Willi syndrome and effects of growth hormone treatment. J Clin Endocrinol Metab. 2006; 91(12): 4911–4915.
  7. de Lind van Wijngaarden RFA, Siemensma EPC, Festen DAM, et al. Efficacy and safety of long-term continuous growth hormone treatment in children with Prader-Willi syndrome. J Clin Endocrinol Metab. 2009; 94(11): 4205–4215.
  8. Carrel AL, Myers SE, Whitman BY, et al. Long-term growth hormone therapy changes the natural history of body composition and motor function in children with prader-willi syndrome. J Clin Endocrinol Metab. 2010; 95(3): 1131–1136.
  9. Miller J, Silverstein J, Shuster J, et al. Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome. J Clin Endocrinol Metab. 2006; 91(2): 413–417.
  10. Nixon GM, Rodda CP, Davey MJ. Longitudinal association between growth hormone therapy and obstructive sleep apnea in a child with Prader-Willi syndrome. J Clin Endocrinol Metab. 2011; 96(1): 29–33.
  11. Llombart M, Chiner E, Gómez-Merino E, et al. Sleep Apnea-Hypopnea Syndrome in a Pediatric Population: Differences Between Children With Tonsillar Hypertrophy and Those With Concomitant Disease. Arch Bronconeumol. 2007; 43(12): 655–661.
  12. Iber C, Ancoli-Israel S, Chesson JrA, et al. The AASM Manual for the scoring of sleep and associated events: rules, terminology and technical specifications, 1st ed. Westchester, Illinois. : 2007.
  13. Wise MS, Nicholas CD, Grigg-Damberger MM, et al. Respiratory indications for polysomnography in children: an evidence-based review. Sleep. 2011; 34: 398A–398AW.
  14. Aurora RN, Zak RS, Karippot A, et al. American Academy of Sleep Medicine. Practice parameters for the respiratory indications for polysomnography in children. Sleep. 2011; 34(3): 379–388.
  15. Marcus CL, Brooks LJ, Draper KA, et al. American Academy of Pediatrics, American Academy of Pediatrics. Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics. 2012; 130(3): 576–584.
  16. Uliel S, Tauman R, Greenfeld M, et al. Normal polysomnographic respiratory values in children and adolescents. Chest. 2004; 125(3): 872–878.
  17. Fietze I, Dingli K, Diefenbach K, et al. Night-to-night variation of the oxygen desaturation index in sleep apnoea syndrome. Eur Respir J. 2004; 24(6): 987–993.
  18. Scholle S, Wiater A, Scholle HC. Normative values of polysomnographic parameters in childhood and adolescence: cardiorespiratory parameters. Sleep Med. 2011; 12(10): 988–996.
  19. Cohen M, Hamilton J, Narang I. Clinically important age-related differences in sleep related disordered breathing in infants and children with Prader-Willi Syndrome. PLoS One. 2014; 9(6): e101012.
  20. De Cock VC, Diene G, Molinas C, et al. Efficacy of modafinil on excessive daytime sleepiness in Prader-Willi syndrome. Am J Med Genet A. 2011; 155A(7): 1552–1557.
  21. Muzumdar H, Arens R. Diagnostic issues in pediatric obstructive sleep apnea. Proc Am Thorac Soc. 2008; 5(2): 263–273.
  22. Palczewska I, Niedźwiecka Z. Somatic development indices in children and youth of Warsaw. Med Wieku Rozw. 2001; 5: 1–118.
  23. Lin HY, Lin SP, Lin CC, et al. Polysomnographic characteristics in patients with Prader-Willi syndrome. Pediatr Pulmonol. 2007; 42(10): 881–887.
  24. Williams K, Scheimann A, Sutton V, et al. Sleepiness and sleep disordered breathing in Prader-Willi syndrome: relationship to genotype, growth hormone therapy, and body composition. J Clin Sleep Med. 2008; 4(2): 111–118.
  25. Deal CL, Tony M, Hoybye C, et al. Consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome. J Clin Endocrinol Metab. 2013; 98: E1072–E1087.
  26. Lindgren AC. increases CO2 response, ventilation and central inspiratory drive in children with Prader-Willi syndrome. Eur J Pediatr. 1999; 158: 936–940.
  27. Katz-Salamon M, Lindgren AC, Cohen G. The effect of growth hormone on sleep-related cardio-respiratory control. Acta Ped. 2012; 101: 643–648.

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Via MedicaWydawcą jest  VM Media Group sp. z o.o., Grupa Via Medica, ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail:  viamedica@viamedica.pl