프라더-윌리증후군 소아의 수면호흡장애에 대한 연구
Descriptive analysis on sleep-disordered breathing in children with Prader-Willi syndrome
0
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Purpose: Sleep-disordered breathing is one of the complicating characteristics in patients with Prader-Willi syndrome (PWS). No detailed description and risk factors are suggested on breathing problems during sleep in Korean children with PWS. Methods: We reviewed clinical and sleep-study data in patients with PWS who underwent polysomnography before they took the growth hormone therapy. Results: Of the 27 patients with PWS, 25 (92.6%) had sleep-disordered breathing, of whom 14 showed moderate to severe sleep apnea. Obstructive dominance was prevalent (64%), followed by central dominance (24%). The apnea-hypopnea index (AHI) increased with increasing weight-for-height z-score (WHZ) (r=0.50, P=0.009), but did not differ by age. Apnea duration of over 12 months was longer in the patient group than in the infant group (15.1±4.3 seconds vs. 9.4±1.7 seconds, P=0.001) and in the obese than non-obese groups (16.8±4.3 seconds vs. 10.0±2.0 seconds, P=0.003). Desaturation below 70% was more common in the obese than nonobese subjects (3/9 vs. 0/18, P=0.029). Age was not different between the central and obstructive apnea groups, but patients with central apnea tended to be younger than patients with obstructive apnea (median [range]: 8.0 months [6.0–12.0 months] vs. 16.5 months [8.5–79.5 months], P=0.092). In addition, patients with obstructive apnea showed higher AHI (12.8 [5.9–19.2] vs. 3.9 [3.4–4.5], P=0.045). Conclusion: Sleep-disordered breathing is common in PWS children with different intensity and patterns according to age and BMI. Close monitoring of breathing problems during sleep is required in PWS patients.
Abstract
Purpose: Sleep-disordered breathing is one of the complicating characteristics in patients with Prader-Willi syndrome (PWS). No detailed description and risk factors are suggested on breathing problems during sleep in Korean children with PWS. Methods: We reviewed clinical and sleep-study data in patients with PWS who underwent polysomnography before they took the growth hormone therapy. Results: Of the 27 patients with PWS, 25 (92.6%) had sleep-disordered breathing, of whom 14 showed moderate to severe sleep apnea. Obstructive dominance was prevalent (64%), followed by central dominance (24%). The apnea-hypopnea index (AHI) increased with increasing weight-for-height z-score (WHZ) (r=0.50, P=0.009), but did not differ by age. Apnea duration of over 12 months was longer in the patient group than in the infant group (15.1±4.3 seconds vs. 9.4±1.7 seconds, P=0.001) and in the obese than non-obese groups (16.8±4.3 seconds vs. 10.0±2.0 seconds, P=0.003). Desaturation below 70% was more common in the obese than nonobese subjects (3/9 vs. 0/18, P=0.029). Age was not different between the central and obstructive apnea groups, but patients with central apnea tended to be younger than patients with obstructive apnea (median [range]: 8.0 months [6.0–12.0 months] vs. 16.5 months [8.5–79.5 months], P=0.092). In addition, patients with obstructive apnea showed higher AHI (12.8 [5.9–19.2] vs. 3.9 [3.4–4.5], P=0.045). Conclusion: Sleep-disordered breathing is common in PWS children with different intensity and patterns according to age and BMI. Close monitoring of breathing problems during sleep is required in PWS patients.
참고문헌 (25)
- Fehrm J, "Effectiveness of adenotonsillectomy vs watchful waiting in young children with mild to moderate obstructive sleep apnea : a randomized clinical trial", JAMA Otolaryngol Head Neck Surg, 146, pp.647~654 (2020)
- Kaditis AG, "Obstructive sleep disordered breathing in 2-to 18-year-old children : diagnosis and management", Eur Respir J, 47, pp.69~94 (2016)
- Cassidy SB, "Prader-Willi syndrome", Genet Med, 14, pp.10~26 (2012)
- Gunay-Aygun M, "The changing purpose of Prader-Willi syndrome clinical diagnostic criteria and proposed revised criteria", Pediatrics, 108, pp.E92~ (2001)
- Lin HY, "Polysomnographic characteristics in patients with Prader-Willi syndrome", Pediatr Pulmonol, 42, pp.881~887 (2007)
- Deal CL, "GrowthHormone Research Society workshop summary: consensus guidelines for recombinant human growth hormone therapy in Prader-Willi syndrome", J Clin Endocrinol Metab, 98, pp.E1072~E1087 (2013)
- WHO Multicentre Growth Reference Study Group, "WHO Child Growth Standards based on length", height, weight and age, 2006, pp.450, 76~85
- 김재현, "The 2017 Korean National Growth Charts for children and adolescents: development, improvement, and prospects", Clinical and Experimental Pediatrics, 61(5), pp.135~149 (2018), 대한소아청소년과학회
- "The 2007 AASM Scoring Manual vs. the AASM Scoring Manual v2.0" (2012)[URL]
- Roland PS, "Clinical practice guideline : polysomnography for sleep-disordered breathing prior to tonsillectomy in children", Otolaryngol Head Neck Surg, 145(1 Suppl), pp.S1~S15 (2011)
- Holm VA, "Prader-Willi syndrome : consensus diagnostic criteria", Pediatrics, 91, pp.398~402 (1993)
- Al-Saleh S, "Longitudinal evaluation of sleep-disordered breathing in children with Prader-Willi Syndrome during 2 years of growth hormone therapy", J Pediatr, 162, pp.263~268 (2013)
- O’Donoghue FJ, "Sleep-disordered breathing in Prader-Willi syndrome and its association with neurobehavioral abnormalities", J Pediatr, 147, pp.823~829 (2005)
- Sedky K, "Prader Willi syndrome and obstructive sleep apnea : co-occurrence in the pediatric population", J Clin Sleep Med, 10, pp.403~409 (2014)
- Polytarchou A, "Obstructive events in children with Prader-Willi syndrome occur predominantly during rapid eye movement sleep", Sleep Med, 54, pp.43~47 (2019)
- Miller JL, "Sleep disordered breathing in infants with Prader-Willi syndrome during the first 6 weeks of growth hormone therapy : a pilot study", J Clin Sleep Med, 5, pp.448~453 (2009)
- Cohen M, "Clinically important age-related differences in sleep related disordered breathing in infants and children with Prader-Willi Syndrome", PLoS One, 9, pp.e101012~ (2014)
- Gillett ES, "Disorders of sleep and ventilatory control in Prader-Willi syndrome", Diseases, 4, pp.23~ (2016)
- Pavone M, "Sleep disordered breathing in patients with Prader-Willi syndrome : a multicenter study", Pediatr Pulmonol, 50, pp.1354~1359 (2015)
- Li AM, "Epidemiology of obstructive sleep apnoea syndrome in Chinese children : a two-phase community study", Thorax, 65, pp.991~997 (2010)
- Scammon RE, "The measurement of man" (1930), University of Minnesota Press
- Ishida T, "Patterns of adenoid and tonsil growth in Japanese children and adolescents : a longitudinal study", Sci Rep, 8, pp.17088~ (2018)
- Kothare SV, "Quality measures for the care of pediatric patients with obstructive sleep apnea", J Clin Sleep Med, 11, pp.385~404 (2015)
- Lindgren AC, "Growth hormone treatment increases CO(2)response, ventilation and central inspiratory drive in children with Prader-Willi syndrome", Eur J Pediatr, 158, pp.936~940 (1999)
- Miller J, "Short-term effects of growth hormone on sleep abnormalities in Prader-Willi syndrome", J Clin Endocrinol Metab, 91, pp.413~417 (2006)