Allergy, Asthma & Respiratory Disease

  • 제12권2호
  • /
  • Pages.93-98
  • /
  • 2024
  • /
  • 2288-0402(pISSN)
  • /
  • 2288-0410(eISSN)
대한천식알레르기학회 (The Korean Academy of Asthma, Allergy and Clinical Immunology)
권호 표지
DOI QR코드

DOI QR Code

급성호흡부전이 발생한 다운증후군 23세 여자에서 진단된 폐쇄성 수면무호흡증 1예

A 23-year-old female with Down syndrome accompanied by obstructive sleep apnea and acute respiratory failure

  • 이승재 (서울대학교 의과대학 소아청소년과) ;
  • 이주원 (서울대학교 의과대학 소아청소년과) ;
  • 김경훈 (서울대학교 의과대학 소아청소년과)
  • Seungjae Lee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Joowon Lee (Department of Pediatrics, Seoul National University College of Medicine) ;
  • Kyunghoon Kim (Department of Pediatrics, Seoul National University College of Medicine)
  • 투고 : 2023.10.11
  • 심사 : 2023.11.09
  • 발행 : 2024.04.30
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

This case report details a 23-year-old female with Down syndrome who suffered from acute respiratory failure due to severe obstructive sleep apnea syndrome and accompanying pulmonary arterial hypertension (PAH). The patient presented with obesity, adenotonsillar hypertrophy, and craniofacial anomalies commonly seen in Down syndrome, predisposing her to sleep-disordered breathing. Upon intensive care unit admission, she exhibited cardiomegaly, bilateral pulmonary edema, and right ventricular dysfunction. Polysomnography revealed severe sleep apnea with an apnea-hypopnea index of 108.7/hr. Treatment modalities included noninvasive positive pressure ventilation, diuretics, antibiotics, and positive airway pressure (PAP) devices to manage hypercapnia, pulmonary edema, and sleep apnea. PAH, a recognized complication of untreated sleep apnea, contributed to right ventricular dysfunction. A multidisciplinary approach was vital, with long-term management centered on continuous PAP therapy and comprehensive obesity management. This case underscores the intricate interplay between Down syndrome, sleep apnea, and PAH, highlighting the significance of early recognition and coordinated intervention in individuals with Down syndrome to enhance overall outcomes and quality of life.

키워드

참고문헌

  1. Lumeng JC, Chervin RD. Epidemiology of pediatric obstructive sleep apnea. Proc Am Thorac Soc 2008;5:242-52. https://doi.org/10.1513/pats.200708-135MG
  2. Capdevila OS, Kheirandish-Gozal L, Dayyat E, Gozal D. Pediatric obstructive sleep apnea: complications, management, and long-term outcomes. Proc Am Thorac Soc 2008;5:274-82. https://doi.org/10.1513/pats.200708-138MG
  3. Basil JS, Santoro SL, Martin LJ, Healy KW, Chini BA, Saal HM. Retrospective study of obesity in children with Down syndrome. J Pediatr 2016;173:143-8. https://doi.org/10.1016/j.jpeds.2016.02.046
  4. Trucco F, Chatwin M, Semple T, Rosenthal M, Bush A, Tan HL. Sleep disordered breathing and ventilatory support in children with Down syndrome. Pediatr Pulmonol 2018;53:1414-21. https://doi.org/10.1002/ppul.24122
  5. Adir Y, Humbert M, Chaouat A. Sleep-related breathing disorders and pulmonary hypertension. Eur Respir J 2021;57:2002258. https://doi.org/10.1183/13993003.02258-2020
  6. Hopper RK, Abman SH, Elia EG, Avitabile CM, Yung D, Mullen MP, et al. Pulmonary hypertension in children with Down syndrome: results from the pediatric pulmonary hypertension network registry. J Pediatr 2023;252:131-40. https://doi.org/10.1016/j.jpeds.2022.08.027
  7. Miguel-Diez J, Villa-Asensi JR, Alvarez-Sala JL. Prevalence of sleep-disordered breathing in children with Down syndrome: polygraphic findings in 108 children. Sleep 2003;26:1006-9. https://doi.org/10.1093/sleep/26.8.1006
  8. Trois MS, Capone GT, Lutz JA, Melendres MC, Schwartz AR, Collop NA, et al. Obstructive sleep apnea in adults with Down syndrome. J Clin Sleep Med 2009;5:317-23. https://doi.org/10.5664/jcsm.27541
  9. Bull MJ, Trotter T, Santoro SL, Christensen C, Grout RW; COUNCIL ON GENETICS, et al. Health supervision for children and adolescents with Down syndrome. Pediatrics 2022;149:e2022057010. https://doi.org/10.1542/peds.2022-057010
  10. Verhulst SL, Van Gaal L, De Backer W, Desager K. The prevalence, anatomical correlates and treatment of sleep-disordered breathing in obese children and adolescents. Sleep Med Rev 2008;12:339-46. https://doi.org/10.1016/j.smrv.2007.11.002
  11. Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. American Academy of Pediatrics. Clinical practice guideline: diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2002;109:704-12. https://doi.org/10.1542/peds.109.4.704
  12. Uliel S, Tauman R, Greenfeld M, Sivan Y. Normal polysomnographic respiratory values in children and adolescents. Chest 2004;125:872-8. https://doi.org/10.1378/chest.125.3.872
  13. Mitchell RB. Adenotonsillectomy for obstructive sleep apnea in children: outcome evaluated by pre- and postoperative polysomnography. Laryngoscope 2007;117:1844-54. https://doi.org/10.1097/MLG.0b013e318123ee56
  14. Bhattacharjee R, Kheirandish-Gozal L, Spruyt K, Mitchell RB, Promchiarak J, Simakajornboon N, et al. Adenotonsillectomy outcomes in treatment of obstructive sleep apnea in children: a multicenter retrospective study. Am J Respir Crit Care Med 2010;182:676-83. https://doi.org/10.1164/rccm.200912-1930OC
  15. Lal C, White DR, Joseph JE, van Bakergem K, LaRosa A. Sleep-disordered breathing in Down syndrome. Chest 2015;147:570-9. https://doi.org/10.1378/chest.14-0266
  16. Dudoignon B, Amaddeo A, Frapin A, Thierry B, de Sanctis L, Arroyo JO, et al. Obstructive sleep apnea in Down syndrome: benefits of surgery and noninvasive respiratory support. Am J Med Genet A 2017;173:2074-80. https://doi.org/10.1002/ajmg.a.38283
  17. Resta O, Guido P, Foschino Barbaro MP, Picca V, Talamo S, Lamorgese V. Sleep-related breathing disorders in acute respiratory failure assisted by non-invasive ventilatory treatment: utility of portable polysomnographic system. Respir Med 2000;94:128-34. https://doi.org/10.1053/rmed.1999.0682
  18. Buckle P, Pouliot Z, Millar T, Kerr P, Kryger MH. Polysomnography in acutely ill intensive care unit patients. Chest 1992;102:288-91. https://doi.org/10.1378/chest.102.1.288
  19. Minai OA, Ricaurte B, Kaw R, Hammel J, Mansour M, McCarthy K, et al. Frequency and impact of pulmonary hypertension in patients with obstructive sleep apnea syndrome. Am J Cardiol 2009;104:1300-6. https://doi.org/10.1016/j.amjcard.2009.06.048
  20. Arias MA, Garcia-Rio F, Alonso-Fernandez A, Martinez I, Villamor J. Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Eur Heart J 2006;27:1106-13. https://doi.org/10.1093/eurheartj/ehi807