ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v10i6.900ppharm-161Research ArticleРЕДКИЕ БОЛЕЗНИRARE DISEASESСИНДРОМ ОБСТРУКТИВНОГО АПНОЭ ВО СНЕ У ДЕТЕЙ С МУКОПОЛИСАХАРИДОЗОМ II ТИПА (СИНДРОМОМ ХАНТЕРА)OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN WITH TYPE II MUCOPOLYSACCHARIDOSIS (HUNTER SYNDROME)ВашакмадзеН. Д.VashakmadzeN. D.

MD, head of the medical rehabilitation department for children with cardiovascular diseases at the research institute of PP and MR of the SCCH

vashakmadze@nczd.ruНамазова-БарановаЛ. С.Namazova-BaranovaL. S.

кандидат медицинских наук, заведующая отделением восстановительного лечения детей с сердечно-сосудистыми заболеваниями НИИ ПП и ВЛ НЦЗД

ГеворкянА. К.GevorkyanA. K.АлтунинВ. В.AltuninV. V.КузенковаЛ. М.KuzenkovaL. M.ЧернавинаЕ. Г.ChernavinaE. G.БабайкинаМ. А.BabaykinaM. A.ПодклетноваТ. В.PodkletnovaT. V.КожевниковаО. В.KozhevnikovaO. V.Научный центр здоровья детей РАМН, Москва, Российская ФедерацияРоссияScientific Center of Children’s Health, Moscow, Russian FederationRussian FederationНаучный центр здоровья детей РАМН, Москва, Российская Федерация Первый Московский государственный медицинский университет им. И.М. Сеченова Минздрава Российской Федерации Российский национальный исследовательский медицинский университет им. Н.И. Пирогова, МоскваРоссияScientific Center of Children’s Health, Moscow, Russian Federation First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation Pirogov Russian National Medical Research University, MoscowRussian FederationНаучный центр здоровья детей РАМН, Москва, Российская Федерация Первый Московский государственный медицинский университет им. И.М. Сеченова Минздрава Российской ФедерацииРоссияScientific Center of Children’s Health, Moscow, Russian Federation First Sechenov Moscow State Medical University of the Ministry of Health of the Russian FederationRussian Federation201302122013106Педиатрическая фармакология7681Copyright © Вашакмадзе Н.Д., Намазова-Баранова Л.С., Геворкян А.К., Алтунин В.В., Кузенкова Л.М., Чернавина Е.Г., Бабайкина М.А., Подклетнова Т.В., Кожевникова О.В., 20132013Вашакмадзе Н.Д., Намазова-Баранова Л.С., Геворкян А.К., Алтунин В.В., Кузенкова Л.М., Чернавина Е.Г., Бабайкина М.А., Подклетнова Т.В., Кожевникова О.В.Vashakmadze N.D., Namazova-Baranova L.S., Gevorkyan A.K., Altunin V.V., Kuzenkova L.M., Chernavina E.G., Babaykina M.A., Podkletnova T.V., Kozhevnikova O.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pedpharma.ru/jour/article/view/161

17 children received cardiorespiratory monitoring in order to evaluate spread and dynamics of changes in the primary obstructive sleep apnea syndrome (OSAS) parameters at type II mucopolysaccharidosis. Mild OSAS [apnea/hypopnea index (AHI) – 1.5-5] was diagnosed in 4 patients (23.5%), moderate OSAS (AHI – 5-10) – in 4 patients (23.5%), severe OSAS (AHI>10) – in 2 patients (11.8%). Average AHI at Hunter syndrome was 5.3±6.9/hour. Mild OSAS (AHI – 0.8±0.3/hour) was prevalent in the group of younger children (1-3 years of age); severe OSAS was prevalent in the group of adolescents (AHI – 10.9±9.4/hour); average blood oxygen saturation (SpO2) was 87.5±10.6%, desaturation index – 10.4±13.3/hour. In total, OSAS was observed in 58.8% of children and aggravated in direct proportion to aggravation of the disease course. Thus, cardiorespiratory monitoring is necessary to reveal children with moderate and severe OSAS course with subsequent prevention of life-threatening conditions, which may appear at this syndrome.

синдром обструктивного апноэ во снесидром Хантеракардиореспираторный мониторингдетиobstructive sleep apnea syndromeHunter syndromecardiorespiratory monitoringchildrenReferencesMartin R., Beck M., Eng C., Giurliani R., Harmatz P. et al. Recognition and diagnosis of mucopolysaccharidosis II (Hunter syndrome). Pediatrics. 2008; 121: e377–e386.Martin R., Beck M., Eng C., Giurliani R., Harmatz P. et al. Recognition and diagnosis of mucopolysaccharidosis II (Hunter syndrome). Pediatrics. 2008; 121: e377–e386.Muenzer J., Beck M., Eng C. M. et al. Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome. Genet Med. 2011; 13:95–101.Muenzer J., Beck M., Eng C. M. et al. Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome. Genet Med. 2011; 13: 95–101.Muenzer J. Overview of the mucopolysaccharidoses. Rheumatol. 2011; 50 (Suppl. 5): v4–v12.Muenzer J. Overview of the mucopolysaccharidoses. Rheumatol. 2011; 50 (Suppl. 5): v4–v12.Muenzer J., Beck M., Eng C. et al. Multidisciplinary management of hunter syndrome. Pediatrics. 2009; 124 (6): 1228–1239.Muenzer J., Beck M., Eng C. et al. Multidisciplinary management of hunter syndrome. Pediatrics. 2009; 124 (6): 1228–1239.Вашакмадзе Н. Д., Намазова-Баранова Л. С., Геворкян А. К., Кузенкова Л. М., Христочевский А. Д., Высоцкая Л. М., Дада шев А. С. Мукополисахаридоз II типа. Педиатрическая фармакология. 2011; 8 (3): 66–68.Vashakmadze N. D., Namazova-Baranova L. S., Gevorkyan A. K., Kuzenkova L. M., Khristochevskii A. D., Vysotskaya L. M., Dadashev A. S. Pediatricheskaya farmakologiya = Pediatric pharmacology. 2011; 8 (3): 66–68.Shih S. L., Lee Y. J., Lin S. P., Sheu C. Y., Blickman J. G. Airway changes in children with mucopolysaccharidoses. Acta Radiol. 2002; 43: 40–43.Shih S. L., Lee Y. J., Lin S. P., Sheu C. Y., Blickman J. G. Airway changes in children with mucopolysaccharidoses. Acta Radiol. 2002; 43: 40–43.Namazova-Baranova L., Gevorkyan A., Polunina T., Vashaкmadze N., Toropchina L., Gubanova S., Zelenkova I. The condition of hearing in patients with varying types of mucopolysaccharidosis (MPS). Abstracts for the Lysosomal Disease Network's WORLD Symposium Molecular Genetics and Metabolism. 108 (2013). S. 60.Namazova-Baranova L., Gevorkyan A., Polunina T., Vashaкmadze N., Toropchina L., Gubanova S., Zelenkova I. The condition of hearing in patients with varying types of mucopolysaccharidosis (MPS). Abstracts for the Lysosomal Disease Network's WORLD Symposium Molecular Genetics and Metabolism. 108 (2013). p. 60.Leboulanger N., Louis B., Vialle R. et al. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis. Pediatr Pulmonol. 2011; 46: 587–594.Leboulanger N., Louis B., Vialle R. et al. Analysis of the upper airway by the acoustic reflection method in children with mucopolysaccharidosis. Pediatr. Pulmonol. 2011; 46: 587–594.Kamin W. Diagnisis and management of respiratory involment in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.Kamin W. Diagnisis and management of respiratory involment in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.Баранов А. А., Намазова-Баранова Л. С., Геворкян А. К., Краснов В. М., Кузенкова Л. М., Каркашадзе Г. А., Вашакмадзе Н. Д., Подклетнова Т. В. Опыт внедрения в российскую педиатрическую практику новой технологии лечения детей, страдающих мукополисахаридозами. Педиатрическая фармакология. 2011; 8 (5): 5–12.Baranov A. A., Namazova-Baranova L. S., Gevorkyan A. K., Krasnov V. M., Kuzenkova L. M., Karkashadze G. A., Vashakmadze N. D., Podkletnova T. V. Pediatricheskaya farmakologiya = Pediatric pharmacology. 2011; 8 (5): 5–12.Leighton S. E. J., Papsin B., Vellodi A., Dinwiddie R., Lane R. Disordered breathing during sleep in patients with mucopolysaccharidoses. Int J Pediatr Otorhinolaryngol. 2001; 58: 127–138.Leighton S. E. J., Papsin B., Vellodi A., Dinwiddie R., Lane R. Disordered breathing during sleep in patients with mucopolysaccharidoses. Int J Pediatr Otorhinolaryngol. 2001; 58: 127–138.Lin H. Y., Chen M. R., Lin C. C. et al. Polysomnographic characteristics in patients with mucopolysaccharidoses. Pediatr Pulmonol. 2010; 45: 1205–1212.Lin H. Y., Chen M. R., Lin C. C. et al. Polysomnographic characteristics in patients with mucopolysaccharidoses. Pediatr. Pulmonol. 2010; 45: 1205–1212.Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.Dempsey J. A., Veasey S. C., Morgan B. J., O’Donnell C. P. Pathophysiology of sleep apnea. Physiol Rev. 2010; 90: 47–112.Dempsey J. A., Veasey S. C., Morgan B. J., O’Donnell C. P. Pathophysiology of sleep apnea. Physiol. Rev. 2010; 90: 47–112.Jeong H. S., Cho D. Y., Ahn K. M., Jin D. K. Complications of tracheotomy in patients with mucopolysaccharidoses type II (Hunter syndrome). Int J Pediatr Otorhinolaryngol. 2006; 70: 1765–1769.Jeong H. S., Cho D. Y., Ahn K. M., Jin D. K. Complications of tracheotomy in patients with mucopolysaccharidoses type II (Hunter syndrome). Int. J. Pediatr. Otorhinolaryngol. 2006; 70: 1765–1769.Ginzburg A. S., Onal E., Aronson R. M., Schild J. A., Mafee M. F., Lopata M. Successful use of nasal-CPAP for obstructive sleep apnea in Hunter syndrome with diffuse airway involvement. Chest. 1990; 97: 1496–1498.Ginzburg A. S., Onal E., Aronson R. M., Schild J. A., Mafee M. F., Lopata M. Successful use of nasal-CPAP for obstructive sleep apnea in Hunter syndrome with diffuse airway involvement. Chest. 1990; 97: 1496–1498.Yeung A. H., Cowan M. J., Horn B., Rosbe K. W. Airway management in children with mucopolysaccharidoses. Arch Otolaryngol Head Neck Surg. 2009; 135: 73–79.Yeung A. H., Cowan M. J., Horn B., Rosbe K. W. Airway management in children with mucopolysaccharidoses. Arch. Otolaryngol. Head Neck Surg. 2009; 135: 73–79.

The authors declare that there are no conflicts of interest present.