Preview

Pediatric pharmacology

Advanced search

OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN WITH TYPE II MUCOPOLYSACCHARIDOSIS (HUNTER SYNDROME)

https://doi.org/10.15690/pf.v10i6.900

Abstract

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.


About the Authors

N. D. Vashakmadze
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation
MD, head of the medical rehabilitation department for children with cardiovascular diseases at the research institute of PP and MR of the SCCH


L. S. Namazova-Baranova
Scientific 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, Moscow
Russian Federation


A. K. Gevorkyan
Scientific Center of Children’s Health, Moscow, Russian Federation First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation
Russian Federation


V. V. Altunin
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation


L. M. Kuzenkova
Scientific Center of Children’s Health, Moscow, Russian Federation First Sechenov Moscow State Medical University of the Ministry of Health of the Russian Federation
Russian Federation


E. G. Chernavina
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation


M. A. Babaykina
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation


T. V. Podkletnova
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation


O. V. Kozhevnikova
Scientific Center of Children’s Health, Moscow, Russian Federation
Russian Federation


References

1. 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.

2. 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.

3. Muenzer J. Overview of the mucopolysaccharidoses. Rheumatol. 2011; 50 (Suppl. 5): v4–v12.

4. Muenzer J., Beck M., Eng C. et al. Multidisciplinary management of hunter syndrome. Pediatrics. 2009; 124 (6): 1228–1239.

5. 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.

6. 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.

7. 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.

8. 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.

9. Kamin W. Diagnisis and management of respiratory involment in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.

10. 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.

11. 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.

12. Lin H. Y., Chen M. R., Lin C. C. et al. Polysomnographic characteristics in patients with mucopolysaccharidoses. Pediatr. Pulmonol. 2010; 45: 1205–1212.

13. Kamin W. Diagnosis and management of respiratory involvement in Hunter syndrome. Acta Paediatr. 2008; 97 (Suppl.): 57–60.

14. Dempsey J. A., Veasey S. C., Morgan B. J., O’Donnell C. P. Pathophysiology of sleep apnea. Physiol. Rev. 2010; 90: 47–112.

15. 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.

16. 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.

17. 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.


Review

For citations:


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. OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN WITH TYPE II MUCOPOLYSACCHARIDOSIS (HUNTER SYNDROME). Pediatric pharmacology. 2013;10(6):76-81. https://doi.org/10.15690/pf.v10i6.900

Views: 724


ISSN 1727-5776 (Print)
ISSN 2500-3089 (Online)