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Obstructive Sleep Apnea Syndrome in Children with Mucopolysaccharidoses II (Hunter Syndrome)

DOI: 10.4236/oalib.1101630    577 Downloads   838 Views  

ABSTRACT

In order to assess the prevalence and dynamics of changes of basic parameters of obstructive sleep apnea syndrome (OSAS) at mucopolysaccharidoses II (MSP II) cardiorespiratory monitoring was performed for 17 children. Slight OSAS (apnoea-hypopnoea index (AHI) was 1.5 - 5) was diagnosed in 4 patients (23.5%), moderate OSAS (AHI was 5 - 10)—in 4 patients (23.5%), severe OSAS (AHI was higher than 10)—in 2 patients (11.8%). AHI median at Hunter syndrome was 5.3 ± 6.9/ hour. In the group of infants (1 - 3 years old) slight OSAS (AHI is 0.8 ± 0.3/h) dominated, whereas in the group of teenagers—sever OSAS (AHI is 10.9 ± 9.4/h), a median of blood saturation with oxygen (SpO2) was 87.5 ± 10.6%, desaturation index (DI)—10.4 ± 13.3/hour. On the whole, OSAS was diagnosed in 58.8% of children and increased with increasing of the disease severity. Thus cardiorespiratory monitoring is necessary for revealing children with moderate and moderate-to-severe OSAS, followed by prevention of life-threatening conditions, the occurrence of which is possible at this syndrome.

Conflicts of Interest

The authors declare no conflicts of interest.

Cite this paper

Baranov, A. , Namazova-Baranova, L. , Vashakmadze, N. , Gevorkyan, A. , Kuzenkova, L. , Podkletnova, T. , Kozhevnicova, O. and Altunin, B. (2015) Obstructive Sleep Apnea Syndrome in Children with Mucopolysaccharidoses II (Hunter Syndrome). Open Access Library Journal, 2, 1-8. doi: 10.4236/oalib.1101630.

References

[1] Muhlebach, M.S., Wooten, W. and Muenzer, J. (2011) Respiratory Manifestations in Mucopolysaccharidoses. Paediatric Respiratory Reviews, 12, 133-138.
http://dx.doi.org/10.1016/j.prrv.2010.10.005
[2] Semenza, G.L. and Pyeritz, R.E. (1988) Respiratory Complications of Mucopolysaccharide Storage Disorders. Medicine (Baltimore), 67, 209-219.
http://dx.doi.org/10.1097/00005792-198807000-00002
[3] Nashed, A., Al-Saleh, S., Gibbons, J., et al. (2009) Sleep-Related Breathing in Children with Mucopolysaccharidosis. Journal of Inherited Metabolic Disease, 32, 544-550.
http://dx.doi.org/10.1007/s10545-009-1170-4
[4] Martin, R., Beck, M., Eng, C., Giurliani, R., Harmatz, P., et al. (2008) Recognition and Diagnosis of Mucopolysaccharidosis II (Hunter Syndrome). Pediatrics, 121, e377-e386.
http://dx.doi.org/10.1542/peds.2007-1350
[5] Muenzer, J., Beck, M., Eng, C.M, et al. (2011) Long-Term, Open-Labeled Extension Study of Idursulfase in the Treatment of Hunter Syndrome. Genetics in Medicine, 13, 95-101.
http://dx.doi.org/10.1097/GIM.0b013e3181fea459
[6] Muenzer, J. (2011) Overview of the Mucopolysaccharidoses. Rheumatology (Oxford), 50, v4-v12.
http://dx.doi.org/10.1093/rheumatology/ker394
[7] Muenzer, J., Beck, M., Eng, C., et al. (2009) Multidisciplinary Management of Hunter Syndrome. Pediatrics, 124, 1228-1239.
http://dx.doi.org/10.1542/peds.2008-0999
[8] Shih, S.L., Lee, Y.J., Lin, S.P., Sheu, C.Y. and Blickman, J.G. (2002) Airway Changes in Children with Mucopolysaccharidoses. Acta Radiologica, 43, 40-43.
http://dx.doi.org/10.1034/j.1600-0455.2002.430108.x
[9] Giugliani, R., Harmatz, P. and Wraith, J.E. (2007) Management Guidelines for Mucopolysaccharidosis VI. Pediatrics, 120, 405-418.
http://dx.doi.org/10.1542/peds.2006-2184
[10] Leighton, S.E.J., Papsin, B., Vellodi, A., Dinwiddie, R. and Lane, R. (2001) Disordered Breathing during Sleep in Patients with Mucopolysaccharidoses. International Journal of Pediatric Otorhinolaryngology, 58, 127-138.
http://dx.doi.org/10.1016/S0165-5876(01)00417-7
[11] Leboulanger, N., Louis, B., Vialle, R., et al. (2011) Analysis of the Upper Airway by the Acoustic Reflection Method in Children with Mucopolysaccharidosis. Pediatric Pulmonology, 46, 587-594.
http://dx.doi.org/10.1002/ppul.21409
[12] Berger, K.I., Fagondes, S.C., Giugliani, R., et al. (2013) Respiratory and Sleep Disorders in Mucopolysaccharidoses. Journal of Inherited Metabolic Disease, 36, 201-210.
http://dx.doi.org/10.1007/s10545-012-9555-1
[13] Lin, H.Y., Chen, M.R., Lin, C.C., et al. (2010) Polysomnographic Characteristics in Patients with Mucopolysaccharidoses. Pediatric Pulmonology, 45, 1205-1212.
http://dx.doi.org/10.1002/ppul.21309
[14] Kamin, W. (2008) Diagnosis and Management of Respiratory Involvement in Hunter Syndrome. Acta Paediatrica Supplement, 97, 57-60.
http://dx.doi.org/10.1111/j.1651-2227.2008.00650.x
[15] Jeong, H.S., Cho, D.Y., Ahn, K.M. and Jin, D.K. (2006) Complications of Tracheotomy in Patients with Mucopolysaccharidoses Type II (Hunter Syndrome). International Journal of Pediatric Otorhinolaryngology, 70, 1765-1769.
http://dx.doi.org/10.1016/j.ijporl.2006.05.021
[16] Ginzburg, A.S., Önal, E., Aronson, R.M., Schild, J.A., Mafee, M.F. and Lopata, M. (1990) Successful Use of Nasal-CPAP for Obstructive Sleep Apnea in Hunter Syndrome with Diffuse Airway Involvement. Chest, 97, 1496-1498.
http://dx.doi.org/10.1378/chest.97.6.1496
[17] Yeung, A.H., Cowan, M.J., Horn, B. and Rosbe, K.W. (2009) Airway Management in Children with Mucopolysaccharidoses. Archives of Otolaryngology—Head Neck Surgery, 135, 73-79.
http://dx.doi.org/10.1001/archoto.2008.515

  
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