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

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.

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