TITLE:
Obstructive Sleep Apnea Syndrome in Children with Mucopolysaccharidoses II (Hunter Syndrome)
AUTHORS:
A. A. Baranov, L. S. Namazova-Baranova, N. D. Vashakmadze, A. K. Gevorkyan, L. M. Kuzenkova, T. V. Podkletnova, O. V. Kozhevnicova, B. B. Altunin
KEYWORDS:
Hunter Syndrome, Mucopolysaccharidoses, Cardiorespiratory Monitoring, Obstructive Sleep Apnea Syndrome
JOURNAL NAME:
Open Access Library Journal,
Vol.2 No.6,
June
29,
2015
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.