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Polysomnographic Analysis of Sleep-Disordered Breathing In Children With Mucopolysaccharidoses in Qatar: A
Nadine Asir1, Amal R Al-Naimi2
1Pediatric Pulmonology, Sidra Medicine, Doha, QAT.
Insights
Sleep-disordered breathing (SDB) affects most pediatric mucopolysaccharidoses (MPS) patients in Qatar, with obstructive sleep apnea (OSA) being highly prevalent and often severe. Early screening and intervention are crucial for managing this critical complication.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Sleep Medicine
Background:
- Sleep-disordered breathing (SDB) is a major complication in mucopolysaccharidoses (MPS), affecting 80-90% globally.
- Pathophysiology involves airway tissue changes and skeletal alterations due to glycosaminoglycan deposition.
- SDB prevalence and characteristics in Qatar's MPS population were previously unknown.
Purpose of the Study:
- To determine the prevalence and characteristics of SDB in pediatric MPS patients in Qatar.
- To highlight the need for polysomnographic screening and early intervention in this population.
Main Methods:
- Retrospective chart review of 14 pediatric MPS patients (aged 1-18) who underwent polysomnography (PSG) between September 2019 and July 2023.
- Collected and summarized clinical and PSG data.
Main Results:
- Obstructive sleep apnea (OSA) prevalence was 78.57% (11/14), with 45.45% being severe.
- Mean obstructive apnea-hypopnea index (OAHI) was 13.48 events/hour.
- Interventions included adenotonsillectomy (8/14) and non-invasive positive airway pressure (6/14); no tracheostomies were required.
Conclusions:
- This study reveals a high prevalence and severity of SDB in Qatar's pediatric MPS population, comparable to international data.
- Emphasizes the critical need for polysomnographic screening and prompt therapeutic interventions.
- Recommends future studies on treatment outcomes and genotype-phenotype correlations.
Introduction:
Sleep-disordered breathing (SDB) represents a critical complication in mucopolysaccharidoses (MPSs), with a reported global prevalence rate of 80%-90%. The multifactorial pathophysiology involves glycosaminoglycan deposition in upper airway tissues and complex skeletal alterations. Although SDB is frequently encountered among MPS patients, details on its prevalence and characteristics remain unknown in Qatar. This study highlights different aspects of SDB in our population.
Methods:
A retrospective chart review study was conducted on patients with a confirmed diagnosis of MPS aged one to 18 between September 2019 and July 2023 who underwent polysomnography (PSG) study at Sidra Medicine (Doha, Qatar). Clinical and PSG data were collected and summarized.
Results:
The cohort comprised 14 patients (50% male; mean age 8.2 years (range: 1.42-18.8); mean BMI 21.63 kg/m2 (range: 13.47-42.1)). Obstructive sleep apnea (OSA) prevalence was 78.57% (11/14), with severity distribution as follows: mild (27.27%), moderate (27.27%), and severe (45.45%). Mean obstructive apnea-hypopnea index (OAHI) was 13.48 events/hour. Therapeutic interventions included adenotonsillectomy (eight of 14) and non-invasive positive airway pressure support (six of 14). None of the patients required tracheostomy.
Conclusion:
This first comprehensive analysis of SDB in Qatar's pediatric MPS population reveals high prevalence and severity rates comparable to international cohorts. These results emphasize the crucial need for polysomnographic screening and early therapeutic intervention in this population. Future prospective studies should evaluate short and long-term treatment outcomes and explore potential genotype-phenotype correlations in this demographically distinct cohort.
