Lower airway microbiota in children with Down syndrome compared to controls with similar respiratory symptomatology.

BACKGROUND: Children with Down syndrome (DS) often present with chronic or recurrent respiratory symptoms and generally have a more severe and prolonged disease course in case of infection. This can be caused by anatomical and/or immunological predisposition. With this study, we aim to compare microbial composition in the lower airways of patients with DS versus controls, to see if we can explain the difference in disease course. METHODS: All endoscopic procedures under general anesthesia in patients with DS were reviewed retrospectively. We compared the microbiological data from bronchoalveolar lavage fluid (BALF) cultures (when available) to a cohort of children with chronic respiratory symptoms but without any other relevant medical history. RESULTS: Endoscopic data were available for 65 DS patients, BAL cultures for 47 out of 65 patients (72%). The "control" group consisted of 150 children without significant underlying disease, who were matched for age and sex. BAL culture results were available for 135 out of 150 patients (90%). Microorganisms were categorized and compared between both groups, with no statistical differences. Among the microorganisms tested, the most frequently reported were typical bacteria such as Haemophilus influenzae, Moraxella catarrhalis, Streptococci and Staphylococci. CONCLUSIONS: No significant differences in lower airways microbial composition of children with DS and chronic respiratory symptoms were found when compared to controls presenting similar symptomatology. A suggestion for future research may be to investigate possible differences in drug sensitivity.

Obstructive Sleep Disorders in Down Syndrome's Children with and without Lower Airway Anomalies.

(1) Background: Obstructive sleep apnea (OSA) and lower airway anomalies are both highly prevalent in children with Down syndrome (DS). However, little is known on the interaction between both. We aim to investigate the co-occurrence of OSA (defined as obstructive apnea/hypopnea index (oAHI) ≥ 2/h) and lower airway anomalies in children with DS and explore their impact on OSA severity and treatment outcome. (2) Methods: Retrospective analysis of data from airway endoscopy and polysomnography (PSG) in a cohort of children with DS. (3) Results: Data on both lower airway evaluation and PSG were available for 70 patients with DS. Our study population was relatively young (mean age 3.5 years), not obese and presented with severe OSA (mean oAHI 13.1/h). Airway anomalies were found in 49/70 children (70%), most frequently laryngomalacia, tracheomalacia or a combined airway malformation. In the remaining 21 cases (30%), endoscopy was normal. A comparison between both groups showed a similar distribution of gender, age and BMI z-scores. The prevalence of OSA was not significantly higher in DS patients with airway anomalies (89.6% vs 71.4%, p = 0.078). Additionally, OSA severity or treatment choice (conservative, upper airway surgery or CPAP) were not significantly different. Follow-up data (available for 49/70 patients) showed a significant improvement of OSA in both groups. There is a not significant tendency to more patients with persistent OSA among those with lower airway anomalies (34.3% vs 7.1%, p = 0.075). (4) Conclusions: We found no significant differences in OSA severity, treatment choice or outcome between children with DS with and without lower airway anomalies. Further studies should investigate the role of DISE-directed treatment and compare the outcome of different treatment modalities in larger patient groups.

Pulmonary complications in children with Down syndrome: A scoping review.

CONTEXT: Down syndrome (DS) is a prevalent chromosomal disorder associated with a wide range of congenital anomalies and other health problems. OBJECTIVES: To give a scoping overview of encountered lower airway problems (both infectious and non-infectious) in DS children. DATA SOURCES: We systematically searched the MEDLINE and PubMed databases for relevant publications. STUDY SELECTION: Studies were eligible if they were original studies about pediatric airway problems in DS and were evaluated by the PRISMA guidelines. DATA EXTRACTION: Data concerning patient characteristics, study methods and outcomes were critically reviewed. RESULTS: Sixty papers were included. These were reviewed and summarized by topic, i.e. airway anomalies, dysphagia and aspiration, lower respiratory tract infections (and bronchiolitis in particular), pulmonary hypertension and other. Respiratory problems are proven to be a frequent and a major health burden in DS children. Airway anomalies (both single and multiple) are more prevalent and require a specific approach. A large proportion of DS children have (often silent) aspiration, resulting in protracted and difficult-to-treat symptoms. Respiratory tract infections are usually more severe and associated with an increased need for (prolonged) hospitalization. Pulmonary hypertension, wheeze and some other rare conditions are more commonly encountered in DS. LIMITATIONS: Large number of studies and high levels of study heterogeneity. CONCLUSIONS: Several lower airway problems are more frequent and more complex in children with DS. These findings emphasize the need for a multidisciplinary approach by an experienced team allowing for a prompt diagnosis, proper management and improved long term outcome.

The prevalence of lower airway anomalies in children with Down syndrome compared to controls.

INTRODUCTION: Children with Down syndrome (DS) often present with chronic respiratory symptoms. Congenital airway anomalies have been described but data about prevalence is scarce and a comparison to controls is lacking. We aim to compare the endoscopic and clinical data of children with DS to controls without significant medical history. METHODS: All endoscopic procedures under general anesthesia (broncho- and/or direct laryngoscopy) in patients with DS were reviewed. We compared clinical and endoscopic data to a cohort of children with respiratory symptoms but without any other relevant medical history. RESULTS: Endoscopic data were available for 65 patients with DS. The median age was 2.9 years (range: 0.2-17), 63% were boys. The most common clinical presentation was recurrent respiratory infections (37%). Other major symptoms were chronic cough and/or noisy breathing (23%) and stridor (20%). Endoscopy was normal in 29% of patients. The largest group of patients (44%) had some form of airway malacia. Tracheal bronchus and subglottic stenosis were each isolated findings in 3.1% of patients. Twenty percent presented with combined airway anomalies. The control group consisted of 150 children (matched for age and sex) without significant underlying disease. The most common presentations were chronic cough and/or noisy breathing (29%), persistent radiographic abnormalities (20%), and suspicion of aspiration of a foreign body (15%). In the majority of controls (68%), no airway anomaly was found. Other findings were malacia (22%), tracheal bronchus (1%), and subglottic stenosis (1%). A combined anomaly was found in 5%. CONCLUSION: Congenital airway anomalies were seen in 71% of patients with DS, compared with 32% of controls. Combined anomalies are more frequent in DS. Complete lower airway endoscopy is recommended in patients with DS as it may influence therapeutic decision-making.