Grand RoundsPulmonary Complications of Down Syndrome during Childhood
Section snippets
Pulmonary Vascular Issues
The pulmonary vasculature of children with DS demonstrates vulnerability that may manifest clinically as pulmonary hypertension, pulmonary edema, or pulmonary hemorrhage. Some, but not all, published studies identify DS as a risk factor for development of pulmonary vascular disease.6, 7, 8, 9 Children with DS with increased pulmonary blood flow because of an intracardiac right-to-left shunt may have precocious development of pulmonary artery hypertension (PAH). Significant clinical variability
Parenchymal Lung Disease
Diffuse parenchymal lung disease manifests in children with DS as chronic radiographic changes associated with persistent findings such as dyspnea, cough, wheezing, crackles, or hypoxia. It is occasionally detected incidentally in individuals without symptoms who are undergoing imaging for other reasons. Diffuse parenchymal abnormalities may develop as a primary process, which includes conditions such as pulmonary hypoplasia, pulmonary lymphangiectasia, lymphoid interstitial pneumonitis, and
Sleep-Disordered Breathing and Associated Complications
Sleep-disordered breathing is the most common respiratory disorder affecting children with DS. The prevalence of sleep-disordered breathing equals that of cardiac, ear, and visual disorders in DS combined and far exceeds the risk of hypothyroidism (15%), which is widely recognized to be associated with DS. OSA affects children with DS of all ages, with the prevalence reported to range from 30% to 75%, compared with 2% of the general pediatric population.10, 27, 28, 29 Because children with DS
Airway Abnormalities
A number of upper airway abnormalities characteristic for DS have been described by multiple authors.27, 28, 29, 30, 32, 34, 35, 36, 37, 38 Lower airway anomalies such as tracheobronchomalacia, subglottic stenosis (SGS), or tracheal stenosis are also common and occur in approximately 25% of children with DS.35, 36, 38 In addition, children with DS often have co-morbid conditions of increased oropharyngeal secretions, hypotonia, and, in older children, obesity. These structural and functional
Asthma
Recently published data from a health survey compared 146 children with DS aged 3 to 17 years with 95 control subjects for a variety of health conditions.47 The caregiver-reported rate of “previous asthma” (19.4% versus 13.2%) and “asthma attack in the preceding 12 months” (8.7% versus 5.8%) was higher in children with DS, but did not reach statistical significance. However, multiple earlier studies have reported decreased asthma prevalence in children with DS.48, 49, 50 Two Australian studies,
Infection, Immune Compromise, and Other Respiratory Illnesses
Children with trisomy-21 are at increased risk of respiratory tract infections and are more likely than children without DS to have a severe course and even death from respiratory causes. CHD was only half as likely as respiratory disease to be the admitting diagnosis (44/197 versus 83/197 admissions).50 In the subgroup of patients with DS who had surgery, risk of respiratory infection was approximately 3 times higher than for children with DS who did not have surgery. DS with CHD or
Summary
The pulmonary problems often seen in children with DS include recurrent and more severe respiratory tract infections, airway abnormalities, pulmonary vascular disease, cystic lung disease, and sleep apnea. Common conditions associated with DS such as hypotonia, dysphagia, and immune abnormalities may all contribute to recurrent respiratory issues, thus evaluation for these problems needs to be considered in the assessment and treatment of children with DS with frequent or persistent respiratory
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The authors declare no conflicts of interest.