The classic presentation is recurrent otitis media, chronic sinusitis, and bronchiectasis. Kartagener syndrome, the triad of situs inversus, pansinusitis, and bronchiectasis, accounts for approximately 50% of cases. Males are infertile as a result of immotile sperm. Because the cilia fail to beat normally, secretions accumulate in the airways, and endobronchial infection results. Chronic infection, if untreated, leads to bronchiectasis by early adulthood. Primary ciliary dyskinesia should be suspected in patients with early-onset chronic bronchitis or bronchiectasis associated with recurrent/persistent sinusitis and/or frequent otitis media.
PCD is confirmed by electron microscopy of respiratorycilia, obtained from scrapings/biopsy of nasal or airwayepithelium. Results may be difficult to interpret as chronic infection and inflammation may lead to ultrastructuralabnormalities in nasal cilia. The measurement of nasal nitric oxide has been used as a screening tool for PCD. Low nasalnitric oxide values (<200 parts per billion) are consistentwith PCD, whereas values greater than 400 parts per billionmake the diagnosis less likely. However, the validity of nasalnitric oxide assays as a diagnostic test for PCD has not beenfully substantiated.
Treatment is geared toward treating infections and improving clearance of respiratory secretions. High resolution chestCT scans are useful to confirm and monitor bronchiectasis. Surveillance cultures help identify organisms involved and guide antibiotic therapy. Sinus surgical procedures are often done to manage chronic sinusitis, but their benefit is questionable. Most children require placement of pressure equalization (PE) tubes for management of recurrent otitis media.Chest physiotherapy and prompt treatment of bacterialinfections are helpful, but the course of the disease tends tobe slowly progressive. Preventive antibiotics, such as thrice-weekly macrolides, may reduce the number of exacerbations per year.
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SEE CHAPTER 110.
Pulmonary edema is the seepage of fluid into the alveolar and interstitial spaces. Capillary hydrostatic forces and interstitial osmotic pressures tend to push fluid into the airspaces, whereas plasma osmotic pressures and tissue mechanical forces tend to move fluid away from the airspaces. Under normal circumstances, the sum of these forces favors absorption, so the alveolar and interstitial spaces remain dry. Fluid entering the alveoli is normally removed by pulmonary lymphatics. Pulmonary edema forms when transcapillary fluid flux exceeds lymphatic drainage. Reduced left ventricular function leads to pulmonary venous hypertension and increased capillary hydrostatic pressure, and fluid moves into the interstitial space and alveoli. Fluid initially enters the interstitial space around the terminal bronchioles, alveoli, and arterioles (interstitial edema), causing increased lung stiffness and premature closure of bronchioles on expiration. If the process continues, fluid then enters the alveoli, further reducing compliance and resulting in intrapulmonary shunting (alveolar units that are perfused but not ventilated). Decreased ventilation (hypercarbia) is a late finding.