An 18-year-old female came to the emergency room with cough and problems “catching her breath” for 2 days. Symptoms started with clear rhinitis 2 days ago and yesterday she had a fever to 100.8° with increased cough and thickness of the nasal secretions. The cough was productive but not different than her normal persistent cough due to immotile cilia syndrome that was diagnosed as a preschool child. She also complained of facial and teeth pain for the past 12 hours. The review of systems was otherwise negative. The pertinent physical exam showed a relatively well-appearing female with a coarse cough, but no increased work of breathing. Her respiratory rate was 20, her oxygen saturation was 98% on room air, and she was afebrile. HEENT showed boggy swollen nasal turbinates with thick, yellow-green secretions. She had facial tenderness over the frontal and maxillary sinuses. She had post-nasal drip and no specific tooth pain with palpation. Lungs had mild wheezing that resolved with coughing. The diagnosis of clinical sinusitis was made, and the patient was placed on antibiotics. She had a health maintenance examination set-up in 2 weeks and was to followup then.
Wheezing is an adventitial breath sound caused by narrowing of the airway causing a musical, high-pitched, continuous sound. It occurs with expiration but as severity increases, it also occurs in inspiration. If caused by secretions then wheezing may disappear after a cough, and change in different parts of the lung fields. It is usually associated with intrathoracic processes (usually mid-trachea and below). Stridor is sometimes confused with wheezing but it occurs during inspiration and is usually caused by extrathoracic processes such as croup or vocal cord paralysis. An explanation of other adventitial breath sounds can be foundhere.
Immotile cilia syndrome is an autosomal recessive disease with abnormal ciliary motion and impaired ciliary clearance of mucous. Patients may have cough and wheezing even if they do not have problems such as a pulmonary infection. Other common problems include otitis media, sinusitis and male infertility.
The differential diagnosis of wheezing includes:
- Pulmonary (primarily)
- Bronchopulmonary dysplasia
- Cystic fibrosis
- Tracheo-, bronchial obstruction
- Tracheoesophageal fistula
- Tracheal web
- Tracheal stenosis
- Tumor – internal, e.g. cystic adenomatoid malformation
- Lobar emphysema
- Pulmonary hemosiderosis
- Pulmonary (secondarily)
- Allergic rhinitis
- Gastroesophageal reflux
- Foreign body
- Swallowing disorder
- Smoke inhalation
- Tobacco use
- Tumor – external, e.g. mediastinal tumors
- Drugs – salicylates, beta-blockers
- Congenital heart disease
- Pulmonary embolism
- Vascular ring
- Alpha-1-antitrypsin deficiency
- Immune deficiency
- Immotile cilia syndrome
- Infectious Disease
- Munchausen syndrome
Patients often present with a cough and on physical examination have wheezing, but one may be seen without the other. Their differential diagnoses also overlap. A differential diagnosis of cough can be found here.
Questions for Further Discussion
1. What are the criteria for the diagnosis of asthma?
2. What is the epidemiology and natural history of immotile cilia syndrome?
- Age: Teenager
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Illingworth RS. Common Symptoms of Disease in Children. Blackwell Scientific Publications: Oxford. 1988:156-158.
Fleisher GR, Ludwig S. Synopsis of Pediatric Emergency Medicine. Williams and Wilkins. Baltimore, MD. 1996:272-276.
Sharma GD. Primary Ciliary Dyskinesia. Medscape.
Available from the Internet at http://emedicine.medscape.com/article/1002319-overview#a0199 (rev. 3/27/2012, cited 4/2/12).
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital