Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Kartagener syndrome (primary ciliary dyskinesia, immotile ciliary syndrome)
- Radiologic Findings
- Chest CT images show bronchiectasis in right lingular segment and LML and bronchiolitis in RLL. Stomach gas is seen in the right side suggesting situs inversus. PNS view reveals sinusitis showing haziness in both maxillary sinuses.
- Brief Review
- Kartagener syndrome is a chronic lung disease that occurs when cilia are unable to move. Kartagener syndrome is a rare genetic birth defect. Individuals with Kartagener syndrome have abnormal or absent ciliary motion. Kartagener syndrome is also called Primary Ciliary Dyskinesia, and Immotile Cilia Syndrome, Afzelius syndrome, Kartagener triad, Zivert syndrome, and Zivert-Kartagener triad. Cilia are tiny hair-like structures that are present on the surfaces of many different types of cells. One of the functions of cilia is to move mucus out of the respiratory passages. During infections, more mucus is produced and because the abnormal cilia can't move or move ineffectively, mucus becomes stuck and blocks the respiratory tract, causing various complications. Some common symptoms of Kartagener syndrome are chronic rhinitis, recurrent or chronic sinusitis, recurrent or chronic bronchitis, bronchiectasis, olfactory impairment, and infertility. Treatment is aimed at reducing symptoms and slowing disease progression. Complications of Kartagener syndrome include bronchiectasis, difficulty breasthing, and chronic wheezing and coughing. Bronchiectasis occurs when the bronchial tubes are blocked and chronically infected, causing the tubes to weaken and stretch out. The widened tubes allow more mucus and bacteria to accumulate, leading to infection and possibly pneumonia.
- References
- 1. Nadel HR, Stringer DA, Turner JA, Sturgess JM. The immotile cilia syndrome: radiological manifestations. Radiology. 1985;154:651-5.
2. High-Resolution CT of patients with primary ciliary dyskinesia. AJR. 2007;188:1232-8.
- Keywords
- lung, congenital,