Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Cystic fibrosis
- Radiologic Findings
- Chest radiograph shows patchy and nodular infiltration in the both upper lung zone. HRCT shows bronchiectasis with thickening of the bronchial wall, centrilobular nodules and patchy infiltration more in the upper lobes. Follow-up showed repeated aggravation and relapse of infection.
The sweat test revealed increased chloride level of 102mmol/L, which exceeds diagnostic criteria of cystic fibrosis(60mmol/L). Consequently, the diagnosis of cystic fibrosis was made.
- Brief Review
- Cystic fibrosis is a rare disease in oriental people. An autosomal recessive genetic defect in the structure of the CF transmembrane conductance regulator (CFTR) leads to abnormal chloride transport across epithelial membranes.
The mechanisms are not clear. But an abnormally low water content of airway mucus is at least partially responsible, resulting in decreased mucus clearance, mucous plugging of airways, and an increased incidence of bacterial airway infection. Upper lobes are more involved due to physiologically weak expectoration mechanism.
Radiologic findings are bronchiectasis with or without mucoid impaction, bronchial wall or peribronchial interstitial thickening and tree-in-bud appreance nodules. These findings are caused by secondary infection.
- References
- 1. Cystic Fibrosis: CT Assessment of Lung Involvement in Children and Adults Radiology 1999; 213: 537.
2. Pulmonary cystic fibrosis in the adult: early and late radiologic findings with pathologic correlations Am. J. Roentgenol., Jun 1981; 136: 1131 - 1144.
3. Thoracic Imaging: Pulmonary and Cardiovascular Radiology W.Richard Webb p534 - 536
- Keywords
- Airway, Lung, Congenital, Bronchiectasis,