Guillen Gutierrez Cinthia, Rodriguez Garza Claudia, Elizondo Riojas Guillermo, Hernández Grimaldo Edgar, Garza Acosta Andrea
Final Pr. ID: Poster #: EDU-020 (S)
Cystic fibrosis (CF) is caused by autosomal-recessive mutations in the CF transmembrane regulator (CFTR) gene. Results in production of abnormally viscous mucus and secretions in the lungs of patients
It is the most common genetic disorder leading to chronic pulmonary disease in children.
In the lung, the cystic fibrosis transmembrane regulator (CFTR) is a protein responsible for efflux of chloride and inhibition of the sodium channel's activity which controls the influx of sodium. Pulmonary manifestations of CF includes
Bronchiectasis
Pneumothorax
Recurrent bacterial infection
Pulmonary arterial hypertension
Chest XRAY: is inferior to CT for the assessment of patients with known bronchiectasis. Nevertheless, radiography remains a useful modality for assessing the pulmonary complications associated with bronchiectasis, because of its low cost, availability, low radiation dose, and speed of acquisition
Brasfield scoring system
The score is based on conventional chest radiographic findings and has been reported to have good correlation with pulmonary function.
There is a maximum score of 25 with points subtracted based on the score from each of the following categories:
Air trapping: generalized pulmonary overdistension (sternal bowing, depression of diaphragms, or thoracic kyphosis)
Linear markings Linear opacification due to prominence of bronchi; may be seen as parallel line densities, branching, or “end-on” circular densities (bronchial wall thickening)
Nodular cystic lesions: multiple discrete rounded densities ≥0.5 cm in diameter, with either radiopaque or radiolucent centers (bronchiectasis); does not refer to irregular linear markings; confluent nodules not classified as large lesion
Large lesions: segmental or lobar atelectasis or consolidation, including acute pneumonia.
General severity: impression of overall severity on chest x-ray
HRCT has become indispensable in the monitoring of CF patients and is used to guide therapy and assess response to treatment, as it not only correlates with lung function tests.
Scans are repeated every 6 to 18 months depending on the clinical course.
BHALLA SCORE SYSTEM
Bhalla system can assess the degree of lung involvement and the evolution of the damages caused by lung disease based on various radiological findings. It values
Bronchiectasias
Peribronchial thickening
Extent of bronchiectasias
Extent of mucous plugs
Abscesses or sacculations
Bronchial generations affected
Number of bullae
Extent of emphysema
Collapse or consolidation
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Authors: Guillen Gutierrez Cinthia , Rodriguez Garza Claudia , Elizondo Riojas Guillermo , Hernández Grimaldo Edgar , Garza Acosta Andrea