Cystic fibrosis - Thoracic Imaging

Title: Thoracic Imaging - Cystic fibrosis
Diagnosis: Cystic fibrosis
Author: Martins Martina
Comment: Howarth Nigel
Hospital: HUG
Department: Département de Radiologie
State: Terminée
Date: 21.03.2003
: 3370
KeyWords: Cystic fibrosis; mucoviscidosis;bronchiectasis
Anatomy: Thorax
Chapter: Obstructive disease
ACR: 6.252

Clinical Presentation: 20 year-old patient with a diagnosis ofcystic fibrosis.
Comment: Cystic fibrosis is a hereditary disease resulting fromthe inheritance of a
defective autosomal recessive gene, affecting1 person in 2500 in the Caucasian
population, and associated withprogressive respiratory involvement and pancreatic
insufficiency,first exocrine then endocrine. The respiratory signs are caused byqualitative
and quantitative anomalies of the mucus (synthesis ofan abnormal protein and increased
viscosity). Respiratoryinsufficiency, due to the bronchial obstruction and
secondaryinfections, may require a lung transplant in young adults.Bronchiectasis is
common is adults with cystic fibrosis. Thesepatients are prone to infections by
opportunistic organisms, suchS. aureus, P. aeruginosa and H. influenza. Diffuse
bronchiectasisis found within the lung parenchyma, with a predominance in theupper
Description: The frontal and lateral chest X-rays shows signs ofcystic fibrosis
associated with bilateralbronchiectasis.

Order: 0
Dislocation: 0
Polytrauma: 0
Open: 0
Pathologic: 0
Operation: 00.00.00
Graft: 0

References: Dakin CJ, Pereira JK, Henry RL, Wnag H, Morton JR.Relationship
between sputum inflammatory markers, lung function,and lung pathology on
high-resolution computed tomography inchildren with cystic fibrosis. Pediatr Pulmonol.
2002;33:475-82.Barben J, Robertson D, Olinsky A, Ditchfield M. Bronchial
arteryembolization for hemoptysis in young patients with cystic fibrosis.Radiology. 2002;
224:124-30. Marom EM, McAdams HP, Palmer SM,Erasmus JJ, Spom TA, Tapson VF,
Davis RD, Goodman PC. Cysticfibrosis: usefulness of thoracic CT in the examination of
patientsbefore lung transplantation. Radiology. 1999; 213:283-8. Stern RC.The diagnosis
of cystic fibrosis. N Engl J Med.1997;336:487-91.

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