LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Libro Blanco de las ACES Pediátricas 2024 124 ❚ 3. Be able to recognize digital clubbing c. Imaging 1. Recognize findings of cystic fibrosis on x-ray study of the chest 2. Recognize typical manifestations of cystic fibrosis on computed tomography (CT scan) of the chest 3. Know that findings on x-ray studies of the sinuses are not usually helpful in the management of sinus disease in patients with cystic fibrosis 4. Recognize the indications for bronchial arteriography in a patient with cystic fibrosis 5. Recognize radiographic findings typical of intestinal obstruction caused by distal intestinal obstruction syndrome in a patient with cystic fibrosis d. Pulmonary function tests 1. Recognize pulmonary function findings typical of cystic fibrosis and its progression e. Other investigations 1. Know how to assess pancreatic exocrine deficiency 2. Know the major laboratory criteria for allergic bronchopulmonary aspergillosis in patients with cystic fibrosis 3. Recognize the typical blood gas findings associated with various stages of cystic fibrosis lung disease 4. Recognize that both mucoid and non-mucoid strains of Pseudomonas aeruginosa may be cultured from sputum in patients with cystic fibrosis and that the mucoid strain is secondary to release of exopolysaccharide 5. Appreciate the investigations required prior to liver transplant in cystic fibrosis 6. Know the appropriate approach to screening for and confirmation of cystic fibrosis-related diabetes 7. Understand appropriate physical and laboratory methods for detection of malnutrition in cystic fibrosis patients f. Diagnostic criteria 1. Understand the rationale for a minimum volume or weight of sweat required for an acceptable sweat test 2. Know that a single sweat test result by itself does not confirm or negate the diagnosis of cystic fibrosis 3. Recognize the indications for repeating a sweat chloride measurement 4. Recognize the value and limitations of genetic testing in the diagnosis of cystic fibrosis 5. Know the diagnostic criteria for cystic fibrosis g. Complications 1. Recognize the association between pulmonary hemorrhage and cystic fibrosis 2. Know that pneumothorax is a complication of cystic fibrosis lung disease 3. Know that nasal polyposis is a frequent complication of cystic fibrosis 4. Know that sterility is very common in males with cystic fibrosis 5. Know that portal hypertension may result from hepatobiliary disease in patients with cystic fibrosis 6. Know that dehydration and hypotension may be the initial symptoms in a patient with cystic fibrosis