[Thoracic findings in pediatric patients with cystic fibrosis].
Cystic fibrosis is the most common autosomal recessive metabolic disease in Europe with an incidence of 1:2500. The severity of the lung disease is the most important factor of morbidity and mortality in CF-patients. Because of the better diagnostic and therapeutic modalities life expectancy has increased significantly. The underlying pathology is a defect of chromosome 7, which encodes the regulation of the fluid balance across the cell membrane which effects chloride as well as sodium. The exocrine glands produce a viscous mucus which obstructs the airways and promotes infections. The result is the destruction of lung parenchyma. In daily routine, chest x-ray is still the most important radiological tool, although computed tomography depicts changes in morphology earlier and more exactly. Recent research studies show that MRI has-because of its additional functional options-interesting aspects for the future.
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Abteilung Radiologie, Vestische Kinder- und Jugendklinik, Universität Witten/Herdecke, Datteln. R.Wunsch@kinderklinik-datteln.de
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D000293:Adolescent; D001987:Bronchiectasis / Q000175:diagnosis* / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002897:Chromosomes, Human, Pair 7; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D003952:Diagnostic Imaging; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008171:Lung Diseases / Q000175:diagnosis* / Q000235:genetics; D008297:Male
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator