HUMB2009 Lecture Notes - Lecture 5: Pectus Carinatum, Pectus Excavatum, Bronchiectasis

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Pectus excavatum (funnel chest): congenital chest wall deformity characterised by concave depression of the sternum. Patient presentation: sternum depressed; anterior ribs protrude anterior to sternum. Pectus carinatum (pigeon chest): chest wall deformity in which the sternum protrudes anteriorly. Patient presentation: anterior protrusion of sternum; congenital or acquired. Symptoms: patients with mild disease may be asymptomatic, recurrent pneumona; cough, dyspnea, wheezing, hemoptysis, may be massive, sweat chloride test positive in 98% of patients with cf. Cystic fibrosis: genetic disease affecting exocrine function of the lungs, liver, pancrease and small bowels resulting in progressive disability and multi system failure. Hereditary disease characterised by the secretion of excessive viscous mucus by all the exocrine glands blocking the trachea and bronchi (result of imbalance of sodium and chloride production/reabsorption) Defective gene (chromosome 7) typical in white children. Patients can suffer from recurrent pulmonary infections (resulting in bronchiectasis, large cysts and abscesses) Accounts for up to 25% of adult cases of bronchiectasis.

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