LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Neumología Pediátrica. Anexos ❚ 137 1. Recognize that asphyxiating thoracic dystrophy is associated with severe restrictive lung disease 2. Recognize the pulmonary function abnormalities associated with muscular dystrophy e. Other investigations f. Diagnostic criteria g. Complications 1. Recognize that respiratory failure is the primary cause of death in asphyxiating thoracic dystrophy 2. Recognize that asphyxiating thoracic dystrophy is associated with polydactyly and renal and hepatic disorders 4. Prevention and therapeutic approach a. Prevention b. Therapeutic approach 1. Know that surgical approaches like lateral chest expansion have been used with varying degrees of success in asphyxiating thoracic dystrophy 5. Prognosis a. Natural history 1. Recognize that the course of asphyxiating thoracic dystrophy is variable and is dependent on the degree of pulmonary hypoplasia b. Prognosis with therapy b. Sternum-pectus deformities 1. Epidemiology 2. Etiology/genetics a. Recognize that pectus excavatum occurs as both a congenital and acquired defect, the latter most likely secondary to respiratory disease with increased work of breathing b. Recognize that pectus excavatum is associated with Marfan syndrome 3. Pathophysiology a. Pathology b. Path mechanisms and consequences 4. Diagnosis and clinical manifestations a. History b. Physical examination c. Imaging 1. Recognize findings associated with a pectus excavatum deformity on x- ray study of the chest d. Pulmonary function tests 1. Recognize changes in lung function secondary to a pectus excavatum deformity 2. Recognize the effects of pectus excavatum on exercise tolerance 3. Realize that pectus carinatum does not cause significant changes in cardiopulmonary function e. Other investigations f. Diagnostic criteria g. Complications