LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Neumología Pediátrica. Anexos ❚ 177 b. Know that smoking causes oxidant inactivation of intrapulmonary proteinase inhibitors 2. Therapeutic approach a. Recognize the indications for replacement therapy in patients with alpha1- protease inhibitor deficiency 3. Side effects of therapy f. Prognosis 1. Natural history 2. Prognosis with therapy 2. Ciliary dysmotility a. Epidemiology b. Etiology/genetics 1. Know the mode of inheritance of ciliary dysmotility c. Pathophysiology 1. Pathology a. Know the ultrastructural defects of cilia in patients with ciliary dysmotility 2. Path mechanisms and consequences a. Know how ciliary dysmotility leads to airways disease b. Understand the relationship between ciliary dysmotility and male sterility d. Diagnosis and clinical manifestations 1. History a. Recognize the features of ciliary dysmotility in children b. Know that neonatal respiratory disorders occur in the majority of children with ciliary dysmotility 2. Physical examination 3. Imaging a. Understand the association between situs inversus and ciliary dysmotility 4. Pulmonary function tests 5. Other investigations a. Recognize the indications for ciliary biopsy and/or sperm analysis b. Know approaches to the measurement of ciliary transport rates and ciliary beat frequency c. Know the value of nasal nitric oxide in the diagnosis of ciliary dysmotility 6. Diagnostic criteria a. Know the diagnostic criteria for ciliary dysmotility b. Recognize the limitations of ciliary biopsy in establishing the diagnosis of ciliary dysmotility following an acute viral infection 7. Complications e. Prevention and therapeutic approach 1. Prevention 2. Therapeutic approach f. Prognosis 1. Natural history a. Know the natural history of ciliary dysmotility 2. Prognosis with therapy 3. Oncology