LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Libro Blanco de las ACES Pediátricas 2024 176 ❚ b. Know that the incidence of SIDS has decreased after public health campaigns to: have infants sleep on their backs; remove soft bedding, pillows, quilts, and stuffed animals from cribs; decrease environmental tobacco smoke; avoid overheating of infants 2. Therapeutic approach a. Know the appropriate management of the grieving family of an infant who died of sudden infant death syndrome 3. Side effects of therapy f. Prognosis 1. Natural history 2. Prognosis with therapy Q. Miscellaneous lung diseases 1. Alpha1-Protease Inhibitor Deficiency a. Epidemiology 1. Recognize the association between cigarette smoking and symptomatic alpha1-protease inhibitor deficiency b. Etiology/genetics 1. Know that alpha1-protease inhibitor deficiency is inherited in an autosomal recessive fashion c. Pathophysiology 1. Pathology a. Know that alpha1-protease inhibitor deficiency is associated with the development of emphysema, asthma, and chronic airway inflammation 2. Path mechanisms and consequences a. Understand the protease/antiprotease theory of lung disease associated with alpha1-protease inhibitor deficiency b. Understand the pathophysiology of liver disease in patients with alpha1-protease inhibitor deficiency d. Diagnosis and clinical manifestations 1. History a. Know that the typical age of onset of pulmonary symptoms caused by alpha1- protease inhibitor deficiency is the third or fourth decade 2. Physical examination 3. Imaging 4. Pulmonary function tests a. Recognize the pulmonary function test findings typical of emphysema 5. Other investigations a. Know the significance of the following phenotypes: ZZ, SS, MM, MZ, and NULL 6. Diagnostic criteria 7. Complications e. Prevention and therapeutic approach 1. Prevention a. Know that the onset of symptoms due to alpha1-protease inhibitor deficiency is greatly accelerated in lifelong smokers