Libro blanco de las ACES Pediátricas 2024

Libro Blanco de las ACES Pediátricas 2024 664 ❚ a. Know that neutrophils, monocytes, and alveolar macrophages in patients with chronic granulomatous disease have defective oxidative bursts and cannot produce oxygen radicals b. Know which microorganisms cause morbidity in patients with chronic granulomatous disease c. Know the immune defect(s) in severe combined immune deficiency d. Know the microorganisms that cause morbidity in children with severe combined immune deficiency e. Identify organisms that cause morbidity in patients with IgA deficiency f. Know that 20% to 30% of patients with IgA deficiency have associated IgG subclass deficiency g. Know that recurrent bacterial infections of upper and lower respiratory tracts are the main clinical manifestations of common variable immunodeficiency and that bronchiectasis may develop if optimal therapy is delayed h. Know the common infective agents seen in common variable immunodeficiency: Haemophilus influenzae, Moraxella catarrhalis, Streptococcus pneumoniae, and Staphylococcus aureus d. Diagnosis and clinical manifestations 1. History a. Know that IgG deficiency is associated with chronic (recurrent) pulmonary infections 2. Physical examination 3. Imaging 4. Pulmonary function tests a. Know that lung disease secondary to IgG or IgA deficiency usually has an obstructive ventilatory pattern on pulmonary function testing 5. Other investigations a. Know how to establish the diagnosis of chronic granulomatous disease on the basis of laboratory data b. Know that decreased concentrations of IgG2 and IgG4 are associated with recurrent pulmonary infections c. Know that healthy patients can have decreased concentrations of IgG2 and IgG4 6. Diagnostic criteria 7. Complications a. Know that the incidence of pneumococcal, Haemophilus influenzae and Salmonella pneumonias is increased in patients with sickle cell disease e. Prevention and therapeutic approach 1. Prevention 2. Therapeutic approach 3. Side effects of therapy a. Know that patients with IgA deficiency may have anaphylactic reactions to intravenous immune globulin therapy b. Know which vaccines can safely be administered to patients with immune deficiency