LIBRO + ANEXOS NEUMOLOGÍA PEDIÁTRICA

Neumología Pediátrica. Anexos ❚ 171 a. Know that the risk of postoperative respiratory complications is increased in children younger than 2 years of age who have severe obstructive sleep apnea syndrome, craniofacial abnormalities, and a history of premature birth b. Recognize the complications of mask continuous positive airway pressure in the treatment of obstructive sleep apnea syndrome c. Know that an adenotonsillectomy does not always relieve symptoms of obstructive sleep apnea syndrome, especially in those who are obese or who have craniofacial abnormalities f. Prognosis 1. Natural history 2. Prognosis with therapy a. Know that most children without underlying craniofacial disorders or obesity will have complete resolution of symptoms of obstructive sleep apnea syndrome following tonsillectomy and adenoidectomy 2. Congenital central hypoventilation syndrome a. Epidemiology 1. Know that congenital central hypoventilation syndrome is a rare cause of respiratory failure in children b. Genetics 1. Know that alveolar hypoventilation syndromes may be congenital, acquired, and/or transient 2. Know that certain metabolic and structural abnormalities affecting the central nervous system may contribute to central hypoventilation syndrome 3. Know that mutations of the PHOX2B gene cause congenital central hypoventilation syndrome c. Pathophysiology 1. Pathology 2. Path mechanisms and consequences a. Know that oxygen and carbon dioxide responses are abnormal in patients with congenital central hypoventilation syndrome b. Know that congenital central hypoventilation syndrome is a generalized disorder of the autonomic nervous system c. Know that congenital central hypoventilation syndrome can be associated with Hirschsprung disease and tumors of neural crest origin d. Diagnosis and clinical manifestations 1. History a. Recognize the diagnostic features of congenital central hypoventilation syndrome b. Know the conditions (lung disease, heart disease, brain stem malformations, neuromuscular disease, and metabolic disorders) that should be excluded before making a diagnosis of congenital central hypoventilation syndrome c. Know that some congenital central hypoventilation syndrome patients can present late as children or adults, usually with evidence of an abnormal ventilatory response under stress, such as failure to breathe after general anesthesia 2. Physical examination