Question

Define the following disorders: asthma, cystic fibrosis, chronic lung disease of prematurity, bronchopulmonary dysplasia, idiopathic congenital central hypoventilation syndrome, tracheoesophageal fistula with esophageal atresia, tracheoesophageal fistula without esophageal atresia, and congenital diaphragmatic hernia.

Answer

 

  • Asthma: “an obstructive airway disease that includes hyperreactivity, airway inflammation, and reversible airway obstruction, resulting in airflow limitation and decreased flow rates, causing air trapping with hyperinflation.”
  • Cystic fibrosis: “a genetic disorder inherited in an autosomal recessive pattern that causes abnormal levels of inorganic ions in the serous gland secretions, thickened secretions from mucus secreting glands, and increased susceptibility to colonization of the respiratory tract by certain bacteria.” (pgs. 437-440 of Second Edition or p. 493 of First Edition)
  • Chronic lung disease of prematurity: “having a continuing oxygen requirement at 36 weeks corrected gestational age.”(p. 445 of Second Edition or p. 504 of 2004 First Edition)
  • Bronchopulmonary dysplasia: “persistent respiratory distress with the need for oxygen past the 28th day of life as well as compatible chest x-rays following the treatment of severe respiratory distress syndrome.” (p. 445 of Second Edition or p.504 of First Edition)
  • Idiopathic congenital central hypoventilation syndrome (CCHS): “a rare syndrome also known as Ondine’s curse. This syndrome appears in children who are adequately ventilated during the day, but have a normal respiratory rate and shallow breathing with varying degrees of hypoventilation during sleep.”(p. 449 of Second Edition or p. 508 of First Edition)
  • Tracheoesophageal fistula with esophageal atresia: Although this anomaly has several different presentations, the most common is a proximal atresia of the esophagus, in which the esophagus ends in a blind pouch with a distal tracheoesophageal fistula. (p. 453 of Second Edition or p. 512-513 of First Edition)
  • Tracheoesophageal fistula without esophageal atresia: This disorder is characterized by having an opening between the trachea and a normal esophagus. (p. 453 of Second Edition or p. 512-513 of First Edition)
  • Congenital diaphragmatic hernia (CDH): a disorder in which the diaphragm muscles fail to fully develop or close between the abdominal peritoneum and the chest cavity during fetal development. (p. 456 of Second Edition or p. 516 of First Edition)

 

Engleman. (2011) Chapter 17: “Life Care Planning for the Child with Asthma and Other Chronic Respiratory Conditions”, in Pediatric Life Care Planning and Case Management (2nd Edition). (pages 487-528)

Engleman. (2004) Chapter 21: “Life Care Planning for the Child with Asthma and Other Chronic Respiratory Conditions,” in Pediatric Life Care Planning and Case Management. (pages 487-528)

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