Pulmonary Flashcards
What is Kartagener syndrome?
Subtype of primary cilia dyskinesia, defective production/attachment of ciliary dynein arms
Triad of situs inversus, recurrent sinusitis, and bronchiectasis
May show low nasal NO levels
How is Kartagener syndrome differentiated from cystic fibrosis?
KS has situs inversus, CF has pancreatic insufficiency
What is meconium aspiration syndrome associated with?
Meconium aspiration syndrome is associated with persistent pulmonary hypertension of the newborn (PPHN), which can exacerbate meconium issue or is the result of meconium aspiration
Lung injury –> vascular dysfunction –> persistently elevated PVR –> R-to-L shunting across ductus arteriosus
Treatment for persistent pulmonary hypertension of the newborn?
Oxygenation, ventilation (reduce PVR)
Nitric oxide (pulmonary vasodilator)
You would see differential cyanosis (oxygen saturation right hand > foot)
What is bronchopulmonary dysplasia? How to treat?
Chronic lung disease of prematurity - incomplete alveolar development
Diagnosed when infant still requires supplemental oxygen >=28d after birth
Supportive (Oxygen, nutrition, fluid restriction/diuretics)
What are the complications of bronchopulmonary dysplasia?
Pulmonary hypertension - due to thickened muscular layer of pulmonary arteries from disrupted vasculogenesis, and prolonged alveolar hypoxia
Cardiovascular hypertension - due to circulating catecholamines and antenatal steroids
How do sickle cell acute chest syndrome and asthma relate?
They can each predispose to the other
Laryngomalacia and tracheomalacia are each associated with what kind of stridor?
Laryngomalacia - inspiratory stridor
Tracheomalacia - expiratory stridor
Treatment for apnea of immaturity
Caffeine
Transient tachypnea of the newborn shows what on chest x-ray?
Fluid in interstitial space, not alveoli (so breath sounds should be clear)
Fluid in interlobar fissures
Hyperinflation
Who is at risk for transient tachypnea of the newborn?
Premature
C-section delivery
How is respiratory distress syndrome (seen in prematurity) on x-ray?
Low lung volumes
Ground-glass opacities (diffuse reticulogranular pattern)
Air bronchograms
How is bronchopulmonary dysplasia seen on x-ray?
Mild: diffuse hazy infiltrates, low/normal lung volumes
Severe; Fibrocystic changes, hyperinflation
What is pulmonary sequestration?
Nonfunctional segment of abnormal lung tissue, which can present with respiratory distress in newborns
Chest x-ray would show dense mass
How does poorly controlled maternal diabetes increase risk for neonatal respiratory distress syndrome?
Fetal hyperinsulinism –> antagonize cortisol –> delayed maturation of phosphatidylcholine and phosphatidylglycerol (vital phospholipid components) –> delayed maturation of surfactant
Vascular rings around trachea cause what sounds? What tests?
Biphasic stridor that improves with neck extension
Laryngoscopy, bronchoscopy, echocardiogram
What is caffeine used for?
Caffeine and/or noninvasive respiratory support can be used for apnea of prematurity
Caffeine - methylxanthine - chemically stimulates respiratory drive; given until respiratory centers mature
What is risk of prolonged apnea of maturity?
Cerebral palsy
Treat with caffeine therapy and/or noninvasive respiratory support
What does congenital diaphragmatic hernia do to developing lungs? Appearance on physical exam? Treatment?
Pulmonary hypoplasia
Remodeling of pulmonary vasculature –> arterial muscular hyperplasia, persistent pulmonary HTN
Barrel-shaped chest
Scaphoid abdomen
Intubation with cautious ventilation and gastric decompression
Urgent surgical correction
Drowning treatment involves which steps, in priority?
Correction of hypoxia (supplemental O2, noninvasive PPV, endotracheal intubation)
Bronchodilators to treat bronchospasm
Which neonatal respiratory conditions have inflation or hyperinflation?
Transient tachypnea
Meconium aspiration syndrome
Bronchopulmonary dysplasia
Which neonatal respiratory condition has air bronchograms?
Respiratory distress syndrome
