PANCE Blueprint Pulmonary (12%)

Hyaline membrane disease

Patient will present as → premature infant who is born at 32 weeks and after several hours develops rapid shallow respirations at 60/ min, grunting retractions, and duskiness of the skin. The chest X-ray reveals diffuse bilateral atelectasis, ground glass appearance, and air bronchograms

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Hyaline membrane disease affects premature infants. It occurs when infants are born before the lungs are producing adequate amounts of surfactant. Surfactant helps to prevent the lungs from collapsing.  As the airways collapse, infants will struggle more and more to breathe until they become acidotic and multisystem organ failure begins

  • Hyaline membrane disease is the most common cause of respiratory disease in the preterm infant
  • It is caused by a deficiency in surfactant resulting in poor lung compliance and atelectasis
  • Infants less than 30 weeks gestation at birth

CXR will demonstrate diffuse bilateral atelectasis causing a "ground glass appearance" and air bronchograms

Ground Glass Appearance on CXR

Ground Glass Appearance on CXR

Give antenatal steroid within 24-48 hours of birth - betamethasone IM x 2

  • Artificial surfactant can be given through the endotracheal tube
  • Mechanical ventilation with positive pressure

Babies may suffer permanent respiratory illness because of hyaline membrane disease, but others make a full recovery and suffer no consequences. The best treatment is maintaining a healthy pregnancy to prevent premature birth.

Question 1
You are called to the nursery to see a male infant, born by uncomplicated vaginal delivery. He weighs 2,600 grams and has one deep crease on the anterior third of each foot. Respirations are 88 breaths/minute with expiratory grunting and intercostals retractions. He is cyanotic on room air and becomes pink when placed in 60% oxygen. Chest x-ray shows atelectasis with air bronchograms. Which of the following is the most likely diagnosis?
A
neonatal pneumonia
Hint:
While tachypnea, grunting, retractions and cyanosis may be signs of neonatal pneumonia, they are primarily late findings of progressive respiratory distress and would not be seen immediately at the time of delivery. A chest x-ray in pneumonia would also most commonly reveal an infiltrate or effusion.
B
congenital heart disease
Hint:
While congenital heart disease may present with cyanosis, the chest x-ray will reveal a cardiac abnormality, such as cardiomegaly.
C
hyaline membrane disease
D
chronic lung disease of prematurity
Hint:
Chronic lung disease of prematurity is a complication in about 20% of infants with hyaline membrane disease. It is defined as respiratory symptoms, oxygen requirement and chest x-ray abnormalities at 1 month of age so it cannot be diagnosed at this time in this newborn.
Question 1 Explanation: 
Hyaline membrane disease is the most common cause of respiratory distress in the premature infant. The infant typically presents with tachypnea, cyanosis and expiratory grunting. A chest x-ray reveals hypoexpansion and air bronchograms.
Question 2
An infant born at 30 weeks' gestation begins to have respiratory difficulty shortly after birth. Examination reveals rapid, shallow respirations at 80 per minute with associated intercostal retractions, nasal flaring and progressive cyanosis. Chest x-ray reveals the presence of air bronchograms and diffuse bilateral atelectasis. Which of the following is the most likely diagnosis?
A
Respiratory distress syndrome
B
Spontaneous pneumothorax
Hint:
Although spontaneous pneumothorax will present with respiratory distress at birth, the chest x-ray would reveal findings of lung collapse.
C
Transient tachypnea syndrome
Hint:
While transient tachypnea syndrome also may present at birth with respiratory distress, the chest x-ray would reveal findings of increased pulmonary vascular markings, perihilar streaking and fluid in the interlobular fissures.
D
Meconium aspiration syndrome
Hint:
Meconium aspiration syndrome usually occurs in term or post-term infants. Typical chest x-ray findings include patchy infiltrates, coarse streaking of both lung fields, increased anteroposterior diameter and flattening of the diaphragm.
Question 2 Explanation: 
Respiratory distress syndrome (hyaline membrane disease) is the most common cause of respiratory distress in a premature infant. This diagnosis is supported by the chest x-ray findings of air bronchograms and diffuse bilateral atelectasis, causing a ground-glass appearance.
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