PANCE Blueprint Pulmonary (10%)

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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What causes hyaline membrane disease?
It is caused by a deficiency in surfactant resulting in poor lung compliance and atelectasis

Hyaline membrane disease (also known as newborn respiratory distress syndrome or RDS) 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
  • Infants less than 30 weeks' gestation at birth (most common at 26-28 weeks)

Acute respiratory distress syndrome (ARDS) vs. Hyaline membrane disease/newborn respiratory distress syndrome (RDS) 

  • Hyaline membrane disease/respiratory distress syndrome in preterm infants (RDS) is often due to young gestational age, immature type II alveolar cells, and lack of alveolar surfactant, resulting in inadequate alveolar surface tension during expansion, which results in atelectasis, reduced gas exchange, severe hypoxia, and acidosis.
    • Hyaline membrane disease and RDS are two names for the same thing!
  • Acute respiratory distress syndrome (ARDS) in newborns and children is distinct from RDS/Hyaline membrane disease and involves diagnostic criteria (Montreux standard). Unlike RDS, ARDS of newborns and children is not based on a lack of alveolar surfactant.
  • These diagnostic criteria for ARDS of newborns and children include:
    • Acute exacerbation (within 1 week) after clinical or possible injury
    • Not caused by RDS, transient tachypnea of the newborn (TTN), congenital malformations, atelectasis, local effusions
    • Congenital heart disease that can be explained by pulmonary edema
    • Oxygenation index (OI) value ≥4

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 to maintain 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?
neonatal pneumonia
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.
congenital heart disease
While congenital heart disease may present with cyanosis, the chest x-ray will reveal a cardiac abnormality, such as cardiomegaly.
hyaline membrane disease
chronic lung disease of prematurity
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?
Respiratory distress syndrome
Spontaneous pneumothorax
Although spontaneous pneumothorax will present with respiratory distress at birth, the chest x-ray would reveal findings of lung collapse.
Transient tachypnea syndrome
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.
Meconium aspiration syndrome
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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References: Merck Manual · UpToDate

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