neonatal respiratory distress syndrome (hyaline membrane disease, neonatal RDS)

Etiology

• prematurity

Pathology

• failure to develop functional residual capacity
• atelectasis
• high surface tensions
• absence of pulmonary surfactant

Genetics

• susceptibility is associated with genetic variations in SFTPA1
  • association between SFTPA1 alleles & neonatal RDS is dependent on a variation Ile to Thr at position 131 in SFTPB
• susceptibility is associated with genetic variations in SFTPB
• susceptibility is associated with genetic variations in SFTPC

Clinical manifestations

• apparent within minutes to hours after birth
• rapid shallow respirations increasing to > 60/min
• cyanosis, relatively unresponsive to oxygen
• in milder cases, symptoms reach a peak in 3 days, after which improvement occurs
• death, if it occurs, generally on days 2-7

Laboratory

• arterial blood gas
• complete blood count
• PT, INR
• basic metabolic panel
• liver function tests
• serum osmolality
• also see ARUP consult

Complications

• bronchopulmonary dysplasia in mechanically ventilated infants

Differential diagnosis

• transient tachypnea of the newborn (most common)
• pneumonia
• meconium aspiration syndrome
• pneumothorax
• persistent pulmonary hypertension
• hypoxic-ischemic encephalopathy

Management

• supportive
• oxygen
• CPAP or high-flow nasal cannula
• mechanical ventilation
• prophylaxis: (24-34 weeks of gestation)
  • antenatal steroids for
    • threatened preterm labour
    • antepartum hemorrhage
    • preterm rupture of membranes
    • any condition requiring elective preterm delivery
  • beclomethasone 12 mg IM, 2 doses, 24 hours apart

More general terms

• respiratory distress syndrome (RDS)
• neonatal disorder or disease

Patient information

neonatal respiratory distress syndrome patient information

Database

• OMIM: https://mirror.omim.org/entry/267450