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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 [3]
- also see ARUP consult [2]
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 [3]
Management:
1) supportive
2) oxygen
3) CPAP or high-flow nasal cannula
4) mechanical ventilation
5) prophylaxis: (24-34 weeks of gestation)
a) antenatal steroids for
- threatened preterm labour
- antepartum hemorrhage
- preterm rupture of membranes
- any condition requiring elective preterm delivery
b) beclomethasone 12 mg IM, 2 doses, 24 hours apart [2]
General
respiratory distress syndrome (RDS)
neonatal disorder or disease
Database Correlations
OMIM 267450
References
- Nelson Textbook of Pediatrics, 14th ed., Behrman et al
(eds) WB Saunders, Philadelphia, 1992, pg 463
- ARUP Consult: Fetal Lung Maturity -
deprecated reference
- Lin TY, Ebb DH, Boepple PA et al
Case records of the Massachusetts General Hospital.
Case 12-2015. A newborn boy with respiratory distress, lethargy,
and hypernatremia.
N Engl J Med. 2015 Apr 16;372(16):1550-62
PMID: 25875261