Respiratory Distress Syndrome

Resident Lecture Series Soo Hyun Kwon, MD Neonatal-Perinatal Fellow

Overview
Definition Epidemiology Lung Development Pathophysiology Risk Factors Clinical Manifestations DDx Diagnosis Treatment

Objectives
Define respiratory distress syndrome (RDS). Discuss the epidemiology, pathophysiology, and diagnosis of RDS. List a differential diagnosis for respiratory distress in the neonate. Describe the treatments for RDS. Discuss ventilation strategies that can be used in the infant who has RDS. Describe long-term complications of RDS and its treatments.

Definition
Formerly known as hyaline membrane disease Deficiency of pulmonary surfactant in an immature lung
Disease of prematurity

Epidemiology
Major cause of morbidity and mortality in preterm infants Incidence and severity of RDS are related inversely to gestational age of newborn infant Overall incidence in 501-1500 grams: 42%
501-750 grams: 71% 751-1000 grams: 54% 1001-1250 grams: 36% 1251-1500 grams: 22% 20,000-30,000 newborn infants each year

26-28 weeks' gestation : 50% 30-31 weeks' gestation : <30%

Phases of Lung Development

Lung Development

Surfactant
Complex lipoprotein
Composed of 6 phospholipids and 4 apoproteins 70-80% phospholipids, 8-10% protein, and 10% neutral lipids

Surfactant Metabolism

Assessment of Fetal Lung Maturity

Lecithin/sphingomyelin (L/S) ratio Lamellar body counts Phosphatidylglycerol
After 35 weeks gestation

L/S Ratio

Pathophysiology

Etiology
Preterm delivery Mutations in genes encoding surfactant proteins
SP-B SP-C ATP-binding cassette (ABC) transporter A3 (ABCA3)

Lung Compliance

Normal Lung

Hyaline Membranes

Risk Factors
Prematurity Maternal diabetes C-section delivery Asphyxia

Surfactant Inactivation
Meconium and blood can inactivate surfactant activity (Full-term > Preterm) Proteinaceous edema and inflammatory products increase conversion rate of surfactant into its inactive vesicular form

Oxidant and mechanical stress associated with mechanical ventilation that uses large TV

Clinical Manifestations
Tachypnea Nasal flaring Grunting Intercostal, subxiphoid, and subcostal retractions Cyanosis

Differential Diagnosis
TTN MAS Pneumonia Cyanotic Congenital Heart Disease Pneumomediastinum, pneumothorax Hypoglycemia Metabolic problems Hematologic problems
Anemia, polycythemia

Congenital anomalies of the lungs

Diagnosis
Onset of progressive respiratory failure shortly after birth Characteristic chest radiograph ABG
Hypoxia Hypercarbia

CXR

Prevention
Antenatal glucocorticoids
Enhances maturational changes in lung architecture and inducing enzymes Stimulate phospholipid synthesis and release of surfactant All pregnant mothers at risk for preterm delivery at or below 34 weeks gestation should receive ACS

Treatment
Surfactant Therapy Assisted Ventilation Techniques Supportive Care
Thermoregulation Fluid Management Nutrition

References
Jobe AH. Why Surfactant Works for Respiratory Distress Syndrome. NeoReviews. 2006; 7: 95-106. Pramanik AK, et al. Respiratory distress syndrome. http://emedicine.medscape.com/article/976034-overview. Saker F, Martin R. Pathophysiology and clinical manifestations of respiratory distress syndrome in the newborn. Uptodate. http://www.utdol.com Warren JB, Andersen JM. Respiratory distress syndrome. Neoreviews. 2009; 7: 351-361.

Questions or Comments?