Updated: 1/16/2020

Neonatal Respiratory Distress Syndrome

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Introduction
  • CXRPathophysiology
    • aka hyaline membrane disease
    • immature fetal lungs have a deficiency in surfactant
    • reduced surfactant results in low lung compliance and subsequent atelectasis, intrapulmonary shunting, and acute lung injury
  • Causes
    • prematurity (most important)
      • type II pneumocytes not developed
        • normal source of surfactant production
    • Cesarean section
      • lack of vaginal compression stress on the infant during delivery results in reduced fetal cortisol production and resultant reduction in surfactant production
    • maternal diabetes 
      • high insulin levels decrease surfactant production
        • increased fetal blood glucose results in increased fetal insulin production
Presentation
  • Symptoms
    • dyspnea, tachypnea during first hours of life
    • nasal flaring
    • expiratory grunting,
    • intercostal, subxiphoid retractions
  • Physical exam
    • severe hypoxemia 
      • not responsive to 100% O2
        • atelectasis results in intrapulmonary (right-to-left) shunting
          • perfusion without ventilation
Evaluation
  • ABG
    • hypoxemia
    • respiratory acidosis (elevated PCO2) from poor ventilation 
  • Histology 
    • hyaline membrane surrounds collapsed alveoli
  • CXR
    • "ground-glass" appearance 
  • Amniotic fluid  
    • lecithin:sphingomyelin ratio < 1.5 
Treatment
  • Prevention 
    • corticosteroids given to mother between 23 and 34 weeks gestation at increased risk for preterm delivery within 7 days  
      • results in increased surfactant production
  • Treatment
    • nasal CPAP
    • surfactant replacement (endotracheal, so intubation is needed) 
Complications
  • Bronchopulmonary dysplasia
    • still very common
  • Patent ductus arteriosus
    • normally closed by increased Otension at birth
      • hypoxia keeps shunt open
  • 100% O2 therapy damage
    • free radical damage of eyes and lungs
      • may result in blindness and pulmonary dysplasia 
    • damage also when the neonate is taken off oxygen therapy
      • upregulation of VEGF due to relative hypoxia results in neovascularization in the retina (retinopathy of prematurity)
 
 

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Questions (10)
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(M1.PL.15.70) An infant born prematurely at 28 weeks gestation is immediately noticed to be tachypneic and cyanotic following birth. A chest CT scan is obtained, which is shown in Figure A. A presumptive diagnosis is made based on the clinical picture and chest CT, and treatment is initiated. Which of the following organelles and cell types are involved in this clinical picture? Tested Concept

QID: 106490
FIGURES:
1

Lamellar bodies - type I pneumocytes

2%

(1/65)

2

Mitochondria - type II pneumocytes

3%

(2/65)

3

Lamellar bodies - type II pneumocytes

92%

(60/65)

4

Endoplasmic reticulum - type I pneumocytes

0%

(0/65)

5

Plasma membrane - type II pneumocytes

0%

(0/65)

M 1 D

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(M1.PL.14.70) A P2G1 diabetic woman is at risk of delivering at 29 weeks gestation. Her obstetrician counsels her that there is a risk the baby could have significant pulmonary distress after it is born. However, she states she will give the mother corticosteroids, which will help prevent this from occurring. Additionally, the obstetrician states she will perform a test on the amniotic fluid which will indicate the likelihood of the infant being affected by this syndrome. Which of the following ratios would be most predictive of the infant having pulmonary distress? Tested Concept

QID: 106471
1

lecithin:sphingomyelin > 1.5

6%

(5/86)

2

lecithin:phosphatidylserine > 3.0

6%

(5/86)

3

lecithin:sphingomyelin > 3.0

7%

(6/86)

4

lecithin:sphingomyelin < 1.5

73%

(63/86)

5

lecithin:phosphatidylserine < 1.5

7%

(6/86)

M 1 D

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(M1.PL.14.33) A male is born at 30-weeks gestation. His first few days of life are complicated by multiple issues. On delivery he is profoundly dyspneic and lacks normal coloration. Oxygen saturation reveals severe hypoxemia. When the patient still does not improve with supplemental oxygen, he is intubated and transferred to the ICU. He is treated with artificial surfactant, and his status improves though he requires multiples days on mechanical ventilation with high levels of supplemental oxygen. His hospital course is also complicated by intraventricular hemorrhage. Regarding this patient's pulmonary issues, what would be the diagnostic finding on chest x-ray consistent with this disease, and which of the following fundoscopic images depicts a complication of the treatment for this pulmonary disease? Tested Concept

QID: 105431
FIGURES:
1

The chest x-ray would reveal ground glass opacities and the fundoscopic image depicting a complication of the treatment is Figure C

29%

(17/58)

2

The chest x-ray would reveal tracheal deviation and the fundoscopic image depicting a complication of the treatment is Figure A

3%

(2/58)

3

The chest x-ray would reveal an interstitial infiltrate and the fundoscopic image depicting a complication of the treatment is Figure A

10%

(6/58)

4

The chest x-ray would reveal tracheal deviation and the fundoscopic image depicting a complication of the treatment is Figure C

2%

(1/58)

5

The chest x-ray would reveal ground glass opacities and the fundoscopic image depicting a complication of the treatment is Figure B

53%

(31/58)

M 2 D

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(M1.PL.12.71) A pathologist examines the section of tissue shown in Figure A. A defect in the structure marked with the letter "X" is associated with which of the following diseases? Tested Concept

QID: 100874
FIGURES:
1

Asthma

0%

(0/48)

2

Neonatal Respiratory Distress Syndrome

96%

(46/48)

3

Silicosis

0%

(0/48)

4

Asbestosis

2%

(1/48)

5

Bronchiolitis Obliterans

0%

(0/48)

M 1 D

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(M1.PL.12.75) A neonate suffering from neonatal respiratory distress syndrome is given supplemental oxygen. Which of the following is a possible consequence of oxygen therapy in this patient? Tested Concept

QID: 100878
1

Atelectasis

16%

(11/69)

2

Anosmia

0%

(0/69)

3

Atopy

0%

(0/69)

4

Blindness

84%

(58/69)

5

Cardiac anomalies

0%

(0/69)

M 3 E

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(M1.PL.12.25) An infant male born premature at 30 weeks to a 24-year-old woman is found to be dyspneic during the first few hours of life. The newborn appears hypoxemic, and his SpO2 is not increased by 100% O2 given via facemask. A chest radiograph is shown in Figure A. Which of the following substances, if administered to the mother in the prenatal period, could have prevented this patient's symptoms? Tested Concept

QID: 101919
FIGURES:
1

Oxygen

1%

(1/159)

2

Lecithin

6%

(9/159)

3

Sphingomyelin

3%

(5/159)

4

Insulin

3%

(4/159)

5

Dexamethasone

86%

(137/159)

M 1 E

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