Pathology
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Atlas of Neonatal Pathology
Marta Ježová, Josef Feit
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+ Introduction
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+ Neonatal pathology
Hyaline membrane disease (HMD)
Introduction

Also known as respiratory distress syndrome (RDS). The incidence is inversely proportional to gestational age.

Etiology

Deficiency of pulmonary surfactant.

Vicious cycle: Decreased alveolar surfactant  —  lungs collapse at end expiration with each breath  —  increasing difficulty in breathing  —  exhaustion  —  atelectases (airless areas)  —  hypoxemia  —  endothelial and epitethelial damage: hyaline membrane formation  —  profound hypoxemia, hypercarbia and acidosis  —  progressive atelectases, abundant hyaline membranes.

Surfactant

  • synthesized by type II. pneumocytes
  • consists of lecithin, sfingomyelin and surfactant associated proteins
  • reduces surface tension at the air-liquid barrier in the alveoli
  • produced in considerable amounts after 35 weeks of gestation but modulation by variety of stimuli is possible (hormones, intrauterine stress including natural labor)
  • increased indidence of HMD than expected for gestational age: acute cesarean section before the onset of labor, asphyxia, infants of diabetic mothers
  • decreased indidence of HMD than expected for gestational age: preeclampsia, reccurent vaginal bleeding
  • there is a significantly higher risk of RDS in the second as compared to the first of twin pairs
Clinical signs
  • Signs of respiratory distress: tachypnea, cyanosis, intercostal and subcostal recession, nasal flaring, grunting, rapid increase in oxygen requirement
  • Prevention of HMD
    • administration of surfactant (prophylactic or treatment of symptoms)
    • the incidence of HMD has been reduced by 50% with the use of antenatal corticosteroids to promote lung maturity. The corticosteroids are administered to pregnant women with threatened premature delivery at 24 – 34 weeks of gestation
Macroscopic appearance
  • the classic appearance develops between 12 and 24 hours of life
  • lungs are airless, congested, liver-like consistency
Histology
  • in the early phase basophilic debris of necrotic epithelium
  • full developed phase
    • thick eosinophilic hyaline membranes (consist of necrotic bronchiolar epithelium and fibrin) lining the respiratory bronchioles and alveolar ducts,
    • atelectases
  • reparative changes occur in survivors by 48 hours by phagocytosis of membranes, regeneration of the epithelium and mild fibrosis
Pictures

Hyaline membrane disease: Hyaline membranes, newborn, HE 40x (72311)

RDS, 27-week, 6th day after delivery, twin B: Respiratory distress syndrome, 27-week, HE 60x (74597)