Infant respiratory distress syndrome

Infant respiratory distress syndrome
Classification and external resources
Specialty pediatrics
ICD-10 P22
ICD-9-CM 769
OMIM 267450
DiseasesDB 6087
MedlinePlus 001563
eMedicine article/976034
Patient UK Infant respiratory distress syndrome
MeSH D012127
Orphanet 70587

Infant respiratory distress syndrome (IRDS), also called neonatal respiratory distress syndrome,[1] respiratory distress syndrome of newborn, or increasingly surfactant deficiency disorder (SDD),[2] and previously called hyaline membrane disease (HMD), is a syndrome in premature infants caused by developmental insufficiency of pulmonary surfactant production and structural immaturity in the lungs. It can also be a consequence of neonatal infection.[3][4] It can also result from a genetic problem with the production of surfactant associated proteins. IRDS affects about 1% of newborn infants and is the leading cause of death in preterm infants.[5] The incidence decreases with advancing gestational age, from about 50% in babies born at 26–28 weeks, to about 25% at 30–31 weeks. The syndrome is more frequent in infants of diabetic mothers and in the second born of premature twins.

IRDS is distinct from pulmonary hypoplasia, another leading cause of neonatal death that involves respiratory distress.

Signs and symptoms

IRDS begins shortly after birth and is manifest by fast breathing, a fast heart rate, chest wall retractions (recession), expiratory grunting, nasal flaring and blue discoloration of the skin during breathing efforts.

As the disease progresses, the baby may develop ventilatory failure (rising carbon dioxide concentrations in the blood), and prolonged cessations of breathing ("apnea"). Whether treated or not, the clinical course for the acute disease lasts about 2 to 3 days. During the first day the patient worsens and requires more support. During the second day the baby may be remarkably stable on adequate support and resolution is noted during the third day, heralded by a prompt diuresis. Despite huge advances in care, IRDS remains the most common single cause of death in the first month of life in the developed world. Complications include metabolic disorders (acidosis, low blood sugar), patent ductus arteriosus, low blood pressure, chronic lung changes, and bleeding in the brain. The disease is frequently complicated by prematurity and its additional defects in other organ function.

Histopathology

The characteristic histopathology seen in babies who die from RDS was the source of the name "hyaline membrane disease". Waxy-appearing layers of hyaline membrane line the collapsed alveoli of the lung. In addition, the lungs show bleeding, over-distention of airways and damage to the lining cells.

Pathophysiology

The lungs of infants with respiratory distress syndrome are developmentally deficient in a material called surfactant, which helps prevent collapse of the terminal air-spaces (the future site of alveolar development) throughout the normal cycle of inhalation and exhalation.

Pulmonary surfactant is a complex system of lipids, proteins and glycoproteins that are produced in specialized lung cells called Type II cells or Type II pneumocytes. The surfactant is packaged by the cell in structures called lamellar bodies, and extruded into the air-spaces. The lamellar bodies then unfold into a complex lining of the air-space. This layer reduces the surface tension of the fluid that lines the alveolar air-space. Surface tension is responsible for approximately 2/3 of the inward elastic recoil forces. In the same way that a bubble will contract to give the smallest surface area for a given volume, so the air/water interface means that the liquid surface will tend toward being as small as possible, thereby causing the air-space to contract. By reducing surface tension, surfactant prevents the air-spaces from completely collapsing on exhalation. In addition, the decreased surface tension allows re-opening of the air-space with a lower amount of force. Therefore, without adequate amounts of surfactant, the air-spaces collapse and are very difficult to expand.

Microscopically, a pulmonary surfactant deficient lung is characterized by collapsed air-spaces alternating with hyper-expanded areas, vascular congestion and, in time, hyaline membranes. Hyaline membranes are composed of fibrin, cellular debris, red blood cells, rare neutrophils and macrophages. They appear as an eosinophilic, amorphous material, lining or filling the air spaces and blocking gas exchange. As a result, blood passing through the lungs is unable to pick up oxygen and unload carbon dioxide. Blood oxygen levels fall and carbon dioxide rises, resulting in rising blood acid levels and hypoxia. Structural immaturity, as manifest by decreased number of gas-exchange units and thicker walls, also contributes to the disease process. Therapeutic oxygen and positive-pressure ventilation, while potentially life-saving, can damage the lung.

Diagnosis

The diagnosis is made by the clinical picture and the chest xray, which demonstrates decreased lung volumes (bell-shaped chest), absence of the thymus (after about 6 hours), a small (0.5–1 mm), discrete, uniform infiltrate (sometimes described as a "ground glass" appearance or as of recently described as "diffuse airspace and interstitial opacities") that involves all lobes of the lung, and air-bronchograms (i.e. the infiltrate will outline the larger airways passages which remain air-filled). In severe cases, this becomes exaggerated until the cardiac borders become inapparent (a 'white-out' appearance).

Prevention

Most cases of infant respiratory distress syndrome can be ameliorated or prevented if mothers who are about to deliver prematurely can be given glucocorticoids, one group of hormones. This speeds the production of surfactant. For very premature deliveries, a glucocorticoid is given without testing the fetal lung maturity. The American College of Obstetricians and Gynecologists (ACOG), Royal College of Medicine, and other major organizations have recommended antenatal glucocorticoid treatment for women at risk for preterm delivery prior to 34 weeks of gestation.[6] Multiple courses of glucocorticoid administration, compared with a single course, does not seem to increase or decrease the risk of death or neurodevelopmental disorders of the child.[7]

In pregnancies of greater than 30 weeks, the fetal lung maturity may be tested by sampling the amount of surfactant in the amniotic fluid by amniocentesis, wherein a needle is inserted through the mother's abdomen and uterus. Several tests are available that correlate with the production of surfactant. These include the lecithin-sphingomyelin ratio ("L/S ratio"), the presence of phosphatidylglycerol (PG), and more recently, the surfactant/albumin (S/A) ratio. For the L/S ratio, if the result is less than 2:1, the fetal lungs may be surfactant deficient. The presence of PG usually indicates fetal lung maturity. For the S/A ratio, the result is given as mg of surfactant per gm of protein. An S/A ratio <35 indicates immature lungs, between 35-55 is indeterminate, and >55 indicates mature surfactant production(correlates with an L/S ratio of 2.2 or greater).

Treatment

Oxygen is given with a small amount of continuous positive airway pressure ("CPAP"), and intravenous fluids are administered to stabilize the blood sugar, blood salts, and blood pressure. If the baby's condition worsens, an endotracheal tube (breathing tube) is inserted into the trachea and intermittent breaths are given by a mechanical device. An exogenous preparation of surfactant, either synthetic or extracted from animal lungs, is given through the breathing tube into the lungs. One of the most commonly used surfactants is Survanta, derived from cow lungs, which can decrease the risk of death in hospitalized very-low-birth-weight infants by 30%.[8] Such small premature infants may remain ventilated for months. A study shows that an aerosol of a perfluorocarbon such as perfluoromethyldecalin can reduce inflammation in swine model of IRDS.[9] Chronic lung disease including bronchopulmonary dysplasia are common in severe RDS. The etiology of BPD is problematic and may be due to oxygen, overventilation or underventilation. The mortality rate for babies greater than 27 weeks gestation is less than 10%.

Extracorporeal membrane oxygenation (ECMO) is a potential treatment, providing oxygenation through an apparatus that imitates the gas exchange process of the lungs. However, newborns cannot be placed on ECMO if they are under 4.5 pounds (2 kg), because they have extremely small vessels for cannulation, thus hindering adequate flow because of limitations from cannula size and subsequent higher resistance to blood flow (compare with vascular resistance). Furthermore, in infants aged less than 34 weeks of gestation several physiologic systems are not well-developed, specially the cerebral vasculature and germinal matrix, resulting in high sensitivity to slight changes in pH, PaO2, and intracranial pressure.[10] Subsequently, preterm infants are at unacceptably high risk for intraventricular hemorrhage (IVH) if administered ECMO at a gestational age less than 32 weeks.[11] Also later, given the risk of IVH, it has become standard practice to ultrasound the brain prior to administering ECMO.[10] Therefore, the device cannot be used for most premature newborns.

Acute respiratory distress syndrome (ARDS) has some similarities to IRDS. Transient tachypnea of the newborn presents with respiratory distress syndrome in the preterm newborn.

Famous victims

See also

References

  1. ↑ "neonatal respiratory distress syndrome" at Dorland's Medical Dictionary
  2. ↑ Northway Jr, WH; Rosan, RC; Porter, DY (Feb 16, 1967). "Pulmonary disease following respirator therapy of hyaline-membrane disease. Bronchopulmonary dysplasia". The New England Journal of Medicine. 276 (7): 357–68. doi:10.1056/NEJM196702162760701. PMID 5334613.
  3. ↑ Santosham, Mathuram; Chan, Grace J.; Lee, Anne CC; Baqui, Abdullah H.; Tan, Jingwen; Black, Robert E. (2013). "Risk of Early-Onset Neonatal Infection with Maternal Infection or Colonization: A Global Systematic Review and Meta-Analysis". PLoS Medicine. 10 (8): e1001502. doi:10.1371/journal.pmed.1001502. ISSN 1549-1676. PMC 3747995Freely accessible. PMID 23976885.
  4. ↑ Sinha, Sunil (2012). Essential neonatal medicine. Chichester, West Sussex: John Wiley & Sons. ISBN 9780470670408; Access provided by the University of Pittsburgh
  5. ↑ Rodriguez RJ, Martin RJ; Fanaroff, AA (2002). "Respiratory distress syndrome and its management". In Fanaroff, Avroy A; Martin, Richard J. Neonatal-perinatal medicine: diseases of the fetus and infant. St. Louis: Mosby. pp. 1001–1011. ISBN 978-0-323-00929-4.
  6. ↑ Men-Jean Lee, MD, Debra Guinn, MD, Charles J Lockwood, MD, Vanessa A Barss, MD. "Antenatal use of glucocorticoids in women at risk for preterm delivery". Retrieved December 16, 2013.
  7. ↑ Asztalos, EV; Murphy, KE; Willan, AR; Matthews, SG; Ohlsson, A; Saigal, S; Armson, BA; Kelly, EN; Delisle, MF; Gafni, A; Lee, SK; Sananes, R; Rovet, J; Guselle, P; Amankwah, K; Saleem, M; Sanchez, J; MACS-5 Collaborative, Group (Dec 1, 2013). "Multiple Courses of Antenatal Corticosteroids for Preterm Birth Study: Outcomes in Children at 5 Years of Age (MACS-5)". JAMA pediatrics. 167 (12): 1102–10. doi:10.1001/jamapediatrics.2013.2764. PMID 24126948.
  8. ↑ Schwartz, RM; Luby, AM; Scanlon, JW; Kellogg, RJ (May 26, 1994). "Effect of surfactant on morbidity, mortality, and resource use in newborn infants weighing 500 to 1500 g". The New England Journal of Medicine. 330 (21): 1476–80. doi:10.1056/NEJM199405263302102. PMID 8164699.
  9. ↑ von der Hardt, K; Schoof, E; Kandler, MA; Dötsch, J; Rascher, W (February 2002). "Aerosolized perfluorocarbon suppresses early pulmonary inflammatory response in a surfactant-depleted piglet model". Pediatric research. 51 (2): 177–82. doi:10.1203/00006450-200202000-00009. PMID 11809911.
  10. 1 2 "Concepts Of Neonatal ECMO". The Internet Journal of Perfusionists. 1 (2). last modified on Fri, 13 Feb 09 14:01:21 -0600
  11. ↑ Jobe, Alan H. (August 2004). "Post-conceptional age and IVH in ECMO patients". The Journal of Pediatrics. 145 (2): A2. doi:10.1016/j.jpeds.2004.07.010.
  12. ↑ Altman, Lawrence (2013-07-29). "A Kennedy Baby's Life and Death". The New York Times. Retrieved 6 June 2015.
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