Infant respiratory distress syndrome
|Infant respiratory distress syndrome|
|Classification and external resources|
|Patient UK||Infant respiratory distress syndrome|
Infant respiratory distress syndrome (IRDS), also called neonatal respiratory distress syndrome, respiratory distress syndrome of newborn, or increasingly surfactant deficiency disorder (SDD), and previously called hyaline membrane disease (HMD), is a syndrome in premature infants caused by developmental insufficiency of surfactant production and structural immaturity in the lungs. 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. 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.
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 intracranial hemorrhage. The disease is frequently complicated by prematurity and its additional defects in other organ function.
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.
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.
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).
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. 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.
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).
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%. Such small premature infants may remain ventilated for months. A line of research shows that an aerosol of perfluorocarbon can reduce inflammation in piglets. 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. Subsequently, preterm infants are at unacceptably high risk for intraventricular hemorrhage (IVH) if administered ECMO at a gestational age less than 32 weeks. Also later, given the risk of IVH, it has become standard practice to ultrasound the brain prior to administering ECMO.  Therefore, the device cannot be used for most premature newborns.
The INSURE Method. Henrik Verder is the inventor and pioneer of the INSURE method, a very effective approach to managing preterm neonates with respiratory distress. The method itself has been shown, through meta-analysis; to successfully decrease the use of mechanical ventilation and lower the incidence of bronchopulmonary dysplasia (BPD). Since it’s conception in 1989 the INSURE method has been academically cited in more than 500 papers. The first randomised study about the INSURE method was published in 1994 and a second randomised study in infants less than 30 weeks gestation was published by the group in 1999. In the last 15 years Henrik has worked with lung maturity diagnostics on gastric aspirates obtained at birth. By combining this diagnostic method with INSURE, Henrik has worked to further improve the clinical outcome of RDS. The lung maturity tests used have been the microbubble test, lamellar body counts (LBC) and measurements of lecithin-sphingomyelin ratio (L/S) and by spectroscopy and chemometrics, which involved a collaboration with Agnar Höskuldsson.
- In 1963, Patrick Bouvier Kennedy, son of President John F. Kennedy and First Lady Jacqueline Kennedy, died of RDS two days after his premature birth at 34 weeks gestation.
- In 1974, Daniel Wayne Lessem, first born son of Marjorie Wiesen Lessem and Dr. David S. Lessem, survived RDS (then referred to as hyaline membrane disease). Chances of survival were questionable after his premature birth at 35 weeks gestation and an uncomplicated pregnancy at Booth Memorial Hospital in Flushing, Queens NY where a neonatal incubation unit had not yet existed. After being diagnosed with hyperbilirubinemia and acute cyanosis, Daniel was whisked off by helipad transport service to Long Island Jewish Medical Center for immediate access to their new state-of-the-art neonatal intensive care unit where his condition improved in just under one week. The Wiesen/Lessem family acknowledges the Kennedy Family as well as the magnitude of their unimaginable loss of Patrick Kennedy. Elevated public interest and awareness around the tragedy led to a rapid onslaught of research & development in neonatal care, science and technological advancements which would take place over the course of a decade, allowing for a just-in-time delivery of baby Daniel. The night before Daniel's RDS condition took a turn for the better, his maternal grandmother, Sadonia Wiesen and mother Marjorie had both dreamt vividly of Sadonia's (late) father paying a visit to Daniel's neonatal unit (from the after life). Distinguished Professor, Historian and Author/Biographer, Blanche Wiesen Cook was present throughout the ordeal in support of her younger sister, "Margie".
- Bubble CPAP
- Bronchopulmonary dysplasia
- Pulmonary hypoplasia
- Surfactant metabolism dysfunction
- Surfactant therapy
- Wilson–Mikity syndrome
- "neonatal respiratory distress syndrome" at Dorland's Medical Dictionary
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- 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.
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- 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.
- "Concepts Of Neonatal ECMO". The Internet Journal of Perfusionists 1 (2).
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- 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.
- Stevens, TP; Blennow, M; Soll, RF (2004). "Early surfactant administration with brief ventilation vs selective surfactant and continued mechanical ventilation for preterm infants with or at risk for respiratory distress syndrome". The Cochrane database of systematic reviews (3): CD003063. doi:10.1002/14651858.CD003063.pub2. PMID 15266470.
- "Henrik Verder research profile". www.Researchgate.net. Retrieved 15 July 2014.
- Verder, H; Robertson, B; Greisen, G; Ebbesen, F; Albertsen, P; Lundstrøm, K; Jacobsen, T (Oct 20, 1994). "Surfactant therapy and nasal continuous positive airway pressure for newborns with respiratory distress syndrome. Danish-Swedish Multicenter Study Group.". The New England Journal of Medicine 331 (16): 1051–5. doi:10.1056/nejm199410203311603. PMID 8090164. Retrieved 15 July 2014.
- Verder, H; Albertsen, P; Ebbesen, F; Greisen, G; Robertson, B; Bertelsen, A; Agertoft, L; Djernes, B; Nathan, E; Reinholdt, J (Feb 1999). "Nasal continuous positive airway pressure and early surfactant therapy for respiratory distress syndrome in newborns of less than 30 weeks' gestation.". Pediatrics 103 (2): E24. PMID 9925870. Retrieved 15 July 2014.
- Verder, H; Ebbesen, F; Linderholm, B; Robertson, B; Eschen, C; Arrøe, M; Lange, A; Grytter, C; Bohlin, K; Bertelsen, A; Danish-Swedish Multicentre Study, Group (Jun 2003). "Prediction of respiratory distress syndrome by the microbubble stability test on gastric aspirates in newborns of less than 32 weeks' gestation.". Acta paediatrica (Oslo, Norway : 1992) 92 (6): 728–33. doi:10.1080/08035250310002597. PMID 12856986. Retrieved 15 July 2014.
- Verder, H; Ebbesen, F; Fenger-Grøn, J; Henriksen, TB; Andreasson, B; Bender, L; Bertelsen, A; Björklund, LJ; Dahl, M; Esberg, G; Eschen, C; Høvring, M; Kreft, A; Kroner, J; Lundberg, F; Pedersen, P; Reinholdt, J; Stanchev, H (2013). "Early surfactant guided by lamellar body counts on gastric aspirate in very preterm infants.". Neonatology 104 (2): 116–22. doi:10.1159/000351638. PMID 23942627. Retrieved 15 July 2014.
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- Jessen, Torben E.; Höskuldsson, Agnar T.; Bjerrum, Poul J.; Verder, Henrik; Sørensen, Lars; Bratholm, Palle S.; Christensen, Bo; Jensen, Lene S.; Jensen, Maria A.B. (2014). "Simultaneous determination of glucose, triglycerides, urea, cholesterol, albumin and total protein in human plasma by Fourier transform infrared spectroscopy: Direct clinical biochemistry without reagents". Clinical Biochemistry 47 (13–14): 1306–12. doi:10.1016/j.clinbiochem.2014.05.064. PMID 24943400.
- Hyaline membrane disease photos at: Atlas of Pathology
- Signs & Symptoms
- Respiratory Distress Syndrome