$Unique_ID{BRK03542} $Pretitle{} $Title{Bronchopulmonary Dysplasia (BPD)} $Subject{Bronchopulmonary Dysplasia BPD Respiratory Distress Syndrome Infant Pulmonary Interstitial Emphysema} $Volume{} $Log{} Copyright (C) 1991 National Organization for Rare Disorders, Inc. 845: Bronchopulmonary Dysplasia (BPD) ** IMPORTANT ** It is possible the main title of the article (Bronchopulmonary Dysplasia (BPD) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names and disorder subdivisions covered by this article. Synonyms Information on the following diseases can be found in the Related Disorders section of this report: Respiratory Distress Syndrome, Infant Pulmonary Interstitial Emphysema General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact you personal physician and/or the agencies listed in the "Resources" section of this report. Bronchopulmonary Dysplasia is a chronic bronchial tube and lung disease that affects infants who have been on a ventilator. This disorder usually occurs when an infant is approximately 28 days old, has certain blood-gas and radiographic (x-ray or gamma ray) abnormalities, and there has been an apparent lung injury causing a respiratory disorder. Pulmonary Distress Syndrome (lung disease in infants causing difficulty in breathing and collapsed lungs) are often also present. Symptoms The symptoms of Bronchopulmonary Dysplasia may be inflammation, difficulty breathing, collapsed lungs, flat chest, low birthweight, air flow obstruction, lung air leaks, abnormal blood-gas, and scarring in the air-cell (alveolar) walls of the lungs. When a chest x-ray is taken the lungs may show up hazy. Infection can be found in or near the tracheal tube, and the air cells in the membranes that line the lung cavity begin to separate forming dead tissue. Causes Bronchopulmonary Dysplasia may be associated with the use of ventilators and high concentrations of oxygen given to babies. It is more common among premature infants and often follows Infantile Respiratory Distress Syndrome. In many cases the infant does not wean from the ventilator in the 5 to 6 day period when Respiratory Distress Syndrome should have cleared up. The prolonged use of the ventilator may be a contributing factor in this disorder. Undernutrition may also be a factor in the development of Bronchopulmonary Dysplasia. Because infants with a low birth weight have a very small reserve of calories and nutrients, their capability of fighting this lung disease is greatly diminished. Affected Population Bronchopulmonary Dysplasia affects males and females in equal numbers. Two to sixty-eight percent of infants with Respiratory Distress Syndrome end up developing Bronchopulmonary Dysplasia. Most infants who develop this disorder are about 28 days old, have a low birth weight, have received supplemental oxygen, and have been on a ventilator. In the United States, this has become the most common form of chronic lung disease in infants. Related Disorders Symptoms of the following disorders can be similar to those of Bronchopulmonary Dysplasia. Comparisons may be useful for a differential diagnosis: Infant Respiratory Distress Syndrome is a lung disorder causing difficulty in breathing and collapsed lungs. This syndrome is characterized by a loss of oxygen intake in the lungs of premature babies. There is an absence of a natural lung wetting agent (surfactant) in the immature lungs of the infant. When this surfactant is missing, breathing becomes difficult and the lungs may collapse. (For more information on this disorder, choose "Respiratory Distress Syndrome" as your search term in the Rare Disease Database). Pulmonary Interstitial Emphysema is a disorder in which the normal lung air spaces have air-leaks. This disorder usually occurs when infants are having trouble breathing, are on a ventilator, and have serious lung diseases. Pulmonary Interstitial Emphysema may also occur sporadically. One of both lungs may be involved and the disorder may be concentrated in one area, or widespread within each lung. Therapies: Standard Bronchopulmonary Dysplasia requires constant monitoring of the amount of oxygen in the arteries (arterial oxygenation). Further ventilation and oxygen support may be required. This support should be reduced as tolerated. When chronic lung disease is present, the blood gas level should be allowed to rise above normal while weaning from the ventilator. This procedure may be used as long as the infant is not having too much difficulty breathing and their acid balance remains normal. Nutrition plays an important part in the treatment of infants with Bronchopulmonary Dysplasia. Because of the extra work required for breathing, the infant may need an increase in calories. This increase can be provided by using intravenous or tube feedings. Diuretic therapy (a drug to increase the flow of urine) may be used to help prevent lung congestion. Furosemide or a combination of chlorothiazide and spironolactone may be needed for several weeks after the infant has been weaned off the ventilator. The vaporized drug Terbutaline may be used to help lung mechanics in infants using ventilators. Therapies: Investigational This disease entry is based upon medical information available through April 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Bronchopulmonary Dysplasia, please contact: National Organization for Rare Diseases (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Heart, Blood & Lung Institute (NHBLI) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-4236 American Lung Association 1740 Broadway New York, NY 10019 (212) 315-8700 References THE MERCK MANUAL, 15th Ed.: R. Berkow, ed.-in-chief; Merck & Co., Inc., 1987. Pp. 1870-71. PULMONARY DISEASE AND DISORDERS. 2nd Ed.: Alfred P. Fishman, M.D., et al.; Editors; McGraw-Hill Book Company., 1988. Pp. 2261-62. FLAT CHEST IN CHRONIC BRONCHOPULMONARY DYSPLASIA. D.K. Edward, et al.; Am J Roentgenol (February, 1988, issue 150 (2)). Pp. 1213-6. ANTIREFLUX SURGERY IN INFANTS WITH BRONCHOPULMONARY DYSPLASIA. R.M. Giuffre, et al.; Am J Dis Child (June, 1987, issue 141 (6)). Pp. 648-51. RESPONSE OF PULMONARY MECHANICS TO TERBUTALINE IN PATIENTS WITH BRONCHOPULMONARY DYSPLASIA. D.S. Brudno, et al.; Am J Med Sci (March, 1989, issue 297 (3)). Pp. 166-8. LATE PULMONARY SEQUELAE OF BRONCHOPULMONARY DYSPLASIA. W.H. Northway Jr., et al.; N Engl J Med (December, 1990, issue 323 (26)). Pp. 1793-9.