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$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.