Disease Information for Bullous Emphysema

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Clinical Manifestations
Signs & Symptoms
Epigastric apical impulse
P2 sound accentuated
Dyspnea
Dyspnea on exertion
Dyspnea, chronic progressive
Hemoptysis
Low diaphragms/percussion/symmetric
Respiratory distress
Disease Progression
Course/Progressive
Demographics & Risk Factors
Past History
Past history/Pneumothorax
Laboratory Tests
Abnormal Lab Findings - Decreased
Alpha-1-antitrypsin globulin level (Lab)
Diagnostic Test Results
Other Tests & Procedures
PFT/Abnormal pulmonary function tests
PFT/Vital capacity decreased
Isotope Scan
Isotope/V/Q Lung scan abnormal
Isotope/VQ lung scan/mismatch
EKG (ECG)
EKG/P Wave > 2.5 mV inferior leads (ECG)
EKG/Peaked tall P Waves 2,3,F leads (ECG)
EKG/P-Pulmonale pattern (ECG)
CT Scan
High definition CT/spiral chest
X-RAY
Xray/Chest abnormal
Xray/Cavitation/Cavitary lung lesion/Chest
Xray/Chest/Lung fields/Abnormal
Xray/Hyperaeration/lung/Chest
Xray/Hyperinflated lung/Chest
Xray/Segmental hyperlucency/Chest
Xray/Unilateral hyperlucent lung/Chest
Ultrasound
Echo/Pulmonary artery hypertension
Associated Diseases & Rule outs
Rule Outs
Lung abscess
Associated Disease & Complications
Bullous Emphysema Syndrome
Cor pulmonale
Hypoxia, systemic
Lung cystic disease/acquired
Pneumothorax
Pneumothorax, spontaneous
Pulmonary hypertension, secondary
Respiratory failure/Pulmonary insufficiency
Disease Mechanism & Classification
Class
CLASS/Lung Disorder (ex)
CLASS/Pulmonic (category)
Pathophysiology
Pathophysiology/Ventilation/perfusion inequality
Process
PROCESS/Structural/anatomic/foreign body (category)
Synonyms
Synonym
Bullous lung disease, LUNG DISEASE BULLOUS, Synonym/Giant Bullous Emphysema Var., Synonym/Vanishing Lung Syndrome Var.
Treatment
Drug Therapy - Indication
RX/Oxygen
SX/Lung transplant
Definition

Bullous Emphysema (also known as Bullous Lung Disease; Bullous emphysema is so named when there are multiple large bullae associated with a compromise in pulmonary function; It is usually associated with concomitant emphysema, although occasionally, it can be familial; Bullae are thin-walled, sharply-demarcated areas of lung destruction with avascularity, measuring 1 cm or more in diameter, with thin walls measuring less than 1 mm in thickness; The bullae are commonly subpleural in location and can occur in any of the 4 types of emphysema, but most commonly in the centrilobular and paraseptal types; The bullous lesion can be uni or multi-locular with a septated space; When looking at an autopsy removed lung, a bullous lesion can be seen as a localized empty sac or bag-like protrusion from the lung surface; They are most commonly subpleural in location; Bullae can be associated with pneumothorax formation, infection and/or hemorrhage; They can occasionally disappear, especially after infection or hemorrhage, or even on a spontaneous basis; Bullous Emphysema has three types: Subpleural Type: These subpleural bullae contain only gas with no alveolar remnants or blood vessels; They are often located in the apex of the upper lung zone, and along the costophrenic rim of the middle lobe and lingua, but may be seen in the vicinity of parenchymal scars;

Superficial Type: These bullae are found along the anterior edge of the upper and/or middle lung zones, or lingula, and over the diaphragms; They contains blood vessels and strands of partially-destroyed lung;Deep Type: These bullae are found within the lung substance and contain strands of partially-destroyed lung tissue and blood vessels;

Giant Bullous Emphysema: ("Vanishing Lung Syndrome" or "Primary Bullous Disease of the Lungs"); It is usually associated with young males who show large progressive upper lung zone bullae that are often asymmetric; The giant bullous lesions occupy greater than or equal to one-third of the hemithorax; Oftentimes, the adjacent normal lung is compressed, becoming atelectatic, making it difficult for that portion of the lung to appropriately function; Spontaneous pneumothorax is a common consequence of this disease; Other etiologies for Vanishing Lung Syndrome include pulmonary sarcoid or Langerhans cell hystiocytosis;

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External Links Related to Bullous Emphysema
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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