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23 Cards in this Set
- Front
- Back
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Pulmonary hypoplasia
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Incomplete or defective development of lung causing small size due to less units/acini determined by radial alveolar count
Causes include thoracic constriction, intrathoracic lesions/masses like bronchogenic cysts, diaphragmatic hernia, extrathoracic problems like oligohydramnios or anomalies compressing thoracic cavity |
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Pulmonary agenesis
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No development of one or both lungs
Rare Bilateral is fatal |
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Bronchogenic (foregut) cyst: early development, location, clinical/radiologic presentation, histology
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Early development:
-Abnormal budding of the tracheobronchial anlage of the primitive foregut Located: -Anterior mediastinum -Along tracheobronchial tree Clinical/radiologic presentation: -Infection/obstruction -Incidental radiographic finding Histology -Features similar to normal bronchus -Submucosal glands -Cartilage |
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Congenital diaphragmatic hernia
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Most common location are postero-lateral aspects of diaphragm
Can cause hypoplasia of lung along with abnormal vasculature and pulmonary hypertension On x-ray see bowel gas in thoracic cavity |
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Tracheomalacia
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Congenital cartilage plate deficiency, very rare
Acquired due to prematurity with prolonged nasotracheal intubation |
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Tracheoesophogeal fistula
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VATER, VACTER, VACTERL associations
-Vertebrae, imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb (radial agenesis) anomalies |
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Pulmonary sequestration: extralobar vs intralobar
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Lung tissue (sequestered)
-No communication with tracheobronchial tree -Systemic (not pulmonary) arterial supply Categories (different etiologies): Extralobar form -Outside visceral pleura of lung -Male predominance -Congenital lesion (age <6 months 60%) -Histology: Various appearances, normal lung or CPAM -Associations with: Pulmonary hypoplasia, Diaphragmatic hernia, Cardiovascular malformations, Pectus excavatum Intralobar form -Inside visceral pleura of lung -Acquired lesion (age >20 years 50%) -Histology: Chronic infection, lymphoid hyperplasia, foamy macrophages, fibrosis |
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Congenital pulmonary airway malformation (CPAM): overview, compared to normal lung, subtypes
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Hamartomatous (maldeveloped) mass of lung tissue
-Disorganized -Varying degrees of cystic change Compared to normal lung -Increase in the number of structures resembling terminal bronchioles, often with polypoid growths of cuboidal epithelium and increased underlying stromal elastica and smooth muscle Cystic changes vary with subtype (types 0-4) -Subtype 0 at trachea -Subtype 1 bronchus -Subtype 2 at bronchioles -Subtype 3 at acinar units -Subtype 4 at alveoli |
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CPAM gross appearance and histology
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Gross appearance:
Spongy mass of abnormal tissue comprised of enclosed small cysts Adjacent darker atelectatic lung tissue Histology: Irregular dilated bronchiole-like spaces surrounded by alveoli-like structures Type 2 -Numerous dilated evenly spaced bronchiole-like structures within a background of alveolar structures |
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Bronchiectasis
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Dilation of bronchi/bronchioles due to destruction of elastic tissue and muscle
More prominent changes in distal areas of the lung and lower lobes usually Associated with cystic fibrosis, Kartagener syndrome, ciliary dysmotility syndrome, obstructions, infections including fungal, some autoimmune disorders, post lung transplant |
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Cystic fibrosis: Epidemiology, systems affected, lung involvement
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Most common lethal genetic disease in Caucasians
Affecting approximately 1 in 2500 live births Multisystem disease affecting: -Respiratory & digestive systems -Sweat glands and reproductive tract Lung involvement: -Present in 100% of cases -Cause of death in 95-98% of cases |
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Cystic fibrosis: Genetics
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Gene:
-Located on chromosome 7 (7q31.2) Encodes protein -Called Cystic Fibrosis Transmembrane Receptor (CFTR) -Expressed in epithelial cells in lung, GI tract and elsewhere Most common mutation in Caucasians -Autosomal recessive; occurring in 70 % of cases -Deletion of phenylalanine residue at amino acid 508 (F508) |
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Cystic fibrosis: Pathophysiology
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Chloride channel defect
Sweat duct -Increased chloride and sodium concentration in sweat Airway -Decreased chloride secretion and increased sodium and water reabsorption leading to dehydration of the mucus layer coating epithelial cells, defective mucociliary action, and mucus plugging of airways Pseudomonas aeruginosa causes respiratory infections |
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Cystic fibrosis: Pathologic features
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Widespread bronchiectasis involving all lobes (especially upper)
Inspissated tenacious mucous secretions Mucous plugging |
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Cystic fibrosis: Histology
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Bronchiectasis, with ectasia of the bronchus, chronic inflammation, fibrosis, and irregular thickening of the wall
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Additional pulmonary complications form cystic fibrosis
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Atelectasis
Pneumothorax Hemoptysis Acute respiratory failure Chronic respiratory failure Cor pulmonale |
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Primary ciliary dyskinesia: overview
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Group of disorders of ciliary movement which is
-Absent -Scattered -Uncoordinated Kartagener’s Syndrome is a subset of primary ciliary dyskinesia (50%) which includes: -Bronchiectasis -Sinusitis -Situs inversus |
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Primary ciliary dyskinesia: pathology
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Nonspecific
-Bronchiectasis -Bronchitis -Bronchiolitis Abnormal cilia (ultrastructural) -Absent/shortened dynein arms -Absence of radial spokes -Microtubular disarrangement or absence -Ciliary disorientation |
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Chronic obstructive pulmonary disease: definition and epidemiology
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COPD is the term used for patients who have airflow obstruction that is usually the result of both chronic bronchitis and emphysema
5-15% of adults in industrialized countries; 4th leading cause of death in US Primary cause of both is cigarette smoking (also marijuana smoke, air pollution, occupational exposures) |
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Chrnoic bronchitis: clinical definition, pathology/histology
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Chronic bronchitis- clinical definition= productive cough for 3 months for 2 consecutive years
Pathology/histology= mucous plugging, thickened bronchial walls, mucous gland hyperplasia, increased Reid index (Raito between the thickness of the submucosal mucus secreting glands and the thickness between the epithelium and cartilage the covers the bronchi), increased goblet cells, chronic inflammation |
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Classification of emphysema
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Centrilobular/Proximal acinar
- 85% of cases -worse in upper lobes -Due to smoking -Dilation pimarily in respiratory bronchioles Panlobular/Panacinar - 5% of cases, -worse in lower lobes, -alpha-1-antitrypsin deficiency -enlargement and destruction of airspaces, uniform involvement of the acinus Localized/Distal acinar- 5% , one or few sites of destruction, apex, spontaneous pneumothorax, bullae Paracicatricial/Irregular- adjacent to lung scars |
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Emphysema pathogenesis
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Tobacco generates reactive oxygen species, inactivation of alpha1-AT, and increased neutrophils secreting elastase
Excessive protease activity and reactive oxygen species are additive in their effects and contribute to tissue damage. Alpha1-antitrypsin deficiency can be either congenital or functional as a result of oxidative inactivation |
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Blebs and bullae
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Bullae are within the substance of the lung (enlarged airspaces over 1.0cm); Blebs are within the visceral pleura
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