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Respiratory System: Pathology

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Cause of common cold
Adenovirus
Croup
Acute laryngotracheobronchitis
Acute inflammation of larynx, trachea, and epiglottis
Potentially life-threatening in infants
Harsh cough and inspiratory stridor
Caused by parainfluenza virus, most commonly
Main cause of epiglottitis
H. influenza
Can be life-threatening
Nasopharyngeal carcinoma
Most common in adults (SE Asia) and children in East Africa
Caused by EBV
Squamous cell carcinoma of the nose
most frequently occurring malignant nasal tumor
adenocarcinoma of the nose/throat
responsible for 5% of all the nose/throat malignancies
Plasmacytoma
plasma cell neoplasm that in its extraosseous form, produces tumros in the upper respiratory tract
Singer's nodules histology
cause
location
symptoms
Benign laryngeal polyp
Associated with overuse of the voice and smoking
Localized to true vocal cords
Hoarseness
Laryngeal papilloma
In adults: single lesion, can turn malignant
In children: caused by HPV 6 and 11; multiple lesions, recurs after resection
Squamous cell carcinoma of the larynx
Epidemiology
Presentation
most common malignant tumor of the larynx
Seen in men older than 40
Associated with smoking and alcoholism (combined)
Present with persistent hoarseness
What is glottic carcinoma?
Most common type of laryngeal carcinoma
Has best prognosis
What is supraglottic ad subgglottic carcinoma?
less common laryngeal carcinoma
poorer prognosis
Extrinsic asthma
asthma associated witeh chronic bronchitis, exercise induced, and cold-induced asthma
Begins in adult life
Not assocaited with hx of allergy
clinical Characteristics of asthma
Episodic dyspnea and wheezing on expiration
Narrowing of the airways
Histology of asthmatic airways
Bronchial smooth muscle hypertrophy
Hyperplasia of goblet cells
Thickening and hyalinization of BM
Proliferation of eosinophils
Intrabronchial mucous plugs containing whorl-like accumulations of epithelial cells
Crystalloids of eosinophil-derived proteins
What are Curschmann spirals
Whorl-like accumulations of epithelial cells seen in asthma
What are Carcot-Leyden crystals?
Crystalloids of eosinophil-derived proteins
Complications of asthma?
Superimposed infection
Chronic bronchitiis
Pulmonary emphysema
Status asthmaticus
Definition of chronic bronchitis
Productive cough lasting 3 consecutive weeks over 3 consecutive years
Causes of chronic bronchitis
Cigarette smoking
pollution
infection
genetics
What can chronic bronchitis lead to?
cor pulmonale
Histology of chronic bronchitis
Hypersecretion of mucus d/t marked hyperplasia of mucus secreting submucosal glands
What composes a respiratory acinus?
respiratory bronchiole
alveolar duct
alveoli
Characteristics of centrolobular emphysema
Dilation of respiratory bronchioles
In apex/upper part of pulmonary lobes
Characteristics of panacinar emphysema?
Dilation of the entire acinus, starting in the alveoli and alveolar duct, then respiratory bronchiole
Most commonly occurs in the lower basal zones
Characteristics of paraseptal emphysema
Alveoli and alveoar ducts are dilated
Localizes subjacent to pleur interlobar septa
Which type of emphysema is associated with alpha-1 anti-trypsin deficiency
Panacinar emphysema
What are complications of emphysema
Chronic bronchitis
Interstitial emphysema
Rupture of a surface bleb with resulting pneumothorax
What is interstitial emphysema
air escapes into the interstitial tissue of chest from a tear in the airways
Pathogenesis of emphysema?
Cigarette smoke causes recruitment of neutrophils and macrophages, which release elastase
Elastase destroys elastin unless neutralized alpha-1 anti-trypsin
Cigarettes inactivate alpha-1 anti-trypsin
What is the PiZ allele
structural alteration in alpha-1 anti-trypsin, which interferes with the hepatic secretion of it
What is alpha-1 anti-trypsin deficiency associated with?
panacinar emphysema
hepatic cirrhosis
cholestasis
hepatocellular carcinoma in 1-2% of homozygous adults
What is bronchiectasis?
Permanent abnormal bronchial dilation
What causes bronchiectasis
Chronic infection wiht inflammation and necrosis of the bronchial wall
What are predisposing factors for bronchiectasis?
Bronchial obstruction (often by tumor)
CF
necrotizing bacterial, viral or fungal pneumonai
RA
GVHD
chronic sinusitis with post nasal drip
Kartagener syndrome
Manifestations of Kartagener syndrome
sinusitis
bronchiectasis
situs inversus (left-right reversal of thoracic viscera)
hearing loss
immotile sperm
Cause of Kartagener syndrome
structural defect in dynein arms, impairing ciliary activity
Where in the respiratory tract does bronchiectasis most commonly appear?
Lower lobes of both lungs
Manifestations of bronchiectasis
copious purulent sputum
hemoptysis
recurrent pulmonary infection --> lung abscess
Complications of bronchiectasis
cor pulmonale
metastatic abscess
systemic amyloidosis
Characteristics of interstitial lung diseases
interstitial accumulations of cells or noncellular material within the alveolar walls that restrict expansion and interfere with gaseous exchange
(increased A-a gradient... V/Q mismatch)
EXamples of interstitial lung disease
ARDS
Neonatal respiratory distress syndrome
Pneumoconiosis
Sarcoidosis
Idiopathic pulmonary fibrosis
SLE
Scleroderma
Goodpasture syndroem
Wegener granulomatosis
How does pulmonary fibrosis lead to cardiac failure?
Pulmonary fibrosis --> repeated cycles of cellular injury --> abnormal wound healing b/c of excessive fibroblast proliferation --> cor pulmonale, pulmonary insufficiency and cardiac failure
Pathogenesis of ARDS?
diffuse alveolar damage --> increased capillary permeability --> leakage of protein rich fluid (exudate) into alveoli
Histology of ARDS
intra-alveolar hyaline membrane made of fibrin and cellular debris
Clinical result of ARDS
impairment of respiratory gas exchange with severe hypoxia
Causes of ARDS
toxic agents
shock
sepsis
trauma
uremia
aspiration of gastric contents
acute pancreatitis
O2 toxicity
OD of heroin or bleomycin
SARS
Relationship between SARS and ARDS
SARS (a coronavirus) destroys type II pnemocytes --> diffuse alveolar damage
Things that influence ARDS
Neutrophils that release substances toxic to alveolar wall
Activation of coagulation cascade
O2 toxicity from free radicals
Causes for neonatal respiratory distress syndrome
Surfactant deficiency, from premature birth/premature lungs
seen in diabetic mom, poor glucose control, c-section
Clinical manifestations of neonatal respiratory distress syndrome
Dyspnea
Cyanosis
Tachypnea
Component of surfactant
dipalmitoyl lecithin
Way to determine fetal pulmonary maturity
measure ratio of surfactant lecithin to sphingomyelin in amniotic fluid
[Lecithin] increases from 33rd week of pregnancy, while sphingomyelin remains stable
L:S 2:1 or higher = pulmonary maturity
Factors predisposing to neonatal respiratory distress syndrome
Prematurity
Materal DM
C-section
Pathologic findings of neonatal RDS
lungs are heavier than normal
Areas of atelectasis alternating with dilated alveoli/alveolar ducts
Small pulmonary BV are engorged witeh blood, with leakage of blood products into alveoli
Formation of hyaline membranes within alveoli
Components of hyaline membranes in neonatal RDS
fibrin and cellular debris
Complications/associated conditions with neonatal RDS
Bronchopulmonary dysplasia
PDA
Intraventricular brain hemorrhage
Necrotizing enterocloitis (inflammation of large and small intestines)
Morphology of necrotizing enterocolitis
mucosal edema
hemorrhage
necrosis of terminal ileum and entire gut
Components of coal dust
carbon and silica
markings of simple coal workers' pneumoconiosis
Coal macules around bronchioles
What can progresive massive fibrosis from coal workers' pneumoconiosis lead to?
bronchiectasis
pulmonary HTN
death from respiratory failure
cor pulmonale
Careers associated with silicosis
miners
glass manufacturers
stone cutters
dangers of silicotic nodules
enlarge and obstruct airways and BV
risks associated with silicosis
increased TB risk
can also be concurrent (silicotuberculosis)
Pathogenesis of asbestosis
asbestos fibers are taken up by alveolar macrophages
fibroblast-stimulating GF by macrophages likely released --> diffuse interstitial fibrosis in lower lobes
Ferruginous bodies
seen in asbestosis
yellow-brown rod shaped bodies with clubbed ends that stain for Prussian blue
Complications of asbestosis
bronchogenic carcinoma
malignant mesothelioma
Pathologic changes associated with sarcoidosis
interstitial lung disease
enlarged hilar lymph nodes
anterior uveitis
erythema nodosum of skin
polyarthritis
Immunologic phenomena associated with sarcoidosis
reduced sensitivity to skin test antigens
polyclonal hyperglobulinemia
Clinical abnormalities seen with sarcoidosis
bilateral hilar lymphadenopathy
interstitial lung disease manifest as diffuse reticular densities
Lab findings associated with sarcoidosis
hypercalcemia
hypercalciuria
hypergammaglobulinemia
increased ACE
Way to make definitive dx of sarcoidosis
biospy showing non-casseating granuloma
Progression of idiopathic pulmonary fibrosis
chronic inflammation and fibrosis of alveolar wall
begins with alveolitis --> ibrosis --> distorted fibrotic lung filled with cystic spaces
Honeycomb lung
typical of idiopathic pulmonary fibrosis
Prognosis for idiopathic pulmonary fibrosis
poor
death often within 5 yrs
morphologic changs of eosinophilic granuloma
histiocytic cells closely related ot Langerhans cells of skin
have cytoplasmic inclusions (Birbeck granules) resembling tennis rackets
Prominent monocytes-macrophages, lymphocytes, and eosinophils
Location of eosinophilic granulomata
lung or bony sites (i.e. ribs)

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