Unit 4 - Wk 7 Pathology

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Acute Bronchitis

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Brief, self limited inflammatory process of large/midsized airways; dry or productive cough of less than 3 weeks duration; most prevalent in winter; primarily caused by viruses

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Bacteria associated with acute bronchitis

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Mycoplasma pneumoniae; chalmydophila pneumoniae; bordetella pertussis

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Most common viral cause of acute bronchitis in the winter

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Influenza viruses

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Which other illness must you differentiate from acute bronchitis?

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pneumoniae; commonly s. pneumoniae

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Cystic Fibrosis

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Why are cystic fibrosis males often infertile

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Bilateral absence of vas deferences

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CFTR function in lungs and GI tract

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Secretes Cl- in lungs and GI tract;

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CTFR function in sweat glands

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Reabsorbs cl- from sweat

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Meconium Ileus in newborns

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Cystic fibrosis

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Biochemically what happens to the CTFR

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Mutation causes abnormal protein folding, resulting in degradation of channel before reaching cell surface

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Diagnostic test for cystic fibrosis?

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Increase concentratio nof Cl- ions (>60mmol/liter) in sweat is diagnostic

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What is the most common CFTR mutation

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Phe 508; causes CFTR to be recognized as midfolded in the ER and degraded via UPS

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Bronchiectasis

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Dilation of the bronchi and bronchioles (due to destruction of elastic tissues); caused by chronic necrotizing infections, dilation is permanent

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Primary Ciliary Dyskinesia (Kartagener Syndrome)

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Impaired cilia functioning; recurrent infections and retention of secretions

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Allergic Bronchopulmonary Aspergillosis

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Hypersensitivity reaction to aspergillus fumigatus; can occur in pts with asthma and Cf; can lead to bronchiectasis and pulmonary fibrosis of untreated

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Bronchiectasis; note widely dilated bronchi and thickening of the bronchial walls and fibrosis of the parenchyma; can be secondary to cystic fibrosis

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Note the 2 bronchi and their lumens are filled with pus; fibrovascular granulation tissue in walls with infiltration of inflammatory cells

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Pancreas w cystic fibrosis; the ducts are dilated and plugged with mucin; the parenchymal glands are atrophic and have been replaced by fibrous tissue

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Meconium Ileus; obstruction of the terminal ileum due to accumulation of tenacious meconium

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Obstructive Azospermia

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Lack of semen in sperm; secondary to congenital bilateral absence of the vas deferns in cystic fibrosis

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P. aeruginosa isolated from Cf pt; note thick mucoid capsule

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lungs of CF pt w P. aeruginosa; not characteristic greenish color

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Five distinguishing features of P. aeruginosa are:
The synthesis of pyocyanin (blue) and pyoverdin (yellow-green) pigments under aerobic conditions. Pyocyanin generates superoxide radicals and hydrogen peroxide. Pyoverdin is a siderophore.

Fruity grape-like smell

Oxidase positive

Presence of a mucoid alginate capsule

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Pseudomonas aeruginosa; gram negative rods in pairs or chains; opportunistic so very common source of nosocomial infections; presence of pili and flagella important for establishment of infection in lower respiratory tract

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Small gram negative rods (coccobacilli); polyribol capsule

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Burkholderia cepacia; not common but is a huge concern for Cf pts as it results in a highly accelerated course of the dz and poor prognosis

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Most common tx?

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Antibiotic resistance is a major problem
Acute: Ceftazidime, tobramycin
Chronic: Azithromycin

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Best treatment?

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H. influenzae

Ampicillin or broad spectrum cephalosporins are commonly used
Immunization is of course important for prevention for type b

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Staph aureus
Resistance to penicillins a problem; MRSA in particular
Vancomycin the drug of choice with MRSA
Oxacillin, clindamycin and doxycyline are also commonly used

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Atopic Asthma

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type I IgE mediated hypersensitivity reaction; starts in childhood; triggered by environmental allergins; family history

SKIN TEST FOR ALLERGEN POSITIVE

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Non atopic asthma

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No evidence of allergin sensitization and skin tests are generally negative

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Drug Induced Asthma

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Asprin sensitive asthma in pts w recurrent sinusitis and nasal polyps

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Occupational Asthma

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Triggered by various chemicals

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Late Phase Reaction

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In asthma; leukocyte recruitment secondary to chemokine production by mast cells, epithelial cells, T cells and other cytokines

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Asthma

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Curshmann spirals in asthma (shed epithelium from mucuous plugs)

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Chest radiograph of diffuse interstitial disease

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Bilateral infiltrative lesions that can take the form of nodules, irregular lines and ground glass shows

Lungs decrease in compliance; generally DILD is resistrictive

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Honeycomb lung representative of chronic diffuse interstitial lung disease

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Idiopathic Pulmonary Fibrosis

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Repeated cycles of lung injury and wound healing with increases collagen

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Formation of honeycomb lung; dense fibrosis = destruction of alveolar architecture

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Cryptogenic Organizing Pneomonia Morphology; note polypoid loose plugs of organizing connective tissue located within alveolar ducts, alveoli and bronchiles

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Coal Workers Pneumoconiosis; associated with coal miners (inhaled carbon taken up by macrophages); can resul tin cor pulmonale, Caplan's syndrome; affects upper lobes

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Silicosis

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Inhalatoin of crystalline silicon dioxide (silica); Associated with foundries, sandblasting and mines

Macrophages respond to silica and release fibrogenic factors, leading to fibrosis; if is thought that silica may disrupt phagolysomes and impair macrophages, increasing susceptibility to TB

Affects upper lobes, "eggshell" calcification of hiliar lymph nodes

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Asbestos bodies; golden brown beaded rods with a translucent center (ferruginous bodies = iron coating)

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Sarcoidosis

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Proposed model for sarcoidosis
Antigen presenting cell/helper T cell interaction results in cytokine release leading to granuloma formation. Over time the disorder may resolve or result in chronic disease.

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Asteroid body: stellate inclusions in the cytoplasm of epitheliod or giant cells; sarcoidosis

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Schumann body; laminations composed of calcium and protein

Sarcoidosis

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Organs commonly involved in sarcoidosis

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Eosinophilic Granuloma

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Infiltration of eosinophils