Organ Pathology

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Organ Pathology

• Respiratory System - I

Disorders of lung airiness
Jaroslava Dušková Inst. Pathol. ,1st Med.
Faculty, Charles Univ. Prague
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Lung Function

• oxygenation
• carbon dioxide excretion

normal aeration of the lung tissue -
conditio sine qua non
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Disorders of Lung Airiness

• - atelectasis / collaps
• emphysema
• coexistent in many pathology conditions !

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Atelectasis / Collaps

• Def.
• (cz) A inadequate expansion
• C secondary loss of airiness
• (irrespective of age)
• (eng)
• A inadequate expansion
• A loss of lung volume

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Atelectasis in Children

• Inadequate expansion
• immature lung tissue insufficient surfactant
  (S) production
• Clinical manifestation - RDS
• Regulation of (S) synthesis
• corticoids, thyroxine insuline

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The main events and outcomes in ARDS
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Atelectasis (collaps) in Adults - causes

• obstruction
• compression
• microatelectases
• contraction

• chest wall restriction
• flail chest

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Atelectasis (collaps) in Adults causes I

• obstruction mucopurulent plug (infection,
  mucoviscidosis), bronchomalacia
  bronchiectasis, chronic bronchitis, astma
  foreign body, neoplasm (pores of Kohn)
• compression fluid, air, neoplasms in
  the pleural cavity
• microatelectases loss of surfactant
• contraction loss of lung elasticity mostly
  following fibrosis

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Atelectasis (collaps) in Adults causes II

• chest wall restriction (obesity, scoliosis)
• flail chest (several broken ribs)

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Mucoviscidosis cystic fibrosis, fibrocystic
disease

• autosomal recessive 7th chromosome
• 2-5 heterozygotic carriers in the caucasian
  population
• abnormal viscosity of mucin
• disturbance of the membrane associated protein
  that serves as a calcium channel
• increased concentration of chloride in sweat
• decreased water content in excocrine secrets

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Mucoviscidosis cystic fibrosis, fibrocystic
disease

• Complications
• meconium ileus
• steatorrhea
• pancreatic fibrosis cysts
• bronchitis, bronchopneumonia, bronchiectasia
• sterility

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Bronchiectasis

• Def.
• persistent abnormal dilation of the bronchus.
• Types
• cylindrical
• saccular

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Chronic Obstructive Airways Disease COAD

• limitation to airflow in the lungs due to
• airways resistence increased narrowing
• loss of elastic recoil
• Diseases of COAD type
• chronic bronchitis
• asthma
• emphysema

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Chronic obstructive pulmonary disease (COPD)

• Def.
• chronic productive cough lasting at least three
  months during two subsequent years
• Causes SMOKING, air pollution

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Chronic Bronchitis

• Morphology
• hyperplasia of mucin producing goblet cells
  (1GC 7CC 1GC1CC)
• epithelial hyperplasia ( dysplasia!)
• inflammatory infiltrate

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Asthma

• Def.
• a chronic inflammatory disorder of the airways in
  which many cells and cellular elements play a
  role,
• in particular, mast cells, eosinophils, T
  lymphocytes, macrophages, neutrophils, and
  epithelial cells.

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Asthma

• Clinical manifestation
• recurrent episodes of wheezing
• breathlessness
• chest tightness,
• coughing at night or in the early morning
  
• Prologed attack status asthmaticus

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Asthma - types

• Intrinsic
• abnormal
• ß-adrenergic reaction
• IgE normal
• causes
• coolness
• effort
• infection
• aspirin

• Extrinsic
• type I hypersensitivity
• IgE
• causes
• dust
• pollen
• animal fur
• drugs
• foodsuff.

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Asthma

• Morphology
• bronchial lumina blocked by viscose mucus and
  exudate with eosinophils
• oedema and infiltration of the mucose
• thickening of the muscle layer and basement
  membrane
• Sputum
• Charcot-Leyden crystals derived from eosinophils
• Curshman spirals glycoproteins
• Creola bodies

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Extrinsic allergic astma
Start in childhood, evidence of atopy Allergens
- household dust, organic dusts, pollens
grasses and trees, animal fur, food products,
drugs Reagin mediated type
I hypersensitivity reaction Serum IgE increased,
Skin tests against antigens positive Mast cell
degranulation histamin, bradykinin Smooth
muscle contraction, hyperemia, edema,
eosinophils, mucus retention Leukotrines,
prostaglandins brochoconstriction
vasodilatation, incr. permeability.

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Intrinsic nonallergic astma
Start in adult life, no evidence of
atopy Hyperactivity of airways is response to
nonspecific stimuli e.. Aspirin, cold,
exercise.. Constriction of bronchial wall IgE
levels normal, skin test normal Associated with
nasal polypi and bronchitis Pathogenesis
abnormal ß-adrenergic response
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Emphysema

• Def.
• Increased airiness of the lung tissue
• Abnormal permanent enlargement of gas exchange
  airways (?

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Emphysema

• Types
• non destructive (overinflation)
• destructive
• centriacinar (smoking, chronic
  bronchitis)
• panacinar (a1- antitrypsin deficiency)

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Smoking Emphysema

• numbers of neutrophils macrophages
  in smokers
• elastase activity
• macrophage elastase not blocked by a1-
  antitrypsin
• oxygen free radicals in smoke inhibit a1-
  antitrypsin

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Emphysema - clinical symptoms

• barrel chest, hypertrophy of intercostal
  muscles
• dyspnea, prolonged expiration
• productive cough (if infected)

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Emphysema - complications

• pneumothorax
• polycytemia
• cor pulmonale

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Emphysema - morphology

• Macroscopy
• cushion- like
• light
• pink
• voluminous
• pericardium overlapping
• bullae

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Emphysema - morphology

• Microscopy
• alveolar distension
• centriacinar
• panacinar
• thinning and destruction of alveolar septa
• reduction of capillary bed

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Interstitial emphysema
Def.  Entrance of air into the connective tissue
of the lung, mediastinum and soft tissue
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Interstitial emphysema

• Pathogenesis
• spontaneously
• increased intraalveolar pressue (cough,
  violent vomiting)
• iatrogenous - in patiens on respirator
• traumatic - lung trauma fractured ribs

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Interstitial emphysema

• Symptoms
• swelling of the neck and head
• crackling crepitation