organ pathology respiratory system - i jaroslava dušková inst. pathol.,1st med. faculty, charles...
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Organ Pathology
Respiratory System - I
Jaroslava Dušková
Inst. Pathol. ,1st Med. Faculty, Charles Univ. Prague
Disorders of lung airiness
Lung Function
oxygenation
carbon dioxide excretion
normal aeration of the lung tissue - conditio sine qua non
Disorders of Lung Airiness
- atelectasis / collaps
+ emphysema
coexistent in many pathology conditions !
Atelectasis / Collaps Def.:
(cz) : A: inadequate expansion C: secondary loss of airiness
(irrespective of age)
(eng): A: inadequate expansion A: loss of lung volume
Atelectasis in ChildrenInadequate expansion:immature lung tissue – insufficient
surfactant (S) production Clinical manifestation - RDSRegulation of (S) synthesis:
corticoids, thyroxine insuline
The main events and outcomes in ARDS
Atelectasis (collaps) in Adults - causes
obstruction
compression
microatelectases
contraction
chest wall
restriction
flail chest
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
Atelectasis (collaps) in Adults – causes II
chest wall restriction (obesity, scoliosis)
flail chest (several broken ribs)
Mucoviscidosis cystic fibrosis, fibrocystic disease
autosomal recessive 7th chromosome2-5% 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
Mucoviscidosis cystic fibrosis, fibrocystic disease
Complications:– meconium ileus– steatorrhea– pancreatic fibrosis & cysts– bronchitis, bronchopneumonia,
bronchiectasia– sterility
Bronchiectasis
Def.:
persistent abnormal dilation of the bronchus.
Types: – cylindrical– saccular
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
Chronic obstructive pulmonary disease (COPD)
Def.:
chronic productive cough lasting at least three months during two subsequent years
Causes: SMOKING, air pollution
Chronic Bronchitis
Morphology:
– hyperplasia of mucin producing goblet cells (1GC :7CC 1GC:1CC)
– epithelial hyperplasia (& dysplasia!)– inflammatory infiltrate
AsthmaDef.:
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.
AsthmaClinical manifestation: recurrent episodes of wheezing breathlessness chest tightness, coughing at night or in the early morning
Prologed attack – status asthmaticus
Intrinsic abnormal
β-adrenergic reaction IgE normal causes:
– coolness– effort– infection– aspirin…
Extrinsic type I hypersensitivity IgE causes:
– dust– pollen– animal fur– drugs– foodsuff….
Asthma - types
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 membraneSputum:
– Charcot-Leyden crystals derived from eosinophils– Curshman spirals – glycoproteins– Creola bodies
Start in childhood, evidence of atopyAllergens - household dust, organic dusts, pollens – grasses and trees, animal fur, food products, drugs Reagin – mediated type I hypersensitivity reactionSerum IgE increased, Skin tests against antigens positive Mast cell degranulation – histamin, bradykininSmooth muscle contraction, hyperemia, edema, eosinophils, mucus retention Leukotrines, prostaglandins – brochoconstriction vasodilatation, incr. permeability.
Extrinsic allergic 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 β-adrenergic response
Intrinsic nonallergic astma
Emphysema
Def.:
Increased airiness of the lung tissue
Abnormal permanent enlargement of gas exchange airways (?
Emphysema
Types: non destructive (overinflation) destructive
–centriacinar (smoking, chronic
bronchitis…)–panacinar (α1- antitrypsin
deficiency)
Smoking & Emphysema numbers of neutrophils &
macrophages in smokers elastase activity macrophage elastase not blocked by
α1- antitrypsinoxygen free radicals in smoke inhibit α1-
antitrypsin
barrel chest, hypertrophy of
intercostal muscles
dyspnea, prolonged
expiration
productive cough (if infected)
Emphysema - clinical symptoms
pneumothorax polycytemia cor pulmonale
Emphysema - complications
Macroscopy cushion- like light pink voluminous pericardium overlapping bullae
Emphysema - morphology
Microscopy alveolar distension centriacinar panacinar
– thinning and destruction of alveolar septa
reduction of capillary bed
Emphysema - morphology
Def.: Entrance of air into the connective tissue of the lung, mediastinum and soft tissue
Interstitial emphysema
Pathogenesis:
spontaneously increased intraalveolar pressue
(cough, violent vomiting) iatrogenous - in patiens on respirator
traumatic - lung trauma – fractured ribs
Interstitial emphysema
Symptoms
swelling of the neck and
head
crackling crepitation
Interstitial emphysema