1. Organ Pathology Respiratory System - I Jaroslava Dušková Inst. Pathol.,1st Med. Faculty, Charles Univ. Prague Disorders of lung airiness

2. Lung Function  oxygenation  carbon dioxide excretion normal aeration of the lung tissue - conditio sine qua non

3. Disorders of Lung Airiness - atelectasis / collaps + emphysema coexistent in many pathology conditions !

4. Atelectasis / Collaps Def.: (cz) : A: inadequate expansion C: secondary loss of airiness (irrespective of age) (eng): A: inadequate expansion A: loss of lung volume

5. Atelectasis in Children Inadequate expansion: immature lung tissue – insufficient surfactant (S) production Clinical manifestation - RDS Regulation of (S) synthesis: corticoids, thyroxineinsuline

6. The main events and outcomes in ARDS

7. Atelectasis (collaps) in Adults - causes v obstruction v compression v microatelectases v contraction v chest wall restriction v flail chest

8. Atelectasis (collaps) in Adults – causes I v obstruction – mucopurulent plug (infection, mucoviscidosis), bronchomalacia – bronchiectasis, chronic bronchitis, astma foreign body, neoplasm (pores of Kohn) v compression – fluid, air, neoplasms in the pleural cavity v microatelectases – loss of surfactant v contraction – loss of lung elasticity mostly following fibrosis

9. Atelectasis (collaps) in Adults – causes II v chest wall restriction (obesity, scoliosis) v flail chest (several broken ribs)

10. 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 v increased concentration of chloride in sweat v decreased water content in excocrine secrets

11. Mucoviscidosis cystic fibrosis, fibrocystic disease Complications: – meconium ileus – steatorrhea – pancreatic fibrosis & cysts – bronchitis, bronchopneumonia, bronchiectasia – sterility

12. Bronchiectasis Def.: persistent abnormal dilation of the bronchus. Types: – cylindrical – saccular

13. 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

14. Chronic obstructive pulmonary disease (COPD) Def.: chronic productive cough lasting at least three months during two subsequent years Causes: SMOKING, air pollution

15. Chronic Bronchitis Morphology: –hyperplasia of mucin producing goblet cells (1GC :7CC 1GC:1CC) – epithelial hyperplasia (& dysplasia!) – inflammatory infiltrate

16. 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.

17. Asthma Clinical manifestation:  recurrent episodes of wheezing  breathlessness  chest tightness,  coughing at night or in the early morning Prologed attack – status asthmaticus

18. 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

19. 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

20. 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. Extrinsic allergic astma

21. 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

22. Emphysema Def.:  Increased airiness of the lung tissue  Abnormal permanent enlargement of gas exchange airways(?

23. Emphysema Types:  non destructive (overinflation)  destructive –centriacinar (smoking, chronic bronchitis…) –panacinar (α1- antitrypsin deficiency)

24. Smoking & Emphysema  numbers of neutrophils & macrophages in smokers  elastase activity  macrophage elastase not blocked by α1- antitrypsin  oxygen free radicals in smoke inhibit α1- antitrypsin

25.  barrel chest, hypertrophy of intercostal muscles  dyspnea, prolonged expiration  productive cough (if infected) Emphysema - clinical symptoms

26.  pneumothorax  polycytemia  cor pulmonale Emphysema - complications

27. Macroscopy  cushion- like  light  pink  voluminous  pericardium overlapping  bullae Emphysema - morphology

28. Microscopy  alveolar distension  centriacinar  panacinar –thinning and destruction of alveolar septa  reduction of capillary bed Emphysema - morphology

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

30. Pathogenesis:  spontaneously increased intraalveolar pressue (cough, violent vomiting)  iatrogenous - in patiens on respirator  traumatic - lung trauma – fractured ribs Interstitial emphysema

31. Symptoms  swelling of the neck and head  crackling crepitation Interstitial emphysema