Pathomor phology of kidney diseases

1. Pathomorphologyof kidney diseases As.-proff. V.Voloshyn According:prof. Bodnar Ya.Ya.; prof. Sorokina I.V.; Frank Netter

2. Main aim of lecture • To find out reasons and mechanisms of development of nephropathy. • To expose – macro- and microscopic displays of nephropathy. • To define the basic clinic-morphological displays of chronic kidney insufficiency. 2

3. Actuality • Illnesses of kidneys is group of illnesses which are the numeral and varied as in clinical so morphological displays and takes fifth line in morbidity of population after the traumatism, heart & vessels diseases, tumors and pulmonary pathologies. • Up to now the classification, diagnostics and treatments methods of nephropathy provocate the hot discussions between specialists of different type which diagnose and treat these illnesses 3

4. History of studies about pathomorphology of nephropathy • At the beginning of XVII century R. Brite offered the first classification of nephropathy • At the beginning of XX century clinicist Folgart and pathologist Far divided Brite’s illness on three groups: • - nephrites; • - nephroses; • - nephroscleroses. 4

5. Classification of nephropathy Structural-functional principle lies in the basis of modern clinic-morphologic classification of nephropathy. SELECT SUCH GROUPS OF NEPHROPATHY: • glomerulopathy; • tubulopathy; • tubulostromal nephropathy; • pyelonephritis; • nephrosclerosis • anomalies of development; • tumours. 5

6. Clinical syndromes at nephropathy • Urinary – is the recidive unpain hematuria, oliguria, proteinuria, leucocyturia, cylindruria; • Heart & vessels – is increase of arteriotony (especially diastolic), hypertrophy of the left ventricle of heart; • Nephrotic – is lipidemia, heavy proteinuria, warm white edemata of lower limbs. • Anaemic – toxic influence into marrow (firm (стійка) anaemia) 6

7. Introduction in practical work of nephrologists: - transskin kidney biopsy; - electronic microscopy; - іmmunomorphology The seat of pathomorphology in diagnostics of nephropathy 7

8. The norm structure of glomerulus 8

9. Glomerulopathy • Group of kidneys glomerules diseases which has congenital, primery-acquired and secondary-acquiredorigin. Their pathogeny consists in formation of immune complexes with their fixing on the basale membranes of glomerulus, or (in the case of autoimmune mechanism) formation of autoantibodies to the basale membrane. 9

10. Main types of damage of glomerulus 10

11. NORM Main types of damage of glomerulus 11

12. Classification of glomerulopathy Congenital glomerulopathy: • Alport’s syndrome • Congenital nephrotic syndrome • Inherited (спадковий) kidneyamyloidosis Acquired glomerulopathy: • Glomerulonephrytis with the minimal changing. • Postinfectial (poststreptococcic). • Subacute (semilunar). • Syndrome of Goodpascher. • Chronic glomerulonephrytis 12

13. Congenitalglomerulopathy • The syndrome of Alport is combination of lipoid tubulo- and interstitial nephropathy and glomerulopathy with the lowering of hearing and vision. • The inherited nephrotic syndrome is combination of minimum changing of podocytes, intracapillar productive glomerulonephrytis with polycystosis • Periodic illness – is combination of widespread amyloidosis with polyserositis 13

14. Glomerulonephrytis with the minimal changing is characterized by practically complete absence of changes in preparation which are investigated by light microscope. An electronic microscopy displays out the changing of small appendices of podocytes 14

15. Postinfection (poststreptococcy) glomerulonephrytis The most frequent reason is В-hemolytic streptococcus of A group. • Glomerulus are megascopic, anaemic, polymorphonuclear leucocytes are present in an interstitium. • An electronic microscopy displays out subephithelial deposits, proliferation and swelling of endotheliocytes 15

16. Subacute (quick progressic, malignant, semilunar glomerulonephrytis) “Large white kidney”, ”Large pied (diversity) (строкатa) kidney” ”large red kidney”. There are “crescent” (“halfmoons”) 16

17. Syndrome of Goodpascher • Quick progressicglomerulonephrytiswhich is combined with hemorrhagic-fibrinous pneumonia, and also with iron-deficient anemia. • Specific antibodies against the glicoprotein antigens of uncollogen portion of collogen IV are determined in a whey(сироватці) • In kidneys IgG and C3 are determined at immunofluorescence; At light microscopic – are “halfmoons”. 17

18. Chronic glomerulonephrytis • The independent disease is with the primary damage of glomerulus and secondary the pathological process extends into tubulus and stroma 18

19. Morphological forms of chronic glomerulonephrytis • Mesangioproliferativ • Membranous • Membranous-proliferativ or Mesangiocapillaric. • IgA-nephropathy (illness of Berje) 19

20. Mesangioproliferativic chronic glomerulonephrytis • Pathogeny is unknown • Proliferation of mesangiocytes is aspecific • Focal and segmental proliferation are simultaneous • The increasing of mesangial matrice At shick-reaction 20

21. Membranous chronic glomerulonephrytis Etiology • There is a idiopathic illness in 85 % of all patients. • Reasons of secondary membranous chronic glomerulonephritis are: • infections (syphilis, malaria, hepatitis B); • medicinal and chemical matters (penicillin, gold, mercury, heroin); • tumors (lymphadenomas, bronchogenic tumors); • sickle-cell anemia; • system lupus erythematosus. 21

22. Membranous chronic glomerulonephrytis • 1 stage - the subephithelial laying of deposits • 2 stages - the membranous and subephithelial laying of deposits • 3 stages - the intramembranous and mesangial laying of deposits • 4 stages - the diffuse laying of deposits with deformation of glomerular barrier 22

23. Mesangioproliferative chronic glomerulonephrytis • mesangial laying of deposits; • Ig G and C3 accumulates in mesangium more frequently • focal proliferation of mesangiocytes • it is observed at primary IgA-nephropathy 23

24. IgA-nephropathy (illness of Berje) • most widespread reason of chronic kidney insufficiency • IgA is revealed in a whey • mesangial laying of deposits • IgA accumulates in mesangium • proliferation of mesangiocytes 24

25. Secondary acquired glomerulonephrytes • Diabetic nephropathy • Amyloidosis of kidneys • Glomerulonephrytis at system lupus erytematosis 25

26. Secondary acquired glomerulopathy(Diabetic nephropathy) • Thickening of capillaries walls • Enlargement of volume of mesangium • Knot glome-rulosclerosis (changing of Kimmelsteel-Willson) 26

27. Secondary acquired glomerulopathy(Amyloidosis of kidneys) • Greasy kidney • Accumulation of АА-amyloid in mesangium, basale membranes of vessels and tubulis • Positive reaction on an amyloid • Phasicness of motion: latent, proteinuric, nephrotic, nitrogenemic 27

28. Secondary acquired glomerulopathy(System lupus erytematosus) • Laying of deposits in subepithelial and subendothelial layers • “wire loops” • formation of hematoxylin bodies 28

29. Secondary acquired glomerulopathy(hemolytic-uremic syndrome) • Subendothelial laying of deposits • Thickening of capillaries walls • Thromboses • Infarcts of kidney cortex 29

30. Thank you for attention! 30

31. Tubulopathy Acquired and inherited defeats of epithelium of nephron’s tubules, that is accompanied by violations of their function of concentration, reabsorbing and secretion. • Inherited tubulopathy - is predefined by the various forms of fermenthopathies. • Acquired tubulopathy are: (acute kidney insufficiency, kidney’s myelomas, podagrickidney) 31

32. Acute kidney insufficiency 32 Necrosis of tubules is the morphological substratum of acute kidney insufficiency • Distinguish ischemic (shock) and toxic acute kidney insufficiency. • Reasons of toxic acute kidney insufficiency are the action: ---heavy metals (lead, mercury, bismuth, arsenic), gold and uranium; ---organic solvents (chloroform, three-plus-onechlorous carbon); ---glycols (ethylen- and propilen-glycols); ---sulfanilamides, antibiotics (methacyclines, polimexinum); asteroid antiinflammation preparations; mercury diuretics

33. Tubulo-interstitial nephrite • Analgesic (analgin, aspirin in connection with a phenacetin): • Hypokaliumaemia • Uratic (podagric) • Hypercalciumemia (metastatic calciphylaxis); • Radiation 33

34. Pyelonephritis Successive heterospecific bacterial ascending or descending inflammation of kidney parenchima with predominant localization of pathological process in intermediate tissue. • Risk factors : short and wide urethra in women, stasis of urine of diverse etiology,urinary bladde-ureter reflux, age and hormonal state of patient • Women are ill mainly (1:5). 34

35. Pyelonephritis 35

36. Pathomorphology of chronic kidney insufficiency 36

37. Thank you for attention! 37