PathomorPathomorphologyphology ofof kidney kidney diseasesdiseases

As.-proff. V.Voloshyn

According:prof. Bodnar Ya.Ya.; prof. Sorokina I.V.; Frank Netter

Main aim of lectureMain aim of lecture

1.1.To find out reasons and To find out reasons and mechanisms of development of mechanisms of development of nephropathy.nephropathy.

2.2.To expose – macro- and To expose – macro- and microscopic displays of nephropathymicroscopic displays of nephropathy..

3.3.To define the basic clinic-To define the basic clinic-morphological displays of chronic morphological displays of chronic kidney insufficiencykidney insufficiency. .

2

ActualityActualityIllnesses of kidneys Illnesses of kidneys is group of is group of

illnesses which are the numeral and varied illnesses which are the numeral and varied as in clinical so morphological displays and as in clinical so morphological displays and takes fifth line in morbidity of population takes fifth line in morbidity of population after the traumatism, heart & vessels after the traumatism, heart & vessels diseases, tumors and pulmonary pathologies.diseases, tumors and pulmonary pathologies.

Up to now the classification, diagnostics Up to now the classification, diagnostics and treatments methods of nephropathy and treatments methods of nephropathy provocate the hot discussions between provocate the hot discussions between specialists of different type which diagnose specialists of different type which diagnose and treat these illnessesand treat these illnesses

3

History of studies about History of studies about pathomorphology of nephropathypathomorphology of nephropathy

At the beginning of XVII century R. At the beginning of XVII century R. Brite offered the first classification of Brite offered the first classification of nephropathy nephropathy

At the beginning of XX century At the beginning of XX century clinicist Folgart and pathologist Far clinicist Folgart and pathologist Far divided Brite’s illness on three divided Brite’s illness on three groups:groups: - nephrites;- nephrites; - nephroses;- nephroses; - nephroscleroses.- nephroscleroses.

4

Classification of nephropathyClassification of nephropathy

Structural-functional principle lies in the basis Structural-functional principle lies in the basis of modern clinic-morphologic classification of of modern clinic-morphologic classification of nephropathynephropathy ..

SELECT SUCH GROUPS OF NEPHROPATHY:SELECT SUCH GROUPS OF NEPHROPATHY:glomerulopathy;glomerulopathy;tubulopathy;tubulopathy;tubulostromal nephropathy;tubulostromal nephropathy;pyelonephritis;pyelonephritis;nephrosclerosisnephrosclerosisanomalies of development;anomalies of development;tumours.tumours.

5

Clinical syndromes at Clinical syndromes at nephropathynephropathy

Urinary – is the recidive unpain Urinary – is the recidive unpain hematuria, oliguria, proteinuria, hematuria, oliguria, proteinuria, leucocyturia, cylindruria;leucocyturia, cylindruria;

Heart & vessels – is increase of Heart & vessels – is increase of arteriotony (especially diastolic), arteriotony (especially diastolic), hypertrophy of the left ventricle of hypertrophy of the left ventricle of heart;heart;

Nephrotic – is lipidemia, heavy Nephrotic – is lipidemia, heavy proteinuria, warm white edemata of proteinuria, warm white edemata of lower limbs.lower limbs.

Anaemic – toxic influence into marrow Anaemic – toxic influence into marrow (firm (firm (стійка)(стійка) anaemia) anaemia)

6

The seat of pathomorphology The seat of pathomorphology in diagnostics of nephropathyin diagnostics of nephropathy

Introduction in practical work of Introduction in practical work of nephrologists:nephrologists:

- - transskin kidney biopsy;transskin kidney biopsy; - electronic microscopy;- electronic microscopy; - іmmunomorphology- іmmunomorphology

7

The norm structure of glomerulusThe norm structure of glomerulus

8

GlomerulopathyGlomerulopathy Group of kidneys glomerules diseases Group of kidneys glomerules diseases

which has congenital, which has congenital, primery-primery-acquiredacquired and secondary-and secondary-acquiredacquired origin. Their origin. Their pathogeny consists in formation of pathogeny consists in formation of immune complexes with their fixing on immune complexes with their fixing on the basale membranes of glomerulus, or the basale membranes of glomerulus, or (in the case of autoimmune mechanism) (in the case of autoimmune mechanism) formation of autoantibodies to the basale formation of autoantibodies to the basale membrane.membrane.

9

Main types of damage of glomerulusMain types of damage of glomerulus

10

Main types of damage of glomerulusMain types of damage of glomerulus

11

NORM

Congenital Congenital glomerulopathyglomerulopathy ::1.1. Alport’s syndrome Alport’s syndrome 2.2. Congenital nephrotic syndromeCongenital nephrotic syndrome3.3. Inherited Inherited (спадковий)(спадковий) kidney kidney amyloidosisamyloidosis

Acquired Acquired glomerulopathyglomerulopathy::1.1. Glomerulonephrytis with the minimal changing.Glomerulonephrytis with the minimal changing.2.2. Postinfectial (poststreptococcic).Postinfectial (poststreptococcic).3.3. Subacute (semilunar).Subacute (semilunar).4.4. Syndrome of Goodpascher.Syndrome of Goodpascher.5.5. Chronic glomerulonephrytisChronic glomerulonephrytis

Classification of Classification of glomerulopathyglomerulopathy

12

CongenitalCongenital glomerulopathyglomerulopathy

The syndrome of Alport is The syndrome of Alport is combination of lipoid tubulo- and combination of lipoid tubulo- and interstitial nephropathy and interstitial nephropathy and glomerulopathy with the lowering of glomerulopathy with the lowering of hearing and vision.hearing and vision.

The inherited nephrotic syndrome The inherited nephrotic syndrome is combination of minimum is combination of minimum changing of podocytes, intracapillar changing of podocytes, intracapillar productive glomerulonephrytis with productive glomerulonephrytis with polycystosispolycystosis

Periodic illness – is combination of Periodic illness – is combination of widespread amyloidosis with widespread amyloidosis with polyserositispolyserositis

13

GlomerulonephrytisGlomerulonephrytis with with the minimal changingthe minimal changing

is characterized by is characterized by practically complete practically complete absence of changes absence of changes in preparation which in preparation which are investigated by are investigated by light microscope.light microscope.An electronic An electronic

microscopy displays microscopy displays out the changing of out the changing of small appendices of small appendices of podocytespodocytes

14

Postinfection (poststreptococcy) Postinfection (poststreptococcy) glomerulonephrytisglomerulonephrytis

The most frequent reason is The most frequent reason is В-hemolytic streptococcus В-hemolytic streptococcus

of A group.of A group.Glomerulus are Glomerulus are megascopic, anaemic, megascopic, anaemic, polymorphonuclear polymorphonuclear leucocytes are present in an leucocytes are present in an interstitium. interstitium.

An electronic microscopy An electronic microscopy displays out subephithelial displays out subephithelial deposits, proliferation and deposits, proliferation and swelling of endotheliocytesswelling of endotheliocytes

15

Subacute Subacute (quick progressic, malignant, (quick progressic, malignant, semilunar semilunar glomerulonephrytis)glomerulonephrytis)

““Large white kidney”,Large white kidney”,

””Large pied (diversity) Large pied (diversity) (строкатa) kidney”(строкатa) kidney”

””large red kidney”.large red kidney”.

There are “crescent” There are “crescent” (“halfmoons”)(“halfmoons”)

16

Syndrome of GoodpascherSyndrome of Goodpascher Quick progressicQuick progressic

glomerulonephrytisglomerulonephrytis which which is combined with hemorrhagic-is combined with hemorrhagic-fibrinous pneumonia, and also fibrinous pneumonia, and also with iron-deficient anemia.with iron-deficient anemia.

Specific antibodies against Specific antibodies against the glicoprotein antigens of the glicoprotein antigens of uncollogen portion of collogen uncollogen portion of collogen IV are determined in a wheyIV are determined in a whey (сироват(сироватціці))

In kidneys IgG and C3 are In kidneys IgG and C3 are determined at determined at immunofluorescence; At light immunofluorescence; At light microscopic – are “halfmoons”.microscopic – are “halfmoons”.

17

Chronic Chronic glomerulonephrytisglomerulonephrytis The independent disease is with the The independent disease is with the

primary damage of glomerulus and primary damage of glomerulus and secondary the pathological process secondary the pathological process extends into tubulus and stromaextends into tubulus and stroma

18

Morphological forms of Morphological forms of chronic chronic glomerulonephrytisglomerulonephrytis

MesangioproliferativMesangioproliferativ MembranousMembranous Membranous-proliferativ or Membranous-proliferativ or

Mesangiocapillaric.Mesangiocapillaric. IgA-nephropathy (illness of Berje)IgA-nephropathy (illness of Berje)

19

Mesangioproliferativic chronic Mesangioproliferativic chronic glomerulonephrytisglomerulonephrytis

Pathogeny is Pathogeny is unknownunknownProliferation of Proliferation of

mesangiocytes is mesangiocytes is aspecificaspecificFocal and segmental Focal and segmental

proliferation are proliferation are simultaneoussimultaneousThe increasing of The increasing of

mesangial matrice At mesangial matrice At shick-reactionshick-reaction

20

Membranous chronic Membranous chronic glomerulonephrytisglomerulonephrytis

EtiologyEtiologyThere is a idiopathic illness in 85 % of all There is a idiopathic illness in 85 % of all patients. patients. Reasons of secondary membranous chronic Reasons of secondary membranous chronic glomerulonephritisglomerulonephritis are are: :

infections (syphilis, malaria, hepatitis B);infections (syphilis, malaria, hepatitis B); medicinal and chemical matters (penicillin, gold, medicinal and chemical matters (penicillin, gold,

mercury, heroin);mercury, heroin); tumors (lymphadenomas, bronchogenic tumors);tumors (lymphadenomas, bronchogenic tumors); sickle-cellsickle-cell anemia; anemia; system lupus erythematosus.system lupus erythematosus.

21

Membranous chronic Membranous chronic glomerulonephrytisglomerulonephrytis

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

Mesangioproliferative chronic Mesangioproliferative chronic glomerulonephrytisglomerulonephrytis

mesangial laying of deposits; mesangial laying of deposits;

Ig G and C3 accumulates in Ig G and C3 accumulates in mesangium more frequentlymesangium more frequently

focal proliferation of focal proliferation of mesangiocytesmesangiocytes

it is observed at primary it is observed at primary IgA-nephropathyIgA-nephropathy

23

IgA-nephropathy (illness of Berje)IgA-nephropathy (illness of Berje)

most widespread reason of chronic most widespread reason of chronic kidney insufficiencykidney insufficiencyIgA is revealed in a wheyIgA is revealed in a wheymesangial laying of depositsmesangial laying of depositsIgA accumulates in mesangiumIgA accumulates in mesangium proliferation of mesangiocytesproliferation of mesangiocytes

24

Secondary acquired Secondary acquired glomerulonephrytesglomerulonephrytes

Diabetic nephropathyDiabetic nephropathy Amyloidosis of kidneysAmyloidosis of kidneys GlomerulonephrytisGlomerulonephrytis at system lupus at system lupus

erytematosiserytematosis

25

Secondary acquired glomerulopathySecondary acquired glomerulopathy(Diabetic nephropathy)(Diabetic nephropathy)

Thickening of capillaries wallsThickening of capillaries walls

Enlargement of volume of Enlargement of volume of mesangiummesangium

Knot glome-rulosclerosis Knot glome-rulosclerosis (changing of Kimmelsteel-(changing of Kimmelsteel-Willson)Willson)

26

Secondary acquired glomerulopathySecondary acquired glomerulopathy(Amyloidosis of kidneys)(Amyloidosis of kidneys)

Greasy kidneyGreasy kidneyAccumulation of АА-Accumulation of АА-amyloid in amyloid in mesangium, basale mesangium, basale membranes of membranes of vessels and tubulisvessels and tubulisPositive reaction on Positive reaction on an amyloidan amyloidPhasicness of Phasicness of motion: latent, motion: latent, proteinuric, nephrotic, proteinuric, nephrotic, nitrogenemicnitrogenemic

27

Secondary acquired glomerulopathySecondary acquired glomerulopathy(System lupus erytematosus)(System lupus erytematosus)

Laying of deposits in Laying of deposits in subepithelial and subepithelial and subendothelial layerssubendothelial layers

““wire loopwire loops”s”

formation of hematoxylin formation of hematoxylin bodiesbodies

28

Secondary acquired glomerulopathySecondary acquired glomerulopathy(hemolytic-uremic syndrome)(hemolytic-uremic syndrome)

Subendothelial laying of Subendothelial laying of depositsdeposits

Thickening of capillaries Thickening of capillaries wallswalls

ThrombosesThromboses

Infarcts of kidney cortexInfarcts of kidney cortex

29

Thank you for

attention!

30

TubulopathyTubulopathy

Acquired and inherited defeats of epithelium of Acquired and inherited defeats of epithelium of nephron’s tubules, that is accompanied by nephron’s tubules, that is accompanied by violations of their function of concentration, violations of their function of concentration,

reabsorbing and secretion.reabsorbing and secretion. Inherited tubulopathy - is Inherited tubulopathy - is predefined by the predefined by the

various forms of fermenthopathies.various forms of fermenthopathies.

Acquired Acquired tubulopathy are:tubulopathy are: (acute kidney insufficiency, kidney’s (acute kidney insufficiency, kidney’s

myelomas, myelomas, podagricpodagric kidney)kidney)

31

Acute kidney insufficiencyAcute kidney insufficiencyNecrosis of tubules is the Necrosis of tubules is the

morphological substratum of acute morphological substratum of acute kidney insufficiencykidney insufficiencyDistinguish ischemic (shock) and Distinguish ischemic (shock) and toxic acute kidney insufficiency.toxic acute kidney insufficiency.Reasons of toxic acute kidney Reasons of toxic acute kidney insufficiency are the action:insufficiency are the action:---heavy metals (lead, mercury, ---heavy metals (lead, mercury,

bismuth, arsenic), gold and bismuth, arsenic), gold and uranium;uranium;---organic solvents (chloroform, ---organic solvents (chloroform,

three-plus-onechlorous carbon); three-plus-onechlorous carbon); ---glycols (ethylen- and propilen----glycols (ethylen- and propilen-

glycols);glycols);---sulfanilamides, antibiotics ---sulfanilamides, antibiotics

(methacyclines, polimexinum); (methacyclines, polimexinum); asteroid antiinflammation asteroid antiinflammation preparations; mercury diureticspreparations; mercury diuretics

32

Tubulo-interstitial nephriteTubulo-interstitial nephrite

Analgesic Analgesic (analgin, aspirin in (analgin, aspirin in connection with a connection with a phenacetin):phenacetin):HypokaliumaemiaHypokaliumaemiaUratic (podagric)Uratic (podagric)Hypercalciumemia Hypercalciumemia (metastatic (metastatic calciphylaxis);calciphylaxis);RadiationRadiation

33

PyelonephritisPyelonephritisSuccessive heterospecific bacterial ascending Successive heterospecific bacterial ascending

or descending inflammation of kidney parenchima or descending inflammation of kidney parenchima with predominant localization of pathological with predominant localization of pathological process in intermediate tissue.process in intermediate tissue.

Risk factors : short and wide urethra in women, Risk factors : short and wide urethra in women, stasis of urine of diverse etiology,urinary bladde-ureter stasis of urine of diverse etiology,urinary bladde-ureter reflux, age and hormonal state of patientreflux, age and hormonal state of patient

Women are ill mainly (1:5).Women are ill mainly (1:5).

34

PyelonephritisPyelonephritis

35

Pathomorphology of chronic Pathomorphology of chronic kidney insufficiencykidney insufficiency

36

Organ, tissue Morphological changes

Skin Grey-earthy color as a result of accumulation of urochrome. Uremic powder. Pointed hemorrhages

Mucus membrane

Catarrhal, fibrinous or fibrinous-hemorragic gastritis, enteritis, colitis, laryngitis, tracheitis

Lungs Swelling, serosal, fibrinous or fibrinous-hemorragic focal pneumonia

Serosal membrane

serosal- fibrinous or fibrinous pericarditis, pleuritis, peritonitis

Cerebrum Swelling, hemorrhages, areas of softening

Heart Warty endocarditis, toxic myocarditis

Thank you for

attention!

37