Normal kidney

Ultrasructure of normal Ultrasructure of normal kidneykidney

Clinical presentationsClinical presentations

AzotemiaAzotemia UremiaUremia Nephritic syndromeNephritic syndrome Nephrotic syndromeNephrotic syndrome HematuriaHematuria ProteinuriaProteinuria Acute renal failureAcute renal failure Chronic renal failureChronic renal failure

Nephrotic syndromeNephrotic syndrome

S/SS/S– Heavy proteinuria (3.5 gm%) and hypoalbuminemiaHeavy proteinuria (3.5 gm%) and hypoalbuminemia– EdemaEdema– InfectionsInfections– ThrombosisThrombosis– Hyperlipidemia and lipiduria.Hyperlipidemia and lipiduria.

Disorders with thickening of BM and mesangium.Disorders with thickening of BM and mesangium. Associated common glomerular diseasesAssociated common glomerular diseases

– Membranoproliferative glomerulonephritisMembranoproliferative glomerulonephritis– Membranous glomerulonephritisMembranous glomerulonephritis– Minimal changeMinimal change

Nephritic syndromeNephritic syndrome

Sudden onsetSudden onset S/S: S/S:

– Hematuria, RBC casts.Hematuria, RBC casts.– Proteinuria, mild to moderate, not nephrotic Proteinuria, mild to moderate, not nephrotic

range.range.– HypertensionHypertension

Glomerular diseases with cellular Glomerular diseases with cellular proliferation.proliferation.

Hallmark disease: post streptococcal Hallmark disease: post streptococcal glomerulonephritisglomerulonephritis

Acute Renal failureAcute Renal failure

Kidney stops working.Kidney stops working. Oliguria to anuria.Oliguria to anuria. ReversibleReversible Common cause- acute perfusion failureCommon cause- acute perfusion failure Gross and M/E – Renal cortical necrosisGross and M/E – Renal cortical necrosis Lab Data: increased BUN and Serum CrLab Data: increased BUN and Serum Cr

Hyperkalemia, metabolic acidosis, urinary sodium Hyperkalemia, metabolic acidosis, urinary sodium loss, same plasma and urine osmolality. loss, same plasma and urine osmolality.

Chronic renal failureChronic renal failure

IrreversibleIrreversible Slow progressionSlow progression S/S Uremia, polyuria, bone disease, S/S Uremia, polyuria, bone disease,

anemia. Metabolic compensation.anemia. Metabolic compensation. Common causes: hypertension and Common causes: hypertension and

diabetes.diabetes. Leads to end-stage renal disease. Leads to end-stage renal disease.

End stage renal diseaseEnd stage renal disease

Small, shrunken and Small, shrunken and granulargranular

Misnomer-chronic Misnomer-chronic glomerulonephritisglomerulonephritis

D/D Chronic D/D Chronic pyelonephritispyelonephritis

M/E hyalanized M/E hyalanized glomeruli, tubular glomeruli, tubular atrophy and interstitial atrophy and interstitial fibrosis.fibrosis.

Congenital DisordersCongenital Disorders

Renal agenesis- usually unilateralRenal agenesis- usually unilateral HypoplasiaHypoplasia Ectopic KidneysEctopic Kidneys Horseshoe kidneyHorseshoe kidney

Horse shoe kidney Double ureter

Cystic diseases of kidneyCystic diseases of kidney

Simple cystSimple cyst Adult polycystic kidney diseaseAdult polycystic kidney disease Childhood polycystic kidney diseaseChildhood polycystic kidney disease

Simple renal cyst Adult Polycystic kidney

Adult polycystic kidney Adult polycystic kidney diseasedisease Autosomal dominantAutosomal dominant BilateralBilateral Does not produce symptoms until the 4Does not produce symptoms until the 4thth

decadedecade Slowly progressiveSlowly progressive Associated findingsAssociated findings

– Liver cystsLiver cysts– berry aneurysms.berry aneurysms.

Autosomal recessive kidney disease

Autosomal recessive

Presents early

Bilateral

Liver cysts with proliferation of bile ducts

Renal pathologyRenal pathology

Diseases of the following:Diseases of the following:

Glomeruli- glomerulonephritisGlomeruli- glomerulonephritis( classified on the basis of histopathology)( classified on the basis of histopathology)

VesselsVessels InterstitiumInterstitium TubulesTubules

Pathogenesis of glomerular Pathogenesis of glomerular diseasesdiseases

Immunological:Immunological:– Antibodies reacting with glomerular antigensAntibodies reacting with glomerular antigens

Trapped circulating agent (Membranous)Trapped circulating agent (Membranous) Anti GBM antibodies (Good pasture's disease)Anti GBM antibodies (Good pasture's disease)

– Circulating immune complexes (Post streptococcalCirculating immune complexes (Post streptococcal VascularVascular

– HypertensionHypertension– DiabetesDiabetes

Foreign materialForeign material– AmyloidosisAmyloidosis

Glomerular response to Glomerular response to damagedamageHistological changesHistological changes

HypercellularityHypercellularity BM thickeningBM thickening Hyalinization and sclerosisHyalinization and sclerosis TerminologyTerminology

– Diffuse/focalDiffuse/focal– Global/segmentalGlobal/segmental

Acute glomerulonephritisAcute glomerulonephritis

Acute proliferative(poststreptococcal) Acute proliferative(poststreptococcal) glomerulonephritisglomerulonephritis

Rapidly progressive (crescentric) Rapidly progressive (crescentric) glomerulonephritis.glomerulonephritis.– Reaction to severe glomerular damageReaction to severe glomerular damage– Leakage of fibrin, leading to epithelial cell Leakage of fibrin, leading to epithelial cell

proliferation and crescent formationproliferation and crescent formation– Poor prognosis Poor prognosis

Acute diffuse proliferative Acute diffuse proliferative glomerulonephritisglomerulonephritis 1-2 wks post streptococcal1-2 wks post streptococcal Usually in childrenUsually in children Usually mild and transientUsually mild and transient Clinical presentation- nephritic syndromeClinical presentation- nephritic syndrome DiagnosisDiagnosis

– Histology- increased cellularityHistology- increased cellularity– IF: IgG and C3IF: IgG and C3– EM- granular subepithelial depositsEM- granular subepithelial deposits

Rx supportiveRx supportive

Crescentric Crescentric glomerulonephritisglomerulonephritis

Minimal change diseaseMinimal change disease

In childrenIn children CPCP -Nephrotic syndrome-Nephrotic syndrome HistologyHistology

– H and EH and E NormalNormal– EMEM Fusion of foot processFusion of foot process– IFIF

Rx steroidsRx steroids

Membranous nephropathyMembranous nephropathy Usually adultsUsually adults CP- nephrotic CP- nephrotic

syndromesyndrome Etiology- idiopathic/ Etiology- idiopathic/

secondary to multiple secondary to multiple causescauses– InfectiveInfective– DrugsDrugs– TumorsTumors– SLESLE

DiagnosisDiagnosis H and E- diffuse and H and E- diffuse and

global, BM and global, BM and mesangial thickeningmesangial thickening

IF- IgG and IF- IgG and complementcomplement

EM- Subepithelial EM- Subepithelial deposits (spikes)deposits (spikes)

Membranoproliferative Membranoproliferative glomerulonephritisglomerulonephritis Children and adultsChildren and adults CP- mixed nephrotic and nephriticCP- mixed nephrotic and nephritic Etiology- idiopathic or secondary e.g. SLEEtiology- idiopathic or secondary e.g. SLE Histology- Type 1 and Type 2Histology- Type 1 and Type 2

Type I- increased lobularity of the glomeruli, because of Type I- increased lobularity of the glomeruli, because of mesangial proliferation ( mesangial interposition-tram track mesangial proliferation ( mesangial interposition-tram track appearance)appearance)

EMEM Subendothelial deposits Subendothelial deposits IFIF IgG/ IgM and C3IgG/ IgM and C3

Type II- mesangial proliferation not that prominentType II- mesangial proliferation not that prominent EMEM dense deposit diseasedense deposit disease IFIF C3C3

Course: progressive, poor prognosisCourse: progressive, poor prognosis

Focal glomerulosclerosisFocal glomerulosclerosis

Children and adultsChildren and adults CP- nephrotic syndromeCP- nephrotic syndrome Etiology- Idiopathic/ secondary e.g. Etiology- Idiopathic/ secondary e.g.

HIV.HIV. Poor prognosisPoor prognosis

Kidney and vascular diseaseKidney and vascular disease

Central perfusion failureCentral perfusion failure Large vessel disease e.g. atherosclerosis-Large vessel disease e.g. atherosclerosis-

thromboembolic, renal artery stenosisthromboembolic, renal artery stenosis Small vessel disease e.g. hypertension, Small vessel disease e.g. hypertension,

diabetes and vasculitisdiabetes and vasculitis Pathogenesis- affects glomerular infiltration Pathogenesis- affects glomerular infiltration

and ischemic tubular atrophyand ischemic tubular atrophy Course- usually chronicCourse- usually chronic

Renal infarctsRenal infarcts

Causes:Causes:– accelerated accelerated

hypertensionhypertension– VasculitisVasculitis– EmboliEmboli

Hypertension and kidneyHypertension and kidney

Benign hypertensive nephrosclerosisBenign hypertensive nephrosclerosis– Granular kidneyGranular kidney– Arteriolar hyalinizationArteriolar hyalinization

Malignant hypertensionMalignant hypertension– Flea bitten kidneyFlea bitten kidney– Fibrinoid necrosis of the vesselsFibrinoid necrosis of the vessels

Granular kidney

Hyalanized arterioles

Malignant HypertensionMalignant Hypertension

Fibrinoid necrosis Hyperplastic arteriopathy

Diabetes and kidneyDiabetes and kidney

Diabetes most common cause of end Diabetes most common cause of end stage renal failure.stage renal failure.

Diabetic vascular disease- AS/hyaline Diabetic vascular disease- AS/hyaline arteriosclerosis.arteriosclerosis.

Diabetic glomerulosclerosisDiabetic glomerulosclerosis– Mesangial matrix formationMesangial matrix formation

Diffuse glomerulosclerosisDiffuse glomerulosclerosis Nodular glomerulosclerosis (Kimmelstiel-Nodular glomerulosclerosis (Kimmelstiel-

wilson disease)wilson disease)– BM thickeningBM thickening– Exudative lesions- fibrin capExudative lesions- fibrin cap

Increased infection- PyelonephritisIncreased infection- Pyelonephritis Papillary necrosisPapillary necrosis

Diseases of renal tubules Diseases of renal tubules and interstitiumand interstitium

– Pyelonephritis- acute and chronicPyelonephritis- acute and chronic– Acute tubular necrosisAcute tubular necrosis– Interstitial nephritisInterstitial nephritis

acute and chronicacute and chronic interstitial inflammationinterstitial inflammation Drugs like analgesics and antibioticsDrugs like analgesics and antibiotics

– Metabolic damage to tubules for eg Metabolic damage to tubules for eg calcium and uratecalcium and urate

– Functional tubular abnormalityFunctional tubular abnormality

Acute tubular necrosisAcute tubular necrosis

Reversible acute renal Reversible acute renal failure.failure.

CausesCauses– IschemiaIschemia– DrugsDrugs

Clinical stagesClinical stages– Initiating- oliguriaInitiating- oliguria– Maintenance- polyuriaMaintenance- polyuria– Recovery Recovery

Acute PyelonephritisAcute Pyelonephritis

Bacterial infectionBacterial infection Routes – ascending infection and hematogenousRoutes – ascending infection and hematogenous Predisposing factors: pregnancy, DM, stones, Predisposing factors: pregnancy, DM, stones,

tumor, vesicoureteral refluxtumor, vesicoureteral reflux S/S – pain, fever, often with lower urinary S/S – pain, fever, often with lower urinary

symptoms.symptoms. Gross: small cortical abscessGross: small cortical abscess M/E: neutrophils presentM/E: neutrophils present Course:Course: septicemia, papillary necrosis, peri and septicemia, papillary necrosis, peri and

pyonephrosis.pyonephrosis.

Chronic PyelonephritisChronic Pyelonephritis

Reflux associated/ obstructive.Reflux associated/ obstructive. Gross: irregular cortical scarring, Gross: irregular cortical scarring,

distorted renal calyxdistorted renal calyx M/E: chronic inflammation, fibrosis M/E: chronic inflammation, fibrosis

and tubular atrophy.and tubular atrophy. Specific – Tuberculous pyelonephritis Specific – Tuberculous pyelonephritis

Chronic pyelonephritisChronic pyelonephritis

HYDRONEPHROSISHYDRONEPHROSIS

Dilatation of renal Dilatation of renal pelvis and calyces pelvis and calyces and parenchymal and parenchymal atrophy due to atrophy due to urinary tract urinary tract obstruction.obstruction.

Renal stonesRenal stones FactorsFactors

– Increased conc. of solutesIncreased conc. of solutes– change in urinary Phchange in urinary Ph– decreased urinary volumedecreased urinary volume– infectioninfection

TypesTypes– Calcium (hypercalcinuria, hypercalcemia, excess)Calcium (hypercalcinuria, hypercalcemia, excess)– Magnesium ammonium phosphate stones Magnesium ammonium phosphate stones

(alkaline urine due to infection)(alkaline urine due to infection)– Uric acid stones ( gout, leukemias, acidic urine) Uric acid stones ( gout, leukemias, acidic urine)

Renal tumorsRenal tumors

Benign tumors common.Benign tumors common. Common malignant tumors:Common malignant tumors:

– Renal adenocarcinoma in adults (90%)Renal adenocarcinoma in adults (90%)– Wilm’s tumor in children.Wilm’s tumor in children.

CP- hematuria, pain, mass, metastasis, CP- hematuria, pain, mass, metastasis, paraneoplastic. paraneoplastic.

Prognosis depends on the stage.Prognosis depends on the stage. EtiologyEtiology - cytogenetic abnormalities- cytogenetic abnormalities

Renal Cell CarcinomaRenal Cell Carcinoma

66thth to 7 to 7thth decade decade Male predominance 3:1Male predominance 3:1 Yellow round mass, 3-15 cmYellow round mass, 3-15 cm Often invade renal vein and extend Often invade renal vein and extend

into the inferior vena cavainto the inferior vena cava Chromosome 13Chromosome 13

Wilm’s tumorWilm’s tumor

Childhood tumor composed of Childhood tumor composed of primitive tissue.primitive tissue.

Chromosome 1-loss of cancer Chromosome 1-loss of cancer suppression gene WT-1suppression gene WT-1

Combination of radiotherapy, Combination of radiotherapy, nephrectomy and chemotherapy –nephrectomy and chemotherapy –good results good results