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Diseases of the Kidney
Janos Vasko
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•Congenital anomalies
•Glomerular diseases
•Tubulointerstitial diseases
•Infections
•Vascular diseases
•Stones
•Tumours
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POLYCYSTIC KIDNEY DISEASE
• INFANTILE TYPE
• ADULT TYPEAutosomal dominantCysts of varying size in both kidneysNormal parenchyma between cystsCysts in liver, pancreas, spleenIntracranial aneurysmas
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SIMPLE KIDNEY CYSTS
• Common findings at autopsy
• 50% at 50 years
• No clinical significance (except cancer
diagnosis)
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GLOMERULAR DISEASES• Nephrotic syndrome
proteinuriahypoproteinemiaedemahyperlipidemia
Nephritic syndromehematuriaproteinuriaimpaired kidney functionhypertension
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Nephrotic syndrome
• “Primary” disease• Secondary to systemic diseases
– Diabetic nephropathy– Amyloidosis
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Minimal changes nephropathy (MCD)
• Most usual cause of nephrotic syndrome• Mostly chidren• ”Normal” histology• Loss of foot processes
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Focal segmental glomerulosclerosis (FSGS)
• Primary• Secondary
– (often milder nephrotic syndrome)
– extreme obesity– reflux nefropathy/ single kidney– renal artery stenosis– HIV- nefropathy
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Diabetic nephropathy
• NIDDM - type II diabetes40-50% affected after 20 years
disease• hypertension, metabolic risk factors
”nephrosclerosis”• as in type I diabetes
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Amyloidosis
• Systemic - AL - 'primary’monoclonal Ig light chain
plasma cell diseases - myeloma
• (Reactive) systemic -AA - 'secondary'prealbuminchronic infl dis - RA, Crohn, tbc, chronic
bronchitis
• FAP - ATTR - 'Skelleftesjukan'transthyretin- gen variant
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Nephrotic syndromework up
• Biopsy - compulsory• Systemic disease?
SLE? Diabetes? Myeloma?
• Malignancy?Lymphoma? Cancer?
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Nephritic syndrome
• Proteinuria - ’nephrotic’• Hematuria - micro∼/macro∼• Hypertension• Decreased GFR
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Glomerulonephritis• Acute GN
• Post streptococcal nephritis• Rapidly progressive GN (crescentic
nephritis)• Anti-basalmembrane-nephritis (anti-GBM-
nephritis)• Postinfectious crescentic nephritis• Idiopathic ~(ANCA-GN)
• Chronic GN• Membranous nephropathy - "membranous
GN"• (FSGS)
Membranoproliferative GN (dense deposit
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Poststreptococcalglomerulonephritis
PSGN• unusual in Sweden• "nephritogenic" Gr A streptococcal inf• tonsillitis, scarlatina, impetigo• mostly subclinical, but…• late effects in heart, joints, brain
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RPGN
• Anti-GBM-nephr linear anti-GBM2-20% against Goodpasture
ag
• Postinfect granularimmune complexes
15-50%
• Idiopathic neg p-ANCA/c-ANCA pauci-immune
IFL Antibodies
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RPGN
Renal ltd disease Systemic
• Anti-GBM-nephritis Goodpasture's syndrome
• Immune complex nephritis SLE
• Idiopathic RPGN Wegener / small vesselvasc.
disease
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IgA -nephropathy
• Berger, 1968 - "Berger-nephritis"• most common nephritis in the world• 20-30 years age• 3-6 x more men
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IgA -nephropathy• PAD
mesangial proliferationmesangioproliferative GN
• IFLmesangial IgA deposits
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Systemic diseases withIgA -nephropathy
• Henoch-Schönlein purpura• ulcerative colitis, Mb Crohn, celiac
disease• dermatitis herpetiformis• liver diseases
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SLE-nephritis• WHO classification based on biopsy
findings:•• class I ⇒ normal glomeruli• class II ⇒ mesangial GN• class III ⇒ focal segmental proliferative GN• class IV ⇒ diffuse proliferative GN ± ev crescents)• class V ⇒ membranous nephropathy• (klass VI ⇒ glomerulosclerosis - ”endstage")
• IFL ⇒ "full-house"
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Tubulointerstitial diseases• Infectious Pyelonephritis (TIN)
Endemic nephropathy
• Non-infectious Allergic TINAnalgesic/toxic nephropathyGout nephropathyNephrocalcinosisGranulomatous disease
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ACUTE PYELONEPHRITIS
• ETIOLOGY Bacterial infection (E. coli 80%)
• PATHOGENESIS Ascending infection
• PATIENTS at RISK a) KADb) Obstructive uropathyc) Refluxd) Old or pregnant womene) Diabetes f) Malformation
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CHRONIC PYELONEPHRITIS
• ETIOLOGY Chronic infection?
• PATHOGENESIS Obstruction or reflux
• PRESENTATION Hypertension, progressive
renal failure
• MICRO Interstitial fibrosis,
tubular atrophy, FSGS
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Hemorrhagic fever with renal syndromeNEPHROPATHIA EPIDEMICA
• EPIDEMIOLOGY Most cases in Northern Scandinavia
• ETIOLOGY Puumala virus(Gen Hantavirus;Fam. Bunyaviridae) in rodents (bank vole[skogssork])
• PRESENTATION Fever, malady, acuterenal failure, hematuria
• MICRO Hemorrhagic TIN
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TOXIC ATN• ETIOLOGY Heavy metals
Fungal toxinsDrugs
• PATHOGENESIS Necrosis of epithelium in proximal tubuli
• MICRO Normal BM
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Ischemic Nephropathy (ATN)• ETIOLOGY Major injury/trauma
hypovolemia/ hypoperfusion
• PATHOGENESIS Necrosis of tubularepithelium (distal and/or proximal)
• MICRO Damaged BM.BM. Inflam, cylinders
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VASCULAR DISEASES
Renal damage causes hypertension, hypertension causes renal damage
• Nephrosclerosis benign/malignant• Renal artery stenosis• Infarction• Vasculitis
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BENIGN NEPHROSCLEROSIS• ETIOLOGY / PATHOGENESIS
– Primary hypertension (90-95%)
– Secondary hypertension
• MACRO Small kidneys, thin cortex
• MICRO Hyaline arteriolosclerosis
• PREVALENS In all older people but more pronouced if
hypertension
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MALIGNANT NEPHROSCLEROSIS
• ETIOLOGY Malignant hypertension• PRESENTATION Sympt fr other organs,
hematuria, rapidly progressing renal failure
• MICRO Fibrinoid necrosis in arterioli, thromboticmicroangiopathy
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RENAL ARTERY STENOSISRENAL ARTERY STENOSIS• ETIOLOGY Atherosclerosis
Fibromuscular dysplasia
• PATHOGENESIS Low perfusioncauses high level of renin...
• TREATMENT PTRA
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RENAL INFARCTION
• ETIOLOGY Arterial embolization
(”cholesterol emboli”)
• MACRO Wedge shaped pallor
• SYMPTOMS Sharp flank pain
Hematuria
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HYDRONEPHROSISHYDRONEPHROSIS• (Obstructive uropathy)
• Dilatation of pelvis and calyces, atrophy of
parenchyma
• ETIOLOGY High pressure
– Congenital
– Acquired
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RENAL STONESRENAL STONES
Urolithiasis NefrolithiasisUrethrolithiasisCystolithiasis
• ETIOLOGY– Increased urinary excretion of salt – Lack of inhibitory substances– Residual urin– Infection– pH
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Ca-OXALATE STONES
70 % of all stones in SwedenHypercalcemia / -uria (10%).Idiopathic hypercalcuria (50%)Normal Ca in urin (20%).Hyperoxaluria (eg bowelshuntop)Hyperuricemia (20%)
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MAGNESIUM AMMONIUM PHOSPHATE STONES (5 - 10 %)
- ”Staghorn calculus”- Infection with urea splicing bacteria
URIC ACID STONES- Only 25% has gout
CYSTINE STONES- Hereditary cystinuria
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TUMOURS of the TUMOURS of the KIDNEYKIDNEY
• BENIGN: Adenoma (papillary)OncocytomaAngiomyolipoma
• MALIGNANT: Renal cell carcinoma
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RENAL CELL CANCERRENAL CELL CANCER
2% of all cancer, 1000 cases/year in Sweden
men : women = 11 : 7
elderly people ( >70 yo )
50 % incidental finding!
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• ETIOLOGY Smoking, obesity, high bloodpressure, diabetes mellitus
• HEREDITY < 5 % (von Hippel-Lindau)
• CLIN FEAT Hematuriapainful tumour mass
– anemia– high SR
endocrine activity
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MICRO Clear cell (= ”conventional”)PapillaryChromofobeCollecting duct
TREATMENT SurgeryPROGNOSIS 50% 5 years
-No met. 70 %-Tumour in vein 15%
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PROGNOSTIC FACTORS
GRADING
four grades according to Fuhrman
STAGING
pTNM by UICC 2002