Benign Nephrosclerosis
Benign Nephrosclerosis
Benign Nephrosclerosis
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Malignant Hypertension
• 5% of cases of hypertension
• Super-high blood pressure, encephalopathy,
heart abnormalities
• First sign often headache, scotomas
• Decreased blood flow to kidney leads to
increased renin, which leads to increased BP!
• 5y survival: 50%
• Histology:Necrotising arteriolitis, onion
skinning,
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Malignant nephrosclerosis
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Malignant nephrosclerosis
Renal Pathology Outline
• Introductory stuff
• Glomerular diseases
• Tubular and interstitial diseases
• Diseases involving blood vessels
• Cystic diseases
– Adult polycystic kidney disease
– Childhood polycystic kidney disease
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Adult Polycystic Kidney Disease
• Autosomal dominant
• Huge kidneys full of cysts
• Usually no symptoms until 30s
• Associated with berry aneurysms and
Hypertension,hematuria
• Germline mutation affecting PKD1
gene on chromosome 16(85-90%). or
PKD2 gene on chromosome 4(15%)
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ADPKD
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Adult polycystic kidney disease
ADPKD
• Clinical findings:
- Most patients remain asymptomatic until middle age
- Abdominal mass – massive bilateral kidney enlargement
- Flank pain
- Hypertension
- Gross hematuria
- Berry aneurysms, liver cysts are extrarenal findings
• Diagnosis: ultrasound
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Childhood Polycystic Kidney Disease
• Autosomal recessive
• Numerous small cortical
cysts
• Associated with liver cysts
• Patients often die in
infancy
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Autosomal Recessive (Childhood) Polycystic Kidney disease
• This rare disease has a autosomal recessive pattern, which result from a
mutation in the PKHD1 gene-codes for the fibrocystin gene on
chromosome 6
• It presents bilaterally as small cysts, which are usually found in the
cortex and medulla and give a sponge-like appearance
• Cysts may be found in the liver
• There is a high mortality rate associated with neonates or young infants.
In the surviving patient, liver cirrhosis will ensue
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Childhood polycystic kidney disease