Benign Nephrosclerosis

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Benign Nephrosclerosis

• Found in patients with benign


hypertension
• Hyaline thickening of arterial walls
• Leads to mild functional impairment
• Rarely fatal
• Affects only afferent arterioles
• When seen in diabetes affects both
afferent and efferent arterioles
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Benign nephrosclerosis
Malignant Nephrosclerosis

• Arises in malignant hypertension


• Hyperplastic vessels
• Ischemia of kidney
• “Flea beaten”kidney on gross
appearance
• Medical emergency

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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

• Cysts can be formed anywhere in the kidney


i.e. tubules, collecting ducts, bowman’s
space, etc.

• Expanding cysts cause pressure on the


surrounding tissues and lead to ischemic
changes

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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

• Most patients develop end stage renal failure

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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

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