Polycystic Kidney Disease (9)

Question 1

What is the gross pathology of Polycystic kidney disease?

Answer 1

Kidney enlargement with multiple cyst formations

Question 2

What is the pathogenesis of cyst formation in Polycystic kidney disease?

Answer 2

• Renal tubular cells divide repeatedly leading to saccular cyst formation
• Cysts fill with fluid from glomerular filtrate
• Progressive expansion leads to cysts separating from parent tubule
• Isolated cysts fill with fluid by transepithelial secretion
• Mural epithelium proliferation and transepithelial secretion of sodium chloride and water cause relentless cyst expansion

Question 2

What is the mode of inheritance of Polycystic kidney disease?

Answer 2

Autosomal dominant condition due to mutations in 2 genes: PKD1, PKD2

Question 3

Which other organs in the abdomen can have cyst formation similar to Polycystic kidney disease?

Answer 3

Liver, ovaries, pancreas, and spleen

Question 4

What are the possible complications of Polycystic kidney disease?

Answer 4

1. Renal failure
2. Infection
3. Hypertension

Question 5

What other pathologies are associated with Polycystic kidney disease?

Answer 5

1. Liver cysts
2. Pancreatic cysts
3. Brain aneurysm
4. Mitral valve prolapse

Question 6

What is the associated lesion in the brain with Polycystic kidney disease?

Answer 6

Cerebral aneurysm

Question 7

Why is there pain in Polycystic kidney disease?

Answer 7

Due to the weight of the organ dragging upon its pedicle or stretching of renal capsule by cysts

Question 8

What are the other possible symptoms of Polycystic kidney disease?

Answer 8

Irregular abdominal mass, hematuria, hypertension, and uremia

Question 9

What are the differentials for Polycystic kidney disease?

Answer 9

1. Simple cyst
2. Acquired cystic kidney disease
3. VHL Von Hippel-Lindau disease
4. Medullary sponge kidney
5. Tuberous sclerosis

Question 10

What is the type of matching required before transplant for Polycystic kidney disease patients?

Answer 10

• ABO blood matching and HLA matching
  
  • HLA-A, HLA-B, and HLA-DR are the most important. They are proteins located on the surface of WBC.

Question 11

What types of malignancy occur with immunosuppression?

Answer 11

1. Squamous cell carcinoma of skin, cervix,
2. Basal cell carcinoma
3. Lymphoma
4. Kaposi’s sarcoma

Malignancy is 5 times greater than the normal population

Question 12

Types of graft rejection?

Answer 12

• Hyperacute rejection occurs within minutes of clamp release due to pre-formed antibodies, and immediate loss of graft occurs
• Accelerated acute rejection occurs in the first few days following surgery, involves both cellular and antibody-mediated injury, and pre-sensitization of the donor is a common cause
• Acute rejection is traditionally the most common type, is seen days to weeks after surgery, and is predominantly a cell-mediated process mediated by lymphocytes; organ biopsy demonstrates cellular infiltrates and graft cell apoptosis
• Chronic rejection is an increasingly common problem, typically shows graft atrophy and atherosclerosis, and fibrosis often occurs as a late event