Pathology 2 Flashcards

1
Q

Define agenesis of the kidneys

A

This is absence of one or both kidneys

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2
Q

Define hypoplasia of the kidneys

A

This means that the kidney/s are small but have normal development

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3
Q

What is meant when a kidney is described as having a horseshoe appearance ?

A

There has been fusion of both kidneys at either pole

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4
Q

What is the cystic kidney disease which affects infants ?

A

Infantile polycystic kidney disease

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5
Q

What is the mode of inheritance for infantile polycystic kidney disease?

A

Autosomal Recessive - hence known as ARPKD (autosomal recessive polycystic kidney disease)

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6
Q

What is the result of autosomal recessive polycystic kidney disease ?

A

Terminal renal failure

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7
Q

What are the pathological features of ARPKD?

A

Uniform bilateral renal enlargement. Elongated cysts – dilatation of medullary collecting ducts.

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8
Q

What condition is ARPKD associated with ?

A

Congenital hepatic fibrosis

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9
Q

What is the main cystic kidney disease which will most commonly affect adults ?

A

Adult polycystic kidney disease

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10
Q

What is the mode of inheritance and genetic mutation for adult polycystic kidney disease?

A
  • Autosomal dominant - main mutation is in the PKD1 or PKD2 gene
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11
Q

What do patients with ADPKD usually present with in terms of pathology ?

A
  • Massive bilateral renal enlargement
  • Multiple cysts of varying size, the cysts completely distort the shape of the kidney
  • Often associated cysts in the liver, pancreas, lung
  • Association with Berry Aneurysms in Circle of Willis > subarachnoid haemorrhage. These aneurysms liable to rupture resulting in haemorrhage
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12
Q

What are the main benign renal tumours ?

A
  1. Fibroma
  2. Adenoma
  3. Angiomyolipoma
  4. Juxtaglomerular cell tumour
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13
Q

Match the following benign renal cell tumour to its description:

  • Small yellowish nodules <2cm. Cortical.
  • Causes production of renin resulting in secondary hypertension.
  • Mixture of fat, muscle and blood vessels. Can be multiple and bilateral. Associated with Tuberous Sclerosis.
  • Common. Medullary origin, white nodules.

Fibroma, adenoma, angiomyolipoma, juxtaglomerular cell tumour

A
  1. Common. Medullary origin, white nodules. = Fibroma
  2. Small yellowish nodules <2cm. Cortical. = Adenoma
  3. Mixture of fat, muscle and blood vessels. Can be multiple and bilateral. Associated with Tuberous Sclerosis. = Angiomyolipoma
  4. Causes production of renin resulting in secondary hypertension. = Juxtaglomerular cell tumour
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14
Q

What is the commonest malignant renal cell tumour?

A

Renal cell carcinoma

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15
Q

Descibe the presentation of a renal cell carcinoma

A
  • Presents with abdominal mass
  • Haematuria
  • Flank pain
  • General features of malignant disease
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16
Q

What can renal cell carcinomas sometimes produce ?

A
  • Erythropoietic stimulating hormone which results in increased RBC production hence polycytheaemia
  • Also can produce calcium
17
Q

What cell do renal cell carcinomas most often arise from ?

A

Clear cell type

18
Q

What do renal cell carcinomas often spread to ?

A
  • Lung and bone
  • Commonly invade into the renal vein
19
Q

What is used to stage renal cell carcinomas ?

A

Furhmans staging

20
Q

What is the most common type of bladder cancers and why ?

A
  • Transitional cell carcinoma (urothelial)
  • As transitional epithelium lines from npelvicalyceal area to the external urethra
21
Q

What are the risk factors for developing transitional cell carcinomas ?

A
  • Aniline dyes (B-naphthylamine),
  • rubber industry,
  • schistosomiasis – this is a parasite,
  • smoking.
22
Q

What is the other malignant tumour in the urinary tract associated with Schistosomiasis?

A

Squamous Carcinoma

23
Q

What is the commonest malignant bladder tumour in children ?

A

Embryonal Rhabdomyosarcoma

24
Q

Where in the bladder do most transitional cell carcinomas arise ?

A

The trigone