Pathology 2 Flashcards
Define agenesis of the kidneys
This is absence of one or both kidneys
Define hypoplasia of the kidneys
This means that the kidney/s are small but have normal development
What is meant when a kidney is described as having a horseshoe appearance ?
There has been fusion of both kidneys at either pole
What is the cystic kidney disease which affects infants ?
Infantile polycystic kidney disease
What is the mode of inheritance for infantile polycystic kidney disease?
Autosomal Recessive - hence known as ARPKD (autosomal recessive polycystic kidney disease)
What is the result of autosomal recessive polycystic kidney disease ?
Terminal renal failure
What are the pathological features of ARPKD?
Uniform bilateral renal enlargement. Elongated cysts – dilatation of medullary collecting ducts.
What condition is ARPKD associated with ?
Congenital hepatic fibrosis
What is the main cystic kidney disease which will most commonly affect adults ?
Adult polycystic kidney disease
What is the mode of inheritance and genetic mutation for adult polycystic kidney disease?
- Autosomal dominant - main mutation is in the PKD1 or PKD2 gene
What do patients with ADPKD usually present with in terms of pathology ?
- 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
What are the main benign renal tumours ?
- Fibroma
- Adenoma
- Angiomyolipoma
- Juxtaglomerular cell tumour
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
- Common. Medullary origin, white nodules. = Fibroma
- Small yellowish nodules <2cm. Cortical. = Adenoma
- Mixture of fat, muscle and blood vessels. Can be multiple and bilateral. Associated with Tuberous Sclerosis. = Angiomyolipoma
- Causes production of renin resulting in secondary hypertension. = Juxtaglomerular cell tumour
What is the commonest malignant renal cell tumour?
Renal cell carcinoma
Descibe the presentation of a renal cell carcinoma
- Presents with abdominal mass
- Haematuria
- Flank pain
- General features of malignant disease