Pathology Flashcards
(199 cards)
what is meant by agenesis?
complete absence of an organ
what do duplex system congenital abnormalities of the renal system give an increased risk of?
infection
do simple cysts of the kidney usually cause any problems?
no they are very common, usually no functional disturbance
what is the more rare form of polycystic disease and how is it inherited?
infantile type, autosomal recessive polycystic kidney disease
what does the infantile polycystic kidney disease cause? what is the prognosis?
uniform bilateral renal enlargement- elongated cysts- dilation of medullary collecting ducts. causes terminal renal failure, baby usually dies in neonatal period but less severe cases can survive a few months
what does infantile polycystic disease have an association with?
congenital hepatic fibrosis
what is the more common form of congenital cystic disease and how is it inherited?
adult polycystic disease- autosomal dominant polycystic kidney diease
what is the gene defect in ADPKD 1?
defect on chromosome 16
what is the gene defect in ADPKD 2?
defect on chromosome 4
what percentage of ADPKD is type 1?
90%
when does ADPKD usually present?
usually middle adult life
how does ADPKD usually present?
abdo mass, haematuria, hypertension, chronic renal failure
how many times is the enlargement of a kidney in ADPKD compared to normal?
10 times larger
what can be seen on the kidney in ADPKD?
multiple cysts of varying sizes, distortion of reniform shape of kidney.
where can cysts arise in the nephron ADPKD?
at any part of the nephron
what do 1/3rd of patients with ADPKD also have?
cysts in liver, pancreas and lung - usually no functional effect
what is ADPKD associated with?
berry anuerysms in circle of willis - subarachnoid haemorrhage
what are the cysts in ADPKD filled with?
most filled with clear fluid but some can be filled with blood
what are the 2 types of brain haemorrage someone with ADPKD is at risk of getting and why?
ADPKD associated with subarachnoid haemorrhage. Also at more risk if intracellular haemorrhage due to hypertension caused by ADPKD
what are the 4 most common benign renal tumours?
- fibroma
- adenoma
- angiomyolipoma
- JGCT
what is the origin and appearance of a renal fibroma?
medullary origin, white nodules
what is the appearance a renal adenoma and what size would you expect it to be?
yellowish nodules, often less than 2cm
where in the kidney would you find an adenoma?
cortex
what makes up the contents of an angiomyolipoma on the kidney?
mixture of fat, muscle and blood vessels