Pathology Flashcards
what is agenesis
absence of one or both kidneys
what is the name given to small kidneys but normal development
hypoplasia
what is a ‘horseshoe’ kidney
fusion of the kidneys at either ple
what is duplex system
two kidneys (one on top of the other) on one or both sides of your body
what is a very common cystic disease of the kidney
simple cysts
can be multiple and large
usually clear fluid filled
often incidental finding
what conditions is simple cysts often secondary to
native kidneys in long term dialysis
what are the two subtypes of genetic polycystic disease
infantile type (ARPKD) adult polycystic disease (ADPKD)
features of infantile polycystic disease
rare
causes terminal renal failure
less severe cases can survive for some months
why is infantile polycystic disease terminal
perinatal - not suitable for renal transplant or dialysis
what are pathological features of infantile polycystic disease
Uniform bilateral renal enlargement.
Elongated cysts – dilatation of medullary collecting ducts.
what type of genetic inheritance is infantile polycystic disease
autosomal recessive
what is infantile polycystic disease associated with
congenital hepatic fibrosis
how is infantile polycystic disease diagnosed
prenatal ultrasound
what congenital cystic disease is more common
Adult Polycystic Disease
what type of inheritance is Adult Polycystic Disease
autosomal dominant
what are the two possible chromosomes that can be affect to cause Adult Polycystic Disease
Chromosome 16
Chromosome 4
what chromosome defect is 90% of the cases of Adult Polycystic Disease
Chromosome 16 defect
typical presentation of adult polycystic disease
middle adult life abdominal mass haematuria hypertension chronic renal failure renal stone
pathology of Adult Polycystic Disease
massive bilateral renal enlargement
multiple cysts of varying size arising in any part of nephron
what is seen in 1/3rd of patients with Adult Polycystic Disease
cysts in liver, pancreas, lung but with no functional effect
what is Adult Polycystic Disease associated with
Berry aneurysms in Circle of Willis
what does Berry aneurysms predisposed patients to
subarachnoid haemorrhage.
what other haemorrhage are patients with adult polycystic disease are at risk at and why
intra-cerebral haemorrhage
due to hypertension
most common benign renal tumour and its features
fibroma
Medullary origin, white nodules. No clinical consequence.