Clinical Flashcards
(547 cards)
what is kidney agenesis?
a congenital absence of one or both kidneys
what is kidney hypoplasia?
a congenital condition causing small kidneys with normal development and function
(reduced capacity)
what is a horseshoe kidney?
kidneys congenitally fused at either pole- usually lower
do simple cysts usually cause a functional disturbance?
no
what are the 2 main types of polycystic kidney disease?
- autosomal recessive PKD (prev known as infantile)
- autosomal dominant PKD (prev known as adult)
what is the most common subtype of ARPKD?
perinatal group
what does the perinatal group of ARPKD cause?
terminal renal failure
what happens to the medullary collecting ducts in ARPKD?
cystic dilation
what liver condition is ARPKD associated with?
congenital hepatic fibrosis
what is the most common inherited kidney disease?
autosomal dominant polycystic kidney disease
compare ADPKD 1 and ADPKD2 in terms of chromosomes affected? (give percentages)
ADPKD 1: defect on chromosome 16 (90%)
ADPKD 2: defect on chromosome 4 (10%)
what is the aetiology of ARPKD?
genetic
what is the aetiology of ADPKD?
genetic
when does ADPKD present?
usually in middle adult life
what does ADPKD present with?
abdominal mass
haematuria
chronic renal failure
hypertension
where do cysts arise in ARPKD?
medullary collecting tubules
where do cysts arise in ADPKD?
any part of the nephron
which other organs can be affected with cysts in ADPKD?
liver
pancreas
lung
what functionally happens to the liver, pancreas, lung when affected in ADPKD?
no funcional effect
what aneurysm is ADPKD associated and what can this lead to?
berry aneurysm in circle of Willis
can lead to subarachnoid haemorrhage
compare ARPKD and ADPKD in terms of the gross shape of the kidney?
ARPKD- enlargment but shape is still there
ADPKD- massive enlargement, shape is distorted
why can haematuria occur in ADPKD?
cysts can be filled with blood
compare the causes of intracerebral haemorrhage and subarachnoid haemorrhage in ADPKD?
intracerebral- due to hypertension caused by chronic renal faiulre
subarachnoid- due to berry aneurysm in circle of Willis
what is the most common benign renal tumour?
fibroma