5: Pathology 2 Flashcards
What is agenesis of the kidneys?
Complete failure of one or both kidneys to develop
Hypoplasia describes ___ organs with (normal / abnormal) development.
smaller
normal development
Which poles of the kidneys usually fuse to cause horseshoe kidney?
Inferior poles
Does horseshoe kidney affect renal function?
Usually not
but can obstruct ureters
Congenital abnormalities of the kidney predispose you to ___.
UTIs
What neurological presentation is associated with renal agenesis?
Oligohydramnios
Low volume of amniotic fluid in the placenta
What abnormal facial appearance is associated with oligohydramnios?
Which congenital renal disorder causes it?
Potter’s facies - low set ears, small jaw, flattened tip of the nose
Renal agenesis
What type of kidney disease, with a characteristic appearance, is very common?
Cystic kidney disease
What can cysts contain?
Serous fluid
Why may cystic kidney disease cause abdominal pain?
Ruptured cysts
What are the two types of polycystic kidney disease and how are they inherited?
a) Autosomal dominant PKD - adults, PKD1 on chromosome 16
b) Autosomal recessive PKD - kids, PKDH1, chromosome 6
Is polycystic kidney disease unilateral or bilateral?
Bilateral
What happens to the kidneys in
a) ADPKD
b) ARPKD?
a) Hyperplasia, damage to tubules
b) Hypoplasia, damage to collecting ducts, fibrosis
What is a characteristic feature of ARPKD?
Hypoplasia due to fibrosis
What are the symptoms of polycystic kidney disease?
Haematuria
Polyuria
Chronic loin pain
Hypertension
Because ADPKD causes hyperplasia, what may be seen on examination?
Abdominal mass
What neuro presentation is associated with ADPKD?
Subarachnoid haemorrhage
What diseases are associated with
a) ADPKD
b) ARPKD?
a) Loads - subarachnoid haemorrhage, valve disease, diverticulae and hernia
b) Fibrosis of kidneys