Polycystic Kidney Disease Flashcards
How can you tell that an enlarged abdominal mass is a kidney
It enlarges from the costal margin towards the ipsilateral iliac fossa
Resonant percussion note - due to gas in the overlying colon
Descends on respiration
Ballotable
Bimanually palpable
In most cases only the lower pole of the kidney is palpable - smooth and hemi-ovoid
What other sign might you expect in an advanced renal tumour
Supraclavicular lymphadenopathy
What is the differential diagnosis for an enlarged kidney
Congenital:-
Cystic disease (including PKD)
Horseshoe kidney
Hypertrophic single kidney
Acquired:- Solitary cysts Tumours Hydronephrosis Pyonephrosis Perinephric abscess Renal vein thrombosis Diabetes Amyloidosis SLE
What is hydronephrosis
Swelling of a kidney due to a build up of urine
Due to the inability to drain urine from the kidney to the bladder secondary to an obstruction
*If severe can lead to kidney failure
What is pyonephrosis
Infected hydronephrosis
What are the two types of polycsytic kidney disease
Autosomal dominant (much more common) Autosomal recessive
What chromosomes are affected in AD PCKD
4 and 16
What age do patients usually present with AD PCKD
30s-50s
How does AD PCKD present
Hypertension
Haematuria
Loin pain
Asymmetrical kidney enlargement
What are the extrarenal manifestations of AD PCKD
Liver cysts
Intracranial aneurysms
Colonic diverticulae
Mitral regurgitation
What chromosome is affected in AR PCKD
6
What is the age of presentation in AR PCKD
Perinatal
How does AR PCKD present
Oligohydramnios
Large liver and kidneys (bilateral)
Chronic renal failure
What are the extrarenal manifestations of AR PCKD
Congenital hepatic fibrosis
Sometimes biliary ectasia
What is the prognosis of AR PCKD
All die by age 20
Often die in neonatal period
