Polycystic Kidney Disease Flashcards

1
Q

How can you tell that an enlarged abdominal mass is a kidney

A

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

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2
Q

What other sign might you expect in an advanced renal tumour

A

Supraclavicular lymphadenopathy

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3
Q

What is the differential diagnosis for an enlarged kidney

A

Congenital:-
Cystic disease (including PKD)
Horseshoe kidney
Hypertrophic single kidney

Acquired:-
Solitary cysts
Tumours
Hydronephrosis
Pyonephrosis
Perinephric abscess
Renal vein thrombosis
Diabetes
Amyloidosis
SLE
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4
Q

What is hydronephrosis

A

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

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5
Q

What is pyonephrosis

A

Infected hydronephrosis

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6
Q

What are the two types of polycsytic kidney disease

A
Autosomal dominant (much more common)
Autosomal recessive
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7
Q

What chromosomes are affected in AD PCKD

A

4 and 16

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8
Q

What age do patients usually present with AD PCKD

A

30s-50s

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9
Q

How does AD PCKD present

A

Hypertension
Haematuria
Loin pain
Asymmetrical kidney enlargement

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10
Q

What are the extrarenal manifestations of AD PCKD

A

Liver cysts
Intracranial aneurysms
Colonic diverticulae
Mitral regurgitation

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11
Q

What chromosome is affected in AR PCKD

A

6

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12
Q

What is the age of presentation in AR PCKD

A

Perinatal

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13
Q

How does AR PCKD present

A

Oligohydramnios
Large liver and kidneys (bilateral)
Chronic renal failure

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14
Q

What are the extrarenal manifestations of AR PCKD

A

Congenital hepatic fibrosis

Sometimes biliary ectasia

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15
Q

What is the prognosis of AR PCKD

A

All die by age 20

Often die in neonatal period

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