Renal pathology 1 Flashcards
List the common congenital abnormalities of the renal abnormalities?
Agenesis
Hypoplasia
Horseshoe kidney
Duplex (ureter, pelvis)
What is kidney agenesis?
Absent kidney (or both kidneys)
What is kidney hypoplasia?
Normal kidney develops but at a smaller size
What is horseshoe kidney?
Kidneys fuse at upper or lower pole (more commonly lower pole)
What is the prognosis of kidney agenesis of both kidneys?
Death
Kidney agenesis can result in a characteristic facial appearance. Why?
diminished urine production –>
diminished amniotic fluid –>
squished nose, recessed chin & low ears
What pathology can horseshoe kidney cause?
Obstruction (rarely)
How common are simple cysts?
Common
How do simple cysts affect the kidney?
They don’t usually have a functional effect
What size are simple cysts?
Can be large or small
When might simple cysts be secondary?
Long term kidney dialysis
Are simple cysts uni or bilobular?
Unilobular (but can have multiple cysts)
What are simple cysts filled with?
Fluid (NO blood)
How common is infantile type polycystic kidney disease?
Rare (there are varying subtypes but all are rare)
How does infantile type polycystic kidney disease cause?
Terminal renal failure (may die immediately or survive for several months)
Is infantile type polycystic kidney disease autosomal dominant or recessive?
Recessive (autosomal recessive polycystic kidney disease)
How are the kidneys affected in infantile polycystic kidney disease?
Bilateral enlargement. Maintenance of reniform shape. Dilation of medullary collecting ducts
What is infantile polycystic kidney disease always associated with?
Congenital hepatic fibrosis
How common is adult polycystic kidney disease?
Rare (BUT least rare of all the congenital cystic diseases)
Is adult polycystic kidney disease autosomal dominant or recessive?
Dominant
Where are the gene mutations of ADPCKD type 1 and type 2 respectively? Which is the most common?
Type 1 - chromosome 16 (most common)
Type 2 - chromosome 4
How does adult polycystic kidney disease present?
Middle aged
Abdominal mass
Haematuria
Chronic renal failure –> +/- hypertension
How are the kidneys affected in adult polycystic kidney disease?
Huge bilateral enlargement. Cysts of varying size cause distortion of the reniform shape.
Where are the cysts in ADPCKD found?
Nephron (any part)