CYSTIC DISEASES OF KIDNEY(patho) Flashcards
Name the cystic diseases of kidney?
1.Cystic renal dysplasia
2.adult and childhood polycystic kidney diseases
3.Nephronophthisis-Medullary cystic disease
4.Medullary sponge kidney
5.Aquired cystic diseases
6.Simple cysts
Most common cystic disease in children
Cystic renal dysplasia
Pathogenesis of cystic renal dysplasia
It is a cystic disease due to an abnormality in metanephric differentiation
Characterised by enlarged,irregular,multicystic kidneys
Types of cystic renal disease
Unilateral and bilateral
In unilateral-> function of opposite kidney is normal
Prognosis is excellent
In bilateral -> may lead to renal failure
Accounts for 20% cases of chronic renal failure in children
What is nephronophthisis medullary cystic disease?
It is a group of cystic diseases characterised by cysts in the medulla, usually concentrated at the cortioco-medullary junction
How many variants are there of nephronophthisis Medullary cystic disease?
There are 4 variants
1.Sporadic nonfamilial nephronophthisis = 20%
2.Renal retinal dysplasia
3.Familial juvenile nephronophthisis(most common)
4.Adult onset medullary cystic disease
Characteristics of familial juvenile nephrononthisis and adult onset meduallary custic disease
Familial is autosomal recessive
Adult is autosomal dominant
Both are characterised by corticomedullary cysts and shrunken kidneys
Clinical features of familial are salt retention,polyuria,growth retardation,and anemia
And progressive renal failure beginning in childhood
And in adult progressive renal failure beginning in adulthood
What is medullary sponge kidney?
It is cystic disease that should be restricted to multiple cystic dilatations of the collecting ducts in the medulla
How is medullary sponge kidney discovered?
On intravenous pyelography
Clinical features of medullary sponge kidney?
Hematuria
UTI
Recurrent renal stones
Swiss cheese appearance
What is the most common cause of acquired cystic disease?
Patients with prolonged dialysis
