Urogenital 1 Flashcards
State and explain pathogenesis of TWO examples of developmental defects that arise from abnormalities in size or number of kidneys.
- Renal Agenesis
- lack of metanephric primordium
- failure of ureteral bud development
- failure of contact of ureteral bud and metanephros - Renal Hypoplasia
- reduced number of lobules and calyces (<5; normal >10)
- small kidney
Can one have bilateral renal agenesis?
No. Incompatible with life, infants with this are stillborn
What is the ratio of males and females with bilateral renal agenesis?
25.1 : 1
What is the ratio of males and females with unilateral renal agenesis?
2 : 1
What is an adaptation to unilateral renal agenesis?
Opposite kidney enlarged due to compensatory hypertrophy
How does renal hypoplasia appear microscopically?
Primitive but hypertrophied (oligomeganephronia) glomeruli and tubules in dense fibrous or fatty interstitium
State TWO examples of abnormalities in kidney position or shape.
- Displacement of Kidney
- Horseshoe Kidney
What is the complications of displacement of kidneys?
- Kinking or tortuosity of ureters, resulting in obstruction with urinary infection
What is the pathogenesis of horseshoe kidney?
Fusion of lower (90%) or upper (10%) poles of kidneys
What is the incidence of horseshoe kidney?
1:500 to 1:1000
What is a complication of horseshoe kidney?
Renal calculi
Can cystic disease of kidney be congenital and acquired?
Yes
State 5 types of cystic kidney disease.
- Cystic Renal Dysplasia
- Polycystic Kidney Disease
- Autosomal Dominant (Adult)
- Autosomal Recessive (Childhood) - Medullary Cystic Disease
- Acquired Cystic Disease (Dialysis associated)
- Localised (simple) renal cysts
What is the pathogenesis of cystic renal dysplasia?
Abnormal metanephric differentiation, with persistence of primitive/abnormal structures
What TWO conditions might cystic renal dysplasia be associated with?
Ureteric Atresia
Renal Agenesis
What is the gross appearance of cystic renal dysplasia?
Enlarged, irregular shape, multicystic
What are microscopic findings of cystic renal dysplasia?
- Lobar Disorganisation
- Multiple Cysts lined by flattened epithelium
- Presence of primitive/immature structures
- Islands of undifferentiated mesenchymal cells
- islands of cartilage tissue
- primitive ductal structures
What is the pathogenesis of polycystic kidney disease (autosomal dominant)?
- Mutations in PKD1 and PKD2 genes, which code for proteins polycystin 1 and polycystin 2
- Develop multiple cysts in both kidneys, starting as small cysts that gradually enlarge
- Eventually kidneys are composed almost entirely of cysts with very little parenchyma left
- Leads to renal failure in mid to late adulthood
How common is polycystic kidney disease?
1/400 to 1/1000 live births
What is urolithiasis?
Formation of calculus or calculi (stones) within the urinary tract
Where does urolithiasis usually begin?
Calyces and Pelvis, some in lower urinary tract
What is the primary cause of urolothiasis?
Supersaturation of urine with crystalline material (commonly calcium salts)
What are 4 secondary causes of urolithiasis?
- UTI - crystalline material encrust on a necrotic focus on mucosa calyx, pelvis or bladder
- Indwelling catheter or foreign body in the bladder
- Vitamin A deficiency producing squamous metaplasia of upper urinary tract mucosa
- Low urinary pH predisposes to formation of uric acid stones
What are the 4 types of renal calculi
- Calcium stones (calcium oxalate mixed with calcium phosphate) (65-70%)
- ‘Triple stones’ magnesium ammonium phosphate (15%): usually after infection
- Urate stones (from uric acid) (5-10%): Gout, Leukaemia
- Cystine Stones (1-2%): Genetic Defect in Renal Transport or Amino Acids