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12 Cards in this Set

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Renal System- Developmental Renal Disease by Leonard
Renal System- Developmental Renal Disease by Leonard
Horseshoe kidney, form and position.
-Fusion of upper or lower poles of kidney
-Typically located at midline, may be lower than normal (may be pelvic)
-Lie anterior to great vessels if above bifurcation for common iliac arteries
-Relatively common (~ 1:750 autopsies)
-Ureters may become kinked – prone to obstruction with resultant hydronephrosis
-May have normal kidney function (asymptomatic)
-May be associated with extrarenal malformations
Renal agenesis...2 types.
Agenesis: absence of one or both kidneys and corresponding ureter(s)

1. Unilateral
-Asymptomatic; compensatory hyperplasia of contralateral kidney (may undergo glomerulosclerosis)
-May be other urogenital malformations

2.Bilateral
-Associated with *oligohydramnios; *Potter sequence (flattened nose, low-set ears, recessed chin; limb contractures, pulmonary hypoplasia)
-Incompatible with life (b/c of the pulm hypoplasia)
-all features of Potter Sequence are related to deficiency of amniotic fluid
Renal hypoplasia... you have less of what? It's different from what?
Hypoplasia: failure to develop to normal size
-Reduced number and size of renal pyramids, calyces
-Different from acquired scarring from vascular or other causes
-Very rare
Ectopic kidney...where is it normally?associated with what other conditions?
-Often located in pelvis
-Typically also are abnormally rotated
-May be associated with urine outflow obstruction, hydronephrosis, pyelonephritis
Cystic Renal Diseases, in general, can be..
-Hereditary, developmental, acquired
-Relatively common
-May result in CRF
Autosomal Dominant Polycystic Kidney Disease (ADPKD)... onset...how common....what is the genetic defects?
Adult onset (typically presents in 30s-40s)
Most common symptomatic cystic renal disease
-Most common renal genetic disease (1:500 – 1:1000)
-A leading cause of ESRD (~ 5-10% of dialysis patients)
~ 25% appear to be sporadic (lack family history)
Genetic defects
-PKD1 gene (16p13.3) – codes for *polycystin-1
-Renal tubular epithelial cells; cell-cell and cell-matrix interactions
-Accounts for ~ 80% of cases; worse prognosis than PKD2
PKD2 gene (4q21) – codes for *polycystin-2
-Renal tubular epithelial cells and other tissue/organs; Ca2+ channel
ADPKD clinical features...first presents with? extrarenal congenital malformations? Speed of progression?
Presents initially with renal insufficiency
-May have hematuria, pain, sensation of abdominal/pelvic mass
Extrarenal congenital malformations
-Liver (intrahepatic biliary) cysts (~ 40%); usually asymptomatic
-Cysts in pancreas, spleen, lungs; berry aneurysm; mitral valve prolapse
Renal failure is slowly progressive
Autosomal Recessive Polycystic Kidney Disease (ARPKD)... onset...which gene...appearance
-Childhood onset (perinatal, neonatal, infantile, juvenile)
-PKHD1 gene (6p21-23) codes for fibrocystin*
-Highly expressed in kidney, liver, pancreas
-Congenital hepatic fibrosis: may be predominant feature in older children
-Portal hypertension, splenomegaly
-Compound heterozygote (inherit different mutant allele from each parent)

Kidneys are enlarged by generally thin, radially oriented (perpendicular to renal capsule) cysts within the cortex and medulla (sponge-like appearance)
-Exterior surface of the kidney is smooth, not disrupted by bulging surface cysts
Multicystic Renal Dysplasia...hereditary or no? do you see just kidney tissue?
-Sporadic disorder (not hereditary)
-Developmental abnormality with persistence of abnormal lobules of immature metanephric structures and abnormal, disorganized mesenchymal tissues (e.g., immature cartilage)
-Unilateral or bilateral
-Unilateral: remove affected kidney, excellent prognosis
-Enlarged kidney with large, irregular cysts and compression of residual parenchyma
Acquired Cystic Disease:
Dialysis-associated (ESRD)
-associated with what..
-aka end stage kidneys
-Multiple, variably sized thin-walled uniloculated cysts in cortex (extending into medulla if large)
-Associated with papillary neoplasias
-Papillary adenoma
-Papillary RCC
-Etiology unknown
Most common kind of kidney cyst is...

-appearance
-benign/malignant?
Simple cysts
-Typically only one or a few
-Cortical,; smooth, thin-walled, uniloculated
-Benign; may be hemorrhagic and confused with cystic RCC on certain imaging studies (vascular proliferation, rupture, hemmorhage)