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12 Cards in this Set
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Renal System- Developmental Renal Disease by Leonard
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Renal System- Developmental Renal Disease by Leonard
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Horseshoe kidney, form and position.
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-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 |
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Renal agenesis...2 types.
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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 |
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Renal hypoplasia... you have less of what? It's different from what?
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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 |
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Ectopic kidney...where is it normally?associated with what other conditions?
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-Often located in pelvis
-Typically also are abnormally rotated -May be associated with urine outflow obstruction, hydronephrosis, pyelonephritis |
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Cystic Renal Diseases, in general, can be..
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-Hereditary, developmental, acquired
-Relatively common -May result in CRF |
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Autosomal Dominant Polycystic Kidney Disease (ADPKD)... onset...how common....what is the genetic defects?
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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 |
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ADPKD clinical features...first presents with? extrarenal congenital malformations? Speed of progression?
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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 |
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Autosomal Recessive Polycystic Kidney Disease (ARPKD)... onset...which gene...appearance
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-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 |
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Multicystic Renal Dysplasia...hereditary or no? do you see just kidney tissue?
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-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 |
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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 |
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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) |