Term
| What type of epithelium lines the pelvis and calyces of the renal system? |
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Definition
| 1) transitional epithelium (urothelium) |
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Term
| Describe the blood supply to the kidney |
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Definition
From aorta to IVC:
- renal artery
- interlobar artery
- arcuate artery
- arterioles
- afferent arterioles
- glomerular tuft
- efferent arteriole
- venous vasa recta
- renal vein
- IVC |
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Term
| What type of infarct is seen with renal arteries? |
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Definition
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Term
| What is the approximate length (pole to pole) and weight of the average kidney? |
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Definition
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Term
| What are the four major congenital abnormalities with kidneys? |
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Definition
1) agenesis
2) hypoplasia
3) horseshoe kidney
4) ectopic kidney |
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Term
| Describe agenesis of the kidneys |
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Definition
1) bilateral is rare (incompatible with life
2) unilater is rare; compensatory hypertrophy of solitary kidney |
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Term
| Describe renal hypoplasia |
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Definition
1) unilateral or bilateral
2) small kidneys
3) reduced number of lobes and pyramids
4) renal failure in childhood |
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Term
| Describe horseshoe kidney |
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Definition
- kidneys are fused at lower (most common) or upper poles
2) incidental finding
3) more prone to medical disease |
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Term
|
Definition
- lower anatomic level kidney, may be found in the pelvis
2) prone to develop infections due to ureteral toruosity |
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Term
| Describe cystic renal dysplasia (multicystic kidney) |
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Definition
1) sporadic
2) unilateral; bilateral
3) underdeveloped; abnormally developed kidney; cartilage |
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Term
Describe autosomal dominant polycystic kidney disease (ADPKD):
genetics
clinical
gross features
microscopic features
associated abnormalities |
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Definition
1) AD w/ variable penetrance
2) Clinically: HTN, mass, hematuria, pain, UTI, renal failure
3) Gross: bilateral, large, distorted kidneys; fluid filled cysts in both cortex and medulla
4) microscopically: spherical, thin walled cysts (dilations of nephrone) w/ flattened functional epithelial cells
5) Associated abnormalities: hepatic cysts, cerebrovascular aneurysms, cysts of pancreas, spleen, lungs |
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Term
Describe autosomal recessive polycystic kidney disease (ARPKD)
genetics
clinical
gross features
microscopic features
associated abnormalities |
|
Definition
1) AR
2) abdominal masses/renal insufficiency, uremia
3) bilateral renal enlargement; innumerable small cysts in cortex and medulla
4) elongated cysts of collecting ducts at right angles to cortical surface
5) congenital hepatic fibrosis |
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Term
| Describe medullary cystic disease |
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Definition
- rare
- formations of small cysts in renal medulla |
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Term
| Describe solitary benign renal cysts |
|
Definition
1) renal cortical cysts are asymptomatic
- may mimic renal cell carcinoma
- nonregressed fetal cysts |
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Term
| Describe acquired (dialysis-associated) cystic disease |
|
Definition
- end-stage renal disease on dialysis
- cortical and medullary cysts w/ calcium oxalate
- RCC tumors present |
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Term
| What are the three morphologic compartments of the kidney? Why are these important? Which is most affected by infections/drug toxicity, immune mediated disease, systemic arteriosclerosis/vasculities, ESRD? |
|
Definition
1)
- glomerular
- tubulointerstitial (tubuli, interstitium)
- vascular
2) division of renal diseases into these categories
3) tubules; glomeruli; vessels; common pathway |
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Term
| What are the components of the JGA? What is the function of the JGA? |
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Definition
1) smooth muscle cells of afferent arteriole intima
2) macula densa
3) secrete renin |
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Term
| What are the three cell types of the glomerulus? What are the 2 extracellular structures? |
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Definition
1) mesangial cells; endothelial cells; epithelial cells
2) glomerular basement membrane; mesangial matrix |
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Term
| What ist he mesangium of the glomerulus composed of? |
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Definition
| 1) mesangial (pericytes) cells and mesangial matrix |
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Term
| What are the components of hte ultrafiltration membrane? |
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Definition
1) endothelial cells
2) glomerular basement membrane
3) epithelial cells |
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Term
| What are major characteristics of the endothelial cells of the ultrafiltration membrane? |
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Definition
1) very thin
2) fenestrated
3) negative charge |
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Term
| What is the fxn of the glomerular basement membrane? What are the three layers of the glomerular basement membrane? What is the major protein of the GBM? |
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Definition
1) negatively charged molecular sieve
2) lamina rara interna; lamina densa; lamina rara externa
3) type IV collagen |
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Term
| What are the major proteins involved in maintaining the filtration slit pore structure? What do disorders of these proteins cause clinically? |
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Definition
1) nephrin; podocin; actinin
2) protein loss in the ultrafiltrate |
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Term
| What are the two most common hereditary glomerular diseases? |
|
Definition
1) Alport syndrome
2) thin basement membrane disease |
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Term
Describe Alport syndrome
genetics
clinical characteristics
molecular defect
gross appearance |
|
Definition
1) X-linked AD/AR
2) renal failure, hearing loss, micro- macro-hematuria
3) collagen IV point mutation stop codon
4) thickening with lamellation and splitting |
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Term
Describe thing basement membrane disease
genetics
clinical
molecular defect
gross appearance |
|
Definition
1) AD
2) microhematuria
3) collagen IV mutation
4) normal structure, but thinned GBM |
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Term
| What are the most common causes of ESRD? |
|
Definition
1) diabetes
2) hypertension
3) glomerular disorders
4) cystic kidney disease
5) all others |
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Term
| What is the common cause of glomerular injury in both primary and secondary diseases of the glomerulus? |
|
Definition
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Term
| How are the glomeruli typically destryed in glomerular disorders? |
|
Definition
1) immune complexes
2) antibodies
3) cell mediated injury
4) activation of alternative complement pathwaya
5) drug toxicity
6) cytokines |
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|
Term
| How do immune complexes cause glomerular destruction? |
|
Definition
1) passively trapped and deposited
2) activation of complement; influx of inflammatory cells and glomerular destruction |
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Term
| How do circulating autoantibodies cause glomerular destruction? |
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Definition
1) directed against GBM
2) fix and activate complement
3) elicit glomerular inflammation |
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Term
| How can reduction in kidney mass cause glomerular destruction? |
|
Definition
1) increased GFR in remaining glomeruli
2) hypertrophy and damage to endothelial cell
3) thinning of epithelial foot processes w/ proteinuria
4) intravascular coagulation, fibrointimal proliferation, systemic hypertension |
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Term
| What are the major inflammatory cells of glomerular injury? What are the steps in glomerular inflammation and injury? |
|
Definition
1) neutrophils, macrophages, lymphocytes, mesangial, endothelial cells
2) cytokines (C5a*), O2 radicals, growth factors, NO released
3) influx of neutrophils/macrophages/lymphocytes
4) aggregation of platelets and formation of thrombi
5) release of additional *GF's from platelets (PDGF, cytokines, eicosanoids, 5HT - vasoconstriction, stasis, proliferation
6) complement mediated destruction |
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Term
| What is the effect of loss of 30-50% of nephrons in the kidney result in? |
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Definition
| 1) steady and progressive loss of kidney function and end stage renal disease (ESRD) |
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Term
| Define: glomerulosclerosis |
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Definition
| - glomeruli dmamaged beyond the point of recovery that show the following characteristics: increased mesangial cellularity and matrix; collapse of capillary tufts; closure of capillary lumina |
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Term
| What is the result of glomerulosclerosis on the distal vasculature? |
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Definition
1) no blood flow to the tubule because the tubules are supplied by the vasa recta
2) reult is atrophy of tubule w/ interstitial inflammation and fibrosis |
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Term
|
Definition
| - clinical condition characterized by accumulation of electrolytes and water, accumulation of uremic toxins, and decreased renal hormone synthesis |
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Term
| What is the clinical appearance of a patient with uremia? |
|
Definition
1) volume overload: pulm edema, HTN, edema, uremic pericarditis
2) nausea vomiting, anorexia
3) pruritis, anemia, bleeding diathesis, peripheral neuropathy |
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Term
|
Definition
| 1) decreased glomerular filtration w/ elevation of BUN and creatinine |
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Term
| What is the effect of damage glomeruli on the plasma creatinine? |
|
Definition
| 1) increased creatinine with glomeruli that cannot filter blood creatinine |
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Term
| Under what circumstances will BUN concentration be increased? Decreased? |
|
Definition
1) high protein diet
2) liver disease |
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