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23 Cards in this Set
- Front
- Back
What is the definition of Azotemia?
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Elevated Blood creatinine and BUN associated with decreased GFR
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what are the symptoms associated with uremia?
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neurological (seizures, altered mental state, peripheral neuropathy), GI disturbances (bleeding), and cardiovascular disturbances (pericarditis)
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define nephritic syndrome
- where is it classically seen? |
a glomerular problem - gross hematuria, mild-modest proteinuria, and hypertension
classically seen in post-streptococcal glomerulonephritis |
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define nephrotic syndrome
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gross proteinuria (> 3.5mg/day), hypoalbuminemia, edema, hypertension, hyperlipiduria, hyperlipidemia
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define acute renal failure
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oliguria or anuria, recent onset of azotemia. Can be from glomerular, vascular, or tubular (ATN)causes
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what symptoms are seen in tubular defects?
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polyuria, nocturia, electrolyte imbalances
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what shows up on labs when you've got a UTI?
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bacteruria and pyuria
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Chronic renal failure
- what defines diminished renal reserve? - what defines renal insufficiency? - what defines renal failure? - what defines end stage renal disease? |
diminished renal reserve - GFR < 50% of normal. Azotemia may develop
renal insufficiency - GFR is between 20-50% of GFR. Overt Azotemia with anemia and hypertension. Polyuria and Nocturia due to lack of ability to concentrate urine renal failure - GFR < 20% of normal. Edema, metabolic acidosis and hypocalcemia develop from loss of ability to reabsorb bicarb. end stage renal disease - GFR < 5% of normal. Outright uremia - this is fatal |
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What are patients with unilateral kidney agenesis at risk for?
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progressive glomerular sclerosis
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how can you tell a truely hypoplastic kidney from one that was due to vascular abnormalities, infectious, or other parenchymal disease?
what is Oligomeganephronia? |
truely hypoplastic kidneys have NO scars and reduced number of renal lobes and pyramids
Oligomeganephronia = fewer nephrons that are enlarged due to compensatory mechanisms |
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What characterizes renal cystic dysplasia?
- salient histological presentation? |
abnormal tissues like cartilage, undifferentiated mesenchyme (connective tissue) and immature collecting ducts
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What are the two genes involved in Adult Polycystic Kidney Disease?
- What chromosome are they found on? What kinds of proteins do they express? Mutation of which one has a worse prognosis? Mutation of which one is more common? |
PKD1 - polycystin 1 protein involved in matrix to cell and cell to cell adhesion. PKD1 mutation is more common and associated with earlier onset (53 years) of death
PKD2 = cation channel. Mutation is less ommon (15%) and associated with a less severe disease with death median of 69 years. PKD1 is found on 16 and PKD2 is found on chromosome 4. Note: APDK1 the childhood gene is found on chromosome 6 |
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Why does mutation of PDK1 and PKD2 necessitate polycystic kidney disease?
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PDK1 mediates contact inhibition of cell growth and PDK2 is responsible for allowing Ca++ into the cell to invoke growth mechansms. Possibly both of them work together to regulate intracellular Ca++. Lack ofregulation of Ca++ influx leads to too much growth
- sustained fluid secretion and cell growth leads to cysts |
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in adult polycystic kidney disease where else in the body do cysts show up?
Name 3 other abnormalities that are common in APKD |
In the liver!
-intracranial berry aneurysms affecting the circle of Willis -cardiac valcular abnormalities (mitral prolapse) - 40% die from coronary and hypertensive diseases |
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In APKD
- are the kidneys large or small? - as cysts get larger what can happen? - can you palpate the cysts? - onset of the disease is seen with? |
kidneys can get huge!
cyst enlargement can encroach on the pelvis leading to obstruction YES you can palpate the cysts insidious hematuria, proteinuria and hypertension |
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What gene is responsible for polycystic disease in childhood? Is the inheritance dominant or recessive?
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APKD1 - autosomal recessive
- codes for fibrocystin-1 which has a possible role in collecting duct and biliary differentiation |
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Is childhood polycystic disease more or less malignant than adult version?
Is it normally bilateral or unilateral? |
More malignant!
invariantly bilateral whereas the adult version is sometimes unilateral |
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What are the salient histological features of childhood polycystic kidney disease?
What other organ system is invariably involved and how? What happens to those that actually survive infancy? |
dilated channels at right angles to the cortical surfaces. Cysts have cuboid epithelial suggesting collecting duct etiology
invariably there are cysts in the liver with protal hypertension and proliferation of biliary ducts (recall the APKD-1 protein encodes fibrocystin-1 that is responsible for biliary duct proliferation) They get congenital hepatic fibrosis |
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Is medullary Sponge disease malignant or benign?
Histological salient features? |
benign
papillary ducts are dilated with small cysts lined by urothelium or or cuboid epithelium |
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Nephronophthisis-Medullary Cystic Disease
Affects mostly children or adults? What is the initial injury? What is responsible for the renal insufficiency? How does it present? Salient Histo features? |
Most children and some adults
tubular injury - disease is also calleded hereditary tubulointerstitial nephritis children present with polydipsia and polyuria due to lack of ability to concentrate urine. even though cysts are present the renal insufficiency is due to tubular atrophy Cysts are found mainly at the corticomedullary junction surrounded by fibrous tissue |
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on Nephronophthisis-Medullary Cystic Disease
What genes are responsible for adult vs childhood disease? Are they autosomal dominant or recessive? What do the kidneys look like on gross inspection? |
NPH1,2,and 3 genes responsible for autosomal recessive childhood disease.
MCKD1,2 genes responsible for adult autosomal dominant disease - death happens later in life. The kidneys are small! These are the only cystic diseases where the kidneys get smaller |
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What two problems are people on chronic dialysis prone to?
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worst case scenario is that 7% of dialyzed tend to get renal cell carcinoma after 10 years.
many patients get cysts filled with calcium oxalate crystals |
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how can you tell a siple benign cyst from a malignant cyst?
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they are avascular, have smooth contours, and give fluid signals on ultrasonography.
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