Kidney-chapter 20 Flashcards by Anna Woods

What is the most common mediator/cause of glomerular diseases

Immunologically mediated

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What are most tubular and interstitial disorders disorders frequently caused by

Toxic or infectious agents

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What is defined as increased BUN and creatinine, usually related to decreased GFR

Azotemia

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When is prerenal azotemia encountered

When there is hypoperfusion of the kidneys that impairs renal function in the absence of parenchyma damage

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What is the term for azotemia plus other metabolic and endocrine alterations resulting from renal damage

Uremia

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What disorder is a nephritic syndrome with rapid decline (hours to days) in GFR

Rapidly progressive glomerulonephritis

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What is the definition of acute kidney injury

Rapid decline in GFR, increased BUN, creatinine, most severe- oliguria and anuria

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What is the definition of end-stage renal disease

GFR less than 5% of normal

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What domain of the GBM is important for helix formation and for assembly of collagen monomers into the basement suprastructure

NC1

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What denotes the accumulation of material that is homogenous and eosinophilia by light microscopy

Hyalinosis

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What is a common end result of various forms of glomerular damage

Hyalinosis

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What is sclerosis characterized by

Deposition of extracellular collagenous matrix

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What are the manifestations of fluid and electrolyte balance in chronic renal failure

Dehydration, Edema, hyperkalemia, metabolic acidosis

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What are the cardiopulmonary manifestations of chronic renal failure

HTN, CHF, cardiomyopathy, pulmonary edema, uremia pericarditis

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What disease is induced by immunizing rats with an antigen (megalin) that is present in epithelial cell foot processes

Heymann nephritis

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What is the antigen that underlies most cases of primary human membranous nephropathy

M type PLA2R

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What is heymann nephritis characterized by

Presence of numerous discrete subepithelial electron dense deposits

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What is the pattern in IF of heymann nephritis

Granular

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What is the GBM antigen responsible for classic anti-GBM antibody induced glomerulonephritis and goodpasture syndrome

Component of the NC1 of the alpha3 chain of type 4 collagen

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What complement pathway is activated in dense deposit disease

Activation of alternative complement pathway

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What disease is defined as progressive fibrosis involving portions of some glomeruli developing after many types of renal injury and leads to proteinuria and increased functional impairment

Focal segmental glomerulosclerosis

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What disease is defined as tubulointerstitial injury, manifested by tubular damage and interstitial inflammation

Tubulointerstitial fibrosis

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What disease is often characterized in the glomeruli, usually presenting with hematuria, red cell casts in the urine, azotemia, oliguira, and mild to moderate HTN

Nephritic syndrome

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What is acute proliferative glomerulonephritis defined as

Cluster of diseases characterized by diffuse proliferation of glomerular cells associated with influx of leukocyte

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How long after strep infection does acute proliferative glomerulonephritis typically develop

1-4 weeks after infection

Who is acute proliferative glomerulonephritis most common in

Kids age 6-10

How do adults with acute proliferative glomerulonephritis typically present

With sudden HTN and/or edema and elevated BUN

What is the characteristic EM finding in acute postrreptococcal glomerulonephritis

Humps appearance (discrete, amorphous, electron-dense deposits on the epithelial side of the membrane)

What is the lab finding in poststreptococcal glomerulonephritis

Elevated antistreptococcal antibody titers and decline in serum concentration of C3

What disease is rapid and progressive loss of renal function associated with severe oliguria

Rapidly progressive (crescentic) GN

What is an example of a type 1 rapidly progressive crescentic GN

Goodpasture syndrome

What is an example of type III pauci-immune nephritic disease

ANCA, granulomatosis with polyangiitis

Describe the kidneys in RPGN

Enlarged and pale, often with petechial hemorrhages on cortical surfaces

What is the histology picture of RPGN dominated by

Distinctive crescents

What type of fluorescence does goodpasture syndrome show

Linear GBM

What are the clinical findings of RPGN

Hematuria with RBC casts in urine, moderate proteinuria, variable HTN and edema

What is nephrotic syndrome caused by

Derangement in glomerular capillary walls resulting in increased permeability to plasma proteins

What are the common clinical findings with nephrotic syndrome

Massive proteinuria, hypoalbuminemia, generalized edema, hyperlipidemia and lipiduria

How does lipid appear in the urine in nephrotic syndrome

Oval fat bodies

What is the most frequent cause of nephrotic syndrome in kids

Minimal change disease

Describe the IF of minimal change disease

Negative

Describe the principle lesion in minimal change disease

In visceral epithelial cells; sometimes termed fusion of foot processes

What nephrotic disease is known to have good response to corticosteroids

Minimal change disease

Describe the basement membrane in membranous nephropathy

Basement membrane material is laid down between deposits, appearing as irregular spikes protruding from GBM

What is the most common form of nephrotic syndrome in adults

Focal segmental glomerulosclerosis

Describe the hallmark epithelial damage/change in focal segmental glomerulosclerosis

Hyalinosis and sclerosis stem from entrapment of plasma proteins in extremely hyperpermeable foci and increased ECM deposition

What does the NPHS1 gene involved is FSGS encode

Protein nephrin

What do mutations of NPHS1 give rise to

Congenital nephrotic syndrome of Finnish type

What does NPHS2 encode (involved in FSGS)

Podocin

What does mutation in NPHS2 result in

Syndrome of steroid-resistant nephrotic syndrome of childhood onset

What disease is associated with double contour or tram track appearance

Membranoproliferative GN

Who does membranoproliferative GN typically occur in

Young adults or adolescence

Describe the issues in the complement pathway with dense deposit disease

Decreased C3 but normal C1 and C4; decreased factor B and proper sin

What is the predominant pattern of injury in dense deposit disease

Mesangial proliferative pattern

What immunoglobulin is typically absent on IM in dense deposit disease

IgG

What is another name for IgA nephropathy

Berger disease

What is the major characteristic of Berger disease

IgA deposits in the mesangial regions

What defect is believed to be associated with Berger disease

Defect in O-linked glycans

What can occur in individuals with Berger disease that are children

Henoch-schonlein purpura

What is the abnormality in Alport syndrome

Alpha chains in type 4 collagen

Describe the morphology of Alport syndrome

Basket-weave apperance; GBM shows irregular foci with thickening alternating with attenuation and pronounced splitting and lamination of the lamina densa

What is the most common presenting sign of Alport syndrome

Hematuria

When do symptoms of Alport syndrome typically appear

Age 5-20

Describe the kidneys in chronic glomerulonephritis

Symmetrically contracted and have diffusely granular cortical surfaces

What portions of the kidney are especially vulnerable to acute tubular injury/necrosis

Straight portion of proximal tubule and ascending thick limb

What causes marked ballooning and hydrophobic or vacuolar degeneration of proximal convoluted tubules

Ethylene glycol

What does acute pyelonephritis usually present with

Sudden onset pain at the CVA and systemic evidence of infection (fever, malaise)

What does reflux nephropathy occur as a result of in early childhood

Result of superimposition of urinary infection on congenital vesicoureteral reflux and nitrate all reflux

What type of pyenolonephritis is sometimes confused with renal carcinoma

Xathnogranulomatous pyelonephritis

Describe lab findings with bile cast nephropathy

Serum bilirubin markedly elevated, with bile cast formation in distal nephron segment

What is the consequence of narrowing with benign nephrosclerosis

Patchy ischemic atrophy

What is a common pathological finding with malignant nephrosclerosis

Fibrinoid necrosis and onion-skinning

What in the plasma is markedly elevated in patients with malignant HTN

Renin

What disease has flea-bitten appearance on cortical surface

Malignant nephrosclerosis

What is the most common cause of renal artery stenosis

Atherosclerosis

What is the Pentad for TTP

Fever, neuro symptoms, microangiopathic hemolytic anemia, thrombocytopenia, renal failure

What are the most common abnormalities with sickle cell nephropathy

Hematuria and diminished concentration ability (hyposthenuria)

What are the Defects in with adult polycystic kidney disease

Defects in mechanosensing, calcium flux, and signal transduction

What gene is commonly knocked ou with adult polycystic kidney disease

PKD1

What are most cases of childhood polycystic kidney disease due to mutation of

PKHD1

What does PKHD1 encode

Fibrocystin

Describe the morphology with childhood polycystic kidney disease

Enlarged and have smooth external surface; spongelike appearance

How does nephronopthisis often present in kids

Polyuria and polydipsia' sodium wasting and tubular acidosis often present

What is the most common characteristic with multicystic renal hyperplasia

Presence of islands of undifferentiated mesenchyme

What do the cysts commmonly contain with acquired (dialysis associated) cystic disease

Calcium oxalate crystals

Describe the appearance of renal papillary adenoma

Small, invariable within cortex and appear grossly as yellow-gray, discreet well circumscribed nodules

What is the tubular sclerosis associated with angiomyolipoma characterized by

Lesions of the cerebral cortex

What are oncocytomas composed of

Large eosinophilia cells having small, round benign=appearing nuclei that have large nucleoli; tan or mahogany brown

What is the buzz word for collecting duct carcinoma

Hobnail pattern

What are chromophone renal carcinoma cells

Pale eosinophilic cells

What are the classic clinical features with renal cell carcinomas

Costovertebral pain, palpable mass, hematuria

what congenital kidney issue is defined as conjoined kidney usually connected at lower pole

horseshoe kidney

what is included in potter sequence

lung hypoplasia, flat face with lowset ears, development of defects in extremities

what is the non-inherited congenital malformation of renal parenchyma characterized by cysts and abnormal tissue

dysplastic kidney

what is the inherited defect of the kidney presenting as bilateral enlarged kidneys with cysts in renal cortex and medulla

PKD

how does autosomal recessive PKD present

in infants, worsening renal failure and HTN

how does autosomal dominant PKD present

in adults as HTN, hematuria, worsening renal failure

what is the often cause of death in autosomal dominant PKD

berry aneurysms

describe the result of medullar cystic kidney disease

inherited autosomal dominant defect that the parenchymal fibrosis results in shrunken kidneys and worsening renal failure

what is sen in the urine with acute tubular necrosis

brown granular casts

what is the major hallmark of nephrotic syndrome

proteinuria

what does the hypoalbuminemia of nephrotic syndrome lead to

decrease oncotic pressure, leading to edema

what is the most common cause of nephrotic syndrome in african americans and hispanics

FSGS

what is the hallmark of nephritic syndrome

glomerular inflammation and bleeding

how does alport syndrome clinically present

thinning and splitting of glomerular basement membrane; presents as isolated hematuria, sensory hearing loss, ocular disturbances

describe the presentation of a patient with nephrolithiasis

colicky pain with hematuria and unilateral flank tenderness

what renal neoplasma is defined as hamartoma comprised of blood vessels, smooth muscle, and adipose tissue; increased frequency in tuberous sclerosis

angiomyolipoma

what is the clinical triad associated with renal cell carcinoma

hematuria, palpable mass, flank pain

what does von hippel-lindua disease increase the risk for

hemangioblastoma of cerebellum and renal cell carcinoma

what is von hippel lindua disease caused by

autosomal dominant inactivation of VHL gene

what is wilms tumor comprised of

blastoma, primitive glomeruli and tubules, stroll cells

who is wilms tumor most common in

kids

how does someone with a wilms tumor typically present clinically

large unilateral flank mass with hematuria and HTN

what does WAGR syndrome stand for

wilms tumor, aniridia, genital abnormalities, mental/motor retardation

where does urothelial carcinoma typically occur

bladder

what is the major risk factor for urothelial carcinoma

cigarette smoke

what is the classic presentation of urothelial carcinoma in older adults

painless hematuria