Kidney-chapter 20 Flashcards
What is the most common mediator/cause of glomerular diseases
Immunologically mediated
What are most tubular and interstitial disorders disorders frequently caused by
Toxic or infectious agents
What is defined as increased BUN and creatinine, usually related to decreased GFR
Azotemia
When is prerenal azotemia encountered
When there is hypoperfusion of the kidneys that impairs renal function in the absence of parenchyma damage
What is the term for azotemia plus other metabolic and endocrine alterations resulting from renal damage
Uremia
What disorder is a nephritic syndrome with rapid decline (hours to days) in GFR
Rapidly progressive glomerulonephritis
What is the definition of acute kidney injury
Rapid decline in GFR, increased BUN, creatinine, most severe- oliguria and anuria
What is the definition of end-stage renal disease
GFR less than 5% of normal
What domain of the GBM is important for helix formation and for assembly of collagen monomers into the basement suprastructure
NC1
What denotes the accumulation of material that is homogenous and eosinophilia by light microscopy
Hyalinosis
What is a common end result of various forms of glomerular damage
Hyalinosis
What is sclerosis characterized by
Deposition of extracellular collagenous matrix
What are the manifestations of fluid and electrolyte balance in chronic renal failure
Dehydration, Edema, hyperkalemia, metabolic acidosis
What are the cardiopulmonary manifestations of chronic renal failure
HTN, CHF, cardiomyopathy, pulmonary edema, uremia pericarditis
What disease is induced by immunizing rats with an antigen (megalin) that is present in epithelial cell foot processes
Heymann nephritis
What is the antigen that underlies most cases of primary human membranous nephropathy
M type PLA2R
What is heymann nephritis characterized by
Presence of numerous discrete subepithelial electron dense deposits
What is the pattern in IF of heymann nephritis
Granular
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
What complement pathway is activated in dense deposit disease
Activation of alternative complement pathway
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
What disease is defined as tubulointerstitial injury, manifested by tubular damage and interstitial inflammation
Tubulointerstitial fibrosis
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
What is acute proliferative glomerulonephritis defined as
Cluster of diseases characterized by diffuse proliferation of glomerular cells associated with influx of leukocyte
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
