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Renal/Respiratory > Pathology: Kidney 3 > Flashcards

Pathology: Kidney 3 Flashcards

1
Q

Systemic diseases associated with nephrotic syndrome

A

– Diabetic nephropathy
– Amyloidosis
– Light chain deposition disease

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2
Q

Hereditary glomerular disease

A

– Alport syndrome

– Thin basement membrane disease

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3
Q

Diabetic Nephropathy is the leading cause of what?

A

-Leading cause of ESRD in most Western societies

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4
Q

diabetic nephropathy caues/risk

A

-Can occur in both DM Type I and Type II
– Risk is related to duration of disease
-Risk is multifactorial
– 30-40% of patients will develop nephropathy

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5
Q

pathogenesis of diabetic nephropathy-characteristic of DN

A

hyperfiltration

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6
Q

hyperfiltration characteristics

A
  • Common in early diabetes
  • ↑ GFR due to glucose- dependent afferent arteriolar dilation
  • Angiotensin II mediated constriction of the efferent arteriole
  • Hyperfiltration increases colloid osmotic pressure in the post-glomerular capillaries
  • ↑Na reabsorption in the PT
  • Angiotensin II causes hypertrophic PT growth
  • Can be corrected with good glycemic control
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7
Q

pathogenesis of hypertrophy in diabetic nephropathy

A
  • Seen early in onset
  • The size of the kidney may increase by several centimeters.
  • Associated with an increase in the number of mesangial cells and of capillary loops
  • Increasing filtration surface area.
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8
Q

pathogenesis of mesangial changes in diabetic nephropathy

A
  • The hallmarks of DN
    • mesangial expansion
    • nodular diabetic glomerulosclerosis (the acellular Kimmelstiel-Wilson lesion),
  • Early mesangial lesion is characterized by a increase in mesangial cell number and size and increased deposition of extracellular matrix.
  • Mesangial expansion is mediated by both glucose and glucose-derived AGEs.
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9
Q

pathogenesis of proteinuria in diabetic neuropathy

A

• Widening of the GBM
• accumulation of type IV collagen and net reduction in negatively charged heparin sulfate.
• The podocyte changes
• Increased width of the foot
processes.
• Apoptosis triggered by ANG II and TGF-B
• Migration reduced by ANG II preventing coverage of the BM
• Serum proteins cross the BM due to the disrupted texture, gaps, and holes

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10
Q

diabetic nephropathy on EM

A

BM and podocyte changes

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11
Q

pathogenesis of fibrosis in diabetic nephropathy

A

• Tubulointerstitial fibrosis is seen early in DN
• correlates with prognosis.
• Caused by release of growth factors:
• TGF-β, ANG II
• Tubular cells change their phenotype and
become fibroblasts.
• High glucose concentration and AGEs further stimulate this process.

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12
Q

features of stage 1 of DN

A 
Stage 1: Onset of diabetes
• GFR increase due to glomerular hyperfiltration
• glomerular hypertrophy seen on biopsy
• renal size
• Reversible, transient albuminuria
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13
Q

features of stage 2 of DN

A

Stage 2: Clinically asymptomatic, but biopsy shows
• mesangial expansion
• GBM thickening

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14
Q

features of stage 3 of DN

A

Stage 3: Early nephropathy
• development of hypertension
• persistent microalbuminuria by 24-hr collection
• Urinary albumin excretion 30-300 mg/day

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15
Q

features of stage 4 of DN

A

Stage 4: Overt proteinuria
• urinary albumin > 300 mg/day
• GFR starts to decline
• 50% of patients will reach ESRD within 7-10 years
• Retinopathy present in 90-95% of patients

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16
Q

features of stage 5 DN

A

Stage 5: End-stage renal disease
• Renal replacement therapy necessary
• Occurs a mean of 15 years after onset of Type 1 DM in patients who develop proteinuria (30%)

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17
Q

co-morbidities of DM

A
  • HTN
  • Neuropathy
  • Vascular changes
  • Increased mortality
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18
Q

DN and HTN-potential mechanisms leading to HTN in T2DM

A

exogenous factors–>obesity–>insulin resistance, hyperleptinemia–>sympathetic activation–>HTN

genetic factors–>obesity, T2DM–>insulin resistance, hyperleptinemia–>sympathetic activation–>HTN

genetic factors–> T2DM–> hyperglycemia–> microvasculopathy, AngiotensinII–> HTN

genetic factors–>T2DM–>dyslipidemia–> microvasculopathy–>HTN

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19
Q

DN and complications

A
  • Diabetic retinopathy
  • Polyneuropathy
  • Macrovascular complications
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20
Q

Diabetic retinopathy

A
  • in almost all patients with type 1 diabetes and nephropathy.
  • In 50% to 60% of type 2 diabetes with nephropathy
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21
Q

Polyneuropathy

A
  • Sensory polyneuropathy: Diabetic foot
    • Autonomic polyneuropathy
    • Silent angina
    • Gastroparesis
    • erectile impotence
    • detrusor paresis
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22
Q

Macrovascular complications

A

(5X more frequent)
• Stroke
• coronary heart disease
• peripheral vascular disease

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23
Q

DN and mortality with normoalbuminuria, microalbuminuria, and macroalbuminuria

A
  • low mortality rates with normoalbuminuria
  • higher mortality rates with microalbuminuria
  • mortality greatly increased with macroalbuminuria!
24
Q

treatment of DN

A
  • HTN therapy
  • Glucose control
  • Reduction of proteinuria • Lipid lowering therapy
  • Life style modification
25
Q

HTN prevelance in DM pts with DN

A

In DM pts with DN, HTN is almost always present

26
Q

uncontrolled HTN is associated with…

A
  • more rapid progression of DN
  • increased risk of fatal and nonfatal CV events.
  • antihypertensive therapies improve survival in both type 1 and type 2 diabetics with DN.
27
Q

The current recommended blood pressure target for all diabetics?

A

is below 140/90 mm Hg

• Was 130/80 before JNC 8

28
Q

glucose control and DN

A

•Studies suggest that with good glycemic control helps
• Decrease risk of progression
•Results from the Diabetes Control and Complications Trial
(DCCT)
• reduction in progression from normoalbuminuria to microalbuminuria
• Decrease in and other microvascular complications, specifically retinopathy
• Decrease in cardiovascular (CV) sequelae, this persisted despite later deterioration of the glycemic control.
•Euglycemia that followed isolated transplantation of the pancreas was associated with a regression of the diabetic glomerulosclerosis. (N Engl J Med 1998; 339:69-75)

29
Q

Reducing proteinuria in DN

A
  • Renin-angiotensin-aldosterone system blockade
  • Renoprotective independent of BP
  • Reduces proteinuria
  • May cause up to 30% decline in GFR but renoprotective in the longterm
  • Works through renal hemodynamic changes and blocking non-hemodyanmic effects of Ang II
30
Q

lipid control in DN

A 
• Most patients with DN have:       
     – Low HDL
     – High TGs
     – Smaller LDL particle
• In type 2 diabetic patients with DN, treatment with statins provides substantial CV benefit
     – Not seen in DN with ESRD 
• Current guidelines
     – LDL <70 mg/dl for diabetic patients with CVD
31
Q

Lifestyle modifications for DN

A

• Smoking cessation
– Decreases progression of micro to macro albuminuria
• weight reduction
– Possibly improves renal outcome via reduction in proteinuria

32
Q

Non-diabetic nephrotic

syndromes

A

Amyloidosis

Light chain deposition disease

33
Q

Amyloidosis characterization

A

• Amyloidosis is a generic term for a family of diseases defined by morphologic criteria.
– Characterized by the deposition in extracellular spaces of a proteinaceous material

34
Q

Amyloidosis affecting the kidney characteristics

A

– light chains, secreted by a single clone of B cells
• 20% of cases associated with multiple myeloma
– Usually lambda light chains (AL)
– Systemic amyloidosis (AA) results from chronic inflammation
• Not discussed

35
Q

Kidney manifestations of amyloidosis

A

• kidney is often enlarged and hypertension is absent even when renal function is impaired.
• Proteinuria, mainly albuminuria, occurs in the absence of microscopic hematuria.
• Tubular defects from amyloid deposits
– Renal tubular acidosis (mostly as a part of Fanconi
syndrome)
– polyuria-polydipsia (resulting from urinary concentration defect)

36
Q

amyloidosis on LM

A

deposits seen

37
Q

amyloidosis with Congo-red stain

A

apple green birefringence

38
Q

amyloidosis on IM

A

staining for light chain

39
Q

Extra-renal manifestations of amyloidosis

A

• AL amyloidosis may infiltrate almost any organ other than the brain
– Restrictive cardiomyopathy in 1/3 of pts
– Gastrointestinal tract
• motility disturbances, malabsorption, hemorrhage, or obstruction
– Macroglossia
– Splenomegaly
– Peripheralnerve
• sensory polyneuropathy, autonomic neuropathy(orthostatic hypotension, lack of sweating, bladder dysfunction, impotence)
– Skin
• Purpura (around the eyes) papules, nodules, and plaques, occurring
usually on the face and upper trunk.
– Joint
• Shoulder pain and swelling

40
Q

Light Chain Deposition Disease characteristics

A

• Deposition of excess immunoglobulin light chains in the kidney
– usually 
– 50% of cases coexist with multiple myeloma

41
Q

what do patients with light chain deposition disease develop?

A

– proteinuria
– hematuria
– chronic renal insufficiency

42
Q

LCDD on LM

A

nodular glomerulosclerosis

43
Q

LCDD on IF

A

light chain staining

• Kappa staining

44
Q

LCDD on EM

A

granular deposits along GBM

45
Q

Hereditary glomerular diseases

A
  • Alport Syndrome

- Thin Basement Membrane

46
Q

Alport Syndrome

A

• Most commonly X-linked recessive (80%) but can be autosomal recessive as well
• Mutation of the COL4A5 gene on chromosome Xq22 which encodes the alpha5 chain of type IV collagen
– defect in the basement membrane

47
Q

renal manifestations of Alport Syndrome

A

• Hematuria
– Affected males have persistent microscopic hematuria
– episodicgrosshematuria,precipitatedbyURI
– Present in the first two decades of life.
– More than 90% of females with XLAS have persistent or intermittent microscopic hematuria, but about 7% of obligate heterozygotes never manifest hematuria
• Proteinuria is absent early but develops eventually in all males with XLAS and in both males and females with ARAS.
• Hypertension
• ESRD develops in all affected males with XLAS (90% by age 40)
–rate determined by the underlying COL4A5 mutation.
– Females with XLAS 12% developed ESRD before the age of 40, 30% by age 60 years, 40% by age 80 years

48
Q

extra-renal manifestations of Alport Syndrome

A

• Cochlear defects
– adherence defect of the organ of Corti to the basilar membrane
– 80% of males
– 20-30% of female
• Ocular defects
– 30% to 40% of XLAS males
– 15% of XLAS females
– Anterior lenticonus, pathognomic (15% of males, associated with ESRD by 30 years)
– maculopathy, whitish or yellowish flecks or granulations in a perimacular distribution
• Leiomyomatosis (less common)
– esophagus and tracheobronchial tree

49
Q

pathology of Alport Syndrome on LM

A

LM: Early in disease, glomeruli may appear normal. Later global and segmental glomerulosclerosis, interstitial fibrosis

50
Q

pathology of Alport Syndrome on IF

A

IF: Negative or non-specific IgM, C

51
Q

pathology of Alport Syndrome of EM

A

EM: variable thickening, thinning, basket weaving, and lamellation of the GBM

52
Q

treatment of Alport Syndrome

A 
• No disease-specific therapy
     – RAAS blockade
• Renal replacement is eventually necessary
• Transplant
     – 2-3% will get anti-GMB disease
53
Q

Thin Basement Membrane Disease (Benign Familial Hematuria) characteristics

A

• Usually autosomal dominant inheritance
• Continuous or intermittent microhematuria, with or without gross hematuria, and generally no renal insufficiency
• Previously considered benign
– Proteinuria, HTN and ESRD are unusual
• Extra-renal features are rare

54
Q

pathologic findings of TBMD on LM

A

LM: normal glomeruli

55
Q

pathologic findings of TBMD on IF

A

negative

56
Q

pathologic findings of TBMD on EM

A

___________???

57
Q

treatment for TBMD

A

• Reassurance
• Should be followed
– Very small but real risk of progression to ESRD
– BMP, urinalysis and BP monitored every 1-2 years

Renal/Respiratory (8 decks)

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