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Pathophysiology - Urinary Tract > Systemic/Hereditary/Vascular Renal Diseases > Flashcards

Systemic/Hereditary/Vascular Renal Diseases Flashcards

1
Q

What is the most common disorder leading to end stage renal disease in the US?

A

Diabetic nephropathy

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2
Q

What is the pathogenesis of diabetic nephropathy? What will happen to GFR?

A

Nonenzymatic glycosylation of the glomerular basement membrane as well as arterioles, predominantly the efferent arteriole. Leads to hyaline arteriolosclerosis of vessels and sclerosis of mesangium

-> increases GFR initially, followed by decrease in GFR later in the disease

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3
Q

How does diabetic nephropathy lead to sclerosis? What is the most important cytokine mediating this?

A

Mesangium expands due to increased rates of ECM formation and decreased rates of degradation.

Most of these actions are mediated by TGF-beta, which downregulates ECM degrading enzymes, and upregulates their inhibitors.

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4
Q

When is renal biopsy performed for diabetic nephropathy?

A

Rarely because the morphology is pretty much always the same, but may be for:

  1. Early onset disease, or more rapid clinical progress (i.e. within 10 years of disease diagnosis)
  2. Severe proteinuria without other associated diabetic changes - (i.e. diabetic nephropathy) - might be something else going on
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5
Q

What light microscopic changes of the glomeruli accompanies diabetic nephropathy?

A

Diffuse and nodular glomerulosclerosis

  • > GBM thickening
  • > Exaggerated mesangial expansion, with characteristic nodules
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6
Q

What are the features and what is the name for the characteristic nodules of diabetic nephropathy?

A

Frank, hypocellular nodules with eosinophilic material

-> Kimmelstiel-Wilson nodules

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7
Q

What is the most common early manifestation of diabetic nephropathy
(DN)?

A

Proteinuria -> hyperfiltration leads to microalbuminuria

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8
Q

What are the immunofluorescence features of diabetic nephropathy? What might it be confused with and how can it be differentiated?

A

Linear deposition of IgG and albumin

Looks like ant-GBM disease

  • > differentiated by lack of necrosis or inflammation
  • > DN is not immune-complex mediated, IgG is likely artifact from immunoglobulins interacting with abnormal basement membrane
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9
Q

What is the characteristic TEM picture of diabetic nephropathy?

A

Uniform thickening of GBM without immune complex deposition

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10
Q

What are the two types of large protein deposits which can happen in DN and where do they deposit?

A

Fibrin cap - hyaline mass of protein along periphery of glomerulus (caps the capillary)

Capsular drop: Droplet of protein-containing material within Bowman’s capsule (between parietal cells of capsule)

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11
Q

What happens to the tubules and interstitium in DN?

A

Prominent thickening of GBM can occur, but tubular atrophy due to glomerulosclerosis is most common.

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12
Q

How can the hyalinosis caused by diabetes mellitus be differentiated from hyalinosis caused by systemic hypertension?

A

In DM:
Hyalinosis of both afferent and efferent arterioles

In hypertension:
Only afferent arteriole is affected.

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13
Q

What will the larger vessels of the kidney show pathologically in DN?

A

Severe atherosclerosis -> diabetes is a risk factor for atherosclerosis

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14
Q

What are complications of DN?

A
  1. Pyelonephritis - diabetes predisposes to ascending bacterial or viral infection (think because its very sugary)
  2. Renal papillary necrosis - due to progressive ischemia
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15
Q

What type of kidney disorder occurs in systemic amyloidosis? What are the two main kinds of amyloid?

A

Nephrotic syndrome
-> primarily nephrotic-range proteinuria, but over half of patients may also have renal insufficiency as well (increase in serum creatinine)

  1. Primary amyloidosis (AL)
  2. Secondary amyloidosis (SAA)
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16
Q

What type of birefringence does amyloid show?

A

Apple-green

-> different colors due to heterogenous reaction to polarized light when shown by Congo red

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17
Q

What is the most commonly involved organ in systemic amyloidosis, and where does amyloid tend to deposit in the kidney? How does this cause dysfunction?

A

The kidney
-> tends to deposit in the mesangium, but can deposit anywhere

Causes dysfunction by compressing and decreasing luminal area of surrounding capillaries

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18
Q

What does amyloid deposition in the glomerulus resemble under the microscope and how do you differentiate from this?

A

Looks like amorphous hyaline material depositing in the mesangium, just like diabetes

Differentiated via clinical picture and Congo red stain, (or immunofluoresence to kappa/lamda light chains or SAA protein)

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19
Q

How does amyloid appear by electron microscopy?

A

Thin, non-branching fibrils

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20
Q

What is one infection particularly associated with secondary amyloidosis?

A

Tuberculosis

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21
Q

What is myeloma cast nephropathy / what causes it?

A

Disease caused by reaction of Bence Jones protein (light chain, in patients with multiple myeloma) with Tamm-Horsfall protein
-> large, occlusive casts clog the tubules

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22
Q

How does myeloma cast nephropathy appear under light microscope?

A

Tubules within the cortex / medulla have expanded, large, pink casts with cracked or fractured appearance
-> can often have swirling / lamelled character like psammoma bodies

23
Q

How is myeloma cast nephropathy (myeloma kidney) treated?

A

Treat multiple myeloma

Alkalinize urine

Increase free water excretion

Avoid nephrotoxic insults like radiocontrast media (reason why it can’t be used in patients with MM)

24
Q

A kid comes in with bloody diarrhea and acute renal failure with sudden onset. What do they most likely have and what is mediating it?

A

Hemolytic uremic syndrome
-> mediated by Shigatoxin, produced by S. dysenteriae and E.coli O157:H7

Inhibits 60S ribosome subunit

25
Q

What is the pathogenesis of HUS?

A

Shigatoxin damages endothelial cells in kidney -> swelling + detachment of endothelial cells -> decrease in capillary luminal area with deposition of platelets and fibrin

Leads to microangiopathic hemolytic anemia, thrombocytopenia, and renal symptoms

26
Q

How can TTP be acquired and what are the symptoms?

A

Acquired via auto-antibody to ADAMTS13 which is a vWF metalloprotease

Fever, thrombocytopenia, microangiopathic hemolytic anemia, renal symptoms, and neurologic symptoms-> headaches, seizures, confusion, hemiparesis

27
Q

What are some causes of thrombotic microangiopathies which are not TTP/HUS?

A
  1. SLE - anticardiolipin Abs
  2. Malignant hypertension - barotrauma
  3. Drugs - cyclosporine, tacrolimus (calcineurin inhibitors)
  4. Radiation, pre-eclampsia, other causes of endothelial damage
28
Q

What does LM show for thrombotic microangiopathies?

A

Platelet and fibrin thrombi in glomerular capillaries and arterioles
-> can generally seen blood

29
Q

What are the two most common causes of renal artery stenosis and why do these cause hypertension?

A
  1. Atherosclerosis
  2. Fibromuscular dysplasia

Increased activation of RAA system due to decreased RBF

30
Q

What type of fibromuscular dysplasia is most common and who tends to get it? How can you tell what type it is?

A

Medial (expansion of the media) -> can tell by looking where the internal elastic lamina is

Tends to be in young adult females

31
Q

What will pathology will result from fibromuscular dysplasia in the kidneys?

A

Ipsilateral - mild hyaline arteriolosclerosis

Contralateral - severe arteriolosclerosis

32
Q

What causes autosomal dominant polycystic kidney disease? What is the age of onset?

A

Defect in polycystin 1 or polycystin 2 which are ciliary proteins
-> ciliary dysfunction

Age of onset is adulthood (cysts slowly progress)

33
Q

What are the clinical manifestions of ADPKD? One of these is a cardiac condition.

A

Cysts in the liver, brain, and kidney:

Liver - benign hepatic cysts
Brain - berry aneurysms of Circle of Willis -> may rupture
Kidney - Cysts destroying renal parenchyma, hypertension due to renin secretion
Heart - mitral valve prolapse

34
Q

What will the kidneys show for ADPKD?

A

Grossly enlarged kidneys which may cause back pain -> large numbers of spherical cysts throughout the parenchyma
-> normal landmarks are difficult to see

35
Q

Where do the cysts arise from in ADPKD vs ARPKD?

A

ADPKD - can arise from any duct (cuboidal epithelium)

ARPKD - arise from collecting duct**

36
Q

What are the clinical manifestations of ARPKD and what is the function of the missing protein? When does it present?

A

Missing protein - another nonmotile cilia

Presents in infancy, often prenatally

  • > congenital hepatic fibrosis leading to portal hypertension
  • > renal failure and hypertension
37
Q

What are babies with ARPKD at risk for in the womb?

A

Potter sequence - oligohydramnios due to renal insufficiency

38
Q

What is the treatment for ARPKD, and what is the most common cause of death?

A

Renal transplant

Most common cause of death is lung hypoplasia -> Potter sequence

39
Q

How does ARPKD appear grossly and microscopically?

A

Grossly - Maintained reniform shape, with radially oriented cylindrical tubules which extend through cortex, and are often dilated (collecting ducts)

Microscopically - cystically dilated collecting ducts, but glomeruli and proximal tubules appear normal (although compressed)

40
Q

What is Fanconi’s syndrome characterized by? What type of Rickets does it cause?

A

Proximal renal tubular acidosis, and generalized malabsorption of everything at proximal tubule

Characterized by hypophosphatemic rickets (vitamin-D resistant)

41
Q

What is thought to be the underlying mechanism of Fanconi’s syndrome?

A

Defect in cellular energy metabolism resulting in a decrease in ATP levels -> impairing secondary transport mechanisms

42
Q

What are the inherited causes of Fanconi’s syndrome?

A
  1. Cystinosis
  2. Wilson’s disease
  3. Tyrosinemia
  4. Glycogen storage diseases
43
Q

What are the acquired causes of Fanconi’s syndrome?

A

Lead poisoning
Multiple myeloma
Ingestion of expired tetracyclines
Tenofovir

44
Q

What renal condition is usually unilateral but when bilateral may be easily confused with autosomal recessive polycystic kidney disease? How is it inherited? What is the pathogenesis?

A

Multicystic dysplastic kidney

  • > it is actually a non-inherited congenital abnormality
  • > usually unilateral
  • > Ureteric bud fails to induce differentiation of metanephric mesenchyme
45
Q

How does multicystic dysplastic kidney look microscopically? How can you easily tell it apart from ARPKD?

A

Cysts and connective tissue forming duct-like structures, surrounded by smooth muscle. May be some metaplastic cartilage.

Difference from ARPKD: there will be no normal reniform appearance to the kidney, all dysplastic

46
Q

What is the most common type of kidney stone?

A

Calcium oxalate stones or calcum oxalate + phosphate

47
Q

What are the most common causes of calcium stones?

A
  1. Conditions of high calcium:
    - Idiopathic hypercalcemia
    - Hypercalciuria with hypercalcemia (bone disease, sarcoidosis, hyper-PTH)
  2. Conditions associated with high oxalate:
    - Crohn’s disease -> small bowel damage increases oxalate resorption
    - vegetarians -> diet rich in oxalates
48
Q

Are calcium stones radiotranslucent or radioopaque? How about uric acid stones?

A

Calcium stones - radio-opaque

Uric acid stones - radiotranslUcent

49
Q

What is another name for ammonium magnesium phosphate stones and what conditions precipitate them?

A

Triple stones

  • > precipitated by high pH, usually caused by urease+ bacteria, leading to urine alkalinization
  • > i.e. Proteus, Klebsiella
50
Q

What type of kidney stones is ammonium magnesium phosphate known for forming?

A

Staghorn caliculi -> occupy a large part of the renal pelvis

May also occur in cystinuria

51
Q

Who tends to get uric acid stones?

A
  1. Patients with gout
  2. Patients with tumor lysis syndrome / rapid cell turnover (i.e. leukemia treatment)
  3. Patients with very acidic urine
52
Q

Which types of stones are particularly prone to precipitate in acidic urine?

A

Uric acid stones, cystine stones
-> both types are radiotranslucent and can be treated with acetazolamide for alkalinization

Calcium OXALATE stones are also associated with acidic urine, but not precipitated by it

53
Q

What causes cystinuria?

A

Autosomal recessive PCT cystine-reabsorbing transporter

Poor absorption of four amino acids:
COLA
Cystine
Ornithine
Lysine
Arginine
54
Q

What precipitates calcium oxalate stones?

A

Hypocitraturia, also associated with low urine pH

  • > citrate is needed to bind free calcium, and prevent its precipitation
  • > decreased citrate levels in the urine are associated with metabolic acidosis, which increases citrate reabsorption (we want TCA precursors to be able to use all the lactic acid)

Pathophysiology - Urinary Tract (14 decks)

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