Interstitial and cystic kidney disease Flashcards

1
Q

what is the most severe form of cystic renal dysplasia?

A

multicystic dysplastic kidney

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2
Q

what is the most dangerous complication from ADPKD?

A

subarachnoid aneurysm

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3
Q

who gets screened for aneurysms?

A
  • family history

- normal renal function with PCKD

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4
Q

tuberous sclerosis is a systemic disease characterized by multiple ____________

A

hamartomas

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5
Q

what is pathognomonic for tuberous sclerosis? what is the most common finding?

A
  • cortical brain tubers

- angiomyolipomas

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6
Q

what are the most common clinical manifestations of VHL?

A

retinal angiomas and cerebellar hemangiomas

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7
Q

what are the manifestations of medullary cystic disease?

A
  • sodium wasting
  • anemia
  • renal insufficiency
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8
Q

what is used to diagnose medullary cystic disease?

A
  • excretory urography
  • sonography and CT scan
  • open renal biopsy
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9
Q

what are the manifestations of medullary sponge kidney?

A
  • gross and microscopic hematuria
  • urinary tract infections
  • nephrolithiasis
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10
Q

what is the pathology in medullary sponge kidney?

A
  • defective urinary solute concentrating ability
  • inability to reduce urinary pH to 5.5
  • renal tubular acidosis
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11
Q

what is the histology in medullary sponge kidney?

A
  • spherical, oval, or irregular enlargement of medullary and inner papillary portions of collecting tubules
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12
Q

what are the 3 types of tubulointerstitial injury?

A
  • acute tubular necrosis
  • acute interstitial nephritis
  • chronic interstitial nephritis
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13
Q

what is the classic triad of acute interstitial nephritis?

A
  • low grade fever
  • skin rash
  • arthralgias
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14
Q

what are the lab abnormalities in acute interstitial nephritis?

A
  • elevation in BUN and creatinine
  • abnormal UA
  • eosinophilia
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15
Q

what is the gold standard test for diagnosing acute interstitial nephritis?

A

biopsy

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16
Q

what are the pathological features of acute interstitial nephritis?

A
  • infiltration of inflammatory cells (lymphocytes, PMNs, eosinophils) in interstitium
  • edema
  • glomeruli and blood vessels are usually spared
17
Q

what drug classes can cause drug induced acute (and chronic) interstitial nephritis?**

A
  • abx
  • NSAIDs
  • diuretics
  • miscellaneous
18
Q

what is the presentation for chronic interstitial nephritis?

A
  • slowly progressing renal insufficiency
  • normal or sterile pyuria UA
  • mild proteinuria
  • HTN and anemia with moderate to advanced disease
19
Q

what are the pathological features of chronic interstitial nephritis?

A
  • atrophy of tubular cells with flattened epithelial cells and tubule dilation
  • interstitial fibrosis
  • cellular infiltration within the interstitial compartment and between tubules