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TI and Cystic Disorders > TI and Cysts > Flashcards

TI and Cysts Flashcards

1
Q

What are the features of Fanconi syndrome?

A
  • LMW proteinuria
  • Glycosuria
  • Bicarbinaturia
  • Phosphaturia
  • Uricosuria
  • Carnitinuria
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2
Q

What LMW proteins are secreted in the urine of persons with FS?

A
  • Amino acids
  • B2 microglobulin
  • Cystatin C
  • A1-macroglobulin
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3
Q

Why does phosphaturia tend to disappear in late FS?

A
  • Generally only a feature of early FS

- Disappears as disease progresses as a new steady state is achieved where loss = intake

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4
Q

What are the features of carnitine deficiency?

A
  • poor fatty acid metabolism
  • reduced antioxidant activities
  • poor glycemic control
  • osteoporosis
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5
Q

What diseases are associated with Fanconi syndrome?

A
  • Cystinosis
  • Galactosaemia
  • Hereditary fructose intolerance
  • Tyrosinaemia T1
  • Wlison disease
  • Lowe syndrome
  • Heavy metals (lead, cadmium, mercury, platinum)
  • Drugs (cisplatin, ifosfamide, gent, rifampin, tenofovir, didanosine, acyclovir, valproate, ranitidine)
  • Glue sniffing
  • MM
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6
Q

What drugs are associated with crystalluria at an alkaline urinary pH?

A
  • Ciprofloxacin (>7), needles, stars
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7
Q

What drugs are associated with crystalluria at an acidic urinary pH?

A
  • Sulfadiazine (<5.5), shock of wheat
  • Indinavir (5.5-7)
  • Methotexate
  • Triamterene
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8
Q

What is the pathophysiology of ATIN?

A
  • Inciting agent acts as a happen
  • Binds to an otherwise non-immunogenic kidney
  • Antibodies formed against the inciting agent bind the kidney and induce an inflammatory immunological response.
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9
Q

What other disease processes are associated with eosinophiluria?

A
  • UTIs
  • Prostatitis
  • Bladder malignancy
  • RPGN
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10
Q

What is the draw-back of Wrights stain over Hansel’s stain?

A
  • Wrights stain is dependent on urinary pH
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11
Q

What are the features of allopurinol associated nephrotoxicity?

A
  • ATIN and granulomas
  • TEN
  • Hepatic necrosis
  • Cholangiitis
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12
Q

What are the associations (infectious) of granulomatous ATIN?

A
  • Fungi
  • Mycobacteria
  • Parasites
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13
Q

Anaemia in CTIN is out of proportion to reduction in eGFR. True/False

A

True. This effect is believed to be secondary to damage to the peritubular EPO producing cells

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14
Q

What are the features of Li-induced kidney injury?

A
  • CTIN
  • medullary and cortical cysts
  • Interstitial fibrosis
  • FSGS
  • MCD
  • Nephrogenic DI
  • Distal RTA
  • Hypercalcaemia
  • Hypothyroidism
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15
Q

What are the infection related causes of CTIN?

A
  • Malakoplakia
  • Xanthogranulomatous pyelonephritis
  • HIV IRIS
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16
Q

What is the aetiology of malakoplakia?

A
  • Bacterial
  • Fungal
  • Mycobacteria
17
Q

What is the management of malakoplakia?

A
  • Surgical
  • Antibiotics (quinolone, TMP-SMX)
  • Bethanchol
  • Vit C
18
Q

What are the features of lead CTIN?

A
  • Anaemia with basophilic stippling
  • Gout
  • CKD
  • Peripheral motor neuropathy
  • Cerebellar calcifications
  • Small kidneys
19
Q

What are the features of Cadmium toxicity?

A
  • Hypercalciuria
  • Kidney stones
  • Prox tubular dysfunction
  • Anaemia
  • CKD
20
Q

What are the features of uCKD?

A
  • Mild proteinuria
  • Hyperuricaemia
  • Hypokalaemia
21
Q

What gene mutations are associated with ADTID?

A
  • Umod
  • Muc1
  • Ren
  • HNF1b
22
Q

What are the features of REN mutations?

A
  • Anaemia
  • Early hyperuricaemia
  • Gout
  • Hypovolaemia
23
Q

Which genetic mutation produces a more severe disease phenotype in TS?

A
  • TSC2
24
Q

What is Caroli disease?

A
  • Isolated hepatic fibrosis with non-obstructive dilation of the intrahepatic bile ducts.
25
Q

What are the risks for progression in ADPKD?

A
  • TKV > 600ml per metre of patient’s height or KL >17cm
  • uACR
  • Hypertension
  • Male
  • LBW
  • Higher copeptin level
26
Q

What is the most common stone type in ADPKD?

A
  • uric acid
27
Q

What are the indications for Tolvaptan in ADPKD?

A
  • eGFR > 25 with one of the following:
  1. Mayo class 1c, 1d, or 1e
  2. Kidneys >16.5cm
  3. Aged <55 and eGFR <65
  4. PROPKD >6

TI and Cystic Disorders (1 decks)

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