TI and Cysts Flashcards
What are the features of Fanconi syndrome?
- LMW proteinuria
- Glycosuria
- Bicarbinaturia
- Phosphaturia
- Uricosuria
- Carnitinuria
What LMW proteins are secreted in the urine of persons with FS?
- Amino acids
- B2 microglobulin
- Cystatin C
- A1-macroglobulin
Why does phosphaturia tend to disappear in late FS?
- Generally only a feature of early FS
- Disappears as disease progresses as a new steady state is achieved where loss = intake
What are the features of carnitine deficiency?
- poor fatty acid metabolism
- reduced antioxidant activities
- poor glycemic control
- osteoporosis
What diseases are associated with Fanconi syndrome?
- 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
What drugs are associated with crystalluria at an alkaline urinary pH?
- Ciprofloxacin (>7), needles, stars
What drugs are associated with crystalluria at an acidic urinary pH?
- Sulfadiazine (<5.5), shock of wheat
- Indinavir (5.5-7)
- Methotexate
- Triamterene
What is the pathophysiology of ATIN?
- 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.
What other disease processes are associated with eosinophiluria?
- UTIs
- Prostatitis
- Bladder malignancy
- RPGN
What is the draw-back of Wrights stain over Hansel’s stain?
- Wrights stain is dependent on urinary pH
What are the features of allopurinol associated nephrotoxicity?
- ATIN and granulomas
- TEN
- Hepatic necrosis
- Cholangiitis
What are the associations (infectious) of granulomatous ATIN?
- Fungi
- Mycobacteria
- Parasites
Anaemia in CTIN is out of proportion to reduction in eGFR. True/False
True. This effect is believed to be secondary to damage to the peritubular EPO producing cells
What are the features of Li-induced kidney injury?
- CTIN
- medullary and cortical cysts
- Interstitial fibrosis
- FSGS
- MCD
- Nephrogenic DI
- Distal RTA
- Hypercalcaemia
- Hypothyroidism
What are the infection related causes of CTIN?
- Malakoplakia
- Xanthogranulomatous pyelonephritis
- HIV IRIS
What is the aetiology of malakoplakia?
- Bacterial
- Fungal
- Mycobacteria
What is the management of malakoplakia?
- Surgical
- Antibiotics (quinolone, TMP-SMX)
- Bethanchol
- Vit C
What are the features of lead CTIN?
- Anaemia with basophilic stippling
- Gout
- CKD
- Peripheral motor neuropathy
- Cerebellar calcifications
- Small kidneys
What are the features of Cadmium toxicity?
- Hypercalciuria
- Kidney stones
- Prox tubular dysfunction
- Anaemia
- CKD
What are the features of uCKD?
- Mild proteinuria
- Hyperuricaemia
- Hypokalaemia
What gene mutations are associated with ADTID?
- Umod
- Muc1
- Ren
- HNF1b
What are the features of REN mutations?
- Anaemia
- Early hyperuricaemia
- Gout
- Hypovolaemia
Which genetic mutation produces a more severe disease phenotype in TS?
- TSC2
What is Caroli disease?
- Isolated hepatic fibrosis with non-obstructive dilation of the intrahepatic bile ducts.
What are the risks for progression in ADPKD?
- TKV > 600ml per metre of patient’s height or KL >17cm
- uACR
- Hypertension
- Male
- LBW
- Higher copeptin level
What is the most common stone type in ADPKD?
- uric acid
What are the indications for Tolvaptan in ADPKD?
- eGFR > 25 with one of the following:
- Mayo class 1c, 1d, or 1e
- Kidneys >16.5cm
- Aged <55 and eGFR <65
- PROPKD >6
