KSAP September 2020 Flashcards
Immunotactoid GN
The diagnosis is established by electron microscopy, which demonstrates characteristic organized fibrillar deposits >30 nm in diameter, arranged in parallel arrays of microtubular structures
Patency of AVG is improved by combination therapy with aspirin-dipyramidole. True/False
True
Nephrolithiasis in dRTA is predominantly of what type?
Calcium phosphate
What is primary oxaluria?
- Autosomal recessive disorder
- Associated with calcium oxalate monohydrate stones
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What percentage of patients with Dent’s disease have nephrocalcinosis?
75%
What is the cause for AKI with chronic ketamine use?
What is Fabry disease?
- X-linked disease
- Lysosomal storage disease
- Accumulation of glycosphingolipids in multiple organs
- Acroparaesthesias, angiokeratomas, ocular abnormalities, anhidrosis, stroke, restrictive cardiomyopathy and progressive CKD
- A deficiency of α-galactosidase A, an enzyme that metabolizes glycosphingolipids, is the primary abnormality
- Diagnosis is usually established by demonstrating an α-galactosidase A activity level in leukocytes of <25–30%
What is the treatment for Fabry’s disease?
- Enzyme replacement therapy with alpha galactosidase A
- Migalastat, in patients with suitable Gla mutations
What is migalistat?
Migalastat is an oral pharmacologic chaperone that binds to and stabilizes specific mutant forms of alpha-galactosidase, thereby facilitating proper trafficking of the enzyme to lysosomes and increasing enzyme activity [58]. In preclinical studies, migalastat was shown to increase alpha-galactosidase A (alpha-Gal A) activity and decrease accumulation of globotriaosylceramide (Gb3) in cultured human Fabry disease cell lines and in animal models of Fabry disease [58]. Migalastat has been approved in the United States and Europe for use as first-line therapy in Fabry patients with amenable galactosidase alpha (GLA) gene variants.
What is the main risk associated with the use of dopamine as a vasopressor?
Tachyarrhythmia
What is a renal dose of dopamine?
<5mcg/kg/min- selective agonism of D1 receptors in heart, lungs, and kidney leading to vasodilation
What was the principle finding from the SEPSISPAM study?
Based on the SEPSISPAM study, a MAP target of 80 mm Hg, if achievable, may prevent worsening of AKI to stage 3 or need for dialysis.
What type of crystals are associated with propfol?
Uric acid crystals
Propofol is structurally similar to probenacid, hence has uricosuric effect
What change does propofol cause in the colour of urine?
It changes it to a green colour
Cloudy pink urine suggests presence of uric acid crystals
What was the principle finding of the DOPPS study WRT dialysis bicarbonate levels?
Bicarbonate levels >38 were associated with increased mortality
What is the issue in giving IV Ca to a patient with hypophophataemia?
It can make it worse, hence phosphate should be replete before giving IV Ca2+
What is the benefit of using belatacept in a post-transplant immunosuppression regimen?
Less incidence of post transplant DM
What is the difference between relapsing and recurrent peritonitis?
- Relapsing peritonitis: a second episode with the same organism within 4 weeks of completion of antibiotics for the initial episode.
- Repeat peritonitis is another episode of peritonitis with the same organism >4 weeks after completion of antibiotics for the initial episode.
What is the benefit of mupirocin in tunneled dialysis catheters?
The application of topical mupirocin ointment to the catheter exit site until healed was found to reduce the rate of bloodstream infection by approximately 85%.
What is the significance of a negative urine anion gap?
It suggests GI losses and intact renal mechanisms
What type of RTA is cystinosis associated with?
Proximal RTA
What is Balkan endemic nephropathy?
Balkan endemic nephropathy is a chronic, progressive tubulointerstitial kidney disease, strongly associated with urothelial cancer, that is prevalent in the southeastern region of Europe along the Danube River. It is thought to relate to chronic, low-level exposure to aristolochic acid (AA), although genetic and other predisposing factors may also play a role.
How do thiazides work in nDI?
- ECF volume depletion
- Increased reabsorption of H20 and Na+ in proximal tubule
What type of kidney injury is associated with pamidronate?
- Collapsing FSGS
What is the recommended UF rate with chronic HD?
13ml/kg/hr maximum
What is the best treatment for reducing the frequency of attacks in hypokalaemic periodic paralysis?
Acetazolamide
What mutation in HPP is most likely to respond to acetazolamide?
Patients with CACNA1Smutations may be more likely to respond to acetazolamide therapy.
What are the recommendations for HD in pregnancy?
- >36 hrs per week (assoc with 85% live birth rate)
- Keep BUN <50
