KSAP September 2020

Question 1

Answer 1

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

Question 2

Patency of AVG is improved by combination therapy with aspirin-dipyramidole. True/False

Answer 2

True

Question 3

Nephrolithiasis in dRTA is predominantly of what type?

Answer 3

Calcium phosphate

Question 4

What is primary oxaluria?

Answer 4

• Autosomal recessive disorder
• Associated with calcium oxalate monohydrate stones
  *

Question 5

What percentage of patients with Dent’s disease have nephrocalcinosis?

Answer 5

75%

Question 6

What is the cause for AKI with chronic ketamine use?

Question 7

What is Fabry disease?

Answer 7

• 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%

Question 8

What is the treatment for Fabry’s disease?

Answer 8

• Enzyme replacement therapy with alpha galactosidase A
• Migalastat, in patients with suitable Gla mutations

Question 9

What is migalistat?

Answer 9

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.

Question 10

What is the main risk associated with the use of dopamine as a vasopressor?

Answer 10

Tachyarrhythmia

Question 11

What is a renal dose of dopamine?

Answer 11

<5mcg/kg/min- selective agonism of D1 receptors in heart, lungs, and kidney leading to vasodilation

Question 12

What was the principle finding from the SEPSISPAM study?

Answer 12

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.

Question 13

What type of crystals are associated with propfol?

Answer 13

Uric acid crystals

Propofol is structurally similar to probenacid, hence has uricosuric effect

Question 14

What change does propofol cause in the colour of urine?

Answer 14

It changes it to a green colour

Cloudy pink urine suggests presence of uric acid crystals

Question 15

What was the principle finding of the DOPPS study WRT dialysis bicarbonate levels?

Answer 15

Bicarbonate levels >38 were associated with increased mortality

Question 16

What is the issue in giving IV Ca to a patient with hypophophataemia?

Answer 16

It can make it worse, hence phosphate should be replete before giving IV Ca2+

Question 17

What is the benefit of using belatacept in a post-transplant immunosuppression regimen?

Answer 17

Less incidence of post transplant DM

Question 18

What is the difference between relapsing and recurrent peritonitis?

Answer 18

• 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.

Question 19

What is the benefit of mupirocin in tunneled dialysis catheters?

Answer 19

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%.

Question 20

What is the significance of a negative urine anion gap?

Answer 20

It suggests GI losses and intact renal mechanisms

Question 21

What type of RTA is cystinosis associated with?

Answer 21

Proximal RTA

Question 22

What is Balkan endemic nephropathy?

Answer 22

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.

Question 23

How do thiazides work in nDI?

Answer 23

• ECF volume depletion
• Increased reabsorption of H20 and Na+ in proximal tubule

Question 24

What type of kidney injury is associated with pamidronate?

Answer 24

• Collapsing FSGS

Question 25

What is the recommended UF rate with chronic HD?

Answer 25

13ml/kg/hr maximum

Question 26

What is the best treatment for reducing the frequency of attacks in hypokalaemic periodic paralysis?

Answer 26

Acetazolamide

Question 27

What mutation in HPP is most likely to respond to acetazolamide?

Answer 27

Patients with CACNA1Smutations may be more likely to respond to acetazolamide therapy.

Question 28

What are the recommendations for HD in pregnancy?

Answer 28

• >36 hrs per week (assoc with 85% live birth rate)
• Keep BUN <50