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KSAP Oct 2019 > KSAP Oct 2019 > Flashcards

KSAP Oct 2019 Flashcards

1
Q

What is eosinophilic serositis?

A

It is a benign condition that occurs in patients on PD, it normally occurs within the first few months of initiating PD.

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2
Q

How do you diagnose PD peritonitis?

A

> 100 cells/mm3 and >50% PMNs

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3
Q

What is the genetic mutation in TS?

A

Mutations in TSC1 or TSC2 which code for hamartin and tuberin, respectively.

TSC1 is located on chromosome 9
TSC2 is located on chromosome 16 and TSC2 is contiguous with PKD1.

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4
Q

What are the functions of hamartin and tuberin?

A
  • Translocation of polycystin 1 to the cilium of the collecting duct cells;
  • down-regulation of mTOR
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5
Q

What follow-up imaging is required for small stable AMLs in TS?

A

MRI every 1-3 years

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6
Q

What are the indications for mTOR inhibitors (everolimus) in TS?

A

AML ≥ 3cm, symptomatic, increasing in size AML

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7
Q

Why does the risk of haemorrhage into an AML increase during pregnancy or on OCP?

A

They contain a high concentration of oestrogen and progesterone receptors.

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8
Q

What is the indication for embolisation in AML in TS?

A
  • AML ≥4cm,
  • acute haemorrhage, or
  • intra-AML aneurysm 5mm
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9
Q

How do you estimate renal electrolyte-free water loss?

A

CH20 = V ( 1 - [Una + Uk/Sna])

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10
Q

What percentage of hepatic cyst infections in ADPKD are secondary to G+ organisms or anaerobes?

A

15%

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11
Q

What is the best antimicrobial combination for ADPKD cyst infection?

A

Quinolone and cephalosporin, but quinolone is often enough

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12
Q

What is the mechanism for metformin associated lactic acidosis?

A

Impairs mitochondrial function, leads to anaerobic glycolysis.

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13
Q

What is the procedure for examining acid-base status?

A
  1. First look at the pH, 2. then look at the HCO3- and PCO2 levels, 3. Calculate the anion gap, 4. Apply Winter’s formula, 5. Calculate the delta AG : delta HCO3
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14
Q

What is Winter’s formula?

A

pCO2 (est) = 1.5 x HCO3 + 8 +/-2

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15
Q

What is the use of delta AG to delta HCO3 ratio?

A

Typically, the delta:delta is between 0.7 and 1.7. For every 1mEq/L increase in the anion gap, you would expect a concurrent decrease of roughly the same magnitude in the HCO3 concentration. If the delta:delta is less than expected, suspect a concurrent NAGMA. If greater than expected, is there a second cause for AG metabolic acidosis?

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16
Q

What are the viral risk factors for post transplant hyperglycaemia?

A

CMV and Hep C

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17
Q

HbA1c is useful in the 1st year post-transplant. True/False

A

False. Underestimated because of enhanced Hb production

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18
Q

What is the phenotype of pseudohypoaldosteronism Type II?

A

Young, Caucasian male presenting with hypertension, hyperkalaemia, and a mild metabolic acidosis

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19
Q

What is the cause for pseudohypoaldosteronism Type II?

A

Loss of function mutation in WNK 4 or gain of function mutations in WNK 1; these mutations lead to enhanced function of ENaC and profound inhibition of the ROMK.

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20
Q

What occurs to the Plasma Renin Activity in primary hypoadrenalism?

A

It tends to be high on account of volume depletion

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21
Q

What are the mutations in hypokalaemic periodic paralysis?

A
  • point mutation in the alpha subunit of calcium dihydropyridine-sensitve channel (90%),
  • mutation in the skeletal muscle sodium channel, SCN4A (10%).
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22
Q

What is the cause of pseudohypoaldosteronism type 1?

A

AD disorder caused by a loss of function mutation in the mineralocorticoid receptor.

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23
Q

What is the waiting time for transplant post melanoma, myeloma, and breast cancer?

A

5 years

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24
Q

What degree of nocturnal dipping is considered non-dipping?

A

<10%

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25
Q

What are the risk factors for Encapsulatng Peritoneal Sclerosis?

A

PD for >8years, recurrent episodes of PD peritonitis

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26
Q

What are the presenting features of Encapsulating Peritoneal Sclerosis?

A
  • Recurrent episodes of bowel obstruction, -hypoalbuminaemia,
  • failure of PD,
  • anaemia resistant to ESA,
  • elevated inflammatory markers.
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27
Q

What are the treatments for Encapsulating Peritoneal Sclerosis?

A

Surgery to remove the intestinal cocoon, tamoxifen may have a role, TPN.

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28
Q

What is the principle underlying worsening of hyponatraemia in SIADH by the administration of Normal Saline?

A

If the the osmolality of the urine is greater than the osmolality Normal Saline, the inability to dilute urine leads to an increase in electrolyte free water.

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29
Q

What is the phenotype of thyrotoxic periodic paralysis?

A

Male, Asian/Latino, thyrotoxicosis, weakness in limbs, hypokalaemia.

More common than AD form of disease, which is remarkably rare.

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30
Q

How do DKA and toluene toxicity cause hypokalaemia?

A

Increased sodium delivery to the distal nephron principal cells with a non-reabsorbable anion (ketogenic-acid in DKA and hippuric acid in toluene toxicity).

31
Q

In NAGMA secondary to diarrhoea, what would you expect of the urinary anion gap?

A

A negative urinary AG due to an increase in urinary NH4+.

32
Q

Where is the genetic mutation in Bartter syndrome?

A

In the Na-K-2Cl co-transporter

33
Q

Where is the genetic mutation in Gitelman syndrome?

A

In the thiazide sens NaCl co-transporter.

34
Q

How do you differentiate Bartter and Gitelman’s, biochemically?

A

High Ur Ca in Bartters (impaired paracellular reabsorption) and low Ur Ca in Gitelman’s.

35
Q

How do low calcium diets result in increased risk of nephrolithiasis?

A

Increased absorption of oxalate from the gut because of reduced binding with calcium leading to hyperoxaluria.

36
Q

How do you correct creatinine for ECF volume expansion?

A

CF = TBW x Cum daily fluid surplus/ TBW

37
Q

What percentage of adrenal adenomas are (1) cortisol secreting, (2) phaeochromocytomas, (3) aldosterone secreting adenomas?

A

Cortisol secreting 5%
Phaeo 5%
Aldo secreting 1%

38
Q

For a urinary or plasma metanephrine test to be suggestive of phaemochromocytoma, by what degree would you expect the lab level to be above the ULN?

A

At least 2 times the ULN

39
Q

How does fenofibrate cause AKI?

A

(1) Impairment of the renal vasodilatory effects of prostaglandins,
(2) Possible direct interference with Cr secretion,
(3) Increased risk rhabdo with statins
Class effect of fibrates, but observed less frequently with gemofibrizil.

40
Q

What is the normal value for FEMg in hypomagnesaemia?

A

FEMg should be <2% in hypomagnesaemia.

FEMg = Ur Mg x Serum Cr / Serum Mg x Ur Cr

41
Q

Explain the typical course of HepB infection, in terms of the appearance of the various Ab and Ag in the serum.

A

(1) HBSAg, after incubation of 4-10 weeks
(2) HBcAb IgM + HBeAg + HBV DNA
(3) 1-2 months, HBeAg disappears, then HBsAg.
(4) HBsAb appears one month after disappearance of HBsAg, HBcAb shifts from IgM to IgG after 6 months.

42
Q

How might you reduce the risk of AKI requiring dialysis in a patient undergoing a CABG?

A

Perform off-pump CABG. The CORONARY trial randomized more than 4500 patients to on-pump versus off-pump cardiac surgery. The primary end point of dialysis-requiring AKI was indistinguishable between study groups. However, in patients with CKD, the overall incidence of AKI was lower in the off-pump arm, and the relative risk of dialysis-requiring AKI was lower at 30 days. In addition, a 2010 meta-analysis of randomized trials also showed a 40% lower rate of postoperative AKI in off-pump cases that was statistically significant. A 2018 report of over 7000 patients found a significantly higher risk of death and AKI requiring renal replacement therapy in those undergoing on-pump coronary bypass. Controversy persists regarding the possibility of an increased rate of revascularization procedures in patients treated off-pump, but no difference was seen in this 2018 study.

43
Q

What is post-hypercapnia metabolic alkalosis?

A

Occurs in patients with T2RF who experience rapid correction of their PCO2.

Strategies for management:

(1) Infusion of chloride, i.e. NaCl 0.9%
(2) Preventative strategy- maintain baseline hypercapnia

44
Q

What type of kidney stone is associated with topiramate?

A

Calcium PO4

45
Q

What is the mechanism of nephrolithiasis with topiramate?

A

(1) Metabolic acidosis caused by inhibition of Carbonic Anhydrase in the proximal and distal nephron.
(2) Bicarbonaturia and an elevated urinary pH (favouring precipitation of Ca3PO42 crystals)
(3) Hypocitraturia as citrate is reabsorbed more avidly in the proximal tubule
(4) Hypercalciuria resultant from chronic metabolic acidosis.

46
Q

What should you consider before starting a patient on dapsone for PJP prophylaxis?

A

G6PD deficiency; common in the Mediterranean basin.

47
Q

What glomerular lesions are associated parvovirus B19 infection?

A

Collapsing Glomerulopathy; MPGN

48
Q

What drugs are associated with ANCA vasculitis?

A
  • Hydralazine,
  • propylthiouracil,
  • minocycline,
  • Allopurinol,
  • penicillamine,
  • procainamide,
  • methimazole,
  • clozapine,
  • phenytoin,
  • rifampicin,
  • cefotztaime,
  • isoniazid and
  • indomethacin (13)
49
Q

What was the main finding of the CARESS-HF trial?

A

UF not superior to IV diuretics in the management of ACRS1. In fact, preservation of renal function was better at 96 hours with step-wise approach to diuretics.

50
Q

Rifampicin leads to lower serum tacrolimus levels. True/False

A

True. It is an inducer of CYP 3A4.

51
Q

What inhibitors of CYP3A4 could be commenced to increase levels of tacrolimus in a patient receiving rifampicin?

A

Fluconazole
Diltiazem
Clarithromycin

52
Q

What increases risk of HIVAN?

A

Presence of 2 risk alleles for APOL1.

53
Q

What is the effect of carnitine deficiency in dialysis patients?

A
  • Resistance to ESA
  • Hypotension
  • Cardiomyopathy
  • Muscle cramps
  • Inflammation
  • Lipid abnormalities
54
Q

How do you estimate carnitine deficiency in a dialysis patient?

A

By measuring the ratio of acylcarnitine to free carnitine

Ratio >0.4 suggests deficiency

55
Q

What conditions have an effect on serum testosterone measurements?

A

Decrease levels of SHBG:

  • Nephrotic syndrome
  • Obesity
  • DM

Increase levels of SHBG:

  • HIV
  • Cirrhosis
  • Ageing
56
Q

What are the pharmacological options for PJP prophylaxis?

A
  • Septrin
  • Dapsone
  • Pentamidine
  • Atovaquone
57
Q

What percentage of people with Alport syndrome have anterior lenticonus?

A

20%

Another ophth finding is central fleck retionopathy

58
Q

What percentage of men with Alport syndrome have sensorineural hearing loss?

A

90%

59
Q

What is the pathophysiology of TBMD?

A

Individuals with this disorder typically have heterozygous defects in genes for the α chains of type IV collagen such as COL4A3 or COL4A4 and therefore are “carriers” for autosomal recessive forms of Alport’s syndrome

60
Q

How is the diagnosis of Alport syndrome established?

A

The diagnosis can be established by kidney biopsy, skin biopsy, and or by genetic testing.

61
Q

How does Alport syndrome manifest in women?

A

Women are heterozygous carriers of the disease mutation, and nearly all have microscopic hematuria. Women with X-linked Alport syndrome have a better prognosis than men but a significant number can progress to proteinuria, impairment of kidney function, and ESRD.

62
Q

What is the rate of creatinine excretion per day in an adult male?

A

20-25mg/kg of lean body mass per day

63
Q

How do you calculate Dietary Protein Intake?

A

Urea excretion / 1.6

64
Q

What are the features of collagenofibrotic glomerulopathy on biopsy?

A
  • Nodular sclerosis (similar to Kimmelstein Wilson nodules) in LM
  • Organised 60nm deposits on EM
65
Q

Staining for Congo Red is positive in Fibrillary GN. True/False

A

False. Staining for CR will be negative

66
Q

What has a better prognosis, fibrillary or immunotactoid GN?

A

Immunotactoid GN

67
Q

How would you treat a stag horn calculus in a patient who is not a surgical candidate?

A

Acetohydroxamic acid is a urease inhibitor that has been shown to reduce stone growth and progression in patients with residual or recurrent struvite stone disease after surgery, as well as those in whom stone removal is not feasible. Unfortunately, this agent is associated with significant side effects that include palpitations, edema, nausea, vomiting, and diarrhea, among others.

68
Q

Why has alemtuzumab fallen out of vogue as an induction agent in kidney transplant?

A
  • Associated with late rejection episodes

- Increased risk of DSAs

69
Q

What treatments are available for hyperoxaluria?

A
  • Pyridoxine

- Oxalobacter formigenes

70
Q

What is the cause for Pendred syndrome?

A
  • Pendred syndrome is caused by a defect in pendrin, the apical chloride/bicarbonate exchanger in type B (or non-A, non-B) intercalated cells in the collecting duct of the kidneys.
  • autosomal recessive
  • Patients present with sensorineural hearing loss, typically at a young age, and goiter.
  • Patients usually have normal electrolytes, but thiazide administration or vomiting may cause severe metabolic alkalosis and profound hypokalemia, hypomagnesemia, and hyponatremia.
71
Q

What is Dent disease?

A
  • X-linked recessive
  • Hypercalciuric
  • Hypophosphataemic
  • Rickets
  • Nephrocalcinosis
  • Low molecular weight proteinuria
72
Q

What are the genetic mutations in Dent disease?

A
  • Dent disease 1 = inactivating mutation in the lysosomal chloride transporter gene located on the X chromosome, CLCN5.
  • Dent disease 2 = mutation in OCRL1 gene, also on the X chromosome.
  • Lowe oculocerebral renal syndrome is also caused by mutation in the OCRL1 gene, but renal tubular acidosis, congenital cataracts, and intellectual disability characterize this syndrome.
73
Q

What type of kidney disease is associated with MUC-1?

A
  • Defects in MUC1 lead to intracellular accumulation of mucin-1 resulting in chronic interstitial nephritis, formerly known as medullary cystic kidney disease type 1.
  • Gout is prominent, but more typically occurs as kidney function declines, as opposed to uromodulin disease, in which gout is a major feature well before advanced CKD occurs.
  • Inheritance is autosomal dominant.
74
Q

What type of kidney disease is associated with mutation in HNF1β?

A

HNF1β encodes hepatocyte nuclear factor-1β, a transcription factor. Individuals with mutations in this gene can present with chronic interstitial nephritis. Gout is atypical in this disorder.

KSAP Oct 2019 (1 decks)

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