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Nephrology > Distal Renal Tubular Disorders - Ault > Flashcards

Distal Renal Tubular Disorders - Ault Flashcards

1
Q

What are the targets of the following drugs?

Acetazolamide

Furosemide, bumetanide, ethacrynic acid

Thiazide

Amiloride, tramterene

Spironolactone, eplerenone

A

Acetazolamide - carbonic anhydrase

Furosemide, bumetanide, ethacrynic acid - Na-K-2Cl

Thiazide - NaCl

Amiloride, tramterene - ENaC

Spironolactone, eplerenone - Mineralocorticoid receptor

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2
Q

What are the mutations present in Bartter syndrome?

A

N-K-2Cl

ROMK

Cl- channel (CLCNKA, CLCNKB)

Barttin (Cl channel)

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3
Q

What are the mutations present in Gitelman Syndrome?

A

Thiazide-sensitive NaCl channel

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4
Q

What are the mutations in Liddle Syndrome?

A

Gain of function in ENaC

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5
Q

What is TTKG and what is the equation?

A

Transtubular Potassium Gradient

TTKG = (UK * Posm) / (PK * Uosm)

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6
Q

What are expected values of the TTKG in hypokalemia and hyperkalemia?

A

Hypokalemia < 2

Hyperkalemia > 8

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7
Q

What is the effect of hyperaldosteronism on potassium?

A

Hypokalemia

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8
Q

What are causes of hyperaldosteronism?

A

Primary (tumors)

Dehydration (pyloric stenosis)

Sodium-wasting disorders (Bartter-Gitelman)

Glucocorticoid-remediable hyperaldosteronism (GRA)

Apparent mineralocorticoid excess (AME)

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9
Q

What is the cause of glucocorticoid-remediable hyperaldosteronism (GRA)?

A

Recombination protducing hybrid molecule that makes ldosterone in response to stress

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10
Q

What are the effects of glucocorticoid-remediable hyperaldosteronism (GRA)?

A

Low renin hypertension

Hypokalemia

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11
Q

What is the cause of apparent mineralocorticoid excess (AME)?

A

Increased renal cortisol

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12
Q

What are the effects of apparent mineralocorticoid excess (AME)?

A
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13
Q

What is the effect of hypoaldosteronism and pseudohypoaldosteronism on potassium?

A

Hyperkalemia

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14
Q

What are the causes of hypoaldosteronism?

A

Congenital adrenal hypoplasia

Congenital adrenal hyperplasia

Autoimmune

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15
Q

What are the causes of pseudohypoaldosteronism?

A

PHA Type I

PHA Type II

Tubular injury (obstructive uropathy)

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16
Q

What are symptoms of PHA Type I?

A

Hyperkalemia

Hyponatremia

Hypotension

17
Q

What are the causes of PHA Type I?

A

Mutation in mineralocorticoid receptor

Loss of function of ENaC

18
Q

What is the treatment of PHA Type I?

A

Sodium supplements

High fluid intake

K-binding resin

19
Q

What are symptoms of PHA Type II?

A

Hyperkalemia

Hyperchloremic metabolic acidosis

Hypertension

“Gordon syndrome”

20
Q

What are the causes of PHA Type II?

A

Mutations in NaCl cotransporter

21
Q

What is the treatment of PHA Type II?

A

Thiazide diuretics

22
Q

What should you check first in metabolic acidosis?

A

Plama anion gap

Na - (Cl + CO2)

23
Q

What causes elevated anion gap acidosis?

A

Ketoacidosis

Lactic acidosis

Metabolic errors

Poisons (ethylene glycol)

24
Q

What causes normal anion gap acidosis (hyperchloremic metabolic acidosis)?

A

GI losses of bicarbonate

Exogenous chloride

Renal tubular acidosis

25
Q

What principle does TTKG measure?

A

Aldosterone response in hypo/hyper kalemia

26
Q

What is the anion gap in RTA?

A

Normal anion gap acidosis

27
Q

What are the types of RTA?

A

Type II (proximal) - associated with Fanconi

Type I (classical distal) - very rare

Type IV (hyperkalemic) - syndrome of aldosterone deficiency

28
Q

What are the tests for RTA?

A

Fractional excretion of bicarbonate

Urine pH

Urine anion gap

U-B PCO2

29
Q

What are the fractional excretions of bicarbonate in each type of RTA?

A

Type I (classical distal) - <5%

Type II (proximal) - >10-15%

Type IV (hyperkalemic) - <5-10%

30
Q

What is the urine pH in each type of RTA?

A

Type I (classical distal) - >5.5

Type II (proximal) - <5.5

Type IV (hyperkalemic) - either

31
Q

What is the equation for urine anion gap?

A

Na + K - Cl

32
Q

What is the value of the urine anion gap in diarrhea?

A

Negative

33
Q

What is the value of the urine anion gap in RTA?

A

Positive

34
Q

What is the U-B PCO2?

A

Measure of secreted CO2 following a HCO3 load

35
Q

What are the results of a U-B PCO2 in each type of RTA?

A

Type I (classical distal) - <10-15

Type II (proximal) - 10-15

Type IV (hyperkalemia) - <10-15

36
Q

What is a distinguishing feature of Type II (proximal) RTA?

A

Fanconi syndrome

37
Q

What is a distinguishing feature of Type IV (hyperkalemic) RTA?

A

High plasma K

38
Q

What causes RTA type IV?

A

Mineralocorticoid deficiency / resistance

Renal tubular dysfunction

Nephrology (18 decks)

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