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OS Renal > Disorders of the Distal Tubule > Flashcards

Disorders of the Distal Tubule Flashcards

1
Q

Bartter’s syndrome pathogenesis

A

Defect in Cl- reabsorption in the NK2C cotransporter, or K+ channel ROMK, or Cl- channel, or Barttin (needed to insert Cl channels)

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2
Q

Bartter’s syndrome is similar in effect to which kind of diuretic?

A

Loop

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3
Q

In Bartter’s syndrome, which ions will be altered and how so?

A

Hypochloremia
Hypokalemia
Hyponatremia

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4
Q

What happens to levels of aldosterone and renin in Bartter syndrome?

A

Both up

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5
Q

What tissue changes occur in Bartter’s syndrome and why?

A

Hyperplasia of juxtaglomerular apparatus due to elevated prostaglandin activity

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6
Q

How is Bartter’s inherited?

A

AR

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7
Q

What are some S/S for neonatal Bartter syndrome?

A

Fetal polyuria causing polyhydramnios
Amniotic fluid - elevated PGE2
Salt wasting at birth
Calcium stones and hypercaliuria

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8
Q

Gitelman’s Syndrome is similar to which diuretic?

A

Thiazides

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9
Q

How is Gitelman’s inherited?

A

AR

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10
Q

Gitelman’s effects on ion levels

A

Hypomagnesemia
Hypokalemia
Hypocalciuria

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11
Q

What kind of acid-base disorder to Bartter’s syndrome patients usually have?

A

Hypochloremic metabolic alkalosis

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12
Q

Gitelman’s defect

A

Mutated Na/Cl cotransporter in DCT

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13
Q

What is the typical age of presentation for Bartter’s and for Gitelman’s?

A

Bartter’s is young

Gitelman’s is adults

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14
Q

Explain Gitelman’s pathogenesis

A

Defective Na/Cl symporter in DCT causes increased distal Na delivery, increasing K secretion and H secretion (leading to hypokelamia and alkalosis). The distal delivery increase also increases excreted sodium, leading to RAAS activation.

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15
Q

Gitelman’s S/S

A

Hypomagnesemia
Hypokalemia
Hypocalciuria
Hypochloremic metabolic alkalosis

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16
Q

Is Gitelman’s or Bartter’s more severe and why?

A

Bartter’s is more severe because more sodium wasting since earlier in tubule

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17
Q

Liddle syndrome defect

A

Gain of function of ENaC leading to constitutive activation

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18
Q

Liddle syndrome treatment

A

Salt restriction and diuretics

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19
Q

Liddle syndrome inheritance

A

AD

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20
Q

Liddle syndrome S/S

A 
Hypertension
Low renin
Low aldosterone
Hypokalemia
Metabolic alkalosis
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21
Q

What are some causes of hypokalemia?

A 
Diuretics
Hyperaldosteronism (1 or 2ndary)
22
Q

What are some causes of hyperkalemia?

A

Hypoaldosteronism

Pseudohypoaldosteronism

23
Q

What does the TTKG measure?

A

estimates the ratio of potassium in the lumen of the CCD to that in the peritubular capillaries

24
Q

What are the expected values for TTKG for hypo- and hyperkalemia?

A

Hypokalemia: 8

25
Q

What is a primary cause of hyperaldosteronism?

A

Adrenal tumors

26
Q

What are some secondary causes of hyperaldosteronism?

A

Dehydration
Bartter
Gitelman

27
Q

What is the defect in glucocorticoid-remediable hyperaldosteronism?

A

Recombination of 11BHSD and aldosterone synthase

28
Q

GRA pathogenesis

A

Fusion protein produces hybrid molecule that binds aldosterone receptors in times of stress, causing low renin hypertension and hypokalemia

29
Q

GRA treatment

A

Glucocorticoids

30
Q

Apparent mineralocorticoid excess pathogenesis

A

Mutant kidney enzyme 11BHSD causes increased cortisol in kidney which cross-reacts to activate aldosterone receptor.

Leads to low renin hypertension and hypokalemia, and treated with glucocorticoids

31
Q

S/S of PHA Type I

A

Hyperkalemia
Hyponatremia
HYPOTENSION thru volume depletion

32
Q

PHA Type I defects -2

A

AD mutant mineralocorticoid receptor
or
AR ENaC always off mutation

33
Q

PHA Type I treatment

A

NA supplementation
Lots of fluids
K-binding resins

34
Q

PHA Type II S/S

A

Hyperkalemia
Hyperchloremic metabolic acidosis
HYPERTENSION
Low RAAS

35
Q

PHA Type II treatment

A

thiazide diuretics

36
Q

PHA Type II defect

A

Mutated WNK1 and WNK4 which regulate Na/Cl cotransporter (thiazide target)

37
Q

What are some types/causes of elevated anion gap acidosis?

A

Ketoacidosis
Lactic acidosis
Inborn metabolic errors
Poisons like methanol and ethylene glycol

38
Q

What are some causes of normal gap acidosis?

A

GI loss of bicarb through diarrhea
Exogenous Cl?
**Renal tubular acidosis

39
Q

Type II renal tubule acidosis

A

Associated with Fanconi syndrome

Proximal tubule damage

40
Q

Type I renal tubule acidosis

A

Distal tubule

Very rare

41
Q

Type IV renal tubule acidosis

A

Hyperkalemic

Aldosterone deficiency or unresponsiveness

42
Q

What are some tests for RTA?

A

Fractional excretion of bicarbonate
Urine pH
Urine anion gap
Urine vs blood pCo2

43
Q

What are the expected values of fractional bicarb excretion for the different types of RTA?

A

Proximal RTA (II): <5-10%

44
Q

What are the expected values of urine pH for the different types of RTA?

A 
Proximal RTA (II): 5.5
Hyperkalemic (IV): either
45
Q

What is the formula for urine anion gap

A

Na + K - Cl

46
Q

What would urine anion gap analysis show for the types of RTA?

A

All would show positive value (normal gap)

47
Q

How is U-B pCO2 used to differentiate between types of RTA?

A

Proximal RTA: normal of >10
Type I and IV: abnormally low bicarb clearance

Low values mean lots of bicarb combined with the excess H and was turned into H20 + CO2

48
Q

Explain a quick differential diagnosis for the types of RTA

A

Is there Fanconi? Type II
Is there hyperkalemia? Type IV
If neither one, then it is Type I

49
Q

Defect in Type IV RTA

A 
Mineralocorticoid deficiency
Tubular dysfunction from obstruction or lupus
Aldosterone resistance (pseudohypo...)
50
Q

Does RTA have a negative or normal anion gap?

A

Normal anion gap acidosis

OS Renal (25 decks)

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