Renal Tubular Acidosis Flashcards

1
Q

Define renal tubular acidosis

A

Hyperchloraemic metabolic acidosis with normal anion gap that occurs due to pathology in the tubules of the kidney

Tubules are responsible for balancing hydrogen & bicarbonate ions between blood and urine to maintain normal pH

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2
Q

There are 4 subtypes of renal tubular acidosis; which two are most relevant to clinical practice & likley to come up in exams?

A

Type 1 & Type 4

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3
Q

What is the most common cause/type of renal tubular acidosis?

A

Type 4 (reduced aldosterone secretion)

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4
Q

Briefly summarise the pathology of each of the types of renal tubular acidosis is:

  • Type 1
  • Type 2
  • Type 3
  • Type 4
A
  • Type 1: distal convoluted tubule (unable to excrete hydrogen ions)
  • Type 2: proximal convoluted tubule (unable to reabsorb bicarbonate)
  • Type 3: combination of type 1 & type 1 hence pathology in proximal and distal convoluted tubule
  • Type 4: caused by reduced aldosterone
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5
Q

Discuss what investigations you would do if you suspect renal tubular acidosis, include:

  • Bedside
  • Bloods
  • Imaging
A

Bedside

  • ABG: pH
  • Urine sample: pH of urine

Bloods

  • U&Es: electrolytes
  • Serum bicarb:
  • Serum chloride:
  • Anion gap:
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6
Q

State some potential causes of type 1 renal tubular acidosis

A
  • Genetic (autosomal dominant & recessive)
  • SLE
  • Sjogren’s syndrome
  • Primary biliary cirrhosis
  • Hyperthyroidism
  • Sickle cell anaemia
  • Marfan’s syndrome
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7
Q

Discuss presentation of type 1 renal tubular acidosis, include biochemical hallmarks of type 1 renal tubular acidosis

A
  • Failure to thrive in children
  • Hyperventilation to compensate for metabolic acidosis
  • Renal stone
  • Bone disease
  • CKD (if left untreated)

Biochemical hallmarks:

  • High urinary pH (usually above 6)
  • Hypokalaemia
  • Metabolic acidosis
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8
Q

Discuss the management of type 1 renal tubular acidosis

A
  • Oral sodium alkali or potassium containing alkaline solution e.g. sodium bicarbonate or sodium citrate (Shohl’s solution)
  • **NOTE: treatment should correct other electrolyte abnormalities however may need potassium containing variant of Shohl’s solution if significant potassium deficit*
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9
Q

What is the main cause of type 2 renal tubular acidosis?

A

Faconi syndrome

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10
Q

What is faconi syndrome?

A

Defect in tubular transport at the PCT. Condition may be idiopathic inherited (possibly alongside other conditions e.g. Wilson’s disease) or acquired (e.g. from tubular damage or nephrotoxic drugs such as cisplatin or aminoglycosides). Faconi renal syndrome causes impaired absorption of potassium, amino acids, bicarbone and glucose.

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11
Q

Discuss the presentation of type 2 renal tubular acidosis, include the biochemical hallmarks

A
  • May present with osteomalacia, polyuria or rickets
  • Biochemical hallmarks:*
  • Hypokalaemia
  • High urinary pH (usually above 6)
  • Metabolic acidosis
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12
Q

Discuss the management of type 2 renal tubular acidosis

A
  • Oral sodium alkali or potassium containing alkaline solution e.g. sodium bicarbonate or sodium citrate (Shohl’s solution)
  • Consider vitamin D
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13
Q

Type 3 renal tubular acidosis is rare; true or false?

A

True

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14
Q

Discuss some potential causes of type 4 renal tubular acidosis

A

Type 4 caused by reduced aldosterone which could be due to:

  • Adrenal insufficiency
  • Medications such as ACEinhibitors, spironolactone etc..
  • Systemic conditions which affect kidneys e.g. SLE, diabetes or HIV
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15
Q

Discuss the biochemical hallmarks of type 4 renal tubular acidosis (4)

A
  • Hyperkalaemia
  • High chloride
  • Metabolic acidosis
  • Low urinary pH
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16
Q

Discuss the management of type 4 renal tubular acidosis

A
  • Treat underlying cause
  • Fludrocortisone to replace mineralocorticoids (if cause is due to mineralocortiocid deficiency)
  • Sodium bicarbonate to treat the acidosis
  • May require treatment for hyperkalaemia: including stopping drugs that cause hyperkalaemia, low potassium diet, diuretics (e.g. loops & thiazides) and usual hyperkalaemia treatment
17
Q

Compare how RTA generally presents in adults vs children

A

In adult patients, the diagnosis of RTA is more often made after the observation of unexplained, incidental laboratory findings in blood and urine acid-base parameters and electrolyte concentrations. In children the diagnosis is commonly identified following investigation for growth retardation, rickets, or failure to thrive. Rarely, patients may present with severe acidosis and Kussmaul’s breathing due to respiratory compensation.