• This is a metabolic acidosis due to impaired acid secretion by the kidney
  • There is a hyperchoraemic metabolic acidosis with normal anion gap

Type I

  • Distal RTA due to inability the excrete H+ ions and generate acidic urine in the distal tubule, even in states of severe acidosis
  • It may complicate many renal disorders
  • Features include;
    • Rickets or osteomalacia due to buffering of H+ with calcium in bone
    • Nephrocalcinosis with renal calculi
    • This is due to a combination of hypercalciuria from bone, reduced urinary citrate (which inhibits calcium phosphate precipitation) which is reabsopbed to act as a buffer for H+ and an alkaline urine
    • It is also associated with hypokalaemia as there isn’t H+ to exchange for Na so K+ is exchanged instead
  • Causes;
    • Idiopathic
    • Genetic;
      • Ehlers-Danlos
      • Marfan’s
    • Autoimmune disease;
      • SLE
      • Sjogren’s
      • Autoimmune hepatitis
    • Nephrocalcinosis
      • Hypercalcaemia
      • Medullary sponge kidney
    • Tubulointerstitial disease
      • Chronic pyelonephritis
      • Chronic interstitial nephritis
      • Obstructive uropathy
      • Renal transplant rejection
    • Drugs;
      • Lithium
      • Amphotericin
  • Diagnosis
    • Acid load – oral ammonium chloride – there is a failure to acidify the urine beyond pH 5.5
  • Treatment is with oral sodium bicarbonate or citrate

 

Type II

  • Proximal RTA due to a defect in bicarbonate reabsorption in the proximal tubule resulting in excess bicarbonate in the urine
  • The tubules are able to reabsorb some bicarbonate so can acidify urine during systemic acidosis
  • It is rarer than type I
  • May be an isolated thing or may be associated with Fanconi’s anaemia
  • Hypokalaemia is common – the bicarbonate of the urine causes and osmotic diuresis causing increased flow rate to the distal tubule and therefore increased K+ excretion
  • Causes;
    • Idiopathic
    • Fanconi’s syndrome
    • Tubulointerstitial disease
      • Myeloma
      • Interstitial nephritis
    • Drugs
      • Lead and other heavy metals
      • Acetazolamine
      • Out of date tetracycline
  • Diagnosis
    • Give an IV sodium bicarbonate load – high excretion in the urine
  • Treatment is with high doses of bicarbonate

 

Type III

  • Very rare – combination of Type I and Type II

Type IV

  • Due to hyporeninaemic hypoaldosteronism
  • Hypoaldosteronism causes hyperkalaemia and acidosis (reduced K+ and H+ excretion)
  • Causes;
    • Mild renal impairment
    • Causes of hypoaldosteronism
    • Drugs;
      • K+ sparing diuretics
      • NSAIDs
      • ACEI/ARB

 

Fanconi’s syndrome

  • May be genetic or acquired – heavy metal poisoning, drugs, renal diseases
  • Disturbance of the proximal tubule which does all the reabsorbing so there is defective reabsorption of;
    • Amino acids
    • Potassium
    • Phosphate (leading to hypophosphataemic rickets and osteomalacia)
    • Glucose (glucosuria)
    • Bicarbonate (Type II RTA)
 

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