Renal tubular acidosis: Difference between revisions
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== Types == |
== Types == |
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− | === Type I: |
+ | === Type I: Hypokalemic Distal === |
* Caused by defects in distal tubular H+ secretion |
* Caused by defects in distal tubular H+ secretion |
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* Etiology |
* Etiology |
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** Hereditary |
** Hereditary |
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− | ** Genetic: sickle cell; Fabry disease; Wilson disease; elliptocytosis |
+ | ** Genetic: [[Sickle cell disease|sickle cell]]; [[Fabry disease]]; [[Wilson disease]]; [[elliptocytosis]] |
− | ** Medullary cystic kidney disease |
+ | ** [[Medullary cystic kidney disease]] |
− | ** Autoimmune: Sjögren syndrome, systemic lupus erythematosus |
+ | ** Autoimmune: [[Sjögren syndrome]], [[systemic lupus erythematosus]] |
− | ** Nephrocalcinosis/hypercaliuria |
+ | ** [[Nephrocalcinosis]]/[[hypercaliuria]] |
− | ** Dysproteinemias: amyloidosis, cryoglobulinemia, hypergammaglobulinemia |
+ | ** Dysproteinemias: [[amyloidosis]], [[cryoglobulinemia]], [[hypergammaglobulinemia]] |
− | ** Drugs: amphotericin B, lithium, analgesic abuse |
+ | ** Drugs: [[amphotericin B]], [[Lithium toxicity|lithium]], analgesic abuse |
− | ** Tubulointerstitial disease: reflux nephropathy, |
+ | ** Tubulointerstitial disease: [[reflux nephropathy]], [[obstructive uropathy]], kidney transplant rejection |
− | === Type II: |
+ | === Type II: Proximal === |
* Caused by proximal tubular bicarbonate reabsorption |
* Caused by proximal tubular bicarbonate reabsorption |
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* Etiology |
* Etiology |
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** Primary (hereditary or sporadic) |
** Primary (hereditary or sporadic) |
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− | ** Hereditary carbonic anhydrase deficiency |
+ | ** [[Hereditary carbonic anhydrase deficiency]] |
− | ** Fanconi syndrome |
+ | ** [[Fanconi syndrome]] |
− | === Type IV: |
+ | === Type IV: Hyperkalemic Distal === |
* Caused by impaired ammoniagenesis, often caused by hypoaldosteronism |
* Caused by impaired ammoniagenesis, often caused by hypoaldosteronism |
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* Hyperkalemia, positive urine anion gap, urine pH <5.5 |
* Hyperkalemia, positive urine anion gap, urine pH <5.5 |
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* Etiology |
* Etiology |
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− | ** Primary aldosterone deficiency (Addison disease) |
+ | ** [[Primary aldosterone deficiency]] ([[Addison disease]]) |
** '''Hyporeninemic hypoaldosteronism''' |
** '''Hyporeninemic hypoaldosteronism''' |
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− | *** Acute glomerulonephritis |
+ | *** Acute [[glomerulonephritis]] |
*** Chronic nephropathy |
*** Chronic nephropathy |
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− | **** '''Diabetes''' (most common cause) |
+ | **** '''[[Diabetes mellitus|Diabetes]]''' (most common cause) |
− | **** Systemic lupus erythematosus |
+ | **** [[Systemic lupus erythematosus]] |
− | **** |
+ | **** [[HIV]] |
** Aldosterone resistance: |
** Aldosterone resistance: |
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− | *** Tubulointerstitial disease: urinary obstruction, sickle cell disease, medullary cystic kidney disease, and kidney transplant rejection |
+ | *** Tubulointerstitial disease: [[urinary obstruction]], [[sickle cell disease]], [[medullary cystic kidney disease]], and kidney transplant rejection |
− | ** Drugs: ACE inhibitors, cyclooxygenase inhibitors, trimethoprim, heparin |
+ | ** Drugs: [[ACE inhibitors]], cyclooxygenase inhibitors, [[trimethoprim]], [[heparin]] |
* Mixed proximal/distal |
* Mixed proximal/distal |
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** Carbonic anhydrase mutations |
** Carbonic anhydrase mutations |
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− | ** Drugs: topiramate |
+ | ** Drugs: [[topiramate]] |
== Risk Factors == |
== Risk Factors == |
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− | * Type IV: diabetes |
+ | * Type IV: [[Diabetes mellitus|diabetes]] |
== Investigations == |
== Investigations == |
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* Urine pH |
* Urine pH |
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+ | {| class="wikitable" |
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− | {| |
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! |
! |
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! Type 1 |
! Type 1 |
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|- |
|- |
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| Serum K |
| Serum K |
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− | | ↓ / |
+ | | ↓ / ↑† |
| ↓ |
| ↓ |
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| ↑ |
| ↑ |
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|- |
|- |
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− | | Serum HCO< |
+ | | Serum HCO<sub>3</sub> |
| Variable |
| Variable |
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| 10-20 |
| 10-20 |
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|} |
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− | * a hyperkalemic Type 1 RTA is possible with urinary obstruction, sickle cell, lupus, and renal transplant |
+ | * † a hyperkalemic Type 1 RTA is possible with [[urinary obstruction]], [[Sickle cell disease|sickle cell]], [[lupus]], and renal transplant |
[[Category:Nephrology]] |
[[Category:Nephrology]] |
Latest revision as of 10:51, 24 March 2024
Definition
- Group of diseases characterized by non-anion gap metabolic acidosis and preserved renal function
Types
Type I: Hypokalemic Distal
- Caused by defects in distal tubular H+ secretion
- Hypokalemia, positive urine anion gap, urine pH >5.5
- Etiology
- Hereditary
- Genetic: sickle cell; Fabry disease; Wilson disease; elliptocytosis
- Medullary cystic kidney disease
- Autoimmune: Sjögren syndrome, systemic lupus erythematosus
- Nephrocalcinosis/hypercaliuria
- Dysproteinemias: amyloidosis, cryoglobulinemia, hypergammaglobulinemia
- Drugs: amphotericin B, lithium, analgesic abuse
- Tubulointerstitial disease: reflux nephropathy, obstructive uropathy, kidney transplant rejection
Type II: Proximal
- Caused by proximal tubular bicarbonate reabsorption
- Hypokalemia, negative urine anion gap, urine pH variable
- Etiology
- Primary (hereditary or sporadic)
- Hereditary carbonic anhydrase deficiency
- Fanconi syndrome
Type IV: Hyperkalemic Distal
- Caused by impaired ammoniagenesis, often caused by hypoaldosteronism
- Hyperkalemia, positive urine anion gap, urine pH <5.5
- Etiology
- Primary aldosterone deficiency (Addison disease)
- Hyporeninemic hypoaldosteronism
- Acute glomerulonephritis
- Chronic nephropathy
- Diabetes (most common cause)
- Systemic lupus erythematosus
- HIV
- Aldosterone resistance:
- Tubulointerstitial disease: urinary obstruction, sickle cell disease, medullary cystic kidney disease, and kidney transplant rejection
- Drugs: ACE inhibitors, cyclooxygenase inhibitors, trimethoprim, heparin
- Mixed proximal/distal
- Carbonic anhydrase mutations
- Drugs: topiramate
Risk Factors
- Type IV: diabetes
Investigations
- Serum electrolytes
- Normal anion gap metabolic acidosis
- Hyperkalemia (type IV)
- Urine anion gap: UNa + UK - UCl
- If positive, suggests presence of ammonium
- If kidneys are responding properly, the UAG should be negative
- If defect in acid excretion, UAG should be positive
- Urine pH
Type 1 | Type 2 | Type 4 | |
---|---|---|---|
Acidosis | Severe | Moderate | Mild |
Serum K | ↓ / ↑† | ↓ | ↑ |
Serum HCO3 | Variable | 10-20 | >17 |
Urine pH | >5.3 | <5.3 | <5.3 |
Urine AG | + | ± | + |
- † a hyperkalemic Type 1 RTA is possible with urinary obstruction, sickle cell, lupus, and renal transplant