Diagnosis is based on a low serum ceruloplasmin level, high urinary excretion Type 4 results from aldosterone deficiency or unresponsiveness of the distal tubule to aldosterone.
There are This disorder is the most common type of RTA. It typically occurs sporadically secondary to impairment in the renin- aldosterone -renal tubule axis hyporeninemic hypoaldosteronism , which occurs in patients with the following:. Diabetic nephropathy Diabetic Nephropathy Diabetic nephropathy is glomerular sclerosis and fibrosis caused by the metabolic and hemodynamic changes of diabetes mellitus.
It manifests as slowly progressive albuminuria with worsening Chronic interstitial nephritis Tubulointerstitial Nephritis Tubulointerstitial nephritis is primary injury to renal tubules and interstitium resulting in decreased renal function. The acute form is most often due to allergic drug reactions or to infections Aldosterone synthase type I or II deficiency. Chronic kidney disease Chronic Kidney Disease Chronic kidney disease CKD is long-standing, progressive deterioration of renal function.
Congenital adrenal hyperplasia Overview of Congenital Adrenal Hyperplasia Congenital adrenal hyperplasia is a group of genetic disorders, each characterized by inadequate synthesis of cortisol, aldosterone, or both.
In the most common forms, accumulated hormone precursors HIV nephropathy HIV-Associated Nephropathy HIV-associated nephropathy is characterized by clinical findings similar to those of focal segmental glomerulosclerosis and often biopsy features of collapsing glomerulopathy a variant of focal Potassium-sparing diuretics eg, amiloride , eplerenone , spironolactone , triamterene. Obstructive uropathy Obstructive Uropathy Obstructive uropathy is structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction obstructive nephropathy.
Other drugs eg, pentamidine , trimethoprim. Pseudohypoaldosteronism Pseudohypoaldosteronism Type I Pseudohypoaldosteronism type I is a group of rare hereditary disorders that cause the kidneys to retain too much potassium but excrete too much sodium and water, leading to hypotension. Volume expansion eg, in acute glomerulonephritis or chronic kidney disease Chronic Kidney Disease Chronic kidney disease CKD is long-standing, progressive deterioration of renal function.
RTA is usually asymptomatic. Severe electrolyte disturbances are rare but can be life threatening. Nephrolithiasis Urinary Calculi Urinary calculi are solid particles in the urinary system.
People with type 1 or type 2 RTA may show symptoms and signs of hypokalemia Hypokalemia Hypokalemia is serum potassium concentration 3. Bony involvement eg, bone pain and osteomalacia in adults and rickets in children may occur in type 2 and sometimes in type 1 RTA. Type 4 RTA is usually asymptomatic with only mild acidosis, but cardiac arrhythmias Overview of Arrhythmias The normal heart beats in a regular, coordinated way because electrical impulses generated and spread by myocytes with unique electrical properties trigger a sequence of organized myocardial Suspected in patients with metabolic acidosis with normal anion gap or with unexplained hyperkalemia.
Type 4 RTA should be suspected in patients who have persistent hyperkalemia with no obvious cause, such as potassium supplements, potassium-sparing diuretics, or chronic kidney disease Chronic Kidney Disease Chronic kidney disease CKD is long-standing, progressive deterioration of renal function. ABG sampling is done to help confirm RTA and to exclude respiratory alkalosis as a cause of compensatory metabolic acidosis.
Serum electrolytes, BUN, creatinine, and urine pH are measured in all patients. Further tests and sometimes provocative tests are done, depending on which type of RTA is suspected:. Normal kidneys reduce urine pH to 5.
Type 2 RTA is diagnosed by measurement of the urine pH and fractional bicarbonate excretion during a bicarbonate infusion sodium bicarbonate 0. In type 2, urine pH rises above 7. Because IV bicarbonate can contribute to hypokalemia, potassium supplements should be given in adequate amounts before infusion. Type 4 RTA is confirmed by a history of a condition that could be associated with type 4 RTA, chronically elevated potassium, and normal or mildly decreased bicarbonate.
In most cases plasma renin activity is low, aldosterone concentration is low, and cortisol is normal. Treatment consists of correction of pH and electrolyte balance with alkali therapy. Failure to treat RTA in children slows growth. Potassium citrate can be substituted when persistent hypokalemia Hypokalemia Hypokalemia is serum potassium concentration 3.
It is usually hereditary Adults are given sodium bicarbonate or sodium citrate 0. Potassium supplementation is usually not required when the dehydration and secondary aldosteronism are corrected with bicarbonate therapy.
However, excess bicarbonate replacement increases potassium bicarbonate losses in the urine. Thus, citrate salts can be substituted for sodium bicarbonate and may be better tolerated. Potassium supplements or potassium citrate may be required in patients who become hypokalemic when given sodium bicarbonate but is not recommended in patients with normal or high serum potassium levels.
In difficult cases, treatment with low-dose hydrochlorothiazide 25 mg po bid may stimulate proximal tubule transport functions. In cases of generalized proximal tubule disorder, hypophosphatemia and bone disorders are treated with phosphate and vitamin D supplementation to normalize the plasma phosphate concentration.
Alkalinization is often unnecessary. A few patients need mineralocorticoid replacement therapy fludrocortisone 0. Renal tubular acidosis is a class of disorders in which excretion of hydrogen ions or reabsorption of filtered bicarbonate is impaired, leading to a chronic metabolic acidosis with a normal anion gap. RTA is usually due to abnormal aldosterone production or response type 4 , or less often, due to impaired hydrogen ion excretion type 1 or impaired bicarbonate resorption type 2.
Consider RTA if patients have metabolic acidosis with a normal anion gap or unexplained hyperkalemia. Do other testing to confirm type of RTA eg, acid load test for type 1, bicarbonate infusion for type 2. Treat using alkali therapy and measures to correct low serum potassium in type 2 and sometimes type 1 RTA , and using potassium restriction or potassium-wasting diuretics in type 4 RTA; give other electrolytes as needed.
From developing new therapies that treat and prevent disease to helping people in need, we are committed to improving health and well-being around the world. The Manual was first published in as a service to the community. Learn more about our commitment to Global Medical Knowledge. This site complies with the HONcode standard for trustworthy health information: verify here. Common Health Topics. Videos Figures Images Quizzes Symptoms. Type 1 distal RTA.
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