Proximal renal tubular acidosis: a not so rare disorder of multiple etiologies. Nephrol Dial Transplant. Unwin RJ, Capasso G. The renal tubular acidoses. J R Soc Med. Mutations in SLC4A4 cause permanent isolated proximal renal tubular acidosis with ocular abnormalities. Unraveling the molecular pathogenesis of isolated proximal renal tubular acidosis. Am J Case Rep. Acid and mineral balances and bone in familial proximal renal tubular acidosis. A case of severe osteomalacia caused by tubulointerstitial nephritis with Fanconi syndrome in asymptomotic primary biliary cirrhosis.
BMC Nephrol. Incidence of radiographically evident bone disease, nephrocalcinosis, and nephrolithiasis in various types of renal tubular acidosis. N Engl J Med. Pathologic femoral neck fracture due to Fanconi syndrome induced by adefovir dipivoxil therapy for hepatitis B. Clin Orthop Surg. Ifosfamide-induced renal Fanconi syndrome with associated nephrogenic diabetes insipidus in an adult patient. Sodium valproate-induced Fanconi type proximal renal tubular acidosis.
Topiramate-induced renal tubular acidosis. Am J Med. Topiramate induces type 3 renal tubular acidosis by inhibiting renal carbonic anhydrase. Proximal renal tubular acidosis associated with glycogen storage disease, type 9.
Acta Paediatr Scand. Pathogenesis of acidosis in hereditary fructose intolerance. Fanconi syndrome due to light chain proximal tubulopathy in a patient with multiple myeloma.
Karet FE. Mechanisms in hyperkalemic renal tubular acidosis. Electrolyte and acid-base disturbances in patients with diabetes mellitus. Batlle D, Arruda J. Hyperkalemic forms of renal tubular acidosis: clinical and pathophysiological aspects.
Hyperkalemic distal renal tubular acidosis associated with obstructive uropathy. Mount DB. Thick ascending limb of the loop of Henle. Renal tubular acidosis type IV as a complication of lupus nephritis. Kidney protein dynamics and ammoniagenesis in humans with chronic metabolic acidosis. Prevalence of metabolic acidosis among patients with CKD and hyperkalemia [abstract 89]. Human hypertension caused by mutations in WNK kinases.
Update on cyclooxygenase-2 inhibitors. Clinical analysis of hyperkalemic renal tubular acidosis caused by calcineurin inhibitors in solid organ transplant recipients.
J Clin Pharm Ther. Hyperkalemic distal renal tubular acidosis caused by immunosuppressant treatment with tacrolimus in a liver transplant patient: case report. Transplant Proc. Tacrolimus-induced type IV renal tubular acidosis following liver transplantation. Case Rep Hepatol. Aldosterone resistance in kidney transplantation is in part induced by a down-regulation of mineralocorticoid receptor expression.
Clin Transplant. Clinical and laboratory approaches in the diagnosis of renal tubular acidosis. Combination of furosemide and fludrocortisone as a loading test for diagnosis of distal renal tubular acidosis in a pediatric case. CEN Case Rep. Urinary acidification assessed by simultaneous furosemide and fludrocortisone treatment: an alternative to ammonium chloride. Pathogenesis of distal renal tubular acidosis.
Gut it out: laxative abuse mimicking distal renal tubular acidosis. Kidney Blood Press Res. Plant-based diets to manage the risks and complications of chronic kidney disease. Goraya N, Wesson DE. Management of the metabolic acidosis of chronic kidney disease. Effects of treatment of metabolic acidosis in CKD: a systematic review and meta-analysis. Raphael KL. Metabolic acidosis in CKD: core curriculum Hypocitraturia: pathophysiology and medical management.
Rev Urol. The effect of fruits and vegetables on urinary stone risk factors. Fanconi syndrome in the adulthood. The role of early diagnosis and treatment. J Musculoskelet Neuronal Interact. On the mechanism of renal potassium wasting in renal tubular acidosis associated with the Fanconi syndrome type 2 RTA.
Renal tubular acidosis in infants and children. Clinical course, response to treatment, and prognosis. J Pediatr. Managing hyperkalemia caused by inhibitors of the renin-angiotensin-aldosterone system. Fludrocortisone therapy for persistent hyperkalaemia. Diabet Med. Kidney Disease Improving Global Outcomes. KDIGO clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int Suppl. Eur Heart J. PLoS One. Patiromer in patients with kidney disease and hyperkalemia receiving RAAS inhibitors.
Sodium zirconium cyclosilicate increases serum bicarbonate concentrations among patients with hyperkalaemia: exploratory analyses from three randomized, multi-dose, placebo-controlled trials. Long-term safety and efficacy of veverimer in patients with metabolic acidosis in chronic kidney disease: a multicentre, randomised, blinded, placebo-controlled, week extension. Veverimer versus placebo in patients with metabolic acidosis associated with chronic kidney disease: a multicentre, randomised, double-blind, controlled, phase 3 trial.
Download references. All named authors meet the International Committee of Medical Journal Editors ICMJE criteria for authorship for this manuscript and take responsibility for the integrity of the work as a whole. Biff F. Palmer and Deborah J. Clegg have nothing to disclose. The authors have not received any funding for the current work. Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study. You can also search for this author in PubMed Google Scholar.
Correspondence to Biff F. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. Reprints and Permissions. Palmer, B. Adv Ther 38, — Download citation.
Received : 03 November Accepted : 26 November Published : 26 December Issue Date : February Anyone you share the following link with will be able to read this content:. Sorry, a shareable link is not currently available for this article. Provided by the Springer Nature SharedIt content-sharing initiative. Skip to main content. Search SpringerLink Search. Download PDF. Normal acid—base homeostasis is maintained by the kidneys and respiratory system Renal tubular acidosis RTA occurs when the kidneys are unable to adequately reclaim filtered bicarbonate or excrete sufficient hydrogen ions because of defects in tubular transport Three main types of RTA are common in clinical practice: distal type 1 , proximal type 2 , and hyperkalemic type 4 RTA Differential diagnosis of RTA should consider presenting signs and symptoms, the anion gap, serum biochemistry, and urine pH Treatment options depend on the type of RTA diagnosed; alkali therapy may be used in patients with type 1 or 2 RTA, and newer potassium binders may be beneficial for patients with type 4 RTA.
Digital Features This article is published with digital features, including a summary slide, to facilitate understanding of the article. Introduction The maintenance of acid—base homeostasis is a major function of the body, accomplished by intracellular and extracellular buffering mechanisms, exhalation of carbon dioxide CO 2 through the respiratory system, and acid excretion by the kidneys [ 1 ].
Full size image. Table 1 Summary of renal tubular acidosis classification, diagnostic characteristics, and treatment options Full size table. 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.
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