Renal Tubular Acidosis (Distal Renal Tubular Acidosis)

A group of genetic disorders of the KIDNEY TUBULES characterized by the accumulation of metabolically produced acids with elevated plasma chloride, hyperchloremic metabolic ACIDOSIS. Defective renal acidification of URINE (proximal tubules) or low renal acid excretion (distal tubules) can lead to complications such as HYPOKALEMIA, hypercalcinuria with NEPHROLITHIASIS and NEPHROCALCINOSIS, and RICKETS.

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Networked: 826 relevant articles (17 outcomes, 42 trials/studies)

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Disease Context: Research Results

Related Diseases

1.	Fanconi Syndrome (Syndrome, Fanconi)
2.	Glycosuria
3.	Osteomalacia
4.	Hypophosphatemia
5.	Bone Diseases (Bone Disease)

Experts

1.	Wagner, Carsten A: 14 articles (04/2015 - 03/2002)
2.	Cordat, Emmanuelle: 11 articles (08/2014 - 09/2004)
3.	Yenchitsomanus, Pa-thai: 10 articles (07/2014 - 07/2002)
4.	Reithmeier, Reinhart A F: 10 articles (11/2013 - 09/2004)
5.	Sawasdee, Nunghathai: 9 articles (08/2015 - 07/2002)
6.	Yenchitsomanus, Pa-Thai: 9 articles (08/2015 - 04/2004)
7.	Schwartz, George J: 8 articles (07/2013 - 06/2003)
8.	Alper, Seth L: 7 articles (04/2011 - 01/2002)
9.	Toye, Ashley M: 7 articles (07/2008 - 01/2002)
10.	Akkarapatumwong, Varaporn: 6 articles (08/2015 - 04/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Renal Tubular Acidosis:

1.	AlkaliesIBA 01/01/1986 - "Type 4 renal tubular acidosis was ruled out by the inability to achieve a minimum urine pH. With a mean follow-up period of 28.1 +/- 25.3 months, after alkali therapy at 3.3-3.5 mEq/kg/day had been administered for at least 12 months, the growth parameters improved as follows: the percentile weight (mean +/- SD) increased from the initial 11.8 +/- 7.5 to the final 27.6 +/- 31.3 (p less than 0.003), and the length/height percentile increased from 11.5 +/- 7.3 to 29.7 +/- 24.2 (p less than 0.03). " 08/01/1976 - "Improvement in lacrimal and salivary secretions after alkali therapy in Sjøgren's syndrome with renal tubular acidosis." 02/01/1981 - "Studies done during acid and alkali loading suggested that the defect in urinary acidification resided in the proximal tubule and thus represented a Type II or proximal renal tubular acidosis (RTA)." 08/01/2002 - "Alkali therapy in renal tubular acidosis: who needs it?" 02/01/1988 - "Alkali therapy is used commonly to prevent recurrent stone formation in patients with distal renal tubular acidosis. "
2.	Furosemide (Lasix)FDA LinkGeneric 07/15/2015 - "The inability to acidify urine in response to furosemide serves to diagnose distal renal tubular acidosis (dysfunction of α-intercalated cells). " 01/01/2008 - "(6) This patient failed to acidify urine below a pH of 5.3 after taking simultaneously furosemide (40 mg) and fludrocortrisone (1 mg), which suggested incomplete renal tubular acidosis type 1.We learn from this case that biochemical analysis of stones removed from urinary bladder may be useful. " 03/01/1988 - "A 36-year-old woman with previously undiagnosed distal renal tubular acidosis presented with foot and ankle swelling that was being treated with furosemide. " 11/01/1985 - "Furosemide increases urinary acidification in control subjects and in certain patients with normokalemic or hypokalemic distal renal tubular acidosis (RTA). " 11/01/1985 - "Hyperkalemic renal tubular acidosis: effect of furosemide in humans and in rats."
3.	Uric Acid (Urate)IBA 10/01/2011 - "We included non-stone forming subjects and stone formers with hypercalciuria, distal renal tubular acidosis, idiopathic uric acid nephrolithiasis, or chronic diarrhea. " 07/01/2003 - "Chronic diarrheal syndromes and uric acid stones were associated with one another, and brushite stones were associated with renal tubular acidosis. " 01/01/1979 - "In patients with urate stones and those with renal tubular acidosis a significant decrease in uromucoid excretion was found. " 04/01/2006 - "The main focus is on the two major conditions related to acid-base metabolism that cause kidney stone formation, i.e., distal renal tubular acidosis (dRTA) and abnormally low urine pH with subsequent uric acid stone formation. " 01/01/1977 - "A patient with asymptomatic adult Fanconi syndrome with glycosuria, amino-aciduria, hypophosphatemia, and renal tubular acidosis was found to have hypouricemia (serum uric acid, 1.5-1.8 mg/100 ml) secondary to increased renal clearance of urate (urate clearance/glomerular filtration rate, 32 per cent). "
4.	PotassiumIBA 09/01/2005 - "Both cases improved in both somatic growth and mental development after commencing treatment for renal tubular acidosis in the form of alkaline therapy and potassium supplementation." 11/01/2006 - "Distal renal tubular acidosis and the potassium enigma." 09/01/2006 - "Different types of this disorder termed renal tubular acidosis (RTA) can be distinguished based on the localization of the disturbance along the nephron (proximal vs. distal) and their association with potassium transport (hypo-/hyperkalemic). " 03/01/2001 - "That distal renal tubular acidosis can be associated with low, normal, or high serum potassium concentration is now explainable by identifying the specific defect in transport causing each syndrome." 11/01/2000 - "That renal tubular acidosis can be associated with low, normal, or high serum potassium concentration is now explainable by identifying the specific defect in transport causing each syndrome."
5.	FludrocortisoneIBA 07/01/2013 - "A diagnosis of late onset type 4 renal tubular acidosis was made and he responded well to a course of fludrocortisone. " 11/01/2007 - "Diagnosis of distal renal tubular acidosis: use of furosemide plus fludrocortisone versus ammonium chloride." 05/01/2015 - "Our objectives were to analyse the prevalence of distal renal tubular acidosis (dRTA) in primary SS (pSS) and to compare a novel urinary acidification test with furosemide and fludrocortisone (FF) with the gold standard ammonium chloride (NH4Cl) to detect dRTA. " 01/01/2014 - "This indirect coupling of sodium reabsorption and proton secretion is thought to underlie the fludrocortisone-furosemide test for maximal urinary acidification in patients with suspected distal renal tubular acidosis. "
6.	dirhodium tetraacetate (DRTA)IBA 01/01/2011 - "To investigate clinically and genetically all the distal renal tubular acidosis (dRTA) cases in Cyprus, to study one more family from Greece and to perform the first dRTA prenatal diagnosis. " 08/01/2009 - "In a study group of 40 surgically treated patients with posterior urethral valve (PUV) and normal estimated glomerular filtration rate, we evaluated the change in height standard deviation scores (SDSs) while they were on bicarbonate therapy in the presence of idRTA and complete distal renal tubular acidosis (dRTA). " 10/01/2008 - "This study investigated two naturally occurring point mutations in a4's COOH terminus that cause recessive distal renal tubular acidosis (dRTA), R807Q and G820R. " 06/01/2005 - "We suggest that all children with distal renal tubular acidosis (dRTA) should be subjected to an equilibrium study and audiological evaluation, as well as to a CT or MRI scan. " 11/01/1978 - "Renal biospy studies are reported from 10 patients with distal renal tubular acidosis (DRTA). "
7.	CalciumIBA 07/01/1993 - "Laboratory studies revealed distal renal tubular acidosis and hypocitraturia, but no significant abnormality in calcium metabolism. " 04/01/2007 - "To survey the urinary risk factors associated with recurrent calcium stone and the contribution of renal tubular acidosis to the prevalence of recurrent calcium stone formation in Thai recurrent stone formers. " 04/01/1994 - "We speculate that calcium precipitation may lead to tubular damage, including distal renal tubular acidosis." 12/01/1984 - "Distal renal tubular acidosis has been reported as an uncommon cause of urinary calcium stone disease. " 09/01/1958 - "Effect of renal tubular acidosis on calcium excretion."
8.	MagnesiumIBA 10/01/1972 - "Decreased bicarbonate threshold and renal magnesium wasting in a sibship with distal renal tubular acidosis. " 01/01/1986 - "Magnesium-responsive incomplete distal renal tubular acidosis in patients with liver cirrhosis." 12/01/2004 - "The objective of this study was to examine renal magnesium handling (U(Mg)) in children with primary distal renal tubular acidosis (DRTA). " 07/01/2004 - "Nephrotoxicity includes decreased glomerular filtration rate and distal tubulopathy with urinary loss of potassium and magnesium, renal tubular acidosis, loss of urine concentrating ability, and sometimes Fanconi's syndrome. " 01/01/1985 - "The lowest CIT excretion was measured in incomplete renal tubular acidosis with magnesium wasting. "
9.	Quaternary Ammonium Compounds (Ammonium)IBA 01/01/1992 - "Should the urine PCO2 or the rate of excretion of ammonium be the gold standard to diagnose distal renal tubular acidosis?" 11/20/2008 - "Mice lacking Rhcg have abnormal urinary acidification due to impaired ammonium excretion on acid loading-a feature of distal renal tubular acidosis. " 10/01/1993 - "Impaired urinary ammonium excretion in patients with isolated proximal renal tubular acidosis." 03/01/1991 - "Renal tubular acidosis (RTA): recognize the ammonium defect and pHorget the urine pH." 01/01/1988 - "Urine ammonium: the key to the diagnosis of distal renal tubular acidosis."
10.	maleic acid (maleate)IBA 03/01/1986 - "Microperfusion study of proximal tubule bicarbonate transport in maleic acid-induced renal tubular acidosis." 12/01/1982 - "Dysfunction of the proximal tubule underlies maleic acid-induced type II renal tubular acidosis." 03/01/1986 - "The observations indicate that proximal renal tubular acidosis (RTA) induced by maleic acid is characterized by impaired lumen-to-blood transport of sodium bicarbonate and chloride but not by increased backflux. " 04/01/1985 - "The metabolic pathogenesis of the complex renal tubular dysfunction of type II renal tubular acidosis and Fanconi's syndrome (RTA II/FS) acutely induced by maleic acid could depend on the occurrence of a positive feedback loop in cells of the proximal renal tubule: impaired mitochondrial oxidation----increased glucose uptake----increased formation and concentration of phosphorylated glycolytic intermediates----limitation on availability of cellular inorganic phosphate----more severely impaired mitochondrial oxidative metabolism. " 12/01/1982 - "To investigate whether dysfunction of the proximal tubule underlies maleic acid-(MA) induced type II ("proximal") renal tubular acidosis (RTA II), we intravenously administered either MA or acetazolamide to eight conscious trained dogs undergoing water diuresis and examined the relationship between fractional solute-free water clearance (Ch2o/GFR), a measure of NaCl reabsorption in the post-proximal nephron, and either fractional urine flow (V/GFR), a measure of total solute rejected by the proximal tubule, or the sum of fractional excretion of Cl- and Ch2o/GFR [(Ccl + Ch2o)/GFR], a measure of proximally rejected solute that is potentially reabsorbable by the thick ascending limb. "

Therapies and Procedures

1.	Kidney Transplantation 05/01/1974 - "This testing system has been found to be very useful in investigating patients with analgesic nephropathy, renal tubular acidosis, and after renal transplantation." 01/01/2011 - "Prevalence and risk factors of renal tubular acidosis after kidney transplantation." 07/01/2010 - "We aimed to investigate the prevalence, type, and possible risk factors of renal tubular acidosis (RTA) in Turkish patients with renal transplantation. " 07/01/2010 - "Renal tubular acidosis in renal transplantation recipients." 03/01/2007 - "Renal tubular acidosis after kidney transplantation--incidence, risk factors and clinical implications."
2.	Hormone Replacement Therapy (Therapy, Hormone Replacement) 09/01/1982 - "Renal tubular acidosis in a patient with hypothyroidism due to autoimmune thyroiditis - improvement with hormone replacement therapy."
3.	Nephrectomy 08/10/1996 - "[Case of refractory renal tubular acidosis complicated by idiopathic thrombocytopenic purpura: efficacy of embolization of the right kidney and left nephrectomy]."
4.	Cerebrospinal Fluid Shunts 07/01/2001 - "Furosemide was found to be effective in management of bronchoconstriction accompanying chronic lung disease and narrowing of the upper respiratory airways; in hydrocephalus in infancy to avoid cerebrospinal fluid shunts; in some diagnostic procedures, such as an assessment of fetal and neonatal hydronephrosis; and in evaluation of different types of renal tubular acidosis. "
5.	Jejunoileal Bypass 01/01/1984 - "Renal tubular acidosis following intestinal bypass: an etiological study." 01/01/1984 - "Nine cases of the distal type of renal tubular acidosis (RTA) following intestinal bypass were found. " 09/01/1985 - "Renal tubular acidosis secondary to jejunoileal bypass for morbid obesity." 03/01/2008 - "By using intraoperative papillary biopsy material from kidneys of idiopathic calcium oxalate, intestinal bypass for obesity, brushite, cystine, and distal renal tubular acidosis stone formers during percutaneous nephrolithotomy, we have determined that idiopathic calcium oxalate stone formers appear to be the special case, although the most commonly encountered one, in which stones form external to the kidney and by processes that do not involve the epithelial compartments. "