Distal Renal Tubular Acidosis

11/14/2005

Question:  What are the causes of distal (type 1) renal tubular acidosis?

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<1>

Unique Identifier [PMID]: 15778079

Authors: Laing CM. Toye AM. Capasso G. Unwin RJ.

Institution: Centre for Nephrology, Royal Free and University College Medical School, London NW3, UK.

Title: Renal tubular acidosis: developments in our understanding of the molecular basis. [Review] [50 refs]

Source: International Journal of Biochemistry & Cell Biology. 37(6):1151-61, 2005 Jun.

Abstract: Renal tubular acidosis is a metabolic acidosis due to impaired acid excretion by the kidney. Hyperchloraemic acidosis with a normal anion gap and normal (or near normal) glomerular filtration rate, and in the absence of diarrhoea, defines this disorder. However, systemic acidosis is not always evident and renal tubular acidosis can present with hypokalaemia, medullary nephrocalcinosis and recurrent calcium phosphate stone disease, as well as growth retardation and rickets in children, or short stature and osteomalacia in adults. Renal dysfunction in renal tubular acidosis is not always confined to acid excretion and can be part of a more generalised renal tubule defect, as in the renal Fanconi syndrome. Isolated renal tubular acidosis is more usually acquired, due to drugs, autoimmune disease, post-obstructive uropathy or any cause of medullary nephrocalcinosis. Less commonly, it is inherited and may be associated with deafness, osteopetrosis or ocular abnormalities. The clinical classification of renal tubular acidosis has been correlated with our current physiological model of how the nephron excretes acid, and this has facilitated genetic studies that have identified mutations in several genes encoding acid and base ion transporters. In vitro functional studies of these mutant proteins in cell expression systems have helped to elucidate the molecular mechanisms underlying renal tubular acidosis, which ultimately may lead to new therapeutic options in what is still treatment only by giving an oral alkali. [References: 50]

Publication Type: Journal Article. Review.

<2>

Unique Identifier [PMID]: 12027213

Authors: Unwin RJ. Shirley DG. Capasso G.

Institution: Centre for Nephrology, Department of Physiology, Royal Free and University College Medical School, London, UK. robert.unwin@ucl.ac.uk

Title: Urinary acidification and distal renal tubular acidosis. [Review] [52 refs]

Source: Journal of Nephrology. 15 Suppl 5:S142-50, 2002 Mar-Apr.

Abstract: Historically, renal tubular acidosis (RTA) has been classified on a clinical basis, without any reference to the underlying disorder. Here we review the normal mechanisms of renal acidification and we identify disorders of specific transporters (genetic, disease-related or drug-induced) that lead to the main categories of distal RTA. We also describe the approach to diagnosis and the current treatment of distal RTA. [References: 52]

Publication Type: Journal Article. Review.

<3>

Unique Identifier [PMID]: 11245773

Authors: Sabatini S. Kurtzman NA.

Institution: Department of Physiology, The Combined Program in Nephrology and Renal Physiology, Texas Tech University Health Sciences Center, Lubbock, TX, USA.

Title: Biochemical and genetic advances in distal renal tubular acidosis. [Review] [42 refs]

Source: Seminars in Nephrology. 21(2):94-106, 2001 Mar.

Abstract: Distal renal tubular acidosis is a constellation of syndromes arising from different derangements of tubular acid transport. Recent advances in the biology of urinary acidification have allowed us to discern various molecular mechanisms responsible for these syndromes. This article relates clinical disorders of distal acidification to the underlying defective mechanisms responsible for them. A clinical classification of these disorders is presented which integrates each disorder with the prevailing serum potassium concentration. 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. Copyright 2001 by W.B. Saunders Company. [References: 42]

Publication Type: Journal Article. Review.

<4>

Unique Identifier [PMID]: 9387126

Authors: Capasso G. Saviano C. Rizzo M. Pica A. Capodicasa D. Mascolo N. De Santo NG.

Institution: Chair of Nephrology, Second University of Naples, Italy. gcapasso@cds.unina.it

Title: Update on renal acidification: a physiological view. [Review] [46 refs]

Source: Mineral & Electrolyte Metabolism. 23(3-6):243-8, 1997.

Abstract: The kidney plays a major role in the regulation of acid-base balance. This process is mainly dependent on H+ secretion in the tubular lumen. Two acid extruder proteins are involved: the Na+/H+ exchanger and H(+)-ATPase. Studies using in vivo and in vitro microperfusion and isolated membrane vesicles have clearly demonstrated that the Na+/H+ exchanger is the main mechanism regulating H+ secretion/HCO3- reabsorption along the proximal nephron. Moreover, several reports indicate that this protein is involved in intracellular pH (pHi) regulation. Newer studies using molecular biology techniques have identified at least five isoforms of the Na+/H+ exchanger: NHE-1 is the housekeeping isoform, while NHE-3 seems to be implicated in transepithelial acid-base transport, although other isoforms could be involved too. H(+)-ATPase is the major acid extruder protein along the distal nephron, but it is also expressed along the proximal tubule, where a Na(+)-independent bicarbonate reabsorption has been described. There are a few studies indicating that the proton pump participates in pHi regulation, particularly in the presence of a large acid load. Its absence along the distal nephron may be one of the causes of distal tubular acidosis. [References: 46]

Publication Type: Journal Article. Review.

<5>

Unique Identifier [PMID]: 8933509

Authors: Bastani B. Gluck SL.

Institution: Division of Nephrology, St. Louis University Health Sciences Center, MO 63110, USA.

Title: New insights into the pathogenesis of distal renal tubular acidosis. [Review] [133 refs]

Source: Mineral & Electrolyte Metabolism. 22(5-6):396-409, 1996.

Abstract: The diagnosis and classification of distal renal tubular acidosis (distal RTA) have traditionally been made on the basis of the renal response to physiologic maneuvers, providing only indirect information on the underlying pathophysiology. In the past several years significant advances have been made in our understanding of the molecular basis of distal H+/HCO3- secretion and absorption, at the level of individual transporters. With these advances, a new era of classifying disorders of distal acidification at the molecular level has arrived. In this article we review the cellular and molecular basis of normal acidification mechanisms in the distal nephron. We also review the recent information on the molecular basis of derangements in these mechanisms which lead to distal RTA. [References: 133]

Publication Type: Journal Article. Review.

<6>

Unique Identifier [PMID]: 8694660

Authors: Smulders YM. Frissen PH. Slaats EH. Silberbusch J.

Institution: Department of Internal Medicine, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands.

Title: Renal tubular acidosis. Pathophysiology and diagnosis. [Review] [63 refs]

Source: Archives of Internal Medicine. 156(15):1629-36, 1996 Aug 12-26.

Abstract: The pathophysiology of renal tubular acidosis is slowly being unraveled, which has implications for the traditional classification of the condition. Nonetheless, the diagnosis of renal tubular acidosis is still easy to establish, and identification of the specific pathophysiological subtype is relatively straightforward. The diagnostic information required usually includes only urinary pH and sodium, potassium, and chloride concentrations and serum potassium level. The urinary pH is not a diagnostic test for renal tubular acidosis, but it serves to distinguish between the various subtypes. [References: 63]

Publication Type: Case Reports. Journal Article. Review.

<7>

Unique Identifier [PMID]: 2015240

Authors: Fortenberry JD. Kenney RD.

Institution: Department of Pediatrics, Texas Children's Hospital, Houston.

Title: Distal renal tubular acidosis as the initial manifestation of systemic lupus erythematosus in an adolescent.

Source: Journal of Adolescent Health. 12(2):148-51, 1991 Mar.

Abstract: Distal renal tubular acidosis (dRTA) as the initial manifestation of systemic lupus erythematosus (SLE) has not been described previously. We report a 14-year-old female adolescent, first presenting with findings consistent with dRTA, whose renal biopsy demonstrated immune mesangial deposits consistent with lupus nephritis. Seven months later, she developed classic extrarenal manifestations of SLE. We recommend that adolescents with dRTA be evaluated and followed closely for the possibility of SLE.

Publication Type: Case Reports. Journal Article.

<8>

Unique Identifier [PMID]: 2670024

Authors: Maher ER. Scoble JE.

Institution: University of Cambridge, London.

Title: Renal tubular acidosis. [Review] [17 refs]

Source: British Journal of Hospital Medicine. 42(2):116-9, 1989 Aug.

Abstract: Disordered renal acid-base handling can be associated with a wide variety of diseases. Although early diagnosis is important to avoid complications, renal tubular acidosis is frequently not recognized. The pathophysiology, clinical features, diagnosis and treatment of this group of disorders are described in the light of recent advances in our knowledge of renal tubular transport mechanisms. [References: 17]

Publication Type: Journal Article. Review.

<9>

Unique Identifier [PMID]: 3173596

Authors: Halperin ML. Richardson RM. Bear RA. Magner PO. Kamel K. Ethier J.

Institution: Renal Division, St. Michael's Hospital, Toronto, Ont., Canada.

Title: Urine ammonium: the key to the diagnosis of distal renal tubular acidosis.[see comment].

Comments Comment in: Nephron. 1990;54(2):180-1; discussion 182; PMID: 2314529

Source: Nephron. 50(1):1-4, 1988.

Publication Type: Journal Article.

<10>

Unique Identifier [PMID]: 3127650

Authors: Caruana RJ. Buckalew VM Jr.

Institution: Nephrology Section, Medical College of Georgia, Augusta 30912.

Title: The syndrome of distal (type 1) renal tubular acidosis. Clinical and laboratory findings in 58 cases.

Source: Medicine. 67(2):84-99, 1988 Mar.

Abstract: The clinical and laboratory findings in 14 infants, 2 children and 42 adults with RTA-1 have been retrospectively analyzed and the patients classified as having the hereditary (14%), acquired (31%), or idiopathic (55%) form. In 7 of the 8 hereditary cases, RTA-1 appeared to be a complication of hereditary hypercalciuria. The majority of acquired cases (61%) were secondary to immune-mediated diseases. All of the 14 infants with RTA-1 were classified as idiopathic. All of the idiopathic cases in children and adults were associated with nephrolithiasis and/or nephrocalcinosis, 33% of which had a family history of nephrolithiasis. The 14 infants presented with failure to thrive. Seventy-seven percent of children and adults with RTA-1 had nephrolithiasis and/or nephrocalcinosis and usually presented with symptoms related to this problem. Adults without nephrolithiasis or nephrocalcinosis usually presented with electrolyte disturbances or acidosis. Hypokalemia, the most common electrolyte disturbance, was present in 28% of the entire series. Acidosis was present in all infants and in 70% of children and adults. Clinically apparent bone disease was observed in 3 infants, and in 1 adult with nephrolithiasis. Glomerular function was normal in infants and in the 2 children, but depressed in 40% of adults. Recurrent urinary tract infection was a contributing factor but was not the sole cause of renal failure. Surprisingly, kidney stone number, the number of surgical procedures, and the presence of nephrocalcinosis had no apparent effect on the development of renal failure. Glomerular filtration rate was significantly higher in patients with incomplete RTA-1, and serum total CO2 was significantly correlated with creatinine clearance and minimum urinary pH. Hypercalciuria was present in 32% of patients with nephrolithiasis and/or nephrocalcinosis, and urinary citrate excretion was low in all of 16 patients in whom it was measured. Hypocitraturia appeared to be due in most cases to potassium depletion and renal failure, but may have occurred as a primary defect in 1 patient with hereditary RTA. Urinary uric acid excretion was elevated in 23% of patients with stones in whom it was measured. The mean number of stone-forming events was 51 +/- 14. Although a weak correlation between urinary calcium excretion and stone number was observed, the cause for prodigious stone formation could not be explained. This series emphasizes the variable degree to which the common clinical manifestations of RTA-1 (metabolic acidosis, hypercalciuria, nephrolithiasis, nephrocalcinosis, and potassium depletion) are expressed.(ABSTRACT TRUNCATED AT 400 WORDS)

Publication Type: Journal Article.

<11>

Unique Identifier [PMID]: 3518609

Authors: Rocher LL. Tannen RL.

Title: The clinical spectrum of renal tubular acidosis. [Review] [54 refs]

Source: Annual Review of Medicine. 37:319-31, 1986.

Abstract: Renal tubular acidosis (RTA) is associated with a large group of disorders that interfere with normal tubular hydrogen-ion handling by any of several mechanisms. This review presents an approach to the pathophysiology, diagnosis, and therapy of RTA by defining three major subgroups: proximal RTA, distal RTA presenting with normal or decreased serum potassium (hypokalemic distal RTA), and distal RTA presenting with increased serum potassium (hyperkalemic distal RTA). [References: 54]

Publication Type: Journal Article. Review.

<12>

Unique Identifier [PMID]: 3972559

Authors: Pines A. Kaplinsky N. Olchovsky D. Frankl O. Goldfarb D. Iaina A.

Title: Anorexia nervosa, laxative abuse, hypopotassemia and distal renal tubular acidosis.

Source: Israel Journal of Medical Sciences. 21(1):50-2, 1985 Jan.

Abstract: A case of anorexia nervosa in a 28-year-old woman with laxative abuse, hypopotassemia and severe metabolic acidosis, is described. The diagnosis of classical renal tubular acidosis, Type I, was confirmed by our inability to decrease urinary pH beyond 5.5 and to increase ammonia excretion during an ammonium chloride loading test. A bicarbonate loading test and normal plasma aldosterone with high renin activity excluded proximal renal tubular acidosis, hyporeninemic-hypoaldosteronemic renal tubular acidosis and Bartter's syndrome. The inability to increase ammonium excretion during severe metabolic acidosis following ammonium chloride loading did not favor the possibility of a transient physiological adaptation of ammoniagenesis at the tubular cell level, related to potassium depletion. Although mental disorder, laxative abuse, abstinence from food intake and severe potassium depletion intermingled in a vicious cycle, we assume that one of the following possibilities may explain the clinical presentation in our patient: either two separated and unrelated disorders, or laxative abuse as the cause of renal tubular acidification impairment.

Publication Type: Case Reports. Journal Article.

<13>

Unique Identifier [PMID]: 6463004

Authors: Spruce BA. Baylis PH. Kerr DN. Morley AR.

Title: Idiopathic hypergammaglobulinaemia associated with nephrogenic diabetes insipidus and distal renal tubular acidosis.

Source: Postgraduate Medical Journal. 60(705):493-4, 1984 Jul.

Abstract: Renal tubular dysfunction may be recognized in patients suffering from urinary light chain disease or non-myelomatous hypergammaglobulinaemia. We report a patient who has the combination of distal renal tubular acidosis and nephrogenic diabetes insipidus in association with hypergammaglobulinaemia due solely to increased IgG. We postulate that the abnormalities of distal nephron function resulted from cell-mediated immune damage.

Publication Type: Case Reports. Journal Article.

<14>

Unique Identifier [PMID]: 6674675

Authors: Kurtzman NA.

Title: Acquired distal renal tubular acidosis.

Source: Kidney International. 24(6):807-19, 1983 Dec.

Publication Type: Case Reports. Journal Article.

<15>

Unique Identifier [PMID]: 6876939

Authors: Batlle D.

Title: Renal tubular acidosis.

Source: Medical Clinics of North America. 67(4):859-78, 1983 Jul.

Publication Type: Journal Article.

<16>

Unique Identifier [PMID]: 6873504

Authors: Caregaro L. Lauro S. Ricci G. Gatta A. Zuin R. Ruol A.

Title: Pathogenetic relationships between renal tubular acidosis and sodium metabolism alterations in liver cirrhosis.

Source: Digestion. 26(4):179-86, 1983.

Abstract: 5 cirrhotic patients with latent distal renal tubular acidosis (RTA) and 5 cirrhotic patients with normal distal acidification ability were studied. All the patients were maintained on a 80 mEq/day sodium diet for at least 5 days before the study. Only 2 of the 5 patients with RTA showed a reduced daily sodium excretion while the 3 other patients with RTA had a normal natriuresis. Acidification ability was retested in all the patients after increasing sodium distal delivery by intravenous administration of ethacrynic acid. Some hours after the maximal natriuretic effect induced by ethacrynic acid, a normalization of the acidification test was seen in all patients with RTA. These findings support the concept that although sodium metabolism alterations are not the only factor in the pathogenesis of RTA in liver cirrhosis, reduced availability of sodium at the distal tubule may impair tubular acidification, probably by influencing the distal transtubular potential difference.

Publication Type: Journal Article.

<17>

Unique Identifier [PMID]: 6755051

Authors: Better OS.

Title: Acquired distal renal tubular acidosis in man.

Source: Klinische Wochenschrift. 60(19):1215-7, 1982 Oct 1.

Abstract: Distal renal tubular acidosis (dRTA) may complicate renal transplantation, liver cirrhosis, and obstructive uropathy. Indeed, its occurrence may be an early clue to an episode of rejection of the graft or to obstructive uropathy. The mechanism in most patients with dRTA is impaired distal secretion of protons. In some patients, however, back leak of protons from tubular lumen to blood may abolish distal tubular ability to maintain urine to blood proton gradients. In patients with obstructive uropathy the spectrum of tubular acidosis is widened by the occurrence of additional defects in tubular secretion of potassium and impairment of hydrogen ion secretion secondary to hypoaldosteronism. Hyperkalemia is also seen in "voltage dependent" states such as following the administration of lithium and amiloride. Hyperkalemia per se is conducive to acidosis by a combination of extrarenal and several intrarenal mechanisms.

Publication Type: Journal Article.

<18>

Unique Identifier [PMID]: 7125772

Authors: Hruska KA. Ban D. Avioli LV.

Title: Renal tubular acidosis.

Source: Archives of Internal Medicine. 142(10):1909-13, 1982 Oct.

Publication Type: Case Reports. Journal Article.

<19>

Unique Identifier [PMID]: 6807085

Authors: Batlle D. Kurtzman NA.

Title: Distal renal tubular acidosis: pathogenesis and classification. [Review] [93 refs]

Source: American Journal of Kidney Diseases. 1(6):328-44, 1982 May.

Abstract: Distal renal tubular acidosis results from ineffective addition of hydrogen ions to the lumen of the distal nephron. The syndrome is manifested by hyperchloremic metabolic acidosis often associated with hypokalemia. More recently, it has been recognized that hyperkalemia rather than hypokalemia can be a dominant feature of some cases of distal renal tubular acidosis. It has been generally accepted that all cases of this syndrome ultimately resulted from a similar mechanism. The prevailing view was that the abnormality underlying distal renal tubular acidosis was that of inability to either generate or maintain a steep pH gradient across the distal nephron. Recent advances in our understanding of the process of distal acidification have provided evidence that different mechanisms can alter distal hydrogen ion secretion. In this article, the significance of the various indices of urinary acidification and their use in the characterization of the mechanism underlying distal renal tubular acidosis are revised. A classification of distal renal tubular acidosis on the basis of mechanism is presented. The importance of plasma potassium and renal potassium excretion in the evaluation of patients with distal renal tubular acidosis is emphasized. [References: 93]

Publication Type: Journal Article. Review.

<20>

Unique Identifier [PMID]: 7088070

Authors: Brenner RJ. Spring DB. Sebastian A. McSherry EM. Genant HK. Palubinskas AJ. Morris RC Jr.

Title: Incidence of radiographically evident bone disease, nephrocalcinosis, and nephrolithiasis in various types of renal tubular acidosis.

Source: New England Journal of Medicine. 307(4):217-21, 1982 Jul 22.

Abstract: The syndrome of renal tubular acidosis has been categorized into three physiologic types that have different clinical findings and prognostic and therapeutic implications. We reviewed radiographs of the skeleton and kidneys in 92 patients (56 children and 36 adults) with renal tubular acidosis in order to determine whether the radiologic findings could be related to the type of syndrome. Forty-four patients had Type 1 renal tubular acidosis, 18 had Type 2, and 30 had Type 4. Evidence of skeletal abnormalities was uncommon (17 per cent) and was confined to patients who had the Type 2 disorder or azotemia. The children with Type 2 and skeletal abnormalities had rickets; the adults had osteopenia without pseudofractures. Nephrocalcinosis was evident in approximately one fourth of the group (29 per cent) and was restricted to patients with the Type 1 syndrome. In patients with Type 4, osteopenia was evident in 12 per cent, all of whom were azotemic. Our observations indicate that the radiographic manifestations of renal tubular acidosis are influenced by the physiologic type of renal tubular acidosis.

Publication Type: Journal Article.

<21>

Unique Identifier [PMID]: 6795380

Authors: Batlle DC. Sehy JT. Roseman MK. Arruda JA. Kurtzman NA.

Title: Clinical and pathophysiologic spectrum of acquired distal renal tubular acidosis.

Source: Kidney International. 20(3):389-96, 1981 Sep.

Publication Type: Journal Article.

<22>

Unique Identifier [PMID]: 7012421

Authors: Stinebaugh BJ. Schloeder FX. Tam SC. Goldstein MB. Halperin ML.

Title: Pathogenesis of distal renal tubular acidosis. [Review] [45 refs]

Source: Kidney International. 19(1):1-7, 1981 Jan.

Publication Type: Journal Article. Review.

<23>

Unique Identifier [PMID]: 7453754

Authors: Batlle DC. Arruda JA. Kurtzman NA.

Title: Hyperkalemic distal renal tubular acidosis associated with obstructive uropathy.

Source: New England Journal of Medicine. 304(7):373-80, 1981 Feb 12.

Abstract: We studied renal function in 13 patients with obstructive uropathy and hyperkalemic metabolic acidosis to characterize the pathogenesis of this disorder. Base-line fractional potassium excretion was lower in all patients than in controls with similar glomerular filtration rates. Acetazolamide was given to 11 patients but failed to increase fractional potassium excretion to normal. In five patients, impaired potassium excretion was associated with decreased ammonium excretion, a urinary pH below 5.5 (5.18 +/- 0.07, mean +/- S.E.M.), and aldosterone deficiency. In the remaining eight patients, the urinary pH did not fall below 5.5 (6.4 +/- 0.2) with acidosis, and we failed to lower the urinary pH and increase fractional potassium excretion to normal by administering a mineralocorticoid and sodium sulfate. A syndrome of hyperkalemic distal renal tubular acidosis may occur in patients with obstructive uropathy. In some patients, this syndrome results from a defect in hydrogen and potassium secretion in the distal nephron rather than from aldosterone deficiency. Obstructive uropathy should be included in the differential diagnosis of hyperkalemic acidosis and renal insufficiency.

Publication Type: Journal Article.

<24>

Unique Identifier [PMID]: 25018

Authors: Cogan MC. Arieff AI.

Title: Sodium wasting, acidosis and hyperkalemia induced by methicillin interstitial nephritis. Evidence for selective distal tubular dysfunction.

Source: American Journal of Medicine. 64(3):500-7, 1978 Mar.

Abstract: A 61 year old male patient was studied who manifested dehydration, azotemia, acidosis and hyperkalemia six weeks after exposure to methicillin. Thyroid and adrenal glucocorticoid and mineralocorticoid function were normal. The dehydration was found to be caused by a profound sodium-losing nephropathy; urinary sodium ranged from 78 to 101 meq/day during a salt restricted diet. A distal renal tubular acidosis and a quantitively impaired ability to excrete potassium were also found. These defects were relatively unresponsive to mineralocorticoid or prednisone therapy. A renal biopsy specimen showed an interstitial nephritis which selectively affected distal tubules and was thought to be secondary to methicillin. The data suggest functional impairment specific for the distal tubule, but with only a modest decrease in the glomerular filtration rate.

Publication Type: Case Reports. Journal Article.

<25>

Unique Identifier [PMID]: 797050

Authors: Wilkinson SP.

Title: The kidney in cirrhosis. [Review] [67 refs]

Source: Tijdschrift voor Gastro-Enterologie. 19(3):155-69, 1976.

Publication Type: Journal Article. Review.

<26>

Unique Identifier [PMID]: 783200

Authors: Sebastian A. McSherry E. Morris RC Jr.

Title: Impaired renal conservation of sodium and chloride during sustained correction of systemic acidosis in patients with type 1, classic renal tubular acidosis.

Source: Journal of Clinical Investigation. 58(2):454-69, 1976 Aug.

Abstract: In 10 patients with classic renal tubular acidosis in whom correction of acidosis was sustained with orally administered potassium bicarbonate, renal conservation of sodium was evaluated when dietary intake of sodium was restricted to 9--13 meq/day. In five patients, renal conservation of sodium was impaired by at least one criterion of impairment. In the remaining patients, renal conservation of sodium appeared to be relatively well-maintained, but an impairment could not be excluded. In each of six patients studied during induced water diuresis, including two in whom renal conservation of sodium was not unequivocally impaired, the minimal urinary concentrations of sodium were inappropriately high and the urinary excretion rates of sodium were flow-dependent. These results provide direct evidence that an abnormality in renal transport of sodium can occur in classic renal tubular acidosis, and compel a reconsideration of the pathophysiology of disordered renal transport of sodium in this disorder. The results indicate that in at least some patients with classic renal tubular acidosis impaired renal conservation of sodium is not exclusively a reversible consequence of the renal acidification defect. These findings raise the question whether renal transport of sodium is unimpaired in any patients with classic renal tubular acidosis. In the presently studied patients, the impairment in renal conservation of sodium appeared to be in part the consequence of an impaired ability of the vasopressin-responsive segments of the distal nephron to generate and maintain appropriately steep transepithelial sodium concentration gradients.

Publication Type: Clinical Trial. Journal Article.

<27>

Unique Identifier [PMID]: 3970

Authors: Steinmetz PR. Al-Awqati Q. Lawton WJ.

Title: Nephrology rounds, University of Iowa Hospitals: renal tubular acidosis.

Source: American Journal of the Medical Sciences. 271(1):40-54, 1976 Jan-Feb.

Abstract: We have discussed two patients who had renal tubular acidosis complicated by hypokalemia. The first patient had a distal acidifying defect. Circumstantial evidence has been presented suggesting that exposure to toluene-diisocyanate or toluene-diamine played a role in the pathogenesis. The acidosis and the hypokalemia of this patient were easily corrected by the administration of small amounts of sodium bicarbonate without potassium supplementation. The second patient had an interstitial nephritis of unknown etiology and presented with moderate renal insufficiency, renal tubular acidosis, and proximal as well as distal acidifying defects. The proximal tubular dysfunction was associated with general aminoaciduria and glucosuria. This patient required large quantities of both alkali and potassium to correct the electrolyte abnormalities. The mechanisms of potassium wasting in proximal and distal renal tubular acidosis are reviewed. A classification is presented of cellular defects that may underlie the different renal acidifying defects. Attempts to distinguish between pump and permeability defects from urinary pCO2 levels must take into account the simultaneous HCO-3 concentration, since large pCO2 elevations require the presence of ample HCO-3 in the urine. Permeability defects may impair urinary acidification by either abnormal back flux of H+ out of the lumen or increased influx of HCO-3 into the lumen. In studies of acidification in vitro, amphotericin B causes increased H+ permeability and has little effect on HCO-3 permeability. Toluene-diamine causes a marked permeability defect which is reversible, but remains to be defined in terms of the ion species, HCO-3 or H+, affected. At times, hyperchloremic acidosis is caused by distal defects in net acid excretion that occur without impairment of the H+ gradient. In certain patients with hypoaldosteronism, for example, distal H+ secretion may be reduced without change in the force of the H+ pump.

Publication Type: Journal Article.

<28>

Unique Identifier [PMID]: 4433193

Authors: Lubowitz H. Avioli LV.

Title: The Jewish Hospital of St. Louis. Therapeutic grand rounds number 8: Renal tubular acidosis.

Source: Archives of Internal Medicine. 134(6):1120-4, 1974 Dec.

Publication Type: Journal Article.

<29>

Unique Identifier [PMID]: 4812435

Authors: Halperin ML. Goldstein MB. Haig A. Johnson MD. Stinebaugh BJ.

Title: Studies on the pathogenesis of type I (distal) renal tubular acidosis as revealed by the urinary PCO2 tensions.

Source: Journal of Clinical Investigation. 53(3):669-77, 1974 Mar.

Publication Type: Journal Article.

<30>

Unique Identifier [PMID]: 4583934

Authors: Wilson DR. Siddiqui AA.

Title: Renal tubular acidosis after kidney transplantation. Natural history and significance.

Source: Annals of Internal Medicine. 79(3):352-61, 1973 Sep.

Publication Type: Journal Article.

<31>

Unique Identifier [PMID]: 4600133

Authors: Morris RC Jr. McSherry E.

Title: Symposium on acid-base homeostasis. Renal acidosis. [Review] [116 refs]

Source: Kidney International. 1(5):322-40, 1972 May.

Publication Type: Journal Article. Review.