sodium-bicarbonate and Acidosis--Renal-Tubular

Distal renal tubular acidosis is a relatively infrequent condition with complex pathophysiology that can present with life-threatening electrolyte abnormalities.. We describe a case of a 57-year-old Caucasian woman with previous episodes of hypokalemia, severe muscle weakness, and fatigue. Upon further questioning, symptoms of dry eye and dry mouth became evident. Initial evaluation revealed hyperchloremic metabolic acidosis, severe hypokalemia, persistent alkaline urine, and a positive urinary anion gap, suggestive of distal renal tubular acidosis. Additional laboratory workup and renal biopsy led to the diagnosis of primary Sjögren's syndrome with associated acute tubulointerstitial nephritis. After potassium and bicarbonate supplementation, immunomodulatory therapy with hydroxychloroquine, azathioprine, and prednisone was started. Nonetheless, her renal function failed to improve and remained steady with an estimated glomerular filtration rate of 42 ml/min/1.73 m. Cases of renal tubular acidosis should be carefully evaluated to prevent adverse complications, uncover a potentially treatable condition, and prevent the progression to chronic kidney disease. Repeated episodes of unexplained hypokalemia could be an important clue for diagnosis.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Disease Progression; Female; Glomerular Filtration Rate; Humans; Hypokalemia; Immunomodulation; Middle Aged; Potassium; Sjogren's Syndrome; Sodium Bicarbonate; Trace Elements; Treatment Outcome

Subjects with CKD and reduced glomerular filtration rate are at risk for chronic metabolic acidosis, and CKD is its most common cause. Untreated metabolic acidosis, even in its mildest forms, is associated with increased mortality and morbidity and should therefore be treated. If reduced glomerular filtration rate or the tubule abnormality causing chronic metabolic acidosis cannot be corrected, it is typically treated with dietary acid (H

Topics: Acid-Base Equilibrium; Acidosis; Acidosis, Renal Tubular; Animals; Bicarbonates; Diet; Dietary Proteins; Fruit; Glomerular Filtration Rate; Humans; Renal Insufficiency, Chronic; Sodium Bicarbonate; Vegetables

The human kidneys produce approximately 160-170 L of ultrafiltrate per day. The proximal tubule contributes to fluid, electrolyte, and nutrient homeostasis by reabsorbing approximately 60%-70% of the water and NaCl, a greater proportion of the NaHCO3, and nearly all of the nutrients in the ultrafiltrate. The proximal tubule is also the site of active solute secretion, hormone production, and many of the metabolic functions of the kidney. This review discusses the transport of NaCl, NaHCO3, glucose, amino acids, and two clinically important anions, citrate and phosphate. NaCl and the accompanying water are reabsorbed in an isotonic fashion. The energy that drives this process is generated largely by the basolateral Na(+)/K(+)-ATPase, which creates an inward negative membrane potential and Na(+)-gradient. Various Na(+)-dependent countertransporters and cotransporters use the energy of this gradient to promote the uptake of HCO3 (-) and various solutes, respectively. A Na(+)-dependent cotransporter mediates the movement of HCO3 (-) across the basolateral membrane, whereas various Na(+)-independent passive transporters accomplish the export of various other solutes. To illustrate its homeostatic feat, the proximal tubule alters its metabolism and transport properties in response to metabolic acidosis. The uptake and catabolism of glutamine and citrate are increased during acidosis, whereas the recovery of phosphate from the ultrafiltrate is decreased. The increased catabolism of glutamine results in increased ammoniagenesis and gluconeogenesis. Excretion of the resulting ammonium ions facilitates the excretion of acid, whereas the combined pathways accomplish the net production of HCO3 (-) ions that are added to the plasma to partially restore acid-base balance.

Topics: Acidosis, Renal Tubular; Biological Transport, Active; Humans; Kidney Tubules, Proximal; Phosphates; Sodium Bicarbonate; Sodium Chloride

Metabolic acidosis is a frequent but asymptomatic complication in chronic kidney disease (CKD). In early stages of CKD acidosis is limited to the renal tissue and progresses to reduced serum bicarbonate levels. Reduced renal tissue pH and increased ammoniagenesis are the key mechanisms of the kidney to enhance acid excretion to the urine. The expressed protein patterns in the proximal tubular epithelial cells change remarkably, the proximal convoluted tubule develops hypertrophy, and an intra-renal enhanced renin-angiotensin-system leads to interstitial fibrosis. Since nephrons are numerically reduced in CKD each remaining functional unit has to progressively increase these mechanisms to keep up the equilibrium. The adverse effects of chronic metabolic acidosis include aside from acceleration of progression of kidney disease, the development or exacerbation of bone disease, increased degradation of muscle with muscle wasting, enhanced protein degradation and inflammation. Genome wide association studies demonstrated that tubular acid-base transporters are involved in the development of arterial hypertension. Several retrospective analyses have indicated that low serum bicarbonate predicts death in cohorts with CKD and cardiovascular disease. All studies confirmed a U-shaped association of mortality and serum bicarbonate, indicating that both, acidosis and alkalosis are associated with increased mortality. Randomized controlled trials showed that base substitution, either by modification of the diet or by simply adding alkalizing agents, might halt the decline of kidney function in subjects with CKD. In 2012 a meta-analysis concluded that alkali therapy might provide a long-term favorable effect on renal function in patients with CKD.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Bone and Bones; Humans; Hypertension; Insulin Resistance; Meta-Analysis as Topic; Muscle, Skeletal; Proteins; Randomized Controlled Trials as Topic; Renal Insufficiency, Chronic; Sodium Bicarbonate; Treatment Outcome

The mammalian Slc4 (Solute carrier 4) family of transporters is a functionally diverse group of 10 multi-spanning membrane proteins that includes three Cl-HCO3 exchangers (AE1-3), five Na(+)-coupled HCO3(-) transporters (NCBTs), and two other unusual members (AE4, BTR1). In this review, we mainly focus on the five mammalian NCBTs-NBCe1, NBCe2, NBCn1, NDCBE, and NBCn2. Each plays a specialized role in maintaining intracellular pH and, by contributing to the movement of HCO3(-) across epithelia, in maintaining whole-body pH and otherwise contributing to epithelial transport. Disruptions involving NCBT genes are linked to blindness, deafness, proximal renal tubular acidosis, mental retardation, and epilepsy. We also review AE1-3, AE4, and BTR1, addressing their relevance to the study of NCBTs. This review draws together recent advances in our understanding of the phylogenetic origins and physiological relevance of NCBTs and their progenitors. Underlying these advances is progress in such diverse disciplines as physiology, molecular biology, genetics, immunocytochemistry, proteomics, and structural biology. This review highlights the key similarities and differences between individual NCBTs and the genes that encode them and also clarifies the sometimes confusing NCBT nomenclature.

Topics: Acidosis, Renal Tubular; Animals; Biological Evolution; Chloride-Bicarbonate Antiporters; Humans; Molecular Sequence Data; Sodium Bicarbonate

Renal tubular acidosis (RTA) is common in adults with primary Sjogren syndrome (pSS) but to date this condition has only been identified in 12 pediatric cases of pSS. Here we present the case of a 13-year-old, otherwise asymptomatic girl in whom the search for the etiology of incidentally found nephrocalcinosis led to diagnosis of distal RTA and nephrogenic diabetes insipidus secondary to SS-associated tubulointerstitial nephritis. Immunosupressive treatment and alkali/electrolyte supplementation resulted in stable renal function over the 6-year follow-up. A review of the literature focuses on two aspects of pSS: (1) the difficulties in diagnosing pSS in childhood and (2) clinical-pathological features, treatment and outcome of renal tubulointerstitial disease in childhood pSS. SS should be considered in older children, particularly females with otherwise unexplained RTA. A careful search for other renal dysfunctions is necessary, and renal biopsy may be of value in assessing the extent of renal damage and the need for immunomodulatory therapy.

Topics: Acidosis, Renal Tubular; Adolescent; Electrolytes; Female; Humans; Immunosuppressive Agents; Incidental Findings; Nephritis, Interstitial; Nephrocalcinosis; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

The occurrence and pathogenesis of metabolic acidosis after renal transplantation is reviewed. Posttransplant acidosis is shown to be a key mechanism for major metabolic complications in mineral and muscle metabolism, and for anemia, discussed in the context of both acidosis and renal transplantation.. Continuous improvement in kidney transplant survival has shifted attention to long-term outcomes, specifically to disorders linked to cardiovascular disease, physical capacity and quality of life. Metabolic acidosis is gaining growing acceptance as a clinical entity and has occasionally come into focus in the context of renal transplantation. The possible link to metabolic disturbances resulting in impairment of musculoskeletal disorders and physical limitations, however, has not been considered specifically.. Available evidence suggests a high prevalence of (compensated) metabolic acidosis after renal transplantation, presenting as low serum bicarbonate and impaired renal acid excretion. This condition is associated with relevant disorders in mineral metabolism and muscle function. Current knowledge about the effects of acidosis on renal electrolyte handling, mineral metabolism and protein synthesis suggests that acid/base derangements contribute to the muscle and bone pathology, as well as anemia, encountered after kidney transplantation. Consequently, posttransplant acidosis may be a relevant factor in the causal pathway of impaired physical capacity observed in this patient group.

Topics: Acidosis; Acidosis, Renal Tubular; Anemia; Bone Diseases, Endocrine; Humans; Insulin Resistance; Kidney Transplantation; Muscular Diseases; Nephrons; Prognosis; Quality of Life; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Child; Child, Preschool; Citrates; Citric Acid; Failure to Thrive; Female; Follow-Up Studies; Growth Disorders; Humans; Infant; Sodium Bicarbonate; Sodium Citrate

Topics: Acidosis, Renal Tubular; Diagnosis, Differential; Humans; Hypokalemia; Neurons, Afferent; Peripheral Nervous System Diseases; Prognosis; Sodium Bicarbonate

Metabolic acidosis occurs frequently in small children. The most common causes are hypoxia, sepsis, gastroenteritis and hypovolaemia. Calculation of the anion gap is useful in establishing the cause. An increased anion gap represents unmeasured anions, e.g. lactate in lactic acidosis. Metabolic acidosis was diagnosed in two boys aged one year and six weeks respectively. The first patient had a normal, the second an increased anion gap in blood. By determining the pH and the anion gap in urine it is possible to distinguish between a proximal and a distal tubular disease. The first patient had distal renal tubular acidosis; he recovered after correction of the acidosis. The second patient had a defect in the mitochondrial respiratory chain; he died at the age of seven months.

Topics: Acid-Base Imbalance; Acidosis, Lactic; Acidosis, Renal Tubular; Diagnosis, Differential; Fatal Outcome; Humans; Infant; Male; Mitochondrial Myopathies; Osteomalacia; Sodium Bicarbonate

An infant girl presented with recurrent episodes of Reye-like syndrome associated with hypoketosis and plasma carnitine levels in the high-normal range. A liver biopsy revealed massive macrovesicular steatosis. Ketogenesis was absent after a long-chain triglyceride loading test; in contrast, the medium-chain triglyceride loading test resulted in a brisk rise in plasma ketone concentration. Carnitine palmitoyltransferase I deficiency was demonstrated in cultured skin fibroblasts. Hypoglycemia was only found once in the neonatal period. Renal carnitine handling was normal except for a higher renal threshold for free carnitine. Mild, persistent metabolic acidosis was a constant feature, even during periods between metabolic decompensation. Evaluation of the renal acidification capacity showed a failure to acidify the urine during spontaneous acidosis but increased acid excretion and a normal decrease of urinary pH after acid loading. Also, a small difference between urine and blood PCO2 was found after bicarbonate administration. This acidification defect can best be explained as an abnormality in distal tubular H+ secretion: a rate-dependent distal tubular acidosis.off is speculated that long-chain acylcarnitines, substances that cannot be formed by carnitine palmitoyltransferase I-deficient patients, play an essential role in renal acid-base homeostasis.

Topics: Acidosis, Renal Tubular; Biological Transport; Carnitine; Carnitine O-Palmitoyltransferase; Female; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Kidney Function Tests; Protons; Reye Syndrome; Secretory Rate; Sodium Bicarbonate

The traditionally descriptive classification of DRTA disorders is being refined as molecular defects are identified. Diagnosis of DRTA is easy: Hyperchloremic acidosis should arouse suspicion, and confirmation can be obtained by measuring urinary pH and ammonia excretion. Treatment is also relatively straightforward and readily corrects all manifestations of the acidification defect.

Topics: Acidosis, Renal Tubular; Ammonia; Humans; Hydrogen-Ion Concentration; Kidney Tubules, Distal; Molecular Biology; Sodium Bicarbonate; Urinalysis

In normal adults eating diets with standard protein contents, urinary excretion of NH4 approximates 40 mmol/24 hours and urinary pH is variable. In patients with metabolic acidosis, a urinary pH under 5.5 suggests an extra-renal cause whereas a urinary pH above 5.5 suggests a renal disorder, although there are many exceptions to this rule of thumb. However, urinary excretion of NH4 is always above 70 mmol/24 hours in extra-renal acidosis and less than or equal to 40 mmol/24 hours in renal acidosis; the two situations can readily be differentiated by determining the urinary anion gap which is absent in the former case and present in the latter. Acidosis due to nephron loss is readily diagnosed on the basis of advanced renal failure with an elevation in nonassayed plasma anions, contrasting with the increased serum chloride level found in tubular acidosis. Oral NaHCO3 loading followed by determination of the fractional excretion of HCO3 or, preferably, of the TmHCO3 normalized for glomerular filtration rate differentiates proximal tubular acidosis (decreased TmHCO3) from distal tubular acidosis (normal or increased TmHCO3). In the latter case, decreased serum potassium levels suggest distal tubular acidosis due to defective H(+)-ATPase or H+/K(+)-ATPase pump function (no increase in urinary PCO2 after oral NaHCO3 loading) or to inability of the kidney to develop a normal H+ gradient (normal increase in urinary PCO2). Increased serum potassium levels suggest conditions involving either hypoaldosteronism or alterations in transepithelial voltage or pseudo-hypoaldosteronism. The incidence of distal tubular acidosis with increased serum potassium levels is rising, whereas tubular acidosis with low serum potassium levels remains infrequent.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Ammonia; Bicarbonates; Carbonates; Chlorides; Clinical Protocols; Decision Trees; Glomerular Filtration Rate; Humans; Hydrogen-Ion Concentration; Kidney Tubules, Distal; Kidney Tubules, Proximal; Potassium; Sodium; Sodium Bicarbonate; Urinalysis

Renal tubular acidosis represents a heterogenous group of disorders with various etiologies and mechanisms. The physiopathologic basis of each type of renal tubular acidosis is reviewed, focusing on the laboratory investigations necessary to define the nature of the hyperchloremic renal tubular acidosis. Clinically, the four types of renal tubular acidosis can be associated with complications such as osteomalacia, urolithiasis and failure to thrive. Very often, the chronic administration of alkali results in normal growth and development, and greatly reduces the risk of stone formation or nephrocalcinosis.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Bicarbonates; Child; Female; Growth Disorders; Humans; Infant; Kidney Calculi; Osteomalacia; Sodium; Sodium Bicarbonate

Follow-up of a previously reported family with dominantly inherited adult onset hypophosphatemic osteomalacia with Fanconi syndrome and diabetes mellitus has shown that both the proposita and her affected sister have developed renal glomerular failure. We describe the evolution of renal failure in this family and discuss the possible mechanisms involved. The development of renal tubular acidosis in this condition further impairs renal function and we suggest that correction of systemic acidosis might improve renal function and prevent further decline in these patients.

Topics: Acidosis, Renal Tubular; Adolescent; Adult; Bicarbonates; Child; Child, Preschool; Diabetes Complications; Diabetes Mellitus; Fanconi Syndrome; Female; Follow-Up Studies; Humans; Kidney Failure, Chronic; Male; Osteomalacia; Pedigree; Phosphates; Sodium; Sodium Bicarbonate

Tubular acidosis is diagnosed when hyperchloremic acidosis is associated with inappropriate NH4 excretion (less than or equal to 40 mmol/24 hours). Urinary pH is variable because it depends on the secretion of H+ into the collecting duct and is inversely correlated with the amount of ammonia available in the urine. Administration of NaHCO3 for diagnostic purpose allows to eliminate proximal tubular acidosis and to measure the elevation of urinary PCO2 reflecting the secretion of H+ in the collecting duct. Hypokalemia points towards distal tubular acidosis, either by defect of H(+)-ATPases pumps, or by the incapacity to create a normal gradient of H+. In contrast hyperkalemia suggests distal tubular acidosis associated either with hypoaldosteronism or with diminution of trans-epithelial voltage or with pseudohypoaldosteronism. The incidence of distal tubular acidosis with hyperkalemia is increasing whereas distal tubular acidosis with hypokalemia remain rare.

Topics: Acidosis, Renal Tubular; Bicarbonates; Humans; Hyperkalemia; Hypokalemia; Sodium; Sodium Bicarbonate

Urine pH during acidemia and urine PCO2 upon alkalization both may be useful to indicate H+ secretion from collecting ducts. The urine anion gap has been used to detect urinary NH4+ for differential diagnosis of hyperchloremic metabolic acidosis. We have previously demonstrated that the lack of normal H(+)-ATPase may underlie secretory defect distal renal tubular acidosis (dRTA). In this study we evaluated the diagnostic value of the urine-blood (U-B) PCO2 in H(+)-ATPase defect dRTA, and compared it with that of urine pH and urine anion gap during acidemia.. In H(+)-ATPase defect dRTA, the diagnostic values of three urinary parameters were evaluated: (1) urine pH measured after acid (NH4Cl) loading; (2) urine-to-blood carbon dioxide tension gradient (U-B PCO2) during alkali (NaHCO3) loading; and (3) urine anion gap during acidemia. Seventeen patients were diagnosed as having H(+)-ATPase defect dRTA based on reduced urinary NH4+ and an absolute decrease in H(+)-ATPase immunostaining in intercalated cells on renal biopsy. Eight patients with non-dRTA renal disease served as control patients.. Upon NaHCO3 loading, U-B PCO2 was < or =30 mm Hg in all 17 dRTA patients and >30 mm Hg in all 8 control patients. With NH4Cl loading, urine pH was >5.4 in 15 of 17 dRTA patients and < or =5.4 in 7 of 8 control patients, and the urine anion gap was >5 mmol/L in 13 of 17 dRTA patients and< or =5 mmol/L in 6 of 8 control patients. Therefore, the sensitivity and specificity of U-B PCO2 < or =30 mm Hg during NaHCO3 loading were both 100%, whereas those of urine pH >5.4 or urine anion gap >5 mmol/L during NH4Cl loading were below 90%. In control patients, the U-B PCO2 was found to be well correlated with the urinary NH4+ (r= 0.79, P < 0.05).. The U-B PCO2 during NaHCO3 loading is an excellent diagnostic index of H(+)-ATPase defect dRTA.

Topics: Acidosis, Renal Tubular; Adult; Ammonium Chloride; Anions; Carbon Dioxide; Humans; Hydrogen-Ion Concentration; Kidney Cortex; Proton-Translocating ATPases; Sodium Bicarbonate

Premature infants receiving alimentation with cow milk-based formulas run a considerably high risk of incipient late metabolic acidosis, an early stage developing of manifest late metabolic acidosis. Is bone metabolism involved in pathophysiologic mechanisms characterizing this early stage of retention acidosis?. Urinary ionography was performed in 10 premature infants with spontaneous development of incipient late metabolic acidosis (indicated by urine pH < 5.4 on 2 consecutive days) and 10 pair-matched premature infants with normal values of urine pH; both groups were receiving full oral nutrition with the same standard formula. Moreover, in 37 premature infants with incipient late metabolic acidosis who were randomly allocated to oral therapy with 2 mmol. kg(-1). d(-1) of either NaHCO 3 or NaCl over a period of 7 days, urinary excretion of calcium and phosphorus was assessed on day 1 and day 7.. Incipient late metabolic acidosis was accompanied by increased phosphaturia in premature infants receiving full oral nutrition. Seventeen premature infants receiving NaCl therapy (19 treatment periods) showed increased calciuria from day 1 to day 7, whereas, in 20 premature infants receiving NaHCO 3 therapy (23 treatment periods), calcium or phosphorus excretion in urine did not increase.. The data of urinary calcium and phosphorus excretion in premature infants support the hypothesis that bone mineralization may already be impaired in the early stage of incipient late metabolic acidosis.

Topics: Acidosis, Renal Tubular; Bone and Bones; Bone Development; Calcium; Humans; Hydrogen-Ion Concentration; Infant; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Kidney; Phosphorus; Sodium Bicarbonate; Sodium Chloride

This study was designed to investigate the effect of tacrolimus (FK506) and of cyclosporine (CsA) on tubular function in renal graft recipients. Patients were randomised after renal transplantation to immunosuppressive treatment with FK506 (n = 8) or CsA (n = 8). Patients had a mean age of 45.7 +/- 3.4 yr; there was no difference in age, sex, HLA status or CMV mismatches. Neither was there any difference in the frequency of episodes of acute kidney failure between the groups, nor was there a significant difference in the frequency of episodes of kidney rejection within the first year. The mean FK506 level at the time lay at 14.7 +/- 14.4 ng/mL whole blood, and the mean CsA level at the time of study was 162 +/- 25 ng/mL whole blood. We performed renal function studies 6 months after transplantation: CIn, CPAH, NaHCO3 loading, and Na2SO4 loading. There was no significant impairment of GFR in patients treated with FK506 with 53.6 +/- 2.5 mL/min as compared to 58 +/- 6 mL in group 2. Plasma renin activity (0.6 +/- 0.4 ng/mL vs 2.3 +/- 3; p < 0.01) and aldosterone (69 +/- 17 vs 157 +/- 28.2 pg/mL; p < 0.05) were significantly decreased during treatment with FK506. Fractional HCO3 excretion was low in both groups, indicating that bicarbonate reabsorption in the proximal nephron was unimpaired. Distal renal tubular acidosis was demonstrated in 4 patients of group 1 but in only 1 of group 2. Potassium levels were slightly increased in patients treated with FK506 (5.4 +/- 0.2 mmoL/L) as compared to cyclosporine (4.9 +/- 0.3 mmoL/L; p < 0.05). Distal hydrogen ion secretion, evaluated by the ability to increase urinary pCO2 in a highly alkaline urine, was impaired in patients treated with FK506 (U-B pCO2: 16.1 +/- 4 vs 36 +/- 5.8; p < 0.05) as compared to patients treated with CsA. The maximum acidification capability (NAE) was slightly lowered during therapy with FK506 (67.5 +/- 11.8 versus 86.6 +/- 16.5 mumoL/min, ns). We conclude that FK506 administration results in a decrease in the rate of hydrogen ion secretion by the collecting tubules. This defect was disclosed by the finding of a subnormal pCO2 in a highly alkaline urine. These results show that FK506 is able to induce distal tubular acidosis. Distal tubular acidosis is part of FK506 induced nephrotoxicity, the pathogenesis of this type of hyperkalemic metabolic acidosis found in patients treated with FK506 after renal transplantation has to be further elucidated.

Topics: Acidosis, Renal Tubular; Cyclosporine; Glomerular Filtration Rate; Humans; Hydrogen-Ion Concentration; Immunosuppressive Agents; Kidney Function Tests; Kidney Transplantation; Kidney Tubules, Distal; Middle Aged; Sodium Bicarbonate; Sulfates; Tacrolimus

Two hundred and eighty-two patients with birthweights below 2.0 kg were routinely screened for spontaneous development of maximum renal acid stimulation (urine-pH < 5.4). Sixty episodes in 53 patients of incipient late metabolic acidosis (urine pH < 5.4 on 2 consecutive days) were randomly allocated to oral therapy with 2 mmol/kg/day of either NaHCO3 or NaCl for 7 days. All 27 patients on NaHCO3 therapy, but only 15 from 26 patients on NaCl therapy, showed an increase in urine pH values, combined with a relatively high gain in body weight and a tendency to increased N-assimilation. Eleven patients on NaCl therapy showed persistent maximal renal acid stimulation on all 7 days with possibly lower weight gain and no clear change in N-assimilation. Thus, in patients with incipient late metabolic acidosis, NaCl therapy is not as beneficial as NaHCO3 therapy.

Topics: Acidosis, Renal Tubular; Body Weight; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Infant, Premature, Diseases; Infant, Small for Gestational Age; Prospective Studies; Sodium Bicarbonate; Sodium Chloride

Primary Sjögren's syndrome (pSS) is a chronic slowly progressive autoimmune disease characterised by lymphocytic infiltration of salivary and lacrimal glands with varying degree of systemic involvement. Renal involvement, a recognised extraglandular manifestation of pSS, is commonly related to tubular dysfunction and generally manifests as distal renal tubular acidosis (RTA), proximal RTA, tubular proteinuria and nephrogenic diabetes insipidus. Untreated long-standing RTA is known to cause metabolic bone disease. Here, we present the report of a patient with sclerotic metabolic bone disease related to pSS with combined distal and proximal RTA and negative workup for other causes of sclerotic bone disease. A significant clinical and biochemical improvement, including recovery of proximal tubular dysfunction, was noted with alkali therapy. This case suggests the need to consider pSS in the diagnostic algorithm of a patient presenting with sclerotic bone disease.

Topics: Absorptiometry, Photon; Acidosis, Renal Tubular; Adult; Alkaline Phosphatase; Back Pain; Bone Density; Bone Diseases, Metabolic; Female; Humans; Potassium Citrate; Radionuclide Imaging; Sjogren's Syndrome; Skeleton; Sodium Bicarbonate

A 47-year-old female patient presented nausea and vomiting for half a year and elevated serum creatinine for 3 days. Proximal renal tubular acidosis (RTA) complicated with anemiawas confirmed after admission. Secondary factors, such as autoimmune disease, drugs, poison, monoclonal gammopathy, were excluded. Renal biopsy revealed acute interstitial nephritis. The patient was administrated with daily prednisone 50 mg, sodium bicarbonate 4 g, 3 times per day, erythropoietin 3 000 U, 2 times per week, combined with potassium, calcium, and calcitriol tablets. Serum creatinine reduced to 90 μmol/L. However nausea and vomiting deteriorated with lactic acidosis. Bone marrow biopsy indicated the diagnosis of non-Hodgkin lymphoma, therefore the patient was treated with chemotherapy. Although metabolic acidosis improved gradually after chemotherapy, severe pneumocystis carinii pneumonia developed two weeks later. The patient refused further treatment and was discharged.. 患者女性，47岁。因恶心、呕吐半年，发现肾功能异常（血肌酐255 μmol/L）3 d就诊，入院检查发现近端肾小管酸中毒合并贫血，排除自身免疫病、药物、毒物、单克隆免疫球蛋白病等继发因素，肾脏穿刺活检组织病理提示急性间质性肾炎，予泼尼松50 mg/d；碳酸氢钠4 g，3次/d；促红细胞生成素3 000 U，2次/周；氯化钾缓释片500 mg，3次/d；碳酸钙500 mg，3次/d；骨化三醇0.5 μg，1次/d。患者血肌酐恢复至90 μmol/L，但随诊期间患者恶心呕吐加重，再次检查发现合并乳酸酸中毒（乳酸14.1 mmol/L）。骨髓穿刺提示非霍奇金淋巴瘤，予CHOP方案化疗，期间乳酸酸中毒逐步好转（乳酸由14.5 mmol/L降至3.1 mmol/L），半个月后发生重症耶氏肺孢子菌肺炎，最终放弃治疗出院。.

Topics: Acidosis, Lactic; Acidosis, Renal Tubular; Anemia; Antineoplastic Agents; Biopsy; Creatinine; Erythropoietin; Female; Humans; Lymphoma, Non-Hodgkin; Middle Aged; Nausea; Pneumonia, Pneumocystis; Prednisone; Renal Insufficiency; Sodium Bicarbonate; Treatment Refusal; Vomiting

Topics: Acidosis, Renal Tubular; Adult; Autoantibodies; Calcium; Calcium-Regulating Hormones and Agents; Diagnosis, Differential; Electromyography; Female; Humans; Hypokalemic Periodic Paralysis; Male; Muscle Weakness; Potassium; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

Topics: Acidosis, Renal Tubular; Administration, Oral; Ammonia; Diagnosis, Differential; Exome Sequencing; Female; Fluid Therapy; Food Intolerance; Humans; Hyperammonemia; Hypercalcemia; Infant; Metabolism, Inborn Errors; Potassium Citrate; Sodium Bicarbonate; Treatment Outcome; Vacuolar Proton-Translocating ATPases; Weight Gain

A case of distal renal tubular acidosis occurring as a transient complication in a 13-year-old female greyhound dog with gastric-dilatation-volvulus was diagnosed. The acute renal ischemia and inflammatory condition associated with this syndrome could be considered the main underlying mechanisms responsible for the acute, severe, and complicating renal tubular dysfunction.

Topics: Acidosis, Renal Tubular; Animals; Dog Diseases; Dogs; Female; Gastric Dilatation; Sodium Bicarbonate; Stomach Volvulus; Treatment Outcome

Topics: Acidosis, Renal Tubular; Adult; Cesarean Section; Female; Gestational Age; Heart Rate, Fetal; Humans; Nephrocalcinosis; Potassium Citrate; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Sjogren's Syndrome; Sodium Bicarbonate; Ultrasonography, Prenatal

A 61-year-old woman was admitted to our hospital because of muscle paralysis and was found to have severe hypokalemia. A gallium-67 scintigram revealed a positive accumulation in the bilateral salivary glands, and a labial minor salivary gland biopsy demonstrated a massive lymphocyte infiltrate around the salivary ducts. She was diagnosed with Sjögren's syndrome (SS) associated with renal tubular acidosis. Renal biopsy revealed tubulointerstitial nephritis with a mild focal infiltration of lymphocytes and plasma cells. These pathological features were compatible with SS with renal involvement. Acidosis and hypokalemia were corrected with sodium bicarbonate and potassium chloride, which relieved the patient's symptoms. Although steroid therapy has been reported to be effective in SS-associated tubulointerstitial nephritis, the patient's serum potassium level could be controlled without administering steroids during the first admission. Five years later, she was admitted again because of severe liver dysfunction attributed to autoimmune hepatitis. Oral administration of prednisolone resulted in the normalization of her transaminase levels, and the control of her serum potassium level became easier. It has been reported that patients with SS with salivary gland involvement tend to have hepatic complications, and those with hepatic complications tend to have renal involvement. Physicians should be aware of hepatic involvement, even if there is no liver dysfunction at the initial diagnosis of SS with salivary gland and renal involvement. It remains uncertain whether the administration of a low dose of steroids before the onset of autoimmune hepatitis might have prevented the development of liver dysfunction in our patient.

Topics: Acidosis, Renal Tubular; Administration, Oral; Female; Hepatitis, Autoimmune; Humans; Hypokalemia; Kidney; Lymphocytes; Middle Aged; Nephritis, Interstitial; Plasma Cells; Potassium Chloride; Prednisolone; Severity of Illness Index; Sjogren's Syndrome; Sodium Bicarbonate

A 16-year-old man was transferred to our emergency department seven hours after ingesting 486 aspirin tablets. His blood salicylate level was 83.7 mg/dL. He was treated with fluid resuscitation and sodium bicarbonate infusion, and his condition gradually improved, with a decline in the blood salicylate level. However, eight days after admission, he again reported nausea, a venous blood gas revealed metabolic acidosis with a normal anion gap. The blood salicylate level was undetectable, and a urinalysis showed glycosuria, proteinuria and elevated beta-2 microglobulin and n-acetyl glucosamine levels, with a normal urinary pH despite the acidosis. We diagnosed him with relapse of metabolic acidosis caused by renal tubular acidosis.

Topics: Acid-Base Equilibrium; Acidosis; Acidosis, Renal Tubular; Adolescent; Aspirin; Fluid Therapy; Humans; Male; Sodium Bicarbonate; Urinalysis

A 62-year-old woman was diagnosed with severe osteomalacia caused by renal tubular acidosis associated with Sjögren's syndrome. She was treated with sodium bicarbonate, risedronate, alfacalcidol, and prednisolone (1 mg/kg). By 24 months, renal tubular acidosis was improved and the bone density had normalized. Here we report the successful amelioration of bone lesions through a multidisciplinary approach that improved renal tubular acidosis, with a special focus on treatment of the underlying inflammatory disorder with glucocorticoids.

Topics: Acidosis, Renal Tubular; Bone Density Conservation Agents; Drug Therapy, Combination; Etidronic Acid; Female; Glucocorticoids; Humans; Hydroxycholecalciferols; Middle Aged; Osteomalacia; Prednisolone; Risedronic Acid; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

We report a rare case of a 38-year-old female who presented with sudden onset flaccid quadriplegia and respiratory arrest with no significant past clinical history. She was later found to have hypokalemia due to distal renal tubular acidosis and further diagnosed as case of Sjogrens Syndrome.

Topics: Acidosis, Renal Tubular; Administration, Intravenous; Adult; Antibodies, Antinuclear; Female; Glucocorticoids; Humans; Hypokalemic Periodic Paralysis; Potassium Chloride; Prednisolone; Quadriplegia; Salivary Glands; Sjogren's Syndrome; Sodium Bicarbonate; Treatment Outcome

The human SLC4A5 gene has been identified as a hypertension susceptibility gene based on the association of single nucleotide polymorphisms with blood pressure (BP) levels and hypertension status. The biochemical basis of this association is unknown particularly since no single gene variant was linked to hypertension in humans. SLC4A5 (NBCe2, NBC4) is expressed in the collecting duct of the kidney and acts as an electrogenic ion-transporter that transports sodium and bicarbonate with a 1:2 or 1:3 stoichiometry allowing bicarbonate reabsorption with relatively minor concurrent sodium uptake. We have mutated the Slc4a5 gene in mice, which caused a persistent increase in systolic and diastolic BP. Slc4a5 mutant mice also displayed a compensated metabolic acidosis and hyporeninemic hypoaldosteronism. Analysis of kidney physiology revealed elevated fluid intake and urine excretion and increased glomerular filtration rate. Transcriptome analysis uncovers possible compensatory mechanisms induced by SLC4A5 mutation, including upregulation of SLC4A7 and pendrin as well as molecular mechanisms associated with hypertension. Induction of metabolic alkalosis eliminated the BP difference between wild-type and Slc4a5 mutant mice. We conclude that the impairment of the function of SLC4A5 favors development of a hypertensive state. We reason that the loss of sodium-sparing bicarbonate reabsorption by SLC4A5 initiates a regulatory cascade consisting of compensatory bicarbonate reabsorption via other sodium-bicarbonate transporters (e.g. SLC4A7) at the expense of an increased sodium uptake. This will ultimately raise BP and cause hypoaldosteronism, thus providing a mechanistic explanation for the linkage of the SLC4A5 locus to hypertension in humans.

Topics: Acid-Base Equilibrium; Acidosis, Renal Tubular; Aldosterone; Animals; Atrial Natriuretic Factor; Blood; Blood Chemical Analysis; Blood Pressure; Gene Expression Regulation; Glomerular Filtration Rate; Hydrogen-Ion Concentration; Hypertension; In Situ Hybridization; Kidney; Kidney Tubules; Male; Mice; Mutation; Sequence Deletion; Sodium; Sodium Bicarbonate; Sodium-Bicarbonate Symporters; Urination; Urine

Topics: Acidosis; Acidosis, Renal Tubular; Adult; Aquaporin 2; Biomarkers; Biopsy; Female; Humans; Immunohistochemistry; Kidney Tubules; Nephrocalcinosis; Proton-Translocating ATPases; Sodium Bicarbonate; Tomography, X-Ray Computed; Treatment Outcome

We have identified a novel homozygous nonsense mutation (W516X) in the kidney-type electrogenic sodium bicarbonate cotransporter 1 (NBC1) in a patient with isolated proximal renal tubular acidosis (pRTA). To specifically address the pathogenesis of this mutation, we created NBC1 W516X knock-in mice to match the patient's abnormalities. The expression of NBC1 mRNA and protein in the kidneys of NBC1(W516X/W516X) mice were virtually absent, indicating that nonsense-mediated mRNA decay (NMD) is involved in the defective transcription and translation of this mutation. These mice not only recapitulated the phenotypes of this patient with growth retardation, pRTA, and ocular abnormalities, but also showed anemia, volume depletion, prerenal azotemia, and several organ abnormalities, culminating in dehydration and renal failure with early lethality before weaning. In isolated renal proximal tubules, both NBC1 activity and the rate of bicarbonate absorption were markedly reduced. Unexpectedly, there was no compensatory increase in mRNA of distal acid/base transporters. Sodium bicarbonate but not saline administration to these mutant mice markedly prolonged their survival, decreased their protein catabolism and attenuated organ abnormalities. The prolonged survival time uncovered the development of corneal opacities due to corneal edema. Thus, NBC1(W516X/W516X) mice with pRTA represent an animal model for metabolic acidosis and may be useful for testing therapeutic inhibition of NMD in vivo.

Topics: Acidosis; Acidosis, Renal Tubular; Age Factors; Aging; Analysis of Variance; Anemia; Animals; Aquaporin 2; Bicarbonates; Codon, Nonsense; Corneal Opacity; Disease Models, Animal; Female; Gene Knock-In Techniques; Genotype; Growth Disorders; Homozygote; Humans; Hydrogen-Ion Concentration; Kidney Tubules, Proximal; Mice; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Transgenic; Middle Aged; Phenotype; RNA, Messenger; Severity of Illness Index; Sodium Bicarbonate; Sodium-Bicarbonate Symporters; Transcription, Genetic

A 43- year- old woman on treatment for primary hypothyroidism presented with 1- day progressive weakness of all her limbs and history of similar episodes in the past. Clinical examination revealed grade 2 hyporeflexive weakness. Investigations revealed features of hypokalemia, metabolic acidosis, alkaline urine, and a fractional bicarbonate excretion of 3.5%, consistent with distal renal tubular acidosis. Antithyroid peroxidase and antithroglobulin antibodies were positive, suggesting an autoimmune basis for the pathogenesis of the functional tubular defect. Bicarbonate therapy resulted in a sustained clinical recovery.

Topics: Acidosis, Renal Tubular; Adult; Female; Humans; Hypokalemic Periodic Paralysis; Hypothyroidism; Kidney; Sodium Bicarbonate; Symporters

Incomplete distal renal tubular acidosis (idRTA) has recently been associated with osteoporosis and growth retardation, attributed to the mild persistent metabolic acidosis. We hypothesized a therapeutic benefit from bicarbonate therapy on growth parameters in children with idRTA. 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). Age- and gender-matched healthy subjects constituted the control group (n = 55). Incomplete dRTA was evaluated by ammonium chloride acidification. The baseline height SDS of -1.94 +/- 0.41 and -5.31 +/- 1.95 in the groups with idRTA and complete dRTA, respectively, were significantly lower than that of the controls. After a follow-up period of 24.7 +/- 8.3 months on sodium bicarbonate therapy, the idRTA patients had a 66% increase in height SDS compared with 26% and 3% increases in the patients with PUV with complete dRTA and without dRTA, respectively. At the end of follow-up, mean height SDS in the group with idRTA no longer remained significantly lower than that of the controls (P = 0.42). We concluded that bicarbonate therapy improves height SDS in idRTA. This issue needs further validation in larger studies.

Topics: Acidosis, Renal Tubular; Child; Child, Preschool; Female; Growth Disorders; Humans; Male; Prospective Studies; Sodium Bicarbonate

A 39-year old man had periodic paralysis due to hypokalaemia. Investigations led to the diagnosis of distal renal tubular acidosis (dRTA) and Southeast Asian ovalocytosis (SAO). Both can originate in mutations of the anion-exchanger 1 gene (AE1), which codes for band 3, the bicarbonate/chloride exchanger in both the red cell membrane and the basolateral membrane of the collecting tubule alpha-intercalated cell. The finding of diffuse osteosclerosis led to the suspicion of coexisting fluorosis.

Topics: Acidosis, Renal Tubular; Administration, Oral; Adult; Bone Density Conservation Agents; Calcium, Dietary; Elliptocytosis, Hereditary; Fluoride Poisoning; Fluorosis, Dental; Humans; Male; Osteosclerosis; Potassium Chloride; Quadriplegia; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Aged; Drug Therapy, Combination; Female; Gastrointestinal Agents; Humans; Hyperammonemia; Hyperparathyroidism, Primary; Lactulose; Potassium Chloride; Sodium Bicarbonate; Treatment Outcome; Vitamin D Deficiency

Analyze the clinical and evolutive particularities of complete primary distal renal tubular acidosis in children,. We studied retrospectively 11 cases enrolled in the pediatrics department of Sousse during 10 years period (1993-2002).. It is about 9 boys and 2 girls (sex-ratio = 45) aged 3 month to 5 years (mean age: 18 months). Diagnosis was suspected on clinical and biological data of presumption and confirmed by acidification test. Radiological investigation objectified a nephrocalcinosis in eight patients and urinary lithiasis in two other cases. Auditive exploration showed sensorineural deafness in three patients. The illness appears sporadic in two cases and autosomal recessive in nine other cases. After alkali treatment (sodium bicarbonate), evolution was globally favorable.

Topics: Acidosis, Renal Tubular; Child, Preschool; Female; Humans; Infant; Male; Retrospective Studies; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Adult; Animals; Humans; India; Insect Bites and Stings; Male; Potassium Chloride; Sodium Bicarbonate; Wasp Venoms; Wasps

In this report, we present a case of a child with distal renal tubular acidosis, severe failure to thrive and profound rickets, who was only 7.8 Kg when presented at 6 years of age. His response to treatment and his follow-up for four years is discussed. Although failure to thrive is a common finding in renal tubular acidosis but the physical and x-ray findings in our case were unique.

Topics: Acidosis, Renal Tubular; Child; Drug Therapy, Combination; Failure to Thrive; Follow-Up Studies; Humans; Male; Potassium Chloride; Rickets; Severity of Illness Index; Sodium Bicarbonate; Vitamin D; Vitamins

Topics: Acidosis, Renal Tubular; Animals; Azotemia; Biopsy; Blood Chemical Analysis; Electrolytes; Horse Diseases; Horses; Kidney; Male; Sodium Bicarbonate; Treatment Outcome; Urinalysis

Topics: Acidosis, Renal Tubular; Animals; Diagnosis, Differential; Female; Fluid Therapy; Horse Diseases; Horses; Serum; Sodium Bicarbonate; Treatment Outcome

A 9-year-old spayed female Labrador Retriever was evaluated for anorexia, lethargy, and vomiting of 5 days' duration. Laboratory abnormalities included azotemia, high liver enzyme activities, hyperchloremic metabolic acidosis, glucosuria, ketonuria, proteinuria, and aminoaciduria. These laboratory abnormalities were diagnostic of proximal renal tubular acidosis and Fanconi syndrome. Results of initial and convalescent serologic tests for leptospirosis were negative. The dog was treated with amoxicillin, sodium bicarbonate, and potassium citrate at discharge. Repeated evaluations revealed resolution of the acidosis, azotemia, proteinuria, glucosuria, ketonuria, and high liver enzyme activities. Alkali administration was gradually discontinued, and the dog was clinically normal 8 months after discharge. The dog's clinical condition appeared to have been transient in nature, a phenomenon that is rarely seen in human or veterinary medicine.

Topics: Acidosis, Renal Tubular; Amoxicillin; Animals; Dog Diseases; Dogs; Fanconi Syndrome; Female; Liver; Potassium Citrate; Sodium Bicarbonate; Treatment Outcome

Topics: Acidosis, Renal Tubular; Child; Citric Acid; Diagnosis, Differential; Humans; Kidney Diseases, Cystic; Kidney Medulla; Magnetic Resonance Imaging; Male; Nephrocalcinosis; Osteoporosis; Radiography; Sodium Bicarbonate; Treatment Outcome; X-Rays

FK506 is an immunosuppressant that is thought to be less nephrotoxic than cyclosporine A. However, complications due to renal tubular acidosis (RTA) have recently been reported. We report a case of RTA secondary to FK506 administration in liver transplantation. A 6-month-old girl was treated with FK506 after undergoing living donor liver transplantation for fulminant hepatitis. On postoperative day 17, she demonstrated hyperkalaemia and metabolic acidosis; she was diagnosed to have hyperkalaemic distal RTA with aldosterone deficiency (type IV). Intravenous sodium bicarbonate and furosemide, and intrarectal calcium polystyrenesulfonate were administered to correct the acidosis and promote potassium secretion. Thereafter, the FK506 concentration in whole blood gradually decreased, and the hyperkalaemia and metabolic acidosis following RTA improved. RTA is one type of nephrotoxicity induced by FK506, and it is reversible in mild cases when appropriately treated. The mechanism of RTA induced by FK506 has not yet been clearly elucidated. Surgeons and physicians should therefore be aware of the potential for RTA to occur with FK506 after any organ transplantation. The treatment for acidosis and hyperkalaemia should be started as soon as RTA is diagnosed, and the dosage of FK506 should also be reduced if possible.

Topics: Acidosis, Renal Tubular; Drug Therapy, Combination; Female; Follow-Up Studies; Furosemide; Humans; Infant; Liver Transplantation; Living Donors; Risk Assessment; Severity of Illness Index; Sodium Bicarbonate; Tacrolimus; Transplantation Immunology; Treatment Outcome

Topics: Acidosis, Renal Tubular; Humans; Hypercalcemia; Infant; Male; Nephrocalcinosis; Parathyroid Hormone; Sodium Bicarbonate

We present a case of a 4.5 years old boy suffering from hypercalciuria caused by a not diagnosed distal renal tubular acidosis. In the age of 2.5 years, after a banal infection the routine analysis showed a presence of numerous calcium phosphates in urine. Other diagnostic procedures showed: hypercalciuria, hyperphosphaturia, rather high calcemia and high values of UCa/cr and UPO4/cr ratios. HCO3 in serum 21.2 mmol/l, pH of urine 7.0. Kidneys and urinary tract-usg normal. These results induced the family doctor to make the diagnosis: idiopathic renal hypercalciuria. He advised the therapy with hydrochlorothiazide and limitations of calcium and vitamins D3 oral supply. This decision caused an illusory positive effect: decreased the UCa/cr ratio (to 0.96 mmol/mmol) without any reduction of calcemia. After a period of 12 months the UCa/cr ratio increased up to 1.31 and calcification of renal pyramids appeared. We diagnosed the distal renal tubular acidosis (some medical informations suggested the essential distal renal tubular acidosis) and osteopenia (DXA BMD L1-L4 below -1 s.d.). The therapy with NaHCO3 (about 2.6 mmol/kg) normalized the levels of HCO3a and calcium in serum, decreased the UCa/cr ratio to values 0.09-0.16 mmol/mmol.

Topics: Acidosis, Renal Tubular; Bone Diseases, Metabolic; Calcinosis; Calcium; Child, Preschool; Humans; Kidney Diseases; Male; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Female; Humans; Liver Cirrhosis, Biliary; Middle Aged; Osteomalacia; Sodium Bicarbonate; Treatment Outcome

Metabolic acidosis is almost invariably a consequence of advanced renal failure, although its severity can vary widely. To evaluate the determinants of the severity of metabolic acidosis, with special interest in determining if there is any difference in the prevalence and severity of metabolic acidosis between patients with and without diabetes, 113 predialysis patients with renal failure were studied. Criteria for inclusion onto the study were: creatinine clearance (Ccr)/1.73 m2 less than 30 mL/min, no alkali therapy within the previous 30 days, and the absence of respiratory diseases. Forty-eight patients had diabetes (33 patients with diabetic nephropathy). The following data were analyzed: demographics; cause of renal failure; hematocrit; serum urea, creatinine, uric acid, albumin, glucose, hemoglobin A1c, bicarbonate, sodium, potassium, chloride, calcium, phosphorus, and alkaline phosphatase levels; anion gap; urinary protein excretion; Ccr/1.73 m2; half of the sum of creatinine and urea clearances (Ccr-Cu); protein-equivalent nitrogen appearance (PNA); and whether the patients received diuretics (75 patients), angiotensin-converting enzyme inhibitors (54 patients), and/or calcium channel blockers (55 patients). After the exclusion of eight patients because of hypochloremia (three patients with and five patients without diabetes), mean serum bicarbonate levels were significantly greater in patients with diabetes than in the rest of the patients (20.7 +/- 2.3 v 18.2 +/- 2. 3 mmol/L; P = 0.0001). The mean anion gap (mmol/L) was also significantly less in patients with than without diabetes (19.70 +/- 3.65 v 22.35 +/- 3.64; P = 0.003). Eleven of 105 patients had serum bicarbonate levels of 23 mmol/L or greater (9 patients with and 2 patients without diabetes). Pure elevated anion gap followed by mixed (high anion gap and hyperchloremia) were the most common types of metabolic acidosis observed in both groups. There were no differences in PNA, diuretic treatment, or vomiting history between patients with and without diabetes. By multiple logistic regression analysis, the best determinants for a serum bicarbonate level greater than 19 mmol/L were: the diagnosis of diabetic nephropathy (odds ratio, 0.107; P = 0.0002), Ccr-Cu (odds ratio, 0.824; P = 0. 014), and age (odds ratio, 0.966; P = 0.046). In conclusion, patients with diabetes with advanced renal failure showed a less severe metabolic acidosis, which cannot be explained by gastrointestinal hydrogen

Topics: Acidosis, Renal Tubular; Creatinine; Diabetes Complications; Diabetes Mellitus; Diabetic Nephropathies; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged; Odds Ratio; Regression Analysis; Sodium Bicarbonate; Urea

Southeast Asian ovalocytosis (SAO) is the best-documented disease in which mutation in the anion exchanger-1 (AE1) causes decreased anion (chloride [Cl-]/bicarbonate [HCO3-]) transport. Because AE1 is also found in the basolateral membrane of type A intercalated cells of the kidney, distal renal tubular acidosis (dRTA) might develop if the function of AE1 is critical for the net excretion of acid. Studies were performed in a 33-year-old woman with SAO who presented with proximal muscle weakness, hypokalemia (potassium, 2.7 mmol/L), a normal anion gap type of metabolic acidosis (venous plasma pH, 7. 32; bicarbonate, 17 mmol/L; anion gap, 11 mEq/L), and a low rate of ammonium (NH4+) excretion in the face of metabolic acidosis (26 micromol/min). However, the capacity to produce NH4+ did not appear to be low because during a furosemide-induced diuresis, NH4+ excretion increased almost threefold to a near-normal value (75 micromol/L/min). Nevertheless, her minimum urine pH (6.3) did not decrease appreciably with this diuresis. The basis of the renal acidification defect was most likely a low distal H+ secretion rate, the result of an alkalinized type A intercalated cell in the distal nephron. Unexpectedly, when her urine pH increased to 7.7 after sodium bicarbonate administration, her urine minus blood carbon dioxide tension difference (U-B Pco2) was 27 mm Hg. We speculate that the increase in U-B Pco2 might arise from a misdirection of AE1 to the apical membrane of type A intercalated cells.

Topics: Acidosis, Renal Tubular; Adult; Antiporters; Carbon Dioxide; Diuretics; Elliptocytosis, Hereditary; Female; Furosemide; Humans; Hydrogen-Ion Concentration; Quaternary Ammonium Compounds; Sodium Bicarbonate

We present a 15-year-old girl with distal renal tubular acidosis (dRTA) appearing in what is probably a very early stage of primary Sjögren's syndrome. On the basis of tests evaluating renal handling of H+, we attempt to explain the mechanism of the urine acidification disorder. The inability to decrease urinary pH during systemic acidosis, together with the normal increase of urinary carbon dioxide partial pressure (pCO2) values after sodium bicarbonate and neutral phosphate loading, suggest a gradient-type dRTA. The inability to lower urinary pH in response to furosemide, accompanied by markedly increased urinary excretion of NH4, HCO3, Na, and K, points to a collecting tubule permeability disorder with bicarbonate leak to the tubular lumen. This patient had never been exposed to amphotericin B. To our knowledge, immune-related dRTA as a result of a gradient defect with bicarbonate leak into the tubular lumen has not been described.

Topics: Acidosis, Renal Tubular; Adolescent; Autoantibodies; Autoimmune Diseases; Bicarbonates; Carbon Dioxide; Diuretics; Female; Furosemide; Humans; Hydrogen-Ion Concentration; Kidney Tubules, Collecting; Permeability; Phosphates; Sjogren's Syndrome; Sodium Bicarbonate

Distal renal tubular acidosis has been reported in several diseases associated with hypergammaglobulinemia, particularly Sjögren's syndrome. Since HIV infection is now a common cause of hypergammaglobulinemia, we evaluated renal acidification under basal and dynamic conditions in 8 asymptomatic HIV-seropositive subjects. Basal acid-base status was normal in all except 1 patient who had respiratory acidosis subsequent to recent pneumonia. Acid-loading tests showed normal acid excretion except for 1 patient who had low acid excretion attributed to non-ingestion of the ammonium chloride capsules. Bicarbonate-loading tests showed normal distal acidification indexes in 7 patients. The only patient with a low acidification defect was diagnosed 2 weeks later as having tuberculosis. Our results suggest that hypergammaglobulinemia per se is not a sufficient condition to induce renal tubular acidosis.

Topics: Acidosis, Renal Tubular; Adult; Evaluation Studies as Topic; Female; gamma-Globulins; HIV Infections; Humans; Hydrogen-Ion Concentration; Hypergammaglobulinemia; Male; Sodium Bicarbonate

The aim of this study was to investigate subclinical abnormalities in renal function suggestive of incomplete distal renal tubular acidosis (DRTA) in patients with rheumatoid arthritis (RA), using the gradient between PCO2 in urine and blood (U-B PCO2 gradient), which is a simple and sensitive test. We prospectively selected 45 patients in three groups (G). G 1 (n = 15p), with RA, mean age 44 years and mean disease duration 6.5 years. G2 (n = 10 p), with RA and Sjögren's syndrome (SS), mean age 47 years and mean disease duration 6.6 years. G 3 (n = 20) healthy volunteers, no history neither renal nor rheumatic diseases, mean age 37 years. Patients in G1 and G2 had no history of concurrent disease affecting renal parenchyma, their acid-base status and renal function were normal (Creatinine clearance above 70 ml/min/1.73m2). All patients received NSAIDs but none gold salts and/or D-Penicillamine. The ability to acidify the urine was evaluated in all cases by U-B PCO2 gradient, after a 500 ml NaHCO3 continuous infusion 1 molar solution through a peripheral vein. U-B PCO2 lower than 30 was considered pathological and diagnostic for DRTA. The urinary specimen for pH and PCO2 were kept under mineral oil and processed within 5 minutes of excretion. The blood samples for PCO2 were obtained from a peripheral vein and measured after obtaining a urinary pH 7.8 or above, pH and PCO2 were measured with a BMS 3 MK2 Radiometer, Copenhagen Denmark electrode and analizer. The U-BPCO2 results were (mean 2 sd): G1 = 47 +/- 26; G2 = 49.8 +/- 8.4; G3 = 52.5 +/- 12.2. There were no statistical differences among the three groups (F = 1.228727). In the G1, a single patient presented U-B PCO2 lower than 30 (U-B PCO2 = 5), having a long active disease at the evaluation time. In G2 and G3 no gradient alterations were recorded. We conclude, in spite of the fact that U-B PCO2 gradient is a very useful and sensitive tool for detecting dRTA, that there was no correlation between incomplete type dRTA and RA or between incomplete dRTA and RA associated to SS. In addition, no association was found between NSAID intake and dRTA.

Topics: Acidosis, Renal Tubular; Adult; Aged; Arthritis, Rheumatoid; Carbon Dioxide; Female; Humans; Hydrogen-Ion Concentration; Kidney; Male; Middle Aged; Prospective Studies; Sodium Bicarbonate

We measured sodium-proton (Na+/H+) exchange in lymphocytes and platelets of a 46-year-old woman with the adult Fanconi syndrome before, during, and after treatment with NaHCO3. Kappa light chains in her urine and unique but rarely observed crystalline structures confirmed the presence of light-chain nephropathy. Her glomerular filtration rate was only moderately impaired at 72 ml/min. NaHCO3 at 1, 3, and 5 mmol/kg/day for 5 days increased her serum HCO3 and pH from 17 to 21 mmol/l and 7.28 to 7.39 respectively. Plasma renin and aldosterone values were decreased by NaHCO3. Na+/H+ exchange (delta Hi/min) was measured with the fluorescent marker BCECF after acidification of lymphocytes and platelets with sodium propionate at five (10-50 mM) doses. Na+/H+ exchange was accelerated in this patient compared to normal controls. NaHCO3 treatment significantly decreased Na+/H+ exchange in lymphocytes, but not in platelets. These findings suggest that Na+/H+ exchange can be influenced by NaHCO3 ingestion at doses that only modestly affect systemic pH. Since Na+/H+ exchange is involved in stimulus response coupling, cell growth regulation, cell differentiation, and perhaps the progression of nephrosclerosis, these observations may have clinical relevance.

Topics: Acidosis, Renal Tubular; Blood Platelets; Fanconi Syndrome; Female; Glomerular Filtration Rate; Humans; Immunoglobulin kappa-Chains; Kidney Glomerulus; Lymphocytes; Middle Aged; Sodium; Sodium Bicarbonate; Sodium-Hydrogen Exchangers

Severe hypokalemia is an uncommon cause of rhabdomyolysis. We describe a patient, 28-year-old woman, with distal renal tubular acidosis (DRTA) who developed severe hypokalemia and rhabdomyolysis. Muscle biopsy shows focal muscular necrosis mainly in type II muscle fibers and mild macrophagic reaction. After correcting the acidosis with oral administration of alkalinizing salts, clinical and laboratory improvement was seen. This clearly establish a causal relationship between the positive acid balance, hypokalemia and the muscular manifestation in DRTA.

Topics: Acidosis, Renal Tubular; Adult; Diagnosis, Differential; Female; Humans; Hypokalemia; Potassium Chloride; Rhabdomyolysis; Sodium Bicarbonate

The pathogenesis of renal potassium wasting and hypokalemia in classic renal tubular acidosis (type 1 RTA) remains uncertain. The prevailing theory is that K(+)-Na+ exchange is stimulated due to an inability of the distal tubule to establish a normal steep lumen-peritubular H+ gradient. We encountered a 42-year-old woman with type 1 RTA associated with Sjögren's syndrome, in whom renal potassium wasting and hypokalemia persisted despite sustained correction of systemic acidosis with alkali therapy and increased intake of potassium. In addition, plasma renin activity was markedly increased and the serum aldosterone level was upper-normal despite the hypokalemia. Increased intake of sodium resulted in suppression on the serum aldosterone and correction of renal potassium wasting and hypokalemia. This case shows that secondary hyperaldosteronism, possibly due to an impairment of sodium conservation in the distal tubule, may contribute to the loss of potassium from the distal tubule even after the correction of acidosis.

Topics: Acidosis, Renal Tubular; Adult; Aldosterone; Ammonium Chloride; Bicarbonates; Blood Gas Analysis; Female; Humans; Hydrogen-Ion Concentration; Hyperaldosteronism; Hypokalemia; Kidney; Potassium; Potassium, Dietary; Sjogren's Syndrome; Sodium; Sodium Bicarbonate; Sodium, Dietary; Urine; Water-Electrolyte Balance

Renal tubular acidification function was studied in 12 patients treated with cyclosporine (Cy) for idiopathic uveitis (IU) and in 5 patients with IU not treated with Cy. After intravenous bicarbonate loading fractional bicarbonate excretion was similar in both groups indicating normal proximal tubular acidification function. Plasma renin activity, plasma aldosterone and transtubular potassium gradient were similar in both groups. Distal hydrogen ion secretion evaluated by the ability to increase urine-blood (U-B) pCO2 in a highly alkaline urine was impaired in Cy-treated patients (31.8 +/- 3.2 mm Hg) as compared to controls (47.9 +/- 0.5 mm Hg) (p < 0.005). We conclude that Cy therapy is associated with a distal acidification defect with a low U-B pCO2 gradient during sodium bicarbonate loading. Because none of our Cy-treated patients spontaneously developed over metabolic acidosis one could classify them as having an incomplete form of distal tubular acidosis.

Topics: Acidosis, Renal Tubular; Adult; Aged; Aldosterone; Bicarbonates; Carbon Dioxide; Cyclosporine; Electrolytes; Female; Humans; Hydrogen-Ion Concentration; Kidney Tubules; Male; Middle Aged; Osmolar Concentration; Potassium; Sodium; Sodium Bicarbonate; Urine; Uveitis

Urine-blood (U-B)Pco2 difference in children is usually assessed following urine alkalinization with oral sodium bicarbonate (NaHCO3). Since oral NaHCO3 is often poorly tolerated by children, we compared oral acetazolamide with oral NaHCO3 in a study of (U-B)Pco2. In the first phase of the study 14 children and adolescents aged 11.1 +/- 3.7 years (mean +/- SD) were studied. Eight participants had normal kidney function and 6 had disturbed distal acidification capacity. Each child was studied twice, once with oral NaHCO3 (2.5 mEq/kg) and once with acetazolamide (17 +/- 2 mg/kg). All studies were performed according to the standard protocol. Acetazolamide administration resulted in a lower blood pH than NaHCO3 (7.30 +/- 0.03 vs 7.38 +/- 0.06, P less than 0.001) and a lower serum bicarbonate (HCO3-) concentration (25.1 +/- 2.2 mEq/l vs 27.5 +/- 2.1 mEq/l, P less than 0.025). Acetazolamide also resulted in a higher urine Pco2 (81.9 +/- 26.2 mm Hg vs 71.6 +/- 18.2 mm Hg) than NaHCO3 (P less than 0.025). No significant differences between acetazolamide and NaHCO3 were observed with respect to their effects on urinary pH and HCO3- concentration, plasma Pco2 and (U-B)Pco2. Good linear correlations were found between the effects of acetazolamide and NaHCO3 on urine Pco2 (r = 0.878, P less than 0.001), and on (U-B)Pco2 (r = 0.795, P less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acetazolamide; Acidosis, Renal Tubular; Administration, Oral; Adolescent; Bicarbonates; Biological Transport, Active; Blood Gas Analysis; Carbon Dioxide; Child; Child, Preschool; Consumer Behavior; Female; Humans; Kidney; Male; Reproducibility of Results; Sodium; Sodium Bicarbonate

A patient with deficient activity of cytochrome c oxidase in muscle presented at 1 year of age with extreme failure to thrive. He was found to have dicarboxylic aciduria, renal tubular acidosis, and deficiency of carnitine. Treatment with sodium bicarbonate, riboflavin, and carnitine led to considerable improvement in growth and a significant reduction in the dicarboxylic aciduria.

Topics: Acidosis, Renal Tubular; Amino Acids, Dicarboxylic; Bicarbonates; Carnitine; Cytochrome-c Oxidase Deficiency; Failure to Thrive; Humans; Infant; Male; Muscles; Riboflavin; Sodium; Sodium Bicarbonate

Intravenous sodium bicarbonate (NaHCO3) infusion test was performed in 26 patients with chronic glomerulonephritis (CGN) and 16 with distal renal tubular acidosis (dRTA) in order to evaluate urinary acidifying capacity in chronic renal diseases. Comparative studies with glomerular filtration were planned, so that the patients with CGN were divided by creatinine clearance (Ccr) into 3 groups (G-I greater than or equal to 70, 30 less than or equal to G-II less than 70, G-III less than 30 ml/min). Proximal tubular bicarbonate (HCO3) reabsorption rate increased in CGN as Ccr decreased. Urine to blood carbon dioxide tension gradient (U-B PCO2) was above 30 mmHg in controls and below 20 mmHg in dRTA. In patients with CGN, urine HCO3 concentration (UHCO3) did not increase during NaHCO3 loading as Ccr decreased. However, U-B PCO2 rose above 20 mmHg, when UHCO3 was above 50 ml/min. Fishberg concentrating test was also performed in 15 patients with CGN and 6 with dRTA so that the relationship between urinary concentrating ability and urine acidification might be evaluated. While both functions were decreased in dRTA, U-B PCO2 in alkaline urine remained above 20 mmHg in CGN associated with moderate renal dysfunction (Ccr greater than or equal to 30 ml/min) despite decreased maximal urine osmolality. Intravenous furosemide (FM) injection test was carried out in 8 patients with chronic renal failure (CRF) and 3 with dRTA. Minimal urine pH fell below 5.5 and net acid excretion (NAE) increased in controls, whereas these responses were not seen in dRTA. In CRF, urine pH generally decreased below 5.5 and those who had a similar response to FM as dRTA, seemed to have more severe disturbance of the distal acidification. In conclusion, U-B PCO2 in alkaline urine and lowered urine pH in FM loading appeared to be a useful index of distal tubular acid excretion in patients with renal dysfunction. In CGN with moderate renal dysfunction (Ccr greater than or equal to 30 ml/min), urinary acidifying capacity remained normal in comparison with decreased urine concentrating ability.

Topics: Acidosis, Renal Tubular; Bicarbonates; Chronic Disease; Furosemide; Glomerular Filtration Rate; Glomerulonephritis; Humans; Hydrogen-Ion Concentration; Kidney Concentrating Ability; Kidney Glomerulus; Sodium; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Bartter Syndrome; Bicarbonates; Fanconi Syndrome; Humans; Hypophosphatemia, Familial; Potassium Chloride; Sodium; Sodium Bicarbonate; Vitamin D

Three siblings with neonatal familial hyperparathyroidism diagnosed at age 4 months, 2 months, and 5 days, respectively, were treated. Hypercalciuria, nephrocalcinosis, and renal tubular acidosis were present in each child. In all three, there were higher responses of serum parathyroid hormone to serum calcium and higher elevation of serum calcium with oral calcium loading. The metabolism of vitamin D and calcitonin seemed to be intact. Hypercalcemia associated with the abnormal response of parathyroid hormone secretion disappeared when the children passed the age of approximately 2 years, although renal tubular acidosis and nephrocalcinosis remained. An autosomal recessive inheritance seems likely.

Topics: Acidosis, Renal Tubular; Bicarbonates; Calcinosis; Calcium; Calcium, Dietary; Chromosome Aberrations; Chromosome Disorders; Female; Humans; Hypercalcemia; Hyperparathyroidism; Infant, Newborn; Kidney Diseases; Male; Parathyroid Hormone; Sodium; Sodium Bicarbonate; Vitamin D

Chronic renal failure is associated with increased protein turnover, and recent evidence suggests that this may be due to the accompanying acidosis. Patients with stable chronic renal failure maintained on protein restriction were given sodium bicarbonate supplementation for 8 weeks. This resulted in improvements in acid-base status, plasma urea and uric acid levels, independent of changes in glomerular filtration rate. Acidosis is a potentially reversible toxic effect of chronic renal failure and its correction produces sustained metabolic benefit.

Topics: Acidosis, Renal Tubular; Adult; Bicarbonates; Blood Glucose; Blood Proteins; Female; Humans; Insulin; Kidney Failure, Chronic; Male; Middle Aged; Sodium; Sodium Bicarbonate; Urea; Uremia

Topics: Acidosis, Renal Tubular; Adult; Ammonium Chloride; Bicarbonates; Female; Furosemide; Humans; Kidney; Kidney Function Tests; Male; Sodium; Sodium Bicarbonate; Ultrasonography

Topics: Acidosis, Renal Tubular; Adult; Bicarbonates; Humans; Kidney Tubules, Distal; Male; Sodium; Sodium Bicarbonate; Toluene; Volatilization

Topics: Acidosis, Renal Tubular; Ammonium Chloride; Bicarbonates; Calcium; Humans; Kidney Calculi; Recurrence; Sodium; Sodium Bicarbonate

Two horses were admitted separately for evaluation and treatment of profound hyperchloremic metabolic acidosis without azotemia. One, an 11-year-old Quarter Horse mare, had been depressed and ataxic for 2 days. The other, a 2-year-old Quarter Horse colt, had a 6-week history of depression, anorexia, and weight loss. Both horses responded to fluid and electrolyte therapy, but required daily oral administration of sodium bicarbonate for maintenance. In each case, the diagnosis was renal tubular acidosis.

Topics: Acidosis, Renal Tubular; Animals; Bicarbonates; Combined Modality Therapy; Female; Fluid Therapy; Horse Diseases; Horses; Male; Pregnancy; Pregnancy Complications; Sodium; Sodium Bicarbonate

We prospectively evaluated 30 patients who presented with active systemic lupus erythematosus (SLE) for the presence of tubular abnormalities. All patients fulfilled the American Rheumatology Association criteria for SLE. When appropriate, a renal biopsy was performed. Of the 30 patients studied, 12 had no abnormal tubular study results, whereas 18 patients had some form of defect in the handling of potassium, sodium, or hydrogen ions. Eight patients had distal renal tubular acidosis (dRTA) due to an isolated proton secretory defect. Five had dRTA of the gradient or acid back-leak type. Two had an unresponsive voltage-dependent form of dRTA; one had a responsive voltage-dependent form of dRTA. One individual had hyporeninemic hypoaldosteronism and one had dRTA plus hypoaldosteronism. Clinically, patients with the abnormal tubular study results more often presented with nephritis or nephrotic sediment, peripheral edema, or anemia. Renal biopsies failed to demonstrate any difference in glomerular histologic findings and calculated activity, chronicity, or interstitial indexes. We conclude that SLE may be associated with a variety of tubular defects.

Topics: Acidosis, Renal Tubular; Adult; Ammonium Chloride; Bicarbonates; Female; Humans; Hyperkalemia; Kidney; Kidney Tubules; Lupus Nephritis; Male; Prospective Studies; Sodium; Sodium Bicarbonate; Sulfates

Severe renal tubular acidosis associated with massive osteomalacia is described in a patient with Crohn's disease. To our knowledge this association has not been previously recognized. The possible role of renal tubular acidosis in this patient's osteomalacia is discussed and the factors that could be involved in renal tubular acidosis in the context of Crohn's disease are analyzed.

Topics: Acidosis, Renal Tubular; Administration, Oral; Bicarbonates; Crohn Disease; Gait; Humans; Infusions, Parenteral; Male; Middle Aged; Osteomalacia; Sodium; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Age Factors; Bicarbonates; Child; Child, Preschool; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Sodium; Sodium Bicarbonate

A 32-year old woman developed severe quadriparesis, hypokalemia, and distal renal tubular acidosis following paint sniffing for one week. A review of literature indicated that a spectrum of renal diseases may develop in association with inhalation of toluene-containing substances. Toluene inhalation should be considered in the differential diagnosis in any young patient who presents with an unexplained renal disorder.

Topics: Acidosis, Renal Tubular; Adhesives; Adolescent; Adult; Bicarbonates; Calcium Gluconate; Female; Humans; Hypocalcemia; Male; Potassium Chloride; Sodium; Sodium Bicarbonate; Substance-Related Disorders; Tetany; Toluene

Topics: Acidosis, Renal Tubular; Bicarbonates; Child; Female; Hearing Loss; Hearing Loss, Bilateral; Humans; Sodium; Sodium Bicarbonate

In steady state, the acidosis in the majority of 17 uremic patients was characterized by a persistent bicarbonaturia (FEHCO3 ranging between 0% and 17.65%). An NH4Cl loading test in 17 patients revealed two distinct groups: group A (n = 11) with complete disappearance of the urinary bicarbonate loss and a mean UpH of 5.39 +/- 0.10 at a PHCO3 level of 13.3 +/- 0.5 mEq/L; and group B (n = 6) with urinary acidification disturbances with a persistent FEHCO3 ranging between 1.06% and 3.15% and a mean UpH of 6.53 +/- 0.06 at a PHCO3 level of 13.5 +/- 0.7 mEq/L. Between the two groups, there were no differences in CCr, plasma Na, K, Cl, Ca, PO4, PCO2, and aldosterone levels. Calculation of the THCO3/TNa reabsorption ratio over a wide range of PHCO3 levels revealed no differences between the two groups. The mean levels of circulating PTH were significantly higher in group B compared with group A (40.1 +/- 10.8 mU/dL v 19.3 +/- 4.4 mU/dL; P less than .05), and the spontaneous steady-state FENa was more pronounced in group B than in group A (12.1% +/- 1.5% v 4.9% +/- 0.7%; P less than .05). Four patients from group B with a well-documented salt-losing nephropathy (FENa ranging from 10.20% to 15.10%) were submitted to a progressive dietary salt restriction over several weeks. At this stage, the four patients no longer had bicarbonaturia, and the urinary pH decreased to levels between 5.15 and 5.65 during NH4Cl-induced acidosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acidosis, Renal Tubular; Adult; Aged; Ammonium Chloride; Bicarbonates; Diet, Sodium-Restricted; Homeostasis; Humans; Hydrogen-Ion Concentration; Kidney Failure, Chronic; Middle Aged; Parathyroid Hormone; Sodium; Sodium Bicarbonate

Basic and clinical aspects of acid-base physiology and acidoses are reviewed in this paper. Therapeutic principles on the treatment of metabolic and respiratory acidosis are discussed in detail. As far as buffer substances are necessary for antiacidotic therapy, sodium bicarbonate seems to be the agent of choice today. Sodium bicarbonate has a better extra- and intracellular effect than THAM, and should therefore be preferred in every day practice.

Topics: Acid-Base Equilibrium; Acidosis; Acidosis, Renal Tubular; Acidosis, Respiratory; Bicarbonates; Combined Modality Therapy; Critical Care; Humans; Hydrogen-Ion Concentration; Kidney; Lung; Postoperative Complications; Sodium; Sodium Bicarbonate

Topics: Acidosis, Renal Tubular; Aged; Bicarbonates; Calcium Gluconate; Female; Humans; Hyperkalemia; Injections, Intravenous; Insulin; Ion Exchange Resins; Quadriplegia; Sodium; Sodium Bicarbonate; Spironolactone

Oral sodium bicarbonate is used to treat metabolic acidosis in patients with renal tubular acidosis. Since infants and young children are unable to swallow tablets, those affected must ingest sodium bicarbonate in a powder or liquid form. Pharmacy-weighed sodium bicarbonate is expensive and inconvenient to obtain; some pharmacists are reluctant to provide it. We determined that the sodium bicarbonate contained in 8-oz boxes of Arm and Hammer Baking Soda was sufficiently constant in weight that, dissolved in water to a given volume, it yielded a quantitatively acceptable therapeutic solution of sodium bicarbonate at a cost of approximately 3 percent of that of pharmacy-weighed sodium bicarbonate. Grocery store baking soda can be a safe, economical, and convenient source of sodium bicarbonate for the treatment of chronic metabolic acidosis in infants and young children.

Topics: Acidosis, Renal Tubular; Bicarbonates; Child; Child, Preschool; Humans; Infant; Molecular Weight; Nonprescription Drugs; Sodium Bicarbonate