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PubMed2004

Possible association between cell membrane band 3 impairment function and renal tubular acidosis (liver diseases, malignancies and adverse drug reactions).

Renal tubular acidosis (RTA) more frequently develops in case of chronic diseases of inflammatory-immunological origin. RTA is well known to be associated with chronic liver disease (CLD), with nephrolithiasis, common cases of RTA occur among cancer patients. Abnormalities in the expression or function of band 3 in cell membrane may play a role in the pathogenesis of RTA. Cl-/HCO3- anion exchanger (AE2) is an isoform of band 3 protein, which is expressed in cell membranes of organs such as liver cells and kidney endothelium. There are reports on downregulated AE2 immunoreactivity in the liver of patients with chronic liver diseases and in the kidney tubular tissue of patients with RTA. The proteolytic damage of cell membrane band 3 in tissues could be related to inflammatory-immunological processes. Another important factor able to disturb the band 3 function is medicinal products used in the treatment of certain pathologies. The active substance of a drug itself may have a direct effect on this protein or trigger a pathological process. In such cases ADR can take place and may be evaluated as such. Acid-base disturbances, notably metabolic acidosis, are a serious complication of drug treatment. Reduced AE2 expression or its changed activity (congenital or acquired) could be related with alterations of intracellular pH. This could lead to antigenic changes and autoimmunity. The derangement of band 3 function in organ cell membrane could act as a factor which creates an "acidotic environment" for organ cells. Such circumstances could be the reason for unsuccessful treatment or determine resistance of tumor treatment. The understanding of the mechanisms of RTA development, early diagnostics, and knowledge of the drugs that can cause RTA, are of particular practical significance.

Acid-Base Imbalance↗
PubMed2005

Comparison of growth in primary Fanconi syndrome and proximal renal tubular acidosis.

To compare the difference between primary proximal renal tubular acidosis (PRTA) and Fanconi syndrome (FS), and to find out possible risk factors for growth retardation, we studied the long-term growth, clinical, laboratory, and radiological findings associated with the treatment of six children with primary FS and 15 children with PRTA. The ages of the children with FS were much older than those with PRTA at initial diagnosis (7.03+/-3.82 vs. 1.63+/-1.56 years). The height standard deviation score (SDS) at the start of treatment was significantly lower in FS than in PRTA. Catch-up growth was noted in PRTA at the end of follow-up (initial height SDS -2.13+/-1.10 vs. last height SDS -1.33+/-1.43, P=0.023 by paired t-test), whereas apparent linear growth impairment was found in FS in terms of overall growth velocity index (82.70+/-8.37%) and height SDS (initial -3.25+/-0.95 vs. last -3.15+/-0.31, P=0.791). There was also a higher rate of rickets occurrence in FS (3/6 vs. 0/15 in PRTA). Hypophosphatemia during the follow-up period was more frequent for FS than PRTA (69.2+/-26.1% vs. 7.0+/-25.8%, P<0.001), whereas metabolic acidosis (blood HCO(3)<20 mmol/l) was less efficiently corrected in PRTA (49.1+/-20.5% vs. 25.2+/-21.6% in FS, P=0.028). Moreover, the height Delta SDS correlated well with the mean serum P level during the treatment period in these patients (R=0.528, P=0.014 for all children; R=0.917, P=0.01 for FS patients). Our data suggest that metabolic acidosis may not be the sole factor causing growth impairment in FS. Correction of metabolic acidosis may indeed improve growth in PRTA but not in FS. This study indicates that factors other than metabolic acidosis, such as phosphate depletion and delayed diagnosis/treatment, should be considered to be important causes of growth retardation in FS.

Acidosis↗
PubMed1981

Renal tubular acidosis in primary biliary cirrhosis.

The relationship between renal tubular acidosis (RTA) and copper metabolism has been investigated in a group of 18 patients with primary biliary cirrhosis. RTA, considered when urinary pH remained above 5.4 after an oral load of ammonium chloride of 0.1 g/kg body wt, was found in 6 patients (33%). Plasma copper concentration (PCu) and urinary copper excretion (UCuV) were significantly higher in patients with RTA (PCu = 182.2 micrograms/dl, UCuV = 536.8 micrograms/24 h) than in those without (PCu = 134.2; UCuV = 170.3). Plasma copper concentration and urinary copper excretion correlated with minimal urinary pH achieved after the ammonium chloride load. A higher degree of cholestasis was present in patients with RTA than in those without, and a linear correlation was observed between PCu and UCuV and serum bilirubin. It is concluded that the increased UCuV is related to the cholestasis in primary biliary cirrhosis and that the RTA might be caused by the deposition of copper in the distal renal tubule.

Acidosis, Renal Tubular↗
PubMed1992

Transient neonatal distal renal tubular acidosis with secondary hyperparathyroidism.

We describe a neonate with distal renal tubular acidosis with secondary hyperparathyroidism manifesting as hyperchloraemia, hypercalcaemia, elevated serum parathyroid hormone (PTH) and life-threatening metabolic acidosis. He exhibited general weakness, tachypnoea, dry skin and weight loss. Urinary excretion of titratable acid and ammonium was decreased. Daily alkali (2.5 mEq/kg body weight) was required to maintain a normal plasma bicarbonate (HCO3-). With alkali therapy, the fractional excretion of HCO3- was below 5%. Serum calcium and PTH were restored to normal promptly on initiation of alkali therapy. After 5 months of alkali therapy, normal growth and urine acidifying ability were restored and alkali therapy was discontinued. The acidification defect in this patient was transient. We consider this patient to be consistent with Lightwood's syndrome of "transient infantile renal tubular acidosis".

Acidosis, Renal Tubular↗
PubMed1979

The acidification defect in the syndrome of renal tubular acidosis with nerve deafness.

An 8-year-old boy with renal tubular acidosis and nerve deafness, has been followed for seven years. Repeated studies of his renal acidification defect showed that until the age of six years the tubular defect was mixed, proximal and distal (type 1,2 hybrid). After that age the defect of proximal acidification disappeared and the patient only presented a distal renal tubular acidosis type 1. When this is associated with nerve deafness, it is considered a distinct nosological entity.

Acid-Base Equilibrium↗
PubMed1993

Laboratory evaluation of renal tubular acidosis.

In this article, the renal tubular acidoses are briefly described, individual laboratory tests of urinary acidification are reviewed, and the administration and interpretation of these tests are discussed. A systematic approach to the laboratory evaluation of hyperchloremic metabolic acidosis is included.

Acidosis, Renal Tubular↗
PubMed1977

Lymphoid interstitial pneumonia with associated chronic active hepatitis and renal tubular acidosis.

Various combinations of diffuse interstitial fibrosis, chronic liver disease, and renal tubular acidosis have been recorded. The patient we report is unique in that the features of chronic liver disease and renal tubular acidosis were discovered while the patient was being treated for lymphoid interstitial pneumonia. This sequence of events has not been previously described.

Acidosis, Renal Tubular↗
PubMed1991

Distal renal tubular acidosis: the value of urinary pH, PCO2 and NH4+ measurements.

Distal renal tubular acidosis (dRTA) is not a single disease. The experimental forms of the syndrome are unsatisfactory as models of the naturally occurring disease, not least because they are seldom complicated by nephrocalcinosis, which is present in the majority of patients with spontaneous disease and contributes to the renal tubular defects found in the syndrome. Impairment of minimal urine pH, reduced urine carbon dioxide tension (PCO2) during passage of alkaline urine, and reduced urinary ammonium (NH4+) excretion, have all been advocated as essential criteria for the diagnosis of dRTA. Minimal urine pH, measured during metabolic acidosis, sulphate infusion, or after oral frusemide, is the yardstick against which other criteria should be assessed. A reduced urinary PCO2 is commonly found in dRTA but is not specific for the syndrome and may be accounted for by tubular defects other than those involving reduced distal hydrogen ion secretion. NH4+ excretion is reduced in most patients with renal acidosis whatever the nature of the underlying renal disease; this function is closely related to nephron mass, and is not specifically impaired in renal tubular disease.

Acidosis, Renal Tubular↗
PubMed1971

Renal tubular acidosis and autoimmune liver disease.

Forty-two patients with autoimmune liver disease have been investigated. Renal tubular acidosis was detected in 60% of the patients with primary biliary cirrhosis, in 30% with active chronic hepatitis, and in one out of seven cases with cryptogenic cirrhosis. The presence of the defect of renal acidification was not related to the level of plasma potassium, copper, or total globulin, nor to the pattern of immunological abnormalities detected in the serum. It is suggested that autoimmune liver disease and renal tubular acidosis may be part of a systemic disorder in the pathogenesis of which immunological mechanisms are involved.

Acidosis, Renal Tubular↗
PubMed1985

[Infantile transitory distal renal tubular acidosis with bicarbonate loss].

Apart from the classic distal renal tubular acidosis (RTA), the proximal RTA, and a few cases of distal RTA and renal bicarbonate wasting we know only 2 cases of infantile transient distal RTA with bicarbonate wasting. A 3 month-old male patient is admitted because of deficient suction, vomiting and dehydration. Despite a strong metabolic acidosis (pH 7,09, bicarbonate 8,6 mMol/l, chloride 110 meq/l) the urine is constantly alkaline; clinically the disease manifests itself in the form of an alkali-resistant RTA. Accompanying troubles such as inner ear deafness, G6PDH deficiency, hyperparathyroidism and vitamin D intoxication are to be excluded. A bicarbonate study carried out with care so as to prevent extracellular fluid expansion reveals the lack of excretion of titratable acid (-2.4 to +4.7 mueq/min/1.73 m2), an reduced excretion of ammonium (5 to 24.8 mueq/min/1.73 m2) with regard to GFR (42.4 ml/min/1.73 m2), and a constant loss of bicarbonate (FE HCO3- about 10%) covering most of the bicarbonate plasma concentration, which results in a constantly negative net acid excretion. Even with alkalosis there is no urine minus blood pCO2 increase. The renal excretion of gamma GT is significantly reduced. On substitution with high quantities of bicarbonate (10 meq/kg BW/day) the defect heals up at the age of 13 months. The pathogenesis of this disease is not quite clear, but is similar to that of the Lightwood infantile RTA. The acidification defect may be explained by a deficient hydrogen ions--secretion in the distal tubule; as for kinetics, it is not in the proximal tubule that the bicarbonate wasting occurs but it may be due to increased sodium delivery to the distal nephron.

Acidosis, Renal Tubular↗
PubMed2001

Hypopotassemic paralysis: a rare presentation of proximal renal tubular acidosis.

Neurologic manifestations can accompany systemic diseases, and primary disease can be identified with a careful history, physical examination, and laboratory investigations. A 14-year-old girl with paraplegia and absence of deep tendon reflexes in the lower extremities after 2 days of vomiting and diarrhea was referred to our pediatric neurology department with a diagnosis of Guillain-Barré syndrome. Short stature, dehydration, motor and mental retardation, bilateral cataracts, glaucoma, and band keratopathy were detected on physical examination. Hypopotassemia and severe metabolic acidosis were found on biochemical examination. Her paraplegia improved after appropriate fluid and electrolyte replacement, but metabolic acidosis persisted after cessation of intravenous therapy, and isolated proximal renal tubular acidosis was detected. Because she had isolated proximal renal tubular acidosis and other abnormalities, she was diagnosed with Donckerwolcke-Winsnes syndrome.

Acidosis, Renal Tubular↗
PubMed2000

Atypical distal renal tubular acidosis confirmed by mutation analysis.

In autosomal dominant distal renal tubular acidosis type I (dRTA) impaired hydrogen ion secretion is associated with metabolic acidosis, hyperchloremic hypokalemia, hypercalciuria, nephrocalcinosis, and/or nephrolithiasis. A retardation of growth is commonly observed. In this report we present a family with autosomal dominant dRTA with an atypical and discordant clinical picture. The father presented with severe nephrocalcinosis, nephrolithiasis, and isosthenuria but metabolic acidosis was absent. His 6-year-old daughter, however, suffered from metabolic acidosis, hypokalemia, and hypercalciuria. In addition, sonography revealed multiple bilateral renal cysts but no nephrocalcinosis. Mutation analysis of the AE1 gene coding for the renal Cl-/HCO3(-)-exchanger AE1 displayed a heterozygous Arg589Cys exchange in both patients but not in the healthy family members. This point mutation is frequently associated with autosomal dominant dRTA. Diagnosis of autosomal dominant dRTA is supported in this family by results of AE1 mutation analysis.

Acidosis, Renal Tubular↗