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PubMed1980

Renal handling of water and sodium in children with proximal and distal renal tabular acidosis.

Renal sodium wasting has been observed in both proximal and distal renal tubular acidosis (RTA), although few studies have been reported indicating the tubular localization of such a defect. The use of clearance methodology during hypotonic saline diuresis may give an indirect estimate of proximal tubular reabsorption of sodium, sodium reabsorption at the diluting segments and proportion of sodium load reabsorbed distally. This study was carried out in 17 normal children, in 9 children with proximal RTA, associated in all but one with the Fanconi syndrome, and in 5 children with primary distal RTA. Patients with proximal RTA presented mainly an impaired reabsorption of sodium in the proximal tubule, which was in great part but not completely compensated by an absolute increase in distal sodium reabsorption. Patients with distal RTA showed normal reabsorption of sodium in the proximal tubule but they were unable to reabsorb completely the load of sodium escaping proximal reabsorption due to a defect of sodium reabsorption in the distal diluting segments. These results indicate that the classification of RTA in proximal and distal types is also valid according to the differences found in the tubular handling of water and sodium.

Acidosis, Renal Tubular↗
PubMed2004

Hypokalemic paralysis revealing Sjögren's syndrome.

A rare case of a lady with acute hypokalemic quadriparesis and underlying distal renal tubular acidosis manifesting as a presentation of Sjögren's syndrome is described. The case highlights the concept that acute hypokalemia due to unrecognized renal tubular acidosis may unmask Sjögren's syndrome in patients without sicca symptoms and it may be a marker of more severe renal disease. Acute paralysis is a life threatening consequence of hypokalemia and when due to potassium wasting secondary to renal tubular acidosis may be easily prevented. Underlying Sjögren's syndrome should be considered in all patients of either sex and at any age presenting with hypokalemic paralysis.

Acidosis, Renal Tubular↗
PubMed1994

Renal acidification in children with idiopathic hypercalciuria.

Distal renal tubular acidosis is frequently associated with hypercalciuria. To further investigate the cause-and-effect relationships between the two conditions, we examined 20 children (5 to 18 years of age) with idiopathic hypercalciuria for evidence of renal tubular acidosis. Serum electrolytes and urine citrate levels were normal in all subjects. After a single dose of furosemide, 1 of the 20 subjects did not show a decrease in urine pH < 5.5, which suggests an acidification defect in the cortical collecting duct. Three other patients failed to show an increase in urine-minus-blood partial pressure of carbon dioxide > 20 mmHg after urine alkalinization with orally administered acetazolamide, a finding compatible with a rate-dependent distal renal tubular acidosis. These four subjects underwent acute acid loading with arginine hydrochloride. In all four subjects urine pH decreased < 5.5 but urinary ammonium excretion failed to increase normally; this supports the diagnosis of a defect in distal acidification. Four of six patients with nephrolithiasis had evidence of distal renal tubular acidosis, in contrast to none of the 14 patients without stones (p = 0.003). We conclude that distal acidification abilities seem to be intact in children with hypercalciuria in the absence of nephrolithiasis. We speculate that calcium precipitation may lead to tubular damage, including distal renal tubular acidosis.

Acetazolamide↗
PubMed1979

Pyruvate carboxylase deficiency and lactic acidosis in a retarded child without Leigh's disease.

A child with lactic acidosis, severe mental and developmental retardation, and proximal renal tubular acidosis is presented. Biopsy and autopsy studies show severe hepatic, renal cortical, and cerebral deficiencies in pyruvate carboxylase (EC 6.4.1.1) activity. The patient had 1.81 +/- 0.20 units/g fresh weight at biopsy and 0.75 +/- 0.07 units/g fresh weight hepatic pyruvate carboxylase activity at autopsy compared with 10.9, 11.3, and 9.5 units/g fresh weight in two autopsy and one biopsy controls, respectively. The patient's renal cortical pyruvate carboxylase activity at autopsy was 0.008 +/- 0.004 units/g fresh weight compared with 5.05 units/g in the autopsy control. The patient had no detectable (less than 0.018 units/g fresh weight) cerebral pyruvate carboxylase activity at autopsy compared with 0.44, 0.53, and 0.695 units/g in the autopsy cerebrum of one human and two rhesus monkeys, respectively. Pyruvate dehydrogenase complex, phosphoenolpyruvate carboxykinase (PEPCK, EC 4.1.1.32), and fructose-1,6-bisphosphatase (EC 3.1.3.11) activities were in the normal range. The patient's urine pH was above 7.9 when the total serum CO2 was greater than 7.8 mM. However, the patient was able to acidify the urine to pH 5.1 when the total serum CO2 was 1.6 mM. The neuropathologic examination of the brain at autopsy revealed no sign of Leigh's disease, although developmental and degenerative lesions were observed. This is the first reported patient with a primary deficiency in hepatic, renal, and cerebral pyruvate carboxylase deficiency in whom the neuropathologic lesions, distinct from those of Leigh's disease, and proximal renal tubular acidosis have both been documented.

Acidosis↗
PubMed1990

Effect of lipoic acid in a patient with defective activity of pyruvate dehydrogenase, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase.

Lactic acidosis and accumulation of 3-hydroxybutyrate and other citric acid cycle intermediates were found in an infant with a lethal syndrome of metabolic acidosis and renal tubular acidosis. Nevertheless, the patient was relatively well for 4 mo of life. The activity of the pyruvate dehydrogenase complex, 2-oxoglutarate dehydrogenase, and branched-chain keto acid dehydrogenase were all reduced to levels 9 to 29% of control. In contrast, the activity of lipoamide dehydrogenase was normal. The conversion of 1-14C-leucine and 1-14C-valine to 14CO2 and of U-L-14C-valine to its major metabolic product 3-hydroxyisobutyric acid by fibroblasts derived from the patient was less than 5% of control. Cultivation of the patient's fibroblasts in medium enriched with lipoic acid markedly improved these in vitro conversions of leucine and valine.

3-Hydroxybutyric Acid↗
PubMed1985

Plasma renin activity, plasma aldosterone and distal urinary acidification in diabetics with chronic renal failure.

The renin-angiotensin-aldosterone system and the acidification capacity of the renal tubule were studied in 13 diabetic patients with chronic renal failure. As a whole, the group showed hyporeninaemic hypoaldosteronism (HH). Studied alone, 12 of the 13 patients presented the requirements for HH. This group showed hypercholaemic hyperkalaemic metabolic acidosis with a disturbance in renal acidification which may be classified as Type IV renal tubular acidosis. The results of this group were compared to those of another two groups; one of diabetic patients without chronic renal failure and the other with chronic renal failure (C Cr less than 40ml/min); both were seen to show different behaviour to that of the group affected by the two processes.

Acidosis, Renal Tubular↗
PubMed1995

Medullary nephrocalcinosis: sonographic findings in adult patients.

Medullary nephrocalcinosis occurs in various diseases as a non-specific renal manifestation. We present 5 patients (hypophosphataemic rickets, type 1 renal tubular acidosis, primary hyperparathyroidism, hypercalcaemia of unclear origin, chronic renal insufficiency requiring dialysis) in whom a medullary nephrocalcinosis was demonstrated by means of sonographically detectable changes in the renal medulla region. The sonographic appearance of medullary nephrocalcinosis is characterized by detection of echo-enhanced structures in the region of the renal pyramids. The presence of a medullary nephrocalcinosis can generally be confirmed with adequate reliability on the basis of sonographic findings and characteristic clinical pictures. In individual cases it is difficult to distinguish between medullary nephrocalcinosis and renal calyx calculi.

Acidosis, Renal Tubular↗
PubMedDate not supplied

Renal handling of citrate in children with various kidney disorders.

Citrate (CIT) excretion was measured in 24 h urine of children with various kidney disorders and controls. It was normal in urinary tract infection, idiopathic urolithiasis, and idiopathic hypercalciuria in the absence of renal dysfunction, but reduced in acute glomerulonephritis, distal renal tubular acidosis, and chronic renal failure. Increased citraturia was observed in cystinosis and in idiopathic de Toni-Debré-Fanconi syndrome. Renal handling of CIT was studied in 45 children with various chronic kidney disorders under standard inulin clearance conditions. CIT clearance correlated well with GFR above 50 ml/min/1.73 m2. At lower levels, percent tubular reabsorption of CIT decreased rapidly, reaching levels between 17% and 41% at GFR less than 10 ml/min/1.73 m2; this disproportionate fall might be related to reduced renal CIT utilization at a relatively early stage of renal insufficiency. During acid loading tests, citraturia was lowered, with a decrease of both urinary pH and plasma bicarbonate in tubular disorders and in controls. The lowest CIT excretion was measured in incomplete renal tubular acidosis with magnesium wasting. The findings are discussed in view of recent physiological data on renal metabolism of CIT.

Adolescent↗
PubMed2000

Mesangial nephropathy in Sjögren's syndrome.

We describe a 36-year-old woman with Primary Sjögren's Syndrome (PSS). Purpura, corneal perforation, metabolic acidosis, decreased glomerular filtration, hypokalemia, hyposthenuria, and polyuria were present. Chronic renal insufficiency and renal tubular acidosis type I were diagnosed. Kidney biopsy revealed mesangial glomerulonephritis, interstitial nephritis, and tubular atrophy. Replacement treatment with saliva, tears, and potassium citrate was started. She was given prednisone and cyclophosphamide. This would be the first description of PSS, mesangial glomerulonephritis, and chronic renal insufficiency.

Acidosis, Renal Tubular↗
PubMed1991

Metabolic bone disease associated with systemic disorders.

Osteoporosis occurs in patients with rheumatoid arthritis, acromegaly, anorexia nervosa, chronic liver disease, sickle cell hemoglobinopathies, and mastocytosis. Osteomalacia occurs in patients with renal tubular acidosis, and with tumors.

Acidosis, Renal Tubular↗
PubMed1985

Family history of renal stones in recurrent stone patients.

A survey was carried out on the family history of renal stones among 380 patients in an out-patient stone clinic, most of whom had experienced frequent recurrences. In 55.4% of patients at least one first-degree relative had experienced renal stones. A positive family history was more common in females (64.7%) than in males (51.0%) and was particularly apparent in those who had multiple recurrences. It was also significantly more common in female patients with evidence of renal tubular defects (incomplete forms of renal tubular acidosis). Among the ordinary stone patients 18% of the fathers and 8% of the mothers had also formed renal stones. The corresponding figures for female stone patients with renal tubular defects were 40 and 33% respectively. These findings indicate that tubular dysfunction could be an inherited trait that predisposes to the formation of renal stones. Hypercalciuria or hyperuricosuria was not over-represented among stone formers with a positive family history.

Acidosis, Renal Tubular↗
PubMed1998

Distal tubular acidosis induced by FK506.

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.

Acidosis, Renal Tubular↗
PubMed1998

An infant case of bilateral small kidneys with both proximal and distal tubular dysfunction.

A male infant with bilateral small kidneys associated with both proximal and distal tubular dysfunction, who showed chronic renal failure soon after birth, is reported. He was also noted to have both proximal and distal type of renal tubular acidosis. The small kidneys were thought to be due to renal hypodysplasia associated with bilateral severe vesicoureteral reflux, by radiological findings. An alkalization therapy with chemoprophylaxis seemed to be of benefit in slowing the progression of renal failure in this case.

Acidosis, Renal Tubular↗
PubMed2006

Acquired hypophosphatemia osteomalacia associated with Fanconi's syndrome in Sjögren's syndrome.

Sjögren's syndrome is an autoimmune disorder involving exocrine glands that occurs alone or in association with various autoimmune and connective tissue diseases. The severity of Sjögren's syndrome ranges from isolated sicca syndrome to severe complications such as vasculitis, lung and renal involvement. Overt or latent renal tubular acidosis caused by autoimmune tubulointerstitial nephritis, is a common extraglandular manifestation in Sjögren's syndrome. Osteomalacia is a rare complication of renal tubular acidosis, and it was reported to be associated with distal renal tubular acidosis in Sjögren's syndrome. We report a 60-year-old woman who presented with multiple bone deformity and general muscle weakness. Osteomalacia was secondary to Fanconi's syndrome, and the Fanconi's syndrome was a result of renal involvement in Sjögren's syndrome. Fanconi's syndrome is a rare kidney manifestation in Sjögren's syndrome. It may be latent and may precede the subjective sicca symptoms. These findings suggest that evidence for Sjögren's syndrome should be sought in adult patients with unexplained osteomalacia and renal tubular acidosis, even in the absence of subjective sicca syndrome. Conversely, in patients with Sjögren's syndrome, early investigation and treatment of renal tubular dysfunction may prevent future complications, such as osteomalacia.

Biopsy↗
PubMed1983

Low urinary citrate excretion in nephrolithiasis.

The urinary citrate excretion was examined in patients with nephrolithiasis who were categorized on the basis of different physiologic or metabolic abnormalities. A wide prevalence of low citrate excretion (hypocitraturia) was observed, with over one half of our patients with stones exhibiting it. Hypocitraturia was found in all patient categories except primary hyperparathyroidism and hyperuricosuric calcium oxalate nephrolithiasis. As expected, hypocitraturia was present in renal tubular acidosis and in enteric hyperoxaluria. However, urinary citrate was also low in absorptive and renal hypercalciurias, and in patients in whom an acid-base disturbance was clearly excluded.

Acidosis, Renal Tubular↗
PubMed1994

[Renal manifestation in POEMS syndrome].

The authors report four cases of POEMS Syndrome with renal involvement. Two had renal failure and mild proteinuria. The two others had hyporeninemic hypoaldosteronism with type IV renal tubular acidosis in the first and hyperkalemia alone in the second. In the two cases, renal biopsy showed mesangial proliferation. Evolution was favourable when the plasma cell proliferation could be checked. In POEMS pathophysiology of renal involvement remains unknown. Soluble factor(s) produced by plasma cells may be responsible for most signs and symptoms perhaps as a result of increased vascular permeability.

Acidosis, Renal Tubular↗
PubMed1988

Renal acidification defects in medullary sponge kidney.

Thirteen patients with medullary sponge kidney underwent a short ammonium chloride loading test to investigate their renal acidification capacity. All but 1 presented with a history of recurrent renal calculi and showed bilateral widespread renal medullary calcification on X-ray examination. Nine patients had some form of renal acidification defect; 8 had the distal type of renal tubular acidosis, 2 the complete and 6 the incomplete form. One patient had proximal renal tubular acidosis. These findings, which suggest that renal acidification defects play an important role in the pathogenesis of renal calculi in medullary sponge kidney, have considerable therapeutic implications.

Acidosis, Renal Tubular↗