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At least 1,135 records · Page 63Linked to original sources
PubMed1995

Biochemical markers of renal disease in primary Sjögren's syndrome.

Primary Sjögren's syndrome (SS) is characterized by an inflammatory process in the salivary and lacrimal glands, but the kidneys may also be involved. Renal tubular functions were studied in 27 patients with SS, all females, age 37-78. Both SS-patients with and without known distal renal tubular acidosis (dRTA) were included, dRTA was found in 18/27 (67%), impaired urine concentrating ability in 13/27 (48%). Hypocitraturia was identified in 20/27 (74%) and reduced tubular reabsorption of phosphate (TRP%) in 18/27 (67%). Tubular proteinuria (alpha 1-mikroglobulin) was present in 11/24 (46%), and tubular enzymuria (NAG) in 7/24 (29%). Hypocitraturia and/or dRTA were found in all patients with any kind of abnormal renal tubular function test. All except one of the patients with dRTA not treated with sodium bicarbonate had hypocitraturia. We conclude that distal tubular dysfunction was common in our SS-patients, but a concommitant proximal dysfunction was also seen. Determination of urinary citrate represents a valuable test for detection of renal disease in SS.

Acetylglucosaminidase↗
PubMed2000

[The clinical and biochemical manifestations of Fanconi syndrome: a report of 42 cases].

OBJECTIVE: To recognize the etiologies, clinical, and biochemical manifestations of Fanconi syndnome (FS). METHODS: 42 patients with FS were analyzed retrospectively. RESULTS: 21 cases were idiopathic in 42 (50%) cases, and the other 21 (50%) cases were acquired (sjogren's 5, and interstitial nephritie 8). The patients were characterized by proximal renal tubular acidosis (n = 41) and multiple renal tubular transport dysfunctions, including hypokalemia (n = 21), hypophorphatemia (n = 29), hypourecemia (n = 19), renal glucosuria (n = 38), aminoaciduria (n = 36), low-molecular-weight proteinuria (n = 21). The clinical manifestations commonly presented with muscle weakness, polydipsia, polyuria and renal bone diseases. 18 patients presented with impaired renal function. Renal pathohistological studied in 14 patients showed renal tubulointerstitial changes of different degrees in all cases, and glomerular changes in 4. CONCLUSIONS: The etiology of FS is various and secondary FS is not uncommon. FS usually present proxmal RTA and renal tubulointerstitial lesions in pathology.

Acidosis, Renal Tubular↗
PubMed1975

Nephrocalcinosis in infancy and childhood.

The diangosis, clinical manifestations and management of 11 children with nephrocalcinosis encountered in a 20-year period are presented. Renal tubular acidosis, primary hyperoxaluria, primary hyperparathyroidism, exogenous hyperadrenocorticism and idiopathic hypercalcemia of infancy were the principal causes of nephrocalcinosis in this series. In the presence of normal or near-normal renal function, a 55 per cent or better cure rate can be expected. Children with significantly diminished renal function have a poor prognosis and should be considered for renal transplantation.

Acidosis, Renal Tubular↗
PubMed2005

Tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy and primary Sjögren's syndrome.

We report a case of hypokalemic nephropathy associated with primary Sjögren's syndrome (SS). The patient presented with profound and persistent hypokalemia secondary to distal renal tubular acidosis (RTA). A renal biopsy exhibited tubular degeneration, marked interstitial fibrosis and intense macrophage infiltration. Hypokalemia has been reported to induce macrophage infiltration in experimental animal models but not in humans. This is the first report of intense tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy associated with SS.

Adult↗
PubMed1981

The pathogenesis of hyperchloremic metabolic acidosis associated with kidney transplantation.

The mechanism of persistent hyperchloremic metabolic acidosis developing after kidney transplantation was investigated in six patients. In five patients in whom acidosis failed to lower the urine pH below 5.5, an infusion of sodium sulfate also failed to lower the urine pH. Neutral phosphate infusion failed to increase the urine minus blood (U-B) carbon dioxide tension (pCO2) difference normally in these patients. This abnormal response to both maneuvers indicates the presence of a tubular defect for distal hydrogen ion secretion. In the remaining patient, spontaneous acidosis lowered the urine pH below 5.5 and increased the U-B pCO2 normally with the administration of phosphate, demonstrating that this patient's distal capacity for hydrogen secretion was intact. The plasma aldosterone level was low in this patient, and thus he had the acidification defect characteristic of aldosterone deficiency. Hyperkalemia developed in two patients; both were aldosterone-deficient, and they had a low fractional potassium excretion ion response to stimulation with sodium sulfate or acetazolamide. In all but one patient, who lost his kidney to accelerated rejection, chronic rejection developed. Homogeneous deposition of complement (C3) along the tubular basement membrane was found in three patients. Our data suggest that a secretory type of distal renal tubular acidosis can be an early sign of the immunologic process that leads to chronic rejection.

Acidosis, Renal Tubular↗
PubMed1999

[Metabolic acidosis in children: the usefulness of 'anion gap'].

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.

Acid-Base Imbalance↗
PubMed1977

Renal acidifying ability in subjects with recurrent stone formation.

To determine the incidence of an acidification defect in men in whom calcium stones form and its relationship to parathyroid function 120 such patients were given an acute dosage of 0.1 gm. per kg. oral ammonium chloride and circulating immunoreactive parathyroid hormone was determined. The subjects were divided into 2 groups, according to normal or high parathormone levels. Group 1 consisted of 46 men in whom immunoreactive parathyroid hormone was less than or equal to 60 mulEq. per ml. and group 2 consisted of 74 men with immunoreactive parathyroid hormone greater than 60 mulEq. per ml. Of 8 men in whom the urine failed to acidify to less than a pH of 5.3, 3 were from group 1 and 5 were from group 2. None of the patients had an active urinary tract infection. There was no difference in minimal urine pH among the patients in whom the urine acidified normally regardless of immunoreactive parathyroid hormone. The incidence of abnormal acidification in our population was 6% and all of these patients had the incomplete form of renal tubular acidosis. These findings have important therapeutic implications.

Acidosis, Renal Tubular↗
PubMed2001

ARC syndrome: an expanding range of phenotypes.

AIM: To describe the clinical phenotype in infants with ARC syndrome, the association of arthrogryposis, renal tubular acidosis, and cholestasis. METHODS: The medical records for six patients with ARC syndrome were reviewed, presenting over 10 years to three paediatric referral centres. RESULTS: All patients had the typical pattern of arthrogryposis. Renal Fanconi syndrome was present in all but one patient, who presented with nephrogenic diabetes insipidus. Although all patients had severe cholestasis, serum gamma glutamyltransferase values were normal. Many of our patients showed dysmorphic features or ichthyosis. All had recurrent febrile illnesses, diarrhoea, and failed to thrive. Blood films revealed abnormally large platelets. CONCLUSIONS: ARC syndrome exhibits notable clinical variability and may not be as rare as previously thought. The association of Fanconi syndrome, ichthyosis, dysmorphism, jaundice, and diarrhoea has previously been reported as a separate syndrome: our observations indicate that it is part of the ARC spectrum.

Acidosis, Renal Tubular↗
PubMed2001

IgA-kappa type multiple myeloma affecting proximal and distal renal tubules.

A 45-year-old male was admitted because of chest pain, lumbago, and bilateral ankle pain. Examination disclosed hypophosphatemic osteomalacia, acquired Fanconi syndrome, and abnormalities in distal nephron such as distal renal tubular acidosis and renal diabetes insipidus. Further exploration revealed IgA kappa multiple myeloma excreting urinary Bence Jones protein (kappa-light chain). Renal biopsy revealed thick basement membranes and elec-tron-dense crystals in proximal tubular epithelial cells. Immunofluorescent studies revealed deposition of kappa-light chain in renal tubular epithelial cells that caused the renal tubular damage. Although the osteomalacia was relieved by medical treatment, the urinary Bence Jones protein and the renal tubular defects were not improved by the chemotherapy for the myeloma. The patient died of exacerbation of multiple myeloma at 50 years of age.

Biopsy↗
PubMed1998

Diagnosis and treatment of renal tubular disorders.

There is a wide variety of renal tubular disorders affecting children. Understanding the pathophysiology of each disease assists in the recognition and treatment of these diseases, which can have profound effects on a child's growth and development. We review some of the more common tubular disorders found in children, emphasizing those conditions in which recent advances have been made in understanding of the specific genetic or physiological defect responsible: Fanconi's syndrome, cystinuria, renal tubular acidosis, and the hypokalemic metabolic alkaloses. Current recommendations are presented for diagnosing and treating these conditions.

Acidosis, Renal Tubular↗
PubMed1999

Distal tubular dysfunction in lupus nephritis of childhood and adolescence.

We describe a girl with lupus nephritis who presented with distal renal tubular acidosis and hyporeninemic hypoaldosteronism. While distal tubular dysfunction is well recognized in adult systemic lupus erythematosus (SLE), only a few pediatric patients have been reported. Evaluation of five pediatric patients with SLE revealed that distal tubular dysfunction in childhood and adolescence is rare.

Acidosis, Renal Tubular↗
PubMed1996

Evaluation of urine acidification by urine anion gap and urine osmolal gap in chronic metabolic acidosis.

To investigate the clinical significance of urine anion gap and urine osmolal gap as indirect markers of urine acidification in chronic metabolic acidosis, we evaluated urine ammonium (NH4+), net acid excretion (NAE), urine anion gap (Na(+) + K(+) - Cl-), and urine osmolal gap (urine osmolality - [2(Na(+) + K(+)) + urea]) in 24 patients with chronic renal failure (CRF), eight patients with classic distal renal tubular acidosis (dRTA), and eight NH4Cl-loaded normal controls (NCs). Urine NH4+ excretion was lower (P < 0.001) in the CRF (5.4 +/- 0.6 mmol/d) and dRTA (19.2 +/- 2.7 mmol/d) patients than in the NCs (52.6 +/- 3.7 mmol/d); NAE was also lower (P < 0.001) in the CRF (9.8 +/- 1.6 mmol/d) and dRTA (16.7 +/- 4.7 mmol/d) patients than in the NCs (79.4 +/- 4.7 mmol/d). Urine anion gap was higher (P < 0.001) in the CRF (24.7 +/- 2.2 mmol/L) and dRTA (36.7 +/- 7.7 mmol/L) patients than in the NCs (-16.2 +/- 5.5 mmol/L). Urine osmolal gap was lower (P < 0.05) in the dRTA patients (129.7 +/- 17.0 mmol/L) than in the NCs (319.7 +/- 58.4 mmol/L). When the data from all subjects were pooled, urine anion gap correlated inversely with urine NH4+ (r = -0.70, P < 0.001) and with NAE (r = -0.83, P < 0.001), and urine osmolal gap correlated positively with urine NH4+ (r = 0.69, P < 0.01) and with NAE (r = 0.71, P < 0.05). We conclude that impaired urine acidification in CRF and dRTA patients is associated with an increase in urine anion gap and a decrease in urine osmolal gap, and that both urine anion gap and urine osmolal gap correlate well with NAE as well as with urine NH4+.

Acid-Base Equilibrium↗
PubMed1981

Hypokalemic periodic paralysis in Sjögren's syndrome.

A 30-year-old woman had scleroderma, Sjögren's syndrome, deforming polyarthritis, distal renal tubular acidosis, hypokalemic periodic paralysis, and persistent mild myopathy. During a five-year period the patient's otherwise mild course of disease was complicated by the occurrence of five episodes of severe flaccid muscle paralysis involving both proximal and distal muscle groups. Between the paralytic episodes the patient functioned well without replacement therapy, and had normal potassium levels. The sicca component was mild and went unrecognized for several years. There was no family history of muscle disease. The data presented in this report support the view that the paralytic episodes were due to hypokalemia secondary to renal tubular acidosis associated with Sjögren's syndrome. Hypokalemic periodic paralysis may occur as a rare complication of Sjögren's syndrome and renal tubular acidosis.

Acidosis, Renal Tubular↗
PubMed1986

Loading tests for diagnosis of metabolic anomalies in urinary stone formers.

Four per cent of the population in the industrialized countries of Europe suffer once or several times from urinary calculus in the course of their lives. The high number of recurrences (50 to 60%) necessitates specific prophylaxis. Depending on the precise stone composition, a specific metabolic investigation should be undertaken in cases with recurrent urinary calculus. Within a special laboratory diagnostic program, all major anomalies can be diagnosed by means of loading tests. With the ammonium chloride loading test, renal tubular acidosis is diagnosed; the calcium loading test differentiates the types of hypercalciuria and the purine loading test verifies "latent hyperuricaemia".

Acidosis, Renal Tubular↗
PubMed1995

Diseases of renal adenosine triphosphatase.

Most renal transport is a primary or secondary result of the action of one of three membrane bound ion translocating ATPase pumps. The proximal tubule mechanisms for the reabsorption of salt, volume, organic compounds, phosphate, and most bicarbonate reabsorption depend upon the generation and maintenance of a low intracellular sodium concentration by the basolateral membrane Na-K-ATPase pump. The reabsorption of fluid and salt in the loop of Henle is similarly dependent on the energy provided by Na-K-ATPase activity. Some proximal tubule bicarbonate reabsorption and all distal nephron proton excretion is a product of one of two proton translocating ATPase pumps, either an electrogenic H-ATPase or an electroneutral H-K-ATPase. In this article, the authors review the biochemistry and physiology of pump activity and consider the pathophysiology of proximal and distal renal tubular acidosis, the Fanconi syndrome, and Bartter's syndrome as disorders of ATPase pump function.

Acidosis, Renal Tubular↗
PubMed1997

A new classification for renal defects in net acid excretion.

The traditional classification of the group of disorders called renal tubular acidosis (RTA) into proximal and distal subclasses is based on which nephron segment is thought to have an abnormal function. Nevertheless, such a distinction may not be correct and also does not characterize the pathophysiology of the renal acidosis in each patient. In this article, we propose an alternative classification, one that is based on the component of net acid excretion that is abnormal. We also suggest expanding the definition of net acid excretion to include a term that describes the renal handling of metabolizable organic anions because their loss in the urine represents the loss of "potential bicarbonate." Because a low rate of excretion of ammonium (NH4+) is present in patients with both distal and isolated proximal RTA, our initial clinical step in patients with hyperchloremic metabolic acidosis (HCMA) is to evaluate the rate of excretion of NH4+. The basis for a low rate of excretion of NH4+ is shown by examining the urine pH. If the urine pH is low, further studies are performed to determine why the availability of NH3 is low; if the urine pH is high, further investigations are initiated to examine if the defect in H+ secretion involves the proximal or the distal nephron. Conversely, if the rate of excretion of NH4+ is high in a patient with HCMA, a component of the degree of acidosis could be attributable to a high rate of excretion of metabolizable organic anions. Case examples are provided to illustrate the approach and its implications for future molecular studies.

Acidosis, Renal Tubular↗
PubMed1978

HLA and kidney stone disease.

Normocalcemic patients with recurring kidney stones were HLA typed. 54 patients with an idiopathic high urinary excretion of calcium but without signs of renal tubular defect showed a B27 phenotype frequency which was more than twice that of the controls and a CW1 frequency that was doubled. After correction for the number of tested HLA specificities the increase was not significant. 35 patients with renal tubular acidosis and 16 patients with no metabolic diagnosis had no deviant HLA antigen distribution.

Acidosis, Renal Tubular↗