[A case with voltage dependent-renal tubular acidosis type IV complicated with nephrolith].
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Child↗
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Studies on renal tubular acidosis in collagen diseases. Part II. The response of patients with collagen diseases to an acid load.
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Acidosis↗
Effect of hydrochlorothiazide on proximal renal tubular acidosis in a patient with idiopathic "de toni-debré-fanconi syndrome".
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Acidosis↗
Distal renal tubular acidosis associated with non-autoimmune hypothyroidism.
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Acidosis↗
Familial glomerulopathy with giant fibrillar (fibronectin-positive) deposits: 15-year follow-up in a large kindred.
A 15-year clinical follow-up is reported for a familial glomerulopathy characterized on light microscopy by the glomerular deposition of giant fibrillary deposits (Virchows Arch A Pathol Anat Histol 388:313-326, 1980). On electron microscopy, the deposits consist of randomly oriented fibrils (12 to 16 nm in width and 120 to 170 nm in length). These deposits show positive immunoreactivity for fibronectin. One hundred fifty-seven of 197 family members within five generations were investigated. The disease is characterized by the occurrence of albuminuria in the third to fourth decades of life and slow progression to end-stage renal disease over a period of 15 to 20 years with the occurrence of generalized distal tubular acidosis (renal tubular acidosis type IV), hypertension, and the nephrotic syndrome. The frequent occurrence of otherwise unexplained microalbuminuria in young individuals of generations IV and V could be indicative of incipient glomerular disease. In one affected male individual and in his unaffected sister, renal cell carcinoma was diagnosed, raising the possibility that this familial glomerulopathy might be associated with an increased risk to develop renal cell cancer by direct or indirect (associated genetic predisposition) mechanisms. The disease relapsed in one renal transplant, raising the possibility of the presence of a transferable factor that could be part of the deposited fibrillar material or, alternatively, interfere with the glomerular handling of the deposited material.
Acidosis, Renal Tubular↗
Acid-base disorders and the kidney.
In the normal human body, the extracellular fluid pH of 7.40 is closely protected. Any increase in acidity or alkalinity summons forth three lines of defense, starting immediately with the blood buffers, followed soon by the respiratory system's control of CO2, and finally purged by the renal excretion of the excess acid or base. The complex interrelated processes of the renal responses require a few days to accomplish maximum compensation. We have presented the fundamental principles governing maintenance of the acid-base equilibrium to provide a conceptual framework for understanding the clinical disorders of hydrogen ion metabolism. The somewhat elusive concepts of endogenous acid production and net acid balance have also been reviewed to help reveal the pathophysiology of metabolic acidosis caused by renal tubular acidosis, chronic renal failure, certain infant feedings, and total parenteral nutrition. The development and perpetuation of metabolic alkalosis in relationship to chloride and potassium deficiency have been examined. In the delineation of a clinical acid-base disorder, the clinician must bear in mind the continual interactions of electrolytes and hormonal systems and should be prepared to reevaluate frequently the elected therapy against the changing responses, based on a thorough understanding of physiology. The various types of renal tubular acidosis have manifold facets but the basic understanding of their pathophysiology begins with the concept of the "anion gap," a point of reference that can be used in the differential diagnosis and treatment. In this chapter a number of new causes of type IV renal tubular acidosis--currently considered to be the most common form of renal tubular acidosis--have been pointed out, along with special reference to the mineral, electrolyte, and aldosterone metabolism in the various acidoses and current means of reversing growth failure in the child, especially through bicarbonate treatment. The mechanism of metabolic acidosis in chronic renal failure including metabolic acidosis in children undergoing dialysis and in recipients of kidney transplantation, and its relationships to mineral and electrolyte metabolism have been presented. The pathophysiology of the acidosis related to certain infant formulas and the acidogenic properties of some amino acid solutions employed in total parenteral nutrition have been briefly reviewed. Finally, the metabolic alkalosis seen in a variety of chloride deficiency syndromes, such as Bartter's syndrome and dietary chloride deprivation, has been discussed and a rational approach to evaluation and treatment outlined.
Acid-Base Equilibrium↗
[Long-term follow-up study of bone mineral density in a patient with adult idiopathic Fanconi syndrome].
A 64-year-old woman complaining of severe lumbar pain was admitted to our hospital because of the finding of pre-existing mitral valve regurgitation on examination. Laboratory data revealed the proximal type of renal tubular acidosis, renal glucosuria, phosphaturia, generalized aminoaciduria and low-molecular-weight proteinuria. She did not have any cause of these tubular dysfunctions, and was diagnosed as adult idiopathic Fanconi syndrome. Dual energy X-ray absorptiometry (DEXA) revealed a reduction of bone mineral density in the lumbar spine to about 65% of the age-and gender-matched control value. Alkali agents (sodium citrate and potassium citrate), calcium lactate and 1 alpha-hydroxyvitamin D3 were administered. Bone mineral density estimated with DEXA improved with a reduction of serum alkali phosphate and disappearance of lumbar pain, and was restored to 82% of the age-and gender-matched control value after about 30 months of treatment. DEXA is useful for the long-term follow-up study of bone mineral density in a patient with Fanconi syndrome.
Absorptiometry, Photon↗
Adult Fanconi syndrome secondary to kappa-light chain myeloma: improvement of tubular functions after treatment for myeloma.
A 66-year-old man with kappa-light chain multiple myeloma had adult Fanconi syndrome. Renal tubular transport abnormalities consisted of renal tubular acidosis, renal glycosuria, aminoaciduria, phosphaturia and renal hypouricemia. After therapy for multiple myeloma, urinary Bence Jones protein became undetectable, and all these renal tubular abnormalities except urate wasting were corrected. Histological examination revealed electron-dense tubular and rod-like deposits in proximal tubular epithelium. This clinical observation suggests that the renal tubular transport defects were secondary to the myeloma process, possibly due to Bence Jones proteinuria.
Aged↗
Association between vitamin D receptor gene polymorphisms and tubular citrate handling in calcium nephrolithiasis.
OBJECTIVES: Hypocitraturia is a risk factor for calcium nephrolithiasis. 1,25(OH)2D3 influences renal citrate handling and enhances citraturia. The aim of this study was to evaluate the relationship between vitamin D receptor (VDR) allelic variant and urinary citrate excretion in recurrent stone formers (SF) patients. DESIGN: Case-control study. SUBJECTS: A total of 220 recurrent calcium oxalate SF patients and 114 healthy control (C) subjects were enrolled for this study. Subjects with urinary tract infections, hyperparathyroidism, cystinuria >70 micromol/24 h, gouty diathesis, renal tubular acidosis, renal failure, chronic diarrhoeal states, intake of thiazide diuretics, angiotensin-converting enzyme (ACE)-inhibitors, glucocorticoids or oestrogens were excluded. A standard constant diet was given for 7 days. The 24-h urinary citrate excretion and the active tubular reabsorption of filtered citrate (Rcit) were evaluated. Hypocitraturia was defined as a urinary citrate excretion lower than 1.7 mmol day-1. Stone formers patients and C were genotyped for BsmI and TaqI VDR alleles. Contingency table chi-square tests were used to compare genotype frequencies in hypocitraturic SF patients, normocitraturic SF and C. RESULTS: The prevalence of hypocitraturia in SF patients was 32.7% (72 of 200). Hypocitraturia in these patients resulted from excessive Rcit of a normal load of citrate. We found a different distribution (P < 0.05) of BsmI and TaqI VDR genotypes in hypocitraturic SF patients compared with normocitraturic SF and C. In particular, the prevalence of bb and TT VDR genotypes in hypocitraturic SF was significantly higher than in normocitraturic SF and C. CONCLUSIONS: These results point to a genetic association between BsmI and TaqI VDR polymorphisms and idiopathic hypocitraturia in calcium-oxalate recurrent SF patients.
Adult↗
Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy.
In this phase II trial, 105 eligible patients with no prior chemotherapy and advanced sarcoma received doxorubicin, ifosfamide, and dacarbazine (DTIC) with mesna uroprotection (MAID). Starting doses of these drugs were 60, 7,500, and 900 mg/m2 divided over 72 hours by continuous infusion, respectively. Mesna was given for 84 to 96 hours at 2,500 mg/m2/d. Myelosuppression was dose limiting, causing the only toxic death (sepsis). Nonhematologic toxicity consisted predominantly of anorexia and vomiting. Severe mucositis, macroscopic hematuria, renal tubular acidosis, renal failure, and CNS toxicity occurred in less than 5% of cycles. No cardiotoxicity was detected. The overall response rate (10% complete response [CR]) was 47% (95% confidence intervals, 5% to 18% and 37% to 57%, respectively). Most responses (approximately 70%) were observed within two cycles. Median times to progression were 10 and 9 months, respectively. Histologic high tumor grade, lesions less than 5 cm, and less than 1 year from diagnosis to study entry correlated with the probability of response. The median survival was 16 months. Time from diagnosis to study entry, performance status, and extent of disease, but not histologic grade, correlated with survival. Following CR, two patients remain disease-free at 32 and 16 months. Of the 15 additional patients rendered disease-free with surgery, two remain disease-free at 30 and 18 months with no further therapy. While most relapses occurred in sites of prior involvement, death from CNS metastases occurred in 11 of the 80 patients with high-grade sarcomas, of whom seven were still responding systematically (three complete responders). Because of its substantial response in this phase II trial, the MAID regimen is being compared with doxorubicin and DTIC alone in advanced sarcomas and to observation in the adjuvant treatment of high-grade sarcomas in randomized trials.
Actuarial Analysis↗
Renal cystic disease and ammoniagenesis in Han:SPRD rats.
Cyst formation in conditions associated with increased renal ammoniagenesis (hypokalemia, distal renal tubular acidosis, renal mass reduction) and experimental links between increased ammoniagenesis and interstitial inflammation have suggested a role for ammonia in the pathogenesis of polycystic kidney disease (PKD). To explore this hypothesis, Han:SPRD rats, a PKD model that affects male more severely than female animals, have been used. Heterozygous cystic (Cy/+) and homozygous normal (+/+) male and female offspring of Cy/+ rats were divided at 3 wk of age into control groups drinking water and experimental groups drinking 300 mM NH4Cl, 300 mM KHCO3, 200 mM KHCO3, 200 mM KCl, 200 mM NaHCO3, or 200 mM NaCl. At 2 months of age, the rats were kept fasting from 8:00 p.m. to 8:00 a.m. in metabolic cages and urine samples were collected under mineral oil. The rats were then weighed and anesthetized for the collection of blood and kidneys. The administration of 300 mM NH4Cl, and to a lesser extent that of 200 mM NaCl, was accompanied by an increase in the urinary excretion of ammonia and aggravation of the renal cystic disease. On the other hand, the administration of 300 mM KHCO3, 200 mM KHCO3, or 200 mM NaHCO3 lowered the urinary excretion of ammonia and markedly reduced the severity of the cystic disease and interstitial inflammation. The administration of 300 mM KHCO3, and to a lesser extent that of 200 mM KHCO3, resulted in the precipitation of calcium phosphate in the medullary collecting ducts.(ABSTRACT TRUNCATED AT 250 WORDS)
Ammonia↗
[Two cases of interstitial nephritis with primary Sjögren's syndrome successfully treated by steroid therapy].
We have experienced two cases of interstitial nephritis with Sjögren's syndrome successfully treated by steroid therapy. Case 1. A 50-year-old woman was admitted because of rash and arthralgia of the limb. Although her renal function was normal, serum IgG was extremely high and gallium-67 scintigrams indicated abnormal uptake in both kidneys with swelling on CT scans. Renal biopsy specimens showed a patchy dense interstitial infiltration of lymphocytes, monocytes and partly plasma cells with tubular cell atrophy. To suppress an excessive immune reaction, oral prednisolone was administrated at a dose of 30 mg/day. Six weeks after inception of the therapy, re-biopsy specimens showed a decrease in interstitial infiltration, particularly plasma cells, and interstitial fibrosis did not progress. After 8 weeks, the serum IgG level was normalized. Case 2. A 57-year-old woman was found to have renal involvement(serum creatinine level: 2.2 mg/dl) and anemia. Clinical findings suggested primary Sjögren's syndrome with renal tubular acidosis. Renal biopsy specimens showed a moderate to severe interstitial infiltration of inflammatory cells. After inception of steroid therapy(prednisolone 40 mg/day), renal involvement and renal anemia gradually improved. The renal function has been kept stable(serum creatinine level: 1.5 mg/dl) for 9 years by low-dose steroid therapy. These two cases suggest that steroid therapy plays two important roles: controlling of the acute immune reaction that causes renal fibrosis to progress in case 1, and long-term preservation of the renal function by the continuous use of low-doses in case 2.
Anti-Inflammatory Agents↗
[Tubulointerstitial injuries in heavy metal intoxications].
The kidney is one of the organs susceptible to heavy metal intoxication. The total body burden and "saturation" level in renal tissue are important limiting factors to the onset of renal injuries. Acute or chronic exposure to many of heavy metals can induce renal tubulointerstitial injuries, including acute tubular necrosis, chronic tubulointerstitial nephritis, Fanconi syndrome, renal tubular acidosis, and renal tubular dysfunction without morphological changes. Chronic cadmium intoxication can cause irreversible Fanconi syndrome with chronic tubulointerstitial nephritis. Both urinary low-molecular weight protein excretion and urinary cadmium excretion (greater than 200-400 ppm) are the most reliable earlier markers of tubulointerstitial injury in chronic cadmium intoxication. The role of metallothionein is central to an understanding of cadmium-induced nephropathy. Acute lead intoxication in children can cause reversible Fanconi syndrome. Hypertension, hyperuricemia, and elevated serum creatinine, without Fanconi syndrome, are clinical manifestations of chronic lead exposure in adults. Nuclear inclusion body in proximal tubular cell is characteristic. Chronic exposure to inorganic germanium can cause chronic renal failure without urinary abnormalities, due to tubular degeneration and interstitial fibrosis, mainly in the thick ascending limb of Henle and distal tubulus.
Adult↗
[Tubulointerstitial nephritis in chronic viral diseases of the liver].
The paper reports 4 cases of tubulointerstitial nephritis (TIN) in chronic diffuse diseases of the liver (CDDL). The latter comprised such conditions of virus etiology as chronic active hepatitis, chronic lobular hepatitis. TIN presented with pronounced renal tubular affections: defects of concentration capacity, distal renal tubular acidosis, renal diabetes insipidus. In 3 cases renal lesions occurred 1 and 6 years prior to CDDL detection. TIN pathogenesis in CDDL of viral etiology is discussed.
Adolescent↗
Pseudohypoaldosteronism with normal blood pressure.
In adults, persistent hyperkalemic distal renal tubular acidosis in the absence of impaired renal function is an unusual abnormality usually associated with the syndromes of aldosterone deficiency or resistance. Herein, we present an adult with a clinical picture consisting of a normal blood pressure of 125/80 mmHg, normal blood volume, and glomerular filtration rate, with hyperkalemic distal renal tubular acidosis. The patent could spontaneously lower her urine pH to less than 5.5. Plasma renin activity was normal. Serum aldosterone level was appropriately elevated for the level of serum potassium. Following alkalinization of the urine, she was able to generate a urinary to blood PCO2 gradient [U-B PCO2] of 55 mmHg, and had a ten fold increased potassium secretion. After salt restriction and furosemide administration, her potassium secretion rate increased only twofold despite correction of he acidosis. The acidosis, as well as the hyperkalemia, was completely corrected after 9-alpha-Fluorohydrocortisone administration. Hydrochlorothiazide corrected the acidosis and hyperkalemia. Collectively, this picture suggests an underlying chloride shunt as the possible pathophysiological mechanism. Our case in unique in that it is not associated with hypertension.
Acidosis, Renal Tubular↗
What was wrong with Tiny Tim?
One of the most endearing characters in English literature is Tiny Tim, the crippled son of Ebenezer Scrooge's clerk, Bob Cratchit. Yet the nature of Tiny Tim's multifaceted and implicitly reversible illness is a mystery and open to debate and speculation. From details of the original manuscript and the eight film versions, it is possible to construct a differential diagnosis for Tim's short stature, asymmetric crippling disorder, and curious intermittent weakness that would lead to his death, if untreated, within a period of 1 year. Following the ghostly visitations, Scrooge vows to assist the struggling Cratchit family financially, thereby making available the best medical care money could buy. From review of pediatrics texts from 1830 to 1850, a recommended treatment plan would have included (1) general measures such as country air and exercise, and fish oils such as cod and halibut (vitamin D), and (2) specific treatments of tonics (containing combinations of belladonna, opium, sodium bicarbonate, sodium citrate, and potassium chloride) emphasizing alkalis, and splinting and bracing the limbs. Such treatments with vitamin D and alkalinization with sodium bicarbonate and sodium citrate suggest the plausible speculation that Tiny Tim had renal tubular acidosis (type I), a disorder that is characterized by growth failure and, if left untreated, complicated by osteomalacia with pathologic fractures, hypokalemic muscle weakness and periodic paralysis, nephrocalcinosis leading to renal failure, and death. I propose that Tiny Tim had distal renal tubular acidosis (type I).
Acidosis, Renal Tubular↗
Kidney involvement in primary Sjögren's syndrome.
Kidney involvement is well recognized extraglandular manifestation of primary Sjögren's syndrome. The most common histopathological lesion is an interstitial lymphocytic infiltrate with tubular atrophy and fibrosis. In addition, immune complex associated glomerulonephritis has been reported in sporadic cases with primary Sjögren's syndrome. Here we present our experience on kidney involvement in 36 Greek patients with primary Sjögren's syndrome. The following routine renal laboratory tests were performed in all patients: serum creatinine, creatinine clearance, 24 hour urine protein excretion, urinalysis and plain film of the abdomen. These tests revealed two cases with nephrolithiasis and two with severe proteinuria (24 hour urine protein greater than 3.5 g). Kidney histology in the patients with proteinuria was compatible with membranous and membranoproliferative glomerulonephritis respectively. In fifteen randomly selected patients urinary acidification was studied before and after acute acid loading test (oral administration of NH4Cl). Renal tubular acidosis was found in five patients (one with complete and four with incomplete type). Seven patients voluntarily accepted renal biopsy; five of those had mild form of interstitial nephritis. Two of these patients had renal tubular acidosis. In conclusion, renal involvement (clinical and subclinical) is commonly found in primary Sjögren's syndrome patients.
Acidosis, Renal Tubular↗
Renal distal tubular acidosis in obstructive nephropathy.
The acidifying capacity of the kidney was studied in patients with obstructive nephropathy. After a loading test with ammonium chloride, incomplete renal tubular acidosis (RTA) could be observed in 22.5% and stone formation in 30% of the patients with renal tubular acidosis. Attention is called to the fact that, in addition to the known anatomical and physicochemical factors, the alkali fixed urine due to incomplete tubular acidosis due to urinary tract obstruction also predisposes to calculus formation.
Acidosis, Renal Tubular↗