oxalates and Cystinuria

Topics: Acidosis, Renal Tubular; Amino Acids; Calcium; Carbohydrate Dehydrogenases; Carrier Proteins; Chloride Channels; Cystinuria; Humans; Hyperoxaluria, Primary; Hypoxanthine Phosphoribosyltransferase; Mutation; Organic Anion Transporters; Organic Cation Transport Proteins; Oxalates; Proteinuria; Proton-Translocating ATPases; Purines; Transaminases; Uric Acid; Urinary Calculi

Renal stones have afflicted humans for millennia but there is still no solution to this problem. This review discusses the laboratory and metabolic aspects of the clinical management of patients with renal stones, both primary and secondary in origin. First, non-pharmacological interventions such as increased fluid intake, decreased protein consumption, dietary changes in sodium, calcium, oxalate, potassium, purine, vitamins, and essential fatty acids are considered. Then specific pharmacological treatment to modify urine calcium, oxalate, urate, citrate, and acidity are considered. Finally, more unusual types of stone are examined.

Topics: Acidosis, Renal Tubular; Calcium; Calcium, Dietary; Citrates; Cystinuria; Diet, Protein-Restricted; Humans; Inflammatory Bowel Diseases; Kidney Calculi; Oxalates; Uric Acid; Urinalysis

Topics: Cystinuria; Humans; Metabolic Diseases; Nephritis, Interstitial; Oxalates; Uric Acid

Topics: Calcium; Citric Acid; Cystinuria; Humans; Kidney Calculi; Magnesium Compounds; Oxalates; Phosphates; Struvite; Uric Acid

Topics: Antacids; Benzothiadiazines; Calcium; Cation Exchange Resins; Cellulose; Citrates; Citric Acid; Cystinuria; Diuretics; Humans; Kidney Calculi; Lithotripsy; Magnesium; Oxalates; Penicillamine; Phosphates; Sodium Chloride Symporter Inhibitors; Uric Acid; Urinary Tract Infections; Urine

Whereas the etiology of urinary calculi and their metabolic exploration should be known, it appears unreasonable to conduct exhaustive metabolic explorations in all patients, therapy being usually symptomatic and based on advice on hygiene and diet. However, etiologic diagnosis is essential in a small number of cases: those which could benefit from effective preventive and curative measures and for which morbidity is elevated: cystinuria, hyperparathyroidism, uric acid calculi, patients at high developmental risks. It is possible, by simple, low cost means to select 95% of these patients. After a theoretical study of the distribution and lithiasic etiology, a practical conduct is proposed which takes into account the cost-effective ratio.

Topics: Acidosis; Calcium; Citrates; Cystinuria; Humans; Hypercalcemia; Hyperparathyroidism; Intestinal Absorption; Kidney Calices; Osteoporosis; Oxalates; Sarcoidosis; Uric Acid; Urinary Calculi

Advances in renal lithiasis research have contributed to a better understanding of the many varied factors that contribute to renal calculus formation. Utilizing the newer techniques of ambulatory metabolic evaluation, we can establish a specific diagnosis in 95% of recurrent stone-formers. Since a significant percentage of initial stone-formers will never have a second episode, it is essential to establish the natural history of the patient's stone disease prior to initiating potentially life-long medical therapy. The majority of initial stone-formers can be managed with education concerning modest dietary restrictions and increased fluid intake. For the recurrent stone-former with metabolically active stone disease, it is probably best to design medical therapy to treat the specific urinary chemical abnormality or disease process.

Topics: Acidosis, Renal Tubular; Bacterial Infections; Calcium; Crystallography; Cystinuria; Diuresis; Fluid Therapy; Humans; Intestinal Absorption; Kidney; Kidney Calculi; Magnesium; Magnesium Compounds; Oxalates; Patient Education as Topic; Phosphates; Recurrence; Risk; Struvite; Uric Acid; Urography

Topics: Calcium; Citrates; Clinical Trials as Topic; Cystine; Cystinuria; Humans; Hydrogen-Ion Concentration; Kidney Calculi; Oxalates; Oxalic Acid; Uric Acid

Topics: Adolescent; Adult; Age Factors; Calcium; Child; Child, Preschool; Cystinuria; Female; Humans; Hydrogen-Ion Concentration; Male; Middle Aged; Oxalates; Phosphates; Sex Factors; Uric Acid; Urinary Calculi

Topics: Acidosis, Renal Tubular; Allopurinol; Benzothiadiazines; Calcium; Calcium, Dietary; Cystinuria; Diuretics; Humans; Hyperparathyroidism; Kidney Calculi; Magnesium; Magnesium Compounds; Oxalates; Penicillamine; Phosphates; Quaternary Ammonium Compounds; Sodium Chloride Symporter Inhibitors; Struvite; Uric Acid

Topics: Amino Acids; Biological Transport; Calcium Oxalate; Cystinuria; Dehydration; Digestive System; Glutamine; Hartnup Disease; Humans; Kidney Calculi; Niacinamide; Oxalates; Solubility; Tryptophan

Topics: Calcium; Cystine; Cystinuria; Humans; Nutritional Physiological Phenomena; Oxalates; Phosphates; Uric Acid; Urinary Calculi

Topics: Adenine Phosphoribosyltransferase; Cystinuria; Glycogen Storage Disease Type I; Humans; Hypoxanthine Phosphoribosyltransferase; Kidney Calculi; Metabolism, Inborn Errors; Orotic Acid; Oxalates; Ribose-Phosphate Pyrophosphokinase; Uric Acid; Xanthine Oxidase; Xanthines

Management of most patients with calculous disease has been less than ideal in the past. Too often therapeutic efforts were limited to symptomatic calculi. Stones were allowed to pass or were removed, metabolic studies were incomplete, victims were dismissed and forgotten, and prophylactic measures were negligible and usually confined to milk restriction and use of distilled water. More patients were crippled with and died of recurrent calculous disease, urinary infection and progressive renal insufficiency than from any other upper urinary tract abnormality. During the last decade the development of a renal stone clinic at this institution has allowed a nephrourologic approach to the management of urolithiasis. This account of classification, diagnosis and management of the various syndromes associated with urolithiasis is based on the experiences gained during the last decade with this common but ill-understood urologic problem.

Topics: Acidosis, Renal Tubular; Adult; Child, Preschool; Cystinuria; Female; Humans; Hypercalcemia; Intestinal Diseases; Kidney Calculi; Male; Middle Aged; Nephrocalcinosis; Oxalates; Sepsis; Uric Acid; Xanthines

The identification of a disease entity as one that is the result of a heritable defect offers the physician an opportunity to intervene in a variety of ways. As emphasized, knowledge of the heritable pattern of a particular disease allows the physician an opportunity to counsel family members in personal disease risk and the offspring. Such genetic counseling results in a reduction of affected cases for many inherited diseases. There is every expectation that similar approaches would be effective for inherited renal diseases. The heritable diseases are a favored group for investigative purposes since these diseases result from a single gene defect no matter how plieotropic the effects of that defect. Thus the investigator is capable of constant probing with tools available for identifying that one event or component that lies at the basis of the disease. The emphasis of this chapter is on those inherited renal diseases for which we have reached a high level of understanding of this single defect. In many of these diseases a single enzyme is identified as deficient and is the presumed genetic defect. In others (cystinuria, RTA, and cystinosis) the precise biochemical answers appear close at hand. Thus a variety of therapeutic approaches to overcome either the gene defect or ill effects of the gene defect emerge for diseases involving the kidney and are listed in Table 7. For some of these diseases the new diagnostic technique of prenatal diagnosis can be used (Table 8). This genetic option provides couples at risk for bearing affected offspring with reduced risk. For a number of other diseases that are not identified by amniocentesis, this risk can be effectively lowered to acceptable levels by use of artificial insemination. Thus the inherited diseases of the kidney are amenable to medical intervention at a variety of levels. Such intervention can predictably lead to a lowering of both the incidence and consequences of these gene defects.

Topics: Acidosis, Renal Tubular; Adult; Child; Chromosome Aberrations; Chromosome Disorders; Cystinosis; Cystinuria; Diabetes Insipidus; Fanconi Syndrome; Female; Genes, Dominant; Genes, Recessive; Glycosphingolipids; Humans; Infant, Newborn; Kidney; Kidney Diseases; Kidney Diseases, Cystic; Lesch-Nyhan Syndrome; Lipid Metabolism, Inborn Errors; Male; Metabolism, Inborn Errors; Middle Aged; Nephritis; Orotic Acid; Oxalates; Polycystic Kidney Diseases; Pseudohypoparathyroidism; Sex Chromosome Aberrations; Xanthines

Topics: Acidosis, Renal Tubular; Alkaptonuria; Cystinuria; Diabetes Insipidus; Female; Glycine; Gout; Humans; Hyperlipidemias; Hyperparathyroidism; Kidney Diseases; Lesch-Nyhan Syndrome; Male; Metabolism, Inborn Errors; Nephritis, Hereditary; Oxalates; Porphyrias; Uric Acid; Urologic Diseases; Xanthines

Topics: Acidosis, Renal Tubular; Bacterial Infections; Citrates; Crystallization; Cystinuria; Diphosphates; Female; Gastrointestinal Diseases; Humans; Hypercalcemia; Hyperparathyroidism; Magnesium; Male; Metabolism, Inborn Errors; Mucoproteins; Oxalates; Quaternary Ammonium Compounds; Sarcoidosis; Solubility; Uric Acid; Urinary Calculi; Vitamin D; Xanthine Oxidase

Topics: Acid-Base Equilibrium; Age Factors; Calcium; Cystinuria; Female; Humans; Hypercalcemia; Hyperparathyroidism; Immobilization; Intestinal Absorption; Kidney Calculi; Male; Oxalates; Phosphates; Sex Factors; Uric Acid; Vitamin D

Topics: Calcium; Cystinuria; Diphosphates; Humans; Magnesium; Oxalates; Phosphates; Urea; Urinary Calculi

Topics: Ammonia; Calcium Metabolism Disorders; Chemistry Techniques, Analytical; Cystinuria; Diagnosis; Humans; Kidney Calculi; Oxalates; Statistics as Topic; Surgical Procedures, Operative; Therapeutics; United States; Urine; Xanthines

We investigated ascorbic acid therapy for cystinuria in a study of seven healthy control persons and seven cystinuric patients. The study lasted 9 days. During the first period, we collected 24-h urine specimens from all subjects on 3 consecutive days. Starting on day 4, all were given 5 g ascorbic acid/day for a period of 6 days. On the last 3 days, 24-h urine specimens were again collected. Quantitative amino acid determination was performed using an HPLC method described elsewhere. During ingestion of ascorbic acid, the mean excretion of cysteine by the control group increased from 134.1 to 159 mumol/day, whereas the excretion of cystine decreased from 107.1 to 82 mumol/day. The corresponding values for the cystinuric patients increased from 352.4 to 452.1 mumol/day for cysteine and decreased from 4,131.6 to 3,663.2 mumol/day for cystine. Thus, ascorbic acid seems to have only mild reducing properties in respect to cystine.

Topics: Ascorbic Acid; Chromatography, High Pressure Liquid; Cysteine; Cystinuria; Humans; Oxalates; Oxalic Acid

17 cystine stone patients were treated with high doses of ascorbic acid (5 g p. d.). During the observation period, a total of only two natural passages of cystine stones could be observed. For five patients the therapeutic strategy was altered because the recurrence rate did not change and the cystine concentration in the urine was enhanced. One mixed calcium-oxalate/cystine stone had to be resected. In this case as well as on the occasion of further medical check-ups of other patients, an increased risk of calcium-oxalate stone formation was signalled by an enhanced oxalic-acid concentration in the 24-hour urine. Changes in blood serum and impairment in hepatic and renal functions were not observed. With three patients, the therapy had to be interrupted because of gastritis symptoms. The use of high-dose ascorbic acid therapy is recommended and is continued. In special cases, an additive of low do ses of alpha-mercaptopropionyl-glycine is recommended.

Topics: Adult; Aged; Ascorbic Acid; Child; Clinical Trials as Topic; Cystinuria; Disease Susceptibility; Dose-Response Relationship, Drug; Female; Humans; Hydrogen-Ion Concentration; Kidney Calculi; Long-Term Care; Male; Middle Aged; Oxalates; Oxalic Acid; Recurrence

Topics: Calcium; Citrates; Clinical Trials as Topic; Cystine; Cystinuria; Humans; Hydrogen-Ion Concentration; Kidney Calculi; Oxalates; Oxalic Acid; Uric Acid

Urinary calculi in infants are relatively infrequent, but their incidence has increased in the recent decades. The aim of this study was to investigate the clinical presentation, metabolic risk factors, and urinary tract abnormalities in infants suffering from kidney calculus.. A total of 152 infants were admitted between 2009 and 2012 with ultrasonography-proven urolithiasis. A Foley catheter was fixed and 24-hour urine samples were analyzed for calcium, citrate, oxalate, uric acid, and magnesium. For detecting cystinuria, qualitative measurement of urinary cystine was done by nitroprusside test. Urinary tract structural abnormalities were also evaluated.. The mean age at the diagnosis of kidney calculus was 5.46 months (range, 15 days to 12 months). The most common clinical findings were restlessness and urinary tract infection. A family history of calculi was found in 67.1% of the patients and 68.4% were born to consanguineous marriages. Metabolic abnormalities and urinary tract abnormalities were found in 96.1% and 15.1% of children, respectively. Urinary tract abnormalities were more common in girls. The most common metabolic risk factors were hypercalciuria (79.6%) and hypocitraturia (40.9%). Hyperoxaluria and hypomagnesuria were found in about 28% of patients, both of which were associated with bilateral urolithiasis.. These findings show that urinary metabolic abnormalities are very common in infants with urolithiasis. Appropriate evaluation of urinary metabolic parameters can lead us to proper diagnosis and treatment.

Topics: Calcium; Citric Acid; Cystinuria; Female; Humans; Infant; Infant, Newborn; Iran; Magnesium; Male; Oxalates; Risk Factors; Ultrasonography; Uric Acid; Urinary Calculi; Urinary Tract; Urolithiasis

We evaluated the clinical, radiological and metabolic features of 162 children with urolithiasis or microlithiasis who had been referred to our pediatric nephrology clinics between 1998 and 2008 with suspected urolithiasis. The medical histories of these children (78 girls, 84 boys), who ranged in age from 2 months to 16 years (mean age 5.59 +/- 0.35 years), were reviewed retrospectively for clinical and metabolic features of urinary tract calculi. Urinary tract infections (UTI) were present in 45.9% of the cases. The most common presenting symptoms were flank pain or restlessness (25.3%) and hematuria (21.6%), followed by UTI (16%), whereas 23.5% of the cases were detected incidentally during evaluation for other medical conditions. Other symptoms at presentation included dysuria, passing stones, penile edema, enuresis, vomiting and anorexia. Urine analysis revealed metabolic abnormalities in 87% of the cases, including hypercalciuria (33.8%), hypocitraturia (33.1%), hyperoxaluria (26.5%), hyperuricosuria (25.4%), hypocitraturia + hypercalciuria (21.1%), hyperphosphaturia (20.8%) and cystinuria (5.7%). Almost 50% of the patients had a positive family history for urolithiasis. The most frequently involved site was in the kidneys (86%). Ureters and bladder were involved in 12 and 2% of the cases, respectively. A family history of urolithiasis, presenting symptoms and underlying metabolic abnormalities were similar for microlithiasis and the patients with larger stones. However, in our study population, microlithiasis was mainly a disease of young infants, with a greater chance for remission and often not associated with structural changes. The presenting symptoms of urolithiasis show a wide spectrum, so that a high index of suspicion is important for early detection. A metabolic abnormality can be identified in 87% of cases of urolithiasis. Detection of microlithiasis may explain a number of symptoms, thus reducing invasive diagnostic procedures and allowing early recognition of metabolic abnormalities. These results draw attention to the importance of screening for UTIs in patients with urolithiasis.

Topics: Calcium; Child; Child, Preschool; Citric Acid; Cystinuria; Female; Humans; Hypercalciuria; Hyperoxaluria; Hypophosphatemia, Familial; Infant; Male; Metabolic Diseases; Oxalates; Phosphates; Retrospective Studies; Uric Acid; Urinary Calculi; Urinary Tract Infections; Urolithiasis

A preliminary diagnosis of the disease responsible for the stones is essential to allow appropriate medical treatment of renal stones. In this paper, the authors describe their diagnostic and treatment protocol based on computer-assisted urinary metabolic analysis. 413 subjects, divided into four groups, were prospectively evaluated to calculate their specificity, sensitivity, reproducibility and efficacy and to demonstrate the usefulness of this protocol.

Topics: Absorption; Adolescent; Adult; Aged; Calcium; Citrates; Citric Acid; Clinical Protocols; Creatinine; Cystinuria; Diagnosis, Computer-Assisted; Female; Follow-Up Studies; Humans; Hyperoxaluria; Kidney Calculi; Magnesium; Male; Metabolic Diseases; Middle Aged; Oxalates; Oxalic Acid; Prospective Studies; Reproducibility of Results; Sensitivity and Specificity; Uric Acid

The increasing incidence of urolithiasis makes it important to report about 34 children with urolithiasis seen between 1976 and 1986 at the Department of Pediatrics, University Medical School Vienna. At the time of the first diagnosis 59 percent of the patients were less than 7 years of age; 62 percent of our patients were males. Recurrent chronic urinary tract infection in 32 percent, metabolic disorder (secondary hyperoxaluria 5, idiopathic hypercalciuria 3, cystinuria 2, hyperuricuria 2) in 27 percent were evaluated; in 13 patients the origin of calculi was idiopathic. Most infectious stones contained magnesium ammonium phosphate, most idiopathic stones calcium oxalate. In 21 patients (62%) surgical treatment, in one patient extracorporal shock wave lithotripsie was realized. Adequate metaphylaxis (general, dietetic, medicementous) can lower the rate of occurrence of stone formation.

Topics: Adolescent; Child; Child, Preschool; Cystinuria; Diagnosis, Differential; Female; Humans; Male; Oxalates; Phosphates; Risk Factors; Uric Acid; Urinary Calculi

This article contains an analysis of data compiled from 813 specimens of canine uroliths submitted to the Urinary Stone Analysis Laboratory at University of California School of Veterinary Medicine.

Topics: Animals; Apatites; Calcium Phosphates; Cystine; Cystinuria; Dog Diseases; Dogs; Magnesium; Magnesium Compounds; Minerals; Oxalates; Phosphates; Silicon Dioxide; Struvite; Uric Acid; Urinary Calculi

Topics: Adolescent; Adult; Aged; Calcium; Calcium Phosphates; Child; Cystinuria; Diet; Disease Susceptibility; Female; Humans; Magnesium; Magnesium Compounds; Male; Middle Aged; Oxalates; Phosphates; Risk; Struvite; Uric Acid; Urinary Calculi

During 5 years, between 1978 and 1982, 40 patients (22 male, 18 female) aged 1 to 17 years presented with kidney stones at the Erlangen University Children's Hospital. Stone analysis showed calcium oxalate in 61%, calcium phosphate respectively magnesiumammonium phosphate in 33% and cystine in 6%. Calcium phosphate/ magnesiumammonium phosphate stone bearers were most frequently found in the first 5 years of age and showed more often urinary tract malformations, staghorn renal calculi, urinary tract infections and a higher urinary pH. Calcium oxalate stone bearers were more frequently found in patients of school age and had more often relatives with nephrolithiasis. In the group of calcium oxalate stones hypercalciuria, hyperoxaluria and hyperuricosuria were most frequently observed.

Topics: Adolescent; Calcium; Child; Child, Preschool; Cystinuria; Diagnosis, Differential; Female; Humans; Hydrogen-Ion Concentration; Infant; Kidney Calculi; Magnesium; Male; Oxalates; Oxalic Acid

A procedure for the routine investigation of urinary calculi is described. The investigation scheme was based on existing methods in the routine laboratory for calcium(II), magnesium(II), phosphate and urate in serum. The substance content of oxalate was calculated as non-phosphate-bound calcium(II). Furthermore the test for cystine in urine was utilized. Two specially designed tests were used, one for carbonate apatite and one for verification of the presence of oxalate. The scheme was applied to 30 specimens of human origin. The sum of the mass fractions of identified and calculated components in the calculi was found to be 0.88 on the average (s = 0.05). Low values for this sum may serve as an indication of the presence of rare components that are not included in this analytical programme. A few calculi containing rare components required special methods for the investigation and are most conveniently investigated in a specialized laboratory using X-ray diffraction or infra-red spectrometry. In this paper, however, we describe wet chemistry methods, suitable for reliable cystine determinations and for oxalate in calculi containing, for example, brushite, where the calculated oxalate value is uncertain. These methods may be used as an alternative to the physical methods for many of the rare calculi.

Topics: Autoanalysis; Calcium; Chemical Precipitation; Chemistry Techniques, Analytical; Chemistry, Clinical; Cystine; Cystinuria; Humans; Indicators and Reagents; Oxalates; Phosphates; Reference Values; Spectrum Analysis; Uric Acid; Urinary Calculi

This discussion was selected from the weekly Grand Rounds in the Department of Medicine, University of Washington, Seattle. Taken from the transcription, it has been edited by Drs Paul G. Ramsey, Assistant Professor of Medicine, and Philip J. Fialkow, Professor of Medicine and Chairman of the Department of Medicine.

Topics: Acidosis, Renal Tubular; Calcitriol; Calcium; Cystinuria; Humans; Kidney Calculi; Oxalates; Uric Acid

To evaluate the incidence of heterozygous cystinuria in patients with primary and recurrent calcium oxalate stone disease 24-hour urine samples from 140 patients were examined. The frequency of heterozygous cystinuria in this study was only 1.4 per cent (2 patients). The frequency of other metabolic disorders was in accordance with other reports: hypercalciuria (32 per cent), hyperuricosuria (32 per cent) and hyperoxaluria (17 per cent).

Topics: Adult; Aged; Calcium; Calcium Oxalate; Cystinuria; Female; Heterozygote; Humans; Male; Middle Aged; Oxalates; Oxalic Acid; Uric Acid; Urinary Calculi

We report on our experience with 9 cystine-lithiasis patients who were treated with large doses of ascorbic acid (5 g/day) for periods ranging from 6-27 months. We observed recidive lithogenesis in only 3 patients during this time. The influence of ascorbic acid on the excretion of cystine and oxalate in the urine is discussed. A lack of side effects and the significantly lower frequency of recidivation justify the further use of ascorbic as an alternative medication in cystine lithiasis.

Topics: Adult; Ascorbic Acid; Cystine; Cystinuria; Dose-Response Relationship, Drug; Female; Humans; Kidney Calculi; Male; Middle Aged; Oxalates; Oxalic Acid; Recurrence; Urinary Bladder Calculi; Urinary Calculi

Urolithiasis in children is a very important problem from the theoretical as well as from practical point of view. Better knowledge of its metabolic basis may allow to move the central treatment from surgical to prophylactic methods. The study presents modern view points on mechanisms for concrements sedimentation and actual data on metabolic disturbances in calcium, oxalates, purines, xanthines and cystine and their relations to urolithiasis and urinary tract infection.

Topics: Acidosis, Renal Tubular; Calcium; Child; Cystinuria; Humans; Hypercalcemia; Oxalates; Purine-Pyrimidine Metabolism, Inborn Errors; Uric Acid; Urinary Calculi; Urinary Tract Infections; Xanthines

The analysed material includes 100 children with urolithiasis treated in the Pediatric Clinic of the National Research Institute of Mother and Child in Warsaw between 1976 and 1978. Patients' age was from 3 months to 18 years. The analysed group included 51 boys and 49 girls. Urinary tract infection was found in 54 cases, i.e. 57,4% of the analysed material. The most common bacterial strains were those producing urease. They were detected in 48 children i.e. 88,9% of cases with urinary tract infection. Mostly these were bacteria of Proteus group--sporadically Pseudomonas aeruginosa and Staphylococcus albus. In the analysed patients urinary tract obstruction was observed in 36 children, i.e. 36% of cases. In 77% of the analysed material, localization of concrements was in upper urinary tract in 19% in the ureters and in 4% in the lover urinary tract. While in adult patients the most common compound of urinary stones was calcium oxalate, in children the most common stone compounds were phosphates (found in 38 cases i.e. 58,4% of the analysed material). The second frequent compound was oxalate found in 20 cases (30,7%). Less frequent compounds were uric acid and cystine. Performed study allowed to establish the cause of urolithiasis in 93 out of 100 examined children. Metabolic reasons of urolithiasis were found in 26 cases, i.e. 26% of the analysed material. They were as follows: idiopathic hypercalciuria--12 cases, uric acid urolithiasis--8 cases, primary hyperoxaluria--3 cases, cystinuria--2 cases, and incomplete acidosis of distal renal tubuli--1 case. Urolithiasis of probably metabolic origin was detected in 13 children (13%). Other reasons of urolithiasis in children were: infection (31%), idiopathic urolithiasis (17%) and others (6%). In 7 cases the reason of urolithiasis was not established.

Topics: Acidosis, Renal Tubular; Adolescent; Calcium; Child; Child, Preschool; Cystinuria; Female; Humans; Infant; Male; Oxalates; Uric Acid; Urinary Calculi; Urinary Tract Infections

The present study describes the mode of prophylactic management of urinary stone disease as carried out at the Department of Urology. University of Vienna Medical School. A carefully directed prophylactic regimen results in a significant decrease in the recurrence rate of stone formation.

Topics: Acidosis, Renal Tubular; Calcium; Cystinuria; Humans; Hydrogen-Ion Concentration; Oxalates; Recurrence; Uric Acid; Urinary Calculi

Topics: Acidosis, Renal Tubular; Bicarbonates; Calcium; Child; Cystinuria; Enzymes; Humans; Oxalates; Phosphates; Purines; Urinary Calculi; Xanthines

In an attempt to detect genetic factors linked with urolithiasis, a study based on medical and genetic data and on several biochemical procedures was done on 50 stone formers and on 50 controls. Genetic factors likely to be related to stone forming were found in 4 patients: 2 cases of incomplete renal tubular acidosis, and 2 cases of heterozygous cystinuria. A study of the families of 3 of these individuals revealed 4 additional cases of genetically determined metabolic diseases. Despite the small number of patients for whom genetic factors were determined and the fact that the lithiasis cases with and without family recurrence showed similar behavior with respect to the different biochemical parameters studied, the presence of genetic factors is suggested by the significantly more frequent family history of lithiasis found for stone formers than for the controls. Identifying the cases with family recurrence, in which stone formation occurs earlier and is more frequently recurrent, and the stone-forming patients with genetically determined metabolic disorders, which may benefit from specific measures, will probably contribute to a better prognosis for these patients.

Topics: Acidosis, Renal Tubular; Adolescent; Adult; Blood Chemical Analysis; Child; Cystinuria; Female; Humans; Male; Metabolism, Inborn Errors; Oxalates; Urinary Calculi

Topics: Calcium; Cyclic AMP; Cystinuria; Humans; Hydrogen-Ion Concentration; Hyperparathyroidism; Oxalates; Urinary Calculi; X-Ray Diffraction

Topics: Adult; Aged; Bacterial Infections; Calcium; Child; Cystinuria; Greece; Humans; Kidney Calculi; Oxalates; Uric Acid; Urinary Tract Infections

Topics: Acidosis, Renal Tubular; Asia, Western; Calcium; Cystinuria; Egypt; Europe; Female; Germany, East; History, 16th Century; History, 17th Century; History, 18th Century; History, 19th Century; History, Ancient; History, Medieval; Humans; Magnesium; Male; Oxalates; Phosphates; Uric Acid; Urinary Calculi; Xanthines

Topics: Adult; Calcium; Climate; Cystinuria; Female; Humans; Male; Oxalates; Phosphates; Uric Acid; Urinary Calculi; Urinary Tract Infections; Urination Disorders

Topics: Animals; Calcium; Calcium Oxalate; Calcium Phosphates; Cyclic AMP; Cystine; Cystinuria; Dehydration; Diet; Humans; Hydrogen-Ion Concentration; Hyperparathyroidism; Kidney Calculi; Male; Oxalates; Risk; Uric Acid; Urine

Determining metabolic activity in urolithiasis may avoid excessive laboratory tests and unnecessary treatment. A simple regimen such as increased oral fluids is often effective therapy in metabolically inactive disease. Most cases of renal lithiasis are idiopathic, but a complete examination and laboratory work-up will usually establish an accurate etiologic diagnosis.

Topics: Aged; Calcium; Calcium Metabolism Disorders; Cystinuria; Humans; Male; Medical History Taking; Oxalates; Physical Examination; Radiography; Uric Acid; Urinary Calculi; Xanthines

Topics: Acidosis, Renal Tubular; Aged; Calcium; Calcium, Dietary; Cystinuria; Diet; Humans; Intestinal Diseases; Intestine, Small; Kidney Calculi; Male; Oxalates; Phosphorus; Uric Acid; Urinary Calculi

The causes of, and physiopathological factors underlying the most common metabolic disorders implicated in the formation of renal stones are reviewed. These include hypercalciuria, hyperoxaluria, renal tubular acidosis, cystinuria and disturbances of purine metabolism. Apart from metabolic disorders the risk of stone formation is also influenced by a low inhibitor activity in urine. Though some aspects in the pathogenesis of urolithiasis remain uncertain, the exact knowlege of important aetiological factors of stone formation is the basis of correct treatment and the prevention of recurrence of urinary calculi.

Topics: Acidosis; Adult; Calcium; Cystinuria; Humans; Kidney Tubules; Male; Oxalates; Purine-Pyrimidine Metabolism, Inborn Errors; Uric Acid; Urinary Calculi; Urography

Topics: Calcium; Cystinuria; Diet; Humans; Hydrogen-Ion Concentration; Occupations; Oxalates; Risk; Stress, Psychological; Uric Acid; Urinary Calculi; Urinary Tract Infections

Topics: Adult; Calcium; Cystine; Cystinuria; Humans; Hypercalcemia; Kidney Calculi; Male; Middle Aged; Oxalates; Phosphates; Uric Acid

Topics: Acidosis, Renal Tubular; Adult; Calcium; Crystallization; Cystinuria; Female; Humans; Magnesium; Male; Oxalates; Phosphates; Uric Acid; Urinary Calculi; Xanthines

Topics: Ammonia; Calcium; Calcium Phosphates; Computers; Cystinuria; Humans; Kinetics; Magnesium; Mathematics; Oxalates; Phosphates; Risk; Sodium; Uric Acid; Urinary Calculi

Topics: Calcium; Crystallization; Cystine; Cystinuria; Humans; Kidney Calculi; Oxalates; Uric Acid

Any patient presenting with renal colic requires the taking of a detailed history-- with inquiries about diet and drugs, an examination of the urine, intravenous pyelography and measurement of the calcium concentration in serum and urine. Any stone passed should be analysed. Recurrent stone formation warrants more comprehensive metabolic investigation. The cause of renal calculi in most patients is still not known. When a cause is found, specific treatment can prevent or control stone formation. For the majority in whom no abnormality is detected, various methods of reducing stone formation have been tried but results are disappointing. The most important points in management are the early detection and effective treatment of urinary tract infection or obstruction and the maintenance of a high fluid intake.

Topics: Bacteriuria; Calcium; Child; Cystinuria; Humans; Kidney Calculi; Male; Oxalates; Uric Acid

Knowledge of the crystalline structure of the calculus provides the basis of the therapeutic plan. Laboratory evaluation depends heavily upon routine urinalysis. Assessment of renal function, serum calcium, phosphorus, uric acid and, in some cases electrolytes is usually indicated, as is urography. General principles of management include maintenance of an ample urine volume, eradication of infection and correction of any obstructing lesions or metabolic abnormalities. Specific antistone regimens are indicated for patients with recurrent urolithiasis.

Topics: Anti-Bacterial Agents; Calcium; Cystinuria; Diarrhea; Female; Humans; Hydrochlorothiazide; Intestinal Diseases; Kidney Calculi; Male; Oxalates; Phosphates; Proteus Infections; Uric Acid; Urinary Calculi

Topics: Calcium; Cystinuria; Glycosaminoglycans; Humans; Oxalates; Phosphates; Uric Acid; Urinary Calculi

Topics: Adult; Age Factors; Blood Chemical Analysis; Calcium; Child; Citrates; Cystinuria; Diet Therapy; Female; Humans; Hydrochlorothiazide; Hydrogen-Ion Concentration; Kidney Calculi; Malabsorption Syndromes; Male; Medical History Taking; Oxalates; Phosphorus; Racial Groups; Recurrence; Sex Factors; Uric Acid; Urinary Tract Infections; Urine; Urography

The management of 78 children with upper urinary calculi is described. Boys outnumbered girls by a ratio of 2 to 1. Two-thirds of the patients had identifiable metabolic causes, while the remaining third had infected renal lithiasis. In this latter group, all patients had had multiple urologic procedures, urinary infection, and stasis with diversionary and indwelling drainage devices. Contrary to earlier views, idiopathic renal lithiasis with or without hypercalciuria was the most common metabolic form of nephrolithiasis in children. Sixty-seven patients (86 per cent) were followed for an average of 7 1/2 years. With appropriate therapy, stone disease became inactive in 70 per cent of the children. The remaining 30 per cent continued with active disease--5 died of renal failure and 1 has received a renal allograft. Stone formation may be regarded as a solitary complication or one of several manifestations of a large number of underlying disorders. Along with a thorough search for etiologic factors there must be an equally aggressive therapeutic effort. Because the disease is ofter sporadic, careful long-term followup of the patients with active as well as those with inactive stone disease is mandatory.

Topics: Acidosis, Renal Tubular; Adolescent; Adrenocortical Hyperfunction; Age Factors; Calcium; Child; Child, Preschool; Cystinuria; Female; Humans; Hypercalcemia; Hyperparathyroidism; Infant; Infant, Newborn; Kidney Calculi; Magnesium; Male; Metabolic Diseases; Oxalates; Uric Acid; Urinary Tract Infections; Urography

Topics: Acidosis, Renal Tubular; Acute Disease; Analgesics; Calcium; Chronic Disease; Cystinuria; Female; Humans; Hyperparathyroidism; Infrared Rays; Kidney Calculi; Male; Oxalates; Radiography; Spectrum Analysis; Ureteral Calculi; Urinary Calculi; Urinary Tract Infections; X-Ray Diffraction

Topics: Adolescent; Adult; Aged; Ammonia; Calcium; Cystinuria; Female; Humans; Kidney Calculi; Magnesium; Male; Oxalates; Phosphates; Ureteral Obstruction; Uric Acid; Urinary Tract Infections

Topics: Adult; Aged; Calcium; Cystinuria; Female; Humans; Hypercalcemia; Hyperglycemia; Male; Metabolic Diseases; Middle Aged; New Zealand; Oxalates; Phosphates; Prospective Studies; Radiography; Uric Acid; Urinary Calculi; Urinary Tract Infections

Topics: Calcium; Crystallization; Cystinuria; Diet Therapy; Humans; Kidney Calculi; Oxalates; Recurrence; Uric Acid; Urinary Tract Infections

Topics: Apatites; Calcium; Chemical Phenomena; Chemistry, Physical; Cystine; Cystinuria; Female; Humans; Kidney Calculi; Male; Methods; Oxalates; Spectrophotometry, Infrared; Temperature; Uric Acid; X-Ray Diffraction

Topics: Adult; Bicarbonates; Crystallography; Cystine; Cystinuria; Hematuria; Humans; Hydroxyapatites; Kidney Calculi; Male; Oxalates; Radiography; Water; X-Ray Diffraction

Topics: Acidosis, Renal Tubular; Calcium; Child; Cystinuria; Female; Hematuria; Humans; Male; Metabolic Diseases; Oxalates; Parathyroid Neoplasms; Retrospective Studies; Ureteral Obstruction; Uric Acid; Urinary Calculi; Urinary Tract Infections; Urography

Topics: Acidosis, Renal Tubular; Adult; Calcium; Cystinuria; Diphosphates; Female; Humans; Hyperparathyroidism; Male; Nephrocalcinosis; Oxalates; Peptides; Urinary Bladder Calculi

Topics: Adult; Cystine; Cystinuria; Diet Therapy; Female; Humans; Kidney Calculi; Oxalates; Pregnancy; Urography

10 families with cystinuria were investigated by measuring: (a) quantitative 24 hr urinary excretion of amino acids by column chromatography; (b) endogenous renal clearances of amino acids and creatinine; (c) intestinal uptake of (34)C-labeled L-cystine, L-lysine, and L-arginine using jejunal mucosal biopsies; (d) oral cystine loading tests. All four of these were studied in the probands and the first two in a large number of the family members.49 members of 8 families were found to have a regular genetic pattern as described previously by Harris, Rosenberg, and their coworkers. Clinical or biochemical differences between the homozygotes type I (recessive cystinuria) and homozygotes type II (incompletely recessive cystinuria) have not been found. Both types excreted similarly excessive amounts of cystine, lysine, arginine, and ornithine, and had high endogenous renal clearances for these four amino acids. Some homozygotes of both types had a cystine clearance higher than the glomerular filtration rate. Jejunal mucosa from both types of homozygotes exhibited near complete inability to concentrate cystine and lysine in vitro. This was also documented in vivo with oral cystine loads. The heterozygotes type I were phenotypically normal with respect to the above four measurements. The heterozygotes type II showed moderate but definite abnormalities in their urinary excretion and their renal clearances of dibasic amino acids. Of the four amino acids concerned, cystine was the most reliable marker to differentiate between the heterozygotes type II and the homozygous normals. In this study, type III cystinuria, as described by Rosenberg, was not encountered. In two additional families, double heterozygotes of genotype I/II were found. The disease affecting these is clinically and biochemically less severe than that affecting homozygotes of either type I or type II. With respect to the four parameters used in this study, the double heterozygotes type I/II have results which are intermediate between those of the homozygotes type I and II and those of the heterozygotes type II.

Topics: Adolescent; Adult; Amino Acids; Arginine; Biological Transport; Calcium; Carbon Isotopes; Child; Child, Preschool; Creatinine; Cystine; Cystinuria; Female; Heterozygote; Humans; Intestinal Absorption; Jejunum; Kidney; Lysine; Male; Middle Aged; Ornithine; Oxalates; Pedigree; Phosphates; Uric Acid

Topics: Allopurinol; Calcium; Creatinine; Cystinuria; Diuresis; Humans; Kidney; Kidney Calculi; Magnesium; Metabolic Diseases; Methylene Blue; Oxalates; Penicillamine; Phosphates; Uric Acid; Urinary Tract Infections; Urine; Water-Electrolyte Balance

Topics: Cystine; Cystinuria; Humans; Lithiasis; Medical Records; Oxalates; Urinary Calculi

Topics: Cystine; Cystinuria; Humans; Lithiasis; Medical Records; Oxalates; Radiology; Urinary Calculi