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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Urolithiasis in pediatric patients has been perceived as uncommon, and the appropriate evaluation and management have been controversial. To determine the clinical characteristics, types of stone problems, and outcomes of pediatric patients with urolithiasis encountered in a referral center, we retrospectively assessed 221 patients (113 girls and 108 boys) with urolithiasis examined at the Mayo Clinic between 1965 and 1987. The median age at onset of symptoms was 11 6/12 years among the female patients and 10 6/12 years among the male patients. Analysis of stone constituents in 122 patients showed the proportion of calcium oxalate (44.7%), calcium phosphate (23.6%), and cystine (8.1%) stones to be similar in all age-groups. Overall, struvite stones were found in 17.1% and uric acid stones in 1.6% of patients. Conditions that predisposed to metabolic urolithiasis were identified in 115 patients (52%). Hypercalciuria was confirmed in 49 of 145 patients (33.8%) and hyperoxaluria in 25 of 124 (20.2%). Eight of 96 patients had hyperuricosuria, and 5 of 54 had hypocitraturia. Forty-one patients (18.6%) had infection-related stones. Of 66 patients with structural anomalies of the genitourinary tract, 24 (36%) had metabolic abnormalities and 26 (39%) had chronic infection. Among patients with chronic infection, 29% had metabolic abnormalities. Of the 221 patients, 148 (67%) had two or more stones during a mean follow-up of 59 months. Among 140 patients with 12 months or more of follow-up, metabolic activity was present in 31 (22.1%) at the time of most recent examination. Overall, 166 of 221 children (75.1%) were found to have factors that predisposed to urolithiasis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Urolithiasis in pediatric patients. 847 76

About 80% of all renal stones contain calcium oxalate and/or calcium phosphate as their main crystalline components. The most important risk factors for increases in calcium oxalate crystallization are low urine volume, hyperoxaluria and hypocitraturia. Hypercalciuria, however, is of secondary importance as a cause of increased crystallization: whereas calcium and oxalate crystallize in a 1:1 ratio, the molar concentration ratio in urine amounts to about 10:1 in favor of calcium. Therefore, increases in urinary calcium will not be followed by a rise in crystallization as long as oxalate remains constant, whereas even the slightest increases in urinary oxalate immediately cause more crystals to precipitate. Thus, low calcium diet is not only unnecessary but is contraindicated since it may cause secondary hyperoxaluria (increased intestinal oxalate absorption) and osteopenia (negative calcium balance). On the other hand, overconsumption of animal protein (meat, poultry, fish) induces more pronounced hyperoxaluria and hypocitraturia and contributes to an overall negative calcium balance. It is, however, only by the interplay of "bad" dietary habits with underlying abnormalities such as up-regulation of calcitriol production, incomplete renal tubular acidosis or defective macromolecular crystallization inhibitors, that people become recurrent calcium renal stone formers.
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PMID:[Diagnostic markers in calcium nephrolithiasis--current and traditional ideas with a new look]. 857 Oct 96

The introduction of renal ultrasound technology has shown renal calcification to be more common in infancy than was previously believed. Understanding the role of inhibitors and promoters in crystal formation helps elucidate the pathophysiology of nephrocalcinosis. Identification of the presence or absence of hypercalcemia and hypercalciuria is an effective way to direct the diagnostic work-up of infants with nephrocalcinosis. The sonographic image of renal calcification resolves spontaneously in many infants. Whether microscopic nephrocalcinosis persists below the threshold of ultrasonographic detection is unknown. Renal calcification can be associated with persistent renal function abnormalities if hypercalciuria continues, such as in VLBW infants who receive long-term furosemide therapy after discharge from the hospital. Renal calcification may also progress to renal failure, such as in infants with primary hyperoxaluria, owing to the persistence of hyperoxaluria, a potent promoter of calcium crystal formation.
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PMID:Renal calcification in the first year of life. 861 92

Aim of the study was to establish normal values for calcium/creatinine (Ca/cr) and oxalate/creatinine (Ox/cr) ratio in infants and children. Urine probes of 416 healthy children (25 infants aged 1-7 days and 391 children aged 1 month-14.5 years) were analysed. Oxalate was measured by ion-chromatography. Urinary Ca2+/cr was normally distributed, Ox/cr had log-normal distribution. Ca/cr was the lowest in the first days of life, the highest between 7 month-1.5 years (mean +/- SD = 0.39 +/- 0.28 mmol/mmol), a slight decrease could be observed until 14 years (0.34 +/- 0.18). The highest Ox/cr values were measured during the first month of life (geometric mean/range/ = 133 /61-280 mmol/mmol/), followed by gradual decrease until 14 years (25/6-73/). The measurement of Ca2+/cr and Ox/cr in first morning urine samples is suitable for screening of hypercalciuria and hyperoxaluria. The interpretation of the values requires age specific reference values. Both calcium and oxalate determinations should be the part of the evaluation of patients with hematuria, hypercalciuria or nephrolithiasis.
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PMID:[Normal values of calcium and oxalate excretion in children]. 865 14

Presentation of the analytical results from the patients seen for lithiasic disease (LD) over a two-year period at the Hospital Reina Sofia, Tudela. This Hospital covers a homogeneous Health Area including 22 villages and a population of 76,000 people. The clinical cases of 785 patient diagnosed with LD between May 1988 and 1990 May are analyzed. Microhaematuria in fresh urine is detected in 64.20% patients and crystalluria in 33.37%. Significant bacteriuria is present in 5.73% of total patients with prevalence of E. coli in 42.4%. Only 2 cases of hyperparatiroidism were diagnosed during the study period but later another two cases of HPT were detected in bone injuries studied due to rheumatic disease. No normocalcemic HPT cases were diagnosed among suspected cases. The metabolic studies were of little use in our experience, maybe because of non-availability of basic analytical determinations such as citraturia. Nevertheless, higher values of urinary volume, calciuria and uricemia and lower values of magnesemia and magnesiuria were found in lithiasic patients that in control ones. Neither oxaluria or the remaining analytical parameters provide differential data. Hypercalciuria higher than 300 mg in seen in 28.6% of studied patients and in 12.5% of the control group.
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PMID:[Epidemics of urinary calculi in la Ribera de Navarra (II). Analytic studies]. 866 30

An abnormal erythrocyte transmembrane oxalate flux was described in recurrent idiopathic calcium nephrolithiasis. To verify whether it might represent a risk marker of renal stone disease, two prospective studies were carried out. One hundred ninety patients with idiopathic calcium nephrolithiasis who were enrolled at their first episode of lithiasis during the period 1984 to 1986, form the basis of the first prospective study. The impact of erythrocyte oxalate transport anomaly, gender, familial occurrence of nephrolithiasis, hypercalciuria, hyperoxaluria, and hyperuricosuria on stone recurrence by both bivariate and multivariate analysis of frequencies was assessed. The predictive value of the erythrocyte anomaly for a patient's becoming a stone former was also assessed in five nephrolithiasis families. Recurrence occurred in 57.9% of patients; this was significantly associated with the erythrocyte anomaly, hyperoxaluria, and male gender. However, when using multivariate analysis, only gender and the erythrocyte anomaly were statistically significant and were independent predictors of recurrency. The probability of stone recurrency predicted by the logistic model ranged from 30.1% for women with normal erythrocyte oxalate transport, to 73.4% for men with the erythrocyte anomaly. The family follow-up showed that only subjects with the erythrocyte abnormality become renal stone-formers in the 8-yr survey. By showing the predictive value of the erythrocyte oxalate anomaly for recurrent calcium nephrolithiasis, our findings support the notion that this anomaly is a risk factor in renal stone disease.
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PMID:The abnormal red-cell oxalate transport is a risk factor for idiopathic calcium nephrolithiasis: a prospective study. 872 95

Urolithiasis and/or nephrocalcinosis due to hereditary diseases are a rare event which must be kept in mind of physicians who take care of children (10 to 40% of all causes of lithiases) as well as of adults (less than 15% of all causes of lithiases) since a specific management is usually required. The most frequent inborn disorders are idiopathic hypercalciuria, distal tubular acidosis, cystinuria and hyperoxaluria. Stone formation is always secondary to an increased urine concentration of promotors, i.e. calcium, oxalate, phosphate, cystine, xanthine. One of the most informative diagnosis investigation is infrared spectrophotometry which can identify stone composition. When such a technique is not available, biochemical investigations should be adapted to both personal and family history. In addition to high fluid intake (2 to 3 L/m2/24 h) sometimes associated with alcalinisation, the management of hereditary stone disease requires specific procedure. In all cases, the long-term renal prognosis is related to both primary disease and therapeutic compliance.
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PMID:[Hereditary diseases causing kidney calculi]. 936 14

Calcium urolithiasis is often associated with increased intestinal absorption and urine excretion of calcium, and has been suggested to result from increased vitamin D production. The role of the enzyme 1 alpha-hydroxylase, the rate-limiting step in active vitamin D production, was evaluated in 36 families, including 28 sibships with at least a pair of affected sibs, using qualitative and quantitative trait linkage analyses. Sibs with a verified calcium urolithiasis passage (n = 117) had higher 24-h calciuria (P = 0.03), oxaluria (P = 0.02), fasting and postcalcium loading urine calcium/creatinine (Ca/cr) ratios (P = 0.008 and P = 0.002, respectively), and serum 1,25(OH)2 vitamin D levels (P = 0.02) compared with nonstone-forming sibs (n = 120). Markers from a 9-centiMorgan interval encompassing the VDD1 locus on chromosome 12q13-14 (putative 1 alpha-hydroxylase) were analyzed in 28 sibships (146 sib pairs) of single and recurrent stone formers and in 14 sibships (65 sib pairs) with recurrent-only (> or = 3 episodes) stone-forming sibs. Two-point and multipoint analyses did not reveal excess in alleles shared among affected sibs at the VDD1 locus. Linkage of stone formation to the VDD1 locus could be excluded, respectively, with a lambda d of 2.0 (single and recurrent stone formers) and 3.25 (recurrent stone formers). Quantitative trait analyses revealed no evidence for linkage to 24-h calciuria and oxaluria, serum 1,25(OH)2 vitamin D levels, and Ca/cr ratios. This study shows absence of linkage of the putative 1 alpha-hydroxylase locus to calcium stone formation or to quantitative traits associated with idiopathic hypercalciuria. In addition, there is coaggregation of calciuric and oxaluric phenotypes with stone formation.
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PMID:The 1 alpha-hydroxylase locus is not linked to calcium stone formation or calciuric phenotypes in French-Canadian families. 951 4

Supersaturation with respect to calcium salts (oxalate and phosphate) is the driving force leading to crystalluria and nephrolithiasis. High-molecular-weight urinary inhibitors are recently described molecules capable of altering the process of kidney stone formation. By inhibition of crystal nucleation, growth and aggregation and by inhibition of crystal interaction with tubular cells, these proteins efficiently prevent stone formation and retention in the urinary tract. But in spite of considerable efforts, characterization of these proteins is still under way. Besides the pathophysiology of risk factors for calcium salts supersaturation such as idiopathic hypercalciuria or hyperoxaluria, the renal involvement of protein inhibitors is the most exciting field in the comprehensive approach of nephrolithiasis, a disease that affects up to 10% of people in Western countries.
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PMID:Urinary kidney stone inhibitors. What is the news? 956 42

Idiopathic calcium nephrolithiasis (ICN) is a frequent disease in Western countries. The physicochemical theory of lithogenesis, which explains stone formation by the precipitation, growth, and crystalline aggregation of lithogenic salts in the urine, has contributed greatly to the understanding of the pathogenesis of calcium urolithiasis. However, several aspects are still unexplained; the co-existence of familial occurrence, primary tubular dysfunctions with ICN, and anomalies in the systemic handling of oxalate and calcium led to the development of a cellular hypothesis of ICN. A number of cellular defects in the handling of ions has been reported that involves both anion and cation transport. These anomalies are probably the expression of a still unknown cellular defect in idiopathic calcium stone formers. We suggested that an anomaly in the cell membrane composition might be responsible for the complex array of cell ion flux abnormalities observed in ICN. Recently, a disorder in the n-6 polyunsaturated fatty acid series has been described; it is characterized by a lower linoleic acid content and a higher arachidonic acid concentration in both plasma and erythrocyte membrane phospholipids of renal calcium stone patients. This anomaly could cause an increased activity of ion carriers; furthermore, it may lead to increased prostaglandin synthesis and to secondary phenomena at the kidney, skeletal, and intestinal level. As a consequence, critical conditions for lithogenesis in the kidney may ensue. The data suggest a common pathogenesis for hypercalciuria and hyperoxaluria. The systemic defect in the phospholipid arachidonic acid level may be both of dietary or genetic origin; experimental data suggest that the increase in delta-6 desaturase activity, the limiting enzyme in the metabolic pathway of polyunsaturated fatty acids, might be relevant to the pathogenesis of lipid abnormalities observed in nephrolithiasis and to the pathogenesis of ICN and its related problems (at the kidney, intestinal, and bone level).
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PMID:Pathogenesis of idiopathic calcium nephrolithiasis: update 1997. 959 25


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