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Kidney International Jan 2021Cystinuria (OMIM 220100) is an autosomal recessive hereditary disorder in which high urinary cystine excretion leads to the formation of cystine stones because of the...
Cystinuria (OMIM 220100) is an autosomal recessive hereditary disorder in which high urinary cystine excretion leads to the formation of cystine stones because of the low solubility of cystine at normal urinary pH. We developed clinical practice recommendation for diagnosis, surgical and medical treatment, and follow-up of patients with cystinuria. Elaboration of these clinical practice recommendations spanned from June 2018 to December 2019 with a consensus conference in January 2019. Selected topic areas were chosen by the co-chairs of the conference. Working groups focusing on specific topics were formed. Group members performed systematic literature review using MEDLINE, drafted the statements, and discussed them. They included geneticists, medical biochemists, pediatric and adult nephrologists, pediatric and adult urologists experts in cystinuria, and the Metabolic Nephropathy Joint Working Group of the European Reference Network for Rare Kidney Diseases (ERKNet) and eUROGEN members. Overall 20 statements were produced to provide guidance on diagnosis, genetic analysis, imaging techniques, surgical treatment (indication and modalities), conservative treatment (hydration, dietetic, alkalinization, and cystine-binding drugs), follow-up, self-monitoring, complications (renal failure and hypertension), and impact on quality of life. Because of the rarity of the disease and the poor level of evidence in the literature, these statements could not be graded. This clinical practice recommendation provides guidance on all aspects of the management of both adults and children with cystinuria, including diagnosis, surgery, and medical treatment.
Topics: Adult; Child; Consensus; Cystine; Cystinuria; Humans; Kidney; Quality of Life
PubMed: 32918941
DOI: 10.1016/j.kint.2020.06.035 -
Urolithiasis Oct 2017This meta-analysis summarizes the available evidence on the effectiveness of citrate supplement for preventing the recurrence of nephrolithiasis in patients undergoing... (Meta-Analysis)
Meta-Analysis Review
This meta-analysis summarizes the available evidence on the effectiveness of citrate supplement for preventing the recurrence of nephrolithiasis in patients undergoing extracorporeal shock wave lithotripsy (SWL). Electronic searches were conducted using Medline-PubMed, Web of Science, Embase, BVS (Scielo, Lilacs), and Google Scholar literature databases. The authors worked in pairs to select studies that met the following criteria: randomized controlled trials that were conducted in adults and assessed the effect of potassium citrate supplement before or after SWL therapy for urolithiasis. Our primary aim was to asses the stone-free rate among the groups included in the studies. Fixed effect was used in the meta-analysis with 95% confidence interval (95% CI). Heterogeneity was analyzed by the I value. A total of 2505 references were initially selected. Of those, four were subjected to meta-analysis contributing five samples. These four studies included 374 participants who were followed for a period of 12 months after SWL. Mean potassium citrate dosage was approximately 55 mEq/day (18 mmol). The results showed that citrate supplement significantly protected against the recurrence of nephrolithiasis during 1 year after SWL [RR; 95% CI 0.21 (0.13, 0.31)]. The heterogeneity was not significant across the analyzed studies (p = 0.224). The quality of the analyzed studies was generally low. The available evidence shows that citrate supplement effectively reduces the recurrence of nephrolithiasis in patients undergoing SWL. However, statistical analysis of a larger trial conducted with methodological rigor is warranted.
Topics: Dietary Supplements; Humans; Lithotripsy; Nephrolithiasis; Potassium Citrate; Randomized Controlled Trials as Topic; Secondary Prevention; Treatment Outcome
PubMed: 27915395
DOI: 10.1007/s00240-016-0950-1 -
Clinical and Applied... 2023In patients with liver failure complicated by acute kidney injury, renal replacement therapy (RRT) is often required to improve the internal environment. The use of... (Meta-Analysis)
Meta-Analysis Review
In patients with liver failure complicated by acute kidney injury, renal replacement therapy (RRT) is often required to improve the internal environment. The use of anticoagulants for RRT in patients with liver failure remains controversial. We searched the PubMed, Embase, Cochrane Library, and Web of Science databases for studies. The methodological quality of the included studies was assessed using the Methodological Index for Nonrandomized Studies. A meta-analysis was performed using R software (version 3.5.1) and Review Manager (version 5.3.5). During RRT, 348 patients from 9 studies received regional citrate anticoagulation (RCA), and 127 patients from 5 studies received heparin anticoagulation (including heparin and LMWH). Among patients who received RCA, the incidence of citrate accumulation, metabolic acidosis, and metabolic alkalosis were 5.3% (95% confidence interval [CI]: 0%-25.3%), 26.4% (95% CI: 0-76.9), and 1.8% (95% CI: 0-6.8), respectively. The potassium, phosphorus, total bilirubin (TBIL), and creatinine levels were lower, whereas the serum pH, bicarbonate, base excess levels, and total calcium/ionized calcium ratio were higher after treatment than before treatment. Among patients who received heparin anticoagulation, the TBIL levels were lower, whereas the activated partial thromboplastin clotting time and D-dimer levels were higher after treatment than before treatment. The mortality rates in the RCA and heparin anticoagulation groups were 58.9% (95% CI: 39.2-77.3) and 47.4% (95% CI: 31.1-63.7), respectively. No statistical difference in mortality was observed between the 2 groups. For patients with liver failure, the administration of RCA or heparin for anticoagulation during RRT under strict monitoring may be safe and effective.
Topics: Humans; Heparin; Citric Acid; Heparin, Low-Molecular-Weight; Calcium; Anticoagulants; Citrates; Renal Replacement Therapy; Liver Failure
PubMed: 37186766
DOI: 10.1177/10760296231174001 -
Nephrology, Dialysis, Transplantation :... Aug 2016Hypocitraturia is a known risk factor for nephrolithiasis, present in 20-60% of stone-forming patients. The administration of citrate or other alkali preparations has... (Review)
Review
BACKGROUND
Hypocitraturia is a known risk factor for nephrolithiasis, present in 20-60% of stone-forming patients. The administration of citrate or other alkali preparations has been demonstrated to benefit hypocitraturic stone formers. Dietary modifications that include citrate-containing fluids can be an alternative option to pharmacological agents. We aimed to systematically review, summarize and quantify available evidence on the effects of non-pharmacological interventions on urinary citrate and nephrolithiasis.
METHODS
Manual and electronic database searches (MEDLINE/PubMed, Embase, Cochrane Library, Scopus, Scielo, LILACS) were performed for studies published up to July 2014. Two reviewers independently identified studies for inclusion and extracted data on study characteristics, outcomes and quality assessments. We included controlled studies with non-pharmacological interventions that assessed urinary citrate levels or nephrolithiasis pre- and post-intervention. Meta-analysis was performed by random effects and subgrouped by the type of intervention, and heterogeneity was analysed by I(2).
RESULTS
Of the 427 studies identified, 13 studies were included (18 samples), involving 358 participants with a mean age of 43 ± 11.0 years across the studies. Interventions were grouped as commercial fruit juices, soft drinks, calcium-/magnesium-rich mineral water, high-fiber diet, low-animal-protein diet and plant extract. Almost half of the studies (6/13; 8/18 samples) reported effects in non-stone formers. Two studies included stone formers and non-stone formers. Commercial fruit juice interventions showed high I(2) (88.1%, P = 0.000) and an increase in citraturia levels ( 95% confidence interval) of 167.2 (65.4; 269) mg/day. Other types of intervention did not show important heterogeneity; however, pooled estimates were not significant.
CONCLUSION
Our review indicates that further larger scale trials are required to analyze whether non-pharmacological interventions can increase urinary citrate levels and act in kidney stone prevention.
Topics: Citrates; Diet, Protein-Restricted; Humans; Kidney Calculi; Potassium Citrate
PubMed: 26311217
DOI: 10.1093/ndt/gfv303 -
The Cochrane Database of Systematic... Oct 2015Kidney stones affect people worldwide and have a high rate of recurrence even with treatment. Recurrences are particularly prevalent in people with low urinary citrate... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Kidney stones affect people worldwide and have a high rate of recurrence even with treatment. Recurrences are particularly prevalent in people with low urinary citrate levels. These people have a higher incidence of calcium phosphate and calcium oxalate stones. Oral citrate therapy increases the urinary citrate levels, which in turn binds with calcium and inhibits the crystallisation thus reduces stone formation. Despite the widespread use of oral citrate therapy for prevention and treatment of calcium oxalate stones, the evidence to support its clinical efficacy remains uncertain.
OBJECTIVES
The objective of this review was to determine the efficacy and adverse events associated with citrate salts for the treatment and prevention of calcium containing kidney stones.
SEARCH METHODS
We searched the Cochrane Kidney and Transplant Specialised Register to 29 July 2015 through contact with the Trials' Search Co-ordinator using search terms relevant to this review.
SELECTION CRITERIA
We included randomised controlled trials (RCTs) that assessed the efficacy and adverse events associated with citrate salts for the treatment and prevention of calcium containing kidney stones in adults treated for a minimum of six months.
DATA COLLECTION AND ANALYSIS
Two authors assessed studies for inclusion in this review. Data were extracted according to predetermined criteria. Summary estimates of effect were obtained using a random-effects model, and results were expressed as risk ratios (RR) and their 95% confidence intervals (CI) for dichotomous outcomes, and mean difference (MD) and 95% CI for continuous outcomes.
MAIN RESULTS
We included seven studies that included a total of 477 participants, most of whom had oxalate stones. Of these, three studies (247 participants) compared potassium citrate with placebo or no intervention; three (166 participants) compared potassium-sodium citrate with no intervention; and one (64 participants) compared potassium-magnesium citrate with placebo. Overall, quality of the reporting of the included studies was considered moderate to poor, and there was a high risk of attrition bias in two studies.Compared with placebo or no intervention, citrate therapy significantly reduced the stone size (4 studies, 160 participants: RR 2.35, 95% CI 1.36 to 4.05). New stone formation was significantly lower with citrate therapy compared to control (7 studies, 324 participants: RR 0.26, 95% CI 0.10 to 0.68). The beneficial effect on stone size stability was also evident (4 studies, 160 participants: RR 1.97, 95% CI 1.19 to 3.26). Adverse events were reported in four studies, with the main side effects being upper gastrointestinal disturbance and one patient reported a rash. There were more gastrointestinal adverse events in the citrate group; however this was not significant (4 studies, 271 participants: RR 2.55, 95% CI 0.71 to 9.16). There were significantly more dropouts due to adverse events with citrate therapy compared to control (4 studies, 271 participants: RR 4.45, 95% CI 1.28 to 15.50). The need for retreatment was significantly less with citrate therapy compared to control (2 studies, 157 participants: RR 0.22, 95% CI 0.06 to 0.89).
AUTHORS' CONCLUSIONS
Citrate salts prevent new stone formation and reduce further stone growth in patients with residual stones that predominantly contain oxalate. The quality of reported literature remains moderate to poor; hence a well-designed statistically powered multi-centre RCT is needed in order to answer relevant questions concerning the efficacy of citrate salts.
Topics: Adult; Calcium Oxalate; Calcium Phosphates; Citrates; Drug Combinations; Humans; Kidney Calculi; Magnesium Compounds; Potassium Compounds; Randomized Controlled Trials as Topic; Recurrence; Secondary Prevention
PubMed: 26439475
DOI: 10.1002/14651858.CD010057.pub2 -
The Cochrane Database of Systematic... Nov 2017Nephrolithiasis, or urinary stone disease, in children causes significant morbidity, and is increasing in prevalence in the North American population. Therefore, medical... (Review)
Review
BACKGROUND
Nephrolithiasis, or urinary stone disease, in children causes significant morbidity, and is increasing in prevalence in the North American population. Therefore, medical and dietary interventions (MDI) for recurrent urinary stones in children are poised to gain increasing importance in the clinical armamentarium.
OBJECTIVES
To assess the effects of medical and dietary interventions (MDI) for the prevention of idiopathic urinary stones in children aged from one to 18 years.
SEARCH METHODS
We searched multiple databases using search terms relevant to this review, including studies identified from the Cochrane Central Register of Controlled Trials (CENTRAL, 2017, Issue 1), MEDLINE OvidSP (1946 to 14 February 2017), Embase OvidSP (1980 to 14 February 2017), International Clinical Trials Register (ICTRP) Search Portal and ClinicalTrials.gov. Additionally, we handsearched renal-related journals and the proceedings of major renal conferences, and reviewed weekly current awareness alerts for selected renal journals. The date of the last search was 14 February 2017. There were no language restrictions.
SELECTION CRITERIA
Randomized controlled trials of at least one year of MDI versus control for prevention of recurrent idiopathic (non-syndromic) nephrolithiasis in children.
DATA COLLECTION AND ANALYSIS
We used standard methodologic procedures expected by Cochrane. Titles and abstracts were identified by search criteria and then screened for relevance, and then data extraction and risk of bias assessment were carried out. We assessed the quality of evidence using GRADE.
MAIN RESULTS
The search identified one study of 125 children (72 boys and 53 girls) with calcium-containing idiopathic nephrolithiasis and normal renal morphology following initial treatment with shockwave lithotripsy (SWL). Patients were randomized to oral potassium citrate 1 mEq/kg per day for 12 months versus no specific medication or preventive measure with results reported for a total of 96 patients (48 per group). This included children who were stone-free (n = 52) or had residual stone fragments (n = 44) following SWL. Primary outcomes:Medical therapy may lower rates of stone recurrence with a risk ratio (RR) of 0.19 (95% confidence interval (CI) 0.06 to 0.60; low quality evidence). This corresponds to 270 fewer stone recurrences per 1000 (133 fewer to 313 fewer) children. We downgraded the quality of evidence by two levels for very serious study limitations related to unclear allocation concealment (selection bias) and a high risk of performance, detection and attrition bias. While the data for adverse events were incomplete, they reported that six of 48 (12.5%) children receiving potassium citrate left the trial because of adverse effects. This corresponds to a RR of 13.0 (95% CI 0.75 to 224.53; very low quality evidence); an absolute effect size estimate could not be generated. We downgraded the quality of evidence for study limitations and imprecision.We found no information on retreatment rates.
SECONDARY OUTCOMES
We found no evidence on serum electrolytes, 24-hour urine collection parameters or time to new stone formation.We were unable to perform any preplanned secondary analyses.
AUTHORS' CONCLUSIONS
Oral potassium citrate supplementation may reduce recurrent calcium urinary stone formation in children following SWL; however, our confidence in this finding is limited. A substantial number of children stopped the medication due to adverse events. There is no trial evidence on retreatment rates. There is a critical need for additional well-designed trials in children with nephrolithiasis.
Topics: Administration, Oral; Calcium; Child; Female; Humans; Kidney Calculi; Lithotripsy; Male; Potassium Citrate; Recurrence; Secondary Prevention; Urinary Calculi
PubMed: 29117629
DOI: 10.1002/14651858.CD011252.pub2 -
Current Urology Reports Aug 2023We wanted to analyse the outcomes of surgical (SWL, URS, PCNL) and medical management of cystine stones in the paediatric population in terms of stone-free status and... (Review)
Review
PURPOSE OF REVIEW
We wanted to analyse the outcomes of surgical (SWL, URS, PCNL) and medical management of cystine stones in the paediatric population in terms of stone-free status and complication rates, based on all the available literature evidence.
RECENT FINDINGS
A systematic review of literature was performed for all studies with paediatric cystine stone management. Twelve studies met the eligibility criteria, of which 4 analysed outcomes of SWL, 2 of URS and 3 of PCNL and 3 focused on the effect of either alkalising agents (potassium citrate, citric acid) or cysteine-binding thiol (CBT) agents (tiopronin, penicillamine). The reported SFR in studies ranged from 50 to 83%, 59 to 100% and 63 to 80.6%, with a complication rate of 2.8-51%, 14-27% and 12.9-15.4% with SWL, URS and PCNL, respectively. Paediatric cystine stones treatment should aim at complete stone clearance, preservation of renal function and prevention of further recurrences. SWL achieves inferior results in case of cystine stones. URS and PCNL are safe and effective procedures in the paediatric population, with a low rate of major complications. Adherence to medical prevention therapies may prolong recurrence-free periods.
Topics: Humans; Child; Kidney Calculi; Cystine; Lithotripsy; Ureteroscopy; Ureteral Calculi; Treatment Outcome
PubMed: 37079195
DOI: 10.1007/s11934-023-01162-9 -
World Journal of Urology May 2023Hypocitraturia is a low urinary excretion of citrate and a well-known risk factor for kidney stone development in children. This systematic review aimed to evaluate the...
PURPOSE
Hypocitraturia is a low urinary excretion of citrate and a well-known risk factor for kidney stone development in children. This systematic review aimed to evaluate the dietary management of hypocitraturia in children with urolithiasis.
METHODS
Literature search was performed on 30th September 2022 using Embase, PubMed, and Cochrane Central Controlled Register of Trials. Studies were included if children with stones and hypocitraturia were managed with diet supplements.
RESULTS
Six papers were included. Four studies evaluated the role of oral potassium citrate associated with high fluid intake on stone resolution and recurrence. Two studies assessed the impact of oral potassium citrate on long-term stone recurrence after percutaneous nephrolithotomy and shock wave lithotripsy. All studies demonstrated that the association of potassium citrate and high fluid intake was well tolerated with no side effects and restored normal urine citrate excretion, allowed a reduction in stone size, and, following definitive treatments, was associated with a lower rate of stone regrowth and recurrence compared with controls. These effects were demonstrated across all pediatric ages.
CONCLUSIONS
Our review infers that oral potassium citrate and high fluid assumption are safe and effective in restoring urine citrate excretion, treating and preventing stone recurrence with no serious adverse events, and should probably be the first-line treatment of pediatric patients with asymptomatic stones and hypocitraturia.
Topics: Child; Humans; Potassium Citrate; Urolithiasis; Kidney Calculi; Citric Acid; Citrates
PubMed: 36645461
DOI: 10.1007/s00345-023-04282-6 -
F1000Research 2017Urolithiasis is a disease with high recurrence rate, 30-50% within 5 years. The aim of the present study was to learn the effects of citrus-based products on the urine...
Urolithiasis is a disease with high recurrence rate, 30-50% within 5 years. The aim of the present study was to learn the effects of citrus-based products on the urine profile in healthy persons and people with urolithiasis compared to control diet and potassium citrate. A systematic review was performed, which included interventional, prospective observational and retrospective studies, comparing citrus-based therapy with standard diet therapy, mineral water, or potassium citrate. A literature search was conducted using PUBMED, COCHRANE, and Google Scholar with "citrus or lemonade or orange or grapefruit or lime or juice" and "urolithiasis" as search terms. For statistical analysis, a fixed-effects model was conducted when p > 0.05, and random-effects model was conducted when p < 0.05. In total, 135 citations were found through database searching with 10 studies found to be consistent with our selection criteria. However, only 8 studies were included in quantitative analysis, due to data availability. The present study showed a higher increased in urine pH for citrus-based products (mean difference, 0.16; 95% CI 0.01-0.32) and urinary citrate (mean difference, 124.49; 95% CI 80.24-168.74) compared with a control group. However, no differences were found in urine volume, urinary calcium, urinary oxalate, and urinary uric acid. From subgroup analysis, we found that citrus-based products consistently increased urinary citrate level higher than controls in both healthy and urolithiasis populations. Furthermore, there was lower urinary calcium level among people with urolithiasis. Citrus-based products could increase urinary citrate level significantly higher than control. These results should encourage further research to explore citrus-based products as a urolithiasis treatment.
PubMed: 28529700
DOI: 10.12688/f1000research.10976.1 -
Advances in Clinical Chemistry 2018The measurement of fasting plasma glucose may be biased by a time-dependent decrease of glucose in blood tubes, mainly attributable to blood cell metabolism when...
The measurement of fasting plasma glucose may be biased by a time-dependent decrease of glucose in blood tubes, mainly attributable to blood cell metabolism when glycolysis is not rapidly inhibited or blood cells cannot be rapidly separated from plasma. Although glycolysis inhibitors such as sodium fluoride (NaF) in combination with potassium oxalate (KOx) are currently used for overcoming this drawback, their efficacy for stabilizing blood glucose is seemingly limited, and probably lower than that of newer additives such as the citrate buffer. Therefore, we performed a critical analysis of the current scientific literature aimed to generate evidence-based information about the advantages of using citrate buffer in blood tubes compared to the more conventional NaF additive. The results of our systematic overview of the literature show that citrate blood tubes represent a considerable step forward in achieving more accurate and reliable plasma glucose measurements, thereby limiting the risk of underdiagnosing diabetes due to spurious decrease of glucose concentration in uncentrifuged blood specimens, ensuring higher stability of glucose levels over time, while simultaneously producing less hemolysis compared to NaF blood tubes. Therefore, we suggest that the use of this new mixture should be encouraged for achieving a higher degree of accuracy and standardization of plasma glucose measurements.
Topics: Animals; Blood Glucose; Blood Specimen Collection; Buffers; Citrates; Diabetes Mellitus; Glycolysis; Humans; Sodium Fluoride
PubMed: 29478513
DOI: 10.1016/bs.acc.2017.12.003