Page last updated: 2024-10-20

pyridoxine and Oxaluria, Primary

pyridoxine has been researched along with Oxaluria, Primary in 33 studies

4,5-bis(hydroxymethyl)-2-methylpyridin-3-ol: structure in first source
vitamin B6 : Any member of the group of pyridines that exhibit biological activity against vitamin B6 deficiency. Vitamin B6 deficiency is associated with microcytic anemia, electroencephalographic abnormalities, dermatitis with cheilosis (scaling on the lips and cracks at the corners of the mouth) and glossitis (swollen tongue), depression and confusion, and weakened immune function. Vitamin B6 consists of the vitamers pyridoxine, pyridoxal, and pyridoxamine and their respective 5'-phosphate esters (and includes their corresponding ionized and salt forms).

Research Excerpts

ExcerptRelevanceReference
"Plasma pyridoxine metabolites in plasma and 4-pyridoxic acid excretions in urine were measured in normal subjects, in 7 patients with type-1 hyperoxaluria and in 8 patients with mild metabolic hyperoxaluria, while receiving various doses of pyridoxine."7.68Metabolism of pyridoxine in mild metabolic hyperoxaluria and primary hyperoxaluria (type 1). ( Edwards, P; Rose, GA, 1991)
"Plasma pyridoxine metabolites in plasma and 4-pyridoxic acid excretions in urine were measured in normal subjects, in 7 patients with type-1 hyperoxaluria and in 8 patients with mild metabolic hyperoxaluria, while receiving various doses of pyridoxine."3.68Metabolism of pyridoxine in mild metabolic hyperoxaluria and primary hyperoxaluria (type 1). ( Edwards, P; Rose, GA, 1991)
"Pyridoxine (vitamin B6), given to patients with primary hyperoxaluria of type I, generally leads to a decrease in urinary excretion of oxalate owing to stimulation of conversion of glyoxylate to glycine instead of oxalate."3.67[Pyridoxine can normalize oxaluria in idiopathic renal lithiasis]. ( Burckhardt, P; Jacquet, AF; Jaeger, P; Portmann, L, 1986)
"Accurate diagnosis of primary hyperoxaluria (PH) has important therapeutic consequences."3.01Genetic assessment in primary hyperoxaluria: why it matters. ( Acquaviva, C; Bacchetta, J; Beck, B; Deesker, L; Garrelfs, S; Groothoff, J; Gupta, A; Mandrile, G; Rumsby, G, 2023)
"Type 1 primary hyperoxaluria is a genetic disorder caused by deficiency of the liver-specific peroxisomal enzyme alanine-glyoxylate aminotransferase."2.49Nocturnal home hemodialysis for a patient with type 1 hyperoxaluria. ( Fillaus, JA; Plumb, TJ; Swee, ML, 2013)
"Primary hyperoxaluria type I is a metabolic disorder caused by the deficiency of the peroxisomal alanine:glyoxylate aminotransferase."2.38Primary hyperoxaluria type I. ( Brodehl, J; Latta, K, 1990)
"Primary hyperoxaluria type 1 (PH1) is an inborn error of glyoxylate metabolism, characterized by increased endogenous oxalate production."1.62Endogenous Oxalate Production in Primary Hyperoxaluria Type 1 Patients. ( Garrelfs, SF; Groothoff, JW; Oosterveld, MJS; Peters-Sengers, H; Schierbeek, H; van den Akker, CHP; van Goudoever, JB; van Harskamp, D; Wanders, RJA; Wijburg, FA, 2021)
"Primary hyperoxaluria is a rare genetic disorder characterized by oxalate overproduction, leading to kidney failure due to nephrocalcinosis, and is eventually responsible for systemic oxalosis."1.48Type 1 primary hyperoxaluria: A case report and focus on bone impairment of systemic oxalosis. ( Boivin, G; Caillard, S; Javier, RM; Pijnenburg, L; Rizzo, S, 2018)
"Primary hyperoxalurias are rare inborn errors of metabolism resulting in increased endogenous production of oxalate that leads to excessive urinary oxalate excretion."1.46Clinical spectrum of primary hyperoxaluria type 1: Experience of a tertiary center. ( Abdelaziz, H; Abdelrahman, SM; Badr, AM; Bazaraa, HM; Eweeda, KM; Fayez, A; Ghanim, K; Helmy, R; Kotb, MA; Nabhan, MM; Soliman, NA; Tolba, OA, 2017)
"The hereditary kidney stone disease primary hyperoxaluria type 1 (PH1) is caused by a functional deficiency of the liver-specific, peroxisomal, pyridoxal-phosphate-dependent enzyme, alanine:glyoxylate aminotransferase (AGT)."1.43Effects of alanine:glyoxylate aminotransferase variants and pyridoxine sensitivity on oxalate metabolism in a cell-based cytotoxicity assay. ( Danpure, CJ; Fargue, S; Holmes, RP; Knight, J; Rumsby, G, 2016)
"Primary hyperoxalurias are rare recessive inherited inborn errors of glyoxylate metabolism."1.37[Primary hyperoxaluria]. ( Bacchetta, J; Bertholet-Thomas, A; Cochat, P; Fargue, S; Harambat, J; Sabot, JF, 2011)
"Pyridoxine (VB6) response in type I primary hyperoxaluria (PHI) is variable, with nearly equal numbers of patients showing partial to complete reductions in oxaluria, and resistance."1.33Pyridoxine effect in type I primary hyperoxaluria is associated with the most common mutant allele. ( Milliner, DS; Monico, CG; Olson, JB; Rossetti, S, 2005)
"The detection of type I primary hyperoxaluria is based on the finding of exceedingly high oxalate excretion which is associated with increased glycolate excretion."1.28Glycolate determination detects type I primary hyperoxaluria in dialysis patients. ( Bianco, O; Finocchiaro, P; Linari, F; Marangella, M; Petrarulo, M; Vitale, C, 1991)
"Primary hyperoxaluria type I (PH I) is characterized by an excessive endogenous production and excretion of oxalic and glycolic acid."1.28Vitamin B6 resistant primary hyperoxaluria type I. Report of 5 cases. ( Donckerwolcke, RA; Streefland, M, 1989)

Research

Studies (33)

TimeframeStudies, this research(%)All Research%
pre-19905 (15.15)18.7374
1990's6 (18.18)18.2507
2000's5 (15.15)29.6817
2010's13 (39.39)24.3611
2020's4 (12.12)2.80

Authors

AuthorsStudies
Garrelfs, SF1
van Harskamp, D1
Peters-Sengers, H1
van den Akker, CHP1
Wanders, RJA1
Wijburg, FA1
van Goudoever, JB1
Groothoff, JW1
Schierbeek, H1
Oosterveld, MJS1
Mandrile, G1
Beck, B1
Acquaviva, C1
Rumsby, G3
Deesker, L1
Garrelfs, S1
Gupta, A1
Bacchetta, J2
Groothoff, J1
Medina, PG1
Román, LE1
Lorenz, EC2
Lieske, JC2
Seide, BM2
Olson, JB3
Mehta, R1
Milliner, DS5
Pijnenburg, L1
Caillard, S1
Boivin, G1
Rizzo, S1
Javier, RM1
Dindo, M1
Conter, C1
Oppici, E2
Ceccarelli, V1
Marinucci, L1
Cellini, B2
Sas, DJ1
Harris, PC1
Fargue, S5
Danpure, CJ4
Plumb, TJ1
Swee, ML1
Fillaus, JA1
Meek, AM1
Bergstralh, EJ1
Miyata, N1
Steffen, J1
Johnson, ME1
Koehler, CM1
Reid, ES1
Mills, PB1
Clayton, PT1
Knight, J1
Holmes, RP1
Assimos, DG1
Soliman, NA1
Nabhan, MM1
Abdelrahman, SM1
Abdelaziz, H1
Helmy, R1
Ghanim, K1
Bazaraa, HM1
Badr, AM1
Tolba, OA1
Kotb, MA1
Eweeda, KM1
Fayez, A1
Hoppe, B1
Beck, BB1
Punjabi, OS1
Riaz, K1
Mets, MB1
Cochat, P1
Bertholet-Thomas, A1
Sabot, JF1
Harambat, J1
Asplin, JR1
Monico, CG1
Rossetti, S1
Marangella, M4
Castelló Girona, F1
Riudor Taravilla, E1
Enríquez Civicos, G1
Güell Torné, C1
Fornaguera Soler, F1
Kist-van Holthe, JE1
Onkenhout, W1
van der Heijden, AJ1
Petrarulo, M3
Vitale, C3
Bagnis, C1
Berutti, S1
Ramello, A1
Amoroso, A1
Cosseddu, D1
Linari, F2
Edwards, P1
Rose, GA1
Bianco, O1
Finocchiaro, P1
Latta, K1
Brodehl, J1
Streefland, M1
Donckerwolcke, RA1
Yano, S1
Yoshino, M1
Nishiyori, A1
Nakao, M1
Matsumoto, T1
Ito, Y1
Yamashita, F1
Shimada, A1
Inokuchi, T1
Watts, RW1
Morgan, SH1
Purkiss, P1
Mansell, MA1
Baker, LR1
Brown, CB1
Amato, M1
Donzelli, S1
Lombardi, M1
Salvadori, M1
Carini, M1
Selli, C1
Caudarella, R1
Jaeger, P1
Portmann, L1
Jacquet, AF1
Burckhardt, P1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Outcomes of Metabolic Resuscitation Using Ascorbic Acid, Thiamine, and Glucocorticoids in the Early Treatment of Sepsis.[NCT03422159]Phase 2140 participants (Actual)Interventional2018-02-05Completed
Efficacy of Betaine for Reduction of Urine Oxalate in Patients With Type 1 Primary Hyperoxaluria[NCT00283387]Phase 215 participants (Actual)Interventional2007-02-28Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Change in Sequential Organ Failure Assessment (SOFA) Score

Defined as the day 4 post-randomization SOFA score minus the initial SOFA score. The Sequential Organ Failure Assessment (SOFA) Score is a mortality prediction score that is based on the degree of dysfunction of six organ systems. The score is calculated on admission and every 24 hours until discharge using the worst parameters measured during the prior 24 hours SOFA score ranges from 0 (no organ dysfunction) to 24 (highest possible score / organ dysfunction). (NCT03422159)
Timeframe: 4 days post-randomization

Interventionscore on a scale (Mean)
Treatment Arm2.9
Placebo Arm1.93

Hospital Length of Stay

Time from admitting to discharge of hospital stay. (NCT03422159)
Timeframe: From admission to the hospital until final discharge, up to 28 days.

InterventionDays (Mean)
Treatment Arm11.5
Placebo Arm11

Hospital Mortality

In-hospital mortality rate. (NCT03422159)
Timeframe: Survival until hospital discharge, up to 28 days.

InterventionParticipants (Count of Participants)
Treatment Arm11
Placebo Arm13

ICU Length of Stay

Time from admitting to ICU to discharge. (NCT03422159)
Timeframe: From admission to the ICU until final discharge from the ICU, up to an average of 7 days.

Interventiondays (Mean)
Treatment Arm4.76
Placebo Arm4.66

ICU Mortality

ICU mortality rate (NCT03422159)
Timeframe: From admission to hospital until final discharge from the ICU, up to 28 days.

InterventionParticipants (Count of Participants)
Treatment Arm6
Placebo Arm10

Procalcitonin (PCT) Clearance

PCT at 96 hours minus initial PCT, divided by the initial PCT multiplied by 100. (NCT03422159)
Timeframe: 4 days post-randomization

InterventionPercent (Mean)
Treatment Arm63
Placebo Arm58

Time to Vasopressor Independence (Hours)

Defined as the time from starting the active treatment/placebo to discontinuation of all pressors. (NCT03422159)
Timeframe: From start of vasopressor medication to final discontinuation of vasopressor medication, up to 7 days.

Interventionhours (Mean)
HAT Treatment27
Comparator53

Ventilator Free Days

Number of days alive and off of the ventilator at day 28. (NCT03422159)
Timeframe: 28 Days post-randomization

InterventionDays (Mean)
Treatment Arm22
Placebo Arm22.4

Urinary Oxalate Excretion

"The patients were randomly assigned oral betaine or placebo for 2 months, followed by a 2 month washout. Each patient then received the alternate study medication for 2 months.~Urinary Oxalate Excretion was measured by oxalate oxidase. Two 24 hour urine collections were obtained at baseline, and during the eighth week of each study period." (NCT00283387)
Timeframe: baseline, 2 months, 6 months

Interventionumol/mg (Mean)
Betaine1.43
Placebo1.04

Reviews

9 reviews available for pyridoxine and Oxaluria, Primary

ArticleYear
Genetic assessment in primary hyperoxaluria: why it matters.
    Pediatric nephrology (Berlin, Germany), 2023, Volume: 38, Issue:3

    Topics: Genetic Testing; Genotype; Humans; Hyperoxaluria, Primary; Mutation; Pyridoxine; Transaminases

2023
Molecular basis of primary hyperoxaluria: clues to innovative treatments.
    Urolithiasis, 2019, Volume: 47, Issue:1

    Topics: Alcohol Oxidoreductases; Calcium Oxalate; Gastrointestinal Microbiome; Genetic Therapy; Glyoxylates;

2019
Recent advances in the identification and management of inherited hyperoxalurias.
    Urolithiasis, 2019, Volume: 47, Issue:1

    Topics: Alcohol Oxidoreductases; Fluid Therapy; Genetic Testing; Humans; Hyperoxaluria, Primary; Kidney Calc

2019
Nocturnal home hemodialysis for a patient with type 1 hyperoxaluria.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2013, Volume: 62, Issue:6

    Topics: Adult; Amino Acid Substitution; Arginine; Circadian Rhythm; Combined Modality Therapy; DNA Mutationa

2013
The primary hyperoxalurias.
    Kidney international, 2009, Volume: 75, Issue:12

    Topics: Alcohol Oxidoreductases; Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Kidney Transplanta

2009
Hyperoxaluric calcium nephrolithiasis.
    Endocrinology and metabolism clinics of North America, 2002, Volume: 31, Issue:4

    Topics: Adolescent; Calcium Oxalate; Calcium, Dietary; Child; Dietary Proteins; Drinking; Humans; Hyperoxalu

2002
Transplantation strategies in type 1 primary hyperoxaluria: the issue of pyridoxine responsiveness.
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 1999, Volume: 14, Issue:2

    Topics: Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Kidney Transplantation; Pyridoxine

1999
The primary hyperoxalurias.
    Contributions to nephrology, 2001, Issue:136

    Topics: Humans; Hyperoxaluria, Primary; Kidney Transplantation; Liver Transplantation; Oxalates; Pyridoxine;

2001
Primary hyperoxaluria type I.
    European journal of pediatrics, 1990, Volume: 149, Issue:8

    Topics: Age Factors; Alanine Transaminase; Child; Combined Modality Therapy; Female; Genes, Recessive; Human

1990

Other Studies

24 other studies available for pyridoxine and Oxaluria, Primary

ArticleYear
Endogenous Oxalate Production in Primary Hyperoxaluria Type 1 Patients.
    Journal of the American Society of Nephrology : JASN, 2021, 12-01, Volume: 32, Issue:12

    Topics: Glycine; Glycolates; Glyoxylates; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Oxalates; Pyridoxin

2021
Importance of Assessing Compliance with Conservative Treatment of Primary Hyperoxaluria Type 1: A Case Report of a Patient with I244T/c.969-3C>G Mutation.
    The Permanente journal, 2020, Volume: 24

    Topics: Adolescent; Calcium Oxalate; Conservative Treatment; Female; Fluid Therapy; Follow-Up Studies; Guide

2020
Recovery From Dialysis in Patients With Primary Hyperoxaluria Type 1 Treated With Pyridoxine: A Report of 3 Cases.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2021, Volume: 77, Issue:5

    Topics: Adult; Female; Homozygote; Humans; Hyperoxaluria, Primary; Kidney Failure, Chronic; Middle Aged; Oxa

2021
Type 1 primary hyperoxaluria: A case report and focus on bone impairment of systemic oxalosis.
    Morphologie : bulletin de l'Association des anatomistes, 2018, Volume: 102, Issue:336

    Topics: Adult; Biopsy; Bone Density; Calcium Oxalate; Humans; Hyperoxaluria, Primary; Ilium; Kidney Failure,

2018
Multiple mechanisms of action of pyridoxine in primary hyperoxaluria type 1.
    Biochimica et biophysica acta, 2013, Volume: 1832, Issue:10

    Topics: Animals; CHO Cells; Cricetinae; Cricetulus; Humans; Hyperoxaluria, Primary; Pyridoxine

2013
Sustained pyridoxine response in primary hyperoxaluria type 1 recipients of kidney alone transplant.
    American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons, 2014, Volume: 14, Issue:6

    Topics: Adult; Child; Female; Glomerular Filtration Rate; Humans; Hyperoxaluria, Primary; Kidney Transplanta

2014
Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1.
    Proceedings of the National Academy of Sciences of the United States of America, 2014, Oct-07, Volume: 111, Issue:40

    Topics: Animals; Anti-Infective Agents, Local; CHO Cells; Cricetinae; Cricetulus; Dequalinium; Drug Evaluati

2014
Pyridoxamine and pyridoxal are more effective than pyridoxine in rescuing folding-defective variants of human alanine:glyoxylate aminotransferase causing primary hyperoxaluria type I.
    Human molecular genetics, 2015, Oct-01, Volume: 24, Issue:19

    Topics: Animals; CHO Cells; Cricetinae; Cricetulus; Humans; Hyperoxaluria, Primary; Mutation; Protein Foldin

2015
Effects of alanine:glyoxylate aminotransferase variants and pyridoxine sensitivity on oxalate metabolism in a cell-based cytotoxicity assay.
    Biochimica et biophysica acta, 2016, Volume: 1862, Issue:6

    Topics: Animals; Cell Survival; CHO Cells; Cricetulus; Glycolates; Humans; Hyperoxaluria, Primary; Mutation;

2016
Re: Pyridoxamine and Pyridoxal are More Effective than Pyridoxine in Rescuing Folding-Defective Variants of Human Alanine:Glyoxylate Aminotransferase Causing Primary Hyperoxaluria Type I.
    The Journal of urology, 2016, Volume: 195, Issue:4 Pt 1

    Topics: Alanine; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Pyridoxal; Pyridoxamine; Pyridoxine

2016
Clinical spectrum of primary hyperoxaluria type 1: Experience of a tertiary center.
    Nephrologie & therapeutique, 2017, Volume: 13, Issue:3

    Topics: Adult; Child; Child, Preschool; Cohort Studies; Consanguinity; Egypt; Female; Humans; Hyperoxaluria,

2017
Crystalline retinopathy in primary hyperoxaluria.
    Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus, 2011, Volume: 15, Issue:2

    Topics: Electroretinography; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Infant; Liver Transplantation; M

2011
[Primary hyperoxaluria].
    Nephrologie & therapeutique, 2011, Volume: 7, Issue:4

    Topics: Alanine Transaminase; Disease Progression; Genotype; Humans; Hyperoxaluria; Hyperoxaluria, Primary;

2011
Pyridoxine effect in type I primary hyperoxaluria is associated with the most common mutant allele.
    Kidney international, 2005, Volume: 67, Issue:5

    Topics: Adolescent; Adult; Alleles; Child; Child, Preschool; Dose-Response Relationship, Drug; Female; Genot

2005
[Results of treatment with oral citrate and pyridoxine in patients with primary hyperoxaluria type 1].
    Anales espanoles de pediatria, 1999, Volume: 50, Issue:4

    Topics: Citrates; Female; Humans; Hyperoxaluria, Primary; Infant, Newborn; Kidney; Male; Pyridoxine; Radiogr

1999
Pyridoxine-responsive nephrocalcinosis and glycolic aciduria in two siblings without hyperoxaluria and with normal alanine: glyoxalate aminotransferase activity.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:1

    Topics: Calcinosis; Child; Female; Glycolates; Humans; Hyperoxaluria, Primary; Kidney Diseases; Male; Pyrido

2000
Oxalate balance studies in patients on hemodialysis for type I primary hyperoxaluria.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 1992, Volume: 19, Issue:6

    Topics: Adult; Calcium Oxalate; Humans; Hyperoxaluria, Primary; Kidney; Male; Oxalates; Pyridoxine; Renal Di

1992
Metabolism of pyridoxine in mild metabolic hyperoxaluria and primary hyperoxaluria (type 1).
    Urologia internationalis, 1991, Volume: 47, Issue:3

    Topics: Chromatography, High Pressure Liquid; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Pyridoxic Acid;

1991
Glycolate determination detects type I primary hyperoxaluria in dialysis patients.
    Kidney international, 1991, Volume: 39, Issue:1

    Topics: Adolescent; Adult; Chromatography, High Pressure Liquid; Glycolates; Humans; Hyperoxaluria, Primary;

1991
Vitamin B6 resistant primary hyperoxaluria type I. Report of 5 cases.
    Helvetica paediatrica acta, 1989, Volume: 43, Issue:4

    Topics: Child; Child, Preschool; Drug Resistance; Female; Follow-Up Studies; Humans; Hyperoxaluria; Hyperoxa

1989
Hyperoxaluria type I: therapeutic effects of pyridoxine hydrochloride and inheritance patterns of the disease in a family.
    The Kurume medical journal, 1988, Volume: 35, Issue:3

    Topics: Child; Child, Preschool; Female; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Male; Pedigree; Pyri

1988
Timing of renal transplantation in the management of pyridoxine-resistant type I primary hyperoxaluria.
    Transplantation, 1988, Volume: 45, Issue:6

    Topics: Adolescent; Adult; Drug Resistance; Female; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Kidney Fa

1988
Primary hyperoxaluria: effect of treatment with vitamin B6 and shock waves.
    Contributions to nephrology, 1987, Volume: 58

    Topics: Adult; HLA Antigens; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Kidney Calculi; Lithotripsy; Mal

1987
[Pyridoxine can normalize oxaluria in idiopathic renal lithiasis].
    Schweizerische medizinische Wochenschrift, 1986, Dec-13, Volume: 116, Issue:50

    Topics: Adult; Drug Evaluation; Female; Humans; Hyperoxaluria; Hyperoxaluria, Primary; Jejunoileal Bypass; K

1986