Page last updated: 2024-10-29

iohexol and alpha-Galactosidase A Deficiency

iohexol has been researched along with alpha-Galactosidase A Deficiency in 1 studies

Iohexol: An effective non-ionic, water-soluble contrast agent which is used in myelography, arthrography, nephroangiography, arteriography, and other radiographic procedures. Its low systemic toxicity is the combined result of low chemotoxicity and low osmolality.
iohexol : A benzenedicarboxamide compound having N-(2,3-dihydroxypropyl)carbamoyl groups at the 1- and 3-positions, iodo substituents at the 2-, 4- and 6-positions and an N-(2,3-dihydroxypropyl)acetamido group at the 5-position.

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's1 (100.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Wijburg, FA1
Bénichou, B1
Bichet, DG1
Clarke, LA1
Dostalova, G1
Fainboim, A1
Fellgiebel, A1
Forcelini, C1
An Haack, K1
Hopkin, RJ1
Mauer, M1
Najafian, B1
Scott, CR1
Shankar, SP1
Thurberg, BL1
Tøndel, C1
Tylki-Szymańska, A1
Ramaswami, U1

Clinical Trials (2)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Podocyturia, a Non-Invasive Predictor of Renal Dysfunction in Fabry Nephropathy[NCT02994303]58 participants (Anticipated)Observational2014-09-30Recruiting
A Randomized, Multicenter, Multinational, Phase 3B, Open-Label, Parallel-Group Study of Fabrazyme (Agalsidase Beta) in Treatment-Naïve Male Pediatric Patients With Fabry Disease Without Severe Symptoms[NCT00701415]Phase 331 participants (Actual)Interventional2008-09-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Percent Change From Baseline in GL-3 Clearance From Plasma

Plasma samples were assayed for GL-3 clearance using a validated tandem mass spectrometry with an upper limit of normal plasma GL-3 level of 7.0 μg/mL. Number of participants analyzed=participants with both baseline and post-baseline GL-3 plasma clearance assessment. Here 'n' signifies number of participants with available data for specified category. (NCT00701415)
Timeframe: Baseline, Week 12, 28, 40, 52, 80, 104, 132, 156, 184, 208, 236 and 260

,
InterventionPercent change (Mean)
Week 12 (n=14, 11)Week 28 (n=14, 14)Week 40 (n=13, 14)Week 52 (n=14, 14)Week 80 (n=13, 14)Week 104 (n=13, 14)Week 132 (n=11, 14)Week 156 (n=11, 14)Week 184 (n=12, 14)Week 208 (n=12, 14)Week 236 (n=12, 14)Week 260 (n=11, 14)
Fabrazyme 0.5 mg/kg-52.37-49.06-52.01-52.29-52.91-51.08-61.39-48.72-53.62-48.83-56.44-59.95
Fabrazyme 1.0 mg/kg-52.74-47.55-50.82-45.87-48.93-39.92-52.97-44.83-49.08-46.09-47.25-46.34

Percent Change From Baseline in GL-3 Clearance From Urine

Plasma samples were assayed for total urine GL-3 clearance using a validated tandem mass spectrometry with an upper limit of normal of <0.030 mg/mmoL of creatinine. Number of participants analyzed=participants with both baseline and post-baseline GL-3 urine clearance assessment. Here 'n' signifies number of participants with available data for specified category. (NCT00701415)
Timeframe: Baseline, Week 12, 28, 40, 52, 80, 104, 132, 156, 184, 208, 236 and 260

,
InterventionPercent change (Mean)
Week 12 (n=15, 14)Week 28 (n=15, 15)Week 40 (n=15, 14)Week 52 (n=15, 14)Week 80 (n=14, 14)Week 104 (n=14, 14)Week 132 (n=13, 14)Week 156 (n=13, 14)Week 184 (n=13, 14)Week 208 (n=13, 14)Week 236 (n=13, 14)Week 260 (n=13, 14)
Fabrazyme 0.5 mg/kg-50.77-50.84-44.22-70.1-35.84-21.92-48.79-65.57-76.54-60.94-69.08-57.59
Fabrazyme 1.0 mg/kg-63.39-52.55-63.87-20.7235.22-56.39-45.61-28.92-10.5-50.93-40.09-28.27

Skin Globotriaosylceramide (GL-3) Clearance From Superficial Skin Capillary Endothelium

Skin biopsies were taken at Baseline, Week 52, Week 156 and Week 260 or early withdrawal and analyzed for cellular GL-3 accumulation (inclusions) by light microscopy. Each biopsy was scored for GL-3 accumulation on a severity score-scale of none, mild, moderate, severe (0-1-2-3). Scores are categorized as normal (score = 0) or abnormal (score = 1, 2 or 3). Data was summarized in terms of number of participants with none/trace, mild, moderate and severe biopsy scores. (NCT00701415)
Timeframe: Baseline, Week 52, Week 156 and Week 260

,
InterventionPercentage of participants (Number)
Zero (0) Skin GL-3 Score at BaselineZero (0) Skin GL-3 Score at Week 52Zero (0) Skin GL-3 Score at Week 156Zero (0) Skin GL-3 Score at Week 260Mild (1) Skin GL-3 Score at BaselineMild (1) Skin GL-3 Score at Week 52Mild (1) Skin GL-3 Score at Week 156Mild (1) Skin GL-3 Score at Week 260Moderate (2) Skin GL-3 Score at BaselineModerate (2) Skin GL-3 Score at Week 52Moderate (2) Skin GL-3 Score at Week 156Moderate (2) Skin GL-3 Score at Week 260Severe (3) Skin GL-3 Score at BaselineSevere (3) Skin GL-3 Score at Week 52Severe (3) Skin GL-3 Score at Week 156Severe (3) Skin GL-3 Score at Week 260Missing Skin GL-3 Score at BaselineMissing Skin GL-3 Score at Week 52Missing Skin GL-3 Score at Week 156Missing Skin GL-3 Score at Week 260
Fabrazyme 0.5 mg/kg18.87556.368.86.36.318.812.57506.300000018.818.818.8
Fabrazyme 1.0 mg/kg33.3808066.7013.302066.7013.36.7000006.76.76.7

Trials

1 trial available for iohexol and alpha-Galactosidase A Deficiency

ArticleYear
Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial.
    PloS one, 2015, Volume: 10, Issue:5

    Topics: Adolescent; Biopsy; Brain; Child; Child, Preschool; Demography; Endothelium, Vascular; Fabry Disease

2015
Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial.
    PloS one, 2015, Volume: 10, Issue:5

    Topics: Adolescent; Biopsy; Brain; Child; Child, Preschool; Demography; Endothelium, Vascular; Fabry Disease

2015
Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial.
    PloS one, 2015, Volume: 10, Issue:5

    Topics: Adolescent; Biopsy; Brain; Child; Child, Preschool; Demography; Endothelium, Vascular; Fabry Disease

2015
Characterization of early disease status in treatment-naive male paediatric patients with Fabry disease enrolled in a randomized clinical trial.
    PloS one, 2015, Volume: 10, Issue:5

    Topics: Adolescent; Biopsy; Brain; Child; Child, Preschool; Demography; Endothelium, Vascular; Fabry Disease

2015