cystamine has been researched along with Cystine Diathesis in 12 studies
| Excerpt | Relevance | Reference |
|---|---|---|
| "In nephropathic cystinosis, corneal cystine crystals cause severe photophobia and corneal erosions." | 9.08 | A randomized clinical trial of topical cysteamine disulfide (cystamine) versus free thiol (cysteamine) in the treatment of corneal cystine crystals in cystinosis. ( Gahl, WA; Iwata, F; Kaiser-Kupfer, MI; Kuehl, EM; McCain, LM; Reed, GF, 1998) |
| " However, cysteamine is associated with an offending odor and taste and this, coupled to a rapid first pass metabolism and a 6h dosing regimen, suggest a clear need to improve the therapy." | 5.37 | PEGylated derivatives of cystamine as enhanced treatments for nephropathic cystinosis. ( Cairns, D; Di Salvo, A; Kay, G; Knott, RM; Omran, Z, 2011) |
| "Nephropathic cystinosis is a rare autosomal recessive disease characterised by raised intracellular levels of the amino acid, cystine." | 5.37 | Folate pro-drug of cystamine as an enhanced treatment for nephropathic cystinosis. ( Cairns, D; Hector, EE; Kay, G; Knott, RM; Omran, Z, 2011) |
| "A patient with nephropathic cystinosis and end-stage renal disease was treated with cysteamine, both intravenously and orally." | 5.26 | Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo. ( Crawhall, JC; Olson, DL; Oshima, RG; Schneider, JA; Thoene, JG, 1976) |
| "In nephropathic cystinosis, corneal cystine crystals cause severe photophobia and corneal erosions." | 5.08 | A randomized clinical trial of topical cysteamine disulfide (cystamine) versus free thiol (cysteamine) in the treatment of corneal cystine crystals in cystinosis. ( Gahl, WA; Iwata, F; Kaiser-Kupfer, MI; Kuehl, EM; McCain, LM; Reed, GF, 1998) |
| "As part of our continuing work to obtain new pro-drugs for the treatment of nephropathic cystinosis, a number of glutaric and succinic acid derivatives of cystamine have been designed, synthesised and biologically evaluated in vitro." | 3.77 | Synthesis and in vitro evaluation of novel pro-drugs for the treatment of nephropathic cystinosis. ( Benylles, A; Cairns, D; Kay, G; Knott, RM; Moloney, KA; Omran, Z, 2011) |
| "The efficiency of cysteamine eye drops in the treatment of cystinosis has been demonstrated in several studies: Corneal cystine crystals can be removed by topical cysteamine." | 3.69 | [1H-NMR spectroscopic study of cysteamine eyedrops]. ( Gräf, M; Kalinowski, HO, 1995) |
| "Children suffering from cystinosis, a genetic disease characterized by high levels of lysosomal cystine, are currently being treated with cysteamine to lower the cystine levels in their cells." | 3.67 | Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide. ( Butler, JD; Zatz, M, 1984) |
| " However, cysteamine is associated with an offending odor and taste and this, coupled to a rapid first pass metabolism and a 6h dosing regimen, suggest a clear need to improve the therapy." | 1.37 | PEGylated derivatives of cystamine as enhanced treatments for nephropathic cystinosis. ( Cairns, D; Di Salvo, A; Kay, G; Knott, RM; Omran, Z, 2011) |
| "Nephropathic cystinosis is a rare autosomal recessive disease characterised by raised intracellular levels of the amino acid, cystine." | 1.37 | Folate pro-drug of cystamine as an enhanced treatment for nephropathic cystinosis. ( Cairns, D; Hector, EE; Kay, G; Knott, RM; Omran, Z, 2011) |
| "A patient with nephropathic cystinosis and end-stage renal disease was treated with cysteamine, both intravenously and orally." | 1.26 | Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo. ( Crawhall, JC; Olson, DL; Oshima, RG; Schneider, JA; Thoene, JG, 1976) |
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 5 (41.67) | 18.7374 |
| 1990's | 2 (16.67) | 18.2507 |
| 2000's | 1 (8.33) | 29.6817 |
| 2010's | 4 (33.33) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Auerbach, FN | 1 |
| Khoramnia, R | 1 |
| Auffarth, GU | 1 |
| Carmona Hernandez, JA | 1 |
| Tandogan, T | 1 |
| Sel, S | 1 |
| Osefo, N | 1 |
| Ito, T | 1 |
| Jensen, RT | 1 |
| Omran, Z | 3 |
| Kay, G | 3 |
| Di Salvo, A | 1 |
| Knott, RM | 3 |
| Cairns, D | 3 |
| Hector, EE | 1 |
| Moloney, KA | 1 |
| Benylles, A | 1 |
| Butler, JD | 3 |
| Zatz, M | 2 |
| Gräf, M | 1 |
| Kalinowski, HO | 1 |
| Iwata, F | 1 |
| Kuehl, EM | 1 |
| Reed, GF | 1 |
| McCain, LM | 1 |
| Gahl, WA | 2 |
| Kaiser-Kupfer, MI | 1 |
| Thoene, JG | 2 |
| Oshima, RG | 2 |
| Crawhall, JC | 2 |
| Schneider, JA | 2 |
| Olson, DL | 1 |
| Tietze, F | 1 |
| Schulman, JD | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| Trial of Topical Cysteamine in the Treatment of Corneal Cystine Crystal Accumulation in Cystinosis[NCT00001213] | Phase 2 | 328 participants (Actual) | Interventional | 1986-04-30 | Completed | ||
| Safety and Efficacy Trial of a Proposed NDA Formulation of Topical Cysteamine in the Treatment of Corneal Cystine Crystal Accumulation in Cystinosis[NCT00001736] | Phase 1 | 51 participants | Interventional | 1998-05-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
"Response is defined as a decrease from baseline of at least 1 in Corneal Cystine Crystal Score (CCCS) at any time on study when baseline CCCS is greater than or equal to 1, or CCCS does not increase at least 1 at any time on study when baseline CCCS is less than 1.~The CCCS is based on a library of slit-lamp photographs of corneas with increasing crystal densities (0-3). Slit-lamp photos were to be taken to assess the extent of the corneal crystal accumulation. To minimize bias when assessing the extent of corneal crystal accumulation, photos were centrally graded at the National Eye Institute (NEI) where each photo was graded independently by masked graders. If more than one CCCS was recorded in a given study year, the highest (worst) CCCS value was used for that year.~The results were obtained from a combined analyses of the NIH cysteamine studies evaluating various cysteamine ophthalmic solution formulations from 1986 through 2005." (NCT00001213)
Timeframe: Any Time Point Up to 19 Years
| Intervention | eyes (Number) |
|---|---|
| Cysteamine Topical Solution | 98 |
Since efficacy of ophthalmic cysteamine was established and a New Drug Application (NDA) filed, the post-hoc primary outcome measure is the evaluation of safety information. There was no specified time frame for this outcome measure, as safety data was being collected until the drug became available for commercial purchase in May 2013. (NCT00001213)
Timeframe: Any Time Point up to 27 Years
| Intervention | participants (Number) |
|---|---|
| Cysteamine Topical Solution | 257 |
2 reviews available for cystamine and Cystine Diathesis
| Article | Year |
|---|---|
Gastric acid hypersecretory states: recent insights and advances.
Topics: Cystamine; Cystinosis; Enzyme Inhibitors; Gastric Acid; Gastrins; Histamine; Humans; Proton Pump Inh | 2009 |
Cystine depletion of cystinotic cells by aminothiols.
Topics: Cell-Free System; Cystamine; Cysteamine; Cysteine; Cystinosis; Fibroblasts; Humans; Penicillamine; S | 1977 |
1 trial available for cystamine and Cystine Diathesis
| Article | Year |
|---|---|
A randomized clinical trial of topical cysteamine disulfide (cystamine) versus free thiol (cysteamine) in the treatment of corneal cystine crystals in cystinosis.
Topics: Administration, Topical; Adolescent; Adult; Child; Child, Preschool; Cornea; Corneal Diseases; Cysta | 1998 |
9 other studies available for cystamine and Cystine Diathesis
| Article | Year |
|---|---|
[Bilateral crystalline deposits in corneal stroma].
Topics: Administration, Ophthalmic; Child; Corneal Diseases; Cystamine; Cystinosis; Diagnosis, Differential; | 2016 |
PEGylated derivatives of cystamine as enhanced treatments for nephropathic cystinosis.
Topics: Cells, Cultured; Cystamine; Cystinosis; Fanconi Syndrome; Fibroblasts; Humans; Nephrotic Syndrome; P | 2011 |
Folate pro-drug of cystamine as an enhanced treatment for nephropathic cystinosis.
Topics: Administration, Oral; Cell Line; Cystamine; Cystinosis; Fanconi Syndrome; Humans; Nephrotic Syndrome | 2011 |
Synthesis and in vitro evaluation of novel pro-drugs for the treatment of nephropathic cystinosis.
Topics: Cystamine; Cystinosis; Fanconi Syndrome; Fibroblasts; Glutarates; Humans; Nephrotic Syndrome; Prodru | 2011 |
Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide.
Topics: Cells, Cultured; Child; Cystamine; Cystine; Cystinosis; Fibroblasts; Humans; Kinetics; Pantetheine; | 1984 |
Pantethine depletes cystinotic fibroblasts of cystine.
Topics: Cells, Cultured; Cystamine; Cystine; Cystinosis; Dose-Response Relationship, Drug; Fibroblasts; Huma | 1983 |
[1H-NMR spectroscopic study of cysteamine eyedrops].
Topics: Cornea; Corneal Diseases; Cystamine; Cysteamine; Cystinosis; Dose-Response Relationship, Drug; Drug | 1995 |
Cystinosis. Intracellular cystine depletion by aminothiols in vitro and in vivo.
Topics: Child; Cystamine; Cysteamine; Cystine; Cystinosis; Female; Fibroblasts; Glutathione; Humans; Leukocy | 1976 |
Cysteamine depletes cystinotic leucocyte granular fractions of cystine by the mechanism of disulphide interchange.
Topics: Cystamine; Cysteamine; Cystine; Cystinosis; Cytoplasmic Granules; Disulfides; Ethylmaleimide; Half-L | 1985 |