Increased human dermal microvascular endothelial cell survival induced by cysteamine

Besouw, Martine; Heuvel, Lambertus P. van den; Eijsden, Rudy van; Bongaers, Inge; Kluijtmans, Leo A. J.; Dewerchin, Mieke; Levtchenko, Elena

doi:10.1007/s10545-013-9588-0

Cited by 8 publications

(8 citation statements)

References 26 publications

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“…Cysteamine derivatives have shown encouraging efficacy as antimelanoma agents in several studies . Besouw et al published an interesting review in 2013 on the potentials of cysteamine as an anticarcinogenic agent and as a new treatment for other pathologies in human.…”

Section: Discussionmentioning

confidence: 99%

Significant therapeutic response to cysteamine cream in a melasma patient resistant to Kligman's formula

Kasraee¹,

Mansouri

Farshi

2018

J of Cosmetic Dermatology

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Summary L‐Cysteamine is a biological antioxidant produced during the coenzyme A metabolism cycle and is naturally present in all mammalian cells. The efficacy of topical cysteamine for the treatment of melasma has been recently shown in two double‐blind, randomized, and placebo‐controlled clinical trials. Herein, we report a 44‐year‐old patient with melasma resistant to Kligman's formula (Pigmanorm cream), who was successfully treated with topical cysteamine as a new depigmenting agent. Skin colorimetric measurements, MASI score determination, and standard photographies after 2 and 4 months of once daily application of cysteamine cream showed a marked improvement of the hyperpigmented lesions. Telangiectasia and perilesional hypopigmentation improved rapidly after the discontinuation of Kligman's formula and starting the treatment with topical cysteamine. After 4 months, the therapeutic results were maintained through a biweekly application regimen of cysteamine cream. The use of cysteamine cream was well tolerated and did not induce any side effects during the 3‐year follow‐up of the patient. Cysteamine is a natural molecule with an excellent safety profile and known antimutagenic, antimelanoma, and anticarcinogenic effects. Considering the high efficacy of cysteamine cream, it is possible that it could replace mutagenic and carcinogenic depigmenting agents such as hydroquinone in near future.

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Section: Discussionmentioning

confidence: 99%

Significant therapeutic response to cysteamine cream in a melasma patient resistant to Kligman's formula

Kasraee¹,

Mansouri

Farshi

2018

J of Cosmetic Dermatology

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“…Although cysteamine which reduces the intra-lysosomal cysteine concentration was first introduced for treatment of cystinosis in 1976, it was approved by the US Food and Drug Administration in 1994 and has been used safely in the treatment of cystonosis by oral route [18,24]. Bromelain on the other hand has recently been approved for wound debridement by European Medicines Agency that is marketed as gel or powder.…”

Section: Discussionmentioning

confidence: 99%

“…Cysteamine prevents selenite induced cataract in rats [15], reduce invasion and metastasis in mouse model of pancreatic cancer [16]. Cysteamine is a reducing aminothiol [17], used for treating cystinosis [18,19]. We hypothesised that since cysteamine (mol.…”

Section: Introductionmentioning

confidence: 99%

Assessment of a novel mucolytic solution for dissolving mucus in pseudomyxoma peritonei: an ex vivo and in vitro study

Pillai

Akhter

Morris

2017

Pleura and Peritoneum

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Background: Pseudomyxoma peritonei (PMP) is difficult to treat. Intraperitoneal delivery of mucolytic solutions might potentially improve therapy, in addition to surgical cytoreduction and hyperthermic intraperitoneal chemotherapy. Methods: Comparison of mucolytic effect of two formulations (control: bromelain 300 µg/mL + N-Acetylcystein 250 mM; test: bromelain 200 µg/mL + 200 mM cysteamine) in vitro on a mucin producing cell lines (HT29) and ex vivo on mucus obtained from 18 PMP patients. Mucin plugs were classified according to their density into three categories: hard, semi hard and soft. Simulation of peritoneal washing ex vivo using a closed heated circulating pump. Results: Solubilisation was faster with the test vs. the control formulation (90 vs. 180 min) for dissolving the soft mucin plugs (p < 0.05). The test solution was also more effective in dissolving the hard mucus plugs compared to control (82.5 ± 2.74 % vs. 36.33 ± 3.27 %). All mucin types disintegrated in simulated peritoneal washing. Cytotoxicity of the test solution on HT29 cell line was time-dependent. Conclusions: The test formulation is more effective and faster than the control formulation in dissolving mucus plugs of various densities. Mucus plugs were all solubilised after 40 min in simulated peritoneal washing. This novel mucolytic formulation might pave the way for an effective and less invasive therapy of PMP in the future.

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“…At higher doses (>1.95 g/m 2 per day) of cysteamine, rare adverse effects have been reported, including bone pain, myalgia, skin striae, and bruise-like lesions on the elbows (reactive angioendotheliomatosis on skin biopsy) [41]. Cysteamine increases human dermal microvascular endothelial cell survival in vitro, which may explain how the drug causes angioendotheliomatosis [42]. As it is metabolized into volatile sulfur compounds (i.e., dimethyl sulfide and methanethiol), treatment is also associated with an unpleasant sulfurous body and breath odor, which can often lead to poor treatment compliance [41].…”

Section: Cysteamine Therapymentioning

confidence: 99%

Effects of long-term cysteamine treatment in patients with cystinosis

Ariceta

Giordano²,

Santos

2017

Pediatr Nephrol

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Cystinosis is a rare autosomal-recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. Patients with infantile nephropathic cystinosis, the most common and most severe clinical form of cystinosis, commonly present with renal Fanconi syndrome by 6-12 months of age, and without specific treatment, almost all will develop end-stage renal disease (ESRD) by 10-12 years of age. Early corneal cystine crystal deposition is a hallmark of the disease. Cystinosis also presents with gastrointestinal symptoms (e.g., vomiting, decreased appetite, and feeding difficulties) and severe growth retardation and may affect several other organs over time, including the eye, thyroid gland, gonads, pancreas, muscles, bone marrow, liver, nervous system, lungs, and bones. Cystine-depleting therapy with cysteamine orally is the only specific targeted therapy available for managing cystinosis and needs to be combined with cysteamine eye drops for corneal disease involvement. In patients with early treatment initiation and good compliance to therapy, long-term cysteamine treatment delays progression to ESRD, significantly improves growth, decreases the frequency and severity of extrarenal complications, and is associated with extended life expectancy. Therefore, early diagnosis of cystinosis and adequate life-long treatment with cysteamine are essential for preventing end-organ damage and improving the overall prognosis in these patients.

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Increased human dermal microvascular endothelial cell survival induced by cysteamine

Abstract: Cysteamine concentrations, similar to those described in plasma of cystinosis patients, stimulate HDMVEC viability and proliferation and increase intracellular GSH content. We postulate that this mechanism might underlie angioendotheliomatosis induced by cysteamine.

Cited by 8 publications

References 26 publications

Significant therapeutic response to cysteamine cream in a melasma patient resistant to Kligman's formula

Significant therapeutic response to cysteamine cream in a melasma patient resistant to Kligman's formula

Assessment of a novel mucolytic solution for dissolving mucus in pseudomyxoma peritonei: an ex vivo and in vitro study

Effects of long-term cysteamine treatment in patients with cystinosis

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