Cysteamine hydrochloride eye drop solution for the treatment of corneal cystine crystal deposits in patients with cystinosis: an evidence-based review

Makuloluwa, Achini; Shams, Fatemeh

doi:10.2147/opth.s133516

Cited by 24 publications

(19 citation statements)

References 60 publications

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“…3,6,15,22 It is known that topical cysteamine is a therapeutic option against corneal disease causing a reduction of cystine crystals density and diminishing symptoms like photophobia. 23,24 However, treatment compliance is poor as there is a need for multiple instillations for a long period of time. Quality of life must always be addressed in all these cases as the frequent and chronic instillation of eye drops may bring significant distress to patient’s daily life when compared with the usefulness of such treatment options.…”

Section: Resultsmentioning

confidence: 99%

“…The ocular treatment proposed was Cystadrops ® 3.8 mg/ml, instilled four times a day. 24 One year after treatment, patient’s complaints, corneal surface (Figure 1) and anterior chamber OCT remained unchanged, so the frequency of treatment was reduced to twice daily. At the last follow-up, the patient refractive status was unchanged and BCVA was 20/20 in both eyes.…”

Section: Case Descriptionmentioning

confidence: 99%

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Ocular manifestations of intermediate cystinosis: To treat or not to treat?

Sousa-Neves

Ribeiro

Saraiva

et al. 2019

European Journal of Ophthalmology

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Purpose: To report a case of intermediate cystinosis with an atypical presentation in which a delayed non-treatment decision has been proved right over a large period of time. Methods: This is a unique case report of a patient who underwent full ophthalmologic evaluations including anterior chamber optical coherence tomography on a regular basis during a 7-year follow-up period. Results: A 30-year-old woman with photophobia was being studied by the Department of Nephrology with a suspicion of Alport syndrome. Slit-lamp examination showed iridescent deposits throughout the corneal anterior stroma and the inferior tarsal conjunctiva bilaterally. Anterior chamber optical coherence tomography showed stromal hyperreflectivity. CTNS gene was found to be positive for c.416C>T (Ser139Phe) mutation. The patient was offered oral and topical cysteamine which was refused. After a period of 5 years of follow-up, general health status remained stable, corneal disease showed no progression and photophobia complaints diminished. However, the patient was advised to start systemic and topical cysteamine because of the unknown development of the disease. Conclusion: In this reported case, a delayed non-treatment decision has been proved right contrary to published evidence of active treatment of photophobia. The decision whether to treat or not to treat corneal involvement of the disease is not straightforward. Besides biomicroscopic evaluations, patients’ complaints and expectations should be taken into account.

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Section: Resultsmentioning

confidence: 99%

Section: Case Descriptionmentioning

confidence: 99%

Ocular manifestations of intermediate cystinosis: To treat or not to treat?

Sousa-Neves

Ribeiro

Saraiva

et al. 2019

European Journal of Ophthalmology

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“…Diffusion of hydrophilic CH through the lipophilic epithelium is low [30]. Due to short corneal contact time, dose regimen is frequent up to 12 times per day and the aqueous solution of CH is unstable at room temperature [31]. These factors impose a significant burden on patients and may lead to poor adherence [31].…”

Section: Discussionmentioning

confidence: 99%

“…Due to short corneal contact time, dose regimen is frequent up to 12 times per day and the aqueous solution of CH is unstable at room temperature [31]. These factors impose a significant burden on patients and may lead to poor adherence [31]. Efficacy of 0.1% CH drops in reduction of crystal density is low even in anterior stroma [12].…”

Section: Discussionmentioning

confidence: 99%

Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study

et al. 2020

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Background: Presence of corneal cystine crystals is the main ocular manifestation of cystinosis, although controversial findings concerning the corneal layer with the highest density have been reported. The aim of this study was the analysis of the characteristics of crystal arrangement in different corneal layers and the assessment of corneal morphological changes with age. Methods: A cross sectional study was carried out in three children and three adults who had nephropathic cystinosis and corneal cystine depositions. All patients underwent a comprehensive ophthalmological examination including best corrected distance visual acuity, slit-lamp examination, in vivo confocal microscopy and anterior segment optical coherence tomography. An evaluation of the depth of crystal deposits and crystal density in different corneal layers was also performed. Due to the low number of subjects no statistical comparison was performed. Results: Anterior segment optical coherence tomography images revealed deposition of hyperreflective crystals from limbus to limbus in each patient. Crystals appeared as randomly oriented hyperreflective, elongated structures on in vivo confocal microscopy images in all corneal layers except the endothelium. In children the deposits occurred predominantly in the anterior stroma, while in adults, the crystals were mostly localized in the posterior corneal stroma with the depth of crystal deposition showing an increasing tendency with age (mean depth of crystal density was 353.17 ± 49.23 μm in children and it was 555.75 ± 25.27 μm in adults). Mean crystal density of the epithelium was 1.47 ± 1.17 (median: 1.5; interquartile range: 0.3-2.4). Mean crystal density of the anterior and posterior stroma of children and adults was 3.37 ± 0.34 (median: 3.4; interquartile range: 3.25-3.55) vs. 1.23 ± 0.23 (median: 1.2; interquartile range: 1.05-1.35) and 0.76 ± 0.49 (median: 0.7; interquartile range: 0.4-1.15) vs. 3.63 ± 0.29 (median: 3.7; interquartile range: 3.45-3.8), respectively. Endothelium had intact structure in all cases. Some hexagonal crystals were observed in two subjects. Conclusions: In vivo confocal microscopy and anterior segment optical coherence tomography confirmed an agerelated pattern of crystal deposition. In children, crystals tend to locate anteriorly, while in adults, deposits are found posteriorly in corneal stroma.

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“…According to a quality assessment of investigational studies in the therapeutic use of cysteamine in cystinosis, only a few references provide an adequate quality level in order to stablish an acceptable strength of recommendation [102]. At present there is only one randomized clinical trial that could report on the efficacy and safety of cysteamine eye drops in preventing eye complications associated with cystinosis [20].…”

Section: Current Situation and Future Perspectivesmentioning

confidence: 99%

Recent Research in Ocular Cystinosis: Drug Delivery Systems, Cysteamine Detection Methods and Future Perspectives

et al. 2020

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Cystinosis is a rare genetic disorder characterized by the accumulation of cystine crystals in different tissues and organs. Although renal damage prevails during initial stages, the deposition of cystine crystals in the cornea causes severe ocular manifestations. At present, cysteamine is the only topical effective treatment for ocular cystinosis. The lack of investment by the pharmaceutical industry, together with the limited stability of cysteamine, make it available only as two marketed presentations (Cystaran® and Cystadrops®) and as compounding formulations prepared in pharmacy departments. Even so, new drug delivery systems (DDSs) need to be developed, allowing more comfortable dosage schedules that favor patient adherence. In the last decades, different research groups have focused on the development of hydrogels, nanowafers and contact lenses, allowing a sustained cysteamine release. In parallel, different determination methods and strategies to increase the stability of the formulations have also been developed. This comprehensive review aims to compile all the challenges and advances related to new cysteamine DDSs, analytical determination methods, and possible future therapeutic alternatives for treating cystinosis.

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Cysteamine hydrochloride eye drop solution for the treatment of corneal cystine crystal deposits in patients with cystinosis: an evidence-based review

Cited by 24 publications

References 60 publications

Ocular manifestations of intermediate cystinosis: To treat or not to treat?

Ocular manifestations of intermediate cystinosis: To treat or not to treat?

Examination of corneal deposits in nephropathic cystinosis using in vivo confocal microscopy and anterior segment optical coherence tomography: an age-dependent cross sectional study

Recent Research in Ocular Cystinosis: Drug Delivery Systems, Cysteamine Detection Methods and Future Perspectives

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