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Cysteamine eye drops deliver limited efficacy in nephropathic cystinosis

10 Sep 2017

The use of topical 0.55% cysteamine eye drops appears to have limited effects in reducing the corneal cystine deposits in patients with severe forms of nephropathic cystinosis, according to a recent study.

A total of 32 patients (mean age 8 years; range, 8 months to 19 years; 19 female) with nephropathic cystinosis were prospectively included in the study, with a mean follow-up period of 4.1 years (range, 2 to 8 years).

There was no clinically significant improvement of photophobia among symptomatic patients. During the follow-up period, participants demonstrated statistically significant worsening of corneal cystine deposits.

In this study that investigated the efficacy of topical cysteamine 0.55% eye drops in the treatment of corneal cystine crystal deposits in patients with nephropathic cystinosis, patients were administered the said medication. They were examined prior to treatment, on each monthly visit and after treatment at the last follow-up.

The main outcome was evaluation of photophobia and resolution of corneal cystine crystals.

Photophobia was classified as grade 0 (none) for no photophobia, grade 1 (mild) for photophobia in bright light, grade 2 (moderate) for photophobia in room light and grade 3 (severe) for photophobia in dim light. Corneal cystine crystals were classified as grade 0 for none, grade 1 for 1 to 10 crystals/mm2, grade 2 for 11 to 50 crystals/mm2, grade 3 for more than 50 crystals/mm2.

Nephropathic cystinosis is a rare genetic disease characterized by defective lysosomal cystine transport and increased lysosomal cystine. [Saudi J Kidney Dis Transpl 2009;20:436-42]

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Most Read Articles
Elvira Manzano, 4 days ago
Bisphosphonates have proven antifracture efficacy and remain to be the cornerstone of osteoporosis treatment. However, a drug holiday is of particular importance with bisphosphonates due to some signals with long-term use of the drug, including rare incidence of atypical femoral fracture (AFF) and osteonecrosis of the jaw (ONJ), says a leading endocrinologist at AFOS 2017.
18 hours ago
Breast cancer patients have notably different microbiomes in the local breast tissue and urinary tract, a recent study reveals. Particularly, species in the Methylobacterium genus are reduced in the local breast tissue while the urinary tract is enriched in gram-positive bacteria.
Pearl Toh, 13 Oct 2017
Women with higher plasma tryptophan concentrations were less likely to have poor sleep quality during pregnancy, especially among those with anxiety symptoms, according to the GUSTO* study.
Tristan Manalac, 20 hours ago
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