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Treatment of corneal cystine crystal accumulation in patients with cystinosis.

Shams F, Livingstone I, Oladiwura D, Ramaesh K - Clin Ophthalmol (2014)

Bottom Line: With increasing age, more severe ocular complications have been reported.Photophobia is a prominent symptom for patients.With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland.

ABSTRACT
Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephropathic cystinosis is the most common and severe phenotype. With the success of renal transplantation, these patients are now living longer and thus more long-term complications within different organs are becoming apparent. Ophthalmic manifestations range from corneal deposits of cystine crystals to pigmentary retinopathy. With increasing age, more severe ocular complications have been reported. Photophobia is a prominent symptom for patients. With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating. This review revisits the basic pathogenesis of cystinosis, the ocular manifestations of the disease, and the treatment of corneal crystals.

No MeSH data available.


Related in: MedlinePlus

Corneal Slit-lamp examination of three patients with nephropathic cystinosis.Note: (A) High magnification slit beam view of cornea with cystine crystals. (B) Retroillumination showing marked diffuse crystals packed within the cornea. (C) Retroillumination with patient looking down showing the extent of crystal deposition up to the peripheral edge of the cornea.
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f2-opth-8-2077: Corneal Slit-lamp examination of three patients with nephropathic cystinosis.Note: (A) High magnification slit beam view of cornea with cystine crystals. (B) Retroillumination showing marked diffuse crystals packed within the cornea. (C) Retroillumination with patient looking down showing the extent of crystal deposition up to the peripheral edge of the cornea.

Mentions: The crystals are typically obvious on slit-lamp biomicroscopy, appearing as needle-shaped, reflective corneal opacities. They tend to diffusely span the entire cornea. While it was initially thought that the endothelium was spared, case reports have since demonstrated crystals within the epithelium, stroma, and endothelium (Figures 2 and 3).32–35


Treatment of corneal cystine crystal accumulation in patients with cystinosis.

Shams F, Livingstone I, Oladiwura D, Ramaesh K - Clin Ophthalmol (2014)

Corneal Slit-lamp examination of three patients with nephropathic cystinosis.Note: (A) High magnification slit beam view of cornea with cystine crystals. (B) Retroillumination showing marked diffuse crystals packed within the cornea. (C) Retroillumination with patient looking down showing the extent of crystal deposition up to the peripheral edge of the cornea.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4199850&req=5

f2-opth-8-2077: Corneal Slit-lamp examination of three patients with nephropathic cystinosis.Note: (A) High magnification slit beam view of cornea with cystine crystals. (B) Retroillumination showing marked diffuse crystals packed within the cornea. (C) Retroillumination with patient looking down showing the extent of crystal deposition up to the peripheral edge of the cornea.
Mentions: The crystals are typically obvious on slit-lamp biomicroscopy, appearing as needle-shaped, reflective corneal opacities. They tend to diffusely span the entire cornea. While it was initially thought that the endothelium was spared, case reports have since demonstrated crystals within the epithelium, stroma, and endothelium (Figures 2 and 3).32–35

Bottom Line: With increasing age, more severe ocular complications have been reported.Photophobia is a prominent symptom for patients.With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland.

ABSTRACT
Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephropathic cystinosis is the most common and severe phenotype. With the success of renal transplantation, these patients are now living longer and thus more long-term complications within different organs are becoming apparent. Ophthalmic manifestations range from corneal deposits of cystine crystals to pigmentary retinopathy. With increasing age, more severe ocular complications have been reported. Photophobia is a prominent symptom for patients. With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating. This review revisits the basic pathogenesis of cystinosis, the ocular manifestations of the disease, and the treatment of corneal crystals.

No MeSH data available.


Related in: MedlinePlus