Spatial and temporal sequence of corneal crystal deposition in nephropathic cystinosis.
We studied 15 patients with infantile nephropathic cystinosis. We found that anterior corneal cystine crystal deposition began early in life and proceeded posteriorly as the patient aged; deposition advanced more rapidly in the periphery. Ultrastructural analysis of a corneal button obtained from a 20-year-old patient undergoing corneal transplantation confirmed our clinical observations that crystals were deposited throughout the entire central stroma.
Department of Ophthalmology, University of California, San Diego, La Jolla 92039.
R B
RB
Melles
J A
JA
Schneider
N A
NA
Rao
B
B
Katz
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003315:Cornea / Q000378:metabolism* / Q000473:pathology; D003319:Corneal Stroma / Q000378:metabolism / Q000473:pathology; D003460:Crystallization; D003553:Cystine / Q000378:metabolism*; D003554:Cystinosis / Q000378:metabolism* / Q000473:pathology; D004728:Endothelium, Corneal / Q000378:metabolism; D006801:Humans; D007674:Kidney Diseases / Q000378:metabolism* / Q000473:pathology; D013997:Time Factors
D003553:Cystine