Abstract
Enhanced Intrinsic Skin Aging in Nephropathic Cystinosis Assessed by High-Definition Optical Coherence Tomography
Highlights
TO THE EDITOR Cystinosis is a rare autosomal recessive lysosomal storage disorder, caused by the mutations in the CTNS gene, which leads to the dysfunction of the cystine transporter cystinosin
In two adult kidney transplantation (KTx) patients with cystinosis, reflectance confocal microscopy and High-definition optical coherence tomography (HD-OCT) imaging were performed at the same location of two punch skin biopsies, at the distal third of the ventral side of the right upper arm
Upon validation of our methodology of HD-OCT image analysis using the OCT signal versus image depth plot adapted from Boone et al (2016a, 2016b), a significant reduction in epidermal (P 1⁄4 0.0074) and papillary dermis (P 1⁄4 0.0062) thickness was seen in patients of all ages who were diagnosed with cystinosis compared with their age- and sex-matched controls
Summary
TO THE EDITOR Cystinosis is a rare autosomal recessive lysosomal storage disorder, caused by the mutations in the CTNS gene, which leads to the dysfunction of the cystine transporter cystinosin. In two adult KTx patients with cystinosis, reflectance confocal microscopy and HD-OCT imaging were performed at the same location of two punch skin biopsies, at the distal third of the ventral side of the right upper arm (see Supplementary Figure S2 online).
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