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A Case-Control Study of the Lymphatic Phenotype of Yellow Nail Syndrome.

Cousins, E; Cintolesi, V; Vass, L; Stanton, AWB; Irwin, A; Heenan, SD; Mortimer, PS (2018) A Case-Control Study of the Lymphatic Phenotype of Yellow Nail Syndrome. Lymphatic Research and Biolog, 16 (4). pp. 340-346. ISSN 1557-8585 https://doi.org/10.1089/lrb.2018.0009
SGUL Authors: Mortimer, Peter Sydney

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Abstract

BACKGROUND: Yellow nail syndrome (YNS) is a rare disease manifesting as a triad of yellow-green dystrophic nails, lymphedema, and chronic respiratory disease. The etiology of YNS is obscure and investigations are few. A single lymphatic pathogenesis has been proposed to account for all the associated features, and despite the lack of evidence for a unifying lymphatic mechanism, this hypothesis prevails. The objective was to explore the lymphatic phenotype in YNS and to establish whether lymphatic dysfunction could be a major contributing factor to the disease process. METHODS AND RESULTS: Four-limb lymphoscintigraphy was performed on patients with YNS and on healthy, age-matched controls. All 17 patients had lower limb swelling, and 14 (82%) had upper limb swelling also, including 5 (29%) with hand involvement. None of the YNS lymph scans was completely normal. Combined qualitative and quantitative assessment showed that 67% of YNS scans were clearly abnormal compared with 36% of healthy control scans. Mean axillary and ilio-inguinal nodal tracer uptakes were 41%-44% lower in the YNS group than in the controls (p < 0.0001). CONCLUSIONS: YNS is a lymphatic phenotype because lymphatic insufficiency was found to exist in all patients and the insufficiency was widespread (upper and lower limbs), with a common mechanistic fault of poor transport. The origin of the lymphatic fault is unclear. In healthy individuals, lymphatic abnormalities may be relatively common in the fifth decade of life onward.

Item Type: Article
Additional Information: Copyright 2018, Mary Ann Liebert, Inc. Final publication is available from Mary Ann Liebert, Inc., publishers http://doi.org/10.1089/lrb.2018.0009
Keywords: lymphatic dysfunction, lymphedema, lymphoscintigraphy, phenotype, yellow nail syndrome, lymphatic dysfunction, lymphedema, lymphoscintigraphy, phenotype, yellow nail syndrome, 1107 Immunology, Oncology & Carcinogenesis
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Academic Structure > Molecular and Clinical Sciences Research Institute (MCS) > Cell Sciences (INCCCS)
Journal or Publication Title: Lymphatic Research and Biolog
ISSN: 1557-8585
Language: eng
Dates:
DateEvent
1 August 2018Published
16 April 2018Accepted
Publisher License: Publisher's own licence
PubMed ID: 30130162
Web of Science ID: WOS:000442259800003
Go to PubMed abstract
URI: https://openaccess.sgul.ac.uk/id/eprint/110305
Publisher's version: https://doi.org/10.1089/lrb.2018.0009

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