Hyperhidrosis: a new and often early symptom in Fabry disease. International experience and data from the Fabry Outcome Survey.

Details

Serval ID
serval:BIB_48CB3CC374D3
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Hyperhidrosis: a new and often early symptom in Fabry disease. International experience and data from the Fabry Outcome Survey.
Journal
International Journal of Clinical Practice
Author(s)
Lidove O., Ramaswami U., Jaussaud R., Barbey F., Maisonobe T., Caillaud C., Beck M., Sunder-Plassmann G., Linhart A., Mehta A.
Working group(s)
FOS European investigators
ISSN
1368-5031 (Print)
ISSN-L
1368-5031
Publication state
Published
Issued date
2006
Peer-reviewed
Oui
Volume
60
Number
9
Pages
1053-1059
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Multicenter Study ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Abstract
Hypohidrosis is a classic feature of Fabry disease; in contrast, hyperhidrosis has only been rarely described. The aim of the study is to characterise the baseline descriptive data on hyperhidrosis (frequency, age at onset, sex ratio and outcome with and without enzyme replacement therapy) in hemizygous male and heterozygous female patients with Fabry disease. We describe case histories of five patients with Fabry disease and hyperhidrosis seen at three different centres. We have also analysed a cohort of 21 paediatric patients in the UK and a large European cohort of patients enrolled in the Fabry Outcome Survey (FOS). Five patients (three female, two male) with hyperhidrosis were originally identified, although each had additional symptoms related to Fabry disease. The age at onset of hyperhidrosis was less than 18 years in four cases. In the cohort of 21 paediatric patients (12 female, nine male), one female had hyperhidrosis; the age at onset of this symptom was 11 years. In the FOS cohort, 66 of 714 patients with Fabry disease had hyperhidrosis (44 of 369 females, 11.9%; 22 of 345 males, 6.4%). The female predominance was observed in seven of nine countries from which data were analysed. Hyperhidrosis is an increasingly recognised feature of the Fabry disease phenotype. It is more prevalent in females than in males and often appears in childhood or adolescence. The efficacy of enzyme replacement therapy on this recently recognised symptom should be assessed.
Keywords
Adolescent, Adult, Age of Onset, Child, Child, Preschool, Fabry Disease/complications, Female, Humans, Hyperhidrosis/etiology, Male, Middle Aged, Prognosis
Pubmed
Web of science
Create date
09/02/2012 16:36
Last modification date
20/08/2019 13:55
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