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Open Access Research article

Skin-impedance in Fabry Disease: A prospective, controlled, non-randomized clinical study

Surya N Gupta1, Markus Ries25, Gary J Murray2, Jane M Quirk2, Roscoe O Brady2, Jeffrey R Lidicker3, Raphael Schiffmann26* and David F Moore4

Author Affiliations

1 Division of Pediatric Neurology, Department of Pediatrics, Penn State University College of Medicine, 500 University Drive, Hershey, Pennsylvania 17033, USA

2 Developmental and Metabolic Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, USA

3 Temple University Center for Statistical and Information Science, Temple University School of Medicine, Philadelphia, Pennsylvania, USA

4 Section of Neurology, Department of Internal Medicine, University of Manitoba, Winnipeg, Manitoba, Canada

5 Shire Human Genetic Therapies, Cambridge, MA, USA

6 Institute of Metabolic Disease, Baylor Research Institute, 3812 Elm Street, Dallas, TX 75226, USA

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BMC Neurology 2008, 8:41  doi:10.1186/1471-2377-8-41

Published: 6 November 2008

Abstract

Background

We previously demonstrated improved sweating after enzyme replacement therapy (ERT) in Fabry disease using the thermo-regularity sweat and quantitative sudomotor axon reflex tests. Skin-impedance, a measure skin-moisture (sweating), has been used in the clinical evaluation of burns and pressure ulcers using the portable dynamic dermal impedance monitor (DDIM) system.

Methods

We compared skin impedance measurements in hemizygous patients with Fabry disease (22 post 3-years of bi-weekly ERT and 5 ERT naive) and 22 healthy controls. Force compensated skin-moisture values were used for statistical analysis. Outcome measures included 1) moisture reading of the 100th repetitive reading, 2) rate of change, 3) average of 60–110th reading and 4) overall average of all readings.

Results

All outcome measures showed a significant difference in skin-moisture between Fabry patients and control subjects (p < 0.0001). There was no difference between Fabry patients on ERT and patients naïve to ERT. Increased skin-impedance values for the four skin-impedance outcome measures were found in a small number of dermatome test-sites two days post-enzyme infusions.

Conclusion

The instrument portability, ease of its use, a relatively short time required for the assessment, and the fact that DDIM system was able to detect the difference in skin-moisture renders the instrument a useful clinical tool.