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Pain in Fabry Disease: Practical Recommendations for Diagnosis and Treatment.
- Source :
-
CNS neuroscience & therapeutics [CNS Neurosci Ther] 2016 Jul; Vol. 22 (7), pp. 568-76. Date of Electronic Publication: 2016 Mar 28. - Publication Year :
- 2016
-
Abstract
- Aims: Patients with Fabry disease (FD) characteristically develop peripheral neuropathy at an early age, with pain being a crucial symptom of underlying pathology. However, the diagnosis of pain is challenging due to the heterogeneous and nonspecific symptoms. Practical guidance on the diagnosis and management of pain in FD is needed.<br />Methods: In 2014, experts met to discuss recent advances on this topic and update clinical guidance.<br />Results: Emerging disease-specific tools, including FabryScan, Fabry-specific Pediatric Health and Pain Questionnaire, and Würzburg Fabry Pain Questionnaire, and more general tools like the Total Symptom Score can aid diagnosis, characterization, and monitoring of pain in patients with FD. These tools can be complemented by more objective and quantifiable sensory testing. In male and female patients of any age, pain related to FD can be an early indication to start disease-specific enzyme replacement therapy before potentially irreversible organ damage to the kidneys, heart, or brain occurs.<br />Conclusion: To improve treatment outcomes, pain should be diagnosed early in unrecognized or newly identified FD patients. Treatment should include: (a) enzyme replacement therapy controlling the progression of underlying pathology; (b) adjunctive, symptomatic pain management with analgesics for chronic neuropathic and acute nociceptive, and inflammatory or mixed pain; and (c) lifestyle modifications.<br /> (© 2016 The Authors. CNS Neuroscience & Therapeutics published by John Wiley & Sons Ltd.)
- Subjects :
- Analgesics, Non-Narcotic therapeutic use
Anti-Inflammatory Agents, Non-Steroidal therapeutic use
Diagnosis, Differential
Enzyme Replacement Therapy
Fabry Disease pathology
Fabry Disease therapy
Female
Ganglia, Spinal pathology
Humans
Life Style
Male
Pain Measurement
Surveys and Questionnaires
Fabry Disease complications
Pain diagnosis
Pain etiology
Pain Management methods
Subjects
Details
- Language :
- English
- ISSN :
- 1755-5949
- Volume :
- 22
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- CNS neuroscience & therapeutics
- Publication Type :
- Academic Journal
- Accession number :
- 27297686
- Full Text :
- https://doi.org/10.1111/cns.12542