Abstract
Most children with Fabry's disease experience bouts of excruciating pain with a causalgic character. Many of them also present with a chronic state of discomfort, making them fear to engage in any physical activity. Most analgesics fail to offer significant relief. The mechanism of pain is not understood. Lipid storage involves dorsal root ganglia and the autonomous nervous system. Peripheral vascular investigations were undertaken in 3 males and 3 females with Fabry's disease. Significant abnormalities were observed, including stenosis at the level of the hands and feet, increased arteriolar resistance, decreased skin blood flow and markedly increased venous tone. Phenoxybenzamine (10-20 mg/day) gave prompt relief from the pain and discomfort, while peripheral blood flow appeared significantly improved. Diabetes, which can give rise to similar disturbances, can be excluded in our patients. Despite its promising action on pain in patients with Fabry's disease, phenoxybenzamine should be used with caution since one of our patients developed priapism shortly after a low dosage was started. It is advisable to try diphenylhydantoin initially (Lockman et al, Neurology: 21, 423, 1971) to relieve the pain. (Supported in part by PHS Grant HD-04612.)
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Philippart, M., Vyden, J. & Funderburk, S. ABNORMAL PERIPHERAL BLOOD FLOW IN FABRY'S DISEASE: ITS RELATIONSHIP TO PAIN. Pediatr Res 8, 393 (1974). https://doi.org/10.1203/00006450-197404000-00321
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DOI: https://doi.org/10.1203/00006450-197404000-00321