Department of Anesthesiology and Pain Medicine, College of Medicine, Yeungnam University, Daegu, Korea.
Korean J Pain. 2010 Sep;23(3):207-10. doi: 10.3344/kjp.2010.23.3.207. Epub 2010 Aug 26.
Fabry disease is an X-linked lysosomal disease caused by deficiency of α-galactosidase, in which early diagnosis may be missed due to the wide variety of clinical symptoms presenting during disease progression. A 13 year-old boy visited our pain clinic complaining of pricking and burning pain in the toe tips of both feet. Continuous epidural infusion for pain management was performed because of oral analgesics ineffectiveness. The patient underwent α-galactosidase A (GLA) enzyme analysis based on the clinical impression of Fabry disease from pain with a peripheral neuropathic component and history of anhidrosis. He was diagnosed with Fabry disease after confirming mutation of the GLA gene through a screening test of GLA activity. Enzyme replacement therapy was initiated and pain was tolerated with oral analgesics.
法布瑞氏病是一种 X 连锁溶酶体贮积病,由α-半乳糖苷酶缺乏引起,由于疾病进展过程中出现的各种临床表现,早期诊断可能会被遗漏。一名 13 岁男孩因双脚脚趾刺痛和烧灼感到我院疼痛科就诊。由于口服止痛药无效,我们为他进行了持续硬膜外输注以进行疼痛管理。根据疼痛伴有周围神经病变成分和无汗症病史的法布瑞氏病的临床印象,我们对该患者进行了α-半乳糖苷酶 A(GLA)酶分析。通过 GLA 活性筛查试验确认 GLA 基因突变后,该患者被确诊为法布瑞氏病。随后开始进行酶替代疗法,同时口服止痛药缓解疼痛。