Murata R, Takatsu H, Noda T, Nishigaki K, Tsuchiya K, Takemura G, Kanoh M, Kunishima A, Sano K, Minatoguchi S, Takagi H, Fujiwara H
Second Department of Internal Medicine, Gifu University School of Medicine.
Intern Med. 1999 Jun;38(6):476-81. doi: 10.2169/internalmedicine.38.476.
A 47-year-old woman with heterozygous Fabry's disease with pre-excitation syndrome has been followed up for 15 years. Diagnosis was confirmed by the typical electron microscopic feature of the endomyocardial specimen and a decreased plasma alpha-galactosidase activity. As the disease progressed, the interventricular septum thickened from 11 to 17 mm as measured by echocardiography, while the AH interval was prolonged from 80 to 140 msec. In Fabry's disease, the PR interval has been reported to be variable from short PR to AV block. Therefore, this case may be helpful to understand the time course in the AV conduction abnormalities with the progression of Fabry's disease.
一名患有杂合性法布里病并伴有预激综合征的47岁女性已被随访15年。心内膜活检标本的典型电子显微镜特征以及血浆α-半乳糖苷酶活性降低证实了诊断。随着疾病进展，经超声心动图测量，室间隔厚度从11毫米增厚至17毫米，而AH间期从80毫秒延长至140毫秒。在法布里病中，据报道PR间期可从短PR间期到房室传导阻滞不等。因此，该病例可能有助于了解法布里病进展过程中房室传导异常的时间进程。
