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The Role of Enzyme Replacement Therapy in Fabry Disease in Cardiology Perspective
Hongo, Kenichi Association for Research of MPS and Rare Diseases 2018 Journal of mucopolysaccharidosis and rare disease Vol.4 No.1
Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.
홍고(Hongo Teruo) 구결학회 2001 구결연구 Vol.7 No.-
This study aims to describe the honorific forms of the `Idu` observed in the Nongpojip in the Kyujangkak collection at Seoul University, which was written at the end of the 16th century and published in 1758 as the first edition. As to the grammatical subject honorific forms, `敎是`[isi] of `宋安廷乙催促赴赴任敎是去乃`[-isi keo na] was apparently interpreted as a causative use. `敎是`[isi] of `夫人敎是等乙`[isin der her] was interpreted as an honorific indication without the function of the inflection. So we can summarize the usage of the `敎是` as falling under the following 5 types; ① `-시-[-si-] (prefinal ending), ② `-ㅎㆍ시-[-hasi-](verb-derivational suffix + prefinal ending), ③ `-ㅎㆍ시-[-hasi-](verbal stem + prefinal ending), ④ `-ㅎㆍ이시-[-haisi-](verbal stem + causative suffix + prefinal ending), ⑤ the usage to be suffixed to honorific nouns. The `敎是` should be estimated to be read as [isi] for all usages except ① which reads as [si]. On the other hand, the usage of `白`[sab] as we have observed involves all three of the honorific forms(the subject honorific, the object honorific and the hearer honorific). More specifically, in 208 out of 279 total instances, the hearer honorific was used instead of the object honorific. This corresponds to other findings which have indicated that the decreased use of the object-honorific form represented by `ㅅㆍㅂ` can be observed in the Hangul written materials of the 17th century. So it might be possible to say that the `Idu` had been affected by the language of its time in view of the honorific forms.