The switch of ERT from agalsidase beta to agalsidase alfa appears to be safe after 1 year of follow-up for Taiwanese patients with Fabry disease.

ObjectiveCurrent studies of newborn screening for Fabry disease in Taiwan have revealed a remarkably high prevalence of cardiac-type Fabry disease with a Chinese hotspot late-onset Fabry mutation (IVS4+919G>A).DesignRetrospective cohort study.SettingTertiary medical centre.Participants21 patients with cardiac-type Fabry disease (15 men and 6 women) as well as 15 patients with classic Fabry disease (4 men and 11 women) treated with biweekly intravenous infusions of agalsidase β (1 mg/kg) or agalsidase α (0.2 mg/kg) for at least 6 months.Outcome measuresThese data were collected at the time before enzyme replacement therapy (ERT) began and followed up after ERT for at least 6 months, including patient demographics, medical history, parameter changes of cardiac status and renal functions, plasma globotriaosylsphingosine (lyso-Gb3) and Mainz Severity Score Index.ResultsAfter 6–39 months of ERT, plasma lyso-Gb3 was found to be reduced in 89% (17/19) and 93% (14/15) of patients with cardiac-type and classic Fabry disease, respectively, which indicated an improvement of disease severity. For patients with cardiac-type Fabry disease, echocardiography revealed the reduction or stabilisation of left ventricular mass index (LVMI), the thicknesses of intraventricular septum (IVS) and left posterior wall (LPW) in 83% (15/18), 83% (15/18) and 67% (12/18) of patients, respectively, as well as 77% (10/13), 73% (11/15) and 60% (9/15) for those with classic type. Most patients showed stable renal function after ERT. There were statistically significant improvements (p<0.05) between the data at baseline and those after ERT for values of plasma lyso-Gb3, LVMI, IVS, LPW and Mainz Severity Score Index. No severe clinical events were reported during the treatment.ConclusionsERT is beneficial and appears to be safe for Taiwanese patients with cardiac-type Fabry disease, as well as for those with the classic type.

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On account of the complicated management, complex business, intricate data, complex appliance of the process of the oil exploration and development, the condition that the disunity, imperfect of the data standard of the oil exploration and development, and the ineffective share of the data, the plan of standardized data structure has been put forward in refer to the international standard of POSC and PPDM. The main aim of this plan is to solve the problem of the disunity of the structured standard between different major and different oil company. The plan will follow the principle of the business drive and begin from the production traffic flow, the analysis of the data traffic flow and through the standardization of the name, meaning, type, dereferencing of the data to form an open data dictionary for oil exploration and development. The dictionary will be public by some effective organization in time, which can increase the sharing and authority of the structured data.