Merkler Marijan, Pećin Ivan, Simić Iveta, Muacević-Katanec Diana, Sućur Nediljko, Reiner Zeljko
Lijec Vjesn. 2014 May-Jun;136(5-6):133-5.
Early diagnosis and management of patients with Fabry disease (FD) requires a multidisciplinary approach of several different experts. The aim of this document is to provide health care professionals with guidelines for management of adult patients with Fabry disease. These guidelines were produced by the staff of the Division of Metabolic Diseases, Department of Internal Medicine, University Hospital Center Zagreb, which is the Referral Expert Center for Rare and Metabolic Diseases of the Ministry of Health, Republic of Croatia. The first guidelines ever published in Croatia concerning a rare metabolic disease are presented. This document provides a short summary on Fabry disease, how to diagnose Fabry disease, management of patients with this disease, follow-up of the patients, and gives recommendations on therapy and genetic testing.
法布里病(FD)患者的早期诊断和管理需要多位不同专家采取多学科方法。本文档的目的是为医疗保健专业人员提供成年法布里病患者的管理指南。这些指南由萨格勒布大学医院中心内科代谢疾病科的工作人员制定,该中心是克罗地亚共和国卫生部罕见和代谢疾病转诊专家中心。本文展示了克罗地亚有史以来首次发布的关于罕见代谢疾病的指南。本文档简要概述了法布里病、如何诊断法布里病、该病患者的管理、患者随访,并给出了治疗和基因检测方面的建议。