Fabry disease is an X-linked disease due to a deficiency of the lysosomal enzyme alpha-galactosidase A. Clinical symptoms in classically affected males include acroparesthesia, anhydrosis and angiokeratoma, which may present during childhood followed by cardiac, cerebral and renal complications. Even though pulmonary involvement is not widely appreciated by clinicians, an obstructive lung disease is another recognized component of Fabry disease. Coronavirus Disease-19 (COVID-19), caused by the SARS-CoV-2 virus was labeled as a global pandemic and patients with Fabry disease can be considered at high risk of developing severe complications. The impact of COVID-19 on patients with Fabry disease receiving enzyme replacement therapy is still unknown. Many patients who receive treatment in the hospital experienced infusion disruptions due to fear of infection. Effects of temporary treatment interruption was described in more detail in other lysosomal storage diseases, but the recommencement of therapy does not fully reverse clinical decline due to the temporary discontinuation. When possible, home-therapy seems to be the most efficient way to maintain enzyme replacement therapy access during pandemic.

Sentence take-home message: Home-therapy, when possible, seems to be the most efficient way to maintain enzyme replacement therapy access during pandemic in patients with Fabry disease.

法布里疾病是由于缺乏溶酶体酶α-半乳糖苷酶A而引起的X连锁疾病。受典型影响的男性的临床症状包括肢体感觉异常，脱水和血管性角膜炎，可能在儿童时期出现，随后出现心，脑和肾并发症。尽管临床医生尚未广泛认识到肺部受累，但阻塞性肺部疾病是法布里氏病的另一种公认组成部分。由SARS-CoV-2病毒引起的冠状病毒病19（COVID-19）被标记为全球大流行，患有法布里病的患者可被视为有发生严重并发症的高风险。尚不清楚COVID-19对接受酶替代疗法的法布里病患者的影响。由于担心感染，许多在医院接受治疗的患者出现输液中断。在其他溶酶体贮积病中更详细地描述了暂时性治疗中断的影响，但是重新开始治疗并不能完全扭转由于暂时性中断而导致的临床下降。如果可能的话，家庭疗法似乎是在大流行期间维持酶替代疗法的最有效途径。

句子带回家的信息：在可能的情况下，家庭疗法似乎是在法布里病患者大流行期间维持酶替代疗法的最有效途径。