Your browser doesn't support javascript.
Show: 20 | 50 | 100
Results 1 - 1 de 1
Filter
1.
Mol Genet Metab ; 130(4): 227-229, 2020 08.
Article in English | MEDLINE | ID: covidwho-548553

ABSTRACT

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.


Subject(s)
Betacoronavirus/pathogenicity , Continuity of Patient Care/standards , Coronavirus Infections/prevention & control , Enzyme Replacement Therapy/standards , Fabry Disease/therapy , Home Infusion Therapy/standards , Lung Diseases, Obstructive/prevention & control , Pandemics/prevention & control , Pneumonia, Viral/prevention & control , Adult , COVID-19 , Continuity of Patient Care/organization & administration , Continuity of Patient Care/statistics & numerical data , Coronavirus Infections/complications , Coronavirus Infections/transmission , Coronavirus Infections/virology , Enzyme Replacement Therapy/statistics & numerical data , Fabry Disease/complications , Fabry Disease/diagnosis , Female , Home Infusion Therapy/statistics & numerical data , Humans , Infection Control/standards , Infusions, Intravenous , Isoenzymes/administration & dosage , Lung Diseases, Obstructive/diagnosis , Lung Diseases, Obstructive/etiology , Male , Middle Aged , Pneumonia, Viral/complications , Pneumonia, Viral/transmission , Pneumonia, Viral/virology , Recombinant Proteins/administration & dosage , SARS-CoV-2 , Severity of Illness Index , Time Factors , alpha-Galactosidase/administration & dosage
SELECTION OF CITATIONS
SEARCH DETAIL