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| http://dx.doi.org/10.1038/s41393-020-0504-9 | DOI Listing |
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Impact of enzyme replacement therapy on clinical manifestations in females with Fabry disease.
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Department of Internal Medicine D, and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Muenster, Germany.
Background: The aim of our multicenter study was to investigate the implementation of the European Fabry guidelines on therapeutic recommendations in female patients with Fabry disease (FD) and to analyze the impact of enzyme replacement therapy (ERT) in treated and untreated females.
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Until the beginning of the century, bleeding management was similar in elective surgeries or exsanguination scenarios: clotting tests were used to guide blood product orders and, while awaiting these results, an aggressive resuscitation with crystalloids was recommended. The high mortality rate in severe hemorrhages managed with this strategy endorsed the need for a special resuscitation plan. As a result, modifications were recommended to develop a new clinical approach to these patients, called "Damage Control Resuscitation".
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