Article ; Online: Pericardial effusion in the course of Fabry disease cardiomyopathy: a case report.
European heart journal. Case reports
2021 Volume 5, Issue 10, Page(s) ytab407
Abstract: Background: Fabry disease (FD) is an X-chromosome-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal α-galactosidase A activity.: Case summary: A 51-year-old Japanese woman with a previous diagnosis of FD ... ...
Abstract | Background: Fabry disease (FD) is an X-chromosome-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal α-galactosidase A activity. Case summary: A 51-year-old Japanese woman with a previous diagnosis of FD presented with pericardial effusion. The exudative pericardial fluid contained globotriaosylsphingosine. Left ventricular hypertrophy progressed despite regular administration of agalsidase alfa every 2 weeks over a 7-year period, with increases in plasma levels of globotriaosylsphingosine and interleukin (IL)-18. In addition, the IL-6 level in the pericardial fluid was markedly higher than that in plasma. Discussion: This case suggests that elevated IL-6 and IL-18 levels in pericardial fluid and plasma indicate the severity of FD cardiomyopathy. |
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Language | English |
Publishing date | 2021-10-07 |
Publishing country | England |
Document type | Case Reports |
ISSN | 2514-2119 |
ISSN (online) | 2514-2119 |
DOI | 10.1093/ehjcr/ytab407 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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