Pericardial effusion in the course of Fabry disease cardiomyopathy: a case report
| Autor: | Yoshimasa Higashi, Toshihiro Tsuruda, Toshihiro Gi, Shoichiro Nakao |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Inflammation
Fabry disease medicine.medical_specialty biology business.industry Cardiomyopathy Pericardial fluid Interleukin Hypertrophy medicine.disease Left ventricular hypertrophy Pericardial effusion Muscle hypertrophy Internal medicine Case report medicine Cardiology biology.protein AcademicSubjects/MED00200 Cardiology and Cardiovascular Medicine business Interleukin 6 Cytokine |
| Zdroj: | European Heart Journal: Case Reports |
| ISSN: | 2514-2119 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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