Increased Serum Interleukin-6 and Tumor Necrosis Factor Alpha Levels in Fabry Disease: Correlation with Disease Burden
| Autor: | Nilton Salles Rosa Neto, Judith Campos de Barros Bento, Valéria de Falco Caparbo, Rosa Maria Rodrigues Pereira |
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| Rok vydání: | 2020 |
| Předmět: |
Male
Medicine (General) medicine.medical_specialty medicine.medical_treatment Inflammation Increased serum interleukin-6 Gastroenterology R5-920 Cost of Illness Internal medicine medicine Humans Interleukin 6 Cytokine Alpha-galactosidase biology business.industry Interleukin-6 Tumor Necrosis Factor-alpha Mainz Severity Score Index General Medicine Enzyme replacement therapy medicine.disease Fabry disease GLA Gene alpha-Galactosidase Cohort biology.protein Fabry Disease Original Article Female medicine.symptom business |
| Zdroj: | Clinics, Volume: 76, Article number: e2643, Published: 16 JUL 2021 Clinics, Vol 76 (2021) Clinics |
| ISSN: | 1980-5322 |
| Popis: | OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal disease caused by variants of the GLA gene; the formation of defective alpha-galactosidase A contributes to the accumulation of substrates in several organs. Chronic inflammation is thought to contribute to organ damage in FD patients. METHODS: In total, 36 classic FD patients (15 men/21 women) and 25 healthy controls (20 men/8 women) were assessed. The Mainz Severity Score Index (MSSI) was established after conducting interviews with the patients and chart review. Serum IL-6, IL-1β, and TNF-α levels were evaluated in both groups. RESULTS: The mean age (years) for FD patients was 43.1±15.4 and that for the controls was 47.4±12.2 (p>0.05). Twenty-two patients (59.5%) were treated with enzyme replacement therapy (ERT). Serum IL-6 and TNF-α levels were significantly higher in FD patients than in the controls. Patients treated with ERT had higher serum IL-6 and TNF-α levels than those not treated with ERT. There was no difference in the serum IL-1β levels between patients treated with ERT and those who were not. The MSSI scores in the patients were correlated with serum levels of IL-6 (r=0.60, p |
| Databáze: | OpenAIRE |
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