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Potential role of vitamin D deficiency on Fabry cardiomyopathy

Please always quote using this URN: urn:nbn:de:bvb:20-opus-132102
  • Patients with Fabry disease frequently develop left ventricular (LV) hypertrophy and renal fibrosis. Due to heat intolerance and an inability to sweat, patients tend to avoid exposure to sunlight. We hypothesized that subsequent vitamin D deficiency may contribute to Fabry cardiomyopathy. This study investigated the vitamin D status and its association with LV mass and adverse clinical symptoms in patients with Fabry disease. 25-hydroxyvitamin D (25[OH]D) was measured in 111 patients who were genetically proven to have Fabry disease. LV massPatients with Fabry disease frequently develop left ventricular (LV) hypertrophy and renal fibrosis. Due to heat intolerance and an inability to sweat, patients tend to avoid exposure to sunlight. We hypothesized that subsequent vitamin D deficiency may contribute to Fabry cardiomyopathy. This study investigated the vitamin D status and its association with LV mass and adverse clinical symptoms in patients with Fabry disease. 25-hydroxyvitamin D (25[OH]D) was measured in 111 patients who were genetically proven to have Fabry disease. LV mass and cardiomyopathy were assessed by magnetic resonance imaging and echocardiography. In cross-sectional analyses, associations with adverse clinical outcomes were determined by linear and binary logistic regression analyses, respectively, and were adjusted for age, sex, BMI and season. Patients had a mean age of 40 ± 13 years (42 % males), and a mean 25(OH)D of 23.5 ± 11.4 ng/ml. Those with overt vitamin D deficiency (25[OH]D ≤ 15 ng/ml) had an adjusted six fold higher risk of cardiomyopathy, compared to those with sufficient 25(OH)D levels >30 ng/ml (p = 0.04). The mean LV mass was distinctively different with 170 ± 75 g in deficient, 154 ± 60 g in moderately deficient and 128 ± 58 g in vitamin D sufficient patients (p = 0.01). With increasing severity of vitamin D deficiency, the median levels of proteinuria increased, as well as the prevalences of depression, edema, cornea verticillata and the need for medical pain therapy. In conclusion, vitamin D deficiency was strongly associated with cardiomyopathy and adverse clinical symptoms in patients with Fabry disease. Whether vitamin D supplementation improves complications of Fabry disease, requires a randomized controlled trial.show moreshow less

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Metadaten
Author: Christiane Drechsler, Benjamin Schmiedeke, Markus Niemann, Daniel Schmiedeke, Johannes Krämer, Irina Turkin, Katja Blouin, Andrea Emmert, Stefan Pilz, Barbara Obermayer-Pietsch, Frank Wiedemann, Frank Breunig, Christoph Wanner
URN:urn:nbn:de:bvb:20-opus-132102
Document Type:Journal article
Faculties:Medizinische Fakultät / Medizinische Klinik und Poliklinik I
Medizinische Fakultät / Institut für Klinische Epidemiologie und Biometrie
Language:English
Parent Title (English):Journal of Inherited Metabolic Disease
Year of Completion:2013
Volume:37
Issue:2
Pagenumber:289-295
Source:Journal of Inherited Metabolic Disease (2014) 37:289–295 DOI 10.1007/s10545-013-9653-8
DOI:https://doi.org/10.1007/s10545-013-9653-8
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 616 Krankheiten
Tag:Fabry nephropathy; Fabry patient; LV mass; cardiac hypertrophy; cornea verticillata; diabetic mouse; enzyme replacement therapy; hypertrophic cardiomyopathy; left ventricular mass; renal fibrosis; septal hypertrophy; urinary protein excretion
Release Date:2016/06/11
Licence (German):License LogoCC BY: Creative-Commons-Lizenz: Namensnennung