التفاصيل البيبلوغرافية
| العنوان: |
A Plasma Pyrophosphate Cutoff Value for Diagnosing Pseudoxanthoma Elasticum. |
| المؤلفون: |
Rubera, Isabelle, Clotaire, Laetitia, Laurain, Audrey, Destere, Alexandre, Martin, Ludovic, Duranton, Christophe, Leftheriotis, Georges |
| المصدر: |
International Journal of Molecular Sciences; Jun2024, Vol. 25 Issue 12, p6502, 8p |
| مصطلحات موضوعية: |
REFERENCE values, PERIPHERAL vascular diseases, PYROPHOSPHATES, DIAGNOSIS, ION exchange chromatography, MEDICAL records |
| مصطلحات جغرافية: |
NICE (France) |
| مستخلص: |
Pseudoxanthoma elasticum (PXE) is a rare inherited systemic disease responsible for a juvenile peripheral arterial calcification disease. The clinical diagnosis of PXE is only based on a complex multi-organ phenotypic score and/or genetical analysis. Reduced plasma inorganic pyrophosphate concentration [PPi]p has been linked to PXE. In this study, we used a novel and accurate method to measure [PPi]p in one of the largest cohorts of PXE patients, and we reported the valuable contribution of a cutoff value to PXE diagnosis. Plasma samples and clinical records from two French reference centers for PXE (PXE Consultation Center, Angers, and FAVA-MULTI South Competent Center, Nice) were assessed. Plasma PPi were measured in 153 PXE and 46 non-PXE patients. The PPi concentrations in the plasma samples were determined by a new method combining enzymatic and ion chromatography approaches. The best match between the sensitivity and specificity (Youden index) for diagnosing PXE was determined by ROC analysis. [PPi]p were lower in PXE patients (0.92 ± 0.30 µmol/L) than in non-PXE patients (1.61 ± 0.33 µmol/L, p < 0.0001), corresponding to a mean reduction of 43 ± 19% (SD). The PPi cutoff value for diagnosing PXE in all patients was 1.2 µmol/L, with a sensitivity of 83.3% and a specificity of 91.1% (AUC = 0.93), without sex differences. In patients aged <50 years (i.e., the age period for PXE diagnosis), the cutoff PPi was 1.2 µmol/L (sensitivity, specificity, and AUC of 93%, 96%, and 0.97, respectively). The [PPi]p shows high accuracy for diagnosing PXE; thus, quantifying plasma PPi represents the first blood assay for diagnosing PXE. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |
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