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SHR Neuro Krebs Kardio Lipid

Gaggl, M; Hofer, M; Weidner, S; Kleinert, J; Fauler, G; Wallner, M; Kotanko, P; Paschke, E; Sunder-Plassmann, G.
Interfering parameters in the determination of urinary globotriaosylceramide (Gb3) in patients with chronic kidney disease.
J Nephrol. 2015; 28(6):679-689
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Autor/innen der Med Uni Graz:
Fauler Günter
Paschke Eduard
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Abstract:
Globotriaosylceramide (Gb3, CD77) represents a pivotal part of the cell membrane. Measuring the urinary Gb3 content can be used to screen patients with chronic kidney disease (CKD) for Fabry disease, a disorder caused by hampered Gb3 degradation. However, little is known about factors influencing urinary Gb3 excretion other than Fabry disease. The aim of the present study was to identify routine diagnostic parameters as predictors of urinary Gb3 excretion in patients with CKD. Our study included 609 subjects with CKD stage I-V. We analyzed the influence of age, gender, renal function, urinary cell content and chemical characteristics on urinary Gb3 concentrations (total Gb3, Gb3-24 isoform, and Gb3-24:18 isoform ratio), determined by direct electrospray ionization mass spectrometry. In 609 subjects the median total urinary Gb3 was 233 ng/mg and the Gb3-24:18 isoform ratio was 1.2. Twenty-one patients, none of whom had Fabry disease, had a Gb3-24:18 isoform ratio ≥2.3. Females excreted a higher total amount of Gb3, but the Gb3-24:18 isoform ratio was comparable to males. Renal function and age had no influence on total Gb3, Gb3 isoforms or the ratio. Only a distinct load of bacteria and leukocytes was associated with an increased Gb3 excretion. Urinary leukocytes, erythrocytes, bacteria, or protein content did not affect the Gb3-24:18 isoform ratio. The Gb3-24:18 isoform ratio is unaffected by several potential influencing variables and may thus be applied for screening for Fabry disease in unselected cohorts of patients presenting with CKD.
Find related publications in this database (using NLM MeSH Indexing)
Adult -
Age Factors -
Aged -
Bacterial Load -
Biomarkers - urine
Creatinine - blood
Creatinine - urine
Fabry Disease - diagnosis
Fabry Disease - urine
Female -
Hematuria - urine
Humans -
Leukocytes -
Male -
Middle Aged -
Nail-Patella Syndrome -
Nephritis, Hereditary -
Protein Isoforms - urine
Renal Insufficiency, Chronic - physiopathology
Renal Insufficiency, Chronic - urine
Sex Factors -
Trihexosylceramides - urine
Urine - cytology
Urine - microbiology

Find related publications in this database (Keywords)
Chronic kidney disease
Fabry disease
Globotriaosylceramide
Lysosomal storage disorder
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