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16 results on '"Eldere, J"'

10. Bile acids in peroxisomal disorders

Author

ELDERE, J. R. VAN, primary, PARMENTIER, G. G., additional, EYSSEN, H. J., additional, WANDERS, R. J., additional, SCHUTGENS, R. B., additional, VAMECQ, J., additional, HOOF, F. VAN, additional, POLL-THE, B.-T., additional, and SAUDUBRAY, J. M., additional

Published
1987
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13. Age-related differences in plasmalogen content of erythrocytes from patients with the cerebro-hepato-renal (Zellweger) syndrome: Implications for postnatal detection of the disease

Author

Wanders, R. J. A., primary, Purvis, Y. R., additional, Heymans, H. S. A., additional, Bakkeren, J. A. J. M., additional, Parmentier, G. G., additional, van Eldere, J., additional, Eyssen, H., additional, van den Bosch, H., additional, Tager, J. M., additional, and Schutgens, R. B. H., additional

Published
1986
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15. Galactomannan serves as a surrogate endpoint for outcome of pulmonary invasive aspergillosis in neutropenic hematology patients.

Author

Maertens J, Buvé K, Theunissen K, Meersseman W, Verbeken E, Verhoef G, Van Eldere J, and Lagrou K

Subjects
Adolescent, Adult, Aged, Aged, 80 and over, Aspergillosis mortality, Autopsy, Female, Galactose analogs & derivatives, Humans, Male, Middle Aged, Survival Analysis, Time Factors, Treatment Outcome, Aspergillosis blood, Biomarkers analysis, Mannans analysis, Neutropenia blood
Abstract

Background: A noninvasive, objective, reproducible, and quantitative Aspergillus-specific surrogate marker is needed for a more accurate assessment of the outcome of invasive aspergillosis (IA) in patients with a hematologic disorder. The quantitative serum galactomannan index (GMI) assay seems to fulfill the requirements of surrogacy for outcome evaluation., Methods: Kappa statistics were used to determine the strength of correlation between GMI outcome and clinical outcome (survival or death), autopsy data, and response outcome of IA in 70 adults with prolonged neutropenia. All patients underwent serial GMI monitoring until discharge or death., Results: The overall correlation between GMI and clinical outcome was good at 6 weeks (kappa=0.5882; 95% confidence interval [95% CI], 0.4023-0.7741) and was excellent at 12 weeks (kappa=0.8857; 95% CI, 0.7766-0.9948). Concordance with autopsy findings was perfect (kappa=1). At 6 weeks, the correlation between GMI and response outcome (favorable or unfavorable) was excellent (kappa=0.7523; 95% CI, 0.5803-0.9243). Survival was significantly better in patients who became GMI-negative (P<.0001)., Conclusions: In neutropenic patients with seropositive IA, serum galactomannan index outcome strongly correlates with survival, autopsy findings, and response outcome. This finding may have implications for patient management and for clinical trial design., (Copyright (c) 2009 American Cancer Society.)

Published
2009
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16. Bile acids in peroxisomal disorders.

Author

Van Eldere JR, Parmentier GG, Eyssen HJ, Wanders RJ, Schutgens RB, Vamecq J, Van Hoof F, Poll-The BT, and Saudubray JM

Subjects
Adrenoleukodystrophy metabolism, Chromatography, Gas, Humans, Refsum Disease metabolism, Bile Acids and Salts metabolism, Lipid Metabolism, Inborn Errors metabolism, Microbodies metabolism
Abstract

We examined serum bile acids in patients with different peroxisomal disorders. Patients with Zellweger syndrome (n = 23), infantile form of Refsum disease (n = 6) and neonatal adrenoleukodystrophy (n = 4) consistently had increased levels of bile acid precursors. Patients with X-linked adrenoleukodystrophy, (n = 5) classical Refsum disease (n = 3), hyperpipecolic acidaemia (n = 4) and rhizomelic chondrodysplasia punctata (n = 9) did not have increased bile acid precursor levels. Total serum bile acids (41 micrograms ml-1) and the percentage of bile acid precursors (80%) were highest in typical Zellweger patients who died young. Long-living Zellweger patients, neonatal adrenoleukodystrophy patients and infantile Refsum disease patients had, on average, less cholestasis and a lower percentage of bile acid precursors. We also observed that total serum bile acids and the percentage of bile acid precursors decreased with age in long-living Zellweger patients. Screening for bile acid precursors, combined with very long chain fatty acids analysis is, in our experience, an easy and reliable first-line approach to the detection of peroxisomal disorders.

Published
1987
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