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Application of a simple methodology to analyze Hydroxypropyl-β-Cyclodextrin in urine using HPLC-LS in early Niemann-Pick disease type C patient.

Application of a simple methodology to analyze Hydroxypropyl-β-Cyclodextrin in urine using HPLC-LS in early Niemann-Pick disease type C patient.

Authors :
Matencio A
Alcaráz-Gómez MA
García-Carmona F
Arias B
López-Nicolás JM
Source :
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences [J Chromatogr B Analyt Technol Biomed Life Sci] 2018 Sep 01; Vol. 1093-1094, pp. 47-51. Date of Electronic Publication: 2018 Jun 25.
Publication Year :
2018

Abstract

A new methodology, based on high resolution liquid chromatography with light scatterin detector, is applied to analyze Hydroxypropyl-beta-Cyclodextrin (HPβCD) in urine samples of a child affected by Niemann-Pick Type C disease. The treatment not only stopped disease progression, but has also increased the life expectancy and quality of our patient. The pharmacokinetic of HPβCD in the patient was studied with a 92.8% of HPβCD recovered. At 88 h, no HPβCD was found in the urine. During the treatment, HPβCD has not shown toxicity. Before application of the new treatment, injections were given every two weeks but, we have demonstrated that this can be increased to every four days.<br /> (Copyright © 2018 Elsevier B.V. All rights reserved.)

Details

Language :
English
ISSN :
1873-376X
Volume :
1093-1094
Database :
MEDLINE
Journal :
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences
Publication Type :
Academic Journal
Accession number :
29990712
Full Text :
https://doi.org/10.1016/j.jchromb.2018.06.051