@Article{Stradomska2014,
journal="Folia Neuropathologica",
issn="1641-4640",
volume="52",
number="2",
year="2014",
title="Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil",
abstract=" X-linked adrenoleukodystrophy is a rare, neurodegenerative peroxisomal disorder connected with mutation in the ABCD1 gene, causing impairment of the peroxisomal -oxidation process and in consequence, accumulation of very long-chain fatty acids (VLCFA) in blood and tissues. In this study we present serum very long-chain fatty acids levels during clinical course in an X-linked adrenoleukodystrophy patient after haematopoietic stem cell transplantation (HSCT) and on Lorenzo’s Oil in a 11 years’ period. The patient was diagnosed at the age of 8 months by family screening. The administration of LO was started at 2 years of age. HSCT from a family donor was performed twice. VLCFA serum levels were detected by the GC method. Chimaerism subsequent to HSCT was also analyzed.  Increasing very long-chain fatty acids levels correlate with a decreasing chimaerism level after haematopoietic stem cell transplantation. The sequential monitoring of very long-chain fatty acids serum levels is important and useful for assessment of engraftment, graft failure or rejection. ",
author="Stradomska, Teresa J.
and Drabko, Katarzyna
and Moszczyńska, Elżbieta
and Tylki-Szymańska, Anna",
pages="159--163",
doi="10.5114/fn.2014.43787",
url="http://dx.doi.org/10.5114/fn.2014.43787"
}