eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
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2/2014
vol. 52
 
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abstract:

Original article
Monitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil

Teresa J. Stradomska
,
Katarzyna Drabko
,
Elżbieta Moszczyńska
,
Anna Tylki-Szymańska

Folia Neuropathol 2014; 52 (2): 159-163
Online publish date: 2014/06/30
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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.
keywords:

adrenoleukodystrophy, haematopoietic stem cell transplantation, very long-chain fatty acids, VLCFA, chimaerism, Lorenzo’s Oil

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