Original article<br>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

doi:10.5114/fn.2014.43787

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

DOI: https://doi.org/10.5114/fn.2014.43787

Online publish date: 2014/06/30

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AMA

Stradomska T, Drabko K, Moszczyńska E, Tylki-Szymańska A. Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil. Folia Neuropathologica. 2014;52(2):159-163. doi:10.5114/fn.2014.43787.

APA

Stradomska, T., Drabko, K., Moszczyńska, E.,  & Tylki-Szymańska, A. (2014). Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil. Folia Neuropathologica, 52(2), 159-163. https://doi.org/10.5114/fn.2014.43787

Chicago

Stradomska, Teresa J., Katarzyna Drabko, Elżbieta Moszczyńska,  and Anna Tylki-Szymańska. 2014. "Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil". Folia Neuropathologica 52 (2): 159-163. doi:10.5114/fn.2014.43787.

Harvard

Stradomska, T., Drabko, K., Moszczyńska, E.,  and Tylki-Szymańska, A. (2014). Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil. Folia Neuropathologica, 52(2), pp.159-163. https://doi.org/10.5114/fn.2014.43787

MLA

Stradomska, Teresa J. et al. "Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil." Folia Neuropathologica, vol. 52, no. 2, 2014, pp. 159-163. doi:10.5114/fn.2014.43787.

Vancouver

Stradomska T, Drabko K, Moszczyńska E, Tylki-Szymańska A. Original articleMonitoring of very long-chain fatty acids levels in X-linked adrenoleukodystrophy, treated with haematopoietic stem cell transplantation and Lorenzo’s Oil. Folia Neuropathologica. 2014;52(2):159-163. doi:10.5114/fn.2014.43787.

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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:

Original articleMonitoring 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

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

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