Statistical Permutation Test Reveals Progressive and Region-Specific Iron Accumulation in the Thalami of Children with Aspartylglucosaminuria

Visa fullständig post



Permalänk

http://hdl.handle.net/10138/320238

Citation

Sairanen, V.; Tokola, A.; Tikkanen, R.; Laine, M.; Autti, T. Statistical Permutation Test Reveals Progressive and Region-Specific Iron Accumulation in the Thalami of Children with Aspartylglucosaminuria. Brain Sci. 2020, 10, 677.

Titel: Statistical Permutation Test Reveals Progressive and Region-Specific Iron Accumulation in the Thalami of Children with Aspartylglucosaminuria
Författare: Sairanen, Viljami; Tokola, Anna; Tikkanen, Ritva; Laine, Minna; Autti, Taina
Utgivare: Multidisciplinary Digital Publishing Institute
Datum: 2020-09-27
Permanenta länken (URI): http://hdl.handle.net/10138/320238
Abstrakt: Aspartylglucosaminuria (AGU) is a rare lysosomal storage disorder causing developmental delay, intellectual disability, and eventual death. A distinct feature in AGU is iron accumulation within the thalamus. Our aim is to demonstrate that susceptibility-weighted images (SWI) could be used as an MRI biomarker to evaluate the response within the AGU population to newly evolving treatments. SWI from 16 patients with AGU and 16 age-matched controls were used in the analysis. Thalamic volume with an iron accumulation was identified using a permutation test. Group differences were investigated for both the complete thalamus and the iron accumulation regions. Group-wise age correlation within these volumes were assessed with analysis of variance and multivariate regression. We found a statistically significant and large difference (<i>p</i>-value = 0.01, Cohen&rsquo;s D = 0.97) for the whole thalamus comparison and an even greater difference in the iron accumulation regions (<i>p</i>-value &lt; 0.01, Cohen&rsquo;s D = 3.52). Furthermore, we found strong evidence for iron accumulation as a linear function of age with R<sup>2</sup> = 0.65 only for AGU. The statistical analysis of SWI provides tools for assessing the degree of iron accumulation. This method could be used to study the response to treatments, in that a successful treatment would be expected to result in a decline in iron accumulation.


Filer under denna titel

Totalt antal nerladdningar: Laddar...

Filer Storlek Format Granska
brainsci-10-00677.pdf 1.602Mb PDF Granska/Öppna

Detta dokument registreras i samling:

Visa fullständig post