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Liver Histology01:27

Liver Histology

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The microscopic anatomy of the liver is a complex and intricate system that comprises numerous structural units known as liver lobules, each of which is comparable in size to a sesame seed. These hexagonal structures consist of plates of liver cells or hepatocytes, which are characterized by their versatility and abundance of cellular apparatus like rough and smooth ER, Golgi apparatus, peroxisomes, and mitochondria.
Hepatocytes perform a variety of essential functions. They secrete...
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Brain morphometry in hepatic Wilson disease patients.

Parya Rahimi1, Stanislav Mareček1, Radan Brůha2

  • 1Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czechia.

Journal of Inherited Metabolic Disease
|November 19, 2024
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Wilson disease (WD) causes brain changes even in early stages. Mild hepatic WD patients show reduced white matter volume, suggesting it

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MRIWilson diseasebrain atrophybrainstemhepaticmorphometrywhite matter

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Area of Science:

  • Neuroimaging
  • Neurology
  • Radiology

Background:

  • Wilson disease (WD) is a genetic disorder characterized by excessive copper accumulation.
  • Hepatic symptoms often precede neurological manifestations in WD.
  • Subclinical brain abnormalities, including T2 hyperintensities and atrophy, occur in WD patients with hepatic symptoms.

Purpose of the Study:

  • To investigate brain morphological changes in patients with mild hepatic Wilson disease.
  • To assess subclinical brain alterations using advanced MRI techniques.

Main Methods:

  • Brain MRI at 3T, including high-resolution T1-weighted images, was performed on 26 WD patients and 28 controls.
  • Volumetric analysis of specific brain regions (e.g., pons, midbrain, white matter) was conducted using Freesurfer v7.
  • Whole-brain analyses (voxel- and surface-based morphometry) were performed using SPM12, with statistical comparisons adjusted for covariates.

Main Results:

  • Volumetric analysis revealed significantly smaller pons volume in WD patients compared to controls.
  • A trend for smaller midbrain and cerebellar white matter (WM) volumes was observed in WD patients.
  • Whole-brain analysis identified reduced volumes in the pons, cerebellar, and lobar WM in the WD group; no significant gray matter differences were found.

Conclusions:

  • White matter (WM) appears vulnerable to copper toxicity in early Wilson disease.
  • Reduced WM volume may serve as a potential marker for assessing early brain involvement in Wilson disease.
  • These findings highlight the importance of neuroimaging in detecting subclinical brain changes in Wilson disease.