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Background <p>Ataxia–telangiectasia (A–T) is an inherited multiorgan disorder with onset in childhood. Liver involvement, with steatosis and subsequent fibrosis, is increasingly recognized in children and young people with A–T.</p> Purpose <p>To evaluate feasibility of T1-weighted two-point mDixon MRI for identification of liver steatosis in children with A–T and conduct exploratory analysis of relationships between MRI-quantified liver fat fraction and clinical and laboratory measures.</p> Study type <p>Post hoc analysis of prospectively collected research data.</p> Population <p>16 participants (8 female) with A–T aged 4.8–16.6&#xa0;years.</p> Field strength/sequence <p>3.0-T, two-point T1-weighted mDixon.</p> Assessment <p>Participants underwent whole-body MRI including T1-weighted mDixon. Water/fat signal percentage images were generated. Hepatic T1 fat fraction (T1-FF) was calculated from regions-of-interest placed in the right anterior, right posterior and left hepatic lobes. T1-FF &gt; 5.56% was used as the diagnostic criterion for hepatic steatosis.</p> Statistical tests <p>Group comparisons of variables between participants with and without previous diagnosis of liver steatosis were made using independent sample Mann–Whitney U. Associations between T1-FF and age, neurological severity and of liver function tests were tested with Spearman correlation. Statistical significance was pre-specified as p &lt; 0.05.</p> Results <p>Analyzable T1-weighted mDixon data was available for 11 participants. Five MRI datasets were discarded due to motion artefact (n = 3) or incorrect archiving of the original water image (n = 2). Median liver T1-FF was 11.3% (4.7–49.7%), and 10/11 (91%) of participants had evidence of steatosis. Participants with previous diagnosis of steatosis had higher T1-FF than those without (median 32.7% [9.7–49.7%], versus 10.3% [4.7–11.3%], <i>p</i> = 0.030). T1-FF correlated most strongly with alanine transaminase (r = 0.76, <i>p</i> = 0.007) and γ-glutamyltransferase (r = 0.76, <i>p</i> = 0.006).</p> Conclusion <p>T1-weighted mDixon MRI is feasible for detecting steatosis in children with A–T, although motion artefacts reduced data completeness. MRI-quantified liver T1-FF correlates with markers of liver health. We found higher prevalence of liver steatosis using T1-weighted mDIXON than previously reported in pediatric A–T cohorts.</p>

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Quantification of liver fat fraction using T1-weighted mDixon MRI in young patients with ataxia telangiectasia undergoing whole-body MRI: an exploratory study

  • 期刊论文

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  • 发表日期：

    2025年6月21日

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  • Soma Kumasaka,
  • Rafal Panek,
  • Renata Neves,
  • Mohnish Suri,
  • Sumit Jagani,
  • Yuka Kumasaka,
  • Yoshito Tsushima,
  • Robert A. Dineen

摘要

Background

Ataxia–telangiectasia (A–T) is an inherited multiorgan disorder with onset in childhood. Liver involvement, with steatosis and subsequent fibrosis, is increasingly recognized in children and young people with A–T.

Purpose

To evaluate feasibility of T1-weighted two-point mDixon MRI for identification of liver steatosis in children with A–T and conduct exploratory analysis of relationships between MRI-quantified liver fat fraction and clinical and laboratory measures.

Study type

Post hoc analysis of prospectively collected research data.

Population

16 participants (8 female) with A–T aged 4.8–16.6 years.

Field strength/sequence

3.0-T, two-point T1-weighted mDixon.

Assessment

Participants underwent whole-body MRI including T1-weighted mDixon. Water/fat signal percentage images were generated. Hepatic T1 fat fraction (T1-FF) was calculated from regions-of-interest placed in the right anterior, right posterior and left hepatic lobes. T1-FF > 5.56% was used as the diagnostic criterion for hepatic steatosis.

Statistical tests

Group comparisons of variables between participants with and without previous diagnosis of liver steatosis were made using independent sample Mann–Whitney U. Associations between T1-FF and age, neurological severity and of liver function tests were tested with Spearman correlation. Statistical significance was pre-specified as p < 0.05.

Results

Analyzable T1-weighted mDixon data was available for 11 participants. Five MRI datasets were discarded due to motion artefact (n = 3) or incorrect archiving of the original water image (n = 2). Median liver T1-FF was 11.3% (4.7–49.7%), and 10/11 (91%) of participants had evidence of steatosis. Participants with previous diagnosis of steatosis had higher T1-FF than those without (median 32.7% [9.7–49.7%], versus 10.3% [4.7–11.3%], p = 0.030). T1-FF correlated most strongly with alanine transaminase (r = 0.76, p = 0.007) and γ-glutamyltransferase (r = 0.76, p = 0.006).

Conclusion

T1-weighted mDixon MRI is feasible for detecting steatosis in children with A–T, although motion artefacts reduced data completeness. MRI-quantified liver T1-FF correlates with markers of liver health. We found higher prevalence of liver steatosis using T1-weighted mDIXON than previously reported in pediatric A–T cohorts.