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20.04.2024 | Hepatobiliary

Multiparametric magnetic resonance imaging of the liver and spleen in Gaucher disease

verfasst von: Daniella Braz Parente, Fernanda Chagas Monteiro de Melo Malta, Renata de Souza Cravo, Ronir Raggio Luiz, Vivian Rotman, Renata Mello Perez, Rosana Souza Rodrigues

Erschienen in: Abdominal Radiology | Ausgabe 9/2024

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Abstract

Purpose

To assess liver and spleen characteristics of a population with Gaucher disease (GD) using multiparametric MRI and MR elastography (MRE) for evaluation of diffuse liver and spleen disease, which includes liver fat fraction, liver and spleen volume and iron deposition, and liver and spleen stiffness correlated with DS3 Severity Scoring System for Gaucher disease (GD-DS3).

Methods

We prospectively evaluated 41 patients with type 1 Gaucher disease using a 3.0 T MRI and MRE between January 2019 and February 2020. Clinical, laboratory, and imaging data was collected. Mann–Whitney, Kruskal–Wallis, and Spearman’s correlation were applied to evaluate liver and spleen MRI and MRE, clinical and laboratory variables, and GD-DS3. ERT and SRT treatment groups were compared.

Results

Hepatomegaly was seen in 15% and splenomegaly in 42% of the population. Moderate and strong and correlations were found between liver and spleen iron overload (rho = 0.537; p = 0.002); between liver and spleen volume (rho = 0.692, p < 0.001) and between liver and spleen stiffness (rho = 0.453, p = 0.006). Moderate correlations were found between liver stiffness and GD-DS3 (rho = 0.559; p < 0.001) and between splenic volume and GD-DS3 (rho = 0.524; p = 0.001).

Conclusion

The prevalence of hepatosplenomegaly, liver fibrosis, and liver iron overload in treated patients with GD is low, which may be related to the beneficial effect of treatment. Liver MRE and splenic volume correlate with severity score and may be biomarkers of disease severity.

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Metadaten
Titel
Multiparametric magnetic resonance imaging of the liver and spleen in Gaucher disease
verfasst von
Daniella Braz Parente
Fernanda Chagas Monteiro de Melo Malta
Renata de Souza Cravo
Ronir Raggio Luiz
Vivian Rotman
Renata Mello Perez
Rosana Souza Rodrigues
Publikationsdatum
20.04.2024
Verlag
Springer US
Erschienen in
Abdominal Radiology / Ausgabe 9/2024
Print ISSN: 2366-004X
Elektronische ISSN: 2366-0058
DOI
https://doi.org/10.1007/s00261-024-04293-w

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