Multiorgan quantitative abdominal MRI in paediatric patients with Cystic Fibrosis
Motivation: Cystic fibrosis (CF) is a multi-system, life-limiting genetic disorder. There is a need for multiorgan assessment in children with CF and the response to modulator therapies. Goal(s): To perform multiorgan MRI in children to quantify lung, liver, pancreas, spleen, and gut function. Approach: 8 children with CF and 3 healthy volunteers (HV) performed the protocol including quantitative assessment (T1, T2, T2*, fat fraction (FF), stiffness) of liver, pancreas and spleen. Results: Increased pancreas FF, T1 and T2, spleen T1 and hepatic arterial velocity in CF compared to HVs. Increased liver T1, T2 and stiffness in a CF child independently diagnosed with liver disease. Impact: This protocol will be used to study the effect of the Cystic Fibrosis Transmembrane Regulator Modulator therapy in children on liver toxicity, pancreas and gut function, and lung health, by performing MRI before and 12 months after commencement of Kaftrio.
Authors
- Nupur B. Dey (ORCID: https://orcid.org/0000-0002-5504-2115)
- Andrew Prayle (ORCID: https://orcid.org/0000-0002-1514-2840)
- A Yule
- Luca Marciani (ORCID: https://orcid.org/0000-0001-9092-4300)
- Neele Dellschaft (ORCID: https://orcid.org/0000-0001-5483-1684)
- Robert E. Spiller (ORCID: https://orcid.org/0000-0001-6371-4500)
- Susan Francis (ORCID: https://orcid.org/0000-0003-0903-7507)
- Zachary Peggs (ORCID: https://orcid.org/0000-0003-4263-6798)
- Ian P. Hall (ORCID: https://orcid.org/0000-0001-9933-3216)
- Jonathan Brooke
- Caroline L. Hoad (ORCID: https://orcid.org/0000-0001-5483-1027)
- Naaventhan Palaniyappan (ORCID: https://orcid.org/0000-0001-7698-8869)
- Alan R Smyth (ORCID: https://orcid.org/0000-0001-5494-5438)
- Guruprasad Padur Aithal (ORCID: https://orcid.org/0000-0003-3924-4830)
- Christabella Ng (ORCID: https://orcid.org/0000-0001-7243-3014)
- Penny Gowland
- Chris Bradley
Publication Details
- Journal
- Proceedings on CD-ROM - International Society for Magnetic Resonance in Medicine. Scientific Meeting and Exhibition/Proceedings of the International Society for Magnetic Resonance in Medicine, Scientific Meeting and Exhibition
- Published
- 2024-11-26
- DOI
- https://doi.org/10.58530/2024/0291
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 0.00