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Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children

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Abstract

Background

Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives.

Objective

To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics.

Materials and methods

This was a single-center, prospective cross-sectional study conducted between April 2020 and March 2022 in patients with CF versus healthy controls. Patients with CF were divided into two subgroups: those with CF-related liver disease and those without. MR images were acquired on a 1.5-tesla machine. Kilopascal (kPa) values were derived from processing MR elastography images. MR spectroscopy was used to measure liver fat fraction, as an indication of hepatosteatosis. Groups were compared using either the Student’s t test or the Mann‒Whitney U test. The chi-square test or Fisher’s exact test were used to compare qualitative variables.

Results

Fifty-one patients with CF (12 ± 3.3 years, 32 boys) and 24 healthy volunteers (11.1 ± 2.4 years, 15 boys) were included in the study. Median liver stiffness (P=0.003) and fat fraction (P=0.03) were higher in the CF patients than in the controls. Median liver stiffness values were higher in CF patients with CF-related liver disease than in those without CF-related liver disease (P=0.002). Liver stiffness values of CF patients with high alanine aminotransferase (ALT), high gamma-glutamyl transferase, and thrombocytopenia were found to be higher than those without (P=0.004, P<0.001, P<0.001, respectively). Only the high ALT group showed a high fat fraction (P=0.002).

Conclusions

Patients with CF had higher liver stiffness than the control group, and patients with CF-related liver disease had higher liver stiffness than both the CF patients without CF-related liver disease and the control group. Patients with CF had a higher fat fraction than the control group. Noninvasive assessment of liver involvement using MR elastography/spectroscopy can support the diagnosis of CF-related liver disease and the follow-up of patients with CF.

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Data availability

Data will be made available on reasonable request.

References

  1. Chen Q, Shen Y, Zheng J (2021) A review of cystic fibrosis: basic and clinical aspects. Anim Model Exp Med 4:220–232

    Article  Google Scholar 

  2. Sakiani S, Kleiner DE, Heller T, Koh C (2019) Hepatic manifestations of cystic fibrosis. Clin Liver Dis 23:263–277

    Article  PubMed  PubMed Central  Google Scholar 

  3. Debray D, Narkewicz MR, Bodewes FAJA et al (2017) Cystic fibrosis-related liver disease: research challenges and future perspectives. J Pediatr Gastroenterol Nutr 65:443–448

    Article  PubMed  Google Scholar 

  4. Cystic Fibrosis Foundation (2021) Patient registry 2021 annual data report. https://www.cff.org/sites/default/files/2021-11/Patient-Registry-Annual-Data-Report.pdf. Accessed 15 Nov 2023

  5. Staufer K (2020) Current treatment options for cystic fibrosis-related liver disease. Int J Mol Sci 21:8586

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Gupta NA, Karpen SJ (2018) Bile formation and cholestasis. In: Kleinman RE, Goulet OJ, Mieli-Vergani G, Sanderson IR, Sherman PM, Shneider BL (eds) Walker’s Pediatric Gastrointestinal Disease, 6th edn. People’s Medical Publishing House, North Carolina, pp 1113–1122

    Google Scholar 

  7. Toledano MB, Mukherjee SK, Howell J et al (2019) The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study. PLoS ONE 14:e0212779

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Martonosi ÁR, Soós A, Rumbus Z et al (2012) Non-invasive diagnostic tests in cystic fibrosis-related liver disease: a diagnostic test accuracy network meta-analysis. Front Med (Lausanne) 8:598382

    Article  Google Scholar 

  9. Guglielmo FF, Venkatesh SK, Mitchell DG (2019) Liver MR elastography technique and image interpretation: pearls and pitfalls. Radiographics 39:1983–2002

    Article  PubMed  Google Scholar 

  10. Huwart L, Sempoux C, Vicaut E et al (2008) Magnetic resonance elastography for the noninvasive staging of liver fibrosis. Gastroenterology 135:32–40

    Article  PubMed  Google Scholar 

  11. Tang A, Cloutier G, Szeverenyi NM, Sirlin CB (2015) Ultrasound elastography and MR elastography for assessing liver fibrosis: part 1, principles and techniques. AJR Am J Roentgenol 205:22–32

    Article  PubMed  PubMed Central  Google Scholar 

  12. Demirtaş D, Ünal E, İdilman İS et al (2023) Magnetic resonance elastography in evaluation of liver fibrosis in children with chronic liver disease. Insights Imaging 14:39

    Article  PubMed  PubMed Central  Google Scholar 

  13. Zhang YN, Fowler KJ, Hamilton G et al (2018) Liver fat imaging-a clinical overview of ultrasound, CT, and MR imaging. Br J Radiol 91:20170959

    Article  PubMed  PubMed Central  Google Scholar 

  14. Hamilton G, Middleton MS, Bydder M et al (2009) Effect of PRESS and STEAM sequences on magnetic resonance spectroscopic liver fat quantification. J Magn Reson Imaging 30:145–152

    Article  PubMed  PubMed Central  Google Scholar 

  15. Özcan HN, Oğuz B, Haliloğlu M et al (2015) Imaging patterns of fatty liver in pediatric patients. Diagn Interv Radiol 21:355–360

    Article  PubMed  PubMed Central  Google Scholar 

  16. de Onis M, Onyango AW, Borghi E et al (2007) Development of a WHO growth reference for school-aged children and adolescents. Bull World Health Organ 85:660–667

    Article  PubMed  PubMed Central  Google Scholar 

  17. Debray D, Kelly D, Houwen R et al (2011) Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros 10:S29-36

    Article  PubMed  Google Scholar 

  18. Suttorp M, Classen CF (2021) Splenomegaly in children and adolescents. Front Pediatr 9:704635

    Article  PubMed  PubMed Central  Google Scholar 

  19. Konuş OL, Ozdemir A, Akkaya A et al (1998) Normal liver, spleen, and kidney dimensions in neonates, infants, and children: evaluation with sonography. AJR Am J Roentgenol 171:1693–1698

    Article  PubMed  Google Scholar 

  20. Di Serafino M, Severino R, Gioioso M et al (2020) Paediatric liver ultrasound: a pictorial essay. J Ultrasound 23:87–103

    Article  PubMed  Google Scholar 

  21. Starekova J, Hernando D, Pickhardt PJ, Reeder SB (2021) Quantification of liver fat content with CT and MRI: state of the art. Radiology 301:250–262

    Article  PubMed  Google Scholar 

  22. Menten R, Leonard A, Clapuyt P et al (2010) Transient elastography in patients with cystic fibrosis. Pediatr Radiol 40:1231–1235

    Article  PubMed  Google Scholar 

  23. Kitson MT, Kemp WW, Iser DM et al (2013) Utility of transient elastography in the non-invasive evaluation of cystic fibrosis liver disease. Liver Int 33:698–705

    Article  CAS  PubMed  Google Scholar 

  24. Calvopina DA, Noble C, Weis A et al (2020) Supersonic shear-wave elastography and APRI for the detection and staging of liver disease in pediatric cystic fibrosis. J Cyst Fibros 19:449–454

    Article  PubMed  Google Scholar 

  25. Dana J, Girard M, Franchi-Abella S et al (2022) Comparison of transient elastography, shearwave elastography, magnetic resonance elastography and FibroTest as routine diagnostic markers for assessing liver fibrosis in children with cystic fibrosis. Clin Res Hepatol Gastroenterol 46:101855

    Article  CAS  PubMed  Google Scholar 

  26. Desmond CP, Wilson J, Bailey M et al (2007) The benign course of liver disease in adults with cystic fibrosis and the effect of ursodeoxycholic acid. Liver Int 27:1402–1408

    Article  CAS  PubMed  Google Scholar 

  27. Dana J, Debray D, Beaufrère A et al (2022) Cystic fibrosis-related liver disease: clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies. J Hepatol 76:420–434

    Article  CAS  PubMed  Google Scholar 

  28. Woodruff SA, Sontag MK, Accurso FJ et al (2017) Prevalence of elevated liver enzymes in children with cystic fibrosis diagnosed by newborn screen. J Cyst Fibros 16:139–145

    Article  CAS  PubMed  Google Scholar 

  29. Egan ME, Schecter MS, Voynow JA (2020) Cystic fibrosis. In: Kliegman R, St. Geme JW (eds) Nelson Textbook of Pediatrics, 21st edn. Elsevier, Philadelphia, pp. 2282–2297

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Acknowledgements

The authors cordially thank Prof. Dr. Musturay Karcaaltincaba for conceptualization of the study.

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Authors and Affiliations

  1. Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey

    Aziz Anil Tan

  2. Department of Pediatrics, Division of Pediatric Gastroenterology, Hacettepe University School of Medicine, Ankara, Turkey

    Duygu Demirtas, Hayriye Hizarcioglu-Gulsen & Hasan Ozen

  3. Department of Biostatistics, Hacettepe University School of Medicine, Ankara, Turkey

    Jale Karakaya

  4. Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey

    Emel Isiyel

  5. Department of Radiology, Division of Pediatric Radiology, Hacettepe University School of Medicine, Sihhiye, 06100, Ankara, Turkey

    Berna Oguz, Mithat Haliloglu & H. Nursun Ozcan

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  1. Aziz Anil Tan
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Contributions

A.A.T., D.D., H.H.G., E.I., H.O., B.O., M.H., and H.N.O. planned the study. A.A.T., D.D., E.I., and H.N.O. collected the data. A.A.T., H.N.O., B.O., and M.H. interpreted radiological data. D.D., H.H.G., E.I., and H.O. interpreted clinical data. J.K. performed the statistical analyses. A.A.T. and H.N.O. drafted the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to H. Nursun Ozcan.

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Ethics approval and consent to participate

Written informed consent was signed by both patients and by legally authorized representatives of patients. The study was approved by Hacettepe University Clinical Research Ethics Committee and performed in accordance with the ethical standards as laid down in the 1964 Helsinki Declaration and its later amendments.

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Tan, A.A., Demirtas, D., Hizarcioglu-Gulsen, H. et al. Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children. Pediatr Radiol 54, 250–259 (2024). https://doi.org/10.1007/s00247-023-05832-1

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  • DOI: https://doi.org/10.1007/s00247-023-05832-1

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