The Efficacy of MRI in the diagnostic workup of cystic fibrosis-associated liver disease: A clinical observational cohort study.

Purpose: To identify independent imaging features and establish a diagnostic algorithm for diagnosis of cystic fibrosis (CF)-associated liver disease (CFLD) in CF patients compared to controls using gadoxetic acid-enhanced MRI.Methods: A total of 90 adult patients were enrolled: 50 with CF, 40 contr...

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Publicado en:European Radiology Vol. 29; no. 2; pp. 1048 - 1059
Autores principales: Poetter-Lang, Sarah, Staufer, Katharina, Baltzer, Pascal, Tamandl, Dietmar, Muin, Dina, Bastati, Nina, Halilbasic, Emina, Hodge, Jacqueline C., Trauner, Michael, Kazemi-Shirazi, Lili, Ba-Ssalamah, Ahmed
Formato: Journal Article
Publicado: Springer Nature Feb2019
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Feb2019
      vid: 29
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      pub: Springer Nature
      place: New York, New York
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        10.1007/s00330-018-5650-5
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        atl: The Efficacy of MRI in the diagnostic workup of cystic fibrosis-associated liver disease: A clinical observational cohort study.
      aug:
        au:
          Poetter-Lang, Sarah
          Staufer, Katharina
          Baltzer, Pascal
          Tamandl, Dietmar
          Muin, Dina
          Bastati, Nina
          Halilbasic, Emina
          Hodge, Jacqueline C.
          Trauner, Michael
          Kazemi-Shirazi, Lili
          Ba-Ssalamah, Ahmed
        affil: General Hospital of Vienna (AKH), Department of Biomedical Imaging and Image-guided Therapy, Medical University Vienna, Waehringer Guertel 18-20, A-1090, Vienna, Austria
      sug:
        subj:
          Cystic Fibrosis
          Liver Diseases
          Algorithms
          Multivariate Analysis
          Liver Diseases Etiology
          Male
          Severity of Illness Indices
          Middle Age
          Prospective Studies
          Retrospective Design
          Adult
          Cystic Fibrosis Complications
          Sensitivity and Specificity
          ROC Curve
          Young Adult
          Contrast Media
          Magnetic Resonance Imaging Methods
          Female
          Scales
          Short Portable Mental Status Questionnaire
          Middle Aged: 45-64 years
          Adult: 19-44 years
          Male
          Female
      ab: Purpose: To identify independent imaging features and establish a diagnostic algorithm for diagnosis of cystic fibrosis (CF)-associated liver disease (CFLD) in CF patients compared to controls using gadoxetic acid-enhanced MRI.Methods: A total of 90 adult patients were enrolled: 50 with CF, 40 controls. The CF group was composed of two subgroups: a retrospective test subgroup (n = 33) and a prospective validation subgroup (n = 17). Controls (patients with normal liver enzymes and only benign focal liver lesions) were divided accordingly (27:13). MRI variables, including quantitative and qualitative parameters, were used to distinguish CFLD from controls using clinical symptoms, laboratory tests and Debray criteria. Disease severity was classified according to Child-Pugh and Albumin-Bilirubin (ALBI) scores. Fifteen qualitative single-lesion CF descriptors were defined. Two readers independently evaluated the images. Univariate statistical analysis was performed to obtain significant imaging features that differentiate CF patients from controls. Through multivariate analysis using chi-squared automatic interaction detector (CHAID) methodology the most important descriptors were identified. Diagnostic performance was assessed by receiver-operating characteristic (ROC) analysis.Results: Three independent imaging descriptors distinguished CFLD from controls: (1) presence of altered gallbladder morphology; (2) periportal tracking; and (3) periportal fat deposition. Prospective validation of the classification algorithm demonstrated a sensitivity of 94.1% and specificity of 84.6% for discriminating CFLD from controls. Disease severity was well associated with the imaging features.Conclusions: A short unenhanced MRI protocol can identify the three cardinal imaging features of CFLD. The hepatobiliary phase of gadoxetic acid-enhanced MRI can define CFLD progression.Key Points: • Using a multivariate classification analysis, we identified three independent imaging features, altered gallbladder morphology (GBAM), periportal tracking (PPT) and periportal fat deposition (PPFD), that could diagnose CFLD with high sensitivity, 94.1 % (95% CI: 71.3-99.9) and moderate specificity, 84.6 % (95% CI: 54.6-98.1). • Based upon the results of this study, gadoxetic acid-enhanced MRI with DWI is able to diagnose early-stage CFLD, as well as its progression.
      pubtype: Academic Journal
      doctype: Journal Article
      ougenre: Article
    language: English
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