title: Standardization of MRI and Scintigraphic Scores for Assessing the Severity of Bone Marrow Involvement in Adult Patients With Type 1 Gaucher Disease.
abstract: OBJECTIVE
  MRI and (99m)Tc-sestamibi scintigraphy are used to estimate bone marrow infiltration in patients with Gaucher disease (GD), but comparison of data obtained at different institutions is difficult because different scores are employed for semiquantitative assessment. We developed normalized scores for comparing data both within a single method (MRI) and between different methods (MRI versus scintigraphy).
  
  
  METHODS
  We evaluated 51 patients with type 1 GD (26 women, 25 men; mean age ± SD, 36.3 ± 10.9 years old). T1- and T2-weighted turbo spin-echo sequences at 1.5 T served to derive the bone marrow burden score (0-16), the vertebra-disk ratio (VDR), the Terk score (0-3), and the Spanish-MRI score (S-MRI, 0-24). Scintigraphy was scored between 0 and 8. Each score was normalized into four categories: 0 = normal, 1 = mild, 2 = intermediate, 3 = severe involvement. Interobserver and intraobserver agreements were evaluated by kappa statistics; nonparametric statistics with Bonferroni correction assessed correlations among the various original and normalized scores.
  
  
  RESULTS
  Interobserver agreement was excellent for the original scores (κ = 0.730-0.843) and even more so for the normalized scores (κ = 0.775-0.940). Intraobserver agreement kappa values ranged from 0.753 to 0.937 for the original scores and 0.851 to 1.000 for the normalized scores. Highly significant correlations were found among the various original scores (r = 0.42-0.86, p values between 0.0296 and < 0.0001), except for VDR versus S-MRI and Terk. Normalization generally induced marginal reductions in statistical significance, whereas S-MRI versus VDR reached statistical significance with the normalized scores.
  
  
  CONCLUSIONS
  Our data indicate no significant loss of statistical information is caused by the normalization we employed. Our approach therefore facilitates comparison of different scores obtained in different institutions with different imaging modalities.
authors.affiliation: 1 Department of Translational Research and Advanced Technologies in Medicine and Surgery, Regional Center of Nuclear Medicine, University of Pisa, Via Roma 67, Pisa I-56126, Italy.
authors.forename: Giuliano
authors.initials: G
authors.lastname: Mariani
authors.affiliation: 1 Department of Translational Research and Advanced Technologies in Medicine and Surgery, Regional Center of Nuclear Medicine, University of Pisa, Via Roma 67, Pisa I-56126, Italy.
authors.forename: Marzio
authors.initials: M
authors.lastname: Perri
authors.affiliation: 2 CNR Institute of Clinical Physiology, Pisa, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Minichilli
authors.affiliation: 3 Department of Diagnostic and Interventional Radiology, University of Pisa, Pisa, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Ortori
authors.affiliation: 4 Agency for Hemophilia and Regional Reference Center for Inherited Bleeding Disorders, "Careggi" University Hospital, Florence, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Linari
authors.affiliation: 5 Department of Cell Biotechnologies and Hematology, University of Rome "La Sapienza," Rome, Italy.
authors.forename: Fiorina
authors.initials: F
authors.lastname: Giona
authors.affiliation: 6 "G. Gaslini" Children's Hospital, Genoa, Italy.
authors.forename: Maja
authors.initials: M
authors.lastname: Di Rocco
authors.affiliation: 7 Department of Internal Medicine, "Policlinico Mangiagalli Regina Elena" Foundation IRCCS, University of Milan, Milan, Italy.
authors.forename: Maria Domenica
authors.initials: MD
authors.lastname: Cappellini
authors.affiliation: 1 Department of Translational Research and Advanced Technologies in Medicine and Surgery, Regional Center of Nuclear Medicine, University of Pisa, Via Roma 67, Pisa I-56126, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Guidoccio
authors.affiliation: 1 Department of Translational Research and Advanced Technologies in Medicine and Surgery, Regional Center of Nuclear Medicine, University of Pisa, Via Roma 67, Pisa I-56126, Italy.
authors.forename: Paola Anna
authors.initials: PA
authors.lastname: Erba
mesh_terms: D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D005269:Femur; D005776:Gaucher Disease / Q000000981:diagnostic imaging*; D006801:Humans; D008159:Lumbar Vertebrae; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D010388:Pelvis; D011877:Radionuclide Imaging; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012720:Severity of Illness Index; D017256:Technetium Tc 99m Sestamibi
chemical_list: D017256:Technetium Tc 99m Sestamibi
keywords: Gaucher disease; MRI; bone marrow; scintigraphy