Translation, cultural adaptation and validation of the English “Short form SF 12v2” into Bengali in rheumatoid arthritis patients

Background: To develop a culturally adapted and validated Bengali Short Form SF 12v2 among Rheumatoid arthritis (RA) patients. Methods: The English SF 12v2 was translated, adapted and back translated into and from Bengali, pre-tested by 60 patients. The Bengali SF 12v2 was administered twice with 14 days interval to 130 Bangladeshi RA patients. The psychometric properties of the Bengali SF 12v2 were assessed. Test-retest reliability was assessed by intra-class correlation coefficient (ICC) and Spearman's rank correlation coefficient and internal consistency by Cronbach's alpha. Content validity was assessed by index for content validity (ICV) and floor and ceiling effects. To determine convergent and discriminant validity a Bengali Health Assessment Questionnaire (B-HAQ) was used. Factor analysis was done. Results: The Bengali SF 12v2 was well accepted by the patients in the pre-test and showed good reliability. Internal consistency for both physical and mental component was satisfactory; Cronbach's alpha was 0.9. ICC exceeded 0.9 in all domains. Spearman's rho for all domains exceeded 0.8. The physical health component of Bengali SF 12v2 had convergent validity to the B-HAQ. Its mental health component had discriminant validity to the B-HAQ. The ICV of content validity was 1 for all items. Factor analysis revealed two factors a physical and a mental component. Conclusions: The interviewer-administered Bengali SF 12v2 appears to be an acceptable, reliable, and valid instrument for measuring health-related quality of life in Bengali speaking RA patients. Further evaluation in the general population and in different medical conditions should be done

Impact of Claudin 10 deficiency on amelogenesis: lesson from a HELIX tooth

International audienceIn epithelia, claudin proteins are important components of the tight junctions as they determine the permeability and specificity to ions of the paracellular pathway. Mutations in CLDN10 cause the rare autosomal recessive HELIX syndrome (Hypohidrosis, Electrolyte imbalance, Lacrimal gland dysfunction, Ichthyosis, and Xerostomia), in which patients display severe enamel wear. Here, we assess whether this enamel wear is caused by an innate fragility directly related to claudin-10 deficiency in addition to xerostomia. A third molar collected from a female HELIX patient was analyzed by a combination of microanatomical and physicochemical approaches (i.e., electron microscopy, elemental mapping, Raman microspectroscopy, and synchrotron-based X-ray fluorescence). The enamel morphology, formation time, organization, and microstructure appeared to be within the natural variability. However, we identified accentuated strontium variations within the HELIX enamel, with alternating enrichments and depletions following the direction of the periodical striae of Retzius. These markings were also present in dentin. These data suggest that the enamel wear associated with HELIX may not be related to a disruption of enamel microstructure but rather to xerostomia. However, the occurrence of events of strontium variations within dental tissues might indicate repeated episodes of worsening of the renal dysfunction that may require further investigations