Validity and Reproducibility of a Self-Administered Food Frequency Questionnaire for the Assessment of Sugar Intake in Middle-Aged Japanese Adults.

We evaluated the validity and reproducibility of estimated sugar intakes using a food frequency questionnaire (FFQ) among middle-aged Japanese adults in the Japan Public Health Centre-Based Prospective (JPHC) study. In subsamples of the JPHC study (Cohorts I and II in multiple areas), we computed Spearman's correlations of FFQ results with urine sugar concentrations and dietary records (DR) for validity; we evaluated correlations between two FFQs for reproducibility. During 1994⁻1998, participants (Cohort I: n = 27 [men], n = 45 [women]) provided two (spring and fall) 24-h urine samples and completed 7-consecutive-day DR per season (I: n = 99, n = 113; II: n = 168, n = 171) and two FFQs (147 food items) at yearly intervals (I: n = 101, n = 108; II: n = 143, n = 146). Sugar intakes from FFQ were correlated with urinary sugar (de-attenuated correlations: 0.40; 95%CI: 0.19, 0.58). After adjustment for sociodemographic and lifestyle variables, correlations between FFQ and DR for men and women were 0.57 (0.42, 0.69) and 0.41 (0.24, 0.55) (I) and 0.56 (0.44, 0.65) and 0.34 (0.20, 0.47) (II), respectively. Correlations between FFQs for men and women were 0.63 (0.49, 0.73) and 0.55 (0.41, 0.67) (I) and 0.66 (0.55, 0.74) and 0.63 (0.52, 0.72) (II). In conclusion, our study showed moderate FFQ validity and reproducibility for sugar intake evaluation.

[Various central nervous system involvements in dystrophinopathy: clinical and genetic considerations].

Duchenne and Becker muscular dystrophies, generically called dystrophinopathy, are caused by mutations of the dystrophin gene. It is not surprising that mutations of the dystrophin gene cause various neurological symptoms, since dystrophin protein is found in the brain tissue as well as in the muscle fiber cell membrane. However, few studies have reported on the frequency of central nervous complications other than mental retardation. Also, the relationship between the types of abnormal dystrophin gene and central nervous symptoms remains to be revealed. Medical records of 200 patients with dystrophinopathy from 167 extended families who had visited our institution during the past 15 years were reviewed to elucidate the frequency of central nervous complications. Fifty-four (27%) had mental retardation (an intelligence quotient less than 70), 15 (7.5%) had autism, 12 (6%) had epilepsy. 8 (4%) had febrile convulsion. 131 of these patients also underwent genetic testing. All patients with central nervous symptoms except one pair of siblings had some form of genetic deficiency or duplication distal to exon 44. Central nervous symptoms other than mental retardation are also common in patients with dystrophinopathy. These central nervous complications may be associated with mutations in the isoforms derived from exon 44 to 79.