The MDFRS is a reliable and valid disease-specific measure of functional status for patients with muscular dystrophy.

Muscular dystrophy (MD) comprises a group of diseases characterized by progressive muscle weakness that induces functional deterioration. Clinical management requires the use of a well-designed scale to measure patients' functional status. This study aimed to investigate the quality of the functional scales used to assess patients with different types of MD. The Brooke scale and the Vignos scale were used to grade arm and leg function, respectively. The Barthel Index was used to evaluate the function of daily living activity. We performed tests to assess the acceptability of these scales. The characteristics of the different types of MD are discussed. This was a multicenter study and included patients diagnosed with Duchenne muscular dystrophy (DMD) (classified as severely progressive MD), Becker muscular dystrophy (BMD), limb girdle muscular dystrophy (LGMD) and facioscapulohumeral muscular dystrophy (FSHD). BMD, LGMD, and FSHD were classified as slowly progressive MD. The results demonstrated that the Brooke scale was acceptable for grading arm function in DMD, but was unable to discriminate between differing levels of severity in slowly progressive MD. The floor effect was large for all types of slowly progressive MD (range, 20.0-61.9), and was especially high for BMD. The floor effect was also large for BMD (23.8%) and FSHD (50.0%) using the Vignos scale. Grades 6-8 of the Vignos scale were inapplicable because they included items involving the use of long leg braces for walking or standing, and some patients did not use long leg braces. In the Barthel Index, a ceiling effect was prominent for slowly progressive MD (58.9%), while a floor effect existed for DMD (17.9%). Among the slowly progressive MDs, FSHD patients had the best level of functioning; they had better leg function and their daily living activities were less affected than patients with other forms of slowly progressive MD. The results of this study demonstrate the acceptability of the different applications used for measuring functional status in patients with different types of MD. Some of the limitations of these measures as applied to MD should be carefully considered, especially in patients with slowly progressive MD. We suggest that these applications be used in combination with other measures, or that a complicated instrument capable of evaluating the various levels of functional status be used.

Sixteen nif and 'nif-associated genes (expressed only under conditions of nitrogen fixation) in Synechococcus sp. strain RF-1 have been cloned and sequenced. All of the nif and nif-associated genes identified in Synechococcus RF-1 were arranged in a continuous cluster spanning approximately 18 kb and containing seven operons. The nifH operon (nifH-nifD-nifK) has been reported previously. nifB, fdXN, nifS8 nifU and nifP were found to be located upstream of the nifH operon. nifB-WxN-nifS-nifU were expressed as an operon. A nifPlike gene was found to be located just upstream of nifB. nifE, nifN, nifX, nifW and the nif-associated hesA, hesB and 'fdx' were found to be located downstream from nifK. The genes located downstream from nifK are arranged nifE-nifN-nifX-orf-nifW-hesA-hesB-' fdx' and span approximately 7 kb. The function of the ORF situated between nifX and niNV is not known. However, it was identified as a counterpart of ORF-2 in Anabaena sp. strain PCC 7120 based on the deduced amino acid sequence. Northern hybridization and primer extension analysis indicated that the nif and nif-associated genes are organized in nifE-nifN, nifx-orf, niW-hesA-hesB and fdx'-containing operons, respectively. According to the results of this study and previous reports, the genes are expressed in a rhythmic pattern with peaks during the dark phase when the culture is grown in a 12 h IighVl2 h dark regimen. The rhythm persisted after the culture was transferred to continuous illumination.

The purpose of this study was to investigate health-related quality of life (HRQOL) and associated factors in patients with chronic neck pain (CNP). The HRQOL of patients with CNP was assessed by the Short Form-36 questionnaire in this cross-sectional study. To evaluate the psychological factors related to HRQOL, the Eysenck Personality Questionnaire, Chinese Health Questionnaire, and Beck Anxiety Inventory were used. The scores for the eight subscales of Short Form-36 were all lower than the Taiwanese age-matched normative values (p < 0.001). The two most strongly affected subscales were the role-physical subscale and the bodily pain subscale; both scores were below half the score of the age-/sex-matched normative values. The physical components summary score, a summary measure, was moderately correlated with age (rho = -0.43), education level (rho = 0.37) and Beck Anxiety Inventory score (rho = -0.36). The mental components summary score was moderately to highly correlated with the Chinese Health Questionnaire score (rho = -0.72), the neuroticism domain of Eysenck Personality Questionnaire (rho = -0.52) and Beck Anxiety Inventory score (rho = -0.41). The HRQOL of patients with CNP was worse than that of normal subjects across all domains. Furthermore, patients with a neurotic personality, minor psychiatric morbidity and higher anxiety status showed poor mental health, as measured by the Short Form-36. We found that patients with CNP had multiple physical and mental health problems in terms of. The mental health of patients with CNP was strongly associated with various psychological factors. Comprehensive assessment of the physical and mental functioning of patients with CNP can improve the management and care of these patients.