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Abstract Objective Becker muscular dystrophy (BMD) is a milder variant of Duchenne muscular dystrophy (DMD), a lethal X‐linked muscular disorder. Here, we aim to investigat the clinical involvement of skeletal, respiratory, cardiac, and central nervous systems in patients with BMD, as well as genotype–phenotype relationships. Methods This nationwide cohort study investigated the clinical manifestations and genotype–phenotype relationships in 225 patients with BMD having in‐frame deletion from 22 medical centers. The primary outcome was to elucidate the association of genotype with skeletal muscle, respiratory, cardiac, and central nervous system disorders. Descriptive statistics were used to analyze the data. Results The average age of the subjects was 31.5 (range, 1–81) years. Initial symptoms of BMD were muscular (60%), followed by asymptomatic hypercreatine kinasemia (32.4%) and central nervous system disorders (5.3%). Gait disturbance was observed in 53.8% of patients and the average age at wheelchair introduction was 36.5 years. The ventilator introduction rate was 6.7% at an average age of 36.6 years. More than 30% of patients had an abnormal electrocardiogram and approximately 15% had heart failure symptoms. Cardiac function on echocardiography varied significantly among the patients. The frequencies of seizures and intellectual/developmental disability were 8.0% and 16.9%, respectively. Exon 45–47deletion (del) was the most common (22.6%), followed by exon 45–48del (13.1%). Patients with exon 45–49del patients demonstrated severe skeletal muscle damage. Patients with exon 45–47del and exon 45–55del patients did not require ventilator use. Interpretation The study provides important prognostic information for patients and clinicians to establish therapy plans and to implement preventative medicine.

Direct numerical simulation (DNS) of a feedback-controlled turbulent channel flow at Reτ = 640 is carried out. As an idealized feedback control, we selectively damp either the small scale wall-normal velocity fluctuations (defined as those with the spanwise wavelength smaller than 300 wall units) or the large scale fluctuations (the spanwise wavelength larger than 300 wall units). The present DNS reveals that the control of small scale fluctuations leads to more drag reduction than that of large scale fluctuations. When the small scale fluctuation is damped, the friction drag is reduced by the amount corresponding to the absence of small scale fluctuation. In contrast, when the large scale fluctuation is damped, the friction drag reduction is much less than that expected from the absence of large scale fluctuation. In the latter case, the contribution from the small scale fluctuation to the friction drag is found to be drastically increased due to the reduction of pressure fluctuation and destruction of Reynolds shear stress.

BACKGROUND: Manually-assisted coughing and mechanical insufflation-exsufflation (MI-E) are commonly used in patients with Duchenne muscular dystrophy (DMD). Few studies have compared cough peak flow (CPF) with manually-assisted coughing to other methods, such as MI-E + manually-assisted coughing. In addition, few studies have reported the reliability of the measured CPF values. This study aimed to compare CPF with different cough-assistance methods and to examine the reliability of CPF data. METHODS: The study included 12 subjects with DMD (mean age, 34 ± 8 y). CPF, CPF + manually-assisted coughing (assisted CPF), maximum insufflation capacity (MIC) + CPF (MIC-CPF), MIC + manually-assisted coughing (MIC+assisted CPF), MI-E (MI-E--CPF), and MI-E + assisted CPF were measured. A spirometer was used to compare CPF measurements obtained with each of the cough-assist techniques. The reliability of the measured CPF values was analyzed using Bland-Altman analysis. RESULTS: CPF was 59 ± 34 L/min, assisted CPF was 113 ± 32 L/min, MIC-CPF was 170 ± 30 L/min, MIC+assisted CPF was 224 ± 62 L/min, MI-E--CPF was 199 ± 40 L/min, and MI-E + assisted CPF was 240 ± 38 L/min. A fixed and proportional bias was found in the CPF measurements made with the peak flow meter and the spirometer. The average 95% CI in the difference between peak flow meter, MI-E, and CPF obtained using the spirometer were -7.45 to -1.95 and -1.45 to 4.95, respectively. Test for correlation was r = 0.54 (P < .001) for CPF (peak flow meter) and CPF (spirometer) and r = 0.17 (P = .17) in CPF (MI-E) and CPF (spirometer), respectively. CONCLUSION: MI-E + assisted CPF was the highest. The CPF measured with the peak flow meter suggested underestimation. [ABSTRACT FROM AUTHOR]

Summary. Fluoranthene ( FA) forms a 1:1 van der Waals complex with benzene in cyclohexane. The 1H NMR spectrum of this complex shows that the FA moiety in the complex state has five kinds of hydrogen atoms and that the 1H NMR peaks assigned to the protons attached to the naphthalene skeleton are largely shifted to higher magnetic field on complex formation with benzene. These observations indicate that the complex takes the structure of C S symmetry, in which the benzene molecule mainly interacts with the p electronic system localized on the naphthalene moiety of FA. The present ab initio calculations reproduce well the 1H NMR spectral shifts mentioned above and the experimentally predicted C S structure of the complex. According to the PPP calculations for the electronic absorption spectral changes on the complex formation, the FA-benzene complex is considered to take a sandwich type structure. [ABSTRACT FROM AUTHOR]