Extra-muscular manifestations in GNE myopathy patients: A nationwide repository questionnaire survey in Japan.

OBJECTIVE: GNE myopathy is a rare autosomal recessive adult-onset distal myopathy caused by biallelic pathogenic variants in GNE. Although some extra-muscular manifestations associated with GNE myopathy have been reported, little is known about whether they are disease-specific and how often they present. This study aimed to characterize extra-muscular manifestations of GNE myopathy. METHODS: We conducted a questionnaire survey of GNE myopathy patients registered in a national registry in Japan. The questionnaire requested information regarding idiopathic thrombocytopenia, cardiac involvement, respiratory involvement, sleep apnea syndrome (SAS), and psychiatric diseases. RESULTS: The response rate was 62.4% (126/198), yielding a total of 51 male and 75 female participants. Of the participants, 4.1% (5/123) had a diagnosis of idiopathic thrombocytopenia, and 16.3% (8/49) of males and 6.6% of females (5/76) had a diagnosis of SAS. In total, 0.8% (1/126) of participants had pervasive developmental disabilities and 14.7% (16/109) had a psychiatric disease. CONCLUSION: The frequencies of idiopathic thrombocytopenia and SAS among Japanese GNE myopathy patients were higher than those observed in the general Japanese population. Routine blood tests and evaluation of sleep-disordered breathing should be considered in order to better manage GNE myopathy patients.

Pregnancy in GNE myopathy patients: a nationwide repository survey in Japan.

BACKGROUND: GNE myopathy is an autosomal recessive adult-onset distal myopathy. While a few case reports have described the progression of GNE myopathy during pregnancy, to our knowledge, none have examined disease progression after delivery or obstetric complications. OBJECTIVE: This study aimed to reveal maternal complications, newborn complications, and the impact of pregnancy on disease progression in GNE myopathy patients. METHODS: We conducted a questionnaire survey on pregnancy, delivery, and newborns involving female GNE myopathy patients who are currently registered in a national registry in Japan. RESULTS: The response rate for the questionnaire survey was 60.0% (72/120). Of the 72 respondents, 44 (61.1%) had pregnancy experience (average, 1.8 pregnancies; 53 pregnancies before onset and 28 after onset). The incidence of threatened abortion was 26.9% among post-onset pregnancies, which was higher compared to those of the general Japanese population (p = 0.03). No other maternal or infant complications were commonly observed. Over 80% were unaware of changes in disease progression during pregnancy (mean age, 32.8 ± 3.5 years) or after delivery (32.9 ± 3.8 years), while 19.0% experienced disease exacerbation within a year after delivery (30.0 ± 1.0 years). Six patients developed myopathy within a year after delivery (29.7 ± 4.6 years), while none developed myopathy during pregnancy. CONCLUSIONS: There were no serious maternal or newborn complications, and subjective progression did not differ during or after delivery in the majority of GNE myopathy patients. However, our findings suggest the importance of considering the possibility of threatened abortion and disease progression after delivery.

Effects of Dietary Supplementation of Astaxanthin and Sesamin on Daily Fatigue: A Randomized, Double-Blind, Placebo-Controlled, Two-Way Crossover Study.

Severe fatigue can negatively affect quality of life, and oxidative stress may play a role in its mechanism. The aim of this study was to evaluate the effect of dietary supplementation of astaxanthin and sesamin (AS), strong food-derived antioxidants, on fatigue. Twenty-four healthy volunteers were supplemented with AS and placebo, each for four weeks. After each supplementation period, participants underwent tasks inducing mental and physical fatigue (visual display terminal task and ergometer task, respectively). Subjective fatigue was evaluated using a visual analogue scale during and after the mental and physical tasks, and daily subjective fatigue was evaluated by the Chalder fatigue questionnaire. Secondary outcomes included other subjective feelings, work efficiency, autonomic nerve activity, levels of an oxidative stress marker (plasma phosphatidylcholine hydroperoxide (PCOOH)) and safety. AS supplementation was associated with significantly improved recovery from mental fatigue compared with placebo. Increased PCOOH levels during mental and physical tasks were attenuated by AS supplementation. No differences between AS and placebo were detected in secondary outcomes, and no adverse effects of AS supplementation were observed. In conclusion, AS supplementation may be a candidate to promote recovery from mental fatigue which is experienced by many healthy people.

Orally Administered Salacia reticulata Extract Reduces H1N1 Influenza Clinical Symptoms in Murine Lung Tissues Putatively Due to Enhanced Natural Killer Cell Activity.

Influenza is a major cause of respiratory tract infection. Although most cases do not require further hospitalization, influenza periodically causes epidemics in humans that can potentially infect and kill millions of people. To countermeasure this threat, new vaccines need to be developed annually to match emerging influenza viral strains with increased resistance to existing vaccines. Thus, there is a need for finding and developing new anti-influenza viral agents as alternatives to current treatments. Here, we tested the antiviral effects of an extract from the stems and roots of Salacia reticulata (SSRE), a plant rich in phytochemicals, such as salacinol, kotalanol, and catechins, on H1N1 influenza virus-infected mice. Following oral administration of 0.6 mg/day of SSRE, the incidence of coughing decreased in 80% of mice, and only one case of severe pulmonary inflammation was detected. Moreover, when compared with mice given Lactobacillus casei JCM1134, a strain previously shown to help increase in vitro natural killer (NK) cell activity, SSRE-administered mice showed greater and equal NK cell activity in splenocytes and pulmonary cells, respectively, at high effector cell:target cell ratios. Next, to test whether or not SSRE would exert protective effects against influenza in the absence of gut microbiota, mice were given antibiotics before being inoculated influenza virus and subsequently administered SSRE. SSRE administration induced an increase in NK cell activity in splenocytes and pulmonary cells at levels similar to those detected in mice not treated with antibiotics. Based on our results, it can be concluded that phytochemicals in the SSRE exerted protective effects against influenza infection putatively via modulation of the immune response, including enhancement of NK cell activity, although some protective effects were not necessarily through modulation of gut microbiota. Further investigation is necessary to elucidate the molecular mechanisms underlying the protective effects of SSRE against influenza infection.

Improvement in Human Immune Function with Changes in Intestinal Microbiota by Salacia reticulata Extract Ingestion: A Randomized Placebo-Controlled Trial.

UNLABELLED: Plants belonging to the genus Salacia in the Hippocrateaceae family are known to inhibit sugar absorption. In a previous study, administration of Salacia reticulata extract in rats altered the intestinal microbiota and increased expression of immune-relevant genes in small intestinal epithelial cells. This study aimed to investigate the effect of S. reticulata extract in human subjects by examining the gene expression profiles of blood cells, immunological indices, and intestinal microbiota. The results revealed an improvement in T-cell proliferation activity and some other immunological indices. In addition, the intestinal microbiota changed, with an increase in Bifidobacterium and a decrease in Clostridium bacteria. The expression levels of many immune-relevant genes were altered in blood cells. We concluded that S. reticulata extract ingestion in humans improved immune functions and changed the intestinal microbiota. TRIAL REGISTRATION: UMIN Clinical Trials Registry UMIN000011732.

A subchronic oral toxicity study of Salacia reticulata extract powder in rats.

The safety of Salacia plant (Salacia reticulata) extract powder, which is used in Ayurvedic medical practices, was studied in a dose range-finding subchronic toxicity study in Crl:CD Sprague-Dawley rats. Male and female rats were randomly assigned to 4 treatment groups and were treated by oral gavage with 0, 10, 65, and 400 mg/kg body weight/day of the powder for 91 days. Body weight, food consumption, and clinical signs were assessed during the treatment period. Urinalysis, hematology, blood chemistry, and organ weights were determined one day after the final treatment. The animals were euthanized at the end of the treatment and were examined for necropsy and histopathological purposes. No adverse toxicity was observed in the Salacia powder-treated groups with a No Observed Adverse Effect Level of ≧400 mg/kg body weight/day in both male and female SD rats.

[Bacterial contamination of mobile phones shared in hospital wards and the consciousness and behavior of nurses about biological cleanliness].

OBJECTIVES: The purpose of this study was to clarify the contamination of mobile phones shared in hospital wards and its relationship with the consciousness and behavior of nurses about biological cleanliness. METHODS: Samples from mobile phones were cultured to detect viable bacteria (n=110) and Staphylococcus aureus (n=54). A questionnaire survey was conducted on 110 nurses carrying mobile phones on the day of sampling. RESULTS: Viable bacteria were detected on 79.1% of the mobile phones, whereas S. aureus was detected on 68.6%. All the nurses were aware of hand washing with water or alcohol after regular work, but 33.6% of the nurses were not conscious of hand washing with water or alcohol after using a mobile phone. There was a significant positive relationship between the frequency of using mobile phones and the number of hand washings with water or alcohol. A significant negative relationship was found between the detection of viable bacteria and the number of hand washings with alcohol. The results of logistic regression analysis showed that the detection of viable bacteria was related significantly with the number of hand washings with alcohol (Odds ratio, 0.350; 95%CI, 0.143-0.857) and that the detection of S. aureus was related significantly with the frequency of using mobile phones (Odds ratio, 0.183; 95%CI, 0.036-0.933). CONCLUSIONS: It is important to be conscious of the fact that mobile phones shared in hospital wards are easily contaminated. Because hand washing with water or alcohol prevents the contamination of the mobile phones, nurses should take standard precautions after using mobile phones.

Biochemical investigation and gene expression analysis of the immunostimulatory functions of an edible Salacia extract in rat small intestine.

Roots and bark from plants belonging to genus Salacia of the family Hippocrateaceae (Salacia reticulata, Salacia oblonga, etc.) have been used for traditional Ayurvedic medicine, particularly for the treatment of diabetes. In our study, we evaluated the gene expression profiles in the small intestinal epithelium of rats that were given a Salacia plant extract to gain insight into its effects on the small intestine. In detail, DNA microarray analysis was performed to evaluate the gene expression profiles in the rat ileal epithelium. The intestinal bacterial flora was also studied using T-RFLP (Nagashima method) in these rats. Expressions of many immune-related genes, especially Th1-related genes associated with cell-mediated immunity, were found to increase in the small intestinal epithelium and the intestinal bacterial flora became similar to those in the case with Salacia plant extract administration. Our study thus revealed that Salacia plant extract exerts bioregulatory functions by boosting intestinal immunity.