Pediatric immune-mediated necrotizing myopathy.

Immune-mediated necrotizing myopathy (IMNM) is a type of inflammatory myopathy. Most patients with IMNM produce anti-3-hydroxy-3-methylglutaryl coenzyme A reductase or anti-signal-recognition particle autoantibodies. IMNM is much rarer in children than in adults. We conducted this mini review focusing on pediatric IMNM to present current evidence regarding its epidemiology, clinical characteristics, diagnosis, and treatment. Our findings indicate that pediatric IMNM often causes severe muscle weakness and is refractory to corticosteroids alone. Furthermore, delayed diagnosis is common because of the clinicopathological similarity between IMNM and inherited myopathy. Raising awareness regarding pediatric IMNM may facilitate early diagnosis and effective treatment.

A mobile platform for characterizing on-road tailpipe emissions and toxicity of ultrafine particles under real driving Conditions.

Acute exposure to fresh traffic-related air pollutants (TRAPs) can be high for road users, including motorbike drivers, cyclists, and pedestrians. However, evaluating the toxicity of fresh traffic emissions from on-road vehicles is challenging since pollution properties can change dynamically within a short distance and time. This study demonstrated a mobile platform equipped with an On-Board Diagnostic II (OBDII) system, a tailor-made portable emission measurement system, and an electrostatic air-liquid interface exposure system with human monocytic THP-1 cells to characterize on-road tailpipe emissions under real driving conditions. High number concentrations up to 106-107 # cm-3 of ultrafine particles (UFPs) were observed for a gasoline engine at the cold-start stage and a diesel engine during particulate filter regeneration. In particular, a substantial fraction of freshly emitted UFPs within the size less than 23 nm were observed and should be cautioned. The potential toxicity of fresh TRAPs was quantified by cell viability, cytotoxicity, oxidative stress, and inflammatory biomarkers. Results show that the decreased cell viability, increased lactate dehydrogenase (LDH) activity, and high oxidative stress induced by the fresh TRAPs were potentially contributed by gaseous pollutants as well as particles, especially driving with the high idling frequency. Moreover, the dominant contributor to the toxicity is different for gasoline's and diesel's TRAPs. Characterizing on-road air pollutant toxicity as well as physicochemical properties using an innovative mobile platform can fill this knowledge gap.

Quantification of food packaging generation and material loss from major retailers in Taipei, Taiwan.

Packaging accounts for the largest demand for global plastic consumption and around 60% is for food and beverage packaging. The amount of packaging is increasing rapidly due to the expansion of retailer industries, especially in supermarkets and convenience stores. Plastic recycling strategies for food packaging in retailer industries need to be developed, but the current consumption and recycling status is not clear. To address this knowledge gap, this study quantifies the food packaging generation from major supermarket and convenience store chains in Taipei, Taiwan. We focus on the composition, recycling habit, and the recycling status to evaluate the packaging recyclability and major pathways for material losses. Based on our field survey and analysis, the total amount of plastic packaging generation was 21846.04 tons in 2020 with 64.99% of it being recycled. We defined the recycling rates as the continued product of Recyclable Content Ratio of the packaging itself, Sorting Accuracy Ratio of consumers, and Re-granulated Ratio in the final treatment facilities. These three ratios for major food categories were also presented to identify the hotspots of material losses. Our results suggest that to promote plastic food packaging recycling, identifying the limitations in different stages and designing corresponding strategies is crucial.

Treatment experience of Taiwanese patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy.

Immune-mediated necrotizing myopathy (IMNM) has emerged as a new subgroup of idiopathic inflammatory myopathy in the past decade, associated with the presence of two autoantibodies against signal recognition particle and 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR). We aim to analyze the clinical, pathological, and imaging phenotypes of the patients with anti-HMGCR myopathy in our cohort. Five patients with anti-HMGCR myopathy have been enrolled who were all female; three were pediatric and two were adult patients. The muscle pathology of patients met the diagnostic criteria of IMNM. On muscle magnetic resonance imaging, adductors were earliest affected while lower legs were relatively preserved with highest degree of involvement in medial head of gastrocnemius. In upper extremities, biceps brachii was the most severely involved, followed by triceps. All patients were refractory to steroid mono-therapy. For pediatric patients, all three patients eventually became responsive to steroid with either intravenous immunoglobulin or rituximab despite variable motor function recovered at present due to different intervention timing. For adult patients, one with statin exposure responded well to steroid and azathioprine use and the motor function returned to the baseline. The other adult patient finally got stabilized and slowly improved with steroid and methotrexate 13 years after the start of therapy. The creatine kinase (CK) levels of all patients were decreased along with clinical severity. In conclusion, muscle imaging might be of help for the diagnosis. Treatment with immuno-suppressants could be considered together with steroid from the beginning.

Clinical, pathological, imaging, and genetic characterization in a Taiwanese cohort with limb-girdle muscular dystrophy.

BACKGROUND: Limb-girdle muscular dystrophy (LGMD) is a genetically heterogeneous, hereditary disease characterized by limb-girdle weakness and histologically dystrophic changes. The prevalence of each subtype of LGMD varies among different ethnic populations. This study for the first time analyzed the phenotypes and genotypes in Taiwanese patients with LGMD in a referral center for neuromuscular diseases (NMDs). RESULTS: We enrolled 102 patients clinically suspected of having LGMD who underwent muscle biopsy with subsequent genetic analysis in the previous 10 years. On the basis of different pathological categories, we performed sequencing of target genes or panel for NMDs and then identified patients with type 1B, 1E, 2A, 2B, 2D, 2I, 2G, 2 N, and 2Q. The 1B patients with LMNA mutation presented with mild limb-girdle weakness but no conduction defect at the time. All 1E patients with DES mutation exhibited predominantly proximal weakness along with distal weakness. In our cohort, 2B and 2I were the most frequent forms of LGMD; several common or founder mutations were identified, including c.1097_1099delACA (p.Asn366del) in DES, homozygous c.101G > T (p.Arg34Leu) in SGCA, homozygous c.26_33dup (p.Glu12Argfs*20) in TCAP, c.545A > G (p.Tyr182Cys), and c.948delC (p.Cys317Alafs*111) in FKRP. Clinically, the prevalence of dilated cardiomyopathy in our patients with LGMD2I aged > 18 years was 100%, much higher than that in European cohorts. The only patient with LGMD2Q with PLEC mutation did not exhibit skin lesions or gastrointestinal abnormalities but had mild facial weakness. Muscle imaging of LGMD1E and 2G revealed a more uniform involvement than did other LGMD types. CONCLUSION: Our study revealed that detailed clinical manifestation together with muscle pathology and imaging remain critical in guiding further molecular analyses and are crucial for establishing genotype-phenotype correlations. We also determined the common mutations and prevalence for different subtypes of LGMD in our cohort, which could be useful when providing specific care and personalized therapy to patients with LGMD.

The natural history of the patients with Duchenne muscular dystrophy in Taiwan: A medical center experience.

BACKGROUND: Duchenne muscular dystrophy (DMD) is the most common hereditary muscular dystrophy and caused by DMD gene mutation. In addition to progressive proximal muscle weakness, respiratory, orthopedic, and gastrointestinal complications are often observed in DMD. The natural history of patients with DMD in Taiwan has not been reported thus far. METHODS: Medical records of 39 patients who received a diagnosis of DMD between 1999 and 2016 at Kaohsiung Medical University Hospital were reviewed. The diagnosis of DMD was confirmed through muscle biopsy or DMD genetic analysis. RESULTS: The mean onset age and mean follow-up period were 2.75 years and 6.76 years, respectively. Seventeen patients (43.5%) had a family history of DMD. The mean full intelligence quotient of the patients was 71.08, and the mean age of walking ability loss was 9.7 years (25 patients). The mean onset age of respiratory insufficiency was 10.64 years with a decline rate of 5.18% per year (25 patients). The mean onset age of cardiomyopathy was 14.69 years (seven patients). The mean onset age of scoliosis was 13.29 years with a progression rate of 11.48° per year (14 patients). Eleven (28.2%) and eight (20.5%) patients had deletions and duplications of DMD, respectively. Fourteen patients (35.9%) had point mutations or small deletions or insertions. Five patients received only multiplex ligation-dependent probe amplification (MLPA) analysis and exhibited neither deletion nor duplication. No mutation was identified in one patient through both MLPA and exon sequencing. CONCLUSION: The clinical phenotypes and disease course in our cohort were consistent with that reported in previous studies. However, the proportion of point mutations or small deletions or insertions in our study was considerably higher than that in reports from other populations. Cardiac ejection fraction was found not a reliable biomarker for identifying cardiac problems, discovering a better parameter is necessary.

Effect of cantharidins in chemotherapy for hepatoma: a retrospective cohort study.

This study aimed to evaluate the effects of cantharidins, a traditional Chinese medicine, in chemotherapy for the treatment of hepatoma. From August 2011 to December 2012, 96 patients with hepatoma, who were eligible for transcatheter hepatic arterial chemoembolization and received cantharidins, were selected for comparison with the control group of 95 patients without cantharidins. The treatment effect, clinical symptoms and adverse effects were analyzed. The results of the study showed that the cantharidins group had a higher overall efficient rate than the control group (p < 0.001). The improvement rate of the Karnofsky score in the cantharidins group was significantly higher than that of the control group (p = 0.014). In the cantharidins group, there was a decrease in white blood cell (WBC) count and gastrointestinal response rates were lower than those of the control group (p < 0.05). Therefore, the traditional Chinese medicine cantharidins showed effects of easing the progress of liver cancer, relieving side effects of chemotherapy and improving the quality of life in the treatment of hepatoma.

Trachway intubating stylet for tracheal intubation in an ankylosing spondylitis patient undergoing total hip replacement under general anesthesia.

The Trachway is a new device which provides better glottic view than conventional direct laryngoscopy during tracheal intubation. This intubating stylet consists of a camera on the distal tip of the style, with a monitor attached to the rechargeable handle, so that it can overcome the difficulty of limited neck motion and mouth opening in tracheal intubation. We present here a 54-year-old man with ankylosing spondylitis, scheduled to undergo total hip replacement. Pre-operative airway assessment revealed a recognized difficult airway. The Trachway was successfully used for oral tracheal intubation at the first attempt. The Trachway can be an alternative choice for intubation in ankylosing spondylitis patients.

Effect of combining ultralow-dose naloxone with morphine in intravenous patient-controlled analgesia: the cut-off ratio of naloxone to morphine for antiemesis after gynecologic surgery.

BACKGROUND/PURPOSE: Admixing an ultralow dose of naloxone with intravenous morphine patient-controlled analgesia (PCA) has been shown to decrease postoperative nausea. However, the cut-off ratio of the naloxone-morphine admixture for antiemetic effects has not been investigated. The purpose of this study was to investigate the cut-off ratio of naloxone-morphine admixture in PCA for antiemesis after gynecologic surgery. METHODS: This double-blind study enrolled 120 female patients who were scheduled for gynecologic surgery under general anesthesia. Patients were randomly allocated to one of three groups (n = 40 for each group). The concentration of naloxone and morphine respectively was 0 microg/mL and 1 mg/mL in group 1, 0.1 microg/mL and 1 mg/mL in group 2 (1:10,000), and 1 microg/mL and 1 mg/mL in group 3 (1:1000). Morphine consumption, verbal rating score of wound pain at rest and with exertion, and morphine-related side effects were investigated at 1, 2, 4 and 24 hours postoperatively. RESULTS: A total of 112 patients completed the study (37 in group 1, 36 in group 2, 39 in group 3). The incidence of nausea during the postoperative 4-24 hours was significantly lower in group 3 than in group 1 (23.1% vs. 56.8%, p < 0.05). Furthermore, the overall incidence of severe nausea was significantly lower in group 3 than in group 1 (2.6% vs. 24.3%, p < 0.05) as was the rescue antiemetic requirements (5.1% vs. 24.3%, p < 0.05). However, there were no significant differences between groups 2 and 1. The pain scores (at rest and with exertion) and 24-hour morphine consumption were not significantly different among the three groups. CONCLUSION: The antiemetic efficacy of ultralow-dose naloxone combined with PCA morphine is limited by a cut-off ratio of naloxone to morphine of 1:10,000.