Conversion and alloying type negative electrodes attracted huge attention in the present research on lithium/sodium-ion batteries (LIBs/SIBs) due to the high capacity delivered. Among these, SnO2 is ...investigated intensively in LIBs due to high cyclability, low reaction potential, cost-effectiveness, and environmental friendliness. Most of the LIB electrodes are explored in SIBs too due to expected similar electrochemical performance. Though several LIB negative electrode materials successfully worked in SIBs, bare SnO2 shows very poor electrochemical performance in SIB. The reason for this difference is investigated here through combined in operando and ex situ X-ray absorption spectroscopy (XAS). For this, the electrodes of SnO2 (space group P42/mnm synthesized via one-pot hydrothermal method) were cycled in Na-ion and Li-ion half-cells. The Na/SnO2 half-cell delivered a much lower discharge capacity than the Li/SnO2 half-cell. In addition, higher irreversibility was observed for Na/SnO2 half-cell during electrochemical investigations compared to that for Li/SnO2 half-cell. In operando XAS investigations on the Na/SnO2 half-cell confirms incomplete conversion and alloying reactions in the Na/SnO2 half-cell, resulting in poor electrochemical performance. The difference in the lithiation and sodiation mechanisms of SnO2 is discussed in detail.
Introduction/Aims
Very few studies analyzing the pattern of muscle involvement in magnetic resonance imaging (MRI) of patients with McArdle disease have been reported to date. We aimed to examine the ...pattern of muscle fat replacement in patients with McArdle disease.
Methods
We performed a retrospective study including all patients with genetically confirmed McArdle disease followed in our center from January 2010 to March 2021. Clinical data were collected from the medical record. Whole‐body MRI was performed as part of the diagnostic evaluation. The distribution of muscle fat replacement and its severity were analyzed.
Results
Nine patients were included. Median age at onset was 7 y (range, 5–58) and median age at the time when MRI was performed was 57.3 y (range, 37.2–72.8). At physical examination, four patients had permanent weakness: in three the weakness was limited to paraspinal muscles, whereas in one the weakness involved the paraspinal and proximal upper limb muscles. Muscle MRI showed abnormalities in six of the seven studied patients. In all of them, fat replacement of paravertebral muscles was found. Other muscles frequently affected were the tongue in three, subscapularis in three, and long head of biceps femoris and semimembranosus in two.
Discussion
Our findings suggest that paraspinal muscle involvement is common in McArdle disease and support the need to include this disease in the differential diagnosis of the causes of paraspinal muscle weakness. Involvement of the tongue and subscapularis are also frequent in McArdle disease.
Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron disease (MND) that shares a common clinical, genetic and pathologic spectrum with frontotemporal dementia (FTD). It is highly ...heterogeneous in its presentation and features. Up to 50% of patients with MND develop cognitive-behavioural symptoms during the course of the disease, meeting criteria for FTD in 10-15% of cases. In the absence of a precise biomarker, neuropathology is still a valuable tool to understand disease nosology, reach a definite diagnostic confirmation and help define specific subgroups of patients with common phenotypic, genetic and biomarker profiles. However, few neuropathological series have been published, and the frequency of FTLD in MND is difficult to estimate. In this work we describe a large clinicopathologic series of MND, analysing the frequency of concurrent FTLD changes and trying to define specific subgroups of patients based on their clinical, genetic and pathological characteristics. We performed an observational, retrospective, multi-centre case study. We included all cases meeting neuropathological criteria for MND from the Neurological Tissue Bank of the FRCB-IDIBAPS-Hospital Clínic Barcelona Biobank between 1994 and 2022, regardless of their last clinical diagnosis. While brain donation is encouraged in all patients, it is performed in very few, and representativeness of the cohort might not be precise for all patients with MND. We retrospectively reviewed clinical and neuropathological data, and describe the main clinical, genetic and pathogenic features, comparing neuropathologic groups between MND with and without FTLD changes and aiming to define specific subgroups. We included brain samples from 124 patients, 44 of whom (35.5%) had FTLD neuropathologic features (i.e. FTLD-MND). Pathologic TDP-43 aggregates were present in 93.6% of the cohort and were more extensive (higher Brettschneider stage) in those with concurrent FTLD (p < 0.001). Motor symptom onset was more frequent in the bulbar region in FTLD-MND cases than in those with isolated MND (p = 0.023), with no differences in survival. We observed a better clinicopathological correlation in the MND group than in the FTLD-MND group (93.8% vs 61.4%; p < 0.001). Pathogenic genetic variants were more common in the FTLD-MND group, especially C9orf72. We describe a frequency of FTLD of 35.5% in our series of neuropathologically confirmed cases of MND. The FTLD-MND spectrum is highly heterogeneous in all aspects, especially in patients with FTLD, in whom it is particularly difficult to define specific subgroups. In the absence of definite biomarkers, neuropathology remains a valuable tool for a definite diagnosis, increasing our knowledge in disease nosology.
•Rituximab is effective in patients with myasthenia gravis.•Patients with Anti-MusK antibodies respond better.•Severe infections may appear after rituximab treatment.•Hypogammaglobulinemia could ...influence in the risk of infection after rituximab.
The aim of this study is to evaluate the long-term efficacy, safety, and impact on immunoglobulin G (IgG) levels of rituximab in patients with myasthenia gravis (MG). A retrospective, observational study of drug-refractory MG patients treated with rituximab was done. The MG Foundation of America postintervention status (MGFA-PIS) was used to evaluate clinical response. Serum IgG levels were determined at baseline and post-treatment. Hypogammaglobulinemia was defined as IgG<7g/L. Thirty patients were included, 12 with anti-MuSK and 18 with anti-AChR antibodies. Mean (SD) follow-up was 85.5 (48) months. All 12 MuSK+ patients but only six (33%) AChR+ patients achieved minimal manifestations or remission (p<0.01). Nine severe infections were observed in five patients (17%). One patient was diagnosed with progressive multifocal leukoencephalopathy. At baseline, two patients (2/24; 8%) had hypogammaglobulinemia. During follow-up, hypogammaglobulinemia was observed in 60% (3/5) of patients who developed an infection and in 33% (7/21) who did not. Two of these patients died of infection-related complications. This study supports the effectiveness of rituximab in patients with MG, especially those with anti-MuSK antibodies. Severe infections may appear after rituximab treatment and hypogammaglobulinemia might play a role on it. A standard protocol would be needed to closely monitor IgG levels in MG patients treated with rituximab.
•NEM type 10 is caused by bi-allelic mutations in the LMOD3 gene.•NEM type 10 is usually characterized by a severe congenital nemaline myopathy.•We report two adult patients with mild NM, due to a ...new missense variant in LMOD3.
Nemaline myopathy (NEM) type 10, caused by biallelic mutations in LMOD3, is a severe congenital myopathy clinically characterized by generalized hypotonia and muscle weakness, respiratory insufficiency, joint contractures, and bulbar weakness. Here, we describe a family with two adult patients presenting mild nemaline myopathy due to a novel homozygous missense variant in LMOD3. Both patients presented mild delayed motor milestones, frequent falls during infancy, prominent facial weakness and mild muscle weakness in the four limbs. Muscle biopsy showed mild myopathic changes and small nemaline bodies in a few fibers. A neuromuscular gene panel revealed a homozygous missense variant in LMOD3 that co-segregated with the disease in the family (NM_198271.4: c.1030C>T; p.Arg344Trp). The patients described here provide evidence of the phenotype-genotype correlation, suggesting that non-truncating variants in LMOD3 lead to milder phenotypes of NEM type 10.
COVID-19 vaccines have proven safe and efficacious in reducing severe illness and death. Cuban protein subunit vaccine Abdala has shown safety, tolerability and efficacy (92·3% 95% CI: 85·7‒95·8) ...against SARS-CoV-2 in clinical trials. This study aimed to estimate Abdala's real-world vaccine effectiveness (VE).
This retrospective cohort study in Havana analyzed Cuban Ministry of Public Health databases (May 12-August 31, 2021) to assess VE in preventing severe illness and death from COVID-19 (primary outcomes). Cox models accounting for time-varying vaccination status and adjusting by demographics were used to estimate hazard ratios. A subgroup analysis by age group and a sensitivity analysis including a subgroup of tested persons (qRT-PCR) were conducted. Daily cases and deaths were modelled accounting for different VE.
The study included 1 355 638 persons (Mean age: 49·5 years SD: 18·2; 704 932 female 52·0%; ethnicity data unavailable): 1 324 vaccinated (partially/fully) and 31 433 unvaccinated. Estimated VE against severe illness was 93·3% (95% CI: 92·1-94·3) in partially- vaccinated and 98·2% (95% CI: 97·9-98·5) in fully-vaccinated and against death was 94·1% (95% CI: 92·5-95·4) in partially-vaccinated and 98·7% (95% CI: 98·3-99·0) in fully-vaccinated. VE exceeded 92·0% in all age groups. Daily cases and deaths during the study period corresponded to a VE above 90%, as predicted by models.
The Cuban Abdala protein subunit vaccine was highly effective in preventing severe illness and death from COVID-19 under real-life conditions.
Cuban Ministry of Public Health. Genetic Engineering and Biotechnology Centre.
The butyric acid fermentation, responsible for late blowing of cheese, is caused by the outgrowth in cheese of some species of Clostridium, resulting in texture and flavor defects and economical ...losses. The aim of this study was to evaluate the effectiveness of different antimicrobial compounds against vegetative cells and spores of C. tyrobutyricum, C. butyricum, C. beijerinckii and C. sporogenes strains isolated from cheeses with late blowing defect. Minimal inhibitory concentration (MIC) for reuterin, nisin, lysozyme and sodium nitrite were determined against Clostridium strains in milk and modified RCM (mRCM) after 7d exposure. Although the sensitivity of Clostridium to the tested antimicrobials was strain-dependent, C. sporogenes and C. beijerinckii generally had higher MIC values than the rest of Clostridium species. The majority of Clostridium strains were more resistant to antimicrobials in milk than in mRCM, and vegetative cells exhibited higher sensitivity than spores. Reuterin (MIC values 0.51–32.5mM) and nisin (MIC values 0.05–12.5μg/ml) were able to inhibit the growth of vegetative cells and spores of all assayed Clostridium strains in milk and mRCM. Strains of C. tyrobutyricum exhibited the highest sensitivity to lysozyme (MIC values<0.20–400μg/ml) and sodium nitrite (MIC values 18.75–150μg/ml). These results suggest that reuterin and nisin, with a broad inhibitory activity spectrum against Clostridium spp. spores and vegetative cells, may be the best options to control Clostridium growth in dairy products and to prevent associated spoilage, such as late blowing defect of cheese. However, further studies in cheese would be necessary to validate this hypothesis.
•Clostridium sensitivity to antimicrobials was strain dependent and varied with the type of bacteria cell and growth media.•Reuterin and nisin, with the broadest anti-clostridial spectrum, inhibited all Clostridium strains.•Sodium nitrite and lysozyme had limited inhibitory effect against Clostridium strains.
Adjunct cultures originating from artisanal cheese environments may play an important role in recreating and developing traditional cheese flavours, thanks to their enzymatic activities, involved in ...different metabolic pathways that occur during cheese ripening. In this work, Ligilactobacillus salivarius SP36, a strain isolated from an old cheese seal, was added as an adjunct culture to the cheese’s raw milk, and its effect on the microbiological, physical–chemical and sensory characteristics of the cheese was studied. The use of L. salivarius SP36 in cheese manufacturing had no significant (p > 0.05) effect on the cheese microbiota, gross composition (fat percentage, protein, total solids, moisture and NaCl concentration), colour or texture of the cheese. However, L. salivarius SP36 increased (p < 0.01) the formation of 25 volatile compounds, including 10 esters, 1 aldehyde, 8 alcohols and 6 carboxylic acids. In addition, cheeses made with L. salivarius SP36 received higher scores (p < 0.01) for aroma intensity and quality than control cheeses. L. salivarius SP36 proved to be a good candidate as an adjunct culture for cheesemaking, since it improved the cheese flavour by making it more intense and recovering typical sensorial notes of traditional cheeses.
BACKGROUND AND OBJECTIVESEvidence regarding the safety and efficacy of messenger RNA (mRNA) vaccines in patients with myasthenia gravis (MG) after immunosuppressive therapies is scarce. Our aim is to ...determine whether the mRNA-1273 vaccine is safe and able to induce humoral and cellular responses in patients with MG. METHODSWe performed an observational, longitudinal, prospective study including 100 patients with MG of a referral center for MG in our country, conducted from April 2021 to November 2021 during the vaccination campaign. The mRNA-1273 vaccine was scheduled for all participants. Blood samples were collected before vaccination and 3 months after a second dose. Clinical changes in MG were measured using the MG activities of daily life score at baseline and 1 week after the first and second doses. A surveillance of all symptoms of coronavirus disease 2019 (COVID-19) was conducted throughout the study. Humoral and cellular immune responses after vaccination were assessed using a spike-antibody ELISA and interferon gamma release assay in plasma. The primary outcomes were clinically significant changes in MG symptoms after vaccination, adverse events (AEs), and seroconversion and T-cell immune response rates. RESULTSNinety-nine patients completed the full vaccination schedule, and 98 had 2 blood samples taken. A statistically significant worsening of symptoms was identified after the first and second doses of the mRNA-1273 vaccine, but this was not clinically relevant. Mild AEs occurred in 14 patients after the first dose and in 21 patients after the second dose. Eighty-seven patients developed a humoral response and 72 patients showed a T-cell response after vaccination. A combined therapy with prednisone and other immunosuppressive drugs correlated with a lower seroconversion ratio (OR = 5.97, 95% CI 1.46-24.09, p = 0.015) and a lower T-cell response ratio (OR = 2.83, 95% CI 1.13-7.13, p = 0.024). DISCUSSIONOur findings indicate that the mRNA vaccination against COVID-19 is safe in patients with MG and show no negative impact on the disease course. Patients achieved high humoral and cellular immune response levels. CLASSIFICATION OF EVIDENCEThis study provides Class IV evidence that patients with MG receiving the mRNA-1273 vaccine did not show clinical worsening after vaccination and that most of the patients achieved high cellular or immune response levels.
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