Espondiloencondrodisplasia con desregulación inmune relacionada a ACP5. Reporte de cuatro casos / Spondyloenchondrodysplasia with immune dysregulation related to ACP5. A report of 4 cases

La espondiloencondrodisplasia con desregulación inmune relacionada a ACP5 (SPENCDI #607944, por la sigla de spondyloenchondrodysplasia with immune dysregulation y el número que le corresponde en OMIM, Online Mendelian Inheritance in Man) es una displasia inmuno-ósea poco frecuente con manifestaciones heterogéneas y gravedad variable. Presenta lesiones espondilometafisarias, disfunción inmune y compromiso neurológico. Se reportan aspectos clínicos, radiológicos y genéticos de cuatro niñas con SPENCDI en un hospital pediátrico. Todas presentaron manifestaciones esqueléticas y tres de ellas enfermedad inmunológica grave. Se encontró en tres pacientes la variante probablemente patogénica c.791T>A; p.Met264Lys en homocigosis, y en una paciente las variantes c.791T>A; p.Met264Lys y c.632T>C; p.lle211Thr (variante de significado incierto con predicción patogénica según algoritmos bioinformáticos) en heterocigosis compuesta en ACP5. La presencia de la variante repetida c.791T>A sugiere la posibilidad de un ancestro en común en nuestra población. El reconocimiento y diagnóstico de esta entidad es importante para lograr un oportuno abordaje, que deberá ser multidisciplinario, orientado hacia la prevención de posibles complicaciones.

Spondyloenchondrodysplasia with immune dysregulation related to ACP5 (SPENCDI, OMIM number 607944) is an uncommon immune-skeletal dysplasia with heterogeneous manifestations and variable severity. It is characterized by spondylar and metaphyseal lesions, immune dysfunction, and neurological involvement. Here we report the clinical, radiological and genetic aspects of 4 girls with SPENCDI treated at a children's hospital. They all had skeletal manifestations and 3 developed severe immune disease. In 3 patients, the likely pathogenic variant c.791T>A; p.Met264Lys (homozygous mutation) was observed, while 1 patient had variants c.791T>A; p.Met264Lys and c.632T>C; p.lle211Thr (variant of uncertain significance with pathogenic prediction based on bioinformatics algorithms) caused by a compound heterozygous mutation in ACP5. The repeated presence of variant c.791T>A suggests the possibility of a common ancestor in our population. The recognition and diagnosis of this disorder is important to achieve a timely approach, which should be multidisciplinary and aimed at preventing possible complications.

Hepatic and skeletal muscle tissue immunometabolism in trained rats / Inmunometabolismo del tejido hepático y muscular esquelético de ratas entrenadas / Imunometabolismo tecidual hepático e muscular esquelético de ratos treinados

ABSTRACT Introduction: Physical exercise can be an alternative for preventing and treating the harmful effects of obesity, mainly inflammatory effects on skeletal muscle and liver tissues. However, no consensus exists regarding this purpose's best physical training model. Objective: Evaluate morphological, metabolic, and inflammatory alterations in rats' skeletal and hepatic muscle tissues caused by aerobic and resistance training. Methods: 24 Wistar rats were divided into sedentary (S), aerobic (AE), and resistance training (R) groups. Blood glucose, total cholesterol, and serum triglycerides were measured periodically. After euthanasia, body mass was measured to calculate the total mass gain during the experiment. High-density lipoprotein (HDL) was measured. Adipose tissue was extracted to calculate its percentage relative to body mass and the liver, soleus, and gastrocnemius muscles for morphological analyses and concentrations of glycogen, lipids, and Tumor Necrosis Factor α (TNF-α). The Kruskall-Wallis test and Dunn's post-test were performed for statistical analysis, adopting p<0.05. Results: Both training models reduced the percentage of adipose tissue, body mass gain, and hepatic TNF-α concentration (p<0.05). AE increased serum HDL, gastrocnemius fiber diameter and reduced the fractal dimension in the soleus (p<0.05). R reduced blood glucose and serum and liver lipids, increased liver and soleus glycogen concentrations, increased gastrocnemius fiber diameter, and decreased TNF-α (p<0.05). Conclusion: Both training models reduced body mass, relative visceral adipose tissue, serum total cholesterol concentration, and liver inflammation. However, resistance training was more effective in promoting metabolic effects in the liver and skeletal muscle and reducing muscle inflammation in rats. Level of Evidence V; Expert Opinion.

RESUMEN Introducción: El ejercicio físico puede ser una alternativa para prevenir y tratar los efectos nocivos de la obesidad, principalmente los efectos inflamatorios sobre los tejidos del músculo esquelético y del hígado. Sin embargo, no existe consenso sobre cuál es el mejor modelo de entrenamiento físico para este fin. Objetivo: Evaluar las alteraciones morfológicas, metabólicas e inflamatorias del entrenamiento aeróbico y de resistencia en sobre los tejidos músculo esqueléticos y hepáticos de ratas. Métodos: 24 ratas Wistar se dividieron en grupos sedentarios (S), aeróbicos (AE) y de entrenamiento de resistencia (R). Se midieron periódicamente glucosa en sangre, colesterol total y triglicéridos. Después de la eutanasia, se midió la masa corporal para calcular la ganancia de masa total durante el experimento. Se midió la lipoproteína de alta densidad (HDL). Se extrajo tejido adiposo para calcular su porcentaje relativo a la masa corporal, así como hígado, músculos sóleo y gastrocnemio para análisis morfológicos y concentraciones de glucógeno, lípidos y Factor de Necrosis Tumoral α (TNF-α). Para el análisis estadístico fueron utilizados Kruskall-Wallis y el post-test de Dunn, adoptando p<0,05. Resultados: Ambos entrenamientos redujeron el porcentaje de tejido adiposo, masa corporal y la concentración de TNF-α hepático (p<0,05). AE aumentó el HDL sérico, el diámetro de la fibra del gastrocnemio y redujo la dimensión fractal en el sóleo (p<0,05). R redujo la glucosa en sangre y los lípidos séricos y hepáticos, aumentó las concentraciones de glucógeno hepático y sóleo, aumentó el diámetro de la fibra del gastrocnemio y disminuyó el TNF-α (p<0,05). Conclusión: Ambos modelos de entrenamiento redujeron la masa corporal, el tejido adiposo visceral relativo, la concentración sérica de colesterol total y la inflamación hepática. El entrenamiento de resistencia demostró ser más eficaz para promover los efectos metabólicos en el hígado y el músculo esquelético, además de reducir la inflamación muscular en ratas. Nivel de Evidencia V; Opinión del Especialista.

RESUMO Introdução: O exercício físico pode se apresentar como uma alternativa para prevenção e tratamento de efeitos deletérios da obesidade, principalmente efeitos inflamatórios sobre os tecidos muscular esquelético e hepático. No entanto, não há consenso quanto ao melhor modelo de treinamento físico para tal finalidade. Objetivos: Avaliar alterações morfológicas, metabólicas e inflamatórias dos treinamentos aeróbico e resistido sobre os tecidos muscular esquelético e hepático de ratos. Métodos: 24 ratos Wistar foram divididos nos grupos sedentário (S), treinamento aeróbico (AE) e resistido (R). Glicemia, colesterol total e triglicerídeos séricos foram mensurados periodicamente. Após a eutanásia, a massa corporal foi mensurada para calcular o ganho total de massa durante o experimento. A lipoproteína de alta densidade (HDL) foi dosada. O tecido adiposo foi extraído para cálculo de sua porcentagem relativa à massa corporal assim como o fígado e os músculos sóleo e gastrocnêmio para as análises morfológicas e das concentrações de glicogênio, lipídios e Fator de Necrose Tumoral α (TNF-α). Para análise estatística, foram utilizados o teste de Kruskall-Wallis e o pós-teste de Dunn, adotando-se p<0,05. Resultados: Ambos os modelos de treinamento reduziram o percentual de tecido adiposo, ganho de massa corporal e concentração hepática de TNF-α (p<0,05). AE aumentou o HDL sérico, o diâmetro das fibras do gastrocnêmio e reduziu a dimensão fractal no sóleo (p<0,05). R reduziu a glicemia e os lipídios séricos e hepáticos, aumentou a concentração de glicogênio hepático e sóleo, aumentou o diâmetro das fibras gastrocnêmicas e diminuiu o TNF-α (p<0,05). Conclusão: Ambos os modelos de treinamento reduziram a massa corporal, o tecido adiposo visceral relativo, a concentração sérica de colesterol total e a inflamação hepática. No entanto, o treinamento resistido mostrou-se mais eficaz em promover efeitos metabólicos no fígado e no músculo esquelético, além de reduzir a inflamação muscular em ratos. Nível de Evidência V; Opinião do Especialista.

Changes related to muscle autophagy after exhaustive exercise and blunt trauma / Cambios relacionados con la autofagia muscular tras ejercicio exhaustivo y traumatismo contuso / Alterações relacionadas à autofagia muscular após exercício exaustivo e trauma contuso

ABSTRACT Objective: To study the temporal changes of autophagy related factors in skeletal muscle of rats after exhaustive exercise and blunt trauma. Methods: Forty-two male SD rats were divided into 7 groups with 6 rats in each group: Quiet control group (C), immediately after exhaustive exercise (E0), 24 hours after exhaustive exercise (E24), 48 hours after exhaustive exercise (E48), immediately after blunt trauma (D0), 24 hours after blunt trauma (D24), 48 hours after blunt trauma (D48). All groups of rats were killed and samped respectively at different time points specified above, and the right gastrocnemius muscle was taken, which was divided into two parts, one for mRNAs of, Lamp-2, BNIP3 and NIX by real-time fluorescent quantitative PCR, and the other for p62 protein by Western blotting. Results: (1) Compared with group C, mRNA levels of p62, Lamp-2 and NIX in group E48 were significantly increased after exhaustive exercise(P<0.05), suggesting that autophagy increased in 48h after exhaustive exercise. (2) Compared with group C, p62mRNA and Lamp-2 mRNA levels were significantly increased immediately after blunt trauma(P<0.05) and decreased significantly in 48h after blunt trauma(P<0.05), suggesting that autophagy activity was enhanced immediately after blunt trauma and decreased in 48h after injury. Conclusions: Generally, there were differences at each recovery phase between blunt trauma and exhausted exercise models, and the basal autophagy factors and mitochondrial autophagy factors were also inconsistent. Basal autophagy factors p62 and Lamp-2 increased significantly 48 hours after eccentric exhaustive exercise and immediately after blunt trauma. Mitochondrial autophagy factor BNIP3 did not increase after exhaustive exercise and blunt trauma, but NIX only increased after exhaustive exercise. Its molecular mechanism needs to be further studied. Level of Evidence III; Therapeutic Studies Investigating the Results of Treatment.

RESUMEN Objetivo: Estudiar los cambios temporales de los factores relacionados con la autofagia en el músculo esquelético de ratas tras el ejercicio exhaustivo y el traumatismo contuso. Métodos: Se dividieron 42 ratas SD macho en 7 grupos con 6 ratas en cada grupo: grupo de control silencioso (C), inmediatamente después del ejercicio exhaustivo (E0), 24 horas después del ejercicio exhaustivo (E24), 48 horas después del ejercicio exhaustivo (E48), inmediatamente después de un traumatismo contuso (D0), 24 horas después de un traumatismo contuso (D24), 48 horas después de un traumatismo contuso (D48). Todos los grupos de ratas fueron sacrificados y rotulados, respectivamente, en diferentes momentos especificados anteriormente, y se extrajo el músculo gastrocnemio derecho, dividido en dos partes, una para los ARNm Lamp-2, BNIP3 y NIX mediante PCR cuantitativa fluorescente en tiempo real, y la otra para la proteína p62 mediante Western blotting. Resultados: (1) En comparación con el grupo C, los niveles de ARNm de p62, Lamp-2 y NIX en el grupo E48 aumentaron significativamente tras el ejercicio exhaustivo (P<0,05), lo que sugiere que la autofagia aumentó en las 48 horas posteriores al ejercicio exhaustivo. (2) En comparación con el grupo C, los niveles de ARNm de p62 ARNm y Lamp-2 aumentaron significativamente inmediatamente después del traumatismo contuso (P<0,05) y disminuyeron significativamente a las 48 horas después del traumatismo contuso (P<0,05), lo que sugiere que la actividad de autofagia aumentó inmediatamente después del traumatismo contuso y disminuyó a las 48 horas después de la lesión. Conclusión: En general, hubo diferencias en cada fase de recuperación entre los modelos de traumatismo contuso y ejercicio exhaustivo, y los factores de autofagia basal y los factores de autofagia mitocondrial también fueron inconsistentes. Los factores de autofagia basal p62 y Lamp-2 aumentaron significativamente 48 horas después del ejercicio excéntrico exhaustivo e inmediatamente después del traumatismo contuso. El factor de autofagia mitocondrial BNIP3 no aumentó tras el ejercicio exhaustivo y el traumatismo contuso, pero NIX sólo aumentó tras el ejercicio exhaustivo. Su mecanismo molecular debe investigarse con más detalle. Nivel de Evidencia III; Estudios Terapéuticos que Investigan los Resultados del Tratamiento.

RESUMO Objetivo: Estudar as alterações temporais dos fatores relacionados à autofagia no músculo esquelético de ratos após exercício exaustivo e trauma contuso. Métodos: Quarenta e dois ratos machos SD foram divididos em 7 grupos com 6 ratos em cada grupo: Grupo de controle silencioso (C), imediatamente após o exercício exaustivo (E0), 24 horas após o exercício exaustivo (E24), 48 horas após o exercício exaustivo (E48), imediatamente após o trauma contuso (D0), 24 horas após o trauma contuso (D24), 48 horas após o trauma contuso (D48). Todos os grupos de ratos foram mortos e rotulados, respectivamente, em diferentes momentos especificados acima, e o músculo gastrocnêmio direito foi retirado, dividido em duas partes, uma para mRNAs de Lamp-2, BNIP3 e NIX por PCR quantitativo fluorescente em tempo real, e a outra para a proteína p62 por imunotransferência. Resultados: (1) Em comparação com o grupo C, os níveis de mRNA de p62, Lamp-2 e NIX no grupo E48 aumentaram significativamente após o exercício exaustivo (P<0,05), sugerindo que a autofagia aumentou em 48 horas após o exercício exaustivo. (2) Em comparação com o grupo C, os níveis de mRNA de p62mRNA e Lamp-2 foram significativamente aumentados imediatamente após o trauma contuso (P<0,05) e diminuíram significativamente em 48 horas após o trauma contuso (P<0,05), sugerindo que a atividade de autofagia foi aumentada imediatamente após o trauma contuso e diminuiu em 48 horas após a lesão. Conclusão: Houve, via de regra, diferenças em cada fase de recuperação entre os modelos de trauma contuso e de exercício exaustivo, sendo que os fatores de autofagia basal e os fatores de autofagia mitocondrial também foram inconsistentes. Os fatores de autofagia basal p62 e Lamp-2 aumentaram significativamente 48 horas após o exercício excêntrico exaustivo e imediatamente após o trauma contuso. O fator de autofagia mitocondrial BNIP3 não aumentou após o exercício exaustivo e o trauma contuso, mas o NIX aumentou somente após o exercício exaustivo. Seu mecanismo molecular precisa ser investigado com mais detalhes. Nível de Evidência III; Estudos Terapêuticos que Investigam os Resultados do Tratamento.

Ângulo de fase como preditor de massa muscular em pacientes com doença inflamatória intestinal / Phase angle as a predictor of muscle mass in patients with inflammatory bowel disease

ABSTRACT Malnutrition/sarcopenia is frequent in patients with inflammatory bowel diseases (IBD), and results in muscle catabolism, impacting treatment response, postoperative complications, and quality of life. Objective: This study aims to assess whether the phase angle (PhA) is a parameter for predicting reduced muscle mass in patients with IBD. Methods: Adult patients with IBD were included in this cross-sectional study. For the estimation of muscle mass and the calculation of the PhA, we used bioelectrical impedance analysis (BIA). Crohn's disease (CD) and ulcerative colitis (UC) activity scores were defined using the Harvey-Bradshaw index and partial Mayo score, respectively. The area under the ROC curve was calculated to identify the PhA cut-off point for reduced muscle mass. Results: The sample consisted of 145 patients, with 39 (26.9%) with IBD in the active phase. There was a correlation of the PhA with skeletal muscle mass (SMM) (rs 0.35, P<0.001) and with the skeletal muscle mass index (SMI) (rs 0.427, P<0.001), and the associations remained in the most active form (moderate or severe) of IBD. The ROC curve analysis indicated that the cut-offs points of the PhA ≤5.042 for female and PhA ≤6.079 for male can be used to predict muscle mass reduction. Conclusion: The PhA can be considered a predictor of muscle mass reduction in IBD patients, and we can use it for screening and monitoring the evolution of malnutrition.

RESUMO A desnutrição/sarcopenia é frequente em pacientes com doenças inflamatórias intestinais (DII), resultando em catabolismo muscular, com impacto nas respostas aos tratamentos, complicações cirúrgicas e na qualidade de vida. Objetivo: Este estudo tem como objetivo, avaliar se o ângulo de fase (AF) é um parâmetro para a predição de redução de massa muscular em pacientes com DII. Métodos: Pacientes adultos com DII foram incluídos neste estudo transversal. A estimativa da massa muscular e o cálculo do AF foram realizados a partir do exame de bioimpedância elétrica (BIA). As atividades da doença de Crohn e retocolite ulcerativa foram definidas pelo índice Harvey-Bradshaw e escore parcial de Mayo, respectivamente. A área de curva ROC foi calculada para identificar o ponto de corte do AF para a massa muscular reduzida. Resultados: A amostra foi composta por 145 pacientes, sendo 39 (26.9%) com DII em fase ativa. Houve correlação do AF com massa muscular esquelética (MME) (rs 0.35, P<0.001) e com o índice de massa muscular esquelética (IMME) (rs 0.427, P<0.001), mantendo-se as associações na forma mais ativa (moderada ou grave) da DII. A análise da curva ROC indicou que os pontos de corte de AF ≤5.042 para mulheres e ≤6.079 para homens podem ser usados para prever a redução da massa muscular. Conclusão: O AF pode ser considerado um preditor de redução de massa muscular nos pacientes com DII e ser utilizado para triagem e acompanhamento da evolução da desnutrição.

Relationship between skeletal muscle mass loss and metabolic dysfunction-associated fatty liver disease among Chinese patients with metabolic dysregulation

SUMMARY OBJECTIVE: The aim of this study was to explore the correlation between skeletal muscle content and the presence and severity of metabolic dysfunction-associated fatty liver disease in patients with metabolic dysregulation in China. METHODS: A cross-sectional study was conducted among patients from the endocrinology outpatient department at Ningbo First Hospital, in Ningbo, China, in April 2021. Adult patients with metabolic dysregulation who accepted FibroScan ultrasound were included in the study. However, those without clinical data on skeletal muscle mass were excluded. FibroScan ultrasound was used to noninvasively evaluate metabolic dysfunction-associated fatty liver disease. The controlled attenuation parameter was used as an evaluation index for the severity of liver steatosis. Bioelectrical impedance analysis was used to measure the skeletal muscle index. RESULTS: A total of 153 eligible patients with complete data were included in the final analysis. As the grading of liver steatosis intensifies, skeletal muscle index decreases (men: Ptrend<0.001, women: Ptrend=0.001), while body mass index, blood pressure, blood lipid, uric acid, aminotransferase, and homeostatic model assessment of insulin resistance increase (Ptrend<0.01). After adjusting for confounding factors, a negative association between skeletal muscle index and the presence of metabolic dysfunction-associated fatty liver disease was observed in men (OR=0.691, p=0.027) and women (OR=0.614, p=0.022). According to the receiver operating characteristic curve, the best cutoff values of skeletal muscle index for predicting the metabolic dysfunction-associated fatty liver disease presence were 40.37% for men (sensitivity, 87.5%; specificity, 61.5%) and 33.95% for women (sensitivity, 78.6%; specificity, 63.8%). CONCLUSION: Skeletal muscle mass loss among patients with metabolic dysregulation was positively associated with metabolic dysfunction-associated fatty liver disease severity in both sexes. The skeletal muscle index cutoff value could be used to predict metabolic dysfunction-associated fatty liver disease.

Advances of pathogenesis and drug development in amyotrophic lateral sclerosis / 中国药理学通报

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting both upper and lower motor neurons. ALS patients develop progressive muscle atrophy, muscle weak and paralysis, finally died of respiratory failure. ALS is characterized by fast aggression and high mortality. What' s more, the disease is highly heterogeneous with unclear pathogenesis and lacks effective drugs for therapy. In this review, we summarize the main pathological mechanisms and the current drugs under development for ALS, which may provide a reference for the drug discovery in the future.

Association between skeletal muscle mass and metabolic syndrome in middle-aged and elderly community residents / 上海预防医学

ObjectiveTo explore the correlation between skeletal muscle mass and metabolic syndrome （MS） disease risk among middle-aged and elderly community residents in Urumqi， and to provide a theoretical basis for understanding the relationship between skeletal muscle mass and MS among middle-aged and elderly community residents in China. MethodsA total of 1 438 community residents ≥ 50 years old were selected as the research subjects from July 2018 to January 2019 in Urumqi. They were selected from a multi-ethnic natural population cohort in Xinjiang. Data were collected through questionnaires， physical examination， bioelectrical impedance analysis （BIA）， laboratory tests， etc. Skeletal muscle mass was evaluated using the limb skeletal muscle mass index （SMI） corrected for body weight； MS was defined as it at least includes three of the following： abdominal obesity， hypertension， hyperglycemia， high triglycerides and low high-density lipoprotein cholesterol. SMI was divided into four quantile arrays of Q1‒Q4. Trend χ2 test was applied to explore whether there was a correlation between SMI changes and MS. A multivariate logistic regression model was used to analyze whether there is a difference in the risk of MS between the higher SMI group （Q2， Q3， Q4） and the reference group Q1. ResultA total of 560 MS patients were detected in this study， with a prevalence rate of 38.94%. Among them， the prevalence rate of MS was 39.16% in males and 38.80% in females. The increase in male SMI grading level is not correlated with the prevalence of MS （trend P>0.05）； After adjusting for confounding factors （model 4）， the increase in SMI was still not related to the prevalence of MS （Ptrend=0.995）. There was no statistical difference in the risk of MS between the lowest quartile group Q1 and the highest quartile group Q4 （OR=1.01， 95%CI： 0.69‒1.78）. The prevalence of MS in women gradually decreased with the increase of SMI grading level （Ptrend<0.001）； After adjusting for confounding factors （model 4）， there was still a correlation between the increase of SMI and the prevalence of MS （Ptrend=0.005）. With the lowest quartile of SMI Q1 as the reference group， the risk of MS in Q2 （OR=0.63， 95%CI： 0.40‒1.00）， Q3 （OR=0.56， 95%CI： 0.34‒0.94）， Q4 （OR=0.42， 95%CI： 0.23‒0.76） decreased. ConclusionAn increase in skeletal muscle mass may be beneficial for preventing MS， especially among middle-aged and elderly female residents. Considering the intensification of aging in China and the close relationship between MS and related comorbidities， managing skeletal muscle mass may contribute to potential MS prevention.

Activation of Nrf2/HO-1/NQO1 Signaling Pathway by Shenqi Tangluo Pill Improves Oxidative Stress Injury of Skeletal Muscle of Type 2 Diabetes Mellitus Mice / 中国实验方剂学杂志

ObjectiveTo investigate the effect and mechanism of Shenqi Tangluo pill （SQTLP） on oxidative stress injury of skeletal muscle of type 2 diabetes mellitus （T2DM） mice based on nuclear factor erythroid 2-related factor 2 （Nrf2）/heme oxygenase-1 （HO-1）/NAD（P）H quinone oxidoreductase 1 （NQO1） pathway. MethodA total of 60 7-week-old male db/db mice ［specific pathogen-free （SPF） grade］ were selected and fed for one week for adaption. They were divided into the model control group， SQTLP low-， medium- and high-dose （19， 38， and 76 g·kg-1） groups and metformin group （0.26 g·kg-1） by gavage. Each group consisted of 12 mice. Twelve male db/m mice of the same age were selected as the blank group. The intervention was implemented continuously for 8 weeks. Fasting blood glucose （FBG） was detected. Fasting serum insulin （FINS） levels were detected by enzyme-linked immunosorbent assay （ELISA）， and the homeostasis model assessment-insulin resistance （HOMA-IR） index and the homeostasis model assessment-insulin sensitivity index （HOMA-ISI） were calculated. Oral glucose tolerance test （OGTT） and insulin tolerance test （ITT） were conducted. The activities of superoxide dismutase （SOD） and glutathione peroxidase （GSH-Px） and the contents of malondialdehyde （MDA） and reduced nicotinamide adenine dinucleotide phosphate （NADPH） in skeletal muscle tissues were detected by biochemical kits. Hematoxylin-eosin （HE） staining was used to observe the pathological changes in skeletal muscle tissues. The levels of reactive oxygen species （ROS） and 4-hydroxynonenal （4-HNE） in skeletal muscle tissue were detected by immunofluorescence （IF）. The expression levels of Nrf2， HO-1， NQO1 and glutamate-cysteine ligase catalytic subunit （GCLC） proteins in skeletal muscle tissues were detected by Western blot. ResultCompared with those in the blank group， FBG， FINS and HOMA-IR in the model group were significantly increased （P<0.05）， while HOMA-ISI was decreased （P<0.05）. The results of OGTT and ITT showed that blood glucose was significantly increased at all time points （P<0.05）， and glucose tolerance and insulin tolerance were significantly impaired. SOD and GSH-Px activities in skeletal muscle tissues were significantly decreased （P<0.05）， and MDA and NADPH contents were significantly increased （P<0.05）. In skeletal muscle tissues， the arrangement of muscle fibers was loose， the nucleus was disordered， and inflammatory cells were infiltrated. The expression levels of ROS and 4-HNE in skeletal muscle tissues were significantly increased （P<0.05）. The protein expression levels of Nrf2， HO-1， NQO1 and GCLC in skeletal muscle tissues were significantly decreased （P<0.05）. Compared with those in the model group， FBG， FINS and HOMA-IR in the metformin group were significantly decreased （P<0.05）， while HOMA-ISI was increased （P<0.05）. The results of OGTT and ITT showed that blood glucose in the metformin group was significantly decreased at all time points （P<0.05）. The activities of SOD and GSH-Px in skeletal muscle tissues were significantly increased （P<0.05）， while the contents of MDA and NADPH were significantly decreased （P<0.05）. No obvious abnormality was found in the skeletal muscle tissue of the metformin group. The expressions of ROS and 4-HNE in skeletal muscle tissues were decreased （P<0.05）. The protein expression levels of Nrf2， HO-1， NQO1 and GCLC in skeletal muscle tissues were significantly increased （P<0.05）. Compared with those in the model group， FBG， FINS and HOMA-IR in the SQTLP medium- and high-dose groups were significantly decreased （P<0.05）， while HOMA-ISI was increased （P<0.05）. The results of OGTT and ITT showed that the glucose tolerance and insulin tolerance of mice were improved in each dose group of SQTLP. The GSH-Px activity in the SQTLP low-dose group was significantly increased （P<0.05）， and the NADPH content was decreased （P<0.05）. The activities of SOD and GSH-Px in the SQTLP medium- and high-dose groups were significantly increased （P<0.05）， while the contents of MDA and NADPH were significantly decreased （P<0.05）. The skeletal muscle tissue injury of mice in each dose group of SQTLP was ameliorated to different degrees. In the SQTLP medium- and high-dose groups， the expressions of ROS and 4-HNE were decreased （P<0.05）， and the protein expression levels of Nrf2， HO-1， NQO1 and GCLC were significantly increased （P<0.05）. Compared with those in the SQTLP low-dose group， FBG and HOMA-IR in the SQTLP high-dose group were significantly decreased （P<0.05）， while HOMA-ISI was increased （P<0.05）. The results of OGTT and ITT showed that the SQTLP high-dose group significantly improved the glucose tolerance and insulin tolerance of mice. The activities of SOD and GSH-Px in skeletal muscle tissues were significantly increased （P<0.05）， while the contents of MDA and NADPH were significantly decreased （P<0.05）. No obvious abnormality was found in the skeletal muscle tissue， the expressions of ROS and 4-HNE were decreased （P<0.05）， and the protein expression levels of Nrf2， HO-1， NQO1 and GCLC were significantly increased （P<0.05） in the skeletal muscle tissue of the SQTLP high-dose group. ConclusionSQTLP can significantly improve IR in T2DM mice， and the mechanism is related to SQTLP activating the Nrf2/HO-1/NQO1 signaling pathway， promoting the expression of antioxidant enzymes， and thus improving the oxidative stress injury in the skeletal muscle.

Verma-Naumoff syndrome: a case report / Síndrome de Verma-Naumoff: a propósito de un caso

In skeletal dysplasias, there are short rib polydactyly syndromes, which traditionally differentiate into four lethal types. This report describes a case of Type III, which presented characteristics of Types I and II. A 38-year-old woman presented fetal growth restriction at 17 weeks and 6 days, decreased amniotic fluid, enlarged and hyperechogenic kidneys, and long bones below the 3rd percentile. Three weeks later, she developed anhydramnia. The couple did not consent to the performance of an invasive test for genetic diagnosis and chose to maintain the pregnancy. At 33 weeks, due to premature labor and interactivity, a cesarean section was performed, giving birth to a female baby, who died due to respiratory failure there were no vocal cords and no trachea visible at laryngoscopy. On physical examination, he had the phenotypic characteristics of the syndrome. An X-ray showed short ribs and severe pulmonary hypoplasia. After birth, the parents chose not to carry out a genetic study or an anatomical examination. Researchers have suggested that there is an intersection of the anatomical changes of the types. This case report supports this theory.

En las displasias esqueléticas, existen síndromes de polidactilia de costillas cortas, que tradicionalmente se diferencian en cuatro tipos letales. Este reporte describe un caso del tipo III, que presentó características de los tipos I y II. Mujer de 38 años con restricción del crecimiento fetal a las 17 semanas y 6 días, líquido amniótico disminuido, riñones agrandados e hiperecogénicos y huesos largos por debajo del percentil 3. Tres semanas después, desarrolló anhidramnia. La pareja no consintió en la realización de una prueba invasiva de diagnóstico genético y optó por mantener el embarazo. A las 33 semanas, debido al parto prematuro y la interactividad, se realizó una cesárea, dando a luz a un bebé, que murió debido a una insuficiencia respiratoria: no había cuerdas vocales ni tráquea visible en la laringoscopia. Al examen físico presentaba las características fenotípicas del síndrome. Una radiografía mostró costillas cortas e hipoplasia pulmonar severa. Después del nacimiento, los padres optaron por no realizar un estudio genético ni un examen anatómico. Los investigadores han sugerido que existe una intersección de los cambios anatómicos de los tipos. Este reporte de caso apoya esta teoría.

COVID-19 related to sarcopenia: Current perspectives on etiology, clinical implications, and nutritional rehabilitation

Sarcopenia is a progressive skeletal muscle disorder characterized by reduced strength and quality. Pathophysiological mechanisms, clinical aspects, and nutritional points were related to sarcopenia in COVID-19 found in skeletal muscle during and after the disease course, which corroborated the development of adverse events. Declining physical activity, insufficient protein intake, and worsened proinflammatory response have been shown to have negative consequences on muscle protein synthesis, potentiating the risk of acute sarcopenia. Obesity sarcopenia has also been shown to worsen the prognosis of patients with SARS-CoV-2. Nutritional rehabilitation is used to prevent or minimize the development of acute sarcopenia. Dietary recommendations include increased energy supply and protein intake of 1.2 to 2.0 g/kg of body weight. Evidence suggests that aging with sedentary behaviors, pathophysiological changes, and inflammation alter body composition. In addition, nutritional deficiencies are predictors and aggravators of acute sarcopenia in COVID-19.

Sarcopenia é um distúrbio progressivo do músculo esquelético caracterizado pela redução da força e qualidade. Mecanismos fisiopatológicos, aspectos clínicos e nutricionais foram relacionados à sarcopenia no COVID-19 encontrada no músculo esquelético, durante e após o curso da doença, o que corroborou para o desenvolvimento de eventos adversos. O declínio da atividade física, a ingestão insuficiente de proteínas e piora da resposta pró-inflamatória demonstraram ter consequências negativas na síntese de proteínas musculares, potencializando risco de sarcopenia. A obesidade sarcopênica também demonstrou piorar o prognóstico de pacientes infectados com SARS-CoV-2. A reabilitação nutricional pode prevenir ou minimizar o desenvolvimento de sarcopenia. As recomendações dietéticas incluem maior oferta de energia e maior ingestão de proteínas de 1,2 a 2,0 g/kg de peso corporal. Evidências sugerem que o envelhecimento com comportamentos sedentários, alterações fisiopatológicas e inflamação, alterações na composição corporal, deficiências nutricionais são preditores e agravantes da sarcopenia aguda na COVID-19.

Validity of the Maturation Stage Method in the Individual Assessment of Midpalatal Suture Ossification Before Maxillary Expansion: A Systematic Review / Validez del Método de la Etapa de Maduración en la Evaluación Individual de la Osificación de la Sutura Palatina Mediana Previo a la Expansión Maxilar: Una Revisión Sistemática

In 2013, midpalatal suture maturation stage assessment was proposed for the evaluation of patients before performing maxillary expansion. In this study, we aimed to analyze the association between the midpalatal suture maturation stages assessed by CBCT, according to the method described by Angelieri et al., and other objective methods used to assess skeletal maturation or bone fusion. A computerized database search was conducted using PubMed, Cochrane Library, SciELO, LILACS, Web of Science, and Scopus, without language restriction. Unpublished literature was searched on ClinicalTrials.gov, the National Research Register, and Pro-Quest Dissertation Abstracts and Thesis database. Authors were contacted when necessary, and reference lists of the included studies were screened. Search terms included midpalatal suture, maturation, correlation, diagnostic performance, classification, evaluation, assessment, and relationship. Quality assessment was performed using the Observational Cohort and Cross-Sectional Studies tool developed by the National Heart, Lung, and Blood Institute. Eleven studies met the inclusion criteria. Of all the studies included, 81.9% had fair qualit y and 18.1% good quality, respectively. Eight out of eleven studies assessed the correlation between the midpalatal suture maturation method and the skeletal maturity evaluated by CVM method (Spearman's correlation coefficient: 0.244-0.908). Two out of eleven studies evaluated the correlation between midpalatal suture maturation method and the skeletal maturity assessed by HWM method (Spearman's correlation coefficient: 0.904-0.905) Even though midpalatal suture maturation stage assessment needs an exhaustive training and calibration process, it is a valid method to evaluate skeletal maturation or bone fusion. From a clinical perspective, for patients at CS4, CS5 and CS6, an assessment of the midpalatal suture on CBCT is indicated. A similar assessment should be done in patients at SMI 7-9.

En 2013, se propuso un nuevo método para la evaluación del estadio de maduración de la sutura palatina mediana para la evaluación de los pacientes antes de realizar la expansión maxilar. En este estudio, nuestro objetivo fue analizar la asociación entre las etapas de maduración de la sutura palatina mediana evaluada en CBCT, según el método descrito por Angelieri et al., y otros métodos objetivos utilizados para evaluar la maduración esquelética o la fusión ósea. Se realizó una búsqueda en las bases de datos PubMed, Cochrane Library, SciELO, LILACS, Web of Science y Scopus, sin restricción de idioma. Se buscó literatura no publicada en ClinicalTrials.gov, el Registro Nacional de Investigación y la base de datos Pro-Quest Dissertation Abstracts and Thesis. Se estableció contacto con los autores cuando fue necesario y se revisaron las listas de referencias de los estudios incluidos. Los términos de búsqueda incluyeron sutura palatina mediana, maduración, correlación, rendimiento diagnóstico, clasificación, evaluación, valoración y relación. La evaluación de la calidad se realizó mediante la herramienta de Estudios transversales y de cohortes observacionales desarrollada por el Instituto Nacional del Corazón, los Pulmones y la Sangre. Once estudios cumplieron con los criterios de inclusión. Del total de estudios incluidos, el 81.9% tuvo calidad regular y el 18.1% calidad buena, respectivamente. Ocho de once estudios evaluaron la correlación entre el método de maduración de la sutura palatina mediana y la madurez esquelética evaluada por el método CVM (coeficiente de correlación de Spearman: 0.244-0.908). Dos de once estudios evaluaron la correlación entre el método de maduración de la sutura palatina mediana y la madurez esquelética evaluada por el método HWM (coeficiente de correlación de Spearman: 0.904-0.905). Aunque la evaluación del estado de maduración de la sutura palatina mediana necesita un proceso exhaustivo de entrenamiento y calibración, es un método válido para evaluar la maduración esquelética o la fusión ósea. Desde una perspectiva clínica, para pacientes en CS4, CS5 y CS6, está indicada una evaluación de la sutura palatina mediana en CBCT. Se debe realizar una evaluación similar en pacientes con SMI 7-9.

Explaining Skeletal System Anatomy with Classical Method, Video Assisted Method and 3D Imaging Techniques and Comparison of Learning Levels Between Methods

SUMMARY: The aim of this research is to introduce the ideal lecture technique to the literature by explaining the anatomy of the skeletal system using the classical method, video-assisted method and 3D imaging techniques. The research was carried out with 180 students. The number of samples was determined by power analysis (a=0.05,b=0.20, effect size=0.25). Participants were pre-screened and divided into 4 groups with the closest group mean (group 1: control group: the group that did not take anatomy lessons, group 2: video-assisted anatomy education, group 3: 3D anatomy course, group 4: classical anatomy education group). The courses in the training groups were organised as 4 hours/day, 2 days/week for 5 weeks. At the end of the course, the students were re-examined and scaled to determine the difference in scores and self-efficacy between the groups. A one-way ANOVA test was performed because the data were normally distributed when comparing between groups. The mean scores were calculated as group 1=30.22±6.24, group 2=39.02±9.15, group 3=49.77±9.20 and group 4=59.28±8.95. In the post hoc comparison, in pairwise comparisons between all groups, the differences were highly significant (pgroup 3>group 2>group 1 (p<0.001). According to the results of this study, the laboratory method in skeletal anatomy teaching is the best alternative to 3D anatomy teaching.

El objetivo de esta investigación es introducir la técnica de lectura ideal en la literatura, explicando la anatomía del sistema esquelético, utilizando el método clásico, el método asistido por video y las técnicas de imágenes en 3D. La investigación se llevó a cabo con 180 estudiantes. El número de muestras se determinó mediante análisis de potencia (a=0,05, b=0,20, tamaño del efecto=0,25). Los participantes fueron preseleccionados y divididos en 4 grupos con la media de grupo más cercana (grupo 1: grupo de control: el grupo que no tomó lecciones de anatomía, grupo 2: educación de anatomía asistida por video, grupo 3: curso de anatomía 3D, grupo 4: grupo de educación en anatomía clásica). Los cursos en los grupos de formación se organizaron con 4 horas/día, 2 días/semana durante 5 semanas. Al final del curso, los estudiantes fueron reexaminados y escalados para determinar la diferencia en puntajes y autoeficacia entre los grupos. Se realizó una prueba de ANOVA de una vía debido a que los datos se distribuyeron normalmente al comparar entre grupos. Las puntuaciones medias se calcularon como grupo 1=30,22±6,24, grupo 2=39,02±9,15, grupo 3=49,77±9,20 y grupo 4=59,28±8,95. En la comparación post hoc, en comparaciones por pares entre todos los grupos, las diferencias fueron altamente significativas (pgrupo 3>grupo 2>grupo 1 (p<0,001). Según los resultados de este estudio, el método de laboratorio en la enseñanza de la anatomía esquelética es la mejor alternativa a la enseñanza de la anatomía en 3D.

Evaluation of Head and Cervical Spine Posture after Therapy with Maxillary Protraction Appliances / Evaluación de la Postura de la Cabeza y la Columna Cervical Después de la Terapia con Aparatos de Protracción Maxilar

SUMMARY: The objective of this study was to evaluate the changes of head and cervical spine posture of skeletal class malocclusion in adolescent with maxillary protraction. Thirty cases of skeletal class malocclusion were randomly selected from the Stomatological Hospital of Shanxi Medical University. High-quality lateral cephalograms were collected including pre- and posttreatment to compare the changes of head and cervical spine posture. Data were processed using SPSS 26.0 statistical software. The paired-t test was used to compare pre- and posttreatment mean angular measurements.A significant difference in the SNA(p<0.001), SNB(p<0.01), and ANB(p<0.001) between T1 and T2 showed an improvement in the sagittal relationships. A significant change was observed in middle cervical spine posture, while upper cervical spine posture variables showed no significant difference after treatment. Skeletal class with maxillary protraction appliance not only led to the improvement of sagittal relationship, but also changed the middle cervical spine posture.

El objetivo de este estudio fue evaluar los cambios en la postura de la cabeza y la columna cervical debido a la maloclusión clase esquelética en adolescentes con protracción maxilar. Treinta casos de maloclusión de clase esquelética fueron seleccionados al azar del Hospital Estomatológico de la Universidad Médica de Shanxi. Se recogieron cefalogramas laterales de alta calidad, incluidos el tratamiento previo y posterior, para comparar los cambios en la postura de la cabeza y la columna cervical. Los datos se procesaron con el software estadístico SPSS 26.0. Se utilizó la prueba t pareada para comparar las medidas angulares medias antes y después del tratamiento. Una diferencia significativa en SNA (p <0,001), SNB (p <0,01) y ANB (p <0,001) entre T1 y T2 mostró una mejora en las relaciones sagitales. Se observó un cambio significativo en la postura de la columna cervical media, mientras que las variables de postura de la columna cervical superior no mostraron diferencias significativas después del tratamiento. La clase esquelética con aparato de protracción maxilar no solo condujo a la mejora de la relación sagital, sino que también cambió la postura de la columna cervical media.

Body morphology and bone age in girls with obesity and without obesity aged 8 to 15 years: a cross-sectional study / Morfologia corporal e idade óssea em meninas com obesidade e sem obesidade de 8 a 15 anos: um estudo transversal

Introduction: childhood obesity is one of the main public health problems worldwide, leading to health status repercussions and growth and maturation process implications in both children and adolescents. Objective: the aim of this study was to verify body morphology and bone age variations in girls with obesity and without obesity. Methodology: this comprises a cross-sectional study conducted with 140 girls aged 8 to 15 years old, 70 with obesity and 70 without obesity. Hip and waist circumferences, body mass, height and and Body Mass Index (BMI) were determined. For maturation status determinations, bone ages were determined by a left wrist and hand radiography employing the Fels method. Results: the findigs indicate significant correlations between nutritional and maturation statuses (r=0.80; p˂0.01). Girls with obesity presented higher weight and BMI values, larger waist and hip circumferences and more advanced bone age compared to girls without obesity (p˂0.01). The same significant differences (p˂0.01) were noted in the contrasting maturational group analysis, where girls presenting advanced maturation always exhibited the highest parameter values. Conclusion: nutritional status is associated to maturation status, and girls with obesity exhibit more advanced bone age than girls without obesity.

Introdução: a obesidade infantil é um dos principais problemas de saúde pública mundial, com repercussões no estado de saúde e implicações no processo de crescimento e maturação de crianças e adolescentes. Objetivo: verificar a variação da morfologia corporal e da idade óssea em meninas com e sem obesidade. Metodologia: estudo transversal conduzido com 140 meninas de 8 a 15 anos de idade, sendo 70 meninas com obesidade e 70 sem obesidade. Foram mensuradas as circunferências do quadril e da cintura, massa corporal, altura e o Índice de Massa Corporal (IMC). Para o status maturacional foi determinada a idade óssea por meio de radiografia de punho e mão esquerdos pelo Método Fels. Resultados: os resultados apontaram a existência de correlação entre o status nutricional e o status maturacional (r=0,80; p˂0,01). As meninas com obesidade apresentaram maior peso, IMC mais elevado, circunferências maiores e idade óssea mais avançada quando comparadas às meninas sem obesidade (p˂0,01). Na análise dos grupos maturacionais contrastantes as mesmas diferenças se apresentaram com valores significativos (p˂0,01), sendo as meninas avançadas maturacionalmente sempre com valores superiores. Conclusão: o status nutricional apresentou correlação com o status maturacional, e as meninas com obesidade apresentam idade óssea mais avançada que aquelas sem obesidade

Sarcopenia em pacientes de 40 a 64 anos hospitalizados por insuficiência cardíaca / Sarcopenia in patients aged 40­64 years hospitalized for heart failure

Introdução: a insuficiência cardíaca (IC) e a sarcopenia são condições prevalentes e inter-relacionadas, figurando como importantes causas de limitações funcionais. Objetivo: avaliar critérios de sarcopenia, e suas relações com parâmetros cardiometabólicos, em pacientes de 40 a 64 anos hospitalizados por IC. Metodologia: estudo de corte transversal com caráter analítico envolvendo indivíduos com IC confirmada. A massa muscular apendicular (MMA) foi avaliada através da absorciometria por raios-X de dupla energia (DXA), considerando-se baixa MMA mulheres com MMA/altura² <5,5 kg/m² ou MMA/índice de massa corporal (IMC) <0,512 e homens com MMA/altura² <7,0 kg/m² ou MMA/IMC <0,789. Baixa força de preensão manual (FPM) foi considerada quando <16 kg em mulheres e <27 kg em homens. Resultados: avaliou-se 109 pacientes (50,5% mulheres), com mediana de idade de 58 anos. Constatou-se baixa MMA em 41,3% e baixa FPM em 64,2%, não havendo correlação significativa entre FPM e MMA em nenhum dos gêneros. Baixa MMA se associou ao gênero masculino (68,9% versus 35,9%; p=0,001), a maiores idades (60,0 [53,0-63,0] versus 57,0 [51,3-60,0] anos; p=0,039) e maiores níveis séricos de paratormônio (48,0 [30,5-94,4] versus 29,9 [23,0-54,1] pg/mL; p=0,009). Baixa FPM se associou a maior sintomatologia cardíaca (75,7% com baixa FPM tinham classificação funcional da New York Heart Association III-IV, versus 51,3% daqueles com FPM normal; p=0,009). Conclusões: há uma relevante prevalência de sarcopenia em pacientes de 40 a 64 anos hospitalizados por IC, observando-se maior frequência de baixa MMA nos homens, associação entre baixa FPM e sintomatologia cardíaca, e maiores níveis de paratormônio naqueles com perda muscular.

Introduction: heart failure (HF) and sarcopenia are prevalent and interrelated conditions, being important causes of functional limitations. Objective: to evaluate sarcopenia criteria, and their relationship with cardiometabolic parameters, in patients aged 40­64 years hospitalized for HF. Methodology: Cross-sectional study including patients with established HF. Appendicular skeletal muscle mass (ASMM) was assessed using dual-energy X-ray absorptiometry (DXA), considering low ASMM women with ASMM/height² <5.5 kg/m² or ASMM/body mass index (BMI) <0.512 and men with ASMM/height² <7.0 kg/m² or ASMM/BMI <0.789. Low handgrip strength (HGS) was considered when <16 kg in women and <27 kg in men. Results: we evaluated 109 patients (50.5% women), with a median age of 58 years. Low ASMM was found in 41.3% and low HGS in 64.2%, with no significant correlation between HGS and ASMM in either gender. Low ASMM was associated with male gender (68.9% versus 35.9%; p=0.001), older age (60.0 [53.0-63.0] versus 57.0 [51.3-60, 0] years; p=0.039) and higher serum parathyroid hormone (48.0 [30.5-94.4] versus 29.9 [23.0-54.1] pg/mL; p=0.009). Low HGS was associated with greater cardiac symptoms (75.7% with low HGS had a New York Heart Association III-IV functional classification, versus 51.3% of those with normal HGS; p=0.009). Conclusions: there is a relevant prevalence of sarcopenia in patients aged 40­64 years hospitalized for HF, observing a higher frequency of low ASMM in men, an association between low HGS and cardiac symptoms, and higher levels of parathyroid hormone in those with muscle wasting.

Predictive factors of the contracture test for diagnosing malignant hyperthermia in a Brazilian population sample: a retrospective observational study

Abstract Introduction Malignant Hyperthermia (MH) is a pharmacogenetic, hereditary and autosomal dominant syndrome triggered by halogenates/succinylcholine. The In Vitro Contracture Test (IVCT) is the gold standard diagnostic test for MH, and it evaluates abnormal skeletal muscle reactions of susceptible individuals (earlier/greater contracture) when exposed to caffeine/halothane. MH susceptibility episodes and IVCT seem to be related to individual features. Objective To assess variables that correlate with IVCT in Brazilian patients referred for MH investigation due to a history of personal/family MH. Methods We examined IVCTs of 80 patients investigated for MH between 2004‒2019. We recorded clinical data (age, sex, presence of muscle weakness or myopathy with muscle biopsy showing cores, genetic evaluation, IVCT result) and IVCT features (initial and final maximum contraction, caffeine/halothane concentration triggering contracture of 0.2g, contracture at caffeine concentration of 2 and 32 mmoL and at 2% halothane, and contraction after 100 Hz stimulation). Results Mean age of the sample was 35±13.3 years, and most of the subjects were female (n=43 or 54%) and MH susceptible (60%). Of the 20 subjects undergoing genetic investigation, 65% showed variants in RYR1/CACNA1S genes. We found no difference between the positive and negative IVCT groups regarding age, sex, number of probands, presence of muscle weakness or myopathy with muscle biopsy showing cores. Regression analysis revealed that the best predictors of positive IVCT were male sex (+12%), absence of muscle weakness (+20%), and personal MH background (+17%). Conclusions Positive IVCT results have been correlated to male probands, in accordance with early publications. Furthermore, normal muscle strength has been confirmed as a significant predictor of positive IVCT while investigating suspected MH cases.

Análisis bidimensional de la permeabilidad de vías aéreas con el tamaño de senos maxilares y su relación con el desarrollo de la clase esqueletal en una población mexicana / Two-dimensional analysis of airway permeability with maxillary sinus size and its relationship with the development of skeletal class in a Mexican population

Comparar la permeabilidad de las vías aéreas y el tamaño de los senos maxilares en relación con la clase esqueletal. se midieron 90 radiografías lateral de cráneo, divididas en 3 grupos, comparando las 3 clases esqueletales, las cuales se determinaron con la medida ANB de Steiner, y estas a su vez en dos subgrupos que fueron hombres y mujeres, en las cuales se utilizó el análisis de McNamara para el análisis de vías aéreas y para el área del seno maxilar se tomaron dos medidas una antero-posterior y cefálica-caudal. Al comparar los hombres con las mujeres se identificó significancia estadística en vía área superior de clase II (p=≤0.017), vía aérea inferior de clase III (p=≤0.006). Al comparar las clases esqueletales en hombres se identificó diferencias en la vía aérea superior en las clases I vs III (p=≤0.05), inferior en la clase I vs III (p=≤0,001) y II vs III (p=≤0.044). Con respecto a mujeres se identificó significancia en la vía aérea superior al comparar la clase I vs II (p=≤0,043), vía aérea inferior en la clase II vs III (p=≤0.05), longitud del seno maxilar al comparar clase I vs II (p=≤0.017). Entre la clase I esqueletal y la clase II, el tamaño de los senos maxilares resulto menor en longitud en las mujeres de clase II esqueletal. Entre la clase I y clase III esqueletal en hombres, se encontró una longitud menor en la vía aérea superior e inferior en la clase I. Las vías aéreas resultaron en menor tamaño en sujetos de clase II.

SUMMARY: To compare the airway permeability and the size of the maxillary sinuses in relation to the skeletal class. 90 lateral skull radiographs were divided into 3 groups, comparing the 3 skeletal classes, which were determined with Steiner's ANB measurement, and these were once in two subgroups that were men and women, in any McNamara analysis was used for the analysis of airways and for the maxillary sinus area measurements were made an antero-posterior and cephalic-caudal. When comparing males with females, statistical significance was identified in the upper class II route (p=≤0,017), lower class III airway (p=≤0.006). At least skeletal classes in men, differences were identified in the upper airway in classes I vs III (p=≤0.05), lower in class I vs III (p=≤0.001) and II vs III (p=≤0.044). With respect to women, significance was identified in the upper airway when comparing class I vs II (p=≤0.043), lower airway in class II vs. III (p=≤0.05), maxillary sinus length to class I vs II (p=≤0.017). Between skeletal class I and class II, maxillary sinus size was shorter in length in skeletal class II women. Between class I and skeletal class III in men, a lower length was found in the upper and lower airways in class I. The airways were found to be smaller in class II subjects.

Surgical-exposure-of-teeth-in-cleidocranical-dysplasia-under-procedural-sedation-case-report

Cleidocranial Dysplasia (CCD) is a rare autosomal dominant condition which is mainly characterised by skeletal abnormalties like hypoplastic or aplastic clavicles, increase in transverse diameter of the cranium, delayed closure of fontanelles with presence of open sutures and skeletal changes in maxilla and mandible. 2,4 These patients usually presents with delayed exfoliation of deciduous teeth, delayed or failed eruption of the permanent dentition with multiple supernumerary teeth, protruding mandible and mid- face retrusion.2 We present a case report of a 16 year old girl undergoing multiple surgical exposure of impacted permanent teeth under conscious sedation so as to move them in normal occlusion with orthodontic traction.

Stress perception among the elderly and its effect on some muscle strength parameters

Background: With improvement in living conditions in the population and the availability of treatments for various communicable and non-communicable diseases, the life expectancy and consequently the elderly population have increased. Stress leads to mental and physical problems. Aim and Objective: The aim of this study was to assess the effect of stress on muscle functions in the elderly. Materials and Methods: One hundred apparently healthy persons (50 males and 50 females) took part in the study. Perceived stress scale was used to measure their level of stress. Maximum voluntary contraction (MVC) and endurance time were measured with the help of a handgrip dynamometer. Results: The elderly population sample in our study showed a moderate level of stress, but there was no significant difference between the three age groups under study. Conclusion: A significant positive correlation between MVC and stress level was observed in our study subjects.

Effect of dexmedetomidine on tourniquet-induced skeletal muscle injury

SUMMARY OBJECTIVE: The aim of this study was to investigate whether dexmedetomidine could reduce tourniquet-induced skeletal muscle injury. METHODS: C57BL6 male mice were randomly assigned to sham, ischemia/reperfusion, and dexmedetomidine groups. Mice in the ischemia/reperfusion and dexmedetomidine groups received normal saline solution and dexmedetomidine intraperitoneally, respectively. The sham group underwent the same procedure as the ischemia/reperfusion group, with the exception of tourniquet application. Subsequently, the ultrastructure of the gastrocnemius muscle was observed, and its contractile force was examined. In addition, Toll-like receptor 4 and nuclear factor-κB expression within muscles was detected by Western blot. RESULTS: Dexmedetomidine alleviated myocyte damage and increased the contractility of skeletal muscles. Moreover, dexmedetomidine significantly inhibited the expression of Toll-like receptor 4/nuclear factor-κB in the gastrocnemius muscle. CONCLUSION: Taken together, these results demonstrate that dexmedetomidine administration attenuated tourniquet-induced structural and functional impairment of the skeletal muscle, partly through inactivation of the Toll-like receptor 4/nuclear factor-κB pathway.