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Objective: To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD) in children. Methods: For the case series study, 21 children with AESD from Peking University First Hospital, Provincial Children's Hospital Affiliated to Anhui Medical University, Children's Hospital of Fudan University, and Shanxi Children's Hospital who were diagnosed and treated from October 2021 to July 2023 were selected. Clinical data were collected to summarize their clinical information, imaging, and laboratory tests, as well as treatment and prognostic characteristics. Descriptive statistical analysis was applicated. Results: Of the 21 cases with AESD, 11 were males and 10 were females, with the age of onset of 2 years and 6 months (1 year and 7 months, 3 years and 6 months). Of the 21 cases, 18 were typical cases with biphasic seizures. All typical cases had early seizures within 24 hours before or after fever onset. Among them, 16 cases had generalized seizures, 2 cases had focal seizures, and 7 cases reached the status epilepticus. Of the 21 cases, 3 atypical cases had late seizures in biphasic only. The late seizures in the 21 cases occurred on days 3 to 9. The types of late seizures included focal seizures in 12 cases, generalized seizures in 6 cases, and both focal and generalized seizures in 3 cases. Diffusion-weighted imaging (DWI) test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named "bright tree sign" in all cases. The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease. Among 21 cases, 20 had been misdiagnosed as autoimmune encephalitis, central nervous system infection, febrile convulsions, posterior reversible encephalopathy syndrome, acute disseminated encephalomyelitis, and hemiconvulsion-hemiplegia-epilepsy syndrome. All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect. By July 2023, 18 cases were under follow-up. Among them, 17 cases were left with varying degrees of neurologic sequelae, including 11 cases with post-encephalopathic epilepsy; 1 recovered completely. Conclusions: AESD is characterized by biphasic seizures clinically and "bright tree sign" on DWI images. Symptomatic and supportive treatments are recommended. The immunotherapy is ineffective. The prognosis of AESD is poor, with a high incidence of neurological sequelae and a low mortality.
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Masculino , Femenino , Niño , Humanos , Lactante , Preescolar , Síndrome de Leucoencefalopatía Posterior/complicaciones , Convulsiones/etiología , Encefalopatías/diagnóstico por imagen , Estado Epiléptico , Convulsiones Febriles/diagnóstico por imagenRESUMEN
In this study, we studied the solubility and permeability of matrine, oxymatrine, sophoridine, and oxysophocarpine, four alkaloids in the Mongolian herbal medicine Sophorae Flavescentis Radix, and evaluated the absorption mechanism with the Caco-2 cell model, so as to provide a basis for the new drug development and efficacy evaluation of Sophorae Flavescentis Radix. The results showed that all the four alkaloids had high solubility and high permeability and can be well absorbed, belonging to the class-I drugs of Biopharmaceutical Classification System(BCS). The absorption(AP→BL) and excretion(BL→AP) of matrine and oxymatrine were not affected by the concentration while the absorption depended on P-gp protein. The absorption(AP→BL) and excretion(BL→AP) of sophoridine and oxysophocarpine were positively related to the concentration and time, and the absorption process was independent from P-gp protein. The results provide scientific reference and an experimental basis for the development of Mongolian medical prescriptions containing Sophorae Flavescentis Radix.
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Humanos , Alcaloides , Productos Biológicos , Células CACO-2 , Medicamentos Herbarios Chinos , Medicina de Hierbas , SophoraRESUMEN
OBJECTIVE@#To investigate the mechanism of Radix Kansui (RK) stir-fried with vinegar (VRK) decreased hepatotoxicity in mice.@*METHODS@#According to a random number table, 40 mice were randomly divided into negative control group (0.5% carboxymethylcellulose sodium, 20 mL/kg), positive control group (0.1% mixture of carbon tetrachloride in soybean oil, 20 mL/kg), RK group (the ethyl acetate extracts of RK, 250 g crude drug/kg) and VRK group (the ethyl acetate extracts of VRK, 250 g crude drug/kg) with 10 mice per group. All mice were administered orally by gavage daily for 7 continuous days. The morphology of liver tissues was examined to assess the liver injury by a transmission electron microscope. Hepatocyte apoptosis in vivo was determined by terminal deoxynucleotidyltransferase-mediated dUTP-biotin nickend labeling (TUNEL) assay. Immunohistochemical technique was adopted to detect the expression of particular antiapoptotic and proapoptotic proteins in the mitochondrial pathways, including B-cell lymphoma (Bcl-2) and caspase-3, as well as the expression of inflammatory mediators, including nuclear factor kappa B (NF- κ B) and intercellular adhesion molecule-1 (ICAM-1).@*RESULTS@#Liver injury and hepatocyte apoptosis were observed in RK mice, and the liver injury were significantly reduced in VRK-treated mice. In immunohistochemistry study, compared with the negative control group, RK inhibited dramatically the Bcl-2 protein expression and significantly increased the expression of caspase-3, NF- κ B and ICAM-1 (all P<0.01). Compared with the RK group, VRK group induced significant increase on Bcl-2 protein expression, and decreased the caspase-3, NF- κ B and ICAM-1 protein expression (P<0.05 or P<0.01).@*CONCLUSION@#The mechanism of reduced hepatotoxicity of VRK may be associated with the reduced inflammation, regulation of antiapoptotic and proapoptotic mediators in the mitochondrial pathway.
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Multi-template molecularly imprinted solid phase extraction not only has the advantages of high selectivity, large adsorption capacity, easy preparation, reuse and low environmental pollution, but also can realize the enrichment and separation of many kinds of compounds. It has attracted wide attention in the extraction and separation of traditional Chinese medicine components. This study summarizes the latest development of multi-template molecularly imprinted solid phase extraction. At the same time, based on the classification of active components of traditional Chinese medicine (flavonoids, alkaloids, phenylpropanol, terpenes, etc.), the latest application of multi-template molecular imprinting solid phase extraction in multi-component separation of traditional Chinese medicine was reviewed, with a view to better application of multi-template molecularly imprinted polymer in active multi-component extraction and separation of traditional Chinese medicine and provide reference for the material basic research of the efficacy of traditional Chinese medicine.
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This study integrates metabolomics and network pharmacology techniques to systematically analyze the possible mechanism of Pudilan Xiaoyan oral liquid (PDL) in the treatment of acute respiratory infections. GC-MS metabolomics analysis found 8 endogenous metabolites, 3-phosphoglycerate, α-aminoadipate, D-ribulose-5-phosphate, β-mannosylglyceric acid, D-fructose, urea, D-maltose and ornithine in the serum of mice with acute respiratory infection induced by LPS; these substances can be used as biomarkers for PDL use in the treatment of acute respiratory infections. Biological network studies revealed 10 potential targets for intervention by PDL in the glycolysis and pentose phosphate pathways, including GPI, G6PD, H6PD, PFKM, TALDO1, TKT, GAPDH, HK1, PKLR and TPI1. All animal experiments were carried out with approval of the Animal Ethics Committee of Nanjing University of Chinese Medicine. Our findings indicate that the strategy of combining metabolomics and network analysis can provide information on the possible mechanism of PDL in acute respiratory infections, and reveal that PDL may ameliorate the pathological process of acute respiratory infections by regulating disordered metabolic pathways.
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Mineral medicine is an indispensable part of traditional Chinese medicine and has a long history of application. Among them, mineral-based hemostatics have been widely applied for the treatment of various hemorrhagic diseases with extensive clinical experience and established efficacy. Gypsum Fibrosum (GF), a commonly used mineral medicine in clinical, can clear away heat, and relieve anxiety and thirst. Gypsum Ustum (GU) is the processed product of GF after calcining at high temperature. It is mainly composed of anhydrous calcium sulfate (CaSO4) with the functions of moisturizing, promoting muscle growth, astringent sores and hemostasis. GU is often used externally to treat ulcer, itching, eczema, water and fire scalds, trauma bleeding, etc. Studies on the mechanism of hemostasis have shown that Ca2+ (coagulation factor Ⅳ) is involved in many key processes of the internal and external coagulation cascades and can prevent bleeding by regulating platelet activation and aggregation, and promoting the production of insoluble fibrin and the ultimate formation of a blood clot. GF and GU both contain Ca2+ which provide an important material basis of hemostatic effect for both compounds, but GU has a significant hemostatic effect, while GF has no hemostatic effect. After processing, the taste and efficacy of the GF have been obviously changed which reflects the characteristics of processing, but the processing mechanism of GU has not been fully clarified. Therefore, based on studies of GF before and after calcining, this paper focused on these aspects including calcining process, crystal form comparison, element content, efficacy comparison, and summarized various aspects of Ca2+ involved in hemostasis. In addition, the hemostatic properties of other calcium-containing mineral medicines and new calcium-containing hemostatic materials such as calcium alginate, mesoporous calcium silicate and nanogel hemostatic materials were also discussed. The paper aimed to provide a reference for elucidating processing mechanism and clinical dialectical use of GU, also to promote development of new calcium-containing hemostatic materials.
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Mongolians have a long history of using prescriptions, which can be classified into four stages as follows: the germination and experience accumulation stage before the 13 th century, the theoretical formation stage from the 13 th to 16 th century, the rapid development stage from the 17 th to 20 th century, and the leaping development stage from the mid-20 th century to the present. The prescriptions from the ancient classical or representative medical books have always been used by Mongolian physicians for generations, and they are still in use due to the definite curative effects. In 2008, the Notice on Issuing the Supplementary Provisions to the Registration and Management of Traditional Chinese Medicine(TCM) described that China has attached more importance to the excavation and development of classical prescriptions. As stipulated in the Law of the People's Republic of China on Traditional Chinese Medicine, the classical prescriptions should be those available in ancient TCM classics and still in wide use, with exact curative effects, distinct features, and obvious advantages. This paper expounded the historical formation and development of classical prescriptions in Mongo-lian medicine, introduced the five most influential ancient medical books revealing the formation and development of these classic prescriptions, and traced the origin of such classical prescriptions as Wenguanmu Siwei Decoction, Shouzhangshen Bawei Decoction, Jianghuang Siwei Decoction and summarized the origin, development history and characteristics of classical prescriptions in Mongolian medicine, aiming to provide a reference for their further research and development.
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Humanos , Libros , China , Medicamentos Herbarios Chinos , Medicina Tradicional China , Medicina Tradicional Mongoliana , PrescripcionesRESUMEN
B / T lymphuocyte attenuator (BTLA) is one of the important checkpoint molecules in immune regulation. BTLA belongs to the CD28 superfamily, and its protein structure is similar to programmed cell death-1 (PD-1) and cytotoxic T lymphocyte associated antigen-4 (CTLA-4). BTLA is mainly expressed in B and T cells, and has also been found in some innate immune cells such as dendritic cells and monocytes. To date, the herpesvirus entry mediator (HVEM) is the only ligand for BTLA found in human cells. HVEM belongs to the Tumor necrosis factor receptor (TNFR) superfamily, and its interaction with BTLA directly connects CD28 and TNFR families. The binding of BTLA with HVEM often mediates immunosuppressive effects and plays an important role in maintaining immune tolerance. However, recent studies have found that the BTLA / HVEM pathway not only provides negative regulatory signals, but also promotes the survival of T cells. This bidirectional signaling system renders BTLA-mediated immune regulation more sophisticated and complex. Growing evidence has shown that BTLA is involved in T cells, B cells and dendritic cell-mediated immune regulation, and therefore plays an important role in a variety of immune related diseases, such as inflammation, tumor, infectious diseases and autoimmune diseases. Based on the latest research progress at home and abroad, this paper reviews the role of BTLA in immune regulation and immune related diseases.
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Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.
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Niño , Femenino , Humanos , Lactante , Glucocorticoides , Imagen por Resonancia Magnética , Miositis/tratamiento farmacológico , RituximabRESUMEN
Objective: To explore the classification and reconstruction strategy of defects in lateral face region after operation of malignant tumors. Methods: Eighteen cases with the reconstruction of complicated defects after resection of tumors in the region of lateral face from January 2015 to January 2018 in Hunan Cancer Hospital were retrospectively reviewed. There were 14 males and 4 females, aged from 32 to 68 years. According to the presence or absence of bony scaffold, complicated defects were divided into two main categories: soft tissue perforating defects and soft tissue defects combined with bony scaffold defects. All soft tissue perforating defects in 5 cases were repaired with free anterolateral femoral flaps. Among 13 cases with soft tissue plus bony scaffold defects, 3 were repaired with free fibular flaps, 6 with free fibular flaps combined with free anterolateral femoral flaps, and 4 with chimeric deep circumflex iliac artery perforator flaps combined with anterolateral femoral flaps. Results: All flaps survived well. Two patients complicated with fistula in floor of mouth, but the wound healed after dressing change. Transoral feeding was resumed within 2 weeks after surgery in all patients. One year follow-up evaluation showed that 14 cases had symmetrical face and 10 cases had mouth opening more than 3 transverse fingers. After 36-50 months of follow-up, 6 patients died, with an overall 3-year survival rate of 66.7%. Conclusion: The classification of defects with or without bony stent loss is conducive to the overall repair design, the recovery of facial contour stent, the effective fill of dead space and the maintain of residual occlusal relationship. Good reconstruction results require a multi flap combination of osteocutaneous and soft tissue flaps.
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Femenino , Humanos , Masculino , Neoplasias Faciales , Colgajos Tisulares Libres , Colgajo Perforante , Procedimientos de Cirugía Plástica , Estudios Retrospectivos , Trasplante de Piel , Traumatismos de los Tejidos Blandos/cirugía , Muslo/cirugíaRESUMEN
OBJECTIVE@#To investigate the current status of antibiotic use for very and extremely low birth weight (VLBW/ELBW) infants in neonatal intensive care units (NICUs) of Hunan Province.@*METHODS@#The use of antibiotics was investigated in multiple level 3 NICUs of Hunan Province for VLBW and ELBW infants born between January, 2017 and December, 2017.@*RESULTS@#The clinical data of 1 442 VLBW/ELBW infants were collected from 24 NICUs in 2017. The median antibiotic use duration was 17 days (range: 0-86 days), accounting for 53.0% of the total length of hospital stay. The highest duration of antibiotic use was up to 91.4% of the total length of hospital stay, with the lowest at 14.6%. In 16 out of 24 NICUs, the antibiotic use duration was accounted for more than 50.0% of the hospitalization days. There were 113 cases with positive bacterial culture grown in blood or cerebrospinal fluid, making the positive rate of overall bacterial culture as 7.84%. The positive rate of bacterial culture in different NICUs was significantly different from 0% to 14.9%. The common isolated bacterial pathogens Klebsiella pneumoniae was 29 cases (25.7%); Escherichia coli 12 cases (10.6%); Staphylococcus aureus 3 cases (2.7%). The most commonly used antibiotics were third-generation of cephalosporins, accounting for 41.00% of the total antibiotics, followed by penicillins, accounting for 32.10%, and followed by carbapenems, accounting for 13.15%. The proportion of antibiotic use time was negatively correlated with birth weight Z-score and the change in weight Z-score between birth and hospital discharge (r=-0.095, -0.151 respectively, P<0.01), positively correlated with death/withdrawal of care (r=0.196, P<0.01).@*CONCLUSIONS@#Antibiotics used for VLBW/ELBW infants in NICUs of Hunan Province are obviously prolonged in many NICUs. The proportion of routine use of third-generation of cephalosporins and carbapenems antibiotics is high among the NICUs.
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Humanos , Lactante , Recién Nacido , Antibacterianos , Peso al Nacer , Recien Nacido con Peso al Nacer Extremadamente Bajo , Unidades de Cuidado Intensivo Neonatal , Encuestas y CuestionariosRESUMEN
To explore the optimal therapy time for the treatment of severe coronavirus disease 2019(COVID-19)by traditional Chinese medicine(TCM)and its influence on the therapeutic effect and prognosis. The clinical data,laboratory findings,and outcomes of 64 patients with severe COVID-19 treated with TCM and western medicine in Chongqing from January 20,2020, to March 11,2020 were retrospectively analyzed.Patients were divided into early intervention group[TCM was initiated within 3 days (including day 3) after the first diagnosis of severe type/critical type COVID-19]and late intervention group[TCM was initiated after 7 days (including day 7) after the first diagnosis of severe type /critical type COVID-19].The changes in clinical parameters during the course of disease were compared between the two groups. On day 14,the oxygenation index was 292.5(252.0,351.0)mmHg in the early intervention group,which was significantly higher than that in the late intervention group [246.0(170.0,292.5)mmHg](=0.005).The length of hospital stay [(18.56±1.11)d (24.87±1.64)d,=0.001],duration of ICU stay [(14.12±0.91)d (20.00±1.53)d,=0.000] and time to negativity [(16.77±1.04)d (22.48±1.66)d,=0.001] in the early intervention group were significantly shorter than those in the late intervention group.The intubation rate(7.3%)in the early intervention group was significantly lower than that in the late intervention group(30.4%)(=0.028). Early TCM therapy within three days after a diagnosis of severe COVID-19 can shorten the length of hospital stay,duration of ICU stay,and time to negativity and decrease intubation rate.
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Humanos , Betacoronavirus , Infecciones por Coronavirus , Quimioterapia , Medicina Tradicional China , Pandemias , Neumonía Viral , Quimioterapia , Pronóstico , Estudios RetrospectivosRESUMEN
Objective::To establish the HPLC fingerprint of carbonized ginger and to determine the contents of zingerone, 6-gingerol, 6-shogaol, 10-gingerol, 8-shogaol and 10-shogaol with quantitative analysis of multi-components by single marker (QAMS). Method::The fingerprint of carbonized ginger was established by HPLC. All samples were analyzed by Waters SymmetryShield™ RP18 column (4.6 mm×250 mm, 5 μm) with gradient elution by acetonitrile(A)-water(B) (0-30 min, 25%-70%A; 30-50 min, 70%-90%A; 50-60 min, 90%A), the flow rate was 1.0 mL·min-1, the detection wavelength was set at 240 nm and the column temperature was 30 ℃. Zingerone, 6-gingerol, 8-gingerol, 6-shogaol, 10-gingerol, 8-shogaol and 10-shogaol was chosen as marker ingredients to establish HPLC fingerprint of carbonized ginger decoction pieces. Taking 6-gingerol as internal reference standard, the contents of zingerone, 6-shogaol, 10-gingerol, 8-shogaol and 10-shogaol were determined at the detection wavelength of 220 nm and 280 nm according to the relative correction factor. Result::The HPLC fingerprint of carbonized ginger was obtained and 10 common peaks were designated, and 7 of them were identified as zingerone, 6-gingerol, 8-gingerol, 6-shogaol, 10-gingerol, 8-shogaol and 10-shogaol, respectively. And there were no significant differences between the quantitative results of external standard method and QAMS. It is suggested that the content limits of carbonized ginger should be not less than 0.020%of zingerone (C11H14O3), 0.050%of 6-gingerol (C17H26O4), 0.120%of 6-shogaol (C17H24O3), 0.080%of 10-gingerol (C21H34O4), 0.030%of 8-shogaol (C19H28O3) and 0.050%of 10-shogaol (C21H32O3) calculated with reference to the dried products, respectively. Conclusion::The developed method is accurate and feasible, which can provide a simple and effective method for the quality control of carbonized ginger.
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Based on the concept of network pharmacology, the main nephroprotective components in Erzhi Pill reported in previous studies, were used to predict the targets through the PharmMapper method. Molecular docking was applied to screen for potential targets and biological information annotation databases (DAVID) was used to analyze the molecular function and biological process of the action targets. The Cytoscape software was used to construct the “ingredient-target-pathway” network of Erzhi Pill for renal injury treatment. TTD and GAD database were then applied to screen for the targets of renal disease for building “ingredient-core target” network. We found that 17 major active ingredients of Erzhi Pill regulated 32 targets (including ESR1, ESR2, GCK, MMP3) and affected 6 pathways, such as PI3K-Akt signaling pathway, estrogen signaling pathway and purine metabolism. This study reflected the nature of traditional Chinese medicine as multi-ingredients, multi-targets and multi-pathways, providing new clues for basic science research on the nephroprotective pharmacological mechanism of Erzhi Pill.
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Objective: To investigate the expression of IL-38 and MIP-2 in lung tissue of rats with pulmonary fibrosis induced by bleomycin, and to explore the significance of IL-38 and MIP-2 in pulmonary fibrosis in rats. Methods: 45 Wistar rats were randomly divided into saline control group ( group N), bleomycin group ( group B) and dexamethasone group ( group D) according to the random and control principle. On the 7 th, 14 th, and 28 th day, 5 rats were killed in each group. The pathological changes of lung tissue were observed by hematoxylin-eosin ( HE) staining in lung tissue. The expression of IL-38 and HYP in lung tissue of rats was measured by enzyme linked immunoassay ( ELISA) and the expression of MIP-2 in lung tissue of rats was measured by RT-PCR method. Results: (1) HE staining showed that the lung tissue from group B and group D developed from normal to inflammatory changes to pulmonary fibrosis. (2) The expression of IL-38 in group B and D decreased gradually, and the decrease was most obvious at 28 th day, which was lower than that in group N ( P<0. 05), and the expression of IL-38 in group B was lower than that in D group, and the difference was statistically significant ( P<0. 05). (3) The expression of MIP-2 and HYP increased gradually in group B and D, which were higher than those in group N, and the difference was statistically significant ( P<0. 05). The MIP-2 and HYP expressions in group B were higher than those of group D in the same period, and the difference was statistically significant ( P<0. 05). Conclusion: IL-38 and MIP-2 play an important role in the occurrence and development of bleomycin induced pulmonary fibrosis in rats. The application of dexamethasone can improve the degree of pulmonary fibrosis in rats. The effect may be related to the up-regulation of IL-38 and the downregulation of MIP-2.
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Objective:To observe the clinical efficacy of Gao's nape acupuncture plus swallowing training in treating pharyngeal deglutition disorder after stroke.Methods:One hundred patients with post-stroke pharyngeal deglutition disorder were randomized into a treatment group and a control group,with 50 cases in each group.The two groups both received routine neurological intervention.In addition,the treatment group was given Gao's nape acupuncture plus swallowing training,while the control group was intervened by swallowing training alone.After eight-week treatment,the two groups were observed in terms of the changes in repetitive saliva swallowing test (RSST),modified water swallowing test (MWST),standardized swallowing assessment (SSA) and swallowing-related quality of life (SWAL-QOL).The clinical efficacies of the two groups were also compared.Results:After treatment,the RSST grading,and scores of MWST,SSA and SWAL-QOL changed significantly in both groups (P<0.05 or P<0.01).The RSST grading,and scores of MWST,SSA and SWAL-QOL in the treatment group were significantly different from those in the control group after treatment (P<0.05 or P<0.01).The total effective rate and markedly effective rate were respectively 100.0% and 72.3% in the treatment group,versus 97.9% and 34.0% in the control group.There was a significant difference in the markedly effective rate between the two groups (P<0.01).The difference in the clinical efficacy between the two groups was statistically significant (P<0.01).Conclusion:Gao's nape acupuncture plus swallowing training is an effective approach for post-stroke pharyngeal deglutition disorder.Its therapeutic efficacy is more significant than that of swallowing training alone.
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The patient was a male who was found to be abnormal at the age of 4.5 months. He presented with irritability, motor regression and opisthotonus. Brain MRI revealed bilateral abnormality in the lentiform nucleus, thalamus, deutocerebrum and cerebellar hemispheres. Novel compound heterozygous mutations of SLC19A3 gene, c.950G>A(p.G317E) and c.962C>T(p.A321V), were found in the patient. Further study showed that c.950G>A was inherited from his father and c.962C>T came from his mother. Using bioinformatics software analysis, both of the mutations were found to be harmful. His symptoms were improved remarkably after biotin, thiamine and "cocktail" therapy. One month later a brain MRI revealed that the lesions in basal ganglia and cerebellar hemispheres were improved. The patient was definitely diagnosed with biotin-thiamine responsive basal ganglia disease (BTBGD). BTBGD is a treatable autosomal recessive disease and early administration of biotin and thiamine may lead to clinical improvement.
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Humanos , Lactante , Masculino , Enfermedades de los Ganglios Basales , Llanto , Imagen por Resonancia Magnética , Proteínas de Transporte de Membrana , TiaminaRESUMEN
BACKGROUND@#Studies on the relationship between antiepileptic drug (AED) administration and clinical outcomes in patients with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) remain scarce. Levetiracetam (LEV) is an AED that is neuroprotective in various neurologic disorders. This study aimed to determine the impact of LEV on the outcome of MELAS.@*METHODS@#A retrospective, single-center study was performed based on a large cohort of patients with MELAS with a history of seizures (n = 102). Decisions on antiepileptic therapies were made empirically. Patients were followed up for 1 to 8 years (median, 4 years) and divided into 2 groups based on whether LEV was administered (LEV or non-LEV). The modified Rankin scale (mRS) scores and mortality risks were analyzed in all patients.@*RESULTS@#LEV, carbamazepine, benzodiazepines, topiramate, oxcarbazepine, valproate, and lamotrigine were administered in 48, 37, 18, 13, 11, 9, and 9 patients, singly or in combination, respectively. The mean mRS score of the LEV group (n = 48) was lower than that of the non-LEV group (n = 54; mean ± standard deviation, 2.79 ± 1.47 vs. 3.83 ± 1.93, P = 0.006) up to the end of the study. Nevertheless, there was no difference in the proportion of subjects without disability (mRS ranging 0-1) between the groups (P = 0.37). The multivariate regressions revealed that LEV treatment was associated with lower mRS scores (odds ratio 0.32, 95% confidence interval [CI] 0.15-0.68, P = 0.003) and mortality rates (hazard ratio 0.24, 95% CI 0.08-0.74, P = 0.013). There was a significant difference in the Kaplan-Meier survival curves between the groups (χ = 4.29, P = 0.04).@*CONCLUSIONS@#The LEV administration is associated with lower mortality in patients with MELAS in this retrospective study. Further laboratory research and prospective cohort studies are needed to confirm whether LEV has neuroprotective effects on patients with mitochondrial diseases.
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Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Acidosis Láctica , Quimioterapia , Mortalidad , Anticonvulsivantes , Usos Terapéuticos , Carbamazepina , Usos Terapéuticos , Lamotrigina , Usos Terapéuticos , Levetiracetam , Usos Terapéuticos , Encefalomiopatías Mitocondriales , Quimioterapia , Mortalidad , Oxcarbazepina , Usos Terapéuticos , Estudios Prospectivos , Estudios Retrospectivos , Accidente Cerebrovascular , Quimioterapia , Mortalidad , Topiramato , Usos Terapéuticos , Ácido Valproico , Usos TerapéuticosRESUMEN
Objective To assess the value of MRI combined with serum ferritin analysis in measurement of hepatic iron deposition among patients with hepatic alveolar echinococcosis. Methods A total of 96 patients with definitive diagnosis of hepatic alveolar echinococcosis and 30 healthy volunteers were enrolled and underwent routine 1.5T MR scanning, and all laboratory examination data were captured. The liver-to-muscle signal intensity ratio, serum ferritin level and liver functions were analyzed. Results The liver-to-muscle signal intensity ratio was (1.95 ± 0.57) in patients with hepatic alveolar echinococcosis and (2.22 ± 0.28) in healthy volunteers (t = 2.022, P < 0.05), and the liver-to-muscle signal intensity ratio was negatively associated the serum ferritin level in patients with hepatic alveolar echinococcosis (rs = –0.446, P < 0.01). Conclusions Abnormal iron deposition is detected in the liver of patients with hepatic alveolar echinococcosis, and serum ferritin level may be helpful for the identification of abnormal iron deposition in the liver of patients with hepatic alveolar echinococcosis. The liver-to-muscle signal intensity ratio measured by MRI may be a non-invasive approached used to assess the hepatic iron deposition in patients with hepatic alveolar echinococcosis.
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Objective: To evaluate the diagnostic value of bronchoalveolar lavage (BAL) for pulmonary complications in patients after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and its safety. Methods: Patients with pulmonary complications after allo-HSCT underwent BAL. Microbiological smears, culture, PCR of CMV-DNA, EBV-DNA and TB-DNA, macro genomes new generation sequencing (mNGS) techniques were performed to detect pathogens in BAL fluid (BALF) . Results: A total of 73 allo-HSCT patients with 86 times of pulmonary complications enrolled this prospective study. They underwent 132 times of BAL procedures. The clinical diagnoses of 88.4% cases were made based on BALF analysis. Of them, 67 cases (77.9%) had infectious pulmonary complications, including 29 cases (33.7%) of fungal infection, 18 cases (20.9%) of mixed infection, 11 cases (12.8%) of viral infection and 9 cases (10.5%) of bacterial infection. The other 9 cases (10.5%) of non-infectious pulmonary complications included 8 cases (9.3%) of idiopathic pneumonia syndrome (IPS) and 1 case (1.2%) of pulmonary infiltration of lymphoma. The diagnoses of the remaining 10 cases (11.6%) were not determined. The platelet counts of 33 patients were less than 50×10(9)/L before BAL. None of them developed severe bleeding complications during or after BAL. Transient fever occurred in 10 patients after BAL. Blood cultures showed staphylococcal bacteremia in them and anti-infection therapies were effective. No life-threatening complications occurred in all of the patients during or after BAL. Conclusion: BALF analysis was informative for the diagnosis of pulmonary complication and safe for patients with pulmonary complications after allo-HSCT.