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Objective: To observe the impact of implantable Collamer lens (ICL) implantation surgery on choroidal thickness and blood flow density in myopic patients. Methods: This was a prospective cohort study. Patients undergoing ICL surgery at Qingdao University Affiliated Hospital between June 2021 and May 2023 were consecutively enrolled. Patients were categorized into high myopia (HM) and super high myopia (SHM) groups based on whether their spherical equivalence power exceeded 10.00 D. Comprehensive ophthalmic examinations, including optical coherence tomography, optical coherence tomography angiography, visual acuity assessment, intraocular pressure measurement, and optometry, were performed preoperatively and at 1 week, 1 month, and 3 months postoperatively. Results: A total of 42 patients (84 eyes), with an average age of (25.27±3.18) years, comprising 11 males and 31 females, were enrolled in the study. Among them, 20 patients belonged to the HM group, while 22 patients were in the SHM group. Both choroidal thickness and blood flow density exhibited significant increases at postoperative 1 week and 1 month compared to preoperative levels (P<0.05), but returned to baseline levels by postoperative 3 months. Specifically, the subfoveal choroidal thickness increased from (169.49±61.57) µm preoperatively to (180.16±66.61) µm at 1 week, (186.69±63.32) µm at 1 month, and then reverted to (169.58±60.82) µm at 3 months. The central choroidal blood flow density showed changes from 60.03%±1.60% preoperatively to 61.04%±1.17% at 1 week, 60.42%±1.81% at 1 month, and 60.22%±1.57% at 3 months. Furthermore, the HM group exhibited more pronounced changes in both choroidal thickness and blood flow density across all time points compared to the SHM group. Significant differences were observed in choroidal thickness changes at various areas at 1 month, while changes in blood flow density in specific areas were significant. However, no significant differences were noted at 3 months postoperatively. Correlation analysis revealed a negative correlation of changes in subfoveal choroidal thickness and central choroidal blood flow density postoperatively at 1 week and 3 months with preoperative choroidal blood flow density. Notably, no correlation was found between preoperative choroidal thickness and postoperative changes. Conclusions: In the early period following ICL implantation, the increase in choroidal thickness and blood flow density may be more pronounced in HM compared to SHM, but the two parameters can return to baseline levels by 3 months. ICL implantation transiently affects the fundus microenvironment in myopic patients, with implications of preoperative choroidal blood flow.
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Corioide , Implante de Lente Intraocular , Miopia , Humanos , Corioide/irrigação sanguínea , Feminino , Masculino , Estudos Prospectivos , Adulto , Miopia/cirurgia , Implante de Lente Intraocular/métodos , Lentes Intraoculares Fácicas , Adulto JovemRESUMO
BACKGROUND: Posterior nasal neurectomy (PNN) is a commonly employed surgical approach for the treatment of allergic rhinitis (AR). Due to its denervation effect on the nasal mucosa, PNN may potentially alter the motion and defensive capability of cilia. Previous research on the effects of neural regulation and denervation on cilia has been limited by the absence of a feasible in vivo evaluation method for assessing ciliary function. METHODOLOGY: Utilizing a new system developed by our team for visualizing and analyzing ciliary motion in vivo, we analysed ciliary beat frequency and distance in vivo and histomorphological changes in a murine PNN and AR model. Ovalbumin, histamine and neurotransmitters (acetylcholine chloride, α receptor agonist and ß receptor agonist) were applied to investigate the responsiveness and neural regulation of the nasal mucosa. RESULTS: Denervation resulting from PNN led to a reduction in nasal ciliary beat frequency (CBF) to 78% of the control, as well as diminished response towards allergens and histamine. Among neurotransmitters examined, α receptor agonists exhibited inhibitory effects on in vivo ciliary motion while acetylcholine and ß receptor agonists demonstrated stimulatory effects. PNN did not affect the reactivity of in vivo cilia towards these neurotransmitters. CONCLUSIONS: PNN-induced denervation can reduce ciliary motion, potentially compromising the defensive capability of nasal mucosa. Neural regulation and the neurotransmitters involved have significant effect on ciliary motion.
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Objective: To analyze the clinical features of postpartum hepatitis flares in pregnant women with hepatitis B virus (HBV) infection. Methods: A retrospective study was conducted. Patients who met the enrollment criteria were included. Liver function and HBV virology tests were collected from pregnant women with chronic HBV infection at delivery, 6, 24, 36, and 48 weeks after delivery through the hospital information and test system. Additionally, antiviral therapy types and drug withdrawal times were collected. Statistical analysis was performed on all the resulting data. Results: A total of 533 pregnant women who met the inclusion criteria were included, with all patients aged (29.5±3.7) years old. A total of 408 cases received antiviral drugs during pregnancy to interrupt mother-to-child transmission. There was no significant difference in the levels of alanine aminotransferase (ALT, zâ =â -1.981, Pâ =â 0.048), aspartate aminotransferase (AST, zâ =â -3.956, Pâ <â 0.001), HBV load (zâ =â -15.292, Pâ <â 0.001), and HBeAg (zâ =â -4.77, Pâ <â 0.001) at delivery in patients who received medication and those who did not. All patients ALT, AST, total bilirubin, direct bilirubin, and albumin showed an upward trend within six weeks after delivery. A total of 231 cases developed hepatitis within 48 weeks after delivery. Among them, 173 cases first showed ALT abnormalities within six weeks postpartum. Conclusion: Hepatitis flare incidence peaked six weeks after delivery or six weeks after drug withdrawal in pregnant women with chronic HBV infection.
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Hepatite A , Hepatite B Crônica , Hepatite B , Complicações Infecciosas na Gravidez , Feminino , Humanos , Gravidez , Adulto , Vírus da Hepatite B/genética , Gestantes , Antivirais/uso terapêutico , Estudos Retrospectivos , Complicações Infecciosas na Gravidez/tratamento farmacológico , Antígenos E da Hepatite B , DNA Viral , Transmissão Vertical de Doenças Infecciosas , Exacerbação dos Sintomas , Período Pós-Parto , Hepatite B/tratamento farmacológico , BilirrubinaRESUMO
IMPORTANCE: In-office postoperative voiding trials (VTs) increase health care burden for patients and physicians. Adoption of an at-home VT option may decrease burden without increasing adverse events postoperatively. OBJECTIVE: The purpose of this study was to compare 30-day postoperative outcomes between participants who performed an at-home autofill VT after catheter self-discontinuation during the Assessing Healthcare Utilization and Feasibility of Transurethral Catheter Self-discontinuation (FLOTUS) study and a "historic" control cohort of patients who presented to the office for backfill-assisted VT on postoperative day (POD) 1. STUDY DESIGN: This was a retrospective cohort study of women with postoperative urinary retention after urogynecologic surgery between June 2020 and March 2022. Outcomes from the FLOTUS study were compared with a "historic" control cohort of patients that were identified on chart review from the year before FLOTUS initiation. Demographic, medical history, and procedure-related data were collected. Thirty-day outcome data included office calls/messages, office visits, emergency department visits, complications, and catheterization outcomes. RESULTS: Forty-six participants were included in the FLOTUS cohort and 65 participants in the historic cohort. There was no difference in the POD1 VT pass rate, number of office calls/messages, emergency department visits, or postoperative complications between the 2 cohorts. The FLOTUS patients attended 1 less office visit (1 vs 2 office visits, P <0.001), and this difference persisted on regression analysis (-0.87 office visits; 95% CI, -1.18 to -0.56, P <0.001). CONCLUSION: Patients who had backfill-assisted VTs on POD1 attended 1 additional office visit compared with those who removed their catheters at home.
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Remoção de Dispositivo , Complicações Pós-Operatórias , Cateterismo Urinário , Retenção Urinária , Humanos , Feminino , Estudos Retrospectivos , Pessoa de Meia-Idade , Idoso , Remoção de Dispositivo/estatística & dados numéricos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Retenção Urinária/etiologia , Retenção Urinária/epidemiologia , Cateterismo Urinário/efeitos adversos , Cateterismo Urinário/estatística & dados numéricos , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Cateteres Urinários/efeitos adversos , AutocuidadoRESUMO
Objective: To evaluate the hemostatic efficacy, safety and immunogenicity of recombinant human thrombin in the treatment of liver wounds that still ooze after conventional surgical hemostasis. Methods: A multicenter, stratified randomized, double-blind, placebo-controlled phase â ¢ trial with a planned enrollment of 510 subjects at 33 centers, with a 2â¶1 randomization to the thrombin group versus the placebo group. An interim analysis will be conducted after approximately 70% of the subjects have completed the observation period. The primary efficacy endpoint was the rate of hemostasis within 6 minutes at the point of bleeding that could be evaluated. Safety analysis was performed one month after surgery, and the positive rates of anti-drug antibody (ADA) and neutralizing antibody were evaluated. Results: At the interim analysis, a total of 348 subjects had been randomized and received the study drug (215 were male and 133 were female). They were aged 19-69 (52.9±10.9)years. Among them, 232 were in the thrombin group and 116 were in the placebo group, with balanced and comparable demographics and baseline characteristics between the two groups. The hemostasis rate at 6 minutes was 71.6% (95%CI:65.75%-77.36%) in the thrombin group and 44.0% (95%CI: 34.93%-53.00%) in the placebo group, respectively (P<0.001). No grade≥3 drug-related adverse events and no drug-related deaths were reported from the study.No recombinant human thrombin-induced immunologically-enhanced ADA or immunologically-induced ADA was detected after topical use in subjects. Conclusion: Recombinant human thrombin has shown significant hemostatic efficacy and good safety in controlling bleeding during liver resection surgery, while also demonstrating low immunogenicity characteristics.
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Hemostáticos , Trombina , Humanos , Masculino , Feminino , Trombina/efeitos adversos , Hemostáticos/uso terapêutico , Hemostáticos/efeitos adversos , Fígado , Hemostasia , Resultado do TratamentoRESUMO
OBJECTIVE: To explore the miRNAs that down- regulate calcium-sensing receptor (CaSR) in secondary hyperparathyroidism (SHPT) and their effects on parathyroid hormone (PTH) secretion. METHODS: Whole transcriptome sequencing was performed for 6 normal parathyroid tissue samples and 11 SHPT parathyroid tissue samples. Based on bioinformatic prediction, we screened out 7 candidate miRNAs that regulate CaSR, among which the most likely miRNA for CaSR regulation was identified by double luciferase test. We detected the differential expression of miR-301a-5p and CaSR mRNA in SHPT and normal parathyroid tissue using qRT-PCR, and analyzed the correlation between their expressions and serum PTH levels of the patients. Western blotting was used to detect the expression of CaSR protein in primary SHPT parathyroid cells transfected with miR-301a-5p mimics or inhibitors, and the level of PTH in the supernatant of the cell culture was determined. RESULTS: Among the preliminarily selected 7 miRNAs that potentially regulate CaSR (miR-15a-5p, miR-15b-5p, miR- 16- 5p, miR- 221- 3p, miR- 222- 3p, miR- 301a- 5p and miR- 503- 5p), miR- 301a-5p was significantly upregulated in SHPT compared with normal parathyroid tissue (P < 0.05), and its expression appeared to be positively correlated with PTH level, but this correlation was not statistically significant (P > 0.05); The expression of CaSR mRNA was significantly downregulated in SHPT (P < 0.05), and its expression tended to inversely correlate with the patient's PTH level, but the correlation was not statistically significant (P > 0.05). In primary culture of SHPT parathyroid cells, miR-301a-5p overexpression caused a significant decrease of CaSR protein expression (P < 0.05), and conversely, inhibition of miR-301a-5p expression increased the expression of CaSR protein (P < 0.05). Although miR-301a-5p overexpression did not significantly affect PTH secretion of the cells (P > 0.05), inhibition of iR-301a-5p expression strongly increased the secretion of PTH (P < 0.05). CONCLUSION: MiR-301a-5p affects PTH secretion in SHPT possibly by regulating the expression of CaSR.
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Hiperparatireoidismo Secundário , MicroRNAs , Humanos , Western Blotting , Hiperparatireoidismo Secundário/genética , MicroRNAs/genética , Receptores de Detecção de Cálcio/genética , RNA MensageiroRESUMO
BACKGROUND AND PURPOSE: Chondrosarcoma and synovial chondromatosis of the temporomandibular joint share overlapping clinical and histopathologic features. We aimed to identify CT and MR imaging features to differentiate chondrosarcoma from synovial chondromatosis of the temporomandibular joint. MATERIALS AND METHODS: The CT and MR images of 12 and 35 patients with histopathologically confirmed chondrosarcoma and synovial chondromatosis of the temporomandibular joint, respectively, were retrospectively reviewed. Imaging features including lesion size, center, enhancement, destruction/sclerosis of surrounding bone, infiltration into the tendon of the lateral pterygoid muscle, calcification, periosteal reaction, and osteophyte formation were assessed. A comparison between chondrosarcoma and synovial chondromatosis was performed with a Student t test for quantitative variables and the Fisher exact test or linear-by-linear association test for qualitative variables. Receiver operating characteristic analysis was performed to determine the diagnostic performance for differentiation of chondrosarcoma and synovial chondromatosis based on a composite score obtained by assigning 1 point for each of 9 imaging features. RESULTS: High-risk imaging features for chondrosarcoma were the following: lesion centered on the mandibular condyle, destruction of the mandibular condyle, no destruction/sclerosis of the articular eminence/glenoid fossa, infiltration into the tendon of the lateral pterygoid muscle, absent or stippled calcification, periosteal reaction, internal enhancement, and size of ≥30.5 mm. The best cutoff value to discriminate chondrosarcoma from synovial chondromatosis was the presence of any 4 of these high-risk imaging features, with an area under the curve of 0.986 and an accuracy of 95.8%. CONCLUSIONS: CT and MR imaging features can distinguish chondrosarcoma from synovial chondromatosis of the temporomandibular joint with improved diagnostic performance when a subcombination of 9 imaging features is used.
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PURPOSE: Bone morphogenic proteins (BMPs) regulate gene expression that is related to many critical developmental processes, including osteogenesis for which they are named. In addition, BMP2 is widely expressed in cells of mesenchymal origin, including bone, cartilage, skeletal and cardiac muscle, and adipose tissue. It also participates in neurodevelopment by inducing differentiation of neural stem cells. In humans, BMP2 variants result in a multiple congenital anomaly syndrome through a haploinsufficiency mechanism. We sought to expand the phenotypic spectrum and highlight phenotypes of patients harboring monoallelic missense variants in BMP2. METHODS: We used retrospective chart review to examine phenotypes from an international cohort of 18 individuals and compared these with published cases. Patient-derived missense variants were modeled in zebrafish to examine their effect on the ability of bmp2b to promote embryonic ventralization. RESULTS: The presented cases recapitulated existing descriptions of BMP2-related disorders, including craniofacial, cardiac, and skeletal anomalies and exhibit a wide phenotypic spectrum. We also identified patients with neural tube defects, structural brain anomalies, and endocrinopathies. Missense variants modeled in zebrafish resulted in loss of protein function. CONCLUSION: We use this expansion of reported phenotypes to suggest multidisciplinary medical monitoring and management of patients with BMP2-related skeletal dysplasia spectrum.
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Osteocondrodisplasias , Peixe-Zebra , Animais , Humanos , Peixe-Zebra/genética , Estudos Retrospectivos , Diferenciação Celular , Osteogênese/genética , Proteínas Morfogenéticas Ósseas , Proteína Morfogenética Óssea 2/genéticaRESUMO
OBJECTIVE: Non-small cell lung cancer (NSCLC) ranks high in the incidence of malignant tumors, with limited treatment options and poor prognosis. Ferroptosis is a newly discovered cell death mechanism based on iron and reactive oxygen species (ROS). The role of ferroptosis-related long non-coding RNAs (lncRNAs) and associated prognostic mechanisms in NSCLC require investigation. MATERIALS AND METHODS: We constructed a prognostic multi-lncRNA signature based on ferroptosis-related differentially expressed lncRNAs in NSCLC. The levels of ferroptosis-related lncRNA in normal lung cells and lung adenocarcinoma cells were verified by RT-PCR. RESULTS: We identified eight differentially expressed lncRNAs associated with NSCLC prognosis. The expression of AC125807.2, AL365181.3, AL606489.1, LINC02320, and AC099850.3 was upregulated, while SALRNA1, AC026355.1, and AP002360.1 were downregulated in NSCLC cell lines. Kaplan-Meier analysis showed that a high-risk patient group was associated with poor NSCLC prognosis. A risk assessment model based on ferroptosis-related lncRNAs was superior to NSCLC prognosis based on traditional clinicopathological features. Gene Set Enrichment Analysis (GSEA) identified immune- and tumor-related pathways in low-risk group patients. In addition, The Cancer Genome Atlas (TCGA) showed that T cell function during APC co-inhibition, APC co-stimulation, chemokine receptor (CCR), MHC class I, parainflammation, T cell co-inhibition, and check-point expression differed significantly between low- and high-risk groups. M6A-related mRNA comparisons between these groups also revealed significant differences in ZC3H13, RBM15, and METTL3 expression. CONCLUSIONS: Our new model of lncRNA-associated ferroptosis effectively predicted NSCLC prognoses.
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Carcinoma Pulmonar de Células não Pequenas , Ferroptose , Neoplasias Pulmonares , RNA Longo não Codificante , Humanos , Carcinoma Pulmonar de Células não Pequenas/genética , RNA Longo não Codificante/genética , Ferroptose/genética , Prognóstico , Neoplasias Pulmonares/genética , Imunoterapia , MetiltransferasesRESUMO
Objective: To investigate the impact of the iris and ciliary body morphology on the central vault after phakic posterior chamber intraocular lens (pIOL) implantation. Methods: This research was based on the retrospective analysis of 123 patients (123 eyes) who underwent pIOL implantation in the Department of Ophthalmology, Affiliated Hospital of Qingdao University between June 2018 and June 2020. The anterior segment structure was observed with an ultrasound biomicroscope before surgery, and all morphological parameters of the iris and ciliary body were measured manually using the ImageJ software, including iris span (IS), iris convexity (IC), iris-ciliary body contact distance (ICCD) and iris-lens contact distance (ILCD). The ICCD was divided into Q1, Q2 and Q3 groups according to the equidistant distance of 0.36 mm. The lens thickness was measured with the IOLMaster. The horizontal corneal diameter and anterior chamber depth were measured using the Pentacam. The central vaults were measured by optical coherence tomography at 1 week, 3 months and 1 year after surgery. The relationships between vault values and preoperative parameters of the anterior segment were evaluated using the Pearson correlation analysis, Spearman correlation analysis, and multiple linear regression. The repeated measures ANOVA was applied to identify changes of vault values over time. Results: The mean values of the vaults at 1 week, 3 months and 1 year after surgery were (723±265) µm, (642±255) µm and (613±280) µm, respectively. The difference among them was statistically significant (F=50.143, P<0.001). The vaults continued to decline within 1 year after pIOL implantation, and the total decline was (122±86) µm. The vaults declined by (69±98) µm from postoperative 1 week to 3 months and by (52±54) µm from postoperative 3 months to 1 year. The regression formula showed that the pIOL size and ILCD were positively related with the vault, while the LT, IS and IC were negatively related with the vault at 1 week postoperatively (adjusted R²=0.404, P<0.001). The pIOL size and ILCD were positively related with the vault, while the IS and IC were negatively related with the vault at 3 months postoperatively (adjusted R²=0.342, P<0.001). The pIOL size was positively related with the vault, while the IS and IC were negatively related with the vault at 1 year postoperatively (adjusted R²=0.661, P<0.001). The vault values were higher in group Q3 compared to group Q1 at every timepoint, and the vault value was higher in group Q2 compared to Q1 at 1 year postoperatively. Conclusions: In the early postoperative period, eyes with a larger pIOL, shorter iris span, longer contact distance between the iris and ciliary body, and longer contact distance between the iris and lens were associated with a higher rate of excessive vaults. Meanwhile, eyes with a thicker lens and larger iris reverse convexity were more likely to obtain insufficient vaults. Within one year after surgery, the pIOL size, IS, IC and ICCD continued to impact on the vault. The ICCD, ILCD and IC can reflect the posterior chamber volume and change the haptic location and force, thus affecting the vault.
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Miopia , Lentes Intraoculares Fácicas , Corpo Ciliar , Humanos , Iris , Implante de Lente Intraocular , Miopia/cirurgia , Estudos RetrospectivosRESUMO
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-â . 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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Imageamento por Ressonância Magnética , Miosite , Criança , Feminino , Glucocorticoides , Humanos , Lactente , Miosite/tratamento farmacológico , RituximabRESUMO
OBJECTIVES: Diurnal temperature range (DTR) is an important indicator of global climate change. Many epidemiological studies have reported the associations between high DTR and human health. This study investigated the association between DTR and hospitalisations for ischaemic stroke in Hefei, China. STUDY DESIGN: This is an ecological study. METHODS: Data of daily hospital admissions for ischaemic stroke and meteorological variables from 1 January 2009 to 31 December 2017 were collected in Hefei, China. A generalised additive model combined with distributed lag non-linear model was used to quantify the effects of DTR on ischaemic stroke. The interactive effect between DTR and temperature was explored with a non-parametric bivariate response surface model. RESULTS: High DTR was associated with hospitalisations for ischaemic stroke. The adverse effect of extremely high DTR (99th percentile [17.1 °C]) occurred after 8 days (relative risk [RR] = 1.021, 95% confidence interval [CI] = 1.002, 1.041) and the maximum effect appeared after 12 days (RR = 1.029, 95% CI = 1.011, 1.046). The overall trend of the effect of DTR on ischaemic stroke was decreasing. In addition, there was a significant interactive effect of high DTR and low temperature on ischaemic stroke. CONCLUSIONS: This study suggests that the impact of high DTR should be considered when formulating targeted measures to prevent ischaemic stroke, especially for those days with high DTR and low mean temperature.
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Temperatura Baixa/efeitos adversos , Hospitalização/estatística & dados numéricos , Temperatura Alta/efeitos adversos , AVC Isquêmico/terapia , Idoso , China/epidemiologia , Mudança Climática , Feminino , Humanos , AVC Isquêmico/epidemiologia , Masculino , Pessoa de Meia-Idade , RiscoRESUMO
Objective: To investigate the clinical characteristics of R403C variant in DNM1L gene caused encephalopathy, lethal, due to defective mitochondrial peroxisomal fission 1 (EMPF1). Methods: The clinical data of three patients, who carried R403C variant in the DNM1L gene, diagnosed at Xiangya Hospital from February 2018 to February 2020 were retrospectively summarized. Literature reviewing was performed by taking "DNM1L" or "encephalopathy, lethal, due to defective mitochondrial peroxisomal fission 1" as keywords for searching in online Mendelian inheritance in man (OMIM), PubMed, China national knowledge infrastructure (CNKI), and Wanfang data knowledge service platform up to July 2020. And the clinical manifestation, laboratory examination, imaging, treatment, and prognosis were reviewed. Results: Case 1, a 7-year-old boy, developed seizures after a 9-day course of cough without fever. The seizures manifested as generalized tonic-clonic seizures (GTCS) and soon converted to focal status epilepticus (EPC) or focal myoclonus, which were resistant to multi-anti-epileptic drugs combined with sedative drugs. The boy died at the 2nd week after seizure onset. Case 2, also a 7-year-old boy, developed seizures after a 10-day history of amygdalitis. The seizures manifested as focal to generalized tonic-clonic seizure and then converted to EPC or focal myoclonus. And all seizures showed poor responses to multi-anti-epileptic drugs combined with sedative drugs, ketogenic diet, and methylprednisolone treatment. The boy died after 1 month's treatment. Case 3, a 3-year and 5-month old girl, had seizures onset after a 2-week course of viral pneumonia. The seizures onset manifested as focal clonic seizure and converted to EPC, shortly. She was resistant to multi-anti-epiletic drugs combined with sedative drugs and ketogenic treatment. The girl died 3 months afte seizure onset. All of their images showed multifocal T1 low, T2, fluid attenuated inversion recovery, and diffusion-weighted imaging high signal lesions among the brain, and diffuse brain atrophy in case 3. The blood metabolic and cerebrospinal-fluid immunological assays were normal. Genetic analysis suggested a de novo, heterozygous, NM_012062.4: c.1207C>T, p.R403C variant in the DNM1L gene. According to their clinical manifestations, all of them were diagnosed with EMPF1. Literature review included 11 patients carrying this variant in the world. Summarizing the 14 cases, 8 cases had an infectious history before seizure onset, 8 cases had mild or moderate development delay. All of 14 cases had seizures, and the forms mainly included EPC (n=9), focal myoclonus (n=6), GTCS (n=5) and focal clonic seizures (n=4). All of them were refractory, and no effective anti-epileptic drugs were recommended. Early-stage cranial magnetic resonance imaging results showed multiple intracranial focal lesions (n=10), including thalamus (n=7), hippocampus (n=5), basal ganglia (n=4), frontal lobe (n=3), and temporal lobe (n=2). As the disease progressed, the brain manifested as diffused progressive atrophy (n=10). Five of the 14 cases died at reported age. Conclusions: R403C variant in the DNM1L gene can cause mitochondrial fission dysfunction. Patients carrying this variant may manifest as refractory status epilepticus with or without mild-infection indction, development regression and brain atrophy.
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Encefalopatias , Dinâmica Mitocondrial , Encefalopatias/genética , Criança , China , Dinaminas , Eletroencefalografia , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Convulsões/genéticaRESUMO
AIM: To investigate the imaging features of synovial chondromatosis of the temporomandibular joint (TMJ), which is a rare benign arthropathy with cartilaginous proliferation. MATERIALS AND METHODS: Computed tomography and magnetic resonance imaging examinations of 34 patients with histopathologically confirmed primary synovial chondromatosis of the TMJ were reviewed retrospectively. Imaging features including the lesion epicentre, destruction/sclerosis of surrounding bone, calcification, periosteal reaction, osteophyte, lesion size, and joint space dimensions were assessed. RESULTS: Thirty-one of thirty-four patients (91.2%) showed the superior joint space as the lesion epicentre. For the mandibular condyle, more than one-third of patients (14/34; 41.2%) showed no destruction, and more than half of patients (19/34; 55.9%) showed no sclerosis. Conversely, >70% of patients showed destruction and sclerosis of the articular eminence/glenoid fossa, while >80% of patients (28/34; 82.4%) presented with various calcifications, including the ring-and-arc (9/34; 26.5%) and popcorn (13/34; 38.2%) types. The mean joint space on the affected side was significantly larger than that of the unaffected side (p<0.001). More than three-fourths of patients (76.9%) experienced no interval increase in lesion size during an average of 1.6 years of follow-up. CONCLUSION: Synovial chondromatosis of the TMJ demonstrated several imaging features, including the lesion centre being located in the superior joint space, resultant articular eminence/glenoid fossa-oriented bone changes, ring-and-arc and popcorn calcification, joint space widening, and self-limiting growth. These imaging features may be helpful in differentiating synovial chondromatosis from other lesions of the TMJ.
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Condromatose Sinovial/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Transtornos da Articulação Temporomandibular/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Articulação Temporomandibular/diagnóstico por imagem , Adulto JovemRESUMO
BACKGROUND: Red cell distribution width (RDW) values increase in many diseases and conditions, including sepsis. However, the relationship between RDW fluctuation and prognosis in patients with sepsis or the likely morbidity associated with sepsis-induced disseminated intravascular coagulation (DIC) has not been previously investigated. This study examined the association between dynamic changes to RDW and DIC occurrence in sepsis, as well as the prognostic significance of changes to RDW during hospital stay in patients with sepsis. METHODS: We collected baseline emergency department admissions' data. All RDW values recorded during hospitalization of patients with sepsis were combined to calculate RDW standard deviation (RDW-SD) and the increase rate of RDW; we also collected data on coagulation indicators. The endpoints were 28-day mortality and sepsis-related DIC morbidity. RESULTS: Of 232 patients included in our analysis, 66 were diagnosed with DIC (28.4%), and 86 (37.1%) died within 28 days. The RDW-SD and the increase rate of RDW were independent risk factors for 28-day mortality and sepsis-associated DIC morbidity, respectively. The DIC occurrence and mortality rate increased continually with an increasing rate of RDW of at least 6%. CONCLUSIONS: The RDW-SD and RDW increase rate shown in the study as the indicators of RDW fluctuation can help predict sepsis-related DIC morbidity and prognosis in patients with sepsis.
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Coagulação Intravascular Disseminada , Sepse , Coagulação Intravascular Disseminada/epidemiologia , Índices de Eritrócitos , Humanos , Morbidade , Prognóstico , Estudos Retrospectivos , Sepse/complicaçõesRESUMO
AIM: To investigate the imaging features of chondrosarcoma of the temporomandibular joint (TMJ) and review the literature. MATERIALS AND METHODS: Computed tomography (CT), magnetic resonance imaging (MRI), and integrated positron-emission tomography (PET)/CT images of nine patients with histopathologically confirmed chondrosarcoma of the TMJ were reviewed retrospectively. Imaging features regarding the direction of lesion growth, bone destruction, infiltration into the tendon of the lateral pterygoid muscle (LPM) in the pterygoid fovea, enhancement pattern, calcification, periosteal reaction, markedly hyperintense T2 signal area, and qualitative PET signal intensity were evaluated. RESULTS: Seven of nine patients (77.8%) presented with lesion growth that was outward from the medulla of the mandibular condyle. Infiltration into the tendon of LPM in the pterygoid fovea was observed in all cases, and 77.8% (7/9) of them demonstrated >50% infiltration. All the lesions showed a mixed peripheral and internal enhancement, and revealed a markedly hyperintense T2 signal intensity area, which showed no enhancement. Although five of nine cases demonstrated higher FDG uptake compared with that of the liver, the other four cases showed less FDG uptake than that of the liver. CONCLUSION: Chondrosarcoma of the TMJ demonstrated several imaging features, including outward growth from the mandibular condyle, resultant infiltration into the tendon of LPM in the pterygoid fovea, various patterns of internal enhancement, and a markedly hyperintense T2 signal intensity area. These imaging features may be helpful to differentiate chondrosarcoma from other lesions of the TMJ.
Assuntos
Condrossarcoma/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Transtornos da Articulação Temporomandibular/diagnóstico por imagem , Articulação Temporomandibular/diagnóstico por imagem , Adolescente , Adulto , Idoso , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Músculos Pterigoides/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
Since this article has been suspected of research misconduct and the corresponding authors did not respond to our request to prove originality of data and figures, "The role of miR-99b in mediating hepatocellular carcinoma invasion and migration, by C.-J. Liu, J.-H. Yang, F.-Z. Huang, J.-H. Yang, C.-P. Liu, X.-H. Mao, W.-M. Yi, X.-B. Shen, C. Peng, M.-F. Chen, B. Jiang, J.-S. Wu, published in Eur Rev Med Pharmacol Sci 2018; 22 (8): 2273-2281-DOI: 10.26355/eurrev_201804_14815-PMID: 29762829" has been withdrawn. The Publisher apologizes for any inconvenience this may cause. https://www.europeanreview.org/article/14815.
RESUMO
BACKGROUND AND OBJECTIVE: A systematic review and meta-analysis of randomized controlled trials (RCTs) was undertaken to evaluate the effect of intrathecal dexmedetomidine (DEX) on the duration of postoperative analgesia, postoperative pain scores and incidences of adverse effects. DATABASES AND DATA TREATMENT: Database search was performed from inception until January 2019. All RCTs analysing acute postoperative pain characteristics after intrathecal DEX administration in adults undergoing spinal anaesthesia for elective surgery were included. The primary outcome was postoperative analgesic duration, defined as the time to first analgesic request. The secondary outcomes included pain scores at 6, 12 and 24 postoperative hours and rates of hypotension, bradycardia, shivering and postoperative nausea and vomiting. RESULTS: Twenty-four studies comprising a total of 1,460 patients were included. Postoperative analgesic duration was prolonged with intrathecal DEX compared to placebo, with a pooled mean difference (MD) of 191.3 min (95% CI 168.8-213.8). Patients who received intrathecal DEX reported lower Visual Analogue Scale scores at 24 postoperative hours compared with those patients receiving placebo, with a MD (95% CI) of -1.05 (-1.89 to -0.20, p = 0.02). There were no differences in the incidence of adverse effects, except for a lower rate of postoperative shivering in the intrathecal DEX group (pooled relative risk 0.58, 95% CI 0.34-0.98, p = 0.04). CONCLUSIONS: Compared to placebo, intrathecal DEX prolonged postoperative analgesic duration, reduced 24-hr pain intensity and reduced the incidence of shivering without an increase in other adverse effects. SIGNIFICANCE: The analgesic role of intrathecal DEX is promising due to its ability to significantly increase postoperative analgesic duration when compared with placebo. Its usage can be considered for patients undergoing surgeries with significant postoperative pain, particularly those intolerant of systemic analgesia. However, the optimal dose for various surgeries as well as its long-term neurological effects warrants further studies.