Neurofibromatosis Type 1 （NF1） is an autosomal dominant hereditary neurological disorder. One of the typical manifestations of NF1 is neurofibroma, which can develop gradually over time. When the volume exceeds 100 cm², it is referred to as giant neurofibroma, representing a tumor-like proliferation of Schwann cells within the nerve fiber sheath. The Department of Otolaryngology at the Affiliated Hospital of Guangdong Medical University received a rare case involving a patient with giant neurofibromatosis affecting the maxillofacial region, neck, and chest. The patient underwent successful surgical treatment with the collaboration of various medical disciplines.
Humans ; Head and Neck Neoplasms/surgery* ; Neck ; Neurofibromatoses ; Neurofibromatosis 1/surgery* ; Thoracic Neoplasms/surgery*

Objective:To explore the effects of online mindfulness-based stress reduction(MBSR)on the anxiety and depression status,and quality of life in the caregivers of patients with severe mental disorders.Methods:Ninety-three caregivers for patients with schizophrenia or bipolar disorder,who were hospitalized in Yunnan Provincial Mental Hospital in March 2021,were enrolled and randomly divided into control group(n=47)and MBSR intervention group(n=46).Both groups received basic health education and rehabilitation skill training,while the intervention group received additional online MBSR for 8 weeks.The anxiety and depression status,and the quality of life of the caregivers were evaluated by Self-rating Anxiety Scale(SAS),Self-rating Depression Scale(SDS)and the 36-item Short Form Health Survey(SF-36)before and 8 weeks after intervention,respectively.Results:Thirteen caregivers dropped out of the study,and 80 subjects(40 in each group)were included in the final analysis.At the baseline,there were no significant differences in SAS,SDS and SF-36 scores between two groups(all P>0.05).Compared with the baseline,SAS and SDS scores in the intervention group significantly decreased after 8 weeks of intervention(both P<0.01)and were significantly lower than those in the control group(both P<0.01).There were no significant changes in the control group(all P>0.05).Except the physiological function dimension,the total score and the scores of each dimension of SF-36 in the intervention group were significantly increased after 8-week intervention(all P<0.05),and were significantly higher than those in the control group(all P<0.01).There were no significant changes in the control group before and after intervention(all P>0.05).Conclusion:Online MBSR can reduce the anxiety and depression levels,improve the quality of life in the caregivers of patients with severe mental disorders.

Objective:To study the factors influencing repeatedly hospitalization in patients with acute pancreatitis (AP), and to analyse the predictive value of triglyceride for repeated hospitalization.Methods:The clinical data of 1 958 patients with AP treated at the First Affiliated Hospital of Anhui University of Science and Technology from January 2012 to April 2022 were analyzed. Of 1 733 AP patients who were enrolled, there were 1 000 males and 733 females, with mean ± s. d age being (49.4±16.4) years. Patients were grouped based on their ID numbers to determine their number(s) of hospitalization. Those who were admitted only once were included in the initial hospitalization group ( n=1 030), and those who were admitted twice or more were included in the repeated hospitalization group ( n=703). The factors influencing repeated hospitalization were analyzed by univariate analysis and multivariate logistic regression analysis. The predictive value of triglyceride for repeated hospitalization was evaluated by receiver operating characteristic (ROC) curve. Results:Multivariate logistic regression analysis showed that hypertriglyceridemia ( OR=1.445, 95% CI: 1.144-1.825, P=0.002) and biliary causes ( OR=3.184, 95% CI: 1.978-5.125, P<0.001) were independent risk factors for repeated hospitalization. When triglyceride <10.9 mmol/L, the prediction of AP patients without repeated hospitalization was 90.6%. The area under the ROC curve was 0.589, and the Yoden index was 0.170. Conclusion:Hypertriglyceridemia was risk factor for repeat hospitalization in AP patients and the efficacy of triglyceride in predicting repeat hospitalization in AP patients was good.

Despite complications were significantly reduced due to the popularity of percutaneous coronary intervention (PCI) in clinical trials, reperfusion injury and chronic cardiac remodeling significantly contribute to poor prognosis and rehabilitation in AMI patients. We revealed the effects of HSP47 on myocardial ischemia-reperfusion injury (IRI) and shed light on the underlying molecular mechanism. We generated adult mice with lentivirus-mediated or miRNA (mi1/133TS)-aided cardiac fibroblast-selective HSP47 overexpression. Myocardial IRI was induced by 45-min occlusion of the left anterior descending (LAD) artery followed by 24 h reperfusion in mice, while ischemia-mediated cardiac remodeling was induced by four weeks of reperfusion. Also, the role of HSP47 in fibrogenesis was evaluated in cardiac fibroblasts following hypoxia-reoxygenation (HR). Extensive HSP47 was observed in murine infarcted hearts, human ischemic hearts, and cardiac fibroblasts and accelerated oxidative stress and apoptosis after myocardial IRI. Cardiac fibroblast-selective HSP47 overexpression exacerbated cardiac dysfunction caused by chronic myocardial IRI and presented deteriorative fibrosis and cell proliferation. HSP47 upregulation in cardiac fibroblasts promoted TGFβ1-Smad4 pathway activation and Smad4 deubiquitination by recruiting ubiquitin-specific peptidase 10 (USP10) in fibroblasts. However, cardiac fibroblast specific USP10 deficiency abolished HSP47-mediated fibrogenesis in hearts. Moreover, blockage of HSP47 with Col003 disturbed fibrogenesis in fibroblasts following HR. Altogether, cardiac fibroblast HSP47 aggravates fibrosis post-myocardial IRI by enhancing USP10-dependent Smad4 deubiquitination, which provided a potential strategy for myocardial IRI and cardiac remodeling.

Objective To investigate the effects of p38 mitogen-activated protein kinase (MAPK) short hair RNA (shRNA) delivered by lentiviral vectors (pGLV) on cardiac function after myocardial infarction (MI) in aldosterone overload rats and to explore the mechanism.Methods Aldosterone overload rat myocardial infarction model was obtained by ligating the left anterior descending coronary artery.The pGLV-shRNA was constructed,sequenced and injected into rats via tail vein.Rats were divided into 3 groups:pGLV-shRNA group (n=6),pGLV-shRNA-NC group (n=6,contained a nonsense shRNA) and the sham-operation group (n=6).Cardiac function was measured by cardiac ultrasound.Apoptosis was assessed by transferase (TdT)-mediated biotin-16-dUTP nick-end labelling (TUNEL).The p38 MAPK mRNA expression was analyzed by RT-PCR.The protein expressions of p38 MAPK and caspase-3 were detected by Western blot.Results Compared with the sham-operation group,cardiac systolic function was reduced and myocardial apoptosis index was significantly increased [(31.26 ± 4.45) ％ vs.(15.20 ± 2.18) ％,P ＜ 0.01] in pGLV-shRNA-NC group.The mRNA and protien expressions of p38MAPK and caspase 3 protein expression were significantly increased in pGLV-shRNA-NC group (all P＜0.01).Compared with pGLV-shRNA-NC group,cardiac function was improved,myocardial cell apoptosis index was reduced [(22.35±3.59)％ vs.(31.26±4.45)％,P＜0.05],and the mRNA and protien expressions of p38MAPK and caspase 3 protein expression were decreased in pGLV-shRNA group (all P＜0.05).Conclusions Cardiac dysfunction is associated with p38MAPK-mediated myocardial apoptosis in aldosterone overload MI rats.pGLV-shRNA may inhibit cardiomyocyte apoptosis and improve postMI cardiac function.

Objective To figure the clinical characteristics and genetic bases of Dentinogenesis imperfecta type Ⅱ in a large Mongolian family.Methods Systematic analysis for this family was carried out using clinical detection. Results Affected individuals of Dentinogenesis imperfecta type Ⅱ were consecutively found in a five-generation family. The morbidity of the offsprings is nearly 1/2 and no sexual difference is found. The analysis of clinical features as well as dental x-ray check showed specific finding that were not found in other families. Conclusion Dentinogenesis imperfecta type Ⅱ in this Mongolian family pertains to autosomal dominant disorder with high genetic heterogeneity in clinical phenotype. Further study is warranted to identify the association of this heterogeneity with lifestyle or genetic information.