Fatal familial insomnia,an autosomal dominant prion disease,is rare.We reported the clin-ical symptoms,examination results,diagnosis,treatment,and prognosis of a patient who was diagnosed with fatal familial insomnia.Furthermore,we described the unique clinical manifestations that involuntary movements and laryngeal stridor were significantly correlated with postural changes,aiming to provide reference for the clini-cal diagnosis,treatment,and research of the disease in the future.

Primary central nervous system vasculitis(PACNS)is a vasculitic disorder affecting small to medium-sized blood vessels primarily in the central nervous system,involving the brain,spinal cord,and me-ninges.Tumor-like PNCAS,a rare subtype of PACNS,is often misdiagnosed as intracranial malignancy,and that with spinal cord involvement is even more uncommon.The lack of specific clinical symptoms and imaging manifestations poses a challenge to the diagnosis of PACNS.This report presents a case of tumor-like PACNS with spinal cord involvement based on the pathological evidence,aiming to enrich the knowledge about this condition.

Objective To analyze the clinical features of 17 patients with primary angiitis of the central nervous system(PACNS)and thus facilitate the early diagnosis and treatment,reduce the recurrence and mortal-ity,and improve the prognoses of this disease.Methods We collected the data of patients with PACNS diag-nosed by brain biopsy from January 2009 to June 2023 and analyzed their clinical presentations,laboratory and imaging manifestations,electrophysiological and pathological changes,and treatment regimens and prognosis.Results The 17 patients diagnosed with PACNS via brain biopsy included one child and 16 adults.The subtyp-ing results showed that 10,2,3,2,1,and 1 patients had tumorous,spinal cord-involved,angiography-posi-tive,rapidly progressive,hemorrhagic,and amyloid β-related PACNS,respectively.Eleven(64.7％)of the patients were complicated with secondary epilepsy.All the patients exhibited abnormal manifestations in head MRI,with 94.1％showing lesions with uneven enhancement around the lesions or in the leptomeninges.Mag-netic resonance angiography revealed large vessel abnormalities in 3 patients,and spinal cord involvement was observed in 2 patients.Histopathological typing revealed 7(43.7％)patients with lymphocytic vasculitis and 5(31.2％)patients with necrotizing vasculitis.Eleven patients were treated with glucocorticoids and cyclophospha-mide,which resulted in partial lesion disappearance and symptom amelioration in 6 patients upon reevaluation with head MRI after 3 months of maintenance therapy.Two,1,and 3 patients experienced rapid disease progres-sion,death,and recurrence within 1 year,respectively.Three patients showed insensitivity to hormonotherapy and residual disabilities.Two patients received rituximab after relapse and remained clinically stable during a fol-low-up period of 0.5-1 year.Conclusions Tumorous PACNS was more prone to epilepsy,mainly occurring in males.The most common histopathological type was necrotizing vasculitis,which responded to hormonotherapy and had favorable outcomes.Therefore,for the young patients with epilepsy and intracranial tumorous lesions,the possibility of PACNS should be considered.Spinal cord involvement in PACNS was often located in the thorac-ic and cervical cords,suggesting a poorer prognosis.Electromyography commonly revealed neural conduction ab-normalities in the anterior horn or roots,providing clues for differential diagnosis.For suspected spinal cord in-volvement,comprehensive electromyography is recommended.Rapidly progressive PACNS often presented infrat-entorial lesions,such as lesions in the pons and medulla,with a higher mortality rate.Hemorrhagic PACNS was rare,and a multifocal hemorrhagic lesion with enhancement in the intracranial region,particularly in young pa-tients,should raise suspicion.For the patients with recurrent or progressive disease,rituximab is a recommended therapeutic option.

Objective:To investigate the effect of astragaloside IV (AS-IV) regulating the signal axis of stromal cell-derived factor-1α (SDF-1α)/CXC chemokine receptor 4 (CXCR4) on the mobilization of bone marrow endothelial progenitor cells (EPCs) to peripheral blood in diabetes skin ulcer (DSU) rats.Methods:Twenty four SPF grade male Sprague Dawley (SD) rats were selected to make the model of type 2 diabetes rats by intraperitoneal injection of 30 mg/kg 1% (plastid ratio) streptozotocin, and then round full-thickness skin with a diameter of 2 cm was cut on both sides of the waist and back to make the skin ulcer model of diabetes rats. After that, they were randomly divided into AS-IV group (50 mg/kg AS-IV), blocker group (50 mg/kg AS-IV+ 5 mg/kg AMD3100) and model group. At the same time, a blank group ( n=8) was set up, The drug was administered via intraperitoneal injection, and the model group and blank group were treated with 0.9% NaCl of equal volume. On the 10th day, peripheral blood, femoral bone marrow, and wound neovascularization tissues of rats were collected. The number of EPCs in peripheral blood of each group of rats was measured by flow cytometry, and the protein expression of SDF-1α and CXCR4 in peripheral blood, femoral bone marrow, and wound neovascularization tissues of rats was detected by enzyme-linked immunosorbent assay (ELISA); At the same time, the wound healing rates of each group were tested. Results:On the 10th and 21st day after modeling, the wound healing rate of each group of rats was compared. The blank group healed the fastest, while the model group healed the slowest. The AS-IV group had better healing than the model group and the blocker group, and the difference was statistically significant (all P<0.05). On the 10th day after modeling, the positive rates of peripheral blood EPCs in the white group, AS-IV group, and blocker group were significantly higher than those in the model group (all P<0.05), while the positive rates of peripheral blood EPCs in the blocker group were significantly lower than those in the AS-IV group (all P<0.05). On the 10th day after modeling, the protein expression of SDF-1α and CXCR4 in the wound, serum, and bone marrow of the model group was the lowest, while the protein expression in the blank group was the highest (all P<0.05). The protein expression of SDF-1α and CXCR4 in the wound, serum, bone marrow of the AS-IV group was significantly higher than that of the blocker group and model group, and the difference was statistically significant (all P<0.05). Conclusions:Astragaloside IV can promote the mobilization and migration of endothelial progenitor cells from bone marrow to peripheral blood in diabetes ulcer rats by regulating SDF-1α/CXCR4 signal axis, and can participate in angiogenesis of diabetes ulcer wounds as seed cells to promote the healing of diabetes skin ulcers.

Objective:To investigate the correlation between the annual professional proficiency test results and the theoretical examination score of completion assessment in standardized residency training, as well as the value of the annual professional proficiency test results in predicting whether a resident passes the theoretical examination of completion assessment.Methods:The residents who participated in the annual professional proficiency test of residency training in Affiliated Hospital 2 of Nantong University in 2019-2021 and the completion assessment of residency training in 2020-2022 were selected as subjects, and related data were collected, including sex, education background, personnel type, training specialty, the results of annual professional proficiency test, and the theoretical examination score of completion assessment. According to whether the resident passed the theoretical examination of completion assessment, they were divided into passed group and failed group. SPSS 19.0 was used to perform the chi-square test, the independent samples t-test, and the binary logistic regression analysis; the Pearson correlation coefficient was used for correlation analysis; the sensitivity analysis was represented by ROC curve. Results:Compared with the residents who passed the theoretical examination of completion assessment, the residents who did not pass the examination had a significant reduction in the proportion of the residents from our hospital and a significant increase in the proportion of the residents commissioned by foreign institutions ( χ 2=7.00, P=0.008). The passed group had a significantly higher national percentile of annual professional proficiency test score than the failed group (43.46%±26.61% vs. 23.40%±18.71%, t=6.02, P<0.001). The national percentile of annual professional proficiency test score was positively correlated with the theoretical examination score of completion assessment ( r=0.43, P<0.05). The source of residents commissioned by foreign institutions and the low percentile of annual professional proficiency test score were independent risk factors for failing the theoretical examination of completion assessment ( P=0.020 and P<0.001). The national percentile of annual professional proficiency test score had an area under the ROC curve of 0.73 (95% CI: 0.65-0.80) in predicting the outcome of theoretical examination and had a certain predictive value with a cut-off value of 15.1%. Conclusions:In addition to strengthening homogenization and professional base management for residency training, it is necessary to make full use of the results of annual professional proficiency test in standardized residency training and timely check the professional knowledge of the residents whose a national percentile of <15.1%, so as to effectively improve the pass rate of theoretical examination and the quality of training.

Sarcopenia, which is characterized by progressive and generalized loss of skeletal muscle mass and strength, has been well described to be associated with numerous poor postoperative outcomes, such as increased perioperative mortality, postoperative sepsis, prolonged length of stay, increased cost of care, decreased functional outcome, and poorer oncological outcomes in cancer surgery. Multimodal prehabilitation, as a concept that involves boosting and optimizing the preoperative condition of a patient prior to the upcoming stressors of a surgical procedure, has the purported benefits of reversing the effects of sarcopenia, shortening hospitalization, improving the rate of return to bowel activity, reducing the costs of hospitalization, and improving quality of life. This review aims to present the current literature surrounding the concept of sarcopenia, its implications pertaining to colorectal cancer and surgery, a summary of studied multimodal prehabilitation interventions, and potential future advances in the management of sarcopenia.

Objective:To investigate the clinical efficacy of modified Guipi Decoction combined with omeprazole in the treatment of acute non-variceal upper gastrointestinal bleeding (ANVUGIB) with failure of the spleen to control blood vessels syndrome.Methods:Prospective cohort study. A total of 120 patients from January 2018 to December 2021 Taihe County Hospital of Traditional Chinese Medicine with ANVUGIB of failure of the spleen to control blood vessels syndrome were selected, and the patients were divided into observation group and control group according to the random number table method, with 60 cases in each group. The control group was treated with a large dosage of proton pump inhibitor (omeprazole injection was injected intravenously first, and then omeprazole enteric coated tablets were taken); the observation group took Guipi Decoction on the basis of the control group, and both groups were treated for 7 days. TCM syndrome score, Hemoglobin (Hb) and hematocrit (HCT) levels were measured by colorimetry before and after the treatment. BUN was detected by urease glutamate dehydrogenase method. Prothrombin time (PT), activated partial thromboplastin time (APTT) and fibrinogen (FIB) levels were detected by immunoturbidimetry. The adverse reactions during treatment were recorded and the clinical efficacy was evaluated.Results:Two patients in the observation group and two patients in the control group dropped out of the study. After treatment, the scores of main symptoms, secondary symptoms and total scores in the observation group were lower than those in the control group ( t values were respectively 10.73, 4.45, 7.98, P<0.05). After treatment, the levels of HCT [(41.25 ± 5.03)% vs. (38.19 ± 5.26)%, t=2.95], Hb [(81.09 ± 5.23) g/L vs. (78.39 ± 5.37) g/L, t=2.74] in the observation group were higher than those in the control group ( P<0.01), and BUN [(4.38±0.96) mmol/L vs. (5.39 ± 1.13) mmol/L, t=5.19] was lower than that in the control group ( P<0.01); PT [(12.48 ± 0.67) s vs. (13.22 ± 0.73) s, t=5.69], APTT [(24.66 ± 2.29) s vs. (27.78 ± 2.04) s, t=7.75] were lower than those in the control group ( P<0.01), and FIB [(3.68 ± 0.62) g/L vs. (3.41 ± 0.74) g/L, t=2.13] level was higher than that in the control group ( P<0.05). The total effective rate of the observation group was 93.1% (54/58), and that of the control group was 79.3% (46/58), with statistical significance ( χ2=4.64, P=0.031). During the treatment, the incidence of adverse reactions in the control group was 3.4% (2/58), while that in the observation group was 1.7% (1/58), without statistical significance ( χ2=0.34, P=0.559). Conclusion:High-dosage omeprazole treatment with the addition of internal administration of Guipi Decoction can significantly improve coagulation function, correct the signs and symptoms associated with insufficient blood volume in the body circulation, improve hemostatic efficiency, and reduce the risk of bleeding in patients with ANVUGIB, without increasing the risk of patient safety with the drug.

Primary angiitis of the central nervous system (PACNS) is a rare autoimmune disease of the central nervous system, and spinal cord involvement is a rare subtype. The imaging findings of this subtype lack specificity; it is easily misdiagnosed, missed diagnosed, enjoying high disease recurrence rate, disability and mortality. Therefore, this article reviews the clinical manifestations, diagnoses and treatments of PACNS patients with spinal cord involvement, in order to increase clinicians' understanding of the disease.

Objective:To explore any changes in the electromyographic (EMG) signals from the deep lumbar multifidus (DM) of patients with chronic low back pain (cLBP).Methods:Twenty-five cLBP patients formed the cLBP group, while twenty-eight healthy counterparts similar in sex, age and education background were chosen as the control group. EMG signals were recorded during maximum isometric voluntary contraction of the DM. Two-way repeated measures analysis of variance was applied to compare the two groups′ signals′ Lempel-Ziv (LZ) complexity values at rest and during the maximum strength, strength endurance and relaxation stages of contraction. Pearson correlation coefficients were computed relating the LZ complexity to pain duration and intensity, as well as to Oswestry disability index (ODI) values in the cLBP group.Results:The cLBP patients reported a mean symptom duration of 5.96±4.69 years, with an average VAS score of 4.00±1.04 and ODI of 17.12±10.49. They reported greater pain intensity during needle insertions, needle removal, muscle contraction and relaxation than the healthy controls. There were significant differences in LZ complexity among the four stages of contraction with all of the subjects. The LZ complexity was significantly lower in the maximum strength and strength endurance states, but higher in the relaxation after contraction states in the cLBP group. Pain duration was negatively correlated with the nonlinear index of DM during contraction.Conclusion:Continuous pain stimulation will affect the coordinated control of the deep multifidus muscle, leading to decreased control of core muscles via the central nervous system. That provides insight into the mechanisms underlying activation and coordinated control during chronic pain.

Objective:To survey the cognition of general practice residency training and the willingness of teaching among specialists.Methods:A questionnaire survey was conducted among 221 specialists from 24 departments in Nantong First People's Hospital from May 2021 to June 2021 to investigate the their cognition of general practice residency training program and the teaching willingness.Results:Total 221 questionnaires were distributed and 185 valid ones were retrieved with a response rate of 83.70%. The results showed that 49 specialists (26.49%) well knew the national general practice training policy, 70 (37.84%) knew the most, 52 (28.11%) knew basically, 11 (5.95%) knew little, and 3 (1.62%) did not know at all. Meanwhile, 44 specialists (23.78%) well knew the hospital incentive policies about general practice education, 62 (33.51%) knew the most, 57 (30.81%) knew basically, 18 (9.73%) knew little, and 4 (2.16%) did not know at all. Whether they holding the teaching certificate of general practice was significantly associated with the cognition of national general practice training policy (χ2=14.28, P=0.003) and with their knowledge of residency training program (χ2=16.79, P=0.001), but not associated with knowing the hospital-level incentive policy (χ2=8.18, P=0.075). A total of 170 (91.89%) participants were willing to be clinical teachers of general practice. The reasons for the willingness of teaching were as following: learning more from the teaching in 161 participants (94.71%), expanding sources of patients from rural areas in 102 (60.00%), facilitating promotion in 77 (45.29%), and others in 30 (17.60%). Among 62 specialists holding teaching certificate, 60 (96.77%) were willing to teach general practice residents; while among 123 specialists without teaching certificate, 110 (89.43%) were willing to teach (χ2=4.92, P=0.027). In all hospital incentive policies, promotion of professional titles was most attractive one (82, 44.32%), followed by performance appraisal (63, 34.05%), priority for in-service training (25, 13.51%), and appraisal for excellence award (15, 8.11%). Conclusions:Strengthening trainings for general practice the faculty is helpful to improve their cognition of the general practice residency training programs. And rational hospital incentive policies can enhance the willingness of specialists to teach general practice residency.