Objective:To summarize the clinical characteristics of focal cerebral arteriopathy (FCA) in children, and to analyze its influencing factor of prognosis.Methods:A retrospective cohort study was conducted. Clinical data from 40 children with FCA who were hospitalized at the Department of Neurology, Beijing Children′s Hospital, Capital Medical University, from September 2015 to August 2024 were collected. A centralized follow-up was conducted in October 2024 via outpatient clinics or the internet. The pediatric stroke outcome measure (PSOM) was used to evaluate their outcomes. Based on the PSOM, the children were further divided into a group with normal neurological function and another group with abnormal neurological function. Differences between groups were analyzed using the Mann-Whitney U test and Fisher exact test. Univariate Logistic regression analysis was performed to identify the influencing factors for neurological outcomes in children with FCA. Results:A total of 40 children were included, with 20 males and 20 females, and the onset age of 9.2 (6.8, 12.5) years. Among them, 12 cases (30%) had a history of varicella within 1 year before onset. There were 23 cases (58%) presenting with transient ischemic attack (TIA) or recurrent fluctuating symptoms of onset, while 3 cases (8%) developed progressive stroke within the first month of onset. The M1 segment of the middle cerebral artery was the most commonly affected vascular site, with a total of 16 cases (40%). Arterial occlusion occurred in 8 cases (20%). Lumbar puncture was completed in 36 children, and white blood cell counts in cerebrospinal fluid was increased in 6 cases. All 23 patients who completed magnetic resonance vessel wall imaging (VWI) showed circular enhancement of the arterial wall. A total of 28 patients (70%) received antiplatelet or anticoagulation therapy, and 16 patients (40%) received hormone therapy. At admission, the pediatric National Institute of Health Stroke Scale (PedNIHSS) score was 6.0 (2.0, 8.8) points, which decreased to 0.5 (0, 3.0) points at discharge. The follow-up duration was 1.6 (0.8, 4.9) years, with 1 case lost to follow-up. There was 1 case presenting with recurrence course manifesting as TIA. Among the 39 cases who completed the follow-up, 23 cases (59%) were assessed as neurologically normal by PSOM, while 16 cases (41%) were assessed as neurologically abnormal. Among the 29 cases who completed the imaging review, magnetic resonance angiography (MRA) review in 23 cases indicated stability or improvement in the original arterial stenosis, with 6 cases experiencing transient worsening of arterial stenosis early in the disease course (within 2 months), which later improved. Arterial stenosis progression occurred in 6 cases at the final review of 29 cases who completed the imaging review, with 1 case developing progressive cerebral arteriopathy. The proportion of patients with headache, altered consciousness, and aphasia in the abnormal neurological function group, as well as the PedNISS scores at admission and discharge, were all higher than those in the normal neurological function group (all P<0.05). Univariate Logistic regression analysis revealed that only a PedNISS score>6 points at onset was an influencing factor for abnormal neurological function ( OR=20.58, 95% CI 3.93-107.70, P<0.001). Conclusions:Childhood FCA often presents with fluctuating onset, and the proximal segment of the middle cerebral artery is frequently affected. Progression of arterial stenosis is common within 2 months of the disease course, but clinical progression and new ischemic lesions are uncommon. Most patients have a favorable long-term prognosis. PedNIHSS score>6 points at admission is related to abnormal neurological function outcomes.

Objective:To explore the molecular mechanism by which the methionine adenosyltransferase 2A (MAT2A)/β-catenin/zinc finger E-box binding homeobox 1 (ZEB1)/epithelial-mesenchymal transition (EMT) pathway regulates neural tube defect (NTD) through intracellular S-adenosylmethionine (SAM).Methods:A mouse NTD model was induced using the SAM metabolic disorder inhibitor ethionine. Eighty specific pathogen-free C57BL/6 mice were divided into three groups: a normal group (36 mice), an ethionine group (46 mice), and an ethionine+SAM group (44 mice). Phosphate-buffered saline (PBS), ethionine, and ethionine+SAM were respectively injected intraperitoneally on embryonic day 7.5 (E7.5), and the mice were sacrificed on E10.5. Embryonic tissues were collected, and the morphology of embryos in each group was observed under a stereomicroscope. The interaction between ethionine and MAT2A was analyzed using Autodock software. The expression levels of MAT2A, β-catenin, ZEB1, and EMT-related proteins in the brain tissues of embryos from the three groups were measured using immunofluorescence, immunohistochemistry, Western blotting, enzyme-linked immunosorbent assay (ELISA), and real-time quantitative polymerase chain reaction (RT-qPCR). Variance analysis was used for intergroup comparisons.Results:(1) Autodock analysis results showed that MAT2A binds to ethionine through covalent bonds, exhibiting a complementary effect, thereby accelerating the expression of MAT2A. (2) After successful construction of the NTD model, normal embryos were plump with well-developed brains. NTD embryos showed delayed development, obvious anencephaly, unclosed neural tubes, and asymmetry. (3) The levels of SAM and SAH in the embryonic tissues of the ethionine group were significantly lower than those in the normal group (1 737.56±95.64 vs. 872.33±205.11, and 89.17±9.50 vs. 51.25±9.48, respectively). The SAM and SAH levels in the ethionine+SAM group was 1 197.00±222.27 and 66.61±12.25, significantly higher than those in the ethionine group ( P<0.017). Compared with the normal group and the ethionine+SAM group, the expression of MAT2A mRNA in the embryonic brain tissue of the ethionine group was significantly upregulated (1.00±0.00, 1.59±0.52, and 2.42±0.53, respectively, F=49.64, P<0.001; pairwise comparisons between groups P<0.017). (4) Compared with the normal group, the expression of Ctnnb1 in the ethionine group was reduced, and the expression of Ctnnb1 in the ethionine+SAM group was higher than that in the ethionine group (1.00±0.00, 0.38±0.16, and 0.76±0.10, respectively, F=149.03, P<0.001; pairwise comparisons between groups P<0.017). (5) The expression of ZEB1 in the ethionine group was higher than that in the normal group and the ethionine+SAM group (2.91±0.55, 1.00±0.00, and 1.61±0.20, respectively, F=150.01, P<0.001; pairwise comparisons between groups P<0.017). (6) The expression levels of E-cadherin and Vimentin in the ethionine group were lower than those in the normal group. In contrast, the expression of N-cadherin was higher than that in the normal group. After SAM supplementation, the expression levels of E-cadherin and Vimentin were upregulated, and the expression level of N-cadherin was downregulated (0.54±0.12, 1.00±0.00, and 0.72±0.14, respectively, F=87.44; 0.53±0.17, 1.00±0.00, and 0.76±0.09, F=87.44; 3.11±0.53, 1.00±0.00, and 2.13±0.56, F=95.54; all P<0.001; pairwise comparisons within the same index group P<0.017]). Conclusions:Ethionine promotes the expression of MAT2A, leading to reduced SAM production. Ethionine regulates the level of ZEB1 by increasing MAT2A and inhibits the EMT process to interfere with methionine cycle metabolism, ultimately resulting in NTD.

Objective:To explore and summarize the key points and main treatment measures for successfully treating sever Rickettsia japonica infection. Methods:A 69-year-old female with severe Japanese spotted fever was admitted to Yichang Central People’s Hospital on May 15 in 2024 and was successfully treated. The clinical data of the patient were retrospectively analyzed.Results:The patient was quickly progressed to severe pneumonia，septic shock，dry gangrene of the extremities during the treatment process，and hepatic dysfunction，cardiac dysfunction，renal dysfunction and gastrointestinal hemorrhage were observed. After active anti-infection，anti-shock，continuous renal replacement therapy，and symptomatic supportive treatment，the patient’s condition improved，but the dry gangrene of the limbs was irreversible，ultimately leading to amputation.Conclusion:Timely diagnosis，early and active anti infective treatment，and effective management of various complications are the key to successfully treating cases of Ricketttsia Japonica

Objective To investigate the effects of different use methods of tourniquet on hidden blood loss and knee joint swelling in total knee arthroplasty(TKA),and to explore its potential benefits for postoperative rehabilitation.Methods A prospective study was conducted from March 2018 to March 2023 in Xuzhou Municipal Hospital Affiliated to Xuzhou Medical University,involving 131 patients who underwent TKA.The patients were divided into three groups based on the method of tourniquet use:44 patients routinely used a tourniquet in group A,48 patients only used a tourniquet during the application of bone cement in group B,and 39 patients did not use tourniquet in group C.Operation time,dressing changes,intraoperative blood loss,total blood loss,explicit blood loss,hidden blood loss,percentage of hidden blood loss,postoperative blood transfusion,hemoglobin(Hb),hematocrit(HCT),C-reactive protein(CRP),and creatine kinase(CK)were compared among groups.The pain and functional recovery were evaluated by visual analogue scale(VAS)and knee society clinical rating system(KSS)before surgery,and 3 days,1 month,and 3 months after surgery.The degree of limb swelling and the range of motion of the knee were also compared among groups.Results Group A had shorter operation time and less frequency of postoperative dressing changes than the other two groups(P<0.05).The hidden blood loss and total blood loss in group A were significantly less than those in group C(P<0.05),and the hidden blood loss and total blood loss volume in the three groups from low to high was group A

Objective:To explore the molecular mechanism by which the methionine adenosyltransferase 2A (MAT2A)/β-catenin/zinc finger E-box binding homeobox 1 (ZEB1)/epithelial-mesenchymal transition (EMT) pathway regulates neural tube defect (NTD) through intracellular S-adenosylmethionine (SAM).Methods:A mouse NTD model was induced using the SAM metabolic disorder inhibitor ethionine. Eighty specific pathogen-free C57BL/6 mice were divided into three groups: a normal group (36 mice), an ethionine group (46 mice), and an ethionine+SAM group (44 mice). Phosphate-buffered saline (PBS), ethionine, and ethionine+SAM were respectively injected intraperitoneally on embryonic day 7.5 (E7.5), and the mice were sacrificed on E10.5. Embryonic tissues were collected, and the morphology of embryos in each group was observed under a stereomicroscope. The interaction between ethionine and MAT2A was analyzed using Autodock software. The expression levels of MAT2A, β-catenin, ZEB1, and EMT-related proteins in the brain tissues of embryos from the three groups were measured using immunofluorescence, immunohistochemistry, Western blotting, enzyme-linked immunosorbent assay (ELISA), and real-time quantitative polymerase chain reaction (RT-qPCR). Variance analysis was used for intergroup comparisons.Results:(1) Autodock analysis results showed that MAT2A binds to ethionine through covalent bonds, exhibiting a complementary effect, thereby accelerating the expression of MAT2A. (2) After successful construction of the NTD model, normal embryos were plump with well-developed brains. NTD embryos showed delayed development, obvious anencephaly, unclosed neural tubes, and asymmetry. (3) The levels of SAM and SAH in the embryonic tissues of the ethionine group were significantly lower than those in the normal group (1 737.56±95.64 vs. 872.33±205.11, and 89.17±9.50 vs. 51.25±9.48, respectively). The SAM and SAH levels in the ethionine+SAM group was 1 197.00±222.27 and 66.61±12.25, significantly higher than those in the ethionine group ( P<0.017). Compared with the normal group and the ethionine+SAM group, the expression of MAT2A mRNA in the embryonic brain tissue of the ethionine group was significantly upregulated (1.00±0.00, 1.59±0.52, and 2.42±0.53, respectively, F=49.64, P<0.001; pairwise comparisons between groups P<0.017). (4) Compared with the normal group, the expression of Ctnnb1 in the ethionine group was reduced, and the expression of Ctnnb1 in the ethionine+SAM group was higher than that in the ethionine group (1.00±0.00, 0.38±0.16, and 0.76±0.10, respectively, F=149.03, P<0.001; pairwise comparisons between groups P<0.017). (5) The expression of ZEB1 in the ethionine group was higher than that in the normal group and the ethionine+SAM group (2.91±0.55, 1.00±0.00, and 1.61±0.20, respectively, F=150.01, P<0.001; pairwise comparisons between groups P<0.017). (6) The expression levels of E-cadherin and Vimentin in the ethionine group were lower than those in the normal group. In contrast, the expression of N-cadherin was higher than that in the normal group. After SAM supplementation, the expression levels of E-cadherin and Vimentin were upregulated, and the expression level of N-cadherin was downregulated (0.54±0.12, 1.00±0.00, and 0.72±0.14, respectively, F=87.44; 0.53±0.17, 1.00±0.00, and 0.76±0.09, F=87.44; 3.11±0.53, 1.00±0.00, and 2.13±0.56, F=95.54; all P<0.001; pairwise comparisons within the same index group P<0.017]). Conclusions:Ethionine promotes the expression of MAT2A, leading to reduced SAM production. Ethionine regulates the level of ZEB1 by increasing MAT2A and inhibits the EMT process to interfere with methionine cycle metabolism, ultimately resulting in NTD.

Objective:To explore and summarize the key points and main treatment measures for successfully treating sever Rickettsia japonica infection. Methods:A 69-year-old female with severe Japanese spotted fever was admitted to Yichang Central People’s Hospital on May 15 in 2024 and was successfully treated. The clinical data of the patient were retrospectively analyzed.Results:The patient was quickly progressed to severe pneumonia，septic shock，dry gangrene of the extremities during the treatment process，and hepatic dysfunction，cardiac dysfunction，renal dysfunction and gastrointestinal hemorrhage were observed. After active anti-infection，anti-shock，continuous renal replacement therapy，and symptomatic supportive treatment，the patient’s condition improved，but the dry gangrene of the limbs was irreversible，ultimately leading to amputation.Conclusion:Timely diagnosis，early and active anti infective treatment，and effective management of various complications are the key to successfully treating cases of Ricketttsia Japonica

Objective:To investigate the epidemiological characteristics of human brucellosis in Yantai City of Shandong Province, and provide a basis for scientifically formulating brucellosis prevention and control strategies.Methods:A retrospective study was conducted to collect data on the epidemic situation of brucellosis in Yantai City from 2020 to 2022 from the "Infectious Disease Reporting Information Management System of China Disease Prevention and Control Information System". Descriptive analysis was conducted on the epidemic situation of brucellosis, the distribution of cases (region, time, population distributions), clinical symptoms and signs.Results:A total of 493 cases of brucellosis were reported in Yantai City from 2020 to 2022, including 129 cases in 2020, 172 cases in 2021, and 192 cases in 2022. The incidence rate was 1.905 3/100 000, 2.506 3/100 000, and 2.901 9/100 000, respectively. There were statistically significant differences between different years (χ 2 = 13.87, P = 0.001). The main distribution of brucellosis cases was in Laizhou City, Laiyang City, Zhaoyuan City, and Longkou City, accounting for 69.37% (342/493) of the total cases. Among them, Laizhou City had the highest proportion, accounting for 31.24% (154/493). There were reports of brucellosis cases in every month of the year, with the onset mainly concentrated from March to August, the cumulative cases accounted for 63.69% (314/493) of the total cases. In the population distribution, male cases were the main ones, with a male to female ratio of 2.24 ∶ 1.00 (341 ∶ 152). The age was (54.97 ± 13.59) years old, mainly concentrated in the age group of 45 - 59 years old, accounting for 43.20% (213/493). Farmers were the main occupation, accounting for 81.95% (404/493). The clinical symptoms of 493 cases of brucellosis were mainly fever, muscle and joint pain, and fatigue, accounting for 62.27% (307/493), 61.87% (305/493), and 61.46% (303/493), respectively. A few cases were accompanied by splenomegaly and hepatomegaly, accounting for 3.45% (17/493) and 1.22% (6/493), respectively. Direct contact with animals was the main route of infection for brucellosis cases, accounting for 57.00% (281/493). Conclusions:From 2020 to 2022, there are reports of brucellosis cases in every month of the year in Yantai City, with a high incidence in spring and summer. The main affected population is middle-aged male farmers, and direct contact with animals is the main route of infection. It is necessary to strengthen disease monitoring and health education for key populations during high incidence seasons.

Objective:To investigate the epidemiological characteristics of human brucellosis in Yantai City of Shandong Province, and provide a basis for scientifically formulating brucellosis prevention and control strategies.Methods:A retrospective study was conducted to collect data on the epidemic situation of brucellosis in Yantai City from 2020 to 2022 from the "Infectious Disease Reporting Information Management System of China Disease Prevention and Control Information System". Descriptive analysis was conducted on the epidemic situation of brucellosis, the distribution of cases (region, time, population distributions), clinical symptoms and signs.Results:A total of 493 cases of brucellosis were reported in Yantai City from 2020 to 2022, including 129 cases in 2020, 172 cases in 2021, and 192 cases in 2022. The incidence rate was 1.905 3/100 000, 2.506 3/100 000, and 2.901 9/100 000, respectively. There were statistically significant differences between different years (χ 2 = 13.87, P = 0.001). The main distribution of brucellosis cases was in Laizhou City, Laiyang City, Zhaoyuan City, and Longkou City, accounting for 69.37% (342/493) of the total cases. Among them, Laizhou City had the highest proportion, accounting for 31.24% (154/493). There were reports of brucellosis cases in every month of the year, with the onset mainly concentrated from March to August, the cumulative cases accounted for 63.69% (314/493) of the total cases. In the population distribution, male cases were the main ones, with a male to female ratio of 2.24 ∶ 1.00 (341 ∶ 152). The age was (54.97 ± 13.59) years old, mainly concentrated in the age group of 45 - 59 years old, accounting for 43.20% (213/493). Farmers were the main occupation, accounting for 81.95% (404/493). The clinical symptoms of 493 cases of brucellosis were mainly fever, muscle and joint pain, and fatigue, accounting for 62.27% (307/493), 61.87% (305/493), and 61.46% (303/493), respectively. A few cases were accompanied by splenomegaly and hepatomegaly, accounting for 3.45% (17/493) and 1.22% (6/493), respectively. Direct contact with animals was the main route of infection for brucellosis cases, accounting for 57.00% (281/493). Conclusions:From 2020 to 2022, there are reports of brucellosis cases in every month of the year in Yantai City, with a high incidence in spring and summer. The main affected population is middle-aged male farmers, and direct contact with animals is the main route of infection. It is necessary to strengthen disease monitoring and health education for key populations during high incidence seasons.

Objective:To summarize the clinical characteristics of focal cerebral arteriopathy (FCA) in children, and to analyze its influencing factor of prognosis.Methods:A retrospective cohort study was conducted. Clinical data from 40 children with FCA who were hospitalized at the Department of Neurology, Beijing Children′s Hospital, Capital Medical University, from September 2015 to August 2024 were collected. A centralized follow-up was conducted in October 2024 via outpatient clinics or the internet. The pediatric stroke outcome measure (PSOM) was used to evaluate their outcomes. Based on the PSOM, the children were further divided into a group with normal neurological function and another group with abnormal neurological function. Differences between groups were analyzed using the Mann-Whitney U test and Fisher exact test. Univariate Logistic regression analysis was performed to identify the influencing factors for neurological outcomes in children with FCA. Results:A total of 40 children were included, with 20 males and 20 females, and the onset age of 9.2 (6.8, 12.5) years. Among them, 12 cases (30%) had a history of varicella within 1 year before onset. There were 23 cases (58%) presenting with transient ischemic attack (TIA) or recurrent fluctuating symptoms of onset, while 3 cases (8%) developed progressive stroke within the first month of onset. The M1 segment of the middle cerebral artery was the most commonly affected vascular site, with a total of 16 cases (40%). Arterial occlusion occurred in 8 cases (20%). Lumbar puncture was completed in 36 children, and white blood cell counts in cerebrospinal fluid was increased in 6 cases. All 23 patients who completed magnetic resonance vessel wall imaging (VWI) showed circular enhancement of the arterial wall. A total of 28 patients (70%) received antiplatelet or anticoagulation therapy, and 16 patients (40%) received hormone therapy. At admission, the pediatric National Institute of Health Stroke Scale (PedNIHSS) score was 6.0 (2.0, 8.8) points, which decreased to 0.5 (0, 3.0) points at discharge. The follow-up duration was 1.6 (0.8, 4.9) years, with 1 case lost to follow-up. There was 1 case presenting with recurrence course manifesting as TIA. Among the 39 cases who completed the follow-up, 23 cases (59%) were assessed as neurologically normal by PSOM, while 16 cases (41%) were assessed as neurologically abnormal. Among the 29 cases who completed the imaging review, magnetic resonance angiography (MRA) review in 23 cases indicated stability or improvement in the original arterial stenosis, with 6 cases experiencing transient worsening of arterial stenosis early in the disease course (within 2 months), which later improved. Arterial stenosis progression occurred in 6 cases at the final review of 29 cases who completed the imaging review, with 1 case developing progressive cerebral arteriopathy. The proportion of patients with headache, altered consciousness, and aphasia in the abnormal neurological function group, as well as the PedNISS scores at admission and discharge, were all higher than those in the normal neurological function group (all P<0.05). Univariate Logistic regression analysis revealed that only a PedNISS score>6 points at onset was an influencing factor for abnormal neurological function ( OR=20.58, 95% CI 3.93-107.70, P<0.001). Conclusions:Childhood FCA often presents with fluctuating onset, and the proximal segment of the middle cerebral artery is frequently affected. Progression of arterial stenosis is common within 2 months of the disease course, but clinical progression and new ischemic lesions are uncommon. Most patients have a favorable long-term prognosis. PedNIHSS score>6 points at admission is related to abnormal neurological function outcomes.

Objective:To evaluate the clinical efficacy and safety of hematoporphyrin monomethyl ether (HMME) -mediated photodynamic therapy (PDT) in the treatment of facial port-wine stains (PWS) in children with Sturge-Weber syndrome (SWS) .Methods:A retrospective analysis was conducted based on the clinical data from SWS children treated with HMME-PDT at the Department of Dermatology, Children's Hospital, Capital Institute of Pediatrics from December 2020 to January 2022. HMME was intravenously injected at a dose of 5 mg/kg, followed by the irradiation of SWS lesions with a 532-nm light-emitting diode light source, and the treatment interval was 8 weeks. The efficacy of HMME-PDT for SWS was evaluated based on the subsidence of erythema and changes in the number and density of blood vessels under a dermoscope before and after treatment; adverse events after treatment were recorded. Fisher's exact test was used to analyze differences in efficacy.Results:A total of 15 children with SWS were included, comprising 7 males and 8 females, with an average age of 4.74 years (range, 1 - 14 years). There were 10 cases of clinical phenotype Ⅰ and 5 cases of type Ⅱ; 10 patients were accompanied by glaucoma, 6 by epilepsy, and 10 showed abnormalities on craniocerebral imaging. After HMME-PDT treatment, 4 out of 15 patients achieved complete remission of SWS lesions, 3 showed marked improvement, and 5 achieved improvement. Among 8 cases receiving 2 sessions of treatment, 1 achieved marked improvement and 4 showed improvement; among 7 cases receiving 3 or more sessions of treatment, 4 achieved complete remission, 2 achieved marked improvement, and 1 showed improvement; the proportions of patients achieving complete remission and marked improvement were significantly higher among those receiving 3 or more sessions of treatment than those receiving 2 sessions (both P < 0.05). Among 7 patients with pink-type PWS, 1 recovered completely, 2 achieved marked improvement, and 4 showed improvement; among 4 patients with purplish-red-type PWS, 3 recovered completely and 1 showed marked improvement; among 4 patients with thickened-type PWS, 1 achieved improvement; there was a significant difference in the proportions of patients achieving marked improvement or improvement among the patients with different types of PWS (both P < 0.05). Among 14 patients with lesions involving the central face region, 4 achieved marked improvement and 2 showed improvement; among 15 with lesions involving the lateral face region, 5 recovered completely, 3 achieved marked improvement, and 4 showed improvement; the recovery rate of lesions was higher in the lateral face region than in the central face region ( P < 0.05). Under a dermoscope, the skin lesions showed 4 vascular patterns: short rod-shaped vessels in 3 cases, linear vessels in 4, reticular vessels in 5, and mixed-type vessels in 3. The 3 patients with short rod-shaped vessels all recovered completely; among the 4 patients with linear vessels, 2 achieved marked improvement, and 2 showed improvement; among the 5 patients with reticular vessels, 1 recovered completely, 1 achieved marked improvement, and 3 showed improvement; the 3 patients with mixed-type vessels all showed poor response to the treatment; the proportions of patients who recovered completely and those who achieved improvement significantly differed among the patients with 4 different vascular patterns (both P < 0.05). All the children experienced varying degrees of pain, swelling, purpura, and crusting after treatment, but none exhibited exacerbation of ocular or neurological complications. Conclusion:HMME-PDT was safe and effective in the treatment of PWS in children with SWS, and its efficacy was related to the number of treatment sessions, lesion types and locations.