Objective:To apply combined serological diagnostic methods for anti-BP180-type and anti-laminin 332 (Ln332) -type mucous membrane pemphigoid (MMP), and to summarize their clinical and immunoserological characteristics.Methods:A retrospective analysis was conducted on the data from 52 patients clinically suspected of having MMP at the Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2017 to February 2022. Serum samples were collected, and combined serological tests, including indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting, were performed to analyze the immunoserological and clinical characteristics of the patients. The Fisher′s exact test was used to compare the lesion occurrence rates between groups.Results:Among the 52 patients clinically suspected of MMP, 32 (61.5%) were diagnosed with anti-BP180-type MMP, 10 (19.2%) with anti-Ln332-type MMP, and 4 (7.7%) with anti-BP180- and anti-Ln332-type MMP due to the presence of both anti-BP180 and anti-Ln332 antibodies; 2 tested positive for IgG on the epidermal side of salt-split skin, but no target antigens were identified by serological tests, and they were diagnosed with MMP (subtype pending) ; 4 tested negative by immunoserological tests. Ocular involvement was observed in 6 out of 10 patients with anti-Ln332-type MMP, whereas only 6 out of 32 anti-BP180-type MMP patients (18.8%) had ocular symptoms, and there was a significant difference in the occurrence rate of ocular involvement between the two groups ( P = 0.02) ; the occurrence rates of nasal involvement and multi-mucosal involvement were significantly higher in the anti-Ln332-type MMP patients (50%[5/10], 90%[9/10], respectively) than in the anti-BP180-type MMP patients (0, 25%[8/32], respectively, both P < 0.001). Scar formation occurred in 6 out of 10 anti-Ln332-type MMP patients, but occurred in only 6 out of 32 anti-BP180-type patients (18.8%, P = 0.02) . Conclusion:The combination of indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting could effectively identify anti-BP180 and anti-Ln332 autoantibodies in MMP patients, with BP180 being the most common target antigen; compared with anti-BP180-type MMP, anti-Ln332-type MMP appeared to be more frequently involve the ocular and nasal mucosae, associated with the involvement of multiple mucosal sites, carrying a higher risk of scar formation.

Traumatic main bronchus rupture is a relatively rare injury in thoracic trauma, which is extremely critical, with a mortality rate as high as 70% - 80%. The complete rupture and displacement of the traumatic cervical trachea can lead to asphyxia, hypoxia, and cardiac arrest, even death of the patient in a short time. We performed emergency surgery with the support of extracorporeal membrane oxygenation for a case of traumatic cervical tracheal trunk complete rupture and displacement combined with cardiac arrest and achieved a successful rescue. We summarized our experience and found that timely surgical reconstruction of the airway is the key to increasing the traumatic main bronchus rupture survival of patients.
Humans ; Extracorporeal Membrane Oxygenation ; Heart Arrest/etiology* ; Rupture ; Trachea/surgery*

OBJECTIVES: To investigate the clinical features and gene mutation characteristics of combined oxidative phosphorylation deficiency type 7 (COXPD7) caused by mutations in the C12orf65 gene, and to enhance the awareness of this disease. METHODS: A child diagnosed with COXPD7 in the Department of Neurology, Children's Hospital Affiliated to Zhengzhou University in 2021 was included, along with 10 patients reported in the literature. All subjects were analyzed for their genotypes and clinical phenotypes. RESULTS: A total of 11 patients with COXPD7 were included, comprising 1 reported in this study and 10 from the literature. Among the 11 patients, 9 had homozygous mutations in the C12orf65 gene, while 2 had compound heterozygous mutations, which were identified as frameshift or nonsense mutations. The age of onset ranged from 1 day to 2 years, and clinical manifestations included optic nerve atrophy and delays in intellectual and motor development. Eight patients exhibited external ophthalmoplegia, and five patients displayed spastic paralysis. Cranial magnetic resonance imaging revealed optic nerve atrophy in all 11 patients, abnormal brainstem signals in 10 patients, and a lactate peak on brainstem magnetic resonance spectroscopy scans in 3 patients. CONCLUSIONS COXPD7 associated with the C12orf65 gene results from homozygous or compound heterozygous mutations, with primary clinical manifestations of optic nerve atrophy and delays in intellectual and motor development. Some patients may also present with spastic paralysis or external ophthalmoplegia. Cranial imaging reveals symmetrical abnormal signals in bilateral basal ganglia and brainstem, and a lactate peak is observed on brainstem magnetic resonance spectroscopy scans.
Child, Preschool ; Female ; Humans ; Infant ; Male ; Mitochondrial Diseases/genetics* ; Mitochondrial Proteins/genetics* ; Mutation ; Oxidative Phosphorylation ; Infant, Newborn

Low-altitude unmanned technology represented by unmanned aerial vehicle (UAV) has shown broad application prospects in the medical field. Since June 2023, Affiliated Hospital of Jiaxing University The First Hospital of Jiaxing, focusing on the demand for medical supplies transport among hospitals, has led the construction and operation practice of a low-altitude transfer system for medical supplies featuring UAV. By establishing a management organizational structure, building a low-altitude digital intelligence platform, constructing UAV take-off and landing platforms, formulating and approving flight routes, and improving the medical supply distribution process, it successfully achieved the transportation of medical blood and biological specimens between Jiaxing Central Blood Station and relevant medical institutions, as well as among different campus and medical alliance units of Affiliated Hospital of Jiaxing University The First Hospital of Jiaxing. As of June 2024, a total of 6 routes have been opened within the Jiaxing municipal area, with a cumulative flight distance exceeding 5 000 km. A total of 64 flights for medical blood, 720 flights for blood specimens, and 5 flights for pathological specimens have been transported. Compared with land transportation methods, UAV transportation effectively shortened transportation time, enhanced the service capabilities and diagnosis and treatment quality of grassroots medical institutions, and also saved costs. It can provide a reference for other medical institutions to carry out low-altitude medical supplies distribution.

Objective:To apply combined serological diagnostic methods for anti-BP180-type and anti-laminin 332 (Ln332) -type mucous membrane pemphigoid (MMP), and to summarize their clinical and immunoserological characteristics.Methods:A retrospective analysis was conducted on the data from 52 patients clinically suspected of having MMP at the Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2017 to February 2022. Serum samples were collected, and combined serological tests, including indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting, were performed to analyze the immunoserological and clinical characteristics of the patients. The Fisher′s exact test was used to compare the lesion occurrence rates between groups.Results:Among the 52 patients clinically suspected of MMP, 32 (61.5%) were diagnosed with anti-BP180-type MMP, 10 (19.2%) with anti-Ln332-type MMP, and 4 (7.7%) with anti-BP180- and anti-Ln332-type MMP due to the presence of both anti-BP180 and anti-Ln332 antibodies; 2 tested positive for IgG on the epidermal side of salt-split skin, but no target antigens were identified by serological tests, and they were diagnosed with MMP (subtype pending) ; 4 tested negative by immunoserological tests. Ocular involvement was observed in 6 out of 10 patients with anti-Ln332-type MMP, whereas only 6 out of 32 anti-BP180-type MMP patients (18.8%) had ocular symptoms, and there was a significant difference in the occurrence rate of ocular involvement between the two groups ( P = 0.02) ; the occurrence rates of nasal involvement and multi-mucosal involvement were significantly higher in the anti-Ln332-type MMP patients (50%[5/10], 90%[9/10], respectively) than in the anti-BP180-type MMP patients (0, 25%[8/32], respectively, both P < 0.001). Scar formation occurred in 6 out of 10 anti-Ln332-type MMP patients, but occurred in only 6 out of 32 anti-BP180-type patients (18.8%, P = 0.02) . Conclusion:The combination of indirect immunofluorescence on salt-split skin, enzyme-linked immunosorbent assay, and immunoblotting could effectively identify anti-BP180 and anti-Ln332 autoantibodies in MMP patients, with BP180 being the most common target antigen; compared with anti-BP180-type MMP, anti-Ln332-type MMP appeared to be more frequently involve the ocular and nasal mucosae, associated with the involvement of multiple mucosal sites, carrying a higher risk of scar formation.

Objective To explore the preventive value of electroacupuncture combined with antithrombotic compression banding for deep venous thrombosis(DVT)in high-risk patients with neurocritical venous thromboembolism(VTE).Methods A total of 98 high-risk patients with neurocritical VTE who were admitted to the First Hospital of Jiaxing from April 2023 to May 2024 were selected as research subjects.They were divided into pressure band group and electroacupuncture group by random number table method,with 49 cases in each group.Both groups of patients were given low molecular weight heparin to prevent thrombosis.The pressure band group was given antithrombotic compression banding intervention,and electroacupuncture group was combined with electroacupuncture intervention on the basis of antithrombotic compression banding.Both groups were treated continuously for 14 days.The changes in levels of D-dimer(D-D),interleukin-6(IL-6),fibrinogen(FIB),intercellular adhesion molecule-1(ICAM-1),activated partial thromboplastin(APTT),tumor necrosis factor-α(TNF-α),prothrombin time(PT)and the scores of activity of daily living(ADL)scale of two groups of patients before treatment and after 7 days and 14 days of treatment were compared.The incidences of VTE,tenderness and superficial varicose veins of two groups of patients were statistically analyzed.Results After 7 days and 14 days of treatment,the levels of D-D,FIB,IL-6,ICAM-1,and TNF-α in both groups were significantly lower than those before treatment,APTT and PT were significantly longer than those before treatment,and ADL scores were significantly higher than those before treatment(P＜0.05).After 7 days and 14 days of treatment,the levels of D-D,FIB,IL-6,ICAM-1 and TNF-α in electroacupuncture group were significantly lower than those in pressure band group,and ADL score was significantly higher than that in pressure band group(P＜0.05).The APTT and PT of patients in electroacupuncture group after 7 days of treatment were significantly longer than those in pressure band group(P＜0.05).The incidences of VTE,tenderness and superficial varicose veins in electroacupuncture group were significantly lower than those in pressure band group(P＜0.05).Conclusion The application of electroacupuncture combined with antithrombotic compression banding in neurocritical VTE high-risk patients can alleviate inflammatory responses,improve coagulation function,and reduce the occurrence of VTE,tenderness,and superficial varicose veins.

Objective To explore the preventive value of electroacupuncture combined with antithrombotic compression banding for deep venous thrombosis(DVT)in high-risk patients with neurocritical venous thromboembolism(VTE).Methods A total of 98 high-risk patients with neurocritical VTE who were admitted to the First Hospital of Jiaxing from April 2023 to May 2024 were selected as research subjects.They were divided into pressure band group and electroacupuncture group by random number table method,with 49 cases in each group.Both groups of patients were given low molecular weight heparin to prevent thrombosis.The pressure band group was given antithrombotic compression banding intervention,and electroacupuncture group was combined with electroacupuncture intervention on the basis of antithrombotic compression banding.Both groups were treated continuously for 14 days.The changes in levels of D-dimer(D-D),interleukin-6(IL-6),fibrinogen(FIB),intercellular adhesion molecule-1(ICAM-1),activated partial thromboplastin(APTT),tumor necrosis factor-α(TNF-α),prothrombin time(PT)and the scores of activity of daily living(ADL)scale of two groups of patients before treatment and after 7 days and 14 days of treatment were compared.The incidences of VTE,tenderness and superficial varicose veins of two groups of patients were statistically analyzed.Results After 7 days and 14 days of treatment,the levels of D-D,FIB,IL-6,ICAM-1,and TNF-α in both groups were significantly lower than those before treatment,APTT and PT were significantly longer than those before treatment,and ADL scores were significantly higher than those before treatment(P＜0.05).After 7 days and 14 days of treatment,the levels of D-D,FIB,IL-6,ICAM-1 and TNF-α in electroacupuncture group were significantly lower than those in pressure band group,and ADL score was significantly higher than that in pressure band group(P＜0.05).The APTT and PT of patients in electroacupuncture group after 7 days of treatment were significantly longer than those in pressure band group(P＜0.05).The incidences of VTE,tenderness and superficial varicose veins in electroacupuncture group were significantly lower than those in pressure band group(P＜0.05).Conclusion The application of electroacupuncture combined with antithrombotic compression banding in neurocritical VTE high-risk patients can alleviate inflammatory responses,improve coagulation function,and reduce the occurrence of VTE,tenderness,and superficial varicose veins.

Low-altitude unmanned technology represented by unmanned aerial vehicle (UAV) has shown broad application prospects in the medical field. Since June 2023, Affiliated Hospital of Jiaxing University The First Hospital of Jiaxing, focusing on the demand for medical supplies transport among hospitals, has led the construction and operation practice of a low-altitude transfer system for medical supplies featuring UAV. By establishing a management organizational structure, building a low-altitude digital intelligence platform, constructing UAV take-off and landing platforms, formulating and approving flight routes, and improving the medical supply distribution process, it successfully achieved the transportation of medical blood and biological specimens between Jiaxing Central Blood Station and relevant medical institutions, as well as among different campus and medical alliance units of Affiliated Hospital of Jiaxing University The First Hospital of Jiaxing. As of June 2024, a total of 6 routes have been opened within the Jiaxing municipal area, with a cumulative flight distance exceeding 5 000 km. A total of 64 flights for medical blood, 720 flights for blood specimens, and 5 flights for pathological specimens have been transported. Compared with land transportation methods, UAV transportation effectively shortened transportation time, enhanced the service capabilities and diagnosis and treatment quality of grassroots medical institutions, and also saved costs. It can provide a reference for other medical institutions to carry out low-altitude medical supplies distribution.

Objective:To analyze the etiological features and clinical characteristics of herpes zoster cases under 20 years old in Beijing City from 2017 to 2021.Methods:Herpes zoster cases were collected from a surveillance system in Beijing City from December 2017 to April 2021. The cases included individuals under 20 years old from seven sentinel hospitals located in two districts (Miyun District and Changping District). The basic information, the rash date of rash onset and the location and number of lesions were investigated at the first visit to the hospital, and the lesion swab samples were collected for laboratory testing. A telephone follow-up was conducted 21 days after the onset of the rash to investigate the degree of pain, duration of the rash and duration of pain. The individuals who still experienced neuralgia were further investigated for their pain condition at 90 days after the onset of the rash, to discover cases with postherpetic neuralgia. DNA was extracted from the rash fluid, and the ORF62 gene region was amplified and sequenced to obtain the viral sequence. The wild-type strain or chickenpox vaccine strain was identified by using sequence alignment, and the clinical characteristics of cases with different varicella vaccinations were compared.Results:A total of 78 herpes zoster cases under 20 years old were investigated during 2017-2021 in Beijing City, and 61 cases completed the follow-up survey. The age range of 61 cases was 1.83 to 20.54 years with a median age of 17.50 years. There were 36 males (59.02%) and 25 females (40.98%). Among them, there were 29 cases with the chickenpox vaccine immunization history (18 cases with one dose, 5 cases with two doses and 6 cases with unknown doses), 13 cases with no vaccination history and 19 cases with unknown vaccination history. Among the 78 cases, the herpetic fluid samples of 64 cases were positive for VZV, including 62 cases identified as wild-type strains and two cases as vaccine strains. The two vaccine strain cases were both 2-year-old girls who had received one dose of varicella vaccine and developed herpes zoster 3 months and 13 months after vaccination. Among the 29 cases with chickenpox vaccine immunization history, the majority had 10 to 49 lesions, accounting for 58.62% (17 cases). The trunk was the most common site of lesions, accounting for 44.83% (13 cases). About 51.72% (15 cases) reported "no or mild" pain intensity. The median ( Q1, Q3) scores for the worst pain, duration of pain and the time to crusting of lesions in the herpes zoster cases were 3 (1.5, 5) points, 10 (1.5, 12.5) days and 10 (6.5, 13) days, respectively. There was no statistically significant difference in the constituent ratio of the location of lesions, number of lesions and pain degree among the cases with vaccination history, without vaccination history and with unknown vaccination history ( P>0.05). There was also no statistically significant difference in the distribution of pain score, duration of lesions and duration of pain across the three groups ( P>0.05). Conclusion:Wild strains are the predominant pathogens in herpes zoster cases under 20 years old in Beijing City during 2017-2021. The varicella vaccination has no significant impact on the clinical manifestations of herpes zoster cases.

Objective:To compare the prognostic values of different classification by using transpulmonary pressure gradient (TPG), diastolic pressure gradient (DPG) and pulmonary vascular resistance (PVR) in patients with pulmonary hypertension due to left heart disease (PH-LHD), and investigated hemodynamic and clinical factors associated with mortality in patients with PH-LHD.Methods:This was a single-center prospective cohort study. In-hospital patients diagnosed with PH-LHD via right heart catheterization at the Department of Cardiology, the First Affiliated Hospital of Nanjing Medical University, from September 2013 to December 2019 were enrolled. Patients were divided according to TPG (cutoff value 12 mmHg; 1 mmHg=0.133 kPa), DPG (cutoff value 7 mmHg), PVR (cutoff value 3 Wood Units), and the combination of TPG and PVR. Baseline characteristic was recorded. All patients were followed up until the occurrence of endpoint event, defined as all-cause death that occurred during the follow-up period, or until April 18, 2022. Receiver operating characteristic curves were used to compare the predictive value of 3 classification methods for all-cause death in PH-LHD patients. The optimal cutoff values were calculated using Jorden index. Survival analysis was performed using Kaplan-Meier analysis, and log-rank test was used to compare the predictive efficacy of classification methods based on optimal cutoff values or guidance-recommended thresholds for the survival of PH-LHD patients. Variables showing statistical significance in the univariate analysis were incorporated into multivariate Cox regression model to analyze the independent risk factors for all-cause mortality.Results:A total of 243 patients were enrolled, aged (54.9±12.7) years old, including 169 (69.5%) males. During a median follow-up of 57 months, there were 101 (41.6%) deaths occurred. Grouping results were as follows: (1) TPG: TPG≤12 mmHg group 115 patients, TPG>12 mmHg group 128 patients; (2) DPG: DPG<7 mmHg group 193 patients, DPG≥7 mmHg group 50 patients; (3) PVR: PVR≤3 Wood Units group 108 patients, PVR>3 Wood Units group 135 patients; (4) TPG and PVR: TPG≤12 mmHg and PVR≤3 Wood Units group 89 patients, TPG>12 mmHg and PVR>3 Wood Units group 109 patients. PVR ( AUC=0. 698,95% CI:0.631-0.766) had better predictive value for all-cause mortality than TPG ( AUC=0.596, 95% CI: 0.523-0.669) and DPG ( AUC=0.526, 95% CI: 0.452-0.601) (all P<0.05). The optimal cutoff values for TPG, DPG, and PVR were13.9 mmHg, 2.8 mmHg, and 3.8 Wood Units, respectively. Kaplan-Meier analysis based on the optimal cutoff values or guidance-recommended thresholds showed that PVR and TPG were the predictors of survival ( P<0.05), while DPG did not showed significance ( P>0.05). Multivariate Cox regression analysis showed that age, PVR and log 2N-terminal pro-B-type natriuretic peptide were independent risk factors for all-cause mortality in PH-LHD patients (all P<0.05). Conclusion:Classification according to PVR was most valuable in predicting all-cause death in PH-LHD patients, while TPG showed moderate predictive ability and DPG had no predictive value.