A novel variant in the GJB6 gene in a large Chinese family with a unique phenotype of Clouston syndrome / 医学前沿

Clouston syndrome (OMIM #129500), also known as hidrotic ectodermal dysplasia type 2, is a rare autosomal dominant skin disorder. To date, four mutations in the GJB6 gene, G11R, V37E, A88V, and D50N, have been confirmed to cause this condition. In previous studies, the focus has been mainly on gene sequencing, and there has been a lack of research on clinical manifestations and pathogenesis. To confirm the diagnosis of this pedigree at the molecular level and summarize and analyse the clinical phenotype of patients and to provide a basis for further study of the pathogenesis of the disease, we performed whole-exome and Sanger sequencing on a large Chinese Clouston syndrome pedigree. Detailed clinical examination included histopathology, hair microscopy, and scanning electron microscopy. We found a novel heterozygous missense variant (c.134G>C:p.G45A) for Clouston syndrome. We identified a new clinical phenotype involving all nail needling pain in all patients and found a special honeycomb hole structure in the patients' hair under scanning electron microscopy. Our data reveal that a novel variant (c.134G>C:p.G45A) plays a likely pathogenic role in this pedigree and highlight that genetic testing is necessary for the diagnosis of Clouston syndrome.

Clinical features and genetic analysis of a patient with type 2 neurofibromatosis manifested as oculomotor nerve palsy / 中华医学遗传学杂志

OBJECTIVE@#To report on a rare case of Neurofibromatosis type 2 (NF2) manifesting as oculomotor nerve palsy and explore its genetic basis.@*METHODS@#A patient with NF2 who had presented at Beijing Ditan Hospital Affiliated to Capital Medical University on July 10, 2021 was selected as the study subject. Cranial and spinal cord magnetic resonance imaging (MRI) was carried out on the patient and his parents. Peripheral blood samples were collected and subjected to whole exome sequencing. Candidate variant was verified by Sanger sequencing.@*RESULTS@#MRI revealed bilateral vestibular Schwannomas, bilateral cavernous sinus meningiomas, popliteal neurogenic tumors, and multiple subcutaneous nodules in the patient. DNA sequencing revealed that he has harbored a de novo nonsense variant of the NF2 gene, namely c.757A>T, which has replaced a codon (AAG) encoding lysine (K) at position 253 with a stop codon (TAG). This has resulted in removal of the Merlin protein encoded by the NF2 gene from position 253 onwards. The variant was not found in public databases. Bioinformatic analysis suggested that the corresponding amino acid is highly conserved. Based on the guidelines from the American College of Medical Genetics and Genomics (ACMG), the variant was rated as pathogenic (PVS1+PS2+PM2_Supporting+PP3+PP4).@*CONCLUSION@#The heterozygous nonsense variant c.757A>T (p.K253*) of the NF2 gene probably underlay the disease in this patient with an early onset, atypical but severe phenotype.

Psychological effect of physical function dependence on maintenance hemodialysis patients and their primary family caregivers / 中华肝脏病杂志

Objective: To investigate the psychological effect of physical function dependence on maintenance hemodialysis (MHD) patients and their primary family caregivers. Methods: The study was a cross-sectional survey. The MHD patients in the hemodialysis centre, the Third Affiliated Hospital of Guangzhou Medical University from March 2022 to June 2022 were enrolled. The patients' demographics and laboratory data were collected. Katz and Lawton-Brody questionnaires were used to assess patients' physical function, and Five Item Mental Health Inventory (MHI-5) was used to evaluate the psychological conditions of the patients and their primary family caregivers. Multiple linear regression analysis was used to analyze the influencing factors of MHI-5 scores of the patients and their primary family caregivers. Results: A total of 116 patients were included, with 61 males and 55 females. There were 47 patients (40.5%) with physical function dependence. In the physical function dependence group, serum albumin (t=-2.512, P=0.013), MHI-5 scores of patients and their primary family caregivers (t=-8.461, P < 0.001; t=-8.533, P < 0.001) and male ratio (χ2=8.467, P=0.002) were significantly lower, and the age (t=9.754, P < 0.001) and the proportions of hypertension (χ2=20.421, P < 0.001), diabetes (χ2=10.470, P=0.002), cardiovascular and cerebrovascular diseases (χ2=9.898, P=0.003) were significantly higher than those in the normal physical function group. The incidence of mental disorders in MHD patients was 39.7%(46/116), and the incidence of mental disorders in the physical function dependence group was significantly higher than that in the normal physical function group [72.3%(34/47) vs. 17.4%(12/69), χ2=35.275, P < 0.001]. The incidence of mental disorders in the primary family caregivers was 32.8%(38/116), and the incidence of mental disorders in the primary family caregivers of physical function dependence group was significantly higher than that in the normal physical function group [66.0%(31/47) vs. 10.1%(7/69), χ2=39.536, P < 0.001]. The incidence of mental disorders in the primary family caregivers of MHD patients who were not spouses was significantly higher than that of spouses [46.0%(29/63) vs. 17.0%(9/53), χ2=11.028, P=0.001], and in physical function dependence group, the incidence of mental disorders in non-spouses was significantly higher than that in spouses [80.6%(25/31) vs. 37.5%(6/16), χ2=8.749, P=0.003]. Multiple linear analysis showed that bathing (β=-5.182, P=0.015), doing laundry (β=-7.053, P < 0.001), taking medication (β=-8.680, P=0.003), and female patients (β=-2.982, P=0.030) were the influencing factors of MHI-5 scores decline in MHD patients. Bathing (β=-4.404, P=0.032), preparing meals (β=-3.954, P=0.041), managing money (β=-5.067, P=0.021), and female patients (β=-2.466, P=0.042) were the influencing factors of MHI-5 scores decline in primary family caregivers. Conclusions: The incidence of physical function dependence in MHD patients is high, and its manifestations and influencing factors are diverse. The incidence of mental disorders in MHD patients and their primary family caregivers is also high, especially in patients with physical function dependence and non- spouse family caregivers. Clinicians should pay attention to and assess the physical function dependence of MHD patients as early as possible, and intervene in time. At the same time, they should also pay attention to the mental health of MHD patients and their primary family caregivers.

Analysis of risk factors of mortality in infants and toddlers with moderate to severe pediatric acute respiratory distress syndrome / 中华儿科杂志

Objective: To identify the risk factors in mortality of pediatric acute respiratory distress syndrome (PARDS) in pediatric intensive care unit (PICU). Methods: Second analysis of the data collected in the "efficacy of pulmonary surfactant (PS) in the treatment of children with moderate to severe PARDS" program. Retrospective case summary of the risk factors of mortality of children with moderate to severe PARDS who admitted in 14 participating tertiary PICU between December 2016 to December 2021. Differences in general condition, underlying diseases, oxygenation index, and mechanical ventilation were compared after the group was divided by survival at PICU discharge. When comparing between groups, the Mann-Whitney U test was used for measurement data, and the chi-square test was used for counting data. Receiver Operating Characteristic (ROC) curves were used to assess the accuracy of oxygen index (OI) in predicting mortality. Multivariate Logistic regression analysis was used to identify the risk factors for mortality. Results: Among 101 children with moderate to severe PARDS, 63 (62.4%) were males, 38 (37.6%) were females, aged (12±8) months. There were 23 cases in the non-survival group and 78 cases in the survival group. The combined rates of underlying diseases (52.2% (12/23) vs. 29.5% (23/78), χ2=4.04, P=0.045) and immune deficiency (30.4% (7/23) vs. 11.5% (9/78), χ2=4.76, P=0.029) in non-survival patients were significantly higher than those in survival patients, while the use of pulmonary surfactant (PS) was significantly lower (8.7% (2/23) vs. 41.0% (32/78), χ2=8.31, P=0.004). No significant differences existed in age, sex, pediatric critical illness score, etiology of PARDS, mechanical ventilation mode and fluid balance within 72 h (all P>0.05). OI on the first day (11.9(8.3, 17.1) vs.15.5(11.7, 23.0)), the second day (10.1(7.6, 16.6) vs.14.8(9.3, 26.2)) and the third day (9.2(6.6, 16.6) vs. 16.7(11.2, 31.4)) after PARDS identified were all higher in non-survival group compared to survival group (Z=-2.70, -2.52, -3.79 respectively, all P<0.05), and the improvement of OI in non-survival group was worse (0.03(-0.32, 0.31) vs. 0.32(-0.02, 0.56), Z=-2.49, P=0.013). ROC curve analysis showed that the OI on the thind day was more appropriate in predicting in-hospital mortality (area under the curve= 0.76, standard error 0.05,95%CI 0.65-0.87,P<0.001). When OI was set at 11.1, the sensitivity was 78.3% (95%CI 58.1%-90.3%), and the specificity was 60.3% (95%CI 49.2%-70.4%). Multivariate Logistic regression analysis showed that after adjusting for age, sex, pediatric critical illness score and fluid load within 72 h, no use of PS (OR=11.26, 95%CI 2.19-57.95, P=0.004), OI value on the third day (OR=7.93, 95%CI 1.51-41.69, P=0.014), and companied with immunodeficiency (OR=4.72, 95%CI 1.17-19.02, P=0.029) were independent risk factors for mortality in children with PARDS. Conclusions: The mortality of patients with moderate to severe PARDS is high, and immunodeficiency, no use of PS and OI on the third day after PARDS identified are the independent risk factors related to mortality. The OI on the third day after PARDS identified could be used to predict mortality.

Progress in perioperative management of ABO-incompatible pediatric liver transplantation / 中华外科杂志

ABO incompatible(ABO-I) liver grafts will affect the prognosis of liver transplantation. With the improvement of perioperative treatment,including plasma exchange,rituximab,splenectomy,etc.,the prognosis of ABO-I liver transplantation has been greatly improved. Because children's immune systems are not fully developed,the perioperative management of ABO-I pediatric liver transplantation is significantly different from that of adults. Reducing the perioperative anti-donor ABO antibody titer is the key to the perioperative management of ABO-I liver transplantation. This article summarizes literatures on the perioperative management of ABO-I pediatric liver transplantation, including the perioperative anti-rejection regimen in pediatric recipients of different ages, splenectomy, postoperative monitoring and postoperative complications, etc.

Predictive value of different critical values of slow gait speed on adverse outcomes in elderly maintenance hemodialysis patients / 中华肾脏病杂志

Objective:To explore the predictive value of different critical values of slow gait speed on adverse outcomes in elderly maintenance hemodialysis (MHD) patients.Methods:The study was a prospective cohort study. The clinical data of elderly patients (≥ 60 years old) who received MHD treatment in the Third Affiliated Hospital of Guangzhou Medical University from March 1 to June 30, 2021 were collected, including demographic characteristics, diseases-related data and laboratory examination results. The follow-up period was one year. The six-meter walking test was used to measure the gait speed (m/s), and 0.6 m/s, 0.8 m/s and 1.0 m/s were used as the different critical values of the gait speed for grouping. The differences of clinical data between different groups were compared. Logistic regression analysis method was used to assess the association of slow gait speed with adverse outcomes (falls and hospitalization) in elderly MHD patients. The receiver operating characteristic (ROC) curve was performed to evaluate the best critical value of slow gait speed to predict the risk of falls and hospitalization.Results:A total of 108 elderly patients with MHD were included, with 57 males and 51 females. There were 43 patients (39.8%) of falls and 34 patients (31.5%) of hospitalization. There were statistically significant differences in age, Charlson's comorbidity index, and the proportions of hypertension, family support needed in daily life, walking aids needed, falls and hospitalization events among the four groups of the patients grouped according to gait speed (all P < 0.05). Multivariate logistic regression analysis results showed that the risk of falls predicted by gait speed of 0.6- < 0.8 m/s was higher than that by gait speed of > 1.0 m/s ( OR=3.973, 95% CI 1.116-14.136, P=0.033). The risk of hospitalization predicted by gait speed < 0.6 m/s was higher than that by gait speed > 1.0 m/s ( OR=9.147, 95% CI 1.658-50.453, P=0.011). The logistic regression analysis was performed with the critical values of 0.6 m/s, 0.8 m/s and 1.0 m/s as the classification variables, and the results showed that the gait speed of < 0.8 m/s was an influencing factor of the falls risk in elderly MHD patients (≥ 0.8 m/s as reference, OR=3.200, 95% CI 1.099-9.318, P=0.033). The gait speed < 0.8 m/s and < 0.6 m/s were influencing factors of hospitalization (≥ 0.8 m/s as reference, OR=3.899, 95% CI 1.355-11.216, P=0.012; ≥ 0.6 m/s as reference, OR=4.226, 95% CI 1.107-16.140, P=0.035). The area under the ROC curve for gait speed of < 0.6 m/s, < 0.8 m/s and < 1.0 m/s to predict the risk of falls were 0.605(95% CI 0.493-0.717, P=0.065), 0.668(95% CI 0.562-0.774, P=0.003), and 0.634 (95% CI 0.529-0.739, P=0.019), respectively. The best critical value of slow gait speed to predict the risk of fall was 0.73 m/s, and the area under the ROC curve was 0.720(95% CI 0.623-0.817, P < 0.001), with the sensitivity and specificity of 0.846 and 0.512, respectively. The area under the ROC curve for gait speed of < 0.6 m/s, < 0.8 m/s and < 1.0 m/s to predict the risk of hospitalization were 0.629(95% CI 0.509-0.749, P=0.032),0.683(95% CI 0.573-0.793, P=0.002), and 0.608(95% CI 0.497- 0.719, P=0.073). The best critical value of slow gait speed to predict the risk of hospitalization was 0.81 m/s, and the area under the ROC curve was 0.688(95% CI 0.576-0.800, P=0.002), with the sensitivity and specificity of 0.689 and 0.676, respectively. Conclusion:The critical value of gait speed 0.8 m/s can be used to predict the risk of falls and hospitalization in elderly MHD patients.

Psychological effect of physical function dependence on maintenance hemodialysis patients and their primary family caregivers / 中华肾脏病杂志

Objective:To investigate the psychological effect of physical function dependence on maintenance hemodialysis (MHD) patients and their primary family caregivers.Methods:The study was a cross-sectional survey. The MHD patients in the hemodialysis centre, the Third Affiliated Hospital of Guangzhou Medical University from March 2022 to June 2022 were enrolled. The patients' demographics and laboratory data were collected. Katz and Lawton-Brody questionnaires were used to assess patients' physical function, and Five Item Mental Health Inventory (MHI-5) was used to evaluate the psychological conditions of the patients and their primary family caregivers. Multiple linear regression analysis was used to analyze the influencing factors of MHI-5 scores of the patients and their primary family caregivers.Results:A total of 116 patients were included, with 61 males and 55 females. There were 47 patients (40.5%) with physical function dependence. In the physical function dependence group, serum albumin ( t=-2.512, P=0.013), MHI-5 scores of patients and their primary family caregivers ( t=-8.461, P < 0.001; t=-8.533, P < 0.001) and male ratio ( χ2=8.467, P=0.002) were significantly lower, and the age ( t=9.754, P < 0.001) and the proportions of hypertension ( χ2=20.421, P < 0.001), diabetes ( χ2=10.470, P=0.002), cardiovascular and cerebrovascular diseases ( χ2=9.898, P=0.003) were significantly higher than those in the normal physical function group. The incidence of mental disorders in MHD patients was 39.7%(46/116), and the incidence of mental disorders in the physical function dependence group was significantly higher than that in the normal physical function group [72.3%(34/47) vs. 17.4%(12/69), χ2=35.275, P < 0.001]. The incidence of mental disorders in the primary family caregivers was 32.8%(38/116), and the incidence of mental disorders in the primary family caregivers of physical function dependence group was significantly higher than that in the normal physical function group [66.0%(31/47) vs. 10.1%(7/69), χ2=39.536, P < 0.001]. The incidence of mental disorders in the primary family caregivers of MHD patients who were not spouses was significantly higher than that of spouses [46.0%(29/63) vs. 17.0%(9/53), χ2=11.028, P=0.001], and in physical function dependence group, the incidence of mental disorders in non-spouses was significantly higher than that in spouses [80.6%(25/31) vs. 37.5%(6/16), χ2=8.749, P=0.003]. Multiple linear analysis showed that bathing ( β=-5.182, P=0.015), doing laundry ( β=-7.053, P < 0.001), taking medication ( β=-8.680, P=0.003), and female patients ( β=-2.982, P=0.030) were the influencing factors of MHI-5 scores decline in MHD patients. Bathing ( β=-4.404, P=0.032), preparing meals ( β=-3.954, P=0.041), managing money ( β=-5.067, P=0.021), and female patients ( β=-2.466, P=0.042) were the influencing factors of MHI-5 scores decline in primary family caregivers. Conclusions:The incidence of physical function dependence in MHD patients is high, and its manifestations and influencing factors are diverse. The incidence of mental disorders in MHD patients and their primary family caregivers is also high, especially in patients with physical function dependence and non- spouse family caregivers. Clinicians should pay attention to and assess the physical function dependence of MHD patients as early as possible, and intervene in time. At the same time, they should also pay attention to the mental health of MHD patients and their primary family caregivers.

Effects of thunder-fire moxibustion combined with external application of Chinese herbal ointment at the trigger point on lumbodorsal myofascial pain syndrome of cold-damp stagnation type / 中国基层医药

Objective:To investigate the clinical efficacy of thunder-fire moxibustion combined with external application of Chinese herbal ointment at the trigger point on lumbodorsal myofascial pain syndrome of cold-damp stagnation type.Methods:A total of 90 patients with lumbodorsal myofascial pain syndrome of cold-damp stagnation type admitted to Zhejiang Provincial Hospital of Integrated Traditional Chinese and Western Medicine from September 2021 to April 2022 were included in this study. They were randomly divided into three groups ( n = 30/group) using the random number table method. Patients in the Chinese herbal ointment group were treated by external application of Chinese herbal ointment at the trigger point. Patients in the thunder-fire moxibustion group were treated with thunder-fire moxibustion. Patients in the combined therapy group were treated with thunder-fire moxibustion combined with external application of Chinese herbal ointment at the trigger point. All patients were treated for 28 consecutive days. Clinical efficacy was compared among the three groups. Before and after treatment, the Visual Analogue Scale score, local tenderness score, Oswestry Disability Index score and Pittsburgh Sleep Quality Index score were compared among the three groups. Results:Total response rate (96.67%) in the combined therapy group was significantly higher than 73.33% in the thunder-fire moxibustion group and 66.67% in the Chinese herbal ointment group ( χ2 = 9.01, 4.70, both P < 0.05). Visual Analogue Scale score and local tenderness score in the combined therapy group were (1.96 ± 0.93) points and (1.00 ± 0.69) points, respectively, which were significantly lower than (2.43 ± 0.87) points and (1.37 ± 0.56) points in the thunder-fire moxibustion group and (2.77 ± 0.86) points and (1.50 ± 0.57) points in the Chinese herbal ointment group ( F = 6.22, 5.38, both P < 0.05). The Oswestry Disability Index score in the combined therapy group was (19.80 ± 3.80) points, which was significantly lower than (22.30 ± 2.82) points in the thunder-fire moxibustion group and (23.60 ± 3.71) points in the Chinese herbal ointment group ( F = 9.07, both P < 0.05). After treatment, the Pittsburgh Sleep Quality Index score in the combined therapy group was (5.30 ± 1.12) points, which was significantly lower than (6.50 ± 1.33) points in the thunder-fire moxibustion group and (6.73 ± 1.41) points in the Chinese herbal ointment group ( F = 10.59, both P < 0.05). Conclusion:Thunder-fire moxibustion combined with external application of Chinese herbal ointment at the trigger point is highly effective on lumbodorsal myofascial pain syndrome of cold-damp stagnation type than monotherapy. The combined therapy can markedly reduce pain and greatly improve lumbodorsal function and sleep quality.

Analysis of risk factors for hemorrhage in patients with acute poisoning treated with hemoperfusion / 中华劳动卫生职业病杂志

Objective: To explore the clinical characterist ics and risk factors of hemorrhage complicated by hemoperfusion therapy in patients with acute poisoning. Methods: In January 2021, the clinical data of 196 patients with acute poisoning who received hemoperfusion therapy in the Second Affiliated Hospital of Air Force Military Medical University from January 2018 to December 2020 were analyzed, and the patients were divided into bleeding group and non-bleeding group according to whether the patients were complicated with bleeding. Multivariate logistic regression was used to analyze the independent risk factors for hemorrhage in patients treated with hemoperfusion. Results: A total of 21 patients in the bleeding group and 175 patients in the non-bleeding group were included. There was no significant difference in general data such as gender, age, and body mass index between the two groups (P>0.05) . Organophosphorus pesticides (χ(2)= 4.56, P=0.030) , HA230 perfusion device (χ(2)=4.12, P=0.042) , platelet count (t=-2.33, P=0.009) and activated partial thromboplastin time (t=14.53, P<0.001) at 2 h of perfusion were the influencing factors of hemorrhage in patients with acute poisoning treated with hemoperfusion. Among them, organophosphorus pesticides, 2 h perfusion activated partial thromboplastin time ≥35 s and other factors were independent risk factors forcomplicated bleeding (P<0.05) . Conclusion: Patients with acute poisoning, especially organophosphorus pesticide poisoning, are at greater risk of bleeding during hemoperfusion therapy. Monitoring of changes in activated partial thromboplastin time should be strengthened and the dose of anticoagulants should be adjusted in time to reduce the risk of bleeding.

Clinical and molecular characteristics of Streptococcus pneumoniae-associated hemophagocytic lymphohistiocytosis in children / 中华儿科杂志

Objective: To summarize the clinical features of Streptococcus pneumoniae-associated hemophagocytic syndrome (SP-HLH), and the serotypes and drug-resistant characteristics of the isolated strains. Methods: There were 15 children with SP-HLH admitted to the Pediatric Intensive Care Unit (PICU) of Beijing Children's Hospital, Capital Medical University from January 2013 to December 2020 were included in this study. Clinical data including children's general characteristics, clinical features, laboratory examinations, treatments, prognosis and the outcomes of follow-up by May 2021 were analyzed retrospectively. The serotypes and drug resistance of the isolated strains were identified. All children were divided into the clinical improvement group and the death group. Mann-Whitney U test, Fisher's exact test were used to compare the data of the two groups. Results: Among the 15 children with SP-HLH, 8 were males and 7 were females. The age of these children was 1.0 (1.0, 2.5) years. Regarding the primary infection, there were 9 cases of severe pneumonia, 3 cases of meningitis and 3 cases of blood stream infection. None of these children had received pneumoniae conjugate vaccine (PCV) and all of them were admitted to the PICU. Respiratory failure was observed in 10 patients, acute renal injury in 5, and hemolytic uremic syndrome in 3 patients. All children received glucocorticoids and high-dose intravenous immunogloblin (IVIG) in addition to anti-infective treatment. Eight of the children were cured while the other 7 died. The neutrophil count in the death group was lower than that in the clinical improvement group ((5.0 (1.7, 9.3) × 109 vs. 5.2 (3.4, 10.5) ×109/L, Z =-2.43, P<0.015), and the length of hospital stay and days of PICU stay in the death group were both shorter than those in the improvement group statistically (3 (1, 11) vs. 39 (34, 48) d, 2 (1, 4) vs. 19 (12, 31) d, Z=-3.25, -3.24, both P=0.001). Ten serotypes of Streptococcus pneumoniae were identified, including 4 strains of 19F, 3 of 19A, 1 of 23F, 1 of 15A and 1 of 14, among which 9 strains (9/10) were covered by PCV13. All strains were resistant to erythromycin yet sensitive to vancomycin and linezolid. Conclusions: SP-HLH is more common in children under the age of 3, with a high mortality rate. The death cases have lower neutrophil count and rapid disease progression. The comprehensive treatment is anti-infective combined with glucocorticoids and high-dose IVIG. The predominant serotypes are 19F and 19A and all isolated strains were susceptible to vancomycin and linezolid.

Review of risk evaluation scores for benign end stage liver diseases recipients / 中华外科杂志

Liver transplant is an unreplaceable method for benign end-stage liver disease. The risk evaluation for the waiting list recipients and for post-transplant survival could provide practical indication for organ allocation. In recent years, there are two major kinds of evaluation scores. The first kind of evaluation scores is based on model for end-stage liver disease(MELD) score,including SOFT/P-SOFT score,UCLA-FRS score and BAR score. The other evaluation system is based on the concept of acute-on-chronic liver failure,including CLIF-C-ACLF score,TAM score,AARC-ACLF score and COSSH-ACLF score. The scores based on ACLF have been shown superior power in predicting waiting list survival and post-transplant prognosis than MELD. This article reviews the two kinds of evaluation scores,aiming for the better allocation policy and the better prognosis of benign end-stage liver disease.

Review of risk evaluation scores for benign end stage liver diseases recipients / 中华外科杂志

Liver transplant is an unreplaceable method for benign end-stage liver disease. The risk evaluation for the waiting list recipients and for post-transplant survival could provide practical indication for organ allocation. In recent years, there are two major kinds of evaluation scores. The first kind of evaluation scores is based on model for end-stage liver disease(MELD) score,including SOFT/P-SOFT score,UCLA-FRS score and BAR score. The other evaluation system is based on the concept of acute-on-chronic liver failure,including CLIF-C-ACLF score,TAM score,AARC-ACLF score and COSSH-ACLF score. The scores based on ACLF have been shown superior power in predicting waiting list survival and post-transplant prognosis than MELD. This article reviews the two kinds of evaluation scores,aiming for the better allocation policy and the better prognosis of benign end-stage liver disease.

Gastrointestinal reconstruction by intestinal auto-transplantation after radical resection of neoplasms involving superior mesenteric artery: a preliminary consideration / 中华外科杂志

When abdominal neoplasms originating from the pancreas or nearby organs locally involving the superior mesenteric artery (SMA), complete resection is still the only hope for cure. However, SMA resection and reconstruction is a complex surgical procedure associated with high postoperative morbidity and mortality. Intestinal autotransplantation has recently emerged in clinical practice as a treatment option for selected patients with neoplasms involving the SMA. The original procedure involved en bloc removal of a tumor together with the intestine, ex vivo resection and reconstruction of gastrointestinal tract by an intestinal autograft. To further refine this complex procedure, a modified method was developed in which a segmental bowel autograft is selected and harvested first during the initial stage of the operation, and radical resection of the neoplasm is carried out thereafter. The modification would better protect a healthy bowel autograft from potential damage due to prolonged warm ischemia and allow the subsequent lengthy process of dissection to be performed in an unrushed manner. Furthermore, this alteration would better adhere to the general principles of minimal tumor manipulation during operation and potentially decrease the risks of tumor implantation during in vitro organ perfusion. Although intestinal autotransplantation has expanded eligibility for resection of otherwise unresectable lesions involving the SMA, its operative complexity, high risks, and post-operative complications largely limit its clinical applications.

Research Progress on Computer-Aided Skeleton-Based Individual Identification in Forensic Radiology / 法医学杂志

Individual identification based on imaging data of the skeleton of a corpse is a key technique for forensic identification. To reduce the influence of artificial factors, computer-aided semi-automatic or automatic individual identification has become one of the research directions of skeleton-based individual identification in forensic radiology. Therefore, this paper reviews and summarizes literatures related to estimation of anthropological information such as, age and sex by computer-aided forensic radiology bone characteristics and individual identification based on bone imaging characteristics, in order to provide reference on skeleton-based individual identification in forensic radiology.

Forensic Application of Plant Evidence / 法医学杂志

With the increasingly obvious role of plant evidence in case detection, forensic botany, which provides clues and evidence in crime scene investigation by using botanical research method has attracted growing attention. The common experimental techniques used in forensic botany are morphological examination, physical and chemical examination, molecular genetic examination, and so on. This paper briefly expounds the advantages and disadvantages of different test methods, summarizes the problems that need to be paid attention to in the application of forensic botany by arranging the related literatures and cases of forensic botanical research, in order to provide reference for scene investigation of cases.

Practice of pre-hospitalization in operation department of a hospital in Guangzhou / 中华医院管理杂志

Since the performance appraisal of national tertiary public hospitals was carried out, higher requirements have been put forward for the operation and management of hospitals. Under the premise of ensuring the quality of medical service and medical safety, how to save hospital operating costs and improve the efficiency is an urgent problem for hospital managers. Supported by information upgrading, a tertiary hospital in Guangzhou reformed the treatment process and carried out pre-hospitalization in surgical departments. Data showed that pre-hospitalization can significantly shorten the length of stay, reduce hospitalization costs, and improve the operation efficiency of the hospital.

Novel sternoclavicular hook-plate for treatment of proximal clavicle fracture with dislocation of sternoclavicular joint / 中国骨伤

OBJECTIVE@#To explore the efficacy of a novel sternoclavicular hook-plate for treatment of proximal clavicle fracture with dislocation of sternoclavicular joint.@*METHODS@#Retrospective analysis of 13 cases of proximal clavicle fracture with dislocation of sternoclavicular joint treated with sternoclavicular hook-plate from June 2011 to January 2019 in our department. There were 9 males and 4 females, aged 26 to 78 years old, with an average age of (54.08±13.91) years old. All the patients had closed injuries without damage of blood vessels and nerves. The patient's operation time, intraoperative blood loss, hospital stay time, and postoperative complications were recorded. Fracture healing and reduction were evaluated according to X-ray and CT after operation. Constant-Murley score and Rockwood sternoclavicular joint score were used to evaluate limb function at 12 months after operation.@*RESULTS@#All the patients were treated with sternoclavicular hook-plate. The operation time ranged from 50 to 76 min, with a mean of (54.08±13.91) min. The intraoperative blood loss ranged from 20 to 56 ml, with a mean of (46.08±11.15) ml. The hospital stay time ranged from 6 to 14 d, with a mean of (8.31±2.32) d. X-ray and CT examination on the second day after operation showed that all fractures and dislocations were anatomically reduced, and shoulder joint function exercise was performed early. All patients were followed up, and the duration ranged from 12 to 24 months, with a mean of (16.77±4.63) months. The healing time ranged from 9 to 13 d, with a mean of (11.00±1.75) d;and the bone healing time ranged from 3 to 4 months, with a mean of (3.65±0.46) months. There were no complications such as infection, internal fixation failure and nerve injury. At 12 months follow-up, the constant Murley score ranged from 78 to 100, with a mean of 87.83± 11.26; and Rockwood score ranged from 9 to 15, with a mean of 13.70±1.85. Among them, 11 cases were excellent, 1 case was good, and 1 case was general.@*CONCLUSION@#The use of the novel sternoclavicular hook-plate for treatment of proximal clavicle fracture with dislocation of sternoclavicular joint is an effectively internal fixation with high safety, allowing early functional exercise for patients.

CT appearances and short-term changes of COVID-19 in subclinical period / 中华放射学杂志

Objective:To explore the characteristics and short-term changes of high resolution CT (HRCT) in subclinical stage of COVID-19.Methods:The HRCT images of 17 COVID-19 patients in subclinical stage were analyzed retrospectively in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology and Wuhan Jinyintan Hospital from January 15 to 31, 2020. There were 4 males and 13 females, age ranged from 25.0 to 51.0 (39.8±7.5) years, who were closely contacted with other COVID-19 patients. The follow-up CT examination was performed within 3 to 6 days after the initial CT examination in all patients. Six patients were treated using anti-virus, anti-inflammation and symptomatic therapy, while the other 11 patients were untreated. The subclinical CT findings and short-term follow-up were analyzed, and the CT changes of short-term follow-up were summarized.Results:The lesions of 17 COVID-19 patients were mainly located at the lower lobes of bilateral lungs (at the left lower lobe in 9 cases and at the right lower lobe in 10 cases in the subclinical stage, at the left lower lobe in 9 cases and at the right lower lobe in 11 cases in the short-term follow-up). The number of involved lung segments increased from 46 in the subclinical stage to 90 in the short-term follow-up. In the subclinical stage, the multiple lesions were found in 13 cases, while in the short-term follow-up, the number of cases with multiple lesions decreased by 7, however the cases with focal lesions increased by 6 and diffuse lesions by 4. The lesions were mainly distributed under the pleura (13 cases) or along the bronchovascular bundle(10 cases). In the short-term follow-up, the lesion in 1 case expanded from the subpleural area to neighbouring bronchovascular bundle. There were 3 main types of the lesions, including ground glass nodule, ground glass opacity and crazy-paving pattern. In the subclinical stage and short-term follow-up, the ground glass opacities were seen in all the 17 cases. In the short-term follow-up, the number of cases with ground glass nodules decreased by 4 and crazy-paving pattern increased by 4. In 6 patients after treatment, the multiple ground glass nodules became single one in 3 cases, and in other 3 cases the multiple ground glass opacities were getting smaller. However, the scope of lesions in 11 patients without treatment enlarged.Conclusion:The HRCT features of the COVID-19 in subcilincal stage have some charicteristics, such as small ground glass nodule, ground glass opacity along the subpleural area or along the bronchovascular bundle, and the great changes can be seen in the short-term follow-up.

Effects of different lymph node dissection methods in radical resection of intrahepatic cholangiocarcinoma on the number of harvested lymph nodes and prognosis of patients / 中华消化外科杂志

Objective:To investigate the effects of different lymph node dissection methods in radical resection of intrahepatic cholangiocarcinoma (ICC) on the number of harvested lymph nodes and prognosis of patients.Methods:The retrospective cohort study was conducted. The clinicopathological data of 57 patients with ICC who underwent radical resection in the Second Affiliated Hospital of Nanchang University from January 2011 to January 2016 were collected. There were 25 males and 32 females, aged from 45 to 72 years, with an average age of 59 years. Of the 57 patients, 36 undergoing en bloc fusion lymph node dissection and 21 undergoing conventional lymph node dissection were allocated into observation group and control group, respectively. Observation indicators: (1) intraoperative situations; (2) results of postoperative pathological examination; (3) postoperative complications; (4) follow-up. Follow-up using outpatient examination or telephone interview was performed once every six months after hospital discharge to detect survival of patients up to January 2019. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was done using the t test. Measurement data with skewed distribution were described as M (range), and comparison between groups was analyzed by the Mann-Whitney U test. Count data were described as absolute numbers or percentages, and comparison between groups was analyzed using the chi-square test or Fisher exact propability. Comparison of ordinal data between groups was analyzed using the Mann-Whitney U test. The survival curve and rate were drawn and calculated using the Kaplan-Meier method, and survival of patients was analyzed by the Log-rank test. Results:(1) Intraoperative situations: operation time, volume of intraoperative blood loss, hepatic portal occlusion time, cases with <3 segments of hepatectomy and ≥3 segments of hepatectomy for the observation group were (218±53)minutes, (266±24)mL, (21±9)minutes, 5, 31, respectively, versus (207±45)minutes, (270±23)mL, (19±8)minutes, 2, 19 for the control group, showing no significant difference in the above indicators between the two groups ( t=-0.530, 0.774, 0.590, χ2=0.004, P>0.05). (2) Results of postoperative pathological examination: the number of harvested lymph nodes and percentage of positive lymph nodes of the observation group were 11±3 and 16.58%(66/398), respectively, versus 5±2 and 6.80%(7/103) of the control group, showing significant differences in the above indicators between the two groups ( t=-9.454, 2.148, P<0.05). (3) Postoperative complications: cases with Clavien-Dindo grade Ⅰ-Ⅱ complications or Clavien-Dindo grade Ⅲ-Ⅳ complications, cases with bile leakage, cases with postoperative hemorrhage, cases with incision infection, cases with gastroparesis were respectively 35, 1, 6, 1, 8, 0 in the observation group and 19, 2, 3, 1, 1, 2 in the control group. There was no significant difference in the cases with complications of Clavien-Dindo classification, cases with bile leakage, cases with incision infection between the two groups ( χ2=0.236, 0.000, 1.870, P>0.05) and no significant difference in the cases with postoperative hemorrhage, cases with gastroparesis between the two groups ( P>0.05). (4) Follow-up: 57 patients were followed up for 5-42 months, with a median time of 36 months. The survival time and postoperative 3-year survival rate were respectively 36 months (range, 8-42 months) and 66.7% of the observation group, versus 23 months (range, 5-39 months) and 38.1% of the control group, showing significant differences in the above indicators between the two groups ( Z=-2.253, χ2=5.317, P<0.05). Conclusion:For radical resection of ICC, the en bloc fusion lymph node dissection is beneficial to increase the number of harvested lymph nodes and improve survival rate of patients.

Transcriptomic Features of Echinococcus granulosus Protoscolex during the Encystation Process

Cystic echinococcosis (CE) is a zoonotic infection caused by Echinococcus granulosus larvae. It seriously affects the development of animal husbandry and endangers human health. Due to a poor understanding of the cystic fluid formation pathway, there is currently a lack of innovative methods for the prevention and treatment of CE. In this study, the protoscoleces (PSCs) in the encystation process were analyzed by high-throughput RNA sequencing. A total of 32,401 transcripts and 14,903 cDNAs revealed numbers of new genes and transcripts, stage-specific genes, and differently expressed genes. Genes encoding proteins involved in signaling pathways, such as putative G-protein coupled receptor, tyrosine kinases, and serine/threonine protein kinase, were predominantly up-regulated during the encystation process. Antioxidant enzymes included cytochrome c oxidase, thioredoxin glutathione, and glutathione peroxidase were a high expression level. Intriguingly, KEGG enrichment suggested that differentially up-regulated genes involved in the vasopressin-regulated water reabsorption metabolic pathway may play important roles in the transport of proteins, carbohydrates, and other substances. These results provide valuable information on the mechanism of cystic fluid production during the encystation process, and provide a basis for further studies on the molecular mechanisms of growth and development of PSCs.