This study analyzed the clinical and laboratory data of 3 children diagnosed with mitochondrial disease-associated primary adrenal insufficiency (PAI) at the Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from October 2018 to November 2023.All patients were normal at birth but gradually developed symptoms and were diagnosed with PAI between the ages of 1 year and 1 month and 7 years and 3 months.The children presented typical clinical symptoms of PAI, including skin and mucosal hyperpigmentation (3 cases), electrolyte disturbances (3 cases), and hypoglycemia (2 cases), as well as multisystem abnormalities related to mitochondrial disease, including recurrent infections, growth retardation, cachexia, and hyperlactatemia.Genetic testing revealed significant single deletions in mitochondrial DNA in all patients: Patient 1: m.11219_15954del, Patient 2: m.8483_13459del, and Patient 3: m.8649_16084del.Treatment with Hydrocortisone acetate replacement therapy improved the electrolyte disturbances and hypoglycemia, but issues with recurrent infections, growth retardation, and cachexia persisted.This study suggests that in clinical practice, the possibility of mitochondrial disease should be highly suspected when PAI patients present with multisystem abnormalities, especially in conjunction with hyperlactatemia.

This study analyzed the clinical and laboratory data of 3 children diagnosed with mitochondrial disease-associated primary adrenal insufficiency (PAI) at the Guangzhou Women and Children′s Medical Center, Guangzhou Medical University from October 2018 to November 2023.All patients were normal at birth but gradually developed symptoms and were diagnosed with PAI between the ages of 1 year and 1 month and 7 years and 3 months.The children presented typical clinical symptoms of PAI, including skin and mucosal hyperpigmentation (3 cases), electrolyte disturbances (3 cases), and hypoglycemia (2 cases), as well as multisystem abnormalities related to mitochondrial disease, including recurrent infections, growth retardation, cachexia, and hyperlactatemia.Genetic testing revealed significant single deletions in mitochondrial DNA in all patients: Patient 1: m.11219_15954del, Patient 2: m.8483_13459del, and Patient 3: m.8649_16084del.Treatment with Hydrocortisone acetate replacement therapy improved the electrolyte disturbances and hypoglycemia, but issues with recurrent infections, growth retardation, and cachexia persisted.This study suggests that in clinical practice, the possibility of mitochondrial disease should be highly suspected when PAI patients present with multisystem abnormalities, especially in conjunction with hyperlactatemia.

'Zhizhang Guhong Chongcui' is a new cultivar of Prunus mume with cross-cultivar group characteristics. It has typical characteristics of cinnabar purple cultivar group and green calyx cultivar group. It has green calyx, white flower, and light purple xylem, but the mechanism remains unclear. In order to clarify the causes of its cross-cultivar group traits, the color phenotype, anthocyanin content and the expression levels of genes related to anthocyanin synthesis pathway of 'Zhizhang Guhong Chongcui', 'Yuxi Zhusha' and 'Yuxi Bian Lü'e' were determined. It was found that the red degree of petals, sepals and fresh xylem in branches was positively correlated with the total anthocyanin content. MYBɑ1, MYB1, and bHLH3 were the key transcription factor genes that affected the redness of the three cultivars of flowers and xylem. The transcription factors further promoted the high expression of structural genes F3'H, DFR, ANS and UFGT, thereby promoting the production of red traits. Combined with phenotype, anthocyanin content and qRT-PCR results, it was speculated that the white color of petals of 'Zhizhang Guhong Chongcui' were derived from the high expression of FLS, F3'5'H, LAR and ANR genes in other branches of cyanidin synthesis pathway, and the low expression of GST gene. The green color of sepals might be originated from the relatively low expression of F3'H, DFR and ANS genes. The red color of xylem might be derived from the high expression of ANS and UFGT genes. This study made a preliminary explanation for the characteristics of the cross-cultivar group of 'Zhizhang Guhong Chongcui', and provided a reference for molecular breeding of flower color and xylem color of Prunus mume.
Animals ; Anthocyanins ; DNA Shuffling ; Flowers/genetics* ; Porifera ; Prunus/genetics* ; Glutamine/analogs & derivatives* ; Plant Extracts

Objectives:To explore the biological characteristics of human nucleus pulposus decellularized extracellular matrix(NP-dECM)scaffolds with different degrees of degeneration(Ⅱ,Ⅲ,Ⅳ),and to select the NP-dECM scaffold with the best biological characteristics.Methods:Nucleus pulposus mesenchymal stem cells(NPMSCs)were isolated by collagenase type n digestion method.Cell adhesion,cell morphology and growth of NPMSCs were observed under microscope.The cell surface markers CD73,CD90,CD105,CD34,CD45,HLA-DR were detected by flow cytometry,and the differentiation ability of NPMSCs was observed by adipogenic,osteogenic and chondrogenic induction,so as to confirm that they were the seed cells needed for this study.Human NP-dECM scaffolds with different degrees of degeneration were prepared by physical,stain remover and nuclease(group Ⅱ-D,Ⅲ-D and Ⅳ-D).The decellular effect of NP-dECM scaffold was evaluat-ed by visual observation,quantitative and qualitative detection of DNA and extracellular matrix components,and SEM.The cytocompatibility of human NP-dECM scaffolds with different degrees of degeneration was e-valuated using CCK-8 method and living/dead cells in vitro.Results:Human NPMSCs isolated and cultured from the nucleus pulposus tissue grew adherent,and the cells were short spindle shaped and spindle-shaped.The third passage of human NPMSCs expressed high levels of surface markers such as CD73,CD90 and CD105,and low levels of surface markers such as CD34,CD45 and HLA-DR.It met the criteria of mes-enchymal stem cells.Human NP-dECM scaffolds with three degrees of degeneration remained intact,and the scaffolds of each group were loose and porous under electron microscope.HE staining,DAPI staining and DNA determination showed that the NP-dECM scaffold effectively removed cell components in the three de-grees of degeneration.The quantitative and qualitative detection results of GAG and HYP showed that ECM was well retained,and the GAG retention was in the order of group Ⅱ-D＞group Ⅲ-D＞group Ⅳ-D from high to low.There was no statistical significance in HYP content between groups Ⅱ-D and Ⅲ-D,which was higher than that in group Ⅳ-D.The results of CCK-8 and live/dead cell staining showed that groups Ⅱ-D,Ⅲ-D and Ⅳ-D showed good cytocompatibility.Conclusions:The Ⅱ degree degeneration of human nucleus pulposus tissue is the best choice for the preparation of human NP-dECM scaffold.

Objective:To investigate the expression levels and internal relations of matrix metalloproteinase-9(MMP-9), interleukin(IL)-1β and tissue inhibitor of metalloproteinase-1(TIMP-1) within 24 h after cerebral hypoxia and ischemia(HI) in neonatal rats.Methods:SD rats aged 7 days were randomly divided into HI group( n=40), and sham operation group(sham group, n=40). The HI models were established and divided into 5 subgroups with those at 8 cases in each group at 5 postoperative time points(0, 4, 8, 12, 24 h). Pathological changes of cerebral cortex were observed by HE staining, and the expressions of MMP-9, IL-1β and TIMP-1 were detected by Western blot and RT-PCR.In addition, the hypoxia models of mouse hippocampal neuron HT22 cells were established.With adding MMP-9 inhibitor and MMP-9 agonist, the relations among MMP-9, IL-1β and TIMP-1 were further confirmed. Results:Slight nuclear abnormalities and cell body swelling occurred in cerebral cortex at 4 h after HI, and neuronal eosinophilic changes were most obvious at 12 h. Compared with those at 8 h and 12 h, the expressions of MMP-9 and IL-1β at protein and mRNA level significantly increased at 24 h after HI( P<0.01). While MMP-9 inhibitor was added to HT22 cells under hypoxia at 4 h, IL-1β mRNA expression was down-regulated. Conclusion:In the early stage of hypoxic ischemic encephalopathy in newborn rats, the expressions of MMP-9 and TIMP-1 are time-dependent.Inhibition of MMP-9 expression could reduce the level of Interleukin-1β.

Objective:To investigate and analyze the energy metabolism characteristics and the correlation between energy metabolism and the risk of secondary bacterial infection in patients with hepatitis B virus-related chronic liver disease (HBV-CLD).Methods:Data of 183 cases admitted to the Mengchao Hepatobiliary Hospital of Fujian Medical University from November 2017 to November 2020 were retrospectively analyzed. 79 cases of chronic hepatitis B, 51 cases of hepatitis B-related liver cirrhosis, and 53 cases of hepatitis B-related liver failure were collected. Among them patients with liver failure and decompensated liver cirrhosis were defined as severe liver disease group. The Quark RMR indirect calorimetry (COSMED Corporation, Italy) was used to exam the patients' energy metabolism condition, and the incidences of secondary bacterial infection of the patients during hospitalization were recorded. Shapiro-Wilk test and normal QQ plot were used to analyze the normal distribution of continuous variable data, which was consistent with the normal distribution and was described by mean ± standard deviation. In addition, if it did not conform to the normal distribution, the median and interquartile distance were used to describe it. Levene’s test was used to test the homogeneity of variance of the data, which was consistent with the normal distribution. The t-test was used to compare the means of the two groups of samples. One-way analysis of variance was used to compare the mean values of the three groups of samples, and then the Tukey's test was used to compare the two groups. If the variance was uneven or did not conform to the normal distribution, the Wilcoxon rank sum test was used to compare the differences between the two groups. Kruskal-Wallis test (H test) was used to compare the differences between the three groups of samples, and then the Dunnett’s test (Z test) was used for comparison between the two groups. Categorical variable data were analyzed using chi-square test. Logistic regression analysis was used to screen independent risk factors, and the criteria for variable inclusion ( P < 0.05). Results:The respiratory entropy (RQ) and non-protein respiratory entropy (npRQ) of the three groups had statistically significant difference ( P < 0.05). Among them, the RQ and npRQ of the chronic hepatitis B group were higher than hepatitis B-related liver cirrhosis group and hepatitis B-related liver failure group. There were statistically significant differences in fat oxidation rate (FAT%) and carbohydrate oxidation rate (CHO%) between the three groups ( P < 0.05). Compared with hepatitis B-related liver cirrhosis group and hepatitis B-related liver failure group, chronic hepatitis B group ( P < 0.05) had lower FAT% and higher CHO%. There were no statistically significant differences in the measured and predicted resting energy expenditure and protein oxidation rate (PRO%) between the three groups. The incidence of secondary bacterial infection in patients with severe liver disease was 48.39% (45/93). Compared with the non-infected group, the RQ and npRQ values ??of the infected group were significantly decreased ( P < 0.05), while FAT% was significantly increased ( P < 0.05). Logistic regression analysis showed that glutamyltransferase, cholesterol, and npRQ were independent risk factors for secondary bacterial infections in patients with severe liver disease. Glutamyltransferase elevation, and cholesterol and npRQ depletion had suggested an increased risk of secondary bacterial infection. Subgroup analysis of patients with hepatitis B-related liver failure also showed that compared with non-infected group, RQ value and npRQ value of secondary bacterial infection group were significantly decreased ( P < 0.05), while FAT% was significantly increased ( P < 0.05). Conclusion:Patients with hepatitis B virus-related chronic liver disease generally have abnormal energy metabolism. Low RQ, npRQ, CHO% and high FAT% are related to the severity of the disease; while npRQ reduction is related to the risk of secondary bacterial infection in patients with severe liver disease, and thus can be used as a clinical prognostic indicator.

Objective: To analyze the information about coronavirus disease 2019 (COVID-19) on WeChat official accounts of centers for disease control and prevention (CDCs) in Zhejiang Province from January 20 to February 5,2020,so as to provide reference for improving the effects of health communication by WeChat official accounts. Methods: The number,content and pageview of the information about COVID-19 on WeChat official accounts of one provincial and eleven municipal CDCs from January 20 to February 5 were collected and analyzed. The number of new followers and WeChat communication power index (WCI) were employed to evaluate the communication effect. Results: By February 5,those WeChat public official accounts pushed 629 pieces of information about COVID-19. The pageviews were 3 713 428 in total and 5 903.70 on average. There were totally 633 008 followers,including 110 341 new followers which contributed to a growth rate of 21.11%. The average WCI was 677.81. The WCIs of eight official accounts were higher than 500,with “Zhejiang Health Education” the highest (1 021.95). The daily pageviews peaked on January 20,21,25 and 31. Among the top 15 pieces of information in pageviews,there were 7 pieces for epidemic announcements,3 pieces for popular science and 5 pieces for behavioral intervention. Conclusions The WeChat official accounts of CDCs in Zhejiang Province pushed the information about COVID-19 in line with the progress of the epidemic and the demand of the public,leading to a higher attention and better communication effect.

The pathogenesis of icteric hepatitis is that pathogenic dampness blocks the middle energizer or blood stasis blocks the bile duct,so that the bile is not excreted normally and moves to the skin surface,which makes the skin yellow.Traditior al Chinese medicine (TCM) has unique advantages in the prevention and treatment of icteric hepatitis.This article systematically introduces the etiology and pathogenesis of icteric hepatitis and the TCM syndrome differentiation therapy for icteric hepatitis,in order to provide a reference for the clinical treatment of icteric hepatitis and the improvement in its prognosis.

Objective To apply neural contrast sensitivity (NCS) on evaluating the dark adapted and night vision goggle (NVG) aided visual performance upon the comparison with contrast sensitivity (CS) and to investigate the value of NCS in the assessment of visual performance.Methods Eight healthy male volunteers (8 eyes) were recruited for this study,and the right eye was tested in this experiment.CS curves of volunteers in light adaptation,dark adaptation and with NVG were measured by Vision Monitor System in different environment luminance levels (10-1-10-4 cd/m2).Modulation transfer function (MTF) curves were measured by optical quality analysis system.NCS was defined as the ratio of CS and MTF.Results In lower and medium frequency range (spatial frequency was 0.6-3.4 cycle/degree),dark adaptation made subject's NCS and CS mount up considerably in the scotopic vision environment,but less effect in mesopic vision environment.NVG considerably improved subject's NCS and CS both in scotopic and mesopic vision environments (spatial frequency was 1.1-7.1 cycle/degree) and more effective than in dark adaptation.In high spatial frequency range,neither dark adaptation nor NVG had obvious effect on improving subject's NCS and CS.Conclusions Both dark adaptation and snooper scopes can improve visual performance in the scotopic vision environment,but snooper scopes is better.CS curves can represent the comprehensive visual performance,NCS reflects the function of the retina-brain neural system.But NCS and MTF would be helpful to find the reasons in case of poor CS measured.The examination combined NCS and MTF with CS is suggested.

Objective To apply neural contrast sensitivity (NCS) on evaluating the dark adapted and night vision goggle (NVG) aided visual performance upon the comparison with contrast sensitivity (CS) and to investigate the value of NCS in the assessment of visual performance.Methods Eight healthy male volunteers (8 eyes) were recruited for this study,and the right eye was tested in this experiment.CS curves of volunteers in light adaptation,dark adaptation and with NVG were measured by Vision Monitor System in different environment luminance levels (10-1-10-4 cd/m2).Modulation transfer function (MTF) curves were measured by optical quality analysis system.NCS was defined as the ratio of CS and MTF.Results In lower and medium frequency range (spatial frequency was 0.6-3.4 cycle/degree),dark adaptation made subject's NCS and CS mount up considerably in the scotopic vision environment,but less effect in mesopic vision environment.NVG considerably improved subject's NCS and CS both in scotopic and mesopic vision environments (spatial frequency was 1.1-7.1 cycle/degree) and more effective than in dark adaptation.In high spatial frequency range,neither dark adaptation nor NVG had obvious effect on improving subject's NCS and CS.Conclusions Both dark adaptation and snooper scopes can improve visual performance in the scotopic vision environment,but snooper scopes is better.CS curves can represent the comprehensive visual performance,NCS reflects the function of the retina-brain neural system.But NCS and MTF would be helpful to find the reasons in case of poor CS measured.The examination combined NCS and MTF with CS is suggested.