[Objective]To investigate and explore the characteristics and influencing factors of glucose metabolism in children with β-thalassemia major(β-TM)after allogeneic hematopoietic stem cell transplantation(allo-HSCT).[Methods]The follow-up data of 41 patients with β-TM who underwent HSCT at Hematopoietic Stem Cell Transplantation Department of Children's Medical Center of Sun Yat-sen Memorial Hospital,Sun Yat-sen University were retrospectively analyzed.Their glucose metabolism characteristics were evaluated through laboratory tests and the related influencing factors were analyzed.[Results]In the study,41.46％(17/41)of patients developed abnormal glucose homeostasis after HSCT.Among them,82.35％(14/17)characterized by insulin resistance,but no cases of diabetes mellitus were found.The results of insulin releasing test and oral glucose tolerance test(OGTT)showed that 45.00％(9/20)of patients had abnormal insulin releasing curve and 40.0％(8/20)had delayed serum glucose peak.The average age of HSCT in abnormal glucose homeostasis group was significantly older than that in the normal glucose homeostasis group[(8.8±3.9)years old vs(6.0±3.1)years old,P=0.015].[Conclusions]Patients with β-TM after HSCT may develop abnormal glucose homeostasis,consists largely of insulin resistance.The elder age of HSCT(≥7 years old)is a risk factor for abnormal glucose homeostasis in β-TM patients after HSCT.It is recommended to regularly monitor glucose metabolism indicators in β-TM children after HSCT,especially in elderly transplant recipients.

Objective:To understand the burden of chronic kidney disease (CKD) and its risk factors in Fujian Province during 1990-2019.Methods:Based on the Global Burden of Disease Study 2019, the incidence rate, mortality rate and disability-adjusted life years (DALYs) of CKD in Fujian from 1990 to 2019 were calculated. An age-period-cohort model was used to estimate the effects of age, period, and cohort on age-standardized incidence rate (ASIR), age-standardized mortality rate (ASMR), and age-standardized DALY rate (ASDR) of CKD. Comparative risk assessment theory was used to calculate the potential attributable DALYs due to risk factors.Results:In 2019, the ASIR of CKD in Fujian exceeded the national average. The ASIR of CKD showed an increasing trend from 1990 to 2019, but the ASMR and ASDR of CKD exhibited decreasing trends during the same period. In 2019, the ASIR of CKD was higher in women than in men, while the ASMR and ASDR were higher in men than in women. Age-period-cohort analysis indicated that ASIR, ASMR, and ASDR of CKD increased with age. The period effect for ASIR decreased first before increase, while the period effect for ASMR and ASDR displayed fluctuating trends. The cohort effect showed an upward trajectory for ASIR, but a stable status before downward trajectories for ASMR and ASDR. Compared with 1990, except the increase in the ASDR of CKD attributed to high BMI and high temperatures, the ASDR of CKD attributed to other risk factors all showed decreases in 2019. However, the ASDR attributed to high sodium intake remained higher compared with the global average.Conclusion:The burden of CKD remains heavy in Fujian, and it is necessary to reduce the attributable risk factors, such as high sodium intake and high BMI, to address this problem.

Objective:To understand the burden of chronic kidney disease (CKD) and its risk factors in Fujian Province during 1990-2019.Methods:Based on the Global Burden of Disease Study 2019, the incidence rate, mortality rate and disability-adjusted life years (DALYs) of CKD in Fujian from 1990 to 2019 were calculated. An age-period-cohort model was used to estimate the effects of age, period, and cohort on age-standardized incidence rate (ASIR), age-standardized mortality rate (ASMR), and age-standardized DALY rate (ASDR) of CKD. Comparative risk assessment theory was used to calculate the potential attributable DALYs due to risk factors.Results:In 2019, the ASIR of CKD in Fujian exceeded the national average. The ASIR of CKD showed an increasing trend from 1990 to 2019, but the ASMR and ASDR of CKD exhibited decreasing trends during the same period. In 2019, the ASIR of CKD was higher in women than in men, while the ASMR and ASDR were higher in men than in women. Age-period-cohort analysis indicated that ASIR, ASMR, and ASDR of CKD increased with age. The period effect for ASIR decreased first before increase, while the period effect for ASMR and ASDR displayed fluctuating trends. The cohort effect showed an upward trajectory for ASIR, but a stable status before downward trajectories for ASMR and ASDR. Compared with 1990, except the increase in the ASDR of CKD attributed to high BMI and high temperatures, the ASDR of CKD attributed to other risk factors all showed decreases in 2019. However, the ASDR attributed to high sodium intake remained higher compared with the global average.Conclusion:The burden of CKD remains heavy in Fujian, and it is necessary to reduce the attributable risk factors, such as high sodium intake and high BMI, to address this problem.

ObjectiveTo explore the clinical features and causative genes of short stature children with unknown etiology， providing evidence for precise clinical diagnosis and treatment. MethodsThe study recruited children with suspected but undiagnosed short stature from the pediatric endocrinology department in our hospital between January 2018 and August 2022. A retrospective analysis was performed on the clinical manifestations， laboratory test and whole exome sequencing （WES） results. Causative genes were classified and analyzed according to different pathogenic mechanisms. ResultsA total of 48 children （30 boys and 18 girls） were enrolled， aged 7.73 ± 3.97 years， with a height standard deviation score （ HtSDS） of -3.63 ± 1.67. Of the patients， 33 （68.8%） suffered from facial anomalies， 31 （64.6%） from skeletal abnormalities， 26 ［54.2%， 61.5% of whom born small for gestational age （SGA）］ from perinatal abnormalities， 24 ［50.0%， 87.5% of whom with growth hormone （GH） peak concentration below normal］ from endocrine disorders and 21（43.8%） had a family history of short stature. Laboratory tests showed that GH peak concentration following stimulation test was （9.72 ± 7.25） ng/mL， IGF-1 standard deviation score was -0.82 ± 1.42， the difference between bone age and chronological age was -0.93 ± 1.39 years. Of the 25 cases with mutant genes found by WES， 14 （56.0%） had pathogenic mutation， 6 （24.0%） likely pathogenic mutation， and 5 （20.0%） mutation of uncertain significance. Pathogenic and likely pathogenic variants were identified in 14 genes， including 10 affecting intracellular signaling pathways （PTPN11， RAF1， RIT1， ARID1B， ANKRD11， CSNK2A1， SRCAP， CUL7， SMAD4 and FAM111A） and 4 affecting extracellular matrix （ECM） components or functions （ACAN， FBN1， COL10A1 and COMP）. ConclusionsA rare monogenic disease should be considered as the possible etiology for children with severe short stature accompanied by facial anomalies， disproportionate body types， skeletal abnormalities， SGA， GH peak concentration below normal and a family history of short stature. WES played an important role in identifying the monogenic causes of short stature. This study indicated that affecting growth plate cartilage formation through intracellular signaling pathways and ECM components or functions was the main mechanism of causative genes leading to severe short stature in children. Further research may help discover and study new pathogenic variants and gene functions.

OBJECTIVE To comprehensively evaluate the quality of Eriobotrya japonica leaves from different producing areas. METHODS The contents of alcohol-soluble extracts were determined by hot-dipping method using 30 batches of E. japonica leaves from different producing areas as samples. The contents of total flavonoids and total triterpene acids were determined by ultraviolet spectrophotometry. The contents of five kinds of triterpenic acids （euscaphic acid，crataegolic acid，corosolic acid，oleanolic acid and ursolic acid） were determined by HPLC. The quality of E. japonica leaves from different producing areas was comprehensively evaluated by using entropy weight technique for order preference by similarity to an ideal solution （TOPSIS）. The bivariate correlation analysis of E. japonica leaves was conducted by SPSS 22.0 software in terms of weight， comprehensive evaluation value， the content of alcohol-soluble extract， the contents of total flavonoids， total triterpene acids and five triterpenic acids. RESULTS The contents of alcohol-soluble extract in 30 batches of E. japonica leaves were （24.56±0.08）%-（34.85±0.13）%； the contents of total flavonoids were （4.69±0.11）-（14.23±0.27） mg/g； the contents of total triterpene acid were （27.58±0.59）- （63.95±1.27） mg/g； the contents of euscaphic acid， crataegolic acid， corosolic acid， oleanolic acid and ursolic acid were （0.728± 0.011）-（6.064±0.063）， （0.526±0.013）-（3.245±0.022）， （1.222±0.025）-（8.807±0.094）， （0.856±0.021）-（2.931±0.075）， （4.704±0.087）-（11.806±0.283） mg/g， respectively. The analysis result of entropy weight TOPSIS method showed that the top three samples with comprehensive evaluation values （No.Kjcx-5） were S14 （Huotian Town， Yunxiao County， Zhangzhou，Fujian）， S19 （Qinnan District， Qinzhou， Guangxi） and S29 （Guoyang County， Bozhou， Anhui）. Comprehensive evaluation 0596-2559522。E-mail：jxrcwxp@163.com of E. japonica leaves was positively correlated with the contents of five kinds of triterpenic acids， such as euscaphic acid， crataegolic acid， corosolic acid， oleanolic acid and ursolic acid （P＜0.01）. The weight of E. japonica leaves was positively correlated with the comprehensive evaluation value （P＜0.01）. CONCLUSIONS The qualities of E. japonica leaves from different producing areas are very different. Among them， the qualities of E. japonica leaves from Huotian Town， Yunxiao County， Zhangzhou of Fujian， Qinzhou Qinnan District of Guangxi， and Bozhou Guoyang County of Anhui are relatively better. The weight of E. japonica leaves is positively correlated with their quality.

Objective To analyze the clinical manifestations and gene mutations of rare causes of primary adrenal insufficiency (PAI) in childhood.Methods The clinical features,laboratory tests and gene mutation of 13 patients with PAI in our hospital from September 2010 to August 2017 were analyzed retrospectively.Patients with congenital adrenal hyperplasia,X-linked adrenoleukodystrophy with neurological onset or a clear family history,and autoimmune adrenal insufficiency were excluded.Results The median age of 13 cases (12 males,1 female) was 3 years and 10 months.Medical history or clinical manifestations on the first visit included hyperpigmentation,electrolyte imbalance/salt-wasting crisis,gastrointestinal symptoms,and fatigue,etc.All developments of external genitalia were normal.All cases presented with decreased serum cortisol and increased ACTH levels.Some of the cases showed decreased aldosterone level and plasma renin activity,while 17α-hydroxyprogesterone,testosterone,and androstenedione were in the normal range.Part of cases revealed delayed bone age and adrenal atrophy.Three gene mutations were detected in 13 patients,including NR0B 1 gene (9/13),ABCD 1 gene (3/13),and CYP 11A 1 gene (1/13).NR0B1,and ABCD1 gene mutations were pathogenic mutations,consistent with clinical characteristics.CYP11A1 gene mutation was heterozygote,which cannot fully explain the clinical features.Conclusion PAI in childhood presents common clinical manifestations of adrenal insufficiency,e.g.hyperpigmentation and electrolyte imbalance/sah-wasting crisis,but without specificity.Gene mutational analysis is necessary for precise diagnosis and prognosis estimation.NR0B1 and ABCD1 gene mutations were common in childhood with rare causes of PAI.

Background Retinal Müller cells are important gliocytcs and the source of retinal stem cells.Researching the biological behavior of Müller cells is of important significance to the study on retinal physiopathological process and stem cell therapy of retinal diseases.To establish a stable culture method of Müller cells is a solid basis of relative basic research.Objective This study was to establish a simple and stable method of isolation and culture of human retinal Müller cells and provide sufficient and high-quality Müller cell source.Methods Human retinal Müller cells were isolated from healthy human donor eyes.The mixture solution of hyaluronidase (100 U) and 0.25％ trypsin were used to digest chopped retinal tissue.The DMEM/F12 medium with 20％ fetal bovine serum (FBS) was added to stop the digestion process.RPMI1640 medium with 20％ FBS was used to culture the cell for 72 hours and then replaced the half medium.The cells were passaged by the RPMI1640 medium with 20％ FBS.The morphology of the cells were examied under the optical microscope,and the expressions of glial fibrillary acidic protein (GFAP),a marker of gliocytes,and glutamine synthetase (GS),a special marker of retinal Müller cells,were detected by immunochemistry and immunofluorescence technology.Results Human retinal Müller cells were successfully isolated by enzyme mixture solution of hyaluronidase (100 U) and O.25％ trypsin.The cells were adherent to walls 24 hours after primary culture and completely merged 9-10 days after culture.The cells showed oval in shape with abundant cytoplasm,and a part of cells presented with cone-shaped bulge bilaterally and ectasia in the posterior containing large nuclei.After cells passage,the cells were enlarged and grew toward polygonal shape.The positive expression of GFAP was observed in more than 95％ cells and strongly positive expression of GS was observed in more than 90％ cells by immunohistochemstry and immunofluorescent staining.Conclusions Human retinal Müller cells can be successfully isolated by hyaluronidase combined with trypsin digestion.Abundent and pure human retinal Müller cells can be obtained by successively using RPMI1640 medium with 20％ FBS and 10％ FBS.

Background Stem cell transplantation represents a promising treatment option for patients suffering from degenerative disorders.Accumulating evidences indicate that mesenchymal stem cells (MSCs) are able to differentiate into retinal pigment epithelial (RPE)-like cells.However,MSCs are difficult to obtain.Human adipose mesenchymal stem cells (ADSCs) are proved to have similar properties to MSCs,but relevant study is less.Objective This study was to assess the feasibility of human ADSCs differentiating into RPE-like cells and the safety of its application in vivo.Methods The third generation of human ADSCs were incubated into 6-well plate,and 100 ng/ml epithelial growth factor,50 μ mol/L taurine and 5×10-7 mol/L retinoic acid were added into the medium 12 hours after cultured to induce the cells,and conventional cultured cells were used as the control group.Induced cells were traced with PKH26,and Pan-cytoke ratin (Pan-CK) monoclonal antibody was used to identify the cells under the fluorescence microscope.Induced RPE-like cell suspension of 1 μl was intravetreally injected in the right eyes of 6 BALB/c mice,and equal volume of PBS was used in the same way in another 6 mice.The animals were sacrificed 1 month after injection,and the retinal morphology was examined by histopathology under the optical microscope.The ultrastructure of retinal ganglion cells (RGCs) was examined by the transmission electron microscope.The use and care of the animals complied with Regulations for the Administration of Affair Concerning Experimental Animals by State Science and Technology Commission.Results Cultured human ADSCs grew well with the slender polygone shape.Cell membranes showed the red fluorescence for PKH26 after induced.In addition,Pan-CK was expressed in the cell membranes with the red fluorescence in the induced cells,but the response was absent in the control cells.One month after intravitreal injection,induced cells located on the retinal surface,and the retinal morphology was clear under the optical microscope.No abnormality in RGCs was seen under the transmission electron microscope.Conclusions Human ADSCs can differentiate into RPE-like cells after induction.PKH26 can mark induced cells well.There is no adverse effect of induced cells on retina after intravitreal injection in a short-term duration in mice.

Pathology is an important basic course in higher vocational nursing,with a wealth of humanistic education.But in the actual teaching process,the humanistic education is often ignored because of too much basic knowledge teaching.Our teaching and research section used questionnaire to find the gaps between current situation,goal of humanistic education and students' needs.By enhancing teachers' humanistic awareness and discovering the entrance to humanistic education,we targeted to professional ideological education,guided students to pay attention to humanistic education and helped them to cultivate humanistic caring capability,and students all had good evaluation on it.It helped to solve the problem of both teaching and education to students in short time under pressure.

AIM: To observe the effect of ginkgolide B (CB) on the intracellular calcium ion concentration ( [ Ca~(2+) ]_i) and mitochondrial function of cultured rat retinal neurons in vitro. METHODS: in vitro primary culture of rat retinal neurons was used in the experiment. The apoptosis model of glutamate - induced retinal neurons was established and co - cultured with ginkgolide B. The [ Ca~(2+) ]_i and mitochondrial membrane potential of the retinal neurons were detected by laser scanning confocal microscope. RESULTS: Glutamate decreased the survival rate of retinal neurons, increased the apoptosis and the [ Ca~(2+) ]_i, lowered the mitochondrial membrane potential. The [ Ca~(2+) ]_i was clearly diminished and the mitochondrial membrane potential was significantly increased with the GB intervention, and the apoptosis decreased significantly. CONCLUSION: GB protects retinal neurons from glutamate induced neurotoxicity. The effect of GB on retinal neurons might be due to its ability to decrease the [Ca~(2+) ]_i and increase mitochondrial membrane potential.