Objective To investigate the effects of anti-HLA donor-specific antibodies(DSA)and desensitization for DSA+patients on engraftment of haploidentical hematopoietic stem cell transplantation(haplo-HSCT).Methods The patients who underwent haplo-HSCT and examinations for HLA antibodies and DSA in our department from March 2017 to July 2023 were recruited in this study.The effects of desensitization measure on engraftment in the DSA+patients after haplo-HSCT were analyzed.Results Among the 70 patients who underwent haplo-HSCT and test for HLA antibodies,15(21.4％)patients were DSA positive,including 7(46.7％)cases of strong positive,3(20.0％)cases of moderate positive,and 5(33.3％)cases of weak positive.The median duration for neutrophil implantation was significantly extended in the DSA+patients than the negative patients(P=0.027).For the 6 patients developed graft failure(GF),4 were DSA+which was statistically higher than the DSA-patients(P=0.025).Multivariate regression analysis showed that DSA was an independent factor affecting GF(HR=9.273,95％CI:1.505～57.124,P=0.016).Among the 10 patients(7 strong positive and 3 moderate positive DSA)received desensitization therapy,4 patients received combination desensitization,with a 100％rate of successful transplantation,and 6 received single desensitization,with 4(66.7％)experiencing GF,so the GF rate was obviously lower in the combination than the single desensitization(P=0.008).Conclusion In haplo-HSCT patients,DSA is an important factor leading to implantation delay and GF.While,single desensitization treatment has limited efficacy.In combined DSA desensitization therapy,the decrease of antibody titer should be dynamically monitored to ensure the successful implantation of stem cells and reduce GF rate.

Objective To investigate the change in mucosal-associated invariant T (MAIT) lymphocytes in peripheral blood of children with metabolic associated fatty liver disease (MAFLD) and its clinical significance. Methods A total of 18 children with MAFLD who attended Hunan Children's Hospital from March to May, 2022, were enrolled as MAFLD group, and 20 normal children who attended the hospital during the same period of time were enrolled as control group. Peripheral blood samples were collected, and flow cytometry was used to measure the percentages of MAIT lymphocytes (CD3 + CDl61 + TCRVα7.2 + cells), different MAIT lymphocyte subsets (CD4 + CD8 - MAIT cells, CD4 - CD8 - MAIT lymphocytes, CD4 - CD8 + MAIT lymphocytes, and CD4 + CD8 + MAIT lymphocytes), and MAIT lymphocytes expressing PD-1, CD69, perforin, CD107α, CXCR3, CXCR6, and CCR6. The correlation of MAIT lymphocyte frequency with liver inflammation, fat content, and fibrosis degree was analyzed. The t -test was used for comparison of normally distributed continuous data between two groups, and the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups. The Spearman correlation analysis was used for correlation analysis. Results Compared with the control group, the MAFLD group had significant increases in the percentage of MAIT lymphocytes in peripheral blood, the percentages of MAIT cells expressing PD-1, CD69, CD107α, CXCR3, CXCR6 and CCR6, and the percentages of CD4 + CD8 - MAIT cells and CD4 + CD8 + MAIT lymphocytes among MAIT cell subsets (all P < 0.05), as well as a significant reduction in the percentage of CD4 - CD8 + MAIT cells among MAIT cell subsets ( P < 0.001). The percentages of CD4 + CD8 + MAIT lymphocytes and CD107α-positive MAIT lymphocytes were negatively correlated with alanine aminotransferase ( r =-0.474 and -0.550, P =0.047 and 0.018). Conclusion The migration of peripheral blood MAIT lymphocytes to the liver plays a protective role against liver inflammation in children with MAFLD.

Objective:To analyze the clinical features of 6 patients with spontaneous intracranial hypotension caused by high-flow spinal cerebrospinal fluid leaks.Methods:The clinical characteristics, auxiliary examinations, treatment, and outcomes in 6 patients of spontaneous intracranial hypotension caused by high-flow spinal cerebrospinal fluid leaks enrolled in the Xuanwu Hospital, Capital Medical University from February 2021 to April 2022 were retrospectively reviewed.Results:All the 6 patients had orthostatic headaches. Brain magnetic resonance imaging showed dural enhancement and brain sagging and magnetic resonance myelography showed longitudinal extradural collection in all the patients. The high-flow spinal cerebrospinal fluid leaks were demonstrated in upper thoracic segments by the dynamic myelography. The headache disappeared after conservative treatment in 2 patients and treatment with targeted epidural blood patch in 4 patients.Conclusions:The diagnosis of spontaneous intracranial hypotension caused by high-flow spinal cerebrospinal fluid leaks with typical orthostatic headache and brain magnetic resonance imaging and myelography findings is not difficult. However, the localization of the site of high-flow spinal cerebrospinal fluid leaks in spontaneous intracranial hypotension depends on the dynamic myelography. Targeted epidural blood patch is effective, but conservative treatment does not always work.

Objective:To explore the clinical and laboratory characteristics of acquired immunodeficiency syndrome (AIDS) patients complicated with disseminated nontuberculous mycobacterial (NTM) disease, in order to provide basis for clinical therapy.Methods:The clinical findings, imaging and etiological data of the 71 AIDS patients complicated with disseminated NTM disease admitted to Beijing You′an Hospital from June 2016 to June 2021 were retrospectively analyzed.Results:Among 71 patients with disseminated NTM disease, the most common initial symptom was fever, followed with cough, expectoration, fatigue, poor appetite, abdominal pain and diarrhea. Seventy point four percent (50/71) of these patients had at least two comorbidities, with oral candida infection, cytomegalovirus infection, syphilis, pneumocystis pneumonia and bacterial pneumonia being the most common. Hemoglobin ((87.8±24.2) g/L) and albumin ((27.3±7.0) g/L) levels significantly decreased, while erythrocyte sedimentation rate ((59.8±28.6) mm/1 h) and C-reactive protein ((74.7±50.8) mg/L) levels increased in most cases. The median CD4 + T lymphocyte count was 7×10 6/L. The median time of positive blood culture of NTM was 260 h. Among the 71 patients, 40 cases (56.3%) were infected with Mycobacterium avium, 15 cases (21.1%) with Mycobacterium intracellulare, 10 cases (14.1%) with Mycobacterium colombiense, three cases (4.2%) with Mycobacterium marseillense and three cases (4.2%) with Mycobacterium kansasii.The frequent imaging findings were patchy and nodular shadows in lungs, and most patients had mediastinal or hilar lymph node enlargement and splenomegaly. Conclusions:AIDS complicated with disseminated NTM disease is prevalently occurred in patients with severe immune deficiency, and most of the bacteria belong to the Mycobacterium avium- intracellulare complex. Early obtaining positive etiological results of NTM is essential to guide the correct clinical diagnosis and accurate treatment.

Objective:To explore the correlation between dietary fiber intake and intestinal barrier function in patients with irritable bowel syndrome with diarrhea (IBS-D).Methods:IBS-D patients were recruited from May 2019 to October 2019 at the clinic of gastroenterology department in China-Japan Friendship Hospital and healthy controls (HCs) were recruited by advertisement. Clinical manifestations, psychological status and quality of life were assessed using standardized questionnaires. A food frequency questionnaire was used to assess dietary habits in the preceding year. Serum diamine oxidase (DAO) was measured via ELISA.Results:64 IBS-D patients and 35 HCs were enrolled, with no significant difference in sex ratio, age and BMI between the two groups. Second to health concern, food avoidance was the dominant impacting factor for quality of life in IBS-D patients. The intake frequency of dietary fiber was decreased in IBS-D patients, and the intake frequencies of dietary fiber-rich foods were significantly lower in IBS-D patients ( P < 0.01 for tubers, P = 0.002 for vegetables, P = 0.019 for fruits and P = 0.045 for legumes). On the other hand, the intake frequencies of processed meat ( P < 0.01), greasy food ( P = 0.009), barbecued food ( P = 0.002) and animal offal ( P = 0.003) were significantly higher in IBS-D patients compared with HCs, indicating the increased intake frequency of fat. Multivariate logistic regression showed that tubers might reduce the risk of IBS-D ( OR = 0.409,95% CI: 0.232 to 0.722, P = 0.002). The frequency of abdominal pain was positively associated with the intake frequency of greasy food in IBS-D patients. Serum DAO was measured in 37 IBS-D patients and 27 HCs. IBS-D patients had significantly higher serum DAO than HCs ( 77.0 [55.3, 100.6] μg/L vs 42.5 [28.0, 58.2] μg/L, P < 0.01). Among all the participants with serum DAO test results, the level of DAO was negatively associated with the intake frequencies of tubers, vegetables and fruits while positively associated with the intake frequencies of processed meat and barbecued food. Conclusions:Food avoidance was an important impacting factor for quality of life in IBS-D patients. IBS-D patients might have insufficient dietary fiber intake and excessive fat intake. Tubers could possibly reduce the risk of IBS-D. The decreased intake frequency of dietary fiber might have a role in intestinal barrier dysfunction in IBS-D patients.

Objective: To investigate the mutual prediction relationship between peer relationship and internalizing problem. Methods: One-year follow up survey was conducted with a sample of 220 preschool children from 4 kindergartens in Shandong province. The quality of peer relationship and degree of internalizing problem were evaluated with the Peer Relationship Scale and the Child Behavior Checklist Cross-lagged panel analysis was used for mutual prediction among variables. Results: The quality of preschool children’s peer relationship showed an increasing trend with grade(F=6.40, 4.81, P<0.01), while the degree of internalizing problem showed a downward trend(F=7.65, 5.46, P<0.01). The predictive effect of pre-test peer relationship and internalizing problem on post-test corresponding behaviors were all statistically significant (β=0.56, 0.49, P<0.01). The predictive effect of pre-test peer relationship on post-test internalizing problem was statistically significant(β=-0.19, P<0.05). Conclusion Both peer relationship and internalizing problem has a certain stability across time, and early peer relationship and internalizing problem could predict later corresponding behaviors. Early peer relationship can predict later internalizing problem, while early internalizing problem cannot predict later peer relationship.

Objective:To investigate the etiology, prognosis and prognostic factors of pediatric acute liver failure(PALF), in order to provide the basis for clinical treatment of PALF.Methods:The clinical data of children with PALF hospitalized at Hunan Children′s Hospital from May 2008 to May 2018 were collected, and the causes and prognosis were analyzed.According to the prognosis, the patients were divided into the death group and the survival group, whose biochemical indexes were then compared.After that, the statistical analysis of different data were carried out by using t-test, Wilcoxon test and χ2 test separately. Results:In 120 PALF cases, there were 68 males and 52 females, and there were 36 infants, 34 toddlers, 22 preschoolers and 28 school-age children.Twenty cases (16.7%) were caused by sepsis, 19 cases (15.8%) by genetic metabolic diseases, 18 cases (15.0%) by poisoning, 12 cases (10.0%) by viral infection, 6 cases (5.0%) by drugs, 1 case (0.8%) by bile polyp, and 1 case (0.8%) by tumor disease.Besides, the etiology of 43 cases (35.9%) was unknown.Among the cases with known etiologies, genetic metabolic and infectious diseases were the main cause of disease in infants, toddler patients were mostly caused by infectious diseases and drug/toxicants, and drug/toxicants and hereditary metabolic diseases were the dominant cause of disease in school-age children and preschoolers.Mortality rate of children with PALF was 50.0%.Among them, the mortality of Epstein-Barr virus-associated hemophagocytic syndrome, sepsis, Citrin deficiency and Tyrosinemia was higher than that of other diseases.Compared with the survival group, the total bilirubin (TB)[159.00(73.05, 274.00) μmol/L vs.62.75(2.65, 221.75)μmol/L], direct bilirubin(DB)[83.00(41.43, 160.00) μmol/L vs.38.74(10.98, 128.75) μmol/L], prothrombin time (PT)[39.60(24.93, 62.60) s vs.24.65(21.43, 29.83) s], international standardized ratio (INR)[3.40(2.30, 6.74) vs.2.09(1.85, 2.84)], and blood ammonia (NH 3) levels [109.50(85.25, 149.75) μmol/L vs.80.00(60.25, 102.75) μmol/L] in the death group were significantly increased, and the diffe-rences were statistically significant(all P<0.05); while the levels of albumin[(28.72±5.88) g/L vs.(33.69±4.96) g/L], alanine aminotransferase (ALT) [586.50(223.25, 1 082.00) U/L vs.1 434.00(615.00, 3 334.50) U/L]and aspartate aminotransferase (AST) [827.50(545.00, 2 024.00) U/L vs.1 663.50(821.00, 4 886.75) U/L]in the death group were significantly decreased, and the differences were statistically significant(all P<0.05). However, the blood glucose and cholesterol levels in both groups had no statistically significant difference. Conclusion:The mortality of children with PALF is high, and different age groups have different etiologies.The increase of TB, DB, PT, INR, NH3 and the ratio of hepatic encephalopathy, and the decrease of albumin, AST and ALT suggest poor prognosis.

Objective: To investigate the clinical features of children with infectious mononucleosis (IM), to compare the difference of therapeutic effects between general treatment to antiviral therapy for IM. Methods: This prospective study analyzed the clinical data and laboratory test results of 201 cases with IM in our hospital from January 1, 2016 to December 31, 2016. The follow-up period was 6-12 months. The patients were divided into two groups according to the order of admission. The clinical symptoms and laboratory test results of the two groups were observed after hospitalization. Results: Of the total of 201 patients, male to female is 1.72∶1; Age: 8 months to 13 years 6 months (average 4.8±2.8 years), The disease frequently occurred in summer and autumn, accounted for 64.18%.The major clinical manifestations was fever (97.51%), angina (79.10%), enlarged of lymph node(68.66%), eyelid swelling (67.16%), hepatomegaly (53.73%) and splenomegaly (46.77%). There was no statistical difference in duration of fever, improved angina time, lymph nodes(liver, spleen) enlargement recovery time, heterotypic lymphocytes normalization time, lymphocyte function normalization time.There was significant difference in reducing the serum/plasma or total blood EBV-DNA in the short term between antiviral group and general treatment group (P<0.01), but for the long-term reduction of EBV-DNA, there was no significant difference (P>0.05). The low affinity EBV-CA-IgG was converted to high affinity EBV-CA-IgG in 89.41% of patients after 3 months and in 98.68% of patients after 6 months. With the recovery of the disease, the positive rate of EBV-CA-IgM and EBV-EA-IgG decreased. But the positive rate of EBV-NA-IgG increased. Serum/plasma EBV-DNA was completely overcast after 3 months, but after 12 months, the patient’s total blood EBV-DNA positive rate was still 71.11%. Conclusions In general IM patients, there was no significant difference between antiviral therapy and general treatment, so antiviral therapy is not generally recommended. EBV DNA in the whole blood of patients with IM will continue to be positive more than 6-12 months, indicating that to observe changes of IM should not use whole blood samples for EBV DNA testing.

Purpose To investigate estrogen receptor ( ER ) , progesterone receptor ( PR ) expression in PTEN-deficient endometrial carcinoma and to analysis the relationship of PTEN, ER and PR protein expression with histological grade, pathological staging and muscle invasive. Methods Immunohistochemistry EnVision was used to detect the expression of PTEN, ER and PR protein in 100 ca-ses of endometrial carcinoma. Results The rate of PTEN-deficient endometrial carcinoma was 52. 0% (52/100). The expression rate of ER, PR protein on PTEN-deficient endometrial carcinoma was 15. 4% (8/52), 19. 2% (10/52), respectively. And the expression rate of ER, PR protein on PTEN over-expression endometrial carcinoma was 72. 7% (8/11), 63. 6% (7/11), respectively. The rate of PTEN, ER and PR expression types shown PTEN-ER-PR- was 42. 0% (42/100), which was higher than other types (P <0. 05). Different PTEN, ER and PR expression types were well correlated with histological grade and pathological staging (P<0. 05), but not with muscle invasive. Conclusion Combined detection of PTEN, ER and PR may have an important reference value for prog-nosis and treatment of endometrial carcinoma.