[Haploidentical donor peripheral blood stem cell transplantation using third-party cord blood compared with matched unrelated donor transplantation for patients with hematologic malignancies].

Objectives: To assess the efficacy of cord blood-assisted haploid peripheral blood stem cell transplantation (haplo-cord-PBSCT) versus unrelated donor peripheral blood stem cell transplantation (UD-PBSCT) in the treatment of malignant hematological diseases. Methods: A retrospective analysis was performed on one hundred and four patients with malignant hematological diseases who underwent haplo-cord-PBSCT and fifty-two patients who underwent UD-PBSCT at Xiangya Hospital of Central South University between January 2016 and December 2021. Results: ①The median implantation time for neutrophils in the haplo-cord-PBSCT and UD-PBSCT groups was 13 (9-22) days and 13 (10-24) days, respectively (P=0.834), whereas the median implantation time for platelets was 15 (7-103) days and 14 (8-38) days, respectively (P=0.816). The cumulative implantation rate of neutrophils at 30 days after transplantation in the haplo-cord-PBSCT group and the UD-PBSCT group was 100% (P=0.314), and the cumulative platelet implantation rate at 100 days after transplantation was 95.2% (95% CI 88.3% - 98.1% ) and 100% (P=0.927), respectively. 30 days after transplantation, both groups of patients achieved complete donor chimerism, and no umbilical cord blood stem cells were implanted. ②The cumulative incidence rates of grade Ⅱ-Ⅳ acute GVHD within 100 days after transplantation in the haplo-cord-PBSCT group and the UD-PBSCT group were 29.1% (95% CI 20.1% -38.1% ) and 28.8% (95% CI 17.2% -41.6% (P=0.965), respectively. The cumulative incidence rates of grade Ⅲ/Ⅳ acute GVHD were 7.8% (95% CI 3.6% -14.0% ) and 9.6% (95% CI 3.5% -19.5% ) (P=0.725). The cumulative incidence rates of 2-year chronic GVHD in the haplo-cord-PBSCT group and the UD-PBSCT group were 45.3% (95% CI 36.1% -56.1% ) and 35.1% (95% CI 21.6% -44.1% ), respectively (P=0.237). The cumulative incidence rates of severe chronic GVHD at 2 years after transplantation were 13.6% (95% CI 7.6% -21.3% ) and 12.9% (95% CI 5.1% -24.3% ), respectively (P=0.840). ③The 2-year CIR after transplantation in the haplo-cord-PBSCT group and UD-PBSCT group were 12.8% (95% CI 7.0% -20.5% ) and 10.0% (95% CI 3.6% -20.2% ), respectively (P=0.341), and the NRM were 14.7% (95% CI 8.4% -22.6% ) and 16.2% (95% CI 7.4% -28.0% ), respectively (P=0.681). ④The 2-year OS rates in the haplo-cord-PBSCT and UD-PBSCT groups after transplantation were 82.2% (95% CI 74.8% -90.3% ) and 75.5% (95% CI 64.2% -88.7% ), respectively (P=0.276). The 2-year DFS rates were 69.9% (95% CI 61.2% -79.8% ) and 73.8% (95% CI 62.4% -87.3% ), respectively (P=0.551). The 2-year rates of GVHD-free/recurrence-free survival (GRFS) were 55.3% (95% CI 44.8% -64.8% ) and 64.7% (95% CI 52.8% -79.3% ), respectively (P=0.284) . Conclusion: The findings of this study indicate that haplo-cord-PBSCT and UD-PBSCT have comparable efficacy and safety in the treatment of malignant hematological diseases and can be used as an alternative treatment options.

[Effect of different observations on evaluation of cosmetic shoulder balance in adolescent idiopathic scoliosis patients with thoracic curve].

Objective: To investigate the correlations between cosmetic and radiographic parameters of shoulder balance, as well as the variations in cosmetic shoulder balance observed from different perspectives, among patients with adolescent idiopathic scoliosis (AIS) characterized by thoracic curves. Methods: A total of 43 patients with thoracic curves treated from July to October in 2022 in Nanjing Drum Tower Hospital were recruited in this study. There were 9 males and 34 females with a mean age of (14.3±1.5) years. All participants underwent comprehensive radiographic assessments and were photographed both from posterior and anterior views, focusing on the shoulder region as well as a higher level (maintaining a consistent vertical distance of 180 cm from the ground). Six cosmetic parameters were measured on the photographs: shoulder angle(α1), axilla angle(α2), shoulder area index 1(SAI1), shoulder area index 2 (SAI2), inner shoulder height (SHi) and outer shoulder height (SHo). Eight radiographic parameters were measured on the radiographs: radiographic shoulder height difference (RSHD), first rib angle (FRA), clavicle-rib cage intersection (CRCI), coracoid process height (CPH), T1 tilt, clavicle angle(CA), clavicle chest cage angle difference (CCAD) and Cobb angle. Differences among bilateral cosmetic indicators from different perspectives were analyzed and compared, and their correlation with bilateral radiographic indicators was studied. Results: There was no significant differences between anterior cosmetic parameters and posterior cosmetic parameters at the same level of observation(all P>0.05). However, when observing SHi, SHo, α1, and α2 at the shoulder level, it became evident that they exhibited significantly higher values compared to the corresponding higher level on the same side of the patients' bodies. This contrast was observed in both the dorsal [SHo: (0.11±1.20) cm vs (-0.44±1.39) cm, P=0.005; SHi: (0.64±0.86) cm vs (0.32±0.56) cm, P=0.003; α1:-0.47°±2.27° vs -0.77°±2.49°, P=0.014; α2:-3.06°±3.23° vs -2.21°±3.03°, P=0.034] and ventral [SHo: (0.12±1.29) cm vs (-0.48±1.35) cm, P=0.007; SHi: (0.61±0.88) cm vs (0.30±0.59) cm, P=0.006; α1:-0.46°±2.18° vs -0.69°±2.35°, P=0.018; α2:-3.26°±3.12° vs -2.05°±2.97°, P=0.029] aspects of the patients. SHi and SHo were more sensitive to this difference of height. The correlation coefficients between radiographic parameters and cosmetic aspects at the shoulder level varied from 0.374 to 0.767. Similarly, the correlation coefficients between radiographic parameters and cosmetic factors at the higher level ranged from 0.273 to 0.579 (all P<0.05). Conclusions: The cosmetic parameters had significant difference between different perspective of observation, the cosmetic parameters are needed to be observed at the shoulder level in the evaluation of patients' shoulder balance.

[Analysis on the iron status and associated factors during the first trimester of pregnancy].

OBJECTIVE: To investigate the impact of dietary and underlying factors on the iron status of women in early pregnancy and to provide evidence for preventing iron deficiency and iron deficiency anemia, thereby reducing the incidence of associated adverse outcomes. METHODS: From November to December 2018, women in the first trimester of pregnancy (< 12 weeks gestation) who established prenatal records at the Shunyi District Maternal and Child Health Hospital, Beijing, were enrolled in this study, in which 388 participants were accessed for data including demographic characteristics, anthropometric measurements, parity, biomarkers reflecting iron status, and food-frequency questionnaire. SPSS 26.0 were used for statistical analysis. Dietary patterns were extracted using principal component analysis, and factor scores of each dietary pattern were calculated. Two-sided Fisher exact probability test and one-way ANOVA were conducted to access differences in iron status among the groups, and the differences were significant if P < 0.05. Iron deficiency was defined as serum ferritin(SF) < 30 µg/L. To analyze the potential role of dietary factors on iron deficiency during the first trimester, the collected data listed above were adopted as independent factors for the cross-sectional Logistic regression. We used Logistic regression to analyze the potential effects of baseline characteristics and dietary factors on iron status. RESULTS: Among the 388 participants included in the analysis, 121 (32.2%) were iron deficient, in which 107 (27.6%) were iron depletion (ID), 8 (2.1%) were iron deficiency erythropoiesis (IDA), 6(1.5%) were iron deficiency anemia. The mean SF concentration was (50.4±35.3) µg/L. Multiparity(OR=3.9, 95%CI: 1.81-8.42, P=0.001)was a risk factor for iron deficiency during early pregnancy. No significant iron status differences were found among the participants with different educational levels and anthropometric measurements. In contrast, age (OR =0.96, 95%CI: 0.94-0.97, P < 0.001) was a protective factor. For multiparas, taking iron-containing supplements might have a protective effect for iron deficiency (OR=0.27, 95%CI: 0.09-0.83, P=0.022). The balance-diet pattern (OR=0.81, 95%CI: 0.66-1.00, P=0.054) only showed a marginally significant effect. CONCLUSION: Increasing attention should be paid to the iron status of pregnant multiparas and young pregnant women. For those women of reproductive age with the risk factors listed above, especially for multiparas, iron-containing supplements should be recommended to prevent gestational iron deficiency. The effect of the "balance" dietary pattern on iron status in the first trimester and following requires further research and discussion.

[Clinical features and prognosis of 118 children with histiocytic necrotizing lymphadenitis].

Objective: To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis (HNL). Methods: The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed. The clinical symptoms, laboratory examination, imaging examination, pathological findings, treatment and follow-up were analyzed. Results: Among the 118 patients, 69 were males and 49 were females. The age of onset was 10.0 (8.0, 12.0) years, ranging from 1.5 to 16.0 years. All the children had fever lymph node enlargement, blood system involvement in 74 cases (62.7%), skin injury in 39 cases (33.1%). The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases (76.3%), decreased hemoglobin in 58 cases (49.2%), decreased white blood cells in 54 cases (45.8%) and positive antinuclear antibody in 35 cases (29.7%). Ninety-seven cases (82.2%) underwent B-mode ultrasound of lymph nodes, showing nodular lesions with low echo in the neck; 22 cases (18.6%) underwent cervical X-ray and (or) CT; 7 cases (5.9%) underwent cervical magnetic resonance imaging. Lymph node biopsy was performed in all 118 cases, and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection, suggesting HNL. Fifty-seven cases (48.3%) recovered without treatment, 61 cases (51.7%) received oral steroid therapy, and 4 cases (3.4%) received indomethacin as anal stopper. The 118 cases were followed up for 4 (2, 6) years, ranging from 1 to 7 years, 87 cases (73.7%) had one onset and did not develop into other rheumatological diseases, and 24 cases (20.3%) had different degrees of recurrence, 7 cases (5.9%) had multiple system injuries, and all of the tested autoantibodies were positive for medium and high titers. All of them developed into other rheumatic immune diseases, among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren's syndrome; 7 cases were given oral steroid therapy, including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy. Conclusions: The first-onset HNL portion is self-healing, hormone-sensitive and has a good prognosis. For HNL with repeated disease and multiple system injury, antinuclear antibody titer should be monitored during follow-up, and attention should be paid to the possibility of developing into other rheumatological diseases, with poor prognosis.

[Efficacy and safety of belimumab treatment in childhood-onset systemic lupus erythematosus].

Objective: To evaluate the efficacy and safety of belimumab in children with systemic lupus erythematosus (cSLE). Methods: The clinical data of 49 cSLE patients admitted into the Department of Rheumatology and Immunology, Children's Hospital affiliated to the Capital Institute of Pediatrics, from August 2018 to December 2021 were collected. They were divided into the belimumab treatment group (18 cases) and the traditional treatment group (31 cases) according to whether they received belimumab treatment. The cSLE patients in the traditional group had similar baseline SLEDAI scores with the belimumab group and were treated with traditional immunosuppressive drugs. The clinical symptoms and improvements before and after treatment, as well as the differences in adverse events during follow-up were compared between the two groups. Results: In belimumab group, the age was (11.9±1.8) years old, the median (Q1, Q3) of disease duration was 7.5 (2.0, 16.8) months, 3 patients were male, and 15 patients completed 24 weeks of treatment. In the traditional treatment group, 31 patients with cSLE were enrolled, with an average age of (11.3±2.4) years, a median (Q1, Q3) of disease duration of 7.0 (2.5, 10.5) months, among whom 6 patients were male, and 25 patients completed 24 weeks of treatment. At baseline, the belimumab treatment group had a significantly lower oral prednisone dose than the conventional treatment group [(29.58±12.43) mg/d vs (38.20±14.11) mg/d, P=0.037]. After 24 weeks of treatment, the dosage of prednisone in both groups was reduced, and the dosage of prednisone in the belimumab group was (14.12±5.86) mg/d, which was lower than that in the traditional treatment group [(23.51±9.79) mg/d] (P=0.002). After 24 weeks of treatment, the levels of complement C3 and C4 increased, the dsDNA levels and SLEDAI score decreased in both groups (all P>0.05). The incidence of adverse events in belimumab group (3/15, 3 cases) was lower than that in traditional treatment group (32.0%, 8/25) (P>0.05). Conclusions: Belimumab in the treatment of cSLE can reduce the initial dose of prednisone and facilitate the reduction of prednisone dose, significantly improve the clinical symptoms and organ involvement, and reduce the disease activity. The incidence of adverse events was low during belimumab treatment.

The association between sociodemographic status and COPD and asthma mortality, DALY and YLD in southern China, 2005-2015.

BACKGROUND: Little is known about the disease burden of chronic obstructive pulmonary disease (COPD) and asthma in southern China. METHOD: We calculated the mortality, disability-adjusted life-years (DALY), years lived with disability (YLD) and years of life lost (YLL) for COPD and asthma in Guangdong province between 2005 and 2015. We examined the significance of trends of mortality, DALY, YLD and YLL rates for COPD and asthma with the Cochran-Armitage trend test. We also analyzed their association with sociodemographic factors by negative binomial models. RESULT: The age-standardized mortality, DALY, YLD and YLL rates of COPD and asthma decreased significantly in Guangdong, except for an increase of 11.3% in the age-standardized YLD rate of COPD between 2005 and 2015 (all P < 0.05). Compared with females, the respective adjusted mortality rate ratio of males was 2.11 for COPD, and 0.74 for asthma. Compared with other regions, the richest region, Pearl River Delta, had the lowest mortality, DALY, YLD and YLL rate ratios (RR) of COPD and asthma (all P < 0.05). COPD and asthma mortality, DALY, YLD and YLL rates increased substantially with age. Specifically, when compared with the 25-49 years age group, the respective adjusted DALY RR of asthma was 1.91, 2.02 and 22.21 for 0-24, 50-74 and ≥75 years age group; the respective adjusted YLD RR was 2.27, 1.33 and 7.17 for 0-24, 50-74 and ≥75 years age group. CONCLUSIONS: Disease burden of COPD and asthma decreased in Guangdong province in southern China between 2005 and 2015; however, a disproportionate burden of COPD and asthma in terms of age, sex and regions was observed. The relatively high disease burden and high rate of impaired public health from the less developed regions highlight the need for focused policy making to address the problem.

[Exploration on teaching reform of cancer epidemiology course].

This study aims to explore optimized teaching mode of cancer epidemiology for undergraduates, and provide scientific ideas and basis for improving teaching quality. Non-randomized concurrent control study was used. Undergraduates, enrolled in 2018, from the department of preventive medicine in A and B medical universities were selected as research objects. Traditional teaching mode was used for cancer epidemiology course in A medical university, and innovative teaching mode named "one core, four dimensions" was adopted in B medical university. After the course, questionnaire method was used to investigate self-cognition of students, teaching satisfaction and class preparation time of teachers in B Medical University. The post-class test method was used to compare the students' grades of cancer epidemiology in the two universities. The results indicated that among the 58 students of B medical university, 94.83% (55/58) students were familiar with common types of epidemiological studies and 86.21% (50/58) mastered the evaluation indicators of screening research. Among the nine teaching faculties from B medical university, seven reported that the new teaching plan helped students to learn frontier knowledge of cancer epidemiology, and eight reported the new teaching model was conducive to the interaction between teachers and students. The text score of students in B medical university was 50.34±4.90, significantly higher than that in A medical university (46.21±4.91, t=5.20, P<0.001). The optimized teaching mode of cancer epidemiology is highly praised by students and teachers, which has the potential to improve students' grasp of cancer epidemiology, the ability to combine theory with practice, and the teaching effect of cancer epidemiology.

[Relationships between lead-induced learning and memory impairments and gut microbiota disturbance in mice].

Objective: To explore the effect of lead exposure on the neurobehavior and gut microbiota community structure in mice. Methods: In August 2019, 64 C57BL/6 mice were randomly divided into 4 groups: control group (0 ppm) , low lead exposure group (20 mg/l) , medium lead exposure group (100 mg/l) and high lead exposure group (500 mg/l) . During the experiment, they were free to eat and drink. The drinking water of the lead exposure group was mixed with lead acetate, and sodium acetate was added in the control group. After 10 weeks of exposure, the Morris water maze was used to test the learning and memory ability of each group of mice, and then they were sacrificed for sampling. ICP-MS was used to detect lead content in whole blood and brain tissue. ELISA was used to determine the level of IL-1ß in mouse serum. 16S rRNA sequencing was used to detect the structural diversity of the intestinal flora in feces, and then the correlation between the flora and behavior indicators was analyzed. Results: In the Morris water maze experiment, compared with the control group, there was no significant difference in the body weight and swimming speed of the mice in the lead exposure groups. The escape latency of the mice in the 100 mg/l and 500 mg/l dose groups was prolonged, and the number of platform crossings decreased (P<0.05) ; meanwhile, the staying time of the mice in the 500 mg/l Pb-treated group in the target quadrant was lower than that of the control group, and the difference was statistically significant (P<0.05) . Compared with the control group, the blood lead content of the mice in each lead exposure group was significantly increased, and the brain lead content of mice in the 500 mg/l dose group was significantly elevated (P<0.05) . The serum IL-1ß levels of mice in each lead exposure group were higher than those of the control group (P<0.05) . At the phylum level, the relative abundance of the Proteobacteria phylum in all of Pb-treated groups was significantly increased (P<0.05) ; at the genus level, Allobaculum, Desulfovibrio, Lachnospiraceae_NK4A136_group, Turicibacter and Ureaplasma were significantly increased (P<0.05) . Among them. The relative abundance of Desuffaoibrio, Turici bacter, and Ureaplasma was negatively correlated with the residence time of mice in the quadrant of the platform (r=-0.32, -0.29, -0.44, P<0.05) . Conclusion: Lead exposure induced learning and memory impairments in mice, which may be related to the disturbance of the gut microbiota.

Bactericidal and antibiofilm properties of Rumex japonicus Houtt. on multidrug-resistant Staphylococcus aureus isolated from milk.

Multidrug-resistant (MDR) Staphylococcus aureus and its biofilm formation have been challenging to control in milk and dairy industries. Biofilms formed by Staph. aureus may result in the failure of antibacterial agents and disinfectants to penetrate the biofilm in an attempt to control contamination. Novel natural antibacterial agents are required to combat MDR bacteria and biofilms. In this study, we evaluated the bactericidal, antibiofilm, and antimotility effects of Rumex japonicus Houtt. (RJH) extract on MDR Staph. aureus isolated from milk. The RJH extract exhibited good antibacterial activity against MDR strains with minimum inhibitory concentrations (MIC) ranging from 0.78 to 6.25 mg/mL and minimum bactericidal concentrations ranging from 3.125 to 12.5 mg/mL. The extract showed strong inhibition of biofilm formation (81.9%) at sub-MIC value and eradication of biofilm at higher concentrations. The motility of Staph. aureus was effectively blocked by the extract. Major compounds emodin, chrysophanol, and physcion were identified in RJH extract using HPLC-linear trap quadrupole (LTQ)/Orbitrap-mass spectrometry. The extract was nontoxic to human epithelial cell lines such as Caco-2 and HT-29 cell lines at concentrations ranging from 0.1 to 0.5 mg/mL, and from 0.1 to 0.75 mg/mL, respectively. These findings suggest that RJH extract could be an alternative to synthetic preservatives in milk and dairy products.

[A study on the identification of threshold for early warning on adverse weather events based on the association of apparent temperature and years of life lost].

Objective: To identify the threshold of a health warning system based on the association of apparent temperature and years of life lost (YLL). Methods: Daily mortality records and meteorological data were collected from 364 Chinese counties for 2006-2017. Distributed lag nonlinear model and multivariate Meta-analyses were applied to estimate the association between the apparent temperature and YLL rate. A regression tree model was employed to estimate the warning thresholds of the apparent temperature. Stratified analyses were further conducted by age and cause of death. Results: The daily YLL rate was 23.6/105. The mean daily apparent temperature was 15.7 ℃. U-shaped nonlinear associations were observed between apparent temperature and YLL rate. The actual temperature-caused YLL rate for the elderly was higher than the young population. The daily excess deaths rate increased with the higher effect levels. Conclusions: Regression tree model was employed to define the warning threshold for meteorological health risk. The present study provides theoretical support for the weather-related health warning system.

[CacyBP promotes the proliferation and invasion of non-small cell lung cancer].

Objective: To investigate the effects and the mechanism of Calcyclin-binding protein (CacyBP) on the proliferation and invasion of non-small cell lung cancer (NSCLC) cells. Methods: Six lung cancer tissues and paired normal lung tissues were collected from NSCLC patients who underwent surgical treatment in Jinan Central Hospital during 2016. The expression of CacyBP in these tissues was examined by western blot. The protein and mRNA expression of CacyBP in human bronchial epithelial cells (16HBE), NSCLC cell lines including A549, H1299, H460 and H1975 were examined by western blot and reverse transcription-polymerase chain reaction (RT-PCR), respectively. RNAi and shRNA against negative control (NC) or CacyBP were transfected into A549 cell which were denoted as siNC group, siCacyBP-1 group, sicacyBP-2 group, shNC group and shCacyBP group, respectively. Control and Flag-CacyBP plasmids were transfected into A549 cells which were denoted as NC group and Flag-CacyBP group, respectively. Cell counting kit-8 (CCK-8), plate clone formation assay and flow cytometry assay were used to assess cell proliferation ability and cycle of A549. Wound healing assay and transwell assay were used to assess abilities of A549 cells migration and invasion. The protein expressions of epithelial-mesenchymal transition (EMT) markers including E-cadherin, N-cadherin, Snail1, Vimentin, and phosphorylation of protein kinase B (p-Akt) were examined in CacyBP depleted or overexpressed A549 cells. Results: The CacyBP protein level in NSCLC tissues was 0.41±0.23, significantly higher than 0.11±0.04 in normal lung tissues (P<0.05). The CacyBP protein expression levels in different NSCLC cell lines including A549, H1299, H460 and H1975 were 0.35±0.01, 0.38±0.01, 0.32±0.01 and 0.41±0.01, respectively, which were significantly higher than 0.03±0.01 in 16HBE cells (P<0.05). The result of RT-PCR was consistent with that of western blot. Compared with siNC group (absorbance was 1.54±0.03), siCacyBP-1 group and siCacyBP-2 group showed decreased cell proliferation (absorbances were 1.38±0.04 and 1.34±0.03, P<0.05). The number of cell colony in shNC group was 41.33±3.21, significantly higher than 22.00±3.61 in shCacyBP group (P<0.05). The proportion of G(1) phase in shCacyBP group was (61.35±5.45)%, higher than (49.61±1.54) % in shNC group (P<0.05). The proportion of S phase was (25.41±3.21)%, which was lower than (38.68±0.46)% of shNC group (P<0.05). The cell migration rate of shCacyBP group was (12.67±0.71)%, which was significantly lower than (35.50±2.07)% of shNC group (P<0.05). The numbers of cell migration and invasion in shNC group were 406.33±7.37 and 92.33±8.50, respectively, which were significantly higher than 224.67±10.01 and 66.00±7.94 in shCacyBP group (P<0.05). Compared with siNC group, the expression of epithelial marker E-cadherin was up-regulated, while the expressions of mesenchymal markers including N-cadherin, Vimentin, Snail1 and p-Akt were down-regulated in CacyBP depleted A549 cells. Compared with NC group, overexpression of CacyBP inhibited E-cadherin expression while promoted the expressions of N-cadherin, Snail1, Vimentin and p-Akt, which could be restored by LY294002. Conclusion: CacyBP may promote the proliferation and invasion of NSCLC cells by regulating Akt signal pathway.

[Analysis of 13 cases with pediatric rheumatic disease combined with endocrine disorder].

Objective: To summarize the clinical characteristics of children with rheumatic disease combined with endocrine disorder. Methods: A retrospective analysis was performed on the clinical data, including sex, age, clinical presentation, laboratory tests, treatment and outcome, of 13 patients with rheumatic diseases combined with endocrine disorder, who were admitted to our department in Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2020. Results: Among the 13 cases, 3 were males and 10 were females, without family history. Their age was (10±4) years. And the average course of disease was 4.1 months. Eight of them were diagnosed with systemic lupus erythematosus (JSLE), 2 with juvenile idiopathic arthritis (JIA), 1 with childhood vasculitis, 1 with juvenile-onset systemic sclerosis (JSSc) and 1 had juvenile dermatomyositis (JDM). Regarding the initial presentation, 10 cases had symptoms of rheumatic disease, 2 had polydipsia and polyuria, and 1 had goiter. All the 13 patients had multiple system involvement. Regarding endocrine disorder, 10 had thyroiditis or subclinical thyroiditis, 4 had diabetes mellitus and one had both thyroid and pancreas involvement. Thyroid stimulating hormone in 10 patient with thyroid involvment was 19.6 (5.2-34.0) mU/L, and their total thyroxine was 75.3 (45.2-105.4) nmol/L. Besides, thyroid peroxidase antibody or thyroglobulin antibody was positive in 7 cases. The blood glucose of 4 children with pancreatic injury was 25.0 (17.0-33.0) mmol/L, and C-peptide was 0.4 (0.3-0.5) mg/L. Glutamate dehydrogenase antibody, protein tyrosine phosphatase antibody and zinc transporter 8 antibody were positive in two cases. After treatement with immunosuppressant or immunoglobulin combined with glucocorticoid or nonsteroidal antiinflammatory drugs for rheumatic symptoms, and levothyroxine or insulin for endocrine diseases, they were all followed up for more than 6 months and maintained clinical stability. Conclusions: Rheumatic diseases in children can be complicated with endocrine disorders, and the involved organs are usually thyroid and pancreas. In children with rheumatic disease, thyroid injury usually has subtle onset, whereas pancreas injury develops rapidly, even life-threatening. Insulin should be used persistently under the instruction of endocrinologist.

[Analysis of low-dose computed tomography compliance and related factors among high-risk population of lung cancer in three provinces participating in the cancer screening program in urban China].

Objective: To analyze the compliance and related factors of low-dose computed tomography (LDCT) screening among the high-risk population of lung cancer in three provinces participating in the cancer early diagnosis and early treatment program in urban areas of China. Methods: From October 2017 to October 2018, 17 983 people aged between 40 and 74 years old at high risk of lung cancer were recruited from Zhejiang, Anhui and Liaoning provinces. The basic demographic characteristics, living habits, history of the disease and family history of cancer were collected by using a cancer risk assessment questionnaire, and the data of participants examined by LDCT were obtained from the hospitals participating in the program. The screening compliance was quantified by the screening participation rate, and it was calculated as the proportion of participants completing LDCT scan among high-risk population. The related factors of LDCT screening compliance were analyzed by using a multivariate logistic regression model. Results: The age of 17 983 participants was (56.52±8.22) years old. Males accounted for 51.9% (N=9 332), and 69.5% (N=12 495) had ever smoked, including former smokers and current smokers. A total of 6 269 participants were screened by LDCT, and the screening participation rate was 34.86%. The results of multivariate logistic regression analysis showed that the age group of 50 to 69 years old, female, passive smokers, alcohol consumption, family history of lung cancer and history of chronic respiratory diseases were more likely to be screened by LDCT, while the compliance of LDCT screening in current smokers was low. Conclusions: The LDCT screening compliance of the high-risk population of lung cancer in urban areas of China still needs to be improved. Age, sex, smoking, drinking, family history of lung cancer and history of chronic respiratory disease are associated with screening compliance.

[The prognostic impact of diabetic mellitus and hyperglycemia during DLBCL treatment on patients with diffuse large B-cell lymphoma].

Objective: This study aims to investigate the clinical features and prognostic factors of patients with diffuse large B-cell lymphoma (DLBCL) and assess the prognostic value of diabetes mellitus (DM) and hyperglycemia during DLBCL treatment in DLBCL. Methods: The clinical data of 481 newly diagnosed DLBCL patients from January 1, 2009 to December 31, 2019 at Tianjin Medical University Cancer Institute and Hospital and Sun Yat-sen University Cancer Center were retrospectively collected, focusing on their blood glucose levels before and during treatment. Cox regression method was used for univariate analysis to assess prognostic factors, and the Kaplan-Meier method was used to draw survival curves to assess the prognostic value of DM and hyperglycemia during DLBCL treatment in patients with DLBCL. Results: Eighty-two (17.0%) patients had DM before DLBCL diagnosis and treatment, and 88 (18.3%) patients had at least one blood glucose increase during DLBCL treatment. Cox univariate analysis showed that age, Ann Arbor stage, international prognostic index, and DM were associated with overall survival (OS) and progression-free survival (PFS) (all P<0.05) . The pairwise comparison between the two groups showed that the OS (P=0.001) and PFS (P<0.001) of patients with pre-existing DM were significantly worse than those of patients without abnormal blood glucose. Moreover, the OS (P=0.003) and PFS (P<0.001) of patients with hyperglycemia during DLBCL treatment were significantly worse than those of patients without abnormal blood glucose. No significant difference exists between patients with DM and patients with hyperglycemia during DLBCL treatment (OS, P=0.557; PFS, P=0.463) . Additionally, patients with adequate glycemic control during chemotherapy had a better prognosis compared with patients with poor glycemic control (OS, P=0.037; PFS, P=0.007) . Conclusion: DM is an important factor affecting the prognosis of patients with DLBCL. Moreover, hyperglycemia during treatment is related to the poor prognosis of patients with DLBCL.

[Analysis of the feasibility and prognostic value of circulating tumor DNA in detecting gene mutations in small cell lung cancer].

Objective: To investigate the feasibility of circulating tumor DNA (ctDNA) in detecting small cell lung cancer (SCLC) gene mutations and its prognostic value in chemotherapy and/or radiotherapy for SCLC patients. Methods: A total of 77 SCLC patients who were admitted to the Department of Thoracic Medical Oncology and the Department of Thoracic Radiation Oncology of Zhejiang Cancer Hospital from July 2016 to November 2019 were included. There were 66 males and 11 females, with a median age of 60 years. Among them, 42 cases were in limited stage (LS) and 35 cases were in extensive stage (ES). Next-generation sequencing (NGS) of patients' plasma ctDNA was performed before treatment. The differences of mutated genes and signaling pathways between LS and ES patients were analyzed and compared. Blood-based tumor mutation burden (bTMB) was calculated according to detected somatic cell mutations. Patients were divided into the high bTMB and the low bTMB groups according to the optimal threshold calculated by R software. Log-rank tests were used to compare progression-free survival (PFS) between the high bTMB and the low bTMB groups. Results: Among the 77 patients, 76 patients had gene mutations detected in their plasma, and the positive rate of ctDNA test was 98%. Among the 76 patients, the genes with the highest mutation frequency were TP53 (89%), RB1 (70%), LRP1B (34%), CREBBP (21%), MLL3 (21%), MLL2 (16%), NOTCH1 (13%), ROS1 (13%), BRCA2 (12%), and PTPRD (12%). The most common mutated genes in LS patients were TP53 (90%), RB1 (68%), LRP1B (24%), MLL2 (22%), and BRCA2 (17%); the most common mutated genes in ES patients were TP53 (89%), RB1 (71%), LRP1B (46%), CREBBP (31%), and MLL3 (29%). The mutation rates of NOTCH1 and CREBBP genes were significantly higher in ES patients (31.4% and 22.9%) than those in LS patients (11.9% and 4.8%) (both P<0.05). Signaling pathway analysis showed that there were more NOTCH pathway gene variations in ES patients. Among LS patients, patients in the high bTMB group (≥ 6.96 mutations/Mb) had a longer PFS than that in the low bTMB group (<6.96 mutations/Mb) (P=0.033); but no such difference was noted in ES patients. Conclusion: Plasma ctDNA sequencing detected SCLC gene mutation profiles similar to those reported in previous literature, thus ctDNA could be used as a tool to study SCLC genomics; the mutation spectra of ES-SCLC and LS-SCLC were different. bTMB has potential prognostic value in LS-SCLCs treated with chemoradiotherapy.

[Infliximab in infantile Takayasu arteritis: a case report and literature review].

Objective: To analyze the clinical characteristics of infantile Takayasu arteritis and the efficacy of infliximab (IFX). Methods: Clinical manifestations, laboratory investigations and infliximab intervention of a case with infantile Takayasu arteritis, who was admitted to Department of Rheumatism and Immunology, Children's Hospital, Capital Institute of Pediatrics in January 2018, were reviewed and analyzed. The related literature published from the beginning to March 2020 were retrieved from CNKI, Wanfang, SinoMed and PubMed with the keywords of"Takayasu arteritis","Infant" in both Chinese and English. Results: This case was a 70-day-old boy admitted due to recurrent fever for 20 days. On admission, his blood pressure were 104/90, 95/59, 125/80, and 152/125 mmHg (1 mmHg=0.133 kPa) in the right arm, left arm, right leg, and left leg, respectively. The complete blood cell count showed leukocytosis (22.6×109/L), thrombocytosis (858×109/L) and mild anemia (80 g/L). He also had elevated erythrocyte sedimentation rate (119 mm/1h), serum ferritin (598 µg/L) and C-reactive protein (112 mg/L). Computed tomographic angiography (CTA) showed narrowing of the thoracic and abdominal aorta, with thickening and heterogenous enhancement of the vessel wall. Coronary artery ultrasound detected dilatation and wall thickening of the bilateral coronary arteries, and uneven dilatation of the middle segment of the right coronary artery, showing bead-like change. Vessel wall thickening was also found in the other main arteries, including both femoral arteries, axillary arteries, carotid arteries, and subclavian arteries, and both superficial femoral arteries were slightly narrowed in the distal segments. The diagnosis of TA was confirmed, and the boy was treated with infliximab monotherapy (5 mg/(kg·every time), a total of 13 times). Then his body temperature and all inflammatory markers were normalized, and the vascular pathology was resolved according to the radiography. No side effects such as allergy or infection were noted during the treatment. During the 2 years and 6 months of follow-up, the boy maintained normal growth and development. Literature review found 8 related articles, and one of them was in Chinese but had limited information. In the other 7 papers, a total of 7 infants with TA were reported. The most common symptom was fever (5 cases), and inflammatory markers usually elevated, and the most common affected artery was abdominal aorta (6 cases). Most cases were treated with glucocorticoid. Conclusions: TA is a rare disease in infants, usually presents with fever and increased inflammatory markers. At the early stage, infliximab monotherapy could effectively control the symptoms and ensure normal growth and development.

KLF13 loss-of-function variation contributes to familial congenital heart defects.

OBJECTIVE: Congenital heart defect (CHD) represents the most common form of human developmental abnormality and contributes to substantial morbidity, mortality, and socioeconomic burden worldwide. Accumulating evidence underscores the strong genetic basis of CHD. Nevertheless, CHD is of pronounced genetic heterogeneity, and the genetic determinants underlying CHD in most patients are still unclear. This study was mainly sought to identify the causative gene for CHD in a consanguineous Chinese family. PATIENTS AND METHODS: Whole-exosome sequencing and bioinformatics analyses were performed in a Chinese family with CHD (double-outlet right ventricle and ventricular septal defect), which was transmitted in an autosomal dominant pattern. A total of 312 unrelated healthy individuals were then genotyped for the identified genetic variation. The functional effect of the identified variation was characterized by utilizing a Dual-Luciferase reporter assay system. RESULTS: A novel heterozygous variation, NM_015995.3: c.370G>T; p.(Glu124*), was identified in the KLF13 gene, which encodes Kruppel-like factor 13 key to proper heart development. Genetic analysis of the pedigree unveiled that the variation co-segregated with CHD, with complete penetrance. The variation was absent from 624 control chromosomes. The biological analysis revealed that the Glu124*-mutant KLF13 protein failed to transactivate its cardiac target genes ACTC1 and ANP. Furthermore, the variation disrupted the synergistic transactivation between KLF13 and GATA4, as well as GATA6, two other genes that have been recognized to cause CHD. CONCLUSIONS: These findings firstly indicate that genetically defective KLF13 predisposes to familial CHD, implying potential implications for genetic counseling and an improved prophylactic strategy in a subset of CHD patients.

[Research and application of assessment tools for Residents' Nutrition Literacy].

Nutrition literacy is an important part of health literacy, as well as an significant factor to enhance the quality of population, improving the nutritional status of residents and preventing nutrition-related diseases. In 2010, China developed an evaluation tool for health literacy and began to monitor residents' health literacy. So far, eight national surveys on health literacy have been completed, providing an important basis for health promotion intervention strategies and related policies. However, in health literacy evaluation system, there is neither evaluation content of nutrition literacy, nor evaluation tools. In order to achieve the goals of "national nutrition plan (2017-2030)"and evaluate the implementation effect, it is urgent to carry out the assessment and monitoring of nutrition literacy. According to the nutritional characteristics of different populaitons, this research organizes national experts in related fields, following the principles of scientificity, conciseness and generality and through the scientific formulation procedures to construct the nutrition literacy assessment tools for different populations. This assessment tool can enhance the pertinence and scientificity of nutrition education and improve nutrition development strategy. The establishment of the nutrition literacy assessment tool is the premise of gradually establishing the nutrition literacy assessment system of the residents, and also lays a solid foundation for further conducting the national nutrition literacy evaluation work.

[Establishment of nutrition literacy core items for Chinese pregnant women].

Objective: To establish the nutrition literacy core items for Chinese pregnant women. Methods: The framework system and preliminary items of nutrition literacy were established through literature review and experts consultation. E-Delphi method was used to determine the nutrition literacy items.11 experts in the field of maternal and child nutrition, obstetrics and gynecology, health education and nutrition and diseases were invited to score the importance of each nutrition literacy item via Email. The judgment basis and familiarity of experts towards the items, active coefficient, authority coefficient and harmony coefficient were evaluated and analyzed to generate the final list of nutrition literacy items. Results: The active coefficients of consultation in two rounds were 81.8% (9/11) and 87.5% (8/9).The authority coefficients were (0.86±0.11) and (0.85±0.06).The average scores of importance were (4.32±0.84) and (4.58±0.57) and the harmony coefficients were 0.387 (χ²=90.472, P<0.001) and 0.290 (χ²=46.752, P=0.002) respectively. After the second round of consultation, all selected items met the inclusion criteria. We identified the final list of nutrition literacy items consisting of 3 scales (basic knowledge and concept, lifestyles and dietary behaviors, and basic skills), 10 sub-scales (basic nutrition concept, food and nutrition knowledge, nutrition and disease knowledge, lifestyles, dietary behaviors, preparation for breastfeeding, gestational weight management, gestational disease management, acquisition, understanding and application of nutrition information, judgement of nutrition information, and nutrition decision making) with 24 items in total. Conclusions: The framework system and core items of nutrition literacy are established for Chinese pregnant women based on E-Delphi method. The experts involved in the consultation process present a performance with good representativeness, enthusiasm and authority. All experts' opinions are coordinated and unified, which could satisfy the demand of developing nutrition literacy core items for target population.