Objective:To summarize the clinical features and outcomes of deflazacort-induced Steven Johnson syndrome (SJS)-toxic epidermal necrolysis (TEN) to raise awareness among patients with Duchenne muscular dystrophy (DMD), neurologists as well as other deflazacort users.Methods:The clinical data of a boy with DMD who had SJS induced by deflazacort treated at the Department of Rheumatology & Clinical Immunology of Tianjin Children′s Hospital in July 2024 was analyzed retrospectively. Taking "deflazacort" "Steven-Johnson syndrome" "toxic epidermal necrolysis" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2024. The clinical characteristics, treatment and outcomes of deflazacort-induced SJS-TEN were summarized.Results:A 12-year-old boy was admitted with a 3-day history of rash. He was diagnosed with DMD at the age of 3 and had been treated with prednisolone since the age of 8. Forty-four days before admission, the patient started deflazacort to replace prednisolone. Three days before admission, progressively worsening erythematous maculopapular rashes, blisters and skin peeling (8% body surface area), oral mucosal erosion, and exudative conjunctivitis occurred, thus deflazacort was discontinued. Complete remission of SJS was achieved after treatment with intravenous immunoglobulin (IVIG, total 1.4 g/kg), 2 doses of etanercept (0.9 mg/kg, once), subcutaneous injection and intravenous methylprednisolone (0.7 mg/(kg·d)). Based on the literature, there were 5 reports in English while none in Chinese, altogether 7 cases were reported. All the patients were male, aged 3-45 years. Duration of deflazacort exposure was 2-8 weeks. Dermatology diagnosis of our case was SJS, and 5 cases were TEN. One patient was diagnosed with exudative erythema multiforme, and subsequent deflazacort oral challenge test was positive. Treatment included methylprednisolone or dexamethasone in 5 cases, IVIG in 6 cases, etanercept in 3 cases and cyclosporine in 1 case. All patients recovered completely.Conclusion:The synthetic corticosteroid deflazacort can cause rare but severe adverse reactions such as SJS-TEN, which needs close monitoring and prompt recognition and management.

Objective:To investigate the long-term safety and effectiveness of stent implantation for residual pulmonary artery stenosis after complicated congenital heart disease.Methods:The symptoms, signs, echocardiography, cardiac CT, cardiac catheterization, six-minute walking distance, and BNP of 41 patients diagnosed from January 1996 to January 2020. In this group, 41 patients, 30 males and 11 females, aged 1.3-14.5 years old, mean (6.1±3.6) years old, and weighed 8-43 kg, mean (18.9±9.4)kg, compared the diameter of the target vessel, pressure difference across stenosis, cardiac function before and postoperative follow-up, and evaluated the long-term effect of stent implantation in the treatment of pulmonary artery stenosis.Results:All 41 patients were not lost to follow-up, no death, and there were no serious adverse events such as stent fracture, artery dissection and pulmonary embolism during follow-up. The median follow-up time was 7.1 years (3.1 to 13.8 years). As of January 2023, the echocardiographic results showed that the diameter of the target vessels in 41 patients increased from preoperative (3.9±1.5) mm to (6.0±1.5) mm ( P<0.05), the pressure difference across the stenosis decreased from preoperative (51.4±19.1) mmHg to (33.1±19.7) mmHg (1 mmHg=0.133 kPa, P<0.05); Heart spiral CT showed that the ratio of target vessel diameter to distal vessel diameter increased from preoperative 0.4±0.2 to 0.9±0.3( P<0.05). All patients had no slow growth and development, no recurrent lung infection, 39 patients (95.1%) had gradeⅠcardiac function, and 2 patients (4.9%) had gradeⅡcardiac function.As children in school age, the walking distance of 6 min was 462 to 633 m, mean( 529.9±57.1)m, the respiratory score was 0.5-1, and the lower limb force score was 6-12. There were 5 long-term adverse events, including 4 cases of target vessel restenosis (9.7%), and 1 case (2.4%), two of the patients with restenosis with repeated target vessel stenosis and lateral pulmonary hypertension were surgically intervention: stent removing and pumonary expanding, after 4, 13 years of stent implantation.And the others were still in follow-up, and no further intervention was made. The Cox multivariate survival analysis suggested that right ventricular systolic blood pressure was a risk factor for endpoint events before stent implantation ( P<0.05). Conclusion:The treatment of residual pulmonary artery stenosis after complicated congenital heart disease after percutaneous stent implantation can effectively relieve the right heart pressure overload, improve pulmonary blood flow, stabilize cardiac function, improve the long-term prognosis of patients with complicated congenital heart disease, reduce the chest opening rate of reoperation, and have stable long-term curative effect.

In pursuit of effective agents for hepatocellular carcinoma derived from the Artemisia species, this study built upon initial findings that an ethanol (EtOH) extract and ethyl acetate (EtOAc) fraction of the aerial parts of Artemisia dubia Wall. ex Bess. exhibited cytotoxicity against HepG2 cells with inhibitory rates of 57.1% and 84.2% (100 μg·mL^-1), respectively. Guided by bioactivity, fourteen previously unidentified sesquiterpenes, artemdubinoids A-N (1-14), were isolated from the EtOAc fraction. Their structural elucidation was achieved through comprehensive spectroscopic analyses and corroborated by the comparison between the experimental and calculated ECD spectra. Single crystal X-ray diffraction provided definitive structure confirmation for artemdubinoids A, D, F, and H. Artemdubinoids A and B (1-2) represented unique sesquiterpenes featuring a 6/5-fused bicyclic carbon scaffold, and their putative biosynthetic pathways were discussed; artemdubinoid C (3) was a novel guaianolide derivative that might be formed by the [4 + 2] Diels-Alder reaction; artemdubinoids D and E (4-5) were rare 1,10-seco-guaianolides; artemdubinoids F-K (6-11) were chlorine-containing guaianolides. Eleven compounds exhibited cytotoxicity against three human hepatoma cell lines (HepG2, Huh7, and SK-Hep-1) with half-maximal inhibitory concentration (IC₅₀) values spanning 7.5-82.5 μmol·L^-1. Artemdubinoid M (13) exhibited the most active cytotoxicity with IC₅₀ values of 14.5, 7.5 and 8.9 μmol·L^-1 against the HepG2, Huh7, and SK-Hep-1 cell lines, respectively, which were equivalent to the positive control, sorafenib.
Humans ; Artemisia/chemistry* ; Sesquiterpenes/chemistry* ; Cell Line ; Hep G2 Cells ; Crystallography, X-Ray ; Molecular Structure

Objective:To evaluate the predictive value of peripheral blood neutrophil to lymphocyte ratio (NLR) in mortality of patients with acute paraquat poisoning.Methods:In March 2022, all literatures about the studies on NLR assessing the mortality of patients with acute paraquat poisoning were searched in the National Library of Medicine PubMed, Embase, Cochrane Library Database, Web of Science, CNKI, Wanfang Medicine Database, Weipu Database, China Biology Medicine disc (SinoMed). The data updated by March 2022, without the limitation of languages. Two researchers extracted literature information independently and conducted literature quality evaluation using QUADAS-2. And the data extracted from the literatures were analyzed with Stata 16 software.Results:A total of 9 studies were included in this Meta-analysis, including 967 patients. And the Meta-analysis results showed that the combined sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio were 0.77 (95% CI: 0.72-0.82, P<0.05), 0.83 (95% CI: 0.74-0.90, P<0.05), 4.63 (95% CI: 2.99-7.15, P<0.05), 0.27 (95% CI: 0.22-0.34, P<0.05) and 17.06 (95% CI: 10.22-28.48, P<0.05), and the area under the curve (AUC) of the summary receiver operator characteristics curve (SROC) was 0.85 (95% CI: 0.81-0.88) . Conclusion:NLR has predictive value in 30-day mortality of patients with acute paraquat poisoning.

Objective:To evaluate the predictive value of peripheral blood neutrophil to lymphocyte ratio (NLR) in mortality of patients with acute paraquat poisoning.Methods:In March 2022, all literatures about the studies on NLR assessing the mortality of patients with acute paraquat poisoning were searched in the National Library of Medicine PubMed, Embase, Cochrane Library Database, Web of Science, CNKI, Wanfang Medicine Database, Weipu Database, China Biology Medicine disc (SinoMed). The data updated by March 2022, without the limitation of languages. Two researchers extracted literature information independently and conducted literature quality evaluation using QUADAS-2. And the data extracted from the literatures were analyzed with Stata 16 software.Results:A total of 9 studies were included in this Meta-analysis, including 967 patients. And the Meta-analysis results showed that the combined sensitivity, specificity, positive likelihood ratio, negative likelihood ratio and diagnostic odds ratio were 0.77 (95% CI: 0.72-0.82, P<0.05), 0.83 (95% CI: 0.74-0.90, P<0.05), 4.63 (95% CI: 2.99-7.15, P<0.05), 0.27 (95% CI: 0.22-0.34, P<0.05) and 17.06 (95% CI: 10.22-28.48, P<0.05), and the area under the curve (AUC) of the summary receiver operator characteristics curve (SROC) was 0.85 (95% CI: 0.81-0.88) . Conclusion:NLR has predictive value in 30-day mortality of patients with acute paraquat poisoning.

Objective : To investigate the correlation between nocturnal sleep duration combined with snoring in the first trimester of pregnancyand small for gestational age(SGA) ，large for gestational age(LGA) ． Methods : Multi- variate Logistic regression model was used to analyze the association between nocturnal sleep duration ，snoring， their combined effects and SGA，LGA. Results : Compared to nocturnal sleep duration 7 to 9 h in the first trimester of pregnancy，sleep duration＜7 h was positively correlated with SGA in male newborn( OR = 4. 22，95% CI : 1. 69 - 10. 52) ; After stratified by snoring，the sleep duration of snoring women＜7 h was positively correlated with SGA ( OR = 5. 68，95% CI : 1. 02－31. 51) ，and the sleep duration of non-snoring women＜7 h was positively correlated with LGA ( OR = 2. 10，95% CI : 1. 16 －3. 81) ． Conclusion Sleep duration＜7 h in the first trimester of preg- nancy is a risk factor for SGA and LGA，and snoring may enhance the association between sleep duration＜7 h in the first trimester of pregnancy and SGA．Pregnant women should keep adequate nocturnal sleep duration to reduce the risk of abnormal neonatal weight.

OBJECTIVE: To understand the mental health status of subway employees, and to explore the relationship among their mental health, psychological capital and work engagement. METHODS: Using the typical sampling method, a total of 2 758 subway employees from a rail transit group Co., Ltd in a city were selected as the study subjects. The Symptom Check List 90, Psychological Capital Questionnaire, and Utrecht Work Engagement Scale were used to investigate the mental health, psychological capital and work engagement of the study subjects. RESULTS: The screening positive rate of mental disorder of the study subjects was 15.6%(431/2 758), and the median scores of mental health, psychological capital, and work engagement were 108, 109, and 62 points, respectively. The mental health scores of the study subjects were negatively correlated with their psychological capital and work engagement scores [Spearman correlation coefficient(r_S) were-0.55 and-0.57, respectively, P<0.01]. The psychological capital score was positively correlated with the work engagement score(r_S=0.70, P<0.01).Mental health played a partial mediating role in the influence of psychological capital on work engagement(mediating effect was 0.07, 95% correlation coefficient: 0.06-0.08, P<0.01). CONCLUSION: The mental health status of the subway employees is good. Psychological capital of subway employees can not only directly affect work engagement, but also indirectly affect work engagement by influencing mental health.

OBJECTIVE: To detect potential mutation in a Chinese pedigree affected with congenital limb malformations. METHODS: Clinical data was collected. Genomic DNA was extracted from peripheral blood samples of family members. The zone of polarizing activity regulatory sequence (ZRS) were amplified by PCR and subjected to direct sequencing. RESULTS: Among the 13 individuals in this pedigree, there were 4 PPD patients, who were characterized by varying degrees of deformity. The female patients suffered triphalangeal thumb and preaxial polydactyly, while the male patients only had preaxial polydactyly. Only one patient had foot involvement. TA heterogeneous mutations was discovered in the ZRS (105C>G) in all patients, the same mutation was not detected in 2 healthy family members. CONCLUSION The inheritance pattern of PPD was autosomal dominant inheritance. There was a significant variability of symptoms among family patients. The heterozygous mutation of the ZRS (105C>G) probably underlie the disease.
Female ; Genetic Testing ; Hand Deformities, Congenital ; genetics ; Humans ; Limb Deformities, Congenital ; genetics ; Male ; Membrane Proteins ; genetics ; Pedigree ; Polydactyly ; genetics ; Thumb ; pathology

Objective: To detect potential mutation in a Chinese pedigree affected with congenital limb malformations. Methods: Clinical data was collected. Genomic DNA was extracted from peripheral blood samples of family members. The zone of polarizing activity regulatory sequence (ZRS) were amplified by PCR and subjected to direct sequencing. Results: Among the 13 individuals in this pedigree, there were 4 PPD patients, who were characterized by varying degrees of deformity. The female patients suffered triphalangeal thumb and preaxial polydactyly, while the male patients only had preaxial polydactyly. Only one patient had foot involvement. TA heterogeneous mutations was discovered in the ZRS (105C>G) in all patients, the same mutation was not detected in 2 healthy family members. Conclusion The inheritance pattern of PPD was autosomal dominant inheritance. There was a significant variability of symptoms among family patients. The heterozygous mutation of the ZRS (105C>G) probably underlie the disease.

Objective To study the pharmacokinetic status of Cyclophosphamide (CYC) in children with lupus nephritis (LN),as well as the relationship between the pharmacokinetic results and clinical indicators,adverse reactions,curative effect evaluation.Methods Thirty patients hospitalized at Tianjin Children's Hospital from January 2014 to December 2016 were treated with glucocorticoid (GC) combined with CYC.Blood samples were collected in 6 point-in-time after intravenous CYC,a high performance liquid chromatography (HPLC) method was used to detect the blood drug concentration,and the pharmacokinetic results were calculated.All patients were followed up for 24 weeks,and the levels of serum albumin (ALB),24 hours urinary protein,serum creatinine (Scr),complement 3 (C3),therapeutic effect and systemic lupus erythematosus disease activity index scores were evaluated at the same time.Results Pharmacokinetic curve showed the second chamber model,the area under the curve (AUC) of time-effect relationship was (143.55 ±42.43) g/(L · h),peak concentration (Cmax) was (20.02 ± 3.55) g/L,and half-time period (T1/2) was(4.21 ± 0.96) h.Age and gender had no influence on AUC,Cmax and T1/2,and statistically significant correlation was found between serum ALB and T1/2,which was positively correlated (r =0.517,P ＜ 0.05).After 16 weeks of treatment,7 patients were partially responsive,22 cases had a complete remission,and the invalid case was only one.Followed up for 24 weeks,6 patients were partially responsive,24 cases had a complete remission.After 16 weeks of treatment,there were statistical differences in AUC among patients with complete response,partial response or no remission.And after 24 weeks,the patients between complete remission and partial remission had no statistically significant difference in the AUC.After 16 weeks and 24 weeks,T1/2 and Cmax had no statistical differences among those groups of patients.Adverse reactions included leukopenia (3 cases,10.00％),gastrointestinal symptoms (4 cases,13.33％) and respiratory tract infection (6 cases,20.00％).There was no statistical relationship between pharmacokinetic parameters and the occurrence of adverse reactions.Single factor analysis showed that there was a statistical significance between ALB,urine protein quantity and treatment effect,but multi-factor analysis showed that the relation between factors and therapeutic effect had no statistical significance.Conclusions HPLC method can be used for the detection of cyclophosphamide blood drug concentration.The cyclophosphamide pharmacokinetic status of children and adult is similar.ALB and T1/2 show a linear positive correlation.Single factor analysis shows a correlation between ALB and treatment effect.Therefore,the increasing level of plasma propagated before treatment can improve therapeutic effect possibly.Cyclophosphamide pharmacokinetic status has nothing to do with the occurrence of adverse drug reactions in the study.