Objective To analyze the characteristics and related influencing factors of transfusion reactions in pediatric patients,so as to provide evidence for clinical treatment and prevention of transfusion reactions.Methods A retrospective study was conducted on children who received blood transfusions at our hospital from 2019 to 2023 in terms of the incidence,types,time of occurrence,and related influencing factors of transfusion reactions.Results A total of 69 926 transfusions were performed from 2019 to 2023,with 711 cases of transfusion reactions,resulting in an incidence of 1.02%.The inci-dence of transfusion reactions decreased annually from 2019(1.89%)to 2022(0.50%).The incidence of transfusion reac-tions to apheresis platelets,frozen plasma,suspended leukocyte-depleted red blood cells and cryoprecipitate coagulation fac-tor were 2.16%(551/25 565),0.50%(92/18 277),0.25%(65/25 679)and 0.74%(3/405),respectively,with a-pheresis platelet transfusion of a significantly higher incidence compared to other blood components(P<0.05).As of trans-fusion reaction types,allergic reactions accounted for 86.22%(613/711),febrile non-hemolytic transfusion reactions(FNHTR)accounted for 13.08%(93/711),and acute hemolytic transfusion reactions accounted for 0.70%(5/711).Multivariate logistic regression analysis showed that apheresis platelet transfusion was an independent risk factor for allergic reactions(P<0.05),while suspended leukocyte-depleted red blood cells transfusion was an independent risk factor for FNHTR(P<0.05).In terms of the occurrence time of transfusion reactions,compared to apheresis platelets and frozen plas-ma,transfusion reactions caused by suspended leukocyte-depleted red blood cells transfusion occurred later(apheresis platelets P<0.05,frozen plasma P<0.05).Conclusion The results suggest that blood components transfused are significant influencing factors for the characteristics of transfusion reactions in pediatric patients,affecting the incidence,types,and oc-currence time of transfusion reactions,which provides reference for clinical treatment and prevention of transfusion reactions.

Objective:To analyze the gene mutation type and clinical phenotype of patients with PRRT2 mutation, and explore their relations with prognosis. Methods:A total of 18 patients with PRRT2 gene mutation (1 patient with novel mutation in PRRT2 gene, and 17 probands in 17 families with PRRT2 gene mutation) were enrolled in Department of Neurology, First Affiliated Hospital of Air Force Medical University from January 2018 to July 2023. Serum of the patients was collected for whole exon sequencing, and mutation sites and types of PRRT2 gene were analyzed. SWISS-MODEL website was used to predict the changes in protein structure caused by PRRT2 gene mutation. The relations of gene mutation type and clinical phenotype with prognosis of these patients were analyzed. Results:(1) All 18 patients with PRRT2 gene mutation were heterozygous mutation, including 12 frameshift mutations, 5 missense mutations, and 1 integer mutation. The clinical phenotype included benign familial infantile epilepsy (BFIE) in 5 patients, epilepsy in 6 patients, exercise-induced paroxysmal kinesigenic dyskinesia (PKD) in 5 patients, and infantile convulsion and choreoathetosis (ICCA) in 2 patients. A total of 8 mutation sites were found in 18 patients with PRRT2 gene mutation, of which 3 mutation sites have been reported, and 5 mutation sites have not been reported, including c.647(exon2)C>A, c.647(exon2)C>G, c.170(exon2)delC, c.981(exon3)C>G, and lossl(EXON: 2)(all). (2) Eighteen patients mainly accepted oxcarbazepine, levetiracetam, and sodium valproate in combination or monotherapy. Among them, 5 BFIE patients, 2 ICCA patients and 3 epilepsy patients were seizure-free after treatment. PKD patients did not respond well to oxcarbazepine. (3) Three frameshift mutations (mutation sites: c.649 [exon2]_c.650 [exon2] insC, c.640 [exon2]_c.641 [exon2] insC, and c.170 [exon2] delC) led to premature termination of protein translation, resulting in significant changes in protein structure. Four missense mutations (mutation sites: c.640[exo2]G>C, c.647[exon2]C>A, c.647[exon2]C>G, and c.981[exon3]C>G) had little effect on protein structure changes. No relation was found between changes of protein structure caused by different mutation types and prognosis. Conclusion:PRRT2 gene mutation patients with clinical phenotypes of BFIE and ICCA have good prognosis, but the mutation type is not related with the prognosis of patients.

Objective To develop and evaluate the equivalence of the Mandarin test material for tracking of noise tolerance(TNT)test.Methods Six different speech materials were developed(themes including daily life,entertainment,family,festivals,outdoors,and school).Four-minute TNT tests were measured in 21 normal hear-ing subjects using six different test materials.For each session,the tolerable noise level(TNL)and TNT scores were acquired and calculated for 3 time windows(31～240 s,31～120 s,151～240 s).Results Statistic analysis showed significant differences in the TNL(F=43.611,P＜0.05)among the normal hearing listeners.There were statistically significant differences in standardize z-scored TNT scores of the six different materials in the three time windows(P＜0.05).Post-hoc comparisons revealed that all significant differences involved the family and daily life themes.Conclusion Entertainment,festival,outdoors and school themed test materials can serve as the materials of Mandarin tracking of noise tolerance test and can be appied in research and clinical testing.

Objective:To analyze clinical efficacy and safety of dermabrasion for the treatment of familial benign chronic pemphigus.Methods:Clinical data were retrospectively collected from 6 patients with familial benign chronic pemphigus, who underwent dermabrasion in the Department of Dermatology, the First Affiliated Hospital of Dalian Medical University from February 2019 to July 2020. There were 3 males and 3 females, they were aged from 39 to 65 years, and their disease duration ranged from 10 to 40 years. All the patients were postoperatively followed up for 14 - 34 months. Response rates were calculated, and adverse reactions and recurrence were observed.Results:Dermabrasion was performed on the 6 patients in 55 body areas. After operation, complete recovery was observed in 36 areas (65.5%), marked improvement was noted in 13 areas (23.6%), and moderate improvement was observed in 6 areas (10.9%) ; there were no areas with no response, resulting in an overall response rate of 89.1%. Postoperatively, the patients experienced no obvious discomfort, and only slight hypopigmentation and mild scars remained. During the follow-up of 14 to 34 months, no recurrence was observed in the treated areas.Conclusion:Dermabrasion was safe and effective for the treatment of familial benign chronic pemphigus.

Objective:To investigate the application value of Pelvic Inflammation No.1 formula of transdermal meridian point-targeted drug delivery therapy in the treatment of patients with chronic pelvic inflammatory disease(damp-heat stasis type).Methods:All patients with chronic pelvic inflammatory disease(damp-heat stasis type)visited Danzhou Hospital of Traditional Chinese Medi-cine from August 2017 to March 2021 and were randomly grouped,57 patients in the control group were treated with simple Western medicine,and 57 patients in the observation group were combined with Pelvic Inflammation No.1 formula transdermal meridian target-ed transdermal medication,and the therapeutic efficacies were compared after 3 months of treatment.Results:After treatment,serum matrix metalloproteinase-9(MMP-9),D dimer(D-D),IL-6 and other indexes in the observation group were lower than those in the control group,and IL-10 was higher than that in the control group,the difference was statistically significant(P<0.05).After treat-ment,the carbohydrate antigen CA125 in the observation group was lower than that in the control group,and the CD4+/CD8+and CD4+cells in the observation group were higher than those in the control group(P<0.05).After treatment,the score of summary of health status surveys(SF-36)in the observation group was higher than that in the control group,and the total score of TCM symptoms was lower than that in the control group(P<0.05).The total effective rate in the observation group was 96.49%(55/57),which was higher than 82.46%(46/57)in the control group(P<0.05).Conclusion:For patients with chronic pelvic inflammatory disease(damp-heat stagnation type),combined with Pelvic Inflammation No.1 formula transdermal via acupoints targeted drug therapy can help to regu-late inflammatory factors,regulate immune function,and improve clinical efficacy.

Objective:To investigate value of contrast-enhanced ultrasound(CEUS)in predicting ischemic-type biliary lesions(ITBLs)in patients with thickened hilar bile duct wall at early stage after liver transplantation.Methods:A total of 45 patients,who underwent liver transplantation at the Liver Transplantation Center of Beijing Friendship Hospital Affiliated to Capital Medical University from June 25,2020 to December 28,2022,and occurred hilar bile duct wall thickening at early stage after surgery,were prospectively included.CEUS was performed on biliary tract when the thickened hilar bile duct wall was first detected by routine ultrasound,and the enhanced mode of duct wall at each phase was recorded.Subsequently,according to the results of cholangiography,these patients were divided into ITBLs group(15 cases)and non-ITBLS group(30 cases).The enhanced degree of each phase of CEUS of two groups was qualitatively analyzed and compared,and the diagnostic efficacy of CEUS for ITBLs after liver transplantation was evaluated.Results:There were no significant differences in source of liver donor,biliary anastomosis,autoimmune liver disease,hepatic artery occlusion(HAO),rejection,cytomegalovirus infection and cholangitis between the two groups(P>0.05).The compared results of the enhanced mode of CEUS at arterial phase between the two groups indicated that 25 patients(83.3%)were hyper-enhancement,and 5 patients(16.7%)were iso-enhancement,and 0 patient was hypo-enhancement or non-enhancement in non-ITBLS group.The compared results also indicated that 3 patients(20.0%)were hyper-enhancement,and 4 patients(26.7%)were iso-enhancement,and 8 patients(53.3%)were hypo-enhancement or non-enhancement in ITBLs group.The difference of above results between the two groups was statistically significant(x2=22.946,P<0.000).There was no significant difference between the two groups in the enhanced mode at the late phase(P>0.05).The accuracy,sensitivity,specificity,positively predictive value and negatively predictive value of the prompted hypo-enhancement or non-enhancement at arterial phase of CEUS on biliary tract were respectively 84.4%,53.3%,100%,100%and 84.4%in diagnosing ITBLs.For 8 patients who were diagnosed as ITBLs by CEUS,the diagnostic time of CEUS for ITBLs was 1 to 6 months[3.0(1-5)months]ahead of that of cholangiography.Conclusion:CEUS can more accurately predict ITBLs before the biliary tract occurs significant morphological change,which can significantly advance the diagnostic time for ITBLs.

Objective:To evaluate the efficacy and safety of a China original liquid pulsation system for the treatment of meibomian gland dysfunction (MGD).Methods:A non-randomized controlled clinical trial was conducted.Twenty-two patients (44 eyes) diagnosed with MGD in Eye and ENT Hospital of Fudan University from February to August 2022 were enrolled.The patients were assigned into two groups according to their willingness.Of the 22 patients (44 eyes), 10 patients (20 eyes) in single liquid pulsation system group were treated with single liquid pulsation system for 12 minutes, and 12 patients (24 eyes) in intense pulsed light (IPL) group were treated with a course (4 times) of IPL, warm compresses and meibomian gland massage at three-week intervals.There was no difference in age and other baseline clinical indexes between the two groups (all at P<0.05). The meibum grading, quality grading of tear film lipid layer, Symptom Assessment Questionnaire in Dry Eye (SANDE) questionnaire score, first and average tear breakup time (BUT), corneal fluorescein sodium staining (CFS) score, tear meniscus height (TMH), and the area of meibomian gland loss were determined at baseline, 1 and 3 months after treatment.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Eye and ENT Hospital of Fudan University (No.2021069). Written informed consent was obtained from each patient before any medical examination. Results:Statistically significant group effects and time effects were found in the quality of tear film lipid layer ( Hgroup=4.39, P=0.036, Htime=6.30, P=0.043) and average BUT ( Fgroup=4.41, P=0.038; Ftime=4.08, P=0.049) in the two groups.The meibum grading, first BUT and TMH 1 and 3 months after treatment were significantly better than before treatment in single liquid pulsation system group (all at P<0.05). Compared with before treatment, there was no significant improvement in the meibum grading, distribution of tear film lipid, first BUT and TMH at 1 and 3 months after treatment in IPL group (all at P>0.05). In both groups, the SANDE and CFS scores 1 and 3 months after treatment were better than those before treatment, showing statistically significance (all at P<0.05). In terms of safety, neither instrument-related adverse events nor extra complaints of discomfort were reported in the single liquid pulsation system group.In both groups, the number of patients with positive CFS staining significantly decreased, and no new cases with positive CFS appeared after treatment. Conclusions:This China original liquid pulsation system is a safe and effective physical therapy in improving tear film dysfunction and ocular surface symptoms of MGD patients within 3 months after treatment.

【Objective】 To explore the relationship between chronic comorbidity and the physical and mental health of relatives of elderly people during the nursing home confinement, and to analyze the mediating effects of perceived stress and intolerance of uncertainty in this context. 【Methods】 A total of 568 family members of elderly people in nine elderly institutions in Shaanxi Province were selected. The survey included the short version of the Perceived Stress Scale, Intolerance of Uncertainty Scale, and The World Health Organization-5 Well-being Index. The data were analyzed with Stata for correlation and mediation effects. 【Results】 ① The comorbidities of chronic diseases was positively correlated with the perceived stress (r=0.16, P<0.001) and intolerance of uncertainty (r=0.11, P=0.006) of the family members, but negatively correlated with the physical and mental health of the family members (r=-0.13, P=0.002). ② The mediating effect of perceived stress between chronic disease co-morbidity and physical and mental health of family members in older adults was -0.023, accounting for 18.8% of the total effect; the mediating effect of intolerance of uncertainty between chronic disease co-morbidity and physical and mental health of family members in older adults was -0.041, accounting for 33.5% of the total effect. 【Conclusion】 During closed management in a nursing facility, the physical and mental health of family members of older adults with chronic co-morbidities is poorer than that of family members of non-chronic co-morbidities. And it can lead to a decline in physical and mental health of family members through increased perceived stress and intolerance of uncertainty.

Objective:The therapeutic effect of less polar ginsenosides on rats with rheumatoid arthritis was studied, and the metabolic pathway that produce anti-inflammatory effect of less polar ginsenosides was identified.Methods:Rats were randomly divided into the control group, the model group, methotrexate treatment group, and high dose, medium dose, and low dose less polar ginsenosides groups. After 30 days of oral administration, less polar ginsenosides reduced the disease activity significantly in rats with rheumatoid arthritis. Blood and ankle synovial tissue metabolisms were measured by ultra performance liquid chromatography (UPLC) tandem mass spectrometry (MS) to explore the mechanism of less polar ginsenosides.The resulting data were subjected to principal component analysis and orthogonal partial least squares discriminant analysis(OPLS-DA).Results:Compared with the model group, erythrocyte sedimentation rate and RF decreased significantly in the high dose of less polar ginsenosides ( P<0.01). Metabolomics showed that R2X and R2Y of serum OPLS-DA were 0.626 and 0.904 respectively. The R2X and R2Y of synovial OPLS-DA were 0.429 and 0.689 respectively. Major differential metabolites were identified in the model group of rats, including arachidonic acid, valine, linoleic acid, and guanine nucleoside, etc. The main differential metabolites were identified in rats in the high dose group of less polar ginsenosides, including linoleic acid, betaine, eicosapentaenoic acid, alanine, methionine sulfoxide, isoleucine, etc. Conclusion:The metabolic spectrum has shown that inflammation is associated with linoleic acid metabolism, valine, leucine and isoleucine degradation, arachidonic acid metabolism. Less polar ginsenosides regulatethe linolenic acid metabolism, methionine metabolism and glucose alanine cycle.

Objective To observe the ultrasonic manifestations and outcomes of fetal congenital kidney anomalies(CKA)during the second trimester of pregnancy.Methods Data of 223 singleton fetuses with CKA detected with ultrasound during the second trimester of pregnancy were retrospectively analyzed.The ultrasonic manifestations of fetal CKA during the second trimester of pregnancy were observed,and the changes till the third trimester of pregnancy were analyzed.Results Among 223 CKA fetuses,simple renal pelvis separation was detected in 142 fetuses,while simple double renal pelvis malformation in was found in 41,polycystic kidney disease in 11,absent kidneys in 7,simple renal cysts and ectopic kidneys each in 6,smaller kidneys in 4,horseshoe kidneys and unilateral renal pelvis separation complicated with contralateral double renal pelvis malformation each in 2,unilateral renal cyst swelling complicated with contralateral double renal pelvis malformation and unilateral renal double renal pelvis malformation complicated with renal pelvis separation was noticed each in 1 fetus.Abnormalities such as renal pelvis separation,renal pelvis malformation,renal cysts and smaller kidney changed during the third trimester of pregnancy,while polycystic kidney disease,absent kidney,ectopic kidney and horseshoe kidney not significantly changed.Conclusion Fetal CKA had various types being able to change dynamically with the development of pregnancy,among which renal pelvis separation was the most common type.