Objective: To analyze the demographic characteristics of patients with red blood cell transfusion reactions, the usage of red blood cell preparations, and the differences in the composition ratio of adverse reactions based on multi-center data from the Haemovigilance Network, in order to reveal the clinical characteristics of red blood cell transfusion and its underlying issues. Methods: Clinical data of patients who experienced transfusion reactions after red blood cell transfusion in the Haemovigilance Network from 2018 to 2023 were collected. The demographic characteristics of patients who experienced transfusion reactions with different types of red blood cell preparations, the utilization of these preparations, and the differences of the composition ratios of transfusion reactions were analyzed. Count data were expressed as numbers (n) or percentages (%), and comparisons between groups were performed using the Chi-square test. Results: Red blood cell transfusion reactions were more common in females (53.56%), with the majority of patients aged 50-69 years (35.54%). The Han polulation accounted for the vast majority of patients (92.77%), and patients in the hematology and obstetrics/gynecology departments had a relatively high proportion of transfusion reactions (13.26% and 14.26%, respectively). Leukocyte-reduced red blood cells and suspended red blood cells were the most common types of transfusion reactions reported among red blood cell preparations. Allergic reactions and non-hemolytic febrile reactions were the most common transfusion reactions, and there were significant differences in the composition ratios of allergic reactions (χ =869.89, P<0.05) and non-hemolytic febrile reactions (χ =812.75, P<0.05) across various types of red blood cell preparations. Conclusion: There are differences in the demographic characteristics and composition ratio of transfusion reactions among different red blood cell preparations. The management of red blood cell transfusion reactions should be tailored to patient characteristics and conditions, and the selection and use of blood products should be optimized to reduce or avoid the occurrence of transfusion reactions, such as considering the use of washed red blood cells for patients with a history of transfusion allergies or those prone to allergies.

Objective:To summarize experience of managing adult Langerhans cell histiocytosis(LCH) in hypothalamic-pituitary region(HPR) from Shanghai Huashan Hospital.Methods:Adult HPR-LCH patients diagnosed at oar endocrinology department from January 2013 to February 2022 were included. Clinical characteristics and treatment response were retrospectively analyzed.Results:A total of 27 adult HPR-LCH patients were included, with 14 cases involving the hypothalamus(H group) and 13 cases without(group NH). The common radiological findings included thickening of the pituitary stalk(25/27, 92.6%). At the time of diagnosis, 14 cases(51.9%) presented with panhypopituitarism, and 19 cases(70.4%) exhibited metabolic abnormalities. The group H had higher proportions of adrenal insufficiency, central hypothyroidism, panhypopituitarism, and diabetes compared to group NH(78.6% vs 23.1%; 78.6% vs 23.1%; 92.9% vs 30.8%, 35.7% vs 0%, respectively, all P<0.05). Hypothalamus syndrome was identified in 71.4%(10/14) of group H. The inital diagnosis rate was 79.2%(19/24), with 48.1% and 51.9% through biopsy of sellar and extrasellar lesions, respectively. Repeated biopsies confirmed the diagnosis in 25.9%(7/27) of cases. The peripheral lesions included bone, thyroid, lung, lymph node, thymus and liver. Out of 20 cases treated with chemotherapy, the objective response rate was 85% at 12 weeks. Four cases received local therapy, one case received traditional Chinese medicine treatment, one case abandoned treatment, and one case was lost to follow-up. The median follow-up time was 28(range 15 to 54) months. During this period, there were 3 deaths in group H and 1 death in group NH. Conclusion:Adult HPR-LCH patients presented with diabetes insipidus and high prevalences of hypopituitarism, hypothalamus syndrome and metabolic abnormalities. Typical imaging features were pituitary stalk thickening. A solitary mass in the HPR was usually very small, posing a great challenge for early diagnosis. Systemic evaluation would help to clarify the diagnosis. Patients with hypothalamus involvement had a higher mortality rate, suggesting the hypothalamus as a risk organ with poor prognosis.

Objective:To investigate the carriage status of Neisseria meningitidis ( Nm) among the healthy population in Hebei Province for the prevention and control of meningitis. Methods:From 2015 to 2022, throat swabs were collected from health people, which were selected by cluster random sampling in 11 cities of Hebei.The positive rate of Nm was detected by bacterial culture. The serogroups of isolated strains were identified.The laboratory detection results of Nm strains, combined with epidemiological survey data, were synthetically analyzed. Results:A total of 20 245 people were investigated; 249 strains of Nm were isolated; the overall Nm carriage rate was 1.23%. The carriage rate was significantly higher in men than in women(χ 2=28.831, P<0.05). The positive rates of Nm in different age groups were significantly different(χ 2=428.018, P<0.05), with the highest rates in the 15-19 year-old group(4.90%, 149/3 042). The positive rates of Nm were significantly different in different regions(χ 2=177.512, P<0.05), with the highest positive rate of Nm in Xingtai, Shijiazhuang, Chengde and Baoding city in sequence. Among the isolated Nm strains, ungroupable serogroups, serogroup B, serogroup C, and serogroup W accounted for 71.49%(178/249), 13.65%(34/249), 6.83%(17/249) and 4.42%(11/249), respectively. Conclusions:The carriage rate of Nm among healthy population is generally low in Hebei Province. It is recommended to continue to strengthen monitoring, pay attention to the changes and distribution characteristics of Nm, and formulate scientific and targeted prevention and control measures of meningococcal disease.

Objective To strengthen the supervision of medical equipment procurement and construct a scientific and systematic evaluation system for procuring agency supervision,providing a reference for public hospitals to implement government procurement of medical equipment.Methods The Delphi method was employed to establish the indexes and weights of the su-pervision evaluation system.Subsequently the fuzzy comprehensive evaluation method was applied to comprehensively analyze the procuring agencies X and Y.Results The evaluation results of agencies X and Y were compared and analyzed in terms of work efficiency,business capability,organization of bid evaluation,and service quality.Additionally,the results were regularly fed back and improved.Conclusion The establishment of the supervision and evaluation system for procuring agencies is conducive to promoting the standardization and efficiency of government procurement activities of medical equipment in public hospitals and safeguarding the legitimate rights and interests of procurement participants as well as the public interests.

Surfactant is a kind of substance that can significantly reduce the surface/interface tension.Surfactant is an important substance that affects the preparation technology,stability and safety of nanoformulation.By combing the structure classification of surfactants and their function mechanism in nanoformulation,combined with the research status at home and abroad,the application and research of surfactants in the listed nanoformulation were described,in order to provide reference for the research and development of new nanoformulation.

Objective:To investigate the iodine nutritional status of pregnant women in Hengshui City, so as to provide a basis for adjusting the prevention and control strategies of iodine deficiency disorders in key populations in Hengshui City.Methods:Using a cross-sectional survey method, 100 pregnant women were randomly selected from iodine deficient counties (cities, districts) in Hengshui City from 2017 to 2023 each year. Random urine samples and household salt samples were collected once a year to measure urine iodine and salt iodine levels. The iodine nutritional status of pregnant women was evaluated and influencing factors were analyzed.Results:A total of 6 570 urine samples from pregnant women were tested, with a median urinary iodine level of 158.5 μg/L. Pregnant women with urinary iodine levels below 150 μg/L accounted for 46.0% of the total population (3 022/6 570). There was a statistically significant difference in the median urinary iodine levels among pregnant women in different years ( H = 51.52, P < 0.001), but there was no statistically significant difference in the median and frequency distribution of urinary iodine among pregnant women in different age groups ( Z = - 1.34, P = 0.181; χ 2 = 1.35, P = 0.717). There was a statistically significant difference in the median urinary iodine levels among pregnant women during different stages of pregnancy ( H = 10.61, P = 0.005), with the median urinary iodine levels in early pregnancy being higher than those in late pregnancy. Spearman rank correlation analysis results showed that the urinary iodine level of pregnant women was positively correlated with the edible salt iodine level ( r = 0.08, P < 0.001). Conclusions:The overall iodine nutrition level of pregnant women in Hengshui City has reached the recommended adequate level (150 - 249 μg/L), but some pregnant women still face the risk of iodine deficiency. We should pay more attention and do a good job in supplementing iodine for pregnant women during pregnancy.

ObjectiveTo investigate the immunological characteristics of the patients with aplastic anemia （AA） and elevated hemogram parameters treated with Yiqi Yangxue prescription combined with Western medicine and the predictive effects of immunological indexes on elevated hemogram parameters， thus providing a reference for the prediction of the treatment efficacy and the adjustment of the treatment regimen. MethodA retrospective study was conducted， involving 77 AA patients treated with Yiqi Yangxue prescription combined with Western medicine for 6 months in 19 medical institutions including Xiyuan Hospital， China Academy of Chinese Medical Sciences from September 2018 to March 2021. The patients were assigned into two groups according to the elevations in hemogram parameters ［including hemoglobin （HGB）， white blood cell count （WBC）， platelet （PLT）， and absolute neutrophil count （ANC）］ after 6 months of treatment. One group had the elevation <50%， and the other group had the elevation ≥50% compared with the baseline. The clinical and immunological characteristics were compared between the two groups. Result① Compared with the group with HGB elevation<50%， the group with HGB elevation≥50% showed elevated level of CD3⁺ human leukocyte antigen-DR （HLA-DR）⁺ and increased proportion of patients with T-helper cell type 2 （Th2）<5%， CD8⁺≥50%， and CD3⁺HLA-DR⁺≥9% before treatment （P<0.05， P<0.01）. The multivariate Logistic regression analysis showed that CD8⁺≥50% before treatment was the independent influencing factor for HGB elevation ≥50% ［odds ratio （OR）=12.000， 95% confidence interval （CI） 2.218， 64.928， P<0.01］. ② Compared with the group with WBC elevation<50%， the group with WBC elevation≥50% showed increased proportion of patients with CD3⁺HLA-DR⁺<6% and T-box transcription factor （T-bet）≥200% before treatment （P<0.05）. The multivariate Logistic regression analysis showed that CD3⁺HLA-DR⁺<6% （OR=2.998， 95%CI 1.036， 8.680， P<0.05） and T-bet≥200% （OR=3.634， 95%CI 1.076， 12.273， P<0.05） before treatment were independent influencing factors for WBC elevation≥50%. ③ Compared with the group with PLT elevation<50%， the group with PLT elevation≥50% presented lowered Th1 and CD3⁺HLA-DR⁺ levels and increased proportion of patients with Th1<12%， CD4⁺≥6%， and CD3⁺HLA-DR⁺<5% before treatment （P<0.05， P<0.01）. The multivariate Logistic regression analysis showed that CD3⁺HLA-DR⁺<5% before treatment was the independent influencing factor for PLT elevation≥50% （OR=16.190， 95%CI of 3.430 to 76.434， P<0.01）. ④ Compared with the group with ANC elevation<50%， the group with ANC elevation≥50% showed no significant changes in the hemogram parameters before treatment. ConclusionAs for the AA patients with rapid elevation in HGB， Yiqi Yangxue prescription combined with Western medicine demonstrate significant effects in the patients with Th2<5% and CD3⁺HLA-DR⁺≥9%， especially those with CD8⁺≥50%. As for the AA patients with rapid elevation in WBC， the therapy was particularly effective in the patients with CD3⁺HLA-DR⁺<6% and T-bet≥200%. As for the AA patients with rapid growth in PLT， the therapy was particularly effective in the patients with Th1<12% and CD4⁺≥6%， especially those with CD3⁺HLA-DR⁺<5%.

Objective To observe the clinical effect of Ziyin Qingshenyin on lupus nephritis(LN)with Yin deficiency and internal heat syndrome.Methods Sixty patients diagnosed with LN were selected from June 2020 to May 2022 from our hospital as research partici-pants and randomly divided into control group(n=30)and combination group(n=30).The control and combination groups were adminis-tered conventional Western medicine and conventional Western medicine with Ziyin Qingshenyin,respectively.Clinical efficacy,traditional Chinese medicine(TCM)syndrome score and efficacy,renal function index[serum albumin(ALB),serum creatinine(sCr),hemoglobin(Hb),blood urea nitrogen(BUN),and 24 h urinary protein quantitation(24 h Upro)],immunological indicators[complement C3,com-plement C4,immunoglobulin(Ig)M,IgG,and IgA],inflammatoryindicators[C-reactive protein(CRP)and erythrocyte sedimentation rate(ESR)],disease activity score of systemic lupus erythematosus(SLEDAI),and safety were determined.Results The total effective rate(86.67%,83.33%)of the combination group was higher than that of the control group(63.33%,60.00%)(P＜0.05).Following treatment,the five syndrome scores of heat,sore throat,night sweats,and macula in combination group were lower than those in the control group(P＜0.05).After treatment,24 h Upro,BUN,sCr,IgM,IgG,IgA,CRP,ESR,and SLEDAI scores in the combination group were lower than those in control group,while ALB,Hb,complement C3,and complement C4 were higher than those in the control group(P＜0.05).There was no significant difference in the incidence of adverse reactions between the two groups(P＞0.05).Conclusion The Ziyin Qingshen-yin treatment has notable effects on LN with Yin deficiency and internal heat syndrome,which can improve renal function and immunolog-ical indexes,reduce inflammatory factors,and contribute to disease control.

Primary ciliary dyskinesia(PCD)is a rare monogenic disorder primarily associated with structural and functional abnormalities of motile cilia.It is typically inherited in an autosomal recessive pattern.The disease affects multiple organs,and the variability in clinical phenotypes,along with genetic heterogeneity significantly complicates its diagnosis.Although the application of clinical exome sequencing has significantly improved the diagnostic rate of PCD,more than 30％of patients are still unable to obtain a definitive diagno-sis.This article reviews and discusses the pathogenesis,diagnostic methods,and expanding genetic diag-nostic approaches for patients with PCD that are negative for exome sequencing.The aim of this article is to assist clinicians in selecting more advanced emerging genetic testing technologies,with the hope of increasing the positive diagnostic rate of PCD,deepening the understanding of its genetic pathogenesis,and laying a foundation for the practice of gene therapy in the future.

Temporomandibular joint (TMJ) diseases are common clinical conditions. The number of patients with TMJ diseases is large, and the etiology, epidemiology, disease spectrum, and treatment of the disease remain controversial and unknown. To understand and master the current situation of the occurrence, development and prevention of TMJ diseases, as well as to identify the patterns in etiology, incidence, drug sensitivity, and prognosis is crucial for alleviating patients′suffering.This will facilitate in-depth medical research, effective disease prevention measures, and the formulation of corresponding health policies. Cohort construction and research has an irreplaceable role in precise disease prevention and significant improvement in diagnosis and treatment levels. Large-scale cohort studies are needed to explore the relationship between potential risk factors and outcomes of TMJ diseases, and to observe disease prognoses through long-term follw-ups. The consensus aims to establish a standard conceptual frame work for a cohort study on patients with TMJ disease while providing ideas for cohort data standards to this condition. TMJ disease cohort data consists of both common data standards applicable to all specific disease cohorts as well as disease-specific data standards. Common data were available for each specific disease cohort. By integrating different cohort research resources, standard problems or study variables can be unified. Long-term follow-up can be performed using consistent definitions and criteria across different projects for better core data collection. It is hoped that this consensus will be facilitate the development cohort studies of TMJ diseases.