The clinical practice guidelines of traditional Chinese medicine （TCM） have problems such as limited clinical application and unclear implementation effects， which may be related to the lack of clinical practice evidence. To provide reliable and precise evidence for clinical practice， this article proposes a model of combining TCM guidelines with real-world study， which includes 4 steps. Firstly， during the implementation process of the guidelines， a high-quality research database is established. Secondly， the recommendations in the guidelines are evaluated based on the established database in multiple dimensions， including applicability， effectiveness， safety， and cost-effectiveness， and thus their effectiveness in practical applications can be determined. Thirdly， based on the established database， core prescriptions are identified， and the targeted populations and medication plans are determined. That is， the best treatment regimen is established based on the analysis of abundant clinical data regarding the effects of different medication frequencies， dosages， and duration on efficacy. Fourthly， the guidelines are updated according to the real-world evidence. The research based on this model can provide real-world evidence for ancient and empirical prescriptions， improving their application in clinical practice. Moreover， this model can reduce research costs and improve research efficiency. When applying this model， researchers need to pay attention to the quality of real-world evidence， ensuring that it can truly reflect the situation in clinical practice. In addition， importance should be attached to the clinical application of guideline recommendations， ensuring that doctors can conduct standardized diagnosis and treatment according to the guidelines. Finally， full-process participation of multidisciplinary experts is encouraged to ensure the comprehensiveness and scientificity of the study. In conclusion， the application of this model will contribute to the development of TCM guidelines responsive to the needs of clinical practice and achieve the goal of promoting the homogenization of TCM clinical diagnosis and treatment.

Objective:To investigate the efficacy and safety of protein A immunoadsorption (PAIA) combined with rituximab (RTX) in highly sensitized patients who underwent haplo-hematopoietic stem cell transplantation (haplo-HSCT) .Methods:The clinical data of 56 highly sensitized patients treated with PAIA and RTX before haplo-HSCT at the First Affiliated Hospital of Soochow University and Soochow Hopes Hematonosis Hospital between March 2021 and June 2023 were retrospectively analyzed. The number of human leukocyte antigen (HLA) antibody types and the mean fluorescence intensity (MFI), humoral immunity, adverse reactions during adsorption, and survival within 100 days before and after adsorption were measured.Results:After receiving the PAIA treatment, the median MFI of patients containing only HLA Ⅰ antibodies decreased from 7 859 (3 209-12 444) to 3 719 (0-8 275) ( P<0.001), and the median MFI of HLA Ⅰ+Ⅱ antibodies decreased from 5 476 (1 977-12 382) to 3 714 (0-11 074) ( P=0.035). The median MFI of patients with positive anti-donor-specific antibodies decreased from 8 779 (2 697-18 659) to 4 524 (0–15 989) ( P<0.001). The number of HLA-A, B, C, DR, and DQ antibodies in all patients decreased after the PAIA treatment, and the differences were statistically significant (A, B, C, DR: P<0.001, DQ: P<0.01). The humoral immune monitoring before and after the PAIA treatment showed a significant decrease in the number of IgG and complement C3 ( P<0.001 and P=0.002, respectively). Forty-four patients underwent HLA antibody monitoring after transplantation, and the overall MFI and number of antibody types decreased. However, five patients developed new antibodies with low MFI, and nine patients continued to have high MFI. The overall survival, disease-free survival, non-recurrent mortality, and cumulative recurrence rates at 100 days post-transplantation were 83.8%, 80.2%, 16.1%, and 4.5%, respectively. Conclusions:The combination of PAIA and RTX has a certain therapeutic effect and good safety in the desensitization treatment of highly sensitive patients before haplo-HSCT.

ObjectiveTo tentatively understand the status of radioactive contamination in nuclear medicine personnel. MethodsA total of 34 radiation staff engaged in nuclear medicine diagnosis and treatment were selected from two hospitals in Shanghai as the survey subjects.Among the 34 medical staff， 8 were nuclear medicine doctors， 14 were nuclear medicine technicians and 12 were nuclear medicine nurses. After surface contamination monitoring was first carried out to confirm that they had no surface radioactivity contamination， whole body scanning was performed with a whole body counter to determine whether they were internally contaminated with artificial radionuclides. ResultsThe α surface contamination was not detected in the nuclear medical staff. The β surface contamination of the nuclear medicine doctors， technicians and nurses was （13.8±0.8）， （14.1±0.8） and （14.0±0.7） times per second， respectively. There were 2， 2， and 4 nuclear medicine doctors who were contaminated with ¹⁸F， ^99mTc and ¹³¹I， 3， 5， and 2 nuclear medicine technicians who were contaminated with ¹⁸F， ^99mTc and ¹³¹I， and 6， 8， and 5 nuclear medicine nurses who were contaminated with ¹⁸F， ^99mTc and ¹³¹I， respectively. The ¹⁸F activity of nuclear medicine technicians was 1 997‒9 401 Bq， and the ^99mTc activity of nuclear medicine technicians and nurses was 3 699‒18 692 and 652‒388 22 Bq， respectively. One nuclear medicine nurse had a ^99mTc activity of 35 389 Bq. According to the preliminary estimation of ¹³¹I internal irradiation dose， the maximum committed effective dose of nuclear medicine doctors， technicians and nurses could reach 0.370， 0.018 and 0.584 mSv， respectively. ConclusionThe nuclear medicine staff are exposed to radioactive contamination， and it is important to monitor and evaluate their internal radiation doses.

Background Based on numerous epidemiological studies, radionuclide ¹³¹I can result in thyroid disease. Objective To study the contamination level of thyroid ¹³¹I in ¹³¹I treatment associated radiation workers in nuclear medicine departments in Shanghai. Methods Based on a general survey on basic situation of nuclear medicine in Shanghai, the level of internal exposure of radiation workers involved in ¹³¹I treatment was assessed with questionnaires and on-site monitoring. A portable γ spectrometer was used for on-site detection, the intake and annual effective dose were estimated according to the measurement results. Physical examination reports were collected for radionuclide positive workers, and chromosome testing was performed. Results There were 579 nuclear medicine workers and 175 were engaged in ¹³¹I treatment in Shanghai. ¹³¹I was detected in thyroid of 18 workers, with a detection rate of 10.3%. Among them, the detection rates of thyroid ¹³¹I in nurses and cleaners were 23% and 25% respectively. The mean measured activity of thyroid ¹³¹I was 306.1 Bq and the maximum measured activity was 3716.9 Bq (nurse). The maximum intake was estimated at 37544 Bq and the median was 786.4 Bq. The average annual committed effective dose was 1.22 mSv and the maximum value was 14.87mSv. The chromosomal aberration rates of all detected workers were negative, and all blood indicators were normal except one with low hemoglobin. Conclusion The annual dose of nuclear medical staff engaged in ¹³¹I treatment does not exceed the national standard limit, but the internal exposure of nurses and cleaners should not be ignored. On the basis of strengthening protection and decontamination, routine internal exposure monitoring should be carried out.

ObjectiveTo explore the biological mechanism of drought improving the quality of Rhizoma Atractylodis Chinensis and establish a new method for the production of high-quality medicinal materials. MethodThe fresh roots of Atractylodes chinensis were soaked in 0 （control）， 5%， 10%， and 20% PEG-6000 solutions. The changes in reactive oxygen species （ROS） level， antioxidant enzyme activity， activities of key enzymes in primary metabolism and secondary metabolism， and content of secondary metabolites were compared. ResultCompared with the control group， the treatment with 20% PEG for 2 days elevated the levels of superoxide anion radicals （O2-·）， hydrogen peroxide （H₂O₂）， and malondialdehyde （MDA） by 172.5%， 56.9%， and 14.7%， respectively. The treatment did not change the activity of superoxide dismutase （SOD）， reduced the peroxidase （POD） activity， and increased the catalase （CAT） activity by 10.8%. It increased the activities of 3-hydroxy-3-methylglutaryl-CoA reductase （HMGR）， phosphoenolpyruvate carboxylase （PEPC）， and acetyl-CoA carboxylase （ACC） by 49.9%， 12.1%， and 19.0%， respectively. Furthermore， the content of atractylodin， β-eudesmol， atractylone， and atractylenolide Ⅱ was increased by 51.0%， 36.9%， 47.1%， and 91.5%， respectively. The simulated drought stress can cause the burst of ROS in the fresh roots of A. chinensis， induce the physiological state of plants under drought， change the antioxidant system， and promote the massive synthesis of secondary metabolites in a short time. ConclusionPEG-6000-simulated drought stress can greatly improve the quality of A. chinensis in cultivation.

OBJECTIVE: To explore the genetic basis for 7 families with gonadal mosaicism for Duchenne muscular dystrophy (DMD). METHODS: For the 7 families presented at the CITIC Xiangya Reproductive and Genetic Hospital from September 2014 to March 2022, clinical data were collected. Preimplantation genetic testing for monogenic disorders (PGT-M) was carried out for the mother of the proband from family 6. Peripheral venous blood samples of the probands, their mothers and other patients from the families, amniotic fluid samples from families 1 ~ 4 and biopsied cells of embryos cultured in vitro from family 6 were collected for the extraction of genomic DNA. Multiplex ligation-dependent probe amplification (MLPA) was carried out for the DMD gene, and short tandem repeat (STR)/single nucleotide polymorphism (SNP)-based haplotypes were constructed for the probands, other patients, fetuses and embryos. RESULTS: The results of MLPA showed that the probands and the fetuses/probands' brothers in families 1 ~ 4, 5, 7 had carried the same DMD gene variants, whilst the probands' mothers were all normal. The proband in family 6 carried the same DMD gene variant with only 1 embryo (9 in total) cultured in vitro, and the DMD gene of the proband's mother and the fetus obtained through the PGT-M were normal. STR-based haplotype analysis showed that the probands and the fetuses/probands' brothers in families 1 ~ 3 and 5 have inherited the same maternal X chromosome. SNP-based haplotype analysis showed that the proband from family 6 has inherited the same maternal X chromosome with only 1 embryo (9 in total) cultured in vitro. The fetuses in families 1 and 6 (via PGT-M) were both confirmed to be healthy by follow up, whilst the mothers from families 2 and 3 had chosen induced labor. CONCLUSION Haplotype analysis based on STR/SNP is an effective method for judging gonad mosaicism. Gonad mosaicisms should be suspected for women who have given births to children with DMD gene variants but with a normal peripheral blood genotype. Prenatal diagnosis and reproductive intervention may be adapted to reduce the births of further affected children in such families.
Male ; Pregnancy ; Child ; Humans ; Female ; Muscular Dystrophy, Duchenne/diagnosis* ; Dystrophin/genetics* ; Mosaicism ; Exons ; Prenatal Diagnosis/methods* ; Nucleotides

Autapses selectively form in specific cell types in many brain regions. Previous studies have also found putative autapses in principal spiny projection neurons (SPNs) in the striatum. However, it remains unclear whether these neurons indeed form physiologically functional autapses. We applied whole-cell recording in striatal slices and identified autaptic cells by the occurrence of prolonged asynchronous release (AR) of neurotransmitters after bursts of high-frequency action potentials (APs). Surprisingly, we found no autaptic AR in SPNs, even in the presence of Sr²⁺. However, robust autaptic AR was recorded in parvalbumin (PV)-expressing neurons. The autaptic responses were mediated by GABA_A receptors and their strength was dependent on AP frequency and number. Further computer simulations suggest that autapses regulate spiking activity in PV cells by providing self-inhibition and thus shape network oscillations. Together, our results indicate that PV neurons, but not SPNs, form functional autapses, which may play important roles in striatal functions.
Parvalbumins/metabolism* ; Corpus Striatum/metabolism* ; Interneurons/physiology* ; Neurons/metabolism* ; Neostriatum

Acromicric dysplasia(AD) is a rare skeletal dysplasia characterized by severe short stature, short hands and feet, normal intelligence, mild facial dysmorphism, and radiological characteristics. The clinical data and genetic test results of one patient with AD in our hospital were analyzed, and the clinical characteristics of this case were summarized. The main manifestations of the child were short stature, short hands and feet, mild facial dysmorphism, short and stubby metacarpals and phalanges on hand X-ray. One mutation, FBN1: c.5141T>G(p.Met1714Arg), was identified in this child, the mutation is inherited from her short mother and grandfather. AD is a rare congenital skeletal dysplasia disorder associated with mutations in the FBN1 gene. It conforms to the pathogenesis of autosomal dominant genetic disease.

Objective: To analyze under 5 years old mortality rate during 2016-2021 in Xicheng District, Beijing, and to provide scientific basis for further reducing infant mortality rate and under 5 years old mortality rate. Methods: A descriptive statistical analysis was conducted on the mortality surveillance data of children under 5 years old reported by Preventive Health Department of Xicheng District Community Health Service Center during 2016-2021. Results: During 2016-2021, under 5 mortality, infants and newborn mortality in Xicheng District decreased from 2.31‰, 1.85‰ and 1.12‰ to 1.55‰, 1.07‰ and 0.48‰ respectively. There was no significant difference in mortality rate between 2016 and 2021( P >0.05). The leading cause of death among children under 5 years of age included other congenital abnormalities(except congenital heart disease and down s syndrome)(18.79%), preterm birth/low birth weight (16.36%) and birth asphyxia(7.88%). Infant deaths accounted for 69.23%- 80.00 % of deaths of children under 5 years of age, and neonatal mortality accounted for 44.44%-71.43% of infant mortality. Conclusion It is necessary to carry out relevant preventive intervention and quality management based on the main causes of death, and to further improve the management of critically ill neonates and reduce neonatal mortality.

Objective:To summarize the experience on accurate prevention and control of children′s emergency department during the epidemic of novel coronavirus Omicron variant.Methods:We retrospectively analyzed the strategies and management experience of emergency prevention and control of novel coronavirus infection in emergency department at Children′s Hospital of Fudan University from March to May 2022.Results:As a designated hospital for treating pediatric patients who contracted novel coronavirus in Shanghai, the emergency department in our hospital was confronted with the dual pressure of critical patients treatment and pandemic prevention and control.We carefully studied a series of laws and regulations, as well as the newest edition of Chinese clinical guidance for novel coronavirus pneumonia diagnosis and treatment, and combined with the characteristics of novel coronavirus infection in children, then formulated the independent emergency department, fever clinics and novel coronavirus clinics; Updated the emergency department pre-examination triage process, the precautions pratice of clinical stuffs and disfection strategy, and established the second emergency department.From the beginning of March to the end of May 2022, a total of about 12 000 patients were admitted to the emergency department in our hospital, including 704 patients in the resuscitation room, 652 patients in the observation room, and 164 patients in the emergency ward.There were six patients with novel coronavirus infection in the emergency department.Neither nosocomial infection nor occupational exposure occurred.Conclusion:After 3 months of practice, the results showed that it can fully guarantee the timely treatment of critically ill children and achieved zero cross-infection in the hospital, which has important reference significance for the treatment of children, epidemic prevention, control during the novel coronavirus epidemic.