Objective:To comprehensively evaluate treatment outcomes and assisted reproductive approaches in infertility patients with CYP17A1-deficient congenital adrenal hyperplasia [including 17α-hydroxylase/17,20-lyase deficiency (17-OHD) and isolated 17,20-lyase deficiency (IDL)]. Methods:In this study, PubMed, Embase, Web of Science, Ovid, CNKI, Wanfang Data base, and the Chinese Biomedical Literature Database were systematically searched. The data extracted included basic patient information, treatment plans, and pregnancy outcomes, which were then analyzed descriptively.Results:Totally 19 articles were included, covering 23 cases of successful live births. Among the 23 patients with successful live births, there were 19 patients with 17-OHD, including 17 cases became pregnant through in vitro fertilization and embryo transfer (IVF-ET), 1 case through natural pregnancy, and 1 case through oocyte donation pregnancy; there were 4 cases with IDL, including 2 cases through natural pregnancy and 2 cases through IVF-ET pregnancy. Conclusion:IVF-ET is the preferred fertility treatment option for patients with partial form of 17-OHD. For IDL patients who have natural ovulation, natural pregnancy can be attempted. Glucocorticoids play a crucial role in improving pregnancy outcomes, but their use needs to be individualized.

Objective To evaluate the methodological quality and measurement attribute quality of the evaluation tool for pediatric palliative care quality assessment tools,and to provide references for medical staff to select the best assessment tools.Methods The PubMed,Embase,Cochrane Library,Web of Science,CINAHL,Scopus,China National Knowledge Infrastructure(CNKI),Wanfang Database,VIP Database,Chinese Biomedical Literature Database,GIN,NGC,NICE,NRAO,medlive,WHO,AAHPM,WHPCA,APHN were searched from inception to March 28,2024.Data were screened and extracted independently by 2 researchers.The consensus-based standards for the selection of health measurement instruments(COSMIN)checklist and quality criteria were employed to evaluate the methodological quality and psychometric properties of the included pediatric palliative care quality assessment tools.Finally,recommendations were formulated based on these evaluations.Results A total of 13 articles were included,involving 9 pediatric palliative care quality assessment tools.Among them,the PICU-QODD,PaPEQu and QCPCI demonstrated good content validity and internal consistency,and are recommended as Grade A.The remaining assessment tools are recommended as Grade B or C.Conclusion The PICU-QODD,PaPEQu and QCPCI are recommended for use,but further validation of their psychometric properties is still needed.

Objective:To comprehensively evaluate treatment outcomes and assisted reproductive approaches in infertility patients with CYP17A1-deficient congenital adrenal hyperplasia [including 17α-hydroxylase/17,20-lyase deficiency (17-OHD) and isolated 17,20-lyase deficiency (IDL)]. Methods:In this study, PubMed, Embase, Web of Science, Ovid, CNKI, Wanfang Data base, and the Chinese Biomedical Literature Database were systematically searched. The data extracted included basic patient information, treatment plans, and pregnancy outcomes, which were then analyzed descriptively.Results:Totally 19 articles were included, covering 23 cases of successful live births. Among the 23 patients with successful live births, there were 19 patients with 17-OHD, including 17 cases became pregnant through in vitro fertilization and embryo transfer (IVF-ET), 1 case through natural pregnancy, and 1 case through oocyte donation pregnancy; there were 4 cases with IDL, including 2 cases through natural pregnancy and 2 cases through IVF-ET pregnancy. Conclusion:IVF-ET is the preferred fertility treatment option for patients with partial form of 17-OHD. For IDL patients who have natural ovulation, natural pregnancy can be attempted. Glucocorticoids play a crucial role in improving pregnancy outcomes, but their use needs to be individualized.

OBJECTIVES: To investigate the subcellular localization and biological functions of transmembrane protein 240 (TMEM240). METHODS: NCBI BLAST and TMHMM bioinformatics software were used for protein sequence analysis and prediction of transmembrane domain of TMEM240. Brain tissues from male C57BL/6 mice (18-20 days old) were examined for distribution of TMEM240 using in situ hybridization, and qPCR and Western blotting were used to detect TMEM240 expression in different mouse tissues and in cortical neurons at different time points (n=3). In the in vitro experiment, HepG2 and Neuro-2a cells were transfected with plasmids for overexpression of TMEM240, and subcellular localization of TMEM240 was analyzed using cell imaging. In primary cultures of cortical neurons isolated from C57BL/6 mice, TMEM240 expression and its biological functions were investigated using qPCR, Western blotting, and immunofluorescence staining. RESULTS: Human and mouse TMEM240 proteins share a 97.69% similarity in the protein sequences, and both are transmembrane proteins with two transmembrane domains. TMEM240 mRNA and protein were highly expressed in mouse brain tissues and cortical neurons. In isolated mouse cortical neurons, TMEM240 expression reached the peak level after primary culture for 9 days and distributed in scattered spots within the cells. In HepG2 cells, TMEM240 was characterized as intracellular membrane structures and showed 80% colocalization with peroxisomes. In Neuro-2a cells, TMEM240 overexpression caused significant enhancement of the expressions of Rho GDP dissociation inhibitor β (ARHGDIB) at both the mRNA and protein levels. CONCLUSIONS TMEM240 is a novel intracellular subcellular structure specifically expressed in neurons with significant potential for targeted cellular function regulation.
Animals ; Humans ; Mice ; Peroxisomes/metabolism* ; Membrane Proteins/genetics* ; Mice, Inbred C57BL ; Neurons/metabolism* ; Male ; rho-Specific Guanine Nucleotide Dissociation Inhibitors ; Hep G2 Cells ; Brain/metabolism*

Objective To evaluate the methodological quality and measurement attribute quality of the evaluation tool for pediatric palliative care quality assessment tools,and to provide references for medical staff to select the best assessment tools.Methods The PubMed,Embase,Cochrane Library,Web of Science,CINAHL,Scopus,China National Knowledge Infrastructure(CNKI),Wanfang Database,VIP Database,Chinese Biomedical Literature Database,GIN,NGC,NICE,NRAO,medlive,WHO,AAHPM,WHPCA,APHN were searched from inception to March 28,2024.Data were screened and extracted independently by 2 researchers.The consensus-based standards for the selection of health measurement instruments(COSMIN)checklist and quality criteria were employed to evaluate the methodological quality and psychometric properties of the included pediatric palliative care quality assessment tools.Finally,recommendations were formulated based on these evaluations.Results A total of 13 articles were included,involving 9 pediatric palliative care quality assessment tools.Among them,the PICU-QODD,PaPEQu and QCPCI demonstrated good content validity and internal consistency,and are recommended as Grade A.The remaining assessment tools are recommended as Grade B or C.Conclusion The PICU-QODD,PaPEQu and QCPCI are recommended for use,but further validation of their psychometric properties is still needed.

Objective To explore the neuro-protective effect of remimazolam(Rem)on traumatic brain injury(TBI)in rat models.Methods A TBI rat model was constructed.The rats were randomly divided into control group,traumatic brain injury group(TBI group),low-dose and high-dose remimazolam groups(Rem-L,Rem-H groups),and high-dose remimazolam+Jagged1 group(Rem-H+Jagged1 group),with 12 rats in each.All rats were evaluated for neurological deficits.The serum level of inflammatory factors like tumor necrosis factor α(TNF-α)and interleukin-1β(IL-1β)were detected by ELISA.HE staining microscopy was used to observe the changes of brain histopathology.The expression of glial fibrillary acidic protein(GFAP)and ionized calcium-binding adaptation molecule 1(IBA1)were detected by immunohistochemistry.Western blot was applied to detect the ex-pression of Notch,Notch 1 intracellular domain(NICD)and Hes-1 protein in the brain tissue.Results Compared with the control group,the TBI group showed a larger area of brain tissue defect and edema,with more activated glial cells and fragmented,concentrated and deeply stained neuronal nuclei,indistinct nucleoli,deeply stained cy-toplasm,and partial neuronal necrosis The neurological deficit score was higher,level of TNF-α and IL-1β and the expression of GFAP,IBA1,Notch,NICD,and Hes-1 all elevated(P＜0.05).Remazolam reduced brain tissue defect area,alleviated edema,inhibited glial cell activation and neuronal apoptosis,and reduced nerve function deficit score,the level of TNF-α and IL-1β,and the expression of GFAP,IBA1,Notch,NICD and Hes-1(P＜0.05).Jagged1 could aggravate brain tissue injury,increase neural function deficit score,levels of TNF-α and IL-1β and expressions of GFAP,IBA1,Notch,NICD and Hes-1(P＜0.05).Conclusions Remimazolam may have neuroprotective effects as shown by TBI rat models,and the underlying mechanism is potentially related to the inhibition of the Notch/Hes-1 signaling pathway.

Background::Ebstein’s anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein’s anomaly.Methods::A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020. 32 patients had previously received cardiac surgery in other hospitals. The clinical data including diagnosis, operative indications, techniques, pathological changes, and survival rates were collected and analyzed.Results::Anatomical correction was performed in 203 (78.7%) operations, 1? ventricle repair in 38 (14.7%) operations, tricuspid valve repair only in four operations (1.6%), tricuspid valve replacement in ten (3.9%), total cavopulmonary connection (TCPC) in two (0.8%), and Glenn operation in one operation (0.4%). Reoperation was performed in five patients (2.0%) during hospitalization. Among them, tricuspid valve replacement was performed in one patient, 1? ventricle repair in two patients, and tricuspid valve annulus reinforcement in two patients. Five patients died with an early mortality rate of 2.0%. Complete atrioventricular conduction block was complicated in one patient (0.4%). A total of 244 patients was followed up (four in the 253 patients lost) with a duration of 3.0-168.0 (87.6 ± 38.4) months. Cardiac function of 244 patients improved significantly with mean New York Heart Association (NYHA) functional class recovery from 3.5 to 1.1. The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5. Three late deaths (1.2%) occurred. The survival rates at five and ten years after surgery were 98.6% and 98.2%, respectively. Reoperation was performed in five patients (2.0%) during the follow-up period.Conclusion::Based on our management strategies and operative principles and techniques, anatomical correction of EA is capable of achieving excellent long-term results, and low rates of TCPC, 1? ventricle repair and valvular replacement.

Objective:To systematically evaluate the hospice care needs of end-stage AIDS patients.Methods:Qualitative research on the hospice care needs of end-stage AIDS patients was electronically searched on China National Knowledge Infrastructure, WanFang Data, China Biology Medicine disc, PubMed, Cochrane Library, PsychINFO, Web of Science, Embase, and so on. The search period was from database establishment to November 20, 2023. Quality evaluation criteria for qualitative research of the Joanna Briggs Institute Evidence-Based Health Care Center was used to evaluate the quality of literature. The Meta-synthesis method was used to integrate the results.Results:A total of 14 articles were included. Sixty-nine results were extracted and classified into six categories, resulting in five integrated results, including personalized support and care needs, psychological counseling and dignity maintenance, social assistance, spiritual sustenance, and information and decision-making needs.Conclusions:The end-stage AIDS patients have multi-dimensional needs for hospice care. Hospital and community staff should pay attention to the hospice care needs of end-stage AIDS patients and provide adequate care for patients.

This paper summarized Professor PENG Peichu's experience in the differentiation and treatment of prostate cancer in three phases and four stages. It is considered that prostatic cancer is categorized into root deficiency and branch excess， with depletion of healthy qi as the root， and the accumulation of cancer toxin as the minifestation. Clinical diagnosis and treatment of prostatic cancer can be divided into three phases and four stages according to the exuberance and decline of pathogenic and healthy qi and the changes of deficiency and excess of yin and yang. In the initial accumulation phase of cancer toxin （yang excess stage）， the key pathogenesis is the accumulation of dampness， heat and static blood， and internal generation of cancer toxin， and the treatment should be resolving toxins， fighting cancer and dispelling yang excess. In the phase of healthy qi deficiency and toxin accumulation （yin deficiency stage）， with the lung and kidney yin deficiency， dampness， heat and static toxin accumulation as the key pathogenesis， the treatment should be centered on mutual generation between metal and water to nourish kidney yin， supplemented with the method of clearing heat and draining dampness， activating blood and resolving toxins， for which self-made Nanbei Formula（南北方）is usually used. In the phase of yang deficiency and cold stagnation （yang deficiency stage and yin excess stage）， with the spleen and kidney yang deficiency， cold dampness stagnation， static heat and toxin accumulation as the key pathogenesis， the treatment should be warming and tonifying spleen and kidney to dissipate cold accumulation； for deficiency of both yin and yang， and excess pathogen obstruction， modified Yanghe Decoction（阳和汤） is recommended， while for yang deficiency， cold congealing and blood stasis， self-made Wenshen Sanjie Formula（温肾散结方） can be used， and for cold dampness binding with cancer toxin， and cold complex with heat， self-made Quanan Formula （泉安方） is advised.

Objective To understand the research status,hotspots and trends of Wuzhuyu Decoction;To provide reference for relevant research.Methods The literature related to Wuzhuyu Decoction was retrieved from CNKI,VIP,Wanfang Data and CBM databases from the establishment to February 28,2023.NoteExpress 3.6 was used to merge and deduplicate,and the author,organization and keywords were mapped and interpreted by CiteSpace 6.1.R6 software.Results A total of 822 articles were included,and the number of publications showed a wave upward trend;the top journals were New Chinese Medicine,Henan Traditional Chinese Medicine and Sichuan Traditional Chinese Medicine;the main research institutions were Beijing University of Chinese Medicine,China Academy of Chinese Medical Sciences and Shandong University of Traditional Chinese Medicine;a total of 566 authors were involved,and the authors with more publications included Wang Zhimin(12),Gong Muxin(9)and Bi Kaishun(5);high-frequency keywords included"headache","Shang Han Lun","TCM therapy"and so on.Conclusion Wuzhuyu Decoction is effective in treating chronic gastritis,hypertension and other primary diseases,which is a research hotspot in this field.It is a research trend in this field to explore its active components by high performance liquid chromatography and explain its action mechanism and target at the molecular level.