RAG mutations cause a spectrum of severe immunodeficiencies ranging from classical severe combined immunodeficiency( SCID) and Omenn syndrome to an increasing number of peculiar phenotypes. Based on the distinct levels of RAG expression in various patients, their immunophenotypes, histopathological findings and clinical manifestations are diverse. The subtypes of RAG-defect diseases have been described as classical SCID,SCID with maternal T cells,classical Omenn syndrome,atypical Omenn syndrome,granuloma-tous inflammation,predominance/expansion of γδ-T cells and maternal T-cell engraftment. The complete failure or partial blockage of lymphocyte development and differentiation can cause repeated infections with autoimmune reactions frequently,and may be lethal. Thorough assessment and interpretation of various phenotypes will guide accurate diagnosis,definitive treatment and the mechanism research.

Objective To evaluate the effects of mild hypothermia performed at different times on the levels of glutamate, Bcl-2 and Bax during global cerebral ischemia-reperfsusion (I/R) in rats. Methods Twenty-four male SD rats weighing 230-270 g were randomly divided into 4 groups according to the time at which mild hypothermia was performed ( n =6 each): group A, B, C and D. Global cerebral ischemia was produced by occlusion of 4 vessels (cauterization of bilateral vertebral arteries and occlusion of bilateral common carotid arteries) in the 4 groups. In group B, C and D, the nasopharyngeal temperature was reduced to 32.5-33.5 ℃ and maintained for 1 h. Ischemia was performed after termination of cooling in group B. While ischemia was performed, cooling was started in group C. While reperfusion was performed, cooling was started in group D. Rewarming was started after termination of cooling in group B, C and D. Samples of dialysate in hippocampal CA1 area were collected every 10 min during 100 min reperfusion for determination of glutamate concentrations by high-performance capillary electrophoresis with laser-induced fluorescence detection ( HPCE-LIF). The brain tissues were taken at 3 h of reperfusion for determination of the expression of Bax and Bcl-2 in hippocampal CA1 area, and the ratio of Bcl-2/Bax was calculated. Results Compared with group A, the glutamate concentrations were significantly decreased, the Bcl-2 expression was up-regulated, the Bax expression was down-regulated and the ratio of Bcl-2/Bax was increased in the other thee groups ( P ＜ 0.05). Compared with group B, the Bcl-2 expression was significantly down-regulated, the Bax expression was up-regulated and the ratio of Bcl-2/Bax was decreased in group C ( P ＜0.05). The glutamate concentrations were significantly increased, the Bax expression was up-regulated and the ratio of Bcl-2/Bax was decreased in group D compared with group C ( P ＜ 0.05 ). Conclusion The earlier cooling is performed, the better the cerebral protective effect is during global cerebral I/R in rats.

Objective The aim of the study was to observe the features of nail fold microcirculation in systemic sclerosis (SSc) patients and to compare these findings in SSc patients with patients with other connective tissue diseases.Methods Forty patients with SSc and thirty-seven patients with other connective tissue diseases were included in the study and all the patients reported symptoms of Raynaud's phenomenon in the hands were also included.Nail fold capillaroscopy (NFC) was performed and the abnormality of nail fold microcirculation between the two groups were compared.The relations between nail fold capillaroscopic findings and clinicolaboratory parameters in SSc patients were analyzed.Statistical analysis were carried out by t-test and Chi-square.Results The loss of capillaries and dilated and giant capillaries and hemorrhage as well as neoangiogenesis were hallmarks of the scleroderma capillary findings,which could be detected by nail fold capillaroscopy.The abnormalities of nail fold microcirculation in SSc patients were more severe and more specific than those in other connective tissue disease patients.The total scores of nail fold capillaroscopy test were obviously higher in SSc patients with lung or esophagus involvement than those patients without these organ involvement,meanwhile,the total scores of nail fold capillaroscopic findiugs were elevated in SSc patients with anti-Scl70 antibody than those with negative group.Conclusion The nail fold capillaries of patients with SSc have specific abnormalities,and nail fold capill-aroscopy could distinguish between SSc and other connective tissue diseases,therefore it could be used as a promising tool for early detection of patients who may have the potential to develop scleroderma and it is also helpful in assessing disease severity.

Objective To investigate the influence of proline-rich tyrosine kinase 2 (Pyk2)gene RNA interference on proliferation,invasion and migration of Hep3B hepatocellular carcinoma cells.Methods The Pyk2 gene RNA interference vector was transfected in Hep3B hepatocellular carcinoma cells by lipofectamine. The Hep3B cells divided into three groups:siRNA group (the vector with Pyk2 RNAi gene was transfected), negative control group (the vector without Pyk2 RNAi gene was transfected),and blank control group (no vectors was transfected).Pyk2 mRNA and protein were detected using reverse transcription reverse transcription-poly-merase chain reaction (RT-PCR)and Western blotting.The biological behavior including cell proliferation,inva-sion and migration were detected by 3-(4,5-dimethyl-2-thiazoly)-2,5-diphenyl-2H-tetrazolium bromide (MTT), transwell and wound healing assay,respectively.Results The expression of Pyk2 mRNA of Hep3B cell line in siRNA group (0.1 6 ±0.03)was significantly decreased than those in negative group (0.74 ±0.1 3)and blank control group (0.77 ±0.1 6),with statistically significant differences (t=51 .46,P=0.000;t=53.21 ,P=0.000).The expression of Pyk2 protein of Hep3B cell line in siRNA group (0.24 ±0.06)was significantly decreased than those in negative group (0.83 ±0.05)and blank control group (0.91 ±0.06),with statisti-cally significant differences (t=57.29,P=0.000;t=68.53,P=0.000).The cell proliferation inhibition rate at 48 hours in siRNA group (26.1 7%±0.28%)was significantly raised than those in negative group (9.28%± 0.22%)and blank control group (6.47%±0.31%),with statistically significant differences (t=31 .45,P=0.004;t=34.64,P=0.002).The number of transmembrane cells in siRNA group (32.5 ±8.5)/1 0 HP was significantly declined than those in negative group (98.4 ±1 2.3 )/1 0 HP and blank control group (1 1 2.6 ± 1 1 .3)/1 0 HP,with statistically significant differences (t=95.64,P=0.000;t=1 05.1 7,P=0.000).The wound healing assay in siRNA group (28.1 7%±1 .46%)was significantly lower than those in negative group (77.38%±2.24%)and blank control group (79.41%±3.1 7%),with statistically significant (t=85.86,P=0.000;t=89.37,P=0.000).Conclusion Pyk2 gene involves the proliferation,invasion and migration of Hep3B cells,which has close correction with development and metastasis of hepatocellular carcinoma.Pyk2 gene is very helpful to become a molecular target for the diagnosis and treatment of hepatocellular carcinoma.

OBJECTIVE:To establish the quality standard for Tongmai pill. METHODS:TLC was used for the qualitative iden-tification of Salvia miltiorrhiza,Angelica sinensis and Carthamus tinctorius. HPLC was used for the content determination of feru-lic acid. The column was Diamonsil C18 with mobile phase of acetonitrile-0.1% aqueous phosphoric acid (17∶83,V/V) at a flow rate of 1 ml/min,the detection wavelength was 316 nm,the column temperature was 30 ℃,and the injection volume was 10 μl.. RESULTS:The TLC of S. miltiorrhiza,A. sinensis and C. tinctorius showed clear spots and good separation. The linear range of ferulic acid was 8-80 μg/ml(r=0.999 8);RSDs of precision,stability and reproducibility tests were lower than 2%;recovery was 97.2%-99.6%(RSD=1.2%,n=6). CONCLUSIONS:The standard can be used for the quality control of Tongmai pill.

Objective To describe the application of Vapocoolant spray for alleviating pain during intravenous cannulation in foreign countries. Methods We interviewed literature related with Vapocoolant spray for alleviating pain during intravenous cannulation. The databases included China National Knowledge Infrastructure, and Wanfang Data, Web of Science, PubMed. Results It is still controversial that Vapocoolant spray could alleviating pain during intravenous cannulation and there are few studies about Vapocoolant spray in China. Conclusions More high quality randomized controlled studies are needed to provide evidence for the controversy. Provide reference for medical staff with alleviate pain during intravenous cannulation.

Chinese medicine has been used for centuries to treat a range of health conditions.This history has produced a wealth of classical literature,case studies and clinical research data detailing its use and effectiveness.However,high-quality and conclusive evidence that meets modern requirements for clinical decision support is lacking.This evidence gap limits the integration of Chinese medicine with contemporary medicine,which in turn limits global access and acceptance of Chinese medicine as a form of safe and effective health care.Over the past 20 years,researchers and organisations around the world,including the World Health Organization(WHO)and United Nations,have worked to support the integration of traditional medicines,such as Chinese medicine,with conventional medicines to improve global health care.This paper provides an overview of Chinese medicine studies published in the top four general medical journals(BMJ,JAMA,Lancet and New England Journal of Medicine)from February 2005 to February 2024 in the past 20 years to highlight the progress in the development of this evidence base.It also highlights key actions taken to promote evidence-based clinical Chinese medicine,including product and practitioner regulation,formalising education standards,and international collaborations.Research conducted at the China-Australia International Research Centre for Chinese Medicine demonstrates the benefits of such a collaboration.Through development of its unique and inclusive'whole-evidence'approach,plus clinical studies and systematic reviews,the Centre has significantly contributed to the evidence base for clinical Chinese medicine.In addition,its high-impact papers and groundbreaking monographs have been cited in international conventional medicine guidelines.While progress has certainly been made during the past 20 years to build a stronger evidence base for clinical Chinese medicine,there is still a considerable gap that limits its integration with conventional medicine.Future funding and research are needed to continue this work and achieve to safe,effective and accessible traditional medicine as part of the WHO's Universal Health Coverage strategy.

Various forms of complementary and alternative medicine are used in psoriasis. Among these, herbal medicines are frequently used as systemic and/or topical interventions either as a replacement for or in conjunction with conventional methods. The benefit of such use is unclear. This review is to provide an up-to-date review and discussion of the clinical evidence for the main kinds of herbal therapies for psoriasis. Searches of the biomedical databases PubMed (including MEDLINE), EMBASE and CINAHL were conducted in December 2011 which identified 32 clinical studies, all published in English. Twenty of these primarily tested topical herbal medicines and were thus excluded. The 12 studies that evaluated systemic use of herbal medicines were included in the review. Four were case series studies and the other 8 were controlled trials. In terms of interventions, 4 studies tested the systemic use of plant oils combined with marine oils and 8 studies tested multi-ingredient herbal formulations. The clinical evidence for plant and animal derived fatty acids is inconclusive and any benefit appears to be small. For the multi-herb formulations, benefits of oral herbal medicines were shown in several studies, however, a number of these studies are not controlled trials, a diversity of interventions are tested and there are methodological issues in the controlled studies. In conclusion, there is promising evidence in a number of the studies of multi-herb formulations. However, well-designed, adequately powered studies with proper control interventions are needed to further determine the benefits of these formulations. In addition, syndrome differentiation should be incorporated into trial design to ensure effective translation of findings from these studies into Chinese medicine clinical practice.
Administration, Oral ; Clinical Trials as Topic ; Drugs, Chinese Herbal ; administration & dosage ; therapeutic use ; Humans ; Plant Oils ; therapeutic use ; Psoriasis ; drug therapy

Objective:To compare the differences of postoperative clinical target volume of internal mammary lymph node (CTV ImlN) by different delineation methods, and to explore the reasonable method of CTV ImlN delineation after internal mammary lymph node dissection (ImlND). Methods:A total of 20 breast cancer patients who had undergone modified radical mastectomy (MRM) with ImlND on the affected side and had complete preoperative and postoperative CT images were selected. The CTV (CTV pr-I, CTV pr-a) of both sides of ImlN were delineated on preoperative CT images according to RTOG guideline. On postoperative CT images, three different methods including deformation image registration (DIR) method, visual contrast method and precise measurement method, were employed to delineate the postoperative CTV ImlN of the affected side. The targets were named as CTV DIR, CTV V and CTV M, respectively. The central displacement, target volume, degree of inclusion (DI) and conformity index (CI) of CTV pr-a, CTV V, CTV M and CTV DIR were compared. Results:The central displacement of CTV V, CTV M and CTV DIR from CTV pr-a was 2.17 cm, 1.44 cm and 1.25 cm, respectively. The target volume of CTV pr-a, CTV pr-I, CTV V, CTV M and CTV DIR was 2.10 cm 3, 2.17 cm 3, 2.04 cm 3, 1.88 cm 3 and 2.07 cm 3 respectively. There was no significant difference in the target volume (all P>0.05). The CI values of CTV V-CTV pr-a and CTV M-CTV pr-a were both 0.16, and that of CTV DIR-CTV pr-a was 0.43. The CI value of CTV DIR was significantly higher than those of CTV V and CTV M (both P<0.01). The DI values of CTV V-CTV pr-a, CTV M-CTV pr-a and CTV DIR-CTV pr-a were 0.26, 0.24 and 0.58, respectively. The DI value of CTV DIR was significantly higher than those of CTV V and CTV M (both P<0.01). Conclusions:It is difficult to accurately delineate the CTV ImlN for patients after ImlND. However, the spatial position fitness of the target region delineated by DIR method is better than those by visual contrast and precise measurement methods.

Objective This study aims to explore and evaluate internal fixation with double titanium plates in mandibular condylar fracture. Methods Twenty-eight patients with mandibular condylar fracture were treated by rigid internal fixation with double titanium plates. The effects of the treatment on occlusion, mouth opening, and titanium plate rupture were evaluated by pantomograms or 3D CT. Results Good retention was observed in all 28 patients with mandibular condylar fracture. Occlusion and mouth opening returned to normal. None of the patients experienced titanium plate rupture or fracture displacement. Two patients suffered from mild facial nerve injury but recovered within 3 months after neurotrophic drug treatment. Two patients suffered from temporo-mandibular joint disease but improved after plate removal. Conclusion Rigid internal fixation with double titanium plates is an ideal treatment for mandibular condylar fracture.