Clinicopathological features and prognosis of clear cell papillary renal cell carcinoma / 现代泌尿外科杂志

【Objective】 To investigate the clinicopathological features and prognosis of clear cell papillary renal cell carcinoma (CCPRCC). 【Methods】 The clinicopathological and follow-up data of 40 CCPRCC patients treated during Jun. 2011 and Oct.2021 were retrospectively analyzed. The prognosis was compared with that of 40 cases of clear cell renal cell carcinoma (ccRCC) and 19 cases of papillary renal cell carcinoma (PRCC) treated in the same period. Survival analysis was performed by Log-rank test and Kaplan-Meier survival curves were plotted. 【Results】 Among the 40 patients, 28 were male and 12 were female, aged 31-84 years; 38 cases had unilateral and 2 cases had bilateral tumors; 3 cases had multifocal lesions. All patients received surgery. The maximum diameter of the masses ranged from 3.0 to 95.0 mm, with an average of (27.6±18.1) mm. Pathological grade was Fuhrman 1-2 in all cases. Immunohistochemical tests were positive for CK7 and CA-IX. During the follow-up of 5-129 (average 56) months, 1 case died after bone metastasis, 2 had ipsilateral recurrence, and 1 developed primary esophageal cancer. CCPRCC patients had a significantly better prognosis than CCRCC (P<0.001) and PRCC (P=0.005) patients, while there was no significant difference in the prognosis between CCRCC and PRCC patients (P=0.93). 【Conclusions】 CCPRCC has low malignancy. The diagnosis relies on characteristic pathological and immunohistochemical features. Surgery is an effective treatment. CCPRCC has a better overall prognosis than CCRCC and PRCC.

Risk factors and their warning value for the occurrence of sepsis in patients with severe multiple trauma / 中华创伤杂志

Objective:To investigate the risk factors and their warning value for the occurrence of sepsis in patients with severe multiple trauma.Methods:A retrospective cohort study was conducted to analyze the clinical data of 92 patients with severe multiple trauma admitted to Yuyao People′s Hospital from July 2019 to October 2021. There were 71 males and 21 females, with the age range of 36-55 years [(45.5±13.6)years]. The injury severity score (ISS) was 20-29 points [(25.3±6.4)points]. The patients were divided into sepsis group ( n=32) and non-sepsis group ( n=60) according to whether sepsis occurred during hospitalization. Data were recorded for the two groups, including gender, age, basic diseases, cause of injury, number of injury sites, ISS, post-injury complications, and levels of aryl hydrocarbon receptor (AHR), C-reactive protein (CRP) and procalcitonin (PCT) at 1, 3 and 5 days after injury. The above data were analyzed to identify their correlation with the occurrence of sepsis in patients with severe multiple trauma by univariate analysis. The independent risk factors for sepsis in patients with severe multiple trauma were determined by multivariate Logistic regression analysis. The warning value of the single or combined risk factors for the occurrence of sepsis in patients with severe multiple trauma was evaluated by the receiver operating characteristic (ROC) curve and area under the curve (AUC). Results:By univariate analysis, it was demonstrated that the occurrence of sepsis was correlated with ISS, level of AHR at day 1 after injury, level of CRP at day 3 after injury and level of PCT at day 3 after injury ( P<0.05 or 0.01), but not with age, sex, basic diseases, level of AHR at 3, 5 days after injury, level of PCT at 1, 5 days after injury and level of CRP at 1, 5 days after injury (all P>0.05). By multivariate Logistic regression analysis, higher ISS ( OR=1.12, 95% CI 1.01, 1.24, P<0.05), level of AHR at day 1 after injury ( OR=1.30, 95% CI 1.10, 1.52, P<0.01) and level of PCT at day 3 after injury ( OR=1.81, 95% CI 1.08, 3.03, P<0.05) were found to be strongly correlated with the occurrence of sepsis. ROC curve analysis showed that higher ISS (AUC=0.69, 95% CI 0.57, 0.76) and level of AHR at day 1 after injury (AUC=0.79, 95% CI 0.68, 0.90) had warning value for the occurrence of sepsis, and the warning efficiency of combined panel was much better (AUC=0.86, 95% CI 0.77, 0.95). Conclusions:Higher ISS, level of AHR at day 1 after injury and level of PCT at day 3 after injury are independent risk factors for the occurrence of sepsis in patients with severe multiple trauma. ISS, AHR and combination of both exhibit good warning value for the occurrence of sepsis in patients with severe multiple trauma.

Expert consensus guidelines on clinical use of Xiyanping injection for acute infectious diseases

Xiyanping injection, a traditional Chinese medicine injection made of andrographolide sulfonate, consisting of well-defined ingredients with antiviral, antibacterial, anti-inflammatory and antipyretic efficacy, has been widely used for treating infectious diseases of respiratory and digestive systems. However, its wide applications may easily lead to unreasonable clinical medication. In order to guide the precise clinical application and rational use of Xiyanping injection, experts in related fields conducted systematically literature review, evaluated and deliberated the application of Xiyanping injection in treating acute infectious diseases using evidence-based medicine method, and jointly drafted the consensus to summarize types of acute infectious diseases in children and adults that can be treated with Xiyanping injection, and recommend the intervention time, usage and dosage, course of treatment and combined medication of the injection. Besides, the consensus elucidates the safety, precautions and contraindications of the injection, so as to provide guidance for clinical use.

Chinese expert consensus on early prevention and intervention of sepsis

Sepsis is currently a major problem and challenge facing the medical community. With rapid development and progress of modern medicine, researchers have put more and more attention on sepsis; meanwhile, the morbidity and mortality of sepsis remains high despite great efforts from experts in various fields. According to updated guidelines, sepsis is defined as life-threatening organ dysfunction caused by a dysregulated host response to infection. Infection is the initial step of sepsis progression, and development from infection to sepsis is a complex pathophysiological process, including pathogen invasion, cytokine release, capillary leakage, microcirculation dysfunction, etc. which finally leads to organ metabolic disorders and functional failure. According to the latest recommended international guidelines of Sepsis 3.0, the presence of infection and SOFA score ≥ 2 are considered as the diagnostic criteria for sepsis, and the 'rescue' measures mainly focus on reversal of organ dysfunction. However, despite nearly two decades of efforts, the 'Save Sepsis Campaign' has not achieved satisfactory results. Emergency medicine is the frontier subject of acute and severe illness which treats patients with acute infections at the earliest. If at this stage, physicians can predict the possibility of sepsis progression from demographic characteristics, localize the pathogen and infection, detect the inflammatory storms by tests of cytokines and evaluate the severity of the infection with more effective clinical scoring system, and then take effective measures to prevent infection from developing into sepsis in high-risk patients, the morbidity and mortality of sepsis in patients with acute infection will be greatly reduced. Based on this situation, Chinese emergency medicine experts proposed the concept of 'preventing and blocking' sepsis, and launched the nationwide 'Preventing Sepsis Campaign in China (PSCC)' nationwide. The main concept is summarized as 'three early and two reduces' which includes early detection, early diagnosis and early intervention during the 'pre-symptomatic' and 'peri-septic' stage in order to reduce the incidence of sepsis and it proposed a new approach for diagnosis and treatment of acute severe infection. This consensus is jointly advocated, discussed and written by four academic associations in the field of emergency medicine and five scholarly publishing organizations. More than 40 experts from fields of emergency medicine, critical care medicine, infectious diseases, pharmacy and laboratory medicine have participated in several rounds of deliberation and finally reached consensus on the criteria of identifying patients with acute infection, taking anti-infective treatments, screening of high-risk patients with sepsis, detection and treatment of inflammatory storm, protection of vascular endothelial cells and the regulation of coagulation function, as well as strategies of liquid support and organ function protection etc. The consensus summarizes the commonly used clinical diagnosis criteria and treatment measures of sepsis both in Western medicine and traditional Chinese medicine for clinicians in order to provide evidence for the diagnosis and treatment of the disease.

A case report of BCL11B mutation induced neurodevelopmental disorder and literature review / 中华儿科杂志

Objective@#To analyze the clinical , immunological and genetic features of a child with BCL11B mutation induced neurodevelopmental disorder.@*Methods@#The clinical data and genetic test of a child with BCL11B mutation hospitalized in the Department of Rheumatology and Immunology in Children′s Hospital of Chongqing Medical University in December 2018 were extracted and analyzed. The literature was searched with "BCL11B mutation" and "immunodeficiency 49" as key words in Chinese databases and Pubmed until January 2019 was reviewed.@*Results@#A male patient aged 3 years and 11 months with facial dysmorphisms and delayed language and motor development was admitted due to neurodevelopmental retardation over two years. Laboratory tests showed normal human immunoglobulin (IgG 12.90 g/L, IgA 1.02 g/L, IgM 1.15 g/L, IgE 532 000 U/L), Trec (228) and proliferation of T and B cells. The lymphocyte subsets revealeda reduced percentage of B cells (0.108) but normal absolute numbers (0.574×10-3/L), and an increased percentage (0.828) as well as absolute numbers (4.415×10-3/L) of T cells. A heterozygous BCL11B mutation was detected by sanger sequencing, showing a de novo frameshift mutation c.1887_c.1893delCGGCGGG in exon 4. Two papers were found which were all in English, with total of 14 patients(13 patients with complete information). Thirteen mutations were reposed, including 7 frameshift, 2 nonsense, 2 missense, and 2 chromosomal rearrangements; Thirteen patients had heterozygous mutations. All patients had delayed language and motor development and facial dysplasia which were mainly hypertelorism, thin eyebrows and small palpebral fissures. Some patients had dental anomalies, ametropia and allergy, and a few were combined with immune impairment, but without overt signs of immunodeficiency. Only one patient had multisystem anomalies and profound immune deficiency.@*Conclusions@#BCL11B is essential for development of the nervous and the immune system. In this study, the de novo mutation of BCL11B gene resulted in neurodevelopmental and immunological disorders.

Clinical phenotype and immunological features of a patient with A20 haploinsufficiency / 中华儿科杂志

Objective@#To explore the clinical phenotype, immunological features, pathogenesis and gene variation of a case with A20 haploinsufficiency (HA20).@*Methods@#A patient diagnosed with tumor necrosis factor α-induced protein 3 (TNFAIP3) mutated HA20 was admitted into Shenzhen Children′s Hospital in May,2019.The clinical data was analyzed. Flow cytometry was used to detect the patient′s peripheral blood lymphocyte subsets, and also, the percentage of follicular helper T cell (TFH) cells in the patient and thirteen healthy controls. After the construction of empty vector, wild-type and mutant plasmid vectors, a wild-type or mutant overexpression system of the TNFAIP3 gene was established in 293T cells and Hela cells. Then, the expression level of A20 in 293T cells and the expression of inhibitor K binding α (IKBα) in green fluorescent protein (GFP)+Hela cells before and after tumor necrosis factor α (TNF-α) stimulation were measured, to verify the pathogenicity of this variation.@*Results@#A 5 years and 11 months old boy, presented with recurrent oral ulcer, abdominal pain, joint swelling and arthralgia. Oral ulcer, chronic skin rashes, knee joint swelling were observed. The levels of inflammatory markers were increased. Colonoscopy showed congestion of mucosa and multiple ulcers in terminal ileum and ileocecus. The absolute number of naive B cells was 124×106 cells/L (reference range 147×106-431×106 cells/L), accounting for 0.430 of the total B cells (reference range 0.484-0.758). Compared to healthy controls (0.016-0.071), the percentage of TFH cells in CD4+T cells was much lower (0.008).A heterozygous mutation of TNFAIP3 gene (c.909_913 del, p.L303fs) was identified by genetic analysis. In vitro study showed that truncated A20 protein was expressed in TNFAIP3 mutant overexpressed 293T cells, which verified the pathogenicity of this variation. Besides, after TNF-α stimulation, the degradation rate of IkBα protein in mutant overexpressed Hela cells (35%) was between the other two groups (15% in the wild-type group and 57% in the non-loaded group).@*Conclusions@#This case with HA20 due to a de novo TNFAIP3 gene mutation presents with early onset Behcet-like autoinflammatory syndrome. This variation leads to expression of truncated A20 protein, enhanced degradation of IkBα, and further activation of nuclear factor κB signaling pathway.

The battle between 2019-nCoV and host immune system: implications for therapy / 中华微生物学和免疫学杂志

Currently, there are many reports on the clinical phenotypes and epidemiological data of COVID-19. Understanding the underlying pathogenesis is important for disease management. With the implications from studies on severe acute respiratory syndrome (SARS), this review mainly discussed the pathogenesis of COVID-19 and focused on the host immune response to viruses. Direct damage to tissues and organs and excessive inflammatory responses induced by 2019-nCoV are associated with the pathological process and disease progression. Viral load and high inflammatory response are related to disease severity. Peripheral lymphocytes are closely associated with the acute lung injury, and can be used to predict disease severity. 2019-nCoV-specific antibody and T cell immune response can be detected in patients with 2019-nCoV infection. Given the two sides of inflammation and hormone therapy, steroid as a broad-spectrum immunosuppressant needs to be used with caution. Biologics targeting IL-6 and convalescent plasma therapy are promising treatment for COVID-19. Further research on viral features, pathogenesis, immunopathogenesis and specific immune defense would be conducive to better understanding of COVID-19 and achieving precision medicine.

Research progress of autoimmune lymphoproliferative syndrome / 中华实用儿科临床杂志

Autoimmune lymphoproliferative syndrome (ALPS) is a human genetic disorder of lymphocyte apoptosis resulting in an accumulation of lymphocytes and childhood onset chronic lymphadenopathy, splenomegaly, multilineage cytopenias, and an increased risk of B-cell lymphoma.Since then, with more than 500 patients with ALPS diagnosed worldwide.In this paper, it is hoped that harmonizing the diagnosis and classification of ALPS will foster be-tter understanding of the pathogenesis of ALPS.

Iodine nutritional status of pregnant women in Jinan City, 2018 / 中华地方病学杂志

Objective To investigate the iodine nutritional status of pregnant women in Jinan City,which may provide a basis for scientific supplementation of iodine.Methods Ten counties (districts) in Jinan City were divided into five areas as east,west,south,north and middle in 2018.At least one township (street) was selected from each area,and 20 pregnant women were selected from each township (street).Personal information was collected through standardized questionnaires,and household salt samples and urine samples of pregnant women were collected.Salt iodine and urinary iodine were detected by the "General Test Method in Salt IndustryDetermination of Iodine" (GB/T 13025.7-2012) and "Method for Determination of Iodine in Urine by As3+-Ce4+ Catalytic Spectrophotometry" (WS/T 107-2006),respectively.Results A total of 1 169 pregnant women's household salt samples were collected,the median of salt iodine was 22.6 mg/kg.The coverage rate of iodized salt was 90.5％ (1 058/1 169),the qualified rate of iodized salt was 75.8％ (802/1 058),and the consumption rate of qualified iodized salt was 68.6％ (802/1 169).A total of 1 169 urine samples of pregnant women were tested,the median of urinary iodine was 141.9 μg/L,indicating the urinary iodine of pregnant women was insufficient (＜ 150 μg/L).Conclusions The iodine nutrition of pregnant women is slightly inadequate in Jinan City.It is necessary to strengthen the measures in prevention and control of iodine deficiency,and promote the health education measures in pregnant women to improve their iodine deficiency status.

Study on Protective Effect of Total Flavonoids from the Leaves of Mongolian Medicine Choerospondias axillaris on Myocar- dial Ischemia Reperfusion Injury Model Rats / 中国药房

OBJECTIVE： To investigate the protective effect of total flavonoids from the leaves of Choerospondias axillaris （TFLC） on myocardial ischemia reperfusion injury （MIRI） model rats. METHODS： Male SD rats were randomly divided into sham operation group， model group， positive control group （verapamil， 0.02 g/kg）， TFLC low-dose and high-dose groups （0.1， 0.4 g/kg）， with 10 rats in each group. Administration groups were intragastrically given relevant medicine （2 mL/100 g）； sham operation group and model group were given constant volume of normal saline intragastrically， once a day， for consecutive 7 d. After last medication， MIRI model was induced by modified ligation method. The times and duration of ventricular tachycardia （VT） and ventricular fibrillation （VF） in rats were recorded with biological function experiment system during reperfusion period.The activity of CK and contents of TNF-α， IL-6， NF-κB and NO in serum were determined by ELISA double antibody clip art assay. The morphological characteristics of myocardial tissue was observed by HE staining. The myocardial infarction scope （i.e. the ratio of myocardial tissue mass to ventricular mass） was measured by TTC method. RESULTS： Compared with sham operation group， the times and duration of VT and VF were increased or prolonged significantly in model group； CK activity， serum contents of TNF-α， IL-6 and NF-κB were enhanced or increased significantly， while NO content was decreased significantly （P＜0.05 or P＜0.01）. Obvious myocardial infarction focus， serious cell structure damage， disorderly muscle fibers arrangement， cell nucleus pyknosis and accompanied inflammatory cell infiltration were all observed in cardiac tissue； the mass of infarcted myocardial tissue and ventricular as well as the scope of myocardial infarction increased significantly （P＜0.01）. Compared with model group， the times and duration of VT and VF were decreased or shortened significantly in administration groups； CK activity， serum contents of TNF-α， IL-6， NF-κB were decreased significantly， while NO content was increased significantly （P＜0.05 or P＜0.01）. The above symptoms of myocardial injury were improved；the mass of infarcted myocardial tissue and ventricular as well as the scope of myocardial infarction was significantly reduced （P＜0.05 or P＜0.01）. CONCLUSIONS： TFLC can relieve MIRI-induced ischemic arrhythmia and myocardial damage， reduce the release of inflammatory factors， promote the recovery of myocardial and endothelial cell function， reduce the scope of myocardial infarction and has a certain protective effect.

Flurbiprofen axetil: analgesic effect and adverse reaction

Flurbiprofen axetil is a targeted analgesic and non steroidal analgesic with lipid microspheres as drug carrier. It can selectively accumulate in surgical incision and reduce the allodynia a caused by surgical trauma. In this paper, the experimental subjects were divided into three groups to analyze the difference in the analgesic effect of different doses of flurbiprofen axetil for postoperative analgesia. The patients in group A, B and C were treated with flurbiprofen axetil injection 100, 150, 200mg, respectively. The results showed that MAP, HR, static and dynamic VAS scores and postoperative pain incidence in group B and group C were lower than those in group A, and B group had better analgesic effect and lower incidence of adverse reactions. In the future, we should continue to explore the correlation between the effect of flurbiprofen axetil for postoperative analgesia on coagulation function and the range of dose safety

De novo NFκB2 gene mutation associated common variable immunodeficiency / 中华儿科杂志

Objective@#To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) .@*Methods@#A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. The Chinese and PUBMED databases were searched with the key words "NFκB2" and "immune deficiency" and related literatures were reviewed.@*Results@#The patient had 4 episodes of pneumonias and one otitis media since the age of 6 months. The serum immunoglobulin levels were IgG 2.73 g/L, IgA<0.07 g/L, and IgM 0.12 g/L. The percentage of peripheral lymphocyte subsets demonstrated increased CD3+T lymphocyte (81.8%), increased CD4+ naïve T cell (39.1%), normal B cell (14.1%), low switched memory B and plasmablast B (respectively 0.1% and 0), and lightly diminished natural killer(NK) cell (4.13%). Within the peripheral CD4+T cells, the percentage of regulatory T cells (1.49% (control 4.08%)), T follicular helper (3.66% (control 11.0%)), and T helper 17 (9.65% (control 15.7%)) were decreased, while the percentage of T helper 2 (60.9% (control 46.5%)) was elevated. T lymphocyte proliferative response and T cell receptor repertoire diversity were normal. NK-cell cytotoxic activity was impaired. The whole-exome sequencing harbored a de novo heterozygous nonsense mutation in exon 22 (c.2557C>T; p. Arg853X) in the C-terminus of NF-κB2. The western blotting confirmed the decreased expression of NF-κB2 (p52) protein. The patient received intravenous immunoglobulin infusion monthly (400-600 mg/kg), followed by improvement of pulmonary infection. After searching the databases, a total of 28 cases (1 Chinese and 27 non-Chinese) were identified. There were 12 cases of nonsense mutation (5 were gain-of-function mutation), and 8 cases of missense and frameshift mutations, respectively. The main clinical manifestation was respiratory infection, followed by autoimmune diseases such as alopecia and trachyonychia. Fifteen cases developed adrenocorticotrophic hormone (ACTH) deficiency.@*Conclusions@#NF-κB2 signaling pathway played an important role in T and B lymphocyte differentiation, and NK-cell cytotoxic activity. NFκB2 mutation should be considered in cases with recurrent infections, hypogammaglobulinemia, and decreased memory B cells and plasma cells, especially when combined with ACTH deficiency.

Clinical and genetic analysis of 11 cases with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome / 中华儿科杂志

Objective@#To investigate the clinical, inflammatory and genetic characteristics of cases with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.@*Methods@#Clinical and inflammatory manifestations and gene sequencing of 11 cases with PFAPA were retrospectively analyzed. Inflammatory markers including white blood cell (WBC) , C reactive protein (CRP) , and serum amyloid A (SAA) were compared between febrile period and intermittent period. Fifteen normal children were taken as healthy controls. The levels of plasma inflammatory cytokines including interleukin(IL)1β, IL-6, IL-17, tumor necrosis factor(TNF)-α, interferon (IFN)-γ, and granulocyte-colony stimulating factor(G-CSF) were compared between febrile period and intermittent period with paired-sample t test, and compared between febrile cases and healthy controls with independent t test.@*Results@#A total of 11 cases (7 females and 4 males) were included. The median onset age was 24 (3-60) months, and the median age of diagnosis was 69 (11-151) months. The median febrile duration was 4 (1-8) days, and the intermittent period lasted 1 to 8 weeks. All the cases had periodic fever and pharyngitis/tonsillitis, 7 of whom had combined lymphadenitis, and 5 of whom suffered from oral ulcers. Compared to intermittent-period-status,WBC ((14.7±4.1) ×109/L vs. (8.4±1.9) ×109/L, P<0.05), CRP((24.2±21.1) vs. (3.3±2.1)mg/L, P<0.05), SAA ((136.4±47.7) vs. (7.1±1.1)mg/L, P<0.05) were significantly elevated in febrile period. Compared to intermittent-period-status and healthy controls, plasma levels of IL-6 ((38±10) vs. (8±4) and (8±5)ng/L, t=6.514 and 6.830 respectively, P<0.05), IFN-γ ((132±43) vs.(49±21) and (53±21)ng/L, t=4.069 and 4.276 respectively, P<0.05), G-CSF ((403±12) vs. (175±90) and (121±49)ng/L, t=4.219 and 9.047 respectively, P<0.05) were significantly higher in febrile period, while no differences were found in levels of IL-1β, IL-17 and TNF-α. Gene sequencing found MEFV gene heterozygous variation in 8 cases.@*Conclusions@#PFAPA often manifests as periodic fever, pharyngitis, tonsillitis, aphthous stomatitis and adenitis. Gene sequencing analysis, detection of inflammation markers and cytokines could help with the diagnose of this disease.

Clinical study of CT-guided radiofrequency ablation plus intratumoral chemotherapy for patients with stage Ⅲ non-small cell lung cancer / 国际肿瘤学杂志

Objective To explore the clinical efficacy and safety of CT-guided radiofrequency ablation plus intratumoral chemotherapy in patients with stage Ⅲ non-small cell lung cancer (NSCLC).Methods From February 2014 to May 2015,38 patients with stage Ⅲ NSCLC who did not receive systematic chemotherapy due to advanced age were selected in our hospital.The patients were divided into experimental group (n =19) and control group (n =19) by stratified random method.The patients in experimental group received CT-guided radiofrequency ablation plus Lobaplatin intratumoral chemotherapy,and the patients in control group only received CT-guided radiofrequency ablation.The adverse events,Karnofsky performance system (KPS) scores,1-year overall survival rates and short-term curative effects of patients in the two groups were observed.Results All 38 patients completed the course of therapy successfully.The 3-month response rates and 6-month response rates in experimental group and control group were 89.5％ vs.63.2％ and 78.9％ vs.52.6％,with no significant differences (P =0.124;P =0.170).The KPS score improvement rates were 42.1％ (8/19) and 31.6％ (6/19) in experimental group and control group,the KPS score stable rates were 52.6％ (10/19) and 52.6％ (10/19) in the two groups,and the KPS score deterioration rates were 5.3％ (1/19) and 15.8％ (3/19) in the two groups,with no significant differences (P =0.737;P =1.000;P =0.290).The 1-year survival rate of patients in experimental group was higher than that in control group (89.5％ vs.73.7％),with a significant difference (x2 =5.573,P =0.034).The rates of less than or equal to grade 3 pain (31.6％ vs.42.1％),low fever (21.1％ vs.26.3％),aerothorax (31.6％ vs.42.1％),gastrointestinal reaction (15.8％ vs.31.6％) and bone marrow depression (5.3％ vs.15.8％) in experimental group were lower than those in control group,but the differences were not statistically significant (P =0.501;P =0.703;P =0.501;P =0.252;P =0.290).Conclusion CT-gnided radiofrequency ablation plus intratumoral chemotherapy for stage Ⅲ NSCLC can improve short-term survival rate,and it does not increase the adverse reaction.While,the long-term overall survival rate has yet to be followed up.

Application of information technology in clinical teaching quality control / 中华医学教育探索杂志

Under the guidance of the teaching goal management philosophy, process and result quality of teaching task and its implementation are transformed into the goal scores of teachers, clinical departments and teaching research departments. Teaching administration departments regularly conduct tar-geted work inspection, collect relevant data for statistical analysis, feedback and request the corresponding improvement. In order to improve the efficiency of clinical teaching management and realize the seamless connection between clinical teaching management and clinical teaching, by combing the associations of clinical teaching, learning, management, information technology is used to establish the corresponding teaching work module. Meanwhile, by data analysis of clinical first-line teaching tasks and implementation process, the real-time information input and the summary statistics feedback output system of teaching, learning and management is established,so that the synchronization of teaching and teaching quality moni-toring can be realized, teaching documents are normalized and paperless office can be achieved.

The relationship between lumbar disc degeneration of lumbar spinal stenosis and the dural sac cross-sec-tional area by MRI measurement / 实用医学杂志

Objective To explore the relationship between lumbar disc degeneration (LDD) of lumbar spinal stenosis(LSS)and the dural sac cross-sectional area(DSCA)by MRI measurement. Methods 91 patients with central degenerative LSS were randomly selected and 91 age-and sex-matched people without LSS were select-ed as a control group. LDD was classified into five grades by MRI detection according to the method proposed by Pfirrmann and DSCA were measured. Results LDD was not associated with age in LSS. The proportion of severe degenerated disc in lower lumbar levels were higher than that of L2/3 in the two groups;DSCA in severe degenerat-ed disc group was significantly smaller than that in light degenerated group only in L2/3 and L3/4 in LSS. There were no statistical differences in every lumbar level in the control group. Conclusions LDD in L4/5 and L5/S1 of LSS is more severe than that of the normal people. DSCA and LDD are positively correlated in L2/3 and L3/4,but not in L4/5 and L5/S1 for LSS.

Clinical and immunological analysis of the patient with autoimmunity due to germline STAT3 gain-of-function mutation / 中华儿科杂志

Objective@#To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3.@*Method@#A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children′s Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed.@*Result@#The patient was a four years old girl. The clinical manifestation consisted of autoimmune pancytopenia, lymphadenopathy and recurrent infections. Multiple exams showed that peripheral blood leukocyte count was (2.2-4.9)×109/L, red blood cell count was (2.09-5.75)×109/L, hemoglobin level was 64-165 g/L, platelet count was (52-138) ×109/L. Percentages of lymphocyte subsets showed that CD3+ T lymphocyte was 0.716 0 (CD4+ T lymphocyte was 0.326 0, CD8+ T lymphocyte was 0.323 0 and CD4- CD8-T TCRαβ+ lymphocyte was 0.029 0), CD19+ B lymphocyte was 0.235 0 (transitional B was 0.004 3), NK was 0.032 0. Percentages of CD4+ T lymphocyte release IL-4, IFN-γ, IL-17 and IL-21 were 0.014 9, 0.213, 0.024 0 and 0.021 0, respectively. Lymphocyte proliferation function and TCRVβ diversity were normal. The serum immunoglobulin levels were 16.4 g/L (IgG), 1.53 g/L (IgA), 3.99 g/L (IgM) and 3.20 kU/L (IgE). The patient carried a missense variant in the 21st exon of STAT3, c. 1974G>C, p.K658N, which was previously described as a gain-of-function mutation. The patient was treated with methylprednisolone and prednisone intermittently. There were significant improvements of hepatosplenomegaly, lymphadenopathy and pancytopenia. We searched internal database and literature for cases with gain-of-function mutations in STAT3. A total of 19 cases were identified, all were non-Chinese. Among 16 cases who had clinical data, age of onset of 11 patients was less than 5 years. 14 cases had autoimmune hemolytic anemia, autoimmune thrombocytopenia or autoimmune neutropenia. Twelve patients had lymphadenopathy while 11 had infections and 5 had endocrine abnormalities.@*Conclusion@#The patient with Primary immunodeficiency disease (PID) due to gain-of-function mutation in STAT3 gene often has early-onset autoimmune disorders, lymphadenopathy and recurrent infections. Since the routine immunological examination may be normal or slightly abnormal, comprehensive evaluation of immune function should be done. Genetic testing ultimately helps to confirm the diagnosis.

Clinical and immunological analysis of patients with activated phosphoinositide 3-kinase δ syndrome resulting from PIK3CD mutation / 中华儿科杂志

Objective@#To explore the clinical and immunological features, gene mutations, treatment and prognosis in patients with activated phosphoinositide 3-kinase δ syndrome (APDS) caused by PIK3CD gene heterozygous germline mutation.@*Method@#The data of clinical, immunological phenotype, treatment, and prognosis of 15 patients with APDS, who visited Children′s Hospital of Chongqing Medical University, Peking Union Medical College Hospital, and Shenzhen Children′s Hospital from June 2014 to November 2016, were collected and analyzed.@*Result@#Of the 15 patients, 11 were males, remaining 4 patients were females. The median age of disease onset was 1 year, and median age at diagnosis was 4 years and 4 months. All patients had the de novo heterozygous germline mutation in PIK3CD (c. 3061G>A, p. E1021K). The common initial symptoms were respiratory infections, including pneumonia (12 cases) , bronchiectasis (5 cases). Other common clinical manifestations were recurrent and chronic diarrhea (11 cases), Epstein-Barr virus (EBV) and/or cytomegalovirus (CMV) viremia (10 cases), hepatosplenomegaly (13 cases), and lymphadenopathy (10 cases). The main immunological features were increased IgM (11 cases), decreased IgG (6 cases), decreased numbers of CD4+ T cell (7 cases) especially naïve CD4+ T cell (9 cases), reduced numbers of B cells (11 cases) particularly naïve B cells (9 cases), increased numbers of transitional B cells (5 cases) and CD8+ terminally differentiated effector memory T cells (5 cases). After 1-29 months follow up, 13 of the 15 cases remain survived, of whom 5 cases received regular intravenous immunoglobulin (IVIG) therapy, with reduced frequency of infections and improved severity of infections; of whom 3 cases received oral rapamycin therapy at the dosage of 1 mg/ (m2·d) and with a decrease in nonneoplastic lymphoproliferation.@*Conclusion@#E1021K is a hotspot for mutation in the PIK3CD gene in patients with APDS. Regular IVIG can improve their quality of life. Targetel treatment with rapamycin could mitigate hepatosplenomegaly.

Two families of X-linked lymphoproliferative disease type 1 characterized by agammaglobulinemia / 中华儿科杂志

Objective@#To investigate the clinical and immunological laboratory features, mutations in SH2D1A gene and SAP protein expression in four children of two families with X-linked lymphoproliferative disease type 1(XLP-1).@*Method@#Four patients (Family A including Patient 1 and Patient 2, Family B including Patient 3 and Patient 4) and their maternal relatives were enrolled in this study. The clinical manifestation, EBV infection status and chest CT scan were analyzed. The absolute and relative numbers of lymphocyte subsets, T lymphocyte proliferative response, SAP protein expression were assessed by flow cytometry. Quantification of signal joint TCR rearrangementexcision circle (sjTRECs), CDR3 spectratyping of TCRvβ and gene mutation of SH2D1A were detected by PCR based on genomic DNA or cDNA.@*Result@#Four male patients from two families were diagnosed with XLP-1. The ages of disease onset were more than 1 year, more than 1 year, more than 1 month and 6 months. The ages at diagnosis were nine years and ten months, sixteen years and eight months, fourteen years and ten months, four years and nine months. All patients had recurrent infections and EBV infection. Patients 1, 2, and 3 had agammaglobulinemia and Patient 4 had hypogammaglobulinemia. Chest CT scan showed all patients had atelectasis and pneumonia, and Patient 3 had bronchiectasis. Patient 3 was diagnosised as Burkitt lymphoma. For immunological function, all patients exhibited reduced CD4/CD8 ratios, increased numbers of exhausted T lymphocyte, decreased number of NK cell. The numbers of total B lymphocyte and naïve B lymphocyte were normal, but the number of memory B lymphocyte declined in all cases. Four patients′ copy numbers of sjTRECs were low and CDR3 spectratypings of TCRvβ showed mildly skewed. But their T lymphocyte proliferative response was normal. SAP protein expression in four cases were measured by flow cytometry. Two patients from Family A were absent and two patients from Family B showed decreased values. SH2D1A gene sequence analysis showed that the patients of Family A harbored a nonsense mutation (c.163 C>T; p.R55X) in exon 2. Their mother and two sisters were carriers. A missense mutation of SH2D1A gene (c.278 G>A; p.G93D) in exon 3 was found in the patients of Family B. The mother was carrier. Four patients remain survived, Patient 3 gave up treatment, other three patients received IVIG therapy.@*Conclusion@#Four patients with XLP-1 from two families characterized by agammaglobulinemia have an extreme vulnerability to Epstein-Barr virus (EBV) infection. The functions of T cell, B cell and NK cell are impaired at different stages. The detection of SAP protein and SH2D1A gene are the key methods for diagnosis of XLP-1.

Comparison of the effect of urokinase and batroxobin in the treatment of acute cerebral infarction / 中国基层医药

Objective To compare the efficacy and safety of urokinase and batroxobin in the treatment of acute cerebral infarction(ACI).Methods According to the digital table,88 patients with ACI were randomly divided into two groups.The observation group (44 cases) was given thrombolytic therapy,the control group (44 cases) was given alteplase treatment.The treatment effects and safety were compared between the two groups.Results The NIHSS scores before treatment,24h,1 week and 2 weeks after thrombolysis had no statistically significant differences between the two groups (all P ＞ 0.05).After treatment for 1 month,29 cases (65.9 ％) of nerve function good progno sis in the observation group,28 cases(63.6％) in the control group,there was no significant difference between the two groups(x2 =0.049,P =0.958).In the control group,1 case of cerebral hemorrhage,and occlusion in 1 case,1 case of other adverse reactions (hematuria),the incidence rate of complications and adverse reaction was 6.82％ (3/44).In the observation group,2 cases of hemorrhage (including 1 case of cerebral hemorrhage and 1 case of gastrointestinal bleeding),1 case of reperfusion injury and restenosis,2 cases of other adverse reactions (including hematuria and allergic rash,each in 1 case),the incidence rate of complications and adverse reaction was 13.64％ (6/44).There was no statistically significant difference in the incidence rate of complications and adverse reaction between the two groups (x2 =0.495,P =0.597).Conclusion Urokinase and alteplase in the treatment of ACI have considerable effect in improving the symptoms of patients with neurologic impairment,alteplase is slightly in terms of security advantages,but the price is relatively expensive,urokinase in the treatment of ACI in primary hospital is still desirable.