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Group 3 innate lymphoid cells (ILC3) are an ILC subset that is characterized by the expression of retinoic acid-related orphan nuclear receptor γt (RORγt) and interleukin 22 (IL-22). This review summarizes the role of ILC3 in coordinating innate immunity and adaptive immunity based on current research and elaborate the significance of ILC3 from the perspective of immune system evolution. In addition, based on immune-related functions, we propose a possible time when ILC3 appears in the evolution of the immune system. And then, the research limitations and prospects are discussed.
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Immunity, Innate , Lymphocytes , TretinoinABSTRACT
【Objective】 To determine molecular basis of a rare HLA-A typing results carrying triple A alleles in potential allo-HSCT donor and her family. 【Methods】 HLA-A, -B, -C, -DRB1, -DQB1, -E, -F, -G of 5 members in the family were genotyped at a high-resolution level using next-generation sequencing (NGS). HLA-A of probosita was re-checked using polymerase chain reaction-sequence-based typing (PCR-SBT), and SNP oligonucleotide probes (SNP-array)were scanned with genomic DNA of probosita. 【Results】 There was 162.9Kb duplication in 6p22.1(29, 803, 377-29, 966, 301)of probosita who carried triple A alleles A*02∶01∶01, A*11∶01∶01, A*24∶02∶01. Other two family members were found to carry this haplotype: A*02∶01∶01, A*24∶02∶01, B*54∶01∶01, C*01∶02∶01, DRB1*04∶05∶01, DQB1*04∶01∶01, E*01∶01∶01∶03, F*01∶01∶01, G*01∶01∶01∶01, which as a Mendelian gene was segregated and stably transmitted through two generations. 【Conclusion】 Tiny gene duplication induces one haplotype carries two HLA-A alleles in a potential healthy donor for allo-transplantaion and stably transmits through two generations.Routine HLA typing laboratories should pay more attention to this situation and accurately report.
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Objective@#To describe eraly and midterm outcome of the Rastelli repair in Fuwai hospital Patients.@*Methods@#From May 2010 to March 2017, 71 patients with transposition of the great arteries(TGA)with ventricular septal defect(VSD)and right ventricular outflow tract obstruction(RVOTO) or double outlet right ventricle(DORV)with VSD and RVOTO underwent Rastelli repair. 48 cases male , 23 cases female . Age at operation is(4.7±2.7) years. There are 10 TGA cases, 27 DORV cases, 34 CTGA cases in this group. 30 patients(42.3% , 30/71)received palliative operation prior to the Rastelli procedure, including 13 BT shunt and 17 bi-Glenn operation. 31 patients(43.7%, 31/71 )underwent the Rastelli procedure with VSD enlargement. Right ventricle-to-pulmonary artery connection were created with the use of 9 homografts, 56 valved bovine jugular vein, 6 man-made valved Gore-Tex conduit. The overall mean right ventricle-to-pulmonary artery conduit size was(17.9±3.3)mm.@*Results@#CPB time was(209.0±83.4)minutes, aortic crossclamping time was(132.0±71.1)minutes, mechanical ventilation time was(102.6±81.7)h. Early mortality was 1.4%(1/71). morbidity in hospital was 16.9%, 4 patients with Ⅲ AVB implanted permanent pacemaker, Subxiphoid pericardial window drainage in 3 cases, delayed sternal closure in 3 and re-thoratomy for hemaostsis in 2.Follow up is from 4 months to 6.8 years. Overall survival was 97.2% and 97.2% at 1 and 5 years, respectively. Freedom from RVOTO was 98.6% and 84.1% at 1 and 5 years, respectively. Freedom from reintervention was 98.6% and 90.0% at 1 and 5years, respectively. 1 patients performed a conduit replacement. Seven patients performed 10 times balloon dilatation . Time-related freedom from recurrent LVOTO on echocardiogram in all patients, and the pressure gradient of the LV to the aorta was(10.5±8.8 )mmHg at the most recent follow-up.@*Conclusion@#The Rastelli operation remains the preferred procedure for part of the DORV , CTGA , TGA with VSD and severe fixed valvular or subvalvular PS. The Rastelli procedure can be performed with low early mortality. There is frequent need for late reoperation, especially for conduit replacement.
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Objective@#To explore the relationship between driver gene mutation (JAK2, MPL and CALR) and disease type in BCR-ABL negative myeloproliferative neoplasms (MPNs) including primary myeloid fibrosis (PMF), essential thrombocytosis (ET) and polycythemia vera (PV).@*Methods@#A total of 32 MPN related genes were detected by high-throughput sequencing in 156 MPN patients. The relationships between disease type and patients′ general performance, the characteristics of driver gene mutations, concomitant gene mutations were analyzed.@*Results@#In the population with JAK2 V617F positive mutation, the proportion of patients over 60 years old in PMF was higher than that with ET or PV. By high-throughput sequencing, 22 concomitant gene mutations were detected in 46 patients with JAK2, MPL or CALR mutations, including 4 (8.3%) in PV, 20 (29.4%) in ET, and 22 (55.0%) in PMF. DNMT3A mutation was detected only in patients with PV, while splicing factor related genes including SF3B1, SRSF2 and U2AF1 were only accompanied by PMF. According to the variation allele frequency (VAF) value of JAK2 V617F mutation, the VAF value associated with PV was the highest (68.15%), followed by PMF (37.7%) and ET (23%). However, there were significant differences in the incidence of JAK2 V617F homozygous among 3 different diseases. In patients with JAK2 mutation, the proportion of other gene mutations in PV and ET was significantly lower than that in PMF.@*Conclusions@#Under the condition of common driver gene mutations (JAK2, MPL and CALR), patients′ age, VAF value and homozygous state, concomitant gene mutations are closely related to different disease type. These correlations help to improve clinical understanding of disease characteristics and risk assessment.
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Objective:To describe eraly and midterm outcome of the Rastelli repair in Fuwai hospital Patients.Methods:From May 2010 to March 2017, 71 patients with transposition of the great arteries(TGA)with ventricular septal defect(VSD)and right ventricular outflow tract obstruction(RVOTO) or double outlet right ventricle(DORV)with VSD and RVOTO underwent Rastelli repair. 48 cases male , 23 cases female . Age at operation is(4.7±2.7) years. There are 10 TGA cases, 27 DORV cases, 34 CTGA cases in this group. 30 patients(42.3% , 30/71)received palliative operation prior to the Rastelli procedure, including 13 BT shunt and 17 bi-Glenn operation. 31 patients(43.7%, 31/71 )underwent the Rastelli procedure with VSD enlargement. Right ventricle-to-pulmonary artery connection were created with the use of 9 homografts, 56 valved bovine jugular vein, 6 man-made valved Gore-Tex conduit. The overall mean right ventricle-to-pulmonary artery conduit size was(17.9±3.3)mm.Results:CPB time was(209.0±83.4)minutes, aortic crossclamping time was(132.0±71.1)minutes, mechanical ventilation time was(102.6±81.7)h. Early mortality was 1.4%(1/71). morbidity in hospital was 16.9%, 4 patients with Ⅲ AVB implanted permanent pacemaker, Subxiphoid pericardial window drainage in 3 cases, delayed sternal closure in 3 and re-thoratomy for hemaostsis in 2.Follow up is from 4 months to 6.8 years. Overall survival was 97.2% and 97.2% at 1 and 5 years, respectively. Freedom from RVOTO was 98.6% and 84.1% at 1 and 5 years, respectively. Freedom from reintervention was 98.6% and 90.0% at 1 and 5years, respectively. 1 patients performed a conduit replacement. Seven patients performed 10 times balloon dilatation . Time-related freedom from recurrent LVOTO on echocardiogram in all patients, and the pressure gradient of the LV to the aorta was(10.5±8.8 )mmHg at the most recent follow-up.Conclusion:The Rastelli operation remains the preferred procedure for part of the DORV , CTGA , TGA with VSD and severe fixed valvular or subvalvular PS. The Rastelli procedure can be performed with low early mortality. There is frequent need for late reoperation, especially for conduit replacement.
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Objective:To explore the characteristics and clinical significance of clonal heterogeneity in patients with acute lymphoblastic leukemia(ALL).Methods:From January 2016 to June 2019, 170 newly diagnosed ALL patients were enrolled in the Department of Hematology, Henan Cancer Hospital, including 93 males and 77 females, with a median age of 17 (2-80) years. Fifty-two ALL-related genes were detected by high-throughput sequencing technique. The clonal heterogeneity of mutations was analyzed according to the variant allele frequency (VAF) and the results of flow cytometry. The prognostic value of mutations was also evaluated.Results:Gene mutations were detected in 121 (71.2%, 121/170) patients, of which 2 or more clones were detected in 18 (52.9%, 18/34) T-cell acute lymphoblastic leukemia patients, while only 23 (16.9%, 23/136) B-cell acute lymphoblastic leukemia patients were positive of multiple mutations ( P<0.01).Gene mutation-related clonal heterogeneity analysis showed that 2 or more clones were frequent in patients with NOTCH1 mutations (13/19 patients) ( P<0.01). Event free survival (EFS) in patients with 3 or more clones was significantly lower than other patients (χ 2=10.330, P=0.016). Child ALL patients had similar result, that multiple clones predicted lower overall survival (OS) and EFS (OS: χ 2=7.974, P=0.047; EFS: χ 2=10.860, P=0.013). Conclusion:Clonal heterogeneity in ALL patients is closely related to the different origin of lymphocyte lineages and the age of onset, which may reveal the nature of the disease and predict the clinical outcome.
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Objective:To explore the relationship between driver gene mutation (JAK2, MPL and CALR) and disease type in BCR-ABL negative myeloproliferative neoplasms (MPNs) including primary myeloid fibrosis (PMF), essential thrombocytosis (ET) and polycythemia vera (PV).Methods:A total of 32 MPN related genes were detected by high-throughput sequencing in 156 MPN patients. The relationships between disease type and patients′ general performance, the characteristics of driver gene mutations, concomitant gene mutations were analyzed.Results:In the population with JAK2 V617F positive mutation, the proportion of patients over 60 years old in PMF was higher than that with ET or PV. By high-throughput sequencing, 22 concomitant gene mutations were detected in 46 patients with JAK2, MPL or CALR mutations, including 4 (8.3%) in PV, 20 (29.4%) in ET, and 22 (55.0%) in PMF. DNMT3A mutation was detected only in patients with PV, while splicing factor related genes including SF3B1, SRSF2 and U2AF1 were only accompanied by PMF. According to the variation allele frequency (VAF) value of JAK2 V617F mutation, the VAF value associated with PV was the highest (68.15%), followed by PMF (37.7%) and ET (23%). However, there were significant differences in the incidence of JAK2 V617F homozygous among 3 different diseases. In patients with JAK2 mutation, the proportion of other gene mutations in PV and ET was significantly lower than that in PMF.Conclusions:Under the condition of common driver gene mutations (JAK2, MPL and CALR), patients′ age, VAF value and homozygous state, concomitant gene mutations are closely related to different disease type. These correlations help to improve clinical understanding of disease characteristics and risk assessment.
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Objective@#To establish a new method for chimerism analysis after allogeneic hematopoietic stem cell transplantation by using multiple nucleotide polymorphism sequencing (MNPseq) , and to explore its feasibility and superiority.@*Methods@#One hundred MNP fragments were screened and chimeric analysis was performed by high-throughput sequencing technology. The accuracy and sensitivity of the method were verified by simulating chimeric samples and post-transplant samples and comparing them with short tandem repeat (STR) analysis, fusion gene quantitative detection and flow cytometry for minimal residual disease.@*Results@#The accuracy and sensitivity of MNPseq were better than those of STR, in which the sensitivity could reach 0.01%, about 100 times more sensitive than STR. MNPseq could further distinguish 42 STR fully chimeric samples, and after corrected by cutoff value, it was correlated with the quantitative detection of fusion gene. MNPseq could correct false positive of STR caused by the shadow peak, and could be used to detect chimeric samples lacking pre-transplant information from donors and recipients.@*Conclusion@#MNPseq analysis based on high-throughput sequencing is a more accurate and sensitive chimerism detection method, and it solves the problem that chimerism cannot be detected due to the lack of pre-transplant information, which has extremely high clinical application value.
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Objective To investigate pregnancy outcomes of the patients with polycystic ovary syndrome(PCOS)after frozen embryo transfer(FET). Methods Data of 2367 PCOS patients received in vitro fertilization-embryo transfer [including fresh embryo transfer (fET) and FET] from January 2009 to December 2015 in Peking University Third Hospital were evaluated retrospectively. The basal characteristics, pregnancy complications and outcomes were analyzed, then identified the relative factors followed. Results Totally 2367 patients received in vitro fertilization-embryo transfer:1106 were treated with fET, and the rest 1261 cases were treated with FET. The incidence of gestational diabetes mellitus (GDM)was lower in FET group [4.04％(51/1261) versus 6.15％(68/1106)], the difference was statistically significant (P<0.05). Singletons born after FET had higher birth weight than fET [(3406±548) versus (3360± 533) g], the difference was statistically significant (P<0.05). There was no difference of other pregnancy complications between the two groups (all P>0.05). fET was an independent risk factor for GDM (adjusted OR=1.570, 95％CI:1.075-2.294). Conclusion Compared with fET, FET could decrease the risk of GDM and receive better neonatal outcomes in patients with PCOS.
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Objective To compare the effect of laparoscopic thymectomy on immune function and inflammatory factors in patients with myasthenia gravis.Methods 80 patients with myasthenia gravis treated by thymectomy were selected,and they were divided into observation group and control group according to the random number method,each group in 40cases.The observation group was treated with video assisted thoracoscopic surgery,the control group was treated by thoracotomy.All the patients were followed up for outpatient visits in 3 months.The incidences of myasthenia gravis crisis in the two groups were compared and the ratio postoperative humoral immunoglobulin levels and inflammation related factor changes were observed.Results The operation time of the observation group [(118.6±11.3)min] was shorter than (138.5±13.8)min of the control group (t=7.056,P<0.05).The amount of bleeding of the observation group [(96.6±3.8)mL] was less than (125.5±5.6)mL of the control group (t=27.008,P<0.05).The hospitalization time of the observation group [(7.2±0.3)d] was shorter than (10.8±1.1)d of the control group (t=19.969,P<0.05).The incidence rate of myasthenia gravis crisis in the observation group was significantly lower than that in the control group (1 patient vs 9 cases,x2=5.600,P=0.018).The levels of IgM,IgG and IgA in the observation group were (1.65±0.03)mg/L,(9.55±0.12)mg/L,(3.99±0.20)mg/L respectively, which were higher than (1.31±0.02)mg/L,(8.82±0.10)mg/L,(1.81±0.13)mg/L in the control group(t=59.640,29.557,57.800,all P<0.05).The IL-6,TNF-α,IL-1,hs-CRP levels in the observation group were (34.5±6.1)ng/mL,(12±0.2)ng/mL,(0.60±0.1)g/mL,(10.3±1.0)mg/L respectively, which were lower than (153±14.1)ng/mL,(18.1±0.5)ng/mL,(0.92±0.2)g/mL,(31.3±2.0)mg/L in the control group (t=48.784,71.641,9.051,59.397,all P<0.05).Conclusion Laparoscopic thymectomy for myasthenia gravis has short operative time and less bleeding.It has little effect on the humoral immune function of the patients and can significantly reduce the inflammatory response and promote the recovery of the patients.
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Objective The aim of this study was to evaluate the clinical and phychology profile after total cavopulmonary connection procedure and to identify the significant determinants of quality of life.Methods Data from 21 patients underwent total cavopulmonary connection procedure from January 2008 to December 2015 were retrospectively analyzed.Patients under went echocardiography aud cardiopulmonary exercise testing.Used ST-36 questionnaire to perceived health status.The NT-ProBNP levels was test.Results 21 patients aged between 19 to 36 years(mean 26.6 years) were enrolled.The mean follow-up time was(44.2 ± 29.9) months.The mean EF was (59.4 ± 6.2) ％,and the peak oxygen uptake was (19.9 ± 3.6) ml · kg-1 · min-1,perceut predict value was(52.5 ± 8.9) ％.SF-36 score was well.Independent risk for impaired exercise capacity was interval time from surgery.Conclusion The exercise capacity of total cavopulmonary connection patients was impaired.Identify more predict factors of the quality of these patients need further study.
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Objective The aim of this study was to evaluate the clinical and phychology profile after total cavopulmonary connection procedure and to identify the significant determinants of quality of life.Methods Data from 21 patients underwent total cavopulmonary connection procedure from January 2008 to December 2015 were retrospectively analyzed.Patients under went echocardiography aud cardiopulmonary exercise testing.Used ST-36 questionnaire to perceived health status.The NT-ProBNP levels was test.Results 21 patients aged between 19 to 36 years(mean 26.6 years) were enrolled.The mean follow-up time was(44.2 ± 29.9) months.The mean EF was (59.4 ± 6.2) ％,and the peak oxygen uptake was (19.9 ± 3.6) ml · kg-1 · min-1,perceut predict value was(52.5 ± 8.9) ％.SF-36 score was well.Independent risk for impaired exercise capacity was interval time from surgery.Conclusion The exercise capacity of total cavopulmonary connection patients was impaired.Identify more predict factors of the quality of these patients need further study.
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This study was aimed to discuss the anti-inflammatory and immunization properties ofRe-Du-Ning (RDN) injection in the treatment of acute exacerbation of chronic obstructive pulmonary disease (AECOPD) with phlegm-heat stagnated in the lung syndrome. A total of 110 in-patients were collected from January 2012 to December 2013. Cases were randomly divided into the treatment group (basic treatment plan + 20 ml RDN injection + 250 mL of 5% GS injection, once a day, intravenous injection) and the control group (basic treatment plan + 20 ml of 0.9% NS injection + 250 mL of 5% GS injection, once a day, intravenous injection), with 55 cases in each group. The treatment course was 14 days. Observations were made on traditional Chinese medicine (TCM) symptom score, clinical effect, blood routine examination, blood gas analysis and T lymphocyte subgroups before and after the treatment in both groups. The results showed that in the aspects of TCM symptom score and clinical effect, the posttreatment TCM symptom score and total integration in the treatment group were obviously improved compared to the control group except for shortness of breath, dry mouth and thirsty, flushing (P < 0.05). The total effective rate was obviously better than that of the control group. In the aspect of anti-inflammation, the total white blood cell (WBC) count and the normal neutrophil percentage of both pretreatment and posttreatment in the treatment group were obviously decreased compared to pretreatment (P < 0.05). However, there were no statistical differences on WBC count and normal neutrophil percentage compared to posttreatment in the control group. In the aspect of blood gas analysis, the posttreatment PaCO2 and PaO2 of the treatment group were obviously better than the control group (P < 0.05). In the aspect of immune regulation, the posttreatment T lymphocyte subgroups CD3+ and CD4+ of the treatment group were higher than the control group. The expression inhibition / cytotoxic lymphocyte (CD8+) was obviously lower than the control group (P < 0.05). The CD4+ / CD8+ was back to the normal reference level. It was concluded that in the treatment of AECOPD with phlegm-heat stagnated in the lung syndrome, on the basis of western medicine symptomatic treatment plan, RDN injection was assisted to clear heat, relieve toxin and remove phlegm. It can obviously improve patient’s clinical symptoms and increase the clinical therapeutic effects. The treatment was especially targeted to infection-induced respiratory failure patients combined low immunity with possible identified therapeutic effects.
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In medical clinical study, the researchers and the ethics committee members' personal economic in-terests and responsibilities conflicts may produce damage to experimental research and the subjects, may make the study being questioned the authenticity and the objectivity of clinical trial results, also damage to the subjects' rights and interests and damage the credibility of hospital. In order to prevent the happening of the conflict, put forward the strategies:strengthen legislation construction, improve the treatment method, set up a conflict of interest com-mittee, adhere to the principle of open, review and restriction to the personnel, regularly organize researchers and ethics committee members attend the training.
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This article elaborated the theory of chronic and complex disease treatment based on collaterals. It put forward that complex virtual evil stasis was the common pathogenesis of chronic and complex disease treatment. Combined with articles published in recent 5 years, from the perspective of cytokines and genes, it summarized traditional Chinese medicine (TCM) compound targets in chronic and complex disease treatment. It put forward the transforming growth factor-β1 (TGF-β1), tumor necrosis factor-α(TNF-α), interleukin-6 (IL-6), vascular endothelial growth factors (VEGFs), matrix metalloproteinases/tissue inhibitor of metalloproteinase (MMPs/TIMPs) were its high-frequency targets. It provided a basis for future experiment design on chronic and complex disease treatment based on collaterals as well as new drug development.
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Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic and progressive fibrosing inter-stitial pneumonia. Till now, it is lack of effective and safe treatment. In recent years, more and more clinical trials of traditional Chinese medicine (TCM) have been conducted with different evaluation criteria and indexes. The knowl-edge is disunity and unsystematic. From the perspective of combination of disease and TCM syndrome model, this ar-ticle summarized and organized the current data and medical standards, and then come up with the new evaluation system of clinical effect and describe important indexes and methods. This article was aimed to improve the clinical trial design and provide high quality evidences for evidence-based medicine.
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Objective To analyze the restricted usage of TCRβ V/J subfamily and CDR3 repertoire in patients with peripheral T-cell lymphoma unspecified (U-PTL).Methods The total RNA was respectively extracted from lymph node of U-PTL and reverse transcriptase,then multi-PCR was used to amplify the complete DNA sequence(CDS) of TCR β-chain.The recombinant plasmids were sequenced and sequence was analyzed by using online TCR resources.Results There were 9 TCR β chain CDS obtained from four patients.TCRβ-chain presented specific repertoire skewing in patients with U-PTL.There were restricted usage of BV2,BV4S2,BV14,BV29S1 of BV subfamily and BJ1S4,BJ2S3,BJ2S5,BJ2S7 of BJ subfanily.The clonal proliferation T cells had different CDR3 amino acid sequences.Conclusions There are restricted usage of TCR β V/J subfamily in patients with U-PTL.The sequences of CDR3 in different TCR clone proliferation are mostly different.
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Idiopathic pulmonary fibrosis(IPF), as one of the chronic and complex diseases, is with high incidence and mortality rate. It is also a difficult to treat disease in modern medicine. Traditional Chinese medicine (TCM) syndrome differentiation and treatment embodies characteristics and advantages. In recent years, the collateral disease theory guiding the prevention and treatment of the chronic and complex diseases has become a hot point and frontier in medical research, and achieved certain effects. This article is to summary the structures and functions of pulmonary collaterals based on the collateral disease theory. Combined with clinical manifestations of IPF, we put forward Fei-Re Luo-Y u as the fundamental role in the pathogenesis of IPF in its acute phase to lay the theoretical foundation to the further exploration of syndrome prevention and differentiation on IPF in its acute exacerbation.
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Objective To analyze the restricted usage of TCR BV,BJ subfamily and their sequences in patients with breast cancer.Methods The total RNA was extracted from tissues of lymph node metastasis,then reverse transcripted.The complete DNA sequence of TCR β-chain was amplified by multi-PCR.The recombinant plasmids were sequenced by using online TCR resources.Results 5 TCR β-chain CDS were obtained in two patients.TCR β-chain presented specific repertoire skewing in metastatic lymph node.There were selected usage of BV 2,BV 14,BV 29S1 of BV subfamily and BJ1S1,BJ2S2,BJ2S3,BJ2S5 of BJ subfamily.The clonal proliferation T cells had different CDR3 amino acid sequences.Conclusions There are selected usages of TCR BV,BJ subfamily in patients with breast cancer.The sequences of CDR3 are mostly different in TCR clone proliferation.