Objective To analyze the functional changes and the clinical significance of B cell specific mono-clonal murine leukemia virus integration site -1 (Bmi -1 )and Th1 /Th2 cells in children with newly diagnosed im-mune thrombocytopenia(ITP)by testing the mRNA expressions of Bmi -1,helper T cell -related cytokine interferon (IFN)-γand interleukin(IL)-4 in children with newly diagnosed ITP.Methods Thirty -six cases of patients with newly diagnosed ITP in the experimental group came from the inpatient and outpatient children admitted to the Depart-ment of Pediatrics of the First Affiliated Hospital of Xinxiang Medical University from April to December 201 3.In the control group,26 cases of children requiring selective operation were admitted to the Department of Pediatric Surgery during the same period.The mRNA expressions of Bmi -1,IFN -γand IL -4 in the peripheral blood lymphocytes were detected by means of the reverse transcription -polymerase chain reaction(RT -PCR)method,and were analyzed and compared by t test and linear correlation analysis.Results (1 )The mRNA expressions of Bmi -1,IFN -γand IL -4 in peripheral blood lymphocytes in the experimental group were 2.63 ±0.54,3.84 ±0.43 and 1 .44 ±0.39,respec-tively;while the mRNA expressions of Bmi -1,IFN -γand IL -4 in the peripheral blood lymphocytes in the control group were 3.91 ±0.92,2.88 ±0.57 and 1 .87 ±0.34,respectively.The levels of IFN -γof the experimental group were significantly higher than those of the control group (P <0.001 )and the levels of Bmi -1 and IL -4 in the experi-mental group were significantly lower than those in the control group (all P <0.001 ).(2)The mRNA expressions be-tween IFN -γand IL -4 in the peripheral blood lymphocytes in the experimental group were in negative correlation (r =-0.667,P <0.001 ).The mRNA expressions between IL -4 and Bmi -1 in the same group were in a positive correlation (r =0.776,P <0.001 ).There were no correlation in the mRNA expressions between IFN -γand Bmi -1 (r =-0.206,P >0.05).Conclusions Bmi -1 may be involved in the pathogenesis of ITP by regulating Th cell, and Th cell dysfunction may occur in the children with ITP,and the disproportion between Th1 and Th2 may be due to the advantages of Th1 .

Objective To explore the efficacy and safety of tacrolimus among Chinese Takayasu arteritis (TAK) patients. Methods This was a single center, prospective study of active TAK patients treated with tacrolimus. Clinical manifestations, white blood cell count, hemoglobin level, erythrocyte sedimentation rate (ESR), hypersensitivity C reactive protein (hsCRP), alanine and aspartate aminotransferase and serum creatinine were recorded before and during tacrolimus treatment. Vascular changes were repeated every 6 months during tacrolimus treatment. All data were analyzed by statistical product and service solutions (SPSS) 20.0 statistical software, unpaired t test and Fisher exact probability and Kruskal-Wallis H test were used for statistical analysis. Results A total of 19 consecutive patients with an average age of (26 ±6) years were analyzed in this study. Sixteen of them were women. Pulselessness, fatigue, asymmetric blood pressure and fever were the most common clinical findings. Cervical and subclavian artery were more vulnerable. The most common artery involvement pattern was Numano type Ⅰ, followed by type Ⅱa and type Ⅴ. The median tacrolimus dosage was 2(2, 3) mg. Tacrolimus was effective in 9 out of the 19 patients. Patients who responded to tacrolimus tended to have lower mean ESR [(33±29) mm/1 h vs (42±20) mm/1 h, t=-0.776, P=0.448] and hsCRP [(20 ±31) mg/L vs (54 ±45) mg/L, t=-1.758, P=0.099] levels. However, no statistical significance was observed. During tacrolimus treatment, no drug related side effect was observed. Conclusion Tacrolimus is an alternative and effective therapy for some of the TAK patients.

It is necessary to use objective and accurate methods to assess the changes of the consciousness of patients emergencing from general anesthesia. In this way, adverse medications during the waking period can be avoided, and it can ensure the stable and safe recovery of consciousness of the patients, quickly remove the adverse factors affecting the patients, and strive to reduce the occurrence of complications during the waking period. This article briefly reviews the research progress of bispectral index and other common clinical anesthesia depth monitoring techniques used to assess the changes of consciousness of patients awakening from general anesthesia, and explores the regular pattern of recovery of consciousness in patients awakening from general anesthesia, in order to reduce complications in the recovery period .

One 51 years old man was admitted to the rheumatology department with a history of prominent eyes, headache and blurred vision for half year. The main manifestations included retrobulbar inflammatory pseudotumor and retroperitoneal fibrosis. He was initially diagnosed as granulomatosis with polyangiitis. Prednisone and cyclophosphamide were administrated and effective. New mass of dura mater and osteosclerosis presented during follow up. Finally Erdheim Chester disease(ECD) was diagnosed by biopsy and pathological examination. Vemurafenib, a v-raf murine sarcoma viral oncogenes homolog B1 (BRAF) inhibitor, 480 mg was given twice a day. The patient′s condition is stable and still in follow-up. Although ECD is a rare histiocytosis, clinicians should pay attention to its manifestations and differential diagnoses.

Objective:To analyze risk factors for duration of small or medium-sized coronary artery aneurysms (CAA) in children with Kawasaki disease (KD) so as to provide clinical guidance for early and full course treatment.Methods:The clinical data of 68 children diagnosed with KD in the Department of Pediatrics, the First Affiliated Hospital of Xinxiang Medical University from January 2018 to January 2021 were retrospectively analyzed.According to duration of CAA, all cases were divided into 2 groups, duration of CAA ≥ 8 weeks group and duration of CAA <8 weeks group.Risk factors associated with CAA duration were screened using univariate analysis, and then independent risk factors for CAA duration in children with KD were analysed using multiple Logistic regression analysis. Results:A total of 68 cases were enrolled in this study.Among these cases, 45 cases (66.18%) were male and 23 cases (33.82%) were female.The onset age was from 3 months to 10 years old, and the median onset age was 1.59 (1.02-3.19). There were 31 cases in the group with CAA duration ≥8 weeks and 37 cases in the group with CAA duration <8 weeks.Univariate analysis showed that patients with the total fever course >10 days[45.16%(14/31 cases) vs.21.62%(8/37 cases)], time of treatment with intravenous immunoglobulin (IVIG)>10 days[54.84%(17/31 cases) vs.16.22%(6/37 cases)], platelet (PLT)>600×10 9/L[32.26%(10/31 cases) vs.10.81%(4/37 cases)], hypersensitive C-reactive protein (HsCRP) >100 mg/L[38.71%(12/31 cases) vs.13.51%(5/37 cases)] (all P<0.05 ) in the group with CAA duration ≥8 weeks were significantly more than those in the group with CAA duration <8 weeks.However, there were no significant differences in gender, age, type of KD, etiology evidence, hormone application, duration of fever before IVIG application, IVIG sensitivity, IVIG application way, urine leukocytes, white blood cells, hemoglobin, percent of neutrophilic granulocyte, erythrocyte sedimentation rate, glutamic-pyruvic transaminase between the 2 groups (all P>0.05). Multivariate Logistic regression analysis showed that the course of IVIG before application >10 days ( OR=6.589, 95% CI: 1.678-25.867, P=0.007)and HsCRP >100 mg/L ( OR=7.949, 95% CI: 1.947-32.461, P=0.004)were independent risk factors for predicting the duration of KD complicated with small and medium-sized CAA ≥8 weeks. Conclusions:The course of IVIG before application >10 days and HsCRP>100 mg/L are independent risk factors for KD complicated with small and medium-sized CAA lasting ≥8 weeks.

Objectives:To analyze the clinical features of juvenile myelomonocytic leukemia (JMML) and investigate the characteristics of diagnosis and treatment of this disease.Methods:The clinical data of seven children patients with JMML who received treatment in The First Affiliated Hospital of Xinxiang Medical University between April 2015 and February 2020 were retrospectively analyzed. The clinical efficacy of different treatments was analyzed.Results:The median age at diagnosis of JMML was 8 months and 4 days for seven children patients. Fever was the principal cause of treatment, and it was mostly accompanied by hepatosplenomegaly. The median value of peripheral blood leukocyte count was 36.1 × 10 9/L, and it was 4.5 × 10 9/L for mononuclear cell count, 88 g/L for hemoglobin level, and 47 × 10 9/L for platelet count. Myeloid immature cells were found in peripheral blood smears of six patients. Chromosome examination results revealed 7-monomer in one patient, and normal karyotype in six patients. Hemoglobin level was increased in six patients. Gene detection results revealed PTPN11+NF1 mutation in one patient, N-RAS mutation in two patients, and K-RAS mutation in one patient. Three patients gave up treatment, three patients received low-intensity chemotherapy , and these six patients died of complicated infection. One patient received allogeneic hematopoietic stem cell transplantation and the patient survived without any event after 14 months of follow-up. Conclusion:The age of JMML onset is low. JMML has poor clinical specificity. Gene detection is helpful for early diagnosis of JMML. Low-intensity chemotherapy can prolong survival period, but it can not improve prognosis. Infection is the principal cause of death in patients with JMML. Hematopoietic stem cell transplantation is the only possible method to cure the disease.

Objective:To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA).Methods:The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity.Results:The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24), P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h, P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L, P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion:EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.

Objective To investigate the clinical features of Takayasu's arteritis (TAK)with glomerulonephropathy and to improve physicians' understanding of this complication in patients with TAK. Methods Clinical data were retrospectively collected including manifestations, laboratory tests, image findings and treatment of 8 patients diagnosed as Takayasu's arteritis with glomerulonephropathy from January 2002 to January 2017 in Peking Union Medical College Hospital. Results Glomerulonephropathy was confirmed based on percutaneous renal biopsy. There were 6 women and 2 men. The median onset age and median disease duration were 24 (18-37) years and 42 (3-360) months, respectively. Five patients had hypertension. The 24 hour urinary protein was 0.18-14.91 g. Red blood cells and casts in urine were tested among 4 and 2 patients, respectively. Three patients had renal artery stenosis. Three patients demonstrated mesangial proliferative glomerulonephritis, two with IgA nephropathy, two with minimal change disease and one with membranoproliferative glomerulonephritis. Seven patients received glucocorticoid combined with cyclophosphamide therapy (glucocorticoid 40-60 mg/d, prednisone or equivalent; cyclophosphamide 0.4 g/week iv. or cyclophosphamide 0.1 g/d po.). Uninary blood cells removed and 24 hour urinary protein decreased from 1.65 g to 0.90 g after treatment for 12 months in one patient. The other 7 patients were missing. Conclusion Glomerulonephropathy is occasionally observed among TAK patients. Mesangial proliferative glomerulonephritis is the most common pathological subtype. Glucocorticoid combined with cyclophosphamide therapy could be an optional therapy for Takayasu's arteritis with glomerulonephropathy.

Objective:To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022. The demographic characteristics, clinical manifestations, concurrent events, treatment, and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results:There were 71, 45, and 31 cases of granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatous polyangiitis (EGPA), respectively, among 147 patients. The ANCA positivity rates in the three groups were 91.5%, 95.6%, and 19.4% ( χ2=76.68, P<0.01), respectively. The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA, and the kidneys and lower respiratory tract were the main organs involved in MPA. In addition, cardiac and neurological involvement and thrombosis rates were significantly higher in EGPA patients than in GPA and MPA (12.9%, 9.7%, 41.9% and 19.4%, respectively; χ2=8.51、7.13、7.54、0.02, P<0.05) .The median follow-up time for the three groups of patients was 43, 28, and 46 months respectively.Relapse was more common in patients with GPA and EGPA (up to 59.2% and 64.5%; χ2=11.26, P=0.004), with the lungs and ENT being the most common relapse organs (GPA of 61.9% and 40.5%, EGPA of 55.0% and 50.0%), the lungs and kidneys were the most common manifestations in MPA relapse (64.3% and 60.0%, respectively). The main therapeutic agents were glucocortoid (95.9%), cyclophosphamide (71.4%), methotrexate (54.4%), tripterygium wilfordiz (34.0%),mycophenolate mofetil (31.3%), azathioprine (29.3%), leflunomide (19.0%), rituximab (19.0%), and tacrolimus/cyclosporine (8.8%). There were 6 deaths (4.1%) occurred during the follow-up period of this study. Conclusion:The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.

Objective:To evaluate the efficacy and safety of rituximab(RTX) as remission-mainten-ance therapy in antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods:Patients with AAV, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), treated with rituximab (RTX) in Peking Union Medical College Hospital during September 2005 to June 2021 were included into this study. Clinical data, relapse rate, time of first relapse and adverse events were collected and analyzed. The cumulative relapse rate was calculated by Kaplan-Meier, t test, and Man-Whithey U test and chi-square were used to compare differences between two groups. Results:① Thirty-nine AAV patients were enrolled, including 36 GPA and 3 MPA. During the 20(3, 104) months follow-up, 59.0%(23/39) patients had suffered relapses. The time for first relapse was 11(3, 42) months after remission. ② There were no difference in the relapse rate [60.0%(18/30) vs 55.6%(5/9), χ2=0.06, P=1.000), the time of first relapse [15(3, 42) vs 10(9, 30), Z=0.45, P=0.678], CD19 + B [23.5 (5, 148) cell/μl vs 3(2, 15) cell/μl, Z=0.57, P=0.605] and serum IgG [7.09(5.13, 13.90) g/L vs 9.72(5.32, 12.0) g/L, Z=0.36, P=0.770] between standard dose and low-dose groups. The rate of major relapse-free was significantly less in patients treated with standard dose than patients with reduced dose of RTX {87.1%[95% CI(73.4%, 100.8R%)] vs 64.3%[95% CI(23.1%, 105.4%)], χ2=7.59, P=0.006}. ③ There were no difference in relapse rate [50.0%(3/6) vs 60.6%(20/33), χ2=0.24, P=0.674], time of first relapse [23(6, 25) vs 11(3, 42), Z=0.05, P=0.982], CD19 + B[35(15, 50) cell/μl vs 10(0, 148) cell/μl, Z=0.95, P=0.382] and serum IgG[6.70(5.91, 7.49) g/L vs 7.69(3.78, 13.90) g/L, Z=0.48, P=0.700] between the fixed interval dosage and the on-demand dosage groups. There was no difference in the rate of major relapse-free between the two groups (100% vs 77.8%, χ2=1.79, P=0.181). ④ The incidence of infusion reaction was 5.1%(2/39) and infection was 20.5%(8/39). Serum IgG level was 4.37(3.78, 13.4) g/L at infection. There was no difference in safety between the standard and low-dose groups or between fixed interval and on-demand dosage groups ( P>0.05). Conclusion:There is no significant difference in relapse rate bet-ween the standard RTX dose and low-dose RTX induction therapy group, but the major relapse rate is sign-ificantly reduced in the standard dose RTX therapy. The relapse rate of fixed intervals dosage group is similar to that of on-demand dosage group. The safety profile of the standard dose and low-dose induction therapy groups or fixed intervals and on-demand dosage groups is similiar.