Objectives:To clarify the radiological anatomy features of adrenal arteries derived from digital subtraction angiography(DSA)in patients with primary aldosteronism(PA). Methods:The DSA images of 119 patients diagnosed with PA and underwent percutaneous selective adrenal artery embolization from January 2018 to December 2019 at the 2nd Affiliated Hospital of Nanchang University were retrospectively analyzed,and the number,origin,distribution,angle and diameter of adrenal arteries were analyzed. Results:The mean age of 119 PA patients was(49±11)years,with 71(59.7%)males,and at least one adrenal artery was successfully identified in all patients,a total of 192 adrenal arteries were analyzed.There were 20(10.4%)upper,40(20.8%)middle and 132(68.8%)lower adrenal arteries.Adrenal arteries originating from renal arteries accounted for 42.7%(82/192),adrenal arteries originating from the abdominal aorta accounted for 37.5%(72/192),and those from the inferior diaphragmatic arteries accounted for 19.8%(38/192).72.8%(83/114)of left adrenal arteries and 60.3%(47/78)of right adrenal arteries distributed at the level of the first lumbar vertebrae.28.1%(54/192)adrenal arteries distributed at the level of the second lumbar vertebrae,and 4.2%(8/192)adrenal arteries at the level of the 12th thoracic vertebrae.The angle range of left adrenal arteries intersecting with the origin arteries was 35.90° to 160.07°(renal artery),27.08° to 171.99°(accessory renal artery),0° to 158.70°(abdominal aorta);for right adrenal arteries,it was 18.43° to 172.53°(renal artery),69.26° to 114.62°(accessory renal artery),12.32° to 232.85°(abdominal aorta),respectively.The average diameters of left and right adrenal arteries were(0.98±0.45)mm and(1.27±0.42)mm,respectively. Conclusions:This study provides more detailed radiological anatomy data of adrenal arteries in PA patients.As a supplement to human anatomical features,the described data can provide practical guidance for adrenal artery interventional treatment.

Objective:To investigate the safety and efficacy of Belintoumab on the treatment of children with acute B-lymphoblastic leukemia (B-ALL).Methods:The clinical data of 10 children with CD 19+ B-ALL who were admitted to the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from September 2021 to May 2022 and treated with Belintoumab were analyzed retrospectively. Results:Among the 10 cases, there were 6 recurrent cases, 3 cases with persistent minimal residual disease (MRD) positive after an initial treatment, and 1 case complicated with invasive candidiasis.Before treatment, bone marrow blasts ≥0.25, and that ranged 0.05-<0.25 were detected in 2 cases and 1 case, respectively.Seven cases had a complete remission (CR) of bone marrow, 6 of which were MRD positive and 1 case was MRD negative.After treatment with Belintoumab, the CR rate was 66.7% (2/3). The overall MRD negative rate was 88.9% (8/9), and the negative rate in previously MRD positive children was 100% (6/6). The median follow-up time was 4.1 (1.6-10.0) months after the application of Belintoumab.The overall survival (OS) rate was 70.0% (7/10). Eight MRD negative children received hematopoietic stem cell transplantation, and the OS rate was 75% (6/8). Survived children did not relapse until the last follow-up visit.Fever (90%, 9/10) was the most common adverse events, followed by neutropenia (90%, 9/10). One case (10%, 1/10) of neurotoxicity was seizures (grade 2) and one case (10%, 1/10) suffered cytokine release syndrome (grade 2), which did not influence the therapeutic efficacy of Belintoumab after symptomatic treatment.Conclusions:Belintoumab is safe and effective on the treatment of children with recurrent/refractory CD 19+ B-ALL, and those with MRD positive who have achieved CR in bone marrow have a higher rate of turning negative.Belintoumab can also be used as a bridge scheme for CD 19+ B-ALL children who cannot tolerate chemotherapy.

【Objective】 To analyze the causes of immune hemolytic transfusion reaction in one case, identify related antibodies, and explore transfusion compatibility testing. 【Methods】 ABO/Rh blood group identification, unexpected antibody identification of serum and diffusion fluid, direct antiglobulin test(DAT) and cross matching were conducted by saline method and/or microcolumn gel method. 【Results】 The patient′s blood group was O, and Rh phenotype was identified as DCCee. The DAT was negative, with strong anti-E antibody and weak anti-c antibody detected. Acute hemolytic transfusion reaction occurred in the patient after the last transfusion. 【Conclusion】 Currently, immune hemolytic transfusion reaction in China are mainly caused by Rh blood group system antibodies. The absence of unexpected antibody screening before blood transfusion and the weak anti-c antibody which resulted in missed detection of non compatibility in cross matching led to acute hemolytic transfusion reaction. It is recommended to conduct unexpected antibody screening before blood transfusion, and to collect blood sample for testing as soon as possible to improve the accuracy of DAT when acute hemolytic transfusion reaction is suspected.

Objective:To investigate the clinical efficacy, safety and compliance of Voriconazole suspension formula on the prevention and treatment of invasive fungal infection (IFI) in children with allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:Clinical data of 25 children treated Voriconazole suspension formula for the prevention and treatment of IFI during the period of allo-HSCT in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from August 1, 2020 to April 30, 2021 were retrospectively analyzed.The plasma trough concentration of Voriconazole was detected by high-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS), and the genotype of CYP2C19 was detected by polymerase chain reaction-restriction fragment length polymorphism (RFLP). The effect of CYP2C19 genotype on Voriconazole trough concentration was analyzed by rank-sum test, and Fisher′ s accurate test was used to analyze the influence of severity of gastrointestinal mucositis on serum trough concentration of Voriconazole in children with allo-HSCT. Results:A total of 25 children, including 18 males and 7 females were recruited.The median age at allo-HSCT was 6 (2-13) years.After initial administration of conventional dose of Voriconazole suspension formula during transplantation, plasma trough concentration of Voriconazole was intermittently monitored.Only 13 cases (52.0%) reached the target plasma trough concentration, 11 cases(44.0%) reached the target plasma trough concentration after adjusting the dose according to the plasma concentration, and 1 cases(4.0%) failed to reach it after increasing the dose twice.Genotype detection of CYP2C19 was performed in 20 children, involving 4 cases of poor metabolizers (PM), 9 cases of intermediate metabolizers (IM), 6 cases of extensive metabolizers (EM), and 1 case of ultra extensive metabolizer (UEM). A significant difference in plasma trough concentration was detected among all groups ( F=24.012, P<0.01). During the transplantation, 12 cases developed mild to moderate gastrointestinal mucositis, and 7 cases had severe gastrointestinal mucositis.The stan-dard rate of plasma trough concentration in children with severe gastrointestinal mucositis (1/7 cases, 14.3%)was significantly lower than those with mild to moderate gastrointestinal mucositis (9/12 cases, 75.0%) ( P=0.02). Five children (71.4%) with severe gastrointestinal mucositis could reach the target trough concentration after increasing the drug dose, suggesting that severe gastrointestinal mucositis had a great influence on the plasma concentration of Vorico-nazole suspension.The incidence of IFI in 25 children with allo-HSCT was 0, and the compliance of children taking Voriconazole dry suspension was 100.0%.The incidence of adverse reactions was 24.0% and all adverse reactions were relieved after symptomatic treatment. Conclusions:The plasma concentration of Voriconazole varies greatly among children and in different states of the same patient.Therefore, it is necessary to monitor the trough concentration of the drug and adjust the drug dose.The use of Voriconazole suspension formula for the prevention and treatment of fungal infection during allo-HSCT in children is clinically safe and effective, with a good compliance in children.

Objective:To investigate the clinical characteristics and treatment of bronchiolitis obliterans syndrome (BOS) after hematopoietic stem cell transplantation (HSCT) in children.Methods:Clinical data of 7 patients with BOS after HSCT in the Department of Hematology and Oncology, Children′s Hospital, the First Affiliated Hospital of Zhengzhou University from September 2015 to June 2019, who had a survival of longer than 100 days were retrospectively analyzed.Results:At the last follow-up visit, the incidence of BOS was 4.6%(7/152 cases), including 5 males and 2 females.The median time from HSCT to the diagnosis of BOS was 15 (9-27) months.Among the 7 cases, 5 cases had dry cough and shortness of breath after activity, and 2 cases had no obvious clinical symptoms.Pulmonary function was moderate in 5 cases and severe in 2 cases of obstructive ventilatory disorder.High-resolution CT showed mosaic sign in 5 cases and bronchial wall thickening in 4 cases.Bronchoalveolar lavage (BAL) was performed in 4 cases, and flocculent secretion was found in the bronchus.Membranous substance was formed in the bronchus in 3 cases, and some lumens were completely occluded and dredged by foreign body forceps.After treatment with Fluticasone, Azithromycin and Montelukast sodium (FAM regimen), the pulmonary function of 5 cases(71.4%) was significantly improved, but ineffective in 2 cases.Conclusions:BOS after HSCT in children mainly begins with dry cough and shortness of breath after activity.Regular screening of pulmonary function is beneficial to identify asymptomatic children.BAL can clear inflammatory cytokines, which is conductive to the following drug treatment.If necessary, foreign forceps should be used to dredge the occluded bronchus to relieve symptoms quickly.FAM regimen is an effective treatment method, and timely adjustment of treatment according to the disease situation can improve the prognosis.

Objective:To investigate the clinical value of the expression levels of biological protein markers regenerating islet-derived protein 3-alpha(REG3α), soluble tumor suppressor factor 2(sST2) and tumor necrosis factor receptor 1(TNFR1) in peripheral blood in the diagnosis and efficacy evaluation of intestinal acute graft-versus-host disease (aGVHD) in children after allogeneic hematopoietic stem cell transplantation (allo-HSCT).Methods:Retrospective analysis of 50 children who underwent allo-HSCT, in the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from January 2020 to February 2021 were enrolled, including 39 males and 11 females [median age: 8.5 (1-13) years]. The expression levels of above 3 biological proteins were detected before transplantation, 1 week, 2 weeks, 3 weeks, 5 weeks, 7 weeks, 9 weeks, 11 weeks and 13 weeks after transplantation, when intestinal aGVHD occured, and after treatment.Children with intestinal aGVHD were taken as the observation group, and children without intestinal aGVHD were taken as the control group.Whether differences in the expression levels of the 3 biological proteins in the peripheral blood of the 2 groups of children were statistically significant was analyzed.The receiver operating characteristic(ROC) curve was used to evaluate the diagnostic value of the above three biological proteins for intestinal aGVHD, and independent sample t test was performed to compare the expression levels of the 3 biological proteins before and after treatment in children with intestinal aGVHD. Results:(1) The concentrations of REG3α, sST2, and TNFR1 in the peripheral blood of the observation group were (33 985.42±24 631.33) ng/L, (139 899.66±115 825.65) ng/L, (3 041.65±2 418.72) ng/L, respectively, which were higher than the control group of (7 457.39±4 547.49) ng/L, (32 059.57±23 452.85) ng/L, (1 944.51±1 170.35) ng/L, the difference was statistically significant ( t=6.04, 5.19, 2.17, all P<0.05). (2) The area under ROC curve (AUC) of REG3α combined with sST2 in the diagnosis of intestinal aGVHD was 0.952 (95% CI: 0.851-0.992, P<0.001), the maximum Youden index was 0.894, the corresponding sensitivity was 83%, and the specificity was 99%.Its diagnostic value was better than REG3α, sST2 and TNFR1 ( Z=1.763, 1.332, 3.001, all P<0.05). (3) The concentrations of REG3α, sST2, and TNFR1 before treatment in the peripheral blood of children having received effective treatment were (31 343.01±25 364.71) ng/L, (146 629.52±110 501.04) ng/L and (2 489.00±859.70) ng/L, respectively, which were (12 104.37±11 704.60) ng/L, (93 539.55±81 920.93) ng/L and (2 048.15±813.47) ng/L after treatment, lower than those before treatment.The expression levels of REG3α and sST2 were significantly reduced ( t=-3.23, -2.10, all P<0.05), while the difference of the expression level of TNFR1 before and after treatment was not statistically significant ( P>0.05). Conclusions:REG3α and sST2 can be used as important reference indicators for clinical auxiliary diagnosis of intestinal aGVHD, and have good auxiliary diagnostic value.REG3α and sST2 can be used as objective indicators to evaluate the efficacy of clinical treatment of intestinal aGVHD.

Objective:To investigate the mechanism of programmed death 1(PD-1)/ programmed death ligand 1(PD-L1) signaling pathway and its feasibility as a potential therapeutic target and prognostic predictor by detecting the expressions, of PD-1 and PD-L1 in bone marrow mononuclear cells of children with acute lymphoblastic leukemia (ALL), and to provide new ideas for the diagnosis and treatment of ALL as well.Methods:Bone marrow samples were collected from 59 children with ALL in the First Affiliated Hospital of Zhengzhou University from September 2018 to July 2019.Flow cytometry was applied to detect the expression of PD-1 and PD-L1 in bone marrow mononuclear cells in 59 ALL patients, including 47 newly-diagnosed ALL patients and 12 relapsed ALL patients, respectively, at initial diagnosis, after induction therapy and early intensive treatment.Their relevant clinical data were collected and compared with the bone marrow specimens of 12 children suffering from non-malignant blood diseases as the control group of the same hospital during the same period.Results:There was no significant difference in the expression of PD-1 in the bone marrow mononuclear cells of the primary diagnosis group, recurrence group and control group ( H=2.402, P>0.05). The expression of PD-L1 in the relapsed and refractory group [(7.32±3.60)%] and the newly diagnosed group [(3.18±2.37)%] was higher than that in the control group [(0.84±0.39)%], and the differences were statistically significant ( H= 28.048, P<0.05). In the initial treatment group, the expression of PD-L1 in the bone marrow mononuclear cells was the strongest expression before treatment ( B=1.293), followed by after induction treatment ( B=0.036) and after early intensive treatment ( B=0.000), suggesting that there was a downward trend as the continued treatment.The expression of PD-L1 was the weakest expression in the low-risk group ( B=-3.912) than in the medium-risk group ( B=-3.595) and high-risk group ( B=0.000), revealing that the expression of PD-L1 is related to the risk grades of ALL.The higher the risk rating is, the higher the PD-L1 protein expression is. Conclusions:The high expression of PD-L1 may be involved in the pathogenesis and be used as an adverse predictor of ALL childhood and an evaluation index of chemotherapy efficacy.PD-1 / PD-L1 signaling pathway may be a potential therapeutic target of ALL childhood.

Objective:To explore the clinical efficacy and safety of Ruxolitinib, a Janus kinase inhibitor, in combination with Methylprednisolone as a bridge to allogeneic hematopoietic stem cell transplantation (allo-HSCT) for relapsed/refractory Epstein-Barr virus-associated hemophagocytic syndrome (EBV-AHS) in pediatric patients.Methods:The clinical data of 4 patients with relapsed/refractory EBV-AHS treated with Ruxolitinib in combination with Methylprednisolone as a bridge to allo-HSCT at the Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University from August 2018 to February 2020 were retrospectively analyzed, and the disease characteristics, diagnosis and treatment process, clinical experience and related research progress were analyzed and summarized.Results:Among 4 patients with relapsed/refractory EBV-AHS, 2 patients were treated with low-dose Ruxolitinb in combination with Methylprednisolone for 6-10 weeks after partial remission.The disease did not progress, and they survived after being bridged to allo-HSCT.One patient was treated with large-dose Ruxolitinib in combination with Methylprednisolone due to the intolerance to chemotherapy, with the biochemical indicators of hemophagocytic syndrome significantly improved, and then the bridging to allo-HSCT was performed 2 months ago and this patient survived.One patient with EBV-AHS relapsed was relieved by chemotherapy again, then was given maintenance therapy with Ruxolitinib and Methylprednisolone, but the condition still progressed and the treatment was ineffective.This patient underwent allo-HSCT for salvage treatment more than 1 year ago and survived.Except that 1 patient developed mild anemia, the other 3 patients had no significant Ruxolitinib-related toxicities.Conclusions:Ruxolitinib in combination with Methylprednisolone can be safely employed as a salvage treatment for pediatric patients with relapsed/refractory EBV-AHS and a bridge to allo-HSCT, which has favorable safety, efficacy and tolerance in clinical practice.

Objective:To report our experience in the admission and perioperative management of 88 patients with lower extremity fracture in a mildly affected area in the epidemic of COVID-19.Methods:A retrospective analysis was conducted of the 88 patients with 97 lower extremity fractures who had been admitted to Department of Orthopedic Trauma, Honghui Hospital Affiliated to Xi'an Jiaotong University College of Medicine from 23rd January, 2020 to 22nd February, 2020. They were 43 males and 45 females, aged from 15 to 95 years (average, 65.5 years). The patients underwent screening for COVID-19 infection before admission. Their fractures were located at the femoral neck in 33 cases and at the femoral trochanter in 26. Open reduction and internal fixation was performed for 29 cases, internal fixation with proximal femoral nail anti-rotation (PFNA) for 25 and hip replacement for 28. The time from injury to admission, time from admission to surgery, operation time, fracture reduction, hospital stay, and perioperative deep venous thrombosis (DVT) of lower limbs were recorded. COVID-19 infection was observed in the medical staff and patients as well.Results:All the 88 patients were COVID-19 negative in the screening before admission. The time from injury to admission averaged 4.5 days, the time from admission to surgery 3.7 days and hospital stay 6.6 days. The prostheses were all well located in the 28 patients undergoing hip replacement. The rate of functional and anatomic reduction was 94.2%(65/69) in the 69 patients undergoing internal fixation. Peri-operatively, DVT occurred in 25 cases (28.4%). High temperature was observed in 7 patients within 3 days after operation, which was diagnosed as absorption fever. No medical staff or patients were infected by COVID-19.Conclusion:In the epidemic of COVID-19, orthopedic surgeons in a medical institute in a mildly affected area can still provide effective and safe medical services for fracture patients and reduce nosocomial infection, as long as they comprehend the diagnosis and treatment guidelines for the epidemic, strictly screen the patients accordingly, stick to operative indications, protect against possible infection cautiously, and carry out the procedures in a standard manner.