OBJECTIVE: To analyze the clinical characteristics of patients with 11q23 rearrangement acute lymphoblastic leukemia (ALL) with non-KMT2A::AFF1 fusion genes. METHODS: The clinical data of 10 patients with KMT2A fusion gene positive and partner gene non-AFF1 ALL admitted to Henan Cancer Hospital from December 2016 to December 2024 were retrospectively summarized. The immunophenotype, molecular genetic characteristics, clinical manifestations and disease prognosis of these patients were analyzed. This research has been approved by the Medical Ethics Committee of Henan Cancer Hospital (Ethics No.: 2019342). RESULTS: Among the 10 patients, the fusion genes were KMT2A::MLLT1 in 7 cases, KMT2A::MLLT4, KMT2A::MLLT3 and KMT2A::MLLT10 in 1 case each. The European Group for the Immunological Classification of Leukemias (EGIL) classification included 6 cases of T-ALL, 2 cases of pro-B-ALL, 1 case of Common-B-ALL and 1 case of pre-B-ALL. 4 cases of B-ALL all expressed CD19, cCD79a, CD38 and HLA-DR, and some expressed CD34 and CD22, without expression or weak expression of CD10, without expression of CD20. One case was accompanied by myeloid marker CD15 expression. 6 cases of T-ALL all expressed CD34, CD7, most expressed CD38, and some expressed CD3, CD5, CD2, CD4 and CD8, and 1 case expressed CD4 and CD8 together. Chromosomal abnormalities were detected in 3 cases, 5 cases were positive for WT1 fusion gene, and 6 cases had gene alterations. 9 patients achieved the first complete remission (CR1) during chemotherapy, and 1 patient relapsed within 6 months after CR1. At the last follow up, 1 patient (the fusion gene was KMT2A::MLLT4) remained unrelieved. There were 2 cases of KMT2A rearrangement (KMT2A-r) persistent positive (+/+) and 8 cases of KMT2A-r negative (+/-). The overall survival (OS) rate and leukemia-free survival (LFS) rate of patients with KMT2A-r persistent positive were significantly lower than those of patients with negative change, and the differences were statistically significant (P values were all < 0.05). Among the 3 patients who received chemotherapy+allogeneic hematopoietic stem cell transplantation (allo-HSCT), no relapse was observed until the follow up day. The OS rate and LFS rate of patients with KMT2A::MLLT1 and chemotherapy+allo-HSCT were higher than those of non-KMT2A::MLLT1 and single chemotherapy patients, and the differences were not statistically significant (P values were all ≥ 0.05). There was no significant difference in OS rate and LFS rate between T-ALL and B-ALL patients (P values were all ≥ 0.05). The median LFS time of the 10 patients was 32 (0 ~ 100) months, and the median OS time was 36 (1 ~ 101) months. CONCLUSION The 11q23 rearrangement ALL with non-KMT2A::AFF1 transcript is mainly KMT2A::MLLT1, T-ALL is more common, and the rate of chromosomal karyotype detection is relatively low. Persistent positive KMT2A-r is unfavorable for patient survival, and allo-HSCT during the CR1 period may improve patient survival.
Humans ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics* ; Female ; Male ; Myeloid-Lymphoid Leukemia Protein/genetics* ; Histone-Lysine N-Methyltransferase/genetics* ; Adult ; Adolescent ; Chromosomes, Human, Pair 11/genetics* ; Child ; Transcriptional Elongation Factors/genetics* ; Gene Rearrangement ; Oncogene Proteins, Fusion/genetics* ; Retrospective Studies ; Young Adult ; Middle Aged ; Prognosis ; Child, Preschool ; DNA-Binding Proteins/genetics*

Objective To investigate influencing factors for failed cardiopulmonary resuscitation(CPR)in patients with respiratory and cardiac arrest.Methods A retrospective analysis was con-ducted on the clinical data of 204 patients with respiratory and cardiac arrest.All patients underwent CPR treatment and were divided into success group and failure group based on the CPR outcome.A matched case-control study based on matching design was carried out.The success and failure groups were matched at a ratio of 1 to 2 using gender,age,body mass index(BMI),and the presence or absence of hypertension,hyperlipidemia,and diabetes as covariates.Logistic regression analysis was employed to explore the influencing factors for failed CPR in patients with respiratory and cardiac ar-rest.Results Among the 204 patients,65(31.86％)had successful CPR and were included in success group,while 139(68.14％)had failed CPR and were included in failure group.After a rati-o of 1 to 2 matching design,62 patients in the success group and 124 patients in the failure group were finally included in the study.Multivariate logistic regression analysis revealed that cardiovascular disease as the cause of respiratory and cardiac arrest,CPR performed outside the hospital,a long time interval from the onset of the condition to the initiation of CPR,and a large dose of epinephrine were independent risk factors for failed CPR(P＜0.05).In contrast,the combined use of a bag-mask de-vice and endotracheal intubation during respiratory and cardiac arrest,a prolonged duration of CPR,and electrical defibrillation were independent protective factors for successful CPR(P＜0.05).Con-clusion Cardiovascular disease,out-of-hospital CPR,a long time interval from the onset of the condi-tion to the initiation of CPR,and a large dose of epinephrine are risk factors for failed CPR in patients with respiratory and cardiac arrest.The combined use of a bag-mask device and endotracheal intubation during respiratory and cardiac arrest,prolonging the duration of CPR,and electrical defibrillation are protective factors for successful CPR.The matched case-control study method based on a matc-hing design can reduce the interference of confounding factors,ensure the reliability of the results,and provide a reliable basis for the formulation of CPR intervention protocols.

Objective:To delineate the surgical methodology and therapeutic paradigm of proximal tibial notch retrograde interlocking intramedullary nailing for ameliorating deformities due to osteofibrous dysplasia (OFD) in a pediatric population.Methods:A retrospective assessment was conducted on the medical records of individuals undergoing orthopedic osteotomy complemented by retrograde interlocking intramedullary nailing for OFD of the tibia from January 2016 to December 2019. The cohort comprised 15 patients, with a follow-up exceeding three years, documenting complete data sets. The patient profile included 8 males and 7 females, with 8 left-side and 7 right-side afflictions. The mean age at the time of surgery was 10.1±2.5 years, ranging from 7.1 to 12.6 years. Parameters measured were preoperative and postoperative imaging findings, which encompassed the scope of the lesion (longitudinal lesion length relative to tibial length), coronal and sagittal limb alignments, and lower limb length discrepancies.Results:The mean follow-up duration was 3.4±1.3 years, ranging from 3 to 6.6 years. Preoperatively, prominent anterior tibial arch deformities and limping were present, with 7 cases reporting fatigue-induced pain and 3 instances of pathological fractures. Post-surgery, pain symptoms were resolved, gait disturbances were improved in 9 patients, and completely resolved in 6. Tibial osteotomy or fracture healing of 15 patients averaged 3.9±0.7 months (range 3-5 months). The lesion range before surgery was 0.41±0.17, immediately after surgery was 0.38±0.17, and at the last follow-up was 0.30±0.16, with no statistical significance ( F=0.101, P=0.904). Lesion range showed no significant change throughout treatment, but radiographic density within the lesion notably increased post-surgery, suggesting bone improvement. The anterior tibial arch Angle was 28.30°±6.62° (range 20°-45°) before surgery, 4.73°±1.53° immediately after surgery, and 6.87°±1.36° at the last follow-up, with statistical significance ( F=159.739, P<0.001). A significant correction in the anterior tibial arch deformity was achieved and maintained postoperatively. There was no significant angular deformity of the tibia in the coronal plane before operation, and the medial proximal tibial angle (MPTA) and lateral distal tibial angle (LDTA) were 87.50°±1.46° and 88.30°±1.62°, 88.40°±1.46° and 88.70°±1.45° immediately after surgery, and 88.00°±1.39° and 89.10°±1.53° at the last follow-up, the differences were statistically significant ( F=1.741, P=0.188; F=1.016, P=0.371), there was no coronal deformity of tibia. The limb length discrepancy (LLD) was 0.60±0.98 cm before surgery, 0.18±0.93 cm at the last follow-up, with statistical significance ( t=0.096, P=0.761). There were no incidents of postoperative complications such as infection. Conclusion:In pediatric cases of tibial deformities attributed to osteofibrous dysplasia, a therapeutic strategy involving osteotomy for lower limb realignment, sans curettage or bone grafting of the lesion, followed by retrograde interlocking intramedullary nailing, yields favorable outcomes. Importantly, this implantation technique does not compromise the integrity of the proximal tibial epiphyseal plate in children and adolescents.

Objective:To explore the clinical effect of plating after lengthening to assist the consolidation of tibial shortening deformity in children.Methods:A retrospective analysis was conducted on 10 children with tibial shortening who were treated with circular external fixator lengthening and replacement with plate-assisted internal fixation (study group) in the Department of Pediatric Orthopedics of Tianjin Hospital from November 2019 to October 2022, and 16 children who were treated by circular external fixator only during the same period were enrolled as the external fixator group (control group). Among the 26 cases, there were 15 males and 11 females, 10 left knees and 16 right knees. The average age at the time of surgery was 8.8±4.7 years (range 5.1-13.8 years). The gender, side, age at the time of surgery were compared between the two groups. The extension length, external fixator index, healing index, Kolcaba comfort scale score, knee and ankle joint range of motion, complications and average total hospitalization costs were recorded.Results:There were no significant differences in gender, side, age at the time of surgery between the study group and control group. The extended lengths were 5.44±1.25 cm and 5.78±1.11 cm respectively without significant difference ( t=0.096, P=0.096). The external fixator index and healing index were 17.86±2.94 d/cm vs. 50.97±7.03 d/cm and 40.94±6.63 d/cm vs. 45.24±5.98 d/cm in study group and the control group with significant differences ( t=13.299, P<0.001; t=1.289, P=0.033). The Kolcaba comfort scale score of the children in the study group was higher than that of the control group, with significant difference ( t=6.821, P=0.001). Comparing the range of motion of the knee and ankle joints between the two groups, there were no significant differences before surgery, at the end of extension surgery, and at the final follow-up. When the extension end was healed, the range of motion of the knee joint was137.89°±4.40° vs. 114.09°±13.60° and ankle joint was 64.35°±5.50° vs. 56.65°±8.86° in the study group and control group with significant difference ( t=17.235, P<0.001; t=7.821, P=0.002). In the study group, 4 cases had pin tract infection, but no refracture occurred; in the control group, pin tract infection occurred in 13 cases, and refracture occurred in 2 cases after removal of the external fixator. The average total hospitalization cost of the study group was higher than that of the control group with significant difference ( t=3.745, P=0.036). Conclusion:The clinical effect of replacing plate-assisted internal fixator during the mineralization period of tibial shortening in children is reliable, and can significantly shorten the time for using external fixator. It is beneficial to the healing of the extended end of the osteotomy and the functional recovery of the knee and ankle joints. Being more comfortable for children with fewer complications, it is applicable for children who need long-distance extension, multiple extensions with limited joint function, poor tolerance for external fixators and low treatment compliance.

Objective:To explore the clinical efficacy of composite pin-rod external fixation in the treatment of open tibial fractures in children.Methods:The clinical data were retrospectively analyzed of the 21 pediatric patients with open tibial fracture who had been treated at Department of Pediatric Orthopeadics, Tianjin University by composite pin-rod external fixation from January 2019 to December 2021. There were 15 boys and 6 girls, and 9 left sides and 12 right sides. The age at the time of operation was (8.6±4.1) months. According to the Gustilo-Anderson classification, there were 3 cases of type Ⅰ, 12 cases of type Ⅱ, 3 cases of type ⅢA, 1 case of type ⅢB, and 2 cases of type ⅢC. Twenty cases had a concomitant fibular fracture, 3 cases multiple injuries, and 3 cases a segmental fracture. All the operations were performed at the emergency department. After debridement, the fractures were reduced and immobilized with a triangle external fixation of composite pin-rod system. In the patients whose conditions permitted, the wounds were closed at one stage, and no other external fixation was added after operation. Wound healing, fracture healing, frame time, knee and ankle functional results and complications were evaluated.Results:All patients were followed up for (20.7±15.8) months. Primary wound closure was achieved in 18 cases; the wound was covered by vacuum sealing drainage after debridement and reverse skin removal in the other 3 cases, of which 2 healed smoothly, and 1 was complicated with superficial infection of Gustilo type ⅢC treated eventually by an anterolateral thigh flap. Fractures healed satisfactorily in 20 cases, with a frame time of (4.5±1.7) months. At the last follow-up, there was no significant difference in the range of motion of the knee and ankle joints on the affected side compared with the uninjured side ( P>0.05). According to the Johner-Wruhs evaluation criteria, the functional recovery was excellent in 16 cases and good in 4 cases. Fracture nonunion happened in one child after skin flap operation, but fracture occurred again after reoperation for fracture, yielding a poor Johner-Wruhs evaluation. Needle tract infection was found in 9 cases but responded to therapy with no other complications. Conclusions:In the treatment of open tibial fractures in children, the composite pin-rod external fixation may lead to satisfactory clinical outcomes. It is easy in operation, provides stable fixation, and allows early rehabilitation after surgery. It can be used as a terminal treatment for wounds that can be closed at one stage.

Objective:To investigate the efficacy of open exploration, ulnar osteotomy and annular ligament reduction (three-in-one surgery) in the treatment of chronic Monteggia fracture in children.Methods:The data were analyzed retrospectively of the 35 children with chronic Monteggia fracture who had been admitted to Department of Pediatric Orthopaedics, Tianjin University from June 2017 to September 2021. There were 22 boys and 13 girls, and 19 left and 16 right sides, with an age of (6.8±2.5) years. Anterolateral dislocation of the radial head occurred in 30 cases and anterolateral dislocation in 5 ones. The time from injury to operation was (17.9±9.9) months. All patients were treated with three-in-one surgery. The elbow flexion and extension, forearm rotation and The Hospital for Special Surgery (HSS) elbow score were measured and compared in all patients at 12 months after operation. Their complications were also recorded.Results:All children were followed up for 12 months. The osteotomy ends of the ulna achieved bony union 1.5 to 4.0 months after operation. The elbow extension (-1.0°±5.9°) and flexion (128.5°±4.9°) at 12 months after operation were significantly improved compared with those before operation (-9.3°±18.0° and 108.4°±17.3°) ( P<0.05). The forearm pronation (61.5°±19.4°) at 12 months after operation was significantly limited compared with that before operation (72.7°±22.4°) ( P<0.05). There was no significant difference between the forearm supination (86.7°±4.5°) at 12 months after operation and that before operation (81.0°±17.4°) ( P>0.05). The HSS elbow score at 12 months after operation (93.5±5.2) was significantly higher than that (80.6±9.3) before operation ( P<0.05). The efficacy evaluated by the HSS elbow score at 12 months after operation was excellent in 21 cases, good in 12 cases, and fair in 2 cases, giving an excellent and good rate of 94.3% (33/35). Postoperative re-subluxation was observed in one patient. Conclusion:Open exploration, ulnar osteotomy and annular ligament reduction are a safe and effective three-in-one surgery for chronic Monteggia fracture in children, because it may lead to significantly improved elbow flexion and extension after operation, though the forearm pronation may be limited.

Objective:To explore the method of developing a canal-shaped implant template using a combined automatic pre-planning and 3D printing technology, and assess its impact on dose and efficacy improvement.Methods:Retrospective analysis of 15 patients with advanced cervical cancer admitted to the Affiliated Cancer Hospital of Guangxi Medical University from September 2020 to September 2022 was performed. Patients had characteristics such as vaginal stump recurrence, tumor eccentric growth, and previous hysterotomy, etc. Three-dimensional images were obtained by CT scan after automatic pre-planning. The PMT 3D software was used to analyze digital imaging and communications in medicine (DICOM) radiotherapy data, capture the coordinates of the pre-planned stay points to establish the implant channel, and generate the size and shape of the canal-shaped implant template based on patients' physiological structure. Dosimetric parameters, such as conformity index (CI), were evaluated. The changes of tumor size before and after treatment were analyzed by paired t-test. Results:Fifteen patients were treated with the canal-shaped implant template. The CI was 0.74±0.26, the total radiation dose (HR-CTV) D 90% (EQD 2, α/β=10) was (85.5±6.8) Gy, and the D 2 cm3 (EQD 2, α/β=3)for bladder, rectum, small intestine, and colon were (72.2±4.2), (65.8±6.1), (65.2±4.4), and (69.8±3.7) Gy, respectively, meeting clinical needs. After the treatment, the tumor volume was significantly decreased. The template had a good fit with the vaginal cavity, and a small amount of air gap on the sidewall did not affect the dose. Non-parallel needle insertion increased the utilization of the cavity space and implant needles. Conclusion:The method of developing the canal-shaped implant template using automatic pre-planning and 3D printing technology is efficient and effective, meets the requirements of modern precise radiotherapy, and has practical clinical value.

Objective:To retrospectively analyze and evaluate the effect of interlocking intramedullary nail in the treatment of proximalfemoral fibrous dysplasia (FD) and to discuss its application techniques and treatment strategies.Methods:The patients with proximal femoral FD who received treatment with interlocking intramedullary nail in our department from April 2014 to August 2018 were retrospectively reviewed. The inclusion criteria included having complete data with more than 3 years follow-up. A total of 31 cases with 32 thighs were included in the present study. Among them, there were 24 males and 7 females. The mean age at operation was 11.4±2.3 years. The mean follow-up duration was 3.9±1.1 years. The surgical methods were osteotomy or in situ interlocking intramedullary nailing, and femoral neck reconstruction nails pass through the epiphyseal plate in some cases. Preoperative, postoperative and final follow-up radiographic changes, including femoral neck shaft angle, proximal femoral diaphyseal deformity, and femoral alignment were compared. Univariate analysis of the influencing factors of postoperative femoral neck shaft angle loss.Results:In this group, 13 patients had coxa varus deformity, and the angle of the neck shaft was 107.3±7.1°, and 23 patients had the bending deformity of the proximal femur, and angled 29.5°±9.1°. 10 cases of coxa varus combined with proximal femoral diaphyseal deformity received double osteotomy and internal fixation, 17cases of simple coxa varus or diaphyseal deformity received single osteotomy and internal fixation, and 5 cases had no significant deformity, underwent in situ intramedullary nailing due to pathological fractures and pain. Postoperative femoral neck shaft angle was 133.2°±5.2°. Complete correction of proximal femoral flexion deformity was obtained and the diaphyseal axis was within the normal range. At the last follow-up, the neck shaft angle of 7 patients (21.9%, 7/32) decreased from 133.6°±3.9° to 125.7°±3.4°. Univariate analysis showed that the proportion of neck shaft angle loss in cases with proximal femoral locking nails passing through the epiphyseal plate was significantly lower than that in the cases without (0 vs. 43.8%, χ 2=6.58, P=0.010). Conclusion:The application of interlocking intramedullary nails in the treatment of proximal femoral fibrous dysplasia can achieve good results, according to the situation of coxa varus or diaphysis bending deformity, single or double osteotomy can be performed. There is a risk of femoral neck shaft angle loss after surgery, which can be avoided by femoral neck reconstruction locking screw fixation crossing epiphyseal plate.

Objective:To evaluate the feasibility of CT volume rendering technology in the assessment of the physeal bar in children.Methods:A retrospective analysis of the relevant CT data of 20 patients with physeal bar from January 2019 to December 2021, 13 boys and 7 girls, age 9.94±2.91 years. The etiology included 17 cases of trauma, 1 cases of tumor, and 2 cases of unknown. The affected sites included 9 cases of distal femur, 5 cases of proximal tibia, 3 cases of distal tibia, and 3 cases of distal radius. Evaluate using volume rendering technology and traditional surface reconstruction technology respectively and the index include the size and type of physeal bar.Results:All the children were successfully examined at one time, and none of them required sedation. The average exposure time was 3.81±0.83 s. During scanning, only low-dose radiation was performed on the affected joints, and routine radiation protection was performed. Using volume rendering technology to evaluate the proportion of the physeal bar, the results obtained by the three testers were 25.36%±15.36%, 24.75%±16.18%, 26.70%±17.72%, and the intraclass correlation coefficient value ( ICC) was 0.976, the three repeated measurements by one tester were 25.36%±15.36%, 25.41%±15.20%, 25.74%±16.00% ( ICC=0.990). Compared with the traditional curved planar reconstruction technical evaluation, the results obtained by the three testers were 28.36%±16.74%, 23.66%±19.87%, 35.25%±15.92% ( ICC=0.737), the three repeated measurement results by one tester were 28.36%±16.74%, 31.66%±13.06%, 30.89%±12.52% ( ICC=0.875). The volume rendering technology was better than the curved planar reconstruction technique. Paired t test was performed on the measurement results of three evaluators and the three repeated measurements of the same evaluator, and the differences were statistically significant ( P<0.05). The same results were acquired by using volume rendering technology to evaluate the type of phseal bar with three testers; but there were 18 cases had the same results by using curved planar reconstruction technology and 2 cases had differences, showing the volume rendering technology was more accurate. Conclusion:Low-dose CT scanning volume rendering technology is an effective method to evaluate the physeal bar in children. It can more intuitively and accurately evaluate the proportion and type of the bar while reducing the radiation exposure of children. The consistency with inter-group and intra-group is better than traditional curved planar reconstruction technique with good reproducibility and clinical significance.

OBJECTIVE To study the effect of Longsheng zhi capsule on lear ning and memory ability of vascular dementia model rats and explore its mechanism based on metabonomics. METHODS Totally 90 SD rats were randomly divided into sham operation group ，model group ，Dihydroergotoxin mesylate tablet group （positive control ，0.54 mg/kg），Longshengzhi capsule high-dose，medium-dose and low-dose groups （2.16，1.08 and 0.54 g/kg），with 15 rats in each group. In addition to sham operation group （only threading without ligation ），the vascular dementia model was prepared by permanent ligation of bilateral common carotid arteries in each group ，which was administered by gavage for 28 d. Morris water maze test was used to determine the learning and memory ability of rats ；hematoxylin eosin （HE）staining was used to observe the histopathological changes of hippocampus；the serum levels of superoxide dismutase （SOD），malondialdehyde（MDA）and glutathione peroxidase （GSH-Px） were detected ；the serum metabolic map was analyzed by ultra high performance liquid chromatography quadrupole time of flight tandem mass spectrometry （UPLC-Q-TOF/MS），the specific metabolites were screened by multivariate statistical analysis ，and the metabolic pathway was enriched and analyzed. RESULTS Morris water maze test showed that compared with model group ，the escape latency of rats in each administration group was significantly shortened ，the number of crossing the platform was significantly increased ，and the residence time in the target quadrant was significantly prolonged （P＜0.01 or P＜ 0.05）. The results of serum biochemical indexes showed that compared with model group ， the s erum level of SOD increased significantly in Dihydroergotoxine mesylate tablet group and Longshengzhi capsule high-dose group ，the serum level of GSH-Px increased significantly while the MDA level decreased significantly in each administration group （P＜0.05 or P＜0.01）. HE staining showed that Longshengzhi capsule could improve the histopathological damage of hippocampus in vascular dementia model rats. A total of 14 differential metabolites were screened and identified by UPLC-Q/TOF-MS and orthogonal partial least squares discriminant analysis model （VIP＞1 and P＜0.05）. The results of metabolic pathway enrichment analysis showed that the metabolic pathways involved in vascular dementia in rats mainly included vitamin B 6 metabolism，fatty acid metabolism and steroid hormone biosynthesis. CONCLUSIONS Longshengzhi capsule can improve the learning and memory ability of rats caused by vascular dementia. Its effect may be related to improving the oxidative stress injury caused by lipid accumulation in the process of vascular dementia. The metabolic pathways involved mainly include vitamin B 6 metabolism，fatty acid metabolism and steroid hormone biosynthesis.