OBJECTIVES: To investigate the clinical characteristics and prognosis of acute erythroleukemia (AEL) in children. METHODS: A retrospective analysis was conducted on the clinical data, treatment, and prognosis of 8 children with AEL treated at the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023. RESULTS: Among the 7 patients with complete bone marrow morphological analysis, 4 exhibited trilineage dysplasia, with a 100% incidence of erythroid dysplasia (7/7), a 71% incidence of myeloid dysplasia (5/7), and a 57% incidence of megakaryocytic dysplasia (4/7). Immunophenotyping revealed that myeloid antigens were primarily expressed as CD13, CD33, CD117, CD38, and CD123, with 4 cases expressing erythroid antigens CD71 and 2 cases expressing CD235a. Chromosomal analysis indicated that 2 cases presented with abnormal karyotypes, including +8 in one case and +4 accompanied by +6 in another; no complex karyotypes were observed. Genetic abnormalities were detected in 4 cases, with fusion genes including one case each of dup MLL positive and EVI1 positive, as well as mutations involving KRAS, NRAS, WT1, and UBTF. Seven patients received chemotherapy, with 6 achieving remission after one course of treatment; 2 underwent hematopoietic stem cell transplantation, and all had disease-free survival. Follow-up (median follow-up time of 6 months) showed that only 3 patients survived (2 cases after hematopoietic stem cell transplantation and 1 case during treatment). CONCLUSIONS Children with AEL have unique clinical and biological characteristics, exhibit poor treatment response, and have a poor prognosis; however, hematopoietic stem cell transplantation may improve overall survival rates.
Humans ; Male ; Female ; Prognosis ; Child, Preschool ; Retrospective Studies ; Child ; Leukemia, Erythroblastic, Acute/diagnosis* ; Infant ; Adolescent

OBJECTIVES: To investigate the clinical features and prognosis of children with non-Down-syndrome-related acute megakaryoblastic leukemia (non-DS-AMKL). METHODS: A retrospective analysis was conducted on the medical data of 17 children with non-DS-AMKL who were admitted to Children's Hospital of The First Affiliated Hospital of Zhengzhou University from January 2013 to December 2023, and their clinical features, treatment, and prognosis were summarized. RESULTS: Among the 17 children with non-DS-AMKL, there were 8 boys and 9 girls. Fourteen patients had an onset age of less than 36 months, with a median age of 21 months (range:13-145 months). Immunophenotyping results showed that 16 children were positive for CD61 and 13 were positive for CD41. The karyotype analysis was performed on 16 children, with normal karyotype in 6 children and abnormal karyotype in 9 children, among whom 5 had complex karyotype and 1 had no mitotic figure. Detected fusion genes included EVI1, NUP98-KDM5A, KDM5A-MIS18BP1, C22orf34-BRD1, WT1, and MLL-AF9. Genetic alterations included TET2, D7S486 deletion (suggesting 7q-), CSF1R deletion, and PIM1. All 17 children received chemotherapy, among whom 16 (94%) achieved complete remission after one course of induction therapy, and 1 child underwent hematopoietic stem cell transplantation (HSCT) and remained alive and disease-free. Of all children, 7 experienced recurrence, among whom 1 child received HSCT and died of graft-versus-host disease. At the last follow-up, six patients remained alive and disease-free. CONCLUSIONS Non-DS-AMKL primarily occurs in children between 1 and 3 years of age. The patients with this disorder have a high incidence rate of chromosomal abnormalities, with complex karyotypes in most patients. Some patients harbor fusion genes or gene mutations. Although the initial remission rate is high, the long-term survival rate remains low.
Humans ; Male ; Female ; Leukemia, Megakaryoblastic, Acute/etiology* ; Child, Preschool ; Infant ; Child ; Retrospective Studies ; Prognosis ; Down Syndrome/complications*

A moxibustion device with the functions of auricular fumigation moxibustion and heat-sensitive moxibustion is designed. The smoke of the ignited moxa stick is used for the fumigation moxibustion at the external auditory canal, while the heat generated works on Dazhui (GV 14) for heat-sensitive moxibustion. The device consists of five parts, i.e. combustion chamber, smoke pipe, smoke processing chamber, power module and connector. It solves the limitations such as unpleasant experience in treatment, unfavorable temperature control, easy scalding and excessive manual dependence induced by usual fumigation moxibustion and during heat-sensitive moxibustion. This moxibustion device may improve the safety and convenience when delivering the treatment with fumigation moxibustion and heat-sensitive moxibustion, as well as the work efficiency of medical staff.
Humans ; Moxibustion ; Hot Temperature ; Fumigation ; Smoke ; Temperature

An automatic ash-removal heat-sensitive moxibustion device was developed, which could keep relatively constant temperature of heat-sensitive moxibustion, and realize the automatic ignition and automatic ash removal of moxa sticks during heat-sensitive moxibustion. The automatic ash-removal heat-sensitive moxibustion device comprises a bracket and a moxibustion box fixed on the top of the bracket; the bracket is composed of a base and a movable telescopic arm. This device can solve the problems of temperature instability, moxa ash blocking heat transfer and moxa ash falling during heat-sensitive moxibustion, avoiding the scalding caused by moxa ash falling, and reduce the workload of medical staff.
Humans ; Hot Temperature ; Moxibustion ; Temperature

Objective To compare the perioperative efficacy and safety of da Vinci robot-assisted thoracoscopic surgery (RATS) for treatment of anterior mediastinal tumors through subxiphoid versus lateral thoracic approaches under the laryngeal mask anesthesia. Methods We retrospectively analyzed the clinical data of 102 patients with anterior-mediastinal tumors treated by RATS under laryngeal mask anesthesia completed by the same operator. Forty-five patients underwent the subxiphoid approach (subxiphoid group), and 57 patients were treated with the lateral thoracic approach (lateral thoracic group). The operating time, intraoperative bleeding, and total postoperative drainage volume in the two groups were compared and analyzed. Results All patients successfully completed resection of the anterior mediastinal tumor without the occurrence of perioperative death. In terms of total postoperative drainage volume, postoperative drainage time, postoperative hospital stay, and VAS pain on postoperative days 2 and 3, the subxiphoid group was more advantages (P < 0.05). No statistically significant difference was found between the two groups in terms of operative time, docking time, total operative time, intraoperative bleeding volume, postoperative day 1 VAS pain score, or postoperative complications (P > 0.05). Conclusion The subxiphoid approach of RATS is safe and feasible for resection of anterior mediastinal tumors. Compared with the lateral thoracic approach, the subxiphoid approach has advantages in terms of rapid postoperative recovery and postoperative pain.

Objective:To compare the clinical results of modified posterolateral approach combined medial approach versus traditional posterolateral approach combined with medial approach in open reduction and internal fixation of trimalleolar ankle fracture.Methods:A retrospective cohort study was used to analyze the clinical data of 46 patients with trimalleolar ankle fracture admitted to the First Affiliated Hospital of Naval Military Medical University from June 2013 to June 2019, including 14 males and 32 females, at age of 19-71 years [(49.2±14.9)years]. There were 33 patients with supination-external rotation type IV ankle fracture and 13 with pronation-external rotation type IV ankle fracture according to Lauge-Hansen classification. Open reduction and internal fixation was performed through the modified posterolateral approach combined with medial approach in 25 patients (modified approach group), and through the traditional posterolateral approach combined with medial approach in 21 patients (traditional approach group). The visual analogue score (VAS) at 3 days and 1 week after surgery, fracture healing time, range of ankle flexion and extension and Baird-Jackson score at the final follow-up and postoperative complications (numbness of the affect limb, wound necrosis, etc.) were compared between the two groups.Results:All patients were followed up for 11-21 months [(14.8±2.2)months]. There was no statistical difference in VAS or fracture healing time at 3 days after surgery between the two groups (all P>0.05). The VAS was 3.0 (3.0, 4.0)points in modified approach group at 1 week after surgery, significantly lower than 4.0 (3.0, 5.0)points in traditional approach group ( P<0.05). At the final follow-up, there was no statistical difference in range of ankle plantarflexion between the two groups ( P>0.05), but range of ankle dorsiflexion was significantly greater in modified approach group [(11.8±2.8)°] than that in traditional approach group [(8.1±3.5)°] ( P<0.01). At the final follow-up, Baird-Jackson score was not statistically different between the two groups ( P>0.05). There were 4 patients with numbness and 2 with wound necrosis in traditional approach group, but no numbness or wound necrosis occurred in modified approach group ( P<0.01). Conclusion:Both the modified posterolateral approach combined with medial approach and traditional posterolateral approach combined with medial approach can achieve good clinical results in open reduction and internal fixation of trimalleolar ankle fractures, but the former has advantages of better pain relief, better recovery of ankle dorsiflexion and less complications.

OBJECTIVE: To identify new biomarkers and molecular pathogenesis of Down syndrome (DS) by analyzing differentially expressed miRNAs in the placentas and their biological pathways. METHODS: Whole transcriptome sequencing was used to identify the differentially expressed miRNAs in DS (n=3) and normal placental samples (n=3) diagnosed by prenatal diagnosis. The target genes were predicted using miRWalk, Targetscan and miRDB, and GO and KEGG pathway analyses were performed for gene enrichment studies. RESULTS: We identified a total of 82 differentially expressed miRNAs in the placental tissues of DS, including 29 up-regulated miRNAs (fold change ≥2, P < 0.05) and 15 down-regulated miRNAs (fold change ≥2, P < 0.05), among which 10 miRNAs with relatively high expression abundance were selected for further analysis, including 4 up-regulated and 6 down-regulated miRNAs. These selected miRNAs shared the common target genes BTBD3 and AUTS2, both of which were associated with neurodevelopment. GO analysis showed that the target genes of the selected miRNAs were mainly enriched in protein binding, hydrolytic enzymes, metal ion binding protein combining, transferase activity, nucleotide, cytoplasmic constituents, nucleus composition, transcriptional regulation, RNA metabolism regulation, DNA-dependent RNA polymerase Ⅱ promoter transcriptional regulation, eye development, and sensory organ development. KEGG enrichment analysis showed that the target genes of these differentially expressed miRNAs were involved in the signaling pathways including tumor-related signaling pathway, PI3K-Akt signaling pathway, Ras signaling pathway, Rap1 signaling pathway, cytoskeletal regulatory signaling pathway, purine metabolization-related signaling pathway and P53 signaling pathway. CONCLUSION The differentially expressed miRNAs may play important roles in placental damage and pregnancy pathology in DS and provide clues for the prevention and treatment of mental retardation-related diseases.
Cytoskeletal Proteins/metabolism* ; Down Syndrome/metabolism* ; Female ; Gene Expression Profiling ; Humans ; MicroRNAs/metabolism* ; Nerve Tissue Proteins ; Phosphatidylinositol 3-Kinases/metabolism* ; Placenta/metabolism* ; Pregnancy ; Transcription Factors/metabolism* ; Transcriptome ; Whole Exome Sequencing

An 8-year-old female child was admitted to Children’s Hospital of Nanjing Medical University due to paroxysmal dyspnea on July 19, 2019. After thorough examination, she was considered to suffer from pulmonary lymphangioma and underwent an exploratory thoracotomy. The diffuse distribution of lymphangioma was seen during surgery, and part of the lymphangioma tissues was excised for histopathology. The patient was diagnosed as diffuse pulmonary lymphangiomatosis according to the history, CT imaging and postoperative pathology. She was treated with sirolimus at a starting dose of 0.9 mg every 12 h. Through monitoring the blood drug concentration regularly and adjusting the drug dose, the blood drug concentration was maintained at 10-15 ng/ml. Two weeks later, the child’s dyspnea improved but she developed refractory chylothorax, which was considered related to surgical wound. Considering that the child had thoracic drainage tubes for a long time, 10 mg of betamethasone, which was dissolved in 20 ml of physiological saline, was injected into each drainage tube to reduce the effusion of pleural effusion, and the drainage tubes were successfully removed after 3 injections. Unfortunately, the child’s symptoms recurred two months later, and she eventually died of respiratory failure despite active treatment, with a duration of nearly one year. The earlier the onset of diffuse pulmonary lymphangiomatosis occurs, the worse the prognosis is. Surgical resection may not be appropriate for such diffuse lesions, and there is a risk of refractory chylothorax, further research should explore the pathogenesis of the disease and targeted drug therapy.

Objective: To analyze the clinical characteristics of children with Burkitt lymphoma (BL) and to summarize the mid-term efficacy of China Net Childhood Lymphoma-mature B-cell lymphoma 2017 (CNCL-B-NHL-2017) regimen. Methods: Clinical features of 436 BL patients who were ≤18 years old and treated with the CNCL-B-NHL-2017 regimen from May 2017 to April 2021 were analyzed retrospectively. Clinical characteristics of patients at disease onset were analyzed and the therapeutic effects of patients with different clinical stages and risk groups were compared. Survival analysis was performed by Kaplan-Meier method, and Cox regression was used to identify the prognostic factors. Results: Among 436 patients, there were 368 (84.4%) males and 68 (15.6%) females, the age of disease onset was 6.0 (4.0, 9.0) years old. According to the St. Jude staging system, there were 4 patients (0.9%) with stage Ⅰ, 30 patients (6.9%) with stage Ⅱ, 217 patients (49.8%) with stage Ⅲ, and 185 patients (42.4%) with stage Ⅳ. All patients were stratified into following risk groups: group A (n=1, 0.2%), group B1 (n=46, 10.6%), group B2 (n=19, 4.4%), group C1 (n=285, 65.4%), group C2 (n=85, 19.5%). Sixty-three patients (14.4%) were treated with chemotherapy only and 373 patients (85.6%) were treated with chemotherapy combined with rituximab. Twenty-one patients (4.8%) suffered from progressive disease, 3 patients (0.7%) relapsed, and 13 patients (3.0%) died of treatment-related complications. The follow-up time of all patients was 24.0 (13.0, 35.0) months, the 2-year event free survival (EFS) rate of all patients was (90.9±1.4) %. The 2-year EFS rates of group A, B1, B2, C1 and C2 were 100.0%, 100.0%, (94.7±5.1) %, (90.7±1.7) % and (85.9±4.0) %, respectively. The 2-year EFS rates was higher in group A, B1, and B2 than those in group C1 (χ²=4.16, P=0.041) and group C2 (χ²=7.21, P=0.007). The 2-year EFS rates of the patients treated with chemotherapy alone and those treated with chemotherapy combined with rituximab were (79.3±5.1)% and (92.9±1.4)% (χ²=14.23, P<0.001) respectively. Multivariate analysis showed that stage Ⅳ (including leukemia stage), serum lactate dehydrogenase (LDH)>4-fold normal value, and with residual tumor in the mid-term evaluation were risk factors for poor prognosis (HR=1.38,1.23,8.52,95%CI 1.05-1.82,1.05-1.43,3.96-18.30). Conclusions: The CNCL-B-NHL-2017 regimen show significant effect in the treatment of pediatric BL. The combination of rituximab improve the efficacy further.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Burkitt Lymphoma/drug therapy* ; Child ; Disease-Free Survival ; Female ; Humans ; Lactate Dehydrogenases ; Lymphoma, B-Cell/drug therapy* ; Male ; Prognosis ; Retrospective Studies ; Rituximab/therapeutic use* ; Treatment Outcome

An 8-year-old female child was admitted to Children’s Hospital of Nanjing Medical University due to paroxysmal dyspnea on July 19, 2019. After thorough examination, she was considered to suffer from pulmonary lymphangioma and underwent an exploratory thoracotomy. The diffuse distribution of lymphangioma was seen during surgery, and part of the lymphangioma tissues was excised for histopathology. The patient was diagnosed as diffuse pulmonary lymphangiomatosis according to the history, CT imaging and postoperative pathology. She was treated with sirolimus at a starting dose of 0.9 mg every 12 h. Through monitoring the blood drug concentration regularly and adjusting the drug dose, the blood drug concentration was maintained at 10-15 ng/ml. Two weeks later, the child’s dyspnea improved but she developed refractory chylothorax, which was considered related to surgical wound. Considering that the child had thoracic drainage tubes for a long time, 10 mg of betamethasone, which was dissolved in 20 ml of physiological saline, was injected into each drainage tube to reduce the effusion of pleural effusion, and the drainage tubes were successfully removed after 3 injections. Unfortunately, the child’s symptoms recurred two months later, and she eventually died of respiratory failure despite active treatment, with a duration of nearly one year. The earlier the onset of diffuse pulmonary lymphangiomatosis occurs, the worse the prognosis is. Surgical resection may not be appropriate for such diffuse lesions, and there is a risk of refractory chylothorax, further research should explore the pathogenesis of the disease and targeted drug therapy.