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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.
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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.
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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 0.05).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 .
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To discuss the treatment effects and costs of surgery and Gamma Knife on hippocampal sclerosis [HS]-induced intractable epilepsy of children below age 10 years. The children below age 10 years who suffered from HS-induced intractable epilepsy from June 2010 to June 2012 were subjected to surgical and Gamma Knife treatments respectively according to their preference. The short-term curative rates of the surgical group and the Gamma Knife group were 93.51% and 54.87%, respectively. The average expenses of the two groups were 10,000 CNY [Chinese Yuan] and 22,000 CNY, respectively. The two groups were treated safely and effectively, but the surgical treatment led to better results at a reduced cost