Objective:To improve the understanding of acute lymphoblastic leukemia (ALL) secondary to Burkitt lymphoma (BL) in children.Methods:The clinical data of a child with ALL secondary to BL who was admitted to Children's Hospital Affiliated to Zhengzhou University in June 2024 were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient was a boy with the age of 8 years and 8 months. He presented with a neck mass at the age of 4 years and 6 months, and pathological examination revealed a diagnosis of BL with clinical stage Ⅲ. The patient was given regular chemotherapy according to the Chinese Children's Lymphoma Group non-Hodgkin lymphoma mature B-cell 2017 protocol-B2 regimen. PET-CT showed recurrence of lymphoma in 6 months after the suspension of treatment. The patient was given with placement of 125I particles, oral etoposide and dexamethasone, and traditional Chinese medicine. The patient was admitted to hospital at the age of 8 years and 8 months with fever and skin hemorrhagic spots, bone marrow morphology, immunology, cytogenetics and molecular biology typing indicated a diagnosis of B-ALL with TCF3::PBX1 fusion gene. The patient received induction chemotherapy according to the Chinese Children's Leukemia Group-ALL 2018 protocol. A review of bone marrow cytology achieved complete remission on the 33rd day of chemotherapy, and minimal residual disease detected by flow cytometry indicated less than 0.01%. TCF3::PBX1 fusion gene was negative. Conclusions:ALL secondary to BL in children is rare, and the ALL treatment regimens are effective.

Objective: To investigate the characteristics of rabies-exposed population in Wenzhou City, Zhejiang Province from 2014 to 2023, so as to provide insights into the prevention and control of rabies. Methods: Data of rabies-exposed population in Wenzhou City from 2014 to 2023 were collected through Wenzhou Rabies-Exposed Population Summary Sheet reported by dog injury clinics. The species of animals causing injuries, exposure time, exposure grade, exposure site, and post-exposure treatment were descriptively analyzed. Results: Totally 709 900 patients were admitted to dog injury clinics in Wenzhou City from 2014 to 2023, and the exposure rate showed an increasing trend (Z=7.238, P<0.001), with an average annual exposure rate of 750.75/105. The number of cases with exposure to rabies peaked in July (79 230 cases, 11.16%) and August (78 570 cases, 11.07%). Dogs were predominant animals causing injuries (448 900 cases, 63.23%), and the exposure rate showed a downward trend (Z=-5.921, P<0.001); cats were the second (175 142 cases, 24.67%), and the exposure rate showed an upward trend (Z=23.314, P<0.001). The upper (379 695 cases, 53.49%) and lower limbs (287 521 cases, 40.50%) were the main exposure sites. There were 21 034 cases (2.96%) exposed to head and face, and the exposure rate of head and face showed an upward trend (Z=3.549, P<0.001). Grade II exposure was the most common (403 881 cases, 56.89%), and the exposure rate showed an upward trend (Z=8.769, P<0.001). The proportion of using human rabies immune globulin was 23.13% in Grade III exposed population, showing a downward trend (Z=-12.848, P<0.001). Conclusions The exposure rate of rabies in Wenzhou City showed an upward trend from 2014 to 2023, with July and August as the peak months of exposure. Injuries mainly caused by dogs, while the exposure rate of cat bites showed an upward trend. The proportion of using human rabies immune globulin needs to be improved.

Objective To explore the potential influencing factors and complex pathways affecting rehabilitation effect for children with autism spectrum disorder(ASD)from the perspective of parental efficacy in doctor-patient communication,and to provide evidence for improving the quality of rehabilitation service for children with ASD. Methods An anonymous face-to-face questionnaire survey was conducted to collect general demographic information of parents of children with ASD at designated rehabilitation institutions in Gansu province.The data included paren-tal efficacy in doctor-patient communication,parental compliance of treatment and children's rehabilitation out-comes were collected.A structural equation model was used to explore the impact mechanism of parental commu-nication efficacy on the rehabilitation outcomes of children with ASD. Results Data from 519 parents at 37 rehabilitation institutions across 13 cities/counties in Gansu province were collect-ed.Significant positive correlations were found between parental efficacy in doctor-patient communication,doc-tor-patient relationships,parental compliance with treatment and rehabilitation outcomes for children with ASD(P＜0.05).Through structural equation model analysis,the standardized direct effect of children with ASD from parents'doctor-patient communication efficacy was 0.151(P=0.023).The mediating effect of doctor-patient rela-tionship and parental compliance with treatment were 0.160(P=0.001)and 0.111(P=0.001),respectively,with a chained mediating effect of 0.035(P=0.001).The pathway"parental efficacy in doctor-patient communica-tion → doctor-patient relationship → ASD child's rehabilitation outcome"accounted for the highest proportion of the total mediating effect,at 52.29%. Conclusion Parental doctor-patient communication efficacy may positively impact on the rehabilitation outcomes of chil-dren with ASD directly,and indirectly through the doctor-patient relationship and parental compliance with treat-ment.Rehabilitation institutions should focus on fostering parental communication skills and enhancing high-quality and humanized rehabilitation services.

Objective:To investigate the clinical characteristics of children with high-risk acute lymphoblastic leukemia (ALL) receiving lasting remission after severe infection.Methods:The data of 3 children with high-risk ALL who were treated in Children's Hospital Affiliated of Zhengzhou University in 2014, 2015 and 2017 were analyzed retrospectively. The clinical and laboratory characteristics of all patients were also analyzed, and the relevant literatures were reviewed.Results:All 3 children were clinically classified as high-risk ALL with severe infection. A variety of anti-infective drugs and blood products were used in the treatment, and all achieved lasting remission.Conclusions:Children with high-risk ALL after severe infection can acquire lasting remission, which may be related with the production of infection stimulating inflammatory factors and cytokines to activate certain immune pathways, or various kinds of antibiotics, blood products participating in the immune regulation to make the body regain the immune surveillance function of the tumor cells.

Protein neddylation is catalyzed by a three-enzyme cascade, namely an E1 NEDD8-activating enzyme (NAE), one of two E2 NEDD8 conjugation enzymes and one of several E3 NEDD8 ligases. The physiological substrates of neddylation are the family members of cullin, the scaffold component of cullin RING ligases (CRLs). Currently, a potent E1 inhibitor, MLN4924, also known as pevonedistat, is in several clinical trials for anti-cancer therapy. Here we report the discovery, through virtual screening and structural modifications, of a small molecule compound HA-1141 that directly binds to NAE in both

OBJECTIVES: To comprehensively analyze the characteristics of cognitive impairment of temporal lobe epilepsy (TLE), and to explore the effects of different lateral patients' cognitive impairment and different clinical factors on cognitive impairment of TLE. METHODS: A total of 84 patients, who met the diagnostic criteria for TLE in the Department of Neurology, Xiangya Hospital, were collected as a patient group, with 36 cases of left TLE and 48 cases of right TLE. A total of 79 healthy volunteers with matching gender, age and education level were selected as a control group. The Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), and the scores of Arithmetic Test, Information Test, Digit Symbol Substitution Test (DSST), Block Design Test (BDT), Hayling Test and Verbal Fluency Test (VFT) of the revised Chinese Adult Wechsler Intelligence scale were retrospectively analyzed in the 2 groups.Multiple regression analysis was used to analyze the relationship between the clinical factors and the cognitive impairment score. RESULTS: Compared with the control group, the TLE patient group had low scores in all neuropsychological tests, with significant difference (all CONCLUSIONS There are multiple cognitive domain dysfunctions in TLE, including language, short-term memory, long-term memory, attention, working memory, executive function and visual space function. Left TLE has greater impairment of executive function and right TLE has greater damage in working memory. Long pathography of disease, hippocampal sclerosis and a history of febrile convulsions may lead to more severe cognitive impairment. Earlier identification and earlier intervention are needed to improve prognosis of patients.
Adult ; Cognitive Dysfunction/etiology* ; Epilepsy, Temporal Lobe/complications* ; Executive Function ; Humans ; Neuropsychological Tests ; Retrospective Studies

OBJECTIVETo comprehensively analyze the clinical characteristics, treatment strategies and outcome of patients with thrombotic thrombocytopenic purpura (TTP).

METHODSA retrospective survey of 51 TTP patients confirmed in our database. Relevant statistical analyzes were performed by GraphPad Prism 5 software.

RESULTS51 cases of patients with acquired TTP were identified as idiopathic TTP. In our study, only 18 cases (35.29%) had typical pentalogy of TTP, where thrombocytopenia (100.00%), microangiopathic hemolytic anemia (92.16%) and neurologic abnormalities (88.24%) were more common than fever (72.55%) and renal abnormalities (70.59%). Plasma ADAMTS13 activity was detected in 37 patients with TTP with ADAMTS13 deficiency confirmed in 31 patients (83.78%). Plasma exchange with response of 72.3% was still the preferred strategy in TTP with individuation. Among 36 survival TTP patients, 8 patients (22.22%) relapsed. 15 patients (29.41%) died in our study. The mean ages of responders and deaths were of (37.5±14.5) and (50.1±18.9) respectively; whereas total bilirubin level of responders and deaths were of (43.3±23.5)μmol/L and (63.7±39.7) μmol/L respectively, the differences were statistically significant. Conversely, body temperature, WBC, HGB, PLT, serum creatinine and LDH showed no significant differences (P>0.05).

CONCLUSIONThe diagnosis of TTP was based on comprehensive analysis of clinical manifestations. Plasma ADAMTS13 activity test had a higher clinical practical value. The therapeutic alliance with corticosteroids, immunosuppressive agents and Rituximab significantly improved its outcome. The age and high total bilirubin level at onset were associated with less sensitive to plasmapheresis and poor prognosis.

ADAM Proteins ; blood ; ADAMTS13 Protein ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Plasma Exchange ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; therapy ; Retrospective Studies ; Young Adult

Objective To study the effect and safety of posterior Tenon subtenon triamcinolone acetonide (TA) injection as treatment for diffuse diabetic macular edema.Methods Forty-two patients (54 eyes) with diffuse diabetic macular edema were diagnosed by fundus fluorescence angiography and optical coherence tomography,posterior Tenon subtenon injection of TA 20 mg for treatment.All eyes were examined before treatment and the different time points after treatment by testing visual acuity,intraocular pressure,thickness of macular fovea.Results Preoperative visual acuity was 0.26 ± 0.15,postoperative was 0.29 ± 0.13,0.34 ± 0.14,0.43 ± 0.16,0.41 ± 0.13,0.38 ± 0.17 at 2 weeks,1 month,2 months,3 months and 6 months,respectively,visual acuity improved significantly,there was no significant difference between 2 weeks postoperative and preoperative (P ＞ 0.05),the remaining time points compared with preoperative differences were statistically significant (P ＜ 0.01).Preoperative thickness of macular fovea was (473.9 ±121.8) μ m,postoperative was (462.6 ± 93.3) μ m,(342.8 ± 87.1) μ m,(302.6 ± 67.8) μ m,(321.1 ±79.1) μ m,(355.2 ± 47.6) μ m at 2 weeks,1 month,2 months,3 months and 6 months,respectively,there was no significant difference between 2 weeks postoperative and preoperative (P ＞ 0.05),the remaining time points compared with preoperative differences were statistically significant (P ＜ 0.01).The intraocular pressure at 2 months were significantly higher than preoperative (P ＜ 0.05).No other complications were observed.Conclusion Posterior Tenon subtenon injection of TA as treatment for diffuse diabetic macular edema is safe and effective,which can inject repeatly.

Objective: To investigate the effects of Ginkgo biloba extract (EGb 761) on the morphology and function of retinal ganglion cells (RGC) in guinea pigs with optic nerve transection. Methods: Seventy-five albino guinea pigs were randomly divided into five groups: normal control group, sham-operated group, untreated group, normal saline group and EGb 761 group. No operation was performed in the normal control group. Optic nerve was merely exposed in the sham-operated group, but transected at 1.0 mm from posterior pole of the eye ball in the untreated, normal saline and EGb 761 groups. Guinea pigs in the EGb 761 group or the normal saline group received daily intraperitoneal injection of EGb 761 (100 mg/kg) or corresponding volume of normal saline from 7 days before experiment to 28 days after experiment. Three guinea pigs in each group were sacrificed for apoptosis assay (TUNEL method) of RGC. Pattern electoretinograms (PERGs) were recorded 14 and 28 days after transection, respectively. At the end of the examination, six guinea pigs were killed for histological examination and RGC count. Results: No TUNEL-positive cells were observed in the normal control, sham-operated and EGb 761 groups, but there were TUNEL-positive cells in the untreated group and the normal saline group. The numbers of RGCs in the untreated and normal saline groups were less than those in the normal control and sham-operated groups at 14 days or 28 days (P<0.05). Although the number of RGCs in the EGb 761 group was less than those in the normal control and sham-operated groups (P<0.05), it was more than those in the untreated and normal saline groups (P<0.05). N(95) amplitude in EGb 761 group was higher than those in the untreated and normal saline groups (P<0.05) and close to those in the normal control and sham-operated groups (P>0.05) at 14 days or 28 days. The number of RGCs was positive correlated to N(95) amplitude (r=0.859, P=0.001 5). Conclusion: EGb 761 can inhibit the apoptosis of RGCs in guinea pigs after optic nerve transection, thus protect the morphology and function of RGCs.

Objective　To study the correlation between the rabbit glucocorticoid receptor(GR) and steroid-induced glaucoma(SIG).Methods　The GR binding in peripheral blood leukocytes and plasma cortisol were measured in 15 adults pigmented rabbits on the first day of experiment, 0.5mg dexamethasone was injected in the right eyes of each rabbit once every 2 days, totally 15 times for 30 days. Right eyes intraocular pressure(IOP) and aqueous outflow facility were measured every week, the relation between GR, plasma cortisol, IOP and aqueous outflow facility(C) was analyzed. Results　After the experiment the IOP increased by 0.67kPa±0.49kPa(P＜0.01),C decreased by 0.08±0.11(P＜0.01).GR may closely be related to IOP and C(r=0.56,P＜0.025; r=0.60,P＜0.01).Plasma cortisol was independent of IOP and C(r=0.27, P＞0.05;r=0.34,P＞0.05).Conclusion　The level of GR has positive correlation with the sensitivity of tissue of eye to glucocorticoid.