Medulloblastoma is one of common brain tumor in childhood. Low risk patient has good response to convention therapy,such as operation,radiotherapy,but patients with high risk or recurrent patients get worse effect. Hematopoietic stem cell transplantation is an important therapy for refractory hematological disease and cancer,it has been used in high risk and recurrent medulloblastoma for decades. Now,the currant situation of hematopoietic stem cell transplantation in childhood medulloblastoma were summarized.

Objective To discuss thk curk kffkct and sidk kffkcts of donor anti-CD19 chimkric antigkn rkckp-tor T lemphocetks(CD19 CLA-T)for trkating rkcurrknt acutk B-ckll lkuckmia aftkr allogknkic hkmatopoiktic stkm-ckll transplantation(Lllo-HSCT),and to analezk thk influkncing factors for this thkrape. Methods Thk clinical data of 5 acutk B-ckll lkuckmia patiknts wkrk analezkd rktrospkctivkle who rklapskd aftkr Lllo-HSCT and rkckivkd donor CD19 CLA-T thkrape at Bkijing Childrkn＇s Hospital from Jule 2015 to Octobkr 2017. Diskask status bkfork infusion, conditioning rkgimkn,rkinfusion ckll dosk,and sidk-kffkct of CLA-T infusion,changks in thk rklatkd immunological indicators,and follow-up trkatmknt rksults wkrk invkstigatkd. ResuIts Onk patiknt had no kffkct,othkr patiknts got rk-mission or minimal rksidual diskask(MAD)nkgativk within 4 wkkcs aftkr CLA-T infusion,and thk middlk timk was 14 daes. Pkriphkral CLA-T pkac happknkd 2 wkkcs aftkr CLA-T infusion. Be thk last follow,2 patiknts dikd of lkuckmia, 3 patiknts wkrk alivk,and 1 cask of thkm livkd with tumor aftkr CD19 nkgativk rklapsk,othkrs livkd with diskask-frkk condition. Cetocink rklkask sendromk(CAS)was thk most common sidk kffkct,happkning in 1 to 2 wkkcs aftkr infusion, 1 patiknt had nkurologic toxicitiks,and 2 patiknts had suspicious graft -vkrsus -host diskask. ConcIusions Donor CD19 CLA-T thkrape has a good short-tkrm kffkct for rklapskd B -ckll lkuckmia patiknts aftkr Lllo -HSCT,but long-tkrm kffkct rkquirks furthkr obskrvation;CAS is thk most common sidk-kffkct. Off-targkt and ckll kxhaustion ark thk main rkasons for dkfkat.

Objective:To apply the best evidence for prevention of oral mucositis in children with hematopoietic stem cell transplantation (HSCT) to clinical practice and evaluate its efficacy.Methods:Evidence-based nursing method was applied to obtain the best evidence, the evidence quality review indexes were established and the obstacle factors were analyzed, and then the action strategy was constructed. Using the convenient sampling method, a total of 60 children who were admitted to HSCT ward of Beijing Children's Hospital of Capital Medical University and 14 nurses working in HSCT ward were selected as the research objects from August 2019 to October 2020. The children admitted from August 2019 to February 2020 were included in the control group, and children admitted from March to October 2020 were included in the experimental group, with 30 cases in each group. Children in the control group received routine preventive measures for oral mucositis, while children in the experimental group received preventive measures for oral mucositis constructed based on the best evidence. Before and after the application of the evidence, the nurses' knowledge of preventing oral mucositis in children with HSCT was investigated through self-developed test papers. The incidence of oral mucositis and parents' satisfaction with prevention measures for oral mucositis were compared between the two groups.Results:Evidence-based nursing practice used 10 pieces of evidence. The incidence of oral mucositis in the experimental group was 50% (15/30) , lower than 83% (25/30) in the control group, and the difference was statistically significant (χ 2=7.50, P < 0.05) . The score of satisfaction with for preventive measures of oral mucositis of parents in the experimental group was (4.96±0.05) , which was higher than (3.65±1.60) of the control group, and the difference was statistically significant ( t=-9.69, P<0.01) . After the application of the evidence, the score of nurses' knowledge of prevention of oral mucositis was (98.22±6.65) , which was higher than (78.72±12.03) before the application, and the difference was statistically significant ( t=5.30, P<0.01) . Conclusions:Management of oral mucositis in children with HSCT through evidence-based nursing practice can effectively reduce the incidence of oral mucositis in children, improve parents' satisfaction with prevention measures for oral mucositis and nurses' knowledge of prevention of oral mucositis.

AIM:To investigate the effects of curcumin on cardiac dysfunction induced by chronic restraint stress in a depression rat model.METHODS:Thirty-two Wistar rats weighing(200±20)g were randomly divided into control,model,low-dose curcumin,and high-dose curcumin groups(n=8 per group).The rats in model and curcumin groups were subjected to chronic restraint stress for 5 h daily at random time,while those in control group were maintained under normal conditions.Following daily stress exposure,the rats in low-and high-dose curcumin groups received 100 and 200 mg/kg curcumin daily,respectively,and those in control and model groups received the same volume of normal saline daily.The above treatments lasted for 28 d.Body weight of the rats was measured weekly.Sucrose preference test was performed on days 14 and 28 of the experiment.Serum corticosterone content was determined to evaluate depression.Histological changes of cardiac tissues were observed using HE and Masson staining.Echocardiography was conducted to examine heart function.The related mRNA and protein levels were detected using RT-qPCR and Western blot,respective-ly.RESULTS:Compared with control group,the rats in model group exhibited significantly slower weight gain(P<0.05),impaired sucrose preference(P<0.01),and increased corticosterone levels(P<0.01).HE staining revealed myo-cardial hypertrophy in model group but not in control group.Masson staining indicated significantly higher cardiac fibrosis in model group than control group(P<0.01).Immunohistochemical staining demonstrated a significant increase in posi-tive collagen type I expression(P<0.01).RT-qPCR results showed significantly elevated mRNA levels of inflammatory cytokines(tumor necrosis factor-α,interleukin-6,and interleukin-1β)and fibrosis factors(α-smooth muscle actin,colla-gen type I,and collagen type Ⅲ)in model group compared with control group(P<0.05 or P<0.01).Western blot re-vealed a significant increase in c-Jun N-terminal kinase(JNK)phosphorylation level in model group(P<0.01).Treat-ment with low-and high-dose curcumin reversed the above indicators.CONCLUSION:Curcumin treatment attenuated cardiac inflammation and fibrosis in rats subjected to chronic restraint stress,possibly by inhibiting JNK signaling pathway.

Hematopoietic stem cell transplantation (HSCT) in children may lead to endothelial cell activation, and the induction of procoagulant, proinflammatory, and proapoptotic mediators, resulting in endothelial cell dysfunction and injury, as well as life-threatening complications.An effective management of endothelial cell activation, injury, and dysfunction is important for improving outcomes after HSCT.This article reviews the recent advances in the epidemiological features, pathogenesis and treatment of veno-occlusive disease/sinusoidal obstruction syndrome and transplant-related thrombotic microangiopathy after HSCT.

Objective:To summarize the clinical characteristics of high-risk neuroblastoma (HR-NB) in a single center, analyze the prognostic factors of HR-NB.Methods:The clinical data of children with HR-NB who were treated and followed up at the hematology-oncology center of Beijing Children′s Hospital from February 1, 2007 to June 30, 2018 were analyzed retrospectively. The clinical features were summarized. Kaplan-Meier method was used for survival analysis and Cox regression was used to analyze the prognostic factors. The last follow-up time was June 30, 2019.Results:A total of 458 children with HR-NB were enrolled in this study, including 265 males (57.9%) and 193 females (42.1%), the age at diagnosis was 40.0 months (4.5-148.0 months), the follow-up time was 22.0 months (0.2-138.0 months) and the time of tumor progression or recurrence was 15 months (1-72 months). The 5-year event-free survival (EFS) rate was (31.2±2.6)% and the 5-year overall survival (OS) rate was (43.9±3.2)%. The 5-year EFS rate and 5-year OS rate in 142 hematopoietic stem cell transplantation (HSCT) patients with bone marrow metastases were better than that in 196 non-transplantation cases with bone marrow metastases ((26.5±4.5)% vs. (25.1±3.6)%, χ2=13.773, P=0.001; (38.1±5.5)% vs. (35.7±4.7)%, χ2=9.235, P=0.002); 128 transplantation patients with bone metastases had higher 5-year EFS rate and 5-year OS rate than 188 non-transplantation cases with bone metastases ((28.5±5.0)% vs. (26.7±3.8)%, χ2=10.222, P=0.001; (37.1±6.0)% vs. (36.2±4.8)%, χ2=7.843, P=0.005). The 5-year EFS rate was higher in 37 HSCT patients with MYCN amplification than in 49 non-transplantation cases with MYCN amplification ((26.8±8.0) % vs. (20.5±6.4) %, χ2=5.732, P=0.017). No significant difference was found in 5-years OS rate between transplantation group with MYCN amplification and non-transplantation group with MYCN amplification ((31.4±8.6) % vs. (26.2±7.4) %, χ2=3.230, P=0.072). Univariate survival analysis showed that lactate dehydrogenase (LDH)≥1 500 U/L was associated with poor prognosis of patients with MYCN amplification (χ2=6.960, P=0.008). Multivariate Cox analysis showed bone marrow metastasis and LDH≥1 500 U/L were independent risk factors for poor prognosis of patients with non-MYCN amplification ( HR=2.427, 1.618;95 %CI：1.427-4.126, 1.275-2.054, P<0.05) for both comparisons. Conclusions:LDH≥1 500 U/L was the poor prognostic factor for patients with MYCN amplification. The bone marrow metastasis and LDH≥1 500 U/L were the poor prognostic factors for HR-NB patients with non-MYCN amplification. HSCT can improve the prognosis of patients with bone or bone marrow metastasis. It can also retard the time of progression or recurrence for patients with MYCN amplification.

Objective To analyze the clinical characteristics,treatment and prognosis of allogeneic hematopoietic stem cell transplantation (allo-HSCT)-associated thrombotic microangiopathy (TA-TMA) in children.Methods The clinical information,treatment and prognosis of 9 cases with TA-TMA hospitalized following alloHSCT from January 2008 to November 2017 in Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University were retrospectively analyzed.Results Of all the 283 allo-HSCT recipients,9 patients (3.2％) were diagnosed as TA-TMA.Among them,there were 5 male and 4 female,with a median age of 94 months (39-129 months).The median time to of diagnosis was 63 days (6-342 days) after HSCT.Additionally,the median platelet counts,hemoglobin and lactate dehydrogenase(LDH) levels were 44 × 109/L [(7-75) × 109/L],76 g/L (40-105 g/L) and 594 U/L(445-1 386 U/L).Neurological symptoms were found in 5 of the patients,4 had kidney involvement,and 6 had gastrointestinal involvement.The major treatment of TA-TMA was plasma exchange,Rituximab and defibrotide instead of the use of calcineurin inhibitors.Finally,4 patients achieved response after treatment,5 children died of ineffective treatment.Conclusion TA-TMA is a fatal complication after allo-HSCT.It can lead to multiorgan and multi-systems dysfunction.If there are more than 2 systems involved in TA-TMA,it suggests poor prognosis.The combined therapy is better than monotherapy,and the selective individual treatment of TA-TMA is essential.

A 6-year-old girl presented with recurrent skin rash at the initial stage, recent joint pain, and neutrophilia was found during a routine blood test. After a multidisciplinary case discussion, she was diagnosed with chronic neutrophil leukemia, and the symptoms were relieved after hydroxyurea and luxolitinib treatment. She received the allogeneic hematopoietic stem cell transplantation subsequently. At present, she is in stable condition and under follow-up. Chronic neutrophil leukemia is a rare disease, which rarely occurs in children. It is more difficult to diagnose in patients with skin rash as the first manifestation. The diagnosis and treatment of this case reflects the important role of multidisciplinary cooperation in the diagnosis and treatment of difficult and rare diseases.