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Objective:To investigate the clinical manifestations, pathological features, treatment and prognosis of primary bone lymphoma in children.Methods:The clinical data of children who were initially diagnosed as primary bone lymphoma and treated in Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to January 2020 were retrospectively analyzed, including gender, onset age, primary involvement site, clinical stage, pathological type, fracture, and clinical outcome. The related literature was reviewed.Results:All 11 children were initially diagnosed as primary bone lymphoma, with a median age of onset of 8.6 years old (2.7-12.3 years old), including 7 males and 4 females. There were 7 cases of diffuse large B-cell lymphoma (DLBCL), 3 cases of B lymphoblastic lymphoma (BLL), and 1 case of anaplastic large cell lymphoma (ALCL). The initial symptoms were bone pain in 8 cases, local swelling in 1 case, limp in 1 case, and fever in 1 case. One case was in stage Ⅰ, 7 cases were in stageⅡ, and 3 cases were in stage Ⅳ, and the most common sites of involvement were femur and tibia. All 11 cases were treated with chemotherapy according to different pathological types, with a median follow-up time of 45 months (7-80 months). Ten cases got complete remission, 1 case of BLL died of bone marrow recurrence after chemotherapy remission.Conclusions:The clinical manifestations of primary bone lymphoma in children are insidious, DLBCL is the most common pathological type, and the prognosis is good after standardized treatment.
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Objective:To explore the clinical features of childhood lymphoma complicated with Pneumocystis jirovecii pneumonia (PJP).Methods:The clinical data, diagnosis and treatment of 5 children with lymphoma complicated with PJP admitted to Beijing Children's Hospital from January 2013 to April 2022 were retrospectively analyzed.Results:Among 5 patients, there were 3 males and 2 females, the median onset age was 7 years old; 4 cases were non-Hodgkin lymphoma and 1 case was Hodgkin lymphoma. Fever and cough occurred 5-18 months after chemotherapy; typical mosaic sign could be seen in 2 cases without pneumothorax and pleural effusion as well as other pathogenic infection; all 5 cases had hypoxemia; 4 cases were diagnosed by next-generation sequencing (NGS). The CD4/CD8 ratio decreased in all cases, and the median CD4 positive T-cell was 200/μl. Trimethoprim-sulfamethoxazole (TMP-SMZ) was irregularly used in 3 cases. During the treatment, all cases received mechanical ventilation, TMP-SMZ intravenously dripping combined with caspofungin, glucocorticoid and gamma globulin. All 5 cases of PJP were cured and there was no recurrent infection.Conclusions:Lymphoma children are susceptible to PJP due to immunocompromise caused by chemotherapy, and their condition progresses rapidly. When encountering fever, shortness of breath, severe lung symptoms and mild signs of children, it is necessary to improve the vigilance of PJP. NGS can help diagnosis, and TMP-SMZ should be actively treated and prevented. Early diagnosis and active treatment can achieve a good prognosis.
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Gaucher′s disease (GD) is a rare autosomal recessive metabolic disease caused by the functional deficiency of the lysosomal enzyme β-glucocerebrosidase (GBA). Variants in the GBA1 result in the deficiency or reduction of GBA activity, leading to the accumulation of its substrate glucocerebroside (Gb1; also known as glucosylceramide, GlcC) in mononuclear phagocytes of organs, including the liver, spleen, kidney, bone, lung, and even brain.Glucosylsphingosine (lyso-Gb1), a deacylated derivative of Gb1, is highly sensitive and specific for GD.This study reviews the role of lyso-Gb1 in the diagnosis, curative effect, prognosis evaluation and follow-up monitoring of GD, aiming to improve the understanding of the diagnosis and treatment progress of GD.
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A male child, aged 5 years and 3 months, was admitted to the Oncology Department with a history of pain in both hip joints, headache, and diplopia lasting for 40 days. Physical examination did not reveal definitive signs or obvious abnormalities in the nervous system. Imaging studies showed only abnormalities in the craniocerebrum and spinal cord. Routine cerebrospinal fluid (CSF) analysis revealed elevation in the total number of white blood cells, mainly mononuclear cells. Biochemical analysis of CSF showed normal glucose and chloride levels, and increased protein concentrations. The possibility of central nervous system (CNS) infection was initially considered. Subsequently, antibacterial and antiviral therapy was administered; however, this treatment was ineffective. Further examination of CSF through immunophenotyping revealed mature B-cell lymphoma with CNS involvement; there were no neoplastic lesions detected elsewhere in the body. Thus, the patient was diagnosed with primary central nervous system lymphoma (PCNSL). Complete remission was achieved after chemotherapy with the CNCL-2017-mature B-cell lymphoma regimen. Thus far, all chemotherapy cycles have been completed, the patient remains in complete remission, and the follow-up is ongoing. Clinicians should pay close attention to PCNSL in children.
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Objective:To investigate the efficacy of low-dose uric acid oxidase in treating children with aggressive mature B-cell non-Hodgkin lymphoma accompanied by hyperuricemia.Methods:Clinical data of children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia, who were treated in Beijing Children′s Hospital, Capital Medical University from January 2016 to June 2021 were retrospectively analyzed.The serum uric acid concentration was monitored in all pediatric patients from the day before chemotherapy to the seventh day of chemotherapy.Low-dose uric acid oxidase [0.05-0.10 mg/(kg·dose)] was intravenously injected into the patients when the serum uric acid level exceeded the upper limit of the normal range.The therapeutic effect and clinical medication experience of uric acid oxidase were summarized.The change of serum uric acid levels with time before and after the application of different doses of uric acid oxidase was analyzed by a repeated measures ANOVA. Results:A total of 106 children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia were enrolled in this study.There were 88 males and 18 females, with a median age of 6.5 (3.5, 10.0) years.The pathological subtypes comprised Burkitt′s lymphoma in 95 cases (89.6%), high-grade B-cell lymphoma in 7 cases (6.6%), and diffuse large B-cell lymphoma in 4 cases (3.8%). Additionally, 39 cases (36.8%) were in clinical stage Ⅲ and 67 cases (63.2%) were in stage Ⅳ.All cases had high tumor burden, including renal involvement in 52 cases (49.1%), tumor lysis syndrome in 42 cases (39.6%), and acute kidney injury in 27 cases (25.5%). Totally, one dose of uric acid oxidase was intravenously injected into 41 children (38.7%), 41 children (38.7%) were given 2 dosages, 20 children (18.9%) were given 3 dosages, and 4 children (3.8%) received 4 dosages.Moreover, 9 cases (8.5%) were supplemented with continuous renal replacement therapy.Serum uric acid concentrations before chemotherapy and 12 hours after injecting the first dose of uric acid oxidase were (741.4±312.9) μmol/L and (210.8±148.6) μmol/L, respectively.The difference was statistically significant ( t=5.288, P<0.001). The change of serum uric acid levels over time before and after the application of different doses of uric acid oxidase in children was compared, and no significant difference was found ( F=0.225, P=0.879). No delay in chemotherapy or death arising from tumor lysis syndrome and acute kidney injury occurred within 28 days after chemotherapy. Conclusions:Low-dose and on-demand application of uric acid oxidase can rapidly and effectively reduce serum uric acid levels in children with aggressive mature B-cell non-Hodgkin lymphoma in the early stage of chemotherapy.
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Objective:To investigate the efficacy of low-dose uric acid oxidase in treating children with aggressive mature B-cell non-Hodgkin lymphoma accompanied by hyperuricemia.Methods:Clinical data of children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia, who were treated in Beijing Children′s Hospital, Capital Medical University from January 2016 to June 2021 were retrospectively analyzed.The serum uric acid concentration was monitored in all pediatric patients from the day before chemotherapy to the seventh day of chemotherapy.Low-dose uric acid oxidase [0.05-0.10 mg/(kg·dose)] was intravenously injected into the patients when the serum uric acid level exceeded the upper limit of the normal range.The therapeutic effect and clinical medication experience of uric acid oxidase were summarized.The change of serum uric acid levels with time before and after the application of different doses of uric acid oxidase was analyzed by a repeated measures ANOVA. Results:A total of 106 children with primary aggressive mature B-cell non-Hodgkin lymphoma and hyperuricemia were enrolled in this study.There were 88 males and 18 females, with a median age of 6.5 (3.5, 10.0) years.The pathological subtypes comprised Burkitt′s lymphoma in 95 cases (89.6%), high-grade B-cell lymphoma in 7 cases (6.6%), and diffuse large B-cell lymphoma in 4 cases (3.8%). Additionally, 39 cases (36.8%) were in clinical stage Ⅲ and 67 cases (63.2%) were in stage Ⅳ.All cases had high tumor burden, including renal involvement in 52 cases (49.1%), tumor lysis syndrome in 42 cases (39.6%), and acute kidney injury in 27 cases (25.5%). Totally, one dose of uric acid oxidase was intravenously injected into 41 children (38.7%), 41 children (38.7%) were given 2 dosages, 20 children (18.9%) were given 3 dosages, and 4 children (3.8%) received 4 dosages.Moreover, 9 cases (8.5%) were supplemented with continuous renal replacement therapy.Serum uric acid concentrations before chemotherapy and 12 hours after injecting the first dose of uric acid oxidase were (741.4±312.9) μmol/L and (210.8±148.6) μmol/L, respectively.The difference was statistically significant ( t=5.288, P<0.001). The change of serum uric acid levels over time before and after the application of different doses of uric acid oxidase in children was compared, and no significant difference was found ( F=0.225, P=0.879). No delay in chemotherapy or death arising from tumor lysis syndrome and acute kidney injury occurred within 28 days after chemotherapy. Conclusions:Low-dose and on-demand application of uric acid oxidase can rapidly and effectively reduce serum uric acid levels in children with aggressive mature B-cell non-Hodgkin lymphoma in the early stage of chemotherapy.
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Objective:To analyze the clinicopathological features, treatment and prognosis of Epstein -Barr virus(EBV) positive diffuse large B cell lymphoma(DLBCL) in children, so as to improve the knowledge of pediatricians on this disease.Methods:The data of 6 cases of EBV positive DLBCL who were initially diagnosed and regularly treated in Beijing Children′s Hospital from January 2016 to December 2019 were collected, including basic information (gender, age, first symptom, and course of disease), pathological results [immunohistochemistry, EBV encoded RNA(EBER), latent membrane protein(LMP), and C- MYC gene], immune function, EBV index, treatment group, treatment plan and prognosis. Results:There were 4 males and 2 females, with the average age of 6.67 years.The uric acid was 266.2 μmol/L, lactic dehydrogenase(LDH) was 346.5 U/L at early stage, and 1 patient had immunodeficiency.The immune function test before chemotherapy indicated that the proportion of auxiliary T cells decreased in 4 cases, and the humoral immune function was normal in all patients.There was no evidence of recent infection in 6 patients, and EBV-DNA increased in 3 patients.There were 2 cases of stage Ⅲ, 4 cases of stage Ⅳ, 1 case of giant tumor, 2 cases of symptom B, 6 cases of extranodal invasion, 4 cases of central invasion and 1 case of bone marrow invasion.Three patients died and three survived.Immunohistochemistry showed that: (1) CD 19, CD 20, and CD 79a were expressed in all patients, and CD 30 was expressed in 5 patients.(2) C- MYC gene was detected by immunofluorescence in situ hybridization method in all patients, and no MYC break, Bcl-2 and Bcl-6 break and amplification were found.(3) EBV: EBER and LMP-1 were expressed in all patients. Conclusions:The pathological changes of EBV positive DLBCL are similar to those adults.The origin of non-germinal center and extranodal and central invasion are more common.The prognosis of the patients with central nervous system invasion is very poor, and the recurrence and progress of the disease often occur in the treatment or in the early stage of drug withdrawal.At present, there is no effective and feasible treatment plan.It is suggested that the patients in the late stage should receive allogeneic hematopoietic stem cell transplantation as soon as possible after intensive treatment, so as to improve the survival rate.
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@#Objective To explore the risk factors of hemorrhagic transformation and prognosis of acute ischemic stroke (AIS) after intravenous thrombolysis. Methods From June 2017 to June 2019,211 patients with AIS undergoing intravenous thrombolysis were selected as the research object. Among them,34 patients with hemorrhagic tromsformation (HT) were recorded as HT group,and 177 patients without hemorrhagic transformation The patient was recorded as a non-HT group,with a bleeding conversion rate of 16.11%. The relationship between intensive blood pressure reduction and bleeding conversion and prognosis in patients with AIS intravenous thrombolysis was analyzed during intravenous thrombolysis. Results Univariate analysis revealed gender,age,time interval between onset to start of treatment (OTT),blood glucose at admission,baseline blood pressure,and baseline from the onset to start of treatment (reombinant tissue plasminogen activator,rt-PA) The National Institute of Health stroke scale (NIHSS) score,type 2 diabetes,atrial fibrillation,and venous thrombolysis have statistically significant differences (P<0.05 or P<0.01),which is a vein Risk factors related to HT after thrombolysis;binary logistic analysis showed that the baseline NIHSS score of 17.5 was divided into independent risk factors for the transformation of venous thrombolysis hemorrhage in AIS patients (OR=1.639,P=0.013). Atrial fibrillation (OR=9.129,P<0.01) is an independent risk factor for the prognosis of patients with intravenous thrombolysis. Conclusion The baseline NIHSS score of 17.5 is divided into independent risk factors for HT after intravenous thrombolysis in AIS patients,and intensive blood pressure reduction during intravenous thrombolysis is a related risk factor for HT;atrial fibrillation is an independent risk factor for the prognosis of patients with intravenous thrombolysis.
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Objective@#To analyze the therapeutic effect of a modified LMB89 Group C regimen in the treatment of pediatric high-risk Burkitt lymphoma.@*Methods@#The clinical data of 172 children with newly diagnosed high-risk Burkitt lymphoma from January 2007 to April 2017 were retrospectively analyzed. All the cases were treated with the modified LMB89 Group C regimen.@*Results@#The median age of the patients was 6 (1-14) years. The sex ratio was 5.1∶1, 144 boys (83.7%) and 28 girls (16.3%) . According to St. Jude staging classification, 2 patients (1.2%) were in stage Ⅱ, 54 (31.4%) in stage Ⅲ and 116 (67.4%) in stage Ⅳ. Of them, 46 patients (26.7%) had mature B cell acute lymphoblastic leukemia (B-ALL) , and 52 patients had central nervous system (CNS) involvement. According to risk group, the patients can be divided into group C1 (CNS1, without testicles/ovaries involvement, n=65) , group C2 (CNS2, testicles/ovaries involvement, n=55) and group C3 (CNS3, n=52) . A total of 145 patients received rituximab combined with chemotherapy during the treatment, 10 patients suffered from progressive disease and died, and 5 patients relapsed. Treatment-related mortality was 2.9%. With a median follow-up of 36.0 (0.5-119.0) months, 3-year overall survival (OS) rate was (88.9±2.4) % and event free survival (EFS) rate was (87.9±2.6) % for all patients. 3-year EFS rates were (96.9±2.1) %, (90.9±3.9) % and (73.4±6.5) % for Group C1, C2 and C3 respectively, and that of Group C3 was significantly lower than that of Group C1 (χ2=12.939, P=0.001) and Group C2 (χ2=6.302, P=0.036) . The 3-year EFS rates were (79.3±6.8) % and (44.4±16.6) % for patients in group C3 treated with chemotherapy combined with rituximab and chemotherapy alone (χ2=5.972, P=0.015) . Multivariable Cox regression analysis showed that Stage Ⅳ (including B-ALL) , residual diseases in mid-term evaluation were independent unfavorable prognostic factors[HR=4.241 (95%CI 1.163-27.332) , P=0.026; HR=32.184 (95%CI 11.441-99.996) , P<0.001].@*Conclusions@#The modified LMB89 Group C regimen has ideal effect for the children with high-risk Burkitt lymphoma.
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Objective@#To summarize the clinical data of diffuse large B-cell lymphoma (DLBCL) in children and to evaluate the efficacy of Beijing Children′s Hospital B cell lymphoma protocol in the treatment of pediatric DLBCL.@*Methods@#The data (clinical, pathology, lab and image data) of 46 pediatric DLBCL admitted to the treatment group of Beijing Children′s Hospital from January 2005 to June 2017 were collected and analyzed retrospectively. According to the risk factors of staging, existence of poor prognosis genes and giant tumors, stratified treatment was carried out according to the international standard modified LMB89 regimen with high dose and short course. The Kaplan-Meier method was used to calculate the event free survival (EFS) and the overall survival (OS).@*Results@#(1) Among the 46 cases, there were 33 males and 13 females. The median age was 8.0 years. The time from the initial symptom onset to the diagnosis was more than 15 days in 45 children. Fourteen cases had B group symptoms (fever, night sweat, and weight lost), 25 cases had extranodal disease, 39 cases were stage Ⅲ and Ⅳ, 12 cases had bone marrow involvement, 3 cases had jawbone involvement. Thirty cases were group B and 16 cases were group C in the treatment group. (2) Initial symptoms: 6 cases had cervical mass, 20 cases had abdominal mass, 10 had abdominal pain with acute abdomen, 8 cases had fever, 2 cases had snore or upper respiratory tract obstruction. (3) Pathology result: 40 cases were germinal center B cell DLBCL, 6 cases were non germinal center B cell DLBCL, no case had the MYC gene rupture, double hit lymphoma and triple hit lymphoma. (4) Complication and evaluation: the tumor lysis syndrome was seen in 3 cases initially, severe infection and delayed treatment was seen in 1 case, no treatment related death. The first evaluation showed all cases were sensitive to chemotherapy (shrink>25%), the second evaluation showed 1 case had residual disease, the others were complete remission. (5) Treatment and outcome: the 5 year-EFS was the same with 5 year-OS, both were (97.8±2.2) %. Two cases relapsed after treatment off, early relapse was seen in 1 case, and died because of abandoning treatment. Late relapse was seen in 1 case and got a complete remission after Rituximab+group C protocol treatment.@*Conclusions@#Pediatric DLBCL was common in school aged boys, most cases were at middle and late stage at the time of diagnosis. DLBCL had a good prognosis after the treatment with Beijing Children′s Hospital′s B cell lymphoma protocol, but late relapse could be seen.
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Objective@#To analyze the clinical features and prognostic factors of childhood Burkitt's lymphoma and to summarize the therapeutic effect of the mature B-cell lymphoma regimen of Beijing Children's Hospital.@*Methods@#It was a retrospective study. From January 2007 to December 2015, 186 patients below 18 years of age with newly diagnosed, untreated Burkitt's lymphoma were enrolled. Three cases were eliminated because of the abandonment of the treatment and 183 cases were stratified and treated according to the mature B-cell lymphoma regimen of Beijing Children's Hospital, groups were as follows: A, n=1; B, n=59; C, n=123 and 97 patients in group C received combined rituximab therapy during the treatment. The clinical features and therapeutic effects of patients were analyzed, overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method. COX regression was used to identify the prognostic factors.@*Results@#The median age at diagnosis was 5 (1-14) years. There were 159 males (85.5%) and 27 females (14.5%) , the male-to-female ratio was 5.9∶1. A total of 174 cases (93.5%) evolved to stage Ⅲ and Ⅳ. Eight cases did not achieve remission and progressed to death, 9 cases relapsed. Only 5 patients (2.7%) died of treatment-related complications. With a median follow-up time of 48.0 (0.5-128.0) months, the 5-year OS rate and EFS rate were (89.1±2.3) % and (87.8±2.5) %. There was significant difference in the 5-year EFS rate between group B and C ( (94.9±2.9) % vs. (84.0±3.4) %, χ2=4.258, P=0.039). The 5-year EFS rate was (73.1±8.7) % and (86.7±3.7) % for patients in the group C treated with chemotherapy only and those treated with chemotherapy combined rituximab, but no statistical difference was found between them (χ2=3.360, P=0.067) . Central nervous system (CNS) involvement, insensitivity to early phase chemotherapy, residual diseases in mid-term evaluation were independent unfavorable prognostic factors (HR=6.167, 9.102, 3.104, 95%CI: 2.293-16.592, 1.837-45.107, 1.182-8.153) .@*Conclusions@#The large dose, short course treatment of mature B-cell lymphoma regimen of Beijing Children's Hospital is effective for pediatric Burkitt's Lymphoma. Combined treatment with rituximab can improve the efficacy. CNS involvement, insensitivity to early phase chemotherapy, residual diseases in mid-term evaluation are associated with increased risk of poor prognosis.
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Objective@#To review the clinical-pathology characteristics of 19 relapsed pediatric mature B cell lymphoma and to find the risk factors for recurrence and the feasible treatment after relapse.@*Method@#Data of 212 pediatric B cell lymphomas cases in Beijing Children′s Hospital from January 2006 to June 2015 were collected retrospectively. All the patients were treated according to the B cell lymphoma regimen of Beijing Children′s Hospital. During the study period, 19 of 212 cases were relapsed; the clinio-pathological characteristics of relapsed patients before treatment and after relapse were analyzed retrospectively, the treatment outcomes after relapse were summarized and the patients were followed-up.@*Result@#Nineteen of 212 cases had relapsed disease, for these relapsed patients: the median age at initial diagnosis was 5.5 years old, the median level of uric acid was 384(range, 121-713)μmol/L, the median level of lactate dehydrogenase(LDH) was 1 323(range, 146-6 370)U/L. Among 19 relapsed patients, 10 had local relapse and 9 had multiple relapses; 17 were Burkitt′s lymphoma and 2 were diffuse large B cell lymphoma. Staging: 2 cases were stageⅡ, 3 cases were stage Ⅲ and 14 cases were stage Ⅳ. Risk group: 6 cases were group B and 13 cases were group C. Nine cases had bone marrow involvement and 10 cases had central nervous system(CNS) involvement. Acute tumor lysis syndrome was seen in 6 cases during the early treatment and 13 cases had delayed treatment. Treatment after relapse: 10 cases received further treatment after relapse (rituximab + 1-4 courses high intensity second-line chemotherapy), 3 cases received autologous stem cell transplantation. There was no chemotherapy or infection related death, 3 cases achieved complete remission (CR). For all the 212 patients, the median follow-up time was 47 (range, 1-131)months and the 5-year event free survival(EFS)rate was (89.4±0.2)%. For the 19 relapse cases, the 5-year overall survival (OS) rate was (21.1±0.1)%, CR rate after relapse was 30%, patients died of the progression of the primary disease, no treatment related death occurred. Univariate analyses showed that bulky disease, stage Ⅳ, maxillofacial and CNS involvement, LDH>1 000 U/L, delay treatment, day 7 evaluation shrink <25%, residual diseases after 3 months treatment are relapse risk factors (all P<0.01).@*Conclusion@#Patients relapse during the treatment or at the early stage after the end of all chemotherapy have poor prognosis. So far there is no effective method for early relapse patients; the late relapse patients had the possibility of CR if they are sensitive to salvage treatment. In conclusion, to improve the outcome, the key point is to reduce the relapse.
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Objective To evaluate the MRI findings and clinical features of methotrexate-induced acute encephalopathy in children.Methods The clinical data and brain MRI obtained in 13 children with methotrexate-induced acute encephalopathy were retrospectively reviewed.The MRI features were analyzed , including information on the location , the signal intensity and follow-up MRI study was performed.Results Of the 13 patients , 2 patients suffered from seizure.Five patients had dysphasia , of which 4 patients had evidence of hemiparesis , 1 patient had right facial palsy.Five patients had unilateral weakness.And left hemiparesis was observed in 1 patient.DWI revealed well demarcated asymmetrical hyperintensity lesions within the centrum semiovale and/or periventricular white matter in 10 patients, corresponding to areas of hypointensity on ADC maps.One case showed hyperintensity areas in the bilateral supratentorial cortex and subcortical white matter on T 2-weighted images with subtle high-intensity on DWI.In all 10 cases there were resolution of the diffusion abnormality , 8 cases displayed residual FLAIR signal abnormalities involving areas of previously seen diffusion restriction , 5 cases showed decreased range of the lesion , 1 case was progressive, and 2 cases were stable.One case with hyperintensity areas in the supratentorial cortex and subcortical white matter showed small residual hyperintensity on T 2-weighted images and resolution of the diffusion abnormality.Conclusions MTX-induced acute encephalopathy often manifests as stoke-like symptoms.DWI is the imaging modality of choice for the detection of acute MTX neurotoxicity , and asymmetrical restricted diffusion in the deep white matter is the characteristic sign.Cytotoxic edema induced by MTX is transient and reversible .
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Objective To discuss the imaging features of skeletal changes in children with Gaucher disease on X-ray and MRI images.Methods One hundred and nine children with Gaucher disease were enrolled in this study.They all received routine X-ray for spine with anterior-posterior(A-P)and lateral view and bilateral femurs with A-P view.Among them.18 patients received X-ray for pelvic with A-P view.14 patients received X-ray for left wrist with A-P view.and 14 patients received MRI scan for femur.The MRI scan included T1-weighted imaging,T2-weighted imaging and fat-suppressed T2-weighted imaging with short tau inversion recovery(STIR)sequence.The imaging features of the X-ray and MRI images were analyzed retrospectively.Results The most common feature is osteoporosis,which presented in 91 cases (83.5%).Besides this,decreased density of metaphysis occurred in 86 cases(78.9%).erlenmeyer flask deformity of metaphysis occurred in 89 patients(81.7%),thinner cortex occurred in 69 cases(63.3%),osteolytic destruction occurred in 31 cases(28.4%).pathological fractures occurred in 26 cases (23.9%),osteosclerosis occurred in 12 cases(11.0%).cystic degeneration of bone occurred in 16 cases (14.7%),and dislocation of the hip occurred in 4 cases.All 14 patients received MRI presented abnormal signals.Among them,4 patients presented low signal intensity both on T1-weighted and T2-weighted images in bone marrow;the other ten presented high signal intensity mixed in low signal intensity areas on T2-weighted and fat-suppressed T2-weighted images.Conclusions The imaging features of skeletal changes in children with Gaucher disease are of some characteristics,which could provide useful information for the clinical treatment.
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Objective To investigate the value of Fluorine-18 fluorodeoxyglucose positron emission tomography (~(18)F-FDG-PET)in staging and detecting residual disease during follow-up of non-Hodgkin's lymphoma in children. Methods The results of~(18)F-FDG-PET of 88 podiatric NHL before and after chemotherapy was analyzed respectively.The results were compared with CT scans simultaneously.Results 67 cases were performed for initial staging.Of the 1072 anatomy regions were analyzed by PET and CT with concordalice of 88.62%regions(positive in 11.10%and negative in 77.52%1.Disconcordance were found in 11.38%regions(PET positive but CT negative in 6.8 1%,PET negative but CT positive in 4.57%).PET was significantly better than CT scan in the staging of podiatric NHL.35 scans were performed in the follow-up.The true residual disease positive rate of PET and CT were 87.50%and 57.14% respectively.The false positive rate of PET and CT were 12.50%and 50.00%respectively.Conclusion ~ (18)SF-FDG-PET imaging is of great value for the staging of the pediatric patients with NHL It's a useful technique to identify the residual disease,avoid unnecessary treatment and biopsy and Can predict eady relapse.
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Objective: To study the effect of Juglans Mandshuria on tumor cells. Methods : The apoptotic effect on the Hela、PC-3 of tumor cells was observed by agarose gel electrophoresis of DNA fragments. Results : It has directly cytotoxicity on tumor cells, LC 50 is about 9~15?g?mL -1 . Based on this, the inoculating trial on entity tumor in vivo administrated by hypodermic injection on mouse had the same effect as in vitro, the high-dose group (12mg?kg -1 ) can decrease the weight of tumor to 42.21%. Conclusion : Juglans Mandsshurica has the inhibition of tumor.