Reflections on the diagnosis and treatment of high myopia / 中华眼底病杂志

High myopia has become a global public health issue, posing a significant threat to visual health. There are still some problems in the process of diagnosis and treatment, including the definition of high myopia and pathological myopia, opportunities and challenges of artificial intelligence in the diagnosis and treatment system, domestic and international collaboration in the field of high myopia, the application of genetic screening in children with myopia and high myopia patients, and the exploration of new treatment methods for high myopia. Nowadays, myopia and high myopia show the characteristics of early onset age and sharp rise in prevalence, and gradually become the main cause of low vision and irreversible blindness in young and middle-aged people. Therefore, it is of great significance to accurately define high myopia and pathological myopia, combine artificial intelligence and other methods for screening and prevention, promote cooperation in different fields, strengthen gene screening for early-onset myopia and adopt new and effective ways to treat it.

Clinical analysis of Blinatumomab on the treatment of refractory or relapsed precursor B-cell acute lymphoblastic leukemia / 中华实用儿科临床杂志

Objective:To evaluate the clinical efficacy and safety of Blinatumomab on the treatment of refractory or relapsed precursor B-cell acute lymphoblastic leukemia (R/R BCP-ALL) in children.Methods:Clinical data of children with R/R BCP-ALL treated with Blinatumomab in the Department of Hematology, Children′s Hospital of Soochow University, from August 2021 to June 2022 were retrospectively analyzed.Children were divided into<45 kg group and ≥45 kg group according to their weight at admission.They were treated with different dosages of Blinatumomab, and bone marrow remission was assessed at about 15 days.Clinical indicators and adverse events during the treatment period were recorded.The rank sum test of two independent samples were used to compare the differences between groups.The Fisher′ s test was used for comparing categorical variables. Results:Among the 16 children with R/R BCP-ALL, 12 cases (75%) achieved complete response (CR) and minimal residual lesion (MRD) turned negative at about 14 days.Among them, 5 out of 9 children with bone marrow primitive naive cell ratio≥0.5 achieved CR, and 7/7 children with bone marrow primitive naive cell ratio<0.5 achieved CR.The peak value of interleukin-6 (IL-6) in children with CR was significantly higher than those without CR ( Z=2.50, P=0.012). Twelve cases achieved CR on bone marrow assessment around day 15, and 3 cases who did not achieve CR remained in remission on day 28, with an efficacy prediction accuracy of 93.8%(15/16). Adverse events included fever, neutropenia, hypokalemia, abnormal liver function, hypocalcemia, edema, rash, hypertension, myocardial damage, abdominal pain, hypotension, and cytokine release syndrome, which were all grade 1.Neurotoxicity and death were not reported. Conclusions:The remission rate of R/R BCP-ALL in children treated with Blinatumomab was high, especially in patients with a low tumor load.The toxicity and adverse events of Blinatumomab treatment are minor and controllable.Day 15 is the optimal time point to evaluate the efficacy of Blinatumomab on children with R/R BCP-ALL, and a higher IL-6 peak can be served as a predictor of its efficacy.

Clinical characteristics of 15 children with mixed phenotype acute leukemia / 中华实用儿科临床杂志

Objective:To analyze the clinical characteristics, treatment and prognosis of mixed phenotypic acute leukemia (MPAL) in children, and to provide reference for clinical optimization of diagnosis and treatment and improve the remission rate.Methods:Based on the diagnostic criteria of the World Health Organization (WHO) in 2016, the bone marrow cell morphology, immune typing, cytogenetics, molecular biological characteristics, treatment regimen, and prognosis of 15 children with MPAL who were admitted to Children′s Hospital Affiliated to Soochow University from January 2012 to December 2020 were retrospectively analyzed.The count data were compared between groups using the χ2 test, the measurement data that conformed to the normal distribution were compared using the t test, and the measurement data that were not normally distributed were compared using the rank sum test.Survival was estimated using the Kaplan- Meier ( K- M) method and the Log- rank method was used for comparison. Results:A total of 15 children with MPAL were admitted to Children′s Hospital Affiliated to Soochow University in the past 8 years, including 8 males and 7 females, with a median age of 6.8 years.Nine patients expressed B lymphocyte and myeloid phenotype, 5 patients expressed T lymphocyte and myeloid phenotype, and 1 patient expressed B and T lymphocyte phenotype.Karyotype of 11 children was examined, and the results showed that there were 2 cases of normal karyotype, 2 complex karyotype, 6 pseudodiploid and 1 subdiploid.Fusion genes were detected in 5 children, including 3 AML- ETO positive, 1 BCR- ABL positive, and 1 MLL gene positive.Thirteen patients were in complete remission (CR) after chemotherapy, with a total CR rate of 86.6% and a 2-year over survival (OS) rate of (68.2±13.4)%.Among the 15 children, 14 received induction chemotherapy and 1 gave up treatment for personal reasons.There were 10 patients with the first choice of acute lymphoblastic leukemia (ALL) chemotherapy regimen and 1 patient receiving CR, with a total CR rate of 10%.There were 4 cases of acute myeloid leukemia (AML) with the preferred chemotherapy regimen and 3 cases with CR in the first course of treatment, and the total CR rate was 75%.One case without remission was relieved after changing ALL chemotherapy regimen.The 2-year OS rates of 8 patients with hematopoietic stem cell transplantation (HSCT) and 6 patients without HSCT were (70.0±18.2)% and (66.7±19.2)%, respectively, with no significant difference ( χ2=0.318, P=0.573). Conclusions:Children with MPAL is a rare malignant tumor, mainly characterized by the co-expression of lymphoid and myeloid antigens.Chemotherapy alone or HSCT can achieve a good prognosis in the short term, but the long-term efficacy remains to be further observed.

The effect of vitrectomy combined total versus fovea-sparing peeling of internal limiting membrane for myopic foveoschisis / 中华眼底病杂志

Objective:To systematically evaluate the effect of pars plana vitrectomy (PPV) combined total peeling of internal limiting membrane (ILM) versus fovea-sparing peeling of ILM for myopic foveoschisis.Methods:A evidence-based medicine study. Chinese and English as search terms for myopic foveoschisis, vitrectomy, and peeling of internal limiting membrane were used to search literature in China National Knowledge Infrastructure, Wanfang database, VIP database, PubMed of National Library of Medicine, Medline, Embase, and Cochrane Library. The high myopic macular schisis was selected as the research object, the intervention method was PPV combined with complete ILM peeling and combined with foveal preservation ILM peeling surgery clinical control study between Jan 1, 2010, and Jun 31, 2021. Incomplete or irrelevant literature and review literature were excluded. The method of Newcastle-Ottawa Scale system was used to evaluate the included literature. The literature was meta-analyzed by RevMan5.3 software. The mean difference ( MD) and a confidence interval ( CI) of 95% were used to describe the effect sizes of continuous data, fixed effects model was performed. The data including the best corrected visual acuity (BCVA), central fovea thickness (CFT), and postoperative macular hole (MH) were analyzed. Results:In those databases, 232 articles based search stratery were totally retrieved, and 10 articles (417 eyes) were finally included for meta-analysis with 245 eyes for PPV combined total peeling of ILM and 172 eyes for PPV combined fovea-sparing peeling of ILM. Meta-analysis results showed there was no significant difference in BCVA and CFT between the two groups (BCVA: MD=0.05, 95% CI 0.00-0.11; P>0.05; CFT: MD=-4.79, 95% CI -18.69-9.11, P>0.05). It was compared with the incidence of MH, the difference was statistically significant (odds ratio=5.70, 95% CI 2.22-14.61, P<0.05). Conclusion:BCVA and CFT could be improved by PPV combined total and fovea-sparing peeling of ILM for myopic foveoschisis; compared with complete ILM peeling, the incidence of MH was lower after foveal-sparing ILM peeling.

Interobserver agreement of international classification of myopic maculopathy / 中华眼底病杂志

Objective:To observe the interobserver agreement of classification of macular degeneration in severe pathological myopia (PM) by ophthalmologists with different clinical experience.Methods:A retrospective study. From January 2019 to December 2021, 171 eyes of 102 patients with severe PM macular degeneration who were examined at Eye Center of Beijing Tongren Hospital of Capital Medical University were included in the study. The clinical data such as age, gender, axial length, spherical equivalent power, fundus color photography, and optical coherence tomography (OCT) were collected in detail. Six independent ophthalmologists (A, B, C, D, E, F) classified each fundus photography based on META-PM and ATN classification of atrophy (A) system and interobserver agreement was assessed by Kappa statistics. According to the classification standard of traction (T) in the ATN classification, the OCT images were interpreted and classified, in which T0 was subdivided into retinal pigment epithelium (RPE) and choroidal thinning, choroidal neovascularization (CNV) with partial RPE and choroidal atrophy, RPE, and choroidal atrophy. Lamellar macular hole can't be classified by ATN system, which was defined as TX. Kappa ( κ) test was used to analyze the consistency of classification results between physicians A, B, C, D, E and F. κ value ≤0.4 indicates low consistency, 0.4 < κ value ≤ 0.6 indicates moderate consistency, and κ value >0.6 indicates strong consistency. Results:Among the 171 eyes of 102 cases, there were 20 males with 37 eyes (19.6%, 20/102), and 82 females with 134 eyes (80.4%, 82/102); age was 61.97±8.78 years; axial length was (30.87±1.93) mm; equivalent spherical power was (-16.56±7.00) D. Atrophy (A) classification results in META-PM classification and ATN classification, the consistency of physician A, B, C, D, E and physician F were 73.01%, 77.19%, 81.28%, 81.28%, 88.89%; κ value were 0.472, 0.538, 0.608, 0.610, 0.753, respectively. In the ATN classification, the T0, T1, T2, T3, T4, and T5 were in 109, 18, 11, 12, 9, and 8 eyes, respectively; TX was in 4 eyes.Conclusions:There are differences in the consistency of classification of severe PM macular lesions among physicians with different clinical experience, and the consistency will gradually improve with the accumulation of clinical experience.

The efficacy and safety of eltrombopag in 13 children with chronic immune thrombocytopenia / 中国医师进修杂志

Objective:To explore the efficacy and safety of eltrombopag in children with chronic primary immune thrombocytopenia (cITP).Methods:The clinical data of 13 children with cITP in Children′s Hospital of Soochow University from July 2017 to December 2021 were retrospectively analyzed.Results:Among 13 children with cITP, 7 males and 6 females, the median age was 5 years old, the median duration of illness was 2.2 years. The median therapy duration of eltrombopag was 5 months, the median maintenance dose was 25.0 mg/d, the median time to response was 4 weeks; complete response was in 5 cases, partial response was in 2 cases; 2 cases had skin disease, 1 case had arthralgia, 1 case had liver function abnormal, all complications did not cause serious impact.Conclusions:Eltrombopag was an effective and safe therapeutic option for children with cITP.

The risk factors for hemophagocytic syndrome in childhood Epstein-Barr virus-associated infectious mononucleosis / 中国医师进修杂志

Objective:To explore the risk factors for hemophagocytic syndrome (HPS) in childhood Epstein-Barr virus (EBV)-associated infectious mononucleosis (IM).Methods:From January 2013 to December 2017, the medical charts of all children who were diagnosed with EBV-associated IM and HPS in Children′s Hospital of Soochow University were analyzed retrospectively. Statistical analyses were performed using SPSS version 22.0.Results:A total of 316 IM and 59 HPS were enrolled. The age was (4.26 ± 2.95) years old with a male-to-female ratio of 1.2∶1. In addition to the diagnostic criteria of HPS, there were significantly lower rates of fever >10 d, hepatomegaly, jaundice, alanine aminotransferase >500 U/L, aspartate aminotransferase >500 U/L, LDH >1 000 U/L, C-reactive protein >50 mg/L and hypoalbuminemia in children with EBV-associated IM compared to those with HPS, and the differences were statistically significant ( P<0.05). Multivariate Logistic regression analysis showed that fever >10 d, eyelid edema, lymphadenopathy and purulent tonsils were independent predictors of HPS in children with EBV-associated IM ( P<0.05). Hepatomegaly and fever >10 d were risk factors ( OR = 16.079 and 12.138, 95% CI 2.788 to 92.744 and 2.878 to 51.180). Eyelid edema, lymphadenopathy and purulent tonsils were protective factors ( OR = 0.087, 0.006 and 0.031; 95% CI 0.010 to 0.723, 0.001 to 0.058 and 0.007 to 0.146). Conclusions:Hepatomegaly and fever >10 d are the risk factors for hemophagocytic syndrome in childhood EBV-associated infectious mononucleosis.

Clinical observations of the therapeutic effect of CLAG or FLAG bridging BuCy conditioning regimen in the treatment of childhood acute leukemia / 中国输血杂志

【Objective】 To determine the therapeutic efficacy of cladribine combined with BuCy conditioning regimen for childhood acute leukemia, and compare it with fludarabine. 【Methods】 The clinical data of 70 children with acute leukemias who underwent all-HSCT from August 2018 to June 2020 were collected. The data of pretreatment-related toxicity, hematopoietic reconstitution, graft-versus-host disease, virus infection, relapsed and survival between CLAG and FLAG group were statistically analyzed. 【Results】 EBV infection in CLAG group was significantly more than that in FLAG group(P0.05). Multivariate analysis showed that there was no significant difference in the effect of conditioning regimen on relapsed and survival. Among other risk factors, types of diseases were significantly correlated with OS (P<0.05, HR: 0.088). Relapse was significantly correlated with bone marrow morphological remission before transplantation and the matching degree of donor and recipient HLA(P<0.05, HR: 34.678; P<0.05, HR: 0.024). 【Conclusion】 There was no significant difference in RRTs, hematopoietic reconstitution, GVHD occurrence, OS and relapsed between CLAG group and FLAG group. The overall efficacy of CLAG group was not inferior to FLAG group.

Validation of the Caprini risk model for predicting deep venous thrombosis in hospital-ized patients with malignant tumors / 中国肿瘤临床

Objective:To evaluate the effectiveness of the Caprini risk assessment model in predicting deep venous thrombosis in hos-pitalized patients with malignant tumors. Methods:Deep venous thrombosis screening was performed in 504 patients with malignant tumors who were hospitalized in Beijing Shijitan Hospital between January 2015 and January 2017. Their Caprini thrombosis risk mod-el scores and risk classifications were analyzed and compared with those of the Khorana risk model. Results:The median Caprini score of patients with deep venous thrombosis was 6 (range 4-8), which was higher than the score of 5 (range 4-7) in the group without deep venous thrombosis (Z=10.033, P=0.004). Statistically significant differences in the incidence of deep venous thrombosis were found among the low-medium, high-, and extremely high-risk groups (Z=-1.933, P=0.053). The area under the receiver-operating char-acteristic curve (AUC) of the Caprini scores was 0.611 [95% confidence interval (CI): 0.54-0.69, P=0.004], and the cutoff value was 6 points, with the largest Youden index. The AUC of the Khorana model was 0.65 (95% CI: 0.57-0.72, P<0.001), and the difference be-tween the Khorana and Caprini models was not statistically significant (Z=0.674, P=0.500). The AUC of the Caprini model was 0.85 (95% CI: 0.66-0.96, P<0.01) and that of the Khorana model was 0.68 (95% CI: 0.47-0.84, P=0.18) in the patients who underwent malig-nant tumor surgery. The AUC of the Khorana model was 0.72 (95% CI: 0.61-0.82, P=0.01) and that of the Caprini model was 0.55 (95% CI: 0.44-0.67, P=0.54) in the non-operative patients who received chemotherapy. Conclusions:The Caprini and Khorana risk assess-ment models have certain predictive values, but the discrimination is not good. The Caprini model is providing better predictability in patients with malignant tumors treated with surgery. The Khorana model is suitable for non-operative patients who received chemo-therapy. Further studies on the application of the Caprini risk assessment model in patients with malignant tumors are needed.

Comparison of two kinds of induction therapy in childhood acute myeloid leukemia / 临床儿科杂志

Objective To compare the efficacy and safety of induction therapy in 3+7 protocol and 3+10 protocol in children with acute myeloid leukemia (AML). Methods Two protocols were carried out in our hospital during January 2010 to January 2015, namely 3+7 protocol(AML-06,A group) and 3+10 protocol (modified AML protocol, B group). A total of 56 cases aged from 1 year-old to 13 year-old were enrolled in A group with male to female ratio at 31:25. Five of them were classified as FAB M1, 25 as M2, 11 as M4, 10 as M5, 2 as M6 and 3 as M7. Another 44 cases aged from 1 year to 12 years were enrolled in B group with a male to female ratio at 26:18, and 17 cases were classified as FAB M2, 14 as M4, 9 as M5, 2 as M6, and 2 as M7. Efficacy and adverse events were compared between the two groups. Results The complete remission rate (CR) of B group was 70.4%, while CR in A group was 48.2%. Considering the CR, 3+10 protocol showed higher efficacy than 3+7 protocol (P< 0.05). The major adverse event was bone marrow suppression. Treatment-related mortality (TRD) in A group was 1.8%, which was lower than that in B group (2.3%). The overall survival rate in A group was 75.0%, which was lower than that in B group (86.4%, P< 0.05). Conclusions The induction therapy of 3+10 protocol and 3+7 protocol showed effectiveness for AML treatment. The 3+10 protocol showed a higher CR than 3+7 protocol with no TRD increase, indicating that the 3+10 protocol should be recommended for AML treatment in children.

Effect of chemotherapy regimen CCLG-ALL-2008 on children with TEL/AML1 fusion gene positive of acute lymphoblastic leukemia / 临床儿科杂志

Objective To evaluate the predictive role of TEL/AML1 fusion gene in protocol CCLG-ALL-2008 and to identify relevant factors influencing the outcome of ALL with TEL/AML1 fusion gene. Methods Ninety-nine patients with ALL harboring TEL/AML1 fusion gene (positive) and 329 cases without any specific fusion genes (negative) at diagnosis of B-lineage ALL from June 2008 to December 2014 were enrolled and their clinical and biological features were analyzed. Following-up ended in October 2015, the survival status was calculated by K-M curve and prognostic factors were analyzed by COX model. Results There were no differences between the two groups in age, white blood cell at the diagnostic stage, and treatment responses at 4 time points, namely, prednisone good response on day 8, M3 status of BM on D15, and the minimal residual disease (MRD) more than 1.0×10-3 on day 33 and 12th week. During the follow-up period, the relapse rate was lower in the positive group than that in the negative group (14/99 vs 69/329), the mortality rate of the negative group was twice of that in the positive group (55/329 vs 8/99). The five-year overall survival (OS) rate, relapse-free survival (RFS) rate and event-free survival (EFS) rate of the positive group were (86.1 ± 4.9)%, (80.7 ± 5.1)% and (78.9 ± 5.1)%, respectively, and (79 ±2.8)%, (72± 3.1)%, and (69.6+ 3.1)% for the negative group as well. COX regression analysis indicated that relapse and MRD level at the 12th week were independent prognostic factors on OS, RFS, and EFS (P<0.05) for the two groups. Conclusions TEL/AML1 fusion gene could be regarded as a relatively good indicator of risks in ALL children treated by CCLG-ALL-2008 protocol. ALL patients with TEL/AML1 are recommended to receive more intensive therapy including hematopoietic stem cell transplantation when the patients were high level of MRD on the 12th week after treatment.

Characteristics and prognosis of parvovirus B19 infection in Pediatric leukemia patients in Suzhou / 临床儿科杂志

Objective To explore the effect of parvovirus B19 (VB19) infection on pediatric leukemia patients. Methods The pediatric leukemia patients were enrolled in the study in the Children's Hospital of Soochow University. Expression levels of VB19-DNA-PCR were detected using the polymerase chain reaction. Positive patients would be monitored and treated by conventional treatment as well until VB19 gene became negative. The data was compared according to the VB19 clearance time, clinical symptoms and blood counts to evaluate the effect. Results In the 3009 samples from 824 pediatric leukemia patients, there were 36 samples (1.2%) from 12 cases (1.5%) of pediatric leukemia paients with positive VB19 infection. Among the positive patients, 11 cases (1.9%) were from 582 with acute lymphoblastic leukemia (ALL) patients and 1 (0.45%) was from 212 with acute myeloid leukemia (AML). According to the treatment stage, 3 cases were in initially diagnosed period, 2 cases in early stage of consolidation chemotherapy, 4 cases in delayed enhanced chemotherapy period, and 3 cases in maintenance chemotherapy period. According to the treatment response, 4 cases were in continuous treatment, 2 cases were sensitive to treatment, and 3 cases were drug resistant. In the drug resistance group, 2 cases developed into the pure red cell aplastic anemia (PRCA). After treatment, one was recovered from PRCA with VB19 cleared, the other one remained PRCA with continuously positive VB19. Conclusions More VB19 virus infection in pediatric ALL happened in delayed enhanced chemotherapy period. The persistent presence of VB19 infection on pediatric leukemia patients is closely related with PRCA.

Evaluation of clinical features in predicting relapse of childhood acute lymphoblastic leukemia treated with CCLG-2008 protocol / 临床儿科杂志

Objective To evaluate the prognostic factors in predicting relapse of childhood acute lymphoblastic leukemia (ALL) treated with CCLG-2008 protocol. Methods From December 1st 2008 and December 31st 2012, 358 patients diagnosed with ALL and treated with the CCLG-ALL 2008 protocol were enrolled in this study. All patients were followed up until September 1st, 2015. Prognostic impact of clinical features, response to treatment, biological features were analyzed and multivariate analysis of predicted value was performed by Cox-regression analysis. Results After treatment of CCLG-ALL 2008 protocol, 79 patients suffered from relapse. The relapse rate in the standard-risk, intermediate-risk and the high-risk groups were 13.3%, 17.6%, and 41.3%, respectively (P?100×109/L, non-remission in 15th day of induction (M3), the level of minimal residual disease (MRD) on 12w (12w-MRD)?>10-4 were signiifcantly higher, their corresponding hazard ratio were 3.17 (1.58?~?6.36), 1.87 (1.07?~?3.30), and 1.90 (1.12?~?3.20), respectively (P?10-4 were the independent prognostic factors for childhood B-ALL.

The expressions and significances of miR-155 in 52 bone marrow samples of preliminary pediatric acute myeloid leukemia / 中华实用儿科临床杂志

Objective To investigate the differential expression of miR-155 in newly diagnosed pediatric acute myeloid leukemia(AML) and its clinical significances.Methods Fifty-two AML children and 30 non-malignant disease matched children were recruited as the controls.The preliminary AML children were divided into favorable group,moderate group and poor group according to the National Comprehensive Cancer Network(NCCN) 2013.Real-time quantitative polymerase chain reaction was applied to validate the expressions of miR-155 in bone marrow samples (the data presented by 2-△△Ct).Results By comparing expressions of miR-155 between AML patients and controls,the miR-155 expressions were significantly higher in the AML children than those in the controls (Z =-5.391,P ＜ 0.001).There were significant differences among different prognostic groups,with a significantly lower level in the favorable group compared with others (x2 =12.586,P =0.002).It was also found that differential expressions existed not only in kinds of mutation cohort,with the highest level in FLT3-ITD and the lowest one in FLT3-TKD mutation group (x2 =11.216,P =0.024),but also among fusion gene subgroups (x2 =12.254,P =0.016),with the highest level in AML-ETO group and the lowest level in PML-RARa group:meanwhile,the expressions of miR-155 were statistic different according to French-America-British (FAB) subtypes (x2 =17.814,P =0.013),which was lower in M3 patients than non-M3 patients (Z =-3.291,P =0.001).Conclusions It indicates that the expressions of miR-155 may increase sharply in preliminary AML children,and the lower expression of miR-155 is closely related to favorable prognosis.

Clinical characteristics and clinical observation of granulocyte sarcoma in children / 中华实用儿科临床杂志

Objective To discuss the mode of onset,clinical characteristics,treatment and prognosis of children with granulocyte sarcoma (GS),in order to provide guidance for early diagnosis and effective treatment of GS.Methods Six cases of children with GS diagnosed at the Department of Hematology,Children's Hospital Affiliated to Soochow University between June 2009 and June 2014 were analyzed,the data including the mode of onset,clinical manifestation,diagnosis,treatment and outcome.Results There were 2 cases with a painless mass onset (1 case was 2 years old,characterized by right waist mass,about 10 cm × 5 cm;the other case was 6 years old,characterized by axillary lump,about 2 cm × 3 cm),and both of them received surgical removal of the tumor,then the postoperative tumor was examined by pathologic and immunohistochemical method,and at last the primary granulocyte sarcoma was diagnosed.The third case was a 7 years old girl,she was onset characterized by scalp lump,about 2 cm × 3 cm,and was diagnosed by the pathologic and immunohistochemical method,and changes in hematological system appeared a month later and acute myeloid leukemia(AML) was confirmed by bone marrow examination.The onset ages of other 3 cases were in 10 months,1 year and 7 months,13 years and 3 months old respectively,characterized by scalp lump (about 2 cm × 3 cm),spinal canal tumor (about 1.0 cm × 1.5 cm),intracranial tumors (6.0 cm × 4.9 cm),with AML occurring at the same time,which was confirmed by surgical pathology,immunohistochemistry and bone marrow cell morphology,immune classification,chromosome,and fusion gene diagnosis.Four cases were hematopoietic malignancies by pathology,2 cases of then belonging to small round cell tumor.The immune pathology showed 5 cases of myeloperoxidase positive,CD68-positive,3 cases of CD43-positive,CD123-positive.All children CD3,CD20 levels in all children were negative.Four cases underwent surgery combined with chemotherapy,other 2 cases received surgery and then gave up treatment,1 case discontinued follow-up 3 months later,and the other case died of intracranial hemorrhage after 3 months,which induced by thrombocytopenia.The treated 4 cases were followed up 3 to 58 months,and all had disease-free survival.Conclusions Children with GS have low incidence and non-specific diagnostic criteria,its diagnosis depends on immune pathology,and the treatment is mainly in accordance with AML program for high-dose chemotherapy.The systematic chemotherapy helps to prolong overall survival;at the same time,the hematopoietic stem cell transplantation with bone marrow may help to improve the prognosis.

Expression and significance of mTOR signaling pathway in T cells in childhood aplastic anemia / 中华实用儿科临床杂志

Objective To sudy the changes in mTOR signaling pathway in childhood aplastic anemia(AA) by detecting the expression levels of the molecules of mTOR signaling pathway in T cells,and to explore immunologoical pathogenesis of AA in children from T cell intracellular signal transduction pathway.Methods Peripheral blood samples were collected from 16 newly diagnosed severe AA(SAA) patients and 8 patiens treated with effective immunosuppressive therapy,and the findings were compared with those of 17 healthy children (normal controls) and CEM cells (positive controls).The expressions of p-Akt,p-TSC2,p-mTORC1,p-4EBP1,p-p70S6K in CD3 + T cells in peripheral blood were detected by flow cytometry(FCM).Results 1.The expressions of p-Akt,p-TSC2,p-mTORC1,p-4EBP1,pp70S6K of the newly diagnosed SAA group were higher than those of the normal control group (P ＜ 0.05),but were lower than the postive control group (CEM group) (P ＜ 0.05).The mean fluorescence intensity (MFI) of p-Akt of three groups was 8.04 ± 3.78,2.59 ± 1.01 and 20.23 ± 8.98 respectively ;p-TSC2 was 49.73 ± 19.49,16.10 ± 8.04 and 101.05 ± 29.78 respectively ; p-mTOR was 13.90 ± 9.32,2.92 ± 1.09 and 34.3 ± 19.03 ;p-4EBP1 was 142.69 ± 53.36,26.91 ± 13.70,256.01 ± 53.79 ; p-p70S6 K were 17.67 ± 10.48,3.69 ± 2.22,31.73 ± 12.85 respectively.2.The expressions of p-Akt,p-TSC2,p-mTORC1,p-4EBP1,p-p70S6K of the effective treatment groups were lower than those of the newly diagnosed SAA group (P ＜ 0.05) ; the expressions of p-Akt,p-TSC2,p-mTORC1,p-p70S6K were similar to those of the normal control group(P ＞ 0.05),but the expressions of p-4EBP1 were higher(P ＜ 0.05).The MFI was followed by 3.28 ± 1.27,16.50 ± 10.91,3.54 ± 1.66,74.89 ± 49.69 and 4.21 ± 1.69.Conclusions 1.The expressions of p-Akt,p-TSC2,p-mTORC1,p-4EBP1,p-p70S6K were increased in the newly diagnosed SAA patients,the mTOR signaling pathway was activated in SAA patients.2.The expressions of p-Akt,p-TSC2,p-mTORC1,p4EBP1,p-p70S6K were lower than those of the newly diagnosed SAA patients.The degree of activation of mTOR signaling pathway was associated with disease status.The signaling pathways may be involved in the T cells of AA of the immune abnormalities.

Application of evidence-based medicine concept in orthopedic clinical teaching for eight-year program medical students / 中华医学教育探索杂志

Objective To discuss the effect of applying evidence-based medicine (EBM) in orthopedic clinical teaching for eight-year program medical students in military medical university. Methods Totally 50 eight-year medical students in Shanghai Changzheng Hospital were equally divided into two groups:experimental group(EBM teaching group) and control group. The teacher of experimental group selected appropriate cases and set up the questions according to the difficulties encountered in clinical situation. Students retrieved the relevant medical literature and found out the most useful information for EBM system analysis. The results combined with the current cases were discussed. The teachers summarized clinical experiences and data according to the relevant depart-ment. Traditional direct infusion teaching mode was used in control group. Clinical work experiences were used to guide the students . After-department examination was conducted for both group after finishing the internship and questionnaire investigation was made among students in experiment group. Statistical analysis was carried out on the examination results with SPSS 17.0 software. Mea-surement data were expressed as x±s and comparison on mean differences between two groups was analyzed by t test. Results Average scores of subjective topic and military related topic in the theory exam were higher in experimental group than in traditional teaching group, with statistically significant differences(P<0.001). Excellent rate was 80%(20/25) in case discussion in experimental group and 60%(15/25) in control group. Experimental investigation questionnaire showed that: students got enhanced in learning initiative and broadened knowledge. 96%(24/25) students strengthened the learn-ing efficiency of military related chapters. 88%(22/25) students accepted this method and expected to continue. Conclusions Using the method of evidence-based medicine in eight-year program medical students in military medical university can cultivate the spirit of the stu-dents' self-study actively and rigorous earnest attitude. Students can grasp learning methods and sci-entific thinking. It also can strengthen students' awareness of military medicine and research.

The expressions of miR-196b and its prognostic significances in pediatric acute myeloid leukemia / 临床儿科杂志

Objective To evaluate the expression of miR-196b in newly diagnosed pediatric acute myeloid leukemia (AML) and its clinical signiifcance. Methods Fifty-two AML children were enrolled in this study and 30 non-leukemia com-pared children were selected as controls. The expressions of miR-196b were detected in bone marrow samples by real-time quan-titative PCR (q-RT-PCR) and the results were expressed in 2-??Ct. Results miR-196b expressions were signiifcantly higher in M4-5 and lower in non-M4-5 of AML children than those in control (P<0.01), with a lowest level in t (15;17) and a highest level in MLL subtypes (P<0.01). The miR-196b expressions were signiifcantly different among different prognosis groups (P<0.01) and the level in the favorable prognostic group was lower than in poor prognosis group. It was also found that miR-196b expres-sion was lower in remission group than that in no-remission group after the ifrst induction remission therapy (P<0.05). Mean-while, the expression of miR-196b in the children with WBC≥100×109/L were statistically higher than that in the children with WBC<100×109/L (P<0.01), and miR-196b level was positively correlated with the platelet counts (r=0.302, P=0.030). Conclu-sions miR-196b expression is increased in poor prognosis group of AML children, and high expression of miR-196b is related with low response rate and poor prognosis. miR-1966 is expected to become a new target for the treatment of AML.