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1.
Article in Chinese | WPRIM | ID: wpr-880138

ABSTRACT

OBJECTIVE@#To retrospective analyze the reason of death in children with acute lymphoblastic leukemia (ALL) treated with CCLG-ALL 2008 protocol, and the experience was summarized in order to reduce the mortality.@*METHODS@#916 children diagnosed as ALL and accepted CCLG-ALL 2008 protocol from April 2008 to April 2015 in our hospital were enrolled, the dead cases in them were analyzed retrospectively.@*RESULTS@#169 children died, including 111 (65.7%) males and 58 (34.3%) females. Recurrence was the main reason of death. 150 (88.7%) children died due to recurrence, among them, 86 (57.3%) cases gave up directly. The second reason of death was infection. The main clinical sites of infection were concentrated in respiratory system and digestive system. Bacterial infection was most common (Gram-negative was common).@*CONCLUSION@#Enough finance and improving family compliance can decrease the mortality in children with ALL. Early rational use of antibiotics can reduce infection-related mortality in children with ALL.


Subject(s)
Antineoplastic Combined Chemotherapy Protocols , Child , Disease-Free Survival , Female , Humans , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prognosis , Retrospective Studies
2.
Article in Chinese | WPRIM | ID: wpr-880028

ABSTRACT

OBJECTIVE@#To analyze the outcomes of the children suffered from philadelphia chromosome positive acute lymphoblastic leukemia (Ph@*METHODS@#21 cases of firstly diagnosed Ph@*RESULTS@#Among 21 patients, 17 were male and 4 were female with a median age of 8 years old (range, 4-12 years), the median follow-up time was 30 moths (range, 10-133 months). All the patients were treated with chemotherapy induced by the high-risk project of CCLG-ALL 2008. Among 14 patients treated with TKI plus chemotherapy, nine patients achieved complete remission. During 3 months after treatment, patients without complete molecular response or with the second complete remission and intensity desire of transplantation were treated with allo-HSCT, among 9 patients with allo-HSCT, six patients achieved long term survival.@*CONCLUSION@#At TKI era, TKI combined with strong chemotherapy can make Ph


Subject(s)
Aged , Child , Female , Hematopoietic Stem Cell Transplantation , Humans , Infant , Male , Philadelphia Chromosome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/therapy , Protein Kinase Inhibitors , Retrospective Studies
3.
Article in Chinese | WPRIM | ID: wpr-781460

ABSTRACT

OBJECTIVE@#To analyze the clinical efficacy and side effects of reduced-dose of cyclophosphamide combined cyclosporine A for severe aplastic anemia(SAA) children.@*METHODS@#Ten pediatric patients with SAA from January 2008 to May 2012 were enrolled. All the patients were treated with reduced dose of cyclophosphamide combined cyclosporine A. The dose of cyclophosphamide was 30 mg/(kg·d)×4 d, the dose of cyclosporine A gradually increased >15 mg/L accroding to the blood concentration.@*RESULTS@#The median follow-up time of the 10 pediatric patients was 100 months (6-126 months). Among 10 children with SAA, 4 cases achieved complete response(CR), 3 cases obtained partial response (PR) and the overall response rate was 70%, the remaining 3 cases showed no response (NR). One refractory patient treated by cyclophosphamide was progressed to paroxysmal nocturnal hemoglobinuria(PNH) at 25 months and was dead at 42 months after therapy.@*CONCLUSION@#The results show that reduced-dose cyclophosphamide (30 mg/kg·d for 4 consecutive days) combinated with CsA (initial dose 4 mg/kg·d, and drugvallery concentration >150 ng/ml) can make 7 of 10 children with severe aplastic anemia achieve complete response or partial response, and this regimen may be the second line regimen selected for some SAA children.

4.
Article in Chinese | WPRIM | ID: wpr-828676

ABSTRACT

OBJECTIVE@#To study the significance of CD20 combined with white blood cell (WBC) count at diagnosis in the prognosis assessment in children with B-lineage acute lymphoblastic leukemia (ALL).@*METHODS@#A retrospective analysis was performed on the medical data of 821 B-ALL children who were treated with CCLG-ALL2008 regimen from April 2008 to April 2015. Their survival status was followed up.@*RESULTS@#Among the 821 children, 547 (66.6%) were negative, while 274 (33.4%) were positive for CD20 expression. Among 694 children with WBC50×10/L (higher WBC count), the 5-year EFS rates was 64.3%±7.7% and 53.7%±5.5% for CD20 positive and negative patients respectively (P=0.135); the 5-year OS rate was 81.4%±6.4% and 58.6%±5.6% for CD20 positive and negative patients respectively (P=0.022); CD20 positive expression was an independent protective factor for OS (HR=0.367, P=0.016).@*CONCLUSIONS@#In children with B-ALL who are treated with CCLG-ALL2008 regimen, those with CD20 positive expression in lower WBC count at diagnosis have a poor prognosis; however, those with CD20 positive expression in higher WBC count at diagnosis have a better long-time survival.


Subject(s)
Antigens, CD20 , Antineoplastic Combined Chemotherapy Protocols , Child , Disease-Free Survival , Humans , Leukocyte Count , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma , Diagnosis , Prognosis , Retrospective Studies
5.
Article in English | WPRIM | ID: wpr-828570

ABSTRACT

Acute lymphoblastic leukemia (ALL) is a common pediatric cancer. The second malignant neoplasms (SMNs) in long-term survivors of pediatric ALL are relatively rare. Herein we report a 10-year-old girl who was diagnosed as primitive neuroectodermal tumor (PNET) 5 years after the initial diagnosis of ALL with radiotherapy-free treatment. PNET is an exceedingly rare neoplasm in SMNs of survivors of childhood ALL. It is predisposed to be misdiagnosed and the pathogenesis is unclear. The outcome is poor. Long-term follow-up is necessary for the survival children of ALL.

6.
Journal of Experimental Hematology ; (6): 1075-1080, 2020.
Article in Chinese | WPRIM | ID: wpr-827158

ABSTRACT

OBJECTIVE@#To study the long-term efficacy of CCLG-ALL2008 protocol used for treatment of childhood acute lymphoblastic leukemia (ALL).@*METHODS@#Nine hundred and forty children with newly diagnosed ALL from January 2008 to April 2015 were treated with CCLG-ALL2008 protocol. Overall survival (OS) and event-free survival (EFS) rates were estimated by the Kaplan-Meier method. Cox proportional hazards model was used for analyses of prognostic factors.@*RESULTS@#Among the 940 newly diagnosed ALL patients, 570 patients were male, and 370 patients were female, the median age of onset was 5 years old (from 1 to 15 years old). The complete reaction rate (CR) was 96.7%. Survival analysis of 916 ALL patients with CR estimated by follow up [ (median follow up period 64 months (from 3 to 123 months) ] showed that, the expected 10 year OS rate was (78.6±1.5)% and the EFS rate was (66.0±1.8)%. The long-term OS rate of standard risk, intermediate risk and high risk patients was (93.0±1.5)%, (77.6±2.7)%, and (59.3±3.7)%, respectively, and the long-term EFS rate in standard risk, intermediate risk and high risk patients was (84.2±2.2)%. (67.8±2.9)%, and (42.1±3.9)% respectively. 10 year OS rate in B-ALL patients (79.8±1.6)% was significantly higher than that in T-ALL patients (53.5±6.3)% (P<0.01). Among of all the patients, patients 201 (21.9%) relapsed, the median relapse time was 19 months (from 2 months to 81 months). The 10 year EFS rate was (81.7±3.7)% in the patients with MRD rate <0.01% after induction therapy, which was significantly higher than that in the patients with MRD rate>0.01% (48.4±9.8)%.@*CONCLUSION@#The therapeutic efficacy of the CCLG-ALL2008 protocol is closed to the level of supior study group in the world. Risk stratification can improve the outcome for childhood ALL. Immunophenotyping shows the outcome of B-ALL is better than that of T-ALL. MRD negative patients after induction therapy shows better prognosis compared with that of MRD positive patients.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Child , Child, Preschool , China , Disease-Free Survival , Female , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Prognosis , Prospective Studies , Treatment Outcome
7.
Journal of Experimental Hematology ; (6): 1831-1836, 2020.
Article in Chinese | WPRIM | ID: wpr-879979

ABSTRACT

OBJECTIVE@#To investigate the consistency between FCM and PCR on the detecting of MRD in TCF3-PBX1@*METHODS@#55 cases of paediatric TCF3-PBX1@*RESULTS@#Among the 55 children with TCF3-PBX1@*CONCLUSION@#The detection result of MRD in TCF3-PBX1 detect by FCM and PCR shows better consistency. MRD positivity detected by FCM at the end of induction therapy (day 33) predicts a high risk of relapse in TCF3-PBX1 ALL patients.


Subject(s)
Adolescent , Bone Marrow , Child , Child, Preschool , Female , Humans , Male , Neoplasm, Residual , Oncogene Proteins, Fusion/genetics , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Recurrence
8.
Article in Chinese | WPRIM | ID: wpr-879790

ABSTRACT

OBJECTIVE@#To study the clinical significance of minimal residual disease (MRD) in B-lineage acute lymphoblastic leukemia (B-ALL) pediatric patients with different fusion gene backgrounds.@*METHODS@#A retrospective analysis was performed on the medical data of 441 B-ALL children who were treated from January 2008 to April 2015. Among the 441 children, 336 had negative fusion gene, 79 had positive @*RESULTS@#In patients with negative fusion gene, the positive MRD group had significantly lower overall survival (OS) rate and event-free survival (EFS) rate (@*CONCLUSIONS@#MRD has the most definite prognostic significance in pediatric B-ALL patients with negative fusion gene, while it has unsatisfactory prognostic significance in those with positive


Subject(s)
Child , Core Binding Factor Alpha 2 Subunit , Disease-Free Survival , Homeodomain Proteins , Humans , Neoplasm, Residual/genetics , Oncogene Proteins, Fusion/genetics , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/genetics , Prognosis , Retrospective Studies
9.
Article in Chinese | WPRIM | ID: wpr-879771

ABSTRACT

OBJECTIVE@#To study the pharmacokinetic characteristics, clinical effect, and safety of pegylated recombinant human granulocyte colony-stimulating factor (PEG-rhG-CSF) in children with acute lymphoblastic leukemia (ALL).@*METHODS@#A prospective study was performed on children with ALL who cyclophosphamide, cytarabine, and 6-mercaptopurine were used for consolidation therapy. PEG-rhG-CSF (PEG-rhG-CSF group) or rhG-CSF (rhG-CSF group) was injected after chemotherapy. The plasma concentration of PEG-rhG-CSF was measured, and clinical outcome and safety were observed for both groups.@*RESULTS@#A total of 17 children with ALL were enrolled, with 9 children in the PEG-rhG-CSF group and 8 children in the rhG-CSF group. In the PEG-rhG-CSF group, the peak concentration of PEG-rhG-CSF was 348.2 ng/mL (range 114.7-552.0 ng/mL), the time to peak was 48 hours (range 12-72 hours), and the half life was 14.1 hours (range 11.1-18.1 hours). The plasma concentration curve of PEG-rhG-CSF was consistent with the mechanism of neutrophil-mediated clearance. Compared with the rhG-CSF group, the PEG-rhG-CSF group had a significantly shorter median time to absolute neutrophil count (ANC) recovery (P0.05).@*CONCLUSIONS@#The pharmacokinetic characteristics of PEG-rhG-CSF in children with ALL receiving consolidation chemotherapy are consistent with the mechanism of neutrophil-mediated clearance, with a short half life and fast recovery of ANC, and there are no significant differences in safety between PEG-rhG-CSF and rhG-CSF.


Subject(s)
Child , Granulocyte Colony-Stimulating Factor/therapeutic use , Humans , Neutropenia , Polyethylene Glycols , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Prospective Studies , Recombinant Proteins
10.
Chinese Pharmaceutical Journal ; (24): 1433-1438, 2020.
Article in Chinese | WPRIM | ID: wpr-857597

ABSTRACT

OBJECTIVE: To investigate the effect and mechanism of Eucommia ulmoides polysaccharide (EUP) on db/db diabetic mice's glycolipid metabolism. METHODS: The db/db type 2 diabetes mice model were randomly divided into model control group, EUP low, middle and high dose group and positive control metformin group,-+/db mice of the same age were set as normal control group. The mice were weighed, and the changes of fasting blood glucose (FBG), blood lipid (TG, TC, LDL-C, HDL-C), interleukin (IL-8, IL-1β, IL-6), superoxide dismutase (SOD), glutathione peroxidase (GSH-Px) and malondialdehyde (MDA) were detected after drug delivery for 9 weeks. HE staining and oil red O staining were used to evaluate the pathological changes and steatosis of liver in mice. Western blot assay were used to detect the protein expressions of NF-κB p65 and HO-1 in liver tissues of mice. RESULTS: EUP could reduce the body weight of db/db mice, reduce the levels of FBG, TG, TC, LDL-C, IL-8, IL-1β, IL-6, MDA, NF-κB p65 proteins and increase the levels of HDL-C and HO-1 proteins. EUP could alleviate pathological changes and steatosis of liver in db/db mice. CONCLUSION: The obesity, hyperglycemia, hyperlipidemia and liver injury of db/db mice with type 2 diabetes are all improved by EUP, and the mechanism is related to the regulation of inflammatory response and oxidative stress.

11.
Article in Chinese | WPRIM | ID: wpr-776659

ABSTRACT

OBJECTIVE@#To investigate the complications and clinical outcome of children with acute myeloid leukemia (AML) undergoing mitoxantrone-cytarabine-etoposide (MAE) induction therapy.@*METHODS@#A total of 170 children with AML were given MAE induction therapy, and the complications and remission rate were analyzed after treatment.@*RESULTS@#The male/female ratio was 1.33:1 and the mean age was 7.4 years (range 1-15 years). Leukocyte count at diagnosis was 29.52×10/L [range (0.77-351)×10/L]. Of all children, 2 had M0-AML, 24 had M2-AML, 2 had M4-AML, 48 had M5-AML, 3 had M6-AML, 7 had M7-AML, 69 had AML with t(8;21)(q22;q22), and 15 had AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22). The most common complication was infection (158/170, 92.9%). Among these 158 patients, 22 (13.9%) had agranulocytosis with pyrexia (with no definite focus of infection), and 136 (86.1%) had definite focus of infection (including bloodstream infection). Other complications included non-infectious diarrhea, bleeding, and drug-induced hepatitis. Treatment-related mortality was observed in 10 children, among whom 8 had severe infection, 1 had multiple organ failure, and 1 had respiratory failure. Remission rate was evaluated for 156 children and the results showed a complete remission rate of 85.3%, a partial remission rate of 4.5%, and a non-remission rate of 10.3%.@*CONCLUSIONS@#Induction therapy with the MAE regimen helps to achieve a good remission rate in children with AML after one course of treatment. Infection is the main complication and a major cause of treatment-related mortality.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Child , Child, Preschool , Cytarabine , Drug Administration Schedule , Etoposide , Female , Humans , Infant , Leukemia, Myeloid, Acute , Drug Therapy , Male , Mitoxantrone , Remission Induction
12.
Article in Chinese | WPRIM | ID: wpr-774063

ABSTRACT

OBJECTIVE@#To study the long-term clinical effect of CCLG-ALL2008 regimen in the treatment of children and adolescents, aged >10 years, with newly diagnosed acute lymphoblastic leukemia (ALL).@*METHODS@#A retrospective analysis was performed for the clinical data of 150 ALL children and adolescents aged >10 years who were treated with CCLG-ALL2008 regimen from April 2008 to April 2015. The Kaplan-Meier method was used to analyze overall survival (OS) rate and event-free survival (EFS) rate.@*RESULTS@#Among the 150 children and adolescents, there were 87 (58.0%) boys and 63 (42.0%) girls, with a median age of 11 years (range 10-15 years). Of the 150 children and adolescents, 84 (56.0%) had intermediate risk and 66 (44.0%) had high risk; 122 (81.3%) had B-lineage acute lymphoblastic leukemia (B-ALL) and 28 (18.7%) had T-lineage acute lymphoblastic leukemia (T-ALL). The fusion gene test yielded positive results in 51 children and adolescents (34.0%), among whom 16 (31%) had positive BCR-ABL, 11 (22%) had positive TEL-AML1, 8 (16%) had positive E2A-PBX1, and 16 (31%) were positive for other fusion genes. The complete remission rate was 96.0% (144/150) after one course of treatment with CCLG-ALL2008 regimen. The median follow-up time was 52 months (range 3-122 months). The 5-year OS rate was 79.0%±3.5%, and the 5-year EFS rate was 67.3%±4.1%. There were no significant differences in 5-year OS and EFS rates between the children with intermediate or high risk, as well as between the children with B-ALL or T-ALL (P>0.05). The children and adolescents who achieved complete remission of bone marrow at the end of induction therapy had significantly higher 5-year OS and EFS rates than those who did not achieve complete remission (P10 years, CCLG-ALL2008 regimen can help to achieve high complete remission rate, 5-year OS rate and 5-year EFS rate. The children and adolescents failing to achieve complete remission at the end of induction therapy tend to have a poor prognosis.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Child , Disease-Free Survival , Female , Humans , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Prognosis , Remission Induction , Retrospective Studies
13.
Article in Chinese | WPRIM | ID: wpr-775109

ABSTRACT

OBJECTIVE@#To study the association of platelet level at diagnosis with prognosis in children with acute lymphoblastic leukemia (ALL).@*METHODS@#A total of 892 children with ALL who underwent chemotherapy with the CCLG-ALL 2008 regimen were enrolled. According to the platelet count at diagnosis, these children were divided into normal platelet count group (platelet count ≥100×109/L; n=263) and thrombocytopenia group (platelet count 0.05). The normal platelet count group still had a significantly higher 10-year EFS rate than the thrombocytopenia group after the children with MLL gene rearrangement were excluded (P0.05). The <20×10/L subgroup had significantly lower 10-year EFS and OS rates than the normal platelet count group, the (50- <100)×10/L subgroup, and the (20- <50)×10/L subgroup (P<0.05). After the children with MLL gene rearrangement were excluded, the <20×10/L subgroup still had significantly lower 10-year EFS and OS rates than the normal platelet count group, the (50-<100)×10/L subgroup, and the (20- <50)×10/L subgroup (P<0.05).@*CONCLUSIONS@#ALL children with MLL gene rearrangement often have the clinical manifestation of thrombocytopenia. Platelet level at diagnosis is associated with the prognosis of ALL children. The children with normal platelet count have a low recurrence rate and good prognosis, and those with a platelet count of <20×10/L have the worst prognosis.


Subject(s)
Child , Disease-Free Survival , Humans , Immunophenotyping , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Recurrence
14.
Article in Chinese | WPRIM | ID: wpr-775087

ABSTRACT

OBJECTIVE@#To study the long-term clinical effect of the CCLG-ALL2008 regimen in the treatment of children newly diagnosed with acute lymphoblastic leukemia (ALL) with different molecular biological features.@*METHODS@#A total of 940 children who were newly diagnosed with ALL were enrolled in this study. The children were treated with the CCLG-ALL2008 regimen. A retrospective analysis was performed for the long-term outcome of ALL children with different molecular biological features.@*RESULTS@#Among the 940 children with ALL, there were 570 boys and 370 girls, with a median age of onset of 5 years (range 1-15 years) and a median follow-up time of 65 months (range 3-123 months). The complete response (CR) rate was 96.7%, the predicted 10-year overall survival (OS) rate was 76.5%±1.5%, and the event-free survival (EFS) rate was 62.6%±3.0%. After CR was achieved after treatment, the overall recurrence rate was 21.9%. The children with positive ETV6-RUNX1 had the lowest recurrence rate and were prone to late recurrence, and those with positive MLL rearrangement had the highest recurrence rate and were prone to early recurrence. The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year OS rate than those with positive TCF3-PBX1, BCR-ABL, or MLL rearrangement and those without molecular biological features (P<0.05). The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year EFS rate than those with positive BCR-ABL or MLL rearrangement (P<0.05).@*CONCLUSIONS@#Molecular biological features may affect the long-term prognosis of children with ALL, and positive MLL rearrangement and BCR-ABL fusion gene are indicators of poor prognosis. Children with positive ETV6-RUNX1 fusion gene have the highest long-term survival rate.


Subject(s)
Adolescent , Antineoplastic Combined Chemotherapy Protocols , Therapeutic Uses , Child , Child, Preschool , Disease-Free Survival , Female , Fusion Proteins, bcr-abl , Humans , Infant , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Drug Therapy , Prognosis , Retrospective Studies
15.
Article in Chinese | WPRIM | ID: wpr-802321

ABSTRACT

Objective:To explore the molecular mechanism of Lycii Fructus and Salviae Miltiorrhizae Radix et Rhizoma (FLRSM) in the treatment of retinitis pigmentosa (RP) based on network pharmacology and bioinformatics. Method:Possible intake active components and targets of FLRSM were screened out and predicted by traditional Chinese medicine systems pharmacology database and analysis platform(TCMSP). Gene targets related to RP were mined through disease gene databases. Protein-protein interaction (PPI) network of component-targets and disease-targets were mapped by functional protein association networks (STRING), and the intersection of the two networks was obtained. The gene ontology and kyoto encyclopedia of genes and genomes(KEGG) pathway of the intersection network were analyzed by the database for annotation, visualization and integrated discovery(DAVID). CytoHubba analysis was used to screen out the key targets. Result:A total of 390 active ingredients related to FLRSM were retrieved from TCMSP. According to pharmacokinetic parameters, 110 active ingredients were screened out, 19 active ingredients were further screened out, and 208 targets related to these constituents were retrieved. Totally 206 genes directly related to RP were obtained from the disease gene databases. And 79 genes were obtained from the intersection of PPI networks of component targets and disease targets. These genes mainly involved in biological processes, such as protein autophosphorylation, transcriptional regulation and cell proliferation, and the molecular functions mainly involved adenosine triphosphate binding, transcription factor activity, core promoter binding, and were enriched in nuclear, transcription factor complex, nucleus, cytoplasm and other regions. It was mainly related to neurotrophin signaling pathway, cell cycle related pathway and Wnt signaling pathway. And 8 key gene targets for FLRSM treatment of RP were identified by further screening. Conclusion:The material basis of pharmacodynamic action of FLRSM involves 19 active ingredients, such as porous sterol and tanshinone ⅡA. The key targets of FLRSM in the treatment of RP include 8 genes, such as E2F transcription factor 1(E2F1) and retinoblastoma gene1(RB1).The main mechanism is related to the regulation of neurotrophin signaling pathways, cell cycle related pathways and other signaling networks.

16.
Article in Chinese | WPRIM | ID: wpr-696172

ABSTRACT

Objective To explore the new clinical significance of these routine test indexes and find the early biomarkers for predicting hypertension disorder complicating pregnancy(HDP) by the comparative analysis between pregnant women with HDP and normal pregnant women before 20 weeks,including biochemical,blood coagulation and blood routine indexes.Methods A case-control study was conducted to review the clinical data of pregnant women who were undertaken prenatal examination and delivered in Guangzhou Women and Children's Medical Center from 2012 to 2016.The pregnancy were divided into HDP group and control group according to the gestational week and age.Analyzed the related test indexes before 20 weeks of pregnancy.The two groups variables were analyzed by Kolmogorov-Smirnov for normal distribution.Approximate measurement data of normal distribution were mean± standard deviation (x± s) for statistical description,while the data of the skewed distribution was median and percentile[M(P25~P75)]for statistical description.The diagnostic value was analyzed by logistic regression equation and receiver operating characteristic curves (ROC).Results The level of biochemical indexes (AST,γ GT and UA) of HDP group was significantly higher thanthe normal group (t=2.50,3.34,4.56,P< 0.05).Meanwhile the level of blood indexes (RBC,PLT and HCT) of HDP group was significantly higher than the normal group (t=2.89,4.51,3.29,all P<0.01).Other indicators of two groups (ALT,TBIL,Cr,Urea,PT,APTT,TT,FIB and HGB) were not significantly different (t=0.25~ 1.85,all P>0.05).Then the logistic regression model equation was Y=-5.497+0.010 * PLT+0.043 * γ-GT+0.007 * UA+0.045 * AST.The area under ROC curve (AUC) was 0.746 for the combination of the four indexes.The combination resulted in a higher sensitivity of 0.818 and specificity of 0.523.Conclusion Before 20 weeks of gestation,compared with normal group,liver and kidney function in patients with HDP and high blood coagulation state was damaged.Can by combined detection of AST,UA,γ-GT and PLT index,early prevention and diagnosis of gestational hypertension,effective intervention measures taken as soon as possible to improve the prognosis of pregnancy.

17.
Article in Chinese | WPRIM | ID: wpr-351407

ABSTRACT

<p><b>OBJECTIVE</b>To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA).</p><p><b>METHODS</b>The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed.</p><p><b>RESULTS</b>The 5-year overall survival rate of the 231 patients was 82.7%. Except for 18 cases of early deaths, 213 patients were evaluated for IST efficacy. Among the 231 patients, cytogenetic abnormalities for at least two chromosome metaphase were detectable in 14 (7.4%) patients, and PNH clones were detectable in either peripheral red blood cells or neutrophils for 95 patients. Among the 213 patients evaluated for IST efficacy, 15 patients experienced clonal evolution after IST. Five patients had PNH and trisomy 8 which were defined as favorable progressions, and ten patients experienced monosomy 7 and MDS/AML as unfavorable progressions. The 5-year accumulative incidence of favorable and unfavorable progression were (2.2±2.2)% and (4.8±3.3)%, respectively. Until the last follow-up, 100% (5/5) of patients with favorable progressions and 50% (5/10) of patients with unfavorable progressions survived. WBC>3.5×10/L, CD3T cell percentage>80%, dosage of antithymocyte globulin >3.0 mg/(kg·d) and no response to IST were related to unfavorable progressions by univariate analysis. Cox multivariate analysis revealed that an increased CD3T cell percentage (>80%) and no response to IST were independent risk factors for unfavorable progressions.</p><p><b>CONCLUSIONS</b>The children with SAA/VSAA who have an increased CD3T cell percentage at diagnosis or have no response to IST are in high risks of unfavorable progressions.</p>


Subject(s)
Adolescent , Anemia, Aplastic , Drug Therapy , Genetics , Allergy and Immunology , Mortality , Child , Child, Preschool , Chromosome Aberrations , Clonal Evolution , Female , Humans , Immunosuppressive Agents , Therapeutic Uses , Infant , Male , Proportional Hazards Models , Retrospective Studies
18.
Article in Chinese | WPRIM | ID: wpr-261243

ABSTRACT

<p><b>OBJECTIVE</b>To identify the incidence of PAX5 deletion in childhood B-lineage acute lymphoblastic leukemia (B-ALL) without reproducible chromosomal abnormalities and to investigate the association between PAX5 abnormalities and prognosis of ALL.</p><p><b>METHODS</b>Multiplex ligation-dependent probe amplification was used to determine the copy numbers of PAX5 gene in children newly diagnosed with B-ALL without reproducible chromosomal abnormalities between April 2008 and April 2013 and controls (children with non-hematologic diseases or tumors). The patients were classifiied into deletion group and non-deletion group based on the presence of PAX5 deletion.</p><p><b>RESULTS</b>Eighteen (21%) out of 86 children with B-ALL had PAX5 deletion. The deletion group had a significantly higher total white blood cell count at diagnosis than the non-deletion group (P=0.001). The Kaplan-Meier analysis demonstrated that the deletion group had a significantly lower disease-free survival (DFS) rate than the non-deletion group (0.69±0.12 vs 0.90±0.04; P=0.017), but there was no significant difference in the overall survival rate between the two groups (P=0.128). The Cox analysis showed that PAX5 deletion was a risk factor for DFS (P=0.03).</p><p><b>CONCLUSIONS</b>PAX5 deletion is an independent risk factor for DFS in B-ALL children without reproducible chromosomal abnormalities.</p>


Subject(s)
Acute Disease , Adolescent , Cell Lineage , Child , Child, Preschool , Chromosome Aberrations , Disease-Free Survival , Female , Gene Deletion , Humans , Infant , Male , PAX5 Transcription Factor , Genetics , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma , Genetics , Mortality
19.
Article in Chinese | WPRIM | ID: wpr-261155

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the association between clinical outcome and gene mutations in children with Fanconi anemia (FA).</p><p><b>METHODS</b>A retrospective analysis was performed for the clinical data of six children with the same severity of FA and receiving the same treatment. At first, single cell gel electrophoresis and chromosome breakage induced by mitomycin C were performed for diagnosis. Then the gene detection kit for congenital bone marrow failure diseases or complementation test was used for genotyping of FA. Finally the association between the clinical outcome at 3, 6, 9, or 12 months after treatment and gene mutation was analyzed.</p><p><b>RESULTS</b>Of all the six FA children, five had FANCA type disease, and one had FANCM type disease; four children carried two or more FA gene mutations. Among the children with the same severity of FA, those with more FA mutations had a younger age of onset and poorer response to medication, and tended to progress to a severe type.</p><p><b>CONCLUSIONS</b>Children carrying more than two FA mutations have a poor clinical outcome, and hematopoietic stem cell transplantation should be performed as soon as possible.</p>


Subject(s)
Child , Child, Preschool , Fanconi Anemia , Genetics , Female , Humans , Male , Mutation , Retrospective Studies
20.
Article in Chinese | WPRIM | ID: wpr-279901

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the application of multiplex ligation-dependent probe amplification (MLPA) in the detection of copy number variations (CNVs) in pediatric ETV6/RUNX1-positive acute lymphoblastic leukemia (ALL), to compare this method with conventional karyotype analysis and fluorescence in situ hybridization (FISH), and to evaluate the value of MLPA.</p><p><b>METHODS</b>The clinical data of 95 children with ETV6/RUNX1-positive ALL who were treated from January 2006 to November 2012 were analyzed retrospectively, including clinical features, results of karyotype analysis, and results of FISH. CNVs were detected with MLPA.</p><p><b>RESULTS</b>CNVs were detected in 73 (77%), and the median number of CNVs was 1 (range 0-6). The CNVs of EBF1, CDKN2A/2B, PAX5, ETV6, RB1, and BTG1 were detected in more than 10% of all the patients. The changes in the chromosome segments carrying the genes with CNVs detected by MLPA were not detected by conventional karyotype analysis. The coincidence rate between the CNVs in ETV6 gene detected by FISH and those detected by MLPA was 66%.</p><p><b>CONCLUSIONS</b>MLPA is an efficient and convenient method to detect CNVs in children with ETV6/RUNX1-positive ALL.</p>


Subject(s)
Adolescent , Child , Child, Preschool , Core Binding Factor Alpha 2 Subunit , DNA Copy Number Variations , Female , Humans , In Situ Hybridization, Fluorescence , Infant , Male , Multiplex Polymerase Chain Reaction , Methods , Oncogene Proteins, Fusion , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Genetics
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