Objective To investigate the clinical outcomes of patients undergoing microsurgery for cerebellar medulloblastoma. Methods This retrospective analysis of the clinical and follow-up data involves 57 patients who received microsurgery for pathologically confirmed cerebellar medulloblastorna, and the microsurgical techniques for medulloblastoma were discussed. Results Among the 57 patients, 42 had total tumor resection, 13 had subtotal and 2 had partial resection of the tumors. Patency of the midbrain aqueduct was achieved in all the cases after the surgery. Hydrocephalus was found in 43 patients before the operation and only in 16 patients after the operation. Tumor relapse occurred in 19 patients 2 years after the operation, including 8 with implantation metastasis compromising the central nervous system and 1 patient with frontal lobe metastasis who received reoperations. The earliest tumor relapse occurred 20 days after the surgery. The 2- and 5-year postoperative survival rates in these patients were 68.4% and 49.1%, respectively. Conclusion Good therapeutic effects can be achieved with total resection of the tumors and postoperative whole brain and spinal cord radiotherapy in patients with medulloblastomas.

The "Medium and Long-term Plan for the Prevention and Control of Chronic Non-communicable Diseases in Shanghai (2018-2030)" was officially released in August 2018.From the perspective of public health, this paper analyzes the background of the plan from the epidemic situation, response and challenges Shanghai City is facing, expounds the comprehensive prevention and control system of chronic diseases including four functional systems, and explains the key preventive and control measures on the different stages of chronic diseases, comparing the evaluation indicators with those of the national plan.This paper will help to better understand the new blueprint for the prevention and control of chronic diseases in Shanghai in the next ten years.

In addition to its well established role as a neurotransmitter, extracellular ATP has been considered as a paracrine/autocrine factor, either released from sperm or epithelial cells, in the male reproductive tract and shown to play a versatile role in modulating various reproductive functions. This review summarizes the signal pathways through which ATP induces anion secretion by the epithelia of the epididymis, as well as its epithelium-dependent modulation of smooth muscle contraction of the vas deferens. Finally, the overall role of ATP in coordinating various reproductive events in the male genital tract is discussed.
Adenosine Triphosphate ; physiology ; Animals ; Epididymis ; physiology ; Epithelium ; physiology ; Humans ; Male ; Muscle Contraction ; Muscle, Smooth ; physiology ; Signal Transduction ; Urogenital System ; physiology ; Vas Deferens ; physiology

OBJECTIVETo investigate the chromosomal abnormalities and evaluate the prognostic value of post-remission chemotherapy in children with t (8;21) acute myeloid leukemia (AML).

METHODSThe diagnosis of AML and its subtyping were performed using morphological, immunological, and cytogenetic methodologies in 79 children. Induction therapies included homoharringtonine and cytarabine (HA), daunorubicin and cytarabine (DA), or homoharringtonine and daunorubicin and cytarabine (HAD). Allogeneic stem cell transplantation or 5-6 cycles of intensive chemotherapy was performed after remission therapy.

RESULTSAdditional chromosomal abnormalities, including loss of sex chromosome (n = 40, 50.6%), del (9q) (n = 9, 11.4%), and complex abnormality (n = 7, 8.9%) were identified in 55 patients (69.6%). Three patients had more than 90 chromosomes and duplicate t (8;21) tetraploid karyotype, and their prognoses were poor. The complete remission (CR) rates were 81.7% (49/60) and 94.8% (55/58), respectively, after one and two cycles of induction chemotherapy. The 3-year event-free survival rate (EFS), disease-free survival rate (DFS), and overall survival rate (OS) were (26.2 +/- 6.8)%, (31.3 +/- 6.7)%, and (27.6 +/- 6.6)%, respectively. Twenty-nine patients received 5 or more cycles of chemotherapy after CR and demonstrated an improved 3-year DFS [(51.7 +/- 9.3)%]. The 3-year DFS was not significantly differently in patients with or without additional abnormalities other than sex chromosome (P = 0.36). Post-remission consolidation by high dose cytarabine (HDAC) was significantly superior to standard chemotherapy (66.7% vs. 27.3%, P = 0.03).

CONCLUSIONMost children with t (8;21) AML have additional chromosomal abnormalities, although they do not affect the prognosis and long-term survival. Few patients have more than 90 chromosomes and duplicate t (8;21) tetraploid karyotype, which may result in poor prognosis. Childhood t (8;21) AML usually has high CR rate with relatively good prognosis, and post-remission consolidation by HDAC can improve the survival.

Adolescent ; Child ; Child, Preschool ; Chromosome Aberrations ; Chromosomes, Human, Pair 21 ; Chromosomes, Human, Pair 8 ; Female ; Humans ; Karyotype ; Leukemia, Myeloid, Acute ; genetics ; therapy ; Male ; Prognosis

OBJECTIVE To observe the clinical effect of Sanhuang Tang in the treatment of polycystic ovary syndrome(P-COS)of phlegm dampness heat accumulation type.METHODS Sixty-five PCOS patients of phlegm dampness heat accumula-tion type were randomly divided into the Chinese medicine plus group and the western medicine control group.Both group were taking metformin orally,on the basis of this the Chinese medicine group was also taking Sanhuang Tang granules orally.The clinical effect was observed for three months.Clinical symptoms,sex hormone levels,body mass and insulin resistance meta-bolic indexes were analyzed before and after the treatment.RESULTS The improvements of clinical symptoms,sex hormone levels,body mass and insulin resistance indexes in the Chinese medicine plus group were better than those in the control group(P<0.05～0.01).CONCLUSIONS The clinical effect of Sanhuang Tang plus group is better than that of the western medi-cine alone control group.

OBJECTIVETo 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.

METHODSThe 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.

RESULTSCNVs 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%.

CONCLUSIONSMLPA is an efficient and convenient method to detect CNVs in children with ETV6/RUNX1-positive ALL.

Adolescent ; Child ; Child, Preschool ; Core Binding Factor Alpha 2 Subunit ; analysis ; DNA Copy Number Variations ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Infant ; Male ; Multiplex Polymerase Chain Reaction ; methods ; Oncogene Proteins, Fusion ; analysis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics

OBJECTIVETo identify IKZF1 gene copy number abnormalities in BCR/ABL-negative B-lineage acute lymphoblastic leukemia (B-ALL) in children, and to investigate the association between such abnormalities and prognosis.

METHODSMultiplex ligation-dependent probe amplification (MLPA) was applied to detect IKZF1 gene copy number abnormalities in 180 children diagnosed with BCR/ABL-negative B-ALL. These children were classified into IKZF1 deletion group and IKZF1 normal group according to the presence or absence of IKZF1 gene deletion. The association between IKZF1 copy number abnormalities and prognosis of children with BCR/ABL-negative B-ALL was analyzed retrospectively.

RESULTSAmong 180 children, 27 (15.0%) had IKZF1 deletion; among the 27 children, 4 had complete deletions of 8 exons of IKZF1 gene, 17 had deletion of exon 1, 3 had deletions of exons 4-7, and 3 children had deletions of exons 2-7. Compared with those in the IKZF1 normal group, children in the IKZF1 deletion group had higher white blood cell (WBC) count and percentage of individuals with high risk of minimal residual disease at the first visit. IKZF1 deletions often occurred in BCR/ABL-negative children with no special fusion gene abnormalities. They were frequently accompanied by abnormalities in chromosomes 11, 8, 5, 7, and 21. The analysis with Kaplan-Meier method showed that disease-free survival (DFS) in the IKZF1 deletion group was significantly lower than that in the IKZF1 normal group (0.740 ± 0.096 vs 0.905 ± 0.034; P=0.002). Cox analysis showed that after exclusion of sex, age, initial WBC count, cerebrospinal fluid state at the first visit, prednisone response, and chromosome karyotype, IKZF1 deletion still affected the children's DFS (P<0.05).

CONCLUSIONSSome children with BCR/ABL-negative B-ALL have IKZF1 deletion, and IKZF1 deletion is an independent risk factor for DFS in children with BCR/ABL-negative B-ALL.

Adolescent ; Child ; Child, Preschool ; Female ; Fusion Proteins, bcr-abl ; analysis ; Gene Dosage ; Humans ; Ikaros Transcription Factor ; genetics ; Infant ; Male ; Multiplex Polymerase Chain Reaction ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; mortality ; Prognosis

OBJECTIVETo investigate the methylation rate of cyclin-dependent kinase inhibitor 2A (CDKN2A) and cyclin-dependent kinase inhibitor 2B (CDKN2B) in the 9P21 region in children with acute myeloid leukemia (AML) and the association of gene methylation with clinical features and outcomes.

METHODSThe clinical data of 58 children who were newly diagnosed with AML between January 2010 and December 2012 were retrospectively analyzed. Thirty-eight healthy children were recruited as the control group. Genomic DNA was extracted from bone marrow or peripheral blood of the 58 patients and 38 healthy children. The methylation status of CDKN2A and CDKN2B was analyzed by methylation-specific multiplex ligation-dependent probe amplification.

RESULTSGene methylation was not found in healthy children. Methylation probes of 44 patients were detected in 58 patients. The methylation of CDKN2A was detected with 136 bp and 237 bp methylation probes. The methylation of CDKN2B was detected with 130 bp, 210 bp, 220 bp, and 417 bp methylation probes. The methylation rate of CDKN2A was 5%, while the methylation rate of CDKN2B was 76%. The methylation detected by some probes was associated with sex, hemoglobin, and platelet count at the first visit.

CONCLUSIONSThe methylation of CDKN2B is a common event in children with AML, while the methylation of CDKN2A is relatively rare.

Adolescent ; Child ; Child, Preschool ; Cyclin-Dependent Kinase Inhibitor p15 ; genetics ; Cyclin-Dependent Kinase Inhibitor p16 ; genetics ; DNA Methylation ; Female ; Humans ; Infant ; Leukemia, Myeloid, Acute ; genetics ; Male

OBJECTIVETo study the long-term efficacy of CAMSBDH-ALL chemotherapy protocol for the treatment of childhood acute lymphoblastic leukemia (ALL).

METHODSThree hundred and eighteen children who were newly diagnosed with ALL between January 1999 and December 2007 were enrolled in this study. Among the 318 children, 83 children who hospitalized before December 2002 were treated with CAMSBDH-ALL99 protocol, including 48 patients of standard risk and 35 patients of high risk. The patients (n=235; 131 in standard risk and 104 in high risk) who hospitalized after December 2002 were treated with CAMSBDH-ALL03 protocol. Patients in the CAMSBDH-ALL99 protocol group were treated with conventional chemotherapy. CAMSBDH-ALL03 protocol was modified based on the CAMSBDH-ALL99 protocol.

RESULTSThe long-term overall survival (OS) and event-free-survival (EFS) in the CAMSBDH-ALL03 group was significantly higher than in the CAMSBDH-ALL99 (P<0.01). The long-term OS and EFS of standard risk and high risk patients in the CAMSBDH-ALL03 protocol group were significantly higher than in the CAMSBDH-ALL99 protocol group (P<0.01). The CAMSBDH-ALL03 protocol group showed a significantly lower recurrence rate (28.9%) than in the CAMSBDH-ALL99 protocol group (50.6%) (P<0.05). The mortality rate in the CAMSBDH-ALL03 protocol group was 28.5% vs 56.6% in the CAMSBDH-ALL99 protocol group (P<0.05).

CONCLUSIONSThe therapeutic effect of the CAMSBDH-ALL03 protocol is supior to the CAMSBDH-ALL99 protocol group for childhood ALL, with a higher long-term survival rate.

Adolescent ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Clinical Protocols ; Female ; Humans ; Infant ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; mortality ; Recurrence

OBJECTIVETo 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.

METHODSMultiplex 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.

RESULTSEighteen (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).

CONCLUSIONSPAX5 deletion is an independent risk factor for DFS in B-ALL children without reproducible chromosomal abnormalities.

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