Objective To study the clinical features of diagnosis and treatment in children with low-risk myelodysplastic syndrome(MDS) and improve the diagnosis and treatment.Methods The examinations of 17 children with low-risk MDS were analyzed.The blood concentrations of cyclosporine,one of the therapeutic drugs,were monitored and the responses to the treatments were evaluated.Results Exa-mination of full blood count showed that the reductions of 3 cell types,2 cell types and 1 cell type were 11 (64.7%)cases,5(29.4%)cases and 1(5.9%)case,respectively.Reticulocyte count showed an increase in 82.4% of the patients and normal in 3 cases.Fourteen in 17 cases were hyperplastic marrow and 3 cases were hypoplastic marrow.Among all cases,one lineage,2 lineages and 3 lineages dyspoiesis were seen in 8(47.1%),7(41.2%) and 1 (5.9%)cases,respectively.One case showed no dyspoiesis.Cytogenetics examination showed normal in 10(58.8%) cases and abnormal in 7(41.2%) cases.Fifteen (88.2%) cases had normal proportions of CD59 negative cells,while 2 cases had higher proportions.The blood concentrations of cyclosporine that were tested in 9 cases at the end of the third week were in a range of 95.3-316.5 ?g/L.The therapeutic effect of 10 cases were evaluated at the end of the third month after being treated.Eight cases achieved haematological improvement and 2 cases didn′t.The rate of improvement was 80%.Conclusions The patients of low-risk MDS are mostly school-aged children and pancytopenia is the most common sign.The combination of predisone,cyclosporine and stanozolol agents shows good effect to treat low-risk MDS.The absorption of cyclosporine is different individually,so it is significant to adjust the dosage of cyclosporine according to the concentration regularly in clinical practice.

Adult ; Aminosalicylic Acid ; therapeutic use ; Female ; Humans ; Manganese Poisoning ; drug therapy

Objective To explore the clinical characteristics of nosocomial infection in children with acute leukemia and the strategy of prevention and treatment.Methods One hundred and thirty-three cases of nosocomial infection in children with acute leukemia were analyzed by retrospective study.The relationship between nosocomial infection and stage of leukemia,hospitalization duration,and the rate of infection were investigated.Results Nosocomial infection rate was 53.4%(71/133 cases),significant difference of infection rate between acute lymphoblastic leukemia and nonlymphoblastic leukemia group was found(P0.05).The main pathogens of septicaemia were gram negative bacilli,and they were generally sensitive to Amicacin and Pi-peracillin/tazobactam.Conclusions Children with acute leukemia have high nosocomial infection rate.The occurrence of nosocomial infection was related to the type and stage of leukemia and hospitalization duration but not to the prognosis.The main pathogens of septicaemia were gram negative bacilli.

The main biological function of cytotoxic T cell-associated protein 4 (CTLA-4) is to suppress the T cell response and suppress the immune response, and its mutation will cause a series of immune related abnormalities. This case reports a rare case of onset of lymphocytosis, immune hemolysis, repeated infection, and other similar symptoms of autoimmune lymphoproliferative syndrome which caused by CTLA4 Exon2 c. 151 C＞T mutation. Sequencing validation was performed to clarify the source of gene mutation. We review the pathogenesis of CTLA4 and new progress in treatment in this case, and the follow-up treatment for the patient was prospected.

Objective To study the regulatory effect of interferons(IFNs) on retinoic acid-inducible gene-Ⅰ(RIG-Ⅰ) and the roles of RIG-Ⅰ in IFNs signaling pathway. Methods RIG-Ⅰ expression before and after IFNs treatment in mouse embryonic fibroblasts(MEFs) were analyzed with Northern blotting and semi-quantitative RT-PCR assay.MEFs isolated from wild-type and RIG-Ⅰ-/-mice were used to test growth inhibition and antiviral activity of IFNs with MTT assay and cytopathic effect inhibition assay. Results Both IFN-? and IFN-? could induce RIG-Ⅰ expression in MEFs.Treated with 100 U/mL IFN-?,growth inhibition and antiviral activity of MEFs from wild-type mice were more significant than those from RIG-Ⅰ-/-mice.With the absence of RIG-Ⅰ,the antiviral protective role IFN-? plays was significantly weaker than the wild type. Conclusion RIG-Ⅰ gene is a novel mediator of interferon effects on cells.It may participate in the inflammation responses mediated by IFNs through modulating cytokines production.

Objective To investigate the condition of exercise, medicine compliance and the rehabilitation of patients with arteriosclerosis obliterans (ASO) in lower extremity after the surgery, and also to study the influence of self-efficiency and social support to their rehabilitation. Methods The questionnaire survey was performed in 102 patients who had surgical or interventional therapy of arteriosclerosis obliterans in the lower extremity in our hospital, investigated the general information of the patients, their knowledge of postoperative rehabilitation, medicine compliance, exercise condition, general self-efficiency and social support. Results The postoperative time for the patients with arteriosclerosis obliterans was 2 to 22months, the knowledge rate for postoperative rehabilitation was 7.8%-46.1%.The Morisky score for the patients of taking medicine for the treatment of vascular disease was 2.0,and there was only 6 patients(5.9%)with good medicine compliance. There were 22 patients(21.0%) had completed the ambulation training according to the guidelines, and there were 25 patients(24.5%) had completed the Buerger training. The self-efficiency score of the patients was 2.78 ± 0.76, and the gross score of social support was 32.38 ± 7.89. Spearman correlation analysis shows that the Morisky score for the patients of taking medicine for the treatment of vascular disease was in positive correlation with self-efficiency (r=0.289, P=0.003), but in negative correlation with social support (r=–0.286, P=0.006). Conclusions Most of the patients with arteriosclerosis obliterans have poor medicine compliance, and do not do exercise as required by the guidelines. Patients with high self-efficiency demonstrates medicine noncompliance, however the enhancement of social support will help patients improve their medicine compliance.

Biomechanical Phenomena ; Electromyography ; Gait ; Humans ; Lower Extremity ; Muscle, Skeletal ; Walking

OBJECTIVEThe study was aimed to investigate the feasibility and clinical significance of quantitative detection of minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) by real-time quantitative polymerase chain reaction (RQ-PCR).

METHODSClonal IgH gene rearrangements of samples at diagnosis were identified by standard PCR assay with consensus primers. Monoclonal IgH gene rearrangements were analyzed using DNAPLOT software. Upstream primers were designed with the Primer Express software and allele specific oligonucleotide developed complementary to the V-D or D-J junction. Samples at diagnosis were serially diluted to generate the patient specific standard curves. RQ-PCR method was used to quantify the MRD of the follow up samples collected at five time points during chemotherapy. To check the quantity and quality of DNA, the investigators used RQ-PCR analysis for the albumin gene.

RESULTSTotally 16 monoclonal IgH gene rearrangements were identified from 34 patients with B-ALL. The analysis of the 16 monoclonal rearrangements showed that the most frequently used V segment was from V3 family and J segment from J4 and J6. The RQ-PCR sensitivity of 10(-4) to 10(-5) was mostly reached. Non-specific amplification was seen in 6 patients. The number of inserted and deleted nucleotides did not appear to be related to the sensitivity (P > 0.05). The correlation coefficients of all 16 standard curves were excellent (> or = 0.99). The mean slope of the standard curves was -3.4 +/- 0.37 and the mean intercept was 24.3 +/- 2.95. MRD analysis of follow up samples from the 16 patients showed an association between high degree of MRD and relapse. There was no apparent relationship between MRD degree at the end of induction chemotherapy and other high risk factors of ALL (P > 0.05).

CONCLUSIONThe study showed that the above approach with RQ-PCR was applicable to clinical detection of MRD in childhood ALL. Quantitative and dynamic study of MRD was of prognostic importance.

Child ; Gene Rearrangement, B-Lymphocyte ; Humans ; Neoplasm, Residual ; diagnosis ; genetics ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; drug therapy ; genetics ; Prognosis

Objective To evaluate the risk factors of breast cancer in Asian women and to provide evidences for establishing a risk assessment model. Methods Published studies concerning risk factors of breast cancer in Asian women were searched systemically and assessed by NOS (Newcastle-Ottawa Scale) items between 1995 and 2010. RevMan 4.2 software was used for data analysis and for calculating OR and its 95%CI on every risk factor. Results 27 studies including 403170 women were selected for Meta-analysis. According to NOS items, 20 studies were classified as A degree and 7 studies were evaluated as B degree. The risk factors of breast cancer and its pooled odds ratio values with statistical significance were as follows: 3.00 (95%CI: 1.68-5.36) when number of abortions≥3; 2.39 (95%CI: 1.78-3.21 ) when with family history of breast cancer; 1.54(95%CI: 1.30-1.82) when age at first live birth ≥30 (year); smoking was 1.50(95%CI: 1.03-2.20); 1.48(95%CI:1.20-1.83) with no live births; 1.29 (95%CI: 1.12-1.47) with no breast feeding; 1.26 (1.07-1.49)with age at menarche ≤12 (year) and 1.16(95%CI: 1.01-1.32) with alcohol drinking. Conclusion Number of abortions≥3, family history of breast cancer, age at first live birth ≥30 (year) ,smoking, no live births, no breast feeding, age at menarche ≤ 12 (year), and alcohol drinking were among the priorities in the establishment of breast cancer risk assessment model for Asian women.

Objective To investigate the relationship of serum resistin and pancreatic necrosis in the patients with severe acute pancreatitis. Methods Twenty-eight patients with SAP admitted to our hospital from March 2008 to November 2008 were divided into two groups according to the CT scan imaging: necrotic group and non-necrotic group. The enzyme-linked immunosorbent assay (ELISA) was used to test the serum resistin levels. An ROC curve was depicted to predict the pancreas necrosis. Results There were 21 patients in the non-necrotic group and 7 in the necrotic group, and there was no significant difference in terms of sex, age and baseline disease (P>0.05). The resitin levels ranged from 0.1730 ng/ml to 7.4923 ng/ml, with a mean (3.7102±1.6987) ng/ml. The area under the curve of resistin values was 0.884±0.108 (95%CI:0.672～1.097), asymptote signals 0.003, then it was calculated that P=0.003, which was>0.50. Conclusions The serum resistin may be of clinical value to predict the pancreatic necrosis.