Primary myelofibrosis is a kind of chronic myeloproliferative neoplasms.The discovery of the JAK2V617F mutation as well as other molecular abnormlities underly the pathogenesis of Philadelphia negative myeloproliferative neoplasms (MPN).The initial description of JAK2V617F mutation in 2005,the reporting of calreticulin (CALR) mutations last year and the clinical application of JAK inhibitors,showed the gradually deepening understanding with regard to the pathogenesis and the development of therapeutic strategies for primary myelofibrosis (PMF).This article reviewed the progress in diagnosis,risk stratification of of PMF,the benefits and potential side effect of JAK inhibitors,which reported on the 2014 European Hematology Association annual congress.

Acute myeloid leukemia (AML) is a kind of genetic heterogeneous clonal hematopoietic stem cell disorder.Although there were improvements in the outcomes of selected younger patients and those with specific cytogenetic and molecular genetic characteristics,the overall survival for older patients remains dismal.In the last few years,next-generation sequencing technologies have identified recurrent mutations in genes encoding proteins involved in the epigenetic regulation of transcription in most patients with AML.This discovery has led to new insights into the role of the epigenome in AML and opens the possibility of epigenetically targeted therapies.This article will review the most important recurrent mutations in epigenetic regulatory genes and highlight the current and future treatment strategies that attempt to exploit epigenetic targets with the use of hypomethylating agents,which were reported on the 56th American Society of Hematology annual congress in 2014.

Objective To compare the characteristics of food and nutrition intake in type 2 diabetes mellitus (T2DM) patients with or without carotid atherosclerosis and analyze the relationship between diets/C-reactive protein (CRP) and carotid intima-media thickness (C-IMT). Methods Sixty patients with T2DM were enrolled and divided into two groups based on C-IMT: group A (C-IMT ＜ 1 mm, n=30) and group B (C-IMT≥1 mm, n=30). All subjects were investigated with questionnaires including 3-day food recall They all took somatometric measurement. Blood and urine samples were collected in all subjects to examine the levels of high sensitive-CRP,C-peptide, blood glucose, glycosylated hemoglobin, blood lipid, renal function, urine microalbumin, and other indicators. Results The intakes of vegetables, fruits, and aquatic products were significantly higher in group A than in group B ( P ＜ 0. 05 ). The intake of vitamin C in group A was significantly higher than that in group B ( P ＜0. 05 ). The levels of CRP in group B was significant higher than that in group A (P = 0. 000). Positive correlation was found between CRP level and C-IMT in T2DM patients ( r = 0. 36, P = 0. 004). Furthermore, CRP was negatively correlated with the intakes of vegetables and fruits ( r = - 0. 334, P = 0. 01 ), aquatic products ( r = -0. 315, P = 0. 016), and vitamin C ( r = - 0. 2786, P = 0. 038 ), respectively. The intake of fruits was negatively correlated with C-IMT (r, = -0. 33, P = 0. 01 ). Conclusions T2DM patients without carotid atherosclerosis intake more vegetables, fruits, aquatic products and vitamin C than those with atherosclerosis. Vegetables, fruits,sea foods and vitamin C may be the protective factors against atherosclerosis in T2DM patients. CRP is associated with the development of atherosclerosis in T2DM patients.

Sina 21 century it have seen major leaps in the understanding of the molecular genetic mutations that act as drivers of acute myeloid leukemia (AML). Clinical trials of targeted drugs against specific mutant proteins, such as FLT3-internal tandem duplications (ITD) and isocitrate dehydrogenase mutations (IDH) are ongoing. This review discusses agents in clinical trials that target specific gene mutations and/or epigenetic targets from the 57th American Society of Hematology annual meeting.

Objectives:To observe the efficacy of enteral nutrition with short peptide elemental diet(Pepti 2000) in patients with gastrointestinal fistulas and evaluate the sensitivity of nutrition associated index in nutrition monitoring. Methods:Ten cases with gastrointestinal fistulas were administered total enteral nutrition(TEN).They received 146 kJ/(kg?d) of non protein calorie(NPC) and 0.25 g/(kg?d) of nitrogen per day. Body weight,serum albumin, transferrin(TFN), prealbumin(PA),fibronectin(FN),triglyceride and cholesterol were monitored on before and 7th and 28th after TEN. Results:Seven days after TEN,serum PA and FN levels rised significantly( P

Great advances have been made in understanding the pathogenesis,diagnosis,risk stratification and treatment of classic bcr-abl-negative myeloproliferative neoplasms (MPN).This article reviews current situation in the treatment of these MPNs presented in the 56th American Society of Hematology (ASH) annual meeting.

Objective To compare the clinical effect of intraosseous versus traditional infusion in critical pediatric patients under resuscitation.Methods 56 critical pediatric patients under resuscitation,who were difficult to establish a venous access,were divided into two groups:intraosseous infusion group (IOI group)and traditional group.The clinical characteristics,lab tests,clinical efficacy and complications of all patients were recorded in detail. Results The general characteristics of two groups were comparable.The mean operation time to establish an emergency infusion access in the IOI group was (1.01 ±0.53)min,which was significantly shorter than (8.97 ±2.36)min of the traditional group(t =4.856,P <0.01).The IOI group had a greater efficacy over the traditional group (50.0% vs 36.7%),in the IOI group,the patients showed obvious positive effect and less patients had no efficacy(χ2 =18.476, P <0.05 ).In addition,lower complication rate and mortality were observed in the IOI group.Conclusion For critical pediatric patients under resuscitation whose intravascular access cannot be achieved through peripheral venous,intraosseous infusion is prior to traditional infusion.

Objective To explore the clinical efficacy,adverse reactions and serum levels of IL -10 and TNF-αin children with peptic ulcer undergoing sequential therapy.Methods 68 children with peptic ulcer were selected and randomly divided into two groups,34 cases in each group.The control group received quadruple therapy, namely omeprazole,amoxicillin,clarithromycin and bismuch treatment for seven days.The treatment group underwent sequential therapy:the first 5 d of oral omeprazole,amoxicillin treatment,and the next 5 d omeprazole,amoxicillin and inidazole treatment.The clinical efficacy,adverse reactions,IL-10 and TNF-αlevels of the two groups were com-pared.Results The total effective rates after treatment of the control group and treatment group were 88.24% and 91.18% respectively,there was no statistically significant difference between the two groups(χ2 =1.21,P>0.05). After treatment,the levels of IL-10 and TNF-αin the control group were (24.93 ±6.29)pg/mL and (37.93 ± 8.28)pg/mL,which were significantly decreased (t=5.52,P<0.05,t=8.51,P<0.01).And the levels of IL-10 and TNF-αin the treatment group were (21.36 ±6.31)pg/mL and (29.67 ±6.38)pg/mL,which were significantly decreased(t=11.15,12.29,all P<0.01).The levels of IL-10 and TNF -αof the treatment group were much significant than those of the control group after the sequential therapy (t=3.32,P<0.05,t=8.71,P<0.01). Conclusion Sequential therapy for the treatment of children with peptic ulcer has better effect than the quadruple therapy,and can reduce serum IL-10 and TNF-αlevels,it is worthy of promoting.

Objective To analyze the therapeutic effect of qiweiqingyan aerosol combined with ribavirin in the treatment of herpetic angina of children hand-foot-mouth disease,and to compare the clinical effects with sim-ple use of ribavirin.Methods 160 children with hand foot and mouth disease were divided into two groups by random number table.They were the observation group (80 cases)and control group (80 cases),respectively.Two groups were both given ribavirin aerosol in the treatment,the observation group were added with qiweiqingyan aerosol agent to carry out treatment,then the clinical curative effects of the observation group and control group were copared.Results In the observation group,the total effective rate was 96.25%,and the total effective rate of the control group was 85.00%,the difference between the two groups had statistical significance (χ2 =5.959,P<0.05).Marked effective rate of the observation group was 86.25%.In the control group,the significant efficiency was 37.50%.The difference between the two groups had statistical significance (χ2 =40.300,P<0.05 ).Defervescence time and bleb disappear time were shorter in the observation group compared to the control group,the difference between the two groups was statistically significant (t=47.880,8.063,5.100,all P<0.05).The adverse reactions in the observation group were significantly lower than those in the control group (χ2 =9.608,P<0.05 ),after statistical analysis.Conclusion Using qiweiqingyan aerosol combined with ribavirin in the treatment of children hand foot and mouth herpangina,can significantly shorten the time of treatment,reduce adverse reactions and improve the cure rate.

Objective To investigate the change of blood lactate level in children with severe sepsis,and its relationship with clinical prognosis.Methods 90 children with severe sepsis who treated in our hospital from February 201 3 to May 201 4 were selected as the study subjects.According to the prognosis of children,they were divided into survival group and death group,45 cases in each group.The blood lactate levels at different time points, blood lactate clearance rates between the two groups at different time points as well as fibrin,oxygenation index and D -dimer levels were compared after admission.Results After treatment,the fibrin,oxygenation index and D -dimer levels in the two groups were improved.The fibrin and D -dimer levels in the survival group[(2.71 ±0.31 )ng/mL, (0.89 ±0.1 0)mg/L)]were lower than those in the death group[(2.89 ±0.21 )ng/mL,(1 .26 ±0.1 8)mg/L)],the differences were significant(t =3.224,P =0.001 ;t =1 2.053,P =0.000).The oxygenation index of the survival group[(1 96.23 ±1 4.69)mmHg)]was higher than that of the death group [(1 80.23 ±21 .03 )mmHg)],the difference was significant(t =4.1 84,P =0.000).The EGOT compliance rate,APACHE Ⅱscore and MODS incidence rate of survival group were significantly lower than those of the death group,the differences were significant(t =7.200,P =0.007;t =9.1 49,P =0.000;t =29.298,P =0.000).The blood lactate levels at each time points in the survival group were significantly lower than the death group,the differences were statistically significant(t =50.543, P =0.000;t =33.932,P =0.000;t =1 7.91 5,P =0.000;t =28.703,P =0.000).The 6 h,24 h blood lactate clearance rates≥1 0% of the survival group (73.33%,80.00%)were significantly higher than those of the death group(37.78%,44.44%),the differences were significant(χ2 =1 1 .520,P =0.000;χ2 =1 2.1 00,P =0.000). Conclusion Lactate level in children with sepsis is an important indicator of prognosis in children with severe sepsis,with guidance for the treatment of children with sepsis.