Objective To evaluate the effect of breathing exercise program (shrinking lips abdominal breathing combined with vertical breathing gymnastics) on dyspnea, pulmonary function, exercise tolerance and quality of life in patients with moderate to severe chronic obstructive pulmonary disease(COPD). Methods A total of 90 hospitalized patients with moderate to severe COPD were randomly divided into the experimental group and the control group with 45 cases in each group. The experimental group lost one case and the control group lost three cases, 86 patients finished the experiment. During hospitalization, on the base of conventional treatment and care, the experimental group
received training of shrinking lips abdominal breathing combined with vertical breathing gymnastics after medical staff′s care, with folk music as the background. Exercise frequency:morning, afternoon, 2 times per day, 15 min per time and continue to exercise with researchers′ guidance after discharge. In the control group:take exercises by self, take routine follow-up after discharge. The intervention continued for three months. The indicators such as dyspnea, pulmonary function, exercise tolerance and life quality of both groups were assessed respectively before and after the intervention. Results There were no significant differences in dyspnea symptoms, lung function, exercise endurance, quality of life between two groups before intervention (P>0.05). The dyspnea scored 1.43±0.87 after intervention in the experimental group, and 1.93 ±0.97 in the control group, there was significant difference (Z=-2.293, P=0.022). The 6 min walking test distance was (371.34 ± 67.74) m after intervention in the experimental group, and (301.57 ± 61.67) m in the control group, there was significant difference(t = 4.988, P =0.000). The St. George′s Respiratory Questionnaire (respiratory symptoms, limited activity, influence disease) score and total score were 54.73 ± 11.96, 52.55 ± 14.48, 55.45 ± 10.01, 54.56 ± 10.79 after intervention in experimental group respectively, and 61.19 ± 10.72, 61.35 ± 14.66, 60.48 ± 9.39, 60.93 ± 10.16 in the control group, there were significant differences(t=-2.815--2.397, P<0.05). There was no significant difference in lung function after intervention between two groups (P>0.05). Conclusions The program of shrinking lips abdominal breathing combined with vertical breathing gymnastics can relieve dyspnea in patients with moderate to severe COPD, improve exercise tolerance and quality of life, which is a safe and effective rehabilitation for patients with COPD.

Objective To investigate the energy intake and complications of continuous and intermittent pump feeding in acute stroke patients and provide a theoretical evidence to support for clinical treatment.Methods From April 2012 to June 2016,69 acute srtoke patients on the nasogastric tube feeding and admitted in the Department of Neurology intensive care unit in Xijing Hospital were enrolled and randomly assigned to have continuous or intermittent pump feeding.The primary endpoint was the efficacy in supplying the desired amount of nutrients by the fifth day and complications(hospital-acquired pneumonia,diarrhea,gastric retention,gastrointestinal bleeding)during the first week.The secondary endpoint was nutritional assessments(albumin,prealbumin,transferrin,hypersensitive c-reactive protein)in the first week.Results Both groups were comparable in acute physiology and chronic health evaluation Ⅱ(13.3±4.7 vs.12.9±4.5),Glasgow coma scale[10(7.2,14)vs.9.5(7.7,12)],National Institute of Health stroke scale[17(15,19)vs.16(13,20)],and Barthel scores[5(0,12.5)vs.10(5,15)](all P>0.05).It was no significantly different in the achievement percentage of the energy determined in the fifth day [93.9％(77.9％,99.8％)in continuous group and 84.8％(75.7％,93.9％)in intermittent group(U=0.144,P>0.05).Intermittent pump feeding significantly reduced the rate of hospital-acquired pneumonia in the first week when compared with continuous pump feeding(58.3％vs.33.3％,X2=4.327,P=0.038),and both groups displayed a moderate number of digestive complications without significant differences,including diarrhea(30.6％vs.27.3％,X2=0.09,P=0.764),gastric retention(2.78％vs.3.03％,X2=0.001,P=1.000),and gastrointestinal bleeding(5.56％vs.9.10％,X2=0.010,P=0.920).No difference could be demonstrated in serum protein markers between two groups in the first week,including prealbumin[0.17(0.13,0.20)g/L vs.0.18(0.15,0.24)g/L,P=0.195),transferrin[1.90(1.52,2.20)g/L vs.1.94(1.65,2.06)g/L,P=0.747),and hypersensitive C-reactive protein[22.5(8.3,50.1)mg/L vs.14.6(6.5,30.5)mg/L,P=0.205].Conclusions Both continuous and intermittent pump feeding can reach the target predictive nutrition(100％of calculated caloric requirements)without statistical differences in the incidences of gastrointestinal complications,and the rate of HAP is lower in intermittent group.Intermittent enteral nutrition can be used as an appropriate method of enteral nutrition support to improve the nutritional status in critically acute stroke patients.

OBJECTIVETo analyze the clinical, laboratory characteristics and PIG-A gene mutations in patients of myelodysplastic syndromes (MDS) with PNH clones.

METHODS218 MDS patients diagnosed from August 2013 to August 2015 were analyzed. The PIG-A gene mutations were tested in 13 cases of MDS with PNH clones, 17 cases of AA-PNH and 14 cases of PNH selected contemporaneously by PCR and direct sequencing.

RESULTS13 (5.96%) MDS patients were detected with PNH clones (13/218 cases). 9 patients were treated with cyclosporin A (CsA). Patients showed hematological improvement (HI). There were significant differences between MDS-PNH and PNH patients in terms of granulocyte clone size, red cell clone size and LDH levels [19.2% (1.0%-97.7%) vs 60.2% (3.1%-98.0%), P=0.007; 4.3% (0-67.2%) vs 27.9% (2.5%-83.6%), P=0.026; 246 (89-2014) U/L vs 1137 (195-2239) U/L, P=0.049], while the differences were not statistically significant in patients between MDS-PNH and AA-PNH patients [19.2% (1.0%-97.7%) vs 23.2% (1.5%-96.0%), P=0.843; 4.3% (0-67.2%) vs 14.4% (1.1%-62.8%), P=0.079; 246 (89-2014) U/L vs 406 (192-1148) U/L, P=0.107]. PIG-A gene mutations were detected in 7 MDS-PNH patients, of them, six were missense mutations, one were frameshift mutation and four cases with the same mutation of c.356G>A (R119Q). The PIG-A gene mutations were also detected in 9/11 AA-PNH patients and 11/14 PNH patients, both of them had the mutation of c.356G>A (R119Q). The PIG-A gene mutations of MDS-PNH, AA-PNH, PNH patients were all small mutations, the majority of those (59%) were missense mutation and mainly located in exon 2.

CONCLUSIONMDS patients with PNH clones had better response to CsA, smaller PNH clone size. The PIG-A gene mutations of MDS-PNH patients mainly located in exon 2, which could be a mutational hotspot of these patients.

Anemia, Aplastic ; genetics ; Clone Cells ; Erythrocytes ; cytology ; Exons ; Granulocytes ; cytology ; Hemoglobinuria, Paroxysmal ; genetics ; Humans ; Membrane Proteins ; genetics ; Mutation ; Myelodysplastic Syndromes ; genetics ; Polymerase Chain Reaction

OBJECTIVETo estimate the long-term outcomes and the prognostic factors of homoharringtonine, cytarabine, daunorubicin or idarubicin (HAD/HAI) as induction chemotherapy in de novo acute myeloid leukemia (AML).

METHODSThe CR rate, overall survival (OS) rate, relapse free survival (RFS) rate were retrospectively assayed in 143 de novo AML patients who received the HAD/HAI induction chemotherapy. The outcomes were compared among prognostic groups according to world health organization (WHO) classification, genetic prognosis and initial white blood cell (WBC) count. The role of consolidation chemotherapy consisting of middle-dosage Ara-C (MD-Ara-C) on long term survival was evaluated.

RESULTSOf 143 patients, 112 (78.3%) achieved CR after the first course of HAD/HAI induction treatment, and early death occurred in only one case. Notably, the CR rate of patients with an initial WBC count ≥100×10(9)/L was not significantly different from those with an initial WBC count<100× 10(9)/L (70.4% vs 80.2%, P=0.266). The CR rate for the patients with favorable, intermediate and unfavorable integrated genetics risk factors was 93.7%, 71.4% and 61.3%, respectively, the difference between groups was statistically significant (P=0.001). Patients with FLT3-ITD mutation obtained similar CR rate (70.6%) to that of patients with FLT3 wild type (79.3%, P=0.528).The estimated 5-year OS rate and 5-year RFS rate for all patients was 40.0% and 37.0%, respectively, with a median follow-up of 24 (range 1-104) months. The median survival time was 30 [95%CI (12, 48)] months. 5-year OS and 5-year RFS of the 96 patients who achieved CR after first course chemotherapy without undergoing allo-HSCT in complete remission was 47.0% and 38.0%, respectively. 5-year OS was significantly higher in MD-Ara-C consolidation group than in no MD-Ara-C consolidation group among CR patients without allo-HSCT (58.0%, 19.0%, respectively, P=0.004). In patients who obtained CR after first course and received MD-Ara-C consolidation without allo-HSCT, the 5-year OS of patients with hyperleukocytosis was not significantly lower than that of patients without hyperleukocytosis (55.5%, 58.8%, respectively,P=0.419). FLT3-ITD mutation patients showed similar 5-year OS to that of wild type FLT3 patients (51.4%, 60.2%, respectively, P=0.482). And furthermore, 5-year OS of favorable, intermediate and unfavorable integrated genetics groups were 59.1%, 62.5%, 51.9%, respectively (P=0.332) in this subgroup.

CONCLUSIONHAD/HAI induction chemotherapy with sequential consolidation of MD-Ara-C could obtain satisfactory CR rate and long-term survival rate in de novo AML, especially for patients with hyperleukocytosis or FLT3-ITD mutation. It yet remains to be verified by large sample, prospective studies.

Cytarabine ; therapeutic use ; Daunorubicin ; therapeutic use ; Harringtonines ; therapeutic use ; Humans ; Idarubicin ; therapeutic use ; Induction Chemotherapy ; Leukemia, Myeloid, Acute ; drug therapy ; Leukocyte Count ; Prognosis ; Prospective Studies ; Remission Induction ; Retrospective Studies ; Survival Rate

OBJECTIVETo investigate the value of myeloproliferative neoplasms Symptom Assessment Form total symptom score (MPN-SAF-TSS)in assessing constitutional symptoms among Ph/BCR- ABL negative myeloproliferative neoplasm (MPN)patients.

METHODSA cohort of 628 MPN patients were evaluated by MPN- SAF- TSS.

RESULTSFatigue was the most common symptom (76.0%, 76.2%vs 89.9%)and the highest average severity of all the symptoms (3.46±2.97, 3.47±2.99vs 4.74±3.04 scores)among polycythemia vera (PV), essential thrombocythemia (ET)and primary myelofibrosis (PMF)patients. Using the MPN- SAF- TSS analysis, PMF patients showed highest burden of symptoms (28.9 ± 19.1), followed by PV patients (19.2 ± 16.8), and finally ET patients (17.1 ± 15.3). Instinct differences were observed between PMF and PV patients (χ(2)=6.371,P=0.021), PMF and ET patients (χ(2)= 14.020,P<0.001). No significant difference was found between PV and ET patients (χ(2)=2.281,P=0.191).

CONCLUSIONMPN- SAF- TSS was effective in evaluating the symptomatic burden among Ph/BCRABL negative MPN patients and could be used for serial assessment in this clinical setting.

Humans ; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative ; diagnosis ; physiopathology ; Polycythemia Vera ; complications ; Primary Myelofibrosis ; complications ; Thrombocythemia, Essential ; complications

Objective: To study the characteristics of gene mutations in Chinese myelodysplastic syndromes (MDS) patients. Methods: A total of 511 Chinese patients with MDS performed 112-gene targeted sequencing were retrospectively analyzed. Results: Eighty-three distinct mutant genes were found in 511 patients with MDS. Amongst these, the most frequent mutations was associated with epigenetics (50%) , followed by spliceosome (37%) , signal transduction (34%) , transcription factors (24%) and cell cycle/apoptosis (17%) . 439 subjects (86%) had at least one gene mutation. The mean number of mutations in refractory anemia with unilineage dysplasia (RCUD) was 1.25, refractory anemia with multilineage dysplasia (RCMD) was 1.73, refractory anemia with ring sideroblasts (RARS) was 2.79, refractory anemia with excess blasts-1 (RAEB-1) was 2.22, RAEB-2 was 2.34, MDS with isolated 5q- was 2.67, MDS, unclassified (MDS-U) was 2.00. U2AF1 mutant subjects were more likely to have isolated+8[Q<0.001, OR=4.42 (95% CI 2.23-8.68) ]and less likely to have complex karyotypes[Q=0.005, OR=0.22 (95% CI 0.04-0.72) ]. According to the number of gene mutations, all subjects were categorized into three groups, namely group with 0-1 mutation, with 2 mutations and with three or more mutations. There was a significant difference in overall survival (OS) among three groups (P=0.041) . Conclusion About 90% patients with MDS have at least one gene mutation. Genes associated with epigenetics and spliceosome are most common mutated genes in MDS. The increased numbers of gene mutations accompany with disease evolution and associate with poor prognosis.

OBJECTIVE To compare the phar macokinetics o f ligustrazine hydrochlori de，salvianic acid and rosemarinic acid from Shenxiong glucose injection （SGI）in normal and acute myocardial ischemia （AMI）rats. METHODS Male SD rats were randomly divided into normal group and model group ，with 9 rats in each group. AMI model was established by isoproterenol hydrochloride modeling method. Three rats in each group were selected for model verification. The remaining 6 rats in each group were given SGI （1.2 mL/kg）or equal volum of normal saline via tail vein ；0.3 mL blood was collected through orbital venous bush 0.083，0.167，0.333，0.5，0.75，1，1.5，2，3，5 h after administration. Using luteoloside as internal standard ，the plasma concentrations of ligustrazine hydrochloride ，salvianic acid and rosemarinic acid were determined by ultra performance liquid chromatography-tandem mass spectrometry. Pharmacokinetic parameters were fitted by WinNonlin 8.1 software，and statistical analysis was performed by SPSS 18.0 software. RESULTS The linear ranges of ligustrazine hydrochloride ，salvianic acid and rosmarinic acid were 0.06-29.96，0.01-5.15 and 0.006-3.09 μ g/mL（all r＞0.99），respectively. The results of methodological investigation were all in line with the requirements of Chinese Pharmacopoeia （2020 edition）. Compared with normal rats ，CLz of ligustrazine hydrochloride in AMI model rats was significantly increased （P＜0.05）；t1/2 and Vz of salvianic acid were significantly prolonged or increased （P＜0.05）；but the cmax and AUC 0－5 h were significantly decreased （P＜0.05）；AUC0－5 h of rosmarinic acid was significantly decreased （P＜0.05）. CONCLUSIONS The exposure levels of salvianic acid and rosmarinic acid in SGI are lower in AMI model rats than in normal rats ，and the elimination of ligustrazine hydrochloride in AMI model rats is stronger than that in normal rats.

OBJECTIVETo evaluate the prognostic value of cytogenetics in Chinese with primary myelofibrosis (PMF).

METHODSFour hundred and thirty-nine Chinese patients with PMF were retrospectively analyzed. The Kaplan-Meier method, the Log-rank test, the likelihood ratio test and the COX proportional hazards regression model were used to evaluate the prognostic scoring systems.

RESULTSFour hundred and thirty-nine Chinese patients with PMF were analyzed with a median age of 56 years (range: 8-83), including 298 males and 141 females. The DIPSS-plus system could effectively evaluate prognosis in Chinese patients with PMF. There was significantly higher predictive power for survival for the DIPSS-plus group compared with the DIPSS group (P=0.006, -2 log-likelihood ratios of 989.5 and 1001.9 for the DIPSS-plus and DIPSS systems, respectively). Univariate analysis indicated that the patients with a normal karyotype, a complex karyotype that was not a monosomal karyotype, +8 only or a balanced translocation only had better survival. Following two cytogenetic risk categories were constructed: favorable karyotype including subjects with a normal karyotype, a complex karyotype that was not a monosomal karyotype, +8 only or a balanced translocation only and unfavorable karyotype included all others. The modified DIPSS-Chinese prognostic model was proposed by adopting cytogenetic categories and DIPSS- Chinese risk group. The median survival of patients classified in low risk (163 subjects), intermediate-1 risk (187 subjects), intermediate-2 risk (82 subjects) and high risk (7 subjects) were not reached, 74 (95% CI 42-106), 39 (95% CI 26-52) and 12(95% CI 1-25)months, respectively, and there was a statistically significant difference in overall survival among the four risk groups (P<0.01).

CONCLUSIONThe DIPSS-plus had significantly higher predictive power than the DIPSS group in Chinese patients with PMF and the modified DIPSS-Chinese system based on the cytogenetic features of Chinese patients was proposed and worked well for prognostic indication.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Female ; Humans ; Karyotyping ; Male ; Middle Aged ; Primary Myelofibrosis ; diagnosis ; Prognosis ; Young Adult

OBJECTIVETo investigate the long- term outcome of cyclosporin A (CsA) combined with thalidomide regime for Chinese patients with IPSS low/intermediate- 1 myelodysplastic syndromes (MDS) without del（5q）and the predictive variables which could impact the response to the therapy.

METHODSSeventy-six MDS patients who were treated with these drugs at a single institute in China were retrospectively analyzed. The polymorphism of cereblon gene, rs1672753, was detected in patients of this cohort by PCR and direct sequencing.

RESULTSA total of 53% of patients showed hematological improvement（HI）to the therapy. Thirty-one patients（31/73, 43%）achieved erythrocyte response（HI-E）; 15 patients（15/50, 30%）achieved neutrophil response（HI-N); 18 patients（18/58, 31%）achieved platelet response（HI-P). Twenty-seven of the 50 patients（46%）who were dependent on red blood cell transfusion achieved HI- E and became independent of transfusion. The median duration of response among the responders was 22 months (range, 1- 131 + months). Bone marrow blasts ≤2% was the only factor associated with longer response duration in univariate analysis （P=0.010）. There was no significant difference between the two groups of celeblon gene rs1672753 polymorphism either on the response rate or the response duration. The median survival of 67 patients without stem cell transplantation was 82 months. In multivariate analyses, factors significantly correlated with survival were IPSS-R（HR=3.461, 95%CI 1.126-10.639, P=0.030), age ≥ 60 y（HR=4.120, 95%CI 1.070-15.867, P=0.040）and HI-N（HR=7.733, 95%CI 1.007-59.396, P=0.049).

CONCLUSIONCsA combined with thalidomide regime could improve the anemia symptom in low/int-1 risk MDS patients without del（5q）. The predictive value of cereblon gene polymorphism, rs1672753, could not be verified in this study.

Anemia ; Blood Platelets ; Blood Transfusion ; Bone Marrow ; China ; Cyclosporine ; therapeutic use ; Erythrocytes ; Humans ; Myelodysplastic Syndromes ; classification ; drug therapy ; Neutrophils ; Peptide Hydrolases ; metabolism ; Remission Induction ; Retrospective Studies ; Thalidomide ; therapeutic use ; Treatment Outcome