To analyze the disease burden and trends of motor neuron disease(MND) in China and globally from 1990 to 2021, providing evidence for the formulation of relevant health strategies inChina.

Data on the incidence, prevalence, and disability-adjusted life years(DALYs) of MND in China and globally from 1990 to 2021 were extracted from the 2021 global burden of disease(GBD) database. The Joinpoint model was used to analyze trends through the average annual percentage change(AAPC). The disease burden differences were further analyzed by age and gender.

From 1990 to 2021, the number of MND incident cases in China increased by 6.87%, while globally it increased by 74.54%.The number of prevalent cases in China rose by 29.78%, compared to a 68.43% increase globally. DALYs due to MND in China increased by 40.08%, while globally they increased by 105.59%. The age-standardized incidence rate(ASIR) of MND showed a declining trend both in China(AAPC=-0.006, 95% CI: -0.006 to -0.006, P < 0.001) and globally(AAPC=-0.001, 95% CI: -0.001 to -0.001, P < 0.001). The age-standardized prevalence rate(ASPR) in China showed an increasing trend(AAPC=0.006, 95% CI: 0.006 to 0.007, P < 0.001), while the global trend was not significant(AAPC=0, 95% CI: -0.001 to 0, P= 0.082). The age-standardized DALY rate(ASDR) in China showed a declining trend(AAPC=-0.022, 95% CI: -0.033 to -0.011, P < 0.001), whereas globally it showed an increasing trend(AAPC=0.033, 95% CI: 0.028 to 0.039, P < 0.001). Additionally, the MND disease burden was higher in males than in females across most age groups in China, with the burden being particularly heavy in the 45-74 age group.

The overall disease burden of MND in China is lower than the global level, with ASIR and ASDR showing a declining trend. However, the annual number of incident cases, prevalent cases, and DALYs continues to increase. Furthermore, there are gender and age differences in the MND disease burden in China, with middle-aged and elderly males being the key targets for prevention and control.

Objective:To investigate the prevalence and risk factors of diabetic peripheral artery disease (PAD) in patients with type 2 diabetes mellitus (T2DM) managed in primary health care in China.Methods:A total of 2 528 T2DM patients were selected using a two-stage cluster random sampling method based on the baseline survey of the "China Diabetic Foot Prevention Model Project." The study was conducted in 2015 among T2DM patients in 8 primary healthcare centers in Changshu county and Jiang'an district of Wuhan, China. Data collection methods included a questionnaire, body measurement, and blood glucose detection. The Ankle-Brachial Index (ABI) is the most widely used noninvasive vascular test. A binary logistic regression model was used to analyze the influence factors.Results:The prevalence of PAD was 11.2% among the diabetic patients managed in primary health care in the two cities. The prevalence of PAD under 55 years old, 55- years old, 65- years old, and ≥75 years old were 7.8%, 6.0%, 12.9% and 22.5%, respectively. Multivariate stepwise logistic regression identified influence factors included older age, higher education level, smoking, drinking, postprandial glucose uncontrol, and prior myocardial infarction or angina. Compared to age <55 years, the odds ratio for PAD were 0.74 for 55- years (95% CI: 0.43-1.28), 1.72 for 65- years (95% CI: 1.05-2.81), 3.56 for 75 years and above (95% CI: 2.07-6.11), respectively. Compared to patients with education in primary school and below, the odds ratio was 1.37 (95% CI: 0.97-1.94), 2.48 (95% CI: 1.73-3.55), 1.99 (95% CI: 1.26-3.13) for those with education levels of junior high school, senior high school, and college, respectively. Current smoking ( OR=1.49, 95% CI: 1.02-2.17), current drinking ( OR=0.45, 95% CI: 0.28-0.71), postprandial glucose uncontrol (2 h postprandial plasma glucose >10.0 mmol/L: OR=1.72, 95% CI: 1.22-2.43), and prior myocardial infarction or angina ( OR=2.32, 95% CI: 1.50-3.61) were influencing factors of PAD. Conclusions:Despite the high prevalence of PAD in diabetes managed in primary health care; multiple risk factors are not effectively aware of and under control. It is urgent to promote ABI screening and standardized management for diabetes, especially in primary health care.

Objective: To investigate the prevalence and risk factors of diabetic peripheral neuropathy in type 2 diabetic patients under community management programs. Methods: A cross-sectional study was conducted on T2DM patients in eight communities in Wuhan and Changshu cities. Data would included questionnaire, body measurement, blood testing and clinical examination. The criterion of diabetic peripheral neuropathy was under the combination of symptoms with five physical examinations. Binary logistic regression model was used to analyze the influential factors. Results: The overall prevalence of peripheral neuropathy was 71.2% among the diabetic patients who were managed in primary care health services in the two cities. The binary logistic regression method identified older age (≥60 years, OR=2.39, 95%CI:1.95-2.94), longer diabetic duration (≥10 years, OR=1.25, 95%CI: 1.02-1.54), and worse postprandial glucose control (2 h postprandial plasma glucose >10.0 mmol/L: OR=1.65, 95%CI:1.33-2.04) (all P<0.05) as risk factors for the presence of diabetic peripheral neuropathy, while higher education level was protective factor (compared to patients with education levels of primary school or below, OR=0.52, 95%CI: 0.41-0.66; OR=0.59, 95%CI: 0.44-0.79; OR=0.64, 95%CI: 0.44-0.94 for those with education levels of junior high school, senior high school, and college, respectively). Conclusions High rates of diabetic peripheral neuropathy among T2DM patients suggested the urgent need for early screening and standardized management at the community levels. It is necessary to promote appropriate screening techniques and methods to identify the peripheral neuropathy, in the primary health service institutions.

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.

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.

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 explore the relationship between gastric juice pH and hospital-acquired pneumonia ( HAP) , the gastric bacterial colonization and etiology of HAP in neurologic intensive care unit patients by monitoring gastric juice pH value.Methods From October 2014 to May 2015, consecutive seventy-two tube feeding patients admitted in the Department of Neurology Intensive Care Unit in Xijing Hospital were enrolled in this research.The type and concentration of pathogens from gastric contents were collected, while samples from upper respiratory tract and pharynx were detected dynamically at the same time.Results (1)The group with new onset HAP was higher in gastric juice pH (6.4(5.4,6.4) vs 5.4 (2.5, 6.4), Z=-2.37, P=0.01); (2) The isolation rate of colonized bacteria in gastric cavity was associated with the pH of gastric juice , achieving 60.8% ( 42/69 ) in HAP group; ( 3 ) When the gastric juice pH was >4, the isolation rate of Gram-negative bacilli in gastric cavity obviously increased (63.6%(28/44) vs 35.7%(10/28),χ2 =5.323, P=0.021); (4)The same pathogens were found in stomach-pharynx-upper respiratory tract in 7 cases ( 17.5%) of the total 40 HAP patients.Conclusion Increased gastric juice pH was associated with gastric colonization , especially Gram-negative bacilli , and may lead to a higher incidence of new onset HAP in patients on enteral feeding.

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