Objective To explore the differences of clinical features and relationship between aplastic anemia paroxysmal nocturnal hemoglobinuria syndrome(AA PNH syndrome)and typical paroxysmal nocturnal hemoglobinuria(t PNH).Methods A case control study on the discrepancies of clinical and laboratory features between patients with AA PNH syndrome and t PNH was carried out.Results Compared with t PNH,AA PNH syndrome showed following features:①Lower frequencies of venous thrombosis,jaundice and enlarged liver or spleen.②Higher percentages of pancytopenia and bone marrow hypoplasia.③Lower percentages of positive hemolysis tests.The percentages of CD55 and CD59 of peripheral blood cells were not significantly different in most cases of both groups.④Immunoglobulins and subgroups of T lymphocytes were normal in cases of both groups.⑤Adrenocortical hormone was effective in cases of both groups.Conclusion AA PNH syndrome shares a same pathophysiology with t PNH;CD55 and CD59 tests can improve the diagnosis of AA PNH syndrome.

Objective To review the implications for diagnosis,pathogenesis and potential for new therapeutic option for chronic neutrophilic leukemia (CNL).Data sources Data cited in this review were obtained mainly from PubMed and Medline from 1993 to 2013 and highly regarded older publications were also included.The terms "chronic neutrophilic leukemia" and "diagnosis" were used for the literature search.Study selection We identified,retrieved and reviewed the information on the clinical and laboratory features,the new genetic findings,prognosis and disease evolution and management of CNL.Results The discovery of high-frequency granulocyte-colony stimulating factor receptor (CSF3R) mutations in CNL identifies a new major diagnostic criterion,and lends more specificity to the World Health Organization (WHO) diagnostic criteria for CNL,which are variably applied in routine clinical practice.Conclusions In patients for whom the cause of neutrophilia is not easily discerned,the incorporation of CSF3R mutation testing can be a useful point-of-care diagnostic to evaluate the presence of a clonal myeloid disorder,as well as providing the potential for genetically informed therapy.The oncogenic CSF3R mutations are molecular markers of sensitivity to inhibitors of the SRC family-TNK2 and JAK kinases and may provide a new avenue for therapy.

Objective To explore the clinical characteristics and causes of death in patients with acute heart failure at aged 75 and over.Methods The prospective study collected 175 patients with acute heart failure from January 2012 to December 2014.They were divided into ≥75 years old group and＜75 years old group and the general clinical data were recorded.Follow-up was performed mainly by telephone with supplemented hospitalization follow-up and outpatient follow-up.Survival rates were assessed by Kaplan-Meier method.The survival rate difference between the two groups was compared using the log-rank test.Multivariate Cox proportional hazards regression analysis was used to determine the independent risk factors for death.Results The proportions of ischemic heart disease,hypertension and old myocardial infarction were higher in the elderly group than in the young group with a higher proportion of male,diabetes and body mass index in ＜75 years old group.Elderly group had a higher level of left ventricular ejection fraction(LVEF)and a lower level of total cholesterol,triglycerides and low density lipoprotein cholesterol(LDL-C).Kaplan-Meier curves showed that allcause mortality(x2 =4.005,P =0.045) and non-cardiovascular mortality(x2 =4.418,P =0.041) were significantly higher in the elderly group than in the younger group,whereas cardiovascular mortality had no significant difference between the two groups (x2 =0.754,P =0.385).In patients with noncardiovascular mortality,12 cases (63.2％)died of pulmonary infection in elderly group,3 cases(25.0％) died of lung infection in younger group,and the difference was statistically significant between the 2 groups (x2 =4.288,P =0.038).Multivariate Cox proportional hazards regression analysis showed that age≥75 years was an independent predictor for both non-cardiovascular mortality [HR(95％CI):2.71(1.50-6.55),Wald x2 =2.266,P=0.038]and all-cause mortality[HR(95 ％CI):1.75(1.28-3.13),Wald x2 =2.914,P=0.026]in patients with acute heart failure.Conclusions Age ≥75 years is an independent risk factor for all dead patients with acute heart failure and noncardiovascular death,but it is not the independent risk factors for cardiovascular death,which is of great significance to establish a more rational treatment strategy for senile heart failure.

Objective To investigate the changes of serum leptin and bone speed of sound (SOS) with gestational age (GA) and relationship between leptin and bone SOS in appropriate-for-gestational-age (AGA) neonates. Methods A total of 65 AGA neonates were recruited and divided into three groups according to their gestational age:preterm infant (GA 31-34 w, 14 cases), late preterm infant (GA 34-37 w, 13 cases), and full-term infant (GA≥37 w, 38 cases). Anthropometric parameters, including birth weight, length, leg length, skin fold thickness were measured in all the subjects, and the neonatal nutritional status and body fat content were evaluated by Ponderal Index (PI) and Weststrate equation (F%) respectively. Serum leptin concentration and tibial SOS were measured within 7 days after birth. Results There were signiifcant differences in GA (F=140.199, P<0.001), birth weight (F=47.042, P<0.001), birth length (F=46.877, P<0.001), leg length (F=17.543, P<0.001), PI (F=11.898, P<0.001) and F%( F=21.955, P<0.001) among three groups. Serum leptin and tibial SOS were signiifcantly different among these groups ( F=49.724, 20.052 respectively, P<0.001), and both of them were positively correlated with gestational age and birth weight (P<0.01). In addition, leptin was positively correlated with tibial SOS, but the correlation disappeared after adjustment for GA and anthropometry. According to the multivariate forward stepwise regression analysis, tibial SOS was found to be signiifcantly positively associated with gestational age and birth weight in the three groups. Conclusions Both bone SOS and serum leptin are signiifcantly correlated with gestational age and birth weight in AGA neonates, and leptin is related with but not the independent direct predictor of bone SOS.

Objective To observe the therapeutic effect of electroacupuncture (EA) on acupoints located along anterior and posterior thigh muscles plus isokinetic muscle strength training for poststroke lower limb dysfunction. Methods Fifty-two qualified mild hemiplegia cases were randomized into control group A ( N=17) , control group B (N=17), and observation group (N=18 ). Control group A was given conventional rehabilitation training, control group B was given conventional rehabilitation training and task-targeting knee strength-coordination isokinetic muscle strength training based on visual feedback, and observation group was given EA on acupoints located along anterior and posterior thigh muscles plus the treatment for control group B. The treatment for the three groups was performed once a day, five days a week, lasting 3 weeks. Before and after treatment, the lower limb motor function, peak torque ( PT) of knee flexion and extension muscles, and gait speed and gait symmetry were monitored. Results After treatment for 3 weeks, the lower limb motor function, PT and gait symmetry were much improved in the three groups (P<0.05 compared with those before treatment) . Except that the gait speed and lower limb muscle tension of observation group did not differ from those of control group B ( P>0.05) , the improvement of observation indexes of control group B was superior to that of control group A, and the improvement of observation indexes of observation group was more obvious than that of the two control groups ( P<0.05) . Conclusion EA on acupoints located along anterior and posterior thigh muscles plus isokinetic muscle strength training is more effective for improving lower limb motor function of poststroke lower limb dysfunction patients than conventional isokinetic training.

Objective To determine the changes of heart structure and function in early stage after lung transplant and to study the relationship between decreased pulmonary artery pressure and changes of heart structure and function.Methods 45 cases of lung transplant recipients were included in the study,their preoperative and postoperative echocardiography data were reviewed,and the postoperative early changes of cardiac structure and function were analysed.Then,the Pearson correlation analysis was used to compare the relationships between the decrease of pulmonary artery pressure and the changes of cardiac structure and function.Results After lung transplant,pulmonary artery systolic pressure(PASP) decreased significantly [(62.3 ± 27.2) vs (36.20 ± 7.8)mm Hg,P ＜0.01],and right ventricular dimensions zoomed down,tricuspid valve and pulmonary valve regurgitation reduced,left atrial diameter (LAD) and left ventricular end-diastolic diameter (LVDD) were enlarged,stroke volume (SV) increased [(43.85 ± 14.78) vs (58.68 ± 13.85)],but left ventricular ejection fraction (LVEF) was decreased [(69.31 ± 7.50)％ vs (62.82 ± 8.12) ％],those differences above were statistically significant (P ＜ 0.05) compared with preoperative echocardiography date.Pearson linear correlation analysis show that after lung transplantation the more decreased PASP,the more increased LAD and LVDD,and the more decreased LVEF (P ＜0.05).Conclusions In early stage after lung transplant,the structure of right ventricular was quickly normalized,the function of right ventricular improved,LAD enlarged,SV increased,but left ventricular function reduced,there were a linear correlation between those changes and PASP reduced.Echocardiography has good reference value for early lung transplant patient.

ObjectiveTo observe the clinical effect of acupoint application on lipid metabolism in elderly diabetics with hyperlipidemia.Methods60 elder diabetics were divided into two groups.In the drugs group,patients were purely treated with simvastatin or lipanthyl.In the acupoint group, patients received acupoint application.The serum total cholesterol(TC), triglyceride (TG), low-density lipoprotein (LDL-C), high-density lipeprotein (HDL-C), aPoprotein A(ApoA) and apoprotein B(ApoB) ere measured respectively.Adverse events were observed.ResultsThe levels of TC、TG、LDL-C、ApoB were significantly dechned(all P ＜0.01 ～0.05).However,the rates of reduce lipid had no significant difference in two groups(P ＞0.05).The levels of HDL-C and ApoA were increased in the drugs group,but the homologous levels in the acupoint group showed no significantly change(all p ＞ 0.05).The adverse reaction in the acupoint group was significantly lower than that in the drugs group(P ＜0.05).ConclusionAcupoint application could decrease the blood lipid spectrum in the senile diabetics with hyperlipidemia.This therapeutics could reduce adverse drug reactions.

ObjectiveTo determine the risk factors and the optimal management of hepatic artery complications (HAC) after orthotopic liver transplantation.MethodsThe clinical data of 180 orthotopic liver transplantation patients performed between January 2005 and September 2007 was reviewed.The incidence of HAC between primary liver carcinoma and benign diseases of liver was compared.ResultsTwelve (6.7％) episodes of HAC were identified.3 were hepatic artery thrombosis (HAT) and 9 were hepatic artery stenosis (HAS).The incidence of HAC in patients with primary liver carcinoma (6/39) was higher than benign disease (6/141)(P＜0.05).ConclusionsThe keys to management of HAC after orthotopic liver transplantation are to diagnose the complication in time and to select the proper treatment based on the type of HAC.

Objective To evaluate the clinical value of noninvasive positive-pressure ventilation (NPPV) treatment in acute respiratory distress syndrome caused by acute pancreatitis. Methods A retrospective study of 27 cases, with acute respiratory distress syndrome (ARDS) caused by acute paucreatitis,who were admitted to our department from Jan 2007 to May 2010 and treated with NPPV, was performed. The changes of heart rate, respiratory rate, PaO2, oxygenation index (OI) and PaCO2 before and after treatment were compared. Results The heart rate, respiratory rate of 25(92.6％ ) patients decreased from (118.4 ±13.4)/min, (32.1 ± 1.7)/min to ( 81.9 + 8.5 )/min, ( 19.9 ± 2.1 )/min; PaO2, OI and PaCO2 increased from (74.1 ±5.0)mmHg, (148.2 +10.0)mmHg, (28.7 ±1.6)mmHg to (110.4 ±20.8)mmHg, (204.5±71.1)mmHg, (38.4 +3.6)mmHg 48 h after NPPV, respectively, and they recovered and were transited to oxygen supply by Venturi mask. 2 (7.4％) patients deteriorated and were transited to invasive positive-pressure ventilation support. Conclusions NPPV could effectively improve oxygenation of patients with ARDS caused by acute pancreatitis. The procedure of NPPV is relatively easy to use and to learn with few complications, and worth of clinical application.