Objective: To evaluate clinical characteristics and prognosis of primary myelofibrosis (PMF) patients with thrombocytopenia in varied degrees. Methods: Clinical features and survival data of 1 305 Chinese patients with PMF were retrospectively analyzed. The prognostic value of thrombocytopenia in patients with PMF was evaluated. Results: 320 subjects (47%) presented severe thrombocytopenia (PLT<50×10(9)/L), 198 ones (15.2%) mild thrombocytopenia [PLT (50-99)×10(9)/L] and 787 ones (60.3%) without thrombocytopenia (PLT ≥ 100×10(9)/L). The more severe the thrombocytopenia, the higher the proportions of HGB<100 g/L, WBC<4×10(9)/L, circulating blasts ≥ 3%, abnormal karyotype and unfavourable cytogenetics (P<0.001, P<0.001, P=0.004, P<0.001 and P<0.001, respectively) were observed in this cohort of patients. The more severe the thrombocytopenia, the lower the proportion of JAK2V617F positive (P<0.001) was also noticed. Platelet count was positively correlated with splenomegaly, HGB and WBC (P<0.001, correlation coefficients were 0.131, 0.445 and 0.156, respectively). Platelet count was negative correlated with constitutional symptoms and circulating blasts (P=0.009, P=0.045, respectively; correlation coefficients were -0.096 and -0.056, respectively). The median survival of patients with severe thrombocytopenia, mild thrombocytopenia and without thrombocytopenia were 32, 67 and 89 months, respectively (P<0.001). Multivariate analysis identified thrombocytopenia in varied degrees (HR=1.693, 95%CI 1.320-2.173, P<0.001) and Dynamic Internation Prognostic Scoring System(DIPSS) prognostic model (HR=2.051, 95%CI 1.511-2.784, P<0.001) as independent risk factors for survival. Conclusion: PMF patients with severe thrombocytopenia frequently displayed anemia, leucopenia, circulating blasts and short survival, so active treatment measures should be taken especially in these patients.
Humans ; Primary Myelofibrosis ; Prognosis ; Retrospective Studies ; Thrombocytopenia

Objective To investigate the effect of Clostridium butyricum tablets combined with conventional therapy on the cholinesterase levels and albumin ( ALB) levels in patients with liver cirrhosis.Methods The 236 patients with liver cirrhosis from October 2016 to February 2018 in the Department of Gastroenterology of our hospital were selected as the study subjects.The patients were divided into the treatment group and the control group.The patients in the treatment group were given 2 g Clostridium butyricum tablets orally ,on the basis of general symptomatic treatment,3 times per day for two weeks ;the patients in the control group were stan-dard treatment.The plasma cholinesterase , ALB levels were determined by automatic analyzer.Results The cholinesterase levels in the control group and the treatment group were ( 2.87 ±1.82 ), (7.94 ±2.45) U· L-1, respectively; the ALB levels in the 2 groups were (34.02 ±3.26),(47.63 ±4.05) g· L-1,respectively;comparison between the two groups , the difference was significant ( all P ＜0.05 ) . Conclusion Clostridium butyricum tablets can significantly increase plasma cholinesterase and ALB levels in patients with liver cirrhosis .

Objective To evaluate the clinical safety and efficacy of percutaneous interventional therapy in pediatric patients with secundum atrial septal defect(ASD).Methods Clinical data of 40 patients(age≤2 years)with secundum atrial septal defect treated in our hospital from February 2014 to December 2017 were analyzed retrospectively. There were 13 males and 27 females in these patients.Ultrasound of heart showed that there were 37 patients with single ASD,3 patients with multiple ASDs.One associated with pulmonary stenosis(PS),and 1 associated with patent ductus arteriosus.There were 6 patients with pulmonary hypertension, and the diameter of ASD was (10.6 ± 2.0) mm. All patients were proved to have secundum atrial septal defect before intervention.In the intervention,the transport system was delivered along the femoral vein,inferior vena cava and right atrium through atrial septal defect to the left atrium,and the occluder was released there. Results Of the 40 patients, 38 cases were successfully implanted, and the other two patients were not satisfied with the location of occlusion.The diameter of the ASD occluder was(12.0±2.1)mm and the transport sheath 7-9 F.Plug2 occluder was implanted in the patient with patent ductus arteriosus.To the patient with PS,pulmonary valve balloon angioplasty was performed,and then the pressure gradient reduced obviously, after that ASD occlusion was performed. The total follow up period was from 2 months to 3 years.No residual shunt and unsatisfactory device position were found during the follow up period.The pulmonary pressure reduced to normal,and the right atrium and right ventricle were smaller in a different degree. All patients had no arrhythmia and other complications.Conclusion Transcatheter closure of ASD is safe,reliable,and has fewer complications.It is worthy of popularization and application.Appropriate occluder should be selected according to the size and edge of ASD to reduce complications,such as residual shunt and valve injury.

This study was carried out to investigate the effect of oral administration of Dendrobium huoshanense on the expressions and activities of hepatic microsomal cytochrome P450s in mice, and to provide a reference for the evaluation of drug-drug interactions between D. huoshanense and clinical drugs. The C57BL/6 mice were randomly divided into blank control group, D. huoshanense low dose group (crude drug 1.25 g·kg⁻¹), D. huoshanense high dose group (crude drug 7.5 g·kg⁻¹), and phenobarbital positive control group (0.08 g·kg⁻¹). Each group was intragastrically administered with drugs for 2 weeks. The mice were sacrificed and their liver microsomes were prepared. The expressions of major subtypes of P450 enzyme were determined by Western blot and the probe drugs were used to detect the enzyme activities of P450 subtypes with protein expression changes. Western blot analysis showed that the protein expressions of CYP1A1, CYP1A2 and CYP2B in liver tissues were up-regulated in D. huoshanense-treated group. In vitro enzyme activity tests showed that there were no significant difference in metabolism of 7-ethoxyresorufin (a probe drug for CYP1A1) and bupropion (a probe drug for CYP2B) between D. huoshanense group and control group. The metabolism of phenacetin (a probe drug for CYP1A2) showed a statistical difference in rate Vmax, and it was significantly increased by approximately 20% in D. huoshanense group as compared with the blank control group, and the clearance CLint in treated group was also increased by about 32%. Therefore, oral administration of D. huoshanense had no effects on the activities of most hepatic P450 enzymes in mice, with no drug-drug interaction related to the P450 enzyme system in most clinical drugs theoretically. However, oral administration of D. huoshanense may accelerate the metabolism of CYP1A2-catalyzed drugs, which needs to be considered in clinical practice.
Animals ; Cytochrome P-450 CYP1A1 ; metabolism ; Cytochrome P-450 CYP1A2 ; metabolism ; Cytochrome P-450 Enzyme System ; metabolism ; Dendrobium ; chemistry ; Drugs, Chinese Herbal ; pharmacology ; Mice ; Mice, Inbred C57BL ; Microsomes, Liver ; drug effects ; enzymology ; Random Allocation