Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.

Objective:To establish methods for HPLC fingerprints and simultaneous determination of multi-index components before and after compatibility of Salvia miltiorrhiza and Angelica sinensis, so as to analyze the dissolution rate of the main compounds. Methods:The extracts of Salvia miltiorrhiza, Angelica sinensis and their compatibility were prepared. The separation was performed on an Eclipse XDB-C18 column (4.6 mm×250 mm,5 μm), mobile phase with 0.1% phosphoric acid aqueous solution-acetonitrile for gradient elution, flow rate at 1.0 ml/min, column temperature was maintained at 35 ℃, and the detection wavelength was set at 280 nm. The HPLC fingerprint were established before and after the compatibility of Salvia miltiorrhiza and Angelica sinensis, and the shared patterns of the fingerprint were obtained to gain chromatographic peaks. The content of 9 components Danshensu, caffeic acid, rosmarinic acid, salvianolic acid B, salvianolic acid A, tanshinone Ⅱ A, ferulic acid, chlorogenic acid and Yangchuanxiong lactone were determinated, and the changes of dissolution rate of each compound before and after the compatibility were analyzed. Results:The determination method for the multi- components with HPLC is precise and the components (waiting to be determinate) in the solution were stable within 48 hours, and the RSD values of each chromatographic peak were <5.0%. The nine components showed good linear relationships within their own ranges, and the recovery rate was in compliance with regulations. The fingerprint similarities of each sample were ?0.9. After the compatibility of Salvia miltiorrhiza and Angelica sinensis, a total of seventeen common peaks were calibrated, ten of which were from Salvia miltiorrhiza, seven from Angelica sinensis. No new components was found under this chromatographic condition. After the combination of these two material medicica decoction, the average dissolution rates of rosmarinic acid, salvianolic acids and Danshensu in Salvia miltiorrhiza were significantly lower than those of the single decoction group ( P<0.05 or P<0.01); the average dissolution rates of caffeic acid in Salvia miltiorrhiza was significantly higher than that of the single decoction group ( P<0.01); the average dissolution rates of chlorogenic acid and ferulic acid in Angelica sinensis were significantly higher than that of the single decoction group ( P<0.05 or P<0.01); the average dissolution rate of Yangchuanxiong lactone after the compatibility was not statistically different than that of single decoction group. Conclusion:The characteristic peaks of HPLC fingerprint of the compatibility of Salvia miltiorrhiza and Angelica sinensis did not increase under this chromatographic condition, which had a significant effect on the dissolution of index components.

Objective:To investigate the efficacy of peritoneal perfusion combined with intravenous and oral chemotherapy in the treatment of gastric cancer with peritoneal metastasis and its correlation with prognosis.Methods:The clinical data of 142 patients with peritoneal metastasis of gastric cancer admitted to the Affiliated Hospital of Xuzhou Medical University from March 2012 to June 2019 were retrospectively analyzed.According to different treatment schemes, the patients were divided into observation group (47 cases treated with tegafur capsule orally+ docetaxel+ cisplatin combined with recombinant human tumor necrosis factor intraperitoneal perfusion) and control group (oral administration of tegafur capsule+ oxaliplatin). The clinical efficacy and prognostic factors were compared between the two groups.Results:The short-term efficacy after two courses of chemotherapy: the effective rate was 44.7% (21/47) in the observation group and 8.4% (8/95) in the control group, the difference was statistically significant (χ 2=25.438, P<0.001); the disease control rate was 97.9% (46/47) in the observation group and 60.0% (57/95) in the control group, the difference was statistically significant (χ 2=22.639, P<0.001). Long term efficacy: the progression free survival time of the observation group was higher than that of the control group (6.50 months vs.3.83 months, χ 2=16.399, P<0.001); the total survival time of the observation group was higher than that of the control group (15.60 months vs.10.83 months, χ 2=9.219, P=0.002). Univariate analysis showed that ascites volume, chemotherapy, palliative surgery, liver metastasis, Lauren classification and Her-2 expression were related to prognosis (all P<0.05); there was no significant difference in cumulative survival rate of different gender, age and ECOG score (all P>0.05). Multivariate analysis showed that liver metastasis ( HR=1.996, 95% CI=1.194-3.338, P=0.008 ), Lauren type ( HR=1.979, 95% CI=1.265-3.094, P=0.003 ), Her-2 expression ( HR=3.872, 95% CI=2.202-6.808, P<0.001 ), chemotherapy regimens(whether combined with intraperitoneal chemotherapy) ( HR=1.826, 95% CI=1.046-3.188, P=0.034 ) and abdomina volume ( HR=2.785, 95% CI=1.644-4.718, P<0.001 ) were independent risk factor for the prognosis of patients. Conclusion:Intraperitoneal perfusion combined with intravenous and oral administration has a good clinical effect in the treatment of peritoneal metastasis of gastric cancer, which can effectively improve the clinical symptoms and improve the survival rate of patients.The presence or absence of liver metastasis, Lauren classification, Her-2 expression, chemotherapy method (combined with intraperitoneal perfusion or not) and ascites volume are independent risk factors for the prognosis of peritoneal metastasis of gastric cancer.

Objective To analyze the prognostic impact of Ikaros family zinc finger 1(IKZF1)mutation on adult Philadelphia chromosome (Ph1) positive acute lymphoblastic leukemia (ALL) patients.Methods IKZF1 mutation was detected in 63 adult Phi positive ALL patients at diagnosis using capillary electrophoresis.Recruited patients were treated in our center and other three hospitals in Ningbo from January 2014 to January 2017.Clinical data were collected and retrospectively analyzed.Results Thirty-nine (61.9％) patients were positive IKZF1 mutation in this cohort.The white blood cell (WBC) count in IKZF1 mutation group was significantly higher than that of mutation negative group [(64.6±11.3)× 109/L vs.(33.7±5.6)×109/L,P＜0.05].Patients with WBC count over 30×109/L accounted for 56.4％ in IKZF1 mutation group.Complete remission (CR) rate in the IKZF1 mutation group was also lower than that of negative group after induction chemotherapy (64.1％ vs.75.0％,P＞0.05).IKZF1 was a negative prognostic factor but not independent factor for survival by univariate and multivariate analyses.Patients were divided into chemotherapy and allogeneic transplantation groups.The 3-year overall survival (OS) rate and 3-year leukemia-free survival (LFS) rate in IKZF1 mutation group were significantly lower than those of negative group in both transplantation group (42.3％ vs.59.3％;31.2％ vs.50.0％;respectively,both P＜0.05) and chemotherapy group (24.8％ vs.40.0％;19.0％ vs.34.3％;respectively,both P＜0.05).Conclusion IKZF1 mutation is a poor prognostic factor for adult Ph1 positive ALL patients.

OBJECTIVE： To optimize the extraction technology of total vitamin E in Euryale ferox. METHODS： With the extraction amount of total vitamin E as reference index， using extraction time， extraction times， ultrasound power and comminution degree as reference factors， single fator test and Box-Behnken design-response surface methodology was used to optimize the extraction technology of total vitamin E from E. ferox. The validation tests were conducted for 3 times （the amounts of E. ferox were 2.0， 20.0， 40.0 g）. RESULTS： The optimal extraction technology of vitamin E included that extraction time of 80 min， extraction times of 3 times， ultrasound power of 240 W， comminution degree of 80 mesh. In validation test， extraction rates of total vitamin E were 2.063， 2.103， 2.085 mg/g （RSD＝2.6％， 1.5％， 1.3％， n＝3）， the relative errors of which to predicted value （2.092 mg/g） were 0.14％， 0.53％ and 0.33％， respectively. CONCLUSIONS： The optimal extraction technology is reasonable， stable and feasible， and can be used for the extraction of total vitamin E in E. ferox.

Objective To investigate the mid-term efficacy and complications of bone cement or biotype hemiarthroplasty for the femoral neck fracture in the elderly patients.Methods From January 2011 to January 2014,sixty-seven elderly patients (67 hips) with femoral neck fracture were treated with hemiarthroplasty.All patients were treated with bipolar femoral head prosthesis,and the prosthesis included into bone cement and biologic type,according to the fixation of the femoral stem.The 3 year postoperative Harris score and surgeryrelated complications (joint dislocation,prosthesis loosening,acetabular subsidence,and mortality) were evaluated.Results All patients were followed up for 3 years.The excellent and good rate of Harris score was 87.1％ (27/31) in the bone cement group and 80.6％ (25/31) in the cementless group (X2 =0.47,P＞0.05).There was no significant difference in the complications between the two groups (P＞0.05).Conclusion The hemiarthroplasty is an effective and safe methodfor the treatment of femoral neck fracture in the elderly.The key to achieve excellent results is to choose the femoral prosthesis based on the preoperative planning and operation.

Objective To evaluate the clinical value of MDCTA (Multi-detector computed tomographic angiography) in reconstruction of the soft tissue defect of the limbs combined with the main vascular defects by using Flow-through anterolateral thigh flap.Methods From September,2013 to May,2016,12 cases of limb soft tissue with main vascular defects were examined by MDCTA.The length of vascular defect was measured.The length of bridging vessel needed to design a Flow-through anterolateral thigh flap was measured.The points of anterolateral thigh flap perforator were positioned.The relationship between the perforation and the trunk vascular pedicle was defined.The outcomes were evaluated based on Berton scores for upper extremities and John-Wruh scores for lower extremities.Results The length of the main vascular defects,the positions of flap perforators and the configuration of vascular pedicles were successfully imaged through MDCTA.The length of the main vascular defects were 5-12 cm,which was consistent with the intraoperative findings.The length of bridging vessel for the defects were 7-14 cm,which was enough for the vascular defects.There were not vessel grafts.Twenty perforators were imaged by MDCTA preoperatively,and 22 perforators were found during the operations,which means 2 perforators were not imaged.All flaps survived completely.All patients were followed-up from 6 to 21 months (average,11 months).According to the Berton scores for upper extremities and John-Wruh scores for lower extremities,the excellent/good were 6 in 8 cases and 4 in 4 cases,respectively.Conclusion MDCTA can be used to evaluate the pedicle conditions of the free Flow-through anterolateral thigh flap for the reconstructions.It is worthy to be popularized in clinical application.

Objective To analyze the effects of quality supervision and continuous improvement system on optimizing in-hospital diagnosis and treatment process in patients with acute ischemic stroke (AIS).Methods From September 2013 to May 2016,424 consecutive patients with AIS treated with intravenous thrombolysis and/or endovascular therapy in Changhai Hospital,the Second Military Medical University were enrolled retrospectively.They were analyzed according to the annual running process (the first year[from September 2013 to August 2014],the second year[from September 2014 to August 2015],and the third year[from September 2015 to May 2016]).The spend time and delay (DTN>60 min,DTP>90 min) rate of each treatment process in the first,second,and third year (time from door-to-imaging[DTI],door-to-needle[DTN],imaging-to-needle (ITN),door-to-groin puncture (DTP) and imaging-to-groin puncture (ITP) were compared.Taking the time periods (>median) of having significant differences of the spend time of the treatment processes as the dependent variables in the first,second,and third year,the influence of the years and treatment modalities on delay was observed.The difference of constituent ratio of the reasons for delay in intravenous thrombolysis and endovascular therapy (objective reasons/other reasons) in different years were analyzed.Results (1) DTIs were 23.0 (11.0,42.0) min,22.0 (10.1,39.0) min,and 13.0 (6.0,27.0) min,respectively,and DTNs were 50.0 (30.0,77.1) min,45.0 (30.0,70.2) min,and 36.0 (24.0,57.0) min,respectively in the first,second,and third year.The spending time was shortened year by year.There were significant differences among the different years (all P<0.01).The spending time of DTP had a tendency to be shortened,but there were significant differences among different years (P=0.06).There were no significant differences between the spending time of ITN and ITP (all P>0.05).(2) The DTN delay rates were 33.3% (40/120),20.7% (29/140),and 8.1% (9/111),respectively in the first,second,and third year.There were significant differences among the 3 years (x2=22.111,P<0.01).There were no significant differences among the DTP delay rates (P=0.08).(3) Multivariate Logistic regression analysis showed that taking the first years as a reference,the risk of DTI delay was reduced in the third year (OR,0.174,95%CI 0.101-0.298,P<0.01),the risks of DTN delay were reduced in the second and third year (OR,0.564,95%CI 0.338-0.941;OR,0.180,95%CI 0.101-0.320,all P<0.05).For simple intravenous thrombolysis,bridging therapy was a protective factor for the improvement of treatment efficiency in the DTI process (OR,0.530,95%CI 0.297-0.943,P=0.031).Compared with the bridging therapy,the direct endovascular therapy was a protective factor for DTP treatment (OR,0.427,95%CI 0.202-0.901,P=0.025).The remaining independent variables were not associated with the occurrence of DTN and DTP delay (all P>0.05).(4) During the three years,the delay of intravenous thrombolysis was mainly due to objective reasons.The constituent ratio of other reasons caused delay of intravenous thrombolysis was decreased year by year.There was no other reasons for delay in the third year).There was no significant difference in the constituent ratio of the delay reasons in endovascular treatment (x2=3.622,P=0.164).Conclusion Under the existing process and resource allocation,setting the DTN target time and implementing continuous quality improvement are conducive to the effective implementation of brain CT scan and continuous optimization of intravenous thrombolysis in the processes in AIS patients with the first diagnosis.

To study the efficacy of CAG regimen used in 35 cases of newly diagnosed acute myeloid leukemia in elder-ly patients. Methods:Totally 35 cases of elderly patients with acute myeloid leukemia were randomly divided into two groups. The pa-tients in the control group (15 cases) were treated with standard chemotherapy treatment, while the CAG regimen group (20 cases) was treated with CAG regimen (cytarabine, aclarubicin and granulocyte colony-stimulating factor). After one treatment course, the chemotherapy efficacy was observed, and the neutrophil and platelet recovery time and the incidence of adverse reactions in the two groups were compared. Results:The treatment efficacy of the CAG regimen group was 90%, which was higher than that of the control group (60%, P<0. 05). The neutrophil and platelet recovery time of the CAG regimen group was significantly shorter than that of the control group (P<0. 05). The incidence of adverse reactions in CAG regimen group was much lower than that of the control group(P<0. 05). Conclusion:The efficacy of CAG regimen used in the 35 cases of newly diagnosed acute myeloid leukemia in elderly pa-tients is promising, which can effectively control the progression of the disease and accelerate blood cell recovery with high safety.

A 37-year-old male patient presented with persistent generalized itching erythema and papules for more than 1 month.The patient had received surgical treatment for type B3 thymoma in stage Ⅳ.Five months prior to the presentation,he developed myasthenia gravis.He also complained of chronic diarrhea for two years.Physical examination revealed white patches in the oral mucosa as well as scaly erythema of varying size on the face,trunk and extremities.Lamellar scales could be seen after scratching,while removal of scales could not result in the appearance of bleeding points.There was scaly hyperkeratotic erythema of palms and soles.Histopathological examination revealed psoriasis-like epidermal hyperplasia and parakeratosis with multiple dyskeratocytes,and some of the dyskeratocytes were surrounded by lymphocytes.There was a perivascular infiltration with a small number of lymphocytes.Immunohistochemical study showed positive staining for CD3,CD4 (dermis),CD8 (epidermis) and CD45RO,but negative staining for CD20,CD68 and CD30.The patient was diagnosed as thymoma-associated graftversus-host-like disease,myasthenia gravis and mucosal candidiasis.After treatment with tacrolimus and prednisone,the condition was gradually relieved.