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.

Objectives:To study the pharmacokinetics and relative bioavailability of glipizide in healthy male volunteers. Methods:The glipizide concentrations in plasma were determined by HPLC UV. The column: Lichrospher C18 (5 ?m,150 mm?4.6 mm),the mobile phase: methanl∶0.01 mol/L sodium acetale buffer (pH 4.8) (59 ∶41); the flow rate:1 ml/min, the detection wavelength: 225 nm. The test and reference formulations of glipizide were given to 20 healthy male volunteers. Results: The calibration curve was linear within the range of (25～1 000)?g/L, r =0.999 4. The minimum detection limit was 25 ?g/L. The mean recovery was 89.84%, CV of inter day and intra day were no more than 5%.After a single oral dose of 10 mg glipizide test or reference tablet, the main pharmacokinetic parameters AUC 0-15, AUC 0-∞, T max ,C max and t 1/2 were (3 502.78?635.82) , (3 214.23?590.46)?g/( L?h),(3 868.22?699.93), (3 593.94?638.60)?g/(L?h),(3.85?1.44), (3.76?1.13)h, (550.80?110.19), (531.15?148.42)?g/L,(3.57?1.11)h and (3.80?1.06)h ,respectively. The relative bioavailability F 0-15 ,F 0-∞ were (110.6?19.8)% and (108.8?17.9)%. Conclusions: No significant difference exists among the pharmacokinetic parameters for the experimental tablets and the reference. The two formulations were bioequivalent.

Objective To investigate the strategies of nutritional therapy for severe acute pancreatitis (SAP) patients.Methods Two hundred and eight patients with SAP were randomly divided into early enteral nutrition (EEN) group,late enteral nutrition (LEN) group and total enteral nutrition (TPN) group.EEN group received enteral nutrition through nasojejunal tube feeding within 72 hours in the course of disease,LEN group received the same treatment after 6 d in the course of disease,TPN group received total enteral nutrition.The index of nutrition,incidence of complication were recorded,efficacy and safety were analyzed.Results EEN group included 67 cases,while 72 cases in LEN group and 69 cases in TPN group.The incidence rate of malnutrition after 14 d of treatment in EEN group and LEN group was significantly lower than that in TPN group [59.7％ (40/67),58.3％ (42/72) vs.84.1％ (58/69),P ＜0.05],but the difference between EEN group and LEN group was not statistically significant (P ＞ 0.05).There was no statistically significant difference in the incidence of hyperlipidemia and hyperglycemia among three groups (P ＞ 0.05).The expression level of albumin,prealbumin and retinol-binding protein after 14 d of treatment in three groups compared with those before treatment and the differences were statistically significant (P ＜ 0.05).The expression level of albumin,prealbumin and retinol-binding protein after 14 d of treatment in EEN group and LEN group compared with those in TPN group and the differences were statistically significant (P＜ 0.05),there was no statistically significant difference between EEN group and LEN group (P＞ 0.05).The incidence rate of total infections,abdominal infections,bloodstream infections,secondary superinfections in TPN group were significantly lower than those in EEN group and LEN group [62.3％(43/69) vs.25.4％ (17/67),19.4％(14/72) ;21.7％(15/69) vs.4.5％(3/67),6.9％ (5/72);39.1％(27/69) vs.14.9％(10/67),11.1％(8/72) ;33.3％ (23/69) vs.9.0％ (6/67),9.7％(7/72),P ＜ 0.05],there was no statistically significant difference in the incidence of peripancreatic infections,lung infections among three groups (P ＞ 0.05).There was no statistically significant difference in the incidence of total infections,different infections between EEN group and LEN group.There was no statistically significant difference in the incidence of noninfections among three groups (P ＞ 0.05).The rate of recovery in EEN group and LEN group was significanby higher than that in TPN group [91.0％ (61/67),94.4％ (68/72) vs.81.2％ (56/69),P ＜ 0.05],but the difference between EEN group and LEN group was not statistically significant (P ＞ 0.05).Conclusion Early enteral nutrition therapy for SAP patients is safe and effective,and could significantly improve the prognosis of patients.

Purpose To study the application of MR diffusion tensor imaging (DTI) quantitative parameters in grading of cerebral glioma on a 3.0T scanner. Materials and Methods DTI mapping of 51 cases of cerebral glioma confirmed by pathology were retrospective analyzed. All the cases were divided into two groups: low-grade gliomas (grade I-II, 18 cases) and high-grade gliomas (grade III-IV, 33 cases). Value of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), radial diffusivity (RD),λ1, λ2, and λ3 of the tumor, peritumoral edema and contralateral tissue area were recorded and compared. Results rMDt, rADt, rRDt, λ1t, λ2t and λ3t of tumor in the low-grade glioma group were higher than those in the high-grade glioma group, and the difference was statistically significant (t=-3.235- -2.458, P<0.05). rFAt was lower in the low-grade glioma group, and the difference was not statistically significant (t=1.554, P>0.05). rFAe of peritumoral edema in the low-grade glioma group was higher than those in the high-grade group, while rMDe, rADe, rRDe, λ1e, λ2e and λ3e were lower in the low-grade group. All differences were not statistically significant except λ1e (t=2.052, P<0.05). ROC analysis showed the area under the curve (Az) of rMDt, rADt, rRDt, λ1t, λ2t, λ3t and λ1e were 0.746, 0.710, 0.762, 0.735, 0.722, 0.705 and 0.374, respectively. Az value of rMDt, rADt, rRDt,λ1t, λ2t, λ3t were statistically different between the low- and high-grade gliomas (Z=3.287-4.605, P<0.001). Conclusion Among DTI quantitative parameters on glioma grading, rMD, rAD, rRD, λ1, λ2, and λ3 of tumor area are helpful in grading gliomas.

Objective To establish the animal model of chronic thromboembolic pulmonary hypertension(CTEPH) and to compare the accuracy of dual-energy CT (DECT) pulmonary angiography and histopathology for detecting CTEPH. Methods Eighteen canines were included in the study. All canines underwent paracentesis, embolization, CT scanning, pressure measurement and tranexamic acid feeding. The procedures were repeated every two weeks, until systolic/diastolic pressure in canines was≥30/15 mmHg or mean pulmonary artery pressure ≥ 20 mmHg.And then canines were sacrificed for histopathology examination. For CT pulmonary angiography (CTPA)in DE mode and DECT lung perfused blood volume (Lung PBV) images, the presence or absence of PE or perfusion defects were recorded on a per-canineand aper-lobe basis. With histopathological results as reference standard, the sensitivity, specificity of CTPA and lung PBV to detect PE were computed for two readers. The pairedχ2 test (McNemar test) was used to analyze the difference in diagnostic accuracy between CTPA and Lung PBV. Inter-reader agreement was also calculated with kappa test. Results CTEPH was demonstrated in 13 canines. On a per-canine basis, both readers found uneven and peripheral perfusion defects with DECT in 11 canines (84.6%, 11/13); while 5 canines (38.5%, 5/13) had cutoff or sudden stenosis of pulmonary arteries with CTPA;on a per-lobe basis,
both readers had sensitivities of 14.3%(5/35), 83.3%(30/36), specificities of 100.0%(30/30), 100.0%(29/29), accuracies of 53.8%(35/65), 90.8%(59/65)for CTPA and DECT, respectively. DECT had a higher sensitivity(χ2=-4.690,P<0.01)and accuracy(χ2=8.284,P<0.01) in detecting CTEPH. Excellent and moderate inter-reader agreements were observed with CTPA and DECT (Kappa=0.938, 0.572, both P<0.001). Conclusions It is feasible to make a CTEPH animal model with autologous thrombus. DECT shows a higher accuracy than CTPA to detect CTEPH in this canine model study.

Objectives To detect the abnormalities of CD34+ cells differentiation and bone marrow cell cycle in myelodysplastic syndrome (MDS). Methods Fifty newly diagnosed MDS ( 17 in low risk and 33 in high risk), 8 acute myeloid leukemia preceded by MDS (MDS-AML) and 25 normal controls were enrolled into this study. Their CD34+ CD38+, CD34+CD38- bone marrow cells and bone marrow cell cycle were measured with flow cytometry. Results The mean percentages of CD34+ cells in bone marrow karyocyte of high risk [ (2.29 ±2.17)% ] and MDS-AML groups [ ( 18.69 ± 17.47)% ] were significantly higher than that of control group [ ( 0.36 ± 0.49 )%, P ＜ 0.05 ]. The mean percentages of CD34+CD38+ cells were significantly lower in low risk, high risk and MDS-AML groups [ ( 86.09 ± 7.79 )%, ( 81.68 ± 11.82)% and (82.88 ±2.60)%, respectively] than that in control group [ (92.21 ±3.85)%, P＜0.05], thus the percentages of CD34+CD38- cells were significantly higher in either MDS (low risk and high risk) or MDS-AML groups [ (13.91 ±7.79)%, (18.32 ±11.82)% or (17.13 ±2.60)%, respectively] than that in control group [ (7.79 ± 3.85 )%, P ＜ 0.05 ]. The percentages of CD+34 CD-38 cells of MDS cases correlated directly with their International Prognostic Scoring System (IPSS) (r =0.493, P =0.001 ) and WHO Adapted Prognostic Scoring System (WPSS) ( r = 0.586, P = 0.000 ) scores. The percentages of bone marrow mononuclea cells (BMMNCs) in G0/G1 phase of in low risk, high risk and MDS-AML groups [ (94.52 ±4.32)%, (96.07 ± 3.88 )% and (94.65 ± 4.55 )%, respectively ] were significantly higher than that in control group[ (88.94 ±7.30)%, P ＜0.01 ], thus the percentages of BMMNCs in S and G2/M phase were significantly lower in either MDS (low risk and high risk) or MDS-AML groups than that in control group (P＜0.05). MDS patients with low percentages of CD34+CD38- cells presented higher therapeutic efficacy than those with high percentages of CD34+CD38- cells, while without significant differences ( P ＞ 0.05 ) .Conclusions There are abnormalities of differentiation of CD34+ bone marrow cells and high proportion of G0/G1 cells which indicates a G1 phase arrest in MDS that might be involved in the pathogenesis of MDS. So the examination of CD34+ bone marrow cells and cell cycle might be helpful for MDS diagnosis and assessment of prognosis and therapeutic effects.

Objective To assess the efficacy and safety of monoclonal antibody rituximab combined with cyclophosphamide (CTX) in the treatment of refractory and recurrent autoimmune hemolytic anemia.Methods Seven cases with refractory and recurrent autoimmune hemolytic anemia ( including 1 case of Evans syndrome) were recruited during January,2007 to December,2010.Treatment regimens were as follows:rituximab:375 mg/m2,1 time/week,2-6 courses; CTX:1 g,1/10 d,2-7 courses; combined with intravenous immunoglobulin (IVIG) 5 g,1 time/week,given 1 day after rituximab administration.The efficacy and safety of this regimen were assessed during follow-up.Results All the patients showed good responses (7/7).Six patients achieved complete remission (6/7) and one achieved partial remission ( 1/7 ).Average follow-up time for the patients was 27 months.All patients remained in remission during the 12-month follow-up visits.Two patients showed elevated indirect bilirubin and increased reticulocyte counts within 24 months.One patient achieved complete remission after additional rituximab therapy,and another patient remained partial remission after cyclosporine therapy.At the time of 36-month follow-up visit,the patient relapsed and was retreated with 3 courses of rituximab combined with CTX and eventually achieved partial remission.All patients tolerated the treatment well with few mild side effects.Conclusions Rituximab combined with CTX is effective and relatively safe in patients with refractory and recurrent autoimmune hemolytic anemia.Additional treatment to relapse patients about 12-24 months after drug withdrawal continues to be effective.

Objective:To explore the effect of miR-7 on the formation of abdominal aortic aneurysm by up-regulating the expression of ERK and MMP-9 proteins.Methods:Download the miRNAs expression profile chip data from the GEO database, the differentially expressed miRNAs were screened out by GEO2R and the correlation between target genes and ERK genes was analyzed; twenty 4-week male C57BL/6J mice with SPF grade were selected and randomly divided into a model group (only 0.6% BAPN solution was given, n=10) and miR-7 group (fed with 0.6% BAPN solution+ injected with 1×10 9 PFU/ml lentivirus containing miR-7 over expression gene via tail vein, n=10), after 7 weeks of continuous feeding, the mice were anesthetized intraperitoneally with chloral hydrate. After the abdominal cavity and thorax were dissected, the abdominal aorta was separated from the left ventricle perfusion with normal saline under physiological pressure and pathological sections were prepared. Masson staining and α-SMA staining were used to evaluate the lesion degree of abdominal aortic vessels in each group; the protein expression levels of p-ERK1/2 and MMP-9 in the diseased abdominal aorta of each group were detected by WB. Results:By screening differential genes, we found that miR-7 was highly expressed in patients with abdominal aortic aneurysm, and further analysis revealed that miR-7 was positively correlated with ERK1/2; Masson staining showed that the tumor of abdominal aortic aneurysm in miR -7 group was significantly larger than that in the model group, and the difference was statistically significant ( P<0.05), the results of α-SMA histochemical staining showed that the number of α-SMA positive VSMC in miR-7 group was significantly lower than that in the model group, and there was almost no-SMA staining in some VSMC; WB results showed that the highest expressions of P-ERK1/2 and MMP-9 proteins in the abdominal aorta of miR-7 group were significantly higher than that of the model group, with statistically significant differences ( P<0.05). Conclusion:MiR-7 accelerated the formation of abdominal aortic aneurysms by up-regulating the expression of ERK and MMP-9 proteins.

Objective: To investigate the levels of NK cells and their relevant cytokines (IL-10, TGF-β and IFN-γ) in patients with primary immune thrombocytopenia (ITP) . Methods: All samples were obtained from 42 patients (22 newly diagnosed and 20 in remission) and 20 healthy volunteers. The levels of IL-10 and IFN-γ in blood serum were detected by enzyme-linked immunosorbent assay (ELISA) . The percentage of CD3^- CD56⁺ NK cell, CD3^- CD56^bright CD16^- NK cell, CD3^- CD56^dim CD16⁺ NK cell in peripheral blood lymphocyte were detected by flow cytometry. The NK cells were isolated by immunomagnetic microbeads. The mRNA expression levels of IL-10, TGF-β, and IFN-γ in NK cells were detected by real-time fluorescent quantitative PCR. Correlation between the above measured results was analyzed. Results: ① The blood serum level of IFN-γ in newly diagnosed ITP patients [ (653.0±221.6) ng/L] was higher than that in remission ITP patients [ (484.4±219.5) ng/L] and healthy control [ (390.9±253.5) ng/L] (P=0.022, P=0.001) . The blood serum level of IL-10 in newly diagnosed ITP patients was lower than that in healthy control [ (52.09±26.66) ng/L vs (79.44±38.43) ng/L, P=0.007]. ②The percentage of NK cell in newly diagnosed and remission ITP patients [ (9.53±3.93) %, (9.03±3.78) %] were significantly lower than that in healthy control [ (13.72±7.42) %] (P=0.013, P=0.007) . The ratio of CD3^- CD56^bright CD16^- NK cell/total NK cells in newly diagnosed ITP patients was higher than that in healthy control [ (6.85±4.43) % vs (4.05±2.81) %, P=0.032]. The ratio of CD3^-CD56^dim CD16^- NK cell/total NK cells in newly diagnosed ITP patients was lower than that in healthy control [ (93.14±4.43) % vs (95.94±2.81) %, P=0.032]. ③ There was no significant difference in the mRNA expression level of IFN-γ in NK cells of ITP patients and healthy control (all P>0.05) . The mRNA expression levels of IL-10 and TGF-β in NK cells in newly diagnosed ITP patients were significantly higher than that in healthy control (1.82±1.32 vs 1.02±1.03, P=0.023; 2.80±2.31 vs 1.46±1.37, P=0.028) . The ratio of CD3^-CD56^bright CD16^- NK cell/total NK cells was positively correlated with the mRNA expression levels of IL-10, TGF-β in NK cells (r=0.424, P=0.001; r=0.432, P<0.001) . Conclusion NK cells may compensate for the deficiency of the number by enhancing the secretion of negative regulation cytokines, acting as "protective" roles in the disease.

OBJECTIVETo learn more about the clinical and laboratory features of patients with paroxysmal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years.

METHODSClinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively.

RESULTSIn comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cases showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18.5% to 38.5%); more cases without hemoglobinuria (from 24.2% to 38.5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differentiation were found in 19.2%, 12.2% and 8.9% of the PNH patients, respectively. 12.3% of the patients had bone marrow hypoplasia, and most of them had no hemoglobinuria. Ham's tests were negative in about 34.2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100.0% of the cases, suggesting that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortical hormone was effective in 83.8% of the patients, 54.2% of whom relapsed within one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2 - 6 mg x d(-1); Prednisone 0.5 mg x kg(-1) x d(-1)). Five (62.5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients.

CONCLUSIONSPNH, an acquired blood disease seen more often among adult males, can be diagnosed more sensitively by hemocyte member CD55 and CD59 tests and treated more effectively with adrenocortical hormone or low dose chemotherapy.

Adolescent ; Adult ; Aged ; Child ; Female ; Hemoglobinuria, Paroxysmal ; diagnosis ; physiopathology ; Humans ; Male ; Middle Aged ; Retrospective Studies