OBJECTIVETo study psychiatric features and the manifestations of central nervous system involvement in Chinese patients with primary Sjogren's syndrome (PSS).

METHODSThe Minnesota Multiphasic Personality Inventory (MMPI) was used to study 27 PSS female patients and 57 healthy women. The results from two groups were compared. Ten SS patients were evaluated by electroencephalography (EEG), transcranial doppler ultrasound (TCD) and magnetic resonance imaging (MRI).

RESULTSOf 27 patients tested, the three highest clinical mean scales included Hypochondriasis (Hs), Hysteria (Hy), Psychasthenia (Pt) were found. When PSS group was compared with normal control, T scores of SS patients were significantly higher than the healthy women in Hs, Hy. Abnormal EEG was found in 3 of 9 PSS patients. TCD was abnormal in 5 of 9 patients. MRI in 1 patient showed abnormality.

CONCLUSIONSThis study shows that the personality of PSS patients is abnormal, the features are neuroticism. Central nervous system was markedly involved in Chinese patients with PSS, including abnormal EEG and TCD. The changes of central nervous system may be related to abnormalities of psychiatric changes.

Adult ; Aged ; Electroencephalography ; Female ; Humans ; MMPI ; Magnetic Resonance Imaging ; Middle Aged ; Sjogren's Syndrome ; diagnostic imaging ; physiopathology ; psychology ; Ultrasonography, Doppler, Transcranial

OBJECTIVETo detect gene mutations associated with autosomal dominant congenital stationary night blindness(ADCSNB) in a large Chinese family.

METHODSGenomic DNAs were extracted from peripheral blood samples of 16 affected and 14 unaffected family members. According to 5 missense mutations in 3 genes reported previously, 4 pairs of primers were designed and corresponding exons containing the five mutation sites were amplified by polymerase chain reaction. Amplified products were purified and sequenced by MegaBACE1000 capillary array electrophoresis DNA sequencer. Full field electroretinogram (ERG, ISCEV) of patients was recorded and analyzed by Roland Consult System.

RESULTSDark-adapted ERG showed a-wave was normal, but b-wave of the patients was markedly decreased. None of the five missense mutations were detected in 16 affected and 14 unaffected family members.

CONCLUSIONThe molecular pathogenesis of ADCSNB in this family does not involve point mutations or deletions of these five sites, which indicates the heterogeneity of ADCSNB.

Adult ; Base Sequence ; DNA Mutational Analysis ; Female ; Humans ; Male ; Molecular Sequence Data ; Night Blindness ; congenital ; genetics ; Pedigree ; Point Mutation

OBJECTIVETo study the inhibitory effects of Panax Notoginseng Saponins(PNS) on apoptosis induced by hypoxia/hypoglycemia and reoxygenation in cultured rat hippocampal neurons.

METHODApoptosis were measured by flow cytometry, intracellular free calcium concentration([Ca2+]i) was measured with confocal laser scanning microscopy, morphological changes and neuronal necrosis were observed with fluorescence microscope, and meanwhile the leakage of lactic dehydrogenase(LDH) was measured.

RESULTHypoxia/hypoglycemia cultures for 5 hours and reoxygenation induced neuronal apoptosis and necrosis, and significantly increased neuronal [Ca2+]i and the leakage of LDH. The effects were increased with the extending time of reoxygenation. PNS has could significantly decrease the percentage of neuronal apoptosis and necrosis, and reduce neuronal [Ca2+]i and the leakage of LDH.

CONCLUSIONPNS has inhibitory effect on neuronal apoptosis. This effect might be related to its effect of decreasing intracellular free calcium concentration.

Animals ; Apoptosis ; drug effects ; Calcium ; metabolism ; Cell Hypoxia ; Cells, Cultured ; Fetus ; Ginsenosides ; isolation & purification ; pharmacology ; Hippocampus ; cytology ; Hypoglycemia ; pathology ; L-Lactate Dehydrogenase ; metabolism ; Neurons ; cytology ; Panax ; chemistry ; Plants, Medicinal ; chemistry ; Rats ; Rats, Wistar

OBJECTIVETo construct recombinant lentiviral vectors carrying Rheb gene and its mutant Rheb'D60K gene, and examine their expression in human liver cancer cells.

METHODSRheb gene was amplified by PCR to construct the recombinant plasmid LV31-Rheb-WT and LV31-Rheb-D60K. HEK-293 FT cells were contransfected with the recombinant lentiviral vector together with a lentiviral package plasmid to produce the lentiviral particles. The expression of PS6 protein was detected in the lentivirus-infected MCF-7 cells. The apoptosis of SK-HEP-1 cells transfected with LV31-Rheb-WT or LV31-Rheb-D60K was observed.

RESULTSThe recombinant LV31-Rheb-WT and LV31-Rheb-D60K vectors were confirmed by PCR and DNA sequencing. Western blotting showed that PS6 protein expression was increased in LV31-Rheb-WT-transfected cells while decreased in LV31-Rheb-D60K-transfected cells. LV31-Rheb-D60K-transfected SK-HEP-1 cells showed more obvious apoptosis after starvation than LV31-Rheb-WT-transfected cells.

CONCLUSIONLentiviral vectors carrying Rheb gene and its mutant has been successfully constructed, which can be useful in further investigation of the role of Rheb gene in cancer cells.

Apoptosis ; genetics ; Carcinoma, Hepatocellular ; metabolism ; pathology ; Genetic Vectors ; genetics ; HEK293 Cells ; Humans ; Lentivirus ; genetics ; metabolism ; Liver Neoplasms ; metabolism ; pathology ; MCF-7 Cells ; Monomeric GTP-Binding Proteins ; biosynthesis ; genetics ; Mutant Proteins ; genetics ; Neuropeptides ; biosynthesis ; genetics ; Ras Homolog Enriched in Brain Protein ; Recombinant Proteins ; biosynthesis ; genetics ; Transfection

OBJECTIVETo compare the efficacy and adverse effects between arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) in patients with acute promyelocytic leukemia (APL).

METHODSThe clinical data of 71 patients with newly diagnosed APL were retrospectively analyzed. Two groups were classified according to the induction regimens, namely ATO group (n = 41) and ATRA group (n = 30). The complete remission (CR) rate and the time to CR were compared between these two groups.

RESULTSThe CR rate was 97.5% in ATO group and 93.3% in ATRA group (P > 0.05). The median time to CR was 29 days (21-45 days) in ATO group, which was significantly shorter than 38.5 days (24-63 days) in ATRA group (P < 0.001). Retinoic acid syndrome occurred in 52.9% of patients treated with ATRA, which affected the further use of ATRA.

CONCLUSIONSBoth ATO and ATRA have high response rates for newly diagnosed patients with APL. Compared with ATRA, ATO induction therapy has shorter time to achieve CR and less adverse effects, and therefore may be the first-line therapy for APL.

Adolescent ; Adult ; Aged ; Arsenicals ; adverse effects ; therapeutic use ; Female ; Humans ; Leukemia, Promyelocytic, Acute ; drug therapy ; Male ; Middle Aged ; Oxides ; adverse effects ; therapeutic use ; Remission Induction ; Retrospective Studies ; Treatment Outcome ; Tretinoin ; adverse effects ; therapeutic use ; Young Adult

Objective To explore the efficacy and safty of sorafenib in Child-Pugh class B to class C hepatocellular carcinoma (HCC).
Methods In this three-center open-label study from November 2011 to May 2013, we randomly assigned 189 patients with advanced Child-Pugh class B or C HCC patients into two groups, one group with 95 patient to receive sorafenib (400 mg a time, twice a day) and the other group with 94 patients to receive best supportive care. The primary end points were progression-free survival and overall survival.
Results The median progression-free survival was 2.2 months and 1.9 months in the sorafenib group and best supportive care group respectively (Hazard ratio in the sorafenib group, 0.55; 95% confidence interval, 0.40-0.75;P=0.002). The median overall survival was 4.0 months and 3.5 months in the sorafenib group and best supportive care group respectively (Hazard ratio in the sorafenib group, 0.48;95%confidence interval, 0.35-0.68;P<0.001). The main adverse effect of sorafenib was rash and acne of the skin (in 51.7%patients). The incidences of severe rash, diarrhea, and dry skin were 5.6%, 5.6%, and 2.2%in the sorafenib group. One patient reached partial response in the sorafenib group.
Conclusions Sorafenib is safe in patients with liver function impaired advanced HCC. It is effective in terms of progression-free survival and overall survival compared with best supportive care. Liver functions are the important predictive factors.

OBJECTIVETo evaluate the efficacy of porcine anti-human lymphocyte globulin (P-ALG) plus cyclosporine A (CsA) therapy for severe aplastic anemia (SAA).

METHODSForty-eight SAA patients (31 males, 17 females) including 17 very severe aplastic anemias (vSAA) were treated with ALG plus CsA between 1999 to 2009 in our hospital and the outcomes were analyzed retrospectively for early mortality, response rate and quality, survival rate, toxicity and complications.

RESULTSThe median age was 28 (13 - 64) years. The interval from diagnosis to treatment was 45 days. The median neutrophil count at diagnosis was 0.178 × 10(9)/L. Overall response was 83.3% (54.2% complete, 29.2% partial) with a median time of 90 (23 - 380) days. 10.4% died of infection within 30 days mainly of fungi infection. Only 1 patient relapsed 2 years after treatment. No clonal disease was found. The 1.5-year survival rate was 87.5%. vSAAs had less response, higher early mortality and less survival (64.7%, 29.4% and 51.8%, respectively) compared to that of SAA (93.5%, 0, 100%, respectively, P < 0.05). Grouped patients with different age, gender, intervals between diagnosis and treatment and pre-existing infections had similar response. The main side effects were fever and skin rash (52.1%), serum sickness (16.7%), impaired liver function (60.4%) and hemorrhage (2.1%). No treatment-related mortality was found.

CONCLUSIONP-ALG plus CsA is an ideal and well tolerated treatment for SAA but not for vSAA.

Adolescent ; Adult ; Anemia, Aplastic ; drug therapy ; Animals ; Antilymphocyte Serum ; therapeutic use ; Cyclosporine ; therapeutic use ; Female ; Humans ; Immunosuppressive Agents ; therapeutic use ; Lymphocytes ; immunology ; Male ; Middle Aged ; Retrospective Studies ; Swine ; Treatment Outcome ; Young Adult

OBJECTIVETo evaluate the efficacy and safety of recombinant human thrombopoietin (rhTPO) combined with glucocorticoid in treatment of severe newly diagnosed primary immune thrombocytopenia (ITP).

METHODSFrom June 2009 to December 2012, 24 male patients and 38 female patients with the diagnosis of severe primary ITP in our hospital were randomized into trial group (31 cases) or control group (31 cases), the median age was 50 years (range: 21-84 years). Trial group was treated with rhTPO combined with glucocorticoid, and control group was treated with glucocorticoid only.

RESULTSAt the day 3, 7 and 14 from the beginning of treatment, the average platelet count (APC) in trial group[(35.5±24.9)×10⁹/L, (135.2±94.9)×10⁹/L and (192.0±109.1)×10⁹/L]were significantly higher than that in control group[(24.5±15.6)×10⁹/L, (78.2±121.9)×10⁹/L and (95.8±60.5)×10⁹/L, P=0.022, 0.009 and 0.001, respectively]. There was no significant difference in APC between the two groups at day 28 and 90 after treatment[(147.8±59.1)×10⁹/L vs (105.1±56.9)×10⁹/L, P=0.243; (137.4±52.3)×10⁹/L vs (104.3±59.8)×10⁹/L, P=0.568, respectively]. At the day 7, 14 and 28, the complete response rates in trial group were 61.3%, 87.1% and 80.6%, which were also significantly higher than that in control group (16.1%, 29.0% and 48.3%, P=0.000, 0.000 and 0.004, respectively). The median time to response in trial group was 3 days while in the control group was 5 days; the median duration of complete response in trial group was 76 days while in the control group was 54 days. In trial group, there were 4 cases treated with platelet transfusion, while in control group there were 11 cases, respectively.

CONCLUSIONFor patients with severe primary ITP, rhTPO combined with glucocorticoid could rapidly increase the platelet count, significantly improve the complete response rate and prolonged the effect with a low incidence of tolerable adverse events compared to single use of glucocorticoid. rhTPO combined with glucocorticoid could be a new therapeutic choice to those patients.

Adult ; Aged ; Aged, 80 and over ; Female ; Glucocorticoids ; therapeutic use ; Humans ; Male ; Middle Aged ; Platelet Count ; Platelet Transfusion ; Purpura, Thrombocytopenic, Idiopathic ; drug therapy ; Recombinant Proteins ; therapeutic use ; Thrombopoietin ; therapeutic use ; Treatment Outcome ; Young Adult

Objective:To explore the relationship between interleukin 6 (IL-6) ,C reactive protein (CRP) and the staging of multiple myeloma (M M ) patients and their role in predicting efficacy .Methods:Serum IL-6 and CRP levels in M M patients admitted to the Department of Hematology ,Zhongshan Hospital Affiliated to Fudan University ,between January 1 , 2015 and January 31 ,2016 were reviewed and the differences of serum IL-6 and CRP levels in MM patients with different DS stages ,ISS stages and chemotherapy efficacy (4 circles) were analyzed .Results:There was no significant difference in serum IL-6 and CRP levels in the patients with different DS stages or ISS stages .There was statistical significance (P< 0 .05) in serum CRP levels in the patients pre-treatment between the high-efficiency group ,the medium-efficiency group ,and the ineffective group .Conclusions:Elevated CRP might indicate poor efficacy of chemotherapy in MM patients ,and IL-6 level is not enough to reflect the status of myeloma IL-6 signaling pathway .

Objective:To analyze the Frailty score in the prognosis of elderly multiple myeloma.Methods:Twenty nine multiple myeloma patients aged above 65 year-old admitted from January 1,2015 to February 29,2016 were enrolled in the study.Frailty score assessment was performed and its relation with clinical outcome was analyzed.Results:The 1 3 patients were classified into high risk group (44.8%),5 cases in mediate group (17.2%),and 11 cases in low risk group (37.9%). There were no statistical significance in the aspects of ISS stage (P= 0.281)or chemotherapy intensity (P= 0.475)found among the three groups.More patients (69.2%)in the Frailty high risk group suffered severe hematologic adverse events (≥grade 3),which was significantly higher than low risk group (18.2%,P= 0.014)and mediate risk group (0.0,P= 0.011). The occurrence of adverse reaction in severe non-hematologic group (≥grade 3)(84.6%)was higher than that of low risk group(18.2%,P= 0.001)and that of mediate risk group(20.0%,P= 0.011).There were 69.2% of patients in high risk group had chemotherapy discontinuation,delay or chemotherapy intensity reduction,which was significantly higher than low risk group (9.1%,P= 0.004),and no statistical significance was observed in the mediate risk group (40.0%,P= 0.268).In the terms of therapy efficacy,30.8%,23.1%,and 46.2%patients obtained complete remission or very good remission(CR+VGPR),partial remission (PR),and no remission (NR)in the high risk group,which were significantly lower than low risk group(CR+VGPR 63.6%,PR 36.4%,NR 0.0,P= 0.027).No statistical significance of the efficacy was found between high risk group and mediate risk group (CR+VGPR 40.0%,PR 20.0%,NR 40.0%,P= 0.751).Conclusions:The Frailty score can predict the adverse reaction and treatment efficacy,but with poor prognosis in high risk patients,and its clinical value in prognosis required further research.