Objective To investigate clinical presentation, diagnosis,gestational outcome and treatment of Eisenmenger’s syndrome during pregnancy. Methods Two patients with Eisenmenger’s syndrome during pregnancy were reported retrospectively. Results The first case was admitted in labor at 34 weeks and 4 days in pregnancy,She had dysphoria,tachypnea and dyspnea right after vaginal delivery and died immediately The diagnosis of pulmonary embolism was highly suspected clinically. The second case was admitted because of fetal growth restriction Cesarean section was performed after cyanosis worsened at 35 weeks and 4 days She was discharged from the hospital two weeks after delivery. Conclusion Eisenmenger’s syndrome during pregnancy was associated with extremely poor maternal and fetal outcome Pregnancy is contraindicated in such patients Contraception is necessary after marriage Pregnancy should be terminated as early as possible in such women. In cases who intends to continue the pregnancy,closely observation must be provided to ensure the safety for both mother and the fetus.

A novel chitosan derivant, N-octyl-N-arginine chitosan (OACS) with a mimetic structure of cell-penetrating peptides was synthesized by introducing hydrophilic arginine groups and hydrophobic octyl groups to the amino-group on chitosan's side chain. Structure of the obtained polymer was characterized by FT-IR and 1H NMR. The substitution degree of octyl and arginine groups was calculated through element analysis and spectrophotometric method, separately. The critical micelle concentration of OACS was 0.12 - 0.27 mgmL(-1) tested by fluorescence spectrometry. The solubility test showed OACS could easily dissolve in pH 1 - 12 solutions and self-assemble to form a micelle solution with light blue opalescence. The OACS micelles have a mean size of 158.4 - 224.6 nm, polydisperse index of 0.038 - 0.309 and a zeta potential of +19.16 - +30.80 mV determined by malvern zetasizer. AFM images confirmed free OACS micelle has a regular sphere form with a uniform particle size. MTT test confirmed that OACS was safe in 50 - 1 000 micromol-L(-1). The result of HepG2 cell experiment showed that the cell internalization of OACS micelles enhanced with increased substitution degree of arginine by 40 folds compared to chitosan. Thus, OACS micelles were a promising nano vehicle with permeation enhancement and drug carrier capability.

To discuss the quantitative management and assessment of clinical internship of medical laboratory science. Instruments of the quantitative management and assessment of integrated clinical abilities have been established to enhance management of internship and to improve the quality of internship training.

Objective To investigate the effect of serum of esophageal squamous cell carcinoma (ESCC) patients with blood stasis syndrome (BSS) on proliferation and cycle of EC9706 cells, and to explore the action of blood micro-environment of ESCC patient with BSS on EC9706 cells. Methods Human EC9706 cells were cultured in an incubator with RPMI-1640 medium containing fetal bovine serum ( FBS) , at 37℃ and under 5% saturated humidity for 24 h. After EC9706 cells were starved in serum-free medium for another 24h, the three experimental groups were treated with serum of ESCC patients with BSS, serum of ESCC patients with spleen-qi deficiency syndrome (SQDS), and serum from healthy volunteers, respectively. Cell proliferation was determined by methyl thiazolyl tetrazolium ( MTT) assay, EC9706 cell morphology was observed under light microscope, and cell cycle was measured by flow cytometer (FCM). Results The serum concentrations of ESCC patients with BSS and ESCC patients with SQDS for obtaining 50 percent cell proliferation rates ( PI50) were 71.1 μL/mL and 118 μL/mL, respectively. And the proliferation of EC9706 cells in the both groups all arrived to the peak values at culturing hour 48. The light microscopy results showed that the feature of EC9706 cells in both groups presented as spindle-like or polygon-like shape, and cell count in BSS group was larger than SQDS group. FCM assay results for EC9706 cell cycle showed that the percentage of G1-phase EC9706 was decreased and the percentage of S-phase EC9706 was increased in BSS group as compared with those in SQDS group ( P<0.05). Conclusion Serum micro -environment in ESCC patients with BSS is more beneficial to EC9706 cells proliferation than ESCC patients with SQDS, and the mechanism may be related to the regulation of cell cycle.

Objective To investigate the effect of median nerve electrical stimulation on the activity, speech and quality of life in patients after severe traumatic brain injury. Methods 60 patients with severe traumatic brain injury were divided into 2 groups: the control group (A) and the treated group (B). The patients in Group A were treated with the routine medicine and rehabilitation. Based on the routine therapy, the patients in Group B were treated with the right median nerve electrical stimulation. Their outcome, activity, speech and quality of life were assessed. Results The scores of Glasgow Outcome Scale (GOS), speech assessment, and the quality of life in Group B improved compared with that in Group A (P<0.05), the activity seemed to improve, but not significantly. Conclusion The right median nerve electrical stimulation is effective to promote the recovery of neurological function and improve the quality of life in patients with severe traumatic brain injury.

Objective To investigate the effects of sevoflurane pretreatment on the inflammatory response in patients undergoing cardiac valve replacement with cardiopulmonary bypass (CPB) and the mechanism of myocardial protection.Methods Twenty NYHA class Ⅱ or Ⅲ patients of both sexes,aged ＜ 60 yr,undergoing cardiac valve replacement with CPB,were randomly divided into 2 groups (n=10 each): sevoflurane group (group S) and control group (group C).The patients were premedicated with intramuscular morphine 0.1 mg/kg and scopolamine 0.3 mg.Anesthesia was induced with iv injection of midazolam 0.05-0.08 mg/kg,fentanyl 10-15 μg/kg and pipecuronium 0.08-0.10 mg/kg.The patients were tracheal intubated and mechanically ventilated.Anesthesia was maintained with intermittent iv boluses of midazolam0.03-0.06 mg/kg,fentanyl 5-10μg/kg and pipecuronium 0.04-0.08 mg/kg.Sevoflurane was inhaled before aortic clamping and the end-tidal concentration was rapidly adjusted to 1.0％ and maintained at this level for 30 min in group S.Blood samples were taken from the central vein before skin incision,immediately after aortic clamping,immediately after aortic unclamping and at 30 min after aortic unclamping,at 2,6,12 and 24 h (T1-8) after operation for determination of the concentrations of plasma tumor necrosis factor-α (TNF-α),interleukin-6 (IL) and interleukin-8 (IL-8),intercellular adhesion molecule-1 (ICAM-1),cardiac troponin I (cTnI) and activity of creatine kinase MB (CK-MB).The requirement for cardiovascular drugs was recorded after release of aortic cross clamp.Results Compared with group C,the plasma concentrations of TNF-α,IL-6 and IL-8 were significantly decreased at T3-8,the plasma concentrations of ICAM-1 and cTnl were significantly decreased at T4-8,the activity of plasma CK-MB was significantly decreased at T8,and the requirement for cardiovascular drugs was significantly reduced after release of aortic cross clamp in group S (P ＜0.05).Conclusion Sevoflurane pretreatment can inhibit the inflammatory response and provide myocardial protection to some extent in patients undergoing cardiac valve replacement with CPB.

Objective To investigate proteolipid protein 1 (PLP1) mutations in six pedigrees with Pelizaeus-Merzbacher disease (PMD),and to provide prenatal consulting and prenatal diagnosis.Methods Subjects were six probands with PMD admitted in Department of Pediatrics,Peking University First Hospital from July 2006 to November 2011 and their family members.Genomic DNA sarnples were extracted from peripheral bloods of probands and their family members.Multiplex ligation-dependent probe amplification (MLPA) technique was used to detect PLP1 duplication mutation.Direct DNA sequencing was used to detect point mutation.Genetic diagnosis were based on PLP1 mutation genotype from probands.Prenatal diagnosis of nine fetuses were performed from seven PLP1 mutation female carriers by fetuses' DNA extracted from amniocytes or villus cells.Results PLP1 duplications were found in probands 1-4 (P1-4) whose mothers and the aunt of proband 1 (P1) were PLP1 duplications carriers.The two cases of point mutation,c.96C＞G(p.F32L) and c.623G＞T (p.G208V),were found in proband 5 (P5) and proband 6 (P6).Hcterozygous changes of the same mutations were found in P5' and P6' mothers with normal phenotypes.Seven female PLP1 mutation carriers were pregnant again.Prenatal diagnosis of PLP1 for nine fetuses presented one PLP1 duplication,one point mutation,one PLP1 duplication carrier,and six wildtypes.A segmental crossing over of X chromosome was detected in one male fetus of PLP1 wildtype.Conclusions PLP1 mutation analysis could help to diagnose PMD pedigree and to identify female PLP1 mutation carrier in the family.The following prenatal diagnosis and proper genetic counseling are very important to prevent PMD child from being delivered.

Objectives To provide genetic counseling and prenatal molecular diagnosis for two families with megalencephalic leukoencephalopathy with subcortical cysts (MLC).Methods Two MLC patients (probands 1 and 2) were admitted to the Department of Pediatrics of Peking University First Hospital in June 2011 and June 2009,respectively.Peripheral blood was collected and DNA sequencing was performed for genetic analysis for the two MLC patients and their parents.Amniotic fluid and villus of two fetuses (fetus 1 and 2) were collected at 21+4 and 12+3 weeks of gestational age from their mothers when they were pregnant again.The genomic DNA of the two fetuses was extracted and corresponding sites of MLC1 gene were sequenced.Haplotype analysis using a combination of 3 microsatellite markers (AR,DXS6807 and DXS6797) on chromosome X and sex determining region of Y chromosome was performed to detect maternal cell contamination.Verification of the prenatal molecular diagnosis and follow up study after birth were conducted for both fetuses.Results Macrocephaly,motor development delay and typical findings on brain MRI were identified in the two probands,and were clinically diagnosed with MLC.Compound heterozygous mutations were detected in proband 1 [c.353C＞T (p.T118M) and c.803C＞G (p.T268R)] and proband 2 [c.353C＞T (p.T118M) and c.836T＞C(p.L279P)],respectively.MLC was genetically diagnosed.Heterozygous variation in c.353[c.353C＞T (p.T118M)] and wild c.803C were identified in fetus 1,and both wild c.353C and c.836T were found in fetus 2.No maternal cell contamination was detected in both fetuses.Sequencing the corresponding sites after birth confirmed the prenatal diagnosis,and the head circumference and motor development were normal in fetus 1 at 5 months old.No macrocephaly was found and no DNA sequencing was done in fetus 2 at one month old.Conclusions Genetic counseling and prenatal molecular diagnosis for MLC families combined with clinical and genetic diagnosis are important in preventing MLC.Haplotype analysis with a combination of three microsatellite markers on chromosome X and sex determining region of Y chromosome is useful in detecting maternal cell contamination and avoiding its influence on prenatal diagnosis,and confirming the reliability of prenatal diagnosis.

Objective To summarize the clinical features of patients with ovarian tumor associated anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis,and to explore the influence of operation patterns on the prognosis.Methods The clinical data of ovarian tumors with anti-NMDAR encephalitis in the Third Affiliated Hospital of Sun Yat-sen University from January 2009 to December 2018 were collected,and the clinical characteristics,treatment and prognosis were analyzed.Results A total of 110 patients with ovarian tumors and anti-NMDAR encephalitis were collected.All patients were positive for anti-NMDAR antibodies in serum and/or cerebrospinal fluid.The age of onset ranged from 10 to 35 years (24.6 ±5.7).About 60.9％ patients'initial symptom was psychosis.The main clinical features were psychosis (97.3％),seizures (83.6％),conscious disturbance (72.7％),autonomic dysfunction (64.5％),dyskinesia (64.5％),central hypoventilation (54.5％) and impaired memory (31.8％).Most patients'symptoms were relieved after surgery and immunotherapy.Postoperative pathological results revealed that ovarian teratoma accounted for 97.8％ of the ovarian tumor.The prognosis of patients with surgery was better than the patients without surgery,with statistically significant difference (P ＜ 0.05).The prognosis of patients with adnexectomy was better than the patients with ovarian tumor excision,with statistically significant difference (P ＜ 0.05).Conclusions Anti-NMDAR encephalitis,with psycho-neurological symptoms as dominant manifestation,is usually associated with ovarian tumor.For young female patients with psychosis,clinicians should be alert to the possibility of anti-NMDAR encephalitis and screen for tumor.Patients with adnexectomy and immunotherapy can get a better prognosis.

Objective: To evaluate the efficacy of reduced-intensity conditioning allogeneic hematopoietic stem cell transplantation (RIC-allo-HSCT) for patients with myelofibrosis (MF). Methods: The clinical data of 10 patients with myelofibrosis (MF) who underwent RIC-allo-HSCT. Results: Of all 10 patients, 6 were male and 4 women, with a median age of 28.5 (22-54). Using fludarabine/busulfan plus total body irradiation (FB+TBI) pretreatment scheme based. Hematopoiesis reconstitution was achieved in 9 patients (90%). The median time of neutrophil and platelet engraftment was 13.5 (10-22) day and 16.5 (13-40) day, respectively. Acute GVHD occurred in 4 cases while chronic GVHD in 5 cases. The prospective OS for 3 years was (90.0±8.5)% after a median follow-up time of 17 months. Transplant related mortality was 1 case. Conclusion RIC-HSCT with FB+TBI is a feasible and effective alternative for MF patients.