Tuberous sclerosis complex (TSC) is a genetic multisystem disorder that has an impact on many organ systems.It is a common neurocutaneous syndrome in children, the main clinical manifestations characterized by impairment of skin and nervous system.The most frequent neurological symptoms are seizures,which occur in up to 90％ of patients and often are intractable,followed by autism spectrum disorders, intellectual disability, attention deficit-hyperactivity disorder, and sleep, et al.Conventional treatment has frequently proven insufficient for neurological and behavioral symptoms, particularly seizure control.Currently, there are many studies focus on the mechanism-based treatment in TSC.Therefore, this review will focus on the genetic mechanism of TSC, further exploring personalized medicine and prognosis for TSC shows good prospects.

Objective To evaluate the CT appearances and diagnostic value of malignant obstructive jaundice.Methods The CT signs of malignant obstructive jaundice proved by surgery and pathology in 50 cases were analyzed ,among them ,there were 21 cases of cholangiocarcinoma,15 cases of the head of pancreas, 8 cases of ampullary carcinoma, 4 cases of gallbladder carcinoma and 2 cases of cholangiocarcinoma complicated with gallbladder carcinoma.Results CT features of malignant obstructive jaundice included dilatation of the bile ducts which presented as“soft rattan sign”,other findings were “truncation sign”,“soft tissue mass”,“double duct sign”,lymphadenopathy and ascites .Conclusion CT examination has important value in diagnosing malignant obstructive jaundice. It can supply accurate location and causes of obstructive jaundice.

Objective　To establish a rapid,effective,convenient method for detecting human adenoviruses and other viral pathogens using magnetic bead separation and PCR amplification.Methods　A biotinylated oligonucleotide primers were hybridized to adenovirus DNA in stool samples.Setreptavidin coated magnetic beads were then added to isolate the DNA-oligonucleotide hybrid.The procedure allows for the recovery of viral DNA suitable for amplification by polymerase chain reaction.Results　Ten samples collected from clinical stool were detected; six of them were positive.Results indicated that this nucleic acid separation technology is very effective in concentrating and purifying adenoviruses DNA while removing PCR inhibitors in stool samples.It also effectively increase the sensitivity of PCR amplification.Conclusion　This technique can rapidly,reliably detect adenoviruses in clinical samples,and it can be used to detect other viral pathogens.

Objective To study the changes in pulmonary diffusing capacity (DL), alveolar capillary membrane diffusing capacity (Dm) and pulmonary capillary blood volume (Vc) in patients with severe acute respiratory syndrome (SARS), and to elucidate the underlying pathophysiogical mechanism of reduction of pulmonary diffusing capacity. Method Spirometry was performed in 26 SARS patients and 12 healthy individuals in resting condition. DLco were measured by single breath method, estimations of Dm and Vc were done by the method of Roughton and Forster. Results DLco in SARS patients was significantly lower than that in normal control, the same was true for Dm and Vc. The severer the pulmonary lesion, the heavier the damage to the pulmonary diffusing funetion. Conclusion The changes in Dm and Vc were both found in patients with SARS. Their measurements were helpful for detecting pulmonary involvement in SARS and defining the reason of DL abnormality in SARS. Dm and Vc were important and sensitive for monitoring pulmonary diffusing function in SARS patients

As the indexes of reflecting whole blood coagulation and fibrinolytic capacity, thrombelas-tography are being increasingly used in the fields of guiding intraoperative blood transfusion, hypercoagulable state monitoring and correction, treatment of trauma patients, and clotting mechanism research. This article reviews the clinical application value and limitation of thrombelastography in patients with ischemic cerebrovascular disease.

Objective To evaluate the antitumor effects of chenodeoxycholic acid-verticinone ester ( CDCA-Ver ) on tumor growth and immune system of H22-bearing mice. Methods Antitumor activity against a solid tumor mass was evaluated in Kunming mice. H22 cells were transferred into the abdomen cavity of Kunming mice. H22 cells were inoculated through subcutaneous injection at the right armpit of the mouse to establish a solid tumor model. At 24 h after H22 tumor cells inoculation, 40 tumor-bearing Kunming mice were randomly divided into 4 groups according to random number table ( n=10 each group):model control group, cyclophosphamide ( CTX) group, intraperitoneal CDCA-Ver injection group and intravenous CDCA-Ver injection group. In model control group, sterile 0. 9% sodium chloride solution (10 mL·kg-1 ) was intraperitoneally injected once daily. In CTX group and intraperitoneal CDCA-Ver injection group, CTX (20 mg·kg-1 ) and CDCA-Ver (20 mg·kg-1 ) was intraperitoneally injected once daily, respectively. In intravenous CDCA-Ver injection group, CDCA-Ver ( 20 mg · kg-1 ) was injected through tail vein once daily. CDCA-Ver, CTX and NS were injected into the mice of the experimental groups once daily for 10 days, respectively. The dose volume was 0. 1 mL · ( 10 g )-1 body weight. The positive control drug was cyclophosphamide. Ten mice were treated with 20 mg · kg-1 CDCA-Ver through intravenous injection ( i. v. ) . Ten mice were treated with 20 mg·kg-1 CDCA-Ver through intraperitoneal injection. The thymus and spleen indices and the tumor inhibition rate were assessed, and histopathological examination with haematoxylin and eosin ( H&E) staining was carried out to evaluate the antitumor effects of CDCA-Ver. Results CDCA-Ver ( ivor ip) suppressed the growth of solid tumor in H22-bearing mice. The inhibition rate was 48. 3% at the dose of 20 mg·kg-1 CDCA-Ver (ip). There was no significant difference between CDCA-Ver (ip) and CTX treated group (P<0. 05). Compared with the control, the weight of thymus and spleen of CDCA-Ver (ip) treated group was not obviously changed. But a significant weight loss of thymus and spleen in CTX group was observed, which was attributed to the immune suppression from CTX. The thymus and spleen indices in the CTX-treated mice were significantly lower than those of the control group (P<0. 01). We further conducted histopathological examination to confirm the results. The immune system was not suppressed by CDCA-Ver ( ip ) in tumor-bearing animals. The low toxicity of CDCA-Ver was an outstanding advantage for the development of newly anticancer drug. Conclusion CDCA-Ver treatment can significantly inhibit tumor growth in mice.

Objective To obeserve the effects of acute hypervolemic hemodilution(AHH) with hypertonic .sodium chloride hydroxyethyl starch 40(HSH 40) on hemodynamics and fluid balance in patients under general anesthesia.Methods Fifty patients undergoing radical surgery for gastral cancer under general anesthesia were randomly divided into 2 groups with 25 patients each.Acute hypervolemic bemodilution (AHH) was performed with HSH 40 6 ml/kg in group A or with hydroxyethyl statch(HES) 6 ml/kg in group,which was infused within 30 minuts.HR,MAP,CVP were recorded before(T_0),at 30 min (T_1),60 min (T_2) after infusionand and the end of operation (T_3).The amounts of bleeding,HSH 40 and HES and urine output were recorded as well.Results There were no significant diferences in HR and MAP between two groups at all time points.CVP was sighificantly higher at T_1-T_3 than that at To in two groups.The urine output was more in groups A than that in group B(P<0.05).Conclusion AHH with HSH 40 can effectively expand blood vlume and increase urine output in surgical patients under general anesthesia.

Stem cells are crucial for embryonic development and in the maintenance of adult cellular homeostasis.Understanding the regulatory network of stem cells,including embryonic and adult stem cells,will allow us to learn the pathogenesis and possibly design novel approaches to treat many diseases (such as cancer and degeneration).The retinoblastoma (Rb) pathway controls cellular proliferation,differentiation and death.More and more evidences support an important role of Rb activity in the biology of stem and progenitor cells.Transiently inactivating Rb pathway might favor the expanding of functional stem cell populations,thus have values in the future stem cell applications.

Objective To observe the expressions of DNA methyltransferases (DNMTs) 1,3a and 3b in retinoblastoma (RB).Methods Sixty-two RB samples and six normal retinas were studied,including 17 poorly differentiated and 45 well differentiated samples; 16 invasive and 46 non-invasive samples.The expressions of DNMT1,3a,and 3b,and Ki-67 were detected using immunohistochemical analysis.Brown staining of nuclei was considered to represent the positive stain for DNMT1,3a and 3b,and ki-67,blue staining as negative.The level of high expression of nuclear staining was,positive cells in DNMT1≥65 ％,in DNMT3a≥60％ and in DNMT3b≥40％.The correlations of DNMT1,3a and 3b expression in RB samples,and MIB-1 labeling index were analyzed.Results Viewed under the light microscope,negative expressions of DNMT1,3a and 3b were demonstrated in normal retinas,however,positive expression was observed in RB samples,with 100％ in DNMT1,98％ in DNMT3a and 92％ in DNMT3b.Comparing well differentiated RB samples with poorly differentiated samples,significant differences were found in high expression of DNMT1 (x2 =12.57,P＜0.05) and DNMT3a (x2 =10.54,P＜0.05) ; also in the positive cells of DNMT1 (U=179,P＜0.05) and DNMT3a (U=198,P＜0.05).No significant difference was found comparing high expression (x2=1.5,P＞0.05) and positive cells (U=307,P＞0.05) of DNMT3b.When comparing invasive tumor tissues with non-invasive tumors,significant differences were shown between high expression (x2 =4.72,P＜0.05) and positive cells comparing DNMT1 (U=236,P＜0.05).No significant difference was shown in high expression (x2=3.53,0.84; P＞0.05) in DNMT3a and DNMT3b,or in comparison with positive cells (U=338,257; P＞0.05).The expression of DNMTs was positively correlated with the MIB-1 labeling index in RB tissues (R2=0.554,0.376,0.219; P＜0.05).Conclusion There are high expressions of DNMT1,3a,and 3b in RB.

Objective To observe the expression and relationship of high-mobility group A(HMGA) 1,HMGA2,MIB-1 labeling index (LI) and let-7 in retinoblastoma (RB).Methods Forty-four RB samples were studied,including 11 poorly differentiated samples,33 well-differentiated samples; eight invasive and 36 non-invasive samples.The expression of HMGA1,HMGA2 and MIB-1 LI in RB were analyzed by immunohistochemitry.The HMGA1,HMGA2 were scored on a scale of 0 to high expression.0: no expression ; low: 1％ - 10 ％ ; medium: 11 ％ - 50 ％ ; high: ＞50 ％.The MIB LI were scored on a scale of 0to high expression.O: no expression;low: 1％ - 40％;high: ＞ 40％.Semiquantitative reverse transcription-polymerase chain reaction was used to assay the let-7 expression level: ≥ 80％ showed no significantly decreased expression; 60％ - 79％ showed medium decrease in expression; ＜ 60％ highly decreased in expression.Results In 44 RB samples,there were 14 cases with no HMGA1 expression (32％),11 cases with low expression (25％),10 cases with medium expression (23％),and nine cases with high expression (20％).Expression level of HMGA1 was significantly higher in poorly differentiated RB than in well-differentiated RB (x2 =11.3,P＜0.01) ; however,no statistically significant difference was found between invasive tumors and noninvasive tumors (x2 -5.9,P＞0.05).There were 11 cases with no HMGA2 expression (25％),11 cases with low expression-(25％),nine cases with medium expression (20％),and 13 cases with high expression (30％).Expression level of HMGA2 was significantly higher in poorly differentiated and invasive RB than in well differentiated and noninvasive RB respectively (x2=20.9,8.7; P＜0.05).There were 4 cases with no MIB-1 LI expression (9％),18 cases with low expression (41％),and 22 cases with high expression (50％).Expression level of MIB1 LI was significantly higher in poorly differentiated RB than in well-differentiated RB (t=5.2,P＜0.05).Higher expression of MIB-1 LI was found in invasive tumors than in noninvasive tumors,with no significant difference (t=-1.1,P＞0.05).Twenty seven cases had no significantly decreased expression of let-7 (61％).There were eight cases with medium decreased expression (18％) and nine cases with highly decreased expression (21％).Correlation analyses revealed that MIB1 LI expression significantly correlated with HMGA1and HMGA2 proteins (r=0.327,0.602; P＜0.05).A significantly inverse correlation existed between let-7 expression and HMGA1,HMGA2 proteins and MIB-1LI respectively (r=-0.247,-0.310,-0.392; P＜0.05).Conclusions Overexpression of HMGA1,HMGA2 and MIB-1 LI and down regulation of let-7 were demonstrated in RB.Supplying let-7 to RB cells can possibly inhibit HMGA1 and HMGA2 expression.