Objective To explore the liver pathology from live related liver transplatation(LRLT) of Wilson′s disease(WD) in children,and evaluatethe indication of LRLT.Methods The sample of this study,including the donater and patient,came from the LRLT. It was observed with HE,MASSON,Timm′s and Rubeanic staining.Results With HE and MASSON stainning,hepatocyte showed degeneration,fiber hyperplasic and false lobule formed;Timm′s and Rubeanic stainning showed typical black deposit of granules and conglomerations.Liver pathology revealed that liver cirrhosis appeared,and it was diffrentent from Child Pugh.Conclusions It is of great significance that the liver pathology is useful for the treatment of WD.If liver pathology supportes,the indication of LRLT can be measurably broadened.

Aged, 80 and over ; Humans ; Male ; Maxillary Sinus Neoplasms ; pathology ; Neoplasms, Muscle Tissue ; pathology

In this study, plate transparent circle by Davis method was introduce firstly screening ?-Rhamnosidase high-yield strain. The spore-sprouted Aspergillus niger 8-hour were mutagenized by ethyl methane sulphonate and pre-screened via transparent circle. 11% mutants yield 40% higher of ?-rhamnosidase than the original strain. A high-yield strain, T-226 with the highest ?-rhamnosidase activity of 373.4 U/mL was finally selected from these potential high-yield mutants after rescreened by shake flask fermentation twice. When the T-226 strain was fermented in 5 L bioreactor, the enzyme activity could reach to 631.9 U/mL after 84 h. Thus, the established screening method is highly efficient to isolate ?-rhamnosidase high-yield mutant of A. niger.

Objective To study whether hyperthermia and ionizing radiation could produce combined effects on the neurobehaviors of neonatal rats.Methods These rats who were pregnant for 8 or 9 days received respectively total body irradiation by 60Co-rays .When the rats were pregnant for 10 days,the temperature of rat anus was kept at (37?0.5) ℃,(41?0.5) ℃,(42?0.5) ℃,respectively in warming boxes and lasted for 2,3,4,5 minutes.The neurobehaviors of neonatal rats were observed.Results In contrast to control group and 37 ℃group ,when pregnant rats were exposed to high temperature.the neonate rats were delayed for the appearance of four physiologic markers,the age of acquisition for four reflexes and the time of learning and memory(P

OBJECTIVETo investigate the clinical features of Crohn disease according to the Montreal classification.

METHODSClinical data of 43 surgical patients with Crohn disease (surgical group) and 125 non-surgical patients with Crohn disease (non-surgical group) were retrospectively analyzed and compared between two groups. The Montreal classification was used.

RESULTSIn the surgical group, 28 patients (65.1%) were A2, 14 (32.6%) were A3 and only one was A1, which was not significantly different as compared to the non-surgery group. The proportions of L1, L2, L3, and L4 subtype in the surgical group were 41.9%, 25.6%, 30.2%, and 2.3%, respectively, which was not significantly different as compared to that in the non-surgery group. In the surgical group,B1 disease was found in 1 case (2.3%), B2 in 26 cases (60.5%), and B3 in 16 cases (37.2%), while in the non-surgical group, B1 was found in 79 cases (63.2%), B2 in 44 cases (35.2%) and B3 in 2 cases (1.6%). Differences were significant between two groups in disease behavior (P=0.001, P=0.004, P=0.001).

CONCLUSIONSMost surgical patients of Crohn disease are A2. L1 and L3 are the main lesion location. As disease behavior, B2 and B3 are the main reasons for operation.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Crohn Disease ; classification ; pathology ; surgery ; Female ; Humans ; Male ; Middle Aged ; Reference Standards ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate the characteristics of the phenylalanine hydroxylase (PAH) gene mutations in patients with phenylketonuria (PKU) in Henan province, China, in order for providing basic information for clinical genetic counseling and prenatal diagnosis.

METHODSAll the exons and partial flanking introns of the PAH gene were detected by polymerase chain reaction (PCR) and bi-directional sequencing in 34 patients with PKU from Henan province.

RESULTSA total of 23 different disease-causing mutations were identified which corresponded to 92.65% (63/68) of the PAH alleles, including 12 missense mutations, 4 nonsense mutations, 4 splicing junction mutations, and 3 deletion mutations. Among them, A156P and P69_S70delinsP(delCTT) were novel mutations; IVS2+ 5G to C, G332E, IVS10-14C to G and L367 to Wfs were reported in Chinese population for the first time according to the PAH database (www.pahdb.mcgill.ca). Among all the 13 exons, exon 7 harbored the most type of mutations, exon 11 and exon 5 the second. The most common mutations included R243Q (17.65%, 12/68), V399V (11.76%, 8/68), IVS4-1G to A (8.82%, 6/68), R400T(7.35%, 5/68), Y166X(5.88%,4/68) and G247R(5.88%, 4/68). In addition, 9 other gene variations were found in this study.

CONCLUSIONThe mutation spectrum and frequency of the PAH gene of patients with phenylketonuria in Henan province were slightly different from those from other parts of China.

Asian Continental Ancestry Group ; genetics ; Base Sequence ; Child ; Child, Preschool ; China ; DNA Mutational Analysis ; Female ; Genetic Counseling ; Humans ; Infant ; Male ; Molecular Sequence Data ; Mutation ; genetics ; Phenylalanine Hydroxylase ; genetics ; Phenylketonurias ; diagnosis ; genetics ; Prenatal Diagnosis

OBJECTIVESTo construct small interfering (siRNA) Sox9 expression plasmid and transfer it into human chondrosarcoma cells HTB-94, and to check the mRNA and protein expression of Sox9 and cell growth and apoptosis of HTB-94 human chondrosarcoma cells.

METHODSsiRNA(Sox9) expression plasmid was designed and synthesized. And it was transferred into HTB-94 human chondrosarcoma cells. Then the expression of the mRNA and protein of Sox9, cell growth and apoptosis in transferred HTB-94 human chondrosarcoma cells were checked.

RESULTSThe recombinant plasmid was confirmed by enzyme digestion analysis and DNA sequencing. The expression of the mRNA and protein expression of Sox9 in transferred HTB-94 were significantly reduced. The cell growth of HTB-94 was inhibited, and the apoptosis of HTB-94 was remarkably increased.

CONCLUSIONsiRNA (Sox9) expression plasmid could be transferred into HTB-94 human chondrosarcoma cells. And it can reduce the mRNA and protein expression of the HTB-94, inhibit the cell growth and cause the apoptosis of the tumor cells.

Apoptosis ; Cell Proliferation ; Chondrosarcoma ; metabolism ; pathology ; Genetic Vectors ; Humans ; Plasmids ; genetics ; RNA, Messenger ; genetics ; RNA, Small Interfering ; genetics ; SOX9 Transcription Factor ; genetics ; metabolism ; Transfection ; Tumor Cells, Cultured

OBJECTIVEThe aim of this study was to investigate the incidence of nipple-areola complex (NAG) involvement in stage I - II a breast cancer patients who underwent skin-sparing mastectomy and to determine the associated risk factors, to provide a theoretical basis for modified radical mastectomy preserving NAC and breast reconstruction in early stage breast cancer patients.

METHODSA total of 68 women with primary breast cancer were included in this study. The following associated risk factors of NAC involvement were assessed and compared with those of non-involvement: the distance from the tumor site to the edge of areola (D), axillary lymph node status, over-expression of HER-2/neu, location of tumor, TNM stage, abnormal nipple (nipple indentation, erosion, discharge), tumor size, age, histological type, as well as status of estrogen receptor (ER) and progesterone receptor (PR), by Chi-square test.

RESULTSThe positive rate of NAG involvement was 13.2%. It decreased with an increase in the distance from the tumor site to the edge of the areola (D) (chi2 = 10.68, P <0.01)), and higher incidence of NAG involvement was found in patients with axillary lymph node metastasis (chi2 = 14. 61, P < 0.01) and over-expression of HER-2/neu (chi2 =6.83, P <0.01). Location of tumor (P <0.01), TNM stage (chi2 =3.85, P <0.05), abnormal nipple (chi2 = 11.65, P<0.01), and tumor size (chi2 =4.13, P <0.05) also had influence on the NAG involvement. No significant correlation between NAC involvement and age (P > 0.05)), histological type (chi2 = 0.07, P > 0.05)), as well as status of estrogen receptor (ER) (chi2 = 0.06, P > 0.05) and progesterone receptor (PR) (chi2 = 0.04, P > 0.05) was found. Most of the NAG involvement was caused by ductal infiltration.

CONCLUSIONIn the stage I - II a breast cancer patients, location of tumor, TNM stage, the distance from the tumor site to the edge of areola (D), abnormal nipple, over-expression of HER-2 and metastases in axillary lymph nodes are the primary influential factors of NAG involvement.

Adult ; Aged ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Carcinoma, Ductal, Breast ; metabolism ; pathology ; surgery ; Carcinoma, Intraductal, Noninfiltrating ; metabolism ; pathology ; surgery ; Female ; Humans ; Lymphatic Metastasis ; Mammaplasty ; Mastectomy, Modified Radical ; Middle Aged ; Neoplasm Invasiveness ; Neoplasm Staging ; Nipples ; pathology ; surgery ; Receptor, ErbB-2 ; metabolism ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Tumor Burden

Objective To explore the influence of full-time nurses on service of pre-hospital emergency care.Methods There were 978 emergency cases between March and May in 2011 as control group received the pre-hospital emergency care by nurses form emergency department,and 1 023 energancy cases between 7and September in 2011 as experimental group received the pre-hospital emergency care by full-time nurses.The activation times,the test of theoretical knowledge and operating skills of nurses were compared before and after the application of pre-hospital full-time nurses.Results After the application of pre-hospital full-time nurses,the activation times decreased and the rate of reach the standard increase from 99.3％ to 100.0％,there was a significant difference between before and after (x2 =5.44,P ＜ 0.05).The nurses' knowledge of special and operating skill were enhanced after the application,significant difference was found (P ＜ 0.05),while no significant difference was found in the operation test results of defibrillator and portable breathing machine (P ＞ 0.05).Conclusions Applying full-time nurses model can effectively decrease the activation times and all the activation times can reach the standard,and it also can effectively improve the nurses' knowledge of special and operating skill.

OBJECTIVETo observe the effect of orthotopic liver transplantation (OLT) on hepatopulmonary syndrome (HPS) and investigate risk factors predicting the prognosis of OLT.

METHODSTwenty-six cases of HPS and 30 cases of non-HPS were analyzed treated from April 2004 to January 2006. Survival rates after OLT were compared and risk factors predicting the prognosis of OLT in HPS were researched by univariant and COX analysis.

RESULTSThe 28 days survival rate in HPS after OLT was 76.9% (20/26), half a year survival rate and one year survival rate were both 61.5% (16/26). Whereas the one year survival rate of patients without HPS was 100%(P < 0.05). By univariant analysis, shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs, PaO2 and PaO2/FiO2 in room air before operation were relative to the prognosis of peri-operative period and half a year outcome after OLT in HPS (P < 0.05). Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs (OR = 1.182, P = 0.001), and mechanical ventilation time (OR = 1.003, P = 0.053) after OLT were independent risk factors predicting the prognosis of OLT in HPS by COX analysis. Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs > or = 28.4%, or PaO2 < or = 56 mm Hg (1 mm Hg = 0.133 kPa) before OLT predicted the poor outcome of OLT in HPS. The sensitivity were 83.3% and 85.0% respectively, and the specificity were 95.0% and 83.3% respectively.

CONCLUSIONSOLT is an effective treatment for HPS.Shunt of (99)Tc(m)-labeled macro-aggregated albumin in lungs before OLT and mechanical ventilation time after OLT were independent risk factors for the prognosis of OLT in HPS.

Adult ; Aged ; Female ; Follow-Up Studies ; Hepatopulmonary Syndrome ; surgery ; Humans ; Liver Transplantation ; Male ; Middle Aged ; Prognosis ; Risk Factors ; Survival Analysis ; Treatment Outcome