Objective To share the experience of surgical repair for cor triatriatum in chidren.Methods Between July 2008 and June 2013,24 children with cor triatriatum underwent surgical correction in Cardiac Center of Children's Hospital of Fudan University.The minimum age at the time of operation was 1 month,median age was 5 month;the minimum body weight at the time of operation was 3.5 kg,median body weight was 7.4 kg.Retrospectively analyzed their cardiac anatomy,clinical data,surgical procedures and follow-up data.Results Of all 24 patients,there were 4 patients diagnosed semplice cor triatriatum,and 20 patients diagnosed cor triatriatum associated with other malformation includes cardiac and non-cardiac issues.Procedures were performed on 22 patients to correct all cardiac defects in one stage.1 patient underwent surgical repair for cor triatriatum and Glenn procedure as well.1 patient,diagnosed as cor triatriatum and functional single ventricle S/P Glenn,underwent surgical repair for cor tiratriatum only.2 patients recovered from pulmonary artery hypertension crisis after operation.1 patient dead 17 days post procedure caused by respiratory failure.23 patients discharged from hospital.The longest follow-up was 73 months,at least 12 months after discharge.All alive patients were in New York Heart Association's function class Ⅰ-Ⅱ.Echo assessment revealed normal size of left atriums of almost all alive patients one year after discharge.Till April 2015,no postoperation intervention,no blood flow obstruction at level of pulmonary veins,left atrium or mitral valve.Conclusion Pediatric patients diagnosed cor tiratriatum sinister need intervention,no matter hemodynamic change occurs or not.Right time of surgery is important for successful treatment.

OBJECTIVETo explore the clinical symptom and effect of arthroscopic treatment of symptomatic anterior cruciate ligament (ACL) cysts of the knee.

METHODSClinical data from 12 symptomatic ACL cysts patients from January 2005 to December 2010 were retrospectively analyzed,including 8 males and 4 females,with an average age of (33.7±9.5) years old (ranged, 19 to 53 years old). The locations were the left knee in 5 cases and the right knee in 7 cases. The disease duration ranged from 3 to 48 months,with a mean of (15.8±13.2) months. All cysts were arthroscopically resected. Range of motion was measured preoperatively and postoperatively, and Lysholm scoring system was used to evaluate the knee function.

RESULTSAll the incisions healed by first intention, and no complications occurred. Twelve patients were followed up for an average of (32.3±6.6) months(ranged, 24 to 48 months). The symptoms of arthralgia,swelling and interlocking of the affected knees disappeared. There was no recurrence during the follow-up. There were significant differences in the range of motion and Lysholm score between pre-operation and post-operation.

CONCLUSIONArthroscopic surgery, showing its advantages of minimal invasion and rapid recovery,is an effective measure in the treatment of ACL cysts.

Adult ; Anterior Cruciate Ligament ; physiopathology ; surgery ; Arthroscopy ; methods ; Cysts ; physiopathology ; surgery ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Range of Motion, Articular

S-adenosylmethionine-dependent uroporphyrinogen III methyltransferase (SUMT) is a novel red fluorescence indicator. However, the production of SUMT in Escherichia coli is restricted by its relatively low solubility, and little is known about the red fluorescent materials that are associate with SUMT. Two individual SUMT mutations, L166A and L88R/L89G double mutant were produced by site-directed mutagenesis. Both mutants were overexpressed in E. coli and purified by Ni-NTA chromatography. The reddish mixtures isolated from the purified L88R/L89G double mutant were analyzed by UV-visible spectra scanning and mass analysis(MS). The L88R/L89G double mutant has enzymatic activity in vivo, whereas L166A mutant loses the activity. Trimethylpyrrocorphin is identified as the main constituent in the isolated pigments. The purified L88R/L89G mutant increases protein solubility, which is applied potentially as the fluorescent indicator denoting the solubility of protein fusion partner.
Amino Acid Substitution ; Electrophoresis, Polyacrylamide Gel ; Escherichia coli ; genetics ; Fluorescence ; Mass Spectrometry ; Methyltransferases ; chemistry ; genetics ; metabolism ; Molecular Weight ; Mutagenesis, Site-Directed ; methods ; Mutation ; Pigments, Biological ; chemistry ; metabolism ; Plant Proteins ; chemistry ; genetics ; metabolism ; Recombinant Proteins ; chemistry ; metabolism ; Solubility ; Spectrophotometry, Ultraviolet ; Zea mays ; enzymology ; genetics

The dynamic changes of germination percentage, germination potential, thousand-seed weight, antioxidase activity in Desmodium styracifolium seeds with different storage time were tested, and electrical conductivity, contents of soluble sugar, soluble protein, starch in seed leach liquor were also determined in order to reveal the mechanism of seed deterioration. The results as the following. (1) The germination percentage, germination potential and thousand-seed weight of D. styracifolium seeds declined, while the seed coat color darkened with the extension of storage time. (2) The activities of superoxide dismutase (SOD) and peroxidase (POD) decreased with the prolongation of storage period. The SOD activity declined fastest in 1,095-1,185 d of storage, while the POD activity declined significantly in 365-395 d of storage. (3) The electrical conductivity and the contents of soluble sugar, starch in seed leach liquor increased, while the content of soluble protein declined with the extension of storage time. (4) Correlation analysis indicated that the germination percentage, germination potential and thousand-seed weight of D. styracifolium seeds have a significantly positive correlation with SOD and POD activity, while have a significantly negative correlation with the electrical conductivity, contents of soluble sugar and starch. It can be concluded that during the storage of D. styracifolium seeds, physiological and biochemical changes including decrease in antioxidase activity, rise in electrical conductivity, degradation effluent of soluble sugar and starch, degradation of soluble protein were the main factors leading to the seed deterioration.
Color ; Fabaceae ; chemistry ; enzymology ; growth & development ; metabolism ; Germination ; Peroxidases ; metabolism ; Plant Proteins ; metabolism ; Seeds ; chemistry ; enzymology ; growth & development ; metabolism ; Starch ; metabolism ; Superoxide Dismutase ; metabolism ; Time Factors

OBJECTIVETo explore whether the antibody subtypes against the extracellular 1-2 (EC1-2) epitopes of pemphigus vulgaris antigen (PVA) are related to the pathogenesis of PVA.

METHODSEC1-2 fusion protein, emulsified with complete Freund's adjuvant (CFA) or aluminum hydroxide hydrate [Al ( OH)3], was used to immunize C57BL/6 mouse. After immunization, the cytokine types, specific antibody titers, and antibody subtypes were detected. Also, a neonatal mice model was used to evaluate the pathogenesis of different antibodies.

RESULTSTh1 type cytokine interferon gamma (IFNgamma) was elevated in CFA group, while Th 2 type cytokine interleukin-4 (IL-4) was increased in Al (OH)3 group. The antibody subtypes were different in both groups. After the two groups were transferred with antisera separately, the neonatal mice developed erosion on the skin from Al(OH)3 group, with acantholysis histopathologically and bright immuno-fluorescence deposition, which was not seen in CFA group.

CONCLUSIONDifferent antibody subtypes may contribute to the pathogenesis of disease.

Animals ; Antibody Specificity ; Autoantibodies ; immunology ; Desmoglein 3 ; immunology ; Epitopes ; immunology ; Mice ; Mice, Inbred C57BL ; Pemphigus ; immunology ; pathology

Pten gene is the first antioncogene with dual phosphatase activity discovered so far, pten gene regulates the cell cycle progress, apoptosis, metastasis and invasion of the tumor cells through negatively regulating the multiple signaling transduction pathways. Multiple myeloma (MM) is a malignant tumor occurring in terminal stage of B cell differentiation. The genetic changes are considered as the important factors in MM pathogenesis, among which the deletion of antioncogene is a critical genetic change. However, little is known about the genetic change of pten in MM. This review summarizes the research advance on pten in MM including structure of pten, mechanism of pten effect and correlation of pten with MM in order to provide some references for the investigating new gene target to treat the MM.
Humans ; Multiple Myeloma ; metabolism ; therapy ; PTEN Phosphohydrolase ; genetics ; metabolism ; Signal Transduction

BACKGROUNDSnake venom contains a number of components with different pharmacological and biological activities, especially in cancer therapy, and has increasingly become a research focus. This study was designed to isolate and purify a novel anti-clotting protein component from the venom of Agkistrodon acutus, and to explore its physico-chemical properties and biological activity.

METHODSThe venom of Agkistrodon was isolated and purified by ion-exchange chromatography on diethylaminoethyl (DEAE)-Sepharose Fast Flow, molecular sieve filtration through Sephadex G75, SP-Sepharose Fast Flow and molecular sieve filtration through Sephadex G50. We detected the activated partial thromboplastin time (APTT) of the eluant to select the anti-clotting protein component of interest. The molecular weight was determined by sodium dodecyl sulfate-polyacrylamid gel electrphoresis (SDS-PAGE) and liquid chromatography. Its protein content was detected by bicinchoninic acid (BCA).

RESULTSSDS-PAGE vertical gel electrophoresis showed that the anticoagulant factor is a tripolymer composed of three proteins whose molecular weights are 25 KDa, 30 KDa and 50 KDa. The factor contains about 65% percent protein.

CONCLUSIONSA novel anti-clotting protein component was purified by ion-exchange chromatography and molecular sieve filtration from the venom of Agkistrodon acutus and was found to be composed of three kinds of proteins.

Agkistrodon ; metabolism ; Animals ; Anticoagulants ; chemistry ; isolation & purification ; Chromatography, High Pressure Liquid ; Chromatography, Ion Exchange ; Crotalid Venoms ; chemistry ; Electrophoresis, Polyacrylamide Gel ; Proteins ; chemistry ; isolation & purification

OBJECTIVETo analyze and summarize the clinical and pathological characteristics of intracranial haemangioblastoma and to improve surgery effect.

METHODSeventy-two patients with intracranial haemangioblastoma who were proven by operation and pathology from 1970 to 1988 were analyzed retrospectively.

RESULTSIntracranial haemangioblastoma tends to occur in the hemisphere of cerebellum (83 tumours, 87%) and the age of them ranged from 20 to 40 years (47 cases, 58.3%) mostly. The ratio of men (46 cases) was higher than women (26 cases). The diagnosis of the disease depends on CT and MR substantive haemangioblastoma. The most effective and reliable treatment of intracranial haemangioblastoma is surgical resection.

CONCLUSIONIntracranial haemangioblastoma is benign tumour which can be, cured by total surgical resection. The key recurrence factors include the young age of initial onset, mistaken exploration and incomplete extirpation of tumour.

Adolescent ; Adult ; Brain Neoplasms ; pathology ; surgery ; Child ; Female ; Hemangioblastoma ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVETo evaluate the feasibility and efficacy of transcatheter closure of paravalvular leak (PVL) with Chinese-made occluder.

METHODSFive PVL patients were involved in this study, 2 out of the 5 patients underwent aortic mechanical valve replacements, 2 underwent mitral bioprosthetic valve replacements, and the remaining 1 underwent double mechanical valve replacement.Left ventricular end diastolic diameter, left atrial diameter and the systolic pulmonary artery pressure were assessed by echocardiography before and post the procedure.

RESULTSComplete occlusion without residual regurgitation was achieved in 2 patients with aortic PVL, for the 3 patients with mitral PVL, there was only tiny or mild mitral paraprosthetic leak remained post closure procedure. Cardiac perforation and pericardium tamponade occurred in 1 patient with aortic PVL during interventional closure and the patient recovered post emergent pericardiocentesis. Transient severe hemolysis and hemoglobinuria occurred in 3 patients with mitral PVL post closure procedure and they recovered after 1 to 3 weeks conservative therapy. During 3 months follow up, left ventricular end diastolic diameter [(52.2 ± 6.8) mm vs. (61.1 ± 7.2) mm, P < 0.05], the systolic pulmonary artery pressure [(40.0 ± 5.4) mm Hg (1 mm Hg = 0.133 kPa) vs. (57.0 ± 3.6) mm Hg, P < 0.05] and left atrial diameter of mitral PVL patient [(49.0 ± 4.3) mm vs. (56.0 ± 6.3) mm, P < 0.05] were significantly reduced compared to before closure procedure.

CONCLUSIONPercutaneous or transapical left ventricular access closure of PVL is feasible, effective and relative safe in selected patients.

Adult ; Aged ; Aged, 80 and over ; Aortic Valve ; surgery ; Cardiac Catheterization ; methods ; Contraindications ; Female ; Heart Valve Prosthesis ; Heart Valve Prosthesis Implantation ; adverse effects ; Humans ; Male ; Middle Aged ; Mitral Valve ; surgery ; Postoperative Complications ; surgery ; Retrospective Studies

OBJECTIVETo develop a novel method for preparing decellularized liver biological scaffold (DLBS) for liver tissue engineering.

METHODSDLBS was prepared by treatment of rat livers with detergent and enzymatic cell extraction and observed under optical and scanning electron microscopes. To assess the biocompatibility of the product, C3A cells and bone marrow-derived mesenchymal cells (BM-MSCs) were cocultured with DLBS as the scaffold, and the effect of DLBS on the proliferation of C3A cells was evaluated by MTT assay. DLBS was also implanted under the dorsal skin of SD rats to evaluate the tissue biocompatibility of this material.

RESULTSApplication of the detergent and enzymatic extraction allowed full extraction of the cells in the liver, leaving an extracellular matrix scaffold composed mainly of collagen and elastic fibrin. The coculture experiment showed that C3A cells and BM-MSCs could grow on and adhere to DLBS. The result of MTT assay showed that DLBS could promote the proliferation of C3A cells.

CONCLUSIONThis cell-free DLBS, which retains intact extracellular matrix and promotes cell attachment, proliferation, growth and differentiation, can be an ideal biological matrix scaffold material.

Animals ; Biocompatible Materials ; Cells, Cultured ; Coculture Techniques ; Extracellular Matrix ; chemistry ; Female ; Liver ; cytology ; Male ; Mesenchymal Stromal Cells ; metabolism ; physiology ; Rats ; Rats, Sprague-Dawley ; Tissue Engineering ; methods ; Tissue Scaffolds ; chemistry