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.

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

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

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 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 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

The aim was to construct a prokaryotic expression plasmid encoding the fusion gene of single-chain variable fragment and monocyte chemotactic protein-3 (MCP-3). The cDNAs of immunoglobulin (Ig) VH and Ig VL were amplified by RT-PCR and assembled into the single-chain variable fragment (scFv) by recombinant PCR method. The cDNAs of Ig VH and Ig VL were connected by a (Gly4Ser)3 linker. Then, the fragments of scFv and MCP-3 were connected with a NDAQAPKS spacer, using recombinant PCR method again. The results indicated that the fusion gene of scFv-MCP-3 were constructed correctly and cloned into the prokaryotic expression plasmid successfully identified by sequencing and restriction endonucleases examination. Finally, the fusion protein was expressed in E coli DH5alpha under induction by arabinose. And the fusion protein was 65 kD and account for 30% of the total protein of the bacteria. In conclusion, a prokaryotic plasmid, encoding the fusion gene of single-chain variable fragment with MCP-3 and expressing idiotype protein vaccination against B cell lymphoma, was constructed correctly.
Amino Acid Sequence ; Animals ; Cancer Vaccines ; biosynthesis ; genetics ; immunology ; Chemokine CCL7 ; biosynthesis ; genetics ; immunology ; Genetic Vectors ; Humans ; Immunoglobulin Idiotypes ; immunology ; Immunoglobulin Variable Region ; biosynthesis ; genetics ; immunology ; Lymphoma, B-Cell ; immunology ; Mice ; Mice, Inbred BALB C ; Molecular Sequence Data ; Plasmids ; genetics ; Prokaryotic Cells ; metabolism ; Recombinant Fusion Proteins ; biosynthesis ; genetics ; immunology ; Vaccines, DNA ; biosynthesis ; genetics ; immunology

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

OBJECTIVETo study the alteration of thymus matrix lymphocyte generator (TSLP) and change of the Th factor in the course of disease development, and to analyze the curative effect of inhalation of Vitamin A (VA) with corticosteroid for the treatment of asthmatic pneumonia.

METHODSAsthmatic pneumonia models were prepared by challenging rats with inhalation of ovalbumin for 4 weeks, and rested for 1 week. The treatment with VA and corticosteroid inhalation for 1 week was followed. The rat thymus and lung specimen were examen by histochemical and immunofluorescence staining.

RESULTSAfter 4 - 5 weeks of stimulation, there were more TSLP-positive cells and alveolar macrophages (AM) found in thymus and lung tissue of asthmatic group, the cell proliferation in spleen and thymus was obvious, and blood Th factors elevated. The inflammation within the lung tissue aggravated gradually. In VA group, the expression of TSLP and Th2 factors were all lowered at the 4th week. The TSLP expression slightly increased at the 5th week, and the cell proliferation within T-cell zone of spleen and thymus was strong at first and weakened later. Alveolar microphages (AM) increased significantly and the inflammation in the lung subsided gradually at the 5th week. In the hormone group, TSLP and Th2 factors expression in both thymus and lung were decreased at the 5th week, while the cell proliferation in thymus and lung was gradually increased. The quantity of AM was decreased, whereas the inflammation of the lung was increased gradually at the 5th week.

CONCLUSIONDuring asthmatic period elevated TSLP expression was accompanied by Th2 type responses while VA and corticosteroid both suppressed TSLP and Th2 factors expression. VA alone promoted T lymphocyte proliferation as well as the antigen elimination function by AM, after ceasing the usage, the lung inflammation abated gradually. In contrast, after ceasing the use of corticosteroid, inflammation aggravated.

Administration, Inhalation ; Adrenal Cortex Hormones ; administration & dosage ; therapeutic use ; Animals ; Asthma ; complications ; Beclomethasone ; administration & dosage ; therapeutic use ; Cytokines ; metabolism ; Pneumonia ; drug therapy ; metabolism ; Rats ; Rats, Sprague-Dawley ; Vitamin A ; administration & dosage ; therapeutic use