Acute Disease ; Atrial Fibrillation ; diagnosis ; etiology ; therapy ; Child ; Child, Preschool ; Electrocardiography ; Humans ; Infant ; Myocarditis ; complications ; virology ; Virus Diseases ; complications

Forehead ; surgery ; Humans ; Nose ; surgery ; Rhinoplasty ; Surgical Flaps

OBJECTIVETo study the relationship between collgen (Col) IV, VEGF secreted by the tumor cells and vasculogenic mimicry (VM).

METHODS158 bi-phase differential malignant tumor specimens were alloted and made into tissue microarray. These tissue microarray sections were stained with CD31, periodic acid-Schiff (PAS) and Col IV. Subsequently, distributive trait of Col IV and the difference of VEGF expression were analyzed.

RESULTSThe basement membrane of VM was PAS and Col IV positive. The expression of VEGF in bi-phase differential malignant tumor with VM was less than that in those without VM. The difference of VEGF expression in malignant melanoma and alveolar rhabdomyosarcoma was significant (P < 0.05).

CONCLUSIONCollgen IV and periodic acid-Schiff positive material take part in constructing the basement membrane of vasculogenic mimicry. The difference of the VEGF expression proves that vasculogenic mimicry can sustain the tumor blood supply.

Collagen Type IV ; analysis ; Humans ; Immunohistochemistry ; Neoplasms ; blood supply ; chemistry ; pathology ; Periodic Acid-Schiff Reaction ; Vascular Endothelial Growth Factor A ; analysis

OBJECTIVETo investigate the expression of ghrelin and its receptor, growth hormone secretagogue receptor (GHS-R), in the hypothalamus and gastrointestinal tract in rats with chronic renal failure (CRF) and explore their relationship with the disorder of gastrointestinal tract motility.

METHODSSD rats were randomly divided into sham-operated group (n=8) and CRF group (n=16), and in the latter group, the rats were subjected to 5/6 nephrectomy to induce CRF. Real-time PCR and immunohistochemical staining were used to detect the distribution of mRNA and protein of ghrelin and GHS-R in the gastric fundus, duodenum, and hypothalamus.

RESULTSThe rats in the CRF group showed a significantly higher expression of ghrelin mRNA and protein in the gastric fundus but a lower expression in the hypothalamus than those in the sham-operated group (P<0.01), but the expression in the duodenum was similar between the two groups (P>0.05). The expression of GHS-R mRNA and protein in the gastric fundus was significantly higher in the CRF group than in the sham-operated group (P<0.01), while in the hypothalamus and duodenum, the expression was significantly lower in the CRF group (P<0.01).

CONCLUSIONThe different distribution patterns of ghrelin and GHS-R in the tissues may be an important pathological basis of gastrointestinal motility disorder in CRF.

Animals ; Gastrointestinal Tract ; metabolism ; Ghrelin ; genetics ; metabolism ; Hypothalamus ; metabolism ; Kidney Failure, Chronic ; metabolism ; Male ; RNA, Messenger ; genetics ; metabolism ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Receptors, Ghrelin ; genetics ; metabolism

OBJECTIVETo establish a method of SYT-SSX fusion gene detection by FISH and to explore its diagnostic value for synovial sarcoma.

METHODSThe presence of SYT-SSX fusion gene was determined by FISH using a tissue microarray containing 62 known synovial sarcomas, 60 non-synovial sarcomas and 133 equivocal synovial sarcomas. FISH results were compared with those of RT-PCR published previously.

RESULTSOverall, 96.9% (247/255) of the cases were successfully analyzed by FISH. The sensitivity of FISH for known synovial sarcomas was 96.7% (58/60), and the specificity for the non-synovial sarcoma was 100% (59/59). Moreover, SYT-SSX gene fusion was detected in 78.1% (100/128) of the equivocal synovial sarcomas. The concordance rate between FISH and RT-PCR was 83.6% (102/122) in those equivocal synovial sarcomas, and overall 79.7% (106/133) of these cases were confirmed as synovial sarcomas either by RT-PCR or by FISH.

CONCLUSIONSThe sensitivity and specificity of FISH detection of SYT-SSX fusion gene are high. FISH and RT-PCR are complementary to each other in the confirmation of synovial sarcomas, particularly those questionable cases.

Biomarkers, Tumor ; analysis ; genetics ; Humans ; In Situ Hybridization, Fluorescence ; methods ; Nucleic Acid Hybridization ; methods ; Oncogene Proteins, Fusion ; genetics ; isolation & purification ; Pathology, Molecular ; methods ; Reverse Transcriptase Polymerase Chain Reaction ; Sarcoma, Synovial ; diagnosis ; genetics ; Soft Tissue Neoplasms ; diagnosis ; genetics

OBJECTIVETo investigate the mode of angiogenesis between highly invasive malignant melanoma and poorly invasive malignant melanoma by immunohistochemistry and periodic acid-Schiff stain (PAS) and to discuss whether the tumor cells in highly invasive malignant melanoma carry vasculogenic mimicry through self-metamorphosis, thus acquiring blood supply to sustain their growth.

METHODSThirty cases of highly invasive malignant melanoma and 30 cases of poorly invasive malignant melanoma were retrieved and reprocessed as tissue microarray for further investigations. The tissue microarray sections were then stained with CD34 and PAS; and the positivity rates were compared.

RESULTSThere was a significant difference between CD34 and PAS staining in highly invasive malignant melanoma (P < 0.01). The difference was not statistically significant in poorly invasive malignant melanoma (P > 0.05).

CONCLUSIONVasculogenic mimicry exists in some cases of highly invasive malignant melanoma. It is possible that the tumor cells can acquire blood supply to sustain growth and metastasize via this mechanism.

Antigens, CD34 ; analysis ; Antigens, Neoplasm ; Humans ; Immunohistochemistry ; Keratins ; analysis ; Melanoma ; blood supply ; metabolism ; pathology ; Melanoma-Specific Antigens ; Neoplasm Proteins ; analysis ; Neovascularization, Pathologic ; metabolism ; pathology

OBJECTIVEThe beta-thalassemia major is a common hereditary hematology disease in southern China. The combination of blood transfusion and iron chelation is now the reference treatment. The allogeneic hematopoietic stem cell transplantation is the only curative therapy for beta-thalassemia major. In this study the investigators observed and evaluated the effects of umbilical cord blood transplantation (UCBT) for patients with beta-thalassemia major.

METHODSTwelve cases of beta-thalassemia major aged from 1.3 to 8.3 years (8 male and 4 female) received UCBT. Eleven of the twelve donors were siblings and one was unrelative. Eight patients received no antigen and four patients received two antigen disparate grafts. According to the Pesaro's classification for thalassemia, 10 patients were at grade I or II, and 2 were at grade III. The HLA-identical patients accepted the conditioning regimen consisting of busulfan, cyclophosphamide and antithymocyteglobulin. The HLA-mismatched patients accepted the conditioning regimen consisting of hypertransfusions, continuous iv desferrioxamine, hydroxyurea, fludarabine, busulfan, cyclophosphamide and antithymocyteglobulin. The harvest stem cells contained 3.63 - 16.0 x 10(7)/kg of nucleated cells, 0.11 - 1.03 x 10(6)/kg of CD(34)(+) cells and 0.17 - 1.18 x 10(5)/kg of colony-forming-unit-granulocyte macrophages. Cyclosporine alone or in combination with mycophenolate mofetil (MMF) was given for acute graft-versus-host disease (aGVHD) prophylaxis.

RESULTSOf the 12 patients, 10 were engrafted. Ten patients had neutrophil recovery (> 0.5 x 10(9)/L) and seven patients had platelet recovery (> 50 x 10(9)/L). The median time was 18.1 and 57.3 days, respectively. Seven patients had disease-free survival (DFS) at a median follow up of 23 months (range 4 - 63 months). Three patients had rejection and autologous hematopoitic reconstitution. Two patients were not engrafted. One patient acquired severe aplastic anemia, another patient died of severe infection. The incidences of grade I and grade II aGVHD were 60% (6/10) and 40% (4/10), respectively. There were no long-term complications in the disease free survivors.

CONCLUSIONSGrade I-II beta-thalassemia major patients receiving sibling UCBT had high DFS. UCBT is an effective way to treat beta-thalassemia major.

Child ; Child, Preschool ; Cord Blood Stem Cell Transplantation ; adverse effects ; Female ; Graft vs Host Disease ; epidemiology ; Hematopoiesis ; Humans ; Infant ; Male ; beta-Thalassemia ; mortality ; therapy

OBJECTIVETo assess the diagnostic values of immunohistochemistry and SYT-SSX fusion gene detection for synovial sarcoma.

METHODSBased on clinical features, histological and immunohistochemical profiles, 195 cases of tumors were divided into three diagnostic categories: definitive synovial sarcoma, probable synovial sarcoma and possible synovial sarcoma. RT-PCR Detection of the SYT-SSX fusion gene was performed using paraffin embedded tissue samples. Comparison between RT-PCR and immunohistochemistry results was carried out and their diagnostic value was evaluated.

RESULTSThere were 62 (31.8%) definite synovial sarcomas, 49 (25.1%) probable synovial sarcomas and 84 cases (43.1%) possible synovial sarcomas. SYT-SSX fusion gene was detected in 140 (78.2%) cases overall, including 94.7% (54/57) definite synovial sarcomas, 86.0% (37/43) probable synovial sarcomas and 62.0% (49/79) possible synovial sarcomas. In tumors in the certain and probable synovial sarcoma categories, the positive rates of epithelial membrane antigen (EMA) were significantly higher in the SYT-SSX positive cases than SYT-SSX-negative cases (P = 0.022, P = 0.010, respectively). EMA was positively correlated with the presence of SYT-SSX (r(s) = 0.431, P = 0.001, r(s) = 0.463, P = 0.002, respectively). However, such a correlation was not seen in cytokeratin (CK), vimentin or S-100 protein immunostains (P > 0.05). In tumors of possible synovial sarcoma category, there were no significant differences of CK, EMA, vimentin or S-100 protein between SYT-SSX-positive and SYT-SSX-negative tumors.

CONCLUSIONSSYT-SSX fusion gene detection is not needed when the conventional approaches are diagnostic. EMA positivity has a similar diagnostic value to that of SYT-SSX by RT-PCR for tumors in the probable synovial sarcoma category. However, detection of SYT-SSX is very important for diagnosis of the tumors in the category of possible synovial sarcoma.

Adolescent ; Adult ; Aged ; Biomarkers, Tumor ; metabolism ; Child ; Child, Preschool ; Female ; Humans ; Immunohistochemistry ; Keratins ; metabolism ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Oncogene Proteins, Fusion ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; S100 Proteins ; metabolism ; Sarcoma, Synovial ; diagnosis ; metabolism ; pathology ; Soft Tissue Neoplasms ; diagnosis ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult

OBJECTIVEThe aim of this study was to analyze the efficacy and toxicity of RNCE regimen in the treatment of relapsed or refractory B cell non-Hodgkin's lymphoma (NHL).

METHODSFrom January 2000 to December 2005, 46 patients with relapsed or refractory B cell NHL were treated by RNCE regimen with or without radiotherapy for the involved field. The clinical characteristics, response, toxicity and long-term survival results were analyzed retrospectively.

RESULTSA total of 46 patients were eligible. The complete response rate of second-line therapy was 52.17% (24/46), and the overall response rate was 82.61% (38/46). The median follow-up duration in this series was 69 months (range:6 to 102 months). The overall 1, 3, 5-year survival rate was 74.8%, 48.3%, 40.1%, respectively, with a median survival time of 30.2 months (5 to 65 months), and median progression free survival time of 10.9 months (2 to 31 months). The major toxicities were myelosuppression, GI toxicity, fatigue, fever and alopecia.

CONCLUSIONOur data show that RNCE regimen treatment is effective and well tolerated in patients with relapsed or refractory B cell non-Hodgkin's lymphoma.

Adolescent ; Adult ; Aged ; Alopecia ; chemically induced ; Antibodies, Monoclonal, Murine-Derived ; adverse effects ; therapeutic use ; Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Cisplatin ; administration & dosage ; Disease-Free Survival ; Drug Resistance, Neoplasm ; Etoposide ; administration & dosage ; Fatigue ; chemically induced ; Female ; Follow-Up Studies ; Humans ; Leukopenia ; chemically induced ; Lymphoma, B-Cell ; drug therapy ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Remission Induction ; Retrospective Studies ; Rituximab ; Survival Rate ; Thrombocytopenia ; chemically induced ; Vinblastine ; administration & dosage ; analogs & derivatives ; Young Adult

OBJECTIVETo investigate the mutation of glucose-6-phosphatase gene (G6PC gene) in a patient with glycogen storage disease Ⅰa.

METHODSPCR was used to amplify all five exons of G6PC gene. The PCR products were directly sequenced to detect the mutations.

RESULTSA heterozygous 743G>A mutation was found in the patient and his mother, resulting in the substitution of glycine (G) by arginine (R) in codon 222(G222R) in the putative membrane-spanning domain in human G6Pase, but not in his father and his sister.

CONCLUSIONSG222R mutation in G6PC gene was first identified in a patient with glycogen storage disease Ⅰa in mainland China.

Child, Preschool ; Glucose-6-Phosphatase ; genetics ; Glycogen Storage Disease Type I ; genetics ; Humans ; Male ; Mutation ; Sequence Analysis, DNA