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Survival rate of children with rhabdomyosarcoma and prognostic factors
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Khadijeh Arjmandi Rafsanjani, Parvaneh Vossough, Ali Bashardoust, Mohammad Faranoush |
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Background: Rhabdomyosarcoma is the most frequent tumor of soft tissue in children. The survival rate of the patients is significantly increased since the 1970s. This study was undertaken to evaluate the 5- and 10-year survival rates of patients with rhabdomyosarcoma in a single center in Iran.
Methods: A total of 77 children with rhabdomyosarcoma up to 15 years old who had been treated at the Hematology and Oncology Department in Ali Asghar Children Hospital from 1993 to 2003 were evaluated for their age and gender, as well as histology, stage and primary site of the tumor at diagnosis.
Results: The mean age of the patients was 6.58 years (SD=4.02, median 6 years). In this series, 46 patients (59.7%) were male and 31 (40.3%) female, and the mean survival time of patients was 8 years (95% CI: 8-9). The 5-year survival rate was the highest in patients with localized tumor, stage I and II (82.25% and 86.88% respectively). The survival rate of patients with embryonal tumor was 86.8%, in those with primary orbital tumor was 94%, and in those with genitourinary tumor was 85.71%. The 5- and 10-year overall survival rates were 79.54% and 77.92%, respectively.
Conclusions: Children with rhabdomyosarcoma of lower stage, embryonal histology, and orbital and genitourinary primary sites had a better survival rate. Poor prognosis was associated with metastasis of the tumor at the time of presentation, alveolar histology (48%), and tumor of the extremities (58%). In our study, only the stage of the tumor was significantly different in the variables (P=0.0077) because of the small number of patients. Children who survived the first 5 years after diagnosis were found to have an excellent survival rate.
Key words: rhabdomyosarcoma; survival rate; age; primary site; stage; pathology
World J Pediatr 2007;3(1):36-40 |
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[Abstract] [Full Text] [PDF]
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Construction of the right ventricular outflow tract in children with pulmonary atresia associated with ventricular septal defect
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Jing-Hao Zheng, Jin-Fen Liu, Zhi-Wei Xu, Zhao-Kang Su, Wen-Xiang Ding |
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Background: It is difficult to build up the right ventricular outflow tract for pediatric patients with pulmonary atresia complicated by ventricular septal defect (VSD). This study was undertaken to evaluate the surgical procedures for the reconstruction of the right ventricular outflow tract (RVOT) in infants and young children.
Methods: From June 1999 to December 2004, 81 patients with PA associated with VSD were treated by primary repair, palliative repair or staged repair. These patients were divided into 2 groups according to different surgical procedures. In group I (32 patients), 17 underwent biventricular primary repair and transannular patching with autologous pericardial flake. Group II (49 patients) underwent palliative repair including construction of the right ventricular outflow tract according to preoperative conditions. Fifteen patients underwent staged radical repair, during which transannular patching was performed with homovariant pericardial flake in 8 patients.
Results: Four patients (12.5%) died after primary radical repair. Three patients died (6.1%) after palliative repair. But no death occurred for stage II radical repair. Follow-up for 3 months to 2 years showed satisfactory results. The oxygen saturation (SatO2) was 79%-87% for the palliative repair group, and the McGoon index was increased from 0.4-0.7 to 1.1-1.6.
Conclusions: With the development of pulmonary artery and pulmonary artery confluence, RVOT construction by the palliative method includes transannular autologous pericardial patching, Gortex conduit, and pericardial rolling. By the radical procedure, the back wall of the traverse pulmonary artery trunk is anastomosed to the incision and homograft besides the transannular autologous pericardial patch.
Key words: congenital heart defect; cardiac surgical methods; pulmonary atresia; infants; young children |
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[Abstract] [Full Text] [PDF]
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Surgical treatment of mediastinal neoplasm in children
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Xi Zhan, Wen-Dong Wang, Jian-Jun Wang |
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Background: Mediastinal tumors in children comprise a heterogeneous group of lesions from a range of embryonic origins. They may present as benign cysts or malignant lesions. Mediastinal tumors in children and adolescents result in significant morbidity and mortality. Lymphoma is more common in the anterior mediastinum and neurogenic tumors are more common in the posterior mediastinum. Surgery is an important measure used in the diagnosis and treatment of such lesions. This study was to evaluate the strategy of surgical treatment of mediastinal tumors in children.
Methods: We retrospectively analyzed the clinical data from 61 children with mediastinal tumor treated in our department from 1994 to 2006.
Results: Forty-four patients had benign tumors (72.1%), and 17 malignant tumors (27.9%). Fifty patients were subjected to complete resection, 8 to partial resection, and 3 to biopsy. Thirty-nine patients were followed up for 3 years; the survival rate of patients with benign tumors was 100%, and that of patients with malignant tumors was 44%.
Conclusions: Thoracic computerized tomography (CT) scan is the first choice before operation; patients with benign mediastinal tumors may have good outcome after surgical treatment. Anaesthesia and perioperative management are of crucial importance.
Key words: mediastinal tumor; surgical treatment; children |
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[Abstract] [Full Text] [PDF]
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Separation and identification of endothelial progenitor cells from rat peripheral blood
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Si-Lin Pan, Quan-Sheng Xing, Long Sun |
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Background: Kawasaki disease (KD) as the most commonly acquired heart disease in children worldwide is an acute systemic febrile illness with endothelial injury. The incidence of KD varies in different countries, but it is higher in Asian than in Western countries. Endothelial progenitor cells (EPCs) can differentiate into endothelial cells and serve as a potential therapeutic agent for KD. The present study aimed at exploring the simple procedures of isolation, cultivation and purification of EPCs from peripheral blood.
Methods: Five milliliters of peripheral blood was collected from the femoral artery of each Sprague-Dawley rat. Mononuclear cells were isolated by Ficoll density gradient centrifugation and cultured in a special medium, including vascular endothelial growth factor and basic fibroblast growth factor. After 6 days of continuous culture on 24-well fibronectin-coated plates, the cells expanded and differentiated into endothelial-like progenitor cells. The expression of Flk-1, von Willebrand factor (vWF), CD31, and CD34 was assessed. Surface lectin staining was performed using fluorescently labeled UEA-1 lectin at 10 µg/ml. Meanwhile, the ability for live cells to take up fluorescently labeled acetylated low-density lipoprotein (DiI-Ac-LDL) was assessed by incubation with DiI-Ac-LDL.
Results: The cells harvested by this procedure were CD31, CD34, Flk-1 positive and demonstrated double-positive fluorescence for LDL, and lectin-UEA-1.
Conclusion: Relatively purified EPCs can be obtained by certain procedures of isolation and culture. For its differentiating potential, EPCs act as an important source of mature endothelial cells (ECs).
Key words: peripheral blood; mononuclear cell; endothelial progenitor cells; cell culture |
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[Abstract] [Full Text] [PDF]
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Insulin-like growth factors in lung development of neonatal rats and effect of dexamethasone andretinoic acid on their expression
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Han-Chu Liu, Li-Wen Chang, Zhi-Hui Rong, Hua-Ping Zhu, Qian-Sheng Zhang, Hong-Bing Chen, Wen-Bin Li |
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Background: The lung is prone to being attacked by intrinsic/extrinsic factors and lung injury is formed. With the increased survival rate of very low birth weight, extremely low birth weight infants, respiratory failure and mortality rate caused by lung development arrest and dysplasia have increased accordingly. This study was designed to investigate the role of insulin-like growth factors (IGFs) in lung development of neonatal rats and effect of dexamethasone (DEX) and retinoic acid (RA) on their expression.
Methods: Eighty timed pregnant Sprague-Dawley rats were randomly divided into 4 groups (n=20): control group (group A), DEX treatment group 1 (group B), DEX treatment group 2 (group C), and RA treatment group (group D). Groups A, B and D were injected subcutaneously with normal saline, intraperitoneally with DEX and RA, respectively on gestational day 18-20; group C was injected subcutaneously with DEX on postnatal day 1-3. Lung tissues were collected at the following time points: gestational day 18, 20, 21 and postnatal day 1, 3, 5, 7, 10, 14, 21 respectively for histopathological examination and expression level determinations of IGF-I, IGF-II polypeptides and mRNA.
Results: The highest expression level of IGF-I in groups A and D occurred on postnatal day 5-7. There was a positive correlation between IGF-I and alveolar development. The highest expression level of IGF-I in group B was observed on postnatal day 3. The expression level of IGF-I was much higher in group B than in group A from gestational day 18 to postnatal day 3 (P<0.01); but it was significantly lower at other time points (P<0.01). The expression level of IGF-I in group C was lower but it was higher in group D than that in group A at any time points (P<0.01). The peak level of IGF-II expression occurred on gestation day 18 and reduced to trace gradually. No differences were found among all groups in the level of IGF-II expression (P>0.05). RT-PCR showed that the trends of IGF-I, -II mRNA expression in the 4 groups were in parallel with those of their polypeptides.
Conclusions: IGF plays an important role in lung development of neonatal rats and there is a close link between DEX, RA and IGF-I, -II. DEX could restrain but RA could accelerate lung development by influencing the expression of IGF-I, -II.
Key words: dexamethasone; retinoic acid; lung development; insulin-like growth factor |
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[Abstract] [Full Text] [PDF]
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Long-term effects of recurrent seizures on learning, behavior and anxiety: an experimental study in rats
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Xiu-Yu Shi, Ji-Wen Wang, Ge-Fei Lei, Ruo-Peng Sun |
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Background: Animal models have suggested that seizures in the developing brain cause less macroscopic structural damage than seizures in adulthood but accumulating evidence shows that recurrent seizures in infants are associated with a high incidence of cognitive deficits. In this study, we determined the effects of recurrent neonatal seizures on cognitive tasks using a set of behavioral paradigms when the animals were in adulthood.
Methods: Recurrent seizures were induced by injecting rat pups with pilocarpine on postnatal day 2 (P2), P4 and P7. In adulthood (P52-P56), spatial learning was examined by the Morris water maze; activity level was assessed by an open field test; and anxiety was examined by the elevated plus maze.
Results: Compared with controls, rats with recurrent seizures had deficient spatial learning and a greater degree of anxiety.
Conclusions: Our findings indicate that recurrent seizures during the neonatal period result in life-long impairment of certain cognition. Key words: epilepsy; development; pilocarpine; long-term effects; behavioral testing; water maze |
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[Abstract] [Full Text] [PDF]
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