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Molecular and epidemiologic characterization of wilms tumor from Baghdad, Iraq

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World Journal of Pediatrics 2018年第6期14卷 585-593页

作者： Hannah M.Phelps Mazin F.Al-Jadiry Natasha M.Corbitt Janene M.Pierce Bingshan Li Qiang Wei Raina R.Flores Hernan Correa Stefania Uccini Haydar Frangoul Adel R.Alsaadawi Safaa A.F.Al-Badri Amir F.Al-Darraji Raghad M.Al-Saeed Salma A.Al-Hadad Harold N.LovvornⅢ Vanderbilt University School of Medicine 2215 Garland AvenueNashvilleTN 37232-9780USA Oncology Unit Children’s Welfare Teaching HospitalBaghdad University Medical CityBaghdadIraq Department of P ediatric Surgery Vanderbilt University Medical CenterNashvilleUSA Department of Molecular Physiology and Bioph ysics Vanderbilt University School of MedicineNashvilleUSA Division of P ediatric Pathology Vanderbilt University Medical CenterNashvilleUSA Department of Clinical and Molecular Medicine Sapienza UniversityRomeItaly Division of P ediatric Hematology and Oncology Vanderbilt University Medical CenterNashvilleUSA Department of P athology Baghdad University Medical CityBaghdadIraq Oncology Unit Children’s Welfare Teaching HospitalWasit University College of MedicineWasitIraq

Background wilms tumor (WT) is the most common childhood kidney cancer worldwide, yet its incidence and clinical behavior vary according to race and access to adequate healthcare resources. To guide and streamline therapy in the war-torn and resource-constrained city of Baghdad, Iraq, we conducted a first-ever molecular analysis of 20 WT specimens to characterize the biological features of this lethal disease within this challenged population. Methods Next-generation sequencing of ten target genes associated with WT development and treatment resistance (WT1, CTNNB1, WTX, IGF2, CITED1, SIX2, p53, N-MYC, CRABP2, and TOP2A) was completed. Immunohistochemistry was performed for 6 marker proteins of WT (WT1, CTNNB1, NCAM, CITED1, SIX2, and p53). Patient outcomes were compiled. Results Mutations were detected in previously described WT 'hot spots' (e.g., WT1 and CTNNB1) as well as novel loci that may be unique to the Iraqi population. Immunohistochemistry showed expression domains most typical of blastemal-predominant WT. Remarkably, despite the challenges facing families and care providers, only one child, with combined WT1 and CTNNB1 mutations, was confirmed dead from disease. Median clinical follow-up was 40.5 months (range 6–78 months). Conclusions These data suggest that WT biology within a population of Iraqi children manifests features both similar to and unique from disease variants in other regions of the world. These observations will help to risk stratify WT patients living in this difficult environment to more or less intensive therapies and to focus treatment on cell-specific targets.

关键词： Iraq Low- and middle-income countries Next-generation sequencing Pediatric cancer wilms tumor

METTL1 gene polymorphisms and wilms tumor susceptibility in Chinese children:A five-center case-control study

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Chinese Medical Journal 2023年第14期136卷 1750-1752页

作者： Linqing Deng Ruixi Hua Zhengtao Zhang Jinhong Zhu Jiao Zhang Jiwen Cheng Suhong Li Haixia Zhou Guochang Liu Jing He Wen Fu Department of Pediatric Surgery Guangzhou Institute of PediatricsGuangdong Provincial Key Laboratory of Research in Structural Birth Defect DiseaseGuangzhou Women and Children’s Medical CenterGuangzhou Medical UniversityGuangzhouGuangdong 510623China Department of Clinical Laboratory BiobankHarbin Medical University Cancer HospitalHarbinHeilongjiang 150040China Department of Pediatric Surgery The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan 450052China Department of Pediatric Surgery The Second Affiliated Hospital of Xi’an Jiaotong UniversityXi’anShaanxi 710004China Department of Pathology Children Hospital and Women Health Center of ShanxiTaiyuanShanxi 030013China Department of Haematology The Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical UniversityWenzhouZhejiang 325027China

To the Editor:wilms tumor,or nephroblastoma,is the most frequently diagnosed pediatric kidney cancer,accounting for>90%of all renal tumors in children 1–7 years old,and the disease may be related to genetic factors.^([1])As an emerging research spot,the epitranscriptome has been implicated in different RNA modifications in human diseases,including cancers.[2]Methyltransferaselike 1(METTL1),a critical RNA N7-methylguanosine(m7G)methyltransferase,can catalyze the formation of a m7G modification(mostly at nucleotide position 46 in the variable region of transfer RNAs[tRNAs])by complexing with WD repeat domain 4(WDR4).Depending on the different positions,the tRNA m7G modifications have various functions.

关键词： wilms diseases kidney

Expression of proliferating cell nuclear antigen in wilms’tumor

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Chinese Medical Journal 1997年第5期 32-33页

作者：陈方刘国华张忠德李衷初蔡威

Objective To investigate the expression of proliferating cell nuclear antigen (PCNA) and its clinical significance in wilms’ tumor. Methods PCNA immunoreactivity on paraffin embedded tissues was assessed retrospectively in 35 cases using labelled streptaridin biotin (LSAB) procedure, and its association with clinical and other biological features was studied. Results Twelve tumors (34.29%) expressed PCNA. Expression rate increased with the advance of stage (Pwilms’ tumor.

关键词：刘国华 proliferating cell nuclear antigen wilms tumor 蔡威 Expression

wilms tumor with dilated cardiomyopathy: A case report

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World Journal of Clinical Oncology 2019年第8期10卷 293-299页

作者： Saviga Sethasathien Chane Choed-Amphai Kwannapas Saengsin Lalita Sathitsamitphong Pimlak Charoenkwan Kanokkan Tepmalai Suchaya Silvilairat Division of Cardiology Department of PediatricsFaculty of MedicineChiang Mai UniversityChiang Mai 50200Thailand Division of Hematology and Oncology Department of PediatricsFaculty of MedicineChiang Mai UniversityChiang Mai 50200Thailand Division of Pediatric Surgery Department of SurgeryFaculty of MedicineChiang Mai UniversityChiang Mai 50200Thailand

BACKGROUND wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the wilms tumor. CASE SUMMARY A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. CONCLUSION Abdominal mass, dilated cardiomyopathy and hypertension can indicate wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.

关键词： Dilated cardiomyopathy Heart failure Hypertension wilms tumor Case report

Survival Outcome of wilms Tumor with Multi-Modality Treatment at Jimma Hospital, Southwest Ethiopia

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Open Journal of Urology 2023年第6期13卷 185-193页

作者： Melese Birara Gashaw Messele Gersam Abera Diriba Fufa Department of Surgery University of Gondar Hospital Gondar Ethiopia

Background: wilms’ tumor (WT), the most common malignant neoplasm of the urinary tract of children [1], accounts for 5.9% of childhood cancers and affects one in every 10,000 children worldwide before the age of 15 years. The care of children with Wilm’s tumor in sub-Saharan Africa is compromised due to resource deficiencies that range from inadequate healthcare budgets to paucity of appropriately trained personnel. Childhood wilms tumor is surging as an important paediatric problem in developing and sub-Saharan Africa countries. The objective of the study is to establish an understanding on the treatment challenges and outcomes of Wilm’s tumor in South West Ethiopia. Results: Forty-three Wilm’s tumor patients who were admitted from January 2017 to December 2021 were included in the study. The most frequent presentation was painless abdominal swelling in 40 (93%) patients. Fourteen patients (32.6%) were hypertensive at the time of diagnosis and the other 13 (30.2%) were normal. In abdominal examination, 31 (72.1%) patients had abdominal mass not crossing the midline and 12 (27.9%) had mass crossing the midline. After multimodal treatment, 37.5% had improvement, 11.6% came back with relapse. Most patients (41.7%) abandoned treatment and 9.3% of the cohort died in the course of treatment. Conclusion: The outcomes in the treatment of wilms Tumor have been found to be poor in this review. The main reason for poor outcome has been not receiving adequate chemotherapy after surgery. Doses of chemotherapy received after surgery significantly affected treatment outcomes (p = 0.026).

关键词： wilms Survival Treatment Childhood Tumor

Prognostic Impact of HER/2 Expression on Survival of Preoperatively Treated Children with wilms Tumor at South Egypt

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Journal of Cancer Therapy 2017年第9期8卷 801-813页

作者： Heba A. Sayed Abeer Refaiy Mohammed A. Salem Department of Pediatric Oncology South Egypt Cancer Institute Assiut University Assiut Egypt Surgical Oncology Department South Egypt Cancer Institute Assiut University Assiut Egypt Pathology Department Faculty of Medicine Assiut University Assiut Egypt

Aim: wilms tumor (WT) is the most frequent type of pediatric renal tumors. Her/2 is an oncoprotein, its over-expression revealed to play a very vital role in the progress and improvement of certain tumors. This study evaluates the possible role of Her/2 as a prognostic indicator in formerly treated WT. Method: Immunohistochemical expression of Her/2 was studied in paraffin material of 40 WT patients followed SIOP 9 protocol. Patients’ medical records reviewed for clinical, pathological and outcome data and correlated with HER2 expression. Additional 15 samples of normal surrounding kidney tissue specimens were included. Results: Her/2 was often expressed in normal kidney tissue (renal tubules but not glomeruli) and at variable levels in the three elements of WT. At a median of 84 months, 70% of patients are living and under follow-up, surgical stage and pathologic subtypes were the only two factors significantly affect the outcome of our patients (p = 0.000, p = 0.007 & p = 0.004, p = 0.005 for OS (Overall survival) and DFS (Disease Free survival) respectively). Her/2 expression was associated with epithelial differentiation (p wilms tumor. Non-conclusive results regarding influence of Her/2 expression on the result of WT patients were found.

关键词： wilms Tumor Her/2 Preoperative Chemotherapy

Interaction between the wilms tumour factor-1 element in the promoter of Amh and a downstream enhancer is required for a strong expression of the gene in pre-pubertal sertoli cells

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American Journal of Molecular Biology 2013年第3期3卷 165-172页

作者： David W. Dresser The Ashworth Laboratory The University of Edinburgh Edinburgh UK

Amh (anti-Müllerian hormone) is a single copy gene which is expressed strongly in Sertoli cells in the foetal testis and participates in the onset of sexual differentiation. Its promoter driving the expression of a reporter gene (d2EGFP) has been used to analyse the role of certain defined putative elements and a downstream enhancer element in gene expression. These experiments were carried out in vitro using a line of pre-pubertal mouse Sertoli cells, transienly transfected with circular DNA constructs with variously mutated promoter elements. A downstream enhancer element, situated immediately 3’ of the polyadenylation (PA) signal for Amh, has been inserted in an equivalent position in the d2EGFP construct. When the Amh promoter is unmodified, the downstream enhancer (DE) is positively associated with a large increase in EGFP expression. This is at least partly the consequence of an increased rate of expression by individual cells. Experiments using variously truncated Amh promoters indicate that an upstream region (-214 to -336) may play a minor role in facilitating enhancement. However mutation of the wilms tumour factor-1 element, situated between the tata box and the start of translation, results in an almost complete suppression of enhancement.

关键词： Mouse Cell Lines In Vitro Amh Promoter SMAT Pre-Pubertal Sertoli Downstream Enhancer wilms Tumour Factor Element

Identification of a constitutional mutation in the WT1 gene in Taiwan Residents patients with wilms tumor

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Advances in Bioscience and Biotechnology 2014年第3期5卷 230-234页

作者： Meng-Yao Lu Wen-Chung Wang Chiao-Wen Lin Alice Chang Yen-Chein Lai Department of Obstetrics and Gynecology Jen-Ai Hospital Taichung Taiwan Department of Pediatrics National Taiwan University Hospital Taipei Taiwan School of Medical Laboratory and Biotechnology Chung Shan Medical University Taichung Taiwan

The overall frequency of WT1 gene alterations in wilms tumor is still unclear in Taiwan. Here we conducted molecular genetic analysis of the WT1 gene in Taiwan Residents patients with wilms tumor. Polymerase chain reaction and direct sequencing were performed on DNA samples from blood and paraffin-embedded tumor specimens. A constitutional mutation in the WT1 gene was found in one DNA sample from peripheral blood lymphocytes. The remaining DNA samples from peripheral blood lymphocytes and paraffin-embedded tumor specimens were tested negative for both constitutional mutations and somatic mutations. Thus, mutations at other wilms tumor loci may play an important role in wilms tumor development.

关键词： wilms Tumor WT1 Tumor Suppressor Gene Nephroblastoma Denys-Drash Syndrome

Juvenile myelo-monocytic leukemia (JMML): No effect of granulocyte monocyte-colony stimulating factor (GM-CSF) on wilms Tumor gene (<i>WT</i>1) by nested Polymerase Chain Reaction (nPCR) and flow cytometry

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Advances in Bioscience and Biotechnology 2014年第2期5卷 155-159页

作者： Sana Khan Marie Olszewski Wei Huang Morris Kletzel Stem Cell Transplant Chimerism Laboratory Depts. of Hema-tology-Oncology Transplant Ann & Robert H. Lurie Children’s Hospital of Chicago Chicago USA Stem Cell Transplant Chimerism Laboratory Depts. of Hema-tology-Oncology Transplant Ann & Robert H. Lurie Children’s Hospital of Chicago Chicago USA Northwestern University Feinberg School of Medicine Chicago USA

This study was to determine whether GM-CSF induced WT1 gene expression and to establish an association with markers of proliferation CD71+CD34+ using nPCR and flow cytometry respectively, in samples obtained from 5 newly diagnosed JMML patients. Overtime (day 0 to day 14) there was an insignificant difference in WT1 gene expression and CD71+CD34+ in JMML samples when compared to peripheral blood of normal volunteers (n = 3). Our study suggests that there is a correlation between WT1 gene expression and cellular proliferation and that GMCSF in vitro does not create a significant difference in JMML samples.

关键词： Juvenile Myelo-Monocytic Leukemia wilms Tumor Nested PCR JMML WT1 GM-CSF nPCR