Abstract:
:According to the Fifth National Wilms Tumor Study (NWTS-5), tumor-specific loss of heterozygosity (LOH) for chromosomes 1p and 16q identifies a subset of patients with Wilms tumor (WT) who despite having favorable histology (FH) have a significantly increased risk of relapse and death. We aimed to find out 1p and 16q LOH frequencies in patients with FH-WT as well as its correlation to survival outcome and epidemiologic and clinical characteristics. Data of patients with FH-WT presenting to the National Cancer Institute, Egypt, were retrospectively analyzed. Paraffin blocks were tested for 1p and 16q LOH using polymorphic loci that span the minimal regions of LOH at this area. The study included 100 patients with a median age of 5 years. Thirty-nine patients (39%) showed LOH at 1p (n = 14), 16q (n = 13), or both (n = 12). LOH was most frequently encountered in patients above 10 years (5/5), advanced stages disease (80% of stage V and 50% of stages IV and III each). The 3-year overall survival (OS) and event-free survival (EFS) were significantly lower in patients with double LOH (75% and 50%, respectively), followed by 16q (92% and 54%), in comparison with 1p (93% each) and negative LOH (97% and 100%) cases, respectively (p = 0.001). Combined LOH (1p+16q), followed by 16q LOH alone, was predictive of poorer outcome and was associated with lower OS and EFS in patients with FH-WT. Our results showed a higher-risk disease that would suggest the need for an intensified upfront therapy in this group of patients.
journal_name
Pediatr Hematol Oncoljournal_title
Pediatric hematology and oncologyauthors
Fawzy M,Bahanassy A,Samir A,Hafez Hdoi
10.3109/08880018.2015.1071902subject
Has Abstractpub_date
2015-01-01 00:00:00pages
548-56issue
8eissn
0888-0018issn
1521-0669journal_volume
32pub_type
杂志文章abstract::Three cases of vitamin B12 deficiency that occurred during infancy are presented. These cases appeared to be the result of pre-existing maternal deficiency. All three infants demonstrated evidence of neurodevelopmental delay at presentation, and one had sustained loss of milestones and developed involuntary motor move...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018909034282
更新日期:1989-01-01 00:00:00
abstract::Outlining the treatment for an unclassifiable lymphoid malignancy is often difficult. A highly undifferentiated lymphomatous mass that relapsed in spite of intense chemotherapy and autologous transplant is reported. At relapse, there was differentiation into myeloid lineage. Though remission was achieved with AML-type...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010902754883
更新日期:2009-03-01 00:00:00
abstract::The development of myeloid leukemias and myelodysplastic syndrome (MDS) is common in children with trisomy 8 mosaicism. However, the mechanisms by which the presence of an additional chromosome 8 translates to an increased risk of leukemias and MDS is currently unknown. The authors describe the analysis of stromal cel...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010490276917
更新日期:2004-04-01 00:00:00
abstract::We present the management challenge provided by a patient with kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon. A female child presented at 14 months of age with an ecchymotic swelling of her right upper arm and axilla. Subsequently, she developed profound thrombocytopenia and hypofibrinoge...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019809014013
更新日期:1998-07-01 00:00:00
abstract::It is well known in the literature that cancer creates educationally related barriers for all children, which may or may not contribute to clinically relevant school problems. The goal of this study was to obtain a base rate for the characteristic pattern of school functioning for children with leukemia, so that the c...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019709030898
更新日期:1997-03-01 00:00:00
abstract::Increased susceptibility to infection is reported in patients with beta-thalassemia major due to toxic effect of iron on neutrophil functions and reticuloendothelial system dysfunction. This study investigated the association between the neutrophil apoptosis and frequency of infection episodes, desferrioxamine treatme...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/713842280
更新日期:2003-04-01 00:00:00
abstract::A recently isolated peptide hormone, hepcidin, is thought to be the principal regulator of iron homeostasis. Hepcidin acts by limiting intestinal iron absorption and promoting iron retention in reticuloendothelial cells. Its precursor peptide form is called pro-hepcidin. The aims of this study were to determine serum ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010600629213
更新日期:2006-06-01 00:00:00
abstract::Endothelial protein C receptor (EPCR) is primarily localized on the endothelial cells of large blood vessels and is very low or absent in the microvascular endothelium of most tissues. EPCR augments the thrombin/thrombomodulin-dependent activation of protein C by 5- to 20-fold. EPCR appears to be physiologically signi...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010701198811
更新日期:2007-04-01 00:00:00
abstract::An episode of transient encephalopathy after the first course of intravenous high-dose methotrexate (HD-MTX; 1000 mg/m2) was observed in a 4-year-old girl with acute lymphoblastic leukemia. The neurological abnormalities took place 5 days after HD-MTX therapy. She experienced complex partial seizure and left hemipares...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019209018331
更新日期:1992-04-01 00:00:00
abstract::Methotrexate (MTX) is an antimetabolite with a major role in the treatment of acute lymphoblastic leukaemia (ALL). The authors report the management of a 9-year-old boy who developed an anaphylactoid reaction to high-dose MTX infusion, after a first challenge with intrathecal administration, during induction therapy f...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010701885268
更新日期:2008-03-01 00:00:00
abstract::Although survival rates in childhood cancer have distinctly improved, pediatric cancer patients often experience various disease- and treatment-related side effects with long-term consequences. Despite current studies investigating inactivity and limitations in physical functioning and quality of life in pediatric can...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.776155
更新日期:2013-05-01 00:00:00
abstract:PURPOSE:To describe clinical and CT features of L-asparaginase-associated pancreatitis (L-AP) and to correlate CT grades with clinical parameters. METHODS:A total of 16 children (M:F = 9:7; mean age, 8.1 years) who developed L-AP after L-asparaginase (L-asp) treatment and underwent abdominal CT scan were included. We ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2014.918681
更新日期:2014-10-01 00:00:00
abstract::During the last 5 years massive chemotherapy and autologous bone marrow transplantation have been increasingly explored in the treatment of pediatric solid tumors, mainly for neuroblastoma, Ewing's sarcoma, rhabdomyosarcoma, Wilms' tumor, germ cell tumors, osteosarcoma, and retinoblastoma. Although the disease course ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019009034318
更新日期:1990-01-01 00:00:00
abstract::Ghosal-type hemato-diaphyseal dysplasia is a rare autosomal recessive disorder with distinctive diaphyseal and metaphyseal dysplasia of long bones and steroid-dependant anemia. The authors describe a 20-month-old girl who had had a severe transfusion-dependent anemia since late infancy and marked locomotion difficulti...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:
更新日期:2003-07-01 00:00:00
abstract::Factor XIII deficiency is a very rare bleeding disorder. We report here on the clinical outcome of a young child with intracranial bleeding due to factor XIII deficiency. Clinicians should bear in mind that severe factor XIII deficiency is associated with a significant risk of unexpected intracranial hemorrhage (ICH)....
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.798059
更新日期:2013-11-01 00:00:00
abstract:BACKGROUND:The authors report the experience at the Children's Hospital Los Angeles with brain tumors diagnosed before 6 months of age, describing the characteristics of the patients, their tumors, treatment strategies, and prognostic factors. METHODS:Thirty-three children who were identified between 1979 and 2005 wer...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880010903447342
更新日期:2010-03-01 00:00:00
abstract::Background: Preclinical studies show that TLR9 agonists can eradicate leukemia by induction of immune responses in vivo against AML and ALL. These studies demonstrated that TLR9 agonists induce an immediate NK response followed by adaptive T and B cells responses resulting in long term anti-leukemia immunity. Methods:...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880018.2019.1667461
更新日期:2019-11-01 00:00:00
abstract::A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinica...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880018909014579
更新日期:1989-01-01 00:00:00
abstract::Eighty-six patients with Ewing's sarcoma were analyzed as to the role of radiation therapy. Fifty-eight patients (P group) had removal of the involved part of the bone after preoperative chemotherapy, and 28 (NP group) had local treatment either at the same time or before chemotherapy. Thirty-six of 58 P-group patient...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018609031206
更新日期:1986-01-01 00:00:00
abstract::A 9-year-old girl presented at the Pediatric Emergency Department with an acute onset of gastrointestinal symptoms due to hypercalcemia. Despite the absence of circulating blast, bone marrow biopsy was diagnostic of acute lymphoblastic leukemia. The hypercalcemia was initially treated with intravenous hydration and fu...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.1080/08880010490477248
更新日期:2004-09-01 00:00:00
abstract::A major problem of hemophilia A (HA) treatment is the development of factor VIII (FVIII) inhibitor, which usually occurs shortly after initiating replacement therapy. Several studies showed the correlation between inhibitor development and polymorphisms in inflammatory and immune response genes of HA patients; however...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.1080/08880018.2019.1585503
更新日期:2019-02-01 00:00:00
abstract:BACKGROUND:Alterations in the tumor suppressor gene TP53 have been associated with poor outcome in adult hematological malignancies. We have earlier reported an increased expression of the TP53 encoded protein p53, in bone marrow samples from pediatric patients with aggressive leukemia. Our aim was now to evaluate p53 ...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880018.2014.898723
更新日期:2014-05-01 00:00:00
abstract::Ten children receiving 5 to 6 week courses of radiotherapy after brain tumor surgery were given ondansetron treatment for persistent nausea and emesis. All patients continued the ondansetron treatment until the end of their radiotherapy course. Nausea, emesis, appetite, and adverse events were scored throughout the on...
journal_title:Pediatric hematology and oncology
pub_type: 临床试验,杂志文章
doi:10.3109/08880019609030823
更新日期:1996-05-01 00:00:00
abstract::A single institution's experience of three patients with nephroblastoma extending via the inferior vena cava to the right heart is described. The case reports and the pertinent literature illustrate that preoperative failure to detect the intracardial extension increases the risk of right outflow obstruction and pulmo...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,评审
doi:10.3109/08880019109028807
更新日期:1991-10-01 00:00:00
abstract::Since the mid-1960s intrathecal chemotherapy (methotrexate [MTX], cytarabine [Ara-C], or both, plus hydrocortisone) has constituted the standard approach to prophylaxis and treatment of central nervous system (CNS) leukemia and lymphoma. Intrathecal chemotherapy-related neurotoxicity has been described in a variable p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019509029588
更新日期:1995-07-01 00:00:00
abstract::A patient with well-differentiated monoblastic leukemia (ANLL FAB-M5b) is described in whom acute leukemia was diagnosed 25 months after having completed postoperative adjuvant chemotherapy for osteogenic sarcoma of the femur. All analyzed metaphases showed 48xy, dup 1(q12), +3, +9. ...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018809031250
更新日期:1988-01-01 00:00:00
abstract::Beta-thalassemia major is a severe, transfusion-dependent anemia that also causes infertility due to iron deposition to endocrine organs after overtransfusion. Very few pregnancies have been reported among such patients after modern therapies. In this study, 32 women with thalassemia who were admitted to Ali Asghar Ch...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章,多中心研究
doi:10.1080/08880010500313306
更新日期:2006-01-01 00:00:00
abstract::In 33 children, 23 with acute lymphoblastic leukemia (ALL) and 10 with solid tumors, the phenotype of the enzyme adenosine deaminase (ADA) was detected in the erythrocytes by electrophoresis in cellulose acetate. In all children the ADA enzyme activity was also determined in the plasma by spectrophotometry at the onse...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880019609030840
更新日期:1996-07-01 00:00:00
abstract::Hyperleukocytosis in patients with leukemia is associated with early mortality, especially due to the pulmonary and neurological complications of leukostasis. The prompt use of leukapheresis may improve patients' survival in the initial treatment period. The medical records of all previously untreated acute leukemia p...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:10.3109/08880018.2013.818747
更新日期:2014-05-01 00:00:00
abstract::Breastfeeding is well-known to have a protective effect against infection in infants. It has been suggested that breast milk may play a role in the prevention of certain childhood cancer. We investigated this issue in a case-control study comprising 300 patients with childhood cancer. There was 73 patients (24.3%) wit...
journal_title:Pediatric hematology and oncology
pub_type: 杂志文章
doi:
更新日期:2015-01-01 00:00:00