Abstract:
:Familial adenomatous polyposis (FAP) due to APC mutation is associated with an increased risk of hepatoblastoma. All cases of hepatoblastoma in patients with FAP reported in the literature were reviewed. One hundred and nine patients were identified. Thirty-five patients (of 49 with data) were diagnosed with hepatoblastoma prior to a later diagnosis of FAP (often in association with advanced colorectal carcinoma), emphasizing a need to identify patients earlier with germline APC mutations for early colorectal carcinoma screening. Hepatoblastoma may present at birth, and screening for hepatoblastoma in infancy in families with FAP prior to APC mutation testing results may be warranted.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Trobaugh-Lotrario AD,López-Terrada D,Li P,Feusner JHdoi
10.1002/pbc.27103subject
Has Abstractpub_date
2018-08-01 00:00:00pages
e27103issue
8eissn
1545-5009issn
1545-5017journal_volume
65pub_type
杂志文章,评审abstract::Twenty-eight patients were maintained on subcutaneous immunoglobulin replacement for persistent B-cell aplasia and agammaglobulinemia following CD19-targeted chimeric antigen receptor T-cell therapy for B-cell lymphoblastic leukemia. Patients were transitioned from intravenous to subcutaneous immunoglobulin replacemen...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28092
更新日期:2020-03-01 00:00:00
abstract:BACKGROUND:Childhood cancer is relatively rare and tends to present specific age distribution, as a prognostic factor for some of these diseases. Information on how young age affects prognosis, response to chemotherapy, and local control options in children versus AYA with osteosarcoma (OST) is minimal. METHODS:In ord...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25459
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Although screening for familial retinoblastoma has been shown to be beneficial we suspected that such screening programs may be less than optimal in developing countries (DC). METHODS:Retrospective cohort study comparing patients with familial retinoblastoma from five centers in DC (Argentina, Brazil, Turke...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21970
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:Patients with relapsed or refractory Ewing sarcoma have a poor outcome with conventional therapies. Cytarabine decreases EWS/FLI1 protein levels in Ewing sarcoma cells and has demonstrated preclinical activity against Ewing sarcoma in vitro and in vivo. The purpose of this phase II clinical trial was to esti...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21822
更新日期:2009-03-01 00:00:00
abstract:BACKGROUND:Radiotherapy is often deferred in very young children with medulloblastoma, in favor of more intense chemotherapy and stem cell rescue; however, posterior fossa radiation has been shown to improve overall survival (OS) and event-free survival compared with adjuvant chemotherapy alone. This study was performe...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27972
更新日期:2019-12-01 00:00:00
abstract::The FIP1L1-PDGFRA fusion gene is the most frequent genetic aberration in myeloid neoplasms associated with eosinophilia and abnormalities of PDGFRA, PDGFRB, or FGFR1. Affected patients in adult populations are very sensitive to imatinib therapy. Pediatric cases are rare and so far only one case of FIP1L1-PDGFRA positi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22655
更新日期:2010-10-01 00:00:00
abstract:BACKGROUND:Sleep disordered breathing (SDB) is emerging as a significant health condition for children. The purpose of this study is to evaluate SDB symptoms in childhood cancer survivors and identify associations with quality of life (QOL) and psychological symptoms. PROCEDURE:A sample of 62 survivors aged 8-18 years...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25394
更新日期:2015-04-01 00:00:00
abstract::Efficacy of therapeutic strategies relative to patient- and family-centered outcomes in pediatric oncology must be assessed. We sought to identify outcomes important to children with acute myeloid leukemia and their families related to inpatient versus at-home management of neutropenia. We conducted qualitative interv...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.26927
更新日期:2018-04-01 00:00:00
abstract::This review of the experience of high dose chemotherapy in patients with recurrent or refractory intracranial germ cell tumor confirms that sustained tumor control can be achieved with this modality both in germinoma and non-germinomatous germ cell tumors. Data from cooperative groups in Europe and North America sugge...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.22376
更新日期:2010-04-01 00:00:00
abstract::A 17-year-old girl with B-cell precursor acute lymphoblastic leukemia (BCP-ALL) with persistent minimal residual disease (MRD) who underwent standard chemotherapy was found to have a BCR-ABL1-like gene expression pattern. Genome sequencing revealed a JAK2 mutation not previously described in BCP-ALL and a potential th...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26328
更新日期:2017-05-01 00:00:00
abstract::With the increasing number of long-term survivors of childhood cancer, there continues to be a critical need for development and implementation of evidence-based recommendations for clinical follow-up. In order to establish and maintain health-related follow-up guidelines, it is important to recognize the attributes o...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20609
更新日期:2006-02-01 00:00:00
abstract:BACKGROUND:Survivors of childhood brain tumors are at increased risk for neurocognitive impairments, including deficits in abilities supported by frontal brain regions. Catechol-O-methyltransferase (COMT) metabolizes dopamine in the prefrontal cortex, with the Met allele resulting in greater dopamine availability and b...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24649
更新日期:2014-01-01 00:00:00
abstract::Most publications report the adverse (negative) health issues in childhood cancer survivors. Presenting information to the newly diagnosed patient in a positive manner is advocated, while noting that recurrence is the most likely adverse event. Re-analysis of population-based studies on life-threatening toxicities fro...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.22975
更新日期:2011-12-15 00:00:00
abstract:BACKGROUND:Little is known about the visual outcome of children affected by an optic pathway glioma (OPG). PROCEDURES:We evaluated the long-term visual outcome of 32 consecutive children affected by OPG without neurofibromatosis type-1 referred to the Pediatric Department of Padua University and managed according to s...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.22748
更新日期:2010-12-01 00:00:00
abstract:BACKGROUND:Several sickle cell clinical trials have closed due to inability to enroll patients. To limit the early cessation of a proposed clinical trial due to low accrual rates, we sought to better understand barriers and facilitators to enrolling parents of children with sickle cell anemia (SCD) into clinical trials...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,随机对照试验
doi:10.1002/pbc.24486
更新日期:2013-08-01 00:00:00
abstract:BACKGROUND:Pegasparaginase (PEG) is important for treatment of Acute Lymphoblastic Leukemia (ALL). Despite conjugation to polyethylene glycol to reduce immunogenicity, allergic reactions still occur and may be severe. Traditionally, PEG is given via intramuscular (IM) injection but recent protocols have shown it can be...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23380
更新日期:2012-09-01 00:00:00
abstract:BACKGROUND:Although weight loss and lack of linear growth occur in children with cancer, growth history is not included in research that aims to determine nutritional status in children newly diagnosed with cancer. Therefore, this study aimed to determine weight loss and lack of linear growth in this patient group. PR...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.25301
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:The Wnt/β-catenin pathway plays a central role in the pathogenesis of most hepatoblastomas (HBs), that is, up to 60-80% carry activating CTNNB1 mutations. HBs can however also be the first manifestation of familial adenomatous polyposis (FAP). As this is a severe disease, it is important for the patient and ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26991
更新日期:2018-06-01 00:00:00
abstract::High-risk (HR) acute lymphoblastic leukemia (ALL) remains one of the greatest challenges in pediatric oncology. Relapsed ALL is a leading cause of death in young people, and further improvements in outcome will required the development of therapeutic approaches directed against rational therapeutic targets, as escalat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.22996
更新日期:2011-06-01 00:00:00
abstract:BACKGROUND:Cancer is the second most common cause of childhood deaths in the United Kingdom and infection contributes to a quarter of all cancer-related deaths. This study aimed to estimate the risk, aetiology and outcome of bloodstream bacterial and fungal infections in children with cancer within a geographically def...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.24995
更新日期:2014-07-01 00:00:00
abstract:BACKGROUND:Thyroid dysfunction has been reported in up to 52% of patients 1.4 years after treatment with (131) I-Metaiodobenzylguanidine (MIBG) in children with neuroblastoma (NBL), despite the use of potassium-iodide (KI). Our aim was to investigate if the incidence and severity of thyroid damage increases in time. M...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24681
更新日期:2013-11-01 00:00:00
abstract:BACKGROUND:Central nervous system irradiation (CNS-RT) has played a central role in the cure of acute lymphoblastic leukemia (ALL), but due to the risk of long-term toxicity, it is now considered a less-favorable method of CNS-directed therapy. PROCEDURES:Retrospectively, we estimated the effect of CNS involvement and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26191
更新日期:2017-02-01 00:00:00
abstract:BACKGROUND:Children with cancer experience a wide range of conditions that require urgent evaluation in the emergency department (ED), yet variation in admission rates is poorly documented. PROCEDURE:We performed a retrospective cohort study using the Pediatric Health Information System of ED encounters by children wi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28140
更新日期:2020-06-01 00:00:00
abstract::The molecular detection of minimal residual disease (MRD) is standard of care in acute lymphoblastic leukemia to personalize the stratification of patients to appropriate intensity chemotherapy regimens. High-throughput sequencing (HTS) techniques are driving changes to MRD methodologies. Our study demonstrates HTS ca...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27787
更新日期:2019-08-01 00:00:00
abstract:AIM:Osteopontin (OPN) has been investigated as a biomarker for cancer and nonmalignant diseases during the last decades. Data about OPN as a potential biomarker in childhood diseases are still sparse, and reference values are not available in children. We aimed to establish reference values for children from birth to y...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28272
更新日期:2020-07-01 00:00:00
abstract:BACKGROUND:In a national pediatric case-control study, we observed a very high relative risk of leukemia in patients who had received continuous etoposide (CE) over 6 months or more, but we could not estimate the absolute risk. The purpose of the present study was to estimate this absolute risk after CE. PROCEDURES:We...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20380
更新日期:2005-07-01 00:00:00
abstract::Aplastic anemia (AA) is an immune-mediated disease. Although most patients are responsive to immunosuppressive therapy (IST) with a combination of anti-thymocyte globulin (ATG) and cyclosporine (CsA), some patients relapse or are refractory to IST. Sirolimus (rapamysin) inhibits the serine-threonine kinase mammalian t...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22865
更新日期:2011-07-01 00:00:00
abstract::Medulloblastomas (MB) are classified in four subgroups: the well defined WNT and Sonic Hedgehog (SHH) subgroups, and the less defined groups 3 and 4. They occasionally occur in the context of a cancer predisposition syndrome. While germline APC mutations predispose to WNT MB, germline mutations in SUFU, PTCH1, and TP5...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24765
更新日期:2014-02-01 00:00:00
abstract::Ameloblastic fibro-odontosarcoma (AFOS) is an extremely rare malignant odontogenic tumor. Complete surgical excision is the treatment of choice. Deaths due to disease recurrence and/or progression are documented. Here, we report the case of a 15-year-old female with multiple recurrent AFOS. She responded to chemothera...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25627
更新日期:2015-11-01 00:00:00
abstract:BACKGROUND:Infection in neutropenic children is a major cause of morbidity and mortality in children treated for cancer. In developing countries, children with cancer are often malnourished at diagnosis. In Blantyre, Malawi, children with Burkitt lymphoma are treated with a local protocol with limited toxicity. The aim...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22032
更新日期:2009-07-01 00:00:00