Abstract:
:The commonly accepted definition of Fanconi anemia (FA) relying on DNA repair deficiency is submitted to a critical review starting from the early reports pointing to mitomycin C bioactivation and to the toxicity mechanisms of diepoxybutane and a group of nitrogen mustards causing DNA crosslinks in FA cells. A critical analysis of the literature prompts revisiting the FA phenotype and crosslinker sensitivity in terms of an oxidative stress (OS) background, redox-related anomalies of FA (FANC) proteins, and mitochondrial dysfunction. This re-appraisal of FA basic defect might lead to innovative approaches both in elucidating FA phenotypes and in clinical management.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Pagano G,d'Ischia M,Pallardó FVdoi
10.1002/pbc.25452subject
Has Abstractpub_date
2015-07-01 00:00:00pages
1137-43issue
7eissn
1545-5009issn
1545-5017journal_volume
62pub_type
杂志文章,评审abstract:BACKGROUND:Griscelli syndrome type 2 (GS2), the X-linked lymphoproliferative (XLP) and the Chédiak-Higashi (CHS) syndromes are diseases that all may develop hemophagocytic syndromes. We wanted to investigate whether the treatment protocols for hemophagocytic lymphohistiocytosis (HLH) can also be used for these syndrome...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21790
更新日期:2009-02-01 00:00:00
abstract:BACKGROUND:Opioids are a cornerstone of palliation of pain. We sought to assess variation in opioid prescription during the last week of life among a cohort of pediatric oncology patients who died while hospitalized. PROCEDURE:We used detailed hospital administrative data from the Pediatric Health Information System (...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.21824
更新日期:2009-07-01 00:00:00
abstract::Aciclovir prophylaxis was previously given to all immunocompromised patients treated by our unit, following contact with varicella zoster. In 2003, we changed practice according to National Guidelines, giving prophylaxis only to patients without serum varicella zoster immunoglobulin G antibody (VZ IgG) at diagnosis of...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21521
更新日期:2008-10-01 00:00:00
abstract:BACKGROUND:Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy associated with morbidity and mortality. We sought to understand family planning intentions of parents of young children with SCD including the awareness of three reproductive options (adoption, in vitro fertilization with egg/sperm donatio...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28227
更新日期:2020-05-01 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:Neuroblastoma is characterized by heterogeneity of histology, biology, and clinical behavior. Most epidemiology studies are based on Western and Japanese populations; there are very few studies on neuroblastoma from Southeast Asia. PROCEDURE:Cases of Thai children with neuroblastoma were retrospectively rev...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23325
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND:The purpose of this study was to determine the prevalence of pulmonary dysfunction in pediatric hematopoietic cell transplant (HCT) survivors and to identify associated risk factors. PROCEDURE:In a cross-sectional study, patients surviving at least 5 years after pediatric HCT were requested to undergo pulmo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20531
更新日期:2006-10-15 00:00:00
abstract:BACKGROUND:The median age of patients with Ewing sarcoma (EwS) at diagnosis is around 14-15 years. Older age is associated with a worse outcome. The correlation of age at diagnosis on sites of disease has not been fully described. OBJECTIVE:The goal of this study was to evaluate the differences in sites of primary tum...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27251
更新日期:2018-09-01 00:00:00
abstract:BACKGROUND:Anthracycline cardiomyopathy is of concern in children treated for acute myeloid leukaemia (AML), but there are few data on the incidence and natural history of cardiotoxicity after AML treatment in the United Kingdom, where regimens have included high anthracycline exposure. PROCEDURE:Prevalence and predic...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22908
更新日期:2011-04-01 00:00:00
abstract:BACKGROUND:Adolescents with acute lymphoblastic leukaemia (ALL) have languished in the shadow of success of the outcome of therapy in childhood ALL. Their treatment has always been incorporated into either paediatric or adult clinical trials depending on the mode of referral and hence there is a need to address an age ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20749
更新日期:2007-03-01 00:00:00
abstract:BACKGROUND:Worldwide, hemoglobinopathies affect millions of children. Identification of hemoglobin disorders in most sub-Saharan African countries is delayed until clinical signs of the disease are present. Limited studies have been conducted to understand their prevalence and clinical presentation among newborns in re...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.26735
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:The views and clinical practice of children's cancer units were surveyed regarding management of central venous catheter (CVC) occlusion (CVC-occlusion), CVC-related thrombosis (CVC-thrombosis) and thromboembolism (CVC-thromboembolism). PROCEDURE:A questionnaire was sent to all 22 United Kingdom Children's ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.21332
更新日期:2008-04-01 00:00:00
abstract:BACKGROUND:Better predictors of outcome would allow improved risk-adapted therapy for pediatric nonmetastatic osteosarcoma of the extremity. We investigated the predictive value of MR imaging-based measures of absolute and relative tumor size and volume at the time of diagnosis. We also assessed the relation of tumor s...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20077
更新日期:2004-12-01 00:00:00
abstract:OBJECTIVE:Current guidelines recommend high-priority treatment of severe sickle cell disease (SCD) pain with opioids; however, patients with SCD have historically been undertreated. We used mixed methods to assess pediatric residents' perceptions toward opioid use in SCD pain management. METHODS:We distributed a surve...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27535
更新日期:2019-02-01 00:00:00
abstract::In this retrospective study of patients with overt orbital retinoblastoma, we evaluated minimally disseminated disease (MDD) in bone marrow and cerebrospinal fluid (CSF) using CRX and/or GD2 synthase as markers. Ten patients were evaluated-five (50%) at diagnosis and five upon relapse. MDD was detected in four cases (...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27662
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:Prophylactic use of cranial radiation therapy (CRT) in young children with acute lymphoblastic leukemia (ALL) is associated with significant long-term morbidity. Therefore, current treatment protocols for pediatric B-precursor ALL have abandoned prophylactic CRT in favor of intrathecal chemotherapy, combined...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.10392
更新日期:2004-01-01 00:00:00
abstract:BACKGROUND:Males have a higher incidence of medulloblastoma (MB) than females, but the effect of gender on survival is unclear. Studies have yielded conflicting results, possibly due to small sample sizes or differences in how researchers defined MB. We aimed to determine the effect of gender on survival in MB using a ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21832
更新日期:2009-01-01 00:00:00
abstract::Malignant degeneration frequently arises from preexisting plexiform neurofibroma in patients with neurofibromatosis type 1 (NF1). Image guided biopsy for diagnostic purposes, such as with CT guidance, can be technically challenging in these patients, as CT cannot distinguish malignant from benign areas within the same...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24668
更新日期:2013-12-01 00:00:00
abstract::A diverse panel of pediatric cancer advocates and experts, whose collective experience spans the continuum of international academic medicine, industry, government research, and cancer advocacy, recently discussed challenges for pediatric cancer research in the context of coronavirus disease 2019 (COVID-19). Specifica...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28435
更新日期:2020-09-01 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: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:Traditionally, physicians have believed that limb-salvage surgery has functional and cosmetic advantages over amputation, yet the literature is equivocal. Therefore, we sought to compare the psychosocial and functional outcomes in osteosarcoma survivors after limb-salvage surgery and amputation. We hypothesi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22419
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND:Physical fitness is an important determinant of quality of life (QOL) after hematopoietic stem cell transplantation. Cardiac function can influence exercise performance. The aim of this study was to assess these factors and their interrelationship. PROCEDURE:Children underwent cardiopulmonary exercise testi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27499
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:Sickle cell disease (SCD) is characterized by frequent disease-related events that require acute care. It is unknown to what extent patients utilize multiple hospitals for acute care. We examined the continuity pattern of acute care visits to the hospital or emergency department. We hypothesized that among p...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24028
更新日期:2012-10-01 00:00:00
abstract:PURPOSE:To assess the safety of delayed high dose intravenous (i.v.) sodium thiosulfate (STS) in a case series of 12 children with malignant brain tumors who were treated with intraarterial (i.a.) carboplatin in conjunction with blood-brain-barrier disruption (BBBD). METHODS:Twelve children ages 17 months-12 years und...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20529
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND:Stroke is a severe complication of sickle cell anemia (SCA). The role of glucose-6-phosphate dehydrogenase (G6PD) deficiency in the development of stroke in children with SCA is controversial. PROCEDURE:The aim of this study was to investigate the association of clinical ischemic stroke, high-risk transcran...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25924
更新日期:2016-06-01 00:00:00
abstract::Acute lymphoblastic leukemia (ALL) is the most common type of pediatric neoplasia. Highly heterogeneous, ALL includes several genetic subtypes with varying clinical outcome. Although, some features are well established as prognostic predictors, the details of the molecular mechanisms underlying different phenotypes ar...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.24167
更新日期:2012-10-01 00:00:00
abstract:BACKGROUND:Pediatric venous thromboembolism (VTE) is an increasingly common problem. We hypothesized that VTE occurs most commonly in tertiary care settings and that the pattern of associated illnesses may have changed from earlier reports. METHODS:The Kids' Inpatient Database 2006 was utilized to identify children ≤ ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23388
更新日期:2012-08-01 00:00:00
abstract::We report the case of a drug interaction between methotrexate (MTX) and chloral hydrate (CH) observed in a child treated for acute leukemia. Significantly slower MTX clearance and increased MTX exposure occurred on the first three courses of a high-dose chemotherapy when co-administered with CH despite normal renal fu...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24393
更新日期:2013-03-01 00:00:00
abstract::Immunotherapy with the anti-GD2 monoclonal antibody ch14.18, or dinutuximab, represents an important therapeutic advance in the treatment of pediatric high-risk neuroblastoma and is now considered part of standard of care in this patient population. To date, transverse myelitis as a result of dinutuximab therapy has n...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26732
更新日期:2018-01-01 00:00:00