Abstract:
:Germline or acquired mutations involving the GATA-binding protein gene (GATA2) have been linked to a variety of clinical conditions. In addition, patients harboring GATA2 mutations have a striking predisposition to develop myeloid malignancies, such as myelodysplastic syndrome or acute myeloid leukemia, but not acute lymphoblastic leukemia (ALL). We report here a unique occurrence of early T-cell precursor ALL in a young child with GATA2 haploinsufficiency.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Esparza O,Xavier AC,Atkinson TP,Hill BC,Whelan Kdoi
10.1002/pbc.27649subject
Has Abstractpub_date
2019-06-01 00:00:00pages
e27649issue
6eissn
1545-5009issn
1545-5017journal_volume
66pub_type
杂志文章abstract::Congenital amegakaryocytic thrombocytopenia (CAMT) is characterised by neonatal thrombocytopenia, with reduced or absent bone marrow megakaryocytes, leading eventually to pancytopenia. The mean age for progression to bone marrow failure is four years, with the earliest reported being six months. We describe a CAMT pat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24566
更新日期:2013-09-01 00:00:00
abstract:BACKGROUND:The level of minimal residual disease (MRD) prior to allogeneic hematopoietic stem cell transplantation (HSCT) has been shown to be an independent prognostic factor for outcome of pediatric patients with high-risk acute lymphoblastic leukemia (ALL). Retrospective studies which used (semi-) quantitation of cl...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.20794
更新日期:2007-01-01 00:00:00
abstract:BACKGROUND:The aims of this study were to define the mRNA expression profiles of MYCN, DDX1, TrkA, and TrkC in biopsy tumor samples from 64 Brazilian patients with neuroblastomas of different risk stages and to correlate altered expression with prognostic values. PROCEDURE:Patients were retrospectively classified into...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22823
更新日期:2011-05-01 00:00:00
abstract::The incidence of bone marrow metastasis (BMM) in newly diagnosed Ewing sarcoma (ES) is variable across studies. An optimal staging strategy for detecting BMM is not defined. While bone marrow (BM) biopsy and/or aspirate (BMBA) have been the gold standard, [F-18]fluorodeoxyglucose positron emission tomography (FDG-PET)...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28807
更新日期:2021-02-01 00:00:00
abstract:BACKGROUND:There are no tests to identify critically ill children at high risk of deep venous thrombosis (DVT). In this exploratory study, we aimed to identify proteins that are associated with incident DVT in critically ill adolescents. PROCEDURE:Plasma samples were obtained from critically ill adolescents within 24 ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.28159
更新日期:2020-04-01 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:BACKGROUND:Burnout is a work-related syndrome consisting of emotional exhaustion, depersonalization, and diminished feelings of personal accomplishment. Physicians who care for patients with life-threatening illnesses are at high risk for developing burnout. This survey evaluates the prevalence of burnout among pediatr...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23121
更新日期:2011-12-15 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:Sickle cell disease (SCD) is an inherited blood disorder characterized by a chronic hemolytic anemia that can contribute to fatigue and global cognitive impairment in patients. The study objective was to report on the feasibility, reliability, and validity of the PedsQL™ Multidimensional Fatigue Scale in SCD...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.24776
更新日期:2014-01-01 00:00:00
abstract::Langerhans cell histiocytosis is known to mimic many other conditions. We present two patients where anti-tubercular therapy was instituted when clinical and radiological features suggested tuberculosis. The correct diagnosis of histiocytosis was reached only on further work-up including immunohistochemistry following...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21930
更新日期:2009-07-01 00:00:00
abstract::While encapsulated bacterial agents, particularly Streptococcus pneumoniae, are recognized as important microbes that are associated with serious illness in hosts with sickle cell disease (SCD), multiple pathogens are implicated in infectious manifestations of SCD. Variations in clinical practice have been an obstacle...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.25911
更新日期:2016-05-01 00:00:00
abstract:BACKGROUND:M6620 is a novel inhibitor of the DNA damage repair enzyme ATR, and has potentiated the activity of cisplatin and irinotecan in non-small cell lung cancer and colon cancer xenografts, respectively. PROCEDURES:M6620 was tested in vitro at concentrations ranging from 1.0 nM to 10.0 μM and at 75 nM in combinat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26825
更新日期:2018-02-01 00:00:00
abstract::Chronic myeloid leukemia (CML) is a rare disease in children. Different from that in adults, childhood CML involves transformative events occurring over a short time period. CML transformation to lymphoid blast phase (BP) is associated with copy number abnormalities, characteristic of BCR-ABL1 positive acute lymphobla...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27570
更新日期:2019-04-01 00:00:00
abstract::Hemorrhagic cystitis (HC) is a common complication after stem cell transplantation (SCT) that occurs more frequently in patients with Fanconi anemia (FA) because of hypersensitivity of their cells to the agents used in the preparation for SCT (chemo and radiation). Many HC cases respond to therapy with hyperhydration ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20692
更新日期:2007-11-01 00:00:00
abstract:BACKGROUND:Children treated with cranial radiotherapy (CRT) for leukemia are at risk of developing central nervous system injuries. Magnetic resonance imaging (MRI) represents the examination method of choice for evaluating radiation-induced brain complications. The purpose of this report is to describe the spectrum of...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22923
更新日期:2011-08-01 00:00:00
abstract:BACKGROUND:The identification of tissue-specific differentially methylated regions (tDMRs) is key to our understanding of mammalian development. Research has indicated that tDMRs are aberrantly methylated in cancer and may affect the oncogenic process. PROCEDURE:We used the MassARRAY EpiTYPER system to determine the q...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24282
更新日期:2013-03-01 00:00:00
abstract:INTRODUCTION:Regret about loss is one of the most intense types of regret experienced in life. Little is known about the bereavement regret of parents whose child has died of cancer. Although knowledge about parents' experiences after their child's death is vital for supporting these families, parents' regret is mostly...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28265
更新日期:2020-06-01 00:00:00
abstract::Six patients with high-risk neuroblastoma underwent the second stem cell collection round with G-CSF (5 μg/kg/day) + plerixafor (0.24 mg/kg/day) because the amount of CD34(+) cells collected during the first collection round with G-CSF alone was insufficient. The number of CD34(+) cells collected in the second collect...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24506
更新日期:2013-08-01 00:00:00
abstract::Adherence is a critical consideration in ongoing efforts to improve outcomes among adolescents and young adults (AYAs) with cancer. In this narrative review, we embed existing conceptualizations of adherence within a developmental context to provide a novel vantage point from which to examine this important issue. App...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28128
更新日期:2020-04-01 00:00:00
abstract:BACKGROUND:We sought to better define the role of hematopoietic cell transplantation (HCT) in first remission (CR1) for high-risk pediatric acute myeloid leukemia (AML). PROCEDURES:Outcomes were compared among patients aged less than 21 years with cytogenetically defined poor-risk AML treated with chemotherapy, matche...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24739
更新日期:2014-02-01 00:00:00
abstract:OBJECTIVE:A National Thalassemia Screening Program was adopted in Taiwan in 1993. This report examined that program's results and impact. METHODS:Patients with beta-thalassemia major born between 1994 and 2003 were recruited through the help of all thalassemia clinics in Taiwan. A structured questionnaire was designed...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21185
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Severe pandemic influenza A (H1N1) infection can lead to acute respiratory failure (ARF) with associated high mortality. Children with malignancy may be at higher risk of H1N1-associated ARF because of underlying primary disease or immunosuppression associated with chemotherapy. PROCEDURE:We describe the cl...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22973
更新日期:2011-10-01 00:00:00
abstract:BACKGROUND:The clinical impact of influenza in children undergoing therapy for cancer is not well-described in the literature. PROCEDURE:Laboratory-documented influenza infection in pediatric oncology patients cared for in a single regional pediatric medical center between July 2000 and June 2005 was identified by rev...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.21472
更新日期:2008-05-01 00:00:00
abstract:BACKGROUND:There is a need for more knowledge about how survivors of childhood cancer perceive their lives and what influence current health status has on their quality of life. The purpose was to describe this among a group of long-term survivors and among a comparison group. PROCEDURE:Telephone interviews were perfo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22492
更新日期:2010-08-01 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:INTRODUCTION:To describe the quality of life (QOL) of pediatric brain tumor survivors (PBTSs) prospectively and to identify potential medical, personal and family contextual factors associated with QOL. METHODS:Ninety-one PBTSs (8-16 years) who were off treatment and attending a regular classroom participated. Self- a...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26481
更新日期:2017-09-01 00:00:00
abstract::Survivorship care is a major area of focus in the holistic management of childhood cancer with current knowledge and information almost exclusively from high-income countries. In this review, we summarize the state of scientific knowledge, service delivery, advocacy initiatives, and research efforts in this field in I...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28556
更新日期:2020-09-01 00:00:00
abstract::Thymic epithelial neoplasms consist of thymomas, thymic carcinoids, and thymic carcinomas. Carcinomas are malignant tumors of the thymus characterized by obvious cytological anaplasia. They constitute only 4%-14% of thymic epithelial neoplams. Thymic carcinoma rarely occurs in children. Research in the English literat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.20468
更新日期:2006-08-01 00:00:00
abstract:BACKGROUND:The objective was to investigate factors associated with patient-related timing (PRT) to seek healthcare and health service-related timing (HSRT) to diagnose cancer and provide treatment to children without social security in Mexico. PROCEDURE:A cross-sectional survey was conducted in 13 Ministry of Health ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28331
更新日期:2020-09-01 00:00:00
abstract::We describe an African American family with Hoyeraal-Hreidarrson syndrome (HHS) in which 2 TERT mutations (causing P530L and A880T amino acid changes) and two in the DKC1 variants (G486R and A487A) were segregating. Both genes are associated with dyskeratosis congenita and HHS. It was important to determine the import...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24389
更新日期:2013-06-01 00:00:00