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 transcranial Doppler measurements (TCD), and hematological features with molecular variants usually linked to G6PD deficiency or with the biochemical activity of G6PD in a cohort of 395 Brazilian children with SCA. G6PD activity was quantitatively determined using an enzymatic-colorimetric assay. G6PD mutations were determined by PCR-RFLP and sequencing. Clinical and hematological data were retrieved from the children's records. RESULTS:The prevalence of molecularly defined deficiency (hereafter, molecular deficiency) was 4.3% (95% confidence interval: 2.3-6.3%). The mean G6PD activity was 16.88 U/g hemoglobin (Hb) (standard error of the mean [SEM] 0.28) in the group without G6PD molecular deficiency and 8.43 (SEM 1.01) U/g Hb in the group with G6PD A(-) molecular deficiency. G6PD molecular deficiency was not associated with any hematological features. No effects of G6PD molecular deficiency on clinical ischemic stroke or high-risk TCD were detected. The mean G6PD activity was similar in children who had clinical ischemic stroke and in those without stroke. Similar results were obtained in analyses comparing children who had high-risk TCD and those without high-risk TCD. CONCLUSIONS:Our study demonstrated that G6PD molecular deficiency was not associated either with clinical ischemic stroke or high-risk TCD. Similarly, we found no associations between G6PD enzyme activity and stroke or high-risk TCD. Small sample size precludes definitive conclusions.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Belisário AR,Rodrigues Sales R,Evelin Toledo N,Velloso-Rodrigues C,Maria Silva C,Borato Viana Mdoi
10.1002/pbc.25924subject
Has Abstractpub_date
2016-06-01 00:00:00pages
1046-9issue
6eissn
1545-5009issn
1545-5017journal_volume
63pub_type
杂志文章abstract:PURPOSE:To report the final analysis of survival outcomes for children with newly diagnosed high-grade glioma (HGG) treated on the "Head Start" (HS) II and III protocols with chemotherapy and intent to avoid irradiation in children <6 years old. PATIENTS AND METHODS:Between 1997 and 2009, 32 eligible children were enr...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26118
更新日期:2016-10-01 00:00:00
abstract::Children with sickle cell anemia (SCA) have low bone mass though bone turnover has not been well described. In this study, growth and pubertal development were assessed twice, 1 year apart, in 80 young subjects with type-SS SCA, while whole body bone mineral content (BMC) and density where measured in a subset (n = 46...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21147
更新日期:2008-03-01 00:00:00
abstract:BACKGROUND:The prognostic significance of having extraskeletal (EES) versus skeletal Ewing sarcoma (ES) in the setting of modern chemotherapy protocols is unknown. The purpose of this study was to compare the clinical characteristics, biologic features, and outcomes for patients with EES and skeletal ES. METHODS:Patie...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26096
更新日期:2016-10-01 00:00:00
abstract:BACKGROUND:Clinical characteristics and optimal treatment strategies for spindle cell/sclerosing rhabdomyosarcoma (ssRMS) have not been well established because of its rarity. PROCEDURE:Retrospective re-evaluation of sarcoma specimens (1997-2014) identified 16 ssRMSs (median age 20 years, range 7-39 years). Clinicopat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25367
更新日期:2015-06-01 00:00:00
abstract::Social media are powerful means of communication that can also have an important role in the healthcare sector. They are sometimes seen with diffidence in the healthcare setting, partly because they risk blurring professional boundaries. This issue is particularly relevant to relations between caregivers and adolescen...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26958
更新日期:2018-05-01 00:00:00
abstract:BACKGROUND:The Kids ITP Tools (KIT) is a disease-specific measure of health-related quality of life for children with immune thrombocytopenia (ITP). To facilitate use in international trials it has been cross-culturally adapted for France, Germany, the United Kingdom and Uruguay. This study assessed the validity and re...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24257
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:This study was conducted to evaluate oral health and dental anomalies in children treated for acute lymphoblastic leukemia (ALL) and to compare results with those of a group of healthy children matched for sex and age. PROCEDURE:Fifty-six children treated for ALL and 56 healthy controls were examined for de...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22108
更新日期:2009-09-01 00:00:00
abstract::Ependymoblastoma (EBL) is a rare malignant CNS tumor of early childhood, listed as a subgroup of primitive neuroectodermal tumors (PNET) in the 2007 WHO Classification of Tumours of the Central Nervous System. Histologically, EBL can be defined by multilayered, mitotically active "ependymoblastic" rosettes with centra...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24915
更新日期:2014-06-01 00:00:00
abstract::Optic pathway gliomas represent approximately 5% of all pediatric intracranial tumors. While these tumors are most frequently low grade astrocytomas, they follow a highly variable clinical course, and accordingly, there is much debate regarding their optimal management. Their propensity to occur in very young children...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.20655
更新日期:2006-05-01 00:00:00
abstract::Malignant mesothelioma (MM) is a highly aggressive malignancy that is extremely rare in children. This case report documents a 7-year-old male without previous asbestos exposure with peritoneal MM that initially responded to chemotherapy with cisplatin and gemcitabine but ultimately metastasized to his chest. He was d...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24284
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Progressive/recurrent high-grade and diffuse intrinsic pontine gliomas (DIPGs) are fatal. Treatments targeting molecular pathways critical for these cancers are needed. METHODS:We conducted a phase 1 study (rolling-six design) to establish the safety and maximum tolerated dose (MTD) of dasatinib, an oral pl...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27035
更新日期:2018-07-01 00:00:00
abstract:BACKGROUND:Treatment intensification was considered a suitable strategy to increase the cure rate of lymphoblastic lymphoma (LBL) in children. PROCEDURE:The AIEOP LNH-97 trial was run between 1997 and 2007 for newly diagnosed LBL in patients aged less than 18 years. Treatment schedule was based on the previous, LSA2-L...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.25469
更新日期:2015-08-01 00:00:00
abstract::Torsades de Pointes (TdP) is a life-threatening ventricular arrhythmia that can be associated with metabolic abnormalities, exposure to arrhythmogenic medications, and congenital long-QT syndrome. This report describes a patient with ALL and multiple complications of therapy who developed TdP. The patient had no evide...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20712
更新日期:2007-12-01 00:00:00
abstract::The treatment of malignancy in cancer predisposition syndromes that also confer exquisite sensitivity to standard chemotherapy and radiation regimens remains a challenge. Bloom syndrome is one such disorder that is caused by a defect in DNA repair, predisposing to the development of early-onset age-related medical con...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28815
更新日期:2021-02-01 00:00:00
abstract::As a sympathetic nervous system-derived tumor, aggressive neuroblastoma (NB) is currently attracting interest from researchers seeking diagnostic and prognostic markers via less invasive procedures. The analysis of circulating tumor DNA (ctDNA) in peripheral blood can provide genetic information from multiple tumor le...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28311
更新日期:2020-10-01 00:00:00
abstract:BACKGROUND:Follicular variant of papillary thyroid carcinoma (FVPTC) has been shown to be an intermediate entity between papillary (PTC) and follicular/Hurtle cell (FTC) thyroid carcinoma in adults. However, the tumor characteristics and prognosis of FVPTCs has not been studied in the pediatric population and is the fo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25623
更新日期:2015-11-01 00:00:00
abstract::Developing countries with an uncontrolled AIDS epidemic have new challenges to meet in ITP. Secondary ITP, HIV related, becomes an increasing problem, which has many aspects that need addressing, including medical, effective counseling, psychosocial and unresolved management issues. Assistance in developing treatment ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21020
更新日期:2006-10-15 00:00:00
abstract:BACKGROUND:Pain is the most common complication of sickle cell disease requiring emergency department (ED) visits and hospitalization. A Clinical Practice Guideline (CPG) to manage acute sickle cell pain offers clinicians a standardized approach for the provision of evidence-based, cost-effective care. After CPG implem...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21847
更新日期:2009-03-01 00:00:00
abstract:BACKGROUND:Undernutrition impacts clinical outcome adversely in children with cancer. This study aimed to validate a nutritional algorithm with specific application to the low- and middle-income country (LMIC) setting. PROCEDURE:Fifty children with a new diagnosis of cancer were enrolled in this randomized interventio...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,随机对照试验
doi:10.1002/pbc.27980
更新日期:2019-12-01 00:00:00
abstract:BACKGROUND:Fever and neutropenia (F&N) is a pediatric oncology emergency due to the risk of disseminated infection. Quality improvement (QI) efforts to improve time to antibiotics for F&N in the emergency department have been documented, but the issue has not been studied in the established inpatient setting. PROCEDUR...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25712
更新日期:2016-01-01 00:00:00
abstract:BACKGROUND/OBJECTIVES:β-Thalassemia intermedia (β-TI) accounts for up to one-fourth of β-thalassemia patients. Evaluating and improving quality of life (QOL) should be a goal in β-TI follow-up and management strategies. Patients' perceptions of their illness and its treatment may impact their QOL. This study aimed to e...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.27735
更新日期:2019-07-01 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::Essential thrombocythemia is a rare myleoproliferative disorder in pediatrics. This myleoproliferative disorder is characterized by excessive proliferation of megakaryocytes and sustained elevation of platelet count. Reactive thrombocytosis is a more common cause of elevated platelet counts among children. We describe...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21218
更新日期:2008-04-01 00:00:00
abstract::Acute gastrointestinal graft-versus-host disease (GVHD) refractory to first-line treatment with systemic corticosteroids results in increased morbidity and potential mortality. We retrospectively assessed the feasibility and efficacy of catheter-directed intra-arterial platelet infusion (IAPI) in two pediatric patient...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.25633
更新日期:2015-12-01 00:00:00
abstract:BACKGROUND:Acute lymphoblastic leukemia (ALL) is the most common cancer in children. Because of major improvements in treatment protocols, the survival rate now exceeds 80%. However, ALL treatments can cause long-term neurocognitive sequelae, which negatively impact academic achievement and quality of life. Therefore, ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.27259
更新日期:2018-09-01 00:00:00
abstract:BACKGROUND:Sorafenib is an inhibitor of multiple kinases (e.g., VEGF receptors, PDGFR, FLT3, RET, BRAF, KIT) and is approved by FDA for treatment of two adult cancers. The activity of sorafenib was evaluated against the PPTP's in vitro and in vivo panels. PROCEDURES:Sorafenib was evaluated against the PPTP in vitro pa...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22712
更新日期:2010-12-01 00:00:00
abstract:BACKGROUND:The aim of this study is to evaluate the relationship between the latency to diagnosis (LD) and the time to completion of chemotherapy (TCC) with clinical outcomes in children with osteosarcoma. METHODS:We performed a retrospective analysis of all patients who received treatment for osteosarcoma in two tert...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.28180
更新日期:2020-04-01 00:00:00
abstract::Phytosterolemia is a rare autosomal recessive sterol storage disease caused by mutations in ABCG5 and ABCG8 genes. A 9-year-old Turkish boy who was presented with exclusively hematologic abnormalities had elevated plant sterol levels. Sequencing of ABCG5 and ABCG8 genes revealed a novel homozygous IVS10-1 G>T mutation...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24934
更新日期:2014-08-01 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
abstract:BACKGROUND:In the pediatric population, pathologic bleeding is often challenging to identify. The pediatric bleeding questionnaire (PBQ) was developed as a screening tool for von Willebrand disease (VWD) but was designed to be self-completed by children above 12 years of age. The study objective was to determine whethe...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27709
更新日期:2019-06-01 00:00:00