Abstract:
BACKGROUND:Clinical outcomes of children with sickle cell disease (SCD) who undergo total or partial splenectomy (PS) are poorly defined. The purpose of this retrospective study was to initiate an Internet-based registry to facilitate analysis of clinical outcomes for these children. We hypothesized that both surgical procedures would be well tolerated and would eliminate risk of splenic sequestration. METHODS:We developed a web-based registry using the Research Electronic Data Capture (REDCap) platform. Children were included if they had SCD and underwent total splenectomy (TS) or PS between 2003 and 2010. Clinical outcomes were compared between cohorts, with follow-up to 1 year. RESULTS:Twenty-four children were included, TS (n = 15) and PS (n = 9). There were no differences in surgical time or intraoperative blood loss. The length of stay was longer after PS (4.1 ± 1.7 days) compared to TS, (2.4 ± 1.2 days, P = 0.02). Within 30 days of surgery, 2 (20%) patients had acute chest syndrome (ACS) following TS and 2 (15%) patients had ACS after PS. During 1-year follow-up, no patient in either cohort had recurrent splenic sequestration, venous thrombosis or overwhelming postsplenectomy sepsis. All children who were transfused preoperatively to prevent recurrent splenic sequestration successfully discontinued transfusions. CONCLUSIONS:Both TS and PS result in favorable hematologic outcomes and low risk of adverse events for children with SCD. A REDCap-based registry may facilitate data entry and analysis of clinical outcomes to allow for comparison between different types of splenectomy.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Mouttalib S,Rice HE,Snyder D,Levens JS,Reiter A,Soler P,Rothman JA,Thornburg CDdoi
10.1002/pbc.24057subject
Has Abstractpub_date
2012-07-15 00:00:00pages
100-4issue
1eissn
1545-5009issn
1545-5017journal_volume
59pub_type
临床试验,杂志文章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:Children and adolescents with Down syndrome (DS) and acute lymphoblastic leukemia (ALL) are reported to have increased relapse rates and therapy-related mortality (TRM). Treatment regimens for DS-ALL patients often include therapy modifications. Dana-Farber Cancer Institute (DFCI) ALL Consortium protocols ha...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.27256
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:Dexamethasone is more efficacious than prednisone in the treatment of acute lymphoblastic leukemia (ALL), but has also been associated with greater toxicity. We compared neuropsychological outcomes for patients treated on DFCI ALL Consortium Protocol 00-01, which included a randomized comparison of the two s...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,随机对照试验
doi:10.1002/pbc.24666
更新日期:2013-11-01 00:00:00
abstract::Although awareness of implicit bias and its influence on providers and patients is increasing, the effects of implicit bias on the field of pediatric hematology-oncology are less clear. This Special Report reviews the literature on implicit bias in pediatrics and medical oncology and further provides case examples and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28204
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND:This study investigates the effect of using patient reported outcomes (PROs) about health-related quality of life (HRQOL) in clinical practice on the type and amount of psychosocial topics discussed during a paediatric oncology consultation. PROCEDURE:Children (N = 193) with cancer participated in a sequent...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.24089
更新日期:2012-07-15 00:00:00
abstract::Anaphylactic/anaphylactoid reaction to methotrexate (MTX) is uncommon. It may occur with the first dose (non-allergic reactions) or after a previous exposure to the drug (allergic or specific reactions). Desensitization has been shown effective in children with allergic-type reactions permitting the continuation of hi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22616
更新日期:2010-09-01 00:00:00
abstract::Steroid-induced neuropsychological side effects impact quality of life in children with acute lymphoblastic leukemia. Dexamethasone induces more metabolic side effects than prednisone. To evaluate whether dexamethasone also leads to more neuropsychological side effects, we reviewed all available literature. Randomized...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.24988
更新日期:2014-07-01 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:Osteopenia has been reported in children surviving acute lymphoblastic leukemia and brain tumors, apparently as a consequence of therapy. It has been suggested that cranial irradiation may play a role in the development of this complication. In order to explore that possibility, we examined survivors of brai...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20512
更新日期:2006-03-01 00:00:00
abstract:BACKGROUND:The combined inhibition of insulin-growth factor type 1 receptor (IGF-1R) and the mammalian target of rapamycin (mTOR) has shown activity in preclinical models of pediatric sarcoma and in adult sarcoma patients. We evaluated the activity of the anti-IGF-1R antibody cixutumumab with the mTOR inhibitor temsiro...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.25334
更新日期:2015-03-01 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::Germline DICER1 pathogenic variants predispose to numerous benign and malignant tumors. In this report, we describe DICER1 gene analysis in an adolescent diagnosed with multinodular goiter, ovarian Sertoli-Leydig cell tumor, and lung cyst. DICER1 mutational screening at the DNA level failed to detect any pathogenic va...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27005
更新日期:2018-06-01 00:00:00
abstract::The global burden of sickle cell disease (SCD) is now being increasingly realized. SCD poses a significant public health problem in sub-Saharan Africa, the Middle East, some regions of India, the Caribbean, and Brazil. In many of these regions, progress in the management of SCD has been slow. Long-term North-South and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.24175
更新日期:2012-08-01 00:00:00
abstract::Minimal residual disease (MRD) is an independent predictor of relapse risk in children with leukemia and is widely used for risk-adapted treatment. This article summarizes current evidence supporting the use of MRD, including clinical significance, current international clinical practice, impact statement, and recomme...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,实务指引,评审
doi:10.1002/pbc.25939
更新日期:2016-06-01 00:00:00
abstract::Severe congenital neutropenia (SCN) is a bone marrow failure disease with an autosomal dominant inheritance from mutations in ELANE. Here, we report a 7-week-old Korean male with SCN. His elder sister died from pneumonia at 2 years. Direct sequencing of ELANE in the proband identified a heterozygous novel frameshift m...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25654
更新日期:2015-12-01 00:00:00
abstract:BACKGROUND:Previous studies of children with homozygous sickle cell anemia (SCA) show impaired growth and maturation. The correlation of this suboptimal growth with metabolic and hematological factors during puberty is poorly understood. PROCEDURE:We studied a group of pre-adolescent children with SCA (19 males, 14 fe...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22137
更新日期:2009-10-01 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::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::Because of increasing survival rates in pediatric oncology, attention is focusing on cancer and its treatment-related side effects. Rehabilitation may reduce their impact. However, the literature does not provide strong evidence regarding rehabilitation pathways. Therefore, the Italian Association of Pediatric Hematol...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.28681
更新日期:2020-12-01 00:00:00
abstract::X-linked lymphoproliferative syndrome (XLP) is caused by mutations in SH2D1A, and is associated with overwhelming infectious mononucleosis, aplastic anemia, hypogammaglobulinemia, and B-cell lymphomas. However, the frequency of SH2D1A mutations in males who present with B NHL is unknown. Five cases of XLP were diagnos...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24525
更新日期:2013-09-01 00:00:00
abstract:BACKGROUND:SIOPEL protocols have recommended liver transplantation for unresectable hepatoblastoma (HBL) after chemotherapy in absence of visible extrahepatic disease. METHODS:This retrospective single center study includes 13 children treated following SIOPEL 3 or 4 protocols who underwent orthotopic liver transplant...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23301
更新日期:2011-12-15 00:00:00
abstract:BACKGROUND:In malaria-endemic countries in West Africa, sickle cell disease (SCD) contributes to childhood mortality. Historically, Liberia had regions wherein hemoglobin S and beta-thalassemia trait were mutually exclusive. Data on hemoglobinopathies in the Monrovia, the capital, are outdated and do not reflect urban ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25875
更新日期:2016-04-01 00:00:00
abstract::Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a cas...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26781
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:The male excess in childhood cancer incidence is well-established; however, the underlying biologic mechanisms remain unknown. Examining the association between male sex and childhood cancer by single year of age and tumor type may highlight important periods of risk such as variation in growth and hormonal ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27620
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:The improved survival rates for childhood acute lymphoblastic leukemia (ALL) may be jeopardized by the development of a second cancer, which has been associated with thiopurine therapy. PROCEDURE:We retrospectively analyzed three sequential Nordic Society of Paediatric Haematology and Oncology's protocols c...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26518
更新日期:2017-10-01 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:BACKGROUND:Children with cancer may be at an increased risk of infection with hepatitis B virus (HBV) when levels of hepatitis B antibodies are reduced owing to chemotherapy-induced immunosuppression. This study evaluated the changes in HBV antibody status and HBV infections after chemotherapy in children with acute ly...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27904
更新日期:2019-12-01 00:00:00
abstract:BACKGROUND:Currently, there is no standardized treatment for adolescents, aged 15 years or older, with mature B-cell non-Hodgkin lymphoma (B-NHL), although this age group has been reported to have a poorer prognosis than younger patients. PROCEDURE:The present study analyzed the data of 321 patients with B-NHL, enroll...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.27068
更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:Children with terminal cancer and their families describe a preference for home-based end-of-life care. Inadequate support outside of the hospital is a limiting factor in home location feasibility, particularly in rural regions lacking pediatric-trained hospice providers. METHODS:The purpose of this longitu...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28921
更新日期:2021-02-01 00:00:00
abstract:BACKGROUND:Children less than 5 years of age with malignant central nervous system (CNS) tumors, continue to have a high rate of morbidity and mortality following administration of conventional therapy. In an attempt to avoid the neurologic sequelae associated with craniospinal radiation, strategies such as high-dose c...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21381
更新日期:2008-05-01 00:00:00