Abstract:
INTRODUCTION:The rate of bacterial infections in children with sickle cell disease (SCD) has decreased in recent years, mainly due to penicillin prophylaxis and vaccination. OBJECTIVES:To determine the rate of severe bacterial infection (SBI) in a cohort of children with SCD and to describe low-risk factors for confirmed SBI (CSBI) and acute chest syndrome (ACS). METHODS:This 11-year retrospective cohort study included children with febrile SCD admitted to a reference hospital in Spain. A case-control study was performed comparing patients diagnosed with SBI to those without SBI, and subanalyses for groups with CSBI and ACS were carried out. RESULTS:A total of 316 febrile episodes were analyzed; 69 (21.8%) had confirmed or possible SBI. Thirteen of those had CSBI (4.1%), eight urinary tract infection, and five bacteremia/sepsis. Among the cases of possible SBI, the majority had ACS (54/56; 96.4%). Age >3 years, absence of central venous catheter, hemodynamic stability, and procalcitonin <0.6 ng/ml were low-risk factors for CSBI, whereas normal oxygen saturation and C-reactive protein <3 mg/dl were low-risk factors for ACS, with negative predictive values (NPV) of 98.3%, 97.4%, 96%, 97.2%, 87.5%, and 85.8%, respectively. CONCLUSION:In this cohort of children with SCD who were well vaccinated and received adequate prophylaxis, we found a low rate of bacteremia and CSBI. We described several low-risk factors for CSBI and ACS, all of them with a high NPV. These findings may help to develop a risk score to safely select the patients that could be managed with a more conservative approach.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Rincón-López EM,Navarro Gómez ML,Hernández-Sampelayo Matos T,Saavedra-Lozano J,Aguilar de la Red Y,Hernández Rupérez B,Cela de Julián E,RETRO-DREP Study Group.doi
10.1002/pbc.27667subject
Has Abstractpub_date
2019-06-01 00:00:00pages
e27667issue
6eissn
1545-5009issn
1545-5017journal_volume
66pub_type
杂志文章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::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:The transition from the pediatric setting to adult care is a well-described period of morbidity and mortality for persons with sickle cell disease (SCD). We sought to measure the feasibility and effectiveness of providing skill-based educational handouts on improving self-management and transition readiness ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27722
更新日期:2019-07-01 00:00:00
abstract:BACKGROUND:Primary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X-linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected primary HLH in Sweden ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25308
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Neonatal leukemia characterized by early stem cell origin and extramedullary infiltration in the first 4 weeks of life is rare. We analyzed the features and outcome of neonatal leukemia in Japan to establish an appropriate treatment strategy for this rare disorder. PROCEDURE:Patients with infant leukemia re...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.20599
更新日期:2006-09-01 00:00:00
abstract::The incompatibility causing fetal and neonatal alloimmune thrombocytopenia (FNAIT) results from a fetus inheriting a paternal human platelet antigen (HPA), which is different from the maternal HPA. We present a unique case of FNAIT in a pregnancy involving an oocyte recipient mother with Turner syndrome. This is the f...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26447
更新日期:2017-08-01 00:00:00
abstract:BACKGROUND:Compared with healthy children, pediatric oncology patients have impaired sleep and engage in less physical activity (PA). Socioeconomic status (SES) may be one determinant of PA and sleep among pediatric oncology patients. PROCEDURE:Between November 12, 2009 and March 27, 2013, 50 pediatric oncology patien...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26143
更新日期:2016-11-01 00:00:00
abstract::In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21039
更新日期:2007-02-01 00:00:00
abstract:BACKGROUND:Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. METHODS...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26022
更新日期:2016-08-01 00:00:00
abstract:BACKGROUND:Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of pathological Langerhans cells, for which the aetiology and pathogenesis remain largely unknown. PROCEDURE:Information on the 101 children with LCH registered with the population-based Manchester Children's Tumour Registry (MCTR) between...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20884
更新日期:2007-05-01 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::Data on prognostic factors in pelvic PNET are minimal. We analyzed patients with pelvic PNET treated between June 2003 and November 2011 for prognostic factors. Forty-eight (13%) of 374 patients with PNET were pelvic PNET with median age 14.5 years (range: 5-33); 31 (65%) had metastases. After median follow-up of 20.4...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.24552
更新日期:2013-09-01 00:00:00
abstract::Poikiloderma with neutropenia (PN) is a genodermatosis characterized by poikiloderma, permanent neutropenia, recurrent infections, nail abnormalities, and palmoplantar hyperkeratosis. We report the case of a Tunisian patient with PN. Skin lesions started from the face and spread to the extremities and trunk. Neutropen...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27262
更新日期:2018-09-01 00:00:00
abstract::Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare autosomal recessive disorder characterized by thrombocytopenia from failure of megakaryopoiesis. CAMT is one of the bone marrow failure syndromes, and the disease progression may involve other lineages leading to pancytopenia. The genetic background of CAMT ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22842
更新日期:2011-02-01 00:00:00
abstract::Both hepatoblastoma and hypoplastic kidneys are rare in children. A review of all patients with hepatoblastoma treated at our institution between 1993 and 2011 revealed three cases of hepatoblastoma occurring in children with hypoplastic kidneys and significantly impaired renal function. Two patients were treated with...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24936
更新日期:2014-08-01 00:00:00
abstract:BACKGROUND:Bleomycin is associated with pulmonary toxic side effects including pneumonitis and pulmonary fibrosis. We evaluated the prevalence of long-term pulmonary function abnormalities in children receiving bleomycin therapy in the context of current chemotherapeutic regimens. METHODS:A retrospective review of pat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25098
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:MYCN amplification marks poor prognosis in neuroblastoma (NB) tumors. In evaluating the mechanisms by which retinoic acid (RA) or nerve growth factor (NGF) decrease cell number in MYCN amplified NB cells, we have identified a number of proteins whose expression either decreases (E2F, CDC2, CDK6, cyclin depen...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21195
更新日期:2008-02-01 00:00:00
abstract:BACKGROUND:We wanted to ascertain patterns of recurrence, re-treatment, and outcome among 188 eligible patients treated for localized orbital sarcoma on IRSG Protocols III/IV, 1984-1997. PROCEDURE:Retrospective chart review. RESULTS:Twenty-four of 188 patients (12.8%) developed local (n = 22) or distant relapse (n = ...
journal_title:Pediatric blood & cancer
pub_type: 历史文章,杂志文章
doi:10.1002/pbc.24289
更新日期:2013-03-01 00:00:00
abstract:BACKGROUND:(131) I-metaiodobenzylguanidine ((131) I-MIBG) is a targeted radiopharmaceutical for patients with neuroblastoma. Despite its tumor-specific uptake, the treatment with (131) I-MIBG results in whole-body radiation exposure. Our aim was to correlate whole-body radiation dose (WBD) from (131) I-MIBG with tumor ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25816
更新日期:2016-03-01 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:Nephrogenic rests (NRs) are abnormally persistent foci of embryonal cells, thought to be the precursor lesion of Wilms tumors (WTs). To date, their presence has not been systematically examined in WTs treated with preoperative chemotherapy. METHODS:A systematic analysis of the data on NRs in WTs treated wit...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.26547
更新日期:2017-11-01 00:00:00
abstract::To search for novel mechanisms that contribute to the pathophysiology of idiopathic thrombocytopenic purpura (ITP), we determined the whole blood gene expression profile in five ITP patients and five control samples. Using DNA microarrays that contained 24,473 unique putative genes, we found 176 cDNAs that were strong...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20981
更新日期:2006-10-15 00:00:00
abstract:BACKGROUND:SCH 727965 is a novel drug in clinical development that potently and selectively inhibits CDK1, CDK2, CDK5, and CDK9. The activity of SCH 727965 was evaluated against the PPTP's in vitro and in vivo panels. PROCEDURES:SCH 727965 was tested against the PPTP in vitro panel using 96 hours exposure at concentra...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24073
更新日期:2012-12-15 00:00:00
abstract:BACKGROUND:Although maternal thrombocytopenia during pregnancy is common, its effect on neonatal platelets has not yet been fully evaluated. METHODS:We retrospectively evaluated the rate of thrombocytopenia among 767 healthy term neonates (gestational age 37-42 weeks) born to 723 mothers with pregnancy-induced thrombo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22514
更新日期:2010-07-15 00:00:00
abstract:OBJECTIVE:To investigate the effect of Helicobacter pylori eradication on platelet recovery in childhood chronic idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS:A multi-center randomized controlled trial was conducted. Patients aged 4-18 years, diagnosed with chronic ITP, defined by platelet count belo...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/pbc.21991
更新日期:2009-07-01 00:00:00
abstract:BACKGROUND:Choroid plexus tumors (CPT) are rare, and predominate in early childhood. An association with the Li-Fraumeni syndrome (LFS) has been reported, but the biological and clinical implications of this association remain poorly defined. We have investigated the clinical features and overall survival of all CPT pa...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23349
更新日期:2012-06-01 00:00:00
abstract::We describe here the outcomes of reduced-toxicity alternate-donor stem cell transplant (SCT) with posttransplant cyclophosphamide (PTCy) for primary immunodeficiency disorders (PIDs) in eight children (haploidentical-seven and matched unrelated donor-one). The conditioning was with serotherapy (alemtuzumab-3/rabbit-an...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26783
更新日期:2018-01-01 00:00:00
abstract:BACKGROUND:Neuroblastoma (NB) is the most common solid extracranial tumor in childhood. Despite advances in therapy, the prognosis is poor and optimized therapies are urgently needed. Therefore, we investigated the antitumor potential of interleukin-15 (IL-15)-activated cytokine-induced killer (CIK) cells against diffe...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26147
更新日期:2016-12-01 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::Survivors of childhood cancer are at risk for dental late effects. This systematic review summarizes associations between treatment exposures and dental late effects among survivors of childhood cancer. We included investigations with at least 20 study participants conducted for 2 or more years after completion of chi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.24842
更新日期:2014-03-01 00:00:00