Abstract：
BACKGROUND:Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis in children. The mortality and morbidity of JXG with extracutaneous lesions remain unclear. METHODS:Data of patients aged < 18 years who were diagnosed with JXG between 2001 and 2010 were retrospectively collected through a nationwide survey. RESULTS:Twenty patients (11 male and nine female) had extracutaneous lesions. The median observation time was 10 years (range, 0-17). Six patients presented with symptoms at birth. The median age at diagnosis was 8.5 months (range, 0 month-13 years). Fifteen patients underwent treatment for JXG, including chemotherapy (n = 11), and five did not receive treatment. All patients except one survived; 17 were disease-free and two survived with disease. One newborn-onset patient with liver, spleen, and bone marrow involvement died of the disease. Permanent sequelae included central diabetes insipidus, growth hormone deficiency, and panhypopituitarism detected at diagnosis in three, one, and two patients, respectively. Four patients had visual impairment (optic nerve compression and intraocular invasion in two each), three had epilepsy, one had mental retardation, and one had a skin scar. Eight patients who had intracranial lesions were older at diagnosis, and had a higher frequency of disease-related comorbidities and permanent sequelae than those without intracranial involvement. CONCLUSIONS:Patients with extracutaneous JXG had good outcomes, although those with intracranial lesions had serious permanent sequelae. Effective and safe treatment regimens for patients with intracranial JXG need to be developed.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Maeda M,Morimoto A,Shioda Y,Asano T,Koga Y,Nakazawa Y,Kanegane H,Kudo K,Ohga S,Ishii E,Histiocytosis Study Group of the Japanese Society of Pediatric Hematology\/Oncology.doi
10.1002/pbc.28381subject
Has Abstractpub_date
2020-07-01 00:00:00pages
e28381issue
7eissn
1545-5009issn
1545-5017journal_volume
67pub_type
杂志文章abstract：BACKGROUND:Several sickle cell clinical trials have closed due to inability to enroll patients. To limit the early cessation of a proposed clinical trial due to low accrual rates, we sought to better understand barriers and facilitators to enrolling parents of children with sickle cell anemia (SCD) into clinical trials...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,随机对照试验
doi：10.1002/pbc.24486
更新日期：2013-08-01 00:00:00
abstract：:Although investigators have been engaged in the study of human energy metabolism for over 100 years, there remain unanswered questions regarding the role that energy expenditure plays in human health and disease. A critical examination of available methods for measurement of the various components of daily energy expe...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.23369
更新日期：2012-01-01 00:00:00
abstract：BACKGROUND:Alveolar soft-part sarcoma (ASPS), a rare vascular sarcoma with a clinically indolent course, frequently presents with metastases. Vascular endothelial growth factor (VEGF) is a promising therapeutic target. In a phase-II trial of the VEGF receptor inhibitor cediranib for adults with ASPS, the partial respon...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,多中心研究
doi：10.1002/pbc.27987
更新日期：2019-12-01 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：:Epidural spinal cord compression as the initial presentation of acute lymphoblastic leukemia (ALL) is a rare and serious complication. Extramedullary disease is rarely reported in patients with ALL. The most common sites are bone, followed by soft tissue, skin and lymph nodes. We describe a child with common B-lineage...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22682
更新日期：2010-10-01 00:00:00
abstract：BACKGROUND:ABT-751, an orally bioavailable sulfonamide, binds beta-tubulin to inhibit microtubule polymerization. We described response and event-free survival (EFS) in children with neuroblastoma and other solid tumors receiving ABT-751, assessed in vitro cytotoxicity of ABT-751 and evaluated the effect of ABT-751 on ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22267
更新日期：2010-01-01 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：BACKGROUND:Testicular germ cell tumors (T-GCTs) occur from infancy to adulthood, and are the most common solid tumor in adolescent and young adult males. Traditionally, pediatric T-GCTs were perceived as more indolent than adult T-GCTs. However, there are few studies comparing these groups and none that specifically ev...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24773
更新日期：2014-03-01 00:00:00
abstract：:We report two second malignant neoplasms (SMNs) of the parotid gland. Patient 1 was initially diagnosed with precursor B-cell lymphoblastic lymphoma of the scalp. Eight years after her initial diagnosis she presented with a small, painless mass in the region of her parotid gland. Patient 2 was diagnosed with pre-B-cel...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20306
更新日期：2005-12-01 00:00:00
abstract：:Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor arising mainly in the orofacial bones. Among more than 200 cases described in articles, only seven cases involved orbital regions. We present a case of newborn with recurrent MNTI in the lateral wall of right orbit. The patient underwent complete surgic...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.21607
更新日期：2008-09-01 00:00:00
abstract：:This review of the experience of high dose chemotherapy in patients with recurrent or refractory intracranial germ cell tumor confirms that sustained tumor control can be achieved with this modality both in germinoma and non-germinomatous germ cell tumors. Data from cooperative groups in Europe and North America sugge...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,评审
doi：10.1002/pbc.22376
更新日期：2010-04-01 00:00:00
abstract：BACKGROUND:Children with sickle cell disease (SCD) are at increased risk of death from invasive bacterial infections. Emergent evaluation of fever allows early treatment of potentially fatal infections. Limited data exist regarding caregiver adherence to physician recommendations of prompt medical evaluation of fever i...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.25634
更新日期：2015-11-01 00:00:00
abstract：BACKGROUND:Retinoblastoma (Rb) is the most common intraocular primary malignancy in children. In industrialised countries, the cure rate is about 95%. We present the results of a prospective study on the management of Rb in the paediatric oncology unit of Gabriel Touré Teaching Hospital and African Institute of Tropica...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.27101
更新日期：2018-08-01 00:00:00
abstract：BACKGROUND:Childhood germ cell tumors (cGCTs), believed to arise from transformed primordial germ cells by an unknown mechanism, provide a unique model system for investigating cell signaling, pluripotency, and the microenvironment of neoplastic stem cells (NSCs) in vivo. This is the first report of proteomics of cGCTs...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.23282
更新日期：2012-05-01 00:00:00
abstract：BACKGROUND AND AIM:We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS:Patients enrolled on the proto...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.27482
更新日期：2019-01-01 00:00:00
abstract：:Undifferentiated embryonal sarcoma of the liver (UESL) is a rare aggressive mesenchymal pediatric tumor. Previously, reported outcomes have been very poor. Here, we report a single-center experience of five patients with UESL treated with upfront gross total resection and adjuvant chemotherapy. We have a median follow...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.26154
更新日期：2016-12-01 00:00:00
abstract：:A 4(3/4)-year-old male with Down syndrome (DS) presented with unsteady gait and fatigue. Neuroimaging revealed a cerebellar mass with concomitant obstructive hydrocephalus and additional metastatic lesions. He was successfully treated and is still in complete remission 5 years from diagnosis. The present case illustra...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22109
更新日期：2009-12-01 00:00:00
abstract：BACKGROUND:Premature newborns are vulnerable to iron imbalance, although the iron homeostasis during the perinatal period remains unclear. To clarify the iron metabolism of premature infants, we measured serum prohepcidin concentrations of preterm infants, and analyzed the association with iron parameters. METHODS:Sev...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22773
更新日期：2011-02-01 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:To evaluate intellectual decline in children with posterior fossa (PF) tumors treated with different therapeutic protocols. PROCEDURE:Forty children had a complete neuropsychological evaluation prospectively twice, at least 6 months year (y) after the end of their treatment. Patients were classified into fo...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20329
更新日期：2005-11-01 00:00:00
abstract：:Hemophilia is an excellent example in medicine where clinical translation of basic science discoveries has transformed the gloomy outlook of the disease. This review provides an overview of clinical advances in hemophilia management with a specific focus on the molecular heterogeneity of the disease and progress in ma...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,评审
doi：10.1002/pbc.23193
更新日期：2011-12-01 00:00:00
abstract：BACKGROUND:Diamond-Blackfan anemia (DBA) is a rare inherited bone marrow failure syndrome. The mainstays of treatment involve chronic red cell transfusions, long-term glucocorticoid therapy, and stem cell transplantation. Systematic data concerning endocrine function in DBA are limited. We studied patients in the DBA R...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.25780
更新日期：2016-02-01 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：BACKGROUND:To determine the maximum-tolerated duration and dose-limiting toxicity of a daily schedule of orally administered cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors. METHODS:Patients received oral cyclophosphamide (50 mg/m2/dose) in the morning followed ...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.10456
更新日期：2004-01-01 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：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：BACKGROUND:Primary stroke prevention programmes for children with sickle cell disease (SCD) have been shown to be feasible interventions in resource-poor countries. Different hydroxyurea (HU) regimens have been utilised in ameliorating the severity of SCD. OBJECTIVE:To determine the long-term outcomes of the stroke pr...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28906
更新日期：2021-02-01 00:00:00
abstract：BACKGROUND:Cancer diagnoses pose challenges to adolescents' and young adults' (AYA) physical, mental, and emotional health, and developmental tasks. In order for AYAs, caregivers, clinicians, and other collaborators to optimize health outcomes (coproduction of health), understanding what living well means for AYAs who ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,随机对照试验
doi：10.1002/pbc.28599
更新日期：2020-10-01 00:00:00
abstract：:Familial adenomatous polyposis (FAP) is an inherited condition causing numerous adenomatous colorectal polyps and a markedly elevated risk of colon cancer. FAP may be associated with various extracolonic manifestations such as desmoid fibromatosis and osteomas (termed Gardner's syndrome) and brain tumors, usually medu...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20985
更新日期：2008-02-01 00:00:00
abstract：:Chronic hypoxemia is a common manifestation among patients with sickle cell anemia (SCA) who develop chronic lung disease. We report the beneficial effect of hydroxyurea on chronic hypoxemia in three pediatric patients with SCA and recurrent episodes of acute chest syndrome (ACS). All three patients improved rapidly a...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.21480
更新日期：2008-06-01 00:00:00
