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 1954 and 1998 was extracted from the records of the MCTR. This included age, sex, date of diagnosis, systems affected at diagnosis and follow-up. RESULTS:The overall incidence rate for LCH was 2.6 cases per million child years. In those under 1 year of age the incidence rate was 9.0 cases per million child years, compared to 0.7 cases per million in those aged 10-14 years (P < 0.0001 for age trend). There was no evidence of seasonal variation in presentation by month of birth or first symptom. Bone was the most common site of disease involvement (67% of cases), followed by skin (37%) and soft tissue (22%). The overall survival rate has improved over time, from 57% in 1954-1968 to 74% in 1985-1998. Ninety percent of deaths were due to disease progression, the remainder were due to complications of intensive therapy. The site of LCH lesions and extent of disease present at diagnosis strongly predicted survival outcome. Patients with initial liver involvement had a 5-year survival rate of 25% compared with 93% for those with bone lesions alone at diagnosis. CONCLUSIONS:Incidence rates varied significantly by age at diagnosis, and have been stable over time. Survival has improved considerably over time, but varies strongly by age and systems affected at diagnosis.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Alston RD,Tatevossian RG,McNally RJ,Kelsey A,Birch JM,Eden TOdoi
10.1002/pbc.20884subject
Has Abstractpub_date
2007-05-01 00:00:00pages
555-60issue
5eissn
1545-5009issn
1545-5017journal_volume
48pub_type
杂志文章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:To evaluate efficacy, pharmacokinetics (PK) and pharmacodynamics of single-agent everolimus in pediatric patients with radiographically progressive low-grade glioma (LGG). METHODS:Everolimus was administered at 5 mg/m2 once daily as a tablet or liquid for a planned 48-week duration or until unacceptable tox...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28787
更新日期：2021-02-01 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：: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：BACKGROUND:Self-concept was compared between adult survivors of childhood acute lymphoblastic leukemia (ALL) and sibling controls. Adult survivor subgroups at greatest risk for negative self-concept were identified. PROCEDURE:Survivors (n = 578) aged > or =18 years, treated before age 20 years on Children's Cancer Gro...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.10434
更新日期：2004-03-01 00:00:00
abstract：:Granulocytic sarcoma (GS) is a localized tumor composed of immature myeloid cells. This extramedullary tumor can present before, concurrent with or after the diagnosis of acute myeloid leukemia. GS is extremely uncommon in acute promyelocytic leukemia (APL). As a proportion of patients never develop systemic disease, ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.21190
更新日期：2008-03-01 00:00:00
abstract：BACKGROUND:Distinguishing alveolar rhabdomyosarcoma (ARMS) from embryonal rhabdomyosarcoma (ERMS) has historically been of prognostic and therapeutic importance. However, classification has been complicated by shifting histologic criteria required for an ARMS diagnosis. Children's Oncology Group (COG) studies after IRS...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.26645
更新日期：2017-12-01 00:00:00
abstract：BACKGROUND:The death of a child is a devastating event that results in profound grief and significant psychosocial and physical morbidities in parents. The parental grief journey is a complex phenomenon necessitating the utilization of newer models of bereavement with a focus on relationships and exploration of parents...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.26046
更新日期：2016-09-01 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：:A contemporaneous presentation of a second breast cancer in a mother and an extremity rhabdomyosarcoma (RMS) in her daughter led to the diagnosis of the Li Fraumeni syndrome (LFS). Although the association between LFS and RMS in young patients is well recognised 1 there are no guidelines as to how this knowledge shoul...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20795
更新日期：2007-03-01 00:00:00
abstract：BACKGROUND:Primary mediastinal germ cell tumors (GCTs) are rare in children and still represent a challenge for both adult and pediatric oncologists because of their worse outcome compared to their gonadal counterpart. PROCEDURE:Prospectively collected data concerning patients enrolled in the Italian Association of Pe...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1002/pbc.25895
更新日期：2016-05-01 00:00:00
abstract：BACKGROUND:The use of carboplatin for the treatment of pediatric low grade gliomas (PLGG) is often limited by the development of carboplatin hypersensitivity. Reported rates of carboplatin hypersensitivity reactions vary between 6% and 32% in these patients. Here we report the frequency of carboplatin hypersensitivity ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.25686
更新日期：2016-01-01 00:00:00
abstract：:Global variations in the incidence of pediatric cancers have been described; however, the causes of such differences are not known. We investigated the relationship between the incidence of embryonal tumors and human development index on a global scale. Increasing incidence of neuroblastoma correlates significantly wi...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.26244
更新日期：2017-02-01 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：BACKGROUND:Fanconi anaemia is a genetic disease characterized by congenital abnormalities, progressive bone marrow failure, and a higher predisposition of oral squamous cell carcinoma. The purpose of this study was to evaluate the prevalence of oral mucosa lesions in patients with Fanconi anaemia without hematopoietic ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.25417
更新日期：2015-06-01 00:00:00
abstract：BACKGROUND:Sorafenib targets multiple pathways thought to be crucial in growth of plexiform neurofibroma (PN) in children with neurofibromatosis type 1 (NF1). Sorafenib has been tolerated with manageable toxicities in adults and children with refractory cancer. We conducted a separate study in this population. Monitori...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24281
更新日期：2013-03-01 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：BACKGROUND:Total body irradiation (TBI) is an important component of hematopoietic stem cell transplant (SCT) for pediatric malignancies. With increasing survival rates, late effects of SCT become more important. Younger children may be at particular risk of late effects of radiation and SCT. METHODS:We retrospectivel...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24252
更新日期：2013-04-01 00:00:00
abstract：:Hyperglycemia during induction chemotherapy for childhood acute lymphoblastic leukemia (ALL) has been inconsistently associated with risk of infection. We investigated the incidence of hyperglycemia during induction for childhood ALL in a retrospective cohort study of 144 patients treated on a single national protocol...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24689
更新日期：2013-12-01 00:00:00
abstract：BACKGROUND:Cisplatin (CDDP) ototoxicity is a significant side effect of the current treatment of medulloblastoma (MB). Cumulative dose of CDDP and age are recognized risk factors for hearing loss, but inter-individual susceptibility limits our ability to identify patients at risk for hearing loss. We describe the kinet...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24307
更新日期：2013-02-01 00:00:00
abstract：:Juvenile myelomonocytic leukemia (JMML) is a rare myelodysplastic/myeloproliferative disorder of early childhood characterized by mutations of the RAS-RAF-MAP kinase signaling pathway. We report the case of a child with a diagnosis of JMML carrying two mutations of NRAS gene (c.37G>C and c.38G>A) independently occurri...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.23401
更新日期：2012-09-01 00:00:00
abstract：BACKGROUND:Patients with sickle cell anemia (SCA) experience a broad range of psychiatric disorders, placing them at risk for more complicated and longer hospitalizations for vaso-occlusive crises (VOC). The current study examined the frequency of psychiatric disorders in SCA patients (ages birth to 20 years) admitted ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.23117
更新日期：2012-02-01 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/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：:Childhood, adolescent, and young adult (CAYA) cancer survivors may be at risk for a severe course of COVID-19. Little is known about the clinical course of COVID-19 in CAYA cancer survivors, or if additional preventive measures are warranted. We established a working group within the International Late Effects of Chil...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,多中心研究
doi：10.1002/pbc.28702
更新日期：2020-12-01 00:00:00
abstract：BACKGROUND:The neurokinin-1 receptor antagonist aprepitant, plus a 5HT3 antagonist and corticosteroid is well-tolerated and effective in preventing chemotherapy-induced nausea and vomiting in adults but has not been formally assessed in adolescents. PROCEDURE:Patients age 11-19 years old receiving emetogenic chemother...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,随机对照试验
doi：10.1002/pbc.21811
更新日期：2009-02-01 00:00:00
abstract：BACKGROUND:Children with cancer have high utilization of the emergency department (ED), but little is known about which outcomes are most important to them and their caregivers when they seek care in the ED. PROCEDURE:A qualitative evaluation of ED experience for children with cancer and their caregivers was performed...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.27903
更新日期：2019-10-01 00:00:00
abstract：BACKGROUND:Previous studies have found that parents of children with cancer desire more prognostic information than is often given even when prognosis is poor. We explored in audio-recorded consultations the kinds of information they seek. METHODS:Ethnographic study including observation and audio recording of consult...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28802
更新日期：2021-03-01 00:00:00
abstract：PURPOSE:Ectopic nephrogenic rests (ENR) are extremely rare, and their diagnosis and treatment have not yet been standardized. Our study aimed to analyze the clinical and pathological features of ENR in children and explore the optimal methods for diagnosis and treatment. METHODS:A retrospective, single-center, case se...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.26985
更新日期：2018-06-01 00:00:00
abstract：BACKGROUND:The outcome for patients with Ewing sarcoma family of tumors (ESFTs) of bone with metastases at diagnosis remains poor despite new approaches to treatment. We evaluated whether a dose-intensity chemotherapy regimen improved survival for patients with ESFTs of bone with metastases at diagnosis. METHODS:We en...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.21233
更新日期：2007-12-01 00:00:00
