Abstract：
BACKGROUND:Langerhans cell histiocytosis is a rare disease of unknown etiology. We wanted to assess the population-based incidence of LCH in a well-defined cohort of children. METHODS:We identified all children <15-years old treated with LCH during the 10 years period 1992-2001 at the Department of Pediatrics, Karolinska University Hospital in Stockholm, the referral center for children with LCH in Stockholm County. We also contacted the Departments of Dermatology, Orthopedics, and Neurosurgery for possible additional patients. RESULTS:Twenty-nine children (16 males) with LCH were identified, with a median age at diagnosis of 3.8 years (2 months-13.7 years). All children but one had a definitive diagnosis of LCH. The minimum incidence of LCH is estimated to 8.9/10(6) children per year. At diagnosis, 20 children (69%) had single system (SS) and 9 (31%) multisystem (MS) manifestations. Five of the 20 children with SS eventually developed MS disease, thus 14 (48%) had MS involvement at the maximal extent of disease (4.3/10(6) children per year). Interestingly, 22 children (76%) were diagnosed during the fall (September-November, n = 12) and winter (December-February, n = 10) seasons, as compared to seven children during the spring (March-May = 1) and summer (June-August = 6) seasons (P = 0.005, Chi-square). CONCLUSIONS:The incidence of childhood LCH in our study is higher than previously reported. In our patient cohort, LCH was more commonly diagnosed during the fall and winter season as compared to the spring and summer season. Whether this seasonal variation can be confirmed in larger studies and whether it has relevance for LCH pathophysiology remains to be elucidated.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Stålemark H,Laurencikas E,Karis J,Gavhed D,Fadeel B,Henter JIdoi
10.1002/pbc.21504subject
Has Abstractpub_date
2008-07-01 00:00:00pages
76-81issue
1eissn
1545-5009issn
1545-5017journal_volume
51pub_type
杂志文章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：:The fundamental purposes underlying formal health care transition from the pediatric to adult setting for young adult survivors of childhood cancer are to facilitate the continuous, medically and developmentally appropriate implementation of risk-based guidelines for the monitoring and management of late effects of ch...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,评审
doi：10.1002/pbc.21455
更新日期：2008-05-01 00:00:00
abstract：BACKGROUND:Central nervous system (CNS) malignancies are the most common solid tumors among children, and novel therapies are needed to help improve survival. Pomalidomide is an immunomodulatory agent that displays antiangiogenic and cytotoxic activity, making it an appropriate candidate to explore in pediatric CNS tum...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28756
更新日期：2021-02-01 00:00:00
abstract：BACKGROUND:Better predictors of outcome would allow improved risk-adapted therapy for pediatric nonmetastatic osteosarcoma of the extremity. We investigated the predictive value of MR imaging-based measures of absolute and relative tumor size and volume at the time of diagnosis. We also assessed the relation of tumor s...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20077
更新日期：2004-12-01 00:00:00
abstract：BACKGROUND:PG11047 is a novel conformationally restricted analog of the natural polyamine, spermine that lowers cellular endogenous polyamine levels and competitively inhibits natural polyamine functions leading to cancer cell growth inhibition. The activity of PG11047 was evaluated against the PPTP's in vitro and in v...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22797
更新日期：2011-08-01 00:00:00
abstract：BACKGROUND:Children with hepatoblastoma (HB) are at risk of sarcopenia due to immobility, chemotherapy, and malnutrition. We hypothesized that children with HB have a low preoperative total psoas muscle area (tPMA), reflecting sarcopenia, which negatively impacts outcome. PROCEDURE:Retrospective study of children (1-1...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28862
更新日期：2021-01-12 00:00:00
abstract：BACKGROUND:Drug shortages require clinical teams to decide how to allocate drugs in limited supply among their patients. Ethical frameworks are invaluable for promoting rational approaches to drug distribution, but gaps remain between ethical theory and clinical application. The goal of this work was to explore how dec...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.26331
更新日期：2017-07-01 00:00:00
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：: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
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:Histology after intensive induction chemotherapy is expected to become a beacon indicating when and how extensively radical surgery and lymph node dissection should be performed in advanced neuroblastoma. A thorough histologic review of surgical specimens was undertaken. PROCEDURE:All specimens from 34 pati...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20345
更新日期：2005-10-15 00:00:00
abstract：:Increasingly young people survive cancer in childhood and as a result complications of its treatment are becoming more common and important. Premature ovarian failure is recognized as a complication of radiotherapy to a field that includes the pelvis and alkylating-agent-based chemotherapy. Young pre-pubertal girls ar...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,评审
doi：10.1002/pbc.22012
更新日期：2009-08-01 00:00:00
abstract：BACKGROUND:Corticosteroids increase risk for decreased bone mineral density, which can be worsened by vitamin D insufficiency (VDI) or deficiency (VDD). PROCEDURE:In the Vanderbilt cancer survivorship clinic, we obtained screening total 25-hydroxy vitamin D levels (VDL) in 171 cancer survivors <23 years old who were t...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.24844
更新日期：2014-04-01 00:00:00
abstract：:Leydig cell tumors account for 3% of testicular tumors and have never been reported after treatment for Ewing's sarcoma. We report the unusual occurrence of a patient who developed a Leydig cell tumor of the testis 18 years after successful treatment for Ewing's sarcoma. Additional monitoring for second malignancies m...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20825
更新日期：2007-12-01 00:00:00
abstract：BACKGROUND:As pegylated asparaginase is becoming the preferred first-line asparaginase preparation in the chemotherapy regimens of childhood acute lymphoblastic leukemia (ALL), there is a need to evaluate this treatment. METHODS:The aim of this study was to evaluate the pharmacokinetics of prolonged upfront biweekly P...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章,随机对照试验
doi：10.1002/pbc.26686
更新日期：2017-12-01 00:00:00
abstract：:For many children with cancer, participation in oncology camp programs is an important component of healing that offers opportunities for fun and can have substantial impacts on social and physical well-being. Optimal medical care and infectious screening for children attending oncology camp is critical to maximize sa...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28107
更新日期：2020-03-01 00:00:00
abstract：BACKGROUND:Carboplatin is the most effective drug in retinoblastoma but systemic clearance is variable in young patients. While most regimens use a flat dose, individualized targeting may provide a more adjusted systemic exposure. PATIENTS AND METHODS:We compared carboplatin doses between two groups of children with r...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22467
更新日期：2010-07-15 00:00:00
abstract：BACKGROUND:The level of minimal residual disease (MRD) prior to allogeneic hematopoietic stem cell transplantation (HSCT) has been shown to be an independent prognostic factor for outcome of pediatric patients with high-risk acute lymphoblastic leukemia (ALL). Retrospective studies which used (semi-) quantitation of cl...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.20794
更新日期：2007-01-01 00:00:00
abstract：BACKGROUND:The treatment of central nervous system (CNS) germ cell tumors (GCT) remains controversial. The purpose of this study was to demonstrate efficacy of a chemotherapy only strategy, with less morbidity, when compared to regimens with irradiation. METHODS:Between January 2001 and December 2004 newly diagnosed p...
journal_title：Pediatric blood & cancer
pub_type： 临床试验,杂志文章
doi：10.1002/pbc.22381
更新日期：2010-03-01 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:Alvespimycin (17-DMAG, KOS-1022), a potent small-molecule inhibitor of the protein chaperone Hsp90, is being developed as an anticancer agent because of the multiple Hsp90 client proteins involved in cancer cell growth and survival. PROCEDURES:Alvespimycin was tested against the in vitro panel of the Pediat...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.21508
更新日期：2008-07-01 00:00:00
abstract：:Rhabdomyosarcoma arising within a congenital cystic adenomatoid malformation (CCAM) is an unusual entity. The patient underwent a lobectomy of his right lower lobe of lung due to a CCAM at the age of two. One year later, he developed a solid embryonal rhabdomyosarcoma at the same location. He received 1-year period ch...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20481
更新日期：2005-11-01 00:00:00
abstract：:Twenty-eight patients were maintained on subcutaneous immunoglobulin replacement for persistent B-cell aplasia and agammaglobulinemia following CD19-targeted chimeric antigen receptor T-cell therapy for B-cell lymphoblastic leukemia. Patients were transitioned from intravenous to subcutaneous immunoglobulin replacemen...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.28092
更新日期：2020-03-01 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：: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：:Opsoclonus, myoclonus, ataxia syndrome (OMA) is a severe neurologic disorder often associated with neuroblastoma. It is challenging to treat and can have long-term neurologic sequelae. Current recommended therapies include intravenous immunoglobulin, corticosteroids, rituximab, and chemotherapy (cyclophosphamide). We ...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.27110
更新日期：2018-08-01 00:00:00
abstract：BACKGROUND:The potential role of VEGF in osteosarcoma has been evaluated in several studies. The majority of them included heterogeneous and limited series of patients, giving conflicting results. The aim of presented study is to evaluate the prognostic role of VEGF-A in biopsy samples of clinically homogeneous group o...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.23021
更新日期：2011-07-15 00:00:00
abstract：BACKGROUND:Ewing tumors are the most frequent malignant tumors of the chest wall in children and young adults. Surgical management of these tumors can be challenging. Optimal local control remains controversial. The aim of this study was to analyze treatment, outcome, and surgical procedures in patients with thoracic t...
journal_title：Pediatric blood & cancer
pub_type： 更正并重新发布的文章,杂志文章
doi：10.1002/pbc.27884
更新日期：2019-08-01 00:00:00
abstract：BACKGROUND:There is little information about factors modulating bone mineral density (BMD) in survivors of childhood acute lymphoblastic leukemia (ALL). PROCEDURE:We analyzed data from 57 survivors (26 male, 52 Caucasian) who underwent two serial quantitative computed tomography (QCT) studies of BMD. Using multiple li...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.20553
更新日期：2006-01-01 00:00:00
abstract：:Clinically apparent jaundice is unusual in patients with beta-thalassemia major. Co-inheritance of Gilbert syndrome has been reported to cause hyperbilirubinemia in these subjects. Crigler-Najjar syndrome is another rare disorder of bilirubin metabolism caused by mutation in the gene coding the enzyme UGT1A1. We repor...
journal_title：Pediatric blood & cancer
pub_type： 杂志文章
doi：10.1002/pbc.22313
更新日期：2010-04-01 00:00:00
