Abstract:
BACKGROUND:Langerhans cell histiocytosis (LCH) is a rare disease, frequently affecting young children. PROCEDURE:We performed a retrospective study in patients younger than 16 years old manifesting with skin symptoms, and documented their different cutaneous lesions and systemic symptoms. We compared subgroups of children with single-system, skin-only, and multisystem disease and sought signs predictive for multisystem disease. In a small sample of patients, BRAF mutations were analyzed in archived biopsies. RESULTS:A wide spectrum of cutaneous presentations varying from crusted nodules and papules, blisters, vascular tumor-like lesions, scaling orange to red macules (frequently in seborrheic regions) to purpuric macules, and papules was documented in our cohort of 32 children. Otitis externa was a common manifestation and mucosal lesions were seen in three patients. A novel manifestation was a red-blue nodule that appeared in a patient after a vaccination. None of the cutaneous lesions was predictive for the classification or final outcome as a single-system or multisystem disease. However, later onset and a more protracted course of skin lesions were more frequent findings in multisystem LCH. Mucosal lesions and otitis externa were almost exclusively seen in patients with multisystem disease, a finding that warrants further investigation. Both wild-type (WT) and mutated BRAF were found not only in multisystem LCH, but also in skin-only LCH. Two cases with rapidly resolving congenital lesions had WT BRAF. CONCLUSIONS:Late onset and a protracted course of skin lesions are associated with MS-LCH, whereas WT BRAF is found in rapidly resolving skin lesions.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Morren MA,Vanden Broecke K,Vangeebergen L,Sillevis-Smitt JH,Van Den Berghe P,Hauben E,Jacobs S,Van Gool SWdoi
10.1002/pbc.25834subject
Has Abstractpub_date
2016-03-01 00:00:00pages
486-92issue
3eissn
1545-5009issn
1545-5017journal_volume
63pub_type
杂志文章abstract:BACKGROUND:Parents and physicians may have different understandings of a child's risk of future limitations due to cancer or cancer treatment. We evaluated alignment between parent- and physician-estimated risk of late effects. METHODS:We surveyed 352 parents of children with cancer within 12 weeks of diagnosis, and t...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27020
更新日期:2018-07-01 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:Sleep disordered breathing (SDB) is emerging as a significant health condition for children. The purpose of this study is to evaluate SDB symptoms in childhood cancer survivors and identify associations with quality of life (QOL) and psychological symptoms. PROCEDURE:A sample of 62 survivors aged 8-18 years...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25394
更新日期:2015-04-01 00:00:00
abstract:BACKGROUND:Ewing sarcoma (ES) is a malignant tumor of bone and soft tissue of children and young adults. Patients with ES are treated with intensive chemotherapy regimens. We describe predictors of acute chemotherapy-associated toxicity in this population. PROCEDURE:In this retrospective cohort study, records of ES pa...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24031
更新日期:2012-10-01 00:00:00
abstract::Aplastic anemia (AA) is an immune-mediated disease. Although most patients are responsive to immunosuppressive therapy (IST) with a combination of anti-thymocyte globulin (ATG) and cyclosporine (CsA), some patients relapse or are refractory to IST. Sirolimus (rapamysin) inhibits the serine-threonine kinase mammalian t...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22865
更新日期:2011-07-01 00:00:00
abstract::Non-Hodgkin lymphoma (NHL) is a heterogeneous group of lymphoid malignancies with high incidence in adolescents and young adults (AYAs). The most common diseases include diffuse large B-cell lymphoma, anaplastic large cell lymphoma, Burkitt lymphoma, lymphoblastic lymphoma, and primary mediastinal large B-cell lymphom...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.27073
更新日期:2018-08-01 00:00:00
abstract:BACKGROUND:Irinotecan is highly active against rhabdomyosarcoma (RMS), yet its tolerability and efficacy in combination with radiation is unknown. We examined local control and toxicities in RMS patients treated with radiotherapy (RT) in combination with radiosensitizing agents irinotecan + carboplatin (I + C). PROCED...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24205
更新日期:2013-02-01 00:00:00
abstract::Juvenile xanthogranuloma (JXG) is a disorder of disputed origin thought to be related to the dermal/interstitial macrophage. A 5-year-old female presented with an aggressive systemic JXG that developed 5 months after the diagnosis of T-cell acute lymphoblastic leukemia (T-ALL). Examination of the T-cell receptor gamma...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22756
更新日期:2011-05-01 00:00:00
abstract:BACKGROUND:Accurate risk stratification of Langerhans cell histiocytosis (LCH) is essential as management can range from conservative in single system, low risk for central nervous system (CNS) involvement lesions to intensive chemotherapy for multisystem or high-risk disease. Additionally, being able to differentiate ...
journal_title:Pediatric blood & cancer
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更新日期:2021-01-14 00:00:00
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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
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24934
更新日期:2014-08-01 00:00:00
abstract::Thymic epithelial neoplasms consist of thymomas, thymic carcinoids, and thymic carcinomas. Carcinomas are malignant tumors of the thymus characterized by obvious cytological anaplasia. They constitute only 4%-14% of thymic epithelial neoplams. Thymic carcinoma rarely occurs in children. Research in the English literat...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.20468
更新日期:2006-08-01 00:00:00
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26991
更新日期:2018-06-01 00:00:00
abstract::Developing countries with an uncontrolled AIDS epidemic have new challenges to meet in ITP. Secondary ITP, HIV related, becomes an increasing problem, which has many aspects that need addressing, including medical, effective counseling, psychosocial and unresolved management issues. Assistance in developing treatment ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21020
更新日期:2006-10-15 00:00:00
abstract::Squamous cell carcinoma of the anal canal in children is rare. To date, the etiology and outcome of this condition have been not fully understood. Here, we report an 11-year-old child with anal canal cancer who had concomitant disorders of sex development. Radiotherapy followed by salvage surgery achieved disease-free...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25988
更新日期:2016-07-01 00:00:00
abstract:BACKGROUND:The introduction of the United Kingdom Medical Research Council's 10th AML trial (MRC AML 10) protocol incorporating high-dose anthracycline therapy has improved outcome of children with acute myeloid leukemia (AML). In this study, we review the results of childhood AML therapy in a Singapore university hosp...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20834
更新日期:2007-03-01 00:00:00
abstract:BACKGROUND:Little is known about the clinical significance of coronary artery dilation (CAD) and left ventricular hypertrophy (LVH) in patients with sickle cell disease (SCD). PROCEDURE:In a retrospective cohort, we studied the prevalence of CAD and LVH in 101 children with SCD in comparison to 93 healthy African-Amer...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25239
更新日期:2015-01-01 00:00:00
abstract::A 4-year-old male presented with rapid-onset cranial nerve palsy and ataxia. Brain magnetic resonance imaging (MRI) revealed a pontine mass lesion with discordant conventional and advanced imaging. A stereotactic core biopsy revealed glioblastoma with immunostaining suggestive of histone H3K27M and TP53 mutation, cons...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26416
更新日期:2017-08-01 00:00:00
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 confir...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27667
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:Pediatric papillary thyroid carcinoma (PTC) is clinically and biologically distinct from adult PTC. We sequenced a cohort of clinically annotated pediatric PTC cases enriched for high-risk tumors to identify genetic alterations of relevance for diagnosis and therapy. METHODS:Tumor DNA and RNA were extracted...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28741
更新日期:2021-01-01 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::We evaluated the effects of body mass index (BMI) on the risk of thromboembolism (TE) in children (<18 years) with hematological malignancies during the period 1990-2009 (n = 359). Obesity was prevalent in 12% of patients: 6% versus 17% prior to and after the year 2000 (P = 0.02). Sixty-one (17%) patients developed TE...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23355
更新日期:2012-08-01 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::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:The histological response to neoadjuvant chemotherapy (NAC) in pediatric patients with Ewing sarcoma family of tumors (ESFT) can predict the disease-free survival. Therefore, a noninvasive method for response assessment is needed. Using the currently established imaging modalities, mass reduction does not al...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28605
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:The hallmark of sickle cell disease (SCD) is recurrent, painful vaso-occlusive episodes (VOC) and is the most common reason for hospitalization in SCD patients. Narcotics, particularly morphine, along with fluid hydration are standard treatments for painful episodes but have been associated with the developm...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.20403
更新日期:2005-10-15 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
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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
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journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25985
更新日期:2016-08-01 00:00:00
abstract::The rejection rate in cord blood transplants for chronic Epstein-Bar virus-associated T or natural killer cell lymphoproliferative diseases using our standard reduced-intensity conditioning "LPAM140 regimen," which includes fludarabine, melphalan (LPAM), etoposide, and antithymocyte globulin, has been high. To ensure ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28536
更新日期:2020-09-01 00:00:00