Abstract:
BACKGROUND:Conflicting data exist regarding whether low bone mineral density (BMD) is associated with Fanconi anemia (FA). The current study identified the frequency of low BMD in FA, expecting low BMD even in childhood and before HCT. PROCEDURE:Thirty-seven FA patients (18 prior HCT, 19 no prior HCT), participating in an IRB-approved database, had clinical assessment of DXA of lumbar spine BMD. Four had used androgens, one later underwent HCT. Most had used glucocorticoids after HCT (prolonged in five), and one more with no HCT. BMD [in standard deviation units from mean for age (SD), gender, and ethnicity (BMD Z-score)] was then adjusted for height age, and separately for bone maturation (BA). Data were collected for height SD, pubertal stage, and duration since HCT. RESULTS:BMD Z-score (without adjustment) was <-1 SD in half of FA children. BA-adjusted BMD Z-score was similar. (BA was not usually delayed, although most patients were short.) In contrast, height age-adjusted BMD Z-score was normal in most with FA (only below -2.0 in one child after prolonged glucocorticoids). Mean duration after HCT until DXA test was 6.2 years (median 4.2 years, range 1-18 years). CONCLUSIONS:Children and adolescents with FA have normal BMD prior to and after HCT, when DXA results are adjusted for bone size/height age. In contrast, BA-adjustment of BMD was not useful in this population. Individual BMD results may be influenced by gonadal function, transplantation status, and prolonged glucocorticoid therapy.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Rose SR,Rutter MM,Mueller R,Harris M,Hamon B,Bulluck AF,Smith FOdoi
10.1002/pbc.22956subject
Has Abstractpub_date
2011-12-01 00:00:00pages
1034-8issue
6eissn
1545-5009issn
1545-5017journal_volume
57pub_type
杂志文章abstract:BACKGROUND:For children with juvenile myelomonocytic leukemia (JMML) who undergo stem cell transplantation (SCT), the role of immunological interventions including withdrawal of immunosuppressive therapy (IST) and donor lymphocyte infusion (DLI) for treatment of disease recurrence remains uncertain. PROCEDURE:We analy...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24259
更新日期:2013-01-01 00:00:00
abstract::This report compares a traditional full-dose ifosfamide administration modality (24-hr hyperhydration and mesna infusion) with a simplified 9-hr hyperhydration and mesna infusion for use in outpatients. Acute ifosfamide toxicity was the same, suggesting that ifosfamide could be safely administered to outpatients, redu...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.20958
更新日期:2008-02-01 00:00:00
abstract:BACKGROUND:Hypogammaglobulinemia (hypo-IgG) is common early post-HSCT in children, occasionally necessitating long-term immunoglobulin (Ig) G replacement therapy. IgG replacement may not reduce mortality, although infectious complications are decreased PROCEDURE:Clinical data and samples from 86 children were analyzed...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25409
更新日期:2015-05-01 00:00:00
abstract::The commonly accepted definition of Fanconi anemia (FA) relying on DNA repair deficiency is submitted to a critical review starting from the early reports pointing to mitomycin C bioactivation and to the toxicity mechanisms of diepoxybutane and a group of nitrogen mustards causing DNA crosslinks in FA cells. A critica...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.25452
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:Few studies have evaluated social determinants of outcomes disparities for children with acute lymphoblastic leukemia (ALL). We investigated the association of area deprivation index (ADI), a measure of neighborhood socioeconomic disadvantage, with overall survival (OS) among children and adolescents with AL...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28525
更新日期:2020-09-01 00:00:00
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 chil...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25834
更新日期:2016-03-01 00:00:00
abstract::Spermatogonial stem cells open novel strategies for preservation of testicular tissue and fertility preservation in boys and men exposed to gonadotoxic therapies. This review provides an update on the physiology of spermatogonial stem cells in rodent and primate testes. Species-specific differences must be considered ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,评审
doi:10.1002/pbc.22002
更新日期:2009-08-01 00:00:00
abstract:BACKGROUND:This study evaluated the outcome of retinoblastoma patients, when employing a telemedicine-based twinning program in Jordan. PROCEDURE:This cohort study included patients at the King Hussein Cancer Centre (KHCC; Amman, Jordan) who received consultations for retinoblastoma from March 2003 to September 2006. ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.21489
更新日期:2008-08-01 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::Acute bilineal leukemias are rare and are commonly associated with t(9;22) and MLL abnormalities. Herein, we report a pediatric case of bilineal T/myeloid acute leukemia associated with del (9q)(q13q22) and TLX3/BCL11B fusion due to the cryptic t(5;14)(q35;32). FISH studies confirmed the TLX3/BCL11B fusion in both the...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22850
更新日期:2011-03-01 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:Physical fitness is an important determinant of quality of life (QOL) after hematopoietic stem cell transplantation. Cardiac function can influence exercise performance. The aim of this study was to assess these factors and their interrelationship. PROCEDURE:Children underwent cardiopulmonary exercise testi...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.27499
更新日期:2019-01-01 00:00:00
abstract::Limited high-quality evidence supports the management of iron deficiency anemia (IDA). To assess our institutional performance in this area, we retrospectively reviewed IDA treatment practices in 195 consecutive children referred to our center from 2006 to mid-2010. The majority of children were ≤4 years old (64%) and...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25861
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:Cancer is the second most common cause of childhood deaths in the United Kingdom and infection contributes to a quarter of all cancer-related deaths. This study aimed to estimate the risk, aetiology and outcome of bloodstream bacterial and fungal infections in children with cancer within a geographically def...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.24995
更新日期:2014-07-01 00:00:00
abstract:BACKGROUND:Abandonment of treatment is one of the toughest challenges to deal with in pediatric oncology. It leads to unnecessary mortality and morbidity in patients from low- and middle-income countries. PROCEDURE:The objective of our retrospective study was to determine the prevalence and predictors for abandonment ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26818
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:Image-guided percutaneous core needle biopsy (PCNB) is increasingly utilized to diagnose solid tumors. The objective of this study is to determine whether PCNB is adequate for modern biologic characterization of neuroblastoma. PROCEDURE:A multi-institutional retrospective study was performed by the Pediatri...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.28153
更新日期:2020-05-01 00:00:00
abstract:BACKGROUND:We report on the treatment of children and adolescents with acute lymphoblastic leukemia (ALL) in first relapse. The protocol focused on: (1) Intensive chemotherapy preceding allogeneic stem cell transplantation (SCT) in early bone marrow relapse; (2) Rotational chemotherapy in late relapse, without donor; (...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章
doi:10.1002/pbc.22946
更新日期:2011-08-01 00:00:00
abstract:BACKGROUND:Although respiratory syncytial virus (RSV) is the leading cause of pediatric lower respiratory tract infections, the burden of RSV in children with sickle cell disease (SCD) is unknown. METHODS:We conducted a retrospective, nested, case-control study of children with SCD <18 years who had respiratory viral ...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.28759
更新日期:2021-01-01 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:BACKGROUND:Neuroblastoma is characterized by heterogeneity of histology, biology, and clinical behavior. Most epidemiology studies are based on Western and Japanese populations; there are very few studies on neuroblastoma from Southeast Asia. PROCEDURE:Cases of Thai children with neuroblastoma were retrospectively rev...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23325
更新日期:2012-05-01 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::Obesity is increasing in prevalence in the United States with over 65% of adults considered overweight and 16% of children with BMI > 95 percentile. The heritability of obesity is estimated between 40% and 70%, but the genetics of obesity for most individuals are complex and involve the interaction of multiple genes a...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.23372
更新日期:2012-01-01 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:Although hematopoietic stem cell transplantation (HSCT) is offered as a curative therapy for pediatric myelodysplastic syndrome (MDS), it may cause severe complications and mortality. Several reports have shown the efficacy of immunosuppressive therapy (IST) in adult patients with refractory anemia (RA), but...
journal_title:Pediatric blood & cancer
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/pbc.22121
更新日期:2009-12-01 00:00:00
abstract:BACKGROUND:Deaths during induction chemotherapy for pediatric acute lymphoblastic leukemia (ALL) account for one-tenth of ALL-associated mortality and half of ALL treatment-related mortality. We sought to ascertain patient- and hospital-level factors associated with induction mortality. PROCEDURE:We performed a retros...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.24855
更新日期:2014-05-01 00:00:00
abstract:BACKGROUND:Radiation therapy (RT)-induced effects in children treated for low grade glioma (LGG) can result in worsening of neurologic symptoms and clinical and radiographic deterioration. Treatment for radiation-induced tumor enlargement is based on symptom control and usually involves steroids. PROCEDURE:We conducte...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.25277
更新日期:2015-02-01 00:00:00
abstract:BACKGROUND:Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal brain tumor that occurs mainly in early childhood. Although most of the tumors are characterized by inactivating mutations of the tumor suppressor gene, SMARCB1, the biological basis of its tumorigenesis and aggressiveness is still unkn...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.26398
更新日期:2017-07-01 00:00:00
abstract:BACKGROUND:Opioids are a cornerstone of palliation of pain. We sought to assess variation in opioid prescription during the last week of life among a cohort of pediatric oncology patients who died while hospitalized. PROCEDURE:We used detailed hospital administrative data from the Pediatric Health Information System (...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.21824
更新日期:2009-07-01 00:00:00
abstract:BACKGROUND:Right hemicolectomy (RHC) in patients with neuroendocrine tumors (NET) of the appendix has been advocated for tumors more than 2 cm, or with mesoappendix/vessel invasion, or with raised mitotic activity. No study demonstrated prolonged survival with RHC over appendectomy alone. The aim of this study was to c...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章,多中心研究
doi:10.1002/pbc.25823
更新日期:2016-04-01 00:00:00
abstract:BACKGROUND:Hepatoblastoma is a rare malignancy of childhood. The scarcity of adequate cell models has limited our understanding of this tumor. Here we describe and characterize a new human liver tumor cell line, Hep293TT, derived from an aggressive childhood hepatoblastoma. PROCEDURES:Hep293TT cells were established u...
journal_title:Pediatric blood & cancer
pub_type: 杂志文章
doi:10.1002/pbc.22187
更新日期:2009-12-01 00:00:00