Pediatric blood & cancer
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Pediatric blood & cancer · Dec 2016
Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes.
The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. ⋯ FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss.
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Pediatric blood & cancer · Sep 2016
Parental Grief Following the Death of a Child from Cancer: The Ongoing Odyssey.
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' perceived meanings of the experience. ⋯ The narratives highlighted that parents whose child died of cancer experience a unique and evolving form of grief and they wish to continue their bond with the deceased child. We recommend that healthcare providers and institutions incorporate support systems into a comprehensive bereavement program for families of children who die from cancer.
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Pediatric blood & cancer · Aug 2016
Response Assessment in Paediatric Phase I Trials According to RECIST Guidelines: Survival Outcomes, Patterns of Progression and Relevance of Changes in Tumour Measurements.
RECIST guidelines constitute the reference for radiological response assessment in most paediatric trials of anticancer agents. However, these criteria have not been validated in children. We evaluated the outcomes and patterns of progression of children/adolescents enrolled in phase I trials in two paediatric drug development units. ⋯ Response assessment according to RECIST correlated with OS in children/adolescents treated on phase I trials. The reduction in SLD at best response correlated with more prolonged responses. Tumour size did not constitute an optimal method to assess disease progression in one third of patients with measurable disease. Further refinement of current response assessment guidelines will enable the development of paediatric-specific radiological criteria.
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Pediatric blood & cancer · Aug 2016
Feasibility of Early Palliative Care Consultation for Children With High-Risk Malignancies.
Children with cancer suffer significant morbidity throughout therapy and often face an uncertain prognosis. Because palliative care teams can provide an additional layer of support with symptom management and communication, we conducted a prospective study assessing the feasibility of early palliative care consultation for children with high-risk malignancies. ⋯ Early palliative care consultation is feasible for children with high-risk cancer and is acceptable to children, families, and pediatric oncologists.
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Pediatric blood & cancer · Aug 2016
Multicenter StudyThrombopoietin Receptor Agonist Use in Children: Data From the Pediatric ITP Consortium of North America ICON2 Study.
Data on second-line treatment options for pediatric patients with immune thrombocytopenia (ITP) are limited. Thrombopoietin receptor agonists (TPO-RA) provide a nonimmunosuppressive option for children who require an increased platelet count. ⋯ Our results demonstrate that TPO-RA agents are being used in children with ITP of varying duration and severity. The response was similar to clinical trials, but the sustainability of response varied. Future studies need to focus on the ideal timing and rationale for these medications in pediatric patients.