Purpose
The successful transition of childhood cancer survivors from pediatric- to adult-focused long-term follow-up care is crucial and can be a critical period. Knowledge of current transition ...practices, especially regarding barriers and facilitators perceived by survivors and health care professionals, is important to develop sustainable transition processes and implement them into daily clinical practice. We performed a systematic review with the aim of assessing transition practices, readiness tools, and barriers and facilitators.
Methods
We searched three databases (PubMed, Embase/Ovid, CINAHL) and included studies published between January 2000 and January 2020. We performed this review according to the PRISMA guidelines and registered the study protocol on PROSPERO; two reviewers independently extracted the content of the included studies.
Results
We included 26 studies: six studies described current transition practices, six assessed transition readiness tools, and 15 assessed barriers and facilitators to transition.
Conclusion
The current literature describing transition practices is limited and overlooks adherence to follow-up care as a surrogate marker of transition success. However, the literature provides deep insight into barriers and facilitators to transition and theoretical considerations for the assessment of transition readiness. We showed that knowledge and education are key facilitators to transition that should be integrated into transition practices tailored to the individual needs of each survivor and the possibilities and limitations of each country’s health care system.
Implications for Cancer Survivors
The current knowledge on barriers and facilitators on transition should be implemented in clinical practice to support sustainable transition processes.
High-dose methotrexate (HD-MTX) is used in the treatment of different childhood cancers, including leukemia, the most common cancer type and is commonly defined as an intravenous dose of at least ...1 g/m
body surface area per application. A systematic review on late effects on different organs due to HD-MTX is lacking.
We conducted a systematic literature search in PubMed, including studies published in English or German between 1985 and 2020. The population of each study had to consist of at least 75% childhood cancer survivors (CCSs) who had completed the cancer treatment at least twelve months before late effects were assessed and who had received HD-MTX. The literature search was not restricted to specific cancer diagnosis or organ systems at risk for late effects. We excluded case reports, case series, commentaries, editorial letters, poster abstracts, narrative reviews and studies only reporting prevalence of late effects. We followed PRISMA guidelines, assessed the quality of the eligible studies according to GRADE criteria and registered the protocol on PROSPERO (ID: CRD42020212262).
We included 15 out of 1731 identified studies. Most studies included CCSs diagnosed with acute lymphoblastic leukemia (n = 12). The included studies investigated late effects of HD-MTX on central nervous system (n = 10), renal (n = 2) and bone health (n = 3). Nine studies showed adverse outcomes in neuropsychological testing in exposed compared to non-exposed CCSs, healthy controls or reference values. No study revealed lower bone density or worse renal function in exposed CCSs. As a limitation, the overall quality of the studies per organ system was low to very low, mainly due to selection bias, missing adjustment for important confounders and low precision.
CCSs treated with HD-MTX might benefit from neuropsychological testing, to intervene early in case of abnormal results. Methodological shortcomings and heterogeneity of the tests used made it impossible to determine the most appropriate test. Based on the few studies on renal function and bone health, regular screening for dysfunction seems not to be justified. Only screening for neurocognitive late effects is warranted in CCSs treated with HD-MTX.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background
A cancer diagnosis during childhood or adolescence causes nursery and school absences to various degrees. Attending school and meeting classmates gives many children and adolescents some ...normality back. Nevertheless, it can cause fears and concerns among the teachers. We are currently lacking information about the fears and needs of teachers having a child or adolescent diagnosed with cancer or with a cancer history in their classes. With this study, we aim to close this knowledge gap and assess the teachers’ fears, worries and information needs having a child or adolescent diagnosed with cancer in the class to develop a suitable information tool (flyer).
Methods
We performed an online survey including teachers covering all grades from nursery to vocational school within the catchment area of our hospital. The survey included separate questions for experience with students still receiving active treatment and those in follow-up care. Answer options included tick boxes and open-ended questions, which we grouped thematically. We used descriptive analysis to describe the survey findings, resulting in a newly developed flyer.
Results
In total 358 teachers participated in the survey, 80% were female, 63% worked in nursery or primary school. One quarter (26%) had experience with a student diagnosed with cancer. Most teachers with (81%) and without (85%) experience reported at least one concern. The top three concerns reported were: (1) how to inform the class, (2) the resilience of the student and (3) how to deal with the student and his or her family. The teachers preferred oral information by physicians or parents and written information equally. Information on resilience, guidelines with an emergency situation, and the need for cancer-specific information were considered important by about 75-94% of the teachers.
Conclusion
Most teachers reported concerns, which we cover in a newly developed information flyer. However, such a flyer cannot replace individual communication between health care professionals and teachers. The identified concerns are likely to be transferable to other school systems and countries.
Abstract
Childhood and adolescent cancer survivors are at risk for chronic medical conditions. Longitudinal studies help to understand their development and course. We hypothesize that collecting ...follow-up data according to the modified CTCAE criteria and embedded in regular care, is feasible and results in a rich database. We recruited 50 Swiss survivors treated at our institution between 1992 and 2015, who completed their treatment and are still alive. Information on cancer diagnosis, treatment, and medical conditions from follow-up visits, graded according to the modified CTCAE criteria, were added in the database. We described the cohort, assessed the prevalence of medical conditions at the most recent visits and the time needed for data entry. Survivors had a median age of 10 years at diagnosis with 16 years of follow-up. 94% of survivors suffered from at least one medical condition. We registered 25 grade 3 or 4 conditions in 18 survivors. The time needed for data entry at enrollment was < 60 min in most survivors and much less for follow-up visits. Standardized assessment of medical conditions is feasible during regular clinical care. The database provides longitudinal real-time data to be used for clinical care, survivor education and research.
Diffuse intrinsic pontine glioma (DIPG) is a fatal brain cancer that arises in the brainstem of children, with no effective treatment and near 100% fatality. The failure of most therapies can be ...attributed to the delicate location of these tumors and to the selection of therapies on the basis of assumptions that DIPGs are molecularly similar to adult disease. Recent studies have unraveled the unique genetic makeup of this brain cancer, with nearly 80% found to harbor a p.Lys27Met histone H3.3 or p.Lys27Met histone H3.1 alteration. However, DIPGs are still thought of as one disease, with limited understanding of the genetic drivers of these tumors. To understand what drives DIPGs, we integrated whole-genome sequencing with methylation, expression and copy number profiling, discovering that DIPGs comprise three molecularly distinct subgroups (H3-K27M, silent and MYCN) and uncovering a new recurrent activating mutation affecting the activin receptor gene ACVR1 in 20% of DIPGs. Mutations in ACVR1 were constitutively activating, leading to SMAD phosphorylation and increased expression of the downstream activin signaling targets ID1 and ID2. Our results highlight distinct molecular subgroups and novel therapeutic targets for this incurable pediatric cancer.
Celotno besedilo
Dostopno za:
DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Survival of children and adolescents diagnosed with central nervous system (CNS) tumors massively improved over the last decades due to better diagnostics, treatment, and supportive care. However, ...morbidity is still the highest of all cancer entities in this age group with neurocognitive late-effects being one of the most severe.
With this systematic review, we aim to summarize interventions designed to prevent or improve neurocognitive late-effects in CNS tumor patients.
We searched PubMed on August 16
2022 and included publications studying interventions for neurocognitive late-effects in pediatric and adolescent patients and survivors diagnosed with a CNS tumor. We included any form of neurocognitive intervention during treatment or following treatment completion. We considered all types of studies except for expert opinions and case reports.
The literature search resulted in 735 publications. We included 43 publications in the full text screening and 14 met our inclusion criteria. Of those, two assessed the impact of pharmacological interventions, three of exercise interventions, five of online cognitive training, and four assessed behavioral interventions. Different neuropsychological test batteries and imaging were used to measure the impact of the respective interventions. Most studies showed a positive impact of the interventions in single to several of the subtests used.
We found several intervention studies indicating improvement of neurocognitive problems in children and adolescent CNS tumor survivors. In this population exercise interventions or online cognitive training might mitigate or improve neurocognitive late-effects.
•Nationwide cohort study spanning over two decades including 2,960 incident cases.•Combined dispersion modeling and land-use regression to model ambient NO2.•Traffic-related air pollution is ...associated with an increased risk of childhood AML.•Analyses of time-lagged exposure suggest relevant window is close to diagnosis.•Indications of an increased risk also observed for non-Hodgkin lymphoma.
Motor vehicle exhaust is a major contributor to air pollution, and exposure to benzene or other carcinogenic components may increase cancer risks. We aimed to investigate the association between traffic-related air pollution and risk of childhood cancer in a nationwide cohort study in Switzerland. We identified incident cases from the Swiss Childhood Cancer Registry diagnosed < 16 years of age between 1990 and 2015 and linked them probabilistically with the census-based Swiss National Cohort study. We developed land use regression models to estimate annual mean ambient levels of nitrogen dioxide (NO2) and benzene outside 1.4 million children’s homes. We used risk-set sampling to facilitate the analysis of time-varying exposure and fitted conditional logistic regression models adjusting for neighborhood socio-economic position, level of urbanization, and background ionizing radiation. We included 2,960 cancer cases in the analyses. The adjusted hazard ratios (HR) and 95% confidence intervals for exposure to NO2 per 10 μg/m3 were 1.00 (95%-CI 0.88–1.13) for acute lymphoblastic leukemia (ALL) and 1.31 (95%-CI 1.00–1.71) for acute myeloid leukemia (AML). Using exposure lagged by 1 to 5 years instead of current exposure attenuated the effect for AML. The adjusted HR for exposure to benzene per 1 μg/m3 was 1.03 (95%-CI 0.86–1.23) for ALL and 1.29 (95%-CI 0.86–1.95) for AML. We also observed increased HRs for other diagnostic groups, notably non-Hodgkin lymphoma. Our study adds to the existing evidence that exposure to traffic-related air pollution is associated with an increased risk of childhood leukemia, particularly AML.
The successful transition of childhood cancer survivors (CCSs) from pediatric to adult long-term follow-up care is a critical phase, and determining the right time point can be challenging. We ...assessed the feasibility of the use of existing transition readiness tools in the context of the Swiss health care system, assessed partly transition readiness in Swiss CCSs, and compared our findings with Canadian CCSs for which these tools were originally developed.
We officially translated the Cancer Worry Scale (CWS) and Self-Management Skill Scale (SMSS) into German and integrated them into this cross-sectional study. We included CCSs attending the long-term follow-up (LTFU) clinic in the Division of Oncology-Hematology, Department of Pediatrics, Kantonsspital Aarau. We used descriptive statistics to describe transition readiness.
We randomly recruited 50 CCSs aged ≥18 years at participation. The CCSs had a median CWS score of 62 (interquartile range 55-71), indicating a moderate level of cancer-related worry. Despite high self-management skills, some answers showed a dependency of CCSs on their parents. Our experience shows that the CWS and SMSS are easy for Swiss CCSs to use, understand, and complete. The interpretation of the results must take differences in health care systems between countries into account.
The translated CWS and SMSS are appropriate additional measures to assess transition readiness in CCSs. These scales can be used longitudinally to find the individual time point for transition and the completion by CCSs enables the health care team to individualize the transition process and to support the CCSs according to their individual needs.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Recent studies have revealed that the majority of pediatric low-grade astrocytomas (PLGA) harbor the BRAF-KIAA1549 (B-K) fusion gene resulting in constitutive activation of the RAS/MAPK pathway. ...However, the clinical significance of this genetic alteration is yet to be determined. We aimed to test the prognostic role of the B-K fusion in progression of incompletely resected PLGA.
We retrospectively identified 70 consecutive patients with incompletely resected "clinically relevant" PLGA. We added 76 tumors diagnosed at our institution between 1985 and 2010 as controls. We examined BRAF alterations by reverse transcriptase PCR, FISH, and single-nucleotide polymorphism array analysis and correlated that with progression-free survival (PFS).
Overall, 60% of tumors were B-K fusion positive. All patients with B-K fused PLGA are still alive. Five-year PFS was 61% ± 8% and 18% ± 8% for fusion positive and negative patients, respectively (P = 0.0004). B-K fusion resulted in similarly significant favorable PFS for patients who received chemotherapy. Multivariate analysis revealed that B-K fusion was the most significant favorable prognostic factor in incompletely resected PLGA and was independent of location, pathology, and age. In vitro, BRAF overexpression resulted in growth arrest associated with DNA damage (γH2AX expression). Five-year PFS was 68% ± 15% and 0% for patients with B-K fused and γH2AX-expressing PLGA versus negative tumors (P = 0.001).
These data suggest that B-K fusion confers a less aggressive clinical phenotype on PLGA and may explain their tendency to growth arrest. Combined analysis of B-K fusion and γH2AX expression can determine prognosis and may be a powerful tool to tailor therapy for these patients.
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