RESUMEN
BACKGROUND: Passive exposure to cigarette smoke has negative effects on respiratory health. Childhood cancer survivors (CCS) are at increased risk for respiratory disease due to treatment regimens that may harm the respiratory system. The objective of this study was to assess the prevalence of parental smoking among CCS and investigate its association with respiratory outcomes. PROCEDURE: As part of the Swiss Childhood Cancer Survivor Study, between 2007 and 2022, we sent questionnaires to parents of children aged ≤16 years who had survived ≥5 years after a cancer diagnosis. Parents reported on their children's respiratory outcomes including recurrent upper respiratory tract infections (otitis media and sinusitis), asthma, and lower respiratory symptoms (chronic cough persisting >3 months, current and exercise wheeze), and on parental smoking. We used multivariable logistic regression to investigate associations between parental smoking and respiratory outcomes. RESULTS: Our study included 1037 CCS (response rate 66%). Median age at study was 12 years (interquartile range 10-14 years). Eighteen percent of mothers and 23% of fathers reported current smoking. CCS exposed to smoking mothers were more likely to have recurrent upper respiratory tract infections (OR 2.1; 95%CI 1.1-3.7) and lower respiratory symptoms (OR 2.0; 95%CI 1.1-3.7). We found no association with paternal smoking. CONCLUSIONS: A substantial proportion of CCS in Switzerland have parents who smoke. Exposure to maternal smoking was associated with higher prevalence of upper and lower respiratory problems. Healthcare providers can support families by addressing caregiver smoking behaviors and providing referrals to smoking cessation programs.
RESUMEN
OBJECTIVES: To investigate the associations of physical activity (PA) and sedentary behaviour in early childhood with asthma and reduced lung function in later childhood within a large collaborative study. DESIGN: Pooling of longitudinal data from collaborating birth cohorts using meta-analysis of separate cohort-specific estimates and analysis of individual participant data of all cohorts combined. SETTING: Children aged 0-18 years from 26 European birth cohorts. PARTICIPANTS: 136 071 individual children from 26 cohorts, with information on PA and/or sedentary behaviour in early childhood and asthma assessment in later childhood. MAIN OUTCOME MEASURE: Questionnaire-based current asthma and lung function measured by spirometry (forced expiratory volume in 1 s (FEV1), FEV1/forced vital capacity) at age 6-18 years. RESULTS: Questionnaire-based and accelerometry-based PA and sedentary behaviour at age 3-5 years was not associated with asthma at age 6-18 years (PA in hours/day adjusted OR 1.01, 95% CI 0.98 to 1.04; sedentary behaviour in hours/day adjusted OR 1.03, 95% CI 0.99 to 1.07). PA was not associated with lung function at any age. Analyses of sedentary behaviour and lung function showed inconsistent results. CONCLUSIONS: Reduced PA and increased sedentary behaviour before 6 years of age were not associated with the presence of asthma later in childhood.
Asunto(s)
Asma , Ejercicio Físico , Conducta Sedentaria , Humanos , Niño , Asma/epidemiología , Asma/fisiopatología , Adolescente , Masculino , Preescolar , Europa (Continente)/epidemiología , Femenino , Lactante , Acelerometría , Estudios Longitudinales , Encuestas y Cuestionarios , Volumen Espiratorio Forzado , Espirometría , Recién Nacido , Capacidad Vital , Cohorte de NacimientoRESUMEN
BACKGROUND: The adverse effects of high air pollution levels on childhood lung function are well-known. Limited evidence exists on the effects of moderate exposure levels during early life on childhood lung function. We investigated the association of exposure to moderate air pollution during pregnancy, infancy, and preschool time with lung function at school age in a Swiss population-based study. METHODS: Fine-scale spatiotemporal model estimates of particulate matter with a diameter <2.5 µm (PM2.5) and nitrogen dioxide (NO2) were linked with residential address histories. We compared air pollution exposures within different time windows (whole pregnancy, first, second, and third trimester of pregnancy, first year of life, preschool age) with forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) measured cross-sectionally using linear regression models adjusted for potential confounders. RESULTS: We included 2182 children, ages 6-17 years. Prenatal air pollution exposure was associated with reduced lung function at school age. In children aged 12 years, per 10 µg·m-3 increase in PM2.5 during pregnancy, FEV1 was 55 mL lower (95% CI -84 to -25 mL) and FVC 62 mL lower (95% CI -96 to -28 mL). Associations were age-dependent since they were stronger in younger and weaker in older children. PM2.5 exposure after birth was not associated with reduced lung function. There was no association between NO2 exposure and lung function. CONCLUSION: In utero lung development is most sensitive to air pollution exposure, since even modest PM2.5 exposure during the prenatal time was associated with reduced lung function, most prominent in younger children.
RESUMEN
OBJECTIVES: In our study, we aimed to characterise adult childhood cancer survivors (ACCS), assess their health issues, gauge health-related quality of life (HRQOL) and evaluate visit satisfaction. DESIGN: Prospective cohort study using data from clinical visits and questionnaires. SETTING: Interdisciplinary follow-up programme for ACCS based on the long-term follow-up (LTFU) guidelines of the Children's Oncology Group and overseen by internists in two Swiss hospitals. PARTICIPANTS: ACCS attending our LTFU clinics between April 2017 and January 2022 were eligible. INTERVENTIONS: We documented medical history, current health status and assessed HRQOL using Short Form-36 V.2, comparing it with Swiss general population (SGP) norms (T mean=50, SD=10; age stratified). 3 months post visit, a feedback questionnaire was distributed. MAIN RESULTS: Among 102 ACCS (mean age: 32 years (range: 18-62 years), 68% women), 43 had no prior follow-up (36 ACCS>28 years, 7 ACCS≤28 years). A notable 94% had health issues, affecting an average of 6.1 (SD=3.3) organ systems. HRQOL was lower in ACCS>28 years than the SGP>28 years (physical: 44.8 (SD=11.65) vs 49.3 (SD=10.29), p=0.016; mental: 44.4 (SD=13.78) vs 50.53 (SD=9.92), p=0.004). Older ACCS (>28 years) reported inferior physical (44.8 vs 50.1 (SD=9.30), p=0.017) and mental HRQOL (44.4 vs 50.3 (SD=7.20), p=0.009) than younger ACCS. The majority of respondents reported high levels of satisfaction with the consultation, exceeding 90%. CONCLUSION: ACCS attending LTFU clinics face diverse health issues impacting multiple organ systems and exhibit lower HRQOL compared with the SGP. Thus, internist-led LTFU clinics are crucial for optimising follow-up care.
Asunto(s)
Supervivientes de Cáncer , Neoplasias , Calidad de Vida , Humanos , Femenino , Masculino , Supervivientes de Cáncer/psicología , Estudios Prospectivos , Adulto , Suiza , Persona de Mediana Edad , Adolescente , Adulto Joven , Neoplasias/psicología , Neoplasias/terapia , Encuestas y Cuestionarios , Satisfacción del Paciente , Estudios de Seguimiento , Estado de SaludRESUMEN
Psychological distress is an important and frequent health problem. The Brief Symptom Inventory (BSI) allows screening for psychological distress in clinical, general and research populations. We aimed to provide normative data for the BSI and the BSI-18 for the Swiss general population: We 1) present psychometric properties, 2) develop a Swiss T-standardization and validate it using a clinical sample, 3) describe psychological distress in the Swiss general population and the clinical sample, and 4) compare the means and T-standardized scores of the Swiss general population to different German norm populations. Using a cross-sectional study design, we invited a representative sample of the Swiss general population aged 18-75 years to the study. A sample of psychotherapy outpatients had competed the BSI before start of their therapy. We calculated scores for the nine scales of the BSI (three of them constitute the BSI-18), the T-standardization and the following BSI indices: Global Severity Index (GSI), Positive Symptom Total (PST), Positive Symptom Distress Index (PSDI), and Caseness (reaching T≥63 on the GSI or T≥63 on at least two of the scales). A total of 1238 general population participants completed the BSI (41.8% male; mean age 48.9 years). The BSI had good psychometric properties. The Swiss T-standardization showed good validity when applied in the clinical sample. Females reached a significantly higher GSI score than males (p<0.001). Older participants (p = 0.026), those with higher education (p <0.001), and those employed or retired (p<0.001) reached lower scores than participants aged 18-25 years, those with compulsory schooling, and unemployed participants, respectively. A total of 18.1% (CI: 16.0-20.5) participants of the general population and 75.2% (CI: 73.7-76.7) of the psychotherapy patients were considered cases with psychological distress. Our study presents detailed normative data for the BSI and the BSI-18 based on a representative sample of the Swiss general population. This information will be helpful for clinical applications and research in the Swiss and international context.
Asunto(s)
Distrés Psicológico , Psicometría , Humanos , Persona de Mediana Edad , Adulto , Masculino , Femenino , Suiza/epidemiología , Anciano , Adolescente , Psicometría/métodos , Estudios Transversales , Adulto Joven , Estrés Psicológico/diagnóstico , Encuestas y CuestionariosRESUMEN
STUDY QUESTION: What is the prevalence of infertility and ectopic pregnancies among individuals with primary ciliary dyskinesia (PCD)? SUMMARY ANSWER: We found that 39 of 50 men (78%) and 72 of 118 women (61%) with PCD were infertile and that women with PCD had an increased risk of ectopic pregnancies (7.6 per 100 pregnancies, 95% CI 4.7-12.2). WHAT IS KNOWN ALREADY: PCD is a heterogeneous multiorgan disease caused by mutations in genes required for the function and structure of motile cilia. Previous studies identified a link between PCD and infertility, but original data on prevalence of infertility and risk of ectopic pregnancies, the use and efficacy of medically assisted reproduction (MAR), and the association of fertility with PCD genotype are extremely limited. STUDY DESIGN SIZE DURATION: We performed a cross-sectional survey about fertility within the Living with PCD study (formerly COVID-PCD). Living with PCD is an international, online, participatory study that collects information directly from people with PCD. People with PCD of any age from anywhere in the world can participate in the study. At the time of the survey, 482 adults with PCD were registered within the Living with PCD study. PARTICIPANTS/MATERIALS SETTING METHODS: We sent a questionnaire on fertility on 12 July 2022, to all participants older than 18 years enrolled in the Living with PCD study. Responses were collected until 8 March 2023. The fertility questionnaire covered topics related to pregnancy attempts, use of MAR, and pregnancy outcomes. Data were collected via the Research Electronic Data Capture (REDCap) platform. We defined infertility as failure to achieve a clinical pregnancy after 12 months or use of MAR for at least one pregnancy. MAIN RESULTS AND THE ROLE OF CHANCE: In total, 265 of 482 adult participants (55%) completed the fertility questionnaire. Among 168 adults who had tried to conceive, 39 of 50 men (78%) and 72 of 118 women (61%) were infertile. Of the infertile men, 28 had tried MAR, and 17 of them (61%) fathered a child with the help of MAR. Among infertile women, 59 had used MAR, and 41 of them (69%) became pregnant with the help of MAR. In our population, women with PCD showed a relatively high risk of ectopic pregnancies: 1 in 10 women who became pregnant had at least one ectopic pregnancy and 7.6% of pregnancies were ectopic (95% CI 4.7-12.2). We evaluated the association between fertility and affected PCD genes in 46 individuals (11 men, 35 women) with available genetic and fertility information, and found differences between genotypes, e.g. all five women with a mutation in CCDC40 were infertile and all five with DNAH11 were fertile. LIMITATIONS REASONS FOR CAUTION: The study has limitations, including potential selection bias as people experiencing problems with fertility might be more likely to fill in the questionnaire, which may have influenced our prevalence estimates. We were unable to validate clinical data obtained from participant self-reports owing to the anonymous study design, which is likely to lead to recall bias. WIDER IMPLICATIONS OF THE FINDINGS: The study underlines the need for addressing infertility in routine PCD care, with a focus on informing individuals with PCD about their increased risk. It emphasizes the utility and efficacy of MAR in PCD-related infertility. Additionally, women attempting conception should be made aware of the increased risk of ectopic pregnancies and seek systematic early consultation to confirm an intrauterine pregnancy. Fertility, efficacy of MAR, and risk for adverse pregnancy outcomes differ between people with PCD-depending on genotypes-and close monitoring and support might be needed from fertility specialists to increase chances of successful conception. STUDY FUNDING/COMPETING INTERESTS: Our research was funded by the Swiss National Science Foundation, Switzerland (SNSF 320030B_192804), the Swiss Lung Association, Switzerland (2021-08_Pedersen), and we also received support from the PCD Foundation, USA; the Verein Kartagener Syndrom und Primäre Ciliäre Dyskinesie, Germany; the PCD Support UK, UK; and PCD Australia, Australia. M. Goutaki received funding from the Swiss National Science Foundation, Switzerland (PZ00P3_185923). B. Maitre participates in the RaDiCo-DCP funded by INSERM France. The study authors participate in the BEAT-PCD Clinical Research Collaboration supported by the European Respiratory Society. All authors declare no conflict of interest. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov ID NCT04602481.
RESUMEN
Since the publication of the European Respiratory Society (ERS) Task Force reports on the management of preschool wheezing in 2008 and 2014, a large body of evidence has accumulated suggesting the clinical phenotypes that were proposed, episodic (viral) wheezing and multiple-trigger wheezing, do not relate to underlying airway pathology and may not help determine response to treatment. Specifically, using clinical phenotypes alone may no longer be appropriate, and new approaches that can be used to inform clinical care are needed for future research. This ERS Task Force reviewed the literature published after 2008 related to preschool wheezing and has suggested the criteria used to define wheezing disorders in preschool children should include age of diagnosis (0 to <6â years), confirmation of wheezing on at least one occasion, and more than one episode of wheezing ever.Furthermore, diagnosis and management may be improved by identifying treatable traits, including inflammatory biomarkers (blood eosinophils, aeroallergen sensitisation) associated with type-2 immunity and differential response to inhaled corticosteroids, lung function parameters, and airway infection. However, more comprehensive use of biomarkers/treatable traits in predicting the response to treatment requires prospective validation. There is evidence that specific genetic traits may help guide management, but these must be adequately tested. In addition, the Task Force identified an absence of caregiver-reported outcomes, caregiver/self-management options, and features that should prompt specialist referral for this age group. Priorities for future research include a focus on identifying i) mechanisms driving preschool wheezing, ii) biomarkers of treatable traits and efficacy of interventions in those without allergic sensitisation/eosinophilia, iii) the need to include both objective outcomes and caregiver-reported outcomes in clinical trials, iv) the need for a suitable action plan for children with preschool wheezing and v) a definition of severe/difficult-to-treat preschool wheezing.
RESUMEN
BACKGROUND: Childhood cancer survivors may experience psychological distress due to the disease, cancer treatments, and potential late effects. Limited knowledge exists regarding longitudinal changes in psychological distress after childhood cancer. We aimed to determine changes in psychological distress over time and explore determinants of changes. METHODS: The Swiss Childhood Cancer Survivor Study collected data at baseline (2007-2009) and follow-up (2010-2012). Psychological distress was measured using the Brief Symptom Inventory 18 (BSI-18), including three symptom scales (somatization, depression, anxiety) and an overall distress index (Global Severity Index, GSI). Sum-scores were T-standardized (mean = 50; standard deviation [SD] = 10). Survivors with a score ≥57 on the GSI or two symptom scales were classified as cases with distress. We used linear mixed effects regression to identify potential sociodemographic and clinical determinants of change in psychological distress. RESULTS: We analyzed 696 survivors at baseline (mean age = 24 years [SD = 4], 49% females, mean time since diagnosis = 16 years [SD = 4]). On follow-up (2.4 years, SD = 1), 317 survivors were analyzed, including 302 participants with repeated measures. We found that 13% (39/302) were cases at baseline, and 25% (76/302) were cases on follow-up. Those older at study and longer since diagnosis, females, diagnosed with central nervous system (CNS) tumors, and those reporting late effects were more likely to experience higher levels of distress. Females and unemployed are at higher risk for developing or persisting psychological distress than males and those who are employed or in training. CONCLUSION: We observed an increase in psychological distress score over time, with higher proportion of psychological distress on follow-up. Anticipatory guidance and screening should be implemented in regular follow-up care.
Asunto(s)
Supervivientes de Cáncer , Neoplasias , Distrés Psicológico , Humanos , Masculino , Femenino , Supervivientes de Cáncer/psicología , Neoplasias/psicología , Adulto , Estudios de Seguimiento , Niño , Adolescente , Adulto Joven , Estudios Longitudinales , Suiza/epidemiología , Estrés Psicológico/etiología , Estrés Psicológico/epidemiología , Calidad de Vida , PronósticoRESUMEN
Childhood cancer survivors (CCS) require specialized follow-up throughout their lifespan to prevent or manage late effects of cancer treatment. Knowing the size and structure of the population of CCS is crucial to plan interventions. In this scoping review we reviewed studies that reported prevalence of CCS in Europe. We searched Medline, Web of Science, and Embase using permutations of terms referring to childhood, cancer, survivors, prevalence, registries, and Europe. We followed PRISMA-ScR guidelines to select studies and The Joanna Briggs Institute Prevalence Critical Appraisal Tool to evaluate their quality. From 979 unique studies published between 1989 and 2022, 12 were included. Limited-duration prevalence (LDP) for all childhood cancers, assessed in three studies using counting method, varied between 450 and 1240 persons per million. Complete prevalence (CP) of survivors of any childhood cancer except skin carcinomas, reported in three studies using observed data complemented with modelled data for the unobserved period, varied between 730 and 1110 persons per million. CP of survivors of an embryonal tumour was estimated by completeness index method in six studies. In four of them CP ranged from 48 to 95 persons per million for all embryonal tumours, while CP for those occurring in central nervous system was 43 per million in one study and CP for rhabdomyosarcoma was 17 per million in another. Information on prevalence of CCS in Europe is fragmented and inconsistent. The large variations in LDP and CP estimates were linked to differences in data availability, the selection of populations, prevalence measure, statistical method, incidence period, index date, age at diagnosis and prevalence, cancer types, sex, and, for LDP, also the length of follow-up. Standardisation of methodology and reporting are needed to systematically monitor and compare CCS prevalence in Europe and provide data to help address survivors' needs.
RESUMEN
Clonal hematopoiesis of indeterminate potential (CHIP) describes recurrent somatic gene mutations in the blood of healthy individuals, associated with higher risk for hematological malignancies and higher all-cause mortality by cardiovascular disease. CHIP increases with age and is more common in adult patients after chemotherapy or radiation for cancer. Furthermore, in some adult patients undergoing autologous stem cell transplantation (ASCT) or thereafter, CHIP has been identified. In children and adolescents, it remains unclear how cellular stressors such as cytotoxic therapy influence the incidence and expansion of CHIP. We conducted a retrospective study on 33 pediatric patients mostly with solid tumors undergoing ASCT for presence of CHIP. We analyzed CD34+ selected peripheral blood stem cell grafts after several cycles of chemotherapy, prior to cell infusion, by next-generation sequencing including 18 "CHIP-genes". Apart from a somatic variant in TP53 in one patient no other variants indicative of CHIP were identified. As a CHIP-unrelated finding, germline variants in CHEK2 and in ATM were identified in two and four patients, respectively. In conclusion, we could not detect "typical" CHIP variants in our cohort of pediatric cancer patients undergoing ASCT. However, more studies with larger patient numbers are necessary to assess if chemotherapy in the pediatric setting contributes to an increased CHIP incidence and at what time point.
RESUMEN
PURPOSE: Childhood cancer and its treatment can cause damage to the musculoskeletal system. We aimed to determine the incidence and prevalence of musculoskeletal health conditions (MSHC) in survivors, and to investigate differences by cancer-related characteristics. METHODS: We used data from the Childhood Cancer Registry and the Swiss Childhood Cancer Survivor Study, including survivors (≥5 years since diagnosis; diagnosed 1976-2015 at <20 years of age) aged ≥15 years at study. Cumulative incidence and prevalence of MSHCs (osteoporosis, limb length discrepancy, limited joint mobility, bone/joint pain, scoliosis, changes to chest/ribs and amputation) were calculated from self-reported data. RESULTS: We included 2645 survivors (53% men; median age 24 years, range 15-59 years). Prevalence and cumulative incidence of any MSHC was 21% and 26%, respectively. Incidence rate for any MSHC was 15.6/1000 person-years. Scoliosis (8%), bone/joint pain (7%) and limited joint mobility (7%) were the most prevalent MSHC. MSHC co-occurred with other health conditions in 87% of survivors. We found increased rates of MSHC in women (RR = 1.4, 95%CI: 1.2-1.7), bone tumour survivors (RR = 6.0, 95%CI: 4.5-7.9), survivors older at diagnosis (11-15 years: RR = 1.8, 95%CI: 1.5-2.3), after a relapse (RR = 1.5, 95%CI: 1.3-1.9), treatment with surgery (RR = 1.2, 95%CI: 1.0-1.5), chemotherapy (RR = 1.4, 95%CI: 1.1-1.8) or stem cell transplantation (RR = 1.6, 95%CI: 1.0-2.5), and more recent year of diagnosis (2011-2015: RR = 4.3, 95%CI: 2.8-6.8). CONCLUSION: MSHCs are prevalent in survivors, the risk is increasing in younger survivor cohorts, and MSHCs usually occur in multimorbid survivors. Strengthening of rehabilitation services and appropriate referrals are needed to mitigate the effects of the cancer and cancer treatment.
Asunto(s)
Supervivientes de Cáncer , Enfermedades Musculoesqueléticas , Neoplasias , Humanos , Adolescente , Supervivientes de Cáncer/estadística & datos numéricos , Femenino , Masculino , Adulto Joven , Incidencia , Suiza/epidemiología , Prevalencia , Adulto , Enfermedades Musculoesqueléticas/epidemiología , Enfermedades Musculoesqueléticas/etiología , Neoplasias/epidemiología , Persona de Mediana Edad , Niño , Sistema de RegistrosRESUMEN
BACKGROUND: Myeloid neoplasms account for 50% of cases of pediatric leukemias in infants. Approximately 25%-50% of patients with newborn leukemia have cutaneous extramedullary disease (EMD). In less than 10% of patients, aleukemic leukemia cutis or isolated extramedullary disease with cutaneous involvement (cEMD) occurs when skin lesions appear prior to bone marrow involvement and systemic symptoms. Interestingly, in acute myeloid leukemia with cutaneous EMD (AML-cEMD) and cEMD, spontaneous remissions have been reported. METHOD: This is a multicentric retrospective cohort study aiming to describe characteristics, treatment, and outcome of infants with either cEMD or presence of cutaneous disease with involvement of the bone marrow (AML-cEMD). This study included patients born between 1990 and 2018 from Italy, the Netherlands, Switzerland, and the United States, diagnosed between 0 and 6 months of life with cEMD or AML-cEMD. Descriptive statistics, Fisher's exact test, Kaplan-Meier method, and log rank test were applied. RESULTS: The cohort consisted of n = 50 patients, including 42 AML-cEMD and eight cEMD patients. The most common genetic mutation found was a KMT2A rearrangement (n = 26, 52%). Overall 5-year event-free survival (EFS) and overall survival (OS) were 66% [confidence interval (CI): 51-78] and 75% [CI: 60-85], respectively. In two patients, complete spontaneous remission occurred without any therapy. Central nervous system (CNS) involvement was found in 25% of cEMD patients. No difference in outcomes was observed between the AML-cEMD and cEMD groups, but none of the latter patients included in the study died. KMT2A rearrangements were not associated with poorer prognosis. CONCLUSION: In the largest cohort to date, our study describes the characteristics of infants with cutaneous involvement of myeloid neoplasms including cytomolecular findings and survival rates. Further prospective biologic and clinical studies of these infants with myeloid neoplasms will be required to individualize therapy for this rare patient population.
Asunto(s)
Leucemia Mieloide Aguda , Neoplasias Cutáneas , Humanos , Estudios Retrospectivos , Femenino , Leucemia Mieloide Aguda/mortalidad , Leucemia Mieloide Aguda/patología , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/terapia , Lactante , Masculino , Recién Nacido , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/mortalidad , Neoplasias Cutáneas/terapia , Neoplasias Cutáneas/genética , Estudios de Seguimiento , Tasa de Supervivencia , PronósticoRESUMEN
BACKGROUND: Pediatric-onset inflammatory brain diseases are a group of potentially life-threatening central nervous system disorders. Overall, pediatric-onset inflammatory brain diseases are rare and therefore difficult to study. Patient registries are well suited to study the natural history of (rare) diseases and have markedly advanced the knowledge on pediatric-onset inflammatory brain diseases in other countries. Following their example, we established a national pediatric-onset inflammatory brain disease registry in Switzerland (Swiss-Ped-IBrainD). AIMS: The Registry aims to describe epidemiology, demographics, diagnostics, management, and treatment, since these areas remain understudied in Switzerland. Additionally, we want to promote research by fostering the knowledge exchange between study centers and setting up studies such as national quality of life surveys. We further aim to facilitate the access to national and international studies for patients with a pediatric-onset inflammatory brain disease living and/or treated in Switzerland. METHODS: The Swiss-Ped-IBrainD is a multicentric, population-based, observational cohort study (IRB number: 2019-00377) collaborating with 11 neuropediatric centers in Switzerland. Patient screening, information and recruitment is mainly conducted by the local principal investigators. The data collection is organized centrally by the Executive Office of the registry. The collected data is purely observational. Medical records are the primary data source. All patients who have been diagnosed with a pediatric-onset inflammatory brain disease since 2005 are eligible. We aim to include all pediatric-onset inflammatory brain disease patients living and/or treated in Switzerland who meet the inclusion criteria. Considering existing literature and our single-center experience we anticipate 300-400 eligible patients. STATUS: Currently, all 11 neuropediatric centers have been initiated and are recruiting. As of the first of May 2023, we have identified 275 eligible participants and obtained informed consent from 101 patients and/or families. None of the informed patients and/or families have refused participation.
Asunto(s)
Encefalopatías , Calidad de Vida , Humanos , Niño , Suiza/epidemiología , Sistema de Registros , Recolección de Datos , Estudios Observacionales como AsuntoRESUMEN
PURPOSE: Childhood cancer survivors, in particular those treated with radiation therapy, are at high risk of long-term iatrogenic events. The prediction of risk of such events is mainly based on the knowledge of the radiation dose received to healthy organs and tissues during treatment of childhood cancer diagnosed decades ago. We aimed to set up a standardized organ dose table to help former patients and clinicians in charge of long-term follow-up clinics. METHODS AND MATERIALS: We performed whole body dosimetric reconstruction for 2646 patients from 12 European countries treated between 1941 and 2006 (median, 1976). Most plannings were 2- or 3-dimensional. A total of 46% of patients were treated using Cobalt 60, and 41%, using a linear accelerator. The median prescribed dose was 27.2 Gy (IQ1-IQ3, 17.6-40.0 Gy). A patient-specific voxel-based anthropomorphic phantom with more than 200 anatomic structures or substructures delineated as a surrogate of each subject's anatomy was used. The radiation therapy was simulated with a treatment planning system based on available treatment information. The radiation dose received by any organ of the body was estimated by extending the treatment planning system dose calculation to the whole body, by type and localization of childhood cancer. RESULTS: The integral dose and normal tissue doses to most of the 23 considered organs increased between the 1950s and 1970s and decreased or plateaued thereafter. Whatever the organ considered, the type of childhood cancer explained most of the variability in organ dose. The country of treatment explained only a small part of the variability. CONCLUSIONS: The detailed dose estimates provide very useful information for former patients or clinicians who have only limited knowledge about radiation therapy protocols or techniques, but who know the type and site of childhood cancer, sex, age, and year of treatment. This will allow better prediction of the long-term risk of iatrogenic events and better referral to long-term follow-up clinics.
RESUMEN
BACKGROUND: Epidemiological studies often rely on self-reported health problems and validation greatly improves study quality. In a study of late effects after childhood cancer, we validated self-reported cardiovascular problems by contacting general practitioners (GPs). This paper describes: (a) the feasibility of this approach; and (b) the agreement between survivor-reports and reports from their GP. METHODS: The Swiss Childhood Cancer Survivor Study (SCCSS) contacts all childhood cancer survivors registered in the Swiss Childhood Cancer Registry since 1976 who survived at least 5 years from cancer diagnosis. We validated answers of all survivors who reported a cardiovascular problem in the questionnaire. Reported cardiovascular problems were hypertension, arrhythmia, congestive heart failure, myocardial infarction, angina pectoris, stroke, thrombosis, and valvular problems. In the questionnaire, we further asked survivors to provide a valid address of their GP and a consent for contact. We sent case-report forms to survivors' GPs and requested information on cardiovascular diagnoses of their patients. To determine agreement between information reported by survivors and GPs, we calculated Cohen's kappa (κ) coefficients for each category of cardiovascular problems. RESULTS: We used questionnaires from 2172 respondents of the SCCSS. Of 290 survivors (13% of 2172) who reported cardiovascular problems, 166 gave consent to contact their GP and provided a valid address. Of those, 135 GPs (81%) replied, and 128 returned the completed case-report form. Survivor-reports were confirmed by 54/128 GPs (42%). Of the 54 GPs, 36 (28% of 128) confirmed the problems as reported by the survivors; 11 (9% of 128) confirmed the reported problem(s) and gave additional information on more cardiovascular outcomes; and seven GPs (5% of 128) confirmed some, but not all cardiovascular problems. Agreement between GPs and survivors was good for stroke (κ = 0.79), moderate for hypertension (κ = 0.51), arrhythmias (κ = 0.41), valvular problems (κ = 0.41) and thrombosis (κ = 0.56), and poor for coronary heart disease (κ = 0.15) and heart failure (κ = 0.32). CONCLUSIONS: Despite excellent GP compliance, it was found unfeasible to validate self-reported cardiovascular problems via GPs because they do not serve as gatekeepers in the Swiss health care system. It is thus necessary to develop other validation methods to improve the quality of patient-reported outcomes.
Asunto(s)
Supervivientes de Cáncer , Médicos Generales , Insuficiencia Cardíaca , Hipertensión , Neoplasias , Accidente Cerebrovascular , Trombosis , Humanos , Niño , Autoinforme , Estudios de Factibilidad , Neoplasias/complicaciones , Neoplasias/epidemiologíaRESUMEN
PURPOSE: Langerhans cell histiocytosis (LCH) is a rare disease characterized by dysregulated proliferation of myeloid marrow progenitors and subsequent organ infiltration. While LCH is associated with a favorable prognosis, some survivors may develop chronic health conditions (CHC) because of the disease. In this study, we aimed to assess the spectrum and prevalence of CHC among LCH survivors compared with siblings and identify factors associated with the development of CHC. METHODS: The Swiss Childhood Cancer Survivor Study sent questionnaires to all ≥ 5-year LCH survivors registered in the Swiss Childhood Cancer Registry and diagnosed between 1976 and 2015. Siblings also received similar questionnaires. We compared CHC prevalence between LCH survivors and siblings and used logistic regression to identify determinants of CHC. RESULTS: A total of 123 LCH survivors participated in the study, with a response rate of 69%. Median time since diagnosis was 13 years (interquartile range 9-20). Among LCH survivors, 59% had at least one CHC. Cardiovascular (13% vs. 6%), endocrine (15% vs. 2%), musculoskeletal (22% vs. 13%), and digestive (15% vs. 8%) CHC were more common among LCH survivors compared to siblings (all p < 0.05). Factors most strongly associated with the occurrence of CHC were multisystem LCH, multifocal bone involvement, and involvement of the pituitary gland. CONCLUSIONS: More than half of long-term LCH survivors suffered from one or more CHC and were affected considerably more than siblings. IMPLICATIONS FOR CANCER SURVIVORS: LCH survivors in follow-up care should be screened especially for cardiovascular, endocrine, musculoskeletal, and digestive conditions.
RESUMEN
Childhood, adolescent, and young adult (CAYA) cancer survivors are at risk of pulmonary dysfunction. Current follow-up care guidelines are discordant. Therefore, the International Late Effects of Childhood Cancer Guideline Harmonization Group established and convened a panel of 33 experts to develop evidence-based surveillance guidelines. We critically reviewed available evidence regarding risk factors for pulmonary dysfunction, types of pulmonary function testing, and timings of surveillance, then we formulated our recommendations. We recommend that CAYA cancer survivors and healthcare providers are aware of reduced pulmonary function risks and pay vigilant attention to potential symptoms of pulmonary dysfunction, especially among survivors treated with allogeneic haematopoietic stem cell transplantation, thoracic radiotherapy, and thoracic surgery. Based on existing limited evidence and current lack of interventions, our panel recommends pulmonary function testing only for symptomatic survivors. Since scarce existing evidence informs our recommendation, we highlight the need for prospective collaborative studies to address pulmonary function knowledge gaps among CAYA cancer survivors.
RESUMEN
PURPOSE: Low-acuity pediatric emergency department (PED) visits are frequent in high-income countries and have a negative impact on patient care at the individual and health system levels. Knowing what drives low-acuity PED visits is crucial to inform adaptations in health care delivery. We aimed to identify factors associated with low-acuity PED visits in Switzerland, including socioeconomic status, demographic features, and medical resources of families. METHODS: We conducted a prospective, questionnaire-based study in the PEDs of two Swiss tertiary care hospitals, Bern and Lausanne. We invited all consecutive children and their caregiver attending the PED during data collection times representative of the overall PED consultation structure (e.g. day/night, weekdays/weekends) to complete a questionnaire on demographic features, socioeconomic status, and medical resources. We collected medical and administrative data about the visit and defined low-acuity visits as those meeting all of the following criteria: (1) triage category 4 or 5 on the Australasian Triage Scale, (2) no imaging or laboratory test performed, and (3) discharge home. We used a binary multiple logistic regression model to identify factors associated with low-acuity visits. RESULTS: We analysed 778 PED visits (September 2019 to July 2020). Most children visiting our PEDs had a designated primary care provider (92%), with only 6% not having seen them during the last year. Fifty-five per cent of caregivers had asked for medical advice before coming to the PED. The proportion of low-acuity visits was 58%. Low-acuity visits were associated with caregiver's difficulties paying bills (aOR 2.6, 95% CI 1.6 - 4.4), having already visited a PED in the last 6 months (aOR 1.7, 95% CI 1.1 - 2.5) but not with parental education status, nor parental country of birth, parental employment status or absence of family network. CONCLUSION: Economic precariousness is an important driver for low-acuity PED visits in Switzerland, a high-income country with compulsory health coverage where most children have a designated primary care provider and a regular pediatric follow-up. Primary care providers and PEDs should screen families for economic precariousness and offer anticipatory guidance and connect those in financial need to social support.
Asunto(s)
Visitas a la Sala de Emergencias , Servicio de Urgencia en Hospital , Niño , Humanos , Centros de Atención Terciaria , Suiza , Estudios Transversales , Estudios Prospectivos , Encuestas y Cuestionarios , Hospitales PediátricosRESUMEN
BACKGROUND: Childhood cancer survivors are at risk of subsequent gliomas and meningiomas, but the risks beyond age 40 years are uncertain. We quantified these risks in the largest ever cohort. METHODS: Using data from 69,460 5-year childhood cancer survivors (diagnosed 1940-2008), across Europe, standardized incidence ratios (SIRs) and cumulative incidence were calculated. RESULTS: In total, 279 glioma and 761 meningioma were identified. CNS tumour (SIR: 16.2, 95% CI: 13.7, 19.2) and leukaemia (SIR: 11.2, 95% CI: 8.8, 14.2) survivors were at greatest risk of glioma. The SIR for CNS tumour survivors was still 4.3-fold after age 50 (95% CI: 1.9, 9.6), and for leukaemia survivors still 10.2-fold after age 40 (95% CI: 4.9, 21.4). Following cranial radiotherapy (CRT), the cumulative incidence of a glioma in CNS tumour survivors was 2.7%, 3.7% and 5.0% by ages 40, 50 and 60, respectively, whilst for leukaemia this was 1.2% and 1.7% by ages 40 and 50. The cumulative incidence of a meningioma after CRT in CNS tumour survivors doubled from 5.9% to 12.5% between ages 40 and 60, and in leukaemia survivors increased from 5.8% to 10.2% between ages 40 and 50. DISCUSSION: Clinicians following up survivors should be aware that the substantial risks of meningioma and glioma following CRT are sustained beyond age 40 and be vigilant for symptoms.
Asunto(s)
Neoplasias del Sistema Nervioso Central , Glioma , Leucemia , Neoplasias Meníngeas , Meningioma , Neoplasias Primarias Secundarias , Humanos , Adolescente , Adulto , Persona de Mediana Edad , Meningioma/etiología , Meningioma/complicaciones , Factores de Riesgo , Neoplasias Primarias Secundarias/epidemiología , Neoplasias Primarias Secundarias/etiología , Neoplasias del Sistema Nervioso Central/epidemiología , Glioma/epidemiología , Sobrevivientes , Leucemia/epidemiología , Europa (Continente)/epidemiología , Neoplasias Meníngeas/epidemiología , IncidenciaRESUMEN
PURPOSE: Reported prevalence of cancer-related fatigue (CRF) among childhood cancer survivors (CCS) varies widely, and evidence on factors associated with CRF among CCS is limited. We aimed to investigate the prevalence of CRF and its associated factors among adult CCS in Switzerland. METHODS: In a prospective cohort study, we invited adult CCS who survived at least 5 years since last cancer diagnosis, and were diagnosed when age 0-20 years and treated at Inselspital Bern between 1976 and 2015 to complete two fatigue-measuring instruments: the Checklist Individual Strength subjective fatigue subscale (CIS8R; increased fatigue 27-34, severe fatigue ≥ 35) and the numerical rating scale (NRS; moderate fatigue 4-6, severe fatigue 7-10). We collected information about previous cancer treatment and medical history, and calculated ß coefficients for the association between CIS8R/NRS fatigue scores and potential determinants using multivariable linear regression. RESULTS: We included 158 CCS (participation rate: 30%) with a median age at study of 33 years (interquartile range 26-38). Based on CIS8R, 19% (N = 30) of CCS reported increased fatigue, yet none reported severe fatigue. CRF was associated with female sex, central nervous system (CNS) tumors, sleep disturbance, and endocrine disorders. Lower CRF levels were observed among CCS age 30-39 years compared to those younger. CONCLUSIONS: A considerable proportion of adult CCS reported increased levels of CRF. IMPLICATIONS FOR CANCER SURVIVORS: CCS who are female and < 30 years old, have a history of CNS tumor, report sleep disturbance, or have an endocrine disorder should be screened for CRF.