Publications by authors named "Eva Frey"

19 Publications

  • Page 1 of 1

Clinical considerations for the treatment of secondary differentiated thyroid carcinoma in childhood cancer survivors.

Eur J Endocrinol 2020 Sep;183(3):P1-P10

Department of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, the Netherlands.

The incidence of differentiated thyroid carcinoma (DTC) has increased rapidly over the past several years. Thus far, the only conclusively established risk factor for developing DTC is exposure to ionizing radiation, especially when the exposure occurs in childhood. Since the number of childhood cancer survivors (CCS) is increasing due to improvements in treatment and supportive care, the number of patients who will develop DTC after surviving childhood cancer (secondary thyroid cancer) is also expected to rise. Currently, there are no recommendations for management of thyroid cancer specifically for patients who develop DTC as a consequence of cancer therapy during childhood. Since complications or late effects from prior cancer treatment may elevate the risk of toxicity from DTC therapy, the medical history of CCS should be considered carefully in choosing DTC treatment. In this paper, we emphasize how the occurrence and treatment of the initial childhood malignancy affects the medical and psychosocial factors that will play a role in the diagnosis and treatment of a secondary DTC. We present considerations for clinicians to use in the management of patients with secondary DTC, based on the available evidence combined with experience-based opinions of the authors.
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http://dx.doi.org/10.1530/EJE-20-0237DOI Listing
September 2020

Evidence-based recommendations for the organization of long-term follow-up care for childhood and adolescent cancer survivors: a report from the PanCareSurFup Guidelines Working Group.

J Cancer Surviv 2019 Oct 8;13(5):759-772. Epub 2019 Aug 8.

Department of Paediatric Oncology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.

Purpose: To facilitate the implementation of long-term follow-up (LTFU) care and improve equality of care for childhood, adolescent, and young adult (CAYA) cancer survivors, the PanCareSurFup Guidelines Working Group developed evidence-based recommendations for the organization of LTFU.

Methods: We established an international multidisciplinary guideline panel. A systematic review of the literature published from 1999 to 2017 was completed to answer six clinical questions. The guideline panel reviewed the identified studies, developed evidence summaries, appraised the quality of the body of evidence, and formulated recommendations based on the evidence, expert opinions, and the need to maintain flexibility of application across different healthcare systems.

Results: We provide strong recommendations based on low level evidence and expert opinions, regarding organization of LTFU care, personnel involved in LTFU care, components of LTFU care and start of LTFU care. We recommend that risk-adapted LTFU care provided under the guidance of a cancer survivorship expert service or cancer centre should be available and accessible for all CAYA cancer survivors throughout their lifespan.

Conclusion: Despite the weak levels of evidence, successful and effective implementation of these recommendations should improve LTFU, thereby leading to better access to appropriate healthcare services and an improvement in health outcomes for CAYA cancer survivors.

Implications For Cancer Survivors: To improve health outcomes and quality of survivorship of current and future survivors, continued age-adapted education of survivors about the cancer, its treatment, risk of late effects, importance of health behaviours, and necessity of LTFU is important along the cancer and survivorship trajectory.
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http://dx.doi.org/10.1007/s11764-019-00795-5DOI Listing
October 2019

The PanCareSurFup consortium: research and guidelines to improve lives for survivors of childhood cancer.

Eur J Cancer 2018 11 1;103:238-248. Epub 2018 Oct 1.

Department of Oncology, Great Ormond Street Hospital for Children NHS Foundation Trust, London WCIN3JH, UK.

Background: Second malignant neoplasms and cardiotoxicity are among the most serious and frequent adverse health outcomes experienced by childhood and adolescent cancer survivors (CCSs) and contribute significantly to their increased risk of premature mortality. Owing to differences in health-care systems, language and culture across the continent, Europe has had limited success in establishing multi-country collaborations needed to assemble the numbers of survivors required to clarify the health issues arising after successful cancer treatment. PanCareSurFup (PCSF) is the first pan-European project to evaluate some of the serious long-term health risks faced by survivors. This article sets out the overall rationale, methods and preliminary results of PCSF.

Methods: The PCSF consortium pooled data from 13 cancer registries and hospitals in 12 European countries to evaluate subsequent primary malignancies, cardiac disease and late mortality in survivors diagnosed between ages 0 and 20 years. In addition, PCSF integrated radiation dosimetry to sites of second malignancies and to the heart, developed evidence-based guidelines for long-term care and for transition services, and disseminated results to survivors and the public.

Results: We identified 115,596 individuals diagnosed with cancer, of whom 83,333 were 5-year survivors and diagnosed from 1940 to 2011. This single data set forms the basis for cohort analyses of subsequent malignancies, cardiac disease and late mortality and case-control studies of subsequent malignancies and cardiac disease in 5-year survivors.

Conclusions: PCSF delivered specific estimates of risk and comprehensive guidelines to help survivors and care-givers. The expected benefit is to provide every European CCS with improved access to care and better long-term health.
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http://dx.doi.org/10.1016/j.ejca.2018.08.017DOI Listing
November 2018

Long-term survivors of childhood cancer: cure and care-the Erice Statement (2006) revised after 10 years (2016).

J Cancer Surviv 2018 10 26;12(5):647-650. Epub 2018 Jun 26.

Klinik für Kinder und Jugendmedizin, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Germany.

Purpose: The number of persons who have successfully completed treatment for a cancer diagnosed during childhood and who have entered adulthood is increasing over time, and former patients will become aging citizens.

Methods: Ten years ago, an expert panel met in Erice, Italy, to produce a set of principles concerning the cure and care of survivors of childhood and adolescent cancer. The result was the Erice Statement (Haupt et al. Eur J Cancer 43(12):1778-80, 2007) that was translated into nine languages. Ten years on, it was timely to review, and possibly revise, the Erice Statement in view of the changes in paediatric oncology and the number and results of international follow-up studies conducted during the intervening years.

Results: The long-term goal of the cure and care of a child with cancer is that he/she becomes a resilient and autonomous adult with optimal health-related quality of life, accepted in society at the same level as his/her age peers. "Cure" refers to cure from the original cancer, regardless of any potential for, or presence of, remaining disabilities or side effects of treatment. The care of a child with cancer should include complete and honest information for parents and the child.

Conclusions And Implication For Cancer Survivors: Some members of the previous expert panel, as well as new invited experts, met again in Erice to review the Erice Statement, producing a revised version including update and integration of each of the ten points. In addition, a declaration has been prepared, by the Childhood Cancer International Survivors Network in Dublin on October 2016 (see Annex 1).
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http://dx.doi.org/10.1007/s11764-018-0701-0DOI Listing
October 2018

The role of epistatic interactions underpinning resistance to parasitic Varroa mites in haploid honey bee (Apis mellifera) drones.

J Evol Biol 2018 06 17;31(6):801-809. Epub 2018 Apr 17.

Molecular Ecology, Institute of Biology/Zoology, Martin-Luther-University Halle-Wittenberg, Halle an der Saale, Germany.

The Red Queen hypothesis predicts that host-parasite coevolutionary dynamics can select for host resistance through increased genetic diversity, recombination and evolutionary rates. However, in haplodiploid organisms such as the honeybee (Apis mellifera), models suggest the selective pressure is weaker than in diploids. Haplodiploid sex determination, found in A. mellifera, can allow deleterious recessive alleles to persist in the population through the diploid sex with negative effects predominantly expressed in the haploid sex. To overcome these negative effects in haploid genomes, epistatic interactions have been hypothesized to play an important role. Here, we use the interaction between A. mellifera and the parasitic mite Varroa destructor to test epistasis in the expression of resistance, through the inhibition of parasite reproduction, in haploid drones. We find novel loci on three chromosomes which explain over 45% of the resistance phenotype. Two of these loci interact only additively, suggesting their expression is independent of each other, but both loci interact epistatically with the third locus. With drone offspring inheriting only one copy of the queen's chromosomes, the drones will only possess one of two queen alleles throughout the years-long lifetime of the honeybee colony. Varroa, in comparison, completes its highly inbred reproductive cycle in a matter of weeks, allowing it to rapidly evolve resistance. Faced with the rapidly evolving Varroa, a diversity of pathways and epistatic interactions for the inhibition of Varroa reproduction could therefore provide a selective advantage to the high levels of recombination seen in A. mellifera. This allows for the remixing of phenotypes despite a fixed queen genotype.
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http://dx.doi.org/10.1111/jeb.13271DOI Listing
June 2018

The virulent, emerging genotype B of Deformed wing virus is closely linked to overwinter honeybee worker loss.

Sci Rep 2017 07 12;7(1):5242. Epub 2017 Jul 12.

Institute for Biology, Martin-Luther-University Halle-Wittenberg, Hoher Weg 8, 06120, Halle (Saale), Germany.

Bees are considered to be threatened globally, with severe overwinter losses of the most important commercial pollinator, the Western honeybee, a major concern in the Northern Hemisphere. Emerging infectious diseases have risen to prominence due to their temporal correlation with colony losses. Among these is Deformed wing virus (DWV), which has been frequently linked to colony mortality. We now provide evidence of a strong statistical association between overwintering colony decline in the field and the presence of DWV genotype-B (DWV-B), a genetic variant of DWV that has recently been shown to be more virulent than the original DWV genotype-A. We link the prevalence of DWV-B directly to a quantitative measure of overwinter decline (workforce mortality) of honeybee colonies in the field. We demonstrate that increased prevalence of virus infection in individual bees is associated with higher overwinter mortality. We also observed a substantial reduction of infected colonies in the spring, suggesting that virus-infected individuals had died during the winter. Our findings demonstrate that DWV-B, plus possible A/B recombinants exhibiting DWV-B at PCR primer binding sites, may be a major cause of elevated overwinter honeybee loss. Its potential emergence in naïve populations of bees may have far-reaching ecological and economic impacts.
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http://dx.doi.org/10.1038/s41598-017-05596-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5507926PMC
July 2017

Childhood adversity impacts on brain subcortical structures relevant to depression.

J Psychiatr Res 2017 03 19;86:58-65. Epub 2016 Nov 19.

Center for Integrative Psychiatry, University of Lübeck, Lübeck, Germany.

Childhood adversity plays an important role for development of major depressive disorder (MDD). There are differences in subcortical brain structures between patients with MDD and healthy controls, but the specific impact of childhood adversity on such structures in MDD remains unclear. Thus, aim of the present study was to investigate whether childhood adversity is associated with subcortical volumes and how it interacts with a diagnosis of MDD and sex. Within the ENIGMA-MDD network, nine university partner sites, which assessed childhood adversity and magnetic resonance imaging in patients with MDD and controls, took part in the current joint mega-analysis. In this largest effort world-wide to identify subcortical brain structure differences related to childhood adversity, 3036 participants were analyzed for subcortical brain volumes using FreeSurfer. A significant interaction was evident between childhood adversity, MDD diagnosis, sex, and region. Increased exposure to childhood adversity was associated with smaller caudate volumes in females independent of MDD. All subcategories of childhood adversity were negatively associated with caudate volumes in females - in particular emotional neglect and physical neglect (independently from age, ICV, imaging site and MDD diagnosis). There was no interaction effect between childhood adversity and MDD diagnosis on subcortical brain volumes. Childhood adversity is one of the contributors to brain structural abnormalities. It is associated with subcortical brain abnormalities that are relevant to psychiatric disorders such as depression.
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http://dx.doi.org/10.1016/j.jpsychires.2016.11.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5564511PMC
March 2017

Parasites and Pathogens of the Honeybee (Apis mellifera) and Their Influence on Inter-Colonial Transmission.

PLoS One 2015 9;10(10):e0140337. Epub 2015 Oct 9.

Institute of Biology, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany; Department of Zoology and Entomology, University of Pretoria, Pretoria, South Africa.

Pathogens and parasites may facilitate their transmission by manipulating host behavior. Honeybee pathogens and pests need to be transferred from one colony to another if they are to maintain themselves in a host population. Inter-colony transmission occurs typically through honeybee workers not returning to their home colony but entering a foreign colony ("drifting"). Pathogens might enhance drifting to enhance transmission to new colonies. We here report on the effects infection by ten honeybee viruses and Nosema spp., and Varroa mite infestation on honeybee drifting. Genotyping of workers collected from colonies allowed us to identify genuine drifted workers as well as source colonies sending out drifters in addition to sink colonies accepting them. We then used network analysis to determine patterns of drifting. Distance between colonies in the apiary was the major factor explaining 79% of drifting. None of the tested viruses or Nosema spp. were associated with the frequency of drifting. Only colony infestation with Varroa was associated with significantly enhanced drifting. More specifically, colonies with high Varroa infestation had a significantly enhanced acceptance of drifters, although they did not send out more drifting workers. Since Varroa-infested colonies show an enhanced attraction of drifting workers, and not only those infected with Varroa and its associated pathogens, infestation by Varroa may also facilitate the uptake of other pests and parasites.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0140337PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4599887PMC
June 2016

Survivorship after childhood cancer: PanCare: a European Network to promote optimal long-term care.

Eur J Cancer 2015 Jul 6;51(10):1203-11. Epub 2015 May 6.

German Childhood Cancer Registry, University Medical Centre Mainz, Germany.

Survival after childhood cancer has improved substantially over recent decades. Although cancer in childhood is rare increasingly effective treatments have led to a growing number of long-term survivors. It is estimated that there are between 300,000 and 500,000 childhood cancer survivors in Europe. Such good survival prospects raise important questions relating to late effects of treatment for cancer. Research has shown that the majority will suffer adverse health outcomes and premature mortality compared with the general population. While chronic health conditions are common among childhood cancer survivors, each specific type of late effect is very rare. Long-term effects must be considered particularly when addressing complex multimodality treatments, and taking into account the interaction between aspects of treatment and genotype. The PanCare Network was set up across Europe in order to effectively answer many of these questions and thereby improve the care and quality of life of survivors. The need for a structured long-term follow-up system after childhood cancer has been recognised for some time and strategies for implementation have been developed, first nationally and then trans-nationally, across Europe. Since its first meeting in Lund in 2008, the goal of the PanCare Network has been to coordinate and implement these strategies to ensure that every European survivor of childhood and adolescent cancer receives optimal long-term care. This paper will outline the structure and work of the PanCare Network, including the results of several European surveys, the start of two EU-funded projects and interactions with relevant stakeholders and related projects.
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http://dx.doi.org/10.1016/j.ejca.2015.04.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5916870PMC
July 2015

The views of European clinicians on guidelines for long-term follow-up of childhood cancer survivors.

Pediatr Blood Cancer 2015 02 8;62(2):322-328. Epub 2014 Nov 8.

Newcastle University, Newcastle upon Tyne, UK.

Background: Evidence-based guidelines are needed to guide effective long-term follow-up (LTFU) of childhood cancer survivors (CCS) at risk of late adverse effects (LAEs). We aimed to ascertain the use of LTFU guidelines throughout Europe, and seek views on the need for pan-European LTFU guidelines.

Procedures: One expert clinician from each of 44 European countries was invited to participate in an online survey. Information was sought regarding the use and content of LTFU guidelines in the respondent's centre and country, and their views about developing pan-European LTFU guidelines.

Results: Thirty-one countries (70%) responded, including 24 of 26 full EU countries (92%). LTFU guidelines were implemented nationally in 17 countries (55%). All guidelines included recommendations about physical LAEs, specific risk groups and frequency of surveillance, and the majority about psychosocial LAEs (70%), and healthy lifestyle promotion (65%). A minority of guidelines described recommendations about transition to age-appropriate LTFU services (22%), where LTFU should be performed (22%) and by whom (30%). Most respondents (94%) agreed on the need for pan-European LTFU guidelines, specifically including recommendations about surveillance for specific physical LAEs (97%), action to be taken if a specific LAE is detected (90%), minimum requirements for LTFU (93%), transition and health promotion (both 87%).

Conclusions: Guidelines are not universally used throughout Europe. However, there is strong support for developing pan-European LTFU guidelines for CCS. PanCareSurFup (www.pancare.eu) will collaborate with partners to develop such guidelines, including recommendations for hitherto relatively neglected topics, such as minimum LTFU requirements, transition and health promotion. Pediatr Blood Cancer 2015;62:322-328. © 2014 Wiley Periodicals, Inc.
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http://dx.doi.org/10.1002/pbc.25310DOI Listing
February 2015

Autumn invasion rates of Varroa destructor (Mesostigmata: Varroidae) into honey bee (Hymenoptera: Apidae) colonies and the resulting increase in mite populations.

J Econ Entomol 2014 Apr;107(2):508-15

The honey bee parasite Varroa destructor Anderson & Trueman can disperse and invade honey bee colonies by attaching to "drifting" and "robbing" honey bees that move into nonnatal colonies. We quantified the weekly invasion rates and the subsequent mite population growth from the end of July to November 2011 in 28 honey bee colonies kept in two apiaries that had high (HBD) and low (LBD) densities of neighboring colonies. At each apiary, half (seven) of the colonies were continuously treated with acaricides to kill all Varroa mites and thereby determine the invasion rates. The other group of colonies was only treated before the beginning of the experiment and then left untreated to record Varroa population growth until a final treatment in November. The numbers of bees and brood cells of all colonies were estimated according to the Liebefeld evaluation method. The invasion rates varied among individual colonies but revealed highly significant differences between the study sites. The average invasion rate per colony over the entire 3.5-mo period ranged from 266 to 1,171 mites at the HBD site compared with only 72 to 248 mites at the LBD apiary. In the untreated colonies, the Varroa population reached an average final infestation in November of 2,082 mites per colony (HBD) and 340 mites per colony (LBD). All colonies survived the winter; however, the higher infested colonies lost about three times more bees compared with the lower infested colonies. Therefore, mite invasion and late-year population growth must be considered more carefully for future treatment concepts in temperate regions.
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http://dx.doi.org/10.1603/ec13381DOI Listing
April 2014

The ENIGMA Consortium: large-scale collaborative analyses of neuroimaging and genetic data.

Brain Imaging Behav 2014 Jun;8(2):153-82

Imaging Genetics Center, Institute for Neuroimaging and Informatics, Keck School of Medicine, University of Southern California, 2001 N. Soto Street, Los Angeles, CA, 90033, USA,

The Enhancing NeuroImaging Genetics through Meta-Analysis (ENIGMA) Consortium is a collaborative network of researchers working together on a range of large-scale studies that integrate data from 70 institutions worldwide. Organized into Working Groups that tackle questions in neuroscience, genetics, and medicine, ENIGMA studies have analyzed neuroimaging data from over 12,826 subjects. In addition, data from 12,171 individuals were provided by the CHARGE consortium for replication of findings, in a total of 24,997 subjects. By meta-analyzing results from many sites, ENIGMA has detected factors that affect the brain that no individual site could detect on its own, and that require larger numbers of subjects than any individual neuroimaging study has currently collected. ENIGMA's first project was a genome-wide association study identifying common variants in the genome associated with hippocampal volume or intracranial volume. Continuing work is exploring genetic associations with subcortical volumes (ENIGMA2) and white matter microstructure (ENIGMA-DTI). Working groups also focus on understanding how schizophrenia, bipolar illness, major depression and attention deficit/hyperactivity disorder (ADHD) affect the brain. We review the current progress of the ENIGMA Consortium, along with challenges and unexpected discoveries made on the way.
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http://dx.doi.org/10.1007/s11682-013-9269-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4008818PMC
June 2014

Transitional care of a childhood cancer survivor to adult services: facilitating the process of individual access to different models.

Curr Opin Support Palliat Care 2013 Sep;7(3):309-13

St Anna Children's Hospital, Medical University Vienna, Vienna, Austria.

Purpose Of Review: In the past 20 years, several papers have been published concerning healthcare transition from pediatric after-care to long-term follow-up in adult health service for childhood cancer survivors. Initially, the papers were optimistic and included guidelines and models of care. However, after this optimistic beginning, more recent research has found that transitional care is not a successful process.

Recent Findings: Although the number of papers regarding transition in the past 2 years is few, they include revealing results about barriers and facilitators of the transition process.

Summary: There is some evidence that joint pediatric/adult transition clinics are more successful in providing transitional care. The use of tools to assess individual psychological factors is important for the transition process and could lead to more individualized models of transition.
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http://dx.doi.org/10.1097/SPC.0b013e32836484bbDOI Listing
September 2013

Activation and interruption of the reproduction of Varroa destructor is triggered by host signals (Apis mellifera).

J Invertebr Pathol 2013 May 30;113(1):56-62. Epub 2013 Jan 30.

University of Hohenheim, Apicultural State Institute, 70593 Stuttgart, Germany.

The reproductive cycle of the parasitic mite Varroa destructor is closely linked to the development of the honey bee host larvae. Using a within colony approach we introduced phoretic Varroa females into brood cells of different age in order to analyze the capacity of certain stages of the honey bee larva to either activate or interrupt the reproduction of Varroa females. Only larvae within 18 h (worker) and 36 h (drones), respectively, after cell capping were able to stimulate the mite's oogenesis. Therewith we could specify for the first time the short time window where honey bee larvae provide the signals for the activation of the Varroa reproduction. Stage specific volatiles of the larval cuticle are at least part of these activation signals. This is confirmed by the successful stimulation of presumably non-reproducing mites to oviposition by the application of a larval extract into the sealed brood cells. According to preliminary quantitative GC-MS analysis we suggest certain fatty acid ethyl esters as candidate compounds. If Varroa females that have just started with egg formation are transferred to brood cells containing host larvae of an elder stage two-thirds of these mites stopped their oogenesis. This confirms the presence of an additional signal in the host larvae allowing the reproducing mites to adjust their own reproductive cycle to the ontogenetic development of the host. From an adaptive point of view that sort of a stop signal enables the female mite to save resources for a next reproductive cycle if the own egg development is not sufficiently synchronized with the development of the host. The results presented here offer the opportunity to analyze exactly those host stages that have the capacity to activate or interrupt the Varroa reproduction in order to identify the crucial host signals.
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http://dx.doi.org/10.1016/j.jip.2013.01.007DOI Listing
May 2013

Three QTL in the honey bee Apis mellifera L. suppress reproduction of the parasitic mite Varroa destructor.

Ecol Evol 2011 Dec;1(4):451-8

Varroa destructor is a highly virulent ectoparasitic mite of the honey bee Apis mellifera and a major cause of colony losses for global apiculture. Typically, chemical treatment is essential to control the parasite population in the honey bee colony. Nevertheless a few honey bee populations survive mite infestation without any treatment. We used one such Varroa mite tolerant honey bee lineage from the island of Gotland, Sweden, to identify quantitative trait loci (QTL) controlling reduced mite reproduction. We crossed a queen from this tolerant population with drones from susceptible colonies to rear hybrid queens. Two hybrid queens were used to produce a mapping population of haploid drones. We discriminated drone pupae with and without mite reproduction, and screened the genome for potential QTL using a total of 216 heterozygous microsatellite markers in a bulk segregant analysis. Subsequently, we fine mapped three candidate target regions on chromosomes 4, 7, and 9. Although the individual effect of these three QTL was found to be relatively small, the set of all three had significant impact on suppression of V. destructor reproduction by epistasis. Although it is in principle possible to use these loci for marker-assisted selection, the strong epistatic effects between the three loci complicate selective breeding programs with the Gotland Varroa tolerant honey bee stock.
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http://dx.doi.org/10.1002/ece3.17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3287329PMC
December 2011

Gastrointestinal stromal tumours in children and young adults: a clinicopathologic series with long-term follow-up from the database of the Cooperative Weichteilsarkom Studiengruppe (CWS).

Eur J Cancer 2011 Jul 11;47(11):1692-8. Epub 2011 Apr 11.

Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz, Austria.

Background: Studies on gastrointestinal stromal tumours (GIST) in the paediatric population are limited to case reports or small case series.

Patients And Methods: We conducted a retrospective study to describe the long-term outcome of children and adolescents with GIST registered in the database of the Cooperative Weichteilsarkom Studiengruppe (CWS).

Results: Sixteen patients (female, n = 11) were identified. Median age at diagnosis was 13.5 years. In four female patients presence of thoracic masses in addition to GIST led to the diagnosis of complete or incomplete Carney triad. Three female patients had metastatic disease at diagnosis, the remaining thirteen GIST were localised. The stomach was the most common primary site of the tumour, followed by the small bowel and colon/abdomen. All patients underwent tumour resection. Receptor tyrosine kinase inhibitors (RTKI) were administered in five patients. With a median follow-up of 96 months all patients are alive, nine of them in first CR. Four female patients developed local or distant recurrence; three of them achieved second CR and one a PR. Two individuals have extensive progressive (n = 1) or stable (n = 1) disease. Estimated progression-free survival at 5 years is 0.63 (95%CI: 0.50-0.86).

Conclusions: Although long-term overall survival is favourable, approximately 30 percent of patients develop disease progression. International cooperation in registration, tissue collection and molecular studies are required to obtain reliable data on the clinical course of these rare tumours in the paediatric population. Biological studies are a prerequisite for initiation of studies with RTKI.
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http://dx.doi.org/10.1016/j.ejca.2011.03.014DOI Listing
July 2011

Natural course of HCV infection in childhood cancer survivors.

Support Care Cancer 2010 Nov 27;18(11):1413-20. Epub 2009 Oct 27.

Department of Pediatric Hematology, Oncology, and Bone Marrow Transplantation, Giannina Gaslini Children's Hospital, Largo Gerolamo Gaslini 5, 16147 Genoa, Italy.

Goals Of Work: To describe the course of hepatitis C in a cohort of 105 survivors after childhood cancer.

Patients And Methods: Data on chemo/radiotherapy, clinical status, serial alanine aminotransferase (ALT) evaluation, and virological parameters after the end of treatment were collected for each patient. Liver biopsies, when performed, were centrally evaluated by a pathologist.

Main Results: All patients were alive at the end of follow-up and did not show hepatic insufficiency. ALT evaluation along the entire follow-up showed a moderate (87%) or a remarkable (13%) cytolytic pattern. Young age at diagnosis, hematopoietic stem cell transplantation, and duration of infection significantly correlate with a worse hepatic activity. Type of tumor and chemo and/or radiotherapy regimens did not influence the pattern of hepatic cytolysis. Liver biopsy, centrally reviewed in 30% of the cohort, showed one case of cirrhosis and mild fibrosis in 71% of the group. Higher degrees of fibrosis did not seem to be related to any exposition to chemo/radiotherapy but correlated significantly with the more remarkable cytolytic course.

Conclusions: The outcome of hepatitis C in our patients is comparable to the one described in European cohorts of adult cancer survivors and perinatally infected subjects. Nevertheless, progression to high degrees of hepatic damage has to be monitored by a careful follow-up.
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http://dx.doi.org/10.1007/s00520-009-0763-7DOI Listing
November 2010

Subcutaneous anti-D globulin application is a safe treatment option of immune thrombocytopenia in children.

Ann Hematol 2010 Apr 13;89(4):415-8. Epub 2009 Oct 13.

Division of Hemato-Oncology, St. Anna Children's Hospital, Kinderspitalgasse 6, 1090, Vienna, Austria.

Subcutaneous (sc) administration of anti-D seems to offer the same efficacy as intravenous administration but with less side effects. Here we report our experience with sc anti-D for pediatric immune thrombocytopenia (ITP). A total of 12 children with a median age of 11.2 years had been treated by sc anti-D. They received a median of 2 sc anti-D applications (range 1-31) with a dosage of 250-375 IE/kg body weight. Only in one out of a total of 102 single applications, a minimal and self-limited side effect (chills) had been observed. The mean platelet count was almost doubled after sc anti-D (p < 0.0001). After a median follow-up of 11.4 months, all patients are alive without major bleeding and stay well. We conclude that sc anti-D: is not only an efficient means of treating ITP in children but is also a safe and convenient one.
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http://dx.doi.org/10.1007/s00277-009-0848-xDOI Listing
April 2010

Quality of life in long-term childhood cancer survivors and the relation of late effects and subjective well-being.

Support Care Cancer 2005 Jan 23;13(1):49-56. Epub 2004 Nov 23.

St. Anna Children's Hospital, Kinderspitalgasse 6, 1090, Vienna, Austria.

Goals: The purpose of the present study was the assessment of health-related quality of life in adult long-term survivors of childhood cancer and the detection of possible treatment- and cancer-related late effects. The relation of objective medical data and subjective appraisal was also examined by combining the doctor's and the patient's views of the perceived health status.

Patients And Methods: Seventy-eight young adults at the age of at least 18 years who had been diagnosed with cancer in the years 1975-1995 and were off therapy at least 5 years with no evidence of disease participated. Cancer survivors completed self-rating instruments [SF-36 Health Survey, Nottingham Health Profile (NHP), Beck's Depression Inventory (BDI), State-Trait Anxiety Inventory (STAI) trait-anxiety scale]; the doctor assessed the patient's health by means of the Common Toxicity Criteria (CTCv2).

Main Results: The cancer survivors showed a mostly higher-than-average positive subjective rating of the various areas of quality of life. In comparison to the general population, the cancer survivors showed lower extents of depression. In 64.1% of the cancer survivors, medical rating and self-assessment regarding the perceived health status corresponded. Cancer survivors who felt severely impaired regarding their subjectively perceived well-being showed worse results in some dimensions of quality of life than persons who subjectively felt mildly impaired. The so-called "unfortunate" persons showed the worst assessment of quality of life and the highest extent of depression and anxiety.

Conclusions: The results of the present study may be helpful in planning an appropriate multidisciplinary, long-term follow-up of childhood cancer survivors.
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http://dx.doi.org/10.1007/s00520-004-0724-0DOI Listing
January 2005