@article{RueeggKriemlerZuercheretal.2017, author = {Rueegg, Corina S. and Kriemler, Susi and Zuercher, Simeon J. and Schindera, Christina and Renner, Andrea and Hebestreit, Helge and Meier, Christian and Eser, Prisca and von der Weid, Nicolas X.}, title = {A partially supervised physical activity program for adult and adolescent survivors of childhood cancer (SURfit): study design of a randomized controlled trial [NCT02730767]}, series = {BMC Cancer}, volume = {17}, journal = {BMC Cancer}, doi = {10.1186/s12885-017-3801-8}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-172497}, year = {2017}, abstract = {Background: Beyond survival of nowadays >80\%, modern childhood cancer treatment strives to preserve long-term health and quality of life. However, the majority of today's survivors suffer from short- and long-term adverse effects such as cardiovascular and pulmonary diseases, obesity, osteoporosis, fatigue, depression, and reduced physical fitness and quality of life. Regular exercise can play a major role to mitigate or prevent such late-effects. Despite this, there are no data on the effects of regular exercise in childhood cancer survivors from randomized controlled trials (RCTs). \(Primary\) \(outcome\) of the current RCT is therefore the effect of a 12-months exercise program on a composite cardiovascular disease risk score in childhood cancer survivors. \(Secondary\) \(outcomes\) are single cardiovascular disease risk factors, glycaemic control, bone health, body composition, physical fitness, physical activity, quality of life, mental health, fatigue and adverse events (safety). Methods: A total of 150 childhood cancer survivors aged ≥16 years and diagnosed ≥5 years prior to the study are recruited from Swiss paediatric oncology clinics. Following the baseline assessments patients are randomized 1:1 into an intervention and control group. Thereafter, they are seen at month 3, 6 and 12 for follow-up assessments. The intervention group is asked to add ≥2.5 h of intense physical activity/week, including 30 min of strength building and 2 h of aerobic exercises. In addition, they are told to reduce screen time by 25\%. Regular consulting by physiotherapists, individual web-based activity diaries, and pedometer devices are used as motivational tools for the intervention group. The control group is asked to keep their physical activity levels constant. Discussion: The results of this study will show whether a partially supervised exercise intervention can improve cardiovascular disease risk factors, bone health, body composition, physical activity and fitness, fatigue, mental health and quality of life in childhood cancer survivors. If the program will be effective, all relevant information of the SURfit physical activity intervention will be made available to interested clinics that treat and follow-up childhood cancer patients to promote exercise in their patients.}, language = {en} } @article{EiseleBoczorRakebrandtetal.2017, author = {Eisele, Marion and Boczor, Sigrid and Rakebrandt, Anja and Blozik, Eva and Trader, Jens-Martin and Stork, Stefan and Herrmann-Lingen, Christoph and Scherer, Martin}, title = {General practitioners' awareness of depressive symptomatology is not associated with quality of life in heart failure patients - cross-sectional results of the observational RECODE-HF Study}, series = {BMC Family Practice}, volume = {18}, journal = {BMC Family Practice}, doi = {10.1186/s12875-017-0670-9}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-172445}, year = {2017}, abstract = {Background Depression is a common comorbidity in patients with chronic heart failure (HF) and linked to a wider range of symptoms which, in turn, are linked to a decreased health-related quality of life (HRQOL). Treatment of depression might improve HRQOL but detecting depression is difficult due to the symptom overlap between HF and depression. Therefore, clinical guidelines recommend to routinely screen for depression in HF patients. No studies have so far investigated the treatment after getting aware of a depressive symptomatology and its correlation with HRQOL in primary care HF patients. Therefore, we examined the factors linked to depression treatment and those linked to HRQOL in HF patients. We hypothesized that GPs' awareness of depressive symptomatology was associated with depression treatment and HRQOL in HF patients. Methods For this observational study, HF patients were recruited in primary care practices and filled out a questionnaire including PHQ-9 and HADS. A total of 574 patients screened positive for depressive symptomatology. Their GPs were interviewed by phone regarding the patients' comorbidities and potential depression treatment. Descriptive and regression analysis were performed. Results GPs reported various types of depression treatments (including dialogue/counselling by the GP him/herself in 31.8\% of the patients). The reported rates differed considerably between GP-reported initiated treatment and patient-reported utilised treatment regarding psychotherapy (16.4\% vs. 9.5\%) and pharmacotherapy (61.2\% vs. 30.3\%). The GPs' awareness of depressive symptomatology was significantly associated with the likelihood of receiving pharmacotherapy (OR 2.8; p < 0.001) but not psychotherapy. The patient's HRQOL was not significantly associated with the GPs' awareness of depression. Conclusion GPs should be aware of the gap between GP-initiated and patient-utilised depression treatments in patients with chronic HF, which might lead to an undersupply of depression treatment. It remains to be investigated why GPs' awareness of depressive symptomatology is not linked to patients' HRQOL. We hypothesize that GPs are aware of cases with reduced HRQOL (which improves under depression treatment) and unaware of cases whose depression do not significantly impair HRQOL, resulting in comparable levels of HRQOL in both groups. This hypothesis needs to be further investigated.}, language = {en} } @article{BurlinaSimsPoliteietal.2011, author = {Burlina, Alessandro P. and Sims, Katherine B. and Politei, Juan M. and Bennett, Gary J. and Baron, Ralf and Sommer, Claudia and Moller, Anette Torvin and Hilz, Max J.}, title = {Early diagnosis of peripheral nervous system involvement in Fabry disease and treatment of neuropathic pain: the report of an expert panel}, series = {BMC Neurology}, volume = {11}, journal = {BMC Neurology}, number = {61}, doi = {10.1186/1471-2377-11-61}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-135309}, pages = {1-11}, year = {2011}, abstract = {Background: Fabry disease is an inherited metabolic disorder characterized by progressive lysosomal accumulation of lipids in a variety of cell types, including neural cells. Small, unmyelinated nerve fibers are particularly affected and small fiber peripheral neuropathy often clinically manifests at young age. Peripheral pain can be chronic and/or occur as provoked attacks of excruciating pain. Manifestations of dysfunction of small autonomic fibers may include, among others, impaired sweating, gastrointestinal dysmotility, and abnormal pain perception. Patients with Fabry disease often remain undiagnosed until severe complications involving the kidney, heart, peripheral nerves and/or brain have arisen. Methods: An international expert panel convened with the goal to provide guidance to clinicians who may encounter unrecognized patients with Fabry disease on how to diagnose these patients early using simple diagnostic tests. A further aim was to offer recommendations to control neuropathic pain. Results: We describe the neuropathy in Fabry disease, focusing on peripheral small fiber dysfunction - the hallmark of early neurologic involvement in this disorder. The clinical course of peripheral pain is summarized, and the importance of medical history-taking, including family history, is highlighted. A thorough physical examination (e. g., angiokeratoma, corneal opacities) and simple non-invasive sensory perception tests could provide clues to the diagnosis of Fabry disease. Reported early clinical benefits of enzyme replacement therapy include reduction of neuropathic pain, and adequate management of residual pain to a tolerable and functional level can substantially improve the quality of life for patients. Conclusions: Our recommendations can assist in diagnosing Fabry small fiber neuropathy early, and offer clinicians guidance in controlling peripheral pain. This is particularly important since management of pain in young patients with Fabry disease appears to be inadequate.}, language = {en} } @article{WagnerKraemerBlohmetal.2014, author = {Wagner, Martin and Kr{\"a}mer, Johannes and Blohm, Elisabeth and Vergho, Dorothee and Weidemann, Frank and Breunig, Frank and Wanner, Christoph}, title = {Kidney function as an underestimated factor for reduced health related quality of life in patients with Fabry disease}, doi = {10.1186/1471-2369-15-188}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-111159}, year = {2014}, abstract = {Background: Impairments of health related quality of life (HRQoL) are frequently observed in Fabry disease (FD) and are known to be related to neuropathic pain and cardiovascular events. This study aimed to explore the role of chronic kidney disease (CKD) in a large cohort of patients with FD. Methods: In 96 patients (53\% female; age 40 ± 12 yrs) with genetically proven FD, HRQoL was assessed by the Medical Outcomes Study (SF-36) questionnaire. All patients were na{\"i}ve to enzyme replacement therapy. Three categories for kidney dysfunction were chosen, eGFR ≥/<60 ml/min/1.73 m2 or need of renal replacement therapy (RRT). Minor (e.g. arrhythmia, angina pectoris, etc.) and major (e.g. myocardial infarction, coronary artery bypass, stroke or implantable cardioverter-defibrillator) vascular events as well as pain and pain therapy were considered in linear regression analyses with the dimensions of HRQoL. Results: Ten patients (10\%) had impaired kidney function and a further nine were on RRT (9.4\%). Kidney function and pain emerged as the main factors associated with lower scores on the SF 36, in particular on physical components (PCS beta-coefficients for CKD -6.2, for RRT -11.8, for pain -9.1, p < 0.05, respectively), while controlling for gender, vascular event and pain-therapy. Relationships were found for mental aspects of HRQoL. Age and history of vascular events were not related to HRQoL. Conclusion: Cardiovascular events and pain are important factors related to HRQoL, social functioning and depression. Our study highlights impaired chronic kidney disease, in particular after initiation of RRT, as a strong determinant of reduced HRQoL in FD.}, language = {en} } @article{MeuleFathRealetal.2013, author = {Meule, Adrian and Fath, Katharina and Real, Ruben G. L. and S{\"u}tterlin, Stefan and V{\"o}gele, Claus and K{\"u}bler, Andrea}, title = {Quality of life, emotion regulation, and heart rate variability in individuals with intellectual disabilities and concomitant impaired vision}, series = {Psychology of Well-Being: Theory, Research and Practice}, journal = {Psychology of Well-Being: Theory, Research and Practice}, doi = {10.1186/2211-1522-3-1}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-96247}, year = {2013}, abstract = {Background Positive associations have been found between quality of life, emotion regulation strategies, and heart rate variability (HRV) in people without intellectual disabilities. However, emotion regulation and HRV have rarely been investigated in people with intellectual disabilities. Assessment of subjectively reported quality of life and emotion regulation strategies in this population is even more difficult when participants are also visually impaired. Methods Subjective and objective quality of life, emotion regulation strategies, and HRV at rest were measured in a sample of people with intellectual disabilities and concomitant impaired vision (N = 35). Heart rate was recorded during a 10 min resting period. For the assessment of quality of life and emotion regulation, custom made tactile versions of questionnaire-based instruments were used that enabled participants to grasp response categories. Results The combined use of reappraisal and suppression as emotion regulation strategies was associated with higher HRV and quality of life. HRV was associated with objective quality of life only. Emotion regulation strategies partially mediated the relationship between HRV and quality of life. Conclusions Results replicate findings about associations between quality of life, emotion regulation, and HRV and extend them to individuals with intellectual disabilities. Furthermore, this study demonstrated that quality of life and emotion regulation could be assessed in such populations even with concomitant impaired vision with modified tactile versions of established questionnaires. HRV may be used as a physiological index to evaluate physical and affective conditions in this population.}, language = {en} }