SARAspeech-Feasibility of automated assessment of ataxic speech disturbance.

Ataxias are a group of movement disorders that are characterized by progressive loss of balance, impaired coordination and speech disturbance, which together lead to markedly reduced quality of life. Speech disturbance is clinically diagnosed, but methods for objective assessment of severity are lacking. Using 71 sets of speech recordings from ataxia patients, we developed an automated classification system. With a tolerance of ±1 point, this classification system correctly predicted experts' ratings of speech disturbance according to item 4 of the Scale for Assessment and rating of ataxia (SARA) in 80% of cases. We thereby demonstrate feasibility of computer-assisted voice analysis for automated assessment of severity of speech disturbance.

Intra-articular human deciduous dental pulp stem cell administration vs. pharmacological therapy in experimental osteoarthritis rat model.

OBJECTIVE: The aim of the present study was to compare the molecular and morphological effects of diacerein and glucosamine-chondroitin drug treatment and intra-articular injection therapy of human deciduous dental pulp stem cells (hDPSCs) in a rat knee model of induced osteoarthritis (OA). MATERIALS AND METHODS: Thirty-six adult male rats were randomly separated into six groups: Control group (without induction of OA), osteoarthritis group 60 (induction of OA, saline gavage started on day 14 and performed for 60 days, followed by euthanasia), osteoarthritis group (induction of OA and euthanasia after 14 days), diacerein group, glucosamine-chondroitin group, and mesenchymal stem cell group. The drug-treated groups were gavaged with 50 mg/kg of diacerein and 400/500 mg/kg of glucosamine-chondroitin starting on dat 14 for 60 days. The cell therapy-treated group received an intra-articular single dose of 8 × 105 hDPSCs on day 14, and euthanasia was performed after 60 days. Lateral femoral condyles were collected and prepared for immunohistochemistry and light microscopy procedures. RESULTS: The morphological features and immunoexpression of SOX-5, IHH, MMP-8, MMP-13, and Type II collagen were statistically analysed. Our data suggest that hDPSC therapy contributes more actively and effectively in the structural reorganization of lateral femoral condyles. In contrast, the glucosamine-chondroitin sulphate treatment was more effective in inflammatory control, while diacerein showed better results associated with the maintenance of the primordial cartilage. CONCLUSIONS: The positive therapeutic effect of daily administered conventional drugs can be confirmed in a rat model of OA. However, one single dose of locally administered hDPSCs provides significant improvement in tissue regeneration in an OA model.

Identifying ecological production functions for use in ecosystem services-based environmental risk assessment of chemicals.

There is increasing research interest in the application of the ecosystem services (ES) concept in the environmental risk assessment of chemicals to support formulating and operationalising regulatory environmental protection goals and making environmental risk assessment more policy- and value-relevant. This requires connecting ecosystem structure and processes to ecosystem function and henceforth to provision of ecosystem goods and services and their economic valuation. Ecological production functions (EPFs) may help to quantify these connections in a transparent manner and to predict ES provision based on function-related descriptors for service providing species, communities, ecosystems or habitats. We review scientific literature for EPFs to evaluate availability across provisioning and regulation and maintenance services (CICES v5.1 classification). We found quantitative production functions for nearly all ES, often complemented with economic valuation of physical or monetary flows. We studied the service providing units in these EPFs to evaluate the potential for extrapolation of toxicity data for test species obtained from standardised testing to ES provision. A broad taxonomic representation of service providers was established, but quantitative models directly linking standard test species to ES provision were extremely scarce. A pragmatic way to deal with this data gap would be the use of proxies for related taxa and stepwise functional extrapolation to ES provision and valuation, which we conclude possible for most ES. We suggest that EPFs may be used in defining specific protection goals (SPGs), and illustrate, using pollination as an example, the availability of information for the ecological entity and attribute dimensions of SPGs. Twenty-five pollination EPFs were compiled from the literature for biological entities ranging from 'colony' to 'habitat', with 75% referring to 'functional group'. With about equal representation of the attributes 'function', 'abundance' and 'diversity', SPGs for pollination therefore would seem best substantiated by EPFs at the level of functional group.

Intra-articular human deciduous dental pulp stem cell administration vs. pharmacological therapy in experimental osteoarthritis rat model

OBJECTIVE: The aim of the present study was to compare the molecular and morphological effects of diacerein and glucosamine-chondroitin drug treatment and intra-articular injection herapy of human deciduous dental pulp stem cells (hDPSCs) in a rat knee model of induced osteoarthritis OA. MATERIALS AND METHODS: Thirty-six adult male rats were randomly separated into six groups: Control group without induction of OA, osteoarthritis group 60 induction of OA, saline gavage started on day 14 and performed for 60 days, followed by euthanasia, osteoarthritis group induction of OA and euthanasia after 14 days, diacerein group, glucosamine-chondroitin group, and mesenchymal stem cell group. The drug-treated groups were gavaged with 50 mg/kg of diacerein and 400/500 mg/kg of glucosamine-chondroitin starting on dat 14 for 60 days. The cell therapy-treated group received an intra-articular single dose of 8 × 105 hDPSCs on day 14, and euthanasia was performed after 60 days. Lateral femoral condyles were collected and prepared for immunohistochemistry and light microscopy procedures. RESULTS: The morphological features and immunoexpression of SOX-5, IHH, MMP-8, MMP-13, and Type II collagen were statistically analysed. Our data suggest that hDPSC therapy contributes more actively and effectively in the structural reorganization of lateral femoral condyles. In contrast, the glucosamine-chondroitin sulphate treatment was more effective in inflammatory control, while diacerein showed better results associated with the maintenance of the primordial cartilage. CONCLUSIONS: The positive therapeutic effect of daily administered conventional drugs can be confirmed in a rat model of OA. However, one single dose of locally administered hDPSCs provides significant improvement in tissue regeneration in an OA model.

Epidemiology, treatment, costs, and long-term outcomes of patients with fireworks-related injuries (ROCKET); a multicenter prospective observational case series.

OBJECTIVE: People in the Netherlands are legally allowed to celebrate New Year's Eve with consumer fireworks. The aim of this study was to provide detailed information about the patient and injury characteristics, medical and societal costs, and clinical and functional outcome in patients with injuries resulting from this tradition. METHODS: A multicenter, prospective, observational case series performed in the Southwest Netherlands trauma region, which reflects 15% of the country and includes a level I trauma center, a specialized burn center, a specialized eye hospital, and 13 general hospitals. All patients with any injury caused by consumer fireworks, treated at a Dutch hospital between December 1, 2017 and January 31, 2018, were eligible for inclusion. Exclusion criteria were unknown contact information or insufficient understanding of Dutch or English language. The primary outcome measure was injury characteristics. Secondary outcome measures included treatment, direct medical and indirect societal costs, and clinical and functional outcome until one year after trauma. RESULTS: 54 out of 63 eligible patients agreed to participate in this study. The majority were males (N = 50; 93%), 50% were children below 16 years of age, and 46% were bystanders. Injuries were mainly located to the upper extremity or eyes, and were mostly burns (N = 38; 48%) of partial thickness (N = 32; 84%). Fifteen (28%) patients were admitted and 11 (20%) patients needed surgical treatment. The mean total costs per patient were €6,320 (95% CI €3,400 to €9,245). The most important cost category was hospital admission. Only few patients reported complaints in patient-reported quality of life and functional outcome after 12 months follow-up. CONCLUSION: This study found that young males are most vulnerable for fireworks injuries and that most injuries consist of burns, located to the arm and hand, and eye injuries. On the long-term only few patients experienced reduced quality of life and functional limitations.

Comprehensive assessments and related interventions to enhance the long-term outcomes of child, adolescent and young adult cancer survivors - presentation of the CARE for CAYA-Program study protocol and associated literature review.

BACKGROUND: Improved, multimodal treatment strategies have been shown to increase cure rates in cancer patients. Those who survive cancer as a child, adolescent or young adult (CAYA), are at a higher risk for therapy-, or disease-related, late or long-term effects. The CARE for CAYA-Program has been developed to comprehensively assess any potential future problems, to offer need-based preventative interventions and thus to improve long-term outcomes in this particularly vulnerable population. METHODS: The trial is designed as an adaptive trial with an annual comprehensive assessment followed by needs stratified, modular interventions, currently including physical activity, nutrition and psycho-oncology, all aimed at improving the lifestyle and/or the psychosocial situation of the patients. Patients, aged 15-39 years old, with a prior cancer diagnosis, who have completed tumour therapy and are in follow-up care, and who are tumour free, will be included. At baseline (and subsequently on an annual basis) the current medical and psychosocial situation and lifestyle of the participants will be assessed using a survey compiled of various validated questionnaires (e.g. EORTC QLQ C30, NCCN distress thermometer, PHQ-4, BSA, nutrition protocol) and objective parameters (e.g. BMI, WHR, co-morbidities like hyperlipidaemia, hypertension, diabetes), followed by basic care (psychological and lifestyle consultation). Depending on their needs, CAYAs will be allocated to preventative interventions in the above-mentioned modules over a 12-month period. After 1 year, the assessment will be repeated, and further interventions may be applied as needed. During the initial trial phase, the efficacy of this approach will be compared to standard care (waiting list with intervention in the following year) in a randomized study. During this phase, 530 CAYAs will be included and 320 eligible CAYAs who are willing to participate in the interventions will be randomly allocated to an intervention. Overall, 1500 CAYAs will be included and assessed. The programme is financed by the innovation fund of the German Federal Joint Committee and will be conducted at 14 German sites. Recruitment began in January 2018. DISCUSSION: CAYAs are at high risk for long-term sequelae. Providing structured interventions to improve lifestyle and psychological situation may counteract against these risk factors. The programme serves to establish uniform regular comprehensive assessments and need-based interventions to improve long-term outcome in CAYA survivors. TRIAL REGISTRATION: Registered at the German Clinical Trial Register (ID: DRKS00012504, registration date: 19th January 2018).

Characterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA).

Sporadic adult-onset ataxia of unknown etiology (SAOA) is a non-genetic neurodegenerative disorder of the cerebellum of unknown cause which manifests with progressive ataxia without severe autonomic failure. Although SAOA is associated with cerebellar degeneration, little is known about the specific cerebellar atrophy pattern in SAOA. Thirty-seven SAOA patients and 49 healthy controls (HCs) were included at two centers. We investigated the structural and functional characteristics of SAOA brains using voxel-based morphometry (VBM) and resting-state functional imaging (rs-fMRI). In order to examine the functional consequence of structural cerebellar alterations, the amplitude of low-frequency fluctuation (ALFF) and degree centrality (DC) were analyzed, and then assessed their relation with disease severity, disease duration, and age of onset within these regions. Group differences were investigated using two-sample t tests, controlling for age, gender, site, and the total intracranial volume. The VBM analysis revealed a significant, mostly bilateral reduction of local gray matter (GM) volume in lobules I-V, V, VI, IX, X, and vermis VIII a/b in SAOA patients, compared with HCs. The GM volume loss in these regions was significantly associated with disease severity, disease duration, and age of onset. The disease-related atrophy regions did not show any functional alternations compared with HCs but were functionally characterized by high ALFF and poor DC compared with intact cerebellar regions. Our data revealed volume reduction in SAOA in cerebellar regions that are known to be involved in motor and somatosensory processing, corresponding with the clinical phenotype of SAOA. Our data suggest that the atrophy occurs in those cerebellar regions which are characterized by high ALFF and poor DC. Further studies have to show if these findings are specific for SAOA, and if they can be used to predict disease progression.

Low N-terminal pro-brain natriuretic peptide levels are associated with non-alcoholic fatty liver disease in patients with type 2 diabetes.

AIM: Natriuretic peptides (NPs) have emerged as important regulators of lipid metabolism. Reduced levels of NPs are reported in obesity and in patients with type 2 diabetes (T2D). This NP deficiency may affect their ectopic fat distribution and lead to high risk of non-alcoholic fatty liver disease (NAFLD). METHODS: In this cross-sectional study, the association between N-terminal pro-B-type natriuretic peptide (NT-proBNP) and liver fat content was quantified using 1H-magnetic resonance spectroscopy in 120 patients with T2D. RESULTS: NAFLD (defined as liver fat content ≥ 5.6%) was found in 57 (48%) of the T2D patients, who also had significantly lower NT-proBNP (P = 0.002) levels compared with patients without NAFLD, but did not differ as regards the presence of cardiovascular disease (CVD) or in kidney function. After adjusting for potential confounders (age, gender, HbA1c, BMI, HOMA2-IR, CVD, eGFR), the odds ratio for the presence of NAFLD was increased by 2.9 (P = 0.048) for NT-proBNP levels < 45 ng/L. In a multivariable linear regression model, the relationship with NT-proBNP was further analyzed as a continuous variable, and was independently and inversely associated with increasing liver fat content after full adjustment (P = 0.031). CONCLUSION: Reduced plasma NT-proBNP levels are independently associated with high liver fat content in patients with T2D. The present study suggests that NP deficiency may play a role in the development of NAFLD in T2D.

[Firework injuries in the south-western region of the Netherlands around the turn of the year 2017-2018]. / Vuurwerkletsel in Zuidwest-Nederland.

OBJECTIVE: To report the number of patients with firework-related injuries treated in December 2017 and January 2018 in a hospital in the south-west Netherlands trauma region, and to provide details about the types of firework used and the specific injuries. DESIGN: A prospective multicentre cohort study (NTR6793). METHODS: Patients of all ages with firework-related injuries were eligible for inclusion. The injury had to have been sustained between 1 December 2017 and 31 January 2018, and treated at a hospital in the south-west Netherlands trauma region (approximately 2.5 million inhabitants). Data were extracted from patients' medical files and additional information was obtained from patient interviews. RESULTS: Fifty-four patients were included. The majority were male (93%) and the median age was 15 years. Twenty-five (46%) patients were bystanders and 12 (22%) were injured by illegal fireworks. Fifty patients were injured by bangers (n=22) or decorative fireworks (n=28). The patients had a total of 79 injuries, of which 29 (37%) were localised to the upper extremity and 19 (24%) to the eyes. Most upper extremity injuries were burns (69%), primarily partial thickness. Of the eye injuries, 14 were caused by blunt trauma, seven by chemical trauma, and one by penetrating trauma. Three patients sustained indirect firework-related injuries. CONCLUSION: Between 1 December 2017 and 31 January 2018 in the south-west Netherlands trauma region mainly teenage males and bystanders sustained firework-related injuries. Most injuries were upper extremity burns and eye injuries, mainly due to legal fireworks and bangers or decorative fireworks. The extent of the sample indicates that the study findings can be extrapolated to the rest of the Netherlands.

Type, severity, management and outcome of ocular and adnexal firework-related injuries: the Rotterdam experience.

PURPOSE: To study the type, severity, management and outcome of firework-related adnexal and ocular injuries during New Year's Eve festivities. METHODS: A retrospective analysis of 123 injured patients (143 eyes) treated at the Rotterdam Eye Hospital between 2009 and 2013. All ages were included and analysed according to age, gender, active participant or bystander, laterality, location, dimension and severity of injury. Outcome parameter was the final best-corrected visual acuity. RESULTS: The mean age was 22 ± 13 years with 87% males and 53% bystanders. 52% were ≤18 years. There was a higher number of female than male bystanders (63% versus 51%, p = 0.30). 50% of the eyes sustained mild, 13% moderate and 37% severe trauma. Adults suffered more from severe injuries compared to children (42% versus 31%). The most frequent intervention was gunpowder removal (20%), followed by traumatic cataract surgery (12%) and amniotic membrane grafting (8%). 76% of patients were followed over 1 year. At the end of follow-up, 88 (61.5%) eyes had recovered fully, while 55 (38.5%) eyes suffered from persistent complications with reduced vision ≤0.8 in 30% of injured eyes. 15 patients (12%, 10 adults, five children) were considered legally blind (vision ≤0.1). Three (2%) eyes were subject to evisceration. CONCLUSION: Every year, around New Year's Eve 30-45 victims were referred to the Rotterdam Eye Hospital; 50% sustained moderate-to-severe trauma. In severe firework injuries, patients required multiple treatments that may not prevent permanent blindness and/or functional/cosmetic disfigurement. The majority was bystander and younger than 18 years.

Burden of cardiovascular risk factors and cardiovascular disease in childhood cancer survivors: data from the German CVSS-study.

Aims: The cardiac and vascular late sequelae in long-term survivors of childhood cancer (CVSS)-study aimed to quantify the prevalence of cardiovascular risk factors (CVRF) and cardiovascular disease (CVD) in German childhood cancer survivors (CCS). Methods and results: In the CVSS-study (NCT02181049), 1002 CCS (age range 23-48 years) diagnosed with neoplasia prior to 15 years of age between 1980 and 1990 prospectively underwent a systematic, standardized clinical and laboratory cardiovascular screening, identical to the population-based Gutenberg Health Study (GHS) cohort. For 951 individuals, prevalences of CVRF and CVD were primarily compared to the GHS sample and to two further German population-based cohorts. Using log-binomial regression models, an increased risk for occurrence of arterial hypertension [relative risk (RR) 1.38, 95% confidence interval (95% CI 1.21-1.57)] and dyslipidaemia [RR 1.26 (95% CI 1.12-1.42)] was found. This indicates a premature occurrence compared to the general population of approximately 6 and 8 years, respectively [rate advancement period estimator, RAPhypertension 5.75 (95% CI 3.5-8.0) and RAPdyslipidaemia 8.16 (95% CI 4.4-11.9)]. Overall, no differences were observed for obesity and diabetes. Overt CVD was present in 4.5% (95% CI 3.0-6.6%) of CCS [RR 1.89 (95% CI 1.34-2.66), RAPCVD 7.9 (95% CI 4.1-11.7)], of which the most frequent entities were congestive heart failure and venous thromboembolism. Prevalences of CVRF and CVD increased with age without reaching a plateau over time. Conclusion: This large CCS screening examination revealed consistently in comparison to three population samples a considerably increased risk for premature CVD. The findings in these young adult CCS indicate a high burden of cardiovascular morbidity and mortality in the long term. Clinicaltrials. gov-Nr: NCT02181049.

Within-day energy deficiency and reproductive function in female endurance athletes.

We aimed to estimate and compare within-day energy balance (WDEB) in athletes with eumenorrhea and menstrual dysfunction (MD) with similar 24-hour energy availability/energy balance (EA/EB). Furthermore, to investigate whether within-day energy deficiency is associated with resting metabolic rate (RMR), body composition, S-cortisol, estradiol, T3 , and fasting blood glucose. We reanalyzed 7-day dietary intake and energy expenditure data in 25 elite endurance athletes with eumenorrhea (n = 10) and MD (n = 15) from a group of 45 subjects where those with disordered eating behaviors (n = 11), MD not related to low EA (n = 5), and low dietary record validity (n = 4) had been excluded. Besides gynecological examination and disordered eating evaluation, the protocol included RMR measurement; assessment of body composition by dual-energy X-ray absorptiometry, blood plasma analysis, and calculation of WDEB in 1-hour intervals. Subjects with MD spent more hours in a catabolic state compared to eumenorrheic athletes; WDEB < 0 kcal: 23.0 hour (20.8-23.4) vs 21.1 hour (4.7-22.3), P = .048; WDEB < -300 kcal: 21.8 hour (17.8-22.4) vs 17.6 hour (3.9-20.9), P = .043, although similar 24-hour EA: 35.6 (11.6) vs 41.3 (12.7) kcal/kg FFM/d, (P = .269), and EB: -659 (551) vs -313 (596) kcal/d, (P = .160). Hours with WDEB <0 kcal and <-300 kcal were inversely associated with RMRratio (r = -.487, P = .013, r = -.472, P = .018), and estradiol (r = -.433, P = .034, r = -.516, P = .009), and positively associated with cortisol (r = .442, P = .027, r = .463, P = .019). In conclusion, although similar 24-hour EA/EB, the reanalysis revealed that MD athletes spent more time in a catabolic state compared to eumenorrheic athletes. Within-day energy deficiency was associated with clinical markers of metabolic disturbances.

[Social awareness campaign for a ban on consumer fireworks: 10 tips; doctor on the barricade]. / Maatschappelijke actie voor een verbod op consumentenvuurwerk: 10 tips.

- Describe the problem, and show why a social awareness campaign is needed- Know what you are talking about, and explain the medical jargon in plain language- Collect data and facts- Create support among colleagues- Get the media involved- Get support from the community- Create a coalition of supporters via a website- You will discover who the opposition is when you are on the barricade- Offer a safe and healthy alternative - Draw up a long-term roadmap, and persevere.

Treatment of relapse after allogeneic stem cell transplantation in children and adolescents with ALL: the Frankfurt experience.

Therapy for post-transplant relapse of paediatric ALL is limited. Standardised curative approaches are not available. We hereby describe our local procedure in this life-threatening situation. A total of 101 ALL patients received their first allogeneic stem cell transplantation (SCT) in our institution. After relapse, our primary therapeutic goal was to cure the patient with high-dose chemotherapy or specific immunotherapy (HDCHT/SIT) followed by a second SCT from a haploidentical donor (transplant approach). If this was not feasible, low-dose chemotherapy and donor lymphocyte infusions (LDCHT+DLI) were offered (non-transplant approach). A total of 23 patients suffered a post-transplant relapse. Eight patients received HDCHT/SIT, followed by haploidentical SCT in 7/8. Ten received LDCHT+DLI. The eight patients treated with a second transplant and the ten treated with the non-transplant approach had a 4-year overall survival of 56% and 40%, respectively (P=0.232). Prerequisites for successful treatment of post-transplant relapse by either a second transplant or experimental non-transplant approaches are good clinical condition and the capacity to achieve haematological remission by the induction treatment element.

Accessory Extraocular Muscle as a Cause of Restrictive Strabismus.

Restrictive strabismus resulting from the presence of an accessory extraocular muscle has rarely been reported in the literature. Most articles written on this topic are isolated case reports. The purpose of this paper is to describe a series of 7 similar patients presenting with atypical restrictive strabismus associated with enophthalmos in the affected eye, which was found to be caused by an accessory extraocular muscle attached to the posterior globe near the optic nerve. The medical records of 7 patients who shared these clinical characteristics were retrospectively analyzed. Orbital imaging was obtained in the 7 cases, which were compared. Three of the patients were females and four were males. The left eye was affected in all 4 males and the right eye was affected in the 3 females. The 7 patients presented with the following clinical characteristics: enophthalmos, restriction to eye movements in most fields of gaze, and presence of an anomalous orbital structure that was interpreted on magnetic resonance imaging (MRI) to be an accessory extraocular muscle inserting onto the posterior surface of the globe in the affected eye. The fellow eye was normal in all cases. Five of the 7 patients underwent surgical correction with partial improvement in only one patient. The presence of an accessory extraocular muscle should be included in the differential diagnosis of patients with atypical restrictive strabismus. Orbital computed tomography or MRI are essential for correct diagnosis in these cases.

Ten-year outcome of early childhood traumatic brain injury: Diffusion tensor imaging of the ventral striatum in relation to executive functioning.

PRIMARY OBJECTIVE: The long-term effects of TBI on verbal fluency and related structures, as well as the relation between cognition and structural integrity, were evaluated. It was hypothesized that the group with TBI would evidence poorer performance on cognitive measures and a decrease in structural integrity. RESEARCH DESIGN: Between a paediatric group with TBI and a group of typically-developing children, the long-term effects of traumatic brain injury were investigated in relation to both structural integrity and cognition. Common metrics for diffusion tensor imaging (DTI) were used as indicators of white matter integrity. METHODS AND PROCEDURES: Using DTI, this study examined ventral striatum (VS) integrity in 21 patients aged 10-18 years sustaining moderate-to-severe traumatic brain injury (TBI) 5-15 years earlier and 16 demographically comparable subjects. All participants completed Delis-Kaplan Executive Functioning System (D-KEFS) sub-tests. MAIN OUTCOMES AND RESULTS: The group with TBI exhibited lower fractional anisotropy (FA) and executive functioning performance and higher apparent diffusion coefficient (ADC). DTI metrics correlated with D-KEFS performance (right VS FA with Inhibition errors, right VS ADC with Letter Fluency, left VS FA and ADC with Category Switching). CONCLUSIONS: TBI affects VS integrity, even in a chronic phase, and may contribute to executive functioning deficits.

The International Haemovigilance Network Database for the Surveillance of Adverse Reactions and Events in Donors and Recipients of Blood Components: technical issues and results.

BACKGROUND AND OBJECTIVES: The International Haemovigilance Network's ISTARE is an online database for surveillance of all adverse reactions (ARs) and adverse events (AEs) associated with donation of blood and transfusion of blood components, irrespective of severity or the harm caused. ISTARE aims to unify the collection and sharing of information with a view to harmonizing best practices for haemovigilance systems around the world. MATERIALS AND METHODS: Adverse reactionss and adverse events are recorded by blood component, type of reaction, severity and imputability to transfusion, using internationally agreed standard definitions. RESULTS: From 2006 to 2012, 125 national sets of annual aggregated data were received from 25 countries, covering 132.8 million blood components issued. The incidence of all ARs was 77.5 per 100 000 components issued, of which 25% were severe (19.1 per 100 000). Of 349 deaths (0.26 per 100 000), 58% were due to the three ARs related to the respiratory system: transfusion-associated circulatory overload (TACO, 27%), transfusion-associated acute lung injury (TRALI, 19%) and transfusion-associated dyspnoea (TAD, 12%). Cumulatively, 594 477 donor complications were reported (rate 660 per 100 000), of which 2.9% were severe. CONCLUSIONS: ISTARE is a well-established surveillance tool offering important contributions to international efforts to maximize transfusion safety.

Erectile function after treatment for rhabdomyosarcoma of prostate and bladder.

INTRODUCTION: Rhabdomyosarcoma (RMS) accounts for 5% of all pediatric tumors; 15-20% of these tumors are located in the urogenital tract, mostly originating from the prostate or bladder. In the light of the steadily improving prognosis for patients with RMS through interdisciplinary-multimodal study protocols with 60-70% long-term survivors, non oncological aspects such as erectile function (EF) have become increasingly important. The aim of this study was to evaluate EF in patients having undergone treatment for RMS of the bladder and prostate. DESIGN: The medical records of 24 male patients having undergone surgical treatment for pelvic RMS between 1975 and 2014 were reviewed, and follow-up was obtained. Erectile function was determined using the Self-Estimation Index of Erectile Function-No Sexual Intercourse (SIEF-NS) and the Erection Hardness Scale (EHS). Potential prognostic factors were evaluated in respect to their impact on EF. RESULTS: Thirteen patients were included in the study (median age 20 years). Median follow-up period was 12.7 years (1.09-39.85). All patients completed the EHS; nine patients completed the SIEF-NS. All three patients with preserved erectile function (EHS = 4) showed a score indicating no or minimal impairment on sexual function on SIEF-NS (median 33). None of these patients had undergone external radiotherapy, and radical cystoprostatectomy had been performed before the third year of life in two out of three. The remaining patients had erectile dysfunction (EHS = 0). Three patients had an unsatisfying treatment attempt with sildenafil. Seven patients, including all with failures of oral PDE-5-inhibitors, were successfully treated with intracavernous injection of Alprostadil (Summary Table). DISCUSSION: This was the largest study, to date, evaluating erectile EF in patients treated for RMS of the bladder or prostate. EF was preserved in some patients, despite aggressive treatment modalities. Patients affected by erectile dysfunction after therapy showed limited response to PDE-5 inhibitors, but even after failure of the latter, an intracavernous injection of Alprostadil showed a significant improvement in EHS and SIEF-NS. Limitations of the study included the retrospective nature, small sample size, and heterogeneity of underlying disease, stage, and treatment modalities used. CONCLUSIONS: The results suggested that in a subset of patients, EF was preserved after radical surgical treatment of RMS, especially in young boys. Intracavernous injection of Alprostadil was effective, even after failure of PDE-5-inhibitors, and should be offered to patients without spontaneous erections, whereas PDE-5-inhibitors appeared to be largely ineffective. External radiation therapy appeared to have a negative impact on EF.