Innsbruck Medical University Research Articles

Video-polysomnographic findings after acute COVID-19: REM sleep without atonia as sign of CNS pathology?

ObjectiveSleep complaints are frequent after acute COVID-19. Aim of this study was to videopolysomnographically evaluate sleep and sleep disorders after SARS-Cov2 infection. MethodsPatients with suspected sleep disorders after acute COVID-19 underwent video-polysomnography (v-PSG) at the Sleep Disorders Clinic, Department of Neurology, Medical University Innsbruck. V-PSG was conducted 4.2 (SD = 1.3) months after diagnosis of SARS-CoV-2 infection. ResultsEleven patients [nine men, age 52.5 (SD = 11.7) years; BMI 29 (SD = 5.2) kg/m2] were included. At 60 days follow-up after diagnosis, persisting breathing complaints were present in 7/11 (64%) patients. After v-PSG four patients (36%) were diagnosed with obstructive sleep apnea (OSA). Respiratory frequency during sleep was normal and no tachypnea, thoracoabdominal asynchrony, or periodic deep sighing were detected. Four patients (36%) showed REM sleep without atonia (RWA), and two additional patients showed an RWA index within the highest range of normality. ConclusionWe report videopolysomnographic findings in a series of eleven patients after acute COVID-19. A major finding of this study was the presence of isolated RWA, a recognized prodromal stage of RBD, in more than one third of the patients. Future videopolysomnographic investigations including quantification of RWA in patients after COVID-19 will give more insights into a possible acute or post-infectious CNS pathology related to the SARS-CoV-2 infection.

Amplitude-integrated electroencephalography signals in preterm infants with cerebral hemorrhage

ObjectiveTo evaluate whether preterm infants with cerebral hemorrhage show alterations of aEEG signals in the first four weeks of life. Study designPreterm infants (n = 536) born before 32 completed weeks of pregnancy at Innsbruck Medical University Hospital were included in the study. AEEG recordings were evaluated for the Burdjalov score and cerebral hemorrhage was diagnosed by cerebral ultrasound. ResultsEighty preterm infants with cerebral hemorrhage (median gestational age 28.9 weeks, median birth weight 1157 g) and 456 preterm infants without cerebral hemorrhage (median gestational age 30.0 weeks, median birth weight 1300 g) were investigated. Burdjalov total scores were significantly lower in infants with cerebral hemorrhage. Infants with mild cerebral hemorrhage showed higher Burdjalov total scores compared to infants with severe cerebral hemorrhage in the first days of life. A Burdjalov total score of seven or more was predictive for no development of a cerebral hemorrhage, with a highest area under the curve (0.613) at postnatal day three. ConclusionPreterm infants with cerebral hemorrhage show alterations in aEEG signals in the newborn period. In future aEEG could be used as a supplemental method to monitor preterm infants at risk for cerebral hemorrhage. The use of aEEG in early life could reduce the number of ultrasound examinations and limit cumulative stress and discomfort in preterm infants.

Tangential Excision of Rhinophyma with the Disposable Razor Blade: Simple and Cost Efficient with Aesthetically Good Results.

Medical therapies for rhinophyma have been described but these only delay progression. Therefore, surgery is the method of choice. Plenty of modalities have been described including cold-knife surgery, electrosurgery, hydrosurgery, laser-assisted treatments, and dermabrasion.Settings and Design:In this two-center study, patients’ charts and photodocumentation were analyzed retrospectively.Materials and Methods:Surgery was performed under general anesthesia with an additional local anesthesia of the affected areas of the nose. We removed the hypertrophic tissue in thin layers with a sterile disposable razor blade under constant visual control of the underlying cartilage and adnexal structures. A dressing with Mepithel and gauzes was applied. Patients presented weekly to monitor the wounds. Follow-up was 1 year.Results:From 2016 to 2019, nine male patients with rhinophyma underwent surgical therapy at AGAPLESION Markus Hospital, Frankfurt am Main, Germany and at the Department of Plastic, Reconstructive and Aesthetic Surgery, Innsbruck Medical University, Austria. The mean age of the patients was 66 years. Mean time to complete re-epithelization equaled 31.5 days. No recurrences were noted within the follow-up period of 1 year. Patients’ satisfaction was very high. Only one patient had hypertrophic scars at the wing of the nose and another one developed a superficial fistula without connection to the nasal cavity.Conclusion:To the best of our knowledge, this is the first case series describing the use of a disposable razor blade for rhinophyma treatment supporting its efficiency described in previous anecdotal publications. We can highly recommend the technique, as it is cost-efficient and simple and provides excellent aesthetic results.

Impaired fibrinolysis in critically ill COVID-19 patients

BackgroundCritically ill coronavirus disease 2019 (COVID-19) patients present with a hypercoagulable state with high rates of macrovascular and microvascular thrombosis, for which hypofibrinolysis might be an important contributing factor. MethodsWe retrospectively analysed 20 critically ill COVID-19 patients at Innsbruck Medical University Hospital whose coagulation function was tested with ClotPro® and compared with that of 60 healthy individuals at Augsburg University Clinic. ClotPro is a viscoelastic whole blood coagulation testing device. It includes the TPA test, which uses tissue factor (TF)-activated whole blood with added recombinant tissue-derived plasminogen activator (r-tPA) to induce fibrinolysis. For this purpose, the lysis time (LT) is measured as the time from when maximum clot firmness (MCF) is reached until MCF falls by 50%. We compared COVID-19 patients with prolonged LT in the TPA test and those with normal LT. ResultsCritically ill COVID-19 patients showed hypercoagulability in ClotPro assays. MCF was higher in the EX test (TF-activated assay), IN test (ellagic acid-activated assay), and FIB test (functional fibrinogen assay) with decreased maximum lysis (ML) in the EX test (hypofibrinolysis) and highly prolonged TPA test LT (decreased fibrinolytic response), as compared with healthy persons. COVID-19 patients with decreased fibrinolytic response showed higher fibrinogen levels, higher thrombocyte count, higher C-reactive protein levels, and decreased ML in the EX test and IN test. ConclusionCritically ill COVID-19 patients have impaired fibrinolysis. This hypofibrinolytic state could be at least partially dependent on a decreased fibrinolytic response.

Long-term outcome after hand and forearm transplantation - a retrospective study.

SummaryBetween 2000 and 2014, five patients received bilateral hand (n = 3), bilateral forearm (n = 1), and unilateral hand (n = 1) transplants at the Innsbruck Medical University Hospital. We provide a comprehensive report of the long‐term results at 20 years. During the 6–20 years follow‐up, 43 rejection episodes were recorded in total. Of these, 27.9% were antibody‐related with serum donor‐specific alloantibodies (DSA) and skin‐infiltrating B‐cells. The cell phenotype in rejecting skin biopsies changed and C4d‐staining increased with time post‐transplantation. In the long‐term, a change in hand appearance was observed. The functional outcome was highly depending on the level of amputation. The number and severity of rejections did not correlate with hand function, but negatively impacted on the patients´ well‐being and quality of life. Patient satisfaction significantly correlated with upper limb function. One hand allograft eventually developed severe allograft vasculopathy and was amputated at 7 years. The patient later died due to progressive gastric cancer. The other four patients are currently rejection‐free with moderate levels of immunosuppression. Hand transplantation remains a therapeutic option for carefully selected patients. A stable immunologic situation with optimized and individually adopted immunosuppression favors good compliance and patient satisfaction and may prevent development of DSA.

Adult T-cells impair neonatal cardiac regeneration

Abstract Background Cardiac remodeling and subsequent heart failure remain critical issues after myocardial infarction (MI). Complete cardiac regeneration was shown recently in a neonatal mouse model of MI. This cardiac regenerative potential is limited to the first few postnatal days and its decline parallels with the maturation of the adaptive immune system. Purpose Herein, we hypothesized that the T-cell maturation status critically impacts the myocardial healing outcomes in neonates and contributes to the shift from regenerative to scarring phenotype observed shortly after birth. Methods The post-MI immune responses were characterized in postnatal day one (P1, regenerative) compared to seven-day old (P7, scarring) mice subjected to permanent left anterior descending artery (LAD) ligation. The myocardial leukocyte infiltrate was phenotyped by flow cytometry at 36 hours and five days after LAD ligation. Next, we studied neonatal post-MI repair in lymphocyte-deficient Rag2 knock-out (KO) mice subjected to LAD ligation. Moreover, we adoptively transferred syngeneic splenic Thy 1.1+ T-cells obtained from adult donors into P1 versus P7 recipients and then assessed their impact on post-MI healing. Results LAD ligation induced a robust early inflammatory response (36h post-MI) in both age groups. The in situ inflammation was, nevertheless, rapidly resolved in P1-, but not in P7-infarcted animals. The distinct age groups showed a similar profile of cardiac myeloid cell infiltration but showed remarkable differences in the lymphoid compartment. P1-infarcted mice showed an early recruitment of γδT-cells, whereas P7-infarcted mice exhibited a prominent infiltration of αβT-cells. Of note, neonatal cardiac regeneration was not altered in neonatal lymphocyte-deficient (Rag2 KO) animals. However, the adoptive transfer of adult T-cells had several consequences in neonatal and one week old mice subjected to ischemic injury. P1-infarcted mice transferred with adult T-cells showed an adult-like healing phenotype, marked by an irreversible cardiac functional impairment (assessed by echocardiography) and increased fibrosis. This is in sharp contrast to the regenerative phenotype typically observed in untreated age-matched controls. Furthermore, P7-infarcted mice transferred with adult T-cell showed significantly decreased survival rate after LAD ligation. Conclusion Neonatal hearts demonstrate rapid clearance of the ischemia-induced leukocyte infiltration, further reflecting the known fact of fast cardiac regeneration in newborn rodents. Of note, the adoptive transfer of adult T-cells into neonate recipients partially blocked cardiac regeneration and promoted an irreversible functional impairment. These data indicate that the cardiac repair process, and its related “regeneration vs. scarring” dichotomy, is critically impacted by the T-cell development status. Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Innsbruck Medical University, Medizinischer Forschungsfonds Tirol

Linkage of alterations in systemic iron homeostasis to patients\u2019 outcome in sepsis: a prospective study

BackgroundSepsis, a dysregulated host response following infection, is associated with massive immune activation and high mortality rates. There is still a need to define further risk factors and laboratory parameters predicting the clinical course. Iron metabolism is regulated by both, the body’s iron status and the immune response. Iron itself is required for erythropoiesis but also for many cellular and metabolic functions. Moreover, iron availability is a critical determinant in infections because it is an essential nutrient for most microbes but also impacts on immune function and intravascular oxidative stress. Herein, we used a prospective study design to investigate the putative impact of serum iron parameters on the outcome of sepsis.MethodsSerum markers of iron metabolism were measured in a prospective cohort of 61 patients (37 males, 24 females) with sepsis defined by Sepsis-3 criteria in a medical intensive care unit (ICU) and compared between survivors and non-survivors. Regulation of iron parameters in patients stratified by focus of infection and co-medication as well as association of the markers with sepsis severity scores and survival were investigated with linear and logistic regression corrected for sex and age effects.ResultsPositive correlations of increased serum iron and ferritin concentrations upon ICU admission with the severity of organ failure (SOFA score) and with mortality were observed. Moreover, high TF-Sat, elevated ferritin and serum iron levels and low transferrin concentrations were associated with reduced survival. A logistic regression model consisting of SOFA and transferrin saturation (SOFA–TF-Sat) had the best predictive power for survival in septic ICU patients. Of note, administration of blood transfusions prior to ICU admission resulted in increased TF-Sat and reduced survival of septic patients.ConclusionsOur study could show an important impact of serum iron parameters on the outcome of sepsis. Furthermore, we identified transferrin saturation as a stand-alone predictor of sepsis survival and as a parameter of iron metabolism which may in a combined model improve the prediction power of the SOFA score.Trial registrationThe study was carried out in accordance with the recommendations of the Declaration of Helsinki on biomedical research. The study was approved by the institutional ethics review board of the Medical University Innsbruck (study AN2013-0006).

Aviation Sports Crashes in the Austrian Mountains: A 10-Year Retrospective Study

We aimed to describe the epidemiology and injury patterns of aviation sports crashes in the Austrian mountains and identify risk factors. In this retrospective cohort study, out-of-hospital data on patients who sustained crashes when participating in aviation sports from January 1, 2006 through December 31, 2015 were assessed. The out-of-hospital data were merged with in-hospital data obtained from Innsbruck Medical University Hospital. A total of 2037 persons were involved in 1856 aviation sports crashes. Data on 126 in-hospital patients were available. Wind and pilot error were the most common causes. Most injuries occurred in paragliders (n=111, 88%). Most commonly, paragliders sustained injuries to the lumbar spine and hang gliders to the thoracic spine. Rescue operations were undertaken mainly by helicopter emergency medical services (n=87, 69%) or combined rescue forces (ground and helicopter, n=100, 79%.). The Injury Severity Score was 15±15, with a peak in patients with isolated injuries of the lower extremities (n=38, 32%) and a second peak in patients with multiple trauma (n=44, 35%). In the Austrian mountains, wind and pilot errors are the most common causes of aviation crashes. Aviation sports crashes frequently resulted in severe injuries and multiple trauma. The lumbar spine is particularly at risk in paragliders, whereas the thoracic spine is commonly affected in hang gliders. Injuries frequently caused long-term paralysis and limitations in quality of life. To minimize long-term consequences and save lives, skilled and well-equipped teams may be beneficial to provide effective on-site care and safe transportation to a trauma center.

Canyoning Accidents in Austria from 2005 to 2018.

Canyoning has become a popular recreational sport. Nevertheless, little is known about injuries or diseases associated with canyoning. The aim of this study was to examine accident causes, injury patterns, out-of-hospital and in-hospital treatment and outcomes. For this purpose, national out-of-hospital data from the Austrian Alpine Safety Board and regional in-hospital data from Innsbruck Medical University Hospital were analysed for the period from November 1, 2005 to October 31, 2018. Nationally, 471 persons were involved in such accidents; 162 (34.4%) were severely injured, nine of whom died. Jumping (n = 110, 23.4%), rappelling (n = 51, 10.8%), sliding (n = 41, 8.7%) and stumbling (n = 26, 5.5%) were the most common causes of canyoning accidents. A large proportion of injuries were documented for the lower extremities (n = 133, 47.5%), followed by the upper extremities (n = 65, 23.2%) and the spine (n = 44, 15.7%). Death was mainly caused by drowning. Overall mortality was 1.9% (n = 9), and the absolute risk was 0.02 deaths per 1000 hrs of canyoning. Many uninjured persons required evacuation (n = 116, 24.6%), which resulted in a substantial expense and workload for emergency medical services. Increased safety precautions are required to reduce accidents while jumping and rappelling and fatalities caused by drowning.

Early preterm infants with abnormal psychomotor neurodevelopmental outcome at age two show alterations in amplitude-integrated electroencephalography signals

IntroductionRecent studies showed that neurodevelopment in preterm infants can be predicted by using amplitude-integrated electroencephalography (aEEG)-derived parameters. In our previous study we demonstrated that aEEG could be useful in predicting neurodevelopmental outcome in very preterm infants at the corrected age of 2 years. AimThe aim of this study was to further evaluate aEEG for predicting neurodevelopmental outcome at the at the corrected age of 2 years in preterm infants. MethodsBetween July 2010 and June 2016 440 very preterm infants were eligible for the study at Innsbruck Medical University Hospital. The aEEG was evaluated for the Burdjalov score in 306 preterm infants (mean gestational age 29.5 weeks; range: 24.1–31.9 weeks). At the corrected age of 2 years outcome was assessed by the Bayley Scales of Infant and Toddler Development. ResultsThe cohort was divided into three subgroups: 248 infants with normal outcome, 40 infants with delayed outcome and 18 infants with abnormal outcome. Burdjalov scores were lower in infants with delayed outcome than in infants with normal outcome and even lower in infants with abnormal outcome. Post-hoc analysis showed significant differences between normal and delayed psychomotor outcome at 18–24 h (5 (3;6) versus 3 (3;5), p = .024), 30–36 h (6 (4;8) versus 4 (4;6), p = .033), 42–48 h (7 (5;8.5) versus 4 (4;7), p = .003), 54–60 h (7 (6;9) versus 5 (4;7), p = .003), 66–72 h (8 (6;9) versus 6.5 (4.25;7.75), p = .027) and week one (8 (7;10) versus 6.5 (5;8), p = .021). Additionally, when comparing normal to abnormal outcome, a significant difference was found at week four (12 (9;12) versus 8 (7;10), p = .024). The Burdjalov score was only predictive for a delayed psychomotor outcome, presenting the highest area under the curve (0.690) at week two of life. ConclusionWe observed differences in aEEG signals and neurodevelopmental outcome at the corrected age of 2 years, especially for psychomotor outcome. The predictive value of the Burdjalov score regarding neurodevelopmental outcome at the corrected age of 2 years in preterm infants was low.

Sudden Cardiac Arrest and Cardiopulmonary Resuscitation with Automated External Defibrillator in the Austrian Mountains: A Retrospective Study.

Background: Few data exist on the likelihood of surviving sudden cardiac arrest in the mountains. The aim of this study was to analyze the epidemiology and outcomes of patients suffering sudden cardiac arrest and undergoing cardiopulmonary resuscitation (CPR) with automated external defibrillator (AED) in the Austrian mountains. Materials and Methods: We analyzed all cardiac arrest cases in the Austrian mountains reported in the nationwide Austrian Alpine Police database from October 26, 2005, to December 31, 2015. To obtain information on outcomes, these patient data were manually merged with patient data from the main Austrian referral center for mountain emergencies, Innsbruck Medical University Hospital. Results: Overall, 781 cases of sudden cardiac arrest in the Austrian mountains were recorded. In 136 cases (17%), CPR with AED was attempted. The most frequent activities at the time of sudden cardiac arrest were hiking (n = 63, 46%) and skiing or snowboarding (n = 44, 32%). In the nationwide Austrian Alpine Police database, only 4 (3%) patients survived, whereas in the Innsbruck Medical University Hospital database, there were seven survivors who received CPR and AED. All survivors had received immediate CPR with an AED. Five patients had good neurological outcome (cerebral performance category 1-2). Conclusions: In the Austrian mountains, CPR was attempted in less than 20% of sudden cardiac arrest cases. The few that survived had received immediate CPR with an AED. To better understand the circumstances and outcome of sudden cardiac arrest in the mountains, out-of hospital and in-hospital data should be linked.

P6427Positive family history of cardiovascular disease and long-term outcomes after coronary artery bypass grafting in younger patients: a genetic paradox?

Abstract Background Parental cardiovascular disease (CVD) is a known risk factor for premature CVD in both men and women. It is unknown whether a positive family history (PFH) of premature CVD also affects long-term outcomes after coronary artery bypass grafting (CABG). Purpose We estimated the prevalence of PFH of premature CVD in a large population of CABG patients younger than 60 years of age. We evaluated whether PFH is an independent predictor of survival and freedom from major adverse cardiac and cerebrovascular events (MACCEs) following CABG. Methods Data come from a prospective longitudinal study of first, non-emergent, CABG patients consecutively recruited at the Innsbruck Medical University between August 2001 and February 2018 (n=5389). Patients were followed up for a median of 8 years. From this study, 2553 patients with premature CAD undergoing CABG under the age of 60 years were identified. Self-reported PFH data was available for 99.3% of patients; n=2535 patients were eligible for these analyses. In line with the Framingham offspring study, a premature PFH of CVD was defined when a patient's father and/or mother experienced their first CV event at <55 (father) and <65 (mother) years of age, respectively. Adjusted multivariable Cox proportional hazards regression was used to assess the effect of PFH on overall and MACCE-free survival. Results Premature PFH was found in 54.2% of patients (n=1375). Within these patients, 66.1% had a father who experienced a premature CV event (n=909), 27.8% a mother (n=382) and 6.1% both a mother and a father (n=84). In the majority of cases the patient's parent had experienced a premature cardiac event (85.9%, n=1181), 14.1% of patients with PFH reported their parent(s) had a premature stroke (n=194). Patients with a PFH had lower rates of smoking, diabetes and renal disease but were more likely to be hypertensive. Following CABG, PFH was associated with improved long-term survival (adjusted HR, 0.66; 95% CI, 0.50–0.91; p=0.011) and MACCE-free survival (adjusted HR, 0.73; 95% CI, 0.68–0.89; p=0.01). Among the covariates adjusted for, age, diabetes, renal insufficiency, peripheral arterial disease, impaired left ventricular function, previous cerebrovascular events and previous mediastinal radiation were associated with poorer outcomes. In contrast, multiple arterial grafting by bilateral internal thoracic arteries improved both survival (adjusted HR, 0.52; 95% CI, 0.36–0.74; p<0.001), and MACCE-free survival (adjusted HR, 0.41; 95% CI, 0.31–0.54; p<0.001). Conclusion In this cohort of high-risk patients undergoing CABG under 60 years of age, PFH was highly prevalent. Whilst it is evident that a PFH increases the risk of requiring CABG at younger ages, this study shows that PFH is also, paradoxically, protective regarding long-term outcomes; PFH is associated with both improved overall and MACCE-free survival following CABG.

Exploring the limits of hepatic surgery for alveolar echinococcosis—10-years’ experience in an endemic area of Austria

Hepatic alveolar echinococcosis is a slow-growing, destructive parasitosis for which radical surgical resection is currently the only curative treatment. In case of complications by infiltrated vascular or biliary structures, interventional techniques are used. Pharmacotherapy is recommended postoperatively and for inoperability. The rare zoonotic occurs in the northern hemisphere and still poses a challenge in diagnostic and therapeutic management. Based in an endemic area in Austria, we evaluated a decade of surgical treatment for alveolar echinococcosis (AE) at our department. Clinical data of patients undergoing hepatic resection for AE at our department between 2005 and 2014 were collected. Every diagnosis was again verified by histology and PCR. The PNM staging of preoperative imaging was done in six-eye principle. This study was approved by the local Ethics Committee of the Medical University Innsbruck (registration number 20170307-1537). Nine of 12 patients had an R0 resection. In one case, cure was achieved by a combination of surgery and radiofrequency ablation, one patient had R2 resection due to unexpected AE spread and another patient underwent salvage operation with lethal outcome. Three patients experienced early postoperative complications. Late postoperative complications included incisional hernia and cholestatic hepatopathy. 9 patients have been disease free for a mean period of 6 years. The relevance and perioperative risks of an extensive AE resection and avoidance of R2 resections are reflected in the present study. Determination of PNM stage and evaluation of in-all resectability build the foundation for successful surgical treatment even in an advanced AE stage.

Amplitude-integrated electroencephalography shows alterations in children born preterm displaying poor literacy precursor skills.

AimTo assess whether amplitude‐integrated electroencephalography (aEEG) alterations in the newborn period are associated with poor precursor skills of literacy at five years of age in children born preterm.MethodsBetween October 2007 and September 2011 248 preterm infants were eligible for the study at Innsbruck Medical University Hospital. aEEG was analysed for dominating background activity, calculation of the percentage of continuous activity, the Burdjalov scoring system, the minimum, mean and maximum amplitude. At the age of five years, we evaluated preterm born children by the Bielefelder screening (BISC) to assess for early diagnosis of reading problems and weak spelling and classified them as normal performers (n = 64) or poor performers (n = 20). Completion of testing was not possible for one infant.ResultsThe minimum amplitude was significantly lower in the poor BISC performance group as compared to the normal BISC performance group at postnatal week two. The percentage of continuous background activity was significantly higher in infants with normal BISC performance than in infants with poor BISC performance at postnatal week three.ConclusionChildren with poor developed precursor skills of literacy showed alterations in aEEG signals. The aEEG could be useful in further diagnosing preterm infants at risk for developmental complications.

521 Cases of blunt hepatic & splenic injury - multidisciplinary, non-operative management (NOM) in a large Western-Europe Trauma centre

Background: Despite widespread use of NOM for blunt injury to liver and spleen, data from European centres are scarce. We aimed to investigate the outcome of these patients at a large trauma referral centre in Austria and develop a clinical algorithm to detect the risk factors for failure of non-operative management. Methods: Retrospective review based on all emergency patients with admission to Medical University Innsbruck 2006-2016. Injury severity details, clinical, operative and non-operative treatment parameters, morbidity and mortality were evaluated. On these factors a systematic, therapeutic algorithm was created. Results: 521 patients were treated with splenic and/or hepatic injury, 227 had splenic injury, 238 hepatic injury and 56 cases splenic + hepatic trauma. 84.1% underwent initial NOM, of which 2.1% received embolization and/or ERCP. All other NOM cases were managed with bed-rest. The secondary failure rate of NOM was 3.2%. Reasons for failure were: persisting haemorrhage (72%), haematoma (14%) and infections (14%). The rate of patients per year with NOM did not significantly change during the study-period. In-hospital mortality rate was 1.7%. Risk factors for failure of NOM will be presented, as well as a proposed algorithm for management of traumatic liver and spleen injury. Conclusion: NOM is the standard of care for blunt hepatic and splenic injury and successful in >80% of patients. The presented clinical algorithm is primarily guided by clinical parameters and is safe with a low mortality rate <2%.

SYNE1-ataxia: Novel genotypic and phenotypic findings

IntroductionSYNE1 encodes nesprin-1, a scaffold protein which is involved in the binding between cytoskeleton, nuclear envelope and other subcellular compartments. In 2007, recessive truncating SYNE1 mutations have been linked to a genetic form of pure cerebellar ataxia with adult onset and mild phenotype. Subsequent reports described a number of patients with SYNE1-ataxia and widespread neurological involvement including features of motor neuron disease. Recently, heterozygote missense SYNE1 mutations have been associated with muscular disorders, such as Emery-Dreifuss muscular dystrophy, arthrogryposis multiplex congenita and dilated cardiomyopathy. MethodsHerein we describe novel genotypic and phenotypic findings in an independent cohort of 5 patients with SYNE1-ataxia referring to the Department of Neurology of the Innsbruck Medical University and performed a review of the related literature. ResultsWe report 3 novel mutations and describe for the first time myocardial involvement in a patient with a complicated spastic-ataxic phenotype and C-terminal mutation. In the literature, mutations associated with additional motor neuron signs spanned over the entire gene, but patients with a particularly severe phenotype and premature death bore C-terminal mutations. ConclusionOur findings support a genotype-phenotype correlation in SYNE1-ataxia and suggest the need for a systematic cardiologic evaluation in the setting of complicated spastic-ataxia phenotypes.

High Prevalence and Clinical Impact of Malnutrition in Older Patients with a Hematological Malignancy—Basis for Patient Orientated Guidelines and Healthcare Interventions

Intro Malnutrition represents a frequent problem in cancer patients. However, data on the prevalence and the clinical impact of malnutrition in older patients with hematological malignancies are so far rare.Objectives Objective of this study was to investigate prospectively the nutritional status at baseline in older patients with blood cancer and to analyze the impact of malnutrition on clinical performance and outcome. Goal was to form a rational basis for interventions and practice guidelines in this continuously growing group of patients.Methods In 149 newly diagnosed patients with a hematological malignancy aged ≥ 70 years from the Department of Internal Medicine V (Haematology and Oncology), Innsbruck Medical University, different nutritional parameters including loss of appetite, recent weight loss, Mini Nutritional Assessment (MNA), body mass index, serum albumin and the inflammation marker C-reactive protein (CRP) were assessed and compared with status in multidimensional geriatric assessment and with overall survival (OS). SPSS (IBM Corporation, Armonk, NY, USA) was used for all statistical analyses. Two-year overall survival was analyzed with Kaplan-Meier methods and the log rank test. Hazard ratios were first analyzed in a univariate Cox proportional hazard regression. Parameters statistically significant in the univariate analyses were then included in the multivariate Cox regressions, additionally assessing the significance of the parameters with a bootstrapped model. Canoco 5.10 was used for principal component analyses (PCA).Results A moderate or severe decrease of appetite was reported by 23 % and 40% of patients. Moreover, a recent weight loss of 1-3 or >3kg was present in 19 and 31% and a BMI <23kg/m2 was found in 29% of patients. Based on MNA-evaluation, 44% revealed a risk of malnutrition and 14% of manifest malnutrition. Prevalence of lowered serum albumin <3.5 g/dL was detected in 14% of patients. Geriatric impairments >3 were detected in about one third of the patients (36%). PCA demonstrated clustering of impairments in performance status and in IADL as well as of low appetite, low serum albumin, fatigue and depression in the first principal component accounting for a variability of 24%. Loss of appetite, recent weight loss, impaired MNA, low serum albumin and elevated CRP were significantly associated with shortened OS. Recent weight loss >3kg and low BMI remained significant predictive parameters for OS in a Cox regression analysis (HR: 2.01 (1.19-3.38), p=0.009 and HR: 2.46 (1.53-3.96), p<0.001, respectively).Conclusion These analyses reveal malnutrition at initial diagnosis in a relevant proportion of older patients with a hematological malignancy. Importantly, impaired nutritional status is associated with shortened overall survival. The correlation between malnutrition, fatigue, impaired performance and functional capacities, and inflammation might suggest an underlying common pathway. Thus, assessment of nutritional status is essential in studies and in daily practice and forms the basis for interventions to improve nutritional status. More robust data on the clinical impact of malnutrition in hematological malignancies and the effect of geriatric interventions on essential oncologic and non-oncologic outcomes such as survival and quality of life are needed. DisclosuresStauder:Celgene: Honoraria, Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Teva: Research Funding.

Lightning accidents in the Austrian alps \u2013 a 10-year retrospective nationwide analysis

BackgroundLightning strikes are rare but potentially lethal. The risk for suffering a lightning strike in a mountain environment is unknown. The aim of this nationwide study was to analyse all lightning accidents in the Austrian Alps from 2005 to 2015, to assess the circumstances of the accident, the injury pattern as well as the outcome.MethodsFrom 2005 to 2015, data from the national Austrian Alpine Police database as well as the Clinical Information System of Innsbruck Medical University Hospital were searched for the keywords lightning injury, lightning strike, lightning as well as ICD-10 Code T75.0. Additionally, the archive data of Innsbruck Medical University Hospital was searched manually.ResultsThe Austrian Alpine Police database, containing 109.168 patients for the years 2005–2015, was screened for lightning accidents. Sixty-four patients had been hit by lightning in the Austrian Alps, 54 were male. Four persons died on scene; survival rate was 93.8%. Two deceased persons were hunters, who were killed by the same lightning strike. Sixty-three patients suffered a lightning strike while doing a recreational activity, mostly hiking (n = 55), a few hunting and only one doing occupational timberwork. Sixty-three patients suffered a lightning strike between June and August with nearly half (46.9%) of the accidents happening on a Saturday or Sunday, and mainly (95.3%) between 12:00 and 22:00 h.DiscussionPersons who perform recreational outdoor and occupational activities in an alpine environment during summer and after noon incur a higher risk of sustaining a lightning strike. The primary risk group includes young male mountaineers and hunters. The mortality rate was low.Trial registrationThe study was approved by the Ethics Committee of the Medical University of Innsbruck (AN4757 315/4.4) and retrospectively registered with Clinical Trials NCT03405467, January 19, 2018.

The neuropsychiatric phenotype in CACNA1A mutations: a retrospective single center study and review of the literature.

CACNA1A encodes the α1 subunit of the neuronal calcium channel P/Q. CACNA1A mutations underlie three allelic disorders: familial hemiplegic migraine type 1 (FHM1), episodic ataxia type 2 (EA2) and spinocerebellar ataxia type 6 (SCA6). A clear-cut genotype-phenotype correlation is often lacking since clinical manifestations may overlap. Several case reports have described cognitive and behavioral features in CACNA1A disorders, but studies in larger case series are lacking. Genetically confirmed CACNA1A cases were retrieved from the database of the ataxia outpatient clinic of the Department of Neurology at Innsbruck Medical University. Clinical charts and neuropsychological test results were retrospectively analyzed. In addition, a review of the literature including only genetically confirmed cases was performed. Forty-four CACNA1A cases were identified in our database. Delayed psychomotor milestones and poor school performance were described in seven (four FHM1, three EA2) and eight (three FHM1, five EA2) patients, respectively. Psychiatric comorbidities were diagnosed in eight patients (two FHM1, six EA2). Neuropsychological testing was available for 23 patients (11 FHM1, 10 EA2, two SCA6). Various cognitive deficits were documented in 21 cases (all patients except one SCA6). Impairments were predominantly seen in figural memory, visuoconstructive abilities and verbal fluency. In the literature, an early psychomotor delay is described in several children with EA2 and FHM1, whilst reports of cognitive and psychiatric findings from adult cases are scarce. Neuropsychiatric manifestations are common in episodic CACNA1A disorders. In the case of otherwise unexplained developmental delay and a positive family history, CACNA1A mutations should be considered in the differential diagnosis.

Frostbite Injuries in the Austrian Alps: A Retrospective 11-Year National Registry Study.

Frostbite is a cold injury mostly affecting the extremities. The objective of this study was to reveal the incidence of frostbite injuries in the Austrian Alps, to search for frostbite risk factors, and thereby optimize prevention and treatment. Out-of-hospital data in the National Registry of Alpine Accidents from January 1, 2005, to December 31, 2015, were screened for frostbite injuries. Cases in the registry were merged with clinical data from the major trauma center in western Austria, Innsbruck Medical University Hospital, and statistically analyzed. Documented in the National Registry are 114,595 injured persons in the 11-year study period. Thirty-one frostbite cases were documented nationwide, 18 (58%) of which occurred in the western states of Austria and were therefore potentially referred to the Innsbruck Medical University Hospital. Six (19.6%) patients were female. Frostbite was almost exclusively related to fingers and toes (90% of cases). Frostbite injuries in the Austrian Alps are rare. With an incidence of 0.07/100,000, three to four clinically relevant frostbite injuries occur annually. Men are at greater risk for frostbite injuries than women. Fingers and toes are at greatest risk. Proper preparation of outdoor activities and cold-protective gear can help prevent frostbite injuries.