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1.
Anaesthesist ; 69(2): 137-148, 2020 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-32002561

RESUMO

Adequate analgesia is one of the most important measures of emergency care in addition to treatment of vital function disorders and, if indicated, should be promptly undertaken; however, a large proportion of emergency patients receive no or only inadequate pain therapy. The numeric rating scale (NRS) is recommended for pain assessment but is not applicable to every group of patients; therefore, vital signs and body language should be included in the assessment. Pain therapy should reduce the NPRS to <5 points. Ketamine and fentanyl, which have an especially rapid onset of action, and also morphine are suitable for analgesia in spontaneously breathing patients. Basic prerequisites for safe and effective analgesia by healthcare professionals are the use of adequate monitoring, the provision of well-defined emergency equipment, and the mastery of emergency procedures. In a structured competence system, paramedics and nursing personnel can perform safe and effective analgesia.

2.
Anaesthesia ; 2020 Feb 06.
Artigo em Inglês | MEDLINE | ID: mdl-32030734

RESUMO

Intra-operative hypotension is a known predictor of adverse events and poor outcomes following major surgery. Hypotension often occurs on induction of anaesthesia, typically attributed to hypovolaemia and the haemodynamic effects of anaesthetic agents. We assessed the efficacy of fluid optimisation for reducing the incidence of hypotension on induction of anaesthesia. This prospective trial enrolled 283 patients undergoing radical cystectomy and randomly allocated them to goal-directed fluid therapy (n = 142) or standard fluid therapy (n = 141). Goal-directed fluid therapy patients received fluid optimisation based on stroke volume response to passive leg raise before induction; those with positive passive leg raise received intravenous crystalloid fluid boluses until stroke volume was optimised. Baseline mean arterial pressure was measured on the morning of surgery and on arriving in the operating theatre. This post-hoc analysis defined haemodynamic instability as either a > 30% relative drop in mean arterial pressure compared with baseline or absolute mean arterial pressure < 55 mmHg, within 15 min of induction. Forty-two (30%) goal-directed fluid therapy patients underwent fluid optimisation after finding an intravascular fluid deficit via passive leg raise testing; 106 (75%) goal-directed fluid therapy and 112 (79%) standard fluid therapy patients met criteria for haemodynamic instability. There was no significant difference in the incidence of haemodynamic instability between the goal-directed fluid therapy and standard fluid therapy groups using absolute mean arterial pressure drop below 55 mmHg (p = 0.58) or using pre-surgical testing or pre-surgical mean arterial pressure values as baseline (p = 0.21, p = 0.89, respectively); however, the difference in the incidence of haemodynamic instability was significant using the operating theatre baseline mean arterial pressure (p = 0.004). We conclude that fluid optimisation before induction of general anaesthesia did not significantly impact haemodynamic instability.

3.
Anaesthesist ; 2020 Feb 13.
Artigo em Alemão | MEDLINE | ID: mdl-32055885

RESUMO

BACKGROUND AND OBJECTIVE: Difficult airway management is a key skill in preclinical emergency medicine. A lower rate of subjective difficult airways and an increased success rate of endotracheal intubation have been reported for highly trained emergency physicians. The aim of this study was therefore to analyze the effect for different specialists and the individual state of training in the German emergency medical system. MATERIAL AND METHODS: In a retrospective register analysis of 6024 preclinical anesthesia procedures, the frequencies of airway devices, neuromuscular blocking agents, capnography and difficult airways were analyzed with respect to specialization and status of training. Additionally, low, medium and highly experienced emergency physicians in airway management were summarized by specialization and status of training according to the Dreyfus model of skill acquisition and compared. RESULTS: The incidence of subjective difficult airway situations was 10% for anesthesiological emergency physicians compared to 15-20% for other disciplines. The latter used supraglottic airway devices more often (7-9% vs. 4%) and video laryngoscopes less often (3% vs. 5%) compared to anesthesiological emergency physicians. The discipline-related state of training was inhomogeneous and revealed a reduced rate of supraglottic airway devices for internal specialists with further training (10% vs. 2%). Anesthetists specialized in intensive care medicine used capnography less frequently compared to other anesthetists (79% vs. 72%). With higher levels of experience in airway management, the frequency of endotracheal intubation (86% vs. 94%), neuromuscular blocking agents (59% vs. 73%) and video laryngoscopy (3% vs. 6%) increased and the incidence of subjective difficult airway situations (16% vs. 10%) decreased. CONCLUSION: The level of training in airway management especially for non-anesthetists is inhomogeneous. The recently published German S1 guidelines for prehospital airway management recommend education and training as well as the primary use of the video laryngoscope with Macintosh blade. The implementation could lower the incidence of subjective difficult airways.

4.
Ann Oncol ; 31(3): 422-429, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32067684

RESUMO

BACKGROUND: Long-term survival of high-risk neuroblastoma patients is still below 50% despite intensive multimodal treatment. This trial aimed to address whether the addition of two topotecan-containing chemotherapy courses compared to standard induction therapy improves event-free survival (EFS) of these patients. PATIENTS AND METHODS: An open-label, multicenter, prospective randomized controlled trial was carried out at 58 hospitals in Germany and Switzerland. Patients aged 1-21 years with stage 4 neuroblastoma and patients aged 6 months to 21 years with MYCN-amplified tumors were eligible. The primary endpoint was EFS. Patients were randomly assigned to standard induction therapy with six chemotherapy courses or to experimental induction chemotherapy starting with two additional courses of topotecan, cyclophosphamide, and etoposide followed by standard induction chemotherapy (eight courses in total). After induction chemotherapy, all patients received high-dose chemotherapy with autologous hematopoietic stem cell rescue and isotretinoin for consolidation. Radiotherapy was applied to patients with active tumors at the end of induction chemotherapy. RESULTS: Of 536 patients enrolled in the trial, 422 were randomly assigned to the control arm (n = 211) and the experimental arm (n = 211); the median follow-up time was 3.32 years (interquartile range 1.65-5.92). At data lock, the 3-year EFS of experimental and control patients was 34% and 32% [95% confidence Interval (CI) 28% to 40% and 26% to 38%; P = 0.258], respectively. Similarly, the 3-year overall survival of the patients did not differ [54% and 48% (95% CI 46% to 62% and 40% to 56%), respectively; P = 0.558]. The response to induction chemotherapy was not different between the arms. The median number of non-fatal toxicities per patient was higher in the experimental group while the median number of toxicities per chemotherapy course was not different. CONCLUSION: While the burden for the patients was increased by prolonging the induction chemotherapy and the toxicity, the addition of two topotecan-containing chemotherapy courses did not improve the EFS of high-risk neuroblastoma patients and thus cannot be recommended. CLINICAL TRIALS. GOV NUMBER: NCT number 03042429.

5.
Nat Med ; 2020 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-32094925

RESUMO

Retinal gene therapy has shown great promise in treating retinitis pigmentosa (RP), a primary photoreceptor degeneration that leads to severe sight loss in young people. In the present study, we report the first-in-human phase 1/2, dose-escalation clinical trial for X-linked RP caused by mutations in the RP GTPase regulator (RPGR) gene in 18 patients over up to 6 months of follow-up (https://clinicaltrials.gov/: NCT03116113). The primary outcome of the study was safety, and secondary outcomes included visual acuity, microperimetry and central retinal thickness. Apart from steroid-responsive subretinal inflammation in patients at the higher doses, there were no notable safety concerns after subretinal delivery of an adeno-associated viral vector encoding codon-optimized human RPGR (AAV8-coRPGR), meeting the pre-specified primary endpoint. Visual field improvements beginning at 1 month and maintained to the last point of follow-up were observed in six patients.

6.
Retina ; 40(1): 160-168, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30308560

RESUMO

PURPOSE: Choroideremia (CHM) is a rare inherited retinal degeneration resulting from mutation of the CHM gene, which results in absence of functional Rab escort protein 1 (REP1). We evaluated retinal gene therapy with an adeno-associated virus vector that used to deliver a functional version of the CHM gene (AAV2-REP1). METHODS: THOR (NCT02671539) is a Phase 2, open-label, single-center, randomized study. Six male patients (51-60 years) with CHM received AAV2-REP1, by a single 0.1-mL subretinal injection of 10 genome particles during vitrectomy. Twelve-month data are reported. RESULTS: In study eyes, 4 patients experienced minor changes in best-corrected visual acuity (-4 to +1 Early Treatment Diabetic Retinopathy Study [ETDRS] letters); one gained 17 letters and another lost 14 letters. Control eyes had changes of -2 to +4 letters. In 5/6 patients, improvements in mean (95% confidence intervals) retinal sensitivity (2.3 [4.0] dB), peak retinal sensitivity (2.8 [3.5] dB), and gaze fixation area (-36.1 [66.9] deg) were recorded. Changes in anatomical endpoints were similar between study and control eyes. Adverse events were consistent with the surgical procedure. CONCLUSION: Gene therapy with AAV2-REP1 can maintain, and in some cases, improve, visual acuity in CHM. Longer term follow-up is required to establish whether these benefits are maintained.

7.
Resuscitation ; 146: 66-73, 2020 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-31730900

RESUMO

AIM: The aim of this study was to develop a score to predict the outcome for patients brought to hospital following out-of-hospital cardiac arrest (OHCA). METHODS: All patients recorded in the German Resuscitation Registry (GRR) who suffered OHCA 2010-2017, who had ROSC or ongoing CPR at hospital admission were included. The study population was divided into development (2010-2016: 7985) and validation dataset (2017: 1806). Binary logistic regression analysis was used to derive the score. The probability of hospital discharge with good neurological outcome was defined as 1/(1 + e-X), where X is the weighted sum of independent variables. RESULTS: The following variables were found to have a significant positive (+) or negative (-) impact: age 61-70 years (-0·5), 71-80 (-0·9), 81-90 (-1·3) and > = 91 (-2·3); initial PEA (-0·9) and asystole (-1·4); presumable trauma (-1·1); mechanical CPR (-0·3); application of adrenalin > 0 - < 2 mg (-1·1), 2 - <4 mg (-1·6), 4 - < 6 mg (-2·1), 6 - < 8 mg (-2·5) and > = 8 mg (-2·8); pre emergency status without previous disease (+0·5) or minor disease (+0·2); location at nursing home (-0·6), working place/school (+0·7), doctor's office (+0·7) and public place (+0·3); application of amiodarone (+0·4); hospital admission with ongoing CPR (-1·9) or normotension (+0·4); witnessed arrest (+0·6); time from collapse until start CPR 2 - < 10 min (-0·3) and > = 10 min (-0·5); duration of CPR <5 min (+0·6). The AUC in the development dataset was 0·88 (95% CI 0·87-0·89) and in the validation dataset 0·88 (95% CI 0·86-0·90). CONCLUSION: The CaRdiac Arrest Survival Score (CRASS) represents a tool for calculating the probability of survival with good neurological function for patients brought to hospital following OHCA.

8.
Adv Exp Med Biol ; 1185: 165-168, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31884606

RESUMO

Recombinant adeno-associated virus (rAAV) is a widely used tool for gene delivery due to its high efficiency to transduce postmitotic cells. However, host immune reactions targeting AAV can limit its therapeutic benefit in clinical applications. While most studies focused on adaptive immunity, initial innate immune responses are the first line of defense against viral vectors and help modulate subsequent adaptive immune responses. The understanding of innate immune responses to AAV can potentially improve safety and therapeutic efficiency of AAV. This article provides an overview of innate immune responses to AAV vectors.


Assuntos
Dependovirus , Vetores Genéticos , Imunidade Inata , Terapia Genética , Humanos
9.
Schweiz Arch Tierheilkd ; 161(10): 649-658, 2019 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-31586927

RESUMO

INTRODUCTION: Thermographic examination of 157 German Holstein heifer calves was performed to investigate the feasibility of this technique for on-farm recording of surface temperature of the head and to examine potential factors that affect the recordings. Baseline values were obtained from six defined locations on the head including both eyes, both horn buds, the muzzle and the mucous membrane of the muzzle using a high-end thermographic camera (ThermoPro TP8, Firma DIAS Infrared GmbH). Evaluation of the influence of various factors on thermographic measurements showed that ambient temperature had the largest effect on surface temperature of the head (regression coefficient, 0.10 to 0.32, p ≤ 0.01) whereas humidity had no effect (in t-test p ≥ 0.33 over all locations). There was a no correlation between rectal temperature and surface temperature (rp ≤ 0.05). The surface temperature decreased with increasing age of the calves (regression coefficient, - 0.42 to - 0.14, p ≤ 0.01). The agreement between double readings made shortly after one another was excellent at all locations (r ≥ 0.95). The emission of infrared energy varied among different locations; the most infrared energy was emitted by the eyes and the least by the muzzle. Paired locations (eyes and horn buds) had symmetric emission patterns of infrared energy. Measuring the surface temperature of the head of calves in their normal barn environment using a standardised protocol was feasible and thus could potentially be used for monitoring calves under field conditions.

10.
HNO ; 2019 Sep 19.
Artigo em Alemão | MEDLINE | ID: mdl-31538214

RESUMO

BACKGROUND: The current German medical licensing system from 2013 dictates the mandatory keeping of logbooks for final year students. A field-specific logbook for the practical year was created by the national otorhinolaryngology (ORL) society in 2012, which has been in use since then in all academic teaching and university hospitals nationwide. OBJECTIVE: After this time interval it seemed appropriate to evaluate this logbook for its usefulness by associate lecturers and students. The objectives were whether the actual learning targets could be considered relevant, suitable and feasible concerning department location, practical and clinical education for medical students. MATERIAL AND METHODS: A total of 165 associate lecturers of all registered national ORL departments were asked to participate using a 2-step evaluation. The logbook was evaluated by 77 lecturers in general and 17 lecturers also evaluated each of the 78 learning targets. Out of the 24 final year students from the Universities of Freiburg and Dresden who were contacted, 16 evaluated the logbook. RESULTS: Analysis demonstrated that the ORL logbook was used in 55% without alterations to the content and in 36% a medical faculty-specific logbook was used. The lecturers' judgement revealed no significant differences between the two logbooks. They approved the 78 learning targets with a majority vote of between 60-100%. Only 3 learning targets (otoacoustic emission, rhinomanometry and sonography) showed approval of less than 60%, associated with a request for modifications. Students evaluated the ORL logbook as being significantly better than the faculty's version concerning practical education. CONCLUSION: The results demonstrated the nationwide acceptance of the ORL logbook by lecturers and students. The amount of critical learning targets which will have to be changed is moderate. This evaluation confirmed the relevance and balance of the ORL logbook content in the practical year training and the possibility of use independent of the location of ORL departments.

11.
Acta Biomater ; 97: 681-688, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31419565

RESUMO

TiO2 nanostructures represent a key platform for biomedical applications, due to the combination of biocompatibility and high surface area. Especially TiO2 nanotube layers have been widely investigated due to controllable nanotopographic effects as well as for electrodes in electrostimulation experiments. In the present work we produce Ar/H2-reduced 'black' TiO2 nanotube arrays with a strongly enhanced electrical conductivity and explore their interaction with mesenchymal stem cells when used as electrodes to apply electric fields (EF) across the cells. While we observe no significant change in cell adhesion and their focal contact formation on these high conductivity nanotubes, we do observe a rapid stem cell response when EF is engaged using the 'black' TiO2 nanotube arrays as electrodes. Compared to as-formed nanotube arrays, a faster stem cell growth was observed and a lower EF intensity caused an intracellular calcium level elevation. Our results indicate that the increased conductivity in TiO2 nanotubes significantly enhances the early stem cell response to minimal electric field stimuli. STATEMENT OF SIGNIFICANCE: The use of TiO2 nanostructures in biomedical applications is widely investigated, especially considering the nanostructured surface influence on the biomaterial-cell interactions. We have previously shown that an applied electric field (EF) on stem cells grown on TiO2 nanotubes leads to synergistic osteogenic stimulation in the absence of biochemical bone-inducing supplements. Here we report that black (i.e. highly conductive nanotubes obtained by reduction treatments) TiO2 nanotubes enable short-time EF effects on stem cells: we observe a faster stem cell growth and a significantly enhanced early stem cell response to minimal EF stimuli. The application of such nanostructures under electric field is promising for therapeutic interventions for bone regeneration and tissue engineering approaches.

12.
JAMA Ophthalmol ; 2019 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-31465092

RESUMO

Importance: Choroideremia (CHM) is a rare, degenerative, genetic retinal disorder resulting from mutation of the CHM gene, leading to an absence of functional ras-associated binding escort protein 1 (REP1). There is currently no approved treatment for CHM. Objective: To assess the safety and efficacy of retinal gene therapy with an adeno-associated virus vector (AAV2) designed to deliver a functional version of the CHM gene (AAV2-REP1) for treatment of patients with choroideremia. Design, Setting, and Participants: Tübingen Choroideremia Gene Therapy (THOR) was a single-center, phase 2, open-label randomized clinical trial. Data were collected from January 11, 2016, to February 26, 2018. Twenty-four-month data are reported for 6 men with a molecularly confirmed diagnosis of CHM. Intention-to-treat analysis was used. Interventions: Patients received AAV2-REP1 by a single, 0.1-mL subretinal injection of 1011 genome particles during vitrectomy into 1 eye randomly assigned to receive treatment. Main Outcomes and Measures: Primary end point was change in best-corrected visual acuity (BCVA) on the Early Treatment Diabetic Retinopathy Study chart from baseline to month 24 in the treated eye vs the control eye. Secondary end points included microperimetry variables, change in fundus autofluorescence, and spectral-domain optical coherence tomographic evaluations from baseline to month 24 in the treated eye vs the control eye. Results: On enrollment, the mean (SD) age of the 6 men included in the study was 54.9 (4.1) years. The mean (SD) BCVA score was 60.3 (13.4) (approximately 20/63 Snellen equivalent) in the study eyes and 69.3 (20.6) (approximately 20/40 Snellen equivalent) in the control eyes. At 24 months, the BCVA change was 3.7 (7.5) in the treated eyes and 0.0 (5.1) in the control eyes (difference, 3.7; 95% CI, -7.2 to 14.5; P = .43). Mean change in retinal sensitivity was 10.3 (5.5) dB in the treated eyes and 9.7 (4.9) dB in the control eyes (difference, 0.6; 95% CI, -10.2 to 11.4; P = .74). A total of 28 adverse events were reported; all were consistent with the surgical procedure (eg, conjunctival hyperemia, foreign body sensation), and none were regarded as severe. Conclusions and Relevance: Among 6 participants, gene therapy with AAV2-REP1 was associated with maintenance or improvement of visual acuity, although no significant difference was found from control eyes. All safety issues were associated with the surgical procedure and none were judged severe. Continued investigations could more precisely define the efficacy and safety of gene therapy with AAV2-REP1 in CHM. Trial Registration: ClinicalTrials.gov identifier: NCT02671539.

13.
Internist (Berl) ; 60(10): 1111-1117, 2019 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-31444523

RESUMO

Despite many novel diagnostic strategies and advances in treatment, infective endocarditis (IE) remains a severe disease. The epidemiology of IE has shifted and staphylococci have replaced streptococci as the most common cause and nosocomially acquired infections, invasive procedures, indwelling cardiac devices and acquired infections due to intravenous drug abuse are more frequent. The incidence of IE has steadily increased in recent years and the patients affected are older and have more comorbidities. The modern treatment of IE is interdisciplinary. The pharmacotherapy of IE depends on the pathogen and its sensitivity. The presence of a bioprosthetic valve and implantable cardiac devices plays a significant role in selection of antibiotics and duration of treatment. This article provides an update and overview of the current clinical practice in diagnostics and pharmacotherapy of IE in adults with a special focus on partial oral therapy and the role of aminoglycosides.


Assuntos
Antibacterianos/uso terapêutico , Endocardite/tratamento farmacológico , Infecções por Bactérias Gram-Negativas/microbiologia , Infecções por Bactérias Gram-Positivas/microbiologia , Micoses/tratamento farmacológico , Adulto , Comorbidade , Endocardite/diagnóstico , Endocardite/epidemiologia , Endocardite Bacteriana/diagnóstico , Endocardite Bacteriana/tratamento farmacológico , Endocardite Bacteriana/epidemiologia , Infecções por Bactérias Gram-Negativas/diagnóstico , Infecções por Bactérias Gram-Negativas/tratamento farmacológico , Infecções por Bactérias Gram-Positivas/diagnóstico , Infecções por Bactérias Gram-Positivas/tratamento farmacológico , Humanos , Incidência , Micoses/diagnóstico , Micoses/epidemiologia
14.
Epidemiol Infect ; 147: e219, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31364561

RESUMO

In 2013, the national surveillance case definition for West Nile virus (WNV) disease was revised to remove fever as a criterion for neuroinvasive disease and require at most subjective fever for non-neuroinvasive disease. The aims of this project were to determine how often afebrile WNV disease occurs and assess differences among patients with and without fever. We included cases with laboratory evidence of WNV disease reported from four states in 2014. We compared demographics, clinical symptoms and laboratory evidence for patients with and without fever and stratified the analysis by neuroinvasive and non-neuroinvasive presentations. Among 956 included patients, 39 (4%) had no fever; this proportion was similar among patients with and without neuroinvasive disease symptoms. For neuroinvasive and non-neuroinvasive patients, there were no differences in age, sex, or laboratory evidence between febrile and afebrile patients, but hospitalisations were more common among patients with fever (P < 0.01). The only significant difference in symptoms was for ataxia, which was more common in neuroinvasive patients without fever (P = 0.04). Only 5% of non-neuroinvasive patients did not meet the WNV case definition due to lack of fever. The evidence presented here supports the changes made to the national case definition in 2013.

15.
J Magn Reson ; 305: 195-208, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31306985

RESUMO

The design, simulation, assembly and testing of a novel dedicated antisymmetric transmit/receive (Tx/Rx) coil array to demonstrate the feasibility of cardiac magnetic resonance imaging (cMRI) in pigs at 7 T was described. The novel antisymmetric array is composed of eight elements based on mirrored and reversed loop orientations to generate varying B1+ field harmonics for RF shimming. The central four loop elements formed together a pair of antisymmetric L-shaped channels to allow good decoupling between all neighboring elements of the entire array. The antisymmetric array was compared to a standard symmetric rectilinear loop array with an identical housing dimension. Both arrays were driven in the parallel transmit (pTx) mode forming an 8-channel transmit and 16-channel receive (8Tx/16Rx) coil array, where the same posterior array was combined with both anterior arrays. The hardware and imaging performance of the dedicated cardiac arrays were validated and compared by means of electromagnetic (EM) simulations, bench-top measurements, phantom, and ex-vivo MRI experiments with 46 kg female pig. Combined signal-to-noise ratio (SNR), geometry factor (g-factor), noise correlation maps, and high resolution ex-vivo cardiac images were acquired with an in-plane resolution of 0.3 mm × 0.3 mm using both arrays. The novel antisymmetric array enhanced the SNR within the heart by about two times and demonstrated good decoupling and improved control of the B1+ field distributions for RF shimming compared to the standard coil array. Parallel imaging with acceleration factor (R) up to 4 was possible using the novel antisymmetric coil array while maintaining the mean g-factor within the heart region of 1.13.

16.
Nat Commun ; 10(1): 2604, 2019 06 13.
Artigo em Inglês | MEDLINE | ID: mdl-31197157

RESUMO

Quantum communication protocols based on nonclassical correlations can be more efficient than known classical methods and offer intrinsic security over direct state transfer. In particular, remote state preparation aims at the creation of a desired and known quantum state at a remote location using classical communication and quantum entanglement. We present an experimental realization of deterministic continuous-variable remote state preparation in the microwave regime over a distance of 35 cm. By employing propagating two-mode squeezed microwave states and feedforward, we achieve the remote preparation of squeezed states with up to 1.6 dB of squeezing below the vacuum level. Finally, security of remote state preparation is investigated by using the concept of the one-time pad and measuring the von Neumann entropies. We find nearly identical values for the entropy of the remotely prepared state and the respective conditional entropy given the classically communicated information and, thus, demonstrate close-to-perfect security.

17.
Neuromuscul Disord ; 29(4): 321-329, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30954388

RESUMO

Proximal spinal muscular atrophy (SMA) causes severe physical limitations but also has a major impact on the lives of parents. The aim of this study was to investigate participation and mental well-being (burden, emotional distress and satisfaction with participation) of parents of home-living patients with SMA. Caregiver burden was assessed with the Caregiver Strain Index, emotional distress with the Hospital Anxiety and Depression Scale and satisfaction with participation with the Utrecht Scale for Evaluation of Rehabilitation-Participation. Because the majority of parents were mothers of home-living SMA patients (76%), further analyses were restricted to mothers. Seventy-seven percent of mothers of patients with SMA had paid work. A substantial proportion of mothers (76%) perceived high caregiver burden. Burden, emotional distress and satisfaction with participation were comparable between mothers of children and mothers of adults with SMA. Caregivers' participation in leisure activities was significantly related to their perceived level of caregiver burden, emotional distress and satisfaction with participation. Mothers engaging in more social and leisure activities reported lower emotional distress and caregiver burden. Considering the high level of burden attention should be paid to mental well-being of primary caregivers of patients with SMA. Caregivers should be motivated to keep participating in social/leisure activities.

18.
Anaesthesist ; 68(5): 270-281, 2019 May.
Artigo em Alemão | MEDLINE | ID: mdl-30887074

RESUMO

BACKGROUND AND OBJECTIVE: Due to multiple factors the performance of preclinical emergency anesthesia is fraught with risks even for experienced emergency physicians. In order to support emergency physicians in monitoring and management of anesthesia, the German practice management guidelines for preclinical emergency anesthesia in adults were published in 2015; however, current data on adherence to the guidelines are not available. MATERIAL AND METHODS: In a retrospective register analysis of preclinical anesthesia from 2015-2017 in Baden-Württemberg, the recorded anesthetic agents, monitoring, airway management and medical disciplines of emergency physicians were analyzed. The anesthetic agents utilized were compared to the emergency scenarios in the guidelines (e.g. cardiac patients, patients with acute respiratory insufficiency or acute neurological disorder and trauma patients). RESULTS: Midazolam and propofol were predominantly used in the 12,605 cases of preclinical emergency anesthesia evaluated. The adherence to the guidelines was 35% for cardiac patients, 51% for patients with acute respiratory insufficiency or 52% for acute neurological disorders and 79% for trauma patients. Securing the airway was carried out in 88.5 % with endotracheal intubation (capnography 79%). Discipline-related differences occurred in airway management for the devices used, capnography, muscle relaxation and the frequency of the subjectively difficult airway. A higher adherence for trauma patients and patients with acute neurological disorders was found for emergency physicians who were anesthesiologists compared to non-anesthesiologists. CONCLUSION: The study of the current state of preclinical emergency anesthesia in Germany showed a deficient implementation of the pharmacological recommendations for action except for trauma patients. Reasons for divergence could arise due to different availability of rescue equipment, training concepts or discipline of emergency physicians. Suitable education and training could improve the quality of prehospital anesthesia in Germany.

19.
Klin Monbl Augenheilkd ; 236(3): 236-243, 2019 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-30897643

RESUMO

Since its first description in 1872, there has been a lively academic debate about the natural history of choroideremia. Due to the low prevalence of choroideremia, interest in this discussion has been limited to subspecialists. However, the current development of novel, potentially disease-modifying therapies has sparked the attention of a larger professional audience. This review summarises the literature around the natural history of the disease and illustrates its key aspects using a simple two-stage model. Apart from a comprehensive discussion of ubiquitous clinical modalities, the manuscript reviews scientifically relevant questions, such as intra-individual symmetry and the utility of novel endpoints for use in clinical studies. Furthermore, it examines the limitations of past and current studies and develops recommendations for further observational trials.


Assuntos
Coroideremia , Coroideremia/diagnóstico , Coroideremia/terapia , Humanos
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