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Contains fulltext : 287509.pdf (Publisher’s version ) (Open Access) BACKGROUND AND PURPOSE: Patients with neuromuscular conditions are at increased risk of suffering perioperative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here, we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC Workshop on Anaesthesia in Neuromuscular Disorders. METHODS: International experts in the field of (paediatric) anaesthesia, neurology, and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and Embase, the main findings of which were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the AGREE II (Appraisal of Guidelines for Research & Evaluation) reporting checklist. The level of evidence has been adapted according to the SIGN (Scottish Intercollegiate Guidelines Network) grading system. The final consensus statement was subjected to a modified Delphi process. RESULTS: A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for (i) neuromuscular junction disorders, (ii) muscle channelopathies (nondystrophic myotonia and periodic paralysis), (iii) myotonic dystrophy (types 1 and 2), (iv) muscular dystrophies, (v) congenital myopathies and congenital dystrophies, and (vi) mitochondrial and metabolic myopathies. CONCLUSIONS: This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders.

Objective For fenestrated endovascular aneurysm repair (FEVAR), the implementation of the VesselNavigator (Philips Healthcare, Best, The Netherlands) to provide a 3-dimensional vessel roadmap has been shown to reduce patient radiation exposure. Unfortunately, FEVAR radiation doses remain substantial despite utilization of this technology. Traditionally, registration of the live fluoroscopy with the pre-operative CTA is performed via the acquisition of a low-dose cone-beam CT scan. However, this registration can also be accomplished with the acquisition of 2D X-rays using the c-arm in 2 different projection angles. We hypothesized that the 2D image acquisition for vessel roadmap development would result in a significant reduction in patient radiation dose in comparison to the 3D CT registration without compromising image quality or increasing procedural length. Methods This single-center, retrospective study included FEVARs performed from January 2015 to May 2019. For patient data, the cumulative reference air kerma (RAK) was presented as geometric mean and standard deviation. A general linear model with log-normal distribution was used to test the difference in patient RAK between 2D X-ray and 3D CT VesselNavigator registration after adjusting for BMI and the number of vessel fenestrations (1 to 2 vs. 3 to 4). Fluoroscopy time was recorded and used as a surrogate for case complexity. All analyses were done in SAS 9.4 (SAS Institute, Inc., Cary, North Carolina). Results One hundred and sixty four FEVARs were performed on a Philips Allura Xper FD 20 fluoroscopy system equipped with clarity technology. The VesselNavigator registration was completed using 3D CT mapping in 99 cases and 2D X-rays in 65 procedures. On average, utilization of 2D mapping versus 3D mapping for the VesselNavigator resulted in a 20.4% reduction in patient RAK after controlling for BMI and number of vessel fenestrations, P = 0.0135. There was no significant difference in fluoroscopy time between the 2 study groups (P= 0.81) suggesting that image quality was not compromised by the use of 2D mapping leading to the need for additional fluoroscopy. Conclusion Acquisition of 2D films rather than a 3D CT scan for VesselNavigator registration allows for a significant reduction in patient radiation dose during FEVAR without increasing the case complexity or compromising image quality.

To compare short- and mid-term outcomes of patients with femoropopliteal (FP) occlusive disease treated with a retrograde vs antegrade crossing strategy.Few studies have directly compared procedural details and outcomes after retrograde vs antegrade crossing of FP lesions.Patients undergoing retrograde approaches to FP lesions were identified from the multicenter Excellence in Peripheral Artery Disease (XLPAD) registry between 2007 and 2015. These patients were matched 1:1 to patients treated with antegrade crossing strategies based on age, gender, comorbidities, indication for procedure, and lesion characteristics. Technical success, major adverse limb events (MALEs), and overall device cost were compared between retrograde and antegrade-only crossing.A total of 116 patients (58 antegrade and 58 retrograde) were included. The retrograde group had higher prevalence of coronary artery disease and presence of chronic total occlusions. The retrograde approach was associated with significantly longer procedural time (186 ± 70 minutes vs 124.4 ± 60 minutes; P.001), but similar technical success (91.4% vs 96.6%; P=.24). There was no significant difference in perioperative morbidity. Patients treated with a retrograde approach had a lower total amputation rate (8.6% vs 22.4%; P=.04) and no difference in overall mortality (8.6% vs 5.2%; P=.47). Mean procedural costs were similar in the antegrade and retrograde groups.In patients with similar disease characteristics, a retrograde approach to FP occlusive disease was associated with longer procedural time, but improved limb salvage, without significant difference in procedural cost.

Spaß an der eigenen Lehre haben Wenn Lehrende keine Freude an ihrer Tätigkeit empfinden, bereitet dies Probleme für alle Beteiligten. Weitere Didaktik-Tools und -Methoden bringen die Lehrenden dann nur bedingt weiter. Die Lehrenden erfahren in diesem Buch stattdessen, wie sie Schritt für Schritt eine coachende Haltung aufbauen und diese für die Hochschullehre und die Betreuung von Studierenden nutzen können. Durch diese grundlegend andere Herangehensweise an die Hochschullehre können sich Selbstwirksamkeit, Motivation und Freude verstärken oder zurückkehren - bei den Lehrenden und den Studierenden.

Spinal muscular atrophy (SMA) is an autosomal recessive disorder causing progressive proximal muscular, respiratory, and bulbar weakness. We present outcome data on motor function, ventilation, nutrition, and language development of SMA patients treated with nusinersen in Switzerland. This multicenter, observational study included 44 patients. At treatment initiation, after 2 months and then every 4 months we assessed motor function with the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP–INTEND), Hammersmith Functional Motor Scale expanded (HFMSE) and 6-Minute Walk Test (6MWT). At treatment initiation, patients were 0.1–44.6 years old, treatment duration ranged from 6 to 41 months. All 11 SMA type 1 children achieved higher CHOP-INTEND scores at the last assessment compared to treatment initiation, 4 acquired stable sitting. Six type 1 children were

Female gender is considered a risk factor for worse perioperative outcomes after fenestrated endovascular aneurysm repair (FEVAR). We hypothesized that women would have more unfavorable anatomy, increasing case complexity and leading to higher radiation doses. Our aim was to evaluate the effect of gender on radiation dose during FEVARs.This single-center retrospective study was performed from 1/2015 to 2/2018. For patient data, linear model and stepwise variable selection algorithm were used. All dose measurements were log transformed before analysis. Significance level for parameter estimates and corresponding 95% confidence intervals were all transformed back using an exponential function. P-value of0.05 was considered statistically significant. All analyses were performed in SAS 9.4 (SAS Institute Inc., Cary, NC).A total of 169 FEVARs (45 women) were performed on a Philips Allura Xper FD 20 fluoroscopy system equipped with clarity technology. There was no difference in body mass index (BMI) or operative time between genders, P = 0.9. The median reference air kerma for women was significantly lower than that for men (1,672 mGy vs. 2,496 mGy), P 0.001. Women had on average a 28% total dose reduction after controlling for BMI, number of vessels fenestrated, operative time, and type of device, P 0.001. The median fluorography and fluoroscopy doses for women were significantly lower than those for men (973 mGy vs. 1,401 mGy and 659 mGy vs. 1,008 mGy), resulting in a 24% fluorography dose reduction and a 38% fluoroscopy dose reduction for women, P 0.001.FEVARs can be performed successfully in women with comparatively lower radiation doses.

AIM To determine predictors of full-scale IQ (FSIQ) in an international pediatric opsoclonus myoclonus syndrome (OMS) cohort. METHOD In this retrospective and prospective cohort study at three academic medical centers (2006-2013), the primary outcome measure, FSIQ, was categorized based on z-score: above average (≥+1), average (+1 to -1), mildly impaired (-1 to -2), and impaired (

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Background: Risdiplam is an orally administered therapy that modifies pre-mRNA splicing of the survival of motor neuron 2 (SMN2) gene and is approved for the treatment of spinal muscular atrophy. The FIREFISH study is investigating the safety and efficacy of risdiplam in treated infants with type 1 spinal muscular atrophy versus historical controls. The primary endpoint of part 2 of the FIREFISH study showed that infants with type 1 spinal muscular atrophy attained the ability to sit without support for at least 5 s after 12 months of treatment. Here, we report on the safety and efficacy of risdiplam in FIREFISH part 2 over 24 months of treatment. Methods: FIREFISH is an ongoing, multicentre, open-label, two-part study. In FIREFISH part 2, eligible infants (aged 1-7 months at enrolment, with a genetically confirmed diagnosis of spinal muscular atrophy, and two SMN2 gene copies) were enrolled in 14 hospitals in ten countries across Europe, North America, South America, and Asia. Risdiplam was orally administered once daily at 0·2 mg/kg for infants between 5 months and 2 years of age; once an infant reached 2 years of age, the dose was increased to 0·25 mg/kg. Infants younger than 5 months started at 0·04 mg/kg (infants between 1 month and 3 months old) or 0·08 mg/kg (infants between 3 months and 5 months old), and this starting dose was adjusted to 0·2 mg/kg once pharmacokinetic data were available for each infant. The primary and secondary endpoints included in the statistical hierarchy and assessed at month 12 have been reported previously. Here we present the remainder of the secondary efficacy endpoints that were included in the statistical hierarchy at month 24: the ability to sit without support for at least 30 s, to stand alone, and to walk alone, as assessed by the Bayley Scales of Infant and Toddler Development, third edition gross motor subscale. These three endpoints were compared with a performance criterion of 5% that was defined based on the natural history of type 1 spinal muscular atrophy; the results were considered statistically significant if the lower limit of the two-sided 90% CI was above the 5% threshold. FIREFISH is registered with ClinicalTrials.gov, NCT02913482. Recruitment is closed; the 36-month extension period of the study is ongoing. Findings: Between March 13 and Nov 19, 2018, 41 infants were enrolled in FIREFISH part 2. After 24 months of treatment, 38 infants were ongoing in the study and 18 infants (44% [90% CI 31-58]) were able to sit without support for at least 30 s (p

Zum Ende des Jahres 2021 ist die Einfuhrung eines flachendeckenden Neugeborenenscreeningprogramms fur die spinale Muskelatrophie (SMA), spezifisch 5q-SMA, in Deutschland geplant, da inzwischen mehrere gezielte Behandlungsmoglichkeiten existieren. Das Neugeborenenscreening auf 5q-SMA basiert auf dem Nachweis einer homozygoten Deletion von Exon 7 im SMN1-Gen durch eine molekulargenetische Analyse aus der Trockenblutkarte. In allen Fallen muss eine Konfirmationsdiagnostik mittels einer zweiten Blutprobe mit Bestimmung der SMN2-Kopien-Zahl durchgefuhrt werden. Erfahrungen aus den bisher in Teilen Deutschlands durchgefuhrten Pilotprojekten werden dargestellt, Vor- und Nachteile von Screeningprojekten werden diskutiert. Die weitere Beratung und Therapie sollten in einer Abteilung fur Neuropadiatrie mit Erfahrung in der Betreuung von Kindern mit 5q-SMA erfolgen, die alle aktuellen Behandlungsoptionen fur das Kind anbieten kann, damit diese wenn notig innerhalb des ersten Lebensmonats starten kann.

Juvenile myasthenia gravis (JMG) is a rare, antibody-mediated disorder of the neuromuscular junction. Treatment strategies in JMG are largely informed by adult MG treatments as the pathophysiology is similar. Rituximab is increasingly considered as a treatment option in refractory JMG but has not yet been systematically investigated in this patient group We conducted a retrospective study from five international centres with expertise in paediatric myasthenia. 10 JMG patients treated with rituximab were identified. Following rituximab treatment all patients had a reduction in JMG-related hospital admissions. At 24 month follow up, 6 patients (60%) had achieved complete stable remission or pharmacological remission and 7 patients were able to reduce immunomodulatory treatment(s). The main side-effect was infusion-related reactions (30%) which resolved in all patients with symptomatic treatment. We compared our cohort to previously reported JMG cases treated with rituximab and noted similar response rates but a slightly higher side-effect profile. Rituximab is a safe and effective treatment option in moderate to severe JMG and most patients have an improvement in MG symptoms post treatment.

Long-term radiation exposure from fluoroscopically guided interventions (FGIs) can cause cataracts and brain tumors in the operator. We have previously demonstrated that leaded eyewear does not decrease the operator eye radiation dose unless lead shielding has been added to the lateral and inferior portions. Therefore, we have developed a disposable, lightweight, lead-equivalent shield that can be attached to the operator's eyewear that conforms to the face and adheres to the surgical mask. In the present study, we evaluated the efficacy of our new prototype in lowering the operator brain and eye radiation dose when added to both leaded and nonleaded eyewear.The attenuating efficacy of leaded eyewear alone, leaded eyewear plus the prototype, and nonleaded eyewear plus the prototype were compared with no eyewear protection in both a simulated setting and clinical practice. In the simulation, optically stimulated, luminescent nanoDot detectors (Landauer, Inc, Glenwood, Ill) were placed inside the ocular, temporal lobe, and midbrain spaces of a head phantom (ATOM model-701; CIRS, Norfolk, Va). The phantom was positioned to represent a primary operator performing right femoral access. Fluorography was performed on a plastic scatter phantom at 80 kVp for an exposure of 3 Gy reference air kerma. In the clinical setting, nanoDots were placed below the operator's eye both inside and outside the prototype during the FGIs. The median and interquartile ranges were calculated for the dose at each nanoDot location for the phantom and clinical studies. The average dose reduction was also recorded.Wearing standard leaded eyewear alone did not decrease the operator ocular or brain radiation dose. In the phantom experiment, the leaded glasses plus the prototype reduced the radiation dose to the lens, temporal lobe, and midbrain by 83% (P .001), 78% (P .001), and 75% (P .001), respectively. The nonleaded glasses plus the prototype also reduced the dose to the lens, temporal lobe, and midbrain by 85% (P .001), 81% (P .001), and 71% (P .001), respectively. A total of 15 FGIs were included in the clinical setting, with a median reference air kerma of 98.4 mGy. The use of our prototype led to an average operator eye dose reduction of 89% (P .001).Attaching our prototype to both leaded and nonleaded glasses significantly decreased the eye and brain radiation dose to the operator. This face shield attachment provided meaningful radiation protection and should be considered as either a replacement or an adjunct to routine eyewear.

Die spinale Muskelatrophie (SMA: Unter dieser Bezeichnung werden wir nur auf die «5q-assoziierte spinale Muskelatrophie» eingehen) ist eine progrediente neuromuskuläre Krankheit, welche die unteren Motoneurone befällt und zu einer zunehmenden Muskelschwäche führt. Lange Zeit eine der hauptsächlichsten genetischen Ursachen der Kindessterblichkeit, haben die wissenschaftlichen Fortschritte der letzten zwei Jahrzehnte die ersten spezifischen Behandlungen ermöglicht und zu einer Revolution in der Betreuung dieser Patienten geführt. Wir werden hier die Krankheit, die pathogenetischen Mechanismen, das klinische Bild und die derzeit verfügbaren therapeutischen Möglichkeiten besprechen.

proximale liée au chromosome 5q ») est une maladie neuromusculaire progressive touchant les motoneurones inférieurs et causant une faiblesse musculaire croissante. Longtemps considérée comme une des principales causes génétiques de mortalité infantile, les avancées scientifiques des 2 dernières décennies ont permis le développement des premiers traitements spécifiques, marquant une révolution dans la prise en charge. Nous allons ainsi passer en revue la maladie, son mécanisme pathogénique, ses présentations cliniques, et les options thérapeutiques actuellement disponibles.

The introduction of a comprehensive newborn screening program for spinal muscular atrophy (SMA), specifically for 5q-SMA, is planned for the end of 2021 in Germany. Several targeted treatment options have become available for all patients with SMA.Newborn screening for 5q-SMA is based on the detection of a homozygous deletion of exon 7 in the SMN1 gene by molecular genetic analysis from the dried blood card. In all cases a second blood sample must be drawn as a part of confirmation diagnostics including the determination of the SMN2 copy numbers.Insights from pilot projects performed in parts of Germany are presented. Advantages and disadvantages of the screening project are discussed.Consultation and treatment should be carried out in a department of neuropediatrics with experience in the treatment of children with 5q-SMA, which is able to provide all current treatment options for the child, so that, when necessary, the treatment can be started within the first month of life.HINTERGRUND: Zum Ende des Jahres 2021 ist die Einführung eines flächendeckenden Neugeborenenscreeningprogramms für die spinale Muskelatrophie (SMA), spezifisch 5q-SMA, in Deutschland geplant, da inzwischen mehrere gezielte Behandlungsmöglichkeiten existieren.Das Neugeborenenscreening auf 5q-SMA basiert auf dem Nachweis einer homozygoten Deletion von Exon 7 im SMN1-Gen durch eine molekulargenetische Analyse aus der Trockenblutkarte. In allen Fällen muss eine Konfirmationsdiagnostik mittels einer zweiten Blutprobe mit Bestimmung der SMN2-Kopien-Zahl durchgeführt werden.Erfahrungen aus den bisher in Teilen Deutschlands durchgeführten Pilotprojekten werden dargestellt, Vor- und Nachteile von Screeningprojekten werden diskutiert.Die weitere Beratung und Therapie sollten in einer Abteilung für Neuropädiatrie mit Erfahrung in der Betreuung von Kindern mit 5q-SMA erfolgen, die alle aktuellen Behandlungsoptionen für das Kind anbieten kann, damit diese wenn nötig innerhalb des ersten Lebensmonats starten kann.

Mitochondrial aconitase is the second enzyme in the tricarboxylic acid (TCA) cycle catalyzing the interconversion of citrate into isocitrate and encoded by the nuclear gene ACO2. A homozygous pathogenic variant in the ACO2 gene was initially described in 2012 resulting in a novel disorder termed "infantile cerebellar retinal degeneration" (ICRD, OMIM#614559). Subsequently, additional studies reported patients with pathogenic ACO2 variants, further expanding the genetic and clinical spectrum of this disorder to include milder and later onset manifestations. Here, we report an international multicenter cohort of 16 patients (of whom 7 are newly diagnosed) with biallelic pathogenic variants in ACO2 gene. Most patients present in early infancy with severe truncal hypotonia, truncal ataxia, variable seizures, evolving microcephaly, and ophthalmological abnormalities of which the most dominant are esotropia and optic atrophy with later development of retinal dystrophy. Most patients remain nonambulatory and do no acquire any language, but a subgroup of patients share a more favorable course. Brain magnetic resonance imaging (MRI) is typically normal within the first months but global atrophy gradually develops affecting predominantly the cerebellum. Ten of our patients were homozygous to the previously reported c.336C>G founder mutation while the other six patients were all compound heterozygotes displaying 10 novel mutations of whom 2 were nonsense predicting a deleterious effect on enzyme function. Structural protein modeling predicted significant impairment in aconitase substrate binding in the additional missense mutations. This study provides the most extensive cohort of patients and further delineates the clinical, radiological, biochemical, and molecular features of ACO2 deficiency.

The autologous deep vein is a suitable alternative to prosthetic or cadaveric grafts as a bypass conduit because of its superior durability and lower risk of complications. Aneurysmal degeneration of deep vein grafts is rarely seen but can potentially be fatal when it does occur. We describe the case of an 87-year-old woman who presented with acute vaginal bleeding due to the rupture of an aneurysmal femorofemoral bypass vein graft into the vagina. The patient presented 13 years after the initial procedure, highlighting the need for long-term monitoring and the early recognition of this potentially catastrophic complication. Keywords: Infected bypass graft, Aneurysm, Rupture of femoral-femoral bypass graft

ZusammenfassungIm Dezember 2020 hat der Gemeinsame Bundesausschuss beschlossen, dass das Screening auf spinale Muskelatrophie (SMA) in das allgemeine Neugeborenenscreening aufgenommen werden soll. Grundlage dieser Entscheidung war die Tatsache, dass inzwischen gezielte Behandlungsmöglichkeiten für die Patienten mit SMA zur Verfügung stehen und der Zeitpunkt, zu dem die Behandlung begonnen wird, entscheidend für den Erfolg der Therapie ist.Das Neugeborenenscreening auf eine SMA basiert auf dem Nachweis einer homozygoten Deletion von Exon 7 im SMN1-Gen durch eine molekulargenetische Analyse aus der Trockenblutkarte. In allen Fällen muss eine Bestätigungsdiagnostik aus einer zweiten Blutprobe im Rahmen der Konfirmationsdiagnostik mit Bestimmung der SMN2-Kopien-Zahl durchgeführt werden. Die weitere Beratung und Therapie sollten in einer neuropädiatrischen Ambulanz mit Erfahrung in der Betreuung von Kindern mit SMA erfolgen.

Contains fulltext : 236903.pdf (Publisher’s version ) (Closed access)

BACKGROUND Type 1 spinal muscular atrophy (SMA) is a progressive neuromuscular disease characterized by an onset at 6 months of age or younger, an inability to sit without support, and deficient levels of survival of motor neuron (SMN) protein. Risdiplam is an orally administered small molecule that modifies SMN2 pre-messenger RNA splicing and increases levels of functional SMN protein in blood. METHODS We conducted an open-label study of risdiplam in infants with type 1 SMA who were 1 to 7 months of age at enrollment. Part 1 of the study (published previously) determined the dose to be used in part 2 (reported here), which assessed the efficacy and safety of daily risdiplam as compared with no treatment in historical controls. The primary end point was the ability to sit without support for at least 5 seconds after 12 months of treatment. Key secondary end points were a score of 40 or higher on the Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP-INTEND; range, 0 to 64, with higher scores indicating better motor function), an increase of at least 4 points from baseline in the CHOP-INTEND score, a motor-milestone response as measured by Section 2 of the Hammersmith Infant Neurological Examination (HINE-2), and survival without permanent ventilation. For the secondary end points, comparisons were made with the upper boundary of 90% confidence intervals for natural-history data from 40 infants with type 1 SMA. RESULTS A total of 41 infants were enrolled. After 12 months of treatment, 12 infants (29%) were able to sit without support for at least 5 seconds, a milestone not attained in this disorder. The percentages of infants in whom the key secondary end points were met as compared with the upper boundary of confidence intervals from historical controls were 56% as compared with 17% for a CHOP-INTEND score of 40 or higher, 90% as compared with 17% for an increase of at least 4 points from baseline in the CHOP-INTEND score, 78% as compared with 12% for a HINE-2 motor-milestone response, and 85% as compared with 42% for survival without permanent ventilation (P

Several hundred genetic muscle diseases have been described, all of which are rare. Their clinical and genetic heterogeneity means that a genetic diagnosis is challenging. We established an international consortium, MYO-SEQ, to aid the work-ups of muscle disease patients and to better understand disease etiology., info:eu-repo/semantics/published

Symptomatic chronic mesenteric ischemia (CMI) is a relatively rare condition, yet there are significant clinical consequences for those affected by the disease. CMI not only reduces quality of life but also increases an individual's risk for life-threatening acute mesenteric ischemia. Surgery is the definitive treatment to reduce the risk for this potentially fatal outcome, and both endovascular and open surgical strategies are currently utilized. This review article discusses the approach to making a diagnosis of CMI, as well as the surgical techniques used to revascularize the mesenteric circulation.

Ocular radiation exposure from fluoroscopically guided interventions (FGIs) can cause cataracts. Standard lead eyewear may not significantly reduce eye radiation dose as the majority of scattered radiation penetrates the operator's eye obliquely. Our aim was to evaluate the efficacy of standard leaded eyewear and a customized eyewear design in lowering eye radiation dose to vascular surgeons.The attenuating efficacy of three forms of leaded eyewear (standard eyewear, eyewear with built-in leaded side shields, and our modified eyewear) was tested in both a simulated setting and clinical practice. The modified design consisted of safety eyewear with 0.75 mm of added lead shielding attached to the lateral and inferior borders of the eyewear frame to attenuate oblique radiation. We performed simulated experiments using an anthropomorphic head phantom (ATOM model 701; CIRS, Norfolk, Va) positioned to represent a primary operator performing right femoral access. Optically stimulated, luminescent nanoDot detectors (Landauer, Glenwood, Ill) were placed inside the phantom's ocular spaces and at the surface of the left eye within and outside the leaded glasses to measure the eye radiation dose reduction provided by each eyewear type. All three eyewear types were also tested during clinical FGIs by placing nanoDots below the operator's left eye, inside and outside of the eyewear coverage. Means and standard errors were calculated using a pooled linear mixed model with repeated measurements.This prospective, single-center study included 60 FGIs, 30 with traditional eyewear and 30 with our modified design. There was no significant eye radiation dose reduction (P.05) with the standard eyewear or leaded side shield eyewear in both the simulated and clinical settings. In the simulated environment, our modified design resulted in an 86% radiation dose reduction to the surface of the left eye and an 80% reduction in left lens radiation dose (P .0001). In the clinical FGIs, the modified eyewear led to a 62% left ocular radiation dose reduction (P .0001).Standard lead-equivalent glasses are ineffective at reducing ocular radiation dose during FGIs. Eyewear modification with lateral and inferior lead shielding molded to the operator's face significantly decreases radiation exposure to the eye closest to the X-ray source.

The development of new therapeutic agents for the treatment of Duchenne muscular dystrophy has put a focus on defining outcome measures most sensitive to capture treatment effects. This cross-sectional analysis investigates the relation between validated clinical assessments such as the 6-minute walk test, motor function measure and quantitative muscle MRI of thigh muscles in ambulant Duchenne muscular dystrophy patients, aged 6.5 to 10.8 years (mean 8.2, SD 1.1). Quantitative muscle MRI included the mean fat fraction using a 2-point Dixon technique, and transverse relaxation time (T2) measurements. All clinical assessments were highly significantly inter-correlated with p

The mechanical recyclability of the technical biopolymers polytrimethylene terephthalate (PTT), cellulose acetate butyrate (CAB), polybutylene succinate (PBS) and polyhydroxy alkanoate blend (PHBV/PBAT) was evaluated by assessing the effect of repeated polymer processing (extrusion without further compounding with virgin material or additives) on the material structure and mechanical properties. Reprocessing-induced hydrolytic degradation was found to be the prevalent aging mechanism of the investigated biopolymer grades. However, susceptibility to hydrolysis, and thus maintenance of the performance characteristics, differed strongly between the biopolymer types. To that effect, PTT and CAB especially exhibit a high potential for mechanical recycling. By taking advantage of appropriate additivation, the mechanical recyclability of PBS and PHBV/PBAT is also assumed to be high.

A broad spectrum of commercially available technical biopolymers is screened with respect to applicability as absorber materials in solar collectors. Technical biopolymers nominated as potentially suitable (bio-polyamide, poly(lactic acid), cellulose acetate, cellulose acetate butyrate, poly(trimethylene terephthalate)) are characterized in detail and in particular with regards to their application relevant aging behaviour. Candidate materials are subjected to various exposure conditions (in water at 35 °C and 80 °C, in air at 60 °C, 70 °C and 80 °C and to the Xenontest – depending on potential collector type) for up to 1000 h. Continuously both application relevant and aging-indicative properties such as thermal, mechanical and morphological characteristics are analysed. Consequently property changes and underlying aging mechanisms are discussed. Based on these results potential application opportunities of the materials are considered. Concisely, the study reveals a high potential of technical biopolymers for solar thermal applications.