Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unre...Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients ful ll criteria for acute liver failure (ALF). Methods: Forty children diagnosed with AIH in our center between 2000 and 2018 were included in this study. All of them ful lled revised diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) for probable or con rmed AIH, and other etiologies of liver diseases were excluded. Patients were divided into two groups: acute AIH (A-AIH) or chronic AIH (C-AIH). Results: Acute onset of AIH occurred in 19/40 children (48%). Six of them ful lled the criteria of ALF with coagulopathy and encephalopathy. Five of 6 children with ALF suffered from exacerbation of previ- ously undiagnosed chronic AIH, among which 4 children were histologically con rmed as micronodular cirrhosis. The remaining one patient had fulminant AIH with centrilobular necrosis, but no histological signs of previous chronic liver damage. We observed signi cantly lower levels of albumin, higher levels of aminotransferases, bilirubin, INR, IgG, higher IAIHG score and more severe histological ndings in A- AIH than in C-AIH. No differences in patient age and presence of autoantibodies were observed between A-AIH and C-AIH. All children, including those with ALF and cirrhosis, were treated with corticosteroids, and are alive and achieved AIH remission. Liver transplant was not indicated in any patient. Conclusion: Rapid and accurate diagnosis of A-AIH may be di cult. However, timely start of immunosup-pressive therapy improves prognosis and decreases number of indicated liver transplantations in children with AIH.展开更多
3D printing is nowadays used for many applications far beyond pure rapid prototyping.As tools to prepare custom-made objects which may be highly complex,different 3D printing techniques have emerged into areas of appl...3D printing is nowadays used for many applications far beyond pure rapid prototyping.As tools to prepare custom-made objects which may be highly complex,different 3D printing techniques have emerged into areas of application where the mechanical,thermal,optical and other properties have to meet high requirements.Amongst them,applications for space,e.g.for microsatellites,make extreme demands regarding the stability under high temperatures.Nevertheless,polymeric 3D printed materials have several advantages for space application in comparison with metal objects.Here we thus investigate the impact of temperatures up to 85◦C and 185◦C,respectively,on typical 3D printing materials for fused deposition modeling or stereolithography(SLA)with inexpensive 3D printers.The materials are found to differ strongly in terms of mechanical properties and dimensional stability after the treatment at a higher temperature,with SLA resins and co-polyester showing the best dimensional stability,while acrylonitrile–butadiene–styrene and SLA resin after long UV post-treatment has the best mechanical properties.展开更多
Microelectromechanical system(MEMS)devices,such as accelerometers,are widely used across industries,including the automotive,consumer electronics,and medical industries.MEMS are efficiently produced at very high volum...Microelectromechanical system(MEMS)devices,such as accelerometers,are widely used across industries,including the automotive,consumer electronics,and medical industries.MEMS are efficiently produced at very high volumes using large-scale semiconductor manufacturing techniques.However,these techniques are not viable for the costefficient manufacturing of specialized MEMS devices at low-and medium-scale volumes.Thus,applications that require custom-designed MEMS devices for markets with low-and medium-scale volumes of below 5000–10,000 components per year are extremely difficult to address efficiently.The 3D printing of MEMS devices could enable the efficient realization and production of MEMS devices at these low-and medium-scale volumes.However,current micro-3D printing technologies have limited capabilities for printing functional MEMS.Herein,we demonstrate a functional 3D-printed MEMS accelerometer using 3D printing by two-photon polymerization in combination with the deposition of a strain gauge transducer by metal evaporation.We characterized the responsivity,resonance frequency,and stability over time of the MEMS accelerometer.Our results demonstrate that the 3D printing of functional MEMS is a viable approach that could enable the efficient realization of a variety of custom-designed MEMS devices,addressing new application areas that are difficult or impossible to address using conventional MEMS manufacturing.展开更多
Geospatial data are fundamental in most global-change and sustainability-related domains.However,readily accessible information on data quality and provenance is often missing or hardly accessible for users due to tec...Geospatial data are fundamental in most global-change and sustainability-related domains.However,readily accessible information on data quality and provenance is often missing or hardly accessible for users due to technical or perceptual barriers,for example,due to unstructured metadata information or missing references.Within an interdisciplinary process encompassing perspectives of data users,data producers,and software developers,we identified major needs to facilitate effectivefitness-for-use assessments by data users and developed approaches to address these.We provided a stylized analysis of large-scale land use data to showcase selected approaches.To support data users,interoperable quality and provenance information need to be meaningfully represented.Data producers need efficient workflows and tools supporting them in creating high-quality,structured and detailed quality and provenance information.Our newly developed approaches to increase the availability of structured metadata synthesize new and existing tools to extract metadata or to generate provenance data during processing.Within our approaches to improve interoperability and accessibility we present novel tools to support(i)the creation of curated and linked registers of data quality indicators and thematic terms,and(ii)linked visualization of data quality and provenance information.Following our approaches increases transparency,facilitatesfitness-for-use assessments,and ultimately improves research quality.展开更多
Whereas prone positioning of intubated patients suffering from acute respiratory distress syndrome represents the standard of care,proning non-intubated patients,so-called“awake prone positioning(APP),”has only rece...Whereas prone positioning of intubated patients suffering from acute respiratory distress syndrome represents the standard of care,proning non-intubated patients,so-called“awake prone positioning(APP),”has only recently gained popularity and undergone scientific evaluation.In this review,we summarize current evidence on physiological and clinical effects of APP on patients’centered outcomes,such as intubation and mortality,the safety of the technique,factors and predictors of success,practical issues for optimal implementation,and future areas of research.Current evidence supports using APP among patients suffering from acute hypoxemic respiratory failure due to COVID-19 and undergoing advanced respiratory support,such as high-flow nasal cannula,in an intensive care unit setting.Healthcare teams should aim to prone patients at least 8 h daily.Future research should focus on optimizing the tolerance of the technique and comprehensively evaluating benefits in other patient populations.展开更多
BACKGROUND： Sclerosing cholangitis（SC） is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC acco...BACKGROUND： Sclerosing cholangitis（SC） is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS： We retrospectively followed up 25 children with SC over a period of 4-17 years（median 12）. The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis（ASC）; other patients were included in a group of primary sclerosing cholangitis（PSC）. The incidence of the following complications was studied： obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS： Fourteen（56%） patients had PSC and 11（44%） had ASC. Patients with ASC were significantly younger at the time of diagnosis（12.3 vs 15.4 years, P=0.032） and had higher Ig G levels（22.7 vs 17.2 g/L, P=0.003）. The mentioned complications occurred in 4（16%） patients with SC, exclusively in the PSC group： one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION： In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.展开更多
Dear Editor,Deep burrowing and soil surface feeding earthworms,so-called strict-anecic and epi-anecic species(Hoeffner et al.,2019),are a highly influential soil faunal group in many natural and manmade environments.T...Dear Editor,Deep burrowing and soil surface feeding earthworms,so-called strict-anecic and epi-anecic species(Hoeffner et al.,2019),are a highly influential soil faunal group in many natural and manmade environments.Typically,they are relatively large and contribute substantially to the formation and processes of mull-type soils through effective foraging and incorporation of plant litter(Edwards and Bohlen,1996).The wide,vertical,and deep burrows of anecic earthworms produce a unique influence on soil physical properties(Lamparski,1985;Shipitalo and Butt,1999;Nuutinen et al.,2001).Lumbricus badensis(Michaelsen 1907)is an intriguing example of this type of earthworm.展开更多
Introduction:Pazopanib is an oral protein kinase inhibitor(PKI)that targets vascular endothelial growth factor(VEGF)receptors,fibroblastic growth factor receptors,platelet-derived growth factor receptors,and stem cell...Introduction:Pazopanib is an oral protein kinase inhibitor(PKI)that targets vascular endothelial growth factor(VEGF)receptors,fibroblastic growth factor receptors,platelet-derived growth factor receptors,and stem cell factor that inhibits VEGF-induced cellular proliferation.Pazopanib is approved for use in advanced renal cell carcinoma and subtypes of advanced soft-tissue sarcoma(Deguchi et al.,2018).Major adverse drug reactions of pazopanib include hypertension,high-grade hyponatremia and posterior reversible encephalopathy syndrome(PRES)(Berardi et al.,2016;Deguchi et al.,2018).In clinical trials,few investigations have been conducted to determine the aetiology of PKI-associated hyponatremia,the mechanism remains therefore unknown.Only rare cases of PKI-induced syndrome of inappropriate secretion of antidiuretic hormone(SIADH)(Largeau et al.,2019),and none with pazopanib,have been reported.PRES is a clinical and radiological entity where a bilateral white matter oedema,occurring predominantly in the posterior occipital and parietal lobes,is associated with several neurologic symptoms.Interestingly,a recent review suggests that SIADH could be a symptom of PRES(Largeau et al.,2019).To our knowledge,this is the first case published where pazopanib-induced PRES occurs contemporaneously with possible SIADH.展开更多
基金supported by grants from the European Re-gional Development Fund-Project ENOCH(CZ.02.1.01/0.0/0.0/16_019/0000868)the Ministry of Health,Czech Republic–conceptual development of research organization(MH DRO grant FNOL,0098892)
文摘Background: Autoimmune hepatitis (AIH) is a rare progressive liver disease, which manifests as acute hepatitis in 40%-50% of pediatric cases. This refers predominantly to spontaneous exacerbations of previ- ously unrecognized subclinical AIH with laboratory and histological signs of chronic hepatitis, or to acute exacerbations of known chronic disease. Only a few of these patients ful ll criteria for acute liver failure (ALF). Methods: Forty children diagnosed with AIH in our center between 2000 and 2018 were included in this study. All of them ful lled revised diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) for probable or con rmed AIH, and other etiologies of liver diseases were excluded. Patients were divided into two groups: acute AIH (A-AIH) or chronic AIH (C-AIH). Results: Acute onset of AIH occurred in 19/40 children (48%). Six of them ful lled the criteria of ALF with coagulopathy and encephalopathy. Five of 6 children with ALF suffered from exacerbation of previ- ously undiagnosed chronic AIH, among which 4 children were histologically con rmed as micronodular cirrhosis. The remaining one patient had fulminant AIH with centrilobular necrosis, but no histological signs of previous chronic liver damage. We observed signi cantly lower levels of albumin, higher levels of aminotransferases, bilirubin, INR, IgG, higher IAIHG score and more severe histological ndings in A- AIH than in C-AIH. No differences in patient age and presence of autoantibodies were observed between A-AIH and C-AIH. All children, including those with ALF and cirrhosis, were treated with corticosteroids, and are alive and achieved AIH remission. Liver transplant was not indicated in any patient. Conclusion: Rapid and accurate diagnosis of A-AIH may be di cult. However, timely start of immunosup-pressive therapy improves prognosis and decreases number of indicated liver transplantations in children with AIH.
基金The study was partly funded by the German Federal Ministry for Economic Affairs and Energy via the AiF,based on a resolution of the German Bundestag,Grant Number KK5129708TA1by the Silesian University of Technology Rector’s Grant No.14/030/RGJ21/00110.We acknowledge funding of the climate chamber by the Ministry of Culture and Science of the State of North Rhine-Westphalia via the‘Zukunftsfonds’.
文摘3D printing is nowadays used for many applications far beyond pure rapid prototyping.As tools to prepare custom-made objects which may be highly complex,different 3D printing techniques have emerged into areas of application where the mechanical,thermal,optical and other properties have to meet high requirements.Amongst them,applications for space,e.g.for microsatellites,make extreme demands regarding the stability under high temperatures.Nevertheless,polymeric 3D printed materials have several advantages for space application in comparison with metal objects.Here we thus investigate the impact of temperatures up to 85◦C and 185◦C,respectively,on typical 3D printing materials for fused deposition modeling or stereolithography(SLA)with inexpensive 3D printers.The materials are found to differ strongly in terms of mechanical properties and dimensional stability after the treatment at a higher temperature,with SLA resins and co-polyester showing the best dimensional stability,while acrylonitrile–butadiene–styrene and SLA resin after long UV post-treatment has the best mechanical properties.
基金supported by the Swedish Foundation for Strategic Research(SSF)(GMT14-0071)the Wenner-Gren scholarship(UPD2020-0119).
文摘Microelectromechanical system(MEMS)devices,such as accelerometers,are widely used across industries,including the automotive,consumer electronics,and medical industries.MEMS are efficiently produced at very high volumes using large-scale semiconductor manufacturing techniques.However,these techniques are not viable for the costefficient manufacturing of specialized MEMS devices at low-and medium-scale volumes.Thus,applications that require custom-designed MEMS devices for markets with low-and medium-scale volumes of below 5000–10,000 components per year are extremely difficult to address efficiently.The 3D printing of MEMS devices could enable the efficient realization and production of MEMS devices at these low-and medium-scale volumes.However,current micro-3D printing technologies have limited capabilities for printing functional MEMS.Herein,we demonstrate a functional 3D-printed MEMS accelerometer using 3D printing by two-photon polymerization in combination with the deposition of a strain gauge transducer by metal evaporation.We characterized the responsivity,resonance frequency,and stability over time of the MEMS accelerometer.Our results demonstrate that the 3D printing of functional MEMS is a viable approach that could enable the efficient realization of a variety of custom-designed MEMS devices,addressing new application areas that are difficult or impossible to address using conventional MEMS manufacturing.
基金funding GeoKur under grant numbers 16QK04A,16QK04B,and 16QK04C.
文摘Geospatial data are fundamental in most global-change and sustainability-related domains.However,readily accessible information on data quality and provenance is often missing or hardly accessible for users due to technical or perceptual barriers,for example,due to unstructured metadata information or missing references.Within an interdisciplinary process encompassing perspectives of data users,data producers,and software developers,we identified major needs to facilitate effectivefitness-for-use assessments by data users and developed approaches to address these.We provided a stylized analysis of large-scale land use data to showcase selected approaches.To support data users,interoperable quality and provenance information need to be meaningfully represented.Data producers need efficient workflows and tools supporting them in creating high-quality,structured and detailed quality and provenance information.Our newly developed approaches to increase the availability of structured metadata synthesize new and existing tools to extract metadata or to generate provenance data during processing.Within our approaches to improve interoperability and accessibility we present novel tools to support(i)the creation of curated and linked registers of data quality indicators and thematic terms,and(ii)linked visualization of data quality and provenance information.Following our approaches increases transparency,facilitatesfitness-for-use assessments,and ultimately improves research quality.
文摘Whereas prone positioning of intubated patients suffering from acute respiratory distress syndrome represents the standard of care,proning non-intubated patients,so-called“awake prone positioning(APP),”has only recently gained popularity and undergone scientific evaluation.In this review,we summarize current evidence on physiological and clinical effects of APP on patients’centered outcomes,such as intubation and mortality,the safety of the technique,factors and predictors of success,practical issues for optimal implementation,and future areas of research.Current evidence supports using APP among patients suffering from acute hypoxemic respiratory failure due to COVID-19 and undergoing advanced respiratory support,such as high-flow nasal cannula,in an intensive care unit setting.Healthcare teams should aim to prone patients at least 8 h daily.Future research should focus on optimizing the tolerance of the technique and comprehensively evaluating benefits in other patient populations.
基金supported by a grant from the Czech Ministry of Education,Youth and Sports(NPU LO 1304)
文摘BACKGROUND： Sclerosing cholangitis（SC） is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate longterm results in children with SC according to the types of SC.METHODS： We retrospectively followed up 25 children with SC over a period of 4-17 years（median 12）. The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis（ASC）; other patients were included in a group of primary sclerosing cholangitis（PSC）. The incidence of the following complications was studied： obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease.RESULTS： Fourteen（56%） patients had PSC and 11（44%） had ASC. Patients with ASC were significantly younger at the time of diagnosis（12.3 vs 15.4 years, P=0.032） and had higher Ig G levels（22.7 vs 17.2 g/L, P=0.003）. The mentioned complications occurred in 4（16%） patients with SC, exclusively in the PSC group： one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, twoother children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications.CONCLUSION： In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.
基金Field work of Drs.Kevin R.BUTT and Visa NUUTINEN in the Schwarzwald area was supported by Faculty-based funds(No.KSG500)a travel grant from the University of Central Lancashire,UK.
文摘Dear Editor,Deep burrowing and soil surface feeding earthworms,so-called strict-anecic and epi-anecic species(Hoeffner et al.,2019),are a highly influential soil faunal group in many natural and manmade environments.Typically,they are relatively large and contribute substantially to the formation and processes of mull-type soils through effective foraging and incorporation of plant litter(Edwards and Bohlen,1996).The wide,vertical,and deep burrows of anecic earthworms produce a unique influence on soil physical properties(Lamparski,1985;Shipitalo and Butt,1999;Nuutinen et al.,2001).Lumbricus badensis(Michaelsen 1907)is an intriguing example of this type of earthworm.
文摘Introduction:Pazopanib is an oral protein kinase inhibitor(PKI)that targets vascular endothelial growth factor(VEGF)receptors,fibroblastic growth factor receptors,platelet-derived growth factor receptors,and stem cell factor that inhibits VEGF-induced cellular proliferation.Pazopanib is approved for use in advanced renal cell carcinoma and subtypes of advanced soft-tissue sarcoma(Deguchi et al.,2018).Major adverse drug reactions of pazopanib include hypertension,high-grade hyponatremia and posterior reversible encephalopathy syndrome(PRES)(Berardi et al.,2016;Deguchi et al.,2018).In clinical trials,few investigations have been conducted to determine the aetiology of PKI-associated hyponatremia,the mechanism remains therefore unknown.Only rare cases of PKI-induced syndrome of inappropriate secretion of antidiuretic hormone(SIADH)(Largeau et al.,2019),and none with pazopanib,have been reported.PRES is a clinical and radiological entity where a bilateral white matter oedema,occurring predominantly in the posterior occipital and parietal lobes,is associated with several neurologic symptoms.Interestingly,a recent review suggests that SIADH could be a symptom of PRES(Largeau et al.,2019).To our knowledge,this is the first case published where pazopanib-induced PRES occurs contemporaneously with possible SIADH.
