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  • 1
    In: Orphanet Journal of Rare Diseases, Springer Science and Business Media LLC, Vol. 17, No. 1 ( 2022-10-23)
    Abstract: The development and approval of disease modifying treatments have dramatically changed disease progression in patients with spinal muscular atrophy (SMA). Nusinersen was approved in Europe in 2017 for the treatment of SMA patients irrespective of age and disease severity. Most data on therapeutic efficacy are available for the infantile-onset SMA. For patients with SMA type 2 and type 3, there is still a lack of sufficient evidence and long-term experience for nusinersen treatment. Here, we report data from the SMArtCARE registry of non-ambulant children with SMA type 2 and typen 3 under nusinersen treatment with a follow-up period of up to 38 months. Methods SMArtCARE is a disease-specific registry with data on patients with SMA irrespective of age, treatment regime or disease severity. Data are collected during routine patient visits as real-world outcome data. This analysis included all non-ambulant patients with SMA type 2 or 3 below 18 years of age before initiation of treatment. Primary outcomes were changes in motor function evaluated with the Hammersmith Functional Motor Scale Expanded (HFMSE) and the Revised Upper Limb Module (RULM). Results Data from 256 non-ambulant, pediatric patients with SMA were included in the data analysis. Improvements in motor function were more prominent in upper limb: 32.4% of patients experienced clinically meaningful improvements in RULM and 24.6% in HFMSE. 8.6% of patients gained a new motor milestone, whereas no motor milestones were lost. Only 4.3% of patients showed a clinically meaningful worsening in HFMSE and 1.2% in RULM score. Conclusion Our results demonstrate clinically meaningful improvements or stabilization of disease progression in non-ambulant, pediatric patients with SMA under nusinersen treatment. Changes were most evident in upper limb function and were observed continuously over the follow-up period. Our data confirm clinical trial data, while providing longer follow-up, an increased number of treated patients, and a wider range of age and disease severity.
    Type of Medium: Online Resource
    ISSN: 1750-1172
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 2225857-7
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  • 2
    In: Swiss Medical Weekly, SMW Supporting Association
    Type of Medium: Online Resource
    ISSN: 1424-3997
    Language: English
    Publisher: SMW Supporting Association
    Publication Date: 2020
    detail.hit.zdb_id: 2031164-3
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  • 3
    Online Resource
    Online Resource
    American Association for the Advancement of Science (AAAS) ; 2021
    In:  Science Vol. 371, No. 6527 ( 2021-01-22), p. 375-379
    In: Science, American Association for the Advancement of Science (AAAS), Vol. 371, No. 6527 ( 2021-01-22), p. 375-379
    Abstract: Understanding how the local environment of a “single-atom” catalyst affects stability and reactivity remains a challenge. We present an in-depth study of copper 1 , silver 1 , gold 1 , nickel 1 , palladium 1 , platinum 1 , rhodium 1 , and iridium 1 species on Fe 3 O 4 (001), a model support in which all metals occupy the same twofold-coordinated adsorption site upon deposition at room temperature. Surface science techniques revealed that CO adsorption strength at single metal sites differs from the respective metal surfaces and supported clusters. Charge transfer into the support modifies the d-states of the metal atom and the strength of the metal–CO bond. These effects could strengthen the bond (as for Ag 1 –CO) or weaken it (as for Ni 1 –CO), but CO-induced structural distortions reduce adsorption energies from those expected on the basis of electronic structure alone. The extent of the relaxations depends on the local geometry and could be predicted by analogy to coordination chemistry.
    Type of Medium: Online Resource
    ISSN: 0036-8075 , 1095-9203
    RVK:
    RVK:
    Language: English
    Publisher: American Association for the Advancement of Science (AAAS)
    Publication Date: 2021
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    detail.hit.zdb_id: 2066996-3
    detail.hit.zdb_id: 2060783-0
    SSG: 11
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  • 4
    Online Resource
    Online Resource
    Oxford University Press (OUP) ; 2021
    In:  Nephrology Dialysis Transplantation Vol. 36, No. Supplement_1 ( 2021-05-29)
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 36, No. Supplement_1 ( 2021-05-29)
    Abstract: Acute kidney injury (AKI) is common and associated with increased mortality and morbidity. The impact of AKI on subsequent heart failure remains largely unknown. Method The Basics in Acute Shortness of Breath Evaluation Study (BaselV) prospectively enrolled patients presenting the emergency department with acute dyspnea. Two independent specialists adjudicated the final cause of dyspnea. Serum creatinine concentrations were prospectively assessed throughout the hospitalization. AKI was defined according to the serum creatinine criteria of the 2012 KDIGO clinical practice guideline. AKI adjudication occurred blinded to the cause of dyspnea. Mortality and rehospitalizations were prospectively assessed during follow-up (median:768 days [IQR:290-950]. Renal recovery was defined as a discharge creatinine & lt;1.25x baseline creatinine. Results AKI occurred in 809 (40%) of 2021 patients and was associated with increased all-cause (adjusted Hazard Ratio [aHR] 1.33, 95%CI 1.13-1.55; p & lt;0.01) and cardiovascular mortality (aHR 1.43, 95%CI 1.16-1.75; p & lt;0.01). However, the impact of AKI on mortality was time-dependent with the highest impact on early 30-day mortality (aHR 2.47, 95%CI 1.62-3.76; p & lt;0.01) (Figure 1A). AKI was not associated with all-cause or cardiovascular mortality in patients achieving renal recovery (p=0.10 and p=0.30). In contrast, AKI displayed a time-independent association with subsequent hospitalisations for heart failure (hHF) (aHR 1.49, 95%CI 1.15-1.94; p & lt;0.01) (Figure 1B). The association with hHF was stronger for higher degrees of AKI (stage 2/3 aHR: 1.89; 95%CI 1.33-2.83; p & lt;0.01). This association with hHF persisted in patients with non-cardiac dyspnea (aHR 2.43, 95%CI 1.05-5.59; p=0.04), even after renal recovery (aHR 2.56, 95%CI 1.00-6.54; p=0.05). Again, in patients with non-cardiac dyspnea the association of advanced AKI (stage 2/3) with hHF was even stronger (aHR 4.25, 95%CI 1.59-11.36; p & lt;0.01). Conclusion AKI independently increases the risk of hHF by almost 50%. This association persists in patients with non-cardiac dyspnea, even after renal recovery by discharge. This suggests AKI to be a novel risk-factor for the development of clinically significant HF.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1465709-0
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  • 5
    In: Circulation: Heart Failure, Ovid Technologies (Wolters Kluwer Health), Vol. 15, No. 6 ( 2022-06)
    Abstract: Current guidelines recommend interpreting concentrations of NPs (natriuretic peptides) irrespective of the time of presentation to the emergency department. We hypothesized that diurnal variations in NP concentration may affect their diagnostic accuracy for acute heart failure. Methods: In a secondary analysis of a multicenter diagnostic study enrolling patients presenting with acute dyspnea to the emergency department and using central adjudication of the final diagnosis by 2 independent cardiologists, the diagnostic accuracy for acute heart failure of BNP (B-type NP), NT-proBNP (N-terminal pro-B-type NP), and MR-proANP (midregional pro-atrial NP) was compared among 1577 daytime presenters versus 908 evening/nighttime presenters. In a validation study, the presence of a diurnal rhythm in BNP and NT-proBNP concentrations was examined by hourly measurements in 44 stable individuals. Results: Among patients adjudicated to have acute heart failure, BNP, NT-proBNP, and MR-proANP concentrations were comparable among daytime versus evening/nighttime presenters (all P =nonsignificant). Contrastingly, among patients adjudicated to have other causes of dyspnea, evening/nighttime presenters had lower BNP (median, 44 [18–110] versus 74 [27–168] ng/L; P 〈 0.01) and NT-proBNP (median, 212 [72–581] versus 297 [102–902] ng/L; P 〈 0.01) concentrations versus daytime presenters. This resulted in higher diagnostic accuracy as quantified by the area under the curve of BNP and NT-proBNP among evening/nighttime presenters (0.97 [95% CI, 0.95–0.98] and 0.95 [95% CI, 0.93–0.96] versus 0.94 [95% CI, 0.92–0.95] and 0.91 [95% CI, 0.90–0.93] ) among daytime presenters (both P 〈 0.01). These differences were not observed for MR-proANP. Diurnal variation of BNP and NT-proBNP with lower evening/nighttime concentration was confirmed in 44 stable individuals ( P 〈 0.01). Conclusions: BNP and NT-proBNP, but not MR-proANP, exhibit a diurnal rhythm that results in even higher diagnostic accuracy among evening/nighttime presenters versus daytime presenters. Registration: URL: https://www.clinicaltrials.gov ; Unique identifiers: NCT01831115, NCT02091427, and NCT02210897.
    Type of Medium: Online Resource
    ISSN: 1941-3289 , 1941-3297
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2022
    detail.hit.zdb_id: 2428100-1
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  • 6
    In: Journal of Clinical Medicine, MDPI AG, Vol. 10, No. 11 ( 2021-05-25), p. 2288-
    Abstract: Previous studies have indicated an association between coronavirus disease 2019 (COVID-19) and acute kidney injury (AKI) but lacked a control group. The prospective observational COronaVIrus-surviVAl (COVIVA) study performed at the University Hospital, Basel, Switzerland consecutively enrolled patients with symptoms suggestive of COVID-19. We compared patients who tested positive for SARS-CoV-2 with patients who tested negative but with an adjudicated diagnosis of a respiratory tract infection, including pneumonia. The primary outcome measure was death at 30 days, and the secondary outcomes were AKI incidence and a composite endpoint of death, intensive care treatment or rehospitalization at 30 days. Five hundred and seven patients were diagnosed with respiratory tract infections, and of those, 183 (36%) had a positive PCR swab test for SARS-CoV-2. The incidence of AKI was higher in patients with COVID-19 (30% versus 12%, p 〈 0.001), more severe (KDIGO stage 3, 22% versus 13%, p = 0.009) and more often required renal replacement therapy (4.4% versus 0.93%; p = 0.03). The risk of 30-day mortality and a composite endpoint was higher in patients with COVID-19-associated AKI (adjusted hazard ratio (aHR) mortality 3.98, 95% confidence interval (CI) 1.10–14.46, p = 0.036; composite endpoint aHR 1.84, 95% CI 1.02–3.31, p = 0.042). The mortality risk was attenuated when adjusting for disease severity (aHR 3.60, 95% CI 0.93–13.96, p = 0.062). AKI occurs more frequently and with a higher severity in patients with COVID-19 and is associated with worse outcomes.
    Type of Medium: Online Resource
    ISSN: 2077-0383
    Language: English
    Publisher: MDPI AG
    Publication Date: 2021
    detail.hit.zdb_id: 2662592-1
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  • 7
    In: ESC Heart Failure, Wiley, Vol. 7, No. 5 ( 2020-10), p. 3219-3224
    Abstract: Acute kidney injury (AKI) during acute heart failure (AHF) is common and associated with increased morbidity and mortality. The underlying pathophysiological mechanism appears to have prognostic relevance; however, the differentiation of true, structural AKI from hemodynamic pseudo‐AKI remains a clinical challenge. Methods and results The Basics in Acute Shortness of Breath Evaluation Study (NCT01831115) prospectively enrolled adult patients presenting with AHF to the emergency department. Mortality of patients was prospectively assessed. Haemoconcentration, transglomerular pressure gradient ( n  = 231) and tubular injury patterns ( n  = 253) were evaluated to investigate pathophysiological mechanisms underlying AKI timing (existing at presentation vs. developing during in‐hospital period). Of 1643 AHF patients, 755 patients (46%) experienced an episode of AKI; 310 patients (19%; 41% of AKI patients) presented with community‐acquired AKI (CA‐AKI), 445 patients (27%; 59% of AKI patients) developed in‐hospital AKI. CA‐AKI but not in‐hospital AKI was associated with higher mortality compared with no‐AKI (adjusted hazard ratio 1.32 [95%‐CI 1.01–1.74]; P  = 0.04). Independent of AKI timing, haemoconcentration was associated with a lower two‐year mortality. Transglomerular pressure gradient at presentation was significantly lower in CA‐AKI compared to in‐hospital AKI and no‐AKI ( P  〈  0.01). Urinary NGAL ratio concentrations were significantly higher in CA‐AKI compared to in‐hospital AKI ( P  〈  0.01) or no‐AKI ( P  〈  0.01). Conclusions CA‐AKI but not in‐hospital AKI is associated with increased long‐term mortality and marked by decreased transglomerular pressure gradient and tubular injury, probably reflecting prolonged tubular ischemia due to reno‐venous congestion. Adequate decongestion, as assessed by haemoconcentration, is associated with lower long‐term mortality independent of AKI timing.
    Type of Medium: Online Resource
    ISSN: 2055-5822 , 2055-5822
    URL: Issue
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2814355-3
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  • 8
    Online Resource
    Online Resource
    SMW Supporting Association ; 2021
    In:  Swiss Medical Weekly Vol. 151, No. 0910 ( 2021-03-01), p. w20482-
    In: Swiss Medical Weekly, SMW Supporting Association, Vol. 151, No. 0910 ( 2021-03-01), p. w20482-
    Abstract: BACKGROUND Data about patients in Europe with corona virus disease-2019 (COVID-19) and acute kidney injury (AKI) are scarce. We examined characteristics, presentation and risk factors of AKI in patients hospitalised with COVID-19 in a tertiary hospital in Switzerland. METHODS We reviewed health records of patients hospitalised with a positive nasopharyngeal polymerase chain reaction test for SARS-CoV2 between 1 February and 30 June 2020, at the University Hospital of Basel. The nadir creatinine of the hospitalisation was used as baseline. AKI was defined according the KDIGO guidelines as a 1.5× increase of baseline creatinine and in-hospital renal recovery as a discharge creatinine 〈 1.25× baseline creatinine. Least absolute shrinkage and selection operator (LASSO) regression was performed to select predictive variables of AKI. Based on this a final model was chosen. RESULTS Of 188 patients with COVID-19, 41 (22%) developed AKI, and 11 (6%) required renal replacement therapy. AKI developed after a median of 9 days (interquartile range [IQR] 5-12) after the first symptoms and a median of 1 day (IQR 0-5) after hospital admission. The peak AKI stages were stage 1 in 39%, stage 2 in 24% and stage 3 in 37%. A total of 29 (15%) patients were admitted to the intensive care unit and of these 23 (79%) developed AKI. In-hospital renal recovery at discharge was observed in 61% of all AKI episodes. In-hospital mortality was 27% in patients with AKI and 10% in patients without AKI. Age (adjusted odds ratio [aOR] 1.04, 95% confidence interval [CI] 1.01–1.08; p = 0.024), history of chronic kidney disease (aOR 3.47, 95% CI 1.16–10.49;p = 0.026), C-reactive protein levels (aOR 1.09, 95% CI 1.03–1.06; p = 0.002) and creatinine kinase (aOR 1.03, 95% CI 1.01–1.06; p = 0.002) were associated with development of AKI. CONCLUSIONS AKI is common in hospitalised patients with COVID-19 and more often seen in patients with severe COVID-19 illness. AKI is associated with a high in-hospital mortality.
    Type of Medium: Online Resource
    ISSN: 1424-3997
    Language: Unknown
    Publisher: SMW Supporting Association
    Publication Date: 2021
    detail.hit.zdb_id: 2031164-3
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  • 9
    In: Swiss Medical Weekly, SMW Supporting Association, Vol. 151, No. 2930 ( 2021-07-29), p. w20572-
    Abstract: AIMS The aim of this study was to analyse the demographics, risk factors and in-hospital mortality rates of patients admitted with coronavirus disease 2019 (COVID-19) to a tertiary care hospital in Switzerland. METHODS In this single-centre retrospective cohort study at the University Hospital Basel, we included all patients with confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection hospitalised from 27 February 2020 to 10 May 2021. Patients’ characteristics were extracted from the electronic medical record system. The primary outcome of this study was temporal trends of COVID-19-related in-hospital mortality. Secondary outcomes were COVID-19-related mortality in patients hospitalised on the intensive care unit (ICU), admission to ICU, renal replacement therapy and length of hospital stay, as well as a descriptive analysis of risk factors for in-hospital mortality. RESULTS During the study period we included 943 hospitalisations of 930 patients. The median age was 65 years (interquartile range [IQR] 53–76) and 63% were men. The numbers of elderly patients, patients with multiple comorbidities and need for renal replacement therapy decreased from the first and second to the third wave. The median length of stay and need for ICU admission were similar in all waves. Throughout the study period 88 patients (9.3%) died during the hospital stay. Crude in-hospital mortality was similar over the course of the first two waves (9.5% and 10.2%, respectively), whereas it decreased in the third wave (5.4%). Overall mortality in patients without comorbidities was low at 1.6%, but it increased in patients with any comorbidity to 12.6%. Predictors of all-cause mortality over the whole period were age (adjusted odds ratio [aOR] per 10-year increase 1.81, 95% confidence interval [CI] 1.45–2.26; p 〈 0.001), male sex (aOR 1.68, 95% CI 1.00–2.82; p = 0.048), immunocompromising condition (aOR 2.09, 95% CI 1.01–4.33; p = 0.048) and chronic kidney disease (aOR 2.25, 95% CI 1.35–3.76; p = 0.002). CONCLUSION In our study in-hospital mortality was 9.5%, 10.2% and 5.4% in the first, second and third waves, respectively. Age, immunocompromising condition, male sex and chronic kidney disease were factors associated with in-hospital mortality. Importantly, patients without any comorbidity had a very low in-hospital mortality regardless of age.
    Type of Medium: Online Resource
    ISSN: 1424-3997
    Language: Unknown
    Publisher: SMW Supporting Association
    Publication Date: 2021
    detail.hit.zdb_id: 2031164-3
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  • 10
    In: Advanced Materials Interfaces, Wiley, Vol. 8, No. 8 ( 2021-04)
    Abstract: Iron oxides (FeO x ) are among the most common support materials utilized in single atom catalysis. The support is nominally Fe 2 O 3 , but strongly reductive treatments are usually applied to activate the as‐synthesized catalyst prior to use. Here, Rh adsorption and incorporation on the () surface of hematite (α‐Fe 2 O 3 ) are studied, which switches from a stoichiometric (1  ×  1) termination to a reduced (2  ×  1) reconstruction in reducing conditions. Rh atoms form clusters at room temperature on both surface terminations, but Rh atoms incorporate into the support lattice as isolated atoms upon annealing above 400 °C. Under mildly oxidizing conditions, the incorporation process is so strongly favored that even large Rh clusters containing hundreds of atoms dissolve into the surface. Based on a combination of low‐energy ion scattering (LEIS), X‐ray photoelectron spectroscopy (XPS) and scanning tunneling microscopy (STM) data, as well as density functional theory (DFT), it is concluded that the Rh atoms are stabilized in the immediate subsurface, rather than the surface layer.
    Type of Medium: Online Resource
    ISSN: 2196-7350 , 2196-7350
    URL: Issue
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2750376-8
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