Mechanische Thromboseprophylaxe mittels A-V Impulssystem® in der Hüftalloarthroplastik: eine randomisierte, prospektive, klinische Studie

Inhalt: - Einsatz einer zusätzlichen mechanischen Thromboseprophylaxe während der Implantation zementfreier Hüfttotalendoprothesen (A-V Impulssystem® - ein mechanisches Pumpsystem, welches an der ipsilateralen Fußsohle intra- und weitere 24 h postoperativ zur Anwendung kommt.) - Diagnose der thromboembolischen Ereignisse mittels einer prä- und postoperativen Lungenperfusionsszintigraphie - insgesamt 94 Patienten im Alter bis 80 Jahre Ergebnisse: - Keine signifikante Senkung der thromboembolischen Inzidenz mittels zusätzlicher Anwendung des A-V-Impulssystems® (p= 0,308), positive Tendenz - Präoperativ festgestellte Perfusionsdefizite (43,6%) kamen postoperativ konstant zur Darstellung - Patienten mit positiven neuen embolietypischen Ausfällen im Lungenperfusionszintigramm fehlten jegliche klinische Symptome - Zusammenhang zwischen präoperativ erfaßten paraklinischen und klinischen Parametern und postoperativ neu aufgetretenen embolietypischen Ausfällen mittels Kontingenzkoeffizient C geprüft und ausgeschlosse

Umm Qays, Jordanien. Das Objekt im Blick. Untersuchung und ­experimenteller Nachbau. Bauwerkserhaltung im ›alten‹ Dorf von Umm Qays (Jordanien). Die Arbeiten des Jahres 2021

Integrated into a building conservation training program that has been ­running since 2016, a team of German craftsmen and Jordanian and Syrian workers restored a former guest and meeting room in the old village of Umm Qays in accordance with the requirements for monument preservation. The restoration measures were based on previous scientific research. Due to the increasing loss of craftmanship, many of the historical construction techniques had to be reacquired on site. It was only through the mutual knowledge gained from research and practice, supplemented by the ­exchange with the local population, that the measures could be successfully ­completed

Zwischen Beamtentum und Börse: Positionierungen der Telekom

Die Verfasserinnen analysieren die Deregulierung der Telekommunikation in der Bundesrepublik anhand von drei Beispielen: der Transformation des Beschäftigungsverhältnisses der Beamten, der Regulierung des de facto verbliebenen Fast-Monopols der "letzten Meile" der Leitungen in die Haushalte im Telefon-Festnetz und dem Börsengang der Telekom. Sie untersuchen damit die rechtlichen, technischen und wirtschaftlichen Dimensionen des Deregulierungsprozesses. Die genannten Beispiele machen deutlich, wie ein ehedem öffentliches Monopolunternehmen unter Bedingungen weit gehender Privatisierung und Re-Regulierung weder zum Spielball seiner Umwelt wird noch umfassend mit seiner Vergangenheit bricht. Das Unternehmen sucht die Bestände an Regelungen, Wissen, Kompetenz und Technologie des ehemaligen Monopolisten selektiv in Ressourcen zu konvertieren, die unter den spezifischen Marktbedingungen des Telekommunikationssektors seine Handlungsfähigkeit garantieren. Die Parallelführung von inszeniertem Strukturbruch und institutioneller Kontinuität sichert der Telekom Flexibilisierung und Strategiefähigkeit. (ICE2

Efficacy and safety of Privigen® in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label Phase III study (the PRIMA study)

This prospective, multicenter, single-arm, open-label Phase III study aimed to evaluate the efficacy and safety of Privigen (R) (10% liquid human intravenous immunoglobulin [IVIG], stabilized with l-proline) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients received one induction dose of Privigen (2g/kg body weight [bw]) and up to seven maintenance doses (1g/kg bw) at 3-week intervals. The primary efficacy endpoint was the responder rate at completion, defined as improvement of 1 point on the adjusted Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale. The preset success criterion was the responder rate being 35%. Of the 31 screened patients, 28 patients were enrolled including 13 (46.4%) IVIG-pretreated patients. The overall responder rate at completion was 60.7% (95% confidence interval [CI]: 42.41%-76.43%). IVIG-pretreated patients demonstrated a higher responder rate than IVIG-naive patients (76.9% vs. 46.7%). The median (25%-75% quantile) INCAT score improved from 3.5 (3.0-4.5) points at baseline to 2.5 (1.0-3.0) points at completion, as did the mean (standard deviation [SD]) maximum grip strength (66.7 [37.24] kPa vs. 80.9 [31.06] kPa) and the median Medical Research Council sum score (67.0 [61.5-72.0] points vs. 75.5 [71.5-79.5] points). Of 108 adverse events (AEs; 0.417 AEs per infusion), 95 AEs (88.0%) were mild or moderate in intensity and resolved by the end of study. Two serious AEs of hemolysis were reported that resolved after discontinuation of treatment. Thus, Privigen provided efficacious and well-tolerated induction and maintenance treatment in patients with CIDP

SH3TC2, a protein mutant in Charcot-Marie-Tooth neuropathy, links peripheral nerve myelination to endosomal recycling

Patients with Charcot-Marie-Tooth neuropathy and gene targeting in mice revealed an essential role for the SH3TC2 gene in peripheral nerve myelination. SH3TC2 expression is restricted to Schwann cells in the peripheral nervous system, and the gene product, SH3TC2, localizes to the perinuclear recycling compartment. Here, we show that SH3TC2 interacts with the small guanosine triphosphatase Rab11, which is known to regulate the recycling of internalized membranes and receptors back to the cell surface. Results of protein binding studies and transferrin receptor trafficking are in line with a role of SH3TC2 as a Rab11 effector molecule. Consistent with a function of Rab11 in Schwann cell myelination, SH3TC2 mutations that cause neuropathy disrupt the SH3TC2/Rab11 interaction, and forced expression of dominant negative Rab11 strongly impairs myelin formation in vitro. Our data indicate that the SH3TC2/Rab11 interaction is relevant for peripheral nerve pathophysiology and place endosomal recycling on the list of cellular mechanisms involved in Schwann cell myelinatio

Endocrine Active UV Filters: Developmental Toxicity and Exposure Through Breast Milk

Several UV filters exhibit endocrine activity. Evidence for transdermal passage and presence in the food chain (fish) suggests potential exposure of humans during development. Developmental toxicity was studied in rats for the estrogenic UV filters 4-methylbenzylidene camphor (4-MBC, 0.7, 7, 24, 47 mg/kg/day) and 3-benzylidene camphor (3-BC, 0.07, 0.24, 0.7, 2.4, 7 mg/kg/day) administered in chow to the parent generation before mating, during pregnancy and lactation, and to the offspring until adulthood. Neonates exhibited enhanced prostate growth after 4-MBC and altered uterine gene expression after both filters. 4-MBC and 3-BC delayed male puberty and affected reproductive organ weights of adult offspring. Interactions with the thyroid were noted. Expression and estrogen sensitivity of target genes and nuclear receptor coregulators were altered at mRNA and protein levels in adult uterus, prostate and brain. Female sexual behavior was affected by 4-MBC and 3-BC, estrous cycles by 3-BC. Classical endpoints exhibited LOAELs/NOAELs of 7/0.7 mg/kg/day for 4-MBC and 0.24/0.07 mg/kg/day for 3-BC. Molecular endpoints were affected by the lowest doses. In order to obtain information on human exposure, we conducted a monitoring study on human milk with three series of mother–child pairs (2004, 2005, 2006), with focus on cosmetic UV filters in relation to other endocrine disrupters. Methods for UV filter analysis followed the principles of European standardized methods for pesticide residue analysis (EN 15289). In cohorts 2004 and 2005, 78.8% of women reported use of product(s) containing cosmetic UV filters in a questionnaire, and 76.5% of milk samples contained these filters. Use of UV filters and concentration in human milk were significantly correlated. The results agree with the idea of transdermal passage of UV filters. They also indicate that it may be possible to reduce human exposure during critical periods such as pregnancy and lactation by transiently abstaining from use

PLEKHG5 deficiency leads to an intermediate form of autosomal-recessive Charcot-Marie-Tooth disease

Charcot-Marie-Tooth disease (CMT) comprises a clinically and genetically heterogeneous group of peripheral neuropathies characterized by progressive distal muscle weakness and atrophy, foot deformities and distal sensory loss. Following the analysis of two consanguineous families affected by a medium to late-onset recessive form of intermediate CMT, we identified overlapping regions of homozygosity on chromosome 1p36 with a combined maximum LOD score of 5.4. Molecular investigation of the genes from this region allowed identification of two homozygous mutations in PLEKHG5 that produce premature stop codons and are predicted to result in functional null alleles. Analysis of Plekhg5 in the mouse revealed that this gene is expressed in neurons and glial cells of the peripheral nervous system, and that knockout mice display reduced nerve conduction velocities that are comparable with those of affected individuals from both families. Interestingly, a homozygous PLEKHG5 missense mutation was previously reported in a recessive form of severe childhood onset lower motor neuron disease (LMND) leading to loss of the ability to walk and need for respiratory assistance. Together, these observations indicate that different mutations in PLEKHG5 lead to clinically diverse outcomes (intermediate CMT or LMND) affecting the function of neurons and glial cell

Heterozygous Loss-of-Function Mutations in DLL4 Cause Adams-Oliver Syndrome.

Adams-Oliver syndrome (AOS) is a rare developmental disorder characterized by the presence of aplasia cutis congenita (ACC) of the scalp vertex and terminal limb-reduction defects. Cardiovascular anomalies are also frequently observed. Mutations in five genes have been identified as a cause for AOS prior to this report. Mutations in EOGT and DOCK6 cause autosomal-recessive AOS, whereas mutations in ARHGAP31, RBPJ, and NOTCH1 lead to autosomal-dominant AOS. Because RBPJ, NOTCH1, and EOGT are involved in NOTCH signaling, we hypothesized that mutations in other genes involved in this pathway might also be implicated in AOS pathogenesis. Using a candidate-gene-based approach, we prioritized DLL4, a critical NOTCH ligand, due to its essential role in vascular development in the context of cardiovascular features in AOS-affected individuals. Targeted resequencing of the DLL4 gene with a custom enrichment panel in 89 independent families resulted in the identification of seven mutations. A defect in DLL4 was also detected in two families via whole-exome or genome sequencing. In total, nine heterozygous mutations in DLL4 were identified, including two nonsense and seven missense variants, the latter encompassing four mutations that replace or create cysteine residues, which are most likely critical for maintaining structural integrity of the protein. Affected individuals with DLL4 mutations present with variable clinical expression with no emerging genotype-phenotype correlations. Our findings demonstrate that DLL4 mutations are an additional cause of autosomal-dominant AOS or isolated ACC and provide further evidence for a key role of NOTCH signaling in the etiology of this disorder

Ein Gedankenaustausch

Was bedeutet der mit der wachsenden Forderung nach „open science“ verbundene Druck, Forschungsdaten öffentlich zugänglich zu machen für die ethnographische Forschung? Welche Herausforderungen, Probleme und Chancen sind mit der Aufbereitung und Archivierung von ethnologischen Forschungsdaten in Repositorien, insbesondere für eventuelle spätere Nachnutzungen verbunden? Welche Grenzverschiebungen im Verhältnis von Öffentlichkeit und Privatheit gehen mit der Verfügbarmachung von ethnographischen Daten einher und welche Materialien sind überhaupt dafür geeignet? Wieviel Aufwand bedeutet die Aufbereitung von Forschungsmaterialien und welche Öffentlichkeiten sollen überhaupt adressiert werden? Diese und weitere Aspekte wurden im Dezember 2021 von insgesamt neun an unterschiedlichen Universitäten und Forschungseinrichtungen tätigen Sozial- und Kulturanthropolog*innen in einer Online Gesprächsrunde diskutiert. Bei dem hier vorliegenden Text handelt es sich um die sprachlich leicht redigierte, aber ansonsten unveränderte und von den Gesprächspartner*innen freigegebene Fassung dieses Gedankenaustauschs, mit dem die Gruppe zum Nachdenken über diese zentrale Thema anregen möchte.01/2

Ein Gedankenaustausch

Was bedeutet der mit der wachsenden Forderung nach „open science“ verbundene Druck, Forschungsdaten öffentlich zugänglich zu machen für die ethnographische Forschung? Welche Herausforderungen, Probleme und Chancen sind mit der Aufbereitung und Archivierung von ethnologischen Forschungsdaten in Repositorien, insbesondere für eventuelle spätere Nachnutzungen verbunden? Welche Grenzverschiebungen im Verhältnis von Öffentlichkeit und Privatheit gehen mit der Verfügbarmachung von ethnographischen Daten einher und welche Materialien sind überhaupt dafür geeignet? Wieviel Aufwand bedeutet die Aufbereitung von Forschungsmaterialien und welche Öffentlichkeiten sollen überhaupt adressiert werden? Diese und weitere Aspekte wurden im Dezember 2021 von insgesamt neun an unterschiedlichen Universitäten und Forschungseinrichtungen tätigen Sozial- und Kulturanthropolog*innen in einer Online Gesprächsrunde diskutiert. Bei dem hier vorliegenden Text handelt es sich um die sprachlich leicht redigierte, aber ansonsten unveränderte und von den Gesprächspartner*innen freigegebene Fassung dieses Gedankenaustauschs, mit dem die Gruppe zum Nachdenken über diese zentrale Thema anregen möchte.01/2