Untersuchung neuer Membranen am in-vitro-Modell des Molecular Adsorbent Recirculating System (MARS) und der Multiple Pass Albumin Dialysis (MPAD)

Ziel der Arbeit ist die Testung von Albumindialysemembranen mit ansteigender Porengröße anhand eines Zwei-Kompartement-Modells und darauf basierend die Entwicklung einer neuen Entgiftungsmethode. Es gibt keine Unterschiede bezüglich der Entgiftung wasserlöslicher Toxine zwischen den Membranen. Hochalbuminaffine Toxine werden nur bei Membranen mit weiten Poren entfernt. Dies geht mit einem noch akteptablen Plasmaproteinverlust einher. Allerdings besteht ein inverser Zusammenhang zwischen der Entfernung schwach albuminaffiner Toxine zur Porengröße. Dies wird mit Oberflächendiffsion erklärt

Nerve ultrasound characterizes AMN polyneuropathy as inhomogeneous and focal hypertrophic

Abstract Objective High-resolution nerve ultrasound (HRUS) is a painless tool to quickly evaluate peripheral nerve morphology in vivo. This study set out to characterize peripheral nerve involvement in X-linked adrenomyeloneuropathy (AMN) by HRUS. Methods Thirteen adults with genetically proven AMN were examined using the Ultrasound pattern sum score (UPSS) to evaluate morphological abnormalities of peripheral nerves, vagal nerves, as well as cervical nerve roots. Ultrasound results were correlated with clinical findings and nerve conduction studies. Results UPSS was increased in six out of 13 patients. Nerve enlargement was mostly inhomogeneous and regional. The median, ulnar, and vagal nerves presented with more prominent alterations than nerves of the lower limbs. The proximal-to-distal ratio was significantly enlarged for the median nerve. HRUS findings matched nerve conduction studies, but identified one patient with enlarged nerves and yet normal conduction velocities. Sonographic findings did not correlate with disease duration or disease severity as assessed by the spastic paraplegia rating scale. Conclusion HRUS reveals significant multifocal regional nerve swellings with reduced echo intensity as the morphological equivalent of electrophysiological peripheral nerve affection in AMN patients. Ultrasound and NCS characteristics in AMN seem to differ from other demyelinating neuropathies like CIDP or CMT1a. Trial registration German clinical-trial-register (DRKS) (DRKS-ID 00005253) Registered 15 October 2013

Human Cerebrospinal fluid promotes long-term neuronal viability and network function in human neocortical organotypic brain slice cultures

Abstract Pathophysiological investigation of CNS-related diseases, such as epilepsy or neurodegenerative disorders, largely relies on histological studies on human post mortem tissue, tissue obtained by biopsy or resective surgery and on studies using disease models including animal models, heterologous expression systems or cell culture based approaches. However, in general it remains elusive to what extent results obtained in model systems can be directly translated to the human brain, calling for strategies allowing validation or even primary investigation in live human CNS tissue. In the work reported here, we prepared human organotypic slice cultures from access tissue of resective epilepsy surgery. Employing different culture conditions, we systematically compared artificial culturing media versus human cerbrospinal fluid (hCSF) obtained from patients with normal pressure hydrocephalus (NPH). Presented data demonstrates sustained cortical neuronal survival including not only maintenance of typical cellular electrophysiological properties and activity, such as robust action potential generation and synaptic connectivity, but also preservation of tonic and phasic network activity up to several weeks in vitro. As clearly delineated by immunocytochemistry, single cell patch clamp and extracellular recordings, we find that in contrast to artificial culturing media, hCSF significantly enhances neuron viability and maintenance of network activity