Monthly video consultation for children and adolescents with type 1 diabetes mellitus during the COVID-19 pandemic.

Aims: To explore the impact of primarily telemedical care for children and adolescents with type 1 diabetes by monthly video consultations on metabolic control and parents' treatment satisfaction and disease-specific burden during the COVID-19 pandemic.

Methods: In this 12-month multicenter observational follow-up VIDIKI 2.0 study, 100 participants (3-18 years) received monthly video consultations, which partially replaced quarterly outpatient clinic appointments during the pandemic.

Unveiling a key catalytic pocket for the ruthenium NHC-catalysed asymmetric heteroarene hydrogenation.

The chiral ruthenium(ii)bis-SINpEt complex is a versatile and powerful catalyst for the hydrogenation of a broad range of heteroarenes. This study aims to provide understanding of the active form of this privileged catalyst as well as the reaction mechanism, and to identify the factors which control enantioselectivity. To this end we used computational methods and NMR spectroscopy to study the hydrogenation of 2-methylbenzofuran promoted by this system.

Pubertal development in 46,XY patients with NR5A1 mutations.

Purpose: Mutations in the NR5A1 gene, encoding the transcription factor Steroidogenic Factor-1, are associated with a highly variable genital phenotype in patients with 46,XY differences of sex development (DSD). Our objective was to analyse the pubertal development in 46,XY patients with NR5A1 mutations by the evaluation of longitudinal clinical and hormonal data at pubertal age.

Methods: We retrospectively studied a cohort of 10 46,XY patients with a verified NR5A1 mutation and describe clinical features including the external and internal genitalia, testicular volumes, Tanner stages and serum concentrations of LH, FSH, testosterone, AMH, and inhibin B during pubertal transition.

An Integrated Evanescent Field Sensor for the Simultaneous Measurement of Layer Refractive Index and Thickness.

A novel integrated sensor for the simultaneous measurement of layer refractive index and thickness based on evanescent fields is proposed. The theoretical limits for the accuracy of the sensor were examined for the example of a TiO2 layer. The influence of production tolerance on the accuracy was evaluated.

Complex Evolution of Light-Dependent Protochlorophyllide Oxidoreductases in Aerobic Anoxygenic Phototrophs: Origin, Phylogeny, and Function.

Light-dependent protochlorophyllide oxidoreductase (LPOR) and dark-operative protochlorophyllide oxidoreductase are evolutionary and structurally distinct enzymes that are essential for the synthesis of (bacterio)chlorophyll, the primary pigment needed for both anoxygenic and oxygenic photosynthesis. In contrast to the long-held hypothesis that LPORs are only present in oxygenic phototrophs, we recently identified a functional LPOR in the aerobic anoxygenic phototrophic bacterium (AAPB) Dinoroseobacter shibae and attributed its presence to a single horizontal gene transfer event from cyanobacteria. Here, we provide evidence for the more widespread presence of genuine LPOR enzymes in AAPBs.

Mechanistic Understanding of the Heterogeneous, Rhodium-Cyclic (Alkyl)(Amino)Carbene-Catalyzed (Fluoro-)Arene Hydrogenation.

Recently, chemoselective methods for the hydrogenation of fluorinated, silylated, and borylated arenes have been developed providing direct access to previously unattainable, valuable products. Herein, a comprehensive study on the employed rhodium-cyclic (alkyl)(amino)carbene (CAAC) catalyst precursor is disclosed. Mechanistic experiments, kinetic studies, and surface-spectroscopic methods revealed supported rhodium(0) nanoparticles (NP) as the active catalytic species.

Consensus model of a cyanobacterial light-dependent protochlorophyllide oxidoreductase in its pigment-free apo-form and photoactive ternary complex.

Photosynthetic organisms employ two different enzymes for the reduction of the C17 = C18 double bond of protochlorophyllide (Pchlide), yielding the chlorophyll precursor chlorophyllide. First, a nitrogenase-like, light-independent (dark-operative) Pchlide oxidoreductase and secondly, a light-dependent Pchlide oxidoreductase (LPOR). For the latter enzyme, despite decades of research, no structural information is available.

Ternary Complex Formation and Photoactivation of a Photoenzyme Results in Altered Protein Dynamics.

The interplay between protein dynamics and catalysis remains a fundamental question in enzymology. We here investigate the ns-timescale dynamics of a light-dependent NADPH:protochlorophyllide oxidoreductase (LPOR), a photoenzyme crucial for chlorophyll synthesis. LPORs catalyze the light-triggered trans addition of a hydride and a proton across the C17═C18 double bond of the chlorophyll precursor protochlorophyllide (Pchlide).

Simple ruthenium-catalyzed reductive amination enables the synthesis of a broad range of primary amines.

The production of primary benzylic and aliphatic amines, which represent essential feedstocks and key intermediates for valuable chemicals, life science molecules and materials, is of central importance. Here, we report the synthesis of this class of amines starting from carbonyl compounds and ammonia by Ru-catalyzed reductive amination using H. Key to success for this synthesis is the use of a simple RuCl(PPh) catalyst that empowers the synthesis of >90 various linear and branched benzylic, heterocyclic, and aliphatic amines under industrially viable and scalable conditions.

Low-Temperature Hydrogenation of Carbon Dioxide to Methanol with a Homogeneous Cobalt Catalyst.

Herein we describe the first homogeneous non-noble metal catalyst for the hydrogenation of CO to methanol. The catalyst is formed in situ from [Co(acac) ], Triphos, and HNTf and enables the reaction to be performed at 100 °C without a decrease in activity. Kinetic studies suggest an inner-sphere mechanism, and in situ NMR and MS experiments reveal the formation of the active catalyst through slow removal of the acetylacetonate ligands.

The impact of pericytes on the blood-brain barrier integrity depends critically on the pericyte differentiation stage.

The blood-brain barrier consists of the cerebral microvascular endothelium, pericytes, astrocytes and neurons. In this study we analyzed the differentiation stage dependent influence of primary porcine brain capillary pericytes on the barrier integrity of primary porcine brain capillary endothelial cells. At first, we were able to induce two distinct differentiation stages of the primary pericytes in vitro.

Extracorporeal fibrinogen adsorption--efficacy, selectivity and safety in healthy subjects and patients with foot ulcers.

The elimination of fibrinogen from plasma improves plasma viscosity and whole blood viscosity. For extracorporeal adsorption of fibrinogen the pentapeptide gly-pro-arg-pro-lys was coupled to sepharose CL-4B. Columns containing 100 ml of coupled sepharose CL-4B were used to eliminate fibrinogen from the plasma of 8 healthy male subjects (mean age 27.

The outcome in myasthenia gravis patients--an eight-year follow-up after finishing immunoabsorption therapy.

Eight years ago four patients suffering from myasthenia gravis (MG) type C and E according to Compston with failed drug therapy were treated three times (one patient 11 times) by protein A immunoabsorption (Immunosorba, Excorim, Fresenius Hemocare GmbH, StWendel, Germany). No further immunoabsorption treatments have been carried out. In addition, three patients were given a thymectomy.

Two cases of refractory endocrine ophthalmopathy successfully treated with extracorporeal immunoadsorption.

Endocrine ophthalmopathy (EO) is a severe disease entity that is characterized by retrobulbar swelling due to accumulation of glycosaminoglycans on an autoimmune basis. This disorder can lead to the loss of vision and often is resistant to conventional therapy. There is a relation to Graves' hyperthyroidism, but probably no close association.

Three cases of C-ANCA-positive vasculitis treated with immunoadsorption: possible benefit in early treatment.

Wegener's granulomatosis is a vasculitic disease predominantly affecting the upper respiratory tract, lungs, and kidneys. Three patients with Wegener's granulomatosis and rapidly progressive glomerulonephritis were treated with an intensified regimen of immunoadsorption (IA) (Excorim or Therasorb) in addition to cyclophosphamide (CYC) and methylprednisolone (PRE). Patient A had been in remission under oral CYC/PRE.

The fine specificity of the myelin oligodendrocyte glycoprotein autoantibody response in patients with multiple sclerosis and normal healthy controls.

Antibodies directed against the extracellular immunoglobulin (Ig)-like domain of the myelin oligodendrocyte glycoprotein (MOG(Igd)) mediate demyelination in experimental autoimmune encephalomyelitis (EAE) and are implicated in the immunopathogenesis of multiple sclerosis (MS). In this study we investigated the epitope specificity of MOG(Igd)-specific autoantibodies immunopurified from MS patients (n=17) and normal healthy controls (HD; n=9). ELISA, using a panel of synthetic MOG(Igd) peptides, revealed that the epitope specificity of this response was heterogeneous in both groups.

Surface treated catheters with ion beam based process for blood access.

Infection, thrombosis, and stenosis are among the most frequent complications associated with blood contacting catheters. Because these problems are usually related to surface properties of the base catheter material, surface treatment processes such as ion implantation and ion beam assisted deposition (IBAD) (silver based coatings) can be used to mitigate such complications. Because these ion beam based processes affect only the near-surface region (approximately the outer 1 microm), there is little effect on bulk material properties.

Removal of immunoglobulins by a protein A versus an antihuman immunoglobulin G-based system: evaluation of 602 sessions of extracorporeal immunoadsorption.

Elimination of IgG can be achieved by extracorporeal immunoadsorption (IA) based on specific binding to either staphylococcal protein A (Excorim) or sheep polyclonal antibodies directed against human IgG (Therasorb). In 602 analyzed sessions of IA, elimination of IgG was 60% through 80% depending on the treated plasma volume, with no significant difference between the mentioned systems. However, the decrease of IgM and IgA was approximately 50% in the anti-IgG compared to 20-40% in the protein A system.

Therapeutic apheresis in myasthenia gravis patients: a six year follow-up.

Six years ago 4 patients suffering from myasthenia gravis (MG) types C and E according to Compston with failed drug therapy were initially treated 3 times (1 patient, a total of 11 times) by protein A immunoadsorption (Immunosorba, Excorim AB, Lund, Sweden). No further immunoadsorption treatments have been carried out. In addition, 3 patients were given a thymectomy.

LDL-apheresis as long-term treatment in severe hyperlipidemia using differing methods.

In 34 patients (18 women, 16 men) suffering from familial hypercholesterolemia resistant to diet and lipid lowering drugs, low density lipoprotein (LDL) apheresis was performed during 72.8 + 43.2 months.

Apheresis technologies for prevention and regression of atherosclerosis: clinical results.

The objective of this multicenter trial, which included 28 clinical centers, was to determine if repeated low density lipoprotein (LDL)-apheresis using the Liposorber LA-15 system (Kaneka Corporation, Osaka, Japan) could lead to an additional acute and time averaged lowering of total cholesterol (TC) and LDL-cholesterol (LDL-C) in severely hypercholesterolemic patients whose cholesterol levels could not be controlled by appropriate diet and maximal drug therapy. A total of 6,798 treatments were performed on 120 patients, including eight homozygous familial hypercholesterolemia (FH), 75 heterozygous FH, and 37 unclassified FH or other hyperlipidemias, from 1988 through 1994. The mean TC and mean LDL-C levels at baseline were 410.

Surface treated large bore catheters with silver based coatings versus untreated catheters for extracorporeal detoxification methods.

Infection, thrombosis, and stenosis are among the most frequent complications associated with blood contacting catheters. Because these problems are usually related to surface properties of the base catheter material, surface treatment processes, such as ion implantation and ion beam assisted deposition (silver based coatings), can be used to mitigate such complications. Because these ion beam based processes affect only the near-surface region (approximately the outer 1 microm), there is little effect on bulk material properties.

Immunoadsorption--a new therapeutic possibility for multiple sclerosis?

A 46 year old woman suffers from chronic progressive multiple sclerosis. She was diagnosed in 1993. Because of many complications seen in conservative treatment, plasma exchange was started.

Protein A immunoadsorption (i.a.) in renal transplantation patients with vascular rejection.

Vascular rejection after renal transplantation is connected with a higher frequency of allograft dysfunction or graft loss. Plasmapheresis as an adjunctive therapy in the treatment of humoral mediated acute graft rejection was compared with protein A immunoadsorption. Eleven patients with acute graft rejection and high titers of cytotoxic HLA-Ab and/or signs of vascular rejection at graft biopsy (performed in 9 patients) have been treated.