Reaction Kinetics and Process Model of the Polyacrylonitrile Fibers Stabilization Process Based on Dielectric Measurements.

Microwave-based dielectric heating is a suitable method for energy- and time-efficient processes. Considering the energy required in the production of carbon fibers, it is evident that microwave-based dielectric heating during the different phases of the production needs to be considered too. Nevertheless, the dielectric properties of the processed material needs to be known for the design of an appropriate microwave applicator. When looking at the first stage in the production, the stabilization stage of the PAN fiber, the important data about the dielectric properties is very limited in literature. For this reason, first in-situ temperature-dependent measurements of the dielectric properties during the stabilization stage are presented. The impact of raising temperatures and chemical reactions on the dielectric properties of the heated PAN fiber is discussed. Secondly, the steps taken to set up the reaction kinetics from the dielectric loss point of view are given. This enables determination of the reaction degree as a function of the measured dielectric loss for the first time. The established correlation opens the potential for the application to processes such as an in-situ quality determination. The strong temperature impact on the process is shown, and reaction kinetics are analyzed accordingly. In a final third step, a heat transfer model is presented. It utilizes the evaluated reaction kinetics data and microwave heating, creating a first modelling approach for monitoring and controlling the desired fiber temperature, leading towards an online process.

Human exome and mouse embryonic expression data implicate ZFHX3, TRPS1, and CHD7 in human esophageal atresia.

INTRODUCTION: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) occurs approximately 1 in 3.500 live births representing the most common malformation of the upper digestive tract. Only half a century ago, EA/TEF was fatal among affected newborns suggesting that the steady birth prevalence might in parts be due to mutational de novo events in genes involved in foregut development. METHODS: To identify mutational de novo events in EA/TEF patients, we surveyed the exome of 30 case-parent trios. Identified and confirmed de novo variants were prioritized using in silico prediction tools. To investigate the embryonic role of genes harboring prioritized de novo variants we performed targeted analysis of mouse transcriptome data of esophageal tissue obtained at the embryonic day (E) E8.5, E12.5, and postnatal. RESULTS: In total we prioritized 14 novel de novo variants in 14 different genes (APOL2, EEF1D, CHD7, FANCB, GGT6, KIAA0556, NFX1, NPR2, PIGC, SLC5A2, TANC2, TRPS1, UBA3, and ZFHX3) and eight rare de novo variants in eight additional genes (CELSR1, CLP1, GPR133, HPS3, MTA3, PLEC, STAB1, and PPIP5K2). Through personal communication during the project, we identified an additional EA/TEF case-parent trio with a rare de novo variant in ZFHX3. In silico prediction analysis of the identified variants and comparative analysis of mouse transcriptome data of esophageal tissue obtained at E8.5, E12.5, and postnatal prioritized CHD7, TRPS1, and ZFHX3 as EA/TEF candidate genes. Re-sequencing of ZFHX3 in additional 192 EA/TEF patients did not identify further putative EA/TEF-associated variants. CONCLUSION: Our study suggests that rare mutational de novo events in genes involved in foregut development contribute to the development of EA/TEF.

Identification of co-occurrence in a patient with Dent's disease and ADA2-deficiency by exome sequencing.

Patients with co-occurrence of two independent pathologies pose a challenge for clinicians as the phenotype often presents as an unclear syndrome. In these cases, exome sequencing serves as a powerful instrument to determine the underlying genetic causes. Here, we present the case of a 4-year old boy with proteinuria, microhematuria, hypercalciuria, nephrocalcinosis, livedo-like rash, recurrent abdominal pain, anemia and continuously elevated CRP. Single exome sequencing revealed the pathogenic nonsense mutation p.(Arg98*) in the CLCN5 gene causing the X-linked inherited, renal tubular disorder Dent's disease. Furthermore, the two pathogenic and compound heterozygous missense variants p.(Gly47Ala) and p.(Pro251Leu) in the CECR1 gene could be identified. Mutations in the CECR1 gene are associated with a hereditary form of polyarteritis nodosa, called ADA2-deficiency. Both parents were carriers of a single heterozygous variant in CECR1 and the mother was carrier of the CLCN5 variant. This case evidently demonstrates the advantage of whole exome sequencing compared to single gene testing as the pathology in the CECR1 gene might have only been diagnosed after the occurrence of signs of systemic vasculitis like strokes or hemorrhages. Therefore, treatment and prevention can now start early to improve the outcome of these patients.

Foreign body in the bladder mimicking nephritis.

Cases of foreign bodies in the bladder self-inserted via urethra are not rare in childhood. Urinary tract infection, dysuria, lower abdominal pain, or haematuria with and without pain are common symptoms. We report on a 11-year-old boy with accidentally detected microscopic haematuria, proteinuria and leukocyturia. Because of increasing proteinuria up to 2330 mg/g creatinine and elevated antistreptolysin titre glomerulonephritis was suspected. However, some echogenic material was detected in the bladder by ultrasound. X-ray of the pelvis showed a 30 cm long tube projecting onto the bladder. The boy then admitted having had inserted a plastic tube into the urethra two years ago. The foreign body was removed cystoscopically. Four weeks after cystoscopy erythrocyturia, leucoyturia and proteinuria had disappeared. We state that symptoms of a local inflammation caused by a foreign body in the bladder can imitate the symptoms of nephritis.

Metabolic control analysis of the Warburg-effect in proliferating vascular smooth muscle cells.

The accumulation and proliferation of vascular smooth muscle cells (VSMC) within the vessel wall is an important pathogenic feature in the development of atherosclerosis. Glucose metabolism has been implicated to play an important role in this cellular mechanism. To further elucidate the role of glucose metabolism in atherogenesis, glycolysis and its regulation have been investigated in proliferating VSMC. Platelet derived growth factor (PDGF BB)-induced proliferation of VSMCs significantly stimulated glucose flux through glycolysis. Further evaluating the enzymatic regulation of this pathway, the analysis of flux:metabolite co-responses revealed that anaerobic glycolytic flux is controlled at different sites of gycolysis in proliferating VSMCs, being consistent with the concept of multisite modulation. These findings indicate that regulation of glycolytic flux in proliferating VSMCs differs from traditional concepts of metabolic control of the Embden-Meyerhof pathway.