Idiopathic thrombocytopenic purpura in a liver transplant recipient with previous primary biliary cirrhosis

The loss of immunotolerance has been implicated in the pathogenesis of both primary biliary cirrhosis (PBC) and idiopathic, immune-mediated thrombocytopenic purpura (ITP). An association between these two autoimmune diseases has been well described. We describe a 41-year-old woman in whom ITP developed 457 days after liver transplantation for PBC while receiving immunosuppressive medications sufficient to maintain allograft function. Our case report, the first to describe post-transplant ITP in association with PBC, demonstrates the persistence of the underlying immune dysregulation of PBC after transplantation. The practice of decreasing the dosage of immunosuppressive medication to maintenance levels after transplantation may unmask the effects of this defect in immunotolerance.     

Incisional hernia after laparoscopic nephrectomy with intact specimen removal: caveat emptor

The choline-containing phosphoglycolipid, MfGL-II, is the major polar lipid of Mycoplasma fermentans PG18. Anti-MfGL-II antisera raised in rabbits using the purified MfGL-II as an immunogen were employed in immunogold electron microscopic and immunofluorescence studies showing that MfGL-II is uniformly distributed and exposed on the cell surface of M. fermentans cells. The specificity of the antibodies was determined by immunostaining of lipid extracts separated by thin layer chromatography. The antibodies recognize lipids specific to M. fermentans but did not cross-react with lipid extracts of M. penetrans, M. capricolum, M. gallisepticum or Acholeplasma laidlawii. As phosphocholine almost completely abolished antibody interaction with MfGL-II in an ELISA assay it is suggested that the anti-MfGL-II repertoire is composed primarily of anti-phosphocholine antibodies. The anti-MfGL-II antisera inhibit the attachment of M. fermentans to Molt-3 lymphocytes suggesting that MfGL-II plays a major role in M. fermentans-host cell interaction.     

Benign intracranial hypertension in children

Ceftriaxone has a very high plasma protein binding (up to 98%) that is saturable and decreases with higher concentrations. This high protein binding results in high concentrations in plasma that are frequently related to the anti-infective activity. However, because only the free fraction of the drug is pharmacologically active and most of the infections are located in the tissues, it is more relevant to evaluate unbound concentrations in the interstitial space. Plasma and tissue pharmacokinetics of ceftriaxone in rats after single intravenous administration were investigated at two different concentrations (50 and 100 mg/kg). Both plasma and tissue samples were taken simultaneously from the same animal and analyzed by reversed-phase high-performance liquid chromatography. Free tissue levels in the thigh muscle were measured by microdialysis. The concentration in plasma is much higher than the free concentration in tissue. After determination of nonlinear protein binding by microdialysis and including these parameters in the pharmacokinetic model, it is possible to predict free concentrations in the interstitial space from plasma levels for any given dose.     

Controlled filling and external cleaning of multi-wall carbon nanotubes using a wet chemical method

A controlled, material-independent and adaptable cold wet chemical procedure is described. It allows organic and inorganic compounds to be confined inside multi-wall carbon nanotubes. The procedure mainly consists of a lyophilization process to fill multi-wall carbon nanotubes (MWCNTs), and a washing method to clean their outer surface without removing the encapsulated material. The technique was tested by synthesizing CdS crystals inside the nanotubes. Morphological and structural studies of the CdS crystals demonstrated a complete control of the synthesis process, and the possibility of maximizing the crystal size and the filling efficiency that approached 70% of open-ended nanotubes.     

New method for monitoring of adsorption column saturation and regeneration I. Demonstration of the measurement principle

A new method suitable for the on-line monitoring of adsorption column saturation/regeneration cycles was developed based on the discovery that the electrical resistance of adsorbents changes dramatically upon adsorption and returns to its original value upon desorption, and that this change can be measured reliably. The phenomenon was demonstrated on four different adsorbent/adsorbate systems, on both single particles and in a packed bed between parallel plates. The observed phenomenon was studied on the carbon/CO2 system in more detail, because of the availability of extensive literature data. It was found that the resistance change in this system correlated with the amount of CO2 adsorbed. The correlation was used to construct an adsorption isotherm based on resistance change data.     

Aerosol Retention in the Vicinity of a Breach in a Tube Bundle: An Experimental Investigation

This article summarizes the main results of a bench-scale program focused on experimentally assessing the aerosol retention near the tube breach in a tube bundle. The major variables investigated were particle nature (polydispersed TiO ₂ agglomerates vs. solid, monodisperse SiO ₂ spheres) and Re _D (0.8?2.7· 10 ⁵ ). In addition, comparisons to other data sets provided insights into the particle aerodynamic size effect on retention efficiency. Results showed that particle nature substantially affects aerosol retention in the tube bundle: mass retention efficiency was low for TiO ₂ agglomerates (less than 30%) whereas it was much higher for SiO ₂ particles (around 85%). Retention efficiency is also affected by Re _D : its sensitivity was found to follow a log-normal behavior with a maximum retention attained at Re _D near 1· 10 ⁵ . This evolution with Re _D was similar for both types of compounds. Particle size also influences retention efficiency: the bigger the TiO₂ agglomerates the lower retention efficiency (no data were available for SiO ₂ ). Among all these variables, particle nature was noted to have a prime importance for in-bundle retention, whereas Re _D and particle aerodynamic size, although also affect retention efficiency, did not play such a key role. In light of the results, the presence of retention-inhibiting mechanisms such as fragmentation, resuspension or bouncing has been discussed. The data recorded will enhance the overall understanding of the governing mechanisms involved and will serve as a database against which compare model predictions. Nevertheless, further experimental data would be desirable to set up a sound database.     

CRITICAL FLUX DETERMINATION IN ULTRAFILTRATION OF WASTEWATER FROM A FOOD INDUSTRY BY OPTIMIZATION METHOD

Two ultrafiltration membranes with different geometries (spiral polymeric and tubular ceramic) but similar cutoffs were used to treat wastewater from a food industry. Hydrodynamic conditions were optimized by statistical methods as a strategy to get more accurate values of the critical parameters and then to produce higher water flux and minimization of membrane fouling. The validation of the optimization method was obtained by experimental critical flux determination at critical parameters. Membrane fluxes revealed significant differences during filtration. The polymeric membrane showed an optimal flux of 45.60 Lh^?1 m^?2 at 3.21 bar while operating at a stable time of 11.61 h, whereas optimal flux of the ceramic membrane was 32.43 Lh^?1 m^?2 at 3.98 bar for 16.03 h. Experimental critical flux values were only slightly lower than optimal fluxes for both membranes, showing the validity of the statistics models applied. Negligible osmotic pressure was found on the two membranes at critical flux parameters, indicating irreversible fouling for both cases. The polymeric membrane revealed strong fouling behavior and the ceramic membrane showed a weak form; the flux decline occurred first in the polymeric membrane, whereas the ceramic membrane exhibited high stability during the filtration operations. A high degree of purification of wastewater was obtained by this membrane at critical flux conditions.     

SEC23B Loss-of-Function Suppresses Hepcidin Expression by Impairing Glycosylation Pathway in Human Hepatic Cells

Biallelic pathogenic variants in the SEC23B gene cause congenital dyserythropoietic anemia type II (CDA II), a rare hereditary disorder hallmarked by ineffective erythropoiesis, hemolysis, erythroblast morphological abnormalities, and hypo-glycosylation of some red blood cell membrane proteins. Abnormalities in SEC23B, which encodes the homonymous cytoplasmic COPII (coat protein complex II) component, disturb the endoplasmic reticulum to Golgi trafficking and affect different glycosylation pathways. The most harmful complication of CDA II is the severe iron overload. Within our case series (28 CDA II patients), approximately 36% of them exhibit severe iron overload despite mild degree of anemia and slightly increased levels of ERFE (the only erythroid regulator of hepcidin suppression). Thus, we hypothesized a direct role of SEC23B loss-of-function in the pathomechanism of hepatic iron overload. We established a hepatic cell line, HuH7, stably silenced for SEC23B. In silenced cells, we observed significant alterations of the iron status, due to both the alteration in BMP/SMADs pathway effectors and a reduced capability to sense BMP6 stimulus. We demonstrated that the loss-of-function of SEC23B is responsible of the impairment in glycosylation of the membrane proteins involved in the activation of the BMP/SMADs pathway with subsequent hepcidin suppression. Most of these data were confirmed in another hepatic cell line, HepG2, stably silenced for SEC23B. Our findings suggested that the pathogenic mechanism of iron overload in CDA II is associated to both ineffective erythropoiesis and to a specific involvement of SEC23B pathogenic variants at hepatic level. Finally, we demonstrated the ability of SEC23B paralog, i.e., SEC23A, to rescue the hepcidin suppression, highlighting the functional overlap between the two SEC23 paralogs in human hepatic cells.