Adaptative response of alpha- and beta-glucosidase kinetics along the villi of rat self-filling jejunal blind loops.

Four and 12 days after the construction of self-filling jejunal blind loops in the rat, the apparent Km- and Vmax-values of lactase/beta-glucosidase and neutral alpha-glucosidase were determined by in-situ quantitative enzyme histochemistry, and changes in the villus-crypt architecture of the mucosa were examined by microdissection. The results were compared with corresponding data from sham-operated controls. The kinetics data were obtained from the base and the transition zone between medium and apical villus third by the use of a microdensitometric technique. The apparent Vmax of lactase/beta-glucosidase is significantly smaller than in the control rats at both measuring sites of the villi and even decreases from day 4 to day 12. The apparent Vmax of neural alpha-glucosidase is not affected, and thus the same increase in enzyme activity along the villi as in the controls is observed. The apparent Km of this enzyme, however, is already significantly increased on day 4 at both villus positions in the blind loops. A pronounced increase in villus surface area is detected in the blind loops as a result of an increase in crypt cell proliferation. The results indicate that enzymatic adaptation in the self-filling blind loops of rat jejunum exhibits different patterns for brush border alpha- and beta-glucosidases and is at least in part accomplished independently of the pronounced mucosal transformation occurring in this experimental condition.     

STIG study: real-world data of long-term outcomes of adults with Pompe disease under enzyme replacement therapy with alglucosidase alfa

Journal of Neurology - Pompe disease is one of the few neuromuscular diseases with an approved drug therapy, which has been available since 2006. Our study aimed to determine the real-world...     

Phenotypically resembling myeloid derived suppressor cells are increased in children with HIV and exposed/infected with Mycobacterium tuberculosis

Increased disease susceptibility during early life has been linked to immune immaturity, regulatory T‐cell/TH2 immune biasing and hyporesponsiveness. The contribution of myeloid derived suppressor cells (MDSCs) remains uninvestigated. Here, we assessed peripheral MDSC in HIV‐infected and ‐uninfected children with tuberculosis (TB) disease before, during and after TB treatment, along with matched household contacts (HHCs), HIV‐exposed, ‐infected and ‐uninfected children without recent TB exposure. Serum analytes and enzymes associated with MDSC accumulation/activation/function were measured by colorimetric‐ and fluorescence arrays. Peripheral frequencies of cells phenotypically resembling MDSCs were significantly increased in HIV‐exposed uninfected (HEU) and M.tb‐infected children, but peaked in children with TB disease and remained high following treatment. MDSC in HIV‐infected (HI) children were similar to unexposed uninfected controls; however, HAART‐mediated MDSC restoration to control levels could not be disregarded. Increased MDSC frequencies in HHC coincided with enhanced indoleamine‐pyrrole‐2,3‐dioxygenase (IDO), whereas increased MDSC in TB cases were linked to heightened IDO and arginase‐1. Increased MDSC were paralleled by reduced plasma IP‐10 and thrombospondin‐2 levels in HEU and significantly increased plasma IL‐6 in HI HHC. Current investigations into MDSC‐targeted treatment strategies, together with functional analyses of MDSCs, could endorse these cells as novel innate immune regulatory mechanism of infant HIV/TB susceptibility.     

Ras-associated small GTPase 33A, a novel T cell factor, is down-regulated in patients with tuberculosis

Ras-associated small GTPases (Rabs) are specific regulators of intracellular vesicle trafficking. Interference with host cell vesicular transport is a hallmark of many intracellular pathogens, including the notable example Mycobacterium tuberculosis. We performed, by quantitative polymerase chain reaction, gene-expression analyses for selected Rab molecules in peripheral-blood mononuclear cells from patients with tuberculosis (TB) and healthy control subjects, to identify candidate genes that are critically involved in the host immune response. Comparison revealed significant differences in the expression of genes for Rab13, Rab24, and Rab33A. Rab33A gene expression was down-regulated in patients with TB and was predominantly expressed in CD8+ T cells. We excluded possible influences of differences in T cell percentages between the 2 study groups, demonstrating that Rab33A gene expression changes on the single-cell level. In vitro, Rab33A RNA expression was induced in T cells on activation and by dendritic cells infected with M. tuberculosis. Our findings identify Rab33A as a T cell regulatory molecule in TB and suggest its involvement in disease processes.     

Diffuse normolipemic plane xanthomatosis]

The case of a diffuse plane normolipaemic xanthomatosis in a 74-year-old woman is reported which has developed since about 5 years. There was no sign of any disease of the reticulhistiocytic system. With regard to the prognosis the arterial hypertension is of particular interest.     

Seasonal burning palate in allergy against grass pollen

An immediate type I allergy elicited by IgE antibodies against pollen of grasses and cereals was demonstrated as the rare cause of palatinal stomatopyrosis (burning palate). The casual relationship was suggested through history and proven by skin, radioallergosorbent and exposition tests.