Mosaic pattern of sucrase isomaltase deficiency in two brothers

The pathophysiology of mucosal changes observed in infants with chronic protracted diarrhea is poorly understood. We report on two brothers suffering from a special form of sucrase isomaltase (SI) deficiency. The children presented with weight loss and dyspepsia after sucrose exposition. We performed an H respiration test, which showed a pathologic result in the younger brother. Analysis of the brush border enzyme activities showed low expression of lactase and SI. Immunoelectron microscopy of duodenal biopsies showed an isolated SI deficiency in a mosaic pattern [e.g., 42% (14%) crypt enterocytes and 64% (59%) villus enterocytes with decreased amounts of SI on microvilli], whereas lactase and aminopeptidase n (ApN) were present at the apical membrane of all cells in a normal range. The SI mosaic pattern of these patients shows that the enterocytes contain low amounts of SI on the apical membrane but express normal quantities of other disaccharidases. These findings suggest the existence of different clonal expressions or specific (posttranslational) mechanisms of postGolgi transportation for individual brush border enzymes. It remains unresolved whether the mosaic distribution is part of a normal maturation process or caused by a lack of an overall control mechanism in the expression of brush border hydrolases.

Las Islas Malvinas y el empr��stito Baring Brothers

Fil: Luzi, Alejandra Noem��. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educaci��n; Argentina.

Las Islas Malvinas y el empr��stito Baring Brothers

Fil: Luzi, Alejandra Noem��. Universidad Nacional de La Plata. Facultad de Humanidades y Ciencias de la Educaci��n; Argentina.

Search for the pathogenesis of the differing phenotype in two compound heterozygote Hungarian brothers with the same genotypic triosephosphate isomerase���deficiency

In a Hungarian family with triosephosphate isomerase (TPI) deficiency, two compound heterozygote brothers were found with the same severe decrease in TPI activity, but only one of them had the classical symptoms. In search for the pathogenesis of the differing phenotype of the same genotypic TPI deficiency, an increase in red cell membrane fluidity was found. There were roughly 100% and 30% more 16:0/20:4 and 18:0/20:4 diacyl-phosphatidylcholine species in erythrocytes from the two TPI-deficient brothers than in the probes from healthy controls. The activities of acethylcholinesterase and calmodulin induced Ca2+ ATPase were significantly enhanced in erythrocytes from the propositus as compared with those of the neurologically symptom-free brother and other members of the TPI-deficient family as well as to those from healthy controls. Both enzymes are crucially involved in the function of nerve cells. The observed differences in membrane fluidity and enzyme activities between the erythrocytes from the phenotypically differing TPI-deficient brothers underline the importance of investigations into the effect of biophysical changes in the lipid environment of the membrane proteins on the development of disseminated focal neurological disorders of unknown pathogenic origin.

Slim Styles and the Brothers of the Light

Slim Styles and the Brothers of the Light consists of a short novella, intended to be part of a series, introduced by a narrative essay. The work is about the search for identity, the pursuit of happiness, and the struggle to maintain self assurance while finding a place in the world. The story follows the main character as he slowly learns the truth about an organization he joined looking for a sense of value and worth. With his back against the wall he has to return to the home he was taught to be ashamed of, face the friends he left behind, and apologize to the lover he took for granted. The manuscript is a work in progress.

Alegato presentado a la Sala 1a. de la Corte de Apelaciones como abogado de los sen��ores Ro��sing Brothers & Co. [et al]

At head of title: La cuestio��n "R. Samper y Ci��a".

Catalogue of a collection of pictures, including examples of the works of the brothers Le Nain, and other works of art.

"Suggested classification of the works of the brothers Le Nain," signed R. C. W. [i.e. Robert C. Witt]: p. 7-20.

The Dalton Brothers and their astounding career of crime /

Disbound Original Held in Oak Street Library Facility.