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Biomedical subjects

S Cornwall

Publications and source records attributed to S Cornwall.

10 recordsLinked to original sources

Vitamin E deficiency and impaired cellular immunity related to intestinal fat malabsorption.

This report describes a patient in whom a severe vitamin E deficiency developed secondary to an intestinal malabsorptive disorder. In vivo and in vitro impairment of T-cell function, as well as a polyneuropathy, were observed in conjunction with this vitamin deficiency. Repletion of the vitamin deficiency was associated with marked improvement in the T-cell functions and modest improvement in the neuropathy. Observations in this patient suggest that severe vitamin E deficiency in humans may impair T-cell activity and that correction of the deficient state may reverse these T-cell abnormalities. Further studies will need to be performed to confirm these findings.

Fats

Macrophage heterogeneity in normal colonic mucosa and in inflammatory bowel disease.

Immunohistological techniques using monoclonal antibodies were employed to study the morphology and phenotypic expression of macrophage like cells in ulcerative colitis, Crohn's colitis and histologically normal colonic mucosa. The antibody RFD1 identifies interdigitating (antigen presenting) cells whereas RFD7 binds to mature tissue macrophages. In normal colonic mucosa, the majority of cells recognised by these reagents were positive for Class II antigen expression and a median 87% (range 80-95%) were positive for both RFD1 and RFD7, with 6.5% (ranges 1-14%) positive for either antibody alone. There was much greater macrophage heterogeneity in the ulcerative colitis and Crohn's colitis biopsies than in normal mucosa. Clusters of RFD9+ cells (epithelioid cells) were found in Crohn's colitis and, to a lesser extent, in ulcerative colitis. Some Crohn's colitis sections showed replacement of the normal colonic macrophage phenotype with RFD1-RFD7+ cells (classical scavenger macrophages). The degree of this replacement correlated with the histological severity of the disease. By contrast, large numbers of RFD1+ RFD7- cells, with long dendritic processes, were found in intimate association with the lymphoid infiltrates in the lamina propria of the ulcerative colitis sections. Future studies of the factors controlling macrophage differentiation in tissues may help to explain the greater macrophage heterogeneity in inflammatory bowel disease and the differences between ulcerative colitis and Crohn's colitis observed in this study.

Colitis, Ulcerative

A family demonstrating inheritance of the Leach phenotype: a Gerbich-negative phenotype associated with elliptocytosis.

The proposita of a Canadian family and 2 of her sibs have the rare Leach phenotype and provide the first evidence that the Leach phenotype is an inherited condition. Their red cells are Ge: -2, -3 and fail to react, by the antiglobulin test, with the monoclonal antibodies GERO, BRIC 4 and BRIC 10. The minor sialoglycoproteins beta, beta 1 and gamma are apparently absent from their red cell membranes. A proportion of their red cells are elliptocytes indicating that beta and/or beta 1 and/or gamma have a function in maintaining normal red cell shape.

Antibodies, Monoclonal

A third example of haemolytic auto-anti-Vel.

A non-transfused, 43 year old Caucasian female presented with acute haemolytic anaemia and splenomegaly. Sections of bone marrow showed erythroid hyperplasia. The patient's red blood cells gave a negative reaction with polyspecific antiglobulin serum, but a positive reaction with specific anti-IgM. A heat eluate prepared from her red cells showed anti-Vel specificity. Her serum agglutinated only Vel-positive cells including her own. All papain pre-treated red cells including her own and Vel-negative cells were completely haemolysed at 37 degrees C. The percentage of haemolysis of Vel-positive cells was greater than that of Vel-negative cells.

Adult