1/4. plasmapheresis treatment in patients with severe atopic keratoconjunctivitis.A 19-year-old woman with hyperimmunoglobulinemia E (the hyper-IgE syndrome [HIE]) and a 62-year-old man with atopic dermatitis experienced dramatic improvement in their chronic ocular symptoms and signs of atopic keratoconjunctivitis after plasmapheresis was instituted. Both patients had previously received topical and oral corticosteroids as well as topical cromolyn sodium without significant beneficial effect. The authors recommend a trial of plasmapheresis in selected patients with recalcitrant and debilitating atopic keratoconjunctivitis when standard therapy proves unsuccessful.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
2/4. Eosinophilic gastroenteritis in extreme allergy. Immunopathological comparison with nonallergic gastrointestinal disease.Intestinal mucosal biopsy immunoglobulin content in a patient with eosinophilia, allergic gastroenteropathy (atopy, food sensitivities, protein-losing enteropathy, iron deficiency anemia, and growth retardation), and hyperimmunoglobulinemia E (68,000 units) was compared with that of a control group and a group with eosinophilic gastroenteritis who had no atopic features. The patient had no evidence of cellular or humoral immunodeficiency but was severely hypersensitive to multiple inhalant and dietary allergens. In contrast to the findings in patients with nonallergic eosinophilic gastroenteritis, whose intestinal immunoglobulin content was similar to that of controls, tissue immunoglobulin IgE and IgG was markedly increased in the allergic patient. These results support an intestinal reaginic mechanism in the etiology of the allergic form of eosinophilic gastroenteritis.- - - - - - - - - - ranking = 1keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
3/4. Vernal conjunctivitis in the hyperimmunoglobulinemia E syndrome.Hyper-IgE syndrome (HIE) appears to be related to an immunoregulatory imbalance characterized by severe deficiency of suppressor T cells, elevated levels of IgE antibodies, and repeated infection of various organ systems. We report the association of HIE syndrome in two definite cases and one probable case of vernal conjunctivitis. This association suggests that T cell-mediated imbalance may be one factor in the pathogenesis of vernal conjunctivitis.- - - - - - - - - - ranking = 4keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |
4/4. Severe staphylococcal disease associated with allergic manifestations, hyperimmunoglobulinemia E, and defective neutrophil chemotaxis.Neutrophil granulocyte function was determined in three patients with systemic staphylococcal infection, clinical manifestations of generalized allergic disease, and hyperimmunoglobulinemia E. Each of the patients had urticarial skin rashes before or at the time of development of staphylococcal suppurative lymphadenitis, pneumonia, or sepsis. Neutrophil chemotaxis, random migration, phagocytosis, and bactericidal capacity were assessed to determine if an abnormality in these functions might have contributed to the development of severe staphylococcal infections. Each of the three patients with generalized urticaria was found to have a marked defect in neutrophil chemotaxis. The mean chemotactic index of the patients was 12 /- 4, whereas that of 20 controls was 72 /- 11. Neutrophil random migration, phagocytosis, and bactericidal capacity were normal in each patient. The serum or plasma of the patients did not inhibit chemotaxis of control neutrophils and did not contain an increased concentration of the chemotactic-factor inactivator found in normal serum. Treatment of the neutrophils of these three patients with the competitive histamine H2 receptor blocking agent, burimamide, produced a significant increase in chemotactic responsiveness. These studies suggest the possibility of pharmacologic modification of neutrophil granulocyte function.- - - - - - - - - - ranking = 5keywords = hyperimmunoglobulinemia (Clic here for more details about this article) |