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Hie syndrome (hyperimmunoglobulinemia e syndrome) 5


HIE Syndrome (Hyperimmunoglobulinemia E Syndrome) 5

General: Mononuclear cells inhibit normal neutroph 616d31g il and monocyte chemotaxis; caused by Staphylococcus aureus and Candida trachomatis; autosomal dominant phenotype; onset at age 1 to 8 weeks; marked elevation of immunoglobulin E.

Ocular: Atopic keratitis; keratoconjunctivitis; photophobia; corneal ulcer; chorioretinal scars; lid edema.

Clinical: Pruritic dermatitis; dry skin; skin abscesses; pneumonia; upper respiratory infections; reduced resistance; coarse facial features.

Aswad MI, et al. Plasmapheresis treatment in patients with severe atopic keratoconjunctivitis. Ophthalmology 1988; 95:444-447.

Butrus SI, et al. Vernal conjunctivitis in hyperimmunoglobulinemia E syndrome. Ophthalmology 1984; 91: 1213-l216.

Leung DYM, Geha RS. Clinical and immunological aspects of the HIE syndrome. Hematol Oncol Clin North Am 1988; 2:81-l00.




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