Gastric pathology in pediatric Crohn’s disease
نویسنده
چکیده
Tufting enteropathy First described in 1994, tufting enteropathy is a clinicopathological entity in which infants present with watery diarrhea in the first month of life. The pattern of inheritance appears to be autosomal recessive. Jejunal biopsy shows partial or subtotal villous atrophy with normal or hyperplastic crypts. The lamina propria is not inflamed and intraepithelial lymphocytes are not increased. The most striking finding is of epithelial cell tufts, that is, closely packed enterocytes with rounded apical plasma membranes. While this appearance may be seen focally in other enteropathies, in tufting enteropathy between 80% and 90% of the surface exhibits the change. Treatment at present is with total parenteral nutrition. Patients with autoimmune enteropathy present with diarrhea in the first year of life and may present in the neonatal period. A family history of autoimmune disease is often present. In some patients, the disorder may occur in the context of a primary immunodeficiency syndrome. Jejunal biopsies show mild, partial to subtotal villous atrophy with crypt hyperplasia. Surface enterocytes show numerous intraepithelial lymphocytes and numerous vacuolated and individually necrotic surface enterocytes as a marker of cytopathic damage. The lamina propria shows dense lymphocytic and plasma cell infiltration (although in agammaglobulinemia and hypogammaglobulinemia plasma cells are absent) and there may be crypt abscesses. Antigut antiepithelial or antigoblet cell antibodies are detected in the blood. Treatment is directed to the underlying immune defect in immunodeficient patients and immunosuppressive therapy in those who do not have a demonstrable immunodeficiency.
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