A 29-year-old man with Crohn's disease involving the ileum and cecum was seen. He had angioneurotic edema with C1' esterase inhibitor deficiency. Later, his 50-year-old mother was evaluated because of abdominal pain. She had recurrent urticaria, C1' esterase inhibitor deficiency and radiographic studies showed Crohn's disease of the ileum. A maternal family history revealed other members affected with either Crohn's disease or angioneurotic edema. The clinical observations in this family suggest that angioneurotic edema associated with C1' esterase inhibitor deficiency may be closely linked genetically with a familial form of Crohn's disease.
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Hugh James Freeman (2000) studied this question.
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