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Gastrointestinal microsporidiosis is a major cause of chronic diarrhea in people with acquired immune deficienc syndrome. However, it can also affect individuals with inherited or congenital immunodeficiency. We reported a case of intestinal microsporidiosis and salmonellosis in a young boy with Hyper IgM syndrome. He presented with severe diarrhea and fever for 5 days. Stool examination showed heavy infection of Microsporidia spp. PCR confirmed the species as Enterocytozoon bieneusi. He was treated with albendazole for 25 days which abated the symptoms. To our knowledge, this is the first reported case of intestinal microsporidiosis in inherited immunodeficiency, X-linked Hyper IgM (XHIGM).
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