Recurrent Infections in an Ethiopian Boy with Autosomal Recessive Major Histocompatibility Complex Type I Deficiency: a Case Report on a Very Rare Primary Immunodeficiency Disorder and a Review of Principles in Evaluation and Management

Little is known about major histocompatibility complex type I deficiency, a rare form of primary immunodeficiency. This report describes the presentation of a three-year-old Ethiopian boy with recurrent sinopulmonary infections and genetic analysis showing him having autosomal recessive major histocompatibility complex type I deficiency—the first such report in a child of black African descent—and follows it with a summary of existing literature on the epidemiology, presentation, and diagnosis as well as principles of management of this disorder.