title: A syndrome involving immunodeficiency and multiple intestinal atresias.
abstract: Multiple intestinal atresias (MIA) is a severe form of intestinal atresias throughout the gastrointestinal tract. In two reports, MIA have been associated with severe immunodeficiency. We report a newborn girl who had profound humoral and B cell immunodeficiency and impaired T cell function. The patient had agammaglobulinemia and decreased blood lymphocytes, with virtually no B cells in the blood or in intestinal lymph nodes. T cells were reduced in number and weakly proliferated to mitogens. These data suggest that a syndrome involving the development of MIA is associated with various forms of severe immunodeficiency, and therefore newborns with MIA should be examined for immunodeficiency.
authors.affiliation: Division of Immunology, Harvard Medical School, Children's Hospital, Boston, MA, 02115, USA.
authors.forename: M E
authors.initials: ME
authors.lastname: Rothenberg
authors.forename: F V
authors.initials: FV
authors.lastname: White
authors.forename: B
authors.initials: B
authors.lastname: Chilmonczyk
authors.forename: T
authors.initials: T
authors.lastname: Chatila
mesh_terms: D000361:Agammaglobulinemia / Q000150:complications / Q000276:immunology / Q000473:pathology; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000276:immunology / Q000473:pathology; D007231:Infant, Newborn; D007409:Intestinal Atresia / Q000150:complications* / Q000276:immunology / Q000473:pathology; D018655:Lymphocyte Count