A syndrome involving immunodeficiency and multiple intestinal atresias.
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.
Rothenberg
M E
ME
Division of Immunology, Harvard Medical School, Children's Hospital, Boston, MA, 02115, USA.
White
F V
FV
Chilmonczyk
B
B
Chatila
T
T
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