[Hypoplasia of the thymus gland in children].
Morphological changes of the thymus, tonsils, lymph nodes, spleen, and other organs in two observations of severe combined insufficiency (SCIN) in infants of the first year of life are described. In both cases there was hypoplasia of the thymus with the lack or occasional thymic bodies and poor content of thymocytes in the lobules. In one infant, hypoplasia of the thymus was combined with intestinal angiomatosis which suggested the syndrome of ataxia-teleangiectasia. In the other infant, SCIN was associated with severe granulocytopenia of the Kostman type. In this case epithelial cells of the lobules formed adenomatous structures. Both infants died with sepsis developing against the background of SCIN.
D000380:Agranulocytosis / Q000151:congenital; D000798:Angiomatosis / Q000151:congenital; D003110:Colonic Neoplasms / Q000151:congenital; D004062:DiGeorge Syndrome / Q000151:congenital* / Q000473:pathology; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000151:congenital*; D007223:Infant; D008297:Male; D013950:Thymus Gland / Q000473:pathology*
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