title: [New data on thymomegaly as a syndrome of congenital (primary) immunodeficiency].
abstract: Basing on a combined clinicomorphological investigation of the thymicolymphatic and neuroendocrine systems including measurements of blood thymic hormones and their levels in thymic tissue it is shown that congenital thymomegaly can be considered a dysfunction of the hypothalamo-hypophysial system. The dysfunction manifests with polyglandular endocrinopathy and congenital immunodeficiency related to the group of thymus-dependent immunodeficiencies. Thymomegaly is often coupled with congenital malformations.
authors.forename: O V
authors.initials: OV
authors.lastname: Zaĭrat'iants
authors.forename: V V
authors.initials: VV
authors.lastname: Serov
authors.forename: L G
authors.initials: LG
authors.lastname: Kuz'menko
mesh_terms: D002675:Child, Preschool; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000151:congenital* / Q000209:etiology / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D008221:Lymphoid Tissue / Q000473:pathology; D009490:Neurosecretory Systems / Q000473:pathology; D009929:Organ Size; D013601:T-Lymphocytes / Q000473:pathology; D013950:Thymus Gland / Q000473:pathology; D013952:Thymus Hyperplasia / Q000151:congenital* / Q000209:etiology / Q000473:pathology
vernacular_title: Novye dannye o timomegalii kak sindrome vrozhdennogo (pervichnogo) immunnogo defitsita.