title: [Generalized progressive vaccinia in a child with primary humoral and cellular immunodeficiency (author's transl)].
abstract: A case of generalized progressive vaccinia with lethal outcome after smallpox vaccination observed in an 8 months old girl during 1968 is reported. This complication was the first sign of an underlying immune deficiency in this child. The most conspicious findings suggesting a humoral immune defect were an absent serum IgM in combination with decreased IgA and IgG levels. An additional cellular defect was suggested by a generalized hypoplasia of the thymus and the entire lymphatic system as shown during autopsy. Vaccinia virus could be found not only in skin eruptions intra vitam but also in lung, liver and brain tissue in post mortem studies.
mesh_terms: D001344:Autopsy; D005260:Female; D006801:Humans; D007111:Immunity, Cellular; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000276:immunology / Q000473:pathology; D007223:Infant; D008208:Lymphatic System / Q000473:pathology; D013950:Thymus Gland / Q000473:pathology; D014615:Vaccinia / Q000209:etiology*
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M
vernacular_title: Vaccinia generalisata progressiva bei Antikörpermangel-Syndrom (primäre kombinierte immunologische Defizienz)