[Various hereditary immune deficiencies linked to the X-chromosome].
The organs of immune system in hereditary immunodeficiencies linked to X-chromosome, particularly in Nezelof's syndrome and chronic granulomatosis (4 cases) were studied histologically. The alterations of thymus found in Nezelof's syndrome suggest the disturbance of its morphogenesis at the embryonal stage, those found in chronic granulomatosis suggest the postnatal disturbance of thymus morphogenesis. The children die of polytopic infections foci. Generalized BCG-itis is observed in children with chronic granulomatosis.
D002675:Child, Preschool; D006105:Granulomatous Disease, Chronic / Q000235:genetics / Q000473:pathology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000473:pathology; D007223:Infant; D008099:Liver / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D013154:Spleen / Q000473:pathology; D013577:Syndrome; D013950:Thymus Gland / Q000473:pathology; D014960:X Chromosome
Nekotorye nasledstvennye immunodefitsity, stseplennye s X-khromosomoĭ.