thymosin and DiGeorge-Syndrome

Topics: Animals; Antigens, Surface; Cattle; Cyclic AMP; DiGeorge Syndrome; Guinea Pigs; Hormones; Humans; Immunity, Cellular; In Vitro Techniques; Infant, Newborn; Mice; Mice, Nude; Peptide Fragments; Rabbits; Rats; Swine; T-Lymphocytes; Thymalfasin; Thymectomy; Thymic Factor, Circulating; Thymopentin; Thymopoietins; Thymosin; Thymus Gland; Thymus Hormones

Topics: Blood Transfusion; Bone Marrow; Bone Marrow Cells; Bone Marrow Transplantation; Child; DiGeorge Syndrome; Female; Fetus; Humans; Immunity, Cellular; Immunoglobulins; Immunologic Deficiency Syndromes; Infant; Male; Plasma; Thymosin; Thymus Gland; Transfer Factor; Transplantation, Homologous

A Hispanic infant girl with DiGeorge syndrome underwent successful bone marrow transplantation (BMT) at age 28 1/2 weeks. She had typical facies, a cardiac defect, hypoparathyroidism, severe T and B cell immunodeficiency, and low levels of facteur thymique serique (FTS). In vitro incubation of the peripheral blood lymphocytes with thymosin alpha 1 showed no increase in the number of T cells on two occasions. A fetal thymus for transplantation was not available, and further review of past experience with thymic cells or factors revealed inconsistent and incomplete responses. Because of the patient's worsening clinical and immunologic status, BMT was performed, with her histocompatible brother as donor. The patient has had a good clinical and immunologic response to BMT, with evidence of T cell engraftment, improved B cell function, and increased levels of serum FTS. This experience indicates that minimal thymic influence is necessary for successful BMT and that patients with DiGeorge syndrome with significant T cell deficiency may benefit from this treatment.

Topics: Adult; Age Factors; B-Lymphocytes; Bone Marrow; Bone Marrow Transplantation; DiGeorge Syndrome; Evaluation Studies as Topic; Female; Humans; Immunoglobulin G; Immunologic Deficiency Syndromes; Infant, Newborn; Leukocyte Count; Male; T-Lymphocytes; Thymalfasin; Thymic Factor, Circulating; Thymosin

A child with the cerebro-hepato-renal syndrome of Zellweger, who was originally diagnosed as having the DiGeorge syndrome, was studied and transplanted unsuccessfully with cultured thymus. The pertinent literature is reviewed and the importance of distinguishing the two disorders emphasized. Autopsy studies reveal that transplanted cultured thymic fragments can attract lymphoid aggregates as early as 2 wk after transplantation.

Topics: Abnormalities, Multiple; Brain; Diagnosis, Differential; DiGeorge Syndrome; Humans; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Kidney; Liver; Lymphoid Tissue; Male; Mitochondria; Rosette Formation; Syndrome; Thymosin; Thymus Gland

Reconstruction of the T-cell immune defect in patients with the DiGeorge syndrome has been accomplished in the past by fetal thymus transplantation. Because of the risk of fatal graft-versus-host reaction with fetal thymus transplantation in patients with abnormal T-cell immunity, we have examined the effects of a thymus tissue extract, thymosin fraction 5, on the in vitro and in vivo immune function in children with the DiGeorge syndrome. T-cell numbers were increased with thymosin F5 in vitro in three of five patients. T-cell number and function was improved in three of four patients treated with thymosin F5 in vivo. Spontaneous improvement in the immune function of these patients cannot be excluded. These results suggest, however, that further trials with thymosin F5 therapy may be indicated in patients with the DiGeorge syndrome.

Topics: DiGeorge Syndrome; Humans; Immunologic Deficiency Syndromes; Infant; Infant, Newborn; Thymosin; Thymus Hormones

Topics: DiGeorge Syndrome; Drug Hypersensitivity; Histamine Release; Humans; Immunoglobulin E; Immunologic Deficiency Syndromes; Infant, Newborn; Leukocytes; Male; Streptolysins; T-Lymphocytes; Thymidine; Thymosin; Thymus Hormones

Topics: Adult; Ataxia Telangiectasia; Candidiasis; Chronic Disease; DiGeorge Syndrome; Eczema; Humans; Immunoglobulins; Immunologic Deficiency Syndromes; Lymphocyte Culture Test, Mixed; Male; Thymosin; Thymus Gland; Thymus Hormones; Wiskott-Aldrich Syndrome

Topics: Animals; Candidiasis, Chronic Mucocutaneous; Cattle; Cell Differentiation; DiGeorge Syndrome; Epithelium; Humans; Immunologic Deficiency Syndromes; Mice; T-Lymphocytes; Thymic Factor, Circulating; Thymopoietins; Thymosin; Thymus Gland; Thymus Hormones; Wiskott-Aldrich Syndrome; Zinc