Severe combined immunodeficiency disease

Outlined severe combined immunodeficiency disease (severe combined immunodeficien cy disease, SCID), the Swiss-type Hypogammaglobulinemia. thymic lymphoid tissue stunting and network organization dysplasia is a heavy immune deficiency disease. Its characteristics are congenital and hereditary B T-cell abnormalities. This group of diseases is autosomal recessive or-genetic chain. 50% with positive family history of SCID. Network organization associated with hypoplasia of SCID by the Department of primitive hematopoietic stem cells defect; Swiss-type Hypogammaglobulinemia of lymphoid stem cell defect; Some T cells from SCID poorly differentiated mature B cells and obstacles. Clinical manifestations of Health more than three months after the start of infection, molds, bacteria and protozoa, and recurring pneumonia, chronic diarrhea, Oral candidiasis with skin infections and middle ear to the other. Children growth retardation. Generally not superficial examination of lymph node and tonsil. No chest radiographic examination baby thymus shadow. If due to negligence of smallpox vaccination for children with vaccines or take polio vaccine, cause fatal disease of smallpox and polio. In addition to the importation of children with immune activity of whole blood lymphocytes will occur graft-versus host disease. Hypoplastic network organization is the most weight SCID-type, characterized by immune-system deficiencies and serious agranulocytosis. Most of sepsis due to streptococcus after birth and to die within a week. SCID also associated with bone hypoplasia resulting short limb dwarfism, and the hair back from that disease and red Ichthyosis damage. With adenosine dehydrogenase (ADA) for lack of SCID autosomal recessive inheritance. Clinical manifestations and ordinary SCID similar, but more bone damage, often involving cartilage connecting, spine, pelvis and the scapula. ADA is the catabolism of purine enzyme catalysis; ADA lack of mechanisms through several immunomodulatory effects arising from the disease. Detection of fetal red blood cells and cells of ADA activity, a clear diagnosis and prenatal diagnosis to provide a basis. Treatment to prevent graft-versus-host disease, should be lost in the whole blood or blood products irradiation application, inactivated immune activity in cells, or too cold blood. Application of bone marrow transplantation for the treatment of immune reconstitution of the disease is the most effective method. Using HLA matching compatible bone marrow compatriots, the highest success rate; HLA as incompatible applications for pulp, almost all occurred graft-versus-host disease and failure. Qualifier with phytohemagglutinin, monoclonal antibody and complement or treatment Immunotoxins donor bone marrow, to remove the cause graft-versus-host disease in mature T cells, can significantly improve bone marrow transplantation HLA incompatible success rate. Furthermore, it can also transplanted fetal liver or fetal thymus, but the effects are limited. Lack of ADA - SCID many losers after replacement and irradiated red blood cells have a certain effect. In addition, weekly intramuscular injection of a large dose of a combination of bovine ADA polyethylene glycol (PEG - ADA) also has a good effect . SCID is assisted screening system immunodeficiency disease, humoral and cellular immune function was significantly abnormal. But laboratory tests showed many changes. Usually, IgG, IgM and IgA low, but a small number of patients may have a two-Ig normal. Almost universal no antibody response. In some cases, blood and lymphoid tissue B cells decreased, while the other cases may be more normal. All the cells abnormal immune tests, peripheral blood T cells were significantly reduced; Memory antigen tests and skin tests phytohemagglutinin very poor response. In vitro T cell function tests also obvious anomaly; Mitogen-proliferation absent.