1/3. Dysequilibrium/ataxic diplegia with immunodeficiency.A girl with purine nucleoside phosphorylase (PNP) deficiency is described. The nature of the motor disorder is similar to other children since found to have PNP deficiency. It is suggested that the diagnosis be considered in any child with unexplained dysequilibrium/ataxic diplegia. Other previously unreported features are intracytoplasmic neutrophil inclusion bodies and an improvement in the neutropenia after intravenous immunoglobulin.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/3. Ataxia-telangiectasia. X,14 translocation, progressive deterioration of lymphocyte numbers and function, and abnormal in vitro immunoglobulin production.Three children with ataxia-telangiectasia have been followed up since their early childhood. Sequential immunological, biochemical and chromosome studies have been performed over the last 7 years. All the children showed progressive cerebellar ataxia and inexorable neurological deterioration. Further evidence for the progressive nature of this condition is the fall in lymphocyte counts, deterioration of lymphocyte transformation responses to mitogens, and an increase in chromosomal translocations and breakage. Elevated serum alpha-fetoprotein levels are a highly characteristic and useful diagnostic finding in this condition. Two of the patients had an X,14 translocation. in vitro studies of immunoglobulin synthesis suggest an intrinsic defect in B-cell synthesis as well as decreased helper T-cell activity. In spite of moderately severe and progressive abnormalities in the immune system, sinopulmonary infections have not been prominent in our patients.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/3. Analysis of lymphocyte subsets in a renal allograft recipient infected with human immunodeficiency virus.OBJECTIVE: To study the change in lymphocyte subsets of a renal allograft recipient who acquired human immunodeficiency virus (hiv) infection. PATIENT AND methods: A 53-year-old Japanese man who acquired hiv infection at the time of renal transplantation and died from opportunistic infection 3 years later was studied. lymphocyte subsets were examined serially throughout his clinical course. RESULTS: The numbers of CD4 positive cells were severely decreased but those of CD8 positive cells were stable; the CD 4/8 ratio was extremely low. The numbers of B cells were also decreased but production of immunoglobulin was increased. In spite of this immunosuppressive state, graft rejection was noted when the patient's immunosuppressive medication was reduced. CONCLUSION: The lymphocyte subsets of renal transplant patients with hiv infection should be closely observed, as should the nature and timing of opportunistic infection in relation to transplantation, and the administration of immunosuppressive medication.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |