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Biomedical subjects

R Buckley

Publications and source records attributed to R Buckley.

51 records · Page 3Linked to original sources
PubMed1986

Appearance of multiple benign paraproteins during early engraftment of soy lectin T cell-depleted haploidentical bone marrow cells in severe combined immunodeficiency.

Recent advances in the prevention of graft-versus-host disease through postthymic T-cell depletion have allowed the use of haploidentical bone marrow cells for immunologic reconstitution of severe combined immunodeficiency disease. We report a male infant with severe combined immunodeficiency (with normal adenosine deaminase) who developed two IgG kappa and one IgA lambda paraproteins 7 weeks following the administration of 1.4 X 10(9) maternal bone marrow cells depleted of postthymic T cells by soy lectin agglutination and sheep erythrocyte rosetting. Serum IgG rose from 128 to 820 mg/dl, and IgA from 0 to 2400 mg/dl, peaking at 10 weeks postgrafting. By 14 weeks posttransplantation T-cell numbers and function had risen to normal (all dividing T cells had the donor karyotype) and paraprotein concentrations began to decline. These observations strongly suggest that the later-appearing T cells regulated the B-cell clones from which the paraproteins were derived. Failure of such function to appear could account for the increased incidence of B-cell lymphomas in severe combined immunodeficiency.

B-Lymphocytes↗
PubMed1986

Variability in B cell maturation and differentiation in X-linked agammaglobulinemia.

Among seven males with X-linked agammaglobulinemia in an extended pedigree, serum immunoglobulins and antibodies were extremely low in all but one who had a normal IgA (78 mg/dl) and tetanus antibodies (1:19,683). Following bacteriophage phi X 174 immunizations, the oldest failed to clear phage and had no primary or secondary antibody responses. The youngest had normal phage clearance, low primary and secondary antibody responses, and no amplification or switching to IgG. The other four affected had normal or slightly delayed phage clearance, low primary and secondary responses, but some amplification and switching from IgM to IgG which increased with age. Normal percentages of surface immunoglobulin positive cells were present in the two youngest patients, but all seven affected had very low percentages of cells reacting with monoclonal antibodies to B cell surface antigens. Immunoglobulin production by cultured blood B cells was very low and not increased by pokeweed mitogen. However, a majority of Epstein-Barr virus (EBV)-transformed lymphoblastoid cells derived from the blood of four of the patients bore IgD and IgM and reacted with all of the monoclonal antibodies to B cell antigens. Culture supernatants from those lines contained significant quantities of IgM and lesser amounts of IgG and IgA. The studies presented here provide further support for the hypothesis that the primary abnormality in X-linked agammaglobulinemia affects B cells at more than one stage of development rather than just at the level of the pre-B cell.

Adolescent↗
PubMed1979

In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.

The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined immune dysfunction from which they suffer. Earlier studies from this laboratory showed that 2'-deoxyadenosine causes the irreversible inactivation of the enzyme S-adenosylhomocysteine hydrolase by an active site-directed, "suicide-like" process. In this communication we have demonstrated similar inactivation of S-adenosylhomocysteine hydrolase in hemolysate and in intact erythrocytes, as well as a striking deficiency of S-adenosylhomocysteine hydrolase activity in the erythrocytes of three adenosine deaminase-deficient patients. In vivo suicide-like inactivation of S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine may contribute to the cytotoxicity of 2'-deoxyadenosine and to the immune dysfunction in adenosine deaminase deficiency.

Adenosine Deaminase↗
PubMed1977

Latent form of Scrapie virus: a new factor in slow-virus disease.

Scrapie is an unusual slow-virus disease of sheep which is very much like kuru and Creutzfeldt-Jakob disease, both fatal, slow neuological diseases of man. In mice, scrapie usually has an incubation period of about 6 months. Intraperitoneal inoculation of virus particles into newborn mice caused no disease, and there was no detectable virus replication for 1 year, but high titers of scrapie were present in the spleen and brain at 18 months. Virus replication occurred in mice injected from 4 days after birth by all inoculation routes, wheter or not they were injected with scrapie virus on day 0. The results suggest that scrapie virus replicates peripherally only in thymocytes, which are not present in mice until a few days after birth. The latent state suggests that the comparable human diseases could appear in later life as a result of perinatal infection. In some respects these diseases resemble premature senility.

Aging↗
PubMedDate not supplied

Mycobacterium fortuitum infection occurring after a punch biopsy procedure.

Mycobacterium fortuitum is a rapidly growing atypical mycobacterium frequently reported as a postsurgical wound complication from a major surgical procedure. We present a unique case of M. fortuitum infection occurring in a 4-year-old boy after a minor punch biopsy surgical procedure. As far as we know there has been no published case of M. fortuitum occurring after a punch biopsy procedure.

Biopsy↗