917 severe combined immune deficiency disease – a t-cell disorder

Select a language for the TTS:
UK English Female
UK English Male
US English Female
US English Male
Australian Female
Australian Male
Language selected: (auto detect) - EN

Nature

Play all audios:

ABSTRACT Severe combined immune deficiency disease (SCID) represents a spectrum of disorders characterized by the inability to manifest normal cell-mediated and humoral immunity. Recognition

and grouping of these variants of SCID are important in order to provide guidelines for investigation and to evaluate and select appropriate therapeutic modalities. In contrast to earlier

suggestions that these patients manifest abnormalities of lymphoid stem cells, our studies have demonstrated T and B-cell precursor cells in the majority of SCID patients. We have used in

vitro assays for monitoring the induction of T-cell differentiation and have characterized the responsiveness of patient precursor T-cells to specific induction regimens including contact

with thymic epithelium, epithelium conditioned medium, thymosin and theophylline. In this way, we have identified different groups of patients with an arrest of T-cell differentiation at

different stages of maturation. In most of the patients with normal adenosine deaminase, the combined immune deficiency could be shown to reflect the failure of normal T-cell differentiation

and consequent failure of T-cell dependent maturation of B lymphocytes to an antibody-secreting stage, rather than intrinsic abnormalities of the B cells themselves. These data emphasize

the thymic dependence of specific B-cell differentiation in man and demonstrate the functional integrity of B lymphocytes in many patients with SCID. ARTICLE PDF AUTHOR INFORMATION AUTHORS

AND AFFILIATIONS * Division of Immunology, Hospital for Sick Children, Toronto, Canada Erwin W Gelfand & Hans-Michael Dosch Authors * Erwin W Gelfand View author publications You can

also search for this author inPubMed Google Scholar * Hans-Michael Dosch View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints

and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Gelfand, E., Dosch, HM. 917 SEVERE COMBINED IMMUNE DEFICIENCY DISEASE – A T-CELL DISORDER. _Pediatr Res_ 15 (Suppl 4), 595 (1981).

https://doi.org/10.1203/00006450-198104001-00942 Download citation * Issue Date: 01 April 1981 * DOI: https://doi.org/10.1203/00006450-198104001-00942 SHARE THIS ARTICLE Anyone you share the

following link with will be able to read this content: Get shareable link Sorry, a shareable link is not currently available for this article. Copy to clipboard Provided by the Springer

Nature SharedIt content-sharing initiative