Erwin W Gelfand, Department of Pediatrics, National Jewish Medical and Research Center, Denver, Colorado, USA

The discovery in 1972 that an inherited disorder of adenine nucleotide metabolism was associated with a specific immunodeficiency disease in humans stimulated a major resurgence of interest in the metabolism and effects of purine nucleosides. The association of disorders of purine nucleoside cata-bolism with severe immunologic dysfunction provides compelling evidence for the role of these metabolic pathways in the biochemical control of lymphocyte differentiation and function. The link between the biochemical explanations for the immunologic disorders, the remarkable tissue specificity of the diseases, and the pathologic effects of these inborn errors of metabolism remains somewhat uncertain.

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