Abstract
It has been demonstrated that an accumulation of deoxyadenosine (dAd) in adenosine deaminase (ADA) deficient individuals proves to be markedly toxic for lymphoid cells. The immunological function of such children is usually normal at birth, apparently due to the transplacental removal of toxic metabolites during gestation. We studied the possibility that this immunological normality might also be due to a different lymphocyte physiology in neonates. To do so, we cultured mononucleated cells from cord blood of 33 term-born neonates and added to the cell suspensions different concentrations of dAd after inhibiting ADA activity with deoxycoformicine. We observed that in this artificial situation of ADA deficiency dAd significantly inhibits the proliferation of cord blood lymphocytes, both regarding the response to PHA (19,99±5,81% of the response in control cultures) and to ConA (15,73±3,17%). However, the inhibitory effect was significantly smaller (p < 0,01) than that observed in controls (7,79±2,91% with PHA and 8,10±0,78% with ConA). The different response may be due to the different proportion of cord blood cell populations but also to intrinsic differences which might contribute to the delay in the clinical expression of this enzymatic defficiency.
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Romo, A., Lorente, F. & Salazar, V. 108 CONTRIBUTION TO THE STUDY OF SCID PATHOGENESIS DUE TO ADA DEFICIENCY. EFFECT OF 2′ DEOXYADENOSINE COMBINED WITH 2′ DEOXYCOFORMYCINE ON NEONATAL LYMPHOCYTE PROLIFERATION. Pediatr Res 20, 1052 (1986). https://doi.org/10.1203/00006450-198610000-00163
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DOI: https://doi.org/10.1203/00006450-198610000-00163