PREGNANCY AND ARGININOSUCCINIC ACIDURIA

We present the outcome of a pregnancy in a woman with mild argininosuc cinic lyase deficiency to add to the collective experience of the mate rnal and fetal effects of urea cycle defects. In females affected with argininosuccinic lyase deficiency, careful clinical and biochemical m onitoring of pregnancy will minimize the risk of metabolic decompensat ion in the perinatal period. Furthermore, it would appear that arginin osuccinate is not teratogenic to the development of the human fetus.