Maternal γ-cystathionase deficiency: Absence of both teratogenic effects and pregnancy complications, , ,

γ-Cystathionase deficiency (cystathioninemia-cystathioninuria) is a disorder of the transsulfuration pathway characterized by the accumulation of cystathionine in blood and urine. There are probably no clinical consequences. However, maternal γ-cystathionase deficiency has not been reported. We studied 2 pregnancies and the offspring of these pregnancies in a woman with the pyridoxine-nonresponsive form of the disorder. The outcomes were favorable, suggesting that maternal γ-cystathionase deficiency may not be deleterious to the pregnant woman or the fetus. (Am J Obstet Gynecol 1999;181:753-5.)