TETRAHYDROBIOPTERIN IN THE TREATMENT OF INFANTILE HYPERTROPHIC PYLORIC-STENOSIS

Evidence is emerging that reduced nitric oxide production may be invol ved in the pathogenesis of hypertrophic pyloric stenosis, Nitric oxide synthase (NOS) requires tetrahydrobiopterin (BH4) for activity, Four infants with hypertrophic pyloric stenosis were treated with oral BH4 (10 mg/kg/day) for 2.5 days. Although plasma total biopterin increased significantly at 3, 27, and 51 h after BH4 administration, there was no effect on the production of plasma cGMP, nitrite, nitrate, or citru lline, Ultrasound investigations before and after the ingestion of BH4 revealed no changes in the hypertrophic pyloric stenosis, We conclude that oral BH4, in the dose utilized in our investigations, does not m odify the cause of hypertrophic pyloric stenosis, presumably because i t did not restore nitric oxide production in the nonadrenergic nonchol inergic nerves of the enteric nervous system. (C) 1997 Academic Press.