Systemic hypertension in two patients with ASL deficiency: a result of nitric oxide deficiency?
N Brunetti-Pierri, A Erez, O Shchelochkov… - Molecular genetics and …, 2009 - Elsevier
Argininosuccinic aciduria (ASA) is an inborn error of ureagenesis which if untreated leads to
hyperammonemia, accumulation of argininosuccinic acid and arginine depletion. The
presence of high blood pressure in patients with ASA has been reported so far as transient
in one newborn. We describe the first two patients, one child and one young adult, with ASA
and persistent systemic hypertension. Extensive evaluation of both patients excluded
secondary causes of systemic hypertension. The intriguing link between nitric oxide (NO) …
hyperammonemia, accumulation of argininosuccinic acid and arginine depletion. The
presence of high blood pressure in patients with ASA has been reported so far as transient
in one newborn. We describe the first two patients, one child and one young adult, with ASA
and persistent systemic hypertension. Extensive evaluation of both patients excluded
secondary causes of systemic hypertension. The intriguing link between nitric oxide (NO) …
