Journal
MOLECULAR GENETICS AND METABOLISM
Volume 98, Issue 1-2, Pages 195-197Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.ymgme.2009.06.006
Keywords
Argininosuccinic aciduria; Urea cycle disorders; Nitric oxide; Hypertension
Funding
- Baylor College of Medicine General Clinical Research Center [RR00188]
- Mental Retardation and Developmental Disabilities Research Cente [HD024064]
- Child Health Research Center [HD041648]
- NIH [DK54450, RR019453]
- National Urea Cycle Disorders Foundation Fellowship
- UCD RDCRN O'Malley Foundation Fellowship
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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) production and hypertension lead us to hypothesize that the deficiency of endogenously synthesized arginine Caused by ASL deficiency is responsible for the increased blood pressure. (C) 2009 Elsevier Inc. All rights reserved.
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