Arginine Supplements May Help Treat Pulmonary Hypertension in Sickle Cell Disease

Laurie Barclay, MD

June 30, 2003

June 30, 2003 — Arginine supplements may be a new treatment for pulmonary hypertension in sickle cell disease patients, according to the results of a small trial published in the July 1 issue of the American Journal of Respiratory and Critical Care Medicine.

"Pulmonary hypertension is a life-threatening complication of sickle cell disease," write Claudia R. Morris, MD, from Children's Hospital Oakland in California, and colleagues. "L-Arginine is the nitrogen donor for synthesis of nitric oxide, a potent vasodilator that is deficient during times of sickle cell crisis. This deficiency may play a role in pulmonary hypertension."

Because arginase hydrolyzes arginine to ornithine and urea, it may compete with nitric oxide synthase, leading to decreased nitric oxide production. Inhaled nitric oxide therapy has improved pulmonary hypertension in sickle cell disease, and other studies suggest that arginine therapy may be helpful in primary and secondary pulmonary hypertension.

After five days of oral arginine therapy, 10 patients with pulmonary hypertension and sickle cell disease had a 15.2% mean reduction in estimated pulmonary artery systolic pressure (63.9 ± 13 to 54.2 ± 12 mm Hg; P = .002). Arginase activity nearly doubled ( P = .07) in patients with pulmonary hypertension, which may limit arginine bioavailability. The authors also suggest that elevated arginase activity may contribute to the pathogenesis of pulmonary hypertension in sickle cell disease.

Study limitations include lack of cardiac catheterization, uncontrolled design, and small sample size.

"With limited treatment options and a high mortality rate for patients with sickle cell disease who develop pulmonary hypertension, arginine is a promising new therapy that warrants further investigation," the authors write. "Arginine is a well tolerated, nontoxic nutritional supplement with few side effects."

Am J Respir Crit Care Med. 2003;168:63-69

Reviewed by Gary D. Vogin, MD

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