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. 2012 Sep;161(3):557-9.
doi: 10.1016/j.jpeds.2012.04.044. Epub 2012 Jun 1.

Nonketotic hyperglycinemia presenting as pulmonary hypertensive vascular disease and fatal pulmonary edema in response to pulmonary vasodilator therapy

Juan José Menéndez Suso  1 , María Jesús Del Cerro Marín, Paloma Dorao Martínez-Romillo, Carlos Labrandero de Lera, Luis Fernández García-Moya, José Ignacio Rodríguez González
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Nonketotic hyperglycinemia presenting as pulmonary hypertensive vascular disease and fatal pulmonary edema in response to pulmonary vasodilator therapy

Juan José Menéndez Suso et al. J Pediatr. 2012 Sep.

Abstract

The association of pulmonary hypertensive vascular disease with nonketotic hyperglycinemia is rare. We describe 5 infants diagnosed with nonketotic hyperglycinemia, in whom pulmonary hypertensive vascular disease was the main presenting feature, and who developed severe pulmonary edema in response to pulmonary vasodilators.

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