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

Juan José Menéndez Suso; María Jesús Del Cerro Marín; Paloma Dorao Martínez-Romillo; Carlos Labrandero de Lera; Luis Fernández García-Moya; José Ignacio Rodríguez González

doi:10.1016/j.jpeds.2012.04.044

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

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

Authors

Juan José Menéndez Suso¹, María Jesús Del Cerro Marín, Paloma Dorao Martínez-Romillo, Carlos Labrandero de Lera, Luis Fernández García-Moya, José Ignacio Rodríguez González

Affiliation

¹ Department of Pediatric Intensive Care Medicine, La Paz University Hospital, Madrid, Spain.

PMID: 22658788
DOI: 10.1016/j.jpeds.2012.04.044

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.

MeSH terms

Female
Humans
Hyperglycinemia, Nonketotic / complications
Hyperglycinemia, Nonketotic / diagnosis*
Hyperglycinemia, Nonketotic / mortality
Hyperglycinemia, Nonketotic / physiopathology
Hypertension, Pulmonary / drug therapy
Hypertension, Pulmonary / etiology*
Hypertension, Pulmonary / pathology
Hypertension, Pulmonary / physiopathology
Infant
Lung / pathology
Male
Pulmonary Edema / chemically induced*
Pulmonary Edema / diagnostic imaging
Pulmonary Edema / mortality
Radiography
Respiratory Insufficiency / etiology
Retrospective Studies
Vasodilator Agents / adverse effects*

Substances

Vasodilator Agents