Nonketotic hyperglycinemia presenting as pulmonary hypertensive vascular disease and fatal pulmonary edema in response to pulmonary vasodilator therapy.
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.
Department of Pediatric Intensive Care Medicine, La Paz University Hospital, Madrid, Spain.
Juan José
JJ
Menéndez Suso
María Jesús
MJ
Del Cerro Marín
Paloma
P
Dorao Martínez-Romillo
Carlos
C
Labrandero de Lera
Luis
L
Fernández García-Moya
José Ignacio
JI
Rodríguez González
D005260:Female; D006801:Humans; D020158:Hyperglycinemia, Nonketotic / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D007223:Infant; D008168:Lung / Q000473:pathology; D008297:Male; D011654:Pulmonary Edema / Q000139:chemically induced* / Q000000981:diagnostic imaging / Q000401:mortality; D011859:Radiography; D012131:Respiratory Insufficiency / Q000209:etiology; D012189:Retrospective Studies; D014665:Vasodilator Agents / Q000009:adverse effects*
D014665:Vasodilator Agents