Inhaled nitric oxide enhances oxygenation but not survival in infants with alveolar capillary dysplasia☆,☆☆,★
Section snippets
METHODS
Each infant was born at term after a normal pregnancy and delivery and received care at a different medical center. The mean birth weight was 2780 ± 228 (SD) gm. Four infants were boys. Four infants had other congenital anomalies, including phocomelia (n = l), absent gallbladder (n = l), imperforate anus (n = l), Hirschsprung disease (n = l), and intestinal malrotation (n = 2). All infants died (mean age = 19.2 ± 3.7 days); four died after prolonged courses of extracorporeal life support.
RESULTS
Clinical responses to inhaled NO are detailed for each infant in the Table. After initiation of inhaled NO, the arterial partial pressure of oxygen increased by 207 ± 62 mm Hg for the five infants. The Pa o 2 subsequently decreased despite continued inhaled NO in each infant. In each infant, the concentration of inhaled NO was increased in response to the falling Pao2, and all patients were receiving at least 80 ppm nitric oxide before initiation or resumption of extracorporeal membrane
DISCUSSION
We have described five infants, each with histologic features of ACD confirmed by examination of a lung specimen obtained during a postmortem examination. Each infant had an immediate, dramatic improvement in Pao2 after inhalation of NO. This improvement was not sustained in any infant, and each infant died.
This experience may provide important information about the pathophysiology of ACD, as well as long-term inhalation of NO. Because a prominent pathologic feature of ACD is paucity of
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From the Departments of Pediatrics and Physiology, Children's Hospital and State University of New York at Buffalo, Buffalo, New York; the Departments of Pediatrics and Pathology, Hospital for Sick Children, Toronto, Ontario, Canada; the Department of Pediatrics, University of California, San Francisco, California; the Department of Pediatrics, Royal Alexandra Hospital, Edmonton, Alberta, Canada; and the Department of Pediatrics, Children's Hospital and Ohio State University, Columbus, Ohio.
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Reprint requests: Robin H. Steinhorn, MD, Neonatology, Children's Hospital of Buffalo, 219 Bryant St., Buffalo, NY 14222.
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0022-3476/97/$5.00 + 0 9/21/78222