Congenital Misalignment of Pulmonary Vessels and Alveolar Capillary Dysplasia: How to Manage a Neonatal Irreversible Lung Disease?
| Autor: | M. Somaschini, A. Colombo, Silvia Riva, D Chinaglia, Cristina Bellan |
|---|---|
| Rok vydání: | 2000 |
| Předmět: |
Alveolar capillary dysplasia
Hemoptysis Pulmonary Circulation medicine.medical_specialty Pathology Biopsy Hypertension Pulmonary medicine.medical_treatment Autopsy Lung biopsy Pulmonary Artery Nitric Oxide Hypoxemia Nitric oxide Arteriovenous Malformations High-Frequency Jet Ventilation chemistry.chemical_compound Fatal Outcome Internal medicine Administration Inhalation medicine Extracorporeal membrane oxygenation Humans Respiratory Distress Syndrome Newborn Respiratory distress business.industry Infant Newborn Obstetrics and Gynecology respiratory system medicine.disease Bronchodilator Agents Capillaries Pulmonary Alveoli chemistry Pulmonary Veins Pediatrics Perinatology and Child Health Cardiology Female Pulmonary hemorrhage medicine.symptom business |
| Zdroj: | Journal of Perinatology. 20:189-192 |
| ISSN: | 1476-5543 0743-8346 |
| DOI: | 10.1038/sj.jp.7200335 |
| Popis: | Congenital misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia is a rare condition consisting of anomalous veins in bronchovascular bundles, a decreased number of alveolar capillaries, and increased muscularization of pulmonary arterioles. In the literature, infants reported as having such a malformation developed respiratory distress with persistent pulmonary hypertension and ultimately died. We report the case of an infant with MPV and alveolar capillary dysplasia who was unresponsive to maximal cardiorespiratory support, including high-frequency oscillatory ventilation and inhaled nitric oxide; the infant died of pulmonary hemorrhage after 19 days, during venoarterial extracorporeal membrane oxygenation bypass. We conclude that the diagnosis of MPV and alveolar capillary dysplasia should be considered during autopsy of infants who have died of irreversible persistent pulmonary hypertension. If a lung biopsy in infants with prolonged refractory hypoxemia confirms such diagnosis before death, expensive and invasive treatments such as extracorporeal membrane oxygenation could be avoided. |
| Databáze: | OpenAIRE |
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