Abstract
The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.
© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.
MeSH terms
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Administration, Inhalation
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Bosentan
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Bronchodilator Agents / administration & dosage
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Dose-Response Relationship, Drug
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Drug Therapy, Combination
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Epoprostenol / administration & dosage
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Epoprostenol / analogs & derivatives*
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Epoprostenol / therapeutic use*
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Fatal Outcome
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Female
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Follow-Up Studies
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Humans
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Infant, Newborn
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Nitric Oxide / administration & dosage*
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Persistent Fetal Circulation Syndrome / diagnosis
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Persistent Fetal Circulation Syndrome / drug therapy*
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Persistent Fetal Circulation Syndrome / physiopathology
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Piperazines / administration & dosage
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Piperazines / therapeutic use*
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Platelet Aggregation Inhibitors / administration & dosage
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Platelet Aggregation Inhibitors / therapeutic use
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Pulmonary Alveoli / abnormalities*
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Pulmonary Alveoli / physiopathology
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Purines / administration & dosage
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Purines / therapeutic use
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Sildenafil Citrate
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Sulfonamides / administration & dosage
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Sulfonamides / therapeutic use*
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Sulfones / administration & dosage
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Sulfones / therapeutic use*
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Time Factors
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Vasodilator Agents / administration & dosage
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Vasodilator Agents / therapeutic use
Substances
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Bronchodilator Agents
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Piperazines
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Platelet Aggregation Inhibitors
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Purines
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Sulfonamides
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Sulfones
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Vasodilator Agents
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Nitric Oxide
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beraprost
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Sildenafil Citrate
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Epoprostenol
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Bosentan
Supplementary concepts
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Alveolar capillary dysplasia