Infantile pulmonary capillary haemangiomatosis: a lethal form of pulmonary hypertension
| Autor: | Ethna Phelan, Paul McNally, D. Hunter Best, Maureen J. O'Sullivan, Kelli Sumner, Colin J. McMahon, Eimear McGovern |
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| Rok vydání: | 2015 |
| Předmět: |
Male
medicine.medical_specialty Hypertension Pulmonary Occlusive disease 030204 cardiovascular system & hematology 03 medical and health sciences Pulmonary capillary haemangiomatosis Fatal Outcome 0302 clinical medicine Internal medicine medicine Humans Lung business.industry Infant Newborn Infant General Medicine medicine.disease Pulmonary hypertension Vascular Neoplasms Capillaries medicine.anatomical_structure 030228 respiratory system Pediatrics Perinatology and Child Health Cardiology Etiology Female Pulmonary Veno-Occlusive Disease Hemangioma Cardiology and Cardiovascular Medicine business Pulmonary vasodilators |
| Zdroj: | Cardiology in the Young. 26:663-668 |
| ISSN: | 1467-1107 1047-9511 |
| Popis: | We describe the cases of two children who both presented in infancy with recurrent severe pulmonary hypertensive crises. Exhaustive clinical work-up failed to identify an underlying aetiology. The patients had no clinical response to steroids, immunoglobulins, or pulmonary vasodilators. Post-mortem examination revealed extensive invasive pulmonary capillary haemangiomatosis. There was no evidence of pulmonary venous occlusive disease. Given the lethal nature of this condition, early consideration of referral to a lung transplant centre should be considered in selected patients. |
| Databáze: | OpenAIRE |
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