Pulmonary Hypertension in Childhood
| Autor: | Pelin Asfuroglu, Deniz Oğuz, Tugba Sismanlar Eyuboglu, Ayse Tana Aslan, Zeynep Reyhan Onay, Sedef Tunaoğlu, Tugba Ramasli Gursoy, Semiha Tokgoz, Serdar Kula, Fatma Incedere |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Down syndrome biology business.industry Bronchiolitis obliterans Furosemide Angiotensin-converting enzyme Disease medicine.disease Gastroenterology Cystic fibrosis Pulmonary hypertension Bronchopulmonary dysplasia Internal medicine medicine biology.protein business medicine.drug |
| Zdroj: | Paediatric bronchology. |
| DOI: | 10.1183/13993003.congress-2020.3519 |
| Popis: | Objective: Pulmonary hypertension (PH) is a rare condition associated with diverse cardiac, pulmonary, and systemic diseases in childhood. We aimed to detect the frequency of PH due to pulmonary diseases and clinical features of them in a pediatric pulmonology department. Method: Between 2008-2019, all patients with PH who were followed in pediatric pulmonology department were evaluated in terms of age of diagnosis, cause of PH, treatments and outcome. Results: A total of 41 patients were followed up with PH, 53% of them were female. Mean age of diagnosis was 4.07±5.1 years. Mean age of diagnosis was 3.14±4.85 years in patients with cardiac or pulmonary anomaly and 6.01±5.28 years in patients with idiopathic PH (p>0.05). Of these, 19 patients (46%) had cardiac, 20% had pulmonary disease related with PH, 3% had genetic disorder and 31% had idiopathic PH. Seven of the patients with cardiac anomaly had accompanying Down syndrome. Four of 19 patients with cardiac anomaly had accompanying pulmonary disorders. In pulmonary disease group, 6 patients had bronchopulmonary dysplasia, one had bronchiolitis obliterans, one had cystic fibrosis, and one patient had surfactant protein C deficiency. Patients were treated with endothelin receptor antagonists and/or phosphodiesterase inhibitor and/or furosemide and/or digital and/or angiotensin converting enzyme blockers and 11 of all them needed oxygen supplementation. In the follow up four patients were died. Method: PH is a serious disease in childhood with high mortality. Cardiac, pulmonary, genetic and systemic diseases should be investigated in patients with PH. Patients may have more than one systemic cause of PH even they have a cardiac anomaly related with PH. |
| Databáze: | OpenAIRE |
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