Article
Inherited pulmonary surfactant metabolism disorders in Argentina: Differences between patients with SFTPC and ABCA3 variants.
Pediatric pulmonology - 1 Feb 2023
Balinotti Juan E, Mallie Camila, Maffey Alberto, Colom Alejandro, Epaud Ralph, de Becdelievre Alix, Fanen Pascale, Delestrain Céline, Medín Martín, Teper Alejandro
Abstract excerpt
BACKGROUND: Patients with inherited pulmonary surfactant metabolism disorders have a wide range of clinical outcomes and imaging findings. Response to current anti-inflammatory therapies has been variable and efficacy is unclear. OBJECTIVE: To describe and compare genetic, clinical, histological, and computed tomography (CT) outcomes in a cohort of patients with variants in the genes encoding surfactant protein C...
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