Pulmonary alveolar proteinosis in hereditary and autoimmune forms with 2 cases
Pediatric Emergency Care, cilt.36, sa.8, ss.470-472, 2020 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 36 Sayı: 8
- Basım Tarihi: 2020
- Doi Numarası: 10.1097/pec.0000000000001536
- Dergi Adı: Pediatric Emergency Care
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, CAB Abstracts, CINAHL, EMBASE, MEDLINE
- Sayfa Sayıları: ss.470-472
- Anahtar Kelimeler: autoimmune, hereditary, pulmonary alveolar proteinosis, whole-lung lavage
- Ankara Yıldırım Beyazıt Üniversitesi Adresli: Hayır
Özet
Pulmonary alveolar proteinosis (PAP) is a respiratory pathology characterized by the accumulation and increase of surfactant-derived material in the lungs. In clinical practice, PAP may present as the primary form, which includes autoimmune and hereditary PAP, or as the secondary form. Diffuse alveolar radiopacities on chest x-ray and the crazy-paving pattern on high-resolution computed tomography are important, although not specific findings for PAP. Bronchoalveolar lavage biopsy is a diagnostic method, and whole-lung lavage remains the criterion standard for the treatment of PAP. Evidence is required regarding treatment with exogenous anti-granulocyte/macrophage colony-stimulating factor. Here, we present a 13-year-old male patient with hereditary PAP and a 15-year-old female patient with autoimmune PAP who presented with complaints of easy fatigability and weakness to emphasize the importance of keeping in mind PAP as a differential diagnosis in patients with respiratory failure findings.