Congenital interstitial lung diseases: What the anesthesiologist needs to know

Abstract

Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Some diseases have a very poor prognosis, whereas others have a benign course with appropriate treatment. The current manuscript reviews congenital interstitial lung diseases that typically affect neonates and young children and may be encountered by the pediatric anesthesiologist.

HLA-DRB1*14:54 Is Associated with Pulmonary Alveolar Proteinosis: A Retrospective Real-World Audit

“…We performed a retrospective study to analyze HLA-DRB1 allelic polymorphism in aPAP and sPAP patients. The representativeness of the patients was satisfactory as the sex ratios, clinical manifestations and signs, imaging manifestations on CT, histopathological characteristics, and the frequency of HLA-DRB1*08:03 in aPAP group were in line with the previously reported data [8,14,[19][20][21]. We found similar HLA-DRB1 allele frequencies between both groups from the statistical viewpoint.…”

Congenital pulmonary lymphatic duct hypoplasia in a fetus with hydrops fetalis found at delivery: A case report

“…Cases with only one site of plasma cavity fluid before delivery are atypical fetal edema. Fetal edema is divided into immune and non-immune factors, with a morbidity and mortality rate of 40%–95%, and non-immune edema accounting for 76%–90% of cases [ 6 ]. The etiology of hydrops fetalis is complex, and common causes include structural abnormalities of organs, such as cardiovascular malformations; airway and thoracic pathologies, such as congenital high airway obstruction syndrome and laryngeal atresia; systemic diseases such as infections and anemia; chromosomal disorders/genetic syndromes, genetic metabolic disorders such as lysosomal storage disorders.…”