Histopathologic and Genetic Features of Alveolar Capillary Dysplasia with Atypical Late Presentation and Prolonged Survival.

Edwards, Jonathan J; Murali, Chaya; Pogoriler, Jennifer; Frank, David B; Handler, Stephanie S; Deardorff, Mathew A; Hopper, Rachel K · J Pediatr · 2019

case_report · Level V

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Abstract

Alveolar capillary dysplasia typically presents with neonatal pulmonary hypertension and early mortality. However, there is growing evidence for a subset of disease with atypical late onset and/or prolonged survival. Here, we present the variable clinical, genetic, and pathology findings of 4 such patients.

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