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J Pediatr. 2019 Mar 8. pii: S0022-3476(19)30137-4. doi: 10.1016/j.jpeds.2019.01.047. [Epub ahead of print]

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

Author information

1
Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. Electronic address: jonathanedwardsmd@gmail.com.
2
Division of Genetics, The Children's Hospital of Philadelphia, Philadelphia, PA.
3
Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA.
4
Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA; Penn Cardiovascular Institute, University of Pennsylvania, Philadelphia, PA.
5
Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee, WI.
6
Division of Cardiology, The Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA; Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA.

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.

KEYWORDS:

ACD; FOXF1; MPV; case report

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