About: Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation

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About: Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation Goto Sponge NotDistinct Permalink

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Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
title	Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation
Creator	Eghtesady, Pirooz Hamvas, Aaron Sen, Partha Stankiewicz, Pawel Szafranski, Przemyslaw Sweet, Stuart Grady, R Sessions Cole, F Towe, Christopher Wambach, Jennifer Wegner, Daniel White, Frances
source	Elsevier; Medline; PMC
abstract	Objective To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation. Study design We reviewed clinical history, diagnostic studies, explant histology, genetic sequence results, and post-transplant course for 6 infants with atypical ACDMPV who underwent bilateral lung transplantation at St. Louis Children's Hospital. We compared their histology with infants with classic ACDMPV and compared their outcomes with infants transplanted for other indications. Results In contrast with neonates with classic ACDPMV who present with severe hypoxemia and refractory pulmonary hypertension within hours of birth, none of the infants with atypical ACDMPV presented with progressive neonatal respiratory failure. Three infants had mild neonatal respiratory distress and received nasal cannula oxygen. Three other infants had no respiratory symptoms at birth and presented with hypoxemia and pulmonary hypertension at 2-3 months of age. Bilateral lung transplantation was performed at 4-20 months of age. Unlike in classic ACDMPV, histopathologic findings were not distributed uniformly and were not diffuse. Three subjects had apparent nonmosaic genetic defects involving FOXF1. Two infants had extrapulmonary anomalies (posterior urethral valves, inguinal hernia). Three transplanted children are alive at 5-16 years of age, similar to outcomes for infants transplanted for other indications. Lung explants from infants with atypical ACDMPV demonstrated diagnostic but nonuniform histopathologic findings. Conclusions The 1- and 5-year survival rates for infants with atypical ACDMPV are similar to infants transplanted for other indications. Given the clinical and histopathologic spectra, ACDMPV should be considered in infants with hypoxemia and pulmonary hypertension, even beyond the newborn period.
has issue date	2018-03-31(xsd:dateTime)
bibo:doi	10.1016/j.jpeds.2017.10.026
bibo:pmid	29198536
has license	els-covid
sha1sum (hex)	9ae9f0bc8676b51929df1e3d9bb9ca1dc9bde8b9
schema:url	https://doi.org/10.1016/j.jpeds.2017.10.026
resource representing a document's title	Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation
has PubMed Central identifier	PMC5826830
has PubMed identifier	29198536
schema:publication	The Journal of Pediatrics
resource representing a document's body	covid:9ae9f0bc8676b51929df1e3d9bb9ca1dc9bde8b9#body_text
is schema:about of	named entity 'PRESENTATIONS' named entity 'ALVEOLAR CAPILLARY DYSPLASIA' named entity 'ATYPICAL' named entity 'DISEASE COURSE' named entity 'BILATERAL LUNG TRANSPLANTATION' named entity 'OBJECTIVE' named entity 'PRESENTATIONS' named entity 'ATYPICAL' named entity 'BILATERAL LUNG TRANSPLANTATION' named entity 'INFANTS' named entity 'HISTOPATHOLOGY' named entity 'TO DESCRIBE' named entity 'ALVEOLAR CAPILLARY DYSPLASIA' named entity 'INFANTS' named entity 'OUTCOMES' covid:arg/9ae9f0bc8676b51929df1e3d9bb9ca1dc9bde8b9 named entity 'describe' named entity 'infants' named entity 'presentations' named entity 'histopathology' named entity 'Alveolar' named entity 'Pulmonary Veins' named entity 'Dysplasia' named entity 'SFTPB' named entity 'pulmonary veins' named entity 'cardiac catheterization' named entity 'ANNOVAR' named entity 'hypoxemia' named entity 'FOXF1' named entity 'Medial wall' named entity 'nasal cannula' named entity 'FOXF1' named entity 'Lobular' named entity 'deletion mapping' named entity 'abdominal distension' named entity 'nitric oxide' named entity 'diffusing capacity' named entity 'hypoxemia' named entity 'vasodilators' named entity 'point mutations' named entity 'nitric oxide' named entity 'pulmonary hypertension' named entity 'gastroesophageal reflux' named entity 'lung transplant' named entity 'nasal cannula' named entity 'hypoxemic' named entity 'developmental mechanisms' named entity 'idiopathic' named entity 'chest CT' named entity 'respiratory symptoms' named entity 'ground glass opacities' named entity 'lung transplantation' named entity 'congenital anomalies' named entity 'spirometry' named entity 'airflow obstruction' named entity 'forced vital capacity' named entity 'supplemental oxygen' named entity 'surfactant deficiency' named entity 'proximal' named entity 'alveolar capillaries' named entity 'respiratory symptoms' named entity 'Neonates' named entity 'histology' named entity 'lung transplant' named entity 'pulmonary hypertension' named entity 'oxygen' named entity 'pulmonary capillaries' named entity 'bronchodilator' named entity 'oxygen' named entity 'arterioles'

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