Histopathologic features of alveolar capillary dysplasia with misalignment of pulmonary veins with atypical clinical presentation

2021 ◽  
Vol 50 ◽  
pp. 107289
Author(s):  
Murad Alturkustani ◽  
Duo Li ◽  
Joshua T. Byers ◽  
Linda Szymanski ◽  
David M. Parham ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Atypical Clinical Presentation ◽  
Alveolar Capillary ◽  
Histopathologic Features

Early prenatal diagnosis of alveolar capillary dysplasia with misalignment of pulmonary veins due to a 16q24.1 deletion

2021 ◽  
Author(s):  
Chloé Puisney‐Dakhli ◽  
Francesca Gubana ◽  
François Petit ◽  
Hanane Bouchghoul ◽  
Valérie Gautier ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary

237: CONGENITAL HEART DISEASE AND ALVEOLAR CAPILLARY DYSPLASIA WITH MISALIGNMENT OF PULMONARY VEINS

2018 ◽  
Vol 46 (1) ◽  
pp. 101-101
Author(s):  
Katharine Robb ◽  
Sook-kyung Kwon ◽  
Veerajalandhar Allareddy ◽  
Aditya Badheka
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary

scholarly journals Alveolar capillary dysplasia with misalignment of the pulmonary veins: clinical, histological, and genetic aspects

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401879514 ◽  
Author(s):  
Evelien Slot ◽  
Gabriëla Edel ◽  
Ernest Cutz ◽  
Arno van Heijst ◽  
Martin Post ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Molecular Mechanism ◽  
Pulmonary Veins ◽  
Clinical Aspects ◽  
Lung Disorder ◽  
Genetic Studies ◽  
Ongoing Research ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary ◽  
Severe Lung

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal disorder mainly involving the vascular development of the lungs. Since its first description, significant achievements in research have led to a better understanding of the underlying molecular mechanism of ACD/MPV and genetic studies have identified associations with genomic alterations in the locus of the transcription factor FOXF1. This in turn has increased the awareness among clinicians resulting in over 200 cases reported so far, including genotyping of patients in most recent reports. Collectively, this promoted a better stratification of the patient group, leading to new perspectives in research on the pathogenesis. Here, we provide an overview of the clinical aspects of ACD/MPV, including guidance for clinicians, and review the ongoing research into the complex molecular mechanism causing this severe lung disorder.

scholarly journals Novel FOXF1 Deep Intronic Deletion Causes Lethal Lung Developmental Disorder, Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins

2013 ◽  
Vol 34 (11) ◽  
pp. 1467-1471 ◽  
Author(s):  
Przemyslaw Szafranski ◽  
Yaping Yang ◽  
Melissa U. Nelson ◽  
Matthew J. Bizzarro ◽  
Raffaella A. Morotti ◽  
...  
Keyword(s):  
Developmental Disorder ◽  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary

Congenital Misalignment of Pulmonary Veins with Alveolar Capillary Dysplasia Causing Persistent Neonatal Pulmonary Hypertension: Report of Two Affected Siblings

2000 ◽  
Vol 3 (3) ◽  
pp. 271-276 ◽  
Author(s):  
Carmen Gutierrez ◽  
Angeles Rodriguez ◽  
Sylvia Palenzuela ◽  
Carolina Forteza ◽  
Jose L. Diaz Rossello
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Veins ◽  
Duodenal Atresia ◽  
Persistent Pulmonary Hypertension ◽  
Pulmonary Vessels ◽  
Alveolar Capillary Dysplasia ◽  
Pulmonary Abnormality ◽  
Alveolar Capillary

Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.

A late presenter and long-term survivor of alveolar capillary dysplasia with misalignment of the pulmonary veins

2015 ◽  
Vol 174 (8) ◽  
pp. 1123-1126 ◽  
Author(s):  
Yukie Ito ◽  
Takuma Akimoto ◽  
Kazutoshi Cho ◽  
Masafumi Yamada ◽  
Mishie Tanino ◽  
...  
Keyword(s):  
Pulmonary Veins ◽  
Late Presenter ◽  
Alveolar Capillary Dysplasia ◽  
Term Survivor ◽  
Alveolar Capillary

scholarly journals Two deletions overlapping a distantFOXF1enhancer unravel the role of lncRNALINC01081in etiology of alveolar capillary dysplasia with misalignment of pulmonary veins

2014 ◽  
Vol 164 (8) ◽  
pp. 2013-2019 ◽  
Author(s):  
Przemyslaw Szafranski ◽  
Avinash V. Dharmadhikari ◽  
Jennifer A. Wambach ◽  
Chris T. Towe ◽  
Frances V. White ◽  
...  
Keyword(s):  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary

Inversion upstream ofFOXF1in a case of lethal alveolar capillary dysplasia with misalignment of pulmonary veins

2013 ◽  
Vol 161 (4) ◽  
pp. 764-770 ◽  
Author(s):  
Toshima Parris ◽  
Ali Moussavi Nik ◽  
Sailesh Kotecha ◽  
Claire Langston ◽  
Khalil Helou ◽  
...  
Keyword(s):  
Pulmonary Veins ◽  
Alveolar Capillary Dysplasia ◽  
Alveolar Capillary
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