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Published in: Pediatric and Developmental Pathology 6/2005

01-12-2005 | CASE REPORT

Alveolar Capillary Dysplasia in an Infant With Trisomy 21

Authors: Bahig M. Shehata, Carlos R. Abramowsky

Published in: Pediatric and Developmental Pathology | Issue 6/2005

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Abstract

We present a case of an infant with Down syndrome (trisomy 21) who was affected by alveolar capillary dysplasia and other complications including endocardial cushion defect, hypothyroidism, and intrauterine growth restriction. The patient was the product of a third pregnancy to a 33-year-old woman with no significant risk factors. The child lived for 3 months, during which he developed intractable dyspnea, hypoxemia, and cardiac dysfunction and he eventually died from septicemia and multiorgan failure. In addition to the facial phenotypic features and cardiac anomalies, the autopsy revealed the characteristic microscopic pulmonary findings of alveolar capillary dysplasia with misalignment of pulmonary veins. This appears to be the first reported case of this anomaly associated with trisomy 21. In addition to the many reasons for pulmonary hypertension that occur in children with trisomy 21, alveolar capillary dysplasia may have to be included in the differential diagnosis although it appears to be a rare association.
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Metadata
Title
Alveolar Capillary Dysplasia in an Infant With Trisomy 21
Authors
Bahig M. Shehata
Carlos R. Abramowsky
Publication date
01-12-2005
Publisher
Springer-Verlag
Published in
Pediatric and Developmental Pathology / Issue 6/2005
Print ISSN: 1093-5266
Electronic ISSN: 1615-5742
DOI
https://doi.org/10.1007/s10024-005-2137-6

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