A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmona...

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Autores principales: Demir, Nihat, Peker, Erdal, Ece, İbrahim, Kaba, Sultan, Ağengin, Kemal, Tuncer, Oğuz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4452873/
https://www.ncbi.nlm.nih.gov/pubmed/26078906
http://dx.doi.org/10.1155/2015/274639
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author Demir, Nihat
Peker, Erdal
Ece, İbrahim
Kaba, Sultan
Ağengin, Kemal
Tuncer, Oğuz
author_facet Demir, Nihat
Peker, Erdal
Ece, İbrahim
Kaba, Sultan
Ağengin, Kemal
Tuncer, Oğuz
author_sort Demir, Nihat
collection PubMed
description Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.
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spelling pubmed-44528732015-06-15 A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome Demir, Nihat Peker, Erdal Ece, İbrahim Kaba, Sultan Ağengin, Kemal Tuncer, Oğuz Case Rep Pulmonol Case Report Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil. Hindawi Publishing Corporation 2015 2015-05-20 /pmc/articles/PMC4452873/ /pubmed/26078906 http://dx.doi.org/10.1155/2015/274639 Text en Copyright © 2015 Nihat Demir et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Demir, Nihat
Peker, Erdal
Ece, İbrahim
Kaba, Sultan
Ağengin, Kemal
Tuncer, Oğuz
A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title_full A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title_fullStr A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title_full_unstemmed A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title_short A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome
title_sort rare cause of persistent pulmonary hypertension resistant to therapy in the newborn: short-rib polydactyly syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4452873/
https://www.ncbi.nlm.nih.gov/pubmed/26078906
http://dx.doi.org/10.1155/2015/274639
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