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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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Pulmonology |
| Online Access: | http://dx.doi.org/10.1155/2015/274639 |
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| author | Nihat Demir Erdal Peker İbrahim Ece Sultan Kaba Kemal Ağengin Oğuz Tuncer |
| author_facet | Nihat Demir Erdal Peker İbrahim Ece Sultan Kaba Kemal Ağengin Oğuz Tuncer |
| author_sort | Nihat Demir |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-13ffa4fc26b64a0eb2f208b018a91a6d |
| institution | Kabale University |
| issn | 2090-6846 2090-6854 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pulmonology |
| spelling | doaj-art-13ffa4fc26b64a0eb2f208b018a91a6d2025-08-20T03:54:33ZengWileyCase Reports in Pulmonology2090-68462090-68542015-01-01201510.1155/2015/274639274639A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly SyndromeNihat Demir0Erdal Peker1İbrahim Ece2Sultan Kaba3Kemal Ağengin4Oğuz Tuncer5Department of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyDepartment of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyDepartment of Pediatrics, Division of Cardiology, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyDepartment of Pediatrics, Division of Endocrinology, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyDepartment of Pediatric Surgery, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyDepartment of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, TurkeyShort-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.http://dx.doi.org/10.1155/2015/274639 |
| spellingShingle | Nihat Demir Erdal Peker İbrahim Ece Sultan Kaba Kemal Ağengin Oğuz Tuncer A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome Case Reports in Pulmonology |
| 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 |
| url | http://dx.doi.org/10.1155/2015/274639 |
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