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Case Reports in Pulmonology
Volume 2015 (2015), Article ID 274639, 3 pages
http://dx.doi.org/10.1155/2015/274639
Case Report

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

1Department of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey
2Department of Pediatrics, Division of Cardiology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey
3Department of Pediatrics, Division of Endocrinology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey
4Department of Pediatric Surgery, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey

Received 9 January 2015; Revised 24 March 2015; Accepted 20 April 2015

Academic Editor: Kentaro Watanabe

Copyright © 2015 Nihat Demir et al. 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.

Abstract

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.