A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome. (20th May 2015)

• Record Type: Journal Article
• Authors: Demir, Nihat; Peker, Erdal; Ece, İbrahim; Kaba, Sultan; Ağengin, Kemal; Tuncer, Oğuz
• Other Names: Watanabe Kentaro Academic Editor.
• Is Part Of: Case reports in pulmonology. Volume 2015(2015)
• Publication Date: 2015-05-20
• Subjects: Lungs -- Diseases -- Periodicals; Respiratory organs -- Diseases -- Periodicals; Respiratory infections -- Periodicals; Lung Diseases; Respiratory Tract Diseases; Respiratory Tract Infections; Lungs -- Diseases; Respiratory infections; Respiratory organs -- Diseases; Periodicals; Case Reports; Electronic journals; Periodicals; 616.2
• DOI: 10.1155/2015/274639
• Languages: English
• ISSNs: 2090-6846

Abstract

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.