title: A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome.
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.
authors.affiliation: Department of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: Nihat
authors.initials: N
authors.lastname: Demir
authors.affiliation: Department of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: Erdal
authors.initials: E
authors.lastname: Peker
authors.affiliation: Department of Pediatrics, Division of Cardiology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: İbrahim
authors.initials: İ
authors.lastname: Ece
authors.affiliation: Department of Pediatrics, Division of Endocrinology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: Sultan
authors.initials: S
authors.lastname: Kaba
authors.affiliation: Department of Pediatric Surgery, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: Kemal
authors.initials: K
authors.lastname: Ağengin
authors.affiliation: Department of Pediatrics, Division of Neonatology, Yuzuncu Yil University School of Medicine, 65080 Van, Turkey.
authors.forename: Oğuz
authors.initials: O
authors.lastname: Tuncer