title: Abnormal Muscularization of Intra-acinar Pulmonary Arteries in Two Cases Presenting as Sudden Infant Death.
abstract: Abnormal muscularization of acinar arteries is the hallmark of persistent pulmonary hypertension of newborn (PPHN), an uncommon disease with high rate of morbidity and mortality. PPHN presents with signs of respiratory distress immediately following birth. We herein report 2 cases presenting as a witnessed sudden unexpected death in the late neonatal period, preceded by respiratory deterioration and in whom the presence of abnormal muscularization of the acinar pulmonary arteries was reminiscent of PPHN. The significance of this report is twofold: to increase the awareness among pediatricians and pathologists of this feature that can present in some cases of Sudden Unexpected Death in Infancy/Sudden Infant Death Syndrome, and to highlight the importance of performing a thorough autopsy in order to identify the abnormality.
authors.affiliation: 1 Department of Histopathology, Sheffield Children's Hospital Foundation Trust, Western Bank, Sheffield, UK.|2 Department of Forensic Medicine, Hospital Sultanah Aminah, Johor Bharu, Johore, Malaysia.
authors.forename: Khairul
authors.initials: K
authors.lastname: Zainun
authors.affiliation: 1 Department of Histopathology, Sheffield Children's Hospital Foundation Trust, Western Bank, Sheffield, UK.
authors.forename: Kirsten
authors.initials: K
authors.lastname: Hope
authors.affiliation: 3 Department of Histopathology, Royal Brompton and Harefield Hospitals NHS Foundation Trust, and National Heart and Lung Division, Imperial College, London, UK.
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Nicholson
authors.affiliation: 1 Department of Histopathology, Sheffield Children's Hospital Foundation Trust, Western Bank, Sheffield, UK.
authors.forename: Marta C
authors.initials: MC
authors.lastname: Cohen
mesh_terms: D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009131:Muscle, Smooth, Vascular / Q000473:pathology*; D010547:Persistent Fetal Circulation Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D011651:Pulmonary Artery / Q000473:pathology*; D013398:Sudden Infant Death / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology
keywords: SIDS; acinar arteries; autopsy; persistent pulmonary hypertension of newborn; sudden unexpected death in infancy