Am J Perinatol 2005; 22(3): 121-125
DOI: 10.1055/s-2005-863787
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Idiopathic Infantile Arterial Calcification and Persistent Pulmonary Hypertension

Juliet Farquhar1 , Nawal Makhseed4 , Michael Sargent3 , Glenn Taylor2 , Horacio Osiovich1
  • 1Division of Neonatology, Children's and Women's Health Centre of British Columbia, University of British Columbia, Vancouver, British Columbia, Canada
  • 2Department of Pathology, Children's and Women's Health Centre of British Columbia, University of British Columbia, Vancouver, British Columbia, Canada
  • 3Department of Radiology, Children's and Women's Health Centre of British Columbia, University of British Columbia, Vancouver, British Columbia, Canada
  • 4Metabolic Investigative Facility, Children's and Women's Health Centre of British Columbia, University of British Columbia, Vancouver, British Columbia, Canada
Further Information

Publication History

Publication Date:
18 March 2005 (online)

ABSTRACT

Idiopathic infantile arterial calcification is a rare and usually fatal disorder, which is characterized by widespread vaso-occlusive disease. Presentation is variable, but refractory hypertension is typical, with symptoms of cardiorespiratory failure. Some present in utero with evidence of fetal compromise, which may manifest as hydrops fetalis and premature delivery or stillbirth. Presentation otherwise is usually in the neonatal period with 85% of cases being fatal within the first 6 months. Coronary artery involvement is a poor prognostic feature. The pathophysiology of this disorder is well documented but the underlying etiology remains unknown. An autosomal recessive metabolic disorder is thought likely and this is supported in this case. Pulmonary hypertension has not previously been reported to be a primary feature of this disease. Here we describe such a case in which a newborn underwent extracorporeal membrane oxygenation, and during this time a diagnosis of idiopathic arterial calcification was made.

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Horacio OsiovichM.D. F.R.C.P.C. 

Division of Neonatology, Room 1R11, Children's and Women's Health Centre of British Columbia

4480 Oak Street, Vancouver, BC, Canada V6H 3V4