Document Detail

Idiopathic infantile arterial calcification and persistent pulmonary hypertension.
MedLine Citation:
PMID:  15838744     Owner:  NLM     Status:  MEDLINE    
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.
Juliet Farquhar; Nawal Makhseed; Michael Sargent; Glenn Taylor; Horacio Osiovich
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of perinatology     Volume:  22     ISSN:  0735-1631     ISO Abbreviation:  Am J Perinatol     Publication Date:  2005 Apr 
Date Detail:
Created Date:  2005-04-19     Completed Date:  2005-07-26     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8405212     Medline TA:  Am J Perinatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  121-5     Citation Subset:  IM    
Division of Neonatology, Children's and Women's Health Centre of British Columbia, University of British Columbia, Vancouver, British Columbia V6H 3V4, Canada.
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MeSH Terms
Arterial Occlusive Diseases / complications,  diagnosis*,  physiopathology
Calcinosis / complications,  diagnosis*,  physiopathology
Coronary Angiography
Coronary Vessels / pathology
Extracorporeal Membrane Oxygenation
Fatal Outcome
Hypertension, Pulmonary / etiology*,  pathology,  therapy
Infant, Newborn
Metabolism, Inborn Errors / pathology
Phosphoric Diester Hydrolases / deficiency
Pyrophosphatases / deficiency
Tunica Intima / pathology*
Reg. No./Substance:
EC 3.1.4.-/Phosphoric Diester Hydrolases; EC pyrophosphatase phosphodiesterase 1; EC 3.6.1.-/Pyrophosphatases

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