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Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation
Author(s) -
Glatz Andrew C.,
Pawel Bruce R.,
Hsu Daphne T.,
Weinberg Paul,
Chrisant Maryanne R. K.
Publication year - 2006
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2005.00414.x
Subject(s) - medicine , intensive care medicine , transplantation , calcification , referral , disease , heart transplantation , pediatrics , surgery , pathology , family medicine
Idiopathic infantile arterial calcification (IIAC) is a rare, but important, cause of rapidly progressive ischemic heart disease in children. In this paper, we report two recent cases of IIAC seen at tertiary referral hospitals. Both cases presented in infancy with signs of heart failure and, ultimately, died with the diagnosis of IIAC confirmed at postmortem examination. A thorough review of the literature reveals approximately 160 reported cases of IIAC. The clinical outcomes, radiographic findings and pathologic details are summarized. Proposed etiologic mechanisms are reviewed, including promising research into the role of inorganic pyrophosphate as a regulatory factor in the development of IIAC. Because of the typically fatal outcome of IIAC and the lack of proven therapies, the potential role for cardiac transplantation is discussed.