Generalized Arterial Calcification of Infancy (GACI): Optimizing Care with a Multidisciplinary Approach Article Swipe
Kenji Kawai
,
Yu Sato
,
Rika Kawakami
,
Atsushi Sakamoto
,
Anne Cornelissen
,
Masayuki Mori
,
Saikat Ghosh
,
Robert Kutys
,
Renu Virmani
,
Aloke V. Finn
·
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.2147/jmdh.s251861
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.2147/jmdh.s251861
Kenji Kawai,1,* Yu Sato,1,* Rika Kawakami,1 Atsushi Sakamoto,1 Anne Cornelissen,1 Masayuki Mori,1 Saikat Ghosh,1 Robert Kutys,1 Renu Virmani,1 Aloke V Finn1,2 1CVPath Institute, Gaithersburg, MD, USA; 2University of Maryland, School of Medicine, Baltimore, MD, USA*These authors contributed equally to this workCorrespondence: Aloke V Finn, 19 Firstfield Road, Gaithersburg, MD, 20878, USA, Tel +301.208.3570, Fax +301.208.3745, Email [email protected]: It is very unusual to see evidence of arterial calcification in infants and children, and when detected, genetic disorders of calcium metabolism should be suspected. Generalized arterial calcification of infancy (GACI) is a hereditary disease, which is characterized by severe arterial calcification of medium sized arteries, mostly involving the media with marked intimal proliferation and ectopic mineralization of the extravascular tissues. It is caused by inactivating variants in genes encoding either ENPP1, in a majority of cases (70â 75%), or ABCC6, in a minority (9â 10%). Despite similar histologic appearances between ENPP1 and ABCC6 deficiencies, including arterial calcification, organ calcification, and cardiovascular calcification, mortality is higher in subjects carrying the ENPP1 versus ABCC6 variants (40% vs 10%, respectively). Overall mortality in individuals with GACI is high (55%) before the age of 6 months, with 24.4% dying in utero or being stillborn. Rare cases show spontaneous regression with age, while others who survive into adulthood often manifest musculoskeletal complications (osteoarthritis and interosseous membrane ossification), enthesis mineralization, and cervical spine fusion. Despite recent advances in the understanding of the genetic mechanisms underlying this disease, there is still no ideal therapy for the resolution of vascular calcification in GACI. Although bisphosphonates with anti-calcification properties have been commonly used for the treatment of CAGI, their benefit is controversial, with favorable results reported at one year and questionable benefit with delayed initiation of treatment. Enzyme replacement therapy with administration of recombinant form of ENPP1 prevents calcification and mortality, improves hypertension and cardiac function, and prevents intimal proliferation and osteomalacia in mouse models of ENPP1 deficiency. Therefore, newer treatments targeting genes are on the horizon. In this article, we review up to date knowledge of the understanding of GACI, its clinical, pathologic, and etiologic understanding and treatment in support of more comprehensive care of GACI patients.Keywords: generalized arterial calcification of infancy, ectonucleotide pyrophosphatase/phosphodiesterase 1, ATP binding cassette subfamily C member 6, vascular calcification
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Metadata
- Type
- review
- Language
- en
- Landing Page
- https://doi.org/10.2147/jmdh.s251861
- https://www.dovepress.com/getfile.php?fileID=81186
- OA Status
- gold
- Cited By
- 25
- References
- 66
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4282047362
All OpenAlex metadata
Raw OpenAlex JSON
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https://openalex.org/W4282047362Canonical identifier for this work in OpenAlex
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https://doi.org/10.2147/jmdh.s251861Digital Object Identifier
- Title
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Generalized Arterial Calcification of Infancy (GACI): Optimizing Care with a Multidisciplinary ApproachWork title
- Type
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reviewOpenAlex work type
- Language
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enPrimary language
- Publication year
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2022Year of publication
- Publication date
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2022-06-01Full publication date if available
- Authors
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Kenji Kawai, Yu Sato, Rika Kawakami, Atsushi Sakamoto, Anne Cornelissen, Masayuki Mori, Saikat Ghosh, Robert Kutys, Renu Virmani, Aloke V. FinnList of authors in order
- Landing page
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https://doi.org/10.2147/jmdh.s251861Publisher landing page
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https://www.dovepress.com/getfile.php?fileID=81186Direct link to full text PDF
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YesWhether a free full text is available
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goldOpen access status per OpenAlex
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https://www.dovepress.com/getfile.php?fileID=81186Direct OA link when available
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Calcification, Ectopic calcification, Medicine, Disease, Internal medicineTop concepts (fields/topics) attached by OpenAlex
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25Total citation count in OpenAlex
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2025: 6, 2024: 12, 2023: 7Per-year citation counts (last 5 years)
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66Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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