Generalized Arterial Calcification of Infancy

Also known as: GACI, idiopathic arterial calcification of infancy, IACI, occlusive infantile arterial calcification, occlusive infantile arteriopathy.

What is generalized arterial calcification of infancy?

Generalized arterial calcification of infancy is a rare genetic disorder present at birth (congenital) that is characterized by calcium buildup in all blood vessels throughout the body (though affecting mainly larger and medium sized arteries). This causes hardening and narrowing of the arteries and results in an increased risk for many medical problems.

What causes generalized arterial calcification of infancy?

Generalized arterial calcification of infancy is a recessive genetic disorder that is passed along from both unaffected parents to their children. There are two different genetic types (type 1 and 2) which present similarly during infancy but differ as the children grow older.

What are the symptoms of generalized arterial calcification of infancy? 

Type 1 (the more common Type) presents early in infancy and babies frequently die within the first 6 months of life. They can present in the uterus with decreased fetal activity, increased fluid around the fetus (polyhydramnios); after birth they present with blush color to the skin (cyanosis), heart failure, high blood pressure, fast breathing, swelling, distended abdomen and and kidney failure.  If they survive they may present with soft bones, bow legs and some features of Rickets, and deafness.
Type 2 disease leads to abnormalities in the elastic tissue of the skin, eyes and blood vessels (plus other organs like the digestive system). The skin may have yellowish bumps (on the neck and other areas), and eye changes. They can also develop the problems associated with calcification and narrowing of the arteries.

What are generalized arterial calcification of infancy care options? 

Certain medications can reduce the calcium buildup in the blood vessels and help to manage generalized arterial calcification of infancy.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 1/11/2018 1:29:13 PM

From the Newsdesk

March Patient of the Month: Theodore
03/15/2018 — Meet our March Patient of the Month, Theodore. Theodore was diagnosed with cleft palate, cleft lip and a heart problem when he was only 18 weeks old. After he was born, Theodore had to be admitted into the NICU to be able to perform the necessary surgeries for him to live a healthy life.
Duchenne Regional Family Summit
12/02/2017 — CureDuchenne Cares and The Nicklaus Children’s Hospital Brain Institute hosted a free educational and social event for Duchenne family and caregivers. Attendees were able to learn about important DMD care topics, share their practical advice and connect with other families.