Original article
Idiopathic infantile arterial calcification in siblings: Radiologic diagnosis and successful treatment

https://doi.org/10.1016/S0022-3476(78)80427-2Get rights and content

Idiopathic infantile arterial calcification is a disorder of unknown etiology manifested by widespread arterial calcification. This usually leads to early death from coronary artery occlusion. In 12 of the 75 cases in the literature, radiographs were taken and it was possible to make the diagnosis in them all. We present two patients, siblings, in whom the diagnosis was established radiologically. The nature of the calcium deposits was studied in one of the infants and proved to be calcium hydroxyapatite. Therapy with diphosphonate was apparently successful in the other child.

References (15)

  • DuranteG

    Athérome congénitale de l'aorte et de l'arterie pulmonaire

    Bull Soc Anat Paris

    (1899)
  • BryantJH et al.

    A case of calcification of the arteries and obliterative endarteritis, associated with hydronephrosis in a child age six months

    Guys Hosp Rep

    (1901)
  • StrykerWA

    Arterial calcification in infancy with special reference to the coronary arteries

    Am J Pathol

    (1946)
  • WeensHS et al.

    Infantile arteriosclerosis

    Radiology

    (1956)
  • BaconJF

    Arterial calcification in infancy

    JAMA

    (1964)
  • MoranJJ et al.

    Idiopathic arterial calcification of infancy

    Am J Clin Pathol

    (1959)
  • Lussier-LazaroffJ et al.

    Idiopathic infantile arterial calcification: Roentgen diagnosis of a rare cause of coronary artery occlusion

    Pediatr Radiol

    (1973)
There are more references available in the full text version of this article.

Cited by (76)

  • Skeletal abnormalities secondary to antenatal etidronate treatment for suspected generalised arterial calcification of infancy

    2020, Bone Reports
    Citation Excerpt :

    A broad phenotypic variation ranging from a spontaneous resolution in childhood to an early death despite adequate treatment with the bisphosphonates has been reported in the siblings carrying identical mutations (Le Boulanger et al., 2010). Etidronate, a first generation bisphosphonate, is a synthetic PPi analogue and leads to the cessation of the hydroxyapatite crystal formation and elimination of radiographically evident arterial calcifications in GACI (Meradji et al., 1978). It typically takes from two weeks to two years for radiological resolution of arterial calcifications (Meradji et al., 1978).

  • Etidronate halts systemic arterial calcification in pseudoxanthoma elasticum

    2020, Atherosclerosis
    Citation Excerpt :

    Most patients die within the first six months of life and bisphosphonate use is associated with prolonged survival in these patients [17]. Several case reports into GACI show that arterial calcification in the common carotid artery, coronary arteries, aorta, pulmonary, renal, iliac and femoral arteries decreases and even resolves after etidronate treatment[11,18–23], although regression without etidronate has also been described [24–26]. PPi is one of the strongest calcification inhibitors in the human body.

View all citing articles on Scopus
View full text