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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 887-888
Generalized arterial calcification in a still born: An autopsy case report

Department of Pathology, NRI Medical College and General Hospital, Chinakakani, Mangalagiri Mandal, Guntur dt., Andhra Pradesh, India

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Date of Web Publication27-Oct-2010

How to cite this article:
Koti K, Kande S, Pidakala P, Ravi K. Generalized arterial calcification in a still born: An autopsy case report. Indian J Pathol Microbiol 2010;53:887-8

How to cite this URL:
Koti K, Kande S, Pidakala P, Ravi K. Generalized arterial calcification in a still born: An autopsy case report. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Aug 5];53:887-8. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/887/72040


Generalized arterial calcification of infancy (GACI), also referred to as idiopathic infantile arterial calcification, is a rare autosomal recessive disease of unknown etiology that can affect large and medium sized arteries. [1] It is observed in live and stillborn infants; 85% of affected infants die by 6 months of age. [2] The clinical manifestations are usually attribu table to blockage of the coronary arteries. These cases can be diagnosed in utero with the assistance of sonography, but the radiographic findings may be very subtle and majority of cases are diagnosed during post mortem. [3]

A 20 year old primigravida, with no history of consanguinity, came to our institute after her uneventful antenatal checkups elsewhere. Her main complaint was loss of appreciation of fetal movements for the past 2 months. Her blood group was ORh negative and other routine laboratory test results were normal. The ultrasound scan showed a dead fetus corresponding to 27 ± 2 weeks of gestational age. Autopsy was performed. The fetus and placenta weighed 1 kg and 300 g, respectively. There were no external or internal anomalies. The most outstanding lesion macroscopically was rigidly calcified pulmonary artery, aorta and its branches. The placenta showed an infarct.

Hematoxylin and eosin stained tissue sections showed calcification varying from minimal incrustation of the internal elastic lamina to large irregular masses distorting the media of both systemic (aorta) and pulmonary arteries [Figure 1] and [Figure 2]. Heart showed foci of dystrophic calcification in the myocardium as a telltale sign of myocardial ischemic necrosis consequent to coronary vessel calcification [Figure 3]. Histologically, there was no calcification in other sites and thus metastatic calcification was excluded. The diagnosis of generalized arterial calcification in a stillborn was made exclusively on the autopsy findings, as there was no antenatal suspicion of GACI.
Figure 1: Photomicrograph showing calcification of the aorta (H and E, ×200)

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Figure 2: Photomicrograph showing calcification of the pulmonary arteriole (H and E, ×200)

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Figure 3: Photomicrograph showing calcification of the coronary artery. (H and E, ×200); inset shows dystrophic calcification in the myocardium (H and E, ×400)

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The recently described mechanism of arterial calcification involves mutations in ectonucleotide pyrophosphatase 1 gene (ENPP1), which lead to decreased inorganic pyrophosphate concentration and result in calcium deposition in the vasculature. Rutsch et al, [4] found ENPP1 gene mutation in approximately 75% of their subjects. Some of the cases of GACI present in utero with evidence of fetal compromise, which may manifest as hydrops fetalis and premature delivery or in seldom occasions as stillbirth. Isolated reports of spontaneous regression with survival to adult life and successful medical therapy with bisphosphonates have been obtained. [5] This case is a rare presentation of GACI as a definite cause of intrauterine death at 27 ± 2 weeks of gestation in a primigravida.

Ever since it was first described in 1901 by Bryant and White [1] though more than 170 case reports have been published on this disease, many cases go undiagnosed or unreported. [5] A case report like ours is essential to determine the natural history of the disease. It would certainly endeavor the researchers to pursue and identify some more biological changes of life in GACI, which could definitely influence the genetic counselling and future screening of the siblings at risk.

   References Top

1.Bryant JH, White WA. A case of calcification of the arteries and obliterative endarteritis associated with hydronephrosis in a child aged 6 months. Guys Hosp Rep 1901; 55:17-28.  Back to cited text no. 1
2.Moran JJ. Idiopathic arterial calcification of infancy a clinocopathological study. Pathol Ann 1975;10:393-17.  Back to cited text no. 2
3.Rosenbaum DM, Blumhagen JD. Sonographic recognition of idiopathic arterial calcification of infancy. AJR Am J Roentgenol 1986;146:249-50.  Back to cited text no. 3
4.Rutsch F, Ruf N, Vaingankar S, Toliat MR, Suk A, Hohne W, et al. Mutations in ENPP1 are associated with "idiopathic" infantile arterial calcification. Nat Genet 2003;34:379-81.  Back to cited text no. 4
5.Ramjan KA, Roscioli T, Rutsch F, Sillence D, Munns CF. Generalized arterial calcification of infancy: Treatment with bisphosphonates. Nat Clin Pract Endocrinol Metab 2009;5:167-72.  Back to cited text no. 5

Correspondence Address:
Kalyan Koti
NRI Medical College & General Hospital, Chinakakani, Mangalagiri Mandal, Guntur dt., Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.72040

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  [Figure 1], [Figure 2], [Figure 3]


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