Peri and intraglomerular haematoxylinophilic deposits in a newborn: answer
JPNIM Vol. 2 N. 1 - Cover


calcium deposits
idiopatic infantile arterial calcification

How to Cite

Nemolato, S., Sanna, A., Gerosa, C., Fanni, D., Palmas, G., Puddu, M., Loddo, C., Fanni, C., Van Eyken, P., & Faa, G. (2013). Peri and intraglomerular haematoxylinophilic deposits in a newborn: answer. Journal of Pediatric and Neonatal Individualized Medicine (JPNIM), 2(1), 98-100.


Idiopathic infantile arterial calcification (IIAC) is a rare disease characterized by abnormal calcification of the arterial vessels, resulting in calcium deposits in the wall of medium-sized and large arteries. IIAC is caused by mutations in the ENPP1 gene, localized on chromosome 6q22, resulting in deficiency of the enzyme PC-1 nucleoside triphosphate pyrophosphohydrolase (NPP). Clinical presentation may occur during the intrauterine life, with fetal hydrop, aorto-pulmonary calcification, or as fatal hypertensive cardiomyopathy. In other patients, the clinical presentation is in the postnatal period with hypertrophic cardiomyopathy, with a fatal outcome within 6 months due to intractable heart failure.

Here we report the clinico-pathological findings of a preterm affected with IIAC, with particular emphasis on renal glomerular pathological lesion not previously described in this disease.