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

newborn
kidney
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. https://doi.org/10.7363/020110

Abstract

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.

https://doi.org/10.7363/020110
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