Generalized Arterial Calcification of Infancy

Vascular calcification or ectopic mineralization in blood vessels is an active, cell-regulated process, increasingly recognized as a general cardiovascular risk factor. Ectopic artery mineralization is frequently accompanied by decreased bone mineral density or disturbed bone turnover and development of the osteoporosis. The latest data support the correlation of osteoporosis and atherosclerosis, indicating the parallel progression of two tissue destruction processes with increased fatal and nonfatal coronary events, as well as a higher fracture risk. Patients with osteoporosis, have a higher risk of cardiovascular diseases than subjects with normal bone. Many proteins responsible for bone formation and resorption have been identified in the arterial wall. Vascular calcification includes mostly osteogenic and, to a lesser extent chondrogenic differentiation of osteoblasts and osteoclast-like cells. It has been shown that many of the regulators of bone formation and resorption some bone structural proteins, such as osteoprotegerin (OPG), receptor activator of nuclear factor-κB ligand (RANKL) are also expressed in the atherosclerotic plaque. When RANKL binds to RANK, osteoclasts are activated and bone resorption occurs and processes of vascular calcification become also activated. OPG, protein homologue to receptor activator of nuclear factor-κB (RANK), can bind to RANKL, blocking the binding of RANKL to RANK, that results in inhibition of differentiation of preosteoclasts to mature osteoclasts, lower osteoclast capacity for resorption of bone mineral matrix, and development vascular calcification. The latest data supports that cathepsin K, a cysteine protease, can efficiently degrade type I and II collagen, both of which are major matrix components of the bone and atherosclerotic plaque. These findings further underscore the potential of cathepsin K as a target for novel molecules to treat osteoporosis and atherosclerosis. Thus, the discovery of the cytokine RANKL-RANK-OPG system and significant role of the cathepsin K in the process of bone remodeling, vascular calcification and atherosclerosis has made progress in understanding the mechanisms of disease development and possibly to develop new dual therapies. New therapies for osteoporosis and atherosclerosis that may potentially improve or augment existing treatments include the recently approved anti-receptor activator of NF-κB-ligand monoclonal antibody fms (denosumab) and the cathepsin K inhibitor odanacatib, presently in the late stage of clinical development.

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Molecular Biology Becomes Diagnostic Tool in the Newborn Emergency Department: A Rare Case of Child Idiopathic Arterial Calcification

Borromini¹,

Rossi²

2015

J Pediatr Care

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This case report aims to share with readers the experience of the medical team of the hospital A. Manzoni in Lecco, who has had to handle a sudden cardiac arrest in an infant of three months old, a context in which the clinical features remained unclear and blurred all the time of resuscitation, until the exploration autopsy and pathological investigation outlined in its components.The baby, 96 days old, had been conducted in pediatric emergency from the parents, because during the night she had appeared agitated; parents also reported excesses of uncontrollable coughing and power failure during the previous 12 hours. The patient was hypothermic (35.9°C rectal temperature), with episodes of unconsolable crying; then she was borught to the attention of a pediatrician with a priority value equal to 4 (noncritical) [1,2].Physical examination shows fairly good general conditions, weight of 4, 820 grams (reportedly on the rise), drowsiness Glasgow Coma Scale=11 (be awakened by a painful stimulus), plaintive cry, photometer reflex present; no meningeal signs; Moro reflex present; pale-pink skin with signs of hypothermia over the extremities and perioral cyanosis; breath leaning; at auscultation chest the murmur was tightened, but without the presence of noise added; no organomegaly. Given the difficulties in finding a peripheral venous access, the anesthetist and the neonatologist were contacted to collaborate in assisting the infant [3,4].On their arrival the general conditions were already deteriorating. The breath had become rapidly tachi-dyspnoeic and therapy with oxygen mask with reservoir was started. The peripheral Molecular Biology Becomes Diagnostic Tool in the Newborn Emergency Department: A Rare Case of Child Idiopathic Arterial Calcification AbstractA Turkish infant of 96 days is accepted to the pediatric emergency room after being carried by their parents because of inconsolable crying, persistent cough, and difficulty in feeding during the previous day.Although she was aware and nor signs nor symptoms was suggestive of an impending MOF, in a few minutes it takes over an asystolic cardiac arrest. It was found the exitus after 75 minutes of resuscitation.The autopsy report indicates as pathological cause of death a rare form of childhood idiopathic calcific arterial disease caused by mutation of the gene encoding the same name EPP1 enzyme.

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Generalized Arterial Calcification of Infancy

Cited by 3 publications

References 10 publications

Artériosclérose diffuse inexpliquée chez une jeune patiente de 26 ans

Artériosclérose diffuse inexpliquée chez une jeune patiente de 26 ans

Vascular Calcification, Atherosclerosis and Bone Loss (Osteoporosis): New Pathophysiological Mechanisms and Future Perspectives for Pharmacological Therapy

Molecular Biology Becomes Diagnostic Tool in the Newborn Emergency Department: A Rare Case of Child Idiopathic Arterial Calcification

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