medial calcific sclerosis

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sclerosis

 [sklĕ-ro´sis]
an induration or hardening, especially of a part from inflammation, or in disease of the interstitial substance. The term is used chiefly for such a hardening of the nervous system due to hyperplasia of the connective tissue or for hardening of the blood vessels. Called also induration. adj., adj sclerot´ic.
amyotrophic lateral sclerosis see amyotrophic lateral sclerosis.
arteriolar sclerosis arteriolosclerosis.
disseminated sclerosis multiple sclerosis.
familial centrolobar sclerosis a progressive familial form of leukoencephalopathy marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.
focal glomerular sclerosis focal segmental glomerulosclerosis.
glomerular sclerosis glomerulosclerosis.
hippocampal sclerosis loss of neurons in the region of the hippocampus, with gliosis; sometimes seen in epilepsy.
lateral sclerosis a form seated in the lateral columns of the spinal cord. It may be primary, with spastic paraplegia, rigidity of the limbs, and increase of the tendon reflexes but no sensory disturbances, or secondary to myelitis, with paraplegia and sensory disturbance.
medial calcific sclerosis (Mönckeberg's sclerosis) Mönckeberg's arteriosclerosis.
multiple sclerosis see multiple sclerosis.
systemic sclerosis systemic scleroderma.
tuberous sclerosis a congenital heredofamilial disease, transmitted as an autosomal dominant trait, characterized principally by the presence of hamartomas of the brain (tubers), retina (phakomas), and viscera, mental retardation, seizures, and adenoma sebaceum, and often associated with other skin lesions.

medial calcific sclerosis

medial calcific sclerosis

A type of dystrophic calcification in which calcium is deposited in the tunica media of arteries.

Clinical findings
Medial cystic sclerosis is usually asymptomatic, but may be associated with atherosclerosis, hypertension, diabetes and chronic kidney disease. Areas affected are “pulseless” due to the calcified encasement; areas beyond the sclerosis have a “bounding” pulse.

Imaging
Affected medium-sized arteries in the upper and lower limbs have linear calcifications, resulting in a so-called pipestem appearance.
 
Pathogenesis
Uncertain; an array of calcium-regulating proteins have been implicated, including osteopontin, osteoprotegerin, matrix GLA protein, fetuin-A, receptor activator of NF-kappa-B, receptor activator of NF-kappa-B ligand and TNF-related apoptosis-inducing ligand.
References in periodicals archive ?
Medial calcification, or Monckeberg's arteriosclerosis, is the pathological deposition of calcium-phosphate mineral along the elastic fibers in the middle layer of arteries and is associated with chronic kidney disease (CKD), diabetes, and ageing.
The pathologic diagnostic criteria for calciphylaxis utilized at the Mayo Clinic require skin biopsy evidence of medial calcification and intimai fibroplasia of pannicular arterioles with cutaneous necrosis.
To quantify vascular medial calcification, the aortic roots (from the aortic opening to 0.
Medial calcification is an abnormality that produces stiffening of the arteries resulting in increased pulse pressure, increased pulse wave velocity, and increased systolic blood pressure.
It has been shown that lack of osteoprogerin in rats develops osteoporosis and medial calcification in the aorta.
His name has since been associated with medial calcification, though, it is not clear whether the condition modern pathologists call Monckeberg calcific medial sclerosis is the same pathology he described.
Medial calcification has been observed in both young and middle-aged patients, and is closely associated with the duration of hemodialysis and calcium-phosphate disorders (Giachelli, 2004; London et al.
This entity has been variously termed uremic small-vessel disease, uremic small-artery disease, uremic gangrene syndrome, medial calcification, and intimal hyperplasia.
The characteristic "subcutaneous necrosis" is a result of medial calcification of the arterioles leading to ischaemia.
Elastin Degradation and Vascular Smooth Muscle Cell Phenotype Change Precede Cell Loss and Arterial Medial Calcification in a Uremic Mouse Model of Chronic Kidney Disease.

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