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Calciphylaxis due to poor compliance in a child with end stage renal disease: A case report
Author(s) -
Toprak Demet,
Bircan Zelal,
Gokalp Ayse Sevim
Publication year - 2005
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.2005.02019.x
Subject(s) - calciphylaxis , medicine , secondary hyperparathyroidism , adipose tissue , calcification , end stage renal disease , hyperparathyroidism , hyperphosphatemia , buttocks , surgery , parathyroid hormone , pathology , kidney disease , hemodialysis , calcium
Calciphylaxis (calcific uremic arteriolopathy) is a vasculopathy that is often defined in patients due to renal failure, however, some patients with calciphylaxis do not show secondary or tertiary hyperparathyroidism. It results from mural calcification of arteries and arterioles in the deep dermis and subcutaneous adipose tissue, leading to vessel occlusion. The resulting ischemia may be so severe that infarction of the downstream tissue may develop, the most affected sites being the skin and subcutaneous adipose tissue. The regions of the body with thicker subcutaneous adipose tissue such as the breast, abdomen, thighs and buttocks may be more frequently affected. 1 The accepted concept is that reduced glomeruler filtration rate, even in the early stages of end-stage renal disease (ESRD), results in diminished renal phosphorus excretion and an increase in serum phosphorus concentration. The retained inorganic phosphorus combines with plasma calcium and the calcium × phosphate product with CaHPO4 concentration rise. 2 This leads to the stimulation of parathyroid hormone secretion and secondary hyperparathyroidism as well as the resorption of calcium and phosphorus from bone with resulting osteodystrophy and metastatic calcification. Cutaneous metastatic calcification was reported as long ago as 100 years. The lesions are quite characteristic, starting as areas of painful purple discoloration in the extremities that are plaquelike or nodular. They usually progress to necrotizing gangrenous areas and ulcerations. Some reports describe actual gangrene and self-amputation of digits or extremities. 3 In this case report, we present an 8-year-old girl who experienced cutaneous and widespread visceral calcifications due to poor compliance of her parents to the treatment of chronic renal failure. Case report