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Keywords.
hyperphosphatemia, hypocalcemia, calciphylaxis, chronic kidney failure, vascular calcification; bone density conservation agent; debridement
Calciphylaxis is a complication of chronic kidney disease characterized by necrotic lesion in the skin. Histological examination reveals microcalcification of medium- sized blood vessels. We report on a 21-month-old girl with end-stage renal disease with severe calcium-phosphate imbalance. Calciphylaxis process started when she received calcium gluconate intravenously the day before the surgery to correct hypocalcemia and continued progressively despite peritoneal dialysis and forced stopping calcium-containing medication. Pamidronate, 0.5 mg/kg/ d, was administered for 6 days and then once a week for 5 weeks. After 1 week, the skin lesion started to heal and circulation improved, and after 6 weeks, all skin lesions completely recovered. Pamidronate was effective to stop calciphylaxis in this case with advanced renal insufficiency and severe calcium-phosphate imbalance. Medical or surgical debridement are not suggested and lesions might recover without scar by pamidronate.
IJKD 2015;9:67-70
www.ijkd.org
INTRODUCTION
Calciphylaxis is a rare but fatal complication of chronic kidney disease, seen in 1% to 4% of patients. It is characterized by vascular calcification, thrombosis, and necrotic lesions in the skin. 1 However the exact mechanism is unclear, it seems an imbalance of promoting and inhibitory factors of calcification attribute to the development of calciphylaxis. The presence of some risk factors such as end-stage renal disease (ESRD), hypercoagulability state, inflammatory status, female sex, obesity, and administration of vitamin D and calcium-based phosphate binders predispose the patients to caciphylaxis. To the best of our knowledge, there is no report on successful management of calciphylaxis in children with ESRD.2-4 We report on a successful therapy of a calciphylaxis due to chronic kidney failure with pamidronate.
CASE REPORT
A 21-month-old girl with ESRD due to diffuse mesengeal sclerosis was referred for implantation of a Tenckhoff catheter. She was the first child of a nonconsanguineous marriage who presented with edema at the age of 10 days. She had been under standard therapy for congenital nephrotic syndrome. Physical examination was unremarkable except for the developmental delay. Laboratory tests results on admission were as follows: blood urea nitrogen, 150 mg/dL; serum creatinine, 5 mg/ dL; serum uric acid, 10 mg/dL; serum calcium, 4 .5 mg/dL; serum phosphate, 13.6 mg/dL; alkaline phosphatase, 1017 IU; parathyroid hormone,...