Περίληψη:
Metastatic calcinosis is a common feature of chronic renal failure. Its
first manifestations are bone demineralization and non-visceral and/or
visceral calcification with mostly mural deposits in arteries and
arterioles. It is initially characterized by hyperphosphataemia followed
by secondary or tertiary hyperparathyroidism. Cutaneous involvement is a
rare complication, Histologically, the lesions show vascular
calcification with ischaemic skin necrosis, Extreme cases may produce
calcinosis cutis (calciphylaxis), i.e. disseminated calcification of the
subcutaneous tissue and dermis in the form of hard painful cutaneous
nodules and plaques with subsequent ulceration. Metastatic calcinosis is
a disease affecting adults, while the dystrophic or idiopathic type can
develop in children. We present the case of a 6-year-old boy with
end-stage renal disease, attributed to congenital renal hypoplasia, and
accompanied by secondary hyperparathyroidism. He developed fulminant
tertiary hyperparathyroidism and metastatic calcinosis of the lungs, as
well as cutaneous necrosis of the buttocks and legs, subsequent to
calcification of arteries and arterioles. A maternal renal transplant
failed to function. The serum parathormone, calcium and phosphate levels
could not be controlled by maintenance dialysis, phosphate binders and
calcitriol. Total parathyroidectomy without autotransplantation of
parathyroid tissue rapidly returned the serum parathormone, calcium and
phosphate levels to normal. In addition, topical treatment using
merbromine solution and hydrocolloid dressings, healed the ulcers with
significant scar formation, within 2 . 5 months after parathyroidectomy.
A renewed increase of the calcium x phosphate product, 2 months after
parathyroidectomy, was attributed to mobilization of calcium compounds
from the viscera, as confirmed by a chest X-ray.
Συγγραφείς:
Zouboulis, CC
BlumePeytavi, U
Lennert, T
Stavropoulos, PG
and Schwarz, A
Runkel, N
Trautmann, C
Orfanos, CE