Paget’s Disease of the Bone: a Clinical Report

Abstract

Pagetova bolest kostiju (PBK) kronični je poremećaj remodeliranja kostiju s povećanom resorpcijom posredstvom osteoklasta te stvaranjem nove kosti, što rezultira neorganiziranim mozaikom „divlje“ i lamelarne kosti na pogođenim područjima kostura. Ta se bolest obično klinički ne zapaža prije dobi od 40 godina. Kliničke manifestacije mogu biti u rasponu od asimptomatičnih do bolnih deformiteta više od jedne kosti. Od PBK najčešće obolijevaju bijelci europskog podrijetla, ali i crnci, no vrlo rijetko žuta rasa. Mnogi se simptomi mogu liječiti antiosteoklastnom terapijom, poput kalcitonina i bifosfanata. Nažalost gotovo da i nema dokumentiranih dugoročnih rezultata. U ovom radu izvještavamo o kliničkom slučaju Pagetove bolesti kostiju i dajemo kratak pregled literature.Paget’s disease of bone (PDB) is a chronic bone remodeling disorder characterized by increased osteoclast-mediated bone resorption with subsequent new bone formation resulting in a disorganized mosaic of woven and lamellar bone at affected skeletal sites. The disease is usually not clinically apparent under 40 years of age. The clinical manifestations may range from asymptomatic to painful deformity of more than one bone. PDB is most common in white people of European descent, but it also occurs in black population, whereas it is rare in people of Asian descent. With anti-osteoclast therapy such as calcitonin and bisphosphonates many of the symptoms are relieved. But the risk of future complications still remains. Unfortunately, very little evidence of long-term results is available. We report a case of Paget’s disease of bone with a brief review of literature