What is Endocrinology - What gives rise to Paget's illness?
Both hereditary and nongenetic factors have been linked to the pathophysiology of Paget's disease, despite the fact that its aetiology is unknown. There are several polymorphisms that have been linked to Paget's disease. Up to 50% of patients with familial disease and 10% to 20% of instances that are sporadic have the SQSTM1 mutation. Patients with this mutation have more severe illness than those without it. A role for environmental factors in the aetiology of this disease is supported by the discovery of monostotic disease, the variable penetrance of Paget's disease in families with a genetic disposition, and the observation that the incidence of Paget's disease has been declining over the past 25 years. A viral origin is suggested by reports of paramyxovirus nucleocapsid-like structures in the osteoclasts of active pagetic bone. As etiologic agents, the measles virus, respiratory syncytial virus, and canine distemper virus have all been proposed; however, no virus has ever been cultivated from pagetic osteoclasts or osteoclast precursors up to this point.
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