Radiographic findings in a canine model of mucopolysaccharidosis I. Changes associated with bone marrow transplantation.
Eight dogs with mucopolysaccharidosis I (MPS I) were studied and their radiographic lesions compared with those reported in human MPS I. Three of the dogs received bone marrow transplants from unaffected littermates at 5 months of age. These dogs, and two affected control littermates were radiographed at 3, 6, 9, 12, 15, 18 and 20 months post-transplantation to evaluate the effects in chondro-osseous tissues. Although bone marrow transplantation did not alleviate all radiographically detectable changes, there was delayed onset of some lesions and reduced severity of most lesions in the dogs receiving transplants. Those lesions most closely associated with clinical lameness or gait abnormalities in untreated canine MPS I were most improved (or eliminated), and this was reflected in marked clinical improvement.
D000818:Animals; D001846:Bone Development; D016026:Bone Marrow Transplantation; D001842:Bone and Bones / Q000000981:diagnostic imaging; D004195:Disease Models, Animal; D004285:Dogs; D008059:Mucopolysaccharidosis I / Q000000981:diagnostic imaging / Q000628:therapy*; D011859:Radiography