[Osteoarthropathy in three siblings with Wilson's disease].
Osteoarthropathies are one of the less usual manifestations of Wilson's disease. They appear in different forms such as osteoporosis (the most frequent), inflammatory changes in small joints, osteomalacia, osteoarthritis in younger ages, spine osteochondritis, fractures and heterotopic ossification. This article describes the different osteoarthropathies in three children: two brothers and one sister with Wilson's disease that first manifested in early childhood with severe neurological signs. After drug treatment and an intense rehabilitation program, the clinical signs stabilized. During the course of the disease, all three children presented fractures of the ulna and radius after low energy trauma, several heterotopic ossifications, some of which were asymptomatic, and inflammatory processes such as hip synovitis and reduction in mineral bone density. The etiology of this kind of manifestation is not yet clear, although the most widely accepted explanation is alteration in calcium and phosphate metabolism.
Servicio de Rehabilitación y Medicina Física, Hospital Universitario Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain.
N
N
Rodríguez Nieva
A
A
Febrer Rotger
M
M
Meléndez Plumed
A
A
Vernet Bori
D000293:Adolescent; D004200:Diseases in Twins; D005260:Female; D050723:Fractures, Bone / Q000150:complications; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D008297:Male; D009999:Ossification, Heterotopic / Q000150:complications*; D010024:Osteoporosis / Q000150:complications*; D013585:Synovitis / Q000150:complications*