Albers-Schönberg disease

Albers-Schönberg disease /-shœn″burg, -shōn″-/ [Heinrich E. Albers-Schönberg, German radiologist and surgeon, 1865–1921] , a rare condition transmitted as an autosomal-dominant trait in which there are bandlike areas of condensed bone at the epiphyseal lines of long bones and condensation of the edges of smaller bones. See also osteopetrosis. ▪ OBSERVATIONS: Fractures are frequent, and recovery is slow. Deformities of the head, chest, or spine may occur. Anemia may also be present as the bone mass encroaches on bone marrow spaces. The cranial nerves may also be affected as bone is deposited in the skull. ▪ INTERVENTIONS: Management is supportive and not curative. Bone marrow transplantation may be employed to provide cells that can be converted to osteoclasts. Other treatments include the administration of gamma interferon, a protein that delays progression of the disease, or calcitriol, a vitamin D compound that stimulates osteoclasts to dissolve and absorb bone. ▪ PATIENT CARE CONSIDERATIONS: Health care professionals should offer surveillance, symptomatic treatment, and the prevention of complications. Measures to promote quality of life are important. Genetic counseling should be offered to families.