[Therapy of bone and joint changes in Albers-Schönberg osteopetrosis]. 1988

P Griss, and T Schäfer
Orthopädische Klinik, Philipps-Universität Marburg/Lahn.

The history, genetic heterogenity, clinical appearance, radiologic morphology and specific pathology of Albers-Schönberg disease are described, with special reference to its orthopedic aspects, on the basis of 14 case histories. The orthopedic problems (osteomyelitis, delayed fracture and osteotomy healing, growth deviations of long bones, spinal pathology, arthrosis and fatigue fractures) are described in detail and documented. The disease is often recognized only by chance when routine X-ray examinations are performed. More serious manifestations of the disease, however, such as spontaneous osteomyelitis, fatigue fractures, coxarthrosis or gonarthrosis with or without growth aberrations, pose difficult problems of treatment. Delayed callus formation is routinely observed; this demands special osteosynthesis techniques must be constantly borne in mind during and after operative care.

UI MeSH Term Description Entries
D010022 Osteopetrosis Excessive formation of dense trabecular bone leading to pathological fractures; OSTEITIS; SPLENOMEGALY with infarct; ANEMIA; and extramedullary hemopoiesis (HEMATOPOIESIS, EXTRAMEDULLARY). Albers-Schoenberg Disease,Marble Bone Disease,Osteosclerosis Fragilis,Albers-Schonberg Disease,Albers-Schonberg Disease, Autosomal Dominant,Albers-Schönberg Disease,Autosomal Dominant Osteopetrosis Type 2,Congenital Osteopetrosis,Marble Bones, Autosomal Dominant,Osteopetrosis Autosomal Dominant Type 2,Osteopetrosis, Autosomal Dominant 2,Osteopetrosis, Autosomal Dominant, Type II,Osteosclerosis Fragilis Generalisata,Albers Schoenberg Disease,Albers Schonberg Disease,Albers Schonberg Disease, Autosomal Dominant,Albers Schönberg Disease,Disease, Albers-Schoenberg,Disease, Albers-Schonberg,Disease, Albers-Schönberg,Disease, Marble Bone,Osteopetroses,Osteosclerosis Fragilis Generalisatas
D005593 Fracture Fixation, Internal The use of internal devices (metal plates, nails, rods, etc.) to hold the position of a fracture in proper alignment. Osteosynthesis, Fracture,Fixation, Internal Fracture,Fixations, Internal Fracture,Fracture Fixations, Internal,Fracture Osteosyntheses,Fracture Osteosynthesis,Internal Fracture Fixation,Internal Fracture Fixations,Osteosyntheses, Fracture
D005598 Fractures, Spontaneous Fractures occurring as a result of disease of a bone or from some undiscoverable cause, and not due to trauma. (Dorland, 27th ed) Fractures, Pathological,Fracture, Pathologic,Fracture, Pathological,Fractures, Pathologic,Pathological Fracture,Pathological Fractures,Fracture, Spontaneous,Pathologic Fracture,Pathologic Fractures,Spontaneous Fracture,Spontaneous Fractures
D006622 Hip Prosthesis Replacement for a hip joint. Femoral Head Prosthesis,Femoral Head Prostheses,Hip Prostheses,Prostheses, Femoral Head,Prostheses, Hip,Prosthesis, Femoral Head,Prosthesis, Hip
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000671 Amputation, Surgical The surgical removal of part of, or all of, a limb or other appendage or outgrowth of the body. Amputation,Amputation, Multiple, Surgical,Multiple Amputation, Surgical,Surgical Amputation Procedures,Amputation Procedure, Surgical,Amputation Procedures, Surgical,Amputation, Surgical Multiple,Amputations,Amputations, Surgical,Amputations, Surgical Multiple,Multiple Amputations, Surgical,Procedure, Surgical Amputation,Procedures, Surgical Amputation,Surgical Amputation,Surgical Amputation Procedure,Surgical Amputations,Surgical Multiple Amputation,Surgical Multiple Amputations
D016025 Bone Transplantation The grafting of bone from a donor site to a recipient site. Grafting, Bone,Transplantation, Bone,Bone Grafting

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