osteopetrosis

osteopetrosis /os·teo·pe·tro·sis/ (-pĭ-tro´sis) a hereditary disease marked by abnormally dense bone, and by the common occurrence of fractures of affected bone.

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os·te·o·pe·tro·sis (st--p-trss)

n.

An inherited disorder in which bone and cartilage, especially in long bones, become dense and hardened to an extent that marrow is obliterated, and anemia, spleen and liver enlargement, blindness, and progressive deafness occur; it begins in infancy and sometimes results in early death. Also called Albers-Schönberg disease, marble bone disease, marble bones.

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oste·o·pe·trotic (-trtk) adj.

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osteopetrosis

[os′tē·ōpētrō′sis]

Etymology: Gk, osteon + petra, stone, osis, condition

an inherited disorder characterized by a generalized increase in bone density, probably caused by faulty bone resorption resulting from a deficiency of osteoclasts. In its most severe form, transmitted as an autosomal-recessive condition, the bone marrow cavity is obliterated, causing severe anemia, marked deformities of the skull, and compression of the cranial nerves, which may result in deafness and blindness and lead to an early death. A milder, benign form, transmitted as an autosomal-dominant trait, is characterized by short stature, fragile bones that fracture easily, and a tendency to develop osteomyelitis. Also called ivory bones, marble bones, osteosclerosis fragilis. See also Albers-Schönberg disease. osteopetrotic, adj.

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osteopetrosis (ostēōpetrō´sis),

n an osteosclerosis of unknown origin that obliterates the bone marrow regions, with resultant anemia. Delayed tooth eruption and severe osteomyelitis or necrosis after dental infection may be associated with the disease. Also known as
Albers-Schönberg disease, marble bone.

Osteopetrosis.

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osteopetrosis

in mammals, a hereditary disease marked by abnormally dense bone, and by the common occurrence of fractures of affected bone. It may lead to obliteration of the marrow spaces, causing anemia. Readily diagnosable radiographically. Occurs in rats, rabbits and cattle, in which it is usually associated with other inherited, congenital skeletal defects including shortness of long bones. In calves needs to be differentiated from similar lesions caused by infection in utero by the bovine virus diarrhea virus.

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avian osteopetrosis

occurs as part of the avian leukosis, rous sarcoma complex. All bones are affected but the long bones are most obviously deformed. Called also thick leg disease of poultry.

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osteopetrosis

Albers-Schoenberg disease, marble bone disease Pediatric endocrinology A heterogeneous group of rare AD and cortical and trabecular osteosclerotic disorders, characterized by ↓ osteoclastic activity and ↓ bone resorption, with accumulation of sclerotic bone, compromising marrow space Management IFN-gamma-1b, BMT, high-dose calcitriol