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Albers-Schoenberg illness

Albers-Schoenberg an illness (Heinrich Ernst Albers-Schoi berg, 1865 — 1921, the German surgeon and the radiologist) — a marble illness of bones. Inborn generalized diffusion osteosclerosis. Fossil of bones, giperostatichesky dysplasia. Autosomal and recessive inheritance. The illness meets seldom, this general disease consists in disharmony of development of bone and hemopoietic tissue the disturbance of a mineral exchange leading to overdevelopment of the compact bone substance filling as well marrowy channels.
Extremity pain, fatigue when walking are expressed. Fragility of bones, increase in a liver and spleen, a delay growth of disorder of gait are noted. Hypochromia anemia, a leukocytosis at children and a leukopenia at adults are in some cases observed an atrophy of an optic nerve. The tendency to osteomyelitis and caries of teeth is found. Quite often calcification of internals, anemia, sepsis, a tetany come to light.
On roentgenograms the sclerosis of all bones as "marble" drawing of a bone is noted, marrowy cavities are not visible.
Treatment can be symptomatic. At fractures of bones - an immobilization a plaster bandage.
The forecast — usually death at children's age. Also the form with autosomal and dominant inheritance differing in a favorable current is described.

 
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