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Endocrinology

To Gosha illness

To Gosha illness (Qaucher Ph. Ch. E., 1854 — 1918, the French dermatologist) — adjournment of cells to Gosha in marrow and fabric with their subsequent decalcification and destruction.
Frustration of a lipoid and protein metabolism is the cornerstone of a disease. Products of incomplete disintegration of lipoproteids — cerebrosides (kerasin) — in a large number collect in reticuloendothelial structures. In a spleen, a liver, lymph nodes, marrow there is a plentiful adjournment of cells to Gosha, is observed at any age, mainly at persons is a female. Most often long tubular bones are surprised. Sites of defeat can be solitary or multiple. The disease slowly progresses, spontaneous fractures of bones are frequent.
At patients weakness, ostealgias, frequent bleedings, a xanthopathy, blood changes, increase in a spleen and liver, a conjunctiva thickening at a cornea, etc. are noted. Early symptoms of an illness — pigmentation and change in peripheral blood. On a face, a neck, hands, that is the sites of skin which are affected by light appear dark brown pigmentation. Later develop a thrombopenia, the amount of hemoglobin and number of erythrocytes decreases that leads to hemorrhagic diathesis and hypochromia anemia.
Radiological there is well visible thickening more often at the lower end of a hip, consolidation of a head of a hip. Also the thickening of bodies of vertebrae is established. The cortical layer of tubular bones becomes thinner. After a spontaneous change the outline of bones happens uneven owing to formation of a callosity. In the beginning widespread osteoporosis is noted, later — there are sites, as if corroded a moth, with a rough trabecular structure.
Symptomatic treatment.

 
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