shagreen$74263$ - определение. Что такое shagreen$74263$
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Что (кто) такое shagreen$74263$ - определение

RARE MULTISYSTEM GENETIC DISEASE THAT CAUSES BENIGN TUMORS TO GROW IN THE BRAIN AND ON OTHER VITAL ORGANS
Tuberous Sclerosis; Bourneville-Pringle disease; Bourneville's disease; Tuberous scelerosis; Tuberous Scelerosis; Sclerosis tuberosa; T.S.C.; Tuberous sclerosis complex; Bourneville disease; Bourneville phakomatosis; Shagreen lesions; Epiloia; Bourneville-Pringle syndrome; Tuberous Sclerosis, type 1; Tuberous Sclerosis, type 2; Intraepithelial epithelioma; Subependymal nodule; Tuberous sclerosis type 2; Tuberous sclerosis type 1; Bourneville–Pringle disease; Tuberous Sclerosis Complex
  • Computed tomography showing multiple angiomyolipomas of the kidney in a patient with lung lymphangioleiomyomatosis on CT: suspected TSC
  • TSC is inherited in an [[autosomal dominant]] fashion.
  • [[Désiré-Magloire Bourneville]]
  • From top to bottom: Hypopigmented macules, Shagreen patch and periungual fibroma of tuberous sclerosis.
  • A case of tuberous sclerosis showing facial angiofibromas in characteristic butterfly pattern.
  •  publisher = Mac Keith Press }}</ref>
  • TSC in MRI

shagreen         
  • Two small decorative elephants made of silver and shagreen.
FISH LEATHER
Galuchat; Shark skin; Chagrin (leather); Shark leather
[?a'gri:n]
¦ noun
1. sharkskin used for decoration or as an abrasive.
2. untanned leather with a rough granulated surface.
Origin
C17: var. of chagrin in the sense 'rough skin'.
Shagreen         
  • Two small decorative elephants made of silver and shagreen.
FISH LEATHER
Galuchat; Shark skin; Chagrin (leather); Shark leather
·vt To Chagrin.
II. Shagreen ·adj ·Alt. of Shagreened.
III. Shagreen ·noun The skin of various small sharks and other fishes when having small, rough, bony scales. The dogfishes of the genus Scyllium furnish a large part of that used in the arts.
IV. Shagreen ·noun A kind of untanned leather prepared in Russia and the East, from the skins of horses, asses, and camels, and grained so as to be covered with small round granulations. This characteristic surface is produced by pressing small seeds into the grain or hair side when moist, and afterward, when dry, scraping off the roughness left between them, and then, by soaking, causing the portions of the skin which had been compressed or indented by the seeds to swell up into relief. It is used for covering small cases and boxes.
The Shagreen Bone         
  • [[Menuet]] from the opera-[[entr'acte]]<br> ''The Shagreen Bone''}}
OPERA
The Shagreen Bone (, ) Op. 37-38 (1990) is a full-length ballet in three acts and an opera entr'acte in one act by Yuri Khanon, to a libretto by the composer based on Balzac's 1831 novel The Shagreen Skin.

Википедия

Tuberous sclerosis

Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain and on other vital organs such as the kidneys, heart, liver, eyes, lungs and skin. A combination of symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung disease, and kidney disease.

TSC is caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin, respectively, with TSC2 mutations accounting for the majority and tending to cause more severe symptoms. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation.

Prognosis is highly variable and depends on the symptoms, but life expectancy is normal for many.

The prevalence of the disease is estimated to be 7 to 12 in 100,000. The disease is often abbreviated to tuberous sclerosis, which refers to the hard swellings in the brains of patients, first described by French neurologist Désiré-Magloire Bourneville in 1880.