Lipoids

Lipoids (reticuloendotheliosis) is a group of diseases, which are based on specific congenital disorders of lipid metabolism (see Hereditary diseases). The most studied diseases in this group are Niemann - pick disease (see), Gaucher disease (see), amaurocichla family idiocy (illness, and Tay - Sachs), the disease Hand - shyullera - Christian. These diseases are caused by a pathological accumulation of some lipids (Terezin, sphingolipids and others) in the cells of reticuloendothelial and the Central nervous system due to a defective enzyme systems.
Lipoids occur mainly in children. The etiology of lipidosis not clear. The hereditary disease that is transmitted in an autosomal recessive type (see Hereditary diseases), observed in children whose parents are blood relations.
Major clinical manifestations of lipidosis are stunted, gipogenitalizm (see), mental underdevelopment. The clinical picture is very early, there is an increase liver, spleen, lymph nodes, and secondary anemia. Often the disease is manifested skin changes: urticarnae, papular-maloletnie rash. Pathogenetic therapy of lipidosis not currently exist. The adverse outcomes.

Lipoids is a group of diseases characterized by disturbance of lipoid metabolism and related diseases accumulation. The nature of lipids accumulated in the cytoplasm of the cell, these diseases are divided into xanthomatose (cytoplasm contains cholesterol and cholesterol esters) and sphingolipidosis (cytoplasm contains compounds complex lipids). When lipovtsah is intracellular lipid metabolism due to a defective enzyme systems with the subsequent deposition of certain groups of lipids in the cell cytoplasm.
Violation of cholesterol metabolism, mainly by the deposition of cholesterol, cholesterol-esters and neutral fats within the cells of the reticuloendothelial system (xanthomas) occurs in diseases of the group of histiocytosis X[Hand - shyullera - Christian disease (see), in a small degree Letterer - Ziv disease (see), as well as transitional forms between them]. For histiocytosis X (synonym to retico-loandeals) is characterized by systemic granulomatous lesions of the connective tissue (see Langerhans). Sick children up to 9-10 years. Diseases are not hereditary in nature. The etiology is not clear. Still unresolved is the question: are these diseases by Wegener with secondary accumulation of lipids or the accumulation of lipids is the result of violation of intracellular metabolism.
Violation of lipid metabolism containing sphingosine, typical for disease groups sphingolipidoses (tourismou). Sphingolipid relate to complex lipids and are divided into photosynthesize and glimepiride. To postsymposium owns all of sphingolipids containing phosphorus, including sphingomyelin, glycosphingolipids - all sphingolipids that contain carbohydrate groups, including the cerebrosides and gangliosides. When the metabolic certain groups of sphingolipids are developing different kinds of diseases.
The preferential accumulation of sphingomyelin marked with Niemann - pick disease (see), and marvel at the epithelial, reticular and ganglion cells. With Gaucher disease (see) is observed accumulation in reticuloendothelial cells glucosteroids - Karazina and glucocerebroside. When illness Tay - Sachs is an accumulation of gangliosides mainly in the cells of the Central nervous system. When gargarismi (see) in metabolic glucosteroids in nerve cells and the accumulation of mucopolysaccharides in the connective tissue.
Sphingolipidosis usually congenital disease, often family, inherited recessive. Their etiology is not clear.
Perhaps they are a consequence of genetically caused failure of various enzymes. They are more common in genetically isolated villages, affecting mainly children and young people.