serw-MX  [xml]  
 


    
 DeCS Categories

C08 Respiratory Tract Diseases .
C08.618 Respiration Disorders .
C08.618.490 Hoarseness .
C08.618.490.500 Lipoid Proteinosis of Urbach and Wiethe .
C10 Nervous System Diseases .
C10.228 Central Nervous System Diseases .
C10.228.140 Brain Diseases .
C10.228.140.163 Brain Diseases, Metabolic .
C10.228.140.163.100 Brain Diseases, Metabolic, Inborn .
C10.228.140.163.100.435 Lysosomal Storage Diseases, Nervous System .
C10.228.140.163.100.435.825 Sphingolipidoses .
C10.228.140.163.100.435.825.400 Gaucher Disease .
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities .
C16.320 Genetic Diseases, Inborn .
C16.320.565 Metabolism, Inborn Errors .
C16.320.565.189 Brain Diseases, Metabolic, Inborn .
C16.320.565.189.435 Lysosomal Storage Diseases, Nervous System .
C16.320.565.189.435.825 Sphingolipidoses .
C16.320.565.189.435.825.400 Gaucher Disease .
C16.320.565.398 Lipid Metabolism, Inborn Errors .
C16.320.565.398.641 Lipidoses .
C16.320.565.398.641.803 Sphingolipidoses .
C16.320.565.398.641.803.441 Gaucher Disease .
C16.320.565.595 Lysosomal Storage Diseases .
C16.320.565.595.554 Lysosomal Storage Diseases, Nervous System .
C16.320.565.595.554.825 Sphingolipidoses .
C16.320.565.595.554.825.400 Gaucher Disease .
C16.320.850 Skin Diseases, Genetic .
C16.320.850.595 Lipoid Proteinosis of Urbach and Wiethe .
C18 Nutritional and Metabolic Diseases .
C18.452 Metabolic Diseases .
C18.452.132 Brain Diseases, Metabolic .
C18.452.132.100 Brain Diseases, Metabolic, Inborn .
C18.452.132.100.435 Lysosomal Storage Diseases, Nervous System .
C18.452.132.100.435.825 Sphingolipidoses .
C18.452.132.100.435.825.400 Gaucher Disease .
C18.452.584 Lipid Metabolism Disorders .
C18.452.584.687 Lipidoses .
C18.452.584.687.803 Sphingolipidoses .
C18.452.584.687.803.441 Gaucher Disease .
C18.452.648 Metabolism, Inborn Errors .
C18.452.648.189 Brain Diseases, Metabolic, Inborn .
C18.452.648.189.435 Lysosomal Storage Diseases, Nervous System .
C18.452.648.189.435.825 Sphingolipidoses .
C18.452.648.189.435.825.400 Gaucher Disease .
C18.452.648.398 Lipid Metabolism, Inborn Errors .
C18.452.648.398.641 Lipidoses .
C18.452.648.398.641.803 Sphingolipidoses .
C18.452.648.398.641.803.441 Gaucher Disease .
C18.452.648.595 Lysosomal Storage Diseases .
C18.452.648.595.554 Lysosomal Storage Diseases, Nervous System .
C18.452.648.595.554.825 Sphingolipidoses .
C18.452.648.595.554.825.400 Gaucher Disease .
D02 Organic Chemicals .
D02.065 Amides .
D02.065.277 Benzamides .
D02.065.277.067 Bezafibrate .
D02.241 Carboxylic Acids .
D02.241.081 Acids, Acyclic .
D02.241.081.114 Butyrates .
D02.241.081.114.968 Isobutyrates .
D02.241.081.114.968.500 Fibric Acids .
D02.241.081.114.968.500.249 Bezafibrate .
D02.241.223 Acids, Carbocyclic .
D02.241.223.100 Benzoates .
D02.241.223.100.100 Benzamides .
D02.241.223.100.100.120 Bezafibrate .
D02.241.223.100.200 Chlorobenzoates .
D02.241.223.100.200.249 Bezafibrate .
D02.355 Ethers .
D02.355.726 Phenyl Ethers .
D02.355.726.305 Fibric Acids .
D02.355.726.305.249 Bezafibrate .
D02.455 Hydrocarbons .
D02.455.426 Hydrocarbons, Cyclic .
D02.455.426.559 Hydrocarbons, Aromatic .
D02.455.426.559.389 Benzene Derivatives .
D02.455.426.559.389.127 Benzoates .
D02.455.426.559.389.127.085 Benzamides .
D02.455.426.559.389.127.085.101 Bezafibrate .
D02.455.426.559.389.127.250 Chlorobenzoates .
D02.455.426.559.389.127.250.249 Bezafibrate .
D02.455.426.559.389.657 Phenols .
D02.455.426.559.389.657.654 Phenyl Ethers .
D02.455.426.559.389.657.654.305 Fibric Acids .
D02.455.426.559.389.657.654.305.249 Bezafibrate .
 
 Terms
 Synonyms & Historicals
Documents
LILACS e MDL
 
Lipidoses .
Lipoidosis .
Lipidosis .
Lipoidoses .
Conditions characterized by abnormal lipid deposition due to disturbance in lipid metabolism, such as hereditary diseases involving lysosomal enzymes required for lipid breakdown. They are classified either by the enzyme defect or by the type of lipid involved. .
1.00
182576
 
Gaucher Disease .
Acid beta-Glucosidase Deficiency .
Acid beta-Glucosidase Deficiency Disease .
Acute Neuronopathic Gaucher Disease .
Chronic Gaucher Disease .
GBA Deficiency .
Gaucher Disease Type 3 .
Gaucher Disease, Acute Neuronopathic .
Gaucher Disease, Acute Neuronopathic Type .
Gaucher Disease, Chronic .
Gaucher Disease, Chronic Neuronopathic Type .
Gaucher Disease, Infantile .
Gaucher Disease, Infantile Cerebral .
Gaucher Disease, Juvenile .
Gaucher Disease, Juvenile and Adult, Cerebral .
Gaucher Disease, Neuronopathic .
Gaucher Disease, Non-Neuronopathic Form .
Gaucher Disease, Noncerebral Juvenile .
Gaucher Disease, Subacute Neuronopathic Form .
Gaucher Disease, Subacute Neuronopathic Type .
Gaucher Disease, Type 1 .
Gaucher Disease, Type 2 .
Gaucher Disease, Type 3 .
Gaucher Disease, Type I .
Gaucher Disease, Type II .
Gaucher Disease, Type III .
Gaucher Splenomegaly .
Gaucher Syndrome .
Gaucher's Disease .
Gauchers Disease .
Glucocerebrosidase Deficiency .
Glucocerebrosidosis .
Glucosyl Cerebroside Lipidosis .
Glucosylceramidase Deficiency .
Glucosylceramide Beta-Glucosidase Deficiency .
Glucosylceramide Lipidosis .
Infantile Gaucher Disease .
Kerasin Histiocytosis .
Kerasin Lipoidosis .
Kerasin thesaurismosis .
Lipoid Histiocytosis (Kerasin Type) .
Non-Neuronopathic Gaucher Disease .
Subacute Neuronopathic Gaucher Disease .
Type 1 Gaucher Disease .
Type 2 Gaucher Disease .
Type 3 Gaucher Disease .
Cerebroside Lipidoses, Glucosyl .
Cerebroside Lipidosis Syndromes .
Cerebroside Lipidosis, Glucosyl .
Deficiencies, GBA .
Deficiencies, Glucocerebrosidase .
Deficiency Disease, Glucocerebrosidase .
Deficiency Diseases, Glucocerebrosidase .
Deficiency, GBA .
Deficiency, Glucocerebrosidase .
Disease, Chronic Gaucher .
Disease, Gaucher .
Disease, Gaucher's .
Disease, Gauchers .
Disease, Glucocerebrosidase Deficiency .
Disease, Infantile Gaucher .
Disease, Juvenile Gaucher .
Disease, Neuronopathic Gaucher .
Disease, Non-Neuronopathic Gaucher .
Diseases, Gauchers .
Diseases, Glucocerebrosidase Deficiency .
GBA Deficiencies .
Gaucher Disease, Non Neuronopathic Form .
Gaucher Disease, Non-Neuronopathic .
Gauchers Diseases .
Glucocerebrosidase Deficiencies .
Glucocerebrosidase Deficiency Diseases .
Glucocerebrosidoses .
Glucosyl Cerebroside Lipidoses .
Glucosylceramide Lipidoses .
Histiocytoses, Kerasin .
Histiocytoses, Lipoid (Kerasin Type) .
Histiocytosis, Kerasin .
Histiocytosis, Lipoid (Kerasin Type) .
Juvenile Gaucher Disease .
Kerasin Histiocytoses .
Kerasin Lipoidoses .
Kerasin thesaurismoses .
Lipidoses, Glucosyl Cerebroside .
Lipidoses, Glucosylceramide .
Lipidosis Syndrome, Cerebroside .
Lipidosis Syndromes, Cerebroside .
Lipidosis, Glucosyl Cerebroside .
Lipidosis, Glucosylceramide .
Lipoid Histiocytoses (Kerasin Type) .
Lipoidoses, Kerasin .
Lipoidosis, Kerasin .
Non Neuronopathic Gaucher Disease .
Splenomegaly, Gaucher .
Syndrome, Cerebroside Lipidosis .
Syndrome, Gaucher .
Syndromes, Cerebroside Lipidosis .
thesaurismoses, Kerasin .
thesaurismosis, Kerasin .
Cerebroside Lipidosis Syndrome .
Glucocerebrosidase Deficiency Disease .
Glucosylceramide Beta-Glucosidase Deficiency Disease .
Neuronopathic Gaucher Disease .
Gaucher Disease Type 1 .
Gaucher Disease Type 2 .
GAUCHER'S DISEASE .
An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement. .
0.72
833964
 
Lipoid Proteinosis of Urbach and Wiethe .
Hyalinosis Cutis et Mucosae .
Lipoidproteinosis .
Urbach-Wiethe Lipoid Proteinosis .
Urbach-Wiethe Syndrome .
Lipoid Proteinosis, Urbach-Wiethe .
Urbach Wiethe Disease .
Urbach Wiethe Lipoid Proteinosis .
Lipoproteinosis .
Urbach-Wiethe Disease .
Lipoidproteinosis .
An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN. .
0.64
23346
 
Bezafibrate .
Azufibrat .
BM-15.075 .
Befibrat .
Beza-Lande .
Beza-Puren .
Bezabeta .
Bezacur .
Bezafibrat PB .
Bezafisal .
Bezalip .
Bezamerck .
Béfizal .
Cedur .
Difaterol .
Eulitop .
Lipox .
Reducterol .
Regadrin B .
Sklerofibrat .
Solibay .
durabezur .
BM 15.075 .
BM15.075 .
Beza Lande .
Beza Puren .
An antilipemic agent that lowers CHOLESTEROL and TRIGLYCERIDES. It decreases LOW DENSITY LIPOPROTEINS and increases HIGH DENSITY LIPOPROTEINS. .
0.63
251134