vrozené poruchy metabolismu [Metabolism, Inborn Errors]

tematický
711
Termíny

dědičné metabolické poruchy
dědičné poruchy metabolismu
metabolismus - vrozené poruchy
vrozené metabolické poruchy

 

Inborn Errors of Metabolism
Metabolism Errors, Inborn

Perzistentní odkaz   https://www.medvik.cz/link/D008661
Definice

Poruchy metabolických procesů vyvolané genetickými mutacemi, které jsou dědičné nebo získané in utero.

Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.

Anotace
general or unspecified; prefer specific types; differentiate from DEFICIENCY DISEASES
DUI
D008661 MeSH Prohlížeč
CUI
M0013496
Historická pozn.
65
Veřejná pozn.
65

C Nemoci
C16.320.051 Alagillův syndrom 19
C16.320.100 Brugadův syndrom 40
C16.320.129 CADASIL 8
C16.320.165 CHARGE syndrom 1
C16.320.170 cherubismus 1
C16.320.184 ciliopatie 9
C16.320.188 Costellův syndrom 3
C16.320.190 cystická fibróza 1 328
C16.320.215 Donohueův syndrom 1
C16.320.240 nanismus 97
C16.320.306 syndrom Frasier
C16.320.314 deficit GATA2 1
C16.320.365 hemoglobinopatie 68
C16.320.467 Kallmannův syndrom 10
C16.320.488 laminopatie 2
C16.320.540 Marfanův syndrom 105
C16.320.565.176 familiární amyloidóza 16
C16.320.565.663 peroxizomální poruchy 20
C16.320.565.753 progerie 27
C16.320.577 svalové dystrofie 140
C16.320.728 osteochondrodysplazie 123
C16.320.812 pyknodysostóza 2
C16.320.925 Wernerův syndrom 14
C18.452 metabolické nemoci 1 196
C18.452.284 poruchy opravy DNA 10
C18.452.479 hyperlaktatemie 5
C18.452.625 metabolický syndrom 1 988
C18.452.648.176 familiární amyloidóza 16
C18.452.648.663 peroxizomální poruchy 20
C18.452.648.753 progerie 27
C18.452.811 porfyrie 252
C18.452.915 syndrom chřadnutí 13

3-Hydroxy-3-Methylglutaryl-CoA Synthase 2 Deficiency Disease MeSH Prohlížeč

3-Hydroxyacyl-CoA Dehydrogenase Deficiency Disease MeSH Prohlížeč

3-Methylglutaconic Aciduria Disease MeSH Prohlížeč

3-Methylglutaconic Aciduria Type IV Disease MeSH Prohlížeč

3-Methylglutaconic Aciduria, Type I Disease MeSH Prohlížeč

3-Methylglutaconic Aciduria, Type V Disease MeSH Prohlížeč

5-Nucleotidase syndrome Disease MeSH Prohlížeč

6-Phosphogluconolactonase Deficiency Disease MeSH Prohlížeč

Acetylcarnitine deficiency Disease MeSH Prohlížeč

Acholinesterasemia Disease MeSH Prohlížeč

Acid Phosphatase Deficiency Disease MeSH Prohlížeč

Adenine phosphoribosyltransferase deficiency Disease MeSH Prohlížeč

Amobarbital, Deficient N-Hydroxylation of Disease MeSH Prohlížeč

Arene Oxide Detoxification Defect Disease MeSH Prohlížeč

Aromatase deficiency Disease MeSH Prohlížeč

Aryl Hydrocarbon Hydroxylase Inducibility Disease MeSH Prohlížeč

Butyrylcholinesterase Deficiency, Fluoride-Resistant, Japanese Type Disease MeSH Prohlížeč

Butyrylcholinesterase deficiency Disease MeSH Prohlížeč

Carnitine Acetyltransferase Deficiency Disease MeSH Prohlížeč

Carnitine palmitoyl transferase 2 deficiency Disease MeSH Prohlížeč

Chromate Resistance Disease MeSH Prohlížeč

Combined Malonic and Methylmalonic Aciduria Disease MeSH Prohlížeč

Combined Oxidative Phosphorylation Deficiency 1 Disease MeSH Prohlížeč

Combined Oxidative Phosphorylation Deficiency 4 Disease MeSH Prohlížeč

Combined Oxidative Phosphorylation Deficiency 5 Disease MeSH Prohlížeč

Congenital chloride diarrhea Disease MeSH Prohlížeč

Copper deficiency, familial benign Disease MeSH Prohlížeč

Costeff optic atrophy syndrome Disease MeSH Prohlížeč

Coumarin Resistance Disease MeSH Prohlížeč

Coumarin Sensitivity Disease MeSH Prohlížeč

Deafness hyperuricemia neurologic ataxia Disease MeSH Prohlížeč

Deoxyribose-5-Phosphate Aldolase Deficiency Disease MeSH Prohlížeč

Diarrhea 3, Secretory Sodium, Congenital Disease MeSH Prohlížeč

Diarrhea, Glucose-Stimulated Secretory, with Common Variable Immunodeficiency Disease MeSH Prohlížeč

Dihydropyrimidinase Deficiency Disease MeSH Prohlížeč

Diphenylhydantoin, Defect in Hydroxylation of Disease MeSH Prohlížeč

Drug Metabolism, Poor, CYP2C19-Related Disease MeSH Prohlížeč

Drug Metabolism, Poor, CYP2D6-Related Disease MeSH Prohlížeč

Dystonia, Dopa-Responsive, due to Sepiapterin Reductase Deficiency Disease MeSH Prohlížeč

Enterokinase Deficiency Disease MeSH Prohlížeč

Ethanolaminosis Disease MeSH Prohlížeč

Finnish lethal neonatal metabolic syndrome Disease MeSH Prohlížeč

Fumaric aciduria Disease MeSH Prohlížeč

Glucocorticoid Receptor Deficiency Disease MeSH Prohlížeč

Glutamate formiminotransferase deficiency Disease MeSH Prohlížeč

Glycoprotein Storage Disease Disease MeSH Prohlížeč

Glyoxalase II Deficiency Disease MeSH Prohlížeč

Growth Factors, Combined Defect of Disease MeSH Prohlížeč

Hyaluronan Metabolism, Defect in Disease MeSH Prohlížeč

Hypercalcemia, Idiopathic, of Infancy Disease MeSH Prohlížeč

Hypoadiponectinemia Disease MeSH Prohlížeč

Hypokalemia, Familial Disease MeSH Prohlížeč

Hypoproteinemia, Hypercatabolic Disease MeSH Prohlížeč

Inosine Triphosphatase Deficiency Disease MeSH Prohlížeč

Intrinsic Factor and R Binder, Combined Congenital Deficiency of Disease MeSH Prohlížeč

Kallikrein, Decreased Urinary Activity of Disease MeSH Prohlížeč

L-Gulonolactone Oxidase, Nonfunctional Disease MeSH Prohlížeč

Lactate Dehydrogenase B Deficiency Disease MeSH Prohlížeč

Lactic Aciduria due to D-Lactic Acid Disease MeSH Prohlížeč

Leukotriene C4 Synthase Deficiency Disease MeSH Prohlížeč

Malonic aciduria Disease MeSH Prohlížeč

Mannose 6-Phosphate Receptor Recognition Defect, Lebanese Type Disease MeSH Prohlížeč

Mannose-Binding Protein Deficiency Disease MeSH Prohlížeč

Medium Chain 3-Ketoacyl-CoA Thiolase Deficiency Disease MeSH Prohlížeč

Methemoglobin Reductase Deficiency Disease MeSH Prohlížeč

Methylcobalamin Deficiency, CblG Type Disease MeSH Prohlížeč

Methylmalonyl-Coenzyme A mutase deficiency Disease MeSH Prohlížeč

Mitochondrial Complex II Deficiency Disease MeSH Prohlížeč

Mitochondrial Dna Depletion Syndrome, Encephalomyopathic Form, With Methylmalonic Aciduria, Autosomal Recessive Disease MeSH Prohlížeč

Myeloperoxidase Deficiency Disease MeSH Prohlížeč

N acetyltransferase deficiency Disease MeSH Prohlížeč

Pancreatic Insufficiency, Combined Exocrine Disease MeSH Prohlížeč

Phenacetin O-Deethylase, Deficiency of Disease MeSH Prohlížeč

Phenol sulfotransferase deficiency Disease MeSH Prohlížeč

Phosphoglycerate Kinase 1 Deficiency Disease MeSH Prohlížeč

Proguanil, Poor Metabolism of Disease MeSH Prohlížeč

Retinol-Binding Protein Deficiency Disease MeSH Prohlížeč

Stomatocytosis I Disease MeSH Prohlížeč

Stomatocytosis II Disease MeSH Prohlížeč

Succinic Acidemia Disease MeSH Prohlížeč

Transcobalamin I Deficiency Disease MeSH Prohlížeč

Trimethylaminuria Disease MeSH Prohlížeč

Warfarin Sensitivity Disease MeSH Prohlížeč

Weinstein Kliman Scully syndrome Disease MeSH Prohlížeč

Wiedemann Oldigs Oppermann syndrome Disease MeSH Prohlížeč

Xanthinuria, Type I Disease MeSH Prohlížeč

alpha-Fetoprotein Deficiency Disease MeSH Prohlížeč

familial gynecomastia, due to increased aromatase activity Disease MeSH Prohlížeč