Ornithine Carbamoyltransferase Deficiency Disease [nemoc z nedostatku ornithinkarbamoyltransferázy]

topical
19
Terms

deficit ornitintranskarbamoylázy
deficit OTC
ornithinkarbamoyltransferasa - nedostatek
ornithintranskarbamylasa - nedostatek

 

Deficiency Disease, Ornithine Carbamoyltransferase
Deficiency Disease, Ornithine Transcarbamylase
Ornithine Carbamoyltransferase Deficiency
Ornithine Transcarbamylase Deficiency
Ornithine Transcarbamylase Deficiency Disease
Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To
OTC Deficiency

Persistent link   https://www.medvik.cz/link/D020163
Definition

An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)

DUI
D020163 MeSH Browser
CUI
M0328321
Previous indexing
Ornithine Carbamoyltransferase/deficiency (1966-1999)
History note
2000
Public note
2000

C Diseases
C10.228.140 Brain Diseases 1 178
C10.228.140.163 Brain Diseases, Metabolic 84
C10.228.140.163.100.937 Urea Cycle Disorders, Inborn 7
C10.228.140.163.100.937.124 Argininosuccinic Aciduria 2
C10.228.140.163.100.937.374 Citrullinemia 2
C10.228.140.163.100.937.500 Hyperargininemia 2
C16.320.322.030 Aicardi Syndrome
C16.320.322.068 Barth Syndrome 6
C16.320.322.092 Choroideremia 3
C16.320.322.100 Dent Disease 1
C16.320.322.108 Dyskeratosis Congenita 9
C16.320.322.124 Fabry Disease 187
C16.320.322.186 Focal Dermal Hypoplasia 6
C16.320.322.235 Hemophilia B 214
C16.320.322.241 Ichthyosis, X-Linked 9
C16.320.322.562 Muscular Dystrophy, Duchenne 133
C16.320.322.937 Wiskott-Aldrich Syndrome 20
C16.320.565.100.940 Urea Cycle Disorders, Inborn 7
C16.320.565.100.940.124 Argininosuccinic Aciduria 2
C16.320.565.100.940.374 Citrullinemia 2
C16.320.565.100.940.500 Hyperargininemia 2
C16.320.565.189.937 Urea Cycle Disorders, Inborn 7
C16.320.565.189.937.124 Argininosuccinic Aciduria 2
C16.320.565.189.937.374 Citrullinemia 2
C16.320.565.189.937.500 Hyperargininemia 2
C18.452 Metabolic Diseases 1 196
C18.452.132.100.937 Urea Cycle Disorders, Inborn 7
C18.452.132.100.937.124 Argininosuccinic Aciduria 2
C18.452.132.100.937.374 Citrullinemia 2
C18.452.132.100.937.437 Hyperargininemia 2
C18.452.648.100.940 Urea Cycle Disorders, Inborn 7
C18.452.648.100.940.124 Argininosuccinic Aciduria 2
C18.452.648.100.940.374 Citrullinemia 2
C18.452.648.100.940.437 Hyperargininemia 2
C18.452.648.189.937 Urea Cycle Disorders, Inborn 7
C18.452.648.189.937.124 Argininosuccinic Aciduria 2
C18.452.648.189.937.374 Citrullinemia 2
C18.452.648.189.937.437 Hyperargininemia 2