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Methemoglobinemia And Ambiguous Genitalia

Disease ID: disease_node_17165

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DbxrefMIM:250790
SubclassofDOID_1923, DOID_0050737
Data SourceDOID
SynonymsMETAG, methemoglobinemia due to deficiency of cytochrome b5, methemoglobinemia type IV, pure isolated 17,20-lyase deficiency
Doid Labelmethemoglobinemia and ambiguous genitalia
Doid DescriptionA disorder of sexual development characterized by severely reduced 17,20-lyase activity of CYP17A1, sex steroid deficiency with no deficiency in glucocorticoid and mineralocorticoid reserves, absent or disturbed pubertal development, and mild to severe methemoglobinemia that has_material_basis_in homozygous or compound heterozygous mutation in the CYB5A gene on chromosome 18q22.3.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_17165
Doid IdDOID_0112316
LabelMethemoglobinemia And Ambiguous Genitalia