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Hyaline Fibromatosis Syndrome

Disease ID: disease_node_12420

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DbxrefMESH:D057770, MIM:228600, NCI:C98297, ORDO:498474, SNOMEDCT_US_2023_03_01:238861002, UMLS_CUI:C2745948
SubclassofDOID_0050737, DOID_65
Data SourceDOID, MESH
SynonymsHFS, inherited systemic hyalinosis, puretic syndrome, systemic hyalinosis
Mesh IdD057770
Mesh LabelHyaline Fibromatosis Syndrome
Mesh SubclassofD012873
Doid Labelhyaline fibromatosis syndrome
Doid DescriptionA connective tissue disease characterized by abnormal growth of hyalinized fibrous tissue especially around the subcutaneous regions on the scalp, ears, neck, face, hands, and feet, gingival hypertrophy, joint contractures, and osteolytic bone lesions that has_material_basis_in homozygous or compound heterozygous mutation in the ANTXR2 gene on chromosome 4q21.21.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_12420
Doid IdDOID_0111669
LabelHyaline Fibromatosis Syndrome