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Smith-Magenis Syndrome

Disease ID: disease_node_12534

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DbxrefGARD:8197, ICD10CM:Q93.5, MESH:D058496, MIM:182290, ORDO:819
SubclassofDOID_0060388
Data SourceDOID, MESH
Synonyms17p11.2 microdeletion syndrome, chromosome 17p11.2 deletion syndrome
Mesh IdD058496
Mesh LabelSmith-Magenis Syndrome
Mesh SubclassofD000015, D025063, D021081
Doid LabelSmith-Magenis syndrome
Doid DescriptionA chromosomal deletion syndrome that is characterized by mild-to-moderate infantile hypotonia, minor skeletal anomalies, prepubertal short stature, brachydactyly, ophthalmologic and otolaryngologic abnormalities, peripheral neuropathy, developmental delay, cognitive impairment, and behavioral abnormalities that has_material_basis_in a 3.7-Mb interstitial deletion in chromosome 17p11.2 or sometimes by mutations in the RAI1 gene in the same region.
Disease Node Iddisease_node_12534
Doid IdDOID_0060768
LabelSmith-Magenis Syndrome