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Nestor-Guillermo Progeria Syndrome

Disease ID: disease_node_19959

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DbxrefGARD:11008, MIM:614008, ORDO:280576
SubclassofDOID_0081332, DOID_0050737
Data SourceDOID
SynonymsProgeria syndrome, childhood-onset, with osteolysis
Doid LabelNestor-Guillermo progeria syndrome
Doid DescriptionA progeroid syndrome that is characterized by lipoatrophy, osteoporosis, and very severe osteolysis. Patients have no cardiovascular impairment, diabetes mellitus, or hypertriglyceridemia, but suffer profound skeletal abnormalities that affect their quality of life and that has_material_basis_in homozygous mutation in the BANF1 gene on chromosome 11q13. Onset is after 2 years of age.
Existence Starts DuringHP_0011463
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_19959
Doid IdDOID_0081334
LabelNestor-Guillermo Progeria Syndrome