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Pulmonary Venoocclusive Disease 2

Disease ID: disease_node_13240

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DbxrefMIM:234810
SubclassofDOID_5453, DOID_0050737
Data SourceDOID
SynonymsFAMILIAL PULMONARY CAPILLARY HEMANGIOMATOSIS
Doid Labelpulmonary venoocclusive disease 2
Doid DescriptionA pulmonary venoocclusive disease that has_material_basis_in homozygous or compound heterozygous mutation in the EIF2AK4 gene on chromosome 15q15 and that is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules, and is frequently associated with pulmonary capillary dilatation and proliferation.
Has Material Basis InGENO_0000148
Disease Node Iddisease_node_13240
Doid IdDOID_0081269
LabelPulmonary Venoocclusive Disease 2