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HUMAN PHENOTYPE - ANNOTATIONS

The Human Phenotype Ontology (HPO) is downloaded weekly from http://compbio.charite.de/hudson/job/hpo/lastStableBuild/artifact/ontology/release/hp.obo. The file downloaded is considered the "last stable build" available for the ontology. For more about the HPO, view their website at http://www.human-phenotype-ontology.org/.

Term:Pulmonary lymphangiomyomatosis
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Accession:HP:0012798 term browser browse the term
Definition:Infiltration of smooth muscle-like cells in lymph vessels as well as the lung (pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum). Focal emphysema can develop because of airway narrowing, and the thoracic duct may be obliterated. Pulmonary lymphangiomyomatosis may lead to multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls.
Comment:Pulmonary lymphangiomyomatosis is a manifestation of lymphangioleiomyomatosis, which may also affect extrapulmonary locations. It can be seen in tuberous sclerosis.
Synonyms:exact_synonym: Pulmonary myomatosis
 related_synonym: Lymphangioleiomyomatosis
 xref: MESH:D018192;   SNOMEDCT_US:73017001;   UMLS:C0238399;   UMLS:C0751674



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Term paths to the root
Path 1
Term Annotations click to browse term
  Human phenotype 0
    Phenotypic abnormality 0
      Abnormality of the respiratory system 0
        Neoplasm of the respiratory system 0
          Neoplasm of the lung 0
            Pulmonary lymphangiomyomatosis 0
Path 2
Term Annotations click to browse term
  Human phenotype 0
    Phenotypic abnormality 0
      Abnormality of the cardiovascular system 0
        Abnormality of the vasculature 0
          Abnormality of the lymphatic system 0
            Abnormal lymphatic vessel morphology 0
              Lymphatic vessel neoplasm 0
                Pulmonary lymphangiomyomatosis 0
paths to the root