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MedGen for PubMed (Select 23110262)

Items: 3

1.

Idiopathic fibrosing alveolitis, chronic form

Familial pulmonary fibrosis (FPF in this GeneReview) is defined as idiopathic interstitial pneumonia (IIP) in two or more first-degree relatives (parent, sib, or offspring). Up to 20% of cases of IIP cluster in families. The clinical findings of IIP are bibasilar reticular abnormalities, ground glass opacities, or diffuse nodular lesions on high-resolution computed tomography and abnormal pulmonary function studies that include evidence of restriction (reduced VC with an increase in FEV1/FVC ratio) and/or impaired gas exchange (increased P(A-a)O2 with rest or exercise or decreased diffusion capacity of the lung for carbon monoxide [DLCO]). FPF usually presents between ages 50 and 70 years. FPF may be complicated by lung cancer; bronchoalveolar cell carcinoma, small-cell carcinoma, and adenocarcinoma have been described. [from GeneReviews]

MedGen UID:
321462
Concept ID:
C1800706
Disease or Syndrome
2.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a nonneoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known cause. [from ORDO]

MedGen UID:
832773
Concept ID:
CN200819
Finding
3.

Pulmonary fibrosis

Replacement of normal lung tissues are progressively replaced by fibroblasts and collagen. [from HPO]

MedGen UID:
505156
Concept ID:
CN002000
Finding
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