Familial idiopathic pulmonary fibrosis in association with bone marrow hypoplasia and hepatic nodular regenerative hyperplasia: a new “trimorphic” syndrome

This is the first report of familial idiopathic pulmonary fibrosis associated with hepatic nodular regenerative hyperplasia and bone marrow hypoplasia. Four members of one family presented with this triad of organ dysfunction. The response to immunosuppressive treatment was poor and all four members...

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Bibliographic Details
Published in:Thorax 2009-05, Vol.64 (5), p.440-443
Main Authors: Talbot-Smith, A, Syn, W-K, MacQuillan, G, Neil, D, Elias, E, Ryan, P
Format: Article
Language:English
Subjects:
Adult
Aged
Biological and medical sciences
Biopsy
Bone marrow
Bone Marrow - abnormalities
Cardiology. Vascular system
Dyspnea
Enzymes
Fatal Outcome
Focal Nodular Hyperplasia - genetics
Gastroenterology. Liver. Pancreas. Abdomen
Histology
Humans
Hyperplasia
Hypertension
Idiopathic Pulmonary Fibrosis - genetics
Liver
Liver Regeneration
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Male
Medical sciences
Middle Aged
Other diseases. Semiology
Pedigree
Pneumology
Pulmonary fibrosis
Respiratory system : syndromes and miscellaneous diseases
Serology
Syndrome
Ultrasonic imaging
Veins & arteries
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Description
Summary:This is the first report of familial idiopathic pulmonary fibrosis associated with hepatic nodular regenerative hyperplasia and bone marrow hypoplasia. Four members of one family presented with this triad of organ dysfunction. The response to immunosuppressive treatment was poor and all four members succumbed to the disease processes. The current literature is reviewed and mechanisms that could have been involved in the development of this new syndrome are proposed.
ISSN:0040-6376
1468-3296
DOI:10.1136/thx.2008.099796