Pulmonary venoocclusive disease: diagnosis during life in 4 patients

Justo, R. N., Dare, A. J., Whight, C. M. and Radford, D. J. (1993) Pulmonary venoocclusive disease: diagnosis during life in 4 patients. Archives of Disease in Childhood, 68 1: 97-100. doi:10.1136/adc.68.1.97

Author Justo, R. N.
Dare, A. J.
Whight, C. M.
Radford, D. J.
Title Pulmonary venoocclusive disease: diagnosis during life in 4 patients
Journal name Archives of Disease in Childhood   Check publisher's open access policy
ISSN 0003-9888
Publication date 1993-01
Sub-type Article (original research)
DOI 10.1136/adc.68.1.97
Volume 68
Issue 1
Start page 97
End page 100
Total pages 4
Place of publication London, United Kingdom
Publisher BMJ
Language eng
Abstract Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension of unknown aetiology. Four cases were diagnosed in young patients. The diagnosis was suspected on the basis of clinical, radiological, echocardiographic, and catheter evidence and confirmed by taking a lung biopsy sample. In all patients the histology showed obstruction of the pulmonary veins by intimal fibrosis. The clinical course of all patients has been one of progressive deterioration. Although there is no specific treatment for this disease, to establish the diagnosis during life is of great importance in overall clinical management, including counselling the patient and family.
Keyword Venoocclusive disease
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
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