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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
Diffuse interstitial pneumonia and pulmonary hypertension: A novel manifestation of chronic granulomatous disease
European Respiratory Journal, Volume 33, No. 6, Year 2009
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Description
The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease. Copyright©ERS Journals Ltd 2009.
Authors & Co-Authors
Khaldi, H.
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Tunisia, Ariana
Université de Tunis el Manar, Hôpital Abderrahmène Mami
Marchand-Adam, Sylvain
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Kannengiesser, Caroline
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
France, Paris
Cib Phenogen Paris
Fabre, A.
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Debraye, M. P.
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Danel, Claire J.
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Gougerot-Pocidalo, Marie Anne
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
France, Paris
Cib Phenogen Paris
France, Paris
Centre de Recherche Biomédicale Bichat-beaujon
El-Benna, Jamel
France, Paris
Centre de Recherche Biomédicale Bichat-beaujon
Humbert, Marc
France, Clamart
Hopital Antoine Beclere
Aubier, Michel A.
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Crestani, Bruno
France, Paris
Hôpital Bichat-claude-bernard Ap-hp
Statistics
Citations: 11
Authors: 11
Affiliations: 5
Identifiers
Doi:
10.1183/09031936.00138107
ISSN:
09031936
e-ISSN:
13993003
Research Areas
Noncommunicable Diseases