The European respiratory journal · 2009
Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Khaldi H, Marchand-Adam S, Kannengiesser C, Fabre A, Debray M P, Danel C, Gougerot-Pocidalo M-A, El Benna J, Humbert M, Aubier M, Crestani B
Аннотация
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.
Предметные рубрики MeSH
AdultBiopsyBronchoalveolar LavageBronchoscopyDiagnosis, DifferentialDiureticsFurosemideGranulomatous Disease, ChronicHemodynamicsHumansHypertension, PulmonaryLung Diseases, InterstitialMalePhosphodiesterase InhibitorsPiperazinesPurinesRespiratory Function TestsSildenafil CitrateSmokingSulfonesTomography, X-Ray Computed