کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4129211 1270954 2006 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
La fibrose pulmonaire du syndrome de Hermansky Pudlak n'est pas totalement commune
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی آسیب‌شناسی و فناوری پزشکی
پیش نمایش صفحه اول مقاله
La fibrose pulmonaire du syndrome de Hermansky Pudlak n'est pas totalement commune
چکیده انگلیسی
A 55-year-old female patient with Hermansky-Pudlak syndrome (albinism, thrombopathia and ceroid accumulation) underwent a single lung transplantation for pulmonary fibrosis. Examination of explanted lung showed usual interstitial pneumonia pattern associated with two unusual lesions: presence of numerous macrophages containing ceroid pigments within fibrosis and characteristic foamy swelling of pneumocytes. This later lesion, which has only been recently described, seems related to the mechanism of the disease by defect of surfactant secretion. This lesion allows the histological individualization of pulmonary fibrosis associated with Hermansky-Pudlak syndrome from idiopathic usual interstitial pneumonia.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Annales de Pathologie - Volume 26, Issue 6, December 2006, Pages 445-449
نویسندگان
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