کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4210743 1280608 2012 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Different features of lung involvement in Niemann-Pick disease and Gaucher disease
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی پزشکی ریوی و تنفسی
پیش نمایش صفحه اول مقاله
Different features of lung involvement in Niemann-Pick disease and Gaucher disease
چکیده انگلیسی

SummaryBackgroundNiemann-Pick disease (NPD) and Gaucher disease (GD) are well-known lysosomal storage diseases. Respiratory system involvement is an important cause of morbidity and mortality in patients with NPD and GD.ObjectivesWe tried to assess the clinical, radiological, and histological features of GD and NPD patients with lung involvement.MethodsWe reviewed medical history, physical examination, radiological, and histological data of 10 NPD and 7 GD patients.ResultsThe most common respiratory symptoms were recurrent lung infection and dyspnea. Although lung examination results in 6 NPD patients were normal, they had lung involvement; 3 patients were diagnosed as NPD directly via lung biopsy during investigation of recurrent lung infection or interstitial lung disease. All GD patients but 1 had respiratory system symptoms at the time of diagnosis. Hepatopulmonary syndrome was present in 4 GD patients. A ground-glass pattern and atelectasis were 2 important high-resolution computed tomography features in the NPD and GD patients. Flexible bronchoscopy and bronchoalveolar lavage were used for emergency extraction of bronchial casts in 1 NPD patient.ConclusionsLung involvement in NPD and GD patients should be included in the differential diagnosis of interstitial lung disease. Besides interstitial appearance on HRCT, atelectasis related to bronchial cast and bronchiectasis are other radiological findings in these group of patients. Analysis of bronchoalveolar fluid and lung biopsy provide very important clues for diagnosis. Hepatopulmonary syndrome is an important vascular complication observed in GD patients.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Respiratory Medicine - Volume 106, Issue 9, September 2012, Pages 1278–1285
نویسندگان
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