Differentiating pulmonary lymphangioleiomyomatosis from pulmonary langerhans cell histiocytosis and Birt-Hogg-Dube syndrome
نویسندگان
چکیده
منابع مشابه
New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.
Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In thi...
متن کاملBirt-hogg-dube Syndrome: a Case Report.
Background: Here we present a case of 56 year old female who presented with mild shortness of breath and right side pain. Chest tomography indicated bilateral bullous pulmonary lesions as the causal of the patient’s discomfort and pain. The patient had familial history of cancer and pneumothorax, and had undergone surgery on the left lung due to bullous lesion and pneumothorax. The patient has ...
متن کاملPulmonary langerhans cell histiocytosis
Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the b...
متن کاملPulmonary Langerhans cell Histiocytosis.
Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocy...
متن کاملBirt-Hogg-Dube syndrome is a novel ciliopathy.
Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder where patients are predisposed to kidney cancer, lung and kidney cysts and benign skin tumors. BHD is caused by heterozygous mutations affecting folliculin (FLCN), a conserved protein that is considered a tumor suppressor. Previous research has uncovered multiple roles for FLCN in cellular physiology, yet it remains unclear how the...
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ژورنال
عنوان ژورنال: Lung India
سال: 2013
ISSN: 0970-2113
DOI: 10.4103/0970-2113.120611