Prevalence of Birt-Hogg-Dubé syndrome in patients with apparently primary spontaneous pneumothorax.
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چکیده
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منابع مشابه
Risk of renal and colonic neoplasms and spontaneous pneumothorax in the Birt-Hogg-Dubé syndrome.
The Birt-Hogg-Dubé syndrome, a genodermatosis characterized by benign tumors of the hair follicle, has been associated with renal and colonic neoplasms and spontaneous pneumothorax, but the risk of developing these disorders is unknown. We identified risk factors for renal tumors and spontaneous pneumothorax in 98 patients affected with the Birt-Hogg-Dubé syndrome, in 13 Birt-Hogg-Dubé haplotyp...
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INTRODUCTION Birt-Hogg-Dubé syndrome is a rare genodermatosis characterized by hair follicle hamartomas, renal tumors and spontaneous pneumothorax. We present the case of a patient with pulmonary cysts and recurrent spontaneous pneumothorax. She had typical skin lesions, and was found to have a hybrid oncocytoma which was surgically excised. CASE PRESENTATION A 60-year-old Caucasian woman had...
متن کاملAn inherited cause of pneumothorax--the Birt-Hogg-Dubé syndrome.
A 38-year-old woman presented in 2004 a spontaneous pneumothorax (Figure 1a). Outcome was favorable with treatment with chest tube drainage. Eight years later, she complained of dyspnea. A computed tomography scan of the chest revealed lung cysts (Figure 1b). She presented 2–4mm, white, dome-shaped papules involving the neck, which appeared during the last 2 years. Her brother also presented mu...
متن کاملBirt-Hogg-Dubé syndrome: a rare cause of pulmonary cysts.
2Department of Surgery, Vancouver General Hospital and University of British Columbia, Vancouver, BC, V5Z 1M9 Canada. irt-Hogg-Dubé syndrome is a rare autosomal-dominant inherited disorder characterized by the presence of firm facial papules that on biopsy are shown to represent fibrofolliculomas [1–3]. Patients with Birt-HoggDubé syndrome have a high predisposition to malignant renal tumors, w...
متن کاملBirt-Hogg-Dubé Syndrome: Answering Questions Raised by a Case Report Published in 1962
In 1962, J.J. Collins from the United States Naval Medical Research Laboratory published an unusual case of air embolism precipitated by decompression in The New England Journal of Medicine [1962;266:595-598]. The case was unusual because it was the first where multiple pulmonary cysts were discovered after a successful recompression treatment. Although various hypotheses were put forward by th...
متن کاملRecurrent spontaneous pneumothorax in a patient with Birt-Hogg-Dubé syndrome.
Recurrent spontaneous pneumothorax is a disorder often managed by thoracic surgeons. Most etiologies are benign in nature; however, there are several syndromes that are associated with potentially fatal pulmonary or systemic manifestations. One such example is Birt-Hogg-Dubé syndrome, a rare inheritable syndrome characterized by papular skin lesions involving the scalp, head, face and neck; pul...
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ورودعنوان ژورنال:
- The European respiratory journal
دوره 45 4 شماره
صفحات -
تاریخ انتشار 2015