Birt-hogg-dube Syndrome: a Case Report.
نویسندگان
چکیده
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 been operated in our clinic due to right lung bullous lesions; resected bullous tissue has been evaluated histologically in a further center. Objectives: Birt Hogg Dubè (BHD) syndrome is an inherited genodermatosis. Spontaneous pneumothorax and pulmonary bullous lesions, renal cyst or tumor has a triad with lesions on the skin. We aimed to evaluate the patients presenting recurren primary spontaneous pneumothorax with family history of pneumothorax and with having atypical bullae localization for BHD syndrome. Conclusions: Patients with BHD syndrome must be subject to routine cancer screening and watchful waiting due to the fact that lung, gastrointestinal, ovarian, breast and pancreatic cancer and especially renal cancer risk is very high when compared to the society. *Corresponding author: Arife Zeybek, Asistant Professor, Department of Thoracic Surgery, Mugla Sıtkı Koçman University, School of Medicine, Turkey, Tel: 90 252 2114855; E-mail: [email protected] Received September 04, 2013; Accepted October 30, 2013; Published November 01, 2013 Citation: Zeybek A, Şahan L, Çelik Öİ, Özbudak İH, Kalemci S (2013) Birt Hogg Dubè Syndrome: A Case Report. J Pulmon Resp Med S14: 008. doi:10.4172/2161105X.S14-008 Copyright: © 2013 Zeybek A, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
منابع مشابه
Pneumothorax and Birt–Hogg–Dube syndrome: diagnostic and therapeutic aspects
We report a case of spontaneous recurrent pneumothorax in a 32-year-old male with a family history of Birt–Hogg–Dube syndrome. Specific aspects of the surgical treatment for a pneumothorax within this particular setting are discussed as well as the potential underdiagnosis of this complex genodermatosis. The literature linking the syndrome to spontaneous pneumothorax is reviewed.
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ورودعنوان ژورنال:
- Genetic counseling
دوره 26 3 شماره
صفحات -
تاریخ انتشار 2015