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Laparoscopic Endoscopic Surgical Science Birt-Hogg-Dub Sendromu ve Sarkoidozun Nadir Birliktelii [Respir Case Rep]
Respir Case Rep. 2021; 10(3): 186-189 | DOI: 10.5505/respircase.2021.40327

Birt-Hogg-Dub Sendromu ve Sarkoidozun Nadir Birliktelii

Celal Satc1, Gizem Kyba1, Aye Koyukan2, Ekrem Cengiz Seyhan1
1Salk Bilimleri niversitesi, Yedikule Gs Hastalklar Ve Gs Cerrahisi Eitim Ve Aratrma Hastanesi, Gs Hastalklar anabilim Dal, stanbul
2Salk Bilimleri niversitesi, Yedikule Gs Hastalklar Ve Gs Cerrahisi Eitim Ve Aratrma Hastanesi, Patoloji Anabilim Dal, stanbul

Birt-Hogg-Dube (BHD) sendromuna sahip hastalarda FLCN geni tarafndan kodlanan folliculin proteininin T-helper-1 hcre aktivasyonuna yol aarak sarkoidoza yol aabilecei ve bu sebeple bu iki hastalk arasnda patofizyolojik bir ortaklk olabilecei dnlmektedir. Burada nadir bir BHD sendromu ve sarkoidoz birliktelii olgusunu sunuyoruz. BHD tans alan hastalarn toraks bilgisayarl tomografilerinin hem parankim hem de mediasten penceresinde olas bir mediastinal lenfadenopatiyi gzden karmamak adna detayl olarak incelenemeli ve patolojik boyutta bir lenf nodu mevcut ise biyopsi yaplmaldr.

Anahtar Kelimeler: Birt Hogg Dub, sarkoidoz, fibrofolikulom, mediastinal lenfadenopati.

A Rare Coexistence of Birt-Hogg-Dub Syndrome and Sarcoidosis

Celal Satc1, Gizem Kyba1, Aye Koyukan2, Ekrem Cengiz Seyhan1
1Department of Chest Disesases, University of Health Sciences, Yedikule Research and Training Hospital for Chest Diseases and Chest Surgery, stanbul, Turkey
2Department of Pathology, University of Health Sciences, Yedikule Research and Training Hospital for Chest Diseases and Chest Surgery, Istanbul, Turkey

A pathophysiological link may exist between Birt-Hogg-Dub syndrome (BHD) and pulmonary sarcoidosis such that the folliculin protein encoded by the FLCN gene may lead to the development of sarcoidosis through the upregulation of T- helper 1 cell activation. We present here a rare case of coexisting BHD and sarcoidosis. Enlarged mediastinal lymphadenopathy may be underdiagnosed in patients with BHD, and so both the mediastinal and lung window of a thoracic CT scan should be evaluated carefully and a biopsy should be performed if pathologic lymphadenopathies are indicated.

Keywords: Birt Hogg Dub, sarcoidosis, fibrofolliculoma, mediastinal lymphadenopathy.

Celal Satc, Gizem Kyba, Aye Koyukan, Ekrem Cengiz Seyhan. A Rare Coexistence of Birt-Hogg-Dub Syndrome and Sarcoidosis. Respir Case Rep. 2021; 10(3): 186-189

Sorumlu Yazar: Celal Satc, Trkiye
Makale Dili: ngilizce
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