Birt-Hogg-Dube Sendromu:  Az Tan�nan ve Multidisipliner Yakla��m Gerektiren Olgu Serisinde BT Bulgular�

Cimsit, Canan; Eryuksel, Emel; Cimsit, Nuri Cagatay; Olgun, Sehnaz; Seckin, Dilek; Saricam, Merve Hatun; Demirkesen, Cuyan

Cilt : 5 Say� : 1 Y�l : 2024

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Birt-Hogg-Dube Sendromu: Az Tan�nan ve Multidisipliner Yakla��m Gerektiren Olgu Serisinde BT Bulgular� [Respir Case Rep]

Respir Case Rep. 2016; 5(1): 5-9 | DOI: 10.5505/respircase.2016.76476

Birt-Hogg-Dube Sendromu: Az Tan�nan ve Multidisipliner Yakla��m Gerektiren Olgu Serisinde BT Bulgular�

Canan Cimsit¹, Emel Eryuksel², Nuri Cagatay Cimsit¹, Sehnaz Olgun², Dilek Seckin³, Merve Hatun Saricam³, Cuyan Demirkesen⁴
¹Marmara �niversitesi Pendik E�itim ve Ara�t�rma Hastanesi, Radyoloji Anabilim Dal�, �stanbul
²Marmara �niversitesi Pendik E�itim ve Ara�t�rma Hastanesi, G��s Hastal�klar� Anabilim Dal�, �stanbul
³Marmara �niversitesi Pendik E�itim ve Ara�t�rma Hastanesi, Dermatoloji Anabilim Dal�, �stanbul
⁴�stanbul �niversitesi Cerrahpa�a T�p Fak�ltesi, Patoloji Anabilim Dal�, �stanbul

Birt-Hogg-Dub� Sendromu (BHDS) deride fibrofolik�lomalar, renal t�m�rler ve akci�er kistleri ile karakterizedir. Bu olgu serisinde, Birt-Hogg-Dub� sendromlu iki olgu ve aile �yelerinin BT bulgular� sunulmaktad�r. Di�er kistik akci�er hastal�klar� ile ay�r�c� tan� tart��lmaktad�r. Olas� malignitelerin tespit edilebilmesi a��s�ndan takip ve tarama ile multidisipliner yakla��m�n �nemi vurgulanmaktad�r. BHDS seyri s�ras�nda y�ll�k hasta takibi yan� s�ra pn�motoraks riski a��s�ndan �zen gerekmektedir.

Anahtar Kelimeler: Birt-Hogg- Dub� Sendromu, fibrofolik�loma, akci�er kistleri, renal kitle, pn�motoraks

Birt-Hogg-Dub� Syndrome: CT Findings of an Under Recognized Disease Requiring Multidisciplinary Approach

Canan Cimsit¹, Emel Eryuksel², Nuri Cagatay Cimsit¹, Sehnaz Olgun², Dilek Seckin³, Merve Hatun Saricam³, Cuyan Demirkesen⁴
¹Department of Radiology, Marmara University Training and Research Hospital, �stanbul, Turkey
²Department of Pulmonary And Critical Care Medicine, Marmara University Training and Research Hospital, Istanbul, Turkey
³Department of Dermatology, Marmara University Training and Research Hospital, �stanbul, Turkey
⁴Department of Pathology, �stanbul University Cerrahpa�a Faculty of Medicine, �stanbul, Turkey

Birt-Hogg-Dub� Syndrome (BHDS) is characterized by hair follicle hamartomas, renal tumors, and pulmonary cysts. In this case series, CT findings of two index patients with BHDS, and their family members, are presented. Differential diagnosis with other cystic lung diseases is discussed. The importance of follow-up and screening for the early detection of possible malignancies, as well as multidisciplinary approach, is emphasized. The course of BHDS requires not only annual patient follow-up but also care due to the risk of pneumothorax.

Keywords: Birt-Hogg-Dub� syndrome, fibrofolliculoma, lung cysts, renal tumors, pneumothorax.

Sorumlu Yazar: Canan Cimsit, T�rkiye
Makale Dili: �ngilizce

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