Respir Case Rep. 2017; 6(2): 110-113 | DOI: 10.5505/respircase.2017.93585

Yeni bir taným: Kombine pulmoner fibrozis ve amfizem sendromu

Dildar Duman, Hakan Günen
Süreyyapaþa Göðüs Hastalýklarý ve Göðüs Cerrahisi Eðitim Araþtýrma Hastanesi, Ýstanbul

Kombine pulmoner fibrozis ve amfizem sendromu (KPFA), kendine özgü klinik bulgularý olan ve radyolojik olarak üst lob amfizemi ve alt lob fibrozisi ile karakterize yeni tanýmlanan bir sendromdur. Hastalýk iyi bilinmediði için yeterince taný konulamamaktadýr. Progresif nefes darlýðý þikayetiyle baþvuran, 60 paket/yýl sigara öyküsü olan erkek hastanýn çekilen toraks tomografisinde KPFA sendromuna özgü üst loblarda amfizem ve alt loblarda fibrozis izlendi. Hastalýðýn bir diðer önemli özeliði olarak FEV1 kýsmen korunmuþ, DLCO ise oldukça düþük bulundu. hastada pulmoner hipertansiyon saptandý. Tüm bulgularýyla KPFA sendromu tanýsý konulan olgu, bu sendromuna dikkat çekmek için literatür eþliðinde sunuldu.

Anahtar Kelimeler: amfizem, fibrozis, pulmoner hipertansiyon

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A new definition: Combined pulmonary fibrosis and emphysema syndrome

Dildar Duman, Hakan Günen
Süreyyapaþa Chest Diseases and Thoracic Surgery Trainig and Research Hospital, Istanbul, Turkey

Combined pulmonary fibrosis and emphysema syndrome (CPFE) is a newly defined syndrome with unique clinical findings, characterized by presence of emphysema and pulmonary fibrosis seen radiologically. Since awareness of the syndrome is insufficient, CPFE is under-recognized. Male patient presented with progressive dyspnea and history of smoking 60 packs per year. Upper lobe emphysema and lower lobe fibrosis, which are specific for CPFE syndrome, were observed in chest computed tomography scans. Forced expiratory volume 1 was relatively well preserved, but diffusing capacity of the lungs for carbon monoxide was very low, and is an important feature of the disease. Pulmonary hypertension has also been determined to be characteristic finding of the syndrome. Patient was diagnosed as CPFE syndrome based on presence of all characteristic features and case is presented with review of the literature to draw attention to this disease.

Keywords: emphysema, fibrosis, pulmonary hypertension

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ATIF KOPYALA