Özlem Kar KURT, Zehra YAŞAR, Betül ARI, Fahrettin TALAY, Safiye GÜREL

SIK TEKRARLAYAN AKCİĞER ENFEKSİYONU NEDENİ OLARAK MOUNİER-KUHN SENDROMU: OLGU SUNUMU

Trakeobronkomegali, trakea ve büyük bronşların anormal genişlemesi ile karakterize bir sendromdur. Trakea ve ana bronşlarda elastik ve müsküler dokudaki atrofi veya eksiklik sonucu oluştuğu düşünülmektedir. Solunum fonksiyonlarının iyi korunduğu minimal hastalıktan bronşektazi, tekrarlayan solunum yolu enfeksiyonları ve solunum yetmezliğine kadar değişen farklı klinik prezentasyonlar gösterilmiştir. Daha önce astım nedeniyle takipli 61 yaşında erkek hasta bir haftadır olan öksürük, ateş ve hırıltı şikâyetleri ile başvurdu. Son 1 yılda 3 kez pnömoni nedeniyle hastaneye yatırılarak tedavi öyküsü olan olgunun toraks bilgisayarlı tomografisinde trakea çapında genişleme ve her iki akciğerde bronşektazi saptandı. Tekrarlayan akciğer enfeksiyonunun eşlik ettiği, trakeobronkomegali olarak kabul edilen bu nadir olgu sunuldu ve ilgili literatür gözden geçirildi.

Anahtar Kelimeler:

Mounier-Kuhn syndrome, tracheobronchomegaly, bronchiectasis, pneumonia

MOUNIER-KUHN SYNDROME AS A CAUSE OF RECURRENT PULMONARY INFECTIONS: A CASE REPORT

Tracheobronchomegaly is a syndrome characterized by abnormal dilatation of the trachea and main bronchi. This syndrome is thought to be caused by atrophy or absence of elastic fibers and smooth muscle cells in trachea and main bronchi. A broad spectrum of clinical presentations has been documented ranging from minimal disease with good preservation of pulmonary function to bronchiectasis, recurrent respiratory tract infection and respiratory failure. We present the case of a 61-year-old male patient who was admitted to hospital because of cough, fever and wheezing for one week. The patient had a history of hospitalization due to pneumonia 3 times in the last one year. Computed tomography scan showed tracheomegaly and bilateral bronchiectasis. This rare case, considered as tracheobronchomegaly accompanying recurrent pulmonary infection, is presented and the pertinent literature is reviewed.

Keywords:

Mounier-Kuhn sendromu, trakeobronkomegali, bronşektazi, pnömoni,

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