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Yetikinlikte Tan Alan Williams-Campbell Sendromu: Nadir Bir Bronektazi [Respir Case Rep]
Respir Case Rep. 2022; 11(1): 50-54 | DOI: 10.5505/respircase.2022.94546

Yetikinlikte Tan Alan Williams-Campbell Sendromu: Nadir Bir Bronektazi

Kbra Gl Klnarslan1, Fatma Tokgz Akyl1, Neslihan Boyrac1, Metin Sucu2, Melih Akay Arslan1, Hlya Abal1, Kaan Kara1, Seda Tural nr1
1Yedikule Gs Hastalklar ve Gs Cerrahisi Eitim ve Aratrma Hastanesi, Gs Hastalklar Blm, stanbul
2Kadky Florence Nightingale Hastanesi, Radyoloji Blm, stanbul

Williams-Campbell sendromu, bron duvar kartilaj oluum eksikliinden veya tamamen yokluundan kaynaklanan, distal hava yolu kollaps ve bronektaziye neden olan nadir bir sendromdur. Sendrom ilk olarak infant dnemde tanmlanm olup ocukluk dneminden itibaren, ksrk wheezing gibi obstrktif semptomlar ve tekrarlayan pnmoniler ile seyreder. Erikin yata olgular ok nadir bildirilmitir. Krk yanda erkek hasta, bir haftadr giderek artan nefes darl, ksrk, ate ikayetleriyle bavurdu. On yldr bronektazi tans olan hastann ylda birka kez enfeksiyon geirme yks mevcuttu. Enfeksiyon parametrelerinde ykseklik saptanan hasta enfekte bronektazi tans ile yatrld. nspiryumda ve ekspiryumda ekilen tomografilerde youn hava hapsi, ekspiryumda hava yolu kollaps izlenen hastann kistik broniektazi etyolojisi aratrld. Galaktomannan antijeni negatifti, immnglobulin dzeyleri ve alfa 1-antitripsin deerleri normal snrlarda saptand. Hastaya klinik ve radyolojik bulgular eliinde Williams-Campbell sendromu tans konuldu. Antibiyoterapi sonras klinik yant alnan hasta pulmoner rehabilitasyon programna alnarak akcier nakli asndan deerlendirilmek zere ynlendirildi. Sonu olarak, Williams-Campbell sendromu erikin yata da tan konulabilen nadir bir sendromdur ve tm yalarda bronektazi etyolojisinin aratrlmas hasta ynetimine katk salayacaktr.

Anahtar Kelimeler: Williams-campbell sendromu, broniektazi, konjenital anomali

Williams-Campbell Syndrome Diagnosed in Adulthood: A Rare Entity of Bronchiectasis

Kbra Gl Klnarslan1, Fatma Tokgz Akyl1, Neslihan Boyrac1, Metin Sucu2, Melih Akay Arslan1, Hlya Abal1, Kaan Kara1, Seda Tural nr1
1Department of Chest Diseases, Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, stanbul, Turkey
2Department of Radiology, Kadky Florence Nightingale Hospital, stanbul, Turkey

Williams-Campbell syndrome is a rare condition caused by a deficiency or complete absence of bronchial wall cartilage formation, resulting in distal airway collapse and bronchiectasis. The syndrome, generally encountered first during infancy, progresses with recurrent pneumonia with such obstructive symptoms as cough and wheezing from childhood onwards, while adult cases are rarely reported. A 40-year-old male patient presented with complaints of shortness of breath, cough and fever for one week. He was hospitalized with a diagnosis of bronchiectasis exacerbation due to elevated infection parameters, when radiology revealed extensive cystic bronchiectasis, air trapping and airway collapse at expiration. After excluding other etiologies, the patient was diagnosed with Williams-Campbell syndrome in light of the clinical and radiologic findings. He was discharged following clinical response and started on a pulmonary rehabilitation program, was referred to a lung transplantation clinic. The case is representative of the set of rare adult cases showing that Williams-Campbell syndrome can be diagnosed in adulthood, and signaling that the etiology of bronchiectasis should be further evaluated in advanced ages.

Keywords: Williams-campbell syndrome, bronchiectasis, congenital anomaly

Olgunun Grnt Kesitleri




Kbra Gl Klnarslan, Fatma Tokgz Akyl, Neslihan Boyrac, Metin Sucu, Melih Akay Arslan, Hlya Abal, Kaan Kara, Seda Tural nr. Williams-Campbell Syndrome Diagnosed in Adulthood: A Rare Entity of Bronchiectasis. Respir Case Rep. 2022; 11(1): 50-54

Sorumlu Yazar: Kbra Gl Klnarslan, Trkiye
Makale Dili: ngilizce
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