A rare diagnosis: Keutel Syndrome

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A rare diagnosis: Keutel Syndrome [Medeniyet Med J]

Medeniyet Med J. 2019; 34(3): 329-332 | DOI: 10.5222/MMJ.2019.91979

A rare diagnosis: Keutel Syndrome

Saniye Girit¹, Ebru Senol²
¹Division of Pediatric Pulmonology, Department of Pediatrics, Medeniyet University Medical Faculty Goztepe Research and Training Hospital, Istanbul, Turkey
²Department of Pediatrics, Health Sciences University Medical Faculty Kartal Dr. Lutfi K�rdar Educational and Research Hospital, Istanbul, Turkey

Tracheobronchial cartilage calcification is a very uncommon finding in pediatric population. Keutel Syndrome (OMIM 245150) is a very rare syndrome characterized by diffuse calcification of the cartilages, brachitelephalangia, pulmonary stenosis, mid-line defects, stippled epiphysis in infancy and hearing loss accompanied by recurrent respiratory infections and asthma-like attacks. Here, we present a 14 year old patient previously diagnosed as asthma, didn't relieve with appropriate asthma treatment and diagnosed with Keutel Syndrome with cartilage calcification on tracheobranchial tree and auricula, atypical facial features, recurrent otitis, asthma-like symptoms and hearing loss.

Keywords: Asthma, child, Keutel Syndrome, pathologic calcification

Nadir bir tan�: Keutel Sendromu

Saniye Girit¹, Ebru Senol²
¹�ocuk G��s Hastal�klar� Bilim Dal�, Medeniyet �niversitesi T�p Fak�ltesi G�ztepe E�itim ve Ara�t�rma Hastanesi, �stanbul T�rkiye
²�ocuk Sa�l�� ve Hastal�klar� Ana Bilim Dal�. Sa�l�k Bilimleri �niversitesi Kartal Dr L�tfi K�rdar E�itim ve Ara�t�rma Hastanesi, �stanbul T�rkiye

�ocuk pop�lasyonda trakeobron�ial k�k�rdak kalsifikasyonu �ok s�k g�r�lmeyen bir bulgudur. Keutel sendromu (OMIM 245150) ast�m benzeri ataklar ve tekrarlayan alt solunum yolu enfeksiyonlar� ile birlikte diff�z k�k�rdak kalsifikasyonu, brakiotelofalanji, orta hat defektleri, i�itme kayb� ile karakterize nadir g�r�len bir sendromdur. Biz burada ast�m tan�s�yla takip edilen ancak ast�m tedavilerine yan�ts�z 14 ya��nda bir olgu sunuyoruz. Bu hasta trakeobron�ial a�a� ve aurikulada kalsifikasyon, atipik y�z g�r�n�m�, tekrarlayan otitis media, i�itme kayb� ve tekrarlayan ast�m benzeri ataklar ile Keutel sendromu tan�s� ald�.

Anahtar Kelimeler: Ast�m, �ocuk, Keutel sendromu, patolojik kalsifikasyon

Saniye Girit, Ebru Senol. A rare diagnosis: Keutel Syndrome. Medeniyet Med J. 2019; 34(3): 329-332

Corresponding Author: Saniye Girit, T�rkiye

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