English
English
DOI:
https://doi.org/10.5281/zenodo.7718553Anahtar Kelimeler:
EnglishÖzet
Amaç: Çocuklarda kozmetik ve fonksiyonel sekel oranı yüksek bir hastalık olan juvenil lokalize sklerodermayı çocuk romatoloji perspektifinden değerlendirmek.
Gereç ve Yöntem: Çocuk romatoloji kliniğimizde 2015-2022 yılları arasında juvenile lokalize skleroderma tanısı almış <18 yaş altı, takiplerine düzenli gelen hastalar retrospektif olarak incelendi. Hastalara ait demografik, klinik, tedaviye ilişkin ve prognostik veriler kaydedildi.
Bulgular: Juvenil lokalize skleroderma tanısı olan 19 hastanın 12’si (63.2%) kız, 7’si (36.8%) erkek cinsiyetteydi. Kız/erkek oranı 1,7’ydi. JLS tanısı olan hastalardan 8’i (42.1%) cicumscribed, 8’i (42.1%) lineer, 2’si (10.6%) mix ve 1’i generalize tipte idi. Şikayetlerin ortalama başlangıç yaşı 8.2±5.5 yaş, tanı yaşı ise ortalama 9.4±4.9 yaştı. En sık tutulan anatomik bölge 15 hasta (78.9%) ile extremitelerdi. Eklemi geçen lezyon oranı 57.9% iken eklem hasarı 21.1% hastada görüldü. Kozmetik sekel oranı 73.7%’ydi. Hastaların 52.6%’sında antinükleer antikor pozitifliği vardı. Takip süresince hiçbir hastada sistemik tutulum gelişmedi. En sık kullanılan tedavi metotrexat idi. 2 hastada (10.6%) tam remisyon sağlanabildi.
Sonuç: Yüksek oranda kozmetik ve fonksiyonel sekellere yol açabileceğinden juvenil lokalize sklerodermanın erken tanınması ve erken agresif tedavisi gerekmektedir. Özellikle çocuk hekimlerinin şüphelendikleri lezyonlardan bekletmeden biyopsi yaptırması veya bu hastaları çocuk romatoloji kliniklerine yönlendirmesi zaman kaybetmemek adına önemlidir.
İndirmeler
Referanslar
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