Çocukluk çağında mastositoz
Küçük Resim Yok
Tarih
2005
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Mastositoz, özellikle deride olmak üzere, pek çok organda mast hücrelerinin birikimine ve mast hücre kökenli mediatörlere bağlı klinik belirtileri olan bir tablodur. Genellikle, erken çocukluk çağında başlayan kronik bir cilt hastalığıdır. Süt çocukluğu, erken çocukluk dönemi ve orta yaş grubunda pik yapar. Hastalığın belirtileri deriyle sınırlı ise, kutanöz mastositoz, kemik iliği, gastrointestinal sistem, karaciğer, dalak gibi deri dışı organların da etkilenmesi durumunda ise, sistemik mastositoz söz konusudur. Olguların büyük çoğunluğunda (%90) sadece deri tutulumu vardır. Darier belirtisi, kaşıntılı deri lezyonlarında çarpma veya manüplasyon sonucu lezyon etrafında ürtiker plağı ortaya çıkması, kaşıntı artışı ve ödem olarak tanımlanır ve mastositoz tanısında önemli bir bulgudur. Standard bir tedavi protokolü bulunmamakta, hastaya göre veya semptomatik tedavi önerilmektedir. Mast hücre degranülasyonunu tetikleyici faktörlerden kaçınma (eliminasyon) önemlidir.
Mastocytosis is a clinical picture resulting mast cell accumulation in various organs, particularly in skin and from mediators released by mast cell. Usually, it is a chronic skin diseases with early childhood onset. It shows a peak incidence in infancy and early childhood. If the symptoms of the disease is confined to skin, it is called as cutaneous mastocytosis. However if the disease involves bone marrow, gastrointestinal system, liver and spleen, then it is called as systemic mastocytosis. Most of the patients (90%) have only skin involvement. Darier sign is the appearance of urticarial plaque with pruritus and edema around the lesion after a hit over the lesion or manupulation of the lesion. This sign is an important sign in the diagnosis of mastocytosis. Though a standardized treatment approach is not present currently, symptomatic treatment or treatment tailored according to each patient is advised. Avoiding of factors which trigger mast cell degranulation (elimination) is important in the management of the disease.
Mastocytosis is a clinical picture resulting mast cell accumulation in various organs, particularly in skin and from mediators released by mast cell. Usually, it is a chronic skin diseases with early childhood onset. It shows a peak incidence in infancy and early childhood. If the symptoms of the disease is confined to skin, it is called as cutaneous mastocytosis. However if the disease involves bone marrow, gastrointestinal system, liver and spleen, then it is called as systemic mastocytosis. Most of the patients (90%) have only skin involvement. Darier sign is the appearance of urticarial plaque with pruritus and edema around the lesion after a hit over the lesion or manupulation of the lesion. This sign is an important sign in the diagnosis of mastocytosis. Though a standardized treatment approach is not present currently, symptomatic treatment or treatment tailored according to each patient is advised. Avoiding of factors which trigger mast cell degranulation (elimination) is important in the management of the disease.
Açıklama
Anahtar Kelimeler
Pediatri
Kaynak
Ege Pediatri Bülteni
WoS Q Değeri
Scopus Q Değeri
Cilt
12
Sayı
3
