Turkiye Klinikleri Journal of Nursing Sciences

.: REVIEW
Ailesel Akdeniz Ateşi Hastalığı Olan Çocuğa Hemşirelik Yaklaşımı
Nursing Approach to Child with Familial Mediterranean Fever
Nagihan SEMİNa, Mehmet Süleyman SABAZb
aÇocuk Sağlığı ve Hastalıkları Hemşireliği AD, Marmara Üniversitesi Sağlık Bilimleri Fakültesi,
aAnesteziyoloji ve Reanimasyon AD, İstanbul Sağlık Bilimleri Üniversitesi Bakırköy Dr. Sadi Konuk Eğitim ve Araştırma Hastanesi, İstanbul, TÜRKİYE
Turkiye Klinikleri J Nurs Sci. 2019;11(1):81-90
doi: 10.5336/nurses.2018-61196
Article Language: TR
Full Text
ÖZET
 Ailesel Akdeniz ateşi (AAA) sıklıkla karın ağrısı, göğüs ağrısı, eklem ağrısı gibi klinik bulguların eşlik ettiği tekrarlayan ateş nöbetleri ile karakterize otozomal resesif otoinflamatuar bir hastalıktır. Ailesel Akdeniz ateşi Türkler, Ermeniler, Araplar ve Yahudiler'de sık görülmektedir. Yüksek risk popülasyonları olarak adlandırılan bu ırklarda hastalık sıklığı yaklaşık 1.000'de 1-3'dür. Ancak, patogenezinden sorumlu MEFV gen mutasyonunun tanılanmasından sonra, İtalyan, Yunan ve Amerikalı gibi çok nadir olduğu düşünülen populasyonlarda daha sık tanı konmaya başlanmıştır. AAA atakları hastaların yaklaşık %90'ında 20 yaşından önce ortaya çıkmaktadır. Yarıdan fazlasında hayatın ilk 10 yılında ortaya çıkmakta ve erkeklerde kızlardan daha fazla görülmektedir (13/10). Pyrin adı verilen gende oluşan mutasyon sonucunda klinik bulgular ortaya çıkmaktadır. Genellikle bu yakınmalar 3-4 gün içinde kendiliğinden geçmektedir. Tekrarlayan ataklar zaman içinde vücutta amiloid adı verilen proteinin birikmesine neden olmakta ve amiloid en sıklıkla böbreklerde birikerek, çocukta kronik böbrek yetmezliğine varan ciddi tablolara yol açabilmektedir. AAA'nın acil tedavi ve hemşirelik bakımında sırasıyla; vücut sıcaklığının normal aralarda olmasının sağlanması, ağrıyı azaltacak uygulamaların yapılması, çocuk ve ebeveynlerin bilgilendirilmesi ve gerekiyorsa çocuğun hastaneye yatırılması yer almalıdır.

Anahtar Kelimeler: Ailesel Akdeniz ateşi; hemşirelik bakımı; ateş; çocuk
ABSTRACT
 Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disorder characterized by recurrent febrile episodes accompanied by clinical symptoms, such as abdominal pain, chest pain, arthralgia. FMF is common in Turks, Armenians, Arabs and Jews. In these races called high-risk populations, the disease frequency is 1-3 in about 1.000. However, after the diagnosis of the MEFV gene mutation responsible for its pathogenesis, it has been diagnosed more frequently in populations such as Italian, Greek and American. FMF attacks start in about 90% of patients before age 20. More than half of them appear in the first 10 years of life and are more common in males than females (13/10). Clinical symptoms occur as mutations in the gland, called Pyrin. Usually these complaints spontaneously pass within 3-4 days. Recurrent episodes cause the accumulation of protein called amyloid in the body over time, and amyloid accumulates most commonly in the kidneys, leading to severe tabulations that lead to chronic kidney failure in the child. In emergency treatment and nursing care of FMF, respectively; body temperature should be kept within normal range, practices to reduce pain, informing children and parents, and hospitalization if necessary.

Keywords: Familial Mediterranean fever; nursing care; fever; child
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