Ankara Üniversitesi Tıp Fakültesi Mecmuası 0365-8104 1307-5608 Ankara University Urology Üroloji Friedreich's Ataxia Seen in 3 Children of a Family Bir Ailenin 3 Çocuğunda Görülen Friedreich Ataksisi Ege Binnaz 07 01 1980 33 1 115 122 04 01 1980 06 30 1980 Copyright © 1947, Ankara Üniversitesi Tıp Fakültesi Mecmuası 1947 Ankara Üniversitesi Tıp Fakültesi Mecmuası

All of the three children of a family ha-ve Friedreich ataxa.The oldest child has the findings of myocarditis in ECG. She also has some hearing defects, such as decreased the hearing of low and high freguency sounds. These are rare systemic manifestations of Friedreich ataxia in children. The early onset and the rapid progression and even the presence of typical diagx)stic findings of the disease in a 3 year old child are the characteristics of our cases.

Bir ailenin 3 çocuğunda birden görülen Friedrich ataksisi vak'ası bildirilmiştir. Vak'alardan birinde, myokardm hastalığa katıldığını belirleyen EKG bulgularının ve kulak muayenesinde, yüksek ve düşük frekanslı seslerin duyulmasında azalmanın saptanmasl, çocuklarda, Friedreich ataksisinde seyrek raslanan sistemik bulgulardandrr. Hastallžrn erkenden baflamasr, sur'at'li bir seyir takip etmesi 3 yayndaki hastada bile, tam koydurabilecek belirtilerin görülmesi, vak'alanmmn özelliklerini olusturmktadlr

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