Orak hücreli anemili çocuklarda inme
[ N/A ]
Tarih
2004
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Serebrovasküler hastalıklar açısından özel risk grubu niteliğinde olan hasta grupları, etiyolojik değerlendirme ve klinik özellikleri açısından farklı öneme sahiptir. Orak hücreli anemi 20 yaş altı inmelerin etiyolojisinde önemli yer tutar. Çocukluk çağı serebrovasküler hastalık risk faktörleri için konjenital kalp hastalıklarından sonra en sık ikinci nedenin orak hücreli anemi olduğu ve bu hastaların benzer yaş gruplarına göre 250 kat daha fazla serebral infarkt riski taşıdığı bildirilmiştir. Türkiye'deki tüm hemoglobinopatilerin %17.49'unun Hatay doğumlu olduğu saptanmıştır. Bu çalışmada orak hücreli anemi taşıyıcılığının %10.5 olduğu Hatay ilinde 1994 yılından itibaren hemoglobin elektroforezi ile orak hücreli anemi tanısı almış ve bugüne kadar izlenmiş olan 506 hastada görülen inme olguları bildirildi. 403 olgu HbSS (%79.6), 99 olgu HbSS+ Thalessemi (%19.6), 2 olgu HbSD (%0.4), 2 olgu HbSE idi. 506 hastanın izlendiği 10 yıllık izlem süresinde, inme gelişen 5 hastanın en küçüğü 3, en büyüğü 15 yaşındaydı. Olguların hematolojik parametreleri ile inmelere ait klinik ve etiyolojik özellikler incelendi. Otozomal resesif bir hastalık olan ve kromozom 1 İp.15.4 bölgesinde bulunan, hemoglobin beta genindeki nokta mutasyon oluşan orak hücreli anemi hematolojik nitelikleri yanında inme açısından yüksek risk oluşturma özelliğine sahiptir. Ülkemizde gen taşıyıcılarının yoğun olarak bulunduğu bölgelerde erken tanı ve izlemle komplikasyonlarm önlenmesinde yapılacak çalışmalar çocukluk çağı inmeleri açısından da önem taşımaktadır.
Patient groups at high risk with respect to cerebrovascular disease has a particular importance in terms of etiological and clinic evaluations. Sickle cell anemia has an important place in the etiology of patients who have had stroke before the age of 20. Among the risk factor of cerebrovascular diseases occurring during childhood, sickle cell anemia is the second important cause following the congenital heart diseases. The probability of a patient with sickle cell anemia to have cerebral infarction is 250 times higher when compared to those who don't have this disease at same age. Those who are born in Hatay forms the 17.49% of the all hemoglobinopathy cases reported in Turkey. In this study, the data on stroke obtained from 506 cases by hemoglobin electrophoresis since 1994 in Hatay, where 10.5% of the population is the carrier, was reported. Of the cases, 403 (79,6%), 99 (19,6%), 2 (0,4%) and 2 (0,4%) were HbSS, HbSS+Thalessemia, HbSD and HbSE, respectively. During a period of 10 year, 5 patients had stroke. Among them, the youngest one was 3 yr old while the oldest one was 15 yr old. The data on hemopatolgical parameters and on clinical and etiological were investigated. Being an autosomal recessive disease and arising from the mutation of hemoglobin beta gene, sickle cell anemia is considered to be a high risk factor for stroke besides the hematological features. In the regions where gene carriers are densely populated, studies on early diagnosis and monitoring appears to be highly important in the detection and prevention of childhood strokes.
Patient groups at high risk with respect to cerebrovascular disease has a particular importance in terms of etiological and clinic evaluations. Sickle cell anemia has an important place in the etiology of patients who have had stroke before the age of 20. Among the risk factor of cerebrovascular diseases occurring during childhood, sickle cell anemia is the second important cause following the congenital heart diseases. The probability of a patient with sickle cell anemia to have cerebral infarction is 250 times higher when compared to those who don't have this disease at same age. Those who are born in Hatay forms the 17.49% of the all hemoglobinopathy cases reported in Turkey. In this study, the data on stroke obtained from 506 cases by hemoglobin electrophoresis since 1994 in Hatay, where 10.5% of the population is the carrier, was reported. Of the cases, 403 (79,6%), 99 (19,6%), 2 (0,4%) and 2 (0,4%) were HbSS, HbSS+Thalessemia, HbSD and HbSE, respectively. During a period of 10 year, 5 patients had stroke. Among them, the youngest one was 3 yr old while the oldest one was 15 yr old. The data on hemopatolgical parameters and on clinical and etiological were investigated. Being an autosomal recessive disease and arising from the mutation of hemoglobin beta gene, sickle cell anemia is considered to be a high risk factor for stroke besides the hematological features. In the regions where gene carriers are densely populated, studies on early diagnosis and monitoring appears to be highly important in the detection and prevention of childhood strokes.
Açıklama
Anahtar Kelimeler
Konu Ataması Yapılmamış
Kaynak
Türk Serebrovasküler Hastalıklar Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
10
Sayı
3
