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Coronary artery bypass graft in a 10-year-old child with familial hypercholesterolemia [J Behcet Uz Child Hosp]
J Behcet Uz Child Hosp. 2018; 8(1): 69-71 | DOI: 10.5222/buchd.2018.069  

Coronary artery bypass graft in a 10-year-old child with familial hypercholesterolemia

Tülay Demircan1, Özgür Kızılca2, Nuh Yılmaz3, Cüneyt Zihni4, Bahattin Öncü4, Mustafa Kır4, Nurettin Ünal4, Baran Uğurlu5
1Department Of Pediatric Cardiology, İzmir Tepecik Training And Research Hospital, İzmir, Turkey
2Department Of Pediatric Cardiology, Trakya University, Faculty Of Medicine, Tekirdağ, Turkey
3Department Of Pediatric Cardiology, Mustafa Kemal University, Faculty Of Medicine, Hatay, Turkey
4Department Of Pediatric Cardiology, Dokuz Eylül University, Faculty Of Medicine, İzmir, TurkeyDepartment Of Pediatric Cardiology, Mustafa Kemal University, Faculty Of Medicine, Hatay, Turkey
5Department Of Cardiovascular surgery, Dokuz Eylül University, Faculty Of Medicine, İzmir, Turkey

Homozygous familial hypercholesterolemia (HoFH) is a result of a disorder of LDL recepto function which is due to a gene abnormality located on the short arm of chromosome 19. Homozygous and heterozygous types available. In homozygous types emerge of coronary lesions due to early atherosclerosis in 1. decade.This untreatment patients are die nearly 20 years old.
After the six months of diagnosis of the familial hypercholesterolemia patient admitted to our outpatient clinic with the complaint of exertional chest pain. A coronary angiography was performed because of the patological exercise stress test. Significant stenosis was detected in the coronary artery. Then the patient has underwent successfully coronary bypass grafting. This case is presented to highlight the early development of coronary lesions in patients with hyperlipidemia.

Keywords: Homozygous familial hypercholesterolemia, coronary bypass, child


Familyal Hiperkolesterolemi nedeni ile 10 yaşında koroner bypass yapılan çocuk olgu

Tülay Demircan1, Özgür Kızılca2, Nuh Yılmaz3, Cüneyt Zihni4, Bahattin Öncü4, Mustafa Kır4, Nurettin Ünal4, Baran Uğurlu5
1İzmir Tepecik Eğitim Ve Araştırma Hastanesi Çocuk Kardiyoloji
2Trakya Üniversitesi Tıp Fakültesi Çocuk Kardiyoloji
3Mustafa Kemal Üniversitesi, Çocuk Kardiyoloji
4Dokuz Eylül Üniversitesi Tıp Fakültesi Çocuk Kardiyoloji
5Dokuz Eylül Üniversitesi Tıp Fakültesi Kalp Damar Cerrahisi Bölümü

Familyal hiperkolesterolemi 19. kromozomun kısa kolunda yer alan düşük yoğunluklu lipoprotein(LDL) reseptör genindeki defekt sonucu lipoproteinlerin dolaşımdan yeteri kadar temizlenememesi ile ortaya çıkan otozomal dominant bir hastalıktır. Homozigot ve heterozigot tipleri mevcuttur. Homozigot tiplerde erken ateroskleroza bağlı 1. dekadda koroner lezyonlar ortaya çıkmakta ve tedavisiz hastalar genelde 20’li yaşlarda kaybedilmektedir.
Familyal hiperkolesterolemi tanısından 6 ay sonra eforla göğüs ağrısı olan hasta kliniğimize başvurdu. Efor testi pozitif saptanan hastaya koroner anjiyografi yapıldı ve sol koroner arterde belirgin darlık saptandı. Hastamıza başarılı bir şekilde koroner bypass operasyonu yapıldı. Bu vaka hiperlipidemili hastalarda koroner lezyonların erken dönemde gelişebileceğine dikkat çekmek için sunulmuştur.

Anahtar Kelimeler: Ailesel homozigot hiperkolesterolemi, koroner bypass, çocuk


Tülay Demircan, Özgür Kızılca, Nuh Yılmaz, Cüneyt Zihni, Bahattin Öncü, Mustafa Kır, Nurettin Ünal, Baran Uğurlu. Coronary artery bypass graft in a 10-year-old child with familial hypercholesterolemia. J Behcet Uz Child Hosp. 2018; 8(1): 69-71

Corresponding Author: Tülay Demircan, Türkiye


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