Osman BAŞPINAR, Hakkı KAZAZ, Bahadır DAĞLAR, Ziya BAYRAKTAROĞLU, Vedat DAVUTOĞLU, Alper İ. DAİ

Agressive management in a child with homozygos famiiial hypercholesterolemia

Homozigot ailevi hiperkolesterolemi nadir ölümcül bir hastalıktır. Anjinal semptomlar ve diffüz ksantomlar ile başvuran 12 yaşındaki hastamız uygun diyet kısıtlması, oral yüksek doz statinler, ezetimib, lipoprotein kolesterol aferezi ve koroner arter bypass greft cerrahisi ile agresif bir şekilde tedavi edilmiştir. Üç senedir izlenen hasta herhangi bir problem olmadan takip edilmektedir.

Anahtar Kelimeler:

Ailevi hiperkolesterolemi, Çocuk , Aferez, Koronerbypass, Ezetimid

Ailevi homozigot hiperkolesterolemide yoğun medikal tedavi

Homozygous familial hypercholesterolemia is a rare and fatal disease. Twelve year-old girl patient with anginal symptoms and diffuse xanthomas were treated with diet restriction, per oral high dose statins, ezetimibe, low-density lipoprotein cholesterol apheresis and coronary artery bypass grafting. She has been on follow-up visit in stable condition.

Keywords:

-,

PDF

___

1.Stare TJ. Management of hyperlipidemia in children. Prog Pediatr Cardiol. 2001;12:205-213
2.Rallidis L, Naoumova RP, Thompson GR, Nihoyannopoulos P. Extent and severity of atherosclerotic involvement of the aortic valve and root in familial hypercholesterolaemia. Heart. 1998;80:583-590
3.Keller C, Schmitz H, Theisen K, Zollner N. Regression of valvular aortic stenosis due to homozygous familial hypercholesterolemia following plasmapheresis. Klin Wochenschr. 1986;64:338-341
4.Gagne C, Gaudet D, Bruckert E; Ezetimibe Study Group. Efficacy and safety of ezetimibe coadministered with atorvastatin or simvastatin in patients with homozygous familial hypercholesterolemia. Circulation. 2002; 105:2469-2475