Doğuştan Metabolik Hastalıkların Tedavisinde Ekstrakorporeal Yöntemler

Yıl 2015, Cilt: 24 Sayı: 4, 509 - 519, 15.09.2015

İlknur Tolunay Rıza Yıldızdaş Özden Horoz Ercüment Petmezci Faruk Ekinci Neslihan Mungan

https://doi.org/10.17827/aktd.48450

Cited By: 2

Öz

Doğuştan metabolik hastalıklar, metabolik yollarda kesinti sonucu kesintinin ardında biriken maddelere veya son ürünün yapılamamasına bağlı bir grup kalıtsal hastalıktır. Doğuştan metabolik hastalıklar organ hasarı, sekel, hatta ölümle sonuçlanabilecek durumlara yol açabildiğinden tedavide hızlı ve agresif davranmak gereklidir. Bu yazıda medikal tedaviler dışında kan değişimi, periton diyalizi, hemodiyaliz, plazmaferez, ekstrakorporeal membran oksijenasyon gibi ekstrakorporeal tedavi yöntemleri tartışılacaktır.

Anahtar Kelimeler

Doğuştan metabolik hastalık, ekstrakorporeal tedavi yöntemleri, çocuk yoğun bakım ünitesi

Extracorporeal Methods in Treatment of Inborn Errors of Metabolism

Öz

Inborn errors of metabolism are hereditary disorders that are related to the accumulation of toxic substances or deficiency of proteins because of enzymatic blokage in the metabolic pathways. Treatment should be quick and aggressive as inborn errors of metabolism might lead toorgan damage and even death. This article discusses extracorporeal methods like exchange transfusion, peritoneal dialysis, hemodialysis, plasmapheresis and extracorporeal membran oxygenation for acute management of inborn errors of metabolism apart from medical therapies.

Anahtar Kelimeler

Extracorporeal treatments, inborn errors of metabolism, pediatric intensive care unit

Kaynakça

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• Correspondence Address / Yazışma Adresi