Classical Maple Syrup Urine Disease successfully treated with living donor liver transplantation
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Dosyalar
Tarih
2021
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
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Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Maple syrup urine disease (MSUD) is a disease that causes ketoacid accumulation in body. Diffusion-weighted imaging (DWI) is an
important imaging modality for the diagnosis. Two children were diagnosed with MSUD at the neonatal period. They had uncontrolled
ketosis and epileptic seizures although they were in compliance with their medical nutrition. Their DWIs were similar and showed
high signal intensity localized within the myelinated white matter areas. Both of the patients were treated with living donor liver
transplantation. The patients with classic form of MSUD are normal at birth. If the disease is not diagnosed and treated early, it can
lead to serious neurological complications. Most researchers conclude that, the best choice for detecting MSUD encephalopathy
in newborns is DWI. The traditional treatment of MSUD had been a protein-restricted diet until the liver transplantation became an
alternative and better option for the cure of the patients..
Açıklama
Anahtar Kelimeler
Kaynak
Annals of Medical Research
WoS Q DeÄŸeri
Scopus Q DeÄŸeri
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Künye
Mert Dogan, G., Murat Dogan, S., Okut, G., Sigirci, A., & Yilmaz, S. (2021). Classical Maple Syrup Urine Disease successfully treated with living donor liver transplantation . Annals of Medical Research
