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Neonatal Hemokromatozis

Neonatal Hemochromatosis

Journal Name:

  • Selçuk Tıp Dergisi

Publication Year:

  • 2011

Key Words:

Keywords (Original Language):

Author NameUniversity of Author
Abstract (2. Language): 
Neonatal hemochromatosis is a rare disease clinically defined as severe neonatal liver disease in association with extrahepatic siderozis. The etiology of neonatal hemochromatosis is not understood exactly. However, according to a theory neonatal hemochromatosis is accepted to be an alloimmune disorder causing liver injury in fetus. After an effected one in the pregnancy the recurrence rate of neonatal hemochromatosis is ~80%. Hepatocellular failure which occurs in the first days of life with coagulopathy, hypoglycemia, hypoalbuminemia, hypofibrinogenemia, thrombocytopenia, anemia, and direct and indirect hyperbilirubinemia characterizes neonatal hemochromatosis. In order to diagnose neonatal hemochromatosis there are some certain criteria that sould be taken into account such as a positive family history, high serum ferritin levels, high serum alpha-fetoprotein levels and siderozis demonstrated with histology or with magnetic resonance. Since an affective medical treatment has not been found yet, liver transplantation is almost always required. The prognosis of neonatal hemochromatosis is generally poor. This review will discuss neonatal hemochromatosis that leads to liver failure in the fetus or newborn.
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Abstract (Original Language): 
Neonatal hemokromatozis, ekstrahepatik siderozis ile birlikte olan ve klinikte şiddetli neonatal karaciğer hastalığı olarak tanımlanan nadir bir hastalıktır. Neonatal hemokromatozisin etiyolojisi tam olarak anlaşılamamıştır. Ancak fetüsta karaciğer hasarına yol açan alloimmun bir bozukluğun neden olduğu kabul edilir. Neonatal hemokromatozisin sonraki gebeliklerde tekrarlama oranı yaklaşık olarak %80'dir. Neonatal hemokromatozis koagülopati, hipoglisemi, hipoalbuminemi, hipofibrinojenemi, trombositopeni, anemi, direkt ve indirekt hiperbilirubinemi ile yaşamın ilk gününde ortaya çıkan hepatosellüler yetmezlik ile karakterizedir. Pozitif aile öyküsü, yüksek serum ferritin düzeyi, yüksek alfa-fetoprotein düzeyleri ve histolojik veya manyetik rezonans görüntüleme ile siderozisin gösterilmesi neonatal hemokromatozis tanısını koymada göz önünde bulundurulan kriterlerdir. Etkili bir medikal tedavisi olmadığı için sıklıkla karaciğer transplantasyonu gerekmektedir. Prognozu genellikle kötüdür. Bu derlemede fetüs ya da yenidoğanda karaciğer yetmezliğine yol açan neonatal hemokromatozis tartışılacaktır.
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  • 4
255-259
  • Turkish

REFERENCES

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