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Abstract

Neonatal hemochromatosis (NH) is a severe rare liver disease in neonatal period associated with ekstrahepatic siderosis. This disease is characterized by hepatocellular insufficiency that presented with jaundice, hypoglycemia, hypoalbuminemia, low fibrinogen levels, thrombocytopenia, anemia, direct and indirect hyperbilirubinemia from the first days of life. Herein we reported a case with Rh incompatibility whose jaundice was noted at the first day of life and referred to our hospital for exchange transfusion, but thereafter diagnosed as NH and reviewed the literature in the view point of the latest developments related to the topic. Key IncompatibilityNeonatal words: Hemochromatosis, Jaundice, Rh

Keywords

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Nadir Bir Neonatal Karaciğer Yetmezliği Nedeni: Neonatal Hemokromatozis

Abstract

Neonatal ekstrahepatik siderozis ile birliktelik gösteren, nadir görülen ciddi bir karaciğer hastalığıdır. Hepatosellüler yetmezlikle karakterize bu hastalık yaşamın ilk gününden itibaren koagülopati, hipoglisemi, hipoalbüminemi, düşük fibrinojen, trombositopeni, anemi, direkt ve indirekt hiperblirübinemi ile kendini gösterir. Yazımızda annesi ile arasında Rh uygunsuzluğu bulunan, birinci gün sarılığı farkedilen ve kan değişimi yapılmak üzere sevk edilen, ancak neonatal hemokromatozis tanısı konan bir vakayı sunduk ve konu ile ilgili son gelişmeleri gözden geçirdik

Keywords

• Neonatal Hemokromatozis, Sarılık, Rh Uygunsuzluğu

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