Therapeutic management of neonatal hemochromatosis: report of four cases and literature review.
Neonatal hemochromatosis (NH) is a rare disease of iron metabolism that starts at intrauterine period causing liver failure and extrahepatic siderozis. The etiology of NH has not been understood exactly, yet it is accepted that a maternofetal alloimmune disorder that leads to liver failure in fetus causes the illness. The prognosis of NH is generally bad and death is inevitable if left untreated. The efficiency of chelation-antioxidant coctail used in medical treatment is between 10% and 20% and these patients frequently need liver transplantation. In our study, we presented four newborn cases diagnosed as NH and treated medically. Of the four patients, one died of pulmonary hemorrhage and another died of multiorgan failure in the first week of hospitalization. The other two patients' clinical status and laboratory parameters recovered with medical treatment. However, since liver transplantation was not carried out, one of these patients died at the age of two and a half months and the other at eighth month due to sepsis. In this study, we would like to emphasize the importance of early liver transplantation in patients recovered with medical treatment.
Division of Neonatology, Selcuk University Meram Medical Faculty, Konya, Turkey. aliannagur@yahoo.com
Ali
A
Annagür
Hüseyin
H
Altunhan
Hasan Ali
HA
Yüksekkaya
Rahmi
R
Örs
D000111:Acetylcysteine / Q000627:therapeutic use; D000975:Antioxidants / Q000627:therapeutic use; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D006432:Hemochromatosis / Q000175:diagnosis* / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D007231:Infant, Newborn; D012643:Selenium / Q000627:therapeutic use; D017262:Siderophores / Q000627:therapeutic use; D014810:Vitamin E / Q000627:therapeutic use
D000975:Antioxidants; D017262:Siderophores; D014810:Vitamin E; D012643:Selenium; D003676:Deferoxamine; D000111:Acetylcysteine