Therapeutic management of neonatal hemochromatosis: Report of four cases and literature review
Küçük Resim Yok
Tarih
2011
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
SAGE PUBLICATIONS LTD
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
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.
Açıklama
Anahtar Kelimeler
neonatal hemochromatosis, neonatal iron storage disease, acute liver failure, alloimmune disease, liver transplantation, neonate
Kaynak
HUMAN & EXPERIMENTAL TOXICOLOGY
WoS Q Değeri
Q3
Scopus Q Değeri
Q2
Cilt
30
Sayı
10
