Subacute hepatic failure after the perinatal period with haemochromatotic siderosis at the age of 11 months: an unusual perspective on neonatal haemochromatosis.
Neonatal haemochromatosis is a rare disease characterized by the early onset of liver failure, a rapidly deteriorating clinical course and high mortality. An unusual case is presented of subacute hepatic failure in which autopsy findings at the age of 11 mo showed a haemochromatotic pattern of siderosis. Despite the similarity in the pattern of iron distribution to neonatal haemochromatosis, the clinical course was highly atypical in that the infant had later and milder presentation at 1 mo of age and slow progression into liver failure. This case illustrates the heterogeneous nature of the haemochromatotic phenotype in newborns and infants.


CONCLUSIONS
The entity "neonatal haemochromatosis" may reflect a spectrum of diseases with diverse aetiologies. Fulminant liver disease in the newborn period can also result in similar pattern of iron distribution.
Ku
S W
SW
Departments of Paediatrics, and Pathology, Queen Elizabeth Hospital, Hong Kong, PR China. alkul298@hkstar.com
Luk
I S C
IS
Yuen
M K
MK
Wong
C P
CP
D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006432:Hemochromatosis / Q000150:complications* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000209:etiology*; D012806:Siderosis / Q000150:complications* / Q000473:pathology