Neonatal haemochromatosis with reversible pituitary involvement.
Neonatal haemochromatosis is a rare alloimmune gestational disease with a high mortality. The hallmark of neonatal haemochromatosis is severe neonatal liver failure associated with extrahepatic siderosis. Thus far, no pituitary dysfunction has been reported to result from the tissue damage associated with extrahepatic siderosis. The present report describes a neonate with neonatal haemochromatosis and secondary hypothyroidism associated with pituitary iron deposition. Both the conditions were successfully treated by ABO-incompatible liver transplantation. Pituitary gland dysfunction is another possible extrahepatic manifestation of neonatal haemochromatosis, and it is reversible after liver transplantation.
Paediatric Hepatology, Meyer Children Hospital, Firenze, Italy.
Giuseppe
G
Indolfi
Rita
R
Bèrczes
Isabella
I
Pelliccioli
Michela
M
Bosisio
Cristina
C
Agostinis
Massimo
M
Resti
Marco
M
Zambelli
Alessandro
A
Lucianetti
Michele
M
Colledan
Lorenzo
L
D'Antiga
D000017:ABO Blood-Group System / Q000276:immunology; D001787:Blood Group Incompatibility; D006432:Hemochromatosis / Q000150:complications / Q000628:therapy*; D006801:Humans; D007037:Hypothyroidism / Q000628:therapy; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male; D010900:Pituitary Diseases / Q000209:etiology*
D000017:ABO Blood-Group System
ABO-incompatible liver transplantation; hypopituitarism; hypothyroidism; neonatal haemochromatosis