Correspondence To

the editor: Homozygosity for a novel nonsense mutation (G66X) of the HJV gene causes severe juvenile hemochromatosis with fatal cardiomyopathy Juvenile hemochromatosis (JH) is a rare autosomal recessive disorder of iron metabolism. The early onset of severe iron overload and the frequent occurrence of cardiomyopathy, reduced glucose tolerance, and hypogonadism distinguish JH from the more common HFE-related hereditary hemochromatosis.1 JH is genetically heterogeneous: most families are related to the recently cloned hemojuvelin (HJV) gene,2-5 and a small subset of JH patients was shown to harbor mutations in the HAMP gene encoding hepcidin.6-7 A 25-year old man, originating from a small village in Transylvania, Romania, presented with abdominal pain and hepato-megaly, later supraventricular tachycardia and dyspnea. Echocardi-ography revealed reduced ejection fraction (21%), pericardial fluid