Hereditary hemochromatosis (HH) is a common autosomal recessive disorder of iron metabolism, with a worldwide prevalence in whites of approximately 3 to 5 per 1000 population.1–3  Unrecognized HH can lead to cirrhosis, diabetes mellitus, hyperpigmentation, and impotence in untreated individuals. Although usually not symptomatic until the ages of 50 to 70, HH has been described in children. Screening first-degree relatives of a person with HH has identified many children although symptomatic HH remains uncommon in children. However, with such a high prevalence of HH it is safe to say that all pediatricians have seen an affected child, but in the asymptomatic period. One of the principle tasks of the pediatrician is prevention; HH is a condition that, identified in the presymptomatic period, can be treated, avoiding potentially life-threatening complications. Recently a genetic defect has been described on the short arm of chromosome 6 that is found in nearly...

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