New approaches to the management of hepatitis and endocrine disorders in Cooley's anemia

Abstract

Hepatitis C infection is common in patients receiving life-long blood transfusion therapy. Interferon-alpha induces long-term viral clearance in 25-30% of patients suffering from Cooley's anemia. Ribavirin, an orally active guanoside analogue together with interferon-alpha produces a sustained response in up to 40% of patients with cirrhosis, who had previously failed single agent treatment. Growth retardation in iron-overloaded patients is the result of growth hormone deficiency in up to 30% of patients. Height gain can be successfully achieved in these patients with growth hormone treatment. Pregnancy in women with Cooley's anemia is now a reality, and over 100 pregnancies have been documented. Conception may be spontaneous or the result of ovulation induction. Cardiomyopathy and diabetes require careful assessment in these patients before a decision is made to treat with gonadotrophins to induce ovulation.