New approaches to the management of hepatitis and endocrine disorders in Cooley's anemia.
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.
Department of Haematology, Whittington Hospital, London.
B
B
Wonke
A V
AV
Hoffbrand
P
P
Bouloux
C
C
Jensen
P
P
Telfer
D000998:Antiviral Agents / Q000627:therapeutic use; D002648:Child; D004700:Endocrine System Diseases / Q000209:etiology / Q000628:therapy*; D005260:Female; D006128:Growth; D006526:Hepatitis C / Q000209:etiology / Q000628:therapy*; D006801:Humans; D015994:Incidence; D016898:Interferon-alpha / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000209:etiology / Q000628:therapy; D010024:Osteoporosis / Q000453:epidemiology; D011247:Pregnancy; D011248:Pregnancy Complications; D012254:Ribavirin / Q000627:therapeutic use; D012727:Sex Characteristics; D017086:beta-Thalassemia / Q000150:complications* / Q000628:therapy*
D000998:Antiviral Agents; D016898:Interferon-alpha; D012254:Ribavirin