Management of hemochromatosis : Iron overload, public health, and genetics.
The complications of iron overload in hemochromatosis can be avoided by early diagnosis and appropriate management.
Therapeutic phlebotomy is used to remove excess iron and maintain low normal body iron stores, and it should be initiated in men with serum ferritin levels of 300 mug/L or more and in women with serum ferritin levels of 200 mug/L or more, regardless of the presence or absence of symptoms.
Typically, therapeutic phlebotomy consists of 1) removal of 1 unit (450 to 500 mL) of blood weekly until the serum ferritin level is 10 to 20 mug/L and 2) maintenance of the serum ferritin level at 50 mug/L or less thereafter by periodic removal of blood.
Hyperferritinemia attributable to iron overload is resolved by therapeutic phlebotomy.
When applied before iron overload becomes severe, this treatment also prevents complications of iron overload, including hepatic cirrhosis, primary liver cancer, diabetes mellitus, hypogonadotrophic hypogonadism, joint disease, and cardiomyopathy.
In patients with established iron overload disease, weakness, fatigue, increased hepatic enzyme concentrations, right upper quadrant pain, and hyperpigmentation are often substantially alleviated by therapeutic phlebotomy.
Patients with liver disease, joint disease, diabetes mellitus and other endocrinopathic abnormalities, and cardiac abnormalities often require additional, specific management. (...)
Mots-clés Pascal : Hémochromatose, Conduite à tenir, Diagnostic, Phlébotomie, Traitement, Complication, Evaluation, Efficacité, Homme, Recommandation alimentaire, Fer, Métabolisme pathologie, Enzymopathie, Traitement instrumental
Mots-clés Pascal anglais : Hemochromatosis, Clinical management, Diagnosis, Phlebotomy, Treatment, Complication, Evaluation, Efficiency, Human, Dietary allowance, Iron, Metabolic diseases, Enzymopathy, Instrumentation therapy
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0019422
Code Inist : 002B22E03. Création : 31/05/1999.