(HOME) Subject: siderosis/lung/kidney

   
   Semin Hematol 1998 Jan;35(1):77-86
   
Secondary iron overload disorders.

    Bottomley SS
    
   Department of Medicine, University of Oklahoma College of Medicine,
   Oklahoma City, USA.
   
   Diverse clinical disorders distinct from hereditary hemochromatosis
   are associated with accumulation of excess body iron in heterogeneous
   patterns and through various mechanisms. A deranged iron turnover
   somehow relates to the altered physiological barrier for iron
   absorption in several defined chronic anemias with ineffective
   erythropoiesis. Unexcretable excess iron acquired from transfusions
   provides a therapeutic challenge. Genetic defects of proteins
   essential for transport of iron into and out of cells (transferrin and
   ceruloplasmin) deprive the erythron of the metal and cause its
   accumulation in other vital organs. The hemochromatosis alleles
   predictably contribute to an iron burden from other causes, commonly
   facilitate the expression of porphyria cutanea tarda, and their
   clinical expression may be accelerated by hereditary hemolytic
   anemias. Even minimal iron excess in liver disease may contribute to
   the hepatocellular injury from factors such as alcohol and viruses.
   Uniquely localized siderosis occurs in the lung and kidney where iron
   cannot turn over and causes variable tissue damage. The most
   devastating iron overload disorder, neonatal hemochromatosis, is
   understood least of all.
   
   Publication Types:
     * Review
     * Review, tutorial
       
   PMID: 9460811, UI: 98122143
     _________________________________________________________________

(HOME)