Haemochromatosis

In haemochromatosis.,total body iron is increased to 20-60 g,with high incidence of HCC.
..Etiology=
.Increased absorptn of dietary iron over years at 40-60 years of age it manifest
..Inheritance as autosomal recessive
.90% pt r males
.PATHOLOGY=
.Excess iron deposited in tissues resulting in damage to liver,pancreas,heart,pituitary nd skin.
.C/F=
.Cirrhosis with hepatosplenomegaly ,loss of libido ,testicular atrophy ,loss of body hair ,jaundice ,ascites
.DM in 60% pt
.Heart failure nd cardiac arrhythmias
.Leaden grey skin pigmentatn due to excess melanin in exposed parts,axillae
.Arthitis nd chondrocalcinosis
.Hypogonadism
.Hepatocellular carcinoma
.INVESTIGATION=
.Plasma iron conc raised
.Plasma iron binding capacity more than 70%
.Serum ferritin levels r raised to more than 600 micro gm/l
.CT scan shows raised density of liver
.Liver biopsy
.MANAGEMENT=
weekly venesection of 500 ml blood until serum iron is normal
.Chelating therapy with desferrioxamine
.Treatment of cirrhosis
.Treatment of diacetes
.Supplement of vitamin c