- Discussion:
- rare disorder manifested by tissue accumulation of iron affecting
liver pancrease, heart, and gonads;
- approx 1.7% of men & 0.6 percent of women are homozygotes;
- hereditary disorder of iron metabolism
- diabetes is common (bronze diabetes)
- 20% develop joint involvement;
- most pts are male between ages of 40-60 yrs;
- it is associated w/ heavy wine intact;
- produces arthritis and chrondrocalcinosis in 50% of pts;
- treatment:
- phlebotomy can prevent the development of symptoms;
- Lab Studies:
- measuring the transferrin saturation appears to be most sensitive
method of detecting the homozygous genotype;
- threshold normal value of transferrin saturation during fasting be set
at 60 % for men and 50 % for women;
- serum ferritin concentration correlates well w/ level of hepatic iron
stores and has been used as marker in many screening studies;
- Iron:
- Males 65-175 ug/dL; Females 50-170 ug/dL
- Increased:
- Hemochromatosis, Hemosiderosis caused by excessive Iron intake,
excess destruction or decreased production of erythrocytes,
liver necrosis;
- Decreased:
- Anemia of Infection and Chronic dz, Cirrhosis, Nephrosis,
- Iron Binding Capacity (total) TIBC:
- 250-450 ug/dL;
- normal Iron/TIBC ratio is 20-50%; <15% is almost diagnostic of Iron
defficiency Anemia;
- Increased: Acute and Chronic Blood Loss, Iron deficiency Anemia,
Hepatitis, OC;
- Decreased: Anemia of Infection and Chronic dz, Cirrhosis, Nephrosis,
Hemochromatosis;
- Ferritin:
- Male 15-200 ng/mL; Female: 12-150 ng/mL;
- Decreased: Iron Defficiency (earliest & most sensitive test before red
cells show any morphological change);
- Transferrin:
- 220-400 mg/dL
- Increased: Iron Deficiency;
-
Decreased: Poor Nutritional Status, Chronic and Acute Inflammatory
States, Chronic Liver dz;
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Iron supplementation after femoral head replacement for patients with
normal iron stores [see comments.
Hip Arthropathy in Genetic Hemochromatosis: Radiographic and Histologic
Features.
