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[pronut-hiv] African Siderosis
- From: "George M. Carter" <fiar@verizon.net>
- Date: Tue, 13 Jun 2006 07:54:03 -0400
http://www.irondisorders.org/Disorders/Siderosis.asp
African Siderosis
African iron overload (AIO) (previously known as African siderosis) is
a condition observed in sub-Saharan Africans. It is characterized by the
accumulation of large amounts of iron in the reticuloendothelial cells of
the liver, spleen and bone marrow as well as in hepatocytes. While the
distribution of iron differs from that seen in classical type 1
haemochromatosisas, organ dysfunction and high levels of body iron of
similar magnitude may be found in AIO. AIO was thought to be solely due to
the consumption of a traditional beer contaminated with iron. The beer has
a high lactic acid content which is thought to be the reason why iron is
leached out of the iron containers in which it is brewed. The iron
concentrations can be as high as 80 to 100 mg per litre and, as the alcohol
content is low (1 to 3%), consumption of several litres per day is
possible. Recent work has suggested that a genetic defect in iron
regulation, together with high dietary iron consumption may be responsible
for AIO. A defect in the iron transporter ferroportin, responsible for iron
release from the gut and reticuloendothelial cells, is thought to be a
possible cause. A mutation in the ferroportin 1 gene has been found to be
common in populations of West and Southern African origin and is associated
with a tendency to higher serum ferritin levels. Other mutations of this
gene are associated with iron overload with a similar tissue distribution
to that seen in AIO.
Risk Groups: Anyone of African descent. Iron overload is a common condition in Africa where the prevalence of excessive body iron sufficient to cause liver damage affects as many as 10% of the population in some areas.
Spectrum of disease: Cirrhosis and its complications including
hepatocellular carcinoma. Osteoporosis secondary to ascorbic acid
deficiency is common in malnourished individuals. Association with heart
disease, elevated sugar or diabetes and increased pigmentation has been
described.
Detection: An attending physician can measure serum ferritin and fasting
serum iron and TIBC (total iron binding capacity) to determine the
transferrin iron saturation percentage (Tsat%). Characteristically, the
serum ferritin rises before the development a high Tsat% and a serum
ferritin greater than 300ng/mL in adult males 200ng/mL adult females with
normal Tsat% (<45%) may indicate AIO. Liver biopsy will show the
characteristic distribution of iron.
Treatment: Therapeutic phlebotomy
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