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PubMed1981

Vitamin C and iron.

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Administration, Oral↗
PubMed1992

Iron overload in Africa. Interaction between a gene and dietary iron content.

BACKGROUND AND METHODS: In contrast to hemochromatosis, which in white populations is inherited through a gene linked to the HLA locus, iron overload in sub-Saharan Africa is believed to result solely from increased dietary iron derived from traditional home-brewed beer. To examine the hypothesis that African iron overload also involves a genetic factor, we used likelihood analysis to test for an interaction between a gene (the hypothesized iron-loading locus) and an environmental factor (increased dietary iron) that determines transferrin saturation and unsaturated iron-binding capacity. We studied 236 members of 36 African families chosen because they contained index subjects with iron overload. Linkage to the HLA region was tested with use of lod scores. RESULTS: In the index subjects, increased iron was present in both hepatocytes and cells of the mononuclear-phagocyte system. Among family members with increased dietary iron due to the consumption of traditional beer, transferrin saturation in serum was distributed bimodally, with 56 normal values (less than 60 percent saturation) and 44 elevated values; the mean serum ferritin concentration was five times higher in the subjects with elevated transferrin saturation (P less than 0.005). The pedigree analysis provided evidence of both a genetic effect (P less than 0.005) and an effect of increased dietary iron (P less than 0.005) on transferrin saturation and unsaturated iron-binding capacity. In the most likely model, increased dietary iron raised the mean transferrin saturation from 30 to 81 percent and lowered the mean unsaturated iron-binding capacity from 38 to 13 mumol per liter in subjects heterozygous for the iron-loading locus. The hypothesis of tight linkage to HLA was rejected. CONCLUSIONS: Iron overload in Africa may be caused by an interaction between the amount of dietary iron and a gene distinct from any HLA-linked gene.

Adult↗
PubMed1978

Separation of younger red cells with improved survival in vivo: an approach to chronic transfusion therapy.

Transfusion of donor blood containing predominantly younger red cells with prolonged survival in vivo could significantly reduce the iron overload of patients requiring chronic transfusion. Age-dependent separation of red cells can be obtained by buoyant density centrifugation on isotonic solutions of arabino-galactane. By this technique, rabbit red cells were separated on a single layer of arabino-galactane and the appropriate fraction, after being labeled with (51)Cr, was reinfused into the donor. The survival in vivo was calculated by a mathematical model which corrects for both (51)Cr elution and random loss. There was a significant difference in survival in vivo between the light young red cells and the heavy old red cells. The potential survival in vivo of the 50% lightest red cells was 56 days, compared to 28 days for the heaviest red cells. Arabino-galactane appeared to be devoid of acute toxicity and of strong antigenicity and it did not appear to adhere to the red cell stroma. These data extrapolated to humans indicate that it may be feasible and advantageous to use red cells fractionated by this technique for transfusion. The 50% lightest human red cells can be expected to have a mean survival of 88 days, compared with 60 days for unfractionated blood. Transfusion of young red cells could significantly reduce the iron overload for patients requiring chronic transfusion, by avoiding infusion of the oldest red cells, which contribute equally to iron overload yet offer only transient survival in vivo.

Animals↗
PubMed1995

Siderophores: structure and function of microbial iron transport compounds.

Siderophores are common products of aerobic and facultative anaerobic bacteria and of fungi. Elucidation of the molecular genetics of siderophore synthesis, and the regulation of this process by iron, has been facilitated by the fact that E. coli uses its own siderophores as well as those derived from other species, including fungi. Overproduction of the siderophore and its transport system at low iron is in this species well established to be the result of negative transcriptional repression, but the detailed mechanism may be positive in other organisms. Siderophores are transported across the double membrane envelope of E. coli via a gating mechanism linking the inner and outer membranes.

Agriculture↗
PubMed2006

Cochlear implantation in superficial siderosis.

Superficial siderosis (SS) of the central nervous system has been thought to be a rare condition that generates progressive hearing loss, ataxia, pyramidal signs, and dementia. The main cause of hearing loss by SS is thought to be neuronal. Because there is no histopathologic report of the human temporal bone in SS, there is a debate about the possibility of cochlear involvement. We present a 25-year-old man who was investigated for bilateral progressive sensorineural hearing loss and vestibular failure after head trauma. On brain MRI, SS of the central nervous system was detected. Distortion product otoacoustic emission (DPOAE) and auditory brainstem response (ABR) showed no response on both sides. However, integrity of the eighth nerve was proved by the electrical ABR test on the right side and the patient benefited significantly from cochlear implantation. The sensorineural hearing loss in SS seems to be related to cochlear damage as well as neuronal damage. So, cochlear implantation would be a hearing rehabilitation modality for the sensorineural hearing loss caused by SS.

Adult↗
PubMed1993

Hepatobiliary changes in Nigerians with sickle cell anaemia.

The histology of a liver specimen from the autopsy of each of 58 sickle cell anaemia patients aged 3-45 years was examined. Sinusoidal distension (present in all specimens), haemosiderosis and erythrophagocytosis (56), portal triaditis (47), cholestasis (24), focal necrosis and fibrosis (23), and extra-medullary erythropoiesis (3) were observed. The livers from the older patients (30-45 years) showed severe haemosiderosis, chronic inflammation, pigment stones and fatty change.

Adolescent↗