Anemia with iron storage

Copper 80 mg 2-3 mg Metal storage/transport (ceruloplasmin) enzymes in synthesis of cartilage, bone, myelin interaction with iron Occurs in malnutrition, TPN anemia, neutropenia, skeletal and neurological defects Danger in Wilson s disease... 

In one prospective study in 17 patients with hemoljdic anemia (aged 5-25 years) lens opacities were found in 41%, changes in the retinal pigment epithelium in 35%, tortuosity of retinal vessels in 24%, dilatation and sheathing of retinal vessels in 18%, defects in color vision in 29%, and abnormal dark adaptation in 18% (56). In many other studies much lower frequencies were found. Perhaps retinal injury is related to the depletion of metals such as zinc, copper, and/or iron (57). On the other hand, ocular and auditory disturbances are not infrequent in patients with thalassemia, iron storage diseases (58,59), or uremia (45), and may be coincidental in patients receiving deferoxamine (60). 

Hemosiderosis, hemochromatosis, and some anemias are conditions associated with iron overload and iron storage diseases. 

The most common cause of iron overload is thalassemia, particularly in the parts of the world where it is prevalent (see earlier section). Indeed, the cardiac complications of iron overload are among the most common causes of death in I-thalassemia major. Sideroblastic anemias are a group of iron-loading disorders, many of which are of unknown cause. In a hereditary type of this disorder, there is deficiency of erythroid specific 5-aminolevulinic acid synthetase in RBC precursors because of mutations involving the X-linked gene that encodes this enzyme. Iron storage is common in patients with congenital dyserythropoietic anemia and may be found in patients with red cell enzyme deficiencies, particularly pyruvate kinase deficiency. ... 

The evidence that ceruloplasmin (Cp) (E.C. 1.12.3,1) is a direct molecular link between copper and iron metabolism is summarized. Copper deficiency results in low plasma Cp and iron, reduced iron mobilization, and eventually anemia, even with high iron storage in the liver. Cp controls the rate of iron uptake by transferrin. Transferrin plays a key role in the availability of iron for the biosynthesis of hemo-globin in the reticulocytes. The ferroxidase activity of Cp results in the reduction of free iron ion generating a conr centration gradient from the iron stores to the capillary system, thus promoting a rapid iron efflux in the reticuloendothelial system. It has been confirmed both in vivo and in the perfused liver that lOr M Cp specifically induces a rapid rise in plasma iron. 

Hemosiderin, a mammalian non-heme iron storage protein with a similar function to ferritin. It contains iron oxyhy-droxide cores similar to those of ferritin, and it has been reported that these cores are present as large, dense, membrane-bound aggregates in vivo. It is assumed that hemosiderin is produced by lysosomal degradation of ferritin or possibly of ferritin polymers. Hemosiderin is deposited in the liver and spleen, especially in diseases such as pernicious anemia or hemochromatosis. The deposits are yellow to brown-red pigments. The iron content of hemosiderin is about 37%. Nonheme iron is also abundantly present in the brain in different forms. In the so-called high-molecular-weight complexes, iron is bound to hemosiderin and ferritin. The total amount of iron may differ in health and disease [F. A. Fischbach et al, J. Ultrastruct. Res. 1971, 37, 495 M. P. Weir, T. J. Peters, Biochem.J. 1984, 223, 31]. 

Hemosiderin an iron storage protein of the mammalian organism, functionally related to Ferritin (see). H. is deposited in the liver and spleen (hemosiderosis), particularly in diseases associated with increased blood destruction, such as pernicious anemia, or with increased iron resorption (hemochromatosis), or even in hemorrhages Most of the deposits are located in the liver, which may contain up to 50 g H., compared with the normal content of 120 to 300 mg H. H. from horse spleen consists of 26-34 % iron(III), and up to 35 % protein (aposiderin). The rest is made up of octasubstituted porphyrin, mucopolysaccharides and fatty acid esteis. 

Patients with CKD suffer from a decrease in erythropoietin production because erythropoietin is produced mainly in the kidneys.4,5 Finally, in patients with anemia of chronic disease, there is a blunted erythropoietin production as well as a diminished response to erythropoietin.9 Anemia of chronic disease also affects iron homeostasis, causing iron sequestration into storage sites and decreasing the amount available to the rest of the body.9... 

The earliest and most sensitive laboratory change for iron-deficiency anemia is decreased serum ferritin (storage iron), which should be interpreted in conjunction with decreased transferrin saturation and increased total iron-binding capacity (TIBC). Hb, hematocrit, and RBC indices usually remain normal until later stages of iron-deficiency anemia. 

In the initial phase of depletion of the iron content of the body, the iron stores maintain normal levels of hemoglobin and of other iron proteins. With exhaustion of storage iron, hypochromic and microcytic anemia becomes manifest. 

Anemia and Poor Growth Children with xerophthalmia and night blind mothers tend to be anemic relative to peers without eye disease. VA-supplemented trials often show improvement in indicators of iron status, including reductions in anemia. Mechanisms involved in this interaction are not clear but may involve enhanced iron absorption, storage, and transport as well as direct effects on hematopoiesis in the presence of adequate iron stores. 

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