Nanozyme-synbiotics to improve iron-deficiency anemia.

Hepcidin in HFE-associated hemochromatosis: Another piece of the “iron” puzzle

Decision letter: Iron status influences mitochondrial disease progression in Complex I-deficient mice

Editor's evaluation: Iron status influences mitochondrial disease progression in Complex I-deficient mice

Author response: Iron status influences mitochondrial disease progression in Complex I-deficient mice

1. Ann Chen Wu, MD* 2. Leann Lesperance, MD, PhD* 3. Henry Bernstein, DO*† 1. *Pediatric Health Associates, Hunnewell Ground Children’s Hospital 2. †Associate Professor of Pediatrics, Harvard Medical School, Boston, MA After completing this article, readers should be able to: 1. Determine the most common cause of iron deficiency in the United States. 2. Describe the pathogenesis of iron deficiency. 3. List populations at high risk for iron deficiency. 4. Outline the common signs and symptoms of iron deficiency. 5. Specify the American Academy of Pediatrics recommendations for screening for iron deficiency. In the March and April issues of Pediatrics in Review, we published a two-part article on managing anemia in a pediatric office practice. This article expands on the various tests for iron deficiency, including some relatively new ones. These articles should be read as complementary.—RJH Iron deficiency is the most common nutritional deficiency in the world, responsible for a staggering amount of ill health, lost productivity, and premature death. Although its prevalence in the United States has declined since the late 1960s, iron deficiency with or without anemia still is seen frequently in infants, toddlers, adolescent females, and women of childbearing age. In fact, iron deficiency anemia remains the most common hematologic disease of infants and children. Anemia is defined as a low hemoglobin (Hgb) concentration or red blood cell (RBC) mass compared with age-specific norms. Anemia may be caused by decreased RBC production, increased RBC destruction, or blood loss. Based on the size of the RBC, hematologists categorize anemia as macrocytic, normocytic, or microcytic. Iron is found in different compartments within the body. Total body iron (measured by ferritin), transport iron (measured by transferrin saturation), serum iron, and other hematologic and biochemical markers are used to describe the degrees of iron deficiency. Iron depletion refers to the earliest stage of diminishing iron stores in the setting of insufficient iron supply. Iron deficiency (without anemia) develops as these iron stores are depleted further and begin to impair Hgb synthesis. Finally, iron deficiency anemia results …

*University of Lancashire, Lancashire, United Kingdom; †University of Milano, Milano, Italy; ‡University of Lund, Malmo, Sweden; §Hopital des Enfants Malades, Paris, France; Hopital Necker Enfants-Malades, Paris, France, ¶Umea University, Umea, Sweden; #University of Munich, Munich, Germany; **Free University of Amsterdam, Amsterdam, The Netherlands; ††Royal Veterinary and Agricultural University, Frederiksberg, Denmark; ‡‡CHUV University Hospital, Lausanne, Switzerland; §§University of Liege, Liege, Belgium; Medical University of Warsaw, Warsaw, Poland; and ¶¶University of Glasgow, Glasgow, United Kingdom

IRON IN BIOORGANIC EVOLUTION. An Introduction to the Nature of Iron Transport and Storage. Iron-Dependent Enzymes in Mammalian Systems. Parallels in the Mode of Regulation of Iron Assimilation in All Living Species. Iron and Oxygen: A Dangerous Mixture. STRUCTURE OF PROTEINS OF IRON STORAGE AND TRANSPORT. Transferrin and Lactoferrin. Ferritin. MOLECULAR BIOLOGY OF PROTEINS OF IRON TRANSPORT AND STORAGE. Molecular Biology of the Transferrins. Molecular Analysis of the Transferrin Receptor and Its Gene. Ferritin Gene Structure and Expression. ROLE OF IRON AND PROTEINS OF IRON METABOLISM IN CELL PROLIFERATION AND MALIGNANCY. Iron-Transferrin Requirements and Transferrin Receptor Expression in Proliferating Cells. Ribonucleotide Reductase. Ferritin in Malignant Cells. IRON METABOLISM IN SPECIFIC TISSUES. Comparative Aspects of Iron Transport and Storage in Different Tissues. Developmental Changes in Iron Storage (the Ferritins) and Iron Transport (the Transferrins and Transferrin Receptors). Intestinal Iron Metabolism and Absorption. Erythroid Cell Iron Metabolism and Heme Synthesis. Iron Metabolism in the Reticuloendothelial System. Modulation of the Availability of Intracellular Iron. Iron and Proteins of Iron Metabolism in the Central Nervous System.

Hereditary hemochromatosis: update for 2003

Control of iron metabolism – Lessons from neonatal hemochromatosis

Iron deficiency in children: detection and prevention.

Iron deficiency is a manifestation of celiac disease (CD) usually attributed to a decreased absorptive surface, although no data on the regulation of iron transport under these conditions are currently available. Our aim was to evaluate divalent metal transporter 1 (DMT1), duodenal cytochrome b (Dcytb), ferroportin 1 (FP1), hephaestin, and transferrin receptor 1 (TfR1) expression, as well as iron regulatory protein (IRP) activity in duodenal biopsies from control, anemic, and CD patients. We studied 10 subjects with dyspepsia, 6 with iron-deficiency anemia, and 25 with CD. mRNA levels were determined by real-time PCR, protein expression by Western blotting or immunohistochemistry, and IRP activity by gel shift assay. Our results showed that DMT1, FP1, hephaestin, and TfR1 mRNA levels were significantly increased in CD patients with reduced body iron stores compared with controls, similar to what was observed in anemic patients. Protein expression paralleled the mRNAs changes. DMT1 protein expression was localized in differentiated enterocytes at the villi tips in controls, whereas with iron deficiency it was observed throughout the villi. FP1 expression was localized on the basolateral membrane of enterocytes and increased with low iron stores. TfR1 was localized in the crypts in controls but also in the villi with iron deficiency. These changes were paralleled by IRP activity, which increased in all iron-deficient subjects. We conclude that duodenal DMT1, FP1, hephaestin, and TfR1 expression and IRP activity, thus the iron absorption capacity, are upregulated in CD patients as a consequence of iron deficiency, whereas the increased enterocyte proliferation observed in CD has no effect on iron uptake regulation.

The discovery of the new haemochromatosis gene: Feder JN, Gnirke A, Thomas W, Tsuchihashi Z, Ruddy DA, Basava A, et al. A novel MHC class I-like gene is mutated in patients with hereditary haemochromatosis [Nat Genet 1996;13:399–408

Anemia in pregnancy is a common and significant condition that can adversely affect both maternal and fetal health. Among the various types of anemia, iron deficiency anemia (IDA) is the most prevalent and is primarily caused by insufficient iron intake, impaired absorption, or increased iron demand during pregnancy. Iron plays a crucial role in hemoglobin production, and its transport and regulation are key factors in maintaining adequate iron levels for oxygen delivery. This review explores the mechanisms of iron transport and regulation in pregnancy and their implications for anemia. Understanding these biological processes is essential for improving the diagnosis, treatment, and prevention of iron deficiency anemia during pregnancy. The regulation of iron homeostasis involves a complex network of proteins and hormones that control iron absorption, transport, and storage. Hepcidin, a hormone produced by the liver, is the primary regulator of iron metabolism, modulating the absorption of iron from the gastrointestinal tract and its release from storage sites. During pregnancy, the body adapts to meet the increased iron demand by enhancing iron absorption and mobilizing iron stores. However, disruptions in iron regulation, such as increased hepcidin levels due to inflammation or inadequate dietary intake, can lead to functional iron deficiency, even in the presence of normal iron stores. The review discusses these regulatory mechanisms and their impact on iron deficiency anemia in pregnancy. Keywords: anemia, pregnancy, iron transport, iron regulation, maternal health

Hepcidin: clinical utility as a diagnostic tool and therapeutic target

Hfe Acts in Hepatocytes to Prevent Hemochromatosis