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A

aceruloplasminemia: lack of ceruloplasmin, a copper-containing serum protein essential for normal function of transferrin iron transport

acidosis: excessive accumulation of compounds such as carbon dioxide that cause reduction of serum pH values to below 7.4

adrenals: a pair of endocrine glands that rest above kidneys; the outer layer (cortex) forms an array of steroid hormones; the middle layer (medulla) produces adrenaline (epinephrine)

ATDS: acquired immune deficiency syndrome; caused by a retrovirus that is transmitted congenitally or by contact with infected body fluids; the virus attacks T-lymphocytes,thus markedly increasing risk for opportunistic infections and cancers

alkalosis: elevation of serum p1l value due to reduced level of carbon dioxide by prolonged vomiting, too rapid breathing, congestive heart failure

allele: one of the variant forms of a gene at a particular location on a chromosome

ALP (alkaline phosphatase): liver enzyme that can concentrate in liver and bone. increased serum levels may indicate cirrhosis, hepatoma or biliary obstruction

alpha-fetoprotein (AFP): oncofetal protein produced by fetal liver during first trimester. Increased maternal serum level may indicate neural tube or abdominal wall defect in fetus. Increased non-maternal AFP may indicate hepatoma or other cancers or liver cell necrosis.

ALT (alanine transaminase): liver enzyme (also see SGPT); increased serum level indicates liver cell injury

alveoli: tiny air sacs in the lungs in which exchange of carbon dioxide and oxygen occur

Alzheimer’s disease: presenile dementia associated with cortical cerebral sclerosis; a chronic progressive disorder that accounts for over 50% of cases of dementia

amenorrhea: absence of menstruation

amino acids: small molecules that are building blocks of proteins. Of 20 essential amino acids, ten must be acquired through diet.

aminoglycosides: a class of antibacterial antibiotics that includes amikacin, gentamicin, kanamycin, neomycin, netilomycin, streptomycin and tobramycin. Can combine with iron to cause damage to kidneys and hearing.

amosite: a form of asbestos with a high content of iron that is carcinogenic

amylase: an enzyme that digests starch

amyloidosis: disorder in which starch-like glycoproteins (amyloids) accumulate in tissues and impair function

ANA (anti-nuclear antibody): detected in serum in patients who have systemic lupus erythematosis (SLE), a chronic auto-immune disease

anemia: reduction below normal in number of circulating red blood cellsanemia, aplastic: lack of red blood cell production due to bone marrow failure

anemia, Cooley's: (thalassemia) chronic, hemolytic anemia due to an inherited gene mutation influencing hemoglobin formation

anemia of chronic disease (ACD) mild anemia that accompanies the body's inflammatory defense mechanisms during episodes of infection, cancer and other specific disorders

anemia, iron deficiency: caused by blood loss, lack of iron assimilation, or rapid growth when iron needs exceed intake and stores

anemia, pernicious: caused by inadequate absorption of vitamin B12 due to the absence of intrinsic factor, a chemical secreted by mucous membranes of stomach anemia, pyridoxine-responsive: corrected by treatment with pyridoxine (B6)

anemia, sickle cell: chronic, hemolytic anemia due to an inherited gene mutation influencing hemoglobin formation; during crisis episodes, red blood cells with sickle shape are detected

anemia, sideroblastic: anemia in which iron is deposited prematurely in developing red blood cells which then are impaired in oxygen transport

angiography (arteriography): radiopaque contrast material is injected into the blood vessel during x-ray filming to detect blood flow abnormalities

angiotension-converting enzyme (ACE) inhibitor: a drug employed to lower blood pressure

anisocytosis: presence of red blood cells with increased variability as measured by red cell distribution width (RDW)

anterior pituitary: master endocrine gland at base of brain; its hormone producing cells are very sensitive to iron toxicity

antibody: specific protein(s) formed by B lymphocytes that can combine with and neutralize specific antigen(s)

antigen: a substance that stimulates the formation of a specific antibody and which will combine with that antibody

antioxidant: a chemical that can neutralize or destroy oxygen radicals that have been formed by the catalytic action of iron

apheresis: a procedure for treatment of iron overload or sickle cell anemia. The blood is filtered to enable removal of red blood cells or other blood components.

apoptosis: programmed cell death, the body's normal method of disposing of damaged, unwanted or unneeded cells

arrhythmia: lack of normal heart beat; may be due to iron loading of cardiac cells

arthralgia: pain in a joint

arthropathy: any joint disease

ascites: accumulation of fluid in the abdomen; may be a complication of cirrhosis, congestive heart failure, kidney malfunction, cancer, peritonitis, or various fungal and parasitic diseases

AST (aspartate transaminase): liver enzyme; increased serum level indicates liver cell injury

atherosclerosis: thickening, loss of elasticity, calcification of artery walls

atransferrinemia: congenital disorder in which little or no transferrin is produced

atrophy: loss of mass and function of a tissue or organ

autoimmune disease: a disorder in which the body's immune system attacks itself

autosomal dominant: inheritance in which a gene mutation on a non-sex chromosome carries the defect and is sufficient for expression

autosomal recessive: gene is located on a non-sex chromosome (autosome); inheritance in which two gene mutation copies, one from each parent, are required for expression of the disorder

basal ganglia: collection of nerve cell bodies

benign: non-cancerous tumor or growth that does not interfere with normal function

bilirubin: red blood cell waste product in bile; elevated amount in serum and skin causes jaundice

biopsy: removal of a small amount of tissue or fluid, usually by needle, for laboratory examination as an aid in diagnosis

bone marrow: soft tissue in sternum and long bones; red portion forms various types of blood cells

bone marrow aspiration: biopsy to determine such conditions as anemias, leukemias, iron deficiency, iron excess, tumors

bone marrow transplant: therapeutic use of bone marrow from healthy, antigen-matched donor in patients who have a variety of neoplastic or metabolic diseases

BUN (Blood urea nitrogen): a serum test that measures urea nitrogen; used to determine liver and kidney function

cachexia: weakness, loss of appetite, emaciation associated with serious infection or cancer

calcification: deposition of blood calcium into tissues in injury, infection or aging; can be part of healing but may lead to impaired organ function

calcium: mineral essential for heartbeat regulation, muscle contraction, nerve impulse transmission, formation of bones and teeth

Candida: a genus o~ vca,3ts (fungi) that normally occurs in the mouth, intestine and vagina. Abnormal growth of Candida results in infection called candidiasis

carcinogen: a chemical or radioactive agent that induces normal cells to become malignant

cardiomyopathy: condition in which heart muscle cells are damaged

cardiotoxicity: damage to heart muscle cells as by certain anti-cancer drugs (e.g., adriamycin) that combine with iron; an iron-trapping agent (dexrazoxane) may be employed with the anti-cancer drug

case-control study: comparison of cases with healthy controls matched for such factors as age and gender

celiac disease: congenital (present at birth) disorder caused by an allergic intolerance to gluten, a protein constituent of most grains

cerebellum: the portion of the brain lying below the cerebrum and above the pons and medulla

cerebrospinal fluid: (CSF) a clear, normally colorless and blood-free fluid that cushions and nourishes the brain and spinal cord

ceruloplasmin (Cp): a copper-containing serum protein essential for the normal transport of iron by transferrin

chelator: a chemical that can tightly bind one or more transition series metals such as iron, manganese, zinc, or copper

cholelithiasis: gallstones

cholesterol: principal animal sterol; normally present in all body tissues, especially brain, spinal cord and all animal fats

chondrosarcoma: a cancer arising in cartilage cells

chromosome: nuclear structure that contains the genes

chrysotile: form of asbestos that is comprised mainly of magnesium (rather than iron) silicate and which is much less carcinogenic than forms that contain high iron

cirrhosis: a chronic, progressive, inflammatory disease of liver; degeneration and death of parenchymal (functional) liver cells; distortion of normal architectural pattern of liver

colitis: inflammatory condition of large intestine

collagen: albuminoid substance of the white fibers of connective tissue, cartilage and bone

colony-stimulating factors: cytokines (hormones) that stimulate the formation of various kinds of blood cells

colostrum: the first milk secreted after the birth of the child; has an unusually high content of lactoferrin which is instrumental in suppressing potential pathogenic bacteria in the infant intestine

congenital: existing at birth

connective tissue disease (collagen disease): various auto-immune conditions characterized by inflammatory changes in small blood vessels and connective tissue

coronary: encircling, for example the blood vessels that supply the heart muscle; a "coronary" indicates blockage of blood supply to the heart coronary thrombosist blood clot obstructing flow in a coronary artery cortisol: steroid hormone produced in cells of adrenal cortex

creatinine: waste product of protein metabolism; increased level in serum indicates kidney insufficiency

crocidolite: form of asbestos with very high content of iron silicate; thus, highly carcinogenic

Crohn's disease: chronic autoimmune inflammatory condition of various sites in gastrointestinal tract

cytokine: hormone-like peptides produced by various cells that act on other cells to stimulate or inhibit their function

cytopenia: a deficiency of cells in the blood

cytotoxic: destructive to cells

DCTI: divalent cation transporter #1; enables enterocytes to accumulate various essential metal ions from diet

Delaney amendment: passed by U.S. Congress in 1970s to prevent adulteration of U.S. foods by carcinogens. Unfortunately, iron (a well-known carcinogen) was exempted because its addition to foods in the U.S. had begun in earlier decades

deoxyribonucleic acid (DNA): the chemical in the cell nucleus that carries the genetic instructions for producing new cells

Deferiprone: oral iron chelating drug used by thalassemic patients in India and Europe; not yet available in U.S.

Desferal: injectible iron chelating drug used by thalassemic patients world-wide

diabetes (mellitus): inability to metabolize glucose due to lack of insulin production (jzype 1) or lack of insulin efficacy or availability (type 2)

dialysis: filtration of waste products from body fluids of patients with kidney insufficiency

dilated cardiomyopathy: heart muscle disease causing enlargement of chambers and impairment of pumping efficiency

Down's syndrome: congenital neurological disorder; iron loading and neuropathology similar to that of Alzheimer's disease

doxorubicin: an antibiotic used to treat several forms of cancer; an example is adriamycin

dyserythropoiesis: abnormal red blood cell synthesis: abnormal chromatin pattern, bizarre shapes, nuclear fragmentation

echocardiography: a test that bounces sound waves off the heart to produce pictures of its internal structures

endocarditis: serious bacterial infection of the membrane lining the heart and of the valves or heart muscle

endocrine glands: organs such as pituitary, pancreas, adrenals, thyroid,

endothelium: cells that form the inner lining of the heart, blood vessels, lymph channels and various body cavities

enzymopathy: disease that results from inability by the patient's cells to form a specific enzyme erythrocyte: red blood cell

erythropoeitin (EPO): a hormone produced by the kidneys that stimulates the formation of red blood cells in the bone marrow

esophageal varices: enlarged veins in lining of esophagus subject to severe bleeding; often appear in patients who have liver disease estrogen: female sex hormone produced by ovaries

erythrocyte sedimentation rate (ESR): test for evidence of inflammatory activity

ferritin: a large protein formed by nearly all living cells to store excess iron; synthesis is increased during infectious and neoplastic cell invasions as well as in response to iron loading

ferroportin: an iron export protein; a mutation in the ferroportin gene may lead, to high macrophage iron and is associated with African siderosis fibrosis: abnormal formation of connective or scar tissue

folic acid: part of the B-complex of vitamins; needed for normal synthesis and function of red and white blood cells

frataxin: a mitochondrial protein; deficiency results in mitochondrial iron accumulation, deficits in mitochondrial enzymes, and cell death gamma globulin: fraction of serum proteins that contain antibody molecules

gastrointestinal tract: esophagus, stomach liver, pancreas, gall bladder gene amplification- an increase in the copies of any particular piece of DNA

gene expression: the process by which proteins are made from the instruction coded by DNA gene transfer: insertion of unrelated D14A into the cells of an organism

genetic marker: a segment of E14A with an identifiable physical location on a chromosome and whose inheritance can be followed

genetic mutation: a substitution of one of the DNA bases to result in an altered amino acid insertion in the gene product

germ line: inherited material that is passed on to the offspring 

GGT (gamma glutamyl transpeptidase): an enzyme predominantly in liver; increased levels in serum indicate liver disease such as hepatitis, cirrhosis

globulins: class of proteins soluble in dilute saline and differentiated from albumens by molecular size and charge

glucose-6-phosphate dohydrogenase (G6PD); an enzyme; inability to form the enzyme is inherited; the deficiency can result in red blood cell hemolysis if sulfonamide or specific antimalarial drugs are used; repeated hemolysis can lead to iron loading

glycogen: carbohydrate polymer formed from glucose,stored mainly in liver

gonadotroph: gonad-stimulating hormone produced by anterior pituitary

granulocyte: circulating white blood cell; at site of invasion, cell releases its granules which contain anti-infective agents including lactoferrin, a powerful iron binding protein

granuloma: a focalized nodule of inflammatory tissue at an invasion site

growth hormone (GH): a hormone produced by the anterior pituitary that stimulates cell growth

HA-A (hepatitis-associated antigen): a protein used to detect hepatitis A

haptoglobin: a liver protein secreted into serum to combine with hemoglobin released from lysed red blood cells; persons who inherit inability to make efficient types of haptoglobin have elevated serum iron

HbA1c: a monitor of blood sugar variation over time; elevation indicates periods of blood sugar elevation

HBV: hepatitis B virus

HCV: hepatitis C virus

Helicobacter pylori: gram negative bacterium that grows in stomach mucosa and which derives nutritional iron from human lactoferrin; associated in some, but not all, persons with gastritis, ulcers and stomach cancer

hematocrit: the percentage of the whole blood volume that is occupied by the formed elements (red cells, white cells, platelets)

hematuria: blood in urine

heme: the iron containing porphyrin within the hemoglobin molecule

hemochromatosis: genetic metabolic disorder, mainly in Caucasians, in which excessive ingested iron may accumulate in liver, pancreas, heart, anterior pituitary, skin, joints, etc to result in decay of functions of the involved organs

hemoglobin: the respiratory protein of erythrocytes using the porphyrin iron to take up oxygen or to release it

hemoglobin electrophoresis: a test that identifies abnormal forms of the protein as in hemoglobin C and H disease, sickle cell disease, and various forms of thalassemia

hemoglobinopathy: a disease in which abnormal hemoglobin molecules are formed

hemolysis: lysis (rupture) of cell membrane of red blood cells to release hemoglobin into the environment

hemolytic jaundice: severe hemolytic anemia that results in a high level of free bilirubin and which leads to a jaundiced appearance hemorrhage: escape of blood from circulatory system

hemosiderin: insoluble complex of ferric hydroxide and protein debris derived from excessive ferritin in iron loaded cells

hemosiderosis: excessive iron in tissues; associated with a great variety of inherited metabolic disorders or from environmental exposure to iron containing substances

hepatitis A: viral infection of liver contracted by ingestion of contaminated food or water; a vaccine is available

hepatitis B: viral infection of liver contracted by exposure to contaminated blood and other body fluids, contaminated needles, etc. a vaccine is available

hepatitis chronic: inflammation of the liver persisting for more than six months; can be due to hepatitis B or C, alcohol, drugs, toxic chemicals, or autoimmune processes

hepatocellular injury: damage to liver cells

hepatoma: malignant tumor whose primary site is the liver

hepatotoxicity: destructive effect on the liver caused by alcohol or other chemicals

hepcidin: liver hormone that inhibits intestinal absorption of excessive iron and, during inflammatory episodes, suppresses macrophage release of iron

heterozygote: an individual who has two dissimilar members of a gene; a carrier of a gene mutation

HFE: protein that combines with transferrin receptor to suppress cellular uptake of excessive iron; specific mutations in the HFE gene can result in hemochromatosis histology: the microscopic study of cells and tissues

HIV: human immunodeficiencv virus (see AIDS)

HLA: human leukocyte antigen; used in determining compatibility of tissue transplants for host recipients

homozygote: an individual who has two identical members of a mutated gene

hormone: chemical produced by cells in one tissue or gland that acts, on cells in other tissues (see cytokine)

Huntington's disease: chronic, progressive hereditary chores (irregular, movements, speech disturbance, increasing dementia)

hypercalcemia: elevated level of serum calcium; often associated with cancers

hypersplenism: proliferation of splenic cells associated with hemolytic conditions

hypertrophic cardiomyopathy: thickening of heart muscle walls, interfering with normal heart function

hypochromic erythrocytes: subnormal content of hemoglobin in cells

hypoferremia: subnormal content of serum iron

hypothyroidism: underactive thyroid gland; may occur in hemochromatosis if anterior pituitary (which normally stimulates thyroid) is injured by iron

hypoxia: lowered availability of oxygen

hysterectomy: total or partial removal of uterus; may be followed by onset of iron loading

ichthyosis: dry harsh skin with adherent scales idiopathic: condition for which no cause is yet known

immunoglobulins (IgG,IgA,IgM) antibodies

infarction: a portion of tissue that undergoes necrosis (cell death) due to loss of blood supply

infection: condition caused by invasion of bacterial, fungal, protozoan or viral pathogens

inflammation: the reaction of tissues to injury

insulin: the antidiabetic hormone produced by pancreatic beta cells

interferons: specific cytokines that participate in inflammatory defense

interstitial: occupies space between tissues

intravenous: into a vein

intrinsic factor: hormone produced in stomach mucosa essential for assimilation of vitamin B 12 from diet

iron dextran: polysaccharide complex of iron employed for intramuscular or intravenous injection of the metal

iron doping: consumption or injection of excessive amounts of iron with the hope that this will improve athletic performance

iron panel: series of tests that measure levels of iron in serum and other tissues

iron overload: a potentially fatal condition in which iron accumulates in various tissues as in some hemolytic anemias, hemochromatosis, and excessive number of blood transfusions

ischemia: decreased blood supply to a body organ or part

jaundice: elevated bilirubin in blood due to liver disease resulting in yellow whites of eyes and skin, dark urine

juvenile hemochromatosis: onset of iron loading prior to age thirty

ketoacidosis: a complication of inadequately treated diabetes mellitus; may also occur in starvation

knockout: inactivation of specific genes in yeast or mice to determine identity of specific function coded by the gene

koilonychia: atrophic deformity of the nails

Kupffer cells: fixed macrophages that line liver sinusoids

lactoferrin: a protein that strongly combines with iron in body fluids and tissues to deprive invading pathogens of the metal

lamina: a thin flat layer or membrane

Langerhans islets: collection of cells in the pancreas that produce insulin

LDL: low density lipoprotein: combines with cholesterol in plaque formation

Legionnaire's disease: a severe, often fatal, non-contagious pneumonia due to inhalation of the pathogen, often from water-borne aerosols

leukemia: white blood cell neoplasm resulting in high concentration of the malignant cells in the blood

leukocyte: circulating white blood cells (neutrophils or granulocytes, eosinophils, basophils)

lipase: fat splitting enzyme in blood and pancreatic secretion

liver function tests: serum assay of levels of such liver enzymes as ALT, (SGPT), AST (SGOT) GGT and ALP; elevation is modest in iron loading, much higher in viral hepatitis

locus: the place on a chromosome where a specific gene is located

lymph: fluid in lymphatic vessels

lymphocyte: circulating white blood cell required for antibody synthesis (B cell) or for inducing cell mediated immunity (T cell)

lymphoma: cancer of a lymphocyte node

lysozyme: enzyme in body fluids that digests bacterial cell walls

macrophage: large white blood cell in spleen, lymph nodes, and many tissue locations; scavenge debris, recycle aged erythrocyte membranes and hemoqlobin, phagocytose and kill microbial invaders

malabsorption: inadequate assimilation of nutrients from the small intestine

malignant: dangerous to life; cancerous

melanoma: a cancer derived from cells that contain melanin pigment

Mendelian inheritance: manner in which genes are passed to offspring; examples include autosomal dominant, autosomal recessive, and sex-linked genes

metabolism: chemical processes that consist of building cell constituents (anabolism) and digesting cell constituents (catabolism)

metacarpals: bones of the hand to which finger bones are attached

metastasis: spread of cancer cells or infectious microbes from their original location to other tissues of the body

MI: myocardial infarction (heart attack)

microcytosis: abnormally small erythrocytes microbes: bacteria, fungi, protozoa; some are pathogenic

mitochondria: self replicating portion of the cell; metabolic function provides energy to the host cell

monocyte: circulatinq macrophage

multiple myeloma: malignancy beginning in plasma cells of bone marrow

myoglobin: small globular protein with a heme porphyrin in muscle; stores oxygen needed for muscle function

NASH: nonalcoholic steatohepatitis; fatty liver not caused by alcohol consumption; can be associated with C282Y mutation, patients have elevated serum ferritin, insulin resistance

neoplasia: a new and abnormal formation of tissue as a tumor

necrotizing enterocolitis : severe infectious bacterial invasion of intestinal lining in non-breast fed infants who lack maternal lactoferrin

nephrotoxicity: damage to kidneys

neuropathy: damage to nerve tissue

neutropenia: white blood cell count below normal

neutrophil: see granulocyte

oncogene: a gene that can cause transformation of normal cells into cancer cells

oncology: study of cancer

osteoarthritis: degenerate joint disease; can be an early sign of iron loading

osteoporosis: loss of bony substance producing brittleness and bone softness; in some cases, associated with iron loading

pancreas: a large gland in upper portion of abdomen with an exocrine and endocrine function; in former, digestive enzymes are secreted into intestine; in latter, insulin is secreted into blood

parathyroid: four small endocrine glands in neck behind thyroid gland; hormone secreted controls calcium and phosphate metabolism

parenchyma: cells in an organ that are responsible for specific function(s) of the organ; for example, the hepatocytes in the liver

parenteral: subcutaneous, intramuscular or intravenous injection of drugs or nutrients to bypass the intestinal route

Parkinson's disease: a neuropathy involving a rhythmic tremor and rigidity of muscle action; associated with accumulation of iron in substantia nigra portion of brain

pericardial: pertaining to membranes surrounding the heart

pernicious anemia: chronic anemia that occurs with Vitamin B12 deficiency due to lack of intrinsic factor

phagocyte: see macrophage

petechiae: dot-size hemorrhaqes into the skin or mucous membranes

Phenotype: an observed trait or characteristic; for example, the increased intestinal absorption of iron in hemochromatosis

phlebotomy: therapeutic withdrawal of blood

phytates: natural compounds in grains that suppress intestinal absorption of excessive non-heme iron

plasma: the liquid portion of the blood and lymph

platelets: small, colorless disks in blood that are essential for clotting

platonychia: thickened spots in center of fingernails

polycythemia: above normal level of red blood cells

porphyria: pathologic levels of porphyrins in body fluids, feces, urine

porphyria cutanea tarda: photosensitivity, increased skin porphyrins; hepatic dysfunction; associated with iron loading and often with hepatitis C

porphyrins: small molecules that contain heme; present in hemoglobin, myoglobin, cytochromes

preeclampsia: toxic complication of pregnancy; increased blood pressure and serum iron; kidney damage; edema

proteins: large molecules comprised of chains of amino acids; include enzymes, antibodies, and many structural components of cells and tissues

pruritis: itching

PSA: prostate specific antigen; level may rise in some cases of prostate cancer

pulmonary edema: fluid accumulation in lungs

pyelonephritis: bacterial infection of one or both kidneys pyridoxine: vitamin B6

Q-T wave: an electrocardiogram measure; in iron loaded heart (and also in congestive heart failure) the Q-T wave is lengthened

quantitative phlebotomy: calculation of total body iron by means of the number of pints of blood phlebotomized

recessive trait: for phenotypic expression, both parents had to have contributed the genetic mutation

red cell indices: tests for size, concentration of hemoglobin in red cells

Reed-Sternberg cell: indicates presence of Hodgkin's disease

renal: pertaining to kidney

restless legs syndrome: increased need to move extremities; decreased level of iron in substantia nigra; possible defect in transferrin receptor synthesis

reticulocytes: young, immature red blood cells

reticuloendothelial system (RES): monocyte/macrophage system; cells primarily involved in defense against microbial and neoplastic cell invaders and in disposal of breakdown products of aged body cells

retina: the light-receptive layer and terminal expansion of the optic nerve in the eye

retinopathy of prematurity: damage to retina in premature infants by exposure of child to excessive iron and oxygen

rheumatoid arthritis: chronic, autoimmune, inflammatory disease of joints; symptoms can be intensified by parenteral iron

sarcoidosis: chronic inflammatory disease of unknown origin that causes small lumps (nodules) in various tissues

septicemia: bacterial infection in which the pathogens have invaded and are multiplying in the blood

serum: blood plasma minus fibrinogen

SGOT (serum glutamic-oxaloacetic transaminase): a test used in cases of suspected coronary occlusion or liver diseases such as hepatitis or cirrhosis

SGPT (serum glutamic-pyruvate transaminase): an enzyme released into the blood by injury or disease affecting the liver

sickle cell anemia (sicklemia): an inherited condition in which abnormal hemoglobin causes red blood cells to form a sickle shape; in cases in which many blood transfusions are given, patient may need to be de-ironed with iron chelating drugs

sideroblastic anemia: disorder in which incorporation of iron into hemoglobin is faulty; the metal accumulates in the red blood cells as they develop to result in "ringed" sideroblasts that fail to mature

sideroblasts: immature red blood cells that contain excess iron

siderosis: a general term for iron leading

Sjogren’s syndrome: an autoimmune disorder characterized by dryness of the mouth and eyes and recurrent salivary gland enlargement

splenectomy: surgical removal of spleen

splenomegaly: enlarged spleen

substantia nigra: a broad, thick plate of large, pigmented nerve cells in the brain; iron loading is associated with development of Parkinson's disease; iron deficit with restless legs syndrome

SIDS (sudden infant death syndrome): unexpected death of normal child under six months of age; no known cause; condition is associated with post-natal iron loading

synovial membrane: thin lining membrane of a joint

thalassemia:(Cooley's anemia): inherited disorder in which abnormal hemoglobin is formed; required repeated blood transfusions result in iron loading with especial impairment of functions of pituitary, heart and pancreas; prevalent in countries bordering the Mediterranean, in the Middle East, and in India and Southeast Asia

TIBC (total iron binding capacity): measurement of the quantity of iron needed to saturate the binding activity of serum transferrin toxemia of pregnancy: sequel to untreated preeclampsia

transaminases: enzymes that transfer an amino group from various amino acids to alpha-ketoglutaric acid to form qlutamic acid

transferrin (siderophilin): protein that transports iron through serum, lymph and cerebrospinal fluid; functions also to remove free (non-protein bound) iron from these fluids

tremolite: form of asbestos consisting entirely of iron silicate; highly carcinogenic especially when used as a whitewash covering in homes

triglyceride: ester of glycerol in which the three hydroxyl groups are combined with a fatty acid

TSH (thyroid stimulating hormone): compound secreted by the anterior pituitary gland to control the release of thyroid hormone from the thyroid gland

UIBC (unbound iron binding capacity): the difference between the TIBC and the serum iron; high in iron deficiency anemia and low in iron overload

urea: the end product of protein catabolism

vascular: pertaining to blood vessels

ventricular fibrillation: rapid, irregular quivering of heart's ventricles with no effective heart beat; can be associated With iron loading of heart muscle

Vibrio vulnificus: a species of gram negative bacteria in coastal seawater and in shellfish; requires highly saturated transferrin iron to grow in body; wounds in coastal waterways and ingestion of raw shellfish can be fatal to iron loaded patients

villi: minute, elongated projections from membrane surfaces

Wilson's disease: inherited disorder of copper metabolism: accumulation of the metal in liver, erythrocytes, central nervous system; anemia, tremors, liver dysfunction, dementia

Yersinia: genus of gram negative bacteria; some species grow in iron loaded body fluids, others require iron loaded macrophages