Hereditary Hemochromatosis (HH) is a genetic disorder in which iron levels in the body build up over time. HH is often known as the iron overload disorder. Hereditary Hemochromatosis (HH) and Hemophilia have a number of similarities when both express an excess of iron build up in the body. In a review article published by the Journal of Clinical Pathology, they compared and contrasted the effects of iron on both the arthopathy of HH and HA, blood disorders that react poorly to the presence of excess ferrous iron …show more content…
During the study, rats, mice, rabbits, and monkeys were used in a variety of ways to run specific tests. In the beginning, female FVIII mice were used to compare characteristics of both rFVIIa and rFVIIa-FP. To compare their findings, all of the dosages were consistent throughout the trials. A total of thirty (30) animals were in placed in each lab group and three of the animals received vehicle to determine a baseline level. Blood samples were taken 2, 5, and 30 minutes and 1, 2, 4, 6, 16, 24, and 48 hours after injection. Some of the other animals that were used (per lab group): eight (8) Rats, three (3) rabbits, and two (2) monkeys. Blood samples from the rats were taken at 5, 15 and 30 min and 1, 2, 4, 8 and 24 hour (hr) increments. Samples of blood were harvested from the rabbits at 1, 5, 10 and 30 min, and 1, 2, 4, 8, 24, 48, 72, 96 and 168 hrs. Blood samples from the monkeys were drawn at 5 and 15 min, and 1, 2, 4, 8, 24, 48, 72, 96 and 120 hrs. This data was analyzed and tabulated into table …show more content…
Each treatment should be treated case-by-case to ensure that the patient is receiving the best possible care. Suggested treatments consist of regular infusions of DDAVP and/or clotting factor, first aid for minor cuts, physical therapy, fibrin sealants and clot-preserving medications. For Hemophilia C, clotting factor XI is available only in Europe and in the United States bleeding episodes are treated through plasma infusions. Clotting factor XI is not available in the United States because Factor XI concentrates are blood products with a concentrated form of Factor XI. Meanwhile, available only in certain areas of Europe, these blood concentrates are made through a series combining thousands of different blood donors. Therefore, the United States only keeps frozen plasma instead. Fibrin sealants work by applying the medication directly to the site of the wound, from there the Fibrin promotes clotting and healing. Clot-preserving medications can also be applied directly to the wound. However, this time the medication preserves the clot from breaking down and assists in ending the
Iron Deficiency Anemia affects millions of individuals across the world. This disease strikes many more women than men and has harmful effects on all who suffer from this deficiency that causes oxygen-carrying capacity to decrease. The causes can vary amongst different groups, but the aggravating symptoms remain constant. Much of the research on Iron Deficiency Anemia concentrates on not only the treatment of this disease, but also the prevention of it. To attain a better understanding of how to treat this problem, one must clearly know what Iron Deficiency Anemia means, what causes this disease, the effects of it, and finally how to cure it.
Hemochromatosis is a genetic disease in which there is too much iron that builds up in your body, this is referred to as an iron overload. Iron is an essential nutrient found in many foods but can be toxic to our bodies if we have to much. “Normally, humans absorb about 8-10% of the iron found in foods that they eat.” People with Hemochromatosis can absorb up to four times more iron than a normal human being. Since our bodies have no natural way to get rid of the extra iron, it gets stored in your body tissue including the liver, heart, pancreas and many other areas of our body can also be infected by this iron overload.
People may not know they have hemochromatosis since in the early stages they may not have any symptoms. In this stage, the only way the disorder can be detected is through routine blood tests and the doctor noticing elevated iron levels in the blood. Usually, the first and most common symptoms of hemochromatosis is joint pain and fatigue, but these are symptoms which occur in several other diseases and disorders, so the diagnosis is frequently held up or even overlooked. “Pain in the knuckles of the pointer and middle finger, collectively called ‘The Iron Fist,’ is the only sign or symptom specific to hemochromatosis. However, not everyone with HHC experiences the Iron Fist” (“WHAT IS HEMOCHROMATOSIS?” n.d.).
Sharon Moalem discusses is hemochromatosis. Hemochromatosis is a disorder that disrupts iron metabolization, by absorbing excess iron instead of passing it through the body, causing iron to build up in the organs, especially the liver, heart and pancreas. The symptoms are joint pain, fatigue, and weakness. It was first described by Armand Trousseau in 1865, but is thought to have originated with the Vikings and spread throughout Northern and Western Europe. The genetic variant for hemochromatosis is very common among people of Western European descent, but only one in two hundred of those people have the disease with all of the assorted symptoms. Hemochromatosis can be diagnosed through blood and genetic tests, and it can be treated through the ancient practice of phlebotomy, or bloodletting. This lowers the amount of iron in the blood, and in the body, to safe levels. But although it is easily treatable, if left unchecked, hemochromatosis can lead to serious complications. As Dr. Moalem says, “Unchecked, hemochromatosis can lead to liver failure, heart failure, diabetes, arthritis, infertility, psychiatric disorders, and even cancer. Unchecked, hemochromatosis will lead to death.” Thus, before the days of genetic and blood testing, hemochromatosis was a very dangerous disease, and often caused death in middle age. It is here that Dr. Moalem raises the question: if hemochromatosis was so deadly, why was it passed down for so many generations? Unless it provided our ancestors with an advantage, such a damaging disease would not have remained in their gene
Hemochromatosis, also called iron overload, is a disease when the body absorbs too much iron from the food we eat and most often affects the liver, heart, pancreas, and skin. It is usually caused by genes in the body that mutate keeping them from working. Some signs and symptoms of this disease are weakness, joint pain, low libido, and/or if the disease has progressed to far diabetes or heart failure. Hemochromatosis is one of the more difficult diseases to diagnose because many symptoms are nonspecific and usually many people don’t show as many symptoms except elevated iron levels in their blood. Most will be treated with blood transfusions until the normal level of iron is reached. If diagnosed early the prognosis is excellent with possible checkups or phlebotomies.
Take hemochromatosis, a hereditary condition that causes iron to accumulate in a person's body. A person having hemochromatosis
Some blood disorders can be prevented while there are others that are out of a person’s hands and have to live with a blood disorder for a life time. It is essential to know the causes of hereditary disease and know how to treat them. It is also important to know what can be done to “cure” other blood disorders and what preventive measures need to be taken in order to stop history from repeating itself. Iron deficiency anemia, sickle cell anemia, and purpura simplex are just a few blood disorders that people suffer from that are either inherited or can be prevented.
Hemochromatosis is a deadly disease in which the body believes that it never has enough iron. The body, as a result is that iron is not filtered out through the intestines, it is always entering the body. This iron runs out of places to be stored, and is spread throughout the body. These iron stores eventually end up changing the body and causing damage to major organs and joints. Hemochromatosis can lead to cancer, heart failure, and a plethora of other problems.
Iron is one of the important minerals that is required for our bodies to function properly. Most of the iron in our body is found in the blood such as haemoglobin, approximately 60 -70% of the human body’s iron is found in the haemoglobin, a protein in the blood that transports oxygen. Iron is also present in muscle tissue and some enzymes. There are two types of iron in the body which are “Heme Iron” from animal products and “Non-Heme Iron” vegetables and
A doctorate holder in human physiology, neurogenetics and evolutionary medicine, Dr.Sharon Moalem makes connections between modern day diseases and how they were necessary for the survival of our ancestors. In his book Survival of the Sickest, Moalem makes many claims, but they are all hypotheses. One of Moalem’s firsts claims is that hemochromatosis, a fairly rare disease today unless one is of Western European descent, is a disease that can cause many complications within the human body due to an iron excess. Moalem claims that hemochromatosis is still in the gene pool and is very common to people of European descent because of the bubonic plague. The plague, a very fatal disease that reared its head in Europe during the 14th century is
Because of this, the body absorbs iron nonstop. As a result, the body will have extremely high levels of iron, leading to fatal outcomes over time. Such consequences include “damaging the joints, the major organs, and overall body chemistry” (Moalem, p. 2). When this disease was described in 1865, it was thought that this disease was extremely rare. But it was soon discovered that this particular disease, soon called hemochromatosis, was caused by a certain gene. In the book, it states “If your ancestors are Western European, the odds are about one in three, or one in four, that you carry at least one copy of the hemochromatosis gene” (Moalem, p. 3). In other words, this is the most common hereditary disease of those of Western European descent. This brings up another question. Why would a disorder be so common in the genetic pool of the Western Europeans? First, it needs to be understood that harmful bacteria feed on iron of the macrophages to thrive. However, people with hemochromatosis have a lack of iron in their macrophages, even though it may seem like the complete opposite. Therefore, those who have hemochromatosis are less prone to infectious agents. For this reason, people with hemochromatosis were less prone to diseases like the bubonic plague and tuberculosis. During the Black Death, for people who had hemochromatosis, “though it would kill them decades later, they are much more likely. . .to survive the plague, reproduce, and pass the mutation to their children” (Moelem, p. 15). Since the mutation helped humans survive an outbreak of the bubonic break in the fourteenth century, the mutation became more common in the gene pool. To treat this genetic disorder, bloodletting is the treatment of choice since it reduces the iron level in their body. I find it interesting that bloodletting, an ancient
Hemochromatosis is when there is excess iron in the body. It is a genetic disorder that is passed down each generation and is inherited by the offspring. Iron overload directly affects the circulatory system but eventually the complications can affect the whole body and many major organs. In addition, hemochromatosis can show no symptoms but the body sometimes shows
A common use is for minor wounds, which are too small to mend by other standard surgical methods. One method to deal with bleeding is to use fibrin glue on the site of the wound. The glue contains both fibrinogen and thrombin and once applied to the site of the injury forms fibrin monomers and eventually a fibrin clot. Alternatively, administration of a hemostatic carrier with thrombin so topical treatment is not necessary if not desired. Medical professionals also use pure thrombin applied in a mist after dissolving it in a saline solution. This final method is typically only for wounds that cover a large
In the test we found that Julia’s mother had a genetic condition called Hereditary Hemochromatosis this means Julia has a likely chance of being a carrier. Hereditary Hemochromatosis is a disorder that causes the body to absorb excessive amounts of iron thus damaging important organs. Hemochromatosis is inherited in an autosomal recessive manner meaning two copies of an abnormal gene must be present in order for the disease or trait to develop. It is possible that Jack may be a carrier as well, as his parents or grandparents could have been carriers so in this case we found need to investigate your family background and history.
Occasionally a baby is born with this disorder and no family history of it. When this happens, it could be caused by a hidden gene, which is when several generations of female carry it, and it has not affected any male members of the family or a spontaneous mutation. With each pregnancy, a woman who is a carrier has a 25% possibility of having a son born with hemophilia. Since the father's X chromosome is what determines if the unborn child will be a girl, all of the daughters born of a man with hemophilia will be carriers. None of his sons, which is determined by the father through his Y chromosome, will have hemophilia. Individuals who suffer from mild hemophilia may choose to use a non-blood product known as Desmopressin acetate (DDAVP) to help treat the small bleeds and/or scrapes. For deep cuts or internal bleeding, the treatment called DDAVP may not be enough and therefore, may need a much more complex treatment. The clotting factor must be replenished so the affected person can form a clot to stop the bleeding. Plasma is one of the ‘human blood products’ than is used for factor replacement. Another factor replacement option is using the recombinant factor, which is produced in a laboratory.