Ludovico Guarini MD Pediatric Hematology/Oncology Fellowship: Columbia University Chief of Pediatric Hematology at Maimonides Medical Center
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Male 1: Sometimes we give transfusions to kids with various reasons thalassemia, a disease about the trait and you accumulate iron at a certain point you have to take excess iron out of the body or is going to do some damages. Is that true? Male 2: That is true. The patient’s two or three population of the patient’s will require as part of agenda and management. Transfusion is over the long period time and prevention for life. The typical example is patient’s so called thalassemia major which is a disease that is affecting people who come from major CBM new released on the way into the fax study in the act and further at least need to sometimes they leave to China. These patients in order to maintain a good level of hemoglobin and not suffering from their disease will require to be transfused generally every three to four weeks. Iron which is an important component of our body is strange in one way to be an antibody, meaning our body is very eager to take it in because it is living, yet has essentially no way to get rid of it. The only way we know we can get rid of the blood is to bleed and so patients who receive transfusion essentially end up with a lot iron than is suits in your body. This iron is actually is very toxic. Male 1: So, in other words doing something good and you get a little problem. Now, how do you handle this excess iron.? Male 2: The problem with accumulating iron is handling by essentially using molecules that are able to bind and is to capture the iron in the body and because of this structure are also able to bend the secreted heme and have to. The ability to either go into the urine or the stool of the patient, so that the iron can actually be. Male 1: So, you do some chelation. Is that true? Male 2: Correct. The name of our diet is chelation. Male 1: Until recently there was a thing called EDTA, it was yours. It is not easy to get. Could you explain how you would give EdTA if you are going to use it. Male 2: EDTA is actually a molecule that will not be used by the rain man in iron over load, but is used and has been used for the management of the man. Voice overlap. Male 1: That poisoning is strictly off. Male 2: And EDTA is something that requires a hospitalization that requires been given to an intravenous line over the time. Rules that have to watch patients with screening carefully because knowledge of the EDTA is not aids very specific for iron. Male 1: So, basic tips of almost every heavy metal, we hope we do not take too much of something you need. Male 2: Correct. Male 1: So, you get to watch the electrolytes. Male 2: Right. It goes essentially first things like calcium which a chemical composition of calcium of such strength is similar to iron may and not be taking out by this molecule in excess and by the same thing to derive a number of other metals in the body that are present in small amounts. Male 1: Like mercury and what iron? Male 2: A number of others have some of that actually at function. Male 1: Is that so? You just cannot go to the doctor’s office, get an IV and walk out and say this is chelation. Is that true? Male 2: Correct and also as anything else in Medicine, but particularly this situation where you are dealing with this molecules to that, a very small amount, but they are very important and to give chelation to somebody who does not need it. The risk some very significant side effects and significant problems. People have eventually done blind, and deaf. They may have some immediate problems. Some of them eventually immediate life threatening then affect the minerals. Male 1: It can cause renal to death necrosis. It is associated with hypotension and convulsions. The rigor 5:05 because of the calcium and other things that takes out of the body. Severe allergic reactions and here is the worst part you might survive going into kidney failure the rest of your life. Maybe even the trans benefit if the kidneys fail. This is not of benign therapy.