الفهرس 
Acknowledgement
Assessment of Iron OverloadOnly 14 pages are availabe for public view
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Abstract
Iron overload is a common clinical problem, arising from disorders of increased iron absorption such as hereditary haemochromatosis or thalassaemia intermedia syndromes or as a consequence of chronic blood transfusions for various blood disorders. Each unit of RBC transfused contains 180 to 200 mg of iron. chronic packed RBC transfusion therapy increases liver iron by approximately 1 mg/mL (by dry weight) for every 15 mL/kg delivered. Excess iron may accumulate in liver,spleen ,Bonemarrow and in other organs that don’t normally store iron, such as the pancreas, heart, joints and skin. With severe iron overload, it can cause gray-colored or bronze-colored skin, shortness of breath, arthritis, liver disease, including cirrhosis or liver cancer, enlarged spleen that may cause abdominal pain or difficulty in eating normal-sized meal,diabetes, shrunken testicles, heart problems, including both heart failure and heart rhythm problems.
Epidemiologic studies estimate the incidence of iron overload as documented by a liver iron concentration of 15 mg/g dry weight or greater amongst 342 patients with transfusion-dependent thalassemia in the National Institutes of Health (NIH) registry was 23% .
In a Japanese cohort of transfusion-dependent patients about one third of all deaths were attributable to iron overload (97% of the deceased had a serum ferritin >1000 ng/mL). Cardiac failure was responsible for 24% and liver failure for 7% of all deaths.
There are various different methods for evaluating the degree of iron overload, including serum ferritin levels, liver iron concentration determined from a biopsy, superconducting quantum interference device (SQUID) and magnetic resonance imaging (MRI).
MRI is a non-invasive method which can quantify hepatic and cardiac iron, replacing liver biopsy for quantification of iron in the liver.
Chelation therapy should be started in patients with a ferritin over 1000 ug/l. A liver iron level above 15 mg Fe/g dry weight is associated with increased organ injury and high risk of cardiac death in thalassemia.
The overall aim of chelation therapy is to maintain a “safe” iron status at all times, by balancing iron intake from blood transfusion with iron excretion by chelation therapy.
Currently, three main iron chelators are available for clinical use: deferoxamine (DFO), deferiprone (DFP), and deferasirox (DFX).Deferoxamine (Desferal®; DFO) was developed more than 40 years ago and it is effective at lowering serum ferritin levels and hepatic iron and in preventing endocrine complications. Long-term therapy with DFO is also associated with a reduction in cardiac complications .
The target dose of deferoxamine should be 20 -40 mg/kg/day for children, and up to 50 mg/kg/day for adults, given over 8 -12 hours for 5- 7 days/week. Doses of DFO higher than 60 mg/kg/day as a continuous intravenous infusion can reverse cardiac iron burden as measured by cardiac T2*. The greatest challenge with DFO is patients’ adherence to therapy.
Deferiprone (Ferriprox®; DFP), was approved for the treatment of iron overload due to blood transfusions in patients with thalassaemia, who had an inadequate response to prior chelation.DFP is a small lipophilic molecule, which binds to iron in a 3:1 ratio and can enter myocytes and capture LPI in specific organelles of cardiomyocytes and macrophages. The target dose of DFP is 75mg/kg/day.
Deferasirox (DFX; Exjade) is the most recent oral iron chelator. DFX is currently approved in many countries worldwide for the treatment of chronic iron overload due to blood transfusions in patients 2 years of age and older.Iron chelation with DFX may be beneficial because of its once-daily formulation, supported by its plasma half-life of 11 to 19 hours.There are now data on compliance with DFX which, in the Evaluation of Patients’ Iron Chelation with Exjade (EPIC) study, was reported to be greater than 80%.
The efficacy of DFX at a dose of 20–30 mg/kg/day was shown to be similar to that of DFO in reducing liver iron concentration and serum ferritin levels.
The aim of this Descreptive study is to detect indication of iron chelation therapy and its type according to the patient condition,also detect effect of iron chelation therapy on serum ferritin level, Montoring the patient for signs of toxicity for early intervention as (liver toxicity-renal toxicity-blood picture) and to detect the patient compliance to the treatment.
The target populations of this Descreptive study were children diagnosed as had chronic anemias that need chronic blood transfusion and were admitted at AUCH during the period from December 2016 to December 2017.
Data of the study showed the following:
1.Data about the age, sex and admission unit ,were recorded in 100% of cases.
2.The study included 100 patients. 54 cases were male and 46 cases were female.43 cases were between the age of three year and six year, 41 cases between the age of seven year and eleven years and 16 cases more than eleven years old;with Mean±SD of age was 8.4±3.94yr.
3.Among 100 patients (91% with Thalassemia Major,7% with Sickle cell anemia and 2%with Applastic anemia).
4.variable types of iron chelator agents was used for the studied children were Deferoxamine (DFO) 1%, Deferiprone (DFP) 20%, Deferasirox (DFX)72%, as monotherapy were 93% ,combination of DFX & DFP 5% and combination of DFX & DFO2%.
5.Data about iron chelator agents initiation include chronically transfused pediatric patients for at least 10-20 times blood transfusion rates and s.ferritin over 1000ug|L.
6. Data about laboratory investigations show that liver enzymes (ALT) was normal in 93% at the start of study and abnormal in 7%,the same result was after three month, then after six months from iron chelation administration 88% was normal and 12%was abnormal ,and finally after nine months 84%was normal and 16% was abnormal.
7. Data about laboratory investigations show that liver enzymes (AST) was normal in 93% at the start of study and abnormal in 7%,the same result was after three month, then after six months from iron chelation administration 87% was normal and 13%was abnormal ,and finally after nine months 84%was normal and 16% was abnormal.
8.Data about laboratory investigations show that Serum Creatinine was normal in 99% at the start of study and abnormal in 1%, after three month from iron chelation administration 97% was normal and 3% was abnormal, then after six months 98% was normal and 2%was abnormal ,and finally after nine months 99%was normal and 1% was abnormal.