DO PANCREATIC FUNCTIONS PREDICT CARDIAC AND LIVER IRON LOADING IN TRANSFUSION-DEPENDENT BETA THALASSEMIA MAJOR PATIENTS USING CARDIOVASCULAR AND LIVER T2-STAR (T2*)MAGNETIC RESONANCE ?
(Abstract release date: 05/21/15)
EHA Library. Elbarbary N. 06/12/15; 102604; PB2020
Disclosure(s): Ain Shams University,Pediatric Department
Assoc. Prof. Nancy Elbarbary
Contributions
Contributions
Abstract
Abstract: PB2020
Type: Publication Only
Background
In Egypt, b-thalassemia is the most common genetically determined chronic hemolytic anemia. Regular and frequent red blood cell transfusions have significantly increased the life expectancy of patients with b-TM. The transfusions reduce some of the consequences of anemia, such as growth deficit. However, when no appropriate chelation therapy is available, patients accumulate iron in the heart, liver, spleen, pancreas, and endocrine glands, leading to progressive organ dysfunction.
Aims
Methods
A total of 44 children and adolescents β-TM patients and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test, and T2* MRI to assess iron content in the heart and liver .
Results
Overt diabetes was found in 9.4% and 45.5% of patients had impaired fasting glucose. Median cardiac T2* was 22ms (12-31 ms) and LIC was 6ms (4-9 ms). CardiacT2* was less than 10ms in 21.4% indicating heavy load with iron in cardiac tissues. There is a significant decrease in serum amylase (87.5 vs. 63.5 IU/L, p =0.003) and lipase (94 vs.70 IU/L, P=0.056) among enrolled patients in comparison to control group. Thalassemic diabetic showed low serum amylase (32.5 vs. 59.5,p = 0.0005),serum lipase (39.5 vs. 68, p = 0.0007),low cardiac T2* was found ( 7 vs. 22 ms,p =0.0006) and low LIC ( 2 vs. 6ms,p = 0.0006) than other β-TM patients without diabetes. Inverse correlation was found between triglyceride index with cardiac T2*(r= - 0.376, P=0.014 ) and low LIC (r=-0.376, P=0.014 respectively) but not with serum lipase ( r= -0.099,P=0.533),(r= -0.222,p=0.1570) and serum amylase (r =-0.191P=0.225),(r=-0.053 ,P= 0.738 ) respectively . In Egypt, β-thalassemia is the most common genetically determined chronic hemolytic anemia.
Summary
Follow up of thalassemic patients with impaired fasting glucose together with intensive chelation therapy may help to prevent the development of cardiac and hepatic siderosis.
Keyword(s): Beta thalassemia, Clinical data, Iron overload, Therapy
Type: Publication Only
Background
In Egypt, b-thalassemia is the most common genetically determined chronic hemolytic anemia. Regular and frequent red blood cell transfusions have significantly increased the life expectancy of patients with b-TM. The transfusions reduce some of the consequences of anemia, such as growth deficit. However, when no appropriate chelation therapy is available, patients accumulate iron in the heart, liver, spleen, pancreas, and endocrine glands, leading to progressive organ dysfunction.
Aims
To assess the correlation between cardiac and hepatic T2*MRI findings with the endocrine and exocrine pancreatic functions in known β-TM patients.
Methods
A total of 44 children and adolescents β-TM patients and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test, and T2* MRI to assess iron content in the heart and liver .
Results
Overt diabetes was found in 9.4% and 45.5% of patients had impaired fasting glucose. Median cardiac T2* was 22ms (12-31 ms) and LIC was 6ms (4-9 ms). CardiacT2* was less than 10ms in 21.4% indicating heavy load with iron in cardiac tissues. There is a significant decrease in serum amylase (87.5 vs. 63.5 IU/L, p =0.003) and lipase (94 vs.70 IU/L, P=0.056) among enrolled patients in comparison to control group. Thalassemic diabetic showed low serum amylase (32.5 vs. 59.5,p = 0.0005),serum lipase (39.5 vs. 68, p = 0.0007),low cardiac T2* was found ( 7 vs. 22 ms,p =0.0006) and low LIC ( 2 vs. 6ms,p = 0.0006) than other β-TM patients without diabetes. Inverse correlation was found between triglyceride index with cardiac T2*(r= - 0.376, P=0.014 ) and low LIC (r=-0.376, P=0.014 respectively) but not with serum lipase ( r= -0.099,P=0.533),(r= -0.222,p=0.1570) and serum amylase (r =-0.191P=0.225),(r=-0.053 ,P= 0.738 ) respectively . In Egypt, β-thalassemia is the most common genetically determined chronic hemolytic anemia.
Summary
Follow up of thalassemic patients with impaired fasting glucose together with intensive chelation therapy may help to prevent the development of cardiac and hepatic siderosis.
Keyword(s): Beta thalassemia, Clinical data, Iron overload, Therapy
Abstract: PB2020
Type: Publication Only
Background
In Egypt, b-thalassemia is the most common genetically determined chronic hemolytic anemia. Regular and frequent red blood cell transfusions have significantly increased the life expectancy of patients with b-TM. The transfusions reduce some of the consequences of anemia, such as growth deficit. However, when no appropriate chelation therapy is available, patients accumulate iron in the heart, liver, spleen, pancreas, and endocrine glands, leading to progressive organ dysfunction.
Aims
Methods
A total of 44 children and adolescents β-TM patients and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test, and T2* MRI to assess iron content in the heart and liver .
Results
Overt diabetes was found in 9.4% and 45.5% of patients had impaired fasting glucose. Median cardiac T2* was 22ms (12-31 ms) and LIC was 6ms (4-9 ms). CardiacT2* was less than 10ms in 21.4% indicating heavy load with iron in cardiac tissues. There is a significant decrease in serum amylase (87.5 vs. 63.5 IU/L, p =0.003) and lipase (94 vs.70 IU/L, P=0.056) among enrolled patients in comparison to control group. Thalassemic diabetic showed low serum amylase (32.5 vs. 59.5,p = 0.0005),serum lipase (39.5 vs. 68, p = 0.0007),low cardiac T2* was found ( 7 vs. 22 ms,p =0.0006) and low LIC ( 2 vs. 6ms,p = 0.0006) than other β-TM patients without diabetes. Inverse correlation was found between triglyceride index with cardiac T2*(r= - 0.376, P=0.014 ) and low LIC (r=-0.376, P=0.014 respectively) but not with serum lipase ( r= -0.099,P=0.533),(r= -0.222,p=0.1570) and serum amylase (r =-0.191P=0.225),(r=-0.053 ,P= 0.738 ) respectively . In Egypt, β-thalassemia is the most common genetically determined chronic hemolytic anemia.
Summary
Follow up of thalassemic patients with impaired fasting glucose together with intensive chelation therapy may help to prevent the development of cardiac and hepatic siderosis.
Keyword(s): Beta thalassemia, Clinical data, Iron overload, Therapy
Type: Publication Only
Background
In Egypt, b-thalassemia is the most common genetically determined chronic hemolytic anemia. Regular and frequent red blood cell transfusions have significantly increased the life expectancy of patients with b-TM. The transfusions reduce some of the consequences of anemia, such as growth deficit. However, when no appropriate chelation therapy is available, patients accumulate iron in the heart, liver, spleen, pancreas, and endocrine glands, leading to progressive organ dysfunction.
Aims
To assess the correlation between cardiac and hepatic T2*MRI findings with the endocrine and exocrine pancreatic functions in known β-TM patients.
Methods
A total of 44 children and adolescents β-TM patients and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test, and T2* MRI to assess iron content in the heart and liver .
Results
Overt diabetes was found in 9.4% and 45.5% of patients had impaired fasting glucose. Median cardiac T2* was 22ms (12-31 ms) and LIC was 6ms (4-9 ms). CardiacT2* was less than 10ms in 21.4% indicating heavy load with iron in cardiac tissues. There is a significant decrease in serum amylase (87.5 vs. 63.5 IU/L, p =0.003) and lipase (94 vs.70 IU/L, P=0.056) among enrolled patients in comparison to control group. Thalassemic diabetic showed low serum amylase (32.5 vs. 59.5,p = 0.0005),serum lipase (39.5 vs. 68, p = 0.0007),low cardiac T2* was found ( 7 vs. 22 ms,p =0.0006) and low LIC ( 2 vs. 6ms,p = 0.0006) than other β-TM patients without diabetes. Inverse correlation was found between triglyceride index with cardiac T2*(r= - 0.376, P=0.014 ) and low LIC (r=-0.376, P=0.014 respectively) but not with serum lipase ( r= -0.099,P=0.533),(r= -0.222,p=0.1570) and serum amylase (r =-0.191P=0.225),(r=-0.053 ,P= 0.738 ) respectively . In Egypt, β-thalassemia is the most common genetically determined chronic hemolytic anemia.
Summary
Follow up of thalassemic patients with impaired fasting glucose together with intensive chelation therapy may help to prevent the development of cardiac and hepatic siderosis.
Keyword(s): Beta thalassemia, Clinical data, Iron overload, Therapy
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