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العنوان
Myocardial disease in Egyptian
Beta Thalassemia patients /
المؤلف
Abd Elhameed,Abd Elhameed Elsayed
هيئة الاعداد
باحث / Abd Elhameed Elsayed Abd Elhameed
مشرف / Azaa Abd El Gawad Tantawy
مشرف / Neven Mamdoh Habeeb
مناقش / Nayera Hazaa Khalil El Sherif
تاريخ النشر
2016.
عدد الصفحات
177p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
أمراض النساء والتوليد
تاريخ الإجازة
1/1/2016
مكان الإجازة
جامعة عين شمس - كلية التمريض - Paediatrics
الفهرس
Only 14 pages are availabe for public view

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from 16

Abstract

Summary
The thalassaemias are common genetic disorders
worldwide and they constitute a major problem for patients,
health providers and society. Beta thalassemia patients
usually require regular blood transfusions to survive,
however the chronic transfusion, combined with
extravascular haemolysis and increased intestinal
absorption of iron, leads to significant haemosiderosis of all
organs, including the heart.
Cardiac complications such as heart failure and
arrhythmias are the major causes of death in Beta
Thalassemia patients. Iron cardiomyopathy is reversible, if
chelation starts in time. Once heart failure develops, the
prognosis is frequently poor.The new parameters of cardiac
function, derived from two-dimensional speckle-tracking
echocardiography could be useful for an early diagnosis of
cardiac involvement in patients with Thalassemia major.
Currently cardiac MRI has been known as a noninvasive
technique of choice for monitoring iron overload
in the heart , also identifies patients at high risk of heart
failure and arrhythmia from myocardial siderosis in
thalassemia major and is superior to serum ferritin and liver
iron
That study aim to detect early myocardial disease in
beta thalassemia and to assess the effect of iron load on the
 Summary
-117-
myocardium by speckle tracking echocardiography
correlating with the efficacy of iron chelation and other risk
factors.
This cross sectional study included 30 transfusion
dependant β-thalassemia patients aged between 11-20 years
with mean 14.77 ±2.45 years a male to female ratio 1.3:1
recruited from the Pediatric Hematology Unit, Children
Hospital, Ain Shams University.
All included patients were subjected to detailed
medical history{including transfusion, chelation, hepatitis
C virus history with calculation of mean serum ferritin in
last 2years}, clinical examination, laboratory investigations
including CBC, liver function tests, markers of hemolysis
(total bilirubin, indirect bilirubin), Radiological
investigation included Echocardiography (Tissue Doppler
and Speckle Tracking),MRI T2*.
Patients were divided into two groups according to
mean serum ferritin in the last 2 years: group 1: included
patients with mean serum ferritin less than 2500ng/dl in last
2 years prior evaluation. group 2: included patients with
mean serum ferritin more than or equal 2500 ng/dl in last 2
years prior evaluation.
There were no significant differences between both
groups as regard age & sex distribution, age at diagnosis,
duration of the disease,chelation history ,compliance to it
and splenectomy . However patients with mean serum
 Summary
-118-
ferritin > 2500 ng/mL in last 2 years prior evaluation
showed significantly higher percentage of patients with
positive family history and significantly shorter interval
between transfusion.
There was a significantly positive correlation between
mean s.ferritin in the last 2 years and the disease duration
and a significantly negative correlation between mean s
ferritin and interval between transfusion.
There were no significant differences between both
groups as regard laboratory data although patients with
mean serum ferritin > 2500 ng/mL in last 2 years prior
evaluation showed significantly higher level of liver
transaminases(ALT).
There were no significant differences between both
groups as regard M mode echocardiographic parameters.
Patients with mean serum ferritin > 2500 ng/mL in last
2 years prior evaluation showed significantly lower
longitudinal strain by speckled echo and there was a
significantly negative correlation between them .
There was no significant difference between both
groups as regard percentage of patients with cardiac
affection by speakled and those with left ventricular
dysfunction.
Patients with mean serum ferritin > 2500 ng/mL in last
2 years prior evaluation showed lower cardiac T2* and
 Summary
-119-
higher liver iron concentration yet results did not reach
statistical significance.
There were no significant differences between both
splenectomized and non splenectomized patients nor
between patients on mono and those on combined chelation
therapy as regard echocardiographic data (M
mode,speckle),affection by speckled and cardiac MRI T2*.
There were no significant differences between both
male and female patients as regard echocardiographic data
(M mode,speckle),affection by speckled and cardiac MRI
T2*.
There were no significant differences between patients
with cardiac affection by speckled and those without as
regard M mode echocardiographic data /