الفهرس 
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Abstract
The thalassemia syndromes are hemoglobin disorders that result
from significantly reduced or absent synthesis of the α- or β-globin
chains. The result is a chronic hemolytic anemia with ineffective
erythropoiesis and bone marrow overstimulation. The combination of
transfusion and chelation therapy has dramatically extended the life
expectancy of thalassemic patients who can now survive into their fourth
and fifth decades of life. Nevertheless, frequent blood transfusion in turn
can result in iron overload which may lead to various complications,
including various endocrine complications such as thyroid dysfunction.
Leptin is a polypeptide hormone which is the product of the obese
gene (ob) that is mainly expressed in bone marrow and adipocytes. It also
has been suggested that leptin affects hematopoietic stem cells and plays
a role in erythropoiesis.The leptin receptor is a member of the
hematopoietin receptor family, expressed in yolk sac, fetal liver, bone
marrow, and hematopoetic stem cells. Leptin and erythropoietin can
synergistically increase erythroid development.
Considering the abnormalities in serum ferritin level in thalassemic
patients and the effects of leptin on erythropoiesis and sexual maturation,
we aimed in this study to investigate the possible difference in serum
leptin level between beta thalassemic major patients and healthy controls
and its possible correlation with serum ferritin level.
Studied populations were divided to groups :
Patients : Consisted of 52 beta thalassemia major cases attended
the Hematology clinic for routine scheduled blood transfusion.
Their diagnosis were based on hematological parameters and
hemoglobin electrophoresis obtained from their records, they were 25 males and 27 females.They were diagnosed to have β-
thalassemia major documented by clinical picture and heamoglobin
electrophoresis, receiving multiple blood transfusion, with high
serum ferritin level, receiving one or more methods of iron
chelation therapy, and above 10 years of age. Patients with any
other hemoglobinopathy or hematological disorder and patients
showing any sign of inflammation were excluded from the study..
Controls : Consisted of 35 clinically healthy children,
socioeconomic, age and sex matched recruited from the outpatients
clinic of Pediatric department, Menoufia University Hospital. They
were 14 males and 21 females. Their age ranged from (10-13)
years.
All Patients were subjected to the following:
Patients were assessed by detailed history taking, thorough clinical
examination and anthropometric measurements (weight, height and
BMI). Laboratory investigations were done including complete blood
picture, serum blood sugar, thyroid function and serum leptin for both
groups. Serum ferritin was assessed for thalassemia group of patients.
The mean age of the thalassemia group patients was 12.78±2.36
SD with age range 10-18 years. They included 25 males and 27 females
with a male to female ratio 1:1.08.
It was found that:
In the present study, regarding growth parameters including BMI is
significantly lower in thalassemia group of patients than in control groups
(p< 0.05). No significant difference regarding age and sex in participants
groups.
frequency every (14–44 days), 35 patients (67.3%) of studied
thalassemic group done splenectomy operation, 44 patients took
desferroxamine (84.6%) either alone or in combination with other
therapies, 29 patients took deferasirox (55.8%) either alone or in
combination with other therapies , while deferiprone was taken by 8
patients (15.4%) either alone or in combination with other therapies.
In the present study, CBC parameters showed that there was severe
anemia in thalassemic patients, Hb%, RBCs count, platelets, HCT,
showed a highly significant lower values in thalassemia group than in
control group (P< 0.001 for all). SBG was significantly higher in
thalassemia group than in control group (p < 0.05).
Serum leptin level was lower in thalassemia group of patients than
in control group of patients with a highly significant difference ( p <
0.001). There was a significant difference between male and female
gender in the thalassemia group of patients regarding serum leptin level
(p = 0.009) being higher in the female group.
In the current study, there was a positive correlation between all
growth parameters and serum leptin level in thalassemia group of patients
but with no significance regarding weight and height (p= 0.59 and p =0.
52 respectively) and with significance regarding BMI (p =0.04).
The current study showed a significant negative correlation
between serum ferritin level and serum leptin level in thalassemia group
of patients (p = 0.009). There was a non significant positive correlation
between serum leptin level and T3 and a non significant negative
correlation between TSH and serum leptin level (p >0.05 for both). On
the other hand, there was a significant positive correlation between T4
and serum leptin level ( p = 0.03).
The current study showed that serum leptin as a marker for beta
thalassemia had a sensitivity of 91% , a specificity of 94% , a diagnostic
accuracy of 92%, a positive predictive value of 96%, and a negative
predictive value of 86% with a highly significant value for serum leptin
as a marker ( p = 0.001).