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Lipid Profile in Children with
Beta – Thalassemia Major in Babylon
Dakhel Ghani Omran
College of science for Women, Department of Biology, University of Babylon
Abstract
The aim of the present study was to investigate the lipid pattern in children with beta
thalassemia major in Babylon province. Forty transfusion dependent beta – thalassemia major patient,
20 males and 20 Females (mean age 9±5 years) that undergo periodical blood transfusion and
desferoxame as a chelating agent, were studied. Twenty controls of matched age and gender were also
included in this study. Beta thalassemia major patients showed significantly lower total cholesterol,
high density lipoprotein cholesterol, and low density lipoprotein cholesterol when compared with
control. Serum triglycerides levels of beta–thalassemia major patients were found to be significantly
higher than levels in healthy children . Results mentioned above revealed that lipid profile changed in
patients with beta–thelassemia major. Many factors such as iron over load, liver injury, hormonal
disturbances and aging might cause these changes .
‫الخالصة‬
‫صممتتهذهمملدذاسة ابم ذسمتممرذاستلحام اهذاستاصم ذ م ذت ممااا ذاسمةه ذونذذ م ذافالممامذاستصممارانذمتم مذاسمنبممعتعاذتممنذ ممو ذرالمماذ‬
‫ذا مم) ذاستصمارانذراملاذاستم مذتامرذلتمهذ‬20‫ذل م اذوذ‬20 ‫ذالمنذتمنذاف مارذواسمل و ذ‬40‫ذشت هذهملدذاسة ابم ذ‬.‫اسكرا ةذ ذتتا ظ ذمارلذ‬
‫ذ ام ذ‬
‫ذو ملسمذلم ذ تم‬.‫ ذ ممن ذبمات ذس تةاممة‬Desferal ‫ت ماستلا ذ م ذت مثذاسمنبممعتعاذم اما ه ذاسممة ذمصمو ةذةو مم ذتماذ ممن ذاسممذ‬
‫ذ ذ‬.‫ذب ذ‬14-4‫ذالنذب عتاذتنذ نذاست بانذ تتتو ذبعا ةذذل اوتهذا تا ذافالامذرانذ‬20‫ت و ذتنذ‬
‫ ذ ذت ااا ذاسكوسبل ومذاسك ذواسر ولا ماهذاسةه عم ذ اسعم ذاسكما م ذ‬P< 0.05 ‫ذرا هذ لائجذهلاذاسمترذا خلاضاذت وعاذمتبلوىذ‬
‫ذ ذ‬.‫وذق ا ذاسكما ذتقا ذمافالامذافصتا ذ‬
‫ ذ ذافالامذاستصمارانذماستقا م ذتماذتتتو م ذاسبمعا ةذ‬P< 0.05 ‫ذسوتظذا للا اذت وعاذ ذقع ذاسك ذوسبل ومذاسمنم ذمتبلوىذ‬
‫ذ ذ‬.
‫ذوانذاس واتمملذاسلم ذلبممر ذ‬.‫ذانذاس لممائجذاستبلخ صم ذتممنذهمملدذاسة ابم ذلرممانذانذاسممةهونذللم م ذ م ذافالممامذاستصممارانذماسمنبممعتعا‬
‫ذ ذ‬.‫هلدذاسلحا اهذمصو ةذ ئعب ذت ااذثماةةذل اثذاستةاةذولتا ذ باجذاسكرةذواضا ا ذاسا تو اهذواسلقة ذماس ت ذ‬
Introduction
Thalassemia are the most common heterogeneous group of genetic disorders in
which the production of normal hemoglobin (Hb) is partly or completely suppressed
because of defective synthesis of one or more globin chains, that very widely in
severity from asymptomatic forms to sever or even fatal entities (Crisaru, et al., 1990;
Melody, et al., 2004). Patients with beta thalassemia major may go through several
complications as the transfusion– related infection like HBV and HIV (Chen et al.,
2006). Iron overload complication are also noticed that includes endocrinopathies,
heart and liver diseases, and chelation therapy complications (Ferrara et al . , 2004) .
Thalassemic patients are also subjected to peroxidative tissue injury. It has
been documented that circulating low density lipoprotein–C (LDL–C in thalassemic
patients show marked oxidative leading to pathogenesis . Free radical production is
increased in patients with iron over load. Iron–over loaded patients have elevated
plasma levels of thiobarbituric acid reactants and increased hepatic levels of aldehyde
protein adducts , indicating lipid peroxideation (Canata, et al., 2001) .
During the past years many scientific evidences have raised the advers effect
of abnormal blood lipid levels, like total cholesterol and other lipids and lipoproteins,
on atherosclerotic disease (Ginsberg 1994).
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Journal of Babylon University/Pure and Applied Sciences/ No.(5)/ Vol.(18): 2010
At this point it should be mentioned that the relation ships between blood
lipids and atherosclerosis might be influenced by several other life style–related
factors, like glucose intolerance, blood pressure levels dietary habits and smoking
habits (William and Waikan , 2001) .
Selimoglu, et al , (2002) suggested that both serum iron and triglycerides are
involved in the pathogenesis of LDL–C oxidation. The present study examined the
lipid profile of thalassemic patients in Babylon province .
Materials and Methods
-Patients:The study population included 40 patients with beta thalassemia major (20
females and 20 males) that undergo periodical blood transfusion and desferoxame as
over four month in thalassemia center in Babylon. The tested group aged from 4-14
years . The diagnoses of beta-thalassemia major were made based on the clinical,
hematological and hemoglobin electrophoresis profiles which is indicative sign of the
presence of beta-thalassemia major. Twenty controls (10 Males and 10 females) of
matched age and gender were also included in this study. All patients were transfusion
dependent at a rate one to tow times monthly. Also , all of them are treated with
desaferal .
-Blood Collection:Blood samples were drawn from the thalassemic patients (whom are free from
HBV , HCV and HIV) in plain tubes and allowed to clot and centrifuged to obtain
serum .
Methods
Serum lipid profile was determined after 12-14 h of fasting including : total
cholesterol (TC), triglycerides (TG) , high density lipoprotein cholesterol (HDL-C)
and low density lipoprotein cholesterol (LDL-C), and these parameters were evaluated
by using analytical kits from Biomagreb company .
Statistical analysis
All quantitative data were expressed as means S.D. student two sample t-test
was used to explain the significance and p0.05 was obtained as a lower limit of
significance .
Results
The results of various lipid analyses of controls and thalassemic children are
explained in the table. Beta-thalassemia major patients had significantly (P<0.05)
lower total cholesterol (T-C), high density lipoprotein cholesterol (HDL-C) and low
density lipoprotein (LDL-C) compared with control. However serum triglycerides
level of beta-thalassemia major males and females patients were significantly higher
(P<0.05) than control males and females .
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Table : Serum lipids (triglycerides , total cholesterol , high density lipoprotein
cholesterol , and low density lipoprotein cholesterol) levels of males and
females children affected with beta-thalasseuia major .
Parameter
Patients
Control
Male
Male
Female
Signficancy
Female
92±18
84±80
Triglycerides (g /dl) 148±37
P> 0.05
136±41
109±22
179±31 168±45
Cholesterol (g/dl)
P> 0.05
124±32
34±13
53±5
49±7
HDL – C (g/dL)
P> 0.05
38±14
58±23
136±38
100±7
LDL-C (g/dl)
P> 0.05
62±28
-Values are means + SD
Discussion
The present study investigated the distribution of some blood lipids in the
blood of Babylon children with beta-thalassemia major, it was found that the majority
of the participants had low total cholesterol levels HDL-C and LDL-C levels in
addition triglycerides levels were substantially high.
Papanastasiou, et al. (1996) have shown that total cholesterol , HDL and LDLcholesterol was significantly decreased , while triglycerides were significantly
increased in the thalassemic patients compared to the control subjects . They also
found a positive correlation between age and triglycerides levels.
The present study also observed similar results regarding blood lipids levels
among patients and healthy controls from general population. Other study on beta
thalassemia major in patients from Italy found that these patients disclosed
significantly lower total cholesterol, LDL cholesterol, HDL-cholesterol and higher
triglycerides concentration (Papanastasiou et al., 1996).
It appears, therefore, that many factors such as iron over load (high ferritin
level), liver injury (disturbances of ratio between AST and ALT) and hormonal
disturbances affects lipid pattern among patients with major form of beta thalassemia .
Some authors suggested that accelerated erythropoiesis and increased uptake
of LDL by macrophages and histocytes of the reticuloendothelial system are the main
determinants of low plasma cholesterol levels in beta thalassemia (Maioli, et al.,
1989; Maioli, et al., 1997). In addition, total serum phospholipids their fractions and
cholesterol were significantly lower among patients with thalassemia major (Giardini,
et al., 2004). These changes were referred to hepatic damage and iron over load
furthermore, some serum lipid polyunsaturated fatty acids were significantly
decreased among patients with beta thalassemia major as compared to normal
controls. Since these alterations are a sign of lipid peroxidation, the causes of this
phenomenon were studied . These differences on blood lipids and lipoprotein levels
could also attribute to the adherence of a heal their lifestyle by people with beta
thalassemia, which could include consumption of healthy food since childhood .
However, other report (Maioli, et al., 1984) showed that adolescents with beta
thalassemia minor have significantly lower cholesterol levels than patients with beta
thalassemia major. The investigator suggested that this has been related to their
disorder and not influenced by age, gender, hemoglobin or ferritin levels at this point
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Journal of Babylon University/Pure and Applied Sciences/ No.(5)/ Vol.(18): 2010
it should be noted that the extrapolation of these findings into other populations with
beta thalassemia major might be under scrutiny since thalassemia is genetically
oriented and various expressions of the related polymorphisms may be involved in the
distribution of blood lipids and lipoprotein levels .
Increased susceptibility of red-blood cells lipids to autooxidation in
thalassemia major has been documented (Canatan, et al., 2001; Hartman et al., 2002).
Since autooxidation may be initiated by free radicals which are constantly formed in
the normal red cells, and may be especially prevalent when unstable hemoglobin is
present (Thein, 1992) .
This study shows similar results in lipid profile of thalassemic patients that
were observed by other studiy, where significant decrease in total cholesterol and
HDL-C were detected (Low , 2005) .
Triglycerides lipase activities (both hepatic and extra hepatic) were significantly
lower in thalassamic patients. Christina et al (2004) speculated that the decreased
levels of these enzymatic activities could play a role in determining the decrease of
HDL-cobserved in thalassmic patients.
The present findings are in agreement with those found by other studies
(Pongana and Pongana, 2002; Flavio, 2005) as there is significant increase in plasma
triglycerides. However other researchers did not find such differences (Maioli, et al.,
1999) they also noted an increase in triglycerides with increasing ferritin values and
positive correlation of the patients triglycerides with both age and oxidative LDL-C
antibodies (Kattwasser and Gottschalk , 1999) .
A decrease in the amount of stored iron only known cause for a low serum
ferritin result (Bishop et al., 2000) ; while increased stored iron (over load) associated
with raised serum ferritin levels (e.g. massive blood transfusion). Also liver disease
or inflammation with result in high serum ferritin (Barton , 2007) .
Because of the regular blood trans fusions , approximately all beta thalasemic
patients had abnormally very high levels of serum ferritin, indicating that these
patients have iron over load, probably due to multiple blood transfusion, increased
dietary iron absorption or in inadequate chelating therapy with desferral (Williamy
and Waikn, 2001). In this study triglycerides levels could be correlated positively with
the serum feiritin levels these result might support the hypothesis that both serum iron
and triglycerides are involved in the pathogenesis of LDL-C oxidation (Selimoglu, et
al. , 2002). The present data showed that LDL-C was significantly decreases in its
values and it is in agreement with those found by other workers (Giardini, et al.,
2004) .
Moreover Maioli, et al., (1997) showed that LDL-C was influenced by both
diagnosis and ferritin levels . In the present study we elicited a significant positive
correlation between ferritin and triglycerides a marker of disturbed lipid profile with
abnormal iron over load and inadequate chelating therapy with desferal. From this
study , we conclude that a higher erythroid bone marrow activity with the enhanced
cholesterol consumption be the dominant mechanism implicated in the lipid
abnormalities of thalassemia major patients .
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