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Indian Pediatr 2020;57:
1072-1073 |
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Lipid
Profile in Children With Thalassemia: A Prospective
Observational Study From Eastern India
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Tarun Kumar,1
Surupa Basu,2
Ritabrata Kundu,1
Ishita Majumdar1
and Devdeep Mukherjee1*
Departments of 1Pediatric
Medicine and 2Biochemistry, Institute of Child
Health, Kolkata, West Bengal, India.
Email: [email protected]
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This was a
prospective observational study to evaluate
abnormalities in lipid profile in 50 children with
transfusion dependent thalassemia. Dyslipidemia
characterized by high triglycerides, low high
density lipoprotein (HDL), and high total
cholesterol: HDL ratio was noted. These pro
atherogenic risk factors may be lead to significant
cardiovascular morbidity in these patients.
Keywords:
Atherosclerosis, Co-morbidity, E beta thalassemia,
Outcome.
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Life expectancy and quality of life of beta-thalassemia
patients have improved in recent years. However,
non-siderotic complications are known to cause
significant morbidity in these patients with beta-thalassemia.
In recent years, many studies have shown risk of
developing subclinical atherosclerosis in beta-thalassemia
patients. Strong association of abnormal serum lipid
levels [low total cholesterol (TC) and (high density
lipoprotein) HDL-cholesterol, high triglycerides
(TG) and TC: HDL ratio) with premature
atherosclerosis have been noted in children with
beta thalassemia [2-5]. Low HDL - cholesterol and
high TC:HDL ratio are pro-atherogenic factors, which
help in cardiac risk stratification and
prognostication [6,7]. Pediatric data regarding
lipid profile in thalassemia is limited. Our primary
objective was to evaluate abnormalities in lipid
profile in children with thalassemia.
A prospective observational study
was performed at Institute of Child Health, Kolkata
between July, 2016 and June, 2017. Children with
transfusion- dependent thalassemia, under regular
follow up in our thalassemia clinic, were included
for this study. The patients had been diagnosed
following appropriate clinical history, physical
examination, complete blood count and high
performance liquid chromatography (HPLC) and were on
regular transfusion and chelation therapy. Children
having family history of dyslipidemia were excluded.
None of the patients had previous history of
cardiovascular illness. Fifty age- and sex-matched
healthy children were taken as control. Ethical
clearance was obtained from the institution ethics
committee and written consent was obtained from care
givers.
Blood samples for serum fasting
lipid profile and ferritin were taken after a 12
hour overnight fast. Spectrophotometry was used for
assessing fasting lipid profile. Statistical
analyses were carried out using GraphPad Prism,
version 5.0. Continuous, non-parametric data were
compared using the Mann-Whitney U test, while
categorical data were compared by chi square test.
P<0.05 was considered as statistically
significant.
Out of a total of 53 eligible
children, 3 were excluded for having family history
of hyperlipidemia. Thus, 50 children (62% males)
with a median age of 2 years 5 month (range 0-18
years) were enrolled. All patients had low
HDL-Cholesterol, 74% had high TG levels, 84% had a
high TC: HDL ratio and 60% had low total cholesterol
(Table I). Out of the 37 patients with
elevated triglyceride levels, 28 had
hyperferritinemia. Low density lipoprotein (LDL) and
very low density lipoprotein (VLDL) were in normal
range. Patients with thalassemia had lower HDL
cholesterol and higher triglycerides and elevated
TC: HDL ratio compared to the controls (Table
I). Children with E beta thalassemia had lower
TC than children with beta thalassemia major (Table
II). TC: HDL ratio was increased and
HDL-cholesterol was decreased irrespective of age
and gender.
Table I Comparison of Fasting Lipid Profile between Children with Beta Thalassemia Patients and Control Group
| Parameters |
Children with |
Control group |
|
thalassemia (n=50) |
(n=50) |
| Total cholesterol |
95.5 (78.75, 111.3) |
156.5 (143.5, 184.8) |
| HDL-cholesterol |
23 (19, 32) |
48.5 (39.8, 54.3) |
| Triglycerides |
258 (142, 415) |
118 (78, 199.3) |
| *TC:HDL ratio |
4.79 (3.79, 6.94) |
3.00 (2.73, 4.08) |
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All values in median (IQR) except total:high
density lipoprotein cholesterol ratio
(TC:HDL). All P<0.001. |
Table II Comparison of Fasting Lipid Profile between Children with Beta Thalassemia Major and E-beta thalassemia
| Parameters |
b-thalassemia major |
E b-thalassemia |
|
(n=29) |
(n=21) |
| Total cholesterol# |
109 (96,147) |
76 (69, 86.5) |
| HDL cholesterol |
23.5 (19.75, 29.75) |
23 (17.25, 33) |
| Triglycerides^ |
176.5 (137.8, 352.5) |
334.5 (254.3 -457) |
|
*TC:HDL ratio |
4.32 (3.79 – 6.29) |
4.9 (3.86 – 7.13) |
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All values in median (IQR). Units for all
values in mg/dL; *TC: Total cholesterol.
#P<0.0001; ^P=0.02 |
Iron overload and oxidative
stress are postulated mechanisms for causing
dyslipidemia in patients with thalassemia [4,8,9].
Similar to our results, hypocholesterolemia in
patients with thalassemia has been demonstrated in
earlier studies [8]. Iron overload contributes to
liver injury contributing to decrease in production
of cholesterol. Increased consumption of cholesterol
due to enhanced erythropoiesis and increased uptake
by the histiocytes are other factors [9]. Elevated
triglyceride levels, possibly because of decreased
lipolytic enzyme activity, have also been reported
previously [2,4,9,10]. Unlike our study, females
were found to have elevated TG in previous studies.
The low HDL-cholesterol levels in our patients weres
presumably due to excessive clearing of HDL by the
activated macrophages [4].
The limitation of our study was a
small sample size. Larger studies with follow up
echocardiography and cardiac MRI will provide
further insight and information regarding the
cardiovascular complications in children with
thalassemia having deranged lipid profile. This may
also help in framing guidelines for monitoring lipid
profile in these children, in order to reduce long
term morbidity and mortality.
Acknowledgements: Dr
Prabal Niyogi, Deputy Director, Institute of Child
Health was involved in planning of the research.
Ethics clearance:
Institution ethics committee of Institute of Child
Health, Kolkata; No. ICH/13/2016, dated 29 July,
2016.
Contributors: TK, SB: were
involved in processing data and statistical
analysis; TK, DM: were involved in writing the
manuscript which was subsequently approved by all
the authors. All the authors were involved in
planning of the research and collection of data.
Funding; None; Competing interest:
None stated.
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