scholarly journals Study of lipid profile in β thalassemia major pediatric patients with multiple blood transfusion and its correlation with serum ferritin level in tertiary care hospital in Kolkata

Author(s):  
Saikat Nandi ◽  
Sanjana Samanta ◽  
Tanushree Mondal ◽  
Sanjay Halder

Background: β-Thalassemia major is associated with iron overload toxicity. This study was done to investigate lipid profile abnormality in β-Thalassemia major patients and to establish their correlation with serum ferritin. Another motto of this study was to signify deleterious effect of multiple blood transfusion and facilitate to understand how it will affect the lipid profile parameters changes so that we can also prevent early dyslipidemia induced complications like atherogenesis by early detection of lipid profile abnormality and also to establish derangement of lipid profile as an indirect evidence of iron overload.Methods: 102 nos. β-Thalassemia major paediatric patients with multiple blood transfusion (≥10) in age group of 6 to12 years admitted in pediatric ward of tertiary care hospital (NRSMCH) were enrolled. Serum ferritin level and lipid profile were investigated and their correlation were assessed.Results: Among 102 β-Thalassemia major pediatric (6 to 12 years) patients 55.88% were male and 66.67% were Hindu by religion and 69.61% children came from lower socio-economic class. The average value of number of previous blood transfusion was 42.79±7.510. The mean value of serum ferritin was 2263.53±833.904 ng/ml. The average value of serum total cholesterol, LDL and HDL were 153.84±5.428 mg/dl and 97.16±3.982 mg/dl and 29.45±4.445 mg/dl respectively and their correlation with serum ferritin were negative (r=-0.941 and r=-0.964 and r=-0.751 respectively). Average value of serum triglyceride and VLDL were 206.22±67.407 mg/dl and 36.16±3.385 mg/dl respectively and their correlation with serum ferritin were positive (r=+0.606 and r=+0.973 respectively).Conclusions: There was significant lipid profile derangement. As the serum ferritin level increases, serum total cholesterol, HDL, LDL decreases and serum triglyceride, VLDL level increases.

Author(s):  
Naresh Manne ◽  
Bharat Kumar Gupta ◽  
Sandeep Kumar Yadav ◽  
Saurabh Singhal ◽  
Archana Dubey

Background: Beta-Thalassemia is a genetic disorder which is associated with a lot of complications. Frequent blood transfusions result in increased iron deposition in various tissues leading to dysfunction of many vital organs. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients. Methods:  This is a descriptive cross sectional study carried out in 100 diagnosed patients of beta- thalassemia major who had visited the OPD/IPD of Subharti Medical College & affiliated Hospitals, Meerut for routine blood transfusion or for any other complication. Patients were clinically examined and investigated for presence of one or more endocrine disorders on their routine appointments. Results: Endocrine disorders were detected in a total of 82 patients. Diabetes mellitus was detected in 12% patients, hypothyroidism in 36% patients and delayed puberty was found in 72% patients. Mean serum ferritin level was found to be 5831.0±2860.5 ng/ml in beta-thalassemia Major patients, while it was in normal range in control subjects. Conclusion: Research concluded with finding of Delayed puberty (72%), Hypothyroidism (36%) and diabetes mellitus as (12%) in beta thalassemia patients who were on regular blood transfusion therapy.  Iron overload as serum ferritin level was found to be highly raised in all study case. On the basis of our study we recommend that early detection and management protocols for these endocrinopathies may improve the life prospects of beta-thalassemia Major patients. Keywords: Endocrine disorders, Hypothyroidism, Delayed puberty, Diabetes Mellitus Serum ferritin, Thalassemia Major.


2017 ◽  
Vol 4 (2) ◽  
pp. 543 ◽  
Author(s):  
Rameshwar L. Suman ◽  
Anuradha Sanadhya ◽  
Pradeep Meena ◽  
Jaskaran Singh ◽  
Rupali Jain ◽  
...  

Background: β-thalassemia major is a secondary iron load state. The high serum ferritin accounts for abnormal lipid profile. This study was done to investigate the lipid profile in children with β-thalassemia major and to know their correlation with serum ferritin.Methods: Fifty-five (55) children of beta thalassemia major in the age group of 4-20 years from thalassemia unit of tertiary care hospital who were on regular blood transfusion and oral iron chelators were enrolled. Serum ferritin levels and lipid profile of these children were investigated and their correlation was assessed. Results: Out of total 55 children 31 (56.4%) children were of female sex and 48(87.3%) were Hindu by religion. The mean rate of blood transfusion was 157.02±21.33 ml/kg/year with average dose and duration of iron chelation with Desferroxamine was 34.4±26.86 mg/kg /day and 2.34±1.86 years respectively. Mean cholesterol level, high density lipoprotein (HDL), low density lipoprotein (LDL) levels were on lower side of the range with values of 124.47±19.81 mg/dl, 36.58±12.22 mg/dl, 63.94±4.57 mg/dl respectively. The mean triglyceride level (TG) was on higher side with an average of 142.93±33.7 mg/dl. The average serum ferritin levels were 2130.33±859.85 ng/ml. There was negative correlation of total cholesterol, HDL, LDL with serum ferritin with coefficient of correlation (r= -0.77, -0.55, -0.72) respectively. The serum triglyceride had positive correlation with serum ferritin with coefficient of correlation(r=+0.85).Conclusions: As the serum ferritin increases, there occurs more derangement of lipid profile in form of decreasing serum cholesterol, HDL, LDL and increasing serum TG levels.


Author(s):  
Saeed Yousefian ◽  
Ghassem Miri Aliabad ◽  
Rana Saleh ◽  
Majid Khedmati

Background: Beta-thalassemia major is a type of inherited blood disease that results in variable outcomes such as severe anemia due to haemoglobin chains. Recurrent and lifelong blood transfusions as a treatment in beta-thalassemia major disease lead to iron deposition in various organs and cause the failure of multiple organs. Failure of affected organs leads to Body mass index (BMI) abnormality. This study aimed to evaluate the association between BMI and serum ferritin level as a marker for iron overload. Materials and Methods: A cross-sectional study designed and conducted with total number of 740 paediatrics, with mean age about 14.2±8.7 years old and with beta-thalassemia major requiring recurrent blood transfusion. Patient information, including demographics, serum ferritin level and percentage of BMI, was recorded and analysed by SPSS 25.0 and the statistical significant level, considered as 0.05.    Results: A total number of 740 paediatrics with beta-thalassemia major disease (mean age about 14.2±8.7 years) were included to study to examine the association between serum ferritin level and their BMI.  The total mean serum level of ferritin calculated about 3326 ± 3859 Nanogram/mililitter (ng/ml). Totally, 447 (60.4%) case of them had BMI percentile less than 5%, 274 (37.02%), 16 (2.16%) and 3 (0.4%) had BMI percentile 5%-85%, 85%-95% and more than 95%. There was no relation between gender and serum ferritin levels. The relationship between age and BMI has been positive (P=0.002). Finally, it resulted that there was a negative relationship between the BMI percentile and mean serum ferritin levels in paediatrics with beta-thalassemia major (P=0.031). Conclusion: Frequent Blood transfusion is associated with elevated serum ferritin level in paediatrics with beta-thalassemia major disease and experiencing lower percentiles of BMI in these patients.


2008 ◽  
Vol 48 (1) ◽  
pp. 33
Author(s):  
Sri Hastuti Andayani ◽  
Nanan Sekarwana ◽  
Ryadi Fadil

Background Multiple blood transfusions in thalassemia patientslead to iron overload in bone tissue. Iron overload can bedetermined by serum ferritin measurement. Several studies haveevaluated association between serum ferritin level and growth,but without bone age examination.Objective To determine the association between age and serumferritin level with bone age in children with thalassemia major.Methods This study was conducted at Hasan Sadikin HospitalBandung during March-May 2007. We performed physicalexamination, serum feritin measurement, and bone ageexamination. Data were analyzed with x 2 to determine associationbetween variables. The association between age and serum ferritinlevel with bone age deficit was analyzed with regression logisticmodel.Results Subjects consisted of 49 patients with thalassemia major.All subjects had bone age deficit. Most boys were in age group of>10 years and had bone age difference >36 months, while mostgirls were <10 years and had bone age difference <36 months.Subjects with bone age difference <36 months mostly had serumferritin level <5,000 ng/dL, while most subjects with bone agedifference >36 months had serum ferritin level =5,000 ng/dL.This was statistically significant (x 2 =4.573, P=0.032). There wasassociation between age and bone age deficit (OR=13.461, 95%CI 3.199;56.640), but not with serum ferritin level (OR= 2.199,95% CI 0.532;9.095).Conclusion In thalassemic children, bone age deficit is associatedwith age, but not with serum feritin level.


Hemoglobin ◽  
2021 ◽  
Vol 45 (1) ◽  
pp. 69-73
Author(s):  
Salah H. AL-Zuhairy ◽  
Mohammed A. Darweesh ◽  
Mohammed A-M. Othman

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5520-5520
Author(s):  
Yongsheng Ruan ◽  
Xuedong Wu ◽  
Xiaoqin Feng ◽  
Yuelin He ◽  
Chunfu Li

Abstract Objectives: To evaluate the influence of pretransplantation serum ferritin on children β-thalassemia major (β-TM) undergoing allogeneic hematopoietic stem cell transplantation. Methods: A retrospective analysis of 266 HLA-matched children with β-TM from January 2009 and November 2014 was performed. Transplantation conditioning regimen of these children was the NF-08-TM protocol. Median follow-up time was 28 months (3~62months). We observed the relationship between pretransplantation serum ferritin level and transplantation complications which included infection, graft versus host disease(GVHD),veno-occlusive disease(VOD) and death. Results: Transplantation-related death occurred in 18 of 266 patients (6.8%). Five-year overall survival (OS) was found to be 92.8%. Among various complications, only infection was significantly associated with the high serum ferritin level (t=-2.673, P=0.008), especially serum ferritin above 3449.5μg/L(P=0.000). Meanwhile infection was the most common complication and severe infection would be main cause of deaths. Conclusions: NF-08-TM conditioning regimen was the optimization for HLA-matched β-TM patients. High pretransplantation serum ferritin level would bring high infection occurrence. Disclosures No relevant conflicts of interest to declare.


Author(s):  
Afshin Fathi ◽  
Firouz Amani ◽  
Samira Araghchin ◽  
Esmaeil Farzaneh

Background: Subcutaneous Deferoxamineis often not tolerated by patients and its rejection leads to iron overload with its complications. So, other methods with better toleration and reduction of Deferoxamine consumption are necessary. The present study aimed to evaluate the effect of intravenous Deferoxamine infusion during blood transfusion on serum ferritin (SF) in thalassemia major patients.Methods: In a retrospective cross-sectional study, thirty four patients with β-thalassemia major treated with monthly blood transfusion at Bu-Ali hospital in Ardabil city from April 2013 to April 2014, were selected and followed for six months. The mean SF rate and the needed subcutaneous Deferoxamine rates before intervention were considered as baseline. All patients received intravenous Deferoxamine concomitant with their routine monthly blood transfusion for six months. After six months mean values for ferritin, subcutaneous Deferoxamine were compared with baseline values. Collected data were analyzed using t-test and paired t-test by SPSS, version 18.P<0.05 was considered as significant.Results: Compared with baseline, the subcutaneous Deferoxamine rate and ferritin level have been decreased significantly after intervention.Conclusions: Intravenous Deferoxamine concomitant use with routine monthly blood transfusion in thalassemia major patients can lead to decreasing of ferritin level. With this method, patient care could be improved, health care costs and complications of treatment effectively reduced.


2010 ◽  
Vol 50 (2) ◽  
pp. 105
Author(s):  
Yovita Ananta ◽  
Pustika Amalia Wahidiyat ◽  
Hanifah Oswari

Background A high incidence rate of hepatitis B or C virusinfection is found among thalassemia children in Indonesia. Thismay influence deferiprone effectiveness.Objective To determine the effectiveness of deferiprone inthalassemia children with or without hepatitis B or C virusinfection.Methods A non-randomized clinical study was performed atThalassemia Center Jakarta. Subjects were thalassemia childrenwith serum ferritin level > 1000 ng/mL who had hepatitis B orC virus infection. A match control pair was recruited based onsimilar duration of transfusion therapy, thalassemia type, andinitial serum ferritin level. All subjects received initial deferipronedose of 50 mg/kg/day for 3 months. Those whose ferritin decreased2:: 10% continued to receive deferiprone of 50 mg/kg/day for thefollowing 3 months. Otherwise, deferiprone dose was adjustedto 75 mg/kg/day.Results Forty-eight subjects were recruited. After 3 months oftreatment, 16/24 subjects without and 6/24 subjects with hepatitisB or C had their ferritin level decreased 2:: 10%. Mean ferritinserum level of all subjects after 6 months was significantly reducedfrom 4734 (SD 2116) to 3695 (SD 1709) ng/mL. Lower meandeferiprone dose, lower mean post- study ferritin serum level andhigher mean percentage of ferritin serum level decrement werefound in subjects without hepatitis B or C infection than thosewith infection.Conclusions Deferiprone 50-7 5 mg/kg/day for 6 months is effectivein reducing serum ferritin level of thalassemia major children; itis more effective in thalassemia children without hepatitis B or Cvirus infection.


Author(s):  
Alaa Mutter Jabur Al-Shibany ◽  
AalanHadi AL-Zamili

Patients with transfusion dependent thalassemia major is often associated with iron overload. Proper use of iron chelators to treat iron overload requires an accurate measurement of iron levels. Magnetic resonance T2-star (T2* MRI) is the preferred method to measure iron level in the liver andthe heart. The goal of our study was to see if there is an association exists between serum ferritin level and T2* MRI results in patients with beta thalassemia major.This study was done in Al-Diwaniya Thalassemia center,Maternity and children teaching hospital,Iraq. During the period from 1st of January to 31st of October. Fifty eight patients with a diagnosis of beta thalassemia major were enrolled in the study. They were older than five years old,transfusion dependent and on chelation therapy. Hepatic and Myocardial T2*MRI and the mean serum ferritin levels were measured during the study period for all patients.There is a significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.018 ). also a significant correlation was observed between serum ferritin and hepatic T2*MRI (p=0.02). Neither cardiac T2* MRI nor hepatic T2* MRI show any correlation with the mean age.our study also showa positive correlation between the patients withcardiac T2* MRI and the development of diabetes mellitus in contrast to hepatic T2* MRI in which there is no any correlation. Hypothyroidism was observedno correlation with either cardiac or hepatic T2* MRI.Our results showed a positiveassociation between hepatic, cardiac T2*MRI and serum ferritin levels.


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