scholarly journals Zinc Status in Beta-Thalassemia Major

2021 ◽  
Vol 9 (B) ◽  
pp. 149-153
Author(s):  
Khalaf Hussein Hasan ◽  
Hasan Abdulla Aswad ◽  
Aspazija Sofijanova
Keyword(s):  
Serum Ferritin ◽  
Ferritin Level ◽  
Disease Onset ◽  
Serum Zinc ◽  
Thalassemia Major ◽  
Adverse Outcomes ◽  
Beta Thalassemia ◽  
Control Group ◽  
Healthy Children ◽  
Zinc Status

BACKGROUND: Zinc is one of the most important minerals incorporated in the enzymes of the human body. Zinc may be deficient in patients with the β-thalassemia major with possible adverse outcomes. AIM: The purpose of this study was to assess the serum zinc status in β-thalassemia major patients in Duhok city. PATIENTS, MATERIAL, AND METHODS: In this case–control study, 70 children with β-thalassemia major (2–12 years) of both genders were enrolled and were matched with 70 apparently healthy children for age and sex. A venous blood sample was obtained from each child for the measurement of serum zinc and serum ferritin levels at Jin Center in Duhok City between January 1 and June 30, 2017. RESULTS: The mean serum zinc in the thalassemia patients (74.79 [±25.14] μg/dl) was significantly lower compared to the control group (93.61 [±15.12] μg/dl), (p = 0.0001). The serum zinc was not significantly different in thalassemia patients in terms of age, disease onset, gender, height, weight, body mass index, amount of blood transfusion, and type of chelation. There was a statistically significant correlation between serum zinc levels with a serum ferritin level of patients. CONCLUSION: The study showed that thalassemia patients have significantly lower serum levels of zinc with no relation to medical factors.

scholarly journals Neurocognitive Function and Its Related Potentials in Children with Beta Thalassemia Major: An Egyptian Study

2019 ◽  
Vol 7 (3) ◽  
pp. 322-328 ◽  
Author(s):  
Inas R. El-Alameey ◽  
Fatma Alzaree ◽  
Manal A. Shehata ◽  
Mones M. Abu Shady ◽  
Mohamed Abdel Atti ◽  
...  
Keyword(s):  
Serum Iron ◽  
Digit Span ◽  
Block Design ◽  
Disease Onset ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Control Group ◽  
Healthy Children ◽  
Duration Of Illness ◽  
Related Potentials

BACKGROUND: Repeated blood transfusions and hemolysis in β-Thalassemia major children lead to iron overload in various organs, including the brain which may cause neurodegeneration. AIM: To evaluate intelligence quotient in children with β-thalassemia major and healthy counterparts and to assess risk factors that cause cognitive problems. SUBJECTS AND METHODS: This case-control study was performed on 50 children aged 6-16 years old with β-thalassemia major as patients group and compared with 50 healthy children as a control group of matched age, sex, and social class. Cognitive functions were evaluated by using the Wechsler Intelligence Scale for Children. Serum ferritin and iron were measured by ELISA. RESULTS: There were significantly lower mean performance and full-scale IQ scores of patients group in comparison with controls, whereas no significant differences between both groups as regards to a verbal IQ score. In thalassemic children, block design, comprehension and arithmetic were negatively correlated with age of disease onset, duration of illness and onset of chelation therapy. Serum iron and ferritin were negatively correlated with similarities and digit span. Serum iron levels were negatively correlated with performance IQ score. CONCLUSION: Children with β-thalassemia major need to receive more academic attention and cognitive assessment to improve their IQ.

scholarly journals Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 100
Author(s):  
Asmaa A. Mahmoud ◽  
Doaa M. Elian ◽  
Nahla MS. Abd El Hady ◽  
Heba M. Abdallah ◽  
Shimaa Abdelsattar ◽  
...  
Keyword(s):  
Cystatin C ◽  
Kidney Injury ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Good Survival ◽  
Control Group ◽  
Healthy Children ◽  
Serum Cystatin C ◽  
Serum Cystatin ◽  
Beta Thalassemia Major

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

scholarly journals THERAPEUTIC VALUE OF COMBINED THERAPY WITH DEFERASIROX AND SILYMARIN ON IRON OVERLOAD IN CHILDREN WITH BETA THALASSEMIA

2013 ◽  
Vol 5 (1) ◽  
pp. e2013065 ◽  
Author(s):  
Adel Abd elhaleim Hagag
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Iron Status ◽  
Iron Chelator ◽  
University Hospital ◽  
Beta Thalassemia ◽  
Control Group ◽  
Healthy Children ◽  
Number Of Patients

abstractBackground: Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring life-long blood transfusion that cause iron overload. Silymarin plays a role as oral iron chelator and hepatoprotective agents in thalassemic patients.The aim of this work was to determine silymarin value as an iron chelator in thalassemic patients with iron overload.Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital having serum ferritin level more than 1000 ng/ml and was divided in two groups. Group IA: Received oral Deferasirox (Exjade) and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade) and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this research. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001). Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: Extensive multicenter studies in large number of patients with longer duration of follow up and more advanced methods of assessment of iron status is recommended to clarify the exact role of silymarin in reduction of iron over load in children with beta thalassemia.  

scholarly journals Nutritional Biomarkers in Children and Adolescents with Beta-Thalassemia-Major: An Egyptian Center Experience

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Laila M. Sherief ◽  
Sanaa M. Abd El-Salam ◽  
Naglaa M. Kamal ◽  
Osama El safy ◽  
Mohamed A. A. Almalky ◽  
...  
Keyword(s):  
Trace Elements ◽  
Folic Acid ◽  
Serum Zinc ◽  
Serum Levels ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Healthy Children ◽  
Multicenter Studies ◽  
Thalassemic Patient ◽  
Beta Thalassemia Major

Background and Aim. Trace elements and vitamins play a vital role in human body to perform its function properly. Thalassemic patients are at risk of micronutrient deficiency. This study estimated levels of vitamins A, C, E, B12, folic acid, total homocysteine (tHcy), and methylmalonic acid (MMA) along with trace elements, zinc, copper, and selenium in Beta-thalassemia-major patients.Methods. This study included 108 patients with Beta-thalassemia-major and 60 age and sex matched healthy children. Serum levels of vitamin A, E, C, tHcy, and MMA were estimated by high pressure liquid chromatography while serum levels of folic acid and B12 were estimated by thin layer chromatography. Serum zinc, copper, and selenium were determined by atomic absorption spectrometry.Results. There was a significant decrease of vitamins A, C, E, and B12 and trace elements zinc, copper, and selenium in thalassemic patients as compared to controls. tHcy and MMA were significantly elevated in patients. No significant correlations were found between the serum levels of the studied vitamins and trace elements as regards age, frequency of transfusion, duration of transfusion, and serum ferritin.Conclusion. The level of various nutritional biomarkers (vitamins A, C, E, and B12 and trace elements zinc, copper, selenium) was reduced in chronically transfused Egyptian thalassemic patient. These patients should have periodic nutritional evaluation and supplementation. Multicenter studies are highly recommended.

scholarly journals Correlation between serum ferritin level, cardiac and hepatic T2-star MRI in patients with β-thalassemia major in Al-Diwaniya thalassemia center

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Alaa Mutter Jabur Al-Shibany ◽  
AalanHadi AL-Zamili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Iron Level ◽  
The Mean ◽  
T2 Mri

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.

scholarly journals Left ventricular dysfunction in transfusion dependent beta Thalassemia major patients in Bangladesh

2016 ◽  
Vol 9 (1) ◽  
pp. 31-35
Author(s):  
Simu Saha ◽  
Tapash Saha ◽  
AKM Amirul Morshed ◽  
Md Lutful Ehsan Fatmi ◽  
Nazneen Umme Zakia ◽  
...  
Keyword(s):  
Diastolic Dysfunction ◽  
Serum Ferritin ◽  
Cardiac Dysfunction ◽  
Serum Ferritin Level ◽  
Ventricular Dysfunction ◽  
Early Stage ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Beta Thalassemia

Background: Thalassemia major is an inherited haemoglobin disorder resulting in chronic haemolytic anaemia. Patients with beta thalassemia major are maintained on continuous blood transfusion regimens resulting in iron overload that adversely affects both the structure and function of the heart and other vital organs which can be easily prevented with iron chelating therapy. The aim of the study was to detect left ventricular dysfunction at an early stage so that early effective intervention can be done.Methods: A total of 50 patients with beta thalassemia were included in the study by non randomized qualitative purposive sampling from July 2013 to June 2014. Their total body iron status was be assessed by doing serum ferritin level. Left ventricular systolic and diastolic function was assessed by echocardiographyResults: Cardiac dysfunction was present in 11 patients with high incidence in patients with low pre-transfusional haemoglobin group (p=0.4) and in patients having high serum ferritin level (p=0.02). Systolic cardiac dysfunction was present in 7(14%) of patients and diastolic dysfunction was present in 4(8%) of patients. There was a weak but significant correlation between left ventricular ejection fraction and serum ferritin concentration (r=-0.22; p=0.03). Only few (8%) patients had diastolic dysfunction.Conclusion: Patients with beta thalassaemia on an adequate transfusion showed an abnormal left ventricular systolic function. In early stage of disease diastolic function was normal but after repeated transfusion there were impaired relaxation indicating diastolic dysfunction. These findings seem mainly to be related to chronic anaemia and serum ferritin levelCardiovasc. j. 2016; 9(1): 31-35

scholarly journals PREVALENCE OF HYPOTHYROIDISM, DELAYED PUBERTY AND DIABETES MELLITUS IN PATIENTS OF Β-THALASSEMIA MAJOR

2020 ◽  
Vol 4 (3) ◽  
Author(s):  
Naresh Manne ◽  
Bharat Kumar Gupta ◽  
Sandeep Kumar Yadav ◽  
Saurabh Singhal ◽  
Archana Dubey
Keyword(s):  
Diabetes Mellitus ◽  
Blood Transfusion ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Delayed Puberty ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Beta Thalassemia Major

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.

Investigation of correlation between H63D and C282Y mutations in HFE gene and serum Ferritin level in beta-thalassemia major patients

2019 ◽  
Vol 26 (4) ◽  
pp. 249-252
Author(s):  
Romina Rahmani ◽  
Parisa Naseri ◽  
Ava Safaroghli-Azar ◽  
Shahriar Tarighi ◽  
Tahereh hosseini ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Hfe Gene ◽  
Beta Thalassemia Major

scholarly journals Thyroid Function Status and Echocardiography Abnormalities in Patients with Beta Thalassemia Major in Bahrain

2013 ◽  
Vol 7 ◽  
pp. CMC.S10702 ◽  
Author(s):  
Taysir S. Garadah ◽  
Najat A. Mahdi ◽  
Ahmed M. Jaradat ◽  
Zuheir A. Hasan ◽  
Das S. Nagalla
Keyword(s):  
Regression Analysis ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Systolic Function ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Tissue Doppler ◽  
Thyroid Stimulating Hormone ◽  
Beta Thalassemia ◽  
Pulsed Doppler

Background Thyroid gland dysfunction and echocardiographic cardiac abnormalities are well-documented in patients with transfusion dependent beta-thalassemia major (β-TM). Aim This cross-sectional analytic study was conducted to investigate left ventricle (LV) diastolic and systolic function using pulsed Doppler (PD) and tissue Doppler (TD) echocardiography and correlate that with serum level thyroid stimulating hormone in patients with β-TM. Methods The study was conducted on patients with β-TM (n = 110, age 15.9 ± 8.9 years) and compared with a control group (n = 109, age 15.8 ± 8.9 years). In all participants, echocardiographic indices of PD and TD were performed and blood samples were withdrawn for measuring the serum level of TSH, free T4, and ferritin. A linear regression analysis was performed on TSH level as the dependent variable and serum ferritin as independent. Stepwise multiple regression analysis was used to determine the odds ratio of different biochemical and echo variables on the risk of developing hypothyroidism. Results Patients with β-TM compared with controls had thicker LV septal wall index (0.65 ± 0.26 vs. 0.44 ± 0.21 cm/M2, P < 0.001), posterior wall index (0.65 ± 0.23 vs. 0.43 ± 0.21 cm/m2, P < 0.01) and larger LVEDD index (4.35 ± 0.69 vs.3.88 ± 0.153 mm/m2, P < 0.001). In addition, β-TM patients had higher transmitral E wave velocity (E) (70.81 ± 10.13 vs. 57.53 ± 10.13 cm/s, P = 0.02) and E/A ratio (1.54 ± 0.18 vs. 1.23 ± 0.17, P < 0.01) and shorter deceleration time (DT) (170.53 ± 13.3 vs. 210.50 ± 19.20 m sec, P < 0.01). Furthermore, the ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus (E/Em) was significantly higher in the β-TM group (19.68 ± 2.81 vs. 13.86 ± 1.41, P < 0.05). The tissue Doppler systolic wave (Sm) velocity and the early diastolic wave (Em) were significantly lower in the β-TM group compared with controls with Sm, 4.82 ± 1.2 vs. 6.22 ± 2.1 mm/sec, P < 0.05 and (Em), 3.51 ± 2.7 vs. 4.12 ± 2.5 mm/sec. P < 0.05, respectively). The tricuspid valve velocity was significantly higher in β-TM patients compared with controls 2.85 ± 0.56 vs. 1.743 ± 0.47 m sec, respectively, P < 0.01). The prevalence of subclinical hypothyroidism in patients with β-TM was 15.4%, with significantly higher mean serum TSH compared with controls (6.78 ± 1.5 vs. 3.10 ± 1.02 μIU/mL, P < 0.01) and positively correlated with the serum ferritin level ( r = 0.34, P = 0.014). On multiple regression analysis, the LV mass, LVEF%, and E/A ratio were not positive predictors of hypothyroidism in patients with β-TM. Conclusion We conclude that patients with β-TM had a high prevalence of subclinical hypothyroidism of 15.4%. Thyroid stimulating hormone was significantly high and positively correlated with the serum ferritin level. Echo cardiographic pulsed Doppler showed a restrictive LV diastolic pattern suggestive of severe diastolic dysfunction with preserved left ventricle systolic function.

EVALUATION OF DIASTOLIC DYSFUNCTION IN CHILDREN WITH ASYMPTOMATIC BETA- THALASSEMIA MAJOR AN

2021 ◽  
pp. 5-7
Author(s):  
Radhabinod Pal ◽  
Akshay Devendra Dhore ◽  
Biswarup Sarkar
Keyword(s):  
Diastolic Dysfunction ◽  
Diastolic Function ◽  
Ferritin Level ◽  
Normal Subjects ◽  
Thalassemia Major ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Control Group ◽  
Cross Sectional ◽  
Medical College

This was an observational cross-sectional study of 96 Patients at R.G Kar Medical College and Hospital, st Kolkata. with diagnosis of β Thalassemia major children aged between 2-12 years between 1 March, th 2016 to 28 February, 2017. A control group of 100 normal subjects matched according to age and sex, with the cases were also studied. Patients who full the inclusion and exclusion criteria were enrolled for the study after getting written informed consent. Echocardiography study was done of all the patients for assesment of diastolic function and determined whether it has any correlation with serum ferritin level in asymptomatic β Thalassemia major. It showed 66.67% of children were suffering from diastolic dysfunction, most of them were in grade 1 diastolic dysfunction followed by grade 3 diastolic dysfunction. The grade of diastolic function was correlated to the serum level of ferritin

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