scholarly journals ASSESSMENT OF SERUM SCLEROSTIN LEVEL AS A BIOMARKER ASSOCIATED WITH BONE DISORDERS IN Β-THALASSEMIA PATIENTS IN AL- NAJAF CITY, IRAQ

2021 ◽  
Vol 29 (31) ◽  
pp. 10-20
Author(s):  
SHARBA Intisar Razzaq ◽  
AL-DUJAILI Arshad Noori
Keyword(s):  
Hematological Parameters ◽  
Thalassemia Major ◽  
Normal Weight ◽  
The Body ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Age Group ◽  
Blood Disorder ◽  
The Relationship ◽  
Sclerostin Level

Background: β-thalassemia is a blood disorder in which the body does not make hemoglobin normally. Aim: To assess serum sclerostin in female patients with beta-thalassemia and compare with the healthy controls and to predict its complication associated with the bone pathophysiology, for designed improvement the lifestyle goodliness for these patients. Material and methods: Sixty-nine female beta-thalassemia (βT) patients (54 βT major and 15 βT Intermedia), aged 8-40 years who dependent on transfused blood, and 20 healthy controls were evaluated serum sclerostin, and was examined the relationship with hematological parameters RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The information of beta-thalassemia patients was collected and records by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was showed in βT patients compared with the healthy controls (10.03±0.68, p  smaller than  0.001) pg/ml. Furthermore, a significant decrease (p smaller than 0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p smaller than 0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p smaller than 0.05) of βT patients. Conclusions: Sclerostin plays an important role in beta-thalassemia patients and can serve as a biomarker associated with the bone pathophysiology and indicator to prevent the continuation of such serious diseases caused by iron overload in these patients.

scholarly journals ASSESSMENT OF SERUM SCLEROSTIN LEVEL AS A BIOMARKER ASSOCIATED WITH BONE DISORDERS IN Β-THALASSEMIA PATIENTS IN AL- NAJAF CITY, IRAQ

2022 ◽  
Author(s):  
Intisar Razzaq SHARBA ◽  
◽  
Arshad Noori AL-DUJAILI
Keyword(s):  
Serum Iron ◽  
Hematological Parameters ◽  
Thalassemia Major ◽  
Normal Weight ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Age Group ◽  
The Relationship ◽  
Sclerostin Level ◽  
Weight Groups

Aim of the study: To assess serum sclerostin in female patients with beta-thalassemia and compare with the healthy controls and to predict its complication associated with the bone pathophysiology, for designed improvement the lifestyle goodliness for these patients. Material and methods: Sixty-nine female beta-thalassemia (βT) patients (54 βT major and 15 βT Intermedia), aged 8-40 years who dependent on transfused blood, and 20 healthy controls were evaluated serum sclerostin, and was examined the relationship with hematological parameters RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The information of beta-thalassemia patients was collected and recorded by the questioner. Results: A significantly increased serum sclerostin level (mean 26.80±0.91) pg/ml was shown in βT patients compared with the healthy controls (10.03±0.68, p < 0.001) pg/ml. Furthermore, a significant decrease (p<0.05) of the sclerostin level was observed in β-thalassemia major compared to intermedia β-thalassemia patients. Serum sclerostin level revealed a significant increase in progress age; it is highest in the age group (30-40) year as compared with age group (8-18) and (19-29) year respectively. Sclerostin showed no associations with the RBC, Hb, PCV, and significantly positively correlated (p<0.05) with serum iron, ferritin levels, WBC, and PLT count. Significantly higher sclerostin levels in splenectomized and underweight groups were observed compared to unsplenectomized and normal-weight groups (p<0.05) of βT patients. Conclusion: Sclerostin plays an important role in beta-thalassemia patients and can serve as a biomarker associated with the bone pathophysiology and indicator to prevent the continuation of such serious diseases caused by iron overload in these patients.

scholarly journals The Influence Of Parathyroid Gland On Beta-Thalassaemia Patients in Kirkuk City

2019 ◽  
Vol 24 (2) ◽  
pp. 10
Author(s):  
Demet F. Saber ◽  
Mossa M. Marbut ◽  
Sami A. Zbaar
Keyword(s):  
Parathyroid Gland ◽  
The Body ◽  
Normal Male ◽  
Healthy Controls ◽  
Age Group ◽  
Significant Elevation ◽  
Anthropometric Measures ◽  
Blood Disorder ◽  
Abnormal Form ◽  
Bone Abnormalities

Thalassaemia is an inherited blood disorder in which the body makes an abnormal form of hemoglobin. The symptoms of thalassemia appear in the first two years of life and include paleness of the skin, anemia, bone abnormalities, weakness and growth retardations. (70) male thalassaemic patients are diagnosed in thalassaemia center at Azadi Teaching Hospital in Kirkuk city  and( 30) normal male healthy Controls were included in this study Anthropometric measures include, Age, BMI, were done for all participants. there is significant elevation, (P> 0.01) in PTH in P2 age group (9-13) years as compare with control healthy subjectsand as compare with P1(4-8) and P3(14-18) age group. http://dx.doi.org/10.25130/tjps.24.2019.023  

scholarly journals RATIONAL EMOTIVE THERAPY IBU YANG MEMILIKI ANAK DENGAN THALASSAEMIA BETA MAYO

2019 ◽  
Vol 12 (1) ◽  
pp. 81-90
Author(s):  
Aprilia Putri Rahmadini
Keyword(s):  
Group Counseling ◽  
Rational Emotive Behavior Therapy ◽  
Thalassemia Major ◽  
The Body ◽  
Beta Thalassemia ◽  
Rational Beliefs ◽  
Rational Emotive Therapy ◽  
Beta Thalassemia Major ◽  
Blood Disorder ◽  
Rational Emotive

Beta Thalassaemia Major is a genetic blood disorder that causes blood cells red rapidly destroyed in 20-30 days so that the body is deprived of blood. Treatment can be done during this time is a lifetime of blood transfusions. Their conditions of denial of information mother about the child's condition, as was the condition of self is not a carrier of thalassemia trait, blaming the pair as a nature, thinking irrational that the child's illness is a punishment from God for their sins in the past, the child has no future again, resulting in the emergence of maternal behavior that may endanger the lives of children like too late to bring the child to transfusion. Research Purposes is to produce a group counseling program with Rational Emotive Behavior Therapy (REBT) approach in order to increasing acceptance of mothers of children with beta thalassemia major. Research Design is one group pretest-posttest. Group counseling with REBT approach is done in 6 meetings. In the process, the mother will discuss issues with members of the group and counselors who help mothers to detect the irrational belief that appears, discriminating rational beliefs, and challenging that belief, to be replaced by a rational belief. The subject are mothers who have children with beta thalassemia major amounted to 2 people. Results  known to both participants are still in bargaining phase. However, prevalence increased mother’s acceptance of children with beta thalassemia major, marked by decreased aspects of denial and anger on both mother. Conclusion Group counseling with REBT approach can improve mother’s acceptance of children with beta thalassemia major.

scholarly journals Thalassemia, a human blood disorder

2023 ◽  
Vol 83 ◽  
Author(s):  
F. Shafique ◽  
S. Ali ◽  
T. Almansouri ◽  
F. Van Eeden ◽  
N. Shafi ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Developmental Stages ◽  
Gene Mutations ◽  
Thalassemia Major ◽  
Marrow Transplant ◽  
The Body ◽  
Beta Thalassemia ◽  
Globin Genes ◽  
Blood Disorder ◽  
Alpha Thalassemia

Abstract A group of inherited blood defects is known as Thalassemia is among the world’s most prevalent hemoglobinopathies. Thalassemias are of two types such as Alpha and Beta Thalassemia. The cause of these defects is gene mutations leading to low levels and/or malfunctioning α and β globin proteins, respectively. In some cases, one of these proteins may be completely absent. α and β globin chains form a globin fold or pocket for heme (Fe++) attachment to carry oxygen. Genes for alpha and beta-globin proteins are present in the form of a cluster on chromosome 16 and 11, respectively. Different globin genes are used at different stages in the life course. During embryonic and fetal developmental stages, γ globin proteins partner with α globin and are later replaced by β globin protein. Globin chain imbalances result in hemolysis and impede erythropoiesis. Individuals showing mild symptoms include carriers of alpha thalassemia or the people bearing alpha or beta-thalassemia trait. Alpha thalassemia causes conditions like hemolytic anemia or fatal hydrops fetalis depending upon the severity of the disease. Beta thalassemia major results in hemolytic anemia, growth retardation, and skeletal aberrations in early childhood. Children affected by this disorder need regular blood transfusions throughout their lives. Patients that depend on blood transfusion usually develop iron overload that causes other complications in the body systems like renal or hepatic impairment therefore, thalassemias are now categorized as a syndrome. The only cure for Thalassemias would be a bone marrow transplant, or gene therapy with currently no significant success rate. A thorough understanding of the molecular basis of this syndrome may provide novel insights and ideas for its treatment, as scientists have still been unable to find a permanent cure for this deadly disease after more than 87 years since it is first described in 1925.

STUDY OF SERUM LIPID PROFILE IN BETA-THALASSEMIA MAJOR PATIENTS

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Lokesh Kumar Meena
Keyword(s):  
Lipid Profile ◽  
Total Cholesterol ◽  
Serum Lipid ◽  
Density Lipoprotein ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Age Group ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Low Levels

Background: To study lipid profile in Beta-Thalassemia Major Patients. Methods: A cross-sectional was done on 50 diagnosed Cases of beta-thalassemia major in the age group of 1 year to 18 years receiving regular blood transfusions; not suffering from any ailment or any other disease leading to deranged lipid profile were included. Results: Lipid analyses of controls and thalassemic children. It is clear from the results that beta thalassemia major patients had significantly lower total cholesterol (TC), high-density lipoprotein cholesterol (HDL) and low-density lipoprsotein cholesterol (LDL) compared with controls. Conclusion: Lipid profile in Beta thalassmia patients show significantly low levels of total cholesterol, LDLC and HDL-C. Keywords:  Beta Thalassemia Major, Lipid Profile, Hypocholesterolemia.

Fibroblast Growth Factor 23 (FGF23) and Klotho Protein in Beta-Thalassemia

2020 ◽  
Vol 52 (03) ◽  
pp. 194-201
Author(s):  
Dimitrios Stefanopoulos ◽  
Narjes Nasiri-Ansari ◽  
Ismene Dontas ◽  
Andromachi Vryonidou ◽  
Antonis Galanos ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor 23 ◽  
Iron Chelation ◽  
Thalassemia Major ◽  
The Body ◽  
Beta Thalassemia ◽  
Fibroblast Growth ◽  
Control Subjects ◽  
Number Of Patients

AbstractDerangements in phosphate and calcium homeostasis are common in patients with beta-thalassemia. Fibroblast growth factor 23 (FGF23) is among the main hormones regulating phosphate levels, while several studies underline an interplay between iron (Fe) and FGF23. Herein, we investigated, for the first time, the serum intact molecule (iFGF23) and the carboxyl-terminal fragment (C-FGF23) and Klotho levels simultaneously in patients with beta-thalassemia major receiving iron chelation regimens in comparison to healthy control subjects. We also correlated them with the body iron burden. The observational case-control study included 81 subjects (40 thalassemic patients and 41 healthy controls). Serum iFGF23, C-FGF23 and Κlotho were measured by ELISA. Parathormone, 25-hydroxycholecalciferol, calcium, and phosphorus were measured in blood and/or urine. The degree of hemosiderosis was evaluated by assessing the serum ferritin levels and performing T2* MRI measurements. Serum C-FGF23 levels were significantly lower in patients compared to control subjects (p=0.04), while iFGF23 and Klotho levels did not differ. Serum C-FGF23 levels were negatively correlated with ferritin (r=–0,421, p=0.018), whereas there were no significant correlations of each of the three factors with the iron chelation therapy. Decreased serum C-FGF23 levels were found in βTh patients which may be attributed to inhibition of proteolytic cleavage of iFGF23. Further studies in a greater number of patients will shed more light on the disturbances of the iFGF23, Klotho and C-FGF23 in thalassemia and their possible role in bone disease of such patients.

scholarly journals STUDY OF SERUM LIPID PROFILE IN BETA-THALASSEMIA MAJOR PATIENTS

2021 ◽  
Vol 5 (12) ◽  
Author(s):  
Poonam Meena ◽  
Satish Meena ◽  
Ashok Meena ◽  
Kailash Meena
Keyword(s):  
Lipid Profile ◽  
Total Cholesterol ◽  
Serum Lipid ◽  
Density Lipoprotein ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Age Group ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Low Levels

Background: To study lipid profile in Beta-Thalassemia Major Patients. Methods: A cross-sectional was done on 30 diagnosed Cases of beta-thalassemia major in the age group of 1 year to 18 years receiving regular blood transfusions; not suffering from any ailment or any other disease leading to deranged lipid profile were included. Results: Lipid analyses of controls and thalassemic children. It is clear from the results that beta thalassemia major patients had significantly lower total cholesterol (TC), high-density lipoprotein cholesterol (HDL) and low-density lipoprsotein cholesterol (LDL) compared with controls. Conclusion: Lipid profile in Beta thalassmia patients show significantly low levels of total cholesterol, LDLC and HDL-C. Keywords: Beta Thalassemia Major, Lipid Profile, LDL, HDL.

scholarly journals FACTORS RELATED TO ANEMIA IN PREGNANT WOMEN EVENTS IN GENERAL HOSPITAL REGIONAL dr. ZAINOEL ABIDIN

2017 ◽  
Vol 1 (2) ◽  
Author(s):  
Faradilla Safitri ◽  
Mizan Jalinan
Keyword(s):  
At Risk ◽  
Pregnant Women ◽  
General Hospital ◽  
Gestational Age ◽  
Univariate Analysis ◽  
The Body ◽  
Bivariate Analysis ◽  
Blood Disorder ◽  
The Relationship ◽  
Education Work

Anemia is a common blood disorder that occurs when levels of red bloodcells in the body becomes too low. Data from Riskesdas the year 2013 thenumber of pregnant women suffer from anemia as much as 37.1%, this isvery different to the incidence of anemia among pregnant women in theDistrict General Hospital dr. Zainoel Abidin with a very low percentage of2.9% to 3.4%. To analyze the relationship between age, parity, education,occupation, gestational age, origin of a referral, and health servicesResearch methodsThis type of research is analytic survey using casecontrol design. Univariate analysis found women with anemia 33.3% andanemia not as much as 66.7%. Bivariate analysis between age <20 yearswith anemia (p = 0.001, OR = 9.549), parity (p = 0.001, OR = 1.955),gestational age (p = 0.027, OR = 1.608), education (p = 0.002, OR =2.338), occupation (p = 0.006, OR = 2.167). Results of multivariateanalysis the dominant variable is the age of the OR (age> 35 years =9.009 and aged <20 years = 6.994). No correlation between age, parity,gestational age, education, work with anemia among pregnant women. allpregnant women at risk for anemia, pregnant women should routinely visitantenatal care, because the mother will get information about thenutritional needs during pregnancy and other factors that can lead toanemia during pregnancy.Keywords: Anemia, age, education, gestational age, parity

scholarly journals Evaluation of Ischaemia Modified Albumin as a Marker of Oxidative Stress in Beta Thalassemia Major Children

2021 ◽  
Vol 41 (1) ◽  
pp. 61-66
Author(s):  
K Jagadish Kumar ◽  
Smriti Bhagiratha ◽  
Prashanth Vishwanath
Keyword(s):  
Oxidative Stress ◽  
Cell Damage ◽  
Significant Negative Correlation ◽  
Thalassemia Major ◽  
Organ Damage ◽  
Beta Thalassemia ◽  
Healthy Controls ◽  
Oxygen Species ◽  
Regression Relationship ◽  
Healthy Control

Introduction: Iron overload in thalassemia catalyses the production of a variety of reactive oxygen species leading to cumulative cell damage. Ischemia modified albumin (IMA) is an end product of oxidative stress. It is imperative to pick up oxidative stress early in order to prevent the organ damage in thalassemia. Therefore this study was undertaken to estimate IMA levels and to see the correlation between ferritin and IMA to establish whether ferritin can be a proxy marker for oxidative stress. Methods: A total of 76 children were included in the study out of which 46 were diagnosed cases of β- Thalassemia major and 30 formed the healthy controls. Pre transfusion haemoglobin, AST, ALT, ferritin and IMA levels were estimated and compared with healthy control children. Correlation was drawn between haemoglobin, AST, ALT, ferritin with IMA. Results: There is significant elevation in the level of IMA and ferritin in children with Thalassemia major as compared to the healthy controls (p = < 0.001). There was a significant positive correlation between ferritin and IMA and a significant negative correlation between haemoglobin % and IMA. Regression relationship between ferritin and IMA established that IMA (ng/ mL) = 246.118 + 0.829 (Ferritin ng/dL). Conclusions: IMA levels were significantly elevated in β- thalassemia major children and correlated positively with ferritin levels. By establishing a regression relationship between ferritin and IMA levels, we can fairly estimate the levels of IMA. Hence, we can utilise ferritin as a proxy marker of oxidative stress instead of IMA.

scholarly journals Thalassemia

2021 ◽  
Author(s):  
Tekin Aksu ◽  
Şule Ünal
Keyword(s):  
Clinical Laboratory ◽  
Hematological Parameters ◽  
Thalassemia Major ◽  
Microcytic Anemia ◽  
Beta Thalassemia ◽  
World Population ◽  
Hypochromic Microcytic Anemia ◽  
The World ◽  
Laboratory Features

Defects in protein structure or synthesis of hemoglobin are called hemoglobinopathies. Thalassemia is the most common hemoglobinopathy, and it is estimated that 5% of the world population carries at least one variant allele of thalassemia. The thalassemias can be classified as alpha or beta thalassemias. Beta thalassemia may present as silent carriers with normal hematological parameters, while beta thalassemia carriers have hypochromic microcytic anemia, associated with a high HbA2. However, patients with beta thalassemia intermedia and beta thalassemia major need transfusion intermittently or regularly and they are called non-transfusion dependent thalassemias or transfusion-dependent thalassemias, respectively. This review focuses on pathophysiology, clinical, laboratory features of thalassemias along with their treatment and follow-up.

Sign in / Sign up

Export Citation Format

Share Document