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

Author(s):  
Intisar Razzaq SHARBA ◽  
◽  
Arshad Noori AL-DUJAILI

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.

Author(s):  
SHARBA Intisar Razzaq ◽  
AL-DUJAILI Arshad Noori

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.


Author(s):  
Lokesh Kumar Meena

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.


Author(s):  
Poonam Meena ◽  
Satish Meena ◽  
Ashok Meena ◽  
Kailash Meena

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.


2021 ◽  
Vol 41 (1) ◽  
pp. 61-66
Author(s):  
K Jagadish Kumar ◽  
Smriti Bhagiratha ◽  
Prashanth Vishwanath

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.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 957-957 ◽  
Author(s):  
Ji Heon (Paul) Lee ◽  
Alfred W. Rademaker ◽  
Bayard L. Powell ◽  
Susan Geyer ◽  
Richard A. Larson ◽  
...  

Abstract Introduction Emerging data are demonstrating that obesity continues to be an epidemic among the pediatric population. A number of studies in children and adults reported that obesity at diagnosis affects survival in some cancers. Some reports show that obesity is an independent prognostic factor in patients in APL.1,2 The impact of obesity and outcome has never been studied in pediatric patients with APL. Methods Patient data were collected and analyzed from APL patients treated on the North American Leukemia Intergroup C9710 study led by the Cancer and Leukemia Group B (Alliance), with participation by the Children’s Oncology Group (COG). Only patients with BMI data were included in these analyses (n=529) across all age groups. Children and adolescents (<20 years old) were divided into weight categories as follows: underweight, less than the 5th percentile; normal weight, from the 5th percentile to less than the 85th percentile; overweight, from the 85th percentile to less than 95th percentile; and obese, equal or greater than the 95th percentile (Center for Disease Control and Prevention; http://apps.nccd.cdc.gov/dnpabmi/). BMI for patients ≥20 years old were divided into weight categories: underweight, normal weight, overweight, obese, using the following cutpoints: underweight (BMI <18.5 kg/m2), normal weight (BMI 18.5-25 kg/m2), overweight (BMI 25-29.9 kg/m2), and obese (BMI ≥ 30 kg/m2). Body weight categories were defined according to age- and sex-specific BMI percentiles. Both univariate and multivariate analysis were calculated using other factors such as age, gender, ethnicity and WBC at diagnosis (high risk if WBC ≥10,000). Recurrence-free survival (RFS) and overall survival (OS) were analyzed using Kaplan and Meier methods, and Log-rank tests used to assess prognostic impact of factors on survival distributions; p-values <0.05 were considered statistically significant. Results In this group of 529 patients, 428 were ≥20 years old (y.o.) vs. 101 who were < 20 y.o. Within the < 20 y.o. group, the majority had normal weight (44.6%) based on BMI while the majority of the ≥20 y.o. group were obese (50.2%). A high percentage of those < 20 y.o. were also obese (33.7%). Median follow-up times for the <20 y.o. vs. >20 y.o. groups were 62.0 months (range, 1-149.8 months) vs. 96.7 months (range, 0.03 – 156.3 months), respectively. When compared to all other weight groups, OS and RFS did not significantly differ between obesity and other weight groups in patients < 20 y.o. (5-year OS rates: 64% vs 57%, p=0.94; and 5-year RFS rates: 83% vs 79%; p=0.60). However, in patients ≥20 years of age, RFS was worse in obese patients versus other weight groups (67% vs 77%, p = 0.026) as was OS (76% vs 85%, p = 0.023). Furthermore, we investigated therapy-related toxicities in the same weight groups. None of the toxicities (hematologic, infection, metabolic, pain, pulmonary) were significantly related to weight group (obese vs non-obese) for either age group (<20 years or >20 years). Discussion This is the first study to report obesity in relation to clinical outcomes or toxicity in children and adolescents with APL <20 years of age. Here, obesity did not confer poorer RFS or OS in this younger age group. In contrast, this study confirms a previous Alliance study showing that increased BMI in adult patients (>18 years) with APL is associated with worse RFS and OS.2 It is not clear why BMI and obesity do not have prognostic influence on clinical outcomes in patients less than 20 years of age. Possible theories include confounding factors in adults such as poor compliance by race or socioeconomic status in obese patients, whereas close monitoring by parents may play a role for pediatric patients in this setting. Future studies would need to be dedicated to address these theories. 1. Breccia M, Mazzarella L, Bagnardi V, et al. Blood. 2012;119(1):49-54. 2. Castillo JJ, Mulkey F, Geyer S, et al. Blood. 2013;122(21):832. Disclosures Rademaker: NIH Grant Review: Honoraria; AACR Faculty: Honoraria; Georgetown Univeristy Advisor: Honoraria. Hijiya:Sanofi: Consultancy; Jazz Pharma: Consultancy; Pfizer: Consultancy; Sigma Tau: Consultancy.


2021 ◽  
Author(s):  
Tekin Aksu ◽  
Şule Ünal

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.


Author(s):  
Intisar Razzaq Sharba ◽  
Arshad Noori Al-Dujaili

E-selectin, as identified (CD62E), is expressed on endothelial cells after stimulation with inflammation cytokines. β-Thalassemia diseases (βT) and early diagnosis are of utmost significance in the entire world population. This study was performed in the Thalassemia Center of the Al-Zahraa Educational Hospital in Al-Najaf Province, Iraq, on sixty-nine with β-thalassemia (54 βT major and 15 βT Intermedia) aged 8-40 years who transfused blood. Compared to 20 healthy volunteers as a control group. In both βT patients and healthy groups were assessed serum E-selectin levels. It was investigated the relationship with RBC, Hb, PCV, WBC, PLT, BMI, splenic status, iron, and ferritin levels. The results revealed a significant (P<0.05) decreased values of HB, RBC, P.C.V, and BMI. In contrast, values of WBC, PLT, Iron, and Ferritin were significantly increased in βT patients as compared to the healthy control groups. A significant (P<0.05) increase in serum E- Selectin level in βT patients (20.55±0.47) ng/ml to compare with the healthy group (9.16±0.50) ng/ml. Furthermore, it was a significant decrease in groups of βT major (19.87±0.42) ng/ml more than in βT intermedia (23±1.42) ng/ml. E-Selectin revealed a significant increase (P<0.05) in progress age and associated with splenectomies and underweight groups compared to splenectomies and the normal weight groups, respectively. Also, E-Selectin levels significantly positively correlated with WBC, PLT value, iron, and Ferritin levels. However, it was no significant with RBC, PCV, Hb. As a conclusion from this study, E- Selectin is an important biomarker in β-thalassemia patients can be identified as the complications associated with iron overload, inflammatory process, and endothelial dysfunction in βT disease.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3851-3851
Author(s):  
Amit Kandhari ◽  
Anupam Sachdeva ◽  
Neeraj Jain ◽  
Subash C. Arya ◽  
Virender K. Khanna ◽  
...  

Abstract Thalassemia is one of the commonest hereditary hemolytic anemia. Survival of patients with thalassemia major has greatly improved during recent years, with the introduction of moderate -transfusion programs and chelation therapy with subcutaneous Desferal (deferrioxamine) and/or Kelfer (Deferiprone). The repeated transfusions in thalassemics lead to deposition of iron in different tissues, leading to damage and dysfunction of various systems. Iron deposition is also reported to exist in the lungs at autopsy. Although lung impairment in thalassemic patients has been reported in the 1980’s, yet it is one of the most under evaluated and functionally not well-characterized complication. Since there have been contradictory results reported in literature ranging from restrictive spirometric pattern to an obstructive one, and there is total paucity of data from India, this study was designed to find out the pulmonary function tests of the beta thalassemic patients attending our hospital. A total of 30 cases of beta thalassemia major who were attending the thalassemia clinic at Sir Ganga Ram Hospital were taken up for the study after a written informed consent. These patients were on moderate -transfusion regimen, maintaining a hemoglobin concentration of more than 9 gm %. Patients with asthma and cardiac disease were excluded. Pulmonary function tests done included Forced vital capacity (FCV), Forced expiratory volume in one second (FEV1), FEV1/FVC percent (FEV1 %) and peak expiratory flow rate (PEFR). Results were interpreted as obstructive, restrictive and normal pulmonary Function test (PFT). The data was analyzed using software SPSS version 10. We included three age groups in our study, with total 30 patients in all. Age group 6–10 yr had 9 patients (mean Serum ferritin 2301 ng/ml), 11–15 yr had 11 children (mean Serum ferritin 3315) and &gt;15 yr had 11 children (mean Serum ferritin 5147 ng/ml) in the study. Mean age was 14.46 with standard deviation of 6.75 (mean ferritin was 3682 ng/ml). Overall out of 30 patients 13 had normal PFT and 17 had abnormal PFT (1 had obstructive PFT and 16 had restrictive PFT). In the age group of 6–10 years 7 had normal and 2 had abnormal PFT and in age group 11–15 yr 3 had normal and 6 had abnormal PFT and in age group &gt;15 yr 3 had normal and 8 had abnormal PFT. Of 17 patients on chelation with Desferal alone 11 had normal and 6 had abnormal PFT. 6 patients were on Kelfer alone of which 1 had normal and 5 had abnormal PFT. Of 7 patients who were on both Desferal and Kelfer, 1 had normal and 6 had abnormal PFT. All patients on Kelfer or Kelfer + Desferal aged &gt;10 years except one which may explain reason for increased number of abnormal results in this group. Critical age above which abnormal results were higher was 11.5 years. Mean Ferritin in patients with normal PFT was 2456 ng/ml and 4621 ng/ml for patients with abnormal PFT. (p value 0.037) We found a negative Pearson correlation between FVC (% of predicted) with increasing ferritin (p value 0.038). There was no difference detected between pre and post transfusion PFT results by using paired t test (p value 0.973). In conclusion the major pulmonary dysfunction in our study group is that of restrictive type. The abnormal PFT were found in older age group. FVC was negatively co-related with increasing ferritin level. The number of abnormal results was higher when the serum ferritin was &gt; 4000 ng/ml and blood transfusion had no effect on the PFT.


2008 ◽  
Vol 11 (10) ◽  
pp. 1085-1088 ◽  
Author(s):  
Zuhal Gundogdu

AbstractAimTo investigate the relationship between BMI and blood pressure as this is of crucial interest in evaluating both public health and the clinical impact of the so-called obesity epidemic.MethodsData were gathered from 1899 children aged between 6 and 14 years, analysing and evaluating a possible relationship between BMI and systolic and diastolic blood pressure values for both girls and boys. Each child was classified on the basis of age- and sex-specific BMI percentile as normal weight (<85th percentile), overweight (≥85th and <95th percentile) or obese (≥95th percentile).ResultsIn comparisons among age–sex–BMI percentile groups, systolic and diastolic blood pressure values were higher in obese and overweight groups than in normal weight groups for both sexes. Although BMI among girls was higher than among boys in all three percentile groups, there were no significant differences between sexes with respect to blood pressure values.ConclusionThe present findings emphasize the importance of the prevention of obesity in order to prevent future related problems such as hypertension in children and adolescents.


Author(s):  
Kristina L Tobing ◽  
Olwin Nainggolan ◽  
Faika Rachmawati ◽  
Helper Sahat P Manalu ◽  
Ratna Dilliana Sagala ◽  
...  

The study aimed to determine the relationship between nutritional status based on Body Mass Index (BMI) and Tuberculosis (TB) in the age group >18 years. This study further analyzes the 2018 Basic Health Research (Riskesdas 2018), a cross-sectional study conducted in all city districts in 34 provinces of Indonesia. Unit samples of this study are population-based in the age group >18 years. The determination of having had TB is if the respondent answered yes to the question: "Has a doctor ever been diagnosed with TB by a period of ≤1 year". Nutritional status based on BMI value by calculating height per body weight. Nutritional status was classified as: underweight: BMI <18.5; normal: BMI ≥18.5 - <25.0; Overweight: BMI ≥25.0 - <27.0; obesity: BMI ≥27.0. The total sample was 624,562. The number of TB sufferers 3,220 (0.5%). The number of underweight respondents 62,456 (9.4%), normal respondents 344,941 (55.2%), overweight and obese respondents 221,021 (35.4%). There is a relationship between nutritional status and TB after controlling for confounding variables such as gender, education, type of work, residence, DM risk factors, and smoking behavior. Underweight has a risk of 7.67 (CI 6.40-9.20) of getting TB compared to overweight/obesity, while normal weight has a risk of 2.55 (CI 2.18-2.98) of getting TB compared to overweight/ obesity after controlled confounding variables. Underweight people have 7.67 times the risk of getting TB, and normal weight has 2.55 times have the prevalence ratio getting TB than obese people. The Additional food program for TB patients whose weight is thin in the age group >18 years.


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