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.

scholarly journals Estimation of iron overloads using oral exfoliative cytology in beta-thalassemia major patients

CytoJournal ◽  
2016 ◽  
Vol 13 ◽  
pp. 6 ◽  
Author(s):  
Swati Leekha ◽  
Amit Kumar Nayar ◽  
Preeti Bakshi ◽  
Aman Sharma ◽  
Swati Parhar ◽  
...  
Keyword(s):  
Iron Overload ◽  
Prussian Blue ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
The Body ◽  
Transport Cost ◽  
Beta Thalassemia ◽  
Safe Procedure ◽  
Beta Thalassemia Major ◽  
Mucosal Cells

Background: Iron overload is a medical condition that occurs when too much of the mineral iron builds up inside the body and produces a toxic reaction. Thalassemia is a genetic disorder of hemoglobin synthesis, which requires regular blood transfusion therapy, and the lack of specific excretory pathways for iron in humans leads to iron overload in the body tissues. It is a major cause of morbidity and mortality in these patients. The estimation of iron levels in exfoliated buccal mucosal cells may provide a simple, noninvasive, and a safe procedure for estimating the iron overload by using the Perls’ Prussian blue stain. Methods: Smears were obtained from buccal mucosa of 40 randomly selected beta-thalassemia major patients and 40 healthy subjects as controls. Smears were stained with Perls’ Prussian blue method. Blood samples were taken for estimation of serum ferritin levels. Images of smears were analyzed using the software image J software version 1.47v and correlated with serum ferritin. Results: Perls’ positivity was observed in 87.5% of thalassemic patients with a positive correlation to serum ferritin levels. Conclusion: The use of exfoliative buccal mucosal cells for the evaluation of iron overloads in the body provides us with a diagnostic medium that is noninvasive, easy to collect, store, and transport, cost effective, and above all reliable.

Insilico identification of a ferroportin inhibitor for the management of iron-overload condition in Beta Thalassemia major patients.

2021 ◽  
Author(s):  
Yasir Sharif ◽  
Saba Irshad ◽  
Muhammad Hamza Tariq ◽  
Muhammad Nadeem Asghar
Keyword(s):  
Iron Overload ◽  
Therapeutic Potential ◽  
3D Structure ◽  
Thalassemia Major ◽  
The Body ◽  
In Vivo Studies ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major

Abstract The reason of high mortality rate in the patients of beta-thalassemia major is iron overload because it leads to many secondary complications. Condition of iron overload is known as hemochromatosis (HC). HC causes distorted formation of HFE protein that disturbs the whole pathway of HAMP protein synthesis which results in unbounded form of ferroportin and hence iron keeps absorbing in the body, leading to iron accumulation. The current study was conducted to identify a potential phytochemical that could bind to ferroportin and inhibits its iron absorbing activity within the body. The 3D structure of Ferroportin was unavailable in protein data bank PDB, therefore, it was developed by using different bioinformatics tools and best structure was identified through SAVES and RAMPAGE analysis. This best structure was docked with a library of 1010 bioactive phytochemicals by using MOE-2009 software. The top-ten ranked potential inhibitors were then evaluated for drug-like properties through molsoft and Molinspiration server followed by ADMET analysis. Our study demonstrated that “Taxifolin’ showed the maximum binding affinity with Ferroportin and also demonstrates maximum drug-like properties. Thus this compound could be used as a potential inhibitor of ferroportin. However, in-vitro and in-vivo studies must be conducted to validate the therapeutic potential of taxifolin against hemochromatosis.

scholarly journals Thyroid profile in patients of thalassemia with multiple blood transfusions and high serum ferritin: a cross-sectional study

2020 ◽  
Vol 7 (8) ◽  
pp. 401-408
Author(s):  
Dr. Rohit Khandelwal ◽  
◽  
Dr. Muralidhar Gundluru ◽  
Dr. Leeni Mehta K. ◽  
◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Thyroid Dysfunction ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
High Serum ◽  
The Body ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Beta Thalassemia Major ◽  
Tsh Levels

Introduction: Beta-thalassemia major patients undergo regular blood transfusion resulting ingrowth faltering and various endocrine problems including thyroid dysfunction due to iron overloadin the body. This study was conducted to determine the frequency of thyroid dysfunction in childrenpresenting with Beta-thalassemia major on regular blood transfusions. Materials and methods:Sixty children were included with proven beta-thalassemia major who reported to the Department ofPediatrics, VIMS, and RC, Bangalore. Inclusion criteria: 1.Children 4 to 18 years age group .2.Thechild received transfusions for more than 2 years. 3.Children with serum ferritin level >700.Results: In this study, four patients(6.8%) had overt hypothyroidism, eight patients(13.6%) hadsubclinical hypothyroidism and 47 patients(79.7%) had euthyroid status. There was a positivecorrelation between Ferritin and T4, TSH levels. i.e., with an increase in Ferritin level, there was anincrease in T4, TSH levels, and vice versa. However, the correlation was significant with TSH. Therewas a significant negative correlation between Ferritin and T3 levels. i.e with an increase in Ferritinlevel, there was a decrease in T3 levels and vice versa. Conclusion: Thyroid dysfunction can exist inthalassemia patients on multiple transfusions and chelation therapy with high serum ferritin levels.Detection of hypothyroidism is important as inexpensive oral replacement therapy is readilyavailable. Hence regular screening of beta-thalassemia major patients for Serum T3, Serum T4,Serum TSH for early detection and timely treatment could improve the life expectancy and quality oflife of 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

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 Thalassemia: With the “Red” in the Bag Amid COVID-19 Reflections

2020 ◽  
Vol 7 (4) ◽  
pp. 439-441
Author(s):  
Veeresh Pavate
Keyword(s):  
Chronic Condition ◽  
Patient Perspective ◽  
Present Situation ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Blood Disorder ◽  
Cooley's Anemia

This narrative piece written from a patient perspective angle who lives with a chronic condition—Beta thalassemia major (also known as Cooley’s Anemia), a serious, life-long, genetic blood disorder. The narrative portrays how the present situation of COVID-19 pandemic adds an extra layer to the challenges in the lives of people living with thalassemia.

scholarly journals Zinc levels in Beta-Thalassemia Major: A Review of the Literature

2021 ◽  
Vol 8 (6) ◽  
pp. 348-351
Author(s):  
Okan Dikker ◽  
Emine Türkkan ◽  
Nevin Çetin Dağ ◽  
Hüseyi̇n Dağ
Keyword(s):  
Trace Element ◽  
Serum Zinc ◽  
Thalassemia Major ◽  
The Body ◽  
Cellular Growth ◽  
Beta Thalassemia ◽  
Essential Trace Element ◽  
Inherited Disease ◽  
Beta Thalassemia Major ◽  
Zinc Levels

Objective: Zinc is an essential trace element for the body that is involved in various significant body functions such as protein synthesis, DNA synthesis, and cellular growth. It is found in almost every cell and plays an important role in the immune system, affecting both innate and acquired immunity. Patients with beta-thalassemia major are at risk of zinc deficiency. Beta-thalassemia major is an inherited disease caused by a reduction or complete absence of beta-globin chains and the affected patients need repeated blood transfusions to survive. Accordingly, it causes oxidative stress and tissue damage, alteration of antioxidant enzymes, and changes in other essential trace element levels due to iron overload. Zinc levels in beta-thalassemia major patients were reported to be significantly reduced in most of the studies. Serum zinc levels of the patients with beta-thalassemia major should be monitored regularly and zinc supplementation should be provided to these patients.

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