Assessment of the Effectiveness of Self-Instructional Module on Knowledge Regarding Home Care Management of Thalassemia Children among the Parents in A.V.B.R. Hospital, Wardha

Background of Study: Thalassemia is a genetic condition in which the production of globin chains is reduced or non-existent. Beta-thalassemia is caused by mutations in the beta-globin gene, which impede the formation of beta-globin chains. Based on clinical severity, beta thalassemia is categorised as follows. The most severe form of beta thalassemia is characterised by severe anaemia and the requirement for blood transfusions. Anaemia caused by beta thalassemia intermedia can be treated with medication or transfusions. Beta thalassemia minima- Also known as beta thalassemia trait, this type is typically asymptomatic. Children with beta-thalassemia major require regular blood transfusions, which can result in issues such as iron overload and the transfer of illnesses such as HIV, HCV, and HBsAg, all of which can shorten their life. Inadequate transfusions, on the other hand, result in severe anaemia as well as fatigue and debility. Objectives: To measure parents' existing knowledge of thalassemia children's home care management. To analyses the efficiency of a self-instructional module on thalassemia children's home care management expertise. To link the post-test knowledge score to the demographic factors chosen. Materials and Methods: A descriptive research design study was conducted to evaluate the efficiency of a self-instructional module on parental understanding of thalassemia children's home care management. The total number of samples in this study was 30. All of them were parents of thalassemia children from A.V.B.R. Hospital in Sawangi (Meghe), Wardha. The study employed a non-probability convenient sampling strategy, with the instrument being designed questionnaires on parental understanding of their children's medical conditions and knowledge of home care management of thalassemia. Based on the aims and hypothesis, the data was statistically analysed using various tests such as descriptive statistics and inferential statistics. Results: The purpose of this study was to determine the efficiency of a self-instructional module on parental understanding of thalassemia children's home care management. Following the installation of the seif-instructional module, 63.33 percent of Thalassemia parents had a poor level of knowledge and 36.67 percent had an average level of knowledge prior to the exam, according to analysis. The lowest knowledge score on the pre-test was 2 and the highest knowledge score was 7. The pre-test mean percentage of knowledge score was 33.559.17, and the pre-test mean knowledge score was 5.031.37. Post-test knowledge scores were average for 23.33 percent of Thalassemia parents and excellent for 76.67 percent of Thalassemia parents. The minimum post-test knowledge score was 8 and the maximum post-test knowledge score was 14. The mean post-test knowledge score was 11.901.84, and the mean percentage of knowledge score was 79.3312.29. Conclusion: The current study's pre-test results reveal that parents of thalassemia children had insufficient information about thalassemia home care management. Following the self-instructional module, it was a modest attempt to develop understanding of thalassemia home care management. Based on their pre-test scores, 19% of subjects had low knowledge, 11% had moderate knowledge, and 0% had strong knowledge, according to the findings. However, according to post-test results, 0% of subjects had low knowledge, 7% of subjects had average knowledge, and 23% of subjects had strong knowledge. As a result, the post-test knowledge score was statistically interpreted to be greater than the pre-test knowledge score.

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Globin-chain specificity of oxidation-induced changes in red blood cell membrane properties

Blood ◽

10.1182/blood.v79.6.1586.1586 ◽

1992 ◽

Vol 79 (6) ◽

pp. 1586-1592 ◽

Cited By ~ 32

Author(s):

SL Schrier ◽

N Mohandas

Keyword(s):

Membrane Damage ◽

Membrane Skeleton ◽

Material Behavior ◽

Membrane Properties ◽

Beta Thalassemia ◽

Beta Globin ◽

Globin Chain ◽

Globin Chains ◽

Alpha Globin

Abstract We have previously shown that excess unpaired alpha- and beta-globin chains in severe alpha- and beta-thalassemia interacting with the membrane skeleton induce different changes in membrane properties of red blood cells (RBCs) in these two phenotypes. We suggest that these differences in membrane material behavior may reflect the specificity of the membrane damage induced by alpha- and beta-globin chains. To further explore this hypothesis, we sought in vitro models that induce similar membrane alterations in normal RBCs. We found that treatment of normal RBCs with phenylhydrazine produced rigid and mechanically unstable membranes in conjunction with selective association of oxidized alpha-globin chains with the membrane skeleton, features characteristic of RBCs in severe beta-thalassemia. Methylhydrazine, in contrast, induced selective association of oxidized beta-globin chains with the membrane skeleton and produced rigid but hyperstable membranes, features that mimicked those of RBCs in severe alpha- thalassemia. These findings suggest that consequences of oxidation induced by globin chains are quite specific in that those agents that cause alpha-globin chain accumulation at the membrane produce rigid but mechanically unstable membranes, whereas membrane accumulation of beta- globin chains results in rigid but mechanically stable membranes. These in vitro experiments lend further support to the hypothesis that membrane-associated alpha- and beta-chains induce oxidative damage to highly specific different skeletal components and that the specificity of this skeletal damage accounts for the differences in material membrane properties of these oxidatively attacked RBCs and perhaps of alpha- and beta-thalassemic RBCs as well.

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Effectiveness of Self Instructional Module (Sim) on Knowledge Regarding Alzheimer's Disease

Journal of Universal College of Medical Sciences ◽

10.3126/jucms.v4i2.19088 ◽

2018 ◽

Vol 4 (2) ◽

pp. 25-29

Author(s):

Nira Neupane ◽

M. Vijaya lakshmi

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Student Nurses ◽

Knowledge Score ◽

Nursing Management ◽

Adequate Knowledge ◽

Instructional Module ◽

Post Test ◽

Level Of Knowledge ◽

Test Level

INTRODUCTION: Alzheimer's disease is a progressive neurodegenerative disease that causes severe deterioration of functional and cognitive abilities. As the leading cause of dementia in adults, it affects one in every 10 people who are more than 65 years of age. There is a vital need for student nurses with knowledge of Alzheimer's disease to provide high quality care for the growing number of patients. The use of effective nursing interventions for the management of Alzheimer's disease can help student nurses promote independence and quality of life for Alzheimer's disease patients. Thus this study is under taken to assess the effectiveness of the self instructional module (SIM) on nursing management of client with Alzheimer's disease among 4th year B. Sc. (N) students.MATERIALS AND METHODS: A quantitative evaluative research approach with pre experimental one group pre test and post test research design & non probability purposive sampling technique was used to select the 60 samples of B.Sc. (N) 4th year students. Self administered structured knowledge questionnaire were used to collect the information from the study samples. The prepared tool was validated by the experts and the reliability was established. The study was conducted in R.V. College of Nursing and Vidyakirana Institute of Nursing Sciences, Bangalore. The data was analyzed by descriptive and inferential statistics using SPSS version 19.RESULTS: Findings of the study shown that, the overall mean percentage pre test level of knowledge of B.Sc. Nursing 4th year studentswas 62.08%, and the pre test knowledge score was 35(58.33%) respondents were having moderately adequate knowledge, 13(21.67%) had inadequate knowledge and 12(20%) were having adequate knowledge regarding nursing management of clients with Alzheimer's disease. In the post test overall mean percentage post test level of knowledge was 84.37%. The post test knowledge score was 54(90%) respondents were having adequate knowledge and 06(10%) had moderately adequate knowledge after the administration of SIM. The overall obtained ''t'' value of knowledge 15.6 is highly significant at P< 0.01 level. The obtained chi-square value showed significant association between the pre test level of knowledge and certain socio demographical variables; gender (χ2 =5.12, df =1, p<0.05) and sources of information (χ2 =4.82, df=1, p<0.05). However, it did not show any significant association with other demographic variables of 4th Year B. Sc. (N) students.CONCLUSION: Findings of the study showed that majority of the student nurses were moderately adequate knowledge before administering the SIM. The SIM facilitated them to gain more knowledge regarding nursing management of client's with Alzheimer's disease which was evident in post test knowledge scores. Hence SIM was an effective strategy for providing information and to improve knowledge of students' nurses.Journal of Universal College of Medical Sciences (2016)Vol.04 No.02 Issue 14, page: 25-29

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Globin-chain specificity of oxidation-induced changes in red blood cell membrane properties

Blood ◽

10.1182/blood.v79.6.1586.bloodjournal7961586 ◽

1992 ◽

Vol 79 (6) ◽

pp. 1586-1592 ◽

Cited By ~ 1

Author(s):

SL Schrier ◽

N Mohandas

Keyword(s):

Membrane Damage ◽

Membrane Skeleton ◽

Material Behavior ◽

Membrane Properties ◽

Beta Thalassemia ◽

Beta Globin ◽

Globin Chain ◽

Globin Chains ◽

Alpha Globin

We have previously shown that excess unpaired alpha- and beta-globin chains in severe alpha- and beta-thalassemia interacting with the membrane skeleton induce different changes in membrane properties of red blood cells (RBCs) in these two phenotypes. We suggest that these differences in membrane material behavior may reflect the specificity of the membrane damage induced by alpha- and beta-globin chains. To further explore this hypothesis, we sought in vitro models that induce similar membrane alterations in normal RBCs. We found that treatment of normal RBCs with phenylhydrazine produced rigid and mechanically unstable membranes in conjunction with selective association of oxidized alpha-globin chains with the membrane skeleton, features characteristic of RBCs in severe beta-thalassemia. Methylhydrazine, in contrast, induced selective association of oxidized beta-globin chains with the membrane skeleton and produced rigid but hyperstable membranes, features that mimicked those of RBCs in severe alpha- thalassemia. These findings suggest that consequences of oxidation induced by globin chains are quite specific in that those agents that cause alpha-globin chain accumulation at the membrane produce rigid but mechanically unstable membranes, whereas membrane accumulation of beta- globin chains results in rigid but mechanically stable membranes. These in vitro experiments lend further support to the hypothesis that membrane-associated alpha- and beta-chains induce oxidative damage to highly specific different skeletal components and that the specificity of this skeletal damage accounts for the differences in material membrane properties of these oxidatively attacked RBCs and perhaps of alpha- and beta-thalassemic RBCs as well.

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Gene Therapy for Beta-Thalassemia: Preclinical Studies on Human Cells.

Blood ◽

10.1182/blood.v108.11.5473.5473 ◽

2006 ◽

Vol 108 (11) ◽

pp. 5473-5473

Author(s):

Giuliana Ferrari ◽

Emanuela Roselli ◽

Francesca Tiboni ◽

Erica Biral ◽

Sarah Marktel ◽

...

Keyword(s):

Gene Therapy ◽

Genetically Modified ◽

Beta Thalassemia ◽

Beta Globin ◽

Hematopoietic Stem ◽

Molecular Defects ◽

Globin Chains ◽

Number Of Patients ◽

Cd34 Cells ◽

The Impact

Abstract Gene therapy for beta-thalassemia is based on the transplantation of genetically-modified autologous hematopoietic stem cells (HSC) into patients affected by the severe form of disease. The genetic treatment of the hemoglobinopathies poses the general challenge of efficient level of gene transfer into HSC and high and persistent transgene expression, in the differentiated progeny of a genetically modified stem cell. The validation of a gene therapy approach to thalassemia requires to obtain results of gene correction in a broad number of patients’ cells, since different molecular defects in the beta-globin gene lead to the clinical phenotype. The heterogeneity in the molecular defects and in the proportion of alpha and non-alpha (beta, gamma and delta) chains will represent a key element to set a threshold in the amount of vector-derived beta-chain required to correct a thalassemic phenotype. Additionally, the impact of some biological parameters, such as the degree of BM erythroid hyperplasia, the BM subpopulations proportion and the apoptotic index, on the successful correction of thalassemic phenotype needs to be studied in the perspective of clinical translation. In order to address these issues, we collected samples from BM aspirates and isolated CD34+ cells from 25 beta+ and beta0 thalassemic patients, characterized by different genotypes and biochemical profiles of globin chains synthesis. A novel, erythroid specific LV expressing human beta-globin from a minimal promoter enhanced by only 2 LCR elements (HS2 and HS3) was used to transduce BM derived CD34+ cells at high efficiency (>80%). The efficacy of the beta-globin LV in correcting the human thalassemic phenotype was tested in an in vitro model of erythropoiesis and in the human-mouse hematological chimera. Upon transduction, normal level of HbA expression was achieved in erythroblastic cultures and BFU-E, associated with a progression towards erythroid maturation, which was impaired in mock-transduced thalassemic cells. Molecular analysis showed proviral integrity, with no detectable rearrangements and an average proviral copy number of 2.4. Analysis of specific globin chains proportion and contribution to phenotype correction in the context of different genotypes is under evaluation.

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The instability of the membrane skeleton in thalassemic red blood cells

Blood ◽

10.1182/blood.v86.10.3945.bloodjournal86103945 ◽

1995 ◽

Vol 86 (10) ◽

pp. 3945-3950 ◽

Cited By ~ 34

Author(s):

J Yuan ◽

A Bunyaratvej ◽

S Fucharoen ◽

C Fung ◽

E Shinar ◽

...

Keyword(s):

Red Blood Cells ◽

Blood Cells ◽

Polyacrylamide Gel Electrophoresis ◽

Sodium Dodecyl ◽

Membrane Skeleton ◽

Beta Thalassemia ◽

Enzyme Linked Immunosorbent Assay ◽

Beta Globin ◽

Partial Explanation ◽

Globin Chains

The thalassemias are a heterogeneous group of disorders characterized by accumulation either of unmatched alpha or beta globin chains. These in turn cause the intramedullary and peripheral hemolysis that leads to varying anemia. A partial explanation for the hemolysis came our of our studies on material properties that showed that beta-thalassemia (beta- thal) intermedia ghosts were very rigid but unstable. A clue to this instability came from the observation that the spectrin/band 3 ratio was low in red blood cells (RBCs) of splenectomized beta-thal intermedia patients. The possible explanations for the apparent decrease in spectrin content included deficient or defective spectrin synthesis in thalassemic erythroid precursors or globin chain-induced membrane changes that lead to spectrin dissociation from the membrane during ghost preparation. To explore the latter alternative, samples from different thalassemic variants were obtained, ie, beta-thal intermedia, HbE/beta-thal, HbH (alpha-thal-1/alpha-thal-2), HbH/Constant Spring (CS), and homozygous HbCS/CS. We searched for the presence of spectrin in the first lysate of the standard ghost preparation. Normal individuals and patients with autoimmune hemolytic anemia, sickle cell anemia, and anemia due to chemotherapy served as controls. Using gradient sodium dodecyl sulfate-polyacrylamide gel electrophoresis analysis, no spectrin was detected in identical aliquots of the supernatants of normals and these control samples. Varying amounts of spectrin were detected in the first lysate supernatants of almost all thalassemic patients. The identification of spectrin was confirmed by Western blotting using an affinity-purified, monospecific, rabbit polyclonal antispectrin antibody. Relative amounts of spectrin detected were as follows in decreasing order: splenectomized beta-thal intermedia including HbE/beta-thal; HbCS/CS; nonsplenectomized beta-thal intermedia, HbH/CS; and, lastly, HbH. These findings were generally confirmed when we used an enzyme-linked immunosorbent assay technique to measure spectrin in the first lysate. Subsequent analyses showed that small amounts of actin and band 4.1 also appeared in lysates of thalassemic RBCs. Therefore, the three major membrane skeletal proteins are, to a varying degree, unstably attached in severe thalassemia. From these studies we could postulate that membrane association of abnormal or partially oxidized alpha- globin chains has a more deleterious effect on the membrane skeleton than do beta-globin chains.

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Cellular and membrane properties of alpha and beta thalassemic erythrocytes are different: implication for differences in clinical manifestations

Blood ◽

10.1182/blood.v74.6.2194.bloodjournal7462194 ◽

1989 ◽

Vol 74 (6) ◽

pp. 2194-2202 ◽

Cited By ~ 1

Author(s):

SL Schrier ◽

E Rachmilewitz ◽

N Mohandas

Keyword(s):

Cell Density ◽

Mechanical Stability ◽

Membrane Properties ◽

Beta Thalassemia ◽

Normal Density ◽

Beta Globin ◽

Globin Chains ◽

Alpha Thalassemia ◽

Dynamic Rigidity ◽

Membrane Skeletons

To define how excess unpaired alpha- and beta-globin chains in severe beta-thalassemia and severe alpha-thalassemia interacting with the membrane might alter cellular and membrane properties, we performed a series of biophysical and biochemical analyses on erythrocytes obtained from affected patients. Detailed analysis of cellular and membrane deformability characteristics showed that both forms of thalassemic erythrocytes have excess surface area in relation to cell volume and increased membrane dynamic rigidity. The deformability characteristics of thalassemic erythrocytes in hypertonic media differed significantly from that of normal erythrocytes of identical cell density. These findings suggest that dynamic rigidity of thalassemic erythrocytes is influenced not only by cytoplasmic viscosity determined by cell hemoglobin concentration but also by the extent and type of globin interacting with the membrane. In contrast to the above-noted similarities, major differences were noted in the mechanical stability of the alpha- and beta-thalassemic membranes and in their state of cell hydration. While the mechanical stability of alpha-thalassemic membranes was normal or marginally elevated, the stability of beta- thalassemic membranes was markedly decreased to half the normal value. Cell-density analysis showed that the alpha-thalassemic erythrocytes were uniformly less dense than normal, while beta-thalassemic erythrocytes had a broad-density distribution, with all populations having both lower and higher than normal density values, implying cellular dehydration in beta-thalassemia and not in alpha-thalassemia. Membrane-protein analysis revealed that excess globin chains were bound to the membrane skeletons of both alpha- and beta-thalassemic erythrocytes, with the highest amounts being found in membrane skeletons derived from erythrocytes of splenectomized individuals with beta-thalassemia intermedia. These data demonstrate that interaction of excess alpha- and beta-globin chains with membranes produces different cellular changes and suggest that the observed differences in the pathophysiology of alpha- and beta-thalassemias may be related to different cellular effects induced by the excess in beta- and alpha- globin chains.

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Prediction of RNA Secondary Structure at IVS1 Mutation of Beta Globin Gene

International Journal of ChemTech Research ◽

10.20902/ijctr.2019.130131 ◽

2020 ◽

Vol 13 (1) ◽

pp. 247-252

Author(s):

Nur Imaniati Sumantri ◽

Dian Rachma Wijayanti

Keyword(s):

Secondary Structure ◽

Rna Secondary Structure ◽

Globin Gene ◽

Regulatory Elements ◽

Beta Thalassemia ◽

Globin Genes ◽

Beta Globin ◽

Beta Globin Gene ◽

Globin Chains ◽

Intronic Mutation

Background: Beta globin gene is responsible for producing beta globin chains that stabilize the structure and function of hemoglobin. This gene expression is controlled by complex interactions of transcriptions factors and its regulatory elements in a specific manner. Disturbed beta globin genes may result in hemoglobinopathies, mainly sickle cell disease and beta thalassemia. It seems interesting that several mutations occurring in intronic region results in severe symptoms to beta thalassemia patients, such an IVS1nt5 G>C. This research aimed to analyze RNA structural alteration effected by intronic mutation of beta thalassemia. Methods: The most prevalent mutation of beta thalassemia in Indonesia was obtained from Ithanet. The RNA secondary structure of IVS1nt5 G>C and beta globin gen (HBB) wildtype were performed by RNAStructure, along with probknot prediction. Results: The result showed that intronic mutation caused conformational change in beta globin secondary structure, either for max expect or base pairing probability approach. The mutant had bigger and more loops that diminished the protein stability. Thus, the structure might undergo dysfunction. Conclusion: The comprehensive structural-functional significance of these findings needs further study.

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Nonsense codon mutations in the terminal exon of the beta-globin gene are not associated with a reduction in beta-mRNA accumulation: a mechanism for the phenotype of dominant beta-thalassemia

Blood ◽

10.1182/blood.v83.8.2031.bloodjournal8382031 ◽

1994 ◽

Vol 83 (8) ◽

pp. 2031-2037 ◽

Cited By ~ 4

Author(s):

GW Hall ◽

S Thein

Keyword(s):

Globin Gene ◽

Beta Thalassemia ◽

Beta Globin ◽

Terminal Exon ◽

Mrna Accumulation ◽

Beta Globin Gene ◽

Globin Chains ◽

Nonsense Codon ◽

Nonsense Codons

We present in vivo evidence that there is no reduction in beta-mRNA accumulation in patients with nonsense codons in the terminal exon of the beta-globin gene. Using reverse transcriptase/polymerase chain reaction (RT-PCR), beta-globin cDNA was isolated from the reticulocytes of individuals heterozygous for nonsense codon mutations in exons II and III of the beta-globin gene. Clinically asymptomatic individuals heterozygous for mutations causing premature termination of translation in exon II [beta(0)39(C-T) and F/S71/72(+A)] were found to have almost no mutant beta-cDNA, whereas patients with nonsense codon mutations in exon III [beta 121(G-T) and beta 127(C-T)] with the clinical phenotype of thalassemia intermedia had comparable levels of mutant and normal beta-cDNA. Translation of the mutant beta-mRNA from patients with nonsense codon mutations in exon III would give rise to truncated beta- globin chains, which could explain the more severe phenotype seen in these individuals.

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EFFICACY OF SELF-INSTRUCTIONAL MODULE ON KNOWLEDGE REGARDING HOME CARE MANAGEMENT OF CHEMOTHERAPY AMONG CANCER PATIENTS

10.36106/paripex/7804123 ◽

2020 ◽

pp. 1-2

Author(s):

Arti Mishra ◽

Rajni Thakur

Keyword(s):

Home Care ◽

Cancer Patients ◽

Cause Of Death ◽

Care Management ◽

Knowledge Score ◽

Posttest Score ◽

Pretest Score ◽

The Mean ◽

Wide Gap ◽

Knowledge And Practice

Cancer is one of the most common leading cause of death worldwide. There will be 1.5 million new cases of cancer acquiring in the united state every year. The result of the study shows that a wide gap between knowledge and practice in home care while discharge of patient after chemotherapy. Result showed that the mean posttest knowledge score (21.54) is apparently higher than the mean pretest knowledge score (10.03). the dispersion of pretest score (S.D. ± 2.101) is more than that of their posttest score (S.D. ± 0.901) and the computed ‘t’ value shows that there is a highly significant differences between pretest and posttest mean knowledge score (t 39 = 41.74, p≤ 0.05 level).

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To Assess the Effectiveness of Self-Instructional Module on Knowledge Regarding Selection of Play Articles for Children and Its Importance among Parents of 6-12 Years Children

Journal of Pharmaceutical Research International ◽

10.9734/jpri/2021/v33i46a32853 ◽

2021 ◽

pp. 161-168

Author(s):

Pritam Meshram ◽

Deeplata Mendhe

Keyword(s):

Cognitive Learning ◽

Knowledge Score ◽

Future Generation ◽

Good Level ◽

The Future ◽

Instructional Module ◽

Post Test ◽

Test Population ◽

Level Of Knowledge ◽

Selection Of

Introduction: Kids are humanity's hope. The children of today are the masters of the world of the future. The saying goes, "Health is richness." Therefore, if children are happy, they will be healthier for the future generation, a healthy nation's result. Play articles are more than just playing things, but while they should be enjoyable, they should also be children's age-appropriate physical and emotional growth. It is necessary to consider playing items or playing articles as a cognitive learning tool for children. Objective: Assess the knowledge and effectiveness of the self-instructional module regarding the selection of play articles for children and its importance among parents of 6-12 years of children. Associate the post-test knowledge score with selected demographic variables among parents of 6-12 years of children. Methods: Study research design is a pre-experimental one-group pre-test post-test. Population in this study parent of 6-12 years children selected area of Wardha city with 80 parents to use close-ended structured questionnaires have been used in this study for assessing knowledge. Results: The finding of the study shows that the post-test, the sample was had a poor level of knowledge score, none of them had a poor level of knowledge score, none of them had an average level of knowledge, none of them had a good level of knowledge score, 52 (78.75%) had an excellent level of knowledge score and 28 (21.25%) have a very good level of knowledge, The minimum score was 13 and the maximum score was 19, the mean score was 16.79 ± 1.290 with a mean percentage score of 55.96%. Conclusion: The conclusion of this study shows that 28 (21.25%) having a very good level of knowledge and 52(78.75%) having an excellent level of knowledge.

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