Thalassemia-A Blood Disorder, its Cause, Prevention and Management

Koneru, Prathyusha; Venkataswamy, Manjunatha M.; Goud, K. Sridivya; Ramanjaneyulu, K.; Himabindu, J.; Raj, K. Saikrupa

doi:10.5958/0975-4377.2019.00033.8

Cited by 16 publications

(9 citation statements)

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“…This study showed a positive correlation between hepcidin with Iron in G1. Patients with this disease require regular blood transfusions to manage their anemia, which can lead to iron overload in the body (41). In Beta-thalassemia Major, chronic transfusion therapy leads to increased iron absorption in the gut and deposition of iron in various organs of the body, including the liver, heart, and endocrine glands (42).…”

Section: The Resultsmentioning

confidence: 99%

Correlation of Hepcidin with Hemoglobin and Iron Parameters in Iraqi Patients with Beta -Thalassemia major

Nahi,

Jabir

2023

Qad Med J

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Background: Thalassemia is characterized by genetic abnormalities in the synthesis of hemoglobin, leading to a decrease or missing production of one or more in the globin chains. Consequently, this disrupts the synthesis of hemoglobin molecules, resulting in anemia, which is a prominent manifestation of thalassemia. Iron is an essential element for cellular health and is involved in various functions, including oxygen transportation, biomolecule synthesis, respiration, and homeostasis. Hepcidin, a low molecular weight peptide produced in the liver, plays a crucial role in regulating iron homeostasis. Objectives: Study the correlation of Hepcidin with Hemoglobin, Ferritin, and Iron Parameters in Patients with Beta-Thalassemia major. Methods: The serum ferritin of all subjects was measured by the ELFA technique (Enzyme Linked Fluorescent Assay), Serum Hepcidin is measured by the ELISA kit, and Iron, UIBC as well as TIBC are measured via colorimetric methods. Results: The { mean ± SD } of Ages and Genders among Patients with healthy groups were not significant. The { mean ± SD } of Hb and serum levels of Iron, UIBC, TIBC, Hepcidin concentration, and BMI between Patients and healthy groups were statistically significant. The study results of the correlation between Hepcidin and other markers in the beta-thalassemia patient's group showed a non-significant negative correlation of hepcidin with TIBC and UIBC. while there is a non-significant weak correlation between hepcidin with Hb and Ferritin. and the results have shown a non-significant positive correlation between hepcidin and Iron. Conclusion: In this study showed a marked reduction in hemoglobin production and high levels of Iron and Ferritin concentration, while the concentration of TIBC and UIBC was observed to decrease in individuals with this disease compared to healthy individuals. This study showed low hepcidin concentration in ?-TM major patients compared to healthy subjects.

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Section: The Resultsmentioning

confidence: 99%

Correlation of Hepcidin with Hemoglobin and Iron Parameters in Iraqi Patients with Beta -Thalassemia major

Nahi,

Jabir

2023

Qad Med J

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“…Hemoglobin production is either totally or partially retarded based on the severity and types of the disorder 3,10 . People suffering from thalassemia are unable to produce enough hemoglobin in the body which results in severe anemia 1,15 . The causes of thalassemia are the inheritance of abnormal and mutated genes involved in the production of hemoglobin from the parents.…”

Section: Introductionmentioning

confidence: 99%

“…Its clinical presentation occurs within 6 to 24 months of birth. Infants affected with Cooley's Anemia fail to grow and become gradually pale having more dreadful consequences 1 .…”

Section: Introductionmentioning

confidence: 99%

Scarcity of Awareness and Level of Misconception about Thalassemia around the Population of Kolkata and Neighbouring Places- A Preliminary Study

Majumdar

Mondal

Rudra

2023

clin. med. & helt. res. jour.

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Thalassemia is a genetic disorder caused by the inheritance of mutated and abnormal genes involved in the production of hemoglobin from the parents. Thalassemia is most seen in Mediterranean region, Middle East, South-East Asia, Africa, and Indian subcontinent. It is also known as The Mediterranean Disease. The study aims to focus on the awareness of thalassemia among the population of Kolkata, emphasizing on college and university going students. A questionnaire-based survey was conducted among 55 willing participants in a 6-week tenure, from June to July 2022. The study used data visualization method and descriptive statistics for understanding the level of awareness, perception about thalassemia among the participants. The mean knowledge percentage was 25.43% which was analyzed by calculating the points obtained by the participants allotted to each question, the point range was 2–22 in which only 6 i.e., 10.9% of people had an excellent knowledge of the disease. The points acquired ranged from 8–22 i.e., 4 to 11 questions, more than half of the participants (60%, 33) believe there is a cure for thalassemia and almost 44% (24) participants believe screening of thalassemia can be done only by blood tests. This work focuses on the scarcity of information and degree of misconception about thalassemia. It calls for an urgent, routine need of thalassemia awareness program in the population.

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“…The most common clinical phenotype is the homozygous form (HbSS or sickle cell anemia) [1]. Compound heterozygous SCD includes HbSC, HbSD, HbSO-Arab, and HbS/beta-thalassemia [10].…”

Section: Introductionmentioning

confidence: 99%

Assessment of serum electrolytes levels among sickle cell patients in South-Eastern Nigeria

Nnaji¹,

Oguji²,

Orji³

et al. 2022

World J. Adv. Res. Rev.

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Background: Sickle cell disease is a common genetic disorder mainly characterized by vascular occlusion and haemolysis and it constitutes a major public health problem. The aim of this study is to assess the serum electrolyte levels in sickle cell patients attending the University of Nigeria Teaching Hospital, Enugu. Materials and methodology: A total of 66 subjects, ages 18 – 40 years, were recruited for this study with thirty sickle cell subjects (female = 16, Male = 14) as test subjects and thirty non-sickle cell subjects (female = 21, male = 9) as control subjects. A total of 5mls of venous blood was collected from each subject. The serum obtained from each sample was analyzed for serum electrolytes (sodium, potassium, chloride, and bicarbonate) by potentiometry using a sensacore autoanalyzer. The data were analyzed using Statistical Package for the Social Sciences (SPSS) version 22. Results: Anthropometric results obtained from this study showed a significant decrease (P < 0.05) in mean ± SD of systolic blood pressure, diastolic blood pressure, and body mass index (BMI) in the test subjects compared to the control subjects respectively. The electrolyte analyses showed a significant increase (P < 0.05) in mean ± SD of potassium and chloride in the test subjects compared to the control subjects respectively. The control subjects have a non-significant increase (P > 0.05) mean ± SD of sodium compared to the sickle cell subjects while the sickle cell subject has a non-significant increase (P > 0.05) mean ± SD of bicarbonate compared to the control subjects. There was a positive correlation between sodium and bicarbonate, while in the other parameters; no significant correlation existed. Conclusion: This study concludes that sickle cell anaemia subjects are predisposed to electrolyte variations, hence, the need to check these electrolyte values in sickle cell subjects.

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Thalassemia-A Blood Disorder, its Cause, Prevention and Management

Cited by 16 publications

References 0 publications

Correlation of Hepcidin with Hemoglobin and Iron Parameters in Iraqi Patients with Beta -Thalassemia major

Correlation of Hepcidin with Hemoglobin and Iron Parameters in Iraqi Patients with Beta -Thalassemia major

Scarcity of Awareness and Level of Misconception about Thalassemia around the Population of Kolkata and Neighbouring Places- A Preliminary Study

Assessment of serum electrolytes levels among sickle cell patients in South-Eastern Nigeria

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