Detection of Renal Insufficiency in a Cohort of Patients With Beta-thalassemia Major Using Cystatin-C

Hamdy, Mahmoud; Shaheen, Iman A.; El-Gammal, Zinab M; Ramadan, Yasmin

doi:10.1097/mph.0000000000002171

Cited by 2 publications

(3 citation statements)

References 21 publications

(59 reference statements)

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“…*In G2, the results of the correlation showed a highly significant weak correlation of Hepcidin with Ferritin at (P ≤ 0.01). However there was no significant negative correlation between Hepcidin with Iron, and the results showed a non-significant weak correlation between Hepcidin and Hb, TIBC, and UIBC as in Figure (2).…”

Section: The Resultsmentioning

confidence: 91%

“…The Correlation of Hepcidin Levels with Some Biochemical Markers in G1 and G2 This correlation is illustrated in Table (2) where *in G1 the results of the correlation showed a non-significant negative correlation of Hepcidin with TIBC and UIBC. while there was a non-significant weak correlation between Hepcidin with Hb and Ferritin.…”

Section: The Resultsmentioning

confidence: 96%

“…Two alpha and two beta globin subunits make up this hemoglobin A. The genes that produce α-globin are located on chromosome 16, whereas the genes that produce β-globin are located on chromosome 11 (2). The most prevalent blood abnormality is β -thalassemia, which affects a large region that includes Melanesia, the Pacific Islands, Southeast Asia, the Indian subcontinent, the Middle East, portions of Africa, and the Mediterranean region.…”

Section: Introductionmentioning

confidence: 99%

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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: 91%

Section: The Resultsmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

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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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The Exploring of Growth Differentiation Factor-15 and H63D Gene Polymorphisms in β-thalassemia Major: Implications for Cardiovascular Risk and Iron Overload

Hamza,

Al-Ziaydi,

Alzamili

2024

Journal of Applied Hematology

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BACKGROUND: β-thalassemia major (βTM) is a genetic disorder characterized by a deficiency in hemoglobin production, ineffective erythropoiesis, chronic hemolysis, lifelong blood transfusions, iron overload, and increased risk of cardiac complications. OBJECTIVE: The study aimed to evaluate the growth differentiation factor-15 (GDF-15) concentration in βTM patients and its correlation with cardiac complications. H63D refers to a specific mutation in the HFE gene, which is associated with hereditary hemochromatosis (HH), a genetic disorder characterized by excessive accumulation of iron in the body. This mutation involves a change of histidine (H) to aspartic acid (D) at position 63 in the HFE protein. This mutation is often only written abbreviated as (H63D). MATERIALS AND METHODS: This case–control study was done on 120 subjects. A total of 60 patient samples were randomly collected from the Genetic Hematology Center at the Babylon Hospital, with an age range of 10–26 years. In addition, 60 samples were collected from healthy children in the same age range as the control group; patients and controls were subdivided into (10–18) and (18–26) year groups. GDF-15 was measured by enzyme-linked immunosorbent assay, and the genotyping of mutation was done by amplification refractory mutation system-polymerase chain reaction technique. RESULTS: The study revealed a significant increase in ferritin (FER) and GDF-15 levels in the patients compared to controls (P < 0.001). GDF-15 showed a direct correlation with age (r = 0.244, P = 0.02) and FER (r = 0.215, P = 0.04). There was a significant difference in H63D mutations between controls and patients (P = 0.044), with a higher proportion of the C-G (heterozygous for the mutant allele) genotype observed in βTM patients (31.67%). Additionally, a notable effect of the H63D mutation on serum ferritin (higher) levels within the βTM group was observed. CONCLUSION: Elevations of the GDF-15 in βTM patients indicate a high risk of cardiovascular complications in patients with βTM. The H63D mutation of the hemostatic iron regulator (HFE) gene is frequently found in βTM. Although a significant effect of the mutation was obtained on serum FER levels, it did not act as a risk factor in βTM patients. However, the frequent presence of the H63D mutation in patients indicated a possible association between single-nucleotide polymorphism and the iron regulation pathway.

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Detection of Renal Insufficiency in a Cohort of Patients With Beta-thalassemia Major Using Cystatin-C

Cited by 2 publications

References 21 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

The Exploring of Growth Differentiation Factor-15 and H63D Gene Polymorphisms in β-thalassemia Major: Implications for Cardiovascular Risk and Iron Overload

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