Neurocognitive Function and Its Related Potentials in Children with Beta Thalassemia Major: An Egyptian Study

BACKGROUND: Repeated blood transfusions and hemolysis in β-Thalassemia major children lead to iron overload in various organs, including the brain which may cause neurodegeneration. AIM: To evaluate intelligence quotient in children with β-thalassemia major and healthy counterparts and to assess risk factors that cause cognitive problems. SUBJECTS AND METHODS: This case-control study was performed on 50 children aged 6-16 years old with β-thalassemia major as patients group and compared with 50 healthy children as a control group of matched age, sex, and social class. Cognitive functions were evaluated by using the Wechsler Intelligence Scale for Children. Serum ferritin and iron were measured by ELISA. RESULTS: There were significantly lower mean performance and full-scale IQ scores of patients group in comparison with controls, whereas no significant differences between both groups as regards to a verbal IQ score. In thalassemic children, block design, comprehension and arithmetic were negatively correlated with age of disease onset, duration of illness and onset of chelation therapy. Serum iron and ferritin were negatively correlated with similarities and digit span. Serum iron levels were negatively correlated with performance IQ score. CONCLUSION: Children with β-thalassemia major need to receive more academic attention and cognitive assessment to improve their IQ.

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Zinc Status in Beta-Thalassemia Major

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2021.5688 ◽

2021 ◽

Vol 9 (B) ◽

pp. 149-153

Author(s):

Khalaf Hussein Hasan ◽

Hasan Abdulla Aswad ◽

Aspazija Sofijanova

Keyword(s):

Serum Ferritin ◽

Ferritin Level ◽

Disease Onset ◽

Serum Zinc ◽

Thalassemia Major ◽

Adverse Outcomes ◽

Beta Thalassemia ◽

Control Group ◽

Healthy Children ◽

Zinc Status

BACKGROUND: Zinc is one of the most important minerals incorporated in the enzymes of the human body. Zinc may be deficient in patients with the β-thalassemia major with possible adverse outcomes. AIM: The purpose of this study was to assess the serum zinc status in β-thalassemia major patients in Duhok city. PATIENTS, MATERIAL, AND METHODS: In this case–control study, 70 children with β-thalassemia major (2–12 years) of both genders were enrolled and were matched with 70 apparently healthy children for age and sex. A venous blood sample was obtained from each child for the measurement of serum zinc and serum ferritin levels at Jin Center in Duhok City between January 1 and June 30, 2017. RESULTS: The mean serum zinc in the thalassemia patients (74.79 [±25.14] μg/dl) was significantly lower compared to the control group (93.61 [±15.12] μg/dl), (p = 0.0001). The serum zinc was not significantly different in thalassemia patients in terms of age, disease onset, gender, height, weight, body mass index, amount of blood transfusion, and type of chelation. There was a statistically significant correlation between serum zinc levels with a serum ferritin level of patients. CONCLUSION: The study showed that thalassemia patients have significantly lower serum levels of zinc with no relation to medical factors.

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Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children ◽

10.3390/children8020100 ◽

2021 ◽

Vol 8 (2) ◽

pp. 100

Author(s):

Asmaa A. Mahmoud ◽

Doaa M. Elian ◽

Nahla MS. Abd El Hady ◽

Heba M. Abdallah ◽

Shimaa Abdelsattar ◽

...

Keyword(s):

Cystatin C ◽

Kidney Injury ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Good Survival ◽

Control Group ◽

Healthy Children ◽

Serum Cystatin C ◽

Serum Cystatin ◽

Beta Thalassemia Major

Background: A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children. Methods: This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted. Conclusion: Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.

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SALIVA IRON AND FERRITIN LEVELS IN PATIENTS WITH THALASSEMIA AND IRON DEFICIENCY ANEMIA

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2012.051 ◽

2012 ◽

Vol 4 (1) ◽

pp. e2012051 ◽

Cited By ~ 9

Author(s):

Duran Canatan ◽

Sevgi Kosaci Akdeniz

Keyword(s):

Iron Deficiency ◽

Iron Deficiency Anemia ◽

Serum Iron ◽

Iron Concentration ◽

Thalassemia Major ◽

The Body ◽

Deficiency Anemia ◽

Control Group ◽

Healthy Children ◽

Positive Correlation

Most of the techniques for measuring iron accumulation such as serum iron concentration, iron binding capacity, serum ferritin level, liver biopsy are invasive and hard methods for patients. The changes in trace element concentrations in saliva at different systemic diseases shows the quantity of the element at the body. The aim of this study was to compare the levels of iron and ferritin in saliva and serum in patients with thalassemia and iron deficiency anemia. For this purpose, 35 healthy children as control group and 71 thalassemia major, 10 thalassemia intermedia and 15 thalassemia trait patients were involved. Their saliva and serum iron and ferritin levels were measured. There was no statistically difference between age and gender in all groups and control group (p>0.05). In all groups saliva iron levels are higher than serum iron levels(p<0.05). Furthermore there was a positive correlation betwen serum and saliva iron levels in thalassemia major, intermedia and trait groups ( p=0.000, r=0.972, r=0.720, r=0.955) and also there was a positive correlation between serum and saliva iron levels in control and iron deficiency group (p= 0.000, r= 0.885, r= 0.368). In conclusion, Saliva iron and ferritin levels increase as well as serum in patients with thalassemia and decrease in patients with iron deficiency anemia. Saliva can be used for diagnosis routinely to shows the iron overload and deficiency of the body and its easy applicability and also a non-invasive procedure is important advantage.

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Relationship between Vitamin B9 (Folic Acid), Vitamin B12 (Cobalamin), and Peripheral Neuropathy in Children with Beta-Thalassemia Major

Global Medical & Health Communication (GMHC) ◽

10.29313/gmhc.v9i2.8106 ◽

2021 ◽

Vol 9 (2) ◽

Author(s):

Uni Gamayani ◽

Titin Junaidi ◽

Nushrotul Lailiyya ◽

Nur Suryawan ◽

Nanan Sekarwana

Keyword(s):

Folic Acid ◽

Peripheral Neuropathy ◽

Vitamin B12 ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Control Group ◽

Case Group ◽

Beta Thalassemia Major ◽

Vitamin B9 ◽

Significant Difference

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.

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THERAPEUTIC VALUE OF COMBINED THERAPY WITH DEFERASIROX AND SILYMARIN ON IRON OVERLOAD IN CHILDREN WITH BETA THALASSEMIA

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2013.065 ◽

2013 ◽

Vol 5 (1) ◽

pp. e2013065 ◽

Cited By ~ 14

Author(s):

Adel Abd elhaleim Hagag

Keyword(s):

Iron Overload ◽

Serum Ferritin ◽

Iron Status ◽

Iron Chelator ◽

University Hospital ◽

Beta Thalassemia ◽

Control Group ◽

Healthy Children ◽

Number Of Patients

abstractBackground: Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring life-long blood transfusion that cause iron overload. Silymarin plays a role as oral iron chelator and hepatoprotective agents in thalassemic patients.The aim of this work was to determine silymarin value as an iron chelator in thalassemic patients with iron overload.Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital having serum ferritin level more than 1000 ng/ml and was divided in two groups. Group IA: Received oral Deferasirox (Exjade) and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade) and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this research. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001). Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: Extensive multicenter studies in large number of patients with longer duration of follow up and more advanced methods of assessment of iron status is recommended to clarify the exact role of silymarin in reduction of iron over load in children with beta thalassemia.

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Pancreatic functions in adolescents with beta thalassemia major could predict cardiac and hepatic iron loading: relation to T2-star (T2*) magnetic resonance imaging

Journal of Investigative Medicine ◽

10.1136/jim-2015-000031 ◽

2016 ◽

Vol 64 (3) ◽

pp. 771-781 ◽

Cited By ~ 2

Author(s):

Galila M Mokhtar ◽

Wafaa E Ibrahim ◽

Nancy S Elbarbary ◽

Randa M Matter ◽

Ahmed S Ibrahim ◽

...

Keyword(s):

Serum Amylase ◽

Thalassemia Major ◽

Tolerance Test ◽

Beta Thalassemia ◽

Control Group ◽

Oral Glucose ◽

Hepatic Iron ◽

Mri Findings ◽

Heavy Load ◽

T2 Mri

The aim of this study is to assess the correlation between cardiac and hepatic T2* MRI findings with the endocrine and exocrine pancreatic functions in known patients with β-thalassaemia major (β-TM). A total of 50 adolescent patients with β-TM and 44 healthy controls were investigated via: serum amylase, lipase, triglyceride index, oral glucose tolerance test and T2* MRI, to assess iron content in the heart and liver. Diabetes was found in 20%, and 40% of patients had impaired fasting glucose (IFG). Cardiac T2* was less than 10 ms in 22% indicating heavy load with iron in cardiac tissues. There was a significant decrease in median serum amylase (63.5 vs 87.5 IU/L, p=0.003) and lipase (63 vs 90 IU/L, p=0.017) among patients in comparison with the control group. Patients with β-TM and diabetes had lower serum amylase (32 vs 68 IU/L), lipase (28 vs 79 IU/L), cardiac and hepatic T2* MRI (7 vs 25.5 ms; 3 vs 6 ms, p<0.001 for all) than those without diabetes. Similar results were found among patients with IFG when compared with others (p<0.001 for all). Cardiac and hepatic T2* were inversely correlated to triglyceride index (r=−0.376, p=0.014 and r=−0.475, p=0.001, respectively) and positively correlated to amylase (r=0.791 and r=0.790) and lipase (r=0.784 and r=0.783; p<0.001 for all). The endocrine and exocrine pancreatic functions might become an equivalent predictor to cardiac and hepatic iron overload, especially in countries where MRI is not available or where it is expensive. The early occurrence of these abnormalities warrants more intensive chelation therapy.

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Targeting TMPRSS6 Using Antisense Technology for the Treatment of Beta-Thalassemia

Blood ◽

10.1182/blood.v126.23.753.753 ◽

2015 ◽

Vol 126 (23) ◽

pp. 753-753 ◽

Cited By ~ 2

Author(s):

Shuling Guo ◽

Mariam Aghajan ◽

Carla Casu ◽

Sara Gardenghi ◽

Sheri Booten ◽

...

Keyword(s):

Mouse Model ◽

Targeted Delivery ◽

Serum Iron ◽

Iron Concentration ◽

Transferrin Saturation ◽

Beta Thalassemia ◽

Control Group ◽

Mrna Levels ◽

Antisense Technology ◽

Wide Range

Abstract Antisense technology is a powerful drug discovery approach for identifying oligonucleotide analogs that can specifically modify RNA expression through multiple mechanisms including RNase H-mediated degradation of RNA and modulation of RNA splicing. We have successfully applied this technology towards targeting a number of transcripts in a wide-range of therapeutic areas. Beta-thalassemia, one of the most common genetic disorders worldwide, is characterized by reductions in beta-globin and ineffective erythropoiesis. This in turn leads to suppression of hepcidin, a peptide hormone that serves as the master regulator of iron homeostasis. Inappropriately low levels of hepcidin trigger increased dietary iron absorption resulting in iron overload, which is the major cause of morbidity and mortality in beta-thalassemia patients. TMPRSS6 is a transmembrane serine protease mainly produced by hepatocytes that negatively regulates hepcidin expression. Previous mouse and human genetic data from multiple groups suggest that lowering TMPRSS6 expression could up-regulate hepcidin and ameliorate many of the disease symptoms associated with β-thalassemia. We identified potent antisense oligonucleotides (ASOs) against mouse TMPRSS6. Downregulation of TMPRSS6 with ASO treatment resulted in dose-dependent hepcidin upregulation and reduction in serum iron and transferrin saturation in normal mice. In a mouse model of beta-thalassemia (th3/+ mice), which effectively recapitulates beta-thalassemia intermedia in humans, TMPRSS6 reduction resulted in induction of hepcidin and dramatic reductions of serum transferrin saturation (from 55-63% in control group down to 20-26% in treatment group). Liver iron concentration (LIC) was also greatly reduced (40-50%). Moreover, anemia endpoints were significantly improved with ASO treatment, including increases in red blood cells (~30-40%), hemoglobin (~2 g/dl), and hematocrit (~20%); reduction of splenomegaly; decreases in serum erythropoietin levels; improved erythroid maturation as indicated by a strong reduction in reticulocyte number and a normalized proportion between the pool of erythroblasts and enucleated erythroid cells. Encouraged by the strong pharmacology of TMPRSS6 suppression in animal models, we initiated an effort to identify a human TMPRSS6 clinical candidate with a liver-targeted delivery strategy. Over 2000 ASOs were screened in cell lines and the most active compounds were evaluated in rodent tolerability studies. A human TMPRSS6 transgenic mouse model was established enabling evaluation of ASO activity toward human TMPRSS6 transcript in vivo. Furthermore, lead compounds were tested in a 3-month study in normal monkeys. With repeated dosing, TMPRSS6 mRNA levels in monkey liver were reduced by >90%, accompanied by time-dependent reductions of serum iron (from ~100-120ug/dl to <40ug/dl), transferrin saturation (from ~30-35% to <10%), and hemoglobin. These compounds were well tolerated in rodents and in monkeys. Collectively, our data demonstrate that TMPRSS6 ASO could be an effective therapeutic for patients with beta-thalassemia and related disorders. A Phase 1 clinical trial is planned to initiate in 2016. Disclosures Guo: Isis Pharmaceuticals: Employment, Other: Shareholder. Aghajan:Isis Pharmaceuticals: Employment, Other: Shareholder. Booten:Isis Pharmaceuticals: Employment, Other: Shareholder. Monia:Isis Pharmaceuticals: Employment, Other: Shareholder.

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Nutritional Biomarkers in Children and Adolescents with Beta-Thalassemia-Major: An Egyptian Center Experience

BioMed Research International ◽

10.1155/2014/261761 ◽

2014 ◽

Vol 2014 ◽

pp. 1-7 ◽

Cited By ~ 22

Author(s):

Laila M. Sherief ◽

Sanaa M. Abd El-Salam ◽

Naglaa M. Kamal ◽

Osama El safy ◽

Mohamed A. A. Almalky ◽

...

Keyword(s):

Trace Elements ◽

Folic Acid ◽

Serum Zinc ◽

Serum Levels ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Healthy Children ◽

Multicenter Studies ◽

Thalassemic Patient ◽

Beta Thalassemia Major

Background and Aim. Trace elements and vitamins play a vital role in human body to perform its function properly. Thalassemic patients are at risk of micronutrient deficiency. This study estimated levels of vitamins A, C, E, B12, folic acid, total homocysteine (tHcy), and methylmalonic acid (MMA) along with trace elements, zinc, copper, and selenium in Beta-thalassemia-major patients.Methods. This study included 108 patients with Beta-thalassemia-major and 60 age and sex matched healthy children. Serum levels of vitamin A, E, C, tHcy, and MMA were estimated by high pressure liquid chromatography while serum levels of folic acid and B12 were estimated by thin layer chromatography. Serum zinc, copper, and selenium were determined by atomic absorption spectrometry.Results. There was a significant decrease of vitamins A, C, E, and B12 and trace elements zinc, copper, and selenium in thalassemic patients as compared to controls. tHcy and MMA were significantly elevated in patients. No significant correlations were found between the serum levels of the studied vitamins and trace elements as regards age, frequency of transfusion, duration of transfusion, and serum ferritin.Conclusion. The level of various nutritional biomarkers (vitamins A, C, E, and B12 and trace elements zinc, copper, selenium) was reduced in chronically transfused Egyptian thalassemic patient. These patients should have periodic nutritional evaluation and supplementation. Multicenter studies are highly recommended.

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Influence of Working Memory on Academic Achievement and Quality of Life in Children with Beta-Thalassemia Major

The Open Psychology Journal ◽

10.2174/1874350101912010076 ◽

2019 ◽

Vol 12 (1) ◽

pp. 76-83 ◽

Cited By ~ 1

Author(s):

Uni Gamayani ◽

Ni Luh Meidha Dini Lestari ◽

Ahmad Rizal Ganiem ◽

Ramdan Panigoro

Keyword(s):

Quality Of Life ◽

Academic Achievement ◽

Working Memory ◽

Digit Span ◽

Ferritin Level ◽

Memory Function ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Beta Thalassemia Major

Background: Children with beta-thalassemia major may suffer from working memory impairment. For a more refined understanding of this issue, we assessed working memory function in beta-thalassemia children and evaluated its influence on academic achievement and quality of life. Methods: This was a cross-sectional study involving 60 beta-thalassemia children aged 8-12 years. All participants underwent a working memory assessment using the digit span and were interviewed using academic achievement and Indonesian version of PedsQL 4.0 questionnaires. Working memory in beta-thalassemia children significantly influences their quality of life, both directly (β=0.32) and indirectly, through their academic achievement (β=0.639). Longer duration of transfusion (p=0.01) is significantly related to poorer working memory, while lower hemoglobin level (p=0.81) and higher ferritin level (p=0.24) are not significantly associated with working memory. Conclusion: We concluded that working memory influences the quality of life in beta-thalassemia children, both directly and indirectly, through their academic achievement.

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DISTURBANCE OF IRON METABOLISM AND ULTRASTRUCTURAL FEATURES OF THE GINGIVA IN PATIENTS WITH Β-THALASSEMIA MAJOR

Российский стоматологический журнал ◽

10.18821/1728-2802-2018-22-5-255-261 ◽

2018 ◽

Vol 22 (5) ◽

pp. 255-261

Author(s):

Ramida Vaqif qizi Shadlinskaya ◽

E. K Gasimov ◽

S. A Israfilova

Keyword(s):

Iron Metabolism ◽

Serum Iron ◽

Binding Capacity ◽

Periodontal Diseases ◽

Thalassemia Major ◽

Control Group ◽

Periodontal Tissues ◽

Metabolism Disorder ◽

Pathogenetic Factor ◽

Iron Binding Capacity

Introduction: β-thalassemia major is a hereditary blood disease, a main pathogenetic factor of which is the disruption of the synthesis of β-chains of hemoglobin. Inevitable metabolic disorders that occur during regular blood transfusion form an additional risk of damage and cellular modification of organs and tissues, including the oral cavity. Aim: determination of the pathogenetic role of iron metabolism disorder in the development of chronic inflammatory periodontal diseases in patients with β-thalassemia. Material and methods: 12 patients with β - thalassemia major who had periodontal diseases were examined in the Republican Center of Thalassemia in Baku (average age is 18 ± 1.3 years). The control group consisted of 16 somatically healthy individuals with intact gingiva tissues (the average age is 18.0 ± 0.7 years). Serum Iron, ferritin, hepcidin, total iron-binding capacity (TIBC), level of Il2, Il6, Il10, TNFα were measured from peripheral blood samples. To determine the ultra-structural study of the periodontal tissues in patients with β-thalassemia major, the method of electron microscopy studies of gingiva biopsies was used. Results of the study: The study revealed an increase in the level of serum iron and ferritin against the background of a progressively decreasing TIBC in patients with β-thalassemia major. Also activation of pro-inflammatory cytokines - TNFα, Il6, Il10 was observed in homozygous patients. Metabolic indicators are compared with ultrastructural features of cellular elements of a gum. Signs of accumulation of iron in cellular elements both of its own plate and of the epithelial cover were compared. The presence of iron was recorded in immunocompetent cells - macrophages and lymphocytes. Conclusion: Summarizing the data obtained, we can conclude that deproteinization of ferritin cores and their detection both in the cytoplasm and in the nucleoplasm of immunocompotent cells leads to irreversible degenerative changes in cellular elements of the gingiva. The authors link the development of the generalized inflammatory - degenerative process in the periodontium with revealed exchange -structural violations.

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