Study of Serum Hepcidin in Hereditary Hemolytic Anemias

Abstract Abstract 5168 Background: Thalassemia is the most common genetic disorder in Egypt composes a major health problem with an estimated carrier rate of 5. 3%-9%. Registered cases in large centers in Egypt September 2007 were 9912 cases, and in Cairo University hematology clinic were alone 2597 cases. Patients with thalassemia major requiring regular blood transfusions accumulate iron that is toxic to the heart, liver, and endocrine systems. Hepcidin, is a 25 amino-acid peptide hormone synthesized in the liver is a key regulator of iron homeostasis. Recently, hepcidin was reported to bind to the trans-membrane iron exporter “ferroportin” which is present on macrophages, the basolateral site of entrecotes, and in hepatocytes. Hepcidin induces the internalization and degradation of ferroportin. liver hepcidin controls reduction of iron uptake and release. There is also evidence for local production of hepcidin by macrophages, fat cells and cardiomyocytes. Thus, hepcidin is involved in different regulatory mechanisms that control iron imbalance. Objective: The aim of this study is to assess the level of serum hepcidin in hereditary chronic hemolytic anemias and correlate its level to the need for blood transfusion (frequency of blood transfusion) and the serum ferritin level. Study design: Seventy pediatric patients with hereditary chronic hemolytic anemias were the subjects of this study; 53 thalassemia major (TM), 10 thalassemia intermedia (TI), 4 congenital spherocytosis(CS) and 3 sickle cell disease(SCD) patients, mean age 7. 8+3. 9 years (range 1–14). Seventy normal children age and sex matched were studied as controls. Serum hepcidin was measured in all patients and controls by ELISA technique. Serum Hepcidin was measured in all patients one day pre transfusion and in 20 TM patients 3–4 days post transfusion. Results: Significant decrease in serum hepcidin levels in all patients compared to controls (mean 22. 9 ±6 vs 132. 4±16. 7 ng/ml, P <0. 001). Hepcidin levels were higher in TM (mean 23. 7±6. 2 ng/ml) than in TI patients (mean =21. 8±4 ng/ml) (Fig 1). The median number of blood transfusions in TM was 70/year (range18–120), in TI the median was 7/year (range 6–17). A 280% increase of serum hepcidin levels of pre transfusion levels was found post transfusion in TM patients (n=20). A significant positive correlation was found between serum hepcidin and frequency of blood transfusion (r=0. 4, p<0. 001), serum ferritin (r=0. 28, p <0. 05) and CRP (r=0. 4, p<0. 05). The hepcidin to ferritin ratio a marker of the hepcidin expression relative to the degree of iron burden was significantly less than one in TM and TI patients (0. 03± 0. 004 and 0. 025± 0. 002 respectively) and far from the level in normal controls (mean 2. 3±0. 7, P<0. 001). Hepcidin and hepcidin/ferritin ratio as markers of iron overload in our patients showed high sensitivity and specificity (99% and 98%, 97% respectively) (table 1, fig 2). Discussion and Conclusion: This study examined serum hepcidin and hepcidin/ferritin ratio in hereditary chronic hemolytic anemias, significant low levels were detected. The increase in serum hepcidin level in TM than TI and its marked increase post transfusion in TM patients can be explained by the positive correlation between frequency of blood transfusion and serum hepcidin level in this study. Hepcidin and hepcidin/ferritin ratio can be used as valid markers of iron overload in hereditary chronic hemolytic anemias. Evidence from laboratories around the world have converged on hepcidin as a rational therapeutic agent for treatment of B-thalassemia. Treatment with a hepcidin agonist, at a carefully defined dose, has the potential to ameliorate several aspects of TI due to the specific reduction of iron overload and splenomegaly. Testing this approach provides an exciting opportunity to improve the current management strategies for these diseases, and our study agrees with this approach. Disclosures: No relevant conflicts of interest to declare.

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Liver Enzymes in Children with beta-Thalassemia Major: Correlation with Iron Overload and Viral Hepatitis

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2015.059 ◽

2015 ◽

Vol 3 (2) ◽

pp. 287-292 ◽

Cited By ~ 7

Author(s):

Khaled M. Salama ◽

Ola M. Ibrahim ◽

Ahmed M. Kaddah ◽

Samia Boseila ◽

Leila Abu Ismail ◽

...

Keyword(s):

Blood Transfusion ◽

Iron Overload ◽

Viral Hepatitis ◽

Serum Ferritin ◽

Liver Enzymes ◽

Transferrin Saturation ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Elevated Liver Enzymes ◽

Hcv Antibody

BACKGROUND: Beta Thalassemia is the most common chronic hemolytic anemia in Egypt (85.1%) with an estimated carrier rate of 9-10.2%. Injury to the liver, whether acute or chronic, eventually results in an increase in serum concentrations of Alanine transaminase (ALT) and Aspartate transaminase (AST).AIM: Evaluating the potentiating effect of iron overload & viral hepatitis infection on the liver enzymes.PATIENTS AND METHODS: Eighty (80) thalassemia major patients were studied with respect to liver enzymes, ferritin, transferrin saturation, HBsAg, anti-HCV antibody and HCV-PCR for anti-HCV positive patients.RESULTS: Fifty % of the patients were anti-HCV positive and 55% of them were HCV-PCR positive. Patients with elevated ALT and AST levels had significantly higher mean serum ferritin than those with normal levels. Anti-HCV positive patients had higher mean serum ferritin, serum ALT, AST and GGT levels and higher age and duration of blood transfusion than the negative group. HCV-PCR positive patients had higher mean serum ferritin and serum ALT and also higher age and duration of blood transfusion than the negative group.CONCLUSION: Iron overload is a main leading cause of elevated liver enzymes, and presence of HCV infection is significantly related to the increased iron overload.

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Disease Specific Modulation of Serum Hepcidin: Impact of GDF-15 and Iron Metabolism Markers in Thalassemia Major, Thalassemia Intermedia and Sickle Cell Disease: A Univariate and Multivariate Analysis.

Blood ◽

10.1182/blood.v112.11.3850.3850 ◽

2008 ◽

Vol 112 (11) ◽

pp. 3850-3850 ◽

Cited By ~ 2

Author(s):

Farzana Sayani ◽

Sukhvinder Bansal ◽

Patricia Evans ◽

Aalim Weljie ◽

Robert C Hider ◽

...

Keyword(s):

Multivariate Analysis ◽

Iron Overload ◽

Serum Ferritin ◽

Serum Iron ◽

Transferrin Saturation ◽

Thalassemia Major ◽

Thalassemia Intermedia ◽

Iron Load ◽

Serum Hepcidin ◽

Disease Specific

Abstract Background. Factors that determine net synthesis of hepcidin and hence iron absorption and distribution depend on a balance of competing factors which may be disease specific. Such factors include anemia, ineffective erythropoiesis (IE), transferrin saturation (Tf sat), iron overload and inflammation. Recently GDF-15, a marker of erythroid maturation and hence IE, has been linked with depression of hepcidin synthesis in vitro and showed elevated levels in beta thalassemia (Tanno et al, Nat Med, 2007). The relationship of hepcidin synthesis to iron overload in sickle cell disease (SCD) is not clear and may differ from thalassemia syndromes because IE is less marked. We wished to establish whether the dominant factors determining net hepcidin synthesis differed between patients with SCD and those with thalassemia intermedia (TI) and thalassemia major (TM). Patients and methods. Serum hepcidin was measured in hypertransfused (Hb>9.5g/dl) patients with TM (n=18), untransfused or sporadically transfused patients with thalassemia intermedia TI (n=18), and multi-transfused patients with SCD (n=24), and related to markers of anemia, iron overload and erythroid expansion. A newly developed mass spectrometry assay (Bansal et al, Anal Biochem, 2008, In Press) was used to determine serum hepcidin. GDF-15 was measured by an ELISA assay. Multivariate analysis was performed using SIMCA-P software and partial least squares for discriminant analysis (PLS-DA), using samples from each of the clinical groups to investigate relationships between hepcidin, serum iron, non-transferrin bound iron (NTBI), transferrin saturation (Tf sat), serum ferritin, liver iron, transfusion history, erythropoietin, hemoglobin and GDF-15. Results. Serum hepcidin levels were higher in TM (13.9 ± 10.0 nmol/L) than SCD (8.51±8.16 nmol/L, p=0.043) whereas values in TI (3.82 ±3.56 nmol/L) were close to healthy controls (4.04 ± 2.06nmol/l). However, when SCD patients were matched for levels of anemia and iron load with TM, plasma hepcidin levels were similar or higher in SCD. GDF-15 values were highest for TI (11,444± 2177 ng/l), than TM (4117 ± 577 ng/l, P<0.001), whilst SCD patients had the lowest values (1227 ± 208 ng/l, P<0.001 vs TM). Univariate analysis in all patients grouped together showed positive correlations of hepcidin with serum ferritin (r=0.55, p <0.0001) and level of anemia (r=0.27, p= 0.045). Disease specific relationships were identified: negative correlations of serum hepcidin with Tf sat (r=−0.43) and NTBI (r=−0.45) were found for TI and TM but not in SCD, whereas ferritin showed a positive correlation in TM and SCD (r=0.51 and r= 0.56) but not in TI. GDF-15 correlated negatively with hepcidin in TI (r=0.51) but showed no relationship in SCD or TM. Positive correlations of GDF-15 with markers of plasma iron metabolism were seen in TI such as serum iron (r= 0.56), NTBI (r=0.45) and transferrin saturation (r=0.45). These were not seen in TM and tended to be negative relationships (r= −0.45, r= 0.25, r=0.59 respectively). In multivariate analysis, the variables responsible for the separation of the 3 patient groups clustered in 3 major categories including iron handling (serum iron, transferrin saturation, NTBI), ineffective erythropoiesis (GDF-15) and iron loading (ferritin, transfusion history). Hepcidin co-clustered with the iron loading group and was inversely correlated with GDF-15. Conclusion. Competing regulatory effects on hepcidin synthesis differ between TM, TI and SCD. In TI, hepcidin synthesis is suppressed by IE as shown by a dominant effect of GDF-15. In TM, GDF-15 effects on plasma hepcidin are less marked, as IE is lower due to hypertransfusion. This difference is particularly striking in patients at UCLH due to the divergent transfusion policies between TI and TM. The dominant modulating factors in TM are positive relationships to iron load (serum ferritin) but negative relationship with NTBI, serum iron and Tf saturation. However it is not yet clear whether the relationship of NTBI to hepcidin implies direct negative regulatory effect. In multi-transfused SCD patients, GDF-15 (IE) and NTBI have insignificant relationships to plasma hepcidin, with iron load (ferritin) showing the dominant effect: other effects in SCD such as those of chronic inflammation were not examined but require further investigation.

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Relation of Serum Ferritin Level with Serum Hepcidin and Fucose Levels in Children with β-Thalassemia Major

Hemoglobin ◽

10.1080/03630269.2021.1898419 ◽

2021 ◽

Vol 45 (1) ◽

pp. 69-73

Author(s):

Salah H. AL-Zuhairy ◽

Mohammed A. Darweesh ◽

Mohammed A-M. Othman

Keyword(s):

Serum Ferritin ◽

Serum Ferritin Level ◽

Ferritin Level ◽

Thalassemia Major ◽

Serum Hepcidin

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Left Atrial Strain Identifies Increased Atrial Ectopy in Patients with Beta-Thalassemia Major

Diagnostics ◽

10.3390/diagnostics11010001 ◽

2020 ◽

Vol 11 (1) ◽

pp. 1

Author(s):

Maria Vlachou ◽

Vasileios Kamperidis ◽

Efthymia Vlachaki ◽

Georgios Tziatzios ◽

Despoina Pantelidou ◽

...

Keyword(s):

Blood Transfusion ◽

Left Atrial ◽

Systolic Pressure ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Tape Recording ◽

Blood Transfusions ◽

Systolic Strain ◽

Peak Systolic Strain ◽

Beta Thalassemia Major

Patients with beta-thalassemia major (β-ΤΜ) may develop cardiac arrhythmias through a multifactorial mechanism. The current study evaluated the association of cardiac structure and function on echocardiography with atrial ectopic burden on 24-hour tape recording in β-ΤΜ patients. This prospective study included consecutive β-ΤΜ patients. Demographic, laboratory, echocardiographic, cardiac magnetic resonance (CMR) T2* and 24-hour tape recording data were prospectively collected. The patients were classified according to the median value of premature atrial contractions (PACs) on 24-hour tape. In total, 50 β-TM patients (37.6 ± 9.1 years old, 50% male) were divided in 2 groups; PACs ≤ 24/day and > 24/day. Patients with PACs > 24/day were treated with blood transfusion for a longer period of time (39.0 ± 8.6 vs. 32.0 ± 8.9 years, p < 0.007), compared to their counterparts. Older age (OR: 1.121, 95% CI: 1.032–1.217, p = 0.007), longer duration of blood transfusion (OR:1.101, 95% CI:1.019–1.188, p = 0.014), larger LV end-diastolic diameter (OR: 4.522, 95% CI:1.009–20.280, p = 0.049), higher values of LA peak systolic strain (OR: 0.869, 95% CI: 0.783–0.964, p = 0.008), higher MV E/E′ average (OR: 1.407, 95% CI: 1.028–1.926, p = 0.033) and higher right ventricular systolic pressure (OR: 1.147, 95% CI: 1.039–1.266, p = 0.006) were univariably associated with PACs > 24/day. LA peak systolic strain remained significantly associated with PACs > 24/day after adjusting for the duration of blood transfusions or for CMR T2*. The multivariable model including blood transfusion duration and LA peak systolic strain was the most closely associated with PACs > 24/day. Receiver operating characteristic curve analysis identified a left atrial peak systolic strain of 31.5%, as the best cut-off value (83% sensitivity, 68% specificity) for prediction of PACs > 24/day. In β-TM patients, LA peak systolic strain was associated with the atrial arrhythmia burden independently to the duration of blood transfusions and CMR T2*.

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Progressive dysfunction of monocytes associated with iron overload and age in patients with thalassemia major

Blood ◽

10.1182/blood.v67.1.105.bloodjournal671105 ◽

1986 ◽

Vol 67 (1) ◽

pp. 105-109

Author(s):

IJ Ballart ◽

ME Estevez ◽

L Sen ◽

RA Diez ◽

J Giuntoli ◽

...

Keyword(s):

Blood Transfusion ◽

Iron Overload ◽

Liver Damage ◽

Thalassemia Major ◽

Lytic Activity ◽

Blood Monocytes ◽

Peripheral Blood Monocytes ◽

Cell Dysfunction ◽

Positive Effect ◽

Increased Susceptibility

We evaluated phagocytic and lytic activities of peripheral blood monocytes (PBMo) from patients with thalassemia major (ThP) using C pseudotropicalis as the target. PBMo from ThP showed decreased lytic activity (P less than .001), whereas the phagocytic activity did not differ from that of the controls. Significant inverse correlations were found between lytic activity of PBMo and age of patients (r2 = .47; P less than .01) and also between lytic activity and serum ferritin levels (r2 = .65; P less than .001). No association was found between lytic activity and other variables (blood transfusion regimens, therapy with desferrioxamine, liver damage, and the presence of sHBAg). Splenectomy showed no positive effect on PBMo functions from ThP. Our results suggest that PBMo from ThP have an intracellular defect in their microbicidal mechanisms associated with iron overload. This cell dysfunction could be responsible, at least in part, for the increased susceptibility to infections reported in ThP.

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Testicular function in patients with regular blood transfusion for thalassemia major

Asian Biomedicine ◽

10.5372/1905-7415.0902.385 ◽

2015 ◽

Vol 9 (2) ◽

Cited By ~ 1

Author(s):

Sukumarn Siripunthana ◽

Taninee Sahakitrungruang ◽

Suttipong Wacharasindhu ◽

Darintr Sosothikul ◽

Vichit Supornsilchai

Keyword(s):

Blood Transfusion ◽

Chelation Therapy ◽

Cell Function ◽

Iron Chelation ◽

Thalassemia Major ◽

Delayed Puberty ◽

Control Group ◽

Blood Transfusions ◽

Testicular Function ◽

Iron Chelation Therapy

AbstractBackgroundRegular blood transfusion and iron chelation therapy have improved the quality of life of patients with thalassemia and increased their longevity, but transfusion also increases the frequency of endocrine complications, possibly because of iron deposition in the pituitary gland or the gonads, or both.ObjectiveTo evaluate testicular function in patients with thalassemia major by basal hormonal study, and identify risk factors for dysfunction.MethodsWe performed a cross-sectional study of 28 patients with thalassemia major aged 11.7 ± 1.8 (8–14.9) years (15 in prepuberty, 13 in puberty with no delayed puberty) who had regular blood transfusions. A normal control group comprised 64 boys who were matched for age and Tanner genital stage.ResultsThe mean level of serum ferritin in the previous year was 1,575 ± 642 ng/mL, and the onset of blood transfusion was at 3.8 ± 2.3 years and iron chelation therapy was 6.6 ± 2.8 years. The trend for anti-Müllerian hormone levels in patients and controls was similar with age, and although higher in the patients, particularly at Tanner stage II, was not significantly different. Testosterone levels were lower in the patients compared with controls; particularly at Tanner stages IV–V (290.88 vs. 537.4 ng/dL,ConclusionPatients who received regular blood transfusions had normal Sertoli cell function. Leydig cell dysfunction may occur, even though the patients had a normal pubertal onset.

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Prevalence of Transfusion Transmissible Infections in Beta-Thalassemia Major Patients of Pakistan: A Systematic Review

Blood ◽

10.1182/blood-2020-138911 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 23-25

Author(s):

Hamid Ehsan ◽

Ahsan Wahab ◽

Muhammad Ammar Shafqat ◽

Muhammad Salman Faisal ◽

Ahmad Muneeb ◽

...

Keyword(s):

Systematic Review ◽

General Population ◽

Blood Transfusion ◽

Low Income ◽

Age Groups ◽

Thalassemia Major ◽

Beta Thalassemia ◽

Blood Transfusions ◽

Medical Database ◽

Qualitative Synthesis

Introduction: β-thalassemia major (TM) is one of the most prevalent inherited hemoglobinopathies in Pakistan. It has one of the highest prevalence of transfusion-dependent TM patients globally, with an estimated greater than 100,000 active cases. Each year, an estimated 5000-9000 new cases of TM are being diagnosed in the country. Blood transfusions (BT) are essential in the management of severe TM; it is critical to have a safe BT to reduce the risk of transfusion transmissible infections (TTIs). Frequent blood transfusions in these patients increase their risk of acquiring TTIs compared to the general population. In this systematic review, we aimed to identify the prevalence of TTIs in transfusion-dependent β -thalassemia major patients in Pakistan. Methods & Material: We performed a systematic literature search to identify studies related to the TTIs and transfusion-related infections in Pakistan from January 1, 2010, to January 31, 2020. The search was conducted using PubMed and PakMediNet (Largest medical database of Pakistan), with initial search retrieved 981 studies. Among these, 166 studies met the inclusion criteria. After further screening by reviewing the articles for relevance and availability of full-length articles, only 14 studies met the final criteria for qualitative synthesis. Results Analysis of 14 studies (n=3786) showed that the seroprevalence of Hepatitis B virus (HBV) of 3.13% (0.66 % to 7.4%) and Hepatitis C virus (HCV) of 26 % (5.56% to 68.2%). There were only two studies reported HIV seroprevalence of 0 % & 0.5% (n=6). The rate of seropositivity for HBV and HCV was directly related to the number of transfusions, higher ferritin levels, and older age groups. There was an increase in the HCV rate with the increasing age of patients. Thalassemia patients who were older than ten years of age had a greater HCV compared to those who were less than ten years of age, i.e., 22% vs. 8.4%, p:0.005, respectively. The mean age was higher in HCV reactive children than non-reactive children. A comparison of HCV in healthy donors vs. thalassemia patients showed a rate of 1.9% vs. 13.1% for T.M. patients. There was HCV infection rate of 74% in the group with greater than 100 BTs compared to 33 % in a group with fewer than 35 BTs. The rate of HCV increased to 75% for the patients who had more than 100 BTs. The majority of the patients were males (51% to 88%). The seroprevalence of TTIs was higher in males than in females (73.4% vs. 26.6%). On average, a single TM patient is exposed to at least 17 different donors annually, requiring 1-2 transfusions every month. The free BT is accessible only in 1 out of 4 thalassemia centers. The majority of patients either need to bring their donors or are dependent on an external source of financial aid as they could not afford the cost of BT treatment. More than half of thalassemia patients (57.2%) need to contact multiple BT centers to search for required blood products. About 42.1% of parents of TM patients did not know about TTIs, whereas 31.6% of them did not know about the bloodborne transmission of HBV and HCV. The majority of parents of TM children had a low income, with 75% of them having income less than 10,000 Pakistani rupees (PKR) per month. The prevalence of TTIs in TM patients was significantly higher (96% vs. 4%) compared to the patients requiring multiple transfusions due to other causes such as leukemia, aplastic anemia, and thrombocytopenia. Conclusion: Our data highlights that the prevalence of transfusion-transmitted infections, especially HCV, is alarmingly higher (26%) in the TM population than in the general population. This is because of a lack of resources, inadequate safety measures, and a fragmented blood transfusion system. These findings warrant the urgent need for better public health measures, safe blood transfusion practices, voluntary remunerated blood-based transfusions, and universal quality-assured donor screening. Without these positive interventions, the current transfusion system can lead to a further worsening of the situation. Large prospective multi-centered clinical trials are required to understand better the high prevalence of TTIs in patients with TM. Disclosures Anwer: Incyte, Seattle Genetics, Acetylon Pharmaceuticals, AbbVie Pharma, Astellas Pharma, Celegene, Millennium Pharmaceuticals.:Honoraria, Research Funding, Speakers Bureau.

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Extreme hyperferritinaemia; clinical causes

Journal of Clinical Pathology ◽

10.1136/jclinpath-2012-201090 ◽

2013 ◽

Vol 66 (5) ◽

pp. 438-440 ◽

Cited By ~ 8

Author(s):

Martin A Crook ◽

Patrick L C Walker

Keyword(s):

Liver Disease ◽

Iron Overload ◽

Serum Ferritin ◽

Malignant Disease ◽

Hepatic Disease ◽

Blood Transfusions ◽

Still’S Disease ◽

Ferritin Concentration ◽

Serum Ferritin Concentration ◽

Multiple Blood

There are many causes of raised serum ferritin concentrations including iron overload, inflammation and liver disease to name but a few examples. Cases of extreme hyperferritinaemia (serum ferritin concentration equal to or greater than 10 000 ug/l) are being reported in laboratories but the causes of this are unclear. We conducted an audit study to explore this further. Extreme hyperferritinaemia was rare with only 0.08% of ferritin requests displaying this. The main causes of extreme hyperferritinaemia included multiple blood transfusions, malignant disease, hepatic disease and suspected Still's disease.

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Thyroid Function in Chronically Transfused Children with Beta Thalassemia Major: A Cross-Sectional Hospital Based Study

International Journal of Pediatrics ◽

10.1155/2018/9071213 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 4

Author(s):

Suraj Haridas Upadya ◽

M. S. Rukmini ◽

Sowmya Sundararajan ◽

B. Shantharam Baliga ◽

Nutan Kamath

Keyword(s):

Thyroid Function ◽

Serum Ferritin ◽

Subclinical Hypothyroidism ◽

Thyroid Dysfunction ◽

Genetic Disorder ◽

Thalassemia Major ◽

Beta Thalassemia ◽

P Value ◽

Blood Transfusions ◽

Beta Thalassemia Major

Background. Thalassemia is the most common genetic disorder worldwide. Use of iron chelators has improved survival but endocrine complications have become more frequent. The frequency of hypothyroidism in Beta Thalassemia Major (BTM) children ranges from 6 to 30 %. Thyroid dysfunction mainly occurs by gland infiltration, chronic tissue hypoxia, free radical injury, and organ siderosis. Objectives. (a) To evaluate the thyroid function status in chronically transfused children with BTM, in the first and second decade of life and (b) to study the influence of factors like duration and amount of blood transfusions, serum ferritin level, and iron chelation therapy on thyroid function. Methodology. BTM children, 3 years old and above, on regular blood transfusions with serum ferritin > 1500 mcg/l were included in the study. Thyroid function and ferritin assessment was done using ELISA kits. Autoimmune thyroiditis was ruled out by antithyroid peroxidase and antithyroglobulin antibody testing. Results. A study population of 83 children consisted of 49 boys (59%) and 34 girls (41%). 4.8% of the children had evidence of subclinical hypothyroidism. Among them two belonged to the first decade and the other two to the second decade of life. Mean TSH, FT4, and ferritin values among children with thyroid dysfunction were 6.38 ± 0.83 mIU/ml, 1.08 ± 0.45 ng/dl, and 3983.0±1698.30 ng/ml, respectively. The severity of thyroid dysfunction was statistically significantly associated with higher serum TSH values in children in the second decade of life with a p value = 0.001. No other significant correlation was found between oral chelation, amount and duration of blood transfusion, or serum ferritin levels. Conclusion. Subclinical hypothyroidism was the thyroid dysfunction observed in our study. Regular blood transfusions with adequate chelation may decrease incidence of thyroid dysfunction.

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