scholarly journals PREVALENCE OF HYPOTHYROIDISM, DELAYED PUBERTY AND DIABETES MELLITUS IN PATIENTS OF Β-THALASSEMIA MAJOR

2020 ◽  
Vol 4 (3) ◽  
Author(s):  
Naresh Manne ◽  
Bharat Kumar Gupta ◽  
Sandeep Kumar Yadav ◽  
Saurabh Singhal ◽  
Archana Dubey
Keyword(s):  
Diabetes Mellitus ◽  
Blood Transfusion ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Delayed Puberty ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Beta Thalassemia Major

Background: Beta-Thalassemia is a genetic disorder which is associated with a lot of complications. Frequent blood transfusions result in increased iron deposition in various tissues leading to dysfunction of many vital organs. Endocrine disorders constitute a major part of such complications increasing the morbidity of thalassemia manifold in the affected patients. Methods:  This is a descriptive cross sectional study carried out in 100 diagnosed patients of beta- thalassemia major who had visited the OPD/IPD of Subharti Medical College & affiliated Hospitals, Meerut for routine blood transfusion or for any other complication. Patients were clinically examined and investigated for presence of one or more endocrine disorders on their routine appointments. Results: Endocrine disorders were detected in a total of 82 patients. Diabetes mellitus was detected in 12% patients, hypothyroidism in 36% patients and delayed puberty was found in 72% patients. Mean serum ferritin level was found to be 5831.0±2860.5 ng/ml in beta-thalassemia Major patients, while it was in normal range in control subjects. Conclusion: Research concluded with finding of Delayed puberty (72%), Hypothyroidism (36%) and diabetes mellitus as (12%) in beta thalassemia patients who were on regular blood transfusion therapy.  Iron overload as serum ferritin level was found to be highly raised in all study case. On the basis of our study we recommend that early detection and management protocols for these endocrinopathies may improve the life prospects of beta-thalassemia Major patients. Keywords: Endocrine disorders, Hypothyroidism, Delayed puberty, Diabetes Mellitus Serum ferritin, Thalassemia Major.

Association of Body mass index and serum ferritin level in pediatrics with Beta-thalassemia major disease

2022 ◽  
Author(s):  
Saeed Yousefian ◽  
Ghassem Miri Aliabad ◽  
Rana Saleh ◽  
Majid Khedmati
Keyword(s):  
Body Mass Index ◽  
Blood Transfusion ◽  
Body Mass ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Major Disease ◽  
Beta Thalassemia Major

Background: Beta-thalassemia major is a type of inherited blood disease that results in variable outcomes such as severe anemia due to haemoglobin chains. Recurrent and lifelong blood transfusions as a treatment in beta-thalassemia major disease lead to iron deposition in various organs and cause the failure of multiple organs. Failure of affected organs leads to Body mass index (BMI) abnormality. This study aimed to evaluate the association between BMI and serum ferritin level as a marker for iron overload. Materials and Methods: A cross-sectional study designed and conducted with total number of 740 paediatrics, with mean age about 14.2±8.7 years old and with beta-thalassemia major requiring recurrent blood transfusion. Patient information, including demographics, serum ferritin level and percentage of BMI, was recorded and analysed by SPSS 25.0 and the statistical significant level, considered as 0.05.    Results: A total number of 740 paediatrics with beta-thalassemia major disease (mean age about 14.2±8.7 years) were included to study to examine the association between serum ferritin level and their BMI.  The total mean serum level of ferritin calculated about 3326 ± 3859 Nanogram/mililitter (ng/ml). Totally, 447 (60.4%) case of them had BMI percentile less than 5%, 274 (37.02%), 16 (2.16%) and 3 (0.4%) had BMI percentile 5%-85%, 85%-95% and more than 95%. There was no relation between gender and serum ferritin levels. The relationship between age and BMI has been positive (P=0.002). Finally, it resulted that there was a negative relationship between the BMI percentile and mean serum ferritin levels in paediatrics with beta-thalassemia major (P=0.031). Conclusion: Frequent Blood transfusion is associated with elevated serum ferritin level in paediatrics with beta-thalassemia major disease and experiencing lower percentiles of BMI in these patients.

Investigation of correlation between H63D and C282Y mutations in HFE gene and serum Ferritin level in beta-thalassemia major patients

2019 ◽  
Vol 26 (4) ◽  
pp. 249-252
Author(s):  
Romina Rahmani ◽  
Parisa Naseri ◽  
Ava Safaroghli-Azar ◽  
Shahriar Tarighi ◽  
Tahereh hosseini ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Hfe Gene ◽  
Beta Thalassemia Major

scholarly journals BETA THALASSEMIA MAJOR;

2017 ◽  
Vol 24 (02) ◽  
pp. 315-321
Author(s):  
Asma Mehreen ◽  
Saeeda Bano ◽  
Bushra Ujala
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Controlled Trial ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Baseline Serum ◽  
Beta Thalassemia Major ◽  
Group A ◽  
Group B

Introduction: β-Thalassemia major (β-TM) is a genetic haemoglobin disorderwhich is relatively common in some geographical areas. β-TM is characterized by severe anaemia,which needs a continuous blood transfusion regimen starting from the first months of life toprolong survival. Objectives: To compare mean reduction in serum ferritin level with deferasiroxand desferrioxamine when used as an iron chelator in multi-transfused beta thalassemia major.Study design: Randomized controlled trial. Setting: Thalassemia clinic, The Children’s Hospitaland The Institute of Child Health, Lahore. Duration of Study with Dates: Study was carried outover a period of nine months from 28-06-2015 to 27-03-2016. Subjects and Methods: A totalof 100 patients (50 patients in each group). The patients were randomly allocated into twogroups using random numbers stable. Group-A received Deferasirox and group-B receivedDesferrioxamine. Results: Mean age of the patients was 7.42±4.13 and 7.87±4.13 in group-Aand B, respectively. Regarding sex distribution, 26 patients (52.0%) in group-A and 28 patients(56.0%) in group-B were male while 24 patients (48.0%) in group-A and 22 patients (44.0%) ingroup-B were female. Reduction from baseline in group-A was 783.60±413.66 ng/ml and ingroup-B 552.80±155.45 ng/ml (P<0.001) There was more reduction in group-A. In group-Abaseline serum ferritin level was 2495.00±1259.10 ng/ml and at 9 month 1712.00±1019.36 ng/ml (P<0.001). Similarly in group-B baseline serum ferritin level was 2422.80±910.43 ng/ml andat 9 month 1883±862.72 ng/ml (P<0.001). Conclusion: In conclusion, deferasirox was moreeffective in terms of reduction in serum ferritin level when compared with desferrioxamine inmulti-transfused beta thalassemia major patients.

scholarly journals Correlation between serum ferritin level, cardiac and hepatic T2-star MRI in patients with β-thalassemia major in Al-Diwaniya thalassemia center

2018 ◽  
Vol 9 (SPL1) ◽  
Author(s):  
Alaa Mutter Jabur Al-Shibany ◽  
AalanHadi AL-Zamili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Iron Level ◽  
The Mean ◽  
T2 Mri

Patients with transfusion dependent thalassemia major is often associated with iron overload. Proper use of iron chelators to treat iron overload requires an accurate measurement of iron levels. Magnetic resonance T2-star (T2* MRI) is the preferred method to measure iron level in the liver andthe heart. The goal of our study was to see if there is an association exists between serum ferritin level and T2* MRI results in patients with beta thalassemia major.This study was done in Al-Diwaniya Thalassemia center,Maternity and children teaching hospital,Iraq. During the period from 1st of January to 31st of October. Fifty eight patients with a diagnosis of beta thalassemia major were enrolled in the study. They were older than five years old,transfusion dependent and on chelation therapy. Hepatic and Myocardial T2*MRI and the mean serum ferritin levels were measured during the study period for all patients.There is a significant correlation was observed between serum ferritin level and cardiac T2*MRI (p=0.018 ). also a significant correlation was observed between serum ferritin and hepatic T2*MRI (p=0.02). Neither cardiac T2* MRI nor hepatic T2* MRI show any correlation with the mean age.our study also showa positive correlation between the patients withcardiac T2* MRI and the development of diabetes mellitus in contrast to hepatic T2* MRI in which there is no any correlation. Hypothyroidism was observedno correlation with either cardiac or hepatic T2* MRI.Our results showed a positiveassociation between hepatic, cardiac T2*MRI and serum ferritin levels.

scholarly journals Left ventricular dysfunction in transfusion dependent beta Thalassemia major patients in Bangladesh

2016 ◽  
Vol 9 (1) ◽  
pp. 31-35
Author(s):  
Simu Saha ◽  
Tapash Saha ◽  
AKM Amirul Morshed ◽  
Md Lutful Ehsan Fatmi ◽  
Nazneen Umme Zakia ◽  
...  
Keyword(s):  
Diastolic Dysfunction ◽  
Serum Ferritin ◽  
Cardiac Dysfunction ◽  
Serum Ferritin Level ◽  
Ventricular Dysfunction ◽  
Early Stage ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Beta Thalassemia

Background: Thalassemia major is an inherited haemoglobin disorder resulting in chronic haemolytic anaemia. Patients with beta thalassemia major are maintained on continuous blood transfusion regimens resulting in iron overload that adversely affects both the structure and function of the heart and other vital organs which can be easily prevented with iron chelating therapy. The aim of the study was to detect left ventricular dysfunction at an early stage so that early effective intervention can be done.Methods: A total of 50 patients with beta thalassemia were included in the study by non randomized qualitative purposive sampling from July 2013 to June 2014. Their total body iron status was be assessed by doing serum ferritin level. Left ventricular systolic and diastolic function was assessed by echocardiographyResults: Cardiac dysfunction was present in 11 patients with high incidence in patients with low pre-transfusional haemoglobin group (p=0.4) and in patients having high serum ferritin level (p=0.02). Systolic cardiac dysfunction was present in 7(14%) of patients and diastolic dysfunction was present in 4(8%) of patients. There was a weak but significant correlation between left ventricular ejection fraction and serum ferritin concentration (r=-0.22; p=0.03). Only few (8%) patients had diastolic dysfunction.Conclusion: Patients with beta thalassaemia on an adequate transfusion showed an abnormal left ventricular systolic function. In early stage of disease diastolic function was normal but after repeated transfusion there were impaired relaxation indicating diastolic dysfunction. These findings seem mainly to be related to chronic anaemia and serum ferritin levelCardiovasc. j. 2016; 9(1): 31-35

scholarly journals Thyroid Function Status and Echocardiography Abnormalities in Patients with Beta Thalassemia Major in Bahrain

2013 ◽  
Vol 7 ◽  
pp. CMC.S10702 ◽  
Author(s):  
Taysir S. Garadah ◽  
Najat A. Mahdi ◽  
Ahmed M. Jaradat ◽  
Zuheir A. Hasan ◽  
Das S. Nagalla
Keyword(s):  
Regression Analysis ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Systolic Function ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Tissue Doppler ◽  
Thyroid Stimulating Hormone ◽  
Beta Thalassemia ◽  
Pulsed Doppler

Background Thyroid gland dysfunction and echocardiographic cardiac abnormalities are well-documented in patients with transfusion dependent beta-thalassemia major (β-TM). Aim This cross-sectional analytic study was conducted to investigate left ventricle (LV) diastolic and systolic function using pulsed Doppler (PD) and tissue Doppler (TD) echocardiography and correlate that with serum level thyroid stimulating hormone in patients with β-TM. Methods The study was conducted on patients with β-TM (n = 110, age 15.9 ± 8.9 years) and compared with a control group (n = 109, age 15.8 ± 8.9 years). In all participants, echocardiographic indices of PD and TD were performed and blood samples were withdrawn for measuring the serum level of TSH, free T4, and ferritin. A linear regression analysis was performed on TSH level as the dependent variable and serum ferritin as independent. Stepwise multiple regression analysis was used to determine the odds ratio of different biochemical and echo variables on the risk of developing hypothyroidism. Results Patients with β-TM compared with controls had thicker LV septal wall index (0.65 ± 0.26 vs. 0.44 ± 0.21 cm/M2, P < 0.001), posterior wall index (0.65 ± 0.23 vs. 0.43 ± 0.21 cm/m2, P < 0.01) and larger LVEDD index (4.35 ± 0.69 vs.3.88 ± 0.153 mm/m2, P < 0.001). In addition, β-TM patients had higher transmitral E wave velocity (E) (70.81 ± 10.13 vs. 57.53 ± 10.13 cm/s, P = 0.02) and E/A ratio (1.54 ± 0.18 vs. 1.23 ± 0.17, P < 0.01) and shorter deceleration time (DT) (170.53 ± 13.3 vs. 210.50 ± 19.20 m sec, P < 0.01). Furthermore, the ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus (E/Em) was significantly higher in the β-TM group (19.68 ± 2.81 vs. 13.86 ± 1.41, P < 0.05). The tissue Doppler systolic wave (Sm) velocity and the early diastolic wave (Em) were significantly lower in the β-TM group compared with controls with Sm, 4.82 ± 1.2 vs. 6.22 ± 2.1 mm/sec, P < 0.05 and (Em), 3.51 ± 2.7 vs. 4.12 ± 2.5 mm/sec. P < 0.05, respectively). The tricuspid valve velocity was significantly higher in β-TM patients compared with controls 2.85 ± 0.56 vs. 1.743 ± 0.47 m sec, respectively, P < 0.01). The prevalence of subclinical hypothyroidism in patients with β-TM was 15.4%, with significantly higher mean serum TSH compared with controls (6.78 ± 1.5 vs. 3.10 ± 1.02 μIU/mL, P < 0.01) and positively correlated with the serum ferritin level ( r = 0.34, P = 0.014). On multiple regression analysis, the LV mass, LVEF%, and E/A ratio were not positive predictors of hypothyroidism in patients with β-TM. Conclusion We conclude that patients with β-TM had a high prevalence of subclinical hypothyroidism of 15.4%. Thyroid stimulating hormone was significantly high and positively correlated with the serum ferritin level. Echo cardiographic pulsed Doppler showed a restrictive LV diastolic pattern suggestive of severe diastolic dysfunction with preserved left ventricle systolic function.

Pubertal status and its relation with serum ferritin level in thalassemia major patients

2019 ◽  
Author(s):  
Morteza Alijanpour Aghamaleki MD ◽  
Ahmad Tamaddoni MD ◽  
Hassan Mahmoodi Nesheli MD ◽  
Mahmoud Hajiahmadi PhD ◽  
Motahareh Amouzadeh Samakoush MD ◽  
...  
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Hypogonadotropic Hypogonadism ◽  
Thalassemia Major ◽  
Careful Examination ◽  
Delayed Puberty ◽  
P Value ◽  
Cross Sectional ◽  
Pubertal Status

Background: Thalassemia major (TM) is one of the most common hereditary anemia with multiple endocrinopathies (especially hypogonadism). So, we evaluated the rate of delayed puberty (DP) and its relation with serum ferritin level in patients. Materials and Methods: This cross-sectional (descriptive-analytical) study was conducted on 100 patients with TM between 14-64 years old, admitted to Amirkola Thalassemia Center, Babol, Iran, in 2016. The pubertal status, (Marshall-Tanner scale), existance of DP, and its different types were evaluated. Mean serum ferritin level was measured and the data were classified to three groups of <1500, 1500-2500, and >2500 ng/ml. Data were analyzed using SPSS (version20). Results: Out of 100 patients, 64 (64%) and 36 (36%) were female and male, respectively. Considering age, 23, 77 patients (%) were under and over 20 years old, respectively. Totally, 69 (69%) of them had DP, of whom 64 (92.8%) ones had secondary (central) hypogonadotropic hypogonadism. Mean serum ferritin level (±SD) was 2707.94±1683.42 ng/ml. In addition, 26, 29, and 45 patients had ferritin level <1500, 1500-2500, and >2500 ng/ml, respectively. Thirty two patients with DP (46.4%) had ferritin level above 2500 ng/ml (p-value= 0.623). Conclusion: The results showed a high frequency of DP in TM patients, requiring careful examination and follow-up in terms of puberty for early diagnosis and proper treatment to improve their quality of life, and prevention of the complications like osteoporosis. We couldn't find any significant relationship between serum ferritin level and hypogonadism, even for cases who received enough iron chelators. Keywords: Delayed Puberty, Ferritin, Hypergonadotropic Hypogonadism, Hypogonadotropic Hypogonadism, Thalassemia Major    

scholarly journals Detection of endocrine disorders in young children with multi-transfused thalassemia major

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Ramadan A. Mahmoud ◽  
Ashraf Khodeary ◽  
Marwa S. Farhan
Keyword(s):  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
High Serum ◽  
Endocrine Disorders ◽  
Inclusion Criteria ◽  
Poor Patient ◽  
Poor Patient Compliance ◽  
Iron Chelating Agents

Abstract Background Beta thalassemia major (TM) is the most common inherited genetic disorder worldwide. Patients are at risk of iron overload, which leads to various forms of tissue damage, including endocrinopathies. The aim of this study was to evaluate the prevalence and risk factors of endocrine disorders in young patients with multi-transfused TM receiving iron chelation therapy. Methods The inclusion criteria included all known cases of TM according to hemoglobin electrophoresis data, aged 12 years or younger, during the study period. The patient’s age, gender, parent’s consanguinity, clinical examination, and types of iron chelating agents used were recorded. Serum ferritin level, complete blood count (CBC), blood glucose homeostasis, thyroid, and parathyroid functions were determined. Results One hundred twenty patients met the inclusion criteria; 70% of them had malnutrition. The presence of endocrine disorders was observed in 28/120 (23.33%) patients. The most common endocrine disorders were thyroid disorders, either subclinical or clinical hypothyroidism in 11/120 (9.17%) patients, followed by abnormalities in glucose homeostasis 9/120 (7.5%). The prevalence of impaired glucose tolerance, impaired fasting glucose, and diabetes mellitus in the present study was 5 (4.17%), 4 (3.33%), and 0 (00%), respectively, while the least frequent endocrine disorder seen in our patients was hypoparathyroidism in 8/120 (6.66%). We noted that high serum ferritin levels and poor patient compliance to therapy were significantly associated with increased endocrine disorders (OR 0.98, 95% CI 0.96–0.99, P = 0.003 and OR 0.38, 95% CI 0.16:0.93, P = 0.03, respectively). Combined chelating iron agents significantly decreased the prevalence of endocrine disorders when compared with monotherapy (OR 0.40, 95% CI 0.16:0.97, P = 0.04). Conclusion Endocrine disorders could occur in TM patients early before or equal to 12 years of life in about one-fourth of the patients. A high serum ferritin level and poor patient compliance to therapy were significantly associated with increased endocrine disorders. Combined iron-chelating agents were associated with a decreased prevalence of endocrine disorders when compared with monotherapy.

scholarly journals Study of lipid profile in β thalassemia major pediatric patients with multiple blood transfusion and its correlation with serum ferritin level in tertiary care hospital in Kolkata

2021 ◽  
Vol 8 (4) ◽  
pp. 1778
Author(s):  
Saikat Nandi ◽  
Sanjana Samanta ◽  
Tanushree Mondal ◽  
Sanjay Halder
Keyword(s):  
Blood Transfusion ◽  
Lipid Profile ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Care Hospital ◽  
Average Value ◽  
Multiple Blood Transfusion ◽  
Multiple Blood

Background: β-Thalassemia major is associated with iron overload toxicity. This study was done to investigate lipid profile abnormality in β-Thalassemia major patients and to establish their correlation with serum ferritin. Another motto of this study was to signify deleterious effect of multiple blood transfusion and facilitate to understand how it will affect the lipid profile parameters changes so that we can also prevent early dyslipidemia induced complications like atherogenesis by early detection of lipid profile abnormality and also to establish derangement of lipid profile as an indirect evidence of iron overload.Methods: 102 nos. β-Thalassemia major paediatric patients with multiple blood transfusion (≥10) in age group of 6 to12 years admitted in pediatric ward of tertiary care hospital (NRSMCH) were enrolled. Serum ferritin level and lipid profile were investigated and their correlation were assessed.Results: Among 102 β-Thalassemia major pediatric (6 to 12 years) patients 55.88% were male and 66.67% were Hindu by religion and 69.61% children came from lower socio-economic class. The average value of number of previous blood transfusion was 42.79±7.510. The mean value of serum ferritin was 2263.53±833.904 ng/ml. The average value of serum total cholesterol, LDL and HDL were 153.84±5.428 mg/dl and 97.16±3.982 mg/dl and 29.45±4.445 mg/dl respectively and their correlation with serum ferritin were negative (r=-0.941 and r=-0.964 and r=-0.751 respectively). Average value of serum triglyceride and VLDL were 206.22±67.407 mg/dl and 36.16±3.385 mg/dl respectively and their correlation with serum ferritin were positive (r=+0.606 and r=+0.973 respectively).Conclusions: There was significant lipid profile derangement. As the serum ferritin level increases, serum total cholesterol, HDL, LDL decreases and serum triglyceride, VLDL level increases.

Investigation of Gonadal Function, Puberty, and their relationship to Serum Ferritin in Male patients with β-Thalassemia major in Syria

2021 ◽  
pp. 3595-3602
Author(s):  
Roula Shakkour ◽  
Taghrid Hammoud ◽  
Yasser Mukhalalaty ◽  
Faizeh Al Quobaili
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
Delayed Puberty ◽  
Beta Thalassemia ◽  
Endocrine Disorders ◽  
Gonadal Function ◽  
Pubertal Status ◽  
Gonadal Dysfunction ◽  
Male Patients

Objectives: Endocrine disorders continue to affect the health of thalassemia patients, foremost of which is hypogonadism being the most frequent endocrine complication that involves 70-80% of beta-thalassemia major (β-TM) patients. Actually, the role of iron overload in endocrine complications is well known. Our study goals were to investigate gonadal function, assess pubertal status among Syrian male patients with β-TM and correlate hormonal panel with serum ferritin as the marker of iron overload. Methods: 56 β-TM regularly transfused male patients were enrolled in this study, they were 21.91±5.01 years old. FSH, LH, Total Testosterone, and Serum Ferritin were measured for all patients, 52 of them undergone pubertal status evaluation. Results: Results showed that 60.7% of patients suffered from hypogonadism, which was hypogonadotropic hypogonadism in 97.06% of them. Delayed puberty was seen in 7.7% of the patients, while arrested puberty was found in 82.69% of them. All patients had iron overload and 92.86% of them suffered from severe iron elevation. Both gonadal and pubertal status were independent of the serum ferritin levels (P=0.73), (P=0.81) respectively. There was significant positive correlation between FSH: LH (r=0.584, P=0.0001), FSH: Testosterone (r=0.562, P=0.0001), LH: Testosterone (r=0.746, P=0.0001), MCHC: Testosterone (r=0.292, P=0.038), and BMI: Hb (r=0.351, P=0.009). Conclusions: Our findings indicated that hypogonadism, arrested puberty and severe iron overload were highly prevalent among male patients with β-TM. Patients with better gonadal reserve have higher BMI than those with gonadal dysfunction. We suggest that hypogonadism in β-TM patients is not directly related to serum ferritin levels; other potential factors (such as chronic anemia, hypoxia, and genetic predisposition) may contribute. Also we suggest that adequate blood transfusion and appropriate iron chelation, along with regular evaluation for gonadal status and timely intervention can improve the management of aforementioned complications, thus ameliorating patients’ quality of life.

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