scholarly journals The Correlation between Pituitary Gonadotrophins, Gonadal Sex Steroid Hormone with Ferritin Level in Pubertal Females with Thalassemia Major at Wassit Province - Iraq 2020

2021 ◽  
Vol 9 (B) ◽  
pp. 806-810
Author(s):  
Ahmed Ansaf ◽  
Safaa A. Faraj ◽  
Safaa Kazem
Keyword(s):  
Serum Ferritin ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Significant Risk ◽  
Thalassemia Major ◽  
Sex Steroid ◽  
P Value ◽  
Primary Amenorrhea ◽  
Female Patients ◽  
Gonadal Failure

 Thalassemia Major still a growing disease in Iraq with no clear plan for prevention.  Failure to get a secondary sexual character, amenorrhea, and then the inability to get pregnant is one the increasingly common problems in female patients with huge burden effects. Despite recent advances in iron chelation therapy, still, excess iron deposition in pituitary gonadotropic and gonadal cells remains one of the major causes for gonadal failure. Objectives: To evaluate the effect of iron overloads measured by serum ferritin on pituitary gonadotrophins measured by FSH, LH, and the gonadal sex steroid measured by estrogen in pubertal females with thalassemia Major. Methods: 41 female patients randomly selected in a cross-sectional study with diagnosed Thalassemia Major registered in Al-Kut Hereditary Blood Disease Center in the south of Iraq. Demographic data were evaluated including (age, type of chelation therapy, compliance for treatment and mean hemoglobin levels, and presence of amenorrhea). Serum ferritin, hemoglobin, FSH, LH, Estrogen levels were collected from patient's files.  Results:   the age of the studied sample were range from 14-43 years. The majority of the studied patients 33(80.5%) were suffering from primary amenorrhea and only six had a normal menstrual cycle. Twenty-six (63.4%) patients had serum ferritin levels of more than 3000 ng/dl. A low average serum FSH, LH, and Estrogen concentration were observed in the group with serum ferritin levels more than 3000ng/dl in a statistically significant p-value, 0.001, 0.002, and 0.003 respectively with inverse Pearson's correlation (-0.4 and p-value 0.0009 for FSH), (- 0.2 with no significant p-value 0.12, for LH) and (-0.3 with significant p. value 0.02 for estrogen level).  Conclusions:  In this study majority of females with thalassemia, Major suffer from disruption of pituitary gonadotropins and possible gonadal sex steroid. High ferritin still is a statistically significant risk factor for gonadal failure. The needs for more strict iron control, early screening, and proper treatment of gonadotropin and gonadal sex steroid deficiency around the age of 13 years are essential to be offered. More studies and a larger sample are needed to confirm and/or add other risk factors in pubertal thalassemic females.      

scholarly journals Relation of Left Ventricular Diastolic Dysfunction to Serum Ferritin in Thalassemia Major

2019 ◽  
pp. 224-233
Author(s):  
Aso Faeq Salih ◽  
Adnan M. Hamawandi
Keyword(s):  
Diastolic Dysfunction ◽  
Diastolic Function ◽  
Serum Ferritin ◽  
Ferritin Level ◽  
Serum Levels ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
Lv Function ◽  
P Value ◽  
Lv Diastolic Function

A prospective study was conducted on 60 thalassemia major patients (33 males and 27 females ) in whom the LV function and its parameters where assessed by echocardiography in relation to serum ferritin during the period from 2nd October 2007 to 2nd February 2008, in Sulaimani pediatric teaching hospital. Patient and method: The age ranges of patients were ranged between 3 – 18 years old.  The diastolic function of LV was assessed by (M-mode, D-mode, and pulse dopplar study of mitral and pulmonary vein) done.  Among 60 patients seen, 25% shows normal LV diastolic function, the rest is 13% relaxation dysfunction, 27% shows Psuedonormal diastolic dysfunction and 35% shows restrictive pattern of LV diastolic dysfunction. Mean serum ferritin in normal and abnormal diastolic LV dysfunction was not so different. In addition nearly all LV parameters as ( LVEDD , LVESD , LA diameter , aortic root diameter , IVC , E ,A ,E/A ratio , A-E time , DT time , S,D,R  waves ) where non significantly related to serum ferritin level , this means that P value is less than 0.05 . This means that the level of serum ferritin has no relation with the above parameters in which 75% of those parameters were already abnormal in comparison to the age of the child. Another finding shows that 25% of normal diastolic function is seen in serum levels above 1500 ng/l, in addition, 50% of diastolic dysfunction will associate serum ferritin of less than 500ng/l . P value is more than 0,05 this means that its non-significant. In conclusion, there is a non-significant correlation between serum ferritin and LV diastolic function, so serum ferritin is not a dependant indicator for myocardial iron deposition.

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.

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 SEVERE LIVER IRON CONCENTRATIONS (LIC) IN 24 PATIENTS WITH Β-THALASSEMIA MAJOR: CORRELATIONS WITH SERUM FERRITIN, LIVER ENZYMES AND ENDOCRINE COMPLICATIONS

2018 ◽  
Vol 10 ◽  
pp. e2018062 ◽  
Author(s):  
Vincenzo De Sanctis
Keyword(s):  
Iron Overload ◽  
Adrenal Insufficiency ◽  
Serum Ferritin ◽  
Chelation Therapy ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Dry Weight ◽  
Thalassemia Major ◽  
Liver Iron

Abstract. Introduction: Chronic blood transfusion is the mainstay of care for individuals with β-thalassemia major (BTM). However, it causes iron-overload that requires monitoring and management by long-term iron chelation therapy in order to prevent endocrinopathies and cardiomyopathies, that can be fatal. Hepatic R2 MRI method (FerriScan®) has been validated as the gold standard for evaluation and monitoring liver iron concentration (LIC) that reflects the total body iron-overload. Although adequate oral iron chelation therapy (OIC) is promising for the treatment of transfusional iron-overload, some patients are less compliant with it and others suffer from long-term effects of iron overload. Objective: The aim of our study was to evaluate the prevalence of endocrinopathies and liver dysfunction, in relation to LIC and serum ferritin level, in a selected group of adolescents and young adult BTM patients with severe hepatic iron overload (LIC from 15 to 43 mg Fe/g dry weight). Patients and Methods: Twenty-four selected BTM patients with severe LIC, due to transfusion-related iron-overload, followed at the Hematology Section, National Center for Cancer Care and Research, Hamad Medical Corporation of Doha (Qatar), from April 2015 to July 2017, were retrospectively evaluated. The prevalence of short stature, hypogonadism, hypothyroidism, hypoparathyroidism, impaired fasting glucose (IFG), diabetes, and adrenal insufficiency was defined and assessed according to the International Network of Clinicians for Endocrinopathies in Thalassemia (ICET) and American Diabetes Association criteria. Results: Patients have been transfused over the past 19.75 ± 8.05 years (ranging from 7 to 33 years). The most common transfusion frequency was every 3 weeks (70.8%).  At the time of LIC measurements, the mean age of patients was 21.75 ± 8.05 years, mean LIC was 32.05 ± 10.53 mg Fe/g dry weight (range: 15 to 43 mg Fe/g dry weight). Their mean serum ferritin level was 4,488.6 ± 2,779 µg/L. The overall prevalence of growth failure was 26.1% (6/23), IFG was 16.7% (4/24), sub-clinical hypothyroidism was 14.3% (3/21), hypogonadism was 14.3% (2/14), diabetes mellitus was 12.5% (3/24), and biochemical adrenal insufficiency was 6.7% (1/15). The prevalence of hepatitis C positivity was 20.8% (5/24). No case of clinical hypothyroidism, adrenal insufficiency or hypoparathyroidism was detected in this cohort of patients. The prevalence of IFG impaired fasting glucose was significantly higher in BTM patients with very high LIC (>30 mg Fe/g dry liver) versus those with lower LIC (p = 0.044). LIC was correlated significantly with serum ferritin levels (r = 0.512; p = 0.011), lactate dehydrogenase (r = 0.744; p = 0.022) and total bilirubin (r = 0.432; p = 0.035). Conclusions: A significant number of BTM patients, with high LIC and endocrine disorders, still exist despite the recent developments of new oral iron chelating agents. Therefore, physicians’ strategies shall optimize early identification of those patients in order to optimise their chelation therapy and to avoid iron-induced organ damage. We believe that further studies are needed to evaluate if serial measurements of quantitative LIC may predict the risk for endocrine complications. Until these data are available, we recommend a close monitoring of endocrine and other complications, according to the international guidelines.  

scholarly journals THE CORRELATION OF RELATIVE WALL THICKNESS, LV MASS/LV MASS INDEX, CARDIAC INDEX AND PCWP IN RELATION TO SERUM FERRITIN IN TRANSFUSION DEPENDENT BETA THALASSMEIC CHILDREN IN NORTH WEST RAJASTHAN

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Sandeep Kulhari ◽  
Himanshu Gupta ◽  
Dinesh Choudhary ◽  
Rajneesh Patel ◽  
Jaipal Bugalia ◽  
...  
Keyword(s):  
Cardiac Index ◽  
Serum Ferritin ◽  
Wall Thickness ◽  
Serum Ferritin Level ◽  
Ferritin Level ◽  
Thalassemia Major ◽  
Relative Wall Thickness ◽  
P Value ◽  
Blood Transfusions ◽  
Lv Mass

Objective: Regular blood transfusions used for long term survival in ß-thalassemia major patients cause a secondary state of tissue iron overload. Myocardial iron deposition can result in cardiomyopathy, and heart failure remains the leading cause of death. This study was planned to see the correlation of Relative Wall Thickness (RWT), LV Mass, Cardiac Index(CI)  and pulmonary capillary wedge pressure(PCWP) in relation to Serum Ferritin in transfusion  dependent  Beta Thalassemia children. Methods: Patients of ß thalassemia major above 2 years of age received regular blood transfusions at least for 1 year duration, attending OPD in the Department of Pediatrics, S.P. Medical College, Bikaner were enrolled. Echo findings of 50 cases were correlated with serum ferritin level(SFL). Result: Mean RWT in SFL group <2500ng/ml was 0.40±0.09, in SFL group 2500-5000 ng/ml, it was 0.41±0.08 and in SFL group >5000 ng/ml, it was 0.49±0.09; p value>0.05. Mean LV mass in SFL group <2500 was 68.76±24.32, in SFL group 2500-5000, it was 90.07±24.18 and in SFL group >5000, it was 123.06±42.42. The difference was found statistically highly significant (p<0.001). Mean PCWP in SFL group <2500 was 11.55±1.53, in SFL group 2500-5000, it was 12.02±2.06 and in SFL group >5000, mean PCWP was 13.31±2.09;p value>0.05. Mean CI in SFL group <2500 was 5.24±0.99, in 2500-5000 group was 5.79±1.07 and in SFL group >5000, it was 5.91±1.26 ; p value>0.05. Conclusion: There was significant positive correlation of serum ferritin level only with LV Mass.Relative Wall thickness, PCWP and cardiac index were insignificantly correlated. . Keywords: ß-thalassemia major; Relative wall thickness, LV mass/ LV mass index, cardiac index, PCWP, Echocardiogram; Tissue Doppler Imaging

scholarly journals Effect of thalidomide in patients with β–thalassemia major

2018 ◽  
Vol 6 (6) ◽  
Author(s):  
Shahzad Ali Jiskani ◽  
Sumair Memon
Keyword(s):  
Serum Ferritin ◽  
Ferritin Level ◽  
Treatment Options ◽  
Tertiary Care ◽  
Thalassemia Major ◽  
Hereditary Disease ◽  
P Value ◽  
Care Hospital ◽  
Female Ratio ◽  
Before And After

Introduction: Thalassemia is among the common hereditary disease, caused by synthesis of defective haemoglobin, leading to ineffective erythropoiesis, chronic haemolytic anaemia and certain clinical complications. Various treatment options are available for treatment of β–thalassemia major including regular blood transfusion, iron chelation therapy and hydroxyurea. Although complications and poor survival is still major problem in these patients. So treatment with alternative drugs can be helpful in management of these patients. Methods and materials: Prospective study, conducted at a tertiary care hospital. Study included 70 known cases of transfusion dependant thalassemia major. Age, gender, haemoglobin and serum ferritin levels were analyzed before and after treatment with thalidomide. All data were collected and analyzed in SPSS 21.0. P–value of <0.05 was considered as statistically significant. Results: Among 70 patients, 46 were males and 24 were females (male to female ratio=1.9:1). Mean age of the patients was 10.31±1.24years. Before and after treatment with thalidomide, mean haemoglobin was 8.93±1.04g/dL and 10.54±1.18g/dL respectively (p=0.011). Before and after treatment, mean serum ferritin level was 3125±143.51ng/mL and 1241±135.94ng/mL (p>0.001). Conclusion: Thalidomide proved to increased haemoglobin levels and reducing ferritin levels in patients with β–thalassemia major.

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

Hemoglobin ◽  
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

scholarly journals 329 Heart failure predictive value of the electric risk score in patients suffering from thalassemia major

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Elisa Tomarelli ◽  
Federica Moscucci ◽  
Anna Annunziata Losardo ◽  
Pellegrina Pugliese ◽  
Mauro Schina ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Score ◽  
Ventricular Hypertrophy ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Myocardial Damage ◽  
Thalassemia Major ◽  
Left Ventricular ◽  
P Value ◽  
Iron Chelation Therapy

Abstract Aims Complications associated with iron accumulation were highly recurrent in thalassemia patients, who underwent frequent blood transfusions, in particular hemosiderotic cardiomyopathy which could lead to heart failure and arrhythmias. Nowadays, the better iron chelation therapy has improved cardiovascular morbidity in these patients; nevertheless, mild impairment should be seek for and eventually treated. The objective of our study was to evaluate the possibility of using early electrocardiographic markers of myocardial damage and predictors of mortality, such as the Electric Risk Score (ERS). Methods and results 73 patients with thalassemia major were enrolled in this study, which were divided into two groups, with 45 years old as cut off. Anamnestic, clinical, electrocardiographic, and echocardiographic data were collected. From ECG, ERS was obtained. over 45 yrs-old group of pts, in addition to a predictable increase in the prevalence of traditional cardiovascular risk factors and drug intake, an alteration of the QRS-T angle (14[30] vs. −4[28], p value: &lt;0.0001) and an increased prevalence of left ventricular hypertrophy (2.88 ± 0.86 vs. 2.40 ± 0.57 p value: &lt;0.05) were found. In patients taking drugs with possible interactions with the ventricular repolarization phase, there is a slight increase in the QT interval, left ventricular hypertrophy and a reduction in Tpeak-Tend (Table 1). Electrocardiographic values in groups of patients with different age groups who are taking therapies that can affect QT. The echocardiogram revealed an increase in the end-diastolic diameter of the right ventricle (26 ± 3 vs. 28 ± 3 mm, P-value: 0.05) in the group of patients over the age of 45, a decrease in the acceleration time of the pulmonary systolic flow (138 ± 25 vs. 125 ± 13 ms, P-value: 0.04) and TAPSE (25 ± 3 vs. 22 ± 4 mm, P-value: 0.002). Conclusions From the data in our study it emerged that an appropriate iron-chelation therapy is able to effectively counteract the hemosiderotic cardiomyopathy of thalassemic patients so as to detect electro- and echocardiographic anomalies only in patients of more advanced age, a result that we think both the consequence, not so much of iron overload, but of an increase in the prevalence of age- and gender-related cardiovascular risk factors. The initial changes in cardiac electromechanics, which can be assessed with the aforementioned methods, we believe, can become a very early sign of specific myocardial damage. 329 Figure 1Electrical risk score parameters.

scholarly journals Zinc Status in Beta-Thalassemia Major

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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