Efficacy and possible adverse effects of the oral iron chelator 1,2- dimethyl-3-hydroxypyrid-4-one (L1) in thalassemia major [see comments]
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Background: Haemoglobin-A2 is considered as a paramount diagnostic parameter for the detection of beta-thalassemia trait which may vary with the fluctuation of body iron stores. The current study aims to evaluate the correlation of serum ferritin as a parameter of body iron stores with haemoglobin-A2 level in beta-thalassemia traits. Methods: This cross-sectional study was conducted on total 134 known beta-thalassemia traits in Rehman Medical Institute-Peshawar, Pakistan from October 2018 to June 2019. Blood samples from the contributors were drawn in EDTA and plain tubes for complete blood counts, haemoglobin-A2 and serum ferritin estimation. Participants were categorized into 3 groups on the basis of iron status; beta-thalassemia traits with low ferritin (Group A), normal ferritin (Group B) and high ferritin levels (Group C). Pearson correlation was applied to analyse the correlation between the variables. Results: Out of total 134 known beta-thalassemia traits, 73 (54.5 %) were males and 61 (45.5%) were females. Participants of group A with low ferritin were 22 (16.4%), group B with normal ferritin were 96 (71.6%) and group C with high ferritin were 16 (11.9%). Group A shows lowest mean haemoglobin-A2 level comparatively to Group B and Group C, with some effect of serum ferritin on haemoglobin-A2 level. Conclusion: Haemoglobin-A2 value decreases when there is a decrease in serum ferritin and show slightly increase with high ferritin level as compared to normal ferritin level or body iron stores in beta-thalassemia traits. However, this correlation is not significant enough to mask the actual diagnosis of the disease. Keywords: Beta Thalassemia Trait; Haemoglobin A2 level; Serum Ferritin
Beta thalassemia
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Hematology
Beta thalassemia
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Objective: To study the correlation of serum TSH levels with serum ferritin levels in children of β thalassemia major presenting at tertiary care hospital. Material and methods: Between April 2020 and October 2020 total 88 patients of β thalassemia major having age 5-15 years either male or female were selected from Department of Pathology, Sheikh Zaid Hospital, Rahim Yar Khan. Correlation of between ferritin and TSH levels was studies. Results: Mean age was 8.02 ± 2.792 years, mean TSH level was 3.9564 ± 2.38263 µIU/ml, mean ferritin level was 3282.84 ± 1782.013 ng/dl and mean duration of blood transfusion was 3.23 ± 1.328 years. (Table 1) Out of 88 patients, 67 (76%) were males and 21 (24%) were females. TSH levels was negatively correlated with ferritin levels which was not significant (r = -0.073, P= 0.496). Conclusion: In this study negative correlation was detected between ferritin and TSH levels. Difference of mean ferritin and TSH level among both gender, age group was detected. Key words: β thalassemia, TSH, ferritin, haemoglobin
Tertiary care
Positive correlation
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Background Transfusion-induced iron overload leads to many complications in patients with β-thalassemia major. This study aimed to compare the level of iron stored in the liver and heart measured by MRI T2 * with ferritin levels in these patients. Materials and Methods This study was done on 52 patients with β-thalassemia major aged 7 to 29 years. Serum ferritin level was checked and heart and liver MRI T2* operation was performed, then the liver and heart iron level measured by MRI T2 * was compared with serum ferritin levels. Results 150-180 cc/kg packed cells were received on average. The mean ± SD serum ferritin level was 2644.8±1988.3 ng/mL. The mean ± SD relaxation times in liver and heart MRI T2* were 4.39±5.8 and 26.59±10.62 millisecond, respectively. A negative significant correlation was found between serum ferritin levels and liver MRI T2* (P 0.05). Conclusion Since there was no correlation between serum ferritin levels and cardiac iron levels, accurate assessment of cardiac iron load using MRI T2* is necessary in patients with β-thalassemia major. Serum ferritin level is reliable for assessing liver iron levels.
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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
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Beta thalassemia
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Liver disease
Chronic liver disease
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Background: Thalassemiais one ofthe most commoninherited single-gene disorder in the world. Every year approximately 100,000 thalassemia major children are born all over the world, and there are about 65, 000-67,000β thalassemia major patients in India, with around 9,000-10,000 cases being added every year. Measurement of serum ferritin level can give idea regarding starting of Iron chelation therapy, which will reduce the concentration of serum ferritin and effective in preventing iron induced tissue injury and prolonging life expectancy. Method: This study was conducted on 60 children between 3 to 17 years, being regularly transfused at department of Pediatrics, Kamala Raja Hospital, Gajra Raja Medical College, Gwalior, for period of 1 year from 2011 to 2012, after taking the informed consent from the parents and explaining them the purpose of study. Detailed history weretaken and serum ferritin level weremeasured by ELIS Abased serum ferritin assay kit. Results: Serum ferritin level was found to be elevated in all the patients of betathalassemiamajorwithrange from 1050 to 5029 µg/l and with a mean value of 3879µg/l. Out of 60 patients, 30 (50%) patients had serum ferritin level below 2000μg/l,20 (33.3%) patients had serum ferritin value between 2001 to 4000 μg/l and rest 10 (17.7%) patients had values above 4000 μg/l. Mean value of serum ferritin was found to be higher in patients who received frequent blood transfusion. Conclusion: Majority of the patients had very high ferritin levels, with a mean value of 3879 µg/l. 50% patients had serum ferritin levels more than 2000μg/l. This cut off value reflect either inadequate chelation therapy or non-affordability of parents to purchase oral chelation therapy.
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Objectives: To study the effect of oral iron chelator on serum ferritin level in patients with thalassemia major, we see the dose and frequency of oral iron chelator and its effect on serum ferritin and BT (Blood transfusion) iron overload. Material and Methods: Patients with thalassemia with 5-18 year of age are taken in our study. Total numbers of patients taken are 50.1st Group is with BT iron overload of 0.2-0.3mg/kg/day is given oral iron chelator in the dose 20mg/kg/day & serum ferritin should be overload of >0.3mg/kg/day is given oral iron chelator in the dose 30mg/kg/day & serum ferritin should be >1500ng/ml. Oral iron chelator is given according to serum ferritin level which is done at frequent intervals. Oral iron chelator is given as per serum ferritin level and maximum dose of oral iron chelator is 40 mg/kg/day. We study the dose of oral iron chelator, change in serum ferritin level and its effects on BT iron overload. Results: Number of patients with BT iron overload in the range of 0.2-0.3 mg/kg is n=25(50%) in 1st Group. Number of patients with BT iron overload in the range of >0.3-0.4 mg/kg is n=15(30%) in 2nd Group. Number of patients with BT iron overload is more than 0.4 is n=10 (20%) in 3rd group. Calculated probability of average dose of oral iron chelator is 3rd group. There is significant decrease in serum ferritin level in 3 groups when we compare it with our beginning level (calculated probability BT iron overload, but BT iron overload is depend on dose of oral iron chelator. Serum iron concentration is also lowered as there is increase in dose of oral iron chelator. Conclusions: In our study we can say that oral iron chelator which can reduce serum ferritin level maximally was 30 mg/kg/day and showing very less side effects (BT iron overload taken for consideration was 0.3-0.4mg/kg/day). Oral iron chelator should be given less than 30mg/kg/day in patients having less BT frequency and less BT iron overload. Keywords: Thalassemia, Oral
Chelation Therapy
Deferiprone
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