Complications In Thalassemia Patients Research Articles

Penyuluhan Osteoporosis dalam Upaya Pencegahan Komplikasi Tulang pada Pasien Talasemia Mayor di Kabupaten Rembang dan Grobogan

Background Osteoporosis is a complication that often occurs in thalassemia major patients. This complication is still not widely known. This community service program aims to increase the knowledge of families of major thalassemia patients in Rembang Regency and Grobogan Regency regarding the complications of osteoporosis in thalassemia patients. Methods Education about osteoporosis, risk of osteoporosis in thalassemia patients, and prevention of osteoporosis. Extension method with presentation and discussion. The activity was attended by 20 thalassemia families in the Rembang Regency and 21 families of thalassemia patients in the Grobogan Regency. Before and after counseling, pretest and posttest questionnaires were completed to measure the knowledge of families of thalassemia major patients regarding bone complications in thalassemia patients. Results and Discussion Nearly half (46.34%) of respondents did not know the risk of osteoporosis in thalassemia. After counseling, 82.93% of respondents knew the risk of osteoporosis in thalassemia, but there were still 17.07% of respondents who still did not know this risk. Conclusion There is a significant increase in family knowledge of thalassemia major patients about the risk of osteoporosis in thalassemia. Further efforts to increase knowledge are needed with written information media (leaflets) given to families of thalassemia major patients

Alterations of Gut Microbiota Related with Status of Iron-Overload in Thalassemia Patients: A Cross-Sectional Pilot Study

Background: Gut dysbiosis can cause an impaired gut-barrier function and may contribute to the pathogenesis of various diseases. Iron overload is a common complication in thalassemia patients and can result in numerous complications, including those affecting the cardiovascular, endocrine, and immune systems. However, it is unclear how iron overload affects the gut microbiota of patients with thalassemia. Objective: To characterize the profiles of the gut microbiome in thalassemia patients compared with healthy participants, as well as to determine the association of these profiles with iron status. Method: Transfusion-dependent thalassemia (TDT) patients and healthy controls aged ≥ 18 years old were enrolled in the study. Stool samples were collected prior to receiving regular blood transfusions in the thalassemia group. Paired-end sequencing of 16s rRNA bacterial DNA extracted from each stool sample was performed. Serum ferritin levels were assessed in each participant to reflect iron status. Results: A total of 14 adult TDT patients and 14 healthy controls were enrolled, with a similar proportion of age and sex. The TDT patients had a significantly higher iron overload status than the controls (median serum ferritin level = 1424 in the TDT group and 197 in the control group; p < 0.001). The Shannon diversity index demonstrated a significant increase in gut microbial diversity in TDT patients as compared to healthy controls (7.06 vs. 6.48; p = 0.001). The TDT group showed a higher abundance of several gram-negative bacterial phyla, such as Bacteroidota, but a reduction in the abundance of Actinobacteriota as compared to the control group. Serum ferritin levels showed a positive correlation with higher gut diversity index. Conclusions: Our study suggests that TDT patients develop gut dysbiotic characteristics compared to the healthy population, with gut diversity associated with serum ferritin levels. This association suggests that iron overload induces gut dysbiosis.

Prevalence of Oral Manifestation of Thalassemic Patients in Sulaymaniyah Thalassemia Center

Thalassemia is a genetic blood disorder characterized by abnormal hemoglobin formation, leading to chronic anemia. The objective of this study was to evaluate orofacial complications in thalassemia patients and to detect the blood profile among these patients. Patients and method included a total of 100 th alassemic patients who were enrolled in this study. Oral examination and dental status of each patient was carried out using OHI-simplified. In addition, each patient took a blood investigation (Hb, RBC, MCV, RDW, and ferritin). Result of this study showed that the majority of the patients were female (68%) of which 50 of them had major Thalassemia, with the most frequent age range was between 21-39 years. Patients had statistically higher frequencies of all oral manifestations (p < 0.001 for all). Of which pale mucosa accounted for most frequency, 79%, followed by halitosis 39%, ulceration, brown pigmentation 33%, atrophic glossitis and actinic cheilitis 11%, hypomineralisation 8%, candidiasis and burning sensation 7%, geographic tongue and fissured tongue 6%, angular cheilitis 5%. Besides poor oral hygiene measure was 42%. Also, there was a significant positive correlation between most oral manifestations and blood transfusion. As a conclusion, thalassemia disease is associated with orofacial manifestation, which can adversely affect the oral health status of the patient. Therefore, dentists should understand the complications and management of the disease.

Spectrum Of HBB Gene Variants And Major Endocrine Complications In Thalassemia Patients Of Pakistan.

To determine the molecular characterisation of beta-thalassemia major patients, pattern of major endocrine complications and its association with haemoglobin subunit beta gene variants. The cross-sectional study was conducted from November 2021 to November 2022 after approval from the ethics review committee of Liaquat University of Medical and Health Sciences, Jamshoro, Pakistan, and comprised of 88 patients with beta thalassemia major aged >8 years and having serum ferritin level >1000 μg/L. The subjects were analysed for haemoglobin subunit beta gene variants and major endocrine complications, like growth retardation, hypogonadism, hypothyroidism, hypoparathyroidism and diabetic abnormalities using an automatic chemistry analyser, fully automatic chemiluminescence immunoassay analyser, enzyme-linked immunosorbent assay and real-time polymerase chain reaction. Data was analysed using SPSS 25. Of the 88 subjects, 40(45.4%) were girls and 48(54.5%) were boys. The overall mean age was 12±2.81 years. Of the total, 55(62.5%) had growth retardation, 41(46.6%) were cases of hypogonadism, 16(18.1%) hypothyroidism, 5(5.7%) hypoparathyroidism, 3(3.4%) diabetes mellitus and 8 (9.1%) had impaired glucose tolerance. Also, 65 (73.9%) patients confronted at least one endocrine complication. Endocrine complications were strongly associated with serum ferritin levels (p=0.000). The most common haemoglobin subunit beta gene variant identified was IVSI-5 (G>C) in 36 (40.9%), and the least identified variant was cluster of differenctiation-CD26(G>A) 1(1.1%). The association between haemoglobin subunit beta gene variants with endocrine complications was statistically non-significant (p>0.05). IVSI-5 (G>C) was found to be the most frequent haemoglobin subunit beta gene variant among beta- thalassemia major patients.

The Role of Doppler Imaging in the Evaluation of Immunological Complications in Thalassemia Patients' Children

The Role of Doppler Imaging in the Evaluation of Immunological Complications in Thalassemia Patients' Children

Efficacy and safety of inhaled nitrite in addition to sildenafil in thalassemia patients with pulmonary hypertension: A 12-week randomized, double-blind placebo-controlled clinical trial

Pulmonary hypertension is a significant complication in thalassemia patients. Recent studies showed that inhaled nebulized nitrite could rapidly decrease pulmonary artery pressure. We conducted a multicenter, randomized, double-blind, placebo-controlled trial in thalassemia patients with symptomatic pulmonary hypertension diagnosed by right heart catheterization. Eleven patients were recruited; five were assigned to the nitrite group and six to the placebo group. Patients were treated with the optimal doses of sildenafil for pulmonary hypertension and randomly assigned into the placebo or nitrite groups. Patients in the nitrite group were given inhaled nebulized 30 mg sodium nitrite twice a day for 12 weeks. The clinical outcomes measured at week 12 were the changes in 6-min walk distance (6MWD), mean pulmonary artery pressure (MPAP), and N-terminal pro B-type natriuretic peptide. The MPAP estimated by echocardiography was significantly reduced from 33.6 ± 7.5 mmHg to 25.8 ± 6.0 mmHg (mean difference = 7.76 ± 3.69 mmHg, p = 0.009 by paired t-test). Furthermore, 6MWD was slightly increased from 382.0 ± 54.0 m to 432 ± 53.9 m (mean difference = 50.0 ± 42.8 m, p = 0.059 by paired t-test) in the nitrite group. At week 12, the nitrite group had lower MPAP than the placebo group (25.8 ± 6.0 vs. 45.7 ± 18.5 mmHg, p = 0.048 by unpaired t-test). No significant difference in 6MWD and N-terminal pro B-type natriuretic peptide between the two groups was observed at week 12. There was no hypotension or other significant adverse effects in the nitrite group.

Prevalence and specificity of red blood cell alloantibodies and autoantibodies in transfused Iranian β-thalassemia patients: A systematic review and meta-analysis.

BACKGROUND:Repeated allogeneic blood transfusions in thalassemia major patients stimulate the patient's immune system to generate antibodies against foreign erythrocyte antigens. This study was carried out to systematically review the findings of available studies about the prevalence of alloantibodies and autoantibodies, as well as the type of causative antigens among transfusion-dependent thalassemia patients in Iran.METHODS:Electronic search was conducted on Medline, PubMed, Cochrane, EMBASE, ScienceDirect, and Persians databases. All relevant articles published from January 1990 to July 2018 were included. s of conference booklets which that been published in the last 5 years were also included in the meta-analysis. The search language was restricted to English and Persian. The quality of studies was evaluated according to a checklist developed by authors, and Cochrane Risk of Bias Assessment Tool was used to evaluate the risk of bias.RESULTS:Twenty-three relevant articles met all the inclusion criteria. The prevalence of alloimmunization was 13%. Our study showed that anti-D (25%) and anti-K (25%) were most prevalent among Iranian β-thalassemia patients. Data analysis shows the autoantibody prevalence to be 1% among 3787 patients. Meta-regression revealed that the prevalence of alloantibodies increases with each year as the average age of the study population increases.CONCLUSION:The prevalence of red blood cell (RBC) alloantibodies in transfused Iranian β-thalassemia patients was high. Appropriate preventive strategies such as RBC phenotyping for patients before beginning transfusion and using extended RBC donor–recipient matching, specifically for Rh and Kell system, could be implemented to avoid complications in thalassemia patients.

Comparative evaluation of cystatin C and neutrophil gelatinase-associated lipocalin in patients with thalassemia major versus thalassemia intermedia

Kidney disorders are long-term complications in thalassemia patients, especially with the high life expectancy of these patients. Proper evaluation of kidney impairment in β-thalassemia patients can be difficult due to higher intake of iron chelators, resulting in renal impairment. Early biomarkers of renal disease are used for the diagnosis of tubular and glomerular abnormalities. The current study was conducted on 88 individuals, 25 healthy people and 63 β-thalassemia patients. Circulating levels of urea, creatinine, cystatin C and neutrophil gelatinase-associated lipocalin were measured in all groups. Compared to healthy control, patients with thalassemia major and intermedia showed a significant increase in both cystatin C and NGAL levels, with no effects on creatinine levels. Furthermore, urea levels were markedly higher in patients with thalassemia major compared to control. As early renal dysfunction markers, cystatin C and NGAL should be routinely evaluated in thalassemia patients major and intermedia.

Evaluation of Relationship between Serum Ferritin Level and Cardiac Complications in Thalassemia Patients in Pasteur Hospital of Bam City in 2018

Introduction and objective: Cardiac complications are the most common cause of mortality in beta thalassemia patients. Hence, this study was conducted with the aim of evaluating the relationship between serum ferritin level and cardiac complications in Thalassemia patients at Specific Diseases Center in Bam city in 2018. Methodology: This is a descriptive-analytical study. Fifty-five patients with thalassemia major in Bam city were selected using a convenient sampling method. They were examined in terms of the prevalence of cardiac complications and their relationship with serum ferritin level was evaluated using SPSS 23 software and descriptive and inferential statistical tests. The significance level was considered p-value< 0.05 in this study. Results: The mean age of the patients was 17.84 ± 6.72. The mean serum ferritin level was 3674.07 ± 2192.21 mg / dl. Only 3 out of 29 patients (5.8%) had an ejection fraction (EF) reduction in echocardiography. In other echocardiographic studies, 13.7% of patients had MILD MR Heart valve dysfunction and 3.9% had MILD TR heart valve dysfunction and 1 person (1.9%) had both dysfunctions simultaneously. Moreover, 13 patients (25.5%) had diastolic cardiac dysfunction. There was no statistically significant relationship between the serum ferritin level of the studied people and echocardiographic findings (p-value <0.05). Discussion and Conclusion: Based on the results of this study and other studies, serum ferritin may not be relied as a definitive indicator for the evaluation of cardiac function. However, it is recommended that the importance of iron removal to be considered in patients’ training programs and different indicators to be used to evaluate the cardiac complications as the most important complication in thalassemia patients. Keywords: Thalassemia, Ferritin, Cardiac complications

Identification of Circulating Endocan-1 and Ether Phospholipids as Biomarkers for Complications in Thalassemia Patients.

Despite advances in our knowledge and attempts to improve therapies, β-thalassemia remains a prevalent disorder with increased risk for the development of cardiomyopathy. Using an untargeted discovery-based lipidomic workflow, we uncovered that transfusion-dependent thalassemia (TDT) patients had a unique circulating lipidomic signature consisting of 387 lipid features, allowing their significant discrimination from healthy controls (Q-value < 0.01). In particular, TDT patients had elevated triacylglycerols and long-chain acylcarnitines, albeit lower ether phospholipids or plasmalogens, sphingomyelins, and cholesterol esters, reminiscent of that previously characterized in cardiometabolic diseases resulting from mitochondrial and peroxisomal dysfunction. Discriminating lipid (sub)classes correlated differentially with clinical parameters, reflecting blood (ether phospholipids) and iron (cholesterol ester) status or heart function (triacylglycerols). We also tested 15 potential serum biomarkers related to cardiometabolic disease and found that both lipocalin-2 and, for the first time, endocan-1 levels were significantly elevated in TDT patients and showed a strong correlation with blood parameters and three ether diacylglycerophosphatidylcholine species. In conclusion, this study identifies new characteristics of TDT patients which may have relevance in developing biomarkers and therapeutics.

Evaluation of effective factors in the acceptance of mobile health technology using the unified theory of acceptance and use of technology (UTAUT), case study: Blood transfusion complications in thalassemia patients

Evaluation of effective factors in the acceptance of mobile health technology using the unified theory of acceptance and use of technology (UTAUT), case study: Blood transfusion complications in thalassemia patients

Impact of iron overload on bone remodeling in thalassemia.

Iron overload, a state with excessive iron storage in the body, is a common complication in thalassemia patients which leads to multiple organ dysfunctions including the bone. Iron overload-induced bone disease is one of the most common and severe complications of thalassemia including osteoporosis. Currently, osteoporosis is still frequently found in thalassemia even with widely available iron chelation therapy. Relevant publications published before December 2019 in PubMed database were reviewed. Both pre-clinical studies and clinical trials were obtained using iron overload, thalassemia, osteoporosis, osteoblast, and osteoclast as keywords. Increased ROS production is a hallmark of iron overload-induced impaired bone remodeling. At the cellular level, oxidative stress affects bone remodeling by both osteoblast inhibition and osteoclast activation via many signaling pathways. In thalassemia patients, it has been shown that bone resorption was increased while bone formation was concurrently reduced. In this review, reports on the cellular mechanisms of iron overload-associated bone remodeling are comprehensively summarized and presented to provide current understanding this pathological condition. Moreover, current treatments and potential interventions for attenuating bone remodeling in iron overload are also summarized to pave ways for the future discoveries of novel agents that alleviate this condition.

Fertility and pregnancy in Iranian thalassemia patients: An update on transfusion complications

Despite the significant advances in thalassemia pathobiology and efficacy of chelation regimens, complications of transfusion therapy have attenuated the reproductive health of thalassemia patients. Depending on clinical profiles, we aimed to assess the fertility status and stresses among thalassemia patients who desired to have children. A total of 213 couples in reproductive ages were enrolled in this study in Tehran. Patients' demographic, clinical, fertility and spouse's health status were documented. We evaluated the pituitary-gonadal axis, serum ferritin, liver enzymes, and alloimmunization before planning a pregnancy and reported them as a function of spontaneous conception and transfusion dependency. Data showed that 131 patients (62%) had 228 spontaneous pregnancies leading to 198 (86.6%) successful pregnancies. A significant difference was observed in spontaneous pregnancy with respect to fertility complications and transfusion dependency. In addition, the clinical conditions of spouses in patients with any spontaneous pregnancy were more thalassemia carriers (P < .05). Moreover, serum ferritin levels had a significant negative correlation with the levels of Testosterone, Estradiol, luteinizing hormone, and follicle-stimulating hormone. Furthermore, a significant positive correlation was reported with the level of liver enzymes. Finally, alanine transaminase and aspartate transaminase had a significant negative correlation with pituitary hormones. We suggest that organised instruction in addition to good iron chelation, especially during the puberty period, would reduce the oxidative damage and related complications in thalassemia patients. Moreover, infertility seems to be attributed to iron deposition in various endocrine organs, pituitary, reproductive system and the liver, contributing to hormonal metabolism.

Evaluation of effective factors in the acceptance of mobile health technology using the unified theory of acceptance and use of technology (UTAUT), case study: Blood transfusion complications in thalassemia patients.

Background: Mobile health or MHealth refers to the use of mobile phone in healthcare services to enhance the health level of people. Before using MHealth, it is necessary to study the effective factors in physicians’ adoption and acceptance of technology in the field of thalassemia. Methods: This cross sectional study was conducted using the survey and correlation methods. The statistical population of the study consisted of hematologists who were selected using the convenience sampling method. In this study, 58 questionnaires along with structural equations modeling based on partial least squares were used. SPSS and SMART PLS2 were used for data analysis. P values less than 0.05 were considered as statistically significant. Results: Based on the outcomes of the model from all theories, the coefficient of variation seems to be positive and the possibility of test is lower than 5%. The results indicated that all factors introduced in the proposed model are significantly effective in MHealth technology adoption. Conclusion: In this study, using the inputs from hematologists in hospitals and clinics in Tehran, it was aimed to find the factors affecting the hematologists’ decision to use mobile health technology in reducing the complications of blood transfusion in patients with thalassemia who needed blood transfusion. Thus, plans were made determine to priorities and the existing conditions to implement this new system. Also, the strengths and weaknesses of each factor were measured to improve the weaker factors. UTAUT was used to determine the acceptance factors. After reviewing the results, the use of this model is recommended to physicians.

MON-381 Endocrinological Evaluation of Adult Thalassemia Patients

Background: Endocrine disorders are among the most common complications in thalassemia patients. Although cardiac complications are the main cause of mortality, endocrinological disturbances have a significant impact on morbidity and quality of life. Methods: Sixty-eight patients (35 F, 33 M; 60 thalassemia major, 8 thalassemia intermedia) admitted to our outpatient clinic between August 2015 - December 2017 were included in the study. Patients were evaluated for short stature, hypogonadism, glycemic abnormalities, hypoparathyroidism, hypothyroidism and osteoporosis. Results: The average height of thalassemia major patients was 165.67±8.8 cm in men and 155.6±6.6 cm in women. Nine patients had short stature (4 F, 5 E), but 91.5% (54/59) of the whole group had low IGF-1 levels. There were 23 thalassemia major patients (11 F, 12 M) who had a history of hormonal induction therapy for delayed puberty. Overall, 60% (n = 36) of the patients were currently receiving hormone replacement therapy for central hypogonadism (19 F, 17 M). The median age at diagnosis of central hypogonadism was 22.5 years in men (IQR: 16.5-27.5) and 18 years in women (IQR: 16-25). There were five diabetic thalassemia major patients in study group whose median age at diagnosis was 20 (16-36). Of the 47 patients who underwent OGTT, 13 thalassemia major patients had prediabetes (27.7%). None of the thalassemia intermedia patients had glycemic abnormalities. Subclinical hypothyroidism was present in 19.7% (13/66) of the whole group, hypoparathyroidism was found in 8.5% (9/59) of thalassemia major patients, and vitamin D deficiency (25OH D < 20 ng/ml) was found in 70.8% (46/65) of all patients. Of 64 patients who underwent BMD, 25 had osteoporosis (39.1%) while 23 hadosteopenia (35.9%). The incidence of pathological fractures in thalassemia major patients was 20% (11/55). Conclusions: The incidence of endocrine disorders may increase in thalassemia patients due to prolonged duration of lifespan. Regular screening for newly emerging endocrinopathies during adulthood has great value. In our study, the most common endocrine disorders were vitamin D deficiency, hypogonadism, osteoporosis and glycemic abnormalities; respectively. Early diagnosis and treatment would prevent patients from having related morbidities and therefore increase quality of life.

Red cell alloantibodies in beta-thalassaemia major patients' blood referring to the regional blood transfusion center of Tehran, Iran.

Introduction: Thalassemia is associated with a genetic decline in the rate of synthesis of one or more types of natural hemoglobin polypeptide chains. One of the major complications in thalassemia patients is alloimmunization, which is antibody production by the patient against transfused red blood cells (RBCs). These RBCs are unknown by the recipient and the formed antibodies against them are called alloantibodies. This study aimed to evaluate the frequency of alloantibodies against RBCs in beta-thalassemia patients referred to Tehran Regional Blood Transfusion Center. Methods: In this study, antibody screening tests (Dia-cell I, II, and III) were performed on 184 thalassemia patients. An identification test by the Dia panel consisting of 11 different O RBCs groups to examine sera with Dia cells (I, II, or III) was performed. Results: In our study, males and females patients comprised 66 (35.87%) and 118 (64.13%), respectively, of whom 116 (63%) had alloimmunization. In addition, 68 thalassemia subjects (37%) lacked alloantibodies. Among 184 patients with beta-thalassemia major, anti-K (Kell system), anti-D, and anti-E (Rhesus system) had the most abundant alloantibody variants with an incidence of 24 (13%), 11 (5.98%), and 10 (5.4%), respectively. Conclusion: Before RBC transfusion, regular RBC antigen phenotypes, as well as problem-solving of alloantibody production by receiving compatible blood for Kell and RH subgroups, are suggested for all cases of transfusion-derived thalassemia.

Factor-Associated Risk Factors of Mild Cognitive Impairment in Thalassemia Patients : Probable Role of FGF21

Background Iron overload is the most common complication in thalassemia patients resulted from multiple blood transfusions and increased intestinal iron absorption. Heart and liver abnormalities remain major organs involved in patients with iron overload. There is limited data regarding the association between the iron overload and the risk of cognitive impairment in patients with thalassemia. Fibroblast growth factor 21 (FGF21) is a protein with wide ranging functions, including cell development, cell proliferation, and wound healing. Our previous study reported the FGF21 level was independently associated with cognitive impairment in non-elderly patients with metabolic syndrome. Therefore, the aim of this study is to determine the prevalence and associated risk factors of cognitive impairment in thalassemia patients. Methods This study was a cross-sectional and single-center study. All thalassemia patients, age 18 years or older, who attended the adult hematology clinic, Faculty of Medicine, Chiang Mai University, between November 2018 and July 2019 were enrolled in the study. The diagnosis of thalassemia was based on a hemoglobin analysis using a high-performance liquid column chromatography (HPLC) method. Patients who had pre-existing neurological disorders, psychiatric problems, history of brain surgery or history of traumatic brain injury were excluded from the study. Cognitive function was assessed using the Montreal cognitive assessment (MoCA) test. Data regarding age, sex, type and severity of thalassemia, level of education, transfusion status, maximum ferritin in the past 5 years and current medications were collected. The laboratory investigations included complete blood count, reticulocyte count, serum creatinine, electrolyte, liver function test and serum ferritin were performed at the time of enrollment. Blood samples for FGF21 level were collected from all participants. Results A total of 76 thalassemia patients were enrolled in this study. Seventy percent of patients were female with a median age of 31 years old (18-59). More than half of patients were graduated with a bachelor's degree. Beta-thalassemia/hemoglobin E disease was the most common type of thalassemia. Splenectomy was done in 48.7% of patients. Majority of patients (76.3%) developed iron overload and were treated with iron-chelating agents. Median serum ferritin and maximum serum ferritin in past 5 years were 1,418.5 ng/ml and 1,945.0 ng/ml, respectively. Mild cognitive impairment (MoCA score &lt;26) was observed in 76.3% of patients. Median serum ferritin and plasma FGF21 levels were 1,463.0 ng/ml, and 390.10 pg/ml, respectively. Plasma FGF21 level was positively associated with serum ferritin and maximum serum ferritin in past 5 years, but showed a negative correlation with hemoglobin level. Interestingly, MoCA score was negative associated with plasma FGF21 level and was not different among type of thalassemia, splenectomy, gender, transfusion status, and hemoglobin level (Figure 1). Conclusions A high prevalence of mild cognitive impairment was occurred in thalassemia patients (76%). FGF21 level was independently associated with cognitive impairment in thalassemia patients. Therefore, the possible role of FGF21 level in cognitive impairment in thalassemia patients should be further investigated. Disclosures No relevant conflicts of interest to declare.

Protein C and Anti-Thrombin-III Deficiency in Children With Beta-Thalassemia.

BackgroundThromboembolic events (TEEs) are recently described complications in thalassemia patients. Many mechanisms were postulated for thrombosis. Conflicting results of natural anticoagulants values were reported in previous studies. Our aim was to investigate protein C and anti-thrombin-III (AT-III) levels in thalassemics and to detect risk factors for their decrement.MethodsA cross-sectional study for 60 beta-thalassemia patients (35 major and 25 intermedia) and 35 healthy children were tested for protein C and AT-III levels, liver function tests and Sr. ferritin.ResultsA significant reduction in protein C and AT-III levels was noticed in patient group compared to healthy children (82.50% (32 - 175) vs. 104% (60 - 204), P = 0.041 and 237.52 ± 53.19 mg/L vs. 322.99 ± 56.57 mg/L, P value ≤ 0.001, respectively). Protein C was lower among older patients (> 10 years) than younger patients (< 10 years), and splenectomized category than non-splenectomized one (P = 0.02 and 0.011, respectively). AT-III was significantly lower among splenectomized patients as compared to those who did not undergo splenectomy (P = 0.04). Significant correlations were found between protein C and AT-III with older age and liver functions.ConclusionsProtein C and AT-III were significantly lower among thalassemics with the main risk factors for their deficiencies being: splenectomy and increasing age. This allows establishment of early prophylactic policy against TEE for the vulnerable group.

Alloimmunization in Thalassemia Patients: New Insight for Healthcare.

Background:Development of alloantibodies against the foreign red blood cell (RBC) (alloimmunization) is a well-known complication in thalassemia patients when performing multiple transfusions. The study was conducted to know the prevalence of alloimmunization in thalassemia patients, in the Caspian Sea coastline.Methods:This study is a descriptive, retrospective analysis of transfusion records of 190 patients with β-thalassemia major who received regular transfusions. To detect the type of alloantibodies, two cells panel tests (kits; Iranian Blood Transfusion Organization [IBTO], 3 RBC cells and IBTO, 11 RBC cells) were used. Results:Forty-seven patients were positive for alloantibodies (24.7%). Of them, 18.4% (35 cases) had only one alloantibody, and 6.3% (12 cases) had at least two or more of alloantibodies. The vast majority of alloantibodies were anti-Kell followed by anti-E, and anti-D, respectively.Conclusions:Blood matching for Rh and K antigens in patients with transfusion-dependent thalassemia could reduce the rate of RBC alloinununization.

Status of glucose metabolism including insulin resistance and beta cell function in overtly iron loaded Thalassemia patients

BACKGROUND Abnormality of glucose metabolism is a frequent complication in Thalassemia patients. Both insulin deficiency and insulin resistance has been proposed in its pathogenesis. Some form of abnormality in glucose metabolism is expected at an earlier age in these patients in developing countries like India and Nepal where iron overload is excessive due to lack of chelation therapy.MATERIALS AND METHODS Fasting serum glucose and fasting serum insulin (FSI) were measured in 40 beta-thalassemia major patients, 40 Ebeta- thalassemia patients and 40 controls, all aged between 5 and 12 years. 2 hours after an appropriate dose of oral glucose feed (Children ingested 1.75 g/kg body weight maximum 75 gram dissolved in 250 to 300 ml water) blood samples were drawn again to measure post prandial serum glucose. Iron overload was assessed by measuring liver size, spleen size, total amount of packed cells transfused and serum ferritin. Insulin resistance (IR), insulin sensitivity (%S) and beta cell functions (%B) were derived from the measured laboratory parameters using the latest version of Homeostatic Model Assessment (HOMA) calculator software.RESULTS No one had impaired glucose metabolism or diabetes mellitus beta-thalassemia major patients showed evidence of insulin resistance in the form of significantly higher fasting serum insulin (p value 0.002), IR (p value 0.003), %B (p value 0.017) and significantly lower %S (0.002) when compared with controls. FSI showed positive correlation with total amount of packed cells received (r=0.372, p=0.018), serum ferritin (r=0.345, p=0.029) and spleen size (r=0.427, p=0.006). Similarly, IR also showed positive correlation with total amount of packed cells received (r=0.388, p=0.013), serum ferritin (r=0.336, p=0.034) and spleen size (r=0.425, p=0.005). %S showed negative correlation with all these parameters. %B didn’t show any statistically significant correlation with these parameters.Ebeta- thalassemia patients didn’t have any statistically significant difference in FSI, IR, %S and %B than controls.CONCLUSION Insulin resistance develops as the earliest abnormality in glucose metabolism in overtly iron loaded beta thalassemia major patients at an early age. Ebeta- thalassemia patients with milder phenotype do not develop dysfunction of glucose metabolism at such an early age.Journal of College of Medical Sciences-Nepal, 2014, Vol-10, No-3, 29-36