scholarly journals Correlations between Iron Load and CD4 in Adult Transfusion-Dependent Beta Thalassemia

Anemia ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Tubagus Djumhana Atmakusuma ◽  
Ralph Girson ◽  
Sukamto Koesnoe

Background. Thalassemia is a hereditary disease, and severe anemia is the main phenotype of major thalassemia. Furthermore, the most important method in the management of this disease is red blood cell transfusion. Regular transfusions administered 1 or 2 times every month improve prognosis and survival. However, there is higher risk of infections and iron overload, especially in transfusion-dependent thalassemia (TDT). Infections are the second leading cause of death in adult TDT, after heart failure. Higher risk of infection is also influenced by multiple blood transfusions which causes alteration in immune response due to alloimmunization, transfusion-related infections, and iron overload. Meanwhile, iron overload in TDT alters both innate and specific immune responses. Furthermore, previous studies have shown the correlation between ferritin with CD4, but this has not been carried out in Indonesia. Therefore, this study aims to determine the correlations between iron overload (serum ferritin and transferrin saturation) and specific immune cells (CD4). Methods. This is a cross-sectional study, and a total number of 64 subjects were examined consecutively. Chest X-ray and blood sera were obtained. The total number of subjects was 64. The seromarkers HBsAg, anti-HCV, and anti-HIV were tested using the ELISA method. Serum ferritin and transferrin saturation was tested using ECLIA, and lymphocyte subsets were analyzed using flowcytometry. Meanwhile, the correlation between variables was determined using Spearman’s test. Results. The results showed that 4.9% subjects were HBsAg positive, 10.7% were anti-HCV positive, and none were anti-HIV positive. There were 4 subjects with lung tuberculosis based on the 41 chest X-ray. Meanwhile, there was a weak negative and insignificant correlation between serum ferritin with CD4 ( p = 0.75 ; r = −0.04) and a weak positive and insignificant correlation between transferrin saturation with CD4 ( p = 0.133 ; r = 0.19). Conclusion. There were no correlations between iron overload (ferritin) and cellular immunity (CD4) in adult transfusion-dependent thalassemia.

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4907-4907
Author(s):  
Faizal Drissa Hasibuan ◽  
Tubagus Djumhana Atmakusuma

Abstract Correlation Between Pancreatic MRI T2* And Iron Overload in Adult Transfusion Dependent Beta Thalassemia Patients With Growth Retardation : A Single Centre Study in Indonesia Faizal Drissa Hasibuan , MD 1,2 , Tb. Djumhana Atmakusuma , MD, PhD 3, 4 1Department of Internal Medicine, 2Faculty of Medicine Yarsi University Jakarta, Indonesia, 3Medical Hematology - Oncology Division of Internal Medicine Department Cipto Mangunkusumo Hospital, 4Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia Correspondence: [email protected] phone +6281533197733 The prevalence of thalassemia in Indonesia is one of the highest in the world. It is estimated that the prevalence of beta thalassemia carrier is around 3-10%. In 2016, thalassemia center in Cipto Mangunkusumo Hospital (RSCM) Jakarta recorded 9031 patients suffering from major thalassemia in Indonesia. 441 are adult thalassemia patients (age 18 and above) recorded in Kiara thalassemia and hematology-oncology clinics in RSCM. Based on a survey from TIF, endocrine aspect of the thalassemia patients is often ignored by the clinicians. Growth retarded patients are commonly found in the thalassemia clinic in RSCM. Publication regarding the pancreas and its correlation with iron overload in adult beta TDT patients is currently not available in Indonesia. Therefore, the aim of this study was to describe iron overload condition based on the pancreatic MRI T2* and its correlation with beta TDT adult patients who suffer from growth retardation. A cross sectional study was conducted to determine the prevalence of endocrine disorders in adult TDT beta patients, followed by looking for correlation of excess iron load with endocrine function in adult TDT beta patients with growth retardation in adult Thalassemia clinic RSCM Jakarta on December 2017. Patients with HBsAg or Anti HCV positive were excluded. Excess iron is defined as Transferin Saturation (ST) greater than 50% regardless of serum ferritin or serum ferritin (FS) levels greater than 1000 ng/mL regardless of ST or both.The growth retardation is defined as the standing height of the research subject which is lower than the Mid Parental Height (MPH) value of both parents. Pancreatic MRI T2* used magneto avanto Siemens 1,5T with CMR software. We found from 58 patients who followed the study, 32 patients underwent the pancreatic MRI T2* examination with 13 female (40,6%) and 19 male (59,4%). Patients with homozygous beta thalassemia are 16 people (50%) and beta/HbE thalassemia 16 people (50%). The Proportion of low pancreatic MRI T2* values was found to be 87.5%, with moderate hemosiderosis in 13 patients (40.63%) and severe hemosiderosis not found (Table 2). The age range of the study subjects was relatively young with a median age of 21 years. Although the median body weight of study subjects was 42 kg, the median BMI still included in normal range. Excess iron content in this study was assessed with serum ferritin, obtained median 4982.5 ng/mL and transferin saturation with a median of 100%, indicates the subject of research are in a state of excess iron load. This is due to the possibility of inflammation, inadequate use of chelation, hemolysis in thalassemia, hypertransfusion to achieve the target of 12 g/dL for women and 13 g/dL for men. There was no significant correlation between serum ferritin and pancreatic MRI T2* value, nor did a significant correlation between transferrin saturation with pancreatic MRI T2* value (Table 3). In this study, there was a high proportion of subjects with low pancreatic MRI T2* value of 28 subjects (87,5%), divided into 15 mild hemosiderosis (46,87%), 13 moderate hemosiderosis (40,62%) and none of severe hemosiderosis. Our study is the first study which look for the correlation of the excess iron load (serum ferritin and transferrin saturation) with endocrine function in adult TDT beta patients with retardation of growth in Indonesia. This research has limitations. First, it was a cross sectional study so it is not known exactly the beginning of endocrine disorders in the subject . The second limitation, analysis of iron chelation therapy did not do in this study. Finally, we concluded that there was no correlation between pancreatic MRI T2* and iron overload based on serum ferritin and transferrin saturation. Further longitudinal studies in adult TDT patients with thalassemia who have not and have retarded growth were needed. Disclosures No relevant conflicts of interest to declare.


2015 ◽  
Vol 3 (2) ◽  
pp. 287-292 ◽  
Author(s):  
Khaled M. Salama ◽  
Ola M. Ibrahim ◽  
Ahmed M. Kaddah ◽  
Samia Boseila ◽  
Leila Abu Ismail ◽  
...  

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.


2015 ◽  
Vol 6 (5) ◽  
pp. 47-50 ◽  
Author(s):  
A Boddu ◽  
A Kumble ◽  
S Mahalingam ◽  
BS Baliga ◽  
B Achappa

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour. DOI: http://dx.doi.org/10.3126/ajms.v6i5.11782Asian Journal of Medical Sciences Vol.6(5) 2015 47-50


Author(s):  
Zeina A Munim Al-Thanoon ◽  
Zeina A Munim Al-Thanoon ◽  
Mustafa Basil ◽  
Nasih A Al-Kazzaz

Iron chelation therapy with deferoxamine (DFO),the current standard for the treatment of iron overload in patients with betathalassemia,requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence,resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox (DFX) is an orally administered iron chelator that has been approved for use in many countries. The requirement of an effective,well tolerated iron chelator with a less demanding mode of administration has led to the development of deferasirox. The present study was aimed to compare the satisfaction and compliance with deferoxamine versus deferasirox (Exjade®),a novel oral iron chelator in patients with transfusion - dependent beta- thalassemia. A cross-sectional,single-center investigation study was carried out in the Thalassemia Center of Ibn-Atheer Teaching Hospital in Nineveh province,Iraq. One hundred and eight thalassemic patients aged between 2- 20 years old having received multiple blood transfusions and a serum ferritin greater than 1500 ng/ml. Patients were randomised into two groups. Group 1 received deferoxamine at a dose of 20-50mg/kg/day and group 2 received deferasirox at the dose of 10-30 mg/kg/day. Another 56 apparently healthy volunteers were used as a control group. The assessment of chelation was done during the period between November 2013 and February 2014 by measurement of serum ferritin. Satisfaction and compliance was assessed by using a special questionnaire prepared by the researcher. Out of the 108 thalassemic patients enrolled there was no discontinuation in treatment with the two drugs under study. The serum ferritin did not change significantly in any of the chelation groups. In comparison with the patients who were treated with DFO,those receiving DFX reported a significantly higher rate of compliance and satisfaction (P < 0.05). However,no significant difference was observed between the two groups regarding their satisfaction (P > 0.05).Compliance with deferasirox (50 %) was more than that with deferoxamine (20 %). Satisfaction with deferoxamine was significantly lower than deferasirox (p= 0.00).


2021 ◽  
Vol 15 (8) ◽  
pp. 2013-2016
Author(s):  
Shahid Ishaq ◽  
Muhammad Imran ◽  
Hashim Raza ◽  
Khuram Rashid ◽  
Muhammad Imran Ashraf ◽  
...  

Aim: To determine correlation of iron profile in children with different stages of chronic kidney disease (CKD) presenting to tertiary care hospital. Methodology: A total of 81 children with chronic kidney disease stage having glomerular filtration rate (GFR) less than 90 (ml/min/m2) aged 1 – 14 years of either sex were included. Three ml serum sample was taken in vial by hospital duty doctor for serum ferritin level, serum iron, transferrin saturation and total iron binding capacity. The sample was sent to hospital laboratory for reporting. Iron profiling was done evaluating hemoglobin (g/dl), serum iron (ug/dl), serum ferritin (ng/ml), transferrin saturation (%) and total iron binding capacity (ug/dl) while iron load was defined as serum ferritin levels above 300 ng/ml. Correlation of iron profile with different stages of CKD was determined applying one-way analysis of variance (ANOVA). Results: In a total 81 children, 46 (56.8%) were boys while overall mean age was 7.79±2.30 years. Mean duration on hemodialysis was 11.52 ± 9.97 months. Iron overload was observed in 26 (32.1%) children. Significant association of age above 7 years (p=0.031) and residential status as rural (p=0.017) was noted with iron overload whereas iron overload was increasing with increase in stages of CKD (p=0.002). Hemoglobin levels decreased significantly with increase in stages of CKD (p<0.001). Serum iron levels increased significantly with increase in the CKD stages (p=0.039). Serum ferritin levels were increasing significantly with the increase in CKD stages (p=0.031). Transferrin saturation also increased significant with increase in CKD stages (p=0.027). Conclusion: High frequency of iron overload was noted in children with CKD on maintenance hemodialysis and there was linear relationship with stages of CKD and iron overload. Significant correlation of hemoglobin, serum iron, serum ferritin and transferrin saturation was observed with different stages of CKD. Keywords: Iron overload, maintenance hemodialysis, ferritin level.


Author(s):  
R. Behzadmehr ◽  
E. Nejadkehkha

Despite many advances in the diagnosis, screening, and rapid treatment of tuberculosis, it is still a public health concern in the world. Due to the importance of this issue in diagnosis and reduction of transmission of infection and treatment of the disease especially where this study is conducted due to the high prevalence of tuberculosis, this study was done to determine The relationship between sputum smear positivity grade and chest X-ray findings in pulmonary tuberculosis patients in a hospital in southeast of Iran. This cross-sectional study was performed on all patients with pulmonary TB referencing the health centers in Zabol city, southeast of Iran from 1 January 2015 to 30 December 2020. Sputum smear and radiographic findings of the chest X-ray were evaluated. Data was collected using a form of information and finally analyzed by SPSS 22. Out of 101 patients examined in the present study, 71 were women and 30 were men. The mean age of the patients was 62.68 ± 13.61 years. The frequency of opacity in patients with grades 1, 2, and 3 was 71.4, 78.5, and 76.5%, respectively. Frequency of cavitation in patients with Grade 1, 2 and 3 was 11.5%, 28.5% and 52.9% respectively (P value 0.001). The frequency of reticulonodular presentations in patients with grade 1, 2, and 3 was 24.2, 7.1, and 0%, respectively.  In general, the results of this study showed that, with increasing grading of smears (1+, 2+, and 3+), the frequency of cavitation presentation increased significantly and the frequency of reticulonodular presentations decreased significantly. In general, the results of this study showed that, with increasing grading of smears (, the frequency of Cavitation presentation increased significantly and the frequency of reticulonodular presentations decreased significantly. The findings of the present study can help physicians better diagnose TB.


2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Joanitah Nalunjogi ◽  
Frank Mugabe ◽  
Irene Najjingo ◽  
Pastan Lusiba ◽  
Francis Olweny ◽  
...  

The WHO END TB strategy requires ≥90% case detection to combat tuberculosis (TB). Increased TB case detection requires a more sensitive and specific screening tool. Currently, the symptoms recommended for screening TB have been found to be suboptimal since up to 44% of individuals with TB are asymptomatic. The chest X-ray (CXR) as a screening tool for pulmonary TB was evaluated in this study, as well as its incremental yield in TB diagnosis using a cross-sectional study involving secondary analysis of data of 4512 consented/assented participants ≥15 years who participated in the Uganda National TB prevalence survey between 2014 and 2015. Participants with a cough ≥2 weeks, fever, weight loss, and night sweats screened positive for TB using the symptoms screening method, while participants with a TB defining abnormality on CXR screened positive for TB by the CXR screening method. The Löwenstein-Jensen (LJ) culture was used as a gold standard for TB diagnosis. The CXR had 93% sensitivity and 65% specificity compared to LJ culture results, while symptoms had 76% sensitivity and 31% specificity. The screening algorithm involving the CXR in addition to symptoms led to a 38% increment in the yield of diagnosed tuberculosis. The number needed to screen using the CXR and symptoms screening algorithm was 32 compared to 45 when the symptoms are used alone. Therefore, the CXR in combination with symptoms is a good TB screening tool and increases the yield of diagnosed TB.


2021 ◽  
Vol 17 ◽  
Author(s):  
Mateusz Puchala ◽  
Andrzej Rydzewski ◽  
Ilona Kowalik ◽  
Małgorzata Wisłowska

Background: GPA is a necrotizing inflammation of the small vessels with granulomas. Kidney involvement deteriorated its prognosis. Objective: Comparison of GPA patients with kidney (KI) and without kidney involvement (nKI). Material and methods: We conducted a cross-sectional study of 50 consecutive adult GPA patients, 25 KI from Nephrology and 25 nKI from Rheumatology Department of Central Clinical Hospital Ministry of Interior in Warsaw. We analyzed clinical features, organ involvement, laboratory, serological, imaging, histopathological data, BVAS, treatment. Results: The mean age of KI patients was statistically older then nKI (67.3±9.5 vs 55.1±15.9, p=0.002). Generalized, severe, resistance disease was observed respectively in 92% vs 44%, p<0.001. The number of red blood cells (3.47 vs 4.41T/l, p<0.001), hemoglobin (10.0 vs 12.9g/dl, p<0.001) was lower in KI, higher mean serum creatinine (3.95 vs. 0.89mg/dl, p<0.001), lower GFR (20.1 vs. 79.3, p<0.001), higher CRP (median: 43.4 vs 2.0mg/l, p<0.001), BVAS (16.6±4.4 vs 10.1±6.2, p<0.001), c-ANCA (median: 119.0 vs 15.2CU, p=0.017). Nodules in 28% KI, in 4% nKI (p=0.048) in chest X-ray, infiltration in 43.5% KI, in 15% nKI (p=0.042) in HRCT were observed. Skin granulomas were found in 61.5% nKI vs 18.2% KI, (p=0.047). Renal biopsy revealed in KI patients focal segmental glomerulonephritis in 11.8%, crescentic glomerulonephritis in 17.6%, pauci-immune crescentic glomerulonephritis in 70.6%. Conclusions: In patients with KI more frequently we found generalized, severe, resistant GPA, higher BVAS in comparison in patients without KI. The results of laboratory parameters, were worse in patients with KI. Aggressive immunosuppressive treatment is often used in KI group.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Lucas Acatauassu Nunes ◽  
Luciene Reis ◽  
Hanna Machado ◽  
Rosse Osório ◽  
Rosa Moyses ◽  
...  

Abstract Background Chronic kidney disease (CKD) is associated with several comorbidities, including anemia, since with decreased renal function there is a decrease in erythropoietin (EPO) production and changes in iron (Fe) metabolism. In hemodialysis patients, prescription of Fe is indicated to supplement the needs of this element by maintaining ferritin levels above 100 mg/dl and transferrin saturation greater than 20%. However, the excess of Fe can generate free Fe not bound to transferrin, and deposit in organs such as liver, heart, and bone marrow, with consequent impairment of their function. In hemodialysis patients, the diagnosis of Fe overload, its clinical significance and therapeutic decision have been poorly studied, unlike thalassemia patients. Aims To assess whether hemodialysis patients with ferritin levels equal to or greater than 1000 mg/l also have Fe overload in liver, heart, and bone marrow, as well as compromise bone density and remodeling. Method This is a cross-sectional analysis that included 28 adult patients on regular conventional hemodialysis. Inclusion criteria were serum levels of ferritin ≥ 1000 mg/l, and ESRD treated by regular hemodialysis for at least 6 months. We excluded patients with HIV, cancer, hepatic disease, patients who received desferroxamine in the latest year, and those previously submitted to a kidney transplant. All patients underwent dual-energy X-ray absorptiometry (DXA), serum ferritin, transferrin saturation index (STI), Fe, C reactive protein (CRP), Calcium(Ca), phosphorus (P), parathyroid hormone (PTH) and alkaline phosphatase (AP) levels were recorded. T2* image acquisition of Magnetic Resonance Imaging (MRI) 1,5 Tesla, were used for the assessment of Fe of liver, and heart. R2* and R2* Water were used of liver and bone (iliac crest). Bone biopsy was also performed. Results We evaluated 28 hemodialysis patients with a mean age of 55.8±13.1, hemodialysis time of 42.5±26.5 and iron use in the year prior to study enrollment of 311.5±179.8 mg/month. Biochemical analysis showed 3 patients with Hb below 9.0 mg/dl and 14 with values above 11.5 mg/dl; 6 patients with SatFe &lt;30% and 12 patients with ferritin &gt;1500mg/dl; 16 patients with PTH &lt;300pg/ml and eight with &gt;600pg/dl. MRI revealed Fe overload in the liver and bone tissue (figure 1) of all patients but not in the heart. Serum ferritin levels correlated with liver and bone overload (figure 2). Densitometry and bone biopsy results were not affected by Fe overload, however, serum Fe levels were associated with lower bone remodeling and mineralization suggesting an effect of this element on osteoblast activity. Conclusion High serum ferritin is associated with liver and bone marrow Fe overload, but not heart, as well as with low bone remodeling and mineralization. We must be aware of these side effects of high doses of Fe that are commonly used in these patients.


Sign in / Sign up

Export Citation Format

Share Document