Thalassemia Patients Research Articles

EFFECT OF Anti-miRNA 144 AND Anti-miRNA 150 ON THE EXPRESSION OF α GLOBIN CHAINS IN PBMC

The excess of unbound α globin chains are the basic pathophysiology of the cause of clinical symptoms in major β thalassemia. Recently there are many alternative therapies by increasing γ globin chains to reduced the effects of unbound α globin chains. Alternative therapies by decreasing α globin chains have not been much noticed. α globin expression involves complex regulatory involving transcription factors and miRNAs. It involves GATA-1, KLFD and MYB transcription factors . It also involves miRNA-144 and miRNA-150. The role of miRNA-144 and miRNA-150 to reduce the α globin chains expression in major β thalassemia patients is an alternative therapy. miRNA-144 and miRNA150 activity need to be known by using anti-miRNA 144 and anti- miRNA 150. Analyzed the effect of anti-miRNA 144 and anti-miRNA 150 on the expression of α globin chains. This study was an experimental study using PBMC of a major β thalassemia patients. PBMC divided into four groups that were not transfected, transfected by anti-miRNA 144, transfected by anti-miRNA 150 and transfected by anti-miRNA 144 and anti-miRNA 150. qPCR examinations to find out expression of miRNA-144 and miRNA-150. Western blot examination to find out the expression of α globin chains. There was significantly lower miRNA-144 expression in PBMC of major β thalassemia patients who had been transfected by anti-miRNA 144 than those not transfected. In line with the result of miRNA-144 expression which was 0.17 times lower than the control group. There was significantly lower miRNA-150 expression in PBMC of major β thalassemia patients who had been transfected by anti-miRNA 150 than those not transfected. In line with the result of miRNA-150 expression which was 0.30 times lower than the control group. There was significantly lower α globin chains expression in PBMC thalassemia patients who had been transfected by anti-miRNA 150 than those not transfected. There was significantly lower α globin chains expression in PBMC thalassemia patients who had been transfected by anti-miRNA 150 and anti-miRNA 144 than those not transfected. This is evidenced by the decreased in the area 10-25 KDa band. Based on this study, the administration of anti-miRNA 150 or anti-miRNA 144 and anti-miRNA 150 is capable of decreasing the expression α globin chains in PBMC of major β thalassemia patients.

Does the use of infusion pumps increase hemolysis during blood transfusion in patients with thalassemia?

BackgroundPatients with thalassemia need regular blood transfusions to maintain normal growth and suppression of ineffective erythropoiesis. Packed red blood cell (RBC) units can be delivered by infusion pumps (IPs); however, IPs may cause mechanical stress-induced RBC lysis. This study aimed to investigate the biomarkers of hemolysis related to transfusion techniques in patients with thalassemia. Material and methodsEighty-one thalassemia patients compared to those 42 healthy controls in terms of hemolysis markers (hemoglobin, plasma free hemoglobin (Hb), haptoglobin, potassium (K), lactate dehydrogenase (LDH)) before transfusion. Considering the age and peripheral venous diameter of the patient, the physician decided on the caliber of vascular access device (22 G or 24 G) for transfusion and the method to be used (gravitational method [GM] or IP). Hemolysis markers were repeated after transfusion in thalassemia patients. ResultsPacked RBC units were transfused to 24 (30 %) patients by IP and 57 (70 %) patients by GM. Plasma free Hb was significantly increased from 4.76 ± 7.92 mg/dL to 9.01 ± 7.66 mg/dL following transfusion (p < 0.001). There was no significant difference between IP and GM in terms of plasma free Hb increase. Post-transfusion plasma free Hb, LDH, and K levels significantly increased in patients who were transfused with 24 G catheters compared to those transfused with 22 G. ConclusionAn elevation in LDH levels was detected after transfusion with volumetric IPs; however, plasma free Hb or K levels were not affected by the transfusion method. Studies are needed to determine the factors associated with hemolysis after transfusion.

Role of myocardial deformation imaging in transfusion-dependent thalassemia—Correlation with severity of myocardial siderosis

ObjectivesTo study clinical and echocardiographic parameters in Transfusion Dependent Thalassemia (TDT) patients, correlating them with T2*CMRI. To identify features of Speckle Tracking Echocardiography (STE) in those with severe TDT to prioritize patients in need of early T2*CMRI and aggressive chelation. BackgroundHemosiderosis from life-long transfusions in TDT is amplified in Low- and Middle-Income Countries (LMIC) when access to resources may be scarce, delaying timely chelation. T2* imaging is the gold standard to non-invasively estimate myocardial iron and guide therapy. In resource constraint areas, we propose use of STE to prioritize those with severe disease to obtain early T2* CMRI. MethodsTDT patients (N = 25) who underwent clinical, echocardiographic, and CMRI evaluation were included, with recruitment of age matched controls. Echocardiographic indices of ventricular function and strain were correlated with T2*. Severe disease was defined as T2* < 10 ms. Differences among patients with severe disease and controls were analyzed in addition to differences between those with normal and abnormal T2*. ResultsThere was moderate (R −0.60, p = 0.002) inverse correlation between T2* and peak systolic global longitudinal strain (GLS) and regional basal strain (R −0.62, p < 0.001) indicating these parameters were lower in patients with severe disease when compared with controls and non-severe TDT group. GLS and most indices of regional strain were lower in patients with severe disease (T2* < 10 ms) when compared with the control group (p < 0.05). The Apex- base ratio reached significance (p = 0.02) in patients with abnormal T2*(<20 ms) when compared with normal T2*(<20 ms) in TDT positive patients. ConclusionsEarly decrease in global and regional strain can serve as a useful tool to identify patients with severe TDT before reduction in ejection fraction. This study makes a case for utilizing STE as an adjunct or initial screen to T2* CMRI in LMIC to prioritize patients in need of T2*CMRI to facilitate aggressive iron chelation protocols.

Prevalence of Kell Ag in Pregnant Women with Thalassemia Minor or Intermedia Beta in Medical Centers Affiliated to Shiraz University of Medical Sciences in 2019-2020

Background: Kell Ag, the main antigen of kell system has the greatest antigenicity after Rh antigen and the associated antibodies are very important. These antibodies are mainly of the Ig G class and can cause side effects like Hemolytic disease of the Fetus and Newborn (HDFN) or Hemolytic Transfusion Reaction (HTR), especially in patients with chronic blood transfusions such as thalassemia patients. The aim of this study was to evaluate Kell antigen in pregnant women with thalassemia minor and intermedia and to determine the appropriate strategy for transfusion in this group of patients Methods: This study is a cross-sectional study on pregnant women with thalassemia minor or intermedia. The samples were taken from patients of Shahid Motahari clinic and those with positive antigen were selected and statistically analyzed. Results: The results showed that from the 64 patients who entered this study, the results of kell ag of 58 patients were negative and 6 were positive. Therefore, 91% of the pregnant women with beta thalassemia minor or intermedia were negative for ag kell and 9% were positive. Conclusion: In summary, the results of this study indicated that a large percentage of pregnant women with beta-thalassemia minor or intermediate are Kell-negative, and due to the complications of alloimmunization, screening of pack cells and pregnant patients is very important.

ENGLISH INJECTION FOR THALASSEMIA: PELATIHAN BAHASA INGGRIS BAGI PENYANDANG THALASSEMIA BANYUMAS DI UNIVERSITAS NAHDLATUL ULAMA PURWOKERTO

Thalassemia is a hereditary disease caused by abnormalities in red blood cells in which the individual has a gene disorder that causes reduced production of hemoglobin in red blood cells. People with Thalassemia, especially the type of Thalassemia Major, need routine therapy and medication. These routine therapy and medication activities sometimes cause various problems and obstacles in the lives of people with thalassemia both physically and psychologically. Individuals with thalassemia also show impaired psychosocial and school functioning. The desire and enthusiasm for learning decreased, giving rise to human resources with low educational backgrounds. The lack of education especially the knowledge of health terms in English is one of the problems. The program of English Injection for Thalassemia intended to create an English learning space for people with Thalassemia. English Injection for Thalassemia aimed to provide English education, especially information about medical terms which are important in the treatment of thalassemia. The object of the program is the members of the Association of Indonesian Thalassemia Patients, commonly called PPTI Banyumas. The method of implementing this program was used fun learning methods and media. It provided English training in groups through indoor and outdoor activities with basic English expressions and English medical terms for Thalassemia

Prevalence of theDirect Antiglobulin Test and Its Clinical Impact on Multiply Transfused Thalassemia Patients: AProspective Study Conducted at aTertiary Care Center in Northern India.

This study was conducted to estimate prevalence of direct antiglobulin test (DAT) positivity and its impact on transfusion support in patients with thalassemia. The DAT testing was performed for patients with β-thalassemia who received transfusion from November 2021 to March 2022. Elution was done for DAT-positive samples. Of 180 patients, 21 (11.6%) were DAT positive. Immunoglobulin G (IgG) was present in 4 (19%) and IgG+C3d was present in 8 (38%). Only complement was present in 9 (42.8%) patients. The IgG-reactive DATs were associated with pan-reactive eluate. Patients who were DAT-positive had significantly higher levels of serum bilirubin, ferritin, and IgG than those who were DAT-negative. Autoantibody formation in multiply transfused thalassemia patients is common and merits equal attention as alloimmunization. It is particularly important as DAT-positive red bloodcells may undergo clinically significant hemolysis, which may increase the transfusion requirements with associated sequalae such as increased serum ferritin and splenomegaly.

Thalassaemia - part 1: a clinical update for the dental team.

Thalassaemia is a genetic disorder that affects haemoglobin function. It is an abnormality of haemoglobin caused by mutation of genes related to alpha or beta globin chains that can be further subdivided into categories. These haematological conditions can vary from mild forms, which present as mild anaemia, to severe forms, that can become life-threatening. The window for dental treatment is often limited by several factors involving medical management, such as the need for antibiotic cover or blood transfusions. The lifetime management of the medical conditions are onerous and can place significant physical and psychological burden on the patient. This paper is part of a two-part series on thalassaemia. Part one focuses on the clinical manifestations patients may present with, treatment regimens and dental implications of such presentations. Part two explores the perspectives of thalassaemia patients on their dental experience.

Evaluation of the efficacy of signal-averaged electrocardiogram testing in the cardiac assessment of beta-thalassemia major patients

BackgroundMore than 70% of thalassemia’s major mortality is due to the cardiac complications of this syndrome, mostly consequent to myocardial Iron overload; therefore, evaluation of such complications is of utmost importance. T2*MRI is used to assess hepatic and myocardial Iron load in thalassemia patients, which is not always available. Signal-Averaged Electrocardiography is a rather easy method of evaluating major thalassemia patients regarding their risk for sudden cardiac death.Methods and materialsIn this cross-sectional study, 48 patients with thalassemia major underwent evaluation with electrocardiography, signal-averaged electrocardiography, echocardiography, T2*MRI, and ferritin level. The association of the existence of ventricular late potentials in SAECG and other cardiac variables was evaluated. Moreover, the association between myocardial and hepatic Iron load and cardiac characteristics was assessed.Results48 patients with a mean age of 30.31 ± 7.22 years old entered the study. 27 (56.3%) of the patients had ventricular late potentials, which were associated with myocardial dry Iron weight (P = 0.011). Nonspecific ST-T changes and premature atrial and ventricular contractions were seen more frequently in patients with late potentials (P = 0.002, 0.031, and 0.031, respectively). Patients with higher myocardial and hepatic Iron loads had longer QTc in their 12-lead surface electrocardiograms.ConclusionPatients with ventricular late potentials assessed by SAECG had a higher myocardial Iron load. Higher myocardial Iron load is associated with higher cardiac complications in patients with beta-thalassemia major; therefore, SAECG can be used as a screening test for cardiac complications in beta-thalassemia major patients.

Pro‐haemostatic effect of DDAVP is partially derived through non‐classical (CD14dim/CD16++) monocytes residing the spleen

The splenic endothelial Weibel-palade bodies are one of the most important candidate organelles to release von Willebrand factor upon stimulation with desmopressin. However, the presence of functional desmopressin-specific receptor has not yet been demonstrated on endothelial cells. Experimental evidences are in favour of an indirect pro-haemostatic effect of desmopressin, but the exact mediator and its cellular origin are largely elusive. Here, we report partially hampered desmopressin response in a splenectomised severe haemophilia A/Beta Thalassemia patient without any genetic variant relevant to his incomplete desmopressin response. To further investigate the role of the spleen in this phenomenon, the release of VWF from desmopressin-treated human splenic endothelial cells was assessed in vitro. As a result, desmopressin induced the release of VWF from endothelial cells when the cells were co-cultured with non-classical (CD14dim /CD16++ ), but not other subtypes of monocytes or PBMCs. This in vitro study which resembles close proximity of endothelial cells of sinusoids to monocyte reservoir reside in parenchyma of subcapsular red pulp of the spleen sheds a light upon the role of this highly vascularized VWF-producing organ in driving indirect effect of desmopressin.

Assessment of lung function by spirometry in transfusion-dependent thalassemia patients in a tertiary care center in Sultanate of Oman

Introductionβ-thalassemia’s are hereditary chronic hemolytic diseases, the mainstay of treatment of thalassemia major is regular blood transfusion and iron chelation. They cause many complications, one of the recognized complications related to respiratory system is pulmonary hypertension. Respiratory functions in those patients are not well studied in most of the world. The studies done to assess respiratory function are inconsistent, some found a predominantly restrictive pattern, others found obstructive pattern, and few found normal spirometry. The aim of this study was to assess the spirometric patterns in asymptomatic Omani patients with transfusion-dependent thalassemia using spirometry studies. MethodsTransfusion-dependent thalassemia patients who are registered at Sultan Qaboos University Hospital who are > 15 years old and able to perform spirometry test were selected for the study after they signed the informed consent. All the patients were free of any respiratory disease. Spirometry was performed in all patients in the sitting position and FVC, FEV1, FEV1/FVC were obtained. ResultsTotal number of thalassemia patients enrolled in the study was 37. 32 patients are suffering from thalassemia major and 5 are suffering from thalassemia intermedia. The mean age of our patients was 29.95 years. We found that 37.8 % of the patients showed normal spirometry. Most patients had abnormal spirometry (62.1 %). Of these, 35.1 % showed a restrictive pattern while 27 % showed obstructive pattern. ConclusionSpirometry assessment of the lung function in thalassemia patients who are receiving regular transfusion showed that majority had abnormal spirometry results despite being asymptomatic from a respiratory point of view.

The Role of Splenectomy in The Treatment of β-Thalassemia Major Patients: A literature review

Thalassemia is a group of inherited blood disorders due to the reduction or absence of globin chain synthesis which can cause hemolytic anemia. β-thalassemia major is a severe type of thalassemia, in which patients require lifelong transfusions for survival. Extravascular hemolysis on the spleen results in splenomegaly, meanwhile, extramedullary hematopoiesis causing hypersplenism to develop in beta-thalassemia major patients. Hypersplenism, symptomatic splenomegaly, and increased annual need for blood transfusion in thalassemia patients are indicators of splenectomy. However, aside from the benefit of splenectomy which may reduce the need for transfusion in people with thalassemia with the goal of reducing iron overload, it may also be linked to several major negative effects that may outweigh any benefits, such as postoperative infections with high mortality rate and thrombotic events.

A rare and novel mutation in a beta-globin gene of thalassemia patient of Pakistan: A case report

Thalassemia is a genetically complex disorder that evolves from a mutation in the beta chain of hemoglobin. Much work has been done on the common mutations, but some rare mutations have been found that impact and diversify the disease spectrum. Our case report is on a young adult who presented with anemia, gall stones, and off-and-on transfusion dependency. A detailed workup revealed that the patient was suffering from thalassemia intermedia. The interesting finding was that the patient, product of non-consanguineous marriage was homozygous for beta thalassemia mutation on genetic analysis. A detailed genetic analysis of the parents revealed them as carriers for the same mutation. It was found that patient was homozygous for a rare and novel mutation -88(C>A)[HBB:c.-138C>A] on whole gene sequencing. The area of genomics in thalassemia is rapidly growing, and our case report aims to update the current knowledge of thalassemia's genomic information in Pakistan. The mutation found in our patient was -88(C>A)[HBB:c.-138C>A], and the data provided by the National Library of Medicine for this mutation as Allele ID: 380597 and variant type of single nucleotide variant shows that only ten such cases exist in the world with this rare mutation. Our case would be the 11th case in the world and 1st in Pakistan according to the literature, reporting above mentioned mutation. Further translational study is required to accurately utilize genomic data as an instrument of precision treatment in thalassemia patients, especially in underdeveloped countries like Pakistan.

Songs in the Key of Life

Songs in the Key of Life

Prevalence of Hepatitis B and C in Patients of Thalassemia in Sargodha, Pakistan

Background: Thalassemia is one of the commonest genetic hematological disorders and a health issue of global concern. Because of repeated blood transfusions, ratio of hepatitis B and C positivity is escalating in these patients day by day who have further complicated the situation in the form of poor treatment outcomes and costs to individuals Aim: To measure the prevalence of hepatitis B and C among patients of thalassemia major in Sargodha, Pakistan. Study design: Cross-sectional study Place and duration of study: Rai Hospital &amp; Hilal-e-Ahmar Hospital, Sargodha from 01-01-20221 to 31-12- 2021. Methodology: Three hundred and sixty thalassemia major patients, both beta and alpha, confirmed on hemoglobin electrophoresis admitted in the hospital were enrolled. Results: The mean age of the participants was 10.24±2.5 years, and 50% were male and 50% females. About 34% of the patients had hepatitis in which 30% had hepatitis C and 1.1% hepatitis B. 2.2% had both. Conclusion: Hepatitis b and C are highly prevalent among patients of thalassemia major. This also calls physicians, hematologists, pediatricians and policy makers to focus on counseling and support of thalassemia patients by providing them safe blood. Keywords: Prevalence, Hepatitis B and C, Thalassemia major, Blood transfusion

Prevalence of Dermatological Problems in Beta-Thalasemic Patients at Al-Khidmat Hospital, Peshawar

Background: Beta-thalassemia is the world's most prevalent single-gene disease with a reduction in the synthesis of hemoglobin b-chain. A lot of skins problems occur in thalassemia patients but, no study have been conducted in Pakistan to determine their prevalence. Objective: To determine the prevalence of dermatological problems in beta-thalasemic patients Methodology: This was cross sectional study carried out at the department of dermatology Al-Khidmat hospital Peshawar for a period of one year from August 2020 to August 2021. All the beta-thalassemia patients were examined for dermatological problems by a single expert dermatologist. All of the data was entered and analyzed by using the SPSS version 20 software. Results: Amongst 150 beta-thalasemic patients, 122 (81.33%) patients were observed with dermatological problems. The most frequent dermatological problem observed in our study was pruritus in 54 (36%) patients followed by xerosis in 34 (22.67%), urticaria in 30(20%), Pigmentary disorders in 33(22%) patient, freckles in 24(16%), tinea infections in 15(10%), pitriasis alba in 13 (8.67%) patients, scars in 13 (8.67%), hypersensitivity to deferoxamine pump in 12 (8%), herpes simplex in 12 (8%), Verruca vulgaris in 10 (6.67%), acne in 8 (5.33%) patients, miliaria in 8 (5.33%) while contact dermatitis was observed in 6(4%) of the patients. Conclusion: Our study concludes that dermatological problems are highly prevalent in beta-thalassemia patients with pruritus as the most common problem followed by xerosis. Keywords: Prevalence; Dermatological problems; Beta-thalasemia; Pruritus; Xerosis.

Height velocity and its relation with serum ferritin and pretransfusion haemoglobin in thalassemia patients: results from a tertiary center in Northeast India

Background: ẞ-thalassemia syndromes are the commonest single gene disorders in the Indian population and growth failure remains a major concern among these patients.Methods: We studied the relation of height velocity with serum ferritin and pretransfusion hemoglobin in 30 transfusion dependent thalassemia patients aged 1-12 years attending our centre. The height measurements were taken every 6 months for one year. Serum ferritin was measured 3 monthly and pretransfusion hemoglobin was measured at each visit.Results: The prevalence of impaired height velocity in transfusion dependent thalassemics in our study was 56.67%. The mean pretransfusion hemoglobin was 7.8±1.065 g/dl and the mean serum ferritin level in our study was 2426±872 ng/ml. In thalassemia patients with height velocity &lt; 3rd percentile mean serum ferritin level was 2637±892.84 ng/ml while the mean serum ferritin level in patients with height velocity &gt;3rd percentile was 1875±564.007 ng/ml with a statistically significant relation (p=0.012). Although the mean pretransfusion hemoglobin in children with height velocity &lt;3rd percentile was found to be lower compared to their counterparts with height velocity &gt;3rd percentile (7.42±0.879 g/dl vs. 7.96±0.998 g/dl), this relation was found to be statistically insignificant (p value=0.127).Conclusions: Our study highlights that even though regular blood transfusions are essential in thalassemic patients, maintaining an optimal ferritin level is equally essential in influencing growth in these children.

The effect of ferritin levels on distal femoral cartilage thickness in patients with beta thalassaemia major.

To the best of our knowledge, the present study is the first in the literature to assess distal femoral cartilage thickness and its relationship with ferritin levels in adult patients with beta thalassaemia major (BTM). 45 patients with BTM and 45 healthy controls were included in the study. Ferritin and haemoglobin levels of the patient and healthy groups were determined by blood analysis and distal femoral cartilage thicknesses were measured via ultrasound. Then, the patient group was divided into subgroups according to whether their ferritin levels were below or above 2500µg/L. They were then compared among themselves and with the healthy control group using the available data. Distal femoral cartilage thickness values were statistically significantly lower in the BTM group compared to the healthy control group (p values < 0.001). Patients with a ferritin level below 2500µg/L had statistically significantly higher right and left average distal femoral cartilage thickness values than the patients with a ferritin level above 2500µg/L (p = 0.029 and p = 0.019, respectively). The right and left average distal femoral cartilage thickness values of the patient subgroup with low ferritin levels were statistically similar to the control group (p = 0.146 and p = 0.164, respectively). Our study showed that thalassaemia patients are more likely to develop osteoarthritis (OA) than the normal population and possible OA development can be prevented by keeping the ferritin levels of these patients in the optimum range.

Assessment of disease knowledge gaps among beta thalassemia major patients and their caregivers

BackgroundThalassemia constitutes a major health issue in Egypt. It is associated with significant mortality and morbidity that arise mainly from iron overload secondary to blood transfusions.MethodsWe conducted this cross-sectional study at Cairo University children hospitals aiming at assessing the patients’ and their caregivers’ knowledge about thalassemia, and to identify factors affecting their knowledge in the period from December 2020 to August 2021.ResultsThe study included 45 thalassemia patients aged 10–14 years and 45 caregivers of patients who were less than 10 years of age. A knowledge questionnaire was generated from an educational script prepared by the investigators and translated into Arabic language. Thirty-one patients and 44 caregivers responded to the questionnaire individually, while 15 patient/parent pairs worked on the questionnaire together at their request. Age of patients ranged from 2 to 14 years, majority were males. Disease duration ranged from 0 to 13 years and the majority were following in the pediatric hematology clinic on a monthly basis. Candidates showed strong knowledge in areas related to symptoms and signs. On the other hand, they showed critical knowledge gaps in the areas of blood transfusions complications and iron chelation. Patients and caregivers scored similarly in various questionnaire topics with no significant difference. Gender, consanguinity, similar conditions in the family and frequent follow-up did not affect the score of the study participants, though longer duration of illness was positively correlated with the overall scores.ConclusionsThalassemia patients and their caregivers have significant knowledge gaps in areas of chelation therapy and transfusion complications.

Prediction of beta -Thalassemia carriers using complete blood count features

beta -Thalassemia is one of the dangerous causes of the high mortality rate in the Mediterranean countries. Substantial resources are required to save a beta -Thalassemia carriers’ life and early detection of thalassemia patients can help appropriate treatment to increase the carrier’s life expectancy. Being a genetic disease, it can not be prevented however the analysis of several indicators in parents’ blood can be used to detect disorders causing Thalassemia. Laboratory tests for Thalassemia are time-consuming and expensive like high-performance liquid chromatography, Complete Blood Count (CBC) with peripheral smear, genetic test, etc. Red blood indices from CBC can be used with machine learning models for the same task. Despite the available approaches for Thalassemia carriers from CBC data, gaps exist between the desired and achieved accuracy. Moreover, the data imbalance problem is studied well which makes the models less generalizable. This study proposes a highly accurate approach for beta -Thalassemia detection using red blood indices from CBC augmented by supervised machine learning. In view of the fact that all the features do not carry predictive information regarding the target variable, this study employs a unified framework of two features selection techniques including Principal Component Analysis (PCA) and Singular Vector Decomposition (SVD). The data imbalance between beta -Thalassemia carrier and non-carriers is handled by Synthetic Minority Oversampling Technique (SMOTE) and Adaptive Synthetic (ADASYN). Extensive experiments are performed using many state-of-the-art machine learning models and deep learning models. Experimental results indicate the superiority of the proposed approach over existing approaches with an accuracy score of 0.96.

Laporan Kasus Manajemen Kegawatdaruratan Trauma Jaringan Lunak Wajah Pada Anak Penderita Talasemia

Facial soft tissue trauma is a common case in the emergency department. Handling this case requires special attention because the face is a prominent part and a marker of identity. Thalassemia is a blood disorder that causes the sufferer to experience anemia, where thalassemia patients must undergo blood transfusions for life. This case will describe a 14-year-old girl who came to the Emergency Department of Hasan Sadikin Hospital Bandung with complaints of bleeding on the eyelids due to a motorcycle accident, from the patient's history it is known that the patient is a thalassemia patient who routinely undergoes transfusions every month. As a result of this hemorrhage caused a significant decrease in hemoglobin value in the patient, so that emergency management was carried out, namely suturing the patient's traumatized eyelid and blood transfusion. Emergency management of facial soft tissue trauma in children with thalassemia must be done carefully to control bleeding and restore the function and aesthetics of traumatized facial tissues, besides that we also collaborate with pediatric colleagues for the management of anemia due to thalassemia in this patient.