Repeated Blood Transfusions Research Articles

Assessment of B2-Microglobulin Analysis for Renal Insufficiency in Iraqi β-Thalassemia Major Patients

Background: Beta-thalassemia is the most prevalent genetic hemoglobin apathy in the world. It is caused by a reduction or absence of beta-globin chain production, which is typically a portion of adult hemoglobin (HbA, which is α2β2). This genetic anomaly will lead to a rapid erythrocyte turnover, severe anemia, and compensatory ineffective erythropoiesis. The purpose of the current research is to reveal diagnostic, and predictive biomarkers that can be performed to detect the decline in renal function in β-thalassemia major patients with early stage renal impairment with high sensitivity and specificity, ascertain changes in B2-microglobulin as a biomarker in β-thalassemia patient. Objective: The samples were collected from Karama Hospital-Genetic Hematology Center/Baghdad during the period from the 1st of September 2022 until the 1st of January 2023. Methods: The patient group consisted of 45 patients with β-thalassemia major with repeated blood transfusion and the control group consisted of 45 individuals who seemed to be healthy. Age ranged between (18-35) years (for patients and control). The serum samples were used to measure biochemical parameters, S.Creatinine, blood urea, and Urine B2-microglobulin were measured from urine samples. B2-microglobulin level in Urine was estimated by Sandwich enzyme-linked immunosorbent assay (ELISA) technique, while serum Blood Urea and Serum Creatinine concentration were calculated by Colorimetric Kit by Spectrophotometer method, and Ferritin level in blood was estimated by BioMrieux Mini Vidas. Results: The statistical examination was carried out using SPSS software. B2-microglobulin biomarkers in the thalassemia Patient group were significantly higher than those in the control group (p<0.001). While serum ferritin, B.Urea and S.Cr increased in patient groups compared to the control group (p<0. 005). Conclusions: All parameters included in this study are significantly higher in β-thalassemia patients than in healthy subjects. Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among β-thalassemia major patients with repeated blood transfusions, which are not found in routine renal investigations.

Iron overload and liver function in patients with beta thalassemia major: A cross sectional study.

In thalassemia major, repeated blood transfusions result in iron overload causing organ damage. The objective of this study was assessment of liver enzymes in patients with Thalassemia major and to observe their association with ferritin. A cross-sectional study was performed, at Islamabad Medical and Dental College and its affiliated Akbar Niazi Teaching Hospital from November 2021 till August 2022. Serum ferritin, AST, ALT, and total bilirubin levels were determined, in 135 patients of beta thalassemia major receiving transfusions. Data analysis was performed using SPSS Version 20. For categorical variables, calculation of frequencies and percentages was performed. Mean (± standard deviation) was determined for quantitative variables. ANOVA with post hoc Tukey's test was used for determining association between liver enzymes and serum ferritin. A p-value of <0.05 was considered significant. The correlation between ferritin and LFTs was determined by Pearson's correlation coefficient. Patients had an age range of 7-30 years, and males constituted 51% of sample. Mean level of ferritin was 6062.61 + 3641.79 ng/ml, with an insignificant difference between the genders (p =0.366). The levels of AST, ALT and bilirubin were perceived to show a significant increase in patients with ferritin levels >5000ng/ml, when compared with patients having ferritin levels < 2,500 ng/ml. A significant positive correlation of increasing serum ferritin levels was observed with ALT (r= 0.682), to a lesser extent with AST (r = 0.532), and only a weak correlation with serum bilirubin (r = 0.350). Liver damage was caused by increased iron deposition. LFTs should be performed regularly to detect and reduce liver damage by increasing chelation therapy, thereby reducing morbidity and mortality due to thalassemia.

Iron Overload Cardiomyopathy

Iron overload cardiomyopathy is a condition of excessive iron accumulation in cardiomyocytes due to abnormal iron absorption or repeated blood transfusion. In the early stages, the patient may be asymptomatic with good ventricular systolic function. Iron deposit in ventricular cause dyspnea on effort due to left ventricular systolic dysfunction then in atrial cause atrioventricular block and supraventricular arrhythmic. For severe symptom due to dilated cardiomyopathy is characterized by left ventricular dilatation and risk of sudden cardiac death. Diagnosis of iron overload cardiomyopathy can be made if there is evidence of heart disease, the presence of iron overload (serum ferritin &gt; 300 ng/mL and transferrin saturation &gt; 55%) and cardiac siderosis with cardiac MRI T2 * &lt; 20 ms as gold standard. Patient management involves lowering systemic iron levels and preventing iron entry into cardiomyocytes. Therapy with phlebotomy or iron chelation as indicated. Administration of calcium channel blockers and resveratrol antioxidant therapy may be considered to reduce morbidity and mortality due to cardiac siderosis.

Role of Quercetin Supplementation on Iron Parameters in Blood Transfusion-Dependent Thalassemia Patients

Background: Thalassemia is a group of inherited blood disorders that affect the production of hemoglobin, a protein in red blood cells that carries oxygen throughout the body. Iron overload is a condition in which the body absorbs and stores too much iron. In addition to repeated blood transfusions, increased gastrointestinal tract (GIT) iron absorption plays an important role in iron overload with thalassemia. Quercetin, a common flavonoid present in fruits and vegetables, exhibits diverse biological effects. Objective: To assess the effect of quercetin on iron overload parameters in blood transfusion-dependent thalassemia patients (TDT). Methods: A randomized, double-blind, placebo-placebo group-led study was conducted on 110 TDT patients, more than 12 years of age, who were supplemented with either quercetin or a placebo capsule daily (500 mg) for 3 months. A blood sample was obtained for laboratory parameters at baseline and at the end of 3 months. Results: At the baseline time of the study, the demographic features and iron overload parameters of patients and the placebo group were not statistically different, while after three months of supplementation, there was a significant decrease in levels of serum iron, UIBC, serum ferritin and ferritin saturation rate, and a significant increase in TIBC in the patients compared with the placebo group. Conclusions: The study shows the significant role of quercetin on iron overload parameters in blood transfusion-dependent thalassemia patients.

Clinico-Laboratory Profile of Hypertriglyceridemia Thalassemia Syndrome: A Case Series in a Paediatric Tertiary Care Centre.

Increased hemolysis and repeated blood transfusion trigger oxidative stress resulting in numerous adverse effects in beta-thalassemia patients. Extreme elevation of triglyceride level is a rare clinical entity seen in these patients. It is presumed to be caused due to an increase in oxidative stress and is termed Hypertriglyceridemia Thalassemia Syndrome. To assess the clinical and laboratory characteristics of beta-thalassemia patients presenting with hypertriglyceridemia and its correlation with the pre-transfusion hemoglobin level. Methods: This hospital record-based retrospective study was conducted at the Dr B C Roy Post Graduate Institute of Paediatric Sciences, Kolkata, India. The study comprised 12 pediatric beta-thalassemia patients whose plasma appeared milky or chylous during a complete hemogram. Clinical examination and laboratory investigations were done to describe their clinico-laboratory features. A whole exome sequencing was carried out to assess their genetic background. Blood hemoglobin and serum triglyceride estimation was carried out initially and at follow-up to determine any correlation between the two. Results: Out of 1482 patients, 12 (0.80 %) were diagnosed with Hypertriglyceridemia Thalassemia Syndrome. The median age of presentation was 12.5 months (Q1:10 months, Q3:14 months)., and the pretransfusion hemoglobin was 4.82 ± 1.16 g/dL. The lipid profile showed a triglyceride level of 858.3 ± 198.4 mg/dl and a total cholesterol level of 117.4± 16.15 mg/dl. Analysis revealed that the triglyceride levels were negatively correlated with the pretransfusion hemoglobin level (repeated measures correlation (rmcorr) = -0.65, 95% CI[-0.794, -0.425], p < 0.001). A genetic study highlighted c.92+5G>C as the commonest mutation. Hypertriglyceridemia was a rare presentation in transfusion-dependent beta-thalassemia patients. The serum triglyceride level significantly reduced when blood transfusion at regular intervals restored the patient's hemoglobin level.

Is postoperative high dependency care really needed for children undergoing supratentorial brain tumour surgery?

Purpose We present our analysis of the existing Paediatric High Dependency Unit (HDU) admission policy at our institution and discuss our thoughts for its revision in the context of paediatric supratentorial tumour surgery. Materials and methods We screened our prospectively maintained database of all children undergoing supratentorial craniotomy for resection of paediatric brain tumours over a fifteen-year period. The post-operative course of each patient was reviewed, assessing the number of patients who had true HDU needs in the immediate post-operative period, and the relative depth of input from paediatric HDU specialists that each patient received. Results Forty-three patients underwent craniotomy for supratentorial tumour resections during the study period. The median age of the children was 8 years old. Forty-two patients in the study cohort did not require any HDU-level monitoring or treatment post-operatively; all these patients were able to be discharged from HDU to a standard ward bed very rapidly post-operatively. Only one patient (2%) from the study cohort had any tangible HDU needs in the acute post-operative period, comprising of invasive cardiovascular monitoring and repeated blood transfusions. This child’s tumour was known to be large, highly vascular, and invasive pre-operatively. Conclusions We would advocate a rational and nuanced approach with regards to predicting which children are most likely need paediatric HDU care following supratentorial craniotomy for resection of a brain tumour. This rationalisation could improve resource availability and reduce financial burdens upon paediatric neurosurgical units.

Hubungan Transfusi Darah Berulang dan Kepatuhan Konsumsi Obat Kelasi Besi dengan Kadar Ferritin pada Pasien Thalasemia di Kota Depok

Repeated transfusions cause iron overload in β thalassemia patients. Therefore, iron chelation therapy is needed to reduce the amount of ferritin and ions in the blood. Compliance with iron chelation therapy is one of the factors that affect the patient's serum ferritin level. This study aims to determine the relationship between repeated blood transfusions and consumption of iron chelating drugs with serum ferritin levels of β thalassemia patients at Depok City Hospital in 2021. This research is a retrospective cohort study. The initial population was 112 people, sampling was carried out using the non-probability method and obtained 69 people who met the inclusion and exclusion criteria. This research was conducted from January 2021 to December 2021 in three medical record installations at Depok City Hospital. Respondents who rarely did repeated blood transfusions were 42 (60.9%), respondents who adhered to taking iron chelation drugs were 45 (65.2%), and respondents who had high ferritin levels were 43 (62.3%). There is a relationship between repeated blood transfusions and ferritin levels in thalassemia patients in Depok City in 2021. There is a relationship between adherence to taking iron chelating drugs and ferritin levels in thalassemia patients in Depok City in 2021. It is recommended that clinicians continue to provide a deeper understanding to parents or the patient's family to continue to carry out regular chelation therapy and check serum ferritin levels regularly.

The relation between serum ferritin level and echocardiographic findings in transfusion dependent thalassemic patients in Slemani City, Kurdistan Region of Iraq.

Background and objectives: Thalassemia patients are at risk of iron overload. Those with transfusion-dependent thalassemia are more likely to experience cardiac problems because repeated blood transfusions cause iron accumulation in the cardiac myocyte. Thus, this study aims to determine the relationship between serum ferritin levels and echocardiographic findings in transfusion-dependent thalassemia patients. Methods: This retrospective analytical cross-sectional study was conducted on 131 patients with transfusion-dependent thalassemia between 1stDecember 2021 to 1stJuly 2022 at the Center for Thalassemia and Congenital Blood Disorder, Slemani, Iraq. The participant’s information, including sociodemographic characteristics such as age, gender, body weight, height, hemoglobin level, serum ferritin, type and duration of chelation therapy, and echocardiographic findings, were collected from the hospital database using a questionnaire. Then, the correlation between serum ferritin levels and echocardiographic results was determined. Results: The mean age of the patients was 21.24 ± 6.28 years, and most of them were males (55.7%), from the urban region (71%) and had an average body weight (68.7%). The mean serum ferritin level was 1876±1743.63 µg/L. The interpretation of the echocardiography results showed 38 patients (29%) had valvular heart diseases. Body mass index has a reverse linear relationship with ejection fraction(P=0.038) and a direct relationship with age(P&lt;0.001). However, no significant association was found between echocardiographic findings and serum ferritin(P=0.83). Conclusions: We found valvular heart diseases among the most common heart disorders in the patients. Suitable interventions and measures can significantly help manage the condition and reduce illness complications.

Autosomal Dominant Hereditary Spherocytosis In An Elderly Patient

Hereditary spherocytosis (HS) is an autosomal dominant inherited hemolytic anemia that usually manifests in early adolescence. A very small proportion (5%) may undergo de novo mutations and present at an elderly age without any positive family history. The disease is characterized by anemia, jaundice, splenomegaly, and the presence of spherocytes in peripheral blood, which are osmotically fragile. A 55-year-old elderly male presented with generalized weakness and a history of repeated blood transfusions for 5 months. He had recurrent jaundice for 8 years. There was no relevant family or drug history. He was anemic, icteric, and had mild splenomegaly. Investigation revealed persistently low Hb with normal red cell indices. There were a lot of spherocytes with polychromasia on the peripheral blood film. There was also evidence of hemolysis with a high reticulocyte count and higher levels of total and indirect bilirubin. Autoimmune hemolysis was excluded by negative direct and indirect Coomb’s tests, and Hb defect was excluded by normal Hb electrophoresis. We also ruled out other infectious causes. The ultrasound confirmed splenomegaly. The osmotic fragility test showed increased osmotic fragility of the patient’s red cells. History, symptoms, and test results strongly point to autosomal dominant hereditary spherocytosis with new mutations. An eosin-5-Maleimide binding test was advised, along with family screening of the patient. Bangladesh J Medicine 2024; 35: 110-113

Role of magnetic resonance imaging (MRI) in liver iron quantification in thalassemic (thalassemia major) patients

BackgroundIron overload is a major problem in beta thalassemia patients due to repeated blood transfusions. The liver is the first organ to be loaded with iron. An accurate assessment of iron overload is necessary for managing iron chelation therapy in such patients. Iron quantification by MRI scores over liver biopsy due to its non-invasive nature.MethodsFifty-one patients with thalassemia major were subjected to 3.0-T MRI. Multiecho T2* sequence was used to cover the entire liver. Region of interest (ROI) was placed in three areas with maximum signal change, and an average T2* value was obtained. Similarly, a single ROI was placed at the mid-interventricular septum in the heart, and T2* value was obtained. T2* values so obtained were converted to iron concentration with the help of a T2* iron concentration calculator. The liver iron values were correlated with serum ferritin value.ResultsThere was a significant negative correlation between liver iron concentration (LIC) and T2* value of the liver (r = − 0.895, p < 0.01) and between cardiac iron concentration (CIC) and T2* value of the heart (r = − 0.959, p < 0.01). There was a slight positive correlation between LIC and serum ferritin (r = 0.642, p < 0.01) and no correlation between CIC and serum ferritin (r = − 0.137, p = 0.354).ConclusionsMRI is a useful tool to titrate the doses of chelating agents as it is accurate and non-invasive, does not involve radiation hazards and hence can be repeated as and when needed. Simultaneous assessment of cardiac iron overload is an added advantage of MRI.

Growth and endocrinopathies among children with β-Thalassemia major treated at Dubai Thalassemia centre

Backgroundβ-Thalassemia major (BTM) is one of the most common hereditary anemias worldwide. Patients suffer from iron overload that results from repeated blood transfusion This in turn leads to multiple organ damage and endocrinopathies. This study aims to assess the prevalence of growth retardation, hypothyroidism, and diabetes mellitus in children and adolescents with BTM treated at Dubai Thalassemia Centre.MethodsA total of 105 children and adolescents were included in this retrospective observational study.Results39 children and 66 adolescents’ data were analyzed. Females composed 51.3% (n = 20) of children and 53.0% (n = 35) of adolescents. Pretransfusion hemoglobin below 9 gm/dl was observed in 10.8% (n = 4) and 10.6% (n = 7) in children and adolescents, respectively. The mean age of menarche was 13.5 years. Among all study participants, 22.6% (n = 14) had normal height velocity whereas 37.1% (n = 23) had reduced height velocity in one year and 40.3% (n = 25) had reduced height velocity in two consecutive years. The proportion of children and adolescents showing reduced height velocity was significantly higher in females compared to the males (90.6% versus 63.3%, respectively, Chi-square = 6.597, p-value = 0.010). Although none of the study participants had diabetes mellitus, 26.1% (n = 12/46) had pre-diabetes. Elevated TSH was observed in 14.7% (n = 5) children and 8.1% (n = 5) adolescents while low FT4 was reported in one child and one adolescent.ConclusionOf all endocrinopathies seen among children and adolescents with BTM, growth delay remains the main concern for this group of patients. Effective treatment is key to further reducing endocrinopathies. Although the sample size is limited, we postulate that the low percentage of endocrinopathies among children with BTM treated at Dubai thalassemia center and the low level of pretransfusion anemia reflect the effective transfusion and chelation at the center.

Blood Transfusion cross match on the basis of Phenotype &amp; Rh Antigen in Donors and Receivers and Analysis of Variations

Background: In brief, knowing the local Rh blood group system frequency helps develop a donor pool for patients who need numerous transfusions and alloantibody-compatible antigen negative blood. Patients have been matched for component blood transfusions using ABO and Rh phenotypes since 2020. Our detection of all blood transfusion-needy patients began. Aim: Our department began To detect C, E, c, and e Rh-specific antigens in multi-transfused patients in 2020. Methods: For the aim of this investigation, 2300 patient samples were obtained from patients who required clinical blood transfusions at our hospital between March 2020 and October 2022. These samples were gathered for the purpose of this investigation. One patient was counted as a single sample even though they required repeated blood transfusions. 1900blood donor samples were provided by the Blood Centre of (duplicated samples were removed based on the identification numbers that were provided by the Blood Centre once they were identified). Results: The study obtained 4200 samples, including 1900donor and 2300patient samples. The allele frequency distribution of blood group antigens in the studied population was compared with the prevalence observed in the present study. The D antigen exhibited a frequency of 99.34% in the studied population, slightly lower than the prevalence observed in the present study at 98.9% (95% CI: 98.5 - 99.0). Conversely, the C antigen was found in 93.1% of the studied population, with a slightly higher prevalence observed in the present study at 99.1% (95% CI: 92.0 - 99.2). Implication: The study suggests serological testing is a cost-effective method for blood transfusion management, identifying Rh phenotypes. Compiling a database of donor Rh genotypes simplifies transfusion selection, reducing unfavorable responses. Pre-transfusion Rh phenotype examination is crucial for matching patient blood type with donor blood, reducing adverse reactions, and improving patient safety. Conclusion: In transfusion applications, serological testing are cost-effective for Rh phenotype identification but not genotypes. Creating a database of blood donors' Rh phenotypes and evaluating each patient before their initial transfusion should lessen adverse reactions and speed up antigen-negative blood availability, saving more patients. Keywords: Blood transfusion, phenotype, Rh antigen, donors, receivers, analysis of variations

A 38 Week and 2 Day G1P0A0 Primigravida in the First Stage of Labor with Thalassemia: A Case Report

Thalassemia is one of the most prevalent inherited blood disorders worldwide. We reported a case of 20-year-old G1P0A0 pregnant woman diagnosed with thalassemia intermedia since childhood based on chronic anemia, facies Cooley, and family history. She had 6 times red blood cell transfusion throughout current pregnancy. On 38 week 2 day gestation, she was admitted with chief complaint of painful regular uterine contractions and bloody show. Physical and laboratory findings were low hemoglobin, hematocrit, MCV, and MCH. Thalassemia can be alpha or beta type, depending on the affected globin chain. Clinical severity varies. The main therapies are repeated blood transfusions and iron chelation. During pregnancy, optimal evaluation and management are crucial to prevent maternal and fetal complications. This patient will undergo cesarean section and needs thorough high-risk antenatal care.

CORRELATIONS BETWEEN FERRITIN LEVELS AND CREATININE IN PEDIATRIC THALASSEMIA PATIENTS PRODIA CILEGON

Thalassemia is a blood disorder that is passed down genetically from parents to their children. The body cannot form normal red blood cells resulting in them being damaged easily and having a short life of less than 120 days and anemia, requiring continuous blood transfusions to maintain hemoglobin levels in the body. A history of repeated blood transfusions can cause excess iron in the body which is characterized by an increase in serum ferritin levels which can cause damage to kidney function by looking at creatinine levels. The purpose of this study was to determine whether there is a relationship between ferritin and creatinine levels in children with thalassemia. This study used a quantitative method with a cross sectional approach. The sampling technique used in this study was the total sampling technique. The sample used was 12 samples. Data processing in this study used the Shapiro-Wilk Normality test and the Pearson Correlation test in bivariate analysis. The results of the analysis of this study indicate that there is no significant relationship between the ferritin and creatinine levels in children with thalassemia where the value of Sig. 0.212 &gt; 0.05 (p&gt; 0.05), and shows a significant negative correlation of -0.389. This study in thalassemia children had high ferrtin levels but creatinine levels were still within normal limits.

Adherence to Hydroxyurea and Patients' Perceptions of Sickle Cell Disease and Hydroxyurea: A Cross-Sectional Study.

Background and Objectives: Hydroxyurea is a crucial treatment for sickle cell disease (SCD), but some patients' adherence to it remains suboptimal. Understanding patients' perspectives on SCD and HU is essential for improving adherence. This study aimed to assess hydroxyurea adherence and patients' perceptions of SCD and hydroxyurea among SCD patients in the Jazan region of Saudi Arabia. Materials and Methods: This cross-sectional study collected data from 217 SCD patients using self-administered questionnaires from August 2022 to January 2023. The survey covered patient demographics, SCD consequences, and other clinical data. We used the Brief Illness Perception Questionnaire (B-IPQ) to measure patients' disease perception and the 8-item Morisky Medication Adherence Scale (MMAS-8) to evaluate patients' adherence to HU. Data were analysed using descriptive, t-test, and chi-square tests, and the p-value was set at <0.05 for significance. Results: More than half of the patients were male, with a mean age of 28.09 ± 8.40 years. About 57.6% of the patients were currently using HU. About 81.6% of HU users reported low adherence. The adherence was lower among individuals with infections/recurrent infections and in patients who received repeated blood transfusions. ICU admission, blood transfusion, and certain SCD complications were associated with HU use. Male patients had a higher perception of SCD consequences, concern, and understanding. ICU-admitted and recurrent hospitalized patients had a higher perception of the SCD-related consequences, symptoms, concerns, and emotional responses. Conclusions: HU seems a well-established and efficacious disease-modifying agent, but its underutilization for SCD patients remains challenging. To overcome the adherence challenges, healthcare providers must educate SCD patients about the role of hydroxyurea in lowering disease severity and addressing side effects to obtain maximum benefits. Healthcare providers may consider tailored educational interventions to improve adherence, particularly for patients with infections, recurrent hospitalizations, or repeated blood transfusions. Further research is needed to identify strategies for improving hydroxyurea adherence and patient education among SCD patients.

Efficacy and safety of deferasirox in thalassemia major patients after repeated blood transfusion: A single-center study

Background: Patients with β-thalassemia major (TM) require lifelong blood transfusions, leading to iron overload, where active intervention is required. Aims and Objectives: The aim of the study was to evaluate real-world efficacy and safety of deferasirox (DFX) in TM patients at a tertiary care teaching hospital. Materials and Methods: A prospective observational study was conducted at thalassemia ward in a tertiary care teaching hospital in Gujarat for a period of 15 months from April 2021 to July 2022. A total of 100 patients were analyzed during the study period. Patient’s demographic profile, laboratory investigation, and treatment details were analyzed using descriptive statistics. Statistical analysis was done through the statistical software GraphPad Prism 9.4.1 (681). Results: A total of 100 patients were included in the study. A serial significant decrease in median serum ferritin levels was observed in &gt;13 years age group after initiation of DFX. A significant increase in the median dose of DFX was observed at 12 months in the age group of 2–≤13 years as compared to baseline (P &lt; 0.05). Adverse reactions observed during the study were increased bilirubin level, increased alanine aminotransferase, and increased serum creatinine, rashes with itching, and arthralgia. Conclusion: A greater reduction in median serum ferritin levels was observed in the adult age group compared to pediatric patients.

Efficacy of intensive therapy for massive intraoperative blood loss in children: experience of the Morozov Hospital

To evaluate the effectiveness of intensive therapy for massive intraoperative blood loss in children. A retrospective analysis of primary medical records of 39 children with massive intraoperative blood loss was performed. Patients were divided into two groups (younger 1 year (n=18) and older 1 year (n=21)). Each group was divided into two subgroups (blood loss <10% and >100% of total blood volume). We analyzed total intraoperative infusion, qualitative composition of transfusions, reinfusion of washed autologous erythrocytes and vasopressor support. In postoperative period, we assessed hemoglobin, platelets, albumin, fibrinogen, lactate, prothrombin index, duration of mechanical ventilation, severity of organ dysfunction (pSOFA score) after 1 and 3 days, ICU stay and incidence of repeated blood transfusions. With regard to transfusion volume, we found a general pattern (3 parts of crystalloids, 2 parts of erythrocyte-containing components and 1 part of fresh frozen plasma in all groups with the exception of children older 1 year with blood loss >100% of total blood volume. The last ones had ratio 3:5:1 due to large volume of reinfusion of washed autologous erythrocytes. In all groups, target levels of hemoglobin, platelets, albumin and prothrombin index were achieved. Serum fibrinogen was slightly lower in the group with blood loss >100% of total blood volume. There was a direct relationship between blood loss and ICU stay (Spearman's test rs=0.421, p<0.05), as well as duration of mechanical ventilation (Spearman's test rs=0.509, p<0.05). Mean pSOFA score upon admission to intensive care unit was 3-4 points in both groups with blood loss <100% of total blood volume. In patients with blood loss >100% of total blood volume, this indicator averaged 9 points and regressed to 3-4 points over the next 72 hours. Intraoperative intensive therapy contribute to minimal severity of postoperative organ dysfunction in children with blood loss < 100% of total blood volume and rapid regression of multiple organ failure in patients with blood loss exceeding this indicator.

Investigating the level of Hba1c and insulin level in β-thalassemia patients

Background: β-thalassemia is an inherited blood disorder characterized by reduced or no synthesis of β globin chain, resulting in chronic anemia, so blood transfusion is required as curative therapy. Repeated blood transfusions lead to iron overload that can lead to multiple organ damage, including pancreatic organs.Objective: This study aimed to describe HbA1c and insulin levels of β-thalassemia patients. The study also tested whether there was a significant difference in insulin and HbA1c levels among patients with different β-thalassemia categories. Method: This research was an analytic observational study. The samples were taken by total sampling and involved 30 patients, and the examination was carried out using the patient's blood plasma.Result: The study found that samples had low HbA1c levels. There was no significant mean difference (p&gt;0.05) between insulin and HbA1c in the mild, moderate, and severe clinical degree groups. There was no significant difference in average (p&gt;0.05) insulin and HbA1c in the thalassemia sufferers with allele β+ and β0 types.Conclusion: There was a decrease in insulin and HbA1c in patients with β-thalassemia, which may be caused by damage pancreatic organ damage.

Frequency of hepatitis C virus infection in patients with pediatric inflammatory bowel disease: a cross-sectional study

BackgroundPatients with pediatric inflammatory bowel disease (PIBD) are believed to be at higher risk of hepatitis C virus (HCV) infection not only due to using of immunosuppressive drugs but also because of repeated blood transfusion and endoscopic and other invasive procedures used for diagnoses and effective controlling of the disease, so this study aimed to assess the frequency of HCV infection, in patients with PIBD at the New Children’s Hospital, Cairo University, and identify the potential risk factors.MethodsThis cross-sectional analytic study included 165 IBD patients between 1 and 16 years old of both sexes who were attending the Pediatric Gastroenterology Clinic in the New Children’s University Hospital, Cairo University. All patients were screened for anti-HCV antibodies using ELISA. Factors related to IBD (severity, modalities of the treatment, and invasive procedures), to infection (blood transfusion history and family history of hepatitis), and liver enzymes were registered. The risk factors were evaluated by multivariate logistic regression analysis.ResultsPresent and/or past HCV infection was found in five (3%) of the IBD patients. The multivariate logistic regression to detect independent predictors of HCV +ve antibodies patients had statistically significant value with number of hospital admission related to IBD with p-value = 0.002, odd ratio (OR) = 1.467, and confidence interval (CI) = 95% (1.145–1.879) and with number of hospital admission unrelated to IBD with p-value = 0.024, OR = 0.750, and CI 95% (0.585–0.963).ConclusionThe frequency of HCV infection in PIBD patients was 3%. Thus, the frequency of HCV infection in PIBD patients is similar to that in the normal population of the developing countries, and it is strongly related to hospital admission due to IBD or non-IBD causes.

Progress and development of CRISPR-Cas tool in the treatment of thalassemia

-thalassemia is the worlds major hemoglobin disorders. It is a hereditary chronic hemolytic anemia caused by a break in the -globin peptide chain genes of hemoglobin, resulting in reduced or complete absence of synthesis of the -globin peptide chains. Patients with -thalassemia rely on blood transfusions for survival, which exacerbates the disease burden on national economies and the financial burden on patients in developing countries. Repeated blood transfusions can also lead to organ damage. The fetuss protein genes are usually silenced early and then replaced by adult protein genes. But mutations that cause gammaglobin to persist in the fetus can improve the electrocution of globin. Therefore, reactivating the protein genes of the fetus is the core of research. CRISPR/Cas9 is the common way to reduce the effects of disease by correcting disease-causing mutations, increasing or disrupting protein expression. CRISPR/Cas9 and considered effective new methods for generating hemoglobin. Hence, the main focus of this review is to explore the details of CRISPR/Cas9 in-thalassemia treatment in the context of Mediterranean -thalassemia.