QJM: An International Journal of Medicine, Oct 1, 2021
Background Transfusion is critical in the management of sickle cell disease (SCD) complications. ... more Background Transfusion is critical in the management of sickle cell disease (SCD) complications. The resultant alloimmunization to RBC group antigens is a major complication of allogeneic blood transfusion and generally presents significant challenges in the management of SCD patients. Aim To measure the frequency of the occurrence of the alloimmune markers in sickle cell disease and to investigate its predicators. Subjects and methods This cross-sectional study included 50 children and young adults with SCD, all patients were subjected to detailed medical history thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis and serum ferritin. The D, C, c, E, e, M, NJKa and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s and Anti Lua antigens were typed by commercially prepared polyclonal anti- human sera. Antibody screening was performed using Ortho screening panel. Results The study Sickle SS was the most common type of SCD representing more than half of the recruited patients. Frequency of antibody positive screening among SCD was 16%. Comparison between SCD patients with positive and those with negative antibody screening showed that higher frequency of positive antibody screening in those with SB+ than those SS and SB0. Patient with positive antibodies screening were significantly older when they received their first blood transfusion and had lower transfusion index (p = 0.037) than those with negative antibodies screening (p=0.013). There were no significant difference between patients with positive and those with negative antibodies screening as regards age, gender, transfusion frequency and as regards comorbidities; painful crisis (p=0.117), stroke (p=0.398), ACS (p=0.363). Conclusion The distribution of the blood group antigens in patients with SCD has important clinical significance especially those who often require regular blood transfusion and who may have developed multiple antibodies. The resultant alloimmunization is serious necessitates early recognition and management.
Background Platelet glycoprotein VI (GPVI) receptor is essential for platelet adhesion and aggreg... more Background Platelet glycoprotein VI (GPVI) receptor is essential for platelet adhesion and aggregation. Eltrombopag is as an effective treatment for chronic immune thrombocytopenia (ITP); yet, its effect on platelet function is not fully characterized. Aim This prospective study investigated the effect of eltrombopag therapy on platelet function through assessment of GPVI receptor expression and soluble GPVI levels among pediatric patients with persistent or chronic ITP. Methods Thirty-six children and adolescents with persistent or chronic ITP were divided equally into two groups either to receive eltrombopag therapy or the standard of care. All patients were followed-up for 12 months with assessment of bleeding score and complete blood count (CBC). Evaluation of GPVI expression using flow cytometry and measurement of its soluble form by ELISA was done at baseline and at 6 months. Results ITP patients on eltrombopag had significantly lower bleeding score after 6 months of therapy w...
Objectives Pancreatic reserve could be preserved by early assessment of pancreatic iron overload ... more Objectives Pancreatic reserve could be preserved by early assessment of pancreatic iron overload among transfusion-dependent sickle cell disease (SCD) patients. This study aimed to measure pancreatic iron load and correlate its value with patients’ laboratory and radiological markers of iron overload. Materials and methods Sixty-six SCD children and young adults underwent MRI T2* relaxometry using a simple mathematical spreadsheet and laboratory assessment. Results The results indicated moderate-to-severe hepatic iron overload among 65.2% of studied cases. None had cardiac iron overload. Normal-to-mild iron overload was present in the pancreas in 86% of cases, and 50% had elevated serum ferritin > 2500 ug/L. There was no significant correlation between pancreatic R2* level, serum ferritin, and hepatic iron overload. Patients with higher levels of hemolysis markers and lower pre-transfusion hemoglobin levels showed moderate-to-severe pancreatic iron overload. Conclusion Chronicall...
Long-term safety and efficacy data on the iron chelator deferiprone in sickle cell disease (SCD) ... more Long-term safety and efficacy data on the iron chelator deferiprone in sickle cell disease (SCD) and other anemias are limited. FIRST-EXT was a 2-year extension study of FIRST (Ferriprox in Patients With Iron Overload in Sickle Cell Disease Trial), a 1-year, randomized noninferiority study of deferiprone vs deferoxamine in these populations. Patients who entered FIRST-EXT continued to receive, or were switched to, deferiprone. Altogether, 134 patients were enrolled in FIRST-EXT (mean age: 16.2 years), with mean (SD) exposure to deferiprone of 2.1 (0.8) years over the 2 studies. The primary end point was safety. Secondary end points were change in liver iron concentration (LIC), cardiac T2∗, serum ferritin (SF), and the proportion of responders (≥20% improvement in efficacy measure). The most common adverse events considered at least possibly related to deferiprone were neutropenia (9.0%) and abdominal pain (7.5%). LIC (mg/g dry weight) decreased over time, with mean (SD) changes fro...
Background:Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairme... more Background:Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairment which may be due to iron overload in brain tissue or hemoglobin polymerization and endothelial dysfunction.Primary objectivewas measuring brain iron content (using R2* values) in the caudate and thalamic regions through quantitative brain MRI in Egyptian adolescents and young adults with multi-transfused SCD in comparison to beta thalassemia major (BTM) and age- and sex-matched healthy controls.Secondary objectiveswere evaluating the impact of brain iron content on neurocognitive functions of SCD patients and its association with MRI assessment of liver iron concentration (LIC) and cardiac iron (myocardial T2*).Methods: 32 children and young adults with SCD (mean age: 15.3 ± 3.7, 19 males and 13 females), 15 BTM (mean age: 19.4 ± 4.3, 7 males and 8 females) and 11 healthy control age- and gender-matched were recruited. Thorough clinical assessment, hematological and serum ferritin wer...
Many people with sickle cell disease (SCD) or other anemias require chronic blood transfusions, w... more Many people with sickle cell disease (SCD) or other anemias require chronic blood transfusions, which often causes iron overload that requires chelation therapy. The iron chelator deferiprone is frequently used in individuals with thalassemia syndromes, but data in patients with SCD are limited. This open-label study assessed the efficacy and safety of deferiprone in patients with SCD or other anemias receiving chronic transfusion therapy. A total of 228 patients (mean age: 16.9 [range, 3-59] years; 46.9% female) were randomized to receive either oral deferiprone (n = 152) or subcutaneous deferoxamine (n = 76). The primary endpoint was change from baseline at 12 months in liver iron concentration (LIC), assessed by R2* magnetic resonance imaging (MRI). The least squares mean (standard error) change in LIC was −4.04 (0.48) mg/g dry weight for deferiprone vs −4.45 (0.57) mg/g dry weight for deferoxamine, with noninferiority of deferiprone to deferoxamine demonstrated by analysis of co...
Aims Measure frequency, severity, and etiology of epistaxis in a tertiary center pediatric out-pa... more Aims Measure frequency, severity, and etiology of epistaxis in a tertiary center pediatric out-patient clinic. Population Pediatric patients presented with epistaxis of an unknown cause. Methods Thorough clinical assessment, ENT examination CBC and coagulation profile and confirmatory tests when needed. Results 100 patients with epistaxis (53 females and 47 males) were recruited out of 30043 visits to pediatric OPC throughout one year (1 in 300). Most of them were younger than 12 and nearly half of them were younger than 6 years. 43 patients had epistaxis only, most probably local bleeding (37 had idiopathic epistaxis, 6 had local ENT cause). 57 had other bleeding manifestations, most probably systemic bleeding (47 had a definite bleeding disorder and 10 had undiagnosed bleeding tendency). There was a statistically significant higher number of patients with a positive family history for epistaxis and being referred from a primary care physician in those with probably systemic cause....
BackgroundSurfactant protein D (SP‐D) is considered a candidate biomarker for lung integrity and ... more BackgroundSurfactant protein D (SP‐D) is considered a candidate biomarker for lung integrity and for disease progression.AimWe determined the level of SP‐D in children and adolescents with SCD and assessed its possible relation to pulmonary complications and lung function.MethodsSerum SP‐D levels were assessed in 50 SCD patients compared with 30 healthy controls. High‐resolution computerized tomography (HRCT) of the chest was done. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), FEV1/FVC% and forced expiratory flow rate during 25‐75% of expiration (FEF25‐75%) were determined.ResultsSP‐D was significantly higher in SCD patients than controls, particularly patients with sickle cell anemia than those with sickle β‐thalassemia. SP‐D levels were significantly associated with increasing severity of interstitial lung disease. The highest SP‐D levels were observed among patients with restrictive lung disease followed by mixed type then obstructive lung disease. SP‐D was...
Purpose: COVID-19 is the most recent pandemic causing morbidity and mortality. Although a part of... more Purpose: COVID-19 is the most recent pandemic causing morbidity and mortality. Although a part of the pathogens causing SARI, it is unique in causing pulmonary thrombosis and lung fibrosis. Thus different management is needed. We aimed o explore the start of SARS-CoV-2 in preserved SARI samples to know the exact time of its emergence in our hospital, to conduct whole-genome sequencing in positive SARS-CoV-2 samples to define its strain. To assess the clinical characteristics of the severe respiratory infection admitted to El-Demerdash hospitals in that same period from our own file reports.Methods: We conducted a retrospective cohort study among SARI patients who were admitted to Ain Shams university hospitals. preserved nasopharyngeal& oropharyngeal swabs from 333 SARI patients were used in SARS-CoV-2 detection by RT-Real time PCR. Moreover, whole-genome sequencing of SARS-CoV-2 positive samples was performed. Clinical characteristics of the SARI patients were analyzed in the same ...
QJM: An International Journal of Medicine, Oct 1, 2021
Background Transfusion is critical in the management of sickle cell disease (SCD) complications. ... more Background Transfusion is critical in the management of sickle cell disease (SCD) complications. The resultant alloimmunization to RBC group antigens is a major complication of allogeneic blood transfusion and generally presents significant challenges in the management of SCD patients. Aim To measure the frequency of the occurrence of the alloimmune markers in sickle cell disease and to investigate its predicators. Subjects and methods This cross-sectional study included 50 children and young adults with SCD, all patients were subjected to detailed medical history thorough clinical examination. Laboratory investigations included complete blood count, markers of hemolysis and serum ferritin. The D, C, c, E, e, M, NJKa and JKb antigens were typed using monoclonal antisera. Fya, Fyb, Jka, Jkb, S, s and Anti Lua antigens were typed by commercially prepared polyclonal anti- human sera. Antibody screening was performed using Ortho screening panel. Results The study Sickle SS was the most common type of SCD representing more than half of the recruited patients. Frequency of antibody positive screening among SCD was 16%. Comparison between SCD patients with positive and those with negative antibody screening showed that higher frequency of positive antibody screening in those with SB+ than those SS and SB0. Patient with positive antibodies screening were significantly older when they received their first blood transfusion and had lower transfusion index (p = 0.037) than those with negative antibodies screening (p=0.013). There were no significant difference between patients with positive and those with negative antibodies screening as regards age, gender, transfusion frequency and as regards comorbidities; painful crisis (p=0.117), stroke (p=0.398), ACS (p=0.363). Conclusion The distribution of the blood group antigens in patients with SCD has important clinical significance especially those who often require regular blood transfusion and who may have developed multiple antibodies. The resultant alloimmunization is serious necessitates early recognition and management.
Background Platelet glycoprotein VI (GPVI) receptor is essential for platelet adhesion and aggreg... more Background Platelet glycoprotein VI (GPVI) receptor is essential for platelet adhesion and aggregation. Eltrombopag is as an effective treatment for chronic immune thrombocytopenia (ITP); yet, its effect on platelet function is not fully characterized. Aim This prospective study investigated the effect of eltrombopag therapy on platelet function through assessment of GPVI receptor expression and soluble GPVI levels among pediatric patients with persistent or chronic ITP. Methods Thirty-six children and adolescents with persistent or chronic ITP were divided equally into two groups either to receive eltrombopag therapy or the standard of care. All patients were followed-up for 12 months with assessment of bleeding score and complete blood count (CBC). Evaluation of GPVI expression using flow cytometry and measurement of its soluble form by ELISA was done at baseline and at 6 months. Results ITP patients on eltrombopag had significantly lower bleeding score after 6 months of therapy w...
Objectives Pancreatic reserve could be preserved by early assessment of pancreatic iron overload ... more Objectives Pancreatic reserve could be preserved by early assessment of pancreatic iron overload among transfusion-dependent sickle cell disease (SCD) patients. This study aimed to measure pancreatic iron load and correlate its value with patients’ laboratory and radiological markers of iron overload. Materials and methods Sixty-six SCD children and young adults underwent MRI T2* relaxometry using a simple mathematical spreadsheet and laboratory assessment. Results The results indicated moderate-to-severe hepatic iron overload among 65.2% of studied cases. None had cardiac iron overload. Normal-to-mild iron overload was present in the pancreas in 86% of cases, and 50% had elevated serum ferritin > 2500 ug/L. There was no significant correlation between pancreatic R2* level, serum ferritin, and hepatic iron overload. Patients with higher levels of hemolysis markers and lower pre-transfusion hemoglobin levels showed moderate-to-severe pancreatic iron overload. Conclusion Chronicall...
Long-term safety and efficacy data on the iron chelator deferiprone in sickle cell disease (SCD) ... more Long-term safety and efficacy data on the iron chelator deferiprone in sickle cell disease (SCD) and other anemias are limited. FIRST-EXT was a 2-year extension study of FIRST (Ferriprox in Patients With Iron Overload in Sickle Cell Disease Trial), a 1-year, randomized noninferiority study of deferiprone vs deferoxamine in these populations. Patients who entered FIRST-EXT continued to receive, or were switched to, deferiprone. Altogether, 134 patients were enrolled in FIRST-EXT (mean age: 16.2 years), with mean (SD) exposure to deferiprone of 2.1 (0.8) years over the 2 studies. The primary end point was safety. Secondary end points were change in liver iron concentration (LIC), cardiac T2∗, serum ferritin (SF), and the proportion of responders (≥20% improvement in efficacy measure). The most common adverse events considered at least possibly related to deferiprone were neutropenia (9.0%) and abdominal pain (7.5%). LIC (mg/g dry weight) decreased over time, with mean (SD) changes fro...
Background:Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairme... more Background:Children with sickle cell disease (SCD) are at a high risk for neurocognitive impairment which may be due to iron overload in brain tissue or hemoglobin polymerization and endothelial dysfunction.Primary objectivewas measuring brain iron content (using R2* values) in the caudate and thalamic regions through quantitative brain MRI in Egyptian adolescents and young adults with multi-transfused SCD in comparison to beta thalassemia major (BTM) and age- and sex-matched healthy controls.Secondary objectiveswere evaluating the impact of brain iron content on neurocognitive functions of SCD patients and its association with MRI assessment of liver iron concentration (LIC) and cardiac iron (myocardial T2*).Methods: 32 children and young adults with SCD (mean age: 15.3 ± 3.7, 19 males and 13 females), 15 BTM (mean age: 19.4 ± 4.3, 7 males and 8 females) and 11 healthy control age- and gender-matched were recruited. Thorough clinical assessment, hematological and serum ferritin wer...
Many people with sickle cell disease (SCD) or other anemias require chronic blood transfusions, w... more Many people with sickle cell disease (SCD) or other anemias require chronic blood transfusions, which often causes iron overload that requires chelation therapy. The iron chelator deferiprone is frequently used in individuals with thalassemia syndromes, but data in patients with SCD are limited. This open-label study assessed the efficacy and safety of deferiprone in patients with SCD or other anemias receiving chronic transfusion therapy. A total of 228 patients (mean age: 16.9 [range, 3-59] years; 46.9% female) were randomized to receive either oral deferiprone (n = 152) or subcutaneous deferoxamine (n = 76). The primary endpoint was change from baseline at 12 months in liver iron concentration (LIC), assessed by R2* magnetic resonance imaging (MRI). The least squares mean (standard error) change in LIC was −4.04 (0.48) mg/g dry weight for deferiprone vs −4.45 (0.57) mg/g dry weight for deferoxamine, with noninferiority of deferiprone to deferoxamine demonstrated by analysis of co...
Aims Measure frequency, severity, and etiology of epistaxis in a tertiary center pediatric out-pa... more Aims Measure frequency, severity, and etiology of epistaxis in a tertiary center pediatric out-patient clinic. Population Pediatric patients presented with epistaxis of an unknown cause. Methods Thorough clinical assessment, ENT examination CBC and coagulation profile and confirmatory tests when needed. Results 100 patients with epistaxis (53 females and 47 males) were recruited out of 30043 visits to pediatric OPC throughout one year (1 in 300). Most of them were younger than 12 and nearly half of them were younger than 6 years. 43 patients had epistaxis only, most probably local bleeding (37 had idiopathic epistaxis, 6 had local ENT cause). 57 had other bleeding manifestations, most probably systemic bleeding (47 had a definite bleeding disorder and 10 had undiagnosed bleeding tendency). There was a statistically significant higher number of patients with a positive family history for epistaxis and being referred from a primary care physician in those with probably systemic cause....
BackgroundSurfactant protein D (SP‐D) is considered a candidate biomarker for lung integrity and ... more BackgroundSurfactant protein D (SP‐D) is considered a candidate biomarker for lung integrity and for disease progression.AimWe determined the level of SP‐D in children and adolescents with SCD and assessed its possible relation to pulmonary complications and lung function.MethodsSerum SP‐D levels were assessed in 50 SCD patients compared with 30 healthy controls. High‐resolution computerized tomography (HRCT) of the chest was done. Forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), FEV1/FVC% and forced expiratory flow rate during 25‐75% of expiration (FEF25‐75%) were determined.ResultsSP‐D was significantly higher in SCD patients than controls, particularly patients with sickle cell anemia than those with sickle β‐thalassemia. SP‐D levels were significantly associated with increasing severity of interstitial lung disease. The highest SP‐D levels were observed among patients with restrictive lung disease followed by mixed type then obstructive lung disease. SP‐D was...
Purpose: COVID-19 is the most recent pandemic causing morbidity and mortality. Although a part of... more Purpose: COVID-19 is the most recent pandemic causing morbidity and mortality. Although a part of the pathogens causing SARI, it is unique in causing pulmonary thrombosis and lung fibrosis. Thus different management is needed. We aimed o explore the start of SARS-CoV-2 in preserved SARI samples to know the exact time of its emergence in our hospital, to conduct whole-genome sequencing in positive SARS-CoV-2 samples to define its strain. To assess the clinical characteristics of the severe respiratory infection admitted to El-Demerdash hospitals in that same period from our own file reports.Methods: We conducted a retrospective cohort study among SARI patients who were admitted to Ain Shams university hospitals. preserved nasopharyngeal& oropharyngeal swabs from 333 SARI patients were used in SARS-CoV-2 detection by RT-Real time PCR. Moreover, whole-genome sequencing of SARS-CoV-2 positive samples was performed. Clinical characteristics of the SARI patients were analyzed in the same ...
Uploads
Papers by Fatma Ebeid