Alloimmunization in Egyptian children with sickle cell disease

2021 ◽  
Vol 46 (1) ◽  
pp. 41
Author(s):  
FatmaS.E Ebeid ◽  
MohsenS Elalfy ◽  
MahmoudA Kenny ◽  
FatimaZ.A Saed
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Egyptian Children

scholarly journals PREVALENCE OF HEPATITIS C AMONG EGYPTIAN CHILDREN WITH SICKLE CELL DISEASE AND THE ROLE OF IL28B GENE POLYMORPHISMS IN SPONTANEOUS VIRAL CLEARANCE

2016 ◽  
Vol 8 ◽  
pp. 2016007 ◽  
Author(s):  
Somaia Mohammed Mousa ◽  
Mona Kamal El-Ghamrawy ◽  
Mona Kamal El-Ghamrawy ◽  
Heba Gouda ◽  
Heba Gouda ◽  
...  
Keyword(s):  
Hepatitis C ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Gene Polymorphisms ◽  
Hcv Rna ◽  
Spontaneous Clearance ◽  
Cell Disease ◽  
Persistent Viremia ◽  
Egyptian Children ◽  
Hcv Antibody

Background and objectives: Hepatitis C virus (HCV) is a major health problem in Egypt with its prevalence estimated to be 14.7% among general population in 2008. Patients receiving frequent blood transfusions like sickle cell disease (SCD) are more exposed to the risk of acquiring HCV. IL28B gene polymorphisms have been associated with spontaneous HCV clearance. This study aims to determine the prevalence of HCV infection among children with SCD and to study the relation between IL28B gene polymorphisms and spontaneous HCV clearance.Methods: Seventy SCD patients were screened for HCV antibody. HCV positive patients were tested for the level of HCV RNA using quantitative real time PCR. IL28B polymorphisms (rs 12979860 SNP and rs 12980275 SNP) were detected using Taqman QRT-PCR and sequence specific primers PCR respectively. Results: Sixteen patients (23%) were HCV antibody positive, 9 of them (56.3%) had undetectable HCV RNA in serum and 7 (43.7%) had persistent viremia. Genotypes CC/CT/TT of rs12979860 were found in 30 (42.9%), 29 (41.4%) and 11 (15.7%) and rs12980275 AA/AG/GG were found in 8 (11.4%), 59 (84.3%) and 3 (4.3%).  There was no significant difference in the frequency of IL28B (rs 12979860 and rs12980275) genotypes among HCV patients who cleared the virus and those with persistent viremia (p=0.308 and 0.724 respectively). Conclusion: Egyptian SCD patients have high prevalence of HCV. Muti-transfused patients still exposed to a risk of transmission of HCV. IL28B gene polymorphisms are not associated with spontaneous clearance of HCV in this cohort of Egyptian children with SCD.

Prevalence of factor V Leiden (G1619A) and prothrombin gene (G20210A) mutation in Egyptian children with sickle cell disease

2012 ◽  
Vol 22 (4) ◽  
pp. 697-702
Author(s):  
Mona Salah El-Din Hamdy ◽  
Heba Mahmoud Gouda ◽  
Iman Abdel-Mohsen Shaheen ◽  
Mervat M. Khorshied ◽  
Rania Hosny Tomerak
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Factor V Leiden ◽  
Factor V ◽  
Cell Disease ◽  
G20210a Mutation ◽  
Egyptian Children ◽  
Prothrombin Gene

Acute Painful Crises of Sickle Cell Disease in Egyptian Children: Predictors of Severity for a Preventive Strategy

2006 ◽  
Vol 83 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Mohammad Al-Haggar ◽  
Hala Al-Marsafawy ◽  
Nabeel Abdel-Razek ◽  
Rizk Al-Baz ◽  
Abdel-Hamid Mostafa
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Preventive Strategy ◽  
Cell Disease ◽  
Egyptian Children

scholarly journals Ocular Manifestations in Egyptian Children and Young Adults with Sickle Cell Disease

2014 ◽  
Vol 30 (4) ◽  
pp. 275-280 ◽  
Author(s):  
Mona Kamal El-Ghamrawy ◽  
Hanan F. El Behairy ◽  
Amal El Menshawy ◽  
Seham A. Awad ◽  
Ahmed Ismail ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Ocular Manifestations ◽  
Egyptian Children ◽  
Children And Young Adults

scholarly journals Iron overload parameters and early detection of cardiac disease among Egyptian children and young adults with β-thalassaemia major and sickle cell disease: a cross-sectional study

F1000Research ◽  
2020 ◽  
Vol 9 ◽  
pp. 1108
Author(s):  
Khaled Salama ◽  
Amina Abdelsalam ◽  
Hadeel Seif Eldin ◽  
Eman Youness ◽  
Yasmeen Selim ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Iron Overload ◽  
Sickle Cell ◽  
Systolic Pulmonary Artery Pressure ◽  
Thalassaemia Major ◽  
Cell Disease ◽  
Cardiac Iron ◽  
Diastolic Velocity ◽  
Egyptian Children

Background: Cardiac, hepatic and pancreatic T2* measured by magnetic resonance imaging (MRI) has been proven to be an accurate and non-invasive method for measuring iron overload in iron overload conditions. There is accumulating evidence that pancreatic iron can predict cardiac iron in young children because the pancreas loads earlier than the heart. The aim of our study was to investigate cardiac function and cardiac iron and their relation to pancreatic iron among patients with β-thalassaemia major (βTM) and sickle cell disease (SCD). Methods: 40 βTM and 20 transfusion-dependant SCD patients were included along with 60 healthy age-matched controls. Echocardiography and Tissue Doppler Imaging were performed for all subjects as well as the control group.  Hepatic, cardiac and pancreatic iron overload in cases were assessed by MRI T2*. Results: The study group consisted of 40 βTM and 20 transfusion dependant SCD patients with mean age 13.7 years and mean frequency of transfusion/year 12. Mean cardiac T2* was 32.9 ms and mean myocardial iron concentration was 0.7 mg/g; One patient had cardiac iron overload of moderate severity. Mean pancreatic T2* was 22.3 ms with 20 patients having mild pancreatic iron overload. Pancreatic T2* correlated positively with main pulmonary artery diameter (p=0.046), peak late diastolic velocity at septal mitral annulus (p=0.038), peak early diastolic velocity at tricuspid annulus (p=0.001) and mitral annular plane systolic excursion (p=0.01); and negatively with end systolic pulmonary artery pressure (p=0.007). We couldn’t test the predictability of pancreatic T2* in relation to cardiac T2* as only one patient had cardiac T2*<20 ms. Conclusion: Assessment of pancreatic T2* in multi-transfused patients with βTM and SCD can predict myocardial dysfunction. No direct relation between pancreatic iron and cardiac siderosis was detected.

Alloimmunization in Egyptian Children with Sickle Cell Disease

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Mohsen Saleh Elalfy ◽  
Mahmoud Adel Kenny ◽  
Fatima Zahrra Abdelkarim Saed ◽  
Fatma Soliman Elsayed Ebeid
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Complete Blood Count ◽  
Blood Group Antigens ◽  
Cell Disease ◽  
Antibody Screening ◽  
Egyptian Children ◽  
Significant Difference ◽  
Detailed Medical History

Abstract 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.

scholarly journals βSglobin gene haplotype and the stroke risk among Egyptian children with sickle cell disease

Hematology ◽  
2017 ◽  
Vol 23 (6) ◽  
pp. 362-367 ◽  
Author(s):  
Heba H. Abou-Elew ◽  
Ilham Youssry ◽  
Shireen Hefny ◽  
Rania H. Hashem ◽  
Nevine Fouad ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Stroke Risk ◽  
Cell Disease ◽  
Egyptian Children ◽  
Gene Haplotype

Microalbuminuria among Egyptian Children and Adult with Sickle Cell Hemoglobinopathy, Single Institution Study.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3361-3361
Author(s):  
Wafaa E. Ibrahim ◽  
Ashraf M. Abdelmonem ◽  
Nermin H. Mahmoud ◽  
Waleed Mostafa
Keyword(s):  
Renal Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Ace Inhibitor ◽  
Sickle Cell Trait ◽  
Early Death ◽  
Cell Disease ◽  
Group Iii ◽  
Group I ◽  
Egyptian Children

Abstract Introduction: Renal failure is one of the most serious complications of sickle cell diseases; it affects 5% – 18% and leads to early death. Protienuria is highly correlated with risk for future chronic renal failure. Aim: to estimate the prevalence of microalbuminuria among Egyptian children and adults with sickle hemoglobinopathy, and to evaluate the effect ACE inhibitor on positive cases. Subject and method: 60 patients (mean age 13.03±9.15) with sickle hemoglobinopathy were included. Patients were divided into 3 groups: sickle cell disease (group I) constituted 20%, Sickle ß-Thalassemia (group II) 46.6% and sickle cell trait (group III) 33.4%. Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Microalbuminuria was measured by turbidimetric assay in 24 hour urine collection. Patients with microalbuminuria were started on a 4 weeks trial of ACE inhibitor after excluding any contraindication for the drug. Patients were closely monitored for side effects and repeat microalbuminuria assay was done at the end of the trial period. Data were coded, entered and processed using SPSS (version 11). Results: The prevalence of microalbuminuria was 32.5% in sickle cell disease, and sickle β thalassemia while in sickle cell trait was 5 % (p=0.02). The highest level of microalbuminuria (93.3+-146.27) was observed in sickle cell disease patients followed by sickle β thalassemia (63.82+-172.38) followed by sickle cell trait(18.75+-12.86). Compared to patients with no microalbuminuria, the group with positive microalbuminuria was significantly older (18.31±4.48 vs. 9.24± 4.96 years), had higher number of blood transfusions / year (7.08±3.55 vs. 5.11±4.05), higher mean number of hemolytic crisis (2.38±1.98 vs. 1.11±1.19), higher mean number of pain crisis (2.92±1.61 vs. 2.2±1.48), and higher mean serum creatinine (0.63±0.31 vs. 0.47±0.19) (p<0.01). After 4 weeks trial of ACE inhibitor there was a statistically significant reduction (77.9% reduction) in number of cases with microalbuminuria as well as level of microalbuminuria (dropped from 171.91 ± 251.60 to 27.11 ± 13.99) (p= 0.005). Conclusion: Microalbuminuria is a significant problem among patients with sickle cell disease. ACE inhibitors have significant impact on improving microalbuminuria.

scholarly journals Factors associated with abnormal cerebral blood flow in Egyptian children with sickle cell disease

2020 ◽  
Vol 4 (1) ◽  
pp. 2514183X2091135
Author(s):  
Foad Abd-Allah ◽  
Mona Eltagui ◽  
Alshaimaa Mahmoud Aboulfotooh ◽  
Nirmeen Adel Kishk ◽  
Mohammad Ahmed Farrag ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Linear Regression Analysis ◽  
Cell Disease ◽  
Predictive Tool ◽  
Cross Sectional ◽  
Transfusion Therapy ◽  
Egyptian Children ◽  
Annual Frequency

Background: Transcranial Doppler (TCD) is a well-established tool for cerebrovascular assessment. Estimating the flow velocity across the intracranial arteries helps to identify children with sickle cell anaemia who are at risk for stroke. Objective: Our aim is to correlate TCD findings with clinical condition in children with sickle cell disease (SCD) to determine the value of TCD assessment as a predictive tool for stroke in SCD and to identify any association of TCD findings with disease severity, transfusion therapy and treatment administered. Methods: Eighty-five paediatric SCD patients aged from 3 years to 18 years of both genders who were followed up at the Hematology Clinic of New Children’s Hospital at Cairo University were included in this cross-sectional observational study. All our participants underwent routine laboratory investigations and TCD assessments. Results: Oof the 85 patients, two patients (2.3%) died before completing the TCD study and eventually 83 patients were included in the analysis. Seventeen (20.5%) patients had abnormal TCD findings, seven (8.4%) patients showed high-risk findings and 10 (12.1%) patients had conditional flow pattern. Logistic linear regression analysis confirmed that annual frequency of blood transfusion and hydroxyurea (HU) dose were associated with a decreased risk of abnormal TCD findings. Conclusion: The current study demonstrates that our TCD data reproduce the findings of other studies and that it is very likely the results from large trials are applicable for Egyptian children. The annual frequency of blood transfusion and HU dose were associated with a decreased frequency of abnormal TCD findings.

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