Multi-center study on mortality in children, and adults with sickle cell anemia-risk factors and causes of death.

Sickle cell disease (SCD) is a major public health burden worldwide with increasing morbidity and mortality. The study evaluates the risk factors associated with mortality in SCD patients, between the years 2006 and 2020 at three hospitals in Oman. The analysis includes clinical manifestations, haematological, biochemical, and radiological parameters, use of antibiotics, and blood and exchange transfusions.

Impact of splenectomy on circulating microparticles in patients with sickle cell anemia.

Introduction: Circulating microparticles (MP) are being described as potential biomarkers for disease activity in a variety of conditions including sickle cell anemia (SCA). However, relatively little is known about the influence of spleen status on MP levels in patients with SCA.

Methods: Using a prospective study design we characterize circulating MP in 144 patients with SCA in steady state by assessing their cellular origin and their relationships to spleen status defined by clinical and imaging findings.

Influence of Voxelotor-hemoglobin complexes in the estimation of hemoglobin S levels by the current standard of care laboratory evaluation techniques.

Background: Sickle cell disease is an inherited disorder characterized by the presence of sickle hemoglobin (HbS). The process of Hb molecule polymerization is a pivotal step in the sickling process. Voxelotor, a recently approved novel therapeutic agent, is known to interfere with polymerization.

Novel missense mutation (A300P) causing pyruvate kinase deficiency in an Omani Kindred-PK deficiency masquerading as congenital dyserythropoietic anemia.

We report herein a child with transfusion-dependent chronic anemia, the cause of which was difficult to establish because of his transfusion dependency. The clinical and laboratory features suggested a chronic nonspherocytic hemolytic anemia (CNSHA) with bone marrow features suggestive of congenital dyserythropoietic anemia (CDA). DNA studies, however, revealed the underlying condition to be due to a novel mutation in the gene responsible for pyruvate kinase deficiency (PKD).

Predicting risk factors for thromboembolic complications in patients with sickle cell anaemia - lessons learned for prophylaxis.

Objective: To assess the clinical and laboratory predictors of venous thromboembolism (VTE) in patients with sickle cell anaemia (SCA) and its relationship to morbidity and mortality.

Methods: This retrospective case-control study analysed data from patients with SCA that experienced VTE compared with matched control patients with SCA but no VTE (2:1 ratio).

Results: A total of 102 patients with SCA were enrolled (68 cases with VTE and 34 controls).

Iron Overload in Patients With Heavily Transfused Sickle Cell Disease-Correlation of Serum Ferritin With Cardiac T2 MRI (CMRTools), Liver T2 MRI, and R2-MRI (Ferriscan®).

The treatment of sickle cell disease (SCD) is mainly supportive, except for a minority, who receive bone marrow transplantation (BMT). Serum ferritin (SF) is routinely available but is notoriously unreliable as a tool for iron-overload assessment since it is an acute-phase reactant. Although blood transfusion is one of the most effective ways to deal with specific acute and chronic complications of SCD, this strategy is often associated with alloimmunization, iron overload, and hemolytic reactions.

Measurement of hematopoietic progenitor cells using XN2000 hematology analyzer.

Introduction: Stem cell enumeration by the hematopoietic progenitor cells (HPC) mode is a novel method available from Sysmex XN2000 hematology analyzer. A small amount of blood (190 μL) is required, and the results are available in a few minutes without manual gating or presample treatment. The present study compares stem cell measurements using XN2000 analyzer HPC mode and FC500 flow cytometry analyzer using peripheral blood (PB) specimens and apheresis products.

Respiratory Viral Infections in Sickle Cell Anemia: Special Emphasis on H1N1 Co-infection.

Objectives: Patients with sickle cell anemia (SCA) are immunocompromised and at an increased risk of developing infections. Our aim was to establish the clinical, laboratory, and radiological manifestations of respiratory viral infections in SCA at Sultan Qaboos University Hospital (SQUH), Oman and assess its impact on disease morbidity and mortality, with special emphasis on H1N1.

Methods: We undertook a retrospective study in SCA patients with respiratory viral infections following up at the hematology department at SQUH.

Predictors of impending acute chest syndrome in patients with sickle cell anaemia.

Acute chest syndrome (ACS) is a major complication of sickle cell anaemia (SCA) and a leading cause for hospital admissions and death. We aimed to study the spectrum of clinical and laboratory features of ACS and to assess the predisposing factors and predictors of severity. A retrospective case-control cohort was studied by retrieving patient information from electronic medical records after ethical approval.

Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients.

Unlabelled: Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records.

Prevalence of Hepatitis B, Hepatitis C, and HIV in Multiply Transfused Sickle Cell Disease Patients from Oman.

Background: In Oman, the prevalence of hepatitis B (HBV) infection is 5.8%, with 2.8-7.

Effect of CYP4F2, VKORC1, and CYP2C9 in Influencing Coumarin Dose: A Single-Patient Data Meta-Analysis in More Than 15,000 Individuals.

The cytochrome P450 (CYP)4F2 gene is known to influence mean coumarin dose. The aim of the present study was to undertake a meta-analysis at the individual patients level to capture the possible effect of ethnicity, gene-gene interaction, or other drugs on the association and to verify if inclusion of CYP4F2*3 variant into dosing algorithms improves the prediction of mean coumarin dose. We asked the authors of our previous meta-analysis (30 articles) and of 38 new articles retrieved by a systematic review to send us individual patients' data.

Fresh frozen plasma (Octaplas) and topical heparin in the management of ligneous conjunctivitis.

Ligneous conjunctivitis is a rare form of chronic recurrent membranous conjunctivitis with reduced plasminogen activity. It is characterized by the formation of characteristic firm ("woody") membranes on the tarsal conjunctiva. Similar lesions may occur on other mucous membranes.

Immunophenotypic Characteristics of T-Acute Lymphoblastic Leukemia in Omani Patients: A Correlation with Demographic Factors.

Objectives: To study and classify the immunophenotypic characteristics of Omani patients diagnosed with T-cell acute lymphoblastic leukemia (T-ALL) and to correlate the results with age and gender as well as biological factors (peripheral and bone marrow blast cells percentage).

Methods: Fifty cases from both genders and of all ages who fulfilled the inclusion criteria with a diagnosis of T-ALL were included in the study. Correlation of T-ALL subtypes with age, gender, and initial bone marrow and peripheral blood blast cells percentage was assessed using ANOVA.

Portal Vein Thrombosis in Adult Omani Patients: A Retrospective Cohort Study.

Objectives: We sought to study the occurrence of portal vein thrombosis (PVT) in adult Omani patients. Methods: We conducted a retrospective cross-sectional study in patients diagnosed with PVT, which was confirmed by radiological imaging, from two tertiary hospitals over a 10-year period. Results: Amongst the 39 patients enrolled in the study, 15 (38.

Impact of Educational Activities in Reducing Pre-Analytical Laboratory Errors: A quality initiative.

Objectives: Pre-analytic errors during diagnostic laboratory investigations can lead to increased patient morbidity and mortality. This study aimed to ascertain the effect of educational nursing activities on the incidence of pre-analytical errors resulting in non-conforming blood samples.

Methods: This study was conducted between January 2008 and December 2015.

First Cytogenetic Profile of Omani Patients with Myelodysplastic Syndromes: Comparison with data from Asia, Africa, Europe and North and South America.

Clonal cytogenetic abnormalities have been reported among 30-80% of patients with myelodysplastic syndromes (MDS); however, 20-70% of patients with MDS show a normal karyotype that may nevertheless harbour a cryptic genetic alteration. Earlier reports have suggested that the distribution of specific chromosomal aberrations varies among Western and Asian countries, with geographical and ethnic differences in the frequency of specific chromosomal aberrations. This article compared the cytogenetic data of 36 adult Omani patients with MDS to previously reported data from other populations.

Regulatory B Cells Are Functionally Impaired in Patients Having Hemophilia A With Inhibitors.

Development of inhibitors remains a major clinical complication in patients with hemophilia A receiving replacement therapy with factor VIII (FVIII). Understanding the immune mechanisms involved in the development of inhibitors can provide valuable information about pathways to human tolerance. Recent evidence indicates that B regulatory (Breg) cells play a pivotal role in controlling the production of antibodies (Abs) while promoting follicular T helper (Tfh) cells and monocytes, expressing the low-density lipoprotein receptor-related protein (LRP/CD91), which is involved in FVIII intake from the circulation.

Alloimmunization in Patients with Sickle Cell Disease and Thalassemia: Experience of a Single Centre in Oman.

Background: Blood transfusion is an integral part of the supportive care for patients with sickle cell disease (SCD) and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy.

Objectives: The aim of this study was to evaluate the prevalence of red cell alloimmunization in Omani patients with sickle cell anaemia and thalassemia.

Impact of Mannose-Binding Protein Gene Polymorphisms in Omani Sickle Cell Disease Patients.

Objectives: Our aim was to study mannose-binding protein (MBP) polymorphisms in exonic and promoter region and correlate it with associated infections and vasoocculsive (VOC) episodes in sickle cell disease (SCD) patients since MBP plays an important role in innate immunity by activating the complement system.

Methods: We studied the genetic polymorphisms in the Exon 1 (alleles A/O) and promoter region (alleles Y/X; H/L, P/Q) of the MBL2 gene, in SCD patients as an increased incidence of infections is seen in these patients. A PCR-based, targeted genomic DNA sequencing of MBL2 was used to study 68 SCD Omani patients and 44 controls (healthy voluntary blood donors).

Red Cell Alloimmunization to Rhesus Antigen Among Pregnant Women Attending a Tertiary Care Hospital in Oman.

Objectives: The detection of maternal alloimmunization against red cell antigens is vital in the management of hemolytic disease of the fetus and newborn. We sought to measure the presence of allosensitization to Rhesus D (RhD) antibodies in antenatal women attending a tertiary care hospital and assess the fetal outcome in sensitized women. 


Methods: We conducted a retrospective review of pregnant Omani women who registered at the Sultan Qaboos University Hospital between June 2011 and June 2013.

Serum Total Bilirubin, not Cholelithiasis, is Influenced by UGT1A1 Polymorphism, Alpha Thalassemia and β(s) Haplotype: First Report on Comparison between Arab-Indian and African β(s) Genes.

Background: We explored the potential relationship between steady state serum bilirubin levels and the incidence of cholelithiasis in the context of UGT1A1 gene A(TA)nTAA promoter polymorphism in Omani sickle cell anemia (SCA) patients, homozygotes for African (Benin and Bantu) and Arab-Indian β(S) haplotypes, but sharing the same microgeographical environment and comparable life style factors.

Methods: 136 SCA patients were retrospectively studied in whom imaging data including abdominal CT scan, MRI or Ultrasonography were routinely available. Available data on the mean steady state hematological/biochemical parameters (n=136), β(s) haplotypes(n=136), α globin gene status (n=105) and UGT1A1 genotypes (n=133) were reviewed from the respective medical records.

Comparison of Methicillin Resistant Staphylococcus Aureus in Healthy Community Hospital Visitors [CA-MRSA] and Hospital Staff [HA-MRSA].

Background: The prevalence of community-associated methicillin-resistant Staphylococcus aureus [CA-MRSA] is unknown in Oman.

Methods: Nasal and cell phones swabs were collected from hospital visitors and health-care workers on sterile polyester swabs and directly inoculated onto a mannitol salt agar containing oxacillin, allowing growth of methicillin-resistant microorganisms. Antibiotic susceptibility tests were performed using Kirby Bauer's disc diffusion method on the isolates.