Determination of vWF, ADAMTS-13 and Thrombospondin-1 in Venous Thromboembolism and Relating Them to the Presence of Factor V Leiden Mutation.

Thrombophilia in venous thromboembolism (VTE) is multifactorial. Von Willebrand factor (vWF) plays a major role in primary hemostasis. While elevated vWF levels are well documented in VTE, findings related to its cleaving protease (ADAMTS-13) are contradicting.

Neutrophil gelatinase-associated lipocalin as a biomarker of nephropathy in sickle cell disease.

Sickle cell nephropathy (SCN) develops via altered hemodynamics and acute kidney injury, but conventional screening tests remain normal until advanced stages. Early diagnostic biomarkers are needed so that preventive measures can be taken. This study evaluates the role of neutrophil gelatinase-associated lipocalin (NGAL) as a biomarker of SCN in steady state and vaso-occlusive crisis (VOC).

Relationship of Thrombospondin 1 to von Willebrand Factor and ADAMTS-13 in Sickle Cell Disease Patients of Arab Ethnicity.

Background: Thrombospondin 1 (TSP-1) is a multifunctional glycoprotein secreted by platelets. In sickle cell disease (SCD), TSP-1 promotes red cell adhesion to the endothelium by binding to von Willebrand factor (vWF) and inhibiting its degradation by the protease ADAMTS-13. We investigated a possible correlation between TSP-1, vWF and ADAMTS-13 in adult and pediatric SCD patients.

The Sub-Phenotypes of Sickle Cell Disease in Kuwait.

Kuwaiti patients with sickle cell disease generally have a mild phenotype, but exhibit considerable heterogeneity, in spite of high Hb F levels. We have carried out a cross-sectional study of patients with sickle cell disease in the five major hospitals in Kuwait. Details of their hemoglobin (Hb) genotypes, clinical presentations and complications are presented.

Evaluation of von Willebrand factor and ADAMTS-13 antigen and activity levels in sickle cell disease patients in Kuwait.

Sickle cell disease (SCD) is a severe form of hemolytic anemia characterized by chronic hemolysis and is associated with increased thrombotic risk. Elevated von Willebrand factor (vWF) levels in SCD have been attributed to increased secretion and impaired processing by its cleaving protease ADAMTS-13. In this study we measured vWF and ADAMTS-13 antigen and activity levels in our SCD patients.

Clinical and Molecular Characteristics of Non-Transfusion-Dependent Thalassemia in Kuwait.

Although not regularly transfused, patients with non-transfusion-dependent thalassemia (NTDT) are prone to iron overload and its complications. Their molecular, phenotypical and laboratory characteristics vary in different populations and there is a need to document local prevailing patterns. We have reviewed the records of our patients with NTDT in Kuwait and documented their clinical and molecular characteristics in addition to iron status [serum ferritin and liver magnetic resonance imaging (MRI) T2*], management and complications.

Circulating cell-free DNA in sickle cell disease: is it a potentially useful biomarker?

Context: Vascular occlusion in sickle cell disease causes increased levels of plasma cell-free DNA as a result of cell death and tissue damage.

Objectives: This study investigates plasma cell-free DNA concentrations in sickle cell disease patients, and aims at exploring the significance of plasma cell-free DNA as a potential biomarker in predicting its complications.

Design: Plasma cell-free DNA levels were measured using real-time quantitative polymerase chain reaction to quantitatively measure β-globin gene in blood samples from 57 sickle cell disease patients with acute vaso-occlusive crisis, 42 patients in steady state, 16 individuals with sickle cell trait, and 40 healthy controls.

Transthoracic echocardiography and 6-minute walk test in Kuwaiti sickle cell disease patients.

Objective: The aim of this study was to investigate cardiac abnormalities in Kuwaiti sickle cell disease (SCD) patients using markers such as tricuspid regurgitant jet velocity (TRJV), pulmonary artery systolic pressure (PASP), and the 6-minute walk (6MW) test and correlate these findings with clinical, hematological, and biochemical parameters.

Materials And Methods: Seventy-three patients with SCD and 70 matched controls were studied. The cardiac status was investigated using transthoracic echocardiography in 57 patients; the 6MW test was carried out in patients and controls.

Therapeutic effect of low-molecular weight heparin and incidence of lower limb deep venous thrombosis and pulmonary embolism after laparoscopic bariatric surgery.

Background: The aim of our study was to determine the therapeutic effect of low-molecular weight heparin after laparoscopic Roux-en-Y gastric bypass.

Methods: We prospectively analyzed data of 39 patients who underwent Roux-en-Y gastric bypass from 1093 consecutive patients who underwent bariatric procedures from May 1999 to May 2012. All patients were given 40 mg enoxaparin subcutaneously once daily preoperatively and continued for 5 days.

Blood transfusion in sickle cell disease.

Sickle cell anemia is an inherited disease that causes chronic hemolytic anemia. Its pathognomonic signs and symptoms are caused by hemoglobin (Hb) S, which results from a single nucleotide substitution in the β-globin gene that places the amino acid valine with glutamic acid at codon 6 of the β-globin chain. Hb S is an insoluble Hb that crystalizes at low oxygen tension and other precipitating conditions leading to rigidity of red cells and clumping in small blood vessels.

Genotypic and phenotypic differences between nodal and extranodal diffuse large B-Cell lymphomas.

Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous group of diseases that have diverse clinical, pathological, and biological features. Here, it is shown that primary nodal and extranodal DLBCLs differ genomically and phenotypically. Using conventional comparative genomic hybridization (CGH), the authors assessed the chromosomal aberrations in 18 nodal, 13 extranodal, and 5 mixed DLBCLs.

Pattern of bone mineral density in sickle cell disease patients with the high-Hb F phenotype.

Low bone mineral density (BMD) is common in sickle cell disease (SCD) patients of all ages due to erythropoietic stress and consequent bone marrow hyperplasia. Kuwaiti SCD patients have a mild clinical phenotype because of their high Hb F level. There has been no previous documentation of BMD in this population of patients.

Hypercoagulable state and methylenetetrahydrofolate reductase (MTHFR) C677T mutation in patients with beta-thalassemia major in Kuwait.

Introduction: Patients with thalassemia major often present with a hypercoagulable state, the pathogenesis of which is still not understood.

Materials And Methods: This study evaluates the risk factors for hypercoagulability in 50 beta-thalassemia major patients and 50 healthy controls. Fasting total homocysteine, protein C (PC), protein S (PS), antithrombin (AT), activated protein C resistance (APCR) and lupus anticoagulant (LA) were assessed.

Determinants and associations of homocysteine and prothrombotic risk factors in Kuwaiti patients with cerebrovascular accident.

Objective: The objective of this study was to evaluate the determinants and associations of some prothrombotic risk factors in patients with cerebrovascular accidents (CVAs).

Subjects And Methods: In this case-control study, plasma total homocysteine (tHcy), lupus anticoagulant, protein C, protein S, activated protein C resistance (APC-R) and antithrombin were measured in 102 patients (60 males and 42 females) and 167 controls (87 males, 80 females). Serum vitamin B(12), folate, red cell folate, creatinine, lipid profile and glucose were also determined.

Body iron stores in relation to the metabolic syndrome, glycemic control and complications in female patients with type 2 diabetes.

Background And Aim: Studies suggest that iron plays a significant role in the development of diabetes and its complications. This study evaluates the associations of iron metabolism parameters with the metabolic syndrome (MS), control and complications in female patients with type 2 diabetes mellitus (T2DM).

Methods And Results: Ferritin, soluble Transferrin Receptor (sTfR), sTfR/Log ferritin ratio (sTfR-F index), iron, full blood count and high-sensitivity C-reactive protein (hs-CRP) were determined in 110 female patients with T2DM.

Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients.

Objective: This study aimed to document the transition of hemoglobin (Hb) F levels from early childhood to adulthood in Kuwaiti sickle cell disease patients, investigating its relationship to sex, Hb genotype and coexistence of alpha-thalassemia trait.

Subjects And Methods: The following parameters were extracted from the patients' records: age, sex, Hb, mean corpuscular volume, mean corpuscular Hb, red blood cell count, Hb F, Hb S, Hb A(2) and alpha-globin genotype. Hb quantitation was performed with cation exchange HPLC, while alpha-globin genotype was determined by PCR.

Plasma homocysteine and hematological factors in patients with venous thromboembolic diseases in Kuwait.

Background: Venous thromboembolic disease (VTE) is a common cause of morbidity in Kuwait, but the risk factors have not been studied. Hyperhomocysteinemia has been suggested as one of the risk factors. We postulate that hyperhomocysteinemia acts synergistically with hematological variables to increase VTE risk.

Determinants of plasma homocysteine in relation to hematological and biochemical variables in patients with acute myocardial infarction.

Background: Elevated plasma total homocysteine (tHcy) is a risk factor for coronary artery disease (CAD), but the mechanism is not known. This study evaluates the determinants and associations of tHcy in patients presenting with acute myocardial infarction (AMI).

Methods: Plasma concentration of tHcy, protein C, protein S, and antithrombin were measured in 210 (177 males and 33 females) patients with first AMI and 167 (87 males and 80 females) controls.

Technetium 99m-diethylene triamine penta-acetic acid aerosol clearance in the evaluation of pulmonary involvement in sickle cell disease.

Background: Pulmonary clearance of inhaled technetium (Tc) 99m-labeled diethylene triamine penta-acetic acid (DTPA) aerosol is a sensitive non-invasive marker of alveolar permeability and patients with interstitial lung diseases show enhanced clearance. However, a previous study in adult patients with diabetes mellitus showed delayed clearance.

Objectives: To investigate DTPA clearance in steady-state, otherwise healthy adult sickle cell disease (SCD) patients and correlate it with pulmonary function tests (PFTs), hematologic and clinical parameters.