Clinical characteristics and thrombophilia associated gene variants in Egyptians with unprovoked venous thromboembolism: three centers experience.

BACKGROUND: Thrombophilias are characterized by excessive venous and arterial thrombosis at regular or unusual sites. It may result from inherited, acquired, or a combination. Hereditary thrombophilia (HT) is detected in 30-40% of patients with thromboembolism. Venous/arterial thrombosis is considered a multifactorial disorder, some patients may have more than one risk factor which may be transient or permanent. OBJECTIVES: Assess the clinical characteristics of patients with unprovoked thromboembolic events and the role of inherited thrombophilia as a causative or additive risk factor. METHODS: 210 consecutive adult patients with unprovoked thromboembolic events were reviewed in hematology units at three tertiary Egyptian centers between September 2022 and September 2023. The diagnosis of thromboembolic events was confirmed by clinical and radiological findings. Laboratory screening for thrombophilia-associated. RESULTS: Among our patients, 53(25.2%) patients presented with isolated DVT, followed by portal vein thrombosis, 32(15.2%) had a pulmonary embolism, and sagittal sinus thrombosis was developed in 23(10.9%) patients. CONCLUSION: Younger people who experience spontaneous thromboembolism run the chance of having hereditary thrombophilia; the more mutations discovered, the higher the risk of thrombosis; the lower leg and deep vein thrombosis were the most common sites. Lastly, MTHFR C677T was the most common polymorphism in Egyptians, detected in almost half of the cases.

Blood phenotype O and indirect bilirubin are associated with lower, early COVID-19-related mortality: A retrospective study.

OBJECTIVES: To evaluate the ABO blood type and indirect bilirubin to predict early mortality in adults with severe COVID-19. METHODS: This retrospective observational study was conducted on 268 adult patients with laboratory-confirmed COVID-19 who had attended the intensive care unit (ICU), Quena general hospital and Luxor International Hospital, and other hospitals or centers for the treatment of COVID-19, during the period from January 2021 till December 2021. RESULTS: Relation between mortality and ABO group were highly significant, as we found non-O blood group with more risk of early mortality and intensive care unit admission ICU. There were significant differences between dead and alive cases as regards platelets, white blood cells WBCs (neutrophil, lymphocyte), albumin, liver enzymes aspartate transeferase (AST), alanine transferase (ALT), total direct and indirect bilirubin, creatinine, and urea. CONCLUSION: There was a highly significant relation between dead cases and ABO blood group as between the O and non-O groups; also, group O was associated with less severe manifestations and or ventilation and less mortality in patients with severe COVID-19 infection. Direct bilirubin >0.5 was found to be the best predictor for mortality in cases with COVID-19 so indirect bilirubin may be considered a good protector against complications of the infection.

GDF-15 is associated with atherosclerosis in adults with transfusion-dependent beta-thalassemia.

OBJECTIVES: To study serum growth differentiation factor-15 (GDF-15) serum level in ß-thalassemia patients and its relation to carotid intima-media thickness. BACKGROUND: Thalassemia is a common genetic disease resulting in decreased beta-chains, leading to manifested anemia. It may be subsequently complicated by iron overload, which induces numerous morbidities and even death. Growth differentiation factor-15 (GDF-15) is a strong and independent predictor of mortality and disease progression in patients with atherosclerosis alongside with carotid-intimal media thickness (CIMT). PATIENTS AND METHODS: This monocentric case-control study was done on 90 subjects in the period from January 2020 to March 2021. Sixty transfusion-dependent beta-thalassemia (TDßT) cases (≥18 years) were selected from the thalassemia clinic of Hematology division at Menoufia University hospitals. We included also 30 sex and age matched healthy as the controls. Routine investigations were done beside. Serum GDF-15 was measured by ELISA. CIMT was measured by Doppler Ultrasonography. RESULTS: CIMT on both sides was statistically significant higher in cases (median of 0.08 cm) than in the controls (median of 0.04). GDF-15 was also significantly higher in cases (median of 1839.89 pg/dl) than in controls (median of 256.14 pg/dl). So, we found that GDF-15 is a predictor of and associated with atherosclerosis in thalassemic adults (OR = 39.198, p value 0.008, 95% CI: 2.576-596.5). CONCLUSION: GDF- 15 is increased in TDßT. CIMT (as a marker of subclinical atherosclerosis) is increased in these patients alongside with GDF-15, is a predictor, and associated with atherosclerosis in thalassemic adults.

Impact of indirect bilirubin and uric acid on outcomes of sepsis-associated acute kidney injury (sAKI).

BACKGROUND: Acute kidney injury (AKI) is one of the most frequent pathophysiologic disorders encountered in hospitalized patients, with sepsis frequently implicated in pathogenesis. Reactive oxygen species (ROS) seem to have a significant contribution to sepsis-induced AKI. Proposed mechanisms include induction of cell membrane lipid peroxidation, protein denaturing, and direct DNA damage, all of which have deleterious effect. These changes constitute oxidative injury to the kidneys. OBJECTIVE: To evaluate the antioxidant actions of indirect bilirubin and uric acid on outcomes of sepsis-associated AKI. METHODS: Ninety-eight patients admitted to the intensive care unit (ICU), at a large tertiary center, with sepsis and AKI were evaluated for serum levels of uric acid, bilirubin (primarily indirect), and procalcitonin. The primary endpoints studied were the need for hemodialysis and death. RESULTS: Thirty-two (33%) patients developed AKI requiring hemodialysis (HD). These patients had higher SOFA scores (p < 0.001) and lower levels of indirect bilirubin (p < 0.001) compared to those not requiring HD. There was no statistically significant difference in serum uric acid levels. Logistic regression analysis identified creatinine level, total and indirect bilirubin levels, and leukocyte count as significant predictors of patient death. CONCLUSION: Higher leukocyte counts and creatinine levels were independently associated with poor outcomes in ICU patients with sepsis. Additionally, lower indirect bilirubin levels were also noted to be associated with similar outcomes. The latter provides insights into oxidative stress as a major player in the pathogenesis of sepsis-induced AKI, with a potential protective role of indirect bilirubin.

Endothelial Activation and Immune Thrombocytopenia: An Engagement Waiting for Consolidation.

Immune thrombocytopenia (ITP) appears to be a heterogeneous disease. In some patients, autoimmunity may be associated with an inflammatory process, and in other patients, low platelets may interfere with other aspects of the coagulation system. Either may predispose to thrombosis or bleeding. Further investigation of the interactions of platelets, with inflammatory cytokines and endothelial biomarkers, may help us to better understand the disease, and to recognize those patients at risk of bleeding, or conversely thrombosis. The aim of this work is to estimate von Willebrand factor (vWF) and vascular cellular adhesion molecule (V-CAM) serum levels in adult immune thrombocytopenic patients (ITP) and to decipher their possible clinical correlates. Eighty adults (≥ 18 years) were enrolled in the study; naive newly diagnosed 40 patients with primary ITP (according to the ASH 2019) and 40 sex and age-matched healthy controls, all groups are subjected for complete blood count (CBC), liver, and renal function tests, ESR, CRP, V-CAM, and VWF-Ag by enzyme-linked immunosorbent assay (ELISA). There was a highly statistically significant difference between case and control as regards to the mean level of VWF-Ag and V-CAM. vWF and V-CAM could serve as biomarkers for endothelial alterations and should be investigated as a predictor of thrombocytopenic bleeding and tailor patient management accordingly.

Thrombo-inflammatory biomarkers to predict sepsis outcome.

BACKGROUND: Sepsis has been redefined recently as life-threatening organ dysfunction caused by dysregulated host responses to infection and septic shock. Soluble urokinase plasminogen activator receptor (SuPAR) and plasminogen activator inhibitor-1(PAI-1) concentration positively correlate to the activation level of the immune system, and are markers of disease severity and aggressiveness. OBJECTIVE: The study aimed to identify the blood level of plasminogen activator inhibitor-1 (PAI-1) and soluble urokinase plasminogen activator receptor (SuPAR) in sepsis and its association with mortality. PATIENT AND METHODS: This is an observational prospective study that enrolled 60 adult patients with sepsis (according to SOFA), admitted to Menoufia and Zagazig university hospitals during the period from December 2019 till October 2020. Plasminogen activator inhibitor-1 (PAI-1) and soluble urokinase plasminogen activator receptor (SuPAR) were checked in all participants. RESULTS: SuPAR and PAI.1 were significant independent predictors of hospital mortality. SuPAR showed sensitivity 100%, specificity 95.9%, and accuracy 94% for prediction of early mortality at a cutoff value of 13.4(pg/ml). While, PAI-1 demonstrated sensitivity 100%, specificity 93.9%, and accuracy of 95% at a cutoff value of 122.5 for predicting mortality. CONCLUSION: PAI-1 and suPAR were significant predictors of hospital mortality among sepsis patients. The sample size was relatively small, which may have decreased the statistical power of the results of the present study. Hence, additional studies with large sample sizes are required for further validation of the present results.