scholarly journals Systemic T Cell Subsets and Cytokines in Patients With Homozygous Sickle Cell Disease and Asymptomatic Urinary Tract Infections in Togo

2021 ◽  
Vol 21 (2) ◽  
pp. 163-172
Author(s):  
Christèle Nguepou Tchopba ◽  
Gnatoulma Katawa ◽  
Essohana Padaro ◽  
Pélagie Edlom Tchadié ◽  
Simplice Damintoti Karou ◽  
...  
Keyword(s):  
Urinary Tract ◽  
T Cell ◽  
Sickle Cell Disease ◽  
Urinary Tract Infections ◽  
Sickle Cell ◽  
T Cell Subsets ◽  
Cell Disease ◽  
Tract Infections ◽  
Homozygous Sickle Cell Disease

scholarly journals Urinary Tract Infection in Febrile Children with Sickle Cell Disease Who Present to the Emergency Room with Fever

2020 ◽  
Vol 9 (5) ◽  
pp. 1531
Author(s):  
Nehal Patel ◽  
Ahmad Farooqi ◽  
Michael Callaghan ◽  
Usha Sethuraman
Keyword(s):  
Risk Factors ◽  
African American ◽  
Urinary Tract ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Retrospective Chart Review ◽  
The United States ◽  
Pediatric Emergency ◽  
Cell Disease ◽  
Tract Infections

Sickle cell disease (SCD) patients are thought to be at higher risk for urinary tract infections (UTIs) compared to the general population secondary to increased sickling, abnormal urinary acidification, and an inability to concentrate the urine. The incidence of UTI in febrile children with SCD in the United States is unknown. Our objectives were to determine the rate of UTI among febrile SCD children and describe the risk factors for UTI in this population. We conducted a retrospective chart review of all febrile SCD patients <4 years of age who presented to a pediatric emergency department from 2012–2017 and who had a sterile sample of urine for analysis. A total of 167 febrile patients with SCD with 464 visits were identified. The majority were African American (95.2%), female (58.7%), and had hemoglobin SS (HbSS) (65.3%). The rate of UTI was 4.1%. All patients with a UTI were African American females with a median age of 19 months (IQR 12–30). On regression analysis, no risk factors were associated with a UTI. We found the rate of UTI in febrile young children with SCD was comparable to non-SCD children. Larger studies are required to identify the presence of risk factors for UTI in this population.

scholarly journals Pattern of Serum Cytokine Expression and T-Cell Subsets in Sickle Cell Disease Patients in Vaso-Occlusive Crisis

2010 ◽  
Vol 17 (4) ◽  
pp. 602-608 ◽  
Author(s):  
Bolanle O. P. Musa ◽  
Geoffrey C. Onyemelukwe ◽  
Joseph O. Hambolu ◽  
Aisha I. Mamman ◽  
Albarka H. Isa
Keyword(s):  
Immune Response ◽  
T Cell ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Interleukin 2 ◽  
High Sensitivity ◽  
T Cell Subsets ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
Th1 And Th2

ABSTRACT The pathogenesis of sickle vaso-occlusive crisis (VOC) in sickle cell disease (SCD) patients involves the accumulation of rigid sickle cells and the stimulation of an ongoing inflammatory response, as well as the stress of infections. The immune response, via cytokine imbalances and deregulated T-cell subsets, also has been proposed to contribute to the development of VOC. In this study, a panel of high-sensitivity cytokine kits was used to investigate cytokines in the sera of SCD patients in VOC. The results were compared primarily with those for stable SCD patients and secondarily with those for normal healthy people who served as controls. The cytokines studied included interleukin-2 (IL-2), IL-4, and IL-10. Lymphocyte subsets of patients with VOC were also studied and were compared with those of both control groups (20 stable patients without crisis [SCD group] and 20 normal healthy controls [NHC]). The VOC group was notable for remarkably elevated levels of IL-4, among the three cytokines tested, compared with those for the SCD and NHC groups. Patients with VOC also differed from stable SCD patients and NHC by having notably lower IL-10 levels, as well as the lowest ratio of CD4+ to CD8+ T cells (0.7). The patterns of the proinflammatory cytokine IL-2 did not differ between VOC and stable SCD patients, but NHC had significantly lower IL-2 levels than both the VOC and SCD groups. Our results demonstrate coexisting levels, both high and low, of TH1- and TH2-type cytokines, as well as diminished levels of T-cell subsets in VOC. These results are discussed in an effort to better understand the importance of the immune system profile in the pathogenesis of sickle cell VOC. Since the possibility that a cytokine imbalance is implicated in the pathogenesis of sickle cell crisis has been raised, our results should prompt further investigation of the host immune response in terms of TH1 and TH2 balance in sickle cell crisis.

Increased Procoagulant Activity of Red Blood Cells from Patients with Homozygous Sickle Cell Disease and β-Thalassemia

1996 ◽  
Vol 76 (03) ◽  
pp. 322-327 ◽  
Author(s):  
Dominique Helley ◽  
Amiram Eldor ◽  
Robert Girot ◽  
Rolande Ducrocq ◽  
Marie-Claude Guillin ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Annexin V ◽  
Mean Value ◽  
Procoagulant Activity ◽  
Dependent Manner ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

SummaryIt has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous β-thalassemia behave as procoagulant cells. The procoagulant activity of β-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i. e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with β-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or (3-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 µM) than in the absence of cells (26 µM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 µM) or β-thalassemia RBCs (mean value: 1.5 pM) was significantly lower compared to normal RBCs (p <0.001). No significant difference was observed between SS-RBCs and p-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and (3-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both β-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.

scholarly journals Streptococcus pneumoniae and Its Virulence Factors H2O2 and Pneumolysin Are Potent Mediators of the Acute Chest Syndrome in Sickle Cell Disease

Toxins ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 157
Author(s):  
Joyce Gonzales ◽  
Trinad Chakraborty ◽  
Maritza Romero ◽  
Mobarak Abu Mraheil ◽  
Abdullah Kutlar ◽  
...  
Keyword(s):  
Streptococcus Pneumoniae ◽  
Sickle Cell Disease ◽  
Virulence Factors ◽  
Sickle Cell ◽  
Biological Activities ◽  
Acute Chest Syndrome ◽  
Antibiotics Resistance ◽  
Cell Disease ◽  
Antibiotic Drug ◽  
Tract Infections

Sickle cell disease (SCD) is one of the most common autosomal recessive disorders in the world. Due to functional asplenia, a dysfunctional antibody response, antibiotic drug resistance and poor response to immunization, SCD patients have impaired immunity. A leading cause of hospitalization and death in SCD patients is the acute chest syndrome (ACS). This complication is especially manifested upon infection of SCD patients with Streptococcus pneumoniae (Spn)—a facultative anaerobic Gram-positive bacterium that causes lower respiratory tract infections. Spn has developed increased rates of antibiotics resistance and is particularly virulent in SCD patients. The primary defense against Spn is the generation of reactive oxygen species (ROS) during the oxidative burst of neutrophils and macrophages. Paradoxically, Spn itself produces high levels of the ROS hydrogen peroxide (H2O2) as a virulence strategy. Apart from H2O2, Spn also secretes another virulence factor, i.e., the pore-forming exotoxin pneumolysin (PLY), a potent mediator of lung injury in patients with pneumonia in general and particularly in those with SCD. PLY is released early on in infection either by autolysis or bacterial lysis following the treatment with antibiotics and has a broad range of biological activities. This review will discuss recent findings on the role of pneumococci in ACS pathogenesis and on strategies to counteract the devastating effects of its virulence factors on the lungs in SCD patients.

scholarly journals Clinical findings associated with homozygous sickle cell disease in the Barbadian population – do we need a national SCD registry?

2014 ◽  
Vol 7 (1) ◽  
Author(s):  
Kim R Quimby ◽  
Stephen Moe ◽  
Ian Sealy ◽  
Christopher Nicholls ◽  
Ian R Hambleton ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Findings ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

Validation of a Questionnaire to Identify Respiratory Tract Infections in Children With Sickle Cell Disease

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Aliva De ◽  
Chika V. Anekwe ◽  
Meyer Kattan ◽  
Yujing Yao ◽  
Zhezhen Jin ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Respiratory Tract ◽  
Sickle Cell ◽  
Respiratory Tract Infections ◽  
Cell Disease ◽  
Tract Infections

scholarly journals Concurrent homozygous sickle‐cell disease and severe haemophilia A: Thromboelastography profiles

Haemophilia ◽  
2019 ◽  
Vol 25 (2) ◽  
Author(s):  
Ahmar U. Zaidi ◽  
Latha Rao ◽  
Michael U. Callaghan ◽  
Madhvi Rajpurkar ◽  
Wendy Hollon ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Cell Disease ◽  
Homozygous Sickle Cell Disease

scholarly journals Factors affecting prepubertal growth in homozygous sickle cell disease.

1996 ◽  
Vol 74 (6) ◽  
pp. 502-506 ◽  
Author(s):  
A Singhal ◽  
J Morris ◽  
P Thomas ◽  
G Dover ◽  
D Higgs ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Factors Affecting ◽  
Homozygous Sickle Cell Disease

scholarly journals Pregnancies and Neonatal Outcomes in Patients with Sickle Cell Disease (SCD): Still a (High-)Risk Constellation?

2021 ◽  
Vol 11 (9) ◽  
pp. 870
Author(s):  
Pia Proske ◽  
Laura Distelmaier ◽  
Carmen Aramayo-Singelmann ◽  
Nikolaos Koliastas ◽  
Antonella Iannaccone ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Neonatal Outcomes ◽  
University Hospital ◽  
High Rate ◽  
Successful Outcome ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
Tract Infections

Background: This monocentric study conducted at the University Hospital of Essen aims to describe maternal and fetal/neonatal outcomes in sickle cell disease (SCD) documented between 1996 to 2021 (N = 53), reflecting the largest monocentric analysis carried out in Germany. Methods/Results: 46 pregnancies in 22 patients were followed. None of the patients died. In total, 35% (11/31) of pregnancies were preterm. 15 pregnancies in eight patients were conceived on hydroxycarbamide (HC), of which nine had a successful outcome and three were terminated prematurely. There was no difference regarding the rate of spontaneous abortions in patients receiving HC compared to HC-naive patients prior to conception. In patients other than HbS/C disease, pregnancies were complicated by vaso-occlusive crises (VOCs)/acute pain crises (APCs) (96%, 23/24); acute chest syndrome (ACS) (13%, 3/24), transfusion demand (79%, 19/24), urinary tract infections (UTIs) (42%, 10/24) and thromboembolic events (8%, 2/24). In HbS/C patients complications included: VOCs/APCs (43%, 3/7; ACS: 14%, 1/7), transfusion demand (14%, 1/7), and UTIs (14%, 1/7). Independent of preterm deliveries, a significant difference with respect to neonatal growth in favor of neonates from HbS/C mothers was observed. Conclusion: Our data support the results of previous studies, highlighting the high rate of maternal and fetal/neonatal complications in pregnant SCD patients.

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