scholarly journals Trajectories of Biological Values and Vital Parameters: An Observational Cohort Study of Adult Patients with Sickle Cell Disease Hospitalized for a Non-Complicated Vaso-Occlusive Crisis

2019 ◽  
Vol 8 (9) ◽  
pp. 1502
Author(s):  
Veil ◽  
Bussy ◽  
Looten ◽  
Arlet ◽  
Pouchot ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Observational Cohort Study ◽  
Cell Disease ◽  
Cell Counts ◽  
Observational Cohort ◽  
Steady State Levels ◽  
White Blood Cell Counts ◽  
Vital Parameters

Hospital admission of patients with sickle-cell disease (SCD) presenting with a vaso-occlusive crisis (VOC) can be justified by pain refractory to usual outpatient care and/or the occurrence of a complication. Yet, the trajectories of vital parameters and standard biomarkers throughout a non-complicated VOC has not been established. In this observational cohort study, we describe the course of routine parameters throughout 329 hospital stays for non-complicated VOC. We used a new spline-based approach to study and visualize non-specific time-dependent variables extracted from the hospital clinical data warehouse. We identified distinct trends during the VOC for hemoglobin level, leukocytes count, C-Reactive Protein (CRP) level and temperature. Hemoglobin decreased after admission and rarely returned to steady state levels before discharge. White blood cell counts were elevated at admission before immediately decreasing, whereas eosinophils increased slowly throughout the first five days of the stay. In over 95% of non-complicated VOC-related stays, the CRP value was below 100 mg/L within the first day following admission and above normal after 48 hours, and the temperature was below 38 °C throughout the entire stay. Knowing the typical trajectories of these routine parameters during non-complicated VOC may urge the clinicians to be more vigilant in case of deviation from these patterns.

Progressive Decline in Estimated GFR in Patients With Sickle Cell Disease: An Observational Cohort Study

2019 ◽  
Vol 74 (1) ◽  
pp. 47-55 ◽  
Author(s):  
Vimal K. Derebail ◽  
Emily J. Ciccone ◽  
Qingning Zhou ◽  
R. Rosina Kilgore ◽  
Jianwen Cai ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Observational Cohort Study ◽  
Cell Disease ◽  
Estimated Gfr ◽  
Progressive Decline ◽  
Observational Cohort

Increased GM-CSF Levels in Sickle Cell Disease Are Associated with Increased Leukocyte Counts and Are Reversed by Hydroxyurea.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3573-3573
Author(s):  
Nicola Conran ◽  
Tohru Ikuta ◽  
Sara T.O. Saad ◽  
Fernando F. Costa
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Fetal Hemoglobin ◽  
Cell Disease ◽  
Gm Csf ◽  
Cell Counts ◽  
Ion Exchange Hplc ◽  
Csf Levels ◽  
White Blood Cell Counts

Abstract The hematopoietic cytokine, GM-CSF, stimulates the proliferation and differentiation of progenitor cells to macrophages, eosinophils and megakaryocytes. Increased levels of GM-CSF have previously been reported in sickle cell disease (SCD) patients with low fetal hemoglobin (HbF) levels (Croizat, Br J Haematol. 87:592; 1994); however, a clearcut association between GM-CSF and HbF levels in SCD has yet to be established, since there is some evidence that levels may depend on other factors (Haider et al., Acta Haematol. 102:140; 1999). To further investigate the possible role of GM-CSF in SCD, plasma samples were collected from normal individuals (AA), steady-state sickle cell anemia patients (SS) and sickle cell patients on hydroxyurea therapy (SSHU; 20-30 mg/kg/day). Plasma GM-CSF levels were measured using a specific immunoenzymatic assay. HbF levels were quantified by ion-exchange HPLC and hematological measurements obtained using an Advia Hematology System. Plasma GM-CSF levels seem to be higher in SS patients than in normal controls (0.828 ± 0.215 pg/ml, n=39; 0.230 ± 0.081 pg/ml, n=9; respectively). In contrast, GM-CSF was significantly lower in SSHU patients than in SS without HU (0.288 ± 0.104 pg/ml; n=14; P = 0.045 compared to SS). A negative correlation between plasma GM-CSF and HbF levels was seen in patients (r = −0.332; P = 0.026; n=45; SS and SSHU groups) and, notably, a positive correlation between GM-CSF and white blood cell counts was observed (r = 0.302; P = 0.037; n= 48; SS and SSHU groups). Furthermore, GM-CSF (2.5 ng/ml) abolished the production of γ globin during erythropoietin-stimulated differentiation of TF-1 hematopoietic cells. These results provide further support to the hypothesis that plasma GM-CSF correlates negatively with HbF in sickle cell disease. Interestingly, in vitro data suggest that increased GM-CSF may, in fact, diminish HbF levels rather than reflect increased haematopoietic stress as a consequence of low HbF. Importantly, GM-CSF appears to correlate with leukocyte numbers in SCD, and levels were significantly decreased in patients taking HU, indicating that this cytokine may contribute to the leukocytosis seen in some SCD patients and may play a role in leukocyte count reduction by HU.

Carbon Monoxide Decreases Leukocytosis in Murine Sickle Cell Disease Models Via Decreased Granulopoiesis.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1433-1433
Author(s):  
Joan D Beckman ◽  
Julie V. Vineyard ◽  
Chunsheng Chen ◽  
Julia Ngyuen ◽  
Michael O Nwaneri ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Bone Marrow ◽  
Flow Cytometry ◽  
Carbon Monoxide ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Cell Counts ◽  
Blood Cell Counts ◽  
White Blood Cell Counts

Abstract Elevated white blood cell counts (WBC) in sickle cell disease (SCD) are risk factors for morbid vaso-occlusive events including acute chest syndrome and stroke. The mechanisms of leukocytosis in SCD include increased inflammation, oxidative stress, increased hematopoiesis, and hyposplenism. Heme oxygenase-1 (HO-1) plays critical roles in metabolizing the excess heme generated during hemolysis and in modulating vaso-occlusion in murine models of sickle cell disease (SCD). The products of HO-1 activity, carbon monoxide (CO), Fe2+/ferritin, and biliverdin/bilirubin have demonstrable anti-oxidant and anti-inflammatory effects. We have previously demonstrated that inhaled CO treatments decrease white blood cell counts (WBC), as well as liver redox-active iron and heme content in heterozygous BERK mice, a mouse model for sickle cell trait. To dissect the mechanism of this decrease in leukocytosis, we hypothesize that prolonged treatment with inhaled CO significantly decreases granulopoiesis in SCD mice. For this study, we exposed S+S-Antilles mice to 250 ppm inhaled CO for 1h 3X/week for 8–10 weeks. Upon completion of the treatment period animals were euthanized, blood was removed by cardiac puncture, and bone marrow and organs were harvested for analysis. Treatment for 10 weeks with 250 ppm CO significantly decreased total WBC (19.19±1.29 × 1000/ul (untreated) to 12.8±1.30 × 1000/ul (CO treated), p<0.05). The decrement in total WBC count was primarily due to a significant decrease in neutrophils (p<0.05) and lymphocytes (p<0.005), There was no significant change in the reticulocyte count, hematocrit, or bilirubin in CO-treated animals compared to controls. The spleens of the control and CO-treated animals had similar weights but no differences in their histopathology, ruling out the possibility of increased sequestration as a cause of the decrease in total WBC. Bone marrow staining reveals that CO-treated mice have a significant decrease in polymorphonuclear (PMN) precursors in bone marrow (p<0.05). Flow cytometry on bone marrow stained for hematopoietic markers CD117, CD45R/B220, SCA1, CD90.1, and CD135 demonstrates a significant (p<0.005) decrease in common lymphoid progenitor (CLP) and common myeloid progenitor (CMP) cells in CO-treated animals compared to controls. Flow cytometry for myeloperoxidase (MPO) and the granulocyte differentiation marker GR-1 (Ly-6G/6C) also demonstrates a significant (p<0.05) decrease in mature granulocytes cells in CO-treated mice. Colony-forming cell (CFC) assays verify the flow cytometry data, with a significant decrease (p<0.05) in CFU-GM in 250 ppm treated bone marrow compared to controls. Consistent with the lack of effect on hematocrit, CO had minimal effects on the erythroid marrow compartment since total CD71 positive erythroid cells were unchanged. In summary, we conclude that inhaled CO treatments decrease total WBC by decreasing granulopoiesis. We speculate that inhaled CO treatments may be a potential therapy for patients with SCD by acting as a modulator of oxidative stress and inflammation.

Retrospective Cohort Study Comparing the Effect of Obesity in Children with Sickle Cell Disease

PEDIATRICS ◽  
2016 ◽  
Vol 137 (Supplement 3) ◽  
pp. 264A-264A
Author(s):  
Parth J. Bhatt ◽  
Dinesh Singh ◽  
Akingbola Olubenga ◽  
Devraj Chavda ◽  
Achint Patel
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Retrospective Cohort Study ◽  
Retrospective Cohort ◽  
Cell Disease ◽  
Obesity In Children

scholarly journals Morbidity and mortality of sickle cell disease patients starting intermittent haemodialysis: a comparative cohort study with non- Sickle dialysis patients

2016 ◽  
Vol 174 (1) ◽  
pp. 148-152 ◽  
Author(s):  
Louise Nielsen ◽  
Florence Canouï-Poitrine ◽  
Jean-Philippe Jais ◽  
Djamal Dahmane ◽  
Pablo Bartolucci ◽  
...  
Keyword(s):  
Cohort Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Morbidity And Mortality ◽  
Cell Disease ◽  
Dialysis Patients ◽  
Intermittent Haemodialysis ◽  
Comparative Cohort Study
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