scholarly journals Oxygen gradient ektacytometry‐derived biomarkers are associated with vaso‐occlusive crises and correlate with treatment response in sickle cell disease

2020 ◽  
Vol 96 (1) ◽  
Author(s):  
Minke A. E. Rab ◽  
Celeste K. Kanne ◽  
Jennifer Bos ◽  
Brigitte A. Oirschot ◽  
Camille Boisson ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Treatment Response ◽  
Sickle Cell ◽  
Cell Disease ◽  
Oxygen Gradient

scholarly journals Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease

Cells ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 811
Author(s):  
Camille Boisson ◽  
Minke A. E. Rab ◽  
Elie Nader ◽  
Céline Renoux ◽  
Celeste Kanne ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Oxygen Gradient ◽  
Acute Complication ◽  
Adults And Children ◽  
The Difference

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.

Methodological aspects of oxygen gradient ektacytometry in sickle cell disease: Effects of sample storage on outcome parameters in distinct patient subgroups

2020 ◽  
pp. 1-4
Author(s):  
Camille Boisson ◽  
Minke A.E. Rab ◽  
Elie Nader ◽  
Céline Renoux ◽  
Brigitte A. van Oirschot ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Genetic Disorder ◽  
Compound Heterozygous ◽  
Cell Disease ◽  
Oxygen Gradient ◽  
Abnormal Hemoglobin ◽  
Methodological Aspects ◽  
Patient Subgroups ◽  
Rbc Deformability

Sickle cell disease (SCD) is a genetic disorder characterized by the production of an abnormal hemoglobin (Hb), which, under deoxygenation, may polymerize and cause a mechanical distortion of red blood cell (RBC) into a crescent-like shape. Recently a method, using ektacytometry principle, has been developed to assess RBC deformability as a function of oxygen tension (pO2) and is called oxygen gradient ektacytometry (oxygenscan). However, standardization of this test is needed to properly assess the tendency of sickling of RBCs under deoxygenation and to allow comparisons between different laboratories. The study compared the oxygenscan responses during blood storage between distinct populations of SCD patients. Blood from 40 non-transfused homozygous SCD patients (HbSS), 16 chronically transfused HbSS patients, and 14 individuals with compound heterozygous hemoglobin SC disease (HbSC)at steady-state was collected in EDTA tubes. Measurements were performed within 4 hours after collection and after 24 hours of storage at 4°C. We showed that storage affected the minimum RBC deformability reached during deoxygenation (EImin) in both non-transfused HbSS and HbSC patients and the maximum RBC deformability (EImax) measured before deoxygenation (i.e., in normoxia) in the three groups. In contrast, the tendency of RBCs to sickle under deoxygenation (i.e., the point of sickling; PoS) remained rather stable between the two time of measurements. Collectively, since the time between blood sampling and analysis affects some key oxygen gradient ektacytometry-derived parameters we recommend that each laboratory performs oxygenscan measurements at a standardized time point.

Alveolar-arterial oxygen gradient in acute chest syndrome of sickle cell disease

1993 ◽  
Vol 123 (2) ◽  
pp. 272-275 ◽  
Author(s):  
Umit Emre ◽  
Scott T. Miller ◽  
Sreedhar P. Rao ◽  
Madu Rao
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Arterial Oxygen ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Oxygen Gradient

Hemolysis in sickle cell disease

1974 ◽  
Vol 133 (4) ◽  
pp. 624-631 ◽  
Author(s):  
T. A. Bensinger
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

Computerized cognitive training in pediatric sickle cell disease: A randomized controlled pilot study.

2020 ◽  
Vol 8 (4) ◽  
pp. 390-401 ◽  
Author(s):  
Taryn M. Allen ◽  
Lindsay M. Anderson ◽  
Samuel M. Brotkin ◽  
Jennifer A. Rothman ◽  
Melanie J. Bonner
Keyword(s):  
Pilot Study ◽  
Sickle Cell Disease ◽  
Cognitive Training ◽  
Sickle Cell ◽  
Cell Disease ◽  
Computerized Cognitive Training ◽  
Randomized Controlled ◽  
Pediatric Sickle Cell Disease
Sign in / Sign up

Export Citation Format

Share Document