scholarly journals Measurement of Plasma Free Hemoglobin Using Hemolysis Index and Bilirubin Interference

2021 ◽  
Vol 43 (4) ◽  
pp. 208-213
Author(s):  
Hanmil Jang ◽  
Yonggeun Cho ◽  
John Hoon Rim ◽  
Hyein Kang ◽  
Sang-Guk Lee ◽  
...  
Keyword(s):  
Free Hemoglobin

scholarly journals Haptoglobin Duplicon, Hemoglobin, and Vitamin C: Analyses in the British Women’s Heart and Health Study and Caerphilly Prospective Study

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Philip A. I. Guthrie ◽  
Mohammad R. Abdollahi ◽  
Tom Gaunt ◽  
Debbie A. Lawlor ◽  
Yoav Ben-Shlomo ◽  
...  
Keyword(s):  
Vitamin C ◽  
Copy Number ◽  
Health Study ◽  
Free Hemoglobin ◽  
Pcr Assay ◽  
Partial Duplication ◽  
Haptoglobin Genotype ◽  
Haptoglobin Gene ◽  
Apparent Association ◽  
Quantitative Pcr Assay

Background. Haptoglobin acts as an antioxidant by limiting peroxidative tissue damage by free hemoglobin. The haptoglobin gene allele Hp2 comprises a 1.7 kb partial duplication. Relative to allele Hp1, Hp2 carriers form protein multimers, suboptimal for hemoglobin scavenging.Objective. To examine the association of haptoglobin genotype with a range of phenotypes, with emphasis on vitamin C and hemoglobin levels.Methods. We applied a quantitative PCR assay for the duplication junction to two population cohorts including 2747 British women and 1198 British men. We examined the association of haptoglobin duplicon copy number with hemoglobin and vitamin C and used the copy number to complete a phenome scan.Results.Hemoglobin concentrations were greater in those with Hp2,2 genotype, in women only (Hp1,1 13.45 g/dL, Hp1,2 13.49 g/dL, Hp2,2 13.61 g/dL;P=0.002), though statistically there was no evidence of a difference between the sexes (zvalue = 1.2,P=0.24). Haptoglobin genotype was not associated with vitamin C or any other phenotype in either cohort.Conclusions. Our results do not support association of haptoglobin genotype with vitamin C or with other phenotypes measured in two population cohorts. The apparent association between haptoglobin genotype and hemoglobin in the women’s cohort merits further investigation.

scholarly journals Long-term survival with sebelipase alfa enzyme replacement therapy in infants with rapidly progressive lysosomal acid lipase deficiency: final results from 2 open-label studies

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Suresh Vijay ◽  
Anais Brassier ◽  
Arunabha Ghosh ◽  
Simona Fecarotta ◽  
Florian Abel ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Phase 2 ◽  
Free Hemoglobin ◽  
Acid Lipase ◽  
Short Term ◽  
Open Label ◽  
Lysosomal Acid ◽  
Enzyme Replacement ◽  
Lysosomal Acid Lipase

Abstract Background If symptomatic in infants, the autosomal recessive disease lysosomal acid lipase deficiency (LAL-D; sometimes called Wolman disease or LAL-D/Wolman phenotype) is characterized by complete loss of LAL enzyme activity. This very rare, rapidly progressive form of LAL-D results in severe manifestations leading to failure to thrive and death, usually by 6 months of age. We report results from 2 open-label studies of enzyme replacement therapy with sebelipase alfa, a recombinant human LAL, in infants with LAL-D: the phase 2/3 Survival of LAL-D Infants Treated With Sebelipase Alfa (VITAL) study (NCT01371825) and a phase 2 dose-escalation study (LAL-CL08 [CL08]; NCT02193867). In both, infants received once-weekly intravenous infusions of sebelipase alfa. Results The analysis population contained 19 patients (9 in VITAL; 10 in CL08). Kaplan–Meier estimates of survival to 12 months and 5 years of age were 79% and 68%, respectively, in the combined population, and the median age of surviving patients was 5.2 years in VITAL and 3.2 years in CL08. In both studies, median weight-for-age, length-for-age, and mid-upper arm circumference-for-age z scores increased from baseline to end of study. Decreases in median liver and spleen volume over time were noted in both studies. Short-term transfusion-free hemoglobin normalization was achieved by 100% of patients eligible for assessment in VITAL, in an estimated median (95% confidence interval [CI]) time of 4.6 (0.3–16.6) months. In CL08, short-term transfusion-free hemoglobin normalization was achieved by 70% of patients eligible for assessment, in an estimated median (95% CI) time of 5.5 (3.7–19.6) months. No patient discontinued treatment because of treatment-emergent adverse events. Most infusion-associated reactions (94% in VITAL and 88% in CL08) were mild or moderate in severity. Conclusions The findings of these 2 studies of infants with rapidly progressive LAL-D demonstrated that enzyme replacement therapy with sebelipase alfa prolonged survival with normal psychomotor development, improved growth, hematologic parameters, and liver parameters, and was generally well tolerated, with an acceptable safety profile.

scholarly journals Measurements of Free Hemoglobin and Hemolysis Index: EDTA- or Lithium-Heparinate Plasma?

2007 ◽  
Vol 53 (9) ◽  
pp. 1717-1718 ◽  
Author(s):  
Janne Unger ◽  
Gerlinde Filippi ◽  
Wolfgang Patsch
Keyword(s):  
Free Hemoglobin

scholarly journals EVALUATION OF A STROMA-FREE HEMOGLOBIN SOLUTION FOR USE AS A PLASMA EXPANDER

1967 ◽  
Vol 126 (6) ◽  
pp. 1127-1142 ◽  
Author(s):  
S. Frederick Rabiner ◽  
J. Raymond Helbert ◽  
Harry Lopas ◽  
Lila H. Friedman
Keyword(s):  
Carrying Capacity ◽  
Vital Signs ◽  
Free Hemoglobin ◽  
Hemoglobin Solution ◽  
Plasma Expander ◽  
Chronic Effects ◽  
Coagulant Activity ◽  
Methemoglobin Formation ◽  
Oxygen Carrying Capacity ◽  
Acute And Chronic Effects

The preparation of large quantities of a stable, stroma-free hemoglobin solution without coagulant activity is described. Following infusion of this solution into phlebotomized dogs, there is no methemoglobin formation, no adverse effects on vital signs, and no demonstrable activation of blood coagulation. The hemoglobin maintains its oxygen-carrying capacity and liberates oxygen into tissues. Acute and chronic effects on renal function following infusion of this preparation were also studied and no effect on clearance of urea, creatinine, or P.A.H. could be demonstrated. There was no change in urinary output and histological sections revealed no lesions attributable to hemoglobin toxicity. It is concluded that a stroma-free hemoglobin solution may have use as a plasma expander.

Cross-linking of stroma-free hemoglobin monitored by high-performance liquid chromatography

1982 ◽  
Vol 246 (1) ◽  
pp. 65-71 ◽  
Author(s):  
Piergiorgio Pietta ◽  
Alma Calatroni ◽  
Gabriele Palazzini ◽  
Angelo Agostoni
Keyword(s):  
High Performance Liquid Chromatography ◽  
Liquid Chromatography ◽  
High Performance ◽  
Cross Linking ◽  
Free Hemoglobin
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