Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may...Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may be a clinically benign trait, whereas others indicate it may behave similarly to a β+ thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a rare double heterozygous disorder with little known clinical characteristics. We report the hematologic and clinical data on three patients with Hb S/KW to help describe the characteristics of this patient population. The first two cases represent first cousins, ages 3 and 2 years. They are clinically asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV) levels without reticulocytosis. The third case is of a 14-year-old male who is non-anemic with no microcytosis. He has been clinically well except for abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients have Hb S levels slightly higher than typically observed with sickle cell trait and a delay of hemoglobin F to adult levels. There exists a need for more reports to better delineate the clinical course and management of these patients.展开更多
Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas an...Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas analysis (LabHb) as a reference. We aimed to determine the extent to which in vivo adjustment (ADJ) for results of initial blood gas analysis would improve the accuracy of SpHb measurement. Methods: Data on LabHb and SpHb levels were collected from cases with hemorrhage of 1000 g or more. Correlations and Bland-Altman analyses were used to determine the associations between data before and after ADJ. Results: ADJ slightly improved the correlation coefficient (Pearson r) between SpHb and LabHb levels from 0.65 to 0.72. In Bland-Altman analysis, ADJ reduced the bias from 1.76 ± 1.47 g/dL to 0.64 ± 1.43 g/dL, while the 95% limits of agreement of -1.12 to 4.64 g/dL (range: 5.77 g/dL) without ADJ improved to -2.16 to 3.44 g/dL (range: 5.59 g/dL) with ADJ. In four-quadrant analysis, other than samples in the exclusion zone, the total number analyzed was 326, and the concordance rate was 82%. Conclusion: Although the accuracy of SpHb measurement improves on ADJ, SpHb measurement cannot substitute for LabHb levels, and it is necessary to determine Hb levels with blood gas analysis in the laboratory. It remains unclear which measurement is superior for determining when to initiate blood transfusion to achieve better outcomes. To comply with conventional methods, LabHb measurements may be necessary.展开更多
Subsequent studies have demonstrated the reversed activity of the aqueous extract of Ceiba pentandra on the deformity of sickled red blood cells in hypoxia conditions. The observation which related to an in vitro stud...Subsequent studies have demonstrated the reversed activity of the aqueous extract of Ceiba pentandra on the deformity of sickled red blood cells in hypoxia conditions. The observation which related to an in vitro study had given rise to hopes as to the management of sickle cell disease (SCD) by the use of this plant species. In this paper, the authors aimed to investigate the effect of the aqueous extract of C. pentandra on the production of fetal hemoglobin in SCD patients. The work carried out hemoglobin electrophoresis, for a period of six months, on blood samples from SCD patients who voluntarily undergone routine treatment, based on the medicinal recipe prepared from the bark of the trunk and branches of C. pentandra, in a hospital center of herbal medicines located in Kinshasa. The medicinal recipe called BEAT-SS is a patented product of the hospital center named Centre de Phytothérapie Moderne NIECA. Blood samples from patients under treatment were taken to evaluate the behavior of different forms of hemoglobin (hemoglobin S, hemoglobin F and hemoglobin A2). Agarose gel electrophoresis with integrated reading was used for the separation of the different forms of hemoglobin, as well as their dosage on each sample of sickle blood. A reduction in the proportion of hemoglobin S and an increase in the proportion of fetal hemoglobin were found in all sickle cell patients during the treatment period. This observation could affirm that the management of sickle cell patients using the recipe prepared from the aqueous extract of C. pentandra could increase the level of fetal hemoglobin in these patients.展开更多
文摘Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may be a clinically benign trait, whereas others indicate it may behave similarly to a β+ thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a rare double heterozygous disorder with little known clinical characteristics. We report the hematologic and clinical data on three patients with Hb S/KW to help describe the characteristics of this patient population. The first two cases represent first cousins, ages 3 and 2 years. They are clinically asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV) levels without reticulocytosis. The third case is of a 14-year-old male who is non-anemic with no microcytosis. He has been clinically well except for abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients have Hb S levels slightly higher than typically observed with sickle cell trait and a delay of hemoglobin F to adult levels. There exists a need for more reports to better delineate the clinical course and management of these patients.
文摘Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas analysis (LabHb) as a reference. We aimed to determine the extent to which in vivo adjustment (ADJ) for results of initial blood gas analysis would improve the accuracy of SpHb measurement. Methods: Data on LabHb and SpHb levels were collected from cases with hemorrhage of 1000 g or more. Correlations and Bland-Altman analyses were used to determine the associations between data before and after ADJ. Results: ADJ slightly improved the correlation coefficient (Pearson r) between SpHb and LabHb levels from 0.65 to 0.72. In Bland-Altman analysis, ADJ reduced the bias from 1.76 ± 1.47 g/dL to 0.64 ± 1.43 g/dL, while the 95% limits of agreement of -1.12 to 4.64 g/dL (range: 5.77 g/dL) without ADJ improved to -2.16 to 3.44 g/dL (range: 5.59 g/dL) with ADJ. In four-quadrant analysis, other than samples in the exclusion zone, the total number analyzed was 326, and the concordance rate was 82%. Conclusion: Although the accuracy of SpHb measurement improves on ADJ, SpHb measurement cannot substitute for LabHb levels, and it is necessary to determine Hb levels with blood gas analysis in the laboratory. It remains unclear which measurement is superior for determining when to initiate blood transfusion to achieve better outcomes. To comply with conventional methods, LabHb measurements may be necessary.
文摘Subsequent studies have demonstrated the reversed activity of the aqueous extract of Ceiba pentandra on the deformity of sickled red blood cells in hypoxia conditions. The observation which related to an in vitro study had given rise to hopes as to the management of sickle cell disease (SCD) by the use of this plant species. In this paper, the authors aimed to investigate the effect of the aqueous extract of C. pentandra on the production of fetal hemoglobin in SCD patients. The work carried out hemoglobin electrophoresis, for a period of six months, on blood samples from SCD patients who voluntarily undergone routine treatment, based on the medicinal recipe prepared from the bark of the trunk and branches of C. pentandra, in a hospital center of herbal medicines located in Kinshasa. The medicinal recipe called BEAT-SS is a patented product of the hospital center named Centre de Phytothérapie Moderne NIECA. Blood samples from patients under treatment were taken to evaluate the behavior of different forms of hemoglobin (hemoglobin S, hemoglobin F and hemoglobin A2). Agarose gel electrophoresis with integrated reading was used for the separation of the different forms of hemoglobin, as well as their dosage on each sample of sickle blood. A reduction in the proportion of hemoglobin S and an increase in the proportion of fetal hemoglobin were found in all sickle cell patients during the treatment period. This observation could affirm that the management of sickle cell patients using the recipe prepared from the aqueous extract of C. pentandra could increase the level of fetal hemoglobin in these patients.