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Sickle Cell Trait (sickle + cell_trait)
Selected AbstractsSickle Cell Anaemia and Deaths in Custody in the UK and the USATHE HOWARD JOURNAL OF CRIMINAL JUSTICE, Issue 1 2006SIMON M. DYSON Sickle cell anaemia is a serious inherited blood disorder disproportionately affecting minority ethnic groups. Sickle cell trait is the genetic carrier state and not an illness. The evidence suggests that the treatment of sickle cell in the criminal justice system is twofold. Justice authorities have misused sickle cell trait to explain away ten sudden deaths, often associated with forced restraint, of African-Caribbean people in custody. Meanwhile, seven deaths have been attributable to lack of provision of health care for those prisoners suffering from the illness sickle cell anaemia. [source] Optical tweezers for measuring red blood cell elasticity: application to the study of drug response in sickle cell diseaseEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 4 2003M. M. Brandão Abstract: The deformability of erythrocytes is a critical determinant of blood flow in microcirculation. By capturing red blood cells (RBC) with optical tweezers and dragging them through a viscous fluid we were able to measure their overall elasticity. We measured, and compared, the RBC deformability of 15 homozygous patients (HbSS) including five patients taking hydroxyurea (HU) for at least 6 months (HbSS/HU), 10 subjects with sickle cell trait (HbAS) and 35 normal controls. Our results showed that the RBC deformability was significantly lower in haemoglobin S (HbS) subjects (HbSS and HbAS), except for HbSS/HU cells, whose deformability was similar to the normal controls. Our data showed that the laser optical tweezers technique is able to detect differences in HbS RBC from subjects taking HU, and to differentiate RBC from normal controls and HbAS, indicating that this is a very sensitive method and can be applied for detection of drug-response in sickle cell disease. [source] Separation of haemoglobin HbE and HbA2 by the fully automated, high-pressure liquid chromatography Tosoh HLC-723 G7 analyzerINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 5 2008G. LIPPI Summary High-pressure liquid chromatography instruments specifically devised for separating haemoglobin (Hb) fractions have been increasingly employed by the hospital laboratories over the recent years since they allow easy and fast screening for several Hb variants. Although such instruments may be proposed as sensitive, specific and reliable alternatives to the classic electrophoretic techniques, a major drawback of this screening strategy is the almost identical retention time of several Hb variants. In particular, at least 18 Hb variants have been reported in the same retention window as HbA2, including HbE, the second most common ,-chain variant in humans after sickle cell trait. Recently, we evaluated the performance characteristics of an improved buffer formulation originally conceived for Hb variants separation procedures on the fully automated high-pressure liquid chromatography instrument Tosoh G7. At variance with other fully automated high-pressure liquid chromatography analyzers, the elution pattern on the G7 in subjects heterozygous for HbE is characterized by the presence of four suggestive peaks (HbF, HbA, HbA2 and HbE), confirming the effective separation of HbE from HbA2. Because of its potential value in the diagnosis of the thalassaemia syndromes, the effective separation of HbA2 from HbE can provide clinical laboratories with a valuable information for the diagnostic reasoning. [source] Sickle Cell Trait Mimicking Multiple Inflicted Injuries in a 5-Year-Old BoyJOURNAL OF FORENSIC SCIENCES, Issue 5 2009Charis Kepron M.D. Abstract:, Sickle cell disease (SCD) and sickle cell trait (SCT) can be associated with sudden unexpected death in the pediatric population, usually due to pulmonary complications occurring within the acute chest syndrome (ACS). Musculoskeletal complications can occur and are classically limited to bone infarcts. The occurrence of bone pathology centered upon the epiphyseal growth plate in SCD/SCT is extremely rare, and multiple such injuries in a single patient have not been previously reported. Herein, we describe a case of sudden unexpected death in a 5-year-old child with undiagnosed SCT due to the ACS, with widespread epiphyseal and periosteal bone lesions mimicking multiple inflicted injuries at autopsy. This case highlights the importance of clinicopathological correlation and is the first to describe SCT pathology as a mimic of nonaccidental injury. [source] Hematological predictors of increased severe anemia in Kenyan children coinfected with Plasmodium falciparum and HIV-1,AMERICAN JOURNAL OF HEMATOLOGY, Issue 4 2010Gregory C. Davenport Malaria and HIV-1 are coendemic in many developing countries, with anemia being the most common pediatric hematological manifestation of each disease. Anemia is also one of the primary causes of mortality in children monoinfected with either malaria or HIV-1. Although our previous results showed HIV-1(+) children with acute Plasmodium falciparum malaria [Pf(+)] have more profound anemia, potential causes of severe anemia in coinfected children remain unknown. As such, children with P. falciparum malaria (aged 3,36 months, n = 542) from a holoendemic malaria transmission area of western Kenya were stratified into three groups: HIV-1 negative [HIV-1(,)/Pf(+)]; HIV-1 exposed [HIV-1(exp)/Pf(+)]; and HIV-1 infected [HIV-1(+)/Pf(+)]. Comprehensive clinical, parasitological, and hematological measures were determined upon enrollment. Univariate, correlational, and hierarchical regression analyses were used to determine differences among the groups and to define predictors of worsening anemia. HIV-1(+)/Pf(+) children had significantly more malarial pigment-containing neutrophils (PCN), monocytosis, increased severe anemia (Hb < 6.0 g/dL), and nearly 10-fold greater mortality within 3 months of enrollment. Common causes of anemia in malaria-infected children, such as increased parasitemia or reduced erythropoiesis, did not account for worsening anemia in the HIV-1(+)/Pf(+) group nor did carriage of sickle cell trait or G6PD deficiency. Hierarchical multiple regression analysis revealed that more profound anemia was associated with elevated PCM, younger age, and increasing HIV-1 status ([HIV-1(,) , HIV-1(exp) , HIV-1(+)]. Thus, malaria/HIV-1 coinfection is characterized by more profound anemia and increased mortality, with acquisition of monocytic pigment having the most detrimental impact on Hb levels. Am. J. Hematol., 2010. © 2010 Wiley-Liss, Inc. [source] Physical fitness indices and anthropometrics profiles in schoolchildren with sickle cell trait/diseaseAMERICAN JOURNAL OF HEMATOLOGY, Issue 2 2007Hisham Moheeb Abstract The current studies aimed at determining physical fitness indices and anthropometrics profiles of school children with sickle cell trait (SCT) and sickle cell disease (SCD). Male school children (150) comprising 3 Groups participated in the studies. Group 1 has 50 normal healthy controls, while Groups 2 and 3 each has 50 children who were suffering from SCT and SCD, respectively. Anthropometrics measurement and parameters of physical fitness were assessed in all subjects. All children were also subjected to a 5-min running exercise test on a flat motorized treadmill at speed corresponding to 5 km/hr. Throughout the test, heart rate was monitored and recoded during exercise and for 10-min during recovery. Blood lactate was measured before and 5 min following the completion of test. The mean values of lean body mass and height were lower in the SCD children (P < 0.05) compared with the healthy subjects and SCT individuals. Children with SCD exhibited a higher mean value (P < 0.05) for percent body fat and fat mass than the normal healthy subjects and SCT individuals. Although all groups tolerated well the treadmill exercise protocol, the SCD group exhibited higher (P < 0.05) mean values of heart rate during exercise than those observed in the SCT and normal control children. In addition, SCD children showed higher serum lactate values before and after treadmill exercise compared to the other groups. Children with SCD exhibit high level of adiposity; low level of fitness and their exercise performance appears to be physiologically more stressful as indicated by heart rate and blood lactate concentration responses. Am. J. Hematol., 2006. © 2006 Wiley-Liss, Inc. [source] Communication of positive newborn screening results for sickle cell disease and sickle cell trait: Variation across states,AMERICAN JOURNAL OF MEDICAL GENETICS, Issue 1 2008Patricia L. Kavanagh Abstract In the US, all states and the District of Columbia have universal newborn screening (NBS) programs for sickle cell disease (SCD), which also identify sickle cell trait (trait). In this project, we surveyed follow-up coordinators, including one in the District of Columbia and two in Georgia, about protocols for stakeholder notification for SCD and trait. The primary outcomes were total number and type of stakeholder informed of a positive screen. We received 52 completed surveys (100% response). Primary care providers (PCPs) (100%), hematologists (81%), hospitals (73%), and families (40%) were the most commonly notified stakeholders of positive SCD screens, while PCPs (88%), hospitals (63%), and families (37%) were most commonly notified for trait. On average, 3.4 stakeholders were notified for a positive screening for SCD, compared to 2.4 stakeholders for sickle cell trait (P,<,0.001). In multivariate analyses for SCD, we found a 2.9% increase in stakeholders notified for each additional year of universal screening mandated in a state (95% CI: 1.4,4.4%). For trait, we found an 8.5% increase in stakeholders notified for each additional follow-up staff (95% CI: 1.3,15.7%), and a 1.3% increase for each additional percent of black births in the state (95% CI: 0.1,2.5%). Wide variation exists in stakeholder notification by NBS programs of positive screenings for SCD and trait. This variation may alter the effectiveness of NBS programs by location of birth. © 2008 Wiley-Liss, Inc. [source] Sickle Cell Anaemia and Deaths in Custody in the UK and the USATHE HOWARD JOURNAL OF CRIMINAL JUSTICE, Issue 1 2006SIMON M. DYSON Sickle cell anaemia is a serious inherited blood disorder disproportionately affecting minority ethnic groups. Sickle cell trait is the genetic carrier state and not an illness. The evidence suggests that the treatment of sickle cell in the criminal justice system is twofold. Justice authorities have misused sickle cell trait to explain away ten sudden deaths, often associated with forced restraint, of African-Caribbean people in custody. Meanwhile, seven deaths have been attributable to lack of provision of health care for those prisoners suffering from the illness sickle cell anaemia. [source] Co-inheritance of ,+ -thalassaemia and sickle trait results in specific effects on haematological parametersBRITISH JOURNAL OF HAEMATOLOGY, Issue 2 2006Sammy Wambua Abstract Both the sickle cell trait (HbAS) and ,+ -thalassaemia are common in many tropical areas. While their individual haematological effects are well described, few studies describe their effects when inherited together. We present data from the Kenyan coast, which suggest that HbAS and ,+ -thalassaemia may interact to produce specific effects on haematological parameters. Overall, the difference in Hb concentrations between non-thalassaemics (,,/,,) and ,+ -thalassaemia homozygotes (,,/,,) was greater in non-HbAS (HbAA) (0·63 g/dl) than in HbAS children (0·25 g/dl). HbAS also ameliorated both the reduced mean cell volume and mean cell haemoglobin normally associated with the ,,/,, genotype. Potential mechanisms and implications are discussed. [source] Renal medullary carcinoma, a rare cause of haematuria in sickle cell traitBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2006Ketan Patel No abstract is available for this article. [source] | ||||||||||