Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Sickle Cell Trait”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 145 records · Page 8Linked to original sources
PubMed1976

Morphological features of red blood cells in subjects with sickle cell trait: changes during exercise.

Occasional complications and even death in subjects with sickle cell trait have been attributed to severe physical exertion. However, the extent to which sickling actually occurs during exercise has not been reported. This study examined the red blood cell morphological features immediately following near maximal upright graded bicycle exercise in five asymptomatic black subjects with hemoglobin AS. Exercise produced minimal sickling in vivo, which was not proportional to the intensity of exercise. The amount of sickling in vivo was small in comparison to that observed in the presence of severe hypoxia in vitro, never exceeding 0.75%. in seven normal subjects with hemoglobin AA, exercise did not cause changes in red blood cell morphological features. We conclude that exercise may initiate sickling in subjects with sickle cell trait.

Adult↗
PubMed2002

Increased efficacy of sulfadoxine-pyrimethamine in the treatment of uncomplicated falciparum malaria among children with sickle cell trait in Western Kenya.

The role of the sickle cell hemoglobin type as a determinant of treatment outcome with sulfadoxine-pyrimethamine was retrospectively studied in young children with uncomplicated falciparum malaria who lived in an area with intense perennial malaria transmission. Between 1993 and 1997, 2795 treatments involving 813 children were monitored. Sickle cell trait (HbAS) was present in 17.7% of the children. Two-and-a-half percent of the children experienced early clinical treatment failure by day 2-3, and 17.3% of the children were parasitemic on day 7. Treatments in HbAS children were less likely than those in HbAA children to result in persistence of parasitemia by day 3 (relative risk [RR], 0.66; 95% confidence interval [CI], 0.47-0.93; P=.02) or in parasitologic treatment failure on day 7 (RR, 0.51; 95% CI, 0.36-0.71; P<.0001). These results suggest that the HbAS phenotype should be included among factors that determine sulfadoxine-pyrimethamine treatment outcome.

Animals↗
PubMed1981

Growth status of children with and without sickle cell trait.

Because of the suggestions that children with sickle cell trait (Hb/AS) are smaller and lighter, the anthropometric measurements of 50 such children from polygamous families were compared with a matched group of 50 children with normal hemoglobin (Hb/AA) who were offspring of the same fathers and were living under identical conditions. This technique was adopted in minimize the effects of heredity and nutrition. When these hereditary and nutritional effects were excluded there were no significant differences between the anthropometric measurements of the two groups.

Adolescent↗
PubMed1990

Sickle cell trait and stroke in the young adult.

Two young patients with sickle cell trait (AS haemaglobinopathy) and ischaemic stroke are reported. The stroke involved the internal carotid artery territory in one and the brainstem in the other. A review of the literature is presented to suggest that the association of sickle cell trait and cerebral infarction is more than coincidental. Haemoglobin electrophoresis should be undertaken routinely in young subjects with ischaemic stroke.

Adolescent↗
PubMed1975

Patterns of hemoglobin assembly in reticulocytes of sickle cell trait individuals.

Venous blood was obtained from five sickle cell trait donors with relatively high hemoglobin S concentrations (40% of total hemoglobin) and five donors with unusually low hemoglobin S concentrations (25 to 30%). A fraction of cells with 15 to 20% reticulocytes was isolated from the blood and incubated with [3H]leucine in a medium supporting protein synthesis for various times from 1.25 to 60 min. Previous studies showed an imbalance in globin chain synthesis in reticulocytes of "low hemoglobin S" donors which suggested the presence of an alpha-thalassemia gene; reticulocytes of "high hemoglobin S" donors had balanced globin chain synthesis (DeSimone, J., Kleve, L., Longley, M.A., and Shaeffer, J. (1974) Biochem. Biophys. Res. Commun. 59, 564-569). In the present study the soluble phase of the 3H-labeled reticulocytes was examined by electrophoresis on strips of cellulose acetate. The tetramer hemoglobins A and S were separated from each other and from a small pool of free, newly synthesized alpha and beta chains. Kinetics of labeling studies showed that the free alpha and beta chains were intermediates in tetramer hemoglobin assembly. The distribution of radioactivity between the alpha and beta chains of each of the electrophoretically isolated components were determined by separation of their globin chains on CM-cellulose columns. After 5 min of 3H-labeling of the reticulocytes from donors with 40% hemoglobin S the ratio of newly synthesized alpha chains to beta chains in the tetramer hemoglobins A and S ranged from 0.37 to 0.58. This ratio increased with longer labeling times. Almost all of the radioactivity of the free chain intermediates was in the alpha chain. These results confirmed the presence of a significant pool of newly synthesized alpha chains and a normal pattern of hemoglobin assembly in which initially unlabeled alpha chains combined with labeled beta chains when the cells were exposed to [3H]leucine. Conversely, in the reticulocytes of donors with 25 to 30% hemoglobin S the ratio of newly synthesized alpha chains to beta chains in the completed hemoglobins A and S ranged from 0.96 to 1.37 and remained unchanged throughout the 3H-labelling period. The radioactivity of the free alpha chain pool was substantially less that the total radioactivity of the betaA and betaS chain pools. These results confirmed the existence of a decreased pool size of soluble alpha chain intermediates and a pattern of hemoglobin assembly consistent with the presence of the alpha-thalassemia gene.

Adult↗
PubMed1983

Pregnancy in women with sickle cell trait.

The complications and outcome of 334 pregnancies in women with sickle cell trait between 1975 and 1981 are reviewed. The perinatal mortality rate was 6 per 1000. The main maternal complications were recurrent urinary tract infections (6%) and haematuria (16.5%). Fetal distress in labour was seen in 17% and 11.5% were delivered by emergency caesarean section. Compared with a comparable group of women without sickle cell trait, the mean birthweight of the babies (3202 g) was not reduced. The mean age of the mothers (25 years), their mean parity (1.46) and the percentage who were para 4 or more (13%) were all significantly increased, with important implications for the continuing transmission of the sickle cell gene.

Adult↗
PubMed1980

Interaction of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in Cameroon.

The prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell trait was determined in 371 Cameroonian males and 668 male blood donors in Chicago. The number of males with both sickle cell trait and G6PD deficiency was significantly greater than expected (p less than 0.05) in Cameroon. The number of males with both sickle cell trait and G6PD deficiency in the Chicago population also exceeded the exptected number, although this was not statistically significant (p greater than 0.30). A young red cell population associated with the sickle cell gene leading to elevated G6PD levels in G6PD-deficient males suggests that sickle hemoglobin may exert a beneficial effect on G6PD deficiency, rather than the opposite, as had previously been proposed. These red cells may be better able to deal with oxidative stress, which can precipitate severe hemolytic disease in G6PD deficiency.

Anemia, Sickle Cell↗
PubMed1975

Physical complaints of patients with sickle cell trait.

Prospective and retrospective experience with physical complaints of 112 oral contraceptive and 67 intrauterine device Black family planning patients with sickle cell trait was evaluated by comparing their cumulative complaint rates with a randomly selected control group of non-sickle cell trait family planning patients having the same method, age and parity distribution as the sickle cell trait patients. Physical complaints considered are: dysmenorrhea, mental depression, vaginal discharge, fatigue, headaches, and nausea-vomiting. No evidence of a significant difference at level .05 is found between cumulative complaint rates of sickle cell trait and control patients for either oral or intrauterine device users for any of the complaint types. Further clinical research may be desirable in this area for dysmenorrhea and mental depression.

Anemia, Sickle Cell↗
PubMed1978

Growth and development in children with sickle-cell trait. A prospective study of matched pairs.

To ascertain if sickle-cell trait (Hb AS) impairs physical growth and cognitive development, we prospectively investigated 50 matched pairs of black children. For each child with Hb AS, an Hb AA child was matched at birth for sex, birth date, birth weight, gestational age, five-minute Apgar score and socioeconomic status. Between the ages of three and five years, the members of each matched pair were evaluated, with one month of one another, by persons "blind" to the hemoglobin genotype. Twelve outcome measurements were obtained at evaluation: height, weight, head circumference, skin-fold thickness, cross-sectional area of arm muscle, bone age, five scores of the McCarthy Scales of Children's Abilities, and the Peabody Picture Vocabulary Test. There were no statistically significant differences in these measurements favoring the AA group. The results show that children with sickle-cell trait in this age group have no deficits in standard measurements of growth and development and emphasize the importance of rigorous methods when clinical groups are assembled and compared.

Anemia, Sickle Cell↗
PubMed1977

Evidence for rapid loss of newly synthesized haemoglobin S molecules in sickle cell anaemia and sickle cell trait.

The present study indicates that newly completed haemoglobin S molecules rather than free betas-chains are preferentially bound to the reticulocyte stroma of individuals with sickle cell trait and sickle cell anaemia. Reticulocytes from indivdiuals with HbAA, AS and SS were incubated with [3H]eucine from 1.25 min to 120 min. Unlike the stroma-free haemolysates, the stroma of all individuals contained an excess of labelled beta-chains relative to alpha-chains after short incubation times. In haemoglobin AA and AS individuals, the stromal betaA radioactivity was 1--2% of the total cellular betaA radioactivity. In haemoglobin AS and SS individuals, the stromal betaS radioactivity was 3--5% and 10--20% of the total cellular betaS radioactivity, respectively. All of the stroma beta-chain radioactivity was associated with completed haemoglobin molecules. Because of the unlabelled free alpha-chain pool found in reticulocytes, after short incubation times newly completed haemoglobin molecules have predominantly labelled beta-chains and unlabelled alpha-chains. These findings suggest that part of the discrepancy between the stroma and stroma-free haemolysate alpha/beta radioactivities seen in HbAS and HbSS individuals may result from normal labelling kinetics. A pulse chase experiment performed on an individual with HbSS revealed that comleted HbS molecules, in addition to being associated with the stroma, were lost from the cell.

Anemia, Sickle Cell↗
PubMed1987

Pulmonary function in sickle cell trait.

Pulmonary function abnormalities, which have been reported to occur in persons with sickle cell trait (hemoglobin AS), could intensify the hypoxic stimulus for the systemic formation of sickle cells at high altitude. We sought to determine whether pulmonary function abnormalities occur as a result of exposure to high altitudes in persons with hemoglobin AS. In a prospective study, 13 men with hemoglobin AS ("cases") and 13 controls (hemoglobin AA) matched by age, sex, and race were exposed to five to seven altitude simulations (ranging from 1524 to 7620 m [5000 to 25,000 ft]) in a hypobaric chamber. Measurements of diffusing capacity for carbon monoxide (DLco), forced vital capacity, forced expiratory volume in one second, and forced midexpiratory flow were obtained before and after each exposure. Data before exposures did not differ statistically between cases and controls. Altitude had no systematic effect on DLco or spirometric values in cases compared with values in controls (p greater than 0.05). Individual declines in forced vital capacity or DLco of more than 10% occurred with similar frequency in both groups. Measurements made after the series of exposures showed no change from those made before. We conclude that short serial exposures to hypoxia at high altitudes does not acutely or cumulatively alter DLco or spirometric values in healthy, nonexercising persons with sickle cell trait.

Adult↗
PubMed1975

Alpha thalassaemia in adults with sickle-cell trait.

Mild forms of alpha thalassaemia are difficult to detect in adults. Since alpha thalassaemia existing with structural defects of the beta chain of haemoglobin may lead to decreased levels of the abnormal haemoglobin, we examined individuals having sickle-cell trait for the possible coexistence of alpha thalassaemia. Patients with sickle-cell trait having haemoglobin-S (Hb-S) levels less than commonly expected were compared to two control groups--one with sickle-cell trait and the usual levels of Hg S and one with normal haemoglobin. Twenty-one patients with sickle-cell trait having Hb-S concentrations below 35% had 65.8% Hb A, 31.8% Hb S, and a mean corpuscular volume of 81.6 fl. Studies of the relative rates of alpha-, betaS- and betaA-chain synthesis in 14 of these individuals showed a mean alpha:beta ratio of 0.76. In patients with normal haemoglobin as well as in sickle-cell trait with Hb-S levels above 35%, this ratio was unity. These findings are consistent with the presence of alpha thalassaemia in patients with sickle-cell trait who have lower than usual levels of Hb S and microcytosis.

Adult↗
PubMed1984

Hypothermic cardiopulmonary bypass in a patient with sickle-cell trait.

A male patient, aged 7.5 years, with known sickle cell trait, presented for correction of Fallot's tetralogy. The successful management under hypothermic cardiopulmonary bypass is described and the behaviour of sickle haemoglobin under hypothermic conditions is discussed.

Anemia, Sickle Cell↗
PubMed1994

Serum lipids and atherogenic risk in sickle-cell trait carriers.

This work studies the relationship between a common genetic marker in our population, sickle-cell trait, and atherogenic risk factors. One hundred and twenty-two healthy subjects were studied. Forty-six subjects had normal haemoglobin (HB-AA) while 76 had sickle-cell trait Hb-AS. Mean cholesterol level was higher in subjects with sickle-cell trait (2.82 +/- 0.21 g/I) than in subjects with normal haemoglobin (2.08 +/- 0.40 g/I) used as controls. The difference was statistically significant (p < 0.05). HDL cholesterol levels remained in the normal range in controls (Hb-AA) (0.560 +/- 0.10 g/I) while subjects with Hb-AS had high HDL-c levels (0.618 +/- 0.30 g/I). Mean LDL cholesterol levels in Hb-AS subjects were about 57% higher than those in controls (1.84 +/- 0.10 g/I and 1.17 +/- 0.30 g/I, respectively) (p < 0.05). Apolipoprotein A1 levels of Hb-AS subjects were lower (1.37 +/- 0.10 g) than those of Hb-AA subjects (1.98 +/- 0.15 g/I), (p < 0.05) while apolipoprotein B concentrations of Hb-AS subjects were higher (1.70 +/- 0.27 g/I) than in controls (1.30 +/- 0.10 g/I) (p < 0.05). The atherogenicity index given by the Apo-B/Apo-AI ratio was higher in Hb-AS subjects (1.24) than in those with normal haemoglobin (0.65). We conclude that Hb-AS subjects are at high atherogenic risk if subsequent diet management is not undertaken.

Adolescent↗
PubMed1979

Gross hematuria associated with sickle cell trait and von Willebrand's disease.

A case of recurrent gross hematuria, sickle cell trait and von Willebrand's disease is reported. The gross hematuria abated promptly after the institution of cryoprecipitate therapy. The importance of considering von Willebrand's disease in the differential diagnosis in patients with sickle cell trait and hematuria is discussed.

Adolescent↗
PubMed1987

Malignant fibrous histiocytoma in bone infarct. Association with sickle cell trait and alcohol abuse.

A 42-year-old black man with sickle cell trait and a history of alcohol abuse had a malignant fibrous histiocytoma of the distal femur. The tumor developed in a bone infarct that had been present for at least 54 months. This is the first reported association of malignant fibrous histiocytoma and bone infarct in which the infarct was related to sickle cell trait and alcohol abuse. A detailed map of the sections from the resected femur disclosed a spectrum of cytologic changes in the sarcoma that ranged from bland fibroblasts in the center of the tumor to high-grade sarcoma at the periphery. Awareness of this spectrum has diagnostic importance regarding the potential underdiagnosis of well-differentiated malignant fibrous histiocytoma.

Adult↗
PubMed2006

Using qualitative and quantitative strategies to evaluate knowledge and perceptions about sickle cell disease and sickle cell trait.

OBJECTIVES: To evaluate knowledge, perceptions and the effectiveness of different sources of information about sickle cell trait (SCT) and sickle cell disease (SCD); to determine individual knowledge of SCT status. METHODS: 28 individuals participated in three focus groups (healthcare providers, people affected by SCD or SCT, and community members). Surveyors interviewed 282 respondents within their neighborhoods. RESULTS: Common themes across the focus groups included the limited general awareness of SCD and SCT, the emphasis on the benign nature of SCT rather than on future implications, and the need for public health education campaigns about SCD and SCT involving media strategies. The majority of community survey respondents (n = 243, 86.2%) had correct general knowledge about the genetic basis and severity of SCD, but only 16% (n = 45) knew their own trait status. When respondents had received information about SCD from friends and acquaintances, they were three times more likely to know their SCT status, compared with respondents who had not received information from a personal source (p < 0.01). CONCLUSIONS: Despite a screening history in the 1970s fraught with controversy, sickle cell disease management and detection can be a model for the empowerment of communities in making informed decisions about theirs and their families' futures, given the burgeoning of genetic information.

Adolescent↗
PubMed1983

A case-control study of the association of sickle cell trait and chronic open-angle glaucoma.

Chronic open-angle glaucoma is reported to be more common in blacks than in whites. The reasons for a higher prevalence of this disease in blacks are unclear. Two recent small case series from clinical practice have been published in which secondary open-angle glaucoma was diagnosed in blacks with sickle cell trait who had experienced either traumatic or nontraumatic hyphema. The association of sickle cell trait and chronic open-angle glaucoma has been tested only in a small case-control study of uncontrolled chronic open-angle glaucoma patients undergoing surgery. No significant association was found. However, the higher prevalence of sickle cell trait in blacks compared with whites raises the question of whether the presence of sickle hemoglobin is a risk factor for chronic open-angle glaucoma in the black population. To test for a possible association between this disease and sickle cell trait, we conducted a case-control study in black ophthalmic patients at the University of Pennsylvania and affiliated hospitals between December 1981 and July 1982. Sickle cell trait, as measured by the sickle cell preparation test, was no more prevalent in patients with chronic open-angle glaucoma than in age- and sex-matched ophthalmic clinic controls. It is unlikely that hemoglobin SA is a risk factor for chronic open-angle glaucoma.

Adult↗