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 217 records · Page 12Linked to original sources
PubMed2007

Haemostatic variables and their relationship to body mass index and blood pressure in adult Nigerians with the sickle cell trait.

Several studies had assessed the rheological and haemostatic parameters in both sickle cell anaemia(HbSS) and haemoglobin AA(HbAA) subjects. The effect of the heterogeneous state on these parameters has not been fully determined. Our aim was to assess the haemorheological and haemostatic parameters and there relationship to body mass index in individuals with the sickle cell trait. Fifty eight males (41 HbAA and 17 HbAS) who were neither smokers nor drinkers were studied. Body mass index was calculated as weight in Kg divided by height in m(2), blood samples were analysed for haematocrit, erythrocyte sedimentation rate, platelets, fibrinogen and factor VII by standard methods. There were no significant differences in the levels of haemostatic and cardiovascular risk factors between the HbAA and HbAS individuals. Fibrinogen correlated positively with BMI (r=0.6014, p=0.0107), systolic blood pressure (r=0.4820, p=0.0570), diastolic blood pressure (r=0.4699, p=0.0570) in HbAS individuals. The body mass index also correlated positively with diastolic blood pressure (r=0.5182, p<0.05), and systolic blood pressure (r=0.6189, p<0.01). Positive associations were also found between fibrinogen and body mass index (r=0.5012, p=0.0008), and fibrinogen and haematocrit (r=0.3696, r=0.0174) respectively in HbAA subjects. We conclude that the lack of significant difference in both haemorheological and cardiovascular risk factors in HbAS and HbAA subjects has further supported the observation that HbAS is a benign condition under physiological settings. Also the positive associations of fibrinogen with other cardiovascular risk factors have further underscored the central role of fibrinogen as an independent risk factors in the development of cardiovascular diseases.

Adult↗
PubMed1980

The risk of sudden death in sickle cell trait: noninvasive assessmanet of cardiac response to exercise.

The effect of symptom-limited maximal treadmill exercise was evaluated in 34 patients with sickle cell trait (SCT) and 43 controls. Systolic time intervals were measured before and after exercise in 15 of the sickle cell trait subjects, and in 11 controls. Rate pressure product, degree of ST segment depression and incidence of ventricular arrhythmias in subjects with SCT were not significantly different from similar measurements in the control group. No significant difference in electromechanical systole QS2, left ventricular ejection time (LVET), pre-ejection period (PEP)/LVET or ejection fraction (EF) were found between the groups. These data indicate that healthy subjects with SCT do not have an increased incidence of exercise-induced ventricular arrhythmias, myocardial ischemia, or diminished ventricular function compared to normals. Further studies are indicated to elucidate factors that may be operative in sudden death syndrome.

Adolescent↗
PubMed1976

Exercise capacity of black sickle cell trait males.

Aerobic capacity data were collected on matched groups of 16 black sickle cell trait males and 16 non-trait males. The subjects were matched on race, age, weight, sex, physical activity profile, skinfolds, and smoking habits. Total treadmill times, predicted and actual Vo2 max, max HR, and recovery HR were compared. The Bruce Multi-Stage Treadmill Test was administered with continuous ECG and OCC (Oxygen Consumption Computer) monitoring. The results indicated no significant difference (P greater than .05) between the trait and non-trait subjects on any of the performance variables.

Adolescent↗
PubMed2002

Sickle Hb polymerization in RBC components from donors with sickle cell trait prevents effective WBC reduction by filtration.

BACKGROUND: RBC components collected from donors with sickle cell trait frequently occlude WBC-reduction filters. In vitro, sickle trait RBCs have the potential for sickle Hb (Hb S) polymerization at low oxygen saturations and high Hb concentrations. STUDY DESIGN AND METHOD: To determine if the low pH and high osmolarity of the CP2D used in the collection contributed to filter failures, the filterability of sickle trait donor RBCs collected in CP2D was compared with RBCs from the same donors collected in heparin. RESULTS: Five of six sickle trait components collected in CP2D did not complete filtration, but all six RBC components collected in heparin filtered completely. RBC components collected in CP2D from four other sickle trait donors were divided in two, and one-half was treated with carbon monoxide to convert Hb S to its liganded form to prevent Hb S polymerization. All four carbon monoxide-treated components filtered within 9 minutes, but only one untreated component filtered completely. RBC components collected by apheresis contained less CP2D, and five of seven sickle trait apheresis components filtered completely; four of the five filtered rapidly (<15 min) and one filtered in 100 minutes. Hb oxygen saturation was greater in the four rapidly filtering apheresis RBC components (68 +/- 9%) than in the three that filtered slowly or incompletely (37 +/- 5%, p = 0.03). CONCLUSIONS: Hb S polymerization appears responsible for RBC WBC-reduction filter failures. Citrate anticoagulant and low oxygen saturation are responsible in part for Hb S polymerization in this setting.

Anticoagulants↗
PubMed1976

Association of Duffy blood groups with the sickle cell trait.

Among a small group of Saudi Arabs the Duffy blood group (Fy(a-b-)) was found to be associated with the sickle cell trait more often than could be explained by random gene combination thus supporting the concept that African gene flow brought the sickle cell trait to the Arabian peninsula. Based on recent observations concerning the possible role of Fy(a-b-) as a resistance factor against vivax malaria, it is proposed that the gene combination AS FyFy is likely to be selected in areas where falciparum and vivax infections are endemic and mixed infections occur frequently.

Anemia, Sickle Cell↗
PubMed1989

Detection of alpha thalassaemia in sickle cell trait patients by Hb-Bart's screening & quantitation of Hb-A & Hb-S.

Haemoglobin fractionation in 27 subjects with sickle cell trait revealed 12 (44%) with sickle cell haemoglobin less than 35 per cent (23.4-34.2%, mean 30.4 +/- 3.3%) suggesting an association of alpha thalassaemia. Electrophoresis of 91 samples of cord blood revealed demonstrable amounts of haemoglobin Bart's in 7 (7.7%); six between 5 and 10 per cent and one with less than 2 per cent. It appeared that the six infants with higher amounts of haemoglobin Bart's were homozygous for alpha-thalassaemia + (-a/-a) genotypes and one was heterozygous for alpha-thalassaemia + (-a/aa) Results of haemoglobin electrophoresis done on 2754 blood samples analysed from hospital records, retrospectively did not reveal haemoglobin-H and haemoglobin Constant Spring in any of the samples.

Anemia, Sickle Cell↗
PubMed1999

Renal medullary carcinoma in an adolescent with sickle cell trait.

We describe the complex presentation of a patient with renal medullary carcinoma, a newly described entity primarily affecting young patients with sickle cell trait. Renal medullary carcinoma is an aggressive, rapidly destructive tumor associated with a delayed diagnosis and a poor outcome. The most common presenting signs and symptoms include hematuria, abdominal or flank pain, and weight loss. Sickle cell trait as the sole cause of hematuria in young black patients is a diagnosis of exclusion. Hemoglobin electrophoresis, intravenous pyelography, and computed tomography scans should be the minimal studies performed in young black patients with hematuria.

Adolescent↗
PubMed1978

Sickle cell trait: possible contributory cause of renal allograft failure.

A black male with known sickle cell trait (hemoglobin AS) received two cadaveric renal transplants in succession. After excellent initial graft function he had what appeared clinically to be an acute rejection episode which could not be reversed by immunosuppressive therapy on either occasion. The histopathology of the removed allografts did not reveal a picture of acute rejection, but instead revealed extensive intravascular sickling of the erythrocytes. This is important especially for the transplant centers in the United States which may have a significant number of the black population among the renal allograft recipients.

Adult↗
PubMed1979

Aorta-coronary bypass in a patient with sickle cell trait.

A triple aorto-coronary bypass was performed in a patient with sickle cell trait. Partial exchange transfusion with normal packed erythrocytes was used in preparation of the patient for extracorporeal circulation, hypothermia and cardioplegic arrest.

Anemia, Sickle Cell↗
PubMed1988

Effect of moderate inspiratory hypoxia on exercise performance in sickle cell trait.

In previous work (Weisman IM, Zeballos RJ, Johnson BD: Cardiopulmonary and gas exchange responses to acute strenuous exercise at 1,270 meters in sickle cell trait. Am J Med 1988; 84: 377-383), no significant differences in cardiopulmonary and gas exchange responses to acute, strenuous exercise were observed between volunteers with sickle cell trait (SCT) and control subjects at an altitude of 1,270 meters. The current study was designed to evaluate the effect of a greater hypoxic stimulus on the response of healthy, black male basic recruits, 11 with SCT (HbAS) and 11 control subjects, to acute strenuous exercise. Simulated 2,300-meter and simulated sea-level conditions were achieved by adjustment of the fraction of inspired oxygen (simulated condition of 2,300 meters equal to 18 percent; simulated sea-level condition equal to 24 percent) at the same barometric pressure (656 mm Hg). For each simulated condition, the subjects performed an incremental exercise test to exhaustion on a cycle ergometer. One steady-state exercise test with radial arterial access for arterial blood gases was performed under each condition on Day 2. Peak incremental exercise values for oxygen consumption (2.9 versus 2.81 liters/minute), heart rate (189 versus 187 beats/minute), oxygen pulse (15.4 versus 15.1 ml/beat), and anaerobic threshold (1.59 versus 1.62 liters/minute), at the simulated 2,300-meter height revealed no significant differences between men with SCT and control subjects, respectively. A 5 to 9 percent decrement in exercise performance at the simulated 2,300-meter level compared with exercise performance at the simulated sea-level condition was noted for both groups. Steady-state exercise values for arterial oxygen tension (64 versus 65 mm Hg), arterial oxygen saturation (90 versus 90 percent), alveolar-arterial oxygen pressure difference (22 versus 21 mm Hg), and physiologic dead space to tidal volume ratio (12 versus 11) at the simulated condition of 2,300 meters were similar for the SCT and control groups, respectively. It is concluded that in a moderate hypoxic environment, the cardiopulmonary and gas exchange responses of persons with SCT during brief episodes of exhaustive exercise were comparable to those of control subjects.

Adult↗
PubMedDate not supplied

Collecting duct carcinoma of the kidney associated with the sickle cell trait.

Collecting duct carcinoma of the kidney is a rare neoplasm that arises from the medullary collecting ducts. It has an aggressive clinical course and patients usually present with metastatic disease. Treatment approaches apart from surgery have been unrewarding. Renal medullary carcinoma is also an aggressive tumour which occurs predominantly in young, black, male patients with sickle cell trait. We describe a case of collecting duct carcinoma in a 61-year-old black female who exhibited the sickle cell trait. Histopathology revealed a high-grade tubular carcinoma with marked desmoplasia, adjacent dysplastic collecting ducts and positive immunohistochemical staining for high and low molecular weight cytokeratins and epithelial membrane antigen. The association of collecting duct carcinoma with sickle cell trait has not been previously reported. This case supports the postulate that collecting duct carcinoma and renal medullary carcinoma may be part of the same spectrum of renal malignancies.

Fatal Outcome↗
PubMed1996

Protracted, gross hematuria in sickle cell trait: response to multiple doses of 1-desamino-8-D-arginine vasopressin.

Gross and microscopic hematuria are well-known complications in patients with sickle cell hemoglobinopathy. Most of these episodes of gross hematuria are self limiting, but rarely may be severe and persistent requiring definitive intervention. Before subjecting these patients to surgical management such as partial or total nephrectomy, several medical therapies of variable benefit have been suggested. We report a patient with sickle cell trait who experienced severe, intractable gross hematuria for 5 months and showed a dramatic response to multiple doses of 1-desamino-8-D-arginine vasopressin (DDAVP) infusion. The remarkable response observed in this patient suggests that treatment with DDAVP infusion may be considered in patients with unremitting gross hematuria associated with sickle cell trait.

Child↗
PubMed2003

Interaction of sickle cell trait with hereditary spherocytosis: splenic infarcts and sequestration.

The association of sickle cell trait (SCT) and hereditary spherocytosis (HS) has been reported in only 18 patients. Three of these 18 patients experienced splenic infarct or acute splenic sequestration. We report here a 46-year-old African-American male, the oldest reported case to date, who experienced episodes of hemolysis and severe left upper quadrant pain for the past 26 years. The patient had compensated hemolysis with splenomegaly. A CT scan of the abdomen revealed a large infarct in the spleen. The diagnosis of SCT was confirmed with isoelectric focusing, cation exchange and reverse-phase HPLC. The presence of a silent, interacting globin variant as the cause of hemolysis and sickling in the spleen was ruled out by sequencing of the alpha1-, alpha2- and beta-globin genes. The diagnosis of HS was established by an osmotic fragility test. The interaction of HS and SCT leads to RBC dehydration with increased MCHC and intracellular Hb S concentration presumably favoring intrasplenic sickling and resultant splenic infarcts and sequestration as seen in this case.

Anemia, Sickle Cell↗
PubMed1976

Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.

A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.

Adolescent↗
PubMed1999

Splenic crisis at high altitude in 2 white men with sickle cell trait.

This article describes 2 white male patients with probable splenic crisis caused by sickle cell trait after travel from sea level to a moderately high altitude. Both did well with return to lower altitude and conservative treatment. Although rare, this entity should be considered in patients presenting to the emergency department with left-sided chest or abdominal pain after travel to moderate or high altitude.

Abdominal Pain↗