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PubMed1974

The myth of sickle cell trait.

Recently emphasis in the problem of sickle hemoglobinopathy has been on mass screening of the black population. Concern about the alleged danger in having sickle cell trait itself is offered as part of the justification. This danger is disputed and a position developed for the benign status of sickle cell trait and the potentially serious social harm to blacks so identified. Programs are suggested to foster improved medical care availability and early detection for those with sickle cell anemia. It is suggested that mandatory patient programs be avoided, and that research receive greater emphasis.

Anemia, Sickle Cell↗
PubMed1979

Splenic infarction in a white man with sickle cell trait.

We report the third case of splenic infarction from sickle cell trait in a young white man traveling through mountains. Apparently the hypoxemia at 10,800 feet, along with a relatively high hemoglobin S concentration of 40%, interacted with the known conditions of splenic blood flow to cause sickling and infarction, even though our patient was not exercising. The optimal management for this rare complication of sickle cell trait remains unknown, but surgery may not be required for either diagnosis or therapy.

Adult↗
PubMed2006

Bacteriuria in pregnant women with sickle cell trait.

OBJECTIVE: The purpose of this study was to compare the following outcome variables in pregnant patients with sickle cell trait and matched pregnant control patients: asymptomatic bacteriuria, acute cystitis, urinary pathogens that were present, and pyelonephritis. STUDY DESIGN: This was a retrospective cohort study that was conducted at a university clinic. Pregnant patients with sickle cell trait (n = 455) were matched with control patients (n = 448) for race, age, gestational age at entry into prenatal care, and number of prenatal visits. RESULTS: Women with sickle cell trait received urine testing significantly more often. There was no difference in the incidence of positive urine cultures, urinary pathogens, or asymptomatic bacteriuria among the comparison groups. Sickle cell trait carriers had significantly higher rates of pyelonephritis, but many affected patients had risk factors, such as previous pyelonephritis or noncompliance with therapy. CONCLUSION: Sickle cell trait carriers were no more susceptible to acute cystitis and asymptomatic bacteriuria than were the control patients. On the basis of these data, we outline recommendations for urinary screening and pyelonephritis prevention in pregnant patients with sickle cell trait.

Acute Disease↗
PubMed2006

Pregnancy loss after first-trimester viability in women with sickle cell trait: time for a reappraisal?

OBJECTIVE: The purpose of this study was to evaluate the obstetric outcomes and pathologic findings in women with sickle cell trait. STUDY DESIGN: In this retrospective case control study, pregnant women with sickle cell trait were studied over a 4-year period (2001-2005). The women who were delivered at > 16 weeks of gestation were compared with a cohort group of subjects with normal hemoglobin levels, and the placentas were sent for pathologic evaluation. RESULTS: A total of 180 pregnancies were studied with a like number of control patients. Subjects who had sickle cell trait demonstrated shorter average duration of pregnancy (233 +/- 45 days vs 255 +/- 34 days; P < .001) and lower birth weight (2114 +/- 1093 g vs 2672 +/- 942 g; P < .001). The rate of fetal death was significantly higher among study group patients (3.5% vs 9.7%; P = .015) when compared with the control group. Additionally, in study women, acute ascending amniotic infection and meconium histiocytosis were noted much more frequently. Sickling in the intervillous space and decidual vessels that were not associated with artifactual change was also found among patients sickle cell trait. CONCLUSION: Patients with sickle cell trait appear to be at increased risk for fetal loss compared with women with normal hemoglobin levels, and placental abnormalities may play a causal role.

Abortion, Spontaneous↗
PubMed2003

Pregnancy outcomes among Palestinian refugee women with sickle cell trait in Damascus, Syria.

OBJECTIVE: The results on pregnancy outcomes of mothers afflicted with sickle cell trait are still contradictory. This study aimed to examine the fetal and maternal outcomes among a cohort of pregnant women. METHODS: This is a prospective cohort study that examined the fetal and maternal outcomes among 98 women with sickle cell trait (HbAS) and 402 women with normal hemoglobin (HbAA). The study was carried out in 4 health centers serving Palestinian Refugees in Damascus, during the period November 2000 to May 2002. Hemoglobin electrophoresis was carried out for all newly registered pregnancy women. Women were then followed up until 40 days after delivery. Data was collected from antenatal records and interviews with women. RESULTS: Outcomes of pregnancy were compared between women with HbAS and HbAA hemoglobin. There was no statistical difference in the rate of abortion, distribution of birth weight and perinatal mortality. Women with AS hemoglobin reported higher incidence of complications after delivery, namely, fever (risks ratio=4.05, 95% confidence interval=1.34-12.3). CONCLUSION: In this study, pregnancies among women with sickle cell trait demonstrated high risk of complications after delivery. Watchful follow up of pregnancies among women with sickle cell trait is very necessary. Doctors and women must know that although the course of pregnancy among women with HbAS can be benign; it may well carry a high risk on women.

Arabs↗
PubMed2004

Acute chest syndrome in sickle-cell trait; Two case reports in persons of Mediterranean origin and review of the literature.

Sickle-cell trait has been characterized as a benign condition. However, life-threatening complications sometimes develop. Acute chest syndrome (ACS) is usually described in homozygous sickle cell disease, but it rarely develops in individuals with sickle-cell trait. Two cases of ACS in patients with sickle-cell trait are reported here. Factors such as advanced age at the time of presentation and absent past medical and family history can be misleading. Although ACS in sickle-cell trait has thus far only been reported in persons of Afro-American origin, persons of Mediterranean origin can, on rare occasions, also experience the syndrome.

Journal Article↗
PubMed2003

Pregnant women with the sickle cell trait are not at increased risk for developing preeclampsia.

The primary objective of this study was to determine whether having the sickle cell trait is independently associated with preeclampsia. We performed a retrospective cohort study of 1998 pregnant patients who either did or did not have the sickle cell trait. All patients were screened for the sickle trait using the "Sickledex" test. Data on neonatal and maternal outcome, including preeclampsia, and potential confounding variables were abstracted from medical records. Unadjusted, stratified, and multiple logistic regression analyses were used to identify interactions, and confounding between multiple variables and the association between sickle cell trait and preeclampsia. With an anticipated 6.5% rate of preeclampsia, and alpha = 0.05, this cohort study has 80% power to detect a relative risk (RR) of 2.3 for preeclampsia. Univariate analysis revealed that the two cohorts were similar with regard to primiparity, maternal age, chronic diseases, birth weight, and gestational age at delivery, but the sickle cell trait cohort was more likely to have gestational diabetes and had a higher mean body mass index (BMI). In the univariate analysis, the sickle cell trait cohort was not at increased risk for preeclampsia [unadjusted RR = 0.5, 95% CI (0.2-1.6)]. After controlling for potential confounding variables with logistic regression analysis, sickle trait was not independently associated with preeclampsia [adjusted RR = 0.5, 95% CI (0.2- 1.6)]. In contrast to prior work, these data suggest that the sickle cell trait is not an independent risk factor for preeclampsia or postpartum complications. In fact, the data are more consistent with the sickle trait being protective for developing preeclampsia.

Adult↗
PubMed1997

Hemoglobin A2 levels in healthy persons, sickle cell disease, sickle cell trait, and beta-thalassemia by capillary isoelectric focusing.

Simultaneously measuring major and minor hemoglobin (Hb) variants by capillary isoelective focusing, we obtained HbA2 intervals in healthy volunteers (n = 412) (reference value) and patients with HbS or beta-thalassemia. We classified normal HbA2 reference intervals into three age groups: 5 months or younger (1.2% +/- 1.5%), 6 months to 1 year (2.2% +/- 0.9%), and 1 year or older (2.4% +/- 0.9%). These intervals were comparable to those used with other methods. Patients 1 year of age or older with HbS had significantly higher HbA2 levels (sickle cell trait, 2.9% +/- 0.9%; sickle cell anemia, 2.8% +/- 1.0%; P < .05). Although reference HbA2 intervals overlapped those in patients with HbS, no overlap in HbA2 levels was noted between these groups and patients with beta-thalassemia (observed range, 4.3% to 7.5%). The higher than normal HbA2 interval in patients with HbS must be considered before a diagnosis of sickle cell trait or sickle cell disease with beta-thalassemia is made.

Anemia, Sickle Cell↗
PubMed2001

Death due to microvascular occlusion in sickle-cell trait following physical exertion.

The heterozygous condition characterized by the presence of hemoglobin AS (sickle-cell trait) occurs in approximately 8% of the American black population. Unlike the homozygous state (sickle-cell disease), sickle-cell trait is not widely recognized as a cause of life-threatening illness or death despite over 30 case reports describing fatal or serious complications of exercise in young black males with this condition. These reports identify heat stress, dehydration, viral illness, and poor physical conditioning as factors which may contribute to exertional rhabdomyolysis and sudden death, suggesting multifactorial etiology. However, since sickling is known to occur postmortem, it remains controversial as to whether the pathogenesis of these exercise related deaths involves microvascular obstruction by sickled erythrocytes. We describe three young black individuals with no significant past medical history who died following physical exertion. In all three cases, postmortem hemoglobin electrophoresis demonstrated hemoglobin AS. In none of the cases was the body temperature found to be elevated. These cases serve to remind the forensic community that, in the proper setting, sickle-cell trait must be viewed as a potentially fatal disorder.

Adult↗
PubMed1984

A family with sickle cell trait and hereditary spherocytosis.

The rare combination of sickle cell trait with hereditary spherocytosis in a Sudanese family is reported. The splenic hypofunction and fibrosis as a result of sickling has ameliorated the clinical course of the 35 year old father. The symptoms of a six years old son seem to be getting milder with age too. The family is managed conservatively and splenectomy was not required.

Adult↗
PubMed1982

Vocational options for those with sickle cell trait: questions about hypoxemia and the industrial environment.

Many patients have parents and siblings who possess the sickle cell trait and who often require not only genetic counseling, but also information about their own health. Some have been informed that they cannot pursue careers in aviation. Some have been told that they are at special risk for the hypoxemic effects of heavy exertion, especially the exertion associated with sports. A few believe that certain elements of the industrial environment are unusually hazardous to them. An examination of the literature reveals little evidence in support of any of these beliefs. Most, if not all, unfavorable reports are clouded by faults of various kinds. All of them are of anecdotal type, based on small numbers of cases; the evidence offered is circumstantial. On the other hand, experiments designed to test the susceptibility of those with sickle cell trait have yielded favorable results. Studies of large populations indicate that those with the trait have normal health and normal life expectancy. There are almost as many favorable anecdotal reports as there are negative ones. We must conclude that people with sickle cell trait are just as tolerant to aviation and industrial environments as those who are homozygous for hemoglobin A. Also, there are strong indications that those with sickle cell trait are not endangered by heavy physical exertion, including the exertion of athletic competition.

Anemia, Sickle Cell↗
PubMedDate not supplied

[One hundred and twenty-three cases of sickle-cell trait (author's transl)].

One hundred and twenty-three cases of sickle-cell trait (122 negro immigrants) are studied in a department of internal medicine. The frequencies of G6PD deficiency, anemia, splenomegaly and tuberculosis are neighbouring at the negro without hemoglobinopathy. The relationship between the sickle-cell trait and the reason of admission of the final diagnosis il likely in 2,4 % of the cases, doubtfully in 15,4 %, null in the other cases. The occasional (hemolytic, thrombotic, painful, visceral, osseous) manifestations of the sickle-cell trait (apart from the constant hyposthenuria) and their mechanism are summarized and discussed.

Africa↗
PubMed1996

Rhabdomyolysis and hemolysis associated with sickle cell trait and glucose-6-phosphate dehydrogenase deficiency.

We report a case of severe oxidative hemolysis and rhabdomyolysis in a patient with sickle cell trait and glucose-6-phosphate dehydrogenase (G6PD) deficiency. The patient was a 34-year-old black man admitted 24 hours after vigorous exercise with myalgias, malaise, myoglobinuria, anemia, low haptoglobin, and a peripheral blood smear with bite cells consistent with oxidative hemolysis. He had two similar episodes within 21 months of the initial admission. Subsequent evaluation resulted in the diagnosis of sickle cell trait and G6PD deficiency; muscle enzyme levels were normal. G6PD deficiency and sickle cell trait can be expected to occur simultaneously in up to 1% of black males. A second red blood cell defect should be considered when severe hemolysis is seen in a person with sickle cell trait.

Adult↗
PubMed2005

Plasma lipids in Iranians with sickle cell disease: hypocholesterolemia in sickle cell anemia and increase of HDL-cholesterol in sickle cell trait.

BACKGROUND: The metabolism of lipids may be altered in sickle cell anemia and beta-thalassemia patients. METHODS: Plasma lipids in 24 patients with sickle cell anemia (SS), 15 patients with sickle/beta-thalassemia (ST), 19 individuals with sickle cell trait (AS) and 62 healthy individuals (AA) were measured. RESULT: Total cholesterol concentrations in both sexes with sickle cell anemia (SS males: 104.7, SS females: 142.7 mg/dl) and sickle/beta-thalassemia (ST males: 116.7, ST females: 137.5 mg/dl) were lower (P<0.05) than AS (AS males: 171.5, AS females: 185.4 mg/dl) and normal individuals (AA males: 173.7, AA females: 183.0 mg/dl). The mean HDL-cholesterol in males with SS (35.6 mg/dl) and ST (40.7 mg/dl) were (P=0.001) lower than AS males (58.9 mg/dl). However, the mean HDL-cholesterol in females with SS (43.2 mg/dl) was lower (P<0.001) than AS females (62.7 mg/dl). The mean LDL-cholesterol of males with SS (53.8 mg/dl) was lower (P<0.01) than AS (89.4 mg/dl) and control males (115.9 mg/dl). Males with ST had a lower (P<0.001) LDL-cholesterol (58.0 mg/dl) compared to control males. In females with SS the LDL-cholesterol (78.3 mg/dl) was lower (P<0.001) than control females (124.3 mg/dl). However, females with ST had lower (P<0.05) LDL-cholesterol (61.6 mg/dl) than AS (101.7 mg/dl) and control females. There was no significant difference in total concentrations of cholesterol and triglycerides between males and females with AS and those with normal hemoglobin. However, the HDL-cholesterol in both genders with AS was higher (P<0.001) than normal subjects. Also, the concentration of LDL-cholesterol in both males and females with AS was lower than control males (P<0.05) and females. CONCLUSIONS: Hemolytic stress could be associated with a significant reduction in plasma lipids and lipoproteins. It appears that patients with sickle cell anemia and individuals with sickle cell trait are at a lower risk for coronary artery disease.

Adolescent↗
PubMed1987

Age and prevalence of sickle-cell trait in a large ambulatory population.

We performed hemoglobin electrophoresis in 30,400 apparently healthy black individuals in the Washington D.C. Metropolitan area, who were participating in a community based sickle-cell screening program during the period 1978-1985. The overall prevalence of sickle-cell trait was 6.7%. The frequency of sickle-cell trait in various age groups, which included young children, adults, and individuals over 65 years of age, ranged from 6.4 to 7.4%. There were no statistically significant differences in the prevalence of sickle-cell trait among the various age groups, nor did we detect a significant trend for increasing or decreasing sickle cell frequency with advancing age (P = 0.418). Thus, in this population sickle-cell trait appears to have no effect on longevity. If the various complications of sickle-cell trait reported in the literature are not fortuitous, their frequency and/or severity must be too low to affect prevalence figures.

Adolescent↗
PubMed1991

Prevalence of the sickle cell trait in an athletic West African population.

The prevalence of the sickle cell trait in a school for elite young athletes in Cameroon, West Africa, was examined in order to evaluate whether the choice of an athletic career was influenced by its presence. The presence of hemoglobin AS was found in 27 of the 145 students attending the school (18.6%). Since the frequency of the sickle cell trait in the general Cameroonian population is 17.3%, we conclude that its presence is not a determinant factor involved in the choice of a professional sportive activity.

Cameroon↗
PubMed1989

Evolution of acute chest syndrome in sickle cell trait: an ultrastructural and light microscopic study.

Light and electron microscopic studies of a patient with sickle cell trait who had an episode of sickling during coronary artery surgery, from which he died, showed fibrin thrombi, focal alveolar wall necrosis, and epithelial cell damage. It is suggested that in cases of sickle trait full precautionary measures should be taken to prevent sickling in these circumstances.

Acute Disease↗
PubMed1989

[2 cases of acute sickle cell crisis in subjects with sickle cell trait following high altitude exposure].

We present two patients with sickle cell trait (HbAS) who developed acute sickle cell crisis after passive exposure to high altitude on the Jungfraujoch (3454 meters, 11,333 ft). Both suffered splenic infarction and the first patient also presented with acute renal failure. Splenic infarction is known to be a complication of altitude exposure in carriers of the trait, whereas acute renal failure due to altitude exposure alone has as far as we know never been described in the literature. These forms of high altitude complications are totally unusual in Switzerland.

Adult↗