Protection afforded by sickle-cell trait against subtertian malareal infection.
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Rouleaux formation of washed red blood cells (r.b.c.s) from subjects with sickle cell trait (HbAS) was measured by the rapid coagulation method. The r.b.c.s were suspended in the donor's native plasma or in 1.3% dextran in phosphate-buffered Ringer solution at pHs of 6.5, 7.4 and 8.2. HbAS cells showed more rouleaux formation than HbAA (normal) cells in plasma (P less than 0.005). Plasma fibrinogen concentration of HbAS and HbAA subjects did not differ significantly (mean 2.9, s.d. 0.7 g l-1 for both groups). In the dextran solution, HbAS cells were more aggregable at pH 6.5 but less at pH 8.2 than HbAA cells. There was no significant difference in dextran-induced aggregation between the two groups of cells at pH 7.4. The results suggest that HbAS subjects may be more prone to intravascular sludging and stasis and that this risk may be enhanced by reduction in pH of blood.
A prospective study was carried out to discern the outcome of pregnancy and distribution of birth weights of infants delivered of 85 women with sickle cell trait (AS) compared with a control group of 85 women with normal hemoglobin (AA) who were matched for race, age, parity, and sex of offspring. The distribution of birth weight of offspring of primiparous and multiparous women and the proportion of low-birth-weight infants did not differ significantly between infants born to mothers with AS and those in the control group. Similarly, there was no statistically significant difference between the birth weight of infants born to primipara or multipara. Also, the overall incidence of complications among women with AS did not differ from the incidence in the control group. The findings do not support previous reports that there may be definable pathologic correlates of childbearing in women with AS.
BACKGROUND: Alteration in the location and number of palmar creases has been found in association with certain disorders. The extra transverse digital crease (ETDC) has been reported in sickle cell disease. This study was carried out to determine the importance of ETDC as a diagnostic tool for sickle cell disease amongst Nigerians. METHODS: Medical students and student nurses with available hemoglobin electrophoresis records were studied. Their palms were examined for the presence of ETDC. RESULTS: An ETDC was present in 80 of 178 (44.9%) cases with genotype AA, 26 of 68 (38.2%) cases with sickle cell trait (AS, 65; AC, 3), and 10 of 22 (45.4%) cases with sickle cell disease (SS). CONCLUSIONS: The results from our study show that the ETDC is not a diagnostic sign of sickle cell disease in Nigerians.
Five episodes of transient monocular blindness during a six-month period occurred in a 42-year-old black man who had sickle cell trait. Other than the hemoglobin abnormality no cause for the transient monocular blindness was determined.
Well-documented information gleaned from the world's literature reveals that in vivo sickling of erythrocytes and vascular occlusive lesions involving the spleen have occurred in individuals with the sickle cell trait (SCT) while flying in unpressurized airplanes or when exposed to hypoxic environments in mountains at intermediate altitudes. The clinical and anatomical manifestations of splenic infarcts are described. Individuals without the trait do not develop splenic infarcts during or following exposure to ambient hypoxia.
We report the case of a patient with heterozygous sickle cell trait presenting with massive gross hematuria. Ureteroscopy revealed bleeding from the tip of papilla of the inferior calica and allowed us to tamponade the bleeding with a balloon ureteral dilator set (Cook(R) Urology). We show the value of the endourological approach in the conservative management of these patients at high risk of recurrent hematuria.
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Opinions differ widely concerning the risks assumed by individuals with sickle cell trait (SCT) while engaged in military activities that involve exposure to hypoxic environments and other stress situations. Some claim that there is no scientific evidence of greater risk to those with SCT than to those without it, and that any and all restrictions are unfounded and unjust. Others believe that the success of military missions with the least possible risk to the health and safety of individuals are major concerns and that restrictions on the selection, training, and assignment to duties are necessary. Clinical observations indicate that in vivo sickling of erythrocytes in individuals with the SCT may occur in association with cardiac, pulmonary, and other primary diseases. Past scientific investigations have been inadequate and the conclusions drawn have been conflicting. Future policies will be based on information from new investigations combined with facts revealed in the literature and from retrospective studies of the experiences of those with and without the SCT while on duty in the Armed Forces.
Bilateral, exercise-induced compartment syndrome occurred in the legs of a 19-year-old black man with sickle trait. Necrosis in the anterior and lateral compartments was so severe that subtotal debridement was ultimately required. Bilateral tibial compartment syndrome is a rare condition. Its occurrence is usually associated with underlying systemic disorders. The authors suggest that sickle cell trait played a role in this patient's dramatic course.
A study of reproductive outcome in Mobile, AL was conducted among a large maternal cohort with sickle-cell disease (Hb SS), sickle-cell trait (Hb AS), and no hemoglobinopathies (Hb AA). It was found that mean gravidity and live births among Hb AS women were significantly higher than among Hb AA women. These findings were surprising since it is generally held that once malarial pressure is alleviated, any reproductive advantage that might be conferred by Hb AS would disappear and fertility levels would reach levels similar to or slightly less than that of Hb AA women. A search of the literature was subsequently conducted and a large cohort study of an African-derived population was found in the United Kingdom. Results from this study also showed that parity was significantly higher among Hb AS women compared to Hb AA women. If survivorship is similar among Hb AS and Hb SS women, findings from these two studies raise doubts whether directional selection is occurring against the Rb S allele in nonmalarial environments. Balancing selection may still be occurring.
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