Trophical splenomegaly and sickle-cell trait.
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Extensive evaluations for intermittent gross hematuria, including selective renal arteriograms, failed to reveal the etiology of bleeding in 2 whtie patients. Sickle cell preparation and hemoglobin electrophoresis, obtained as long shot tests, revealed sickle cell trait in both patients. Both patients were treated successfully with low doses of epsilon aminocaproic acid. Sickle cell prepration and hemoglobin electrophoresis should be included in the evaluation of every patient, white or black, with unexplained hematuria.
In a sample of 424 pregnant Zambian women a series of tests was carried out: sickle cell test, haemoglobin estimation and screening of a thick blood smear for malarial parasites. More anaemia was found in the primigravidae than in the multigravidae. The haemoglobin level was found to be lower with primigravidity and, independently of this gravidity effect, also with malaria. Taking into account the higher malaria frequency in primigravidae, this group must be considered as a high risk group for development of anaemia. There was no significant interaction between sickle cell trait, anaemia and malaria. In addition to the tests during pregnancy, the placentae and umbilical cords of 155 women were screened for malaria as were the mothers just before delivery. More malaria parasites were detected in the placentae of the primiparous than in those of multiparous women. Peripheral parasitaemia was strikingly less prevalent than infestation of the placenta.
Through PCR amplifications of the gene encoding the merozoite surface antigen 2, utilizing allele-specific 3D7 and FC27 probes, we have examined the prevalence of Plasmodium falciparum in children aged from 7 to 14 years living in a village located in the equatorial forest region of Central Africa (Gabon). Using this technique, 61% (100/163) of the blood samples were shown to be infected with P. falciparum with 24 alleles distinguished by size polymorphism and sequence type. The two main families (3D7 and FC27) and hybrid alleles were detected regardless of sex and hemoglobin phenotype. No age-related changes in prevalence of P. falciparum strains were observed; however, the prevalence of infection (42%) was significantly lower in individuals with the sickle-cell trait compared with their normal-hemoglobin counterparts (68%). Mixtures of genetically distinct parasite clones were present in 82% of children carrying the sickle-cell trait but in only 58% of normal-hemoglobin carriers. The significance of these observations regarding the design and interpretation of epidemiological investigations is discussed in the context of malaria transmission in the region studied.
Four cases of splenic infarction/sequestration in sickle cell trait (SCT) patients are presented. All four patients were undergoing moderate exercise at elevations ranging from 5,500 to 12,000 feet. The patients include two African-American males, a Hispanic male, and a white female. All four presented with the acute onset of mid epigastric then left upper-quadrant pain, nausea, vomiting, and respiratory splinting. A review of the literature indicates that splenic infarction with SCT is not uncommon; however, not surprisingly, it is often initially misdiagnosed. This is the first report in the literature of a female with SCT incurring a splenic syndrome with exposure to terrestrial altitude. Although SCT is not a contraindication for moderate- or high-altitude activities, military physicians need to consider the diagnosis of splenic infarction early in any patient regardless of race or sex who presents with left upper-quadrant pain at altitudes above 5,000 feet. Prompt evacuation to sea level may hasten recovery and spare further splenic trauma. Although SCT should be considered a relatively benign entity, the literature also suggests a higher than average risk of sudden death in military recruits with SCT from exertional heat illness and rhabdomyolysis.
The reactivity of human erythrocytes with anti-I and anti-i sera was estimated in 36 normal adults, 18 individuals with sickle cell anemia and 27 individuals with sickle cell trait, by quantitative hemagglutination with a sensitive autoanalyzer system. SS erythrocytes showed significantly increased I and i reactivity when compared to normal erythrocytes. AS erythrocytes also demonstrated some increase in Ii reactivity. No correlation was found between erythrocytic HbF content and i reactivity.
Incremental exercise tests have shown no difference in exercise capacity (EC), maximal oxygen uptake (VO2max), and ventilatory threshold (VT) in subjects with sickle cell trait (SCT) when compared to controls with normal hemoglobin (Hb). Nevertheless, during long and strenuous exercise testing, subjects with SCT exhibit comparable EC but lower oxygen uptake (VO2). Consequently, specific metabolic adaptations in subjects with SCT have been suggested during endurance exercise, consisting of a slight "handicap" in oxygen consumption and compensation by the hypothetical development of a higher anaerobic metabolism. The purpose of the present study was to research differences in the anaerobic exercise performance, and the anaerobic exercise metabolism between 9 sedentary black males with SCT and 9 controls with normal Hb. The two groups of subjects were matched for morphological and hematological variables and for aerobic fitness. The subjects were tested using the force-velocity test (FV test) characterized by the repetition of 6-s maximal exercise bouts on a cycle ergometer. The anaerobic exercise performance parameters were peak anaerobic power (PAnP), maximal velocity (V0), and maximal braking force (F0). The anaerobic exercise metabolism parameters were blood lactate concentration at PAnP and peak lactate, and blood lactate kinetics during the FV test and the recovery period. The results indicated no significant difference between the SCT and control groups for PAnP (792.3 +/- 63.3 watts vs 763.8 +/- 52.5 watts), V0 (185.5 +/- 8.9 rev.min-1 vs 173.7 +/- 5.8 rev.min-1), F0 (17.1 +/- 1.2 kg vs 17.2 +/- 1.1 kg), blood lactate concentration at PAnP (5.3 +/- 0.9 mmol.l-1 vs 4.8 +/- 0.4 mmol.l-1), at peak lactate (7.0 +/- 0.9 mmol.l-1 vs 6.7 +/- 0.7 mmol.l-1), and blood lactate kinetics during the test and the recovery period. The present study indicated that sedentary subjects with SCT and controls with normal Hb had similar anaerobic exercise performance and similar anaerobic exercise metabolism. It did not confirm the hypothetical higher anaerobic metabolism suggested in subjects with SCT.
A haematological study was done in several villages in the northern province of Cabo Delgado in Mozambique. The prevalence of sickle cell trait (HbAS) was found to be about 4%. The prevalence of G6PD deficiency in males was about 18%. No significant differences were found in haemoglobin values between HbAA and HbAS individuals and between G6PD deficient and G6PD normal individuals. The number of males with both HbAS and G6PD deficiency was not significantly greater than expected.
Several mechanisms have been proposed for explaining the protection of young children with hemoglobin AS from severe Plasmodium falciparum malaria. In a previous study carried out in Gabon, we have shown an association between hemoglobin AS carriage and a greater P. falciparum infection complexity. In the present study, we have investigated the presence and fine specificity of merozoite surface protein 2 (MSP2) reactive antibodies using different peptides covering conserved and polymorphic regions (Blocks 1-3) of P. falciparum MSP2 molecules. A cross-sectional study was conducted in the city of Bakoumba (Gabon), where malaria is hyperendemic with perennial P. falciparum transmission. Among the 641 children included, 135 were heterozygous for the sickle cell trait (HbAS). There was no significant difference in age distribution (mean age: 5 years, 0.5-11 years) and sex ratio in both hemoglobin groups (HbAA vs. HbAS). Blood group O was, however, associated with the sickle cell trait (P=0.02). P. falciparum isolates obtained from children with HbAS had a trend to higher infection complexity before the age of 5 years. Plasma samples were tested for the presence of antibodies to the different MSP2 peptides. Total IgG antibodies with a predominant reactivity against the FC27 type (the predominant P. falciparum MSP2 genotype) were found in serum samples from both groups. The profile of the IgG subclasses varied according to the hemoglobin phenotype. IgG3 and IgG2 were predominantly detected in plasma samples from HbAS children, whereas mainly IgG3 was found in children with HbAA. The role of the high multiclonal carriage associated with high family-specific antibodies reactive to MSP2 in HbAS children with asymptomatic P. falciparum parasitism is discussed.
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Whether or not whole blood lactate concentration is the same during a ramp exercise test in subjects with sickle cell trait (AS) as in normal subjects remains a point of controversy in the literature. Some studies have shown that the ability to produce or clear circulating lactate might differ between AS and subjects with normal haemoglobin (AA). If this is indeed so, the lactate distribution in the blood compartments should also differ. To test this hypothesis, lactate concentrations in the whole blood, plasma and red blood cells of AS and AA were compared at rest and in response to exercise. Eight AS and 8 AA performed an incremental exercise test. Whole blood, plasma and red blood cell lactate concentrations, the red blood cell : plasma lactate concentration ratio, the plasma-to-red blood cell lactate gradient, haematocrit and cardiorespiratory variables were analysed at rest and during an incremental exercise test and active recovery. Maximal oxygen uptake and ventilatory thresholds were similar in the two groups. No significant difference in whole blood, plasma or red blood cell lactate concentrations was observed between the two groups at rest, during exercise, or during the immediate recovery. Neither the red blood cell : plasma lactate concentration ratio nor the plasma-to-red blood cell lactate gradient differed between groups. Lactate distribution in the blood compartments did not differ between the two groups and this finding suggests that lactate production and/or clearance is quite similar during exercise in AS and AA.
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