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Dactylitis commonly occurs in patients with homozygous hemoglobin S disease (sickle cell anemia), sickle cell-hemoglobin C disease or sickle cell-beta-thalassemia. A case is reported of dactylitis associated with sickle cell trait, a very rare occurrence. It may be that in this patient the disorder was secondary to severe diarrhea and dehydration.
OBJECTIVE: To report a case of fatal rhabdomyolysis secondary to exertional heat stroke and the potential influence of sickle cell trait on the outcome of this case. DESIGN: Descriptive case report. SETTING: Pediatric intensive care unit in an academic children's hospital. MAIN RESULTS: A description of the presentation and clinical course of exertional heatstroke with rhabdomyolysis in an adolescent athlete. The implications of the patient's clinical course and a possible connection to sickle cell trait are also discussed. CONCLUSION: Young athletes with sickle cell trait may be at an increased risk of heat-related illnesses and their complications. Epidemiologic studies are warranted to address this possibility.
The case of a woman with sickle cell trait who sustained a cardiac arrest and died during a Caesarian section under general anaesthesia is reported. Because the common causes of intraoperative hypoxia and shock were ruled out in this case, we believe that death was due to severe concealed aorto-caval compression. After delivery, the release of a large volume of hypoxaemic, acidotic blood with sickled cells could cause cardiac depression and arrest. The fact that the patient's mucous membranes were pink and she was haemodynamically stable while her uterus was cyanotic prior to delivery provides some positive evidence for this hypothesis. We emphasize that while complications secondary to sickle cell trait during general anaesthesia are very rare, they can occur. We discuss methods of monitoring such patients.
Children born in areas hyperendemic for Plasmodium falciparum are protected by maternal antibodies for up to about five months of life, after which they are subject to intense infection until they acquire sufficient immunity--by about five years of age. Children with sickle cell trait (Hb.AS) are at an advantage during these critical years, probably because of preferential phagocytosis of parasitized red cells. This could lead to either (i) early processing of antigen by macrophages and an accelerated immune response, or (ii) less antigenic stimulus and hence lower antibody production. Immunoglobulin (Ig)G and IgM determinations, agar gel diffusion (Ouchterlony) against soluble P. falciparum antigen, the indirect fluorescent antibody (IFA) test using P. falciparum and P. malariae antigens, and the indirect haemagglutination (IHA) test with P. falciparum antigen were performed on sera from a population with different Hb electrophoretic types in the hyperendemic malarial area of Garki, Kano State, Nigeria. Plasma immunoglobulins and antimalarial antibodies rose with age. After the first year of life, lower mean concentrations of immunoglobulins (especially IgM), and lower mean titres of antibodies specific against P. falciparum (Ouchterlony, IHA and less significantly IFA) were present in Hb.AS compared to Hb.AA; these differences increased with age. Antimalarial intervention was followed by a decline of all values and final levels showed little difference between haemoglobin types. It was unlikely that either a relative inability to produce antibody or a more rapid catabolism of immunoglobulins was responsible for the lower levels in sickle cell trait. The observations are more easily explained by the hypothesis that Hb.AS persons have less antigenic stimulus due to the early removal of parasitized sickled cells by macrophages, which then degrade the antigens. The antibody difference between Hb.AA and Hb.AS increased throughout life, suggesting that this process remained a feature of sickle cell trait even after parasite frequencies and densities were similar in the two Hb groups. These observations have implications in the aetiology of tropical splenomegaly syndrome, which is rarely seen in sickle cell trait subjects. Mean IgG and IgM were slightly higher in Hb.AS than Hb.AA infants, the difference for IgG achieving significance. This suggested that during infancy early phagocytosis of parasitized cells had led to enhanced processing of antigen and hence an earlier immune response in Hb.AS, but this was unlikely to be a major factor in survival. IFA titres against P. malariae were slightly but not significantly lower in Hb.AS, possibly as a result of cross-reaction with P. falciparum antibody or of a slight degree of protection against P. malariae.
A 56-year-old man with confirmed sickle-cell trait AS underwent successful triple-vessel myocardial revascularization with internal mammary artery and vein conduits. Variations in routine technique included limitation in hypothermia (32 degrees C), avoidance of intraoperative autologous salvage, hypoxia, severe anemia (< 25% hematocrit), acidosis and postoperative reinfusion of mediastinal drainage.
Sickle cell trait patients rarely have crises. A case of co-morbidity with acute intermittent porphyria is described in which trans-mural infarction of the distal ileum secondary to red cell sickling resulted in a fatal outcome.
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In this study, we examined the relationship of two common genetic markers in black populations, sickle cell trait and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, to cardiovascular risk factors. The subjects were Nigerian civil servants in Benin City, Nigeria. We measured blood pressure, height, weight, sickle cell hemoglobin, G-6-PD, proteinuria, microalbuminuria and fasting serum cholesterol, high-density lipoprotein cholesterol (HDL), triglycerides, apoprotein (APO) AI, and APO B. Data were collected on age, alcohol consumption, cigarette smoking, job status, and years lived in an urban area. There were 257 males (3 SS hemoglobin, 73 AS, 181 AA) and 69 females (23 AS, 46 AA). In comparing cardiovascular risk factors, males differed only in percent of smokers (31.5 in AS vs. 17.8 in AA, P less than 0.01). Among females, only high-density lipoprotein (HDL) cholesterol differed (61.5 mg/dl in AS vs. 52.4 in AA, P less than 0.01). We hypothesize that females with sickle cell trait are more likely to use oral contraceptives than nontrait females. If so, the high-estrogen oral contraceptives available in Nigeria could elevate HDL. G-6-PD deficiency status among males (52 deficient, 207 nondeficient) and females (1 deficient, 5 carriers, 65 nondeficient) was not related to any of the cardiovascular risk factors. We conclude that sickle cell hemoglobin trait and G-6-PD deficiency are not useful genetic markers for risk factors for cardiovascular disease.
Acute liver insufficiency, followed by heart disease, pneumopathy and infectious syndrome is reported in a 36 year old brown alcoholic man in which the necropsy revealed anatomopathological changes suggesting sickle cell trait. Acute ischemic lesions were noted in the heart, kidneys, central nervous system and liver. In the absence of other causes explaining the ischemic lesions, they were attributed to circulatory changes related to sickle cell trait. Hepatic failure was consequent to functional disturbances derived from the occlusion of sinusoids and necrosis of hepatocytes. The factor responsible for sickling, which caused recent ischemic lesions, may have been the respiratory insufficiency secondary to lung disease. Chronic ischemic lesions have been seen in the heart and may be related to previous sickling episodes. Subsequent to necropsy findings, screening of family members revealed a daughter with sickle cell trait.
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The prevalence and causes of anaemia among the inhabitants of a Southern Lebanese village were studied. Of the 317 patients examined 75, or 24% were found to be heterozygotes for Hb S. There was a reciprocal relation between the presence of sickle cell trait and of iron deficiency anaemia. The prevalence of iron deficiency anaemia defined as the coexistence of Hb below 13 g/dl in adult males and below 12 in adult females and children with two laboratory indicators of iron deficiency, was 10.3% in the normal population as against 1.3% in the sickle cell trait group (P less than 0.008). The reduced frequency of iron deficiency anaemia in sickle cell trait may be explained by increased iron absorption, or alternatively by reduced iron requirements and a lower risk of discrepancy between iron supply and demand. Because of the central role of iron in the function of a great number of enzymes and proteins, it is possible that protection against iron deficiency anaemia by the sickle cell trait may result in improved working capacity and a lower incidence of infections.
A von Willebrand syndrome was present in four patients with sickle cell trait and hematuria. The first two patients had severe anemia and active bleeding and received cryoprecipitate, with prompt cessation of hemorrhage. All of the patients had repetitive laboratory and clinical features; that is, reduced, but detectable, factor VIII-related antigen, heterogeneity of, and incongruities within, the coagulation studies performed by consistently defective platelet aggregation to ristocetin correctable only with normal plasma. Bleeding outside the genitourinary tract never was observed. Because of the focal nature of the hemorrhage, the hematuria may not have been perceived as part of a general hemorrhagic disorder and the diagnosis not pursued. These observations suggest that when sickle cell trait and hematuria occur together, a von Willebrand syndrome should be a major diagnostic consideration that ultimately may point toward a rational, effective, easily administered, and clinicially acceptable form of treatment with cryoprecipitate.
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The aim of this study was to establish the percentage of sickle cell trait (SCT) carriers among French West Indian sprinters selected for the French National Team in 2000. The investigation determined the number of SCT carriers and the number of national records they had established. Sixteen athletes were indexed (6 males and 10 females). The athletes were within the ranges of 20-33 years, 161-186 cm and 60-80 kg. The results showed the presence of SCT carriers in this population among whom three were SCT carriers (2 males and 1 female) (18.75%). Moreover, there is a significantly higher percentage of titles and records held by the SCT carriers (38.6% and 50%, respectively). In conclusion, this study shows that sickle cell trait carriers are able to perform sprint exercises at the highest levels, and it further indicates that brief and explosive exercise involving mainly the alactic anaerobic metabolism may be enhanced by HbS.
In order to assess the performance of subjects with sickle cell trait (SCT) during brief and explosive exercise involving mainly alactic anaerobic metabolism, the percentage of athletes with SCT was determined in Ivory Coast track and field throw and jump champions, both men and women, for the period 1956-1995. Thirty-four (27.8%) sickle cell trait carriers (SCTC) were identified among the 122 national champions that we were able to contact. These 34 SCTC had won 78 national titles (24.5%) and established 37 national records (43.5%), distributed among the throw and jump events. These percentages were significantly higher than the prevalence of SCT in the general Ivory Coast population (12.0%). The women's high jump and men's shot put events had the highest percentages of SCTC record holders (90.9% and 87.5%, respectively). Moreover, the two top national record holders and title winners, one man and one woman, were SCTC athletes, and their hemoglobin S percentage (HbS: 39.1% and 39.4%, respectively) and mean corpuscular volume (MCV) excluded an associated alpha-thalassemia. We conclude that the significantly higher percentage of SCTCs among Ivory Coast track and field champions, as compared to the percentage in the general population suggests that SCT may be a determinant factor for success in brief and explosive track and field events involving mainly alactic anaerobic metabolism.
A longitudinal study of the mental and physical development of 200 children with normal haemoglobin and 21 with the sickle-cell trait was carried out in a small rural community in Jamaica. At about 2 and 10 years of age heights and weights showed no significant differences. At about 10 years of age classroom behaviour, sociability, and educational achievement were similar. The results suggest that the sickle-cell trait does not affect growth and mental development.