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Sickle cell trait as a limiting factor for high-level performance in a semi-marathon.

Of 1506 black males participating in the first Abidjan semi-marathon, 123 subjects with sickle cell trait (SCT) were detected, i.e., 8.7%. Twenty-nine of these subjects with hemoglobin S (HbS) were ranked among the first 332 participants to finish the race, a percentage of 8.2. These percentages did not significantly differ from the prevalence of SCT observed in the general Ivory Coast population (12.0%). Only one subject with SCT was found among the 22 internationally-ranked athletes. The concentration of HbS found in this athlete (37.7%), his mean globular volume (87 fl), and his hemoglobin concentration (13.8 g/100 ml) suggest the coexistence of alpha-thalassemia with SCT. These results indicate that the percentage of SCT individuals participating in a semi-marathon is equal to the prevalence of SCT found in the local population. Furthermore, the general ranking of SCT individuals is comparable to that of non-SCT individuals. Nevertheless, at the level of internationally-ranked performance, no subject with SCT only, was ranked; the one ranked subject with SCT presented an associated alpha thalassemia. We thus hypothesize that SCT may be a limiting factor for high level performance in a semi-marathon and alpha-thalassemia, an enhancing factor for subjects with SCT to succeed in long distance races.

Adolescent↗

Dupuytren's disease and the sickle-cell trait in a female black patient.

Dupuytren's contracture is not often seen in the black-skinned patient. The combination of a Dupuytren's contracture in a black female patient who also carried the sickle-cell trait is described. This association of two diseases in a dark skinned patient has important surgical and anaesthetic implications which must not be forgotten in those countries with a large number of immigrants from the "sickle-cell" areas of the world.

Aged↗

Case report: postpartum hypopituitarism in a patient with sickle cell trait.

The authors report a case of hypopituitarism in a 28-year-old woman who appeared to develop this in conjunction with a pregnancy 4 years previously. The patient had clear-cut evidence of secondary hypoadrenalism and secondary hypothyroidism. A magnetic resonance imaging scan revealed a partially empty sella of normal size. The patient had no prior evidence of peripartum hypotension or other obstetrical catastrophe. Her only recognizable risk factor was the presence of sickle cell trait. The authors speculate that her sickle trait predisposed her to develop pituitary infarction during her first pregnancy. The authors also review three previous cases linking sickle cell syndromes with hypopituitarism and suggest that this case further supports the concept that sickle cell syndromes can predispose affected individuals to pituitary infarction and ultimately to subsequent hypopituitarism.

Adult↗

Distribution of IgG subclass antibodies specific for Plasmodium falciparum glutamate-rich-protein molecule in sickle cell trait children with asymptomatic infections.

Polymorphism in the beta-globin gene (hemoglobin S) has been associated with protection against severe forms of malaria. In a cross-sectional study, 180 young Gabonese children with and without sickle cell trait and harboring asymptomatic Plasmodium falciparum infections, were assessed for the responses to recombinant protein containing the conserved region of glutamate-rich protein (GLURP). We reported increased age-dependence of antibody prevalence and levels of total IgG (p<0.0001), IgG1 (p=0.009), and IgG3 (p<0.03) antibodies to GLURP with a cut-off at 5 years of age. Whatever the hemoglobin type, cytophilic antibodies (IgG1 and IgG3) were prevalent, but GLURP-specific IgG4 antibodies were detected at significantly (p<0.05) lower levels in HbAS children. We showed that the distribution of non-cytophilic IgG antibodies differs according to the hemoglobin type and to the malaria antigens tested. This may have possible implication for the clearance of malaria parasites and for protection against severe malaria.

Age Distribution↗

Blood lactate concentrations during incremental exercise in subjects with sickle cell trait.

The aim of the present study was to assess blood lactate concentrations ([LA], mmol x L(-1)) and oxygen uptake (VO2, L x min(-1), mL x kg(-1) x min(-1)) during incremental exercise in subjects with sickle cell trait (SCT) only, i.e., sedentary subjects with SCT without anemia and/or associated alpha thalassemia. Anemia was ruled out using hemoglobin (Hb) level, and alphathalassemia was ruled out using hemoglobin S (HbS) percentage and concomitant Hb level and mean corpuscular volume (MCV). Comparison was made with control subjects with normal Hb, matched for physical fitness, anthropometric data, and hematological parameters. All subjects underwent an incremental exercise test (IET) using an electromagnetic cycle ergometer. Ventilatory data, i.e., minute ventilation (VE, L x min(-1)), oxygen uptake (VO2, mL x min(-1), mL x Kg(-1) x min(-1)) carbon dioxide production (VO2, mL x min(-1)), ventilatory equivalent for O2(VE x VO2(-1))and for CO2 (VE x VO2(-1)), and respiratory exchange ratio (RER, VO2 x VO2(-1)), were collected every minute during IET and the recovery period using a breath-by-breath automated system. Heart rate (HR, beats x min(-1)) was measured every minute using an EKG. Blood sampling was done every minute during IET and the first 5 min of the recovery period, and then every 5 min until the 20th minute of recovery. [LA] were determined by an enzymatic method with a spectrophotometer. Comparisons of all mean cardioventilatory variables showed no significant differences in subjects with SCT versus controls during IET and recovery. In contrast, analysis of variance revealed significantly lower time courses of [LA] during IET (P < 0.05) and recovery (P < 0.05), whereas time courses of VO2 were similar (P > 0.05). We conclude that the lower [LA] exhibited by subjects with SCT during incremental exercise and the subsequent recovery was not associated with concomitant oxygen uptake impairment.

Adult↗

Albuminuria in Afro-Caribbeans with Type 2 diabetes mellitus: is the sickle cell trait a risk factor?

AIMS: To determine the prevalence of albuminuria [raised albumin-creatinine ratio (ACR)] in an out-patient population of Afro-Caribbeans with Type 2 diabetes mellitus (DM) and to determine if the possession of the sickle cell trait (SCT) is a risk factor. PATIENTS AND METHODS: The ACR in a morning urine sample was determined in each of 181 Afro-Caribbeans with Type 2 DM attending the out-patient clinic at King's Diabetes Centre of King's College Hospital, London. The subjects were genotyped for the SCT, Haemoglobin AS (HbAS). RESULTS: Although a raised ACR was demonstrated more frequently in those with the SCT than in those without, with an odds ratio of 1.19, this was not statistically significant (P = 0.68). CONCLUSIONS: In this study the possession of the SCT does not appear to play a significant role in the development of albuminuria. However, a larger study is needed to clarify its role as a risk factor for development of albuminuria in Afro-Caribbeans with Type 2 DM.

Adult↗