This study compared the hemorheological profile at rest and in response to a short supramaximal exercise test between sickle cell trait (SCT) carriers and a control group. Eight SCT carriers and eight control subjects performed a ramp exercise test on a cycle ergometer conducted to maximal oxygen uptake (VO2max). One week later, they performed a supramaximal exercise test consisting of pedaling for 1 min at 110% VO2max. Blood viscosity (eta(b)), plasma viscosity (eta(p)), hematocrit (Hct) and red blood cell (RBC) rigidity were assessed at rest, at the end of exercise and at the 15th, 30th and 60th min of recovery. Exercise increased eta(b), eta(p) and Hct above resting values in both groups and these parameters remained higher until the 15th or 30th min of recovery as compared to resting values. RBC rigidity was unchanged from baseline values in both groups during exercise and recovery. No difference was observed between the two groups for eta(p) and Hct but eta(b) and RBC rigidity were higher in the SCT carriers at every time point compared with the control group. The higher RBC rigidity and eta(b) found in SCT carriers at rest and in response to a brief supramaximal exercise might constitute a risk factor for microcirculatory complications. Indeed, a short supramaximal exercise test may not be completely inoffensive for SCT carriers.

The hemorheologic changes induced by exercise in the SCT carriers could trigger microcirculatory disorders during the recovery.

MRI is superior to DE for pre and post-treatment evaluation of aortic coarctation. An optimal MR protocol is proposed. Internal measurement of the narrowing does not correspond to the external aspect of the surgical narrowing.

Objectives: In Guadeloupe, an island in the French West Indies, a universal newborn screening programme for sickle cell disease and other abnormal haemoglobins was initiated in 1984. In 1990, a comprehensive sickle cell centre was established to carry on the management programme. We here report the main results from the newborn screening programme from 1984 to 2010, and consider how the establishment of the sickle cell centre affected the programme. Methods: All blood samples were screened for the haemoglobinopathies using two reference methods in a single reference diagnosis laboratory. DNA analyses were also performed for confirmatory tests and analysis of the globin gene status. Results: Between 1 January 1984 and 31 December 2010, 178,428 newborns were screened at birth, and 585 children were diagnosed with major sickle cell syndromes (ie. an overall incidence of 1 in 304 births). Sickle cell anaemia (haemoglobin SS disease) was the most frequently observed (1 in 575 births), followed by haemoglobin SC disease (1 in 771 births) and haemoglobin Sb-thalassemia disease (1 in 4,243 births). Some other rare haemoglobin variants were also detected, the most common being HbD Punjab . The establishment of a comprehensive sickle cell centre resulted in a significant improvement in the screening coverage (p < 0.001) and a significant reduction of the delay between diagnosis and the first medical visit (p < 0.001). Conclusion: The universal screening programme has made it possible to establish the incidence of the major sickle cell syndromes in Guadeloupe, and the management centre has improved its efficiency.

We have studied haplotype of ␤ S chromosome and ␣-globin gene status in 534 patients (255 adults and 279 children of whom 159 neonates) from Guadeloupe with various sickle cell-related conditions, namely SS (n = 298), SC (n = 170), S-␤-thal (n = 56), and other rare forms (n = 10). Haplotype data on ␤ S chromosomes confirm our previous observation that Benin type is the most prevalent (75%) ␤ S chromosome in Guadeloupe, in disagreement with the historical records. Comparison of the frequency of distribution of various ␤ S haplotypes between neonates and adults on the one hand and between SS and SC cases on the other shows that the current ␤ S haplotype distribution in this island is not distorted by haplotype-related differential survival. We also show that the frequency of ␣-thalassemia (−3.7 kb) in Guadeloupe is one of the highest recorded in this region involved in Atlantic slave trade and also failed to reveal any age-dependent increase in frequency. We conclude that the African component of Guadeloupe is distinct from that of Brazil and Cuba but is close to that of Jamaica. Am.