[Evaluation of sexuality among Afro-Caribbean patients homozygous SS sickle cell disease followed in Martinique]. / Évaluation de la sexualité chez les patients afro-caribéens drépanocytaires homozygotes SS suivis en Martinique.

OBJECTIVES: To evaluate and compare retrospectively the sexuality of homozygous sickle cell patients from Martinique related to priapismic events using the IIEF-15 questionnary. PATIENTS AND METHODS: Retrospective survey of 68 patients with sickle cell disease regularly followed up at sickle cell disease's center of Lamentin's hospital according to the occurrence and the type of priapism (intermittent or acute). We shared patients in three groups: no priapism (group 1), intermittent priapism (group 2) and acute priapism (group 3). Information regarding specific and preventive treatments to the disease, and events related to priapism have been gathered for all patients. Each patient's sexuality has been studied with the IIEF-15 questionnary and results have been compared between the groups to show the priapism's impact on sexual activity of these young men. RESULTS: In 37.3%, priapism was observed. The group 1 and 2 had better results than the group 3 on the EF score. There was no significative difference between the group 1 and 2. There was also no significative difference between the three groups on the SD, FO, IS and OS score. The increase of the priapism duration and the repetition of the episodes were associated to a decrease of the EF score. CONCLUSION: Our study showed the lack of major impairment of erectile function in patients with intermittent priapism in opposition to the patients with acute priapism.

[Realities for a Sickle Cell Disease Control Strategy in the World Health Organization African Region]. / Réalités pour une stratégie de lutte contre la drépanocytose dans la région africaine de l'Organisation Mondiale de la Santé: Compte rendu du 7e Symposium International du Réseau d'Étude de la Drépanocytose en Afrique Centrale (REDAC https://www.redacnetwork.org/) Antananarivo, Madagascar. 13 - 15 juin 2018.

The Network for the Study of Sickle Cell Disease in Central Africa or REDAC, is a network of African, European and American researchers whose aim is to combat sickle cell disease. Its congresses take place every year in the partner countries with international symposia alternating with workshops. REDAC enables host countries to obtain from local authorities a real involvement in the fight through resolutions in line with national strategies of fight. The Seventh International Symposium of REDAC was held in 2018 in Antananarivo, Madagascar under the auspices of the Malagasy Minister of Health and the Malagasy Senate Authorities. The theme chosen was that of strategies to combat sickle cell disease recommended by the WHO. The presentations focused on neonatal screening, early diagnosis, management of sickle cell disease and new therapies (marrow transplant, gene therapy and treatment with hydroxyurea).

Association of the +874 T/A interferon gamma polymorphism with infections in sickle cell disease.

Infectious complications are a leading cause of morbidity and mortality in patients with sickle cell disease. Several mechanisms are supposed to contribute to this susceptibility. The exact reasons for the susceptibility of sickle cell patients to infection are not clear and are still a matter of debate. Interferon gamma (IFNgamma) is a key cytokine involved mainly in the defence against intracellular pathogens. We investigated a possible association of an IFNgamma +874 T/A polymorphism and infectious complications in sickle cell patients. Seventy-two sickle cell patients were typed for +874 T/A IFNgamma polymorphism. Genotype frequencies were different between cases and controls. Indeed, the T allele frequency was significantly higher in patients with infections than in patients without infections (P = 0.014). Our results suggest that the +874 T/A IFNgamma polymorphism is associated with infectious complications in sickle cell patients. T allele could be involved in infections in sickle cell patients.

Perioperative transfusion management in patients with sickle cell anaemia undergoing a total hip arthroplasty. Is there a role of red-cell exchange transfusion? A retrospective study in the CHU of Fort-de-France Martinique.

OBJECTIVES: We conducted a retrospective study to examine the optimal regimen of transfusion and whether preoperative transfusion is needed in patients with Sickle cell anaemia (SCA) undergoing a Total hip arthroplasty (THA). Then, we assessed the incidence of perioperative complications rates among patients assigned to different transfusion regimens to propose finally the safety transfusion protocol. BACKGROUND: Preoperative transfusions are usually given to reduce or prevent perioperative complications to SCA patients undergoing THA. There is no consensus however on the best regimen of transfusion. STUDY DESIGN AND METHODS: During the period of 2000 to 2010, 14 patients with SCA (sex-ratio 0.4) with a mean age of 36 years underwent 16 THA (primary or revision). Three groups were differentiated according preoperatively protocol transfusion. Group 1: exchange transfusion (EXT), group 2: simple transfusion (ST), group 3: no transfusion (NT). RESULTS: Overall, preoperative transfusion was performed in 43.7% of cases and complications rate was 50%. In the group 1 (EXT) including five patients (31%), severe complications occurred in four patients (80%). in the group 2, including two patients (12.5%), no complications were observed. In the group 3, including nine patients (56%), complications occurred in four procedures (44.5%), the half of them were haemolytic complications. CONCLUSION: Our results support the decision to transfuse, ST, preoperatively only if the patient is significantly below their steady-state haemoglobin (Hb) level. Transfusion can be used intraoperatively according Hb level and/or the blood loss volume. Exchange transfusion appeared mostly to be related to postoperative morbidity rates.