[Sexuality assessment of homozygous adult sickle cell patients with a history of priapism in Senegal]. / Évaluation de la sexualité des drépanocytaires adultes homozygotes ayant des antécédents de priapisme au Sénégal.

OBJECTIVES: The objective of this study was to evaluate the sexuality of SS sickle cell patients with a history of priapism. METHODS: This was a case-control study of adult SS sickle cell patients. The occurrence of priapism as well as the nature of the priapism had been investigated. The patients were subdivided into three groups: Group 1 (no priapism), Group 2 (intermittent priapism) and Group 3 (acute priapism). The patients' sexuality was studied using the IIEF-15 questionnaire. RESULTS: We interviewed 191 SS sickle cell patients. The mean age was 27.1±7.1 years. Priapism was observed in 43.5 %. Only 77 patients were eligible for the IIEF15 questionnaire. Groups 1 and 2 performed significantly better than group 3 on erectile function (EF) and orgasmic function (OF) scores. There was no significant difference in the EF and OF scores between groups 1 and 2. No significant difference was observed between the three groups for the scores of sexual desire (SD), intercourse satisfaction (IS), and overall satisfaction (OS). The impairment of erectile function in group 2 was related to the age of the first episode of priapism and the last episode. The impairment of erectile function in group 3 was related to the duration of evolution (P<0.05). CONCLUSION: This study shows that priapism is responsible for impaired erectile function in SS adult sickle cell patients. A program to prevent intermittent episodes of priapism should be put in place.

Diagnosis Dilemma of Angioimmunoblastic T-Cell Lymphoma in Tuberculosis Endemic Region.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare hematologic malignancy recognized in the WHO 2016 classification as a clinical and histological entity. It is a very poorly described disease in Africa due to its rarity and diagnostic difficulties, particularly differential diagnosis with tuberculosis. Here, we report a 57-year-old man who presented with fever, weight loss, and lymphadenopathies. The diagnosis of tuberculosis was carried out based on lymph node fine needle aspiration showing the image of tuberculous adenitis and CT images in favor of necrotic lymphadenopathies. The presence of autoantibodies and the failure of tuberculosis treatment led us to perform a biopsy with immunostaining that confirmed pathological features of AITL. The patient was treated by CHOP-based chemotherapy, and complete remission was achieved. This case highlights the difficulty of recognizing AITL and the importance of considering other potential differential diagnoses of tuberculosis in the endemic region.

Hématome sous-galéal révélant une hémophilie A sévère en période néonatale. A propos d'un cas

Introduction : L'hématome sous-galéal est une urgence diagnostique et thérapeutique souvent d'origine traumatique. Cependant, il peut révéler des troubles constitutionnels de l'hémostase.Observation : M.N, de sexe masculin est né par voie basse à terme, avec une notion d'expressions abdominales. Il pesait 3200 g pour un Périmètre Crânien (PC) de 33 cm et une taille de 52 cm. Il est reçu à H24 de vie pour augmentation du PC à 39 cm avec à l'examen un hématome diffus du cuir chevelu décollant les oreilles, une anémie clinique, une détresse respiratoire et neurologique. Le bilan retrouvait une anémie à 2.8g/dl et une thrombopénie. Le diagnostic d'hématome sous-galéal était retenu et il bénéficiait d'une transfusion sanguine, de vitaminothérapie K et d'une hospitalisation de 10 jours. Une semaine après l'exéat, il est revu pour des hématomes en regard des points de prélèvement avec anémie à 9.5g/dl, un taux de plaquettes normal, un TP normal et un TCA allongé. Le dosage des facteurs VIII et IX ramène un taux de facteur VIII bas à 0.9%. Le diagnostic d'hémophilie A est posé et le patient reçoit une injection intraveineuse de facteur anti hémophilique A.Conclusion : La recherche d'une anomalie de l'hémostase doit être systématique devant toute hémorragie chez le nouveau-né

Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country.

INTRODUCTION: The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. PATIENTS AND METHOD: Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. RESULTS: We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/ß0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was -60%. Neither alloimmunization nor viral seroconversion was observed. CONCLUSION: This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

Evaluation of transfusion practices in sickle cell disease in Senegal: cohort study of 1078 patients with sickle cell disease.

Blood transfusion is an essential therapeutic tool in the treatment of sickle cell disease. Its indications and modalities vary from one country to another as a function of the clinical severity of the disease and the availability of blood products. This study seeks to evaluate the frequency and modalities of blood transfusions as well as their indications in patients with sickle cell syndrome at the Dakar hematology department from 1995 through 2015. This retrospective study reviewed the records of a cohort of 1078 patients with SS, SC, Sß0 and Sß + thalassemia sickle cell syndromes and collected data about the type of blood products, indications, modalities, and acute transfusion complications. The frequency of transfusion was adjusted for age, sex, and sickle cell profile. The patients' mean age was 23 years (2-44 years) and the sex ratio 0.9. SS disease was predominant (87.4 %); 28.5% had transfusions during the study period. Patients older than 20 years received more transfusions (P = 0.047). The frequency of transfusions did not differ significantly by gender (P = 0.12), but did by profile: a higher percentage of patients with SS disease required transfusion (P = 0.043). Acute anemia was the most common indication for transfusion (50.03 %). Acute transfusion complications were rare (5.8 %). This study shows that less than one third of sickle cell anemia patients in Senegal have transfusions, a finding that confirms that our patients have fewer transfusions than patients in other countries in Africa or in the West. This limited use of transfusion therapy may be related to a less severe level of disease among patients in Senegal, but also to the availability of blood products.

[Profil course of SC sickle-cell patients in Dakar: a case-control study with SS sickle cell form]. / Profil evolutif de la drepanocytose SC A Dakar: étude cas-témoin avec la forme SS.

AIMS: The study aim to describe the epidemiological, clinico-biological and evolutionary aspects of SC sickle cell patients compared to SS sickle cell disease. PATIENTS AND METHODS: We realized a case-control study of 12 months duration including 98 major sickle cell patients (49 SC and 49 SS sickle cell patients). SS sickle cell patients were randomly selected according to age and sex. Socio-demographic, clinico-biological and evolutionary data were noted for each patient. RESULTS: Mean age was 24.7 years (5 - 53). Sex ratio was 0.8. Mean number of transfusions was 0.06 of SC patients and 0.34 for SS patients (p=0.0008). Mean number of vaso-occlusive crisis per year was 2.24 of SC patients and 2.37 of SS patients (p=0.3). Mean basic hemoglobin level was 10.8 of SC patients and 7.8 of SS patients (p=0.0000). Priapism was found in 2.04% of SC patients and 4.04% of SS patients (p=0.3) and acute anemia in 2.04% of SC and 24.48% of SS patients (p=0.003); 26.53% of SC patients had a chronic complication compared to 18.36% of SS patients (p=0.0001). CONCLUSION: This study shows that SC sickle cell patients are less symptomatology compared to SS patients, however they would develop more chronic complications from where the utility for regular follow-up.

BUTS: Le but de notre étude était de décrire les aspects épidémiologiques, clinico-biologiques et évolutifs des patients SC comparés aux patients SS. PATIENTS ET MÉTHODES: Il s'agissait d'une étude cas témoins d'une durée de 12 mois portant sur 98 patients (49 SC et 49 SS8). Les témoins SS étaient choisis de façon aléatoire après appariement selon l'âge et le sexe. Pour chaque patient nous avons noté les aspects sociodémographiques, clinicobiologiques et évolutifs. RÉSULTATS: L'âge moyen était de 24,7 ans (5 - 53). Le sex ratio était de 0,8. Le nombre moyen de transfusions était de 0,06 chez les SC et 0,34 chez les SS (p=0,0008). Le nombre moyen de CVO/an était de 2,24 chez les SC et 2,37 chez les SS (p=0,3). Le taux moyen d'hémoglobine de base était de 10,8 chez les SC et 7,8 chez les SS (p=0,0000). Le priapisme était trouvé chez 2,04% des SC et 4,04% des SS (p=0,3) et l'anémie aigue chez 2,04% des SC et 24,48% des SS (p=0,003); 26,53% des SC souffraient d'une complication chronique contre 18,36% des SS (p=0,0001). CONCLUSION: Cette étude montre que les drépanocytaires SC ont une symptomatologie moindre par rapport aux patients SS, cependant ils développeraient plus de complications chroniques d'où l'utilité d'un suivi régulier.

[Evaluation of the efficacy of medical screening of blood donors on preventing blood transfusion-transmitted infectious agents]. / Évaluation de l'efficacité de la sélection médicale des donneurs de sang dans la prévention des agents infectieux.

BACKGROUND: The aim of this study was to evaluate the efficacy of medical screening to retain blood donors in window period by comparing the seroprevalence of infectious agents (HIV, hepatitis B and C, syphilis) in deferred versus accepted blood donors. MATERIALS AND METHODS: This prospective and transversal study was performed during 4 months in the National Blood Transfusion Center in Dakar (Senegal). We conducted a convenience sampling comparing the seroprevalence of infectious agents (HIV, HBsAg, HCV and syphilis) in deferred versus accepted blood donors after medical selection. RESULTS: In total, 8219 blood donors were included. Medical selection had authorized 8048 donors (97.92%) and deferred donors were 171 (2.08%). The prevalence of HIV was higher in the deferred than in accepted blood donors (1.75% vs. 0.05%) (P=0.0003; OR=35.91), as well as for HBsAg (12.87% vs. 7.35%) (P=0.006; OR=1.86). HCV antibodies were present in 0.71% of accepted blood donors and 0.58% in deferred blood donors (P=0.65; OR=0.82). Only accepted donors had brought the infection of syphilis (0.34%) (P=0.56; OR=0). CONCLUSION: Medical selection is efficient to exclude blood donors at high risk of HIV transmission and to a lesser extent of HBV. However, current medical screening procedures do not allow us to exclude donors asymptomatic carriers of HCV and syphilis.

[Nutritional status of street children in the district of Manga (Burkina Faso)]. / État nutritionnel des enfants de la rue dans la commune de Manga (Burkina Faso).

Malnutrition is an important indicator of development, and its consequences in children and adolescents produce a serious socioeconomic burden. Children living on the street are more vulnerable than others. Thus, our objective was to analyze the nutritional status of children living on the streets of Manga, through a cross-sectional and analytical study. The snowball technique was used for sampling. Data came from individual interviews, blood samples and medical examinations. Of the 237 children studied, 84.8% were boys; the overall mean age was 11.5 years, and 72.6% were adolescents (aged 10 to 17 years). Growth retardation (15.9%) predominated among the children aged 4 to 9 years, while a weight deficit (27.9%) was most common among those aged 10 to 17. Half of the children (50.2%) with blood tests (N = 119) had anemia. There was a link between anemia and underweight (p = 0.0145). Children who ate at least three times a day were 2.63 times less likely to be anemic (p<0.001). Factors associated with anemia (p<0.005) included survival activities. We frequently found nutritional deficits and anemia in these children. A targeted nutritional program would be a good entry point for their successful reintegration..

Implementing haemophilia care in Senegal, West Africa.

Despite significant progres on haemophilia care in developed world, this disease remains unknown in many sub-Saharan African countries. The objectives of this article were to report Senegalese experience on the management of haemophilia care through 18 years of follow-up. This cohort study included 140 patients (127 haemophilia A, 13 haemophilia B), followed in Dakar's haemophilia treatment centre from 1995 to 2012. Our study reported a prevalence of 2.3/100,000 male births, accounting for 11.6% of what is expected in Senegal. From the period 1995-2003 to 2004-2012, significant progress was seen including 67.9% increase in new patient's identification, 11.3 years reduction in mean age at diagnosis (from 15.5 to 4.2 years), lower mortality rate (from 15.3% to 6.8%) and age at death evolved from 6.5 to 23.3 years. Of the 50 haemophilia A patients who were tested for inhibitor presence, 10 were positive (eight severe and two moderate) that is prevalence of 20%. All patients were low responders since inhibitor titre was between 1.5 and 3.8 BU. Disabilities were seen in 36.5% of patients above 20 years old who had musculoskeletal sequels and 39% had no scholar or professional activities in our setting. Implementing haemophilia care in sub-Saharan Africa is a great challenge as this disease is not yet counted in national health problems in many countries. Lessons learned from this study show a significant improvement in diagnosis and prognosis parameters. This emphasizes the needs to set up such follow-up initiatives and to enhance medical and lay cooperation for better results.

[Diagnostic and evolutionary aspects of hemorrhagic syndromes of the newborn in Dakar]. / Aspects diagnostique et évolutif des syndromes hémorragiques du nouveau-né à Dakar.

BACKGROUND: Hemorrhage is a common and serious symptom in the neonatal period. In developing countries, means of exploration are insufficient. Our purpose was to describe the epidemiological, diagnostic, therapeutic, and evolutionary aspects of hemorrhagic syndromes of the newborn in Dakar, Senegal, to identify the risk factors and diseases associated with them in order to propose recommendations for their management. METHODS: We conducted a case-control study of 82 newborns with 41 cases of hemorrhagic syndromes and 41 controls. We first described the epidemiological, diagnostic, therapeutic, and evolutionary parameters of the group that submitted a hemorrhagic syndrome and compared their diagnostic data with those of the control group to highlight the risk factors associated with the occurrence of hemorrhagic syndrome. RESULTS: The prevalence of hemorrhagic syndrome among hospitalized newborns was 9.2%, the sex-ratio (M/F) was 1.9. Preterm infants accounted for 26.8%. We often found early onset of bleeding (46.4% before 72h of life). Visceral bleeding was predominant, especially respiratory (34.1%), digestive (31.7%), and cerebral (17%), followed by cutaneous hemorrhages (26.8%). The risk factors identified were respiratory distress, shock, and a stained amniotic fluid. The concomitant diagnoses were dominated by neonatal infection (58.5%), hemorrhagic disease of the newborn (21.9%), and disseminated intravascular coagulation (19.5%). The mortality rate was high (34.1%), most often associated with disseminated intravascular coagulation. CONCLUSION: This study reveals the high mortality due to hemorrhagic syndromes caused by these three diseases for which prevention must be stressed.