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.

[Predictive factors of chronic complications in adult sickle cell anemia patients in Dakar, Senegal]. / Facteurs prédictifs des complications chroniques de la drépanocytose homozygote chez l'adulte à Dakar (Sénégal).

PURPOSE: Chronic complications of sickle cell disease (SS) usually involve irreversible organ damage. Several genetic factors have been shown to have predicative value for chronic complications but these data are not always available. The purpose of this study was to assess the value of sociodemographic and clinicobiological features in predicting chronic complications. METHODS: This study included a total of 229 adult SS patients who underwent quarterly follow-up examinations for at least 10 years (range, 10 - 16). All sociodemographic and clinicobiological data were recorded. Screening for complications was performed at least once every three years. The risk of developing chronic complications was analyzed in function of patient follow-up data. RESULTS: Mean patient age was 28.6 years (range, 20 - 57) and sex ratio was 1.3. Prevalence of chronic complications was 34.9% (80/229). The most common complication was bone necrosis in 27 cases (11.7%) followed by gallstones in 24 (10.4%). The only sociodemographic factor with predictive value was patient age (p=0.0008). Multivariate analysis identified two clinicobiological factors with predictive value. History of transfusion was associated with a 3-fold higher risk while hemoglobin F level was associated with decreased risk. CONCLUSION: In this study, age and low hemoglobin F level were the only predictive factors of chronic complications in SS patients.

[Residual risk of transmission of HIV and HBV, in Senegalese national blood bank from 2003 to 2005]. / Risque résiduel de transmission du VIH et du VHB par transfusion sanguine entre 2003 et 2005 au Centre national de transfusion sanguine de Dakar (Sénégal).

BACKGROUND AND AIM: Using of safety blood products did not stop improving these last years. The use of effective methods as well immunologicals as virologicals ones really reduces risk associated to blood transfusion. However, it persists residual risk (RR) of transfusion transmitted viral diseases. The aim of our study was to detect cases of seroconversion for HIV,and HBV among donors in the Senegalese national blood bank. And then, we estimated the RR of these virus. METHODS: We led a transverse retrospective study from 2003 (January 1st) to 2005 (December 31st). Had been included donors with at least two donations of blood during the period of study. They had to be seronegative for HIV and HBV after the first donation. All donors with only one donation had been excluded. RR was estimated by multiplying incidence rates by the durations of the window periods. RESULTS: During 3 years, we collected 425,503 donations; 388 were positive for HIV and 4240 for HBV. But we noted only two cases of seroconversion for HIV and 23 for HBV. So, RR estimated was 3,5 in 100,000 donation for HIV and 102,45 in 100,000 donations for VHB. CONCLUSION: It emerges from this study that the risk of blood transmitted virus is always high. Introduction of more sensitive tests (as nucleic acid testing) would allow us to deliver more safety products.

[Von Willebrand disease and menorrhagia]. / Maladie de Willebrand et ménorragie.

INTRODUCTION: Known since over than seventy years, von willebrand disaese is the most common herediary bleeding disorder. This condition was first described by Pr. Willebrand in 1926 in a family with (positive) history of excesive bleeding tendency. Von Willebrand desease is characterized by a lifelong tendency toward easy spontaneous mucosal or post operative bleeding. In females, excessive or prolonged menorrhagia could be a sign of von willebrand desease; symptoms that are often misunderstood to be gynecologic rather than hematologic problem. In the present work, we have tried to screen for this anomaly in females with menorrhagia, following a simple anamnestic, clinical and biological protocol. PATIENTS AND METHOD: In a seventeen month study, fifty two procreating females with menorrhagia were recruited in the haematology laboratory of Aristide le Dantec hospital with the cooperation of gynecology and obstetric departements of Aristide Le Dantec, Abass Ndao and grand yoff Hospitals. RESULTS: Eight patients were revealed to be von willebrand positive (prevalence: 15%). The diagnosis was retained on the basis of epidemiological, clinical and biological data. CONCLUSION: These simple and accessible criteria should allow better handling of patients with hemorragic disorders.

[Prevalence of antiphospholipid antibodies in patients with sickle cell disease]. / Prévalence des anticorps antiphospholipides au cours de la drépanocytose homozygote.

Structural abnormalities in erythrocyte membrane are more and more claimed as a determinant factor in the sickle cell disease pathogenesis. This being would have been provided by a new anionic phospholipids distribution and conformation. Phosphatidyl-serine exposing and phosphatidic acid enhancing would induce specific immunoglobulins synthesis. In this study, assessment of antiphospholipid antibodies prevalence was carried out among sickle cell trait patients (n = 35) and homozygous patients (n = 59) as compared to healthy subjects (n = 39). Antiphospholipid antibodies, assayed by ELISA procedure, were significantly higher among the homozygous patients than the sickle cell trait patients ones and highlighted as compared to healthy subjects. Pathologic data were only observed among homozygous patients. These specific antibodies, associated with thrombosis and haemolysis, would have constitute a morbid link and a therapeutic target of this sickness, dominated by homodynamic troubles.

[Portal and splenic veins thrombosis reveling a miliary tuberculosis of the liver]. / Une thrombose portale et splénique révelant une tuberculose miliaire du foie.

The etiologies of the portal vein trombosis are dominated by the neoplasic forms with hepatocellular carcinoma; we report a rare case of portal and splenic veins thrombosis revealing a liver military tuberculosis occuring in a HIV 1 infected patient. A 42 years old senegalese woman with no personal or family history of thrombosis was admitted for abdominal upper right quadran, and epigastric pain, with fever and important weight loss. Ultrasound identified endoluminal echogenic images in the portal and splenic veins. There were no lymph nodes or liver tumor. Evaluations of proteins C and S were normal and there was no anticardiolipin antibody. In searching the aetiology of the thrombosis, a liver biopsy was performed, and showed a miliary tuberculosis. an HIV 1 infection was later on diagnosed. The antituberculosis treatment associated with heparine therapy was successful, the thrombosis resolved entirely. This portal and splenic veins thromboses occuring on a miliary tuberculosis of the liver seems to be an exceptional situation. We did not found in the literature a similar case.lt points out the interset of liver biopsy in searching the aetiology and the early heparine therapy set up

[Maternal anaemia: effect on the newborn]. / Anémie par carence en fer maternelle: retentissement sur le nouveau-né.

Pregnancy increases considerably iron needs in mother and her foetus. The purpose of our study is to measure the effect of maternal anaemia on the foetus and the effect of iron supplementation on the maternal and foetal reserves. Therefore, we conducted a three-month cross sectional study at the gynaecological and obstetrics clinics of Aristide Le Dantec Hospital. Ninety-five women aged 16 to 43 years old and having an haemoglobin rate < 11 g/dl were recruited. Most of them were primipares. Among them 69 had a low ferritinemia (< 50 ng/ml), 36, a ferritinemia collapsed (< 30 ng/ml) and 13 virtually non-existent reserves (< 12 ng/ml). All newborns were born in terms with an apgar score >/= in 93 of them. Among them 24 had anemia (rate of haemoglobin < 14 g/dl) and 54.7% a low ferritinemia. There is no relationship between the maternal and foetal rates of haemoglobin; 74% of newborn had a normal rate of haemoglobin. Among 36 women with low ferritinemia only two gave birth to a newborn without iron reserves. In our study, among 68 women who received iron regularly, 41 had normal reserves and 43 gave birth to a newborn with high ferritinemia. There is significant difference between the women having received iron during their pregnancy and those not supplemented as regards the effect on newborn (p = 0.00001). The prevention of iron deficiency and anaemia can be done by the iron systematic and premat supplementation.

[Bone marrow aspiration in diagnosis of hemopathies in Dakar, Senegal]. / Intérêt du myélogramme dans le diagnostic des hémopathies au CHU de Dakar: a propos de 1000 ponctions réalisées de 1991 a 1999.

The cytological study of bone marrow aspirate, is a useful technique in diagnosis and epidemiological surveillance of hemopathies. Between January 1991 and February 1999, we realized 1000 bone marrow puncture in Dakar hospitals, this study aims to analyze the frequency of diagnosed hemopathies, and to appreciate justification of prescription. Mean age was 31.6 (1 to 88 years). Bone marrow puncture was safe because no incident was observed. Interpretation was possible in 937 cases while the 33 authors was diluted by blood. Bone marrow was normal in 550 cases (57.3%) whereas abnormality was detected in 417 cases (42.7%). The more frequent pathology were quantitative or qualitative defect of bone marrow production with respectively 25.8% and 20.1%, followed by chronic myeloid leukemia (16%), lymphoproliferative disorders (16%), acute leukemias (13.9%), bone marrow metastasis (5.9%), and storage diseases (1.9%). The best justification of the prescription was obtained when done on the basis of hemogram abnormalities or signs in hematopoietic organs with a pathology found in 90% of cases. Prescription on the basis of non hematological signs or to search for metastasis when primitive tumor was not identified were less efficient with respectively 20% and 42% of normal bone marrow. These results emphasize the interest of bone marrow aspiration in epidemiological surveillance of hemopathies, and the advantage to respect the rights indications, in consideration of pain and intolerability of the puncture by patients.

[Homozygous sickle cell disease in patients above 20 years of age: follow-up of 108 patients in Dakar]. / La drépanocytose homozygote après l'âge de 20 ans : suivi d'une cohorte de 108 patients au CHU de Dakar.

CONTEXT: The progress realized in the treatment of sickle cell anemia has resulted to a better and longer life for these patients in developed countries. Ongoing challenge are to manage complication and social living for these patients. The purpose of this study was to determine morbidity, mortality and socioprofessional insertion of homozygous sickle cell patients, followed up in Dakar university hospital. PATIENTS AND METHODS: We performed a longitudinal and prospective study including 108 homozygous sickle cell anemia patients, whose age were above 20 years, followed in a regular basis (at least 3 times per year), during 5 years on average (3 to 12 years). All patients had a quarterly hematological check-up and a annual statement to detect chronic complications. Clinic and paraclinical data, as information in socioprofessional insertion were noted in medical records for analysis. RESULTS: Mean age was 27 years (20-51 years). The age between 20-29 years was represented by 67.5%, 26.9% aged 30-39 years and 5.6% were above 40 years of age. Men were slightly predominant with a sex-ratio of 1.25. Concerning morbidity, 96.3% of patients had at least 1 vaso-occlusive crisis per year, 26.9% were transfused and 64.8% had been hospitalized during the follow-up. A chronic complication was found in 49% of patients (53/108). The more frequent of these complications were gall stone (10%), femoral head necrosis (9.2%), priapism (11.6% of men), chronic leg ulceration (4.6%), ophthalmic involvement (3.7%), renal and cardiac complications (2.7% for each one). The presence of complications was not significantly influenced by patient's sex. Five patients died during a mean follow-up of 5 years. Concerning school attendance, 13% reach university level whereas 47% stop their education on the secondary level and 40% on primary level. Professionally, 36.2% of patients had no exercise any activity. CONCLUSION: Our results emphasize that life expectancy can be prolonged in sickle cell anemia patients in Africa, when they have a benefit of a regular follow-up. We show also the respective frequency of chronic complications and then, the necessity of multidisciplinary teams to optimize the take care of sickle cell anemia patients in Africa.

[Epidemiologic aspects and medical-social impact of hemophilia at the University Hospital Center in Dakar]. / Aspects épidémiologiques et impact médico-social de l'hémophilie au CHU de Dakar.

The epidemiology and medico-social impact of hemophilia is poorly understood in Africa due to low incidence of the disease and lack of trained hematologists and adequate laboratory facilities. The purpose of this report is to describe our experience with the organization of regular follow-up for hemophilia patients at the University Hospital Center in Dakar, Senegal. A total of 54 patients with type A hemophilia were included in this three-year study. Moderate forms of hemophilia were the most common (55.6%) followed by severe (29.6%) and minor (14.8%) forms. There was no significant difference in mean patient age according to disease severity, i.e., 19.3 years for patients with severe forms, 13.4 years for patients with moderate forms and 15 years for patients with minor forms. Patients over 20 years of age accounted for 27.9% of the study population. The remaining patients were younger, i.e., between 10-19 years (33.3%) and between 1 and 9 years (38.8%). Eighty-seven percent of patients lived in Dakar and the remaining 13% were from various regions of the country. Among the 54 patients in this study, there were 38 (70.3%) with no hemophiliac brother in the family, 13 (24.1%) with one hemophiliac brother and three (5.6%) with two hemophiliac brothers. Osteoarticular damage was found in 28 patients (53.8%) involving the knee in 22, elbow in 18 and ankle in 9. Four patients were positive for HBs Ag (7.4%) and one patient for HIV. Professional or scholastic activity was possible in 69.2% of patients under 20 years old and 46.6% over 20 years old. Although results are still inadequate, a clear-cut improvement has been noted in the survival and quality of life of hemophiliacs in comparison with previous years. These findings show the value of regular surveillance for hemophilia patients in countries with limited resources.

[Pattern of type A hemophilia in Senegal: prospective study in 54 patients]. / Profil évolutif de l'hémophilie A au Sénégal: étude prospective réalisée chez 54 patients.

Little is known about hemophilia in the developing countries because of the difficulties in the diagnosis and the therapeutical management of this disease. Here we present the results of the follow-up of 54 patients in Senegal. Diagnosis was always confirmed by measuring the biological activity of factors VIII and IX. Patients were treated at home or in the hematology service according to the gravity of hemorrhage events. The severe form represented 29.6%, moderate form was 55.6% and minor form 14.8%. Total number of hemorrhage events was 1078 per year: 449 hemarthrosis (41.7%), 373 exteriorized hemorrhage (34.7%) and 256 hematomas (23.7%). Mean frequency of hemarthrosis per patient per year was 12 in severe form, 8 in moderate form and 3 in minor form. Mean frequency of hematomas per patient per year was 5.2 in severe, 4.9 in moderate and 4.2 in minor form. For exteriorized hemorrhage, the mean frequency was 7.06 in severe, 7.4 in moderate and 6.5 in minor form. The severity of hemophilia significantly influenced the frequency of hemarthrosis (P = 0.02) but not the frequency of hematoma (P = 0.6) and exteriorized hemorrhage (P = 0.6). Treatment of these accidents was performed at home (88.5%), in day hospital (9.5%) or needed hospitalization (1.8%). Three patients have died during this three-year survey, one because of HIV infection and the two others from digestive hemorrhage. In conclusion, lesser morbidity and mortality were observed when compared with previously. The importance of a regular follow up and education of patients must be emphasized especially if factor concentrates are not available.

[Evaluation of thrombotic risk in type 2 diabetes: determination of platelet factor 4 and beta thromboglobulin]. / Evaluation du risque thrombotique dans le diabète de type 2: dosage du facteur 4 plaquettaire et de la bêta thromboglobuline.

We achieved the dosage of platelet factor 4 (PF 4) and beta thromboglobulin (B-TG), two proteins stored in platelet alpha granules and released from the platelets when activated. These proteins were studied by ELISA technics in patients with type 2 diabetes without any complications (n = 32), with thrombotic complication (n = 32) and in a healthy control group (n = 32). The diabetic patients with complications had significantly elevated plasma levels of PF4 and B-TG compared with diabetic patients without complications, whereas levels of these proteins where normal in control group. These high rates of PF4 and B-TG were significantly related to a disturbance of lipidic balance. Our data suggest that platelet hyperactivation may be a reliable element in the pathogenesis of thrombotic complications in type 2 diabetes.

[Fibrinolytic syndrome during surgery for benign prostatic hypertrophy (50 cases in the urology service at the Aristide le Dantec University Hospital Center)]. / Syndrome de défibrination dans la chirurgie de l'hypertrophie bénigne de la prostate (à propos de 50 cas colligés dans le service d'Urologie du CHU Aristide le Dantec).

Prostate's surgery is an haemorrhagic one owing to difficulties of surgical haemostasis and anomalies of the blood coagulation. The aim of this study to assess haemostasis anomalies after protatectomy for benign prostatic hyperplasia (BPH). We have performed a prospective study including 50 men of more of 55 years old. All of them underwent suprapubic open prostatectomy. Significant pertubation of haemostasis, particularly fibrinolysis has been found. We have noticed fibrin degradation product in 88% of cases after intervention versus 62% before (p < 0.001) and a decrease of plasminogen rate in 94.4% of operated patients versus 82% before. The older the patient were, more important were the pertubations. Moreover, the anomalies were most important when the duration of surgery lasted more than one hour and when the BPH was voluminous. However, the disturbance of haemostasis was rarely associated to clinical symptoms. So, we do not recommend any therapeutic when clinical symptoms are absent. Biological screening the day of surgery, the day after and one week later are necessary to prevent haemorrhage.

[Antiphospholipid antibodies and recurrent spontaneous abortions at the Aristide Le Dantec University Hospital Center]. / Anticorps antiphospholipides et avortements spontanés répétés au CHU Aristide Le Dantec.

Antiphospholipids including lupus anticoagulants (LA) and anticardiolipins (ACL) are known to be able to lead to many obstetricals complications like gravidic chorea, eclampsia and repeated spontaneous abortion. Our objective was to determine place of antiphospholipids among women presenting repeated spontaneous abortion in Senegal. We tested prospectively 96 women in a breeding age with a past history of at least 2 spontaneous abortion. 14.6% and 21.1% of tese women present present respectively LA and ACL. The prevalence of antiphospholipids in among women presenting repeated abortion is higher in Senegal and similar to those previously reported confirming its place in this pathology important. A strategy of diagnosis should be defined between obstetricians and biologists in order to improve the obstetrical prognosis.

[Hematologic abnormalities and immunodepression in HIV/AIDS- related pulmonary tuberculosis]. / Anomalies de l'hémogramme et immunodépression dans l'associaton VIH/SIDA-tuberculose pulmonaire.

Our objectives were to describe hemato-immunological abnormalities encountered in tuberculosis patients HIV seropositive (TBVIH+, n = 67) or not (TBVIH-, n = 39) and in HIV asymptomatic patients (aSVIH+, n = 40). We found: a great reduction of mean value of RBC and Hb in TBVIH+ and TBVIH-; a reduction of mean value of leucocytes, total lymphocytes and CD4+ lymphocytes in TBVIH+ and aSVIH+; an inversion of [formula: see text] ratio, more important in TBVIH+ than in aSVIH+. HIV and tuberculosis association, HIV1 serotype and CD4 < 200/mm3 were found to promote significantly hemato-immunological abnormalities.