8 research outputs found
Efficacité d'utilisation du tryptophane cristallisé, sous forme libre ou protégée, pour le dépÎt protéique chez le porcelet
National audienc
Recherche des hĂ©moglobinopathies qualitatives chez les hĂ©mophiles Ă Abidjan, CĂŽte dâIvoire
Introduction : LâhĂ©mophilie est une maladie hĂ©rĂ©ditaire rare Ă transmission rĂ©cessive liĂ©e au chromosome X. Les principaux symptĂŽmes sont lâhĂ©morragie, les hĂ©matomes, lâanĂ©mie et les douleurs articulaires dans les hĂ©marthroses. Ces deux derniers signespeuvent se retrouver au cours des hĂ©moglobinopathies. Ainsi, lâobjectif de cette Ă©tude Ă©tait de rechercher la prĂ©sence dâhĂ©moglobinopathies qualitatives dans une cohorte dâhĂ©mophiles suivis au service dâhĂ©matologie clinique du CHU de Yopougon.MĂ©thodes : Câest une Ă©tude transversale qui sâest dĂ©roulĂ©e de Janvier Ă Juillet 2017 au niveau de lâunitĂ© dâhĂ©matologie du laboratoire central du CHU de Yopougon. Sur les prĂ©lĂšvements de 49 patients, ont Ă©tĂ© effectuĂ©s lâhĂ©mogramme, lâĂ©lectrophorĂšse de lâhĂ©moglobine, le taux de prothrombine (TP), le temps de cĂ©phaline activĂ©e (TCA) et le dosage des facteurs VIII et IX par la mĂ©thode chronomĂ©trique.RĂ©sultats : La moyenne dâĂąge Ă©tait de 17 ans avec 73,5% dâĂ©lĂšves et Ă©tudiants. Les hĂ©marthroses (75,5%) et les hĂ©matomes (36,7%) constituaient lâessentiel des signes cliniques. La complication majeure Ă©tait la dĂ©formation articulaire Ă 34,7%. Sur les 49 patients constituant la cohorte, le pourcentage dâhĂ©mophiles A Ă©tait de 89.8% (44/49) et celui dâhĂ©mophiles B 10,2% (5/49), avec 81,6% (40/49) dâhĂ©mophiles sĂ©vĂšres. La frĂ©quence des hĂ©moglobinopathies qualitatives est de 8,2% (4/49) avec 6,2% de trait drĂ©panocytaire AS (3/49) et 2% dâhĂ©moglobine AC (1/49).Conclusion : Cette Ă©tude a mis en Ă©vidence la prĂ©sence dâhĂ©moglobinopathies qualitatives, pouvant coexister avec lâhĂ©mophilie, ce qui peut compliquer la prise en charge et la qualitĂ© de vie des patients.
Mots clés : Hémophilie, hémogramme, électrophorÚse, Abidjan.
English title: Research of qualitative haemoglobinopathies in hemophiliacs in Abidjan, CĂŽte dâIvoire
Introduction: Hemophilia is a rare hereditary disease with X-linked recessive inheritance. The main symptoms are haemorrhage, hematoma, anemia and joint pain associated with hemarthrosis. These last two signs can also be found during haemoglobinopathies. Thus, the objective of this study was to investigate the presence of qualitative haemoglobinopathies in a cohort of hemophiliacs followed in the clinical hematology department of Yopougon University Hospital.
Methods: This is a cross-sectional study that took place from January to July 2017 at the hematology unit of the central laboratory of the Yopougon University Hospital. On the samples of 49 patients, blood count, haemoglobin electrophoresis, prothrombin time (PT), activated partial thromboplastin time (APTT) and factor VIII and IX were measured by the chronometric method.
Results: The average age was 17 with 73.5% of students. Haemarthrosis (75.5%) and hematoma (36.7%) constituted the bulk of clinical signs. The major complication was joint deformity with 34.7%. Of the 49 patients in the cohort, the proportion of hemophiliacs A was 89.8% (44/49) and that of hemophiliac B was 10.2% (5/49), with 81.6% (40/49) of all patients showing severe haemophilia. The frequency of qualitative haemoglobinopathies was 8.2% (4/49) with 6.2% of haemoglobin AS or sickle cell trait (3/49) and 2% of haemoglobin AC (1/49).
Conclusion: This study highlighted the presence of qualitative hemoglobinopathies that can coexist with hemophilia, which can complicate the management and quality of life of patients.
Keywords: Hemophilia, hemogram, electrophoresis, Abidjan.
 
Upstream stimulating factors: highly versatile stress-responsive transcription factors
International audienceUpstream stimulating factors (USF), USF-1 and USF-2, are members of the eucaryotic evolutionary conserved basic-Helix-Loop-Helix-Leucine Zipper transcription factor family. They interact with high affinity to cognate E-box regulatory elements (CANNTG), which are largely represented across the whole genome in eucaryotes. The ubiquitously expressed USF-transcription factors participate in distinct transcriptional processes, mediating recruitment of chromatin remodelling enzymes and interacting with co-activators and members of the transcription pre-initiation complex. Results obtained from both cell lines and knock-out mice indicates that USF factors are key regulators of a wide number of gene regulation networks, including the stress and immune responses, cell cycle and proliferation, lipid and glucid metabolism, and in melanocytes USF-1 has been implicated as a key UV-activated regulator of genes associated with pigmentation. This review will focus on general characteristics of the USF-transcription factors and their place in some regulatory networks