Crise vaso-occlusive drépanocytaire sévère: aspects cliniques, étiologiques et thérapeutiques au CHU Sylvanus Olympio de Lomé / Clinical, aetiological and therapeutic features of severe sickle cell related vaso-occlusive crisis at the Sylvanus Olympio University Hospital, Lomé

Introduction: la crise vaso-occlusive (CVO) est la plus fréquente manifestation de la drépanocytose et la première cause d´hospitalisation des enfants atteints. L´objectif de cette étude est de décrire les aspects cliniques des CVO sévères, de déterminer les étiologies des syndromes infectieux qui les accompagnent et de décrire leur prise en charge. Méthodes: il s'agit d'une étude transversale descriptive portant sur 137 drépanocytaires majeurs hospitalisés pour CVO sévères du 1er janvier 2009 au 31 décembre 2011 dans le service de pédiatrie du CHU Sylvanus Olympio. Résultats: les drépanocytaires homozygotes SS étaient les plus nombreux (n=98; 71,5%), suivis des doubles hétérozygotes SC (n=28; 20,5). Le délai moyen de consultation était de 4,7 ± 4,4 jours. Le traitement avant l´admission comportait des antibiotiques (28,5%). Les CVO étaient surtout ostéo-articulaires (70,8%). Dans 98,5% des cas, une infection bactérienne associée a été confirmée (48,9%) ou présumée (49,6%). Les principales étiologies étaient le syndrome thoracique aigu (26,3%), l´ostéomyélite aiguë (10,9%), l´infection urinaire (6,6%), la septicémie (3,6%). Un germe a été isolé chez 14,6% des patients, Escherichia coli (30%) étaient en tête suivi de Klebsiella pneumoniae (25%), Staphylococcus aureus (15%), Salmonella typhi (10%), Streptococcus pneumoniae (5%), le Streptocoque D (5%), l´Enterobacter (5%) et l´Acinetobacter (5%). Le taux de mortalité était de 2,2%. La durée moyenne d´hospitalisation était de 11,4 ± 8,8 jours. Conclusion: les CVO drépanocytaires sévères sont en majorité associées aux infections bactériennes en milieu tropical. Une antibiothérapie adaptée et précoce constitue le moyen thérapeutique indispensable pour prévenir ou traiter ces patients.

Introduction: vaso-occlusive crisis (VOC) is the most common manifestation of sickle cell disease and the leading cause of hospitalization among affected children. The purpose of this study is to describe the clinical features of severe VOCs, to determine the etiologies of infectious syndromes that accompany them and to describe their management. Methods: we conducted a descriptive cross-sectional study of 137 adult patients with sickle cell disease hospitalised for severe VOC in the Paediatric Department of the Sylvanus Olympio University Hospital from 1 January 2009 to 31 December 2011. Results: the majority of patients (n=98; 71.5%) had homozygous sickle cell (SS), followed by double heterozygous SC disease (n=28; 20.5). The median of consultation time was 4.7 ± 4.4 days. Treatment before admission was based on antibiotics (28.5%). VOCs were mainly osteoarticular (70.8%). In 98.5% of cases, an associated bacterial infection was confirmed (48.9%) or suspected (49.6%). The main etiologies included acute chest syndrome (26.3%), acute osteomyelitis (10.9%), urinary tract infection (6.6%) and septicaemia (3.6%). One germ was isolated from 14.6% of patients: Escherichia coli (30%), followed by Klebsiella pneumoniae (25%), Staphylococcus aureus (15%), Salmonella typhi (10%), Streptococcus pneumoniae (5%), Streptococcus D (5%), Enterobacter (5%) and Acinetobacter (5%). Mortality rate was 2.2%. The average length of stay in hospital was 11.4 ± 8.8 days. Conclusion: severe sickle cell related vaso-occlusive crisis is mainly associated with bacterial infections in tropical environments. Appropriate and early antibiotic therapy is the essential therapeutic means to prevent or treat these patients.

Severe Acute Bacterial Infections in Children With Sickle Cell Disease in Togo.

BACKGROUND: Bacterial infections are considered a major cause of morbidity and mortality in patients, especially children, with sickle cell disease. OBJECTIVES: This study aims at determining, a year after the introduction of the 13-valent pneumococcal conjugate vaccine the distribution of severe acute bacterial infections and germs in children with sickle cell disease. PATIENTS AND METHODS: Records of children 0 to 15 years of age and admitted from January 1, 2015 to December 31, 2019 (5 y), were examined retrospectively in the four sickle cell monitoring units in Lomé. RESULTS: The main infections found were pleuropulmonary (46.1%), urinary tract (32.8%), and osteoarticular (9.3%). A germ was isolated in 139 of the 265 cases (52.4%). 65.5% of the microorganisms isolated were Gram-negative organisms, with mostly Escherichia coli (31.6%) , and Klebsiella pneumoniae (18%) being the main germs. They were mainly responsible of urinary tract and osteoarticular infections. The majority of these Enterobacteriaceae was Extended-Spectrum Beta-Lactamase-Producing (41.1%, n = 37). Gram-positive cocci were represented by Staphylococcus sp (25.9%), Streptococcus sp (4.3%), Streptococcus pneumoniae (2.9%), and Enterococcus (1.4%). Staphylococcus aureus was the most common germ in pleuropulmonary (40%), osteoarticular (47.3%), and sepsis (28.6%) infections. CONCLUSION: Even if the infections found remained classic, there is a redistribution of germs with a decline in Salmonella and increase of Escherichia coli , Klebsiella pneumoniae , and Staphylococcus aureus .

Management of acute sickle cell priapism in an African (Togo) pediatric department includes conservative measures and intracavernous epinephrine which is safe and efficacious.

Priapism is a well-known urologic complication of sickle cell anemia. This study describes the results of a protocol for the treatment of acute priapism by intracavernous injection of epinephrine due to unavailability of etilefrine. A descriptive cross-sectional study of 18 cases of acute priapism in sickle cell patients treated in the pediatric department of the Sylvanus Olympio CHU from January 1 to December 31, 2020. The average age was 21.7 ± 7.7 years, the youngest patient was 8 and the oldest was 32 years old. Students represented 61.1% of the patients. The hemoglobin profiles were homozygous SS (n = 14) and double heterozygous SC (n = 4). Most of the crisis (83.3%) occurred at night. Most of the patients (66.7%) came to the hospital before the sixth hour of crisis, one patient came by the 48th hour. Walking was the most self-relief method tried by patients (67%). It was followed by a cold penile bath, attempted urination, body bath, and lastly lukewarm bath. Fourteen patients had a history of chronic intermittent priapism. The average pain intensity was 9.5 ± 0.9 with restlessness (33.3%) and crying (33.3%). Fifteen patients were treated upon admission with an intracavernosal injection of epinephrine, and three patients were first drained. Thirteen patients achieved remission immediately, while five patients required a second injection and only one had to be drained before remission. Tolerance was good. One patient had a borderline systolic blood pressure. One erectile weakness case was noticed and no cases of sexual impotence. Epinephrine by intracavernosal injection is an efficient treatment for acute priapism in sickle cell patients. Epinephrine, which has a good tolerance in pediatric and young adult patients, should be used in lieu of etilefrine due to its unavailability in areas where it is unavailable.

MG132 Induces Progerin Clearance and Improves Disease Phenotypes in HGPS-like Patients' Cells.

Progeroid syndromes (PS), including Hutchinson-Gilford Progeria Syndrome (HGPS), are premature and accelerated aging diseases, characterized by clinical features mimicking physiological aging. Most classical HGPS patients carry a de novo point mutation within exon 11 of the LMNA gene encoding A-type lamins. This mutation activates a cryptic splice site, leading to the production of a truncated prelamin A, called prelamin A ∆50 or progerin, that accumulates in HGPS cell nuclei and is a hallmark of the disease. Some patients with PS carry other LMNA mutations and are named "HGPS-like" patients. They produce progerin and/or other truncated prelamin A isoforms (∆35 and ∆90). We previously found that MG132, a proteasome inhibitor, induced progerin clearance in classical HGPS through autophagy activation and splicing regulation. Here, we show that MG132 induces aberrant prelamin A clearance and improves cellular phenotypes in HGPS-like patients' cells other than those previously described in classical HGPS. These results provide preclinical proof of principle for the use of a promising class of molecules toward a potential therapy for children with HGPS-like or classical HGPS.

Child mortality infected with HIV1 followed in 40 pediatric care sites in Togo.

INTRODUCTION: The infection in pediatric HIV is the reason a lot of problems in Africa The objective of our study were to identify factors associated with mortality during follow-up of children receiving antiretroviral therapy in Togo. METHODS: It was a cross-sectional study of 870 children aged files from 7 weeks to 15 years infected with HIV on antiretroviral treatment, covering the period 1 January 2001 to 31 December 2010 taking in 40 sites medical management in Togo. Data processing was done with the software Epi-Info 6.04d and duplicates were treated by the Software ESOPE. RESULTS: All patients were infected with HIV-1. In total forty six (46) deaths is 5.29% of the overall cohort were reported in our series. The lethality of the overall cohort followed for 60 months was 5.29%. The survival rate of the overall effective monitoring in our study was 89.2%. Fifty-eight percent (58%) of deaths had affected children in a state of severe malnutrition and forty two percent (42%) in a state of moderate malnutrition. Sixty two percent (62%) of children under HAART treatment died benefited monitoring a psychologist. CONCLUSION: The diagnostic inadequacies of pediatric HIV strike the prognosis of infected children. Efforts still needs to be done to improve the load take pediatric HIV in Togo.

Knowledge, attitude and practice of the mothers with anemia of children under five years old in the peadiatric department at Sylvanus Olympio teaching hospital in Lomé.

BACKGROUND: Anemia remains a major cause of morbidity and mortality of children in Togo despite of prevention effort, due to the parents lack of implication. AIM: To determine the knowledges, attitudes and practices of mothers, with anemia ofchildren under five years old Methods : Knowledge Attitudes and Practice survey from the first of february to 31 ofmarch 2012, about an interview of a hundred mothers with children under 5, randomly selected in the consultation, vaccination waiting rooms and in the hospitalisation. RESULTS: Forty mothers had never heard about anemia. Health personnel was the main source of mother's information (29%), mass media represented 8% of mothers information source (radio 5%; television 3%). The decreased of blood in the body is the most given definition from the mothers (44%). Malaria (24%) and malnutrition (19%) were the main causes cited by mothers. Iron deficiency has been mentioned by 3% of the mothers. Palmar - plantar pallor (32%) was the clinical signe the best known by the mothers. Most of the mothers (90%) had never assist to an information education and communication message about anemia prevention. When their children had anemia, 25 mothers (65,8%) took their children to the health center, five mothers (13,2%) had given tomatoes. The use of iron in prophylactic treatment was known by 43% of the mothers. The blend tomatoes and milk was the prophylactic treatment mentioned by 3% of the mothers. Most of the mothers (77%) would advice a mother with a children suffering from anemia to take him to the hospital. The knowledge of anemia by the mothers was correlated to then level of instruction. But the knowledge of prevention did not depend on the instruction level. CONCLUSION: anemia is not well known by the mothers of under five children. It's causes, it's treatment are not well known. Mass media are not very implicated on the subject. The reduction of it's frequency goes by information education and communication activities.