Thérapeutique transfusionnelle chez l’enfant drépanocytaire

Sickle cell disease is a genetic hemoglobinopathy characterised by vasoocclusive events and chronic haemolytic anaemia. Transfusion is a major therapeutic modality in this disease by decreasing the percentage of abnormal haemoglobin Hb S while increasing oxygen carrying capacity. Simple transfusions or exchange transfusions can be indicated occasionally or on a chronic transfusion program. Iron overload, vascular access and alloimmunization to erythrocyte antigens are causes of great concern in these young patients.     

Le régime sans gluten chez l’enfant

Children with celiac disease do not share the same risks as adults, and should have an adapted diet and a prolonged follow-up until adulthood. The following indications seem reasonable: in symptomatic children and adults, gluten free diet during several years in children and for the entire life in adults; in children becoming “silent”, without any clinical or biological signs of malnutrition, discussion of coming back to normal diet, under the condition a strict follow-up and restarting gluten free diet at age of 25 and/or in case of pregnancy, to prevent the complications of celiac disease in adulthood; prolonged follow-up for adolescents and young adults returned to a “latent” disease.     

Érythraphérèses chez l’enfant drépanocytaire,expérience d’une unité d’aphérèse pédiatrique

Erythrocyte exchanges on cell separators can be used in children with sickle cell disease and are effective in lowering the level of haemoglobin S. Of the 938 aphereses performed in 2020 in our unit, we observed a low rate of failure of procedures and few complications. Ninety-six percent of erythraphereses were performed in the context of chronic exchange programs, in more than 80% of cases for cerebral vasculopathy or after the occurrence of ischemic strokes. Less than 4% of the procedures were performed for specific indications (preparation for cholecystectomy most often). The vascular access is rarely an obstacle to the realisation of the apheresis. In case of insufficient venous capital, installing an arteriovenous fistula may be considered. Depending on the child's weight, haemoglobin level, and the severity of the sickle cell anaemia, precautions may be necessary when priming the procedure. Nurses experienced in paediatric apheresis and a good medical knowledge of sickle cell disease allowed us to use this technique from the age of 3 years and the weight of 15 kg.     

Les manifestations de la maladie cœliaque chez l’enfant

The knowledge regarding celiac disease has increased dramatically in recent years, due to the availability of accurate serologic markers. Mass screening studies have shown that the prevalence of sensitization can be as high as 1/80. The range of symptoms is wide, from the classic growth failure, denutrition and diarrheoa in infancy to clinically and histologically asymptomatic sensitized subjects. The interest of a routine mass screening is debated. The classical celiac disease in infancy is well known. Atypical symptoms and potentially associated disease are more frequent and potentially confounding. Physicians should be aware of any clue for celiac disease in atypical cases in order to improve the diagnostic yield, and therefore avoiding short or long term consequences.     

Aspects épidémiologiques et cliniques de la leishmaniose cutanée à Kairouan-Tunisie et particularités chez l’enfant

Cutaneous leishmaniasis (CL) remains highly endemic in Tunisia. The governorate of Kairouan (Center) is one of the most affected particularly by zoonotic form due to Leishmania major. The purpose of this study was to describe the epidemiological, clinical and therapeutic features of the disease in this governorate and to identify any particularities in children. Three hundred and ninety cases were registered over 2 years (2012 and 2013). The average age was 29 years one month [± 22.7] (34.6% of patients were under 15 years). The cumulative incidence of CL was equal to 34.4 per 100,000 per year. It was higher in children (39.6 per 100,000 versus 29, p=0.004). Cases were diagnosed especially in autumn and winter (88.3% of cases between October and January, p<0.001). The average delay of consultation was one month and 22 days [± 2.5] after onset of lesions. The average number of lesions per patient was 2.7 [± 2.3] with a mean diameter equal to 2.2 cm. In addition to a higher incidence of the disease, two other features were found in “Children” group which are the presence of similar cases in the neighborhood (27.7% versus 13.4%, p=0.002) and preferential localization in the face (36.4% versus 11%) while the members were more affected in adults (63.5% versus 46.4%) (p<0.001). Meglumine antimoniate was prescribed for most of our patients (93.1%). The intramuscular route was used more than local one, probably because of high proportions of multiple lesions and facial localization.     

Prévalence de l’obésité de l’enfant et de l’adolescent en milieu scolaire à Dakar

Obesity is currently viewed as a serious worldwide public health issue. In this survey, we aim to determine its prevalence among schoolchildren and teenagers in Dakar. The sample of our survey consisted of 2,356 students aged 11-17, including 1,110 boys and 1,246 girls. For each of them, we have calculated their body mass index. Based on the higher value of the norm of that index, referred to the 97th percentile of Rolland-Cachera MF's curves, we have identified obese students according to their age and sex. The prevalence of obesity within our sample is 9.34%, with 2.88% for boys and 6.46% for girls. It is at its peak in the age of 11, though there is no significance (p > 0.05) in its decrease (from age 12 to 17). There are significantly (p < 0.05) more obese students in "catholic private" schools than in "public" schools where schooling is free. Child and teenage obesity is a reality in Dakar schools. Consequently, it is advisable to determine its nationwide prevalence to take on its prevention as well as its cure.     

Prise en charge des troubles de l’hémostase chez l’insuffisant hépatique

Patients with end-stage liver disease have complex alterations that involve all components of hemostasis, with changes both in prohemostatic and in antihemostatic pathways. Routine haemostasis tests such as prothrombin time and platelet count are unable to reflect a bleeding tendency. Bleeding complications are much less related to abnormal hemostasis than previously thought, with portal hypertension playing a more critical role. Systematic prophylactic measures based upon the use of fresh frozen plasma and/or platelet concentrates to improve or correct the abnormalities of the routine coagulation tests are most often inappropriate and may occasionally be deleterious.     

Représentations de l’échec thérapeutique des traitements ARV chez les patients et les professionnels de santé à Dakar

The aim of this qualitative study was to describe and analyze representations of therapeutic failure and the medical and social responses proposed by health care professionals and patients receiving follow-up in the ANRS cohort 1215 in Senegal from 1999 to 2010. Patients’ medical histories show that therapeutic failures are related to complex multifactorial situations, resulting from factors attributable to patients (adherence failure related to various psychosocial problems) but also to health care structures (organization of the health system, training for health care professionals and availability of biological exams and appropriate drugs). Both patients and health care staff recognize these causes. The incidence of the onset of therapeutic failures should be regarded as an indicator of the quality of care provided. Further thought should be given to how well health care systems function when changes occur in these rates.     

Hernies ombilicales étranglées chez l’enfant au Burkina Faso: différences avec les pays développés

Umbilical hernias occur frequently in children but complications are rarely reported. This study assesses the incidence of complicated umbilical hernias in our patients, evaluates data for risk factors, and shows dissimilarities with those encountered in developed countries. This study reports all children operated for complications due to strangulated umbilical hernia over a period of 3 years. On the whole, 162 children had umbilical hernias treated during this period. Thirty (18.5%) of these had complicated hernias. The average age of the complicated group was 3½ years. Twenty-nine cases had a painful irreducible umbilical mass. Twenty-four children had bowel obstruction, while stercoral fistula occurred in one child. The average diameter of the hernia ranged between 1 and 1.5 cm. Five patients had ischemic intestine that required resection. One patient died. When active observation and follow-up after 1 year is difficult or not feasible when the wall defect diameter is 1.5 cm or less, and in suspicion of incarceration (unexplained abdominal pain, and irreducibility), umbilical hernia should be operated.     

Profil épidémio-clinique des toxidermies bulleuses chez l’enfant à Abidjan (Côte d’Ivoire)

Severe cutaneous drug reactions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life threatening in adults. They seem to be less common in children. The purpose of this study was to describe the epidemiological, clinical and etiological profile of these drug reactions in African child. It was about a retrospective study carried on for 10 years at the Dermatology center of University Hospital of Treichville, Abidjan (Cote d’Ivoire).Were included all children aged 0–15 years hospitalized for severe cutaneous drug reaction. They represented 14.1% (27 cases) with an estimated hospital rate of 0.01%. The sex ratio (M/F) was 1.2. The mean age was 10.3 years. 19 children were suffering from SJS (63%) and 9 children (33.3%) from TEN. Sulfonamides were the most commonly used drugs with sulfadoxin-pyrimethamin (25.9%), used for malariae, and cotrimoxazole (22.2%). Self-medication was practiced by 70.4% of parents. The average time to onset of lesions from drug intake was 8.2 days. Only one child was HIV infected. Three children affected by TEN (11.1%) died.     

Profil de l’hémogramme et intérêt de la mesure de l’hémoglobine pré-don chez des donneurs de sang de la région Nord-Ouest du Maroc

Background

Blood donation in Morocco and more particularly in the northwest region is carried out without prior determination of the pre-donation hemoglobin. In addition, we note the lack of scientific research that reports data on the red blood cells, leukocytes and platelet lines in donated blood at the regional or even national level.

Apport du génotypage érythrocytaire à l’immuno-hématologie receveur à travers trois années d’activité à l’EFS Alpes-Méditerranée

Aim of the studyCurrent knowledge of the molecular basis of most blood groups enables genetic testing for blood groups to overcome the limitations of agglutination. A retrospective review was carried out on genotyping assays performed between 2011 and 2013.Methods and patientsThe Molecular Hematology Laboratory of the EFS Alpes-Méditerranée implements commercially available tools (BioArray, Gen-Probe) and other techniques (TaqMan, tetra-primer ARMS-PCR, sequencing). It provides a high-level of expertise in molecular biology, complying with regulatory requirements and standards.ResultsA total of 2382 genotyping assays was performed including 764 extended typings and 115 large extended typings essentially in cases involving multiple transfusion and suspected rare blood type. Phenotype discrepancies linked to the RH system accounted for 1501 genotypings. Discrepancies linked to the D and E were mainly related to an allele coding for weak antigen (weak D type 1, 2, 3 and EIV) while those linked to C, c and e antigens were related to an allele coding for a partial antigen (RN, ces(340), ceMo). A high prevalence of (C)ces haplotype in trans of a DAR allele was observed in Afro-Caribbean (54/62).ConclusionIn transfusion medicine, red-cell genotyping can overcome the limitations of hemagglutination. It must be used only in situations where it provides a benefit either for the patient or resource management. For implementation of appropriate transfusional practices, this technique requires a sound knowledge of the genetic characteristics of blood groups and clinically relevant variants. It also requires competency with molecular biology tools and continuously updated scientific data.     

Prévention de l’anémie chez les donneurs de sang

The prevention of anemia of blood donor is a main issue for donor safety and self-supplying. This prevention is done in one hand by donor deferral whose haemoglobin level is under defined threshold and in other hand by preventing iron deficiency. Some subgroups of donors are at increased risk for developing iron deficiency and adverse effects of iron deficiency: premenopausal females; donors with haemoglobin values near the minimum for eligibility and frequent donors. Different interventions could be used: lengthening the inter-donational interval and/or decreasing the number of donations per year; donor ferritin testing to evaluate iron store and at least donor iron supplementation.