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Background and Aim: Idiopathic nephrotic syndrome or nephrosis causes massive protein leakage in the urine. Its treatment requires steroids (prednisone, methylprednisolone), often for a prolonged period, notably in case of steroid-dependence or steroid-resistance. In children, long-term use of steroids can lead to several side effects such as statural growth retardation/ stunting. This study evaluated the frequency of stunting in idiopathic nephrotic syndrome in children on steroids and identified the associated factors. Material and Methods: This was a retrospective, descriptive cohort study carried out in children aged 0 to 16 years treated at the paediatric nephrology unit of Aristide Le Dantec Hospital in Dakar, between 1 December 2017 and 31 May 2020. All records of nephrotic children treated in outpatient or inpatient setting were included. These children had to be on corticosteroid therapy for at least 30 months and have a height taken regularly during follow-up consultations. Results: Of 259 children followed for idiopathic nephrotic syndrome, 93 were included in the study. The median age was 96.5 months and the sex ratio was 1.9. The mean height of the children at the beginning of the follow-up was -0.26 DS, at the end it was -0.88 DS. At the beginning of the follow-up, 8 children had already stunting. At 12 months follow-up, 72 children (77.4%) had a decrease in z-score; and at 30 months, there were 7 more children (84.9%) who had a decrease in z-score. Methylprednisolone boluses were given to 17 children (18.3%). Calcium supplementation was done in 91 children (97.8%). Vitamin D supplementation was given to 91 children (97.8%). The mean number of relapses was 1.8. Factors associated with stunting were number of relapses ≤3 (p=0.03), duration of corticosteroid therapy >6 months (p100 mg/kg (p=0.04). Conclusion: In prolonged nephrotic syndrome in children, corticosteroids can cause stunting.

Introduction: Febrile seizures are seizures associated with fever in children. They represent a frequent reason for consultation in pediatric emergencies. Febrile convulsions are often qualified as benign pathologies but they are a source of concern, explaining the number of tolls imposed on these children. The aim of the study was to report the practice to a hospital in Senegal. Materials and Methods: This is a retrospective study of children's records, from January 1, 2015 to January 31, 2020. Were included, patients aged less than or equal to 5 years, hospitalized in the pediatric department of the Ziguinchor Peace Hospital, for a febrile convulsion. Newborns and children with psychomotor development abnormalities were excluded. We have studied the clinical and etiological data. Results: We collected 85 (51 boys, 34 girls) cases of febrile convulsions, ie a hospital frequency of 10,6%. The mean age of the patients was 23,6 months, peaking in infants 12 to 18 months. The mean temperature was 38,8° C and the seizure was tonic-clonic in 64,7%. We noted 57 cases of simple febrile convulsions (67,1%) and 28 cases of complicated febrile convulsions (32,9%). The etiology of fever was dominated by otorhinolaryngologic infections (42,4%); malaria (18,8%) and bronchopulmonary infections (08,2%). The average length of hospital stay was 02,8 days. We deplored two deaths (02,4%). Conclusion: Febrile convulsions are common in children under 5 years old. They are secondary to respiratory tract infections and malaria.

Malnutrition is a global health problem. It is very common in developing countries where it contributes to an increase in morbidity and mortality, especially among children under five years of age. The main objective of this study was to describe the management of severe acute malnutrition (SAM) in children six months to five years of age hospitalized at Diamniadio Children’s Hospital (DCH). This was an epidemiological, retrospective, descriptive and cross-sectional study of children aged six to 59 months hospitalized at DCH for SAM. The study took place over a 12-month period (from January 1, 2018 to December 31, 2018). During the study period, 67 children were admitted for SAM, representing a hospital prevalence of 8.4%. The majority (88%) children were less than 24 months old. Reasons for consultations were dominated by diarrhea (57.5%) vomiting (38.8%). Complications related to SAM were mainly: anaemia (74.6%), infections (85%) and severe acute dehydration (40.3%). Factors associated with SAM were young age (infants under 24 months of age), poverty, the first four children of a sibling, poor behaviour of food diversification. The average length of hospitalization was 12 days. The nutritional recovery rate was 73.1% and lethality was 3%. Conclusion: The prevention of malnutrition must involve the fight against poverty, an optimal and well-diversified diet, monitoring the nutritional status of children up to five years of age.

Aims: To firstly determine the incidence of acute renal failure (ARF) related to malaria in a series of 11 cases among admitted patients at the pediatric hospitals in Dakar, Senegal and to lastly measure the performance of rapid diagnostic test (RDT) and microscopy in malaria diagnosis using polymerase chain reaction (PCR) as a gold standard. Study Design: A bi-centric and descriptive study was carried out. Place and Duration of Study: From June 2018 to January 2019 in two university hospitals of Dakar, Senegal: Aristide Le Dantec university hospital and Albert Royer university hospital. Methodology: Pediatric patients aged under 18 years with confirmed malaria by RDT or microscopy and ARF defined by anuria or oligo-anuria and a decrease in glomerular filtration rate were included. Nested PCR was performed to confirm malaria diagnosis and Plasmodium species typing. Results: In total, 11 ARF cases (8.5%) related to malaria among 130 children infected with malaria parasites were included out of 4,474 hospitalized. Affected children were aged between 2 and 16 years with a mean age of 11 years and a sex ratio of 0.57. For malaria diagnosis, RDTs were positive for all patients while microscopy only revealed 6 cases (54.5%) as well as PCR. However, microscopy and PCR presented two discrepancies. Considering PCR as the gold standard tool, RDT showed a relative high sensitivity (100%) and zero specificity with a positive predictive value (PPV) of 54.6% while microscopy respectively showed a sensitivity and a specificity of 66.7 and 60%. Conclusion: This study showed the relatedness between ARF and P. falciparum malaria. Even though microscopy remains the gold standard for the diagnosis of malaria but microscopists must be regularly trained. In addition, RDT should always be confirmed by microscopy and preferably by PCR.

La drepanocytose est une maladie hereditaire de l’hemoglobine, a transmission autosomique recessive, caracterisee par la presence d’une hemoglobine anormale appelee « hemoglobine S ». Les syndromes drepanocytaires majeurs regroupent la forme homozygote « SS » et les heterozygoties composites (SC, SD et S beta thalassemie « Sβ »). Depuis fevrier 2011, une cohorte d’enfants et d’adolescents atteints de syndrome drepanocytaire majeur est suivi au Centre Hospitalier Regional de Saint-Louis, Senegal. Cette cohorte a fait l’objet de notre travail dont l’objectif principal etait de decrire les caracteristiques epidemiologiques, cliniques, biologiques et evolutifs. Il s’agissait d’une etude epidemiologique, retrospective et descriptive portant sur l’ensemble des dossiers des patients âges de 0 a 20 ans, porteurs d’un syndrome drepanocytaire majeur, regulierement suivis a la periode du 1er janvier 2011 au 31 decembre 2016 (6 ans). Le diagnostic de syndrome drepanocytaire majeur etait etabli sur la base d’une NFS et d’une electrophorese de l’hemoglobine. Durant la periode d’etude 191 patients porteurs d’un syndrome drepanocytaire majeur ont ete suivis au Centre Hospitalier Regional de Saint-Louis, Senegal. Cent vingt-sept (127) dossiers ont ete retenus dont 71 garcons et 56 filles. La majorite des patients (111 soit 87%) etaient de profil homozygote « SS ». L’âge moyen au diagnostic de la maladie etait de 4 ans et 10 mois et l’âge moyen de debut du suivi etait de 6 ans 2 mois. Les circonstances de decouverte de la maladie etaient dominees par une crise vaso-occlusive (58,5 % des cas). Le taux d’hemoglobine de base moyen etait de 8,6 g/dl. Les deux principales complications aigues rencontrees etaient une anemie aigue (22 %) et les infections severes (22 %). Les complications chroniques etaient dominees par la lithiase vesiculaire (5,5 %). Le nombre moyen d’hospitalisation etait de 1,9. La letalite etait de 3,1 %. La drepanocytose est frequente au Senegal. Elle est relativement bien toleree malgre son diagnostic et sa prise en charge tardifs. Pour ameliorer la prise en charge et le pronostic des malades, il sera necessaire de mettre en place un programme national de depistage neonatal et la creation de centres de reference de prise en charge et de suivi des patients drepanocytaires. Sickle cell disease is an inherited disease of hemoglobin, with recessive autosomal transmission, characterized by the presence of an abnormal hemoglobin called hemoglobin "S". Major sickle cell syndromes include the homozygote form "SS" and composite heterozygous (SC, SD and S beta thalassemia). Since February 2011, a cohort of children and adolescents with sickle cell disease has been monitored at the St. Louis Regional Hospital Center, Senegal. This cohort was the focus of our work, the main objective of which was to describe epidemiological, clinical, biological, and evolutionary characteristics. This was an epidemiological, retrospective, and descriptive study of all the records of patients aged 0 to 20 years, with major sickle cell syndrome, regularly followed in the period from 1 January 2011 to 31 December 2016 (6 years). The diagnosis of major sickle cell syndrome was based on hemogram and hemoglobin electrophoresis. During the study period 191 patients with major sickle cell syndrome were followed at St. Louis Regional Hospital Center, Senegal. One hundred and twenty-seven (127) were retained, including 71 boys and 56 girls. Most patients (111 or 87%) were of homozygotic "SS" profile. The average age at diagnosis of the disease was 4 years and 10 months and the average age of onset of follow-up was 6 years 2 months. The circumstances of discovery of the disease were dominated by a vaso-occlusive crisis (58.5% of cases). The average baseline hemoglobin level was 8.6 g/dL. The two main acute complications encountered were acute anaemia (22%) and severe infections (22%). Chronic complications were dominated by vesicular lithiasis (5.5%). The average number of hospitalizations was 1.9. The lethality was 3.1%. Sickle cell disease is common in Senegal. It is relatively well tolerated despite its late diagnosis and management. To improve the management and prognosis of patients, it will be necessary to set up a national neonatal screening program and the creation of referral centers for the management and follow-up of sickle cell patients.

Introduction: Congenital heart disease (CHD) is the leading cause of congenital malformations. CHD is a real public health problem in our developing countries due to delays in diagnosis and medical and surgical treatment. The general objective of our work was to study the epidemiological, clinical, paraclinical and therapeutic profile of CHD in a university hospital in Dakar. Methodology: We conducted a retrospective study for a period of 3 years (1st January 2015 to 31st December 2017). Results: During the study period, we retained 156 files with a hospital prevalence of 2.4%. The average pulse oxygen saturation of the patients was 89%. Ventricular septal defect (VSD) was the most frequent (45%), followed by atrial septal defect (ASD) (40%), patent ductus arteriosus (PDA) (26%), pulmonary stenosis (16%), atrioventricular septal defect (ASD) (4%) and Tetralogy of Fallot (2%). The sex ratio was 0.89. 93.10% of the patients were aged up to 24 months (n=148) against 6.90% aged over 24 months (n=11). The average pulse oxygen saturation of the patients was 89%. Ventricular septal defect (VIC) was the most frequent (45%), followed by atrial septal defect (ASD) (40%), patent ductus arteriosus (PCA) (26%), pulmonary stenosis (16%), atrioventricular septal defect (VAD) (4%) and Tetralogy of Fallot (2%).The sex ratio was 0.89. Among the patients, 93.10% were aged up to 24 months (n=148) compared to 6.90% aged over 24 months (n=11). The average age of children was 9 months. Conclusion: CHD are a reality in Africa, their frequencies in all reported series are certainly underestimated due to the very limited means of diagnosis.

L´épilepsie pose un problème de santé publique au Sénégal. L´objectif de l´étude était de décrire les aspects cliniques et paracliniques de l´épilepsie de l´enfant à l´Hôpital de la Paix de Ziguinchor (HPZ). Il s´agissait d´une étude documentaire de dossiers d´enfants épileptiques, du 1erjanvier 2015 au 31 décembre 2018. Ont été inclus, les patients âgés ≤ de 15 ans, suivis pour épilepsie à l´HPZ. Etaient exclus, les dossiers incomplets. Cinquante-cinq (37 garçons et 18 filles) enfants ont été colligés; 70,9% avaient un âge < à 5 ans. L´âge moyen était de 4,3 ans. Les patients étaient originaires du milieu rural (60%) et de famille défavorisée (67,3%). Les crises d´épilepsie étaient généralisées (72,7%) et focales (27,3%). Dix-huit patients présentaient une épilepsie idiopathique, 17 une épilepsie non idiopathique. Les facteurs étiologiques étaient dominés par les anomalies de la grossesse et de l´accouchement (29,1%). L´épilepsie est fréquente à l´HPZ. Elle prédomine en milieu rural chez les garçons de moins de 5 ans, issus de famille défavorisée. Les crises généralisées tonico-cloniques et les crises focales sont les tableaux cliniques les plus fréquentes et les anomalies de la grossesse et de l´accouchement sont les étiologies les plus retrouvées.

The authors report the first case of successful peritoneal dialysis (PD) in a developing country performed about a 13-year-old adolescent followed-up for stage V chronic kidney disease (CKD) with anuria. After 3 months of hemodialysis, the parents opted for continuous ambulatory peritoneal dialysis (CAPD) as they wished to return home located 121km from Dakar. After PD catheter insertion, the plan proposed to the patient consisted 3-4 hours stasis of isotonic dialysate during the day and a night stasis of 8 hours of icodextrin for an injection volume of 1L per session. The patient and his mother were trained and assessed on the PD technique. After dialysis adequacy was tested while hospitalised, they were able to return home and continued the sessions following the same plan prescribed and while keeping in touch, by telephone, with the medical team. The technique assessment at the day hospital every 2 weeks revealed dialysis adequacy and satisfactory tolerance of PD at home after 04 months of observation. It was the first case of successful CAPD in the pediatrics unit in this context. Scaling this technique is a challenge for the pediatric nephrologist in developing countries like Senegal.

La communication interauriculaire (CIA) constitue la seconde cardiopathie congénitale la plus importante chez l'enfant, elle représente 6 à 8% des malformations congénitales cardiaques de l'enfant. Toutefois, de nombreuses questions demeurent ouvertes concernant cette pathologie. Ainsi notre objectif était de rapporter la prévalence hospitalière des CIA mais surtout d'en décrire les aspects cliniques, paracliniques et thérapeutiques. Pour y parvenir nous avons réalisé une étude rétrospective descriptive. Les données ont été recueillies grâce à un questionnaire puis saisies et analysées sur Sphinx (V5). La prévalence hospitalière était de 2 pour 1000. L'âge moyen était de 37mois et le sex-ratio de 0,75. Il n'y a eu aucun cas diagnostiqué en anténatal. Une consanguinité parentale était retrouvée dans 30% des cas. Les infections respiratoires étaient présentes dans 24% des cas. Une cardiomégalie était retrouvée dans 35 cas avec une hyper vascularisation pour 63%. L'échographie notait une prédominance du type ostium secundum; la CIA était large dans 63% des cas. La sténose pulmonaire représentait l'atteinte cardiaque associée la plus importante. L'HTAP était présente dans 63% des cas. Les diurétiques étaient très utilisés et seuls 7 enfants avaient bénéficié d'une cure chirurgicale. L'évolution était favorable chez 39 patients soit 79%. Le diagnostic précoce des CIA doit être amélioré ainsi que la prise en charge chirurgicale des formes avec retentissement du nourrisson.

Introduction: Purulent pleurisy refers to the presence in the large pleural cavity of a purulent, cloudy or lemon-yellow liquid, but containing altered polynuclear cells. It is a frequent pathology, the epidemiology of which is constantly changing in Africa and throughout the world. This is why we conducted this study with the aim of updating epidemiological, clinical, bacteriological and therapeutic data. Methods: This is a retrospective, mono-centric, descriptive study including the records of children aged 0 to 15 years old hospitalized during the period from 20 May 2014 to 26 March 2019 at the Children's Hospital of Diamniadio for purulent pleurisy. The data were entered and analyzed using sphinx, Excel under Windows 8 and SPSS software. Results: We counted 42 cases of purulent pleurisy, i.e. a hospital frequency of 0.7%. Most of the patients were aged between 2 and 12 years (48%). The average age at diagnosis was 46 months. The sex ratio was 2.5. Most of the children came from parents with a low socio-economic status (64%); fever was the most frequent reason for consultation (50%), followed by cough (33%) and respiratory difficulty (29%). In all cases, a liquid effusion syndrome was objectively diagnosed (100%), tachycardia in 36% of cases and a pulmonary condensation syndrome in 33% of patients. X-rays showed a predominantly right-sided effusion (62%) which was very abundant in 71% of cases. In bacteriology, staphylococcus aureus was the most frequently isolated germ (75%), followed by streptococcus pneumoniae (20%) and group D streptococcus (5%). Biology showed an average hemoglobin level of 9.2 and there was a predominantly neutrophilic hyperleukocytosis in 98% of cases. All patients had received antibiotic therapy and pleural drainage in 76% of cases. Mortality was 10%. Conclusion: In the light of this work, we insist on the importance of early diagnosis and adequate management of purulent pleurisy in order to avoid complications and after-effects. Thus, antibiotic therapy should be more closely monitored, and its administration better codified to reduce bacterial resistance and germ selection.

Introduction: Despite much progress, purulent childhood meningitis (PCM) remains a public health problem. The objective of this work was to determine the epidemiological, clinical, therapeutic and evolutionary profile of purulent meningitis in children. Methods: Based on retrospective work carried out over a 4-year period (01 January 2014 to 31 December 2017), we studied the epidemiological, clinical, therapeutic and evolutionary aspects of cases of purulent meningitis hospitalized in the pediatric ward of the Children's Hospital of Diamniadio. Included were all children from 29 days to 15 years of age in whom the diagnosis of purulent meningitis was confirmed by the laboratory. Results: The hospital frequency was 1.9%. The mean age of the patients was 41.0 months. Among the affected children, 68.6% were fully vaccinated. The main germ was Neisseria meningitidis W135 (58.8%). Third generation cephalosporins were the antibiotics of choice (97.1%). No resistance was found to them. The cure rate with sequelae was 5.7%. Streptococcus pneumoniae was the germ responsible for 50% of the objective sequelae. The mortality rate was 7.1%. Conclusion: Neisseria meningitidis W135 is the main germ of purulent meningitis in our study. It is not included in the national routine immunization. It is imperative to adapt vaccination to the epidemiological fluctuation of pathogens in our regions.

Introduction : L'hyperthyroidie de l'enfant semble rare et constitue un probleme de par son retentissement psychosomatique. L’objectif etait de faire le point sur ses aspects epidemiologiques et diagnostiques chez l’enfant a Dakar. Methodes : Il s'agissait d'une etude multicentrique, descriptive sur 15 ans. Etaient analyses les aspects epidemiologiques, cliniques et etiologiques. Resultats : 239 patients selectionnes avec une prevalence de 2.4%, un sex ratio (H/F) de 0.36, un âge moyen de 10.8 ans. A l'inclusion, il s'agissait d'un aine de famille (26.3%), d'une croissance avancee (36.9%), retardee (12.5%), d'une corpulence insuffisante (40.1%). L'etiologie etait la maladie de Basedow dans 90.3% avec un facteur psychoaffectif dans 22.1%. Sur le plan clinique, predominaient la tachycardie (92.4%), le goitre (91.1%), l'exophtalmie (81.8%), l'amaigrissement (69.8%) avec cependant une enuresie (30.2%) et des manifestations psychiques (3.1%). Les manifestations cardiovasculaires et cutanees etaient positivement associees avec l'âge (p < 0.05). Le goitre etait associe au sexe feminin (p = 0.005), aux signes cardiovasculaires (p = 0.02), neuropsychiques (p = 0.03), cutanees (p = 0.03) et a la diarrhee (p = 0.03). La T4 libre etait correlee a l'âge (p = 0.007), la diarrhee (p = 0.021), l'anxiete (p = 0.024), la frequence cardiaque (p = 0.00) et la maladie de Basedow (p = 0.04). Plus le goitre etait volumineux, plus etait augmentee la T4 libre (p = 0.007). Conclusion : L'hyperthyroidie de l'enfant se differencie de celle de l'adulte par les facteurs d'induction, les perturbations sur la croissance et l'enuresie. L’âge et le sexe semble favoriser le tableau clinique de thyrotoxicose et les signes associes. Mots cles : Hyperthyroidie, enfant, Senegal

La drépanocytose pose un problème de santé publique au Sénégal. Elle concerne principalement les enfants et les adolescents. L'objectif de notre travail était de déterminer les profils épidémiologiques, cliniques et hématologiques de la drépanocytose homozygote SS dans une cohorte d'enfants suivis à l'hôpital de la paix de Ziguinchor. Il s'agit d'une étude rétrospective portant sur des dossiers d'enfants drépanocytaires. Etaient inclus, les drépanocytaires SS âgés entre 2mois et 21ans, reçus en phase inter critique durant la période d'étude allant du 1er Janvier 2015 au 31 Août 2017. N'étaient pas inclus dans l'étude les hétérozygotes composites (SC, S béta thalassémie). Nous avons colligé 46 dossiers de drépanocytaires SS (20 filles et 26 garçons). L'âge moyen des enfants était de 8,0ans [11mois-21ans]. Environ 1/3 des enfants (39,1%) avaient un âge inférieur ou égal à 5 ans. Il y avait une diversité ethnique avec une prédominance de diola (30,2%) suivi de mandingue (27,9%) et de poular (25,6%). L'âge moyen des enfants à la 1ére crise était de 35,5mois [7-192 mois]. Plus de 1/3 des enfants (41,3%) avaient fait une 1ére crise avant le deuxième anniversaire. Le type de la 1ére crise, qu'avait présenté l'enfant, était dominé par la crise vaso-occlusive (32,6%) suivi du syndrome pied-main (30,4%). Les signes cliniques en phase inter critique étaient la pâleur 95,6%); l'ictère (36,9%) et la splénomégalie (21,7%). A l'hémogramme, le nombre de globules blancs moyen était de 12465 leucocytes/mm3 [5340-26900]. L'hyperleucocytose, supérieure à 10 000 leucocytes/mm3 était retrouvée chez 34 malades (73,9%). La totalité des malades présentait une anémie avec une moyenne 08,6 g/dl [05,7-11,8]. Le taux d'hémoglobine S variait entre 54,6 et 98,4%. Le diagnostic et la prise en charge médicale de la drépanocytose SS sont tardifs à Ziguinchor. Le dépistage néonatal pourrait favoriser une prise en charge précoce dans la région.Mots clés: Drépanocytose SS, phase inter critique, enfantEnglish Title: Epidemiological, clinical and hematological profiles of homozygous sickle cell disease during the intercritical period among children in Ziguinchor, SenegalEnglish AbstractSickle cell disease poses a public health problem in Senegal. It mainly affects children and adolescents. This study aimed to determine the epidemiological, clinical and hematological profiles of homozygous (SS) sickle cell disease in a cohort of children followed-up at the Peace Hospital in Ziguinchor. We conducted a retrospective study of the medical records from children with sickle cell disease. All patients aged between 2 months and 21 years with sickle cell disease SS during the intercritical period, hospitalized during the study period from 1st January 2015 to 31 August 2017 were included in our study. Compound heterozygous patients (SC, S Beta Thalassemia) were not included. We collected 46 medical records of patients with sickle cell disease SS (20 girls and 26 boys). The average age of children was 8,0 years [11 months-21 years]. Approximately 1/3 of children (39.1%) had an age less than or equal to 5 years. There was an ethnic diversity showing a predominance of the Diola (30.2%) followed by the Mandinga (27.9%) and the Poular (25.6%). The average age of children with first crisis was 35,5 months [7-192 months]. More than 1/3 of children (41.3%) had had first crisis before their second anniversary. In the child, first crisis was dominated by vaso-occlusive crisis (32.6%) followed by hand-foot syndrome (30.4%). Clinical signs during the intercritical period were pallor 95.6%), jaundice (36.9%) and splenomegaly (21.7%). Mean white blood cell count was 12465 leucocytes/mm3 [5340-26900]. Hyperleukocytosis greater than 10 000 leucocytes/mm3 was found in 34 patients (73.9%). All patients had anemia with an average hemoglobin of 08,6 g/dl [05,7-11,8]. Hemoglobin S rate ranged between 54.6 and 98.4%. Diagnosis and medical management of sickle cell disease SS are delayed in Ziguinchor. Neonatal screening may lead to improve early management of patients in the region.Keywords: Sickle cell diasease SS, intercritical period, child

La drépanocytose pose un problème de santé publique au Sénégal. Elle concerne principalement les enfants et les adolescents. L'objectif de notre travail était de déterminer les profils épidémiologiques, cliniques et hématologiques de la drépanocytose homozygote SS dans une cohorte d'enfants suivis à l'hôpital de la paix de Ziguinchor. Il s'agit d'une étude rétrospective portant sur des dossiers d'enfants drépanocytaires. Etaient inclus, les drépanocytaires SS âgés entre 2mois et 21ans, reçus en phase inter critique durant la période d'étude allant du 1er Janvier 2015 au 31 Août 2017. N'étaient pas inclus dans l'étude les hétérozygotes composites (SC, S béta thalassémie). Nous avons colligé 46 dossiers de drépanocytaires SS (20 filles et 26 garçons). L'âge moyen des enfants était de 8,0ans [11mois-21ans]. Environ 1/3 des enfants (39,1%) avaient un âge inférieur ou égal à 5 ans. Il y avait une diversité ethnique avec une prédominance de diola (30,2%) suivi de mandingue (27,9%) et de poular (25,6%). L'âge moyen des enfants à la 1ére crise était de 35,5mois [7-192 mois]. Plus de 1/3 des enfants (41,3%) avaient fait une 1ére crise avant le deuxième anniversaire. Le type de la 1ére crise, qu'avait présenté l'enfant, était dominé par la crise vaso-occlusive (32,6%) suivi du syndrome pied-main (30,4%). Les signes cliniques en phase inter critique étaient la pâleur 95,6%); l'ictère (36,9%) et la splénomégalie (21,7%). A l'hémogramme, le nombre de globules blancs moyen était de 12465 leucocytes/mm3 [5340-26900]. L'hyperleucocytose, supérieure à 10 000 leucocytes/mm3 était retrouvée chez 34 malades (73,9%). La totalité des malades présentait une anémie avec une moyenne 08,6 g/dl [05,7-11,8]. Le taux d'hémoglobine S variait entre 54,6 et 98,4%. Le diagnostic et la prise en charge médicale de la drépanocytose SS sont tardifs à Ziguinchor. Le dépistage néonatal pourrait favoriser une prise en charge précoce dans la région.

L’holoprosencéphalie (HPE) est une malformation cérébrale grave due à un défaut de clivage médian du prosencéphale. C’est une anomalie le plus souvent associée à des malformations cranio-faciales, d’un retard du développement psychomoteur, d’un diabète insipide et de troubles endocriniens variables. Les étiologies sont diverses regroupant les anomalies chromosomiques (trisomie 13, 18), les syndromes polymalformatifs (syndrome de CHARGE). Le diagnostic repose sur l’imagerie cérébrale. Quelques rares cas ont été décrits dans la littérature. Nous rapportons le cas d’une HPE alobaire chez un nourrisson de 10 mois. Le diagnostic a été posé au scanner cérébral devant la constatation d’un retard du développement psychomoteur en l’absence de malformations visibles. Le bilan endocrinien a permis de déceler un diabète insipide central et une hypothyroïdie centrale probablement d’origine hypothalamique.Mots clés: Holoprosencéphalie alobaire, nourrisson, diabète insipide, hypothyroïdie centraleEnglish Title: Alobar holoprosencephaly associated with diabetes insipidus and hypothyroidism in a 10-month old infantEnglish AbstractHoloprosencephaly (HPE) is a serious brain malformation due to a failure of medial forebrain cleavage. This is an abnormality which is more often associated with craniofacial malformations, psychomotor development delay, diabetes insipidus and variable endocrine disorders. It is due to different causes including chromosomal abnormalities (trisomy 13, 18)and polymalformative syndromes (CHARGE Syndrome). Diagnosis is based on brain imaging. A few rare cases have been described in the literature. We here report the case of alobar HPE in a 10-month old infant. Diagnosis was based on cerebral CT scan performed due to delayed psychomotor development and in the absence of visible malformations. Endocrine assessment allowed to detect central diabetes insipidus and central hypothyroidism, probably of hypothalamic origin.Keywords: Alobar holoprosencephaly, infant, diabetes insipidus, central hypothyroidism