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Adrenocorticotropic hormone (ACTH) insensitivity syndrome is one of the rare causes of adrenal insufficiency in children. All described inherited ACTH insensitivity forms are of autosomal recessive origin. In our resource-poor Countries, many of these rare diseases are ignored or not diagnosed due to inadequate technical equipments. We report the case of a 4-month old infant hospitalized for refractory hypoglycaemias. Despite the patient had generalized and severe melanodermia, digestive disorders and ion channel disorders the diagnosis of cortisol deficiency was only diagnosed retrospectively during respiratory arrest with favorable outcome under hydrocortisone therapy. This study aims to highlight the clinical, laboratory and therapeutic features of peripheral cortisol deficiency, without enzymatic blocks, including this adrenocorticotropic hormone (ACTH) insensitivity syndrome.

Introduction: Expanded programme on immunizations in resource-limited settings currently measure vaccination coverage defined as the proportion of children aged 12-23 months that have completed their vaccination. However, this indicator does not address the important question of when the scheduled vaccines were administered. We assessed the determinants of timely immunization to help the national EPI program manage vaccine-preventable diseases and impact positively on child survival in Senegal., Methods: Vaccination data were obtained from the Demographic and Health Survey (DHS) carried out across the 14 regions in the country. Children were aged between 12-23 months. The assessment of vaccination coverage was done with the health card and/or by the mother's recall of the vaccination act. For each vaccine, an assessment of delay in age-appropriate vaccination was done following WHO recommendations. Additionally, Kaplan-Meier survival function was used to estimate the proportion vaccinated by age and cox-proportional hazards models were used to examine risk factors for delays., Results: A total of 2444 living children between 12-23 months of age were included in the analysis. The country vaccination was below the WHO recommended coverage level and, there was a gap in timeliness of children immunization. While BCG vaccine uptake was over 95%, coverage decreased with increasing number of Pentavalent vaccine doses (Penta 1: 95.6%, Penta 2: 93.5%: Penta 3: 89.2%). Median delay for BCG was 1.7 weeks. For polio at birth, the median delay was 5 days; all other vaccine doses had median delays of 2-4 weeks. For Penta 1 and Penta 3, 23.5% and 15.7% were given late respectively. A quarter of measles vaccines were not administered or were scheduled after the recommended age. Vaccinations that were not administered within the recommended age ranges were associated with mothers' poor education level, multiple siblings, low socio-economic status and living in rural areas., Conclusion: A significant delay in receipt of infant vaccines is found in Senegal while vaccine coverage is suboptimal. The national expanded program on immunization should consider measuring age at immunization or using seroepidemiological data to better monitor its impact.

Child hypothyroidism has been little studied in Senegal. The aim of this study was to evaluate the epidemiological, diagnostic and evolutionary aspects of congenital hypothyroidism. We conducted a descriptive-analytical retrospective study of all children treated for congenital hypothyroidism at the Albert-Royer National Children's Hospital Center over the period from 2001 to 2014 (14 years). We collected and analyzed socio-demographic, clinical and evolutionary data from patient medical records. A total of 28 patients were included in the study, an average of 2 cases per year. The average age of discovery of hypothyroidism was 54.25 ± 43 months with a female predominance (Sex-ratio 0.47). Only 2 cases of hypothyroidism were diagnosed in the neonatal period. Consanguinity was present in 68% of patients. Clinical signs were dominated by the delay in psychomotor acquisitions (96%), hypothermia (46%), cranio-facial dysmorphia (43%) and goiter (39%). Growth retardation was constant beyond 6 months. The etiologies were dominated by hormonosynthesis disorders (84.21%). During the study period, mean SD of patients had decreased from -3.5 SD to -2.25 SD for a median treatment duration of 28 months. Mental retardation was present in 73% of cases. Growth retardation and mental retardation were more severe as the diagnosis was late. Our results confirm the inadequacy of early management of patients. It is urgent to implement a routine neonatal screening system in order to improve the mental prognosis of this condition., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêt.

Hemolytic-uremic syndrome (HUS) is a common cause of organic acute renal failure (ARF) in children. It is a progressive complication of acute gastroenteritis (AGE), especially caused by Escherichia coli in children. This study aimed to describe the clinical, therapeutic and evolutionary aspects of this affection in four children. We collected four cases of HUS. The average age was 10,5 months (5-15mois), exclusively boys. Clinical examination revealed a hemolytic anemia (pallor and jaundice), oligoanuria and edematous syndrome (2 cases), arterial hypertension (1 patient), AGE associated with severe dehydration and hypovolemic shock (2 patients), consciousness disorders. ARF was found in all patients as well as thrombocytopenia and schizocytes smear. Direct Coombs test was negative. Hyperkalemia was found in 3 patients, of whom 1 with hyperkalemia level of more than 9.2 mmol/L, hyponatremia at 129 mmol/l (1 patient) and hypernatremia at 153 mmol/l (1 patient). HUS was secondary to pneumococcal pneumonia (1 patient) while AGE was secondary to E. coli (1 patient). The treatment was mainly symptomatic and included fluid restriction, transfusion of red cell concentrates, diuretics, peritoneal dialysis and hemodialysis. The evolution was marked by the onset of chronic renal failure (1 patient) after 6 months of follow-up and by recovery (1 case). Three patients died. HUS is the most common cause of organic acute renal failure in newborns. Diagnosis is essentially biological, treatment is mostly symptomatic.