Infantile Spinal Muscular Atrophy at the Albert Royer National Children’s Hospital Center in Dakar

Introduction: Infantile spinal muscular atrophy (ISA) is an autosomal recessive disease caused by primary degeneration of cells in the anterior horn of the spinal cord, leading to muscle weakness and hypotonia. Its incidence is estimated at 1 in 6000 births worldwide. In Africa, particularly in Senegal, there are few studies interested on this pathology. We therefore deemed this study necessary, which set itself the objective of describing the diagnostic, therapeutic and progressive aspects of infantile spinal muscular atrophy at the Albert Royer National Children’s Hospital Center in Dakar (CHNEAR). Methodology: We conducted a retrospective descriptive study over a period of two (2) years from December 2020 to December 2022. Included were all hospitalized patients in whom the diagnosis of spinal muscular atrophy was made with or without genetic confirmation. The data were collected on a pre-established form then entered and analyzed with the following software: Excel 2013 and R version 4.1.3. Results: During our study period, 2100 children were hospitalized, the annual incidence was 0.76%. The average age of our patients was 9 ± 9 months with a range of 3 months to 32 months and the median was 6.5 months. The sex ratio was 7. The notion of family consanguinity was found in 62.5% of cases and the notion of ISA in the family in 25% of cases. Hypotonia and respiratory distress were found at the forefront in equal proportions (50% of cases). Electromyogram (EMG) was performed in 3 patients (37.5%). Symptomatic medical treatment was administered in 100% of patients, 04 patients had benefited from respiratory physiotherapy, i.e. 50% of cases, and genetic counseling was carried out in one patient (12.5%). The evolution was immediately favorable in 2 patients or 25% of cases, unfavorable in 75% of cases with a death rate of 50% and the average age of death was 5.5 months ± 1 with extremes ranging from 3 to 7 months. Conclusion: The number of Infantile spinal muscular atrophy cases remains low in hospitals in Dakar. Diagnostic means are still difficult to access. The course is difficult to predict and is often marked in the long term by respiratory difficulties which can be fatal.

Congenital Heart Disease in Rural Senegal: A Retrospective Study of 79 Patients

Introduction: Congenital heart disease (CHD) is a malformation of the heart present at birth and resulting from a developmental defect during embryonic life. The aim was to assess CHD in rural areas. Methodology: This is a retrospective study conducted over a period of 2 years in rural Senegal. Results: During the study period, we collected 79 patients with CHD, representing a hospital prevalence of 2.6%. The mean age at diagnosis was 17.05 months and the sex ratio was 1.19. The mean birth weight was 2826 g. The main comorbidities were anaemia (54.43%) and respiratory infections 38 cases (48.10%). Respiratory distress was the most common sign with 98.73%. Radiological cardiomegaly was noted in 86.7%. The most frequent CHD were interventricular septal defect (IVD) 21.51% and atrial septal defect (ASD) 8.86%. No patient was able to receive surgical treatment. Seven children died (8.86%) and 21 children were lost to follow-up (26.58%). On Doppler echocardiography, 16.45% of the patients had pulmonary arterial hypertension (PAH). Conclusion: In light of this work, emphasis should be placed on the quality of antenatal consultations, the quality of management and the regular availability of echocardiography and a cardio-paediatrician in order to reduce morbidity and mortality.

Ischemic Stroke Complicating Neuromeningeal and Pulmonary Tuberculosis in Children: About a Case in Senegal

Tuberculosis is a major public health problem, especially in developing countries. Neuro-meningeal involvement is rare but represents one of the most severe forms with high morbidity and mortality. We report the case of an ischemic stroke complicating neuromeningeal and pulmonary tuberculosis in a four-month-old infant at the National Hospital Center of children Albert Royer (Senegal). The case was a four-month-old, male infant with no history. His vaccination was up to date according to Senegal’s expanded vaccination program. There was no notion of tuberculosis contagion. He was seen for a prolonged fever for more than a month and chronic cough. He had moderate malnutrition. Neurological examination revealed coma, meningeal syndrome, generalized tonic convulsions and hypertonia. The pupils were in bilateral miosis. Osteotendinous reflexes were abolished with a bilateral Babinski sign. He also had severe respiratory distress, bilateral pulmonary condensation syndrome. Brain computed tomography was in favor of an ischemic stroke. The test GeneXpert MTB/RIF was positive on cerebrospinal fluid and gastric tubing fluid. The curative treatment was based on a quadruple therapy based on rifampicin, isoniazid, pirazinamide and ethambutol. The evolution was unfavorable with the death of the patient.

Cardiac Echography in Pediatrics at the Regional Hospital of Diourbel: Indication and Result

Childhood heart disease is a real public health problem. In our contexts, care remains a major challenge. Doppler echocardiography remains the essential examination for diagnosis. The objective of our study was to describe the different clinical indications for cardiac ultrasound in children and the main pediatric heart diseases at the Diourbel Heinrich Lübke Regional Hospital. This was a descriptive and analytical retrospective study spanning from 2020 to 2022;covering a series of 140 cases. The study was carried out using patient clinical observation sheets and consultation sheets. We identified 140 ultrasounds. The average age of patients was 35.96 months with extremes of 0.03 months and 192 months. The female sex was predominant, with an M/F sex ratio of 0.72. Ultrasound was systematically requested in 67.14% of the study population. The indications found were mainly: heart murmur, polymalformative syndrome, respiratory failure, bronchiolitis, cardiomegaly, suspicion of heart disease, cardiomegaly, pre-therapeutic and pre-operative assessment. A total of 71 cardiac abnormalities were found in 65 patients, i.e. a prevalence of 46.42%. Congenital heart disease was more frequent (found in 49 patients, a prevalence of 35%) and was dominated by the CIA. There was a female predominance in almost all heart diseases except in the case of endocarditis where codominance is found. Our study has made it possible, through echocardiography, to evaluate the indications for echocardiography in children, to determine the prevalence of heart disease in children and also to assess the relevance of requests for echocardiography.

Descriptive and Analytical Study of Factors Associated with Mortality in Severe Malaria among Children in Dakar Emergency Departments from July to December 2022

Background: Malaria is the most widespread parasitic disease and remains a public health priority worldwide. The severe form is fatal if not treated early and appropriately. The aim was to carry out a descriptive and analytical study of the factors associated with mortality during severe malaria in children attending emergency departments in Dakar, Senegal. Methods: This is a prospective, observational and analytical study conducted over a 6-month period (July 1 to December 31, 2022), focusing on children hospitalized for severe malaria according to WHO severity criteria. Results: A total of 403 patients were hospitalized, including 78 cases of severe malaria (19.35%). Males predominated (60.26%) (sex ratio 1.51). The average age was 6.56 years [8 months - 14 years], with the [5 - 10 years] age group the most represented (40.26%). The average consultation time was 5.33 days (1 - 19 days). The main reasons for consultation were fever (70.51%), vomiting (24.35%) and convulsions (14.10%). Biological signs of severity were severe anemia (17.95%), renal failure (6.4%) and hypoglycemia (3.85%). Thrombocytopenia was noted in 52.56% of patients, including 32.05% of severe cases (sis in 61.41% of cases. Hyponatremia was noted in 39.74% of cases and hyperkalemia in 2 patients. Artesunate was the main drug used (93.59% of cases). Mortality was estimated at 1.5%. Factors leading to death were coma (P < 0.01), respiratory distress (P Conclusion: Malaria is still a public health problem, with a high mortality rate in emergency departments. Reducing this mortality rate requires effective management of the factors associated with death.

Pulmonary Arterial Hypertension in Congenital Heart Disease with Left-to-Right Shunt: According to a Retrospective Study at Albert Royer’s National Children’s Hospital Center

Pulmonary arterial hypertension PAH is a progressive disease characterized by an increase in pulmonary arterial pressure and resistance. It often complicates congenital heart disease with a left-to-right shunt. The objective of this study is to evaluate the evolutionary profile of the pulmonary pressures of children with congenital heart disease with left-to-right shunt and to identify the factors favoring an evolution towards PAH. This is a retrospective, descriptive and analytical study over a period of four years and six months (from January 1, 2016 to June 30, 2020) at the Center National d’Enfant Albert Royer in Dakar on a series of 87 cases. The hospital frequency was 3.98 per thousand, and the incidence of PAH was 9.44%. The sex ratio of 0.74. The average age was 44.76 months. The average time between the onset of symptoms and the diagnosis of heart disease is 78.81 days. Dyspnea was found in 70.11% of cases, the burst of pulmonary B2 was found in 55%. Global heart failure was noted in 39.08% of cases. Cardiomegaly was found in 89.66% and pulmonary hypervascularization was found in 57.72% of cases. CIV was present in 54.02% of cases, followed by PCA in 21.14% and CAVc in 18.39% of cases. furosemide was used in 97.70% of cases and Captopril in 74.71% of cases. Sildenafil was used in 10.34% of patients, and 12.64% of patients benefited from surgical management. Eisenmenger syndrome was found in 12.64% of patients. PAH in these heart diseases is a formidable and frequent complication, due to an increase in precapillary flow. The major improvement in mortality and morbidity is early surgical management, in the first months of life, to prevent pulmonary vascular disease.

Rheumatic Heart Disease in Children: Epidemiological Aspects. Diagnostic and Evolutive at the Dakar Chu

Rheumatic heart disease is the ultimate consequence of acute articular rheumatism. It remains a public health problem in developing countries. It is a pathology found in all countries of the world, with a clear predominance in developing countries. In Senegal, the hospital prevalence of rheumatic heart disease is 30.5%. Its seriousness lies in the risk of valvular sequel responsible for cardiac dysfunction that can lead to death. The objective of this study was to describe the epidemiological, diagnostic and evolutionary aspects of rheumatic heart disease at the Albert Royer National Hospital Center of child (CHNEAR). We had carried out a retrospective, descriptive and analytical study lasting 3 years from January 1, 2017 to December 31, 2019. All children aged 4 to 17 hospitalized at CHNEAR for rheumatic heart disease were included. The hospital prevalence was 0.8%. The average age was 10.9 years with a sex ratio of 0.875 in favor of girls. 85.47% of cases came from parents who had a low economic level of life. The history of repeated angina and acute articular rhumatism (AAR) was found respectively in 22.69 and 15.13% of cases. The clinical symptomatology was dominated by heart failure with 58.3% of cases, arthralgia in 7.5% of cases. The biological inflammatory syndrome was found in 86.1%. 73% of the patients had a positive antistreptolysin O antibody (ASLO). Cardiomegaly was found in 89.7%. Heart doppler ultrasound found polyvalvular involvement in more than half of the cases. Mitral insufficiency was the predominant valve disease in 98.20% of cases. The evolution was favorable in the majority of cases but 9.32% of deaths were observed.

Congenital Cyanogenic Heart Disease in Children: About 420 Cases in Africa

Congenital cyanogenic heart disease (CCHD) is a malformation of the heart and large vessels characterized by an oxygen desaturation in the arterial blood, responsible for cyanosis. The general objective was to study the profile of CCHD in Senegalese hospitals. This is a retrospective study carried out over a period of 8 years (January 1, 2010 - December 31, 2017) and including all children aged 0 to 16 years followed for a CCHD. The hospital prevalence was 0.87% for 420 cases collected. The sex ratio was 1.44 and the average age at diagnosis was 16 months. First degree parental consanguinity was noted in 36 cases (30.78%). The main reasons for consultation were breathing difficulty in 242 cases (57.62%) and fever in 136 patients (32.36%). Apart from cyanosis, the clinical signs were dominated by the heart murmur in 313 cases (74.7%), tachycardia in 283 cases (67.38%) and digital hippocratism in 162 cases (38.57%). Cardiomegaly was found in 239 patients (83.36%). The main types of CCHD were tetralogy of Fallot and transposition of the large vessels. In biology, 206 patients (49.05%) presented polyglobulia. A complete surgical cure was carried out in 22 patients (5.24%). Complications were anoxic crisis (52 cases) and hemorrhagic syndrome (17 cases). There were 97 deaths (28.28%) during hospitalization. The diagnosis of CCHD is late in our country and surgical management is poor explaining the high mortality.

Impact of COVID19 on Routine Immunization: A Cross-Sectional Study in Senegal

Introduction: The COVID 19 pandemic has prompted the world to implement drastic prevention methods based on limiting population movements that have an impact on public health policies such as vaccination. The objective of this work was to evaluate the impact of these prevention measures on routine vaccination in hospitals since the advent of the pandemic in Senegal. Methodology: This is a retrospective cross-sectional study carried out in August 2020 in the vaccination unit of the Abass NDAO hospital centre. We compared data from the vaccination unit during the period from March to August of the last three years (2018, 2019 and 2020). The parameter studied was the number of vaccine doses administered for the different periods according to the expanded programme of immunization. Results: For the vaccines administered in the sixth week in April, the number of doses was 36 in 2018, 29 in 2019 and 15 in 2020, i.e. a 50% drop compared to March. In July the number of doses administered was 40 in 2018, 35 in 2019 and 15 in 2020, a reduction of 42% compared to 2019. Conclusion: Measures to fight this pandemic should not make us forget routine vaccination, especially in our resource-constrained countries. It is essential to continue vaccination for children and to identify children who have missed vaccine doses for catch-up.

COVID-19 Infection and Homozygous SS Sickle Cell Disease in Children: About Two Cases in Ziguinchor/Senegal

The World Health Organization declared that corona virus diseases-19 (COVID-19) is a public health emergency. The COVID-19 pandemic is more likely to cause disaster in developing area including West Africa due to limited medical resources. COVID-19 reportedly causes severer conditions in adults with advanced age and in patients with underlying comorbidities including sickle cell anemia. We recently experienced two pediatric patients with sickle cell disease (SS), who had COVID-19. We here highlight the difficulties of management and the severity of COVID-19 infection in children with homozygous sickle cell SS.

Particularities of Tuberculosis in Children and Adolescents with Sickle Cell Disease in Senegal

Introduction: Tuberculosis and sickle cell disease are one of the main global health priorities particularly in Africa. We aimed to determine the epidemiological, diagnostic and therapeutic aspects of tuberculosis in children and adolescents with sickle cell disease. Patients and methods: Patients aged 0 - 20 years with sickle cell anemia presented with tuberculosis at the Centre Hospitalier National d’Enfants Albert Royer (CHNEAR), Dakar, Senegal were included in the study. Medical history, risk factors, clinical, bacteriological, and outcome data was collected. Data was analyzed using the SPSS software, version 16. Results: A total of 25 cases of tuberculosis were documented from January 1st, 1991 to December 31st, 2019 (hospital prevalence: 0.97%). Mean age was 12.5 years. The sex ratio was 1.5 (15 girls and 10 boys). Pulmonary tuberculosis in 14 cases was the most frequent followed by lymph nodes in 7 cases involvement and Pott’s disease in 4 cases. In 5 patients Tb was multifocal. Mean duration of treatment was 8.27 months (6 to 12 months). Outcome was good on antituberculosis treatment. None patient died. Conclusion: Children and adolescents with sickle cell disease can be infected with Mycobacterium tuberculosis. Pulmonary tuberculosis, lymph nodes and bone involvement are the most frequent localizations. Outcome is good on antituberculosis treatment.

Epidemiological, Diagnostic and Therapeutic Aspects of Cardiogenic Shock in Children at the Albert Royer Children’s Hospital in Dakar

Introduction: The objective of this study was to evaluate the epidemiological, diagnostic and therapeutic aspects of cardiogenic shock in children at the Albert Royer Children’s Hospital in Dakar. Methods: This was a retrospective, descriptive and analytical study from January 2020 to February 2021, including all children aged 2 months to 16 years hospitalised for cardiogenic shock diagnosed on the basis of clinical and ultrasound criteria. Results: During the study, 38 patients were hospitalised for cardiogenic shock. The hospital prevalence was 4.2%. The mean age of onset of shock was 64 months, and there was a predominance of females with a sex ratio of 1.92. Consanguinity was found in 42% of the patients. Consanguinity was found in 42% of patients. Infection was identified as a trigger for cardiogenic shock in 18 (52.9%) of our patients. The most common type of heart disease was rheumatic heart disease in 12 (32%) of the patients. The mortality rate was 65.8%. Conclusion: Cardiogenic shock is a diagnostic and therapeutic emergency. Its prevalence and mortality are still high in developing countries.

Epidemiology of Admissions in a Pediatric Emergency Department in Albert Royer Hospital Dakar

Background: Pediatric emergencies are a public health priority in Senegal. The objective was to describe the epidemiology of admissions to the emergency department of the Albert Royer National Children’s Hospital in Dakar, Senegal. Methods: Prospective, descriptive and analytical study over a period of 4 months (January-April 2020). The study population was children admitted for vital distress. Results: A total of 300 children met the inclusion criteria and represented the study population (2.83%). The majority (70.67%) were younger than 60 months, compared with 2.00% of newborns. Boys represented 53.8%, sex ratio 1.16. A proportion of 35.1% was referred. More than half of our patients (55.88%) were managed within 30 minutes of arrival. Medicalized transport concerned 21.5% against 78.5% of nonmedicalized. Emergencies were dominated by respiratory distress (56.33%), dehydration (29%) and shock (13.33%). We recorded 16 deaths (5.3%). This mortality was significantly related to the low socio-economic level (p = 0.000), as well as the young age of the children (p = 0.01). Conclusion: Life-threatening emergencies are frequent and responsible for significant lethality. Health policy efforts are still needed to reduce this infant and child morbidity and mortality.

Neonatal Mortality in Rural Area in Senegal

Most newborn deaths occur in two regions of the world, South Asia and sub-Saharan Africa. In Senegal, much progress has been made over the past two decades with a reduction in overall infant mortality by 38%. However, the decline in neonatal mortality has been slower during the same period. The objective of this study is to assess neonatal mortality, to determine the different causes and to make recommendations for improving care in rural areas. This is a retrospective study with a descriptive and analytical aim carried out in the pediatrics department of the Diourbel regional hospital, 130 km from Dakar, the Senegalese capital, over a 12-month period from January 1 to January 31, December 2018. All newborns hospitalized in the pediatric ward during the study period were included. Overall mortality was 30.6%, newborns between 0 and 7 days accounted for 90.6% of deaths, INBORNs accounted for 62.3% of deaths. Newborns whose mother’s age was between 20 and 35 years of age accounted for 69.3% of deaths. Newborns of first-time mothers accounted for 40.2% of deaths and those of multiparous mothers 31%. Newborns who did not reach term accounted for 58.9% of deaths. Newborns admitted for respiratory distress represented the majority of deaths 45.5% (n = 93) followed by those with low birth weight 32.5 (n = 65) followed by those with neurological manifestations 30.5 (n = 62). Conclusion: Neonatal mortality is very high in the Diourbel region and the main cause is the lack of human resources and a very insufficient technical platform. The fight against this mortality involves improving the technical platform and recruiting sufficient and well-trained staff.

Malaria Characteristics in Children with Sickle Cell Disease

Background: The relationship between sickle cell disease and malaria is the subject of much controversy. However, there is a lack of data in our services. Our objective was to study the epidemiological, diagnostic and evolutionary characteristics of malaria in children with sickle cell disease followed in a specialized setting. Patients and Methods: We conducted a retrospective, descriptive, and analytical study of children with sickle cell disease (SCD) who presented with malaria and were followed at the Ambulatory Care Unit for Sickle Cell Children and Adolescents (USAD) at the Albert Royer National Children’s Hospital in Dakar, from January 1st, 2017, to December 31th, 2019. We included all the followed pediatric patients, less than 16 years, with sickle cell disease who presented at least one episode of malaria, confirmed by a positive thick drop, during this 3 years. We did not include patients with incomplete records or those older than 16 years. The clinical and biological signs, the follow up was collected and analyzed with Excel package 2019. Results: Of 3773 patients followed for sickle cell disease, 21 had presented malaria. The frequency was 0.5% or 7 cases/year. However, we exploited the data of 14 of them. The sex ratio was 6 boys for a girl and the mean age at admission was 7.3 years. The highest number of malaria cases was observed in 2018 and the peak frequency was observed in November with 8 cases (57.1%). Fever was the most frequent symptom, observed in 10 patients (71.4%). All patients were SS homozygous, with a mean baseline hemoglobin level of 7.5g/dl. All patients had a positive thick blood smear and Plasmodium falciparum was the only species found in the blood smear, with a mean parasite density of 1693 parasites/ml of blood. All patients had anemia, with a mean hemoglobin level of 7.74 g/dl. Twelve patients (85.7%) were hospitalized and had all received injectable artesunate followed by oral Artemisinin Combination Therapy (ACT). Long-lasting insecticidal nets were used in 9 patients (69.2%). The evolution was favorable in all patients, any death was reported. NO REPRESENTATION OF YOUR DATA IN TABLES OR FIGURES: it was a little serial. Conclusion: The patients who presented the association of malaria and sickle cell disease were all SS homozygotes. However, malaria must be considered as serious in this chronic anemic setting. That’s why it is important to improve prophylaxis.

Primary Immune Deficiencies (PID): Diagnosis Challenges

Background: Primary Immune Deficiencies (PID) are rare, under-determined diseases particularly in sub-Saharan Africa. The diagnosis is often suspected with uncommon clinical signs. Infections are the main diagnostic circumstances in infants. Confirmation is often difficult because some additional examinations are unavailable in many of our countries. Aim: Our aim was to share the challenge of diagnosis and treatment in PID. Case Presentation: It is about two infants, a boy and a girl, with early several infections. Both of them presented a hypo-gammaglobulinemia and to the boy, the immuno-phenotyping lymphocyte showed a decreased level of lymphocytes CD19. We are looking for genetic confirmation but it is not easy. The treatment of these infants requires a substitution for life of immunoglobulin which is unavailable in our countries. Conclusion: PID are suspected with atypical clinical signs. Confirmation genetic diagnosis is difficult in low income countries. To improve the follow up, we need to strengthen clinical-biological collaboration.

Evolutionary Profile of Graves’ Disease in Children at Albert Royer National Children Hospital in Dakar

Graves’ disease is the most common cause of hyperthyroidism. Adequate management is an area of controversy, especially when it comes to children. The objective of our study was to assess the outcome of Graves’ disease treatment in Albert Royer Children Hospital of Dakar. This was a retrospective study conducted from 2001 to 2015, and which involved all children between 0 to 15 years of age who were being monitored in the Albert Royer National Children Hospital. The evolutionary modalities were: stability, remission, failure, relapse, lost to follow-up and death. The data were analyzed with SPSS software version 20.0. For the comparisons, the KHI 2 or Fisher test was used with a significance threshold (p < 0.05). During the study period, 88 children were enrolled. The average age was 8.6 years [ranging from 8 months to 15 years]. The consultation delay was 11.36 months. In our study, 61.4% of the patients were regular in the follow-up, the observance was good in 40.9% of the cases and 19 patients (21.6%) were lost to follow-up. Clinical courses of 69 children after treatment with Carbimazole for 37 months were promising in 21 cases (30.43%), with 17 cases of remission (24.63%) and 4 cases of stability (5.8%). Age older than 10, the initial ATD dose greater than 1 mg/kg/day and the initial free T4 lower than 50 pmol/l were significantly associated with remission with a p value of 0.01;0.024 and 0.004.