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.

Acute Bilirubin Encephalopathy: A Propos of 151 Cases Collected during a Multicentric Study in Senegal

Introduction: Bilirubin encephalopathy is a debilitating complication of severe neonatal unconjugated bilirubin jaundice. The aim of this study was to determine the frequency of occurrence of this complication and to describe the diagnostic and prognostic aspects in Senegal. Materials and Methods: This was a multicenter, descriptive and analytical study conducted in 32 neonatal units of various levels, with retrospective (January to December 2020) and prospective (January to August 2021) collection. All neonates hospitalized for acute bilirubin encephalopathy were included. The diagnostic criteria were the presence of jaundice associated with neurological signs (muscle tone disorders, abnormal movements, convulsions, etc.), with no other obvious etiology found. The data were entered and analyzed using SPSS version 23 software. The significance threshold was retained for a p-value Results: We registered 151 patients. The mean age was 6 days and the sex ratio was 2.5 (clear male predominance). The majority of newborns were from Dakar (51%). Twenty-two (22%) were premature and 32% had low birth weight. Half of the newborns (50%) came from home and 87% were exclusively breastfed. The average time to consult was 3 days. Neurological signs were dominated by decreased primitive reflexes (74%), abnormal movements (59%) and lethargy (50%). Dehydration (30%) and anemia (26%) were often associated. The mean bilirubin level was 416 umol/l. Neonatal infections (19%) and Rhesus (16.7%) and ABO (8.7) incompatibility were the main causes. One third (33%) of patients had received intensive phototherapy and only 2% had received exchange blood transfusion. The case fatality was 48.9%. Conclusion: Bilirubin encephalopathy remains a major concern in Senegal. It is associated with high mortality and numerous neurological sequelae. Progress in terms of early detection and appropriate management is urgently needed on a national scale.

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.

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.

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.

Pneumothorax in Children: Epidemiological Diagnostic and Evolutionary Aspects at the Albert Royer National Children’s Hospital in Dakar

Pneumothorax is defined by the presence of air between the parietal pleura and the visceral pleura. Most of the child’s pneumothorax is traumatic. The spontaneous pneumothorax (PS) of the child is rare, and often associated with a favoring factor. When it is large, it can affect the life expectancy. The objective of our study was to determine the epidemiological, diagnostic and evolutionary aspects of pneumothorax in children aged 0 to 15 years, hospitalized at the Albert Royer National Children’s Hospital in Dakar. This is a retrospective descriptive study of patient records followed at the Albert Royer National Children’s Hospital (CHNEAR) for the management of a pneumothorax during the period from 2020 to 2022. During our study, 15 patients were hospitalized for pneumothorax, with an average annual incidence of 7.5 cases per year. The sex ratio (H/F) was 2.03. The average age was 3.7 years. Chest pain was present in 27% of patients, dyspnea was found in 93%, and cough in 87% of patients. All patients underwent chest radiography and the location of the pneumothorax was predominant on the right in 60% of cases. Thoracic drainage was performed in 93.3% of patients associated with etiological treatment. The average length of hospitalization was 27.8 days with a cure rate of 86.7% and a recurrence rate of 13.3%.

Acute Thoracic Syndrome in Children: Epidemiological, Diagnostic and Evolutionary Aspects at the Albert Royer National Children’s Hospital in Dakar Senegal

Acute chest syndrome (ACS) is a serious pulmonary complication of sickle cell disease. It is estimated to be responsible for a quarter of deaths in the pediatric sickle cell population. In Senegal, there are not enough pediatric studies in this area. The objective of our study was to determine the epidemiological, diagnostic and evolutionary characteristics of ATS at the Albert Royer National Children’s Hospital (CHNEAR) in Dakar. This was a retrospective study in patients hospitalized at CHNEAR for ATS from January 1, 2021 to March 31, 2022. We included patients hospitalized and diagnosed with ATS. We had collected 102 patients, i.e. a hospital incidence of 2.96%. The average age of the children was 9 years old;the sex ratio was 1.04. The main symptoms on admission were hypoxemia (97.06%), chest pain (77.45%), dyspnea (77.45%) and fever (65.69%). 52.94% of patients had an associated vaso-occlusive crisis (VOC). The chest x-ray was abnormal in 92 patients, a rate of 90.20% and showed images of pneumonia (71%);bronchitis (17.65%) and pleurisy (0.98%). None of the children benefited from a pulmonary ultrasound. The treatment associated with analgesics (100%), broad-spectrum antibiotics (100%), oxygen therapy (100%), hydration (95.09%), transfusion (73.53%), non-ventilation invasive (6.86%), intubation (2.94%) and beta 2 mimetics (12.75%). No patient benefited from incentive spirometry. Almost all of the patients 95.10% (n = 97) had a favorable clinical evolution. However, five children (4.90%) had an unfavorable outcome including one case of complication such as stroke (0.98%) and four (4) cases of death. The average hospital stay was 8 days. ATS is common in children with sickle cell disease in Senegal and its etiologies seem to be dominated by infectious causes in our context.

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.

A Severe COVID-19 Infection in Neonates: A Case Report

Coronavirus disease (COVID-19) is a highly contagious infectious disease. First case was reported at the end of 2019 in China. Children and neonate population appears to be relatively free of the virus. We reported a severe and fatal neonate case of COVID-19 in a 22 days old neonate female due to the delta variant strain during the third wave outbreak. She presented with fever, cough, grunting and diarrhea started 5 days before admission. Physical exam’s revealed severe respiratory distress, hypoxia and bilateral pulmonary condensation. PCR test for COVID-19 was positive. Chest X-ray found bilateral infiltrates. Chest CT showed diffuse ground glass images with 75% of lung parenchymal involvement. She was treated with antibiotics, corticostoid, respiratory support (CPAP and oxygen). Death occurred after 15 days of hospitalization in the context of multiple organ failure and pulmonary hypertension.

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.

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.

Pelvic Tuberculosis Simulating an Ovarian Tumor at a Stage of Peritoneal Carcinosis

Tuberculosis is a global public health problem with 10 million people developing an active form each year. The exact incidence of the pelvic form of tuberculosis is not known due to under-reporting of asymptomatic cases, vague symptomatology and lack of reliable diagnosis. We report a case of ovarian tuberculosis mimicking cancer and peritoneal carcinosis. The case involved a 15-year-old girl, hospitalized for severe pelvic pain and secondary amenorrhea. The examination showed an altered general state, a fever, a sensitive distended abdomen. The biology showed an elevation of Ca125 to 357 IU/ml. The CT scan showed a latero-uterine mass taking contrast in a heterogeneous way with an irregular and nodular thickening of the peritoneum making evoke a peritoneal carcinosis. Exploratory laparotomy found a bilateral ovarian mass with ileo-ileal, ileo-parietal and ileo-ovarian adhesions. Biopsy of the mass and the peritoneum showed a caseo-follicular gigantocellular epitheloid granulomatosis.