Continuous ambulatory peritoneal dialysis (CAPD) in children: a successful case for a bright future in a developing country.

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.

[Interauricular communications in children: diagnosis and treatment, about 49 cases at the Children?s University Hospital in Dakar]. / Les communications interauriculaires chez l'enfant: diagnostic et prise en charge à propos de 49 cas aux CHU pédiatriques de Dakar.

Interauricular communication (IAC) is the second most important congenital heart disease in children. It accounts for 6-8% of congenital cardiac malformations in children. However, many questions are still open about this pathology. Thus, this study aims to report the prevalence rate of IAC in hospital but especially to describe its clinical, paraclinical and therapeutic aspects. For this purpose, we conducted a retrospective descriptive study. Data were collected by means of a questionnaire and then entered and analyzed in Sphinx (V5). The prevalence in Hospital was 2x1000. The average age of patients was 37 months, the sex-ratio was 0,75. No prenatal diagnosis was made. Parental consanguinity was detected in 30% of cases. Respiratory infections were found in 24% of cases. Cardiomegaly was found in 35 cases with hypervascularization in 63% of cases. Ultrasound showed a predominance of ostium secundum; wide IAC was found in 63% of cases. Pulmonary stenosis was the most important associated heart attack. Pulmonary arterial hypertension was found in 63% of the cases. Treatment was based on diuretics, which were largely used and only 7 children underwent surgery. Evolution was favorable in 39 patients reflecting a rate of 79%. Early diagnosis of IAC should be improved as well as surgical treatment of IAC affecting infants.

[Heart failure with normal heart revealing complex arteriovenous malformation of the lower limb in a child: case study and literature review]. / Insuffisance cardiaque sur cœur normal révélant une malformation artériole-veineuse complexe du membre inferieur chez un enfant: à propos d'une observation et revue de la littérature.

Arteriovenous malformations (AVM) are congenital high-flow vascular defects. They are very rare in children. Diagnosis and treatment are often delayed due to their atypical place of occurrence and to their variable clinical manifestations. We report the case of a child treated at the National Centre Hospitalier Albert Royer Children, Dakar. A boy aged 9 years was referred from a health facility in rural areas for the treatment of heart failure. Admission clinical examination showed impaired general condition, global heart failure syndrome and voluminous right warm inguinocrural mass extended to the right abdominal wall (iliac fossa and right flank), with poorly defined limits. Auscultation of this mass showed a thrill and diffuse murmur. Cardiac ultrasound showed severe pulmonary arterial hypertension (PAH) with extensive impairement of the heart cavities, without cardiac structural involvement. The diagnosis of AVM was confirmed by doppler ultrasound of the mass complemented by angiography scan. They showed multiple arteriovenous fistulas within the mass. The diagnosis of complex stage IV AVM (according to Schöbinger classification) of the root of the right thigh was retained. Clinical treatment of heart failure was based on furosemide, spironolactone and captopril to obtain hemodynamic stabilization before possible surgical procedure. Arteriovenous malformations of the limbs, in particular of the proximal end of the lower limb in children are still largely unknown, hence frequent diagnostic errors and delays. Their evolution is unpredictable requiring early diagnosis and careful monitoring involving multidisciplinary interaction between pediatrics, surgeons and radiologists.

[Alobar holoprosencephaly associated with diabetes insipidus and hypothyroidism in a 10-month old infant]. / Holoprosencéphalie alobaire avec diabète insipide et hypothyroïdie chez un nourrisson de 10 mois.

Holoprosencephaly (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.

[Difficulties in the management of cerebral venous thrombosis (CVT) complicated by subarachnoid hemorrhage (SAH) in infants: about a case]. / Difficultés de la prise en charge d'une thrombose veineuse cérébrale (TVC) compliquée d'hémorragie sous arachnoïdienne (HSA) chez le nourrisson: à propos d'un cas.

CVT is generally rare, particularly among infants. The occurrence of a SAH associated with CVT is very uncommon and only a few cases have been reported in the literature. Symptoms are variable and often misleading. Outcome can be lethal or it can cause potentially severe sequelae if it is not treated or treated late. We report the case of a 22-month old infant examined for convulsions with Stage II coma, intracranial hypertension syndrome and infectious syndrome. Laboratory tests showed pseudomonas spp sepsis and cerebral CT scan allowed the diagnosis. Treatment was based on antibiotic therapy but mainly on anticoagulation therapy. The patient had a significant clinical improvement and follow-up cerebral CT scan showed disappearance of thrombosis with sequellar right hemoragic images. CVT is a severe disease, usually of infectious origin. Anticoagulant therapy is controversial, in particular in patients with associated hemorrhage, but clinical experience supports the effectiveness and safety of this treatment.

[Epidemiological, clinical and hematological profiles of homozygous sickle cell disease during the intercritical period among children in Ziguinchor, Senegal]. / Profils épidemiologiques, cliniques et hématologiques de la drépanocytose homozygote SS en phase inter critique chez l'enfant à Ziguinchor, Sénégal.

Sickle 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/mm3was 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.

[Hemolytic-uremic syndrome (HUS) in children at the University Hospital Center in Dakar: about four cases]. / Syndrome hémolytique et urémique de l'enfant au Centre Hospitalier Universitaire (CHU) de Dakar: à propos de quatre observations.

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.

[Congenital hypothyroidism in Dakar: about 28 cases]. / Hypothyroïdie congénitale à Dakar: à propos de 28 cas.

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.

[Portal cavernoma in children revealed by gastrointestinal haemorrhage: about a case]. / Cavernome portal chez l'enfant révélé par une hémorragie digestive: à propos d'un cas.

Portal cavernoma is a venous vascular anomaly characterized by the formation of a network of veins whose caliber is increased and carrying portal blood. It is due to a thrombotic and always chronic occlusion of the extra-hepatic portal venous system. This is one of the most common causes of portal hypertension in children. Its severity is mainly associated with an high risk of gastrointestinal haemorrhage. Very few cases have been described mainly in African literature. We report the case of a 4-year old boy admitted with very abundant haematemesis, melena and dizziness associated with anemic syndrome on examination. Laboratory tests showed severe microcytic hypochromic anemia with normal renal and hepatic function. Gastrointestinal endoscopy showed esophageal varices (grade III) with red signs. Abdominal ultrasound showed portal vein formation resulting in the classic "spiderweb", in favor of a cavernoma. Abdominal CT scan confirmed portal cavernoma associated with portal hypertensive syndrome and vascular anomaly like an ectopic splenic vein anastomosis with the trunk formed by the gonadal vein and the inferior mesenteric vein. Therapeutic approach was based on blood transfusion and beta-blocker treatment. Portal cavernoma can be a major complication of vascular malformations often unknown. In case of gastrointestinal haemorrhage in children, diagnosis should be suspected. Its management requires early treatment and should be adapted to the patient's condition in order to prevent a fatal evolution.