Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso.

BACKGROUND: Orofacial clefts are usually isolated cases but can be associated with other congenital malformations that are either recognised or unrecognised syndromes. The reported prevalence and pattern of such associated malformations, however, vary among studies. OBJECTIVES: To assess the frequencies and aetiologies of congenital malformations and associated medical conditions in children with orofacial clefts in Burkina Faso (Western Africa). METHODS: A retrospective descriptive study was carried out at the El Fateh-Suka Clinic in Ouagadougou, Burkina Faso. All children who attended surgery for the repair of a cleft lip and/or palate were included in this study. RESULTS: The frequency of congenital malformations associated with cleft lip and/or palate was 39/185 (21.1%). In the group with multiple congenital malformations of unknown origin (34 patients; 18.4%), 66.7% had cleft lip and palate, followed by isolated cleft lip (27.4%) and isolated cleft palate (5.9%). The digestive system (35.3%), the musculoskeletal system (19.6%), and eye, ear, face, and neck (15.7%) were the most affected systems. In the group of syndromic malformations (five patients; 2.7%), amniotic band syndrome (one patient), Van der Woode syndrome (one patient), Goltz syndrome (one patient), and holoprosencephaly (two patients) were identified. Medical conditions included anaemia (39.4%), infections (9.2%), malnutrition (7.5%), and haemoglobinopathies (4.3%). CONCLUSIONS: Congenital malformations and medical co-morbidities were frequent in children with OFCs. Further studies and a National Malformations Registry are needed to improve the comprehension of OFCs in Burkina Faso.

[Acute lymphoblastic leukemia among children in Ouagadougou (Burkina Faso): the results of treatment according to the protocol of the Franco-African Pediatric Oncology Group 2005]. / Les leucémies aiguës lymphoblastiques de l'enfant à Ouagadougou (Burkina Faso): résultats de la prise en charge selon le protocole du Groupe Franco-Africain d'Oncologie Pédiatrique 2005.

INTRODUCTION: acute lymphoblastic leukemia (ALL) is being diagnosed in an increasing number of children in our Department. In the developed countries, the treatment of this hematologic malignancy can cure almost 80% of children. In developing countries, few studies focus on acute leukemias in children. The results of cancer treatments in children are disappointing in most African countries, with a survival rate of 10-15%. This study aimed to investigate the clinical, biological, therapeutic and evolutionary features of ALL in children. METHODS: we conducted a retrospective study of the medical records of children hospitalized for ALL between November 2009 and October 2011 in the pilot Paediatric Oncology Unit at the Charles de Gaulle University Pediatric Hospital Center, Ouagadougou (Burkina Faso). All children treated according to the protocol of the Franco-African Pediatric Oncology Group 2005 (FAPOG) were included in the study. RESULTS: in total, nine children with ALL were hospitalized during the two year study period. The average age of patients was 10.77± 2.82 years. They were predominantly male. The average time of hospitalization was 43.11 days ± 39.54 days. The main symptoms were alteration of general state and fever. Nearly all the patients had tumor syndrome and bone marrow failure. Myelogram showed ALL type 1 in six of the nine patients. Eight patients underwent chemotherapy according the protocol of FAPOG 2005. Children's evolution was favorable in two patients who experienced remission, four patients had treatment failure. Six patients died. CONCLUSION: thanks to information campaigns, which will contribute to encourage early consultations, capacity-building measures for the medical staff allowing early diagnosis of ALL, the construction of a sufficiently equipped pediatric oncology center and a subsidy of anticancer drugs awarded by the state of Burkina Faso, the treatment of children with ALL would allow for better outcomes.

[Major sickle cell syndromes and infections associated with this condition in children in Burkina Faso]. / Syndromes drépanocytaires majeurs et infections associées chez l 'enfant au Burkina Faso.

INTRODUCTION: This study aims to investigate infections in children with major sickle cell syndrome. METHODS: We conducted a monocentric descriptive retrospective hospital study in Ouagadougou, Burkina Faso, over a ten-year period. All children with major sickle cell syndrome (homozygous SS and double heterozygous SC, SDPunjab, Sß thalassemic, SOArab and SE) hospitalized for microbiologically confirmed infections were enrolled in the study. RESULTS: One hundred and thirty-three patients met our inclusion criteria. The SS phenotype accounted for 63.2% of cases and SC 36.8%. The frequency of infections was 21.8%. In 45.9% of cases, these affected children aged 0-5 years. The most frequent signs were osteoarticular pain (42.1%), cough (25.7%), abdominal pain (23.3%), pallor (43.6%). The major diagnoses were bronchopneumonia (31.6%), malaria (16.5%), osteomyelitis (12.8%) and septicemia (10.5%). The isolated pathogenic organisms were Streptococcus pneumoniae (35.5%) and Salmonella spp (33.3%). Third generation cephalosporins were the most commonly prescribed antibiotics. Gros mortality rate was 7.5%. CONCLUSION: Bacterial infections and malaria dominate the clinical picture of infections in children with major sickle cell syndrome at the at the Pediatrics University Hospital Center Charles De-Gaulle. This study highlights the importance of establishing a national program for the management of sickle-cell anemia, which could help prevent or reduce the occurrence of infections in children with sickle cell syndrome.

[Pepper's syndrome: report of two cases at the Charles de Gaulle University Pediatric Hospital Center, Ouagadougou (Burkina Faso)]. / Le syndrome de Pepper: à propos de deux cas observés au Centre Hospitalier Universitaire Pédiatrique Charles de Gaulle de Ouagadougou (Burkina Faso).

Pepper's syndrome is a neuroblastoma that metastasizes to the liver. It affects infants younger than six months of age. It can regress spontaneously and is associated with a favorable prognosis in 80% of cases. Given its rarity, we here report two cases of Pepper's syndrome observed at the Charles de Gaulle university pediatric hospital center, Ouagadougou (Burkina Faso). Our study involved two female infants in whom the disease manifested as an increase in abdominal volume, hepatomegaly and signs of respiratory distress. Ultrasound enabled diagnosis, which was based on the nodular appearance of the liver in both cases and determination of the primary tumor in one case. Urinary catecholamine test confirmed the diagnosis in one case. Both patients died from complications related to liver compression, chemotherapy (in one of the cases) and lack of treatment (in the other case).