Evaluation of the Nutritional and Hematological Status of Sickle Cell Children Monitored in the Pediatric Department of the University Hospital Center of Yalgado Ouedraogo

Objective: To assess the nutritional and hematological status of sickle cell children followed in the department of pediatrics of the Yalgado Ouédraogo University Hospital Centre (CHU-YO).Methodology: This was a cross-sectional study conducted from September 1, 2017, to February 28, 2018. All children with major sickle cell syndrome followed in the department of pediatrics at the CHU-YO and following their follow-up appointments were included in the study.Results: We included 230 children aged 11 months to 16 years with an average age of 8.5 years. The sex M/F ratio was 1.09. The SC heterozygotes were the most represented with 56.52%. The average hemoglobin level was 9.39 g/dl. The prevalences of wasting, stunting and underweight were respectively 23.04%, 15.65%, and 13.89%. In univariate analysis, the factors associated with emaciation was hyperleukocytosis (p=0.002).The factors associated with stunting were leukocytosis (p=0.01), severe anemia (p=0.01), SS phenotype (p=0.002), age range of 5-10 years (p=0.007), Secondary (P=0.007) and higher level (p=0.001) of father’s education, secondary (p=0.027) and higher level (p=0.034)of mothers’education , farmer(p=0.003) trader (p=0.042), and informal occupation of father (p = 0.002),and breastfeeding duration after 24 months (p=0.006). For underweight associated factors in univariate analysis were SS phenotype (p=0.003) and severe anemia (p=0.01).Conclusion: The prevalence of different types of malnutrition deficiency of sickle cell children followed at CHU-YO was high. It is important to strengthen the nutritional monitoring of children with sickle cell disease for better management of the disease

Congenital vitiligo: A case observed in the cohort of HIV-exposed infants in Bobo-Dioulasso, Burkina Faso

Vitiligo is a dermatological disease; its exact prevalence is unknown among the paediatric population. We are reporting a case of vitiligo at birth for the first time in Burkina Faso, in the Teaching Hospital Souro Sanou of Bobo-Dioulasso, Paediatric Department. He is a male child, born from HIV-1 positive parents; we received him when he was 2 months to be followed in connection with the prevention of motherto- child transmission. He showed achromic lesions on the skin and on skin appendages at birth. In addition to congenital vitiligo we mentioned, several diagnostic hypotheses were discussed. No treatment was decided to face these skin lesions given the very young age of the patient. Psychological support is planned in the long run

Diagnostic path of a genetic disease : a case of Williams-Beuren syndrome in Burkina Faso

Williams-Beuren syndrome (WBS) is a rare neurodevelopmental disorder characterized by a set of somatic, psychological, and behavioral abnormalities, which is caused by a deletion of several genes. Herein we report a 6 year-old boy, who presented with mental retardation and psychological disorders. The result of the first clinical examination was poor, since it didn’t detect any dysmorphic feature which is a major component for the clinical diagnosis of WBS. Despite the multidisciplinary and the multicenter approaches used, the diagnosis of WBS (deletion of chromosome band 7q11. 23) was established more than 3 years after the first medical consultation. Rare partial forms of WBS have been recently described and they are both clinically and genetically difficult to diagnose. Unfortunately, this disorder is still little known by health professionals

Contribution and acceptability of bacteriological collection tools in the diagnosis of tuberculosis in children infected with HIV

Objective: The objective of this study is to evaluate the feasibility and tolerability of new bacteriological samples to diagnose tuberculosis (TB) in HIV-infected children. Method and patients: HIV1-infected children with suspicion of TB in Universitary Hospital Souro Sanon (Burkina Faso) were included in a prospective cohort study. Children underwent three gastric aspirates (GA) if aged <4 years; two GA, one string test (ST) if aged 4-9 years and three sputum, one ST if aged 10-13 years. All children underwent one nasopharyngeal aspirate (NPA) and one stool sample. To assess feasibility and tolerability of procedures, adverse events were identified and pain was rated on different scales. Samples were tested by microscopy, culture, GeneXpert (R) (Xpert (R)). Results: Sixty-three patients were included. Mean age was 8.92 years, 52.38% were females. Ninety-five GA, 67 sputum, 62 NPA, 60 stool and 55 ST had been performed. During sampling, the main adverse events were cough at 68/95 GA and 48/62 NPA; sneeze at 50/95 GA and 38/62 NPA and vomiting at 4/55 ST. On the behavioral scale, the average pain score during collection was 6.38/10 for GA; 7.70/10 for NPA and 1.03/10 for ST. Of the 31 cases of TB, bacteriological confirmation was made in 12 patients. Conclusion: ST, stool is well-tolerated alternatives specimens for diagnosing TB in children. NPA has a poor feasibility and tolerability in children

The Seckel Syndrome: A Case Observed in the Pediatric Department of the University Hospital Center Sourou Sanou (Burkina Faso)

Seckel syndrome-1 or “bird-headed dwarfism”, Online Mendelian Inheritance in Man number 210600, is a rare genetic disease with an autosomal recessive transmission. We report a female child of 56 months diagnosed with SCKL1 at the Pediatric department of the University Hospital Center Sourou Sanou, Burkina Faso. She showed the typical features including facial dysmorphism, dwarfism, microcephalus and mental retardation. Ophthalmic and dental anomaly and extremities were associated. Without a codified etiological treatment, a psychotherapist support, a genetic counseling, a regular pediatric follow-up, a quarterly odontostomatological and ophthalmological follow- up have been recommended