The Destombes-Rosai-Dorfman Disease or Sinus Histiocytosis with Massive Lymphadenopathy: A Rare Case Described in the Paediatrics Department of Yalgado Ouedraogo University Hospital

Destombes-Rosai-Dorfman’ disease (DRD) is an inflammatory non-langerhansian histiocytosis with adenomegaly. It is a rare histiocytic disorder worldwide, less than 1000 reported cases. It is of unknown etiology and is characterized in its classical form by multiple adenomegalia, especially localized at cervical area, but also at mediastinal, axillary and inguinal areas. It is a benign condition, even if it is deforming, with spontaneously resolving evolution. We report a case of DRD disease in a 13-year-old girl, hospitalized in the Department of Pediatrics of the Yalgado Ouédraogo University Hospital Center in Ouagadougou, in March 2015. She was admitted for a voluminous bilateral painless cervical swelling, with no sign of local compression, having been operating for about a year. Biological tests showed signs of chronic inflammation;the imagery specified the benign character of these formations: adenomegalia. Confirmation of the diagnosis was made by pathological examination, describing the aspect of emperipolesis to histology and, immunohistochemistry, polytypic plasmacytosis and PS100 positive/CD1a histiocytosis negative. The treatment could not be properly carried out, in view of the early discharge, against medical advice from the patient. The evolution would have been marked, according to the parents, by a slight involution of swelling.

Characterization of two rat models of cystic fibrosis—KO and F508del CFTR—Generated by Crispr-Cas9

Background: Genetically engineered animals are essential for gaining a proper understanding of the disease mechanisms of cystic fibrosis(CF). The rat is a relevant laboratory model for CF because of its zootechnical capacity, size, and airway characteristics, including the presence of submucosal glands.Methods: We describe the generation of a CF rat model(F508 del) homozygous for the p.Phe508 del mutation in the transmembrane conductance regulator(Cftr) gene. This model was compared to new Cftr-/-rats(CFTR KO). Target organs in CF were examined by histological staining of tissue sections and tooth enamel was quantified by micro-computed tomography. The activity of CFTR was evaluated by nasal potential difference(NPD) and short-circuit current measurements. The effect of VX-809 and VX-770 was analyzed on nasal epithelial primary cell cultures from F508 del rats.Results: Both newborn F508 del and Knock out(KO) animals developed intestinal obstruction that could be partly compensated by special diet combined with an osmotic laxative. The two rat models exhibited CF phenotypic anomalies such as vas deferens agenesis and tooth enamel defects. Histology of the intestine, pancreas, liver, and lungs was normal. Absence of CFTR function in KO rats was confirmed ex vivo by short-circuit current measurements on colon mucosae and in vivo by NPD, whereas residual CFTR activity was observed in F508 del rats. Exposure of F508 del CFTR nasal primary cultures to a combination of VX-809 and VX-770 improved CFTR-mediated Cl-transport.Conclusions: The F508 del rats reproduce the phenotypes observed in CFTR KO animals and represent a novel resource to advance the development of CF therapeutics.

The Difficulties of Congenital Syphilis Diagnosis about 3 Cases at Libreville, Gabon

First described embryo fetopathy, congenital syphilis remains a public health problem mostly in developing countries. The diagnosis mainly based on bacteriological and immunological evidence of mother-child couple is not always easy, as it is shown in our three clinical cases. Those three clinical observations demonstrate the difficulties encountered in the diagnosis of congenital syphilis in our country where only the TPHA (Treponema Pallidum Haemaglutination Assay) and VDRL (Venereal Disease Research Laboratory) tests are the only ones to be routinely carried out. Actually, these tests can be negative at the earliest stage of the syphilis or in case of zonal phenomenon. In addition, maternal antibodies could be found in child blood, even if the baby is in good health. At last, the child could have been contaminated belatedly while tests were negative at the third month of pregnancy. Congenital syphilis still exists in our developing countries and, in order to better manage this pathology, a proposition of an efficient algorithm is submitted.

Management and Development of Newborns from Mothers Carrying HBsAg at the El Rapha Polyclinic in Libreville, Gabon

Introduction: Hepatitis B is a significant public health problem. Infected children <6 years represent a risk population of evolution to chronicity. Objective: We aimed to assess newborns’ management and outcome from reactive HBs Ag mothers at the El Rapha Polyclinic in Libreville, Gabon. Methodology: Prospective longitudinal study, carried out over four years, including all neonates born from reactive HBsAg mothers admitted in the delivery room. The parameters studied were maternal age, gestation, marital status, pregnancy follow-up, hepatitis B vaccination status, knowledge of hepatitis B, anti-Hbc antibodies’ levels, HBeAg. For the newborn: term, birth weight, management at birth, and hepatitis B serological status at 12 months. Results: We included 45 newborns. Mothers’ mean age was 30.9 ± seven years. In 32.6% of cases, antenatal consultations were ≤4, and 9.3% were vaccinated for hepatitis B. Mothers had chronic hepatitis in 74.4% of cases. All neonates were vaccinated for hepatitis B at birth;68.9% received hepatitis B immunoglobulin. At 12 months, 77.8% had HBs antibody positive, and 6.7% were HBsAg positive. Conclusion: Hepatitis B prevalence is significant among parturients. It is essential to set up a national strategy for managing the mother-child couple in a context of positive HBsAg.

Completeness and Timeliness of Hepatitis B Vaccination in Preschool Children: Determinants for Good and Timely Uptake in Libreville, Gabon

Background: Gabon is endemic for hepatitis B, but they have still not reached the WHO goal for Hepatitis B vaccination coverage. We aimed to determine the rate of completeness and timeliness of hepatitis B vaccination among children under 15 years of age in Libreville. Methods: Cross-sectional study conducted over 9 months, including children randomly selected from vaccination centres, nurseries and kindergartens. We collected data on the child, socio-economic characteristics of the families, and dates of administration of hepatitis B vaccines. We determined the compliance of the vaccination dates according to the vaccination start pattern. Results: We included 453 children, of whom 236 were girls, for a sex ratio of 0.92. A total of 87% (95% CI [83.9% - 90.1%]) of children had started and completed their 3 doses of vaccine before the 12th month of age. 149 subjects had received their 3 doses on time according to the original vaccination schedule, i.e. 32.9% (95% CI [28.6% - 37.2%]). Factors significantly associated with completeness were vaccination in a public centre OR = 114 [47.2 - 347] p st dose OR = 3 [1.6 - 5.5] p < 0.001. Age at first dose was the predictor of timeliness aOR = 1.3 (95% CI [1.2 - 1.5] p 0.001). Conclusion: The respect for the vaccination deadlines is insufficient in our context, solutions exist to improve this situation.

Ulcerative Colitis in Infant: A Case Report at the University Hospital Yalgado Ouedraogo, Ouagadougou in Burkina Faso

Ulcerative colitis (UC) is a chronic inflammatory bowel disease (IBD), characterized by acute exacerbations and remissions. We report the clinical case of an infant, an 8 months female baby. The time between disease onset and diagnosis of the disease was 6 months. It was from an unknown etiology. The main clinical picture was weight stagnation, mucoid bloody diarrhea, and rectal bleeding. The macroscopic aspect in anorectoscopy was an erythematous mucosa with healthy beaches intervals. The mucosa was bleeding easily on contact. Pathological examination realized within the various lesions of the association was highly suggestive. The suggested treatment was made of symptomatic, corticosteroids and immunosuppressant’s therapy. Conclusion: Ulcerative colitis is rare in infants and difficult to manage in our tropical context.

Plasma 17-hydroxyprogesterone/cortisol ratio is not a predictor of systemic hypotension in extremely premature infants

Background/aims: To determine whether the 17-hydroxyprogesterone (17-OHP)/cortisol ratio as a marker of immature11-beta hydroxylase activity can predict severe systemic hypotension in preterm neonates. Methods: Serum cortisol and 17-OHP concentrations were measured in capillary blood deposited on blotter paper on day 3 post-natal age (Day 3) in infants less than 32 weeks postmenstrual age (PMA). The predictive value of 17-OHP/cortisol ratio for a first episode of systemic hypotension occurring after Day 3 (FESH) was evaluated. Results: Of 105 infants included, 14 patients (13%) presented a FESH. Neither the 17-OHP/cortisol ratio, nor the 17-OHP or cortisol concentrations were associated with the occurrence of a FESH when adjusted for potential confounding factors. 17-OHP and cortisol were inversely associated to PMA (r = ﹣0.36 and ﹣0.40, respectively). Cortisol, but not 17-OHP, was associated with the type of hospitalization unit, the respiratory support and the presence of a patent ductus arteriosus. The 17-OHP/cortisol ratio was associated with the type of hospitalization unit only. Conclusions: The 17-OHP/ cortisol ratio at Day 3 did not predict the occurrence of a first episode of systemic hypotension after Day3 inpreterm neonates.

Ocular Manifestations among HIV Infected Children in Ouagadougou, Burkina Faso

Ocular manifestations among HIV infected children are diverse and global incidence varies from 7% to 75%. At this age, eye lesions are often unnoticed because of the incapacity to express eye discomfort. The purpose of this study is to describe ocular manifestations among HIV-infected children and hence associated factors in the Department of Paediatrics at the Yalgado Ouédraogo Teaching Hospital. This was a cross-sectional descriptive and analytical study conducted between July 2014 and December 2014. A complete ophthalmic examination was systematically done to all HIV-positive children attending the clinic, as part of their routine medical visit. The most recent socio-demographic, clinical, biological and treatment data were registered. Seventy-nine children had an ocular examination and among them 92.4% were on ARV treatment. The incidence of ocular manifestations was 46.7%. Median age was 8 years old (interquartile 6 - 12 years old). Sex ratio was 1.3. The risk of ocular manifestations involvement among boys was twice than that of girls. More than half (59.5%) of children who had ocular problems had not expressed ocular discomfort. Ocular adnexal lesions were more common (35.4%) compared to eye segments (8.9%) lesions. Anterior segment and posterior segment lesions were statistically associated with immune system depression (p = 0.003 and 0.001). However, this relationship was not statistically significant (p = 0.15). Five out of seven children who had eye fundus lesions had CD4 count 3. Ocular manifestations were very common among HIV infected children in our context. Ophthalmic examination should be systematic at admission and regularly repeated during follow-up.

儿童巨淀粉酶血症1例

We report a case of macroamylasemia in an 11-year-old boy. We compare our clinical and paraclinical data with those described in pediatric literature. Macroamylase resulted in a complex of amylase and immuglobulin. Its fortuitous detection did not reveal,up to now,any associated pathology,in particular any autoimmune disorders or celiac disease. Identification of this biochemical abnormality is essential in order to avoid invasive investigations and/or unnecessary therapies.