[Homozygous Sickle Cell Children Treated with Hydroxyurea in Brazzaville (Congo)]. / Le devenir des enfants atteints de drépanocytose homozygote traités par hydroxyurée à Brazzaville (Congo).

The objective of this study is to reduce the morbidity of children with homozygous sickle cell disease presenting severe forms. We have conducted a longitudinal study between November 2015 and September 2017 at the Brazzaville University Hospital. Children with sickle cell disease requiring treatment with hydroxyurea were included. The variables studied were therapeutic compliance, evolutive profile of nutritional status, indications of hydroxyurea, electrophoresis of hemoglobin, blood count, and toxicity. The statistical test used was Student test with a significance threshold of less than 0.05. One thousand twenty-four children were monitored for sickle cell disease, 107 of which had received hydroxyurea (10.4%). The indications for hydroxyurea were recurrent anemic attacks (≥ 4) in 62 cases (57.9%), neurological crises 19 cases (17.8%), recurrent hyperalgesic crises in 17 cases (15.9%), priapism in 4 cases (3.7), and acute thoracic syndrome in 2 cases (1.9%). Therapeutic compliance was good in 89.5% of them. A rapid and lasting clinical improvement was noted in the majority of patients with hyperalgesic attacks (88.2%) and anemic attacks (88.7%), two recurrences for the cerebrovascular accidents, and an absence of recurrence of priapism and of the acute thoracic syndrome. From the biological point of view, there was a significant increase in fetal hemoglobin (1.2 to 16.2%; P  < 0.05), hemoglobin (7 to 8.3 g/dl; P < 0.05), mean cell volume (80.8 to 96 fl; P  < 0.05) and a significant decrease in mean white blood cell count (15,633 to 9,872/mm3; P  < 0.05) and platelets (387,002 to 324,400/mm3; P  < 0.05). The signs of toxicity observed were mainly vomiting and thrombocytopenia in two cases each, one case with headache and the other with neutropenia. Indications for use of hydroxyurea therapy in children with sickle cell disease in Brazzaville are common. These are dominated by recurrent anemic seizures, strokes, and hyperalgesic seizures. The excellent evolution of these complications under hydroxyurea represents an interesting alternative in our countries with limited resources.

Il s'agit d'une étude longitudinale réalisée entre novembre 2015 et septembre 2017 au CHU de Brazzaville. L'étude a porté sur une série hospitalière d'enfants atteints de drépanocytose homozygote et présentant des complications sévères qui imposaient le recours à l'hydroxyurée. Les variables étudiées étaient l'observance thérapeutique, l'évolution de l'état nutritionnel, des indications de l'hydroxyurée, des éléments de l'hémogramme et de l'électrophorèse de l'hémoglobine au cours du traitement, les effets secondaires et les signes de toxicité. La comparaison des moyennes des éléments hématologiques avant le traitement et la fin de l'étude a fait appel au test t de Student, avec un seuil de significativité fixé à 0,05. Sur 1 024 enfants suivis pour drépanocytose, 107 présentaient une indication de traitement par hydroxyurée, soit 10,4 %. Ces indications étaient des épisodes anémiques récurrents (≥ 4) dans 62 cas, des accidents neurovasculaires dans 19 cas, des hyperalgies récurrentes dans 17 cas, un priapisme permanent dans quatre cas et un syndrome thoracique aigu dans deux cas. L'observance thérapeutique était bonne pour 89,5 % des enfants traités. Une amélioration clinique rapide et durable était notée dans la majorité des enfants, avec 88,2 % pour les crises hyperalgiques, et 88,7 % pour les crises anémiques, deux cas de récidives de crise neurovasculaire, mais aucune pour le priapisme et le syndrome thoracique aigu. Sur le plan biologique, on a constaté une augmentation significative du taux de l'hémoglobine fœtale (1,2 à 16,2 % ; p < 0,05), de l'hémoglobine (7 à 8,3 g/dl ; p < 0,05), du volume globulaire moyen (80,8 à 96 fl ; p < 0,05), et une baisse significative du nombre de globules blancs (15 633 à 9 872/mm3 ; p < 0,05) et des plaquettes (387 002 à 324 400/mm3 ; p < 0,05). Les effets secondaires observés étaient essentiellement des vomissements et une thrombopénie dans deux cas chacun, ainsi qu'un cas de céphalée et une neutropénie. Les indications de traitement par hydroxyurée chez l'enfant atteint de drépanocytose à Brazzaville sont nombreuses. Celles-ci sont dominées par les épisodes anémiques récurrents, les accidents vasculaires cérébraux et les crises hyperalgiques. L'excellente évolution de ces complications sous hydroxyurée représente une alternative intéressante dans nos pays à ressources limitées.

[Evaluation of the Follow-Up and State of Adolescents with Sickle-Cell Disease in Brazzaville (Congo)]. / Suivi et état de santé de l'adolescent atteint de drépanocytose à Brazzaville (Congo).

The experience of the adolescent with homozygous sickle-cell disease is influenced by several factors that differentiate it from the older child entirely dependent on his parents. The objective of this study was to describe the state of health and to assess the quality of follow-up and to identify the determinants of poor monitoring of adolescent sickle-cell followed in Brazzaville for an average of 12 ±â€…9.6 years, starting from a cross-sectional study carried out at the Brazzaville University Hospital from March to September 2016. It is based on a questionnaire composed of elements of assessment of the state of health and the quality of the follow-up. The vaccination coverage of adolescents was low, 81.3% for DTCP, 66.5% for Typhim Vi, 50.2% for viral hepatitis B, 76.4% for pneumococcus, and 59.1% for the ROR. In the last two years prior to the survey, 99 (48.7%) adolescents had only 2 follow-up visits instead of 4 planned per year. Therapeutic compliance was good in 132 (65%). No hospitalizations were reported during this period in 23 adolescents (11.3%); in 180 cases (88.7%), however, adolescents were hospitalized one to three times apart from regular follow-up visits. Since the discovery of the disease, 177 (87.2%) adolescents had already been transfused, more than three times in 89 cases. A history of neurovascular seizures was found in 10 cases (5.2%) and priapism in 35 cases (18.2%). Paraclinical examinations were not systematic during follow-up visits. The socioeconomic level of the family and the level of education of the father had a negative impact on monitoring and adherence (P < 0.01). On clinical examinations, stunting, undernutrition, pubertal delay, tooth decay, enuresis were found in 45.3%, 36%, 53.7%, 27.6%, 15.3%, respectively. The biological examinations carried out during the investigation showed an average inter-critical hemoglobin level between 7 and 8 g/dl, creatinine level was normal in all cases, ferritinemia was elevated in 93.6%, a negative proteinuria was found in 71.4% of the cases, and hematuria in 26.6%. Systematic abdominal ultrasound revealed vesicular lithiasis in 8 cases, hepatomegaly in 10 cases, and splenomegaly in 102 cases. Echocardiography performed in all subjects showed cardiomyopathy in 9 cases. The follow-up of the adolescent sickle-cell in Brazzaville still faces enormous difficulties. The improvement of the standard of living, the therapeutic education and the introduction of a total free of charge of the global management of sickle-cell disease would make it possible to minimize these difficulties which also would improve the future of these teenagers, adults of tomorrow.

Le vécu de l'adolescent atteint de drépanocytose homozygote est influencé par plusieurs facteurs qui le différencient de l'enfant moins âgé entièrement dépendant de ses parents. L'objectif de cette étude était de décrire l'état de santé, d'apprécier la qualité du suivi et d'identifier les déterminants de mauvais suivi de l'adolescent drépanocytaire suivi à Brazzaville depuis en moyenne 12 ±â€…9,6 ans, à partir d'une étude transversale réalisée au CHU de Brazzaville de mars à septembre 2016. Elle était fondée sur un questionnaire composé des éléments d'appréciation de l'état de santé et de la qualité du suivi. La couverture vaccinale des adolescents était faible : 81,3 % pour le DTCP, 66,5 % pour le Typhim Vi, 50,2 % pour l'hépatite virale B, 76,4 % pour le pneumocoque et 59,1 % pour le ROR. Au cours des deux dernières années ayant précédé l'enquête, 99 (48,7 %) adolescents avaient seulement deux consultations de suivi au lieu des quatre prévues par an. L'observance thérapeutique était bonne dans 132 cas (65 %). Aucune hospitalisation n'a été signalée pendant cette période chez 23 adolescents (11,3 %) ; par contre, dans 180 cas (88,7 %), les adolescents étaient hospitalisés une à trois fois en dehors des consultations régulières de suivi. Depuis la découverte de la maladie, 177 (87,2 %) adolescents avaient déjà été transfusés, plus de trois fois dans 89 cas. Les antécédents de crises neurovasculaires étaient retrouvés dans dix cas (5,2 %) et de priapisme dans 35 cas (18,2 %). Les examens paracliniques n'étaient pas systématiques au cours des visites de suivi. Les faibles niveaux socio-économiques de la famille et d'instruction du père impactaient négativement le suivi et l'observance thérapeutique (p ˂ 0,01). À l'examen clinique, un retard statural, une dénutrition, un retard pubertaire, une carie dentaire et une énurésie ont été retrouvés respectivement dans 45,3, 36, 53,7, 27,6 et 15,3 %. Les examens biologiques réalisés pendant l'enquête montraient un taux d'hémoglobine intercritique moyen compris entre 7 et 8 g/dl, une créatininémie normale dans tous les cas, la ferritinémie élevée dans 93,6 %, une protéinurie négative dans 71,4 %, une hématurie dans 26,6 %. L'échographie abdominale systématique révélait une lithiase vésiculaire dans huit cas, une hépatomégalie dans dix cas et une splénomégalie dans 102 cas. L'échocardiographie réalisée chez tous les sujets objectivait une cardiomyopathie dans neuf cas. Le suivi de l'adolescent drépanocytaire à Brazzaville est encore confronté à d'énormes difficultés. L'amélioration du niveau de vie, l'éducation thérapeutique et l'instauration d'une gratuité totale de la prise en charge globale de la maladie drépanocytaire permettraient de minimiser ces difficultés, ce qui améliorerait l'avenir de ces adolescents, adultes de demain.

[Quality of Life of Adolescents with Homozygous Sickle-Cell Disease Managed at the Brazzaville Teaching Hospital (Congo)]. / La qualité de vie des adolescents atteints de drépanocytose homozygote suivis au CHU de Brazzaville (Congo).

Adolescence is a particular age group in terms of psychological and somatic changes. Thus, the experience of a chronic disease such as sickle-cell disease is different compared to the younger child who depends entirely on his parents. The purpose of this study is to assess the quality of life and to identify the determinants of good quality of life of such teenagers. A cross-sectional study of adolescents living with sickle-cell disease has been carried out at the Teaching Hospital of Brazzaville, from March to September 2016. It was based on a questionnaire composed of elements which assess the quality of life according to Short-Form 12 score. The mean overall score for quality of life was 52.3, with 47.7 for the physical component and 56.9 for the mental component. The quality of life was better among adolescents with normal schooling (P ˂ 0.02), normal puberty (P ˂ 0.002), and in non-orphans (P ˂ 0.002), and in those who had not yet received blood transfusion (P ˂ 0.0001) since birth or hospitalized during the last two years prior to the survey (P ˂ 0.0001). The quality of life of adolescents with sickle-cell disease in Brazzaville is still facing many difficulties. The improvement of purchasing power, communication for behavioral change, and the introduction of free global care for people living with sickle-cell disease would minimize these difficulties. This would improve the future of these adolescents, coming adults of tomorrow.

Il s'agit ici d'apprécier la qualité de vie (QdV) de l'adolescent drépanocytaire homozygote à Brazzaville et d'identifier les déterminants d'une bonne QdV à travers une étude transversale portant sur les adolescents drépanocytaires, réalisée au CHU de Brazzaville de mars à septembre 2016. Elle était fondée sur un questionnaire composé des éléments d'appréciation de la QdV selon le test SF-12 (12-Item Short-Form Health Survey). Le score moyen global de QdV était de 52,3 avec un score moyen de 47,7 pour la composante physique et de 56,9 pour la composante mentale. La QdV physique était bonne chez les adolescents ayant une scolarité normale (p ˂ 0,02), une puberté normale (p ˂ 0,002) et un statut social de non-orphelin (p ˂ 0,002), et chez ceux qui n'avaient jamais été transfusés (p ˂ 0,0001) depuis la naissance ni hospitalisés au cours des deux dernières années ayant précédé l'enquête (p ˂ 0,0001). La QdV de l'adolescent drépanocytaire homozygote suivi à Brazzaville est plus faible que celle du sujet normal. L'amélioration du pouvoir d'achat, la communication pour le changement de comportement et l'instauration d'une gratuité de la prise en charge globale de la maladie drépanocytaire permettraient de minimiser ces difficultés. Ce qui améliorerait l'avenir de ces adolescents, futurs adultes de demain.

Outcome of HIV-positive children and adolescents treated with second-line antiretroviral agents in Congo.

Antiretroviral agents (ARVs) are the leading therapeutic weapon against HIV/AIDS infection. When first-line treatment fails, a second-line ARV is needed. OBJECTIVE: To determine the prevalence of HIV-infected children treated with second-line ARVs, to assess their adherence and therapeutic response, and to identify factors of good adherence. MATERIAL AND METHODS: A retrospective multicenter study covering the period from January 2015 to July 2016 in all centers providing care to children with HIV included all children treated with a second-line ARV. RESULTS: A total of 71 children were identified, with a male to female ratio of 1.1 and a mean age of 14.2±3.2 years (range: 5 to 19). Mother-to-child transmission was observed for 97.2 %. In all, 64.8 % of the children had lost at least one parent, 29.6 % both of them. Family socioeconomic status was low for 15.5 % and middle for 74.6 %. Nearly half (46.5 %) were aware of their HIV status, and all received psychological support. The therapeutic regimen used for 54.9 % included ABC + DDI + LPV/r, and the treatment lasted for less than 2 years in 22.5 % and between 2 and 4 years in 38 %. Therapeutic adherence was good in 54.9 %, better in boys (p < 0.01) and in those aged 15 years and younger (p < 0.001). The therapeutic response was good in 89 % at 12 months and 86 % at 4 years, better in those aged 15 years and younger (p < 0.001). CONCLUSION: Despite the small proportion of good therapeutic adherence to second-line ARVs, the rate of good clinical and therapeutic response of 86 % in children and adolescents 4 years after being switched to them is an argument that should encourage prescribers to shift patients to second-line treatment as soon as there is an indication. However, challenges remain in improving the management of children and adolescents treated with a second-line ART.

[Nephrotic syndrome complicated by chylous ascites in a girl of 2 years and 8 months old]. / Syndrome néphrotique compliqué d'ascite chyleuse chez une fillette de 2 ans et 8 mois.

We report on a case of nephrotic syndrome with focal and segmental hyalinosis complicated by chylous ascites in a girl of 2 years and 8 months old. This pure nephrotic syndrome in its early stage was initially treated with intensive steroid treatment at 2mg/kg/day orally for 2 months, followed by a bolus of methylprednisolone. The persistence of proteinuria meant corticosteroid resistance. Renal biopsy then revealed focal and segmental hyalinosis. A recurrence of the edema-ascites syndrome was associated with macroscopic hematuria. Blood pressure and serum creatinine remained normal. Protidemia decreased to 28g/L, with severe hypoalbuminemia at 7g/L. The ascites puncture brought 1 L of aseptic and milky fluid, containing 0.22g/L of proteins, 20 IU/L of amylase, and 331g/L of total lipids. The treatment protocol included a hyperprotein diet, prednisone at 0.5mg/kg/day, cyclophosphamide at 2.5mg/kg/day for 1 month, then once every 2 days for 3 months, and repeated ascites punctures. After 12 ascites punctures performed every 15 days, a polyuric episode occurred and the ascites disappeared. Proteinuria persisted at a nephrotic rate up to the 14th month, without impairment in kidney filtration function, and completely disappeared at the 20th month. After 5 years of follow-up, proteinuria remains undetectable and the physical exam is normal. The occurrence of chylous ascites during the nephrotic syndrome is a rare event. The formation of bowel lymphangiectasia, caused by a slowdown in venous return due to the pressure exerted by ascites, is probably the predominant mechanism.

[Psychosocial issues of mothers whose children have sickle cell disease]. / Vécu psychosocial des mères d'enfants drépanocytaires.

OBJECTIVES: To study and describe the psychological responses (depression and anxiety), the coping strategies, and the impact of sickle cell disease on the quality of life (QOL) of mothers. PATIENTS AND METHODS: A cross-sectional study of mothers with affected children was conducted at the university hospital center of Brazzaville, between February and April 2014. Hamilton and Beck scales, the Brief-COPE, and the Short Form Health Survey were used to assess anxiety, depression, coping strategies, and QOL, respectively. RESULTS: In all, 104 mothers of children with sickle cell disease were surveyed. Of the subjects, 73 (70.2%) had depression and 85 (81.7%) anxiety, while 19 (18.3%) mothers were neither anxious nor depressed. The study shows that depression occurred more often among mothers whose children presented with at least three vaso-occlusive crises (CVO) requiring hospitalization per year (OR=5.1; range=1.8-13.9), at least one blood transfusion (OR=6; range=2.3-15.5), and those whose disease had been known for at least 5 years (OR=3; range=1.2-7.2). On the contrary, maternal anxiety was influenced only by the number of transfusions and CVO requiring hospitalization. The main coping strategy was religious coping (65.4%) followed by acceptance (60%). The QOL of mothers with affected children was impaired in 56.7% of cases. Altered QOL was related to the number of hospitalized CVO per year, i.e., equal to or greater than 3 (OR=6.5; range=2.1-19.6 [P<0.01]), and the number of blood transfusions equal to or greater than 1 (OR=2.9; range=1.2-7.3 [P<0.025]). CONCLUSION: Sickle cell disease is a heavy burden for mothers caring for these children. Mental health disorders are present at the time of diagnosis and in everyday life.

[Epidemiology and outcome of Congolese children who had surgery for heart defects]. / Épidémiologie et devenir des enfants congolais opérés pour cardiopathies congénitales.

AIMS: to determine the principal heart defects for which children underwent surgery and to determine the survival rate. PATIENT AND METHODS: this retrospective cohort study involves Congolese babies treated surgically from September 1989 to September 2010 in France for congenital heart defects (through "Mécénat chirurgie cardiaque" and "Chaîne de l'espoir"). It includes only 110 of the 182 recorded patients during the study period. RESULTS: The sex ratio for the 110 subjects included in the analysis was 1. Their mean age at surgery was 77.4 ± 57.6 months old (range: 8 to 204 months). The main congenital heart defects for which surgery was performed were ventricular septal defect (21.9%), tetralogy of Fallot either isolated (22.8%) or associated with patent foramen ovale (1.8%) or coronary anomalies (1.8%), atrial septal defect associated with other malformations (8.2%), pulmonary atresia with ventricular septal defect (5.5%), aortic stenosis (3.7%), atrioventricular septal defect (0.9%), and Laubry-Pezzi syndrome (0.9%). The median length of follow-up was 42.4 ± 35.6 months (range, 3-240 months). Patients' mean age at the study's end was 121.1 ± 86.3 months (range 20-372 months). The 5-year survival rate was 90% and the 20-year survival, 83.3%. CONCLUSION: Heart surgery for congenital heart defects has improved survival.

[Discharge of children against medical advice at CHU of Brazzaville (Congo)]. / Les sorties contre avis médical en pédiatrie au CHU de Brazzaville (Congo).

In a prospective study conducted from January to December 2010 in the Teaching Hospital of Brazzaville (Congo), we compared the discharge of hospitalized children against medical advice at the parents' request (group 1 - cases) with those admitted during same period with routine discharge (group 2 - controls). Two hundred and seven (7.7%) hospitalized children, 117 boys and 90 girls, were discharged at the parents request. The mean age was 18.0 ± 13.5 months (range: 1 month-5 years). Among these patients, 150 (72.5%) children were from biparental families, and 30.9% of children were the first and/or only child of the household. Among the parents of Congolese nationality, 147 (71%) had a secondary school level education, including 66.7% of mothers and 58% of the fathers 59.9% of children were admitted between 15 and 7 hours, and 75.4% were hospitalized during working days. The duration of hospitalization did not exceed 3 days in 147 cases (71%). The main reasons for hospitalization were digestive disorders (27%), fever (16.4%), convulsions (11.1%) and anaemia (11.1%). Among patients discharged against medical advice, 34.8% left on the first day of hospitalization (72 cases), 36.7% before the start of treatment (76 cases of which 36 during check-up) and 63.3% during treatment (131 cases). The main reasons for discharge request were improvement in the clinical condition improvement (30.9% of cases), lack of money (28% of cases), supposed insufficient medical care (7.7% of cases), unsatisfactory hospitalization conditions (6.3% of cases), care of other children at home (6.3% of cases), traditional and/or religious beliefs (5.8% of cases) and disagreements with nurses (5.3%). Patients discharge against medical advice was correlated strongly with the educational level of parents, age of the child, delay of consultation, time of admission, rank of the child in family and nationality of parents. Discharge against medical advice is associated to the distrust between the patients and nurses. Improvement of hospitalization conditions in our hospitals, improvement of the socioeconomic status of Congolese and establishment of national social security seem to be the main determinants of reduction of discharge against medical advice.