Retinoblastoma in the Democratic Republic of Congo: 20-Year Review from a Tertiary Hospital in Kinshasa

Background. To determine clinical profile and management of retinoblastoma among children at Kinshasa in Democratic Republic of Congo. Patients and methods. The medical records of patients with a diagnosis of retinoblastoma seen at the University Hospital of Kinshasa from January 1985 till December 2005 were retrospectively reviewed. Demographic profile, clinical data, modes of treatment and outcome were analysed. Results. A total of 49 children, of whom 40 had adequate data on record were identified as retinoblastoma (28 males and 12 females). Nine cases had bilateral disease. The median age at the first symptoms was 9 months (range, 1 month to 6 years) for unilateral retinoblastoma and 18 months (range, 1 month to 3.5 years) for bilateral retinoblastoma. The median age at the first oncology consultation was 2.4 years (range, 6 months to 6 years) for unilateral retinoblastoma and 2.4years (range, 9 months to 4 years) for bilateral disease. Leukokoria was present in 67.5% of subjects. Seventy-five percent abandoned the treatment. The mortality was 92.5%. Conclusion. In Democratic Republic of Congo, retinoblastoma remains a life threatening disease characterized by late referral to a specialized unit and affordability of chemotherapy; all leading to an extension of the disease and high mortality

Factors Associated with Growth Retardation in Children Suffering from Sickle Cell Anemia: First Report from Central Africa

Background. The aim of this study was to investigate and determine the risk factors associated with poor growth among SCA children. Methods. A cross-sectional study was conducted in Kinshasa, the capital’s country. The nutritional status was assessed using the Z scores of the anthropometric indices. Results. We gathered data on the 256 patients, 138 females (53.9%), who entered the study. The mean age at presentation was 8.4 ± 4.9 years of age. Underweight, stunting, and wasting were found, respectively, in 47.7%, 10.5%, and 50.3% of SCA children. A history of hand-foot syndrome, more than 3 blood transfusions, being less than 12 months of age when receiving the first transfusion, more than two severe sickle crises per year, a medical history of severe infections, and the presence of hepatomegaly were associated with poor growth. When comparing sickle cell patients under 12 years of age (n=159) to a group of 296 age-matched children with normal Hb-AA, a significantly higher proportion of subjects with stunting and underweight were found among SCA. Conclusion. Nutritional status encountered in Congolese sickle cell children has been described for the first time in this study. A high prevalence of poor growth in SCA children was found in our study

Acute crises and complications of sickle cell anemia among patients attending a pediatric tertiary unit in Kinshasa, Democratic Republic of Congo

In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years’ retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1-24 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8-250 months). The median age at the first transfusion was 36 months (range 4-168). In this series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources deficiencies

Pathologie carieuse et facteurs de risque associés chez les enfants drépanocytaires à Kinshasa, RD Congo

OBJECTIF: Évaluer l’influence des facteurs de risque associés à la pathologie carieuse au sein d’une population d’enfants drépano-cytaires. — MÉTHODES: Une étude descriptive et multicentrique a été menée auprès des enfants homozygotes SS. Les paramètres d’étude étaient: sexe, âge, fréquence, moment et matériel de brossage dentaire, grignotage d’aliments sucrés, visites chez le dentiste, le nombre de crises vaso-occlusives par an. Le test de khi-carré a permis de comparer ces différents paramètres d’étude entre le groupe des enfants avec au moins une carie et celui sans carie. — RÉSULTATS: Au total, 194 enfants drépanocytaires, âgés de 1,3 à 16,9 ans, ont été examinés. Aucune différence significative n’a été établie quant au sexe, à l’âge, à la fréquence, au moment et au matériel de brossage, aux visites chez le dentiste, au nombre de cvo/an entre les enfants avec au moins une carie et ceux sans carie dentaire. Il s’est établi une différence significative entre les enfants avec au moins une carie et ceux sans carie en ce qui concerne le niveau d’hygiène bucco-dentaire (PI) et le grignotage d’aliments sucrés avec respectivement p = 0,001 et p = 0,02. — CONCLUSION: La présente étude révèle clairement qu’un peu plus de la moitié de la population souffre de la pathologie carieuse et que les facteurs de risque associés comme une hygiène bucco-dentaire insuffisante et un grignotage d’aliments sucrés doivent être minimisés par des campagnes de sensibilisation afin de limiter les facteurs de co morbidité à leur état de santé déjà affaibli par la drépanocytose.[Tooth decay and associated risk factors amongst children with sickle cell anemia in Kinshasa, DR Congo] OBJECTIVE: To evaluate the influence of the risk factors associated with tooth decay within a population of children suffering from sickle cell anemia. — METHODS: A descriptive and multi focal study was conducted amongst homozygous children SS. The parameters of study were: sex, age, frequency, moment and equipment of tooth brushing, nibbling of sweetened food, visit to the dentist, number of vaso-occlusive crisis per year. The chi-square test made it possible to compare the various para meters of study between the group of the children with the tooth decay and those without. — RESULTS: As part of the survey, a total of 194 children with age ranging from 1.3 to 16.9 years were examined. The study showed a significant difference between children with tooth decay and children without tooth decay with regard to the level of oral hygiene (PI) and nibbling of sweetened food respectively p = 0.001 and p = 0.02. However, no significant difference was established as for the sex, age, frequency, moment and equipment of brushing, visit to the dentist, number of vaso-occlusive crisis per year between children with less severe tooth decay and children without tooth decay at all. — CONCLUSION: The results of the present study reveal clearly that a little more than half of the population suffers from tooth decay and that the associated risk factors like an insufficient oral hygiene and a nibbling with sweetened food must be minimized through public awareness campaigns in order to limit the factors of comorbidity in the patient’s health already weakened by the sickle cell anemia

Clinical Study Retinoblastoma in the Democratic Republic of Congo: 20-Year Review from a Tertiary Hospital in Kinshasa

Background. To determine clinical profile and management of retinoblastoma among children at Kinshasa in Democratic Republic of Congo. Patients and methods. The medical records of patients with a diagnosis of retinoblastoma seen at the University Hospital of Kinshasa from January 1985 till December 2005 were retrospectively reviewed. Demographic profile, clinical data, modes of treatment and outcome were analysed. Results. A total of 49 children, of whom 40 had adequate data on record were identified as retinoblastoma (28 males and 12 females). Nine cases had bilateral disease. The median age at the first symptoms was 9 months (range, 1 month to 6 years) for unilateral retinoblastoma and 18 months (range, 1 month to 3.5 years) for bilateral retinoblastoma. The median age at the first oncology consultation was 2.4 years (range, 6 months to 6 years) for unilateral retinoblastoma and 2.4years (range, 9 months to 4 years) for bilateral disease. Leukokoria was present in 67.5% of subjects. Seventy-five percent abandoned the treatment. The mortality was 92.5%. Conclusion. In Democratic Republic of Congo, retinoblastoma remains a life threatening disease characterized by late referral to a specialized unit and affordability of chemotherapy; all leading to an extension of the disease and high mortality

Cherubism in sub-Saharan Africa: a first case-report in a child

Cherubism is rare disease and has been rarely reported in African pediatric population. We report here the case of a 10-year-old child who was referred to our hospital for bilateral jaws swelling. Physical examination revealed bilateral swelling symmetry of the face. Histopathological examination of the biopsy specimen showed loose fibrous stroma, proliferating fibrous connective with tissue interspersed with multinucleated giant cells, small thin walled blood vessels and scattered sparse mononuclear inflammatory infiltrate. Our patient presented cherubism. Cherubism is rarely described in children living in sub-Saharan Africa. Genetic and molecular investigations plays an important role in diagnosis but were not available in poor resources settings in developing countries such as the Democratic Republic of Congo

Blood pressure according Hb status and sex.

<p>Blood pressure according Hb status and sex.</p