The Iron Status of Children with and without Sickle Cell Disease at the University of Ilorin Teaching Hospital, Nigeria.

BACKGROUND: Children with sickle cell disease (SCD) are at potential risk of iron overload from chronic transfusion and probable iron deficiency due to accelerated growth. However, only few studies on the iron status of children with SCD in Nigeria are available. METHODOLOGY: A cross-sectional study compared the iron status of 109 children with sickle cell disease with 109 age and sex-matched haemoglobin AA controls at the University of Ilorin Teaching Hospital. Parameters assayed were serum iron, ferritin, transferrin, and haemoglobin (Hb) concentrations. Considering the appropriate reference values for age and sex, these parameters were used to classify the children into high, normal, or low iron status. RESULTS: The median (interquartile range) serum ferritin level of 180.00 (237.50)ng/ml for the SCD subjects was significantly higher than 70.00 (120.00)ng/ml observed among controls, but the mean Hb and median serum transferrin levels were significantly lower in the subjects compared with the corresponding values in the controls, each showing statistical significance (p<0.05). The median serum iron levels did not differ significantly between the SCD (112µg/dl) and non-SCD (128µg/dl), p=0.309. A high proportion of subjects had low HB status (96.3%) compared with controls (56.9%), p=0.001. A significantly higher proportion of subjects (78%) had high ferritin status compared with the controls (48.3%; p <0.001). Ten (9.1%) SCD children had low serum iron status compared to 28 (25.7%) HbAA controls, p=0.002. Thirty-four (31.2) subjects had low transferrin status which was significantly higher than the corresponding number of controls (8;7.3%; p<0.001). CONCLUSION: The children with SCD in the index study were iron-sufficient.

CONTEXTE: Les enfants atteints de la drépanocytose (SCD) sont potentiellement exposés à un excès de fer dû aux transfusions chroniques et à une éventuelle carence en fer due à la croissance accélérée. Cependant, seulement quelques études sur l'état du fer chez les enfants atteints de la SCD au Nigéria sont disponibles. MÉTHODE: Une étude transversale a comparé l'état du fer de 109 enfants atteints de la drépanocytose à celui de 109 témoins de même âge et de même sexe porteurs d'hémoglobine AA à l'hôpital universitaire d'Ilorin. Les paramètres analysés étaient les concentrations de fer sérique, de ferritine, de transferrine et d'hémoglobine (Hb). En utilisant les valeurs de référence appropriées pour l'âge et le sexe, ces paramètres ont été utilisés pour classer les enfants en fonction de leur statut en fer, à savoir élevé, normal ou faible. RÉSULTATS: Le niveau médian (plage interquartile) de ferritine sérique de 180,00 (237,50) ng/ml chez les sujets atteints de SCD était significativement plus élevé que les 70,00 (120,00) ng/ml observés chez les témoins, mais la concentration moyenne en Hb et le niveau médian de transferrine sérique étaient significativement plus bas chez les sujets par rapport aux valeurs correspondantes chez les témoins, chaque différence étant statistiquement significative (p<0,05). Les niveaux médians de fer sérique ne différaient pas de manière significative entre les sujets atteints de SCD (112 µg/dl) et les témoins non atteints de SCD (128 µg/dl), p=0,309. Une proportion élevée de sujets présentait un faible statut en Hb (96,3 %) par rapport aux témoins (56,9 %), p=0,001. Une proportion significativement plus élevée de sujets (78 %) avait un statut élevé en ferritine par rapport aux témoins (48,3 % ; p <0,001). Dix (9,1 %) enfants atteints de SCD avaient un faible statut en fer sérique par rapport à 28 (25,7%) témoins HbAA, p=0,002. Trente-quatre (31,2 %) des sujets avaient un faible statut en transferrine, ce qui était significativement plus élevé que le nombre correspondant de témoins (8 ; 7,3 % ; p<0,001). CONCLUSION: Les enfants atteints de la SCD dans cette étude étaient suffisamment approvisionnés en fer.

Disseminated Tuberculosis in a Nigerian Adolescent with Linear IgA Bullous Dermatosis: A Case Report and Review of Literature.

Linear IgA bullous dermatosis (LABD) is an auto-immune disease affecting young children and adults, characterized by the linear deposition of IgA at the basement membrane zone with resultant complement activation and a cascade of immune reactions. There is a loss of adhesion at the dermo-epidermal junction and subsequent blister formation. It is a rare disease that has a good prognosis with adequate therapy. However, the underlying depressed immunity associated with the disease may expose them to such infections as tuberculosis. We report the case of an 11-years-old Nigerian female adolescent with LABD, diagnosed at the age of four years but defaulted on follow-up, who developed disseminated tuberculosis (pulmonary, lymph nodes, abdominal and pericardial effusion) seven years after the appearance of the initial blistering skin lesions. She commenced anti-tuberculosis drugs, steroids, and a tube pericardiostomy for the pericardial effusion. Dapsone was initiated for the LABD during the continuation phase of anti-tuberculosis therapy, with subsequent disappearance of the skin rash within two weeks.

La dermatose bulleuse linéaire à IgA (DBL) est une maladie auto-immune affectant les jeunes enfants et les adultes, caractérisée par le dépôt linéaire d'IgA dans la zone de la membrane basale, avec l'activation du complément qui en résulte et une cascade de réactions immunitaires. Il y a une perte d'adhérence à la jonction dermo-épidermique et une formation ultérieure de vésicules. C'est une maladie rare qui a un bon pronostic avec un traitement adéquat. Cependant, l'immunité déprimée sous-jacente associée à la maladie peut les exposer à des infections telles que la tuberculose. Nous rapportons le cas d'une adolescente nigériane de 11 ans atteinte de la LABD, diagnostiquée à l'âge de quatre ans mais en défaut de suivi, qui a développé une tuberculose disséminée (pulmonaire, ganglions lymphatiques, épanchement abdominal et péricardique) sept ans après l'apparition des lésions cutanées vésiculeuses initiales. Elle a commencé à recevoir des médicaments antituberculeux, des stéroïdes et une péricardiostomie par sonde pour l'épanchement péricardique. La dapsone a été initiée pour la DLB pendant la phase de continuation du traitement antituberculeux, avec une disparition de l'éruption cutanée en deux semaines. Mots clés: IgA linéaire, dermatose bulleuse, tuberculose disséminée, adolescent.

Febrile Convulsion among Hospitalized Children Aged Six Months to Five Years and Its Association With Haemoglobin Electrophoretic Pattern.

BACKGROUND: Febrile convulsion and sickle cell disease are common in tropical countries and both are associated with significant morbidity and mortality. Worldwide, Nigeria has the highest prevalence of sickle cell disease. However, there is a dearth of knowledge on the haemoglobin electrophoresis in patients with febrile convulsions. METHODS: This was a hospital based, descriptive, cross-sectional study of the relationship between haemoglobin genotype and febrile convulsion at the University of Ilorin Teaching Hospital over a period of 12 months. A self-designed pretested questionnaire was administered on the subjects, and necessary examinations and investigations were conducted. RESULTS: Of a total of 1675 children admitted into the emergency paediatric unit during the study period, children aged 6 months-5 years that presented with febrile convulsions were 167(10%). Of this, 1,212 were aged 6 months-5 years. Thus, the age specific, hospital-based prevalence was 13.8%. The M:F was 1.1:1. Their Haemoglobin genotype distribution was AA 131(78.4%), AS 23(13.8%), AC 6(3.6%), SS 6(3.6%), and 1(0.6%) SC. The mean age of the sickle cell disease patients was higher at 46.0±13.5 months compared to 29.2±15.4 months in the non-sickle cell disease patients (p=0.005). The mean packed cell volume in subjects with sickle cell anaemia was 8.8±1.5%; the only case of haemoglobin SC had packed cell volume of 20%, while the non-sickle cell disease patients had a normal PCV. Malaria was present in 80.4% of them. CONCLUSION: Febrile convulsion remains a common cause of hospitalisation. It is uncommon in haemoglobin SS where severe anaemia is always an accompanying derangement. The packed cell volume is nearly normal in children with normal haemoglobin genotype.

Hypoxaemia in hospitalised under-five Nigerian children with pneumonia.

BACKGROUND: Hypoxaemia constitutes a possible complication of severe respiratory illness which is often under-reported in developing countries. Therefore, the current study was carried out to determine the prevalence and clinical predictors of hypoxaemia in hospitalized under-five children with pneumonia in Ilorin, Nigeria. METHODS: This is a descriptive cross-sectional study of 200 children aged between two months and five years with pneumonia recruited consecutively. Socio-demographic, clinical and laboratory data were obtained. The pulse oximetry measurement was recorded after a stable reading for at least one minute while the child was breathing room air. Hypoxaemia was defined as an arterial oxygen saturation of less than 90%. Data was analyzed using the SPSS 20.0 software. RESULTS: The male/female ratio was 1.5:1.The prevalence of hypoxaemia in the children with pneumonia was 41.5%.Using a linear regression analysis, the clinical features that were significantly associated with hypoxaemia were restlessness, lower chest wall indrawing, bronchial breath sounds and tender hepatomegaly (p<0.05 each). Restlessness had a sensitivity of 22.9%, specificity of 91.5%, while chest wall indrawing had a sensitivity of 86.7% and specificity of 53.3%. Bronchial breath sound had a sensitivity of 16.9%, a specificity of 95.7% whereas tender hepatomegaly had a sensitivity of 48.2% and specificity of 82.9%. CONCLUSION: There is a high local burden of pneumonia-associated hypoxaemia. Restlessness, chest wall indrawing, bronchial breath sounds and tender hepatomegaly could be useful in detecting pneumonia-related hypoxemia in poorly equipped health facilities.