Nocturnal Enuresis in Children with Sickle Cell Anemia

Sickle cell anemia (SCA) is the commonest hemoglobin disorder among the black population worldwide. Children with SCA may eventually end up with end-organ complications: the kidneys being one of the most frequently affected organs. The renal complications arise from medullary ischemia and infarction leading to features of tubular dysfunction such as hyposthenuria and renal tubular acidosis. Early in life, children with SCA may present with hyposthenuria: one of the earliest renal defects in the disease which results in an obligatory urine output of more than 2 l in a day. The symptomatic manifestation as nocturnal polyuria is thought to be the reason for nocturnal enuresis observed in these children. In spite of the more prevalent occurrence of nocturnal enuresis in children with SCA than in their non-SCA colleagues, its precise underlying mechanisms still remain controversial, with divergent conclusions regarding its pathogenesis. However, the consensus is now tilting towards a multifactorial etiopathogenesis in affected children. This book chapter aims to discuss the epidemiologic perspectives of nocturnal enuresis in SCA, as well as the current hypotheses on the etiopathogenesis of this complication

Maternal Diet during Exclusive Breastfeeding can Predict Food Preference in Preschoolers: A Cross-Sectional Study of Mother- Child Dyads in Enugu, South-East Nigeria

Background: The relationship between food preference in early childhood and prenatal exposure to flavor in the amniotic fluid is well documented. Although its association with flavor transmission in the breast milk has also been noted, it is poorly reported in this country. Objective: The present study aims to determine the relationship between mothers’ dietary exposure during exclusive breastfeeding and food preference in their preschool-aged children. Methods: Two hundred and twenty (220) mother-child dyads who met the study criteria were enrolled. A pre-tested, structured questionnaire was administered to the mothers. The relationship between maternal consumption of flour-based snacks and staple foods during exclusive breastfeeding and the child’s preference for these foods was determined using risk estimates. After controlling for potential confounders, logistic regression was used for multivariate analysis. Statistical significance was determined at p < 0.05 and all the risk estimates were presented as odds ratios (OR) at 95% confidence intervals (CI). Results: The relationship between daily maternal exposure to staple foods during exclusive breastfeeding and the children’s preference for this variety of food was not statistically significant (p = 0.847, OR= 1.083, 95% CI = 0.481-2.437). However, the children’s preference for flour-based snacks was significantly related to weekly or fourth-nightly maternal exposure to similar diet during exclusive breast feeding (p = 0.035, OR = 2.405, 95% C.I = 1.064 - 5.435). Conclusion: Transmission of flavor in the breast milk may contribute in shaping children’s feeding behavior early in life

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Co-incident with Autoimune Haemolytic Anemia in an Adolescent Female: A Case Report

Background: Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is a genetic disorder with a deficiency or absence of G6PD, an enzyme required for the proper function of red blood cells. The pattern of inheritance for G6PD gene mutation is sex-linked recessive. It thus affects males and either homozygous or heterozygous females whose unaffected X chromosome is inactivated (lyonized females). Autoimmune hemolytic anemia (AIHA) is a disorder characterized by premature destruction of red blood cells (RBC) by autologous RBC autoantibodies whose presence is demonstrated by a positive direct antiglobulin (Coombs) test (DAT). AIHA is classified into primary or idiopathic and secondary types based on the absence or presence of an underlying disease. In Nigeria AIHA is rare in children but secondary forms are common in teenagers. Chronic granulomatous disease (CGD) is a rare, genetic, immune disorder characterized by neutrophil dysfunction and deficiency or absence of G6PD in both neutrophils and RBC. CGD has both sex-linked recessive and autosomal recessive pattern of inheritance. Therefore CGD with G6PD could occur in a female with X chromosome anomaly in whom co-existence of an immune disorder and G6PD deficiency could worsen hemolysis. Aim: To highlight the possible existence of the rare chronic granulomatous disease. Case: To describe a case of acute hemolytic anaemia on a background of G6PD deficiency, positive direct Coombs test and a seeming neutrophil dysfunction in an adolescent female. Case Report: We report a case of a G6PD deficient 14 year-old female with systemic inflammatory response syndrome, marked neutrophillia yet mild toxic granulation, steroid-responsive hemolytic anemia and positive direct Coombs test. Conclusion: C6PD deficiency and AIHA may co-exist in an adolescent female possibly as part of CGD but a definitive diagnosis of CGD is required