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

Generalized lymphadenopathy: an unusual presentation of burkitt lymphoma in a Nigerian child: a case report

Intoduction: Burkitt Lymphoma is the fastest growing tumor in human and the commonest of the childhood malignancies. Generalized lymphadenopathy is a common feature of immunodeficiency associated Burkitt lymphoma but an uncommon presentation of the endemic type in Human Immunodeficiency Virus (HIV) negative children. Case presentation: The authors report a 6 year old HIV negative boy who presented with generalized lymphadenopathy, cough, weight loss, fever and drenching night sweat and had received native medication as well as treatment in private hospitals. His examination revealed hepatosplenomegaly, bull neck with generalized significant massive lymphadenopathy. Diagnosis was missed initially until a lymphnode biopsy for histology confirmed Burkitt lymphoma. He was managed on combination chemotherapy with complete resolution and now on follow up. Conclusion: To the best of our knowledge, this is the first documented report of its kind of endemic Burkitt lymphoma involving lymphnodes generally as the primary site. High index of suspicion and early biopsy are the key in this uncommon presentation

Glanzmann\u2019s thrombasthenia: a rare bleeding disorder in a Nigerian girl

Introduction: Glanzmann\u2019s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder due to defective platelet membrane glycoprotein GP IIb/IIIa (integrin \u3b1IIb\u3b23). The prevalence is estimated at 1:1,000,000 and it is commonly seen in areas where consanguinity is high. Case Presentation: The authors report a 12 year old Nigerian girl of Igbo ethnic group, born of non-consanguineous parents, who presented with prolonged heavy menstrual bleeding which started at menarche 3 months earlier, weakness and dizziness. She had a past history of recurrent episodes of prolonged epistaxis, gastrointestinal bleeding and gum bleeding during early childhood. On examination, she was severely pale with a haemic murmur and vaginal bleeding. The initial diagnosis was menorrhagia secondary to bleeding diathesis possibly von Willebrand\u2019s Disease. She was on supportive treatment with fresh whole blood, fresh frozen plasma and platelets until diagnosis of GT was made in the USA. Currently, she is on 3 monthly intramuscular Depo-provera with remarkable improvement. Conclusion: To the best of our knowledge, this is the first documented report of GT in our environment where consanguinity is rarely practised. Our health facilities require adequate diagnostic and treatment facilities for rare diseases like GT

Determinants of academic performance in children with sickle cell anaemia

BACKGROUND: Some factors are known to influence the academic performance of children with Sickle Cell Anaemia (SCA). Information on their effects in these children is limited in Nigeria. The factors which influence academic performance of children with SCA in Enugu, Nigeria are determined in this study. METHODS: Consecutive children with SCA aged 5–11 years were recruited at the weekly sickle cell clinic of the University of Nigeria Teaching Hospital (UNTH) Enugu, Nigeria. Their age- and sex- matched normal classmates were recruited as controls. The total number of days of school absence for 2009/2010 academic session was obtained for each pair of pupils from the class attendance register. Academic performance was assessed using the average of the overall scores in the three term examinations of same session. Intelligence ability was determined with Draw-A-Person Quotient (DAPQ) using the Draw-A-Person Test while socio-economic status was determined using the occupational status and educational attainment of each parent. RESULTS: Academic performance of children with SCA showed statistically significant association with their socio-economic status (χ2 = 9.626, p = 0.047), and significant correlation with DAPQ (r = 0.394, p = 0.000) and age (r = -0.412, p = 0.000). However, no significant relationship existed between academic performance and school absence in children with SCA (r = -0.080, p = 0.453). CONCLUSIONS: Academic performance of children with SCA is influenced by their intelligence ability, age and socio-economic status but not negatively affected by their increased school absenteeism

Digestive system complications among hospitalized children with sickle cell anaemia in Enugu, Nigeria

Background: Sickle cell anaemia is a multi-systemic disease with variable clinical manifestations including those involving the digestive system. There is paucity of data on the digestive system complications of sickle cell anaemia in children in our setting.Objective: To determine the pattern of digestive system complications among hospitalized children with sickle cell anaemia in University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.Methodology: A 7-year retrospective observational study of cases of digestive system complications among hospitalized children with sickle cell anaemia was carried out. Relevant clinical data including socio-demographic characteristics, digestive system complications diagnosed, definitive investigations applied, treatment given and possible outcomes were extracted from the case record files of selected cases using a semi-structured questionnaire. Data were analyzed using SPSS version 21.0 (IBM Corp, Armonk, NY 2012) while the level of statistical significance was set at p<0.05.Results: There were 350 children with sickle cell anaemia attending the sickle cell clinic out of which 33 had serious digestive system complications requiring hospitalizations during the period under review. Their mean age was 9.1±4.8years (9months to 18years).Nearly half, 16(48.5%) of the affected children were of low socio-economic background. Acute abdominal crisis, which was found in 12(36.4%) of cases was the most common complication, while cholelithiasis was the least common complication, occurring in one (3.3%) of the cases studied.Conclusion: Acute abdominal crisis is the most common digestive system complication in children with sickle cell anaemia in our setting. Efforts should be made to exclude other potential causes of acute abdomen which may require surgical intervention in children with sickle cell anaemia. History taking, diligent physical examination and relevant laboratory and imaging studies, would readily facilitate the diagnosis, and save lives.Keywords: Acute abdominal crisis, children, haemoglobin, hospitalization, multi-systemi

Health education to promote knowledge about sickle cell disease and newborn screening in pregnant women: a community-based pilot study using the healthy beginning initiative

Abstract Background Pregnancy presents a critical period for any maternal and child health intervention that may impact the health of the newborn. With low antenatal care attendance by pregnant women in health facilities in Nigeria, community-based programs could enable increased reach for health education about sickle cell disease (SCD) and newborn screening (NBS) among pregnant women. This pilot study aimed to assess the effect of education on the knowledge about SCD and NBS among pregnant women using the Healthy Beginning Initiative, a community-based framework. Methods A pre-post study design was used to evaluate knowledge of SCD and NBS in a convenience sample of 89 consenting pregnant women from three communities. Participants were given surveys prior to and following completion of a health education session. McNemar’s test was used to compare the proportion of participants with correct responses. The level of significance was taken as p < 0.05. Results Compared to pre-test values, post-test values showed that participants understood that SCD is hereditary (93.3% vs. 69.7%), both parents must have at least one gene for someone to have SCD (98.9% vs. 77.5) and blood test is the right way to know if one has SCD (98.8% vs. 78.7%). Also, a large proportion of participants (post-test ~ 89.9%; compared to pre-test ~ 23.6%) understood that the chance of conceiving a child with SCD was 25% for a couple with the sickle cell trait (SCT). Knowledge of the possibility of diagnosing SCD shortly after birth was highly increased in the post test phase of the study when compared to the pre-test phase (93.3% vs. 43.9%, respectively). Concerning the overall knowledge scores, those with high level of knowledge significantly increase from 12.6% pretest to 87.4% posttest (p = 0.015). Conclusion The health education intervention was associated with significant improvement on almost all measures of SCD knowledge. Focused health education for pregnant women using community structures can improve knowledge of SCD and NBS