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

Bio-electric impedance analysis of body composition and glycaemic control in children and adolescents with type 1 diabetes in south-east, Nigeria

Background: Metabolic derangements in diabetes grossly affect components of body composition particularly the fat mass. Adequate glycaemic control is key to the reduction of risks of associated complications.Objectives: To determine the pattern of glycaemic control and body composition in children and adolescents with type 1 diabetes mellitus (T1DM).Methodology: This was a cross sectional descriptive study conducted in three centres in South-East Nigeria. Subjects were T1DM patients attending diabetic clinics at the hospitals who were consecutively recruited while controls were normal school children.Body composition was determined using bio-electrical impedance analysis method. Data was collected using a semi -structured proforma designed for the study and was subsequently analysed using SPSS version 22.0. The level of statistical significance was set at p< 0.05.Results: A total of 108 children and adolescents comprising 54 diabetic patients and 54 controls (age and sex matched) were studied. The mean age of the subjects was 16.3 ± 3.5 years.Majority of the patients had high HbA1c, with a mean and range values of 11.3 ± 2.4 and 7.0 – 13.0%, respectively. The body composition parameters among subjects including the body mass index, skeletal muscle percentage, visceral fat percentage and resting metabolic rate were lower than that of the controls.Conclusion: A majority of the subjects had poor glycaemic control with overall lower measured body composition parameters. There is need to improve glycaemic control and maintain normal body composition in children and adolescents with T1DM in order to minimize the risks of diabetic complications in them.Keywords: Body composition; Glycaemic control; Children; Adolescent

Willingness to accept malaria vaccines amongst women presenting at outpatient and immunization clinics in Enugu state, Southeast Nigeria

Abstract Background There are giant steps taken in the introduction of the novel malaria vaccine poised towards reducing mortality and morbidity associated with malaria. Objectives This study aimed to determine the knowledge of malaria vaccine and factors militating against willingness to accept the vaccine among mothers presenting in nine hospitals in Enugu metropolis. Methods This was a cross-sectional study carried out among 491 mothers who presented with their children in nine hospitals in Enugu metropolis, South-East Nigeria. A pre-tested and interviewer-administered questionnaire was used in this study. Results A majority of the respondents, 72.1% were aware of malaria vaccine. A majority of the respondents, 83.1% were willing to receive malaria vaccine. Similarly, a majority of the mothers, 92.9%, were willing to vaccinate baby with the malaria vaccine, while 81.1% were willing to vaccinate self and baby with the malaria vaccine. The subjects who belong to the low socio-economic class were five times less likely to vaccinate self and baby with malaria vaccine when compared with those who were in the high socio-economic class (AOR = 0.2, 95% CI 0.1–0.5). Mothers who had good knowledge of malaria vaccination were 3.3 times more likely to vaccinate self and baby with malaria vaccine when compared with those who had poor knowledge of malaria vaccination (AOR = 3.3, 95% CI 1–6–6.8). Conclusion Although the study documented a high vaccine acceptance among the mothers, there exists a poor knowledge of the malaria vaccine among them