Bioelectric impedance analysis of body composition of children and adolescents with sickle cell anemia in Enugu, Nigeria.

BACKGROUND: Body composition indices are widely used to evaluate growth and nutrition in children, particularly those with sickle cell anemia (SCA), who are known to have impaired growth, impaired skeletal maturation, and delayed puberty. The current study is aimed at determining the body composition of children with SCA. METHODS: Consecutive selection of SCA children aged 6 to 18 years, who served as subjects and their age and gender matched children with HbAA (controls), selected using multi-stage systematic sampling. It was a cross-sectional descriptive study conducted at the pediatric hematology and oncology clinic. Bioelectric impedance analysis was used to determine the body composition parameters, including weight, body fat percentage (BFP), visceral fat percentage, body mass index (BMI), skeletal muscle percentage, and resting metabolic rate. Data were analyzed using SPSS 16.0 at P < 0.05. RESULTS: One hundred and thirty-two subjects and controls respectively were studied. Subjects had lower body composition parameters compared with controls, with the older male subjects aged 10 to 18 years, having lower body composition indices (weight, height, BMI, and BFP) compared with controls. Visceral fat percentage was low in both subjects and controls. CONCLUSIONS: Children with SCA, particularly the older males, have impaired body composition indices. There is a need to conduct further studies to determine the longitudinal aspects of growth as well as quantitative and qualitative assessment of nutritional intake in children with SCA, which will inform higher alimentation requirements for HbSS children as compared with HbAA individuals.

A comparative analysis on risk of pulmonary hypertension in children with Atrio-ventricular (AV) canal defect: a multi-centre study.

Objectives: This study is aimed at determining the risk of pulmonary hypertension in children with AV canal defect when compared with children with other congenital heart disease. Methods: A descriptive study carried out in three institutions over a six-year period among children who presented with AV canal defect and their controls who presented with other congenital heart defects. Results: A large proportion of the children with AV canal (77.5%) had pulmonary hypertension. Among the patients with pulmonary hypertension, 45.2% were males compared 54.8% females (χ2 = 3.2, p = 0.2). There was a positive correlation between pulmonary hypertension and size of VSD and ASD, although the correlation was not significant (Pearson correlation coefficient = 0.01 and 0.4, p = 0.9 and 0.1 respectively). Children with AV canal defect had higher odds of developing most clinical symptoms and pulmonary hypertension than children with other congenital heart disease and this is statistically significant. Conclusion: Majority of children with AV canal defect presented with pulmonary hypertension. These children present with higher odds of having pulmonary hypertension and clinical symptoms than children with other types of congenital heart disease.

Sonographic evaluation of the spleen among sickle cell disease patients in a teaching hospital in Nigeria.

BACKGROUND: Regional variations in size and parenchyma echo-texture of the spleen among sickle cell disease (SCD) patients have been documented in various publications. The objectives of this study were to assess the size and parenchyma echo-texture of the spleen of SCD patients and ascertain the relationship of age, height and weight with the spleen sizes. METHODS: This was a cross sectional study involving 103 each of SCD and age matched control subjects. Aloka ST- 550 -3500 ultrasound machine with 3.5 and 5 MHz convex transducers was used to scan the subjects over a 15 months period (September, 2012 to November, 2013). The age, height and weight of each subject were recorded. RESULTS: The spleen sizes of SCD patients were generally larger than those of the controls (p < 0.05). Abnormal spleen parenchyma of varied appearances was found among the SCD subjects. There were negative correlations between mean spleen sizes and height, weight and age in SCD patients but positive correlations were found between them in the controls. CONCLUSION: Routine sonographic assessment of spleen size and echo-texture is useful in the management of SCD patients.

Velocidade de fluxo sanguíneo cerebral em crianças em crianças e adolescentes com doença falciforme em Salvador, Bahia / Cerebral blood flow velocity in children and adolescents with sickle cell disease in Salvador, Bahia

INTRODUÇÃO: A doença falciforme (DF) é caracterizada por complicações agudas e crônicas. Entre as agudas podemos citar: episódios álgicos, síndrome torácica aguda (STA), priapismo, crise hemolítica, infecções agudas e acidente vascular cerebral (AVC), sendo este útimo responsavel por complicações a longo prazo na infância. A velocidade do fluxo sanguíneo cerebral (VFSC) elevada é o fator de risco mais importante para o desenvolvimento do AVC em crianças com anemia falciforme. A identificação de pacientes de risco associados a velocidades de fluxo sanguíneos cerebrais anormais é realizada pelo Doppler transcraniano (DTC), exame fundamental à prevenção primária do AVC. OBJETIVOS: Avaliar as velocidades de fluxo sanguíneo cerebral em crianças e adolescentes com DF em Salvador-Bahia, para identificar aqueles com risco alto de AVC, além de correlacionar as velocidades de fluxo cerebral com os perfis clínico e hematológico dos pacientes. PACIENTES E MÉTODOS: O DTC por insonação, utilizando uma sonda de 2 MHZ...

BACKGROUND: Sickle cell disease (SCD) is characterized by acute episodes of illnesses (crises) such as bone pain crisis, acute chest syndrome (ACS), priapism, hemolytic crisis, acute infections; and acute and long term complications such as cerebrovascular accident (CVA). Abnormally high cerebral blood flow velocity is the most important risk factor for development of stroke in pediatric patients with sickle cell anemia, and its detection by transcranial Doppler (TCD) is fundamental in primary stroke prevention. Other clinical, hematologic and genetic risk factors of stroke have also been identified. OBJECTIVES: The study aimed at evaluating the cerebral blood flow velocities of children and adolescents with SCD in Salvador, Brazil, detect those at high risk of stroke and correlate the flow velocities with clinical and hematological profiles of the patients. PATIENTS AND METHODS: Transcranial Doppler was performed on subjects aged 2 to 16 years who fulfilled the inclusion criteria, using a 2 MHz...