Estimation of z-scores of cardiac structures in healthy children in Southeast Nigeria.

BACKGROUND: The z-scoring system shows the mean deviation of cardiac dimensions from age-specific population. The use of the European-based z-score studies has resulted in misdiagnosis and late treatment of Nigerian children with heart diseases leading to increased mortality and morbidity. OBJECTIVES: This study aimed to determine the z­scores for various cardiac structures and functions among healthy children in Southeast Nigeria. MATERIALS AND METHODS: This is a cross-sectional observational study where z-scores of the cardiac structure and function of three hundred healthy children were estimated using echocardiography and compared with the standard Detroit values. RESULT: There was a significant positive correlation between age and cardiac valve diameter. The aortic valve strongly correlated positively with age with the Pearson correlation coefficient (ρ) of 0.8 and probability value (P-value) of 0.00. None of the ventricular function variables deviated beyond the +2 or -2 z-score. There was a positive correlation between the z-scores of ventricular function parameters and participants' age, although the strength of the correlation varied. Comparing these values with that of Detroit values, there was a significant difference in the deviation of the mean of the mitral valve and left pulmonary artery diameters from the Detroit values between the males and the females. While the mean z-score mitral valve was 0.8 in males, it was 0.6 in females (P = 0.02). CONCLUSION: Z-scores of cardiac structures and function are derived from children in this locale. These values were similar to that of Detroit values except for the mitral valve and left pulmonary artery.

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.

Folate levels in children with sickle cell anaemia on folic acid supplementation in steady state and crises at a tertiary hospital in Enugu, Nigeria: a prospective, comparative study.

Introduction: Folic acid supplementation is an integral aspect of the management of children with sickle cell anaemia (SCA) especially in Africa. In spite of this, there have been concerns about lower folate levels, especially during crisis. Aim: To determine red cell folate levels of children with sickle cell anaemia in steady state and during crisis and compare with those with haemoglobin AA genotype. Method: This study was prospective, hospital based, and comparative. Fifty children with sickle cell anaemia were recruited during crises and followed up until they met the criteria for attaining steady state. The controls were fifty children matched with those with SCA for age and gender and had haemoglobin AA genotype. Red cell folate estimation was done with the Electrochemiluminescence Immunoassay (ECLIA) method using the automated Roche Cobas e411 equipment. Results: The median (IQR) red cell folate level in children during sickle cell crisis was 265.95 (134.50) ng/ml, which was significantly lower than the median (IQR) of 376.30 (206.85) ng/ml obtained during steady state. Most children with SCA (41 out of 50) had significantly higher folate levels during steady state (T=1081, Z-score= -4.660, p < 0.001). Median level of red cell folate was lower during anaemic crisis compared to vaso-occlusive crisis, though not significantly so (N(50), U = 214.00, Z-score= -1.077, p = 0.305). The median red cell folate level of normal controls was 343.55 (92.90) ng/ml, which was significantly lower than the 376.30 (206.85) ng/ml obtained during steady state (N(50), U= 209.00, Z-score= -7.177, p <0.001). Conclusion: Median red cell folate levels of the study participants were within normal limits, though most children with SCA had significantly higher levels during steady state compared to crisis. Normal controls had significantly lower red cell folate levels than the children with SCA during steady state.

Folate levels in children with sickle cell anemia on folic acid supplementation in steady state and crises at a tertiary hospital in Enugu, Nigeria a prospective, comparative study

Folic acid supplementation is an integral aspect of the management of children with sickle cell anaemia (SCA) especially in Africa. In spite of this, there have been concerns about lower folate levels, especially during crisis. AimTo determine red cell folate levels of children with sickle cell anaemia in steady state and during crisis and compare with those with haemoglobin AA genotype. Method This study was prospective, hospital based, and comparative. Fifty children with sickle cell anaemia were recruited during crises and followed up until they met the criteria for attaining steady state. The controls were fifty children matched with those with SCA for age and gender and had haemoglobin AA genotype. Red cell folate estimation was done with the Electrochemiluminescence Immunoassay (ECLIA) method using the automated Roche Cobas e411 equipment. Results The median (IQR) red cell folate level in children during sickle cell crisis was 265.95 (134.50) ng/ml, which was significantly lower than the median (IQR) of 376.30 (206.85) ng/ml obtained during steady state. Most children with SCA (41 out of 50) had significantly higher folate levels during steady state (T=1081, Z-score= -4.660, p < 0.001). Median level of red cell folate was lower during anaemic crisis compared to vaso-occlusive crisis, though not significantly so (N(50), U = 214.00, Z-score= -1.077, p = 0.305). The median red cell folate level of normal controls was 343.55 (92.90) ng/ml, which was significantly lower than the 376.30 (206.85) ng/ml obtained during steady state (N(50), U= 209.00, Z-score= -7.177, p <0.001). Conclusion Median red cell folate levels of the study participants were within normal limits, though most children with SCA had significantly higher levels during steady state compared to crisis. Normal controls had significantly lower red cell folate levels than the children with SCA during steady state

Left ventricular function and cardiac valvar annular dimensions among children with sickle cell anemia compared to those with hemoglobin AA type in Enugu, Nigeria.

Background: Enumerating the relationship between cardiac structures, function and chamber sizes in children with sickle cell anemia would help in delineating some cardiovascular abnormalities which will aid the Pediatric cardiologist and the cardiac surgeons in a number of decision-making situations. Objectives: The objectives of this study are to assess the dimension of cardiac structures and left ventricular function in children with sickle cell anemia in steady state and controls using echocardiography. Methods: A cross-sectional prospective study that assessed cardiac structures and left ventricular function among fifty-one children with sickle cell anemia (HBSS) and compared with fifty children with HB AA type serving as controls. Results: A significant high proportion of children with sickle cell anemia had abnormal Valvar dimension and left ventricular function above two standard deviations (2-SD) from the mean of the standard population compared to the control group, showing a statistically significant difference (χ2 = 10.42, p= 0.001).All the mean annular valves diameter, left ventricular internal dimension in systole and diastole, inter-sinus distance diameter and sinu-tubular junction diameter are higher in children with sickle cell anemia than controls and this is statistically significant. (p<0.005). Conclusion: This result shows that children with sickle cell anemia have increased valvar size diameter compared with those with normal hemoglobin type. A significantly higher proportion of respondents in type SS group had abnormal left ventricular systolic and diastolic dysfunction when compared with those in type AA group.

Assessment of Nutritional Status of Children With Congenital Heart Disease: A Comparative Study.

Background: Malnutrition poses a great burden to children in the tropics. However, this seems to be accentuated in children with congenital heart disease. Objectives: The present study is therefore aimed at determining the nutritional status of children with congenital heart disease and to compare them with those without congenital heart disease. Methods: This is a cross-sectional study, where congenital heart disease was diagnosed by means of echocardiograph. Anthro software was used to calculate Z scores for weight for age (WAZ), height for age (HAZ), and weight for height (WHZ). Body mass index (BMI) was calculated by the formula BMI = Weight (Kg)/height (M2). Results: The body mass index-for-age z-score (BAZ) and height/length-for-age z-score (HAZ) were calculated for both subjects and controls to determine their nutritional status. It was observed that 38.5% (112/291) of the subjects were wasted (BAZ < -2SD) compared to 6.25% (16/256) of the controls and the difference was statistically significant (χ2 = 81.2, p < 0.001). Stunting (height/length-for-age z-score < -2SD) was also observed in a greater proportion of subjects than controls as 37.8% (107/291) of subjects were stunted compared with 7.0% (18/256) of the controls (χ2 = 69.9, p < 0.001). The under-five subjects had more cases of malnutrition than the controls of same age group as illustrated in Table 6. Whereas 42.9% (96/224) of the under-five subjects were wasted, only 6.2% (12/192) of the controls were wasted. On the other hand, 4.2% (8/192) of the under-five controls were obese compared to 0.9% (2/224) of the subjects of similar age group. Conclusion: Children with congenital heart disease present with varying degrees of malnutrition that is worse compared with children without congenital heart disease. The impact of malnutrition is worse among children under the age of five. Wasting is more prevalent in children with cyanotic heart disease compared with those with acyanotic congenital heart disease. Overweight and obesity were notable features of malnutrition in children with congenital heart disease, but this is worse in children without congenital heart disease.

The effects of ductal size on the severity of pulmonary hypertension in children with patent ductus arteriosus (PDA): a multi-center study.

INTRODUCTION: Patent ductus arteriosus (PDA) is a common acyanotic heart disease that presents with variable symptoms. OBJECTIVES: This study is therefore aimed at determining the relationship between gender, age, and size of PDA and pulmonary hypertension. This study also seeks to determine the prevalence of elevated pulmonary artery systolic pressure in children with PDA. PATIENTS AND METHODS: A descriptive study of children with patent ductus arteriosus was carried out from 2016 to 2020 in three institutions. The data were analysed with the IBM SPSS statistics for windows, version 20 (IBM Corp, Chicago) RESULT: The mean ductal size was 3.78 (2.39) mm, with a minimum of 1.0 mm and a maximum size of 10.0 mm. The mean ductal size for males, 4.02 (2.53) mm was comparable with that of the females, 3.61 (2.28) mm (Student T-test = 0.8, 0.4). The mean pulmonary artery systolic pressure (PASP) of the patients was 43.36 (24.46) mmHg. Also the mean PASP was comparable among the males and the females, 48.37 (26.69) mmHg versus 39.63 (22.16) mmHg (Student T-test = 1.81, p = 0.07). There was no correlation between age and PASP (correlation coefficient = 0.009, p = 0.92). Sixty point two percent (60.2%) (62/103) of children with PDA had pulmonary hypertension. The proportion of males with pulmonary hypertension, 48.39% (30/62) was comparable with that of the females, 51.61% (32/62) (Chi2 = 2.05, p = 0.15) and females are 1.8 times more likely to have pulmonary hypertension as males (odds ratio 1.81, 95% CI 0.8-4.1). There was a positive correlation between ductal size and PASP (Pearson correlation coefficient = 0.26, p value = 0.007). Those with moderate and large sized duct tend to have moderate and severe pulmonary hypertension respectively and this is statistically significant. Chi2 = 17.85, p = 0.007 CONCLUSION: The prevalence of pulmonary hypertension in children with PDA is 60.2%. Moderate and large size duct presents with moderate and severe pulmonary hypertension respectively. Females are 1.8 times more likely to have pulmonary hypertension than the males.

Timed Average Mean Maximum Velocity (TAMMV) of Cerebral Blood Flow of Children and Adolescents with Sickle cell Disease: correlation with clinical and hematological profiles in country.

BACKGROUND: Detection of abnormal TAMMV with transcranial Doppler is fundamental in primary stroke prevention in children with sickle cell disease (SCD). The study aimed at evaluating TAMMV and correlating it with clinical and hematological profiles of children and adolescent with SCD. METHODS: Transcranial Doppler was performed on subjects aged 2-16 years, using a 2 MHz probe placed over the transtemporal windows. Pulse oximetry was used to determine the peripheral oxygen saturation while clinical and hematological profiles were retrieved from their medical records. RESULTS: One hundred and thirty five patients were recruited. The mean TAMMV was 125cm/s. Patients with HbSS had a significantly higher TAMMV (131cm/s) than those with HbSC (107cm/s). Only one (0.74%) patient had abnormal TAMMV. TAMMV correlated inversely with oxygen saturation, Hct and patient's age, and positively with white cell and platelet counts. Previous history of acute chest syndrome (ACS) and recurrent painful crises increased the risk of development of abnormal and conditional velocity. CONCLUSION: Frequency of abnormal TAMMV in this study was low. Younger children and those with HbSS had higher TAMMV. Age, oxygen saturation and haematocrit correlated negatively while white cell and platelet counts correlated positively with TAMMV. Previous history of ACS and recurrent bone pain were associated with increased risk of having abnormal and conditional TAMMV.

Right ventricular function among South East Nigeria children with sickle cell anaemia.

BACKGROUND: Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart. METHODS: The aim of this work is to compare the right ventricular function of children with SCA in steady state (subjects) with those with haemoglobin AA genotype (controls), using echocardiography. It is a cross-sectional study, which echocardiographic measurements to assess right ventricular function among children with SCA and their controls. RESULTS: The mean trans annular plane systolic excursion (TAPSE) in subjects, 28.24 ± 5.23 (Z score: 0.258 ± 1.10) was higher than that in control, 25.82 ± 3.59 (Z score: - 0.263 ± 0.80), and the difference in mean was statistically significant, (t = 2.703, p = 0.008). Significantly higher proportion of subjects with sickle cell anaemia had right ventricular dysfunction (Abnormal TAPSE), 25 (50.0%) when compared with those in control, 11 (22.0%), {χ2 = 8.5, p = 0.0035}. A higher proportion of subjects with sickle cell anaemia (25.5%) had Pulmonary hypertension (RVP) when compared with control (2.0%) and the difference in proportions was found to be statistically significant, (χ2 = 11.668, p = 0.001). The prevalence of right ventricular diastolic dysfunction in subjects was 9.8% while control was 0%. CONCLUSION: Children with sickle cell anaemia present with right ventricular dysfunction. Prevalence of right ventricular systolic and diastolic dysfunction were higher in subjects. More of the subjects in this study (25.5%) had pulmonary hypertension.

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...

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.

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.

Asymptomatic bacteriuria in children with sickle cell anemia at The University of Nigeria teaching hospital, Enugu, South East, Nigeria.

BACKGROUND: Urinary tract infection (UTI) is a common cause of childhood morbidity and mortality in the tropics. Children with sickle cell anemia (SCA) may have compromised kidney function arising from repeated vaso-occlusive episodes and recurrent symptomatic or asymptomatic UTI. OBJECTIVES: This study aims at determining the prevalence of asymptomatic bacteriuria and sensitivity pattern in children with homozygous sickle haemoglobin compared to children with normal haemoglobin. METHODS: One hundred children with SCA in stable state and 100 children with normal haemoglobin aged 2-12 years were screened for asymptomatic bacteriuria using midstream urine samples. The samples were incubated aerobically at 37°C for 24 hours within one hour of collection. Children whose urine samples yielded significant bacteriuria (≥ 105 cfu/ml) on two consecutive cultures were regarded as having asymptomatic bacteriuria. RESULTS: Asymptomatic bacteriuria was noted in 6% of children with SCA and occurred more in females than males (F: M = 5:1) when compared to 2% in children with normal haemoglobin. Escherichia coli was the commonest organism isolated (33.3%). All the organisms were resistant to co-trimoxazole and ampicillin while most were sensitive to gentamicin, ceftriaxone and the quinolones. CONCLUSION: The risk of asymptomatic bacteriuria is three times more common in children with sickle cell anemia than in children with normal haemoglobin. It is therefore important to screen SCA patients, especially the females for UTI and should be treated according to the sensitivity result of the cultured organisms.