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.

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.

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.