Echocardiographic reference z scores of right ventricular dimension and systolic function of children aged 5-12 years.

BACKGROUND: Reference values for right ventricular dimension and systolic function in Nigerian children are scarce despite their high burden of right ventricular abnormalities. Reference values from other countries may not be suitable for use in Nigerian children because of possible racial variations in cardiac size. PURPOSE: To develop reference values for right ventricular dimension and systolic function in healthy Nigerian children aged 5-12 years. METHODS: This descriptive cross-sectional study conducted between July and November 2019 included 480 healthy boys and girls aged 5-12 years. The participants were randomly selected from 6 primary schools in the Ikeja Local Government area of Lagos State and their weights and heights measured. Body mass index and body surface area were calculated. Echocardiography was performed at rest in the left lateral position. RESULTS: The right ventricular end-diastolic basal diameter (RVD1), right ventricular end-diastolic mid-cavity diameter (RVD2), and right ventricular end-diastolic length (RVD3) were obtained. The right ventricular end-diastolic basal diameter (RVD1), right ventricular end-diastolic mid-cavity diameter (RVD2), and right ventricular end-diastolic length (RVD3) were obtained, as well as tricuspid annular plane systolic excursion (TAPSE) and tissue Doppler-derived right ventricular systolic excursion velocity (S'). The overall mean±standard deviation (SD) values for RVD1, RVD2, RVD3, TAPSE, and S' were 32.95±4.2, 25.86±3.5, 54.57±7.5, 20.11±2.3, and 18.24±2.2, respectively. Age- and sex-specific mean and SD values of the same cardiac indices were determined. Z score charts and the mean± 2SD right ventricular dimensions and systolic function were generated. All right ventricular dimensions were positively correlated with weight, height, body surface area, and body mass index. Only height correlated consistently with TAPSE and S'. CONCLUSION: The observed mean right ventricular dimension indices differed from those derived elsewhere, suggesting that values from other countries may be inappropriate for Nigerian children. These reference values are applicable in daily clinical practice.

Rhythm abnormalities among hospitalised children in Lagos: a case series.

Arrhythmias refer to disturbances in heart rate or rhythm which leads to heart rates that are abnormally fast, slow or irregular. Rhythm abnormalities may be common among Nigerian children but there are only a few reports. The current write up is a clinical review of eight patients in various age groups including neonates, infants and older children. It is presented to highlight the different forms of arrhythmias that can occur in children, with varying underlying aetiology, thus, stressing the need for early recognition of arrhythmias in children, appropriate early intervention and challenges involved in their care.

Prevalence and determinants of peripheral arterial disease in children with nephrotic syndrome.

Peripheral arterial disease (PAD) is the least studied complication of nephrotic syndrome (NS). Risk factors which predispose children with NS to developing PAD include hyperlipidaemia, hypertension and prolonged use of steroids. The development of PAD significantly increases the morbidity and mortality associated with NS as such children are prone to sudden cardiac death. The ankle brachial index (ABI) is a tool that has been proven to have high specificity and sensitivity in detecting PAD even in asymptomatic individuals. We aimed to determine the prevalence of PAD in children with NS and to identify risk factors that can independently predict its development. A comparative cross-sectional study was conducted involving 200 subjects (100 with NS and 100 apparently healthy comparative subjects that were matched for age, sex and socioeconomic class). Systolic blood pressures were measured in all limbs using the pocket Doppler machine (Norton Doppler scan machine). ABI was calculated as a ratio of ankle to arm systolic blood pressure. PAD was defined as ABI less than 0.9. The prevalence of PAD was significantly higher in children with NS than matched comparison group (44.0% vs 6.0%, p < 0.001). Average values of waist and hip circumference were significantly higher in subjects with PAD than those without PAD (61.68± 9.1cm and 67.6± 11.2 cm vs 57.03 ± 8.3cm and 65.60± 12.5cm respectively, p< 0.005). Serum lipids (triglyceride, very low density lipoprotein, total cholesterol and low density lipoprotein) were also significantly higher in subjects with PAD than those without PAD [106.65mg/dl (67.8-136.7) vs 45.72mg/dl (37.7-61.3), 21.33mg/dl (13.6-27.3) vs 9.14mg/dl (7.5-12.3), 164.43mg/dl (136.1-259.6) vs 120.72mg/dl (111.1-142.1) and 93.29mg/dl (63.5-157.3) vs 61.84mg/dl (32.6-83.1), respectively p< 0.05]. Increasing duration since diagnosis of NS, having a steroid resistant NS and increasing cumulative steroid dose were independent predictors of PAD in children with NS; p< 0.05 respectively. With these findings, it is recommended that screening for PAD in children with NS should be done to prevent cardiovascular complications before they arise.

Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja.

Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.