Hypertension occurs commonly following renal transplantation and may cause end organ damage, such as cardiac hypertrophy. This study seeks to determine which features of hypertension are related to cardiac hypertrophy in children after renal transplantation. Ambulatory blood pressure monitoring (ABPM) was performed in 45 pediatric patients, 4.9+/-3.0 years after renal transplantation. ABPM data were related to clinical features and echocardiographic measurements. Hypertension was demonstrated in 33% of patients by casual blood pressure (BP) measurement and in 40% by ABPM. The mean percentage nighttime decline in BP (dipping) was 8.9+/-5.0% for systolic and 13.9+/-7.7% for diastolic BP. Abnormal dipping (<10%) was seen in 58% of patients. BP load (percentage of BP recordings above 95th percentile) was >30% in 44% of patients. Patients taking antihypertensive medication had more abnormal dipping and greater nighttime BP load. The prevalence of left ventricular hypertrophy was 72% before transplantation, 75% after transplantation, and 54% near to ABPM. Left ventricular mass (LVM) indexed to height(3) decreased significantly after transplantation. (40.2+/-14.7 vs. 35.0+/-8.3 g/m(3), P=0.0002). There was no significant relationship between ABPM data and LVM. ABPM was not able to differentiate those patients with persistently elevated LVM. The results suggest that hypertension is not always associated with cardiac hypertrophy following pediatric renal transplantation.
OBJECTIVE To document the functional outcome of patients with prenatally detected posterior urethral valves (PUV) in the second decade of life, and to evaluate the possible impact of prenatal diagnosis on the long‐term outcome of this condition PATIENTS AND METHODS We analysed the functional outcome of 25 patients with prenatally detected PUV born between 1984 and 1996, whose mean (range) age at follow‐up was 17.7 (10–23) years. The findings were compared with those in 17 patients (mean age 16.1 years) who had presented clinically to our unit during the same period. The duration of follow‐up in both groups was ≥10 years. Late outcomes were also compared with published data for PUV. Outcome measures included; death, incidence of end‐stage renal failure (ESRF), age at transplantation and the most the recently available plasma creatinine level in untransplanted patients. We also examined any possible association between functional outcome and early predictors, including nadir plasma creatinine level at <1 year and vesico‐ureteric reflux (VUR). RESULTS Three patients died (12%), two as neonates and one aged 3 years. Of five patients who had been shunted in utero, four died or developed early‐onset renal failure. In the 23 prenatally detected patients who survived the neonatal period, four (17%) had a renal transplant at a mean (range) age of 6.5 (3.0–12.0) years. Of 19 patients with prenatally detected PUV who had not been transplanted in the first 12 years of life, only one (5%) developed new‐onset ESRF at 10.0–23.4 years whilst 11 (58%) of these patients had normal creatinine values. In the untransplanted patients there was a statistically significant correlation between age and plasma creatinine level, but no correlation between late functional outcome and nadir creatinine in the first year of life, or bilateral VUR. CONCLUSIONS Prenatal diagnosis had little impact on mortality or ESRF in the first decade of life. This appears to be largely predetermined by renal dysplasia and the severity of intrauterine obstruction. However, the functional outcome of patients with prenatally detected PUV aged 10–23 years was considerably better than published long‐term data and the outcome of clinically presenting patients in our study. These findings suggest that the long‐term prognosis of PUV of intermediate severity might be improved by prenatal diagnosis.
Iron supplementation is required for optimal response to erythropoietin (EPO) in hemodialysis patients. This is due to blood lost in the dialysis tubing after dialysis and the increased demand for iron by EPO therapy. Maintenance intravenous (IV) iron was administered according to a standardized protocol to pediatric patients on hemodialysis in our institution. The effect of this protocol on EPO dose, iron indices, anemia, and medication costs was evaluated. Data on two groups of patients were retrieved from the health records. Group 1 (n=14) consisted of patients treated in the 18 months prior to the protocol. These patients received oral iron supplements and occasional IV iron. Group 2 (n=5) consisted of all patients treated with the IV iron protocol. There was no difference in clinical characteristics and mean values for monthly hemoglobin, serum iron, ferritin, and transferrin saturation between groups. The dose of EPO was significantly reduced in group 2 compared with group 1 (193.9 +/- 121.4 vs. 73.9 +/- 39.0 units/kg per week, P<0.05). Medication costs were reduced by 26% in group 2. No significant adverse events were seen. Maintenance IV iron reduced the dose of EPO required to maintain blood hemoglobin levels. Our results also suggest that maintenance IV iron is a more-economic method of iron supplementation for pediatric hemodialysis patients.
• HSD3B2 enzyme deficiency is one of the rarest forms of congenital adrenal hyperplasia associated with salt wasting due to hypoaldosteronism, hypoglycaemia due to glucocorticoid deficiency, and undervirilisation of the external genitalia in genotypic males due to androgen deficiency. • Bartter syndrome causes hypokalemic, hypochloraemic metabolic alkalosis by activation of the reninangiotensin system. Novel Insights • The coexistence of HSD3B2 enzyme deficiency and Bartter syndrome can result in a state of persistent hypokalemic alkalosis suggestive of high mineralocorticoid activity. • Identification of a rare homozygous mutation in an offspring of non-consanguineous parents should raise suspicion of uniparental isodisomy.
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