Survival in children with ESRD has increased over the last 20 years, but the SMR remains high. Early transplantation and a more vigorous approach toward hypertension and infection may be mandatory in order to further reduce mortality.

Young adult patients with pediatric ESRD are at risk for LVH caused by hypertension and for aortic valve calcification. Diastolic function decreases with age and is enhanced by a current low GFR. Prolonged peritoneal dialysis may enhance aortic valve calcification.

Abstract. Increased arterial stiffness is a risk factor for mortality in adults over 40 yr of age with end-stage renal disease (ESRD). As no data exist on vascular changes in young adults with ESRD since childhood, a long-term outcome study was performed. All living Dutch adult patients with onset of ESRD between 1972 and 1992 at age 0 to 14 yr were invited for carotid artery and cardiac ultrasound and BP measurements. Data on clinical characteristics were collected by review of all medical charts. Carotid ultrasound data were compared with those of 48 age-matched and gender-matched healthy controls. Carotid artery and cardiac ultrasound was performed in 130 out of 187 eligible patients. Mean age was 29.0 (20.7 to 40.6) yr. Compared with controls, patients had a similar intima media thickness but a reduced mean arterial wall distensibility DC (40.0 versus 45.0 kPa Ϫ1 · 10 Ϫ3 ; 95% CI, Ϫ9.1 to Ϫ0.8; P Ͻ 0.001), an increased stiffness parameter ␤ (4.2 versus 3.8; 95% CI, 0.05 to 0.68; P ϭ 0.02), an increased elastic incremental modulus E inc (0.35 versus 0.27 kPa · 10 3 ; 95% CI, 0.02 to 0.12; P Ͻ 0.001). Multiple regression analyses in all subjects revealed that ESRD was associated with an increase in ␤ and E inc . Arterial wall properties of patients currently on dialysis and transplanted patients were comparable. In all patients, current systolic hypertension was associated with increased E inc and decreased DC. In conclusion, carotid arterial wall stiffness is increased in young adult patients with pediatric ESRD. Hypertension is a main determinant and might be a target for treatment of these potentially lethal arterial wall changes.Cardiovascular disease is the main cause of death in adults with end-stage renal disease (ESRD) (1-4). Cardiovascular causes of death are relatively uncommon under the age of 40 yr in the general population. Yet, cardiovascular diseases also account for most deaths in patients with ESRD aged between 25 and 44 yr (1). There are indications that even in children and young adults with ESRD since childhood, cardiovascular disease is the main cause of death, similar to older ESRD patients. We performed a long-term follow up study on the somatic, social, and psychologic outcome of children with ESRD. Over 25% of all patients had died, all of them under the age of 36 yr. We found cardiovascular disease to be the most important cause of death in the whole group, and cardiac death most prominent in those patients who died more than 10 yr after beginning renal replacement therapy (RRT) (5).Clinical studies have shown that increased stiffness of the large arteries independently contributes to the high mortality in dialysis patients over 40 yr of age (6 -8). Recently, studies performed with electron beam CT have shown coronary calcifications in adolescents and young adults with ESRD (9 -11). However, these studies concern only a few patients; to date, no data exist on arterial wall distensibility in young adult patients with ESRD since childhood. The purpose of this study was to assess the pre...

Bone disease is a major clinical problem in young adults with pediatric ESRD. Further follow-up is needed to establish the impact of the low bone mineral densities found in these patients.

No abstract

Since its first identification in Scotland, over 1000 cases of unexplained pediatric hepatitis in children have been reported worldwide, including 278 cases in the UK 1 . Here we report investigation of 38 cases, 66 age-matched immunocompetent controls and 21 immunocompromised comparator subjects, using a combination of genomic, transcriptomic, proteomic and immunohistochemical methods. We detected high levels of adeno-associated virus 2 (AAV2) DNA in liver, blood, plasma or stool from 27/28 cases. We found low levels of Adenovirus (HAdV) and Human Herpesvirus 6B (HHV-6B), in 23/31 and 16/23 respectively of the cases tested. In contrast, AAV2 was infrequently detected at low titre in blood or liver from control children with HAdV, even when profoundly immunosuppressed.AAV2, HAdV and HHV-6 phylogeny excluded emergence of novel strains in cases. Histological analyses of explanted livers showed enrichment for T-cells and B-lineage cells.Proteomic comparison of liver tissue from cases and healthy controls, identified increased expression of HLA class 2, immunoglobulin variable regions and complement proteins.HAdV and AAV2 proteins were not detected in the livers. Instead, we identified AAV2 DNA complexes reflecting both HAdV and HHV-6B-mediated replication. We hypothesize that high levels of abnormal AAV2 replication products aided by HAdV and in severe cases HHV-6B, may have triggered immune-mediated hepatic disease in genetically and immunologically predisposed children.

♦ Objective To analyze the late cardiovascular outcome of end-stage renal disease (ESRD) in children. ♦ Design A nation-wide long-term follow-up study. Determinants of outcomes and causes of death were retrospectively assessed. Patients underwent assessment of overall health state, B- and M-mode ultrasound of the carotid arteries, and echocardiography for cross-sectional analysis. ♦ Results We analyzed the medical course of all 249 adult Dutch patients with ESRD onset between 1972 and 1992 at age 0 – 14 years, and who were born before 1979. Of the 187 living patients, 140 participated in the cross-sectional part of the study. The standardized mortality rate was 31.0. Overall 5-, 10-, and 20-year survival after ESRD onset was 87%, 82%, and 78%, respectively. Cardiovascular disease accounted for most deaths (41%). In the whole group, left ventricular hypertrophy (LVH), aortic valve calcification, and arterial wall stiffening were highly prevalent. LVH was associated with hypertension at time of assessment. Aortic valve calcification was strongly associated with a long total duration of peritoneal dialysis (β = 0.33, p < 0.001). Arterial wall pathology was not associated with current treatment modality. ♦ Conclusions As in adults, cardiovascular disease is the most important cause of death in children with ESRD. Stricter reduction of volume overload, prevention of high serum calcium–phosphate product, and more vigorous treatment of hypertension are important targets to improve cardiovascular survival in children with ESRD.

Dialysis patients with paediatric ESRD have an impaired physical quality of life, but in contrast to dialysis patients with adult ESRD onset, they have a normal mental quality of life.