Of children with idiopathic nephrotic syndrome, 10%-20% fail to respond to steroids or develop secondary steroid resistance (termed initial steroid sensitivity) and the majority progress to transplantation. Although 30%-50% of these patients suffer disease recurrence after transplantation, with poor long-term outcome, no reliable indicator of recurrence has yet been identified. Notably, the incidence of recurrence after transplantation appears reduced in patients with steroid-resistant nephrotic syndrome (SRNS) due to monogenic disorders. We reviewed 150 transplanted patients with SRNS to identify biomarkers that consistently predict outcome of SRNS after transplantation. In all, 25 children had genetic or familial SRNS and did not experience post-transplant recurrence. We reviewed phenotypic factors, including initial steroid sensitivity, donor type, age, ethnicity, time to ESRD, and time on dialysis, in the remaining 125 children. Of these patients, 57 (45.6%) developed post-transplant recurrence; 26 of 28 (92.9%) patients with initial steroid sensitivity recurred after transplantation, whereas only 26 of 86 (30.2%) patients resistant from the outset recurred (odds ratio, 30; 95% confidence interval, 6.62 to 135.86; P,0.001). We were unable to determine recurrence in two patients (one with initial steroid sensitivity), and nine patients did not receive initial steroids. Our data show that initial steroid sensitivity is highly predictive of post-transplant disease recurrence in this pediatric patient population. Because a pathogenic circulating permeability factor in nephrotic syndrome remains to be confirmed, we propose initial steroid sensitivity as a surrogate marker for post-transplant recurrence.

These guidelines cover all aspects of the care of patients who are treated with peritoneal dialysis. This includes equipment and resources, preparation for peritoneal dialysis, and adequacy of dialysis (both in terms of removing waste products and fluid), preventing and treating infections. There is also a section on diagnosis and treatment of encapsulating peritoneal sclerosis, a rare but serious complication of peritoneal dialysis where fibrotic (scar) tissue forms around the intestine. The guidelines include recommendations for infants and children, for whom peritoneal dialysis is recommended over haemodialysis.Immediately after the introduction there is a statement of all the recommendations. These recommendations are written in a language that we think should be understandable by many patients, relatives, carers and other interested people. Consequently we have not reworded or restated them in this lay summary. They are graded 1 or 2 depending on the strength of the recommendation by the authors, and A-D depending on the quality of the evidence that the recommendation is based on.

The clinical features and management of 27 children with cerebral palsy referred with symptoms of lower urinary tract dysfunction were reviewed. The mean age at referral was 9.9 years. Daytime urinary incontinence was the commonest presenting symptom. Videourodynamic studies were abnormal in 23 patients (85%). Only two children had evidence of upper renal tract damage. Treatment was determined by urodynamic findings, and led to improvement in symptoms in all patients for whom there was follow up information.Urinary incontinence may be improved or cured in children with cerebral palsy. These children would therefore benefit from early referral for assessment and treatment. (Arch Dis Child 1993; 68: 739-742) It is generally perceived that urinary incontinence is commoner in children with cerebral palsy than in normal children. Several (TABLE 2)

SummaryBackground and objectives Heart disease is a major cause of death in young adults with chronic kidney disease (CKD). Left ventricular hypertrophy (LVH) is common and is associated with hypertension. The aims of this study were to evaluate whether there is a relationship between LVH and BP in children with CKD and whether current targets for BP control are appropriate.Design, setting, participants, & measurements In this single-center cross-sectional study, 49 nonhypertensive children, (12.6 Ϯ 3.0 years, mean GFR 26.1 Ϯ 12.9 ml/min per 1.73 m 2 ) underwent echocardiographic evaluation and clinic and 24-hour ambulatory BP monitoring. LVH was defined using age-specific reference intervals for left ventricular mass index (LVMI). Biochemical data and clinic BP for 18 months preceding study entry were also analyzed. ResultsThe mean LVMI was 37.8 Ϯ 9.1 g/m 2.7 , with 24 children (49%) exhibiting LVH. Clinic BP values were stable over the 18 months preceding echocardiography. Patients with LVH had consistently higher BP values than those without, although none were overtly hypertensive (Ͼ95th percentile). Multiple linear regression demonstrated a strong relationship between systolic BP and LVMI. Clinic systolic BP showed a stronger relationship than ambulatory measures. Of the confounders evaluated, only elemental calcium intake yielded a consistent, positive relationship with LVMI.Conclusions LVMI was associated with systolic BP in the absence of overt hypertension, suggesting that current targets for BP control should be re-evaluated. The association of LVMI with elemental calcium intake questions the appropriateness of calcium-based phosphate binders in this population.

Control of BP after kidney transplantation remains sub-optimal in paediatric centres in the UK. Just over 25% of patients remain hypertensive 5 years following transplantation. Significant differences between centres remain unexplained and may reflect differences in assessment and management of hypertension.

We reviewed the medical records of seven children with congenital nephrotic syndrome (CNS) treated by unilateral nephrectomy, captopril, and indomethacin since 1990. Clinical response to the treatment was analyzed using the Students' t-test. After a median period of 54 months (range 36-88 months) follow-up, five patients were alive at a median age of 74 (range 43-88) months. Median (range) plasma albumin rose from 11 (6-17) g/l at the start of treatment to 18 (15-22) g/l and 21 (18-25) g/l after 6 and 12 months treatment, respectively (P=0.001 and P=0.0006). Albumin infusions per patient per month decreased from 7 (0-18) to 0 (0-30) in the 6 months post treatment (P=0.017). The median (range) height standard deviation scores at 12 months and 30 months from onset of treatment were -1.56 (−2.96 to 0.41) and -1.43 (−2.40 to 0.90), respectively. In conclusion, management of CNS with captopril and indomethacin therapy in combination with unilateral nephrectomy achieves significant improvements in plasma albumin and reduces the need for albumin infusions and time in hospital, while growth is maintained. Second nephrectomy, dialysis, and transplantation can be delayed until the 3rd year of life or longer.

Overweight and obesity are increasingly common, and are major determinants of high blood pressure in children, in both the developed and developing world. Initial evaluation of the hypertensive child must include carefully confirming if they are hypertensive using published reference data for blood pressure in children, and increasingly through ambulatory blood pressure monitoring. Left ventricular hypertrophy based on echocardiography remains the most widely used indirect marker of hypertensive end organ change. New techniques for assessing target organ damage are being developed.

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