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	Disease-specific issues		Renal-replacement therapy
	Psychosocial issues		Managing transition effectively

The number of children on renal-replacement therapy programmes increased by 211% between 1986 and 2001. Most of them are expected to survive into adulthood and will, therefore, undergo a transition in health care from paediatric to adult renal services. But we lack expertise in helping them to do this and, currently, this essential service is under resourced.

Transition may be defined as a: “multi-faceted, active process that attends to the medical, psychosocial and educational/vocational needs of adolescents as they move from child-orientated to adult-orientated lifestyles and systems(1)”. For this to be effective, the nephrologist needs to be aware of disease-specific and age-specific considerations.

Disease-specific issues

The most common primary cause for end-stage renal failure in children and young adults is renal dysplasia (27.7%). Obstructive uropathy accounts for 19.2% of cases
and glomerulonephritis for 18.5%.

Most children with glomerulonephritis will have normal renal function and disease remission. The prognosis depends on the underlying renal lesion; many will need long-term follow up into adulthood. Some will have an inevitably progressive course to end-stage renal failure. Primary, focal segmental glomerulosclerosis is the main group of glomerulonephritides that results in end-stage renal failure. Some young adults will have frequent steroid-requiring relapses of nephrotic syndrome and therefore be at risk of all the complications of long-term steroid use or other immunosuppressive drugs -- particularly, in this age group, growth retardation and reduced fertility.

Children with chronic renal failure due to renal dysplasia often have large tubular losses of salt and may need salt supplementation. The number of cases of end-stage renal failure secondary to reflux nephropathy fell to 5.3% of the total in 2001, and not all children with reflux will progress to ESRF. But many will need long-term monitoring of renal function, blood pressure and protein excretion at an adult centre. These traditional risk factors for renal progression apply to renal diseases diagnosed in childhood.

Cystinosis and oxalosis are extremely-rare inherited disorders with onset in childhood, but together they constitute nearly 4% of the total causes of end-stage renal failure in childhood. With improved treatment of these conditions, patients are now surviving into adulthood. These inherited metabolic disorders have extra-renal manifestations that need investigation and treatment long into adult life, even after successful renal transplantation.

Special consideration needs to be given to pregnancy in adolescents and in young adults with chronic renal failure. Patients with mild renal failure are likely to have a successful pregnancy, although increased proteinuria -- often into the nephrotic range -- and hypertension -- with an increased risk of pre-eclampsia -- are common. With worsening renal failure, the outcome is more guarded with regards to conception, successful pregnancy to full term, and deterioration in renal function. Young adults considering pregnancy therefore need pre-pregnancy counselling, and potentially teratogenic drugs such as statins and ACE inhibitors should be stopped.

Renal-replacement therapy

According to Renal Registry data, 73.6% of children with end-stage renal failure have a functioning renal transplant. Transplantation provides the best quality of life, improved growth and psychosocial development, and preserves vascular and peritoneal dialysis access for as long as possible. The Renal Association Standards document recommends as standard that pre-emptive transplantation should be undertaken wherever possible. This should be considered once the glomerular filtration rate is 15-20ml/min. Graft survival rates are similar to adults, therefore a young adult is likely to need multiple transplants; so nephrologists need to have a heightened awareness of sensitisation as well as the long-term sequelae of potent immunosuppressive agents, such as risk of malignancy. The UKT gives priority to paediatric recipients for all favourably matched organs. Adherence to drug therapy is a particular problem for adolescents, and this is exacerbated by complex drug regimes and cosmetic side-effects. Where possible, steroids should be administered on an alternate-day basis to minimise growth impairment.

Automated Peritoneal Dialysis is the most common method of dialysis in children. However, with age, an increasing number move to haemodialysis, so that by the time of transition to adult services, the distribution will be approximately equal.

There is a lack of evidence for optimum, paediatric target-dialysis adequacy, so the minimum requirement is that adult Renal Association adequacy standards(2) are met. The adult Renal Association standards for anaemia, biochemistry, nutrition and cardiovascular risk apply to adolescents with end-stage renal failure. Adult nephrologists need to be aware of a lower target blood pressure, based on height and gender, for their younger patients than for the middle-aged or elderly patients they are more accustomed to treating. Recombinant human growth hormone can be given to children of all ages whose height is greater than two standard deviations below the mean and height velocity below the 25th centile despite adequate nutrition, dialysis and metabolic correction.

Acute renal failure requiring dialysis has a mortality of 25% in children: however, in contrast to adults, survivors are expected to have normal renal function. In adolescents, diarrhoea-associated haemolytic uraemic syndrome is the commonest cause of acute renal failure and mortality in this age range has improved to be only 3-5%. There is no consensus about how long young adults with resolved acute renal failure should be followed by specialist nephrology services.