What do we know about chronic renal failure in young adults? II. Adult outcome of pediatric renal disease

Abstract

Congenital abnormalities of the kidney and urinary tract (CAKUT) account for more than half of all renal failure in children. For young adults with CAKUT two questions are paramount: what is the prognosis and what is the best management to improve outcome? The paediatric literature shows that prognostic factors are glomerular filtration rate (GFR) and the presence of proteinuria. We reviewed data from 101 young adult patients with either primary vesico-ureteric reflux and renal dysplasia or obstructive uropathy. Patients had an estimated GFR (eGFR) of <or=60 ml/min per 1.73 m(2) body surface area and had had at least 5 years of follow up (median 162 months). There was a strong correlation between the amount of proteinuria at the start and overall rate of decline. Angiotensin-converting enzyme inhibitors (ACEIs) slowed declining renal function at all levels of function, but this only had a significant effect on renal outcome when eGFR was >35 ml/min. The ACEI benefit increased with time. Rate of decline was slower than reported for other diseases and was only -2.4 ml/min per year for those reaching the start of dialysis. Outcome is predictable by the level of residual renal function (GFR). Nevertheless, function remains stable while proteinuria is minimal. Short-term studies overestimate rates of deterioration.

Timing and Outcome of Renal Replacement Therapy in Patients with Congenital Malformations of the Kidney and Urinary Tract

“…Bilateral renal hypoplasia and dysplasia with or without concomitant urinary tract malformations are present in more than 50% of children and adolescents commencing renal replacement therapy (RRT) (1)(2)(3)(4)(5). In contrast, CAKUT is a much less common cause of ESRD in adult patients undergoing RRT, accounting for less than 5% of a disease spectrum dominated by diabetic and nondiabetic glomerulopathies (6)(7)(8)(9).…”

Poststreptococcal glomerulonephritis in children with congenital anomalies of the kidney and urinary tract

“…[1][2][3][4] The prevalence of CAKUT varies between 3 and 6 per 1000 births. The prognosis is dependent on the severity of malformations (unilateral, bilateral), presence of hypoplasia, dysplasia, and associated urinary tract infections.…”

“…Traditional risk factors for CKD progression are also important such as hypertension, hyperlipidemia, smoking, nephrotoxic agents, obesity. 1,4 CAKUT patients usually have slower progression to ESRD than those with glomerulonephritis. There are few reports on CAKUT patients with poststreptococcal glomerulonephritis (PSGN).…”

Congenital Obstructive Nephropathy: Clinical Perspectives and Animal Models

“…The ItalKid Project found no benefit from angiotensin converting enzyme inhibitors (ACEi) in a population of patients with renal hypodysplasia, many of whom also had congenital obstructive nephropathy (Ardissino, 2007). However, a later study in young adults with congenital obstructive nephropathy or primary VUR with hypodysplasia indicated that ACEi can slow this decline in renal function, but impact renal outcome only when the estimated glomerular filtration rate is greater than 35 ml/min (Neild, 2009b). In patients with post-obstructive bladder dysfunction, an individualized voiding regimen designed to maintain bladder volume below a critical filling volume can stabilize deteriorating renal function (Hale et al, 2009).…”