No abstract

The most significant prognostic factor for the future development of CRF is the GFR at 1 year old. The onset of proteinuria during the follow-up is associated with a worse prognosis. Echogenic renal changes may help to identify those dysplastic kidneys that will develop RI. Neonatal boys in RI who underwent pyelo-ureterostomy had better RF during the first years of life than those who underwent valve ablation.

Bladder dysfunction should be considered as a prognostic factor in renal failure. Those with poor bladder compliance and myogenic failure have the worst outcome, while bladder instability was associated with the lowest incidence of renal failure.

Objective To identify genetic and non-genetic risk factors contributing to the severity of the bladder exstrophy-epispadias complex (BEEC). Study design Patients with BEEC from North America (n=167) and Europe (n=274) were included. The following data were collected: associated anomalies, parental age at conception, mode of conception, periconceptional folic acid supplementation, maternal risk factors during pregnancy, and environmental risk factors. Patients were divided into three subgroups according to phenotype severity: (i) mild - epispadias (E, n=43); (ii) intermediate - classic bladder exstrophy (CBE, n=366); and (iii) severe - cloacal exstrophy (CE, n=31). These subgroups were then compared to identify factors which contribute to phenotype severity. Results Males were overrepresented in all subgroups. A relatively high prevalence of cleft lip with or without cleft palate was observed. Maternal smoking and medical radiation during the first trimester were associated with the severe CE phenotype. Compliance with periconceptional folic acid supplementation was associated with the mildest phenotype (E). Conclusions Periconceptional folic acid supplementation appears to prevent the development of the severe phenotype of BEEC.

Posterior urethral valves are the most common cause of congenital obstructive uropathy, resulting in renal failure in childhood. Nowadays, in most cases, diagnosis is suggested by antenatal ultrasound. However, antenatal intervention has not resulted in a significantly improved outcome. Endoscopic valve ablation is the initial treatment in most of these neonates, but others procedures, like vesicostomy or ureterostomy, can also be justified in some particular cases in order to improve renal function prognosis. Different factors like bladder dysfunction, VUR, polyuria and proteinuria, can be responsible for the slow and progressive deterioration in renal function that some of these patients show over the years. By treating them all, we may prevent or delay the onset of end stage renal disease.