These results suggest that MMF is safe and beneficial as a longer term maintenance immunosuppressive drug in children and adolescents.

MMF based protocols improved long-term graft survival without an increase in side effects. Early ARE were associated with worse half-life of the graft, although more stressed in the AZA group. Thus, to improve quality of life in children for very long-term outcome, ARE should be further decreased and renal function should be better preserved.

Functional voiding disorders and urinary tract infections are common in childhood, but are usually not accompanied by upper urinary tract deterioration. Nevertheless a small group of children remain at risk of developing chronic renal insufficiency (CRI). Clinically these children present day and night wetting. The most important parameter, however, is urinary retention which is reflected by an abnormal voiding pattern in the uroflow curve. After ruling out patients with neurogenic or anatomical disorders, nine girls with psychogenic urine retention were observed for 5 years. Terminal renal insufficiency was seen in one, CRI in five patients and in three patients the kidney function could be maintained, but they all had severe scarring of at least one kidney. Furthermore, all revealed a dilation of the bladder and the upper urinary tract. Vesicoureteral reflux occurred in six and obstruction of the ureterovesical junction in three patients. Two girls underwent repeated reflux surgery resulting in a rapid deterioration of renal function. Three patients developed hypertension and one had a hypertensive crisis with microangiopathic anaemia and acute renal failure. Psychogenic disorders and problematic family settings were observed in all cases. Bladder training, transitory suprapubic catheters, intermittent catheterisation, medication and psychotherapy can avoid severe kidney damage and achieve a stabilisation of renal function. It is important to bear this syndrome in mind when evaluating girls with asymptomatic bacteriuria and urinary retention.

An association of the idiopathic nephrotic syndrome (NS) with certain human leucocyte antigens (HLA) has been reported repeatedly. The aim of this study is to characterize further the clinical and histological features of patients with NS in relation to their HLA phenotypes. HLA antigens were determined in 132 paediatric patients with NS. In 91 steroid-sensitive patients (usually associated with minimal glomerular changes), the antigen frequencies of HLA-DR3, HLA-DR7, and HLA-B8, -DR3 combined were significantly increased compared with controls. The strongest association was observed with the combined occurrence of HLA-B8, -DR3, -DR7 (relative risk 21.5). This association and that with HLA-DR3 alone were strongest in the presence of frequent relapses and steroid dependence compared with children without or with infrequent relapses. The pattern of HLA antigens was similar in the 57 steroid-sensitive patients with biopsy-proven minimal glomerular changes. In 41 children with steroid-resistant NS (usually associated with focal segmental glomerulosclerosis) a similar trend for increased antigen frequencies was found but the data were significant only for the combined occurrence of HLA-B8, -DR3 and -DR7. In all patients combined the frequency of the HLA associations was significantly lower when the age of onset was greater than 8 years compared with that of younger patients. It is concluded that the immunogenetic background of the steroid-sensitive and steroid-resistant NS is different and age-dependent.

Bardet-Biedl syndrome is an autosomal recessive disorder characterized by postaxial hexadactyly, obesity, mental retardation, pigmented retinopathy, hypogonadism, and renal disease. Morphological changes are present in all areas of the kidney, the renal medulla being the most frequently affected site. Cystic and dysplastic changes are prevalent. Seven children from five families were followed from birth through their 5th birthday. Serial renal sonography revealed a number of characteristic features. Bilateral renal enlargement and increased parenchymal echogenicity were present at birth. The usual corticomedullary differentiation was absent. Pyramids were either not seen or deformed. With high-resolution ultrasonography, small cysts were detected at the corticomedullary junction. After the 3rd month of life, there was a striking inversion of normal echogenicity, the inner medulla became more echogenic and was demarcated from the less-echogenic cortex. After 12 months, the kidney size regressed significantly. Fetal lobulation persisted in some patients. In conclusion, ultrasonography is a useful tool to evaluate the extent of renal lesions, but more importantly to differentiate bilateral polycystic kidney diseases in the newborn period.