Tremendous progress was made recently in the understanding of hereditary renal diseases through the identification of the genes and encoded proteins involved in many of the recognized disorders that has influenced diagnostic approaches substantially. These advances have been facilitated by the Human Genome Project, an international cooperative effort initiated in 1990, designed to elucidate the genome sequence of humans and selected experimental animal models. A draft of the human sequence that covers about 94% of our genome has been published in February 2001  and the whole assembled sequence is nearly available.
During childhood, the kidney may be involved in several different systemic diseases including the vasculitic syndromes, collagen vascular di sease, thrombotic microangiopathic, malignant and infectious diseases [1-3]. Even though the incidence of these conditions is much lower in childhood, their occurrence present serious diagnostic and treatment problems.
Chronic renal failure (CRF) is uncommon in children and its management should be under the direction of a specialist centre where the dietitian is a key member of the multi-professional team. Early recognition and treatment are essential for maximizing growth and minimizing complications, which include metabolic acidosis, renal osteodystrophy, hormonal disturbances and malnutrition. The progression of renal failure also may be exacerbated by hypertension , proteinuria  and hyperlipidaemia , all of which have dietary components in their management.
Renal transplantation is the optimal form of renal replacement therapy for children with endstage renal disease (ESRD). This conclusion is based on data demonstrating superior patient survival following transplantation compared to dialysis for all comparable ages , and data demonstrating optimal growth and neuropsychological development, particularly when the duration and degree of uraemia is minimized . Paediatric transplant recipients and their families report improved quality of life and better rehabilitation to either the classroom or the work force compared with those on dialysis.
Renal diseases in childhood cannot be described as common. Yet they are sufficiently frequent and chronic to merit our attention, particularly because of the nutritional challenges that must be overcome if they are to be managed successfully. The kidneys’ multiple metabolic roles, most notably in maintaining the physiologic state described by the French physiologist, Claude Bernard, as homeostasis, explain the multi-system manifestations of these diseases, these diseases’ ability to affect growth and development profoundly, and the secondary involvement of the renal system in systemic diseases not of renal origin.