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Slovenska pediatrija 2015; 22: 266-282

https://doi.org/



Review article

TREATMENT OF END STAGE RENAL DISEASE DUE TO HEREDITARY AND GENETIC KIDNEY DISEASES

N. Battelino
Klinični oddelek za otroško nefrologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

Chronic kidney disease (CKD) is an important health problem in children and adolescents, although it is much less frequent than in adults and the causes in children differ from those in the adult population. CKD can further progress to end-stage renal disease (ESRD), the rate of progress depending on the primary cause of CKD. The most common causes of ESRD in children are congenital anomalies of the kidney and urinary tract (CAKUT), followed by focal segmental glomerulosclerosis (FSGS), ciliopathies, chronic glomerulonephritides, haemolytic uraemic syndrome (HUS), nephritis in systemic lupus erythematosus, cystinosis, and primary hyperoxaluria (1). The majority of causes of CKD and ESRD in children are genetic diseases. Renal transplantation is the optimal form of renal replacement therapy (RRT) in children. Unfortunately, the primary disease can recur in the renal graft, this being observed more frequently in children than in adults. New insights into the pathophysiology of most kidney diseases have dramatically improved in the past few years. These improvements give hope and offer possibilities for the development of novel treatment strategies and support a more individualized approach to the affected child before and after transplantation.

Key words: End-stage renal disease, children, hereditary diseases, dialysis, transplantation.


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