THE AUTOSOMAL-DOMINANT POLYCYSTIC KIDNEY-DISEASE - PATHOGENESIS AND CLINICAL PROGRESSION

The autosomal dominant polycystic kidney disease pathogenesis and clinical progression. The polycystic kidney disease belongs to the most common hereditary autosomal dominant disorders. It is characterized as a systemic disease with not only renal bur. also extrarenal manifestations to a variable extent. The fact that the disease accounts for 10% of the dialysis population offers a challenge for the nephrologist. This indicates that a major part of the affected patients reach end stage renal failure due to the progression of the disease. The morbidity and mortality is mainly determined by the renal manifestation. Ar present exist limited therapeutic possibilities despite new insights on the pathogenesis of cyst growth and advances in genetics. It is important to achieve an early diagnosis and to realize risk constellations. A sufficient treatment of factors affecting the progression as hypertension, cyst: growth and urinary tract infections can favourably influence the course of the disease. There may be hopes that the identification of the genes allow a better understanding of the pathogenetic mechanisms and offer new therapeutic approaches.