polycystic

polycystic kidney disease either of two unrelated hereditary diseases in which there is massive enlargement of the kidney with cyst formation. It occurs in two forms, distinguished by age of onset and other characteristics.

Autosomal dominant polycystic kidney disease (ADPKD, formerly called adult polycystic kidney disease) is the most common type of cystic disease of the kidneys. It is usually manifested during the third decade of life. Renal failure may appear by the fifth decade, with terminal failure occurring in the next ten years, although in some cases it never appears. Although there is rarely any liver dysfunction accompanying this disorder, cyst formation in the liver does occur.

Autosomal recessive polycystic kidney disease (ARPKD), formerly called childhood polycystic kidney disease, is diagnosed at birth or in the first ten years of life and is much less common than the autosomal dominant form. Both the kidney and the liver are involved, causing renal failure and liver failure with portal hypertension. Characteristic symptoms early in the process include pain, hematuria, urinary tract infection, kidney stones, and obstructive uropathy with anuria.

Treatment of both types of polycystic kidney disease is largely symptomatic. Renal dialysis and kidney transplantation during end-stage renal disease can prolong life but offer no cure. Families with histories of polycystic kidney disease require genetic counseling and may need help in coping with the prospect of future offspring afflicted with the disease.

polycystic ovary syndrome (PCOS) a clinical symptom complex associated with polycystic ovaries and characterized by oligomenorrhea or amenorrhea, anovulation (hence infertility), and hirsutism. Both hyperestrogenism (from peripheral conversion of androgen) and hyperandrogenism are present. Excretion of follicle-stimulating hormone and 17-ketosteroids is normal, but infertility is usually persistent, requiring treatment with wedge resection, clomiphene, or gonadotropins. Called also Stein-Leventhal syndrome.

polycystic renal disease polycystic kidney disease.