Risk Factors for the Development of Hepatic Cysts in Autosomal Dominant Polycystic Kidney Disease

Abstract

Hepatic cysts are a major manifestation of autosomal dominant polycystic kidney disease. This study examined 239 autosomal dominant polycystic kidney disease patients and 189 unaffected family members to define the factors that influence the presence and severity of hepatic cysts. Autosomal dominant polycystic kidney disease patients with hepatic cysts were older than autosomal dominant polycystic kidney disease patients without such cysts (44.6 +/- 1.1 yr vs. 32.9 +/- 1.1 yr; p less than 0.0001). The number of hepatic cysts increased with age (r = 0.43; p less than 0.0001). Women were more likely to have massive hepatic cystic disease (greater than 15 cysts) than men (p less than 0.04). Women also had larger maximal cyst size (4.2 +/- 0.4 cm vs. 2.7 +/- 0.3 cm; p less than 0.004). Women with hepatic cysts were more likely to have been pregnant (p less than 0.001) and to have had more pregnancies (2.9 +/- 0.3 pregnancies vs. 1.6 +/- 0.2 pregnancies; p less than 0.0009). Kidney volume (p less than 0.0001), number of cysts (p less than 0.004), percentage of cystic parenchyma (p less than 0.001) and predominant cyst size (p less than 0.001) were greater and creatinine clearance was lower (64.5 +/- 3.1 ml/min/1.73 m2 vs. 94.5 +/- 3.4 ml/min/1.73 m2; p less than 0.001) in autosomal dominant polycystic kidney disease patients with hepatic cysts. By logistic regression, the frequency of hepatic cysts was related to increased age, increased severity of renal cystic disease and decreased creatinine clearance. Number and size of hepatic cysts correlated with the occurrence of pregnancy, female gender, increased age and severity of the renal lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Liver transplantation for adult polycystic liver disease

“…[14][15][16][17] Thirty-four to seventy-eight percent of patients with adult polycystic kidney disease eventually develop hepatic cysts, and few patients with APLD do not have associated renal cysts. 18,19 Hepatic cysts usually remain asymptomatic but some patients may develop complications of their liver disease, including cyst infection, biliary obstruction, and portal hypertension.…”

Medical and surgical treatment options for polycystic liver disease1

“…8 In addition, severity of renal cystic disease, prior pregnancies, and estrogen use predict increase of polycystic liver size in ADPKD. 7,9 Indeed, 1 year of estrogen use in postmenopausal ADPKD patients selectively increases total liver volume by 7%, whereas total kidney volume remains unaffected. 2,10 Symptoms in PLD are probably secondary to the increased total liver volume.…”

“…2 The elevated AP and gGT levels probably reflect activation of cholangiocytes. 9,[23][24][25][26] Serum transaminases are normal or only mildly elevated. 2 Bilirubin is rarely elevated but in advanced cases jaundice may arise due to compression of the common bile duct secondary to a strategically located cyst.…”

Autosomal dominant polycystic kidney disease (ADPKD, MIM 173900, PKD1 and PKD2 genes, protein products known as polycystin-1 and polycystin-2)

“…The number and size of hepatic cysts is also correlated with female gender, parity and severity of the renal disease. 3 Massive polycystic liver disease, although rare, occurs predominantly in female subjects.…”