Autosomal Dominant Polycystic Kidney Disease With Liver and Pancreatic Involvement in Early Childhood

Microstructure and development of the normal and pathologic biliary tract in humans, including blood supply

“…This is an autosomal dominant disease affecting the liver, and the responsible gene(s) are now being identified. In contrast to ductal plate malformation, this disease become manifest in early childhood or even after adolescence (Milutinovic et al, 1989). It is frequently associated with biliary microhamartomas (Ramos et al, 1990) as well as variable dilatation of the peribiliary glands (Kida et al, 1992;Nakanuma et al, 1984).…”

Fine Needle Aspiration Cytology of a Pancreatic Cyst in a Patient with Autosomal Dominant Polycystic Kidney Disease

“…18 The major hereditary type is due to an autosomal dominant gene on chromosomes 16 and 4 (ADPKD-1 and ADPKD-2, respectively), with the minor type due to a recessive gene on chromosome 6. 18 ADPKD is the most common hereditary kidney disorder, 18,20,35 with most patients having variable clinical expression. 18 This systemic, monogenetic disorder affects > 500,000 individuals in the United States and is more common than cystic fibrosis, Huntington's disease and sickle cell anemia.…”

“…18,35 Although it is unusual for pancreatic cysts to be found in patients under 20 years of age, there have been occasional reports of cysts in childhood. 19,20 Usually a solitary cyst is found in the pancreas, in contrast to multiple cysts in other organs. 35 In an ultrasonography study of pancreatic cysts in ADPKD, all cysts were without calcification, with no preferential location in the pancreas, and there was no correlation between the size of the kidney cysts and the size of the pancreatic cyst.…”

“…20 The pancreatic cysts are found only in patients with the autosomal dominant type of polycystic kidney disease, with no cases reported to occur in patients with autosomal recessive polycystic kidney disease or acquired cystic kidney disease. 18 Although it is thought that no complications have been related to pancreatic cysts in ADPKD, 35 it is speculated that cysts may become symptomatic when reaching a large size or becoming infected.…”

Surgical management of polycystic liver disease

“…Finally, patients with ADPKD may have asymptomatic cysts within multiple organs, including the pancreas, spleen, ovaries, and lungs. Pancreatic cysts are the most common of the extrarenal cysts with a reported incidence of 9% among ADPKD patients over 30 years [34][35][36] .…”