【已刪除】94 下列何種囊性腎病變（renal cystic disease）最常合併肝門脈區纖維化？
(A)兒童多囊性腎病（childhood polycystic kidney disease）
(B)成人多囊性腎病（adult polycystic kidney disease）
(C)成人腎髓質囊性病（adult-onset medullary cystic disease）
(D)後天性囊性腎病（acquired renal cystic disease）

Infantile polycystic kidney dz, 

auto  recessive;   therefore it is present  at  birth. Do   you   think  this  baby   is   urinating?   No,   therefore   has oligohydramnios (decreased amniotic fluid).  So, baby is in an amniotic sac, with hardly any amniotic fluid around it, and therefore have malformation due to pressure.  Look at the nose and ears; this is called Potters face, which is a sign of ligohydramnios in polycystic kidney dz: flattened nose, low-set ears, and recessed chin).  This child wasn’t able to breath, and when it tried to breath, it couldn’t; the lungs are hypoplastic – they never fully developed b/c the kid couldn’t fill them up.  These cysts are also seen in the pancreas, the liver and just incompatible with life.  

Adult polycystic kidney disease: APKDz

autosomal dominant dz that is not present at birth b/c AD dz have delayed manifestations. See cysts by 10-12   years   of   age,   always   get  HTN   which   will   then  predisposes   2   types   of   bleeds:  

(1)  Charcot-Bouchard aneurysms (a blood clot)

(2) see blood all over the brain, due to subarachnoid hemorrhage, therefore the blood is due to rupture berry aneurysm.  Subarachnoid hemorrhage = “worst headache of my life”, blood in subarachnoid space. 

Autosomal dominant polycystic kidney disease (ADPKD) 

ARPKD is characterized by non-obstructive fusiform dilatation of the renal collecting ducts and malformations of the biliary tract, with ectasia of the bile ducts and periportal fibrosis . It can manifest in neonates with exaggerated kidney growth, intrauterine renal failure and pulmonary hypoplasia, or may present later with renal failure accompanied by portal and systemic hypertension. ARPKD is associated with a high level of morbidity and mortality in affected individuals who require close monitoring, surgical shunting procedures, and kidney and/or liver transplantation.

https://www.ncbi.nlm.nih.gov/books/NBK373392/