同: autosomal dominant polycystic liver disease

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1. 肝嚢胞性病変の診断およびマネージメント diagnosis and management of cystic lesions of the liver
2. 肝肥大：鑑別診断および評価 hepatomegaly differential diagnosis and evaluation
3. 常染色体優性多発性嚢胞腎の腎外症状 extrarenal manifestations of autosomal dominant polycystic kidney disease
4. 常染色体優性多発性嚢胞腎の診断およびスクリーニング diagnosis of and screening for autosomal dominant polycystic kidney disease
5. 常染色体優性多発性嚢胞腎の経過および治療 course and treatment of autosomal dominant polycystic kidney disease

English Journal

Ursodeoxycholic acid in advanced polycystic liver disease: A phase 2 multicenter randomized controlled trial.

D'Agnolo HM1, Kievit W2, Takkenberg RB3, Riaño I4, Bujanda L4, Neijenhuis MK1, Brunenberg EJ5, Beuers U3, Banales JM4, Drenth JP6.
Journal of hepatology.J Hepatol.2016 Sep;65(3):601-7. doi: 10.1016/j.jhep.2016.05.009. Epub 2016 May 17.
BACKGROUND & AIMS: Ursodeoxycholic acid (UDCA) inhibits proliferation of polycystic human cholangiocytes in vitro and hepatic cystogenesis in a rat model of polycystic liver disease (PLD) in vivo. Our aim was to test whether UDCA may beneficially affect liver volume in patients with advanced PLD
PMID 27212247

Mutations in GANAB, Encoding the Glucosidase IIα Subunit, Cause Autosomal-Dominant Polycystic Kidney and Liver Disease.

Porath B1, Gainullin VG1, Cornec-Le Gall E2, Dillinger EK3, Heyer CM1, Hopp K4, Edwards ME1, Madsen CD1, Mauritz SR1, Banks CJ1, Baheti S5, Reddy B6, Herrero JI7, Bañales JM8, Hogan MC1, Tasic V9, Watnick TJ10, Chapman AB6, Vigneau C11, Lavainne F12, Audrézet MP13, Ferec C13, Le Meur Y14, Torres VE1; Genkyst Study Group, HALT Progression of Polycystic Kidney Disease Group; Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease, Harris PC15.
American journal of human genetics.Am J Hum Genet.2016 Jun 2;98(6):1193-207. doi: 10.1016/j.ajhg.2016.05.004.
Autosomal-dominant polycystic kidney disease (ADPKD) is a common, progressive, adult-onset disease that is an important cause of end-stage renal disease (ESRD), which requires transplantation or dialysis. Mutations in PKD1 or PKD2 (∼85% and ∼15% of resolved cases, respectively) are the known cau
PMID 27259053

Alkaline phosphatase predicts response in polycystic liver disease during somatostatin analogue therapy: a pooled analysis.

Gevers TJ1, Nevens F2, Torres VE3, Hogan MC3, Drenth JP1.
Liver international : official journal of the International Association for the Study of the Liver.Liver Int.2016 Apr;36(4):595-602. doi: 10.1111/liv.12986. Epub 2015 Nov 9.
BACKGROUND & AIMS: Somatostatin analogues reduce liver volumes in polycystic liver disease. However, patients show considerable variability in treatment responses. Our aim was to identify specific patient, disease or treatment characteristics that predict response in polycystic liver disease dur
PMID 26481454

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