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progressive familial intrahepatic 1 Cholestasis

A heterogeneous group of hereditary liver disorders characterized by the early onset of CHOLESTASIS that progresses to hepatic FIBROSIS; LIVER CIRRHOSIS, and END-STAGE LIVER DISEASE before adulthood. Germline mutations in the ATP8B1 gene have been identified. OMIM: 211600
Also Known As:
Cholestasis, progressive familial intrahepatic 1; Byler disease; Byler's disease; Cholestasis, Benign Recurrent Intrahepatic, 1; Cholestasis, Progressive Familial Intrahepatic, 1; Cholestasis, benign recurrent intrahepatic 1; Cholestasis, fatal intrahepatic; FIC1 deficiency; PFIC1 Progressive familial intrahepatic cholestasis type 1; Progressive familial intrahepatic cholestasis; Progressive familial intrahepatic cholestasis type 1 (PFIC 1); Progressive familial intrahepatic cholestasis type 1 (PFIC1); Summerskill syndrome
Networked: 350 relevant articles (13 outcomes, 21 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Pruritus (Itching)
2. Fibrosis (Cirrhosis)
3. Cholestasis
4. Alagille Syndrome (Alagille-Watson Syndrome)
5. Intrahepatic Cholestasis

Experts

1. Jacquemin, Emmanuel: 19 articles (01/2021 - 05/2003)
2. Gonzales, Emmanuel: 15 articles (01/2021 - 01/2009)
3. Knisely, A S: 14 articles (01/2021 - 03/2000)
4. Jankowska, Irena: 11 articles (01/2022 - 01/2002)
5. Davit-Spraul, Anne: 11 articles (01/2021 - 01/2009)
6. Hayashi, Hisamitsu: 10 articles (01/2021 - 04/2005)
7. Shneider, Benjamin L: 9 articles (01/2021 - 03/2004)
8. Kubitz, Ralf: 9 articles (05/2018 - 05/2005)
9. Chen, Huey-Ling: 8 articles (01/2022 - 09/2008)
10. Thompson, Richard J: 8 articles (01/2022 - 03/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to progressive familial intrahepatic 1 Cholestasis:
1. Ursodeoxycholic Acid (Urso)FDA LinkGeneric
2. 4-phenylbutyric acid (Buphenyl)FDA Link
3. Bile Acids and Salts (Bile Acids)IBA
4. odevixibatIBA
5. bile acid binding proteinsIBA
6. VitaminsIBA
7. Nonsense Codon (Nonsense Mutation)IBA
8. Collagenases (Collagenase)FDA Link
9. SaltsIBA
10. Nitroglycerin (Dynamite)FDA LinkGeneric

Therapies and Procedures

1. Liver Transplantation
2. Therapeutics
3. Aftercare (After-Treatment)
4. Hepatectomy
5. Transplantation