Richard J Thompson Selected Research

Intrahepatic Cholestasis

1/2022	Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study.
1/2021	Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency.
1/2021	Mutation Analysis and Disease Features at Presentation in a Multi-Center Cohort of Children With Monogenic Cholestasis.
1/2021	Cholestasis Due to USP53 Deficiency.
5/2018	Outcomes of surgical management of familial intrahepatic cholestasis 1 and bile salt export protein deficiencies.
5/2014	Massive gene amplification drives paediatric hepatocellular carcinoma caused by bile salt export pump deficiency.
7/2010	Differences in presentation and progression between severe FIC1 and BSEP deficiencies.
2/2009	Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing.
10/2008	Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion.

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Richard J Thompson Research Topics

Disease

13	Cholestasis 04/2022 - 11/2002
9	Intrahepatic Cholestasis 01/2022 - 10/2008
8	progressive familial intrahepatic 1 Cholestasis 01/2022 - 03/2004
7	Pruritus (Itching) 01/2022 - 01/2017
7	Liver Diseases (Liver Disease) 01/2022 - 04/2008
4	Hepatocellular Carcinoma (Hepatoma) 12/2015 - 08/2006
3	Neoplasms (Cancer) 08/2022 - 04/2008
3	End Stage Liver Disease 01/2020 - 12/2016
2	Alagille Syndrome (Alagille-Watson Syndrome) 01/2022 - 11/2007
2	Sclerosing Cholangitis 01/2017 - 12/2016
1	Carcinogenesis 04/2022
1	Liver Failure 04/2022
1	Transaldolase Deficiency 01/2022
1	Fever (Fevers) 01/2022
1	Cholelithiasis 12/2020
1	Cytomegalovirus Infections (Inclusion Disease) 01/2020
1	Biliary Atresia 01/2019
1	Protein Deficiency 05/2018
1	Fibrosis (Cirrhosis) 05/2018
1	Hypopituitarism (Sheehan's Syndrome) 01/2018
1	Hyperbilirubinemia 01/2018
1	Septo-Optic Dysplasia 01/2018
1	Intrahepatic Cholestasis of Pregnancy 01/2017
1	Herpes Zoster 07/2015
1	Inflammation (Inflammations) 05/2014
1	Disease Progression 10/2008
1	Neonatal hemochromatosis 08/2006
1	Arthrogryposis renal dysfunction cholestasis syndrome 04/2004
1	Arthrogryposis 04/2004
1	Headache (Headaches) 10/2003
1	Fissure in Ano (Anal Fissure) 10/2003
1	Pseudohypoaldosteronism 07/2002

Drug/Important Bio-Agent (IBA)

11	Bile Acids and Salts (Bile Acids)IBA 01/2022 - 03/2004
9	Member 11 Subfamily B ATP Binding Cassette TransporterIBA 01/2022 - 08/2006
9	Proteins (Proteins, Gene)FDA Link 01/2022 - 04/2008
3	Zonula Occludens-2 ProteinIBA 04/2022 - 04/2014
3	gamma-Glutamyltransferase (gamma-Glutamyl Transpeptidase)IBA 01/2022 - 04/2004
3	Phospholipids (Phosphatides)FDA LinkGeneric 01/2021 - 12/2020
2	odevixibatIBA 01/2022 - 01/2021
2	maralixibatIBA 01/2022 - 01/2022
2	alpha-Fetoproteins (alpha-Fetoprotein)IBA 01/2022 - 05/2021
2	DNA (Deoxyribonucleic Acid)IBA 01/2021 - 01/2019
2	Adenosine Triphosphatases (ATPase)IBA 01/2021 - 01/2021
2	bile acid binding proteinsIBA 01/2021 - 08/2006
2	Messenger RNA (mRNA)IBA 11/2007 - 03/2004
1	Adenosine Triphosphate (ATP)IBA 01/2021
1	Hormones (Hormone)IBA 12/2020
1	3,5-diisopropylsalicylic acid (DIPS)IBA 12/2020
1	Alanine Transaminase (SGPT)IBA 01/2020
1	AntipruriticsIBA 01/2020
1	BilirubinIBA 01/2020
1	Myosins (Myosin)IBA 01/2020
1	Carrier Proteins (Binding Protein)IBA 01/2018
1	LipidsIBA 10/2008
1	MMEIBA 11/2007
1	Neprilysin (Neutral Endopeptidase)IBA 11/2007
1	sodium-bile acid cotransporterIBA 03/2004
1	Nitroglycerin (Dynamite)FDA LinkGeneric 10/2003
1	Ointments (Pastes)IBA 10/2003
1	Pharmaceutical PreparationsIBA 11/2002
1	AldosteroneIBA 07/2002
1	Epithelial Sodium ChannelsIBA 07/2002

Therapy/Procedure

4	Liver Transplantation 01/2020 - 12/2016
1	Surgical Portasystemic Shunt (Portosystemic Shunt) 08/2022
1	Operative Surgical Procedures 05/2018
1	Transplantation 05/2018
1	Precision Medicine 04/2015
1	Therapeutics 02/2009