Claims for Patent: 9,694,018

Summary for Patent: 9,694,018

Title:	IBAT inhibitors for the treatment of liver disease
Abstract:	The present invention regards specific IBAT inhibitors useful in the prophylaxis and/or treatment of a liver disease. It also relates to compositions comprising these IBAT inhibitors, a method for treatment of the disorders and a kit comprising the substances or the compositions.
Inventor(s):	Gillberg; Per-Goran (Molndal, SE), Graffner; Hans (Helsingborg, SE), Starke; Ingemar (Gothenburg, SE)
Assignee:	Albireo AB (Goteborg, SE)
Application Number:	15/276,446
Patent Claims:	1. A method for the treatment of a cholestatic liver disease selected from the group consisting of Alagille syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), comprising orally administering to a subject in need of such treatment a therapeutically effective amount of an IBAT inhibitor, wherein the IBAT inhibitor is of 1,1-dioxo-3,3-dibutyl-5-phenyl-7-methylthio-8-(N--{(R)-.alpha.-[N--((S)-1- -carboxypropyl) carbamoyl]-4-hydroxybenzyl}carbamoylmethoxy)-2,3,4,5-tetrahydro-1,2,5-ben- zothiadiazepine, or a pharmaceutically acceptable salt thereof. 2. The method of claim 1, wherein treatment of the cholestatic liver disease comprises treatment of pruritus. 3. The method of claim 1, wherein treatment of the cholestatic liver disease comprises decreasing the level of serum bile acids in the subject. 4. The method of claim 1, wherein the cholestatic liver disease is ALGS (Alagille syndrome). 5. The method of claim 1, wherein the cholestatic liver disease is PFIC (progressive familial intrahepatic cholestasis). 6. The method of claim 1, wherein the cholestatic liver disease is primary biliary cirrhosis (PBC). 7. The method of claim 1, wherein the cholestatic liver disease is primary sclerosing cholangitis (PSC). 8. The method of claim 1, wherein the cholestatic liver disease is a pediatric cholestatic liver disease selected from the group consisting of ALGS (Alagille syndrome) and PFIC (progressive familial intrahepatic cholestasis). 9. The method of claim 8, wherein the pediatric cholestatic liver disease is ALGS (Alagille syndrome). 10. The method of claim 8, wherein the pediatric cholestatic liver disease selected is PFIC (progressive familial intrahepatic cholestasis). 11. A method for the treatment of a cholestatic liver disease selected from the group consisting of ALGS (Alagille syndrome) and PFIC (progressive familial intrahepatic cholestasis) comprising orally administering to a subject in need of such treatment a therapeutically effective amount of an IBAT inhibitor, wherein the IBAT inhibitor is of 1,1-dioxo-3,3-dibutyl-5-phenyl-7-methylthio-8-(N--{(R)-.alpha.-[N--((S)-1- -carboxypropyl) carbamoyl]-4-hydroxybenzyl}carbamoylmethoxy)-2,3,4,5-tetrahydro-1,2,5-ben- zothiadiazepine, or a pharmaceutically acceptable salt thereof. 12. The method of claim 11, wherein treatment of the cholestatic liver disease comprises treatment of pruritus. 13. The method of claim 11, wherein treatment of the cholestatic liver disease comprises decreasing the level of serum bile acids in the subject. 14. The method of claim 11, wherein the cholestatic liver disease is ALGS (Alagille syndrome). 15. The method of claim 11, wherein the cholestatic liver disease is PFIC (progressive familial intrahepatic cholestasis).