IBAT inhibitors for the treatment of liver diseases
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.
1. A method for decreasing the concentration of serum bile acids in a subject in need thereof, comprising orally administering to the subject a therapeutically effective amount of an IBAT inhibitor, wherein the IBAT inhibitor is 1,1-dioxo-3,3-dibutyl-5-phenyl-7-methylthio-8-(N-{(R)-α-[N—((S)-1-carboxypropyl)carbamoyl]-4-hydroxybenzyl}carbamoylmethoxy)-2,3,4,5-tetrahydro-1,2,5-benzothiadiazepine, or a pharmaceutically acceptable salt thereof;
wherein the subject has a disease selected from the group consisting of: Alagilles syndrome (ALGS), progressive familial intrahepatic cholestasis (PFIC), primary biliary cirrhosis (PBC), liver fibrosis, non alcoholic fatty liver disease (NAFLD), non-alcoholic steatohepatitis (NASH), primary sclerosing cholangitis (PSC), intrahepatic cholestasis, extrahepatic cholestasis, and combinations thereof.
2. The method of claim 1 , wherein the subject is a pediatric subject.
3. The method of claim 1 , wherein the subject has PFIC.
4. The method of claim 1 , wherein the subject has ALGS.
5. The method of claim 1 , wherein the subject has intrahepatic cholestasis.
6. The method of claim 1 , wherein the subject has extrahepatic cholestasis.
7. The method of claim 1 , wherein the subject has pruritus as a comorbidity.
8. The method of claim 1 , wherein the subject has pruritus as a symptom of the disease.
9. The method of claim 1 , further comprising administering a therapeutically effective amount of a bile acid binder.
10. The method of claim 9 , wherein the bile acid binder is a resin.
11. The method of claim 10 , wherein the resin is selected from the group consisting of: cholestyramine, cholestipol, and colesevelam.
12. The method of claim 11 , wherein the resin is cholestyramine, or a pharmaceutically acceptable salt thereof.