detoxification of the liver in psc (dolphin)

Conditions: Primary Sclerosing Cholangitis

Summary: this study is a clinical trial being done to investigate the efficacy of drug brs201 (hydroxocobalamin) as a treatment in patients with primary sclerosing cholangitis. participation in this study will take 8 weeks long and the study is structured as a cross-over study in which participants will take the study drug for 4 weeks and a placebo drug for 4 weeks in a randomized order in the form of an oral medication. participation may also involve receiving an iv dose of the medication. the study will require participants to attend 9 study visits, all of which will be remote. participation will involve taking an oral medication twice daily, tracking the medication in a log, and getting blood drawn and giving a stool sample for a few lab tests throughout the study. for the lab tests, a research nurse will visit the participant in-home for the convenience of the participant.


Eligibility criteria:
inclusion criteria: * a diagnosis of psc for at least 6 months based upon cholangiography (ercp or mrcp) demonstrating intrahepatic and/or extrahepatic biliary strictures, beading or irregularity consistent with psc. * alp \> 1.5 times the upper limit of normal (uln) at screening. * subject must either be on a stable dose of ursodeoxycholic acid for \> 6 months prior to screening or have been discontinued \> 4 weeks prior to screening (enrollment of patients who are on udca will be limited to 60% of all enrolled patients). exclusion criteria: * anticipated need for liver transplant within one year as determined by mayo psc risk score * evidence of decompensated liver disease such as variceal bleeding, ascites, or hepatic encephalopathy. * evidence of advanced liver disease including meld score \> 10, bilirubin \> 3.0, platelet count \ 1.4 * concomitant chronic liver disease including alcohol related liver disease, chronic hepatitis b or c infection, haemochromatosis, wilson's disease, alpha1-antitrypsin deficiency, non-alcoholic steatohepatitis, autoimmune hepatitis, or primary biliary cholangitis * secondary causes of sclerosing cholangitis * patients who have a confirmed malignancy or cancer within 5 years except non-melanoma skin cancers * treatment with any investigational agents, within two months or 5 half-lives of the investigational product, whichever is longer. * active illicit drug or more than moderate alcohol consumption. * evidence of bacterial cholangitis within 6 months of enrollment * in patients with ulcerative colitis, or, if crohn's disease, a need for additional therapy at time of screening. * chronic kidney injury (egfr \< 60) * pregnancy or lactation * uncontrolled hypertension with a systolic bp \> 140 and a systolic bp \> 90 * prohibited medications: current use of vitamin c and prednisone * patients with a history or risk of cardiovascular conditions, including arrhythmia, long qt syndrome, congestive heart failure, stroke, or coronary artery disease * patients with a history of kidney stones * congenital or acquired immunodeficiencies * other comorbidities including: diabetes mellitus, systemic lupus * an episode of acute cholangitis within 4 weeks of screening