Current Therapeutics in Primary Sclerosing Cholangitis

Tan, Natassia; Lubel, John S; Kemp, William; Roberts, Stuart K.; Majeed, Ammar

doi:10.14218/jcth.2022.00068s

Cited by 3 publications

(4 citation statements)

References 164 publications

(348 reference statements)

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“…Several reports have documented the use of MCA in conjunction with ERCP for treating severe benign biliary strictures[ 6 - 8 ]. The magnet placement process is easier when the guide wire can pass through the stenosis.…”

Section: Discussionmentioning

confidence: 99%

“…These strictures can typically be treated with endoscopic balloon dilation and biliary stent implantation[ 4 , 5 ]. However, in cases with severe stenosis or occlusion, endoscopic retrograde cholangiopancreatography (ERCP) treatment may not be successful[ 6 ]. Percutaneous transhepatic biliary drainage is currently the only non-surgical treatment option available for these patients.…”

Section: Introductionmentioning

confidence: 99%

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Magnetic compression anastomosis to restore biliary tract continuity after obstruction following major abdominal trauma: A case report

Zhang,

Tao,

Sha

et al. 2024

World J Gastrointest Surg

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BACKGROUND The combination of magnetic compression anastomosis (MCA) and endoscopy has been used to treat biliary stricture after liver transplantation. However, its use for the treatment of complex biliary obstruction after major abdominal trauma has not been reported. This case report describes the successful use of MCA for the treatment of biliary obstruction resulting from major abdominal trauma. CASE SUMMARY A 23-year-old man underwent major abdominal surgery (repair of liver rupture, right half colon resection, and ileostomy) following a car accident one year ago. The abdominal drainage tube, positioned at the Winslow foramen, was draining approximately 600-800 mL of bile per day. During the two endoscopic retrograde cholangiopancreatography procedures, the guide wire was unable to enter the common bile duct, which prevented placement of a biliary stent. MCA combined with endoscopy was used to successfully achieve magnetic anastomosis of the peritoneal sinus tract and duodenum, and then a choledochoduodenal stent was placed. Finally, the external biliary drainage tube was removed. The patient achieved internal biliary drainage leading to the removal of the external biliary drainage tube, which improved the quality of life. CONCLUSION Magnetic compression technique can be used for the treatment of complex biliary obstruction with minimal operative trauma.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Magnetic compression anastomosis to restore biliary tract continuity after obstruction following major abdominal trauma: A case report

Zhang,

Tao,

Sha

et al. 2024

World J Gastrointest Surg

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“…Cholangiocarcinoma is currently one of the most common causes of death in PSC patients who do not undergo transplantation. 37,38 The usual manifestations are epigastric pain, liver biochemical deterioration, jaundice, and elevated serum CA19.9. Chemotherapy remains the primary palliative treatment for patients with cholangiocarcinoma.…”

Section: Management Of Complications Of Pscmentioning

confidence: 99%

Progress in the Management of Patients with Cholestatic Liver Disease: Where Are We and Where Are We Going?

Luo,

2024

J Clin Transl Hepatol

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Cholestatic liver disease is a group of diseases in which bile acid accumulates in the liver for various reasons, resulting in abnormal liver biochemical indicators and histological damage. Cholestasis can be divided into intrahepatic cholestasis and extrahepatic cholestasis, which will contribute to liver damage and progress to liver fibrosis and cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis are the two most typical cholestatic liver diseases. Ursodeoxycholic acid is currently the first-line treatment for PBC, while obeticholic acid, budesonide and fibrates have also shown good potential in the treatment of PBC. There are currently no official drugs approved to treat primary sclerosing cholangitis, and the use of ursodeoxycholic acid may have certain clinical benefits. At present,

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“…PSC patients can progress to liver cirrhosis and have a great risk of cholangiocarcinoma with liver failure at the end stage. PSC has been considered an orphan disease since the prevalence of PSC is up to 16.2 per 100000 population with variable distribution due to different regions[ 3 - 5 ]. Although the occurrence of PSC is rare, it showed significant morbidity and mortality in PSC patients as there is a very limited medical option to interfere with the course of PSC, and liver transplant is the exclusively therapeutic option at the end-stage of the disease[ 1 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Recombinant adeno-associated virus 8-mediated inhibition of microRNA let-7a ameliorates sclerosing cholangitis in a clinically relevant mouse model

Hua,

Zhao,

Kalagbor

et al. 2024

World J Gastroenterol

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BACKGROUND Primary sclerosing cholangitis (PSC) is characterized by chronic inflammation and it predisposes to cholangiocarcinoma due to lack of effective treatment options. Recombinant adeno-associated virus (rAAV) provides a promising platform for gene therapy on such kinds of diseases. A microRNA (miRNA) let-7a has been reported to be associated with the progress of PSC but the potential therapeutic implication of inhibition of let-7a on PSC has not been evaluated. AIM To investigate the therapeutic effects of inhibition of a miRNA let-7a transferred by recombinant adeno-associated virus 8 (rAAV8) on a xenobiotic-induced mouse model of sclerosing cholangitis. METHODS A xenobiotic-induced mouse model of sclerosing cholangitis was induced by 0.1% 3,5-Diethoxycarbonyl-1,4-Dihydrocollidine (DDC) feeding for 2 wk or 6 wk. A single dose of rAAV8-mediated anti-let-7a-5p sponges or scramble control was injected in vivo into mice onset of DDC feeding. Upon sacrifice, the liver and the serum were collected from each mouse. The hepatobiliary injuries, hepatic inflammation and fibrosis were evaluated. The targets of let-7a-5p and downstream molecule NF-κB were detected using Western blot. RESULTS rAAV8-mediated anti-let-7a-5p sponges can depress the expression of let-7a-5p in mice after DDC feeding for 2 wk or 6 wk. The reduced expression of let-7a-5p can alleviate hepato-biliary injuries indicated by serum markers, and prevent the proliferation of cholangiocytes and biliary fibrosis. Furthermore, inhibition of let-7a mediated by rAAV8 can increase the expression of potential target molecules such as suppressor of cytokine signaling 1 and Dectin1, which consequently inhibit of NF-κB-mediated hepatic inflammation. CONCLUSION Our study demonstrates that a rAAV8 vector designed for liver-specific inhibition of let-7a-5p can potently ameliorate symptoms in a xenobiotic-induced mouse model of sclerosing cholangitis, which provides a possible clinical translation of PSC of human.

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Current Therapeutics in Primary Sclerosing Cholangitis

Cited by 3 publications

References 164 publications

Magnetic compression anastomosis to restore biliary tract continuity after obstruction following major abdominal trauma: A case report

Magnetic compression anastomosis to restore biliary tract continuity after obstruction following major abdominal trauma: A case report

Progress in the Management of Patients with Cholestatic Liver Disease: Where Are We and Where Are We Going?

Recombinant adeno-associated virus 8-mediated inhibition of microRNA let-7a ameliorates sclerosing cholangitis in a clinically relevant mouse model

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