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Primary sclerosing cholangitis — Clinical Guidelines

Overview

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive fibro-inflammatory obliteration of the bile ducts, often associated with inflammatory bowel disease (IBD), particularly ulcerative colitis 1 8.

Diagnosis

Primary Diagnostic Tool: Magnetic resonance cholangiopancreatography (MRCP) is recommended for confirming PSC diagnosis 6.

Confirmatory Test: Endoscopic retrograde cholangiopancreatography (ERCP) is performed when therapeutic intervention is needed 6.

Monitoring: Regular monitoring of liver function tests, imaging, and clinical symptoms is essential 1.

Management

First-Line Treatment: High-dose ursodeoxycholic acid (UDCA) is recommended for PSC patients 6.

Adjunctive Therapies: Vitamin D supplementation may be considered due to frequent deficiencies and potential impact on disease progression 2.

Endoscopic Interventions: ERCP for therapeutic purposes such as stricture dilation and stent placement 6.

Management of Complications: Direct percutaneous embolization for bleeding stomal varices in patients with portal hypertension 5.

Special Populations

Pediatrics: Diagnosis and management guidelines exist, emphasizing tailored approaches for children with IBD and PSC 1.

Comorbidities: Specific attention to complications like peristomal varices in patients post-proctocolectomy 14.

Elderly: No specific guidelines provided in the abstracts; general management principles apply with consideration for comorbidities 6.

Key Recommendations

Use magnetic resonance cholangiopancreatography (MRCP) for diagnosis of PSC (Evidence: Strong 6).

Initiate high-dose ursodeoxycholic acid (UDCA) as first-line treatment for PSC patients (Evidence: Moderate 6).

Monitor for and address severe vitamin D deficiency, given its potential impact on disease progression (Evidence: Moderate 2).

Validate adult prognostic models cautiously in pediatric PSC patients due to lack of specific validation (Evidence: Weak 4).

Consider endoscopic interventions like ERCP for managing biliary strictures and complications (Evidence: Moderate 6).

References

1 van Rheenen PF, Kolho KL, Russell RK, Aloi M, Deganello A, Hussey S et al.. Primary sclerosing cholangitis in children with inflammatory bowel disease: An ESPGHAN position paper from the Hepatology Committee and the IBD Porto group. Journal of pediatric gastroenterology and nutrition 2025. link 2 Ebadi M, Rider E, Tsai C, Wang S, Lytvyak E, Mason A et al.. Prognostic Significance of Severe Vitamin D Deficiency in Patients with Primary Sclerosing Cholangitis. Nutrients 2023. link 3 Chazouillères O, Potier P, Bouzbib C, Hanslik B, Heurgue A, NGuyen-Khac E et al.. Non-invasive diagnosis and follow-up of primary sclerosing cholangitis. Clinics and research in hepatology and gastroenterology 2022. link 4 Deneau MR, Valentino PL, Mack C, Alqoaer K, Amin M, Amir AZ et al.. Assessing the Validity of Adult-derived Prognostic Models for Primary Sclerosing Cholangitis Outcomes in Children. Journal of pediatric gastroenterology and nutrition 2020. link 5 Arulraj R, Mangat KS, Tripathi D. Embolization of bleeding stomal varices by direct percutaneous approach. Cardiovascular and interventional radiology 2011. link 6 Tischendorf JJ, Geier A, Trautwein C. Current diagnosis and management of primary sclerosing cholangitis. Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 2008. link 7 Raymondos K, Panning B, Bachem I, Manns MP, Piepenbrock S, Meier PN. Evaluation of endoscopic retrograde cholangiopancreatography under conscious sedation and general anesthesia. Endoscopy 2002. link 8 Bergquist A, Broomé U. Clinical features in primary sclerosing cholangitis. Clinics in liver disease 1998. link70008-8) 9 Imrie CW, Brombacher GD. Sclerosing cholangitis: a rare etiology for acute pancreatitis. International journal of pancreatology : official journal of the International Association of Pancreatology 1998. link 10 Jorgensen RA, Lindor KD, Sartin JS, LaRusso NF, Wiesner RH. Serum lipid and fat-soluble vitamin levels in primary sclerosing cholangitis. Journal of clinical gastroenterology 1995. link 11 Sood GK, Vij JC, Mohapatra JR, Baijal SS, Broor SL. Primary sclerosing cholangitis with chronic pancreatitis. Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology 1990. link 12 Jonard P, Geubel A, Wallon J, Rahier J, Dive C, Meunier H. Primary sclerosing cholangitis and idiopathic pulmonary fibrosis: a case report. Acta clinica Belgica 1989. link 13 MacSween RN, Burt AD, Haboubi NY. Unusual variant of primary sclerosing cholangitis. Journal of clinical pathology 1987. link 14 Wiesner RH, LaRusso NF, Dozois RR, Beaver SJ. Peristomal varices after proctocolectomy in patients with primary sclerosing cholangitis. Gastroenterology 1986. link90926-1)

Primary sclerosing cholangitis